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Pediatric Ophthalmology

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Pediatric Ophthalmology

Dr. P.K. Mukherjee


MS

Former Professor of Upgraded Department of Ophthamology, Pt. JNM Medial College, Raipur

NEW AGE

NEW AGE INTERNATIONAL (P) LIMITED, PUBLISHES


New Delhi l Bangalore l Chennai l Cochin l Guwahati l Hyderabad Jalandhar l Kolkata l Lucknow l Mumbai l Ranchi

Copyright 2005, New Age International (P) Ltd., Publishers Published by New Age International (P) Ltd., Publishers All rights reserved. No part of this ebook may be reproduced in any form, by photostat, microfilm, xerography, or any other means, or incorporated into any information retrieval system, electronic or mechanical, without the written permission of the publisher. All inquiries should be emailed to rights@newagepublishers.com

ISBN : 978-81-224-2310-5

PUBLISHING FOR ONE WORLD

NEW AGE INTERNATIONAL (P) LIMITED, PUBLISHERS 4835/24, Ansari Road, Daryaganj, New Delhi - 110002 Visit us at www.newagepublishers.com

This Book is Dedicated to Those children whose sight could have been saved by timely intervention

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Preface
With fifteen percent of population in pediatric age group and development of pediatrics as a separate discipline ago, it was but natural that many of the specialities in medicine and surgery ramify into their pediatric sub-specialities and ultimately become super-speciality. Development of Pediatric Ophtalmology is part of this useful diversification. In the developed countries pediatric opthalmology has reached a status of super-speciality. Pediatric opthalmology is still being managed by general opthalmologist in developing countries. To overcome enormous volume of ocular morbidity and blindness is children, WHO has included childhood blindness and errors of refraction on priority in its VISION 2020 programme. The Govt. of India on its part has decided to develop the pediatric opthalmology as a separate speciality manned by opthalmologist specially trained in pediatric ophthalmology. Most of the general ophthalmologists are ill at ease in examining a child with ocular problem. They use the adult method of examination to elicit clinical science is small children with frustrating outcome. Such an impasse may be overcome if the training in pediatric opthalmology is initiated at undergraduate level and developed at postgraduate level. This is immensely hindered by paucity of books in pediatric ophthalmology. The present work is an attempt to produce a concise book on pediatric ophthalmology. The book has separate chapters on individual ocular systems. The initials chapters deal with development of eyes in general, peculiarities of eyes in childern and methods of examination suited for such eyes. The other chapters deal with applied anatomy, physiology, detailed embryology of each system, their disease and disorders. The medical management has been discussed briefly. Emphasis has been given to preventive aspects. Surgical procedures have only been outlined. The surgical steps and their details have not been included, for which the students should refer to standard books on ocular surgery. The book is meant as a handbook for postgraduates, teachers and reference book for undergraduates. The book has been written in English that is understood by English speaking students of third world. Some portions in the book on cursory glance may seem to be repetitive. This has been done in intentionally to emphasize the importance of the topics referred to. Dr. P.K. Mukherjee

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Acknowledgement
I am thankful to a large number of my colleagues and friends who initiated me in writing this book. The real inspiration behind this book remains my wife Protima, who prodded me to keep the schedule. My daughters Protibha and Preeti joined their mother in cajoling me. I am thankful to my daughters for persuading thier spouses Satyadeep and Abir respectively to get involved in the project and spend endless hours in typing, arranging and rearranging the text in initial stages. Padma Shree Dr. A.T. Dabke, Prof. and Head of Department of Pediatrics, Dean Pt. J.N.M. Medical College Raipur, gave me free access to his personal library. He was always available to solve any of the pediatric problems that seemed to dodge me. I extend my heartfelt thanks to him. My sincere thanks are due to members of Upgraded Department of Ophthalmology, Pt. J.N.M. Medical College, Raipur, that includes Dr. S.L. Adile, Prof. and H.O.D., Prof. A.K. Chandrakar, Dr. M.L. Garg, Associate Professor, Dr. Nidhi Pandey, Asst. Professor of Ophthalmology, all of them were generous to keep me supplied with books and journals. Dr. Subhash Mishra and Dr. B.K. Das of mobile unit helped me writing the parts concerned with community ophthalmology. I thank them. Dr. Bijoya Sarkar, Prof. of Chest diseases, helped me in writing chapter of systemic disorders and there ocular manifestation. Dr. Manik Chatterjee, Asstt. Prof. of Anatomy helped me in writing the applied anatomy and development of the eye. Dr. B.P. Sharma, Dr. Preeti Gupta gave useful suggestion in writing chapters on retina, vitreous, squint and glaucoma. I extend my heartfelt gratitude to them. Shri Arup Bhattacharya saw me through some difficult parts of the manuscript. Shri Maneesh Dandekar of Hyper Soft Computers, took immense trouble in typing the final printout of entire manuscript. He also managed my day to day correspondence with efficiency. My thanks are to them. The book would not have been possible but for co-operation of all the members of the staff of New Age International (Pvt.) Publishers, New Delhi. I thank them profusedly. Shri Saumya Gupta, Managing Director of New Age International had been kind enough with useful suggestion. He deserves special thanks for it. Dr. P.K. Mukherjee

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Contents
Preface ................................................................................................................................... (vii) Acknowledgement ................................................................................................................... (ix) 1. Outline of Development of the Eye ...................................................................................... 1 2. Examination of the Eyes in Pediatric Patients ................................................................. 11 3. Disorders of Lids in Children .............................................................................................. 34 4. Motility Disorders of the Lid ............................................................................................... 67 5. Disorders of Lacrimal System in Children ........................................................................ 86 6. Disorders of Conjunctiva in Children ............................................................................... 110 7. Disorders of Cornea in Children ....................................................................................... 159 8. Disorders of Uvea in Children .......................................................................................... 227 9. Disorders of Lens in Children ........................................................................................... 290 10. Glaucoma in Children ....................................................................................................... 334 11. Disorders of the Retina and Vitreous in Children ........................................................... 393 12. Retinoblastoma .................................................................................................................. 471 13. Disorders of Pupil, Accommodation and Convergence .................................................... 497 14. Disorders of Optic Nerve in Children ............................................................................... 510 15. Errors of Refraction in Children ....................................................................................... 546 16. Symptomatic Disturbance of Vision in Children ............................................................. 574 17. Disorders of Extra Ocular Muscles and Paralytic Squint in Children........................... 593 18. Nystagmus ......................................................................................................................... 628 19. Non Paralytic Squint in Children ..................................................................................... 637 20. Disorders of Orbit in Children .......................................................................................... 673 21. Disorders of the Sclera in Children .................................................................................. 720 22. Ocular Manifestation of Systemic Disorders in Children ............................................... 725 Index ................................................................................................................................... 778

(xi)

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CHAPTER

Outline of Development of the Eye


GENERAL PRINCIPLES1-8
The layers of embryo become evident by the end of three weeks of gestations these layers are ectoderm, mesoderm and endoderm, the ectoderm is the outer most. The endoderm does not participate in the formation of the eyes. The ectoderm proliferates successively to form neural plate, neural groove, neural fold and neural tube. From the anterior most part of neural tube develops forebrain. A small optic pit becomes evident at this stage, one on each side of primitive forebrain. This pit gradually fills up and starts out pouching on either side of the mid line forming optic vesicle. The optic vesicle has a globular shape with a narrow neck by which the interior of the optic vesicle communicates with interior of forebrain, the optic vesicle enlarges and its vortex touches the inner side of the surface ectoderm. The space surrounding the optic vesicles is filled by para axial mesoderm. The spot where the optic vesicle touches the surface ectoderm is the place that will gradually develop into the lens plate and get separated from the surface ectoderm. The optic vesicle continues to grow after touching the surface ectoderm and its surface bows inside to form a depression called optic cup that has two layers. This two layered cup is not complete it is open distally and inferiorly. If the optic vesicle fails to invaginate, a cystic eye ball results. In later stage the mesoderm will find its access inside the optic cup to form the vasculature of the eye. The axon of ganglion cells will come out to form the optic nerve. This groove under the optic cup is called embryonic or choroidal fissure. It gradually narrows to close down completely. The closure begins in the middle and extends on each end. The closure should be complete by sixth week. If it fails to fuse, the deficiency results in typical coloboma of uvea. Non fusion of posterior end in less common than that of anterior end, hence coloboma of posterior fundus including optic disc is less frequent than coloboma of anterior uvea. Deficiency in mid fundus is least common.

DEVELOPMENT OF INDIVIDUAL STRUCTURES


Development of the Lens The lens is solely ectodermal in origin. The development of the lens begins at an early stage of 4 mm. 1

2 The development of lens has two distinct stages :

PEDIATRIC OPHTHALMOLOGY

1. A short period when the lens vesicle develops between 4.5 mm to 10 mm. 2. A longer period of development of lens fibers that continues even after the birth of the child. The lens fibers are laid down in two phases of primary and secondary fibers. The cells at the spot where the neuro ectoderm has come in contact with the surface ectoderm starts proliferating to form lens plate. The cells on each side of the lens plate increase and inveginate inwards in the form of a depression called lens pit. The pit gradually deepens to form a deeper cavity with an anterior opening. The opening slowly shortens and the cavity is converted into a hollow spherical structure called lens vesicle. The lens vesicle is single layered which ultimately separates from the surface ectoderm and is pushed towards the optic cup to lie freely within the lips of the optic cup. The surface ectoderm quickly bridges the gap and converts it into an uninterrupted layer of surface ectoderm that will form future corneal epithelium. The space between the surface ectoderm and the lens vesicle is invaded by mesoderm. In early stages of development the lens vesicle is a circular lumen surrounded by single layer of cuboidal cells. The anterior cells remain single layered and cuboidal for rest of the life. . The cells on the posterior part and equator are converted into elongated cells. The elongation of these cells and their multiplication obliterate the lumen of the vesicle. These elongated cells are called the primary lens fibers. From the central core of these fibers will develop the embryonal nucleus. The cavity of the lens vesicles is gradually obliterated, the anterior part of these cells ultimately touch the inner surface of the anterior cuboidal cells and formation of embryonic nucleus is complete. The outer most cells of the lens vesicle form lens capsule, which is in fact a true basement membrane produced by these cells. The so-called lens capsule is thickest at the equator and thinnest posteriorly. After the embryonic nucleus is formed, lens fibers continue to be laid down over it. This procedure continues through out the life and the fibers are called secondary lens fibers. In adult lens there are no nuclei in these cells. This is one of the factors contributing to transparency of lens. The fibers deposited at various times produces zones belonging to different ages. The oldest fibers are more centrally placed than the younger. They show optical difference. Fibers developing later are more transparent than those developing earlier. These various zones are called various nuclei according to chronology of their appearance. They are embryonal, foetal, infantile, (formed during last weeks of foetal life up to puberty) adult and cortex. The cortex is a homogenous material softer than nuclei. Zonules of the lens The Zonules of the lens develop separately along with vitreous, it is both ectodermal and mesodermal in origin. It is also known as tertiary vitreous. It starts developing in the fourth month of intra uterine life between the lens and the ciliary body. If there is a coloboma of ciliary body the zonules at that area become deficient. Development of zonule starts at 65 mm stage and completed at 110 mm stage. The zonuler fibers are derived from both primary vitreous and non pigmented epithelium of ciliary body.

OUTLINE OF DEVELOPMENT OF THE EYE

Vitreous Development of vitreous is complex; its exact origin is not well under stood. It is said to be derived from both mesoderm and ectoderm . The ectoderm mostly develops form inner layer of optic cup in the form of delicate fibrils. The mesodermal tissue forms hyloid vessels. The development of vitreous is divided into three parts : 1. The Primary Vitreous is presumed to be derived from both mesoderm and ectoderm. The hyloid vascular system is mesodermal, while fibrils are ectodermal, secreted by inner layer of optic cup. The surface ectoderm does not contribute to formation of primary vitreous. Primary vitreous starts developing in first month of intrauterine life. The primary vitreous is not atrophied. It lies behind the posterior lens capsule as a conical structure. It is surrounded by secondary vitreous. 2. The Secondary Vitreous starts forming from second month onward replacing primary vitreous that is completely replaced by sixth month, except the Coloquet canal which also disappears at birth. 3. The tertiary vitreous is the zonule of the lens. The Cornea The spot where the optic vesicle touches the surface ectoderm is converted into lens plate that gives rise to lens vesicle which is pinched off from the surface ectoderm. The remaining part of it gives rise to corneal epithelium. Rest of the cornea is mesodermal. The mesoderm encroaches in between the surface ectoderm and the lens. This mesoderm is divided into two distinct parts : 1. Anterior mesoderm that gives rise to stroma and endothelium of the cornea. 2. Posterior mesoderm that gives rise to iris stroma. In the space between the two layers of mesoderm develops the anterior chamber. The Descemet membrane develops from endothelium while Bownans membrane develops due to condensation of stroma under the corneal epithelium. The Development of Sclera1,2,4 Sclera is fully mesodermal in origin. It develops due to condensation of paraxial mesoderm around the optic cup. Its development is divided in two phases: 1. The development of anterior sclera 2. The development of posterior sclera. The sclera is fully differentiated by fifth month. Initially, the limbus is farther back near the future equator where the extra ocular muscles get attached later. By 12th week the posterior condensation encircles the optic nerve and the lamina develops. The scleral spur develops by 16th week and the Tenons capsule by 12th week along with insertion of recti muscles. The sclera plays little part in development of globe that depends upon development of retina. In contrast to this sclera plays an important part in growth of orbit. At birth the sclera is thin and gets a bluish tinge due to under lying uvea.

4 The Development of Extra Ocular Muscles13

PEDIATRIC OPHTHALMOLOGY

All voluntary muscles develop from paramedian mesoderm. The extra ocular muscles are no exception. The extra ocular muscles develop from a common mesodermal mass that is separated as three different groups. From each will develop muscles that are supplied by different cranial nerves i.e. third, fourth and sixth. The nerves grow from brain towards individual muscle mass marked for each nerve. The individual extra ocular muscle starts differentiating at about 9mm stage and can be identified as separate muscle by 20mm stage except the levator. The levator develops from the dorsomedial aspect of the superior rectus. The nerve supply to levator passes through the superior rectus mass hence simultaneous congenital under action of both are a common feature. The Uvea The uvea develops from neuroectoderm and mesoderm. The Iris and ciliary body develop partly from neuroectoderm (that is part of optic cup), and partly from mesoderm. The structures that originate from neuroectoderm are pigment epithelium of iris sphinter and dilator pupilae of iris, epithelium of ciliary body. Melanocytes also arise from ectoderm. Bruchs membrane is partly neuro ectodermal and partly mesodermal. The mesoderm gives rise to strom of iris, ciliary muscle, connective tissue and blood vessels. 1. The Iris. The part of the iris that develops from mesoderm is its stroma. It develops between the lens and surface ectoderm. The anterior tip of the optic cup develops over the scaffolding of mesodermal stroma. The tip of the cup develops into two layered pigment epithelium of the iris. The anterior layer is the continuation of outer layer of optic cup while the posterior layer is continuation of inner layer of optic cup. The pupillary membrane is a transient structure that lateron atrophies and disappears. Initially this stretches across the lip of the optic cup. It is formed by the mesodermal tissue surrounding the margin of the optic cup and tunica vasculosa lentis. Later the pupillary membrane separates from tunica vesculosa lentis. The peripheral part of the pupillary membrane gets vascularised. The central part of the pupillary membrane is eventually completely absorbed forming the pupil. Incomplete absorption of the pupillary membrane is called persistent pupillary membrane. The iris is fully pigmented after birth. If there is less of pigment the iris takes a blue colour. Some times fine vessels are visible on the iris of a new born. 2. The Ciliary body. The ciliary muscle develops from paraaxial mesoderm. The ciliary muscles become evident by third month and is gradually differentiated into longitudinal, oblique and circular muscles. The ciliary epithelium is neuro ectodermal in origin. It develops from the lips of the optic cup. The outer layer is pigmented while the inner layer is non pigmented the ciliary epithelium give rise to 70-75 ciliary processes. 3. The Choroid. Structure wise choroid differs from iris and ciliary body. It does not have an epithelium like the former two. It lacks stroma as in iris or musculature of ciliary body. It is mostly mesodermal except the Bruchs membrane that has both ectodermal and mesodermal origin. The choroid is mostly vascular with connective tissue inbetween. Its

OUTLINE OF DEVELOPMENT OF THE EYE

vasculature develops in three stages. Earliest is development of chriocapillaries. In second phase larger tributaries of venae verticosae develops by the third month of gestation.. The third phase consists of development of vessels from short ciliary vessels. The Anterior Chamber The anterior chamber develops as a cleavage in the paraxial mesoderm that lies between the corneal endothelium and iris stroma. Its presence is noticed at 20mm stage. Anterior chamber starts as a chink in the centre of this mesoderm and spreads to the periphery. Initially it is very shallow but at birth it is fully formed. The angle of anterior chamber is not formed before 6 month i.e. two months after the canal of Schlemm is visible. Schlemms canal starts as venous channel derived from various plexus at the margin of the optic cup. The Retina The retina develops from both the layers of optic cup. The inner layer gives rise to nine layers of sensory retina while the outer layer that remains single layered gives rise to pigment epithelium. The space between the two layers is very large in early stage of development that gradually shrinks to a potential space at the time of complete development of retina. By the time the fetal fissure begins to close the inner layer starts to thicken to form various layers of sensory retina. The pigment epithelium starts acquiring pigment granules at this stage. By seventh month of gestation all the layers of retina are well developed except in macula. The Macula Development of the macula differs from rest of the retina. Initially there is fast development in area of macula upto third month of life, then there is a slowing of growth while rest of retina grows in usual pace. This state of retardation persists upto eighth month then it start growing in the same manner as rest of the retina. Thus its development is not complete by ninth month. To attain full development macula has to wait up to fourth month post natal. At sixth month of foetal life it is thicker than rest of the retina. By seventh - eighth months it starts thinning. The thinning is due to spreading out of ganglion cells from the central part i.e. the fovea which at birth has only one layer of ganglion cells left. The outer nuclear layer is also single layered. The Optic Nerve The optic stalk that joins the fore brain and the interior of the optic vesicle is the future optic nerve. The foetal fissure that develops at the under side of the optic vesicle extends in to the stalk also. The axons of the retina and blood vessels pass through this opening. By third month the mesoderm that forms the connective tissue of optic nerve along with minute capillaries enter the optic nerve. The outer covering of the nerve i.e. dura, arachnoid and pia develop between third and seventh months, the lamina cribrosa develops late. Myelination of optic nerve starts at about seventh month of foetal life towards cephalicend, extending towards the lamina and stops short at lamina. In few cases the myelination may extend on the surface of the retina as medulated nerve fibers.

6 Intra Ocular Vasculature

PEDIATRIC OPHTHALMOLOGY

Intra ocular vasculature can be divided into three components : 1. The hyaloid system 2. The uveal system 3. The retinal circulation All of these arise from paraxial mesoderm that get into the eye through the fetal fissure. Besides supplying nutrient to the eye they play an important part in development of the eye itself. 1. The hyaloid system comprises of : (a) Vessels of pupillary membrane (b) Tunica vasculosa lentis (c) Hyloid artery (a) The vessels of pupillary membranes are formed by small buds from annualar vessels. The central part of the pupillary membrane is almost devoid of any vessel. It forms the pupil as it atrophies. The peripheral thick part persists as part of iris stroma. (b) The tunica vasculosa lentis. It has three parts i.e. the posterior, lateral and anterior. It completely engulfs the developing lens and supplies blood to it. It also forms a connecting channel between intra and extra ocular circulation. (c) The hyaloid artery is a branch of dorsal ophthalmic artery. It supplies blood to the developing lens. This passes through the foetal fissure and courses through middle a developing vitreous, stretching from posterior part of the optic vesicle to posterior pole of the lens. Before reaching the lens it divides into smaller branches and called Vasa hyaloidea propria that anastomose with each other in the primary vitreous and form the posterior part of tunica vasculosa lentis. Atrophy of the hyaloid system. Once the hyaloid system has reached its peak of development and finishes the main task of blood supply to the developing lens it starts to atrophy and disappears completely. Exact stimulus for disappearance of hyaloid system is not well under stood. The posterior part of the tunica vasculosa lentis is first to start to atrophy, followed by lateral. The anterior tunica is last to atrophy without leaving any trace. In hyaloid artery the anterior branches start disappearing first. Sometimes leaving a small part attached to the posterior pole of the lens as Mittendrof s spot. A small part may remain unabsorbed in the Cloquets canal or may remain attached to the optic nerve head as Bergmeisters papillae. 2. The Uveal Circulation. The uveal circulation becomes evident at very early stage as vessels round the posterior part of the optic vesicle. The primitive internal carotid gives off two branches that are precursors of long posterior ciliary arteries i.e. dorsal and ventral ophthalmic artery, the former develops to become temporal long posterior ciliary artery while the latter is destined to become medial long posterior ciliary artery. The dorsal ophthalmic artery also gives rise to short posterior ciliary arteries. The anterior uvea is supplied by muscular branches of ophthalmic artery which form the anterior ciliary system.

OUTLINE OF DEVELOPMENT OF THE EYE

3. The retinal circulation. Exact mode of development of retinal circulation is controversial. Most widely accepted hypothesis is that central retinal artery buds from the posterior end of hyloid artery at the level of optic cup but does not atrophy like rest of the hyloid system and then branches off as temporal and nasal branches that divide into superior and inferior branches. Development of Lid, Conjunctiva and Lacrimal System 1. The Lid. The lid has dual origin, the main mass of the lid is formed by mesoderm while the skin and conjunctiva develop from surface ectoderm. At about 18mm stage, the mesoderm condenses outside the optic vesicle in the form of lid fold. The upper lid develops from frontonasal process in two parts i.e. smaller medial and larger lateral part, while the lower lid develops from maxillary process. Fault in fusion of medial and lateral frontonasal mesoderm results in coloboma of upper lid that may very from a simple notch at the lid margin to extensive loss of tissue. The upper and lower lids are fused between third and sixth month then they separate. Separation is completed well before birth. Failure to separate will result in ankyloblepharon of various degrees. 2. The Conjunctiva. The conjunctiva, cilia, meibomian gland, lacrimal gland and accessory lacrimal glands also develops from surface ectoderm and are associated with development of the lid. 3. Development of lacrimal system7. (a) The lacrimal gland. Lacrimal gland develops from the superior temporal conjunctival fornix, as solid cords of ectodermal cells 8-10 in number which are surrounded by mesoderm that develop into connective tissue of the gland. The ectodermal cords gradually canalise and ramify. (b) The Lacrimal Passage. The lacrimal passage develops in the groove between the maxillary proces and lateral nasal process, the groove or cleft is converted in to a tube. The surface ectoderm gets buried in the mesoderm and progress upwards, at the same time a similar cord of ectoderm develops from the nasal cavity. The upper end will form the two canaliculi, puncta and sac. Subsequently the two cords i.e. the upper and nasal will join each other to form a continuous structure. During third month the central cells of the cord begin to disintegrate and form the nasolacrimal duct. The disintegration is patchy in nature but ultimately becomes continuous. The upper part thickens and dilates to form the lacrimal sac. Development of the Orbit The walls of the orbit develop from the mesoderm around the eye. The floor and lateral walls develop from maxillary mesoderm. The medial wall develops from the lateral nasal process. The roof differs little from these walls, it develops from the mesoderm covering the forebrain. By 14th week the boundaries of orbit are well differentiated. Up to 28th week the orbital margin is at the level of developing equator of the globe, there after it grows rapidly and the rim occupies more anterior position than the globe. The size of orbit is so small at this stage that its walls are snug with the globe. At birth the orbital rim is almost circular, its diameter is relatively large as compared to face.

PEDIATRIC OPHTHALMOLOGY

CONGENITAL ANOMALIES OF THE GLOBE8,9,10


The Congenital anomalies of globe are caused due to faulty embryogeneses before the closure of the embryonic fissure. They can happen : A. During formation and development of primary optic vesicle i.e. Cyclopia, anophthalmos, and extreme degree of microphthalmos. B. During development of optic cup i.e. Congenital cystic eye ball, colobomatous cyst and typical coloboma of eye. (Uvea, retina disc.) C. Maldevelopment of formed eye is Microphthalmos (True nanophthalmos) 1. Cyclopia. This is an extremely rare congenial anomaly that has either a single mid line eye or two developing eyes with deformity of the forebrain and multiple anomalies of mid line. There is either one orbit or two maldeveloped fused orbits. There is one palpebral fissure with two rudimentary lids, and lacrimal apparatus. The IPA is wide, the lids do not close, the rudimentary cornea and conjunctiva are exposed. These children have neonatal death. 2. Anophthalmos. Strictly speaking term anophthalmos is reserved for a condition where no ocular tissue is present in the orbit due to non formation of optic vesicle. However in clinical practice eye with minimal ocular tissues are also called clinical anophthalmos. Generally these eyes do not have any evidence of formed globe. They are also known as extreme microphthalmos. Anophthalmos differs from enophthalmos. In enophthalmos a fully developed globe is pushed back in the orbit due to secondary causes. 3. Microphthalmos. These include all eyes that have size less than normal eye due to congenital cause. They can be unilateral or bilateral. In bilateral cases both eyes are smaller than normal but not of equal size. The defects in one eye need not be the same in other eye. In unilateral cases the other eye may be normal and remain so for rest of the life. Microphthalmos has been divided in following groups more on clinical features rather than embryological. (a) Pure microphthalmos (Nanophthalmos) (b) Colobomatous microphthalmos. (c) Complicated microphthalmos. (d) Microphthalmos with cyst. (e) Microphthalmos associated with systemic syndromes. (a) Nanophthalmos11,12 (pure microphthalmos). Nanophthalmos is a rare congenital condition where a fully developed eye fails to grow like any other eye. This is caused due to failed growth after the embryonic fissure has closed from end to end. There are no colobomas present in the globe, it occupies normal position in the orbit, has normal movement but may be strabismic due to associated high axial hypermetropia. The cornea is smaller, AC is shallow. The sclera is thickened and the vertex veins are narrow11. There is pseudoneuritis, hypoplasia of macula, nystagmus, amblyopia. There are various types of glaucoma i.e. late onset of simple glaucoma, narrow angle glaucoma, precipitation of glaucoma following mydriasis13. These children require high hyper meteoric correction may require near correction. They have been described as phakia children with aphakic correction. The nanophthalmic eyes with glaucoma do not respond well either to medical or surgical treatment, the later is generally

OUTLINE OF DEVELOPMENT OF THE EYE

associated with Chroroideal effusion syndrome13 that may follow any intra ocular surgery or injury to the globe. (b) Colobomatous microphthalmos. colobomatous microphthalmos is a congenitally small eye that have associated failure of embryonic fissure. These eyes have multiple colobomatous defects at the site of fusion of embryonal fissure, mostly in uvea and or retina. These eyes are small in all dimensions, the coloboma may range from a small notch in iris to coloboma extending up to optic nerve. (c) Complicated microphthalmos. Is a term used to denote a congenitally small eye that develops cataract, iridocorneal defects, defects in iris, retina and vitreous. These are due to (i) Failure of development of primary optic vesicle. (ii) Arrest of development after the primary optic vesicle has formed. (d) Microphthalmos with cyst. In case only retinal tissue protrudes through the embryonic cleft a cystic eye ball with coloboma results. The colobomatous cyst may have an almost normal eye with a small indistinguishable cyst or the cyst may be so large that the small eye is not visible. In between are the cases where a formed eye is associated with colobomatous cyst. (e) Microphthalmos associated with various syndromes. There is a long list of conditions that are associated with microphthalmos most of which are cranio facial or mandibulo facial anomalies.

BUPHTHALMOS
This condition is just reverse of microphthalmos it is a large eye associated with various types of congenital glaucoma.

DEVELOPMENT OF OCULAR STRUCTURE FORM EMBRYONIC GERMLAYER


1. The eye along with its adnex develop from ectoderm and mesoderm. The Bruchs membrane and the tertiary vitreous (zonule) have duel origin. Bruchs membrane develops from neural ectoderm and mesoderm. While zonules develop from surface ectoderm and mesoderm. No part of the eye or its adnexa develop from endoderm. 2. The surface ectoderm gives rise to : A. The Lens B. Corneal Epithelium C. Epithelium of all the ocular adnexa i.e. conjunctiva, meibomian gland, glands of Zies and Moll, lacrimal gland, lacrimal passage. 3. The neural ectoderm gives rise to Sensory retina, retinal pigment epithelium, epithelium of ciliary body, pigment epithelium of iris, sphinter and dilator muscle of iris, melanoeytes, neural part of optic nerve.

10 4. The Mesoderm gives rise to : A. Corneal stroma and endothelium of cornea. B. Iris stroma, ciliary muscles and chroid C. Sclera, vitreous and extra ocular muscles. D. Bony orbit E. Blood vessels.

PEDIATRIC OPHTHALMOLOGY

REFERENCES
1. Duke Elder.S. ; System of Ophthalmology, Vol-III, Part-I, First edition, Henry Kimpton, London, 1964. 2. Mann Ida ; Development of Human Eye, Third Edition, British Medical Association, London, 1964. 3. Barber A.N. ; Embryology of Human Eye, The C.V. Mosby St. Louis 1955. 4. Kozart D.M. ; Embryology of the human Eye in Text Book of Ophthalmology, Ninth Edition p79-92, Edited by Schcie H.G., and Albert D.M., W.B. Saunders Company, London, 1977. 5. Vaughan D and Asbury T. ; General ophthalmology, Ninth Edition p9-13, Lange medical publication, California 1980. 6. Hamming Nancy and Apple D. ; Anatomy and embryology of the eye in Principles and Practice of Ophthalmology, Vol-I, p3-20, First Indian edition. Edited by Peyman G.A., Sander D.R. and Goldberg M.F. Jay. Pee Brothers, New Delhi, 1987. 7. Buffam F.V. ; Lacrimal diseases in Text book of ophthalmology, Vol-4, Edited by Podos S.M. p7.1 to 7.3, Gower Medical Publication, London 1993. 8. Nema H.V. Singh V.D and Nema N. ; Congenital anomalies of the eye and its adnexa in Anatomy of the Eye and its Adnexa. Second edition. p162-165, Jay Pee Brothers, New Delhi 1991. 9. Duke Elder.S. ; System of Ophthalmology, Vol-III, Part-2, First Edition p415-495, Henry Kimpton London,1964. 10. Schaffer D.B. ; Abnormalities of the eye as a whole in Text Book of Ophthalmology Ninth Edition., p209-293, Edited by Scheie H.G. and Albert D.M., W.B. Saundes Company, Philaddphia 1977. 11. Dutta L.C. ; Uveal effusion syndrome in Ophthalmology, First Edition p-122-123 Current Books International, Kolkota 1995. 12. Shields M.B. ; Nanophthalmos in Text Book of Glaucoma, Fourth Edition, p-280, William and Wilkins Philadelphia 1999.

CHAPTER

Examination of the Eyes in Pediatric Patients1,2,3,4,5


GENERAL PRINCIPLE
The examination of the eyes in children basically does not differ much from that of adult eyes. The principles of the tests, clinical approach and interpretation of each test is almost the same in all ages with little modification. It is essential to keep in mind that a childs eye is not a condensed form of an adult eye. For purpose of examination, the patients have been divided into following age groups: A. Neonates and infants B. Between one year to five years C. Five to ten years D. Above ten years. Otherwise ocular disorders can also be categorised chronologically. It should be remembered that some of the disease may be obvious in lower age group and spill over to higher age groups. Whereas some of the occult lesions may be present in infancy but become obvious only at later age. The diseases have been classified variously according to: A. Age of onset B. Chronology of disorder C. Tissue involved and D. Clinical Presentation. According to age of onset, the disorder can belong to the following groups and each may have distinct clinical presentations and require specific examination: A. Intra uterine B. Neonatal C. Between one to five year D. Above five year. 11

12 A. Intra uterine

PEDIATRIC OPHTHALMOLOGY

The intra uterine ocular disorders produce mild to sever deformity and visual loss, are generally bilateral, many of them are genetic in nature. Some of them are life threatening. Intra uterine factors that produce ocular malformation can be: 1. Genetic 2. Transplacental. 3. Mechanical. 4. Traumatic 5. Neoplastic (rare). 6. Nutritionational. The genetic factors can be: (a) Inherited genetic defect. (b) Genetic mutation. (c) Chromosomal aberration. (d) Effect of exogenous factors like, drugs, radiation, diet. The transplacental factors causing ocular defects are mostly infection, followed by various drugs taken in first trimester. In developing countries dietary deficiencies have a considerable influence on ocular organogenesis. The common infections that cause ocular morbidly are Rubella, toxoplasmosis, syphilis, cytomegalo virus diseases. Uterine bands, deformed uterus, fibroids and umbilical cord are common mechanical causes of ocular deformity. Intra uterine traumas in the form of amniocentesis, attempted abortion in early pregnancy also cause ocular disorders.

NEONATAL CAUSES OF OCULAR DISORDERS CAN BE


A. Infection. B. Mal developmental. C. Effect of pre maturity . The infection can be acquired during delivery or soon after. The after effects of such infection may cause ocular morbidity which may be noticed later. The same is true of congenital infections. These infections are gonorrhoea, herpes simplex, chlamydia, inclusion conjunctivitis and other bacterial infections. The congenital infections that cause ocular morbidity but do not manifest always at birth are rubella, toxoplasmosis and syphilis. Maldevelopmental causes of ocular disorders are varied, they can involve a single structure or may involve more than one structure in various combinations themselves or their aftermath common among them are. Anophthalmos, microphthalmos, microcornea, limbal dermoids, bluesclera, dysgenesis of anterior chamber, coloboma of uvea, polycoria, aniridia, total or partial

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cataract, ectopia lentis, congenital myopia, primary , associated or secondary congenital glaucoma, persistent primary hyperplastic vitreous. The retina may show congenital folds, detachment coloboma. The macula may be hypoplastic or may have coloboma. Ptosis, various types of squint, non cataractous white reflex in pupillary area. Various craniofacial and mandibulo facial syndromes. Prematurety causes retrolental fibroplasia, and myopia of prematurety.

CAUSES MET BETWEEN ONE TO FIVE YEARS OF AGE ARE


A. Sequel of 1. Intrauterine infection 2. Developmental anomalies. 3. Neonatal infection. 4. Trauma 5. Dietary Deficiency B. Inborn errors of metabolism C. Errors of refraction D. Strabismus E. Glaucoma F. Intraocular tumours G. Orbital tumours H. Allergy J. Infection Endogenous Exogenous Local Systemic

I. Autoimmune disease

K. Degeneration and dystrophies.

CAUSES OF OCULAR DISORDERS SEEN IN CHILDREN BETWEEN FIVE TO TEN YEARS CONSIST OF
A. Residual or continued effect of above B. Infections become common, so are allergies C. Trauma in this age group is frequent D. More children turn up with errors of refraction, squint and amblyopia. Boys in this age group should be tested for colour defect to plan their future occupational training.

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EXAMINATION OF EYES IN NEW BORN


Before embarking on examination of eyes of neonate it is essential to remember features of new born eye. The new born eyes as sensory system are better developed than expected when weighed against its size. The eyes are smaller in size as compared to that of adults. The eye ball of a new born is 16.5 mm to 17.5mm in diameter as compared to 23 to 24mm of an adult eye. The volume of eye ball is 2.8 mm as compared 7.0 mm of adult, the eye reaches its adults size by 14 years of age. By the age of 3 years the eye is about 23 mm in diameters, contrary to general believe there is no spurt in axial length of an emmetropic eye at puberty. The normal eye at birth has axial hypermetropia of about 3 to 4 D this is neutralised by physiological increase in axial length however if the axial length continues to increase two things are possible 1. Parts of this in neutralised by reduction of curvature mostly of lens and partly of cornea4. 2. The eye becomes myopic. A. The Vision. The vision of a new born is less as compared to a child of three years but is not very poor, it has been estimated to be about 1/607 the new born child has good accommodation, however there is no clinical method to measure accommodation and convergence. The fixation reflex is present but poor. B. The movements of the eyes are bizarre; they can fix a strong stimulus for a short while and then give up. Movement of two eyes are independent and non conjugate. C. The inter palpebral fissures are 18 mm in length, the width is less as compared to cornea hence the cornea looks larger and may be mistaken as abnormal. The new born sleeps for almost 18 hours a day so most of the time the eyes are closed. Any attempt to widen the IPA results is contraction of orbicularis and rolling up of eye ball due to Bells phenomenon which is well developed. However the blink mechanism is absent and develops by 7th or 8th week. Hence threatening gesture to find out presence or absence or vision is futile. it is difficult to evert the lids. D. The orbits are smaller, shallower and round. The space between the globe and orbital wall is so less that the orbit seems to sit snugly round the globe. None the orbital contents are visible or palpable. E. The Cornea is relatively large when compared to adult cornea. The diameter of new born cornea is 10.5 mm while that of adult cornea is little less than 12mm. However this growth is far less when compared to axial length of the globe which grows from 18mm at birth to 24mm at maturity. As the IPA is narrow in new born there is a false impression of cornea being large. The cornea is flatter when examined on kertometer. Its curvature is uniform, giving spherical look to the cornea. The Cornea has mild haze that clears within few days. F. The Sclera is thin the under lying uvea shines through it so it has a bluish tinge that passes off within few month and sclera becomes white. G. The Conjunctiva is well developed, the goblet cells are functioning, the conjunctiva is sterile at birth that gets contaminated within few days. The newly acquired organisms are generally non pathological.

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H. The Lacrimal Glands are not fully developed at birth. It takes three to four years for them to adequately develop. The new born cries but does not weep; this is due to lack of reflex tearing that develops after four to six weeks. Psychic tearing takes about six to seven months to develop. I. The Lacrimal Sac is fully developed at birth and its lumen is patent . J. The Lacrimal Passages are canalised at birth. Even if the nasolacrimal duct is not patent at birth its block is not evident till end of first month as there is no reflex tearing before that. K. The anterior chamber is fully formed. Its depth is normal or slightly less than adult depth. The contents are clear, the angle is wide. The pupil can be safely dilated without rise of IOP. L. The pupil is smaller than adult, is shifted nasally and down. It is circular and reacts to bright light both directly and indirectly. M. Accommodation and Convergence can not be tested clinically due to lack of fixation. Accommodation is well developed by 4 months.17 N. The iris is light coloured due to scanty pigments. There may be a few blood vessels on the iris surface.. The dilator muscles are poorly developed. The constrictor muscles are relatively stronger hence it is difficult to dilate the pupil. O. The Lens is fully developed and functional. The lens grows in size throughout the life. The lens is more spherical than adult lens, its average diameter is 6mm in comparison to 9mm of an adult lens. P. The fundus The fundus is visible, the colour is paler than normal, the optic disc is pale, the macula is not fully developed, it continues to grow postnatal. The foveal reflex is absent. The retinal periphery is also pale. Q. The broader nose bridge of a new born gives an appearance of pseudo convergent squint. The movements are not co-ordinated and can not be tested precisely.

EXAMINATION OF THE EYES OF NEONATES AND INFANTS


Examination of the eyes of a neonate is difficult because a neonate sleeps almost eighteen hours of a day the waking period is in short spells and most irregular, a neonate wakes only when hungry or is ill at ease. It is difficult to elicit correct visual response in an infant. The examiner should be able to differentiate normal parameters of signs from the abnormals. Many a times a casual remark by the examiner may send a wave of panic in the parents least the baby may be blind. An infants eyes are required to be examined under following circumstances A. As a part of routine examination of all new borns. The protocols followed in various hospitals differ greatly. The examination is done either by attending neonatologist or resident of pediatric ophthalmology posted in nursery and well baby clinic. Nurses and midwives attending neonates can also be taught to examine a neonates eyes and refer for further examination for abnormal findings, or seemingly abnormal cases.

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B. The neonatiologist has noticed some gross abnormality i.e. craniofacialdysostosis, faciomandibular abnormality, cryptophthalmos, anophthalmos, microphthalmos, large coloboma of the lid, proptosis, unilateral gross ptosis, corneal haze, pupillary abnormalities white reflex in the pupil and refers for detail examination and opinion. C. Signs of infection in the eyes : Edema of lids, purulent or mucopurulent discharge. The above abnormalities are gross enough to be noticed by parents or even a village midwife. D. There is 1. Family history of (a) Lamellar cataract (b) Albinism (c) Nystagmus 2. Maternal history of (a) Suspected rubella in first trimester (b) S.T.D. (c) Some drugs given in the first trimester that cause congenital anomalies. (d) Diabetes under treatment. (e) Myasthenia in mother (f) Consanguinity (g) Prematurity (h) These babies may or may not have been on prolonged oxygen. (i) Difficult labour fetal distress, neonatal apnoea, intensive resuscitation for above causes, birth trauma, specially injury by forceps. (j) A critically ill child who may have congenital toxoplasmosis, herpes simplex galactosemia, hypoglycaemia.

VISION TESTING IN A NEW BORN


One of most difficult tasks in ophthalmology is to give opinion regarding exact quantitative vision present in each eye of a new born. A rough estimation of qualitative vision present is not difficult to express. Vision of a new born is very poor in comparison to adult vision that is attainedby seven to eight year in other wise normal eye in a healthy child. The vision of neonate improves very fast6 from 1/60 to 2/60 at 1 month and 6/60 at four months. With 1/60 vision the child is able to fix a face moving within one meter. These visual standards are based on various complicated methods by expensive instruments on small samples i.e. Optokinetic target, Catford drum test, visual evoked occipital potential (VEP) and forced choice preferential looking (F.P.L)7,8 Definite fixation reflex and following reflexes take about six weeks to develop before that an infant may fix for few seconds and give up. These are associated with bizarre movements,

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these movements disappear with development of clear fixation. More than one types of movements are common in between the fixations. It is said that most suitable infant to examine for vision is an awake, alter and hungry baby. Visual acuity in a new born is determined by optically elicited movements. A. The first step is to observe if the child tries to look and fix a light when switched on in a semidarkned room. This must be repeated several times least some of the bizarre movements may be mistaken for fixation. Each eye should be tested separately. This indicates most primitive form of vision that can be elicited clinically. B. Next step is ability of the child to fix and follow the face of the examiner when brought within the field of vision of the child with both eyes open. This gives a definite clue of vision being present in at least in one eye but can not indicate which eye. For that one eye is closed, and other examined, both eyes are examined in the similar way separately one after the other. The incidence of blindness in neonates is very low if, visible causes are excluded. C. The condition that result in bilateral sub normal vision can be 1. Ocular 2. Systemic The Ocular causes consist of (a) Persistent corneal haze (b) Complete cataract (c) Congenital glaucoma (d) Retino blastoma (e) Aniridia (f) Hypoplasia of optic nerve (g) Sever retinal degeneration (h) Albinism (i) Achromatopsia. (j) Recessive opticatrophy (k) Lebers amaurosis. (l) Taysachs disease. (m) Glioma of chiasma The systemic causes are seen in (a) Premature children (b) Full term with low birth weight (c) Foetal distress (d) Asphyxiated child (e) Birth trauma to occipital cortex

18 (f) Cortical blindness (g) Sagital mild line defects.

PEDIATRIC OPHTHALMOLOGY

Half of the cases of diminished vision in children are genetic in origin.17

EXAMINATION OF PUPILLARY REACTION TO LIGHT


This is most reliable test to determine presence of vision except in cortical blindness. The test is best performed in a semi darkned room because the infants pupil are smaller than normal and constricts in presece of bright light in the room. In the semi dark room the pupil comes to a state of semidilatation that reacts briskly. The light used should be small well focused and bright. All forms of light reflexes i.e. direct, indirect and swing light responses are noted. If the pupil is small and it is difficult to appreciate pupillary reaction a hand held slit lamp may be used. Small sluggish irregular pupil are indication of inflammation, congenital miosis or persistent pupillary membrane.

OTHER EXTERNAL EXAMINATIONS CONSISTS


A. Face. Examination of face for craniofacial and mandibulo facial anomalies, albinism, hemangioma. B. Orbit. Anophthalmos, microphthalmos, cystic eye ball, proptosis. C. Lid. The lids are examined for Ptosis, coloboma, hemangioma. Lagophthalmos is far rarer than ptosis. Unilateral ptosis draws attention earlier than bilateral ptosis. Other anomalies present at birth that draw attention are : epicanthus and ankyloblepharon. D. Palpebral fissure of new born is smaller than adult. It is narrow in ptosis wide palpebral fissure is seen in lagophthalmos, proptosis and rarely in buphthalmos. There is apparent shorting in length in epicanthus. Epicanthus also gives a false impression of pseudo convergent squint that may not be appreciated at birth but becomes obvious after few months. Blepharophimosis that is frequently seen with ptosis and epicanthus and causes shortening of length of I.P.A. E. Lacrimal system. Only anomaly visible in a neonate is congenital mucocele of lacrimal sac. Neonatal nasolacrimal duct obstruction when present is asymptomatic and non elicitable. Ophthalmianeonatorum does not develop within first twenty four hours. F. Conjunctiva Only important finding that may be present in conjunctiva is subconjunctival haemorrhage which is of no consequence and is due to birth trauma the conjunctival sac at birth is sterile that soon gets infected by bacteria, virus and chlamydia. G. The Cornea Even a normal cornea in a new born looks large because of narrow I.P.A. The cornea of a new born is 10-10.5mm at birth any cornea larger than 12mm is abnormal may be buphthalmic. When in doubt about diameter of the cornea it is better measured. Most reliable measuremnent is taken by a corneal calliper, a transparent scale may be usedfor rough estimation. Most important cause of large cornea is buphthalmos that requires early referral, the other cause is megalocornea.

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Microcornea is frequent , more common than megalocornea, it may be associated with microphthalmous and coloboma of the uvea all three may be present singly in an eye or in various combinations. Bilateral small cornea is frequent. Other signs that should be looked for are: 1. Corneal haze. 2. Frank corneal opacity. 3. Limbal dermoid. 4. Rupture in Descemets membranes. 1. Corneal haze Most of the cornea in new borns are relatively hazy, which become bright in few days. Some of the pathological cause of corneal haze are : (a) Buphthalmos (b) Rupture in Descemets membrane (c) Dysgenesis of anterior chamber (d) Mucopoly saccharidosis. Rupture in Descemets membrane is mostly traumatic due to faulty instrumentation during delivery. It is difficult to examine the cornea and conjunctiva due to tightness of the lids, and small palpebral fissure. There is no condition in new born eyes where eversion of the upper lid is needed. Rarely a lid retractor may be required to see the upper limbus and upper bulbar conjunctiva. An infant size lid retractor should be used, if at all necessary. H. Anterior Chamber. The anterior chamber of new born is fully developed but shallow. The angle is wide and pupil can be dilated with out rise of I.O.P. A deep chamber is seen in buphthalmos and megalocornea. Congenital aphakia is a clinical curiosity. Sub luxation of lens is not appreciated unless carefully looked for. Commonest anomaly of anterior chamber is mesodermal dysgenesis of Reigers. Other condition being, Peters anomaly, which causes irregular shallowing of A.C. I. Uvea. Common congenital anomalies are persistent pupillary membrane, coloboma of the iris and ciliary body, anisocoria and cryptocoria. Aniridia is a rare anomaly but is a vision threatening condition that may have Wilms tumour in the abdomen. The iris of new born is lighter in colour. Presence of few blood vessels is common and non pathological. Posterior uvea is examined by direct and indirect ophthalmoscope with dilated pupil. J. Pupil. Pupil of a new born child is relatively moitic due to stronger constrictors than dilators of pupil. It is difficult to dilate the pupil of new born due to same cause. The pupil is examined for it shape, size position and number. Commonest cause of irregular pupil is persistent pupillary membrane. Other causes are mesodermal dysgenesis, Peters anomaly and coloboma of iris.

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K. Lens. Absence of lens is extremely rare in new born, sub luxation is difficult to elicit. Commonest anomaly of interest is presence of cataract. A cataract covering whole of pupillary area requires urgent referral. Pupil should be widely dilated to see if there is any clear zone between the outer border of opacity and dilated pupil. If there is clear zone then the pupil can be kept dilated for light rays to reach the macula till definite treatment is instituted. It is very essential to be able to differentiate between non lenticular causes of white reflex, especially retinoblastoma from cataract. L. Examination of fundus in born. Examination of fundus by both direct and indirect ophthalmoscope is not difficult. It requires little practice with dilated pupil and knowledge to differentiate between normal and abnormal findings in optic nerve, macula and retinal periphery. Drug used to dilate pupil of new born There are many drugs employed to dilate the pupil of a new born. Each ophthalmologist has his or her drug of choice that may be single drug or a combination of two drugs; one sympathomimetic and other parasympatholytic. Atropine is better avoided due to its possible side effects. Commonest combination of drugs is 2.5% phenylpherine with 1% cyclopentolate11. One drop is instilled in each eye twice at an interval of 3 minutes and fundus examined after 45 minute to one hour. Two common phenomena observed during fundus examination are 1. Up rolling of the eye due to Bells protective reflex. 2. Non pathological bilateral lid retraction and down ward movement of globe when the illumination in the room is reduced. This should not be confused with pathological sunset appearance of the globe. The non pathological lid retraction may persist for four to six months. Some ophthalmologists have enough patience to examine the fundus in between involuntary the closure of lids. This may be circumvented by use of neonate size of eye speculum. The neonates tolerate presence of speculum well. The fundus is examined for transparency of media, disc, blood vessels, macula and periphery. Remnants of hyloid system are frequent. The arteries are thin, the fovea is not bright the disc is pale enough to be confused as atrophic. A small pale disc surrounded by yellow halo is suggestive of hypoplasia of disc. M. Squint. Squint is difficult to confirm due to bizarre movements unless there is gross lesion like Mobius syndrome which is other wise very rare. N. Nystagmus. Nystagmus does not develop before two months. Though it is very common to confuse non coordinated movements with nystagmus. O. Proptosis. Proptosis, unilateral or bilateral is not a good sign and requires prompt investigation. P. Enophthalmos in neomates and infants is rare.

EXAMINATION OF EYES IN FIRST YEAR OF A CHILD


Many of the difficulties in examination of a new born childs eye gradually pass away giving way to easy examination and better interpretation.

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A. Recording of vision The vision generally ranges between 6/60 to 6/36 i.e. the child can recognise faces by sight, develops preference for toys and picks up particles from the ground. However vision can not be tested with usual optotypes. The child follows a moving target first by moving the eyes then by turning and lilting the head and moves towards an object of interest. Presence of squint, nystagmus, persisting staring and inattention to object10 are ominous signs sometimes parents may be able to state if the childs vision is subnormal or not, by comparing it with older siblings or children of the same age. This is the age when the children are brought by anxious parents with a definite question Does this child has vision ?. This is a very difficult question to answer. Nothing is worse than informing the parents that the child is blind or normal, that turns out to be opposite later. There are some children who have delayed visual maturation (DVM)7 and behave like a child with sub normal vision. Vision should be considered normal in a child under one year if the media are clear, there is no error of refraction, pupil are central circular and react briskly to direct and indirect light, there is no squint or nystagmus, fundus is normal with normal milestones. Presence of pupillary reaction to light is not equivalent to visual ability, it merely gives an indication that both afferent and efferent paths of light reflex are intact10. Dilatation of pupil in presence of direct light stimulus is seen is Lebers congenital amaurosis, optic nerve hypolplasia congenital cone dystrophy and stationary night blindness7,11 In cortical blindness the pupillary reaction is present, this is due to the fact that the lesions is in optical cortex, commonest cause in children being trauma. However nystagmus is absent in cortical blindness.12 All children suspected to be blind should also under go : 1. Electrophysiological investigations-electro-encephalogram, electro-retinogram and visually provoked response. 2. CT and MRI of ocular structures and brain. Prematurity, low birth weight, prenatal asphyxia, convulsion congenital anomalies of brain may have occipital lesions which requires. CT and MRI to evaluate prognosis. In grown up children the routine is to record the vision in each eye separately followed by binocular vision. In children under three examination of vision with both eyes open is noted first followed by recording of vision in each eye separately, while examining each eye separately it is better to examine the supposed to be good eye first than the suspected faulty eye. This helps gaining confidence of the child. A child who has gross diminished vision in one eye always resents when the better eye is closed, the child may start crying and try to remove the occluder. While testing one eye the other eye should be fully occluded if necessary by a cotton pad and tape otherwise the child may peek over the occluder in the better eye. While recording vision the approximate near vision also be noted in preverbal child. It is good practice to let one of the attendants preferably one of the parents present during examination of the vision. The attendant should be asked not to prompt the child when the vision is being recorded. If the

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child has glasses the power of the glasses should be noted and vision in each eye is recorded as with glasses. It is better not to darken the room, this scares the child it should be semi lit. B. Accommodation, fusion and convergence17 Accomodation is present at one month but is relatively poor. It is well developed by four months. Fusion starts at two months, Fusional convergence develops by six month. It takes steriopis to reach adult level by five to six years of age. It is well developed by four months though it is considered to be present by second month. Once the vision has been recorded other clinical signs should be noted according to a strict protocol instead of jumping from one structure to the other. The other eye should also get same attention as the diseased eye even when the lesion is suspected to be unilateral. The parents may not like this due to preformed idea that the wrong eye is being examined. The parents at this stage should be explained the purpose of examining both eyes. The first step is to observe the child in normally lit room from a distance of about a meter without touching the child. C. The points to be noted in diffuse light are 1. Is the child comfortable, does he keep his lids forcefully shut this means photophobia and blepharosm for the same reason a child may hide his face from any source of bright light, the child may open the eye if the room is darkened sufficiently. A child with searching movement, most probably does not have vision in either eye. Diminished vision in one eye generally goes unnoticed by the parents unless associated with other causes like squint, corneal opacity or white reflex in pupillary area. 2. Look for symmetry of face, and skull : Asymmetry of face is generally seen in cranio facial anomalies. All children with skull larger or smaller than normal should get paediatric consultation. D. The eyebrows have scanty, fine hair normally and the eyebrows are symmetrically arched on both sides. Raised eyebrow with furrows in forehead denotes ptosis. Even a child of one year may have a raised eyebrow in case of ptosis. Unilateral ptosis attracts more attention than bilateral ptosis in children. The chin may be elevated . Flattening of eyebrows, absence of forehead crease and wide IPA denotes lagophthalmos. Persistent abnormal head posture like head turning, tilting or abnormal chin position in various combinations point towards possibility of paralytic squint (for details see examination of strabismus) E. The lids are examined for hemangioma, coloboma of lid margin and distichiasis. Epicanthic fold gives a false impression of pseudo convergent squint while telecanthus gives an impression of divergence. Narrow palpebral fissure is seen in various types of ptosis and pseudo ptosis. In blepharophimosis the horizontal length of palpebral fissure is reduced. It is generally seen with epicanthus and ptosis. F. In all cases of watering from the eye of long duration under one year of age the first condition that needs to be excluded is congenital dacryocystitis which should better be

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called as neonatal nasolacrimal duct obstruction because inflammation of sac is secondary to congenital obstruction. A positive regurgitation test is always confirmatory. In absence of positive regurgitation the condition of lower puncta for its presence and size should be verified. A congenital coloboma of lower lid which is generally seen at the outer one third may also cause epiphora. Most important condition that requires exclusion is buphthalmos, primary secondary or associated. Where the cornea is generally larger than 13mm the AC is deep, cornea may show Haabs lines. Children brought with complains of recent unilateral watering are rarely due to nasolacrimal duct obstruction, buphthalmos or conjunctivitis commonest cause is corneal abrasion that is confirmed by positive fluorescein stain. G. Examination of conjunctiva of lower tarsal conjunctiva, lower fornix and bulbar conjunctiva do not pose much problem so long this is not associated with edema of the lid and blepharospasm. In such cases an infant size lid retractor may be required sometimes two retractors may have to be applied. It is better if a drop of anaesthesia is used before applying the lid retractor, while using lid retractor care should be taken not to injure the cornea. The childs head and body below the shoulder should be immobilised by the assistant it is better to take a conjunctival smear at this stage. The cornea, AC and pupil are also examined along the conjunctiva at this stage. Conjunctiva is examined under focal illumination. 1. Chemosis of conjunctiva without mucopurulent discharge is rarely infectious most probably it is due to a hay fever like conjunctivitis. Chemosis with discharge is seen in acute bacterial conjunctivitis. 2. Presence of membrane over the conjunctiva should be considered to be as diptheritic and treated as such unless proved otherwise, independent of immunisation status of the child. Other cause of membrane over conjunctiva are : (a) Bacterial. Streptococcus, staphylococcus, pneumococcus, meningococcus, Koch Weeks bacillus and Ecoli. (b) Virus. Epidemic kerto conjunctivitis, herpes simplex, infectious mononucleosis. (c) Toxic. Stevens Johnson syndrome. (d) Chemicals (e) Drug allergy. One of the dictums to be remembered is Unilateral redness of eye is seldom conjunctivitis Chronic allergic conjunctivitis both endogenous i.e. phlycten or exogenous-Spring Catarrh is rare are under one year but can not be ruled out. 3. Follicles are very common, mere presence of follicle is of no consequence. However in endemic area trachoma should be ruled out. In children under one month other chlamydia infections are possibile. 4. Papillae are generally not seen under one year of age. 5. Subconjunctival haemorrhage can be (a) Wide spread either due to birth trauma specially due to application of forceps or due to sever cough in children. Whooping cough used to be a common cause of subconjunctival

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haemorrhage in children before universal immunisation came in to vouge. Even today it is seen in non immunised children in developing countries, leukaemia and purpura are common causes of subconjunctival haemorrhages. (b) Petechial haemorrhages are seen in epidemic haemorrhagic conjunctivitis and pneunococcal conjunctivitis. (c) In cases of sub conjunctival haemorrhage where its outer limit is not visible child should be examined for possibility of head injury. 6. Colour Changes in conjunctiva are common. A pale conjunctiva is indicative of anaemia, blue conjunctiva denotes cyanosis. Yellow discoloration is seen in jaundice, patches of black flat spots are due to congenital melanosis. Faints blue hue is universal in children due to thin sclera through which the underlying uvea shines. 7. Conjunctival redness is common in all types of conjunctivitis mostly infective. It can be localised in:- Subconjunctival haemorrhage, abrasion and laceration of conjunctiva, rarely phlycten, spring catarrh and hemangioma. Generalised congestion specially bilateral is seen in infective conjunctivitis. The congestion is most marked in the periphery in infective conjunctivitis. 8. Circumciliary congestion or circum corneal flush is seen in (a) Keratitis, corneal abrasion, corneal ulcer. (b) Uveitis : Anterior uveitis and panuveitis. (c) Glaucoma : Acute and chronic congestive. 9. Episcleral congestion is rarely seen in children when present they are mostly due to buphthalmos or raised episcleral pressure due to an orbital mass. 10. Conjunctival discharge : Purulent discharge is mostly due to gonococci infection or sever other bacterial infection. Mucopurulent discharge is seen in other bacterial infection. Watery discharge is seen in allergic, viral and chemical conjunctivitis. 11. Conjunctival Xeroxis is a common feature and ominous sign of vitamin A deficiency. Bitots spots generally do not develop during first year because if a child is breast fed, mothers milk contains sufficient vitamin. A for the child for first six month. The child usually gets a prophylactic dose of 50,000 of vitamin A at six months. H. Examination of Cornea Consists of examination of transparency, size, shape, curvature, encroachment over the cornea, sensation, vascularisation and deposits on both surfaces. 1. Transparency of cornea. Mild bilateral corneal haze is universal in all full term babies and more common in pre term. The haze passes off within a few days to few weeks. Presence of dense opacities requires attention and management. The cornea is uniformly hazy in congenital glaucoma both, primary or secondary that is generally a bilateral diseases, associated with enlargement of cornea, corneal edema and rupture in Descemets membrane that may result in Haabs lines. This requires differentiation from another common cause i.e. injury during forceps delivery, Haabs striations are horizontal may have whirl like appearance while injury by forceps are generally vertical. Out of all

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cases of corneal clouding congenital glaucoma due to what ever cause requires immediate attention to save the sight. Other causes of uniform corneal haze are relatively rare, they are congenital hereditary endothelial dystrophy, congenital hereditary stromal dystrophy, various mucopoly saccharidoses, mucolipidoses and cystinosis13. In congenital rubella syndrome the opacity is relatively dens but clears in few weeks. Interstitial keratities is seen after five years of age. Peripheral localised opacities are seen sclerocornea and limbal dermoid. In case of Peters anomaly the opacity is generally localised centrally with clear periphery frequently associated with other malformations of anterior segment. Kerato malacia : may be seen as bilateral haziness without circum corneal congestion is developing countries, in children born to mothers who suffer from malnutrition and have not received prophylactic dose of vitamin A in last trimester and the child has not been immunised against measles nor has been administered prophylactic vitamin A. However there are circumstances where the child develops signs of vitamin A Deficiency disease (VAD). These are ; 1. In sever drought the mothers vitamin A store is depleted during gestation and remains low thereafter. 2. The child is deprived of mothers milk due to death of mother, sever malnutrition in mother, or the child has been abandoned and children of orphanage. 3. The child suffers from measles, or chronic diarrhoea. 2. Size of cornea. In a new born child the cornea looks large because of smaller I.P.A. Bilateral large corneas are missed by parents unless associated with watering but unilateral enlarged cornea draws attention early even without tearing, the cornea is enlarged in buphthalmos and megalocornea. The former require urgent management. In case of positive history of buphthalmos in family and suspected rubella syndrome the corneal diameter should be measured by corneal calliper. Any cornea larger than 12 mm should be considered to be buphthalmic unless proved otherwise by measurement of I.O.P. examination of angle and evaluation of optic nerve head. Any cornea larger than 13 mm is definitely buphthalmic. Small cornea is seen in microphthalmos, and microcornea. 3. Shape of cornea. Distortion of shape of cornea is seen in microcornea microphthalmos and phthisis. 4. Corneal curvature. Increased corneal curvature suggestive of keratoconess that is extremely rare in first year. Flattening of cornea is more common and is seen in microcornea, microphthalmos and cornea plana. The former two are generally associated with gross incorrectable vision loss. Phthisis which is rare in first year of life may also present with flat cornea. 5. Enchromement over cornea. The corneal periphery may be as opaque as sclera in a rare instance of sclerocornea, where the opacity extends well into the cornea from the sclera obliterating the limbus. The peripheral opacity may be vascularised. In still rarer occasions the whole of the cornea may be as opaque as sclera. Dermoid14 is more common than the former. The commonest site being the limbus where a dry raised circular plaque of fibro fatty tissue is seen astride the limbus, they generally have few hairs growing over the growth. However they may develop in the middle of the cornea as well.

26

PEDIATRIC OPHTHALMOLOGY

6. Sensation of cornea. Corneal sensitivity can be elicited from birth. It is reduced over the opacities, keratanisation and herpes simplex keratities. There is a very rare condition where corneal sensation is absent from birth called congenital dysautonomia. 7. Vascularisation of cornea. Vascularisation of cornea is rare under one year of age. Vascularisation of cornea always denotes pathology either in conjunctiva or in cornea. The former produces supefecial vascularisation. The corneal vascularisation can be superficial or deep. Causes of superficial vascularisation are infective and allergic kerotoconjunctivitis. The only cause of deep vascularisation in children between 3-5 years is interstitial keratitis. 8. Deposits on the cornea are mostly inflammatory. I. Examination of Sclera. Sclera is not a tissue to have many findings in a child under one year. The sclera of an infant is relatively thin it is not as opaque as adult sclera but not translucent also. The uvea under neath gives it a bluish hue that passes off within few months but not in case of blue sclera brittle bone syndrome in osteogeneses imperfecta. The sclera develops yellowish tinge in jaundice, sub-conjunctival patches of black pigmentation are very common and may cause worry in parents. Sclera is devoid of any inflammatory process under one year of age. Conjenital ectasia may be seen rarely more common cause of ectasia is buphthalmos. J. Examination of anterior chamber : 1. An infants anterior chamber is fully formed at birth is of equal depth in both eyes it reaches adult depth by age of one year AC should be devoid of any thing but aqueous. Difference in depth of AC in two eyes is abnormal so in presence of mesodermal tissue. 2. Deep AC is seen in buphthalmos, keratoglobus and congenital axial myopia. Congenital aphakia is a clinical curiosity only a few cases of spontaneous absorption of lens have been reported in first year, ofcourse surgical aphakia gives a deep A.C. in infants who have undergone lensectomy without IOL. A very few infants may have iris clip IOL16 as part of management of congenital cataract and have relatively deep A.C. In P.C. I.0.L. the depth of A.C. is almost normal. 3. Shallow A.C. is seen in micro cornea, microphthalmos, cornea plana, mesodermal dysgenesis of anterior chamber, Peters anomaly. 4. Contents of anterior chamber Normal content of AC is aqueous humour that is crystal clear, without any visible suspended particle. It is very difficult to detect mild to moderate turbidity under one year of age. Presence of blood is more common in this age group which is mostly traumatic than pus. Presence of any white material in A.C. should be considered to be pus unless proved otherwise. Common cause of white material in A.C. are: hypopyon, pseudohypopyon, cortical material. K. Measuring intra ocular pressure. All infants with large cornea, history of buphthalmos in siblings and abnormal tissue in A.C. should undergo measurement of 10P under general anaesthesia. L. Examination of iris. Iris of a new born has lighter colour than what is expected to be at adult life due to lack of pigment, however the iris becomes pretty dark by one year if not

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27

as dark as adult. An iris that is abnormally light with pink pupil and trans illuminates in a fair child is due to albinism. Iris of one eye may differ from the other eye and is called heterochromia iridium in contrast to difference of colour in the same eye that is known as heterochromia iridis which can be unilateral or bilateral. Presence of congenital holes in iris other than pupil is called polycoria that generally lacks iris sphinters and called pseudopolycoria. True polycoria is rarer, these extra pupil have independent constrictor and dilator muscles and react to light independent of main pupil. Congenital coloboma of the iris is generally situated in lower pole of pupil, may be localised to iris only or may be part of more extensive colobomatous anomaly of uvea. The coloboma of iris may be present as isolated anomaly of the eye or may be seen in microcornea and microphthalmos. Perhaps most common anomalous finding in the iris is persistent pupillary membrane where strands of mesodermal tissue originate from the iris collarette and may pass across the pupil giving an irregular pupil. It disappears with age. Presence of few blood vessels in first two months are no consequence they also disappear with in few months. M. Examination of pupil Examination of pupil alone gives much more information regarding presence of many anomalies than any other examination. Pupil reacting briskly to direct and indirect light stimulus is confirmed sign of intact afferent and efferent precortical visual path. Pupil is examined for 1. Number, 2. position, 3. shape 4. size, 5. colour and 6. pupillary reaction. 1. Number. In rare instances there may be more than one pupil in the same eye either as pseudopolycoria which is more frequent than less common true polycoria both the conditions are associated with multiple anomalies of the eye. Cryptocoria is congenital absence of pupil . 2. Position. Single Pupil with slight nasal and medial shift is normal. However there may be corectopia where a pupil is shifted away from its normal place a part of multiple ocular anomalies. 3. Shape. Normal pupil is circular. All irregularity of pupil are not pathological only irregularity due to inflammation needs treatment. Persistent pupillary member is the commonest cause of irregular pupil under one year of age. Other causes are coloboma of iris, mesodermal dysgenesis of A.C., pseudopolycoria. 4. Size. Pupil of a child under one year is smaller than that of a child of three year due to parasympathetic over action when compared to sympathetic. The constrictor muscles are better developed than sympathetic. Unilateral small pupil with narrow I.P.A. is suggestive of

28

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Congenital Horners Syndrome. Pupil of a child under one year should be examined in diffuse light. Dilatation of pupil requires more time due to strong constrictor. Slight difference in size is common. Difference in size is called anisocorca. Where it is important to find out which pupil is abnormal. Large pupils are seen in congenital myopia and buphthalmos, congenital internal ophthalmoplegia is very rare. However in all cases of large pupil history of instillation of mydriatic or cycloglegic should be excluded. 5. Colour of Pupil. The pupil looks dark even in the most pigmented iris when seen with a torch. On careful examination the normal lens produces two miniature images the third and fourth Purkinje images. Absence of third and fourth are seen in an opaque membrane over the anterior lens capsule and absence of lens (aphakia). A white reflex in pupillary area is always pathological. Causes of white reflex in pupillary area (see chapter on Len and Retinoblastom). 6. Examination of pupillary light reflex. A brisk direct and consensual light reflex denotes intact afferent and efferent neurological precortical path. A child with lesion of visual cortex presents as cortical blindness where the pupil are of same size and react well. Unilateral dilated sluggish pupil with contralateral normal sized, reacting briskly to direct and indirect light is due to iridoplegia that may be therapeutic or neurological. Bilateral fixed dilated pupil are due to 1. Bilateral irideplegia 2. Bilateral sever optic neuropathy. MarcusGunn pupil is seen in unilateral complete lesion of optic nerve. 7. Examination of lens. Lens of an infant is examined mainly for its transparency. Most important opacity is an opacity that covers whole of the pupil but may have clear periphery hence all lenses with central opacity should be examined with full mydriasis for possibility of clear periphery because management of opacity extending beyond fully dilated pupil is urgent lensectomy with management of aphakia and avoidance of ambloypia. A lens with clear periphery may be left with dilated pupil, this allows sufficient light rays to reach the macula for its development and prevention of amblyopia. Sub luxation and dislocation of lens is less common under one year and when present becomes obvious only on maximum pupillary dilatation. Scattered cortical opacities do not cause visual impairment and their presence should be noted in examination card and parents informed about their non progressive nature with instruction for yearly follow up. 8. Examination of vitreous. Vitreous is examined for presence of P.H.P.V., remnants of hyoid system, vitreous haemorrhage, familial exudative vitreo retinopathy, retinopathy of prematurity. Best method to examine vitreous and rest of the fundus is by a binocular indirect ophthalmoscope using a condensing lens that give magnification, steriopsis and moderate field with maximum dilatation. Recording of distant vision in a child. In principle and practice there is no difference in examination of vision in adults and children. The difficulty arises in reaction of the child to

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method of examination, a shy child may not co-operate well to record vision, an-other child under the same circumstances may be most co-operative not only in recording of vision but also examination and manipulation of eyes. Vision is a sensory phenomenon for this co-operation of the child is most important pre requisite. Patience, practice and attention of the examiner in recording vision in a child is as important as attention of the child and co-operation of parents. Sixty parent of afferent input to the central nervous system for development of higher centres come from vision of the child. A child does not complain of gradual loss of vision unless it is discovered by the parents at home. A very common observation is that the child prefers to sit nearer the T.V. than others and resents being shifted away. A teacher may complain of poor attention of the child, the child may fail to copy from the black board. Most often children with poor vision are brought with squint. Unilateral loss of vision may go undetected for longer time unless the child develops squint or accidentally closes the better eye. Acute and painful loss of vision brings a child for examination early. Recording of vision in children can be grouped into following categories. Examination of vision of 1. New born 2. Under One year 3. Between 1-2 years 4. Between 2-3 years 5. Between 3-5 years 6. Above 5 year. Recording of vision above five years is similar to that in adults with little modification, attention of examiner and practice. Co-operation of parents, attendants and teachers is also desired for correct interpretation. Recording of vision under one year of age has been delt with earlier. Recording of vision between 1-2 years of age Recording of vision under two years is slightly easier than under one year. Presence of squint, nystagmus, indicate poor vision at least is one eye. Nystagmus denotes bilateral diminished vision. Presence of unilateral squint invariably means diminished vision in the squinting eye. Preference of fixation by one eye means diminished vision in other eye. Closure of the better eye is resented by the child who may start crying and try to remove the obstruction. A child with searching movement most probably does not have vision in either eye. Various methods to examine distant vision are17,18,19 : 1. Optokinetic drum of Harcourt. 2. Catford drum 3. Preferential looking test. 4. Visually evoked potential 5. Teller acuity card

30 6. OKNOVIS17 7. Boeck candy bead test 8. Cardiff acuity card.

PEDIATRIC OPHTHALMOLOGY

None of the above tests is accurate, some times more than one test has to be used. Rarely children as young as 18 months have responded to Snellens optotypes10 Optokinetic drum test, preferential looking test and visually evoked potential and their modifications are time honoured tests. Some new tests have been evolved for better results. They are broadly divided into two groups: 1. That depend upon the preferential looking 2. That does not depend upon preferential looking. The principle of these test are similar to optokinetic drum. Where alternate black and white strips evoke nystagmus when the drum is activated in front of the childs eye from a close quarter. (a) Catford drum test In this test cylindrical drum is replaced by a circular disc with dots ranging between 0.5 to 15mm in diameter representing vision between 6/6 and 2/60. Rotation of the disc at a distance of 60 cm evokes pendular movement and not nystagmus as seen in O.K.T. The smallest target that starts the pendulular oscillation gives the vision it may over estimate vision many folds and can not be used in ambloyopia screening. (b) Teller acuity card10, 17, 18. This test is modification of preferential looking test. This test takes less time and simple to perform. Testing distance varies with age of the child being tested infants under six month are tested at 36cm while those up to three years are examined at 55cm. The results are obtained in cycles which can be converted to Snellens equivalent. (c) OKNOVIS17 This new technique is based on the principle of arresting an elicited optokinetic nyslagmus by introducing optotypes of various sizes. The instrument is a hand held revolving drum that rotates at speed of 12 revolutions per minutes. The test is done at 60cm to elicit optokinetic nystagmus, coloured pictures on the revolving drum are used. Once nystagmus is elicited optotypes of different sizes are introduced to arrest nystagmus. The test gives a rough estimate of vision. (d) Cardiff acuity cards7. The test depends upon preferential fixation and consists of a series cards depending on principle of vanishing optotypes. The tests that do not depend upon preferential looking (a) Boeck candy bead test. The child identifies an incentive i.e. candy beads of gradually decreasing size at 40cm. The childs hand is guided to locate the candy and then to mouth. This the child finds interesting and worth repeating. (b) Other tests used are : Marble game test, Worth ivory ball test, Sheridan ball test. Recording of vision between 2 to 3 years age By this age the child is verbal has better power of expression some may be conversant with alphabets and numbers. Hence preferential looking test are no more required but in cases of gross visual loss or suspected absence of vision, visual evoked potentials may be required.

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Commonly used methods are various types of optotypes that may be 1. Snellens chart 2. Landolts broken C 3. STYCAR Test (Snellens test for young children and retarded) 4. HOTV Test 5. Dot visual acuity test 6. Coin test 7. Miniature toy test. The last three tests are crude tests and meant for children in second year, by three years the child can recognise shapes hence can be tested on optotypes. Recording of vision between 3 to 5 years By three years most of the urban children start going to some types of schools that teach them alphabets, numbers, recognise shape, dimension and colour. Rural children in developing countries who constitute 80% of paediatric population are difficult to evaluate visually due to illiteracy. Examiner may have to revert to methods employed to examine children between two to three years in these rural children. Commonly used methods are : All the test objects are various types of optotypes, the target of which makes an angle of 5 minutes when kept of 6 meters or 20 feet the testing may be done at 3 meters with proportionally reduced size of the optotypes. Commonly used optotypes are : 1. Snellens chart commonly used is in English but may be in vernacular as well. 2. Snellens E chart 3. Snellens tumbling E test 4. Landots broken C 5. Sjogren Hand or Arrow 6. HOTV test (a) Lipman 4 letters ( It consists of four letters HOTV arranged in a circle) (b) Sneridan 5 letters. X is added to HOTV. Addition of A and U make it seven letters, they can be used at 6mts or three meters with a mirror. There should be no difficulty in recording vision in a child if he is literate, he can be tested on usual Snellens chart at six meters. But a non literate child has to be tested on other charts. It is better to have a smaller hand held version of the test type to be shown to the child at usual reading distance and explain the child, what he is expected to tell. After two or three trials the child is able to correctly tell the position of break in Landolts chart and direction of open end of E. While recording of vision following steps are useful to gain the confidence of the child and his co-operation. 1. Record vision in normally lighted room.

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2. Allow one of the parents or grand parents, preferably mother to be present near the child but forbid her to prompt the child. 3. First note the vision with both eyes open. 4. It is better to examine eye with better vision first and the other eye later instead of usual convention of examining the right eye first and left eye later. Because a child with gross visual loss in one eye will resent closure of better eye and may refuse to be tested. 5. Some children generally above five years of age can memorise the whole of the Snellens chart while sitting in the examination room in such cases it is better to ask the child to read from right to left rather then usual left to right barring of course Urdu, Arabic and some other letters. 6. While examining one eye the other eye should be occluded completely and watch that child does not peek over the occluder. 7. Keep the head of the child in primary position. A child may turn the head to see with the better eye. Examination of near vision in children. Children under fifteen have strong accommodation hence their near vision is good and rarely complain of diminished vision children with high uncorrected hypermetropia may complain of asthenopia and running of letters. If a child complains of diminished near vision the first test is to exclude hypermetropia by cycloplegic refraction. Other causes of diminished near vision are 1. Incorrectable distant vision 2. Nystagmus 3. Cycloplegia (a) Neurological-internal ophthalmoplegia (b) Drug induced cycloplegia 4. Malingering. Examination of colour vision. About 8% of boys suffer from some degree of colour defect which is congenital hence it is very important to note colour vision in boys more for future planning of their career than done for any clinical purpose Examination of field. It is very difficult to elicit field defect under 10 years of age. After 15 years of age the field examination is done as in adults. Between 10-15 years automatic perimeters may be helpful.

REFERENCES
1. Boger W.P and Peterson R.A. ; Paediatric Ophthalmology in Manual of Ocular Diagnosis and Therapy, edited by Deborah Pavan langston, third edition p-251-255, Little Brown. 2. Shaffer D.B.; Normal eye in infancy and childhood in Text book of ophthalmology, edited by Scheie H.G. and Albert D.M., 9th edition p-279-280, W.B. Saunders Company, Philadelphia 1977.

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3. Vaughan D., Asbury T.; Special subjects of paediatric interest in General ophthalmology, 10th edition p-279-285, Lange Medical Publication Singapore 1983. 4. Hoyt. C.S. Nickel BL, Billson F.A.; Ophthalmologic examination of the infant, survophth 26 : 177-189, 1983. 5. Safir A., Merker C. ; Normal eye in infancy and childhood in Text book of Ophthalmology, edited by Scheie H.G. and Albert D.M., 9th edition p-279-280, W.B. Saunders Company, Philadelphia 1977. 6. Duke Elder S. and Cook C. ; Chronology of Development in System of Ophthalmology, Vol. III part 1, p-291-312, Henry Kimpton London 1963. 7. Zwaan J.T. ; Does this baby see in Decision making in Ophthalmology, Edited by van Heuven WAJ and Zwaan J.T. p-124-125, Harcourt Brace 1992. 8. Flyon J.T. ; Evaluation of Visual Function in Neonate and Infant in Paediatric Ophthalmology Vol. I, second edition, Edited by Harley M.D. p-1-25, W B Saunders Company Philadelphia 1983. 9. Zwaan J.T. ; Uncorrectable poor vision in a child in Decision making in ophthalmology , Edited by van Heuven WAJ and Zwaan J.T. p-138-139, Harcourt Brace and Company 1992. 10. Diamond G.R. ; Evaluating Vision in preverbal and preliterate infants. In textbook of ophthalmology, Edited by Podos S.M. and Yanoff M. Vol-5, p-2.1-2.6 Mosby London 1993. 11. Goldhammer Y. ; Paradoxical pupillary reaction in Neuroophthalmology update, Edited by smith J.L. P-39-42, Masson New York 1977. 12. Yee R.D. Balon, R.W. Hanrubia. Y. ; Study of Congenital nystagmus Br Jr. OPL 64 : 926-930, 1980. 13. Zwaan J.T. ; Cloudy cornea in neonate in Decision making in ophthalmology, Edited by van Heuven WAJ and Zwaan J.T. p-128-129, Harcourt Brace and Company 1992. 14. Duke Elder S. ; System of ophthalmology, Vol. III, Part-II, Page 820, Henry Kimpton, London 1964. 15. Mc Dermott M.L. ; Corneal hypesthesia in Decision making in ophthalmology, edited by van Heuven WAJ and Zwaan J T P-174, Harcourt Brace and Company 1992. 16. Singh D. ; Paediatric Cataract CME series All India ophtlamological society. 17. Sharma P. ; The preliminary examination and assessment of vision in Strabismus simplified, 1st Edition p-55-62, Modern Publishers, New Delhi 1999. 18. Khurana A.K. ; Visual acuity contrast sensitivity and test for potential vision in Theory and practice of optics and refraction, first edition p-31-51 BI Churchill Livingstone Pvt. Ltd. , New Delhi 2001. 19. Ramanjit Shihota and Radhika Tandon.; Assesment of visual function in Parsons diseases of the eye. 19th edition. p-93-97. Butterworth. Heinemann Oxford 2003.

CHAPTER

Disorder of Lid in Children


The lids occupy a very prominent position on the face and draw early attention to any deviation from its normal appearance due to pathological process or other wise. A. The lids consist of an assorted number of tissues:- the skin with its glands, muscles, motor and sensory nerves and abundant blood supply. B. The junction of the conjunctiva and the skin form the lid margin hence the lids are seats of dermatological, vascular and neurological disorders besides usual infection, inflammation, allergy, and malignancy common to both. C. The disorders of the lid can be congenital or acquired. The acquired conditions outnumber the congenital anomalies. D. Malignant growth of the lid in childhood is almost non-existent. Benign growths are as common as in adults. In fact most of the benign growths of the lid have their origin in childhood and may remain dormant to become obvious at various stages in adult life. E. Trauma is common disorder in all ages.

ANATOMY OF THE LIDS1,2,3


A. Lids are two multiple layered folds, chief function of which is protective. They protect the eye from external foreign bodies, fluids and gases. Besides protection, the lids have two more functions and they are: 1. Regulate entry of light in the pupil 2. Spread the tear over the ocular surface. The lids indirectly help to drain the tear from the conjunctiva and propel it down the nasolacrimal duct. B. The upper lid is larger than the lower one. It spreads from the eyebrow above to lid margin below and from lateral wall of nose to the zygomatic bone laterally, covering the upper 2 mm of cornea. C. The lower lid extends from the inferior orbital margin to lid margin just reaching the lower limbus. The normal lids in adults can be lifted off the globe and everted without any difficult but in children, the lids are so tight that they cannot be lifted off the globe or everted with ease. D.The space between the two lids is called inter palpebral aperture (IPA) or Palpebral fissure. In adults it is about 30mm in length in contrast to 18. 5 mm at birth. The width of 34

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IPA in adult is 9 mm while at birth it is 8 mm. The IPA is a conch shaped space bounded by the two lids. The lids join each other laterally in acute angle and the junction is called lateral canthus. The corresponding medial junction is called medial canthus that is rounded medially. The structures in the medial canthus are caruncle, semi lunar fold and lacus lacrimalis, upper and lower puncta. E. The caruncle represents the vestigial third lid; it does not have skin of the lid. It is a mound of conjunctiva with small hair along with their glands. It has no definite function. F. The plica semilunaries4 is a vertical arcuate fold of conjunctiva with concavity towards the cornea. It contains some plain muscles2. The epithelium is thickened. It also contains goblet cells. This structure represents the nictitating membrane of lower vertebrates with out any function but is invariably involved in pathological process of the conjunctiva. G. The lacus lacrimalis is a shallow depression in the conjunctival surface of the lower lid in the medial side. H. The two lacrimal puncta initiate the lacrimal drainage. They are situated 2mm lateral to the medial canthus in the inter marginal strip, one in each lid. They are circular or oval about 1mm wide in normal eye, they are constantly in touch with the globe and initiate the lacrimal drainage. There are two canthal ligaments, the medial and lateral. I. The medial canthal ligament or medial canthal tendon is a horizontal fibrous structure that extends form medial canthus to the medial wall of the orbit, to this is attached medial horn of the levator and fibrous extension of the tarsal plate. It is palpable as a horizontal cord under the skin when the lateral canthus is pulled laterally. It lies anterior to the lacrimal sac. It splits into two leaves; an anterior leaf and a relatively smaller posterior leaf. The fundus of the lacrimal sac is enclosed in between these two5. The other function of the ligament is to keep the lids stretched horizontally. J. The lateral canthal ligament is less developed than the medial, to it is attached the lateral horn of L.P.S. and lateral fibers from the tarsal plate.

THE LAYERS OF THE LID


The lid is multi-layered structure of both ecto and mesodermal origin. It is highly vascular, rich in nerve supply and lymphatic. The layers of the lids are A. Skin B. Layer of muscles : 1. The orbicularis 2. levator palpebral superior 3. Mullers muscle. C. The fibrous layer : 1. The tarsalplate 2. The orbital septum

36 3. The canthal ligaments 4. The Whitnall ligament. D. The conjunctivaTarsal conjunctiva E. Lid margin.

PEDIATRIC OPHTHALMOLOGY

A. The skin of the lid is thin and delicate but rich in blood supply and sensory nerves. It is very loose; it can be lifted off the subcutaneous tissue. There is no subcutaneous fat. There are only downy hair with their glands scattered thinly throughout. Due to laxity of the skin, large amount of fluid like blood, pus and other effusion can accumulate under the skin. In fracture of paranasal sinuses, air can get under the skin causing emphysema. The Accumulation of fluid , when present is confined to the lids, only due to firm attachment of the skin to the periostium6. The skin of a newborn is tighter than adult. With age it may become loose enough to hang down at old age. The skin is thrown in horizontal lines and most prominent among them is the supratarsal fold that is used as a land mark during ptosis surgery. These horizontal lines are caused by cutaneous attachment of levator palpabral superior. They are obliterated in ptosis and edema of lid. B. Layer of Muscles 1. The orbicularis oculi is a horizontally placed striated muscle supplied by seventh nerve. It has two parts-the orbital and the palpebral. The palpebral part spills over like a sheet in the temple, cheek and the forehead. It encircles the palpebral fissure like a sphincter and closes the lid. It is a direct antagonist of levator. The palpebral part has two smaller divisions i.e. the perceptal and pretarsal. They have their origin over the fascia of the lacrimal sac and their main function is to cause lacrimal fluid to be pumped into the nasolacrimal duct with each blink. Paralysis of orbicularis results in lagophthalmos. The orbital part surrounds the orbital margin. 2. The levator palpebral superior. This striated muscle, supplied by upper division of the third cranial nerve is the main elevator of the upper lid. Its function is to lift the upper lid and keep lifted. It is direct antagonist of orbicularis. The muscle originates from the under surface of lesser wing of sphenoid above and in front of the optic foramen. It travels between the roof of the orbit above and superior rectus below. It reaches the lid in a wide aponeurosis that is divided in three slips. (a) The main slip is attached to the superior border of the tarsalplate, some fibres blending with the anterior surface of tarsal plate. (b) Some fibers anterior to this pass through the fibers of the orbicularis and get inserted in the skin to form horizontal skin creases. (c) The posterior most fibers are attached to the upper fornix to give it its depth. The lateral horn of the aponeurosis is attached to lateral orbital tubercle and lateral canthal ligament. The medial horn gets inserted in medial canthal ligament and frontomaxillary suture. Part of the aponeurosis blends with Whithnall ligament that acts as check ligament of the levator. 3. The Mullers Muscle8. This in contrast to the levator and orbicularis is a non striated muscle. It lies under the conjunctiva as an ill defined mass 12 x 15mm that originates from under surface of levator and gets inserted on the upper border of the tarsal plate, deep in the

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main aponeurosis, It is supplied by cervical sympathetic. It is an accessory elevator of lid its paralysis causes mild ptosis of upper lid. It augments the action of L.P.S. by 2mm. A similar but less developed muscle arises from the inferior rectus and gets attached to the lower border of tarsus of lower lid. Its paralysis causes elevation of the lower lid margin and the condition is called upside down ptosis. C. The Fibrous Layer 1. The tarsal plate. Each lid has a tarsal plate or tarsus that is responsible for consistency and shape of the lid and gives contour to the lid margin. The tarsal plate along with orbiculris that is loosely attached to its anterior surface, form a strong protective layer when the lids are shut. The upper tarsal plate is larger than lower tarsus in all dimensions. It is about 30mm long; 9 to 12mm in height and 1mm thick. The tarsus are made up of fibrous tissues in which are embedded the meibomian glands that are arranged parallel to each other and have branching ducts. The meibomian glands open on the lid margin and these glands secrete an oily material that contributes maximum to the lipid layer of the tear. The upper tarsal glands are longer and have wider lumen than lower glands. The upper tarsus has about 25 glands while the lower has only 12 to 15 glands. The lower border of the upper tarsus is slightly concave. Either side of the tarsal plate is anchored to the corresponding canthal ligament. The inner surface of the tarsal plate is lined by tarsal conjunctiva. The tarsal glands are visible through the normal tarsal conjunctiva as light streaks. 2. The orbital septum. This structure is also known as palpebral fascia. It is a sheet of fibrous tissue of variable thickness. It is pierced by aponeurosis of levator in the upper lid and fibers from interior rectus in the lower lid. It originates from the periorbita of the orbital margin and gets attached to the peripheral margin of the tarsal plates. Medially it is attached posterior to the fossa for lacrimal sac and laterally to the orbital tubercle. The septum orbitale along with tarsal plate form a vertical barrier that separates the orbit from the lid. It limits the infective process to reach the lid from orbit and vice versa. It is not a very rigid structure. It is pushed posteriorly by effusion in lid and forward by same pathology behind. It ripples with movement of the globe. The septum prevents orbital fat to herniate in the lid. 3. Canthal ligaments. See above. 4. The Whitnall ligament9. This is a fibrous band that runs horizontally above the aponeurosis of levator palpebral superior from Whitnall tubercle on the zygomatic bone. It gives attachment to levator aponeurosis. It is one of the important landmarks in ptosis surgery. D. The tarsal conjunctiva The tarsal conjunctiva lines the tarsi, form the inter marginal strip of the lid margin to the fornices in each eye. The normal tarsal conjunctiva is translucent over the tarsal plate. The meibomian glands are visible through the conjunctiva, so long it is translucent. The duct become obscure due to edema, follicle, papillae and scar in the tarsal conjunctiva. The tarsal conjunctiva cannot be lifted off the tarsus. E. The lid margin The lid margins form the borders of palpebral fissure. They extends from canthus to canthus in each lid are about 3 mm in thickness, its posterior border is sharp and must remain constantly in touch with the globe for proper spread and drainage of tear. The anterior border

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PEDIATRIC OPHTHALMOLOGY

is rounded; the lashes arise from the anterior border in two or three rows. The upper lid being larger has more and longer lashes than lower lid. At the base of the lashes open the modified sebaceous glands the glands of Moll and Zeis. The glands of Moll are modified apocrine sweat glands that pour the secretion in the lash follicle. The glands of Zeis have no lumen they produce lipid. The skin of the lid and conjunctiva merge with each other on the lid margin hence it has characteristics of both skin and conjunctiva and called inter marginal strip. The orifices of the meibomian glands open on the lid margin behind the lashes. There is a faint grey line along the lid margin. The lid can be surgically split into two halves, the anterior containing skin and orbicularis and the posterior containing tarsus and conjunctiva along this line.

THE BLOOD SUPPLY


The lids have very rich blood supply this is the cause of rapid healing of lid wounds. The lids get their arterial supply from both internal and external carotid arteries. The blood supply is broadly divided in superficial system and deeper system the former is again divided into facial and orbital groups. The arteries of the lid ultimately form two arterial arcades in each lid, one above the tarsal plate and other near the lid margin.

THE VENOUS DRAINAGE


The lids have two types of venous drainage i.e. in the pretarsal and retrotarsal, which drain in external juglar, cavernous sinus and pterygoid plexus.

THE LYMPHATIC DRAINAGE


The lymphatic from lateral half drain into pre auricular nodes. Those from medial side drain in sub mandibullar group of nodes.

THE NERVE SUPPLY


The motor supply is from facial and oculomotor, the sensory is from trigeminal, the nerve to Mullers muscles is via sympathetic.

CONGENITAL ANOMALIES OF THE LIDS38,39,40,41


A. Congenital anomalies of the lids are generally bilateral and symmetrical, unilateral congenital anomaly is no exception. The congenital anomalies may be confined to the lid or may be associated with other anomalies of globe and systemic anomalies. A common combination is congenital anomalies of lid with cranio facial and mandibulo facial deformity. Children with congenital lid anomalies are brought to physician generally for cosmetic blemish or exposure of the globe. However, an anomalous lid, if it covers the pupil may result in

DISORDER OF LID IN CHILDREN

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amblyopia that requires early management. Large exposure of the globe due to coloboma of upper lid may also require urgent treatment. B. Congenital anomalies of lid can be : 1. Deformity of lid margin (a) Coloboma (b) Ankyloplepharon (c) Distichiasis (d) Entropion (e) Ectopion 2. Deformity of the interpalpebral aperture (a) Telecanthus (b) Epicanthus (c) Blepharo phimosis (d) Euriblepharon 3. Congenital Ptosis and allied conditions 4. Disorders of the lid skin (a) Blepharochalasis (b) Epiblepharon 5. Minor and rare deformities 1. Deformity of Lid Margin (a) Coloboma of the lid. Coloboma of upper lids result due to improper fusion of medial and frontonasal mesodermal process of face and are situated in the medial one third of the upper lid in the form of a small notch or as one extending considerably upwards. The colobomas generally have a broad base at the lid margin; it may involve the upper punctum and canaliculus. However, a large rectangular defect may develop in the middle of the lid, exposing upper part of the globe and conjunctiva. Coloboma of upper lid is generally unilateral. It is not uncommon to find other congenital anomalies in the same eye i.e. corneal opacity, microphthalmos, coloboma of the pupil. Dermoid or dermolipoma may be present in Goldenhars Syndrome. The lower lid colobomas are seen at the lateral third of lower lid, they are not so well demarcated as coloboma of the upper lip and may be missed unless carefully looked. Lower lid colobomas are wider and shallower; they by themselves are almost symptom less. They are a constant feature of mandibulo facial syndrome i.e. TreacherCollin, Franceschetti syndrome. (b) Ankyloblepharon. The lids are fused between third and sixth month intra uterine life and start separating with development of cilia and glands of the lid. The separation starts from the middle and spreads on each side. The separation is complete at the time of birth. Some times few strands of skin may extend upon the inter palpebral aperture attached to the other lid, these strands are called ankyloblepharon. They are more common on the lateral side, may present as a single skin tag or there may be four or five strands. These are called ankyloblepharon filiformis adnatum. Medial strands are less common, central are least

40

PEDIATRIC OPHTHALMOLOGY

common. The strands do not contain any other tissue except skin and few capillaries. The strands can be cut without difficulty soon after birth. (c) Distichiasis. This is a rare congenital condition where orifices of meibomian glands are replaced by an extra line of cilia in addition to normal three or four that are present at the anterior rounded border of lid. There is an intact inter marginal strip between normal lashes and distichiasis. The distichiatic lashes grow posteriorly and rub against the cornea and conjunctiva. It is not uncommon to confuse them with cicatricial trichiasis in adults. In cicatricial trichiasis, the in turned lashes arise from the anterior margin of the lid. Treatment consists of repeated epilation when they are a few other wise plastic procedures are required to turn the lashed forwards. (d) Entropion. Congenital entropion lids are very rare. They are more common in females, frequently seen with epicanthicfold, they may be confused with epiblepharon, distichiasis and trichiasis. (e) Ectropion. Congenital ectropion is also rare. This is due to vertical shortening of the lid skin following change in subcutaneous tissue. All the lids may be involved; it is more common on the lateral side. It may be present as an isolated defect or may be associated with blepharophimosis, telecanthus, epicanthus inverses or ptosis. 2. Deformity of the Interpalpebral Aperture (a) Telecanthus. Telecanthus is lateral displacement of medial canthi due to abnormal lengthening of medial canthal ligament . It is generally bilateral. Normal inter canthal distance is generally half of inter pupillary distance. In telecanthus this increases proportionately. True telecanthus must be differentiated from hypertelorism which is increased distance between two orbits Telecanthus may be associated with epicanthus. It is also seen in Waardenburgs syndrome. Isolated telecanthus does not produce any symptom and does not require any treatment. (b) Epicanthus. This bilateral symmetrical congenital anomaly is very common in otherwise normal infants and disappear with age. It is a distinct feature of oriental races. It is a consistent finding in Downs syndrome Epicanthus is seen as a vertical semilunar fold of skin on the medial side of the palpebral aperture with concavety towards the cornea. According to its extent, it can be : Epicanthus supraciliary when the skin folds starts just below the eyebrow. Epicanthus palpebralis, here the lid fold starts at the level of normal lid fold this is the commonest type of epicanthus. Epicanthus tarsalis, here the lid fold starts at the level of upper border of tarsus. Epicanthus inverses are same as any epicanthus only difference being that skin folds starts from the crease of the lower lid. Simple epicanthus is almost symptom free. the children are brought for cosmetic blemish. Large epicanthus fold causes pseudo convergent squint that disappears on abliterating the epicanthal fold. In most of the children the epicanthus disappears with age. Ptosis with epicantus is a common presentation Blepharophimosis, ptosis, telecanthus and epicanthus inversus represent Komotos Tetrad42.

DISORDER OF LID IN CHILDREN

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Differential diagnosis consists of epiblebharon where there is no superior palpebral fold but there may be trichiasis. Epicanthus may be associated with punctal stenosis. There may be amblyopia due to associated ptosis. Rarely there may be trichiasis. (c) Blepharophimosis . This is congenital reduction of length and width of interpalpebral fissure. This is always bilateral and symmetrical. There is no defect in muscles. Simple blepharophimosis is rare. It is generally associated with epicanthus inversus, telecanthus and ptosis. The child keeps the chin raised and there are prominent furrows on the forehead to compensate associated ptosis. Blepharophimosis may give impression of pseudo convergent squint. It is corrected for cosmetic reasons by oculoplasitc repair, multiple surgeries may be required. (d) Euriblepharon (Euryblepharon). In this case there is uniform enlargement of interpalpebral fissure, there is displacement of outer canthus laterally. This condition should not be confused with other causes of enlargement of IPA i.e. lagophthalmos, proptosis, ectropion, congenital coloboma of lower lid. 3. Congenital Ptosis and Allied Disorders. See page 43. 4. Disorders of the lid skin (a) Blepharochalasis. This is generally familial bilateral condition where there is atrophy of the dermis of the upper lid skin that hangs loose. The orbital fat may protrude in to the overhanging skin, covering the pupil. It can be congenital or juvenile. The juvenile form starts between seven to twenty years with episodic attacks of lid swelling (b) Epiblepharon. This is a congenital condition where there is a horizontal fold of skin either in the upper or lower lid that runs parallel to the lid margin, lower epiblepharon is more common. It may be associated with congenital entropion of lower lid. 5. Minor and Rare Deformities (a) Ablepharon (Cryptophthalmia). A very rare condition in which there is failure of lid folds to develop in to lid. The skin of the face passes over a deformed eyeball to the cheek without inter-palpebral aperture. (b) Microblepharon. In this case the vertical distance of the lid is short both lids, may be involved. There is mild lagophthalmos. When the patient sleeps a part of the lower bulbar conjunctiva may be visible. (c) Mongoloid obliquity of lid is up and outward obliquity of the outer canthus. This is a constant feature of monogolism. (d) Anti mongoloid Obliquity is downward displacement of outer canthus.

CONGENITAL PTOSIS AND ALLIED CONDITIONS


A. Congenital ptosis is commonest congenital anomaly of the lid for which a child may be brought, specially seeking treatment for cosmetic defect. It may be present at birth but not much attention is given to ptosis, as the neonate keeps the eyes closed most of the time and normal palpebral fissure is very narrow as compared to adults. However by three months of

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age the parents become aware of ptosis especially if it is unilateral. Many a times a child may reach second decade not being aware of ptosis in mild cases. Congenital ptosis has strong hereditary tendency. B. Most of the congenital ptosis are due to mal-development of the levator palpebral superior. However congenital mal-development of third nerve nucleus or its trunk may be a contributory factor. In very small number of cases, congenital Horners Syndrome is seen. Myogenic cause especially neonatal myasthenia may present as ptosis, so may more rare myotonia. C. Congenital ptosis can be simple unassociated with other extra ocular muscle palsy or may be associated with 1. Superior rectus palsy, 2. Superior rectus and inferior oblique under action, 3. Other extra ocular muscle palsy. 4. It can be associated with other deformities of the lid like epicanthus, blepharophimosis D. Simple congenital ptosis may be unilateral or bilateral. Both the sexes are equally affected. Generally there is normal vision, accommodation and pupillary reaction. A miotic pupil with ptosis points towards Horners syndrome. Generally levators function is moderate to mild in congenital ptosis and remains unaffected throughout life. It is not only the elevation that is subnormal downward movement of the lid is also defective (lid lag). These changes are due to dystrophic changes in L.P.S., Lid lag is not seen in any other ptosis except congenital ptosis. Though vision is unaffected amblyopia may develop due to 1. The lid covering the pupillary area 2. Associated myopic astigmatism. 3. Associated strabismus. In orders to have better vision the child uses frontalis, which elevates the lid slightly. Action of the frontalis can be obliterated by pressing this muscle against the frontal bone. This procedure is utilised to measure levator function. The use of frontalis by a child with ptosis throws the forehead skin into deep horizontal furrows and raises the eyebrow in an arched form. Throwing the head back (chin up) helps to compensate lowered position of lid. Raising of chin and arching of eye brow develop with age. They are more marked in bilateral cases. E. Congenital ptosis with superior rectus under action is frequent. Superior rectus and levator palpebral superior develop from same mesodermal mass hence identical dystrophic changes may develop in superior rectus in case of congenital ptosis. Involvement of other extra ocular muscle may be central in origin. The patient may be unable to elevate the globe with ptosis hence it is mandatory that in cases of ptosis action of the superior rectus should also be evaluated. Uniocular and binocular movements of all other extra ocular muscles and orbicularis should also be tested along with corneal sensation this has prognostic importance in surgery. F. Synkinetic movements of the lid. Paradoxical movement of the lid is generally

DISORDER OF LID IN CHILDREN

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present with congenital ptosis. It may be present without ptosis as well 1. Synkinetic movement of lid with ptosis (Jaw winking), Marcus Gunn phenomenon. This is the commonest congenital paradoxical movement of the lid. This is seen with congenital ptosis in infancy. The mother notices abnormal movement of the upper lid in an act of sucking. The paradoxical movement persists for rest of life. It is always unilateral. In primary position, with mouth closed there is mild ptosis as the patient opens the mouth the lid drifts upwards. Raised position of the lid is not maintained if the mouth is kept open. If the jaw is moved towards the side of effected eye, ptosis increases. Sometimes forward protrusion of the jaw may produce same phenomenon Marcus Gunn phenomenon is seen only on sucking or chewing, it is not seen on smiling or coughing. The cause of this phenomenon is congenital misdirection of some of the fibers of trigeminal that supply the pterygoid muscle, to the oculomotor nerve. 2. Synkinetic movements of the lids, with out congentical ptosis (a) Movement of the upper lid with contraction of medial rectus (b) Movement of the lid on contraction of lateral rectus (c) Raising of upper lid on contraction of inferior rectus i.e. looking down.

ACQUIRED DISORDERS OF THE LIDS IN CHILDREN


Acquired disorders of the lids in the children can be A. Non Specific like as 1. Edema 2. Ecchymosis 3. Change of colour. B. Specific like 1. Infection 2. Inflammation 3. Injury 4. New growth 5. Vascular 6. Neurological. A. Non Specific 1. Edema. Edema of the lids is a common feature of lid due to divers cause that can be : (a) Inflammatory (b) Allergic (c) Systemic (d) Traumatic - Blunt, thermal, chemical, radiation. (a) The Inflammatory Causes of the Lids may be (i) Infection of the lids.

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Bacterial-lid abscess, cellulitis lid, stye. Viral-herpeszoster, herpessimplex, chickenpox, measles, molluscum. Fungal. Parasitic. Severe uveitis Endophthalmitis Purulent conjunctivitis Panophthalmitis Epidermic conjunctivitis.

(ii) Infection of globe


(iii) Orbital cellulitis. (iv) Cavernous sinus thrombosis. (v) Proptosis (b) Allergic. Acute angioneurotic edema, insect bite, dermatitis medicamentosa, atopic dermitis. (c) Systemic conditions that produce edema of lids are acute and chronic nephritis, hypoproteinaemia, myxedema and thyrotoxicosis. Edema may be acute or chronic. It may involve any or all the lids. It may be very mild giving a puffy appearance or may be so pronounced as to obliterate inter palpebral fissure requiring lid retractor to separate the lids. The edema may be transient, manifesting only in a particular time of the day.the child may get up with edema of the lids that pass off within few hours. This is generally due to systemic causes requiring pediatric consultation. Treatment of edema. Treatment of lid edema is essentially treatment of its cause. The local infective causes are treated by antibiotic - local or systemic and hot fomentation. Allergic edema may subside with oral and rarely injectable antihistamine. Steroids are indicated in angioneurotic edema. 2. Ecchymosis of the Lid. The lids are very vascular, hence trauma easily causes extravasations of blood in the lids due to laxity of lid skin. Blood accumulates in the subcutaneous space, giving rise to Ecchymosis. The ecchymotic lid is swollen and bluish green in colour. In infants it may have a red discoloration. As time passes, the colour of lid changes from red to bluish green to black. Hence called Black eye. The causes of black eyes are: (a) Blunt injury to the lid, orbit, scalp, middle cranial fossa. (b) Blood dyscrasiaPurpura, haemophilia, leukaemia. (c) Neuroblastoma. Ecchymosis of the lid may be localised to lid only. It may be associated with sub conjunctival haemorrhage with or without rupture of the globe. In injury to scalp, fracture of orbit or fracture base of skull ecchymosis appears few hours to twenty four hours after the initial injury as it takes some time for the blood to trickle down along the fascia, muscle plane, or along the sheath of the extra ocular muscles. Presence of ecchymosis may be frightening but

DISORDER OF LID IN CHILDREN

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not always vision threatening and most of the time disappears without treatment. However ecchymosis of any kind may be just a superficial sign of a deeper malady i.e. fracture base of skull, blood dyscrasia or neuroblastoma or traumatic optic neuropathy. Treatment of ecchymosis. Echymosis of lid per se does not require any treatment. As a first aid cold compress may reduce the swelling. However after few hours cold compress does not help. Analgesics reduce pain and inflammation. It takes about seven to ten days for simple ecchymosis to disappear. 3. Colour change of the lid. Generally the colour of the lids is similar to that of face and forehead. Some times there may be localised colour changes due to ecchymosis which is temporary and passes off. Birthmarks may last for first few years and gradually disappear. However some of the birthmarks are permanent like naevus, Sturge Weber syndrome and neuro fibromatosis. Hypopigmentation is seen following injury burn and vitiligo.

INFLAMMATION OF THE LID


Inflammatory process may involve : A. The lid margin (Blepharitis). B. Glands of the lid margin. C. Skin and subcutaneous tissues of the lid. Inflammation of the lid margin is called Blepharitis1,2,3,4. The Causes of blepharitis are bacterial, seborrhic, parasitic, atopic, and allergic. Blepharitis is broadly divided into squamous, ulcerative (staphylococcal) and parasitic. The former is mostly seborrhic in nature while later is chronic infection by staphylococci or due to its toxins. The squamous (seborrhic) blepharitis This is basically disorders of gland of Zeis and is associated with seborrhic dermatitis of eyebrow, scalp and nasolabial fold. The condition is mild. The lid margin has dry scales or greasy material, which are easily removed and on removal there are no ulcers or hemorrhagic spots that are common in ulcerative blepharitis. Seborrhic blepharitis may produce mild chronic conjunctivitis and punctate keratitis in lower part of cornea. Ulcerative blepharitis This is more troublesome, produces more complication and is difficult to eradicate. It may be simple staphylococcal or it may be a combination of seborrhoea and staphylococcal infection. This makes it difficult to treat. It generally starts after 3 years of age, involves both the lids on both sides. Involvement of upper lid is more extensive than lower lid. There are yellow crusts at the base of the lashes that may extend over the rounded anterior lid margin and the skin. The cursts are difficult to remove, without wetting them. They are best rubbed off after being moistened by warm water for few minutes. On removal of the crusts, the underlying skin is found to be hyperaemic, and small ulcers are seen. On long run there may be tylosis, madarosis and trichiasis. It is always associated with chronic blepharo

46 conjunctivis and may produce superficial punctate keratitis.

PEDIATRIC OPHTHALMOLOGY

Management of blepharitis is difficult. Treatment of seborrhic blepharitis consists of management of systemic seborrhoea i.e. regular cleaning of the face, use of suitable antidandruff shampoo for the scalp and eyebrow. Local instillation of broad-spectrum antibiotic drops, to prevent secondary infection, for months. Treatment of ulcerative blepharitis is prolonged. Strict and energetic regime should be followed which consist of: (i) Removal of crusts by cotton swab soaked in lukewarm water that is applied on the lid margin for 2-3 minutes and the crusts rubbed off with dry cloth. (ii) Broad Spectrum antibiotic is rubbed on the lid margin. The whole procedure is repeated at least three times a day, same antibiotic is instilled in the conjunctival sac at bed time, steroid with antibiotic ointment may be rubbed on the lid marginthree times a day and gradually tapered off. If children do not respond to local treatment systemic antibiotic may be required for seven to ten days. (iii) Personal hygiene. The children should be taught proper hygiene of the face and hands. (iv) Associated errors of refraction. Should be corrected after proper retinoscopy under cycloplegia. Complications of ulcerative blepharitis consist ofTylosis, madarosis, frequent stye, trichiasis, chronic conjunctivis, keratitis and epiphora. Parasitic blepharitis Pediculosis and phthiriasis of lids14,15 Common parasitic involvement of the lid margin is pediculosis. There are three types of these arthropods that involve the lid margin. They are pediculosis-corporis, p. capitis and p. pubis. The child gets the infection due to p. capitis from siblings or playmates, while they get the other two types from their infected parents, servants or baby sitters. The lid margin is infested by these parasites which cling to the skin of the lid margin, they lay nits on the shaft of the lashes. Numbers of parasites vary from a few to numerous. They are visible on naked eye when many, but may require magnification when few. The parasites produce chronic itching, redness and secondary infection of the lid margin, conjunctiva and lower cornea. Treatment. The treatment is mainly treatment of systemic pediculosis in all the contacts. (i) Local Treatment. In co-operative and older children the parasites may be picked up with epilation forceps or destroyed by direct application of cryo. Anti pediculosis shampoo after proper dilution may be applied to the lid margin. This should precede application of any ophthalmic eye ointment in greasy base. The oily base acts as mechanical repellent for aqueous shampoo which other wise may get into the conjunctiva causing chemical conjunctivitis. 20% fluorescein sodium have been used with good result. The drugs commonly used as shampoo are benzene hexachloride. (Lindane) 1%, premethrine 1%. (ii) Follow up. Personal hygiene is the key word in management of pediculosis and phthiriasis. The cloths, bedding, combs hairbrushes should be washed in boiling water to avoid

DISORDER OF LID IN CHILDREN

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re-infection in carriers and infected children. The contacts should also be treated vigorously and repeatedly for pediculosis. B. Disorders of glands of lid margin 1. Stye (Hordeolum externum). The stye is an acute staphylococcal infection of gland of Zeis and Moll. They are very common between five years to fifteen years. Boys and girls are equally affected. Tendency of stye runs in families. It is a painful condition that begins as tender red spot on the lid margin. The position of an early stye in the lid can be pin pointed by moving the finger on the lid margin. The patient winces as the exploring finger passes over the stye. There is diffuse swelling of the lid on either side of the stye. Styes situated near the canthi are more painful than in the middle of the lid. Styes of outer canthi are most painful. The upper lid has more glands of Zeis and Moll, hence styes are more frequent and more numerous in upper lid. There may be a single stye that may be cured spontaneously not to appear again. Repeated attacks of styes are very common. There may be multiple crops of styes. Both lids may be affected. Each lid may have single to multiple styes. All lids may be affected at the same times. Associated conjunctivitis is frequent. Preauricular lymph nodes are always enlarged that may be tender. Development of stye after measles is very common. Other predisposing factors are uncorrected errors of refraction that leads to asthenoipa. A child with asthenopia has tendency to rub the eyes with fingers frequently. This introduces infection in the lid margin resulting in repeated styes that disappear with correction of errors of refraction. Other predisposing factors are chronic seborrhic and staphylococcal blepharitis . Management of Stye Management of stye consists of (i) Treatment of stye. (ii) Prevention of recurrence. (iii) Management of complications. Treatment of Stye The treatment is similar to treatment of a boil that consists of local dry hot fomentation, systemic analgesic, anti-inflammatory drug and systemic antibiotic. Local antibiotic eye ointment at bedtime prevents matting of lid margins at night. With above treatment either the stye subsides by evacuation of pus or the lash round which the stye has developed, pus is epilated. Prevention of recurrence As error of refraction is one of the important predisposing factors, it should be corrected by appropriate glasses following refaction under cycloplegia. Stye itself can induce slight astigmation hence refraction should be under taken only when the child is free from stye. Proper lid hygiene should be maintained. Cleaning the lid margins and application of broad spectrum antibiotic eye ointment at night for at least two months after the stye has subsided. In chronic and recurrent stye doxycyclin 100mg daily for ten days gives relief. However doxycyclin is not preferred in children under eight years as it may cause permanent staining of the teeth. In such children long acting sulpha or erythromycin in consultation with pediatrician should be given.

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PEDIATRIC OPHTHALMOLOGY

Cutting of consumption of sweets like candies, chocolates and supplementation of food with multi vitamin orally helps in reduction of recurrence. Children should be instructed to keep the hands clean and nails trimmed. 2. Chalazion. In contrast to stye, chalazion is a chronic granulomatous inflammation of meibomian glands. Otherwise chalazion share same epidimiology as stye i.e. it is a disorder of children and young adults and it is equally common in both sexes, any or all lids may be involved separately or simultaneously. There may be repeated crops of chalazia. It is more common with uncorrected errors of refraction. It differs from stye in following points: It is chronic granuloma, neither painful nor tender, preauricular glands are not enlarged. The chalazion is heralded by changes in the duct of the meibomian gland which are obstructed due to infection spreading from the lid margin. This results in stagnation of oily meibomian discharge in the acini of the glands. This pent up lipid acts as an irritant that result in a granuloma with giant cell without caceasion. The chalazion is well circumscribed and smooth without a true capsule of a cyst. The skin over the chalazion can be moved. On evertion of the lid the conjunctiva over the chalazion shows a bluish coloration. There may be multiple chalazia in the same lid or in all the lids. Chalazion is a selflimiting inflammation. Small chalazion may resolve without any treatment or simply by frequent hot fomentation. If it does not resolve by it self-following changes may be seen:. (i) The growth increases in size and the central core may liquefy forming a tarsal cyst that may cause pseudo ptosis and induce astigmatism. (ii) It bursts on (a) Most commonly on the conjunctival surface, giving rise to a sessile papillomatous granuloma. (b) It may less frequently rupture on the skin surface. (c) Rarely it may protrude through the opening of meibomian orifice. Chalazia in children never undergo neoplastic change. Though meibomian cell carcinoma after 40 years may initially present as chalazia. (iii) The chalazion gets secondarily infected and becomes severely painful. It is called Hordeolum internum. Sometimes chalazion may be associated with stye as well. Management of chalazia21,22 Many of the chalazia resolve without treatment. However if a chalazion persists it requires surgical drainage and curettage of the granulomatous tissue followed by cauterisation of the wall of the growth by carbolic. In children chalazia should be incised under general anaesthesia. Intra lesion injection of steroid: 10mg of triamcinolone acetonide is injected in the chalazion under local anaesthesia from conjunctival surface. A patient may require two to three such shots. Hordeolum internum requires more energetic treatment that consists of dry hot fomentation, systemic analgesic and anti-inflammatory drugs, systemic broad-spectrum antibiotic. No attempt should be made to drain the acute chalazion unless pain and

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inflammation has subsided. Prevention is similar to that of stye i.e. hygiene of lids, face and hands, correction of errors of refraction, long term local application of broad spectrum antibiotic on the lid margin. Differential diagnosis of burst chalazion consist of : Foreign body granuloma. Papilloma. Haemangioma. Rhinosporidiosis of tarsal conjunctiva. C. Cutaneous and subcutaneous inflammation of the lids All infective processes that involve the skin of the face and forehead can affect the lid. They can be viral, bacterial and fungal. 1. Viral infection of the lids are : (a) Chicken pox (b) Measles (c) Molluscum (d) Herpes zoster (e) Herpes simplex All the above conditions cause eruptions on the lid, may involve the conjunctiva, as well as cornea. Herpes zoster is classically unilateral others are bilateral all the above conditions have or may have systemic involvement. (a) Chicken pox (Varicella)16,18 is very common exanthematous disorder of the lid caused by varicella zoster virus, it starts as hyperaemia with vesicle formation, which subside within 10 to 12 days leaving no scar, the vesicles can be seen on the lid margin as well, may develop in conjunctiva simulating phlycten. There may be ulceration or vesicles on the conjunctiva, may produce superficial keratitis pseudo dendretic keratitis and disciformkeraitis. Frank ulcer is not uncommon. If there is involvement of nasolacrimal duct it may lead to chronic dacryocystitis4. Treatment. There is no known method to immunise the child against chicken pox, there is no specific systemic treatment. The child should be observed for possibility of pneumonia or encephalitis. Antiviral drugs have not proved to be effective. Immunoglobulins have favourable result when started with in three days of onset of skin rashes. Ocular management. Consists of relief of photophobia and watering due to corneal involvement. Antibiotics are used to prevent secondary bacterial infection. The child should get full dose of cycloplegia, if needed atropine may be used. (b) Measles (Rubeola, Morbilli)23,24. Measles is very common among non-immunised children. It mainly affects the respiratory tract and conjunctiva. The lid it is involved as part of generalised hyperaemic rashes that become papules, these are slightly raised and red. They may be separate or merge with each other. Conjunctivial involvement is universal; it starts as

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catarrhal conjunctivitis that may be superimposed by secondary infection. Koplic spots develop on the conjunctiva, there may be sub conjunctival haemorrhage. corneal involvment is common causing blepharospasm. There are various stages of epithelial keratopathy, frank corneal ulcer develop in malnourished children. Blindness due to measles can be as high as 1% of all children affected mostly due to bacterial infection, malnutrition and hypovitaminosis A. There may be edema of the lid, cellulitis lid, multiple styes and blepharitis. Management Treatment is symptomatic. All parents should be encouraged to get children immunised against measles. It is one of major cause of depletion of vitamin A and not only vision threatening but potentially fatal. 1. Compulsory immunisation is essential not only to save the eye but also life of the child. 2. Administration of additional does of water-soluble vitamin A 50,000 I.U.daily for four days orally. 3. Bland lotion to prevent sticking of eyelids. 4. Broad spectrum antibiotic drops for conjunctivitis 5. Cycloplegie if cornea is involved. (c) Molluscum contagiosum. This is a chronic self-limited virus disease of skin caused by poxvirus. The lesions may appear any where on the body as dome shaped, translucent vesicles, which are umblicated, pain less, not associated with fever or any other systemic manifestation. They are not as numerous as eruptions of chicken pox, or small pox, number may vary from few to few dozens all over the body. May be seen on the lid or lid margin or both. Some times they are seen on conjunctiva or cornea. Corneal involvement causes redness, watering and photophobia due to pseudo dendritic ulcer, pannus formation and sub epithelial infiltration. When the lesion is near the puncta it may block the puncta causing epiphora. Keratitis and follicular conjunctivitis are toxic in nature. Treatment. The disease is self-limiting but may last for weeks. It is contagious. There is no known immunisation, anti viral drugs are of not of much use. They are best treated by puncturing each lesion either by needle or electrocautery, the interior of the punctured lesion is curetted by tincture iodine or weak solution of carbolic. The lesion can be treated with cryo as well. Corneal involvement may require broad spectrum anti biotic drops and cycloplegia. (d) Herpeszoster Ophthalmicus18,19,20. Herpeszoster ophthalmicus is an acute eruptive condition of the lid caused by zoster varicella virus that causes chicken pox otherwise. It is morphologically similar to herpes simplex virus but differs in antigen. Herpes zoster ophthalmicus can occur at any age, however it is less common in children. Children tolerate pain of herpes zoster better than adults, may be the pain is less sever in children. Children are not known to suffer from secondary zoster that is seen in immuno compromised adults. Herpeszoster ophthalmicus may be mild or may be very severe. It is known to affect all parts of the eye and adnexa except the lens. In herpes zoster ophthalmicus the infection lies dormant in posterior root ganglion of

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trigeminal nerve following systemic infection either in the form of chicken pox or zoster itself and then travels down the first division of the fifth nerve to reach the eye and adnexa. Herpes zoster ophthalmicus starts as hyperaemic rash over the fore head and skin of the lid or lid margin. May be associated with mild fever and malaise. The eruptions are initially macular and soon converted into vesicles similar to chicken pox. Generally facial pain precedes appearance of rashes. The vesicles are as a rule always unilateral they do to cross the mid line however associated edema of the forehead, lid and face may spill over the mid line giving a false impression of allergic edema or bacterial cellulitis. Preauricular lymph nodes are enlarged. Like chicken pox the vesicles take ten days to dry and crust. The crust fall in due course leaving shallow scars that may disappear in time. If the lesion was deep it may leave permanent scar on the forehead, lid or lateral side of the nose. Scar of the lid margin may cause notching of lid margin or trichiasis. If the vesicles are present on the tip of the nose it means that nasocillary nerve is also involved. This is called Hutchinson sign. This is almost sure indication of corneal involvement. However cornea may be involved without involvement of nasociliary nerve. Lesions of herpes zoster may be divided into (i) Involvement of lid and conjunctiva (ii) Involvement of globe (iii) Neurological lesions (iv) Post herpetic complications (i) Lids may show very mild hyperaemia to server vesicular eruption and edema of the lid obliterating interpalpebral fissure. The vesicle may develop along the distribution of one or all the branches of first division of fifth nerve. Involvement of maxillary division is rare. In initial stages herpes zoster of lid may be confused as allergic edema, insect bite, stye or bacterial cellulitis Conjunctiva may show vesicles, chemosis and subconjunctival haemorrhage. It is very common to develop secondary bacterial conjunctivitis. (ii) Involvement of the globe

Corneal involvement. Involvement of cornea is very common. It may happen without involvement of nasociliary nerve, its severity is not directly proportionate to lid involvement, server corneal involvement may be seen with mild lid involvement. Corneal involvement is varied it may be :Epithelial punctate infiltrates that stain better with rosebengal, microdendrites filamentary keratitis Diminished corneal sensation Numular keratitis Disciform keratitis Corneal involvements do not respond much to anti-viral drugs but respond to local steroids. Numular keratitis is known to change density from time to time without treatment.

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Scleral involvement. Scleritis is less common than keratitis it may be missed due to lid edema and conjunctivitis. Scleritis is less common in children. Uveitis. Anterior uvea may be involved either as primary inflammatory process of or secondary to keratitis. Forty percent of eyes with herpes zoster develop non-granulomatous iridocyctitis. It may be mild with few kps or may have blood tinged hypopyon due to is ischemic necrosis of iris. atropthic patches on the iris that transilluminate and causes irregular semidilated pupil is also caused due to is ischemic necrosis of iris. Such atrophic patches are not seen in herpes simplex, which is also associated with keratouveitis. There may be chroiditis as well as chorioretinitis. Secondary Glaucoma is due to associated trabeculitis and clogging of trabecular meshwork by inflammatory cells. Chronic anterior uveitis and secondary glaucoma may predispose cataract formation.

(iii) Neurological lesions may cause encephalitis and cranial nerve palsy. Commonest being third nerve but any or all the extra ocular muscles may be affected due to involvement of their nerve supply. Optic neuritis is rare complications. Post herpetic complication Post herpatic neuralgia is a common feature, fortunately it is milder and short-lived in children. Children tolerate post herpetic neuralgia better than adults. Neuro tropic keratitis, diminished corneal sensation start from very early phase of corneal involvement, generally recovers within a few months but in some cases it may last for years leading to neuro tropic keratitis requiring tarsorrhaphy. Delayed lid Changes. There may be hypo pigmentation or hyper pigmentation with scaring of the skin, trichiasis is common, there may be notching of the lid margin. Loss of sensation may last for months. Other changes. Mucus secreting conjunctivitis, corneal mucus plaque, neurotrophic perforation of cornea, recurrent episcleritis and scleritis, herpes oticus (Ramsay Hunt Syndrome)18 herpes of palate. Management Management consists of reducing pain, associated inflammation, control of virimia, prevention of secondary bacterial infection, management of uveokeratitis and its squeals. Analgesia. Children tolerate pain better, however water soluble non-steroidal anti flamatory drugs helps to over come pain, edema and inflammation. Antiviral. Antiviral drugs reduce lid inflammation pain and shorten duration of disease but does not affect keratitis. Its role in zoster uveitis is not established. It is claimed to reduce post herpetic neuralgia. Systemic steroids. Definitely reduce pain inflammation and scaring. However it should be given along with systemic antiviral. Local steroids are required for uveo keratitis, the eyes receiving local steroids should be stained frequently, least secondary bacterial infection gets an upper hand in an already anaesthetic cornea. Bland lotion or local antiviral ointment is prescribed for lid vesicle. Local antiviral drops or ointments are given along with local steroid in uveo keralitis.

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(e) Herpes simplex25. Herpes simplex infection is very common infection caused by virus: Herpes hominis. There are two types of herpes simplex i.e. type one that involve the body above the waist and type two that cause lesions below the waist more commonly known as genital herpes, which is a sexually transmitted disease. Herpes simplex can occur as: Neonatal infection, which is due to type two herpes simplex virus transmitted via birth canal of infected mother. If it is known that the mother has genital herpes simplex it is better to deliver the child by caesarean section. Neo natal herpes simplex may present as few vesicles on the lid or conjunctivitis in the neonate. Neonatal herpes simplex is life threatening condition when server. Primary ocular infection: commonest age to get ocular infection is between six months to six years. The lids develop cluster of vesicles without predilection for any particular nerve. The vesicles may be bilateral in contrast to zoster, which is unilateral. The vesicles rapidly develop crust and disappear without scaring. It may be associated with acute folicular conjunctivitis. Half the patients develop harpes simplex keratitis in the form of fine or coarse epithelial keratitis most of the corneal lesions heal without scar. Sometimes it may progress to disciform keratits. Recurrent ocular infection. After primary infection the virus travels to the trigeminal ganglion and lies dormant unless a triger mechanisation stimulates the virus to reach the target organ after a latent period that varies from person to person. That may be few months to years Recurrent herpes simplex involves cornea and uvea and not the lids. Sever form of herpes simplex requires pediatric consultation for management. 2. Bacterial infection of the skin of the lid Boil, cellulitis and abscess of lids. Skin of the lid, like any other exposed part of the body, is prone to get infected. It may start in the hair follicle and result as a boil or may be extensive to cause cellulitis. As the skin of the lid is thin, very lax and very vascular, lots of exudates and pus can accumulate under this and ultimately result in abscess formation. Commonest organism is staphylococcus. Haemophilus is an important cause of lid abscess in children. Lid abscess starts as diffuse swelling of the lid. Initially the lid is tense and red. It may obliterate the inter palpebral fissure. Gradually the skin becomes lax and fluctuation develops, pus may point at a dependent part. It is more common in hot seasons, cellulits lid and lid abscess are preseptal hence the globe is neither involved nor proptosis is produced. Differential diagnosis consists of cavernous sinus thrombosis, retrobulbar haemorrhage retro ocular mass, rhabdomyosarcoma, multiple styes, infected chalazion, pre-eruptive stage of herpes zoster. Treatment consists of 1. Systemic broad-spectrum antibiotic preferably by injection. 2. Hot fomation. 3. Analgesic, anti-inflammatory drugs to reduce pain and inflammation. 4. If pus points or fluctuation develops, pus should be drained by a stab incision.

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TUMOURS OF LID IN PAEDIATRIC AGE GROUP26,27,28


Tumours of the lid in all ages could be both congenital as well as acquired; they can be benign or malignant. All lid tumours are difficult to manage satisfactorily. They have been classified as A. Those involving superficial layers of lid. B. Those involving deeper layers of lid. Other classification is according to structure of the lid (in all ages). A. Epithelial 1. Benign (a) Papilloma. (b) Squamous cell hyperplasia. (c) Pseudo cancerous. (d) Kerato acanthoma. (e) Seborrhic keratosis. (f) Inverted follicular keratosis. 2. Precancerous (a) Active keratosis (b) Intra epithelial epithelioma. (c) XERODERMA PIGMENTOSUM. 3. Malignant (a) Basal cell carcinoma (b) Squamous carcinoma B. Melanocytic 1. Benign 2. Malignant C. Tumours arising from glands of lid Meibomian cell carcinoma. D. Vascular 1. CAPILLARY HAEMANGIOMA 2. NAEVUS FLAMMEUS 3. CAVERNOUS HAEMANGIOMA 4. STURGE-WEBER SYNDROME E. Neural tumours : NEUROFIBROMATOSIS F. Others 1. LYMPHANGIOMA 2. JUVENILE XATHOGRANULOMA

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Condition in capital letters mentioned above are found under 15 years of age and continue to persist in later age group if not treated. All the other tumours are not seen in children. Some of them may be congenital others develop at puberty. Some are self-limiting. Except for neurofibroma and xeroderma pigmentosa none show malignant changes in children.

PHACOMATOSIS29,30
Phakomas are a group of tumours, which are congenital in nature, may be present at birth or manifest later. They have strong hereditary predisposition some of them may be sporadic. The sporadic cases can pass the gene to the next generation, they may be localised to the lid or may involve orbit, or globe, may have intra cranial extension or systemic involvement. All of them are Hamartomas i.e. tumours arising from the tissue components that are normally found at the involved site. They are seen in all races and equal in boys and girls. In fact they are congenital anomalies that result in tumour like malformations. PHAKOMAS A. Neurobibromatosis (von Reckling Hausens disease) B. Encephalo facial angiomatosis (Sturge-Weber syndrome) C. Angio matosis retinae (von Hippel-Lindus disease) D. Tuberous sclerosis (Bournvilles disease) E. Arteriovenous malformation of retina and brain (Wybern Mason syndrome) F. Ataxia talengectasia (Louis Bar Syndrome) G. Cavernous haemangioma of retina, skin brain (Rendu-Osler-Weber syndrome) A. Neurofibromatosis Neurofibromatosis is one of the most common congenital phacomas of the lids. It may be as common as one in every three thousand live births. It can be inherited as autosomal dominant trait. Either of the parents or both may show clinical evidence of disorder or may have subtle changes. Spontaneous mutation may lead to sporadic cases. These sporadic cases may pass the gene to the next generation. Genetically and clinically there are two types of neurofibroma i.e. type I and II commonly known as NF I and NF II. NF II cause bilateral acoustic neuroma besides usual features of neurofibroma. The following components may be seen alone or in various combinations, in neurofibromatosis 1. Skin and lids changes 2. Ocular changes other than lid changes 3. Involvement of nervous system 4. Visceral changes. 1. Skin and lid changes. The changes may be present at birth but generally develop by five years of age. They become marked at puberty. The changes may be seen any where on the body as : (a) Cafaulait spot. These are slightly raised hyper pigmented spots of variable size i.e. pin point to large areas of pigmentation, without sensory loss, they may cross over the midline six hyper pigmented spots or more than five millimetres is significant.

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(b) Fibroma molluscum. These are pedunculated mass of various sizes any where on the skin including lid. (c) Plexiform neuro fibroma. These are most disfiguring growths that are due to involvement of multiple superficial nerves which on palpation feel like a bag of worms. It is most commonly seen in the lid, the whole of the lid is involved and thickened including conjunctiva, the inter palpebral fissure is obliterated initially the lid acquires a S shaped Curve gradually the lid margin develops a convexity down wards, the margin may over hang the lower lid. It may be large enough to be called elephantiasis neuro fibromatosa. There may be hypertrophy of the skin of the face on rare occasion the condition may be bilateral. 2. Ocular involvement. Both neuro ectodermal as well as mesodermal structure are affected. In fact all parts of the eye except the lens may be involved. (a) Conjunctiva. Palpebral conjunctiva may be involved as part of involvement of the upper lid. The bulbar conjunctiva and lower formix may show small nodules between 1mm to 3mm. These are firm non-tender nodules. Otherwise multiple tortuous nerves may be visible on the conjunctiva. (b) Cornea. Prominent corneal nerves may be visible on bio microscopy. (c) Uvea. Commonly involved uveal tissue is iris that may have multiple hyper-pigmented nodules similar to nevi. Chloroid and ciliary body may show localised thickening of the nerve fibers. (d) Optic nerve. Congenital opaque nerve fibers are more common in persons who have evidence of neurofibromatosis. One fifth of optic gliomas are seen in neurofibroma. Neurofibroma may extend into the chiasma and have positive x-ray finding of pre-chiasmal bony destruction. (e) Retina is less commonly involved. (f) Glaucoma is very common with neurofibroma of the lid. Exact mechanism of glaucoma is not well understood. It is termed as associated congenital glaucoma, which has all the features of buphthalmos that may be obscured by pseudo ptoisis. The pathology may be due to presence of neuro fibroma at the angle, fibro vascular closure of angle or due to forward displacement of lens iris diaphragm due to growth of ciliary body. Treatment of glaucoma in neurofibroma is unsatisfactory. (g) Orbit. Bony faults in the sphenoid may lead to pulsating exophthalmos. Otherwise there may be proptosis due to retro bulbar mass with enlargement of orbit. 3. Nervous system involvement. Generally there are multiple neurofibromatous growths in the brain, meningies, cranial nerves specially bilateral acoustic neuroma. Spinal nerves may be involved with involvement of spinal cord. There may be changes in the bony spine. Even autonomic nervous system may be involved. 4. Visceral involvement. Any of the viscera can be involved in neurofibroma. Pheochromocytoma has been reported more commonly in neurofibroma. There may be gonadal changes. Management There is no known treatment to eradicate neurofibromatosis. Treatment is mostly symptomatic minor defects can be treated with plastic reconstruction. If possible amblyopia should

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be treated. Glaucoma may be managed medically or surgically. B. Encephalo trigeminal syndrome (Sturge-Weber syndrome) This congenital hamartomous anomaly differs form other phacomatoses by not being hereditary. It is seen in all races, both boys and girls are equally affected. It is generally unilateral but in ten percent of cases it can be bilateral. It has following components. 1. Skin and lid involvement. 2. Ocular involvement. 3. Central nervous involvement. 4. Visceral and other involvement. 1. Skin involvement is present at birth as port wine stain. The facial angiomatosis that frequently involves both lids may be localised or have extensive involvement of not only facial skin but also the trunk. The size and shape of the cutaneous angioma does not change with age. Some times only dilated conjunctival vessels can be seen in the epsilateral side. Generally there is hemiatrophy of the face on the affected side. Sturge Weber syndrome does not cause ptosis which is a frequent feature of neurofibroma. 2. Ocular involvement. Besides lid, the globe too has angiomatous malformation mostly in the uvea that result into heterochromia of iris, choroidal haemangioma and associated glaucoma. The choroidal haemangioma is generally single, large and situated in the posterior pole on the temporal side of the disc. The haemanagiomas have diffuse out line, they are raised, have orange hue may be mistaken as amelanotic melanoma of choriod. The hemangioma due to its proximity to macula may cause cystoid macular edema. There may be non rhegmatogenous retinal detachment that may lead to secondary glaucoma in an eye that is already predisposed to glaucoma. Glaucoma About 30% of eyes in Sturge Weber syndrome develop glaucoma by second year. A clinical feature of glaucoma is similar to congenital buphthalmos. Including enlargement of globe, large cornea, normal or deep A.C. Stretching of the globe may sub luxate the lens. The most widely accepted theory of raised intra ocular tension is increased episcleral pressure. Other probable causes are malformation of angle, peripheral anterior synechea. Sturge Weber syndrome glaucoma can be controlled by medical treatment, however if the medicines fail to reduce pressure below episcleral pressure these patients reqire surgical treatment. Trabeculectomy may help. Common complications of glaucoma surgery are intra operative hyphaema and large chroidal effusion in post-operative period. 3. Central nervous system involvement. Intra cranial haemangioma of the tapeto meninges on the same side as that of facial angioma is a constant feature. The angiomas and underlying cortex develop calcification that shows up as tram track appearance on x-ray of the skull. Intra cranial calcification develops in half of the cases by second year of life. The angioma cause Jacksonian epileptoform attack on the contra lateral side, which is difficult of treat medically. There may be contra lateral hemipersis. Various degree of mental retardation is also known to take place. There may be atrophy of ipsilateral cerebral and cerebellar cortex. Homonymous hemianopia is frequent. C. Angiomatosis retina

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Angiomatosis of retina is a hereditary hamartomatous disorder of retinal vasculature and optic nerve. It is associated with haemangioblastoma of central nervous system and viscera. In the eye it involves retina mostly and optic nerve head less commonly. It is a autosomal dominant disorder, other parts of the globe orbit or adnexa are not involved. Ocular Involvement. Retinal involvement starts first, it is generally diagnosed in first decade either due to diminished vision or on routine examination. The angiomas are generally multiple, of different sizes. There are mainly three locations where these angiomas are seen, peripheral, peripapillary and papillary. The angiomas are progressive and keep on increasing in size and number. Even after treatment recurrence in the same quadrant is possible. The peripheral lesion arise as endophytum i.e. they arise from inner retinal layer while peripapillary lesions arise from outer retinal layers and are diffuse. The early angiomas are small but have evidence of arterio venous shunt on fluorescein angiography. Larger angiomas are round elevated bright red mass that have a feeding artery and draining vein both are dilated, tortuous and of same caliber. The disc angiomas are similar but do not develop arterio venous shunt. As the angiomas grow. sub retinal fluid and exudates accumulate round the lesion. The exudate may accumulate under the macula forming a macular star and loss of central vision. The endophytic tumours may bleed leading to vitreous band formation and traction detachment. The optic nerve angioimas when situated in the substance of the optic nerve head may present as disc swelling. Recurrent haemorrhage, rhegmatogenous retinal detachment may lead to secondary glaucoma. Systemic involvement. Central nervous system involvement occurs after ocular symptoms. Almost all parts of the brain and spinal cord may develop haemangioblastomas. Similar growth can be seen in liver, kidney, and pancreas. All children with suspected angiomatosis retinae should undergo complete systemic examination including ultra sonography C.T. scan and MRI. Management Treatment consists of obliterating the retinal angiomas as early as possible. This can be achieved by argon laser, cryo or penetrating diathermy. Argon laser is better for posterior polar lesion while cryo is more effective in peripheral lesions. Argon laser therapy should have large spot, low intensity and long duration; multiple treatment sessions may be required. D. Tuberous sclerosis (Bournvilles disease) This is a congenital hamartoma that differs from neurofibromatosis and Sturge Weber syndrome. It does not involve any ocular structure except retina and optic nerve. It is a fatal condition. The children do not live beyond second decade. It is a hereditary condition all races and both sexes are equally affected. The ocular lesions are bilateral may be a symmetrical the components are : 1. Cutaneous, 2. Cerebral, 3. Ocular, 4. Visceral. The Cutaneous involvement. The Cutaneous involvement is not present at birth but

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becomes visible between two to five years of age as ash-leaf area of depigmented patches on the trunk and limbs best seen by Woods ultra violet lamp. This is followed by so called adenoma sebaceum, that does not involve sebaceous glands, are in fact angiofibromas histologically. Adenoma sebaceum are distributed on the face as wings of butterfly on either side of the mid line beyond naso labial fold and nose they are multiple small redish brown nodules. The nodules increase size at puberty, dilated vessels are common over the nodules. Central nervous involvement. Central nervous involvement is wide spread in the brain. It may be cerebral; cerebellar or medullary may be seen in ventricles. It may involve the spinal cord. In fact any part of the brain and spinal cord may be involved. They may cause raised intracranial tension, convulsion and mental retardation. The lesions get calcified and are visible on x-ray. Ocular involvement. Ocular involvement consists of hamartoma of retina that may be single or multiple generally raised and globular or may be flat. Optic nerve changes are not hemartomatous they are due to drusen that is very common with tuberous sclerosis. Visceral Involvement Rounded hamartomas can be seen in almost all organs including heart, which may be the cause of death. Fibromas may be seen under or by the side of the nails. There is no known treatment for this condition.

PRE CANCEROUS EPITHELIAL LID TUMOUR IN CHILDREN


Xerodermapigmentosum31,32,35 Xeroderma pigmentosum is relatively rare precancerous disorder of ectodermal and mesodermal structure with prominent extra ocular involvement. Intraocular structures are not involved. It is an autosomal recessive disease where both the sexes are involved equally. It may affect siblings. The disease is first noticed by second year of life when the child shows signs of intolerance to sunlight. It is due to hypersensitivity to ultraviolet light between 320 nm to 340 nm. The cutaneous signs are : Erythema in all exposed parts of the skin. The skin lesion undergo a chain of changes i.e. erythema bullae formation, development of fine vessels on skin lesions, atrophy of the lid, benign multiple hyperpigmented growth ending in development of various types of diffuse malignant growths that may cause death in adolescence. Various types of malignanies noted are epithelioma, basal cell carcinoma, even malignant melanoma. Ocular structures commonly involved are: lids, conjunctiva and cornea. Lids contain various stages of growth ranging from erythema, hyperpigmented nodules to malignant growth. There is atrophy of skin of the lid; ectropion of lower lid is common. The Conjunctival involvements include nodular growths near limbus, pigmented patches, papillomas dryness of conjunctiva, formation of pterygia and symblepharon. Both eyes are involved, bulbar conjunctiva in the inter palpebral fissure is first to be effected as it is exposed to ultra violet rays more than other parts of the conjunctiva. Corneal involvement is early and may be first to draw attention of the parents due to

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photophobia, lacrimation and redness of the eyes. Corneal involvement may start as early as two years in severe cases, or may be delayed by another two to three years. Whole of the cornea may be involved. Corneal involvement starts as superficial vascularisation of lower part causing intense lacrimation and photophobia. The vessels regress leaving a permanent opacity. Gradually whole of the cornea may become opaque. Other form of corneal involvement is extension of conjunctival neoplasm over the cornea. Management of xerodermapigmentosum is almost hopeless due to high mortality. There is no way to prevent progress of disease. Treatment is palliative aimed to give maximal relief from sensitivity to ultra violet rays. Parents are instructed to keep the children away from direct sun-rays. All the parts of the body are covered by long sleeved shirts and trousers, cover the feet by fully covered shoes, keep long hair, use sun glasses with side protection. Skin protective lotion containing zinc oxide, titanium oxide and para-amino benzoic acid are smeared over the exposed parts. Malignant growths require separate, specialised treatment.

HAEMANGIOMAS OF THE LID


Haemangiomas of the lid are commonest benign tumours of the lid. They are of three types : A. Capillary haemangioma B. Cavernous haemangioma C. Nevusflammeus A. Capillary Haemanagioma34,35 Capillary haemangiomas are commonest types of congenital tumours of the lid, they are seen one in every 200 live births. They may be present at birth, as a red spot on the lid and extend on the forehead generally in the mid line the lesions become prominent when the child cries. The growths are more common on upper lid on the medial side than lower lid. They have a typical evolution. They become manifest most commonly after first month and keep on progressing for six months to one year and then start regressing by third year. Only few will remain visible after five years, they appear as strawberries of irregular raised mass that do not have formed capsule on histology. As most of the tumours regress spontaneously, so they do not require any treatment except reassurance to the parents who themselves may had such lesions in their infancy. If the growth is large it may cause mechanical ptosis producing deprivation amblyopia. The growth by itself can cause astigmatism, anisometropia and result in amblyopia. If the growth is large it may require treatment. Medical treatment consists of oral prednisone 2mg / kg / day, better given in single dose alternate day, most children show significant regression within ten days, then the dose can be tapered. However in some cases reduction in dose may halt regression. Whenever the child is on systemic steroid its benefit should be weighed against side effects. Injection of depot steroid in the lesion is claimed to be as effective as oral treatment but less toxic. Other modes of management

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Laser. various types of lasers i.e. argon NdYAG and carbon dioxide laser have been tried without uniform result. Irradiation. Ortho voltage radiation of 200 rads may regress the growth in two weeks. However proper protective shields should be used during radiation to protect the globe and periorbital tissue. Surgery. Various types of surgical procedures are available but may not be very effective. B. Cavernous Haemangioma Cavernous haemangiomas are far less common than capillary haemangiomas in children. They are mostly orbital, single and well capsulated, are more common in females, that manifest at later years of adolescence. They do not have prominent arterial supply which is present in capillary haemangioma. It too has tendency for spontaneous regression. It may get calcified. C. Nevusflammeus This is flat cutaneous lesion of the lid that does not blench on pressure. It has a purple blue hue hence the name port wine stain. It may be seen separately or in association with Sturge Weber syndrome. It does not require any treatment except cosmetic in fair skinned children when it is too large.

JUVENILE XANTHOGRANULOMA
This dermatological condition of infancy and childhood is of importance not for its involvement of lid or skin but it causes spontaneous uniocular recurrent hyphaema due to involvement of iris. The skin lesions are multiple yellowish, benign tumours that show spontaneous regression. Intra ocular lesions are seen in iris and ciliary body, as pigmented single lesion or may have a salmon colour. Hyphaema is self-limiting but may cause secondary glaucoma.

THE NEUROLOGICAL DISORDERS OF LID


The two most common neurological disorders of lids in children are A. Neurological ptosis See Chapter 4, Page 73-76. B. Lagophthalmos B. Lagophthalmos36 Inability to close the lids fully is called lagophthalmos, in an attempt to close the lids the eye ball rolls up due to protective mechanism of Bells phenomenon. Lagophthalmos can be due to 1. Neurogenic cause or 2. Mechanical cause. 1. Neurogenic. Neurogenic cause of lagophthalmos is paralysis of motor supply to orbicularis oculi. The lesion is mostly peripheral in the facial canal or its peripheral branches.

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Supranuclear lesion of seventh nerve produces total contralateral weakness of lower two third of the face with slight weakness of orbicularis. Weakness of orbicularis in supra nuclear seventh nerve palsy may be so mild as to go unnoticed. It does not require tarsorrhaphy, which is frequently required for peripheral seventh nerve lesion. Neurogenic lagophthalmos is generally unilateral but can be bilateral, Facial diplegia37 is almost always central in origin, common cause in children are head injury, Mobius syndrome that consists of bilateral sixth and seventh nerve palsy, palatal or lingual palsy, deafness, syndactyly, polydactyly, absent fingers and toes, Vestibular nystagmus that can not be elicited by caloric test. Rosenthal Malcarson37 syndrome is a rare condition where there is bilateral infra nuclear facial palsy with furrowed tongue. Leprosy is a common cause of bilateral lagophthalmos in adults. It takes minimum 10-15 year for leprotic lagophthalmos to develop hence it is not seen in children. 2. Mechanical causes of lagophthalmos prevent the lids to close over the globe, even when lid closure is achieved the lids gradually separate to expose the anterior part of the globe. The mechanical cause may be in the orbit or in the lids. The orbital causes are: Proptosis and exophthalmos. Buphthalmos, anterior staphyloma or large ciliary staphylomas do not obstruct closure. Palpebral causes are Large coloboma of the lids, mostly upper lid, cicatricial ectropion of the lids, they are mostly seen following chemical and thermal burns of the lids. Symptoms of lagophthalmos are watering, inability to close the eye, redness of the eye, pain, photophobia. Two mechanisms are involved in watering of the eye in lagophthalmos. 1. Improper closure of the lid, ectropion of the lower lid, early tear film break-up all in combination or alone produce epiphora. 2. Reflex watering is produced due to exposure of the cornea and conjunctiva that result in corneal abrasion and ulceration. which in turn leads to circumciliary congestion. The cause of redness of the eye in is exposure conjunctivitis and circumciliary especially in the lower lid. Signs of lagophthalmos consist of (a) Loss of horizontal folds in the fore head. (b) Flattening of the eyebrow. (c) Widening of the interpalpebral fissure. (d) Weakness of the orbicularis. (e) Ectropion of the lower lid. Initially the inner margin just fails to remain in contact with the globe later it gets everted. If the evertion is much the tarsal conjunctiva also gets everted and becomes keratanised, dry and ulcerated. (f) Conjunctival congestion. (g) Exposure keratitis. Corneal sensation remains normal so long trigeminal is not involved. Once there is loss of sensation the condition becomes grave. The child without corneal sensation does not com-

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plain of pain and watering which he would have done in presence of sensation. This may lead to ulceration, perforation and blindness. Management Management of lagophthalmos depends on severity and duration. Lagophthalmos of mild degree and short duration does not require much attention except to keep the cornea protected by bland lubricant or artificial tear. Antibiotic ointments are given during sleep. Bells palsy is produced secondary to edema of the facial nerve in the facial canal due to uncertain cause is treated by systemic steroids. In case of corneal ulcer, hypopyon tarsorrhaphy is recommended to save the cornea from perforating.

DISORDERS OF LIDMARGIN IN CHILDREN


Important disorders of lid margin in children consist of: A. Congenital 1. Coloboma of the lid margin. 2. Ankyloblepharon 3. Trichiasis 4. Distichiasis 5. Entropion B. Inflammatory 1. Blepharitis 2. Stye 3. Chalazion C. Post inflammatory and traumatic 1. Entropion 2. Ectropion 3. Trichiasis Most of the conditions mentioned above except entropion, ectropion and trichiasis has already been discussed. Entropion. Entropion in children is rare; a congenital entropion may be associated with epicanthic fold. In infants patching of the eye may lead to entropion of short duration that passes off with removal of pad. Cicatricial entropian is seen in children following acid alkali burn or post-inflammatory conditions like Stevens Johnson syndrome membranous conjunctivitis. Children do not develop trachomatous entropion because it takes minimum 1520 years to develop trachomatous entropion. Ectropion. Congenital ectropion is rarer than congenital entropion. Ectropion in chil-

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dren are mostly traumatic or post inflammatory. The infection in such cases are seen on the skin surface rarely it is seen in facial palsy and in acid in alkali burns.

REFERENCE
1. Duke Elder. S. and Wyber K.C. ; System of ophthalmology Vol. II p-503-537 1st edition, Henry Kimpton, London 1961. 2. Nema H.V. ; The lids in Anatomy of the eye and its adnexa 1st edition, p-61-67, Jay Pee Brothers, New Delhi 1991. 3. Banumathy S.P. ; Eye lids in Anatomy of the Eye p-35-43, edited by G. Natchiar, Arvind Eye Hospital & Postgraduate Institute of Ophthalmology, Madurai. 4. Ahmed E. ; Anatomy of the eyelids in A Text book of ophthalmology, 1st edition p-9-12, Oxford University Press, Calcutta 1993. 5. Buftam F.B. ; Lacrimal diseases in Text book of Ophthalmology, Vol. IV, p-7.5, edited by Podos S.M., Yanoff M., Gower Medical Publication, London 1993. 6. Scheie H.G. and Albert D.M. ; Anatomy of the human eye in Text Book of Ophthalmology 9th edition p-50, W.B. Saunders Comp., Philadelphia 1977. 7. Vaughan. D. and Asbury. T. ; Lids and lacrimal apparatus in General Ophthalmology 10th edition p-43-44, Lange Medical Publication, California 1983. 8. Dutta. L.C. ; Disease of eye lids in Ophthalmology Principle and practice 1st edition p-5 Current Books International Calcutta 1995. 9. Dutta L.C. ; Disease of the orbit in Ophthalmology Principle and practice , 1st edition p-331, Current Books International, Calcutta 1995. 10. Khamar B, Mayuri Khamar Trivedi N. and Usha Vyas ; Inflammatory conditions of the lids in Modern Ophthalmology, vol. I, 2nd edition p-4-6, edited by Dutta L.C., Jay Pee Brothers, New Delhi 2000. 11. Dougherty J.M. and McCulley J.P. ; Comparative bacteriology of chronic blephariti B.J.O. 68:524-528 1984. 12. McInnes J., Donna Brown, McCulley J.P. ; Blepharo Conjunctivitis in Current ocular therapy 5th edition p-421-422, edited by Fraunfelder F.T. and Roy FH, W.B. Saunders Company, Philadelphia 2000. 13. Held K.S. ; Blepharitis in Decision making in ophthalmology, first Indian edition, edited by vanHeuven WAJ and Zwann J.T. p-24, Harcourt Brace 1998. 14. Kincaid Marilyn. C. ; Pediculosis and phthiriasis in Current Ocular Therapy 5th edition p-100, edited by Fraunfelder F.M. and Roy F.H., W.B Saunders Comp., Philadelphia 2000. 15. Awan, K.J. ; Cryo therapy in phthiriasis palpebrum Am. J. Oph 83: 906-907 1997. 16. Mukherjee P.K. Jain P.C., Mishra R.K. Exanthemata : A caus of chronic dacryo cystitis

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in children I.J. Oph 17, 27-30 1969. 17. Dekker N. W.H.M. ; Rubeola in Current ocular therapy edited by Fraunfelder F.M. and Roy F.H. p-69, W.B. Saunders Comp., Phildelphia 2000. 18. Marsh R.J. ; Vericella and herpeszoster in Current Ocular therapy 5th Edition, edited by Fraunfelder F.M. and Roy F.H. p-83-82, W.B. Saunders Company Philadelphia 2000. 19. McDermott M.L. ; Corneal Hypesthesia in Decision making in ophthalmology, First Indian edition, Edited by vanHeuven W.A.J. and Zwann. T., p-174, Harcourt Brace 1998. 20. Gittenger J.W. ; Lids and Adenexa in Manual of Clinical Problems in Ophthalmology, Edited by Gittinger J.W. and Asdourian G.K., 1st edition p-5-7 Little Brown and Co., Boston 1998. 21. Dua H.S. and Nilawar D.V. ; Non-surgical therapy of chalazion Am. J. Opl. 94: 424425 1982. 22. Viduarri. U. Jacob.P. ; Inter-lesional Corticosteriod treatment of chalazia. Ann. Oph. 18-339-340 1986. 23. Dekker N.W.H.M. ; Cornea is measles Doc. Oph. 52: 1-120 1981. 24. WHO ; The Child Measles and the eyes, Geneva 1993. 25. Gittenger J.W. ; Cornea and Sclera in Manual of clinical problem in ophthalmology edited by Guttinger J.N. and Asduria G.K. 1st Edition p-46-47, Little Brown and Co., Boston 1998. 26. Ratnakar K.S. ; Pathology of eye lids and adnexa in Pathology the Eye and orbit, 1st edition p-175-187, Jay Pee Brothers, New Delhi 1997. 27. Green C.M. and Ashton N.A. ; Melanosis, naevi and melanomas of the Conjunctiva and lids in Ocular Pathology 1st edition p-85-101, Blackwell Scientific Publication, Oxford 1963. 28. Ahmed E. ; Disease of lids in A Text Book of Ophthalmology 1st edition, Oxford University Press, Calcutta 1993. 29. Boger W.P. and Peterson R.A. ; Paediatric Ophthalmology in Manual of ocular diagnosis and therapy 3rd edition p-274-276 edited by Deborah Pavan Langston Little Brown. 30. Kanski J.J. ; Glacucoma in Clinical Ophthalmology, 2nd edition p-228-230, Buther Worth, London 1989. 31. Kraemer K.H., Lee M. M., Scotto. J. ; Xeroderma Pigmentosum Cutaneous, ocular and neurological abnormalities, in 830 published cases, Arch dermt 123: 241-250 1987. 32. Rao V.A. Srinivasn R., Agrawal K. ; Oculocutaneous manifestation of xeroderma pigmentosum, Ophthal 78: 295-297 1994. 33. Freedman J. ; Xeroderma Pigmentosum in current ocular therapy 5th edition p-136-137, edited by Fraun felder F.T. and Roy H.F., W.B. Saunders Comp. Philadelphia 2000.

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34. Haik.B.G., Karcioglu Z.A., Gordon R.A., Pechous B.P. ; Capillary Hemangioma, Surv. Oph. 38: 339-426 1994. 35. DeVenecia G., Lobee C.C. ; Successful treatment of eye lid hemangioma with prednisone, Archoph 94: 98-102 1970. 36. Jobe Richard. P. ; Lagophthalmos in Current Ocular Therapy 5th edition p-436-438, Edited by Fraunfelder F. T. and Roy H.F., W.B. Saunders Comp. Philadelphia 2000. 37. Mukherjee P.K. and Dongre R.C. ; A Case of acquired facial diplegia, macular oedema and lingua plicata (Melkerson-Rosenthal syndrome) Ind. Jr. Oph. 21-36-39 1973. 38. Duke Elder S. ; Anomalies of the eye lids in System of Ophthalmology Vol. III, part II, 1st edition p-827-871, Henry Kimpton, London 1964. 39. Shaffer D.B. ; Abnormalities of Lid and Lacrimal apparatus in Text Ophthalmology, 9th edition p-287-293, edited by Scheie G.H and Albert D.M., WB Saunders Comp. Philadelphia 1977. 40. Shannon G.M. Flanagan J.C. and Saunders D.H. ; Disorder of the lid in Paediatric ophthalmology, Vol. II, 2nd edition p-412-419, edited by Harley R.D., W.B. Saunders Company, Philadelphia 1983. 41. Nema H.V. ; Congenital Anomalies of eye and its adnexa in Anatomy of the eye and its adnexa, 2nd edition p-162-165, Jay Pee Brothers, New Delhi 1991. 42. Daily R.A. ; Epicanthus in current ocular therapy, 5th edition p-430-431, Edited by Fraunfelder F.T. and Roy F.M., W.B. Saunders Company Philadelphia 2002.

CHAPTER

Motility Disorders of the Lids


Main function of the lids is to protect the globe from external trauma. This is brought about by various reflexes that shut the eye behind, by an almost impermeable wall of lids. However for the light to reach the eye the lids have to keep a clear area in front of the pupil in the form of inter palpebral fissure. There is a delicate balance between keeping the eyes open and closing it. There are three muscles in the lid that control the movements of the lids. Two of them : the levator palpebral superior and orbicularis are striated muscle supplied by third and seventh cranial nerve respectively with opposite actions. The third muscle the Mullers muscle is plane muscle supplied by cervical sympathetic. The levator palpebral superior is the main elevator of the upper lid and the Mullers muscle is the accessory elevator of the lid. There is no well formed depressor of the lower lid however in paralysis of Mullers muscle the lower lid is raised from its normal position upwards. The orbicularis is the muscle that closes the lid. It is directly antagonist of levator palpebral. In life a delicate balance exists between the two muscles and the eyes can be closed or opened at will and reflexly. The motility disorders of the lids are1 1. That narrow the IPA. 2. That widen the IPA 3. Associated with synkinetic movement of lids. The first group consists of ptosis and blepharospasm, the later group consists of lid retraction and lagophthalmos. Ptosis and lid retractions are not vision threatening but lagophthalmos is potentially vision threatening. The third group may be associated with narrowing or widening of IPA. Ptosis1,2,3,8 Normal upper lid covers 2 mm of the upper cornea. If it droops from this position, it is called blepharoptosis or simply ptosis. 67

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Ptosis in children is mostly congenital in origin and less commonly acquired. In adults the incidence of congenital to acquired ptosis is reversed. Ptosis in children have two pressing factors for management i.e. cosmetic blemish and possibility of developments of amblyopia. However acquired ptosis in children have an added factor of systemic neurological involvement that may be crippling, blinding even life threatening. It is of utmost importance that all drooping upper lids do not constitute true ptosis. There are many condition of the lid, globe or orbit that may mimic ptosis and are grouped as pseudo ptosis. Pseudo ptosis can be mechanical when a growth or edema of the upper lid causes narrowing of the interpalpebral fissure. Otherwise the lid may be normal in the function and appearance but it seems to droop due to change in shape, size and position of the eyeball in relation to orbit. Occasionally the lid may be normal with normal inter palpebral aperture but seems to be drooping because the contra lateral eye has a wider inter palpebral fissure which is considered to be normal. Causes of pseudo ptosis can be 1. Lid Edema of lid Growth of lid Hemangioma Neurofibroma Sturge Weber Syndrome Inflammation Large chalazion Tarsal cyst Lid abscess Microphthalmos Microcornea Phthisis Atrophic eye Enophthalmos Anophthalmos Enucleated and eviscerated sockets Fracture floor of orbit Duanes retraction syndrome Hypotropia of contra lateral side Hypertropia of ipsilateral side

2. Globe

3. Orbit 4. Miscellaneous

Classification of ptosis1,2,4 Traditionally ptosis has been divided into two groups: 1. Congenital ptosis 2. Acquired ptosis Other widely used classification is based on anatomical structures involved 1. Congenital (i) Simple congenital - only levator muscle is involved.

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(ii) Complicated congenital ptosis (a) Other muscles along with LPS are involved. (b) Third nerve or sympathetic nerve involved. (c) Along with other congenital anomalies of lids. (d) Ptosis with synkinetic movements. 2. Acquired ptosis (i)Mechanical (a) Edema lids (b) Tumours of lid (ii) Neurogenic (a) Third nerve palsy (i) Upper division (ii) Both divisions (iii) Trunk (iv) Nucleus (b) Along with other cranial nerves. (c) Cervial sympathetic involvement Horners syndrome (iii) Myogenic (a) Myasthenia (b) Muscular dystrophies (c) Chronic progressive ophthalmoplegia and its variations (iv) Aponeurotic Traumato levator aponeurosis Disinsertion of levator Dehiscence of levator (not seen in children) Laceration of IPS Fracture orbit

(v) Trauma Congenital ptosis

This is the commonest form of ptosis seen in children and lingers on to adult life if not corrected. It accounts for about sixty percent of all ptosis in childhood. There are various modes of presentation1 1. Due to congenital fault in (i) Levator alone (ii) Levator Superior rectus complex (iii) Third nerve 2. Birth trauma (i) To LPS (ii) Third nerve (iii) Cervical sympathetic

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Ptosis due to congenital fault in levator palpebral superior only (simple ptosis) This type comprises of 75%-80% of all congenital ptosis. This is also known as simple ptosis. It is inherited as autosomal dominant trait.1 It is unilateral in seventy five percent of cases. The bilateral cases are generally symmetrical. The ptosis is said to be complete when there is no action of the levator. In congenital ptosis some action of levator is always present hence most of them are called incomplete. Boys and girls are involved equally. There may be many members of the family to have congenital ptosis including the siblings. Congenital simple ptosis is non progressive and stationary unless corrected surgically. It is generally present at birth but most often missed because a newborn keeps his eyes closed for eighteen to twenty hours. Of course in cases of rare instances of severe ptosis, the parents may notice its presence at birth. Unilateral ptosis draws attention early than bilateral. The condition may be so small that it goes unnoticed unless the child looks up or develops abnormal head posture. The appearance of bilateral ptosis is characteristic. The forehead skin is thrown in horizontal furrows, the eyebrows are arched up. The head is tilted back. Head tilt and arching of the eyebrows are acquired late when the child realises that by doing so he has better vision. The inter palpebral fissure is narrow with some action of levator. The skin of the lid is smooth, unwrinkled. The tarsal fold is generally absent. The other extra ocular muscles including orbicularis are normal. Pupillary reaction and accommodation are normal. Child with unilateral ptosis may raise the eyebrow on the same side only. The forehead furrow and the head tilt are less marked in unilateral ptosis. The lid droops down over the cornea, the drooping varies in different cases, it may be mild, moderate or severe, depending upon available levator function. In a normal child if the upper lid is everted and child looks up, the lid is automatically corrected but not in congenital ptosis where most of the action of levator is absent. The vision is normal. However associated error of refraction, which is mostly astigmatism and causes subnormal vision. In severe cases the pupil may be obscured with severe loss of vision, unless the lids are lifted. The other cause of diminished vision is amblyopia, which is more often anisometropic than deprivation. Prolonged amblyopia may result in strabismus. The cause of maldevelopment of levator is not well understood. Various suggestions given are: 1. It is due to lack of peripheral differentiation or aplasia of muscle. 2. Dystrophy or degeneration of levator. 3. Unknown trauma to the levator. 4. Abnormal development of levator. 5. The striated muscle fibres are replaced by fibro fatty tissue.5 Congenital ptosis associated with defects in other extraocular muscles These are generally put under heading of complicated congenital ptosis. 1. The commonest association is involvement of superior rectus on the same side.

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During developement of the led a superior rectus and levator develop from a common mesodermal mass and are closely associated with each other until late in development. The nerve supply to the levator i.e. upper division of third nerve also supplies the superior rectus and is also likely to share common dystrophic changes. Hence involvement of both the muscles is a common feature. Involvement of superior rectus does not produce expected diplopia. It may be associated with under action of inferior oblique. There may occasionally be an elevator palsy. Bells phenomenon may be minimally affected or absent. It is also not associated with changes in pupillary reaction or accommodation. Other extraocular muscles have normal movement. There are no synkinetic movements. Presence of diplopia on lifting, the lid denotes involvement of extraocular muscles other than superior rectus alone. 2. Congenital ptosis may be associated with under action of other extra ocular muscles supplied by third nerve due to developmental anomaly like hypoplasia of the third nerve nuclei or its trunk. 3. Rarely there may be congenital anomaly of other cranial nerves supplying the extra ocular muscles. 4. Congenital ptosis associated with under action of cervical sympathetic. This is rare and mostly due to traction trauma to the brachial plexes during delivery.1 5. Rarely neonatal myasthenia may present as congenital ptosis.9 Congenital ptosis associated with other congenital anomalies of lid These consist of Ptosis Inverse epicanthic fold Blepharophimosis Telecanthus The tetrad is called blepharophimosis syndrome.6 Blepharophimosis syndrome The tetrad is an inherited disorder. All the features may not be present in all the cases all the time. 3% to 6% of children with congenital ptosis have blepharophimosis. Some may have just epicanthus inversus and ptosis. In blepharophimosis the inter palpebral fissure is shortened in length as well as width. The epicanthus inversus is a fold of skin on the medial side of the medial canthus extending from lower lid upward. In telecanthus the inter canthal distance is increased. The child keeps the head tilted back. Other anomalies found are squint, punctal displacement, coloboma of optic nerve head6. The condition is bilateral, equally seen in boys and girls. Treatment The definitive treatment is surgical correction of each deformity in various sittings. The surgical treatment can be deferred up to age of five years in absence of amblyopia. The medial canthal deformity is corrected six to twelve months before the ptosis surgery.

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Congenital ptosis associated with synkinetic movements of the lids The synkinetic movements can be 1. Jaw movement 2. Ocular movements 1. Ptosis with jaw movements is also known as Marcus Gunn jaw winking and inverse Marcus Gunn jaw winking. Jaw winking is present in four to six percent of all congenital ptosis.6 Jaw winking is more common than inverse jaw winking. The former is caused due to contraction of external pterygoid muscle and latter is due to contraction of internal pterygoid muscle. The syndrome of jaw winking is due to congenital misdirection of fifth nerve to the levator. Jaw winking is present at birth and first noticed by the mother who observes that the position of the upper lid changes when the child suckles. Later the parents notice that there is involuntary spasmodic jerky retraction of upper lid with certain movements of the lower jaw i.e. moving the jaw to contra lateral side, projecting the jaw forwards or opening the mouth. In inverse jaw winking, the lid may elevate on closing the mouth or clinching the mouth, the lid may be ptotic or may be normal. The jaw winking is generally unilateral, present at birth and remains for rest of the life. The child may learn to suppress it later to overcome embarrassment. It is also known to become less severe as the child grows. It is mostly sporadic, may be seen in several members of the family. Treatment consists of surgical correction, before that any amblyopia or anisometropia present may be corrected. Surgical treatment is definitive treatment that requires correction of two separate features - ptosis and jaw winking present simultaneously. Jaw winking when severe may be worsened by levator resection so one of the modes of surgery is to release the levator aponeurosis that worsen the ptosis which is later corrected by frontalis suspension.7 Congenital ptosis associated with abnormal ocular movements1,3,8,9 Fibres destined for medial rectus are directed to superior rectus or inferior oblique. Fibres of inferior rectus may be directed to the pupil. Hence many combination are possible. Some of the common combinations are 1. Lifting of upper lid in attempted adduction. 2. Paradoxical retraction of upper lid on the affected side in congenital paralysis of sixth nerve. 3. Raising of lid in downward gaze (Pseudo Graefe sign). 4. Lid retraction on occlusion of contra lateral eye.10 The ipsilateral eye is generally ptotic with jaw winking. Paralytic ptosis Paralytic ptosis can be due to 1. Involvement of third nerve. 2. Involvement of cervical sympathetic.

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1. Ptosis in third nerve involvement. It can either be congenital or acquired. Congenital ptosis due to third nerve palsy is less common than congenital ptosis due to levator dysplasia. Ptosis due to oculomotor palsy is far more common in adults than in children. In adults, diabetes, vascular lesions and neoplasm are more common than infection. In children infection is more frequent cause of paralytic ptosis than others. However third nerve involvement in which ptosis is an outstanding feature may be the only sign to begin with, this may be followed by serious central nervous involvement that may not only be vision threatening but crippling or life threatening. Oculomotor ptosis can be due to Nuclear, facicular, basilar and peripheral involvement. The nuclear lesions are rare. It consists of bilateral, symmetric ptosis with superior rectus under-action. May be isolated, may precede palsies of other muscles supplied by third nerve. Due to its proximity to fourth nerve nucleus, fourth nerve involvement is also common. Supra nuclear lesions cause mild ptosis with subnormal gaze palsy and miosis. Mid brain lesions are associated with signs of dorsal or ventral fascicular lesion i.e. Benedicts syndrome, and Webers syndrome. Trunk may be involved either in cavernous sinus or orbit. Treatment should be 1. Directed towards the cause. 2. Surgical intervention is deferred for about six months after ptosis has been stabilised. 3. Amblyopia is treated by standard method. Sympathetic ptosis (Horners syndrome)11,12 Sympathetic ptosis occurs due to involvement of oculosympathetic chain anywhere from hypothalmus to eye causing in a number of signs that constitute Horners syndrome. For diagnostic purposes the oculo sympathetic is divided into three anatomic part consisting of three neuron.13 1. The first neuron extends from posterior hypothalmus to cilio spinal centre of Budge between C8 and T2. 2. The second neuron extends from centre of Budge to superior cervical ganglion in the neck. 3. The third neuron spreads from superior cervical ganglion to the eye via nasociliary and long ciliary nerves. Incidence of Horners syndrome is less common in children than in adults. It can be congenital or acquired. The acquired causes in children are birth trauma mostly to the brachial plexus associated with Klumkes paralysis. Some authors believe that congenital Horners syndrome is nothing but a result of birth trauma. Besides birth trauma accidental injury to neck, enlarged lymph nodes in neck, cavernous sinus disease, inflammation in orbit can cause Horners syndrome.

74 The features of Horners syndrome consists of 1. Permanent signs and 2. Transient features The permanent features are: 1. Ptosis

PEDIATRIC OPHTHALMOLOGY

(i) Ptosis is mild, not more than 2 mm to 3 mm due to paralysis of Mullers muscle. (ii) The lid folds are retained. (iii) There is no lid lag on upward gaze. (iv) The action of LPS and superior rectus are normal. 2. Upside down ptosis of the lower lid: The retractor muscle of lower lid is also supplied by oculo sympathetic hence its paralysis results in upward shift of the lower lid. The normal lower lid that just touches the lower limbus covers the lower 1 mm of the cornea. 3. Narrowing of inter palpebral aperture: This is due to combined effect of ptosis and upside ptosis. 4. Enophthalmos: This is more of a pseudo enophthalmos than true. The eye looks small due to ptosis and upside ptosis. 5. Miosis: The pupil on the affected side is smaller by 0.5 mm to 1.00 mm and not pin point. It is best observed in dim light. The pupil does not dilate with 4% cocaine. The pupil will dilate with hydroxyamphetamine 1% (Paredrine) in pre ganglionic lesion but not in post ganglionic lesion. 6. Anhydrosis of the affected side of the face. 7. Heterochromia of the iris on the affected side. The iris on affected side is lighter. This is characteristic of congenital Horners syndrome in children.13 The transient signs 1. Dilatation of conjunctival vessels. 2. Dilated facial vessels. 3. Reduced intraocular pressure. 4. Increased accommodation. There is no change in convergence and light pupillary reaction. Differential diagnosis consists of all causes of unilateral small pupil - Congenital miosis, traumatic miosis, healed iridocyclitis. Treatment There is no known treatment for Horners syndrome. Attention should be directed to the cause of Horners syndrome, which are confirmed by 1. Pharmacological test (i) Cocaine test (ii) Paredrin test

MOTILITY DISORDERS OF THE LIDS

75

2. x-ray chest 3. CT and MRI 4. Neurological evaluation Neuro muscular (Myogenic) ptosis There are two types of myogenic ptosis: 1. Fault at myoneural junction. 2. Myopathies and muscle dystrophies. Both are more common in adult but can be seen in children and may be congenital or neonatal. 1. Fault in myoneural junction Myasthenia gravis15,16,17: Myasthenia gravis is an auto immune disorder that produces extra ocular muscle hypofunction without involving intrinsic muscle. It is of two types: 1. Ocular and 2. Systemic About 90% of patients with myasthenia have predominantly ocular involvement that may precede systemic involvement in 75% of cases, 80% of cases with ocular involvement will be gradually converted into systemic involvement in two years14,15 Chances of conversion to systemic myasthenia after two years is less common. It is not a hereditary disease but may be seen in members of the same family.3 Infants born to myasthenic mothers are prone to develop neonatal myasthenia, which may be present at birth. Myasthenia is more common in women than in men in a ratio of 2:1. However childhood myasthenia is more common in girls i.e. girls to boys ratio is 6:1.17 Myasthenia can be progressive or remittent. Myasthenia is a common cause of acquired ptosis and diplopia in all ages without other neurological involvement. It can affect any age from birth to late seventh decade but common age group is between 20-40 years. About six percent of cases may develop dysthroid oculopathy later. Exact etiology of myasthenia is not known. It is presumed to be an autoimmune disorder. Acetyl choline, which is a pharmacological transmitter at neuro muscular junction is destroyed fast by an unknown substance present at the neuro muscular junction. This unknown substance19 has curare like action that renders the muscle hypoactive and fatigued. There is reduction of acetylcholine receptors at motor end plate20,21 Use of anticholine estrase drugs rectify this process, this forms the basis of medial treatment of myasthenia. Ocular symptoms of myasthenia gravis Two main symptoms are 1. Ptosis and 2. Diplopia. There may be concurrent or one may follow one another. Ptosis: Drooping of upper lid in myasthenia is variable though it is bilateral, may begin in one eye. The other eye follows soon. In bilateral ptosis drooping is systemic. It may shift from one eye to the other eye. Drooping changes not only in days but may be in hours. It is generally minimal or absent on waking up and gradually increases during active hours becoming maximum in the evening.

76 Variability is demonstrated by following tests:

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1. Cogan lid twitch: The patient is asked to look down for 10-15 seconds and then asked to fix the eye in primary position quickly. This sends the upper lid higher than normal, only to fall to ptotic position. 2. Lid fatigue: With sustained upward gaze, the ptosis becomes more. 3. Cold test: An ice cube is applied to the ptotic eye for about two minutes. On removal of the ice, the lid is markedly elevated which falls to ptotic level in 15 seconds. 4. If the contra lateral lid is lifted mechanically there is enhanced ptosis in ipsilateral side. 5. Under action of orbicularis is almost constant feature in myasthenic ptosis. 6. Improvement of ptosis following Tensilon test. 7. Therapeutic trial by oral prostigmine and pyridostigmine shows improvement in ptosis and reduction of diplopia. Diplopia Diplopia is one of the most common and sometimes first symptom experienced by the patient. It varies in type and extent. It is more often vertical than horizontal. It becomes worse in the evening. Squint Some patient may notice squint. Extraocular muscle involvement does not follow any fixed pattern. Muscle imbalance may simulate inter nuclear ophthalmoplegia or central gaze palsy.16 There may be nystagmoid movements. Extra ocular muscles to be involved other than levator is orbicularis, Mullers muscle is never affected. Patient may present with heterophoria. Commonest being exophoria. Any of the skeletal muscles can be affected. Common are the small muscles of face, laryngeal muscle, muscles of mastification, most probably due to involvement of bulbar muscles.17 Involvement of thymus is common. Myasthenia of childhood differs from adult myasthenia. There are three types of myasthenia in children 1. Transient neonatal myasthenia seen in children born to mothers who have the disease. 2. Persistent neonatal myasthenia: This is similar to transient myasthenia but mothers do not have the disease. 3. Juvenile myasthenia sets in after ten years of age. The presentation is similar to adult myasthenia. Differential diagnosis All cases of variable ptosis, diplopia with orbicularis weakness and normal pupil should be suspected to have myasthenia unless proved otherwise. The conditions that constitute differential diagnosis are: Chronic progressive external ophthalmoplegia and its variations.

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Myotonic dystrophy Inter nuclear ophthalmoplegia Dysthyroid myopathy. Diagnosis of myasthenia Diagnosis of established myasthenia is not difficult however may have to be confirmed by I. Pharmacological testTensilon test.22 1. To do this test first precaution is to have a resuscitation trolley ready and handy. 2. Inject 0.3 mg atropine I.V. (adjust dose in children). 3. Fill a syringe with 10 mg Tensilon. 4. Inject 2 mg I.V. and observe for any adverse reaction or improvement. In adverse reaction, abandon the test and consider therapeutic trial with oral prostigmine. In case of improvement in ptosis, facial expression and diplopia, the test is conclusive that may be further confirmed by injecting remaining 8 mg.23 Three types of responses are possible: 1. An improvement. 2. Worsening: Small tropia becomes large tropia. 3. Reversal: Squint shifts to the other eye. The response in 2 and 3 are not seen in true myasthenia. II. Antibody to anti actycholine is present in 90% cases. III. x-ray chest, CT and MRI of mediastinum may show thymoma. Management Myasthenia is a self limiting disease. In many children it will pass off after one or two episodes without recurrence. Others will require medical treatment and complete amelioration may be achieved and child maintained on a maintenance dose for long time. Most of children in this group will have some residual ptosis and or manifest muscle imbalance that requires surgical correction later. Surgical correction should not be done unless there is no recurrence for one year, ptosis and squint are stable for at least one year. Medical treatment Medical treatment consists of oral a anticholineesterase drugs given in divided doses. Most commonly used drug is pyridostigmine. Its effect starts with in 10-30 minutes following oral administration and lasts for three to four hours with peak in between. Hence the drug has to be repeated every four to six hours. Correct dose is adjusted empirically in consultation with pediatrician. Other drug used is prostigmine which is less effective than pyridostigmine. It takes two to three days for prostigmine to be effective. The side effects of the drug are colic, diarrhoea, nausea due to prasympathomimetic action of the drug. These are reduced if the drug is taken with food or some spasmolytic drugs are taken few minutes after the drug.

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If the patient fails to show desired therapeutic dose, oral steroid are started, initially a single daily dose is given till the desired effect has been reached, then the schedule is changed to every alternate day and gradually reduced. Other drug used in patient is refractory to anticholine esterase drugs is azathioprine. It takes few months to be effective. Greatest drawback with the drug is its hemato and hepato toxicity. Cyclosporine and cyclophosphamide are other immunosuppressive drugs used in selected cases.17 Ancillary ocular methods 1. The child learns by experience to close one eye to avoid diplopia. The eye may be patched to give same effect. 2. Prisms may be given to minimize diplopia. As diplopia is fleeting and changes direction too often many a times prisms may be of little use. 3. Ptosis crutches have limited value. Thymectomy have been claimed to give good result in refractory cases in selected few. Chronic progressive external ophthalmoplegia25 The condition may be seen in children as well as adults. Generally it manifests between second and fourth decade. It is a bilateral disease of slow progress. It is associated with mutation in mitochondrial DNA.24A In fifty percent of cases the disease is hereditary. The first sign that develops after ten years of age is gradually developing ptosis, which progresses to become complete. The ptosis is bilateral and symmetrical. Ophthalmoplegia develops years after ptosis. Involvement of muscles is ill defined. It begins as single muscle involvement resulting in diplopia. As more muscles get involved the eye becomes almost immobile and diplopia disappears. The internal ocular muscles are spared. The child throws the head back to get clearer vision and forehead shows prominent frontal creases. So long only extraocular muscles are involved, the condition is considered as ocular myopathy. If other muscles get involved it is called ophthalmoplegia plus. The condition may be associated with retinal pigment degeneration and heart block. Occasionally there may be involvement of pharyngeal muscle. There is no known specific treatment. Each disorder has to be treated individually. Evaluation of a case of ptosis for surgery Definite treatment of congenital ptosis is surgery. It is mandatory to evaluate each patient separately. Sometimes each eye may require individual evaluation. Common heads under which a ptotic child is evaluated are: 1. History. Find out if ptosis is congenital or acquired. In case of acquired ptosis attention should be diverted to find out the primary cause and its management. In case of acquired ptosis surgical intervention should be undertaken only when the ptosis has been stable for at least one year. Congenital ptosis requires more correction than acquired. However levator resection gives less correction in congenital ptosis.

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2. Examination of the eye 1. VisionVisual acquity is evaluated to diagnosis presence of amblyopia that requires energetic anti amblyopic treatment. 2. Examination of the lids (i) Exclude pseudo ptosis from true ptosis, however some of the pseudo ptosis may require surgical intervention. (ii) Presence of epicanthic fold and blepharophimosis which may require separate surgeries. (iii) Absence of orbicularis action goes more in favour of myasthenia. (iv) Presence of synkinetic movements require multiple surgeries. (v) Width of inter palpebral fissure gives a rough idea about levator function. Width of the inter palpebral fissure depends upon (i) Action of LPS present. (ii) If the action of LPS is poor, the lid will droop down. The usual method to assess position of the upper lid is to find out level of the lid margin in relation to upper limbus and comparing this with normal eye. In unilateral ptosis the other eye acts as control. In normal eye the upper lid covers upper 2 mm of the cornea in primary gaze. The average vertical diameter of cornea is 11 mm. This leaves 9 mm of cornea not covered by lid. If the upper lid droops by 2 mm from usual position i.e. 4 mm from upper limbus leaving on 7 mm of uncovered cornea, the condition is designated as 2 mm ptosis and the ptosis is considered to be mild ptosis. Similarly ptosis of 3 mm is called moderate and more than 4 mm is called severe ptosis. The choice of type of surgery to be undertaken partly depends upon degree of ptosis i.e. mild, moderate or severe and amount of levator function available. The function of levator is assessed by noting the excursion of upper lid from downward gaze to maximum up gaze. In normal eye, this is between 12 mm-15 mm. If excrusion is more than 8 mm, it is called good excrusion while fair excrusion means5-7 mm and less than 4 mm is poor. More is the excrusion better is the action of LPS, which means that the levator if strengthened surgically will eliminate ptosis. 3. Presence of Bells phenomenon. Bells phenomenon is one of the most important defence mechanisms of the eye. It is lost in paralysis of the superior rectus. In absence of Bells phenomenon, if the upper lid is raised the cornea becomes unprotected and exposed to external injuries. Absence of Bells phenomenon is an absolute contra indication for any type of ptosis surgery. 4. Exclude vertical tropia by cover test. This unmasks presence of pseudo ptosis. 5. Action of extraocular musclesUnder action of extraocular muscles result in diplopia that is generally not felt if the upper lid covers the pupil. In presence of ocular palsy, correction of ptosis will result in intractable diplopia, which may be considered worse than cosmetic ptosis by the patient.

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6. Sensation of corneaAbsence of corneal sensation is absolute contra indication for ptosis surgery. 7. Tear film statusPoor tear film status becomes worse following ptosis surgery. Lid retraction25,26 Normal upper lid covers up to 2 mm of the cornea in primary position and the upper lid follows the movement of the globe both in up and down gaze. If the upper lid does not reach the upper limbus or is so placed that a strip of sclera is visible above the cornea it is called lid retraction. This is generally associated with lid lag. Exposure of a strip of sclera below the lower limbus may be seen in case of sympathetic over activity along with upper lid retraction. Visible bare sclera above and below is seen in exophthalmos and proptosis. Lid retraction can be seen in some physiological states also specially in infants and children. 1. In infants: Transient lid retraction is common that pass off within a few weeks. There may be widening of IPA if the illumination in the room is reduced. 2. Children: Children suffering from progressive loss of vision have upper lid retraction as an effort to see better. Pharmacological causes of lid retraction 1. Phenylepherine: In normal person phenylepherine does not cause any change in lid position in relation to cornea but children with tendency towards sympathetic over action show retraction of 1 mm to 2 mm following local instillation of phenylepherine. The effect passes off with in hours. 2. Prostigmine and tensilon when given as injection cause improvement of ptosis and sometimes the lid may be retracted. 3. Infants born to mothers with hyperthyrodism show retraction for two to three weeks. 4. Prolonged use of steroids are also known to cause lid retraction.26 In unilateral ptosis the contra lateral lid may show retraction when the ipsilateral lid is forced to elevate. Pathological lid retraction Lid retraction can either be unilateral or bilateral. In unilateral cases the condition may remain unilateral or the other eye may be involved later. Bilateral lid retraction is generally symmetric. Commonest cause of lid retraction is dysthyroid myopathy due to sympathetic over action of Mullers muscle. This is enhanced by instillation of sympathomimetic drugs and reduced by instillation of sympatholytic drugs. Lid retraction is dysthyroid myopathy is generally bilateral and symmetrical. Other causes of lid retraction are sympathetic over action and dorsal mid brain lesion. Sympathetic lid retraction presents with feature opposite of sympathetic ptosis. It consists of widening of IPA, enhanced lid crease, dilated pupil, mild exophthalmos, sweating on the affected side and lowered temperature.

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Dorsal mid brain lesion is called Colliers sign that consists of bilateral lid retraction, there is conjugate up gaze paralysis of convergence, dissociation of light near reflex. There may be associated retraction nystagmus. Lids may be caught in scar of the lid following infection, burn, trauma accidental or surgical. Over correction of ptosis is a common cause of unwanted lid retraction. Treatment of lid retraction consists of recession of LPS along with Mullers muscle. Lagophthalmos In this condition there is either difficulty in closing the eye or there is complete absence of lid closure. Closure of lids is brought about by the orbicularis muscle that is supplied by facial nerve. Hence it is but natural that affection of facial nerve will lead to lagophthalmos, the lesion may be anywhere from its nucleus to extreme periphery in the orbit. Besides neurological causes there are other causes that are generally put under mechanical causes that prevent the lids to close. These are Large coloboma of the upper lid. Scar on the skin surface of the lids. Extreme degree of symblepharon. Ectropion Proptosis Exophthalmos Over correction of ptosis Myasthenia is the main myogenic cause of lagophthalmos In Mobius syndrome there is bilateral facial palsy with bilateral sixth nerve palsy due to aplasia of their nuclei. Nocturnal lagophthalmos is a mild form of bilateral lagophthalmos, which becomes obvious when the child sleeps. A strip of the IPA remains open during sleep. On examination there is mild weakness of orbicularis. Bells phenomenon is intact. Lagophthalmos is generally unilateral due to peripheral involvement of seventh nerve, i.e. Bells palsy or trauma. Bilateral exophthalmos is mostly central in origin. An exception being leprosy which is the foremost cause of bilateral lagophthalmos in adults due to peripheral involvement. Children do not suffer from lagophthalmos due to leprosy. Other causes of bilateral lagophthalmos (facial diplegia) are Causes of bilateral lagophthalmos have to be central. They are Mobius syndrome30 Melkerrson Rosenthal syndrome28,29 Brain stem trauma30 Brain stem glioma30

82 Lyme disease31 Guillain Barre syndrome Closed head injury Myasthenia Neurological causes of unilateral lagophthalmos

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Supra nuclear and nuclear lesions does not involve orbicularis in a peripheral lesion. A lesion at the pons involves sixth nerve nucleus causing ipsilateral lateral rectus palsy, loss of conjugate gaze toward the same side and hemiplegia of the contra lateral side i.e. Fovilles syndrome. The same lesion with intact conjugate movement is called Millard-Gubler syndrome. In children above lesions are due to pontine glioma or degeneration at the level of pons and not vescular as in adults. A basal lesion involving roots of seventh nerve also affects eighth nerve causing loss of hearing, loss of taste in anterior two third of the tongue and diminished tearing. Common causes for such lesions are fracture base of the skull and basal meningitis. Cerebropontine angle lesions causes ipsilateral facial weakness and hyperacusia. Geniculate ganglion lesion causes Ramsay Hunt syndrome. Commonest cause of which is herpes zoster where vescicles develop in external auditory canal and tympanic membrane. Other lesions being same as lesion of cerebropontine angle tumour. Commonest type of facial palsy in all ages is Bells palsy. Bells palsy is an acute unilateral facial palsy involving the facial nerve in the facial canal. The lesion causes swelling of the nerve in a narrow canal. Commonsest cause is viral infection though auto immune causes can not be excluded. The symptoms consist of inability to close the eye on the affected side and watering. This may be preceded by pain in ear and over the mastoid with hyperacusis. The condition is self limiting clearing in two to three weeks without any residual effect. It is known to recur in 3% to 10% cases rarely on the same side, however there may be some cases where there is not complete recovery. Such cases should be investigated for causes other than viral infection. The signs of Bells palsy: 1. Lagophthalmos - The inter palpebral fissure is wide, there is paralytic ectropion of lower lid with watering from the eye. 2. Watering from the eye is partly epiphora due to dribbling of tears over the lid margin and is partly lacrimation due to irritation of unprotected conjunctiva and cornea. 3. The patient is unable to close the eye. In an attempt to close the eye, the eyeball roles up due to intact Bells phenomenon. 4. However lower part of the cornea remains exposed in spite of intact Bells phenomenon and develops exposure keratitis that may be converted to ulceration on long run.

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5. In few days microbial conjunctivitis develops. If Bells palsy does not subside, keratonisation of conjunctiva may develop. 6. Corneal sensation and other cranial nerve are normal. Management of lagophthalmos The management depends upon the cause of lagophthalmos. It can be divided into: 1. Management of Bells palsy 2. Management of anatomical defects 3. Management of central causes. Bells palsy does not require any specific treatment as it is self limiting. However associated conjunctivitis, keratitis and lagophthalmos requires attention. Conjunctivitis and keratitis are treated with frequent antobiotic drops and lubricants by day and antibiotic ointment by night. If lagophthalmos does not improve with in a fortnight temporary lateral tarsorrhaphy may be required. Anatomical defects like scars and coloboma are treated by appropriate oculoplastic surgery. The central causes require more elaborate investigation like CT and MRI and treatment is directed towards primary cause. Melkerrson Rosenthal syndrome28,29 consist of peripheral facial diplegia, puffiness of face and lingua plicata. It manifests in childhood. Lingua plicata is most probably congenital in origin. Mobius syndromeThis is a multiple cranial nerve palsy of congenital origin, nerves from fifth to twelfth are known to be affected. Commonest combination is sixth nerve with facial diplegia. The child has unilateral or bilateral esotropia. There is inability to abduct the eyes.

REFERENCES
1. Lois J. Martyn : Abnormalities of lid function in Pediatric ophthalmology. Vol. 2, Second edition, p. 807-814. Edited by Harley R.D., WB Saunders Company, Philadelphia, 1986. 2. Duke Elder S. : Congenital anomalies in mobility of lids in System of ophthalmology. Vol. III, part 2. P. 887-905, Henry Kimpton, London, 1964. 3. Honavar S.G., Naik M.N., Geeta K. Vemuganti, Chandrashekhar G. : Ptosis in clinical practice in ophthalmology. First edition. p. 497-502. Edited be Saxena S., Jay Pee Brothers, New Delhi, 2003. 4. Miller S.J.H. : Congenital ptosis in Parsons diseases of the eye. Seventeenth edition. p-316, Churchill Livingstone, London, 1984. 5. Kahn N.D. : Ptosis in Current ocular therapy. Fifth edition. p-447-449. Edited by Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadelphia, 2000.

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6. Slonim C.B. : Blepharophimosis in Current ocular therapy. Fifth edition. p-422-423. Edited by Fraunfelder FT and Roy F.H., WB Saunders Company, Philadelphia, 2000. 7. Beard C. : Ptosis. Third edition. p-113-115. CV Mosby, St. Louis, 1981. 8. Fiona Rowe : Ptosis and pupils in Clinical Orthoptics. First edition, p-164-171. Blackwell Science, Oxford, 1997. 9. Duke Elders S. : Paradoxical movements of the lids in System of Ophthalmology. Vol. III. Part 2. p-898-905, Henry Kimpton, London, 1964. 10. Jain, Seth : Prakash BJO, 40, p-588, 1956. 11. Glasser J.S. : Oculo sympathetic defect in Neurophthalmology. p-177-179. Harper and Row, London, 1982. 12. Kanski J.J. : Horners syndrome in Clinical ophthalmology. Second edition. p-474475, Butterworth International, London, 1989. 13. Bajan Das F.J. and Kline L.B. : Horners syndrome in Neurophthalmology. Third edition. p-121-123, JayPee Brothers, New Delhi, 1989. 14. Lois J. Martyn : Myasthenia Gravis in Pediatric ophthalmology. Vol. II. Second edition. p-881-882, Ed. Harley R.D., WB Saunders Company, Philadelphia, 1983. 15. Bajan Das F.J. and Kline L.B. : Myasthenia and ocular myopathies in Neurophthalmology. Third edition. p-141-145, Jay Pee Brothers, New Delhi, 1989. 16. Glasser J.S. : Myasthenia and ocular myopathies in Neurophthalmology. p-268-273, Harper and Row, London, 1982. 17. Miller N.R. : Myasthenia gravis in Current ocular therapy. Fifth edition. p-221-222. Edited by Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadelphia, 2000. 18. Piest K.L., Berry S.M., Carter John E. : Drooping of upper lid in Decision making in ophthalmology. First Indian edition. p-68. Edited by Heuven WAJ and Zwaan J.T., Harcort Brace, 1998. 19. Kanski J.J. : Myasthenia gravis in Clinical Ophthalmology. Second edition. p-477478, Butterworth, London, 1989. 20. Dutta L.C. and Dutta N.K. : Myasthenia gravis in Modern Ophthalmology. Second Vol., Second edition. p-905-907, Jay Pee Brothers, New Delhi, 2000. 21. Fiona Rowe : Myasthenia gravis in Clinical Orthoptics. First edition, p-227-228, Blackwell Science Ltd., Oxford, 1997. 22. Kanski J.J. : Myasthenia gravis in The eye in systemic disease. Second edition. p-5658, Butter Worth Heinemann. 23. Bajan Das F.J. and Kline L.B. : The Tensilon test in Neuro-ophthalmology. Third edition. p-177, Jay Pee Brothers, New Delhi, 1989. 24. Miller N.R. : Myasthenia in Current ocular therapy. Fifth edition. p-221-222. Edited by Fraunfelder F.T., Roy F.H., WB Saunders Company, Philadelphia, 2000. 24A. Kerrison J.B. and Nancy J. Newman : Chronic progressive external ophthalmoplegia in Current ocular therapy. Fifth edition. p-208-210. Edited by Fraunfelder F.T. and Roy F.H., WB Saunders and Company, Philadelphia, 2000.

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25. Rosenberg M.A. : Ocular myopathy in Principle and practice of ophthalmology. Vol. III, First Indian edition. p-1965-1967. Edited by Peyman G.A., Sanders D.R. and Goldberg M.F., Jay Pee Brothers, New Delhi, 1987. 26. Glaser J.S. : Lid refraction in Neurophthalmology. p-36-37, Harper and Row, London, 1982. 27. Meyer D.R. : Lid retraction in Current ocular therapy. Fifth edition. p-439-440. Edited by Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadelphia, 2000. 28. Duke Elder S. : Melkerrson Rosenthal Syndrome in System of ophthalmology. Vol. XIII. p-21. Edited by Duke Elder S. and Macfaul P.A., Henry Kimpton, London, 1974. 29. Mukherjee P.K. and Dongre R.C. : A case of acquired facial diplegia, macular edema and lingua plicata. Ind. Jr. Oph. 21 : 36-39. 30. Bajandas F.J., Kline L.B. : Facial diplegia in Neurophthalmology. Third edition. p-153, Jay Pee Brothers, New Delhi, 1989. 31. Hedges T.R. and Hedge5 T.R. : Bells palsy in Current ocular therapy. Fifth edition. p-205-206. Edited by Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadelphia, 2000.

CHAPTER

Disorders of Lacrimal System in Children


LACRIMAL APPARATUS1,2,3,4
Lacrimal apparatus consists of three parts : A. That produces tears. B. Tear spreading systems : that transports tear from gland, across the conjunctive and cornea to drain out of the eye and C. The drainage system. D. Tear forming parts consist of 1. lacrimal gland proper, one for each eye. 2. Accessory Lacrimal glands.1,2,3,4 E. Tear spreading system consists of 1. lids 2. conjunctiva. F. The drainage system consists of 1. Puncta one in each lid. 2. Canaliculus one in each lid 3. Common canaliculus one for each eye. 4. Lacrimal sac. 5. Naso- lacrimal duct (a) The lacrimal glands The lacrimal glands are the main source of tear. The lacrimal glands are divided into two groups: 1. Lacrimal gland proper and 2. Accessory glands. 1. The lacrimal gland proper is situated in the fossa for lacrimal gland on the superiotemporal aspect of the orbit behind the septum orbitale. The lacrimal gland is divided 86

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into two unequal parts i.e. a larger superior part known as orbital part and a smaller palpebral part by the lateral expansion of aponeurosis of the levatorpalpebral superior and Whitnall ligament. The division is limited to the front part of the gland. In the posterior part the two lobes are indistinguishable. As the orbital part lies behind the orbital septum, it is normally neither visible nor palpable, it has a superior and an interior surface, an anterior and a posterior border and two ends, the lateral and medial. Superiorily, the periostium lies between the gland and the orbital bone; the inferior surface has lateral part of the levator with its extension and lateral rectus under it. The posterior border rests on the orbital fat. The medial end reaches, the levator muscle and the lateral end reaches the lateral rectus. There are about 1012 lacrimal ducts that originate in the orbital part and travel through the palpebral part. Hence, removal of palpebral part alone will produce the effect of total removal as far as tears are concerned. The palpebral part is about one third of the orbital part2. It lies just above the lateral part of the upper fornix. This part is sometimes visible when the lid is everted and the patient looks down and it may be mistaken as a growth. There are six to eight lacrimal ducts that originate in the lower lobe. The lacrimal ducts from both the lobes open in the conjuctival sac 4 to 5mm above the tarsal plate. Histologically, the lacrimal gland, it is an exocrine tuboalveolar gland that resembles the salivary gland and share similar pathological process i.e. acute infection, by mumps, measles, chronic inflammation i.e. Mikuliczs syndrome and growth like adenoma. Blood Supply The lacrimal gland is supplied by lacrimal artery and some times by infraorbital part of the maxillary artery. The venous drainage is through the lacrimal vein that travels back in the superior ophthalmic vein4. The lymphatics drain in preauricular and submandibular glands. The nerve supply to the lacrimal gland is divided broadly in to two parts i.e. afferent and efferent. The former is via lacrimal nerve, which is a branch of, trigeminal. The efferent consist of both sympathetic and parasympathetic nerves. The para sympathetic fibres originate in the lacrimal nucleus in the pons and reach the lacrimal gland in the lacrimal nerve2,4a 4a. The sympathetic fibres reach the lacrimal gland via sheath of carotid through ophthalmic artery. Role of sympathetic nerve in tear production is uncertain. The tear production mostly controlled by parasympathetic4a. 2. The Accessory lacrimal glands. These glands are situated in the conjunctiva. They are very small and contribute small amount of fluid mostly mucin to the tear film. They are : (a) Goblet cells of conjunctiva, (b) Krauses glands in the fornices (c) Glands of Wolfring near the tarsal plate. (d) Glands of Manz in the bulbar conjunctiva. (e) Glands in the plica and caruncle. (f) Infra orbital glands. Development of lacrimal and accessory lacrimal glands The lacrimal gland is ectodermal in origin and is the only source of epithelial tissue in the orbit that is liable to develop malignant epithelial tumours5. The main lacrimal gland

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develops at the end of second month as eight epithelial buds arising from superio -lateral part of conjunctiva. The buds get transformed into solid cords that get canalised by third month. The mesoderm condenses round these. The developing levator divides the gland into palpebral and orbital part by fifth month. Tear production starts by the first month post natal. The lacrimal glands are fully developed by third year6. The accessory glands develop from the conjunctiva as ectodermal invagination7. (b) The tear spreading system It is essential to keep the cornea and the conjunctiva wet through out the life. This is brought about by constant flow of tear from lacrimal glands. As formation of tear is a constant process it should also be removed from the eye least there is an over flow of tears. A delicate balance between production and exit of the tear from the eye exists. This is brought about by constant evaporation of tears from the cornea and conjunctiva, when the eyes are open and an intact drainage system that work through out day and night. For a smooth flow of tears from the superio temporal region to inferionasal quadrant, the lids should have sharp, regular and continuous margin that remains in contact with the globe and moves freely during blinking. The tear is spread across the globe not only by smooth movement of lid but also by gravity, capillary action and surface tension. (c) The Lacrimal drainage system 1. Puncta. Puncta are two openings situated on the posterior part of each lid margin on the medial side. They are not visible unless the lid margins are everted. It is easier to see the lower punctum than the upper punctum especially in children. The puncta are located 6 and 6.5mm from the medial canthus, the upper punctum is nearer the canthus than the lower punctum, hence the puncta do not over lap each other, when the lids are closed. The punta are the openings of the canaliculi. The puncta are kept in contact with the globe and glide over the globe for drainage of the tear. The lower punctum is more important than the upper as far as drainage of tear is concerned. The lower punctum drains about 75% of total tear. Damage to lower punctum causes more epiphora than upper. 2. Canaliculi. Each lid has one canaliculus that starts from the punctum and ends in common canaliculus. Each canaliculus has two parts i.e. a small vertical part and a longer horizontal part the vertical part is 2mm long while the horizontal part is an 8-8.5mm in length. The two parts join each other almost at right angle, each canaliculus pierces the lacrimal facia separately and the two join to form the common canaliculus which opens on the lateral side of the sac, occasionally the two canaliculi may open separately. The canaliculi lie superficially in the lid hence they are frequently damaged by wounds of lower lid. 3. Common canaliculus. The two canaliculi join to form common canaliculus before opening in the lacrimal sac. 4. Lacrimal sac. The lacrimal sac is the reservoir of tears between the canaliculi and the nasolacrimal duct. The normal sac which is 11-12mm in length, 4 to 5mm in width, and is flattened anterio posteriorly, the anterio posterior depth is only 2 to 3mm. Thus, it is more or less like a club that has been flattered anterio posteriorly. Its volume is 20 cu.mm when not distended. This much of fluid is sufficient to make the sac non visible and non palpable. In normal condition the sac lies in the fossa for lacrimal sac that is situated in the lower medial

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part of the orbital rim. The frontal process of maxilla and lacrimal crest forms the fossa. The fossa has two ridges called lacrimal crests, one formed by maxilla is known as anterior lacrimal crest the other is called posterior lacrimal crest. The upper dome shaped part of the sac is called its fundus while most of the part is called body. The part that joins the nasolacrimal duct is called the neck. There is no strict anatomical landmark to differentiate the three parts. The sac is surrounded by the lacrimal fascia that is formed by splitting of the periorbita. Between the lacrimal fascia and the sac is a plexus of veins. Non-kertanised squamous cells, goblet cells and some columnar cells resembling respiratory columnar cells line the inner wall of the sac. The ethmoidal cells and medial meatus form the medial relation of the sac, on the lateral side is the caruncle and palpebral part of the orbicularis, behind the sac lies the lacrimal part of the orbicular is and posterior limb of medial canthal ligament. Anteriorly, the anterior limb of the medial palpebral ligament crosses the sac mid way between the fundus and neck above the larimial fascia 4a. The angular vein runs vertically 8mm medial to the medial canthus, a tributary to this descends with in 3mm of the sac. This is an important landmark, while exposing the sac. The incision should be with in 3mm of the medial canthus. The incision to expose the sac should be concentric with the fibres of the orbicularis. The best landmark to locate the sac is to feel the medial palpebral ligament first and give a vertical incision over the ligament at right angles to the ligament. For ideal exposure the incision should begin 3mm above the medial palpabral ligaments. The medial palpebral ligament is felt as a horizontal band medial to the medial canthus when the lateral canthus is pulled laterally. As the sac has less space to distend at the fundus, the sac swellings are more marked under the medial canthal ligament. An oval swelling above the medial canthal ligament is more likely to be unrelated to the lacrimal sac. 5. Naso lacrimalduct. The naso lacrimal duct is down ward extension of the lacrimal sac in a bonny canal. It extends from the sac to the interior turbinate of the nose. Its about 18mm in length. It is directed down words. back wards and laterally. The directions of the naso lacrimal duct be taken into consideration while probing in children least a false passage is formed. The resiratory type of columnar cells are more in NLD than in sac. The duct has a larger in introseous (12.5mm) part and a smaller intra mural part. The NLD is surrounded by rich vascular plexus that is similar to seen in interior turbinate. The nasolacrimal canal is formed by maxilla, lacrimal bone and inferior nasal concha. The intramural part open under inferior turbinate that is 30-40mm form the anterior nares in adults and 20mm in children. The lacrimal passage gets its blood supply from superior and inferior branches of the ophthal micartery, in praorbital artery angular artery and spheno palatine artery. The venous drainage is via angular intra orbital, and nasal veins. The lymphalies drain in submandibular and deep cervical glands. Development of the lacrimal drainage system The lacrimal drainage passages have a common origin. They are ectodermal in origin embedded in mesoderm. The lacrimal sac and naso lacrimal duct develop beneath a furrow formed by the lateral nasal process and maxillary process. The lacrimal sac develops as a solid cord of ectoderm at 10mm stage, by 15mm it sends two columns in to the lid to form the canaliculi. The lower canaliculius separates a part of the lid to form the caruncle. At 15mm stage the solid cord descend down to form the nasolacrimal duct that is met by similar structure

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arising from the primitive nasal cord7. The solid cord starts canalisation from both the ocular and nasal ends5 in segments. By birth there is a complete cavity of the sac and patent nasolacrimal duct however some of the ectodermal derbies may remain in the naso lacrimal duct causing congenital ductal block, in about 10% of infants. The canaliculi and puncta become patent after the two lids have separated.

CONGENITAL ANOMALIES IS LACRIMAL DRAINAGE SYSTEM


All the structures from lacrimal puncta to opening of nasolacrimal duct in the inferior meatus may be effected by developmental anomalies singly or in combination. Common congenital anomalies are9 A. Congenital block of nasolacrimal duct B. Congenital mucocele of the sac C. Congenital fistula of the sac. D. Atresia of puncta and canaliculi. A. Congenial Block of Nasolacrimal Duct Congenital block of the nasolacrimal duct is commonest developmental anomaly of the lacrimal apparatus. It is a very frequent anomaly in a child and commonest anomaly of the eye and adexa. It is found in about 6% of new born8. It is more common in premature children. It may be unilateral or bilateral both the sexes are equally involved there may be a positive family history9 commonest site of the obstruction is at the inferior osteum of the duct. The nasolacrimal duct develops as a solid cord in a groove situated in the frontal process of maxilla and lacrimal bone beneath the skin. The duct gradually gets canalised from both the ends but predominantly from upper end. By birth the duct should be fully canalised, there may be few insignificant blocks that get cleared by first month post partum. However in about six percent of cases the canalisation is not complete. Generally there is single obstruction at the lower end. However there may be more than one block. The blockage is caused by epithelial debries. Atrasia of the inferior osteum by a membrane is very common cause of obstruction. The effect of nasolacrimal duct block is not visible until three to four weeks, after birth because the lacrimal gland does not start secreting adequality before third week. A typical presentation is watery discharge in a congested eye with out photophobia. After few days the conjunctiva gets inflamed and there is mucopurulent discharge. At this stage pressure over the sac may result in positive regurgitation. In few months the sac gets distended and there is copious reflux following pressure over the sac. Rest of the eye is not involved in congenital nasolacrimal duct block but becomes prone for recurrent infection. Diagnosis. Diagnosis is straightforward an infant develops epiphora after 3 weeks with or without discharge and positive regurgitation, conditions that may be mistaken as congenital nasolacrimal duct obstruction (congenital dacryocystitis, or neonatal dacryocystitis) are ophthalmic neonatorum, corneal abrasion, primary buththalmos, retino blastoma, Congenital coloboma of the lower lid is a common cause of ipiphora with negative regurgitation test.

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Ophthalmianeonatorum becomes obvious with in first week. It is generally bilateral and there is copious mucopuralent or purulent discharge without regurgitation. Corneal injuries are generally unilateral without mucopurulent discharge and stain with fluoreseein, congenital glaucoma is generally associated with photophobia and the cornea is large, ofcourse the tension is high. The term congenital dacryocystitis is not very appropriate because inflammation of the sac is secondary to duct obstruction, the sac is sterile at birth and the inflammation is noticed after third week post partum hence it will be better to call it as congenital nasolacrimal duct obstruction. Management Management of the condition is simple provided the treatment is initiated before twelve weeks. The treatment consists of opening of the block by external pressure over the sac and treatment of associated infection. Relief of the obstruction is achieved by application of gentle and firm pressure over the sac to force the fluid down the nasolacrimal duct, which dislodges the epithelial derbies. The application of pressure is generally called massaging of the sac. This give relief in 95-98% of cases. The mother is taught this simple method by repeated demonstration and is asked to perform the same in presence of the doctor. She is instructed to trim finger nails and keep the hands clean. Infact she should be asked to wash her hands with soap and water every time before she massages the sac. The sac is pressed with the pulp of the index finger. After the sac has been pressed the mucopurutent discharge should be removed by wet cotton swab and a broad spectrum antibiotic drop is instilled. The process of cleaning the hands pressing the sac, removing the discharge, instilation of antibiotic should be under gone for five to six times a day for six week. After few days of repeated pressure application on the sac, the discharge changes to watery from mucopurulent. The block is often relieved after 3 to 4 weeks but the process should be continued for an other two to three weeks to keep the passage patent. Success of this method depends upon proper compliance, repetition and continuation of the process for five to six weeks. Sooner the massage is started better is the result generally 95% of the cases will be cured by massage and local antibiotic. However this process may not be helpful (1) It started after three weeks of on set of eiphora. (2) There is atrasia of boney canal (3) There is a nasal cause (4) Improper method. In that case probing of the nasolacrimal duct under general anaesthesia through upper puncta is indicated. A single probing done properly results in permanent cure. However sometimes repeated probing may be required. For a successful result the probe should be directed downwards, laterally and backwards10. If repeated probing fails than the condition should be treated either by conventional dacryo cysto rhinostomy, or endoscopic D.CR. Incubation of nasolacrimal duct is another alternative. Complication of Naso Lacrimal Duct Obstruction Commonest organism that is isolated from infected sac is pneumococcus which is a potential danger to the cornea, mild corneal abration in presence of infected sac may result in to disastrous keratitis and corneal ulceration. A chronically infected sac may lead to acute dacryocystites, and fistula of the sac.

92 B. Congenital Mucocele (Dacryocystocele)4a,11

PEDIATRIC OPHTHALMOLOGY

This is a rare condition present at birth as a bluish diffuse swelling medial to the medial canthus. This can be sometimes bilateral. The condition is caused by simultaneous non canalisation of both the canaliculi and nasolacrimal duct. The condition may be confused with a haemangioma. The conditions waxes and wanes size and may altogether disappear following gentle pressure over the sac12,13 otherwise. Probing under general anaesthesia is indicated other condition that may be confused with is meningocele in infants for which suitable tests including C.T. may be done. C. Congenital Fistula of the Lacrimal Sac In this condition of fistulous track develops between the lower part of the sac and the skin on the side of the nose below the medial palpebral ligument . The opening of the fistila may be so small that it may not be noticed. Otherwise there is constant flow of tear form opening . It may occasionally be bilateral. The exact mode of development of the condition is not well under stood. One of the theories put forward is failure of embryonic tissue to close fully14. The diagnosis is confirmed by injection a coloured fluid in the sac through inferior puncta. The dye is seen to trickle through the opening, treatment consists of removal of the fistulous tract and closure of gap in the sac and skin. D. Atresia of Puncta and Canaliculus14 This condition is brought about by failure of budding out of upper part of lacrimal passage into the lid or failure of to canalis the solid mass of cells that eventually become canaliculi. The punctum may be altogether absent or may be rudimentary. Symptoms are epiphora without infection.

INFLAMMATION OF LACRIMAL SAC (DACRYOCYSTITIS) IN CHILDREN


Inflammation of lacrimal sac in children is rarer than seen in adults. The sac may have either acute dacryocystites or chronic dacryocystitis. A. Acute dacryocystitis Acute dacryocystitis is generally pericystic cellulilis secondary to chronic infection of the sac. It may proceed to form an abscess over the sac that may eventually burst on the skin to result into a lacrimal fistula that connects the lumen of the sac with the skin out side. Acute dacryocyititis in children develop when a congenital nasolacrimal duct block has not been relieved, this is generally seen under 5 years of age. Another type develop in presence of chronic bacterial dacryocystitis without congenital block in old children. Symptoms of acute dacryocystitis consist of painful, pink, diffuse swelling between the lateral side of nose and medial canthus which may develop fluctuation and pus forming at most dependent part. Submandibular and preauricular glands are enlarged. Condition may subside only to recur or may form a fistula. Management of acute dacryocystitis is systemic broad spectrum antibiotic (common organisms are H. influenza and pneumococcous) oral or injection, hot fomentation, oral

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analgestic. Local antibiotics are not effective, if the pus points it is drained by stab incision and pus expressed out. The wound is dressed with magsulf and glycerine dipped ribbon gauge. The wound is allowed to heal from beneath. Syringing and probing is contra indicated. When acute infection subsides, external or endoscopic DCR is performed. B. Chronic dacryocystitis in children Chronic dacryocystitis in children is less common than adults. In adults it is caused due to acquired nasolacrimal duct obstruction mostly in females in third and fourth decade who have narrow nasolacrimal canal which gets obstructed due to spread of infection from nose and throat. In children chronic dacryocystitis is caused due to unrelieved partial stenosis of nasolacrimal duct . It may follow chicken pox15 measels, where infection travels down from the conjunctiva, as well ascends from nasopharynx. Other possibility is mid fascial trauma with fracture bony nasolacrimal duct. Lastly rhinosporidiosis is a common cause of chronic inflammation of sac in children in endemic area.16 The symptoms consist of epiphora with or without conjunctivitis, diffuse painless swelling (mucocele) under the medial canthal ligament, regurgitation of pus from the puncta on pressure over the sac. Generally the condition is unilateral. The Signs are mucoid discharge near the medial canthus, mucocele, positive regurgitation test. The mucopurulent discharge may come from any or both puncta. Persistent absence of regurgitation from any puncta in presence of mucocele denotes corresponding canalicular block. Positive regurgitation test is a due to nasolacrimal duct obstruction which is confirmed by 1. Taste test 2. Lacrimal Syringing 3. Jones I and II test 4. Dacryocystography 5. Lacrimal scintilography31

RHINOSPORIDIOSIS OF SAC16
Chronic dacryocystitis of the sac due to rhinosporidiosis is rarely seen outside endemic area of rhinosporidiosis. The disease is most prevalent in tropical and subtropical countries like India, Sri Lanka, Indonesia, Philipines. India from where large number of cases17,18 have been reported too have pockets where the disease is prevalent. These area have high rain fall, humid and warm climate as in Kerala, Tamilnadu, coastal Andhra, Orissa, West Bengal and Chhattisgarh. The disease is caused by an organism Rhinosporidium Seebri Exact mode of spread of infection is not known. A widely put forward theory is what the patients get the infection from infected cattle. When the persons share the same pond for bathing with infected water

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buffalo, the water borne spores get lodged in the anatomical recesses like fornices, plica, nasolacrimal duct. The organism derives its name from the fact that it was first described as a nasal lesion. Nasal lesion happens to be most common involvement. Incubation period of the disease is not known. All persons in the same locality do not get the same disease. It is rarely seen in other the members of the same family, bilateral lesions are infrequent, simultaneous involvement of sac and conjunctiva is also rare. Most probably the infected child has a pre existing total or partial block. Selective involvement of a few persons in the same locality with similar life style point towards an immune mediated mechanism. There are two types of ocular involvement . A. Primary where either the sac or conjunctiva is involve without nasal involvement. B. Secondary. Here the infection spreads to the sac from the nose via nasolacrimal duct and conjunctiva is generally spared. Appearance of the lacrimal sac is similar in both the types. There is a defuse pain less swelling over the sac, the swelling is non tender, skin over the swelling has an orange peel appearance. Though there is associated chronic dacryocystitis, eiphora is not a dominant symptom. The condition is generally unilateral there is no regurgitation, following syregning the swelling increases in size. Which disappears by firm pressure. The nasolacrimal duct is either open or partially blocked. Generally there is bleeding from the nose in case of associated nasal involvement. Lymph nodes are not involved. Management There is no specific systemic or local medial treatment. Associated conjunctivitis should be treated with local antibiotic drops. Definite treatment is surgical removal of the sac. Precaution should be taken to remove whole of the sac without repture. Complete removal of sac ensures freedom from the symptoms and recurrence. It part of the tissue is left behind the condition is bound to recur.

CONGENITAL ANOMALIES OF LACRIMAL GLAND1,9


Congenital anomalies to lacrimal gland are far rarer than that of lacrimal passage. Common anomalies of lacrimal gland are A. Absence of the lacrimal gland B. Alacrima. C. Aberrant lacrimal gland D. Fistula of lacrimal gland E. Congenital cyst of lacrimal gland F. Crocodile tear. A. Absence of lacrimal gland The lacrimal gland develops from the conjunctiva of the upper fornix as buds going up and out from the developing conjunctiva. It is but natural that the lacrimal gland will be absent in absence of the conjunctiva as in cryptopthalomus. Lacrimal gland is present in anophthalmos where the eye ball is absent but conjunctiva is present.

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B. Alacrima In this condition there is partial or total absence of tear production in presence of lacrimal gland and its ducts. It is generally a bilateral condition. The parents may notice that though the child has redness of the eye and intolerance to light, the eyes do not water as is expected and has only thick mucus discharge. On investigation both basic and reflex tearing are absent , exact mechanism is not known most widely accepted theory is that there is lack of neural communication between the gland and the lacrimal nucleus . Others feel that the lacrimal gland itself is hypoplastic. It may be associated with multiple cranial nerve anomaly specially sixth and seventh or may be part of congenital autonomic dysfunction known as Riley- Day syndrome. RileyDay syndrome1,19 Is a rare disorder mostly seen in Jewish children caused by sever depletion of parasympathomimetic neurones in spheno palatine ganglion. It has wide spread systemic features which include, Episodes of systemic hypertension, excess of sweting, cyclic vomiting muscular hypotony, emotional out burst, feeding difficulty. The children are less sensitive to pain, decreased tenden reflexes is common. Ocular symptoms consist of alacrima, absence of emotional tear production, absence of basic and reflex tear production, loss of corneal sensation, corneal opacity, vascularisation. The pupil constricts with very weak solution of pilocarpine (0.0625%). There is no specific treatment management is symptomatic ocular treatment consists of local tear substitute drops by day and ointment by night, local anti biotic. C. Aberrant lacrimal gland Aberrant lacrimal glands other than accessory lacrimal gland are found at any location under the conjunctiva, commonest site is outer half of conjunctiva. It may be seen at limbus and mistaken for limbal dermoid. It can even be seen on iris and choroid. D. Fistula of lacrimal gland Lacrimal gland fishila are seen on outer side of the upper lid above the tarsal plate, they may occasionally be associated with malformation of outer canthus or with congenital cyst of lacrimal gland . The opening is generally associated with a tuft of hair around it. As the opening, communicates with the lacrimal gland, it is obvious that tear should flow out from the opening. The flow is enhanced when there is stimulation of fifth nerve. The tract is common site for recurrent infection. Treatment consists of removal of the tract and closure of the communication with the gland. E. Congenital cyst of the lacrimal gland Congenital cyst of lacrimal gland is seen only in the orbital part of the gland as a tense swelling under the superio temporal aspect of orbit, this may cause a ptosis, S shaped curve in the upper lid and proptosis. It may be present at birth or may become obvious later. Treatment consists of removal by orbitotomy if there is moderate to server proptosis. F. Crocodile Tear4a,11 This is a paradoxical reflex feature that is generally unilateral caused by congenital misdirection of seventh nerve towards lacrimal gland through spheno palatine ganglion. In this condition there is unilateral tearing during mastication. More common is aquired paradoxical tearing following seventh nerve palsy due to aberrant degeneration.

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Systemic condition that produce unilateral tearing due to gusto lacrimal reflex are : brain stem lesions, Ipsilateral sixth nerve palsy is common in congenital crocodile tear.

THE LACRIMAL GLAND DISEASES IN CHILDREN


Disorders of lacrimal gland can be : A. Congenital (very few) B. Acquired. B. The acquired disorders can be : 1. Infective 2. Inflammatory 3. Neoplasia 4. Trauma : Dacryoadinitis, Acute or chronic : Sarcoidosis, (Generally not seen in children) : Not seen in children : Isolated injury to lacrimal gland is rare occurrence it is met in sharp penetrating injury directed to the gland or is seen in sever crush injury of skull and face.

More common than above are : 1. Excessive production of tearLacrimation 2. Scanty production of tearOcular surface disorders. The symptoms of lacrimal gland disorders are : 1. Painful swelling of the main lacrimal gland. 2. Slow progressive swelling of lacrimal gland (chronic dacryodenitis) 3. Discharging sinus 4. Lacrimation. 5. Ocular surface disorder. 6. Proptosis 7. S Shaped Curve of upper lid 8. Ptosis.

ACUTE DACRYOADENITIS4a,4b
It is seen between five years to late teens. It is generally unilateral. The viruses infect the lacrimal glands more commonly than bacteria. Commonest virus is mumps followed by infective mononucleosis rarely by herpes zoster. The disease starts with chills rigor and pain in the superio temporal aspect of orbit. There is a tense swelling over the lacrimal gland, mild proptosis, ptosis, S shaped curvature of upper lid. Conjunctival congestion and rarely restricted movements and optic neuritis, infact any part of the eye except lens and vitreous can be involved by the disease. The disease is caused by mumps virus single attack gives life long immunity. Parotids and other salivary glands are invariably attacked along with lacrimal gland. It commonly

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infects the gonads. Rarely it can cause central nervous system involvement in the form of meningitis, encephalitis, myelitis and cranial neuritis differential diagnosis consists of stye, infected chalazion, acute unilateral ineffective conjunctivitis, cellulitis lid and orbital cellulitis. Management consist of Prophy laxis. Alive, attenuated mumps virus vaccine is safe and effective in child hood. It gives life long immunity. In many countries this has been included in routine vaccination in childhood. Curative there is no known anti mumps chemotheraptic agent. Supportive. Bed rest, analgesic, cool wet compresses, use of steroids is restricted to CNS involvement. Keratitis, scleritis, uveitis are treated by usual methods. Chronic dacryo adenitis is infrequent in children. It may be because by tuberculosis, saccoidosis and rarely fungi.

DISCHARGING SINUSES OF LACRIMAL GLAND


Discharging sinuses of lacrimal gland can be congenital, traumatic or post infective. The sinus opens on the skin surface treatment is excision of the sinus. Recurrence is common.

LACRIMATION
Lacrimation is defined as excessive production of tear. It may be with normal drainage or obstructed drainage. It is due to A. Reflex Stimulation of Fifth nerve either in the Eye or Nasal mucous B. Psychogenic C. Supra Neuclear Lesion. A. Reflex Stimulation of Fifth Nerve can be Brought about by 1. Physical factors. Bright light, cold wind, heat. 2. Chemical. Irritative gases and fumes. 3. Corneal condition that lead to lacrimation are Corneal abrasion, foreign body on the cornea and tarsal plate, super ficial and deep keralitis, corneal ulcer, phlyctenular kerato conjunctivitis, rupture in Desmets membrane (buphthalmos ) endothelial decompensation. 4. Uveal conditions that lead to lacrimation are acute and chronic anterior uveitis 5. Acute allergic conjunctivitis. 6. Glaucoma. Primary congenital buphthatmos, secondary buphthalmos, secondary angle closure glaucoma. 7. Reflex irritation of nasalmucosa. 8. Allergic rhinitis, looking at bright light, tickling of nasal mucosa sheezing coughing.

98 B. Psychogenic

PEDIATRIC OPHTHALMOLOGY

The exact cause of psychogenic tearing is not known however it is the commonest non pathological cause of tearing in children. C. Supra Neuclear Lesion CNS cause include supraneuclear diseases i.e. pseudo bulbar paby, tumours, encephalitis.

EPIPHORA
Epithora is defined as over flow of tears in presence of normal tear production it is a passive phenomenon. Epiphora is produced due to A. Obstruction in tear drainage apparatus i.e. puncta, canaliculi, sac or nasolacrimal duct or B. failure of tear spread i.e. lid deformity like ectropion, cloboma of lower lid, tumors at the lid margin. The tear is produced in the lacrimal gland and accessory lacrimal glands in an aqueous form to these are added mucin from the conjunctival glands and lipid from sebaceous glands of the lid. The lacrimal secretion is released in the outer corner of the superior fornix, from there it is propelled towards the puncta by capillary action, surface tension and gravity across the globe to be collected in a depression in the lower lid called lacus lacrimalis. The puncta, which are in contact with the globe imbibe the tear, that reaches the sac via canaliculi, and when the lids are closed and push the tear down the nasolacrimal duct when the lids open.20 Thus with each blink tear is constantly forced through the drainage passage about seventy five percent of drainage is through the lower punctum and rest from the upper. Epiphora becomes worse if reflex tearing is super imposed to it. C. Causes of Epiphora Cause of epiphora may be single or multiple extending from lid margin to nasal mucosa. For proper drainage the inner lid margin should be sharp and in contact with globe any derangement in this will cause the tear to spill over the lid margin. Lid conditions that cause epiphora are:- facial palsy, ectropion of lower lid, congenital or acquired coloboma of lower lid, growth on the lid margin and tylosis. The puncta may be absent, atrisic or pinpoint. It may be involved in trauma. In ectropion the puncta generally looses its contact with the globe, commonest canalicular cause of epiphora is also trauma however it may be blocked following canaliculitis or dacryolith. Most important cause of epiphora lies in the sac as chronic dacryocystitis either acquired or congenital. In both instances the sac ifself is patent, the obstruction lies in the naso lacrimal duct. However chronically inflamed sac can become fibrosed and loose its lumen. Other cause of epiphora are absence of sac following dacryocystectomy, failure of DCR or nasolacrimal and lacrimalintubation. Sometimes the opening in the inferior meatus may get blocked by a nasal growth i.e. polyp. Tumours of the sac are unknown in children. Trauma involving fossa for lacrimal sac or nasolacrimal duct may also cause epiphora.

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A. Investigation in case of epiphora in children 1. History. Epiphora in infants and children from first month postnatal onwards is due to neonatal block of nasolacrimal duct. Watering with discharge in a new borne is most likely to be ophthalmic neonatorum rather that congenital nasolacrimal duct obstruction. In children in second decade history of chicken pox and measles may be positive. In the same age group history of bleeding from the nose is found in rhinosporidiosis of sac, history of facial injury may be associated with nasolacrimal duct obstruction secondary to fracture of lacrimal canal. 2. Examination should consist of (a) Regurgitation test (b) Taste test (c) Lacrimal syringing (d) Fluoreseine clearance test (e) Jones I and II test (f) Examination of lid margin for ectropion, coloboma of lid margin, growth of lid margin and tylosis. (g) Examination of nose (h) Evidence of scar over the medial canthal ligament (i) Dacryocystography (j) Lacrimal scintillography (k) CT Scan (a) Regurgitations Test. Normally pressure over the sac does not produce regurgitation of mucoid or mucopurulent discharge from any or both the puncta, presence of regurgitation always mean obstruction in the nasolacrimal duct or failure of DCR or intubation. Generally regurgitation from lower puncta is more than from upper puncta. Regurgitation from lower puncta with out regurgitation from upper means naso lacrimal duct block with block in the upper canaliculus. Regurgitation from upper puncta only means block in lower canaliculis along with nasolacrimal duct block. Absence of regurgitation in case of chronic dacryocystitis may be due to (i) Recent evacuated of sac, (ii) Encysted mucocele of the sac (iii) Pseudo sac following incomplete removal of sac. (iv) Patent DCR and intubation (v) Fibrosed sac, (vi) Rhinosporidiosis of sac. (vii) In case of fistula of sac the discharge comes through the opening on the skin, so does any dye put in conjunctive comes. (b) Encysted mucocele is a peculiar state of chronic dacryocystitis generally seen in adult women, however it may be seen in children also, where there is a distended sac, which is

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visible as a diffuse oval swelling between the nose and medial canthus. The swelling is tense, non tender, non reducible without any external sign of inflammation. On exerting pressure over the sac there is no regurgitation of contents in the conjunctival sac. Generally there is no epiphora and conjunctiva is non congested, devoid of any discharge. Lacrimal syringing is not possible. No drug or dye reach the throat following instillation in the conjunctival sac. As epiphora is invariably absent or very scanty, patient seldom complains about it. The patient generally presents with a tense painless swelling. Management is dacryocystectomy . Persistent epiphora that is a common complication of dacryocystectomy is rarely seen following dacryocystectomy in encysted mucocele as both the canaliculi are blocked. DCR or intubation is unnecessary. Purpose of removal of infected sac is to remove a potential source of infection so near the globe. An encysted mucocele may get infected and cause acute pericystitis that may ruputure forming a lacrimal fistula. (c) Taste Test19a, 19b. As tear drains in the throat after reaching the inferior meatus, any fluid instilled in the conjunctival sac is bound to reach the throat and patient can feel the taste of fluid the instilled in the conjunctiva. It can be saline, glucose or a bitter solution. A bitter solution is a better choice. Any bitter therapeutic grade of fluid that will not irritate the conjunctiva can be used. Commonest solution used is chloramphenicol ophthalmic drops. One eye is tested at a time. A drop of chlorimphenicol is put in the conjunctival sac and patient is asked to blink a few times, a second drop is repeated after few minutes. If the lacrimal drainage is patent on the side being examined the patient will feel the bitter taste at the back of the tongue , the other side is tested after forty eight hours because if the drop is put in both the eyes and one side has a block the patient will not be able to state from which side the fluid has come. This test may be used to see the patency of DCR or intubation as well. It fails to differentiate between total and partial block. In case of partial block the drug may reach the throat after few hours or may require pressure over the sac. This test is not of much value is small children who may not complain of bitter taste. (d) Lacrimal syringing. In this test the lower punctum is dilated with punctum dilator under local anaesthesia. A lacrimal syringe is loaded with 2cc of normal saline, lacrimal canule is attached to the syringe, the tip of the canula is attached to the syringe, and is passed through the punctum and canaliculus. The plunger of the syringe is pressed slowly. Following observations are possible

Fluid freely flows into the throat: (a) Naso lacrimal duct is patent. (b) DCR or intubation is patent.

Fluid regurgitates through upper puncta and by the side of the lacrimal cancula in the lower puncta but does not reach the throat this means that there is a block in NLD but both the canaliculi and puncta are patent. Fluid does not regurgitate through upper puncta does not reach throat only regurgitates by the side of lacrimal canulaNaolacrimal duct and upper passage are blocked. Fluid flows out of hole on the skinLacrimal fistula with or without block in the nasolacrimal duct.

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Not possible to do syringing either from upper or lower puncta: both the puncta are blocked.

(e) Fluoreseine Clearance (Disappearance) Test. A drop of 2% fluorescein sodium is instilled in the conjuctival sac. This stains the tear. The colour of the tear becomes lighter as the tear drains. In case of normal drainage passage the fluorescine should disappear from the conjunctiral sac. The presence of fluorescence is examined on a slit lamp with cobalt blue filter. If no or little stain is present, the drainage passage is normal. If most of the stained fluid is retained after 10 minutes the drainage passage is not functioning well, may or may not have blocked nasolacrimal duct. (f) Jones I (Primary dye test)20,21,22. This is a functional test to find lacrimal out flow under normal physiological condition. In principle it is almost same as fluoresccin disappearance test except that instead of observing disappearance of dye from the conjunctiva. It is recovered at the meatal end. A drop of fluorescein 2% is instilled in the conjunctival sac, the nasal mucous is anaesthetised and vaso constrictor drop is used to shrink the mucosa. A cotton tipped wire is passed through the external nares to reach the opening of the nasolacrimal duct under the inferior turbinate. The applicator is withdrawn after one minute and examined for presence of stain, if stain is absent the applicator is reintroduced at an interval of one minute and examined. If the nasolacrimal duct is open the dye is recovered with in five minutes. Other dyes like mercuro chrome may also be used. This test gives false result in about one third cases, mostly due to failure to place the cotton at the opening of the nasolacrimal duct. It should be remembered that the opening is situated 3 cm from the external nares. If the test is negative Jones II (Secondary ) test is done. (g ) Jones II (Secondary dye test). In contrast to Jones I test this is not a physiological test because the dye is pushed down under pressure. A positive test denotes partial block of the nasolacrimal duct. The test is performed as in Jones I upto instillation of dye then the sac is irrigated with normal saline by a lacrimal syringe. If the stained saline is recovered from the nose the test is considered to be positive which means that the dye was held up in the sac due to partial block in the nasolacrimal duct. Care should be taken to prevent the saline going to the throat and not coming out of the nose. This can be achieved by lowering the head of the patient by 45. A modified secondary dye test comprises of asking the patient to blow the nose on a white tissue paper when Jones I test is negative and observe for sprinkled stain on the tissue paper. (h) Examination of lid margin. The lid margin is examined for any evidence that cause loss of contact of lower lid margin with the globe through out its length, any loss of contact will lead to spilling of tears. Common causes are ectropion of the lower lid, coloboma of the lower lid, a small coloboma due to non repair or faulty repair of lower lid margin leads to intractable and difficult to manage epiphora, orbicularis palsy. (i) Evidence of scar over in medial canthal ligament. Scar over the medial canthal ligament denotes previous surgery that may be simple dacryocystectomy, failed DCR or blocked intubatinon or a closed fistula. (j) Examination of nose. As the nasolacrimal duct opens under the inferior turbinate it may be blocked by nasal polyp, nasal growth or deformity of medial turbinate.

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(k) Dacroyocystography. Plain X ray without contrast fails to show any defect in the sac or naso lacrimal duct. To make them visible it is essential to use a contrast dye, then x-rays of posterio anterior and lateral views are taken. This is called simple or conventional dacryocystography. Disadvantage of this procedure is that it fails to demonstrate diverticule and filling defects. For better visualisation a modified method in which a fine catheter is introduced through one of the canaliculus and dye is injected through this. Commonly used dye is lipoidal ultra fluid. The pictures are taken when the dye is being injected. Both the sides may be catheterised simultaneously and both sides are exposed at the same time23. If the x-ray film is kept at some distance from the patient an enlarged view is obtained. (l) Lacrimal Scintillography. Lacrimal scintillography is a reliable reproducible test for canalicular function, it is not a reliable test for naso lacrimal duct block. A positive test shows prolonged pooling of radio isotope at the obstruction site. That is instilled in the the conjunctival sac and examined by gama camera23a,23b. (m) CT Scanning. CT scanning is ordered in case of facial trauma involving the nasolacrimal duct. All the above investigations are always not possible in small children, lacrimal syringing should be done under general anaesthesia in children under three years as a struggling child may cause damage to the lower canaliculus that may lead to perpetual epiphora, stenosis, or may injure the cornea. In infants only test that requires to be done is examination for regurgitation.

ABNORMAL TEAR STATUS IN CHILDREN


The tear is an essential fluid to maintain the integrity of the ocular surface i.e. the portion exposed to atmosphere namely cornea and conjunctiva. Functions of the tear is to keep the ocular surface wet and glistening, it provides oxygen to the cornea. It corrects microscopic irregularities on the surface to give a better optical status to cornea, washes away debris, small foreign particles offending micro organism and chemicals, It keeps the cornea cool. The tear contains lysozyme and lactoferrin24 which are mild bacteriostatic that helps to keep infection to minimum. However these enzymes have a limit, beyond which they are ineffective specially if the organism is virulent. The tear production is divided in to two parts : A. A large amount called reflex tear B. A small amount called basic or resting tear. The tear film or precorneal tear film as its is better known has three layers with distinct origin, chemical composition and function. The most superficial is known as lipid layer followed by aqueous layer and innermost is called mucin layer. The outer, lipid layer is secreted by the meibomian gland, glands of Zeis and Moll25. This is an oily layer that limits evaporation and gives vertical stability to the tear meniscus by increasing surface tension so that the tear does not dribble over the lid margin. It also lubricates the surface of the globe to allow the lids to glide smoothly. The lipid layer is removed by irrigation, frequent instillation of drops or use of local anaesthesia.

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Ninety Five percent of aqueous (middle) layer is secreted by main lacrimal gland and remaining by accessory glands of Krause and Wolfring. This is the thickest layer that dissolves oxygen and contains lysozyme and lacto ferrin. In fact it is the most important constituent of tear by virtues of its fluidly and amount which can be increased many folds when need arises. It acts as a flushing system that washes away debries and micro organisms. One of its main function is optical. The main function of the innermost i.e. layer consisting mostly mucin is to anchor the aqueous layer to the ocular surface. Though the corneal surface looks smooth , in fact it has microscopic rough texture. The epithelial cells have microvilli that project forward making the surface uneven. The epithelium contains lipoprotein that make it hydrophobic i.e. , it is not wetable by aqueous layer . The inner layer is secreted by conjunctival goblet cells, glands of Manz and crypts of Henle. The mucin contain glycoprotein which are hydrophilic and turns the coreal epithelium to a wetable surface absence of mucin layer makes the cornea non wetable and dry. The tear under normal conditions is formed at a constant rates of about 1.2l/min26,27, total amount of tear under physiological condition is six to seven micro litre in each eye. The volume can be in increased to a certain level before it spills over the lid margin in case of reflex tearing . In case of eipphora usual amount of tear also spills over the lid margin. The tear is an optically clear fluid with refractive index of 1.357. This property is utilised in contact lens fitting. The tear contains organic and inorganic compounds in various concentration some of the values can be used to determine their concentration in serum i.e. glucose and urea sodium, potassium and chloride have higher concentration when compared with blood. pH of tear is 7.31 to 7.7. To prevent burning an eye drop should have pH that does not differ much from this. The tear is high in protein concentration. The protein fractions demonstrable in tear on electrophoresis are albumin and globulin. The gamma globulins found in normal tear are IgA. IgE which play a vital role in allergic conjunctivitis. Tear production is reduced following systemic drugs i.e. anticholinergic, antihistamines, phenothiazine, diuretics.

ABNORMALITY OF TEARS CAN BE BROUGHT UNDER THREE GROUPS


A. Excessive production with normal out flow (Reflex lacrimation). B. Over flow of tear with normal production of tear due to block in lacrimal drainage passage. C. Sub normal production of tear involve and composition (Tear film abnormality, Dry eye Syndrome). Dry eye syndrome can be brought about by.( All ages) 1. Aqueous deficiency 2. Mucin deficiency 3. Lipid abnormality 4. Irregularity of corneal or conjunctival surface 5. Abnormality of lid margin.

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Aqueous deficiency tear abnormality is commonest form of dry eye syndrome followed by mucin deficiency. Lipid abnormality is very rare this is confined to absence of meibomian glands either congenital or following extensive trauma. It is seen in chronic blepharitis where a chemical change is brought about in the lipid layer that causes unstability of the tear film28,29. In chronic meibomianitis bacterial lipase hydrolyses the lipid in to fatty acid that causes disturbance in lipid layer causing dry eye.

DRY EYE SYNDROME IN CHILDREN IS BROUGHT ABOUT BY


A. Congenital absence of lacrimal gland, meibomian glands and congenital alacrima (Riley Day syndrome ) B. TraumaticLoss of lid margin, coloboma of lid, inadvertent removal of palpebral part of lacrimal gland, acid alkali burn. C. Exposure conjunctivitis and kertanisation of conjunctiva D. Conjunctival scaring 1. Late stage of trachoma (generally not encountered in first decade) extensive membranous and pseudomembranous conjunctivitis. 2. Stevens Johnson syndrome, ocular pemphigoid, chemical burns mostly alkaline and radiaton. E. Nutritional : Hypovitaminosis A F. Idiopathic G. Facial palsy H. Neuro tropic keratitis I. Kerato conjunctivitis sicca and Sjogrens syndrome which are major causes of dry eye in adult are not seen in children. Clinical Presentation of Dry Eye in Children Symptoms of dry eye develop gradually and are varied in nature. It may be associated with systemic conditions like vitamin a deficiency, Stevens Johnson syndrome or Riley Day syndrome. Trachoma rarely produces dry eye in children as it takes ten to twenty years to develop conjunctival scaring. Lagophthalmos is generally associated with lacrimation due to exposure keratitis and conjunctivitis. Inability to close the lid leads to poor resurfacing of the tear film. In neurotropic keratitis loss of corneal sensation results in less frequent blinking this also leads to poor tear spread. The symptoms are unexplained redness, foreign body sensation muciod discharge with relative scanty lacrimation, blurring of vision, fluctuation of vision. Diagnosis of dry eye History. Detailed history of trauma, adverse drug reaction, hypovitaminosis A should be elicited Examination Lid for coloboma and orbicularis palsy.

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Conjunctiva for evidence of vitamin A deficiency i.e. xerosis, Bitotspot. Corena for vascularisation, opacity and filamentary keratitis, Vital dye staining. Rose bengal and fluoresecin staining. Rose Bengal staining. Rose Bengal is a vital stain similar to fluorescein. It has affinity for mucus and devitalised epithelium of both cornea and conjunctiva. It also stains corneal filaments and corneal plaques. Rose Bengal stains conjunctiva in the interpalpebral aperture in a triangular fashion. The triangle has its base to wards the limbus and apex away. Rose Bengal is used as 1% aqueous solution either as drop or impregnated over a strip of filter paper similar to fluorescein strip, Rose Bengal causes intense irritation when used on unanesthetised conjunctiva and hence not very popular. Xylocain 4% which is usual local anaesthetic agent used, is contra indicated prior to use of rose Bengal as it devitalises the corneal epithelium, safest local anaesthetic is proparacaine 0.5%. In contrast to rose Bengal fluorescein remains extra cellular and does not stain mucus. Alcaine blue is yet another stain that has staining property of Rose Bengal with less irritation. Tear Film Examination30,31 Tear film examination consists of 1. Tear film stabilityTear film break up time 2. Tear quantitySchirmer I and II 3. Tear quality Tear film break up time The precorneal tear film seems to be a continuos, permanent layer of tear over the ocular surface. In fact the tear film breaks down at a fixed and regular interval between the blinks and develops a so-called dry spot i.e. microscopic area devoid of tear film. This formation of dry spot is physiologically essential to start the stimulus for reflex tearing following each blink. The dry spot is repaired only to reappear. Time lapse between the last blink and appearance of a dry spot is called tear break up time or BUT. In normal eyes it ranges between 15 to 34 seconds.26 A tear film break up time less than 10 seconds is abnormal. Tear film break up time is decreased due to fault in constitution of any layer of tear i.e. mucin , lipid or hyposecretion of aqueous layer. To perform the test the patient is seated in a dimly lit room, the tear is stained with a fluorescein and the tear film over the cornea is examined by a bio microscope using a cobalt blue filter no anaesthesia is required30. The patient is asked to blink a few times and then keep the eyes open. The time between the last blink and appearance of first dry spot is tear break up time. Schirmers Test 1a. This measures total quantity of aqueous tear i.e. basic and reflex. To perform the test a specially designed filter paper of Whatman 41 grade, 5mm wide and 35 mm long is used. The paper strip is folded at one end five millimetre from the end. The patient is seated in a dimly lit room, the strip is inserted behind the lower lid on the outer side of the IPA. In such a way that the folding line rests on the inner margin of the lid and remaining 30 mm hangs out side the lid. No anesthetic is used hence irritation of the paper will initiate the reflex tearing along with basal tearing. The patient is permitted to blink but not to squeeze the lids. The tear is

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drawn over the filter paper by capillary action and spreads to the other end. After five minutes the paper is removed and length of the paper wetted by tear is measured. In a normal eye this should be about 10 mm to 30 m wetting. Less than 10 mm is abnormal. If the strip is moist beyond 30 mm in lessthan five minutes it denotes either hyper secretion (lacrimation) or over flow of normal tear secretion. In both the condition dry eye status is excluded. The test should be postponed by half an hour if the lid has been manipulated. Schirmers Test 1b. Principle of the test is to eliminate reflex tearing by anaesthetising the cornea and conjunctiva. The method is same as in schirmer 1a. Only difference is that the eye is anaesthetised by 0.5% proparacaina or 4% Xylocaine. Length of the moistened strip is measured as in Schirmer Test 1a. As basal secretion is less than total secretion a reading less than 8mm is considered abnormal. Schirmer 2 : This measures reflex tearing the procedure is same as in Schirmer 1b only difference is that the ipsilateral nasal mucosa is irritated by a dry cotton swab, the reading is noted after two minutes and a moistening of less than 15mm is considered sub normal. In a modified schemer test a special type of thread is used instead of filter paper. Test for tear quality. These consist of Lysozymeassay lactoferrinassay, tear osmolarity, conjunctival scrapping, conjunctival impression cytology, conjunctival biopsy. The above test are very sophisticated and require elaborate instrument and advanced expertise hence not used commonly. Management of dry eye status in children Fortunately kerato conjunctivitis sicca either idiopathic or secondary to Sjogren syndrome that is seen in adults are not seen in children. Dry eye status in adults is not vision threatening. However abnormal tear film status, when present in children are potentially sight threatening i.e. in Stevens Johnsons syndrome. Xerophtalmia secondary to vitamin A deficiency and alkaliburn. Hypovitaminosis A is fully preventable if the child gets prophylactic does of vitamin A as prescribed for prevention of xerophthalmia under national programme and the child is immunised against measles. Stevens Johnsons syndrome is an unfortunate non curable disorder. Once a dry eye status has set in the first line of treatment consists of :1. Replacement of tear by a tear substitute that can either be cellulose derivative or polyvinyl alcohol. 2. Mucolytic drugs to remove excess of mucin 3. Reduction of tear drainage 4. Conservation of tear already available. The tear substitutes generally used are available as drops, ointment, gel or in the form of slow release inserts. They may have to be applied almost hourly when the child is awake. Bandage contact lenses along with tear substitutes are claimed to give more comfort to the child when it is possible to insert and retain a bandage lens.

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Tarsorrhaphy in presence of orbiecularis palsy or neuro tropic kertalitis not only protects the cornea form exposure but also retains tear for a longer time. Punctual block is indicated if the child require instillation of tear substitute more often than 5 to 6 times a day. Some times occlusion of lower punctum may suffice. It is better to give a trial occlusion by silicon plug available in two sizes. The plug is put in place by a special applicator. If the child tolerates the trial plug a permanent plug is put. Stem Cell transplant has shown encouraging results by repopulating the corneal epithelium in Stevens Johnsons syndrome.

REFERENCES
1. Ahmed E. ; Text book of ophthalmology, first edition pp-12-15, Oxford University Press, Calcutta 1993. 2. Nema H.V. Singh Y.P. and Nema N. ; Anatomy of the eye and its adnexa, second edition p-73-76, Jay Pee Brothers, New Delhi, 1991. 3. Banumathy S.P. ; Anatomy of the eye first edition p-43-49, Arvind Eye Hospital, Maduri. 4. Duke Elder.S. ; System of ophthalmology, Vol. II p-561-580, Henry Kimptom, London 1961. (a) Buffan F.B. ; Lacrimal disease In Text book of ophthalmology, Vol. 4, edited by Podos. S.M. and Yanoff M., Gower Medical Publication, London 1993. (b) Keden I.H. and Meyer R.F. Mumps ; in current ocular therapy, Fifth Edition edited by Fraun felder F.T. and Roy F.H. p-50-51, W.B. Saunder Company, Philadelphia 2000. 5. Koziol. J. ; Anatomy of the orbit in Principle and practice of ophthalmology Vol. I, edited by Peyman G.A., Sander D.R. and Goldberg M.F. p-87, First Indian edition, Jay Pee Brothers, New Delhi 1987. 6. Nema H.V. Singh V.P. and Nema N. ; Anatomy of the eye and adexa, second edition p-139, Jay Pee Brothers, New Delhi 1991. 7. Duke Elder S. ; System of ophthalmology , vol.-III, part-I , p-239-245, Henry Kimpton, London 1964. 8. Swartz N.G. and Cohen M.S. ; Tearing and the lacrimal system in Ophthalmology secrets, p-226-230, Edited by Vander J.F. and Gault J.A., First Indian edition, Jay Pee Brothers, New Delhi 1998. 9. Duke Elder S. ; Developmental anomalies of the lacrimal apparatus in System of ophthalmology, Vol. III, Part-2, p-911-940, Henry Kimpton, London 1964. 10. Grover A.K. and Bhatnagar A. ; Anatomy Physiology and diseases of lacrimal system in Modern ophthalmology, p-159-160, first Indian edition edited by Dutta L.C. Jay Pee Brothers, New Delhi 1994.

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11. Zwaan J.T. ; Nasolacrimal duct obstruction in children in Decision making in ophthalmology, edited by Heuven W.A.J. and Zwaan J.T., Edition, first Indian pp140-141, Harcourt, Brace and Comp., Singapore 1992. 12. Boger W.P. and Peterson R.A. ; Paediatric ophthalmology in Manual of ocular diagnosis and therapy, Third edition p-277, edited by Deborah Pavan Langston, 3rd edition. 13. Weinstein G.S., Biglan, A.W. ; Congenital Lacrimal Sac Mucocele, Amj. Oph. 1982 94 : 106-110. 14. Duke Elder S. ; System of ophthalmal mology Kimpton, London 1964. Vol. III, Part 2 p-936-937, Henry

15. Mukherjee P.K., Jain P.C., Mishra, R.K. ; Exenthemata ; A caus of chronicdacryocystitis in children IJO 17-27-30, 1969. 16. Mukharjee P.K. ; Rhinosporidiosis in current ocular therapy, fifth edition, edited by Fraunfelder F.T. and Roy F.H. pp-66-67, W.B. Saunders Company, Philadelphia 2000. 17. Mukharjee P.K. Shukla I.M. Deshpande and M. Praveena Kher ; Rhinosporidiosis of the lacrimalsac Ind. Jr. Oph. 30:513-514, 1982. 18. Shukla IM mukharjee P.K. Shushma Verma ; Primary rhinospori diosis of the eye int congress of oph Acta XXVI , Vol. 2, 864, 1982. 19. Axelord F.B, Soloman J.M. and DAmico R. ; Familial dysautonomia unclassified diseases or conditions in Current Ocular therapy fifth edition p-285-287, edited by Fraunfelder F.T. and Roy F.H., Saunders Company, Philadelphia 2000. 19. (a) Hornblass A. ; Asimple test of lacrimal obstruction Arch OPH 90: 435-436, 1973. 19. (b) Hornblass A Ingris T.M. ; Lacrimal function tests. Arch oph. 97 : 1656-1679. 20. Kanski J.J. ; The lacrimal system in Clinical ophthalmology, second edition p-54-56 Butter Worth, London 1989. 21. Swartz N.G., Cohen M.S. ; Tearing and the lacrimal system in Ophthalmology Secrets , Edited by Vander J.F. and Gault J.A. , First Indian edition pp-226-227, Jay Pee Brothers, New Delhi 1998. 22. Piest K.L. and Walton M.A. ; Epiphora in Decision making in Ophthalmology, first Indian edition, P-82, edited by Van Heuven W.A.J. and Zwaan J.T., Harcour Brace and Company, Singapore 1998. 23. Scheie H.G. , Albert D.M. ; Ophthalmic raiodlogy. Im Text book of ophthalmology, Ninth edition P-254-256, W.B. Saunders Company 1977. 23. (a) Miller S.J.H. ; Diseases of lacrimal apparatus in Parsons daises of the eye, Seventieth edition p-327, Churchill Livingstone, London 1984. 23. (b) Chavis, R.K., Welham A.N., Maisey M. ; Quantitative lacrimal scientillography Archoph 96; 2066-2068, 1978. 24. Kanski J.J. ; The dry eye in Clinical Ophthalmology, Second edition p-46-52, Butter Worth International, edition 1989. 25. Daughman D. ; corneal Physiology in Principle and practice of Ophthal mology, first Indian Edition, Edited by Peyman. G.A. Sander D.R. and Goldberg M.F. p-356-358, Jay Pee Brothers, New Delhi 1987.

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26. West C. ; Corneal disease in Principle and practice of ophthalmology, first Indian edition. Edited by Peyman G.A., Sander D.R. and Goldberg M.F. P-447-449, Jay Pee Brothers, New Delhi 1987. 27. Kirmaini T.H., Daughan D., Asbury J., Siva reddy P. ; in Fundamentals of Ophthalmology, First edition p-61-62 , Bushandgo and Co., Karachi 1983. 28. Khamar B., Usha M. Vayas, Trivedi N.m Mayuri Khamar ; Dry eye in Modern Ophthalmology, Edited by Dutta, L.C., First Edition p-29-37, Jay Pee Brothers, New Delhi 1994. 29. Dutta L.C. ; Ophthalmology Principle and practice p-65-67, Current Books International, Calcutta 1995. 30. Deborah Pavan Longston ; Ocular examination technique and diagnostic test in manual of ocular diagnosis and therapy third edition p-16-18, Little Brown. 31. Rosomondo R.M., Carlton W H, Trueblood J.H., Thomas R.P.A. ; New Method of evaluting larimal drainage. Arch Oph 88: 523-525, 1972.

CHAPTER

Disorders of Conjunctiva in Children


Conjunctiva acts almost as mucous membrane of the eye. The part of it that covers the anterior part of the sclera and called bulbar conjunctiva. The part that is reflected over the inner surface of the lid and called palpebral conjunctiva, the junction of the two is known as fornix. The conjunctival epithelium is continuous with the corneal epithelium. The junction of cornea and conjunctiva is known as limbus. The conjunctival epithelium blends with epidermis of skin at the anterior margin of the lid.1,2 It is joined to throat and nose via lacrimal sac and nasolacrimal duct. Thus it will be seen that conjunctiva may be invaded by inflammatory process of lid and nasopharynx with ease. The conjunctiva is never sterile except first few hours after birth. This sterility may be jeopardised if the maternal birth canal is infected due to poor asepsis and antisepsis during delivery, or soon after. The conjunctiva may harbour non pathogenic saprophytes. Pathogenic organisms are introduces from various sources mostly external. The common non pathogenic organisms found in conjunctiva are staphyloccous albus, and diphtheroid. All viruses found in conjunctiva are pathogenic. Some of the fungi found in conjunctival sac may be saprophytes. The saprophytes become virulent due to diminished local or systemic immunity or unexplained increase in virulence of the organism. Pathogenic organisms are introduced from atmospheric air, bathing or washing water, foreign bodies, fly. There are some flies besides usual house fly that have affinity for eyes. Infection from skin of the lids and nasopharynx have direct access to the conjunctiva. In spite of being exposed to numerous routes of infection, infection of conjunctiva is kept at bay by low temperature, mechanical effect of blinking, constant irrigation by tear. The tear also contains a bacteriostatic enzyme called lysozyme which along with immunoglobulins in the tear keep infection under check. It is believed that mear presence of bacteria in conjunctiva is not harmful. A bacteria is said to be pathogenic if it is found on living cell. The conjunctiva is highly vascular and contains adenoid tissue. Conjunctiva has been described as a bisected lymph node lined by mucous membrane.1 Epithelium contains unicellular goblet cells that secrete mucin. The conjunctiva is anchored at the limbus. It can not be separated from the tarsal plate. Rest of the conjunctiva is lax and can be lifted off the globe. Its laxity is maximum in the upper fornix which is deeper than lower fornix. 110

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APPLIED ANATOMY OF CONJUNCTIVA1,2


For purpose of description, conjunctiva has been divided into three anatomical parts without any line of demarcation between two adjacent parts. They are : 1. Palpebral 2. Bulbar 3. Fornix 1. The palpebral part is divided into (a) Marginal, (b) Tarsal and (c) Orbital. The marginal conjunctiva. The marginal part is a transitional zone between the skin of the lid on the outer side and conjunctiva proper. It stretches from middle of the lid margin to sulcus subtarsalis on the posterior surface of the lid. The sulcus sub-tarsalis is a groove running all along the tarsal conjunctiva, 2mm from the sharp inner border of the lid. It is more developed in the upper lid than in the lower lid and is a favoured spot for small foreign bodies to get lodged. The two lacrimal puncta open on the marginal conjunctiva. The tarsal conjunctiva extends from the sub tarsal groove to the border of tarsus. A normal tarsal conjunctiva is transparent enough for the tarsal glands and blood vessels to be visible as light coloured streaks through the tarsal conjunctiva. The transparency of tarsal conjunctiva is lost in edema, hyperemia and scarring. The tarsal conjunctiva is attached to the underlying tarsal plate so firmly that it can neither be lifted off the tarsal plate nor can fluid accumulate under it. The orbital part of the palpebral conjunctiva is an ill defined area of loose conjunctiva extending from upper border of tarsal plate and blends with fornix. The Mullers muscle the non striated muscle of the upper lid lies just beneath this part of conjunctiva. The bulbar conjunctiva. This part of conjunctiva extends from limbus to the beginning of fornices. It is firmly attached to the limbus. The conjunctival epithelium blends with the corneal epithelium at the limbus. The deeper part of the bulbar conjunctiva is loosely attached to Tenons capsule up to insertion of four recti. It is almost transparent, the white sclera is visible through normal bulbar conjunctiva. The conjunctival vessels are also visible in normal conjunctiva. With increase in number of vessels in the conjunctiva, the white conjunctiva become hyperemic. The bulbar conjunctiva is very loose 2-3 mm from the limbus and this laxity is used for sub conjunctival injection. A large amount of fluid can accumulate under this bulbar conjunctiva. The bulbar conjunctiva is transparent, moist and glistening in normal conditions. It is non keratanised but gets keratanised if exposed for long time as in lagophthalmos, proptosis, exophthalmos, ectropion of lower lid. It also gets keratanised in Vitamin A deficiency. The limbus3, 4, 5. The limbus is an important surgical landmark of the eye. It is an ill defined arcuate zone that is junction of cornea on one side and conjunctiva and sclera on the other side. Besides having surgical importance the limbus has two more functions. 1. It supplies stem cells for corneal epithelium, 2. The limbal plexus are the sole sources of blood supply to the corneal periphery.

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The limbus is a ring shaped zone widest superiorly and narrow on the side. The limbus has three lines that divide it into two surgical zones. These lines are : 1. Anterior limbal border, 2. Posterior limbal border, 3. Mid limbal line. The anterior limbal line represents the termination of conjunctiva and Tenons capsule at the corneal periphery, this overlies the termination of Bowmans membrane. The posterior limbal line lies over the scleral spur 2mm away, concentric with anterior limbal line. Its presence is not well felt under operating microscope. It is best demonstrated under sclerotic scatter.3 The mid limbal line lies in between the anterior and posterior limbal line. It is 1 mm away from the anterior limbal line. It represents the termination of the Descemets membrane and overlies the Schwalbes line, looks blue under microscope and the limbus beyond it is white. The limbus contains trabecular meshwork internally.4 The Caruncle. The caruncle developmentally is a part of the lower lid that gets separated from the lower lid by the developing lower canaliculus. It is a transient zone between the skin and the conjunctiva, hence has mucocutaneous functions and shares structure of skin as well as conjunctiva. It is an oval structure that lies medial to plical semilunaris in the medial canthus, has a size of 5mm x 3mm. It is covered by stratified epithelium. As a part of conjunctiva it has goblet cell, Krauses cells and accessory lacrimal gland. As part of skin it is endowed by presence of hair follicles and sweat glands. It does not have any definite function but shares pathological processes common both to the skin and conjunctiva. The plica semilunaris. This represents the vestigial nictitating membrane of the lower vertebrates. It is a crescent shaped fold of conjunctiva with concavity towards cornea lateral to the caruncle in the medial canthus. The lateral border is free with a blind recess underneath that disappears if the eye ball is moved laterally. Histopathologically it is similar to bulbar conjunctiva. It also contains melano phores and few non striated muscle fibers. It also does not have any definite function. The Fornix6 The fornix is an irregular annular caul de sac formed by the junction of tarsal and bulbar conjunctiva. The caruncle and plica semilunaris intrude into it on the medial side. It is divided into four unequal parts : (1) The superior fornix, (2) The inferior fornix, (3) The lateral fornix and (4) The medial fornix The superior fornix is largest and deepest of all conjunctival fornices. It is located at the level of superior orbital margin. The deepest part is at 12O clock i.e. roughly 13 mm from the superior limbus. It gradually becomes shallow on either side. Few slips of levator palpebral superior are attached to the deeper surface of the conjunctiva to give it its depth. It is a common site for foreign bodies to be lost causing intractable conjunctival discharge. It is the only part of the conjunctiva that requires double eversion to examine.

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The inferior fornix is smaller than superior fornix. It is located near the inferior orbital margin, its maximum depth is about 8mm at six o clock position. The lateral fornix is more ill defined than the former two, it extends 14mm from equator on the lateral side. The medial fornix is fully occupied by caruncle and plica semilunaris. The fornices have Krauses gland on the deeper side. Histology Microscopically the conjunctiva is broadly divided into two parts 1. The epithelium, 2. Substantia propria. The conjunctival epithelium is multi layered stratified squamous in nature. It is multi layered. Number of layers vary at different locations. The substantia propria is a combination of lymphoid and fibrous tissue over which the epithelium rests. The lymphoid tissue is not present at birth. It takes three to four months for the lymphoid tissue to develop fully hence follicular reaction is not seen in new born. There are no lymphoid tissues in the inter marginal strip as well, even in adults. The conjunctival glands. One of the functions of conjunctiva is to keep the ocular surface moist for which a stable tear film is a pre-requisite that is given by secretions from various glands present in the conjunctiva. The glands of the conjunctiva are2, 6 : 1. The goblet cells 2. The gland of Krause 3. The gland of Wolfring and 4. Henles gland. The goblet cells are not true glands, they are long unicellular structure. Density of goblet cell vary in various parts of conjunctiva. They are most numerous in fornices, less in bulbar conjunctiva and least in palpebral conjunctiva. Main function of goblet cells is to secrete mucin which is the inner most layer of tear film. The gland of Krause. These are accessory lacrimal glands, they are similar to main lacrimal gland in structure, in development and function. They secrete aqueous part of tear film. Glands of Krauses are found in depth of connective tissue in the fornices. The upper fornix has about 40 glands while the lower fornix has only 6-8 glands. The gland of Wolfring. These are also accessory lacrimal glands. They are larger than Krauses glands but far less in number. There are about 3-5 glands mostly in the upper palpebral conjunctiva at the upper border of superior tarsus. Henles gland. Like goblet cells these also are not true glands. They are folds of mucous membrane between the tarsal conjunctiva and fornix. Blood supply of the conjunctiva6. Conjunctiva is very rich in blood supply. This makes large conjunctival wounds to heal fast. The conjunctiva is supplied by palpebral branches

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of nasal and lacrimal artery, anterior ciliary arteries. The conjunctival veins drain in superior and inferior ophthalmic veins. The conjunctiva is very rich in lymphoid tissue and lymphatics. The lymphatic drainage from lateral half of the conjunctiva is in the pre auricular glands while those from the medial side drain in sub mandibular lymph nodes. Nerve supply of the conjunctiva. The sensory supply of the conjunctiva is through trigeminal via frontal, nasociliary and lacrimal branches. Development of the conjunctiva. Development of conjunctiva is closely associated with development of the lids. Epithelium of the conjunctiva develops from surface ectoderm like cornea. Rest of the conjunctiva is mesodermal in origin.2 Congenital anomalies of conjunctiva. Primary conjunctival anomalies of conjunctiva are few and rare. Most of the congenital anomalies are secondary to maldevelopment of the lids. Some of the congenital anomalies met with are : Epitarsus, Congenital lymphedema, Choristomas (dermoids and dermolipomas) and telangiectasia. Epitarsus. This is a rare anomaly. There is an apron like fold of conjunctiva parallel to tarsal plate and attached to it. The edge of the fold is open. Both the surfaces are covered by mucosa, a probe can be passed between the tarsal conjunctiva and the inner surface of epitarsus for some distance. The epitarsus does not require any treatment. However foreign body may get lodged under this causing chronic infection. It may be confused as inflammatory membrane. Congenital conjunctival lymphadenoma. In this rare condition there is congenital solid edema of the lid with similar edema of the limbs. There is a strong hereditary tendency. It may be present at birth or may develop at puberty. Choristomas. These are common congenital growths of the conjunctiva. They have been defined as normal tissue at abnormal location.7 The dermoids were destined to be skin but were displaced on the eye. Conjunctival choristomas are of two types - dermoids and dermolipomas. Dermoids are commonest epibulbar congenital tumours in children. They consist of both ectodermal as well as mesodermal tissue, the former consists of hair, sebaceous glands, nerves, smooth muscles while the latter consists of blood vessels cartilage. Commonest site being at the inferio temporal limbus, but can arise anywhere at limbus. It is generally single. Sometimes they develop solely on the cornea8 without involving the conjunctiva. When developing at the limbus they are observed to be astride on the limbus partly on cornea partly on conjunctiva. They vary in size. They may be very small and missed at birth and infancy. All dermoids have tendency to increase in size at puberty. They may be large enough to protrude through inter palpebral fissure. They are circular, raised dirty white coloured, may have central pigmentation. Conjunctiva over the dermoid can not be moved. The growth itself is fixed to the sclera. There may be small hairs on the surface of the growth which become prominent with age. The dermoid never show neoplastic change. Rarely they arise on the caruncle. Main symptom of dermoid is visible white growth against the black of the eye. It may be sufficiently large to obstruct the pupil and cause diminished vision. Even when it is seen partially over the cornea it produces irregular astigmatism. In rare instance a dermoid

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may be found to extend inside the eye in the vicinity of iris and ciliary body. About one third case of limbal dermoids are associated with systemic syndromes out of which Goldenhar Gorlin syndrome is the commonest. TreatmentSmall dermoids need no treatment, larger dermoids have to be removed for cosmetic purpose. Dermolipoma This choristoma is less common than dermoid and develop away from the limbus. Commonest site being superior temporal aspect of fornix or lateral canthus. It is diffuse, soft in consistency. It mostly contains fat, other tissues like hair, skin or teeth are not present. The Conjunctiva can be moved over the growth. It has pale yellow colour. It may extend back in the orbit. It does not produce any visual change. It is better not to remove the growth unless there is facility to remove the tumour in toto may be by orbitotomy. Choristomas have been found in microphthalmos, Goldenhars syndrome, optic nerve anomaly and macular hypoplasia. Congenital telangiectasis of conjunctiva. This is a hereditary anomaly of mucous membrane and skin. Out of the mucous membrane the conjunctiva is very commonly effected. It may be discovered at birth or infancy. Commonest age group being late adolescence and early adulthood. It may be associated with telangiectasia of retina. It may be seen anywhere on the conjunctiva but common site is bulbar and lower palpebral conjunctiva where it may be present as a star shaped bunch of vessels or a mass of vessels. As similar changes are seen in their mucous membranes the patient may have epistaxis, haematuria. Traumatic conjunctival bleeding is common. Small a telangiectasia may be obliterated by cryo.

SIGNS OF PATHOLOGICAL DISORDERS


Some pathological signs of conjunctival disorders are : 1. Change in colour, Anaemia (Pallor), Hyperemia, Pigmentation 2. Edema, chemosis 3. Haemorrhage 4. Follicle 5. Papilla 6. Xerosis 7. Scaring 8. Discharge 9. Membrane formation 11. Ulcer 12. Cyst 13. Concretion 14. Degeneration 15. Neoplasm Last three groups are not encountered in paediatric age group. Pallor of conjunctiva Conjunctiva is highly vascular, normal conjunctiva is transparent through which the subconjunctival structures like meibomian glands are visible as bluish streaks. The vessels are visible as dark red streaks against the white sclera. In anaemia the conjunctiva like other mucous membranes become pale. Pallor of conjunctiva acts as a rough indication of presence of systemic anaemia. However conjunctiva can be blanched by instillation of vasoconstrictors. Conjunctiva may become pale as porcelain in alkali burn. Hyperemia of conjunctiva. Increased vascularity of conjunctiva is the commonest sign of conjunctival disorder. It may be caused by abnormality of vessels or development

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of abnormal vessels as telangiectasia or haemangioma. Conjunctival vessels may become dilated either by an active process that is arterial in origin, which is far more common than passive dilatation of conjunctival veins. Active arterial dilatation is called conjunctival congestion or conjunctival injection. It can be acute or chronic, unilateral or bilateral. It can be localised or generalised. Simple hyperemia may not be associated with other changes but it is frequently accompanied by follicles, papillae and discharge in various combination. Simple hyperemia is generally mild localised to fornices or on the bulbar conjunctiva. Transient hyperemia is universal following sleep that passes off after few minutes. Otherwise lack of sleep, uncorrected errors of refraction, fumes, dust, cold or hot wind are other causes of hyperemia. Reflex hyperemia is seen in cases of common cold, disorders of nasopharynx. Foreign body, inturned lash or entropion causes mechanical irritation of conjunctiva resulting into dilatation of vessels. Commonest type of conjunctival congestion is seen in conjunctivitis infective or allergic. This type of inflammatory hyperemia should be differentiated from ciliary congestion. Conjunctival congestion 1. Blood vessel involved 2. Distribution 3. Blood flow 4. Colour 5. Effect of weak vasoconstrictor 6. Branching 7. Mobility 8. Ciliary tenderness 9. Extension Posterior conjunctival Prominent in fornix, fade towards limbus From periphery to centre Bright red Instant blanching Profuse branching Vessels move with conjunctiva Absent Spread over cornea without interruption at limbus Disease of conjunctiva Ciliary congestion Anterior ciliary Prominent round limbus fade towards fornix From centre to periphery Light red No or delayed blanching Limited branching Do not move with conjunctiva Present Do not spread over cornea Anterior uveitis Acuta glaucoma Keratitis

10. Cause

Passive congestion of conjunctiva Passive congestion of conjunctiva is less frequent in children. They are seen either in mechanical obstruction of venous flow or rarely due to increased blood viscosity. Mechanical causes of passive congestion of conjunctiva can be either in the globe, orbit or systemic. Causes are : 1. Intraocular growth and absolute glaucoma.

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2. OrbitProptosis, exophthalmos-Increased intra thorasic pressure, polycythemia. 3. Systemic. Treatment of hyperemia of conjunctiva is directed towards the cause : Non inflammatory congestion is treated by instillation of local vasoconstrictors. They produce immediate whitening of the eye that may last from few minutes to few hours only to appear again. There may be rebound congestion or tachyphylexis. Inflammatory congestion is best treated either with antibiotics or anti-inflammatory as the case may be. Pigmentation of conjunctiva Commonest pigmentation of conjunctiva seen in children is staining of conjunctiva and sclera by bile pigment in various types of jaundice including neonatal jaundice. Pigmentation of jaundice is bilateral and most marked in the fornix. It does not require any ocular treatment. Other types of pigmentations are deposits of melanin in conjunctiva as a part of congenital melanosis, melanotic tumours of conjunctiva, Addisons disease. Sometimes it is seen in keratomalacia, vernal catarrh and trachoma. External pigments may be deposited in the fornices following prolonged use of local drugs like : Argyrosis following use of sliver containing drops. Adrenaline and many other drugs taken systemically can also cause pigmentation of the conjunctiva. Staining of dry conjunctiva by Kajal or Surma is very common in children in Indian subcontinent. Chemosis of conjunctiva. Chemosis of conjunctiva is a very common feature of conjunctival disorder. It may be caused by (1) Local conjunctival disease, (2) Intraocular disease, (3) Orbital disorder, or (4) Systemic disorder. There is either an increased permeability of arterial blood supply or stasis of venous flow. Impaired lymphatic drainage may also manifest as edema of conjunctiva. Chemosis of conjunctiva is unilateral in case of ocular or orbital causes. It is bilateral when the causative factor is systemic. Chemosis of conjunctiva may be mild giving a glossy appearance to the conjunctiva, moderate when the conjunctiva is lifted from the sclera and has a translucent fluid laden appearance or it may be severe enough to protrude the conjunctiva through the inter palpebral fissure. It may be congested when the causative factor is inflammatory. Chemosis is least marked in tarsal conjunctiva due to the firm attachment of the conjunctiva to the tarsal plate. The fornices may accumulate fluid without being noticed on the conjunctival surface presenting as puffiness of the lid. The lower lid chemosis of bulbar conjunctiva is most marked and obvious. It develops round the cornea like a crater. In severe chemosis the cornea may be hidden in the over hanging chemosed conjunctiva. If the conjunctiva remains exposed for sufficient time it may develop ulcers. Causes of conjunctival chemosis are : 1. Allergy (a) Exogenous allergyAs seen in seasonal allergic conjunctivitis. (b) Endogenous allergen may produce picture similar to angio neurotic oedema. 2. Inflammation (a) Hyper acute conjunctivitisOphthalmia neonatorum, (b) Panophthalmitis,

118 (c) Endophthalmitis, (d) CellulitisOrbit, lids (e) Cavernous sinus thrombosis.

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3. Systemic condition. Hypoproteinemea, thyrotoxicosis, myxedema, emphysema : (a) In the head neck area, (b) fracture of paranasal sinuses, hypersensitivity to drugs, nephrotic syndrome. Treatment (1) In mild cases of chemosis without infection or systemic involvement local vasoconstrictors relieve the symptoms. (2) Acute allergic chemosis may require local steroid drops. (3) In chemosis secondary to infection like ophthalmia neonatorum, endophthalmitis and panophthalmitis treatment is directed towards the primary cause. 4. Systemic causes are best treated in consultation with pediatrician. Subconjunctival haemorrhage. Sub-conjunctival haemorrhage is yet another common symptom that makes alarmed parents to bring the child to the ophthalmologist. The subconjunctival haemorrhage is most alarming when it occurs in the bulbar conjunctiva where a small speck of redness stands out prominently against white sclera. Subconjunctival haemorrhage in the hidden part of the conjunctiva may go unnoticed. Subconjunctival haemorrhage is in fact an intraconjunctival bleeding when blood accumulates between the sclera and deeper layers of conjunctiva. If the same blood breaks the anterior surface of the conjunctiva then it results into conjunctival bleeding. Subconjunctival haemorrhage may be unilateral or bilateral. It may have only conjunctival involvement or may be associated with orbital or systemic condition. Causes of subconjunctival haemorrhage are : 1. Trauma (a) Direct trauma to conjunctiva. Accidental or surgical trauma may range from small foreign body to large lacerated wound. The wound could be blunt or penitrating. Amount of sub conjunctival haemorrhage is not always proportional to the trauma. Trivial injury can give rise to large haemorrhage while large cuts in conjunctiva may give rise to surprisingly small haemorrhage. (b) Trauma to orbit. In fractures of orbital walls, and para nasal sinuses sub conjunctival haemorrhage appear few hours after the actual incident as it takes time for the blood to trickle down either along the orbital wall or along the recti muscles. These haemorrhages appear gradually, spread towards the cornea, hence the outermost limit of these haemorrhages are not visible. (c) Head Injury : (i) Scalp injuries may cause moderate to severe haemorrhage in the conjunctiva. Generally they are associated with ecchymosis of lids and take hours to manifest following injury. (ii) Fracture base of skull is associated with delayed development of such conjunctival haemorrhage in which outer limit is not visible. There may be epitaxis and bleeding from the ears.

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(d) Remote injuries. Multiple fractures of long bone or crush injury of the chest and abdomen may produce sub conjunctival haemorrhage generally as streaks in the fornices. (e) Indirect injury : (1) Rupture of conjunctival vessels : (i) Sudden recurrent rise of intra thorasic pressure may cause rupture of conjunctival vessels. This is most commonly seen in a child with a whooping cough. It is such a constant feature that if there is no history of trauma in presence of sub conjunctival haemorrhage the first thing that should come to mind of the treating physician is that the child is not immunised against whooping cough and must be directed to pediatrician to exclude possibility of whooping cough. (ii) Rupture of telangiectasia may also lead to sub conjunctival haemorrhage. 2. Infection. Some of the conjunctivitises regularly produce sub conjunctival haemorrhages generally as patechea which may join to form large patch of sub conjunctival haemorrhage. The causes arepneumococcal conjunctivitis, membranous and pseudo membranous conjunctivitis, epidemic conjunctivitis, haemophilus conjunctivitis. 3. Systemic conditions. In adults diabetes, hypertension and arterio-sclerosis are main systemic causes of sub conjunctival haemorrhage. In children blood dyscrasias are main systemic causes of sub conjunctival haemorrhage. It is commonly seen in thrombo cyto penic purpura, leukemia and aplastic anaemia or idiopathic. There may be a group of children with sub conjunctival haemorrhage for which no etiological factor can be pinpointed in spite of numerous investigations. These are mostly due to trivial injuries. Treatment. Traumatic sub conjunctival haemorrhage unless associated with laceration of conjunctiva do not require any treatment except reassurance. However the bright red colour against white background is so alarming that patient shift from ophthalmologist to ophthalmologist till the blood clears. If the outer limit of subconjunctival haemorrhage is not visible. A neuro ophthalmic work up should be done along with CT and MRI. In subconjunctival haemorrhage associated with ocular infection or systemic disorder treatment should be directed towards primary disorder. Conjunctival bleeding Frank bleeding from the conjunctiva is relatively infrequent as compared to sub conjunctival bleeding. It happens when conjunctiva is cut or lacerated or when large sub conjunctival haemorrhage ruptures over the surface. A haemangioma or telangiectasia may also bleed into conjunctival sac. A rhinosporidiosis granuloma in the upper fornix may present as recurrent bleeding from the conjunctiva. Conjunctival follicles Conjunctiva is rich in lymphoid tissue, any assault to it microbial or chemical results in its proliferation as small nodules called follicles. They are less frequent in adults and do not develop during first three months of life. Their presence is not always diagnostic specially if they are in lower fornix or lower tarsal conjunctiva. Their presence in upper tarsal conjunctiva denote chronic viral infection and trachoma. Presence of follicles along with conjunctivitis is called follicular conjunctivitis which is very common in children and are mostly self limiting. Folliculosis can be acute or chronic, in the former they last less than one month. If follicle persists for more than one month, the condition is called chronic follicular

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conjunctivitis. Number, size, position and location of follicles depend upon duration, severity and type of conjunctivitis. The size vary between 1mm to 2mm, they are pale, round, raised bodies. They neither contain blood vessels nor blood vessels pass over them. Causes of follicle formation are : Trachoma, simple follicular conjunctivitis in children and drugs. Treatment. Follicles themselves do not require any treatment. Treatment should be directed to primary cause specially trachoma. Conjunctival papillae Papillae formation is a non specific vascular reaction to infection and allergy in contrast to lymphoid reaction of the follicle. A papilla develops when the conjunctiva gets anchored to either tarsus or limbus by fine fibrous septa. A tuft of blood vessels grow from beneath to reach the basement membrane of the conjunctival epithelium. This vessel then radiates like spokes of a wheel. The papillae are mostly seen on the upper tarsus and near the limbus. They are larger than follicles. Size of papillae vary from 1mm to 5mm. When papillae are small the conjunctiva is velvety and smooth. A hyperemic papillae represent bacterial and chlamydia infection. Large polygonal flat topped papillae in upper tarsus are seen in vernal conjunctivitis. Papillae in lower tarsus is seen in atopic conjunctivitis. Limbal papillae are seen in limbal form of spring catarrh. They are not seen over bulbar conjunctiva or caruncle. Scarring of conjunctiva Conjunctiva being highly vascular and lax at mostof the places. Scarring is less common in bulbar Conjunctiva unless the wound has not been repaired well. To avoid ugly scar in bulbar Conjunctiva, a lacerated conjunctiva should be repaired in layer i.e. conjunctiva and Tenons capsule separately. Stitching should approximate the cut ends properly. Scarring in palpebral conjunctiva is seen best in the tarsal conjunctiva as opaque star shaped areas that obscure the tarsal gland and its ducts commonly in trachoma. Scarring of fornices is seen following mechanical trauma, acid, alkali burn, Stevens Johnsons syndrome and ocular pemphigus. The scarring may be strong enough to produce deformity of the lid resulting in entropion in adults. Conjunctival discharge Normal conjunctiva is always wet due to constant flow of tears. Any irritation of conjunctiva results in reflex production of tear in excess but this does not contain any formed matter like mucus, cells, or pus. Presence of discharge is indication of conjunctival inflammation microbial or allergic. Conjunctival discharge contains exudated fluid from dilated vessels. It also contains tear, mucus, pus, micro-organism, inflammatory cells and sometimes RBC. Conjunctival discharge varies in character in different types of conjunctivitis. Watery discharge is seen in viral conjunctivitis, and chemical conjunctivitis of short duration. If there is super added bacterial infection the discharge becomes thick with more mucus and exudate. Muco purulent discharge is seen in milder form of bacterial conjunctivitis. Purulent discharge is characteristics of gonococcal conjunctivitis. Ropy discharge is typical of spring catarrh. Mucinous discharge is seen in some of the dry eye syndromes.

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Cytology of discharge. Identification of cells in discharge have diagnostic significance. Predominant polymorphonuclear cells in bacterial conjunctivitis. Lymphocytic preponderance is met in chronic conjunctivitis, viral conjunctivitis and toxic conjunctivitis. Eosinophils are seen in allergic conjunctivitis. Conjunctival membrane formation Deposition of coagulated material transudate on the surface of the conjunctiva in a sheet form is called conjunctival membrane. It has been divided in to two types : 1. True membrane, where the coagulum penetrates the conjunctival epithelium and can not be peeled off without tearing the conjunctival epithelium which in turn produces bleeding. 2. Pseudo membrane is a milder form where the sheet can be removed with ease without bleeding. The pseudo membrane is in fact a milder form of inflammation and the same organisms that produce pseudo membrane may, in severe form produce true membrane. Previously diphtheria was the only organism thought to be capable of true membrane formation. The organism that produce pseudo/true membrane could be viral, bacterial, fungal or chemical. The organisms are - Herpes simplex, epidemic kerato conjunctivitis, diphtheria. It may be seen in erythema multi formis, and some alkali burns. Severe form of membrane formation may not only cover the conjunctiva but also may hamper its nutrition. End-result of membranous conjunctiva is xerophthalmia, symblepharon, ankyloblepharon, trichiasis and rarely entropion. Granulomas of conjunctiva Granulomatous lesions of conjunctiva may be due to either exogenous causes or endogenous causes, latter are more common. Exogenous cause of conjunctival granuloma are - Embedded foreign bodies, both organic and inorganic. They may be inert or chemically active. They may be small wooden particles, wings and other parts of insect body, caterpillar hair (ophthalmia nodosa), hair from cacti, suture material and scleral implants. Endogenous granulomas are seen in Tuberculosis, leprosy, syphilis, oculosporidiosis, burst chalazia. Conjunctival ulcers are rare, mild and self limiting. They are seen in tuberculosis, herpes simplex, Stevens-Johnsons Syndrome, chemical burn. Cysts of conjunctiva Cysts of conjunctiva can be part of congenital anomaly or acquired. They may be seen anywhere on the conjunctiva but commonest sites are the fornices and limbus. They may be small enough not to be noticed by the patients or large to draw attention, generally they are painless, non tender, non reducible, translucent, may be punctured only to get filled. Conjunctival cysts may be : 1. Congenital, 2. Acquired. 1. Congenital cystsThese are rare, they can be cystic form of limbal dermoids or congenital corneo scleral cysts. 2. The acquired cysts can be traumatic or non traumatic. A. Traumatic cysts : (i) Accidental. Following lacerated conjunctival wound that has not been repaired properly.

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(ii) Surgical wounds in squint, retinal detachment, pterygium which have not been repaired well. (iii) Cystoid cicatrix following glaucoma surgery or badly repaired wound at limbus. (iv) Cyst over iris prolapse. (v) Conjunctiva covering prolapsed, vitreous and uvea. (vi) Phacocele. This is seen when there is corneo scleral injury and the lens with intact capsule is extruded under the conjunctiva. It is not a true cyst. B. Non-traumatic cyst : (i) Lymphatic cystThese are generally small cysts anywhere in the conjunctiva without symptom, do not require treatment. (ii) Degenerative cystSometimes pterygia undergo cystic degeneration at apex. (iii) Parasitic cystCysticercus and hydatid cyst. (iv) Retention cyst.

INFLAMMATION OF CONJUNCTIVA
Conjunctivitis is commonest eye disease seen in children. It may be seen soon after birth as ophthalmia neonatorum that is very serious in nature and if not attended, may be vision threatening or may be just mild hyperemia. Conjunctivitis may be acute, sub-acute or chronic. They are mostly bilateral. In children they are diffuse. Most of them are self limiting. There are two main groups of conjunctivitis i.e. Microbial and Allergic. Besides this there is a large group of conditions where exact etiology is not known. Some of the systemic diseases may also produce conjunctivitis. Microbial conjunctivitis are generally due to exogenous organisms, while allergic conjunctivitis can be both due to exogenous or endogenous allergen. Microbial (Infectious) conjunctivitis can be caused by Bacteria, virus, chlamydia fungi, parasites. Bacteria causing conjunctivitis can be cocci, bacilli, mixed, may be gram positive or gram negative. However conjunctivitis is more frequent with cocci than, bacilli. Common bacteria that produce conjunctivitis are : 1. Neisseria gonorrhoea and N. meningitis, both produce purulent conjunctivitis, former is more serious. 2. Pneumococci, Koch-Weeks (H aegypti). They produce acute muco purulent conjunctivitis. 3. Haemophilus influenzae produces subacute conjunctivitis. 4. Staphylococcus aureus produces chronic blephroconjunctivitis. 5. Coryy bacterium diphtheriae produces membranous conjunctivitis. 6. Neisseria gonococci and C. diphtheriae may have serious systemic involvement in children. 7. Many of the non pathogenic bacteria may become pathogenic and cause conjunctivitis.

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Viruses responsible for conjunctivitis in children are : Herpes simplex, herpes zoster, measles, chicken pox, pharyngo conjunctival fever (adeno virus 3 and 7), epidemic kerato conjunctivitis (adeno virus 8 and 19), acute haemorrhagic conjunctivitis, molluscumcontagiosum. CONJUNCTIVITIS Conjunctivitis in children differ from those in adult. Some of the conjunctival infections like ophthalmia neonatorum and diphtheretic conjunctivitis are not only vision threatening, they can be life threatening also. Best classification of conjunctivitis would be on the basis of etiology. However some of the organisms produce identical clinical picture and are amenable to same treatment, hence for the sake of convenience infectious conjunctivitis will be discussed according to their mode of presentation i.e. Acute, sub-acute, or chronic under two broad heads i.e. Bacterial and Viral. Rest of the conjunctivitis would be discussed under individual heads. Bacterial conjunctivitis According to mode of onset, acute infectious conjunctivitis can beCatarrhal, muco purulent, and membranous. Catarrhal conjunctivitis is very common among children. They involve both eyes which are red and there is minimal discharge that is mostly serous in nature. There may be accumulation of discharge near the inner canthus during sleep. Occasionally the lids may get glued in the morning. It is a self limiting disease caused by bacteria of low virulence. There are no corneal complication, no lympadenopathy. Muco purulent conjunctivitis This is more severe form of the former. Whole of the conjunctiva is bright red. The congestion is most marked in the fornices and fade towards cornea. Lids are slightly oedematous. There is no lymphadenopathy. There is muco purulent discharge with watering. The discharge may accumulate on the lashes which get matted together. The lids may get stuck in the morning causing discomfort, warm swabs may be needed to separate the lids. Generally there is no pain, have mild discomfort. Excess of watering, blepharospasm, foreign body sensation and occasional pain with onset of congestion round the limbus denotes corneal involvement that vary from superficial keratitis, marginal keratitis to frank ulcer. In severe infection there may be formation of pseudo or true membrane. In cases of pneumococcal infection there may be patecheal haemorrhage. Mucopurulent conjunctivitis is a contagious disease, transmitted directly by discharge. The disease is caused by many organisms, generally single organism is responsible for the disease but mixed infection is not uncommon. Common organisms that produce muco purulent conjunctivitis are : Staphylococci, Koch-Weeks bacilli (H. aegypti), pneumo cocci, strepto cocci, rarely pseudomonas. Pneumococci and haemophilus influenzae are more common in children than other organisms. They are frequent between three to eight years of age. Management. Management is to take a conjunctival smear from the lower fornix for Grams stain examination, culture and sensitivity. A smear stained with grams stain will

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render it possible to find out if the organism is cocci or bacilli, is it gram positive or negative, is it intra cellular or extra cellular and accordingly antibiotic can be started. However when facility of smear examination is not available, clinical judgement should be utilised to choose antibiotic for local instillation. 1. Commonly used antibiotics are : Chloramphenicol 0.3-0.5% solution and 1% ointment ; Gentamycin 0.3% drops ; Framycetin 0.5% drop ; Tobramycin 0.5% dropsciprofloxacein 0.3%; Sulphacetamide 20% drops. 2. The discharge from the lashes and mucus from the fornices are removed. 3. There is no need to irrigate the conjunctival sac. Any of the above drops are instilled one drop at a time in the lower fornix four to six times a day for a week. If the lashes have tendency to stick, same antibiotic in the form of ophthalmic ointment is applied in the lower fornix. There is no need of systemic antibiotic, however, if cornea is involved more vigorous treatment is required along with cycloplegic. Neomycin eye drops are better avoided as ten percent of children are sensitive to neomycin. Similarly combination of two or three drugs should only be used if culture shows mixed infection and sensitivity to multiple drugs. Sub-acute and chronic bacterial conjunctivitis Sub acute conjunctivitis is caused mostly by haemophilus influenzae and less commonly by E. coli and proteus. Chronic conjunctivitis is usually caused by staphylococcus aureus and less frequently by all other organisms that cause acute micro-purulent conjunctivitis, Half hearted treatment in the form of irregular drops in sub-clinical strength and frequency besides or resistance to the antibiotics are other factors to cause chronic conjunctivitis. Other contributory factors areAssociated allergy, smoke, dust, uncorrected errors of refraction, under nourishment etc. The conjunctiva on casual inspection may look normal but on pulling the lower lid down the fornices are congested. The discharge is mild, the lids rarely stick in the morning. Chronic blepharitis is very common in staphylococcal conjunctivitis and ulceration in the lower part of cornea, fortunately it is less common in children. Differential diagnosis of chronic bacterial conjunctivitis includes Trachoma, chronic follicular conjunctivitis, seasonal allergic conjunctivitis. Treatment. Treatment consists of complete elimination of causative organism and prevention of recurrence. For complete elimination it is necessary to find out the causative organism and the antibiotic to which it is sensitive. Both drops and ointments are used for weeks. Special attention should be paid to lid hygiene, correction of error of refraction and malnutrition.

SOME ACUTE BACERIAL CONJUNCTIVITIS IN CHILDREN


Membranous and pseudo membranes conjunctivitis. These two types of conjunctivitis have acute onset and are bilateral. Mode of clinical presentation in the two forms is

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almost same. Membranous conjunctivitis is more severe form of the two. Though many organisms produce membranous conjunctivitis, diphtheretic conjunctivitis is more important than others due to its systemic complication. Diphtheretic conjunctivitis9,10. This form of conjunctivitis was very common when immunisation against diphtheria was not available. It used to occur in epidemics. It still occurs in non immunised children. Sometimes it can occur in immunised children in a milder form. However it should be remembered that there are other causes of membranous conjunctivitis that may mimic as diphtheria conjunctivitis. Commonest age to develop diphtheretic conjunctivitis is between 2 years to 8 years. Diphtheric conjunctivitis may present as pseudo membranous conjunctivitis as well but incidence of pseudo conjunctivitis is less than true membranous conjunctivitis indiptheria. Clinically diphtheretic conjunctivitis can be divided into (1) Stage of infiltration, (2) Stage of membrane formation, (3) Stage of cicatrisation, (4) Corneal involvement, (5) Extra ocular muscle palsy. 1. Stage of infiltration. Generally bilateral, the lids are swollen, hard and temperature is raised. They are tender, board like rigidity is classical of diphtheretic conjunctivitis. The lid can not be separated or everted. The pre auricular lymph nodes are enlarged. It is generally associated with diphtheretic membrane of nasopharynx. 2. Membrane formation. Changes in conjunctiva go along with the changes in the lid. In fact the changes in lid are mostly due to changes in tarsal conjunctiva. A true membrane develops over both the palpebral and tarsal conjunctiva. In severe cases the membrane may slough off leaving a raw bleeding surface. No attempt should be made to peel off the membrane as its bleeds profusely and leave a raw area. 3. Cicatrisation. This follows as the membrane heals and shrinks. The resulting scar may produce xerophthalmia, symblepharon, trichiasis and rarely entropion. 4. Corneal complication. Involvement of the cornea occurs due to two factors : (1) The bacteria can invade intact epithelium without trauma and cause corneal ulceration and perforation. (2) The corneal nutrition suffers due to widespread thrombosis of peri limbal blood vessels. 5. Extra ocular muscle palsy is due to involvement of third, fourth and sixth cranial nerves either as isolated palsy or in combination. There may be paralysis of accommodation and iridoplegia. There can be paralysis of convergence or divergence. Management : 1. All cases of membrane formation on conjunctiva true or pseudo must be treated as diphtheretic unless proved otherwise. 2. As diphtheria produces a potent toxin that can be neutralised by antitoxin only before it gets fixed to the tissues. Antitoxin is administered systemically and locally in consultation with physician. 3. The toxin is not neutralised by antibiotic. Systemic antibiotic only reduces number of bacteria thus diminishing the source of toxin production. It also eliminates other bacteria that are seen frequently in diphtheretic conjunctivitis. The drugs commonly

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used are - Penicillin G 600,000 IU BD for ten days, Erythromycin in dose of 25 mg to 50/kg for days. Other drugs used are ampicillin, clindamycin and tetracyclin. Local treatment consists of : 1. Local administration of antitoxin in the conjunctival sac between 10,000 to 100,000 IU for 24 to 48 hours. 2. Local instillation of fresh solution of Penicillin, Erythromycin and ointment along with antitoxin. 3. Cycloplegic should be used least keratitis develops. Once corneal involvement is noticed it should be treated vigorously. Other causes of membranous and pseudo membranous conjunctivitis : Cause Streptococci E Coli H. Influenzae Pneumococci Staphylococci Gonococci Actinomycosis Candidiasis Epidemic kerato conjunctivitis Herpes zoster Haemorrhagic conjunctivitis Pharyngo conjunctival fever Infectious mono necleosis Erythema multi formis Alakali burn Ligneous conjunctivitis Ophthalmia Neonatorum11,12,13 Ophthalmia neonatorum or conjunctivitis in new born is a serious infection of new born. Any conjunctivitis developing within first month of life is called ophthalmia neonatorum. It is a notifiable disease. Etiology comprises of three groups of causative agents i.e. Bacteria, viruses and chemical. Out of the above three, the bacterial conjunctivitis in neonate is most common. The condition is potentially vision threatening if not managed in time. Previously Gonococcus14 was considered to be commonest organism to cause ophthalmia neonatorum. With better health care and antenatal screening of the mother, incidence of gonococcal ophthalmia neonatorum has come down considerably. However it remains an ocular emergency. All Membrane ++ ++ + + + + + + + + + + + + + Pseudo membrane + + + + + + +

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purulent conjunctivitis in neonate must be considered as gonococcal unless proved otherwise.2 Bacteria other than gonococci that produce ophthalmia neonatorum are : Staphylococcus, pneunococcus, haemophilus3, pseudomonas, and streptococcus, in fact any bacteria that may find its way to conjunctiva of new born can cause purulent conjunctivitis. Out of all bacteria, gonococcus has most severe form of infection and has shortest incubation period of 23 days. It is seen in children born to mothers who suffer from gonococcal infection of birth canal or there is contamination during delivery from sources other than birth passage. It is a bilateral condition that starts as redness of the conjunctiva which is often missed because the infant keeps the eyes closed almost throughout the day and night and has a relatively narrow inter palpebral aperture. The disorder is noticed only after there is out pouring of muco purulent discharge from the eye. The lids are swollen, rigid and may stick together. On separation of the lids that may require use of lid retractor the conjunctiva is chemosed, may protrude through the lids. There may be blood discharge, formation of true or pseudo membrane. Corneal involvement is most serious vision threatening findings. Loss of lustre of cornea is a bad sign. There may be corneal infiltration, central corneal ulcer which may perforate in very short period. Gonococcus is one of the few organisms that can pass through intact corneal epithelium. Once perforation has occurred endophthalmitis and panophthalmitis is very common. Otherwise a small perforation may heal by formation of central leucoma, leucoma adherent and anterior pallor cataract. The eye soon develops nystagmus. Even if there is no perforation gonococcal uveitis is very common. Neonatal conjunctivitis by other bacteria are milder than gonococcal, they have longer incubation period i.e. 4-5 days and are generally due to secondary infection after birth. pseudomonas infection is more common in prematures. Incidence of gonococcal conjunctivitis in neonates has shown a gradual decline due to better maternal and child care and use of prophylactic anti microbial. Non bacterial micro organism that produce ophthalmia neonatorum are Chlamydia trachomatous and herpes simplex. Incidence of neonatal conjunctivitis by Chlamydia and herpes simplex is showing an upward surge. Chlamydia caused inclusion conjunctivitis, takes five to seven days to develop. Source of infection is birth canal. It is far milder than bacterial conjunctivitis. Corneal involvement is milder in the form of micro pannus that may cause permanent scarring. It never perforates. Herpes simplex conjunctivitis is also acquired from infected birth canal. If the mother has active lesion of genital passage a caesarean sections saves the child from getting conjunctivitis. It develops after 10 days as mild conjunctivitis, watery discharge, diffuse or dendritic keratitis. There may be associated skin vesicles. Diagnosis of ophthalmia neonatorum specially bacterial is not difficult. All cases of mucopurulent bilateral conjunctivitis should be considered to be due to gonococcus unless proved to be otherwise. Presence of gonococcus is confirmed by Grams stain and preferably by positive culture in suitable media (Thayer Martin medium). In all cases of purulent conjunctivitis a conjunctival smear should be taken and stained by Gram and Giemsa stain to see if it is gram positive or negative. It also gives nature of intra cellular or extra cellular inflammatory cells i.e. polymorph, basophils, lymphocytes and inclusion bodies. Gram negative intracellular diplococci and polymorpho nuclear neutrophils indicate gonococcal infection. This should be sufficient to start anti gonococcal treatment but

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to find out if the stain is penicillin resistance or not and its sensitivity to other antibiotics, culture and sensitivity test must be done because resistant stain may prove to be life threatening. Management consists of(1) Prophylaxis, (2) Treatment of various microbial ophthalmia in new born. 1. Prophylaxis15,16. This includes (a) Antenatal screening of mother for evidence of gonococcal, chlamydia and herpes simplex of genital tract. If found to be infected, proper treatment should be initiated. In case of herpes simplex of birth canal an elective caesarean section may be done. (b) Complete antisepsis and asepsis should be followed during and after delivery. The eyes of new born should be cleaned by two separate wet cotton swabs, one for each eye from lateral to medial side. (c) Prophylactic anti microbial drop : (i) 1% silver nitrate drop (Creds prophylaxis) One drop of freshly prepared solution (not more than seven days on the shelf) that is stored in coloured bottle and kept in cool place is instilled in each eye. Credes prophylaxis does not always eliminate possibility of ophthalmia neonatorum. It reduces severity of the condition. Credes method was universal prophylaxis against ophthalmia neonatorum but has been replaced by antibiotics because AgNO3 itself can cause chemical conjunctivitis that mimics ophthalmia neonatorum if used in concentration more than 2% and instillation more than two drops in each eye. This chemical conjunctivitis develops earlier than any other conjunctivitis i.e. within 24 hours and is mild and self limiting. Causes redness of conjunctiva, edema of lids discharge and redness of surrounding skin. (ii) PovidonIodine 2.5% has been claimed to be better than AGNO3 without side effects of latter. (iii) Antibiotic. Any of the following antibiotics can be used as drops or ointment 0.3% Ciprofloxicin, 1% Tetracyclin, 0.5% erythromycin, 0.3% gentamycin. In suspected chlamydia infection of the mother 10% sulpha cetamide may be added to any of the above. (iv) If the mother is known to suffer from gonococcal infection, the neonates are at high risk of developing ophthalmia neonatorum. Such infants should get single intra muscular injection of 50,000 IU of crystalline penicillin or single dose of 1m injection of ceftriaxone 125 mg or cefotaxime 100 mg of course the mother also gets sufficient systemic antibiotic. Management of clinically established gonococcal conjunctivitis13, 16 in neonates comprise of : 1. Cleaning of muco purulent discharge frequently using separate swab for each eye and each cleaning. Irrigation with balanced solution have doubtful efficacy. 2. Instillation of fresh aqueous solution of crystalline penicillin G. in strength of 100,000 unit per ml. every five minutes for six times, then every ten minutes for six instillations, then one hourly for six instillation. Then every two hourly. As ointment of penicillin is no more available erythromycin 0.5% may be put to reduce stickiness of lids. Other drugs like 0.3% Ciprofloxicin, gentamycin 0.3% or any ophthalmic drop that is effective against gonococci may

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be used in the same frequency. Recent studies have started doubting need and efficacy of local antibiotic drops. They prefer systemic administration of systemic crystalline penicillin twice a day for seven days or cefotaxime in neo natal dose in consultation with neonatologist. 3. If cornea shows any evidence of involvement 0.5% atropine ointment is put to treat and/or prevent associated uveitis. Atropine drops are better avoided as it is likely to be absorbed from nasolacrimal passage and throat in amount sufficient to produce systemic toxicity. Treatment of other bacterial conjunctivitis : (a) Pseudomonas conjunctivitis requires as prompt treatment as gonococcus specially in pre mature infantsTopical gentamycin or tobramycin in strength of 0.3% as in case of gonococcus with ointment of the same antibiotic three to four times recommended. (b) Haemophilus is best treated either by local gentamycin drops or 10-15 percent sulphacetamide drops. (c) Conjunctivitis with other gram positive cocci and diplococci is treated with 0.5% erythromycin every 2 hourly. (d) Coliform organisms can be treated with gentamycin drops. (e) Acineto bactor17. In recent years hyper acute conjunctivitis similar to ophthalmia neonatorum has been reported from various parts of the world. The organism belongs to family Neissericeae. It is difficult to differentiate it on grams stain from gonococcus, both are gram negative cocci. The acinetobactor fortunately does not cause systemic infection but is penicillin resistant. It is best treated by local 0.3% ciprofloxacein or 0.3% Tobramycin. (f ) Chalmydia conjunctivitis in neonate has become more common than in the past. It is milder than bacterial infection but may have long term local as well as systemic effect i.e. otitis, rhinitis and pneunonitis. Diagnosis of chlamydial conjunctivitis is confirmed by Giemsa stain that shows besophilic cytoplasmic inclusion bodies. Treatment comprises of instillation of 10% sulpha-cetamide drop and erythromycin 0.5% ointment 4 times a day in severe cases erythromycin syrup in dose of 50mg/kg/day in 4 divided doses is given for 2-3 weeks. Herpes virusconjunctivitis is uncommon but it is potentially vision and life threatening infection in neonate, untreated cases may be fatal in as many as 50% of cases. Presence of skin vesicles may point towards diagnosis of herpes simplex. Treatment with systemic anti viral drugs is needed in consultation with neo-natologist. Pneumococcal conjunctivitis Pneumococci are generally present in respiratory system, but can be found as a symptomatic strain in conjunctival sac. It gradually produces systemic infection with ocular involvement and is a common cause of hypopyon. It is one of the commonest organisms that cause chronic dacryocystitis. It can involve the conjunctiva independent of systemic involvement. It is mostly seen in children as acute muco purulent conjunctivitis, with patecheal haemorrhage. It can cause ophthalmia neonatorum, membranous or pseudo membranous conjunctivitis. It is

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spread by hand to hand contact in children. Management is similar to any other bacterial conjunctivitis. Commonly used antibiotics are : 1. Fortified aqueous penicillin G. solution containing 100,000 unit per cc every hourly during day and every four hourly by night, till the acute symptoms subside. 2. Erythromycin 0.5% may be given at night as ointment. 3. Becitricin 10,000 units per cc can also be given as hourly drop. 4. Cefazoline 50mg/cc may be given in place of penicillin. Systemic antibodies do not seem to have any advantage over local instillation. However every child suspected to have pneumococcal conjunctivitis should have a pediatric consultation for possible systemic involvement. Corneal involvement consists of a central ulcer with or without hypopyon. All cases of suspected corneal involvement should be treated as emergency with atropine, vigorous antibiotic instillation and beta blockers. Haemophilus influenzae18 and H. aegyptius, Koch-Weeks bacteria are common causes of conjunctivitis in children, with acute onset that lasts for about 10 days. that can cause patchy sub conjunctival haemorrhage. Inferior corneal infiltration is common, can have serious systemic involvement and peri orbital cellutitus. The organism is best grown on chocolate agar medium.
Other Bacterial Conjunctivitis Bacteria Streptococcus Conjunctival feature Acute bilateral mucopurulent conjunctivitis membranous or pseudo membranous conjunctivitis ophthalmia neonatorum Staphylococcus Hyperemia, papillary reaction chemosis purulent or mucopurulent conjunctivitis E. Coli Hyperemia, chemosis, acute mucopurulent conjunctivitis membrane or pseudo membrane Keratitis Gas in AC Keratitis Erythromycin, sulpha cetamide. bacitricin cefazolin Chlormphenicol Gentamycin, Tobramycin Other feature Keratitis, and orbital cellulitis Treatment Local penicillin drop. erythromycin drop. bacitricin.

Viral conjunctivitis Viral conjunctivitis in children can be (1) Acute or (2) Chronic. 1. Acute (i) Herpes simplex. Conjunctiva is involved in children as primary infection mostly by type I but occasionally by type II virus as well. There are three age groups in which herpes simplex involves a pediatric patients i.e. (1) Neonates, (2) Between six months to 5 years, (3) Teenage. Neonatal herpes simplex conjunctivitis can be congenital or acquired. The former is, rare and associated with severe systemic involvement hence most of the time fatal. In acquired form the neonate gets infection during passage through birth canal.

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The child between 3 to 5 years gets infection from infected adults. The teenager gets it by kissing an infected person and rarely as sexually transmitted disease. Herpes simplex conjunctivitis irrespective of age of onset have cutaneous, conjunctival and corneal involvement in various combinations. It is generally unilateral, incubation period is 3 to 12 days. It develops as follicular conjunctivitis (after three months of age) with lymphadenopathy, the eye is congested. There is watery discharge. Onset of photophobia denotes corneal involvement. There may be pseudo membrane formation. The corneal involvement starts as small epithelial lesions that join together to form typical dendritic keratitis. The skin lesion consists of vesicles over the skin of the lid, forehead, lid margin. The vesicles may cross over the mid line. The infection is self limited, it lasts for two to three weeks. It is difficult to diagnose clinically. As herpes simplex conjunctivitis is a primary infection, the virus may pass into the trigeminal ganglion and remain dormant, recur as herpetic keratitis later. Treatment. As it is a self limiting disease, it does not require any specific treatment. Corneal involvement require local use of anti viral drugs along with cycloplegic. Local antibiotics are prescribed to prevent secondary bacterial infection. Steroids are contra indicated. (ii) Herpes zoster conjunctivitis. Herpes zoster conjunctivitis is caused by vericella zoster virus. It is less common in children but children are not immune. The disease has more serious corneal and lid involvement than herpes simplex. Conjunctivitis is muco purulent always associated with vesicle formation on the lid. There may be actual vescicles on the conjunctiva. The conjunctivitis to beginwith is papillary, there may be follicle formation. In severe cases pseudo membrane develops. Conjunctivitis is self limiting and resolves in a week. Tender pre auricular nodes develop in early stage of disease. It is always unilateral. There may be secondary bacterial infection. Treatment. Conjunctivitis does not require any specific treatment. Treatment is directed towards the disease in toto. (iii) Pharyngo conjunctival fever. Conjunctivitis in pharyngo conjunctival fever is very common in children. It is a part of syndrome consisting of fever, pharyngitis, pre auricular non tender lymphadenopathy. The conjunctivitis is follicular in nature, generally unilateral may become bilateral generally there is a watery discharge. Mucopurulent discharge denotes superimposed bacterial infection. The lids are generally edematous. Cornea may be involved in the form of superficial fine epithelial keratitis. The causative viruses are adeno virus 3 and 7. Sometimes adeno virus 4 may also cause pharyngo conjunctival fever and conjunctivitis. It sometimes develops following bath in a contaminated swimming pool. Incubation period is 5 to 12 days. Treatment. Conjunctivitis is self limiting lasting for seven to fifteen days. However corneal infiltrates may persist for a month. Treatment is non specific and symptomatic. (iv) Epidemic kerato conjunctivitis. This contagious conjunctivitis is common in children. It is caused by adeno virus 8 and 19. Other sero types may also cause the disease. In children it is associated with sore throat, fever and diarrhoea. It has incubation period of 5 to 12 days. One eye is first involved and more severe than the other eye. Conjunctivitis is

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follicular in nature, there may be papillary reaction and pseudo membrane formation. Pre auricular lymphadenopathy is a common feature. The disease spreads among the school children in epidemics. Occasionally it may be spread by contaminated instruments, lotions or drops following ocular examination. Corneal involvement is common, it presents as sub epithelial opacities that may appear after conjunctivitis subsides and may persist for months. Treatment is symptomatic. No anti-viral drugs are required. (v) Acute haemorrhage conjunctivitis. This acute conjunctivitis was first noticed in 1969 and has been known by various eponyms. The epidemics keep on appearing at regular intervals mostly in the months of late June and early July. It is caused by entero virus type70. The incubation period is short i.e. 12 to 48 hours. It is most commonly seen in crowded localities i.e. schools etc. It has an acute onset first in one eye with in few hours the other eye gets involved. The symptoms are intense redness of conjunctiva with copious watery discharge, lid edema and pain ranging from mild discomfort to severe pain. Sub conjunctival haemorrhages are constant feature which generally start as small spots on the periphery of the bulbar conjunctiva later whole of the conjunctiva gets involved. These spots may join together and form a large sub-conjunctival haemorrhage. The patechea disappear early, large haemorrhage take ten to fifteen days to clear. Sometimes there may be chemosis of conjunctiva and follicle formation. There may be lymphadenopathy. Onset of corneal involvement in form of epithelial keratitis heralds photophobia. Conjunctivitis may be associated with fever, malaise, and body ache. Occasionally there may be uveitis, motor paralysis of lower limbs have been reported. The virus is transmitted by close person to person contact and by fomites, water, common linens or ophthalmic instruments used to examine infected eye. Treatment. There is no specific treatment known. The disease does not give immunity. Recurrence in the same patient has been observed. Treatment is directed towards symptoms. Non steroidal anti inflammatory drugs are given to relieve pain. Dark glasses help in reducing photophobia. It is a misconception that dark glasses prevent spread. Broad spectrum antibiotics are given to prevent and treat associated secondary bacterial infection. The disease is self limiting lasting for seven to ten days. In absence of corneal involvement weak solution of steroid drops may shorten duration. Other viral infections that produce conjunctivitis in children : ViralConjunctival lesions. InfluenzaCatarrhal conjunctivitis, sub conjunctival haemorrhage. MumpsPapillary or follicular conjunctivitis, subconjunctival haemorrhage. MeaslesKopliks spot, follicular sub conjunctival haemorrhage. Chicken poxMuco purulent conjunctivitis, vescicle formation, phlycten. Molluscum contagiosumChronic unilateral conjunctivitis, pannus formation, follicle formation, umblicated vesicle may be seen. Chlamydia infection in children19 Chlamydia (formerly known as Bedsonia) are a group of micro-organism in between bacteria and viruses. They are intracellular organisms that are difficult to culture. Their

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intracellular nature gives them partial immunity against drugs. Hence drugs are to be administered for long time. They multiply by binary fission. Common chlamydiae are : 1. C. Trachomatis, 3. C pneumonae and 2. C psittacosis, 4. C. Lympho granuloma venerum.

The former two produce kerato conjunctivitis in children. Out of C Trachomatis and C. Psittacosis former is more common cause of ocular manifestation while latter produces more systemic infection. C. Trachomatis is gram negative organism that is sensitive to sulphonamide, it is almost exclusively human pathogen. C. Trochomatis has many types i.e. A, B, Ba, C that produce trachoma and D to K that produce inclusion conjunctivitis also called paratrachoma. The organism is also known as TRIC where TR stands for trachoma and IC stands for inclusion conjunctivitis. Inclusion conjunctivitis effects patient in two distinct age groups : 1. Neonate, 2. Young adults. Para trachoma causes urethritis, cervicitis in adults and secondary conjunctivitis in adults which resembles trachoma in many aspects but is milder than trachoma. it is a sexually transmitted disease. Neonatal chlamydiae disease is acquired by new born during birth via infected birth canal of the mother. It produces nongonococcal ophthalmia neonatorum. The incubation period is longer than gonococcal ophthalmia i.e. 5 to 12 days. There is lacrimation and serous discharge, the lids are swollen. It is not prevented by usual Crede's prophylaxis but can be prevented by tetracyclin or erythromycin. Untreated cases heal in four to five months but may persist for years causing chronic follicular conjunctivitis and pannus formation. Infants with inclusion conjunctivitis may develop pneumonitis and gastro-enteritis in first six months of life. Trachoma20, 21, 22 Trachoma is a chronic kerato conjunctivitis due to C Trachomatous sero type A, B, Ba and C. It is commonest preventable infectious disease of eye that may otherwise lead to blindness in adults. It is mostly acquired in childhood. Uncomplicated trachoma in children do not cause blindness, however secondary bacterial infection, associated malnutrition and xerophthalmia may lead to corneal ulceration, perforation and loss of eye in children. No race is immune. It is a bilateral condition. In children, boys and girls are equally affected. In adults women have more serious involvement than men. Trachoma is a disease of dry, dusty climate. It is a disease of poor children with poor hygiene. Improvement of health care and awareness of cleanliness has drastically reduced incidence of disease in areas where it used to be endemic. The children get trachoma from infected family members, and playmates. The disease is transmitted from person to person by fomites. Contaminated KAJAL, SURMA and flies play an important part in spread of trachoma in children. It has been noticed that reduction in fly population, regular cleaning of face, availability of free flowing water and ownership of flush latrine in the family reduce spread of trachoma.29 Clinical feature of trachoma Clinical features of trachoma in children differ from those in adults. The trachoma does not reach the cicatricial stage in children as it takes more than decade to develop cicatricial stage. However, most of adults acquire trachoma in childhood.

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Incubation period of trachoma is short i.e. seven days (range 5 to 12 days) It begins as mild conjunctivitis similar to bacterial conjunctivitis and indistinguishable form it. Smear from conjunctival sac in a case of pure trachoma does not grow bacteria on culture. Symptoms. Symptoms are mild in children unless secondary bacterial infection is superimposed. Symptoms are so common in endemic areas that it is taken as natural phenomenon. Symptoms consist of watering, discharge, redness of the eye, moderate swelling of the lids, pre auricular nodes may be enlarged. Signs. Conjunctival congestion, follicular hypertrophy mostly in the fornices and upper part of tarsal conjunctiva, papillae formation, superficial keratitis in the upper part and mild vascularisation. McCallau23, 23A classified the conjunctival signs into following grades : (1) Incipient trachoma, (2) Established trachoma (3) Cicatrising trachoma, (4) Healed trachoma. Various stages are : Stage I : Immature follicles in upper palpebral conjunctiva. Stage II (a) : Prominent follicular hypertrophy. (b) : Papillary hypertrophy masking follicular hypertrophy. Stage III : Follicles and scarring at upper tarsal conjunctiva. Stage IV : Healed trachoma. No sign of inflammation and late scarring of tarsal conjunctiva. Stage III and IV are generally not seen in chidden under fifteen years Above classification has been followed for more than half a century. The classification takes into consideration only conjunctival changes. No weightage is given to keratopathy and ; which have great prognostic value. WHO 198724 adopted a better classification.
Stage of Trachoma Trachoma free Trachoma dubium doubtful trachoma Trachoma stage I Nil Like early trachoma but not confirmed Immature follicles on upper tarsal conjunctiva early corneal stage. Established florid trachoma mature follicles. Papillary hypertrophy follicles or Herberts pits at limbus. Cicatrising trachoma. Conjunctival scar. Some of the signs of stage I and II. No inflammation, disease no longer infectious. Scars present. Clinical feature Code Tr D Tr D Tr I

Trachoma II

Tr II

Trachoma Stage III

Tr III

Trachoma Stage IV

Tr IV

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Blinding trachoma is seen in endemic areas, infection is caused by sero type A, B, Ba and C with secondary bacterial infection superimposed. Flies in unhygienic condition where free flowing water is not available is most important factor in causing blinding trachoma. Trachoma in any stage does not produce perforation unless bacterial infection is superimposed. WHO grading of trachoma as guidelines for treatment : TFTrachomatous inflammationFollicular seen in children, in upper tarsal conjunctiva. Presence of five or more follicles of 0.5 mm or larger is significant. TITrachomatous inflammationIntense, pronounced inflammatory thickening of tarsal conjunctiva that obscure more than 50% of deep tarsal vessels. TSTrachomatous scarring. Presence of easily visible scarring in upper conjunctiva. TTTrachomatous trichiasis. At least on eye lash rubs on the eye ball. COCorneal opacityEasily visible corneal opacity over the pupil. TS TT and CO due to trachoma are not seen in children. WHO recommended for management of trachoma25, 26, 27 : TFTopical antibiotic T1Topical and systemic antibiotic TTSurgery Corneal changes in trachoma Corneal changes in trachoma are early to appear. They can be due to (1) chlamydia infection of corneal epithelium, (2) Lid changes leading to secondary changes in cornea (Not generally seen in children). The corneal changes are : 1. Epithelial and sub epithelial punctate keratitis, these are small in size seen generally in the upper part of the cornea. Sub epithelial punctate keratitis are large enough to be visible without magnification. 2. Vacularization. Vascularization of cornea is superficial in nature. It consists of epithelial invasion of conjunctival vessels with diffuse infiltration and epithelial punctate keratopathy. Initially the vessels lie between epithelium and Bowmans membrane, later the Bowmans membrane may be destroyed. Severity of pannus formation depends upon duration and severity of infection. In early stage it is visible only on slit lamp examination. The pannus of trachoma is mostly seen in the upper part of cornea associated with cloudiness of cornea. However, it can occur at any part. Trachomatous pannus is said to be : (i) Progressive when cellular infiltration extends beyond the terminal end of the newly formed blood vessels. (ii) Regressive when the ends of the vessels extend beyond the cellular infiltration. The cellular infiltration is lymphoid in nature with treatment the vessels disappear leaving a clear cornea, only obliterated vessels may be visible on higher

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magnification. A permanent haze results with destruction of Bowmans membrane. Limbal changes in trachoma Hyperemia, edema and follicle formation are common changes in trachoma. The follicles are generally small and transient, disappear without any trace but larger follicles stay for longer time and when rupture leave depressed areas called Herberts pits which are generally not seen in children. Diagnosis of trachoma Diagnosis of trachoma in endemic area is easy clinically. Characteristic signs of trachoma in children : 1. Follicles in tarsal conjunctiva and/or limbus. 2. Epithelial keratitis. 3. Trachomatous pannus. Combination of trachomatous pannus with any of above two is pathognomic in established trachoma. Early trachoma and trachoma outside endemic area present difficulty in diagnosis because there are other causes that produce similar signs. Diagnosis of early trachoma 1. Conjunctival smear for inclusion bodies. Presence of basophilic intra cytoplasmic inclusion bodies in epithelial cells are diagnostic. 2. Presence of immuno fluorescent monoclonal antibodies is specific. 3. Culture of C. Trachoma on McCoy cells is said to be gold standard but is very difficult and costly. Differential diagnosis of trachoma in children : Consist of all case that produce following bilateral signs either alone or in combination. 1. Superficial vascularisation, 2. Follicle formation, 3. Papillary hypertrophy. Common conditions are : Acute and chronic follicular conjunctivitis, palpebral type of spring catarrh, multiple phlycten, riboflavin deficiency, dry eye syndrome, chronic conjunctivitis, healed interstitial keratitis. Management of trachoma in children : Prophylaxis : (a) Chemo prophylaxis, (b) Awareness about trachoma. Prophylaxis. Trachoma is one of major cause of blindness yet it is one of the most treatable and preventable disease. Chemo prophylaxis All children in endemic area should be put on any of the local anti-trachoma chemo therapeutic drugs :

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1. 1% Tetracyclin, oxy tetracyclin ointment; 2. 1% chloramphenicol ointment, 3. 0.5% Erythromycin ointment twice a day for six weeks. Then a gap of six weeks is given and the regime is repeated. Rifampin eye ointment, ciprofloxin eye ointment have also been found to be effective. In the mean time all the other members of community who have active trachoma are treated for trachoma. Trachoma awareness28, 29, 30, 31. It is emphasised that trachoma is : Preventable and curable disease. Preventing use of KAJAL and SURMA from common container. Face washRegular face wash with running water greatly reduces incidence of trachoma. Vector control. Fly is commonest vector that disseminate trachoma. If fly density can be reduced in the community trachoma can be eliminated. Environment. Availability of running water, ownership of septic latrine in the family/ community reduces incidence of trachoma.29 Treatment of trachoma C. Trachoma is sensitive to both sulpha and many broad spectrum antibiotics. As the organism is obligatory intracellular in nature prolonged treatment in effective dose is required. Local chemotherapeutic drugs (TF Stage) : 1. Sulpha cetamide drops 10% to 20% 4 times a day in both eyes is prescribed for six weeks. 2. Chloramphenicol 0.5% drop or Ciprofloxin 0.3% drop 4 times a day for six weeks. 3. Tetracyclin 1% oxy tetracyclin 1%, Chlortetracyclin 1%, ciprofloxin 0.3% ointment three times a day alone or at the bed time along with local sulpha or antibiotic drops for six weeks. Oral antibiotic agentsIn TT stage : 1. Sulpha methoxazole 30mg/kg in divided doses for 3 weeks along with local antibiotic drops. 2. Tetracyclin 15mg/kg in divided dose along with local antibiotics for 3 weeks not given under eight year of age. 3. Erythromycin 30 mg/kg in divided dose for 3 weeks. 4. Doxycycline 1.5 mg/kg single dose for 15 days. 5. Azithromycin 250 mg of single dose is said to be as effective as 1% tetracyclin TDS for six weeks. Systemic Tetracyclin and doxycycline should not be used in children under eight years of age. WHO schedule for management of trachoma in endemic area consist of SAFE method. where S = stands for surgery A = stands for local and systemic antibiotic

138 F = Face wash (personal hygiene)

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E = Environmental changes (availability of running water, disposal of human waste, vector control) Surgery is not required in children. Allergic conjunctivitis in children32 Allergic conjunctivitis is almost pandemic ocular disorder. Its frequency in children may surpass infective conjunctivitis in developed countries. In under developed countries its presentation and outcome may be over shadowed by prevailing infective conjunctivitis and vitamin A deficiency. Presenting features of allergic conjunctivitis are variable, its symptoms may be so mild and insidious that it is accepted as natural phenomenon or may be so troublesome as to cause loss of manpower in adults and absenteeism from school in children. All allergic conjunctivitis areself limiting, duration vary from few minutes to few years. Allergic conjunctivitis are generally bilateral, may be asymmetrical. No race, sex or age is immune. Clinical presentation33, 34 Allergic conjunctivitis is influenced by many factors which may work separately or in various combinations. These factors areGeography, climate, race, age, sex, genetic predisposition and reaction to allergen. 1. Immediate hypersensitivity (humoral), 2. Delayed hypersensitivity (cellular) or 3. Auto immune. Allergic conjunctivitis may be seen both in atopic and non atopic patients. The allergen responsible for immediate (humoral) hypersensitivity are exogenous. Those responsible for delayed (cellular) hypersensitivity are endogenous. The exogenous allergen are organic matters either of vegetable origin i.e. pollen, hay, husk, moulds or of animal origin i.e. fur, wool, dust, mites, dander, drugs, cosmetics, plastics. Polymers are also capable of producing allergic conjunctivitis. Food and additives are known to produce allergic conjunctivitis. Many a times it is impossible to find out the allergen. Exact mechanism of allergic conjunctivitis is not well understood. Factors that make conjunctiva vulnerable to allergic reaction are : (i) Conjunctiva is most exposed mucous membrane of the body. (ii) It is rich in blood supply. (iii) It is very loose. (iv) It is rich in lymphatic. (v) It is constantly bombarded with exogenous allergen. Most accepted theory regarding allergic conjunctivitis is that exogenous allergens disrupt the mast cell membrane and cause degeneration, releasing a host of chemicals that causevasodilatation, edema, inflammation and exudation. The chemicals released in this process arehistamine, various prostaglandins, leucotriens. The reaction is type one allergy

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and IgE mediated. In milder cases there is preponderance of mast cells. In severe cases there is increase in T cell lymphocytes. Classification of allergic conjunctivitis is difficult. However, following classification may fulfil many of the clinical criteria and possible mode of management. 1. Immediate (humoral) hypersensitivity33 (a) Acute allergic conjunctivitis. (b) Seasonal allergic conjunctivitis. (c) Perennial allergic conjunctivitis. (d) Vernal kerato conjunctivitis. (f) Atopic kerato conjunctivitis. (g) Giant papillary conjunctivitis. All are caused due to exogenous allergen. The first three are more common, less troublesome and easy to manage than latter three which are less frequent but difficult to manage. 2. Delayed hypersensitivity (cellular) 3. Auto immune disease (a) Cicatricial pemphigoid (seen in elders) (b) Erythema multi formis (Stevens Johnson syndrome) minor and major. 1. Acute allergic conjunctivitis. Acute allergic conjunctivitis is equivalent to urticaria of the skin. It is very common among children. It can manifest as early as first year of life. It is due to large inoculum of exogenous allergen. The patient complains of sudden onset of copious watering from the eye which may be unilateral or bilateral. Fast developing chemosis of congested conjunctiva. The conjunctiva may be so edematous as to protrude through the inter palpebral fissure. The cornea forms the floor of the crater (well) of the chemosed conjunctiva. The lids are swollen. There may be moderate papillary reaction, no follicles are seen. Cornea remains unaffected. There is no anterior chamber reaction, vision may be blurred due to over hanging chemosed conjunctiva. There is no lymphadenopathy. The symptoms pass off within half an hour to one hour without leaving any trace. Muco purulent discharge, corneal involvement and lymphadenopathy point towards anti microbial conjunctivitis. Treatment. Best is to avoid the allergen if it is known, otherwise local vasoconstrictors, astringent and short acting systemic antihistamine tablets are sufficient. Cold compress applied within few minutes may give relief. Local steroids are not necessary. Mast cell inhibitors have no role, recurrence are common. 2. Seasonal allergic conjunctivitis. Seasonal allergic conjunctivitis is equivalent to hay fever, hence may be associated with rhinitis, sore throat. They have seasonal predilection. Conjunctival reaction consists of mild to moderate papillae or follicle formation. Cornea is bright. There is no AC reaction. There is no lymphadenopathy. Condition is invariably bilateral with mild itching and slight ropy discharge. Conjunctival scrapping show eosinophils. Symptoms coincide with release of allergen in the atmosphere (pollen, hay etc.) Main symptoms are itching, redness, watering. The condition is to be differentiated from trachoma, dry eye syndrome which are not seasonal nor produce itching.

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Management of seasonal allergic conjunctivitis consists of : (i) Oral non sedative antihistamine. Possible side effects of anti histamines should be weighed against its therapeutic benefits while prescribing to children specially when administration has to last longer than few days. (ii) Instillation of combination of astringent, vasoconstrictor, antihistamine several times a day. (iii) Local mast cell inhibitor 2% NedoCromil two times a day is better than 4% sodium cromoglycate BD 2% cromoglycate has less therapeutic value. It is best used as prophylactic in between the attacks three to four times a day. Mast cell inhibitors are almost devoid of any side effect. (iv) NSAID like ketorolac tromethamine 0.5% drops three times or Flurbiprophen 0.03% drops three times a day. (v) Steroids are best avoided. Perennial allergic conjunctivitis As the name suggests the condition lingers throughout the year without remission or exacerbation. In the past perennial and seasonal allergic conjunctivitis were grouped under single category of chronic allergic conjunctivitis. Seasonal allergic conjunctivitis have definite seasonal predilection which lacks in perennial conjunctivitis. The allergen in perennial allergic conjunctivitis is present in the environment throughout the year which could be vegetable, animal or chemical in nature like house dust mite, animal dander, food allergen and locally used drugs. The symptoms are : (i) Itching, redness and watering. (ii) Signs comprise of conjunctival congestion, follicle formation and papillary reaction. Cornea is never involved. The condition may be confused with trachoma, dry eye syndrome or angular conjunctivitis. Management consists of removal of offending allergen which is not always possible. Local NSAID, mast cell inhibitor specially long acting Nedocromil 2% two times a day are effective. Low dose of non sedative antihistamine tablets give additional relief. In milder cases local anti histamine drops in combination with long acting vaso constrictor are sufficient. Steroids are best avoided. Vernal (spring) kerato conjunctivitis34, 35, 36 It is most troublesome allergic conjunctivitis in children. In spite of its name it need not manifest in spring always. It is more common in summer in tropical and sub-tropical countries. It is a bilateral condition, may be a symmetrical specially when it develops first time. It has remission and exacerbations. It has been reported as early as first year of life. More common age is between six years to sixteen years, after which it may automatically heal. It is a self limiting disease lasting for six to ten years. It is more common in boys who outnumber girls in ratio of six to one. In girls the condition is milder, duration is shorter. It is more common in coloured races and in warmer climate. The disorder is seen in two age groups i.e. pre puberty which forms the bulk of patients, and post puberty. The post puberty cases are less in number, generally not seen in females, are less severe and last for shorter i.e. two to three years. Some authors consider this as variation of atopic kerato conjunctivitis. There

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may be a positive history of vernal catarrh in the parents. Sometimes more than one sibling may be affected. Atopic dermatitis is a frequent dermatological disorder in children with spring catarrh. Topographically there are three types of vernal kerato conjunctivitis : 1. Palpebral (tarsal). This is most common. 2. Bulbar (limbal). This causes early and more corneal involvement is more. Common in black races. 3. Mixed. Least common but most difficult to treat. Symptoms. Commonest symptom is intense itching that may last throughout the day. Besides discomfort itching becomes social embarrassment to the child. Itching is associated with redness that becomes more intense following itching, there is a ropy discharge. There may be watering from the eyes. The child finds it difficult to open the eye in the morning but the lids do not get stuck as seen in acute bacterial conjunctivitis. Itching and watering leads to excoriation of the outer canthus. After sometime a pseudo ptosis develops which gives sleepy look to the child. The skin over the lid may be darker than the surrounding area. Most frequent physical finding is presence of moderate size papillae in upper tarsal conjunctiva, these papillae are flat topped in the area that come in contact with the globe firmly and pointed on the lateral side of the tarsus. The flat topped papillae are polygonal in shape. In between adjacent papillae there are deep furrows that zigzag in between the papillae. There may be pseudo membrane over the papillae (Maxwell Lyons sign)35 in untreated cases. With treatment the papillae shrink in size both in height and width. The surface loose its roughness and becomes velvety. There is a tenacious ropy discharge which is alkaline in nature. The discharge can be pulled out in form of fine threads. The discharge which fills the gap between the papillae, the appearance is called milk spilled over cobble stone as the large papillae have appearance of cobble stone. The papillae are not seen in the lower lid or fornices. In the bulbar form there are mucoid confluent nodular papillae at the limbus. Most commonly at the upper part, these nodules are generally seen in segments but may encircle whole of the cornea. In untreated cases there may be formation of Horner-Trantas spot35 which are discrete white spots at the limbus. The corneal changes in spring catarrh are the cause of lacrimation, photophobia and diminished vision. The corneal changes in spring catarrh comprise of following, they may be present as isolated lesion or in various combination : 1. Epithelial micro erosion generally seen in the upper part of cornea 1mm inside the limbus. 2. Epithelial macro erosionThere is large area of epithelial loss leading to sterile ulcer formation due to their shape. They are called shield or amaeboid ulcers seen in the upper part of the cornea. 3. Plaque over the macro erosion. 4. Superficial vascularisation.

142 5. Sub epithelial scarring. 6. Pseudo arcus. This is segmental in nature.

PEDIATRIC OPHTHALMOLOGY

7. Keratoconus has been observed more frequently with vernal kerato conjunctivitis. The exact cause of keratoconus is not understood, may be a genetic feature. Frequent rubbing of the eye has also been blamed for development of keratoconus. Differential diagnosis. Generally there is no problem in diagnosis of vernal kerato conjunctivitis due to its typical clinical feature of chronic, bilateral, papillary reaction in a male child intense periodic itching with remission and exacerbation that responds dramatically to local steroid drops. Common conditions that mimic as vernal conjunctivitis areOther type of allergic conjunctivitis, chronic follicular conjunctivitis, dry eye status, acnerosacea, blepharo conjunctivitis, trachoma, drug induced conjunctivitis. In under developed countries, clinical features may be overshadowed by infective conjunctivitis and xerophthalmia. Management35, 38, 39 Management of vernal kerato conjunctivitis has special significance as it is seen mostly in children, is chronic and requires prolonged treatment. Children with vernal conjunctivitis try to skip school as perpetual redness of eyes and constant itching is not socially acceptable to the children. Parents should be informed that the condition is recurrent, runs a prolonged course, is self limiting and by itself does not produce blindness but blindness can set in by indiscriminate use of steroids that gives prompt relief. 1. Parents should be persuaded to use nonspecific astringents vasoconstrictor and antihistamine drops in various combinations as local drops rather prompt relief giving steroids. 2. Frequent cold compress. 3. Dark glasses. 4. If these fails mast cell inhibitors are added to above treatment. Sodium cromoglycate 2%-4% three to four times a day gives comfort to the child. Chromoglycate sodium in two percent strength can be used as prophylaxis in between exacerbation. Nedocromil sodium 2% BD has better effect that 4% sodium cromoglycate. Cromoglycates are mast cell inhibitors have almost no side effect. 5. Non steroidal anti inflammatory drugs have been used with good result. They have reduced use and dependence on steroid. They lack side effects of steroid are well tolerated. Drugs used are : 1. Flurbiprophen 0.03% two to three times a day. 2. Ketorolac tromethamine 0.5% two to three times a day. Diclofenac sodium 0.1% may be used above twelve years of age. 3. Systemic aspirin, Ibuprofen, indomethacin, nemesulide have been used without uniform result. 4. In spite of all drawbacks local steroids have remained most effective treatment of choice. Local steroids are used as clear solution. Drawback of suspension is that the

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suspended particles get lodged in between the papillae and act as irritant. The principle of local steroid therapy is to use weakest solution used least frequently should give maximum relief without side effect. Commonly used steroids used are Dexamethasone/Betamethasone 0.1% Prednisolone0.125% to 0.5% Fluorometholone 0.1% In severe cases any of the above drugs is instilled in conjunctival sac every two hourly for first twenty four hours, then frequency is gradually reduced over weeks. After a month the child may be comfortable in once a day instillation. All children under topical steroid should be under constant supervision of ophthalmologist who should monitor - vision, error of refraction, intra ocular pressure and any change in the disc. The lens should be examined for iatrogenic cataract. Addition of mast cell inhibitors reduces requirement of steroid without jeopardising result. 8. Other drugs used in vernal kerato conjunctivitis are : (i) Acetyl cysteine two to ten percent solution in methyl cellulose four times a day dissolves mucus. (ii) Weak solution of acetic acid to change pH of tear from alkaline to acidic. (iii) Tear film substitutes are given to manage ocular surface disorder that is too common. (iv) Cyclosporine 2% in castor oil may be used in those cases that do not respond to steroids. These cases are generally associated with atopia. (v) Shaving of papillae, cryo application to the papillae have not found to be very effective. (vi) Plano T contact lenses may be helpful in persistent superficial keratitis. (vii) Rarely low dose systemic steroid for a short period, may have to be used in severe corneal involvement under strict supervision. Atopic kerato conjunctivitis Fortunately this condition is rare, mostly seen in atopic adults but children in second decade may be affected. It has more systemic symptoms of atopic disease than ocular symptoms. Ocular manifestation consist of blepharitis, papillae formation, corneal epithelial defect, corneal plaque and atopic cataract, keratoconus. Systemic signs consist of eczema in the neck, antecubital fold, asthma, rhinitis. Management consists of local low dose of steroid, sodium cromoglycate, sodium nedocromil, NSAID. Some cases may require topical cyclosporin in oil two times a day. Giant papillary conjunctivitis41, 42, 43 The malady was first reported in 1974. By 1977 a syndrome consisting of contact lens intolerance, itching, mucus discharge, hyperemia, blurred vision has emerged. On

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examination large papillae that are larger than 3mm across are seen in upper tarsal conjunctiva. The symptoms diminished on discontinuation of contact lens. Severity depends produce more encounter in on size of contact lens. Larger the lens more are the symptoms. However it was realised that contact lens was not the offending agent. It was the preservative and protein deposits on the lens that were the causative factors. Extendedwear lenses produced clinical features. It is not associated with atopia. It can affect any age both sexes are equally effected . On long run tops of papillae ulcerate and stain with fluorescein. Besides contact lens giant papillary conjunctivitis is also encountered in protruding mono filament sutures, corneal scleral shell, artificial eye, kerato prosthesis, ocular prosthesis, scleral buckle, cyno acrylate glue, multiple embedded foreign bodies. Management comprises of discontinuation of CL, change of CL polymer and design, proper contact lens hygiene. Use of preservative free contact lens solution, steroids are best avoided. Local NSAID and cromoglycates give sufficient relief. Phlyctenular kerato conjunctivitis44,45,48 This is very common allergic conjunctivitis seen world-wide, more commonly in under developed countries where tuberculosis is endemic, malnutrition is frequent and lack of public health facility common. It is a cell mediated type four allergic reaction where sensitised corneal and conjunctival epithelium when exposed to causative endogenous allergen develops interstitial kerato conjunctivitis in the form of a nodule. Tubercular protein is commonest causative factor. The allergic response is attributed to lipid fraction of tubercular antigen. Other causative organisms that can produce phlycten are 46 staphylococcus, strepto coccus, gonococcus, candida, coccidomycosis, lympho granuloma, ascariasis. Rarely it can be idiopathic as well. Common age to develop phlycten is between three to ten years. After ten years frequency becomes less. After fifteen it becomes very rare. Girls are effected more than boys. It is a bilateral disease, both eyes may be involved simultaneously, or separately. Number of nodules vary from single to multiple when more than one nodule is present they may be away from each other or may coalesce to involve a sector of limbus or in severe cases they may encircle the whole of the cornea. Phlyctens are self limiting in nature. They resolve in ten to fifteen days, never to recur on the same spot. However there may be crops of lesions one after another. Perilimbal conjunctiva is the commonest site to develop phlyctenular conjunctivitis. It has been observed on the bulbar conjunctiva as well as tarsal conjunctiva and inter marginal strip. Other frequent part to be involved is cornea either primarily or due to spread from conjunctiva. Typical conjunctival phlycten develops one to two millimetres away from limbus as a pale vescicle that form a nodule later44. The initial size may be that of a pin head, soon it enlarges to 1 mm to 2 mm. The conjunctiva round the nodule is congested, conjunctival vessels do not pass over the nodule. Two types of presentation are encountered i.e. only phlycten without muco purulent conjunctivitis or phlycten developing in infective conjunctivitis. Role of infective conjunctivitis in production of phlycten is not clear. Most of the time it is coincidental. Other possibility is that due to infection conjunctival epithelium gets sensitised and a phlycten ensues. Acute infective conjunctivitis may facilitate transfer of large amount of tubercular

Comparison between various types of allergic conjunctivitises :

Sl.No. Curative Pollen Variable Constantly present Pollen -DoItching, ropy discharge-papillae, corneal involvement. Itching, follicles blepharitis. Giant papillae upper tarsus, itching pseudo ptosis. Mast cell inhibitor steroid cyclosporin Steroids, Anti histamines, 4% sodium cromo glycali, NDAID Chronic Itching, follicle Antihistamine Mastcellinhibitors -DoMast cell inhibitors Sub acute Itching, hyperemia Mast cellinhibitors antihistamines Mast cell inhibitors Acute Itching watering chemosis Antihistamine weak steroid Mast cellinhibitors Preventive

Type

Age

Sex

Allergen

On set.

Symptoms

Treatment

1.

Acute allergic

Any

Both

2.

Seasonal allergic- Any conjunctivitis

Both

3.

Perennial allergic -Doconjunctivitis

-Do-

DISEASE OF CONJUNCTIVA IN CHILDREN

4.

Vernal kerato conjunctivitis

Under 20 years

Males more

5. Contact lens -Dopreservative

Atopic kerato conjuctivitis

Any

Both

Atopia

-Do-

-Do-

6.

Giant papillary conjunctivitis

Any

Both

Contact lens hygine, Contact lens remove contact hygine Do not use lens. NSAID home made contact lens solution

AH = Antihistamine, MCSmast cell stabilizer, CLContact lens.

145

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protein to the limbus that results in formation of phlycten. Within four to five days the phlycten reaches its maximum size and the surface epithelium gives way to form an ulcer which takes another three to four days to heal without any scar on the conjunctiva. Though tuberculosis has been blamed as major cause of phlycten. Tubercle bacilli have never been isolated from the conjunctiva in phlyctenular keratoconjunctivitis. The histological changes do not show tubercle formation it resembles more or less of a micro abscess. Other form of involvement in phlycten is when it develops at the limbus. It may either spread from conjunctiva and spill over the cornea or may develop directly on the limbus resulting in phlyctenular kerato conjunctivitis which is more congested than simple phlyctenular conjunctivitis. Corneal involvement in phlycten can be of following types : Cornea may be involved as (1) Primary lesion (2) The lesion may spread from conjunctiva. There are two main types of corneal involvement i.e. (1) Ulcerative (non vasularised) and (2) infiltrative (vascularisation)47. The ulcerative form may be (1) Marginal, (2) Fascicular. Marginal corneal phlycten is generally due to a phlycten developing at the limbus. The phlycten sits astride the limbus i.e. half on cornea, half on conjunctiva. Like any phlycten in due course it ulcerates and heals with out any scar. However if the ulcer is deep it destroys the Bowmans membrane and a permanent opacity is formed. An opacity due to marginal phlycten has a typical dome shaped configuration with base at the limbus and convexity towards the pupil. There may be single or multiple marginal phlyctens. More than one may coalesce to form a sectorial ulcer. Fascicular ulcer is a typical corneal presentation of phlycten. It is generally preceded by marginal or keratoconjunctival phlycten. The ulcer starts on the corneal periphery, has a tendency to move towards centre followed by a leash of superficial vessels. The vessels are typically straight and do not branch. The fascicular ulcer while shifting from periphery towards pupil digs deep in the substance of cornea creating a shallow furrow. The vessels develop in this furrow. There may be more than one fascicular ulcer in the same cornea. The fascicular ulcer does not perforate, nor does it produce anterior chamber reaction. The final location of the ulcer where it may become stationary is uncertain. Once it stops shifting it starts healing, leaving a superficial opacity followed by fainter band shaped opacity extending upto limbus. This band represents the place of leash of vessels that followed the ulcer. Third rare possibility is small pin point superficial ulcers all over the cornea. Infiltrative type of corneal phlycten may produce two types of vascularisation : First more common superficial vascularisation and a less frequent deep vascularisation. The superficial vascularisation may develop anywhere on the cornea even all around the cornea and called phlyctenular pannus. It differs from trachomatous pannus in the sense that latter has predilection for upper cornea. The vascularisation ultimately dies down leaving no scar. In a more severe case of longer duration there may be deep vascularisation which leaves permanent mark. Corneal involvement is more when tubercular protein is causative factor. Symptoms. Symptoms of phlyctenular kerato conjunctivitis depend upon number of phlycten; associated bacterial conjunctivitis, and involvement of cornea. Simple conjunctival phlyctens do not produce much symptom except localised redness in the eye, slight irritation

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and watering. Associated infective conjunctivitis invariably overshadows simple phlycten that may be missed altogether or it may become obvious when conjunctivitis has subsided. Though phlycten is an allergic manifestation it does not produce itching. Presence of itching with nodule at limbus points towards limbal spring catarrh. Symptoms become more severe as cornea gets involved. A phlyctenular kerato conjunctivitis produces severe photophobia in children. Fascicular ulcer is associated with severe blepharospasm, photophobia, lacrimation and diminished vision. Vision is greatly reduced if the ulcer is positioned against the pupil. Management 1. Simple phlycten without infective conjunctivitisLocal weak solution of steroid i.e. dexamethasone 0.1% 4 hourly for first day. This reduces symptoms promptly or FML 0.1% every one to two hourly till symptom subsides then the frequency is reduced. 2. Phlycten with infective conjunctivitis is treated as any catarrhal conjunctivitis along with local steroids under supervision. 3. If cornea is involved atropine is added to steroid to reduce ciliary spasm, this gives prompt relief. Keratitis due to tubercular antigen though produce severe symptoms responds better than in cases of staphylococcal antigen induced keratitis. 4. In under developed countries all children with phlycten should be investigated for both pulmonary as well as extra pulmonary tuberculosis. BCG does not prevent a child from developing phlycten. If the child is found to be tubercular a full course of anti-tubercular treatment should be ensured. 5. In case of staphylococcal allergy lid hygiene along with systemic anti staphylococcal antibiotic may be required. Children older than eight years should be put on doxycycline 100 mg once a day for 10 days while smaller children are put on erythromycin in dose of 25/mg/kg for seven to ten days along with local steroid. 6. Children suffering from intestinal helminths should get a course of anti helminth. Albendazole is an effective drug. This is given in form of chewable tablet in single dose. Children above 2 years should get 400 mg single dose to be repeated as required. Differential diagnosis of phlycten comprise of nodules at limbus, trachoma, spring catarrh, interstitial keratitis. Complication. Conjunctival phlycten is devoid of any complication. Corneal complications consist of peripheral dome shaped opacity, Salzmanns nodular dystrophy46, corneal vascularisation and various grades of corneal opacities that may diminish distant vision leading to squint and amblyopia. Erythema multiforme49, 50 Though this disorder is classified along with allergic conjunctivitis. It is not a true allergic conjunctivitis. The exact mechanism is not known. Most acceptable theory is an auto immune disease. 51 It differs from both endogenous and exogenous allergic conjunctivitis by not producing - papillae, follicles or nodule. There is no itching either. It has profound systemic manifest, mostly cutaneous and mucous membrane involvement. It is a disease of children

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and young adults. There are two types i.e. (1) erythema multiform minor (2) Erythema multi forme major (Stevens Johnson syndrome) The latter is not only bilateral sight threatening, it can often be fatal. Etiological factors that can culminate into this troublesome disease are : 1. Drugs. Many drugs that are routinely used for day to day management of various symptoms can cause Stevens Johnson syndrome. Top of the list is taken by sulpha drugs including acetazolamide others are penicillins, phenylbutazone barbiturates, salicylates. 2. Infection. (i) Recurrent infection by herpes simplex type I and II. (ii) Respiratory infection with mycoplasma pneumonae. 3. Idiopathic. The disease has three stages : 1. Prodromal 2. Vesciculo bullus stage 3. Complication. The pro dromal stage consist of malaise, sore throat, fever, joint pain and swelling. In second stage. The muco cutaneous involvement consists of erythematous macules on dorsal hand and feet, exterior surface of forearm and legs. The lesion soon becomes papular, which become bullus. Any part of the mucous membrane may be involved. The lesions are generally symmetrical. This stage lasts for seven to fifteen days. Healing starts within a few days and heals completely. Recurrence has been reported if the child is exposed to same precipitating factors. The ocular lesions Lesions of the eyes seen in 50% of cases. It is bilateral, generally symmetrical, starts as muco purulent conjunctivitis that may become purulent. There may be vescicle formation. Due to vasculitis part of the conjunctiva gets infarcted. There may be formation of pseudo membrane or true membrane. Shedding of these membranes leave raw areas in the conjunctiva. When two such raw areas come in contact with each other adhesion develop. That result in formation of symblepharon. Shrinking of conjunctiva leads to trichiasis, entropion, obliteration of lacrimal ducts, keratanisation. Complications. Erythema multi form minor may pass off without any complication. However Stevens Johnson has far reaching vision threatening complication like xerosis of conjunctiva and cornea, keratanisation of cornea, vascularisation and opacification of cornea. Management. There is no effective treatment of ocular involvement. Local steroid may minimise vasculitis and prevent conjunctival infraction which in turn reduces formation of pseudo membrane. Attempts should be made to prevent adhesion between two raw surfaces by instilling bland lotion systemic steroid started in early stage are life saving and minimise scarring. Conjunctivitis of unknown causes in children : 1. Folliculosis. This is not a true conjunctivitis. It is a benign, bilateral, non-infective follicular hypertrophy in children. The follicles are small, seen mostly in lower fornix, tarsal conjunctiva is not much involved. Cornea is never involved. There is hardly any discharge. There is no itching. It is to be differentiated from other causes of follicles and papillae formation

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like chronic follicular conjunctivitis, trachoma and spring catarrh. The condition does not require any treatment except reassurance to the parents. Chronic follicular conjunctivitis is an infective conjunctivitis of under nourished children. It is a bilateral condition. The follicles are numerous both upper and lower tarsal conjunctiva are involved. There may be minimal discharge. 2. Iatrogenic conjunctivitis (Drug induced). It is a non specific conjunctivitis following prolonged administration of host of commonly used local drops that include - pilocarpine, atropine, home atropine, idoxuridine, neomycin, gentamycin sulpha cetamide. It is generally associated with dermatitis of the lid margin and excoriation of the outer angle. There are some follicles which may be associated with itching and confused with non drug induced allergic conjunctivitis. Treatment consists of stopping the offending drug and replacement by nontoxic drug. Weak solution of steroid. In spite of treatment follicles may persist for few days to weeks. 3. Ligneous conjunctivitis. This is rare type of conjunctivitis seen in children may be seen soon after birth or develop any time even in adults. It is more common in girls, is generally bilateral but asymmetrical. Most important feature is formation of membrane on the tarsal conjunctiva that gives it a hard feeling. There may be formation of granuloma on the tarsal conjunctiva. Exact cause of this condition is not known. However chronic infective process and hypersensitivity are two important factors. There is no satisfactory treatment. Initially the condition may be confused with membranous or pseudo membranous conjunctivitis and StevensJohnson syndrome. Granulomas of conjunctiva Conjunctival granuloma are seen in chronically inflamed conjunctiva. Common cause in children are : 1. 4. 6. Burst chalazia, Tuberculosis, Candida, 2. Foreign body, 3. Rhinosporidiosis, 5. ophthalmia nodosum (caterpillar hair), 7. Coccidomycosis, 8. Ligenous conjunctiva.

Rhinosporidiosis52, 53 (Oculo sporidiosis) Rhinosporidiosis is caused by fungus Rhinosporidium Seebri. Ocular involvement is seen mostly in children in endemic areas. The disease has wide distribution mostly in tropics with moderately to heavy annual rainfall and high humidity. Almost all mucous membranes of the body are known to be involved including conjunctiva and lacrimal sac; lesions of skin and bone have also been reported. The spores of the organism get implanted in the mucous membrane and develop into sporangia, a mature sporangium may contain as many as 16,000 spores. Ocular involvement is said to be primary54 when there is no other evidence of the disease in any part of the body except eye and its adnexa. This is also known as oculosporidiosis. In secondary rhinosporidiosis of the eye the disease spreads from the nasopharynx to the sac. Ocular structures involved in rhinosporidiosis are - conjunctiva, lacrimal sac and sclera. Combined involvement of sac and conjunctiva is very rare. Conjunctival involvement is generally primary.

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Typical conjunctival lesion is a gradually developing painless granuloma in the conjunctiva. Commonly involved are fornices and tarsal conjunctiva. Bulbar conjunctiva is rarely involved. The lesion is more common among boys between 5 to 15 years, who have habit of taking bath in a pool shared by cattle which are invariably infected. The growths in the tarsal conjunctiva are flat and sessile, those at limbus or bulbar conjunctiva are round. While those arising from fornices have long thin pedicle with finger like projection. All the lesions are bright red in colour, have rough surface dotted with samolina (sujee) like white dots that represent sporangia. The lesions bleed on slight trauma. Generally there is only one granuloma in an eye. Bilateral conjunctival involvement is rare. The size of the growth varies from few millimetres to few centimetres. Symptoms. Main symptoms are bleeding in the conjunctiva and visible red mass. If the growth hangs over the cornea, it may cause lacrimation. The lesions are non tender and painless. They are fragile, bleed on slight manipulation like everting the lid. Diagnosis. Diagnosis of conjunctival rhinosporidiosis is simple in endemic areas. Common lesions that form differential diagnosis are burst chalazion, foreign body granuloma, haemangioma, papilloma. However reverse is also true i.e. above conditions may be mistaken as rhinosporidiosis in endemic area. Diagnosis is best confirmed by histopathology of the excised tissue. Other useful diagnostic method is to examine small piece of tissue dipped in saline under high power that show sporangia. Sometimes free spores are demonstrated in the tears of affected child. Involvement of lacrimal sac in rhinosporidiosis52,53,54 is more common than conjunctival involvement. The sac can be involved either as primary lesion i.e. without any involvement of nasopharynx or as a secondary spread from nose and throat. Involvement of the sac is in the form of chronic dacryocystitis where the growth arises from the mucosa of the sac and fills the sac to distend it, that presents as a diffuse non tender swelling on the medial side of the eye mostly below the medial palpebral ligament, may spread under the lower lid. The swelling is compressible but non-reducible, does not change in size on coughing or sneezing. The skin over the swelling has orange peel appearance. Temperature over the swelling is not raised. Regurgitation test is negative, complete block of nasolacrimal duct is rare. Erosion of surrounding bone is common in long standing cases. If sac is secondarily infected it presents as cellulitis. This generally happens following syringing. Bilateral involvement is rare. Exact mechanism of sac involvement is not clear specially as a primary lesion. Partial congenital obstruction of nasolacrimal duct, chicken pox or measles are contributory factors in endemic area. A narrow or obstructed nasolacrimal duct due to any of the above contributory factors allows the spore to settle down in the sac where it reaches via puncta and canaliculi. Once the spore reaches the sac it forms a nidus over which the growths develops. (See page 93-94 as well) Scleral involvement is most rare. It is always associated with a conjunctival growth that hangs over the scleral lesion which is in the form of a staphyloma. The staphyloma is located anterior to the equator. Occasionally it is visible on indirect ophthalmoscope. Management. There is no known drug that is effective against rhinosporidiosis. Surgery is the treatment of choice. The conjunctival lesions can be removed under topical anaesthesia. If needed short term general anaesthesia may be administered. The growth is

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grasped with flat, blunt forceps and pulled away from the conjunctiva or a ligature may be tied round the pedicle. The pedicle is cut with sharp scissors. Even without ligature the bleeding can be stopped by firm pressure. Small growth can be treated with cryo. Surprisingly there is no recurrence in conjunctiva once the growth has been removed surgically even if the child lives in the same environment. Management of rhinosporidiosis of sac is less satisfactory. It is essentially complete removal by surgery. Unless meticulous care is taken to remove every bit of tissue involved recurrence is the rule. While removing the sac the nasal growth should also be taken care of simultaneously. The growth of the sac can also be removed by nasal endoscopic surgery. In scleral involvement the conjunctival growth is removed by usual method. For scleral staphyloma best results are obtained by applying cryo all round the staphyloma. The scleral bulge is indented with scleral explant of suitable size. Intraocular pressure of the eye is kept low either by paracentesis or IV mannitol and maintained low by oral diamox and betablocker drops. Tumours of conjunctiva Tumours of conjunctiva in children are rare. They are generally - Hamartomas, choriostomas, haemangiomas and xeroderma pigmentosa. Hamartomas that have congenital involvement are : Neuro fibromatosis (von Reckling Hausens disease and Enccphalo trigeminal angiomatosis (Sturge Weber Syndrome). Conjunctival involvement in neuro fibromatosis is less common than in Sturge Weber syndrome. Conjunctiva is never involved alone, it is always associated with lid involvement and many have associated systemic involvement. Choriostomas. These are fairly common congenital benign tumours. There are two types of choriostoma : 1. Dermoid, 2. Dermolipoma. Haemangiomas of conjunctiva. These benign tumours are not true tumours. They may develop alone in the conjunctiva or may be seen with haemangioma of lids. (See page 61-62) Cysts of the conjunctiva Various types of conjunctival cysts are : traumatic, retention, lymphatic or developmental. They can be parasitic in nature i.e. cysticercosis, echynoccosis, coenurosis. It is estimated that non traumatic cysts are seen in 43% of eyes mostly in fornices. They vary from pin head size to large enough to fill the fornix. Common causes of cysts in conjunctiva are : trauma, congenital anomaly, parasitic infection and adhesion between two layers of conjunctiva. Cysts can be grouped into two broad groups i.e. traumatic and non-traumatic. Traumatic cysts are mostly epithelial implantation cyst due to trapping of epithelium under the mucosa. They are seen following wounds of conjunctiva, squint surgery, retained conjunctival foreign body.

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Non-traumatic cysts are : Dermoids, parasitic cyst, retention cysts and lymphatic cysts. Retention cysts These generally develop in glands of Krause following chronic inflammation or trauma. The ducts of the glands are obstructed resulting in formation of cysts. They are generally seen in the upper part of the conjunctiva. Symptoms depends on size and position of the cyst. The child may not be aware of small cysts. They may be discovered during routine examination of the eye. Parents may become aware of large cysts. There may be multiple translucent cysts. A large cyst may cause astigmatism or may cause a diffuse swelling in the lid. Management. Small cysts do not require any treatment. Large cysts are removed for cosmetic purpose and to reduce astigmatism. Aim should be to remove the cyst without rupture. Parasitic conjunctivitis Parasitic infestation of conjunctiva is far less common than bacterial or viral infection. Parasites range from protozoa to helminth. Rarely arthropods may invade the conjunctiva. Parasitic infection of conjunctiva is generally seen in tropical and sub-tropical regions. Some of the parasitic conjunctivitis are seen only in endemic areas (river blindness). Some of parasitic conjunctivitis are very rare and may not be seen in children. Common parasitic conjunctivitis seen in children are : Cysticercosis, echinococcosis, coneurosis, thelaziasis, onchocerciasis. (See Chapter Ocular Manifestation of Systemic Diseases) Cysticercosis56, 59 is a systemic parasitic infection. About forty percent of persons infested by the parasite, have ocular involvement. The ocular involvement can be (1) less vision threatening, extra ocular manifestation in the form of conjunctival or orbital cyst. (2) More severe and blinding intraocular involvement. Cysticercosis is caused by Cysticercus cellulose the larval stage of pork worm taenia solium. Humans harbour the parasite in two forms i.e. (1) Definite host form of adult tapeworm or (2) Intermediate host, harbouring larval stage. Pig is the usual and natural intermediate host. Humans get infected by consuming under cooked pork that contains viable cysticersis which mature in human intestine, and become adult worm. Other method of infection is consuming food and water contaminated with ova of the parasite. Latter mode of infection is more common than in former. This is the mode of infection in strictly vegetarians and those persons who shun pork due to religious reasons. The embryo reaches the eye via ophthalmic artery. Left eye is more frequently effected than right eye. Most of conjunctival lesions are located near the medial canthus. However right eye is not immune. Bilateral and multiple unilateral involvement are rare. Involvement of left eye is so frequent that all conjunctival cystic lesions in the left eye should be considered to be cysticercosis unless proved otherwise.57 The swellings are of variable size generally fixed to episcleral tissue or the sclera. Initially the conjunctiva moves freely over the translucent oval swelling. The cyst is painless. It may migrate along the extra ocular muscle

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from behind to be visible on the conjunctiva and extruded56, 60. The cyst may occasionally extrude through the conjunctiva. If the cyst ruptures it causes localised conjunctivitis. Differential diagnosis consist of other parasitic cyst. Diagnosis is confirmed by histopathology after removal. However increased eosinophils in differential count and presence of ova in stool is highly suggestive. Management consists of 1. Prophylaxis, 2. Surgical removal of the cyst. Prophylaxis consists of proper cleanliness and food hygiene. Health authorities should see that infected pigs are not slaughtered for consumption. Pork should be well cooked, raw vegetables should be avoided least they are contaminated. Children should be dewormed regularly either by Mebendazol 100 mg BD for three days or albendazol 400 mg single dose at least once a year. Role of antihelminth as cure of conjunctival cyst is not confirmed. Surgical treatment consists of complete removal of cyst without rupture. Rupture causes severe conjunctivitis. Following successful removal, there is no recurrence. Other parts of the eye that may be invaded by cysticercosis are lids, orbit, retina, vitreous, and anterior chamber. Intra ocular involvement results when the embryo reaches the interior of the eye via posterior ciliary artery. The embryo develops in to an expanding cyst in the choroid that lifts the retina to produce exudative retinal detachment. Perforation of the retina by cysticareus results a free floating cyst in the vitreous. The cyst on rupture produces larval endophthalmitis both large intra vitreal cyst and endophthalmitis cause white reflex in pupillary area. Death of the larvae also causes endophthalmitis severe enough to cause loss of eye. Echinococcosis62 Echinococcosis is systemic parasitic infection caused by larva of T. Echinococci a helminth found in intestine of dogs. Man and other animals can get infected from affected dogs. Human is an intermediate host who gets infected following ingestion of contaminated food. No part of the body is immune to echinoccosis however, liver and lungs are most frequent sites. Ocular involvement consist ofCyst in sub-retinal space, vitreous and anterior chamber. Conjunctival cyst formation is less common than seen in cysticercosis. Orbital cysts cause proptosis, chemosis of conjunctiva and lid. Cyst when present in the conjunctiva may be attached to one of the recti. Management consists of prevention of infection in children. This consists of regular de-worming of the pet and the child. Avoiding green raw vegetables. Definitive treatment is removal of the cyst surgically without rupture. Coenurosis This is systemic and ocular involvement of an intermediate host by larval stage of dog tapeworm multiceps. Humans act rarely as intermediate host which are generally sheep and goats. Children get infected directly by ova of the parasite from soil, vegetable or fur of dogs deposited in the conjunctival sac and develop cyst similar to cysticercosis. Adults get infected by ingestion of contaminated food.

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All the parts of the eye can be infected. In children subconjunctival cyst is most common presentation. In adults it can involve sub retinal space, vitreous or orbit. There is no known medical treatment, surgical removal is best option. Thelaziasis63, 64 Thelaziasis is a nematode infection of ocular tissue mostly in animals. There are about ten species of thelazia that have been reported to infect conjunctiva. Common being thelazia callipaeda and thelazia californiensis. Involvement of human eye is rare. In humans mostly outer eye is involved. Parts involved are conjunctiva, lid and lacrimal passage. The nematode finds its way to conjunctiva through a arthropod most probably a fly that lays eggs in the conjunctiva which hatches there to complete its life cycle and produces conjunctivitis, hyperemia and chemosis of conjunctiva. Unlike other nematodes thelazia does not cause cyst in the ocular tissue. Live worm can be found in the conjunctival sac. Intra ocular involvement have been reported. Intraocular penetration may result through an abraded cornea or sclera. Live worm have been removed from anterior chamber.63 Management of conjunctival involvement is manual removal of worm from conjunctival sac after it has been immobilised by local anaesthetic agent. Worm from AC should be removed surgically. Associated uveitis is treated by usual local cycloplegic and steroid. When surgical removal is not possible, Diethyl carbamazine 50 mg three times for 14 days may prove effective. Ocular myiasis Ocular myiasis or larval conjunctivitis is caused by deposition of eggs of some of the flies. Three species of flies have been identified to cause myiasis. 61 Even common housefly can cause ocular myiasis. The flies are attracted to the conjunctival and nasal discharge of the filthy child. The flies lay eggs in the conjunctiva where the eggs hatch as larvae (maggots). It is the larvae that are the cause of conjunctival inflammation. The maggot infestation is seen mostly in emaciated children. There are two types of conjunctival infection by larvae : 1. Larval conjunctivitis. There is redness, itching, burning and lacrimation. On examination small elongated white larvae are seen crawling in the conjunctival sac. There may be associated marginal keratitis. 2. Destructive myiasis. The larvae penetrate deep under the conjunctiva and skin into the orbit. Occasionally the bones may be eaten away and meninges exposed. Unless the larvae are eradicated promptly they may cause death. Management consists of maintaining proper cleanliness in emaciated child. Use of mosquito net to prevent fly from depositing eggs on the conjunctiva. Attempt should be made to remove the larvae manually as soon as detected.

REFERENCES
1. Duke Elder S. : General consideration in conjunctival diseases in system of ophthalmology. Vol. VIII, Part 1. p-4 Edited by Duke Elder S., Henry Kimptom, London, 1965.

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2. Nema H.V. : Development of ocular adnexa in Anatomy of the eye and its adnexa. Second edition. p-138-139, Jay Pee Brothers, New Delhi 1991. 3. Berger B.B. : Surgical anatomy of the limbus in Principle and practice of ophthalmology. Vol. I, p-532-534. First Indian edition Edited by Peyman G.A., Sonders D.R., Goldberg M.F., Jay Pee Brothers, New Delhi, 1987. 4. Basak S.K. : The limbus in Essentials of ophthalmology. Second edition p-5-6. Current Books International, Calcutta, 1999. 5. Ilene K. Gipson : Anatomy of the conjunctiva and cornea in The cornea. Third edition. p-13-17 Edited by Smolin G. and Thoft R.A. Lippincot, Williams and Willkins, Philadelphia 1994. 6. Khamar B., Mayuri Khamar, Trivedi N. and Usha Vayas : Disease of conjunctiva in Modern Ophthalmology. Vol. I, second edition. p-38-42 Edited by Dutta L.C., Jay Pee Brother, New Delhi 2000. 7. Wilson F.M. : Congenital anomalies of cornea and conjunctiva in The Cornea. Edition third. p-547-548. Edited by Smolin G. and Thoft R.A., Lippincot Williams and Wilkinsons, Philadelphia 1994. 8. Laibon P.R. Owaring : Neonatal corneal opacities in Pediatric Ophthalmology. Vol. I, Edition 2. p-480-483. Edited by Harley R.D., WB Saunders Company, Philadelphia 1983. 9. Chandler J.W. : Diphtheria in Current ocular therapy. Fifth edition p-22-24. Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company Philadelphia 2000. 10. Burd Eileen M. : Bacterial keratitis and conjunctivitis in The Cornea. Third edition p-119. Edited by Samolin G. and Thoft R.A., Lippincot Williams and Wilkins, Philadelphia 1994. 11. Frindly D.S. : Ophthalmia neonatorum. Pediatric clinic of North America. 30:1033-1042. 1983. 12. OHara M. : Ophthalmia neonatorum. Pediatric clinic of North America. 40:715-725. 1993. 13. Khamar B., Usha H. Vyas, Trivedi N. and Mayuri Khamar : Ophthalmia neonatorum in Modern Ophthalmology. First edition, p-19-21. Edited by Dutta L.C., Jay Pee Brothers, New Delhi 1994. 14. Isada C.M., Meisler D.M. : Gonococcal ocular disease in Current ocular therapy. Fifth edition p-28-31, Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Philadelphia 2000. 15. American academy of pediatrics committee on drugs : Prophylaxis and Treatment of neonatal gonococcal infection. Paediatrics 65, 1047-1048, 1980. 16. Laga M., Naamara W., Brunham R.C. : Single dose therapy of gonococcal. Ophthalmia neonatorum. New Eng. Jr. M. 315 : 1382-1385. 1986. 17. Wand M. : Acine bactor : In current ocular therapy. Fourth edition p-3-4. Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Philadelphia 1995.

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18. Deborah Pavon-Langston. Foulks G.N. : Cornea and external diseases in Manual of ocular diagnosis and therapy. Third edition, p-71, Little Brown. 19. Whitcher J.P. Chlamydia : Keratitis and conjunctivitis in The Cornea. Third edition. p-282-292. Edited by Smolin G. and Thoft R.A. Lippincot, Williams and Wilkins, Philadelphia 1994. 20. Munoz B., West S. Trachoma : The forgotten cause of blindness. Epidemiol. Rev. 19:205217, 1997. 21. Kathryn I. Taylor and Taylor H.R. : Trachoma in Current ocular therapy. Fifth edition, p-76-78. Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Philadelphia 2000. 22. Viswalingam N.D., Darougas S. : Trachoma in Current ocular therapy. Fourth edition. p-61-63. Edited by Fraunfelder F.T., Roy F.H., WB Saunders Company, Philadelphia 1995. 23. Duke Elder S. Trachoma S. : System of Ophthalmology. Vol. VIII Part I, p-281-282, Henry Kimpton, London 1965. 23A. McCallan A.F. : The epidemiology of trachoma. Br. Jr. Oph. 15:369, 1931. 24. Thylefors B., Dawson C.R., Jones B.R., West S.K., Tylor HR : A simple system for assessment of trachoma and its complications. Bull WHO 65, 477-483, 1983. 25. World Health Organisation : Future approach to trachoma control. A report of global scientific meeting. Geneva WHO/PBL 96.56. 26. WHO : Primary care level management of trachoma. Geneva. WHO/93.33. 27. Victoria Fransis : Achieving community support for trachoma control. Community Eye Health. 7:28-30, 1994. 28. Courtright P. : Trachoma control : Community health. 7:18-20, 1994. 29. Courtright P., Sheppard, J., Lane S., Sadek A. et al. : Latrine ownership as a protective factor in inflammatory trachoma in Egypt. B.J.O.75:322-325, 1991. 30. West S.K., Munoz B. et al. : Impact of face washing on trachoma in Kongwa, Tanzania. Lancet 345:155-158, 1995. 31. Denise Mabey and Mabey D. : New hope for trachoma control. Community eye health. 7:17-18, 1994. 32. Friedlacnder M.H. : Ocular allergy. International Oph. Clinic. 28 : 261, 1988. 33. Donshik P.C. : Allergic conjunctivitis. International Oph. Clinic. 28, 294-301, 1988. 34. Friedlaender M.H., Okumoto M., Kelly J. : Diagnosis of allergic conjunctivitis. Arch Oph. 102-1190-1195, 1984. 34A. Held K.S. : Itchy eye in Decision making in ophthalmology. First Indian edition p-18-19, Edited by Hunven WAJ and Zivaan J.T., Harcourt Brace and Company Asia Pvt. Ltd. 1988. 35. Douglas S.H. : Vernal kerato conjunctivitis in Current ocular therapy. Fifth edition, p-341-343. Edited by Fraunfelder F.T. and Roy H.F., WB Saunders Company, Philadelphia 2000.

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36. Colby K., Dohlman C. : Vernal kerato conjunctivitis. Int. Oph Clinic. 36:15-20, 1996. 37. Hennawi M.M. : Vernal kerato conjunctivitis in current ocular therapy. Fourth edition p-476-477. Edited by Fraunfelder F.T. Hamptom Roy F. WB Saunders Company Philadelphia 1995. 38. Christiansen Sandra : Evaluation and treatment of the allergic patient. Int. Oph. clinic. 28:290-292, 1988. 39. Buckley R. : Vernal kerato conjunctivitis. Int. Oph. Clinic. 28:303-307, 1988. 40. Sharma A., Gupta R., Ram J. and Gupta A. : Topical ketrolac 0.5% solution for treatment of vernal kerato conjunctivitis. Ind. Jr.Ph. 45 : 177-180, 1997. 41. Allen Smith M.R. : Giant paipllary conjunctivitis in Contact lens wearer. AJO 83:697708, 1977. 42. Srinivasan B.D. : Giant papillary conjunctivitis with ocular prostheses. Arch Oph. 97 : 892-895, 1979. 43. Allen Smith M.R., Ross R.M. : Giant papillary conjunctivitis. Int. Oph. Clinic. Vol. 28, 309-316, 1988. 44. Deborah Pavan Langston and Foulks G.N. : Phlyctenular kerato conjunctivitis in Manual of ocular diagnosis and therapy. Third edition, p-105, Little Brown, 85. 45. Duke Elder S. : Phlyctenular kerato conjunctivitis in System of Ophthalmology. Vol. VIII. Part one, p-461-471, Henry Kimpton, London 1965. 46. Tabbara K.F. : Phlyctenulosis in Current ocular therapy. Fifth edition. p-373-374. Edited by Fraunfelder F.T. and Hampton Roy F., WB Saunders Company, Philadelphia 2000. 47. Wagoner M.D., Bajarat A.M., Allan Smith M.R. : Phlyctenulosis in Current ocular therapy. Fourth edition. p-509-572, Edited by Fraunfelder F.T. and Hampton Roy F., WB Saunders Company, Philadelphia 1995. 48. Phlyctenular kerato conjunctivitis in Clinical ophthalmology. Vol. IV 8:1-6. Harper and Row, Philadelphia 1986. 49. Felberg N.T., Michelson J.B. : Stevens-Johson Syndrome in Pediatric ophthalmology. Vol. 2, second edition p-1178. Edited by Harley R.D., WB Saunders Company, Philadelphia 1983. 50. Dutta L.C. : Erythema multiformis major in Ophthalmology principle and practice. First edition. p-35, Current Books International, Calcutta, 1995. 51. Fraunfelder F.T. and Fraunfelder F.W. : Erythem multiformis major in Current ocular therapy. Fifth edition. p-145-146. Edited by Fraunfelder F.T. and Hamptom Roy F. W.B., Saunders Company, Phildelphia 2000. 52. Mukherjee PK : Rhinosporidiosis in Current ocular therapy. Fifth edition. p-66-67. Edited by Fraunfelder F.T. and Hampton Roy F., W.B. Saunders Company, Philadelphia 2000. 53. Mukherjee P.K., Shukla I.M., Deshpande M., Praveena Kher : Rhinosporidiosis of the lacrimal sac. Ind. Jr. Op. 301 : 513-514, 1982.

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54. Shukla IM, Mukherjee P.K., Sushma Verma : Primary rhinosporidiosis of the eye. Int. Congress of Ophthalmology. ACTA XXVI Vol. 2, 864 : 1982. 55. Lamba P.A., Shukla K.N., Ganapathy M. : Rhinosporidiosis granuloma of conjunctiva with scleral ectasia. Br. J. Oph. 54. 565-568, 1970. 56. Mukherjee P.K., Agrawal S. : Subconjunctival twin cysticercosis. Ind. Jr. Oph. 23 : 2829, 1975. 57. Malik S.R.K. Gupta A.K., Chaudhari S.K. : AmJr. Oph. 66. p-1168, 1968 59. Topilov H.W. : Cysticercosis in current ocular therapy. Fifth edition. p-92-94. Edited by Fraunfelder F.H. and Hampton Roy F., W.B. Saunders Company, Philadelphia 2000. 60. Raina U.K., Taneja S. Lamba P.A., Bansal R.A. : Spontaneous extrusion of extra ocular Cysticercus cyst. Am Jr. Oph 124 : 438-441, 1996. 61. Harley R.D. : Systemic protozoan and metazoan diseases in Pediatric ophthalmology. Vol. II, Second edition. p-931-937, W.B. Saunders and Company, Philadelphia 1983. 62. Lerners S.F. Echinococcsis : In Current ocular therapy. Fifth edition. p-95-96. Edited by Fraunfelder F.T. and Hamptom Roy F., W.B. Saunders Company, Philadelphia 2000. 63. Mukherjee P.K., Sushma Verma, Agrawal S. : Intraocular thelaziaA case report. Ind. Jr. Op. 25 : 41-42, 1978. 64. Mukherjee PK : Thelaziasis in Current ocular therapy. Fifth edition. p-100-101 Edited by Fraunfelder F.T. and Hampton Roy F., W.B. Saunders Company, Philadelphia 2000.

CHAPTER

Disorders of Cornea in Children


Anatomy of cornea1, 2, 3, 4. Cornea is the anterior most part of the outer coat of the eyeball. It is transparent. Its main functions are optical and protective. Cornea forms roughly one third of the outer coat. It sits over the anterior scleral foramen like shining crystal clear watch glass with convexity anteriorly. As cornea is more curved i.e. 7.8 mm than sclera (12 mm) it seems to bulge from the sclera resulting into a step like sulcus at the corneo scleral junction. The bulge is 2.7 mm from the plane of the limbus. Cornea occupies anterior 1.3 sq.cm5 of the eyeball. Though on casual look cornea seems circular, in fact it is not so. Corneal diameter is not equal in horizontal and vertical meridian. Horizontal diameter is longer i.e. 11.6 mm than vertical diameter i.e. 10.6 mm. Corneal diameter more than 12.7 mm is called megalocornea while smaller than 10 mm is called microcornea. This difference of one milliter makes the cornea horizontally oval. The cause of this difference is due to constant pressure of the lid over the cornea making the cornea more curved vertically resulting it more myopic vertically. Corneal curvature is not equal on both the surfaces. Had the curvature been equal on both surfaces, the corneal thickness would have been uniform throughout its length and width. Posterior corneal surface is more curved than the anterior surface. This makes cornea thinnest at the centre and thicker at the periphery. Corneal thickness is 0.52 mm in the centre, it increases to 0.70 mm in the periphery. Central corneal thinness makes cornea more prone for perforation than periphery. The central 4.0 mm that is called optical zone and is almost spherical. The corneal surface gradually flattens on the periphery in adults. In new born cornea is flatter than in adult and its curvature is more in the periphery. Mean corneal diameter at birth is 10 mm, it increases rapidly to reach almost adult size by the end of the first year. The cornea of a new born looks larger because of narrow interpalpebral fissure. Cornea of a new born is slightly hazy that clears within few days. Corneal layers. The cornea comprises of following distinct anatomical layers6,7 : 1. Epithelium 3. Stroma 5. Endothelium 1. The epithelium. The corneal epithelium is continuous with conjunctival epithelium, making it easy for infection to travel from conjunctiva to cornea. The anterior epithelial surface has microvilli that anchor the tear film to the corneal surface. The epithelium is relatively impermeable to water soluble substances. The epithelium acts as effective barrier to 159 2. Bowmans membrane 4. Descemets membrane

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many micro-organism. Thus most of the micro-organism can not invade the cornea so long as the epithelium is intact. The organisms that can bypass the epithelial barrier are gonococcus, meningococcus and C. diptheria. The corneal epithelium is most mitotically active, thus healing of the epithelium is very fast. The corneal epithelium can be rubbed or pealed off with ease. 2. The Bowmans membrane is not a true membrane. It is formed by condensation of anterior layer of stroma. It is one millimetre shorter than rest of the cornea in all directions. The shortness of the Bowmans membrane is because its development is completed before full development of rest of the cornea. Bowmans membrane is easily destroyed and does not degenerate. 3. The stroma forms 90% bulk of the cornea and is continuous with the sclera. 4. The Descemets membrane is the basal membrane of corneal endothelium. Its thickness increases with age. It is tough and can withstand considerable amount of raised intraocular pressure and offers resistance to infection. 5. Corneal endothelium is single layered structure that is metabolically very active. It is continuous with endothelium of the iris. The endothelium maintains deturgescence of the cornea, keeping it transparent and bright. Damage to endothelium leads to edema of the cornea. The damage can be brought about by trauma, inflamation, toxin and dystrophy. Whole of the cornea has high metabolism for which it requires constant supply of oxygen from the atmosphere that is dissolved in tear film, when the lids are open. During sleep, the oxygen permeates through the vessels of tarsal conjunctiva. The other source of nutrition to the avascular cornea is from limbal blood vessels which are present only in the peripheral 1 mm all round. The aqueous supplies nutrition to the endothelium. The nerve supply of the cornea is by trigeminal nerve via ciliary nerve. The nerve fibers enter the stroma from sclera, and conjunctiva. The cornea is very sensitive to pain, all sensation i.e. touch temperature and pressure are converted to pain sensation. Physiology of The Cornea11, 12, 13, 14 The two functions of the cornea are1. Optical, and 2. Protective. 1. Optical. For light to travel uninterrupted with out scatter, the cornea must have (a) An optically regular surface and (b) A transparent medium. Both these are met with due to a smooth epithelial surface, constant supply of nutrient, relative dehydration. The cornea transmit cent percent of white light i.e. 380 nm to 760 nm. Nutrition of Cornea11, 12, 14 Cornea is highly metabolic tissues. It requires constant supply of oxygen and energy. The oxygen is derived (1) mainly from the atmospheric air dissolved in tear film.(2) from the perilimbal blood vessels (3) from the tarsal conjunctival vessels when the lids are closed. Other nutrients are supplied by the aqueous from behind. The energy to the cornea is supplied by metabolism of glucose in the form of adenosine triphosphate (A.T.P.). The epithelium can metabolise glucose both aerobically and anaerobically. Most of the metabolism takes place in the epithelium and endothelium. There are three metabolic pathways

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involved in metabolism of cornea. They are 1. Glycolysis 65% (2) Kerb-cycle (3) Hexose monophosphate shunt. Corneal transparency depends upon following factors : 1. Anatomical 2. Deturgscence of cornea 3. Intraocular pressure 4. Uniform refractive index of various layers. The anatomical factors are : Absence of vessels in the centre of the cornea. There are no pigments in the cornea, the epithelium is multi-layered but the cells are arranged in a regular fashion. The epithelium is ten times thicker than endothelium yet metabolic requirement of endothelium is more than epithelium. The single layered endothelium is arranged in a regular fashion so are the stromal collagen fibrils. The epithelial cells which are exposed to atmosphere for long time remain non keratanised in normal eye. The tear film obliterates whatever irregularity the epithelial surfaces may have in normal eye to give a better optical surface. Deturgscence of cornea. The normal cornea should remain in a state of relative dehydration. Accumulation of fluid causes the stroma to lose its transparency. The state of relative dehydration is done by active transference of fluid from stroma towards epithelium and by way of an endothelial pump system from stroma. Loss of endothelial cells is result of embibement of fluid in stroma and epithelial edema. Intraocular Pressure - Acute rise of intraocular pressure causes derangement of fluid transport across the stroma and causes stromal edema and loss of transparency. Refractive index. The cornea is most important refractive surface of the eye. The anterior surface has more important role as refractive surface than posterior surface. The refractive index of cornea is 1.38. The cornea protects the eye from most of organism and cuts off ultra violet rays as well. It helps to maintain the structural integrity of the globe. Development of cornea6, 7, 8, 9, 10. Cornea develops from two sources, surface ectoderm and mesoderm. The former gives rise to corneal epithelium while rest of cornea is mesodermal in origin. As the lens vescicle separates from the surface ectoderm a space develops. This space is invaded by mesoderm that gives rise to all corneal layers except the epithelium. Endothelium cells are first to be differentiated at 12 mm (5 weeks) stage. The Descemets membrane is visible at 12 weeks. The Bowmans membrane is recognisable at 100 mm (4 months). This is the stage when corneo scleral junction is also well defined. Congenital anomalies of the cornea. Congenital anomalies of the cornea may be localised to the cornea only or associated with other anomalies of the globe. They may be unilateral or bilateral, need not be symmetrical in bilateral cases. Common congenital anomalies of cornea are : Microcornea Megalocornea Cornea plana

162 Keratoglobus Keratoconus26,27,28,29 Anterior embryotoxon Sclero cornea Corneal dystrophies

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Microcornea5, 16, 17. Term microcornea is used to denote a condition where the corneal diameter is less than 10 mm in otherwise normal eye. In these cases whole of the anterior segment is small, hence the term anterior microphthalmia is also used to denote the same condition. The recti are inserted more anteriorly than normal eyes. Corneal curvature is flat, resulting into hypermetropia. Other types of errors of refraction are also possible secondary to variation in curvature of cornea and shape of the lens in relation to the cornea. The inter palpebral fissure is narrow. All microphthalmos eyes have small cornea but all eyes with micro cornea need not be microphthalmic. Sometimes it is difficult to differentiate between pure micro-cornea and microphthalmos especially if there are other associated anomalies of the globe like coloboma, microphakia, mesodermal tissue in angle of anterior chamber. In about one fifth of cases there may be glaucoma due to anomalies of the angle. It should not be confused with another congenital anomaly of the globe i.e. nanophtalmos where the eye is small in all meridian but is normal in function. Megalocornea. This condition is rarer than microcornea and microphthalmos, condition is bilateral, non progressive where anterior segment of the eye is larger than normal. The corneal diameter varies from 13 mm to 16 mm. As the diameter is more there is increase in circumference of the cornea. Corneal curvature is generally normal but may be increased with myopic astigmatism, which is astigmatism with the rule. In absence of myopia vision is unaffected. Anterior chamber is deep. The lens may be large. There may be both phacodonesis as well as iridodonesis. Two common complications met with the condition are glaucoma and cataract. The condition must be differentiated from more serious, progressive vision threatening buphthalmos and keratoglobus that is almost symptomless.
Megalocornea 1. Bilateral, symmetrical 2. Non progressive 3. Exclusively seen in boys 4. Astigmatism with the rule 5. Tension-Normal 6. Cornea clear and bright 7. No lacrimation 8. Angle-normal or may have minor congenital anomalies. 9. No disc change 10. Slight functional defect Buphthalmos 35% cases are unilateral. In bilateral cases involvement may not be symmetrical in two eyes. Progressive Boys to girlsratio is 5 : 3 Astigmatism against the rule. Tension raised Cornea cloudy due to rupture in Descemets membrane Lacrimation prominent feature. Main pathology is gross malformed angle. Cupping prominent Gross functional defect.

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Cornea plana. In this rare condition corneal curvature is reduced. In extreme cases cornea may be flat generally associated with decreased scleral curvature. Sclera may encroach over the cornea giving an impression of small cornea. Sometimes cornea itself is small. There may be diffuse opacities in the cornea. Due to flattening of cornea there is impression of ptosis. The eyes are generally hypermetropic. Anterior chamber is shallow. There may be associated glaucoma. Causes of poor vision is curvature hypermetropia and corneal opacity. Differential diagnosis consists of micro-cornea, microphthalmos, phthisis, atrophic bulbae and nanophtalmos. Keratoglobus. This is a relatively rare bilateral condition where corneal diameter is normal but the corneal curvature is increased. The cornea becomes globular in contrast to keratoconus where cornea is conical. In keratoglobus the cornea is thinner than normal almost throughout. The sclera may also be thinned. The condition is stationary and symptomless cases are detected on routine examination. Tension and angle remain normal throughout. Differential diagnosis include all cases of enlarged cornea and corneal causes of deep anterior chamber. Other members of the family may show keratoconus or irregular myopic astigmatism. It should also be differentiated from pellucid degeneration14,15 of cornea that is generally seen after 30 years of age and has a toric bulge in the lower part of the cornea. Keratoconus (Conical cornea)18, 20, 21, 22, 23, 24, 25. Keratoconus is a non inflammatory ectasia of central cornea due to obscure causes. Some authors classify it as congenital disorder presuming that there is failure of mesoderm from periphery to centre, resulting into weakness. It is worth noting that most of the pathological changes are seen in corneal structure that are mesodermal in origin. Others have put keratoconus under degeneration27 while most put it under dystrophy. Keratoconus is suspected to be due to a kerato softening enzyme14. Keratoconus has world-wide distribution, no race is immune. Proponents of theory of congenital anomaly feel that subtle changes are present years before the disorder becomes manifest. The disease is bilateral and progressive. One eye is generally more involved than the other. In rare instance the disease has been described as unilateral, in such cases the other eye shows irregular myopic astigmatism without corneal thinning and ectasia. The disease is said to be more common in girls. Generally symptoms start round ten years and progress for eight to ten years, then either the progress comes to standstill or is very slow.7 Increase in keratoconus has been noted up to fourth and fifth decade. In few cases there is a steady increase in keratoconus followed by a phase of non progression to be followed by a period of progression again. Generally puberty heralds a year or two of rapid progression. Though contact lenses are prescribed as treatment of keratoconus they may occasionally worsen it. Repeated rubbing of eye has also been thought to be a contributory factor.27 Keratoconus is non hereditary in 95% of cases. In remaining it can either be autosomal dominant or recessive. Keratoconus generally presents as isolated ocular disorder, however, some ocular disorders are more frequently associated with keratoconus than others. These areSpring catarrah, retinitis pigmentosa, ectopia lentis, anirida, anterior polar cataract, blue sclera. Systemic conditions that are frequently associated - atopic dermatitis, Down syndrome Marfans syndrome, Lebers familial amaurosis, Ehler-Danols syndrome, neurofibromatosis, mitral valve prolapse.

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Clinical features consist of diminished distant vision which is progressive, glare, photophobia, uniocular diplopia. Signs. Vary according to severity of the case : In early stages the corneal bulge is minimal and can not be detected on routine examination. Retinoscopy reveals compound myopic astigmatism with oblique axis. Sometimes astigmatism is irregular which is confirmed by Placido disc, Klein keratoscope, and keratometery. Computerised corneal topography is more informative than keratometery. Corneal mapping through photo keratoscope detect very early and subtle changes. Slit lamp biomicroscopy shows fine vertical lines at the level of deep stroma and Descemets membrane, the lines disappear on pressure over the globe. Sometimes prominent corneal nerves are also visible. Late cases do not present any difficulty in diagnosis. In all sporadic cases of increasing myopic astigmatism, keratoconus should always be kept in mind. On oblique illumination, the cornea looks conical on profile with deep anterior chamber. Generally the distance of corneal vertex to plane of limbus is 2.5 mm. In keratoconus this may increase to 4.5 mm to 5 mm. In normal cornea, if a narrow beam of light is thrown at the corneoscleral junction from lateral side, the opposite limbus lightsup in a circular fashion. In case of keratoconus, the light reflex on the other side is conical.28 If the patient is asked to look down, the apex of the cornea produces a notch in the lower lid (Munsons sign). The apex of the cone is not opposite the centre of pupil, it is shifted downwards and medially. The apex may show some opacity. On keratoscopy and keratometry the findings are exaggerated. Keratometer may fail to measure the cone fully and may require additional plus lenses in the keratometer to measure the curvature completely. Retinoscopy with plane mirror shows a central dark spot, corresponding to apex of the cone, surrounded by a zone of pink retinoscopic glow, surrounded by a dark rim and then a pink ring up to periphery. The intermediate dark rim represents the base of the cone where there is near total internal reflection. The retinoscopy shows scissors movements and the axis is generally oblique. On slit lamp biomicroscopy with a bright narrow beam, the central part looks thin while peripheral cornea has uniform, normal thickness. In 50% of eyes an incomplete yellow green ring is visible at the base of the cone. This is due to deposition of hemosiderin superficial to Bowmans membrane. In advanced cases there may be rupture in Descemets membrane, leading to photophobia and lacrimation. Sometimes there may be rupture in Bowmans membrane as well. Hydrops of keratoconus denotes acute edema of the cornea due to rupture of Descemets membrane resulting into imbibement of aqueous through endothelium. The whole of the cornea becomes opaque, associated with pain, lacrimation and rapid fall of vision. The stage of corneal hydrop lasts for 6-10 weeks and clears with healing of the rupture, however, it leaves some degree of stromal scarring.

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Though perforation in high degree of keratoconus has been reported, it is otherwise very rare. Management of keratoconus29 1. Initially fairly good vision is attained by prescription of spectacles which require frequent changes. 2. In moderate cases where spectacles do not give satisfactory visual improvement contact lenses are prescribed initially, later on special piggy back contact lenses which are a combination of hard and soft contact lenses are prescribed where a hard lens is fitted over soft lens. However, sometimes contact lenses have been reported to worsen keratoconus. 3. Penetrating keratoplasty with a large donar button have very good prognosis, however, induced astigmatism may require correction by contact lens. Thermo keratoplasty, lamelar keratoplasty and epikeratoplasty have not proved better than penetrating keratoplasty, which is a definitive surgical treatment for keratoconus. Acute hydrops may require lowering of tension by systemic acetazolamide and betablocker. Local instillation of hypertonic solutions, patching or use of a bandage lens may reduce discomfort. Local non-steroidal anti-inflammation drugs along with cycloplegic give relief from pain and photophobia. Generally steroids are not required. Common Corneal Disorders Seen in Children Children are prone to suffer from various corneal disorders. Some are very mild and may not reduce vision. Some may reduce vision marginally that pass off with treatment. Other cause extensive visual damage that may be permanent even with best treatment. Many of the corneal disorders are preventable. Common corneal disorders seen in children are : 1. Congenital anomalies 3. Infection 5. Nutritional 7. Dystrophies Malignancies of cornea are not known, however a growth of conjunctiva at limbus may invade the cornea. Generally such growths are seen only in adults except the limbal dermoid that may be present at birth or may manifest later. Some Pathological Signs of Corneal Disorder Seen in Children are Disorders of cornea produce following changes in cornea : 1. MorphologyShape, size, curvature, surface, thickness 2. Loss of transparencyOpacity, edema, vascularisation, deposits. 3. Diminished sensation 4. Ulcerationits sequel and complication. 5. Xerosis 6. Degeneration 7. Dystrophies 2. Trauma 4. Inflammation 6. Degeneration

166 Morphological changes in cornea :

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Shape. Cornea looks circular on casual look, however, it is elliptical in shape when seen from front. Its vertical diameter is less than horizontal diameter. The difference becomes obvious when measured by a corneal calliper. In fact the measurement in children should be taken under general anaesthesia along with intraocular tension. In older children local anaesthesia is sufficient. The cornea becomes horizontally oval in soft eyes i.e. phthisis, perforated globe due to pressure of lids. In extreme degree of softness it becomes quadrilateral. Rarely the cornea may be oval vertically or horizontally as a congenital anomaly. There may be an apparent change in corneal shape with encroachment of dermoid or limbal papillae of spring catarrah. Size of cornea : 1. Small cornea. A cornea smaller than 10 mm is called microcornea, while those larger than 13 mm are known as megalocornea. Small corneas are seen as congenital anomalies like microcornea, microphthalmos and nanophtalmos. In micro-cornea it may be as small as 3-4 mm. Other causes of small cornea are perforated globe, phthisis bulbae and atrophic bulbae. 2. Large cornea. Cornea larger than 13 mm is seen in buphthalmos, megalocornea and keratoglobus. Myopic eyes have larger cornea than hypermetropic eyes. 3. Curvature. Curvature of corneaIn normal life, corneal curvature can be appreciated by 1. Looking from temporal side when the patient looks at a distant object. 2. Looking at the depth of AC. 3. Asking the patient to look down. Cornea with normal curvature does not produce any localised dent in the lower lid. In case of increased curvature, the apex of the cornea causes a notch in the middle of the lower lid when patient looks down (Munsons sign). Accurate measurement of cornea is done bykeratoscope, keratometer and photo keratoscope. Corneal curvature is increased inKeratoconus, buphthalmos and keratoglobus. In case of partial corneal staphyloma, the curvature may be increased irregularly. In pellucid degeneration of corneathere is a localised bulge in the lower part. It develops in third decade. Corneal surface. Normal cornea has a smooth, shinning surface that acts as a convex mirror forming miniature, virtual erect image of the object in front of the cornea without any distortion or haze. Irregularity of the corneal surface can be observed by window reflex keratoscope, keratometer, computerised corneal topography. Causes of irregular corneal surface areCorneal ulcer, corneal scar, pterygium, keratoconus, deposits on cornea i.e. foreign bodies, band keratopathy, corneal plaque. Other causes areKeratectasia, adherent leucoma, corneal staphyloma, keratoplasty, growth at the limbus (Dermoid), bulbus keratopathy.

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Corneal brightness is diminished in : 1. loss of corneal epithelium i.e. corneal ulcer, trauma. 2. Corneal opacities 3. Deposits on the cornea 4. Epithelial edema, and stromal edema. 5. Vascularisation of cornea both superficial and deep Changes in corneal thickness. Cornea is not uniform in its thickness. It is thicker at the periphery and thinnest in the centre. Clinically thickness is measured by pachymeter. Increase in corneal thickness is seen in : 1. Corneal edema. 2. It is more marked if endothelium is damaged than epithelium. 3. The other causes of increased corneal thickness is congenital hereditary endothelial dystrophy. Reduced thickness is seen in keratoconus, buphthalmos, Desmetocele corneal ulcer. Changes in Corneal Transparency Main function of cornea is optical. For this it has to remain in a state of utmost transparency that is maintained by multiple factors.2,5 They are : 1. Uniform and regular arrangement of epithelial cells. 2. Uniform size of stromal cells, their uniform arrangement and compactness. 3. Virtual absence of vascularisation. 4. Transparent corneal nerves and a 5. Constant state of deturgescence. Deturgescence of cornea is a state of relative hydration of corneal tissue that is maintained by Na+ K+ cell pump of endothelium and epithelium. Though endothelium and epithelium both contribute in maintaining corneal deturgescence, former has more pronounced effect than the later. Injury to endothelium by trauma, infection or toxin has more serious effect than epithelial damage. The epithelium regenerates quickly thus damage to epithelium is short, it causes localised swelling of epithelium and is transient causing diminished vision for short time. Integrity of epithelial cells depend on (1) Tear film and (2) Available oxygen. Derangement of any of the above factor alone or in combination can cause loss of corneal transparency that may be short lived or permanent. Commonest cause of loss of transparency is replacement of transparent keratocytes by irregularly arranged opaque fibrocyte, due to trauma, infection, inflammation or infiltration. Other causes are superficial and deep vascularisation, incarceration of uvea, raised intraocular tension, degeneration, dystrophies, deposits on the cornea, epithelial down growth, growth of conjunctiva or conjunctival tumour spreading over cornea. Loss of transparency of cornea is called corneal opacity that can be unilateral or bilateral. Shape, size, number and depth varies according to causative factors and duration. Some

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of the opacities are permanent, other may disappear with treatment or spontaneously. Some of the opacities may increase in size, number or depth. Central corneal opacities cause more visual loss. Peripheral opacities with clear pupillary area generally have only cosmetic effect. Corneal opacities in the centre of cornea not only block the light rays from reaching the pupil but also cause scattering of light and irregular astigmatism. Rarely corneal opacities can be congenital. Causes of Corneal Opacities in Children Corneal opacities in children can be caused by any of the following separately or in combinationCongenital, infection, inflammation, allergy, trauma, malnutrition, degeneration and dystrophies. Congenital corneal opacities : Cornea of new born is mildly hazy. The haze gradually disappears in next two to three days. If cloudiness persists following causes should be excluded : 1. Intrauterine infection 2. Birth trauma 3. Infantile glaucoma, mucopolysccharidosis and mucolipidosis. The congenital causes of corneal opacity includessclerocornea. Corneal opacities in children have far reaching consequences not only regarding vision but also mental development. The child may remain permanently visually challenged. Thus corneal opacities are one of the most common but neglected cause of amblyopia and squint when unilateral and nystagmus when bilateral. Causes of bilateral corneal opacities in children (on the basis of age of onset) : 1. New born 2. Neonate 3. First year 4. Two to five years 5. Five year and above Congenital Ill managed ophthalmia neonatorum Xerophthalmia, congenital glaucoma Xerophthalmia, trauma, bacterial corneal ulcer, congenital glaucoma, interstitial keratitis Trauma, xerophthalmia, bacterial corneal ulcer, keratoconus, phlyctenular keratitis, spring catarrah, corneal dystrophies, band keratopathy, interstitial keratitis, limbal dermoid.

Corneal Edema To maintain optical clarity, cornea must remain in a state of relative hydration. Accumulation of fluid in cornea causes it to swell up. Corneal edema may be localised in epithelium, stroma or may involve both. The corneal epithelium and endothelium regulate passage of fluid and ions. Deranged fluid transport across the cornea causes corneal edema. Epithelial edema causes more visual loss due to irregular astigmatism that results following accumulation of fluid in the basal cells of the epithelium and forming a bullae. Epithelial edema presents as diminished vision specially on awakening. In well formed bullae blinking causes foreign body sensation that becomes more marked if the bullae ruptures. There may be

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associated pain, redness and photophobia. Ruptured bullae may result into anterior chamber reaction and formation of sterile hypopyon or may lead to bacterial corneal ulcer. Stromal edema causes less loss of vision and less symptoms. It results in haziness of stroma and thickening of cornea. Common causes of corneal edema are : 1. Trauma to endotheliumaccidental or surgical during IOL implant, placement of glaucoma valve, penetrating keratoplasty. 2. Trauma to epitheliumIll fitting contact lens, chemical burn. 3. Aphakia (a) Due to vitreous in AC (b) Toxicity of irrigating fluid (c) Trauma during manipulation of IOL (d) Silicone oil in AC. 4. Acute rise of tension, persistent raised tension, rupture of Descemets membrane. 5. Keratoconusacute hydrops 6. Herpctic disciform keratitis 7. Fuchs dystrophy 8. Chronic uveitis 9. Keratocele (Descemetocele) 10. Iris incarceration 11. Corneal staphyloma (Anterior staphyloma). Corneal neovascularisation15, 30, 31 Out of all refractive media i.e. cornea, aqueous, lens and vitreous, cornea has maximum refractive power. For a sharp retinal image all factors remaining within normal limits cornea must remain transparent. Avascularity of cornea plays a very important role in keeping cornea bright and transparent. Cornea is almost avascular except 1 mm of periphery adjacent to the limbus and vessels do not grow into corneal substance so long cornea is not diseased. Growth of vessels in cornea is blocked by compactness of corneal tissue and anti vascular chemical factors present in the cornea. Once the compactness of cornea is lost, vessels start growing into the substance of cornea both in epithelium and stroma. The anti vascular factor may be overwhelmed by a vasoformative factor in newly formed vessels. Vessels once formed in cornea never disappear, they may get obliterated leaving tell a tale sign of ghost vessels which are more prominent in deep vascularisation. Presence of corneal vascularisation is always pathological. Factors that encourage corneal vascularisation aretrauma, infection, inflammation, hypoxia, hypo vitaminosis B, allergy and degeneration. Corneal vascularisation can be put into three groups depending upon the layer at which they develop i.e. 1. Superficialmostly epithelial 3. Retro corneal 2. Interstitialmostly stromal

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Superficial vascularisation of cornea is commonest form of vascularisation. This is due to extension of conjunctival vessels into the cornea in front of Bowmans membrane. According to its extent in the cornea it is divided into 1. Micro pannus, 2. Gross pannus. Pannus is a fibro vascular proliferation that extend up to the cornea, it is essentially a defensive process against infection, toxin, trauma etc. It can be sectorial or may involve whole of corneal periphery. It is always preceded by infiltration of corneal epithelium by polymorphonuclear and mononuclear cells. Cellular infiltration is followed by development of tufts of new vessels. It may destroy the Bowmans membrane causing corneal haze. There are three clinical stages of superficial corneal vascularisation i.e. (1) Progressive stage, (2) Stationary stage, (3) Regressive stage. In the progressive stage, blood vessels grow towards the centre of the cornea. They may be parallel to each other. A zone of cellular infiltration extends beyond the tip of the vessel terminal. In regressive stage the zone of infiltration is between the tip of the blood vessels and the limbus, other things remaining the same. Superficial vascularisation can be seen asmicropannus or as gross pannus. Micropannus30 extend only one to two millimeter beyond the normal limbal arcade. It is most commonly seen is contact lens wearer. Milder form of chlamydia kerato conjunctivitis, vernal catarrh, chronic blepharitis. Gross pannus extend more than 2 mm beyond normal limbal vessels. It is seen in trachoma, phlyctenulosis, rosaceakeratitis, atopickerato conjunctivitis, contact lens wearer, severe blepharoconjunctivitis, herpetickeratitis, riboflavin deficiency, corneal graft rejection. In gross pannus sometimes there may be a large and prominent vessel which may be very prominent or there may be a tuft of localised vessels as seen in fascicular ulcer. Deep vascularisation of cornea In this condition anterior ciliary blood vessels invade the cornea at the level of stroma. They have very characteristic feature : 1. They are generally straight and parallel to each other. 2. They do not anastomose with each other. 3. Branch at an acute angle from the main branch giving a bottlebrush appearance. 4. They are generally sectorial in distribution. 5. The vessel seem to disappear at limbus. 6. The vessels grow in the particular plane and remain in the same plane. 7. They are always associated with deep keratitis, and uveitis. 8. When severe, they give a pink hue to the cornea known as Hutchinsons salmon patch. 9. Rarely there may be associated superficial vascularisation at the level of epithelium giving an epaulet appearance to the peripheral cornea.

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10. Deep vascularisation gives a ground glass appearance to the cornea. 11. When the inflammation is under control the vessels obliterate leaving a track of ghost vessels that last for rest of the life. Causes of deep vascularisation areCongenital syphilis, tuberculosis, leprosy, herpes simplex, chemical burn. Retrocorneal vascularisation. This type of vascularisation is mostly seen when blood vessels from uvea develop on the endothelium of the cornea either due to chronic uveitis, diabetic neovascularisation or thrombosis of retinal vein. They may also grow from conjunctiva followingpenetrating injury at the limbus, penetrating keratoplasty, and conjunctival epithelial down growth. Inter corneal vascularisation. This happens rarely when limbal vessels grow under the partial thickness wound of the cornea either accidentally or following large lamellar graft. Rupture and folds in Descemets membrane32, 33, 34 : Descemets membrane is likely to rupture : 1. If the eyeball is compressed suddenly like in birth injury or concussion injury to the globe. 2. The cornea stretches either due to thinning as seen in keratoconus and buphthalmos. Rupture of Descemets membrane is more common in buphthalmos than in keratoconus. In buphthalmos there is not only thinning of the cornea but also an enlargement of cornea that stretches the Descemets membrane. The tears differ in shape, size, number and position. With ophthalmoscope they stand out as dark double lined areas in red background. The area between the two lines also give a red glow. They may be linear, curvilinear or whirl like. Their presence in buphthalmos is known as Haabs line which are curvilinear. Opaque lines that represent healed breaks in Descemets membrane. With rupture of Descemets membrane there is a sudden influx of fluid into the stroma leading to stromal edema, lacrimation and photophobia. Stromal edema may subside with treatment but may persist to cause permanent scarring of cornea that is so common in untreated or poorly managed buphthalmos. Ruptures due to elevated IOP are either horizontal or parallel to limbus. Rupture in Descemets membrane due to trauma are generally central and vertical. Some of the dystrophies may look similar to Haabs striae. Folds in Descemets membrane are more common than tears. They can be caused by trauma, both blunt as well as incised wound. Other causes are prolonged uveitis with hypotony. Keratic precipitate (see diseases of uvea) Loss of corneal sensation. Cornea is very richly supplied by sensory nerve endings of trigeminal nerve. There is only one sensation in cornea i.e. pain. An intact corneal sensation is essential for health of cornea specially the epithelium. It is of diagnostic significance not only for local corneal diseases but also neuro ophthalmic diagnosis. Corneal sensation may be marginally reduced or may be totally absent. Causes of diminished corneal sensation are : 1. Recent use of local anaesthetic agent, 2. Use of contact lens.

172 3. Herpes simplex, herpes zoster, leprosy. 4. Corneal edema, corneal scar, corneal vascularisation. 5. Buphthalmos 6. Repair of corneal wound, keratoplasty 7. Vitamin A deficiency 8. Deposits on cornea i.e. band keratopathy, tattoo 9. Cerbropontine angle tumour 10. Trigeminal neuritis Xerosis of cornea (see xerophthalmia) Corneal Ulcer in Children

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Inflammation of cornea secondary to micro-organisms is called keratitis. Keratitis with loss of epithelium is called corneal ulcer. A large group of cases which are not caused by microbes are grouped under a loose term - Keratopathy. Keratitis in children basically does not differ from those seen in adults, however, complications are more in children. Most of keratitis in spite of good management leave corneal opacity that may produce amblyopia and squint. Keratitis in infancy may be responsible for nystagmus. Hence keratitis requires prompt diagnosis and efficient management. Keratitis in general has been described under various heading. However most accepted classification by Duke Elder 1565 on the basis of etiology still remains most widely accepted classification. Though it too has its short comings. The classification is as follows Keratitis35 : 1. Superficial keratitis 3. Deep keratitis 1. Superficial keratitis has been divided into (a) Diffuse and punctate keratitis (b) Corneal ulcer (c) Degenerative superficial keratitis. (a) Diffuse superficial keratitis can be (i) Acute superficial keratitis as seen in acute bacterial and viral conjunctivitis that may result in corneal ulcer. (ii) Chronic superficial keratitis is seen mostly in trachoma, leprosy, mollouscum contagiosum, phlycten. Chronic superficial keratitis is characterised by pannus formation. Superficial keratitis can be diffuse or punctate. Punctate keratitis stain irregularly and poorly with fluorescein but better with rose bengal. A lesion may stain partly with fluorescein and partly with rose bengal depending upon level of the lesion. The unstained lesions have a whitish appearance. The size, shape and distribution of superficial punctate keratitis vary depending upon severity and duration of the 2. Parenchymatous keratitis

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lesion. They may be localised in epithelium, may be sub-epithelial, there may be a combination of both forms in the same eye. They may be primary corneal or secondary to conjunctival or lid lesions. Sometimes they are seen with various type of skin lesions. Common conditions associated with punctate keratitis are : Staphylococcal blepharoconjunctivitis, trachoma, inclusion conjunctivitis, herpes simplex and zoster, molluscum, vernal conjunctivitis, ultra violet light, trichiasis, juvenile rheumatoid arthritis. Management of superficial keratitis. There is no specific treatment. The probable primary causes should be treated with appropriate antibiotic. If there is photophobia and anterior chamber reaction, short acting cycloplegic like home atropine 2%, pad and bandage at bed time gives early relief. However, the lids should be examined for misdirected lash, blepharitis or foreign body in tarsal plate especially in unilateral cases. Corneal Ulcer35, 36, 37 Corneal ulcer is an inflammatory process (infective or sterile) where there is an epithelial defect with some loss of stroma. However some of the processes like epithelial keratitis may be limited to epithelium. Infectious corneal ulcers are generally associated with stromal infiltration. The epithelial defect stains with fluorescein. Infective corneal ulcer is generally associated with anterior chamber reaction. General consideration : 1. No age, sex or race is immune to corneal ulcer, however, they are more common in second to fourth decade. 2. Corneal ulcers are generally uniocular unless associated with systemic diseases like Vitamin A deficiency, ophthalmia neonatorum, leprosy, blast injury, chemical burn. 3. A breach in the epithelium is a pre-requisite for all infective corneal ulcers exceptgonococcus, meningococcus, coryne bacterium diptheriae. Epithelial break is generally due to trauma, may be a mild abrasion or the traumatic factor may be persistent like ill fitted contact lens, trichiasis, entropion, concretion, foreign body on the cornea or tarsal plate. 4. Intact protective mechanism saves cornea from trauma. Failure in protective mechanism anatomical or neurological predispose corneal ulceration (a) Coloboma of the lid, ectropion of lid, entropion of lid, proptosis. (b) Lagophthalmos (c) Loss of sensation (d) Abnormal tear film. 5. Vitamin A deficiency disease (VAD) 6. Though the conjunctiva is never sterile except first few hours after birth, organisms present are generally nonpathogenic but opportunistic organisms will have a upper hand if the resistance of the cornea is compromised due to(a) Local causes. Indiscriminate use of antibiotics and steroids, repeated use of local anaesthetic agent, ill fitting contact lens, loss of sensation, raised intraocular tension, corneal edema.

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(b) Systemic. Prolonged use of steroid, resistant organisms AIDS. 7. Cornea subjected to constant inoculation by micro-organism i.e. chronic dacryocystitis, blepharoconjunctivitis. 8. Central corneal ulcers are generally due to exogenous infection. Peripheral ulcers are generally non infective. Pathogenesis of corneal ulcer35 Evolution of infective corneal ulcer has following stages : 1. Invasion by micro-organism. Organism generally get access to cornea following trauma, gonococci meningococci and diphtheria however may invade intact epithelium. In herpes simplex and zoster, organism reach cornea via sensory nerve ending. Organisms also reach cornea by direct continuity of conjunctiva, in case of acute bacterial conjunctivitis. 2. Progressive infiltration. Once micro-organism has reached traumatised epithelium, there is infiltration by polymorphonuclear and lymphocytes that results into a white corneal opacity with edema of epithelium over the opacity, this is followed by necrosis of epithelium and formation of ulcer that stains with fluorescein. If proper anti-microbial drugs are used at this stage, further progress can be averted or minimised resulting into clear cornea. 3. Active ulceration. The infected epithelium is necrosed and sequesteredoff with stromal infiltration. The ulcer deepens with infiltration and edema of the stroma. At this stage anterior uveitis develops due to toxins liberated by the organism which percolate to endothelium and spreads to uvea resulting into pain, photophobia, watering, circumciliary congestion disproportionate to size of the ulcer. If the anterior chamber reaction is severe, hypopyon may develop. The ulcer may spread all around towards the periphery and or it may deepen to reach the Descemets membrane which is tougher than other structures of cornea hence is not destroyed with ease. The Descemets membrane bulges through the defective stroma even with normal IOP and forms a keratocele or Descemetocele. If intraocular pressure rises suddenly or remains raised for considerable time, the Descemets membrane gives way and a perforation of cornea results with resultant loss of AC, softening of eye and plugging of perforation by iris. If the perforation is large, intraocular contents including lens may be expelled through the perforation. Commonest site for perforation is centre of the cornea which is thinnest. If the perforation is small but is not closed by iris, a corneal fistula results. 4. Regression. Healing is initiated by natural defence mechanism of host cornea. This is enhanced by treatment. Healing starts with laying down of fibrocytes in place of normal keratocytes. The fibrocytes are opaque and do not confirm with normal corneal architecture, there may be associated superficial vascularisation. Over all result is formation of a corneal opacity depending upon the depth of the opacity it can be - nebular, macular or leucomatous. If iris is incarcerated, there will be a leucoma adherent. In case of a large perforation incarceration of total iris in the margin of the ulcer causes an anterior staphyloma which bulges even with normal intraocular tension. Failure of regression. Small and superficial ulcers may heal in due course with resultant corneal opacity without treatment. Deep and large ulcers heal with treatment. If

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progression of ulcers is not checked, the ulcer is bound to perforate, resulting intoleucoma adherent, anteriorpolarcataract, complicated cataract, cornealfistula, anterior staphyloma, endophthalmitis, pano-phthalmitis, phthisis. Clinical features of corneal ulcer35, 37, 38. For a corneal ulcer to develop breach in epithelium is a prerequisite except in gonococcus, meningococcus and diphtheria. Breach in epithelium is brought about by trauma. Trauma is generally mechanical, severity of trauma is variable, it may be so trivial that the child may not be able to recollect. In infants trauma by own nails is very common. Depending upon type and virulence of offending organism ulcer may develop within hours to days. Fungal ulcers take weeks to manifest. Pneunococcus, pseudomonas have short incubation period. Sometimes there may be more than one offending organism responsible for corneal ulcer. Symptoms : 1. Lacrimation and foreign body sensation. These are the two early symptoms. Foreign body sensation becomes worse and is converted into dull ache, pneumococcal, pseudomonas, Koch-Weeks bacilli produce severe pain. 2. Redness of eye is due to circumciliary congestion. 3. Most of the bacterial corneal ulcers are associated with mucopurulent conjunctivitis. 4. Vision - Visual status depends upon position of ulcer itself, and area of surrounding infiltration. Central ulcers produce more visual loss while peripheral ulcers may not hamper vision at all or visual loss is minimal. With progression of ulcer, vision diminishes fast. 5. Blepharo-spasm is a prominent feature in children, this is associated with photophobia due to irritation or trigeminal nerve. Spasm of the ciliary body is the foremost cause of pain. Other cause of pain is stimulation of corneal nerve endings. Corneal nerve endings become numb with progress of ulcer. 6. In case of hypothesia of cornea that is found in herpes simplex and neurotropic ulcers patient may not complain of pain. 7. Late increase of pain is due to rise of intraocular pressure. Sudden relief of pain is an ominous symptom, it means perforation. Signs. The ulcer starts as an area of greyish infiltration that is lustureless, there is distortion of windows reflex over the ulcer. The surrounding area is edematous and infiltrated by inflammatory cells. Generally there is single central ulcer, peripheral ulcers may be multiple. The ulcer stains bright green with fluorescein. The edge of the ulcer stains brighter than floor. The surrounding edematous and infiltration takes a yellowish green stain. Biomicroscopy shows anterior chamber reaction with flare and cells. In severe ulcer hypopyon may become evident. Circumcorneal congestion is prominent sign of anterior uveal inflammation that follows deepening of the ulcer and liberation of toxin. Corneal sensation is diminished in viral ulcers, large sloughing ulcers and neurotropic ulcers.

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Staining of corneal lesions. Corneal lesions are stained by vital stains. Two commonly used stains are Fluorescein and Rose Bengal. Purpose of staining is to demonstrate presence of a stainable lesion i.e. abrasion, desquamation, ulceration, epithelial edema, mucous, dead and damaged cells. Fluorescein remains extracellular, it stains tear film also but not the dry spot. It stains epithelial defect, large lesions are visible with ordinary flash light, small and faint lesions are best seen under cobalt blue filter with magnification. It is used either as one percent autoclaved solution or as 1% stain impregnated, pre sterilised, single use, disposable strip. Fluorescein does not cause irritation of cornea or conjunctiva. It is water soluble, hence washable. Other purpose is to know the extent of ulcer, its progress or healing. Fluorescein staining should be performed after testing corneal sensation and before instilling local anaesthetic agent. While using fluorescein its sterility should be guaranteed. Fluorescein is notorious to harbour pseudomonas. Other uses of fluorescein are contact lens fitting, applanation tonometry, demonstration of leaking wound, tear film study. Extra corneal examination uses are fluorescein angiography, evaluation of patency nasolacrimal duct i.e. Jones I and II test. Seidel test to demonstrate leaking surgical wound 20% fluorescein solution has been used with some success in treatment of pediculosis and phthiriasis of lid margin. Rose Bengal. Chemically rose bengal is similar to fluorescein and is water soluble. Dry crystals of both the chemicals are brown in colour. Rose bengal is used to stain mucous, dead and damaged cells. It is used along with fluorescein in many superficial corneal lesion. Rose bengal stains peripheral viral laden cells and fluorescein will stain floor of the ulcer in herpatic ulcer. Rose bengal stains filament of epithelium in filamentary keratopathy while base of the filament stains with fluorescein. It does not share infectious tendency of fluorescein. Only drawback with rose bengal is its sting. Rose bengal causes severe stinging sensation of the conjunctiva. The patient should be informed about this phenomenon which is otherwise harmless. Prior instillation of local anaesthetic abolishes sting of rose bengal, however, xylocaine can cause epithelial changes that may stain with rose bengal. Proparacine hydrochloride 1% aqueous drops is the only recommended local anaesthetic that does not interfere with rose bengal stain. Documentation of corneal lesions. Lesions of cornea are documented by drawing with international colour code. Like colour coded drawing of fundus for retinal lesions. Corneal lesions are drawn in two views. One is frontal and other is vertical section of anterior segment as seen on slit lamp. For finer details an optical section of cornea may be drawn. Management of infective corneal ulcer : Management consists of : (i) Confirmation of diagnosis (iii) Ancillary treatment. Confirmation of diagnosisHistory, clinical examination and stain give definite clue to causative factor. (ii) Specific treatment

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Infective ulcers can be caused bybacteria, fungi and viruses. Bacterial ulcer may be caused by single organism or there may be mixed infection. It is very common for viral and fungal ulcers to be contaminated by bacteria. Reverse is also possible but rare. Each group of organism have specific characteristics. Characteristics of bacterial corneal ulcer : They are : 1. Generally central, even when cornea is infected by bacteria on the periphery due to some ill understood phenomenon ulcer starts in the centre. 2. Most of the bacterial ulcers are associated with mucopurulent conjunctivitis. 3. They develop in short time following injury and spread fast. 4. Bacterial corneal ulcers are likely to develop hypopyon more frequently. Most of the bacteria are capable of producing hypoypon. However pneumococci, pseudomonas, streptococcus and staphylococci are more common causative factors than others. 5. Bacterial ulcers are generally associated with severe pain especially in pneumococcal ulcers. 6. They promptly stain with fluorescein. 7. Sensation over clear cornea is generally normal. 8. Scrapping from fresh and untreated ulcer give positive smear, that can be cultured and sensitivity of the organism to antibiotic can be determined. Characteristics of fungal ulcer37, 40, 41. Like bacterial ulcers, fungal ulcers are also central in nature, they are more indolent. In most of the cases there is history of trauma by organic material. It takes seven to fifteen days for a fungal ulcer to develop following injury. If an aggressive ulcer develops following corneal injury within twenty four hours, the chances are that it is a bacterial ulcer. History of prolonged and indiscriminate use of local and systemic steroid is second most predisposition. Exposure keratitis, anaesthetic cornea, edematous cornea are also likely to develop fungal ulcer. Fungal corneal ulcer has a few typical features42 : 1. It is central, indolent, yellowishwhite ulcer with dull, dry surface that has zone of infiltration all round, there are smaller satellite lesions away from the main ulcer. 2. There is pronounced anterior chamber reaction that results in formation of endothelial plaque and thick almost organised hypopyon. 3. Scrapping from the ulcer show fungus in 10% KOH solution, and stains with Giemsa stain, P.A.S. or Gomori methamine silver stain. 4. Fungi are cultured at room temperature on blood agar and Sabouraud medium. 5. Corneal biopsy may be needed some time. Characteristics of viral ulcer. Almost all viruses that invade conjunctiva also involve cornea and produce various types of superficial keratitis only herpes group of viruses i.e. simplex type I, type II and zoster virus are of clinical significance as far as corneal ulceration is concerned. Primary herpes simplex is disease of infants and children. Recurrent ulcers

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are seen in adults. Most characteristic sign of herpetic ulcer is diminish corneal sensation. The ulcer is unilateral, generally central and dendritic in shape without much redness and pain. Initially the ulcer stains poorly with fluorescein and better with rose bengal. In later stage with disquamation of epithelium fluorescein stain becomes prominent. Herpetic ulcer does not develop hypopyon unless secondarily infected by bacteria. The other symptoms of viral keratitis are foreign body sensation, lacrimation, photophobia, diminished vision, sometimes diminished corneal sensation. Initially there is hardly any circumciliary congestion. The ulcers are small and numerous. They stain with fluorescein. Smaller ulcers may require cobalt blue filter to become visible many viral ulcers are bilateral. Characteristics of sterile corneal ulcer. These ulcers are generally peripheral, mostly bilateral, relatively quiet, without circumcorneal congestion. Corneal infiltrates and epithelial defect are minimal or absent. There is no anterior chamber reaction, no hypopyon, corneal sensation is normal. Vision is diminished if the lesion is central. Other symptoms are mild redness and pain. Etiological factors are generally systemic. Morphological distribution of corneal ulcer. Corneal ulcers can be central or peripheral, both can be superficial, deep, infective or non infective. Central corneal ulcers42, 43. Central corneal ulcers are mostly infective, may be caused by bacteria, fungi and viruses. As they develop in front of pupil, diminished vision is early and marked that becomes worse with extension of ulcer. Even successful treatment leaves residual central opacity that causes diminished vision. Diminished vision is not only due to obstruction of light to pass through pupil but also due to accompanied irregular astigmatism. Diminished vision following central corneal ulcer puts child at an added disadvantage of intractable amblyopia and squint. Opacity developing in infancy may cause nystagmus. Peripheral (marginal) corneal ulcers35, 44. Marginal corneal ulcers are as common as central ulcers. They are less dramatic than central ulcers and have a more benign course, hence draw less and late attention than central ulcers. However incidence of peripheral ulcer is less in children than adults. Marginal ulcers in children do not perforate unless secondary bacterial infection is superimposed. Causes of their being superficial and non perforating is better nutrition on the periphery of cornea and enhanced thickness of corneal periphery. Causes of peripheral corneal ulcers are : 1. Extension of conjunctival disease in the cornea i.e. trachoma, phlycten, rosacea, staphylococcal blepharoconjunctivitis. 2. Allergicphlycten 3. Toxin 4. Metabolic Marginal ulcers according to their position can be (1) sectorial, (2) circumferential - may form a ring ulcer. Circumferential ulcers generally result due to coalescence of more than one peripheral ulcer. Circumferential ulcers have tendency to develop heavy superficial vascularisation.

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Identification of specific causative organism is done by : (a) Corneal scrapping and examination of stained slide. (b) Culture of organism (c) Sensitivity of organism to antibiotic. Identification of other ocular diseases that may influence treatment of corneal ulcer i.e. lagophthalmos, lid deformity, loss of sensation, chronic dacryocystitis, raised intraocular tension. Systemic diseases. Malnutrition, vitamin A deficiency, measles. Diabetes is generally not a problem in children but should be kept in mind if it is associated with symptoms of juvenile diabetes. Specific treatment : 1. Anti microbial drugs. Local antibiotics. Once it has been established that the ulcer is infective and its causative organism identified, specific anti microbial drops in suitable strength and frequency will eliminate the organism and enhance healing. However it is not always practical to get smear, culture and sensitivity done, result of sensitivity to antibiotic for bacterial infection takes minimum 24 hours to 48 hours. Fungal culture takes as much as a fortnight to be informative. Culture of virus is done only in few selected and advanced centres. Most practical method is to decide an antibiotic according to clinical feature. Generally a long acting antibiotic that is effective against both gram positive and negative organisms is used as aqueous drops. Frequency depends upon severity of the condition. Commonly used antibiotic in infective corneal ulcer are Erythromycin 0.5%, Chloramphenicol 03.%-0.5%, Gentamycin 0.3%, Tobramycin 0.3%, Ciprofloxicin 0.3%, Framycetin 0.5%, Ofloxacin 0.3%, sparfloxacin 0.3%. Newer antibiotics are being added frequently replacing older antibiotics. These drugs are instilled as drops, one drop each hour in the lower fornix. More than one drop at a time is not retained in the conjunctiva and flows out without additional therapeutic advantage. Sometimes a combination of antibiotics that have synergistic effect is used. A common combination are Neomycin 0.35% + Polymyxine B. 10000 u and Bacitracin 500u. Another popular combination is combination of neomycin with chloramphenicol. In adults and older children subconjunctival injection may be given. Whenever it is not possible to administer subconjunctival injection, fortified antibiotic solutions are instilled instead of ordinary drops. Commonly used fortified solutions used for corneal ulcer are : Gentamycin 14 mg/ml, Cephazoline 33 mg/ml, Tobracin 15 mg/ml Ophthalmic ointment is used at bed time either with or without bandage. Advantage of ophthalmic ointments are : They act as lubricants, they have slow absorption, slow drainage from the conjunctival sac. They need not be applied frequently. They prevent sticking of the lids.

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Subconjunctival injection. Commonly used subconjunctival injections are : Penicillin1 million unit/ml Gentamycin20 mg-40 mg/ml Cephazoline100 mg-125 mg /ml Tobramycin20 mg-40 mg/ml Subconjunctival injection of antibiotic gives a high but short lived peak of antibiotic concentration at the site of injection. Hence they are either given along with local instillation or at bed time with a pad and bandage. Generally subconjunctival injections are given once a day for five to seven days. Systemic antibiotics do not have any added advantage over local antibiotics. (For anti fungal and anti-viral drugs used in corneal ulcer see under specific ulcers) 2. Removal of discharge. Bacterial and fungal corneal ulcers are generally associated with mucopurulent or purulent discharge. Efforts should be made to remove the discharge from the conjunctival sac and lid margin with sterile wet swab. Irrigation of the conjunctival sac has no added advantage. On the contrary it washes away tear lysozyme which has anti microbial property. 3. Cycloplegia. Cycloplegics should be used in all corneal ulcers with anterior chamber reaction. Cycloplegics have no action on cornea, they are not anti-inflammatory, analgesics or anti microbial. Main function of cycloplegic in corneal ulcer is to prevent and treat associated anterior uveitis that is main cause of pain and photophobia. Cycloplegics relive spasm of ciliary body, thus reduce pain indirectly. As all cycloplegics are strong mydriatic also, they dilate the pupil and break posterior synechea. As such pure mydriatics have no role in management of corneal ulcer. Commonly used cycloplegics in corneal ulcer are : (i) Atropine sulphate. Atropine is most potent cycloplegic and prompt mydriatic. Its action last for more than ten days following single instillation, however, in inflamed eye more than one instillation is required. Atropine should be used with caution in children. One drop of one percent atropine contain more than therapeutic dose. It is better to avoid atropine as drop in children. In no case should atropine be used more than twice in twenty four hours. If necessary short acting cycloplegic may be added to atropine to enhance effect of atropine. Prolonged use of atropine may cause allergic dermatitis. While prescribing atropine to children the parents should be appraised of the side effect of atropine i.e. flushed face, fever, and dryness of mouth which are not serious and can be managed by systemic antipyretic and plenty of fluids orally. Dilated pupil makes the child uncomfortable due to glare. (ii) Home atropine hydrobromide 2%. It is less powerful cycloplegic than atropine. Its action does not last more than 48 hours. Its side effects are less than atropine so it can be used frequently. However in children who develop allergy to atropine are also allergic to home atropine. It is generally used in cases of superficial small ulcers.

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(iii) Cyclopentolate hydrochloride. This is used as 1% drop, has action faster than home atropine but action lasts for twenty four hours to forty eight hours, can be used frequently. Tropicamide 1% is very short acting unless used repeatedly has hardly any beneficial effect in corneal ulcer. 4. Pad and bandage. Pad and bandage prevent movement of lid over the ulcer this stabilises the epithelial growth during healing. It does not abolish movement of eye, it only reduces movement of lid that helps epithelisation of ulcer. However pad and bandage raise the temperature of the conjunctiva which encourages proliferation of micro organism, hence, application of pad is contra indicated in presence of copious discharge. 5. Bandage contact lenses are good substitute for usual pad and bandage. 6. Destruction of ulcer : (i) Cauterisation of ulcer. If ulcer fails to respond to usual treatment within a week, debridement of the ulcer is done under local anaesthesia and edges are cauterised by pure carbolic with caution. Other drugs used to cauterise the ulcer are 7% alcoholic solution of iodine, trichlor acetic acid. (ii) Physical methods of destruction of ulcers are Heat cautery, cryo and laser. 7. In case of non healing corneal ulcer, penetrating keratoplasty is indicated. Management of hypopyon corneal ulcer. Hypopyon corneal ulcer denotes presence of severe anterior uveitis due to corneal ulcer. All bacteria are capable of producing hypopyon. Some organisms characteristically produce severe hypopyon, others cause minimal hypopyon. Hypopyon is acculumation of sterile pus in anterior chamber. It contains mostly polymorphonuclear leucocytes, occasional mononeuclear cells, macro-phages and fibrin. The pus is due to out pouring of exudates from iris and ciliary body. In bacterial corneal ulcer, the pus remains sterile so long as Descemets membrane is intact. The pus gets infected following perforation of ulcer. It is a prominent feature in central corneal ulcers and penetrating injury. The pus in bacterial corneal ulcer is worsened by absorption of toxin liberated by causative organism. Hypopyon in fungal ulcer is generally infected because fungus can penetrate intact Descemets membrane. Bacteria produce hypopyon earlier than fungi. Virus by themselves do not produce hypopyon. Presence of pus in viral keratitis denotes presence of secondary bacterial infection. Development of pus is preceded by severe anterior chamber reaction. The aqueous becomes plasmoid and fibrinous that entangles leucocytes. Presence of pus cells in the anterior chamber gives it characteristic white colour, however the pus is tinged green in pseudomonas infection, and is yellowish in fungus, it may be occasionally tinted with blood. As pus is heavier than aqueous, hypopyon settles down at the most dependent part of anterior chamber, it assumes a horizontal level at the upper part. Though hypopyon is thicker than aqueous yet it freely moves unless it gets organised as is seen in fungal ulcer. Amount of hypopyon is directly proportionate to virulence of organism. Some organisms produce early

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and severe hypopyon. Organisms that produce severe hypopyon arepneumococci, pseudomonas, streptococci pyogenes, gonococcus. Extent of hypopyon is measured by its height from bottom of anterior chamber in millimeters or roughly as trace, mild, moderate, half of AC or full of AC. Best position of the eye to look for early hypopyon is to make the patient sit up for few minutes and allow hypopyon to settle down and ask the patient to look down, light is thrown from the above in front of the iris. In recumbent position the pus spreads all over the iris or may pass through the pupil, making it difficult to be visualised. Hypopyon in fungal corneal ulcer takes more time to develop, it is associated with severe anterior chamber reaction. The pus is generally thick, does not move freely with change in position of the eye, is fluffy, yellowish in colour and infected with fungal element. The ulcer has multiple satellite lesion and an endothelial plaque under the original ulcer. Management of hypopyon corneal ulcer is basically the same as any infective ulcer with severe uveitis. Hypopyon corneal ulcer should be treated as an emergency. It is better to hospitalise the patient and start vigorous antibiotic drop instillation. Subconjunctival injection in children is difficult to administer. Hence frequent local instillation of suitable fortified antibiotic is most important part of treatment. In bacterial ulcers hypopyon may disappear with in twenty four hours only to appear after frequency of instillation is reduced unless infection has been eradicated. Bandage lenses and occuserts are good alternative when available and possible. In children sub-conjunctival injection and cauterisation should be done under short acting general anaesthesia or good sedation. All cases of hypopyon should receive atropine sulphate 1% as an ointment. Atropinisation should be done under supervision of trained person. Atropine drops are better avoided because an illiterate attendant may instil atropine frequently in place of antibiotic without anticipating serious complication. Outline of treatment of hypopyonCorneal ulcer should be as follows : 1. Hospitalise the child. 2. Affective and safe atropinisation. 3. Frequent instillation of single or combination of two broad spectrum synergestic antibiotic drops that will eradicate both gram positive and gram negative cocci and bacilli. 4. Corneal scrapping : (i) Examine part of scrapped material after staining with Grams stain, Giemsa stain, 10% KOH to identify bacteria and fungus and find out if the characteristics present are in favour of bacteria or fungi. (ii) Inoculate part of the material to grow bacteria or fungi. (iii) Identify organism and test for sensitivity to antibiotic. 5. Once best possible antibiotic has been identified, replace initial antibiotic with sensitive antibiotic as fortified drops. If possible give sub-conjunctival injection of the same antibiotic.

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6. Cauterise under short acting general anaesthesia. 7. Keep intraocular pressure low by oral carbonic anhydrase, inhibitor and beta blockers. 8. Pad and bandage at night may help in early healing of ulcer. 9. Systemic antibiotics do not have any direct influence on hypopyon, they may be added in consultation with pediatrician for systemic infection when present. Non Healing Corneal Ulcer Aim of treatment of corneal ulcer is to 1. Eliminate infection, 2. Encourage healing, 3. Restore transparency of cornea and 4. Reduce complication. Bacterial ulcers have better prognosis than fungal ulcers. Viral ulcers especially herpes simplex is notorious for recurrence. Generally a bacterial ulcer of moderate size takes ten to twenty days to be free from organism and heal with proper treatment. However sometimes the ulcer does not heal as expected. Causes of non healing corneal ulcers are : 1. Failure to identify the causative organism. 2. Failure to eliminate causative organism. 3. Incomplete management of predisposing factors. 4. Iatrogenic 1. Failure to identify causative organism. It is very common to miss fungus infection and keep on treating patient as bacterial ulcer. Vice versa is also common. It is a widely prevalent belief that ulcers developing following injury are always fungal in nature. Ulcer developing within twenty four to forty eight hours after injury are most probably bacterial. Similarly failure to notice secondary bacterial infection in a herpes simplex ulcer is one of the causes of failure. 2. Failure to eliminate causative organism. This stems from inability to identify the organism, use of antibiotic to which the organism is not sensitive or has developed resistance, use of anti-microbial in sub-clinical strength and frequency is one of the common factors. This may result in use of ordinary antibiotic drop instead of fortified drop or not administering sub conjunctival injection when indicated. Hesitation to cauterise a fulmination ulcer too has similar result 3. Incomplete management of predisposing factorsspecially presence of chronic dacryocystitis, lid of deformity, loss of sensation, dry eye, vitamin A deficiency, rise of intraocular tension, vascularisation of cornea, missed foreign bodies in cornea, upper lid or upper tarsal plate are some of the factors that prevent healing of corneal ulcer. 4. Iatrogenic causes of non healing corneal ulcers are (i) Indiscriminate use of steroid.

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(ii) Introduction of exogenous infection by way of contaminated drops i.e. fluorescein drops, local anaesthetic agent. (iii) Over enthusiastic chemical cauterisation. Management of non healing corneal ulcer is basically treatment of simple corneal ulcer with suitable antibiotic, cycloplegic and correction of above mentioned factors. In case of penicillinase producing organism i.e. staphylococci, methicillin should be used. Pseudomonas corneal ulcers generally require two antibiotic drops one aminoglycocide and other cefazolin. Common ancillary management consists of cauterisation of ulcer edge, sub-conjunctival antibiotic injection, bandage lens, local beta blockers or systemic acetazolamide, cauterisation of superficial vascularisation. Under no condition should a subconjunctival injection or cauterisation be tried in a struggling child. The child should be properly sedated for such procedures. Predisposing factors should be eliminated i.e. epilate the misdirected lash, perform a tarsorrhaphy in lagophthalmos and neurotropic keratitis. All malnourished children should get full dose of vitamin A as per WHO recommendation. Corneal ulcer threatening to perforate : There are two groups of ulcers that perforate : 1. Very fast developing ulcer like pneumococcal, and pseudomonas ulcers and keratomalacia. 2. Slow developing ulcers - Fungal ulcer, stromal involvement in HSV, Herpes simplex keratitis. Perforation of an ulcer is a catastrophe that must be avoided. Once an ulcer perforates, the prognosis changes to worst. It may lead to endophthalmitis or even panophthalmitis. If perforation seals, it leads to adherent leucoma or corneal staphyloma. Management of impending perforation are : 1. Anticipation of perforation. A fast developing ulcer, especially in depth with large hypopyon, severe anterior chamber reaction and pain is most likely to perforate. Intensive instillation of two synergistic antibiotic with subconjunctival injection may stop the progress. 2. Prevent (i) A sudden rise of intraocular pressure by preventing cough, sneezing, vomiting, and constipation. Protect the eye from direct trauma by metal or plastic shield placed properly. Otherwise the edge of shield itself can traumatise cornea. (ii) Fast build up of intraocular tension is managed by local beta blocker two times a day or acetazolamide 15 mg/kg body weight in divided doses for short period. Pilocarpine is contra indicated. (iii) Paracentesis. Paracentesis is a guarded and planned perforation on the corneal periphery. It should be done under general anaesthesia in children. It lowers intraocular tension instantaneously. Advantage of paracentesis are : 1. Pus drains out through the wound. 2. Pus and aqueous so drained can be examined for microbes.

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3. If necessary, intra cameral antibiotics can be injected. 4. Following paracentesis, there is out pouring of fresh aqueous that has healing property. 4. Strengthening the thinned cornea by cynoacrylate substance when the ulcer is small. 5. Keratoplasty. In selected cases with rim of healthy cornea all round, penetrating keratoplasty may be tried. One of the advantages of penetrating keratoplasty is that it removes the infected part of the cornea, which harbours micro-organism, hypopyon is drained through the wound, AC can be washed with balanced salt solution antibiotics can be instilled directly in AC. Chances of dens central corneal opacity are reduced. Complications of corneal ulcer. Aim of treatment of corneal ulcer is to eliminate infection and give a clear cornea. Corneal opacity is almost unavoidable even with best treatment unless it involves the epithelium only. The size and depth of opacity varies with extent of ulcer and promptness of treatment. Ulcers anterior to the Bowmans membrane give faintest opacity unless they are vascularised. Full thickness extension with or without iris incarceration give most dense opacities. Corneal opacities in children not only diminish vision but lead to intractable amblyopia and squint. Opacity developing in infancy lead to nystagmus. Another complication of corneal ulcer that is often overlooked is gradually developing glaucoma specially following perforation. Presence of raised intraocular tension produces early disc changes in children that later on hampers with visual gain even after successful keratoplasty. Commonest sequel of corneal ulcer is loss of transparency. Loss of transparency is commonly known as corneal opacity. The opacity is generally white in colour. According to colour of opacity which in turn depends upon its depth and incarceration of uvea opacities have been divided into following grades : 1. Nebula 2. Macula 3. Leucoma and 4. Leucoma adherent Other sequel and complications are : 1. Ectasia 2. Perforation Corneal opacity is produced due to replacement of keratocytes by fibrocytes, infiltration and edema. Infiltration and edema generally pass off with healing, may take weeks to months to disappear. Corneal opacities following destruction of Bowmans membrane are always permanent. Nebular opacities in corneal ulcer develop due to infiltrating edema and cicatrisation of epithelial defect. They are generally situated in front of the Bowmans membrane. Small nebular opacities may be missed on oblique illumination but are best visualised on slit lamp biomicroscopy. On retinoscopy they cause irregular reflex. Though they do not cause much visual loss diminished vision due to nebulae are sufficient to cause amblyopia.

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Macular opacities are denser than nebulae and visualised by ordinary flashlight. They are due to destruction of Bowmans membrane and superficial stroma. They are permanent in nature. Leucomass are densest opacities that are visible in diffuse light and when large, the parents may notice their presence. They are generally due to lesions extending from epithelium to Descemets membrane. They may cover whole of the cornea, may have facets in them, are often vascularised. They cause maximum loss of vision, amblyopia, and squint in children. As corneal ulcer perforates following events take place : 1. Aqueous drains out causing softening of the eyeball, flattening of cornea, collapse of AC and miosis. 2. The iris is drawn towards the perforation and gets attached to it. 3. The iris plugs the wound over which fibrosis occurs and 4. A leucomatous opacity develops. This is called leucoma adherence. Eye with leucoma adherence has following features : 1. A corneal opacity in which either the iris is adherent or there is an evidence that iris was once incarcerated, which is betrayed by presence of iris pigment in the centre of the opacity. Thus any corneal opacity with evidence of iris incarcerated is adherent leucoma. 2. Key hole pattern of pupil with narrow end towards the opacity. 3. Absence of AC at the site of iris incarceration. 4. Tent like appearance of the iris with apex at the opacity. Ectatic Changes in Cornea Following Perforation 1. Staphyloma. Perforation is very common catastrophic complication of corneal ulcer. They are most common in large central, untreated or poorly managed ulcers that extend deeper than Descemets membrane. Small corneal perforation is plugged by a knuckle of iris to form an adherent leucoma. A large perforation is not immediately sealed. As has been discussed earlier, a perforation leads to draining of aqueous, flattening of cornea, softening of eyeball, miosis and shift of iris lens diaphragm forwards. This forwarded shifted iris blocks the perforation and the constricted pupil is closed by exudate that acts as a scaffolding over which fibrocytes from stroma and epithelium develop to form a white scar or pseudocornea of uneven thickness which is very thin and plastered with iris on posterior surface at places the whole of the scar gets ectetic and bulges forward. This ectatic cicatrix with iris incarcerated is called corneal or anterior staphyloma. It can engulf whole of cornea and called total anterior staphyloma or may be partial. The anterior staphyloma may be vascularised, may have facets. There is always associated secondary glaucoma, which produces loss of vision which is very common. A large staphyloma may bulge through inter palpebral fissure. Generally the staphylomatous eyes are divergent and have nystagmus. Commonly they are not painful, uncontrolled glaucoma or break down of scar make them painful. In unfortunate children anterior staphyloma can be bilateral. Small pox used to be commonest

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cause of large bilateral anterior staphyloma before small pox was eradicated. In under developed countries, keratomalacia still causes bilateral anterior staphyloma. Blast injury especially due to cracker burst is another cause of bilateral, total or partial staphyloma in children. 2. Descemetocele. Bulging of thin cornea is a common feature of poorly managed corneal ulcer. If there is incarceration of iris in ectatic cornea, it results in corneal staphyloma that has been discussed earlier. If there is no perforation, iris does not get incarcerated in the wound and the Descemets membrane bulges through the floor of the ulcer. It is called Descemetocele or keratocele. In the infiltrative stage of corneal ulcer, the ulcer can spread either towards the periphery or may infiltrate deep. In unfortunate conditions both may happen simultaneously. Deeper infiltration is rapid through Bowmans membrane and stroma. The Descemets membrane offers real resistance and can withstand the infiltrative processes for sometime. As the ulcer reaches the Descemets membrane, the membrane bulges forward as a translucent blister through the floor of ulcer under normal intraocular pressure. If intraocular pressure is high, not only will the surrounding Descemets membrane bulge but also surrounding thin cornea. Corneal ectasia may follow thinning of cornea without Descemets membrane bulging. In simple ectatic cicatrix of cornea, there is no incarceration of iris. The cicatrised ectasia is a weak spot in cornea hence is liable to perforate either following trauma or gradual rise of tension. Perforation of ectatic cornea is not sealed so perfectly as a simple small perforation seals. It may seal and anterior chamber may reform only to be lost following opening up of the wound resulting into a corneal fistula. Corneal fistula45. Corneal fistula is a small track that allows anterior chamber to open externally permanently. In corneal ulcer it is generally seen in the centre of the cornea, is not wider than one to two millimetre. It is lined by corneal epithelium that grows down the wall of the fistula. The epithelium may spread over the corneal endothelium and iris. Traumatic corneal fistula may develop anywhere over the cornea. Formation of corneal fistula may occur in following conditions 1. Perforation of a ectatic cicatrix that is not plugged by iris. 2. Perforation of centre of cornea (a) When pupil is widely dilated due to prolonged atropinisation and kept dilated following perforation. This prevents the iris from blocking the hole. (b) A pre-existing wide iridectomy that prevents iris from reaching the perforation. (c) Coloboma of iris. In very rare instances aniridia. The fistula gives all the features of a corneal perforation i.e. flat cornea, absent AC and soft eye. The only difference is that the pupil is not constricted, vision is very poor. Occasionally the fistula may be blocked by debris resulting in reformation of the AC. This allows intraocular pressure to build up, which does not last long as rise of intraocular pressure even within normal range forces the fistula to open and the AC is again lost. The corneal fistula has a typical appearances of a translucent pit surrounded by a zone of infiltration. On careful examination, it will be noted that aqueous leaks through the pit or may be forced to leak on pressing the globe.

188 Complication of corneal fistula are :

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1. Endophthalmitis, panophthalmitis due to introduction of infection. 2. Phthisis due to persistent hypotony. 3. Epithelial down growth. Summary of perforation of corneal ulcer : 1. Small perforation with mobile iris. Adherent leucoma. 2. Moderate perforation with miotic pupil and mobile iriscorneal staphyloma. 3. Large perforation not closed by iris - Extrusion of intraocular content, lens and vitreous resulting in phthisis bulbae. 4. Small central perforation not plugged by iris - Corneal fistula. 5. Small central perforation sealed without incarceration of irisanterior polar cataract. Corneal opacity45, 46. Most of disorders of cornea result in loss of corneal transparency that may be short lived or permanent. Latter are due to fibroblastic reaction of various layers of cornea during healing and cicatrisation. Corneal opacities vary in number from single to multiple, their size also range from pinpoint to covering whole of the cornea. They are of different shape, and depth. According to depth of opacity, they are graded as nebula, macula, leucoma and leucoma adherent. Corneal opacities may be localised to cornea only or may be associated with disorders of other structures like uvea, lens and sclera. Secondary glaucoma is very common associated disorder with large corneal opacities. Difficulty in measurement of tesion over a scarred cornea is difficult, hence glaucoma is invariably missed. Presence of circumciliary congestion with opacity denotes presence of active uveitis or secondary glaucoma. Corneal opacities can be unilateral or bilateral. One eye may show denser or more numerous opacity than the other. Causes of cornal opacity : 1. Commonest cause of corneal opacity is replacement of transparent keratocytes by opaque fibrocytes as seen following keratitis, corneal ulcer and trauma. Bowmans membrane and Descemets membrane once destroyed do not regain transparency. 2. Edema of cornea. It could be epithelial, stromal, endothelial or combined. 3. Inflammatory infiltration. 4. Vascularisation. Superficial or deep vascularisation always leave scar of obliterated vessels. 5. Degeneration of cornea. Band keratopathy is more common in children than in adults. 6. Dystropies of cornea. Many of corneal dystrophies manifest in childhood. 7. Deposits on cornea. Tattoo, corneal mucus plaque.

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8. Encroachment of conjunctiva on cornea : (i) Epithelial surface. Pterygium, pseudo pterygium, conjunctival hood flap, dermoid. (ii) Endothelial surface. Epithelial down growth. 9. Blood staining of cornea 10. Pigment deposit. Generally not seen in childhood. 11. Folds in Descemets membrane 12. Rupture and splitting of Descemets membrane. Symptoms. Symptoms of corneal opacities depend upon their position in relation to pupil. Central opacities even of fainter density may produce pronounced diminished vision that may lead to intractable amblyopia, nystagmus, squint. Peripheral corneal opacities do not produce diminished vision. However a peripheral opacity may be associated with irregular astigmatism. Other symptom is cosmetic blemish, the parents become aware of the opacity and the child may be brought solely for it, not realising that the opacity is also causing diminished vision. Simple corneal opacities are painless and the eye is white. Signs. Only sign of simple corneal opacity is loss of corneal transparency without any evidence of inflammation. Corneal opacities do not stain with fluorescein. However facets in corneal opacity may retain dye without staining. In leucoma adherent, iris is either incarcerated in the opacity or there is evidence that the iris was entrapped in the past in the form of iris pigment in the white opacity. Other signs of leucoma adherence are irregular pupil, key hole pupil, irregular AC, may have anterior polar cataract. Occasionally vessels may grow into an opacity. Corneal sensation is generally absent or very poor over the opacities. Management : 1. Best management is prevention. This is better said than done. Small Pox used to be one of the commonest causes of ugly corneal opacities before the disease was eradicated. Children not immunised against small pox were always at risk of developing corneal complications and children who were immunised never developed small pox related corneal opacities. However vitamin A deficiency remains a formidable cause of preventable corneal opacity in children in underdeveloped countries. Compulsory oral administration should form a part of immunisation in children. Moreover adequate vitamin A also reduces severity of measles and malaria which indirectly lead to malnutrition. All children with trauma should receive prompt local antibiotic drop. Early and proper management of corneal ulcer reduces severity of opacity. In fact aim of treatment of corneal ulcer should be to give a transparent cornea as far as possible. 2. Definitive treatment for corneal opacity is keratoplasty, Lanellar or penetrating depending upon depth of the opacity. Superficial small opacities can be ablated by laser. In failed penetrating keratoplasty or thick opacity where keratoplasty is not possible keratoprosthesis may be tried. 3. Optical iridectomy does not seem to do any good to the patient. Similarly tattoo replaces a white opacity with a dark deposit and is aesthetically not acceptable.

190 4. Painted contact lenses mask the corneal opacity.

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5. Local instillation of ethyl morphine (Dionin), and iodides do not have any beneficial effect even on faint corneal opacity. Bilateral corneal opacities pose more problem than unilateral opacities because they may virtually make a child grossly visually challenged. Common causes of bilateral corneal opacities in children as per age of onset are : 1. Congenital 2. Neonatal Intrauterine infection Buphthalmos Limbal dermoid Dysgenesis of anterior chamber Iridocorneal dysgenesis Ophthalmia neonatorum (Bilateral central leucoma, anterior polar cataract, nystagmus) IatrogenicFolk medicines 3. Infancy 4. Childhood 5. Adolescence Bacterial corneal ulcer Buphthalmos, Iatrogenic Above causes, xerophthalmia cracker injury Interstitial keratitis Phlyctenular keratitis Above Arcus juvenalis Band keratopathy Corneal dystrophies Mucopolysaccharidosis Specific Types of Corneal Ulcer Corneal ulcers according to its position in relation to pupil is divided into two types : 1. Central and 2. Peripheral ulcers. They not only differ from each other in clinical presentation but also in management. Central corneal ulcer. These ulcers are more common and vision threatening which is preventable if etiology can be pinpointed in time and suitable treatment started and carried on long enough. They are : 1. Infectious in nature following epithelial damage. The initial breach in epithelium may be peripheral but the ulcer starts in front of the pupil. Severe bacterial forms are always associated with variable degree of hypopyon, which is sterile. However fungal hypopyon may contain fungal elements. Mixed infection is common. Combinations may be : (i) Multiple bacterial infection of all possible combinations.

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(ii) Bacterial infection over viral infection, reverse is rare. (iii) Bacterial superimposition on fungal ulcer. (iv) Bacterial ulcer may be primary. (v) Corneal pathogen or opportunistic organism which remain dorment without ulcer formation may become active due to loss of resistance of the cornea. (vi) The corneal resistance is compromised due to immunosuppression by way of use of steroids, local anaesthetic, cytotoxic drug, broad spectrum antibiotic, and loss of corneal sensation. Chief causes of central corneal ulcers are : 1. Bacterial. Pneumococcas, pseudomonas, streptococcus, Klebsiellapneumonae, staphyloccous. 2. Fungal 3. Viral 4. Keratoacanthamoeba 5. Keratomalacia. Peripheral corneal ulcers. These ulcers are less vision threatening. They rarely perforate, most of them are painful. They are generally due to acute or chronic bacterial conjunctivitis but they themselves are not infectious. No organism is grown from corneal scrapping from these ulcers. They are due to sensitization to bacterial products. In these cases antibodies from limbal vessels react with corneal antigen to produce keratitis. They develop few millimetres inside the limbus as infiltrates that in duecourse ulcerate. Marginal ulcers are generally self limiting but recurrent. They respond well to local steroids. However peripheral herpes simplex and pseudomonas ulcer should get special attention. Important Bacterial Central Corneal Ulcers 1. Pneumococcal ulcer47, 48. Pneumococcus is by far the commonest cause of central corneal ulcer in all ages. Many strains of pneumococci cause corneal ulcer. Commonest strain is type IV but infection by type III cause more complication than any other form. It has a short incubation period (24-48 hours) following injury, which may be very mild. The ulcer to begin with is central, disc shaped, grey white in colour. The ulcer has a tendency to spread both towards the periphery and penetrate deep. This tendency to creep has been called Acute serpiginous ulcer. The ulcer creeps in one direction and heal in the area away from the advancing edge. The ulcer has tendency to change direction. Pneunococcus is a common inhabitant of normal conjunctiva. The cornea gets infected only following epithelial damage. Chronically inflamed lacrimal sac acts as a reservoir of organism. Pneumococcal corneal ulcer is associated with moderate to severe mucopurulent conjunctivitis. Uveal reaction is always severe resulting into moderate to big hypopyon that is produced due to toxin of the organism, hence the hypopyon is sterile. The hypopyon does not get organised and remains fluid. Management49, 50. Scrapping from the active edge promptly grow Gram positive, diplococci. Occasionally the organism can be seen in short chain. Generally there is a capsule surrounding each pair. The organism should be cultured and its sensitivity to various antibiotics

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graded. The pneumococci is known to become resistant to antibiotics specially penicillin. Generally systemic antibiotics are not recommended unless patient has systemic involvement. Systemic antibiotic should be administered in full dose in consultation with physician. All cases of pneumococcal keratitis threatening to perforate should also get systemic antibiotic. Usually available commercial ophthalmic drops are not sufficient for management of suppurative keratitis. Fortified aqueous drops should be administered frequently. Frequency may be as often as every five minutes for six times followed by every ten minutes for another six times. This is followed by hourly instillation during day and two hourly at night. Commonly used fortified drops are : Penicillin G100,000 1u/cc Bacitracin10,000 IU/cc Cefazolin50 mg/c.c. Other antibiotics used are erythromycin, vancomycin. Ciprofloxacin is less effective. If topical antibiotic drops are not effective or there is a moderate hypopyon concurrent sub-conjunctival injection of either 0.5 to 1.0 milli units of penicillin or 50mg to 100mg of cefazolin BD may evert further extension. As there is always associated severe uveitis, use of atropine is one of the most important steps in suppurative keratitis. Atropine is administered as one percent ointment two times a day. It is better to avoid atropine as drops in children because there is always danger of atropine drop being instilled in dose more than therapeutically permitted. The eye should be bandaged at night only if there is no discharge. Pseudomonas keratitis46, 48, 51. Pseudomonas aeruginosa causes one of the most serious central corneal ulcers and is one of the most common causes of perforating corneal ulcer. The organism is an opportunistic, gram negative, mobile, obligate aerobe that produces a proteolytic enzyme which acts on stroma and is responsible for early perforation. About 5% of population pass pseudomonas in stool and yet be asymptomatic. Common mode of infection are - instillation of contaminated fluorescein or anaesthetic drops in hospitals or by contaminated contact lenses specially extended wear. It has also been cultured from contact lens solutions. Pseudomonas has a short incubation period. Otherwise it may follow mild trauma. Pseudomonas corneal ulcer starts as a small central area of infiltration following trivial injury and spreads in all directions. Occasionally it may start in the periphery. The ulcer spreads rapidly, is always associated with severe pain. Hypopyon develops fast and increases with spread of ulcer. Hypopyon has greenish blue hue due to pigment produced by the organism. Pseudomonas ulcer may spread so fast as to cause large central perforation within twenty four hours. Ulcer is always associated with mucopurulent conjunctivitis that may have satellite conjunctival abscess. Peripheral ulcers may spread into the sclera as tunnel lesions. All fast spreading corneal ulcers in contact lens users should be considered to be due to pseudomonas unless proved otherwise by culture. Management of pseudomonas ulcer does not differ from any other central ulcer. When pseudomonas is suspected but its presence has not been confirmed a combination of fortified cepfazolin and tobramycin should be used as drops very frequently. Once

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pseudomonas has been confirmed cefzolin may be discontinued and any of the following alone or in combination with tobramycin is continued i.e. gentamycin, any of fluroquinols. In no condition should steroid be used. Systemic acetazolamide or local betablockers lower the risk of perforation. Streptococcus pyogenes. Central corneal ulcer caused by streptococcus does not have any particular characteristic except that the surrounding cornea is infiltrated and edematous. It produces a large hypopyon. The organism is sensitive to many antibiotics which should be prescribed as fortified drops to be instilled very frequently. Antibiotics commonly used are erythromycin, cefazolin, gentamycin, chloramphenicol, tetracyclin. However fortified penicillin 100,000 unit/cc be tried as first drug of choice. It can be given as subconjunctival injection also in a dose of 0.5 to 1.0 million unit/cc. Patient should get usual dose of atropine with all precautions. Staphylococcus corneal ulcer. Staphylococcus cause both central as well as peripheral ulcer. Latter is commonly seen in associated with staphylococcal blepharoconjunctivitis. Incidence of staphylococcal central ulcer has increased due to prolonged and indiscriminate use of steroid. The ulcer is indolent, is generally associated with moderate hypopyon. The surrounding cornea is infiltrated. The ulcer is generally superficial hence perforation is relatively less common. The organism is sensitive to many drugs i.e. penicillin, erythromycin, chloramphenicol cefazolin, gentamycin. Central Viral Corneal Ulcers Almost all viral keratitis are central. Most important virus that produce central corneal ulcer belong to herpes group of viruses i.e. : Human herpes virus Varicella zoster virus Cyto megalo virus Epstein Barr virus All the above viruses are D.N.A. viruses, besides ocular involvement all of them have systemic manifestation of various degrees which could be life threatening in children. Herpes simplex keratitis52, 53, 54, 61. Human herpes virus or herpes simplex is most important virus causing keratitis, incidence of which is gradually increasing. It is a self limiting disease in immunocompetent cornea. Most of the time it is recurrent. Management of recurrent disease is more difficult than primary disease. In spite of apparent cure of single incidence, next attack can not be predicted or prevented, which may be delayed for one to two years. Incidence of herpes simplex keratitis increases with age, no age or sex is immune to herpes simplex infection. It is generally unilateral. 1. Herpes simplex infection can be passed as an intrauterine infection, resulting in multiple congenital malformation including ocular. 2. A neonate may acquire the disease from infected birth canal. On the basis of antigenicity, herpes simplex has been classified as type I and type II. Each having various strains, some of the strains are more virulent than others.

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Traditionally type I herpes simplex is said to involve the body above the waste and type two is said to effect below waste (genital herpes). However, infection above waste by type two and infection below waste by type one is also known. Both types infect skin, mucousmembrane, mucocutaneous junction and cornea. Herpes keratitis is ocular counterpart of labial herpes and dendritic ulcers resembles fever blister. Though typical lesions of herpes simplex render their diagnosis easy specially in epithelial lesions. Chronic lesions may simulate many other lesions. In chronic lesion superimposed secondary infection change the clinical presentation grossly. Clinically there are two types of herpes simplex infection : 1. Primary and 2. Recurrent. 1. Primary infection is the infection that develops in person who has not been exposed to HS virus earlier. Unfortunately primary infection is almost universal and may be symptomless. 90% of adult population is sero positive for HSV. Children suffer from HSV can be divided into three groups Primary infection by type I does not occur in children under six months of age due to maternal immunity passed to a child unless infected during birth from infected maternal birth canal. HS infection may prove to be fatal in neonates. Commonest mode of presentation in new born is milder form of non-gonococcal ophthalmia neonatorum. 2. The next age group that develop primary HSV infection is after six months of age when maternal antibodies disappear. This age group spans between six months to 10 years children in this group get infected by virus shed from lesions on the lips, lids even genital of adults. 3. Third age group comprise of sexually active adults and adolescent. Primary herpes simplex infection of the eye and the adnexa consist of - Lid vescicles, blepharitis, lymphadenopathy, follicularconjunctivitis, pseudo membranous conjunctivitis, keratitis, micro dendrile, dendrile, and mild kerato uveitis. Neonates have severe iritis. Primary lesions are generally self limiting. Recurrent herpes simplex infection of cornea is seen in persons who have been exposed to primary infection. The lesions are mostly central but can be peripheral as well that are more difficult to manage. Following primary infection, the virus gains access to the central nervous system and remains dormant in trigeminal or spinal ganglion only to be reactivated by some catalyst (trigger mechanism) like stress, ultra violet ray, fever, menses, systemic infection, general anaesthesia, food allergens etc., resulting into lesions of skin, lips, mouth and followed by corneal lesion. However some of the corneal lesions are independent of trigger mechanism. In recurrent lesions active viruses travel down the nerves to infect the target organ that develop classical lesions. Corneal lesions can be : 1. Epithelial, and 2. Stromal.

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1. Epithelial lesions are54 1. Dendritic ulcer or micro dendrite 2. Geographic (amaeboid) 3. Metaherpetic keratitis Dendritic ulcer is typical of herpes simplex infection of cornea. Initially it may be present as smaller micro dendritic ulcer in the epithelium, in the centre of cornea, less commonly on the periphery. The dendritic ulcer has an irregular linear shape from which branches sprout and progress while the older part of the ulcer heals by epithelial slide. New defects develop on the progressive branch that may form terminal bud. The bed of the ulcer stains with fluorescein and the edges and terminal buds stain with rose bengal. Corneal sensation is dull which masks the pain sensation, preventing the patient from an early consultation. However only symptom may be lacrimation and foreign body sensation, some conjunctival congestion. In the initial stage, there may be no circumcorneal congestion, the vision may be good to begin with. Other epithelial lesion that may be caused by HSV are blotchy epithelial keratitis, satellite epithelial keratitis, and rarely filamentary keratitis. They may precede to formation of dendritic ulcer or may co-exist with it to be transformed into typical dendritic ulcer. Subepithelial opacities may develop under the original epithelial defect in the form of ghost image. The shape of which is identical to original dendritic ulcer but larger. The ghost image may last as long as one year. They may become worse by use of anti viral drugs specially idoxuridine. As ninety five percent of HSV keratitis are unilateral, all cases of unilateral watering of short duration, without any definite history of trauma, or foreign body in the cornea or upper tarsal conjunctiva should be stained and examined under cobalt blue light for ulcer. Staining should be preceded by examination of corneal anaesthesia. Other causes of formation of dendritic ulcers are : Herpes zoster, corneal abrasion, Thygesons keratitis and contact lens. Gepgraphic (amoeboid) ulcer. If dendritic ulcer does not heal it spreads in all direction to form a geographic or amaeboid ulcer that stains with fluorescein. Commonest cause of geographic ulcer is fast growth of virus on the periphery due to compromised immunity of cornea especially following indiscriminate use of local steroid or cytotoxic drugs. Vision is hampered as the epithelial defect is large and covers the pupillary area. Metaherpatic keratitis. These are non viral component of dendritic and geographic ulcer due to problem of healing at the level of damaged basement membrane. They are caused by corneal denervation, they are trophic, indolent in nature. These ulcers last for long time with very little inflammatory reaction. The lesions heal with continuously worn therapeutic contact lens, prolonged pad and bandage, use of lubricants. Stromal involvement. Stromal involvement is commonest complication of recurrent HSV keratitis. It is generally preceded by recurrent attack of dendritic or geographical ulcer. It is commonest cause of diminished vision that is more pronounced than in epithelial keratitis because healing of stromal keratitis is always associated with stromal scarring. Uveal

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involvement is universal in stromal involvement. There may be vascularisation, stromal necrosis, and perforation. Various types of stromal involvement are : Disciform keratitis Interstitial keratitis Immune ring Limbal vasculitis Necrotising keratitis Stromal involvement can be : 1. Necrotising and 2. Non-necrotising Necrotising keratitis is cause by active replication of virus in the lesion. It is an active stromal infiltrative process that may develop with or without epithelial defect. Stroma becomes necrotic with cheesy appearance. It is associated with anterior uveitis with keratic precipitates, just under the infiltration. There is no hypopyon. Presence of hypopyon is associated with secondary infection. Vascularisation and scarring are common. Necrosis of stroma may lead to perforation. Disciform keratitis is an example of non necrotising deep keratitis. It is an antigen antibody reaction, generally associated with local or systemic immuno suppression that is produced following use of strong chemical to cauterise the ulcer, use of strong steroid, or iodoxuridine. There is a localised area of stromal edema generally in the centre of cornea. The epithelium over the stromal thickening is elevated. There may be an endothelial defect just under the stromal thickening. Large white KP are very common just beneath the edematous area which is circular as the term disciform suggests. There may be folds in Descemets membrane. It is always associated with anterior uveitis. IOP may be raised. The condition is self limiting, it may take few weeks to resolve. Even when it resolves, it leaves a ring shaped opacity that represent the periphery of the lesion. Complications of herpes simplex keratitis are : Anterior uveitis, secondary glaucoma, complicated cataract, deep vascularisation of cornea, scleritis, corneal scarring, trophic ulcer, perforation. Management of HSV infection of cornea : Herpes simplex keratitis is said to be a self limiting disease, it takes weeks to month for both epithelial and stromal disease to heal, recurrence keratitis makes prognosis poor. Secondary bacterial and fungal infection are potentially dangerous. Condition becomes worse with indiscriminate use of steroid. Strong chemical cautery, loss of sensation are important causes of perforation. HSV infection does not impart local or systemic immunity. There is no known method of immunisation. Aim of treatment is : 1. Eliminate virus by anti viral drugs - local or systemic. 2. Management of associated uveitis and glaucoma. 3. Judicious use of weak steroid for stromal involvement.

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4. Protect the hyposthetic cornea from physical, chemical and microbial onslaught. 5. Increase local and systemic immunity. 6. Keratoplasty. Antiviral agents used in HSV keratitis52, 53, 54, 55, 56 1. Idoxuridine. This is a thymidine analog. It is used as 0.1% drops or 0.5% ointment. It is effective in epithelial lesions both primary and recurrent. It does not penetrate the stroma and is insoluble in water, hence it has little use in stromal involvement. It is used in dendritic and geographical ulcers. Its main drawbacks areinsolubility in water, local epithelial toxicity and development of resistance, hypersensitivity, development of superficial punctate keratitis. Idoxuridine is used as 0.1% drops every hourly by day and two hourly by night for five days with 0.5% ointments three times a day. It may have to be used for two to three weeks. It is better to discontinue the drug after three weeks due to its corneal toxicity. Trifluridine (Trifluorothymidine) is also an analog to thymidine. It inhibits DNA both in virus as well as host. It is used as 1% clear solution. It is used against both primary and recurrent HSV virus. It is more effective than idoxuridine and vidarabine. It is less toxic than the two. It is effective in stromal keratitis as well. Cross resistance with other antiviral drugs do not occur. It is used as nine times a day for ten days. Vidarabine. This is an adenosine analog, a DNA inhibitor of both virus and host cell. DNA inhibition in host cell is less marked than in virus. It is available as 3% ophthalmic ointment to be used five times a day for five days. It is effective against epithelial keratitis. Treatment is not extended beyond three weeks for fear of corneal toxicity, which is however less than met with idoxuridine. Is is as effective as idoxuridine. Sometimes it is effective when idoxuridine is ineffective. It is less effective than trifluridine. Acyclovir. This is an analog of guanosine. It stops viral replication. It is highly effective against type I and type II HSV. It is used against primary, and recurrent keratitis both epithelial and stromal. It is available as 3% ophthalmic ointment, oral tablet and IV infusion. It is less toxic than idoxuridine and vidarabine. Ointment is used five times a day for five to seven days in adults. Famciclovir. It is a pro drug with action similar to aclycovir. Usual dose in herpes simplex is 250 mg orally three times a day for seven days. It may be used as prophylaxis as 125 mg two times a day for one year. Valaciclovir. Usual oral adult dose is 1 gm two times a day. It can also be used for prophylaxis in half the strength for one year. It is mostly used in treatment of herpes zoster. All oral antiviral drugs should be used in consultation with pediatrician when used under 12 years of age. 2. Other non antiviral drugs used in management of HSV keratitis : (i) Cycloplegic. Cycloplegics play an important role in management of viral keratitis. They have no action of virus multiplication or healing. They are essential in treating associated anterior uveitis which is inevitable in stromal keratitis. They also abolish pain of cyclospasm associated with epithelial defect.

198 Commonly used cycloplegics are :

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1. Atropine sulphate 1% ointment (drops should be avoided in children to eliminate over dosage). 2. Home atropine hydrobromide 5% drops two to three times a day. 3. Cyclopentolate HCL. One percent drops three times a day. (ii) Cortico steroid. These are double edged medicament that are indicated in stromal involvement but contra indicated in epithelial ulcer. They should be used under umbrella of antiviral drug specially those that have stromal penetration, weakest possible solution in least but effective frequency should be used for shortest period. (iii) Antibiotics. Broad spectrum antibiotic drops, ointments are used to prevent bacterial secondary infection especially in anaesthetic cornea, patients under steroid and abnormal tear film. The antibiotics do not have any antiviral effect. (iv) Tear film substitute. Corneal scarring and corneal anaesthesia are associated with abnormality of tear film. A lubrication cum tear film substitute give relief to the patient. (v) Imuno potentiating drugs. Levamesol, BCG, vitamin A, cematidine, antioxidants have been tried without constant result. 3. Non medical methods. Tissue adhesive cyanoacrylate is used in cases of stromal keratitis threatening to perforate or in a small perforation. 4. Debridement. Debridement is single most effective physical treatment for dendritic and geographical ulcer. It is still treatment of choice where antiviral drugs are not available. In the past debridement was always followed by chemical cautery either by iodine or carbolic acid. Chemical cautery has not been found to have any added advantage over simple debridement. On the contrary it is known to produce stromal damage, produce keratitis metaherpatica and predispose perforation. Keratoplasty. Penetrating keratoplasty is the ultimate treatment for corneal scar. However it should be tried only when inflammation has been under control for at least six months, otherwise the graft itself can get infected by HSV. Treatment of individual lesions : 1. Blepharitis and blepharo conjunctivitis. They are often missed as bacterial infection. Treatment consists of local use of idoxuridine 0.1% drop four to five times a day and 0.5% ointment at bed time for four to five days or vidarabine 3% ointment three times a day for four to five days. These drugs when used for more than five days are likely to produce corneal toxicity that may be confused as epithelial defect. Trifluridine and acyclovir should be used when above drugs do not seem to be effective. 2. Primary keratitis. Diagnosis of this condition requires high degree of suspicion on the part of treating physician. Once diagnosis has been established treatment is similar to treatment of dendritic ulcer. 3. Neonatal HSV57 infection is a life threatening disease, it requires management by neonatologist along with local treatment by antiviral drops. 4. Recurrent HSV keratitis. There is no effective method of preventing recurrence because primary infection does not provide any immunity. Latent period between primary

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attack and subsequent recurrence is variable even gaps between attacks of two episodes of recurrence is not definite. Number of recurrence is also uncertain. It is claimed that if trigger mechanism like fever, stress, food allergen, ultra violet exposure can be avoided, recurrence is averted. One of recommendation is to put patients above twelve years on oral 200 mg acyclovir every alternate day few months after patient has received a full course of oral acyclovir i.e. 200 to 400 mg five times a day for five to ten days. 5. Dendritic and Geographic ulcer. (i) First antiviral drug of choice should be trifluridine locally whenever available or acyclovir locally supplemented by oral acyclovir. Idoxuridine and vidarabine are effective against epithelial lesions only idoxuridine is most kerato toxic. (ii) Debridement. Simple debridement is an effective treatment for epithelial keratitis, debridement may be followed by local instillation of trifluridine or acyclovir. Chemical cautery by 7% alchoholic solution of iodine and carbolic was standard treatment before advent of antiviral drugs. Now they have been given up. However when antiviral drugs are not available, they still hold the fort when done carefully. (iii) Cycloplegic. Cycloplegics are neither antiviral nor anti inflammatory analgesic. Their main function is to treat associated anterior uveitis. Whenever there is associated lacrimation and photophobia, instillation of cycloplegic drugs relieve discomfort. 6. Stromal involvement. Stromal involvement both necrotising or non necrotising are most difficult to manage. Idoxuridine and vidarabine have no role in stromal involvement. Trifluridne and acyclovir are effective in stromal herpes. Stromal HSV infection is always associated with uveitis making use of cycloplegic almost mandatory. Use of steroid is one of the trickiest decisions. Whenever steroids are deemed necessary it should be under cover of effective antiviral drug, well supervised and with weakest strength, least frequently for shortest possible period. 7. Metaherpatic keratitis. Besides antiviral drops, lubricants, pad and bandage, bandage contact lens help to epithelise the effected cornea. Varicella Zoster Viral Keratitis Herpes zoster ophthalmicus54. This is an acute multi systemic disease with chronic sequel caused by a DNA virus that produces two distinct type of systemic disease i.e. vericella (chicken pox) and herpes zoster (shingles). The herpes zoster virus is similar to herpes simplex virus in structure but different antigenically and clinically. Both viruses have primary infection followed by a period of latency and a recurrence. In herpes zoster there is a single recurrence that may have a prolonged course while more than one recurrences are common in HSV infection. In both conditions the virus lies dormant in posterior root ganglion only to be activated after an indefinite latent period when the virus travels down the nerve to produce mucocutaneous and ocular lesion. Herpes zoster virus involves single dermatome HSV has irregular distribution. Herpes zoster infection does not cross the midline. Herpes zoster gives life long immunity.

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Herpes zoster virus infection differs from vericella in following ways. Vericella is very common, self limiting disease with predominant skin involvement and less pronounced ocular involvement in children. It is rare in adults, while herpes zoster infection is more common in adults, however children are not immune. Ocular involvement in herpes zoster is known as herpes zoster opthalmicus that has severe vision threatening ocular involvement with neurological defect, which may involve other cranial nerves as well. In rare cases it may produce contra lateral hemiplegia. In severe viremia encephalitis is possible. Involvement of cranial nerves other than ophthalmic is mostly immunological rather than infectious in nature. (See Chapter 22 as well) Though involvement of first division of trigeminal is commonest manifestation. The second and third divisions may also be involved. Involvement of maxillary division produces vesicular eruption in the lower lid, which is otherwise rare. Any of the branches of ophthalmic division may be involved but common involvements are supra orbital, supra trochlear, nasociliary and lacrimal. Involvement of nasociliary nerve that supply the lateral part of the skin of nose and tip of the nose is invariably associated with corneal involvement (Hutchinsons sign). However there may be corneal involvement without involvement of nasociliary nerve, reverse is also possible. There may be localised involvement of any of the branches of ophthalmic division without ocular involvement. Only ocular involvement without cutaneous manifestation has not been observed. Ocular involvement in herpes zoster ophthalmicus can be divided under following heads : 1. Cutaneous, 4. Uveal, 2. Kerato conjunctival, 5. Others. 3. Scleral,

1. Cutaneous. Skin of the lids, forehead and scalp are most commonly involved. There is generally mild pain in the distribution of dermatome where vescicles develop in due course. Initially the lesions are maculo papular which become pustular within few days, that rupture to form crust. The crusts gradually fall off leaving depressed marks similar to chicken pox. The crusts are loaded with live viruses, which are infective to others and can cause chicken pox in children. The papulo macular stage is associated with intense edema that may spill over the mid line but not the rashes. Edema of the lids may obliterate the interpalpebral fissure due to mechanical pseudo ptosis. As the scabs fall of the edema subsides with punched out scar marks that can either be hyperpigmented or hypopigmented. Vescicles on the lid margin may heal by producing notch in the lid margin. It can produce madarosis, trichiasis, ectropion or entropion. There may be ptosis due to various factors i.e. : 1. Pseudo ptosis is generally due to inflammatory edema. 2. True ptosis is due to involvement of upper division of third nerve. Some degree of loss of sensation is always present, may last for years associated with post herpetic neuralgia. Generally orbiculosis is not involved in herpes zoster ophthalmicus. Commonest cause of paralysis of orbicularis is Ramsay Hunt syndrome where vescicles develops in the external auditory canal. However rarely orbicularis palsy may develop independent of Ramsay Hunt

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syndrome. Involvement of nasociliary nerve produces development of vescicle on the lateral side of nose and tip of nose. This is invariably accompanied with corneal involvement. 2. Kerato conjunctival involvement : 1. Conjunctival involvement become obvious within first few days as mucopurulent conjunctivitis. This is always associated with vescicles of the lid. Mucopurulent conjunctivitis becomes worse with obliteration of I.P.A. Rarely there may be vescicles on the conjunctiva itself. Conjunctival sensation is either lost or diminished. 2. Corneal involvement. Corneal involvement is one of the most serious complications of herpes zoster ophthalmicus, effect of which could last for years and may cause permanent damage. (i) Hypoethesia of cornea. First and foremost sign is diminished corneal sensation that may range between reversible hypoethesia to irreversible anaesthesia that on long run may lead to trophic ulcer. (ii) Keratitis. Earliest infective manifestation is punctate lesions in epithelium. They are generally multiple, mostly on the periphery, stain with rose bengal and contain live viruses, which may respond to local antiviral drugs. They appear within forty-eight hours of onset of skin lesion. They are transient in nature, however, some of them may coalesce to form lesions similar to dendrites. Dendrites of herpes zoster are smaller than dendrites of herpes simplex. They stain with rose bengal, they are transient, are seen between fourth to sixth day. By tenth day there is infiltration of superficial stroma in the form of nummular keratitis that may last for months and cause vascularisation of cornea. Disciform keratitis is late feature, by this time the rashes have crusted and may have fallen i.e. three weeks after appearance of rashes. Disciform keratitis are associated with thickening of stroma and anterior uveitis. It may be vascularised and cause lipid degeneration. Loss of sensation leads to formation of trophic ulcer. Due to loss of sensation patient does not complain of pain that may lead to secondary bacterial infection resulting in perforation. Mucous plaque formation is an unique feature of herpes zoster ophthalmicus that develops between third and sixth month in the form of deposit of mucus on swollen epithelium. The plaque can be removed with ease. It is associated with anterior uveitis and stromal infiltration. 3. Scleral. Episcleritis and scleritis are very common in herpes zoster ophthalmicus in first week following onset of rashes, may be missed due to swollen lid and inflamed conjunctiva. Scleritis may cause sclerokeratitis and sclero uveitis 4. Uveitis. Uveitis in herpes zoster ophthalmicus is limited to anterior uvea causing acute iridocyclitis within fortnight. Iridocyclitis in herpes zoster is a non granulomatous in nature. To begin with iridocyclitis is mild with fine KP and few cells and flare. In severe cases hypopyon may develop due to ischemic necrosis of iris which in turn leads to iris atrophy and formation of holes in iris.

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5. A common complication of herpes zoster uveitis is secondary glaucoma that may be overshadowed by keratitis and missed altogether unless sought specifically. It is caused due to associated trabeculitis, the swollon trabecular meshwork and inflammatory debris may cause obstruction of the drainage. Neurological involvement in herpes zoster ophthalmicus : (i) Commonest neurological sign of herpes zoster ophthalmicus is loss of sensation. On the distribution of the dermatome involved leading to anaesthesia of cornea, conjunctiva and skin resulting in delayed healing and may lead to trophic ulcer. Anaesthesia masks pain of corneal and uveal involvement. (ii) The next bothersome symptom is post herpetic neuralgia that may persist for months to years. Fortunately it is rare and milder in children. It is very severe in old age. Exact cause of post herpetic neuralgia in absence of sensation is not well understood. It is called anaesthesia dolorosa. (iii) Other neurological involvements areoptic neuritis, cranial nerve palsy and contra lateral hemiplegia that may take few months to develop. Encephalitis is a rare manifestation of herpes zoster ophthalmicus. Management of herpes zoster ophthalmicus58. Management of herpes zoster ophthalmicus in children does not differ from management in adults. Children have advantage of less frequent post herpetic neuralgia which is very mild. 1. Skin lesions are best treated with oral acyclovir in dose of 50-60 mg/kg/day divided in five doses. Children can get as much as 400 mg acyclovir five times a day for 7 days. This reduces viremia and enhances healing, resulting in scar formation. Acyclovir does not have much effect on corneal lesions or postherpeticneuralgia but is known to diminish uveal involvement. Acyclovir should be started within 48 hours of onset of rashes. Local skin ointment of acyclovir is applied in the vesicular stage. In the vesicular stage combination of calamine with talcum powder or starch should be avoided as it masks the changes in the vescicles, cause secondary infection and deep scar formation. Combination of antibiotic and steroid is recommended at the crusting stage. Systemic corticosteroid under the umbrella of systemic antiviral drugs reduces lid edema, and deep scar. Steroids also helps in reducing post herpetic neuralgia. They help to treat stromal involvement and uveitis. As pain is not a very prominent feature of herpes zoster ophthalmicus in children, mild analgesic given orally is sufficient. Active immunisation against vericella has been recommended to prevent herpes zoster but its efficacy has not been proved beyond doubt. 2. Conjunctival lesions are treated by local broad spectrum antibiotics instilled in the conjunctiva. Role of local antiviral drugs to combat conjunctivitis is not established. 3. Corneal involvement in herpes zoster is a clinical riddle. Local antiviral drugs are of doubtful value. Local steroids must be given under strict medical supervision. Steroid in weakest possible strength is recommended. The patient must be put on steroid for months. Loss of corneal sensation is a perpetual problem that requires frequent instillation of broad spectrum antibiotic, long acting cycloplegic. If

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necessary, tarsoraphy must be performed. Loss of corneal sensation is a contra indication for keratoplasty and contact lens. 4. Optic neuritis, cranial nerve palsy are self limiting. Systemic steroid helps in shortening the course. Adeno virus keratitis in children62 Almost all adeno viruses that cause conjunctivitis in children involve cornea to some degree. Corneal involvement is seen five to seven days after onset of conjunctivitis which has various degree of follicular reaction and sometimes pseudo membrane formation. Adeno viral keratitis is associated with systemic symptom and pre auricular lymphadenopathy. Corneal involvement in adeno viral infection is less common and less troublesome than seen in herpes simplex. Corneal involvement are less marked in children than in adults. They are generally epithelial in nature and present as fine superficial keratitis that stain brightly with fluorescein. Sub-epithelial involvement is less frequent, transient and leave no scar. Fungal keratitis42, 63, 64 Fungal infection of cornea is less frequent than bacterial or viral keratitis. It is generally unilateral and central following history of injury. The injury is caused by foreign body of vegetable origin. It is more common in hot and humid climate than in cold and dry climate. Besides trauma, other conditions that predispose fungal keratitis are situations where corneal defence mechanism is compromised i.e. loss of sensation, stromal herpetic keratitis, prolonged local or systemic steroid therapy, paralysis of orbicularis, dry eye, vitamin A deficiency. Fungal keratitis is a suppurative, indolent corneal ulcer with severe anterior chamber reaction, hypopyon and conjunctival congestion. It takes more than ten to fifteen days for a fungal corneal ulcer to develop following trauma by vegetable material. There is an initial corneal abrasion that either heals spontaneously or is treated as bacterial corneal ulcer generally with antibiotic drops. Use of steroid in such situation makes the condition worse. Severity of infection depends upon size of inoculum and immuno competence of the cornea. Fungi that cause corneal ulcer can be divided into two main groups i.e. 1. Filamentous and 2. Non filamentous. Common fillamentous fungi that cause corneal ulcer are aspergillus, fusarium, cephalosporium. (See Chapter 22 also) Following trauma with vegetable matter, the initial breach in epithelium heals as simple corneal ulcer. After a gap of ten to fifteen days acute fungal keratitis becomes manifest causing lacrimation, photophobia, blurring of vision and pain. The appearance is characteristic; first a sub epithelial gray white lesion develops at the site of trauma. The epithelium is raised over this and a dirty white, raised dry rough lesion with raised margin develops. Sub epithelial and stromal feathery extension may develop. At the end of the feathery extension multiple satellite lesions may develop which vary in number, shape and size. Their appearance is similar to original central ulcer. The satellite ulcer may join to form a ring called Wessely ring. There may be a epithelial plaque formation. The conjunctiva is severely inflamed. There is intense anterior chamber reaction with thick yellowish white hypopyon and severe anterior uveitis. The ulcer may extend in to surrounding sclera.

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It is not always possible to differentiate non filamentous fungal ulcer from filamentous ulcer. Unlike bacterial ulcers there are no characteristic features that can help clinically to differentiate various filamentous ulcers. Management and complication of all fungal corneal ulcers is almost the same. If a fungal ulcer is not treated in time, it invariably perforates and the eye is lost. Even if the ulcer does not perforate, it leaves a dense central opacity that compromises vision to great extend. Other common complications are secondary glaucoma, complicated cataract, and phthisis. Diagnosis : Diagnosis of fungal keratitis depends on : 1. History 3. Its clinical course and 2. Appearance of ulcer 4. Laboratory finding

History of injury by organic matter specially of vegetable origin about a fort night ago with development of ulcer should alert the physician for possible fungal ulcer that have typical features described above and not responding to usual antibiotic and cycloplegic drops. Laboratory finding consists of : 1. Examination of corneal scrapping : 1. In KOH solution65 2. Staining Grams stain Giemsa stain Gomori methamine silver stain Lactophenol cotton blue66 Caleoflour white67 Culture68 Sabourauds medium Blood agar Brain heart infusion 1. 10% KOH solution is used most frequently to visualise septed fungal hyphae directly under microscope. It is a reliable test. 2. Gram stain is useful to visualise candida, it fails to stain filamentous fungi. 3. Giemsa stain is more useful to define filamentous fungus. 4. Gomori stain provide better fungal cytology. Culture is done at room temperature, it may take as long as fifteen to twenty days to show positive result. Management of fungal keratitis : Management of fungal corneal ulcer is one of the most frustrating forms of treatment due to : 1. Failure to diagnose. 2. Non availability of broad spectrum antifungal antibiotic. 3. Frequent serious complications.

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Antifungal drugs69 : Three groups of antifungal drops are available. They are : 1. Polyenes, 2. Imidazoles, 3. Pyrimidines 1. Polyenes. Amphotericin B, nystatin, natamycin 2. Imidazoles. Clotrimazole, miconazole, ketoconazole, econazole 3. Pyrimidine. Flucytocine In all cases of suspected fungal keratitis, initial treatment is instillation of natamycin 5% drop every hourly by day. If stain confirms presence of yeast (candida) nystatin 50,000 IU, flucytosine 1% and amphotericin B 0.1% to 0.2% should be given every hourly. In case of confirmed filamentous fungal ulcer, miconazole, ketaconazole or clotrimazole may be given hourly. Antifungal treatment should be continued for at least ten days before any improvement occurs. All cases should get 1% atropine sulphate as ointment two times a day. In children atropine should be administered under supervision to avoid serious systemic complication. Local betablockers and oral acetazolamide keeps IOP low and lowers incidence of perforation. Local broad spectrum antibiotic drops are given to prevent secondary bacterial infection. Cortico steroids have no role in management of fungal keratitis Surgical treatment. Good debridement if necessary under general anaesthesia helps to remove infected material. Debridement may be followed by chemical cautery. Too enthusiastic cauterisation may enhance perforation hence it should be done carefully under magnification. Lamellar keratoplasty fails to eliminate the infection. Penetrating keratoplasty shows better results. However the donor cornea itself may get infected in course of time. Peripheral keratitis70 Peripheral keratitis may result due to many local and systemic condition both infective as well as non infective. Non infective causes outnumber infective causes. They are generally bilateral, in spite of a benign course they are more painful than infective keratitis. They are caused by allergy, or auto immune disorders. Peripheral ulcers by themselves do not perforate. Perforation is due to secondary bacterial invasion. The keratitis may be self limiting. Most of them respond to local steroid. Anterior chamber reaction is either absent or minimal. Out of many marginal keratitis only phlyctenulosis of cornea is commonly seen in older children. Some times marginal (catarrhal) ulcer may be seen in older children who may also be effected by autoimmune diseases like rheumatoid arthritis. Phlyctenulosis of cornea. Phlyctenular kerato conjunctivitis is very common in developing countries. It may be limited to conjunctiva only, may involve limbus effecting both cornea and conjunctiva or may primarily develop in the cornea.

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Other causes of peripheral keratitis are - Vernal kerato conjunctivitis and rheumatoid arthtitis, Moorens ulcer, which is an important marginal keratitis is not seen in children. Parenchymatous (Interstitial) Keratitis in Children71, 72, 73, 74. Interstitial keratitis is a chronic disease of cornea with acute onset and protracted course. It can be diffuse or central. In children interstitial keratitis is generally diffuse and bilateral. The other eye getting involved few days after the first. It is due to an antigen antibody reaction to spirochete trapenoma pallidum, tuberculosis and leprosy. Other causes are - Lymphogranuloma venereum, herpes simplex, malaria, onchocerciasis. In children commonest type of interstitial keratitis is due to syphilis in the form of congenital syphilis or hereditary syphilis. The child is infected in utro between first and second trimester, interstitial keratitis manifesting between 5 to 15 years of age. Primarily interstitial keratitis is a disease of stroma. The epithelium and endothelium getting involved later. In late stages all the layers of cornea get effected. Anterior uveitis is early and prominent feature. Uveal involvement is so pronounced that it is thought that basic pathology starts in the uvea and cornea gets involved secondarily. Exact pathology of interstitial keratitis is not well understood. Trapenoma has been isolated from syphilitic foetus and new born without evidence of interstitial keratitis. Conversely trapenoma has not been demonstrated in cornea in interstitial keratitis. The disease is self limiting, it responds well to steroids but not to pure anti syphiletic treatment. Interstitial keratitis has a long latent period of five to ten years following intrauterine infection. The clinical picture has been divided into : 1. Progressive ; 2. Florid and ; 3. Regressive stages. 1. Progressive stage lasts for two to three weeks following clinical symptom of lacrimation, photophobia, blepharospasm. On examination there is well developed circumcorneal congestion and haziness of cornea that starts from periphery extending towards centre followed by sprouting of new vessels in the stroma. This is the time when uveitis develops mostly in the form of acute iridocyclitis. There may be anterior choroiditis as well. Changes in iris are masked by corneal haze. There may be involvement of retina and choroid that result in to salt pepper appearance. 2. Florid stageProgressive stage is followed by florid stage that lasts for two to three months. This is the stage when the symptoms are in peak. The cornea develops heavy deep vascularisation that gives a red hue to the cornea. Vascularisation spread from all sides. It is expected that vessels in cornea should have bright red colour, due to corneal haze the redness is toned down to a peculiar pink colour known as Salmon red colour. Causes of haziness are due to changes in all the layers of cornea i.e. from epithelium to endothelium with keratic precipitates. These changes when added result into a hazy cornea (ground glass appearance). Other change seen in cornea on biomicroscopy are; bedewing of epithelium, cellular infiltration round the newly formed vessels in front of the Descemets membrane, which is wrinkled. The Bowmans membrane is wavy while deep vascularisation is

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developing in the stroma. Superficial vascularisation may be superimposed in the epithelium on the periphery resulting into a crecentic pile of blood vessels known as epaulet. 3. Regressive stageAfter four to five weeks, stage of regression starts which lasts for two to three years. Process of regression starts as the advancing vessel meet in the centre. Once the cornea is fully vascularised, inflammation suddenly subsides with clearing of the cornea, improvement in vision, reduction of lacrimation, pain and photophobia. Corneal clearing starts from periphery and slowly regains its transparency leaving only obliterated vessels as white streaks that are visible on slit lamp for years called ghost vessels. Besides ghost vessels late sequele are band keratopathy and overall thinning of cornea. Systemic involvement in congenital syphilis : 1. Dental changesHutchinsons teeth The permanent upper central incisors are peg shaped reduced in length and breadth. They have a notch on the cutting edge. 2. Changes in earpermanent deafness. Interstitialkeratitis, Hutchinsons teeth and deafness constitute Hutchinsons triade. 3. Foreheadshows frontal eminence. 4. NoseSaddle shaped depression. 5. Rhagades at angle of mouth. 6. Other changes areRetarded physical and mental growth, anterior bowing of shin, Cluttons joint, cardio vascular disorders. Differential diagnosis consists ofAll causes of hazy cornea i.e. buphthalmos, trachoma and congenital corneal haze. Diagnosis. Diagnosis is clinically simple, if possibility of interstitial keratitis is kept in mind especially in a child borne to known syphilitic mother. Otherwise stigmata of congenital syphilis i.e. saddle nose, frontal bossing, Hutchinsons teeth, deafness with severely vascularised cornea leads to diagnosis. Laboratory investigations72 include 1. Positive serological test in mother and child. 2. Reactive CSF-VDRL. 3. Elevated cell or protein in CSF without any other cause. Treatment72. Though systemic anti syphilitic treatment does not influence the ocular condition it is mandatory to treat the child with appropriate dose of penicillin to prevent future cardiovascular and maningovascular complication. Interstitial keratitis is self limiting disease which starts improving within two to three months. The condition is very troublesome to the child who has intense lacrimation, photophobia and diminished vision. Local steroids reduce this period of ocular morbidity to few weeks. Cortico steroids are instilled at a rate of one drop every hour during day for first two days then gradually reduced in frequency over months. Once acute phase has subsided subconjunctival depot steroid can be given at an interval of 15 days. To treat associated uveitis that is always

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present and is a dominant feature cycloplegic drugs are used in acute phase, atropine sulphate is used as one percent drop two times a day under supervision along with local steroids. Once acute phase has subsided atropine can be replaced by any of the following short action cycloplegic i.e. 2% home atropine hydrobromide, 1% cyclopentolate hydrochloride or 1% tropicamide. Only sympathomimetic drug like phenyl pherine has no role in management of uveitis. It only produces immediate blanching of conjunctival vessels giving a false impression of white eye. Children on prolonged local steroids should be closely monitored for rise of tension, steroid induced cataract and secondary infection. Acanthamoebae keratitis38, 75, 76 Acanthamoebae keratitis was unknown three decades earlier because the organism, which is found normally in human throat and pharynx was thought to be non-infective as far as eyes were concerned. However, it remains a relatively rare disease, incidence of which is increasing. There are eight species of acanthamoebae that cause corneal ulcer. The organism is found in two forms i.e. trophozoite and cyst. Besides throat and pharynx the organism is commonly found in fresh water, soil, home made contact lens solutions, all types of contact lenses. Keratitis produced by acanthamoebae is indolent, non-suppurative, resistant to antibiotic, antiviral and antifungal drugs. It is difficult to culture. It thrives on gram negative bacteria and cynobacteria at 2535C temperature. Predisposing factors include : 1. Use of any type of contact lens 2. Use of non standard contact lens solutions specially home made. 3. Swimming in a contaminated pool. 4. Trauma by vegetable matter. It is being reported more commonly among non contact lens users following trauma. Corneal lesions may be as mild as epidemic kerato conjunctivitis or as severe as Descemtocele or perforation. The condition can be put in following three stages : Stage I. This initial stage mimic herpes simplex or bacterial keratitis and is confused as such. The changes consist of one or combination of many punctate lesions, pseudo dendrite and epithelial defect. Symptoms at this stage are foreign body sensation, redness and photophobia. Stage II. This consists of anterior stromal ring, disciform keratitis, formation of double ring, limbitis and scleritis. Stage III. Corneal abscess, severe disciform keratitis, Desmetocele, iritis, scleritis, hypopyon and sometimes hyphaema. All the stages are very painful. Pain is out of proportionate to the size of corneal involvement. Diagnosis77, 78, 79. Diagnosis is generally missed. A high index of suspicion in all painful ulcers developing in contact lens users or following trauma that do not respond to usual corneal ulcer treatment generally leads to correct diagnosis. However diagnosis after four weeks of onset is always fraught with failure.

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All suspicious cases of kerato acanthamebae should undergo examination of corneal scrapping to exclude bacterial, viral and fungal ulcer. Unfortunately herpes simplex keratitis is very common with acanthameba keratitis. The scrapped material should be stained with hematoxylin and eosin, Grams stain, Giemsa stain, lactophenol cotton blue and celluflour white. The material can be examined after immuno fluorescent stain. Cysts have a typical polygonal double walled appearance. It is difficult to culture the organism, however, the material may be inoculated on blood agar, non nutrient agar79a or chocolate agar that has been overlaid with killed E.Coli. Management. Treatment of kerato acanthameba is difficult. It requires constant and prolonged treatment with specific drug, result of which may be frustrating. Management consists of76 : 1. Prevention by proper contact lens storage and cleaning. 2. Instillation of anti acanthamebial drugs. 3. Usual treatment of corneal ulcer, uveitis and secondary glaucoma. 4. Penetrating keratoplasty. Anti acanthamebic drugs are : 1. BiguanidesPolymeric-polyhexa methylene biguanide 0.02% solution hourly for first three days then alternate hourly for 2 to 3 weeks. The frequency is reduced over months. 2. Diamidine 1. Propamindine 0.1% 2. Hexamidine 0.1% as solution to be instilled for 2 days, followed by six times a day for 3 months. 3. AntibioticsNeomycin 5.0 mg/drops to be instilled 2 hourly. 4. Antifungal A. Local (i) Ketaconazole (ii) Clotrimazole 1% drop B. Systemic (i) Ketaconazole (ii) Fluconazole Usual treatment of non healing corneal ulcers consists of : (i) Strong cycloplegic i.e. atropine sulphate 1% drop two times a day (in children atropine should be used under supervision). (ii) Repeated debridement (iii) Local beta blockers to keep the tension low. Role of local steroids is controversial and better avoided. Penetrating keratoplasty seems to be the ultimate method to salvage vision and save the eyeball. The infected corneal button removed from the patient should be subjected to histopathological identification of the organism.

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All persons using contact lens should be warned about possibility of contamination by various organism. They should be instructed to use standard cleaning solution. Use of home made solution should be a taboo. Corneal dystrophies80, 81, 82 Corneal dystrophies are a group of bilateral, non-inflammatory disorder of cornea. They are hereditary in nature of varied mode of transmission, however autosomal dominant trait is most common type of inheritance. Exact causes of corneal dystrophies are not known. They may be seen at any age but most of them start in childhood. Some of them may be present at birth. Corneal dystrophies generally involve central cornea, only few extend to the periphery, loss of vision depends upon position of the opacity in relation to the pupil and density of the opacities. Generally there are multiple opacities. Some of the dystrophies have loss of corneal sensation many of them develop recurrent erosion of corneal epithelium, dystrophic cornea do not get vascularised. There is no evidence of any other ocular disease in the affected eye. As corneal dystrophies are relatively uncommon and many of them may not require any treatment hence they are missed most of the time specially in under developed countries where bilateral opacities due to infection, inflammation and malnutrition are very common. All corneas with small central bilateral opacities without congestion and anterior segment reaction should arouse suspicion of corneal dystrophy and as many members of the family in as many generation possible should be examined to confirm diagnosis. There is no known medical treatment for corneal dystrophy. Lamellar corneal graft or penetrating keratoplasty or excimer may help to remove the opacities and improve vision. Recurrence of disease in grafted cornea is known. Classification. Corneal dystrophies are best classified according to topographic location of the dystrophy. A. Affecting anterior membrane (i) Affecting epithelium (ii) Affecting Bowmans membrane (iii) They involve superficial stroma. B. Affecting stroma C. Affecting the posterior membrane i.e. Descemets membrane and endothelium. D. Combined Following are a few common dystrophies that are seen in children. Anterior corneal dystrophies Commonly seen in children : 1. Hereditary juvenile epithelial dystrophy (Meesmanns). This is an autosomal dominant disorder, generally seen between 3 to 4 years of age. It has been reported under one year also. Visual acuity is not much reduced the lesions are best seen on retro illumination as micro vescicles in the deeper layers of epithelium. In the inter palpebral fissure corneal sensation is reduced. The disorder progresses very little. If vision is reduced sufficiently to hamper patients routine then only lamellar keratoplasty is recommended. The epithelial micro vescicles may raise the epithelium, this causes tear film abnormality. There may be irregular astigmatism. If corneal erosion occurs, which is rare, bandage contact lenses may be helpful.

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2. Hereditary epithelial dystrophy (Stocker and Holt). This is an autosomal dominant dystrophy, which is sometimes considered as variant of Messmanns dystrophy. It starts in infancy, loss of vision varies between slight haze to severe loss due to total opacification of the cornea. The epithelial surface is irregular leading to irregular astigmatism. Corneal sensitivity is sub-normal. Treatment depends upon degree of visual loss. In severe visual loss lamellar keratoplasty is advocated. In both Messmanns dystrophy and Stocker and Holt dystrophy, anterior stromal puncture along with bandage corneal lens may help epithelisation of erosion. Anterior stromal puncture is generally done by fine needle under magnification. This can also be done by YAG laser. Excimer laser is also used in case of recurrent corneal erosion. 3. Reis-Bucklers dystrophy. This condition is less common than previous two conditions. Inheritance is autosomal dominant. There are two types of this Bowmans membrane dystrophy i.e. (1) Classical less common variety and (2) More frequent type II. Common age of onset is three to five years. Loss of vision is severe as compared to other anterior dystrophies. Corneal sensation is reduced. Erosion is common. The lesions may lead to central corneal opacification. Management consist of local instillation of lubricating drop and ointment, hyperosmotic agents and antibiotic agent. Patching of the eye at bed time gives much relief to the patient, which can also be achieved by bandage contact lens. Soft contact lens also helps to reduce irregular astigmatism. Lamellar or penetrating corneal graft is required if(1) Vision is much hampered, (2) Lesion has reached anterior stroma, (3) There is vascularisation secondary to recurrent erosion. Excimer laser photo therapeutic keratoplasty helps to ablate the epithelium. This gives much respite from recurrent corneal erosion. 4. Hereditary anterior membrane dystrophy (Grayson-Wilbrandt). This is autosomal dominant dystrophy not seen before ten years of age. There is gradual loss of vision. Corneal sensation remains normal. On slit lamp examination the corneal nerves are found to be thickened corneal opacity may require lamellar keratoplasty. Stromal Dystrophies in Children 1. Lattice dystrophy (Biber-Haab). Lattice dystrophy is an autosomal dominant disorder that manifest at the end of first decade or early. In childhood vision is good that gradually diminish over years. There may be recurrent corneal erosion. There is gradual increase in corneal opacification that extend from limbus to limbus. The opacities are lattice in nature. The branching opacities may be confused with corneal nerve. Corneal sensation is good initially but gets reduced by third decade. Histochemically the opacities stain with Congo red. In childhood vision is good and does not require treatment. Recurrent corneal erosion in third and fourth decade is treated with artificial tear, lubricating ointment, soft contact lens. The eye may require patching. As the lesion is deep, penetrating keratoplasty is preferred. The donor cornea may develop opacities after ten years. Excimer laser, photo therapeutic keratectomy may help in removal of opacities. 2. Granular corneal dystrophy (Groenouws I). The condition has autosomal inheritance, starts in the first decade of life in the central cornea as white granules. Stroma in

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between the granules is clear hence initially there is no visual disturbance. The condition is almost symptomless in childhood. Symptoms manifest after fourth decade as irritation photophobia and diminished vision. Corneal sensation is normal. No treatment is warranted in childhood. Penetrating keratoplasty may be required after forty years of age. 3. Macular dystrophy (Groenouws II). This differs from most of the corneal dystrophies in being autosomal recessive. The disorder starts round about five years of age as greyish white opacities in the mid cornea that may spread up to limbus and involve the endothelium. By third decade there is troublesome corneal erosion. Definite treatment is keratoplasty in third and fourth decade. Children do not require any specific treatment. In suitable cases contact lens may help in improvement of vision. Crystalline dystrophy (Schnyders). This dystrophy is seen under ten years of age, may be present in infants. It has been reported at birth. It is autosomal dominant in inheritance. It starts in the centre of cornea as needle shaped crystals. The needles may come together to form a disc shaped opacity. The condition becomes stationary after third decade. There is no vascularisation. Corneal sensation is normal. No treatment is required. Posterior Dystrophies in Children Cornea deeper to stroma is less frequently involved in dystrophic conditions in childhood. There are two condition that are seen in childhood. They are : 1. Posterior polymorphus dystrophy. 2. Congenital hereditary endothelial dystrophy. Endothelial dystrophies are only conditions that may have other congenital anomalies of anterior segment. Posterior polymorphous dystrophy (P.P.M.D.). This condition is mostly seen at birth, when not associated with other anomalies of anterior segment like lridocorneal dysgenesis, correctopia, lrisatrophy, peripheral synechiae, congenital corneal edema, the condition is almost symptomless, does not cause visual impairment. It is mostly autosomal in nature, rarely it may be recessive. Congenital hereditary endothelial dystrophy (CHED). This dystrophy manifests before ten years, may be present at birth. There are two forms 1. Autosomal dominant. This presents in early childhood, progresses slowly but is symptomatic. 2. Autosomal recessive. This is present at birth but non symptomatic, diagnosed on routine examination. One unique feature of CHED is increased thickness of cornea. Vision may be between 20/40 to CF. Treatment. Most of the children do not require any treatment. Other associated congenital anomalies should be treated individually.

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COMPARATIVE FEATURE OF CORNEAL DYSTROPHY IN CHILDREN


Dystrophy Age of onset Heredity Visual loss Corneal sensation Erosion Complication Treatment

Anterior Dystrophy Meesmanns 3-4 yrs Autosomal dominant Mild to moderate Slight reduction Nil, Not required. BCL may help in erosion Irregular astigmatism L.K., BCL Corneal opacity P.B., CL, P.K, CL

Stocker-Holt

Infancy

Autosomal dominant Autosomal dominant

Mild to severe Severe 6/60 or less

Reduced

Reis-Buckler

3-4 years

Reduced

Recurrent

GraysomWilbrandt

10 yrs

Autosomal dominant

Gradual loss

Normal

Stromal dystrophies Groenouws I Lattice Groenouws II Macular Granular 10 yrs <10 yrs 10 yrs Autosomal dominant Recessive Autosomal dominant Moderate Severe Mild Reduced N ++ ++ Corneal opacity PK Corneal opacity PK Corneal vascularisation Nil Nil

Schnyder

Infancy

Autosomal dominant

Mild

Normal

Nil

Posterior dystrophy Posterior polymorphus corneal dystrophy Congenital Autsomal dominant/ recessive Mild N Nil May develop Glaucoma Endothelial Decompensation Symptomatic

214 BCL = Bandage Contact Lens CL = Contact Lens LK = Lamellar Keratoplasty PK = Penitrating Keratoplasty PB = Pad and Bandage CO = Corneal Opacity. Corneal degenerations83, 85, 86

PEDIATRIC OPHTHALMOLOGY

Degenerations of cornea are far more common than dystrophies. They are not genetically predisposed. They can start at any age. They can be unilateral. Bilateral degeneration are secondary either to bilateral ocular disorder like trauma, uveitis or systemic disorder like abnormal calcium metabolism. Commonest type of corneal degeneration seen in children is Band keratopathy. Band keratopathy84. (See page 248-249 as well) Band keratopathy can be divided into : 1. Primary (rare) 2. Secondary A. Secondary to ocular cause B. Secondary to systemic cause. Band keratopathy secondary to ocular disorder is commonest form of corneal degeneration seen in children. Chronic uveitis, chronic keratitis, penetrating injury, chemical injuries, congenital anomalies are common predisposing factors. Though it takes months to years to develop band keratopathy, it is reported to manifest within two to three weeks following alkali burn. The band keratopathy itself does not produce any symptom initially. It takes few months to years to be symptomatic. Band keratopathy develops in the inter palpebral fissure. It extends across the cornea in a band shaped opacity which starts from periphery and spread towards the centre, pupillary area is last to be affected. However in rare instance it may spread from centre to periphery. The opacity starts as a linear band one millimetre inside the limbus on either side. The outer edge of the opacity is sharply defined while the inner edge is irregular and serrated. Gradually opacities from each end meet to cover the cornea. There are few areas of deficiency in the opacity. They are called holes. Such holes correspond to corneal nerves. The opacity is caused by extra cellular deposition of non crystalline salts of calcium carbonate and phosphate at the level of Bowmans membrane and superficial stroma. The epithelium shows no change except that it may be irregular due to underlying deposits. Corneal sensation is not disturbed. The endothelium is not involved. Anterior chamber reaction when present is legacy of primary disorder i.e. uveitis etc. Exact cause of deposition of calcium at the level of Bowmans membrane in band keratopathy is not well understood, so is its predilection for exposed part of cornea. In case of band keratopathy secondary to ocular disorders, serum calcium is within normal range.

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Band keratopathy has been reported following use of silicone oil, sodium hyaluronate, sodium chordrontin sulphate, phenyl mercuric nitrate. Anterior segment ischaemia produces early band keratopathy. It has been observed in failed keratoplasty. Band keratoplasty secondary to systemic disorder. Band keratoplasty secondary to systemic disorders are less common than the former. Topography of the opacity is similar in both conditions. In latter the calcium is intra cellular and there is an alteration in calcium and phosphorous metabolism. Systemic conditions that cause band keratopathy areVitamin D toxicity, hyper parathyroidism, uremia, milk alkali syndrome, sarcoidosis, chronic renal failure, hereditary. Primary band keratopathy. This is rarest form of band keratopathy. There is lack of unanimity regarding its nature i.e. Is it a degeneration or dystrophy ? as there is overlaping of characteristics of both conditions. The condition is X linked recessive trait, present at birth or early childhood. There is no associated ocular disease. Besides cornea other parts of eye like lens, choroid, retina may also be involved. These eyes generally become blind in early life. Treatment of band keratopathy87. There is no known prophylaxis. However properly managed uveitis, glaucoma and trauma reduce the incidence. In band keratopathy secondary to systemic hyper calcimia, reduction in serum calcium sometimes clear the cornea. Similarly discontinuation of vitamin D. Enthusiastic supply of vitamin D in children should be discouraged. There are two modes of treatment for band keratopathy 1. Use of a chelating agent 2. Keratoplasty Treatment is indicated only in symptomatic cases. The success depends upon presence or absence of effect of primary disorder. Treatment of band keratopathy will restore vision only if the cause of defective vision is corneal. In presence of active inflammatory lesion or reactivation of lesion, the calcium deposit may start again. Use of chelating agent. Method consists of anaesthetising the cornea with local anaesthetic agent. In children the procedure should be done under general anaesthesia. Once the cornea and conjunctiva have been anaesthetised the epithelium is removed by scrapping with any suitable method. The calcium deposit is next scrapped off, the stroma preferably under microscope. A Week-cel sponge is soaked in 0.5% solution of EDTA. This is applied on the exposed calcium deposit, a to-and-from movement helps to remove the deposits better. If 0.5% EDTA fails to produce desired effect the strength of the solution is raised to 1% or 1.5%. Once all the deposits have been removed, the eye is patched with cycloplegic and local antibiotic drops. The aim of chelation is to remove the calcium without scarring of the stroma. A lamellar keratectomy or excimer laser photo therapeutic keratoplasty are better options than chelation. Drawback with excimer laser treatment is that it can cause post operative undesirable hypermetropia. Corneal opacity in new born and infants88, 90. Cornea of a new born is hazier than normal. This haze clears within few days without any treatment and is generally not noticed

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by mother or nurse unless looked for. However a child may be born with corneal haze sufficient to be called opacity. A child may have bright and clear cornea at birth but later becomes hazy. Commonest cause is buphthalmos. Corneal haze in new born may be unilateral or bilateral. Commonest cause of unilateral haze is birth trauma. Buphthalmos that is more advanced in one eye may also produce unilateral hazy cornea. Common causes of corneal opacity are : 1. Sclero cornea of various degrees. 2. Tear in Descemets membrane 3. Ulcer (keratitis) 1. Birth trauma 2. Buphthalmos 1. Herpes simplex 2. Congenital rubella 3. Lid anomaly (Neurotrophic) 4. Metabolic 5. Posterior corneal defect 6. Endothelial defect Mucopolysaccharidosis Anterior chamber cleavage syndrome 1. Congenital hereditary endothelial defect (CHED) 2. Posterior polymorphs dystrophy 3. Congenital hereditary, stromal dystrophies 7. Dermoid 1. Corneal 2. Limbal The above conditions are acronym as STUMPD89 with beginning alphabet of each cause. Interstitial keratitis is a common cause of bilateral corneal haze but seen only after third year of life with redness, photophobia and lacrimation. Xerophthalmia and Keratomalacia91, 92, 93 Logically the term xerophthalmia should include all the conditions that produce non wetability leading to dryness of ocular surface i.e. cornea and conjunctiva. However term xerophthalmia93 is generally used to denote dryness of conjunctiva and cornea due to vitamin A deficiency. Similarly xerosis is a term used for which hypovitaminosis A is the prime cause. There are many other factors that lead to dryness of conjunctiva. Keratomalacia93 denotes softening and aseptic and necrosis of cornea due to deficiency of vitamin A, that is generally preceded by xerophthalmia. Tear is essential to keep the cornea and the conjunctiva bright and moist. Normal tear has three layers of different chemical composition and each has specific purpose. The layers areLipid, aqueous and mucin. Deficiency of any layer will lead to tear film abnormality. Aqueous layer is voluminous when compared to other layers. It is sandwiched between lipid and mucin layer. The lipid layer prevents excess evaporation of aqueous layer and gives a shinning surface while, the mucin layer anchors the aqueous layer to the cornea and the

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conjunctiva. In hypo vitaminosis A there is hyposecretin from goblet cells of conjunctiva. The aqueous and lipid layers are unaffected, lack of mucin causes abnormal break up time resulting in formation of large dry spot on the ocular surface. There is thickening and loss of elasticity of conjunctiva, it may be keratinised which leads to opacification of conjunctiva. There is increased pigment deposit on the conjunctiva that appears as dusty white patch. Normally the conjunctiva is not thrown into vertical folds when the eye is rotated but in case of dry conjunctiva, the conjunctiva is thrown into three to four vertical folds in the intra palpebral aperture. The exact mechanism of dryness of cornea is not understood. Lack of mucin hasten tear break up and delayed bridging of the gap. There is degeneration of Bowmans membrane infiltration of stroma by inflammatory cells and fluid. Vitamin A is essential for maintenance of conjunctival goblet cells that secrete mucous. Daily requirement of vitamin A varies with age. An infant requires five times more than an adult. An infant requires 65 mg/kg of vitamin A while requirement of adult is 12 mg per kg of body weight that roughly comes to 2500 IU of pure vitamin A or 4000 IU of carotene. Retinol is fat soluble vitamin A, that is of animal origin found in fish, meat, poultry and milk. Carotenes are precursors of vitamin A. It is found mostly in green leafy vegetables, yellow fruits, red palm oil. The carotenes are converted to retinol in the small intestine from where it is absorbed and transported to liver, which is reservoir of vitamin A. The retinol stored in liver is released in blood stream as retinol binding protein, hence systemic protein deficiency may precipitate xerophthalmia if it is not corrected. Retinol besides maintaining health of epithelial cells of conjunctiva also plays an essential role in night vision. Retinol combines with protein in rods and act as light stimulus, exciting the optic nerve in scotopic vision. Vitamin A is involved in photo chemistry of night vision. It is involved in breakdown and resynthesis of rhodopsin. Night blindness sets in when serum vitamin A level falls below 50 IU/L. This may happen in : 1. Reduced consumption 2. Increased loss or 3. Reduced storage in liver. 4. Deficiency of vitamin A responds quickly to administration of water soluble vitamin A by intra muscular injection within twenty to twenty four hours. Clinical features. Clinical features depend upon severity, duration, associated secondary infection of the eye and systemic infective diseases like pneumonitis, gastro enteritis, measles etc. Xerophthalmia has been classified variously91 1. Prexerosis, 4. Keratomalasia, 2. Conjunctival xerosis, 5. Perforation. 3. Corneal xerosis,

Most commonly used classification is that given by WHO94 that divides xerophthalmic signs into two broad groups i.e. primary and secondary signs. Each group has been divided into three sub groups :

218 Primary signs96 X X X X 1A 1B 2 3A 3B XN XF XS Conjunctival xerosis Bitots spot with conjunctival xerosis Corneal xerosis Corneal ulcer with xerosis Keratomalacia Night blindness Xerophthalmic fundus Xerophthalmia scar

PEDIATRIC OPHTHALMOLOGY

Secondary signs

Where X stands for xerosis Symptoms of xerophthalmia can be related to 1. Conjunctival and corneal 2. Night blindness. One may precede the other or may be found together. There are instances where the eye may advance into conjunctival and corneal xerosis without night blindness, vice versa is rare. Clinical signs 1. ConjunctivalVertical folds on looking laterally 2. Dryness of conjunctiva 3. Pigmentation on dry conjunctiva 4. Bitots spot. Bitots spot94. These are triangular raised white areas developing mostly on the lateral side of bulbar conjunctiva in the inter palpebral aperture little away from the limbus. The triangle has its base towards the cornea and apex towards outer canthus. The surface of the triangle is foamy, the foamy substance can be removed with ease only to reappear. The foam is caused by action of xerosis bacilli. The surface of Bitots spot does not wet with tear. Kajal and Surma applied to lid margin do not stain normal conjunctiva but the surface of the Bitots spot and dry conjunctiva stains with Kajal and Surma. Bitots spot is not specific of vitamin A deficiency. It can be idiopathic as well. Bitots spot due to vitamin A deficiency disappears after sometimes but idiopathic Bitots spot does not respond to administration of vitamin A and is seen in older children and adults. 2. Corneal signs. Cornea develops dry spots, tear film break up time is less than 10 seconds. Tear production and composition of tear is within normal limits, abnormal tear break up is due to changes in corneal epithelium. The cornea is lusturless. These early changes are reversible and respond promptly to systemic administration of vitamin A. If the condition is not arrested at this stage it proceeds to corneal ulceration which is generally central, surrounded by hazy or opaque cornea. Peculiarity of these ulcers is that they are aseptic in nature. There is minimal congestion and hardly any pain. The stroma is involved following break in epithelium. The stroma is involved more easily and severely in vitamin A deficiency

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than seen in normal eye when epithelium is damaged. At this stage vigorous local and systemic treatment may avert perforation but complete recovery with clear cornea is never achieved. Keratomalacia. This is advanced stage of xerophthalmia. Here the cornea becomes soft and virtually melts away within twenty to twenty four hours. This has not been produced in experimental animals. The central part is covered by a white gelatinous material due to collagenase enzyme. Perforation with protrusion of intraocular tissue is inevitable. Scar following keratomalacia is generally bilateral, one eye may be more advanced than the other. In developing countries with eradication of small pox, keratomalacia has become foremost cause of bilateral corneal staphyloma in children. Night blindness. Night blindness due to vitamin A deficiency is of acute onset. The child prefers to sit in one place and avoid moving. The child is so incapacitated that he may not locate his food in the plate placed in front of him. This has been termed as chicken blindness. Night blindness due to vitamin A deficiency responds to systemic vitamin A therapy quickly. Xerophthalmia due to vitamin A deficiency is never isolated. It is invariably associated with protein caloric malnutrition. It is very common following attack of measles, diarrhoea, malaria, worm infection, mal-absorption syndrome. Management of xerophthalmia97, 98 Management consists of : 1. Prophtlaxis 2. Therapeutics Prophylaxis98. Xerophthalmia is one of leading cause of bilateral blindness in children which is fully preventable by simple and cost effective methods. Moreover vitamin A deficiency is also responsible for a large number of deaths in infants. Prophylaxis in mother and new born. Vitamin A is stored in liver and secreted in mothers milk in sufficient quantity to prevent deficiency in infants. Hence it is of utmost importance that mother has good storage of vitamin A during pregnancy and lactation. The colostrum is rich in vitamin A, hence soon after birth the child should be put to breast of the mother and allowed to take the collustrum. If the child gets sufficient amount of mothers milk and thrives on it, there is no need of any supplementation of vitamin A. The child should be breast fed as long as there is sufficient mothers milk for the child. If top feed is to be started bovine milk is preferred over formula milk. From the age of five months solid food should be started in consultation with pediatrician, adding green leafy vegetables, yellow fruits and vegetables to cereals and pulses. Vitamin A prophtlaxis between 6 months to 6 years. Every child between six months to six years should get an oral dose of 200,000 unit of vitamin A concentrate. Under National Programme of Prevention of Xerophthalmia, vitamin A is distributed free from primary health centres, government hospitals. The first dose may be delayed up to nine months and given along with measles vaccine. It is not clear if vitamin A deficiency precipitates attack of measles or vice a versa. However administration of vitamin A along with management of measles has reduced mortality in measles.

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Fortification of food with naturally occurring vitamin A. Children and young adults should be encouraged to take vitamin rich food as part of daily diet in the form of green vegetables, yellow tubers, yellow fruits. Daily requirement of vitamin in normal individual : 1. Pregnancy and lactation3000-3500 IU (300 mg to 750 mg) 2. 0-4 years1000 to 1200 IU 3. School children2250 IU Management of clinical xerophthalmia : Night blindness99. Intra muscular injection of water soluble 100,000 IU of vitamin A followed by (1) 200,000 IU oral after seven days or (2) Repeat 100,000 IU intra muscular after seven days. Xerosis200,000 IU of oral vitamin. Keratomalacia WHO recommendation : 1. Immediate upon diagnosis - 200,000 IU orally. 2. Next day200,000 IU orally 3. After seven days200,000 IU orally. 4. Repeat 200,000 IU every six months up to six years of age to prevent recurrence. 5. If the child is unable to take oral feed or has vomiting, 100,000 IU of water soluble vitamin A is given as injection. Children less than 8 kg should receive half the above dose and under 4 kg quarter of the above dose. Ocular therapy A. Local instillation of antibiotic drops every 2 hourly and ointment during sleep to prevent sticking of lids. B. AtropineIn all cases of corneal involvement irrespective of actual ulceration atropine is used as 1% ointment two times a day. Atropine as drop is better avoided in children for one drop of atropine contains more than therapeutic dose of atropine. Thus one drop in each eye reaches almost lower level of toxicity especially when the child is under weight. C. Local retinolTretinoin 0.1% as drops in oil three times a day is claimed to retard progress of corneal involvement. D. Local tear substitutesThough aqueous part of tear film is not reduced yet it fails to wet the cornea and conjunctiva due to changes in epithelium. Local artificial tear may help in preventing the cornea from drying. The childs total protein and calories should be calculated and supplied because vitamin A deficiency does not occur in isolation. There should be adequate supply of other vitamins and minerals specially the trace elements.

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Other causes of xerosis. Coloboma of lids, ectropion of lid, lagophthalmos, proptosis, Steven-Johnsons syndrome, Riley Day syndrome, acid alkali burn.

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34. Shields M.B. : Congenital glaucoma in Text Book of glaucoma. Fourth edition. p-198, Williams and Wilkins, Philadelphia, 1998. 35. Duke Elder S. : Inflammation of the cornea in System of ophthalmology. Vol. VIII, Part II. Edited by Duke Elder S. and Leigh A.B. p-729-860, Henry Kimpton, London, 1967. 36. Vastine David : Corneal ulceration : Classification, signs and symptoms in Principle and practice of ophthalmology. Vol. I, p-285-294, Edited by Peyman G.A., Sander D.R. and Goldberg M.F. 1st Indian edition, Jay Pee Brothers, New Delhi, 1987. 37. Dhanda R.P. and Kalever V. : Infected corneal ulcer in A Text Book of Ophthalmology. New edition p-207-216, Galgotia Publication Pvt. Ltd., New Delhi, 1993. 38. Reddy M., Savitri Sharma and Rao G.N. : Corneal ulcer in Modern ophthalmology. Vol. I. Second edition. Edited by Dutta L.C. p-200-214, Jay Pee Brothers, New Delhi, 2000. 39. Coster D.J. and Badenoch P.R. : Bacterial corneal ulcer in Current ocular therapy. Fifth edition. Edited by Fraunfelder F.T. and Roy H.F. p-345-346, W.B. Saunders Company, Philadelphia, 2000. 40. ODay D.M. : Fungal keratitis in Current ocular therapy. Fifth edition, edited by Faunfelder F.T. and Roy H.F. p-360-361, W.B. Saunders and Company, Philadelphia, 2000. 41. Roberson M.C. : Fungal keratitis in Current ocular therapy. Fourth edition, Edited by Fraunfelder F.T. and Roy F.H. p-492-493, W.B. Saunders Company, Philadelphia, 1995. 42. ODay D.M. and Eileen M : Burd Fungal keratitis and conjunctivitis in The Cornea. Third edition. Edited by Smolin G. and Thoft RA. p-229-252, Lippincot William and Wilkins, Philadelphia, 1994. 43. Yee R.W. and Cheng C.J. : Central corneal ulcer in Decision making in ophthalmology. First Indian edition. Edited by Heuven WAJ and Zwaan J.T. p-154-155, Harcort Brace, Singapore, 1998. 44. Liegner J.T. and Yee R.W. : Marginal corneal ulcer in Decision making in ophthalmology. First Indian edition. Edited by Heuven WAJ and Zwaan J.T. p-152-153, Harcort Brace, Singapore, 1998. 45. Duke Elder S. : Corneal scar in System of ophthalmology. Vol. 8, Part II. p-639, Edited by Duke Elder S. and Leigh A.G, Henry Kimpton, London, 1965. 46. Dhanda R.P. and Kalever V. : Corneal scars in A Text Book of Ophthalmology. New First edition. p-258-264, Galgotia Publications Pvt. Ltd., New Delhi, 1993. 47. Ostler H.B., Whitcher J.P. and Cevallos V. : Pneumococcus in Current ocular therapy. Fifth edition, edited by Fraunfelder F.T. and Roy F.H. p-58-59, W.B. Saunders Company, Philadelphia, 2000. 48. Duke Elder S. : Central corneal ulcer in System of Ophthalmology. Vol. VIII, Part II. Edited by Duke Elder S. and Leigh A.G. p-777-800, Henry Kimpton, London, 1965.

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49. Wilkins J. Whitcher J.P. and Margolis T.P. : Penicillin resistant streptococcus pneumonae keratitis. Cornea 15 : 99-100. 1996. 50. OBrien T.P. : Bacterial keratitis study research group. Efficacy of ofloxacin vs. cifazolin and tobramycin in Bacterial keratitis. Arch. Oph. 113:1257-1265, 1995. 51. Laibson P.R. : Pseudomonas aeruginosa : In Current ocular therapy. Fifth edition. p-61-63, Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Philadelphia, 2000. 52. Gittinger J.W. : Herpes simplex keratitis in Manual of clinical problems in ophthalmology. First edition. Edited by Gittinger J.W. and Asdourian G.K. p-46-47, Little Brown and Company, Boston, 1988. 53. Deborah Pavan Langston : Herpes simplex virus kerato conjunctivitis and iritis in Manual of ocular diagnosis and therapy. Fifth edition. p-89-92, Lippincot Williams and Wilkins, Philadelphia, 2002. 54. Deborah Pavan Langston : Herpetic infection in The Cornea. Third edition. p-183-208, Edited by Smolin G. Thoft R.A., Lippincot Williams and Wilkins, Philadelphia, 1994. 55. Mudgal P.C. : Antiviral agents in Diagnosis and management of ocular inflammations. Second edition, p-54-62, Edited by Jain M.R., Jay Pee Brothers, New Delhi, 1990. 56. Missotten L. : Management of herpes simplex keratitis in Diagnosis and management of ocular inflammation. Second edition. p-63-65, Edited by Jain M.R. Jay Pee Brothers, New Delhi, 1990. 57. Malouf D.J. and Oates R.K. : Herpes simplex virus infection in neonate. Jr. Op. Paed. and Clin. health. 31:332-335, 1995. 58. Athmanathan S., Garg P. and Rao Gullapalli N. : Ophthalmic antiviral chemotherapy. An overview. Ind. Jr. Oph. 45 : 203-210, 1997. 59. Kanski J.J. : Dendriticular in Clinical ophthalmology. Second edition. p-99-101, Butterworth, London, 1989. 60. Laibson P.R. and Waring G.P. : Diseases of the Cornea in Paediatric Ophthalmology. Vol. I, p-490-498, Edited by Harley R.D., W.B. Saunders Company, Philadelphia, 1983. 61. Suresh P.S. and Tullo A.B. : Herpes Simplex Keratitis. Ind. Jr. Oph. 47 : 155-165, 1999. 62. Gorden J.S. : Adenoviral ocular infection in The Cornea. Third edition. p-215-225, Edited by Samolin G. and Thoft. P.A., Lippincot Williams and Wilkins, Philadelphia, 1994. 63. Forster R.K. : Fungal keratitis and conjunctivitis in The Cornea. Third edition. p-239251, Edited by Samolin G. and Thoft R.A., Lippincot Williams and Wilkins, Philadelphia, 1994. 64. ODay D.M. : Fungal keratitis in Current ocular therapy. Fifth edition. p-360-361, Edited by Fraunfelder F.T. and Roy H.F., W.B. Saunders Company, Philadelphia, 2000. 65. Savitri Sharma, Silverberg M., Mehta P., Usha Gopinath, Agrawal W. and Naduvilath T.J. : Early diagnosis of mycotic keratitis predictive value of potassium hydroxide preparation. Ind. Jr. Oph. 31-35, 1998.

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66. Thomas P.A., Kuriacose T., Kirupa Shankar M.P. and Maharajan V.S. : Use of lactophenol cotton blue mount of corneal scrapping as an aid to the diagnosis of mycotic keratitis. Diag. microbio. 14 : 219-224, 1991. 67. Arffa R.C., Avni I. Aschibashiy, Robin J. and Kaufmann H.E. : Calco flurol ink potassium hydroxide preparation for identifying fungi. Am. Jr. Oph. 100: 719-723, 1985. 68. Vajpayce R.B. Angra S.K., Sandramoulis, Honavar S.G. and Chabra V.K. : Laboratory diagnosis of keratomycosis, comparative evaluation of direct microscopy and culture results. Annl. Oph. 25 : 68-71, 1993. 69. Jain M.R. : Keratomycosis and antifungal drugs in Diagnosis and management of ocular inflammation. Second edition. p-28-32, Jay Pee Brothers, New Delhi, 1990. 70. Ram J. and Pandey S.K. : Peripheral corneal ulcers in Modern Ophthalmology. Vol. I. Second edition, p-238-241, Jay Pee Brothers, New Delhi, 2000. 71. Duke Elder S. : System of ophthalmology. Vol. VIII, Part II. Edited by Duke Elder S. and Leigh A.G. p-815-830, Henry Kimpton, London, 1965. 72. Ogawa G.S.H. and Hyndiuk R.A. : Non ulcerative keratitis in The Cornea. Third edition p-144-149, Edited by Smolin G. and Thoft R.A. Lippincot Williams and Wilkins, Philadelphia, 1994. 73. Shalaby A. and Dunn J.P. : Acquired and congenital syphilis in Current ocular therapy. Fifth edition. p-2-5, Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Philadelphia, 2000. 74. Margo C.E. and Hamed L.M. : Ocular syphilis. Surv. Ophth. 37 : 207-220, 1992. 75. Wilhelmus K.R. Acanthamoeba keratitis in The Cornea. Third edition. p-262-266, Edited by Smolin G. and Thoft R.A., Lippincot Williams and Wilkins, Philadelphia, 1994. 76. Stuphin J.E. : Acanthamebiasis in Current ocular therapy. Fifth edition, p-89-90, Edited by Fraunfelder F.T. and Roy F.H., Philadelphia, 2000. 77. Singh S. and Sachdeva M.P.S. : Acanthameoba keratitis. B.M.J. 309:273, 1994. 78. Sharma S., Srinivasan M., and George C. : Diagnosis of acanthamoeba keratitis. Ind. Jr. Oph. 38:50-56, 1990. 79. Dart J.K.G. : Clinical features of acanthamoeba keratitis. Community eye health. 8 : 1, 1995. 79.A. Seal S.K. : Acanth amoeba keratitis in G.N. Seals Textbook of ophthalmology 5th Ed. p-162-163, current book int Kolkata. 2002. 80. Barua C.K. and Dutta L.C. : Corneal degenerations and dystrophies in Modern Ophthalmology. Vol. I, second edition. 193-199, Edited by Dutta L.C., Jay Pee Brothers, New Delhi, 2000. 81. Duke Elder S. : Corneal dystrophies in System of ophthalmology. Vol. 8, Part II, p-921-932, Edited by Duke Elder S. and Leigh A.G. Henry, Kimpton, London, 1965. 82. Laibson P.R. and Waring G.O. : Corneal dystrophies and degeneration in Pediatric Ophthalmology. Vol. I, Second edition. p-502-506, Edited by Harley R.D., W.B. Saunders Company, Philadelphia, 1983.

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83. Samolin G. : Corneal dystrophies and degeneration in The Cornea. Third edition. p-499-508, Edited by Samolin G. and Thoft R.A., Lippincot Williams and Wilkins, Philadelphia, 1994. 84. Duke Elder S. : Band shaped keratopathy in System of ophthalmology. Vol. VIII, Part II. p-893-902, Edited by Duke Elder S. and Leigh A.G., Henry Kimpton, London, 1965. 85. Doughman D. : The cornea in Principle and practice of ophthalmology. Vol. I. First Indian edition. p-414-419, Edited by Peyman G.A. Sander D.R. and Goldberg M.P., Jay Pee Brothers, New Delhi, 1987. 86. Tran D.M. and Schanzlin D.J. : Corneal and conjunctival calcification in Current ocular therapy. Fifth edition. p-329-330, W.B. Saunders Company, Philadelphia, 2000. 87. O,Brart D.P.S. and Gartry D.S. : Treatment of band keratopathy by excimer laser photo therapeutic keratectomy. B.J.O. 77 : 702-708, 1993. 88. Peterson R.A. and Boger W.D. : Pediatric ophthalmology in Manual of ocular diagnosis and therapy. Fifth edition. p-288, Edited by Deborah Pavan Langston. Lippincot, Williams and Wilkins, Philadelphia 2002. 89. Liabson P.R. and Waring G.O. : Diseases of the cornea in Pediatric ophthalmology. Vol. I Edition II. p-456-490, Edited by Harley R.D., W.B. Saunders Company, Philadelphia, 1983. 90. Zwaan J.J. : Cloudy cornea in a neonate in Decision making in ophthalmology. First Indian edition. p-128-129, Edited by Vantteuven W.AJ and Zwaan J.T., Harcort Brace, 1992. 91. Duke Elder S. : System of ophthalmology. Vol. VIII. Part 2. p-1115-1125, Edited by Duke Elder S., Leigh A.G. Henry Kimpton, London, 1965. 92. Sommer A. : Xerophthalmia and keratomalacia in Nutritional Blindness. Oxford University Press, 1982. 93. Kenyon K.R. and Chaves H.V. : Morphological and pathological response of cornea and conjunctiva diseases in The Cornea. Third edition. p-71-78, Edited by Smolin G., Thoft R.A. Lippincot Williams and Wilkins, Philadelphia, 1994. 94. WHO : Vitamin A deficiency and xerophthalmia. Technical report No. 590, 1976. 95. Vaughan D. and Asbury T. : Corneal ulcer due to vitamin A deficiency in General Ophthalmology. Tenth edition. p-97, Lange Medical Publication, California, 1983. 96. McLaren D.S. and Frigg M. : Xerophthalmia in Sight and life manual of vitamin A deficiency. Second edition. p-51-62, Published by Task Force Sight and Life, Basel, 2001. 97. Sommer A. : Impact of vitamin A supplemention of childhood mortality. Lancet 1:1169173. 98. Sharma P. : Xerophthalmia in Essentials of ophthalmology. First edition. p-114-116, Modern Publishers, New Delhi, 2000. 99. Sommer A. : Hypovitaminus A in Current ocular therapy. Fifth edition. p-107-108, Edited by Fraunfelder F.T. and Roy. H.F., W.B. Saunders Company, Philadelphia, 2000.

CHAPTER

Disorders of the Uvea in Children


Uvea is the middle vascular pigmented coat of the eye ball. It is divided into three parts.1 1. Iris 2. Ciliary body 3. Choroid. All the three parts develop from neuro ectoderm and mesoderm. The iris Iris is the most anterior part of the uvea that is visible on oblique illumination. It is practically a diaphragm of blood vessels and unstripped muscle fibres, held together by loose stroma. It is coronal to the cornea but not parallel to it. It is 12 mm. in diameter, from root to root, all round with a circular hole in the center the pupil. Thickness of the iris is not uniform. It is thinnest at its insertion (root) to the middle of the anterior border of the ciliary body. Its thickness is maximum at the collarette, then its thickness gradually diminishes towards the pupil in a rounded fashion. The iris divides the aqueous chamber into anterior and posterior chambers. The colour of the iris not only varies from race to race, but also from person to person. It may vary in two eyes or in the same eye. Colour of the iris is due to the presence of pigments in the iris. More the pigment, darker is the iris. Colour of the iris of the new born is lighter than that of adults. Some of the stromal vessels are visible in the iris of the new born, which disappear after a few weeks. Difference in the colour of the iris in two eyes is called heterochromia. Difference in colour of one iris from its counterpart is called heterochromia iridum while difference in colour of iris in the same eye is called heterochromia iridis.2 Heterochromia is generally congenital in nature. In albinism the iris is generally bluish, almost translucent that transilluminates with reflected light. The anterior surface of the iris is rough. It is divided into two zones by a circular raised irregular lines called the collarette i.e. 1. Pupillary zone that is 1.5 mm. wide from the collarette to pupillary margin, is lined by a thin zone of pigment called iris ruff, which is in fact continuation of the posterior pigment layer. The pupillary zone contains the sphincter pupillae muscle that is a smooth muscle and originates from the neuroectoderm and is supplied by parasympathetic nerve. 2. The ciliary zone is a wider zone that extends from collarette to the root of the iris. It is relatively rough due to the presence of series of radial elevated ridges that

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represents radial blood vessels in the stroma. Besides the ridges there are scattered depressions that are formed due to lack of superficial layers of the iris. The peripheral crypts communicate with the anterior chamber.3 As the root of the iris is the thinnest part of iris, it is most liable to be torn following a blunt injury without bleeding. The detachment of the iris from the root is called iridodialysis. The posterior surface though not very smooth, is flatter than the anterior surface. The posterior surface rests on the anterior surface of intact lens and glides smoothly over the lens capsule. The posterior surface is dark brown or black due to the pigment in the epithelium. Microscopically the iris is divided into: 1. Endothelium. 2. Stroma. 3. Posterior epithelium. 1. The endothelium. The presence of endothelium is controversial4,5. Traditionally it was thought to be an extension of the cells that line the posterior layer of cornea. Now it is presumed to be distributed in patches. The endothelial layer is absent over the crypts. 2. The stroma has been divided into: (i) Anterior limiting layer, (ii) Stroma proper. (i) Anterior limiting layer. The Anterior limiting layer is a modification of superficial layer of stroma1. Some authors consider that what was thought to be endothelium of the iris is in fact a layer of condensation of anteriors limiting layer.5 This layer consists of melanocytes, fibroblasts and collagen fibres. Amount of pigment and the thickness of this layer is responsible for the colour of the iris. In blue coloured iris there is less pigment and in dark coloured iris there is more pigment in this layer. Nerve endings and capillaries are present in this layer. This is the place where neovascularisation of iris begins. (ii) Stroma proper. Stroma proper consists of blood vessels, nerves, unstratified muscles, fibroblasts, lymphocytes and mast cells. The blood vessels arise from the greater circle of the iris that is in fact situated in the ciliary body in a radiating fashion towards the collarettes where they anastomose to form the lesser circle of the iris. The vessels in the iris are nonfenestrated, hence not suitable for ordinary fluorescein angiography. The nerves present in the stroma arebranches of trigeminal, oculomotor and sympathetic. The unstratified muscles are: 1. The sphincter pupillae is present in the pupillary zone, it is a narrow strip of about 1 mm. width that encircles the papillary border, it comes in close proximity of the posterior pigment epithelium. The function of the muscle is to constrict the pupil. The pupil continues to constrict even in presence of iridectomy. It is supplied by the parasympathetic fibres of the third nerve. 2. The dilator pupillae lies in the ciliary zone of the iris. Direction of the fibres in this zone is radial, it is more bulky than the sphincter, it lies in the posterior part of the stroma. Its function is to dilate the pupil, it is innervated by cervical sympathetic nerve. The dilator pupillae arises from the anterior epithelial layer. 3. Posterior epithelium. It is two layered. A part of it represents outer wall of the optic cup. It is the forward extension of pigment epithelium of retina and ciliary body and is commonly referred to anterior epithelium.

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The posterior epithelium is the anterior extension of non pigmented epithelium of ciliary body and merges with the anterior epithelial layer. The pigment epithelium acts as blood aqueous barrier besides preventing light from entering the eye. The pupil The pupil is not a tissue, it is a deficiency in the iris. It is circular in shape, situated in the center and slightly nasally in the iris. The periphery of the pupil is black in colour due to posterior pigment epithelium of the iris spilling anteriorly. This is called ruff of the iris. The size of a normal adult pupil is 2 mm. to 4 mm. It is smaller in new born and in old age. Pupil smaller than normal is called miotic pupil, while larger than the normal is called mydriatic pupil. The drugs that constrict the pupil are called miotics, while those that dilate are called mydriatics. Generally the size of the pupil is equal in the two eyes. Difference in the sizes of the pupil in two eyes is called anisocoria. Presence of more than one pupil in each eye is called polycoria, which is a congenital condition. Pupil in true polycoria should have independent sphincter and dilator muscles. Pseudopolycoria can be seen as congenital anomaly i.e. dysgenesis of anterior chamber or may be acquired following trauma which is mostly surgical or may be accidental. In some chronic uveitis, there may be loss of iris tissue resulting in holes in the iris. A deficiency in iris is called coloboma that can be congenital or acquired. It is very common for the coloboma of the iris to extend up to pupil resulting in a vertically enlarged key hole shaped pupil. Such pupil are capable of reacting to light and accommodation to a limited extent. Corectopia is a term used to denote the position of the pupil other than normal, this is generally bilateral and congenital. Normally the pupil is never stationary and its size keeps on changing within a normal range, with change of intensity of light and accommodation. An appreciable flicker of pupil is called hippus, which is of no clinical significance. The function of the pupil is to control the light entering the eye. It acts as communication between anterior and posterior chamber through which aqueous passes from the posterior chamber to the anterior chamber. The ciliary body This is the middle part of the uvea. It extends from scleral spur anteriorly, to ora serrata posteriorly. It encircles the interior of the sclera. In section it looks like an isosceles triangle, the base of which points towards the pupil. The outer edge of the base is attached to the scleral spur, 1.5 mm. away from the limbus. From the middle of the base arises the iris dividing the base into two parts, the anterior part forming the angle of the anterior chamber. This part is visible on gonioscopy only. The posterior part forms the boundary of the posterior chamber. The ciliary body is highly vascular and bleeds profusely on trauma, both blunt and penetrating. Its colour is brownish black. The ciliary body is empirically divided into two parts: 1. Pars plicata and 2. Pars plana.

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1. Pars plicata. The Pars plicata is anterior 2 mm. of the ciliary body. This is the thickest part of the uvea. It comprises of ciliary stroma, ciliary muscles, blood vessels and nerves. The inner surface of para plicata is divided into 70 to 80 ridges or plications. These are known as ciliary processes, they extend towards the lens, in finger like processes. Each of them is 0.5 mm. x 2.00 mm. in size. From the tips of these processes arise the zonules of the lens. The lens and the zonules together form a barrier that separates the vitreous chamber from the aqueous chamber. The ciliary processes are made up of capillaries covered by two layers of ciliary epithelium. They do not have any stroma or muscles, their length depends on tightness of the ciliary muscles3. Besides acting as attachment of zonules, that are responsible for accommodation, the other function of the ciliary process is to secrete aqueous. The ciliary epithelium acts a blood aqueous barrier. The non-pigment epithelium secretes aqueous while the pigment epithelium continues as retinal pigment epithelium. 2. Pars plana. The pars plana is posterior 6 to 7 mm. of the ciliary body. This gradually looses its musculature and tappers as a thin layer to end in ora serrata that ultimately blends with the choroid and the retina. The vascular layer of the pars plana is similar to that of choroid without choriocapillaries. The vitreous base extends from retinal periphery up to 2 mm. of pars plana. The pars plana is thought to secrete mucopolysaccharides of the vitreous.6 The pars plana being less vascular than any other part of the uvea with minimum thickness is the natural choice of entry for intra vitrial surgery and lensectomy. The ciliary muscles form the main bulk of the ciliary body. The muscles are nonstriated muscles that has three parts. The most important are the longitudinal fibres. The middle part is formed by the radial fibres. While the innermost are the circular fibres. Most of the bulk of the ciliary muscle is located in the anterior two-third of the ciliary body. The exact function of the ciliary muscles are not well understood, however, the ciliary muscles take a major part in accommodation. Contraction of the ciliary muscles open up the angle of the anterior chamber to facilitate aqueous out flow. The nerve supply of the ciliary body is mostly by parasympathetic nerve. Sympathetic supply has lesser role to play in the ciliary body. The sensory supply is via trigeminal. The choroid The choroid is the posterior most part of the uvea and forms a major part of the middle layer of the coats of the eyeball. It extends from optic nerve to the ora serrata between the sclera on the outer side and retina on the inner side. It is continuous with ora serrata anteriorly with loose attachment to the 1. The sclera, 2. The exit of the vortex veins near the equator, 3. round the disc at posteriorly. Its thickness is not uniform throughout, at the posterior pole it is 0.25 mm. and anteriorly 0.1 mm. in thickness. Its attachment to most of the sclera is loose. There is a potential space in between the sclera and the choroid called suprachoroidal space that is liable to distended by fluid. The separation is more pronounced anteriorly resulting in a ciliochoroidal detachment following trauma and inflammation. The choroid can not be separated at the exit of the vertex veins and round the disc due to firm attachment. The suprachoroidal space extends anteriorly under the ciliary body as supraciliary space. This space is traversed by ciliary vessels and nerves. The ciliary vessels do not give any branch in the suprachoroidal space but the ciliary nerves give fine branches to the outer layer of the choroid.

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Histologically the choroid is divided into: 1. Supera choroidal lamina 2. Vascular layer 3. Bruchs membrane. 1. Suprachoroidal lamina. The suprachoroidal lamina contains melanocytes, fibrocytes and thin fibres. This layer is attached to the choroid on its outer surface, and is attached to the inner surface of the sclera. 2. The vascular layer forms the main bulk of the choroid. It consists of three layers of blood vessels i.e. (i) The layer of large vessel (ii) The layer of small vessels (iii) The choriocapillaries. The size of the blood vessels diminish in size from outer surface to the inner surface. The outer layer is mostly venous in nature. The veins unite to form vortex veins which are four in numbers, one in each quadrant. The lower vortex veins are formed by union of choroidal vessels 3 mm behind the equator. Each vortex system unites to form an ampula that drains obliquely through a scleral canal. All the vortex veins drain into ophthalmic veins, there are no valves in choroidal veins7. The spaces in between the vessel is occupied by strands of stroma, melanocytes and fibrocytes like iris and ciliary body. There is no muscle in the choroids. Choriocapillaries. The choriocapillaries are the largest capillaries in the body. They lie in between the layers of choroidal vessels and Bruchs membrane. They are channels lined by endothelium in which the choroidal arterioles end. The endothelium is fenestrated in contrast to iris vessels which are non-fenestrated. The choriocapillaries do not anastamose freely, they supply a globular area rather a sector. The choriocapillaries end at ora and rest of the vessels continue in ciliary body. The choriocapillaries are denser and relatively larger under the macula. The main function of the choriocapillaries is to supply nutrients to the outer part of the retina. 3. Bruchis membrane. The Bruchis membrane is an acellular thin structure that develops from neuro ectoderm as well as uvea. It is thickest around the optic disc. In high magnification, it has five layers. The Bruchis membrane forms the blood retinal barrier and acts a filter for metabolic exchange between choriocapillaries and pigment epithelium of the retina8. The functions of the choroids is to supply blood to the outer retina. Other functions are to keep the interior of the globe dark, to regulate the temperature of the eye and enhance the outflow of aqueous through uveoscleral channel. The choroid does not have any motor function. It is not influenced by parasympathetic nerve. Sympathetic nerve supply regulates choroidal circulation. The choroid is said to act like a lymph node following inflammation. Blood supply of uvea4, 7 Uvea is a vascular structure, vascularity is maximum in the choroid and least in the iris. The blood supply to the eye ball is from the ophthalmic artery which gives two independent system of blood supply. The retinal system and the ciliary system, the two

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generally do not anastomose. The former supplies the inner layer of the retina while the latter supplies the outer layer of the retina, uvea and extra ocular muscles. The ciliary circulation consists ofa posterior ciliary system consisting of long and short posterior ciliary arteries and complex of anterior ciliary arteries. The long posterior ciliary arteries are two in number. They arise from the opthalmic artery in the orbit, either by a common trunk or two separate trunks. These vessels travel forward to reach the posterior part of the globe. They pierce the sclera one on the lateral side and other on the medial side of the optic nerve in the horizontal plane obliquely to enter the suprachoroidal space. They do not give any branch before reaching the posterior part of the ciliary body. They divide into two branches on entering the ciliary body. The two branches give off multiple smaller twigs, most of which go into the substance of the ciliary body to anastomose with the seven anterior ciliary arteries to form the major arterial circle of the iris that lies just behind the root of the iris in the ciliary body. One of the branches from the main artery passes backward to from the recurrent branch of choroids that supplies the choroid between the oraserrata and the equator. The posterior ciliary arteries : They also arise from the ophthalmic artery in the orbit as a group of 6 to 8 vessels that divide to form 20 smaller vessels, which travel forward encircling the optic nerve all around. They are more in number on the lateral side to give more blood to the macula. On reaching the choroid these vessels branch profusely. There are two type of vessels in the choroid, a group of shorter vessels that branch and supply the choroid near their entry into the sclera and a group of longer vessels that travel up to the equator to supply the anterior choroid. The anterior ciliary vessels are continuation of muscular arteries that supply the four recti two for each except the lateral rectus that has only one artery. They also give some branches that are not involved with uveal circulation before perforating the sclera near the limbus and pass through the supraciliary space to end in anterior part of the ciliary body. At this level, they anastomose with the tiny long posterior ciliary arteries to form the major arterial circle. 10 to 12 recurrent branches go backward to supply the anterior choroid. The iris gets its blood supply from the major circle of iris that also supplies part of the ciliary body and by minor circle of iris that lies just inside the pupillary border and is formed by centripetal branches from major circle of the iris. Venous drainage of uvea is mostly by vortex veins. The choroid drains via vortex veins exclusively. The iris and ciliary body have two systems of venous drainage-1. A well developed vortex system and 2. A poorly developed ciliary system. The anterior ciliary venous drainage is more developed than the posterior venous drainage. There are no formed lymphatics in the uvea. Development of the uvea9, 10, 11 The uvea as a whole has bipartite development. The choroid is dominantly mesodermal except the Bruchs membrane which is partly mesodermal and partly ectodermal. The iris and the ciliary body are mostly ectodermal in origin except the stroma and the blood vessels. The ectodermal structure in the iris are the two layers of epithelium, the sphincter, the dilator pupillae and melanocytes.

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The epithelium of the ciliary body and the ciliary processes are also ectodermal in origin. The mesodermal structures in iris and ciliary body arethe iris stroma, blood vessels and ciliary muscles. The iris and the ciliary body develop from the anterior lip of the optic cup. The edges of the optic cup grow in front of the lens as a double row of epithelium behind the mesoderm. The two layers of the iris epithelium become pigmented in ciliary body. The nonpigmented ciliary epithelium is thrown into folds in which the vessels develop to form the ciliary process. The sphincter muscles starts developing earlier than the dilator, they develops from the non-pigmented iris epithelium surrounding the pupil. The dilator muscles develop from the non pigmented layer near the root. The mesoderm that lies in front of the developing iris epithelium forms the stroma of the iris. The growth of the iris is influenced by two factors, i.e. Closure of the embryonic fissure (choroidal fissure) and atrophy of tunica vasculosa lentis. The embryonic (foetal) fissure is a deficiency at the under surface of the optic vesicle and optic stalk extending from the tip of the optic cup almost as far as forebrain. The embryonic fissure is the gap through which the mesoderm surrounding the optic vesicle gets entry into the cavity of the cup and form the retinal and hyaloid system of the vessels. The embryonic fissure starts closing at 10-11 mm. stage at the middle of the fissure and spreads in both directions, the anterior end fuses later than the posterior end. The closure is completed by 18 mm. well before the development of the iris. Failure of the closure of the embryonic fissure results in various congenital anomalies of the uvea ranging from notch coloboma of the iris to extensive coloboma involving all parts of the uvea at the inferior surface of the cup. Non closure of the foetal fissure from end to end leads to formation of colobomatous cystic eye ball. The choroid develops from mesodermal mass at about 6 mm. A network of capillaries that surround the optic cup develop into choroid. The Bruchs membrane is secreted by neural epithelial layer. By third month large and medium choroidal vessels develop. The vortex veins also develop at the same time. Congenital anomalies of the uvea Congenital anomalies may involve all the three parts of uvea or individually iris, ciliary body and choroids in various combination. Close proximity of iris and ciliary body and choroids to retina may involve them as well. Frequently the congenital anomalies of uvea are due to faulty closure of the foetal fissure resulting into colomboma of the uvea of various types. A coloboma is said to be complete when all the layers of iris, ciliary body or choroids are involved and incomplete where instead of full thickness involvement only partial thickness is underdeveloped. A coloboma is called typical when it develops at the site of the closure of foetal fissure i.e. inferior nasal part of the uvea and atypical when coloboma is located in place away from foetal fissure. The coloboma may be unilateral or bilateral when present in both the eyes they are generally symmetrical. colobomas are generally hereditary, involve both sexes equally. Coloboma of the iris Coloboma of the iris may be typical or atypical, can be complete or incomplete. Involvement of the iris in coloboma varies from a small notch at the pupillary border to extensive

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involvement of the uvea from pupillary border to the optic nerve. The typical coloboma is situated at inferionasal part of the iris. It extends from pupillary margin up to the root of the iris, may extend in the ciliary body. The eye may be of normal size but most of the times it is smaller than normal. The pupil is inverted pear shaped or key hole like. If the coloboma extends into the ciliary body, a bluish streak is seen through the conjunctiva. The lower end of the lens is visible with its zonules. If the ciliary body is involved there may be absence of zonules at the site adjacent to the coloboma. There may be localized peripheral lenticular opacity in the lens behind the coloboma. Vision varies from almost normal to severe loss depending upon the degree of involvement of intraocular structures. The eye have variable degrees of errors of refraction. Psudo polycoria denotes full thickness defects in the substance of iris. The defect is generally circular, looking like additional pupil. Such pupil do not have sphincter or dilator muscles or pigments on its border. These pupil does not react to light or accommodation independent of usual pupil. A similar hole near ciliary body is called iridodiastasis12. A coloboma is called bridge coloboma when a strand of iris tissue spans over a coloboma, bridging the gap, this tissue is mesodermal remnant of pupillary membrane. No specific treatment is required for coloboma of iris, however, presence of error of refraction should be managed as and when present to prevent amblyopia. Anomalies of position, size and shape of pupil - Normal pupil is central, circular with slight nasal shift. Position of both the pupil is identical in two eyes. If the pupil is shifted from its normal position it is called ectopia pupil or corectopia . These pupil have their own sphincter and dilator muscles. The two pupil generally do not have symmetrical displacement. The corectopia is generally associated with ectopic lens, the lens generally subluxates away from the decentred pupil. Congenital microcoria or miotic pupil is due to faulty development of sphincter pupillae. A pupil is said to be microcoria if its diameter is less than 2 mm. in distant gaze. Anisocoria When the size of two pupil are different the condition called anisocoria. The two pupils are never of the same size. A change of more than 2 mm. is called clinical anisocoria. Dyscoria is a rare condition where there is congenital abnormality in shape of pupil other than the coloboma. It is generally bilateral. The pupil is generally slit like in presence of light but becomes almost circular in dim light. Aniridia13, 13A, 14 Tough the term aniridia should mean total absence of iris, in clinical practice it is not so. Even in most advance cases, there are always some strands of iris present at the roots. In about 50 percent of cases, they obstruct the trabecular mesh work in later life. These strands are generally detected on 360 degrees gonioscopy.13 The condition is present at birth and is bilateral. There are two types of inheritance, 1. Dominant 2. Sporadic. Besides poor development of the iris the eye has other signs as well. The cornea shows peripheral fine pannus, sensory nystagmus, the pupil is almost as large as the cornea, the lens shows various degrees of opacities on slit lamp examination. The zonules are visible all round and the ciliary process are also seen. The macula is hypo plastic under development of macula is the cause of poor vision and nystagmus.

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Secondary glaucoma develops in the second and third decade of life, most probably due to obstruction of trabeculum by iris tags. There may be other causes of glaucoma than simple obstruction of trabecular mesh work. The glaucoma is difficult to manage and the condition does not respond to medical treatment, surgery fails to reach the desired goals unless prophylactic goniotomy is done before onset of glaucoma.13 Half of the sporadic cases may develop Wilms tumor. All sporadic cases of aniridia should undergo abdominal examination and to examination by ultrasonography on first presentation and repeated yearly for next few years. Presence of Wilms tumor and aniridia is known as Millers syndrome.15 Management is difficult, poor vision should be corrected as far as possible and glaucoma controlled as much as possible. The child may require low vision aid and trained as visually handicapped. Persistent pupillary membrane14, 15, 16 It is an associate anomaly that looks like anomaly of the iris. Persistent pupillary membrane is not a true congenital anomaly of the iris. Persistent pupillary membrane are more common than coloboma of the uvea. It has been reported to be present in 96% of new born, most of which disappears by first year. It persists for few years and then gets absorbed, they are very rare in old age. Persistent pupillary membrane may be unilateral or bilateral and equal in genders. They represent anterior part of tunica vasculosa lentis that supplies nutrition to the lens in foetal life, for the first six month of foetal life and then disappears. Failure of complete disappearance results in shred of mesodermal tissue and obliterated blood vessels that are attached to the collarette. Development of pigment is a post natal feature of the shreds. The strands vary in number and length. They may be gross enough to be seen by oblique illumination without magnification or may be fine enough to be seen by slit lamp. The shreds move freely with the movement of the pupil without restricting it. They normally distort pupillary shape but may be mistaken as posterior synechea which are at pupillary margin. The strands may float freely in the aqueous with one end attachment to the collarette. It may span the pupil and get attached to the opposite collarette or may cross the pupil in segments. Sometimes it gets attached to the anterior lens capsules where a small opacity may be present. It may get attached to the posterior surface of the cornea with a faint opacity. The condition does not hamper vision or cause any complication hence does not require any treatment. Coloboma of ciliary body The isolated congenital coloboma of the ciliary body alone is infrequent. It is generally associated with coloboma of the iris, may be associated with the coloboma of choroids. Commonest congenital anomaly of the ciliary body is typical coloboma due to non-fusion of the foetal fissure in six O clock position. However a typical coloboma may be seen at other parts. Coloboma of the ciliary body is most commonly seen in the microphthalmic eyes. It may be associated with subluxation of the lens due to non-development of zonules at the site of the ciliary coloboma. Colobomata are visualised on indirect opthalmoscopy with scleral indentation. Occasionally it can be seen while doing goneoscopy or ultrasonography. It does not require any treatment.

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Coloboma of choroids can be typical when situated at the site of foetal fissure or atypical when at other than foetal fissure. The former is more common. The coloboma when present are bilateral in two third of the cases. It may extend from the iris to optic nerve and sometimes involving it or it may be localized as a single oval patch in the line of the closure of foetal fissure. There may be more than one such patch in the line of closure of the fissure. Generally a choroidal coloboma has a parabolic appearance with its broader end towards the ciliary body and rounded head towards the optic nerve. The colour of the coloboma is white due to exposed sclera underneath, the edges are clear cut, sometimes these may be irregular. The edges are pigmented. The retina is absent over the coloboma so it is better to call it a retino choroidal coloboma. The retinal vessels are seen to traverse over this coloboma. The scleral bed is generally depressed, in extreme cases it may be ectatic. In rare instances of bridge coloboma, a patch of retinal tissue may cross the coloboma from side to side. Sometimes the edges of the coloboma may reach very near the macula but not involve it. Vision is generally poor. There is corresponding negative scotoma. The diagnosis is straight forward. 1. On retinoscopy a white reflex in the lower part amidst a pink glow is seen. 2. Outer margin of the small coloboma is visible with direct opthalmoscopy. 3. The periphery of large coloboma is seen by indirect opthalmoscope that may show up scleral ectasia. 4. Ultrasonography may also show choroidal defect and scleral ectasia. One of the complications besides sub normal vision is development of rhegmatogenous retinal detachment, when holes may develop at the edge of the coloboma. Coloboma of macula Coloboma of the macula may be considered as modified form of atypical choroidal coloboma. It has also been thought to be dysplasia of macula. It is generally bilateral, it may be very small or larger than the optic nerve. The colobomas are punched out horizontally oval areas with clumps of pigments on the periphery and a white floor representing sclera. The area is devoid of choroidal and retinal tissues, rarely abnormal retinal vessel may traverse the gap. The sclera may be ectatic. Vision is greatly reduced leading to nystagmus, squint and amblyopia. A macular coloboma may be mistaken as part of congenital toxoplasmosis. A large coloboma may give rise to grey reflex on retinoscope. Diagnosis is confirmed by direct and indirect opthomoscopy. An X-ray study is done to exclude intera cranial calcification which is seen in congenital toxoplamosis. Albinism18 Albinism is hereditary disorder due to abnormal metabolism tyrosine hydroxylase resulting in complete or partial absence of pigment in the body. Clinically albinism has been broadly divided into two types, 1. Oculo cutaneous and 2. Ocular. The former can be divided in biochemically into two sub groups i.e. tyrosinase positive and tyrosinase negative. Oculo cutaneous albinism have extensive systemic as well as ocular involvement. The other group known as tyrosinase positive, have some feature in milder degree of oculcutaneous albinism, it is usually autosomal recessive inheritance.

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The ocular albinism is generally X-linked and autosomal recessive in inheritance. This involvement is limited to the globe only. The oculocutaneous albinism produces fair coloured skin that is sensitive to light. Hair all over the body are light coloured including eye brows and eye lashes. In ocular albinism the lids, lashes and eye brows are normal. In both the types the eyes are described as red eye because the pupil looks reddish instead of black and the iris is pale blue. The vision is generally greatly reduced. There is nystagmus and squint. The iris may transilluminate at places but there are no holes in the iris. Asymptomatic female carrier of ocular albinism may also have iris trans illumination.19 The eyes generally have various types of errors of refraction, hypermetropia is more common than other types of refractive error. The fundus looks pale against which the retinal and choroidal blood vessels stand out prominently and albinotic fundus is further divided into two types of i.e. with developed macula and without properly differentiated fovea. The eyes with undeveloped macula have congenital colour blindness. In these cases decussation of optic fibers at optic chiasma has also been found to be defective. Management of albinism is difficult. Vision can rarely be improved, however, full correction should be given to salvage as much of vision as possible, use of tinted glasses reduce glare. Contact lenses are not suitable due to associated nystagmus. Albino children may require low vision aids to pursue studies. Heterochromia of the uvea Heterochromia of the uvea is confined to the iris. It can be unilateral, that is one eye has lighter iris than the other. The iris with lighter shade is abnormal. Unilateral heterochromia of iris is called heterochromia iridum. The eyes with different colours are called heterochromia iridus. The exact cause of congenital heterochromia is not known. Acquired difference is generally due to trauma, inflammation and new growth. There are many types of heterochromia. Simple heterochromia is common without any other ocular or systemic involvement. Sympathetic heterochromia is seen commonly with Horners syndrome. Complicated heterochromia is seen in Fuchs heterochromic cyclitis. Some of the systemic anomalies associated with heterochromia of iris are : Waardenburg syndrome, Rombers syndrome and various types of status dysraphicus. Simple heterochromia is symptomless and does not require any treatment. Other types may require ocular treatment i.e. Fuchs heterochromia cyclitis. Systemic conditions require multi system work up and management. Congenital anomalies of uvea of late onset20 There are some conditions that are thought to be congenital in origin but do not manifest before ten years of age and progress relentlessly towards legal blindness by third or fourth decade. Fortunately they are rare, but neither preventable nor treatable. They are 1. Gyrate atrophy of choroid 2. Choroideremia 3. Choroidal sclerosis.

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One of the suspected causes of Fuchs heterochromic cyclitis is congenital in origin, it can be seen at any age in both the sexes, 10% cases are bilateral. However, some consider it be inflammatory or degenerative and do not put in category of congenital anomalies. Gyrate atrophy of choroid This is an inborn error of aminoacid metabolism. It is associated with raised level of ornithine in plasma, aqueous, CSF and urine. The condition is heriditory bilateral presenting in the first decade as progressive night blindness. There is progressive atrophy of choroid as well as retina. The lesions start in mid periphery as oval patches with scalooped border. The size and shape of the lesion vary, they merge with each other to form a larger patch and girdle the mid periphery. Choroid and retina are absent over the patch but the retinal blood vessels remain normal for long time and then attenuate. Macular involvement is late either as macular edema or a patch may cover the macula. Secondary cataract is common EOG and ERG are sub normal. There is no specific treatment. Constant supplementation of diet with pyridoxine (B6) is supposed to delay the progress. Low protein diet is also recommended. Choroideremia This is a bilateral progressive disease, seen only in males, women are carriers who show mild form of the disease. The condition is first noticed between five to ten years of age as progressive night blindness which is misdiagnosed as vitamin A deficiency by general physician or as retinitis pigmentosa unless fundus is examined. The fundus has no resemblance to retinitis pigmentosa. The two features common among the two conditions are night blindness and ring scotoma in the peripheral field. The disease progresses to legal blindness in ten to twenty years. Central vision is retained till late. The disease is caused due to atrophy of choroid and retinal pigment epithelial. There is no known treatment. Low vision aids and mobility support may help. Ultimately the patient has to be rehabilitated as visually challenged. Choroidal sclerosis Choroidal sclerosis has two forms: 1. Adult and 2. Juvenile. The adult form is probably is degenerative process. The juvenile form is most probably congenital in nature and manifest round about fifteen years of age. The lesion begins on the macula that looks similar to patch of choroiditis. The central vision is poor. There is no specific treatment. Low vision aid and rehabilitation are the only paliatives available. Uveitis in children General consideration The term uveitis means inflammation of any part of the uvea. It is the commonest disorder of the uvea. No age sex or ethnic group is spared. It can be unilateral or bilateral. Due to proximity of lens, retina, vitreous and optic nerve to the uvea, these parts are invariably drawn into the clinical manifestation of uveitis as well as complication. Commonest age group inflicted by uveitis is between second to sixth decade after which incidence of uveitis diminishes rapidly. About five percentage of all uveitis is seen in pediatric age group. Uveitis in children may range between mild self limiting anterior uveitis to sever

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vision threatening endophthalmitis involving all parts of uvea as well vitreous. In one third of all uveitis cases no definite cause can be pinpointed as causative factor. This rises to fifty percent in uveitis in children. Undetected uveitis in children may result in permanent sub normal vision leading to strabismus and amblyopia. Some of the uveitis in children are due to systemic infection and are bilateral i.e. tuberculosis, syphilis, toxoplasmosis. There is no satisfactory way to classify uveitis. Various classifications have been put forward depending upon 1. Parts of the uvea involved. 2. Mode of onset and duration. 3. Etiology. 4. Pathology. 5. Associated with various systemic manifestation. 6. Masquerade syndromes. 1. Anatomical classification (i) Anterior uveitis. This is inflammation localized to iris and or ciliary body. Due to lack of anatomical barrier between iris and ciliary body the inflammation may involve both resulting in to iridocyclitis. Inflammation limited to iris is called iritis while that involving ciliary body (pars plicata) is known as cyclitis. (ii) Intermediate uveitis. Here the process involves posterior part of the ciliary body (parsplana) peripheral choroid and retina and called as parsplanitis. (iii) Posterior uveitis. The part involved is mostly choroid, that may involve the whole of choroid, may be localized to macula, may be near the disc or involve the periphery. Choroiditis invariably involves retina and the condition is called chorioretinitis as seen in tuberculosis syphilis or retinochoroiditis when the primary lesion begins in the retina i.e. toxoplasmosis. (iv) Pan uveitis. All the parts of uvea are involved as in sympathetic ophthalmia. (v) Endophthalmitis. In this sight threatening condition there is pan uveitis with involvement of vitreous, not extending beyond sclera. (vi) Panophthalmitis. Panophthalmitis is the severest form of uveitis involving all coats of eye and periocular tissue. It is a blinding condition. (vii) Keratouveitis. This is a very common form where primary lesion starts in cornea and involvement of anterior uvea is due to spread of toxins to the endothelium. In case of interstitial keratitis the inflammation starts in the uvea and spreads to the cornea. (viii) Juxta papillary22. The lesion is a choroidal inflammatory patch near the disc producing sector shaped lesion and corresponding field defect. (ix) Disseminated choroiditis. Small areas of inflammation are scattered all over the fundus behind the equator. (x) Central choroiditis. It is located in the posterior pole involving the macula. (xi) Sclero uveitis is involvement of uvea along with sclera. 2. Onset (Chronological) On the basis of mode of onset, uveitis can be I. Acute II. Chronic III. Recurrent IV. Acute exacerbation on chronic.

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Etiological classification of uveitis is most logical from point of management but it is not possible to pinpoint the exact cause in about one third cases. Eiologically uveitis can be divided into : (i) Microbial (a) Endogenous. (b) Exogenous. (a) Endogenous microbial uveitis is either caused due to direct invasion of uvea by micro organism or due to immune reaction of the micro organism to the uvea. (b) Exogenous microbial uveitis is due to direct entry of pathogenic organism, following perforation of globe, accidental and surgical or spread from outer coat of the eye mostly as a result perforating corneal ulcer. The organism can be bacteria, viruses, fungi, protozoa and nematodes. All bacteria are capable of producing some type of uveitis or other if they get access to the uvea. They can produce acute or chronic lesions. Acute lesions are generally produced by cocci i.e. gonococcus and pneumococcus. The bacilli generally produce chronic uveitis i.e. tuberculosis, syphilis, leprosy23. Viruses that cause uveitis are herpes simplex, herpes zostor Epistein Barr virus, rubella, influenza, measles and mumps.24 Fungi that produce uveitis are candida, fusarium. Out of many protozoan infection that produces uveitis is Toxoplasma gondi.25 It generally produces bilateral congenital or acquired posterior uveitis. Toxocara canis and catis are two round worms of dogs and cats that produce unilateral posterior uveitis in children that is always vision threatening, but not fatal and must be differentiated for more serious retinoblastoma which is both sight threatening as well as life threatening. Both present with white reflex in pupillary area. Other nematodes that cause uveitis are cysticerosis and onchocercisasis. (ii) Auto immune disease. Auto immune disorders that generally have systemic manifestation form the largest group of diseases that produce both acute and chronic uveitis. They are generally associated with certain specific HLA type. These are juvenile rheumatoid arthritis, ankylosing spondylitis, rheumatoid arthritis, Reiters syndrome, Vogt Koyanagi Harada disease, Behcet disease. HLA typing is now considered an important diagnostic test in uveitis.24 Sympathetic ophthalmia is presumed to be due to hypersensitivity to uveal pigment. Lens protein sensatization cause phacogenic uveitis. (iii) Other non specific uveitis arePars planitis, Fuchs heterochromatic iritis, sarcoidosis, malignant tumor of eye, long standing retinal detachment, iritis glaucomatosa, retained intra ocular foreign bodies specially pure copper, anterior segment ischaemia following squint surgery, pulse less disease also produces uveitis but all are not seen in children.

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4. Pathological classification The pathological classification most widely used was suggested by Woods in 194727,28, that divides uveitis into two groups i.e. granulomatous and non-granulomatous, on the basis of histopathology presentation without sharp line of demarcation in clinical presentation. The granulomatous uveitis generally involves all the part of uvea with predilection for choroids. It is more commonly a bilateral involvement. There is general actual invasion of the uvea by organism. There is a proliferation of tissue. The proliferation of tissue due to microbial invasion depends upon the virulence of the organism and the state of immunity of the tissue. The onset is generally insidious without much pain, the keratic precipitates are large mutton fat in nature, aqueous flare is relatively low with scanty cells. Hypopyon is unusual, the vitreous shows marked flare and cells. The posterior synechia are thick, broad and difficult to break. In late cases peripheral anterior synechia are seen. The iris develops nodules. The condition has long slow course. The non-granulomatous uveitis has an acute painful onset, generally uniocular to begin-with, other eye gets involved later, mostly self limiting. An episode lasts for four to six weeks without treatment. There is marked circumciliary congestion. There may be ciliary tenderness. This may involve all parts but anterior uveitis is more common. KPs are generally small and numerous. There may be fibrinous exudation and formation of hypopyon. Anterior chamber reaction is marked with flare and cells. Vitreous does not show cells, pupil is miotic. Posterior synechia are small and narrow, break with ease. In late stages there may be peripheral anterior synchia. Iris module are generally not formed. Division of uveitis into two classes of granulomatous and non granulomatous is not always clinically possible. A case of acute anterior uveitis of non infectious origin may show large mutton flat KPs or a case of chronic anterior uveitis may show indolent character of granulomatous uveitis. Some of the examples areuveitis due to leptospirosis should cause a granulomatous uveitis but in fact it presents as a non granulomatous uveitis. Similarly lens induced uveitis is expected to be an allergic reaction to lens protein and should behave like non granulomatous which on slit lamp, looks like granulomatous. Similarly sympathetic uveitis also presents as granulomatous lesion with epithelial cells and giant cells when it is supposed to be an allergic reaction. Sarcoidosis is a non infective multi systemic disorder. Logically it should produce non granulomatous uveitis but in most of the eye, it present as granulomatous uvietis with nodule formation. Clinical features of uveitis in general Symptoms and signs in uveitis greatly differ in different types. There may just be blurring of vision without pain or redness as seen in pars planitis, white iritis in girls and Fuchs heterochromic iritis to severe loss of vision with pain as in endophthalmitis. Symptoms of posterior uveitis may differ from those of anterior uveitis in such an extent that they may look like two separate entities. Many conditions may masquerade as uveitis when in fact they are either life threatening serious conditions of intra ocular malignancy or benign condition like persistant primary hyper plastic vitreous. Complications and sequaelae may change this clinical picture entirely.

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It is better to divide clinical features related to following types of uveitis, i.e. those belonging to 1. anterior uveitis, 2. posterior uveitis, 3. intermediate uveitis, 4. pan uveitis in separate groups. Moreover the signs and symptoms differ greatly between acute and chronic uveitis. Complications like glaucoma or cataract may overshadow the primary features of uveitis. Symptoms of acute anterior uveitis 1. Onset. Generally sudden and unilateral but may occasionally be bilateral. Simultaneous onset is extremely rare. 2. Pain. Pain may be the first symptoms of acute anterior uveitis. The pain ranges from dull ache to throbbing pain, radiating along the distribution of ophthalmic branch of fifth nerve on the same side. This pain is worst at night. Tenderness of the globe is indicative of ciliary involvement. 3. Lacrimation and photophobia are common symptoms due to irritation of sensory supply to anterior uvea. They are more marked in children. 4. Redness. The eye may present as red eye due to circum ciliary congestion caused by dilated anterior ciliary vessels. 5. Diminished vision. Initially patient may not have diminished vision. Diminished vision if at all present, develops after three four days, or from the beginning in a case of recurrent attack. Causes of diminished vision areEdema of corneal epithelium, deposition of precipitates on the posterior surface of cornea, turbid aqueous, small constricted pupil, exudates in the papillary area, anterior vitreous cells and flares, spasm of ciliary body resulting in the myopia, secondary glaucoma. Recurrent anterior uveitis is commonly associated with macular edema, sometime there may be papillitis result in diminished vision. Signs of anterior uveitis 1. Mild edema of the lids : Exact cause of such edema is not well understood. 2. Circum corneal congestion. Redness around the cornea starts as a pink hue that becomes gradually darker red. The congestion fades towards periphery. The circum ciliary congestion does not blanch with weak solution of vasoconstrictor. 3. Keratic precipitates: Keratic precipitates are deposition of cells, macrophases, uveal pigments or RBC on the endothelium of cornea. It may be a passive process where cells circulating in the aqueous come in contact with the swollen endothelium and settle on the endothelium or as an active process where the endothelial cells become phagocytic and engulf the circulating cells in the aqueous.27 The large keratic precipitates may be visible on bright oblique illumination with corneal loupe. However, to visualise them in details they should be examined with good illumination of slit lamp. The number, shape, size and distribution depend up on severity and duration of inflammation. Large to medium sized keratic precipitates are generally deposited in a triangular fashion with apex up towards the pupil. Fine KPs are generally dusted all over the endothelium, few KPs may be deposited in an irregular fashion.

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Various types of keratic precipitates according to their size, colour and characteristic are: large mutton fat KP., medium KP, endothelial dusting, pigmented KP and red KP. Mutton fat KP. These are largest KPs arranged in a broad based triangular distribution. The apex of the triangle is upwards, larger KPs occupy lower position. Larger KPs may reach upto 1 mm. in size. They are called mutton fat KPs due to their greasy lardaceous appearance. Mutton fat KPs primarily consists of macrophages and clusters of epithelial cells, they have a sandy white colour, they may at times be pigmented, their number varies between ten to twenty. They are generally seen in so called granulomatous uveitis but may be seen in recurrent non granulomatous uveitis also. Common causes beingTuberculosis, toxoplasmosis, endophthalmitis anaphylectica, sympathetic ophthalmia, sarcoidosis. As inflammation subsidies they become hylanised and fade in colour. Small and medium sized KPs are due to lymphocytes and plasma cells, their borders are clear cut. There may be as many as forty to fifty in one eye. They are seen in nongranulomatous anterior uveitis both acute and chronic. Endothelial dusting is caused by very small KPs that may run into hundreds. They are scattered all over with predilection for lower part, commonly seen in acute nongranulomatous anterior uveitis and recurrence of such conditions. Pigmented KPs. These are seen in chronic inflammation of long duration. They are due to entanglement of iris pigment in white KPs or the iris pigment has been engulfed by the cells. Red KPs are seen in uveitis that cause hyphaema i.e. herpes simplex and herpes zoster. Old KPs. As time passes, KPs shrink in size, fade in colour, these borders become crenated, they have ground glass appearance and may be pigmented. 4. Anterior chamber reaction. Normal aqueous is colourless, clear fluid. In inflammation there is outpouring of protein from the anterior uvea resulting into plasmoid aqueous giving it a turbid appearance. Increased turbidity of aqueous is due to suspended particles : cells fibrin, protein, RBC etc. The cells. In aqueous cells originate in the iris and ciliary body due to active migration from uvea to aqueous humor. The cells may circulate in the aqueous by convection current or settle on the endothelium. They may be seen on the iris, ciliary body, trabeculum lens, suspensory ligament and anterior vitreous face.27 Initially the cells are poly-morphonuclear, as days pass, they are replaced by lymphocytes, plasma cells and macrophages. More are the cells, more is the involvement of ciliary body. The cells are examined by the slit lamp with maximum illumination and magnification by a 3 mm. 1 mm. slit. The cells should be counted and graded between 0 to 4+ where 0 stands for absence of cells and 4+ means over 50 cells per field. Aqueous flare. Presence of protein in the aqueous makes it turbid as beam of light passes through the turbid aqueous the suspended particles stand out as shining bodies against the iris background, this phenomena is called aqueous flare. To see a flare an intense beam of light 2 mm. wide is directed on the iris at an oblique angle and obscuration of details of the iris is noted. Clearer details denote less flare and total obscuration is maximum flare. Flare is again graded between 0 to 4+. Flare in not always a sign of active uveitis, it can be seen due to

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leak of damaged vessels without iridocyclitis. To diagnose uveitis a combination of cells and flare must be present. Fibrenous exudates in the AC. In severe anterior uveitis the cells may get enmeshed in fibrinous aqueous and form a sheet over the iris surface and lens capsule. Presence of fibrinous aqueous is called plastic iritis which is a form of severe iritis. Pus in AC. In severe cases of uveitis there is down powering of WBC, that due to their weight gravitate at the bottom of AC. Accumulation of pus in AC is called hypopyon that is sterile. 5. Iris. The iris gets fluid laden due to dilation of radial vessel and cellular infiltration of the stroma. The iris looses its roughness, the contours of the pits and collarettes are lost. As the iris gets fluid filled and increases in thickness it spreads towards the centre causing shrinkage in pupillary diameter. Nodules on iris are late phenomenon mostly seen in recurrent and chronic uveitis. A swollen peripheral iris may come in contact with posterior surfaces of cornea resulting in peripheral anterior synechia. 6. Pupil. It is miotic which on careful examination looks irregular that reacts poorly to light. Causes of miosis are engorged radial vessels, infiltration of stroma by cells, relatively stronger sphincter muscles and irritative action of toxins on the nerve ending. The pupil becomes irregular due to presence of posterior synechia between the posterior surface of iris and anterior surface of lens in phakic eye. Irregularity of pupil becomes more marked when pupil is dilated by mydriatic. Initially the pupil does not dilate at the site of synechia, but dilates inbetween the synechia. This gives the pupil a scalooped or festooned appearance. With repeated use of mydriatic, the synechia are broken resulting into a round dilated pupil and leaving iris pigment on the anterior lens capsule which lasts for days but disappear ultimately. If the synechia persists for few weeks, but do not break, they require stronger mydriatic cum cycloplegia. Long standing posterior synechia are the site where future anterior capsular opacities may develop. In untreated cases posterior synechia may spread all along the circumference and bind down the pupillary margin all around, resulting in to a ring or annular posterior synechia that prevents aqueous from passing through the pupil resulting in rise of pressure in the posterior chamber that pushes the iris forward in a bow like fashion called the iris bombe. Deposition of exudates that obscures pupillary area is called occlusio pupil with loss of third Purkinje image. 7. Lens. In early stages that lens is clear, later the iris gets adhered to it which on dilatation leave fragments of iris pigment on the lens, varying in number and size. In long standing and recurrent cases anterior capsular cataract develops at the site of posterior synechia. In case of recurrence complicated cataract develops starting from posterior capsule which on slit lamp give a polychromatic lusture. In fibrinous iridocyclitis a membrane similar to occlusio pupil develops behind the posterior capsule and is called posterior cyclitic membrane. 8. Vitreous. Changes in vitreous are common specially when ciliary body in inflamed, the changes are in content and structure. Vitreous involvement is directly proportionate to severity of inflammation. The anterior vitreous can be seen by a bright beam of slit lamp. The changes can be in the form of opacities that are due to cells, coagulated exudates and fibrin. Presence of cells in the anterior vitreous is seen in anterior uveites while that in the posterior

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vitreous is due to inflammation of choroid. A comparison between the cell count in anterior chamber and posterior chamber gives a rough idea differentiating involvement of iris and ciliary body. In case of cyclitis cell density in anterior vitreous is more than in anterior chamber, in case of iritis the finding is reversed. 9. Fundus changes A. The media may look hazy due to presence of KPs, fibrin in pupillary area, posterior capsular opacification and vitreous debris. In case of a small rigid pupil fundus may not be visible. B. The retinal changes can be diffuse, peripheral or macular. Diffuse retinal edema may be present in anterior uveitis. Macular changes are seen mostly as cystoid macular edema (CME), best seen by Goldman three mirror lens or Volks + 68 to + 90D and fluorescein angiography. The macular edema is a self limiting feature but may leave permanent pigmentation. Peripheral changes are seen in pars planitis. C. The disc may be blurred due to optic neuritis, this is more common in cyclitis. 10. Intraocular pressure. Intraocular pressure changes are variable, while in most of the eyes it remains normal, in some cases it may become abnormal in initial few days, especially in children. Lowering of intraocular pressure is more pronounced in cyclitis due to lowered secretion in ciliary process. Intraocular pressure rise in acute anterior uveitis is due to plasmoid aqueous and cells obstructing the trabecular meshwork, trabeculitis, peripheral anterior synechia. Symptoms of Intermediate Uveities24, 27, 30, 31 Intermediate uveitis is also known as pars planitis and chronic cyclitis. The symptoms are floaters, diminished or foggy vision that may worsen over years without any attributable cause. Children complaining of floaters in eye should not be dismissed as simple floaters unless proved other wise. Pars planitis is a disease of children and young adults. In 70% cases it is bilateral. Signs of intermediate uveitis There is hardly any sign visible on oblique illumination the eye is white in most of the cases. There are very few cells in anterior chamber. There may be fine KPs in the lower part, the iris is normal, so is the pupil. The anterior vitreous shows more cells than in aqueous, the posterior vitreous is clear. The real diagnosis lies in the peripheral fundus examined by indirect opthalmoscope and scleral dentation that shows peripheral retinitis, perivasculitis and vitritis. The inferior periphery shows maximum changes in the form of white fluffy exudates as spherical deposits or plane sheat referred to as snowball and snow banking. The macula gets involved in late stages in the cystoid macular edema. Rarely there may be papillitis. Posterior sub capsular cataract is a common late feature. Shrinkage of perivascular membrane may lead to traction detachment. Symptoms of posterior uveitis Posterior uveitis can be acute or chronic. It may be localized either in posterior pole or periphery, may be diffuse, scattered all over the choroids. Retina and optic nerve are frequently involved. The symptoms and signs depend upon the above factors.

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Pain : In contrast to anterior uveitis pain is almost always absent unless associated with anterior segment involvement specially in the form of secondary glaucoma. Lacrimation, photophobia, redness and ciliart tenderness are also absent. Diminished vision : Diminished vision is a prominent feature of posterior uveitis specially if the lesion involves the macula, maculopapillar bundle and optic nerve. Peripheral lesions do not produce much of visual symptoms. They are mostly associated with floaters in the field of vision. Acute posterior uveitis with muscular involvement have deterioration of vision that may be preceded by metamorphopsia. Causes of diminished vision in posterior uveitis consists of simple macular edema, cystoid macular edema, pigmentation of the macula, scar or the macula, vitreous haze, papillitis, lenticular opacity and secondary glaucoma. An unilateral lesion of central choroiditis may present as strabismus and amblyopia in a child. In case of congenital toxoplasmosis loss of vision may be detected only when the child is brought as a case of squint. Signs of posterior uveitis are better divided into 1. Acute and 2. Chronic. Signs of posterior uveitis are mostly localized in the posterior pole, however, whole of the fundus should be scanned with a direct ophthalmoscope as far as possible, followed by indirect opthalmoscopy with scleral indentation. Acute lesion of choroids is a patch of choroiditis seen as an area over which retina is invariably involved, the edema may not obscure the retinal vessel, border of the lesion is well defined. Colour of the area involved is yellowish or grayish. A large area in the posterior pole may give grayish reflex and retinoscopy that disappears from retinoscopy field on movement of the eyeball. The posterior polar lesions are best seen with slit lamp. Vitreous changes are very prominent in acute lesions of the choroids. They are in the form of vitreous floaters, vitreous flare and posterior vitreous detachment. In case of endophthalmitis there may be severe vitreitis with accumulation of exudates. In a case of acute choroiditis, sudden loss of red reflex denotes onset of endophthalmitis. The vitreous opacities may vary in shape and size. They may be fine coarse, large or stringy. Fine opacities are due to inflammatory cells. Large opacities on the periphery and snow ball opacities are seen commonly in pars planitis, candidiasis and sarcoidosis32. The vitreous opacities are graded by number of cells counted in a field by direct opthalmoscope and graded between 0 to 4 +.29 This grading has a disadvantage that it fails to measure the actual inflammatory activity in the vitreous which is best seen by indirect opthalmoscope with intermediate magnification, medium field, with diminished illumination, examined by + 20 D lens.33 Visibility of three fundus landmarks i.e. optic nerve head, retinal blood vessels and retinal nerve layer are noted and graded between 0 to 4+. Zero being clear is view of nerve fibre striation and four plus stands for obscuration of optic nerve head.32 Other changes noted on fundus examination are retinitis, vasculitis, papillitis and exudative retinal detachment. Symptoms of chronic posterior uveitis The symptoms are same as in acute posterior uveitis i.e. there is no pain, redness, photophobia or lacrimation. However, seeing of floaters, scotomas and diminished vision persists.

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Causes of diminished vision in chronic posterior uveitis are Complicated cataract mostly posterior capsular opacification with polychromatic lusture, vitreous haze, vitreous bands, scar over the macula and maculopapillar bundle. Cystoid macular edema, macular pigmentation, macular hole, sub retinal neovascularisation, periphlebitis, secondary glaucoma and traction detachment. Signs of chronic posterior uveitis are same as seen in acute choroiditis with changes due to passage of time. Some of the lesions get healed, other may develop satellite lesion adjacent to healed or healing lesion or there may be development of new lesions independent of original lesion. Vitreous floaters which are hallmark of acute posterior uveitis subsides to a great extent, however, some haze may persist. Posterior vitreous detachment is more common with development of traction bands. A healed patch of choroiditis has an irregular margin with clumps of uveal pigment on the periphery. The retina generally disappears over the healed patch but the vessels persist, there may be clumps of choroidal pigment away from the patch. The floor of the patch is white due visible sclera over which the choroid has been destroyed. Large areas of the choroiditis may result into a scotoma that becomes negative with passage of time. Other fundus changes consists of sheathing of blood vessels, traction detachment, post neuritic optic atrophy, macular scar, membrane formation over macula and neovascularisation. Intraocular tension may be normal or raised. Hypotony is general far less frequent than seen in acute anterior uveitis. Complication in acute anterior uveitis: Common complications are secondary glaucoma, hypotony, massive deposit of KPs on the endothelium, changes in refraction, cystoid macular edema, papillitis, hypopyon and hyphaema. Secondary glaucoma in acute anterior uveitis is generally open angle glaucoma but can be narrow angle as well due to extremely narrow angle that gets blocked by swollen iris at the periphery or due to dilatation of pupil by strong cycloplegic like atropine. Causes of secondary open angle glaucoma in acute anterior uveitis are many that may act separately or in combination, they areObliteration of the trabecular mesh work by swelling of the meshwork in the form of trabeculitis. These already narrowed channel may further be clogged by fibrinous aqueous. Obstruction of aqueous flow from posterior to anterior chambers is caused due to ring synechia, aqueous flow can be hampered due to small pupil, extensive contact of iris over the lens without the formation of actual synechia. Hypotony In initial phase of acute iridocyclitis aqueous secretion by ciliary body is diminished more is the inflammation lower is the tension. This is popularly known as ciliary shutdown, complete stoppage of aqueous production never takes place. The hypotony presents as flattering of cornea, shallowing of AC resulting in increased iridocorneal contact that ends in formation of peripheral anterior synechia. In most of the eyes production of aqueous returns to normal and the tension building up to normal level or may overshoot the normal limit to result in glaucoma. In some cases hypotony persists. The tension may be as low as 6 mm to 7 mm.

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Transient epithelial edema, massive deposition of precipitates on the endothelium that may itself lead to stromal and epithelial haze. Myopia is induced due to irritative spasm of ciliary body, plasmoid aqueous and edema of cornea. This is transient and reversed by use of cycloplegic. Cycloplegic itself can cause iatrogenic loss of accommodation and unmasking of facultative hypermetropia. A school going child following bilateral use of atropine may require near correction for the period of treatment. Loss of accommodation persists for two weeks following stoppage of atropine. Cystoid macular edema and papillitis are common feature of loss of vision, exact causes of these are not well understood most probably they are toxic in nature. Hypopyon : Accumulation of pus in anterior chamber is common in many bacterial uveitis. It is most commonly seen in pneumococcal inflammation. It is sterile in nature. Presence of pus in anterior chamber is an emergency that may be ominous sign of ensuing endophthalmitis. It is also seen in ankylosing spondylitis and Behcets disease. Hyphaema in acute uveitis is seen in cases of gonococcal uveitis and uveitis due to herpes simplex and zoster. Complication of chronic anterior uveitis These complications develop after days or months of unchecked uveal inflammation. They can be grouped in following categories : 1. 2. 3. 4. 5. 6. 7. Keratopathy. Iris and pupillary changes. Altered aqueous dynamics. Lenticular changes. Vitreous changes. Fundus changes. Amblyopia and squint in children.

Keratopathy in chronic anterior uveitis Involvement of cornea in long standing anterior uveitis in inevitable due to direct continuation of corneal endothelium and iris endothelium. The commonest chronic form of keratopathy in anterior uveitis is band kerotopathy (See page 214) which develops on the cornea like a horizontal band in the inter palpebral area. It is due to the deposition of calcium carbonate and calcium phosphate in the Bowmans membrane and anterior stroma. The calcium deposited in band keratopathy is extra cellular. The epithelium in the initial stage is normal with intact corneal sensation. On long run the epithelium may become irregular and stains with fluorescein giving a fake impression of inflammatory keratitis. The exact cause of band keratopathy is not known, especially why it should have predilection for central cornea in a horizontal fashion and its more frequent occurrence in children than in senile age group. It is generally seen in badly damaged eyes of long duration. Generally there is grave lose of vision however there may be sufficient salvageable vision. There seems to be an unanimity in the theory that it is a degenerative condition, other hypothesis put forward are that its deposition is influenced by actinic rays, resulting into a chemical change between cornea and air causing

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a low CO2 and low pH in the cornea.27 The corneal hemidesmosome may act as a lattice over which calcium crystals are built.35 Children are more capable of mobilizing calcium than adults. The condition starts as faint vertical line on each cornea 1 mm inside the limbus in the inter palpebral zone and gradually spreads towards the center. The progress is very slow, it may take one to two years for the two ends to meet in front of the pupil. May be slightly below the center of the cornea. The band is wider in the mid cornea. A rare type begins in the center and spreads towards the periphery. The band is white colour with a few deficiencies in between in circular fashion, these deficiencies represents the entry of the corneal nerves. In late stages the cracks appears in the band. The overlying epithelial is not invaded for a long time but ultimately the crystals protrode through the epithelium making it susceptible to infection. The calcium crystals can be scrapped off the Bowmans membrane leaving a clear stroma. Ocular causes of band keratopathy are : chronic anterior and pan uveitis in children, chronic uveitis in young adults phthis bulbae, trauma, absolute glaucoma, failed keratoplasty, anterior segment ischaemia.37 Symptoms Band keratopathy is almost symptomless except gradual diminished vision. Diminished vision is commonest symptoms which the patient attributes to underlying ocular disease. Sometimes there may be recurrent redness, watering and photophobia. Signs Signs consists of horizontally placed sub-epithelial calcarious opacity with holes in between. There is always signs of primary disease i.e. chronic uveitis, glaucoma. Management of band karatpathy is difficult. It is presumed that an early and satisfactory treatment of underlying ocular pathology wards-off band keratopathy. There are two possible modes of treatment, 1. Lamellar corneal graft and 2. Medical treatment. Medical treatment is an outdoor procedure where under local anaesthesia the epithelium is scrapped off the band. The calcarious deposits is rubbed with sodium EDTA 0.5% for about 10 minutes. This should remove all the deposits, if it fails strength of EDTA is increased to 1% and rubbed for 20 minutes, the eye is bandaged with antibiotic and cycloplegic till the cornea is reepithelised. Recently excimer photo keratotomy has given encouraging results.38 Iris changes in chronic anterior uveitis are generally associated with changes in pupil. Common iris changes are formation of nodules, posterior synechia, peripheral anterior synechia, iris atrophy, increased translucency of iris and neovascularisation. Iris nodules : There are many types of nodules that develop on the iris which fade away with treatment, some take just few days to disappear, others may linger for longer duration. Common nodules are Keoppe nodule, Busaccas nodule, tubercular, syphilitic, lepromatous nodules and nodules of sarcoid.27, 32, 39 Keoppe nodules are equivalent of mutton fat KPs. They are seen on the pupillary margin and may project in the pupil. They develop from ectodermal tissue, vary in number, not exceeding more than fifteen in each eye. They are accumulation of large epitheloid cells and lymphocytes. They were thought to be diagnostic feature of granulomatous uveitis, however, they are metwith in both types of uveitis. Generally posterior synechia develops at the site where this nodule comes in contact with the lens capsules.

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Busaccas nodules : These nodules are less common, fewer and smaller than Koeppes nodule. They develop from mesodermal tissue at the periphery of the iris, around the collarette. They take weeks to months to disappear. They may get hyalinised on long run, they are not diagnostic of any specific disease. However they may be allergic in nature. Tubercular nodule : Prior to the advent of anti tubercular drugs tuberculosis was thought to be commonest cause of endogenous both anterior and posterior uveitis. With available effective anti tubucular chemotherapy incidence of tubercular uveitis have come down to a large extent still it remains an important cause of uveitis in developing countries in all ages. The tubucular nodules are scattered throughout the iris and ciliary body mostly in the stroma. They are more common in the pupillary margin, they vary in size from 1 mm. to 1.5 mm. They are grayish yellow in colour some times new vessels develop round the base of the nodule. Incidence of nodules is greatly reduced by systemic chemotherapy. Large single nodule has predilection for iris root. They disappear with systematic chemotherapy and local steroids. Syphilitic nodules40 : The syphilitic nodules are unilateral even in case of bilateral involvement, only one eye develops nodules and can be considered as papule. They are generally located near the pupillary margin, size and number vary. The nodules are generally hyperemic. Like other nodules the syphilitic nodules disappear with treatment, sometimes leaving a patch of atrophy behind. In rare instances one of the nodules may be converted in to a gumma. Hyperemia of iris in syphilitic iridocyclitis is known as roseola. Sacroid nodule : Iritis is most frequent ocular manifestation of sarcoidosis. The nodules of sarcoid look similar to that of tubercular nodule. They may be seen on the pupillary margin or in the stroma of the iris. They are more hyperemic and vascularised than tubercular nodules. The vessels form a lattice like pattern over the nodule. There are many conditions that may mimic iris nodule. Some of them may be benign not requiring any treatment. Posterior synechia in chronic uveitis are generally broad and difficult to break when dilated with strong cycloplegic. These pupil take a festooned appearance. It is directly related to amount of fibrin present in the aqueous. They are generally initiated by Koeppe nodules. Long standing posterior synechia may start anterior capsular opacification. If the posterior synechia involves the whole periphery of the pupillary border it is called seclusio pupillae. In severe cases of whole of this posterior surface of iris can get plastered on the anterior lens capsule that does not separate with maximum permissible cycloplegic drugs. An annular posterior synechia prevents the posterior chamber from communicating with the anterior chamber via pupil, resulting in forward bowing of the iris causing the anterior chamber to be deep in the center and shallow at the maximum bulge, such condition is called iris bombe, the periphery of the iris may touch posterior surface of the cornea resulting into peripheral anterior synechia. Peripheral anterior synechia may result even in absence of iris bombe i.e. due to prolonged contact between edematous iris and corneal endothelium at the periphery. Prolonged dilatation of pupil and nodules on the periphery may obstruct an anatomically narrow angle causing rise of tension. The peripheral anterior synechia may obstruct the trabecular mesh work and form a barrier between the anterior chamber and the trabeculum such a condition is called pseudo angle formation.

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The exudates may be deposited in front of the lens as a white membrane in the pupillary area. The membrane may be dusted by iris pigment obscuring the third Purkinge image making it difficult to evaluate transparency of the lens, this condition is known as occlusio pupillae. Iris atrophy In some types of anterior uveitis especially in herpes zoster and Fuchs heterochromic iritis patches of atrophy develop. In herpes vesicles develops on iris which on healing leaves white areas on the iris. These are areas of hyalinised scars from where pigment is removed. These patches may have sectorial removal of pigment, this accentuates already distorted pupil. The pupil adjacent to the patches of iris atrophy does not react to light or reacts weakly. Fluorescein angiography of iris in healed herpes zoster show occuluded iris vessels at the site of atrophy. In Fuchs heterochromic iridocyclitis patches of atrophy are most marked at the pupillary margin. This is a non-inflammatory process. Areas of atrophic patch of full thickness transmit reflected light of retinoscope and retro illumination. Other condition that allows passage of reflected light through iris areiridectomics, iridotomies, essential iris atrphy and polycoria. Neovascularisation of iris is a rare phenomenon in iridocyclites, it is seen in some chronic anterior uveitis, generally at the site of previous nodule. It can be seen anywhere but is more common at the collarette. New vessels lead to formation of small patches of haemorrhage or may cause frank hyphaema.29, 32 Rubeosis of iris is due to chronic iridocyclitis. It should be differentiated from other causes of neo vascularisation of iris and ciliary body which in adults, are central vein thrombosis, diabetic retinopathy, sickle cell retinopathy, and Coats disease. Altered aqueous dynamics in chronic uveitis: Glaucoma. Formation and flow of aqueous may be normal, maintaining normal intra ocular pressure in majority of cases of chronic anterior uveitis. There is a rise of intra ocular pressure, which may prove to be the important missed cause of loss of vision. Glaucoma thus developed is generally chronic secondary open angle. Sometimes there may be acute rise of tension in eyes that are predisposed to angle closure due to crowding of iris at the periphery following use to long acting cycloplegic. Causes of secondary glaucoma are multiple, which may act separately or in combination. They areInflammatory edema of trabecular meshwork that reduce the diameter of the pore, making it difficult for the plasmoid aqueous to pass through. The outflow is also hampered due to fibrosis and hyalin deposits in the mesh work. Peripheral anterior synechia may plaster the face of the meshwork to form a pseudo angle in front of the original angle. In case of anatomically narrow angle swollen peripheral iris may block the entrance to trabecular meshwork, this is worsened by the use of long acting cycloplegic in anterior uveitis of long duration. There may be neovascularisation of angle. A seclusio pupil may cause not only pupillary block but also angle block. Long term use of cortico steroid locally or systemically in genetically predisposed eyes is a common cause of rise of intra ocular tension, which generally comes down following stoppage of the steroid. Chronic swelling of ciliary body can cause forward rotation of lens iris resulting in angle closure glaucoma. Miotics are contra indicated in all

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types of uveitis as they worsens acute iridocyclitis, reactivate chronic uveitis and results in to pupillary block glaucoma. Glaucoma should be suspected in all cases of anterior uveitis, hence it should be a practice to measure intra ocular pressure not only on first visit but also on subsequent visits also. A reduction of vision with normal macula and no evidence of papillitis should always warn about possibility of glaucoma, specially if the patient is on the steroid or cycloplegic. The tension should be brought down by the use of betablockers, alpha agonist, carbonic anhydrase inhibitors. Latanoprost which is an anti prostglandin drug that increases uveo scleral out flow is better avoided because it can reactivate uveitis. Short term systemic carbonic anhydrase inhibitors may be used to bring down the tension fast. Patients not responding to medical therapy are subject to standard glaucoma surgery. Hypotony Reduction in intra ocular tension is common in initial stages of acute anterior uveitis due to hypo secretion of aqueous. This is a self limiting condition, after a few days aqueous production is resumed and intra ocular tension returns to normal or may rise to cause secondary glaucoma, however some times, this hypotony persists for a long time or may develop after few weeks. It is common is patient with juvenile rheumatoid arthritis. Intra ocular tension as low as 6.5 mm is well tolerated by the eyes without any immediate deleterious effect. Prolonged hypotony leads to shallowing of anterior chamber that brings peripheral iris in contact with cornea resulting in formation of peripheral anterior synechia that may cause pseudo angle closure glaucoma. A narrow inter palpebral fissure with reduced corneal curvature and shallow anterior chamber should arouse suspicion of hypotony. There is no specific treatment cycloplegic and steroid are continued. If the patient is on oral carbonic anhydrase inhibitor or local anti glaucoma drops they are withdrawn. Prolonged hypotony over months may results in total ciliary shutdown and result in phthisis bulbae. Lenticular changes in chronic anterior uveitis Lenticular complications are common aftermath of chronic uveitis of all types, anterior, posterior, intermediate and pan uveitis. Cause of cataract in uveitis is not well understood, it is mostly due to altered bio-chemistry of aqueous, vitreous or lens in various proportion. Cataract formation is more common in cyclitis than in posterior uveitis. It takes months to years for lenticular opacity to develop. The opacity is mostly posterior subcapsular in the form of polychromatic lusture. The cortical fibre in front of this may also be involved, the nucleus becomes yellowish. The anterior capsule also develops sub capsular opacity later, ultimately whole of the lens is opacified. Broad posterior synechias that fail to break develop opacities under the synechia and just ahead of the synechia, these do not progress. Deposition of exudates in occulsio pupilli may be confused as opacity in the lens without a true cataract formation. In prolonged cyclitis the zonules may give way resulting in subluxation or complete dislocation either spontaneously or with minimal trauma. A common cause of posterior capsular opacity is prolonged use of steroid both local and systemic. Vitreous changes in chronic anterior uveitis Vitreous changes in chronic uveitis is less common. The vitreous may develop floaters and liquefaction. Chorioretinitis and vasculitis may result in formation of vitreous bands

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leading to rhegmatogenous retinal detachment. Posterior vitreous detachment is more common than detachment from the base. Fundus changes consists of : Macular edema, macular pigmentation papillitis, peripheral retinal degeneration, peripheral vaseulitis and chronic papillitis or post neuritic optic atrophy. Patches of chroiditis and chorioretinitis may be present. Complications of posterior uveitis consists of loss of vision, scattered scotomas in the field, exudative retinal detachment, neovascularisation, traction detachement complicated cataract, vitreous detachment and liquefaction, optic neuritis and optic atrophy, glaucoma, hypotony, phthisis. Pan uveitis Involvement of all the parts of uvea in an inflammatory process24 is called pan uveitis. All the parts may not be equally involved. Generally pan uveitis is discussed under chronic uveitis, however, it can have both acute and chronic onset with or without recurrence depending upon the etiology. It is generally bilateral, one eye may be involved earlier than the other. Clinical presentation in the eye vary according to etiology, duration and treatment undertaken. The signs and symptoms of pan uveitis are a combination of both anterior and posterior uveitis. It can be bacterial or viral. Common bacteria are tuberculosis, syphilis, brucellosis and lyme disease. The viruses involved in pan uveitis are herpes zoster and simplex, rubella, rubeolla, Aids, Epistein-Barr virus. Commonest parasite causing pan uveitis is toxoplasmosis. Conditions that have uncertain etiology areSympathelic ophthalmia, parsplanitis, Vogt Koyanagi Harada syndrome, sarcoidosis and Behcets disease. The course tends to be chronic with fair prognosis. Endophthalmitis Endophthalmitis is a severe form of pan uveitis with involvement of vitreous, retina and optic nerve. The inflammation is always intra ocular. The organism may reach the eye via the blood stream, may spread from sloughing corneal ulcer. Commonest source of infection is penetrating injury either accidental or surgical. Endophthalmitis can be acute or chronic, it may be infective or sterile. Among infective organism, bacteria are the commonest causative factors, which invariably produces acute endophthalmitis while fungal endophthalmitis develops late and progresses slowly. Viral endophthalmitis is unknown. Panophthalmitis Panophthalmitis is an acute superative inflammation of eye as well as periocular structures basically it is an endophthalmitis that crosses the scleral barrier. It is generally unilateral. With profound loss of vision, pain, copious discharge, edema of lids, proptosis, changes of conjunctivitis or sloughed hazy cornea, massive hypopyon and restricted movement. Management of uveitis Management of uveitis may be very simple with recovery without recurrence or complications. Otherwise it may be frustrating, long drawn with complications. Treatment depends upon its onset, severity, part of the uvea involved, probable cause of uveitis, associated complication, tolerance of the patient towards therapy and compliance.

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Aim of the treatment for acute anterior uveitis is to 1. Reduce discomfort i.e. pain, lacrimation, photophobia. 2. Bring inflammation under control. 3. Eliminate infective process, when detected. 4. Prevent and treat complication as and when they arise. Treatment of acute uveitis is divided into non-specific and specific treatment. Non specific can be 1. Physical : Hot fomentation. This gives some relief from pain in the eye. Dry fomentation is better as it is convenient to apply. The eye is fomented for three to five minutes at a time for two to three times a day. Dark glasses : In initial stages it helps in reducing photophobia, once the pupil is dilated it reduces glare. 2. Medical treatment of acute anterior uveitis consists of Cycloplegic, cortico steroid, non steroidal anti inflammatory drug, immuno suppressive drugs. Specific antibiotic for tuberculosis, syphilis, toxoplasmosis etc. Cycloplegics : Purpose of cycloplegic is to relieve spasm of ciliary body which is a major cause of pain in and around the eye. All cycloplegics are mydriatics. Instillation of cycloplegic also causes much needed mydriasis that keeps the pupil dilated, prevents post synechia, breaks posterior synechia. Mydriatics themselves do not have any therapeutic value in treatment of uveitis, they may be added to cycloplegics to enchance mydiratic effect of the later. Cycloplegics are parasympatholytic agents they are of two types : 1. Long actingAtropine. 2. Short actingHome atropine, cyclopentolate and tropicamide. Action of these drugs are not neutralized by miotics in therapeutic dose. The cyloplegics have local as well as systemic action especially with long acting drug i.e. atropine. Cycloplegics are used as drops, ointment and subconjunctival injections. As drops they may be used singly or in combination of two cycloplegics or a cycloplegic with mydriatic. Commonest combination is that of tropicamide, cyclopentolate with phenyle pherine. Out of all cycloplegics only atropine is available in ointment and injectable form. Cycloplegics are metabolized faster in inflamed uvea, hence their action passes off faster in uveitis and require more frequent instillation. Atropine is strongest of all cycloplegic, it is an alkaloid. Its action starts within one hour and lasts ten to fifteen days in normal eye. In inflamed eye the action starts late and for shorter period, requiring frequent use for long period. Some times increase in concentration may be required to produce desired therapeutic results. Atropine is used as sulphate either as ointment or drops. Commonly used concentration is half to one percent as ointment and one to two percent as drops. Concentration of more than two percent is hazardous in children. Drops should be avoided because each moderate sized drop contains 0.6 mg. of atropine, which is within therapeutic limit, this means that two drops contain 1.2 mg that is more than upper limit of safety. Similarly each drop of two percent will contain 1.2 mg and such two drops when absorbed through conjunctiva and nasolacrimal duct will produce toxicity. Absorption through

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nasolacrimal duct can be minimized if the punctum is obliterated by finger for one minute just following instillation. Common side effect of atropine areGlare, loss of accommodation, unmasking of facultative hypermetropia, dryness of mouth, flushing of face, thirst, rise of body temperature, constipation, in eyes with anatomically narrow angle atropine can precipitate angle closure glaucoma, which is not responsive to local miotics. Fortunately angle closure glaucoma following mydriasis is rare in children. In case there is rise of tension it should be brought down by systemic acetazolamide, local beta blockers or alpha agonist. Atropine is one of the local drugs that is frequent cause of dermatitis medicamentosa. Fortunately this passes off within a few days after stopping the drug. The patient may require local steroid ointment to treat allergic blepharitis and dermatitis of the lids. If pupil does not dilate with BD dose, it is better to add five percent phenyl pherine, rather than increasing strength and frequency of drops in children. In case of allergy to atropine, the drug should be withdrawn and replaced by home atropine hydrobromide 2% drops or cyclopentolate one percent. However many of the patients allergic to atropine are also allergic to home atropine. Similarly cyclo pentolate is known to aggravate iritis. To summarise Avoid atropine drops in children. Inform parents about possible side effect of atropine. Once synechia are broken maintain mydriasis with other cycloplegic or combination of cycloplegic and mydriatic drugs. Sympathomimetic drugs have no role in uveitis except as adjuvant to cycloplegics. They may release uveal pigment in aqueous that may be mistaken as flare. In case of glaucoma control tension by acetozolamide, beta blockers and alpha agonist. Latanoprost and cyclopentolate may aggravate uveitis. Miotics are contra indicated in uveitis and do not counteract cycloplegic action. They also do not counteract drug induced mydriatic in therapeutic dose. If pupil does not dilate with atropine BD or combination of tropicamide / cyclopentolate / home atropine with phenylpherine, every half an hour for four to five instillation, the pupil will most probably not dilate with subconjunctival injection of mydricane that is a combination of atropine, epinephrine and procaine/xylocaine. Cortico steroids Most commonly used drugs in uveitis is steroid. Steroids are effective in all types of uveitis, are easy to be administered, evaluate and adjust their dose. Steroid are very cost effective provided they are administered as per norms otherwise they can be potentially dangerous both locally and or systemic. Exact mode of action of steroid is not well understood, most widely accepted theory is that they act as potent anti inflammatory agent. They do not change the basic pathology. The steroids protect the tissue from the onslaught of inflammation till the immune system is capable enough to overcome inflammation by reducing phagocytes,

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fibrosis and neovascularisation24. As steroid has no effect on micro organism they should be used under umbrella of anti microbial drugs other wise the condition may worsen. Steroids can be divided into two groups i.e. long acting and short acting. Both groups are available as local drops and ointments, tablets and injectable forms. The drops can be clear solution or suspension. The clear solution have better corneal penetration hence are preferred over suspension. Injectable solutions are also available in soluble state or suspension form. The solution are generally short acting i.e. dexamethasone or betamethasone, which are absorbed quickly requiring repeated injection every day or alternate day till the inflammation has been brought under control. The suspensions are absorbed slowly from the site, they form depot from which the drug is released. The depot may remain visible under the conjunctiva as long as 20 to 25 days but its therapeutic efficacy is considerably reduced by seven to ten days. The injection may be repeated every fortnight. If the condition requires prolonged use of steroid they may be given every month. Depot injections are suitable for chronic cases. They are not to be used in acute states. Injectable steroids can be given as sub conjunctival, anterior and posterior subTenon and retrobulbar. They may be give intra muscular or IV as well. Commonly used IV steroid are methylprednisone and ACTH. Subconjunctival and subtenon injections are absorbed by conjunctiva which passes it over the aqueous or via sclera. A part of subconjunctival injection is bound to absorbed by systemic circulation. SubTenon and retrobulbar injections are given for lesions behind the lens and suspensory ligament. Retrobulbar injection is given less frequently mostly for optic nerve involvement and causes less rise of IOP, cataract is also less frequent following retrobulbar injection. The tablets can be given daily or alternate day or in pulse form. The daily dose can be given in divided dose of TDS or QID or as a single dose in the morning just before breakfast. The divided dose is more effective but produce more side effects. Both short and long acting drug can be used. Daily single dose or alternative dose reduces adrenalin suppression, hence they cause less side effects. Short acting steroids like dexamethasone or betamethasone are not suitable for alternate day schedule as steroid in therapeutic dose is not available on the skipped day. The commonest drug used for daily and alternate day regime is prednisolone in a dose of 1 mg to 1.5 mg per kg. In an adult this may be as high as 60 mg to 120 mg. per day. Children are put on steroid in consultation with pediatrician. The steroid should never be stopped abruptly. They are generally tapered over weeks or months. General principles of use of steroid are : Use enough, soon enough, often enough and long enough. The above dictum is true for systemic and local steroids. Local steroids Local steroids are available as ointment as well as drops. Ointments are poorly absorbed, they form an oily layer over cornea that reduces vision and interferes with examination by slit lamp or opthalmoscope. The advantage of ointment is that it has prolonged effect hence can be used during sleep. The drops are available as clear solution or suspension. The frequency of instillation depend upon severity of uveitis. In acute iridocyclitis they may be used initially every hour for first 6 hours followed by seven to eight times a day and gradually tapered to twice or once a

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day. The steroids can be tapered not only in frequency but also in strength. They can be diluted as much as one tenth, not loosing much effectiveness when used for long time. Patients on systemic steroids require less local steroid. It is better to use a broad spectrum antibiotic drop along with local or systematic steroid to ward off possible secondary infection. Such prophylactic use of antiviral and antifungal drugs have doubtful role. It has been observed that eye under steroid therapy requires less cycloplegic to dilate the pupil. Side effect of steroids: Steroids are most potent anti inflammatory agents with a very wide spectrum utility, as expected such potent drugs have far reaching side effects both local as well as systemic. Local and periocular use of steroids produce less side effects but are not altogether free from them. Local steroids are capable of producing corneal complication and rise of intraocular tension like systemic used steroid, they may even produce systemic side effect to less extent when used for a long time. Local complication include delayed healing of both post traumatic and infective process. Reactivation of dormant infective process, corneal staining, rise of intra ocular pressure, moderate mydriasis, posterior subcapsular opacity and ptosis. Systemic use produce all above side effects along with following in various combination : Precipitate latent diabetes in steroid responders, worsens existing diabetes, salt retention leading to weight gain, edema, hypertension. There is potassium loss, generilsed weakness, ptosis, personality change, sleep disturbance, Cushing syndrome, acne and poor healing. Non steroidal anti inflammatory drugs: These drugs are mostly prostaglandin inhibitors. They block the synthesis of enzyme prostaglandinsynthatase and cycloxygenase. They also block polypeptide of kinin system, lysosmal enzyme, lymphokinase. They are available as local drops, ointments, oral tablets and injection. They include age-old analgesic like salicylate, aspirin, indomethacine and recent drugs like ibuprofen, diclofenac sodium ad potassium, nimesulide, ketarolac etc. Newer drugs are being added frequently claming to be more effective and less toxic. Local drops are usually used twice a day. Their use is restricted to mild to moderate anterior uveitis, their simultaneous use with steroid results in lowering requirement of steroid. They do not replace steroid altogether. They can be used to prevent recurrence for long time. They hardly produce any ocular side effect. They antagonise prostaglandin induced miosis which may otherwise be atropine resistant. They also antagonise vasodilatation, and reduce aqueous flare. They are used to enhance preoperative mydriasis and maintain intraoperative mydriasis. They are also used in cystoid macular edema. All NASID are potent analgesic and moderate antipyretic. Systematic side effects produce by NSAID arehyper acidity and peptic ulceration. Systemic allergy has been reported. Anti metabolites These potentially dangerous drugs are used in following conditions: Bilateral vision threatening condition not responding to permissible dose of steroid or where steroids are not tolerated. The patient should follow the instructions and agree for adequate follow-up and have a knowledge of side effects of antimetabolites. There should be no contra indication of their use like tuberculosis, toxoplasmosis, fungal infecton, herpetic disease, cytomegalo virus infection.

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The drug should always be prescribed under supervision of oncologist. Their benefit should always be weighed against their potential dangers. All of them produce cytopenaea and liver toxicity. The dose used in ophthalmology is comparatively low. Hence side effects encountered in ophthalmic use are fewer in comparison to malignancy. They are mostly used in sympathetic ophthalmia, Vogt-Koyanagi- Harada disease, Baheets disease and serpiginous choroditis. They may be used in parsplanitis, chronic cyclitis, retinal vasculitis. Their role in childhood paraplanitis is questionable. Cytotoxic drugs are not available as drops or ointment commonly used drugs are cyclophosphamide, cyclosporin, chlorambucil, azathioprine, methotrexate. Cyclosporine does not come under true antimetabolite, it is basically an antibiotic. Combination of steroid with cyclosporin reduces total dose of cyclosporin which is available as drops. Specific type of uveitis in children: While pediatric population comprises of 15% of general population, incidence of pedriatric uveitis ranges between 2 to 10 percent27, 42, average being 5%. These figures are definitely less when compared to adult uveitis, however, it is important not to miss uveitis in children because it can be bilaterally sight threatening. Some of the peculiarities of pediatric uveitis are : 1. Incidence of bilateral uveitis in children is more than adult uveitis. 2. Unilateral chronic uveitis (white uveitis syndrome) may go undetected. 3. Girls are effected more than boys in a ratio of 6:4 except sympathetic uveitis where boys outnumber girls. 4. Endogenous uveitis is commonest form of childhood uveitis. 5. Acute anterior uveitis is less frequent than chronic recurrent uveitis. 6. One third of childhood uveitis have undetermined etiology. 7. Prognois is poor due to chronicity, uncertain etiology, late diagnosis, poor response and poor compliance. 8. It can be congenital or acquired. Though all adult form of uveitis can inflict patients under fifteen, some are seen less commonly in children. Pediatric uveitis can be: CongenitalToxoplasmosis, rubella, herpes simplex syphilis. Acquired 1. Infective : Generally chronic but may be acute. They may be due to tuberculosis, syphilis, streptococcal, gonococcal. Other organism causing uveitis are pneumococcus, staphylococcus, bacillus cereus, leprosy (rare in children), leptospirosis, propioni bacterium, lyme disease, various fungi, toxoplasmosis, toxocara, herpessimplex herpeszoster, aids. 2. Connective tissue diseases effecting joints43 : Juvenile rheumatoid arthritis, juvenile Reiters syndrome, juvenile spondylitis, inflammatory bowel disease.

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3. Trauma : Sympathetic ophthalmia, sensitization to lens protein following rupture of lens capsule, retained in intraocular foreign body. 4. Others : Vogt Koyanagi - Harada syndrome, Behcets syndrome, sarcoidosis, Fuchs heterochronic uveitis. All these are very infrequently seen in children. All except Fuchs heterochronic iritis generally produce bilateral chronic pan uveitis. Pars planitis is common in children that produce intermediate uveitis. Other causes are ischeamic ocular inflammation following squint and retinal surgery. 5. Masquerade syndromes : These disorders are mostly non inflammatory in nature diagnosed as chronic idiopathetic uveitis. They can be life threatening malignant lesions or non malignant lesions in children. Common examples areRetinoblastoma leukemia, medulo epithelioma, intraocular foreign body, Coats disease, peripheral retinal detachment. Toxoplamosis uveitis Toxoplasmosis has emerged as commonest cause of posterior uveitis. It is caused by protozoa, toxoplasma gondi that is an obligatory, intra cellular parasite. Cat is the first definite host and humans are the intermediate hosts. In cat it is an intestinal parasite that reproduces sexually in the gut and is shed in stool as oocytes which infect the human being by ingestion of food contaminated by oocytes. The food may be directly infected by the dirt containing the parasite or may be transmitted to exposed food by vectors like house fly and cockroach.Toxoplasma can be transmitted via unpasteurised milk, half cooked meat. It is also transmitted by way of inhalation, through blood transfusion, skin wounds and transplants. Once it reaches human intestine it multiplies there and pass to the regional lymph nodes. From the lymph nodes they get circulated in regional circulation44, 45 In intermediate host toxoplasma multiplies within, asexually and form a protective cyst within the cells where the cyst remains protected from the immune system hence dormant occasionally. So long as the organism is intracellular it does not give a positive serological test. Titer of serological test is not related to ocular involvement, however, a rising titer is indicative of recurrence that is very common. Toxoplasmosis occurs in two forms i.e. acquired that is very mild and self limiting does not require any treatment and gives immunity to reinfection, but recurrence due to rupture of toxoplasma cyst is possible. However a pregnant mother who is infected during pregnancy can transmit the infection to the growing foetus. The foetus is infected via plancental blood or by direct extension from a dormant lesion on the uterine wall. A mother who is sero positive for toxoplasma before first pregnancy will not infect the foetus nor the subsequent foetuses. Thus only one of the pregnancies is at risk. There is no prophylaxis against toxoplasma. Congenital toxoplasmosis Once it was thought that all ocular and brain lesions of toxoplasmosis were congenital and consequently lesion as recurrences. However now it is known that these lesions can be acquired also. The congenital lesions have predilection for brain and retina. In the brain it produces encephalitis, the patches of which get calcified in long term. In eye the lesion are patches of retinochoroiditis in the posterior pole on the macula, close enough to the macula to cause loss of vision or juxta papillary. Scattered smaller patches away from the posterior pole without visual symptoms, these lesions are detected only on routine fundus examination by indirect ophthalmoscope.

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All lesions are generally bilateral : The lesions starting at early pregnancy may heal and the child born with a patch of healed choroiditis. The healed patch is not noticed unless looked for particularly. A healed patch is oval in shape may be larger than disc, has patches of pigment on the periphery. The center of the patch is white. The blood vessels pass over the patch. There are no cells in the vitreous or aqueous. A child infected late in pregnancy has active lesion in the posterior pole with fluffy appearance, the retina is edematous. There are no pigments on the periphery, vitreous may have cells. A child with congenital toxoplasmosis has three Cs representing Chorioretinitis, Calcification and Convulsion. All neonates with convulsions should undergo fundus examination and X-ray skull. However, the child may have only ocular lesions. In severely infected child, there is microcephally and mental retardation. Children with ocular lesion are generally diagnosed when they are examined for infentile strabismus which is invariably estropia or diminished vision. Some times there may be nystagmus unilateral cases are generally amblyopic. Recurrence is seen between ten years and forty years. They are more common in teen age girls 24,42 Recurrences generally occur either on the periphery of the healed lesions or as satellite lesion adjacent. The recurrence has all the characteristics of acute lesion with involvement of vitreous that is studded with cells and floaters sufficient to reduce the visibility of the lesion in a head light in the fog manner24. Posterior vitreous detachment is common, the detached vitreous is dusted with vitreous precipitates similar to Kps. Other involvements are - papillitis and vasculitis. Some times the lesions may be punctate, scattered on wide area of retina without much vitreous reaction. Complications include cataract, glaucoma, subretinalneovascularisaton, exudative retinal detachment45 Vitreous haemorrhage, nystagmus, opportunistic systemic infection in immuno compromised individual. Differential diagnosis consist of congenital coloboma of macula, tubercular choroiditis, cytomegalo virus infection, retinoblastoma, herpes simplex chorio retinitis. Diagnosis : Diagnosis of congenital toxoplasmosis is straight forward when the child is brought with systemic symptoms of convulsion, mental retardation, microcephaly and squint. On examination a typical patches of central choroiditis is visible. On X-ray there is invariably intracranial calcification. CT and MRI may show small lesions in the cortico medulary region. Other investigation are (1) serological test for serum antitoxoplasma antibody. Any titer of serum antibody is significant.45 2. Elisa test, 3. IgG and 3 IgM titer - A positive IgG may be present without ocular involvement, positive IgM is indicative of recent infection. Management There is no prophylaxis for toxoplasmosis. There is no treatment for infected cats as cats may be asymptomatic. Best way is to look after personal hygiene, avoid raw vegetables. Meat should be well cooked. Once diagnosis has been confirmed the treatment is by anti-toxoplasmosis chemotherapy, cortico steroid, photo coagulation of peripheral lesions and management of uveitis.

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Chemotherapy Drugs available for treatment arePyrimethamine, Sulphadiazine alone, triple sulpha, sulpha methoxazole with trimethoprim, clindamycin, spiromycin, minocylin and atovaquone, vancomycin, tetracycline. The dose should be administered in consultation with pediatrician who should also monitor for side effects. Once the lesion has healed no treatment is required, but chemotherapy may prevent recurrence. Pregnant mothers who test positive should be given full course of treatment in consultation with gynecologist and neonatologist. For active lesion a combination of pyrimethamine triple sulpha with systemic steroid is the best combination available. Steroid alone may suppress an acute attack for some times only to flare up later. The chemotherapy should be continued for weeks, the steroid is generally tapered after seven to ten days. Pyrimethamine can cause marrow suppression, nausea and leucopenia. Hence it is necessary to get weekly WBC count to overcome the side effects. 5 mg. of leucovorin (folinic) acid is given twice a week. Eye with multiple recurrence and vitreous haemorrhage may require vetrectomy. Rubella uveitis Rubella uveitis in children is part of congenital rubella and over shadowed by congenital cataract and glaucoma. In seventy percent of cases it produces non progressive chorioretinitis giving a pepper and salt fundus appearance with good central vision and normal ERG. Other causes of pepper salt fundus should be excluded. In some percentage of cases, there may be iritis or iridocyclitis. The iris stroma is severely damaged leading to partial hole formation that trans-illuminates reflected ray, pupil is generally small and dilates poorly with atropine. Both dilator and sphincter muscle may be involved. There is no specific treatment, congenital rubella can be prevented only if the mother has been immunized in childhood. Herpes simplex uveitis24, 42, 49 In children herpes simplex uveitis is generally secondary to herpetic keratitis but can be due to systemic involvement as well. It is mostly produced by type II herpes simplex virus, however, type I can also cause cutaneous lesions that are self limiting. Virus may migrate to trigeminal ganglion only to be reactivated with reduced resistance. It can cause non granulomatous anterior uveitis with or without evidence of cutaneous or corneal involvement. It can also cause chorioretinitis and retinitis in older children. The condition is painful with intense lacrimation and photophobia with evidence of active keratitis or stromal keratitis, cells, flares, posterior synechia and occasional hypopyon. The neonate gets the infection during passage through birth canal and lesion develops between two weeks to 18 months after. It may occasionally be life threatening. Other common age groups to be affected is between 6 months to five years. Management is by way of cycloplegic for lacrimation, and photophobia, three times a day and weakest possible steroids under supervision if cornea is not involved. Systemic anti viral drugs should be administered in consultation with pediatrician. Syphilitic uveitis in pediatric age group Syphilis has been called greatest imitator in clinical medicine. It can inflict any age of both sexes, all ethnic groups and involve any part of body in various combinations. It used to

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be a major cause of uveitis both acute and chronic before present era of antisyphilitic treatment came into vogue. In children commonest form of uveal involvement is associated with congenital syphilis, however, acquired syphilis can also be seen in pediatric age group due to frequent child abuse and precocity. Uveal involvement in children may be (1) congenital and quiet at birth, (2) active at birth, (3) may become active at teens, (4) may be acquired in teens.24 There is no primary stage in congenital syphilis. The congenital syphilis in initial years are comparable with secondary syphilis becoming tertiary, if not treated.45,47 Hence uveal involvement in children may show features of secondary or tertiary syphilis. It can be acute (1) Associated with interstitial keratitis (2) Without involvement of cornea (a) Acute iritis (b) Diffuse chorioretinitis. Uveitis in interstitial keratitis preceeds corneal involvement, however, corneal reaction may be severe enough to obstruct examination of the iris and anterior chamber. Finding of the uveitis may only be visible after the cornea has cleared. Corneal endothelium may be damaged by KPs and secondary guttatae may develop, hyaline strands may project in anterior chamber, angle may get hyalinised resulting in glaucoma. The iris may be atrophic.30 Other syphilitic lesions include chronic iridocylitis, chorioretinitis, retinal phlebitis, vitreous opacity.45 Pupillary changes can be due to involvement of iris stroma or part of neurosyphilis. Acute iritis can start as network of fine capillaries, vascular papules and nodules.24 There are multiple synechia resulting into miosis which does not respond to atropine, flare and cells in anterior chamber are common.39 Posterior segment involvement results in salt and pepper spots of chorioretinitis. It is generally bilateral and peripheral may involve posterior pole or a quadrant. There is no visual loss. In later stages, salt and pepper spots may be confused as retinitis pigmentosa. Diagnosis of syphilitic uveitis is often missed as it is generally thought that syphilitic uveitis is only seen in adults. Interstitial keratitis related anterior uveitis is easy to diagnose and responds well to treatment. The diagnosis is confirmed by variety of serological tests. The laboratory investigations in syphilis are same for all ages. They can be (1) NontreponemalVDRL and rapid plasma regain (2) Specific treponemalFTA - ABS - Flourescent treponemal antibody absorption MH4TP - Micro hemo agglutination test. Non treponemal test V.D.R.L. becomes negative in late stages even with or without treatment but FTA-ABS never become negative. Treatment Treatment of syphilitic uveitis with interstitial keratitis does not pose much difficulty. Difficulty arises when uveitis in children is suspected to be due to secondary or tertiary stage. In all such cases it should be confirmed by FTA-ABS and treated as acquired syphilis in consultation with pedriatician.

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Local treatment consist of atropine ointment under five, drops may be used in older children with caution. Once desired mydriasis has been achieved, atropine may be replaced by 1% cyclopentolate. Topical steroids are administered as per severity, to begin with its can be as often as hourly to be reduced to three times a day. Steroids drops may have to be used for months and the child monitored constantly for possibility of side effects. In older children peribulbar steroid can be given in recalcitrant cases. Oral steroids are not required. Uveal involvement is generally hematogenous. As anterior and posterior uvea have different blood supply, the involvement is independent of each other but can be concurrent. Surprisingly involvement of uvea is rare along with active pulmonary lesion. Miliary tubercle of choroids is common in military tuberculosis and meningitis. Tubercular lesion of uvea is thought to be due to sensitization of uvea to tuberculosis protein. The sensitization depends on systemic resistance and host immunity. Inflammatory and destructive process is due to hyper sensitivity while healing is due to host immunity and host resistance. In children, following primary infection a fast developing hypersensitivity takes place due to low or absent immunity. It is possible to get either anterior or posterior uveitis separately or together to results in a pan uveitis. Uveal involvement is generally granulomatous with mutton fat KP, aqueous flare and cells in anterior chamber, edema of iris and ciliary body. Formation of Koeppe nodules at the pupillary margin, broad posterior synechia, exdates over the anterior lens capsule. Formation of tubercular nodule all over the uvea, either as solitary or miliary tuberculosis. Tuberculoma is now a days extremely rare. Vitritis is common, there may be papillitis. Macular involvement leads to early and permanent loss of central vision. A self limiting acute iritis develops following cutaneous injection of tuberculin. This is non granulomatous and without synechia. Posterior uveitis is less common in children than in adults. Common presentations are : (1) Miliary tubercle in the choroid secondary to tuberculous meningitis (2) Circumscribed lesion in the posterior pole (3) Involving the macula. A healed macular involvement may be confused as congenital coloboma of macula or toxoplasmic chorioretinitis. Occasionally there may be perivasculitis with sheathing and new vessel formation that may cause vitreous haemorrhage ranging from small pre retinal haemorrhage to full blown vitreous haemorrhage. Diagnosis Diagnosis of uveal tuberculosis is one of baffling problems. It is done by exclusion, presumption and confirmation. Tuberculosis should be differentiated from other forms of granulmatous uveitis like toxoplasmosis, syphilis, and sarcodosis. Presumptions In case of presumed uveal tuberculosisthe child has raised ESR and lymphocytosis. The uveitis does not respond favourably to local cycloplegic and steroids or becomes worse. A therapeutic trial with isonniazide 10 to 20 mg/Kg per day not exceeding 300 mg is administered to the child as a single dose daily for 2 to 3 weeks with local treatment. If there is significant

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improvement other systemic anti tubercular antibiotic agents are added in consultation with pediatrician.57E Confirmatory investigations : (1) Routine TLC, DLC and ESR. (2) X-ray chest to exclude pulmonary involvement is undertaken. (This generally comes out to be negative.) (3) A thorough examination of abdomen and lymph nodes are done to exclude extra pulmonary tuberculosis and if found to be positive a full course of three drugs anti-tuberculosis chemotherapy is started. Skin test (Mantoux test). Purified protein derivative is used. First 5(TU) of PPDT is used as interdermal injection and reaction is red after 48 hours. If this test is negative 25(TU) is used for confirmation. Any reaction even erythema is suggestive of tubercular origin. However, a positive test is seen in children who have received BCG as a part of immunization or suffered from tuberculosis in the past. Other test : Anti tubercular immunoglobin IgA. IgG and IgM. Polymerase chain reaction is very sensitive in pulmonary tuberculosis and less sensitive in extra pulmonary tuberculosis. Management of tubercular uveitis Management of tubercular uveitis consists of local treatment by cycloplegic and steroids. Posterior synechia of tubercular iridocyclitis are difficult to break hence they require prolonged use of strong cycloplegic like atropine used with usual caution. Once the pupil has been dilated atropine may be replaced by cyclopentolate or a combination of cyclopentolate with phenylephenine. Local steroids have to be used for long time than other non infectious uveitis and gradually tapered off over months. The child should get minimum three drugs for systemic anti tubercular chemotherapy for 6 to 9 months. Toxocara infection of uvea (See page 489 also) Toxocarasis is a nematod infection. It is a c ommon cause of chronic posterior uveitis. It is caused due to ingestion of embryonated ova of an intestinal parasiteToxocara canis round worm of the dogs.50 The puppies that are most infectious, the puppies get infected via two sources1. a more common way is ingestion of infected food and 2. Less common by trans placental infection.42 In both instances this ova hatches in the small intestine to form larvae. The larva penetrates the intestinal wall and is taken up by systemic circulation to reach distant organs. Some larvae reach the trachea and are coughed up. The coughed larvae is reswallowed to reach the intestine and get converted into an adult worm.51 A female adult worm produces 200,000 eggs per day, that are excreted in feces of the dog. The eggs can survive in the soil for years without hatching. They only hatch in the intestine of either dogs or human being. The second stage of infection develops in the intestine of a toddler who may ingest either the soil or food contaminated with by ova. The ova form larvae in the intestine that penetrate the intestinal wall, from where they are carried to distant organs including eye. The larvae do not mature to become mature worm in human beings, nor they return to the gut, hence they are not excreted in the feces of human being. The systemic infection of by toxocara has two distinct stages. (1) Stage of viscereal larva migrans. (2) Distant spread to liver, lungs, brain, muscle and eye. Involvement of eye is commonly referred to as ocular toxocariasis.

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Visceral larva migrance is characterized by malaise, fever, cough, hepato spleenomegaly, leucocytosis, eosionphilia and cutaneous lesion without ocular manifestation. Ocular toxocariasis is a late feature of systemic involvement. It is rarely seen with systemic involvement. Average age of a child with visceral larval migrans is two years while ocular toxocariasis is seen between 18 months to 18 years, average being 7.5 years.51 There is a latent period of few years between visceral larval migrans and ocular toxocariasis. Ocular toxocariasis is always unilateral condition, seen mostly in male, exact cause of this preference is not known. All races are equally affected. Its incidence in under developed countries must be more than reported. Once this larvae reache the eye, it can produce various types of posterior uveitis.51, 52 A typical lesion is posteriorly placed. Anterior segment signs and symptoms are minimal but not altogether absent. Children are mostly brought with strabismus, diminished vision or rarely white reflexes in pupillary area. On examination the eye is generally white with minimum cells and flare in aqueous. Ocular toxocariasis generally produce one of the following types of lesions. 1. Solitary posterior polar granuloma in the macular region or between the macula and nerve head. 2. Peripheral granuloma 3. Chronic endophthalmitis. Solitary posterior polar granuloma is most common type of lesion that causes diminished vision due to involvement of macula. It is mostly seen in age group 6 to 14 years. The lesion is one or two disc diameter, raised yellowish white area, may be surrounded by hard exudates. Retinal vessels disappears in the lesion. There may be peri retinal gliosis with traction lines, there may subretinal haemorrhage with serous retinal detachment. The lesion is discovered either during routine eye examination or by chance or when the child closes the normal eye and discovers diminished vision in uncovered eye or the child may be brought for squint, more rarely with a white reflex in pupillary area. Peripheral retinal granuloma. This is generally associated with parsplanitis, seen above 6 years of age, may be discovered in adults by chance. The peripheral lesion is a solitary mass associated with extensive peripheral gliosis. There may be vitreous bands extending from the lesion to the macula, disc or both causing traction displacement of macula that may present as pseudo squint. As the lesion is peripheral vision remains unaffected unless macula is involved. The vitreous band may cause retinal breaks that lead to localized, or extensive tractional retinal detachment. Chronic endophthalmitis. This type of lesion is generally seen in younger children who are brought with either diminished vision squint or white reflex in pupillary area. There may be involvement of anterior segment with cells, flare, posterior synechia, even hypopyon. The vitreous is infiltrated with cells. In late cases a cyclitic membrane may develop. Vision is grossly diminished and it becomes difficult to visualize the fundus, macular edema and cataract further worsens the vision. Tractional or rhegmatisgenous retinal detachments are common. Less common presentations areExudative retinal detachment, diffuse chorioretinitis, diffuse unilateral sub acute neuro retinitis (DUSN)50, 51, optic neuritis unilateral pars planatis.

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Most commonly mistaken diagnosis is retinoblastoma which cannot always be differentiated clinically, however, toxocariasis does not show intraocular calcification on X-ray and ultrasonography, it is extremely rare to get bilateral toxocariasis. Anterior chamber aspiration does not show tumour cells but gives polymerase chain reaction to toxocara antigens. Other disorders that form differential diagnosis are : Coats disease, other forms of endophthalmitis, persistent hyper plastic primary vitreous, retinopathy of prematurity. Diagnosis Diagnosis of ocular toxocariasis is always presumptive. All cases of unilateral granuloma in a white eye should be suspected to have ocular toxocariasis unless proved otherwise. Contact with infected puppies is so common in general population that history of contact with puppies is of hardly any use, so are the skin changes of viscerallarval migrans. Differential count may show eosinophilia, X-ray orbit, ocular USG, CT and MRI are negative for intraocular calcification. Most reliable test is ELISA test to detect toxocara antibodies in serum, aqueous or vitreous. A titer of 1:8 in serum is diagnostic. However, sometimes serum may give negative results while aqueous/vitreous give positive results that is reliable. Management 1. Prophylaxis. There are no sure way to prevention except personal hygiene and non consumption of uncontaminated food, however, pica cannot be stopped in toddlers who are most susceptible. Role of routine deworming of child may not be sufficient to expel the larvae. However, regular deworming of puppies from second week has been recommended. It is virtually impossible to deworm stray puppies that are the main source of contamination of soil. 2. Therapeutic. (A) Medical I. Anti helminths : Though broad spectrum anti helminths are routinely prescribed in children suspected to be suffering from toxocariasis. There role has been questioned. Anti helminths may cause increased inflammatory reaction due to deaths of the organism. Anti helminths whenever administered should be given under umbrella of full dose of cortico steroids in uveal toxocariasis. II. Corticosteroids : Corticosteroids can be used in conjunction with anti helminths or alone. They can be used orally or by way of periocular injection. Anterior segment reaction is managed by local cycloplegies and steroids. (B) Surgical method. Vitreous surgery not only removes all inflammatory process and antigen but also vitreous bands and helps in placing of scleral buckle. The material removed by vitrectomy may be used for confirm diagnosis. Vitrectomy may be used to remove posterior cyclitic membrane as well. Laser photo coagulation51 of live nematod when detected, should be tried only when the nematode is away from posterior pole. Cryo freezing24: When laser is not available trans scleral cryo coagulation of peripheral lesion may be attempted under direct vision.

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Acquired immuno deficiency syndrome and uveitis Acquired immuno deficiency syndrome is an infectious disease with world wide distribution, both sexes are affected. No age is immune. AIDS can be congenital due to transplacental transmission of human immunodeficiency virus type I (HIV-I) or infection may be acquired during the passage of the child through birth canal of infected mother. Number of children affected by AIDS is increasing. Children who receive blood transfusion regularly due to various blood disorders are also at high risk. In older children HIV can be acquired due to use of contaminated needle especially in drug users. The HIV virus targets the CD4 lymphocytes and destroy it. The HIV virus is a retrovirus known as human immuno deficiency virus type I. It has only RNA in its structure, it uses patients cellular reproductive system to manufacture DNA for multiplication.53 Fifty percent of HIV positive persons are transient non infectious. However, about 90 percent will develop AIDS which is uniformally fatal over the years. There is a gap of few years when an HIV infected person is converted to AIDS. 50% to 75% of AIDS patients will ultimately develop ocular lesion, which may result into blindness. The HIV virus has affinity for T helper lymphocytes (CD4) which are destroyed by HIV virus leading to sever immune deficiency as a result of some organism which may otherwise lie dormant and become active. There may be many fold increase in virulence of infective organism in the body. Some of the microorganisms have greater predilection for AIDS patients. These opportunistic organism can be bacteriamycobacteria, fungi, herpes simplex, herpes zoster, cytomelagic virus, Epstein Barr virus, adeno virus, toxoplasma and pneumocystis. Ocular manifestation may be either due to 1. AIDS itself or 2. AIDS related complex.24 3. AIDS by itself can clog this micro vasculature by deposition of immune complexes or 4. AIDS related opportunistic organism and malignancies. The uveal lesion may be a nongranulomatous pan uveitis due to HIV or due to herpes simplex or herpes zoster. Commonest type of ocular infection is CMV retinitis that cause multiple cottonwool spots, acute retinal necrosis or retinal detachment.53 CMV retinitis is cell CD4 dependent.53 It is seen in patients who have CD4 count less than 50 cells/mm3. The disease is bilateral due to haemotogenous spread. Other ocular manifestations may involve any or many parts of the eye from simple follicular conjunctivitis to severe retinal necrosis leading to bilateral blindness. They include keratitis, retinal vasculitis, retinal haemorrhage, Roths spot, microaneurysm, papillitis and ischemic maculopathy. Kaposi sarcoma is commonest form of malignancy met in patients with AIDS. Though this is malignancy of adults it occurs in teens in patients with AIDS as well. The tumor is of endothelial origin 54, it is seen in 35% of with HIV infection. It involves lids more often than conjunctiva, orbit is involved rarely. It is mostly seen in males. It is considered to be an indicator that the patient has reached the last stage of primary disease55. Once Kaposi sarcoma develops life, expectancy is less than two years. Extremities are involved earlier than periocular structures. The lesions are multicentric. Diagnosis A person is said to have AIDS if following criteria are present.53

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1. Unequivocal evidence of HIV I infection confirmed by ELISA and Western blot test. 2. CD4 cells count less than 200 cells/mm3. 3. Evidence of systemic infection by at least one opportunistic organism. 4. Presence of Kaposi sarcoma. Management AIDS is uniformly fatal in spite of treatment. It is a blinding disease in 60% of patients but it is fully preventable disease. Once the disease sets-in, management is by multiple HIV drugs given systemically. Ocular management is paliative. Malignancy do not require separate treatment however concurrent anti cancer drugs may be required. Ocular treatment for anterior uveitis is by local steroids and cycloplegic. Posterior segment treatment consists of systemic anti HIV drugs and surgical treatment for retinal detachment with uniformly poor prognosis. Connective tissue and uveitis in children Connective tissue disease involving joints are common causes of both acute and chronic anterior uveitis in children most of them are positive for HLA B-27, antinuclear antibodies and negative for rheumatoid factor. Some of the characteristic features of arthritic uveitis areThey are generally acute, unilateral that may be recurrent in the same eye or may alternate between the two eyes, posterior synechia. Hypopyon is common. Common joint disorders associated with uveitis in children are : Juvenile rheumatoid arthritis (JRA), Stills disease, juvenile spondylitis, juvenile Reiters syndrome, arthritis with bowel disease. Juvenile rheumatoid arthritis24, 29, 42, 43 It is the commonest form of arthritis seen in children between 2 to 16 years of age, peak being 2 to 4 years. It is more frequent is girls. Ratio of girls to boys is roughly 3:2. Antinuclear antibodies is positive in 75% of cases while 95% of patients have positive HLA B-27, it is sero negative. According to clinical features in first three months the disease has been divided into three sub groups. 1. Predominantly systemic involvement with minimum uveal involvement. It manifests with intermittent fever, lymphadenopathy, hepato spleenomegaly and maculo papular rash. There may be pericarditis. 2. Polyarticular involvement with moderate uveal involvement. This type affects four or more than five joints during first to three months. Knee joint is mostly involved followed by wrist and ankle. Arthritis may last for many years resulting in permanent joint deformity. The condition is generally symmetrical. 3. Pauci articular involvement with more uveal involvement - About 5% of children with JRA come in this group. Number of joints involved in first three months is four or less, involvement is asymmetric, knee happens to be most commonly involved.

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Twenty percent of children develop uveitis. Arthritis usually precedes ocular involvement by several years, occasionally reverse may be true. Uveitis in juvenile rheumatoid arthritis Uveitis in ninety percent of cases is mostly anterior uveitis, pan uveal involvement is extremely rare so is involvement of vitreous and choroids. It is bilateral in 70%-80% of cases and recurrent. It is non granulomatous in nature. There are two groups of patients who have uveitis. 1. Silent white uveitis seen mostly in girls between 4 to 16 years of age. The onset is so insidious without any discomfort that it is called silent uveitis. The condition may be diagnosed not due to redness, lacrimation or photophobia but due to diminished vision or on routine slit lamp examination that shows one to two plus cells + fine KPS and flare. 2. The pausi articular involvement as idiopathic anterior uveitis in adolescent. The ocular manifests may continue despite treatment and apparent cure of joint involvement leading to frequent complications of band keratopathy, cataract secondary glaucoma and macular edema. Somehow hypotony is also a common feature. The complications are potential threat to vision, hence the child should be under surveillance for at least 6 to 7 years following development of arthritis. Children with iridocyclitis in pausi articular disease should be examined every three months and cases of iris with positive ANA more frequently. Examination should include recording of acuity of vision, measurement of intraocular tension both for glaucoma and hypotony. Child should undergo assessment of errors of refraction and examination of fundus and detailed slit lamp biomicroscopy. Diagnosis is not difficult with history of joint involvement in child with non granulomatous uveitis, positive ANA and HLA B-27 but negative serology for rheumatoid factor. Differential diagnosis consists of idiopathic iritis, infective iritis and sarcoid in children. Management Management depends upon severity and duration of disease, presence of complications are managed as and when they arises. Severity of the disease can be 1. Mild with +1 cells and flares. 2. Moderate attack lasting more than 4 months with +2 to +4 aqueous cells. 3. Moderately severe lasting more than for four months with acute exacerbation. 4. Severeattack lasts for years in spite of treatment, respond poorly to treatment, are more prone to develop complications. Mild to moderate cases can be controlled by cycloplegic and local steroid drops. In initial stages the eye should be atropinised to break the posterior synechia. Once the posterior synechia have been broken the child is put on short acting cycloplegic like home atropine 2%two to three times a day or cyclopentolate once a day. Children should not be put on atropine for long

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times as it may induce atropine induced amblyopia. A school going child under atropine require bifocal for near work. Once the inflammation has been reduced, a short acting cycloplegic like tropicamide at bed time gives sufficient accommodation during day to go to school. For mydriasis induced glare dark glasses are prescribed. Next line of medicines are steroids in the form of drops or periocular injection. Drops may be required hourly for first few days in acute stages, otherwise instillation four times a day is sufficient. Strength of steroid should be minimum with maximum benefit. Ocular side effects of local steroids should be weighed against its advantages and its dose adjusted accordingly. Periocular steroids are given in children in teens, younger children do not cooperate for periocular injection. Systemic steroids should be avoided under ten years of age due to its systemic side effect. If at all it has to be given it should be prescribed in consultation and under supervision of pediatrician. Older children are given steroid as in adults. Non steroidal anti-inflammatory drops are generally prescribed as maintenance therapy for long time. So far no ocular side effect has been reported following local administration of NSAID. Systemic non steroidal anti inflammatory drugs are better avoided in children. Chronic cases not responding to local steroids or intolerant to systemic steroids are put on systemic immune suppressives drugs in consulation with oncologist. Dose of immuno suppressive drugs for uveitis is less than that for malignancy. A constant watch on side effects especially haemotological must be kept. They are also better avoided under ten years of age. Complications are frequent both due to disease itself and sometimes due to prolonged use of steroids. Common complications are cataract, secondary glaucoma, band keratopathy, amblyopia and hyptony. Cataract is managed by surgery for which a quiet eye is a prerequisite. The eye should be white without flare for at least three month before surgery. Surgery should be followed by use of steroid in sufficient dose and frequency for long time. An unnecessary delay in cataract surgery may lead to amblyopia. All surgeries should be done under general anaesthesia with endo tracheal intubation. Thick posterior capsular membrane formation is very common that may require repeated manipulation. Role of intra ocular implant is unsatisfactory, may be avoided as far as possible. Aphakia may be managed either by contact lens, with spectacle reading correction or bifocal. In smaller children contact lens fitting is really difficult though not impossible. Glaucoma Possibility of glaucoma should always be kept in mind, it can be due to inflammatory process and its aftermath, steroid induced or both. Secondary post inflammatory glaucoma respond poorly to anti glaucoma drugs. Miotics and prostaglandin analogs are contra indicated. A child can not be put on oral carbonic anhydrase inhibitor for a long time due to systemic side effect. Surgery also does not give satisfactory results, failure is common. Band keratopathy is managed initially by EDTA otherwise lamellar keratoplasty or excimer photo therapeutic keratectomy may be tried.

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Amblyopia is a tumbling block that is produced due to prolonged use of atropine, opacities in media, macular edema, undetected glaucoma, anisometropia following unilateral lens extraction. Failure to use contact lens and unsatisfactory I0L transplant. Uveitis in ankylosing spondylitis42,45,56 It is a chronic inflammatory disease of unknown origin that involves mostly the axial spine in adults. In children the disease is mostly seen in peripheral joints of lower limb. The disease has positive HLA B 27 in 80 to 90 percent patients. It is sero negative for rheumatoid factor. About 25% children suffering from ankylosing spondylitis develops anterior uveitis. 50% uveitis with positive HLA B 27 suffer from ankylosing spondylitis. Ocular manifestations are mainly anterior uveitis that is acute, recurring, unilateral, non granulomatous. About 80% children with uveitis will develop bilateral disease that is rarely simultaneous. An attack of anterior uveitis generally lasts for 4 to 6 weeks. Recurrence may vary between every month to one year. Uveitis may be proceeded, may be simultaneous or may follow diagnosis of spondylitis. Signs Consists of circum corneal congestion, fine dusty or small KPs cells and flares are generally in the range of 3+ and 4+. In more severe cases there may be frank hypopyon, posterior synechia results in iris bombe and secondary glaucoma. Cataract, CME and band keratopalthy are less common than in juvenile rheumatoid arthritis. It is more common in adolescent boys than girls. Diagnosis Unilateral acute iridocyclitis in second decade in a boy with pain in peripheral joint, positive HLA-B 27 should undergo X-ray of sacro iliac joint for possibility of sacroilitis. Management is with cycloplegic local and periocular steroids. Reiters syndrome is a rare disease of unknown origin, parts of it may be infective, (urethritis and dysentery) it was originally described as triad of urethritis, conjunctivitis and arthritis. However it is associated more commonly with anterior uveitis, keratitis than conjunctivitis. Conjunectivitis when present is generally bilateral and mucopurulent. Arthritis is poly articular and asymmetrical. uveitis is generally unilateral, non granulomatous. Treatment comprises of local treatment of mucopurulent conjunctivitis by frequent antibiotic drops. Uveitis is treated with cycloplegic and steroids. Urethritis is non gonococcal and treated with erythromycin. Dysentery is treated by combination of trimethoprim and sulpha methoxazol or ciprofloxacin. Pars planitis or intermediate uveitis57A,57B Pars planitis is a common chronic bilateral uveitis that is either not diagnosed till late or misdiagnosed. Its exact etiology is not known. It has variable clinical presentation, hence it has been called by various names since 1908. When Fuchs called it cyclitis. The Internaational uveitis study group has finally labelled it as intermediate uveitis in 1987.57C Since than it is being referred as such.

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Pars planitis is a disease of pars plana of ciliary body adjoining choroid, peripheral retina mostly peripheral retinal vessels. In large series it has been reported to be as common as 10 to 15 percent of all uveitis cases observed. It is a disease of young, adults and children. There are reports that it may run in families. Both boys and girls are affected equally. To begin with it is almost symptomless, the earliest symptoms that may attract childs attention is floaters in the affected eye or mild diminished vision. Floaters are generally not taken very seriously by the parents or treating general physician. By the time the next symptoms of diminished vision occurs the disease has progressed far. The vision may be within normal limits initially. Common cause of diminished vision are- Posterior subcapsular cataract and cystoid macular edema. On slit lamp examination there are cells and flares is aqueous. Posterior synechia are so uncommon in pars palanitis that some authors consider that presence of posterior synechia is evidence strong enough to exclude pars planitis.57D Sometimes there may be few KPs and peripheral corneal edema. The anterior vitreous shows flares, cells and strands. Examination of peripheral retina with indirect opthalmoscope and scleral indentation shows evidence of peri artrititis and periphlebitis. Most important finding on examination of peripheral retina are snowball opacities and snow banking mostly in the inferior quadrant. There may be new vessel formation near the snow bank that may be source of haemorrhage58. Cystoid macular edema is a late feature and major cause of diminished vision. The course is variable. Some children may have the disease for years with or without loss of vision, others may have choroidal effusion and serous retinal detachment. Hypotony is common. However some patients may have rise of intraocular pressure due to spilling over of the pathology in the angle. There is no HLA phenotypes. Fundus fluorescein angiography is the diagnostic of CMC and periphlebitis. Differential diagnosis consists of - idiopathic iridocylitis. Absence of joint systems rule out juvenile rheumatoid arthritis associated with uveitis. Peripheral granuloma of toxocara is excluded by negative serology test. Careful examination of retinal periphery, presence of snowball makes it imperative to exclude sarcoid by X-ray chest, gallium scan and raised serum calcium. Management Almost eighty percent of children do not require any treatment. Local and peripbulbar steroids are required if vision falls below 6/18. Cycloplegic do not have any added advantage. Children not improving with local peribulbar steroids should be put on systemic steroid for long time with usual precaution. Immuno suppressive drugs may be required. Rarely surgical intervention like lensectomy or vitrectomy is required. Sympathetic ophthalmia57, 60, 61 Sympathetic ophthalmia is a bilateral potential blinding disorder. It is said to be rare disease in advanced countries and urban areas of developing countries but its incidence in under developed countries must be more than reported. It is a bilateral chronic pan uveitis

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with acute exacerbation and recurrences. It is most commonly seen following penetrating injury within five mm. of limbus with incarceration of uvea. However, rupture of the globe anywhere with incarceration of any intra ocular structure can cause sympathetic ophthalmia. About 60% of the cases are seen following accidental penetrating wound. However, it has been reported following blunt injury. About 30% are seen following surgical manipulation in which the globe is penetrated. Common surgical procedures arelens extraction, any glaucoma operation, iris inclusion surgeries for glaucoma were notorious to produce sympathetic ophthalmia, retinal detachment surgeries with drainage of sub retinal fluid. Even laser photo coagulation have been reported to cause sympathetic ophthalmia. It is said that sympathetic ophlhalmia does not develop if the injured eye has developed suppuration. However, it has been reported following perforation of corneal ulcer. The eye that is injured is called exciting eye and the eye that develops chronic pan uveitis is called sympathizing eye. No race, age or sex is immune. Children are affected more than adults, number of boys developing sympathetic uveitis is more than girls. This is perhaps due to the fact that boys are more prone to injury than girls. Interval between injury in exciting eye and development of sympathetic uveitis varies. It spans between 5 days to 66 years, 70% of cases develop within three months and 90% within first year. Etiology of the condition is not well understood, however, there is sufficient evidence to consider auto immunity as prime factor. The reaction is delayed type-four hypersensitivity to uveal antigens located in retinal pigment epithelium and melanocytes. Anti retinal antibodies have been found in some cases24, 66, 61. The immune reaction results in chronic granulomatous lesion that forms a giant cell similar to tuberculosis without caseation. The chorio capillaries are spared. There is thickening of all the parts of uvea, specially the choroid. Small yellowish white spots called Delen Fuchs nodule are seen scattered in the choroid. They are common in the lower mid periphery, however, they are not diagnosed of sympathetic uveitis. Clinical features Clinical features are variable, all eyes with penetrating injury do not develop sympathetic uveitis. Some may develop it within few days, others may not be effected for decades after initial injury. Clinical presentation depends upon inflammatory process which in turn is perhaps genetically predisposed. The disease generally develops in iris but may start anywhere in the uvea and it is bound to become panuveal. The injured eye has evidence of penetrating injury and its complication that may mask the presence of indolent plastic iridocyclitis. The early symptoms of uveitis in the sympathizing eye are heralded by photophobia, loss of accommodation, lacrimation and blurring of vision. The eye at this stage may not be congested. First sign of sympathetic uveitis is flare the retrolental space. The anterior chamber may show 2+ to 3+ cells and flare. The cornea has various shapes of KPs. Generally they are large mutton flat in nature but may be fine dusty. The iris is greatly thickened. The pupil is miotic and irregular due to strong posterior synechia. There may be both Koeppe and

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Busaccas nodule present. Onset of inflammation in sympathetising eye generally worsens, the signs and symptoms in the exciting eye. The posterior segment involvement results in the posterior vitreous cells, papillitis, retinal vasculitis and serous retinal detachment, the eyes are generally soft. Extra ocular manifestation are rare, they may be vitiligo, poliosis, alopecia and polycytosis of CSF. Common complicationa in untreated cases areComplicated cataract, secondary glaucoma, band keratopathy, retinal detachment, phthises and blindness. Sometimes the exciting eye may have better vision than the sympathetising eye. Diagnosis is mostly clinical based on 1. History of trauma, that has either not been repaired or repaired without freeing of uvea from the wound. Sometime the injured lens may cause lens related uveitis. This does not involve the other eye and can be managed-well by removal of lens material. 2. Clinical evidence of anterior uveitis or choroiditis. 3. Ultrasonography shows thickening of choroids. 4. Fluorescein angiography may initially show areas of hypo fluorescence due to masking of choroid by Delen Fuchs nodule followed by hyper fluorescence due to the leak of the dye. Management Correct and early diagnosis helps to restore vision better and prevent permanent loss of vision. Management of sympathetic ophthalmia can be divided in the two groups (1) Surgical and (2) Medical. Surgical treatment is restricted to injured eye that consists of management of (1) Injury and (2) Enucleation of unmanageable injury. All penetrating injuries in children should be repaired under general anaesthesia, under microscope with use of viscoelastic substance. Before embarking on repair of the visible wound possibilities of other sub conjunctival wounds and wounds under the extra ocular muscle should be explored and repaired. The uvea should be dis-engaged from the wound and excised by standard procedure, if necessary. The lens matter should be removed and if needed anterior vitrectomy done. The anterior segment should be reconstructed as far as possible, a sub conjunctival injection of broad spectrum antibiotic with steroid is given along with atropine. The eye is kept under observation for evidence of uveitis. The other eye is examined regularly. The parents are warned about possibilities of sympathetic ophthalmia and instructed to bring the child for examination if the child develops lacrimation or unexplained blurring of vision. Enucleation If the injured eye is not salvaged it is better to enucleate it within the first ten days of injury. The purpose of enucleation is to remove the uvea in toto, evisceration does not serve this purpose hence it is not recommended. Role of enucleation after onset of sympathetic opthalmia is doubtful. Sometimes the exciting eye may have better vision than sympathizing eye.

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Medical treatment is usual treatment of non infective granulomatous uveitis that can be local and systemic. Local treatment consists of judicious use of atropine and local steroid drops. Initially steroid may be required as frequently as hourly which is generally tapered to two times a day. If steroid drops do not give relief within 48 hours peribulbar injection of steroid is advocated, that may be given two to three times in a week by way of soluble steroids followed by monthly injection of depot steroid. Systemic Systemic drugs used in sympathetic ophthalmia consists of two groups of drugs. They are 1. Long acting steroids and 2. Immunosuppressive drugs. Cortico steroids are the main stay of management of sympathetic ophthalmitis. Most commonly used steroid is prednisolone in a dose of 1 to 1.5 mg/kg/day. It can be given in divided doses, daily single dose or alternate day. It may be started with divided dose and switched over to alternate day dosage and gradually reduced by 5 mg. a week and later maintained either on 5 to 10 mg. a day or 10 to 20 mg. alternate day for months with usual precaution. Immunosuppressive drugs are prescribed when steroids are ineffective or producing local and systemic side effects. Commonly used immunosuppressive drugs are : 1. Cyclosporin A 5 mg/kg/day oral 2. Azothioprine 1 to 2 mg/kg day oral 3. Methotrexate 5 to 30 mg/week 4. Cyclophosphamide 1 to 2 mg/kg/day Immunosuppressive drugs can be given alone or in combination with steroid. They should always be prescribed in consultation with oncologist. Vogt Koyanagi Harada Syndrome24, 63, 64, 65 This is a syndrome of unknown etiology and it looks very similar to sympathetic ophthalmia except that there is no trauma involved in its causation. It is rarer than sympathetic ophthalmia in children. Both the conditions produce chronic panuveitis. There is no exciting eye or sympathizing eye in Vogt Koyangi Harada disease. Extra ocular features dominate in Vogt Koyanagi syndrome over ocular findings while in sympathetic opthalmia ocular presentations overshadow systemic features which are late features as well. The syndrome has (1) Prodromal stage that may be mild and consisting of headache, vertigo, vomiting, neck stiffness and periorbital pain. (2) Systemic features generally precede ocular features, which are : (i) Skin changes in the form of vitiligo, poliosis and alopecia.

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(ii) Neurological signs and symptoms are headache, neck stiffness, meningism, encephalopathy, myopathy, cranial neuropathy, CSF lymphocytosis. (iii) Auditory features are tinitus, vertigo and deafness. The auditory symptoms lasts only few months. (3) Uveitis phase : This can be divided into two groups : (i) Anterior segment involvement or Vogt Koyanagi form - This consists mostly of granulomatous uveitis that begins with blurring of vision, diminished near vision, photophobia, redness, lacrimation, dull pain. On examination, there is ciliary flush, mild mutton fat KPs, anterior chamber reaction, edema of iris, strong and broad posterior synechia, the iris nodule and atrophy of iris stroma. (ii) Posterior segment involvement (Harada syndrome) consists of multiple exudative choroidal lesion that results in exudative retinal detachment which is self limiting and resolve spontaneously. There may be papilitis. Involvement of macula results in metamorphopsia. Fluorescein angiography shows multiple area of hypo fluorescence. Area of retinal edema and detachment show hypo fluorescence. Areas of choroidal ischeamia are better shown by indocyanine green. Exact etiology is not known but it is thought to be a T-cell mediated auto immune reaction to melanocytes resulting into affection of organs that contain melanocytes i.e. skin, uvea etc. Complications consists of complicated cataract, secondary glaucoma band keratopathy, phthises. Management is similar to any chronic non-infective granulomatous uveitis. It is generally treated by local (1) Cycloplegia (2) Steroid drops (3) Periocular injection of depot steroids and (4) systemic steroids in divided multiple dose, daily single dose or alternate day single dose. (5) Immunosuppressive agents are effective in recurrence and helps reduce dose of steroids. Sarcoid uveitis It is a disease of adults but may be seen in children as well. Sarcoid is a systemic disorder of unknown cause of most probably T-cells mediated immunity that involve almost all organs of the body. All parts of the eye may be affected. Uveitis in sarcoidosis can be acute or chronic both granulomatous and non-granulomatous lesion are possible. Both anterior and posterior segments may be involved. Common posterior segment involvement can be vitritis, vitreous haemorrahage, pars planitis with typical snow bank appearance and candle wax lesion in retina. Diagnosis is confirmed by histopathological examination of skin or conjunctival nodule. Management is systemic steroids, local, cycloplegic, local and periocular steroids. Fuchs heterochronic iriodcyclitis It is commonly presumed that Fuchs heterochromic iridocyclitis is a disease confined to adults only but it is known to be present in all ages, in all ethnic groups and both sexes. It is a predominantly uniocular disease. Only 10% cases are bilateral. It is generally asymptomatic. Attention may be drawn to uniocular light colour of iris. The iris with light colour is the affected eye. The eye is generally white, on slit lamp examination small non pigmented

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keratic precipitates are seen. There may be iris nodules but no posterior synechia are seen, there may be vitreous cells. It has a protracted course with good prognosis. Most of the cases do not require any treatment unless cataract develops. Some cases may require local steroids. Cycloplegies are not required. Lens induced uveitis So long as the lens is inside intact capsules it does not produced any reaction either allergy or chemical. Leak of lens material causes two types of reaction. (I) A chemical reaction due to leak of lens protein through non ruptured lens capsule in hyper mature cataract. (II) Type two hyper lens induced hyper sensitivity. In children hyper mature lens may result either in traumatic cataract following blunt injury or in unattended congenital cataract. The condition may produce acute or chronic anterior uveitis and occasional glaucoma. Uveitis subsides with complete removal of lens matter. If lens matter is left, it may result into second type of lens induced uveitis i.e. endophthalmitis phacoanaphylactica. Other mode is traumatic rupture of lens either due to accidental penetrating injury or surgical injury resulting in severe uveitis that may involve all the parts. After uveitis subsides in the first eye, the second eyes get effected in similar ways i.e. if the lens ruptures accidentally or during surgery even years after the original incident. This may be confused with sympathetic uveitis. Treatment consists of local cycloplegic, steroid, beta blockers. Complete removal cortical material in the first eye. Behcets disease42, 66, 67 The disease is seen world over as rare condition, is more common among Japanese. Its share as causative factor of uveitis in children is very less. Exact etiology of the disease is not known. HLAB-51 is closely associated with it. There are two types of ocular involvement. 1. Mostly anterior segment involvement. 2. Mostly posterior segment involvement with systemic involvement that includes recurrent aphthous ulcer of mouth and genitals, skin lesions, joint pain. There may be sudden development of hyppyon in iridocyclitis. There may be cystoid macular edema, retinal exudates and haemorrhage, cataract and glaucoma. Treatment is unsatisfactory. Local treatment consists of cycloplegic and steroids, systemic steroids are given only in cystoid macular edema for short period. Prolong systemic steroids results in poor visual outcome, some times immuno suppressive may be required. Anterior segment ischeamia and uveitis Anterior segment ischemia is mostly an iatrogenic disease following strabismus and retinal surgery. This is more common in adults and incidence rises with age, however, it can occur in children. Basic pathology is cutting blood supply of the anterior uvea by way of disruption of anterior ciliary artery following disinsertion of more than two recti.68 The disorder starts within 24 hours of following surgery with pain, lacrimation, photophobia. It presents as hazy cornea due to edema and folds in Descemets membrane, keratic precipitates, aqueous flare and cells. If not attended to it may lead to iris atrophy causing distorted pupil and complicated cataract. Anterior segment ischeamia may also develop in sickle cell haemoglobinopathy. Flourescein angiography of iris shows well demarcated areas of non

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perfusion.69 Management consists of intensive local steroids and use of cycloplegic. It is presumed that if only two muscles are disinserted at a time, the condition can be avoided. Endophthalmitis Endophthalmitis is one of the most dreaded catastrophes in ophthalmology. It is defined as inflammation of intraocular tissues due to infection by bacteria, fungi and parasite (toxocara). Immune reaction, physical or chemical reaction, vasculitis or neoplasm can also cause it. The lens is not capable of inflammation hence it is spared.70 It is generally acute that can become chronic or may have chronic onset. Clinical presentation depends upon1. Virulence of the causative organism, 2. Chemical composition of the offending causative factor, 3. Treatment received. It is generally unilateral but can be bilateral in endogenous type. No age, sex or race is immune, however, endogenous endophthalmitis is more common in children in developing countries due to prevailing malnutrition, xerophthalmia and systemic pyogenic disease and unattended or poorly managed trauma. Clinically there are two main types of endophathalmitis, 1. Endogeneous (metastatic) end opthalmitisThis is caused by micro organism. Bacteria or fungi reach the eye from a primary site due to hematogenous spread. Viruses are not known to cause endophthalmitis. 2. Exogenous endophthalmitis is caused due to direct entry of bacteria or fungi inside the eye following trauma or spread from neighbouring structure i.e perforated corneal ulcer. The trauma can be (1) accidental with or without retained foreign body, (2) Surgical. Surgical Commonest surgery that results in endophthalmitis is lens extraction of any type with or without IOL. Other surgeries include all intraocular surgeries i.e. paracentesis, iridectomy, trabeculectomy, iris inclusion procedures, mechanical capsulotomy, penetrating keratoplasty, vitrectomy. Rarely extra ocular surgeries may produce endophthalmitis, this happens following inadvertent perforation of globe during squint and retinal surgery, periocular or peribulbar injection. The basic pathology is lodgement of the offfending organism and multiplication of it, in case of micro organism or setting an immune reaction or release of chemicals. It may start in the uvea and spread to the vitreous involving the retina in between or it may start in the vitreous and spread to the uvea and retina. While the first route is generally seen in endogenous endophthalmitis. The second path is commonly seen in exogenous endophthalmitis. In all the cases, the vitreous is severely inflamed as it acts as a good growing medium for microbia to proliferate resulting in formation of a pocket of pus in the vitreous commonly referred to as vitreous abscess. According to the site of its commencement it can be anterior vitreous abscess seen following intraocular surgeries of anterior segment or posterior abscess as in endogenous endophamitis, vitrectomy, fluid gas exchange, retained intraocular foreign body. Ultimately whole of the vitreous is involved in abscess formation. The inflammatory process from choroid spreads to ciliary body and iris resulting in purulent pan uveitis. Endogenous endophthalmitis in children Endogenous endophthalmitis in children is due to spread of micro organism from primary site that can be abdomen, lung, para nasal sinus, infected teeth, middle ear, bones and joints,

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heart, skin, lacrimal sac etc. Causative organisms can either be bacteria or fungus or parasite. Presenting features of bacterial endophthalmitis differ from fungal in many ways. Endogenous endophthalmitis generally has an indolent course that may be initially masked by more serious systemic condition. frequently it can be bilateral. As the infection is hematogenous it starts in posterior uvea. The anterior uveal inflammation lags behind in contrast to exogenous endophthalmitis where anterior segment inflammation is more pronounced than posterior. Endogenous endophthalmitis is more common in children who are malnourished, chronically ill, have under gone extensive abdominal surgery, have been on systemic antibiotic or steroids for long time, under radiation therapy or have immuno compromised status. Fungi are considered to be more common causative organism than bacteria. However, in developing countries bacterial metastatic endophthalmitis is equally common if not more than fungal endophthalmitis. Parasitic endophthalmitis is far more common than expected, about 16% of all white reflexes in pupillary area are caused by toxocara.71 Many fungi have been isolated as causative factor but common are Candida and Aspergillus. Candida is more frequent than aspergillus. Candidal infection starts in the choroids as multifocal round lesions that spread to cause perivasculitis, retinal haemorrhage, may lead retinal necrosis. The symtoms are more indolent as compared to Aspergillus which is more severe, painful and associated with hypopyon. Endogenous endophthalmitis is more effectively treated by parental antibiotic. As the lesion starts and remains under the internal limiting membrane. For the same reason vitrectomy is less effective in endogenous endophthalmitis. Systemic steroids help in controlling inflammation in bacterial endophthalmitis but are contra indicated in fungal endophthalmitis. Local antibiotic drops are prescribed to combat secondary corneal and conjunctival infection while combination of local cycloplegic and steroids are given for anterior uveitis. Exogenous endophthalmitis Exogenous endophthalmitis is three times more common than endogenous endophthalmitis. In adult common type of exogenous endophthalmitis is post surgical, which is less common in children whose accidental wounds with or without retained foreign bodies cause more inflammatory reaction. Retained organic foreign bodies more inflammatory than inorganic. Some of the organic foreign bodies are inert and do not cause any inflammatory reaction but foreign bodies containing copper and iron cause various degrees of chemical changes. Pure copper produces more severe endophthalmitis than copper alloys. Pure copper causes severest form of endophthalmitis. However, sometimes inert foreign bodies can also cause sterile endophthalmitis. Thus exogenous endophthalmitis can be 1. Bacterial 2. Fungal 3. Sterile. All of them can follow surgery or be accidental. Bacterial exogenous endophthalmitis is commonest form of endophthalmitis.72 Post surgical endophthalmitis is more common than accidental. Exact percentage of post operative endophthalmitis is just a guess. It has been reported to be in the range of 0.05% to 0.2%. It is more common following secondary I0L than primary I0L. In penetrating keratoplasty it is perhaps the micro organism in donor cornea that is responsible for the infection. Break down of asepsis and antisepsis protocol is the commonest cause of post surgical endophthalmitis. Organism get access into intraocular structure via1. Contaminated instrument, irritating fluid, visco-elastic, 2. Saprophytes from the lid and conjunctiva that become opportunistic, 3. Undiagnosed nasolacrimal duct obstruction.

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There are three forms of endophthalmitis according to the onset of the disease 1.Acute within first forty eight hours following surgery, always bacterial, caused by gram negative bacteria and gram positive cocci i.e. streptococci and staphylococcus aureus. 2. Delayed between seven to tens days mostly caused by staphylococcus epidermis and coagulase negative cocci. 3. Lateafter four to six weeks, commonest organisms being various fungi, P. acne and staphylococcus epidermis. 4. Remote infected bleb, prolapsed uvea. All types of bacteria both gram positive or negative are capable of causing post operative exogenous endophthalmitis. However, gram positive strains are responsible for 90 to 95% of endophthalmitis.71 Gram negative organism are more virulent, spreads fast and produce endotoxin. In children streptococci are more common causative organism than staphylococci which is a major cause of endophthalmitis in adults. Propioni bacterium acne, which is an anaerobic gram positive commensal is becoming more frequent cause of chronic post surgical endophthalmitis. Symptoms and signs of acute post surgical endophthalmitis arePain more than usually expected pains of surgery, lacrimation, photophobia, drop of vision below anticipated level. On examination, the lids are swollen, the conjunctival congestion is prominent, their may be chemosis of conjunctiva, hazy cornea. Intense flare and cells may have fibrinous aqueous or frank hypopyon, edema of iris. Non reacting, small pupil if cycloplegics have not been used. Exudative membrane over the I0L or vitreous face, retrolenticular flare and cells in phakic eye. The fundal glow is either very faint or absent on retinoscopy and direct ophthalmoscopy. An indirect ophthamoscope may circumviate the exudates in the vitreous and allow visualization of the fundus behind. It also gives extent and depth of the vitreous abscess that can be used to assess progress of the disease. The movements of the eyeball are normal and there is not proptosis. Loss of ocular movement and onset of proptosis denotes panophthalmitis. At this stage ultra sonography may clinch the diagnosis. Delayed post operative endophthalmitis undergoes same pathological process as acute but in a milder form. The symptoms consist of chronic reduced vision. On examination all signs of persistent uveitis are present that are initially responsive to steroids. Thus masking the exact pathology resulting into delay in treatment. On slit lamp examination there are large KPs with 2+ to 3+ flare and cells, aqueous may have fibrous strands. Lower angle of AC should be carefully scrutinized for small hypopyon that changes its level and position with the eyeball. There is generally exudates in the pupillary area. There may be exudates behind the posterior capsule. Generally there is formation of vitreous abscess. Marked reduction of vision and loss of fundal glow are two ominous signs. Fundus when visible may show retinal hemorrhages and exudates. Late onset post surgical endophthalmitis This is generally due to otherwise non virulent commonsels. The eye remains congested with mild to moderate pain and vision that was good is lost in days. On examination, there are signs of recurring pan uveitis with loss of fundal glow or a white reflex in pupillary

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area. Intraocular tesion is variable. Rise of tension results in unexplained pain that makes the child restless. Ultrasonography at this stage helps to clinch the diagnosis and differentiate from other condition that may mimic endophthalmitis. Post operative fungal infection is more often missed than diagnosed, as it has relatively less symptoms than bacterial endophthalmitis, most of these eyes have generally received usual high doses of steroids. The exact features may be masked, even the eye may be relatively white. The child invariably does not complain of lowering of vision. It takes several weeks to months for fungal endophthalmitis to develop. Generally there is a fixed thick hypopyon and rise of intraocular pressure. Vitreous may show snowball and fluffy opacities that may mistaken as cortical matter. Sterile post operative endophthalmitis24,71 is mostly iatrogenic in nature, less frequent than infective endophthalmitis. The condition has to be kept in mind to be correctly diagnosed. The inflammation is caused due to chemical insult of irrigating agents, residue from chemicals sterilizers, lens matter, monomers on the I0L. Too much manipulation of anterior uvea, vitreous disturbance. Inadvertent intraocular injection of xylocain during peribulbar injection and antibodies / steroid during periocular injection talc from the surgical gloves, cotton swabs, suture material are some of the frequent causes of sterile post operative endophthalmitis. Sterile endophthalmitis have an acute or delayed onset and respond fairly well to medical treatment, non responding cases may require vitrectomy. Post traumatic endophthalmitis About 5% of eyes sustaining accidental penetrating injury develop endophthalmitis. Onset may be as short as 1-2 days or as long as 2 to 3 months depending upon the virulence of the organism, size of the inoculum, immune status of the eye and treatment received. Accidental trauma has been found to be responsible for about one third of all cases of exogenous endophthalmitis. Increased frequency of endophthalmitis following accidental injury is high because they are caused by infected objects that are mostly vegetable in nature and carry virulent organism with them along with dirt, dust contaminated fluid etc. Common causes of perforating injuries in children are - Bow and arrow, tip cat (gilli), sling shot (gulel), twigs and leaves, protruding nails from walls, latches of the door. In fact any relatively sharp object cause penetrating injury. They are more common in rural set up and in boys. Most of the time they are uniocular. The overall profile of the organism isolated from post traumatic endophthalmitis are similar to post surgical endophthalmitis.71 Fulminating endophthalmitis develops in short period when infected by B Cereus the organism that has never been isolated in post surgical endophthalmitis. Late onset of endophthalmitis is generally seen in fungal infection. Diagnosis of post traumatic traumatic endophthalmitis is not difficult. The presentation is similar to post surgical endophthalmitis. The difference being disorganization of the globe due to involvement of multiple ocular structures. The eyes are generally soft, they may have retained intraocular foreign body. X-ray orbit is required to exclude presence of radio opaque foreign body. Ophthalmic ultrasonography gives information like position of lens, vitreous haemorrhage, retinal detachment, presence of foreign body.

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Management consists of prompt repair of wounds as per standard method. Construction of anterior segment. Prophylactic use of broad spectrum antibodies is debatable. Once the endophthalmitis develops, it should be treated as any endophthalmitis. Differential diagnosis of post surgical and post traumatic endophthalmitis consists of endophthalmitis phaco anaphylactica, dislocated lens nucleus, large fragments of cortical material, unresolved haemorrhage, ciliochoroidal detachment, retinal detachment. Management of endophthalmitis I. Endogenous 1. Supportive treatment by cycloplegic and steroid locally. 2. Systemic antibiotic 3. Systemic steroid under umbrella of systemic antibiotic 4. Systemic antifungal 5. Vitrectomy II. Exogenous endophthalmitis (post surgical) is an ocular emergency that requires urgent and intense treatment by way of sub conjunctival and intra vitreal injection of antibiotic alone or with steroid as per standard dose. Role of vitrectomy is not yet clear. Panophthalmitis This is acute purulent infection of all the structures of the eye including Tenons capsule, extraocular muscles and orbital tissues. Panopthalmitis is generally bacterial in nature, all pus forming bacteria can cause pan opthalmitis. The organism reach the exterior of the globe following exogenous endophthalmititis which generally follow intraocular surgery or accidental penetrating wound. It may take few days for panophthalmitis to develop following penetrating injury surgical or otherwise. It may also result following perforation of a sloughing corneal ulcer. Xerophthalmic children and eyes with lagophthalmos are more likely to develop panophthalmitis. Sometimes the period may be few hours only and starts with malaise, headache, fever, pain in and around the eye. On examination the lids are swollen red and hot. The interpalpebral fissure is obliterated. Pus trickles through the lids, the conjunctiva is chemosed and hyperemic. Initially the cornea is steamy but later on develops purulent keratitis and sloughs, the anterior chamber is full of pus. Details of this structure beyond the hypopyon is not visible. The whole of the uvea is smothered with pus and the vitreous is almost a bag of pus, the lens may dislocate. The globe is immobile and proptosed. Absence of perception of light is the rule. In post operative eyes pus may exude through the section. It is sometimes difficult to differentiate between severe endophthalmitis and early panophthalmitis. The condition is to be differentiated from orbital cellulitis which generally does not involve the globe and vision is retained. Absence of perception of light and immobile globe is more in favour of panopthalmitis than endophthalmitis. Treatment of panophthalmitis is one of the most frustrating things. Broad spectrum antibiotic may suppress the infection. Visual recovery is almost impossible. The eye when untreated goes into phthisis after a prolonged course. The main role of antibiotics in panophthalmitis is to prevent septicemia, meningitis and cavernous sinus thrombosis.

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Definitive treatment is evisceration of the eye Masquerade syndrome51 This consists of a group of intraocular disease that are not uveitis per se but mimic uveitis in many ways hence diagnosed and managed as uveitis with disastrous result. They are seen mostly under 15 years of age in both the sexes, but may be seen as early as first year of life. Some of them are malignant and potentially life threatening, others may be non malignant growth or non inflammatory conditions. Two common malignant disorders are retinoblastoma and myelogenous leukemia. Medulo epithilioma is a locally invasive tumor, may be malignant. While juvenile xantho granuloma is a benign dermatological condition that causes spontaneous hyphaema in children. Intra ocular foreign bodies and peripheral retinal degeneration are two common non neoplatic condition that may present as uveitis. Other less common conditions are retinitis pigmemtosa, multiple sclerosis and post vaccination status. All the above conditions may cause aqueous flare, cells in AC, posterior synechia, cells in vitreous. Retinoblastoma is generally seen is children under five years of age with strabismus, redness, photophobia which on examination shows a posteriorly placed growth or may come with white reflex in pupillary area, congestion pain. KP flare cells in AC and accumulation of pus like fluid in AC (pseudo hypopyon) which is infact accumulation of tumor cells, posterior synechia, nodules on iris, tension is invariably raised. Pseudo hypopyon may disappear and uveitis subside with local steroid and cycloplegic only to reappear. X-rays shows intera ocular calcification ophthalmic ultrasonography may outline the tumor and intraocular calcification. Serum and aqueous LDH may be raised. Aqueous tap may reveal tumor cells. Leukemia : Acute myeloid leukemia may present as anterior uveitis in children. Spontaneous hypheama, heterochromia and glaucoma. Fundus examination may show Roths spot. Examination of blood gives clue to presence of leukemia. The child is generally ill and pale. Differential diagnosis consists of all causes of white reflex in pupillary area. Investigation includes : Routine Hb%, TLC, DLC, ESR, X-ray chest and orbit, ultrasonography, CT of orbit and MRI. Management depends upon causative factor. All cases of uveitis in children that improves with cycloplegic and steroid and recur should be suspected to be masquerade syndrome. New growth of uvea in children24, 42, 51, 74 New growths of uvea are rare in children, when present they are generally benign or locally invasive. However, malignant melanoma which is malignant growth of sixth decade can sometimes be seen in second decade. Such occurrences are extremely rare. However, malignant melanoma of iris is more common in children than adults. Benign tumor of the iris are hemangioma, may be associated with angioma of the lid and cause secondary angle closure glaucoma. Neuro fibroma of iris is seen as nodule on the iris in association with generalized neurofibromatosis. They do not require any treatment.

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Nevi are common tumors of the iris that make their presence felt just before puberty. They do not require any treatment, unless they start growing in size or there is change in pigmentation, which may be sign of malignant change. Juvenile xanthogranuloma73 is a benign dermatological tumor seen at the age of one year. The skin lesions are yellow multiple nodules. There may be visceral involvement. Ocular lesions are generally associated with cutaneous lesions. Ocular involvement areIris nodules, spontaneous hyphema, secondary glaucoma and uveitis. They lesions may involve all parts of the eye. The condition of self limiting. Hyphema and uveitis are treated with local steroids. Diktyoma75: This is a rare tumor, mostly arising from ciliary body but may arise from iris, retina or optic nerve. It is a congenital growth, commonest age of presentation is between two to four years. It is unilateral slowly progressive may remain confined to globe or may become locally invasive. Metastasis is rare, only occurring when the growth becomes extra ocular. It is also known as medullo epithelioma because it was thought to arise from medullary epithelium of fore brain. The term diktyoma is derived from its lace like appearance when it invades the iris.76 There are two types of medullo epithelioma - The teratoid and the non teratoid. The former besides ciliary epithelium contains hyaline, cartilage, rhabdomyocytes, brain tissue etc. Both types can be benign or malignant. One third of the medulla epitheliomas are malignant. On histology it shows rosettes similar to retinoblastoma. The tumor is very often mistaken as retinoblastoma because retinoblastoma is commonest ocular growth in the same age group. Medullo epithelioma may present as white reflex in pupillary area, secondary glaucoma and proptosis, which are common features of retinoblastoma as well. However, incidence of bilateral retinoblastoma is common presenting feature which is extremely rare in medullo epithelioma. There is no known genetic predisposition or known family history. Presenting features depend upon site, duration and location of the growth. It may only be detected accidentally when the child presents with diminished vision and squint. Otherwise common presenting features include diminished vision, pain and redness of the eye, whitish reflex in the pupillary area, buphthalmos and late proptosis. Diagnosis is difficult, however, unilateral growth without extra ocular involvement, absence of intra ocular calcification should arouse suspicion of medullo epithelioma. All cases should be examined with indirect ophthalmoscope. X-ray, CT, MRI, USG may help to differentiate medullo epithelioma from other disorders. Treatment There is no definite treatment77 for small growth of iris and cilliary body be cured by wide iridectomy or irido cyclectomy. Eyes with large tumors are best enucleated. Role of chemotherapy and radiation are not well established.

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55. Duker J.S. : Acquired immune deficiency syndrome in Current ocular therapy. Edition 5, edited by Fraunfelder F.T., Roy F.H. and Randall J., p. 5-7, W.B. Saunders Company, Philadelphia 2000. 56. Vrabee T.R. and Baldassano V.F. : Ocular manifestation of AIDS in Ophthalmology Secrets, first edition, p. 267-273, Jay Pee Brothers New Delhi 1998. 57. Faunfelders F.T. : Kaposi sarcoma in Current ocular therapy. fifth edition, edited by Fraunfelder F.T. Roy F.H. and Randall J., p. 241, W.B. Saunders Company Philadelphia 2000. 57A. Spitzberg D.H. : Pars planitis in Current ocular therapy. Edition fifth, edited by Fraunfelder F.T., Roy F.H., p. 517-519, W.B. Saunders Company, Philadelphia, 2000. 57B. Biswas J. : Intermediate uveitis in odern ophthalmology. Vol. 2, p. 571-579, edited by Dutta L.C., Jay Pee Brothers, New Delhi, 2000. 57C. Block-Michele and Nussen Blat B. : International uveitis study group : Recommendations for evaluation of intraocular inflammation diseases. AJO, 103-234, 1987. 57D. Knox D.N. : Disorders of uveal tract in Pediatric ophthalmology. Vol. II, Second edition, edited by Harley R.D., p. 528-529, WB Saunders Company, Philadelphia, 1983. 57E. Deborah Pavan Langstone : Uveal tract in Manual of ocular diagnosis and therapy. Third edition, p. 191-196, Lippincott. 58. Dunn J P : Ankylosing spondylitis and Reiters disease in Current ocular therapy, 5th edition, page 555-556, edited by Fraunfelder F.T., Hampton Company, Philadelphia 2000. 59. Roper Hall M.J. : Injuries in Modern ophthalmology. Vol. 3, First edition, p. 442-450, Butterworth, London 1964. 60. Kanski J.J. : Rare idiopathic specific uveitis syndrome in Clinical ophthalmology, 2nd edition, p. 169-170, Butterworth, London, 1989. 61. Duke Elder S. and Perkin E.S. : Disease of the uveal tract in Systems of ophthalmology, Vol. 9, p. 558-593, edited by Duke Elder S., Mosby, St. Louis 1966 62. Rao N.A. : Sympathetic ophthalmia in Retina. Editor Ryan NR, Second edition, 1729 35, Mosby 1994. 62A Rao N.A., Forster D.J. and Spalton D.J. : The uvea in Text book of ophthalmology. Editor Podos S.M., Yanoff M., Mosby, 1992 63. Moorthy R.S. and Rao N. A. : Sympthathetic ophthalmic in Current ocular therapy. Edition 5, edited by Fraunfelder F.T., Roy F.H. and Randall J., p. 320-322. W.B. Saunders Company, Philadelphia 2000. 64. Biswas J. : Common Panuveitic entities in Modern Opthalmology, Vol 2, edition II, p. 561- 598, edited by Dutta L.C., Jay Pee Brothers, New Delhi 2000. 65. Moorthy R.S., Inomata H., Rao N.A. Vogt Koyangi : Harada Syndrome. Sur Opthal 39 : 265-292, 1995. 66. Oku H. : Vogt Koyangi Harada Syndrome in Current Ocular Therapy, edition 5, page 293-295, edited by Fraunfelder F.T. Roy FH and Randall J., W.B. Saunders Company, Philadelphia, 2000.

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67. Kone Paul I., Yurdkul S. and Bahabri, S.A. : Clinical features of Behcets disease. J.Pedia. 134, 721-725, 1998. 68. Masuda K : Behcets disease in Current Ocular Therapy. 5th edition, p. 160-161. Edited by Fraunfelder F.T., Roy F.H., Randall J., WB Saunders Company Philadelphia, 2000. 69. Vinita Singh : Surgical treatment of Strabismus in Modern Ophthalmology, Vol. 2, p. 925, edited by Dutta L.C,, Jay Pee Brothers, New Delhi, 2000. 70. Kottow M.H. : Fluorescein angiography of the anterior segment in Principles and Practice of Opthalmology, Vol. 2, edited by Peyman G.A., Sanders D.R. and Goldberg M.F., Jay Pee Brothers, New Delhi, 1987. 71. Agrawal L.P. : Essentials of Opthalmology, first edition, p. 235, CBS Publishers and Distributors, New Delhi 2000. 72. Verma L., Venkatesh P. and Tewari H.K. : Management of Endothalmitis, CME Series4, All India Opthalmology Society. 73. Hunyor A.P. and Roberston J.E. : Bacterial Endothalmitis in Current Ocular Therapy, edition-5, p. 456-460, edited by Fraunfelder F.T., Roy F.H. Randall J., W.B. Saunder Company, Philadelphia 2000. 74. Wheeler D.T. : Juvenile Xanthogranuloma in Current Ocular Therapy, Edition 5, p. 239-241, Edited by Fraunfelder F.T., Roy F.H. and Randall J., W.B. Saunder Company Philadelphia 2000. 75. Duke Elders and Perkins E.S. : Disease of the uveal tract in Systems of Opthalmology, Vol. 9, first edition, edited by Duke Elders, Henry Kimpton London 1966. 76. Zimmerman L.E. : A critical reappraisal in light of new observation and current concept of embryopic tumors. Am. J. Oph. 72: 1039, 1971. 77. Apple D.J. and Pulkin J.E. : Leucokoria in Principles and practice of ophthalmology, Vol. II, p. 1181-1182, edited by Peyman G.A. Sander D.R. and Goldberg M.F., 1st Indian Edition, Jay Pee Brothers, New Delhi 2000. 78. O Keef M. : Medullo epithelioma in Current Ocular Therapy, Edition-5, p. 249, edited by Fraunfelder F.T., Roy F.H., Randall J., W.B. Saunder Company, Philadelphia 2000.

CHAPTER

Disorders of Lens in Children


GENERAL PRINCIPLE
Lens is a unique tissue in the body. It is solely ectodermal in origin. It has only one function i.e. to focus light on the retina. It is avascular, does not have any nerves hence lens is devoid of any sensation. It is not capable of getting infection, inflammation and degeneration or dystrophic. No new growth is known to develop in the lens. For its nutrition it depends exclusively on aqueous humour in postnatal period. There are only three possible disorders that can afflict the lens they are A. Change in transparency B. Change in shape C. Displacement. Disorders of lens are met from intrauterine life to ripe old age.

ANATOMY OF LENS1,2,3,4
The lens is suspended between the iris and the vitreous. The lens along with its suspensory ligaments divides the interior of the eye into two chambers, a large vitreous chamber behind and a smaller aqueous chamber in front. The posterior surface of iris is in contact with the anterior surface of the lens. The pupil is in front of the central point of the anterior surface around the anterio posterior axis of the lens. The iris glides smoothly over the anterior surface of lens in presence of light, accommodation and convergence. The lens is a disc shaped structure that looks circular when looked from front and back and elliptical when seen from the sides. Average diameter of the adult lens is 9.0 mm, at birth it is 6.00 mm to 6.50 mm, its maximum anterior posterior thickness is 3.50 mm to 4.00 mm in adults. The anterior and posterior surface differ in curvature, the anterior surface is less curved than the posterior surface. The anterior surface has radius of curvature of 10 to 11 mm and that of posterior surface is 6 mm. The curvatures of the lens is variable. In accommodation the curvature increases. It is more curved in infancy and child hood.

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The two surfaces merge into each other in a rounded equator. The central part of the anterior surface is called anterior pole while corresponding posterior point is called posterior pole. The imaginary line joining the two is called axis of the lens. Opacities in the posterior pole and posterior part of axialcortex cause more visual loss than on the anterior pole or anterior part of the axis, extensive, opacities on the periphery do not cause any visual change. The normal lens is for all practical purpose crystal clear in childhood. As age advances the colour becomes yellowish white that gives a false impression of cataract after fifty years of age. So long the colour of the lens does not interfere with vision it should not be called cataract. Anatomically the lens has following parts A. The capsule B. Epithelium C. Lens fibres 1. Cortex 2. Nucleus. D. Zonule A. The structure commonly referred to as capsule of the lens is not a true capsule. It is the basement membrane of the under lying. epithelium. It is a homogenous layer without any cells. It envelops the lens from all sides its thickness is not uniform. It is thickest at the equator, thinner at the anterior pole and thinnest in the posterior pole. This uneven thickness is due to metabolism of epithelial cells and amount of direct stress exerted on it. There are no cells over the posterior capsule, it is supported by the vitreous behind, does not bulge much in accommodation and force directed by contraction of zonules is minimal. There is a small depression in the anterior vitreous called patelar fossa; the posterior capsule rests in this fossa. In infancy and childhood there is an ill-defined adhesion between the posterior capsule and anterior vitreous fossa called capsulo hyaloid adhesion. This adhesion gradually disappears by fifth decade. In children any traction on the lens like attempted intracapsular lens extraction, repeated needing put undue traction on the vitreous that is transmitted to the retinal periphery resulting in traction detachment. The capsule has no healing capability of its own. B. The lens epithelium is metabolically most active part of lens. It consists of a single layer of cuboidal cells that are smallest in the centre of anterior capsule and gradually increase in length towards the periphery. The lens epithelium is limited in an area between the equator and under the anterior lens capsule. The posterior capsule is devoid of any epithelium. The future growth of the lens depends on the integrity of these epithelial cells. The shape of epithelium varies from place to place, in the central part they are polygonal, in prequatioreal area they are cuboidal, at equator they are columnar and gradually become conical with apex towards the centre of the lens. These cells form the lens fibbers. C. There are two types of lens fibbers (1) Primary lens fibres that develop from the posterior layer of the lens vesicle. These are ultimately converted to embryonic nucleus.

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(2) Secondary lens fibres are derived from the equatorial part of the lens epithelium2. The secondary lens fibers keep multiplying through out the life. The older fibers are pushed towards centre and are converted in to the nucleus. The central fibers are the oldest and peripheral are most recent. 1. The peripheral soft fibers are called Cortex while relatively hard fibers are called Nucleus. There is no histological demarcation between the cortex and the nucleus. It may be said, nucleus of present day is the cortex of yesterday5. 2. The nucleus of the lens. The nucleus of the lens is not a separate entity; it is mere condensation of centrally pushed lens fiber. The oldest fiber are deepest, the number of fiber keep on increasing throughout the life without much gain in size of the lens. According to period of development the nucleus has been assigned various names. They are (a) Embryonic (b) Foetal (c) Infantile (d) Adult. (a) The embryonic nucleus is the innermost and earliest to come into existence it is formed by primary lens fibre, initially there is a circular empty space surrounded by single layer of cuboidal cells. The posterior cells elongate to obliterate the space, as the posterior cells elongate the lumen becomes crescent shaped and becomes completely-extinct. (b) The foetal nucleus develops from the secondary lens fibber over the embryonic nucleus. The size of the embryonic and foetal nuclei do not change, in life they are pin point in size. (c) The infantile and adult nuclei are formed after birth. The infantile nucleus is completed before puberty, the adult nucleus develops there after. Size of adult nucleus keeps on increasing without much change in overall dimension of the lens. The lens fiber that are not converted into nucleus are known as cortex. So child has more cortex and small nucleus while a person in sixth decade has larger nucleus and scanty cortex. The nucleus not only enlarges in size it gets sclerosed as the person ages. The secondary fiber are not long enough to span the lens pole to pole, they do not meet at a point. The fiber in the same level meet in a peculiar fashion forming a Y shaped figure called. Lens suture. There are two sutures the anterior one is an erect Y while the posterior one is inverted Y. The sutures start forming in the first two months and seen in foetal life. The advantage of these sutures are that they give an elliptical shape and better optical property to the lens. (d) The zonules are entirely different from the lens, developmentally and functionally, their only purpose is to keep the lens in the papillary area and help change its curvature during accommodation. The zonules are infact tertiary vitreous. They extend from the apices of the ciliary process to the capsule. There are three groups of zonule. The anterior, middle and posterior attaching to anterior, middle and posterior part of equator respectively.

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The anterior zonule extends for a few millimetres on the anterior lens capsule. The posterior zonule may extent from oraseratta to equator of the lens. The tunica vasculosa lentis. The postnatal lens is avascular and gets its nutrition solely form aqueous. In first half of the gestation the lens gets nutrition from a rich framework of vessels that envelop the lens all round and is called tunica vasculosa lentis. Developmentally, it has two parts; the anterior vasculosa lentis and posterior vasculosa lentis. The anterior is a part of pupillary membrane while the posterior is formed by the terminal branches of hyaloid artery which is part of ophthalmic artery and enters the eye through the foetal fissure, it covers the developing posterior capsule and the equator. The tunica vasculosa lentis starts regressing even before the whole of the lens has formed. By fifth month it is fully atrophied.

DEVELOPMENT OF THE LENS


The lens is ectodermal in origin. Its presence becomes evident at 4mm stage7, when the optic vesicle comes in contact with the surface ectoderm. This contact between two surfaces triggers thickening in the surface ectoderm called the lensplate, at the same time another important change takes place in the optic vesicle that starts inveginating to form the opticcup a two layered structure from which the retina develops. The cells of lens plate elongate and increase in number and the single layered structure instead of spreading laterally bows inside forming a pitcher like structure, the mouth of which gradually narrows and by 8mm stage it closes completely and separates from the surface ectoderm. The surface ectoderm closes to form the subsequent corneal epithelium. The closed single layered structure separated from the surface ectoderm is called lens vesicle that starts moving away from the surface ectoderm towards the optic cup in the space between the two will later develop cornea proper, iris and AC. There is difference in development of cell forming the anterior and posterior walls of the lens vesicle, the anterior cells remain cuboidal while the posterior cells elongate forward to obliterate the space of the lens vesicle resulting into a solid globular structure called embryonic lens nucleus. The lens capsule that is a true basement membrane starts forming and by 13mm stage, the capsule envelops the growing lens fully. After formation of embryonic nucleus, formation of new lens fibber is shifted to the equatorial region . These fibber are called secondary lens fiber. As the fibers are formed the nuclei of elongated cells disappear this is one of the causes for clarity of the lens.The fibber meeting at the centre of the core form the anterior and posterior Y sutures. As more and more fibers are formed at the equator, the earlier fiber are pushed centrally and compressed to form various nuclei : the foetal , infantile and adult nucleus.

PHYSIOLOGY OF THE LENS8,9,10


Lens is avascular, depends upon aqueous for its nutrition. It has both anaerobic and aerobic metabolism. The lens contains 65% water and 35% protein, the highest concentration of protein in any tissue. Ninety percent of protein is water soluble, they are devided into three types alpha , beta and gamma crystalline and remaining portion is insoluble albunoid. The

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concentration of insoluble protein increases with age. In cataract almost all the protein is converted into insoluble albunoid. The lens protein is organ specific and not species specific i.e. a body sensitised to lens protein will behave adversely later if exposed to lens protein of any species. This has an important bearing in lens induced uveities and endophthalmitis phacoanaphylactica. The lens has a good concentration of ascorbic acid which is synthesised by the lens and ciliary epithelium. It also contains glutathion both in oxidised and reduced form. The amount of ascorbic acid and glutathion decrease with age and cataract formation. The transparency of the lens depends greatly on its metabolism and physiochemical status of protein.

CONGENITAL ANOMALIES OF THE LENS


Congenital anomalies of the lens are common but all need not be treated as many are asymptomatic. Anomalies that involve embryonic and foetal nuclei are true congenital and others are called developmental defects. Some of the congenital anomalies are obvious at birth, others are detected late due to associated diminished vision, squint and nystagmus. A. The congenital anomalies may be : 1. Limited to Lens 2. Associated with other anomalies of the eye 3. Associated with systemic disorders 4. Combination of all above B. Congenital anomalies can be : 1. Loss of transparency 2. Change in shape of the lens 3. Change in position 4. Various combination of above three. B. Congenital anomalies can be 1. Congenital loss of transparency. Loss of transparency complete or partial is clinically known as congenital or developmental cataract. They are in the form of opacities. The opacities that involve embryonal and foetal nuclei are called congenital while rest excluding traumatic are developmental. Many a times it is difficult to differentiate between the two. The congenital opacities are generally central, dens and may be large enough to cover the entire papillary area and, are generally soft. Congenital and developmental cataracts are often bilateral but can be unilateral. The cataracts in childhood are frequently stationary because the cataractogenic factor effects a particular zone of developing lens, the lens fibers laid before or after the stoppage of cataractogenesies remain clear, 25% to 30% of cataracts are hereditary how ever some time they may be sporadic.

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Etiology Etiology of congenital and developmental cataracts are not well understood it can be: (i) Idiopathic in 40 % to 50 % of cases. These are generally sporadic11. (ii) Heredity one third of all congenital and developmental cataracts are hereditary.12,13,14,15 due to genetically determined chromosomal anomaly. (iii) Maternal

Maternal infection during pregnancy commonest virus resulting in cataract is Rubella, others are cytomegalo inclusion virus, rubeola, mumps influenza. Toxoplasmosis is yet another cause of congenital anomaly if acquired during late months of pregnancy maternal syphilis can also cause congenital cataract.13 Maternal malnutrition specially fat soluble vitamins. Drugs : many of the drugs if taken during first trimester result in foetal cataract i.e. thalidomide, steroids. Maternal radiation during pregnancy. Endocrine disturbance.12 Birth Trauma Placental haemorrhage leading to poor oxygen supply to foetus. Metabolic disorders (i) Foetal (ii) Infantile

(iv) Foetal

Various syndromes Prematurity Persistence of anterior hyloid system Intra ocular tumours.

CLASSIFICATION OF CONGENITAL AND DEVELOPMENTAL CATARACT


tion16 It is difficult to have a uniform, universally accepted classification. A broad classificais dividing these cataracts into the broad categories as : A. Capsulo lenticular (Capsulo cortical) B. Lenticular Congenital cataract can be either nuclear or cortical. It is not uncommon for both to exist in the same eye. It is frequent for nuclear cataract to become total cataract. A. The capsulo lenticular cataracts are 1. Anterior polar and anterior capsular cataract. 2. Posterior capsularMittendorf dot. posterior lenticonous. 3. Posterior polar cataract.

296 B. The lenticular cataracts are 1. Zonular (a) Lamellar (b) Total Cataract (c) Cataracta central pulverulenta. (d) Membrenous 2. Sutural 3. Axial According to shape the catracts can be : 1. Disciform 2. Coraliform 3. Coronary

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4. OthersCorkscrew cataract, spear shaped cataract, Christmas tree cataract, blue dot cataract. A. The Capsulo lenticular cataracts 1. Anterior Polar and Anterior Capsular Cataract. Sometimes these are described separately but as their clinical presentation, pathogenesis and management are similar they are being discussed in one group. Anterior polar cataracts are common but not detected unless specifically looked for under magnification, are generally bilateral and do not block the pupil due to their size that may be pin point to 1 to 2 mm in diameter. They are generally small slightly raised plaque, may project in anterior chamber as a triangular growth with base on the centre of the anterior capsule and called pyramidal cataract they are of two type: (a) Developmental due to delayed separation of anterior lens capsule from the surface ectoderm. (b) Acquired. This occures frequently in badly managed ophthalmia neonatorum resulting in central perforation, loss of A.C., prolonged contact between anterior lens capsule and cornea. These are generally bilateral and associated with nystagmus. Sometimes there may be localised opacification of anterior lens capsule resulting into true anterior capsular cataract. More frequent are reduplication or imprint cataract where a second opacity develops little below the anterior polar cataract with a clear zone in between, the size of the reduplication cataract is either same as the base of the pyramidal cataract or larger. Sometimes there may be a third imprint still posteriorly. Occasionally the two opacities may be joined by a strand of opacity traversing the clear zone in between. The anterior polar cataract may be associated with persistent pupillary membrane or central corneal opacity. The opacities are stationary and do not cause much visual disturbance, hence do not need any treatment. 2. Posterior polar and posterior capsular cataracts. Posterior polar and posterior capsular opacities are as common as their anterior counterpart. There are two forms of posterior polar cataracts, one commonly known as Mittendorf dot. This is formed due to incomplete absorption of anterior part of the hyaloid artery that supplies nutrition to the lens during

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first three months of gestation. Some times a small stalk of vessel may remain attached to the lens. This condition is stationary and cause visual disturbance due to its proximity to the nodal point of the eye, how ever it is generally discovered and diagnosed during routine retinoscopy or direct ophthalmoscopy. It does not require any treatment. The other progressive form causes great visual loss as the opacity increase in size after birth by developing opacities in the posterior cortex either as a sheet of opacity or radiating opacities from a central opacity. The condition is not known to involve the nucleus16. If the opacity is dense enough it causes diminished vision, and amblyopia it should be operated by any standard microsurgical procedure.

CATARACTS INVOLVING THE NUCLEUS AND DEEPER LAYERS OF CORTEX


Most of these cataracts are developmental and have been put under category of lenticular developmental cataract2,16. The commonest in the group is lamellar or perinuclear also refereed as zonular cataract. 1. Lamellar cataract is commonest congenital cataract, it amount for about 50% of all developmental cataracts. It is generally bilateral, symmetrical and stationary, boys are more effected than girls, it may be prenatal or postnatal. There is a rough measurement to find out whether it is prenatal or postnatal which says that if the opacity is smaller than diameter of lens of new born i.e. 5.75 mm it is most probably prenatal otherwise post natal. Actual size varies according to on set of cataractogenesis and level of lens fibres involved. It most commonly develops in foetal and infantile nuclei in lamellar formation, the lamellae may be single or multiple. The opacity inside and out side the affected nucleus are clear. The opacity may be fully opaque or may be translucent. On the surface of the opacity many ridges are visible on oblique illumination. These ridges radiate from the centre and vary in number they may be limited at the periphery of the cataract or may project in the clear cortex with club or spine shaped projections called riders. Most of the lamellar cataracts are hereditary, transmitted as autosomal dominant trait may be present in parents or in siblings. The sporadic cataracts are generally due to derangement of calcium metabolism in the mother with vitamin D deficiency during growth of the lens, the opacity develops in a layer that corresponds with maximum level of noxious substance. The fibres laid before and after this cataractogenesis are clear. On examination the opacity may cover whole of the pupillary area, on dilatation a clear zone of the cortex is visible both on retinoscopy and ophthalmoscopy. Treatment depends up on size and density of the cataract. Translucent lenses with good vision need no treatment, some may benefit with dilatation of the pupil. Otherwise the treatment is removal of opacity by standard microsurgical method preferably with P.C.I.O.L. 2. Congenital nuclear cataract. This type may be confined to the embryonic nucleus due to changes at 3 months gestation. These are dens, powdery hence called pulverulent cataract which are generally bilateral, non progressive with out much visual loss.

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The other type is total nuclear cataract that may involve infantile, foetal or embryonic nuclei. The condition is bilateral, symmetrical and stationary may hamper vision. 3. Other cataracts (a) Sutural Cataracts. These are discrete opacities in the Y sutures of the embryonic nuclei. They are generally bilateral and stationary. One or both the sutures may be involved but need not be symmetrical. A type of sutural cataract seen in premature children are known to fade by third month post natal19. Other variants are : Floriform, (Coralliform) and anterior axial cataract. (b) Blue dot Cataract. This is very common congenital anomaly of lens. The opacities of various sizes, some of them visible only with slit lamp, others may be visible with bright oblique illumination. The opacities are scattered in the centre of the lens. They are not really coloured blue, they are white but due dispersal of violet light20 they look blue. The opacities do not hamper vision, do not cause any complication hence do not require any treatment. (c) Membranous Cataract. These are not separate type of cataracts. They result due to absorption of soft cortical matter in congenital cataract of long standing spontaneously or following trauma . The two capsules come close to each other with some opaque lens fibres in between without nucleus. They generally do not require any surgery but thick membrane may be needled folled by standard correction of aphakia.

RUBELLA CATARACT
Rubella cataract is caused by systemic infection of the mother by rubella virus in first trimester. Infection acquired after first trimester have very less chances of causing congenital defects in the foetus and subsequent pregnancies are not effected by the earlier infection. The rubella virus belongs to a group of toga viruses and has single antigenic type21. The virus is found all over the world with pockets of endemic areas, and can be prevented by prophylactic vaccine given in school going age. There is no antiviral agent known to be effective against rubella virus. The virus passes to the developing foetus via placenta and affects organogenesis of multiple organs, main being eyes, ear and heart. The infected mother has 50% chances of having child with congenital anomalies half of them will have ocular involvement12. The systemic manifestations in infected mother are mild and passed off as common cold with enlargement of occipital lymph glands. In utero the virus causes cells to have increased doubling time and short survival time. The growing lens develops areas of necrosis that loose transparency. The rubella cataract is central dense opacity to begin with, which becomes pearly and spreads to cover whole of the lens and may loose fluid later to become membranous. Hence in all cases of congenital membranous cataracts possibility of rubella should be kept in mind. The virus remains alive in the lens for months, may be as long as three years22 and when liberated in aqueous following surgical or accidental trauma is likely to cause sever and long lasting uveitis that it self may be sight threatening.

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Congenital rubella cataract is part of a syndrome of triad that consist of A. Congenital cataract B. Neurosensory hearing loos C. Congenital heart disease. Besides cataract there are other congenital anomalies of the eye they are : Microphthalmos, rubella retinopathy, iris hypoplasia, transient corneal haze. Congenital glaucoma is a common feature with congenital rubella cataract but is independent of pathological process of cataract formation. The systemic manifestation besides heart disease and neurosensory hearing loss are many. The neurosensory hearing loss is not always present at birth but may develop in preschool age. Bone lesions, hepatitis, thrombocyotopaenic purpura, hemolytic anaemia and occasional central nervous system involvement are infrequent systemic manifestation. Congenital rubella is known to cause still birth, prematurety and mental retardation making the management more difficult. D. Management of rubella Cataract 1. Prophylaxis in mother : Rubella is fully preventable disease following rubella vaccination between 12 to 14 years of age. If rubella vaccination is made part of universal immunisation the scourge of rubella syndrome with its morbidity and mortality can be abolished altogether. 2. There is no known medical treatment for the infected mother or child.. 3. Only definitive treatment of congenital cataract of (a) Surgical removal of Cataract (b) Management of aphakia (c) Prevention and treatment of amblyopia, squint and nystagmus (d) Associate glaucoma requires separate management.

CONGENITAL AND DEVELOPMENTAL CATARACTS DUE TO INBORN ERRORS OF METABOLISM CONSIST OF


A. Galactosaemia B. Hypoglycaemia C. Diabetes mellitus D. Homocytinuriaand other amino aciduria (Lows Syndrome) E. Hepato lenticular degeneration (Wilsons disease) F. Hypoparathyroidisionm. A. Galactosemia Galactosemia is one of the inborn errors of metabolism that causes developmental cataract. Galactose is not available separately as source of food. It is a metabolic product of lactose which is an important carbohydrate content of mammalian milk. The other source being endogendus in form of neurolipids in small quantity. The lactose is hydrolyzed in to glucose and galactose in the intestine, galactose itself is converted into glucose. In galactocemia

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this conversion of galactose to glucose is hampered due to deficiency of one of the three enzymes required to complete the metabolic cycle. A sugar alcohol that is responsible for cataract formation is formed that on entering the lens fibres cause a state of hypertonicity which attracts water to neutralise the alcohol leading to swelling of lens of fibbers, and opacification of the lens. The three enzymes involved in galactose metabolism are Transferase, galactokinase and epimerase. Commonest being transferees deficiency the epimerase deficiency is extremely rare. Transferase deficiency galactosemia is so frequent among other galactosemics that it is called Classical galactosemia The ocular components consists of bilateral central cataract also known as oildropelet cataract due to its appearance. The first change may be posterior lenticonous that on examination under slit lamp within few days of birth, show refractile opacities in the centre of the lens. Concentric refractile rings may develop round the central opacity. The opacity may initially look like a zonular opacity with clear zone all round. The opacity spreads rapidly to cover whole of the lens within few months. The progress of the opacity can be stopped or altogether reversed if milk and milk products are fully eliminated from the infants feeds. The systemic changes are serious and even life threatening they consists of vomiting, diarrhoea hepatospleenomegaly, hepatic and renal dysfunction, cirrhosis, anaemia, failure to gain weight, mental retardation. Glactokinase deficiency galactosemia is rarer than the former it is more benign26 has no systemic changes. The infant is healthy, the cataract develops in first few years of life. The cataracts are central and bilateral, their natural history is similar to transferase deficiency cataract except that kinase deficiency cataract is slower than the former. 1. Diagnosis of galactose cataract consist of (a) Clinical examination of the new born with diarrhoea, vomiting, dehydration and hepatio spleenomegaly (b) Examination of urine for reducing substances. (c) If reducing substances are present the urine should be examined for osazone crystals which are absent in galactosemia to exclude diabetes. The urine is subjected to chromatography for specific band denoting galactosemia, other specific tests consists of (d) Estimation of urinary and serum galactose. (e) Assay for uridyltranasferase in peripheral red cells. (f) Urine examination for albumin and aminoacids. (g) Hepatic function test. 2. Management. As soon as it is conformed that the child has galactosemia all mammalian milk products are withdrawn from the diet. This may retard or reverse the opacity. If opacity persists it is managed by any of the standard procedure.

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B. Hypoglycaemic cataract Hypoglycaemic Cataract in infant is a cataract developing in a hypoglycaemic infant or child. Hypoglycaemia in new born can result due to various causes in the infant. Generally an infant of a diabetic mother without biochemical control of hyperglycaemia, produces sufficient insulin to ward off foetal hyperglycaemia but as soon as the child is born and cord separated this increased insulin present in the child results in sudden lowering of blood sugar leading to acute hypoglycaemia which if not corrected cause formation of lens opacities which may not be detected at birth and may disappear if hypoglycaemia is corrected. Repeated attacks of hypoglycaemia lead to lamellar cataract by 1 to 2 years of age. Some neonates may be hypoglycaemic without mother being diabetic, incidence of hypoglycaemia is more in premature infants. Deprivation of sugar for first forty eight hours also result in hypoglycaemia, repeated diarrhoea is another cause of hypoglycaemia all these condition may precipitate hypoglycaemic cataract which is reversible if treated well in time. C. Diabetic cataract Diabetic cataracts are not seen in new born they are generally met with in juvenile diabetics. It is an acute cataract due to accumulation of glucose in the lens. The accumulated glucose is converted into sugar alcohol which imbibes water and over hydrates the lens , these cataracts are bilateral. To begin with they are snow flake like opacities under the capsule, diabetic cataract has been seen in children as young as one year. Initially these cataracts are reversible if water electrolyte and metabolic disorders are corrected within first two or three days. Management of diabetic cataract is like any other cataract of childhood done with proper control of hyperglycaemia. D. Cataract of childhood in miscellaneous errors of metabolism26,27,28 1. Hypoparathyroidism. Bilateral lens opacities in the shape of punctate opacities and multicoloured crystals in the cortex are seen in untreated cases of long duration of hypoparathyroidism. Progress of cataract can be prevented by treatment of hypoparathyroidism. 2. Manosidosis. Spots like opacities in the posterior capsule is caused due to deficiency of mannosidase. 3. Fabrys Disease. Caused due to lac to galactosidase. It results in spoke like opacities in cortex without disturbance of vision. 4. Lowes syndrome. Also called oculo cerebro renal syndrome effect mostly boys. Cause congenital cataract associated with congenital glaucoma. The lens is microphakic. Lens opacities may be capsular, lamellar, nuclear or total, these children have mental retardation. 5. Wilsons disease (Hepatolenticular degeneration) This is due to inborn error of copper metabolism. Lenticular changes cause anterior capsular sunflower cataract. 6. Homocystinurea (see ectopia lentis)

CHANGE IN SIZE OF THE LENS


Microspherophakia Normal diameter of an adult lens is 9 mm and that of new born is 5.75 mm. The edges of the lens are not visible even with maximum mydriasis. The infantile

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lens gradually increases to adult size in the second decade. There is no condition where the lens has congenitally larger diameter Microspherophakia29 is a congenital anomaly of the lens where the average diameter of the lens is 6.75 as against 9mm of an adult lens. The anterior posterior diameter of the lens is 25% more than normal. Reduced equatorial diameter and increased thickness of the lens gives it an appearances of a spherical lens with small diameter hence the name microspherophakia. The condition is bilateral. The child is brought due to diminished distant as well as reading difficulty. The cornea is of normal size so is the globe. The anterior chamber may be deep on the periphery with mild iridodonesis the real diagnosis is made on dilatation of the pupil. With a widely dilated pupil the periphery of the lens gives a crescent like reflex. On retinoscopy the central part shows high myopia with or without astigmatism. On slit lamp examination the zonules are visible all round in early years but later on there may be rupture of some of the zonular fibres, resulting is subluxation which is common. A peculiar phenomenon called glaucoma inverse results if the pupil is constricted either due to prolonged exposure to bright light or use of miotic. The spherical lens obstructs the miotic pupil resulting in pupillary block and rise of tension. There may be associated angle closure. The tension falls with mydriasis. The lens may occasionally come in the anterior chamber. Generally there are no other congenital anomalies of the globe. The condition may have only ocular component or may be associated with systemic anomalies resulting in Weill-Marchesani syndrome. The exact cause of the deforming is not known. It is presumed that it results due to arrest of development of lens during 5th or 6th month of gestation. Management of the condition is difficult due not only to index myopia, poor accommodation but also secondary pupillary block glaucoma. Besides correction of index myopia a constant watch should be kept on possibility of pupillary block. Prophylactic iridectomy or iridotomy, may prevent rise of tension, use of mydriatic is better avoided because the lens may subluxate in the A.C.. Cataract is common and should be managed like any other cataractus ectopia lentis.

CONGENITAL ANOMALIES OF SHAPE OF THE LENS


There are two types of anomalies of shape of the lens due to congenital cause. A. Lenticonus.31 B. Coloboma of the lens. A. Lenticonous is a condition where the poles of the lenticular capsule bulge out side the confines of the capsule. If the projection is anterior where the lens bulges in the anterior chamber the condition is called anterior lenticonous and when similar changes appear in the posterior capsule with bulge extending into the vitreous the condition is known as posterior lenticonous. The terms lenticonous and lentiglobus are used as interchangable terms. Posterior lenticonous is more common. The cone is situated within 5mm of the posterior pole, however it can be eccentric or peripheral. There is a very rare condition where the posterior cortex develops a local bulge with in the confines of the capsule32.

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The anterior lenticonous are bilateral while posterior lenticonous are unilateral. Changes incurvature of lens is seen equally in both sexes. The exact cause is not known. There may be a history of lenticonous in the family lenticonous may be associated with many systemic congenital anomalies. The condition may not be diagnosed unless the child is brought for improvement of vision or correction of squint which is common. The cause of diminished vision is curvature myopia. amblyoipia is common. On examination there is an oil drop appearances. with dilated pupil The change in curvature is visible on slit lamp. On retinoscopy there is central myopic and peripheral hypermetropic refraction, scissors movement are common. The lens shows progressive opacity in the posterior cortex in the cone and around the cone. Combination of posterior lenticonous and posterior cortical cataract is so common that all cases of unilateral progressive opacification of posterior cortex in an eye with normal dimension should be investigated for possibility of posterior lenticonous. If the media is dense and the bulge is not visible on slit lamp, U S G may delineate the bulge. Management. If there is not much of visual loss, spectacles, contact lens and mydriatics my help. However danger of amblyopia is always present that should be managed as per standard methods. Dense opacities can be managed either by limbal or pars plan route with posterior capsulotomy anterior vitrectomy and IOL. B. Colobama of the lens Coloboma of lens depends upon development and integrity of the zonules. In what is called coloboma of lens, there is no actual loss of substance of the lens matter as the name suggests. A notch in the lens is very rare. There is flattening of the circumference. Generally it is situated at down and in position, a site common for uveal coloboma. The coloboma is generally not visible if the pupil is circular and requires myriasis to be visible. On slit lamp examination zonules are generally absent at the site of coloboma. Coloboma of the lens is usually unilateral, the lens is generally partially cataractous, adjacent to an area of coloboma. It may involve the cortex or adult nucleus and not embryonic or foetal nuclei. As the area of coloboma is deprived of normal pull of the zonule, it is thicker and the periphery is more rounded than normal lens.33 The lens generally has curvature myopia with astigmatism and poor power of accommodation. Occasionally there may be a dent in the lower equator34. The condition is to be differentiated from subluxation of lens the only difference between the two is that colobomalous lens has flatter periphery while subluxaled lens retains usual circular curvature of the equator. One of the complications of colobomatous lens is subluxation. Coloboma of the lens does not require any specific treatment.

CONGENITAL ANOMALIES OF ZONULE


Congenital anomalies of zonules are not uncommon but they are generally not discussed as separate entity simply because the result of such anomaly are reflected as anomalies of the vitreous. The anomalies can be 1. When the zonules are weak alround, they result in

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speherophakia. 2. When the defect is localised the result is a coloboma of the lens, where the lens does not subluxate 3 when the defect is excessive on one side the lens gets sub luxated to the opposite direction 4. if the zonules are lacking all-round the lens is dislocated.

CONGENITAL ANOMALIES IN THE POSITION OF THE LENS


A. Ectopia lentis Next to cataract, displacement of lens is most common congenital anomaly of the lens. It may be mild enough to be missed unless examined with slit lamp under complete mydriasis. The congenital displacement is generally bilateral and nearly symmetrical most of the time the displacement of the lens in the one eye is mirror image of the fellow eye. 1. A lens is said to be ectopic when (a) Its centre is not in the anterio posterior axis of the eye. (b) Some of the zonule are still attached to the lens. (c) The lens is behind the iris. (d) Partly visible in the pupil. (e) The edge of the lens in the pupillary area dividing the pupil into two parts:- one phakic which is generally myopic and the other area aphakic that is hypermetropic. 2. A lens is sub luxated when some of the zonules are still attached to the lens capsule. 3. A lens is dislocated when all the zonules are severed from the lens and the lens can move in the (a) Anterior chamber (b) Incarcerted in the pupil (c) Bobbling in the vitreous (d) Fall at the bottom of the vitreous (e) Get fixed to the retina. Unilateral ectopia is rarely congenital it is mostly due to trauma or other ocular causes like chronic uveits . In all cases of unilateral ectopia lentis the other eye should be carefully examined for subtle signs of ectopia, and systemic cause of ectopia lentis ruled out. B. Symptoms of ectopia lentis depend mostly on 1. Area of the lens present in the pupil, 2. Tilt of the lens, 3. Transparency of the subluxated lens 4. Associated error of refraction and 5. Complications like amblyopia, glaucoma, retinal detachment. 6. Associated systemic condition.

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C. The symptoms can be divided into Mild, moderate, gross and associated complications. 1. A lens that has subluxated but its edge lies in between the pupillary margin and iris root may not produce any visual symptom, may have normal accommodation of the age. Occasionally there may be diminished vision due to errors of refraction independent of subluxation, or due to subluxation i.e. myopic astigmatism. 2. A lens that divides the pupil in two halves, one aphakic and other phakic causes moderate visual symptoms of unilateral diplopia and fleeting visual disturbances. The phakic part is myopic or myopic astigmatism with accommodation. The aphakic part which is hypermetropic and has both diminished distant as well as near vision. Bilateral moderate subluxation may cause polyopia. 3. The edge of a subluxated lens that is not visible in undilated pupil causes gross loss of distant as well as near vision without diplopia because the whole of the pupillary area is uniformly absolute hypermetropic. 4. Ectopia lentis with associated complication have various grades of visual loss due not only to ectopia but also associated lesions like glaucoma, retinal detachment etc. (See below) D. Signs of Ectopia lentis consist of 1. Diminished distant vision that may or may not be fully corrected by glasses or contact lens. 2. Diminished near vision 3. Uniformly or partially deep A.C. 4. Generalised or localised tremulousness of iris 5. Pupillary shape and size are variable, depending upon other associated and anomalies. 6. The edge of the lens may give a crescent shaped reflection on retinoscopy; the phakic area is myopic whereas the aphakic area is hypermetropic. 7 On fundus examinationdoubling of the fundus i.e. a large area seen through the phakic myopic area and a small area of fundus seen through hypermetropic area. 8. The refraction may not be symmetrical and equal in both the eyes. 9. The eye invariably has strabismus in unilateral cases and also when the refraction is grossly different in both the eyes. 10. On slit lamp examination the zonules may be visible in the aphakic area and vitreous may herniate though the aphakic area. 11. Complication (a) Raised intra ocular tension (b) Myopic degeneration (c) Rhegmatogenus retinal detachment 12. Associated anomalies (a) Mesodermal dysgenesis of anterior chamber (b) Megalocornea.

306 (c) Cornea plana (d) Persistent primary hyperplastic vitreous (e) Retinitis pigmentosa (f) Congenital glaucoma. (g) Keratoconus (h) Ectopic pupil (i) Aniridia (j) Ptosis. 13. Associated systemic anomalies (a) Marfans syndrome. (b) Homocytinuria. (c) Weill Marchesani syndrome (d) Reigers anomaly (e) Crouzons syndrome (f) Oxycephaly (g) Hyperlysinemia (h) Sulphite oxidise deficiency (i) Ehlers - Danlos syndrome (j) Mandibulo facial dysostosis. (k) Sturge Weber syndrome.

PEDIATRIC OPHTHALMOLOGY

SIMPLE ECTOPIA LENTIS


Simple ectopia lentis represents isolated ectopia without any other ocular or systemic disorder. It is generally familial, transmitted as autosomal dominant inheritence.35 It may rarely be sporadic, the lens is subluxated up and out. On set of subluxation is gradual and may go unnoticed unless looked for specifically in a child with positive family history or the child is brought with complaints of diminished vision or squint. They generally have good distant as well as near vision for many years but may develop anterior dislocation, glaucoma, cataract or retinal detachment36.

ECTOPIA LENTIS et PUPILLAE37


This is transmitted as autosomal recessive inheritance, is bilateral symmetrical, the normal pupil is replaced by a slit like or oval opening complete with constrictor and dilator muscles, the pupil is shifted opposite the shift of the lens i.e. if the lens is displaced up and out , the pupil is shifted down and in. The edge of the pupil generally bisects the pupillary slit. The angle of anterior chamber is clear. The condition may be considered as mildest form mesodermal dysgenesis.

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MARFANS SYNDROME38,39,40
Marfans syndrome is an autosomal dominant disorder of mesodermal tissue with multi systemic involvement. It is seen equally in boys and girls with world wide distribution. Though the condition beings in infancy its full blown presentation is felt in teens. It has been reported to run in generations and common among the siblings. The disorder does not have any biochemical deficiency that may be linked to the anomaly. The syndrome has following component, all of which need not be present simultaneously: A. Ocular B. Skeletal C. Cardiovascular D. Miscellaneous A. Ocular Most important, frequent and prominent ocular changes are seen in the lens. The changes are bilateral almost symmetrical. 1. The lens. The lens is subluxated upwards in both eyes; they are mirror images of each other. The subluxation is gradual but does not progress much. Occasionally the subluxation may increase and the lens may even dislocate apparently without any reason. Common predisposing factor being trivial blunt injury, subluxation may occur at any age. The lens is generally clear but may have localised opacification at the lower equator. Generally the lens is slightly smaller in diameter it may be microspherophakic. It is usual for patient to have normal accommodation for the age but if the subluxation is more i.e. the aphakic portion covers lower half of the undilated pupil, the child has difficulty in near work. The subluxated lens gives a crescentic dark reflex on oblique illumination due to reflection of light from rounded edge of the clear lens. 2. On retinoscopy the phakic area generally gives a compound myopic astigmatism while the aphakic portion has hypermetropic refraction generally less than +10D. 3. On ophthalmoscopy duplication of fundus is seen. The fundus seen from the phakic area is generally larger while seen through the aphakic area is smaller than emmetropic fundus. The eyes commonly have axial myopia with corresponding myopic fundus changes. 4. On slit lamp examination the zonules are visible some of them may be broken, the lens rarely shows a scalloped lower border or a notch. 5. The anterior chamber is irregularly deep, deeper over the aphakic part and shallow or normal over the phakic part. The lower iris is generally tremulous, occasionally there is phacodonesis. 6. Pupil. The pupil is smaller than normal due to poor development of dilator fibbers. The pupil is resistant to usual mydriatic and cycloplegic. The pupil retains normal light and near reflex. 7. Iris. The surface of the iris is relatively smooth. 8. Angle of A.C. Gonioscopy shows wide angle with multiple changes that include ill defined Schwalbes line.

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9. Cornea. The cornea is normal but may show megalo cornea, keratoconous or even cornea plana. 10. Sclera. The sclera is thin and stretched may have small staphylomas. 11. Error of Refraction. If there is gross difference in refraction between the two eyes there may be squint and ambloyopia, rarely their may be ptosis. 12. Glaucoma: Eight percent of the eyes have developmental glaucoma that is generally open angle glaucoma rarely the lens may be entrapped in the pupil causing pupillary block glaucoma. B. Skeletal Changes Skeletal Changes are prominent and may be the first cause to bring the child to the physician. The child is tall and thin for age, most striking are long slender limbs, the span of out stretched arms is more than the height of the child, the fingers are long, spidery hence called arachnodyctalous. The tip of the thumb passes beyond the ulnar border of the palm when flexed. The thumb and index finger of one hand when wrapped round the wrist of the other hand, over lap each other. The thumb can be extended back wards to touch the radial border of the forearm. The metacarpals and phalanges are long and thin. The large joints are prone for subluxation. The head is often dolicocephalous with high arched palet.The spine shows scolio kyphosis. Occasionally there may be hemivertebrae, and spinabifeda. The chest is long and narrow, the sternum shows pactus deformity.The muscle are hypoplastic, the skin is loose without subcutaneous fat, weakness in abdominal muscles predispose hernia formation. C. Cardiac changes Cardiac changes begin with dilatation of aorticroot, leading to aortic insufficiency, fuciform aneurysm of aorta is common and the aneurysm may show dissecting changes. D. Other changes consists of Winged scapulae, flat foot, contracture of joints, pulmonary and renal anomalies, malformation of ears. The children have normal I.Q and there is no biochemical changes. E. Management Management depends upon severity and duration of the condition it consists of:Improvement of vision 1. Some children may not have much visual disturbance inspite of subluxation if there is no error of refraction. 2. When error of refraction is present it is generally axial myopia, because the aphakic is exclude from the pupil due to small, rigid central pupil. 3. With a large aphakic area in the pupil it should be decided if, the child will benefit by minus glasses with constricted pupil or aphakic correction with dilated pupil. Aphakic power is considerably counter balanced by presence of axial myopia hence it is invariably less than + 10D.

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4. The lens should not be tempered with unless it is : Cataractous, dislocated or causing glaucoma. (a) The lens is generally removed through limbal route. (b) The posteriorly dislocated lens is removed through parsplana with extensive vitrectomy. (c) Aphakia is managed by spectacle or contact lens. It is not possible to put an IOL in the bag in subluxated lens following phacofragmentation. The options open are- iris clip lens, lens in the sulcus or sclera fixated lens all which has their advantages and disadvantages. 5. The retinal detachment can be as a part of axial myopia or vitreous disturbance. It may require extensive surgical procedures.

HOMOCYSTINURIA
Homocystinuria is second most common cause of bilateral subluxated lens. Its skeletal and ocular features have many similarities with that of Marfans syndrome. Important differentiating point are:- Homocytiruria is associated with mental retardation, gives positive bio chemical test for uninary homocystine, and is autosomal recessive in nature41 However it is of utmost importance that the two conditions be differentiated because symptoms of homocystinuria can be eliminated by proper diet and medication. Homocystinuria is one of congenital errors of metabolism due to deficiency of more than one enzyme i.e. methionine metabolism42. The main causes of the clinical features are excess of methionine (an essential amino acid) and homocystine in blood. The patients excrete large amount of homocystine in urine. The syndrome is caused mostly due to lack of cystathionine B. synthase that helps to convert methionine to cystine . The result is rise of methionine and homocystine in body fluid. The disorder is equally common in boys and girls. The clinical signs are not noticed at birth. The condition manifests itself by eight to ten years. However the child may show signs of delayed physical milestone from early age. The mental retardation becomes obvious as the child goes to school. This is initially attributed to illhealth and diminished vision. The subluxation of lens becomes apparent by tenth year. The condition has following components: A. Ocular B. Skeletal C. Mental D. Cardiovascular E. Positive biochemical test. A. The ocular changes comprise of 1. Lens. Bilateral progressive subluxation of lens that has the tendency to fully dislocate. The accommodation is poor or absent. As the zonules are broken which may be matted

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over the lens surface39. The shape of the lens may be small and spherical. The lens may develop lamellar opacification.One of the initial presentation may be painful acute loss of vision due to pupillary block or endothelial damage caused by the lens dislocating in A.C. . If the lens dislocates in vitreous it may start phacogenic uveitis and glaucoma or may remain silent. The aphakia produced due to subluxation or dislocation compensates pre existing axial myopia, thus the aphakic power is generally less than +10 D. 2. Iris. The iris is generally tremulous over the aphakic part. Occasionally there may be aniridia which makes diagnosis more difficult. 3. Sclera. The Sclera may be stretched and thin .May develops staphyloma due to associated buphthalmous. 4. Retina. There is high incidence of retinal detachment mostly due to myopia. However aphakic detachment is very common following lens exaction. 5. Vision. Most of the children have diminished distant vision due to: (a) Error of refraction (b) Glaucoma (c) Squint (d) Amblyopia B. The skeletal changes are The skin is light coloured, with malarflush, the check bones are flat, and the skin is dry. The limbs are long, the fingers are spidery (arachnodactyly), Joints are prone for subluxation. Feet are flat with floppy gait. The spine shows kyphosis and scoliosis. Osteoporosis is common leading to fractures. Hernia is common due to under developed abdominal muscles. C. The mental changes consist of Low IQ and other psycho somatic disorders are common. However some of the children may have normal intelligence. D. Cardio vascular changes consist of Various congenital anomalies of heart. Thromobo embolic episodes are common leading to-cerebrovascular accidents. Myocardial infarction in early adulthood and pulmonary embolism are frequent. All these factors make these children very poor surgical risk. They have high rate of death during general anaesthesia. The child with homocystinura generally does not live beyond third decade. E. Biochemical test43, 44 Cyanide sodium nitropruside test is a good secreting test but not conformatory due to high rate of false positive results.

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The test consists of: Adding two ml of 5% sodium cyanide to 5ml of fresh and acidified urine. This mixture is allowed to stand for ten minutes, then two to four drops of sodium nitropruside is mixed to the previous solution. This gives bright red colour which is common both to homocystine and cystine present in urine. To confirm the diagnosis of homocystinuria electrophoresis is required. Bacterial contamination of urine of normal child may also give false positive result. F. Management Management of homocystinuric child is more complicated due to : (1) Associated mental changes which may hinder visual rehabilitation. (2) Increased risk of thromboembolic phenomenon that makes them poor surgical risk. (3) Only positive point in homocystinuria is that the condition can be helped by : (a) Restricting dietary methonine and supplementation of oral cystine42. (b) High doses of vitamin B6 (Pyridoxine). The dose recommended is 600 mg to 1200 mg daily and folic acid orally. (c) Those individuals not responding to pyridoxine may be put on Betaine that reverses degradation of methonine. F. Ocular Management is similar to any other ectopia lentis. G. Mentally retarded children may have to be put in institutions meant for mentally and visually challenged.

XVII. WEILLMARCHESANI SYNDROME45a,45b,45c


This is the third of the three common disorders that cause ectopia lentis with systemic involvement. It is rarer than Marfans syndrome and homocystinuria both of which have many common features. The only common feature of Weill Marchesani syndrome with the former two is ectopia lentis. It is an inherited disorder of mesoderm. It may have both types of inheritance i.e. autosomal dominant or recessive. The components of the disorder are A. Ocular B. Non ocular A. The Ocular features are 1. Microspherophakia where the lens is small in equatorial diameter but has greater than normal anterio posterior diameter. The shape of the lens is not apparent unless the pupil is dilated, with dilated pupil the lens has a circular bright crescent on the periphery. The zonules are visible on slit lamp examination. The lens has tendency to move anteriorly rather than upward or downward. This results into a ball and socket pupillary block. The block is enhanced by use of miotics. The spherophakia results in index myopia. Occasionally the A.C. is shallow and tremulousness of iris is better seen on the periphery. The lens may completely

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dislocate in anterior chamber with its resultant complications. Posterior dislocation is less commaon. 2. Secondary pupillary block glaucoma is most troublesome complication. As the pupil constricts over the spherical lens a pupillary block results that shuts off the flow of aqueous from posterior chamber to anterior chamber, resulting in shallowing of A.C. and narrowing of angle which itself may have strands of mesodermal tissue. The tension is relived flowing maydriasis or establishing a path between A.C. and P.C. by iridectomy or laser iridotomy. B. Non ocular signs The non ocular signs are striking. The patients have a short stubby stature. Their maximum height seldom exceeds five feet and many are considered to be dwarfs and investigated as such. They have brachycephly, well developed subcutaneous fat, muscles are hypertrophied, arms and legs are short, the hands and feet have squarish shape, and the joints are prominent and stiff. The thorax looks larger as compared to the body. They have tendency to develop carpal tunnel syndrome. There is no fixed pattern of cardiac disorder, the children have assorted cardiac anomalies, there is no mental retardation, life expectancy is better than in homocystinuria and no specific biochemical change have been attributed to the conditions. Management Consists of 1. Correction of error of refraction. 2. Prevention of glaucoma by doing prophylactic surgical peripheral iridectomy or laser iridotomy. 3. Management of lenticular opacity by standard microsurgical procedure. 4. Removal of dislocated lens. Comparison between Marfan, homocystinuria and Weill-Marchesani syndrome.
Marfans Syndrome Inheritance Ocular Changes Lens Sub luxate up and out Non progressive may become cataractous have tendency to dislocate Small resistant mydriatic to Sub luxale down and may dislocate Spherophakia displaced in anterio posterior direction may dislocate Autosomal dominant Homocystinuria Autosomal of recessive Weill-Marchesahi syndrome Intermediate

Pupil Iris

normal No Specific changes except iridodonesis

normal No specific change except iridodonesis

Poorly developed dilator muscles, loss of pattern iridodonesis.

DISORDERS OF LENS IN CHILDREN A.C. Deep in lower part shallow in upper part Pupillary block, phacogenic or due to mesodermal change in angle Axial myopia, myopic astigmatism Retained Retinal detachment rhegmatogenous or traction Deep in upper part shallow in lower part Non-specific

313
Irregular, iris may bulge in A.C. due to pupillary block Inverseglaucoma

Glaucoma

Refraction Accommodation Retina

Axial myopia, myopic astigmatism. Lost early Rhegmatogenous or traction retinal detachment

Index myopia Variable No specific change

Skeletal Changes Height Skull Spine Thorax Subcutaneous Tissue Musculature Limbs Fingers Joints Cardiovascular Change Common Aortic valve dilatation, aneurysm Thrombi embolic episodes Intelligence Life span Biochemical test Nil Normal Normal Nil Variable Common Low Short Sodium Cyanide test Positive Variable Nil Normal Normal Nil Tall, Slender Dolicocephalic Scoliosis, kyphosis Long slender Poorly developed Hypoplastic Long thin Arachno dactyly Loose, hyper extensile Tall Slender Dolicocephalic Scoliosis kyphosis Long slender Poorly developed Hypoplastic Long thin Arachno dactyly Loose hyper extensile Short Stubby Brachycephic No Specific Change Short broad Well developed Hypertrophied Short broad Spade like stiff

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OTHER SYNDROMES WITH ECTOPIA LENTIS


A. Ehlers Danlos syndrome B. Hyperlysinemia C. Sulphite oxidase deficiency A. Ehlers Danlos syndrome is a rare disease generally seen in adults but becomes apparent in childhood, may be noticed in infancy, is caused due to inherent defect in collagen. Its two systemic findings are hyper elasticity of the skin that ruptures on slightest trauma, causing extensive ecchymosis and hematoma. The other systemic presentation is hypermobility of joints. The ocular features are Sub laxation of lens in both eyes, thinning of cornea, kertoconous, microcornea, megalocornea, blue sclera. Angioid streak is a common feature in fundus. Stretching of cornea and sclera predispose laceration of eyeball. Ruptures in Bruchs membrane and stretching of retina result in retinal detachment. There is no biochemical defect, intelligence and life span is normal. There is no specific treatment. Hyperlysinemia45. This is an inborn error of metabolism due to lysinedehydrogenase deficiency. Its ocular manifestations include ectopia lentis, microspherophakia, may have ophthalmoplegia. These children have low IQ and have retardation of growth and laxicity of joints there is no specific treatments. Sulphite oxidase deficiency In the broad sense this is derangement of cystine metabolism due to sulphite oxidase deficiency. The children have short life span and mental retardation there is bilateral dislocation of lens. No specific treatment is known.

OCULAR CAUSES OF ECTOPIA WITH OUT SYSTEMIC INVOLVEMENT


A. High myopia B. Buphthalmos : Primary or Secondary. C. Keratoglobus D. Aniridia E. Reigers anomaly F. Uvitis G. Blunt trauma, (Generally unilateral rarely bilateral.)

PAEDIATRIC CATARACT
Incidence and prevalence of paediatric cataract is far less than adult cataract however cataract in childhood has far-reaching consequences. Adult cataracts have excellent visual

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result following lens extraction, same is not true with childhood cataract. Childhood cataract surgery has poorer visual prognosis and more complications. It is estimated that congenital cataract is found roughly one in every 250 live births46 Very few have clinical symptoms, only few require treatment, incidence is higher in developing countries where hardly any prophylactic measures are taken for maternal rubella, and correction of maternal malnutrition, incidence also increases due to consanguinity47. Congenital cataract is a leading cause of blindness in children about 20% of blindness in children is due to congenital cataracts46. In one third of the cases of cataract no cause can be detected. In developing countries trauma is a major cause of cataract in children. Congenital and developmental cataracts have a strong heredity. However sporadic cases are also common. Though most of the congenital cataracts are bilateral, many of the children present as unilateral cataract, in such cases the other eye must be examined in detail for subtle signs of cataract. Unilateral cataracts have poorer prognosis as compared to bilateral cases. Trauma and intraocular diseases are common causes of unilateral cataracts. A. The cause of congenital cataracts can be 1. Commonest cause being maternal rubella in first trimester, other causes can be maternal diabetes, parathyroid disorder and malnutrition. 2. In another group of children, the mother is normal but the child has various errors of metabolism i.e. galactocimia, hypoglycaemia, homocystinuria and other aminoaciduria. 3. There are numerous syndromes associated with congenital cataract. Some frequent syndromes are Lowes syndrome, Alports syndrome and Turners syndrome. 4. Congenital cataract may be the only anomaly or else it may be associated with other maldevelopments of the eye. 5. All opacities in the lens do not cause visual disturbance and may go unnoticed till later life, only to be discovered on routine examination. B. Symptoms of congenital and developmental cataract46, 48 Symptoms depend on 1. Position of the opacity. Visual loss is more in central cataracts than peripheral, opacity near the nodal point is likely to cause more visual disturbance than one away from it. 2. Size of the opacity. Opacities larger than normal pupil cause more visual loss. The matter is worsened when pupil constricts in bright light or the child has moitic pupil due to any cause. 3. Number of the opacities. Scattered small opacities hardly produce any symptom but nuclear opacity with posterior capsular opacification cause more visual loss than any one of them. 4. Density of the opacities. Denser opacities cause more loss of vision than translucent opacities. 5. Unilateral cataracts. They may go unnoticed if the other eye has good vision; these eyes develop amblyopia and squint more frequently.

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6. Age of on set. Congenital cataracts that are apparent before three months of age cause intractable nystagmus. 7. Associated ocular malformation. Microphthalmos, persistent hyaloid system, persistan primary hyperplastic vitreous, retrolental fibbroplasia, Reigers anomaly have poorer vision and prognosis. 8. Associated with symptoms of other systemic syndromes. 9. Physical under development is a frequent associated feature of congenital cataract. 10. Frank mental retardation is common in homocystinuria. Even children with normal IQ may fail to attain expected academic grades due to poor vision. C. Presenting Features of Congenital and Developmental Cataract Various age groups have different presenting features. 1. An infant may be brought with following complaints : (a) White Reflex in pupillary area (i) A dense opacity that fills the pupillary area is obvious even in natural light and noticed by the mother, midwife or the attending neonatologist. (ii) Faint, Peripheral and posterior opacities are missed initially. Such an infant is brought to ophthalmonologist by parents with the suspicion that the child does not have expected vision. It is only with the dilation of pupil the cataract becomes obvious. (b) The child is brought with nystagmus or squint. (c) There is positive history of developmental cataract in the family, so the child is brought to get it excluded. 2. Older Children are brought with white reflex, nystagmus that dates back to first three months, squint or diminished vision in various combinations. 3. Some children are brought with glare. The child with unilateral cases may close the effected eye to wardoff the glare. 4. Congenital cataract per se does not cause pain, photo phobia or redness of eye. If these symptome are present the child should be investigated for retinoblastoma. 5. Sick children who fail to thrive are likely to have metabolic cataract or rubella cataract. 6. Unilateral cataracts are detected late because the child carries out his routine with better vision in the other eye. They are fist brought with squint. Unilateral dense cataracts draw attention earlier than posterior cortical and posterior polar cataracts. 7. Some of the children are referred by paediatricians who have diagnosed a syndrome that is likely to have cataract.

UNILATERAL CATARACT
Unilateral congenital and developmental cataracts are unique in many ways: A. 30% of idiopathic congenital and developmental cataracts are unilateral. B. Some of the heredity cataracts are also unilateral.

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C. Children with unilateral cataract seek medical help later than bilateral cases. Generally they are brought with strabismus. D. In case of obvious cataract in one eye the other eye should be examined for of subtle signs of cataract. E. All cases of uniocular cataract should be examined in details for evidence of occult or forgotten trauma, uveitis, retinal detachment etc. F. Post operative visual prognosis in unilateral congenital or developmental cataract is always poorer than bilateral cases. G. Unilateral traumatic cataracts have better vision following cataract surgery than congenital and developmental cataract. H. The purpose of operating unilateral cataract is to improve vision even when it is known that there are no chances of improvement of central vision following successful surgery, the lens should be removed to improve peripheral field on that side. I. Developmental cataracts that have relatively clear lens for first few years like posterior lenticonous have good visual prognosis. J. All cases of unilateral congenital and developmental cataracts should be encouraged to have P.C.I.O.L than conventional extra capsular cataract operation49 with spectacle or contact lens.

WORK UP IN A CASE OF PAEDIATRIC CATARACT


A. Eyes of all new born children should be examined routinely by attending obstetrician, neonatologist for evidence of dense lenticular opacity. The traditional midwives and nurses can be taught to screen neonates for lenticular opacities. B. All neonates who have hepato spleenomegaly, abnormal heart sound and fail to thrive should be examined for possibility of galactocemia, rubella and toxoplasmosis. Urine examination for reducing substance will exclude galactosaemia while torch test28 is helpful for rubella and toxoplasmosis. Galactokinase deficiency is seen in otherwise healthy children with congenital cataract. C. Positive family history of developmental cataract in parents, siblings and first cousins should alert the physician for possibility of lamellar cataract. D. All white reflexes in pupillary area in children are not congenital cataracts. Cataract should not be confused with retinoblastoma and vice versa. It should be kept in mind that all non lenticular opacities need not be retinoblastomas. (See differential diagnosis of white reflex in pupillary area.) E. Examination of an eye with congenital cataract 1. As infants can not be examined on usual slit lamp they should be examined either with hand held slit lamp or under operating microscope. In absence of these an examination with uniocular loupe with a bright torch is good enough. Binocular loupes give very low magnification.

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2. Pupillary light reflex in infants is poor due to strong constrictor muscle. It can be seen well under magnification. 3. Anterior chamber is examined for other malformation of anterior segment and evidence of other diseases. 4. Next step should be examination of anterior segment under full mydriasis. The choice of mydriasis is as follows: (a) Tropicamide 0.5% to 1% is most suited for children and infants. (b) Atropine should be used with usual caution to avoid side effects. It is contra indicated in Down syndrome. (c) Cydopentolate 0.5% is also a suitable cycloplegic. (d) Phenylpherine is generally used as 2.5% drop along with 2% home atropine. Poor dilatation of pupil means a rigid pupil that may cause difficulty during subsequent surgery. A widely dilated pupil delineates outer border of the opacity. In lamellar cataract the periphery is clear. Fundus can be examined through this clear zone both by direct and indirect ophthalmoscope. If the eye has a large clear lens on the periphery, surgery can be postponed and child is kept on mydriatic. If no clear zone is visible between the lens and iris, prompt surgery is indicated. 5. The posterior segment is examined for presence of remnants of hyaloid system, retrolental fibroplasia and other fundus changes. 6. The macula is specially examined for evidence of central choroiditis in case of suspected toxoplasmosis. 7. An eye with bright cornea of normal size, clear A.C and brisk pupillary reaction generally has normal, intraocular tension. A large cornea with haze, deep A.C. is most likely to be a buphthalmic eye where examination of tension under generally anaesthesia is a prerequisite. So are the eyes with mesodermal abnormalities. 8. In cases where fundus can not be seen with dilated pupil USG of the eye and some time C.T. may have to be under taken. B.Scan can help to find out posterior capsular defect that is common in many congenital cataracts. In traumatic cataracts, B.Scan delineates vitreouscortexmix, associated detachment, haemorrhage, foreign body, exudates. 9. All children posted for surgery should under go a pre anaesthetic check. A preoperative check up by neonatologist may be ordered by an anaesthetist. 10. It is very important to explain the procedure and its utility to the parents, especially, in case of bilateral dense opacities and all unilateral cataracts. Possibility of IOL and other measure should also be discussed with them as far as in language and terms they can under stand avoiding medical jargons.

MANAGEMENT OF PAEDIATRIC CATARACT


A. Management of paediatric cataract is difficult and frustrating because of many factors some of them are:

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1. The eye of an infant is small and changes rapidly with age. 2. The visual system of a child is immature and likely to suffer irreversible damage if the macula does not receive sharp image of the outer world. Nystagmus and amblyopia develop fast in infancy and child hood. 3. All the opacities are not of uniform nature as regards density and position hence a uniform protocol can not be advised for all cases. 4. Cataracts in childhood may be associated with anomalies of other intra ocular structure that hamper improvement of vision. 5. Cataracts may be associated with larger systemic syndromes including errors of metabolism. 6. The child has a long life to live that includes education, vocational training, choice of profession where diminished vision, diminished accommodation, loss of peripheral field and appearance may be some of the hindering factors. 7. There is not medical treatment for cataract60. 8. Scope of prophylaxis is limited to rubella. 9. Genetic counselling is not always possible. 10. Definitive treatment is surgery which requires high degrees of skill and efficiency. B. Various modes of management available for paediatric cataracts 1. Prophylaxis (a) Prophylaxis is limited to rubella cataract and to some extant galactosemic cataract. If every girl in school going age receives a shot of rubella prophylaxy as part of national immunisation, the danger of rubella cataract can be wiped out altogether. It should be kept in mind that rubella is not only a blinding disease it is also a crippling and some times a fatal disease. (b) In galactosemia, removal of milk products from the childs diet will not only remove the possibility of child developing cataract but may improve transparency of the lens. (c) Trauma as a cause of cataract requires involvement of parents, teacher and social workers. Children should be explained about possibility of blindness by sharp objects like spears, arrows, and catapult, tipcats. 2. Non-Surgical This depends on presence of useful clear zone between the pupillary border and periphery of the opacity. A small opacity that does not cover the whole of normally acting pupil does not require any treatment including mydriasis which is required when the opacity covers the entire normally acting pupil but has useful clear zone when dilated. These are generally various types of zonular cataracts the regime consists of keeping the pupil dilated with lowest dose of atropine that gives maximum mydraiasis. Atropine has a draw back that it abolishes accommodation that is an important factor for learning, in children .These children under atropine may be provided with near correction. Dark glasses during day may also serve in limited way. The children should be taught to sit with back towards the light and light falling on the books.

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Surgery is the definitive treatment for all paediatric cataracts. Lensectomy with IOL is the standard procedure for most of paediatric cataracts, IOL is with held only when its placement is not possible. In such cases Lensectomy followed by contact lens, spectacles or epikeratophakia are some of the alternatives available. The aims of surgery are to preserve vision, prevent amblyopia and maintain fusion as far as possible. IOL in Children Older children can tolerate P.C.I.O.L as much as adults. Till few years ago it was thought that intra ocular lens be implanted only after 2 years of age50 Now a days, implanting a lens within 48 hrs of birth is considered safe47,49. Some of the anatomical difficulties in implanting IOL in children are 1. The eye ball is small in all dimensions. 2. Comparison between various dimensions of a new born and adult eye is given below :
Feature Axial length Volume Lens diameter Corneal diameter A.C. depth New Born 16.5mm 2.8ml 6.00mm 10.00mm 2.5mm Adult 23.5mm 7.1ml 9.1mm 11.8mm 3.2mm

3. The childs axial length will reach its adult size by seven years. At 2 years it is about 20mm51,52 and at four years it is 22 mm. 4. An IOL of 32D will be required to make an eye of a new born (axial length 16.5mm) emmetropic. But this is not correct because the axial length of the child will be as much as 21 to 22mm at 3 to 4 years of age, which, with an IOL of 32D will make it myopic by 8 to 10D. This amount of myopia reduces the distant vision greatly. The most ideal choice will be to use an IOL that will make the eye slightly myopic. This will give a comfortable near vision as well. 5. The other difficulty faced is difficulty in calculating IOL power in children below 2year of age. For accurate reading this has to be done under general anaesthesia which by it self is a highly specialised discipline. 6. Next difficulty is to decide when to implant an IOL. Children with dense opacities, where no part of the fundus is visible under maximum mydriasis should be operated as soon as the child may under go general anaesthesia for considerable time. In bilateral cases lensectomy is followed by IOL implant, otherwise a simple aphakia is left to be corrected by, spectacles, contact lens, and epikeratophakia. The last method has been given up in favour of secondary implant. It is better not to operate both the eye at the same time. 7. Unilateral cataracts of all densities have uniformly poor visual prognosis. 8. If the discs in both eyes are visible, are of normal colour, elevation and size, both maculae are visible with out any abnormality chances are that vision in both eyes are equal. The child should be watched for evidence of increase in size of opacity. An opacity larger than 3 mm requires removal. If the opacity is stationary the eye can be atropinised and bifocal prescribed when the child starts schooling.

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9. If there are evidences suggestive of asymmetry in vision, amblyopia must be treated vigorously. 10. Presence of nystagmus is always a very poor prognostic factor. It generally develops by two month in case of congenital cataract. 11. Next problem is to decide which type of lens should be implanted. A.C. IOL are out of question because of high rate of complication. It is better to leave an eye aphakic rather than pseudophakie with A.C.IOL. In majority of cases P.C.IOL are put with satisfactory results. Of course there is a school of thought that considers the infants eye to be too small and too immature to put a P.C.IOL because except an iris claw lens all lenses are over sized for the placement and fixation14,47 and recommend an iris claw lens with maximum width of 6mm to 7 mm with optics of 4mm. Many of the pediatric aphakics need secondary lens implant, if the posterior capsule is intact. An iris claw lens may be put even in a partially absent posterior capsule. 4. Other methods of management of aphakia in children Commonest cause of aphakia in children is surgical removal of lens. Other causes are spontaneous absorption of a congenital cataract due to posterior leak or absorption following penetrating wound. There may be traumatic dislocations or spontaneous dislocation in buphthalmos, megalocarnea or chronic uveitis. Aphakia in children is managed by (a) Spectacles (b) Contact lens (c) Secondary implant (d) Epikaratophakia (e) Combination of more than one method. (a) Spectacles. Spectacles in children are generally prescribed in all ages more for economic reasons than its optical efficiency in developing countries. Its advantages are: It is cheap, easy to manage, gives fairly good distant vision, bifocals may suffice for near vision. If power is equal in both eyes chances of amblyopia is reduced. They are not very suitable for unilateral alphakia because the child with good vision in other eye prefers normal single vision with out glasses and if the child is forced to wear them, have a tendency to look over the frame. This negating the optical advantage of spectacle. (b) Contact lenses49. Contact lenses are best alternative to IOL, some surgeons fit contact lens just after the lensectomy, others prefer to fit extended bear lens after 10 days. Hard contact lenses are difficult to insert due to tightness of the lid, strong blink reflex, frequent displacement of lens and expulsion of the contact lens. All the parents can not be taught to insert them. Up to age of six daily wear or extended wear contact lenses may be tried. Once the child can manage the contact lenses gas permeable lenses may be prescribed. (c) Combination. Contact lenses do not give use full near vision. They are given near correction in the form of spectacles. Some time spectacles may be required to correct residual power over contact lens or IOL.

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AFTER CATARACT53, 54, 55


A. After cataract is not a true cataract. It is the after math of rupture of lenticular capsule with partial absorption of cortex, opacification of posterior capsule with proliferation of lens fibers. In milder form it is also known as posterior capsular opacification or simply P.C.O. Almost all eyes develop P.C.O. following rupture of lens capsule that may be surgical, accidental or spontaneous. P.C.O. is by far denser in children than in adults. It is a major cause of gradual lowering of vision following uneventful I.O.L. implant. B. Common symptoms of after cataract are 1. White reflex in pupil which may be faint enough to be seen with magnification or denser that is visible in diffused light 2. Non improvement of vision 3. Frequent change of refraction. Common surgical procedures that result in after cataract are extra capsular cataract extraction, micro surgical extra capsular cataract extraction with or without IOL phacoemulsification, needling aspiration of soft cataract. Rarely injury to lens during vitrectomy may also result in after cataract formation. Incidences of after cataract depends upon : 1. Age of the patient : almost all aphakic children following extra capsular lens extraction develop PCO. 2. Duration following lens extraction : may develop soon after if coxtex has not been removed well. Most of the children will develop PCO with in one year. 3. Amount of cortical matter left, 4. Placement of IOL and type of IOL, 5. Associated pre existing uveitis 6. Management of post operative uveitis. C. Mechanism of after Cataract Formation The posterior capsule of lens is devoid of epithelium. The anterior capsule is lined by cuboidal cells up to beginning of the equator where the cuboidal cells get elongated gradually and are converted in to lens fibres. In an extra capsular cataract extraction following capsulotomy as much of cortex as possible is removed, yet some of the fibres are left behind, that continue to proliferate as opaque fibres, these opaque fibbers may spread over the posterior capsule, may proliferate as globular bodies or may be entrapped between the anterior and posterior capsule. The first is called simple P.C.O the second, Elschnigs pearl and the last Soemmerring ring. The after cataracts besides lens fibbers also contain pigments, exududate and blood in the initial stages. Elschnigs pearls are formed due to proliferation and migration of epithelial cells to form a fish spawn like small white translucent bodies. The size varies from pin head to 23 mm in diameter. They are generally seen in the pupillary area. Their numbers vary from single to many. Dilatation of pupil may expose more.

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Soemmerring ring is doughnut shaped ring opacity. This is formed due to adhesion of anterior capsule to posterior capsule entrapping cortex as well as equatorial epithelial cells. The ring is generally hidden behind the iris and visible only with mydriasis, the space inside the ring has fainter P.C.O. The ring may occasionally be dislocated in the anterior chamber. Incomplete capsulotomy as was fashion in traditional operation for congenital and traumatic cataract resulted in thicker after cataract than present day microsurgical capsulorrhexis. Continuous curvilinear capsulorrhexis cause less P.C.O than canopener capsulorrhexis. Posterior capsulorrhexis is done routinely with or without anterior vitrectomy in children to reduce P.C.O. Lens extraction with out P.C. IOL causes more opacity than with P.C.IOL. A biconvex IOL or a lens with posterior convexity also lessens P.C.O. Heparin coated IOL or heparin used in irrigating fluid diminishes chance of after cataract. Occasionally the posterior capsule develops wrinkles this causes, stretch lines on the posterior capsule resulting in fluctuating vision and glare. Capsule contraction syndrome is caused due to contraction of anterior capsular opening this is more common if the eye has suffered from uveitis in the past. Bacteria of low virulence like propionibacteriumacnes and staphylococcus epidermis45 may be entrapped in the capsular sac and cause capsular opacification with out causing endophthalmitis but may cause endophthalmitis when released following capsulotomy. D. Clinical Features of aftercataract depend upon Location of opacity, density of opacity, age of the patient, time lapse after surgery, and associated uveitis. Pre School children do not complain of diminished vision, they have to be tested for vision, and examined for amblyopia strabismus and nystagmus. On set of nystagmus in infants following surgery means that vision has not improved. This generally happens if the child is less than two years of age. In dense after cataracts the parents complain that cataract has not been cleaned well or has developed again. E. Signs consist of white reflex in pupillary area. When examined under mydriasis with slit lamp shows various grades of opacity ranging from translucent to dense white membrane. There may be gaps in between. Other features observed are Elschnigs pearls, Soemmerring ring and posterior capsular traction lines. F. Treatment 1. Best treatment of after cataract is its prevention or reduction in its density. It must be remembered that cent percent children develop some degree of P.C.O. that can be prevented by posterior capsulorrhexis with anterior vitrectomy, large capsulorrhexis, use of biconvex IOL and use of heparin coated IOL. Most important thing is to remove as much of cortical matter as possible during the initial surgery. The post operative uveitis should be managed promptly. 2. Once the after cataract has developed and is dense enough to cause reduction in vision it should be cut either surgically or by laser.

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3. Laser capsulotomy by N.D. YAG is the treatment of choice. (a) The indications of laser capsulotomy are diminished vision, monocular diplopia, glare, to facilitate intra-ocular examination and other manipulation. (b) Relative contra indications consist of (i) Clear view of fundus by direct ophthalmoscope through the centre of the pupil, because if observer can see the fundus, the observed eye should also be able to see. Otherwise other causes of non improvement of vision such as cystoid macular oedema, amblyopia, retinal detachment, vitreous bands and chronic endophthalmitis should be looked for (ii) Central corneal opacity (iii) Corneal oedema (v) Already existing glaucoma and cystoid macular oedema. (v) Peripheral retinal degeneration. (vi) Unwilling parents of the child and uncooperative child. (vii) Glass I0L The laser capsulotomy is done either in normal or semidilated pupil Complications of laser capsulotomy 1. Cystoid macular oedema or worsening of cystroid macular oedema 2. Transient rise of IOP 3. Rhegmatogenous retinal detachment.

TRAUMATIC CATARACT IN CHILDREN


General Consideration Trauma accounts for 80% of cataract in paediatric age group. They are less common in first three years of age, incidence gradually rises as the child grows. Most of them are unilateral however blast injury, cracker injury and chemical injuries, electric shock, radiation can cause bilateral traumatic cataract. Domestic injuries are commonest cause in younger children while sports injuries are more in older children. Ninety percent of injuries are avoidable. Traumatic cataract have better visual prognosis than congenital and developmental cataract even when they are uni-ocular specially if traumatic cataract occurs after development of binocular vision. Posterior segment and retinal involvement remain major cause non improvement of vision. Injuries commonly associated with traumatic cataract are: Penetrating injury with or without retained intra ocular foreign body, blunt injury, chemical injury, electrical injury and radiation. Penitrating injuries do not involve lens in isolation they are associated with corneal, corneo scleral or scleral injuries. Penetrating injuries passing through pupil generally have centre corneal scar and injury to the lens with out injury to iris, but it is common to find iris incarcerated in the wound, otherwise injury to iris and or ciliary body is very common.

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Small penetrating injuries Depending up on the size of the penetrating injury the cataract may be localised if the wound is small and heals fast otherwise complete, if the capsular wound heals quickly. These injuries are generally caused by sewing and knitting needles, nibs, pins, sharp long thorns etc. These small opacities are generally stationary. Large penetrating injuries cause 1. Total opacification in short time that may cause swelling of the lens, shallowing anterior chamber and glaucoma if the cortical matter does not leak. 2. If the capsule ruptures and the opaque cortical matter spills out of the capsular bag it may fill the entire anterior chamber. 3. If the external wound still leaks the eye remains soft. 4. With a watertight wound the tension may rise resulting into secondary glaucoma. 5. If there is associated zonulolysis : sub luxation or dislocation of cataractous lens. In such conditions vitreous may herniate through the aphakic area, some times the cortical matter gets mixed with vitreous. 6. Prolapse of uvea is common. 7. There may be associated hyphema. 8. There may be irido dialysis, chroidal repute or retinal detachment. 9. IOFB are common. Lenticular changes following blunt injury are less dramatic than penetrating injuries. Blunt injury may cause small, faint, discrete opacities that may fade with time. It may be permanent and stationary or may spread over the years. Both contusion and concussion can cause traumatic cataract. These closed globe injuries do not cause incarceration of the uvea. The uvea may suffer the effect of blunt injury along with lenticular changes that always hampers future vision. For traumatic cataract to develop there should be some damage to any part of lens capsule that allows aqueous to reach the cortex. This hydrates the cortex, which converts soluble protein into insoluble protein and leads to ultimate opacification Opacities may be: 1. Transient, static or progressive. 2. Localised as discrete opacities. 3. Rarely may be total. Rosette cataracts Rosette cataracts are common in blunt injuries. It can occur early or late. Early rosette cataract develops with in few days of blunt injury. It develops in the posterior cortex as opaque lines radiating from a central point, each line has feathery appearance due to finer linear opacities radiating from its trunk giving a star like appearance. There is a clear zone between the opacity and the equator of the lens. Late rosette cataract develops in the posterior cortex one to two years after the injury that may have been trivial and forgotten. punctate spots, rosette shaped or scattered subepithelial

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Vossius ring is not a cataract but is a common effect of blunt closed globe injury where black pigments are imprinted on the anterior lens capsule in a circular fashion. The deposits on the lens capsule correspond to constricted pupil. These are transient and may be confused with post inflammatory pigment on the lens capsule. They fade out over months some times leaving behind faint subepithelial opacities. Other uveal injuries associated with concussion and contusion cataract are: Hyphema, rupture of sphinter, iridodialysis, recession of angle of anterior chamber, choriodal haemorrhage, choroidal rupture, retinal haemorrhage, retinal detachment and Berlins oedema. All of them may have adverse effect on visual improvement of vision. D. Symptoms of traumatic cataract may be 1. Acute following penetrating injury in the form of loss of vision, lacrimation, photophobia, with or without rise of tension. 2. Concussion cataract may cause diminished vision after days or months. 3. Diminished vision weeks after blunt injury is seen in early rosette concussion cataract. 4. Late rosette cataracts cause gradual loss of vision after months. 5. No appreciable visual loss is seen in Vossius ring, punctate opacities, localised opacities away from pupil. 6. Unexplained pain in a closed globe with cataract denotes rise of intra ocular tension. 7. Faulty projection should warn about possibility of large retinal detachment. 8. A penetrating wound should always arouse suspension of intra ocular foreign body. 9. Injury with organic matter like thorns, twigs may present as endophthalmities. E. Investigation 1. The first and fore most investigation is careful history taking. In case of penetrating injury the child can not hide the history of injury but may not come out with the truth about the circumstances leading to injury and the offending object causing injury. The child may altogether withhold history of blunt injury. 2. Careful examination of vision in both eyes should be under taken in all cases of injury. In infants, pupillary reaction and projection of light gives a gross assessment of vision. Examination of vision has not only diagnostic and prognostic value. It has far-reaching medico legal value. 3. Examination of media by oblique illumination, retinoscope, direct and indirect ophthalmoscope, slit lamp should be done when ever possible. They will give information regarding status of posterior chapsule associated uveitis, subluxation of lens, type and extant of lenticular opacity, state of vitreous, Berlins oedema, cystoid macular oedema, choroidal tear, retinal detachment, retained intraocular foreign body, vitreous haemorrhage etc. 4. In opaque media when fundus is not visible, the first investigation ordered should be x-ray of the orbit. If the x-ray shows retained intraocular foreign body, it should be localised, using any of the standard method and foreign body extracted. Ocular ultrasonography C.T and M.R.I are useful to visualise small, I.O.F.B. vitreous haemorrhage, endophthalmitis, retinal detachment, and posterior perforation of globe. However USG may not resolve foreign bodies

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less than 2 mm. For such a small foreign body CT is more suited as it can detect bodies as small as 1.06 mm3. Glass fragments are visible only when they are as large as 1.5mm3.MRI is generally not used in suspected metallic foreign bodies because movement of metallic foreign bodies during MRI can result in MRI induced blindness56. Management Management of traumatic cataract in children does not differ basically from adult traumatic cataract but there are some special features that need to taken into consideration. which are : 1. Development of amblyopia and squint. 2. Difficulty in management of resultant aphakia in traumatic cataract. 3. Difficulty in placement of IOL. 4. Frequent development of P.C.O. in children. The management of traumatic cataract in children 1. Prevention: The best management is prevention. It is easier said than done. Parents should see to it that children do not play with pointed objects. The teachers and coaches should be instructed to encourage the children to use standard protective gears when ever required in sports and games. 2. Management of other injuries. 3. Surgery of cataract. Traumatic cataract is very often complicated by corneal, corneo scleral injuries along with damage to uvea, vitreous, retina in various combinations. These injuries are caused due to penetrating injuries. If the lens is already opaque it should be removed along with repair of above injuries as primary process with reconstruction of anterior segment. It may require vitrectomy. Most of the time it is not possible to put a PCIOL in such cases. Presence of central corneal opacity always jeopardises visual improvements. Simple traumatic cataracts are generally due to blunt injuries or small penetrating injuries of the lens with other tissue intact. These are managed by lensectomy, anterior vitrectomy and primary P.C.I.O.L implantation.

OTHER FORMS OF CATARACTS IN PAEDIATRIC AGE GROUP


A. Steroid induced cataract Steroids when used for long time locally or systemic have profound side effects. Two common vision threatening conditions are cataract and glaucoma. Longer and stronger the steroids used more are the chances of development of the cataract. Exact duration and strength that causes cataract is not known, perhaps it is influenced by genetically determined factor. Reduction in concentration, frequency, and duration can arrest progress of cataract formation but does not reverse the process. Children are more prone to develop steroid induced cataract than adults.

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Conditions where steroids are used for long time and most of the time in both the eyes are-vernal catarrh, interstitial keratitis, iridocyclitis due to juvenile rheumatoid arthritis, post operative and post traumatic status, keratoplasty and sympathetic ophthalmitis. In these cases steroid induced cataract may have to be taken as inevitable therapeutic risk and dealt with accordingly. Out of all the conditions listed above spring catarrh is the most offending condition that causes steroid induced cataract in children because the parents are not explained the regime to follow who allow the children to use strongest possible steroid for quick relief. Exact mechanism of production of cataract by steroids is not known. Steroid induced cataract has been reported following use of skin ointment, nasal drops, and inhalers containing steroids of various strength and for a long time. Most widely accepted theory is that steroids act on the lens epithelium that is responsible for hydration of lens fibers. How ever it does not explain why steroid induced cataracts always start in posterior cortex. Exact time taken for cataract to develop is not known. It never develops following use of steroid for short duration even in high dose like sub conjuctival injection. The opacity develops as a posterior sub capsular opacity that is denser in the axis of the lens. It may be in the form of circular dense disc surrounded by lighter areas of opacification. The opacity being near the nodal point causes much visual disturbances for its size. Common symptoms are : Glare, diminished distant vision, diminished vision in bright light, difficulty in near vision. It is frequent to find associated glaucoma that may contribute to further loss of vision. Hence, it is essential that all children who have been on steroid on long term should under go tests for raised intraocular pressure as well. Management. As cataract is a common feature of steroid use, steps should be taken to: 1. Reduce the strength and frequency of steroid to a level that gives best therapeutic response without causing cataract. 2. Replace steroid by non steroidal anti-inflammatory drugs when ever possible. 3. Manage spring catarrh by weakest solution of steroid, mastcell stabiliser, astringent, anti histamine etc. 4. Examine both eyes periodically for diminished vision, glaucoma and amblyopia. 5. Surgical intervention when required with P.C.I.O.L and management of amblyopia. Less common cataracts in children 1. Electric cataract57,58. The child is likely to develop cataract when struck by electric current or lightening of more than 200 volts that passes through the head. The Electric cataract is generally bilateral, how ever it may be denser on the side affected more severely than the other. Generally these children have sever skin and multisystemic involvement that may over shadow the ocular manifestation which becomes apparent only if the child survives and acute systemic effects of electric shock has subsided. There are various types of opacities in electric cataract. They can be vacuoles, streaks, both in anterior and posterior cortex. Generally the complete lens gets opacified rapidly.

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It may some times take months and years to develop cataract. If there are only few vacuoles present in anterior cortex with in first few months chances are that no significant opacities will develop. Management. Cataract is to be managed as any other cataract following blunt injury, after the acute multisystemic signs and symptoms have subsided. 2. Sunflower Cataract59. This is due to electrical dissociation of retained intra ocular copper that does not combine with the cells. The reaction depends upon copper content of retained foreign body. More the copper in the foreign body, more sever and faster the reaction. The copper is mostly deposited on continuous membrane like lens capsule and corneal periphery59 various manifestations of intra ocular copper foreign body are - Endophthalmitis that is supurative in nature, chronic non granulomatous panuveitis and sun flower cataract. The sun flower cataracts involve both anterior and posterior cortex and sub capsular area as yellowish dust in the centre of the lens with radiating yellowish petals pointing towards the periphery. Fully developed sun flower cataract is visible with on ordinary oblique illumination. Copper also gets deposited on corneal periphery as Kayser-Fleischer ring. Glaucoma is common. It is rare in children. Management. It consists of removal of foreign body, vitrectomy, lens extraction and management of glaucoma. C. Cataract in siderosis59 Iron is one of the commonest forms of intraocular foreign bodies in adults it is rare in children. It is rarely elemental iron, it is generally an alloy of iron. It is always associated with penetrating wound. The elemental iron is converted into its ferrous and ferric compounds. The metal is disseminated through out the tissues of the eye by electrolytic dissociation and gets combined with cellular protein that destroys the cells causing atrophy of the cells. The overall effect of dissemination depends upon ferrous content of the foreign body. Occasionally a small particle may get embedded in the avascular lens through the pupil and remain localised as a small dot of lenticular opacity never progressing to full blown cataract. The earliest change is deposition of iron in the anterior sub capsular area of lens. The metal is deposited as brown rings resembling rust ring in the mid periphery. The lens subsequently develops larger opacities. The iris also develops brown iron deposits. The retina develops degenerative changes similar to pigmentary degeneration causing diminished night vision, dyschromatopsia and diminished vision. Diagnosis is based on history of penetrating injury on examination an external wound of entry is always present. There may be hole in the iris through which the foreign body has passed. Inter lenticular particle may be visible. It the media is clear the foreign body can be seen with direct and indirect ophthalmoscope. Brown pigment on lens periphery and iris should arouse suspicion of iron as foreign body. In all cases of suspected intra ocular iron foreign bodies, the X-ray reveals its position unless it is very small CT and USG may show such bodies. MRI is contra indicated in metal foreign bodies. Management depends on correct diagnosis of retained iron foreign bodies. Very small inter lenticular bodies do not require removal but the eye is kept under observation for possibility of development of siderosis on long run. Large foreign bodies are removed after proper

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localisation through vitrectomy. Removal of iron foreign body reduces chance of siderosis. The lens, if opaque is removed by any of the standard procedures. Cataract secondary to ocular inflammation Cataract is a common complication of chronic uveitis of long duration. Exact cause of cataract formation is not known. It is multi factorial. The two common offending factors are the toxic effect of inflammation on the lenticular metabolism and Corticosteroids, which form the sheet anchor of the treatment of chronic uveitis. Commonly opacification starts in the posterior capsule. Later involving posterior cortex and ultimately covering whole of the lens. Sometimes broad posterior synechiea which are difficult to break cause anterior sub capsular opacities that are generally stationary. Management consist of early and proper treatment of uveitis and steroid should be used in its minimum possible strength and duration. No surgical intervention should be undertaken unless the eye is quiet for at least six weak. The surgery should be followed by prolonged instillation of steroids as trauma of surgery results in flare up of uveitis. Cataract in Lowes syndrome Lowes syndrome consists of mental retardation, convulsion, renal stone formation, diabetes, albumen in urine. Ocular manifestation consists of bilateral nuclear cataract that may be microspherophekic. Associated glaucoma may worsen the prognosis. Cataract in myotonic dystrophy Myotonic dystrophy is a heredofamilial disorder where muscles have increased tone that is difficult to relax. This increased tone may be associated with progressive weakness of the muscles. The condition is generally seen in adolescents. There is progressive loss of facial expression and ptosis. Lenticular features consist of fine dots beneath the capsule that may have polychromatic lusture. The stelate appearance of such opacities are called Christmas tree cataract.

REFERENCES
1. Duke Elder.S. and Wyber K.C. ; System of Ophthalmology, Vol. II : Anatomy of the visual system 1st Edition pp.311338, Henry Kimpton, London, 1961. 2. Nema, H.V., Singh V.P. and Nema.N. ; Anatomy of the Eye and Adnexa, 2nd Edition pp.3135, Jay Pee Brothers, New Delhi, 1991. 3. Nancy Anderson Hamming, Apple D. ; Anatomy and embryology of the eye in Principle and Practice of ophthal mology, Edited by Peyman G.A. Sander D.R. and Goldberg M.F. Vol.I pp. 312 First India Edition. Jay Pee Brothers, New Delhi, 1987. 4. Banumathy S.P.; Anatomy of the eye Edited by G.Natchiar P-59-60 published by Arvind Eye hospital and post graduate Institution, Maduri. 5. Sharma P. ; Essentials of Ophthalmology, First Edition pp. 133, Modern Publishers, New Delhi, 2000.

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6. Dutta L.C. ; Ophthalmology Principles and Practice, First Edition Page 226, Current Book International Kolkata, 1995. 7. Scheic H.G. and Albert D.M. ; Embryology of the human eye in Text book of ophthalmology, Ninth Edition, pp. 8182 Wb Saunders Company, Philadelphia. 1977. 8. Ahmed E. ; Physiology of the crystalline lens in A Text book of ophthalmology , First Edition, pp. 5152 Oxford University Press Kolkata.1993. 9. Adler F.H. ; Physiology of the eye, Sixth Edition, Edited Moses R.A. C.V. Mos by Co St. Louis, 1975. 10. Duke Elder S. ; System of ophthal mology, Vol. 4th, The physiology of the eye and of vision, Henry Kimpton, London, 1968. 11. Khurana A.K. ; Diseases of the lens in Ophthalmology second edition pp. 183212 New age international, New Delhi 2000. 12. Berger B.B. ; The lens cataract and its management in Principle and practice of ophthalmology, Editors, Peyman. G.A. 13. Gittinger J.W. ; Congenital Cataracts in Manual of clinical problems in Ophthalmology Editors Gittinger J.W. and Asdourian G.K. , First Edition pp. 7576 Little Brown and Co Boston 1998. 14. Singh D. ; Congenital and Infantile Cataracts in Current Ocular Therapy Fifth Edition, Edited By Fraun felden FT and Roy F.H. P-538-541 W.B. Saunders Comp. Philaled phia 2000. 15. Foster A Gilbert C. Rahi F. ; Epidemiology of Cataract in childhood, A global Perceptive, Jr Cat and Ref. Surgery 23: 601-604(99). 16. Duke Elders ; System of Ophthalmology Vol.-III, Part-II, First Edition pp. 688760. Henry Kimpton London 1564. 17. Miller SJH ; The Lens in Parsons diseases of the eye 17th edition pp. 171173 Churchill Living stone, London 1984. 18. Dobree J.H. ; Cataract in Modern Ophthal Mology Vol-4, First Edition, Edited by A. Sorsby, pp. 598618, Butter work London 1964. 19. Sharma P. ; Essentials of Ophthalmology first edition p. 136-, Modern Publishers New Delhi 2000. 20. Dutta L.C. ; Blue Dot Cataract in Ophthalmology Principle and Practice, First Edition Current Book Inter national Calcutta 1995. 21. Bonuik. V. ; Rebella in Current Ocular, Therapy Fifth Edition, Edited By Fraunfelder F.T. and Roy F.M. pp. 6869 W B Saunders Company. 22. Beger W.P. and Paterson R.A. ; Paediatric Ophthalmology in Manual of ocular diagnosis and therapy, Edited By Deborah Pavan Langston , Third Edition. 23. Alcon D.M. ; Galactocemia in Current Ocular Therapy Edition 5th , Edited by Fraun felder F.T., and Roy F.M. pp. 119120, WB Saunders Company Philadelphia 2000. 24. Cordes F.C. ; Galactosemic Cataract Am.J.Oph Oph 50:115 1960. 25. Epstein R.L. ; Inborn metabolic disorders and the eye in Principles and practice of ophthalmology Vol. III, Edited by Peyman G.A Sauders D.R. , Goldberg M.F., First Indian Edition pp. 17391741 I.F jay Pee Brother , New Delhi 1987.

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26. Digeorge A.M. ; Ocular Changes in endocrine disease, in Pediatric ophthalmology, Vol.II, Edited by Harley R.D Second Edition pp. 9881012, WB saunders company Philadelphia 1983. 27. Kansky J.J. ; Clinical Ophthalmology , Second Edition pp. 234258, Butter Worth London 1989. 28. Calhoun J.H and Hiles D.A. ; Cataract and Intraocular lens implant in Pediatric ophthalmology Vol. I, Edited by Harley R.D. , Second Edition pp. 549582, WB Saunders Company Philadelphia 1983. 29. Duke Elder S. ; Abnormalities in size of lens in system of ophthalmology Vol. III, Part-II, First Edition pp. 694675, Hunry Kimpton London 1964. 30. Guemes A and Elias Iraboulsi ; Microspherophakia in Current ocular therapy fifth edition edited by fraunfelder FT and Roy F.M. pp. 543544, WB saunders company Philadelphia. 31. Guemes A and Elias Traboulsi ; Lenticonous and lengiglobus, in current ocular therapy, fifth edition edited by Fraunfelder FT and Roy F.M pp. 542543. 32. Duke Elders ; Anomalies of the lens in system of ophthalmology Vol. III part-II first edition pp. 688710, Henry Kimplon London 1964. 33. Ida Caroline Mann ; Developmental anomalies of the eye, Second Edition, London 1957. 34. Khurana A.K. ; Congenital anomalies of the lens in Ophthalmology Second Edition, p. 212, New age international (p) Ltd., New Delhi 2001. 35. Casper D.S. Simon J.W. etal ; Familial simple ectopia lentis. A case study , J Pediatric ophth and strab 22 : 227, 230 1985. 36. Nelson L.B. ; Maumene IHEctopia Lentis, Surv Oph 27:143,1982. 37. Cross HE ; Ectopia Lentis et pupillae- Am.J. Oph 88:381 1979. 38. Duke Elders ; Multiple Syndrome in System of ophthalmology , Vol. III, Part-II , pp. 11021112, Henry Kimpton London 1964. 39. Gittinger J.W. ; Ectopia lentis in Manual of clinical problems in ophthal mology, First Edition, Edited by Gittenger and Asdourian G.K. pp. 7880 little Brown and company, Boston 1998. 40. Epistine RL ; In born metabolic disorders of the eyes. In Principles and practice of Ophthalmology Vol. III Edited by Peyman G.A. Sander D.R. Goldberg, M.F First, Indian Edition pp. 17461754, Jay Pee brother , New Delhi 1987. 41. Cross H.E. and Jensen A.D. ; Ocular manifestation of Marfan syndrome and homocystinuria. Am. J. Oph 75 : 405 : 1973. 42. Bloch R.S. ; Homocystinuria in Current ocular therapy , fifth edition, edited by Fraunfelder FT and Roy F.H. pp. 110111, WB Saunder Company Philadelphia 2000. 43. Speath G L Barber G.W. ; Prevalence of homocystimuria among mentally retarded evaluation of specific screening test, Pediatric 40 : 586 : 1967. 44. Hayasaka. S. AsanoY, Tadeka H. ; Lens subluxation in homocystinuria Acta Oph 62-425-431 1984.

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45. Smith T.M. Holland M.G. Woody NC ; Ocular manifestation of familial hyperlysinemia, Tran Am Acc of oph and otolyr 75 : 355 1971. 45a. Walton D.S. ; Weill marchesani Syndrome in current oculartherapy Fifth edition edited by Fracenfeldes. F.T. Roy F.M. p. 179- WB Saunders company Philadelphia 2000. 45b. Wright K.W. ; Chrous G.A. Weill Marchesani Syndrome with bilateral angle closure glaucoma. J. Pedioph Strab 22:129-132, 1985. 45c. Jones R.F. ; The syndrome of Marchessani Br J. oph 45: 377-381, 1961. 46. Zwann, J.T. ; Cataract in children : Diagnosis in Decision making in ophthalmology, First Indian Edition, Edited by VanHeuven , W.A. J and Zwaan J.T. pp. 130131, Har court, Brace and company Asia Ltd. Singapore 1998. 47. Singh D. ; Paediatric Cataract in CME Series, Published by All INDIA OPHTHALMOLOGICAL SOCIETY, New Delhi. 48. Dutta N.K. Dutta L.C. ; Congenital cataract and other congenital abnormalities of the crystalline lens in Modern ophthal mology Vol.-I edited by LE Dutta pp. 308312, First Edition Kay Pee Brothers, New Delhi. 2000. 49. Mehta K.R. ; Lens Implantation in children in Modern ophthal mology Vol. I Edited by Dutta L.C. pp. 372378, Jay Pee Brothers , New Delhi 2000. 50. Dada V.K. ; 10L in Children in 10L secrets , First Edition pp. 1924, Jay Pee Brothers , New Delhi 1992. 51. Jagat Ram Pandey S.K. ; Infantile cataract surgery : Current techniques, complication and their management in Modern ophthalmology Vol. I, Edited by Dutta L.C., pp. 378384, Jay Pee Brothers, New Delhi 2000. 52. Wilson M.E. Apple D.J. Bluestin E.C.Wang XH ; Intra ocular lenses for Pediatric implant, Biomaterials design and sizing, J-cat reft sur 20 : 584-591 1994. 53. Sharma Y.R. Neena Mittal Dinesh ; Pathogeneses and management of posterior capsule opacification in Modern ophthalmology edited by Dutta L.C., Vol. I Edition - 2, pp. 400404, Jay Pee Bro, New Delhi 2000. 54. Allinson R.W. ; After Cataract in Current ocular therapy Ed. Fifth Edited by Fraunfelder F.M. and Roy F.M pp. 535538 W.S. Saunders Company Philadelphia 2000. 55. Gimbal H.V. ; Posterior capsulor rehexis with optic capture in paediatric cataract and intra ocular lens surery ophthalmology 103 : 175 -187 1996. 56. Haywood M ; and Lina M Marouf, Eye Trauma in Decision making in ophthalmology edited by Heuven W.A.J and Znaan J.T., First Indian Edition 1998 pp. 5859, Harcourt Brace & Comp, Singapore. 57. Dutta L.C. ; Diseases of the crystalline lens in Ophthalmology principle and practice, First Edition pp. 224243, Current book international Calcutta 1995 58. Fraunfelder F.T. ; Electric Injury in Current ocular Therapy Fifth Edition, Edited by Fraunfelder FT. Roy F.M. pp. 272, WS Saunders Company Philadelphia 2000. 59. Miller S.J.H. ; Injuries to the eye in Parsoris Diseases of the eye, 17th Edition pp. 249250 Churchill Livingstone, London 1984.

CHAPTER

10

Glaucoma in Children
Anatomy of the structures related to glaucoma : The structures of the eye related to glaucoma can be divided into : A. Structure related to aqueous production. B. Structures related to circulation of aqueous. C. Structures related to drainage of aqueous. D. The limbus A. Ciliary processes1. These are the sites of production of aqueous humour. They are 70-75 in number. They arise from pars ciliaris of the ciliary body and project towards the centre of the eye ball all around. They have following layers : 1. Epithelium, 2. Tuft of blood vessels, 3. Stroma. 1. The epithelium is two layered. (a) Pigmented epithelium2 which is neuro ectodermal in origin and is forward continuation of pigment epithelium of the retina. It is the outer layer of the ciliary process. It extends from ora serrata to the iris. It is a single layer of cuboidal cells. (b) Non pigmented epithelium. This is the inner layer of ciliary process. It is also neuroectodermal in origin. It is continuation of non pigmented layer of the retina. It extends from ora serrata to the root of the iris. The non pigmented and pigmented layers of the ciliary process are firmly attached to each other and do not detach from each other like in retina.3 2. Tuft of blood vessels. Each ciliary process has a tuft of blood vessel in the central core. They are similar to choroidal vessels in nature without choricapillaries. The endothelium of these vessels are thin and fenestreted4. 3. The stroma. The stroma of ciliary processes is very thin, it surrounds the blood vessels and separates them from the surface epithelium. It is mostly muco poly saccaharide with few collagen fibres.4

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B. Structures related to circulation of aqueous. The aqueous is produced from the ciliary processes and poured in posterior chamber from there it passes through the pupil in to the anterior chamber where it circulates and leaves the anterior chamber via angle of anterior chamber. The structures related to circulation of aqueous are : 1. The aqueous chambers of the eye. 2. The angle of the anterior chamber. 1. The aqueous chambers. The ciliary body, zonules and the lens divide the eyeball in two unequal chambers - A large vitreous chamber and a smaller aqueous chamber. The aqueous chamber is again divided into two unequal parts by the iris into : (a) A smaller posterior chamber (b) A larger anterior chamber (a) The posterior chamber is a space bounded anteriorly by the posterior surface of the iris. Posteriorly it is bound by periphery of anterior lens capsule, anterior plane of zonule and anterior part of the ciliary process. (b) The anterior chamber is a convex space with convexity forward. The convex surface is bounded by the posterior surface of the cornea. The flat posterior surface is formed by a chink of anterior surface of ciliary body, the iris and the anterior lens capsule. The anterior chamber is lined by endothelium except on the posterior surface formed by lens capsule. The endothelium is absent in crypts of the iris also. The junction of the anterior and posterior surfaces is the angle of the anterior chamber. Being a plano convex space, its depth is maximum at the centre and gradually diminishes on the periphery where the two surfaces meet. Depth of the anterior chamber is genetically determined. It is shallow in new born, attaining adult depth after two years. It is deep in myopia, buphthalmos, megalo cornea, keratoconus, subluxation of lens and aphakia. It is shallow in micro-ophthalmos, micro-cornea, cornea plana, dysgenesis of anterior chamber, narrow angle glaucoma, swollen lens, hypotony and intraocular tumour pushing from behind. The angle of anterior chamber. The angle formed by the posterior surface with the anterior surface is almost 45. In normal eye the angle is not blocked even with maximum mydriasis. Structures related to drainage of aqueous C. The Trabecular mesh work. At the extreme periphery of the cornea on its inner side of the anterior chamber directly under the limbus is a depression that runs all round the angle of the anterior chamber. This is called scleral sulcus. The posterior lip of the sulcus is sharp and is called the scleral spur. The ciliary body is attached to the scleral spur. A sieve like structure bridges the scleral sulcus and is called trabecular meshwork. The meshwork extends from the scleral spur to periphery of the cornea. The line where the trabecular meshwork is attached to the corneal periphery is called Schwalbes line which is the thickened end of Descemets membrane. It forms an important landmark in gonioscopy. The trabecular meshwork converts the scleral sulcus into canal of irregular calibre called Schlemms canal.

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The trabecular meshwork is a collection of tissues that on section looks more or less like a triangle, the apex of which lies at the Schwalbes line. The base is formed by scleral spur and ciliary body, the outer wall is formed by canal of Schelemms, the inner wall faces the anterior chamber. The meshwork as per histological picture is divided into8 Corneoscleral meshwork, uveal meshwork, and endothelial meshwork. The uveal meshwork is adjacent to canal of Schlemm hence called juxta canalicular meshwork. The canal of Schlemm. This is a venous channel that runs all round the angle of the anterior chamber in the sclera. It has irregular calibre. At most of the places it is a single endothelium lined channel, at places it breaks into tributaries and becomes multi channelled. The tributaries may merge with the main channel at places. On the outer side the canal-of-Schlemm, is joined to episcleral and conjunctival veins. The episcleral veins drain into anterior ciliary veins, the conjunctival veins drain into palpebral and angular veins. D. The limbus. The limbus is most important anatomical landmark for glaucoma surgery. It is an area 1 mm wide all round the cornea representing the junction of cornea on one hand, and conjunctiva, episclera and sclera on the other hand. From surgical point of view it has been divided into two circles and two zones in between5. The circles are formed by anterior limbal border, that is insertion of conjunctiva and Tenons capsule in the cornea. It overlies the termination of Bowmans membrane. The posterior limbal border lies approximately over the scleral spur. An imaginary line between the anterior and posterior limbal border is called mid limbal line that lies over the Schwalbes line. The mid limbal line is the junction of blue corneal limbus to the scleral limbus. The space between the anterior limbal border and mid limbal line is called anterior limbal zone. The space beyond the mid limbal line upto posterior limbal line is called posterior limbal zone. The limbus does not form a ring of uniform width all round the cornea. The anterior limbal zone is narrowest at the horizontal plane of cornea and widest at superior limbus. The development of anterior chamber. The presence of anterior chamber becomes evident at an early stage of embryo genesis i.e. 20 mm (7 weeks)6. As the lens separates from the surface ectoderm, the surface ectoderm is converted into corneal epithelium. A wave of mesoderm invades the space between the corneal epithelium and the lens to develop stroma of the cornea, endothelium of cornea, iris and iris stroma. The space between the iris mesoderm and the corneal stroma is anlage of the anterior chamber.7 This space remains very narrow as a cleft up to fifth month antenatal and then develops rapidly with the growth of the anterior segment. The canal of Schlemm develops at about nine weeks6 as a vascular channel at the level of recess of angle and gradually moves anteriorly in relation to the iris. The angle of the anterior chamber develops two months after the canal of Schlemm.8 The trabecular meshwork develops from vascular mesoderm originating from the margin of the optic cup.6 At birth the canal of Schlemm and the trabeculum both lie at the angle of the anterior chamber.9 The drainage system becomes functional just before birth.

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Congenital anomalies of anterior chamber. Majority of glaucomas in children are either congenital or developmental hence it is necessary to know the common congenital anomalies of anterior chamber where most of the lesions lie. The anterior chamber develops as a chink in the mesoderm between the corneal stroma and iris stroma. It is but natural that congenital anomalies of anterior chamber may involve cornea, iris and angle of anterior chamber in various combinations. Roughly anomalies of anterior chamber can be divided into following groups : A. Iridocorneal dysgenesis. B. Mesodermal dysgenesis (anterior chamber cleavage syndromes) C. Trabeculodysgenesis In the first two groups the cornea is frequently involved and glaucoma is a common feature that manifests in childhood. In trabeculodysgenesis, the trabecular tissue is mostly involved and cornea is either spared or effected minimally. Glaucoma in the form of primary congenital glaucoma is the most important clinical finding. A. Irido corneal dysgenesis13. This is a rare condition, is most commonly known as essential iris atrophy14 and also as Cogan Reese syndrome or Chandler syndrome due to overlapping signs however some authors divide them as different entities. Endothelial defect being a prominent feature, the term iridocorneal defect is more appropriate. The exact etiology of the condition is not known15. This is non-hereditary more common in females, it is unilateral mostly, rarely the other eye may be involved. Common age for it to become symptomatic is third and fourth decade. The disease starts in childhood and is detected during routine eye examination. No associated systemic disorders have been reported with the condition. Specular microscopy reveal peculiar cells in the endothelium, the cells are known as iridocorneal endothelial cells (ICE)16,17,18. The ICE cells are pathognomic and are supposed to be the cause of endothelial damage. The ICE cells migrate to the trabecular meshwork and cause mechanical obstructing resulting in glaucoma. The abnormal endothelial cells also cause corneal decompensation resulting in corneal edema. Corneal edema is independent of raised intraocular tension. The progress of the disease is slow, only 50% of eyes require antiglaucoma treatment for raised intraocular pressure that range between moderate and severe. The ocular findings are : Peripheral anterior synechea, full thickness hole in the iris, pseudo polycoria, shifting of pupil away from the holes towards the peripheral anterior synechea. The iris is wider in the sector where the holes develop, ectropion of uvea, heterochromia of iris. The iris is atrophic at places, there may be nodules on the iris. A thin transparent membrane is seen over the iris surface. The vision is generally poor due to corneal edema and glaucoma. The condition should be differentiated from posterior polymorphic corneal dystrophy which may be seen in children, may be associated with glaucoma and hazy cornea and is bilateral.

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Treatment is directed towards glaucoma. Initially medical treatment is tried if it fails. Trabeculectomy is the best surgical choice. The trabeculectomy hole may be blocked by ICE cells. Next alternative is Molteno valve. B. Mesodermal dysgenesis (Anterior chamber cleavage syndrome) Mesodermal dysgenesis consists of : 1. Posterior embryotoxon 2. Axenfeld anomaly 3. Riegers anomaly 4. Peters anomaly 1. Posterior embryotoxon18, 19. This is the mildest form of mesodermal dysgenesis.20 This anomaly is present in all forms of mesodermal dysgenesis. Ten to thirty percent of normal eyes show posterior embryotoxon without any other symptom21 except cosmetic blemish. The Schwalbes line is prominent and shifted centrally. It is formed by splitting of Descemets membrane into two leaves that contains collagenous tissue that is nearer to trabecular meshwork than cornea. The thickened Schwalbes line projects in the AC as a ridge all around 1 mm inside the limbus or it may be fragmented. Its appearance is similar to arcus juvenilis that develops at the end of the Bownans membrane, rest of the cornea, iris, angle and trabecular mesh work are normal. The condition does not require any treatment. 2. Axenfelds anomaly. This condition may be considered to be more advanced form of posterior embryotoxon. In this condition strands of peripheral iris tissue are attached to the thickened Schwalbes line. Glaucoma need not be present in all cases because angle remains unaffected in most of the eyes. The iris and pupil are also normal. Glaucoma when present is difficult to manage. The condition is bilateral without any predilection for sex. The condition becomes obvious in childhood due to prominent posterior embryotoxon. Glaucoma is not met with in childhood. It is a feature of late adolescence or adulthood. 3. Riegers anomaly22, 23. Riegers anomaly represents gross anomaly of the structures that arise from mesoderm. It involves cornea, iris and trabeculum in various combinations. The corneal change is represented by posterior embryotoxon. Iris shows hypoplasia of stroma with full or partial thickness holes that may be present as deformity of the pupil in the form of corectopia, dyscoria, pseudocoria or slit like pupil. There may be positive transillumination of the iris. The peripheral iris is attached to the thickened Schwalbes line as slender or broad adhensions. These adhesion may be in patches or may encircle the whole of the angle. The trabecular meshwork itself is normal. The iridocorneal adhesion is the cause of glaucoma that is seen in half of the cases. Rise of intraocular tension may go unnoticed unless looked for specifically. Severe rise of tension in infancy causes typical buphthalmos. Other less common findings are23A. Persistent hyaloid vasculature, partial or total absence of choroidal and retinal pigment epithelium, dysplasia of retina. The lens is not primarily involved, lenticular changes are secondary to glaucoma. Skeletal changes are sometimes seen in Riegers anomaly limited to face and teeth. There is hypoplasia of maxilla, hypodontia and micro-dontia. The ocular features are bilateral but not symmetrical. Common inheritance is autosomal, sporadic cases are also known.

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Once glaucoma develops the aim is to keep the tension low medically if that fails surgical intervention may be required. 4. Peters anomaly 24, 25, 26. This condition is rarest among all mesodermal dysgenesises. The most striking feature is a central corneal defect. It is a bilateral condition, present at birth, seen equally among boys and girls, has autosomal recessive inheritance. It can be divided into following groups : (a) Type Iassociated with posterior embryotoxon. It has combined picture of Axenfelds and Riegers anomaly with central corneal defect in the form of central facet at the level of Descemets membrane. The endothelium is absent in the facet. The iris is attached to the cornea all around the defect, leading to stromal opacity. The anterior chamber is slit like in between the central adhesion and peripheral iridocorneal synechea. The lens is in place. (b) Type IINo evidence of Axenfeld or Riegers anomaly. Only feature is central iridocorneal adhesion as in type one. (c) Type IIIThe findings are similar to type II. The anterior lens capsule is adhered to central corneal defect. The anterior chamber is shallow. The lens soon becomes opaque. Glaucoma is seen in fifty percent of cases. Other anomalies include microphthalmos, cornea plana, sclerocornea, vitreo retinal defects. C. Trabeculo dysgenesis27, 28, 29. In this condition the developmental anomaly lies in the trabecular meshwork, rest of the angle, anterior chamber and iris are developmentally normal. It is present at birth, more common in boys, it is familial, more than one sibling may be effected. The inheritance is heterogeneous but may be autosomal recessive. The exact mechanism of the anomaly is not well understood. There are two schools of thought, the first believes that there is an anatomical mal-developmental. The other postulates that there is arrest of maturation. There is an incomplete cleavage of uveal periphery from the corneoscleral wall. The meridional fibres are inserted either in the trabecular meshwork or in Schwalbes line. Gonioscopically the angle is wide but the trabecular meshwork is not clearly visible due to a thin membrane like structure on the surface of the trabeculum. This is eponymed as Barkans membrane. Many authors have doubt about its presence. As the trabecular meshwork is plastered with mesodermal tissue, the aqueous fails to reach the canal of Schlemm resulting into rise of intra ocular tension that causes primary infentile glaucoma or buphthalmos with its consequences. Comparison between iridocorneal dysgenesis and mesodermal dysgenesis.
Irido corneal dysgenesis Age Mesodermal dysgenesis

Symptoms develop in second and third Symptoms develop late but can manidecade, can be discovered in childhood fest in childhood. if looked for. More in females Non hereditary Equal in both sexes Polygenic inheritance

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Laterality Corneal involvement Generally unilateral

PEDIATRIC OPHTHALMOLOGY Generally bilateral

Mostly due to endothelial damage, but Posterior embryotoxon, strands of iris may have corneal opacity at a place touching cornea. where iris touches the cornea. Present, independent of glaucoma Glaucoma is the cause of corneal edema.

Corneal edema Pupil Systemic involvement Visual prognosis

Distorted, shifted towards peripheral Distorted may have pseudo polycoria, anterior synechea. corectopia and ectropion of uvea. Nil Poor May have facial, dental and skeletal abnormality. Poor

Aqueous humour A. This is a crystal clear fluid that fills both the anterior and posterior chamber. The exact time it starts forming is not establishing but must be before birth because the aqueous drainage system is ready to function before birth6. B. The functions of aqueous humour are to : 1. Give and maintain an optimal intraocular pressure compatible with vision. 2. Provide nutrition to avascular structures of the eye i.e. the cornea and lens. 3. Remove waste products of metabolism. 4. Act as an important optical medium. C. The composition of aqueous humour : The aqueous humour differs greatly from plasma from which it seems to be derived by the way of its low, protein, sodium, bicarbonate and glucose. The ascorbate content is very high, chlorides and lactic acid have higher concentration. The aqueous humour is acidic, and slightly hypertonic. The albumin globulin, ratio is same as in plasma. The human aqueous contains only IgG and not IgA and IgM. There is hardly any change in the composition of aqueous in anterior and posterior chamber. The composition of aqueous in phakic and aphakic eyes are the same proving thereby that lens does not participate in formation of aqueous. The refractive index of aqueous is 1.33710 which is less than both the cornea and lens. D. Formation of aqueous humour. The aqueous humour is derived from the plasma in the capillary network of the ciliary process. There are three theories of production of aqueous in the ciliary processes. 1. Diffusion 2. Ultra filtration 3. Secretion The plasma passes through three tissue layer that allow fluid with specific quality to pass through it. They are : The wall of the capillary, the stroma of the ciliary process and the

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ciliary epithelium. None of the above process fulfil all the conditions to be fit into one process. It seems all the processes take part in different proportion. The most important out of the three is secretion. E. Rate of formation of aqueous humour. The rate of aqueous production is called in flow of aqueous. It is measured in micro litres per minute (ml/min). It is not uniform throughout the day and night. It shows a diurnal variation. The normal range in adults is 2.8 to 4.3 ml/mt The production is least in the night12. The inflow increases following drinking of large volume of water and is decreased by ingestion of many locally and systemic acting drugs. F. Rate of outflow. To maintain intraocular pressure within normal range, there should be an equilibrium between inflow and outflow of aqueous. The aqueous is produced in the ciliary processes that passes successively through posterior chamber, pupil and anterior chamber where the aqueous circulates between the cornea and the iris by convection current. The aqueous leaves the anterior chamber via trabecular meshwork, canal of Schlemm and is finally picked up by episcleral vessels. This mode of outflow that is about 85% of total outflow is called conventional outflow, rest is drained by what is known as unconventional outflow. The unconventional path comprises of uveoscleral and uveovertex pathway. Presence of uevoscleral channel is well etablished. There is no unanimity regarding presence of uveo vertex path. The facility of outflow is measured by a non invasive method called tonography. The outflow of facility is expressed in ml/min/mm.Hg. The outflow canbe increased by applying pressure over the globe and by topical drugs. The normal value is about 0.20 ml/mt/mm Hg. Intraocular pressure : A. Normal intraocular pressure ranges between 15-20 mm of Hg. It is hardly influenced by age or sex. It shows a physiological variation of 2 to 3 mm between the highest and lowest pressure during the days and is called diurnal variation. In most of the eyes the tension is maximum in the morning however some persons may show an evening rise. A biphasic rise is also possible. B. Measurement of intraocular pressure - Intraocular pressure is the lateral pressure exerted by intraocular contents on the outer coats of the eye i.e. the cornea and the sclera. There are two clinical methods of clinical methods of measuring it. 1. Indentation tonometer 2. Applanation tonometer The applanation tonometer is more accurate than indentation as it is not influenced by scleral rigidity. The advantages of indentation tonometer are that it is very cheap, easy to handle and gives fairly accurate reading. Out of all applanation tonometers most widely used tonometer is Goldmanns tonometer. The other is Draeger tonometer. The applanation tonometer can be mounted on a slit lamp or may be handheld (Perkins). Other used applanation tonometers are : A. The pneuno tonometer B. The air puff non contact tonometer.

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Gonioscopic anatomy of angle of anterior chamber. The angle of anterior chamber has been called the cockpit of glaucoma. It is not visible in normal eye with oblique illumination. To see it, special optical devices called gonioscopes are employed. The gonioscopic anatomy of the anterior chamber has following visible landmarks from peripheral iris to peripheral cornea. A. Ciliary body. This is the part of the ciliary body visible from the insertion of the iris to the ciliary body to the insertion of the ciliary body in the scleral spur. The width of ciliary body is wider in myopia and narrower in hypermetropia. The colour is grey to dark brown. B. Scleral spur. This is the part of the sclera to which the ciliary body is inserted. This represents the posterior sharp edge of the scleral sulcus. The ciliary body is attached little behind the posterior attachment of trabecular meshwork. On gonioscope, it looks as a prominent white line in front of ciliary band and behind the trabecular meshwork. C. The trabecular meshwork. The trabecular meshwork is porous structure that stretches across the angle of anterior chamber, bridging it between the scleral spur and Schwalbes line. It has two distinct parts, a non-functioning anterior part and a functioning posterior part. The angle of anterior chamber is examined for presence of pigment, new vessels, exfoliations, synechia and foreign body. Common causes of increased pigmentation are acute and chronic glaucoma, uveitis, trauma. D. Schwalbes line. This is the termination of Descemets membrane and represents the anterior limit of the trabecular meshwork. It looks like a translucent circular band. In case of posterior embryotoxon, it is very prominent and shifted anteriorly. E. The Schlemms canal. This is not visible in all eyes. When visible it looks like a darker line in the lower trabeculum. If the episcleral pressure is higher than intraocular pressure blood may be seen in Schlemms canal. However blood can also be seen in hypotony with normal episcleral pressure. F. The angle recess is the term used to denote dipping of iris in the ciliary body as it inserts in the ciliary body. Gonioscopy. Gonioscopy is a non invasive clinical method of visualising the angle of the anterior chamber. The rays arising from the angle of anterior chamber do not leave the eye. They are reflected back hence are not visible because the rays arising from angle pass through aqueous and cornea into air which has refractive index less than the former two. The critical angle of the rays is more than 46 hence they are reflected back. This difficulty is overcome by eliminating air corneal interface and allowing the rays to pass a contact lens making the angle visible. The device is called gonioscope. There are two types of gonioscopes 1. Direct gonioscope 2. Indirect gonioscope The direct gonio-lens has an anterior curvature that does not allow the critical angle to be reached hence the rays are refracted at goniolens air interface. In case of indirect

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gonioscope, it has a contact lens and either a reflecting mirror or a prism. The reflecting angle is almost 90 to the lens air interface hence can be picked up by slit lamp or an operating microscope. A large number of gonioscopes in both the categories are available, each with specific design and purpose. Childhood Glaucoma Roughly two percent of population above forty years suffer from some type of glaucoma or other, mostly chronic symptomsless primary open angle glaucoma. The incidence rises with age and by eighty years the percentage reaches an alarming figure of five percent. Fortunately, awareness of adut prevalence of glaucoma is quite high nowadays and more and more persons are benefited by early detection and proper treatment. Childhood glaucoma differs from adult glaucoma in many ways.30 Most of the cases are symptomatic hence seek medical advice early unless it is unilateral and mild. Loss of vision is fast and gross. If the condition is not treated, permanent visual damage is inevitable. Prevalence of childhood glaucoma is far less than adult glaucoma hence chances of missing it are more. Classification of glaucoma in children. There is no unanimity regarding classification of glaucoma. There are many ways to classify them. Some of them are : A. According to age of onset 1. Those manifesting within first two years of age. 2. Those developing after two years but before puberty. 3. Developing in early childhood. B. According to involvement of ocular structures 1. Those due to developmental anomalies in the eyes. (a) Anomaly in the angle only. (b) Multiple anomalies of anterior segment including angle. 2. Secondary to causes not related to developmental anomalies of the eye. The group A is designated as congenital or developmental glaucoma. The group B consists of conditions that constitute a heterogeneous group of glaucoma called secondary glaucoma, both the groups can have wide or narrow angle. C. The most widely accepted classification that fulfils almost all criterions is one given by Duke Elder in 196430,31. This divides congenital glaucomas into : 1. Developmental buphthalmos (a) Simple buphthalmos (b) Associated buphthalmos 2. Secondary congenital buphthalmos All the eyes under three years of age that have raised intraocular tension will go into buphthalmos irrespective of cause.

344 Simple buphthalmos

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General consideration35, 36. The term buphthalmos is not a specific clinical entity. It embraces many diverse conditions. The only common feature among them is enlargement of the globe secondary to raised intraocular tension. The eye ball is enlarged and looks more or less like a bovine eye, to be specific eye of an Ox. Buphthalmos is seen not only in congenital glaucoma but in all conditions that cause rise of intraocular tension under three years of age. The cornea stops enlarging after three years of age so enlargement of the globe is either arrested or minimal by three years. The term buphthalmos will be used in the text to denote primary developmental glaucoma as a general practice rather than its accuracy. Primary buphthalmos is relatively uncommon disorder. It is inherited as autosomal recessive trait. It may be present in the siblings. Some of the older members of the family may have early onset of chronic simple glaucoma. In sixty percent cases it is bilateral which need not be symmetrical. Due to some ill understood causes it is more common in boys. The ratio between boys to girls is 3:2. The disease may either be present at birth as true congenital glaucoma, or may manifest in first year as infantile glaucoma. On rare instances the disease may have still late presentation. These are the cases where instead of complete shutdown of flow of aqueous, the flow is slower than normal and rise of tension late and slow. In true congenital glaucoma rise of intraocular tension and its consequences are present in intrauterine life. The disorder is caused due to trabecular dysgenesis of angle of anterior chamber32, 33, rest of the eye does not suffer from any other congenital anomaly, the child does not have any other extra ocular or systemic congenital anomaly. The eye in primary buphthalmos : 1. Enlargement of cornea. The most spectacular changes are seen in cornea, which is enlarged in all directions. At birth the diameter of the normal cornea is 10.5 mm to 11.0 mm, by one year it varies between 11.5 mm and 12.0 mm, at two years it should not exceed 12 mm and enlargement virtually comes to stand still. Corneal diameter more than 13 mm at any age and more than 12 mm at 1 year should be seen with suspicion and investigated for buphthalmos.34 In advanced cases of buphthalmos diameter as high as 18 mm have been reported. The size of the cornea depends on range of intraocular tension and duration of raised intraocular tension. Very high tension cause enlargement quickly. Relatively low tension takes more time to reach the same dimension. 2. Stretching of the cornea. Stretching of the cornea in all meridians cause thinning of the cornea that alters the curvature of the cornea, initially the corneal curvature is increased. The enhanced corneal curvature is not uniform. There is a relative flattening in the centre of cornea. As the cornea stretches, the Descemets membrane gives away giving rise to striated opacities that assume various forms. They start as fine branched striae in the central part, the rupture in the centre looks like a horizontal line while ruptures on the periphery are concentric with limbus. The peripheral ruptures are more common in lower part of the cornea. There may be more than one rupture in the same cornea. The lines produced by rupture of Descemets membrane in primary buphthalmos are known as Haabs striations.

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They may be present in other forms of congenital or developmental glaucoma as well. The lines become opaque and more prominent with time. The Haabs striations must be differentiated from rupture in Descemets membrane due to birth trauma, specially following forceps delivery. Such ruptures are vertical. The Haabs striations may become whirl like sometimes. The ruptured Descemets membrane allows aqueous to permeate into the stroma and cause clouding of corneal epithelium, lacrimation and photophobia. 3. The stretching, clouding, development of opacity in the cornea, relative flattening in the centre of the cornea lead to irregular astigmatism. The stretching is not limited to the cornea, whole of the globe undergoes stretching and enlargement, due to high intraocular pressure in an immature eye. 4. Stretching of sclera : 1. The stretching of sclera is not uniform in all directions. It is most pronounced in anterior posterior diameter followed by horizontal diameter. The vertical diameter is least effected. 2. Due to unequal stretching of the globe instead of becoming an enlarged sphere it becomes more or less like an egg. 3. Over all enlargement of globe specially in anterio posterior axis gives an appearance of pseudo proptosis. 4. The other effect of enlargement of outer coat of the eye is obliteration of the corneoscleral sulcus that brings about significant change in the surgical anatomy of the limbus resulting in accidental placing of sclerotomy too posterior during surgery5. 5. Stretching is most marked in pre-equatorial area than posterior sclera. In long standing cases there may be formation of equatorial or ciliary staphyloma. 5. Anterior uvea. Stretched sclera causes an increase in limbal diameter in all meridians. The ciliary body that is attached to the scleral spur, is shifted peripherally resulting in stretching of the zonules and the iris. The stretched iris becomes thin and the pupil becomes larger than normal. Other cause of large pupil in buphthalmos of course is optic nerve change and tension related iridoplegia in late stages. As the sclera moves more peripherally, the suspensory ligament becomes tight and flatten the lens. A strained suspensory ligament may give way, resulting in subluxation or dislocation of the lens. 6. Anterior chamber. The effect of increased corneal curvature, thinning of iris, and flattening of lens is deepening of anterior chamber. A clear deep anterior chamber with large cloudy cornea in a child should be investigated for primary buphthalmos. 7. Lens. Flattening of lens and sometimes subluxation of lens leads to tremulousness of lens. After sometimes the clear lens or lens with variable degree of opacity may dislocate, commonest direction of dislocation is posteriorly, however large pupil can predispose anterior dislocation. 8. The vitreous, choroids and retina do not show any significant change. 9. Optic disc. The changes in optic nerve in primary buphthalmos are prominent. The changes are mostly due to stretching of the sclera and backwards shifting of lamina cribrosa.

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The optic nerve changes are reversible. If the intraocular tension is brought low and kept low, the optic nerve changes shift towards normal. However after three years when anterior segment enlargement stops, the optic nerve changes become accentuated and progress. 10. Intraocular tension. Intraocular tension is generally above 40 mm Hg., when the child presents for the first time. It may be as high as 80 mm Hg. 11. Error of refraction. Due to increased axial length, the eyes are moderately myopic. This myopia is partially neutralised due to flattening of central cornea, flattening of lens and posterior shift of lens. Myopia is invariably associated with irregular astigmatism. 12. Vision. Vision in an eye with primary buphthalmos is always subnormal, due to : 1. Corneal opacity, 2. Error of refraction, 3. Lenticular changes, 4. Changes in optic nerve head, 5. Amblyopia, 6. Nystagmus. Main diagnostic features in primary buphthalmos : A. The main diagnostic feature of primary buphthalmos lies in the angle of anterior chamber. The angle should be examined by a suitable gonioscope in every eye that has large, cloudy cornea with deep and clear anterior chamber. B. The examiner should have a good knowledge of gonioscopic appearance of angle of anterior chamber in neonate and infants that differs from that of an adult angle. C. The angle of a new born has following features : 1. The angle recess is very narrow. 2. The peripheral iris and blood vessels produce a scalloped appearance. 3. The trabecular sheets are translucent.37, 38 4. The anterior part of the ciliary body and its insertion in the scleral spur is seen clearly. D. The gonioscopic examination in case of primary buphthalmos is done by direct gonioscope of Koeppe or its modification. E. The advantages of direct gonioscopy are : 1. It can be done by placing the child on its back. 2. Both the eyes can be examined simultaneously by using two gonioscopes and the changes compared in two eyes. 3. 360 of an angle is visible at a given time. 4. It can be done either by handheld microscope with suitable illumination or operating microscope. 5. An additional advantage of Koeppes gonioscopy is that direct ophthalmoscopy can be done over the gonioscope because it gives extra magnification. Neonate need not be given general anaesthesia to do direct gonioscopy but a child above three months may not co-operate without sedation, may even require general anaesthesia. If the cornea is hazy due to epithelial edema, the epithelium should be scrapped either mechanically by the blunt side of a cataract knife or chemically by rubbing the cornea with swab soaked in alcohol32,33. Instillation of 5% hyper tonic sodium chloride drop few times may also clear epithelial haze.

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F. Gonioscopic findings34,35,36 : 1. Gonioscopic findings are not uniformly spread all over the angle, it need not be symmetrical in two eyes. 2. The other eye should also be examined even when it is apparently normal. 3. In primary buphthalmos, the anterior chamber is of normal depth before corneal stretching and enlargement ensue, with rise of tension and passage of time the anterior chamber deepens. 4. The angle is wide open. 5. On the inner surface of trabecular meshwork a translucent membrane called Barkans membrane may be present. 6. There is no angle recess.34 7. The peripheral iris is inserted in the line of Schwalbe. 8. The scleral spur is not well visible. G. Measurement of Intraocular tensionIntraocular tension is primary buphthalmos is always raised, it may vary between 40 mm to 80 mm Hg depending upon severity of trabecular block, duration and therapeutic measures undertaken. The tension is measured either by Schiotzs tonometer, Perkins handheld applanation tonometer or tonopen. In children over three years tension should be recorded under general anaesthesia under a competent anaesthetist well versed in paediatric anaesthesia. Allowance should be made for general anaesthesia induced rise of tension. Some of the anaesthetic agents themselves alter intraocular tension. halothene and barbiturates lower tension, while ketamine elevates the tension.38,39,40 Tension recorded in a struggling child is not good enough. Digital recording of tension should always be avoided. Diagnosis A. Diagnosis of full blown case of primary buphthalmos is not difficult, provided there is enough awareness among the attending neonatologist, obstetrician and attending nurse. B. Unless looked for carefully, chances of missing the condition in very high specially in bilateral cases with mild to moderate manifestation. C. Unilateral cases draw early attention of the mother to relatively prominent eye. Besides prominent eye/eyes, other symptoms that point towards possibility of buphthalmos are: lacrimation, photophobia, blepharospasm, and large cloudy cornea. The child with buphthalmos has poor appetite and the child is withdrawn. The child with bilateral disease prefers to be in dark place, keeps both the eye closed and moves the face away from light. In unilateral cases the child keeps the effected eye closed. As the child grows, the symptoms become more pronounced. The child may open the eyes at night due to reduced intensity of light and then it is realised that the child has poor vision. D. A positive history in siblings, in first cousins, require thorough examination of a new born. Similarly children and grand children of persons who had early onset of wide angle glaucoma should also be suspected to have primary buphthalmos and investigated.

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E. The diagnosis is confirmed by measuring intraocular tension, corneal diameter, gonioscopy and evaluation of optic nerve head. Differential diagnosis of primary buphthalmos consists of all cases with A. Watering of the eye. B. Photophobia and blepharospasm C. Cloudy cornea D. Prominent eyeball in a neonate. A. Watering should not be mistaken as congenital nasolacrimal duct obstruction, ophthalmia neonatorum or corneal abrasion. 1. The congenital nasolacrimal duct obstruction generally manifests after three weeks of birth, has mucoid or mucopurulent discharge may have positive regurgitation test. The cornea is bright, anterior chamber depth is normal and pupil of normal size and reaction. 2. Ophthalmia neonatorum develops within first three to four days. It is invariably bilateral, the lids are moderately edematous, discharge is purulent. Regurgitation test is negative, cornea AC and pupil are within normal range. 3. Corneal abrasion are mostly due to birth trauma. The discharge is watery. The abrasion stains with fluorescein, otherwise cornea, AC and pupil are normal. B. Photophobia and blepharospasm are generally due to corneal abrasion, neonatal keratitis and anterior uveitis. Interstitial keratitis comes into differential diagnosis only if a child with lacrimation and photophobia present after three years of age. The cornea in interstitial keratitis has uniform haze due to deep vascularisation. C. Cloudy cornea : Cloudy cornea may be due to 1. Endothelial dystrophy 2. Mucopoly saccharoidosis 3. Mucolipidosis D. Prominent eyeball. causes of prominent eyeball in neonate may be : 1. True proptosis due to a retro bulbar mass and craniocynostosis and congenital myopia. E. Other causes of secondary and associated glaucoma in children should be excluded. Clinically and by specialised investigation like : 1. Ultrasonography 2. X-ray skull and orbit 3. CT 4. M.R.I. The conditions that require special attention are retinoblastoma, retinopathy of prematurity, persistent primary hyper plastic vitreous41,42. Management of primary buphthalmos. The definitive treatment of primary congenital glaucoma is surgery. Medical treatment is employed for temporary reduction of

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intraocular pressure till the facility of surgery becomes available, sometime medical treatment may have to be re-introduced after surgery to control residual glaucoma. Treatment of primary buphthalmos is divided into : A. Specific 1. Surgery 2. Laser 3. Medical B. Ancillary treatment consists of management of 1. Associated errors of refraction. 2. Prevention and management of amblyopia. 3. Low vision aid. 4. Rehabilitation. 1. Surgery consists of various microsurgical procedures.34, 35, 37, 40, 41, 42 They are : (a) (i) Goniotomy through operation gonioscope. (ii) Direct goniotomy in cloudy cornea. (iii) Gonio puncture. (b) (i) External trabeculotomy (ii) Trabeculectomy (iii) Trabeculotomy combined with trabeculectomy. (c) Draining devices (d) (i) Laser (ii) Cryo (iii) Ultrasound (a) (i) Goniotomy. Goniotomy is the preferred surgical procedure for all buphthalmic eyes where surgery is indicated. This is a highly skilled microsurgical procedure done through a suitable operating gonioscope under general anaesthesia. It can be done between one month to two years with excellent result. The success rate falls with increasing age. It is less effective after three years. In this procedure a fine specially designed goniotome is introduced in the anterior chamber via a peripheral corneal stab incision under operating gonioscope and operating microscope. The superficial layers of one-fourth circumference of trabecular meshwork are cut just below the Schwalbes line. Some eyes may require repeat surgery. Prior to surgery, intraocular pressure is lowered to a safe limit by oral acetazolamide 15 mg/kg in divided doses. The pupil is constricted by local pilocarpine. It is a very safe procedure in competent hands, some surgeons prefer to do bilateral surgery. It is claimed that only complication during goniotomy is anaesthesia induced. This type of goniotomy is not possible in cloudy cornea. (ii) Direct goniotomy. This is a less accurate method of opening the trabecular meshwork. This is done in presence of cloudy cornea. The goniotomy knife is passed almost

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blindly across the anterior chamber to reach the trabecular meshwork on the opposite side. The superficial meshwork is cut as in classical goniotomy. This method is less effective and produces more complications. (b) (i) External trabeculotomy is a procedure where the conjunctiva is incised and reflected to expose the sclera near the limbus. A vertical scletotomy is done to reach the canal of Schlemm. The canal is divided, the two cut ends are identified. A specially designed trabeculotome is passed in the canal. Once whole length of the trabeculotome has gone inside, the trabeculotome is swept in the anterior chamber ripping open a passage in the trabecular meshwork. The procedure is less effective than goniotomy. It is not suitable for advanced case where due to altered surgical anatomy of the limbus, it is difficult to identify the canal of Schlemm. (ii) Trabeculectomy. Trabeculectomy is more successful in moderately advanced cases does not require special device like goniotome, operating gonioscope or trabeculotome. The difficulty arises due to changed anatomy of the surgical limbus. A sclerotomy wound may be inadvertently placed too posteriorly, resulting in injury to the ciliary body, loss of vitreous, the lens may be injured during the procedure. The advantage of the method is that it can be done in presence of hazy cornea. Thermal sclerotomy, iris inclusion surgery, corneo scleral trephine, cyclodialysis which were done in past have been given up in favour of trabeculectomy. trabeculectomy with use of anti metabolities is said to give better results.43 C. Artificial drainage device44 or aqueous drainage device (A.D.D.)44. These are miniature artificial drainage devices that45 are transplanted under the Tenons capsule to bypass the trabecular meshwork and canal of Schlemm. They are generally used in advanced glaucoma or where previous surgeries have failed. There are three types of glaucoma devices, the first two are of historical importance and are no more in use the devices are : (a) Seton, (b) Shunts, (c) Valves. (a) Seton. This is a solid rod. One end of which is introduced in the AC and the other end is anchored under the subconjunctival tissue. The aqueous drains by the side of rod by capillary action. The drainage is uncontrolled. (b) Shunt. This is a tubular structure one end of which rests in the anterior chamber. The fluid passes through the lumen passively. This is also uncontrolled. (c) The valves. These are tubular structures similar to shunts, have one-way flow from anterior chamber to sub-Tenons space. The flow is regulated, once the tension falls below a critical level the flow stops. These devices are seldom used as primary procedure. They are used only in eyes where other surgical procedures have failed. These devices are associated with high risk of intraoperative and post operative complications. Common complications of these devices are : Perforation of thin sclera. Injury to the ciliary body

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Vitreous loss Endothelial damage during insertion Intra operative hyphaema Expulsive haemorrhages Persistent shallow AC Corneal decompensation Tube touching the iris Moderate to severe uveitis Closure of the lumen Extrusion of the device Laser in buphthalmos. Q switched Nd-YAG has been used to produce laser goniotomy, otherwise laser is mostly used as cyclo destructive procedure. Either transscleral or through pupil46. Transscleral Diode or Nd-YAG is used to ablate ciliary body as cyclo destructive procedure. Argon or Nd YAG is also used to open the clogged end of aqueous drainage device. Laser is also used to make extra iridotomies if need arises. (a). Medical treatment. Medical treatment have limited role in management of primary buphthalmos. Miotics, the drug used widely in adult wide angle glaucoma is only used to constrict the pupil before glaucoma surgery or iridotomy. It is claimed that pilocarpine may cause paradoxical rise of tension due to collapse of trabecular meshwork because of high attachment of uvea in posterior meshwork42. Beta blockers, alpha agonist should be used in consultation with paediatrician. Acetazolamide and IV Mannitol is seldom required. (b). Ancilliary treatment. This is divided into two main parts : 1. Correction of existing error of refraction. 2. Utilising salvageable vision by low vision aids. 1. The buphthalmic eyes are generally myopic with astigmatism. There may be a change in refractive power following surgery, mostly astigmatism. In uniocular cases amblyopia sets in early and if not managed becomes permanent, vision with best corrected glasses is rarely normal due to corneal opacity, irregular astigmatism, optic nerve changes, residual glaucoma and amblyopia. Complication in buphthalmic eyes : A. First and foremost complication is missed diagnosis. Either the glaucoma is mistaken as simple epiphora or conjunctivitis or these conditions are mixed up as glaucoma and the child exposed to unnecessary investigation. The later is a better option. In all cases of buphthalmos occult retinoblastoma should be suspected and excluded. The child must be examined with an indirect ophthalmoscope and if needed an ultrasound may be ordered. B. Amblyopia squint and nystagmus are common both in treated as well as untreated eyes. C. Persistent lacrimation and photophobia may be seen even when tension has been brought under control.

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D. Large equatorial staphylomas are seen in untreated or poorly managed cases. The thin sclera is prone for rupture that may lead to bleeding, warranting removal of eye. E. Subluxation and dislocation of clear or cataracts lens are occasionally seen. F. The buphthalmic eyes generally have high axial myopia hence they are prone for retinal detachment. Associated and secondary congenital glaucomas32. A heterogeneous group of congenital anomalies of eye with or without systemic manifestation produce glaucoma in children. They are : A. Associated causes of congenital glaucoma. In contrast to primary congenital glaucoma where the pathology lies in the trabecular meshwork with normal cornea and iris. The associate glaucomas may or may not have abnormality in the trabecular meshwork. B. The other group consists of few primary conditions that cause secondary glaucoma. These are : 1. Congenital uveitis 2. Congenital keratitis 3. Intrauterine trauma 4. Neoplasm A. The associated causes of congenital glaucoma is long. Some of the causes are : 1. Iridocorneal dysgenesis (see page 337) 2. Mesodermal dysgenesis (see page 338) 3. Congenital anomalies of uvea : (a) Aniridia (b) Congenital ectropion of uvea 4. Congenital anomalies of globe (a) Microphthalmos (b) Nanophthalmos 5. Congenital anomalies of posterior segment (a) Persistent primary hyperplastic vitreous (b) Retrolental fibroplasia 6. Phacomatosis (a) Struge Weber syndrome (b) Neurofibromatosis 7. Miscellaneous (a) Lowes syndrome (b) Nevus of ota (c) Mucopolysaccharidosis

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Most of the common conditions have been discussed in various chapters except congenital ectropion of uvea, glaucoma in Lowes syndrome and nevus of ota. Congenital ectropion of uvea47. This is non progrgessive, unilateral condition. The pupil is circular, react well to light and accommodation. In normal eyes there is no pigment in the stroma, in this condition iris pigments are present in anterior stroma. Glaucoma is caused due to maldevelopment of angle of anterior chamber. Glaucoma develops in childhood or during puberty. The condition may be associated with neurofibromatosis. Glaucoma in Lowes syndrome. Lowes syndrome is a systemic disorder that involves not only eyes but multiple organs. The ocular manifestation are bilateral congenital cataract, glaucoma, iris atrophy, miosis, microphthalmos. Glaucoma is bilateral in one third of cases. Female carriers have cortical lenticular opacity. Management of glaucoma is difficult, goniotomy may help. Cataract and glaucoma are independent of each other. The systemic involvement consists of mental retardation, renal ricket, aminoaciduria and hypotony48. Nevus of ota. In this condition, there is deposition of melanocytes in the skin, and conjunctiva. This is generally an isolated congenital anomaly or may be associated with nevus of skin on the distribution of second and third division of the trigeminal. Besides melanosis bulbae a small percent of cases develop glaucoma on the side of malanosis. The pigments obscure scleral spur and ciliary band, the trabeculum may be fragmented.46 Glaucoma in older children. Glaucoma in children fall in following broad groups : A. True congenital glaucoma, diagnosed between birth and first three months. B. Infentile glaucoma1 is between three months to three years. C. Juvenile glaucoma1 seen over three years of age. Juvenile glaucomas form a large group of conditions of diverse cause that results in rise of intraocular tension. These are either : 1. Late manifestation of congenital glaucoma 2. Early onset of adult chronic simple glaucoma. 3. Secondary glaucoma (a) Wide angle (b) Narrow angle Common causes of junevile glaucoma are : 1. Associated congenital glaucoma29,30, may be hereditary or non-hereditary. 2. Trauma 3. Uveitis 4. Steroid induced 5. Post surgical status 6. Phacogenic 7. Tumour related

354 8. Other less common glaucomas seen in children are : (i) Neovascular glaucoma (ii) Glaucomatocyclitis crisis (iii) Epidemic dropsy

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From the above list of causes it is obvious that except juvenile open angle glaucoma, all other glaucomas are secondary to local or systemic causes. Most of them are wide angle. Presenting signs, symptoms and management of these glaucomas are identical to adult glaucoma. Traumatic glaucoma in children49 All kinds of trauma mechanical, physical, chemical, radiation can result in raised intraocular tension in all ages. As trauma is more common in male children, incidence of traumatic glaucoma is more common in boys. Most of the eyes have a initial fall of intraocular pressure following injury, this is most marked following penetrating injury due to obvious reason that the wound acts as a large channel for aqueous to drain. In contusion the hypotension is due to reduced ciliary secretion, which builds up later to reach normal level and may remain so, in some cases there is a delayed rise that may take months or year to manifest. Immediate rise of tension is seen due to large hyphaema, swollen lens, cortical material filling the anterior chamber. In chemical burns the immediate rise of tension is due to shrinkage of outer coat of the eye and release of prostaglandin50. Post traumatic glaucoma may sometimes be traced to earlier surgical intervention like aphakia, pseudophakia, penetrating keratoplasty, vitrectomy, retinal surgery, laser capsulotomy and laser photo-coagulation. Post traumatic glaucoma can be divided in following groups : (a) Open globe injury (i) Accidental (ii) Surgical (b) Closed globe injury Contusion and concussion (c) Chemical injuries Glaucoma in non surgical penetrating injury. Immediately after penetration of the globe, the eye goes soft due to the open wound draining constantly at a very high rate. If the wound does not close, the eyes goes into a state of perpetual hypotony, phthisis and blindness. If the wound is repaired surgically or heals by natural process, the intraocular pressure builds up. If there is not much of tissue disruption, the intraocular tension remains within normal range, only on rare occasions the tension may rise after months or years. The tissue disruption following penetrating injury and subsequent repair may lead to secondary angle closure, formation of peripheral anterior synechia, pseudo-angleformation, fibrosis of trabecular meshwork and obliteration of canal of Schlemm. There may be pupillary block due to swollen lens, after cataract, occlusio pupillae, seclusio pupillae, iris bombe, cyclitic membrane. The vitreous may finds its way in anterior

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chamber or block of angle. Angle closure may worsen pupillary block and vice versa. Associated infection, inflammation may also worsen the condition. An associated retained intraocular foreign body may contribute to rise of tension separately or with uveitis. Management of glaucoma in penetrating injury. Best management of glaucoma in penetrating injury is to minimise tissue disruption rectify, tissue disruption that is already present, minimise uveitis by use of local and systemic steroids, antibiotics and cycloplegics. There are two methods of repairing the penetration wound : 1. Repairing of the corneal, scleral and corenoscleral wound with removal of incarcerated uvea, lens and vitreous. A non incarcerated lens may too have to be removed by lensectomy along with anterior vitrectomy. Any retained intraocular foreign body is also removed while doing extensive vitrectomy. The next important step is to reform the anterior chamber, this prevents closure of trabecular meshwork. 2. Primary repair of cornea, scleral wound and excision of incarcerated uvea. This is followed by well planned extensive surgery that include lensectomy, various grades of vitrectomies, removal of foreign body, scleral buckling and reformation of anterior chamber with or without intraocular implant after the primary wound has healed well. Once the glaucoma sets-in, inspite of best surgical management of penetrating injury, the eye may be put on medical therapy of glaucoma by way of local beta blockers, alpha agonist, carbonic anhydrase inhibitors or systemic carbonic anhydrous inhibitors for short period. Uveitis is treated by steroids and cycloplegic. Miotics and latanoprost are contra indicated. If medical treatment fails or the child does not comply with the prescribed regime, best alternative is to undertake any of standard anti-glaucoma surgeries. Laser may be used to do an iridotomy or iridoplasty. Laser trabeculoplasty generally fails. In some cases an aqueous drainage device may be the last alternative. Glaucoma following ocular surgery. Both types of surgical wounds i.e. open globe and close globe wounds may lead to secondary glaucoma. Common intraocular surgeries that are associated with glaucoma are : Needling, aspiration, lensectomy in congenital and traumatic cataracts. Pupillary block in aphakia and pseudoaphakia Repair of penetrating injury Vitrectomy Penetrating keratoplasty Closed globe surgeries that cause post operative rise of tension are : Scleral bucking NdYAG posterior capsulotomy Glaucoma following lens surgery. From the conditions mentioned above it is clear that surgery on lens is a common cause of post operative glaucoma. It can be seen in all ages but more common in children, even a surgery of short duration and minimal handling like needling, aspiration is fraught with rise of tension51 that may be seen between 6% and 24%

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eyes. It is also seen in pseudophakia.52, 53 Glaucoma is more common in anterior chamber IOL and iris supported IOL. Exact cause of glaucoma is not known. Most of the times it is secondary, wide angle glaucoma. The causes are multifactorial. They can be due to one of the following or in combination of more than one factor. 1. Distortion of angle of anterior chamber. 2. Formation of peripheral anterior synecha. 3. Effect of visco-elastic. 4. Pupillary blockVitreous in the pupillary area, cortical material in pupil. 5. Angle closure 6. Uveitis glaucoma haemorrhage syndrome 7. Pigment dispersal syndrome. There is initial fall of tension following incision that remains low for first twenty four to forty eight hours and then builds up to normal range and is maintained with in physiological limits. Occasionally there is acute rise of tension following lens surgery, this is due to relatively small eye53 that is prone for angle closure, closure of angle by visco elastic, and obstruction of pupil and angle by cortical material. The vitreous generally does not fill the anterior chamber in congenital cataract but may be liquefied following injury and associated uveitis of long duration. Pupillary block due to vitreous can be prevented if a good vitrectomy is done along with PC IOL in children. A liberal peripheral iridectomy minimises the pupillary block. An irritable child with chronic redness of the eye, lacrimation, photophobia, should warn against possibility of immediate post lensectomy and IOL related glaucoma. That requires confirmation by measurement of intraocular tension and examination of anterior chamber. In some children there is a delayed rise of tension after years following needling or pseudophakia. This type of glaucoma behaves like a wide angle glaucoma. Management consists of : 1. Prophylaxis. This includes removal of as much of cortical matter from AC and pupillary area, removal of all viscoelastic from AC, reformation of AC by BSS or air, anterior vitrectomy, peripheral iridectomy. Judicious use cycloplegic and steroid to ward off post operative uveitis. 2. Medical treatment. Local beta blockers, alpha agonist. Miotics and latanoprosts are contra indicated. Systemic carbonic anhydrase inhibitors for a short period gives prompt relief to the child. 3. Conventional filtering surgery. Conventional filtering surgery has not been very effective but use mitomycin C and 5 fluorouracil as adjustment have better prognosis. 4. Laser trabeculoplasty has no role in paediatric aphakia/pseudophakic glaucoma. However laser is used to do post-operative iridotomy and iridoplasty to by pass pupillary block. 5. In intractable glaucoma aqueous drainage device may be the last method.

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Uveitis glaucoma hyphaema (UGH). This condition was very common in era of anterior chamber IOL. The AC IOL caused, repeated trauma to the iris that resulted in acute non granulomatous uveitis after months of surgery. It presented as haemorrhagic uveitis with rise of tension. Many factors like 10L itself, ischamia of iris, pressure necrosis of iris, chaffing of iris trigger onset of uveitis. P.C. 10L can also cause UGH if the edge of the lens presses against the ciliary body. Management consists of active and prolonged treatment of uveitis, hyphaema does not require specific treatment. Glaucoma is initially treated by medical methods, if it fails surgery may have to be restored to. Glaucoma following vitrectomy. Fortunately few children require vitrectomy. The conditions that need vitrectomy in children are mostly traumatic with or without intraocular lens implant, lost PC IOL and vitreous haemorrhage. It is thought that post vitrectomy glaucoma is seen in about one fifth of the cases. Post vitrectomy rise of intraocular tension is more common when long acting gases are used as temponade. Early rise is caused by expansion of the gases. Silicone oil also causes rise of intraocular tension. Neovascularisation following vitrectomy is another cause of post vitrectomy glaucoma. Management is similar to any open globe post traumatic glaucoma. Glaucoma following penetrating keratoplasty56. Post penetrating keratoplasty glaucoma is more frequent than diagnosed. Glaucoma following P.K. is a major cause of graft failure. It is more common if PK is associated with lens extraction. Intra capsular lens extraction with penetrating keratoplasty is more formidable combination in producing glaucoma than extra capsular lens extraction. Incidence of glaucoma increases with each repeat surgery. Tight sutures, collapse of trabecular meshwork, small trephine size, donor larger than recipient are probable causes of glaucoma. Malignant glaucoma (ciliary block glaucoma, aqueous misdirection glaucoma57,58. Malignant glaucoma is a rare but most difficult of secondary glaucoma that has both pupillary block and angle closure. Classically this form of glaucoma results in uniform shallowing of both central and peripheral anterior chamber, small pupil, very high intraocular tension that becomes worse with miotics but may be relieved by cycloplegic. Though spontaneous cases without any provocation have been reported. Commonest situation where it is seen is following intraocular surgery. Paradoxically antiglaucoma filtering surgery heads the list of surgical procedures that cause malignant glaucoma. They are : 1. Glaucoma surgeries. Filtering and fistulous procedures, iridectomy, both types of iridotomies i.e. surgical or laser. 2. Lens surgeries. Needling, aspiration, extra-capsular cataract extraction, intracapsular lens extraction with any type of IOL or without IOL. 3. Repair of penetrating corneal or corneoscleral injuries. The condition is most commonly seen in incisional procedures following narrow angle glaucoma. Malignant glaucoma is not related to any specific type of glaucoma surgery. Pre operative intraocular tension also does not influence its clinical presentation. Malignant glaucoma is more common is phakic eyes than in aphakic eyes. Rarely it can be bilateral. There are two mechanisms that work in combination, they are :

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1. Ciliary block57. The normal flow of aqueous is from posterior chamber to anterior chamber via pupil and from anterior chamber to canal of Schlemm through trabecular meshwork. Hardly any aqueous percolates to the vitreous. In case of pupillary block, flow of aqueous from posterior chamber to anterior chamber is impeded but aqueous does not find its way into the vitreous. In case of malignant glaucoma there is severe spasm of ciliary body, the ciliary body moves centrally towards the lens. In this process the ciliary body may come in actual contact with peripheral part of lens when it is present. In case of intracapsular lens extraction the ciliary body may throttle the vitreous cone projecting in the pupillary area. In phakic eyes the iris lens diaphragm moves forwards shallowing the AC and obliterating the angle. Here the second mechanism takes over i.e. misdirection of aqueous flow. 2. Aqueous misdirection55. The aqueous instead of finding its usual anterior path via pupil, drains into the vitreous or behind the vitreous. Retrovitreal accumulation is assisted by posterior vitreous detachment. The ever increasing aqueous pushes the vitreous body forward, which in turn pushes the iris lens diaphragm forward. This along with ciliolenticular block worsens the flow of aqueous via conventional anterior route causing rise of tension. Diagnosis. The condition is diagnosed by severe post operative pain and unexplained diminished vision. On examination the cornea is edematous, the anterior chamber is very shallow, the pupil is constricted and tension is very high. Gonioscopy when possible shows almost obliterated angle. Ocular ultrasonography reveals ciliolenticular block and aqueous in or behind the vitreous. Treatment. Treatment consists of59, 60 1. Lowering of tension by A. reducing formation of aqueous by carbonic anhydrase inhibitors orally and local instillation of beta blockers or alpha agonists. B. Reducing volume of vitreous by IV 20% mannitol, oral glycerine. 2. Relieving pupillary block : A. Use of strong cycloplegic i.e. atropine sulphate 1% drops two times a day. If necessary 5% to 10% phenylepherine or 1% tropicamide every five minutes for five times may be supplemented with atropine Pilocarpine is contra indicated. It worsens the ciliary and pupillary block. B. If medical treatment fails, surgical intervention is required that may be surgical iridectomy, laser iridotomy, rupture of anterior vitreous faces by laser or mechanically in aphakic eyes. In phakic eyes a subtotal vitrectomy may be required. Sometimes aspiration of aqueous entrapped in vitreous may also give relief. Closed globe surgical procedures that cause glaucoma60 : Glaucoma following scleral buckling. Glaucoma following scleral buckling has two phases. The first is intra operative, where indentation of the sclera and tightening of the scleral buckle causes sudden rise of intraocular tension. The raised tension may cause central retinal artery obstruction and if not relieved may cause permanent damage. It is mandatory that all retinal surgeries be done with indirect ophthalmoscope and while tying the buckle, the pulsation in the central artery of retina be monitored. Absence of pulsation requires immediate lowering of tension either by loosening the sutures or intra venous injection of a bollus of acetazolamide or mannitol.

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The second phase is late and chronic rise of tension that develops after six to seven days and may go unnoticed. The tension is rarely very high. These eyes generally have low scleral rigidity so recording of tension by indentation tonometer gives a false low tension. Paradoxically some of the eyes that have undergone scleral buckling may develop hypotony that may last for years. Glaucoma following Nd-YAG posterior capsulotomy, laser trabeculoplasty and iridotomy : All these conditions have two types of pressure elevation. The first - that is seen within one hour following the procedure. This is seen in twenty five percent of all eyes, is transient and controlled by local beta blockers two times a day for two to three days. Possibility of this type of rise of tension should always be remembered in paediatrics patients who have cent percent chance of developing PCO following extra capsular cataract extraction with or without IOL. The acute rise of tension is most probably due to lens particles blocking the trabecular meshwork. The second type of glaucoma is late onset of glaucoma in some patients only. The cause is not well understood. Uveitis as Cause of Glaucoma in Children Glaucoma secondary to anterior uveitis is one of the major causes of childhood blindness.60 Its presentation is so varied that a high degree of suspicion is required to diagnose the condition specially in children where an immature eye is liable to suffer visual loss if glaucoma is not treated well. The condition is over shadowed by the overwhelming signs of anterior uveitis. The pitfall can be eliminated if tension is monitored on every visit. Intraocular tension under local anaesthesia can be recorded in children above five years with little persuasion and reassurance. Similarly recording of vision with P.H. retinoscopy and fundus examination should also be done more frequently. It is better to do gonioscopy examination every three months. Pressure changes in uveitis swing between hypotony to moderate elevation. Generally tension in anterior uveitis is either normal or low to begin with, both the conditions can culminate in glaucoma over a period of time61. Initial low tension in anterior uveitis is caused due to ciliary shut down, which results in hypo secretion of aqueous in presence of normal resistance to outflow. However persistent low tension is an important cause of formation of peripheral anterior synechea that causes increased resistance to aqueous outflow. This leads to gradual rise of IOP which reaches normal range or may overshoot to cause glaucoma. Rise of intraocular tension can be acute, intermediate or delayed63,64. Basically there are two types of secondary post inflammatory glaucoma : (a) Open angle glaucoma and (b) closed angle glaucoma65. The latter can either be with pupillary block or without pupillary block. Incidence of open angle secondary glaucoma is more common than closed angle. Exact mechanism of raised intraocular tension is not well understood. It is multifactorial that may work separately or in combination.

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Probable causes of open angle glaucoma in uveitis66 (i) Spread of inflammation into trabeculum leading to trabeculitis. Edema of trabeculum results in narrowing of trabecular meshwork that impairs outflow of aqueous. (ii) This is worsened by increased viscosity of plasmoid aqueous which finds it difficult to pass through narrow meshwork. (iii) The cellular debris and iris pigments may plug the pores of the meshwork. (iv) Prolonged use of steroid in a steroid responder causes rise of tension. (v) Release of prostaglandin also plays an important role in causing glaucoma. (vi) On long run sclerosis and scarring of trabecular meshwork may be the cause of rise in intraocular tension. Probable cause of angle closure glaucoma in uveitis : (i) Closed angle glaucoma with pupillary block - Extensive posterior synechiae leads to formation of ring synechiae which prevents aqueous to escape through the pupil into the anterior chamber. This pushes the iris forward in the form of iris bombe narrowing the peripheral part of anterior chamber and formation of peripheral anterior synechiae. However 360 of posterior synechiae without iris bombe can also cause rise of tension. This is worsened if the pupil is small and covered by exudate. Thickened iris, miosis and plasmoid aqueous also raise tension, so does release of prostaglandin. (ii) Closed angle glaucoma without pupillary block. This is mostly due to deposition of inflammatory debris on the trabecular meshwork, subsequent contracture of iris over the trabecular meshwork. A paradoxical situation arises when dilation of pupil causes embarrassment of the angle. This happens if the angle is already narrow. Multiple peripheral anterior synechiae may form a pseudo angle proximal to trabeculum preventing aqueous to reach true angle. Swollen ciliary body may push the lens iris diaphragm forward, narrowing the angle, resulting rise of IOP. Diagnosis of glaucoma in anterior uveitis is often missed unless its possibility in every case is kept in mind. It is a misconception that glaucoma is rare in children in uveitis. It is a major cause of childhood blindness62. In all cases where vision is low and does not improve with PH, tonometry, retinoscope and ophthalmoscopy should be performed. Gonioscopy helps to differentiate between an open and closed angle. Management. In acute stage and to some extent chronic rise of tension can be lowered by usual treatment of anterior uveitis with cycloplegic and steroids otherwise management depends whether it is an open angle or close angled glaucoma. Open angle glaucoma in uveitis is managed by : Continuing treatment with cycloplegic and steroid/non steroidal anti- inflammatory drugs. Treatment of open angle glaucoma by instillation of local, beta-blockers, alpha agonist or carbonic anhydrase inhibitors. The last is least effective in children, miotics and prostaglandin analogs are contra indicated.

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Systemic use of carbonic anhydrase inhibitors should be employed for short period. Slow release, CAI are better tolerated by children. Surgical treatment consists of modified goniotomy 64 , trabeculodialysis and trabeculectomy. In rare cases aqueous drainage devices may be required. Closed angle glaucoma with pupillary block is managed by establishing a passage between the anterior and posterior chamber first therapeutically and if it fails either by laser or by surgery. Therapeutic measures used are strong cycloplegic to break the synechiae. Frequent use of steroid helps in lysis of synechiae. Addition of epinephrine has synergistic action with cycloplegic that enhances mydriasis. Betablockers and alpha agonists do not have any action on size of pupil, they are used to lower the intraocular tension. Miotics and latanoprost are contra indicate. Acute rise is controlled by oral carbonic anhydrase inhibitors. The argon laser or NdYAG laser iridotomy establishes a permanent passage between anterior and posterior chamber. Surgical iridectomy is indicated when facility of laser iridotomy is not available or when such procedure has failed. Closed angle glaucoma without papillary block is treated medically by betablockers and alpha agonist, surgical management consists of trabeculactomy. Steroid Induced Glaucoma in Children Steroid induced glaucoma is an avoidable iatrogenic glaucoma. It is seen in steroid responders and those who have family history of chronic open angle glaucoma. It can occur in any age and sex. However it is more frequent in children67. Steroid induced glaucoma is secondary open angle glaucoma. Its physical findings are similar to COAG. Pressure elevation depends on many factors that include - response to steroids, family history of chronic open angle glaucoma, strength of drops used, frequency and duration of steroids used. It is more common in steroids like prednisone, prednisolone, dexamethsone, beta methasone, and triamsonolone. It is less common in steroids like medrysone and flurometholone67. However none of the steroids are free from intraocular tension raising property. Generally glaucoma becomes evident after three weeks of instillation of dexa or beta methasone in high steroids responders. It may take months in low responders with same regime. All routes of administration of steroids are fraught with glaucoma. Commonest being ocular use as drops or ointment. This is followed by periocular use. Systemic use of steroids are more likely to result in glaucoma than skin ointment, inhalers and nasal spray. On the top of the list of common ocular conditions that culminate in steroid induced glaucoma in children is spring catarrh where unnecessary, prolonged use of steroids that use cortisol as foundation are the most offending drugs. This is followed by anterior uveitis, trauma both accidental or surgical. The surgical condition that need to be monitored for rise of IOP are : aphakia, pseudoaphakia, glaucoma surgery and keratoplasty. The exact mechanism of rise of tension following use of steroid is not known. It is said to be enzyme related.65 Clinical presentation of steroid induced glaucoma in children is similar to that of chronic open angle glaucoma. Only difference is that in most of the eyes with steroid induced glaucoma

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tension falls within normal limits in two to three weeks after stoppage of drug. Associated field and fundus changes are permanent, these changes may be confused as due to low tension glaucoma in later life. The eye is non congested except in spring catarrh where the eyes remain red due to existing allergic conjunctivitis. The anterior chamber is of normal depth and the angle is wide open. In rare instances where steroid induced glaucoma develops before three years of age, the eye may be buphthalmic. All children under steroid therapy local or systemic, should be warned about possibility of glaucoma, these children should get their IOP and vision recorded every three months and disc evaluated twice a year. Treatment consists of : 1. Prophylaxis 2. Medical treatment 3. Surgical treatment 1. Prophylaxis : (a) Awareness among treating ophthalmologic, paramedical personnel, pediatrician, general physician and dermatologist about possibility of steroid induced glaucoma in children. (b) Parents should be warned about possibility of steroid induced glaucoma especially in spring catarrh. It should be emphasised upon them that, no child goes blind due to spring catarrh but hundreds of children loose sight due to steroid. (c) use of lowest dose of steroid for shortest period that gives the child comfort. (d) Residual allergy, inflammation should be treated by non steroidal anti- inflammatory drugs, local antihistamine and astringents. 2. Medical treatment : (a) Once it has been established that the child has steroid induced glaucoma, the drug should be tapered and steroid replaced by non steroidal anti- inflammatory drugs. This generally brings down the tension to normal range. (b) If tension remains high in spite of discontinuation of steroid or steroid can not be discontinued, the tension is brought down by miotics, beta blockers, alpha agonist, epinephrine and its pro-drug. In case the child was suffering from uveitis, miotics and latano prost are contra indicated. 3. Surgical treatment : Only a few children may require surgical intervention. Eyes with steroid induced glaucoma do not respond well to laser trabeculoplasty. Surgery of choice is trabeculectomy. Glaucoma following blunt injury can be caused by : (i) Blood and blood constituents in anterior chamber.

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(ii) Recession of angle of anterior chamber. (iii) Phacogenic Glaucoma due to blood and blood constituents in the anterior chamber constitute a group of conditions that are grouped under hemogenic glaucoma69 that may present as : Glaucoma secondary to total hyphaema Hemolytic glaucoma Ghost cell glaucoma Out of these, glaucoma secondary to total hyphaema red cell glaucoma67 is most common following severe blunt injury. The eyeball is compressed anterio posteriorly pushing the aqueous laterally. The force of compressed aqueous acts as a shearing force at the angle and causes damage to the root of the iris, and ciliary body. Normal iris generally does not bleed between the root and the pupil but may bleed when injured at the root. The ciliary body bleeds more frequently than iris. The bleeding may occur within few minutes of injury or may be delayed for three to four days. Bleeding after four days is more severe than initial bleeding. Rebleeding is frequent. There is an acute rise of tension as blood fills the whole of the anterior chamber blocking the trabecular meshwork all around. The blood especially the clotted blood may cause pupillary block, worsening the condition. The rise of tension varies between moderate to very high. With sudden rise of tension there is severe ocular and periorbital pain. Pain radiates on the same side of the head. The eye is generally congested with lacrimation, vision is reduced to perception of light or at the most counting finger from close quarter, vision improves as blood settles down at the lower parts of the anterior chamber. The other cause of diminished vision and lacrimation is associated corneal haze. The child is generally irritable may have bouts of vomiting. The fresh blood in anterior chamber is visible through the transparent cornea. The parents may be able to say that there is blood in the eye. With passage of time, the blood starts clotting and its colour becomes darker, ultimately it assumes black and oval shape, such a condition is called eight ball or black ball hyphaema. There may be re-bleeding, that may be spontaneous or follow repeat trauma. There may be rebound bleeding following instillation of epinephrine or its derivatives. Total hyphaema of more than five days duration and raised intraocular tension is most likely to develop blood staining of cornea. Blood staining of cornea is due to infiltration of endothelium and posterior stroma by blood constituents. As the epithelium is also effected there is associated haze of cornea with epithelial edema. Glaucoma secondary to total hyphaema requires early treatment to : 1. Prevent blood staining of cornea, which may last for years causing prolonged diminished vision that leads to squint and amblyopia. 2. Protect the optic nerve from glaucomatous neuropathy and permanent visual loss.

364 Management of glaucoma secondary to total hyphaema : The management consists of : 1. Immediate steps to prevent glaucoma. 2. Management of glaucoma.

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Immediate management is essentially treatment of total hyphaema, which consists of : 1. The child is hospitalised to minimise movement. 2. The affected eye is bandaged over a pad. Bilateral patching does not have advantage over unilateral bandage. 3. The head end is kept raised. This helps the heavy blood particles to settle down at the bottom of the AC to facilitate opening of trabecular meshwork in the upper part. 4. Instillation of local drugs71 - Miotics are contra indicated as they may worsen associated anterior uveitis. Epinephrine is known to cause rebound bleeding hence it is also not used. cycloplegics give relief to cyclopasm and reduce pain. Role of local steroid has not been proved beneficial in every case. Beta blockers are helpful to keep IOP low hence they may be used not only in proved glaucoma but also as prophylaxis against rise of tension. If the child bleeds repeatedly without trauma, the child should be investigated to exclude sickle cell disease or other bleeding disorders. The child may be put on oral fibrinolytic i.e. a aminocaproic acid in a dose of 50 mg/kg/ day in consultation with paediatrician. Raised intraocular tension is brought down by : 1. Oral carbonic anhydrase inhibitor in dose of 10mg-20mg/kg/day in divided dose with potassium supplement. 2. Oral glycerine in dose of 1 to 1.5 cc/kg/day in two divided doses. 3. Intravenous mannitol in dose of 1 gm to 1.5 gm/kg may be given in fast drip. This is generally done in cases where paracentesis is planned. If tension does not come down within forty-eight hours with medical treatment, blood is evacuated either by simple paracentesis or washed with balanced salt solution. To clear clotted blood two stab incisions are given, one at temporal side of the cornea and the other opposite to it. Through the first viscoelastic is introduced which forces the clot out through the other hole.75 Visco-elastic itself is then washed with BSS and anterior chamber reformed, the eye is observed for rebleeding and rise of tension. Once the acute episode has passed, the eye is kept under watch for delayed rise of tension over years which is generally due to recession of angle of anterior chamber. (see page 265). (b) Hemolytic glaucoma69,73. This secondary wide-angle glaucoma is less common than glaucoma associated with hyphaema. It may be an early or late feature in blunt injury. In hemolytic glaucoma the break down products of blood in anterior chamber i.e. red blood cells or hemosiderine are phagocytosed by large macrophages that plug the trabecular meshwork and cause rise of intraocular pressure in milder form, the condition is self limiting. However it is better to keep the tension within normal range in all cases of hyphaema by

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betablockers and alpha agonist. Miotics epinephrine and latanoprost are contra indicated. Some clinicians feel that hemolytic glaucoma is a milder form of ghost cell glaucoma. Ghost cell glaucoma69, 70, 73. The secondary open angle glaucoma was previously known as erytholastic glaucoma.75 It is mostly seen in vitreous haemorrhage with broken anterior vitreous face. It is also met within total hyphaema especially in eight ball hyphaema. In adults it is mostly seen following lens extraction and pre-existing vitreous haemorrhages. If the vitreous face gives away, the blood finds its way to anterior chamber to cause ghost cell glaucoma. In children traumatic vitreous haemorrhage with subluxated or dislocated lens can cause ghost cell glaucoma. The mechanism of glaucoma is multifactorial. The RBCs which are circular, biconvex, and pliable can pass through trabecular meshwork without any obstruction. When these are converted into larger, spherical, rigid bodies in the vitreous, these cells contain denatured haemoglobin called Heinzbodies76 and are called ghost cells. The ghost cells due to rigid walls and spherical shape, fail to pass through the pores of the trabeculum causing rise of intraocular tension. These cells may collect at the bottom of the anterior chamber with a soft-white or khaki coloured pseudo hypopyon. It takes red cells ten to fifteen days to be converted into ghost cells in the vitreous. Rise of intraocular tension that is mild to moderately is directly proportionate to number of ghost cells present, with too many ghost cells, the IOP may be raised sufficiently to cause pain, photophobia and diminished vision due to corneal edema. Management consist of lowering of intraocular pressure by standard anti-glaucoma drugs and keep it within normal range with minimum drug. If medical treatment fails, standard surgical procedure may be required. Miotics epinephrine and latanprost are contra indicated.73 Glaucoma secondary to angle recession71, 77, 80. Angle recession glaucoma may develop months or years after blunt injury. Many a times the patient may even forget about receiving blunt injury in childhood or may think such trivial incident not worth mentioning. This makes enquiry about blunt trauma mandatory in all cases of uniocular wide angle glaucoma. Angle recession glaucoma is generally uniocular. Being traumatic it is more common in boys. In the beginning, it may be masked by hyphaema and hemogenic of glaucoma. Initial hypotony, which is very common, does not exclude possibility of angle recession glaucoma in future. About ninety percent of eyes with blunt injury have some degree of angle recession, only ten percent of eyes with angle recession develop glaucoma. Recession may involve only a small sector or may be extensive. Recession less than 180 is less liable to develop glaucoma. Recession more than 270 is most likely to develop glaucoma. The recession of angle which is in fact a laceration on the face of ciliary body that extends between the longitudinal and circular muscle develops immediately following blunt injury. The recession may initially be missed due to corneal changes and associated hyphaema or when not suspected. Hence all eyes with blunt injury should undergo gonioscopic examination after six weeks. Bilateral gonioscopy facilitates comparison between normal and injured eye.

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The parents of injured children should be told about possibility of glaucoma in later life and instructed to get the childs eye examined for vision, rise of tension, changes in angle and disc every six months and then yearly. It has been widely noticed that angle recession glaucoma is more common in persons who have positive history of wide angle glaucoma in the family. The child may develop chronic simple glaucoma in the uninjured eye later. The exact mechanism of development of glaucoma in angle recession is not well understood. One point is established that angle recession perse does not cause glaucoma. It is the effect of trauma on the trabeculum that is the prime suspect in producing glaucoma. A membrane like structure covers the trabecular meshwork facing the pupil, which is actually proliferation of Descemets membrane. The force may extend in the trabecular meshwork and disrupt it that heals by scarring. The force may rip off the ciliary body from the scleral spur and cause cyclodialysis. Iridodialysis is also very common. Diagnosis is confirmed by gonioscopy. Bilateral gonioscopy by Koeppe lens helps to compare the findings of injured eye with the normal eye, slightest deepening of AC on the injured side is always suspicious finding. Frank tremulouness of iris with deep chamber denotes subluxation of lens. Common findings include mild to moderate mydriasis, widening of ciliary band. The width of ciliary band is irregular, whitening of scleral spur and the trabecular meshwork. The trabeculum may be sprinkled with iris pigment giving a false impression of pigmentary glaucoma. The angle is wide. Treatment of angle recession glaucoma is similar to any secondary open angle glaucoma, which consists of local instillation of betablockers or alpha agonist. Pilocarpine is not favoured because it is presumed that in a traumatised eye with damage to the trabecular meshwork, the outflow is diverted to uveo-scleral outflow that is impaired by miotic79 hence latanoprost may be more effective than miotic. Sustained released carbonic-anhydrase inhibitors may be given orally for short term, local carbonic anhydrase do not have much effect in children. When glaucoma can not be brought under control, surgery may have to be employed. Argon laser trabeculoplasty does not give uniform result78. Conventional trabeculectomy with mitomycine-C or 5 fluro uracit gives better results77. Traumatic phacogenic glaucoma Following blunt injury, the lens may be dislocated or subluxated. Blunt injury rarely cause fast opaification of lens which is very common in penetrating injury. Hence blunt trauma rarely causes phacogenic glaucoma except when either the dislocated lens has come in AC or is trapped in pupil. Rarely the lens may become intumescent and cause both pupillary and angle closure glaucoma. Glaucoma in post surgical status (page 355) Non traumatic Phacogenic glaucoma in children81, 82. Lens may cause glaucoma by any of the following causes separately or in various combinations. The causes can be congenital manifesting in childhood or acquired. Opaque lens is more likely to cause glaucoma than transparent.

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(a) Phaco-morphic (i) Sherophakia (ii) Microphakia (iii) Tumescent (b) Phacotopic (i) Congenital ectopic (ii) Acquired Subluxated Dislocated Incarcerated in the pupil. (c) Phacolytic (i) Cortical matter (ii) Due to leak of protein (iii) Phacoanaphylactic Phaco morphic glaucoma. This type of glaucoma is caused by a small relatively spherical lens that closes the pupil like a ball and socket. The pupil may either be normal or miotic. Small pupil is more likely to cause pupillary block glaucoma. This type of glaucoma is relieved by mydriasis and is called glaucoma inversus or paradoxical glaucoma. Intumescent lens. Intumescent lens as cause of glaucoma is mostly seen in age related cataract where a lens swells to cause pupillary block and secondary angle closure. The same effect is brought about in children when aqueous finds its way through a microscopic wound mostly in blunt trauma and causes the lens to swell. The swollen lens causes pupillary block and secondary angle closure. Management consists of lowering of tension by oral acetazolamide, local beta blockers. Local steroids may be helpful to supress associated uveitis. Once tension has been brought to safe limit an ECCE with PCIOL is the best available treatment. Cortical matter glaucoma. This is a step ahead of intumescent of lens. Here the capsule is ruptured either accidentally as following penetrating injury or has been surgically cut to perform ECCE. The condition is very common following needling in children either for congenital or traumatic cataract. The anterior chamber is filled with soft cortical matter that blocks both pupil and the angle. Management consists of removal of as mush of cortical matter as possible. Microscopic leak of denatured lens protein, which does not cause antigen antibody reaction is ingested by macrophages that swell up and block the trabecular meshwork leading to secondary open angle glaucoma. Management consists of lowering IOP by any standard method followed by removal of lens. Phacoanaphylatic glaucomaIn contrast to above, in rare instances, the lens protein acts as an antigen and causes severe local reaction in the form of anterior uveitis and is associated with glaucoma. This is rare in children.

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Management consists of control of uveitis, lowering of intraocular tension to a safe limit and removal of lens. Chemical injuries as cause of glaucoma83. Both types of chemicals i.e. alkali or acids cause initial rise of tension for first few hours to few days, then it comes to normal. This initial rise of tension is attributed to shrinkage of sclera and cornea and release of prostaglandin.80 This phase is followed by return to normal tension. The tension may be established within normal range and remain so but in many eyes there is rise of tension due to continued action of prostaglandin, uveitis and tissue disruption brought about by action of the chemicals. Management consists of immediate management of chemical burn by standard method, administration of carbonic anhydrase inhibitors for short period. Local betablockers and alpha agonist. Miotics are contra indicated. Frequent instillation of steroids reduces uveitis, if tension is not controlled a fistulising surgery with anti metabolites may be required. In some cases glaucoma implants may be the last resort. Tumour related glaucoma84, 85, 86. All types of intraocular tumours, benign or malignant cause glaucoma. In adults common tumours producing glaucoma are related to uvea, followed by intruocular metastases. In children the common tumours causing glaucoma are retinoblastoma81,82, diktyomo, juvenile xanthogranuloma, leukaemia. Benign hamartomas that produce glaucoma are von Recklinghausens syndrome and Sturge Weber syndrome. Glaucoma by Sturge Weber syndrome inflicts children while that due to Von Recklinghausens syndrome causes glaucoma in young adults. Tumour related glaucoma are generally unilateral. They can cause both open angle as well as narrow angle glaucoma with or without pupillary block. Severity of glaucoma depends on size, location and duration of the growth. Common causes of tumour related glaucoma are86 : (a) Direct infiltration of the angle by tumour. (b) The eyes have associated congenital anomalies (phacomatosis). (c) Angle may be blocked by tumour cells present in anterior chamber as pseudohypopyon (Retinoblastoma). (d) The iris lens diaphragm may be pushed by a large tumour mass. (e) Neovascularisation of angle (Retinoblastoma). (f ) Raised episcleral pressure. All eyes with unexplained unilateral rise of intraocular tension should be examined well, to exclude possibility of intraocular tumour by, indirect ophthalmoscope, gonioscope and ultrasonography. Management of tumour related glaucoma is essentially treatment of primary cause specially if it is life threatening like retinoblastoma or leukaemia. Neovascular glaucoma. Neovascular glaucoma in children is far less common than neovascular glaucoma in adults. Diabetic retinopathy and central vein thrombosis are two causes that top the long list of causes in adults.87, 88 These conditions are not seen in children. Common causes of neovascularisation glaucoma in children are retinoblastoma, Coats

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disease, retrolental fibroplasis. Neovascular glaucoma can be either open angle or closed angle secondary glaucoma. Management of neovascular glaucoma in children is as difficult as in adult and does not differ much. Miotics are contra indicated. Tension should be brought down by betablockers or alpha agonist. Local steroids and cycloplegics help to reduce associated inflammation. Filtering surgeries are not very effective. Best results are cyclodestructive procedures i.e. transcleral cyclocryo, and Nd-YAG cyclophto coagulation. Glaucomcyclitis crisis. This is rarely seen in children. It may present as unilateral periodic rise of tension in late teens with signs of cyclitis. The affected pupil is larger than the other. There are signs of cyclitis. The rise of tension is periodic. Each episode may last from few hours to few weeks. During crisis the tension may reach between 40 mm to 60 mm coming down even without treatment89. Persistent raised tension is controlled by betablockers. Associated uveitis is treated by local steroids and rarely by mydriatic. Surgery is contra indicated. EPIDEMIC DROPSY GLAUCOMA90, 91, 92, 93 Epidemic dropsy is an acute toxic systemic condition with multi-systemic involvement seen as epidemics in certain areas of the world. The endemic countries are mostly located in South East Asia, Fiji and South Africa. Worst effected country is India, specially eastern and northeastern states where mustard oil is used as cooking media or anoint. Epidemics have been reported from other parts of India from time to time outside the endemic state. Mustard oil itself does not cause the diseases. Epidemic dropsy occurs only when edible oil even other than mustard oil gets contaminated by oil of argemone mexicana (yellow prickly poppy) that grows as weed with mustard plants. Mustard oil may be adulterated intentionally or may be contaminated accidentally95, 96, 97. Argemone mexicana contains sangunarine and dihydro sangunarine that cause rise in blood pyruvate level which is thought to be the cause of the disease93. Ocular symptoms are late to develop following systemic involvement. Systemic features are94, 95 1. Warm erythromatous, tender, hypopigmented skin lesions. 2. Pitting edema of feet and legs. 3. Non specific cardiac failure leading to dyspnea. 4. Diarrhea. 5. Malaise and fever. In severe cases the condition can be fatal due to cardiac failure. Ocular signs and symptoms develops six to eight weeks after development of pedal edema. Glaucoma develops mostly after subsidence of systemic signs and symptoms. Glaucoma in epidemic dropsy is bilateral, involving both the sexes. No age is immune. Children in first decade may not be affected. It is as common in children in second decade as in adults.

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The glaucoma is wide angle glaucoma, with normal chamber and angle. Outflow is within normal limits. The aqueous shows raised level of histamine, prostaglandin and protein suggesting that the glaucoma is hypersecretory. There is no anterior chamber reaction. The eye is white, cornea may show haze due to rise of intraocular tension that may be as high as 60 to 80 mm Hg. The patient may only complain of irritation of the eye, coloured haloes, round the light which is marked and persistent. The disc and field changes are similar to those seen in chronic simple glaucoma. The exact mode of hypersecretion is not known, it is said to be of central origin. Other ocular findings are toxic vasculitis, venous dilatation and superficial haemorrhages. In milder form the systemic features subside with discontinuation of adulterated oil from diet. It is worth noting that epidemic dropsy can be caused by consuming other vegetable oils that have been adulterated not only by argemone maxicana but also other chemicals like some mineral oils. Glaucoma starts late and may linger on for long time. It is partly self limiting. Even when it subsides with or without treatment it leaves stigma in the form of glaucomatous disc and corresponding field changes. All patients with juvenile chronic simple glaucoma should be asked about their food habits specially about cooking medium. Management. Glaucoma being hypersecretory does not respond to pilocarpine. Drugs that reduce aqueous formation like epinephrine, its product dipivefrin give better results. Topical beta-blockers also give favourable results. Systemic acetazolamide is employed for short term relief. Systemic indomethasin is used as adjunct with topical drops. It takes few weeks to months to bring the tension to normal range. Once this has been reached the drugs are gradually withdrawn and maintained on minimum dose. The patients are followed up twice a year for next two to three years. If tension can not be brought down, by medical treatment any of the standard filtering surgeries can be employed. Juvenile primary open angle glaucoma98. Juvenile glaucoma is a non specific term used to designate a large group of conditions that cause rise of intraocular tension in a patient after three years of age and before third decade. Most of these conditions are secondary or associated glaucomas30, 31, 32. These may be wide or narrow angled. In some cases pupillary block may be present. In contrast to above conditions juvenile primary open glaucoma is a term that designates a specific disorder, which is similar clinically to adult primary open angle glaucoma with a distinct genetic predisposition98. Some people consider juvenile primary open angle glaucoma to be the late onset of congenital glaucoma where trabecular meshwork was not obstructed enough to cause rise of intraocular tension before three years and was not open enough to maintain normal outflow after three years. Others postulate that the condition is early onset of adult primary open angle glaucoma. Incidence of juvenile primary open angle glaucoma is far less than its secondary counterparts, commonest age of diagnosis is eighteen years. The range being eight to thirty years. The condition is bilateral. There may be a history of P.O.A.G. or myopia in the family. The condition is autosomal dominant. It is more often missed than diagnosed till it is advanced.

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The cornea77 is devoid of stigma of congenital open angle primary glaucoma, or traces of mesodermal dysgenesis. Only suspicious corneal change may be an enlarged cornea. There is no evidence of trauma, uveitis or use of steroids. The children are brought only for diminished vision. On examination the child may be myope. Positive signs that warrant measuring of intraocular tension are changes in the optic nerve head. There may be asymmetry of the cup, notching of the cup, change in CD ratio. Tension can be recorded under local anaesthesia either by Schiotzs tonometer or handheld applanation tonometer in children as young as six years provided the child is explained that the procedure is painless, but is an essential procedure for treatment. Generally the child is more co-operative during subsequent visits. No touch tonometer is better alternative. The tension may be as high as 40-50 mm kg. The anterior chamber is clear with normal depth, the angle is wide without any uveal tags, synaechia or pigments. The field changes are similar to those seen in adult primary wide angle glaucoma. Treatment : The principle and mode of management is same i.e. medical/surgical as in adults. I. Medical treatment consists of (a) Local instillation of 1. Direct acting miotics, 2. Beta blockers, 3. Alpha agonist, 4. Epinephrine and dipiveprin, 5. Local carbonic anhydrase inhibitor. (b) Systemic carbonic anhydrase inhibitors for short term. II. Surgical treatmentSurgery is indicated if local treatment fails to keep the tension within normal range. 1. Goniotomy has uniformly poor result over three years of age. 2. Trabeculectomy gives better results. However the results are not as good as in adults because children have increased tendency for subconjunctival fibrosis that obliterate the opening. The fibrosis can be minimized by use of antimetabolites. Besides keeping intraocular tension within normal range and stabilising the field changes, the childs refractive error should also get due importance. These children are invariably myopes. There is always a change towards myopic astigmatism following filtering surgery. Uniocular poor vision usually produces amblyopia that should be managed by standard procedures. Outline of various modes of management of glaucoma. Aim of management of glaucoma is to lower the intraocular tension, to make it compatible with vision and stop the progress of field and fundus changes. Fundus changes are reversible under three years of age. This can be brought about by : A. Medical treatment B. Surgical treatment C. Non invasive physical methods A. Medical treatment comprises of 1. Local drops 2. Systemic drugs

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1. Local drops mostly act on autonomic nervous system either as agonist or antagonist can either be : (a) Parasympathomimetic99, 100, 105 : (i) Direct acting (ii) Indirect acting (iii) Combined (b) Beta blockers : Non selective. These block both beta one and beta two receptors. Selective. These are also called cardio selective. They are more potent beta one blocker than beta two. (c) Sympatho-mimetics, they can be : (i) Alpha adrenergic agonists (ii) Beta adrenergic agonists (Adrenergic antagonists are no more in vogue in management of glaucoma) (d) Prostaglandin analogue (analogs) Latanoprost (e) Prostamides (LumiganBimatoprost .005%) (f ) Other ocular hypotensive lipids (g) Local carbonic anhydrase inhibitors : (i) Short acting CAI (ii) Delayed acting CAI. 2. Systemic drugs (a) Carbonic anhydrase inhibitors (i) Short acting 1. Oral labels 2. Injectables (ii) Delayed acting (b) Hyperosmotic agents (i) Oral glycerol Isosorbide (ii) Injectable Mannitol Urea The anti-glaucoma drugs can be put in two groups i.e. (i) Those reduce formation of aqueous (ii) Those that increase outflow. The drugs that decrease formation of aqueous are Beta blockers, epinephrine, depivprin, apraclonidine, brimonidine and C.A.I.

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Those that increase outflow are : Miotics, epinephrine, depiveprin, brimonidine and latanoprost. Hyper osmotic agents are altogether different from above two groups, they act by increasing blood osmolarity and drawing water from vitreous. Direct acting parasympathomimetic drugs. Pliocarpine is an alkaloid100 used either as nitrate or chloride in strength of 1% to 2% and rarely 4%. Percentage higher than 4% do not have any additional advantage however side effects increase with increased concentration. It has best IOP lowering effect at pH 6.5. Pilocarpine is mostly used as drops102. However it can be used as gel, ointment, ocuserts and soft contact lens soaked in pilocarpine101, 102. Action of pilocarpine starts with in 20 minutes and passes off in 4 hours. Stronger solutions have more prolonged effect. Hence drops in lower strength have to be used more frequently, action of gel and ocusert last longer than aqueous solution. Addition of betablockers and alpha agonist have synergistic effect on pilocarpine and vice versa. Pilocarpine when used alone has to be instilled four times a day. This frequency is reduced to twice a day when used with beta blockers or alpha agonist. Pilocarpine acts directly on cholenergic receptors. Most visible action of pilocarpine is miosis. It also causes cyclotonia, increasing ciliary tone. Cyclotonia and miosis are inseparable but they lower IOP in two different ways. Miosis is effective in lowering IOP by pulling the iris away from the trabecular meshwork thus opening a mechanically closed angle in primary closed angle glaucoma. The miosis has an adverse effect i.e. pupillary block. Synecheal blocks are however not relieved by pilocarpine. In primary open angle glaucoma, pilocarpine contracts the longitudinal fibres of ciliary body which being anchored to scleral spur, automatically pull the scleral spur and trabecular meshwork, opening the meshwork thus increasing aqueous outflow. Pilocarpine in no way acts on production of aqueous. It has an adverse effect on uveoscleral outflow. Hence pilocarpine is contra indicated in permanent secondary angle closure. Frequent instillation of pilocarpine increases permeability of uvea blood vessels leading to derangement of blood aqueous barrier. This results in plasmoid aqueous rendering pilocarpine unsuitable in inflammatory and neovascular glaucoma. Pilocarpine has no role in buphthalmos. Adverse effects of pilocarpine are local as well as systemic. Side effects depend upon strength, frequency, duration and amount of systemic absorption. Immediate local side effects is burning in the conjunctiva. This is pH dependent, lesser the pH more is the sting, pH 6.5 to 7.5 is better tolerated than ph 4.0. Conjunctival congestion, miosis and spasm of accommodation are other local side effects. Miosis leads to following adverse effects. Distant vision is drastically reduced in presence of central corneal and lenticular opacities, night vision is diminished due to nondilatation of pupil in dark. Generally there is constriction of peripheral field and apparent increase in size of field defect. Cyclotonia leads to spasm of accommodation, transient myopia and frontal headache. Delayed effect consist of epiphora due to spasm of puncta and canaliculi. Cholinergic effect of pilocarpine on lacrimal gland enhances tear production.

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In myopic eyes strong miotics are known to produce retinal detachment and subscapular opacification of lens. Prolonged use of pilocarpine may produce iris border cysts, which can be prevented by use of short acting mydriatic like phenylepherine from time to time. Abnormal tear film status is a common feature of prolonged use of pilocarpine. Increased salivation, colic and diarrhoea are some systemic effects. Other miotics are not used in children to treat glaucoma. B. Sympathomimetic drugs (adrenergic agonist). There are two types of drugs in this group. The drugs that stimulate both alpha and beta receptors are known as non selective alpha agonist. Those drugs that stimulate only alpha two receptors are called selective alpha agonist. These drugs increase outflow facility and to some extent reduce formation of aqueous. The drugs in the first group are : Non selective alpha agonist are : (i) Epinephrine (ii) Depivefrin I. Epinephrine. Epinephrine is used as aqueous drop, commonly used strength is 1% to 2% given either once a day or at the most two times a day. The salts of epinephrine available are hydrochloride, bitarterate and borate. Out of all salts the borate is least irritating due to its pH 7.4, which is nearer to pH of normal tear. Epinephrine directly stimulates both alpha and beta receptors. The IOP lowering mechanism of epinephrine is most probably a dual one, the first an early but short period of decreased aqueous formation and the later a long period of increased outflow. The increased outflow has an early and late phase. The early phase of increased outflow starts within few minutes but the late phase takes weeks to months fully to operate.103, 107 Untowards effect of epinephrine consists of : 1. Tachyphylaxis is common as epinephrine. 2. Besides lowering intraocular pressure, epinephrine causes - constriction of conjunctival vessels leading to blanching of conjunctiva. This is short lived, followed by rebound hyperemia. 3. Mydriasis with epinephrine is prompt without cycloplegia. The mydriasis produced by epinephrine, is not counter acted by any drug in therapeutic dose. Hence epinephrine is contra indicated in narrow angled eyes. 4. Lid retraction. Due to stimulation of Mullers muscle, there is always some lid retraction. 5. Prolonged use of epinephrine causes : (a) Cystoid macular edema specially in aphakic and pseudophakic eyes. (b) Allergic blepharo conjunctivitis. (c) Pigmentation of conjunctiva. 6. The systemic side effects consist of : Tachycardia, cardiac arrhythmia and hypertension.

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Epinephrine is not a suitable drug for children. II. Dipiveprin. This is synthetic epinephrine substitute. It is a pro drug i.e. it undergoes bio-transformation before exhibiting pharmacological action104, 105, 106. The compound is hydrolyzed to epinephrine after absorption in the eye. It is more lypophilic than epinephrine making its corneal penetration many times more than epinephrine. Its action is similar to epinephrine. It is used as 0.1% solution two times a day, its mydriasis is more than epimephrine. It does not stain contact lenses, maculopathy is less common. Additions of betablockers have an additive effect. It can be used in children. Its role in buphthalmos is uncertain. Selective alpha agonist. These drugs selectively stimulate alpha two receptors. They reduce intraocular tension in two ways i.e. reducing aqueous formation and increasing uveoscleral outflow. The two commonly available drugs are Apraclonidine and Brimonidine. Apraclonidine is a selective alpha two agonist. It is a derivative of clonidine107, 108, which is no more used in management of glaucoma due to its untoward side effects. Apraclonidine has prompt IOP reducing property when used as one percent solution. This property has been utilized to reduce sudden rise of tension following anterior segment laser procedures for glaucoma. It is used as 0.5% drops two times a day for management of glaucoma. It crosses the blood brain barrier minimally so its systemic side effects are very low and has no cardiopulmonary contra indication. Minor systemic side effects consists of dry nose and dry mouth. Commonest ocular side effect is chronic follicular conjunctivitis. Other ocular side effects consists of unwanted mydriasis, lid retraction and conjunctival blanching. Brimonidine. It is selective alpha two agonist that reduces production of aqueous and increases uveoscleral outflow. It is used as 0.2% drop once or twice a day. It has minimal systemic side effect. Betablockers. Betablockers are a group of drug that antagonise the adrenergic beta receptors. They are effective locally as well as when used orally or parentally. Locally, they are used only as drops. The local drops have systemic side effects as well. In fact betablockers when used for cardiological disorders were found to lower the intraocular pressure as well. The betablockers are most widely used local drops for glaucoma therapy. They have replaced pilocarpine as first drug of choice. Their main function is to reduce aqueous production. The other modes of action are minimal lowering of episcleral pressure and slight increase in uveascleral outflow106, 107. They do not have any effect on pupil size or ciliary tone. According to their cardio pulmonary action, they have been divided into two groups i.e. 1. nonselective that block both B1 as well as B2 adrenergic receptors and 2. cardio-selective that have no or very mild cardiac action. Both types of betablockers have good additive effect when used with miotic. Commonly used non-selective betablockers are : Timolol 0.25%-0.5% Levobunotol 0.5% Cartelol 1%-2% Metiprantol 0.1%-0.3%

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0.5% Betaxolol is the only cardio selective betablockers presently available. Action of betablockers starts within 30 minutes and reach peak effect in 2 hours. The effect last for twelve to twenty hours. Hence they are administered twice a day. Initial fall of I.O.P with B.D. dosage is very good which becomes less in few weeks. This is called short term escape. Reduction in efficacy over months is called long term drift. Betablockers are effective in all types of glaucoma irrespective of width of angle. They have very little local side effects. Some eyes may experience ocular surface disorder. Other minor ocular side effects are burning, diminished corneal sensation and punctate erosion of cornea. The systemic side effects are bradycardia, reduced cardiac output, arrhythmia, heart failure and syncope. It causes bronchospasm, depression, anxiety and confusion. The side effects can be minimised by using smallest possible drop and obstructing the lower puncta by pressing it with thumb for a minute following instillation in the conjunctiva. In children it should always be used in consultation with pediatrician. (d) Prostaglandin analogues. Prostaglandins are locally acting hormones scattered in various forms in various organs of the body. They are potent ocular hypotensive agents in very low dose. For ocular use they are available as latanoprost 0.005% drop in form of buffered isotonic solution with pH 6.7 and as unoprostane. The latanoprost is used once in twenty four hours preferably at night as it reduces the nocturnal variation better than diurnal variation. Latanoprost have synergistic effect with all anti-glaucoma drugs except pilocarpine. Latanoprost and pilocarpine both are known to reactivate uveitis and they are contra indicated in glaucoma secondary to uveitis. Latanoprost is absorbed through cornea. It is an isopropyl pro drug that is hydrolysed by esterase in cornea to biologically active acid latanoprost. Peak concentration in aqueous is reached in two hours. Its mode of action is by increasing uveoscleral outflow by 100% that reduces IOP by 35%. It is hundred times more potent than timolol 0.5%, has hardly any systemic side effects however hypersensitivity to latanoprost may occur. Local side effects consists of reactivation of dormant anterior uveitis, worsening of existing iridocyclitis. Hence it is contra indicated in uveitis induced glaucoma. It also causes cystoid macular edema, less serious complications are darkening, thickening and elongation of lashes, darkening of skin of the lids. E. Prostamides. These are chemical substances present in the eye like prostaglandin and histamine. They are neither hormones nor enzymes. They are members of fatty acidamide family. Prostamides are potent ocular hypotensive agents. They are believed to be involved in the endogenous regulation of IOP via prostamide sensitive receptors in the eye. Commonest form of prostamide used in bimotoprost that is a structural analogue of prostamide. It is used in once a day or twice a day dosage. Carbonic anhydrase inhibitors (local as well as systemic). Oral carbonic anhydrase inhibitors are being used to reduce intraocular tension for last half a century109,111,112. Local use of carbonic anhydrase inhibitors (CAI) is a recent development and has not yet become

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very popular. Advantages of CAI oral or local as ocular hypotensive agent lies in its versatility to lower IOP in all types of glaucomas, in all ages, irrespective of width of angle, pupillary size and duration. Carbonic anhydrase is an enzyme widely distributed throughout the body that is responsible for the catalytic hydration of CO2 and dehydration of H2CO3. In the eye it is mainly concentrated in the ciliary epithelium where it is an integral part of aqueous formation by maintaining pH at optimum level. Carbonic anhydrase inhibitors are a group of drugs that block the enzyme carbonic anhydrase and reduce formation of aqueous. By nature they are sulphonamides. They reduce formation of aqueous by twenty to twenty five percent. Being a sulphonamide they causes systemic acidosis and depletes serum potassium. Acetazolamide is a prototype of all CAI, it is the most widely used systemic ocular hypotensive agent, mostly orally and rarely as intravenous injection to reduce acutely elevated IOP especially during retinal surgery. Action of acetazolamide starts within one to three hours, reaches its peak in four to six hours. The effect passes off in ten to twelve hours. In children it is given in a dose of 10-20 mg/kg/day in divided doses. Acetazolamide does not change the pupillary size, depth of anterior chamber, width of angle of anterior chamber or structure of trabecular meshwork. Though CAI are mild diuretics, hypotensive property is independent of diuresis. As ocular hypotensive agent it can be used with all local and systemic oculo hypotensive agents. Sustained release acetazolamide has a delayed onset and prolonged therapeutic effect with less untowards effects. Acetazolamide is not administered for more than seven to ten days due to its systemic toxicity. However low dosages of acetazolamide i.e. as low as one fourth of usual dose have been found to give good oculohypotensive effect without much toxicity on long term. Other carbonic anhydrase inhibitors are : Diclorphenamide 50mg once or two time a day, Methazolamide 25mg-50 mg pm once or twice a day. Carbonic anhydrase inhibitors have a long list of systemic toxicity. They consist of 1. Metabolic acidosis, 2. Paresthesia, 3. Fatigue, 4. Headache, 5. Nausea, 6. Diarrhoea, 7. Metallic taste, 8. Diuresis, 9. Renal stone formation, 10. Thrombocytopenia, 11. Mild to severe mucocutaneous involvement may lead to Stevens Johnson syndrome. Only ocular side effect is transient myopia. Locally acting CAI. Action of local carbonic anhydrase is same as systemic CAI i.e. blocking the enzyme carbonic anhydrase at ciliary epithelium due to change in pH thus reducing production of aqueous. The drug is absorbed through cornea. Absorption depends upon its lipid and water solubility, contact time and strength. Commonly used local CAI inhibitors are dorzolamide 2% two times a day, brinzolamide 1% and acetazolamide 5% two times a day. Local CAI are less effective in children than in adults. Common side effects are local irritation, conjunctival congestion, metallic taste. Systemic side effects are rare with local drops. Hyperosmotic agents. Hyperosmotic agents are a group of drugs that lower intra ocular pressure following oral or intravenous administration by reducing vitreous volume. These drugs create a fluid gradient between the vitreous and blood which pulls fluid from the vitreous.

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The hyperosmotic agent can either be given : (i) Orally or (ii) Intravenously. (i) The oral hyperosmotic agents commonly used are : Gylcerol (Glycerine). This is a sweet, viscous fluid administered orally in a dosage of 1gm to 1.5 gm/kg, a single dose once or twice a day. It is generally given as 50% solution with lime or orange juice. Glycerol is distributed throughout the extra cellular body fluid. It has poor ocular penetration that helps to create a better ocular gradient to draw fluid from the eye. It is well metabolised and does not cause diuresis. Ocular hypotensive effects starts in less than half an hour, the peak is reached in one hour. The effect lasts for five to six hours. It can be administered along with CAI in reduced dose. All local antiglaucoma drugs are compatible with oral glycerine. Only side effect is nausea. Isosorbide. This less popular oral hypotensive agent is also given as fifty percent solution that contains 1 to 1.5 gm of isosorbide per kg of body weight, about 90% of it is excreted in the urine. Peak ocular hypotensive effect is reached in one to three hours that lasts for four to six hours. Intravenous ocular hypotensive agents : The two drugs commercially available are : Mannitol and urea. Out of the two, urea is no more in use due to high incidence of side effects. Mannitol is administered as fast intravenous drip. The dose is 1 gm to 2 gm/kg or 5-10 ml/kg solution. The rate of drip should not exceed 60 drops/mt. Thus it takes about 30 minutes for 200 ml drug to be administered. Its action starts with in few minutes, peak is reached in half and hour and effect last for six hours. It is generally given only once a day and repeated. Surgery in childhood glaucoma : Surgical procedures in glaucoma in general. It is difficult to classify surgical procedures in glaucoma because of diverse etiology and clinical presentation. A procedure may be suitable for congenital glaucoma i.e. goniotomy that may utterly fail in other type of childhood glaucoma. Surgery for glaucoma after ten years of age and adult glaucoma are same in principle. The procedure in childhood requires adequate modification. The aim of surgery. The aim of surgery is to give stable, near normal intraocular pressure that prevents further field loss and changes in optic nerve head. A well executed surgery generally meets with above criteria but many a times a particular surgery may fail altogether and require either re-operation or addition of medical treatment. Even when a surgical procedure does not fail it may just be a partial success needing other modes of lowering IOP. Surgery may be 1. A primary procedure. 2. May follow when medical methods have failed. 3. May be repeat procedure.

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Following is an outline of various types of glaucoma that requires different procedures : 1. Glaucoma in childhood : (a) Congenital primary glaucoma (b) Congenital glaucoma associated with anomalies of anterior chamber and angle. (c) Glaucoma in older children. (d) Secondary glaucomas. 2. Glaucoma in adults : (a) Wide angle glaucoma (b) Narrow angle glaucoma (i) With pupillary block (ii) Without pupillary block. 3. Absolute glaucoma : The other possible classification is according to anatomical location of the surgery. 1. Surgery on the iris 2. Surgery on the angle of AC. 3. Filtering surgery. 4. Drainage device 5. Cyclodestructive procedures. 1. Surgery on the iris : (a) Surgical procedures consist of : (i) Peripheral basal iridectomy (ii) Sector or broad based iridectomy (iii) Button hole iridectomy (iv) Sphincterotomy (v) Four dot iridotomy (b) Laser procedures on irisLaser iridotomyArgon, NdYAG 2. Surgery on anterior chamber angle : (i) Primary where the surgical procedure involves the trabecular meshwork only : (a) Goniotomy (b) Trabeculotomy (ii) As part of filtering surgery. Thermal sclerotomy, corneoscleral trephine, iridencleisis, cyclodialysis 3. Glaucoma surgeries can either be a non filtering or a filtering procedures. In the latter the aqueous drains either outside the eye in subconjunctival space as in trabeculotomy or may drain in suprachoroidal space as in cyclodialysis. In trabeculactomy a part of trabecular meshwork is removed. In other procedures the aim is not to disturb the trabecular meshwork. 4. Aqueous drainage devices (See page 350.)

380 The non filtering surgeries are :

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1. Paracentesis. This gives a short term lowering of IOP which returns to previous level in few hours to few days generally used in secondary glaucoma. 2. Goniotomy and trabeculotomy. They are meant to give sustained lowered IOP. Various methods employed to lower sudden rise of intraocular pressure : Acute rise of intraocular pressure may occuras part of ocular disease i.e. primary or secondary acute glaucoma that may be iatrogenic as is common in various retinal surgeries. A growth may press the globe from outside. The procedures to reduced acute rise of IOP are : 1. ParacentesisThis is indicated in acute secondary rise of tension. 2. Retrobulbar of anaesthesia 3. Removal of external implants in retinal surgery. 4. Bollus IV mannitol. 5. Massage of 1. Globe 2. Cornea 3. Orbital contents. Massage of globe and cornea force the aqueous out of the anterior chamber either with normal or wide angle. This method is used prior to lens extraction. The closed angle may open up in acute narrow angle glaucoma for a short time following massage of the globe. The mode of action of orbital massage is not well understood. It has effect of bulbar massage as far as intraocular tension is concerned. Otherwise it expels fluid from retrobulbar structures and muscles. This method is more effective following retrobulbar and peribulbar anaesthesia. Relaxation of extraocular muscles, reduce tension over the sclera. This method is also used in cataract surgery, has no role in glaucoma. However gentle massage of the globe through the lid may be used in failed surgery to re-establish the fistula. Non filtering surgery with permanent lowering of IOP : 1. Surgery to relieve pupillary block. 2. Cyclodestructive procedures. 1. Surgery to relieve pupillary block : (a) (i) Peripheral button hole iridectomy. (ii) Peripheral basal iridectomy (iii) Sector iridectomy (b) Iridotomy (i) Laser iridotomy (ii) Four dot iridotomy (c) Sphincterotomy and iridoplasty

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2. Cyclodestructive procedures : (a) Cyclo cryo (b) Laser cyclo ablation (c) Ultrasonic cycloablation (d) Cyclodiathermy Filtering surgeries 1. External filtration procedures 2. Internal filtration procedures 1. External filtration surgeries are : A. Trabeculectomy B. Sclerotomy C. Sclerectomy D. Iridencleisis E. Corneo scleral trephine F. Cyclodialysis G. Glaucoma valves 2. Internal filtering surgeries are : (a) Goniotomy (b) Goniopuncture (c) Trabeculostomy Surgical procedures for glaucoma in children : 1. Goniotomy 2. Goniopuncture 3. Trabeculotomy 4. Combined trabeculotomy and trabeculostomy 5. Thermal sclerotomy 6. Shunt operations 7. Cyclodestructive surgeries 8. Iridotomy Laser in glaucoma 1. General information about ophthalmic laser. 2. Various uses of laser in glaucoma. General information about ophthalmic laser. Laser is an acronym of light amplification by stimulated emission of radiation115, 116. Laser is artificially created bright light with high source of energy that is formed when electric charge is passed through a medium. The medium can be gas, liquid or solid. The media generate photons to emit light energy.110 The light produced by laser is of uniform in frequency and direction.111

382 (a) Usual laser consist of112, 120

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(i) Source of energy. Energy used is generally electric discharge or burst of white light. (ii) The medium that generates light energy by emitting photons. (iii) A chamber (lasing cavity) to reflect energy back and forth by mirrors, one at each end of the chamber. One transparent, only to emit laser and other totally reflecting mirror. It also houses the medium. (iv) Aperture to control laser output. (v) Delivery system. The delivery system can be a slit lamp, an ophthalmoscope, an endoscope, an operating microscope or a surface probe. (b) Properties of laser. The laser is near parallel, coherent, monochromatic linear, unidirectional, light that can be converted into heat when absorbed by tissue. The laser can be delivered either as continuous wave or in pulse.113 Property, colour and mode of action of laser depends upon wave length and pulse duration. Wave length of laser used in ophthalmology vary between 193 nm (Excimer) which is nearer to ultraviolet and 1060 nm (carbon dioxide) which is nearer to infra-red. Ophthalmic laser passes through clear ocular media without being absorbed except excimer. Laser can get absorbed in hazy media i.e. senile sclerosis of lens. Damage to cornea and lens is more with long wave laser like neodymium (1016 nm) and CO2 (1060 nm). Darker tissues absorbs laser better than lighter tissue. Laser is absorbed by melanin in uvea, RPE, xanthophyll of macula and haemoglobin. The density of photons depend upon spot size of laser and power used. Density of photons increases as spot size is reduced. Energy used in laser is expressed in Joules (MJ) or Watts (Mw) depending upon the mode of transmission. A continuous wave laser like argon is expressed in watts while NDYAG which is pulsed laser is expressed in Joules. C. Various types of accessories used with ophthalmic laser are cooling system and various types of condensing lenses that concentrate the laser beam at desired spot i.e. Abrahams lens or a gonioscope to direct beam on the trabecular meshwork. D. Lasers have various modes of applications in ophthalmology. All of them are not used in glaucoma. The common uses of laser are : (i) Photodisruption to do capsulotomy, iridotomy and rupture the vitreous face. (ii) Photocoagulation of vessels and to create a chorioretinal scar in disorders of choroids, retina and vitreous. (iii) Photo vaporising is utilised in vitreoretinal vascular disorders and to seal fibrovascular fronds at the time of vitrectomy in association with photo coagulation. (iv) Photo radiation is used in malignant melanoma of choroids and A.M.R.D. (v) Photo sublimation is used in excimer laser, which acts without heating or coagulation. E. Laser is delivered to cornea, iris and after cataract by directly focusing the target issue. Intraocular delivery is achieved either by transpupillary or transscleral route. Transscleral route is used in diode laser, which is absorbed minimally by the sclera.

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F. Types of lasers used in ophthalmology can be solid state or gas lasers. The former consist of Ruby and Nd-YAG while latter laser consists of argon, carbon dioxide, krypton, helium, neon, excimer etc. (i) Argon Laser. Argon Laser is one of the most commonly used lasers in ophthalmic disorders. It is used to treat disorders of both anterior segment as well as posterior segment. It has no use in corneal diseases. The medium is argon gas that works on an electric discharge. Wave length for blue argon is 488 nm, green argon has wave length of 514 nm. It is a continuous wave laser. The energy is in watts. It is least absorbed by ocular media but well absorbed by melanin, retinal pigment epithelium, haemoglobin and xanthophyll making it very useful for treatment of glaucoma and vascular retinopathy, as it is absorbed by xanthophyll, it should not be used too close to the fovea. In glaucoma it is used to perform argon laser trabeculoplasty in open angle glaucoma and laser iridotomy in angle closure glaucoma. It has no use in congenital glaucoma and most of the childhood glaucoma except to establish a passage between AC and PC in limited cases. It is delivered either by slit lamp or by fibre optics endoscope for intraocular lesions. To be effective in ALT, a suitable gonio lens and for iridotomy an Abrahams lens is used. The Abrahams lens114 consists of a contact lens which looks similar to Goldmann three mirror gonioscope. On the anterior surface, a plano convex lens of +66D is glued. The plus lens is decentred to focus the laser beam on the iris. The function of the lens is to double the diameter of the beam on the cornea and reduces the beam diameter to half on iris. The advantage of this is that it reduces power density at the cornea to one fourth and increases it by a factor four on the iris. The Wise lens has a button of +113D.115 This is not used very widely. The Abraham lens can be used both for argon and NdYAG laser while using argon laser the magnification in the slit lamp should be x40.114 Optics of eye under treatment does not take any part during argon laser treatment. II. NdYAG Laser (Neodymium Yuttrium Aluminium Garnet). It is commonly used laser for capsulotomy in aphakia and pseudophakia. It can also be used on iris. In this the neodymium atoms are embedded in crystal of yuttrium, aluminium, garnet and energised by xenon flash light.112 It works by photodisruption of tissue. Its function is independent of pigment absorption. It works on relatively transparent media like posterior capsular opacification, after cataract and anterior vitreous band. It has a short pulse but a long wave length of 1064 nm. Its power is set in Joules. When a tissue is exposed to NdYAG, the tissue is ionised and plasma is produced. The plasma has mechanical properties of gas and electric properties of metal. Expansion of plasma sends waves inside the eye. Shock produced by these waves mechanically disrupts the tissue. The shock waves can be transmitted to the retina causing retinal detachment. NdYAG laser can be used to do iridotomy, iridoplasty and cyclo-ablation. Advantages of NdYAG. When compared to argon laser, the NdYAG laser has following advantages : Action of NdYAG is independent of absorption by pigment hence it is most widely used laser for capsulotomy.

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Its action on light coloured iris is as good as dark coloured iris. Latter requires more energy than former. Number of burst in NdYAG is almost one tenth of that required for argon laser. Argon laser produces more tissue edema than NdYAG. Iridotomies done by NdYAG are well circumscribed and have less chances of closure. Pupillary distortion is less in NdYAG. The disadvantages of NdYAG are : Hyphaem : is short lived and stops by pressure bandage. The other disadvantage is retinal detachment in predisposed eye. (iii) Diode Laser. This is a semiconductor solid state laser where two light emitting diodes are used to produce wave length between argon and NdYAG (800-820 nm). Its absorption by melanin is better than that by NdYAG. It has better scleral penetration than argon. It does not require separate cooling system. Its small portable size, durability and low cost of maintenance makes it very popular ophthalmic laser. It is used in treatment of choroidal neovascular membrane without damaging nerve fibre layer above it and for cycloablation. It can be used either through the pupil or over the sclera. (iv) Krypton Laser. There are two types of krypton lasers i.e. yellow and red. Krypton laser is poorly absorbed by haemoglobin and xanthophyll. This makes it useful in treatment of lesions near the macula. Good chorioretinal sear makes it useful in treatment of retinopathies associated with haemorrhage. Presence of blood in vitreous and moderate opacities do not hamper its use. (v) Carbon dioxide laser. It has longest wave length among all ophthalmic lasers i.e. 10,600 nm, which is nearer to infra-red and invisible. It is mostly used in occuloplastic surgeries. Its photo evaporation property is used for treatment of cutaneous nevus. (vi) Ruby Laser. This was the first laser used in ophthalmology. Now this is no more used as better lasers are available. (vii) Excimer laser. The word excimer is contraction of two words i.e. excited and dimer (excited dimer). Dimer is a molecule created by the mixture of gas halogen. When these dimers are subjected to electric field, they are transformed to higher discharge. The excimer has shortest wave length among all ophthalmic lasers. Its wave length varies between 193 nm when argon fluoride is used, to 351 nm when krypton fluoride is used. Excimer laser is absorbed by all layers of the eye specially cornea. This property has been utilised to reshape cornea in treatment of refractive errors i.e. P.R.K., laser in situ keratomileusis (lasik) photo therapeutic keratectomy, removal of superficial corneal opacities 116 and lasek (laser sub-epithelial keratomileusis). The most commonly used wave length is 193 nm because it is just sufficient to break up interatomic links without effecting adjacent tissue. Thermal effect is minimal. Excimer has no use in any type of glaucoma. 2. Various uses of laser in glaucoma122, 123, 124. From the previous paragraphs, it is clear that all glaucomas are not suitable for treatment by all laser procedures. There are specific procedures for specific groups of glaucoma. Laser is not a suitable mode of management

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of glaucoma in children in general and congenital primary glaucoma in particular. Overall result of laser treatment for glaucoma under thirty years of age and secondary glaucomas is unsatifactory. However laser iridotomy has definite places in pupillary block glaucoma. Argon, NdYAG and diode lasers are the commonly used lasers for glaucoma. Commonly used laser procedures in glaucoma are122, 123, 124 (a) Iridotomy (b) Trabeculoplasty (c) Gonioplasty (d) Cyclophoto coagulation (e) Panretinal photo coagulation (f ) Suturelysis (g) Bleb failure. (h) Laser scarring to reduce over filtering bleb also. (i) Gonio photo coagulation (j) Goniopuncture (k) Opening failed sclerotomy (l) Cycloablation

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63. Ritch R. ; Pathophysiology of glaucoma in uveitis. Trans Oph. Soc. of the UK, 101, 321324, 1981. 64. Herndon L.W. ; Glaucoma associated with anterior uveitis in Current ocular therapy; fifth edition, pp. 420471. Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000. 65. Kwon Y.H. and Dreyer E.B. ; Inflammatory glaucoma. Int.Oph. Clin. 36 : 81-89, 1996. 66. Deborah Pavan Langstone ; Secondary glaucoma in Manual ocular diagnosis and therapy. Third edition, p. 188, Little Brown, 1991. 67. Sample J.R. ; Corticosteroid induced glaucoma in Current ocular therapy; fifth edition, pp. 464465. Edited by Fraunfelder F.T. and Roy F.H. WB Saunders company, Philadelphia, 2000. 68. Kaye L.D., Kalenak J.W., Frice RL, Cunningham R. ; Ocular implications of long term prednisone therapy in children : J. Pediate Oph. 112:450, 1991. 69. Welensky J.T. ; Hemogenic glaucoma in Principle and practice of ophthalmology. Ist Indian edition, pp. 706708, Edited by Peyman G.A., Sanders D.R. and Goldberg M.F. JayPee Brothers, New Delhi, 1987. 70. Kanski J.J. and McAllister J.A. ; Miscellaneous acquired secondary glaucoma in glaucoma. A colour manual of diagnosis and treatment. First edition, pp.8489, Butterworth, London. 71. Deborah Pavan Langstone : Glaucoma in Manual of ocular diagnosis and therapy, third edition pp. 243244, Little Brown and Co., 1991. 72. Gittinger J.W. ; Traumatic hyphaema in Manual of clinical problems in ophthalmology; first edition, edited by Gittinger J.W. and Asdourian G.K., pp. 68, Little Brown and Co., Boston, 1998. 73. Phelps C.D., Watzke RE - Hemolytic glaucoma. Am.Jr.Oph. 80:690, 1975. 74. Shields M.B. ; Glaucoma associated with intraocular hemorrhage in Text book of glaucoma; Fourth edition, pp. 333336, Williams and Wilkins, Philadelphia, 1999. 75. Jain, M.R. ; Traumatic glaucoma in Text book of glaucoma; first edition, pp. 163 170, JayPee Brothers, New Delhi, 1991. 76. Julia White Side Michael - Ghost cell glaucoma in Current ocular therapy. Fifth edition, pp. 466469. Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000. 77. Morrison J.C. ; Current ocular therapy. Fifth edition, pp. 469470. Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000. 78. Bartholomew R.S. ; Viscoelastic evacuation of traumatic hyphaema. Br.Jr. Oph. 71 : 2728, 1987. 79. Shields M.B. ; Glaucoma associated with ocular trauma in Text book of glaucoma; fourth edition, pp. 339342. Williams and Wilkins, Philadelphia, 1999. 80. Walton D.S. ; Glaucoma in infants and children in Pediatric ophthalmology. Vol. I, second edition, pp. 585598. Edited by Harley RD. WB Saunders company, Philadelphia, 1983.

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81. Jain MR ; Phacogenic glaucoma in Text book of glaucoma present and future. First edition. Jay Pee Brothers, New Delhi, 1991. 82. Epistein D.L. ; Diagnosis and management of lens induced glaucoma. Ophthalmology 89:227-230, 1982. 83. Paterson C.A., Pfister R.R. ; Intraocular pressure changes after alkali burn. Arch. Oph. 91:211, 1974. 84. Yoshizumu M.O., Thomas J.S., Smith T.R. ; Glaucoma induced mechanism in retinoblastoma. Arch. Oph. 96; 105, 1978. 85. Walton D.S. and Grant W.M. ; Retinoblastoma and iris neovascularisation. Am.J.Oph. 65:598, 1968. 86. Caron L. Shield and Shield J.A. ; Glaucoma associated with intraocular tumour. Currentocular Therepy fifth edition p. 476-479 Ed. Fraunfelder F.T., Roy F.H. W.B. Saunders Company, Philadelphia 2000. 87. Hoskins H.D. ; Neovascular glaucoma - Current concepts. Tr. Ac. Oph. oto 78:330, 1974. 88. Brown G.C., Manargal L.E., Schachat A., Shah H. ; Neovascular glaucoma, etiological considerations Ophthalmology 91 :315, 1984. 89. Dutta N.K., Dutta L.C. ; Secondary glaucoma in Modern Ophthalmology. Vol. 1, second edition, pp. 500510, edited by Dutta L.C.; JayPee Brothers, New Delhi, 2000. 90. Rathod M.K. ; Ophthalmological study of epidemic dropsy. BJO 66 :573-575, 1982. 91. Duke Elder S. ; Epidemic dropsy in System of ophthalmology. Vol. II, pp. 684687, Henry Kimpton, London, 1969. 92. Sood N.N., Sachdev M.S., Gupta S.K. ; The eye in epidemic dropsy VIII National symposium on glaucoma, Ahmedabad, Academy of Ophthalmology, p. 79, 1987. 93. Sachdev M.S., Sood, N.N., Verma L. ; Pathogenesis of epidemic dropsy glaucoma Arch Opthalmology 16 : 1221, 1988. 94. Jain M.R. ; Miscellaneous glaucomas in text book of glaucoma present and future, pp. 213214, Jaypee Brothers, New Delhi, 1991. 95. Ahmed E. ; Epidemic glaucoma in a Textbook of Ophthalmology. Ist edition, pp. 270, Oxford University Press, Calcutta, 1993. 96. Hakim S.A.E. ; Argemone oil, sangunarine and epidemic dropsy glaucoma. British Journal of Ophthalmology. 38:193, 1954. 97. Hakim S.A.E. ; Sangunarine and hypo thalamic glaucoma. Journal of All India Ophthalmic Society, 10., pp. 83102, 1962. 98. Feitl M.E. and Krupin T. ; Juvenile glaucoma in Current ocular therapy. Fifth edition, pp. 483485, edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000. 99. Kanski J.J., McAllister J.A. ; Antiglaucoma drugs in A colour manual of diagnosis and treatment. pp. 104-105, Butterworth, London. 100. Duke Elder S. ; parasympathomimetic drugs in System of ophthalmology. Vol. III, pp. 557561, Henry Kimpton, London, 1962.

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101. Ruben M, Watkin R. ; Pilocarpine dispensation for soft hydrophilic contact lens. Br.J.Oph. 59 :455, 1975. 102. Ramer R.M., Gasset A.R. ; Ocular penetration of pilocarpine, the effect of pH on ocular penetration. Ann.Oph. 7:293, 1975. 103. Nagataki D.J., Brubaker R.E. ; Early effect of epinephrine on aqueous formation in normal human eyes. Ophthalmology 88 : 278, 1981. 104. Sears, M.L. ; The mechanism of action of adrenergic drugs in glaucoma. Invest Ophth, 14 :83, 1975. 105. Shields M.B. ; Adrenergic timulators in Text book of glaucoma. Fourth edition, pp. 398408. WB Saunders company, Philadelphia, 1999. 106. Dutta L.C. - Drugs acting on sympathetic system in Ophthalmology principles and practices; pp. 314315, Current Books International, Calcutta, 1995. 107. Boger WP - III short term escape and long term drift. The dissipation effects of the beta adrenergic blocking agents. Suv Oph. 28:235, 1983. 108. Wand M., Schaffer R.N. ; Open angle glaucoma in Current ocular therapy; Fifth edition, pp. 494498, edited by Fraunfelder F.T. and Roy F.H.; WB Saunders company, Philadelphia, 2000. 109. Wilson R.D. ; The medical treatment in glaucoma in Ophthalmology secrets; pp. 136 141. Edited by Vander J.F., Gault J.A. Jay Pee Brothers, New Delhi, 1998. 110. Camras C.B., Aim A., Watson P., Stymschantz J. ; Latanoprost a prostagalndin analogue for glaucoma efficacy and safety. Ophthalmology. 103:1916-1924, 1996. 111. Becker B. ; Decrease in intraocular pressure in man by carbonic anhydrase inhibitor (Diamox) Amj. Jr. Oph. 37:13, 1974. 112. Ramkrishna R. and Puthalath S. ; Pharmacognosy and pharmaco kinetics and ocular hypotensive agents in Modern ophthalmology. Vol. I, second edition, pp. 527538. Jay Pee Brothers, New Delhi, 2000. 113. Hunter D.G. and West E.C. ; Instrument in Last minute optics. First Indian edition, pp. 115117. Jay Pee Brothers, New Delhi, 1997. 114. Fried W. ; Optics and refraction in Principles of Ophthalmology. Vol. 1, first Indian edition, edited by Pyerman G.A., Sander R. and Goldberg M.F. Jay Pee Brothers, New Delhi, 1987. 115. Shields M.B. ; Principles of laser surgery in glaucoma in Text book of glaucoma. Fourth edition, pp. 461465. Williams and Wilkins, Philadelphia, 1999. 116. Brown N.A.P. ; The laser treatment of glaucoma in Scientific foundation in ophthalmology; first edition pp. 306316, edited by Perkins E.S., William. Heineman Medical Books, London, 1977. 117. Shields M.B. ; Surgery of the iris in Textbook of glaucoma. Fourth edition, pp. 490 500. Williams and Wilkins, Philadelphia, 1999. 118. Wise J.B., Munnerlyn CR, Erickson P. ; A high efficiency iridotomy sphincterotomy lens. Am.J. Op. 101, 546, 1986.

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119. Singh D. ; Is refractive surgery justified. Jr. I.M.A. 98:748-751, 2000. 120. Ramalingam S. ; Laser application in glaucoma in Modern Ophthalmology. Vol. I, second edition, pp. 539, edited by Dutta L.C. Jay Pee Brothers, New Delhi, 2000. 121. Sood N.N., Singhotra R. ; Primary open angle glaucoma in Modern ophthalmology. Vol. I, second edition, pp. 463468. Jay Pee Brothers, New Delhi, 2000. 122. Kanski J.A., McAllister J.A. ; Laser in glaucoma. Glaucoma - A colour manual of diagnosis and treatment. First edition pp. 116123, Butterworth, London. 123. Sharma P. ; Laser ophthalmology in Essentials of ophthalmology. First edition, pp. 363365. Modern Publishers, New Delhi, 2000. 124. Boyds : Lasek ; Laser subepithelial keratomileusis. Highlights of ophthalmology, Indian edition 30 : 15-17, 2002.

CHAPTER

11

Disorders of the Retina and the Vitreous in Children


The retina is highly specialised neural tissue of the eye meant for, vision, colour vision, night vision and field of vision. Rest of the eye is constructed round it, to house it, protect it, nourish it and focus on it. It may be considered as an out pouching of the forebrain. It is so similar to forebrain in structure that half of the layers of sensory retina are considered as cerebral layers1. The light rays reaching the sensory retina form an inverted miniature image of the object of regard on the most photo sensitive part i.e. fovea. This light thus received is converted into electric impulse by a complex photo-chemical process and transmitted to the visual central nervous system2 via optic nerve which is a bundle of axons of retinal ganglion cells. The other similarity between retinal cells and cells of brain is that both have nerve fibres arranged (1) Vertically, (2) Horizontally. The vertical fibres connect photo receptors to the visual centres directly. The three neurons connected arePhoto receptors (rods and cones), bipolar cells and ganglion cells. The horizontal fibres provide connections within the retina mediated by amacrine cells and horizontal cells.2 The retina is cellophane like transparent structure with a pink hue. The colour of the retina is due to underlying pigment epithelium, visual purple in rods and retinal capillaries.3 The retina develops from both the layers of optic cup. The outer layer forms the pigment epithelium. The remaining layers develop from the inner layer of the optic cup with a potential space in between, which is liable to be distended in pathological states separating the two leaves of the retina. There are no separate sensory, motor or autonomic nerves in the retina. The retina does not have pain sensation. Applied anatomy of the retina1,4,5,6 The retina is the innermost layer of three coats of the eyeball. It lies between the choroid on the outer side and vitreous on the inner side. It extends from the edge of the optic nerve posteriorly to ora serrata anteriorly. The ora serrata is not a continuous sheet, it has finger like projections. The spaces between these projections are U shaped gaps, which are open towards the ciliary bodies, these are called oral bays. The attachment of the retina at ora serrata is not uniform all round. It extends more anteriorly nasally and less on temporal side.1 All the layers of retina are resolved into one layer of non pigmented epithelium that is continued over the ciliary body. 393

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Thickness of the retina is not uniform through out, it is thickest at the optic disc7 and thinnest at the centre of the fovea. The outer border of the fovea is the thickest area of the retina.7 In the ora, the arterioles and venules look to have same diameter and colour.8 The ora serrata is represented by an imaginary line joining the attachment of the four recti on the scleral surface. In adults it is 6 mm away from the limbus. The equator is 6-8 mm away farther behind the ora. Functionally the retina can be divided into two areas, the central and peripheral. The central area is 5 mm to 6 mm in diameter which has dense cone population and less rod population, hence it is meant for day vision, fine vision and colour vision. In the centre of the central arc is the macula. Number of cones fall fast on the periphery. The peripheral retina is used for coarse vision, night vision and peripheral field of vision. The macula is a relatively large area as compared to optic disc. It is an area 5.5 mm in diameter without any definite anatomical demarcation. It lies lateral to the optic disc between the superior and inferior temporal retinal vessels. Its medial border is very close to the lateral border of the disc. It subtends an angle of 15 on the nodal point, the disc subtends an angle of 4.5. The centre of the macula is 2 disc diameter away from the lateral margin of the disc and 0.8 mm below the horizontal meridian. The macula looks darker than the rest of the retina because it contains xanthophyll, which is lipid soluble carotinoid in nature with yellow colour. The pigment epithelium is more pigmented under the macula than rest of the retina. The macula is packed with cones. About 10 percent of all cones are located in the macula. The ratio between rods to cones in the macula is 1 : 2. The number of rods increases on the periphery and there is proportionate reduction in number of cones on the periphery. The macula is sub divided into four zonesFoveola, fovea, para fovea and peri fovea. The foveola is innermost 0.2 mm area. This is little depressed than rest of the macula. The fovea contains only photo receptors and their nuclei. This makes the retina thinnest at the foveola. The second and third neurones are displaced circumferentially. The foveaThe surrounding area 1.5 mm in diameter is called fovea. The cones in the fovea are more elongated than in other areas. In contrast to foveola which is thinnest part of the retina, the outer border of the fovea is the thickest part of the retina which is 2.5 mm in diameter and called para fovea, the remaining part of the macula constitutes the peri fovea. The central 0.4 mm to 0.5 mm area of the fovea is devoid of retinal capillaries and called foveal avascular zone (FAZ), which is demonstrated on fluorescein angiography. The explanation for absence of capillaries in the foveola is as follows : The retina has dual blood supply. The inner part up to inner nuclear layer gets blood supplies from the retinal capillaries. The remaining outer layers get blood supply from chorio capillaries. As the inner half of retinal layers are absent in the fovea, the capillaries do not exist at this place. In a cross section of the retina, through the fovea, following points are noted9 There are hardly any rods, the cones are covered directly by internal limiting membrane without other layers in between. The cones are tallest at fovea and are densely packed. The cones are arranged vertically all over the retina except the foveola where they are placed obliquely. In the macula each cone is connected to a single bipolar cell, which synapses with single ganglion cell. This arrangement is the cause of higher visual threshold and best colour

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sense at the macula, both of which fall on the periphery. The macula contributes one third of all fibres to the optic nerve. The macular fibres enter the lateral part of the optic nerve in a straight line and immediately assume the central part of the optic nerve. One of the landmark that denotes location of macula is insertion of the inferior oblique on the sclera. Layers of Retina The retina develops from the two walls of the optic cup with a potential space in between. This potential space divides the retinal layers in two distinct structures 1. The outer leaf called retinal pigment epithelium (RPE) that is firmly attached to the Bruchs membrane and is single layered. 2. The inner leaf called sensory retina is multi-layered. Functionally the sensory retinal layers are arranged in three strata10 1. Visual cells (rods and cones) 2. Bipolar cells 3. Ganglion cells Histopathologically the retinal layers are arranged in following ten layers: 1. Retinal pigment epithelium (RPE) 2. Photo receptors 3. External limiting membrane 4. Outer nuclear layer 5. Outer plexiform layer 6. Inner nuclear layer 7. Inner plexiform layer 8. Ganglion cell layer 9. Nerve fibre layer 10. Internal limiting layer Retinal pigment epithelium layer This is the outermost layer of the retina closely attached to the Bruchs membrane. It is continuous with pigment epithelium of the ciliary body as a single layer of cells. The pigment epithelial cells are cuboidal in shape. They rarely undergo division. Hence their number remains almost constant throughout the life. On a flat preparation, the cells have hexagonal outline. There are micro villi on the inner surface of the pigment epithelium that anchor the outer segment of the photo receptors firmly to the pigment epithelium. The pigment epithelium contains melanin granules, phagosomes and lysosomes. The melanin increases the efficiency of the cones by absorbing scattered light. In albinos these granules are colourless. The RPE forms the blood retinal barrier and help in storage and transport of vitamin A11 Layer of photo receptors The nine sensory layers of the retina are separated from the RPE by a potential space. The photo receptors are located next to this potential space and deepest to other eight layers.

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Though this layers is designated as layer of photo receptors i.e. cones and rods. The photo receptors structure wise are not exclusively localised in this layer. This layer consist only of peripheral processes of rods and cones. The layer of photo receptors in fact extends from the RPE to outer plexiform layer beyond which they are joined to bipolar cells, and horizontal cells. In between the pigment epithelium and outer plexiform almost mid way lies the external limiting membrane that divides the peripheral process from the nuclei of rods and cones. General structure of photo receptors Though the rods and cones have different distribution and function structurally, they have many common points: Each photo receptor has an inner segment and an outer segment demarcated by external limiting membrane. Each photo receptor has a cell body, a nucleus, a cell process. The long axis of the receptor is at right angles to the retinal surface. The rods and cones derive their name from the shape of their outer segment. The rods have long cylindrical shape while the cones have short conical shape. The apexes of the outer segment of rods and cones are attached in the micro villi of the pigment epithelium. The outer segments of the photo receptors consist of a set of lamellar disks made up of lipid proteins. The nuclei of the cones are larger and paler than that of rods. The synaptic end of the rod is called rod spherule and that of cone is called cone pedicle due to their difference in shape. The rods are meant for scotopic vision and peripheral field of vision. The cones are meant for photopic vision, central vision and colour vision. External limiting membrane This is not a true membrane. It is a modified layer of plasma of photo receptors and Mullers fibre. It is so stretched that the photo receptors seem to be suspended in it with peripheral part towards RPE. The external limiting membrane ends at ora serrata anteriorly, posteriorly it blends round the edge of the optic nerve. The outer nuclear layer This layer contains nuclei of photo receptors. The nuclei of cones are larger than that of rods and are situated near the external limiting layer. The nuclei of rods are smaller and placed away from the external limiting membrane. The nuclei are joined to the second order neurones. Each cone is joined to only one bipolar cell. This is not true for rods. Each rods spherule is connected to two to three bipolar cells. The outer plexiform layer Outer plexiform layer is a narrow space. It lies between the outer and inner nuclear layers, containing synapses between photo receptors, bipolar cells and horizontal cells. As it lies midway between the internal limiting membrane and RPE, it does not get sufficient blood supply either from choroio capillaries or retinal artery, hence it is more likely to suffer from metabolic disorder. The inner nuclear layer This layer consists of bipolar cells, horizontal cells, amacrine cells and Muller cells. The capillaries of central retinal artery reach up to this level.

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The inner plexiform layer In the inner plexiform layer the axons of bipolar cells, branches of ganglion cells and amacrine cells synapse. It also contains branches of Mullers cells. The ganglion cell layers This layer contains neurologia in which lie the ganglion cells. It is four to five times thicker under the macula as compared to other parts of the retina. There are about 1.2 million ganglion cells each with an axon. All the axons ultimately get together to form the optic nerve. One ganglion cell serves four to six cones and about a hundred rods. The number of ganglion cells get reduced towards the periphery, they are densest at the posterior pole. The nerve fibre layer It is also called stratum opticum. It consists of axons of ganglion cells. The axons form the unmyelinated nerve fibre and the optic nerve. The nerve fibres are arranged parallel to the surface of the retina. The arrangement of the nerve fibres is not similar all over the retina. The fibres originating from the macula go straight to the temporal side of the disc and are collectively called papillomacular bundle. The fibres arising from the nasal retina travel in radial fashion to reach the disc. The nerves arising temporal to macula sweep over the macula to reach the disc in the upper and lower pole of the disc. The peculiar arrangement of nerve fibre layer is responsible for different shapes of field defect. The internal limiting membrane This is a true basement membrane that separates the vitreous from the nerve fibre layer. Blood supply of the retina12 The retina has double blood supply. The inner layer up to outer plexiform layer, (the cerebral layer) get their blood supply from the retinal arteries. Rest of the retina gets its blood supply from the chorio capillaries. The central retinal artery arises from the first part of the ophthalmic artery near the apex of the orbit. Its orbital part runs anteriorly under the dural sheath of the optic nerve as far as a point 10 mm behind the globe, then it takes an upward course, piercing the dural sheath, arachnoid covering of the optic nerve almost vertically to reach the subarachnoid space, this constitutes its inter vaginal part. It then passes through the substance of the optic nerve as inter neural part. After reaching the middle of the optic nerve its course is forward, as central retinal artery proper to supply the inner layers of the retina and the optic nerve head. Before terminating as artery of the retina, it give following branches - The pial branches, the recurrent retinal branch, the inter neural branches. The central retinal artery divides into two i.e. the superior and inferior branches, each again dividing into temporal and nasal branches. These four branches are arranged in such a way that there is one branch for each quadrant of the optic nerve and corresponding retinal quadrant except the foveal a vascular zone. The rods and cones that are metabolically most important get their blood supply exclusively from the chorio capillaries. The retinal vessels lie superficially in the nerve fibre layer under the internal limiting membrane. Generally there is no anastomosis between the retinal circulation and the chorio capillaries.

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The retinal arteries divided and re-divide to be reduced to arterioles which are end arteries, ultimately to form capillary network. There are two such networks. The first is at the level of nerve fibre layer and the second deeper at the level of outer plexiform layer and inner nuclear layer. There are connecting channels joining the two layers of capillaries. All the blood vessels are controlled by autonomic nerve system except the retinal system.15 The blood retinal barrier13,14 The permeability of retinal blood vessels differ from vessels of other organs1. There is a selective exchange of fluid with protein molecule from retinal capillaries to retinal cells. 2. The retinal capillaries consist of a single layer of non fenestrated endothelium cells that does not allow passage of fluorescein in normal vessels. 3. The retinal blood vessels are without internal elastic lamina and a continuous layer of smooth muscles. 4. A basal lamina covers the outer surface of the vessels. The blood retinal barrier is a physiological barrier meant to optimise fluid passage into the retinal cells. Only a few metabolic products can pass this barrier from outside. The barrier is two layered. Tight junction between the pigment epithelium cells forms the outer barrier. The inner is due to tight junction of endothelial cells in retinal capillaries. Retinal veins The retinal veins have the same name as retinal arteries. The retinal veins start on the periphery and end in the central retinal vein that leaves the optic nerve 12 mm behind the globe, in more angular fashion than the central retinal artery. Thus the central retinal vein has a longer course in the inter vaginal space than retinal artery. This makes it more exposed to pressure changes in the inter vaginal space. The retinal veins have larger diameter than the arteries in a ratio of 3:2. The arteries and veins share a common sheath, generally the artery crosses over the vein. The veins drain the retina in quadrants. The central retinal vein generally drains directly into cavernous sinus. It also partly drain in superior ophthalmic vein, rarely it drains in inferior ophthalmic vein. Development of retina16 The retina except the blood vessels is neuro ectodermal in origin. Its development becomes obvious from very early stage before even the closure of the neural tube i.e. 2 mm stage and continues to develop (macula) after birth. It develops from the optic cup. The two layers of the optic cup are involved in development of the retina. The outer layer gives rise to a single layer of retinal pigment epithelium (RPE). The remaining layers that form the sensory retina develop from the inner layer of the optic cup. A potential space is left in between the two layers throughout the life and when distended causes separation of sensory retina from RPE. Development of retinal pigment epithelium The cells of the external walls of the optic cup become pigmented. Its posterior part actually form the pigment epithelium of the retina. The anterior part continues forward to form the anterior pigment layer of iris and ciliary body16.

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Development of sensory (pars optica) retina To begin with, the inner layer of the optic cup is a single layer of epithelium. It divides further to give rise to nine layers of retina which can be grouped broadly in following three groups: 1. The cells that give rise to rods and cones; 2. The cells that give rise to bipolar cells, ganglion cells and supporting tissue. 3. Cells that form ganglion cells axons and nerve fibre layer. Stages of development of retina16,16A Stage IDevelopment of outer nuclear zone and non nucleated marginal zone. Stage IIDifferentiation into inner non nucleated zone and inward migration of the outer nuclear zone. Stage IIIDevelopment of inner neuroblast and outer neuroblastThe ganglion cells, Mullers fibres and amacrine cells develop from the inner neuroblast. The outer neuroblast gives rise to horizontal cells, nuclei of rods and cones. From the Mullers fibres develop the two limiting membranes, from the ganglion cells develop the nerve fibre layer. Stage IVThis is the final stage of organisation of all nine layers. Development of macula Development of macula differs from development of rest of the retina. All the layers of adult retina are well formed by fifth month of intra uterine life. Vascularisation of inner retinal layers is complete by eighth month. The macula starts developing very early stage of embryogenesis and progresses fast only to be slowed down and then continues to grow several months post natal. The differentiation of macula is fast up to first three months after which there is sluggish differentiation of central area. The rest of the retina continues to be differentiated and grow. This retardation continues up to eighth month. Then the differentiation continues like rest of the retina. At six months of life the macular area is thicker than rest of the retina. By seventh to eighth month the macula starts thinning due to shift of ganglion cells towards the periphery. At birth the ganglion cells at fovea are reduced to single layer. Congenital anomalies of retina Congenital anomalies are numerous and present variable clinical features. They may be symptomless or may cause profound visual loss. They may be obvious at birth, may start at or before birth but manifest late. They can be uniocular or bilateral. They may be present in retina only or may be associated with congenital anomalies of choroid or optic nerve. Anomalies of retinal vessels are very common. As development of vitreous is clearly associated with that of retina, congenital vitreo retinal lesions are common. The congenital anomalies of retina and vitreous have frequent hereditary background, however, sporadic cases are also known. It is difficult to classify congenital anomalies of retina and vitreous due to their clinical diversity. They can be classified in following groups: 1. Ophthalmoscopic appearance. They include coloboma, opaque nerve fibre, Bergmeister papillae, macular anomalies, anomalies of blood vessels, anomalies of pigmentation, albinism, retinal detachment, retinal folds, hamartomas.

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2. Physiological (visual). Diminished night vision, diminished day vision, abnormal colour vision. 3. Anatomical. This is most probably best suited classification. The anatomical classification may be : 1. Congenital anomalies of the retina (a) Anomalies of structures arising from outer layer of the optic cup. (b) Anomalies of the structures arising from the inner layer of the retina (i) Anomalies of the retinal periphery (ii) Anomalies of the macula 2. Congenital anomalies of retinal vessels 3. Congenital anomalies of optic nerve 4. Congenital anomalies of vitreous 5. Combined vitreo retinal anomalies Anomalies of structures arising from outer layer of the retina The only retinal layer that arises from the outer wall of the optic cup is retinal pigment epithelium. The congenital anomalies of this layer are fewer than those of sensory retina. The anomalies mostly lead to pigmentary disturbance. Commonest anomaly is embryopathic pigmentary retinopathy and localised congenital pigmentation of the retina. Congenital embryopathic pigmentary retinopathy This is secondary to intrauterine infection by syphilis, rubella and influenza in the first trimester. The condition is bilateral without any symptoms. The retina is studded with bluish black pigmented dots. The appearance is called salt pepper fundus. The appearance lasts life long. The condition is confused with retinitis pigmentosa. There are no vascular changes. There may be associated optic atrophy. No treatment is required. Other major anomaly of pigmentation is albinism. Albinism18 Albinism is a common congenital disorder of metabolism of amino acid tyrosine. The condition has world-wide distribution, the incidence is as high as 1:20,000. However it is reported to be more among coloured races. This may be due to the fact that change in colour of hair, skin and eyes are more visible in dark person than in blonds. Both the sexes are affected equally. Women are the carriers in x linked ocular albinism where they show some features of albinism. Albinism can be either an autosomal recessive or an autosomal dominant trait. It has been observed that sometimes when both the parents have some traits of albinism, they have normal children, this happens when one of the parents is tyrosinase negative and the other is tyrosinase positive. (See page 236) Clinically albinism can belong to any of the following: 1. Cutaneous albinism 2. Oculo cutaneous albinism

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3. Ocular albinism 4. Albinism associated with systemic syndromes. Whatever may be the clinical presentation, the basic pathology is failure to form melanin, the pigment that is responsible for colour of the skin and hair19. The melanin is formed in a special type of cells called melanocytes which are derived from neural crest. The formation of melanin involves a chemical reaction involving amino acid, tyrosine and enzyme tyrosinase. The melanocytes form melanosomes, which are packed with granules that contain the pigment melanin. The melanin is formed from tyrosine that is converted to tyrosinase, which in turn is converted to dihydro phenylalaline and to quinone. Cutaneous albinism The skin is hypo-pigmented in patches. The patches may be congenital or may develop later. The patches may involve the skin of the lids, lashes or eyebrow without any ocular defect. Oculo cutaneous albinism This is the commonest type of albinism met with. Clinically there are two types20 of oculo cutaneous albinism: 1. Tyrosinase positive oculo cutaneous albinism 2. Tyrosinase negative oculo cutaneous albinism The difference is made out by hair bulb incubation test. The test consists of incubation of plucked hair bulb in tyrosine solution. In case of tyrosinase positive albinism, the hair bulb becomes dark. Tyrosinase negative shows more clinical features than positive group. The ocular features of oculo cutaneous albinism consist of The skin of the lids is white so are the eyebrows and lashes. The conjunctiva looks hyperaemic. The cornea and AC are normal. The iris is light coloured, devoid of any pigment and transmits reflected ray. The pupil is of normal size and reaction, the colour of the pupil is pink in contrast to a black pupil of a normal child. The eyes may have squint due to errors of refraction. Horizontal nystagmus is almost constant feature. Vision is poor. Myopia is more common than hypermetropia. Myopia is frequently associated with high astigmatism. Near vision is normal but the child may not be able to read due to nystagmus. To counteract the nystagmus and use the null zone to maximum benefit, the child develops a head tilt, may have head nodding. Colour vision is fairly good. There is no night blindness. The child is more comfortable in dim light. In bright light the child has photophobia and glare. On fundus examination the retinal vessels and choroidal vessels stand out prominently against the white sclera. The retinal pigment epithelium has granules similar to melanin but no pigments. The macula is hypo plastic. The optic nerve head can not be differentiated, its position is denoted by the convergence of the retinal arteries. All types of oculo cutaneous albinism show some type of abnormal crossing of nerve fibres at the chiasma.20 Less common ocular findings areParesis of extra ocular muscles, corneal opacity, partial aniridia, persistent pupillary membrane, developmental cataract, hypo plasia of retina.

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The common systemic findings consist of pale white skin, which is sensitive to the sun light. The body hair is as white as lashes and eyebrows. Less common extra ocular features areDeafness, mental retardation, and coagulation defect. These features are seen in children associated with syndromes involving multiple systems. Syndromes associated with oculo cutaneous albinism Chediak-Higashi syndrome This is a serious, often fatal condition with multi systemic involvement. Chief among them is chronic lymphadenopathy, lymphomas, mental retardation, hepatospleenomegaly besides oculo cutaneous albinism. The condition is autosomal recessive. The causes of death is recurrent infection and lymphoid infiltration. Ocular findings Usual findings of oculo cutaneous albinism are i.e. Bilateral corneal opacity, developmental cataract, there may be disc edema due to lymphoid infiltration. Waardenburgs syndrome There is partial albinism, localised in some places. The systemic manifestations are more than ocular feature. The systemic findings include - Hearing defect, vestibular dysfunction, epilepsy, and a white forelock. The ocular findings are: Fused eyebrows which may be white, poliosis of lashes, long lashes, wide inter canthal distance, lateral displacement of puncta, frequent chronic dacryocystitis, pseudo or real squint, blue iris, heterochromia of iris. Cuteneous albinismThe skin shows patches of hypo-pigmentation with or without involvement of skin of the lid. It should be differentiated from other causes of vitiligo and poliosis. Ocular albinismThe pigmentary deficiency is exclusively seen in the eyes. Externally the eye looks normal because the iris has normal pigmentation. The pupil is not as pink as in oculo cutaneous albinism but the hypo-pigmented retina may give a pale reflex. Nystagmus is common. The retina is devoid of pigmentation that may be generalised or localised. Women carriers may have less findings in the fundus. Management There is no specific treatment for any type of albinism. The treatment is directed to correct error of refraction, give dark glasses, correct amblyopia as far as possible. Instruct the child to use full sleeves and full pants, use broad brimmed hat to protect the skin from sunrays. Some children may require rehabilitation or low vision aids. Anomalies of structures arising from inner layer of optic cup can be21 1. Dysplasia and a plasia of retina 2. Retinal folds 3. Retinal cysts

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4. Retinal detachment 5. Retinos chisis 6. Retinal dystrophy 7. Retinal degeneration Retinal dysplasia Retinal dysplasia represents a group of congenital conditions that are due to faculty differentiation of retinal premordium. It can be of two types: 1. Congenital rosette formation and 2. Congenital gliosis. Congenital rosette formation can be seen in microphthalmic eye or otherwise normal eye. The rosettes are formed when development of neural element overshoots its normal limits. The number of rosettes in an eye may vary from single to multiple. They may be associated with folds in non pigmented epithelium of the ciliary body. The rosettes may be seen anywhere on the retina as masses that may be embedded in the retina, may protrude in the vitreous. On histopathology the appearance is similar to retinoblastoma with central lumen. In fact sometimes they are mistaken as retinoblastoma. Retinal gliosis There is proliferation of glial tissue in excess of normal limit. The proliferated glial tissue may be mistaken as intraocular tumour. Retinal folds They are caused due to proliferation of inner layer of optic vesicle. Sometimes persistent primary hyper plastic vitreous may cause a traction fold where features of persistent primary hyper plastic vitreous may overshadow the whole picture. Sometimes the folds may span between the optic disc and ciliary body. The common site being inferior temporal retina. Large congenital retinal folds may give a white reflex in pupillary area. Retinal cysts Sometimes retinal dysplasia or microphthalmia may be associated with retinal cyst. Peripheral retinal cysts may be asymptomatic and discovered only on routine indirect ophthalmoscopy. They are presumed to be precursors of retinal dialysis or retinoschisis. Congenital retinal detachment Congenital retinal detachment takes place when the two layers of the optic cup fail to come in intimate proximity due to unequal rate of growth of the two layers. It is generally seen on the periphery. It may be caused by intrauterine inflammation, is common in microphthalmic eyes. Congenital retinoschisis Retinos chisis is a condition where there is a split in the sensory retina, in contrast to a split between sensory retina and retinal pigment epithelium in retinal detachment which is far more common than retinoschisis. In retinoschisis the split is at the level of outer plexiform layer. Congenital retinoschisis is a familial condition. The inheritance is variable. It effects both boys and girls. It is present at birth but diagnosed late either during routine examination or the child presents with diminished vision or squint. A late stage of congenital retinoschisis is termed as juvenile or idiopathic retinoschisis.

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The condition is generally bilateral and symmetric. The inner layer is raised, dome like area of elevated sensory retina. It may have a hole in the inner table. However presence of a full thickness hole will cause rhegmatogenous retinal detachment. Congenital retinal degeneration and dystrophies Almost all degeneration/dystrophies are congenital in nature, may be present at birth but diagnosed later. They are bilateral and almost symmetrical most of the times. Commonest symptoms being diminished night vision with constricted peripheral field. For details (See page 434.) Congenital functional defects of vision due to anomalies of retina are: 1. Congenital night blindness 2. Congenital day blindness 3. Congenital colour blindness Congenital night blindness This is a rare condition that runs in families with variable inheritance. The striking features are: 1. Absence of signs of hypovitaminosis A, which is otherwise commonest cause of diminished night vision in children in developing countries. 2. Normal ophthalmoscopic appearance The central vision remains normal in contrast to retinitis pigmentosa which is commonest cause of non reversible night blindness in older children where central vision may be effected. Diminished vision in congenital night blindness is attributed to associated myopia. The colour vision is normal. Night blindness is stationary and permanent. The fields are normal, may have subnormal ERG and EOG. Differential diagnosis consists of xerophthalmia in developing countries that responds to administration of vitamin A and other causes of diminished night blindness i.e. retinitis pigmentosa and its variations. Congenital day blindness This is even rarer than congenital night blindness. There is better vision in dim light. The colour vision is also defective. The eyes are generally amblyopic. There may be some mental retardation. The cases are generally passed as amblyopia. The condition is hereditary. Congenital colour blindness Except for a few acquired conditions, all colour defects are congenital in origin with strong family history and variable inheritance. Congenital anomalies of retinal vessels Congenital anomalies of retinal vessels are common. They may be in the form ofDivision of arteries, their course, tortuosity, capillary malformation, cilio retinal vessel and telangiectasia. Generally the central retinal artery bifurcates into superior and inferior branches at the optic nerve head. As a congenital anomaly the bifurcation can be in the orbit. The superior and inferior division may trifurcate instead of bifurcation. Abnormal tortuosity is more frequent.

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It may be primary anomaly without any clinical significance or may be part of some other vascular malformation. Telangiectasia is seen in Wyburn-Mason syndrome, Sturge Weber syndrome, Coats disease. Angioma formation is characteristics of von Hippel-Lindau disease. Cilio retinal artery It is seen in about 15% to 20% eyes as a small twig arising from the temporal border of the optic disc and supplying the retina between the macula and the disc though it is called cilio retinal artery and supply the retina, it is in fact part of ciliary circulation. It arises from the ophthalmic artery. It is occasionally seen in both eyes. It is more common in hypermetropic eyes22. The clinical significance of this anomaly is that the macular blood supply is partly sustained in case of central retinal artery block. Congenital anomaly of retinal vessels of late onset There are few conditions of congenital and hereditary origin that manifest late with profound loss of vision. The main pathology in such cases is not presence of congenital anomaly itself but associated endothelial defect that cause escape of exudate, blood or both from the incompetent endothelial lining of abnormal vessels24 causing superficial and deep exudates and haemorrhage. Formation of vitreous bands and circinate retinopathy is common. In all cases with past evidence of exudation, the retinal periphery should be examined with indirect ophthalmoscope and fluorescein angiography. Most of the cases are bilateral and simultaneous but not always symmetric. One eye may be involved more than the other. The common condition seen in pediatric age group are: 1. Retinal telangiectasis (Coats disease) 2. Retinal angiomatosis (von Hippel-Lindau disease) 3. Retinal cavernous hemanioma disease (see chapter on retinoblastoma) 4. Arterio venous aneurysm (Racemose aneurysm). Coats disease (Primary/Congenital retinal telangiectasis)25,26 The condition is also known as Lebers miliary aneurysm. (see chapter on retinoblastoma). It is an exudative retinal vasculopathy that can be seen on any part of the retina but peripheral lesions are more. The temporal retina is most commonly involved. Both the arteries and veins are involved. The condition is non hereditary, boys are affected more than girls in a ratio of 80 : 20. In 80% of cases the condition is unilateral. However the other eye should always be examined for evidence of peripheral lesion because such lesions may be managed better than the original eye, which may be in advanced stage of disease and difficult to treat. Though most of the children with Coats disease seek help only after 8 to 10 years of age. The condition has been divided into two forms - (1) Juvenile, (2) Adult. The adult cases are in fact the juvenile cases that have been asymptomatic. Juvenile form has been detected even before one year of age. As far as progression is concerned earlier the lesion develops faster is the growth and poorer is the prognosis. Some peripheral lesion may become stationary. Coats divided the condition in three groups: 1. No vascular changes, only massive retinal or sub retinal exudation, no haemorrhage.

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2. Multiple vascular changes, retinal and sub retinal haemorrhage, massive retinal and sub retinal exudates 3. Prominent retinal vascular changes, in the form of arterio venous communication and late exudation. This stage was later considered as separate entity not related to Coats disease and named as angiomatosis retinae. To these three groups a separate clinical feature of miliary aneurysm was added and the condition was also known as Lebers miliary aneurysm. However the condition was proved to be a milder and early form of Coats disease.27 The exact cause of the disease is not known, most probably there is incompetence of retinal vessels. Clinical features28,29 Clinical features depend upon stage of the disease at which the child is brought. 1. It may be quiescent (a) Discovered on routine fundus examination (b) The child is brought with unilateral squint, diminished vision. In the above stages the eye is non-congested without any signs in the anterior segment. 2. Stage of white reflexThis is commonest stage when a child either is brought by parents or referred by pediatrician who is justified to think it as retinoblastoma. 3. Stage of complication (a) Congested eye (b) Raised tension (c) Complicated cataract (d) Retinal detachment 4. Stage of blindness due to above conditions and total retinal detachment. Signs in a well established case: 1. There may be a retro lental white reflex in the pupil on oblique illumination. 2. On retinoscopy the reflex is grey instead of usual pink. 3. Fundus examinationInitially the media are clear. The changes are in the (a) Vessels (b) Retina Retinal vessels The changes are seen both in the artery as well as veins. The changes are - Bunches of beading, kinking, and loop formation. The calibre of the vessels are irregular, the vessels are tortuous, may show aneurysm formation. There may be establishment of communication between the artery and the veins. Neovascularisation may also take place. Changes in the retina In type I of original classification of Coats, the changes are predominantly in the retina or sub retinal space with almost normal vessels that become involved later leading the eye to the category of type II.

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The retinal changes consist of ophthalmoscopically visible areas of greenish white or yellowish white areas of exudates with shinning spots. The shinning spots are cholesterol crystals, which are also seen in sub-retinal space. The exudation in the sub retinal space may elevate the retina and form a mass. The retinal detachment is exudative in nature. If not treated the retinal detachment extends leading to total detachment and the retina may be pushed as far as the posterior pole of the lens or very near to it. By this time a white reflex is visible that must be differentiated from retinoblastoma. There may be some haemorrhages in the vitreous as the disease progresses, the eye goes into secondary glaucoma, uveitis, complicated cataract and blindness. It may become painful and require enucleation. Investigation Most important investigation in presence of clear media is fluorescein angiography. The findings are variable according to stage of the disease and type of the disease. Common fluorescein angiographic findings in established cases of Coats disease are: 1. Vascular changes in the form of tortuosity, beading, loop formation, neovascularisation, multiple-aneurysms which were not visible with ophthalmoscope but stand out prominently on FFA. 2. Retinal changes consist of leak, area of non perfusion and capillary drop out. One of the characteristic appearance of fluorescein angiography is light bulb appearance of dilated aneurysm. The next important investigation especially in hazy media is CT of the eye, orbit and skull and ultrasonography of the globe. CT not only delineates the position of the retina, it shows the sub-retinal mass, there is no intraocular calcification which is common in retinoblastoma. CT of the brain excludes possibility of intracranial angiomatosis which is common in von Hippel Lindaus disease, a condition that may otherwise be confused with Coats disease. Differential diagnosis The conditions that come in differential diagnosis are long. It is headed by retinoblastoma which is not only sight threatening but the life of the child is also jeopardised. The main points in favour of Coats disease are - Unilaterality, more common in boys; late age of onset, slow progressive. It never invades choroid or sclera hence it never becomes extraocular, being benign, no metastasis is seen on x-ray and CT. Intraocular calcification is absent in Coats disease. The most important similarity between the two conditions is white reflex in pupillary area. Other conditions that should be excluded are: Persistent hyper plastic primary vitreous Congenital cataract Traumatic cataract Intra ocular toxocariasis Retinal angioma Familial exudative vitreo retinopathy Total retinal detachment

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There is neither prophylaxis nor specific treatment for the condition. The aim is to clinch the correct diagnosis. The treatment is directed towards vascular changes mostly on the peripheral telengectasis. This is achieved by 1. Photo coagulationThis is a preferred treatment. The leaking vessels are treated directly by laser. This may initially increase exudation, which can be reduced by using lesser energy and smaller spots. The preferred laser is argon laser. It takes about six weeks for any improvement to be visible and takes months or years for the exudation to clear. 2. Cryo therapyThe cryo is applied through trans conjunctival route on the periphery and reaction monitored under indirect ophthalmoscope. 3. If retina is elevated, a scleral buckle may be required. Recurrence after adequate treatment are known. Retinal angiomatosis (von Hippel Lindau disease) The angioma formed in this condition is a hamartoma, which comes under broad classification of phacomatosis. The lesion develops from retinal capillaries and endothelium of retinal vessels. In contrast to Coats disease, which is non inherited, this condition is inherited as autosomal dominant trait. It again differs from Coats disease due to multi systemic involvement that can be intracranial or visceral. In fifty percent of cases it is bilateral which is less frequent in Coats disease. It can be seen on optic nerve head as well. It may be present in the members of the same family without any symptoms. The condition is most commonly detected in teens or early twenties. Both the sexes are equally affected. The lesion starts on the periphery either as single or multiple lesions, all lesions need not appear simultaneously. The first change occurs in the capillary bed as micro aneurysm that gradually enlarges to become a small nodule. The final growth is a spherical orange nodule, may be larger than the disc. The nodule has a feeding artery and a draining vein. The calibre and the colour of both the feeding and draining vessels become identical with passage of time.30 The angioma on the optic nerve is generally not associated with abnormal vessels. The systemic involvement is seen in about 25% of cases. The systemic involvement becomes symptomatic later than ocular. It is not always possible to predict which child with angiomatosis retinae will have systemic involvement. Hence all cases should be subjected to neurological and systemic examination including USG for visceral lesions, CT and MRI for brain lesion. The parts of the brain involved areCerebellum, medulla and pons. The spinal cord may also be involved. Commonest cerebellar growth is hemangio blastoma. The visceral involvement consists of cyst in liver, lung, kidney, pancreas, cpididymis and ovaries may also be involved. The skin is never involved, which is common in other vascular hamartomas. Rare systemic involvement consists of polycythemia, hypernephroma and pheo chromocytoma.

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The clinical pictures are variable. The retinal lesion may be dormant for years and become symptomatic when there is exudation under the macular which may form a macular star. In absence of macular involvement, the other causes of diminished vision are retinal detachment, retinal and vitreous haemorrhage, secondary glaucoma and complicated cataract. Management Treatment of choice is Laser photo coagulation of lesions smaller than optic nerve. Xenon arc photo-coagulation gives better result in larger angiomas. Small lesion are treated in single sitting, larger growth require multiple sittings. The aim should be to destroy the growth directly and not the feeding or draining vessels. Peripheral lesions are difficult to treat with laser. They are best treated by cryo coagulation. Retinal cavernous haemangiomas These are rarer than angiomatosis retinae. They are also hamartomas, may be seen in the retina or optic nerve head. The condition is also included in group of phacomas. Skin involvement is frequent. The condition is asymptomatic, does not require any treatment. However the patient should have regular follow up. Arterio venous aneurysm The condition is known by many other names but none of them are appropriate because the lesions are neither aneurysm, talengiectasis or angioma, though sometimes they may resemble one of them. It is a congenital malformation of the main retinal vessels, which have end to end anastomosis without capillary bed in between. Generally there are no systemic involvement. Rarely brain especially the mid-brain may have similar changes with corresponding neuro vascular presentation. Involvement of brain is called Wyburn-Mason syndrome. Occasionally facial bones may be involved. Common age when the diagnosis is made is generally in the third decade. However it is not uncommon to see the condition in first decade. It is equally seen in both sexes. On examination the patient is generally asymptomatic but on a long run due to retinal exudation there is diminished vision. The anterior segment is normal. The fundus picture is characteristic, best seen by indirect ophthalmoscope. The veins and arteries are of same colour and calibre. It is difficult to find out the line of demarcation between the two. There may be some retinal exudation and haemorrhages. Fluorescein angiogram differentiates it from other angiomas. Initially the sensory retina is not involved. In well established cases micro-aneurysms and capillary non perfusion may be seen. Generally there is no leak. A few cases may regress without any treatment. There is no specific treatment. Rarely photo coagulation with laser may be required in selected cases. Congenital anomalies of the macula The development of the macula differs from rest of the retina. It starts differentiating early during embryogenesis, there is a period or retardation followed by growth that lasts in post natal period. The macula gets its blood supply mostly from the underlying chorio capillaries. It is almost exclusively packed with cones hence is the seat of central vision and colour vision. Congenital anomalies may start in early days of gestation or the anomaly may start late and be discovered late.

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Commonest congenital anomaly of macula are macular colobomas. They are frequent but not as common as coloboma of the uvea. They can be unilateral or bilateral and single, or multiple, generally asymmetric coloboma is generally elliptical, are placed horizontally over the macula and extending well beyond the macula. Its size may vary between one disc diameter to ten disc diameter. The edges are generally irregular with uveal pigments. As per appearance of the coloboma it has been classified into following groups (a) With normal retinal vessels (b) With abnormal retinal vessels The second possible classification is (a) Coloboma with pigment (b) Without pigment Both the two groups may overlap. The pigmented coloboma has heaped pigment. The retinal vessels course over the mound of pigment. The chorio capillaries under the macula are absent. Non pigmented colobomaThese are more common. The area is white due to underlying sclera that may be ectatic, there are no vessels seen in the coloboma. On the periphery, which is irregular, clumps of pigmentation are seen. Coloboma with abnormal vesselsThis type of coloboma is rarest. There may be anastomosis between the choroidal and retinal circulation or a vessel may pass from the coloboma towards the vitreous. The macular coloboma has a strong hereditary predisposition. The coloboma may be associated with anomalies of the globe, optic disc or choroid. The exact etiology is not understood. It is said to be a variation of an atypical coloboma of the uvea. This theory is supported by absence of chorio capillaries and presence of retinal vessels in the coloboma. This further gets credence due to extension of vessels from the coloboma into the vitreous. However possibility of maternal toxoplasmosis should always be kept in mind. Common clinical presentations areDiminished central vision, nystagmus, myopia, convergent squint. Hypoplasia of the maculaSome authors consider it as variation of coloboma23. It is generally associated with albinism but can be a primary defect. The exact mechanism is not known. These children have poor vision with absent foveal reflex. Other congenital anomalies of macula consist of degeneration of macula. Retinal diseases in children Retinal diseases in children can be 1. Congenital 2. Acquired Out of all the acquired conditions, the most common group of diseases in children are the retinal dystrophies and degenerations. The sequelae of which spill over adulthood and may terminate in legal blindness in adulthood. In contrast to this vascular retinopathies

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which are a major cause of blindness in adults are less frequent in childhood. Some of them may be seen in childhood in milder form. One of the unique condition that is seen in children is retinoblastoma, a life threatening malignant tumour (It has been discussed in a separate chapter). Rhegmatogenous detachment is also less common in childhood but when present, is a potential cause of blindness if not treated. Trauma and myopia are two common causes of retinal detachment in children. The acquired causes can be 1. Inflammation 2. Trauma 3. Degeneration 4. Retinopathies 5. Tumours 6. Retinal detachment. Symptoms of retinal diseases in the children The symptoms hardly differ from those in adults. The difficulty arises when the child can not express it or fails to realise its importance. This is more so because all retinal diseases per se are painless. The symptoms 1. Vision. Commonest symptom is diminished vision. Degree of diminished vision depends on extent of the macular involvement. A large lesion on the periphery may not produce any dimness of vision, a small lesion on the macula produces extensive loss of vision. Macular lesions are more likely to produce sudden loss of vision except in degenerations and dystrophies. 2. Diminished night vision is foremost cause of retinal dystrophies which is gradual, progressive and associated with poor dark adaptation. 3. Loss of peripheral field again is a symptom of retinal dystrophies. 4. Metamorphopsia is common in retinal detachment and acute macular lesions. 5. Photopsia or flashes of light generally indicate retinal irritation or beginning of retinal or vitreous detachment. 6. Black spots in front of the eyesSudden development of black spots, increase in number and size of black spots in front of eyes require elaborate examination because they may herald retinal detachment. Slowly developing black spots are not harmful. 7. Colour defect may be present in macular degeneration. Signs of retinal diseases The signs of retinal diseases are generally not visible with oblique illumination or routine slit lamp bimicroscopy because the retina is beyond the focus of ordinary slit lamp. The optics of the eye/slit lamp needs to be modified to bring the retina in focus of the slit lamp. Rarely the condition is visible by oblique illumination if it is large enough or near enough the lens as white reflex in the pupillary area.

412 The retina is best examined by 1. Direct ophthalmoscopy 2. Indirect ophthalmoscopy

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3. Slit lamp biomicroscope with minus contact lens - Goldmann three mirror contact lens Non contact lens (a) Minus lensHruby lens (b) Plus lensVolk, El-Bayadi lens 4. Retinoscopy for error of refraction, retinoscopy shows coloboma, large exudate, edema growth, detachment as grey reflex. 5. Fluorescein angiography 6. Ultrasonography, A Scan, B Scan 7. OCT (Optical Coherence Tomography) 8. Dark adaptometry 9. Photo stress test 10. Field examination (a) Central 10 (b) Central 30 Peripheral (i) Kinetic (ii) Static 11. Electro physiological tests (a) Electro oculography (EOG) (b) Electro retinography (ERG) (c) Visual evoked response (VER) 12. X-ray skull, orbit 13. CT 14. MRI 15. Trans-illumination. Principles of some of the methods used in examination of retina Direct ophthalmoscope This instrument uses the optics of the eye as simple magnifier of + 60D, the retina acts as the object. The ophthalmoscope acts partly as source of light to illuminate the retina and partly to focus the image of the retina. This gives a virtual image of the retina which is erect and fifteen times magnified in emmetropia. The magnification is less when the diopteric power of the eye is less i.e. aphakia or high hypermetropia where a small image is seen. In contrast to this, the myopic eye gives a larger image than emmetropia. A raised lesion on the retina or in front of the retinal plane requires addition of plus lenses in the viewing system of the ophthalmoscope. Similarly minus lenses are required to see a spot behind the retinal plane

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i.e. floor of optic cup in glaucoma or floor of posterior staphyloma. The direct ophthalmoscope has a small field i.e. 10 at a time, which requires to be shifted frequently to see a peripheral lesion, retina beyond equator is not visible, scleral indentation which makes peripheral lesion visible by indirect ophthalmoscopy is not possible while using direct ophthalmoscope. The illumination of direct ophthalmoscope is not very bright. The direct ophthalmoscope lacks stereopsis. Its higher magnification is better suited for small lesions of macula and examination of optic nerve head by incorporating a grid, the size of a lesion can be measured by direct ophthalmoscope. Indirect ophthalmoscope The condensing lens of the indirect ophthalmoscope forms an inverted, real and magnified image of the retina between the condensing lens and the observer. The indirect ophthalmoscope uses the principle of astronomical telescope where both the eye pieces and the object are plus lenses. The indirect ophthalmoscope has a stereopsis and a large field, the condensing lens commonly used are + 15D, + 20D and + 30D. Commonly used condensing lens is a plano convex large + 20 lens which has anti reflection coating. More is the dioptre of the condensing lens, lesser is the magnification and wider the field. A condensing lens of + 15 gives magnification of 4x while that of + 20 and + 30 give magnification 3x and 2x respectively. Most commonly used indirect ophthalmoscope is binocular indirect ophthalmoscope which has head held viewing system and a hand held condensing lens. Biomicroscopy of the posterior segment (Slit lamp examination of the retina) Slit lamp is a compound microscope with a limited focal length. It is a versatile instrument to examine the anterior segment up to anterior few millimetres of the vitreous, structures beyond this are out of focus. To see the objects deeper to this, it is essential to modify the optics of the eye suitably. A normal emmetropic eye has a dioptric power of + 60, out of this about + 45D+ 47D is attributed to cornea and rest to the lens. The optics can be modified either by 1. Neutralising the power of + 60D by a 60D lens kept away from the eye i.e. Hruby lens which is placed 10 mm to 12 mm in front of the eye under examination. The pupil should be well dilated. This forms a virtual image 18mm in front of the retina. The biomicroscope is focussed on this, one of the disadvantages of Hruby lens is that the size of the pupil is reduced by high minus glass, this hampers the field of examination. 2. Replacing the corneal power by an afocal lens in contact with the cornea as by Goldmann three mirror gonioscope by which not only the posterior pole of retina, but also peripheral retinal and angle of anterior chamber can be examined. This has a disadvantage of being large and heavy. This is avoided by use of Koeppe lens which is light but focuses only the posterior poles. It is good for examination of macula and optic nerve head. The size of pupil is also reduced by these contact lenses and it reduces the field to 20. 3. Using principle of indirect ophthalmoscopy with slit lamp. Plus lenses between + 60 to + 90 are held in front of the eye with a dilated pupil and image is seen through the slit lamp. The image is real, inverted and of same size. The image is away from

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the observers eye. Stronger the power of the lens lesser is the size of the image, with pan fundic lens which is self illuminating, the magnification is 0.7x. Fundus fluorescein angiography This is one of the most important procedures in all retinal and choroidal disorders especially involving the retinal vessels, the retinal pigment epithelium, chorio capillaries and Bruchs membrane. Fluorescein angiography is less required in children than in adults where diabetic retinopathy, retino phlebitis, central vein thrombosis are main indication. In children it is mostly used in congenital anomalies of the retinal blood vessels, sickle cell retinopathy, retrolental fibroplasia, haemangiomas. Fluorescein is a dye that when exposed to light of short wave length gives fluorescence and becomes visible. Intra vascular fluorescein is not visible in white light. To make it visible it should be excited by blue light in the wave length of 490 nm. This excitement of fluorescein then emits a yellow grey light in the wave length of 530 nm which can be recorded on a fundus camera. For this purpose, the fundus camera which is essential part of fundus fluorescein angiography has two filters: (1) A blue excitation filter and (2) Yellow grey barrier filter. 70-85% of fluorescein when injected in ante cubital vein gets bound to the serum protein and is called bound fluorescein, remaining part is called free fluorescein. Free and bound fluorescein differs from each other by virtue of their ability to pass across the cells, RPE and Bruchs membrane. Neither free nor bound fluorescein can pass through the walls of large choroidal vessels. They also do not pass through tight junction of retinal capillary endothelium. A leak from retinal capillary is abnormal. Free fluorescein can pass through fenestrated walls of the chorio capillaries. It can also pass across the Bruchs membrane. It can escape into extra vascular space as well. Free fluorescein does not pass through tightly placed RPE. Fluorescein injected in ante cubital vein reaches the systemic circulation. It reaches the fundus by two routes: 1. Short posterior ciliary arteries. This reaches the choroid earlier than the central retinal artery. 2. Central retinal artery. This delineates the retinal vessels. The dye drains by central retinal veins. The normal fundus fluorescein angiogram has following phases: 1. Pre arterial (Choroidal) phase. The whole of the choroid gives a bright glow under six seconds. Brightness of the glow depends on amount of pigment present in the RPE. A cilio retinal artery when present stands out in choroidal phases. 2. Arterial phase. It takes about 8 seconds for the retinal arteries to fill up. The superior and inferior branches fill early, then the rest of the retinal vascular pattern stand out brightly.

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3. Arterio venous phase. This indicates beginning of venous drainage. The dye is visible in both arteries and veins. The retinal capillaries become visible. 4. Venous phase. It is divided into three distinct phase i.e. early, mid and late phase. Early venous phase. The fluorescein stains the peripheral part of the venous blood. This is attributed to the lower pressure in the veins. The arteries still show fluorescein in them. Mid venous phase. The lamellar appearance of the dye is no more visible. The whole of the vein is uniformly filled with fluorescein. The arteries still retain some stain. The capillaries become visible. Late venous phase. Fluorescence in the vein is less than mid venous phase. This is followed by an increased fluorescence of short period due to recirculation of the dye. Then the dye fades away. Appearance of normal macula in fluorescein angiogram The macula is avascular, more so in foveal avascular zone. Presence of xanthophyll, tall and more pigmented RPE masks the choroidal flush in early phase. Otherwise also a small area of central macula remains hypo fluorescent. The peri foveal arcade is seen in the venous phase. The optic disc. Fluorescence in the vessels of the optic nerve head show variations in various phases due to difference in blood supply in different depth of the optic nerve. The capillaries at the level of lamina are visible in choroidal phase. The capillaries on the surface of the disc and peri papillary capillaries are seen after the choroidal flush. In late venous phase there is staining of optic nerve head. Fundus fluorescein angiogram helps to differentiate between pseudo neuritis, neuritis and papilledema. Abnormal fluorescence The two types of abnormality of fluorescein areHyper fluorescence and hypo fluorescence. Hyper fluorescence is a state of enhanced fluorescence, this can be localised or generalised. The hyper fluorescent area stands out more prominently than the surrounding area. The causes areAbnormality in the vessels, telangiectasis, aneurysms, shunts, neovascularisation, chorio retinal scar, ARMD (not seen in children), myopic degeneration, choroidal rupture. New growthVarious hamartomas, angiomatosis retinea, retinoblastoma, malignant melanoma (not seen in children). Increased transmissionAlbinism (there is lack of pigment in RPE). Window defectAtrophic RPE, angioid streak, drusen. LeakPoolingSeen in late venous phaseRPE detachment, retinal detachment, cystoid macular edema, central serous retinopathy. StainingDrusen, soft exudate, scar. Hypo fluorescenceThis is a state of fluorescence that is either less than normal fluorescence or is totally absent. It can be brought about either by masking of fluorescence or due to filling defect.

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Masked (blocked) fluorescence can be due to pigment, exudate or serous fluid. The pigments areMelanin, lipofuscin and xanthophyll. Blood is a common cause of blocked fluorescence. It could be a haemorrhage in the choroid, retina. Both hard and soft exudates block the fluorescence so do foreign bodies and scar tissues, CSR, disciform degeneration and serous retinal detachment. Filling defectVascular occlusion Retinal artery obstruction, venous obstruction, choroidal infarct, healed scar of choroiditis, coloboma of choroid. Indocyanine green angiography This is less commonly used method of angiography. It is used to see outline of choroidal circulation because it delineates choroidal vessels better than fluorescein. Indocyanine green is a tricarbocyanine compound containing free iodine.31 It is totally bound to serum protein. It is excreted in bile, peak fluorescence is at 835 nm. It is injected intravenous like fluorescein. The fluorescence of indocyanine green is in the infra red range. The angiogram requires an exciting filter at 835 nm. ICG has 25 percent less fluorescence. It requires infra red sensitive video camera and video cassette recorder. It is mostly used to see choroidal neo vascularisation. It is used alternatively in patient sensitive to fluorescein. As ICG is iodine containing dye it is also likely to cause allergic reaction. Other use of indocyanine green is in maculorrhexis.32 Optical coherence tomography33,72,73,74 This is an imaging technology that give high resolution image of retina and optic disc, with modification it can be used to image cornea, lens and iris in diseases of anterior segment. The principle is almost the same as in ultrasonography, CT or MRI. The only difference is that the ultrasound uses sound, CT uses X-ray, MRI uses spin resonance, the OCT uses low coherent near infra red light beam (820 nm - 830 nm) from a super luminescent diode. It is important to have clear ocular media, the pupil should be atleast 4 mm in diameter. The results are reproducible, the method is non invasive, non contact. The system consist of an inter ferometer that gives high resolution (10 to 20 ) in contrast to 100 of ultrasound. The light is delivered through a slit lamp with + 70D lens. It requires an infra red sensitive video camera. OCT is used to detect disorders of retina, especially the macula. It can differentiate between full thickness and partial thickness macular hole, the exact diameter of the hole and thickness of the retina surrounding the hole can be assessed. It can be used to document the progress of the treatment34. It is useful in central serous chorio retinopathy, macular edema, epiretinal membrane and age related macular degeneration, macular cyst. OCT has proved to be a valuable imaging method in evaluation of optic nerve changes in glaucoma. Ultrasonography35 This is yet another non invasive procedure of multiple uses in ophthalmology. It uses inaudible sound. Sound audible to human ears range between 20 to 20,000 hertz (Hz). Sound used for ultrasonography is by far more than audible sound, it ranges in millions of cycles and noted as MHz or megahertz. Sound does not have any radiation or magnetism. Ultrasound used for ocular ultrasonography does not produce heat, it uses very low energy.

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The ultrasound when directed towards the interior of the eye or orbit is reflected back towards the source as it meets a changed density or elasticity in the media through which it passes. The ultrasound is generated in a transducer, which converts electric energy into sound waves. The transducer contains piezo electric crystal. This not only produces echo but also receives it back and converts it into electric potential that can be either photographed or printed on paper. There are two main modes of ocular ultrasonography(1) A scan, (2) B Scan. Each has its own utility. Sometimes both the methods may be required to clinch the diagnosis. The A scan is time amplitude that gives linear one dimensional picture. The B scan is intensity modulated and gives two dimensional picture. Interpretation of A mode requires more skill than B mode. A scan is used to find out ocular dimension i.e. AC depth, thickness of lens, thickness of the cornea. Length of the globe it is most widely used in calculating power of IOL. It has limited value in diseases of globe or orbit. The tracing is shown as a series of spikes. Distance between the two spikes is the distance between two acoustically different surfaces. Normal aqueous, vitreous do not produce any spike but anterior and posterior surfaces of lens give two spikes of almost same height with space in between. The space in between the two spikes is the thickness of the lens. Other important use of A scan is measurement of corneal thickness Ultrasonic pachymetry (Pachometry). Abnormal content of vitreous like blood, blood clot, foreign body, cysts give positive spike. Retinal detachment, retinal tumours and choroidal growths also give positive spike provided they are in the path of the echo. B scan gives a two dimensional picture of the globe and orbit. Instead of spikes it gives dots. Brightness of the dots depends upon size of the echoes reflected. The resulting picture though not very exact but is comparable to histopathological cross section. Different types of lesions have different shadows, some have specific appearance. B scan is mostly used to see vitreo retinal pathology in opaque media or hazy media where indirect ophthalmoscopy is not possible. It delineates opacities in vitreous. It differentiates between traction detachment, exudative detachment, celio choroidal detachment and rhegmatogenous. It also outlines intra ocular tumours, IOFB, endophthalmitis, cysts and parasites, position of dislocated lens. It is more useful in orbital disease than A scan. It is used in thyroid myopathy, orbital pseudo tumours, tumours of the optic nerve. One of the advantages of ultrasonography is that it can be used as many times as possible in all ages in sitting or recumbent position with out any radiation hazard. Dark adoptometery36,37 This investigation is performed less frequently, it measures the difference between recovery of rod and cone functions after they have been exposed to bright light. The cones recover faster than the rods. The cones have no vision in dark. They are photopic while rods function only in dark, they are scotopic. It is common experience that when some one walks in a dark area after being in the bright light for some time, it takes few seconds to start seeing in the dark.

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Similarly a person who has been in dark for some time, when he goes out in bright light takes few seconds to get adapted to the bright light. The instrument is called dark adaptometer that is used in cases of defective night vision due to diseases of retina and choroid. It is used mostly in rod disorders and has less utility in cone disorders. Light sensitivity of the eye has three grades: 1. Photopic Bright light, normal room light or outdoor, this is cone function. 2. Mesopic Dim room light or twilight, this is partly rod and partly cone function. 3. Scotopic This is in a room without light or dark night outside, this is rod function. Commonly used dark adaptometer is Goldmann Weekers adaptometer. Besides this there are other adaptometers likeFeldman adaptometer, Henkind and Seigel scotometer, Combergs nyctometer. To perform the test both the pupil are fully dilated. The patient is made to sit in a semidark room to get dark-adapted for few minutes. Then the eyes are exposed to bright light similar to pre-adaptation light for six to eight minutes. Dark adaptation is started. Retinal sensitivity is tested for first ten minutes at an interval of ten seconds then every one minute, first in ascending order i.e. the light sensitivity is gradually increased until the patient becomes aware of it, then the illumination is gradually reduced until the patient can not see the light. The average of the two is plotted against time. Illumination is plotted vertically and time horizontally. The normal dark adaptation graph is biphasic. It comprises of two concave curves joined to each other. The first part is short and steep, lasts for initial ten minutes and denotes cone threshold while the remaining a longer and shallower curve denotes rod threshold and lasts for about 30 minutes. The junction of the two is called rod-cone-break.38 The rod threshold is prolonged in retinitis pigmentosa, vitamin A deficiency, retinitis albi punctatus.39 Electro retinography38,40 This is an objective method to measure retinal function. It is used to detect function of both rods and cones, was mostly used for peripheral retinal disorders. With development of better computerised methods it is now being used more frequently to assess macular function as well especially in colour vision defects41 It is relatively crude method where all the layers of the retina participate. However involvement of rods, cones, bipolar cells and Mullers cell are reflected better than remaining layers. This is brought about due to transmission of ions across the retina. The test is done by a computerised oscilloscope that gives a visual picture on a screen which may be obtained as print out (electro retinogram) for permanent record. The instrument has two electrodes, one is a modified contact lens that is applied to cornea and the other electrode is applied on the forehead. ERG can be photopic for cones, scotopic for rods. It can be for colour. Mostly used colours are red (long wave) or blue (short wave). A mesopic response can be seen on the ascending arm of the b wave. The electro retinogram is a continuous tracing along a straight line. The spike below the base line is called negative while those above are known as positive waves.

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The various components of ERG are: 1. Early receptor potential (ERP) 2. a wave 3. b wave 3. c wave 4. d wave Out of these only a and b waves have clinical significance. The a wave is a small negative wave, due to hyper polarisation of rods and cones. The b wave is the largest wave both in amplitude and duration. It is a positive wave, it is due to elevation of extra cellular potassium.42 The waves are measured in amplitude and time. The amplitude is measured in microvolts and time is measured in milliseconds. Method The pupil is fully dilated. The eye is adapted, then flash stimulus are given and the electro retinogram is obtained. Uses of ERG are many. They are all diagnostic and have some prognostic value. 1. Diagnose retinal degeneration and dystrophies. 2. They are utilised to differentiate between disorders of rods from cones. 3. They are used to detect possibility of presence of retinal degeneration in the asymptomatic members of the family. 4. They can be used to detect malingers. 5. They may be used to assess retinal function in presence of opaque media. The common conditions where ERG is useful in conforming the diagnosis are: Retinitis pigmentosa and allied retinal degeneration Lebers congenital amaurosis Achromatopsia Congenital stationary night blindness Oguchis disease Choroideremia Gyrate atrophy of choroid Quinine amblyopia. It has no role in ganglion cell disorder, hence it is not used in glaucoma and diseases of optic nerve. Rubella retinitis gives fundus picture similar to retinitis pigmentosa but have normal ERG. The female carriers in X linked retinitis pigmentosa may show abnormal b wave in absence of night blindness.

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The ERG may be extinguished in advanced retinitis pigmentosa and allied conditions, total retinal detachment, diffuse unilateral sub acute neuro retinitis, ophthalmic artery occlusion. Electro oculograph42 Electro oculograph has relatively less use than ERG in evaluating the retinal functions because in most of the cases that have, either absent or grossly subnormal ERG, also have subnormal EOG except in few cases i.e. stationary night blindness, X linked retino chisis, dominantly inherited Bests vitelli form, macular dystrophy.38 Electro oculography like ERG is an objective method to measure the retinal function like ERG. It also measures mass response of retina. It basically measures difference in cornea and retinal potential on horizontal movement of the eye. The corneal potential is positive while the retinal (back of the eye) is negative. The difference between the two is 6 mv. The difference depends upon excursion of the eye, level of illumination and adaptation. It is also useful in some conditions of subtle extra ocular muscle palsy with modification. It can also be used to record nystagmus, where the instrument is called electro nystagmograph. The instrument is an oscilloscope. It has two electrodes, one is attached near the medial canthus and the other near the lateral canthus. The former is corneal electrode and is positive while the latter is retinal electrode and is negative. For retinal function test, it indirectly records the standing potential of the eye, which is caused due to voltage difference between inner and outer retina. It evaluates retinal pigment epithelium mostly. To get the reading the pupil is dilated. First, the patient is dark adapted. The electrodes are placed one on the medial canthus and the other on the lateral canthus. The patient is asked to move the eye alternately, medially and laterally with approximate 60 excursion between the two. The oscilloscope is activated and the spikes noted. After dark adaptation the response progressively decreases reaching the trough in 8-12 minutes, which is lower than the base line. The light adapted trough is well above the base line and is reached in 6-9 minutes. The whole process requires about 20 minutes for each eye. To obtain the graph, the amplitude in microvolts is plotted against time. The final reading is a ratio between the light peak and dark trough called Arden ratio i.e. Maximum height of light/depth of the dark trough. The normal is 185 or more. Reading between 125 and 150 is subnormal and less than 125 are flat. The response is independent of opacity in media. Myopia, trauma and ocular surgery give low response. Visual evoked response (VER) This is an objective test to assess function of visual path beyond the retinal ganglion cells. It is commonly used to access macular as well as optic nerve function in pre geniculate path. It has some use in amblyopia and colour blindness. It is basically modified form of electroencephalogram. It records electric activity of the visual cortex area 17,18 to light stimulus. The VER records primarily the central visual function43. Opacities in the media do not influence the response.

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The VER is of two types: 1. Flash VER 2. Pattern reversal VER A typical pattern consists of a latency of 75-80 ms and a positive wave of 110 ms. The positive wave has a peak of 9 ms. Method of recording The patient sits in a quiet dark room for sometime in front of a screen at a distance of 1.5 metres. One eye is tested at a time. The potential is recorded by an electrode placed over the occiput. The other electrode is put on the vortex. Hundreds of responses are summated and averaged by a specially programmed computer. In case of flash VER, a flash of light is used to start the response. In case of pattern reversal VER, a chequer board with alternate black and white squares of equal size and intensity are displayed on the screen. The colour of the squares can be reversed i.e. the black becomes white and white becomes black. The flash VER is used to test the function of the macula and visual pathway in non verbal children, mentally retarded children and aphasic children.44 It can detect malinger and visual potential in opaque media especially in large corneal opacity where the pupillary reaction can not be elicited. The form reversal VER uses form sensation hence this gives a rough idea of visual acuity. VER is useful in optic neuritis due to demyelinating disease where VER may be absent in acute stages. VER is abnormal in multiple sclerosis even before diminished vision sets in. It is useful in compression lesions of optic nerve. In cortical blindness the response is absent.45,46 Photo stress test This is a crude form of dark adaptation test. The visual pigments are rendered insensitive to ordinary light causing a temporary relative scotoma which passes in some time with fading of scotoma. The recovery time depends upon ability of the photo receptors to re-synthesise visual pigment. This is used for macular lesion and has hardly any use in peripheral disorders. This differentiates macular cause of loss of vision from optic nerve cause. To perform the test, the distant vision is recorded with best correction. Then the eye is dazzled by the light of indirect ophthalmoscope kept at about 3 cm from the eye and the patient is asked to read the best corrected line. Normally it takes about 30 seconds to recover. In a case of macular disease, this is prolonged over 1 minute. Visual loss due to optic nerve lesion is not effected by photo stress. Interpretation of physical examination of retina (Signs of retinal disorders) The signs of retinal disorders are many and varied. They depend on part of the retina involved and associated involvement of vitreous, choroid and optic nerve. The signs may be limited to retina only where it can be central (macular), peripheral or combined. The signs can be secondary to choroid as chorio retinitis. This is more common than reverse i.e. retino choroiditis that is pathology spreading from retina to choroid. However neuro retinitis is as common as inflammation spreading from retina to optic nerve. Tumours of optic nerve do not metastasise in retina. But spread of retinoblastoma through optic nerve is common.

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Duration of the disease too plays an important role in appearance of the signs. The common signs are: Signs of retinal disorders are essentially situated on the fundus which is visible either by ophthalmoscope or by slit lamp. They are changes in the blood vessels, they include1. Attenuation, 2. Dilatation, 3. Tortuasity, 4. Anastomosis. Attenuation. Normal arteries are thinner than veins but in some pathological cases the arteries become thinner than normal or may look to be so if the veins are dilated. The thinning can be generalised or localised. Generalised thinning is more common than segmented attenuation. The most common causes of generalised thinning of the arteries is retinitis pigmentosa and allied conditions. Other causes of attenuation like arterio sclerosis and hypertension is not seen in children. However retinal artery occlusion may occasionally seen in children. Commonest cause being embolism from the cardiac valves in sub acute bacterial endocarditis. In post papilledematous and consecutive optic atrophy, there is thinning of the arteries. Dilatation of arteries is far less common, it is seen in leukaemia and in angiomatosis retinae or cirsoid aneurysm of the retina. Dilatation of the veins is more common. It is seen in cases of papilledema and venous obstruction. In angiomatosis retinae the vein may be greatly dilated. Tortuosities of both arteries are uncommon in children. They are mostly seen as congenital anomalies like angiomatosis, Coats disease, telangiectasis. Anastomosis. Anastomosis between two systems of circulation i.e. retinal and ciliary and between artery and veins are always pathological. The effect of abnormal vascularity leads to: 1. Hyperemia of the retina 2. Anaemia of the retina 3. Edema of the retina 4. Exudates on the retina 5. Haemorrhages on the retina 6. Neovascularisation. Hyperaemia of the retina may be arterial or venous. Arterial or active hyperaemia is seen in dilatation of the arteries and increased tortuosity of the retina. It can be generalised or localised. Common causes are retinitis and uveitis. Venous or passive congestion is caused due to impeded venous outflow. Common causes being papilledema and papillitis. Other causes are systemic condition like leukaemia, polycythemia and chronic heart failure. Children in teens may have periphlebitis. Other causes are sickle cell retinopathy and cavernous sinus thrombosis. Diabetes and hypertensive retinopathy rarely cause hyperaemia in children. Anemia Commonest cause of pallor of retina is systemic anemia. Not only the general appearance of the retina is pale but the optic nerve also looks pale. Anemic fundus is common in leukemia

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and lipidosis also. Pallor can be true pallor due to diminished blood supply or may look pale with normal blood supply Arterial obstruction is the commonest cause of pallor. It may be generalised when the main trunk of the central retinal artery had been obstructed or segmental when one of the branches has been obstructed. Other causes of generalised pallor of retina with normal blood supply and normal haemoglobin are albinism, myopia, Waardenburgs syndrome, choroideremia. Causes of localised pallor are- Retinal edema, coloboma of retina, retino schisis, flat retinoblastoma, atrophic areas. Retinal edema The main pathology in retinal edema is break down of blood retinal barrier, either the inner barrier or outer barrier. This is brought about by any of the following two or combination of the two1. Excessive leak of fluid from capillaries due to increased permeability as is seen in inflamation or vascular retinopathy. 2 Break down of larger protein molecules. Break down of the inner blood retinal barrier leads to localised edema as cystoid macular edema or post traumatic edema. Diffuse retinal edema is seen in vascular retinopathies. Break down of outer blood retinal barriers lead to central serous retinopathy and wet type of age related macular degeneration. Retinal edema has greater predilection for posterior pole mostly macula. The common causes of retinal edema in children areblunt injury, optic neuritis, papilledema, neuro retinitis, retinitis, chorio retinitis, retinopathies, cystoid macular edema. Edema of the macula is associated with diminished central vision, there may be acquired hypermetropia, central scotoma, metamorphopsia. Peripheral retinal edema may be symptomless or may be associated with localised scotoma. It is difficult to detect mild retinal edema with ophthalmoscope. In moderate edema the retinal surface has an albuminous appearance, the retina looks thickened and has granular appearance. Macular edema generally presents with a circular raised area. A macular edema is said to be clinically significant macular edema if it has following features1. Retinal edema is within 0.5 mm of fovea centralis 2. Retinal edema is larger than 1 disc diameter within one disc diameter of fovea. 3. Hard exudates within 0.5 mm of fovea associated edema of the retina near by. Presence of any one of the above is diagnostic47 Clinically significant macular edema is best elicited by + 90 D on slit lamp biomicroscopy. Separation of nerve fibres due to accumulation of fluid near the disc is visible with red free light of ophthalmoscope. Exudates on the retina Presence of retinal exudate is always pathological. There are conditions that look like exudates but are not exactly exudates. Common among them are- Small opaque nerve fibres and drusen of the retina. Clinically exudates have been classified into two types on the basis of their ophthalmoscopic appearance. They are:

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Soft exudates. They are due to disturbance of axoplasmic flow that result in accumulation of axoplasmic waste product in the nerve fiber layer, hence they are called superficial exudates. Their cotton wool appearance imparts the term soft exudate to them. They are basically vascular in nature. There is sudden focal obliteration of capillaries in small areas resulting in ischemia followed by anoxia. The infracted area leads to proliferation of neural element in the retina. The soft exudates are white in colour, their edges are irregular or feathery, they are scattered, do not coalesce to form specific pattern, are acute in onset, disappear in two to three month. On fluorescein angiography, they are non fluorescent, surrounded by area of dilated capillaries. Common causes are retinitis, hypertensive retinopathy, severe anemia, dysproteinemia, collagen diseases, angiomatosis, telangiectasis. leukaemia, trauma, papillitis, papilledema, septicimia, rheumatic heart disease, sub acute bacterial endocarditis. Hard exudates. Though they are seen more commonly in vascular retinopathy, there are more of exudative nature than vascular. To begin with there is neural degeneration due to defective oxidative process. The products of degeneration are generally lipid in nature, may be associated albumin, cholesterol, hyaline material or even calcium. They are situated in deeper layer of the retina hence are known as deep exudate. They are round in shape with clear-cut edges, size may vary from pin head to as large as disc, generally seen in posterior pole and are always underneath the blood vessels. Lipid content is most probably the causes of their yellowish grey colour. They are responsible for formation of macular star and arranged in a wreath like pattern in non specific circinate retinopathy. They develop over months and take months to disappear. Common causes of hard exudate areDiabetic retinopathy, late stages of hypertensive retinopathy, Doynes choroiditis, disciform degeneration of macula, Coats disease. Both the types of exudates may exist in the same eye at the same time and one may be superimposed on the other. Both the types are non-specific, may be present without haemorrhages. However presence of haemorrhage along with exudate is very common. Retinal haemorrhage Retina is supplied by an elaborate network of blood vessels with large capillary bed. Hence bleeding from them is very common. The causes of retinal haemorrhages are many. Sometimes more than one causative factor may be responsible. Causes of retinal hemorrhages can be broadly divided into: 1. Traumatic. Closed globe and open globe. It is common for retinal vessels to be torn in traction retinal detachment, however there may be tear in the blood vessels in rhegmatogenous retinal detachment as well. 2. Inflammation. More common cause is periphlebitis, less common is periarteritis. 3. Retinopathy. Perhaps largest numbers of retinal haemorrhages are seen in various types of retinopathies, fortunately many of them are not seen in children. Common

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retinopathies areSickle cell retinopathy, malignant hypertension and nephritis. 4. Blood disorders. Anaemia, leukemia, purpura, haemophilia, sickle cell anaemia are common in children. 5. Miscellaneous. Papilledema, papillitis, telangiectasis, hamartomas, Coats disease, retinoblastoma 6. There are two conditions that are not true hemorrhages but look like hemorrhage: (a) Cherry red spot (b) Macular hole Causes of cherry red spot are: 1. Various errors of metabolism and storage diseases - Tay Sachs disease, Niemannpick disease, Farbers disease, Sandhofts disease, Hurlers disease, mucopolysaccharidosis. 2. Central retinal artery occlusion (rare in children). Types of retinal haemorrhages Depending up on anatomical location of the hemorrhage in relation to the retina they have been divided into following groups: 1. Inter retinal 2. Pre retinal 3. Sub retinal (a) Superficial (b) Deep (a) Sub hyaloid haemorrhage (b) Vitreous haemorrhage (a) Choroidal hematoma (b) Sub retinal neovascularisation. Inter retinal haemorrhages There are mostly capillary in nature but occasionally can be arterial or venous. They are called superficial when they are in the nerve fiber layer. Superficial haemorrhages are bright red in colour, are typically flame shaped or leaf shaped with irregular border. They are deeper to the retinal vessels and are known to fade over weeks to months without treatment. Their number and size may vary from single to multiple, from small spots hardly visible to larger than manydisc diameter. They may be localised or scattered. Peripheral hemorrhages do not cause visual disturbance but a small hemorrhage on the fovea causes extensive visual loss. The hemorrhage themselves are non fluorescent and block fluorescein. Common causes are hypertension, papillitis, papilledema, retinitis and trauma. Roths spots are peculiar superficial haemorrhages, which are flame shaped haemorrhages with central white area commonly seen in sub acute, bacterial endocarditis, anaemia and purpura. Deep haemorrhages Haemorrhages in deeper layer are small, circular. Their colour is less brighter than superficial haemorrhages. Compactness of the deeper layers of the retina prevent them to spread, hence

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they are localised and small dot shaped. The retinal vessels pass over them, commonly found in the posterior pole, may be scattered or may be in bunches mimicking retinal micro aneurysm from which they are differentiated by fluorescein angiography. The deep haemorrhages are not fluorescent and block the fluorescein. The micro aneurysms are fluorescent. Commonest cause of deep haemorrhages is diabetic retinopathy. It is not uncommon to see mixture of both superficial and deep haemorrhages in the same eye in case of diabetic retinopathy. Sub hyaloid haemorrhage These are also known as pre retinal haemorrhages. They occur between the internal limiting membrane of the retina and vitreous without invading into the substance of the vitreous. The source of the bleeding is retinal vasculature. They are relatively rare but not altogether absent in children. They may be seen even in new born. The common causes in children areBlunt trauma, retinal tear with or without detachment, Purtschers retinopathy, penetrating injury, accidental perforation of the globe during squint surgery. Children in second decade may develop pre retinal haemorrhage in retro lental fibroplasia, persistent hyaloid artery, Coats disease, angiomatosisretinae, juvenile retino schisis, and drusen of optic nerve. Periphlebitis retinae (Eales disease) is commonest cause of pre retinal and vitreous haemorrhage in young male adults. Commonly seen in third and fourth decade. Eales disease may have an early onset, may be seen as early as fifteen to sixteen years. Similarly diabetic retinopathy is commonest cause of pre retinal and vitreous haemorrhage after fourth decade. In rare instances untreated juvenile diabetes may present as retinal or pre-retinal haemorrhage. Characteristics of pre-retinal (sub hyaloid) haemorrhages Symptoms and signs depend upon position of the haemorrhage, size of the haemorrhage and associated lesions. Symptoms It may be symptomless if the haemorrhage is in the lower part of the retina, away from the macula. Peripheral haemorrhage may go unnoticed. A lesion in front of the macula is cause of sudden painless loss of vision. In very large haemorrhages vision may be reduced to hand movement with good projection. A patient with fresh haemorrhage may complain of red vision (erythropsia) which passes off in few days. Generally vision improves with passage of time and may return to normal as blood absorbs. The patient may complain of black spots, after the vision has come to normal. A typical sub hyaloid haemorrhage if seen within few hours of onset, starts as a circular patch of bright red spot generally in the posterior pole. Gradually the heavy particles of blood settle down at the bottom of the original oval patch. The upper border becomes straight and parallel to the ground and retains its bright red colour. The upper part contains serum hence is either clear or yellowish in colour. The sub hyaloid haemorrhage has no tendency to clot. In case of Tersons syndrome, a sub-arachnoid haemorrhage may find its way on the retina along the optic nerve and may look flame shaped and rarely keel shaped which is a common appearance of pre macular haemorrhage.

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All the cases with sub hyaloid haemorrhage should routinely be examined under mydriasis with indirect ophthalmoscope. The advantages of examination by indirect ophthalmoscope are that it can circumviate a large haemorrhage, and makes examination of the retina possible inspite of its presence. It gives excellent information for peripheral-lesions especially in periphlebitis. It also gives information about the haemorrhage in three dimension. Vitreous haemorrhage So long the outer membrane of the vitreous is intact, it is not affected by haemorrhage. Only when new blood vessels grow in the vitreous, a haemorrhage develops. A small haemorrhage initially behaves like a pre retinal haemorrhage. The causes of vitreous haemorrhages are the same as pre retinal haemorrhage. Generally the haemorrhages absorb without any treatment within days to weeks. However if bleeding occurs frequently, the clearance time is proportionally prolonged and a stage may come when the haemorrhage does not clear and a stage of hemophthalmos develops, which may require vitrectomy followed by endo laser photo coagulation. Fate of retinal haemorrhages Most of the fresh and small haemorrhages absorb without leaving any trace. This happens following hemolysis of RBC when there is no repeat bleeding and the haemorrhage is small. In case of multiple haemorrhages, larger the size, longer is the time taken for absorption. This is generally followed by proliferation of glial tissue called retinitis proliferance, which are vaso proliferative in nature. The proliferation can be from the superficial layer of the retina or the retinal blood vessel. The bands develop more commonly near the disc and extend into the vitreous body. They may be only a few or very extensive. Fine capillaries develop along or on the proliferative bands. As these vessels are unsupported, they bleed more commonly than other retinal blood vessels. The conditions that cause retinitis proliference areTrauma mostly penetrating, retained intra ocular foreign bodies, retro lental fibroplasia, angiomatosis retinae, Coats disease, sickle cell retinopathy, Eales disease. In contrast to this common causes in adults areEales disease, proliferative diabetic retinopathy and venous occlusion. Sub retinal haemorrhage They are not true retinal haemorrhages, they are in fact choroidal in nature. The bleeding occurs between the pigment epithelium of the retina and the choroid. They are less frequent in children than in adults. Frequent causes of sub retinal haemorrhage are - Blunt trauma, choroidal rupture, myopia, drusen of optic nerve head, chorio retinitis and rubella retinopathy. The sub retinal haemorrhage looks like blackish oval raised area over which retinal vessels pass. The lesion is better visualised by indirect ophthalmoscope and biomicroscope, confirmed by fluorescein angiography and ultrasonography. The basic pathology is formation of new vessels under the retina. Retinal aneurysm They are very common in adult diabetics, occasionally can be seen in juvenile diabetic. In adults, they are small hence are called micro aneurysms. In children they are relatively larger. On fundus examination they look similar to bunch of deep haemorrhages. They are best

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demonstrated by fluorescein angiography. The causes of retinal aneurysm in children are Coats disease and early onset peri phlebitis. Anastomosis of retinal vessels The retinal arteries are end arteries, they do not anastomose normally except as congenital anomaly between central retinal artery and cilio retinal artery. There may be anastomosis between veins as in angiomatosis. There may be anastomosis between the retinal circulation and choroidal circulation in toxoplasmic chorio retinitis.48 Inflammation of retina Inflammation localised to retina is less common than retino choroiditis and neuro retinitis. Associated vasculitis is common. Retinitis can be localised or diffuse. It may involve sensory retina or retinal pigment epithelium separately or in combination. Retinitis has been divided into two basic types: 1. Primary, where retina and retinal vessels are involved as primary site. 2. Secondary, when inflammation spreads from choroid mostly and optic nerve less frequently. It can be non specific or specific like viral, bacterial, fungal or parasitic when associated with systemic diseases it can be bilateral, otherwise in case of unilateral retinitis, the other eye may get involved later. Retinitis in children is mostly endogenous. It can be purulent, exudative or granulomatous. Purulent retinitis can be acute or sub acute. Acute purulent retinitis is mostly due to lodging of pus producing bacteria or fungus in septicimic child. Generally the bacteria reaches the retinal vasculature from distant organ and cause purulent retinitis which may pass into endophthalmitis or even panophthalmitis within days or weeks. The condition starts in an acutely ill, malnourished child, as sudden gross loss of vision without pain or congestion. On examination the retina is oedematous with dilated veins. There may be superficial exudate and haemorrhages. If the disease process progresses, it becomes painful and congested, with still further loss of vision going into endophthalmitis and loss of eye. The condition is generally unilateral but may be bilateral. Management is early recognisation of ocular problem and treatment of systemic septicimia. Sub acute infective retinitis (Retinitis of Roth) This is milder form of embolic retinitis where an embolus from heart may get lodge in the capillaries of the retina in the posterior pole. Commonest condition leading to sub acute retinitis is sub acute bacterial endocarditis. The lesion may be single or multiple. There may be recurrence of the episode. There is generally very low grade of retinal reaction. The typical lesion is a large superficial haemorrhage with white centre. A lesion near the macula draws attention early. Most of the time the systemic condition overshadows the ocular pathology. Treatment is directed to the systemic condition. Other causes of Roths spot should be excluded. Specific retinitis of clinical importance in children are: 1. Viral 2. Bacterial Non granulomatous Granulomatous 3. Parasitic

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Viral retinitis in children Many of the viruses with systemic involvement may cause primary retinitis. Viral infection of choroid is invariably associated both DNA group of viruses i.e. herpes viruses which produce chorio retinitis. RNA viruses i.e. rubella, rubeola (measles) cause retinitis. Healthy adults rarely develop viral retinitis. It is common in neonates as part of intra uterine infection, the two examples are rubella and cytomegalo viruses. The viruses are inter cellular, obligatory parasites that cause necrosis and death of the cell leading to permanent damage to the retina. Vasculitis is common in retinitis, the inflammatory response to virus is immune mediated. Retinal hypoxia is frequent. It is due to occlusion of retinal vessels. The patches of the retinitis may be localised or scattered. The after effect may be simple pigmentary disturbance without visual loss to devastating visual loss due to exudates, haemorrhages, gliosis, retinal detachment, secondary glaucoma and cataract. Optic neuritis is more common in lesions of the posterior pole. It is difficult to diagnose the exact virus responsible for retinitis by ophthalmoscopy because most of them have similar features of retinal edema, superficial exudates and haemorrhages, cells in the vitreous. Specific diagnosis is possible only with positive viral culture and serology.49,50 Common viruses that cause retinitis in children are: Rubella, herpes simplex, herpers zoster, cytomegalo virus, Epstein-Barr virus. Rubella retinitis is a congenital bilateral retinitis, also known as embryonic pigmentary retinopathy as the predominant feature is pigmentary disturbance of retina without vascular changes or night blindness. The condition is seen in 50% of children born of mothers who have contracted rubella in first trimester of pregnancy. In absence of more serious ocular involvement like rubella cataract and rubella glaucoma, the condition may go unnoticed unless looked for specifically. The condition does not involve macula, hence there is no diminished vision. However as a late complication there may be sub retinal neovascularisation. As there is loss of pigment from the RPE, fluorescein angiography shows area of hyper fluorescence. There is no treatment for rubella retinitis. The best management is prevention of rubella in mother that is achieved by immunising every girl child by rubella vaccine in childhood. Equally important is to differentiate the fundus picture from other embryonic retinopathies like congenital syphilis, varicella, influenza and radiation to the mother in first trimester by doing torch test and other serological tests for rubella. Commonest retinal dystrophy that presents with pigmentary changes is retinitis pigmentosa and its allied conditions. Retinitis pigmentosa is always associated with night blindness, have attenuated blood vessel and coarse spider like pigments, mostly in mid periphery. The pigments in rubella retinopathy have salt and pepper mottling. Rubeola retinitis (Measles retinitis) Involvement of retina is rarer than involvement of cornea and conjunctiva. Retina is involved when the child is serious enough to develop subacute sclerosing panencephalitis which itself is a devastating neurological disease. The ocular involvement consist of bilateral necrotising retinitis involving macula. There may be evidence of optic neuritis with superficial haemorrhages and exudates which is healed by formation of chorio retinal scar. There is no separate treatment for ocular manifestation. The child should be under treatment of pediatrician. The best management consist of immunisation of children routinely by measles vaccine.

430 Herpes simplex retinitis

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It is seen in neonates who suffer from systemic herpes simplex infection that may include herpes simplex encephalitis. It may develop in children who are on systemic immuno suppressive drugs. Fundus picture consists of superficial exudates, haemorrhages, sheathing of vessels with vitritis. Rarely there may be exudative retinal detachment. Management consists of treatment of systemic herpes simplex. Herpes zoster retinitis As a general rule, herpes zoster infection is rarer in children as compared to adults. Retinitis is still infrequent. It mostly involves posterior pole. It develops after cutaneous lesions have subsided. The intra ocular lesions are immune mediated. There is localised necrosis of retina, vasculitis is common. Treatment consists of systemic antiviral drugs along with judicious use of steroids. Cytomegalo virus retinitis (C.M.V.) Incidence of cytomegalo virus has shown a sharp upward swing in past one decade due to increased awareness among the ophthalmologists and increase in number of susceptible persons. There are two modes of contracting the disease; the first is transplacental spread from the mother and second is seen in immuno- compromised children. More than 80% of general population show antibodies to the virus without manifest signs or symptoms. The incidence of retinal infection rises with fall in immuno compatibility in children by way of spread of HIV from mother to the foetus. Use of infected needles, transfer of infected body fluid, repeated blood transfusion by unsafe blood transfusion, prolonged chemotherapy for malignancy and immuno suppressive drugs for organ transplant increase the risk. Congenital cytomegalo virus retinitis presents as localised necrotising retino choroiditis any where on the retina, common site being macula and retinal periphery that may lead to optic atrophy. On some occasion there may be pan retinal involvement with acute necrosis. Differential diagnosis consists of toxoplasmosis, and nematode infection. The acquired disease is common among children with AIDS, The virus is a member of herpes virus family. It reaches the retina by hematogenous spread. Onset on CMV is CD4 plus cell dependent. It never occurs in AIDS patient who have CD4+ count more than 50/mm3. It begins as unilateral disease to become bilateral soon. The condition is uniformly vision threatening when not treated. Retinitis does not require separate treatment, usual treatment by specific triple anti viral drugs for AIDs. Bacterial retinitis Bacterial retinitis could be acute non granulomatous due to spread of pyogenic organism from systemic focus of infection or may be exogenous due to penetrating injury, most of the time leading to endophthalmitis. Incidence of granulomatous retinitis has been reduced in recent years due to effective chemotherapy of tuberculosis, syphilis, which used to be two main causes. Acquired syphilitic retinitis rarely occurs in children. More common is congenital syphilitic retinitis in children that causes embryonic pigmentary retinopathy with typical pepper salt appearance without night blindness.

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Treatment consists of treatment of systemic syphilis. Tuberculosis produces tubercular chorio retinitis. More common granulomatous retinitis are due to toxoplasmosis and toxocara infestation. Toxoplasmal retinitis Toxoplasmosis retinitis is perhaps the most common causes of retino choroiditis involving macula in children. There are two modes to acquire toxoplasmosis. The commonest is transplacental transfer of the parasite from the infected mother to the foetus. The second less common is acquired-form seen in adults. In between the two is a condition where old lesions are reactivated and present as active lesion little away from the original macular lesion, and called satellite lesion. Systemic acquired involvement is mild, which may be passed off as usual cold unless the patient is pregnant or immuno-suppressed. In an infected pregnant woman there are 40% chances that the foetus will be involved and the child will have multi systemic involvement. The foetus is only at risk if the mother is infected during pregnancy. Subsequent pregnancies are safe from toxoplasmosis. The three clinical features of congenital toxoplasmosis are chorio retinitis, convulsion and intracranial calcification. 80% of infants with congenital toxoplasmosis will have retinal involvement. The parasite has predilection for the nerve fibre layers of the retina. It is bilateral in 80- 85% of cases. Commonest site in retina to be involved is macula, which is generally missed unless looked for or the child has other systemic signs of toxoplasmosis like convulsion, hepatespleenomegaly. All children with convulsion and hepatospleenomegaly should have their fundus examined for evidence of toxoplasmal chorio retinitis. The condition is generally diagnosed late when the child presents with esotropia. By that time the lesion is a healed scar, that may be many times larger than optic disc, with chorio retinal scar on the periphery and white centre. The condition should be differentiated from congenital coloboma of the macula. At this stage there is no effective treatment available. However the child should be under regular observation for years, as there is a possibility of reactivation of the lesion. The reactivated lesions may be single or multiple, always behind the equator. It is associated with anterior chamber reaction, vitreous precipitates, vitreous detachment and papillitis. Diagnosis There are many tests available to diagnose toxoplasmosis, common among them are serological tests to detect anti toxoplasmo anti bodies, Hemagglutination test, indirect fluorescent anti body test, ELISA test. Titers of these tests are lower in ocular lesions. Any positive titer is significant with characteristic fundus picture. x-ray skull is associated with intra cranial calcification. Differential diagnosis Differential diagnosis of congenital toxoplasmosis in congenital coloboma of macula. Commonest presentation of acquired toxoplasmosis of retina is a localised necrotising retinitis. All the

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infective conditions that produce similar picture form a long list. Possible causes are Tuberculosis, syphilis, cytomegalo virus retinitis, herpes simplex retinitis, acute retinal necrosis, candida retinitis. The above conditions may be superimposed on toxoplasma infection, hence mere presence positive serological results for toxoplasma does not rule out these. Management There is no prophylaxis against toxoplasmosis. Treatment consists of: 1. Systemic anti toxoplasma chemotherapy 2. Local treatment of anterior segment involvement 3. Management of squint, amblyopia. Chemotherapy is indicated in vision threatening retino choroiditis involving the macula, papillo macular fibre lesions near the disc or severe vitritis. The basic pathology of toxoplasmosis retinitis is active multiplication of organism accompanied by inflammatory reaction and scar formation. The aim of the treatment is to minimise multiplication and destroying the already present organism by chemotherapy along with reduction in inflammation by anti-inflammatory drugs. Chief out of them being systemic steroids. Common drugs used are (They should be given in consultation with pediatrician) 1. Sulphonamide (a) Sulphadiazine alone (b) Triple sulpha. 2. Pyrimethamine. Adult dose is loading dose of 75 mg-150 mg followed by 25-50 mg daily for four weeks. The drug is folic acid antagonist so it should be seen that the child is not prescribed folic acid during this treatment. Folinic acid in a dose of 5 mg. three times is said to protect bone marrow. 3. Clindamycin is given four times a day for three weeks. Clindamycin is known to produce pseudo membranous colitis. 4. Other newer drugs available are Atovaquone, roxithromycin and azithromycin. Systmic steroid should be used under cover of chemotherapy with usual precaution. Retinal toxocariasis51,52 Ocular toxocariasis is a world wide problem. It is seen more commonly in developing countries and in children who come constantly in contact with puppies that are infected by toxocara canis. Children are infected by swallowing eggs of the parasite that are found in the soil, contaminated by faeces of infected puppies. The eggs are converted as larva in the stomach and intestine of the child and penetrate the wall of the bowel and reach systemic circulation that takes them to distant organs including retina. One of the peculiarities of the parasite is that it is never converted in to adult worm is humans hence the stool of the child never shows evidence of worm infestation. It can infect the eye without systemic involvement. In fact concurrent ocular and systemic infection is rare.

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There are three modes of presentation of intra-ocular toxocariasis: 1. Sub macular granuloma that later involves whole of the macula 2. Peripheral granuloma that may present as parsplanitis 3. Larval endophthalmitis. The macular granuloma is limited to posterior pole hence there is no anterior segment reaction or redness but vision is poor. Solitary localised macular lesions may go unnoticed as they are generally unilateral or may present with squint, otherwise they are discovered on routine vision check up. There is generally pre retinal gliosis that may cause grey reflex on retinoscopy. The peripheral retinal granuloma is also solitary and unilateral with minimal anterior chamber reaction similar to parsplanitis. The lesion is oval nodule with pre retinal gliosis and formation of vitreous bands that may extend back into to macula or even disc causing traction retinal detachment. Larval endophthalmitis is the most extensive form of the infection of chronic nature and is a cause of white reflex in pupillary area. It may terminate in complicated cataract, glaucoma and blindness. Differential diagnosis consists of retinoblastoma, persistent primary hyperplastic vitreous, Coats disease, endo-genous bacterial endophthalmitis, toxoplasmosis, retinopathy of prematurity, congenital and traumatic cataract, familial exudative vitreo retinopathy. The diagnosis is by exclusion. There is no specific test for toxocariasis. ELISA is most sensitive test but also gives false positive result in about 10 percent of cases. Management consists of prevention, as far as possible, deworming of household puppies is best available method. Ant helminthics given to the child have not proved to be effective. Retinal vasculitis Retinal vasculitis is rare in children. Idiopathic periphlebitis (Eales disease), which is commonest form of periphlebitis in adults between 20 to 40 years of age is not seen in children, however there may be secondary vasculitis in older children due to parsplanitis, tuberculosis, cytomegalo virus. They may present as Eales disease. The peripheral retina is involved more than posterior pole. The peripheral lesion may go unnoticed unless they present with diminished vision. Retinal changes include peripheral vasculitis, sheathing, neovascularisation, superficial and subhyaloid haemorrhage, retinal band, traction detachment, non perfusion of vessels. Treatment consists of: 1. Treatment of the primary cause 2. Management of neovascularisation and non-perfusion by laser photo coagulation. 3. Management of non absorbing hemorrhage by vitrectomy with endo laser photo coagulation. 4. Management of retinal detachment.

434 Vascular retinopathies in children

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Vascular retinopathies especially diabetic retinopathy are common cause of blindness in adults, fortunately they are very rare in children. Common vascular retinopathies in adults are: 1. Diabetic retinopathy 2. Hypertensive retinopathy 3. Renal retinopathy 4. Toximia of pregnancy 5. Sickle cell retinopathy 6. Atreosclerotic retinopathy. Proliferative diabetic retinopathy may be seen in older children with type I diabetes53 Similarly hypertensive retinopathy can be seen in secondary hypertension due to any cause as Schies grade IV classification. Chronic glomerulo nephritis may present as renal retinopathy comprising of sclerotic changes in arteries, retinal edema, superficial haemorrhages, occasional deep haemorrhages, soft exudates, may have macular fan and blurring of disc margin. Retinal dystrophies and degenerations Although both the terms dystrophies and degeneration are used as interchangeable terms for same group of disease, they are not synonymous. Dystrophies are abiotrophic disorder, which means that they are genetically determined disorders, which are not evident at birth, manifest late in life. There is premature degradation of highly differentiated cells in specialised tissue like brain, and retina. The cause of dystraphy is supposed to be a programmed death called apoptosis. The dystrophies are progressive, and bilateral, generally symmetric. Some of them may have faculty enzyme or some other bio-chemical defect. Most of the conditions are not preventable and do not have effective treatment. Degenerations are generally acquired, secondary to infection, inflammation and trauma, may be unilateral, may be prevented by directing treatment towards the primary cause and are non-hereditary. The retinal dystrophies are a group of disorders that are difficult to classify. Some of them are more common i.e. retinitis pigmentosa than others. Most useful classification is on the basis of layers of retina involved: 1. Photo receptor dystrophy (i) Rod and rod-cone dystrophy (ii) Cone and cone-rod dystrophy (a) Progressive cone dystrophy Type I and type II (b) Stationary cone dystrophy (c) Cone monochromatism.

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2. Ganglion cell dystrophies (i) Cherry red spots (ii) Pigmentary dystrophies (a) Fundus flavi maculatous (b) Stargards disease (c) Vitelliform dystrophy (d) Dominant drusen Other less common dystrophies are Kandori syndrome and dominant progressive dystrophy. 3. Bruchs membrane dystrophies (a). Sorsby pseudo inflammatory dystrophy (b) Disciform degeneration of macula (not a true dystrophy) (c) Angioid streak Some of the dystrophies, which are altogether different from the above condition also involve vitreous and are known as vitreo retinal dystrophies. They include: 1. Juvenile retinochisis 2. Wagners vitreoretinal dystrophy 3. Familial exudative vitreoretinal dystrophy 4. Goldmann -Favre vitreoretinal dystrophy Out of all the conditions listed above (all are not seen in childhood, very few are seen in first decade), the most common disorder is rod and rod cone dystrophy where rods are affected, predominantly involvement of cone is less marked and of late onset. They are generally known as retinitis pigmentosa and allied conditions. Again the term retinitis is a misnomer because there is no evidence of inflammatory process in the condition. It is better to call the condition as primary neuro sensory dystrophy of the retina or simply primary retinal dystrophy. The conditions in the group can be: 1. Typical retinitis pigmentosa 2. Variation of retinitis pigmentosa 3. Retinitis pigmentosa associated with other ocular disorders - Myopia, chronic simple glaucoma, keratoconus 4. Retinitis pigmentosa with systemic involvement Retinitis pigmentosa54,55,56 This is commonest of all hereditary retinal dystrophies. It is seen among all races with equal distribution all over the world with pockets of large numbers in communities where consanguinity is common. It is seen both in boys as well as girls. Boys are more affected than girls. Women are generally the carrier in X linked diseases. They may be asymptomatic or may have mild symptoms, however fully developed retinitis pigmentosa is not exception in carriers.

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The condition is mostly hereditary, however sporadic cases are also known. Sporadicity is due to sudden mutation of genes. There are about twenty genes that have been blamed for the disease. There are about hundred possible mutations. The sporadic cases are also called isolated cases. They are seen in about 15 to 20 percent cases. There are three modes of inheritance: 1. Autosomal dominant 2. Autosomal recessive 3. x linked Autosomal recessive is the commonest form of transmission followed by autosomal dominant i.e. mildest, with late onset but diagnosed early. The x linked is the least common but worst. It is most disabling and progresses fast. Symptoms 1. Diminished night vision. This is the first symptom developing around ten years of age. It may be seen earlier in some children who may be confused as suffering from vitamin A deficiency and treated as such without any improvement. The night blindness is gradually progressive and becomes worse as the child ages. By third decade the person may be incapacitated in dim light. To begin with the children have prolonged dark adaptation i.e. they take more time to get accustomed to indoor illumination when they come from bright light. 2. Diminished peripheral field. The patient soon becomes aware about his loss of peripheral field. He complains that he has to move his head to see on the periphery. He may bump into others and may find difficulty in negotiating on the road or drive. The end result is greatly reduced peripheral field resulting into a tubular vision as if the patient has been looking down the barrel of a gun. Generally the central part surrounded by scotoma retains good vision for long time. 3. Diminished distant vision. Diminished vision is late to appear unless it is due to myopia which is very common in retinitis pigmentosa. Onset of diminished vision can be due to (i) Macular lesion. Cystoid macular edema, atrophic maculopathy, cellophane maculopathy (ii) Lenticular. Posterior sub capsular cataract is the commonest type of lenticular changes in retinitis pigmentosa. However development of central nuclear sclerosis is also frequent. (iii) Optic nerve. Consecutive optic atrophy is very common in late stage. (iv) Chronic simple glaucoma. Incidence of chronic simple glaucoma is high in retinitis pigmentosa. Field changes of chronic simple glaucoma when superimposed on that of retinitis pigmentosa worsen each other. Signs of retinitis pigmentosa The triad of signs i.e. pigments, attenuation of blood vessels and waxy pale disc are characteristics of retinitis pigmentosa in presence of night blindness.

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1. Pigmentary changes. The pigmentary changes are brought about due to migration of pigment from deeper layer. They start in the mid periphery of the retina. In early stages, they are nothing but mottling of retinal pigment with prominence of chorio capillaries. With passage of time bunches of pigment begin to collect round the veins, then they cover all the vessels. Later the pigments become spider shaped or bone spicule like in shape. In the same eye various shapes and sizes of pigment can be seen. The pigments gradually spread centripetal as well as centrifugal but never reach the posterior pole unless it is central type. Affinity of the pigments for mid periphery is not well understood. Most common argument put forward is paucity of blood supply of the mid periphery. 2. Thinning of retinal blood vessels. Though pigmentary changes are more conspicuous than attenuation vessel, the vessel changes may precede the pigmentary changes. The vessels may become thread like in late stages. 3. Optic atrophy is a late feature. The colour of the disc is waxy pale. It may be associated with other abnormalities like drusen, may have subtle glioma of the disc. 4. Error of refraction. About three fourth of eyes with retinitis pigmentosa have myopia of moderate degree. 5. Vitreous opacities are very common in retinitis pigmentosa, myopia may be partly responsible for it. The opacities increase with age. 6. Macular involvement are more common than previously thought to be. Macular changes are being reported more frequently following fluorescein angiography. It may just be a decreased reflex or as serious as cystoid macular edema. 7. Lenticular changes. Posterior sub capsular opacification is the commonest form of lenticular changes. It may be seen as early as ten to fifteen years of age. The incidence increases loss of central vision due to posterior sub capsular opacification. Posterior polar cataract add to the misery of the patient who already has a very narrow field. 8. Chronic simple glaucoma is seen in a small percentage of cases in third and fourth decade. 9. Other ocular findings areKeratoconus, squint, progressive external ophthalmioplegia and rarely microphthalmos. Variants of a typical retinitis pigmentosa The triad for diagnosis of retinitis pigmentosa are bilateral annular pigmentation in mid periphery, attenuation of retinal vessels, waxy pale disc with diminished vision. In some cases the pigmentation is unilateral or just sectorial. Sometimes night blindness may precede pigmentation or there may be white dots in the periphery with night blindness. In rarer cases the involvement of the posterior pole is disproportionate to peripheral lesion. All these conditions are called atypical form of retinitis pigmentosa. Some authors are of opinion that these cases except those with white dots will culminate into typical retinitis pigmentosa. The common forms are:

438 Sector retinitis pigmentosa

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In this rare form, retinal dystrophy is localised to a sector of the retinal mid periphery. Commonest area being one of the inferior quadrants, generally involvement in both eyes is similar. The pigmentation and attenuation of vessels are same as in typical retinitis pigmentosa. The area of dystrophy being localised, there is no night blindness, field defect corresponds to the area of retina involved. Dark adaptation may be abnormal. ERG is also subnormal. The patient has good central vision. The two common causes of localised pigmentation should be kept in mind before diagnosis of sector retinitis pigmentosa. They are trauma and localised retinal inflammation. The condition does not require any treatment. Central retinitis pigmentosa (Inverse retinitis pigmentosa) In these cases pigmentary changes similar to retinitis pigmentosa occur round the macula. Due to central involvement, these cases are misdiagnosed as cone or choroidal dystrophy. As the central retina is involved, diminished central vision, reduction of vision in bright light, defective colour vision and positive central scotomas are common. On rare occasion the retinal vessels get attenuated and pigmentation spread to the periphery over years. ERG is always subnormal. In adults it should be differentiated from chloroquine maculopathy and other dystrophies of macula. There is no specific treatment. The children may require rehabilitation. Retinitis pigmentosa sine pigmento The patient present with diminished night vision without pigmentation. On examination they have attenuated blood vessels and waxy pale disc. It may develop pigmentation years after onset of night blindness. The ERG is not recordable. Family members may suffer from typical retinitis pigmentosa. Unilateral retinitis pigmentosa Like sector retinitis pigmentosa, there is doubt if unilateral retinitis pigmentosa can exist without involvement of the other eye. The cases that have been reported as unilateral retinitis pigmentosa had abnormal ERG and EOG in the eye with pigmentation. However possibility of trauma, inflammation and vascular anomaly should be excluded. It is possible that the other eye may develop pigmentation later.57 Retinitis punctata albescens This is a condition of diminished vision, has multiple white dots, scattered all over the retina instead of black pigments of typical retinitis pigmentosa. The vessels are thin and the field is constricted like in retinitis pigmentosa. The condition must be differentiated from other causes of white spots on the retina i.e. retinal drusen and asteroid hyalitis. Retinitis pigmentosa associated with systemic disorders The list of systemic disorders that have signs of retinitis pigmentosa are either hereditary or have disturbances of metabolism, is long. They can be divided into: 1. That have predominant skeletal involvementLaurence-Moon-Biedel-Bardet syndrome, Marfans syndrome.

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2. Hearing defect

Refsums syndrome Ushers syndrome Cockaynes disease Alstorm syndrome

3. Renal disease 4. Bone disease 5. Mucopolysaccharidosis 6. Lipidosis

Cystonosis, cystinuria, oxalosis Pagets disease,

7. Spino ponto cerebellar degeneration 8. MiscellaneousProgressive external ophthalmoplegia, myotonic dystrophy. Laurence-Moon-Bardet-Biedle syndrome Obesity in a child is most striking feature, which is present in 90% of cases. On close look, the child may have polydactyly and syndactyly which is obvious at birth and hypogenitalism. These children have low IQ and overall mental retardation. Commonest symptom is night blindness that starts by ten years of age. Soon to this is added diminished central vision due to involvement of macula. Some children may have nystagmus. The fundus picture is similar to retinitis pigmentosa i.e. bone spicule pigments on the periphery, thinning of retinal vessels and waxy pale disc. The field changes, ERG and EOG changes are similar to typical retinitis pigmentosa. Some children may show albipuntate changes. Other systemic disorders include - brachycephaly, short stature that makes these children look more obese, renal disorder, deafness, congenital heart disease. The disease is an autosomal recessive disorder. All children with polydactyly or syndactyly should be observed for unusual weight gain and screened for pigmentation changes in the retina. There is no specific treatment. Rafsums syndrome This is an autosomal recessive disorder. The syndrome consists of peripheral neuropathy, ataxia, night blindness, and atypical pigmentary dystrophy with constricted retinal vessels, poor dark adaptation and sub normal ERG. The pigments are in clumps and look more or less like pepper and salt appearance rather than typical bone spicule. The condition is a disorder of metabolism of fatty acid. Both serum and urine contain phytanic acid. It is claimed that dietary restriction of fatty acid can improve the condition. Other systemic involvements are - Progressive deafness, reduced corneal sensation, loss of hair, icthyosis. There may be conduction defect in the heart that may prove to be cause of sudden death. Ushers syndrome Ushers syndrome is a major cause of genetic hearing defect. It includes neuro sensory deafness, vestibular disturbance and night blindness. Association of neuro sensory defect along with degeneration of pigment may be traced to similarity in development of retinal pigment and the epithelium of organ of Corti. It is an autosomal recessive disorder. Night blindness may start between ten to twenty years of age and progress relentlessly. Associated macular lesion is cause of diminished distant vision. Peripheral field is constricted, there is sub normal ERG, EOG and dark adaptation.

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This is autosomal recessive disorder that has diminished night vision, retinitis pigmentosa, infantile deafness, pre mature old age, dwarfness and mental retardation. Bassen-Kornzweig syndrome (abetalipo proteinemia) It is an autosomal recessive disease seen mostly amongst Jews. The condition is detected in first decade with diminished night vision with pigmentary changes in the retina. RBCs show acanthocytosis. These children generally suffer from malabsorption syndrome. Serum cholesterol and serum beta lipo proteins are less than normal. There may be spinocerebellar ataxia, which progresses slowly and is stabilised in adults. Oral administration of large doses of vitamin E and A are said to be helpful. Causes of retinal pigmentation without dystrophy are: 1. Salt pepper fundus due to congenital rubella, syphilis, influenza, cystinosis, rubeola. 2. Drug induced pigmentationPhenothiazine, chloroquine (macular pigmentation), indomethacin, quinine. 3. TraumaSmall laser burns, cryo spots 4. Congenital melanosis (in clumps) Diagnostic procedure for retinal dystrophy 1. HistoryHistory gives important clue in diagnosis of retinitis pigmentosa. (a) Night blindness. If a child under ten years of age presents with night blindness first is to find out if it is due to vitamin A deficiency or retinal dystrophy. A child with vitamin A deficiency and night blindness invariably has other signs of protein caloric malnutrition, xerosis or even keratomalacia. Such night blindness improves within 24 hours to 72 hours following administration of water soluble injection of vitamin A or within a week following oral administration of vitamin A concentration 200,000 IU on two consecutive days. An adult with night blindness does not improve with vitamin A administration. (b) Positive family history (i) Night blindness. (ii) Proved retinitis pigmentosa among siblings, parents and other blood relatives. (iii) Consanguinity among parents, grand parents. (c) Evidence of other neuro sensory disorders like deafness, ataxia, mental retardation. 2. Field changes (i) Ninety percent of cases with retinitis pigmentosa have ring scotoma, by the time they report with night blindness which is not cured by therapeutic dose of vitamin A. (ii) The field changes usually start as isolated scotoma concentric with equator in the inferior temporal area. This gradually enlarges on either end to become a ring scotoma. Once the ring has been formed, the scotoma expand both anteriorly and backwards leaving only an island of vision.

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3. Dark adaptation. This is a sensitive test second only to ERG to show abnormality even before the fundus findings appear. It shows elevation of rod threshold. The cone threshold may also be raised. 4. Electro retinogram. ERG is almost always sub normal even before the fundus changes develop. Scotopic ERG is more markedly involved than photopic ERG. ERG of the family members may show abnormality without night blindness. Hence this is used to screen members of the family for retinitis pigmentosa. 5. Colour vision defect is more marked in cone dystrophy. 6. Fluorescein angiography. This is not a specific test. It shows thinning of retinal vessels, diffuse hyper fluorescence due to hypertrophy of pigment epithelium. Cone and cone-rod dystrophies These disorders are as common as rod dystrophies but do not have prominent symptoms like night blindness. They are associated with diminished central vision. The subtle cases may be missed or misdiagnosed as amblyopia. The symptoms consist of diminished central vision, poor vision in bright light, photophobia, defective colour vision and nystagmus. The cases are generally sporadic but autosomal dominant and autosomal recessive inheritance is also possible. The conditions are broadly divided into two groups: 1. A rarer form of progressive cone dystrophy which again on the basis of fluorescein pattern have been divided into type I and type II. 2. Commoner form of stationary cone dystrophy. Progressive cone dystrophy The condition is bilateral, first detected in first decade as acquired diminished distant vision, photophobia and nystagmus. On examination the children have severe colour vision defect which may end in achromatopsia in later stages of life. The macula has a bulls eye appearance, which consists of clear central area surrounded by zone of pigmentary changes, which is best seen by fluorescein angiography that have multiple window defects without staining. To begin with, the optic nerve may show temporal pallor which may become pale later. Thinning of retinal vessels also is a late feature. Visual field shows central scotoma. ERGPhotopic ERG is abnormal with small b wave. Scotopic ERG is normal, EOG is generally normal. Stationary cone dystrophy The condition is congenital and non progressive autosomal recessive. The symptoms are similar to progressive cone dystrophy, as far as diminished central vision, abnormal colour vision, nystagmus, photophobia, diminished vision in bright light are concerned. Only difference is that the condition is stationary in nature. The condition is more frequent than progressive form. The colour defect is rodmonochromatism.

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On examination, the retina may look normal with loss of foveal reflex and pigmentary change in the macula. Fluorescein angiogram is normal so is EOG. The dark adaptation and ERG are sub normal. The dark adaptation shows normal rod adaptation and abnormal cone adaptation. In ERG the photopic response is poor. Congenital achromatopsia This is an autosomal recessive disorder of macula without many visible clues. The condition is congenital in nature. It can be rod monochromatopsia (typical) or cone monochromatopia (atypical). The latter is rarer than the former. To these children all colours are but shades of grey. The rod monochromat has diminished central vision, photophobia and nystagmus. Congenital night blindness (stationary) There is early onset of night blindness, associated diminished distant vision. Moderate to high myopia is common. Colour vision is not affected, photopic field is normal. The ERG is sub normal, which is not influenced by mode of inheritance. There are three modes of inheritance i.e. autosomal recessive, autosomal dominant and x linked. The x linked have poorest distant vision. The fundus does not show any change except usual myopic change. The ERG shows normal scotopic a wave and minimal response in scotopic b wave. Ganglion cell dystrophy of retina Cherry red spot Red spot (Cherry red) in the macula is a non specific finding in many retinal conditions mostly due to disturbance of metabolism of specific enzymes. These are generally bilateral, hereditary disorders that have deposition of glycosides of various tissues of the body like central nervous system, besides eye. The fovea is mainly involved. Other non biochemical causes can also cause cherry red spot of macula. Common causes of cherry red spot of macula can be brought about by: 1. Biochemical causes (glycolipids and phospholipid) 2. Macular hole 3. Macular cyst 4. Central retinal artery obstruction 5. Comotio retinae. Cherry red spot is a contrast phenomenon where normal macular vasculature stands out prominently among pale retina. The pallor of the retina is due to deposit of various chemicals in the ganglion cells leading to cell death. Cherry red spot maculopathy Common causes are: Tay-Sachs disease (Infantile, amaurotic, familial idiocy) Batten-Mayou disease Niemann-Pick disease Traumatic Non traumatic

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Sandhoff disease Generalised gangliosidosis Sialidosis Metachromatic leuco dystrophy Most of the above conditions are hereditary, may have family history. Some of them are seen in specific races (Tay-Sachs), starts early in life, have various degrees of visual loss, have multiple systemic involvement. The other type of involvement of macula leads to pigmentation of macula and flecks on retina. They are calledflecked retina syndromes. These disorders are dystrophies of retinal pigment epithelium. The two main causes of flecked retina syndrome are fundus flavimaculatous and Stargardts disease. There is some difference of opinion regarding nomenclature of these conditions. Some consider that Stargardts disease is late manifestation of fundus flavimaculatous because they have similar symptoms, both have same hereditary and age of onset is similar. The main point of differentiation is presence of macular atrophy, which is present in Stargardts disease and absent in fundus flavimaculatous. Fundus flavimaculatous This is an autosomal recessive disorder, is bilaterally symmetrical. The flecks, which are accumulation of acid mucopolysaccharides and lipofucin in the retinal pigment epithelium, develop in the posterior pole. The flecks are small and discrete yellowish white spots with irregular margins. The lesions may be of various shapes i.e. fish like, semi circular or circular. The flecks are known to disappear or absorb leaving areas of atrophy of retinal pigment epithelium. New flecks may appear later so long the flacks are away from the macula, the patient is asymptomatic, the flecks appear on the macula the vision falls and continue to fall gradually, never reaching a level of legal blindness. Involvement of macula may cause poor colour sense. Dark adaptation, ERG and EOG are normal. Fluorescein angiography shows hypo fluorescence in early stage and hyper fluorescence in later stage. The lesions have blurred margin without leak. Stargardts disease58 As mentioned early some authors consider Stargardts disease as a late presentation of fundus flavimaculatous. The characteristics of Stargardts diseases are progressive, bilateral, symmetric loss of vision in young patients who had subnormal central vision due to atrophy of macula. It is autosomal recessive disorder. It is more common in consanguinity. The patient has poor vision in bright light, which he may express as glare or photophobia. Part of the diminished vision is explained on the basis of myopia, which is common error of refraction. In early stages the child is brought with loss of one or two lines on Snellens chart that gradually progresses to 1/60 or less. The children with Stargardts disease have red green colour defect that may terminate into achromatopsia. Diagnosis is on the basis of fundus findings and fluorescein angiography.

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FundusIn initial stages the fundus may look normal with moderate uncorrectable diminished distant vision that may be confused with amblyopia or functional. The first visible sign is loss of foveal reflex. This is followed by bulls eye appearance that has normal retina surrounded by area of de-pigmentation. By this time some flecks may develop in the macula giving a name of varnished macula. This is followed by onset of atrophic changes in macula and the macula has a brownish appearance called beaten bronze appearance. The choroidal vessels may become visible through the atrophic macula. Small flecks may appear round and into the macula. Optic nerve may show temporal pallor. Fluorescein angiography Changes in angiography may be visible before the ophthalmoscopic changes occur. The typical picture in normal retina with zone of hyper fluorescence all round without leak. Dark adaptation and ERG are normal but EOG findings are abnormal. The central field may show relative scotoma. The condition should be differentiated from dominant progressive foveal dystrophy. There is no specific treatment. Differential diagnosis of flecked macula consist of Progressive and stationary albipunctate retinal degeneration and Uyemuras disease all have diminished night vision, abnormal ERG, EOG and dark adaptation as all of them are rod dystrophies with flecks on the retina. Vitelli form macular dystrophy This is autosomal disease, starts between four to ten years of age, may be bilateral but not always simultaneous or symmetric. Mode of presentation is variable. The child may be asymptomatic yet can have a full blown disease. The diagnosis is straight forward, on ophthalmoscopy typical egg yolk appearance of the macula is seen from which it gets its name of vitelli form dystrophy. This is also known as Bests disease. The carriers can be asymptomatic with normal macula but abnormal EOG. Association of hypermetropia, esotropia and amblyopia is common. Symptoms depend upon the stage of the disease. The disease has been divided into pre vitelli form and vitelli form stages, the latter is again divided in to stage of pseudo hypopyon, rupture of cyst and complication of rupture. The pre vitelli form stage is asymptomatic, even fundus changes are absent but EOG done at this stage is abnormal. Suspicion of the disease arises when one eye is already in vitelli form stage. The vitelli form stage consists of diminished central vision. On fundus examination an ill defined yellow spot develops generally over the fovea but may be eccentric. The spot is in the retinal pigment epithelium most probably due to accumulation of lipofuscin forming an orange cyst similar to sunny side up egg yolk that may be 1/3 or half of the optic nerve head. Retinal vessels travel over the cyst. Stage of pseudo hypopyon There is no accumulation of pus. The name is acquired due to settling of the lipofucin at the bottom of the cyst, upper part of the lesion may be absorbed. The pseudo hypopyon blocks the

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fluorescein, the upper part shows hyper fluorescence due to atrophy of the retinal pigment epithelium. It takes about ten to twenty years for this stage to develop, vision is minimally reduced, EOG is abnormal. Stage of rupture of the yolk The cyst changes from sunny side up to appearance of scrambled egg with diminished vision. Stage of complication The complication consist of formation of choroidal neovascularisation, with sudden and profound fall of central vision, there may be vitreous haemorrhage. There may be glial proliferation and formation of a hypertrophic scar. The macula may develop an atrophic patch through which the underlying vasculature may show as pink glow surrounded by white glial tissue. Dominant drusen of the retina This condition should not be confused with more frequent condition i.e. drusen of optic nerve. They are of different etiology, clinical presentation and pathogenesis. In drusen of retina there is deposition of yellowish white material in the posterior pole in retinal pigment epithelium and Bruchs membrane in the macula or round it. There are two types of retinal drusens 1. The primary (familial) 2. The secondary - Secondary to local ocular disease like benign choroidal nevus or systemic conditions. The primary familial drusen is inherited as autosomal dominant disorder. It is a bilateral condition, which becomes evident in second decade of life as yellowish white circular raised area, which may be discrete or may confluent to form large plaque that may get calcified in fifth decade. In early stages the deposits may be confused with other white dots on the retina i.e. hard exudates, fundus albipunctatus, and asteroid hyalopathy. Lesion on the nasal side of the disc is more common in familial type. The drusens in pediatric age group are symptomless and are discovered on routine fundus examination, do not require any treatment. Persons with macular drusen are predisposed towards age related macular degeneration, a major cause of blindness in aged. Peripheral retinal degenerations59 There are many retinal degeneration on the periphery of the retina. Most of them develop after third decade. Some of them predispose rhegmatogenous retinal degeneration, others are diagnosed on routine examination and are asymptomatic. The degenerations are bilateral and may be symmetric. Common peripheral retinal degeneration are: 1. Lattice degeneration 2. Snail track degeneration 3. Cystoid degeneration 4. White with pressure

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6. Peripheral chorio retinal degeneration (paving stone or cobble stone degeneration) 7. Chorio retinal atrophy 8. Retinoschisis All the above conditions are not seen in children though many of them start in childhood. Only peripheral retinal degeneration is seen in childhood is lattice degeneration. Lattice degeneration of the retina Lattice degeneration is the commonest form of peripheral retinal degeneration about eight percent of eyes have asymptomatic lattice degeneration which are discovered on routine examination of the retinal periphery by indirect ophthalmoscope. Commonest site for such patches is lower temporal quadrant near the ora. They are more common in moderately myopic eyes. It is common in Marfans syndrome as well as Ehlers Danlos syndrome and Wagners disease. In about 30% of cases of rhegmatogenous retinal detachment, lattice degeneration may be the predisposing cause. The degeneration starts in about ten years of age as scattered small patches on the periphery. The condition is well established in second decade. There may be family history of retinal detachment. It is equally seen among boys and girls. Fundus picture The lesion may be single or multiple, they are generally parallel with the equator but can be radial, parallel to peripheral blood vessels as in Sticklers syndrome. There may be more than one row of patches of lattice degeneration in the same quadrant. The degeneration derives its name from white lace like pattern caused due to sclerosed vessel in the patch. The area of degeneration is generally a spindle shape patch. The lattice may be associated with snowflakes, white with pressure and hyperplasia of retinal pigment epithelium. Symptom The condition may go without symptoms but may be associated with flashes of light and floaters, which should be taken seriously and eye examined with indirect ophthalmoscope. Retinal holes are common in lattice degeneration, they develop in the substance of the lattice. All holes are not symptomatic, occasionally they are responsible for retinal detachment which is heralded by development of retinal tear. The tear generally develops outside the island of lattice on the posterior border. They are more common in moderate myopes and eyes with acute posterior vitreous detachment. Occasionally latticed part may be seen on the flap of retinal tear. Snail track degeneration There is doubt if this condition is a separate entity or precursor of lattice degeneration or its variation because their position and distribution on the retinal periphery is the same, both are associated with snow flecks. Complications of snail track degeneration are same as lattice degeneration.

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Retinoschisis In retinoschisis, the sensory retina is splits into two layers, in contrast to retinal detachment where the retina separates between the pigment epithelium and sensory retina. In retinoschisis the split occurs between the nuclei of visual cells and bipolar cells. The condition can be divided into juvenile (idiopathic) and senile retinoschisis. (See page 403.) Congenital retinoschisis Congenital retinoschisis is present at birth but not diagnosed unless looked specifically in a neonate, born to parents, either of whom is known to suffer from congenital retinochisis. Inheritance is varied. Commonest inheritance is x linked receive. Generally the condition is diagnosed when the child reaches school age and is more often diagnosed as shallow retinal detachment rather than retino schisis. This is true with other types of retinoschisis as well. Juvenile retinoschisis This is a bilateral, symmetrical condition, the hereditary is same as congenital retino schisis. Some authors feel that juvenile retinoschisis is nothing but late feature of congenital retinoschisis. The lesion starts in the lower temporal quadrant in a hypermetropic eye. The separated inner table balloons inwards, the ballooned layer is almost transparent, with blood vessels. The two separate layers are best seen by +90 D or gonio lens. It may have round holes in it, some times there may be hole in the inner table also. The balloon extends up to ora anteriorly and may involve the macula behind. Management The congenital retinoschisis is generally non progressive, may have fair vision without any surgical intervention. However if the condition progresses to involve the macula or rhegmatogenous retinal detachment develops, then the lesion must be treated by laser photocoagulation and scleral buckling. Retinal detachment60 The term retinal detachment is a misnomer as a clinical diagnosis because whole of the retina is not detached but there is inter retinal separation of the layers and it is better to call it retinal separation. However it is difficult to replace old terminology by new, hence, the term retinal detachment has stayed in clinical practice. The retina develops from the two layers of the optic cup. The outer layer contributes to form the pigment epithelium. The rest of the nine layers i.e. the sensory retina develop form the inner layer of the optic cup. In early stages of embryogenesis, the gap between the two is visible, the gap gradually diminishes and ultimately disappears leaving a potential space which is liable to be opened up through out the life if something could get in to it. What keeps the two layers in contact with each other is not well understood. The two theories that are generally put forward are60: 1. The microvilli of retinal pigment epithelium inter digitate with the outer end of the photo receptors, creating a bond between the two layers.

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2. Acid mucopolysaccharides form biological glue between the photo receptors and the RPE. The other contributory factors are: 1. Presence of semisolid vitreous on the retina, which exerts a force to keep the two layers in apposition. 2. Almost lack of mobility of normal vitreous. 3. Normal intra ocular tension. The factors other than apposed layers of retina that keep this space devoid of fluid are: 1. Active metabolic transport between retinal pigment epithelium and sub retinal fluid 2. Oncotic pressure gradient between sub retinal space and choroid also keep the space free of fluid. The vitreous plays an important role in keeping the retina apposed. So long the vitreous is semi-solid and it does not exert any traction on retina even at places where it is attached i.e. the ora serrata, round the optic nerve, at the fovea and along the major blood vessels. Abnormal vitreo retinal attachments develop over chorio retinal scar. Retinal detachment has been divided into two groups: 1. Rhegmatogenous 2. Non rhegmatogenous (a) Tractional (b) Exudative Rhegmatogenous retinal detachment is most common detachment in children followed by traction. Exudative retinal detachment is least common in children. Rhegmatogenous retinal detachment is a treatable cause of diminished vision and party preventable. The two factors that contribute to the development of rhegmatogenous detachment are: 1. Retinal break (a) Hole. (b) Tear 2. Liquifaction of vitreous Presence of only one of the above rarely leads to retinal detachment. Retinal breaks These are full thickness passages between the vitreous cavity and space between RPE and sensory retina. Retinal breaks may take different shapes with varied size and number. One thing common about retinal breaks is that they predispose retinal detachment.61 The retinal breaks look dark red because the retinal pigment epithelium is exposed. About six percent of eyes have retinal holes without retinal detachment. It develops only in one in seventy such cases62. The circular breaks are called holes while others are called tears. All Retinal breaks

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are almost exclusively seen on the retinal periphery except a macular hole or a traumatic tear. Macular holes, generally do not lead to detach-ment. Macular holes can be full thickness or partial. Macular holes are rare in children. Round holes These are punched out circular holes, size of which vary from pin head to almost as large as the optic disc. There number also vary from single to multiple. When a piece of retina is hinged to one edge of the hole or attached the retracted vitreous near the hole and protrudes in the vitreous, it is called operculated hole. Most of the holes are non operculated, the round holes develop in the area of chorio retinitis or in lattice degeneration. The non operculated holes are also known as atrophic holes. They have low incidence of detachment. Tears according to their shape are called - Horse shoe shaped; giant tear and dialysis. The horse shoe holesThese are crescent shaped tear with concavity towards the periphery. The tongue of the retina towards the tear is called the flap. Sometimes bands arising from the vitreous may be attached to the apex of the flap. They are common in children. They are never as numerous as round holes. Giant tears These are also called limbus parallel tears due to their orientation in relation to limbus. They are slightly posterior to ora serrata and parallel to it. These are the largest tears, generally there is only one giant tear in one eye. The tear extends more than 90 along the circumference, it may rarely extend all round. They are seen in younger patients. In about 80% of giant tears, no predisposing factor is detected. It is common in blunt injury, they can be bilateral and have poor prognosis. Dialysis of retina This is disinsertion of retina at the ora serrata. Commonest site is inferio temporal quadrant, which is most stretched and least developed. Commonest cause is trauma otherwise it can be idiopathic in some cases, they are bilateral. The detachment progresses slowly, as the progression is slow, macular involvement is not felt abruptly. They are more common in young males. Liquefaction of vitreous Normal vitreous is a semisolid gel incapable to flow. The vitreous does not have a formed capsule, still it keeps its shape in the vitreous cavity. It is only liquified vitreous that causes retinal detachment but not unless it has come out of the vitreous cortex. The fluid vitreous when associated with deficiency in the vitreous body flows in between the vitreous body and the retina and fill the newly formed retro hyaloid space. This pushes the vitreous forwards and the condition is called posterior vitreous detachment (P.V.D.). Following PVD the retina looses its support from a stable vitreous. The retina is directly affected by traction of vitreous. The traction may be normal anatomical adhesion or pathological chorio retinal scar. In adults the liquefaction of the vitreous is an ageing process and called synchisis senilis. This does not happen in children. In children the vitreous is liquified following trauma

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more in penetrating injury than blunt injury, chronic inflamation of choroid and retina, retained intra ocular foreign bodies and myopia. In the next stage the fluid vitreous finds its way into the potential space in between the sensory retina and the retinal pigment epithelium and distends the space resulting into a retinal separation or rhegmatogenous detachment. The sub retinal fluid gravitates down to most dependent part and due to this it separates more retinal area from its natural apposition. Hence a hole at 12 oclock is likely to cause more extensive detachment on either side of the hole while a relatively large rent at six oclock detaches smaller area and the progression is slow. Symptoms 1. A shallow detachment at the lower part away from the macula may be symptomless. 2. Two common symptoms of rhegmatogenous retinal detachment are Photopsia (Flashes of light) due to retinal irritation and floaters. The floaters may be due to RBC from the torn blood vessel at the site of tear, or due to liquified vitreous. A ring shaped shadow the Weiss ring denotes posterior vitreous detachment, occasionally a cobweb like opacity may be seen due to condensation of collagen fibres in collapsed vitreous.

3. Metamorphopsia develops in early stages of detachment due to unevenness of the retinal surface. 4. Diminished visionDegree of loss of vision depends on macular involvement. In large detachment with a superior hole, loss of vision may be acute, it may fall from 6/ 6 to hand movement of less. The causes of diminished vision in retinal detachment are: 1. Involvement of macula 2. Overhanging detachment in front of macula 3. Blood in front of macula In large detachment there may be associated loss of projection in the corresponding quadrant. 5. Field changesThe field defects start from the periphery and spread towards the centre. The field defect is brought about by spread of sub retinal fluid behind the equator. The patient perceives the field loss as either a shadow, approaching from the side, or a curtain coming down from above. Lower field defect is generally felt earlier than upper field loss. Signs of retinal detachment are variable. They depend upon site of the detachment, extent of the detachment, duration of detachment. 1. Uncorrectable diminished visionThis may be associated with faulty projection when elicited by small pinpoint light source. 2. Externally the eye is non congested and non tender. 3. PupilIn case of large retinal detachment the pupil may show Marcus Gunn pupil. In small detachments the pupillary reaction may be normal.

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4. Intra ocular tension is lower by 5-6 mm than previously noted IOP. 5. Anterior chamber reaction is common in long standing detachment, may occasionally be present in recent cases. 6. Retinoscopy (a) In case of extensive retinal detachment the retinal glow may be absent. (b) Retinal detachment in the posterior pole may give a grey reflex due to pallor of the detached retina. (c) Hypermetropic shift of refractive error i.e. the pre existing myopic refraction is lessened and hypermetropia increased. 7. Fundus examinationThe clue to diagnosis is in ophthalmoscopic examination of the fundus under maximum mydriasis by indirect ophthalmoscopy, preferably by scleral indentation. Direct ophthalmoscopy may be sufficient to diagnose presence of retinal detachment. It fails to pinpoint the cause i.e. the break, its position, number and size. (i) With direct ophthalmoscope, the detached retina is slightly opaque due to loss of underlying choroidal pattern. The grey surface is convex. The surface in corrugated due to intra retinal edema. The retinal vessels both arteries and veins look darker than in undetached retina. The colour difference between the arteries and veins is less marked. The detached retina may undulate with movement of the eye with out shifting in the fluid. The sub retinal fluid may extend up to the ora serrata. The above findings are better appreciated by indirect ophthalmoscope as it has stereopsis and larger field. (ii) The retinal breaksThe presence of retinal break is the most important sign in rhegmatogenous retinal detachment, both as a diagnostic as well as prognostic feature and mode of treatment to be undertaken. The breaks are generally on the periphery, may vary from solitary hole to multiple holes and breaks. There may be very small or may present as large dialysis. (iii) VitreousVitreous may show large floaters. Posterior vitreous detachment is almost always present. In detachment of recent origin, blood may be present in the subhyaloid space or in the liquefied vitreous. The anterior vitreous may have tobacco dusting. The vitreous may be attached to the operaculum of a hole or flap of a tear. (iv) The periphery besides break may show peripheral degeneration especially lattice degeneration or patches of chorio retinal scar. The fundus finding of long standing retinal detachment differ considerably from that of recent detachment. They are : 1. High-water mark if the retinal detachment is about a month old, pigments are deposited at the border of the detachment which gradually fade. They do not represent edge of sub retinal fluid. They lag behind the edge of sub retinal fluid. 2. Retinal thinning - This looks like retinoschisis. 3. Sub retinal fibrosis- Opaque strands develop on the detached retina.

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4. Intra retinal cysts - If retina remains detached for more than one year, intra retinal cyst may develop that disappear with re attachment of retina. Other investigation that may be required 1. Ultrasonography is indicated in opaque media. 2. Slit lamp biomicroscopy may be required but does not have much advantage over indirect ophthalmoscope. Predisposing factors 1. Males are more predisposed to retinal detachment. 2. Family historyRetinal detachment can be seen in siblings or parents. 3. MyopiaIt is a well known factor that incidence of rhegmatogenous retinal detachment is more in myopia. Incidence of detachment increases with increase in degree of myopia. It is more common in high myopia but low and moderate myopes are not immune. Incidence of rhegmatogenous retinal detachment is higher in myopicaphakics and myopicpseudophakics. Blunt trauma may precipitate detachment more frequently in myopia than in non myopic. Myopic males are more prone to detachment than females. Myopes are more likely to have bilateral retinal detachment. The causes of higher incidence of retinal detachment in myopia are many and may work in combination, they are: (i) The apposition of sensory retina to pigment epithelium is poorer. (ii) Myopes suffer from lattice degeneration more commonly than non myopes. (iii) Myopic retina is more stretched than non myopic retina, hence thin and likely to develop tears. (iv) A genetic factor may also be a predisposing cause. 4. TraumaBoth blunt as well as penetrating injury can cause retinal detachment. Blunt trauma causes more detachment than open globe injury. Most of the time the tear develops at the time of original injury. Any type of break is possible, holes are less common. Out of all tears retinal dialysis is more common that progresses slowly. Liquifaction of vitreous is common following blunt injury so is posterior vitreous detachment. 5. Aphakia (a) Aphakia following intra capsular lens extraction used to be a major cause of senile retinal detachment prior to IOL surgery became popular. However intra capsular lens extraction was never a cause of aphakic detachment in children in whom this surgery was not performed. Traumatic aphakia remains a cause of retinal detachment in children. Spontaneous dislocation of lens in Marfans syndrome is also associated with rhegmatogenous retinal detachment. (b) Detachment following extra capsular lens extraction is less common than intra capsular lens extraction but never free from it. The incidence increases if repeated needling are performed.

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(c) Pseudophakia is not a guarantee against rhegmatogenous retinal detachment. The incidence is higher in children because incidence of PCO is very in high in children that require laser capsulotomy which itself is a cause of retinal detachment. 6. Congenital anomalies - Choroidal coloboma, persistent hyperplastic primary vitreous, congenital pit of the optic nerve head are some of the congenital anomalies that predispose rhegmatogenous retinal detachment. 7. Syndromes associated with rhegmatogenous detachment are - Marfans, Ehler Danlos, Wagner, Pierre-Robinson, Stickler syndrome are frequently associated with rhegmatogenous detachment. Untreated rhegmatogenous retinal detachment may follow any of the following course 1. A small detachment especially with superior hole rapidly expands and may become total. 2.Many a times, lower detachment may not progress and remain so due to formation of line of demarcation. Such detachment can have good anatomical apposition following surgery but visual prognosis is guarded. 3. Small localised detachment have been reported to get reattached over prolonged bed rest only to recur following strain. 4. Most of the untreated detachments develop complicated contract, chronic uveitis, hypotony, rarely phthisis that may terminate in blindness. Differential diagnosis of rhegmatogenous retinal detachment Differential diagnosis consist of two types of disease: 1. Where there is actual retinal detachment, these comprise of non rhegmatogenous retinal detachments (i) Tractional retinal detachment (ii)Exudative retinal detachment 2 Those condition that mimic retinal detachment (i) Congenital and juvenile retinoschisis (ii) Choroidal detachment (iii) Cilio choroidal detachment (iv) Vitreous bands. (v) Dislocated lens. (vi) Parasitic cysts Non rhegmatogenous retinal detachment Non rhegmatogenous retinal detachment may it be tractional or exudative have following common features:

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1. They do not have breaks in retina as a primary cause of detachment. Exudative detachment never develops a break. A tractional detachment can develop a hole due to abnormal traction on the retina. 2. The detachment surface does not have corrugation. 3. The sub retinal fluid is not a liquefied vitreous. 4. There is no hyaluronic acid in sub retinal fluid. Tractional retinal detachment Common cause of tractional retinal detachment in adults are - Proliferative diabetic retinopathy, Eales disease, cataract extraction with incarcerated vitreous. These conditions are not seen in children. The common causes of tractional retinal detachment in children are: 1. Persistent hyper plastic primary vitreous 2. Retinopathy of prematurity 3. Hemoglobinopathy 4. Parsplanitis 5. Intra ocular foreign body 6. Congenital toxoplasmosis 7. Penetrating injury The tractional retinal detachments develop slowly, many a times the primary cause like injury may overshadow and a child may not complain of symptoms especially in unilateral cases. On examination There is no break. The detachment has scalloped border. It does not reach the ora except in parsplanitis, the surface is concave, and the detached retina is peaked at the site of the traction. There is no movement of sub retinal fluid. The elevation is moderate. If breaks develop due to pull of the traction, already existing detachment becomes worse and behaves like a rhegmatogenous detachment, fluorescein angiography does not contribute much in diagnosis. There is no leaking or pooling of dye, occasionally there may be some window defect. Treatment is directed towards reduction of tractional force either by denting the sclera in by way of scleral buckle, scleral resection or scleral encircling band or cutting the traction band. In most of the cases both are required. Exadative retinal detachment The incidence of exudative detachment is for less than rhegmatogenous and tractional detachment in children. The common causes of exudative retinal detachment are: Angiomatosis retinae, Coats disease, retinoblastoma, peripheral uveitis, dysproteinemias, choroidal rupture.

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The characteristics of an exudative retinal detachment areThe surface is convex, the boundaries are convex, neither retinal breaks or traction bands are present. The border does not reach the ora. The sub retinal fluid shifts. The elevation of detached retina is moderate, on fluorescein angiography the sub retinal fluid is tinged by the dye but there is no leak. There is always evidence of primary disease. The condition waxes and wanes, may either spontaneously disappear or retina gets attached with treatment of primary cause. Ultrasonography may show a sub retinal growth, generally a tumour. In case of sub retinal tumour the retina is stretched over the tumour with exudate round the tumour. There may be a tuft of blood vessel on the retinal highest spot of elevation. Such elevations in the past were referred to as solid detachment. Management of rhegmatogenous retinal treatment There is no medical treatment for rhegmatogenous retinal detachment, prolonged bed rest in particular position, prolonged patching of two eyes, use of pinholes and stenopic slits all have proved to be futile. Prophylaxis All children with family history of rhegmatogenous retinal detachment, especially if they have myopia and all children who have sustained closed or open globe injury with history of floaters and photopsia, should be examined by indirect ophthalmoscope with scleral indentation. The examination should be repeated every six month for next few years and if a hole or tear is found, it should be closed adequately by laser or cryo. Some of them may be large enough to be closed by local scleral explant. Some of the points to remember are: 1. A tear is more likely to produce retinal detachment than a hole in the same location. 2. Larger breaks produce more detachment than smaller but size of break is not always proportionate to its ability to produce detachment. 3. Superior breaks produce early and more extensive detachment. 4. Lower breaks produce less and shallow detachment. 5. Breaks behind the ora are more likely to cause detachment than breaks at ora. 6. Breaks with vitreous band attached to its flap are more likely to enlarge in size and cause larger detachment. Such breaks are more difficult to manage than without traction band. 7. All breaks specially holes do not require any prophylaxis. They must be observed regularly. The common conditions that do not require prophylaxis are - Breaks surrounded by pigment all round, small holes near the ora serrata, white without pressure, retinoschisis. Surgical treatment Surgical treatment is the specific treatment of all rhegmatogenous retinal detachment. The outline of the principle of treatment consists of 1. Searching and localising each and every retinal break by indirect ophthalmoscope and scleral indentation.

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2. All breaks associated with detachment should be closed irrespective of their shape, size, number, by the following, either alone or in combination. (i) Cryo coagulation, photo coagulationDiathermy has mostly been given up in favour of the former two. (ii) Scleral indentation either by an explant or rarely by implant. The explants are either localised plombs or large encircling bands. Sometimes local implants may be needed to be put over the large hole under the encircling band. (iii) Drainage of sub retinal fluid is indicated in cases of longstanding retinal detachment, immobile detachment, inferior tears. In some instances a break that was not visible earlier become visible following drainage of sub retinal fluid. (iv) Internal temponade byAir, balanced salt solution and silicone oil. (v) Parsplana vitrectomyIn rhegmatogenous retinal detachment, parsplana vitrectomy is required in giant tear, traction band, for gas fluid exchange.

DISEASES OF VITREOUS
Anatomy of the vitreous63,64,65 Vitreous is a semisolid transparent gel that constitutes the largest intra ocular structure. Even few decades ago it was considered to be of little significance except that it filled a large space, kept the retina in place with optical properties, and when disturbed led to unpleasant events of redness, pain, lacrimation and loss of vision. With better understanding of vitreo retinal disorders, it is clear that vitreous plays an important role in retino vitreal and retino vascular disorders. The vitreous fills the vitreous cavity. In adults, it measures about 4 CC in volume in normal eye. It is surrounded by the posterior lens capsule, zonules, the ciliary epithelium, inter limiting membrane of the retina and the optic nerve. The main function of the vitreous is optical Its refractive index is 1.336. The other function is to stabilise the retina to which it is loosely attached without any potential space. The vitreous is attached to various intra ocular structures. The attachments are: 1. Vitreous baseThis is the strongest of all physiological attachments. The attachment of the vitreous is strongest at ora serrata. The area attached over the ora is called vitreous base, which spans over the ora serrata about 2 mm in front and equal distance behind. The vitreous base is attached to the ciliary epithelium and blood vessels at the ora. The basal attachment lasts life long and if detached following trauma, it takes the pigment epithelium and the blood vessels alongwith. The posterior vitreous does not have firm attachment, hence is capable of detachment. Posterior vitreous detachment (PVD) is a common occurrence. 2. Round the foveaThis is a smaller, localised attachment, it is weaker than other attachments. 3. Round the optic nerveThis is fairly stronger attachment.

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4. Round the retinal blood vesselsThis is controversial, so are the weak attachments to the internal limiting membrane of the retina. 5. Hyaloid capsular attachmentIn childhood the posterior lens capsule has a very strong attachment with the anterior vitreous membrane. The attachment is in the form of a ring to a natural depression in the anterior vitreous face. This depression is called patellar fossa or Bergers space. The adhesion is so strong under 25 years of age that traction on the lens fails to pull the lens out in toto, thus making intra capsular lens extraction impossible under twenty five years. The zonules, which are known as tertiary vitreous, are also strong enough to make intra capsular lens extraction impossible. The adhesion weakens with age and by sixth decade it become non existent. In intrauterine life, the hyaloid artery traverses from the optic nerve head to the posterior part of the lens in the middle of the vitreous to form the tunica vasculosa lentis that supplies blood to the developing lens, through a space called Coloquets canal that disappears at birth leaving a narrow empty space which is straight at birth but becomes curved with age. It is not visible with direct ophthalmoscope. If some part of the hyaloid system has not been absorbed completely it is visible as fixed opacity in the mid vitreous without any symptoms, that may be visible as a black dot during retinoscopy. Pathological adhesion of the vitreous These are mostly vitreo-retinal attachment. They are found in chorio retinal scar, peripheral pigment clumps, posterior border of lattice degeneration, borders of choroidal rupture and borders of coloboma of the choroid, peripheral retinal vessels, site of retinal periphlebitis. The shape of the vitreous body follows the shape of the globe. Its volume is less in microphthalmos. It is not capable of expanding unless something i.e. air or fluid is injected into its substance. However administration of hypertonic solutions orally or intravenous and carbonic anhydrase may shrink its volume considerably. Constant pressure on the globe forces fluid out of the vitreous and the vitreous shrinks. These two methods are employed to reduce vitreous volume during cataract surgery. Vitreous once removed from the eye is not capable of replenishment. The space is occupied by aqueous. The vitreous does not have a true capsule. The peripheral vitreous due to its compact collagen content is arbitrarily called cortex while the central part is called nucleus. The vitreous is devoid of any nerve, hence it is not capable of any sensation. It has no blood supply so it is also not capable of any inflamation. However, vitreous acts as a rich medium for growth of bacteria and fungi. The composition of vitreous Vitreous is 98-99% water to which other solids are dissolved. The solid constituents are Collagen, hyaluronic acid and soluble proteins. Vitreous has more ascorbic acid than in plasma. It also containsSodium, potassium, bicarbonates, chloride and phosphates. There are about

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fifty enzymes in vitreous, the functions of which are not clear. The collagen is capable of binding with water, which could be as much as 200 times the weight of the collagen, to form a hydro gel of highest viscosity. Development of vitreous16,17,18 Developmentally vitreous has three phases: 1. Primary vitreous 2. Secondary vitreous 3. Tertiary vitreous Developmentally vitreous has dual origin it is partly ectodermal and partly mesodermal. The primary vitreous starts developing very early and its development is completed by 13 mm stage. It develops in the space between the lens and the retina. During first month of development cells from the lens and retina form the primary vitreous. The retinal cells dominate over the lenticular cells. The cells from the lens loose their contact from the primary vitreous due to formation of lens capsule. The cells from the retina continue to contribute towards development of primary vitreous. The lens is further isolated from the vitreous by developing tunica vesculosa lentis. The tunica vasculosa lentis is mesodermal part of primary vitreous. The hyaloid artery, which is a branch of developing internal carotid, (actually primitive dorsal ophthalmic artery) enters the optic cup through the embrayonic fissure. The hyaloid artery communicates with the capillaries on the ventral aspect of the developing lens to form the tunica vesculosa lentis. The hyaloid system starts disappearing by 65 mm and at birth there is no evidence of the hyaloid artery only the proximal part of the hyaloid artery remains patent and forms the central artery of the retina. The secondary vitreous The secondary vitreous is the part of the developing vitreous, which lasts for rest of the life. It is avascular and is secreted by the cells from the inner surface of the optic cup and pushes the primary vitreous centrally. The primary vitreous gradually disappears leaving no trace except the Coloquet canal. The tertiary vitreous The tertiary vitreous is secreted by the ciliary epithelium. This occupies a triangular area between the developing ciliary body and the lens. With the development of the eye, the ciliary body that was very near to the lens, withdraws away from the lens and the fibres become more clearly defined. The fibres divide into two groups - The anterior part in just behind the iris and gets attached to the anterior lens capsule, the posterior part runs parallel to the anterior vitreous membrane and get attached to the posterior lens capsule. These fibers, ultimately form the zonules of the lens. Congenital anomalies of the vitreous In spite of being most voluminous intra ocular structure, the congenital anomalies of the vitreous are few. The congenital anomalies directly correspond to the stage of development of the vitreous.

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The congenital anomalies may be as small and insignificant as vitreous floaters or as extensive as to replace the whole of the vitreous by mesodermal tissue. The former is very common while the latter is very rare. The congenital anomalies mostly involve the mesodermal tissue. They can be: 1. Limited to hyaloid vascular system without hyperplastic changes in vitreous or, 2. They may be associated with hyperplastic changes in the vitreous. Anomalies of hyaloid vascular system There are three possibilities: 1. The whole of the artery may be seen to exist between the posterior lens capsule to optic nerve head. The vessel is attenuated, may contain blood. This is very rare. 2. The anterior end of the artery may persist as a free floating stalk attached to the posterior lens capsule. 3. The posterior end may remain attached to the optic nerve head. 4. Occasionally the remnants of the posterior part of the tunica vasculosa lentis may persist as grey opacity in the posterior lens capsule and is called Mittndrof dot. This is almost asymptomatic, may be visible on oblique illumination, look like a black dot in pink retinoscopy glow, seen best by slit lamp. 5. The remnants of the posterior part of the hyaloid artery is known as Bergmeisters papilla. This too is asymptomatic but when large may be mistaken as growth on the optic nerve. The papilla is made up of central core of vessel surrounded by fibro glial tissue. Persistent hyper plastic primary vitreous (see chapter Retinoblastoma) Symptoms of diseases of vitreous Diseases of vitreous are rare in children as compared to adults. The two functions of the vitreous are optical and support to the retina, one or both may be involved. Moreover diseases of surrounding structure may also involve the vitreous i.e. the retinal vessels may bleed to cause bleeding in the vitreous. The vitreous being avascular is not capable of bleeding. The vitreous itself is not capable of inflamation either, but is generally involved in inflamation of choroid or retina that may be either in the form of endophthalmitis or vitritis. Vitritis is a condition where inflammatory cells from choroid, retina or optic nerve invade the vitreous without exudation. Commonest symptom of vitreous disorder is vitreous floaters. Other symptoms are - photopsia, a visible ring shaped opacity, erythropsia, diminished vision, sudden painless loss of vision. The symptoms may be unilateral or bilateral. The vitreous is devoid of any nerve supply, hence disease of the vitreous alone is painless. Signs of vitreous disorders The sings comprise of

460 1. Diminished vision. The vitrial causes of diminished vision are: (i) Large opacities in front of the macula

PEDIATRIC OPHTHALMOLOGY

(ii) Haemorrhage in front of the maculaDiminished vision may range from loss of one to two lines on Snellens chart or the vision may be reduced to perception of light. Disorder of vitreous never causes loss of perception or faulty projection. 2. Oblique illuminationOnly a few physical findings of vitreous can be seen on oblique illumination. They are: (i) Retrolental white mass (ii) Strands of liquid anterior vitreous (iii) Less commonly a bloch of fresh haemorrhage can be seen just behind the lens or in the lower part of the vitreous, when the patient looks down with dilated pupil. 3. Slit lamp biomicroscopyOrdinary biomicroscopy can not focus the whole depth of the vitreous unless the optics of the eye is modified to bring the various depths of vitreous within the focus of the slit lamp biomicroscope. This can be brought about by (i) Goldman three-mirror contact lensIts central part brings the posterior pole in focus. The mirror with an angle of 55 focuses the vitreous up to ora serrata, while the mirror with 67 angulation can focus the peripheral fundus. (ii) Hruby lens this is a pre set concave lens of 55D (iii) El Bayadi lensThis is a pre set convex lens of + 55D. Beside these there are other lenses i.e. + 90D or + 78D that are also used to view the vitreous with slit lamp. 4. OphthalmoscopyBoth direct and indirect ophthalmoscopes are used to examine the fundus. Indirect ophthalmoscope has added advantage of stereopsis, larger field and brighter illumination. 5. UltrasonographyBoth A and B scans are used. B scan gives better view than A scan. Both are useful in opaque media. 6. X-ray. It is indicated only when intra ocular foreign body is suspected. Both AP and true lateral view should be taken. 7. ElectroretinographyERG has limited role in transparent media. In opaque media, a non recordable ERG is not an absolute contra indication for vitreous surgery because a totally detached retina that gives non recordable ERG on successful attachment may gain some visual function. 8. Fluorescein angiography is indicated in proliferative vascular retinopathy and cystoid macular edema that is common following vitreous loss. Vitreous opacities (floaters) Vitreous opacities are commonest symptom of disease of vitreous. They cause loss of transparency of the vitreous, may be in the form of scattered dots, strings, sheets. They are generally mobile, hence called floaters. They may change their position in space in different

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direction of movement, may be felt all through the waking hours or may be more visible after sleep. They are not visible in darkness or when the eyes are closed. Vitreous opacities can result due to 1. Primary change in the vitreous 2. Secondary changes in the vitreous due to pathology in retina, choroid, and optic nerve. Primary changes in vitreous Muscae volitantes These opacities are so common that they are considered to be physiological. They represent remnants of primary vitreous. They can be unilateral or bilateral, are not associated with loss of vision. They are perceived either as scattered dots or dots in string. They are best seen against light coloured background such as sky or white wall. They have no pathological significance nor do they require any treatment. Sudden onset of shower of vitreous floaters should not be taken lightly. All such eyes should be examined with indirect ophthalmoscope, as it may be the beginning of parsplanitis, retinal break or inflammation of the uvea. Floaters are common in moderate to high myopia due to degenerative change in the vitreous. Vitreous degeneration Vitreous is a semisolid gel, does not show any movement or ripple within its body unless it is liquefied. Vitreous in a childs eye is more firm and solid than vitreous in senile group. The liquefication of the vitreous is known as synchisis. It is a common feature of old age. In children liquefication is seen in high myopia and trauma with or without retained foreign body. It is also seen following vitreous haemorrhage, endophthalmitis and severe posterior uveitis. The vitreous looses its semisolid nature and becomes watery in consistency. It may be so fluid as to flow out during intra capsular lens extraction in age related cataract. The liquification invariably leads to syneresis or collapse of vitreous. Liquified vitreous does not require any specific treatment. Synchisis scintillans This is a different kind of liquifaction of vitreous seen in younger patients, is bilateral without much complains. The patient may not be aware of its presence. There is deposition of cholesterol crystals in the vitreous. The crystals are suspended in the vitreous. They may remain hidden at the bottom of the vitreous and become visible on movement of the eye like golden shower. Sometimes they are visible on oblique illumination in clear media. They are best seen by slit lamp. They are also visible with direct ophthalmoscope. On retinoscopy they may be visible as cloudy dots. Synchisis scintillans does not require any treatment and persist life long. They are not to be confused with astroid hyalitis, which is seen in elderly males. They are accumulation of calcium soaps and are fixed bodies, show minimum movement. Vitreous detachment Normal vitreous does not detach from its attachment. For a vitreous to detach its consistency must change towards fluid rather than semi solid gel. It is more common after sixth decade but not exceptional under twenty. The common causes of vitreous detachment in

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children areMyopia, chronic uveitis, trauma, both concussion and penetrating, vitreous haemorrhage, vitreous disturbance during intra ocular surgery. According to configuration of the detachment, it has been divided into two broad groups: 1. Posterior vitreous detachment (P.V.D.) 2. Anterior vitreous detachment. The posterior vitreous detachment is more common than anterior vitreous detachment. Posterior vitreous detachment can be 1. Small peripheral, which may later increases in size. 2. Infundibular or conical detachmentWhen the vitreous is attached round the disc and base but separated from internal limiting layer of the retina all round. 3. Globular detachmentWhen the attachment round the disc has been severed but the basal attachment is maintained. The posterior vitreous detachment can be simple or with collapse. In simple PVD, the detached posterior surface retains its spherical shape. In detachment with collapse, the detached posterior border of the vitreous hangs vertically from the region of its superior base. Anterior vitreous detachment The whole of the anterior vitreous cortex is separated from the lens, zonule and base, pushing the vitreous posteriorly. It can be localised to zonule or lens or both. Symptoms of vitreous detachment are variable. It may be symptomless. Common symptoms are photophobia, and floaters in front of the eye or a Weiss ring, which is an annular vitreous floater due to detachment of posterior vitreous from the optic nerve head. There is no specific treatment for vitreous detachment. All cases of vitreous detachment should be examined by indirect ophthalmoscope for possibility of peripheral retinal degeneration and holes, which may require treatment to prevent rhegmatogenous retinal detachment. Vitreous haemorrhage Vitreous haemorrhage is in fact an inter vitreal bleeding originating from retinal vessels. Vitreous being avascular, is incapable of bleeding. Vitreous haemorrhage is a common causes of painless, sudden loss of vision which when unilateral may go unnoticed in children. If the cause of the bleeding is local, the bleeding is unilateral. It is bilateral if the cause is systemic. Vitreous haemorrhage is less common in children. The commonest cause of bleeding in third and fourth decade is periphlebitis (Eales disease) which in not seen under fifteen years of age. In fourth decade onwards incidence of diabetics proliferative retinopathy and venous obstruction, which are again not seen in children are the main causes of vitreous haemorrhage. However, proliferative retinopathy in juvenile diabetes may on occasion present as vitreous haemorrhage. The common causes of vitreous haemorrhage in children are: 1. CongenitalPersistent primary hyper plastic vitreous, angiomatosis, Coats disease, remnants of hyaloid vessels.

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2. TraumaBy far trauma is the commonest cause of vitreous haemorrhage in children. It can be blunt injury or may be penetrating injury. Blast injuries may cause bilateral vitreous haemorrhage. Rupture of retinal vessels due to vitreo retinal traction, a vessel passing across a large tear can also bleed. 3. Blood dyscrasiaLeukaemia, purpura and other coagulation disorders can cause vitreous haemorrhage. 4. InflammationPeriphlebitis is commonest cause of vitreous haemorrhage in third and fourth decade and parsplanitis is less common cause in that group. Parsplanitis is more likely to cause haemorrhage in children than periphlebitis. Miscellaneous 1. Tersons syndrome, where a sub arachnoid haemorrhage near the optic disc may trickle down to sub hyaloid space. 2. Retinopathy of prematurity 3. Inadvertent perforation a globe during squint, retinal surgery, retro bulbar or peri bulbar injection can also cause vitreous haemorrhage. Symptoms of vitreous haemorrhage A bleeding away from the posterior pole may be asymptomatic and go unnoticed. Commonest symptom of a pre macular haemorrhage is sudden, painless loss of vision that may vary between loss of one to two times on Snellens chart to only perception of light. The patient may have better vision as the blood settles down only to be reduced when the blood comes in front of the macula. One rare occasions, small haemorrhage may present as showers of floaters. In still rarer conditions there may be erythropsia. Signs of vitreous haemorrhage On rare occasions fresh blood in anterior vitreous may be visible on oblique illumination. Commonest form of presentation is a keel shaped sub hyaloid haemorrhage that has red colour with round bottom with a horizontal line denoting the upper limit of settled blood. A massive intra vitrial haemorrhage may obscure the fundus when seen by direct ophthalmoscope. The same when viewed by indirect ophthalmoscope makes it possible to see the retinal periphery that may have the clue to the haemorrhage i.e. tear, degeneration, disinsertion, parsplanitis. Fate of vitreous haemorrhage Vitreous haemorrhage is a self-limiting disease. In four to six weeks the haemorrhage is absorbed without leaving trace of haemorrhage. However, recurrence of bleeding takes more time to absorb than original bleeding. Each episode of bleeding lasts longer than the previous one. Recurrent and non absorbing haemorrhage may lead to formation of vitreous bands and are called retinitis proliferans. It generally starts near the disc where the central retinal artery is rich in mesoblastic tissue. If haemorrhage does not clear within six months the condition

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is called hemophthalmos where no part of the retina is visible on ophthalmoscopy, retinoscopy or slit lamp examination. The only useful investigation in such cases is ocular ultrasonography that may show retinal detachment as well. Management of vitreous haemorrhage There is no specific treatment for vitreous haemorrhage. Most of the vitreous haemorrhage get absorbed by four to six weeks. Some of the new vessels may require closure by laser or cryocoagulation. Vitreous haemorrhage not clearing by three months require parsplana vitrectomy, some may require additional endo laser coagulation. Associated retinal detachment is treated by standard methods. Vitreo retinal degeneration66,67,68,69 These are a group of inherited conditions that manifest in childhood due to close proximity of retina and lens during development of the vitreous. The vitreous is seldom involved alone in these degenerative conditions. The condition can be divided in two broad groups: 1. Those that have only ocular involvement 2. Those that have associated systemic involvement along with vitreo retinal degenerations: The group I consist of Stickler syndrome. Wagners disease. Congenital retinoschisis Favre Goldmann syndrome Exudative vitreo retinopathy Snow flake degeneration The group II Consist of Marfans syndrome. Homocystinuria. Ehlers-Danlos syndrome Lawrence-Moon-Biedl syndrome. Most of the conditions in-group I have predominant ocular involvement but may have systemic involvement as well especially in Stickler syndrome. Stickler syndrome It is an autosomal dominant disorder, the children may have skeletal changes similar to Marfans syndrome. Joint involvement is common, the ocular change consist of vitreo retinal degeneration that consist of liquified vitreous with collapse giving an optically empty vitreous. Translucent membrane may develop on the periphery. The retinal changes include lattice degeneration. There may be sheathing of peripheral retinal vessels. Peripheral retina

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shows clumps of vitreo retinal scar. Retinal detachment is common. There is mild to moderate degree of myopia. Cataract and open angle glaucoma are common complications. Wagners disease This is as an autosomal dominant inheritance. The ocular structures involved besides vitreous are retina, optic nerve and lens. The vitreous changes are similar to those seen in Sticklers disease without retinal detachment. There may be diminished night vision with abnormal ERG. The disease is generally bilateral, starts in infancy and continues in adolescence. Liquification of vitreous is seen after second decade. Congenital retinoschisis (see retina also page 403) It is also known as juvenile retinochisis, though the retinochisis is a dominant feature both vitreous and retina are involved simultaneously. It is commonly seen in childhood, more common in boys. It is known to be present at birth. The children may be brought to ophthalmologist for presence of strabismus and/or nystagmus and on examination found to have congenital retinochisis. The condition grows slowly, there may be periods of worsening in between the main fundus finding consists of retinochisis. The inner layer of retinochisis is extremely thin, the blood vessels on the inner leaf are normal. The inner leaf has large hole resulting into smooth ballooning of immobile inner leaf. The outer layer is difficult to distinguish until it is detached from the RPE, which happens when the outer layer also develops hole. There may be some haemorrhage in the vitreous. The posterior border of schisis may show line of pigmentary demarcation. The retinoschisis may remain on the periphery or may extend into the macula. The maculopathy consists of small cystoid spaces with radial lines, which probably represent folds in the internal limiting membrane. The appearance is called bicycle-wheel pattern, which does not fluorescence. The Cloquets canal seems to be larger, the vision is diminished if either the macula is involved or there is retinal detachment. The condition has x linked inheritance. It is bilateral and ERG shows subnormal B wave with normal A wave. Management There is no specific treatment that may give uniformly satisfactory results. The modalites tried arePhoto coagulation of outer layer this may regress the condition and the balloon may flatten. Photo coagulation is more indicated if there is a hole in the outer leaf. Retinal detachment when present, it is treated by standard retinal detachment surgery. FavreGoldmann syndrome This is a vitreo retinal degeneration that has night blindness as main symptom. It is an autosomal recessive disease. It affects boys and girls equally, it is generally bilateral. The vitreous is liquified, may have peri retinal membrane. Retinochisis and retinal detachment are seen. The other symptoms are diminished vision, floaters, ERG is sub normal or absent. Cataract is a common complication. There is no specific treatment. Familial exudative vitreo retinopathy This autosomal dominant disorder is also known as Criswick-Schepen Syndrome. The ocular involvement is generally bilateral and symmetrical. The condition can be present in two forms:

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1. The child is asymptomatic and the condition is detected on routine ophthalmoscopy. 2. The child is brought with squint and found to have diminished vision. The fundus findings are less in the first group that includes peripheral avascular zone, arterio venous anastomosis, vitreo retinal adhesion. The condition may be mistaken as retinopathy of prematurity. In second groupThe vitreo retinal findings are more extensive and have more similarity to retinopathy of prematurity. This includes retinal and pre retinal haemorrhage, exudation, fibro vascular bands, retinal fold, retinal detachment. The children have moderate myopia, the retina may show white with pressure or without pressure. There is no specific treatment. Snow flake degeneration This is rarer than those described above. It is an autosomal dominant disorder that has myopia and pre senile cataract for which the patient may seek help. On examination it is found to have following: Wide spread areas of white with pressure that may have snow flake deposits. Posterior to snow flakes there may be increased pigment deposit. The peripheral retinal vessels show sheathing, there may be breaks in the retina leading to detachment. Lebers congenital amaurosis70 This is one of the frequent causes of blindness in new born. The child may be blind from birth or goes blind during first year. Poor vision from early months predispose nystagmus. Poor vision and nystagmus herald sttrabismus. Parents of these children with squint and/or nystagmus bring the child to the ophthalmologist to know if the child has vision or not71. Unfortunately they are told that the child has very poor uncorrectable vision. The child develops a peculiar habit of rubbing the eyes. The condition is autosomal recessive in nature. Fundus findings are variable, initially the fundi may look normal or may have pepper and salt like pigmentation all over the retina. Later they may develop bone spicule pigmentation similar to retinitis pigmentosa, which is not seen in such a young child. These children generally have high hypermetropia. The pupillary reaction may be sluggish or paradoxical. ERG both photopic and scotopic are subnormal. The patients may have keratoconus, keratoglobus, and cataract. Mental retardation is common.

REFERENCES
1. Duke Elder S. ; Layers of retina in System of Ophthalmology. Vol. II, pp. 230231, Henry Kimpton, London 1961. 2. Nancy Anderson Hamming and D. Apple ; Sensory retina in Principles and practice of Ophthalmology. Vol. I, first Indian edition, pp. 4758. Edited by Peyman G.A., Sanders D.R. and Goldberg M.F. Jay Pee brothers, New Delhi 1987.

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3. Dutta L.C. ; Anatomy of retina in Ophthalmology, principle and practice. First edition, pp. 142145. Current Book International, Calcutta 1995. 4. Inderbir Singh ; Visual path way in Text book of human neuro anatomy. Sixth edition, pp. 229231. Jay Pee brothers, New Delhi 2002. 5. Nema H.V. ; The retina in Anatomy of the eye and its adnexa. Second edition, pp. 3951, Jay Pee brothers, New Delhi 1991. 6. Banumathy S.D. ; Anatomy of the visual path way in Anatomy of the eye. pp. 6568, Arvind Eye hospital, Maduri. 7. Kumas A. and Nainiwal S.K. ; Macular and sub macular surgery in Clinical practice in Ophthalmology. First edition, pp. 430, Edited by Saxena S., Jay Pee Brothers, New Delhi 2003. 8. Khamas B. ; Anatomy retina and vitreous in Modern Ophthalmology. Vol. II, Second edition, pp. 624629, Edited by Dutta L.C., Jay Pee Brothers, New Delhi 2000. 9. Samant P. M., Hemalini. P. Samant and Nabar S.M. ; Structures of fovea centralis in Ophthalmology theory and practical. First edition, pp. 164165, Bhalani Publishing House, Mumbai 2004. 10. Miller S.J.H. ; Retina in Parsons disease of the eye. Seventeenth edition, pp. 911, Churchill Livingstone, London 1984. 11. Dutta L.C. ; Structure of the retina in Ophthalmology principles and practice. first edition, pp. 143150, Current books international, Calcutta 1995. 12. Duke Elder S. ; Retinal circulation in System of Ophthalmology, Vol. II, pp. 363377, Henry Kimpton, London 1961. 13. Seal S.K. ; Blood retinal barrier in G.N. Seals text book of Ophthalmology. Fifth edition, pp. 293, Current Books International, Kolkata 2002. 14. Dutta L.C. ; Capillaries of retina in Ophthalmology principle and practice. First edition, pp. 149157, Current books International, Calcutta 1995. 15. B. Khamar ; Blood supply of retina in Modern Ophthalmology. Vol. II, second edition, pp. 624622, edited by Dutta L.C. Jay Pee brothers, New Delhi 2000. 16. Ahmed E. ; Embryology of neuro ectodermal Structures in A text book of Ophthalmology. First edition, pp. 4344, Oxford University Press, Calcutta 1983. 16A. Duke Elder S. ; Differentiation of neural ectoderm in System of ophthalmology Vol. III part I pp. 83, Heury Kimpton London, 1963. 17. Nema H.V. ; The vitreous body in Anatomy of the eye and adnexa. Second edition, pp-137138, Jay Pee brothers, New Delhi 1990. 18. Jay B. Carrot W. ; Albinism Recent advances. Tran. Ophth. Soc., UK., pp. 100467, 1980. 19. Dutta L.C. ; Albinism in Ophthalmology principle and practice. 1st Edition, pp. 476 478, Current Books International, Calcutta 1993. 20. Boger W.P., Peterson R.A. ; Albinism in Manual of ocular diagnosis and therapy. Third edition, pp. 273, Edited by Deborah Pavan Langston, Lippincot William and Wilkins, Philadelphia 1991.

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21. Duke Elder S. ; Congenital abnormalities of the inner layer of the optic vesicle in System of Ophthalmology. Vol. III, part 2, pp. 628633, Henry Kimpton, London 1964. 22. Duke Elder S. and Wybar K.C. ; Ciliary circulation in System of Ophthalmology. Vol. II, pp. 345346, Henry Kimpton, London 1961. 23. Mann I. ; Developmental anomalies of the eye. Second edition, 1957. Quoted by Duke elder in System of Ophthalmology. Vol. III, part II, pp. 614. 24. Kini. M.D. ; Retinal vascular anomalies in Manual of ocular diagnosis and therapy. Third edition, Edited by Deborah Pavan Langston, pp. 152153, Little Brown, 1991. 25. Tasman W. ; Retinal telangiectasis (Coats disease) in Pediatric Ophthalmology. Vol. I, Edition two, pp. 612614, Edited by Harley R.D., W. B. Saunders Company, Philadelphia, 1983. 26. Haller. J. ; Coats disease in Retina. Vol. II, edition 2, pp. 14431460, Edited by Ryan S. J., Mosby, St Louis, 1994. 27. Reese A.B. ; Telangiectasia of retina and Coats disease. A.J.O. : 42-1, 1956. 28. Wu Lihteh. ; Coats disease in Current ocular therapy. Fifth edition, pp. 607608, Edited by Fraunfelder F.H. and Roy H.F., W.B. Saunders Company Philadelphia 2000. 29. Asdourian G. ; Vascular anomalies of the retina in Principle and practice of Ophthalmology. Vol. II, First Indian edition, pp. 12991306, Edited by Peyman G.A., Sander D.R., Goldberg M.F., Jay Pee brothers, New Delhi 1987. 30. Kanski J.J. ; Retinal capillary hemangioma in Clinical Ophthalmology. Second edition, pp. 407405, Butterworth, London 1989. 31. Aaberg T. M. ; Fluorescein angiography in Principle and practice of Ophthalmology. Vol. II, first Indian edition, pp. 910911, Edited by Peyman G.A., Sanders D.R., Goldberg M.F., Jay Pee brothers, New Delhi 1987. 32. Kumar A, Nainiwal S.K. ; Macular and sub macular surgery in Clinical practice in Ophthalmology. pp. 440441, Edited by Saxenas, Jay Pee brothers, New Delhi 2003. 33. Harmohina Bagga, Chandra Sekhar G. ; Optical coherence tomography in Clinical practice in Ophthalmology. pp. 122, Edited by Saxena S., Jay Pee Brothers, New Delhi 2003. 34. Saxena S., Nancy M. Holekamp ; Idiopathic macular hole in Modern Ophthalmology. Vol. II, second edition, pp. 781, Edited by Dutta L.C., Jay Pee brothers, New Delhi 2000. 35. Cynthia J. Kendal. ; Ultrasound Principles in Ophthalmic echography. First Indian edition, pp. 122, Jay Pee Brothers, New Delhi 1991. 36. Duke Elder S. ; The light sense in System of Ophthalmology. Vol. VII, pp. 384392, Henry Kimpton, London, 1962. 37. Dark adaptation in Manual of retinal and choroidal dystrophia. First edition, pp. 1315. 38. Caroline R. Baumal and Elizabeth L. Affel ; The electro-retinogram, the electro-oculogram and dark adaptation in Ophthalmology Secrets. First Indian edition, pp. 3036, Edited by Vander J.F. and Gault J.A., Jay Pee Brothers, New Delhi, 1998.

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39. Sharma P. : Adaptometers in Essentials of Ophthalmology. First edition, pp. 8182, Modern Publishers, New Delhi, 2000. 40. Duke Elder S. : Electro retinogram in Symptoms of Ophthalmology. Vol. VII, pp. 426439, Henry Kimpton, London, 1962. 41. Maji A.B., Sharma Y.R., Raj Shekher Y.L. and Nath R. ; Colour vision and colour blindness in Modern Ophthalmology. Vol. II, Second edition, pp. 1035, Edited by Dutta L.C., Jay Pee Brothers, New Delhi, 2000. 42. Sharma P. ; Electro physiological tests in Essentials of Ophthalmology. pp. 7881, Modern Publishers, New Delhi, 2000. 43. Seal S.K. ; Visual evoked response in GN Seals Text book of Ophthalmology. Fifth edition, pp. 1416, Current Book International, Kolkata, 2002. 44. Zwaan J.T. ; Does the baby see in Decision making in Ophthalmology. pp. 124125, Edited by Heuvan WAJ and Zwaan J.T., Mosby, St. Louis, 1998. 45. Dutta H. ; VEP studies in Clinical methods in Ophthalmology. First edition, pp. 149150, JayPee Brothers, New Delhi 1995. 46. Anita Panda ; Visual evoked response in Clinical examination of the eye. First edition, pp. 168, CBS Publishers, New Delhi, 2003. 47. Samant P.M., Hemalini P. Samant and Nabar S.M. ; Diabetic maculopathy in Ophthalmology - Theory and practical. First edition, pp. 171, Bhalani Publishing House, Mumbai, India, 2004. 48. Ahmed E. ; Diseases of the retina in A text book of ophthalmology. First edition, pp. 281, Oxford University Press, Calcutta, 1993. 49. Fischer D.M. ; Viral diseases and retina in Viral diseases of the eye. Edited by Darrell, R.W; pp. 487-497, Lea and Febiger, Philadelphia 1985. 50. Asdourian G.K. ; Viral retinitis in Manual of clinical problems in ophthalmology. Edited by Gittinger JW and Asdourian G.K. Little Brown and Co., Boston, 1998. 51. Ashton N. ; Larval granuloma of the retina due to toxocara. Br. Jr. Oph. 44 : 129-148, 1960. 52. Shield J.A. ; Ocular toxocariasis A review. Survey ophth. 28 : 361-381, 1984. 53. Kini M.D. ; Diabetic retinopathy in Manual of ocular diagnosis and therapy. Third edition, pp. 155, edited by Deborah Pavan Langston, Little Brown. 54. Tasman W. ; Retinitis pigmentosa in Pediatric Ophthalmology. Vol. I, Second edition, pp. 610-612. Edited by Harley, R.D., W.B. Saunders Company, Philadelphia 1983. 55. Fishman G. ; Variants of retinitis pigmentosa in Principles and practice of Ophthalmology. Vol. III, First Indian edition, edited by Peyman GA, Sander DR and Goldberg MA. Jay Pee Brothers, New Delhi, 1987. 56. Asdourian GK ; Retinitis pigmentosa in Manual of clinical problems in clinical Ophthalmology. pp. 115117, Edited by Gittinger JW and Asdourian GK. Little Brown, Boston, 1988. 57. Carr, RE, Siegel IM ; Unilateral retinitis pigmentosa. Arch.oph. 90:21,1973.

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58. Venkatesh P., Verma L., Tewari H.K. ; Fundus flavimaculatous (Stargardts dystrophy) in Clinical practice in Ophthalmology, First edition, pp. 339. Edited by Saxena S., Jay Pee Brothers, New Delhi 2003. 59. Schepens C.L. ; Retinal detachment and allied diseases. WB Saunders Company, Philadelphia, 1983. 60. Namperumalswamy P., Madhvan G., Hirode Dwarkanath D. and Lal S. ; Retinal detachment in Modern Ophthalmology. Vol. II, Second edition, Edited by Dutta L.C., pp. 650674, JayPee Brothers, New Delhi, 2000. 61. Johnson R.L. ; Retinal breaks in Retina, vitreous and choroid clinical procedures. pp. 54, Butterworth - Heinemann, Boston, 1995. 62. Dutta L.C. ; Rhegmatogenous retinal detachment in Ophthalmology principle and practice. First edition, pp. 174, Current Books International, Calcutta, 1995. 63. Duke Elder S. and Wyber K.C. ; The vitreous body in System of Ophthalmology. Vol. II, pp. 294-309, Henry Kimpton, London, 1961. 64. Nema H.V., Singh V.P. and Nema N. ; The vitreous in Anatomy of the eye and its adnexa. Second edition, p. 36-38, Jay Pee Brothers, New Delhi, 1991. 65. Pahwa J.M. and Billore O.P. ; Anatomy of the vitreous in Vitreous. First edition, pp. 19, Oxford and IBH Publishing Company, New Delhi, 1985. 66. Pahwa J. M. and Billore O.P. ; Vitreo retinal degeneration in Vitreous. First edition, pp. 7581. Oxford and IBH Publishing Company, New Delhi. 67. Seal SK. ; Hereditary vitreo retinal degeneration in G.N. Seals Text book of Ophthalmology. Fifth edition, pp. 287, Current Books International, Kolkata, 2002. 68. Kanski J. J. ; Vitreo retinal degeneration in Clinical Ophthalmology. Second edition, pp. 381384. Butterworth international, 1989. 69. Tasman W. ; Disease of the retina and vitreous in Pediatric Ophthalmology. Vol I, pp. 599602, Edited by Harley R.D. W.B. Sanders company Philadelphia, 1983. 70. Tasman. W. ; Lebers congenital amaurosis in Pediatric Ophthalmology. Vol. I, edition II, pp. 611, Edited by Harley R.D. WB Saunders Company Philadelphia, 1983. 71. Zwaan J. T. ; Does this baby see in Decision making in Ophthalmology. First indian edition, pp. 124, Edited by Heuven WAJ And Zwaan J.T., 1992. 72. Verma. L., Ravi K. Nainiwals and Tewari H.K. ; Optical coherence tomography. DOS times 8: 26-27, 2002. 73. Gupta Sonal, Kader M.A, RamKrishnan R. ; Optical Coherence Tomography: A new Tool for glaucoma diagnosis. Journal of Arvind eye care system. Vol. III, 12 3-9, 2003. 74. Tewari H.K., Wagh V.B., Parul Sony, Venkatesh P., Singh. R. : Macular thickness evaluation using the optical coherence tomography in normal Indian eyes. Ind. Jr. Oph. 52-199-204, 2004.

CHAPTER

12

Retinoblastoma1,2,3,4
RETINOBLASTOMA
Retinoblastoma is most common intraocular malignancy of early childhood. It is more frequently encountered malignancy of childhood than rhabdomyo sarcoma, which is commonest orbital neoplasm in children. Retinoblastoma is uniformly fatal5 if not treated. When treated early i.e. at intraocular stage without extension, five years survival rate is as high as 90%. A. Incidence of retinoblastoma varies between 1 in17.000 to 1 in 30.000 per live births6,7. However incident is becoming more frequent. There are many factors for these upwards swing in frequency i.e. 1. Change in environment. 2. Ambient radiation. 3. Higher survival rate in persons who had heritable retinoblastoma and have passed the gene to the next or second generation. 4. Increased awareness among parents about retinoblastoma. 5. Reduction in neonatal death. It is equally common among males and females. No race is immune to retinoblastoma. However there may be clustering of gene in some communities7. It is detected early in developed countries where cure rate is high. It is detected late in under developed countries obviously due to lack of awareness, lack of diagnostic facilities. B. About seventy percent of tumours are unilateral. In bilateral cases, the tumour need not be symmetrical in both eyes, one may be more advanced than the others. Bilateral cases present earlier than unilateral. Average age of presentation of bilateral cases is twelve months and that of unilateral is twenty four months. In under developed countries children with retinoblastoma are brought for treatment late, generally with extra ocular metastasis and poor prognosis. Children with positive family history of retinoblastoma are brought very early. They are often examined for its evidence in neonatal age. Incidence of retinoblastoma shows a sharp decline after three years of age. It becomes very rare after seven years of age. However retinoblastoma has been reported in adulthood. The two possibilities in such presentation are: 471

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1. The adult had retinoblastoma in childhood which has under gone spontaneous regression and only a scar is left. 2. On rare occasion the tumour has remained dormant and shown activity later. C. Retinoblastoma is thought to be a congenital tumour, which may be seen in some cases at birth if looked for specifically. Contrary to belief, retinoblastoma is a slow progressive neoplasm. So long it is intra ocular it takes months to years to become extra ocular. Once the tumour becomes extra ocular distant metastasis is rapid and fatal. D. Heredity Heredity in retinoblastoma. It is confusing to an average ophthalmologist who is not trained in intricacies of genetics. Some of the salient features of heredity in retinoblastoma are: 1. Retinoblastoma is inherited as autosomal dominant trait3 2. 60% of retinoblastomas are non hereditary. 3. 40% are hereditary. 4. Incidence of spontaneous mutation is high 5. Both somatic and germinal mutation are met with in retinoblastoma8 6. Patients with germinal mutation are capable of passing the gene to the offspring. 7. Sporadic cases are generally unilateral 8. Bilateral cases are mostly hereditary 9. 10% to 20% unilateral cases are hereditary 10. 94% of all cases of retinoblastoma are sporadic. 11. Only 6% of all cases of retinoblastoma have family history. 12. To summarise (a) 2030% are bilateral and hereditary (b) 1020% unilateral and hereditary (c) 5560% unilateral non hereditary. (d) None are Bilateral and non hereditary. 13. Genetic mutation is due to chromosome 13 in region of 13q 149. Genetic counselling is assuming more importance in management of retinoblastoma because more children survive to reach reproductive age. If any of the parents is affected with either familial retinoblastoma or bilateral retinoblastoma chances are that their offspring will have 50% chances of inheriting retinoblastoma. Risk of passing the gene to next generation is greatly reduced if the children of parents suffering from familial retinoblastoma are healthy. Clinical Presentation of retinoblastoma depends upon Position of the lesion, size of the lesion, its extension in side the globe, duration of the growth, extra ocular extension and family history.

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Traditionally retinoblastoma has been divided in to following stages. A. Stage of quiescence B. Stage of white reflex C. Stage of secondary glaucoma D. Stage of extra ocular extension E. Distant metastasis All eyes need not go through the above sequence always. A child may pass in to stage of glaucoma or even extraocular extension without going to stage of white reflex in the pupillary area ( Leucokoria) if the growth is near the disc. Ellsworth10 has divided retinoblastoma into five groups that spread over clinical Stages A & B for better planning of management of retinoblastoma. Prognosis depends upon the group in which the tumour belongs. Group I has best prognosis.
Group I II III IV V Number of Tumours A Single B Multiple A Single B Multiple A Any B Single A Many B Any A Single B Vitreous Seeds Size < 4DD > 4DD 4-10 DD 4-10 DD Any < 10 DD > 10 DD Variable Massive Position At or behind equator Behind equator At or behind equator Behind equator Anterior to equator Behind equator Anywhere Anterior to ora Half of retina Prognosis Very favourable Very favourable Favourable Favourable Doubtful Doubtful Unfavourable Unfavourable Most unfavourable Most unfavourable

PRESENTATION
Children with retinoblastoma or suspected to have retinoblastoma seeking medical aid belong to three age groups. A. Between 20 to 30 months these are generally unilateral and non hereditary B. Round about 15 months of age these are generally bilateral and hereditary. C. Within few days of birth, these infants belong to families known to harbour retinoblastoma. Commonest mode of presentation in first two age groups is white reflex in pupillary area Leucokoria: unilateral white reflex is by far most common, even in cases of bilateral involvement one eye has whiter reflex unlesss both eyes have equally advanced growth. The white reflex is generally detected by parents. The colour of the reflex is due to reflection of light from the tumour that lacks pink hue of normal retina. The growth is raised from the

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surface of retina. Sometimes white reflex is visible only when the child moves the eye from side to side. Second mode of presentation is strabismus which is commonly esotropia this makes detailed fundus examination with indirect ophthalmoscope mandatory. The child may be brought with red and watery eyes, the parents may correlate redness to some trivial, sometimes, imaginary injury. By the time the child develops photophobia the eye has passed in to stage of secondary glaucoma. Proptosis is a late presentation and means extra ocular extension.

EXAMINATION OF THE EYE INCLUDES


A. Flash light examination 1. For conjunctival and ciliary congestion 2. Corneal size 3. Anterior chamber for pseudohypopyon 4. Pupil for size and colour leucocoria 5. Iris (a) Heterochromia (b) Nodule (c) Neovascularisation 6. Lens. The lens is examined for its transparency and retro lenticular mass which is mistaken as cataract. Lenticular opacities are late to develop in retinoblastoma. B. Slit lamp examination. To confirm findings of torch light examination the eye should be examined under magnification preferably with a slit lamp. In very small children it is better done under anaesthesia either with a hand held slit lamp or with an operating microscope. C. Indirect ophthalmoscopy, both eyes should be examined with complete mydriasis and sclera indentation under general anaesthesia. D. Recording of intra ocular tension and measurement of corneal diameter should also be performed under general anaesthesia, Anesthetics that cause rise of IOP should be avoided. E. B.scan ultrasonography. If the child does not co-operate it should also be done under general anaesthesia. Retinoblastoma appears as a solid mass arising from retina with high reflectivity. F. Other Investigation 1. X-ray orbit for evidence of intra ocular calcification. 2. CT for intra cranial extension, condition of optic canal, presence of Trilateral Retinoblastoma, intraocular calcification. 3. M.R.I11. This confirms intraocular calcification, shows extra ocular extension including optic nerve infiltration, MRI of brain show presence of Pinealoblastoma (trilateral retinoblastoma) 4. Fine needle biopsy

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G. Systemic examination for 1. Distant metastasis, 2. Primaries that may produce proptosis mimicking retinoblastoma H. Fundus examination of parents grand parents, in case of bilateral tumours.

DIFFERENTIAL DIAGNOSIS OF RETINOBLASTOMA


Differential diagnosis of retinoblastoma comprise of two groups of disorders A. The first group comprises of the most important causes that present with white reflex in the pupillary area. Infact it is best to suspect each case of white reflex in the pupillary area specially if it is retrolental to be retinoblastoma unless proved otherwise. It should be remembered that retinoblastoma is a life threatening condition. B. Second group consists of those diverse conditions that produce proptosis with or without white reflex in the pupillary area.

CONDITIONS THAT PRODUCE WHITE REFLEX CAN BE GROUPED INTO


A. Lenticular B. Retrolenticular C. Pre lenticular Lenticular causes of white reflex comprise of congenital cataract, developmental cataract and traumatic cataract. Retrolenticular causes of white reflex comprise many diverse conditions that give white reflex in pupillary area traditionally they were grouped as Glioma and Pseudoglioma. This is due to early misconception that retinoblastoma was a glioma. It is more logical to group them as Retinoblastoma and non-retinoblastoma The non retinoblastomic causes could be congenital anomalies, iatrogenic (toxic ), inflammatory and vascular.

NON RETINOBLASTOMIC RETROLENTAL CAUSES OF WHITE REFLEX IN PUPILLARY AREA4,12,13,14


A. Congenital 1. Persistent hyperplastic primary vitreous 2. Retinal dysplasia 3. Congenital retinoschisis

476 4. Incontenentia pigmenti 5. Large coloboma of choroid 6. Congenital retinal fold 7. Large opaque nerve fibre B. Iatrogenic Retinopathy of prematurity C. Vascular 1. Persistent hyperplastic primary vitreous 2. Retinopathy of prematurity 3. Coats diseases 4. VonHippels disease 5. Lebers miliary aneurysm D. Inflammatory 1. Ocular toxocariasis 2. Metastatic endophthalmitis 3. Retino choroiditis 4. Metastatic retinitis E. Newgrowths 1. Meduloepithelioma 2. Leukaemia 3. Choriodal hemangioma F. Miscellaneous 1. Norries disease 2. Familial exudative vitreo retinopathy

PEDIATRIC OPHTHALMOLOGY

INFLAMMATORY CAUSES OF WHITE REFLEX IN PUPILLARY AREA CAN BE


A. Prelenticular exudative deposits in front of the lens in the form of membrane obscuring lens i.e. occlusiopupillae. B. Complicated cataract. C. It can produce retrolental pathology mimicking retinoblastoma. These causes are 1. Posterior cyclitic membrane 2. Parsplanitis 3. Metastatic endophthalmitis 4. Parasitic endophthalmitis.

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D. The vascular causes of white reflex that also produce retinal mass are 1. Coats disease 2. Lebers miliary aneurysm 3. Angiometosis retinae E. Proptosis itself is a late mode of presentation. It is produced only when retinoblastoma breaks the scleral barrier. Once the growth is extra ocular, its spread inside the orbit and distant metastasis is fast. Other cause of proptosis that simulate retinoblastoma are 1. Secondries (a) Leukaemia (b) Neuroblastoma 2. Inflammatory (a) Orbital cellulitis (b) Cavernous sinus thrombosis (c) Sub periostial abscess (d) Parasitic cyst (e) Panophthalmitis 3. Primary tumourRhadbomyosarcoma

PATHOGENESIS OF RETINOBLASTOMA
Pathogenesis of retinoblastoma is poorly understood. It may have hereditary predisposition, however, majority of them are sporadic. Advanced parental age and history of cancer in excess of demographically expectation in the family and 13q deletion syndrome are some other predisposing factors9. Primitive precursor of photoreceptors which normally produce rods and cones under go anaplastic change to give rise to retinoblastoma cells in the outer layer of retina. Endophytic tumours originate in inner nuclear layer while exophytic tumours originate in external nuclear layer. The retinoblastoma cells are semi columnar cells with large dense nuclei and scanty cytoplasm. The retinoblastoma cells have a tendency to arrange themselves in the form of rosettes, fleuerettes and pseudorosettes. There are two types of rosettes i.e. FlexnerWintersteiner, and Homer-Wright rosettes. A. Flexner-Wintersteiner rosettes7 are typical of retinoblastoma. The cells are arranged in circular fashion leaving a central empty lumen, that is lined by a clear membrane over which the cells are arranged with large hyperchromic nuclei away from the lumen figure. Multiple rosettes are seen to be scattered all over the field. The membrane over which the cells are arranged is similar to the external limiting membrane of retina. The membrane may be penetrated by blunt processes that are similar to photoreceptors. The lumen contains acid mucopoly saccharides.

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B. Homer-Wright rosettes. These rosettes are less commonly found in retinoblastoma as compared to Flexner-Wintersteiner rosettes they are more common in neuroblastomas and medulo blastomas. The main difference is that there are no acid mucopoly saccharides in the lumen. It is said More are the rosettes better is the prognosis. C. Fleurettes. These are yet another type of retinoblastoma cell arrangement without having a central lumen, the cells are more elongated which are arranged like bouquet in a radiating fashion not forming a complete rosettes. The cells resemble photoreceptors more than rosettes. Sometimes the whole of tumour may be composed of fleurettes only. Fluerettes represent more benign character than rosettes. Tumours composed of only such cells have been termed Retinoma or Retinocytom. D. Retinoma or retinocytoma8.16. This is a rare benign tumour of retina due to mutation of retinobastoma gene. Rarely it may under go malignant change. The retinomas generally do not form rosettes. They generally form fleurettes. Calcification is common. The growth is translucent, homogenous, inter retinal mass. Sometimes the term retinoma is used for spontaneously arrested retinoblasoma with out necrosis. E. Pseudo rosettes. A rosette is called a pseudo rosette if the retinoblastoma cells are arranged round a blood vessel that replaces the central lumen of a true rosette. The cells receive nourishment from these blood vessels and show a luxuriant growth that may be too much for blood supply, resulting in to ischemic, coagulative necrosis of tumour cells away from the blood vessels where calcium is deposited that show up on x-ray, ultra sound, CT or MRI. This ischemic necrosis is most probable cause of spontaneous regression4,8,16 is met with in one to three percent of cases. Other hypothesis is that spontaneous regression is an immune response15. In some cases of spontaneous regression, recurrence has been reported. Genetic predisposition is unaffected by spontaneous regression. Spontaneously regressed tumour looks like a healed scar this makes examination of fundi of parents and siblings by indirect ophthalmoscope an important clinical evaluation. Specially if the child has bilateral involvement. There are two clinical presentation of spontaneous regression. 1. A flat scar similar to scar of post radiation treatment of retinoblastoma. 2. Phthisis bulbae of effected eye due to extensive necrosis of tumour infiltration of optic nerve. These growth have good prognosis. Endophytum retinoblasoma arises from inner nuclear layer and grows preferentially towards vitreous as a pink white well demarcated mass. Calcium deposit over the mass is common, there may be new vessel formation on the mass, vitreous seeding or cells are common. These are more prone to optic nerve involvement. Differential diagnosis consists of various inflammatory causes. Exophytum retinoblastoma this type of tumour arise from outer nuclear layer and spread towards the chorid, lifting the retina, initially causing a retinal detachment and have more haemato genous spread. The tumour is not visible with ophthalmoscope. Differential diagnosis comprise of Coats disease, retinal capillary haemangioma and retinal detachment.

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Differentiation of retinoblastoma into endow or exophytum is limited to early stages. In late stages it is very difficult to separate them from each other.

SPREAD OF RETINOBLASTOMA
Retinoblastoma may have multicentric origin in the same eye. The lesions are independent of each other, may be of same duration or varied duration. In bilateral cases the other lesion has no connection with the fellow eye. However there may be seeding from one of the growths into the vitreous which may get deposited any where on the retina or may be carried into the anterior chamber and implanted on the iris as nodule, may clogs the angle or may be deposited at the bottom of A.C. as Pseudohypopyon. There may be heterochromia of the iris, formation of nodule on the iris or neovascularisation of the iris, leading to secondary glaucoma. It takes few months for retinoblastoma to become extra ocular.

EXTRA OCULAR SPREAD MAY BE


A. In the orbit. This happens when the tumour breaches the sclera. Orbital deposits produce. Proptosis, chemosis, extra ocular muscle involvement, conjunctival infiltration and lid infiltration. Once tumour cells are picked up by conjunctival vessels haematogenous spread occurs. As orbit has no formed lymphatic, logically there should not be any lymphatic spread but lymphatic spread to preauricular and sub mandibular lymphnodes are common. They are secondary to conjunctival and lid involvements, these structures are very rich in lymphatic. B. In the optic nerve. There may be direct infiltration of the optic nerve. Initially it is prelamilar, once it passes through the laminacribrosa, it may either spread along the central retinal vessels or may infiltrate the pial vessels and get deposited in the subdural and subarachnoid space from where it reaches the cerebro spinal fluid and is carried into the brain and spine. Chances of optic nerve involvement are more in large endophytum and in the stage of secondary glaucoma. C. In the choroid. The choroid lies flush with retina, it is highly vascular hence its chances of being infiltrated by tumour cells are more than that of optic nerve. Once the choroid is invaded haematological spread is rapid, so is orbital extension. D. Trilateral Retinoblastoma is infiltration of retinoblastoma in the mid line in the Pineal body in case of bilateral retinoblastoma before five years of age. It may be suprasellar or parasellar. Histopathologically it has appearance similar to retinoblastoma in contrast to distant metastasis which do not have characteristic of retinoblastoma. The trilateral retinoblastoma is best seen on CT16. E. Second Neoplasm in Retinoblastoma If the child survives the first retinoblastoma he still has chances of developing second neoplasm unrelated to original retinoblastoma. Common tumours being osteogenic sarcoma, cutaneous melanoma, fibrous histocystoma and other sarcomas. Second neoplasm is more common in bilateral cases, average age being 13 years but can occur at any age, radiation may also cause second neoplasm.15,17.

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OTHER CONDITIONS THAT PRODUCE ROSETTES


Rosettes are hall mark of diagnosis of retinoblastoma. However , there are other conditions that produce rosettes can be malignent or benign they are: Medulla epithelioma, neuroblastoma, retinal dysplasia, incontinentia pigmenti, and retinoma.

MANAGEMENT OF RETINOBLASTOMA
Management of retinoblastoma consists of A. Definite diagnosis of the condition. In the past eyes have been removed due to missed diagnosis of non retinoblastoma as retinoblastoma. B. To treat the tumour. C. Prevent spread of tumour D. Treat distant metastases E. Prevent recurrence F. Retain as much vision as possible. G. Genetic counselling H. Management of second primary

TREATMENT OF THE NEOPLASM


Depends upon A. Size, number and position of the tumour as per Ellsworth classification group I to III B. Is it still intraocular C. Has it become extra ocular (i) is there evidence of optic nerve involvement (ii) is there orbital deposit (iii) is there distant metastasis. Bilateral retinoblastomas are more difficult to manage than unilateral, but unilateral retinoblastomas generally present late hence are more advanced when seen for the first time. In cases belonging to Ellsworth group I to III local treatment with Cryo, photocagulations, radioactive cobalt plaque or controlled radiation may be sufficient. Cryotherpy is suitable only for tumours anterior to equator.

THE ENUCLEATION IS A DEFINITIVE TREATMENT IN CASES OF


A. The tumour is still intra ocular. B. Uniocular

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C. There is gross loss of vision D. In bilateral cases, one eye is always more advanced than the other, enucleation is advocated for eye containing more advanced tumour. E. Bilateral enucleation is tried only if there no salvageable vision in either eye or other modes like chemotherapy or radiation are not possible. While enucleating all efforts should be made to cut the optic nerve as far from the globe as possible and subject it for histopathological examination. Infiltration of distal end of optic nerve denotes intra cranial extension. The outer surface of the enucleated eye ball should be closely scrutinised for evidence of breach in the sclera. In no case should an eye containing retinoblastoma be eviscerated. It is good practice to subject all materials removed by evisceration in children for any cause for histopathology to exclude masquerade syndromes18. Where a retinoblastoma may present as endophthalmitis or posterior uveitis.

RADIATION4
A. External beam radiation B. Plaque radiation External beam radiation is the mainstay of treatment of retinoblastoma. It can be used as eye preserving device in. 1. Moderate isolated tumour 2. Small multiple tumours 3. Involvement of optic nerve 4. Bilateral cases 5. Cases that do not respond to local treatment by photocogulation or cryotheraopy Advantages of liner accelerator external beam radiation is that the eye under radiation is least damaged and the other eye can be protected. The eye is radiated for five days a week with daily dose of 250 cGy daily, total dose ranges from 2500 to 4500 cGy ( 4000-5000 rads) All bilateral cases should be subjected to radiation either alone or in combination with chemotherapy. Eyes with vitreous seeds do not response well to radiation. Side effects of irradiation have been divided in to 1. Short term 2. Long term effects. The short term complications are:- redness of the conjunctiva and skin, madarosis, dry eye. Long term side effects are radiation cataract, radiation retinopathy and optic neuropathy, dry eye, arrest of orbital growth and second neoplasm. B. Plaque radiations4. This mode of treatment consist of suturing a radio active plaque on the sclera over the tumour commonly used plaques are iodine 125, iridium 192, ruthenium 106. Radon and cobalt 60 are no more popular plaques. The treatment with radioactive plaque is indicated for eyes with relatively large, localised, isolated nodule without involvement of the

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optic nerve or macula. This method can be used either as primary treatment or in case that have failed to respond to: Cryotherapy, photo coagulation or external beam radiation. The aim is to impart radiation of 40 cGy to the apex of the tumour. It should be remembered that the tumour base that is nearest to the plaque gets far more radiation than the apex. The plaques are custom built for each eye. The method has very few complication i.e. post radiation cataract during first year . A thin macular exudate may develop after months. The treatment period with plaque period is short that is 2 days as compared to external bean that requires five weeks16.

CHEMO THERAPY
Various chemotherapeutic agents have been used with variable cure rate and complications. Each agent has its own route of administration i.e. Oral, intra muscular, intra venous and intrathecal. Most commonly used drugs are Vincristine, ActinomycinD and Cyclophosphamide. Other drugs used are methotrexate, doxorubicin, carboplatin. The dose and frequency should be adjusted in consultation with pediatric oncologist.

PROGNOSIS
A. Untreated retinoblastomas are uniformly fatal. Death occurs within two to three years of detection of tumour in such cases. B. Prognosis is largely influenced by cell pattern of the growth : 1. Tumours composed of entirely fleurettes have excellent prognosis. 2. Tumours with rosettes have better prognosis than without rosettes. 3. Tumours with abundant Flexner Wintersteiner cells have good prognosis but less than those which have fleurettes only. 4. Tumours with no rosettes have the poorest prognosis C. Prognosis also depends upon infiltration of the tumour :1. Optic nerve infiltration (a) No infiltration:- mortality is about 8% (b) Invasion upto laminacribrosa : the mortality goes to 15%. (c) Infiltration beyond lamina but confined to the cut (scleral) end : Mortality is 44%. (d) Invasion into the cranial end the mortality is as high as 65%. (e) Trilateral retinoblastoma :- cent percent mortality, even with multi modality treatment. 2. Choriod A. Slight infiltration:- mortality is about 25% B. It rises to 65% in heavy infiltration C. Mortality is 80% to 100% if the sclera is breached.

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3. Distant metastasis have uniformly fatal out come even with best treatment due to multiple organ involvement and side effects of chemotherapy and radiation.

OTHER PARAMETERS USED TO EVALUATE PROGNOSIS IN RETINOBLASTOMA ARE


A. Clinical grouping16 1. Early lesions 2. Moderately advanced lesion 3. Advanced lesion 4. Distant metastasis B. Histological classification19 1. Resse Ellosworth Classification. 2. Essen prognosis Classification4 3. Tumour node metastasis system20 4. St. Judes hospital classification Out of above systems most widely used system are Resse Ellosworth classification and tumour node metastasis evaluation.

DIFFERENTIAL DIAGNOSIS OF WHITE REFLEX IN PUPILLARY AREA( LEUCOCORIA)


Retinoblastoma is the most important cause of leucoria in children as far as loss of vision and life expectancy is concerned. Hence the differential diagnosis should be between:A. Retinoblastoma (Glioma) and B. Non retinoblastoma ( Pseudo glioma) Most of the non retinoblastomic causes are sight threatening but not life threatening. They can be : Prelenticular Lenticular 1. Vascular 2. Inflammatory 3. Neoplastic Exudate in pupillary area Cataract congenital or traumatic See page 475 also Retrolental fibroplasia Persistent Primary hyper plastic vitreous Coats disease. Posterior cyclitic membrane. Endophthalmitis Parasitic granuloma Metastatic retinitis Medullo epithelioma, leukaemia.

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484 4. Retinal Conditions

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Retinal dysplasia, Incontenintia pigmenti, massive retinal fibrosis, congenital non attachments of retina, secondary retinal detachment, juvenile retinochisis, large coloboma uvea.

LENTICULAR CAUSES OF WHITE REFLEX IN PAPILLARY AREA


A. Congenital Cataract. There should be no difficulty in diagnosis of congenital cataract. These are : 1. Generally bilateral 2. Positive family history is frequent. 3. Corneal diameter is within normal limits with normal AC in depth and contents. 4. Pupil are central circular and react brisk to direct and indirect light stimulus. 5. Generally there is no history of prematurity, low birth weight or prolonged administration of oxygen. 6. The third Purkenjes image is present, fourth may be absent if the lens is fully opaque. 7. Intra ocular tension is normal. 8. In case of incomplete cataract the fundus is visible through widely dilated pupil. 9. Ultra sonography of retrolential structure is normal. B. Traumatic cataract. These are still easier to diagnosis, if there is a clear cut history of injury : penetrating or blunt. However the child may hide history of trauma or parents may co-relate presence of white reflex in pupil to some trivial injury this is more common in retinoblastoma than in any other cause. Redness, raised intra ocular tension and presence of flakes of cortical matter in AC lead to more confusion which are best settled by B scan ultra sonography and CT. Traumatic cataract does not show intra ocular classification but may reveal unsuspected intraocular foreign body.

RETRO LENTICULAR CAUSES OF WHITE REFLEX IN PAPILLARY AREA


A. Retro lental fibro plasia (Retinopathy of prematurity)21,22,23,24 This is a vasoproliferative retinopathy seen in premature infants with low birth weight who have been on hyper braic oxygen for more than seven days. However with lesser birth weight and shorter gestational age, the chances of getting retrolental fibroplasias is more. It is less common in children born after 32 weeks of gestation and birth weight more than 1500gms. Twining and maternal diabetes may be contributory factors. It is a bilateral condition seen equally in boys and girls. It is incidence is less in developing countries only due to the fact that most of the children do not get oxygen therapy and die before the condition develops. However prematurity has superseded oxygen as chief cause of retinopathy of prematurity. Hence all children under 1500gms should be examined by indirect ophthalmology after six weeks of birth irrespective of oxygenation.

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Early (acute Phase) is mostly self limiting in 90% of cases. In early retinopathy of prematurity normal development of retina goes awry and there is proliferation of abnormal new vessels which lead to fibrous proliferation. To understand retinopathy of prematurity it is better to recapitulate the development of retinal vasculature and effect of oxygen on it. 1. In intrauterine life normal vascularisation of retina begins at optic disc and spreads towards the periphery in second half of gestation. The temporal periphery is last to get vascularised i.e. by about 36 weeks. However it may take few more days post natal for the temporal periphery to get vascularised fully. 2. In a child born before 32 weeks the vessels are far too short of the periphery. It may be noted that rarely vessels may reach upto periphery even in a premature child, such a child will not develop RLP. 3. The vessels in pre term child are immature and are sensitive to high concentration of oxygen. 4. Toxic effect of hyper baric oxygen has two phases. (a) Oxygen due to some ill understood mechanism causes spasm and obliterate under developed vessels. Obliteration of vessels interrupt further growth of the vessels. At this stage a mesenchymal ridge in the form of complete ring or a broken ring is formed all round. The ridge is more prominent on the temporal periphery. (b) The next phase is conversion of the mesenchymal ridge in to an arterio venious shunt. New capillaries sprout from these shunts anterior to the mesenchymal shelf. In milder form the neovascularisations spontaneously regresses. This type of change is called early retinopathy, while a sever from is called late retinopathy. There is formation of microaneurysms and new vessels that may invade the vitreous bleed resulting in traction vitreo retinal bands leading to traction detachment in the form of a retrolental mass that gives a white reflex in pupillary area. It takes about ten weeks for retinopathy of prematurity to develop.

INTERNATIONAL CLASSIFICATION OF RETINOPATHY OF PREMATURITY


The classification consists of five stages which are :A. Stage of line of demarcation. Here a white tortuous line running parallel to the ora is seen. The line is flat on the retinal surface, mostly on the temporal side. It divides retina in to an anterior avascular area and a vascularised retina. B. Stage of ridge formation. The line of demarcation thickens in width and height . Few vessels on the vascularised retina form localised neovascular tuft but there is no fibro vascular growth from the ridge. C. Stage of fibro vascular proliferation. In this stage extra retinal fibro vascular proliferation is seen on thickened ridge. The retinal blood vessels become tortuous and dilated. There may be retinal or vitreous haemorrhage.

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D. 1. Stage of sub total temporal retinal detachment without macular involvement. 2. Stage of Subtotal traction detachment with macular involvement E. Stage of total retinal detachment

CHARTING OF LESIONS IN RETINOPATHY OF PREMATURITY


Charting of retinopathy of prematurity differs from usual retinal charting where macula is taken as centre of the chart. While charting R.P.P.M. optic disc in taken as center and two concentric circles are drawn dividing the retinal chart in to three zones A. The first zone has diameter twice the distance between the fovea and the disc. B. The second circle extends from centre of disc to nasal ora serrate. The area between first and second circle is second zone. C. Remaining part which is widest on temporal side and almost obliterated on nasal side is called zone three. Lesions extending in zone one are most sever while those localised in zone three are mildest. Clinically on the basis of indirect ophthalmoscopy findings retinopathy of prematurity has been divided in to five stages25,26. They are : Stage 1. Visible line of demarcation, it consists of a clear cut line of demarcation between the avascular and vascular retina. The line is pale in colour, not raised, is as flat as rest of the retina. Stage 2. In this stage the line of demarcation is elevated over the retina and is said to have height and width. It is more towards pink than white as compared to line of demarcation, small tufts of vessels may be seen posterior to the ridge and these vessels do not form the fibro vascular stage that is seen in the next stage. Stage 3. There is a definite ridge of extra-retinal fibro vascular proliferation. Stage 4. This is state of partial traction retinal detachment that has been divided in to two substages i.e. stage 4 a and 4 b former consist of sub total tractional detachment without involving the macula while in the later the macula is involved. Stage 5. This is a stage of total retinal detachment. Plus disease retinopathy of prematurity plus disease denotes progressive vascular compromise. Presence of plus disease is an ominous sign. It is called anterior plus when there is neovascularisation of iris and difficulty in dilation of pupil. The posterior plus represents retinal venous dilation and arterial tortuasity in the posterior pole. Thresh hold disease This stage is defined as presence of at least five contiguous or eight cumulative clock hours (30o) sector of stage 3 in zone I or II in presence of plus disease. Importance of this stage lies in the fact that 50% of children in this group are predicted to go blind if not treated. Prethresh hold disease this comprises of 1. Any stage of disease in zone I 2. Stage 2 disease in zone II with plus disease 3. Extra fibro vascular proliferation zone III.

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The disease is called Rush disease when this occurs in an infant weighing less than 1000 gms who develop the disease earlier and faster between 3 weeks and total retinal detachment is almost certain. Management of retinopathy of prematurity24,25,27 The best method of treatment is prevention of the condition by monitoring oxygen. However retrolental fibroplasia has been observed even in infants who have either been on oxygen for very short period or within recommended period and concentration. Besides gestation period birth weight and oxygen administered there are other factors that may be influencing development of retrolental fibroplasia they are - Vitamin E deficiency, phototoxicity, blood pH, CO2 tension, maternal diabetes and prostaglandin level. Predisposed premature infants must be examined : 1. First between four to six weeks post delivery by indirect binocular ophthalmoscope under complete but safe mydriasis. 2. Then at interval of ten days till there is complete vascularisation of retina all round 3. Then every fortnightly and 4. Every month. Treatment of retrolental fibro plasia is controversial because 1. Spontaneous regression is seen in 80 to 90% of cases in acute stage. 2. All modalities of treatment have poor visual outcome. 3. There is no known clinical parameter that can evaluate efficiency of any or combinations of treatments. 4. Over all prognosis is poor, children with best treatment also have subnormal vision. Most Commonly used therapeutic and surgical methods are 1. Cryo therapy, 2. Laser photocoagulation delivered by indirect ophthalmoscope either diode or argon lasers are used. Anterior lesions of zone III have best prognosis while those in zone I have worst prognosis. Stage four and five are treated by vitreo retinal surgery. Complications of retinopathy of prematurity consist of High myopia, retinal pigmentation, lattice degeneration, dragging of retina, retinal hole formation, rhegmatogenous retinal detachment, secondary glaucoma and squint.

PERSISTENT HYPERPLASTIC PRIMARY VITREOUS28,29,30


This is a congenital anomaly of primary vitreous and hyaloid artery. It is due to failure of regression of primary vitreous. This is non hereditary generally uniocular. These eyes are microphthalmic the child is full term with normal expected birth weight, there is no history of administration of hyperbaric oxygen in first week of life. There are two types of this disorder A. A more common anterior and B. Rarer form of posterior type

488 Anterior type

PEDIATRIC OPHTHALMOLOGY

The eye is microphthalmic the anterior chamber is shallow, undeveloped angle, iris has large visible blood vessels. Initially the lens is clear with visible retrolental white mass. The ciliary processes are pulled towards the mass and are elongated. The retrolental mass may contract and produce a rent in the posterior capsule leading to cataract formation that may cause swelling of the lens, pushing the iris lens diaphragm forward, narrowing the already faulty angle. The cortical matter may absorb leaving a membranous cataract. The retrolental mass may cause traction detachment. Posterior type This is less common than former. Generally cornea is smaller than normal, anterior chamber is either normal or deep, lens is clear and normal in size, there may be coloboma of lens or subluxation of lens. Commonest finding is in the posterior part of the globe resulting in a white opaque membrane or retinal fold extending from optic disc to periphery of the retina, it may extend in the retrolental space resulting in to a white reflex. Posterior variety may be bilateral. Milder form of this anomaly is Bergmeister papillae and Mitten drofs dot. Some times remnants may be seen in the mid vitreous at the level of Cloquets canal. Management. There is no known preventive method or medical treatment. Surgical intervention in the form of lensectomy and vitrectomy if done early may have good vision . As the condition is unilateral, amblyopia is common and must be treated vigorously.

COATS DISEASE31,32,33,34
This is a non hereditary non inflammatory disorder of retinal vascularture. The exact pathogenesis of the condition is not known, the most probable cause is congenital malformation of retinal vessels i.e. telengiectasis that is associated with subretial exudation and non rehegmatiogenous retinal detachment. Condition is invariably seen in male children in first decade and in ninety percent cases it is unilateral. In bilateral cases the changes are minimal. In rare instances the condition is detected in infancy. The lesion starts in the retinal periphery as dilated vessels with capillary drop out, rarely there is vascular sheathing. Exudation is an important feature, the exudation is mostly seen in the posterior pole, sub retinal fluid contains cholesterol crystals but no calcium. The natural history of the condition is progression toward formation of white reflex in pupillary area behind the lens. The cause of white reflex in pupillary area is to exudation and retinal detachment. There may be secondary and complicated cataract. Neovascularisation of iris and angle is common feature in poorly managed cases that lead to neovascular glaucoma which may terminate in enucleation. Many blind eyes with glaucoma in Coats disease have been removed with mistaken diagnosis of retinoblasoma. The eyes may go into phithisis. In rare instances milder forms may regress spontaneously. There should be no difficulty in diagnosis of a case of Coats disease if media is clear. How ever in advanced cases ultra sonography and CT are two most helpful investigation. CT is single most valuable test as it delineates intra ocular morphology.

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Differential diagnosis of coats disease consists of retinoblastoma, angiomatosis retinae (generally seen in third and four the decade) familial exudative vitreo retinopathy, retinopathy of prematurity and endopahthalmitis. Management of Coats disease consists of early detection exclusion of other causes of white reflex in pupillary area confirmation of diagnosis. Best results are seen if the treatment is initiated before exudation starts. There is no medical treatment. Two modes of treatment that are followed are 1. Cryotherapy and 2. Laser phopcoagulation. It takes eight to ten months for exudate to disappear after laser photocoagulation. Recurrence due to formation of new telengectasis is know to occur in poorly managed cases or years after initial successful treatment.

LARVAL GRANULOMA OF EYE - TOXOCARIASIS35,36,37 (See page 264)


This chronic systemic disorder is caused by parasite Toxocara canis a roundworm of dogs. There is doubt if toxocariasis can be caused by toxocara catis the round worm of cats. The child gets infected by the ova of the parasite that is passed in the faeces of the infected dog. The children who develop toxocariasis have history of pica. These children come in close contact with puppies that are infected at an early age, consuming food accidentally infected by ova. The commonest age for a child to get infected is Under two years. By two years the child gets symptoms of systemic involvement. The systemic involvement comprises of low grade fever, hepatosplenomegaly, pneumonitis convulsion and cutaneous erythema. The systemic involvement is known as Viscereal larval migrans which does not cause ocular involvement. Ocular involvement without intestinal involvement is most improbable. Visceral larval migrans causes leucocytosis, and eosinophilia Elisa test is positive in titre of 1:8. Positive Elisa helps to differential ocular toxocaria from retinalblastoma. Ocular toxoariasis In contrast to age of systemic involvement which is two year, average age to develop ocular toxocariasis is 7.5 years. It is generally unilateral. There are three known forms of ocular toxocariasis. They are A. Chronic endophthalmitis. B. Posterior pole involvement C. Peripheral granuloma. Chronic endophthalmitis is most common and devastating. It is seen between 2 to 9 years of age. The ocular involvement is typically a combination of chronic uveitis with vitrities resulting in to loss of vision, leucokoria and strabismus. A combination uvea and vitrities leads to posterior cyclitic membrane, retinal detachment and complication cataract. Visual prognosis is very poor. These eyes are generally enucleated as they become painful blind eyes. The lingering fear of reinoblasoma is perhaps the most important motive to remove such eyes. Histopathology may reveal dead larva. Posterior pole involvement This occurs in slightly older, children i.e. between 6 to 14 years and is unilateral leading to loss of vision. The granulomn is situated between the

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macula and the disc or may be seen over the macula. It is a raised yellowish mass wider than optic nerve head. The mass may be surrounded by retinal edema, hard exudates or stress lines, retinal vessels may dip in the mass. Peripheral granuloma : This is least vision threatening seen in children as well as adult upto third and fourth decade is again unilateral produces least inflammatory signs. Generally vision is unaffected however it may produce cystoid macular edema or retinal detachment. The lesion is a hemi spherical mass anterior to equator with vitreous band. Management The management comprises of prevention of infection, treatment of systemic involvement and treatment of ocular involvement. Prevention All domestic dogs and cat should be dewormed from time to time. Domesticated puppies should be toilet trained. This reduces chances of contamination of food. Pica in the child should be managed in consultation with pediatrician. Deworming of children has doubtful role. Treatment of visceral larval migrans is systemic administration of anti helmenthic drugs like thiabendazole, albendazol, Dead worm may cause systemic allergic reaction. Treatment of ocular involvement: There is no medical treatment that can treat larval granuloma. Curiously by the time ocular involvement takes place systemic signs and symptoms subside with normal WBC count, however Elisa remains positive. Local treatment comprises management of uvetitis with cycloplegic and local steroid mostly peri-ocular depot injection. Surgical treatment Parsplana vitrectomy removes larval antigen cuts posterior cyclitic membrane and releases traction vitreo retinal bands that may cause traction or rhegmatogenous retinal detachment. Retinal detachment in treated by sclera buckling. Over all visual results are poor.
Features Age of Presentation Equal in both Unilateral 70% Size of Globe Ciliary Process Birth Weight Retinoblastoma Bilateral Unilateral-18 Months 12 At Birth Equal in both Bilateral 30% Equal in both Unilateral 90% males Bilateral Normal Not visible Low less than 1500gm Laterality Unilateral Normal Not Visible Normal P.H.P.V. 10 Weeks post Na- tal First decade may R.O.P.M. Coats Disease be seen in infancy Sex

Initial Normal Microphthalmn Later buphthalmic Not Visible Normal Visible Normal

RETINOBLASTOMA Prematurity Effect of Oxygen administration Heredity Development of Retrolentalmass X-ray U.S.G No Not Significant No Not Significant Premature Significant Not significant 2-3 Months Not significant, Non Specific No

491

Not Significant Not Significant Late Not Significant Exudative retinal detachment

May be Significant be Not Significant 1012 Months Intra Ocular Classification At birth Not Significant, Non Specific

Tumour arising May show mass in Mass in anterior from retina that is vitreous viterous acoustically solid, high internal reflection, may show calcification Tumour appears as No significance solid mass with in globe, areas of calcification that has density of bone No significance

CT

Most important investigation in opaque media it delineates intra ocular morphology

INTRA OCULAR GROWTH OTHER THAN RETINOBLASTOMA THAT CAUSE WHITE REFLEX IN PUPILLARY AREA
They can be A. Malignant 1. Meduloepithelioma 2. Leukaemia 3. Glioneuroma B. Benign (Hamartomas) 1. Astrocytoma 2. Haemangiomas (a) Capillary hemangioma (b) Cavernous hemangioma C. Phacomas A. Malignant 1. Meduloepithilioma See page 284. 2. Leukemia40

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PEDIATRIC OPHTHALMOLOGY

Ocular signs are never presenting features of leukaemia. Intraocular involvement is late. It is generally seen as recurrence after initial remission and always have poor prognosis. All the intra ocular structures that have blood vessels in them i.e. uvea, retina and optic disc may be involved. The vitreous may get seeded by leukaemia infiltration. A pseudohypopyon may also develop. Infiltration of optic nerve is always associated with vision loss which is not recovered following apparent cure by radiation or chemotherapy. Infiltration of iris and ciliary body may be mistaken as anterior uveitis specially with pseudohypopyon. There may be localised or diffuse infiltration of choroid. As most of the children with leukaemia are immune compromised either due to malignancy or chemophathapy or both it is difficult to state if the uveal lesion is due to malignant infiltration or due to opportunistic organism. The controversy can be settled by fine needle biopsy of the lesion. The fundus lesions of the retina may be due to 1. Direct infiltration of retina or optic nerve or 2. Due to associated anaemia and thrombo cytopenia. The hemato logical retinal lesions are superficial linear or flame shaped haemorrhages. Superficial haemorrhage with white centres are due to anaemia and thrombo cytopenia. Other lesions may be non rhegmatogenous retinal detachment, retinal pigment epithelial detachment. Management. Treatment is essentially directed to systemic involvement, local external beam radiation to eye results in regression but improvement of vision is not always predictable. 3. Glioneuroma. This is very rare tumour, arises from anterior lip of the optic cup, unilateral involves anterior sigment, produces white reflex but does not involve posterior sigments. B. Benign (Hamartomas) 1. Retinal astrocytoma41,42,43. Retinal astrocytoma is a rare hamartoma of the retina seen in children they are non invasive and non malignant, generally stationary they may be solitary or multicentric. Unilateral lesions are frequent. Commonest lesion is a white superficial, raised lesion little larger than disc. On fluroscein angiography it has slow filling, late staining. It does not require any treatment. The condition is most commonly seen is tuberous sclerosis that has multiple systemic involvements. 2. Retinal haemangiomass41,42,43,44 (a) Retinal capillary haemangioma These rare vascular tumours that share autosomal dominant trait with retinoblastoma. They are tumours of aduldt hood may become apparent in childhood. They also arise from sensory retina and are multicentric may be bilateral. The similarity with retinoblastoma ends here. The difference are: their colour, afferent and efferent vessels, sub retinal and inter retinal exudale. They never metastasise. These growths are haemartomas that may be seen on the retina as well as optic nerve head. They may be confined to eye only or may have systemic involvement in twenty five percent cases and is known as vonHippels disease In the intra ocular growth may be uniocular

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or bilateral may be single growth or there may be more than one growth of different size and duration multiple growths have poorer visual out come. The single haemangioma of the retina is discovered on routine retinoscopy and fundus examination before retinal detachment sets in. The detached retina may produce a white reflex in pupillary area and draw attention. The characteristics of retinal capillary haemangioma are : Spherical , raised, bright red growth with afferent vesseles and efferent vessles. The growth generally are one to two disc diameter in size. Both the feeding and draining vessles are dilated and tortous. Larger growths have larger vessels. There may be inter or sub retinal exudates with macular star formation. These exudates may give a pale reflex during retinoscopy. The exudate cause retinal detachment that may be partial or total. Retinal detachment results in white reflex in pupillary area. Secondary glaucoma and iris neovascularisation are some of the complications. Diagnosis is confirmed by : Indirect ophthalmoscopy, Bscan, CT. MRI and fundus fluorescing angio graphy. Fundus fluorescing angio graphy is most important diagnostic procedure in absence of total detachment. Angiography shows rapid filling of the entire vessels and the tumour, hyper fluorescence of the growth, rapid filling of the draining channels. If is leak from the tumour it may colour the vitreous. Management small peripheral tumours need not be treated but observed periodically for enlargement in size, development of internal and subretinal exudates, retinal detachment, if any of above manifest, the lesion requires treatment. The best mode of treatment is by photo coagulation, peripheral lesion many be treated by cryotherapy. Diathermy has been given up in favour of former two. There is difference of opinion if (i) the growth along with its feeding vessels45 should be treated (ii) only the vessels be obliterated43 and hope that the growth will shrink (iii) the tumour only should be treated42. The best results are claimed to be by Argon laser with large spot size, longer duration and low intensity. The tumour should not be treated in one session. It should be treated by several sittings spread over months. The retina around the dilated vessels and angioma may require scattered photo coagulation to prevent exudation. (b) Cavernous hemangioma of the retina41, 44 This is a rare congenital malformation of retinal vasculature , this is a hamartoma, non progressive, non malignant growth may have positive family history generally uniocular and single. Situated behind the equator either in the retina or on the optic nerve head. It is slowly progressive generally symptom less, diagnosed on routine fundus examination in late teens or early third decade. The retinal growths are inter retinal in nature with slight elevation of the ritual surface. The single growth is in fact a cluster of many smaller thin walled sacular micro aneurysm. The over all size of the growth varies between 2-4 mm in diameter there may be few micro aneurismal

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dilatation in the surrounding area. The surface of the retina invariably shows moderate glial proliferation over the growth that may be confused with superficial exudate. There is no arterial dilatation in contrast to capillary haemangioma. Fluroscein angiograpthy is diagnostic, there is slow filling of the lesion, each saccule accumulates fluorescing individually and remains fluorescent for long time. As the lesion is non progressive and does not have symptoms it does not require any treatment.

REFERENCES
1. Abramson D.H. ; Retinoblastoma - Diagnosis and management, Cancer, 32-130-140, 1982. 2. Biswas J. ; Intra ocular tumours, In Modern Ophthalmology, 1st Edition, pp. 650 669, Edited by Dutta L.C., Jay Pee brothers, new Delhi, 1994. 3. Boger W.P. Peterson.R.A. ; Tumours of childhood in Manual of ocular diagnosis and therapy. Fifth edition edited by Deborah Pavan Langston p- 318-320, Lippin cott William and Wilkins 2002. 4. Augsburger J.J. Yanoft. M. ; Retinoblastoma in Text book of ophthalmology Vol318. pp. 18.118.14, Edited by Podos S.M. and Yanoff.M. Mosby London 1991 . 5. Muhprea A.L., Christensen L.E. ; Retinoblastoma in Current ocular therapy Edition 5, p-262-263, Edited by Fraunfelder F T and Roy F H. W B saunders company Philadelphia 2000. 6. Kanski J.J. ; Tumours of uvea and retina in Clinical ophthalmology, Second Edition pp. 401407 , Butter worth London 1989. 7. Puklin J.E. Apple D.J. ; Retinoblastoma and leucokoria in Principles and practice of ophthalmology Vol. 2 First Indian edition pp. 11361182, Edited by Peyman G.A. Sander D.R. and Goldberg M.F. Jay Pee Brothers New Delhi 1987. 8. Asdourin G.K. ; Retinoblastoma in Manual of Clinical Problem in Ophthalmology first edition p-125-128 Edited by Gittinger J.W. and Asdouria G.K. Little Brown Boston 1998. 9. Shields C.L. ; Retinoblastoma in Ophthalmology Secrets First Indian Edition pp. 322326, Edited by Vander J.F. and Gault J.A. Jay Pee brothers, New Delhi 1998. 10. Ellsworth R.M. ; The practical management of retinoblastoma, Trans Am. Oph So 67: 462-534 1969. 11. DePotter P. ; Orbital imaging in Ophthalmology secrets first Indian edition pp. 231 233, Edited by Vander J.F. and Gault J.A. Jay Pee brothers, New Delhi 1998. 12. Shields J.A. ; Augs burger J.J.,Current approach to the diagnosis and management of retinoblastoma surv. ophth. 25: 347-372 1981. 13. Augstburger J.J., Yanoft. M. ; Benign tumours in the differential diagnosis of retinoblastoma in Text book of ophthalmology vol. 18. pp. 19.119.11, Edited by Podos SM. and Yanoff M. Mosby, London 1991.

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14. Augosburger J.J., Yanoft. M. ; Malignant tumours in the differential diagnosis of retinoblastoma in Text book of ophthalmology vol.18 pp. 20.120.5 edited by Podos SM and Yanoft M mosby, London. 15. Ratnakar K.S. ; Retinoblastoma in Pathology of the eye and orbit first edition pp224-230 Jay Pee brothers New Delhi 1997. 16. Biswas J. and Dutta Nitin ; Retinoblastoma in Modern Ophthalmology Vol. 2 Second Edition, pp-750, Edited by Dutta L.C. Jay Pee Brother , New Delhi- 2000. 17. Ellsworth R.M. and Notic C.M. ; Retinoblastoma in Current ocular therapy , Edition 4, Edited by Fraunfelder F.T. Roy H.F. pp. 355357, W B Saunders Co Philadelphia 1995. 18. Desai V.N. Duker J.S. ; Masquerade Syndromes in Ophthalmology Secrets Edited by Vander J.F. and Gault J.A. First Indian Edition pp. 263266 Jay Pee brothers , New Delhi 1998. 19. Resse A.B., Ellsworth. R.M. ; The evaluation and current concept of retinoblastoma therapy. Tran, Am Acd Oph Oto 67: 164-172 1963. 20. Campbell J.R., Sobin L, Zimmerman L.E. ; The T.N.M. Classification of malignant tumours of the eye and ocular adnexa, Am Jr. Oph. 100:83-84 1985. 21. Donahue S.P. ; Retinopathy of prematurity, Editorial B.J.O. 86 pp. 1071 2002 22. McNamara J.A. Tasman W. ; Retinopathy of prematurity Oph Cli Nor Am, 3: 4134271990. 23. G.E. Quinn ; What do you do about ROP screening in big babies B.J.O. 86: 1072-1073 2002. 24. McNamara J.A. ; Retinopathy of prematurity in ophthalmology secrets edited by Vander J.F Gault J.A. pp. 294299, Jay Pee Brothers , New Delhi 1998. 25. Natrajan S. and Dutta B. ; Retinopathy of prematurity in Modern ophthalmology , Vol. 2, second edition, edited by Dutta L.C. pp. 641649, Jay Pee brothers, New Delhi 2002. 26. Committee for the classification of retinopathy of prematurity, An international classification of retinopathy of prematurity, Arch Oph 105: 906 1984 27. Perterson R.A. and Boger III W P ; Retinopathy of prematurity in Manual of ocular diagnosis and therapy, 5th Edition pp. 32324, Edited Deborah Pavan Langston, Lippincot Williams and Wilkins Philadelphia 2002. 28. Antoszyk, J.K. Antoszy K. A.A. ; Persistent Primary hyperplastic viterous in Current ocular therapy, Edition 5, edited by Fraunfelder F.T. and Roy F.H pp. 642644, W.B. Saunders company Philadelphia 2000. 29. Federman J.L., Shields J.A., Altman B, Koller H. ; The surgical and non surgical management of persistent hyperplastic primary vitreous ophthalmology 89 : 20-24 1982. 30. Augsburger J.A. and Myron Yanoff ; Retinoblastoma in Test book of Ophthalmology Vol.18 pp. 18.118.14, Edited bt Podos S.M. and Yanoft M. Mosby London 1991. 31. Shield A.J. Stephens R.E., Sarin L.K. ; The differential diagnosis of retinoblastoma in Paediatric Ophthalmology vol. 2, Second Edition edited by Harley R.D. pp. 1144 1157, W B Saunders Comp Philadelphia 1983

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32. Wu Lihtech and Murphy R.P. ; Coats disease in Current ocular Therapy edition 5, Edited by Fraun Felder F T and Roy F H. pp. 607608, W B Saunders Company Philadelphia 2000 33. Tasman W. ; Coats Disease in Current ocular therapy edition 4 , Edited by Fraunfelder F T and Roy F H. pp. 777778, W B Saunder Company Philadelphia 1995 34. Ridley M Shields J A Brown J.C. ; Coats Disease evaluation of management Ophthalmology 89: 1381-1384 1982. 35. Shileds J.A. Stephens R.F, Sarin L.K. ; Differential diagnosis of retinoblastoma in Paediatric ophthalmology Vol. 2, Second Edition edited by Harley R.D. pp. 11451147, W B Saunders Company Philadelphia 1983. 36. Greer C. H. ; Ocular Infestation by toxocara in Ocular pathology first edition pp. 54 55, Black Well Scientific Publication Oxford 1963 37. Kanski J.J. ; Uveitis : In Clinical ophthalmology, Second Edition 157-159, Butter worth, London 1989. 38. Gillespie , S H. ; Ocular toxoariasis in Current Ocular Therapy, Edition 5 pp. 9798, Edited by Fraunfelder F H and Roy F H, W B Saunders Company Philadelphia 2000. 39. Gillespic S H Dinning W J Voller ; A Crow crowft N. S.,The spectrum of ocular toxocariasis Eye 7 : 415-418 1993. 40. Augsberger J. J and Yanoft M. ; Malignant Tumours in the differential diagnosis of retinoblastoma in Text book of ophthalmology, Edited by Podo S.M. and Yanoft M. pp. 20.220.5, Vol. 18. Mosby London 1991. 41. Augsberger J J and Yanoff M. ; Benign tumours in the differential diagnosis of retinoblastoma in Text book of ophthalmology edited by Podos S. M. and Yanoff M pp. 19.119.11, Vol. 18. Mosby London 1991. 42. Kanski J J. ; Tumours of uvea and retina in Clinical ophthalmology , Second Edition pp. 405409, Buffer worth, London 1989 43. Shields J.A. Stephens R F and Sarin L. K. ; The differential diagnosis of retinoblastoma in Paediatric Ophthalmology, Vol. 2 Second Edition, Edited by Harley R.D pp. 1144 1157, W B Saunders comp Philadelphia 1983.

CHAPTER

13

Disorders of Pupil, Accommodation and Convergence in Children


GENERAL CONSIDERATION
The choroid and ciliary body are placed parallel to the sclera. The iris is placed at right angles to the sclera at the level of scleral spur like a curtain that divides the aqueous chamber into two unequal parts i.e. a large anterior chamber and a smaller posterior chamber. The two chambers communicate through a natural defect in the centre of the iris called the pupil. The pupil though for all practical purposes is central, in fact is slightly decentered medially and inferiorly with in a physiological limit. When the position of the pupil exceeds the physiological limits, the condition is called corectopia1 or ectopic pupil, which generally occurs as congenital anomaly, trauma or post inflammatory status. In rare instances of congenital anomaly there may be more than one pupil in the same eye. This is known as polycoria.1 A true polycoria should have its own constrictor and dilator muscles independent of main pupil and should react independently. In contrast to this, pseudo polycoria is a condition where there is a hole in the iris without separate constrictor or dilator muscle and the hole does not react to light, or near reflex. Common causes are congenital anomaly of iris and anterior chamber, trauma, accidental or surgical or post inflammatory status. The function of the pupil is mostly optical. It regulates the light reaching the retina. The pupil constricts in presence of bright light and dilates in darkness. The other function is to cut the spherical aberration and chromatic aberration and mildly increases the depth of focus. Depth of focus is inversely proportionate to diameter of pupil. Larger the pupil lesser is the depth of focus. Besides optical purpose, the pupil regulates passage of aqueous from posterior chamber to anterior chamber. A very small pupil may cause pupillary block resulting into rise of intraocular tension. If the pupil is bound down to the structures behind it, as happens in seclusio pupillae, the iris may bulge forward producing iris bombe. The size of pupil varies from person to person. Generally in an adult it is about 2.5 mm in diameter. It is small in extremes of ages i.e. pupil of a new born is smaller than an adult. The pupil in myopia is larger and smaller in hypermetropia in comparison to emmetropia. The most commonly given reason is that a pupil constricts when the eye accommodates, a 497

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myopic eye requires very little accommodation. In contrast to this, the hypermetropic eye requires more accommodation hence the pupil is smaller in hypermetropia and larger in myopia. The size of pupil is equal and shape symmetrical in two eyes in 80% of persons. Only in remaining, the size of the pupil differs in two eyes. This is called anisocoria. Anisocoria is said to be simple or essential when the size of any of the pupil does not change due to change in illumination. If the size of the pupil changes with illumination it means a pathological phenomenon. In such cases it must be decided, which pupil, the larger or the smaller is pathological.2 For example, in Horners syndrome the degree of anisocoria is most marked in dim illumination.4 However in case of unilateral visual defect size of the pupil does not change irrespective of opacity in media, or pathology of retina or optic nerve. The size of the pupil depends on a delicate balance between the potency of constrictor and dilator muscles. Constrictor muscle has an enhanced tone than dilator more so in childhood and irritation of parasympathetic as seen in iridocyclitis. The pupil of a child is more resistant to mydriatic than that of an adult. A light coloured eye has a larger pupil that dilated better than a dark coloured iris. The neural control of constrictor pupillae The constrictor of the pupil is called the sphincter pupillae.5 It is ectodermal in origin and is placed in a circular fashion round the pupil. It is supplied by third cranial nerve via its parasympathetic system. The fibres start in the Edinger-Westphal nucleus and pass in the third nerve up to orbit where the third nerve divides into two branches. The lower branch supplies the inferior oblique. After leaving the inferior oblique, the nerve enters the ciliary ganglion as its motor root. From the ciliary ganglion start the post ganglionic fibres that pass along the short ciliary nerve and enter the sclera around the optic nerve. Once the post ganglionic fibres enter the sclera, they pass in the supra choroidal space and terminate in the iris and ciliary body. Ninety percent of the fibres go to the ciliary body and only three percent to the iris, remaining supply the blood vessels.6 The neuro transmitters released at neuro muscular junction is acetylcholine. Neural control of dilator pupillae The dilator pupillae is also ectodermal in origin, it is arranged in radial fashion away from the pupillary margin. In comparison to constrictor it is a poorer muscle. The dilator muscle is differentiated later than sphincter and continues to develop even after birth. This may be the cause of smaller pupil in neonates. The dilator pupillae is supplied by cervical sympathetic. The post ganglion fibres from superior cervical ganglion accompany the internal carotid artery and then pass along the first division of the trigeminal nerve and emerge from the cavernous sinus with nasociliary nerve and long ciliary nerve to the iris. The neuro transmitter released at neuro muscular junction is nor epinephrine. The pupil is kinetic indicator of functional state of iris, optic nerve, third cranial nerve and ocular sympathetic system. The size of the pupil keeps on fluctuating with change in intensity of illumination, accommodation, convergence and integrity of parasympathetic and sympathetic path. Part of the parasympathetic path is common with visual path.

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The two neural paths involved in pupillary reaction are 1. Light reflex 2. Near reflex (a) Accommodation (b) Convergence (c) Miosis The pupillary light reflex is a four-neurone arc with an afferent and an efferent component. The former spreads from retina to Edinger Weshphal nucleus and latter from Edinger Westphal nucleus to the iris. The first neuron extends from rods and cones to pretectal nucleus in the mid brain. The fibres from the temporal half go straight into the optic tract without decussation. The fibres from nasal retina decussate at the chiasma in the same manner as visual pathway. The crossed and uncrossed fibres travel in the optic tract up to its posterior third where they leave the visual path and turn to pretectal nucleus. The second neuron connects pretectal nucleus on each side to the Edinger Westphal nuclei. The neurones that join the pretectal nucleus to the Edinger Westphal nucleus are called inter nuncial neurons. A decussation take place in the mid brain before entering the Edinger Westphal nucleus. This decussation explains consensual light reflex in the other eye when light is shown in one eye. In normal eye, the consensual light reflex is equal and symmetrical to direct reaction. The third neuron spreads from Edinger Westphal nucleus to ciliary ganglion. The para sympathetic fibres change their relative position in third nerve during its course. Between the, mid brain and the cavernous sinus, the pupillo motor fibres are superficial in the nerve and are liable for a compressive lesion. Fortunately these lesion are less common in children. In the cavernous sinus and beyond, the pupillo motor fibres become central hence are not affected by mass lesion even when the third nerve is paralysed. In the orbit, the pupillomotor fibres reach the ciliary ganglion via nerve to inferior oblique. The fourth neuron leaves the ciliary ganglion and accompanies the short ciliary nerve to reach the sphincter pupillae. The whole of the pupillary light reflex is subcortical.

NEAR REFLEX
This is not a true reflex. It is better called near synkinesis of accommodation, convergence and miosis.7 In contrast to pupillary light reflex where Edinger Westphal nucleus acts as nucleus for pupillary light reflex, there is no definite nucleus for near reflex. These probably are supra nuclear areas one each in frontal and occipital lobe that mediate the reflex. The various components of the synkinesis can be modified either by optical device i.e. lens/prism or therapeutically. The accommodation can be relaxed by plus lenses while it can be enhanced by minus lenses. The convergence can be neutralised by base out prism and enhanced by base in prism. The miosis is overcome by mydriatic. Cycloplegic will abolish both accommodation and miosis in normal eye. The reflex can be initiated either by accommodation

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or convergence. Vision is not needed for near reflex. It can be elicited in blind eyes if the patient is made aware of a near object like his own index finger and asked to look at it when kept near the eye. There is no condition where light reflex is present but near reflex is absent.2,4,7 The near reflex path is also parasympathetic in nature. The near reflex starts in visual cells of retina. It follows the same path as light reflex up to the posterior third of the optic tract where the pupillary light reflex fibres leave the tract to join the pretectal nucleus. The near reflex fibres continue with visual fibres along the optic radiation to the occipital cortex area17,18. Here the visual fibres terminate. The near reflex fibres continue to the frontal occulomotor centre where they become part of a mass reflex that is mediated through cortico-tectal tract to the occulomotor nuclei. The efferent path starts from the Edinger Westphal nucleus and follows the third nerve like pupillary light reflex. Convergence reflex can not be separated from other two synkinesises i.e. accommodation and miosis. Convergence enhances both accommodation and miosis. Miosis due to bright light does not influence accommodation or convergence. However accommodation strengthens convergence and increases miosis. The reflex arc for convergence starts from medial rectus and follows the third nerve up to third nerve nucleus complex. The Perlias nucleus is supposed to control the convergence. The efferent path extends from the Perlias nucleus to the ciliary ganglion and its post ganglionic fibres. Other pupillary reflexes The other pupillary reflexes are not associated with either light or near reflex. They are Orbicularis reflex Vestibular and cochlear reflex Trigeminal reflex Vagotonic reflex Psychosensory reflex. The orbicularis reflex is constriction of pupil on the side of forceful contraction of orbicularis against resistance. It is present in about 80% of persons. The exact path of the mechanism is not known. The vestibular and cochlear reflex have little value in ophthalmic evaluation. They represent caloric, acoustic and rotational sensation from vestibular and cochlear area in the mid brain. The trigeminal reflex - On touching the cornea, there is momentary mydriasis followed by miosis. The exact path of the reflex is not known. It is presumed that the afferent trigeminal fibres also stimulate the third nerve via mid brain. The vagotonic reflex consists of mild dilatation of pupil on inspiration and slight constriction on expiration.

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The psychosomatic reflexThis represents an overall sympathetic over action. Hence the pupil are dilated in surprise fear, anxiety and hysteria. Effect of drugs on pupil and accommodation5,8,10 The drugs that affect the pupil and accommodation can be 1. Peripheral (ocular) action 2. Centrally acting. The drugs acting on the intraocular muscles act via autonomic nervous system. They are 1. Cholinergic 2. Adrenergic A. B. A. B. Agonist Antagonist Agonist Antagonist Miotic and cyclotonic Mydriatic, cycloplegic Mydriatic Miotic

The cholinergic agonists are commonly known as parasympathomimetic drugs, while the cholinergic antagonists are commonly known as para-sympatholytic drugs. The parasympathomimetic drugsMiotics can be 1. Direct stimulators of cholenergic receptorsPilocarpine, methacholine. 2. Drugs causing release of acetylcholine at nerve endingCarbachol. 3. Drugs conserving acetylcholine, Anticholine esterase drugs. (I) Short actingPhysostigmine, neostigmine, pyridostigmine, edrophonium (Tensilon) ( I I ) Long actingDFP Echothiophate, demecarium. All the above drugs are miotics and cyclotonics. Some of them are miotics on local instillation i.e. pilocarpine, methacholine, carbachol, but may have systemic side effect. Indirectly acting drug i.e. physostigmine, DFP, echothiophate and demecarium are also locally acting drugs which are no more in use. Edrophonium (Tensilon) has no therapeutic use, it is used to diagnose myasthenia gravis. Pyridostigmine and neostigmine are used to treat myasthenia gravis. Other two miotic drugs used in diagnosis of pupillary abnormalities are - Pilocarpine 0.125% and methacholine 2.5%. They do not constrict normal pupil in such low strength but constrict pupil in Adies pupil. Other miotics These are 1. Locally acting sympatholytic drugs They are A. Those that block alpha repectors i.e. thymoxamine, phentolamine and priscoline. (These drugs are rarely used) B . Those that block sympathetic neuron Guanethidine (These also have very limited use in adults only).

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These are prostaglandines and histamine. They do not have any therapeutic use. 3. Centrally acting These are central depressents that reduce supra nuclear inhibition on third nerve i.e. morphine and barbiturates. Mydriatics and Cycloplegics Mydriatics are the drugs that dilate the pupil. They are not cycloplegics. They can be locally acting or centrally acting. Cycloplegics are the drugs that paralyse ciliary body and abolish accommodation. They are mydriatics as well. They can be locally acting or centrally acting. Mydriatics and cycloplegics can be put into two broad groups i.e. 1. Cholinergic antagonistsCycloplegic cum mydriatic. 2. AdrenergicAgonistMydriatic. 1. Cholinergic antagonistThese are also known as parasympatholytic drugs. They are A. Block cholinergic receptors (Atropine, home atropine, cyclopentolate, tropicamide, scopolamine and eucatropine. The last two are no more used in clinical practice. B . Drugs that block parasympathetic nerve ending i.e. Botulium toxin. 2. Adrenergic agonist - These are also known as sympathomimetic drugs. They are (a) Directly stimulating alpha receptorsEpinephrine, phenyle-pherine, nor adrenaline. The last has no therapeutic use. (b) Drugs that release nor adrenaline from nerve ending - Ephidrine and hydroxyamphetamine. They do not have much therapeutic use. Hydroxyamphetamine (Paredrine 1%) is used to diagnose level of lesion in Horners syndrome. Cocaine is a sympathomimetic drug that blocks the re uptake of nor adrenaline at nerve ending is a good mydriatic and local anaesthetic, is no more in use. It has synergistic action with parasympatholitic drugs. It is exclusively used to diagnose Horners syndrome. Cocaine 4% will dilate normal pupil but not Horners pupil. Abnormal pupillary reaction 11 Abnormality of pupillary reaction can be abnormality of light reflex or abnormality of near reflex. Or there may be a dissociation of light near reflex. The abnormalities can be in:Afferent path of third nerve Efferent path of third nerve Oculosympathetic chain The anomalies are either neurological or pharmacological.

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Afferent pupillary defect are 1. Total afferent pupillary defect. 2. Relative afferent pupillary defect. 3. Wernickes hemianopic pupil. 4. Argyll Robertson pupil The characteristics of afferent pupillary defects are 1. The lesion may be anywhere from retinal visual cells to the pretectal nucleus. 2. There is decreased amplitude of contraction to light stimulus. 3. The latency in reaction to light stimulus is increased. 4. The pupil dilates on prolonged exposure to light. 5. Both the direct and consensual reflexes are involved. 6. The afferent pupillary light reflex can be present in blind eye if the cause of blindness is retro chiasmal. While testing pupillary light reflex, it should be ensured that the accommodation is at rest. The room is dimly lit and the source of light is small, sharp and bright. Total afferent pupillary defect (TAPD) This is also known as amaurotic pupil.11 The lesion causing the defect is unilateral either as an extensive damage to the retina or complete loss of conduction in the optic nerve anywhere from disc to the chiasma. There is no perception of light in the affected eye. Both the pupil are of same size. In case of bilateral blindness due to pre geniculate lesion, the pupil tend to be larger than normal. When light is thrown in the pupil of the blind eye neither pupil react i.e. there is absent direct pupillary reaction in the affected eye and absent indirect reaction on the contra lateral side. When normal eye is stimulated by light, both the pupil react. Any amount of opacity in media is not capable of producing total afferent pupillary defect. Relative afferent pupillary defect (RAPD) This happens when the affected eye has some vision. The cause of which is located in anterior visual path i.e. extensive retinal lesion or lesion in optic nerve. There is asymmetric conduction defect. If both eyes have diminished vision, the eye with poorer vision develops relative afferent defect. The difference between the conduction in two eyes can be estimated with use of neutral density filter. The condition is also called Marcus Gunn pupil and is demonstrated by swinging flash light test. Swinging flash light test consists of following steps :11,12 1. Vision in both eyes is noted with and without correction separately. 2. The room is semi-darkened. 3. The patient is asked to fix at a distant object. 4. Size of the pupil is noted in diffused light. 5. Direct and indirect light reflex are noted. 6. The eye withless vision, larger pupil and sluggish direct light reaction in the eye effected.

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7. Throw bright well-focussed light in one eye and note the reaction. 8. Quickly shift the light to the other eye and note the reaction. 9. Repeat above steps by moving the light from one eye to the other and note the reaction. Interpretation 1. In normal eye both the pupil constrict equally and symmetrically i.e. isocoria. 2. In presence of RAPD, the affected pupil shows reduced amplitude of miosis and enhanced dilatation. The swinging flash light test is positive when only afferent pre-chiasmal conduction defect is present and efferent arc is intact. A modified and less sensitive test is 1. Occlude the normal eye for sometime, observe the affected eye. The pupil of unexposed but affected eye will be larger than before the normal eye was occluded. 2. Occlude the affected eye in same fashion. The pupil of the non occluded normal eye will be smaller. The causes of RAPD are 1. Optic neuritis 2. Optic atrophy 3. Retro bulbar neuritis 4. Traumatic optic neuropathy 5. Advanced glaucomatous optic atrophy 6. Advanced and extensive retinal disease Pupillary reaction in mid chiasmal lesion The nasal fibres of retina decussate at the middle of the chiasma, the temporal fibres pass without decussation. Hence in a mid chiasmal lesion, light directed from temporal field produce less pupillary constriction than light directed from nasal field. The causes are pituitary tumour, cranio pharyngioma, tubercular meningitis and trauma. Pupillary reaction in anterior junctional lesion Anterior chiasma syndrome is caused due to a lesion involving one optic nerve and half of chiasma on the same side. The pupil when seen in diffuse light are slightly dilated and large. The pupillary reaction is a combination of Marcus Gunn pupil in one eye and chiasmatic hemianopia pupillary reaction in the other eye. Importance of this type of pupillary reaction lies in the fact that it is seen in craniopharygioma and parasellar extension of pituitary tumour which are common in children. Pupillary reaction in lesion of optic tract Lesions of anterior optic tract cause Wernikes hemianopic pupil. A lesion near the lateral geniculate body does not produce any pupillary change because the pupillary fibres leave the optic tract anterior to the junction of posterior 1/3 to anterior 2/3. Wernickes

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hemianopic pupil requires special efforts to demonstrate its presence. It is done by throwing a bright narrow beam of light from the field of hemianopia that produces less constriction than light thrown from the side of normal field. A bright beam from slit lamp give best result. The lesion is associated with incongruous homonymous field defect. It is seen in trauma to temporal lobe, abscess in temporal lobe and Schilders disease. A lesion near the end of the optic tract and pretectal area causes loss of light reflex with preserved accommodation. This is called light near dissociation.13 A classical example of light near dissociation is Argyll Robertson pupil due to neurosyphilis which is not seen in children but there are other causes of light near dissociation which are put collectively as pseudo Argyll Robertson pupil and are seen in children. The exact site of lesion in classical Argyll Robertson pupil is not known. Commonly agreed lesion is pretectal. In Argyll Robertson pupil accommodation reflex is retained while light reflex is lost but vision is maintained. Characteristics of Argyll Robertson are 1. It is a bilateral condition. 2. One eye is more involved than the other. 3. It is seen after third decade. 4. The pupil are miotic and irregular. 5. Pupillary light reflex is absent. 6. Pupil dilates poorly with atropine. 7. Accommodation reflex is present. 8. Vision is good. 9. Patches iris atrophy are present these may be mistaken as heterochromia. 10. Orbicularis reflex is often retained. 11. The cases are always associated with positive serological test for syphilis. Sometimes unilateral Argyll Robertson pupil like reaction has been reported for which the lesion has to be small that blocks input to only one Edinger Westphal nucleus. Inverse Argyll Robertson pupil is very rare where the pupillary light reflex is present with failure of pupil to near reflex is usually associated with failure to converge the eye. Most probable site of the lesion is Perlias nucleus in mid brain. Pseudo Argyll Robertson pupil In these condition, the pupil is generally normal in size or mildly dilated but light reflex is poor. There may be spasm of accommodation, diminished amplitude of accommodation. Other presenting signs may be lid retraction, ptosis, nuclear oculomotor palsy, convergence palsy, spasm of convergence, vertical gaze palsy and vertical nystagmus.12 Commonly seen in Parinauds syndrome, aqueductal stenosis and aberrant regeneration of third nerve. The list of conditions that cause light near dissociation are 1. Pseudo Argyll Robertson pupil. 2. Parinauds syndrome. 3. Aqueductal stenosis.

506 4. 5. 6. 7. Unilateral Argyll Robertson pupil. Aberrant regeneration of third nerve. Adie-Holmes pupil. Juvenile diabetes.

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Efferent pupillomotor defect Size of the pupil and its reaction to light is a delicate balance between parasympathetic and sympathetic system. The parasympathetic system is little more effective than its sympathetic counterpart. As the efferent path of the pupillary reaction is parasympathetic in nature, slightest under action results in relatively greater loss of pupillary and ciliary reflex. The efferent pupillomotor fibres may be involved anywhere between the third nerve nucleus and the eye. It is a peripheral lesion, generally unilateral. It may involve only the pupil but more common is internal ophthalmoplegia that is a combined paralysis of pupil and ciliary body. It is present in total third nerve palsy and in total ophthalmoplegia. However pupil is spared in diabetic third nerve. A third nerve lesion in cavernous sinus has normal sized pupil because it is also associated with involvement of oculosympathetic under action. The mydriasis of third nerve palsy in cavernous sinus is counteracted by miosis of sympathetic, resulting in normal sized pupil in cavernous sinus disease. Before diagnosing efferent pupillary defect, it is essential to rule out 1. Congenital anomaly of pupil 2. Trauma to iris 3. Instillation of mydriatic and cycloplegic in the eye Pharmacological mydriasis and cycloplegia is not counteracted by instillation of pilocarpine but a neurological mydriasis will respond to pilocarpine. 1. The causes in children may be in the mid brain i.e. Pineal tumour, encephalitis, brain abscess, trauma. 2. Oculomotor nerve 3. Ciliary ganglionAdies pupil 4. Short ciliary nerveTrauma. In total oculomotor nerve palsy the eyeball is deviated down and out with ptosis and dilated pupil that does not react either to light or accommodation, convergence is also absent. Hutchinsons pupil This is a common pupillary defect due to compression of third nerve due to an expanding subtentorial or subdural lesion. Commonly seen in head injury, there is always lowering of consciousness with raised intracranial pressure. The mechanism consists of raised intracranial pressure that compresses the third nerve either against the pteroclinoid ligament or dorsum sella causing progressive herniation of brain, resulting acute internal ophthalmoplegia, which is an ominous sign following head injury requiring early surgical intervention. Classical Hutchinsons pupil consists of 1. Initial homelateral miosis due to irritation of third nerve. 2. Followed by moderate dilatation of same pupil with sluggish light reflex and convergence.

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3. 4. 5. 6.

Dilatation of pupil with loss of light and convergence Signs of third nerve palsy. Miosis of contra lateral pupil. Bilateral dilated pupil.

Adies pupil (Tonic pupil) This is an efferent disorder of pupil with light near dissociation. It is caused by denervation of post ganglionic constrictor fibres and ciliary muscle. Most probable cause is presumed to be viral in origin. In 80% cases, it is unilateral generally seen in women between 20-40 years. It can be seen less frequently in children. If the tonic pupil in a child is hypersensitive to methacholine, the child has familial dysautonomia i.e. Riley Day syndrome. Characteristics of Adies pupil 1. The pupil is large and circular. 2. The pupil when examined in dim light after exposure to usual light is smaller. 3. Light reflex in affected pupil is either absent or very slow or is localised as vermiform movement of a sector of iris when seen on slit lamp. 4. Near reflex miosis is slow so is redilatation on distant vision. 5. Accommodation is low, amplitude of accommodation is less, relaxation of accommodation is poor resulting in to temporary blurred vision for distance following near work. 6. Consensual reflex is intact. 7. Vision is normal. 8. The pupil is hypersensitive to parasympathomimetic drops. Normal pupil does not constrict following instillation of 0.125% of pilocarpine or 2% methacholine. In Adies pupil the pupil constricts to both the drugs in 30 minutes. Horners pupil 12,15,16 The pupillary anomaly is part of a syndrome which has following characteristics 1. It is mostly acquired but may be congenital or may be due to a birth trauma. 2. It is generally unilateral. 3. There is mild ptosis not more than 2 mm due to paralysis of Mullers muscles. There is no change in superior rectus. 4. Upside down ptosis i.e. slight upward shift of lower lid margin due to weakness of inferior tarsal muscle. 5. Miosis due to unopposed action of sphincter pupillae. 6. Pupil is central, circular, reacts normally to light and accommodation. 7. Heterochromia is seen mostly in congenital cases. 8. Reduced sweating on the side on the lesion if the lesion is below the superior cervical ganglion. 9. The miosis is greater in dim light. 10. There is increased amplitude of accommodation.

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11. Hypotony may be present. 12. Enophthalmos 13. There is rise of skin temperature of the ipsilateral lid and face. 14. Brittle and dry hair Diagnosis Diagnosis is easy. A case of unilateral mild ptosis, miosis, enophthalmos with normal extraocular muscles, preserved light and near reflex is diagnostic. Diagnosis is confirmed, specially the site of lesion by pharmacological tests. 1. Cocaine test. The test confirms sympathetic denervation. Normal pupil will dilate with 4%-10% cocaine when instilled in the eye in about 30 minutes to 45 minutes. One drop of 4-10% cocaine is instilled in each eye. The drop is repeated after one minute and size of the pupil is noted after 30 to 45 minutes. In a positive test the affected pupil either does not dilate or dilates poorly irrespective of level of lesion. 2. Paredrine (Hydroxy amphetamine) - One percent paredrine will dilate the pupil only in pre ganglionic lesion and not in post ganglionic lesion. 3. Adrenaline test - Adrenaline 1:1000 is instilled in both eyes. (a) In normal eyes, pupil will not dilate. (b) In pre ganglionic lesion both pupil will not dilate. (c) Horners pupil will dilate with 1:1000 adrenaline. Phenylepherine in 1% drop does not dilate normal pupil but will dilate a pupil in post ganglionic Horners syndrome. In children, common causes of Horners syndrome arebirth trauma due to stretching of brachial plexus, mediastinal and neck neuroblastoma. Surgical or accidental trauma to mediastinum or neck may also cause the defect. Horners syndrome may be associated with cluster headache i.e. Raeders para-trigeminal syndrome.

REFERENCES
1. Duke Elder S. : Polycoria in System of Ophthalmology. Vol. III, Part II, pp. 592-596, Henry Kimpton, London, 1964. 2. Glasser J.S. : Essential anisocoria in Neurophthalmology. pp. 174 : Harper and Row, London, 1978. 3. Brain W. Russel : The pupil and the eye lids in Diseases of nervous system. Sixth edition, pp. 71-75, Oxford University Press, London, 1962. 4. Kanski J.J. : Abnormalities of the pupil in Clinical Ophthalmology. Second edition, pp. 472-475, Butterworth, London, 1989. 5. Seal S.K. : Pupillary pathways and reflexes in G.N. Seals Textbook of Ophthalmology. Fifth edition, pp. 21-25, Current Book International, Kolkata, 2002.

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6. Rosenberg M.A. : The pupil physiology and anatomy in Principles and practice of ophthalmology. Vol. III, First Indian edition, pp. 1943-1951, Edited by Peyman G.A., Sander D.R. and Goldberg M.F. Jay Pee Brothers, New Delhi, 1987. 7. Natchair G. Subhuram P. : Normal and abnormal pupils in Modern Ophthalmology. Vol. 2, Second edition, pp. 941-944, Jay Pee Brothers, New Delhi, 2000. 8. Duke Elder S. : Autonomic effectors in System of Ophthalmology. Vol. VII, pp. 535585, Henry Kimpton, London, 1962. 9. Garg A. : Mydriatics and cycloplegics in Textbook of ocular therapy. First edition, pp. 139-146, Jay Pee Brothers, New Delhi, 2001. 10. Garg A. : Miotics in Textbook of ocular therapy. First edition, pp. 78-85. Jay Pee Brothers, New Delhi, 2001. 11. Samant P.M., Hemalini Samant and Nabar S.M. : Abnormal pupillary reaction in Ophthalmology theory and practice. First edition, pp. 313-316. Bhalani Publishing House, Bombay, 2004. 12. Harley R.D. : Pretectal pupillary defects in Pediatric ophthalmology. Vol. II, Second edition, pp. 785-787, WB Saunders Company, Philadelphia, 1983. 13. Shirley H. Wray : Argyll Robertson pupil in Manual of ocular diagnosis and therapy. Third edition, pp. 357, Edited by Devorah Pavan Langston, Lippincot Williams and Wilkins, Philadelphia, 1991. 14. Dutta L.C. : Argyll Robertson pupil in Ophthalmology principle and practice. First edition, pp. 404. Current Book International, Calcutta, 1995. 15. Kanski J.J., Thomas D.J. and Holmes ; Adies pupil in The eye in systemic disease. Second edition, pp. 64, Butterworth - Heinnemann, London. 16. Laties A.M. : Horners syndrome in Textbook of Ophthalmology. Ninth edition, pp. 516, Edited by Scheic H.G. and Albert D.M., WB Saunders Company, Philadelphia, 1977. 17. Gittinger J.W. : Horners syndrome in Manual of clinical problems in Ophthalmology. First edition, pp. 190-192, edited by Gittinger J.W. and Asdourian G.K. Little Brown and Company, Boston, 1998.

CHAPTER

14

Disorders of Optic Nerve in Children


Optic nerve, the second cranial nerve is not a true nerve. It is a white matter tract of the brain1, 2, 3 that joins the ganglion cells of the retina to the chiasma. It does not have power to regenerate. Hence severance of optic nerve leaves permanent loss of its function. Optic nerve has five types of nerve fibres out of which presence of the first two i.e. the afferent visual and afferent pupillary fibres have been proved beyond doubt. Presence of efferent fibres to retina, photostatic and autonomic fibres have not been proved conclusively. Each optic nerve extends from the optic nerve head in the globe to the anterior part of the chiasma in the brain. The length of the optic nerve varies between 3.5 cm to 5.5 cm in average person. However there is large variation not only from person to person but may also be in two sides in the same person. It is comparatively short in children. It has been divided into following parts4 : 1. Intraocular 0.7 mmThe smallest part 2. Intra orbital 3.0 cmThe longest part 3. Intra canalicular 6.0 mm to 1.00 cm. 4. Intra cranial 1.0 cm The intraocular part extends from the surface of the optic nerve to the posterior end of scleral foramen. The visible part of the intraocular part is called the optic nerve head or papilla. The optic nerve head is a circular structure of about 1.5 mm width. In children it is smaller in size. It is not myelinated. It has pink appearance due to multiple fine capillaries on its surface. If the capillaries become less the papilla loses its colour and becomes pale. The disc of a new born is pale. There is an eccentric pale saucer shaped depression in the centre of the disc. This is called cup of the disc. From the bottom of the cup emerge the central retinal vessels. The floor of the cup may have blue dot like appearance. They represent the underlying lamina cribrosa. The intraocular part is divided into two parts i.e. pre lamilar and retro lamilar4. The retina stops short at the temporal side of the disc but the underlying choroid continues up to the disc margin resulting in a choroidal crescent in the form of a visible pigmentary crescent. If both choroid and retina fall short of the optic nerve head, the resulting pale crescent is called scleral crescent. The intraocular part passes backward through a vent in the sclera called scleral canal that is narrower anteriorly. The scleral canal is bridged by lamina cribrosa, a sieve like 510

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structure. Through the gaps of sieve pass the bundles of optic nerve fibres. The lamina is slightly curved backwards. The optic nerve anterior to the lamina cribrosa in non-myelinated. Myelation begins behind the lamina. The optic nerve is the last nerve in the body to be myelinated.3 Intraorbital part. The intra orbital part of the optic nerve is the longest part of the optic nerve. It extends from back of the globe to the anterior end of the optic canal. The optic nerve is not a straight structure, it has a downward bend in the anterior part and a medial bend in the posterior part. This curve prevents the optic nerve from being stretched. It becomes straight in extreme lateral gaze and proptosis. The intra orbital part lies in the centre of the muscle cone surrounded by the orbital fat. It is covered throughout, its intra orbital cover by dura, arachnoid and pia. These coverings are continuous with meninges of the brain. The dura is the outermost and the pia is the innermost layer. The dura is continuation of intra cranial dura that passes through the optic canal and on reaching the orbit divides into two, the outer becomes periorbita while the inner continues to travel on the optic nerve up to back of the sclera where it merges with the outer surface of the sclera. The arachnoid lies deeper to the dura. In between lies the sub dural space, unlike brain the sub dural space of the optic nerve is only a potential space. The arachnoid also blends with the sclera. The space between the arachnoid and the pia ends blindly at the posterior part of the sclera but continues intra cranially. The space is filled with cerebro spinal fluid. The central retinal artery and central retinal vein passes through the sub arachnoid space from beneath to get into the optic nerve, 10-12 mm behind the globe. During its course through the sub-arachnoid space, the vein may be pressed by the increased CSF pressure, resulting into edema of the disc. At the apex of the orbit, the nerve is surrounded by annulus of Zinn from which the four recti originate. The superior and medial recti at their origin are closely attached to the dural sheath of the optic nerve. Stretching of this attachment when the eye is moved causes pain in inflammation of the optic nerve. The ciliary ganglion lies lateral to the nerve almost in the middle of muscle cone. The lower division of oculomotor, the abducent, the nasociliary nerve, the sympathetic fibres and sometimes the ophthalmic veins lie between the nerve and the lateral rectus. The nerve is surrounded by the long ciliary nerves, short ciliary nerves and posterior ciliary arteries at the back of the sclera. Most posteriorly the nerve is crossed above by the ophthalmic artery. The intra canalicular part. This part is about 1 cm in length and is the immobile part of the nerve. It is well protected in the bony canal. The canal not only transmits optic nerve but also its meninges, ophthalmic artery, the sympathetic fibres. The ophthalmic artery has a close relation with the optic nerve. The ophthalmic artery crosses the nerve inferiorly in the dural sheath. The sphenoidal and posterior ethmoidal sinuses lie medial to the optic nerve in the canal. The nerve is separated from the sinuses by very thin bone, hence infection of the sinuses can infect the nerve with ease.

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The intra cranial part. The intra cranial part of optic nerve lies in the middle cranial fossa. It extends from the posterior end of the optic canal to the anteriolateral angle of optic chiasma. It measures about 1 cm. It is a flattened band, spear shaped in section with apex laterally. The dura and arachnoid that covers the optic nerve from back of the globe to the end of the optic canal stop short at the back of the optic canal. The intra cranial part is covered by pia only. The intra cranial part of optic nerve first lies on the diaphragma sella and then on the anterior part of the cavernous sinus. The internal carotid lies atfirst below the nerve and than lateral to the nerve. The opthalmic artery originates from the internal carotid under the nerve in the middle of the nerve. Blood supply of the optic nerve : The blood supply of the optic nerve is divided into supply to : 1. Optic nerve head 2. Intra orbital 3. Intra canalicular and 4. Intra cranial The main blood supply of the optic nerve is derived from : 1. Internal carotid via its branches 2. Anterior cerebral artery Blood supply to the optic nerve head is most important in relation to glaucoma. Blood supply to the optic nerve head5, 6. Blood supply of the optic nerve is derived from two sources(1) Retinal, (2) Ciliary. For description, blood supply of optic nerve head is divided into following groups : 1. Blood supply to the surface nerve fibres. 2. Blood supply to pre laminar and laminar region. 3. Blood supply to retro laminar area. The surface of the optic nerve gets its blood supply from branches of central retinal artery that anastomose with branches of the prelaminar area. The prelaminar and laminar area get blood supply from short posterior ciliary artery. The retro lamilar area gets its blood supply from both retinal and ciliary circulation. Development of Optic Nerve7, 8 The optic stalk is the precursor of the optic nerve. It joins the eye and the forebrain. The embryonic fissure that develops at the lower side of the optic cup also extends into the optic stalk. The optic stalk elongates with a groove at its underside. By the end of six weeks axons from the optic cup (retina) gets into the optic stalk (nerve). At the same time the hyaloid artery also enters the optic nerve. The axons surround the hyaloid artery.

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By third month of gestation small capillaries develop in the nerve. The coverings of the optic nerve i.e. the pia, arachnoid and dura become well defined by seventh month. The lamina cribrosa develops partly from the choroid and partly from the sclera. As the axons of the ganglion cells grow in the optic nerve, some retinal cells get cut off from the main body and get separated as a small clump of glial tissue. This clump of glial tissue forms the early optic nerve head. At birth the raised area atrophies to form the optic cup. Optic nerve is myelinated very late. Myelination of the optic nerve actually starts in the lateral geneculate body9 and spread towards the eye. By birth the myelination is complete and stops generally behind the lamina.

Congenital Anomalies of Optic Nerve


Congenital anomalies of optic disc are common10. They may vary from complete absence to megalo papilla11 both of which are very rare. The congenital anomalies of optic nerve are rarely confined to the optic nerve itself. It is generally associated with developmental anomalies of retina, uvea, central nervous system and mid facial defects. They are caused mostly due to defect in 1. Closure of embryonal fissure. 2. Failure of axons to reach the optic stalk. 3. Failure of hyaloid system to atrophy. 4. Midline facial defects. The common congenital anomalies can be clinically divided into 1. Opaque nerve fibre 2. Abnormality of hyaloid system 3. Coloboma Total Partial Pits Morning glory syndrome. 4. Dysplasia 5. Dysversion 6. Pseudo papilledema (pseudo neuritis)12 Common symptoms of congenital anomalies of optic nerves are : 1. Diminished vision ranging from loss of few lines on Snellens chart to total absence of vision. 2. Sluggish or absent pupillary reaction. 3. Diminished colour sense. 4. Field defect. 5. Associated symptoms of error of refraction, squint, amblyopia, and nystagmus.

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Importance of correct diagnosis of congenital anomalies of optic nerve lies in the fact that they may masquerade as optic neuritis or papilledema and the child may be subjected to unnecessary investigations. Myelinated nerve fibres (opaque nerve fibre). This is most probably the commonest congenital anomaly of the disc and the retina. Generally myelination of the anterior visual path begins in the lateral geniculate body and progresses towards the optic nerve head. The myelination starts late in the intra uterine period (seventh month). The myelination stops short at the lamina. However due to some unknown causes in some infants, the myelination extends well beyond the disc and spreads over the axons of ganglion. The myelination is not equally distributed on the retina. It is generally seen in patches extending from the disc towards the retinal periphery in leaf like pattern. On rare occasions it may spread all round the disc. Less frequently the opaque nerve may develop away from the disc as isolated patch. The condition may be bilateral but need not be symmetrical. It is more common among the boys. It is not present at birth. It takes about a month to be visible in a new born. Hence it may be considered as a developmental anomaly13 of the retina. Symptoms. The symptoms depend on involvement of retina and macula. Patches near the disc do not cause any visual symptoms. Loss of vision occurs only when the macula is involved. Signs. There are no external signs of myelinated (medulated) nerve fibres. However large patches may give a gray reflex on retinoscopy. The condition is diagnosed following ophthalmoscopy. The ophthalmoscopic picture is characteristic and diagnostic. The picture consists of white, fluffy, irregular patches extending from disc towards the retinal periphery. The patches are frayed and feathered. The large vessels pass over the white patch on the periphery but near the disc they are entirely or partially covered by the medulated fibres. Rarely the medulated fibres may obscure the disc itself. Generally the macula is spared. It may be surrounded by two arches of opaque nerve fibres one above and the other below. The medulated fibres do not transmit light hence they produce scotomas. The scotomas as they are gradual to develop, are negative in nature. The scotomas may cause enlargement of the blind spot either locally or all round the disc depending upon the spread of the myelination. The enlarged blind spot may be joined by arcuate nerve fibre defect or there may be isolated scotoma away from the disc corresponding to peripheral patches. The myelination are permanent feature. only occasions when they have been seen to disappear are following optic atrophy, due to demyelination or ischaemia.10 There are no complications known to be caused by myelinated nerve fibres and the condition does not require any treatment. Congenital crescent (conus) of the optic disc. This condition is also a common ophthalmoscopic finding. The condition presents as a semi lunar white area adjacent to the optic nerve. The maximum length of the crescent is parallel to the long axis of the disc, which is invariably oval. The condition is present at birth, does not change its shape or size with age. Cup of the disc is nearer the crescent. The edge of the disc away from the crescent is

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raised. The condition is generally associated with error of refraction which is astigmatic in nature, both myopia or hypermetropia are common. The cause of astigmatism is due to associated change in corneal curvature. The crescent may cause nerve fibre defect in the corresponding area. The exact mode of formation of crescent is not well understood. Some think it to be a modified coloboma of the optic nerve head. Hence this is also known as Fuchs coloboma. The other theory is that it is a fault in mesodermal development of the eye. The condition itself is symptomless but the child is brought with diminished vision, which is a commonly associated with feature due to error of refraction. The treatment is correction of error of refraction. Congenital Coloboma of the Disc Congenital coloboma of the disc may be localised to the disc itself or may be associated with uveo retinal coloboma. The cause of both the forms is faulty closure of the embryonal fissure, hence the coloboma is situated in the lower part of the disc. The coloboma when associated with coloboma of choroid and retina may be extensive enough to spread from pupillary margin to the optic nerve head. In severe form the retina and choroid may herniate through the embryonal fissure below resulting in to congenital cystic eyeball. In less extensive case a chink of uveo-retinal fissure may divide the coloboma in two parts resulting into a bridge coloboma. This must be differentiated from duplication of optic nerve head that is extremely rare. In case of congenital coloboma the scleral canal is wider than normal and the nerve fibres are generally atrophic, the blood vessels are normal. The symptoms of coloboma localised to disc itself is generally superior nerve fibre defect only. The coloboma associated with retinouveal coloboma is always associated with gross visual loss and field defect. The coloboma of the disc should be differentiated from glaucomatous cup. There is no specific treatment. The associated error of refraction should be corrected to give best possible vision. Morning glory syndrome10, 14, 15. Morning glory syndrome is a rare unilateral congenital condition. It is perhaps a colobomatous disorder. The optic nerve head is enlarged, pale and excavated. The base of the cup contains remnants of hyaloid system. The blood vessels instead of arising from the centre of the disc arise from the rim in spoke of wheel pattern. The disc is surrounded by rim of chorioretinal tissue that is elevated. This is surrounded by a pale zone all round. The appearance resembles the flower morning glory after which the syndrome is named. The condition is associated with non rhegmatogenous retinal detachment. The large lesion may give a gray reflex on, retinoscopy and the condition may be confused as leucocoria that is excluded by examination with indirect ophthalmoscopy and ultra sonography. There is no known treatment. Congenital pit of the optic nerve. This condition was considered a rarity. It is proving to be more common than expected. The incidence is as high as 1 in 10,000 live births16. The

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condition is generally unilateral but in one fifth instances it can be bilateral. The exact cause of the condition is not known but fault in closure of embryonal fissure is most widely accepted theory. The pits are present in childhood but symptoms develop in second or third decade. It is equally common among boys and girls. It may be present in siblings who may or may not be symptomatic. The symptoms vary from no symptoms to severe loss of vision, metamorphopsia. On examination the effected disc is generally larger than the unaffected. The pits are more prominent than the holes in lamina. The number, shape, size and position of the pits vary from eye to eye. There may be one pit or may be multiple pits. The shape may be circular dot like, slit like or triangular. The pit is mostly seen in inferio temporal quadrant. Pits situated on temporal side produce more symptoms than on the central part. The pit may be pigmented occasionally. The cause of diminished vision is macular involvement. The macular lesion is serous detachment of sensory retina. The lesion looks similar to central serous retinopathy and is often mistaken as such. The commonest shape of the macular lesion is tear drop raised area with apex towards the disc, rarely the lesion may be circular. There may be a gray membrane on the floor of the disc. The field changes are common. They are - Enlargement of blind spot, central or paracentral scotoma and arcuate scotoma. The central scotoma may be absolute or relative. The eyes are externally normal. In all cases of central serous retinopathy, the disc should be carefully examined by direct ophthalmoscope or +90D lens. The spread of the lesion is best seen on indirect ophthalmoscopy. The detachment of the sensory retina may be : (1) Self limiting, (2) There may be permanent loss of vision due to scarring and subretinal neovascularisation. The nature and source of sub retinal fluid is controversial, three possible sources are : (1) CSF (2) Vitreous and (3) Leak from the capillaries.10 As the exact etiology and natural history of the disease is not well understood, there is no specific treatment. Laser photo coagulation of the leaking vessels is most logical treatment but the result have not been uniform. The differential diagnosis consists of : 1. Central serous retinopathy 2. Small coloboma of the disc. 3. Traumatic maculopathy 4. Subretinal neovascularisation. Hypoplasia of the optic disc10, 12, 17 This is again one of the congenital anomalies that was thought to be rare but the prevalence is more than previous estimates.

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The effected nerve is smaller than usual. It is almost one third of the average size. The colour vary from pink to pale white. The retinal vessels are of normal size and distribution. The hypoplastic disc is surrounded by a halo of hypo-pigmentation of retina and choroid. The condition is bilateral in sixty percent of children. The symptoms are mostly visual and cause of subnormal vision. Vision may be normal or slightly reduced and mistaken for amblyopia and child subjected to anti amblyopia treatment. However in severe form of hypoplasia the vision may be reduced severely. In presence of severe loss of vision, there is generally an afferent pupillary defect. Many a times the children are brought with squint and nystagmus. The hypoplastic optic disc may be seen as an isolated defect in otherwise normal eye or the eye may have associated aniridia, nystagmus, absent foveal reflex. The optical canal is narrower on the involved side. This is a good diagnostic feature in unilateral cases where the small optic foramen and canal can be compared with the normal side. The condition may be associated with mild to severe deformity of the central nervous system, which are more common in bilateral cases. There may be deformity of ventricle, such patients have short stature and low level of growth hormone. The exact cause of hypoplasia of optic disc is not understood. The commonest theory advanced is primary failure of retinal ganglion cells and nerve fibres in otherwise normal retina18. An absence of axons in the nerve reduce the size of the disc. There is no specific treatment. Children with bilateral cases may have to be treated as visually challenged children. Bergmeisters Papilla The normal lens during its development gets nutrition from the hyaloid artery that extends from the optic disc, passes through the vitreous and reaches the lens. It normally regresses during the development of the eye and should disappear at birth. However in some children it may be seen in patches in the anterio posterior axis of the eye. When the posteriormost part fails to regress, the condition is called Bergmeisters papilla. The papilla is in fact, is a central core of vascular tissue surrounded by fibroglial tissue. Sometimes there may only be fibroglial tissue that looks like a translucent membrane over the disc or may project in the vitreous. The condition does not cause any symptoms. No complications are known to arise from it, hence, the condition does not require any treatment. Drusen of Optic Nerve Drusen is not a congenital anomaly in true sense as it is not detectable clinically at birth. It is better called a developmental anomaly. There is no structural change in the tissue

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of the optic nerve. Exact cause of drusen is not known, it is said to be due to excess of neurolgia that undergoes degeneration and calcification. 12 The drusens contain calcium, mucopolysaccharides, carbohydrate and iron.19 There is strong heredity. It is inherited as autosomal dominant trait. The drusen may be seen in siblings and parents. This feature is so constant that in all cases of suspected drusen in the eye of a child, fundus of his parents and siblings should be examined. If they have fundus picture suggestive of drusen the child may be spared of costly investigations for papilledema. Drusen is bilateral in 75% of cases. It is equally seen in boys and girls. It is rarely detectable in first decade. They become well established in second and third decade. In advanced case the drusen may be visible as an irregular growth over the disc. About 0.3% of children have drusen but they go undetected because drusen by itself does not cause any visual symptoms. Presence of drusen comes to light on routine examination for causes other than ocular. Mostly the children are referred as cases of papilledema. To begin with drusens are deep in the optic nerve head, always anterior to lamina and not visible by direct ophthalmoscope. Examination by Hruby or other suitable lens may give away their presence. Number of drusen may vary from solitary to multiple, when more than one, they may join to form a larger mass. Ophthalmoscopic finding of drusen are : 1. The colour of the disc is pinkish pale but not hyperemic. 2. Blood vessels on the disc have abnormal branching. 3. Spontaneous vernous pulsation is present in 80% of the eye. 4. The optic cup is shallow to begin with, gradually it fills up and after years, the surface of the disc may be beyond the plane of retina, which is normal. 5. The veins are not dilated and can be traced to the centre of the disc. 6. On fluorescein angiography there is no leak. The drusen shows auto florescence. 7. Ultrasonography is helpful as drusens are good reflector of ultrasound wave even at low decibel. 8. CT of optic nerve head may show buried drusen. Field changes. Depending upon the position of the drusen in relation to retinal fibres, the field changes can beenlargement of the blind spot, arcuate or nerve fibre defect. Constriction of lower nasal field is most characteristic.18 Differential diagnosis. Other causes of elevated disc in children i.e. papilledema, neuritis, pseudoneuritis, hamartoma of the disc, Bergemeister papilla. Complications. The condition is benign, does not cause blindness. However on rare occasions there may be haemorrhage in the lesion. Management. The condition per se does not require any treatment. However child should be kept under observation for few years least the diagnosis of raised intracranial pressure has been missed. This is best done by serial photographs of the disc.

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Pseudo neuritis. Pseudo neuritis sometimes mentioned as pseudo papilledema is neither neuritis nor papilledema in true sense. Both of which are acquired. It is better to call it congenital disc swelling. The condition is very common and unless examined carefully, the child is subjected to lots of unnecessary investigation. The condition is reverse coloboma of the disc where the scleral canal is large and nerve fibres are deficient. In pseudo neuritis the scleral canal is smaller for number of nerve fibres. The condition is ectodermal in origin. In 80% cases it is bilateral, may be seen in other members of the family. The condition is common with hypermetropia and hypermetropic astigmatism. However it can be seen in emmetropes as well. The condition is a asymptomatic. It is generally discovered when children are examined for symptoms of hypermetropia. In such cases the corrected vision remains less normal. The fundus picture is diagnostic. The disc is small, the margins are indistinct. There is disc swelling that does not exceed +3D. The blood vessels are normal in calibre, and distribution. The retina round the disc is normal, no exudates or haemorrhages are seen. The retina has a peculiar appearance known as shot silk look. There are no field changes. The pupil are normal. Differential diagnosis consists of all the conditions that cause swelling of the optic disc. They are drusen of optic nerve, remnants of hyaloid system, Bergmeister papilla, neuritis and papillaedema. Fluorescein angiography differentiate pseudoneuritis from early papillaedema and neuritis. The condition is non progressive and symptomless, hence it does not require any specific treatment except prescription of glasses for error of refraction when present.

Disorders of Optic Nerve


Disorders of the optic nerve can beCongenital or acquired. The acquired cases can be : 1. Infective and/or inflammatory 2. Non infective or non infective. (a) Papillaedema (b) Degeneration (c) Dystrophy (d) Traumatic (e) Neoplastic (f ) Nutritional (g) Vascular

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Disorders of optic nerve not only produce visual disturbance that be temporary or permanent but also be indicators of serious systemic disorders that may not only be vision threatening but also fatal. Symptoms of optic nerve disorder : 1. Visual. Commonest symptom is diminished vision that may range from slight recoverable vision loss to total and permanent loss. However, there may not be any visual loss in the beginning as in papilledema. 2. Amaurosis. Temporary sudden loss of vision (with recovery) is common in papilledema. 3. Colour sense is always affected in disorder of the optic nerve. It is not a total loss of colour vision but the coloured objects look dull especially the red coloured objects. 4. Light brightness is reduced. 5. Field changes. Central and centrocecal field changes are very common in inflammation of optic nerve. In papilledema there is enlargement of blind spot. Peripheral constriction of field is sign of onset of optic atrophy in neuritis and papilledema. 6. Pupillary changes. Afferent pupillary defect is common when the afferent pathway is involved. It is very common in children. 7. Pain on movement of the eyeball is common in acute inflammation of posterior part of the optic nerve. The disorder of the optic nerve can be present at birth as congenital anomaly or present as developmental anomaly some year after birth or it may be acquired. The acquired condition can be : 1. Inflammatory and/or infectious 2. Non inflammatory and/or non infectious. In the first group come papillitis, optic neuritis and retrobulbar neuritis. The second group has a long list of conditions that include : 1. Papilledema 2. Degenerations 3. Dystrophies 4. Trauma 5. Toxins 6. Nutritional 7. Neoplastic 8. Vascular 9. Unclassified.

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Papilledema22 Papilledema23 is not a disease. It is a sign that reflects a more serious primary systemic condition mostly intra cranial. Papilledema has great diagnostic, therapeutic and prognostic value. It reflects the pressure in the intra cranial structures. It also represents the pressure gradient between the pre lamilar and retro lamilar part of the disc. The term papilledema should be used only to denote bilateral passive edema of the optic disc due to raised intracranial pressure. The retro lamilar part of the disc is not involved in papilledema. The rise of intracranial pressure may be due to lesions of intracranial structures or the cause may be systemic. The latter is less frequent than the former. Other cause of elevation of the disc should be designated as disc swelling only. The causes of which can either be orbital or ocular. Papilledema can be seen in all ages equally in both sexes all over the world and does not have any genetic predisposition. However in children it can be seen as early as one year. It is very common between one to five years.20 However it should be remembered that other age groups are not immune. Papilledema develops fast in children because the location of the primary lesion is such that it causes more embarrassment to CSF flow than others. In children infra tentorial lesion both infective and neoplastic are common causes of papilledema. Progress of papilledema is slow before the sutures are united. However a fast growing growth may cause separation of sutures and cause silver beaten impression on the cranium. Common growths that cause papilledema are tumours of cerebellum, fourth ventricle, tempro sphenoidal lobe of cerebral hemisphere and mid brain tumours.20 Tumours of pons do not produce early papilledema. All patients with papilledema may not have intracranial tumour. Papilledema develops fast and subsides slowly. 21 In case of acute intracranial hemorrhage, it may develop with in 2-8 hours. Otherwise it may take 1-5 days to develop in case of raised CSF pressure. There is no possibility of papilledema subsiding spontaneously. It may regress only when the primary cause of raised intra cranial pressure has been eliminated either surgically or medically. In such occasions it takes 6-8 weeks for papilledema to start subsiding. Pathogenesis23. The exact pathogenesis of development of papilledema is not clear. One fact that is universally accepted in production of papilledema is rise of CSF pressure. The other factors are secondary to raised CSF pressure. Following are some possible mechanism to cause raised intracranial pressure. 1. Block in circulation of CSF by congenital lesion like aqueduct stenosis or by acquired condition like an intracranial tumour. 2. Space occupying lesions 3. Retarded absorption of CSF due to cortical venous thrombosis or block of arachnoid villi by blood or protein.23 Most widely accepted theory is derangement of axoplasmic flow secondary to raised intra cranial tension that forces cerebrospilal fluid under pressure to throttle the central

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retinal vein as it passes through the sub-arachnoid space round the optic nerve. The increased pressure in the sub arachnoid space changes the pressure gradient behind the lamina. There is intracellular axonal swelling which is followed by extra cellular edema. Commonest disorders that cause papilledema in children are : 1. Congenital anomalies 2. Infection Various forms of craniostenoses, syrengiomyelitis. Meningitis, encephalitis Lateral sinus thrombosis Brain abscess, tuberculoma 3. Trauma Intracranial hemorrhage Subarachnoid hemorrhage Subdural hematoma 4. Tumours Benign Malignant Secondary 5. Drugs Hyper vitaminosis A Steroid over dose Tetracyclin Nalidoxic acid 6. Miscellaneous Pseudo tumour cerebre Hydrocephalus Hypoparathyroidism Cysticercosis Leukemia Symptoms of papilledema. Symptoms of papilledema are essentially symptoms of raised intracranial pressure. The symptoms are directly proportionate to level of intracranial pressure and its duration. Papilledema of short duration are generally seen following head injury with extensive intracerebral bleeding, subarachnoid haemorrhage or encephalitis. Systemic symptoms of all these conditions overshadow ocular symptom. Symptoms of papilledema can be grouped into : (1) General, (2) Ocular General symptoms consist of headache, nausea, vomiting, neurological deficiency. Cases associated with infective process may have fever. Headache. Headache may be acute or chronic, there is no localisation of pain. It is generally worse in the morning and worsened by coughing, sneezing and straining. Location of pain in the cranium have no localising value. Vomiting is generally projectile, it may be inter-spaced by nausea. Neurological deficiencies are late to develop. Ocular symptoms differ in early and late stages.

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Early ocular symptoms are mostly visual. First visual symptom is sudden loss of bilateral vision for less than a minute with recovery to original level. The blurring of vision may be just like a veil that lifts spontaneously, to total loss of light sense. The colour sense during these episodes are also diminished. Diplopia. Diplopia may be the presenting sign, it is generally due to sixth nerve palsy. There may be skew deviation. Late ocular symptoms are : 1. Diminished visionSudden diminished vision in fully developed papilledema is due to haemorrhage over the macula or infarction in the optic nerve itself. Gradual loss of vision is due to onset of secondary optic atrophy that may culminate in loss of perception of light. There may be associated diminished colour sense. 2. Diplopia persists and may become permanent unless the cause of papilledema is relieved. Signs of papilledema. In initial stages of papilledema, the eyes look normal. Onset of paralytic squint in a child with headache, vomiting is an ominous sign and always points towards possibility of papilledema. A sluggish pupillary reaction, afferent pupillary reaction and absent pupillary reaction are seen only when secondary optic atrophy has set in. Ophthalmoscopic signs. Most important signs are ophthalmoscopic. Ophthalmic signs can be divided into following groups24 1. Early papilledema 2. Established papilledema 3. Late papilledema (chronic) 4. Optic atrophy (atrophic) Early papilledema. Diagnosis of papilledema on the basis of ophthalmoscopic findings is one of the great riddles in clinical ophthalmology. It needs to be corroborated with careful history and other investigations i.e. field changes, fluorescein angiography, X-Ray, CT, MRI etc. 1. The earliest change is blurring of disc margin that does not follow a set pattern but generally it starts at upper pole followed by lower then nasal and lastly the temporal margin. 2. Hyperemia of the disc due to capillary dilatation. 3. Filling of optic cup, swelling of the disc. 4. Swelling of the disc is most important sign. It may have to be examined by +90D to confirm. 5. Loss of spontaneous venous pulsation - It should be remembered that in 20% of persons venous pulsation is absent so its absence is not confirmatory but presence of venous pulsation excludes papilledema. 6. Blurring of peripapillary nerve layers. They look striated. Established papilledema. Once papilledema is well established, diagnosis is not difficult.

524 1. The veins become dilated.

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2. The disc surface is elevated over the surrounding retina which itself may be edematous. Difference of 3D between the top of the disc and retina means elevation of 1 mm. The disc may develop as much as 2 to 3 mm of elevation. The blood vessels over the disc may be obscured by edematous nerve fibres. Exudates and haemorrhages. Initially the exudates are soft cotton wool in appearance associated with flame shaped haemorrhages. As the condition progresses changes develop on the macula in the form of hard exudates that radiate from the centre of the fovea towards the disc in the form of a macular fan. Chronic papilledema. It takes months to develop chronic changes where the elevation is maximum almost like a small pink mushroom in which the retinal vessels disappear. By this time, the exudates and haemorrhages disappear. Secondary optic atrophy. The edema of the disc diminishes and acquire a milky white appearance. The gliosis develops not only on the disc but also spreads on the blood vessels round the disc in the form of sheathing. The lamina remains invisible. Generally it takes 6 to 8 months to develop secondary optic atrophy. Investigations in papilledema25, 26. Fundus fluorescein angiography is useful in differentiating pseudo elevation from true papilledema i.e. drusen, pseudoneuritis and high hypermetropia. Fluorescein angiography does not differentiate papilledema from papillitis, both show diffuse staining with profuse leaking that spreads into surrounding nerve layers. Field changes. Fundus changes are not conclusive in papilledema. The two changes seen are : 1. Enlargement of the blind spot. This happens only when the papilledema is well established. 2. Peripheral constriction of field, this is met with in stage of secondary optic atrophy. X-Ray skull, CT and MRI are used to pinpoint the location and size of the intracranial lesion. Differential diagnosis of papilledema : Papilledema should be differentiated from all other causes of disc edema i.e. 1. Papillitis 2. Pseudoneuritis 3. Drusen of optic nerve 4. Bergmeister papilla.

Unilateral Disc Swelling


It is better not to term unilateral disc swelling as papilledema. Though the basic pathology is the same i.e. venous stasis with derangement of axoplasmic flow, the lesion may extend well beyond the lamina, which never happens in papilledema. The causes can be : 1. Ocular and 2. Orbital

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Ocular causes of disc swelling are : 1. Papillitis 2. Posterior uveitis 3. Hypotony 4. Acute rise of intraocular pressure. Orbital causes of disc edema are Retro bulbar growths may be inflammatory like specific granuloma, orbital abscess, non specific pseudo tumour of orbit. Treatment. Management of papilledema is essentially management of its cause. Once intraocular pressure has been reduced to normal, the papilledema starts regressing. It takes six to eight weeks for papilledema to subside. Vision may improve with regression of edema. However nerve fibre damage may have some residual effect. Vision lost due to optic atrophy is permanent. In sudden rise of intra cranial pressure I.V. mannitol, may relieve the tension. In case of head injury, use of mannitol may be given in consultation with neurosurgeon. In chronic papilledema, the mass may be treated medically when infective. Growths both benign or malignant are removed surgically. Sometimes a shunt may be required to drain CSF in the peritoneum in the form of ventriculo peritoneal or lumbo peritoneal shunt. Local fenestration of optic nerve sheath has not been reported to give uniform good result.

Optic Neuritis26, 27, 28


The term optic neuritis is non specific that includes all the conditions, which have loss of central vision, dyschromatopsia and central field defect, which can be acute or chronic due to involvement of optic nerve from papilla to beginning of the chiasma. In 20% to 30% cases no cause can be found and are put in group of idiopathic optic neuritis. Other causes in children are : 1. Inflammation/infection 2. Demyelination 3. Toxic 4. Hereditary 5. Post vaccination 6. Degeneration Direct invasion by micro-organism is rare. Most of the time, it is the inflammatory process of microbial infection that is responsible for optic neuritis, which can be26 Ocular Orbital Brain and its coverings Systemic Ocular. The optic nerve is direct extension of axonal fibres of the retina so it is very easy for infection to travel from retina to optic nerve in the form of neuro retinitis.

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Other causes are choroiditis, endophthalmitis, panophthalmitis, sympathetic ophthalmia. Orbital causes are Orbital cellulitis, thrombophlebitis, arteritis, Wegner granulomatosis, other granulomas, foreign bodies, secondary to sinus diseases. Intracranial diseases : Meningitis Encephalitis Meningo encephalitis Systemic disease : Tuberculosis Various types of pneumonias Poliomyelitis, influenza, mumps, measles. Demyelination. Demyelination is a major cause of optic neuritis in adult. It is less commonly seen in younger children but children in teens may be effected by demyelination. Two common forms are Devics neuro myelitis optica and Schilders disease. ToxicMany drugs when given systemically can cause optic neuritis. Common drugs areethambutal, isoniazide, streptomycin, chloramphenicol, fluoroquinolones, metronidazole, chloroquin. The list is very long. One thing worth noting is that almost all of them are widely used for systemic diseases in children. Hereditary. Lebers optic neuropathy. According to location of the lesion, optic neuritis can be : Papillitis. Inflammation of optic nerve head. Neuroretinitis Retrobulbar neuritisAcute or chronic. If the papillo macular bundles are predominantly involved. It is called axial neuritis. If the peripheral nerves are involved, it is perineuritis. General features of optic neuritis. It is generally an acute condition mostly unilateral. Simultaneous symmetrical involvement of other side is rare. However bilateral involvement is more common in children than adults. Optic neuritis is slightly more common in girls. Incidence of optic neuritis after fifth decade is less than in second and third decade. The main symptoms are : I. Visual that consists of : 1. Diminished central vision 2. Dyschromatopsia 3. Defective contrast sensitivity 4. Defective stereopsis 5. Uthoff phenomenon 6. Scotoma

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II. Pain in the eye III. Neurological symptoms Vision in optic neuritis : 1. Unilateral acute loss of vision that may deteriorate within hours to days. Vision may go down to perception of light. 2. Recovery of vision without treatment starts by second week and original vision is reached by four to five weeks. 3. Normal vision with signs of central scotoma which is relative, diminished colour and depth sense. 4. Failure to recover vision generally, is common in adults who have multiple sclerosis is not seen in children. 2. Dyschromatopsia. The coloured objects look drab and washed out. There is no real colour blindness. 3. Defective contrast. The surrounding area looks dim as if the lights in the room have been turned down. Defective stereopsis. The patient may not be able to judge the depth well. Bilateral optic neuritis is commonly seen in Devics neuromyelitis optica, Schilders disease, Acute disseminated encephalo myelitis, post vaccination status. Uhthoff phenomenon. In this phenomenon vision is decreased if body temperature is raised either by exercise or taking hot food and drink, hot bath, hot weather, and anger. Even stress and anxiety can precipitate the phenomenon. Previously positive Uhthoff effect was considered to be diagnostic of multiple sclerosis. It has been pointed out that there are other conditions like Lebers optic neuritis, toxic neuropathy, Friedreichs ataxia where this effect can be positive. Scotoma. Commonest field defect is central scotoma, which is more for colour than for white. Scotoma for red is most effected, other scotomas are centrocecal and paracecal, nerve bundle defect and even sectorial peripheral field loss is possible. Kind of scotoma is directly related to type of nerve fibres involved. In axial neuritis where central i.e. papillo macular fibres are involved the scotoma is central. Pain in the eye. In case of optic neuritis patient may complain of pain with or without movement of eye. Pain on movement of eye is more common in optic neuritis and may be absent in papillitis. It is more marked if the lesion is near the apex of orbit. There are two possible explanations : 1. Due to contraction of superior or medial rectus muscles. The dural sheath that is pain sensitive gets stretched and pain is precipitated. 2. The sheath of optic nerve is stretched in swelling of the nerve and becomes painful. There may be tenderness at the insertion of superior rectus. Pain may precede loss of vision sometimes.

528 Fundus in optic neuritis :

PEDIATRIC OPHTHALMOLOGY

1. Early changes consist of blurring of disc margin and hyperemia. In some cases disc may look normal in spite of diminished vision and central scotoma when lesion is away from the disc. 2. It may be difficult to differentiate between fully developed optic neuritis and early papilledema. 3. Late changes are pallor of disc. 4. Fully developed neuritis is always associated with cells in the vitreous in front of the disc. Neurological symptoms. Sometimes patient have numbness of limbs which the patient may ignore because loss of vision is more alarming than numbness. In neuromyelitis optica the disc involvement may precede myelitis or vice versa.

Optic Neuritis in Children52


Optic neuritis in pediatric age group differs from adult optic neuritis in many ways though pathology in both age groups is the same. 1. Optic neuritis in children is less frequent than adults. 2. Both boys and girls are affected equally. 3. Optic neuritis in children is bilateral more often than in adults. 4. Involvement of anterior part of nerve is more common in children. 5. Commonest cause of optic neuritis is viral infection. Optic neuritis starts two to three weeks after onset of systemic viral infection. 6. Optic neuritis is a frequent complication of many vaccines. Optic neuritis sets in after 2-3 weeks of vaccination. Antiviral vaccine cause neuritis, more commonly than antibacterial vaccines. 7. Multiple sclerosis that is a major cause of optic neuritis in adult inflicts children less frequently. 8. Severity is more in children. Initial loss of vision is profound and disc swelling relatively more than in adults. 9. Chances of visual recovery in children is very good. 10. Optic neuritis in children responds well to steroids. Management of optic neuritis. Management of optic neuritis is unsatisfactory. There is no specific treatment that will be equally effective in all cases. All efforts should be made to find out the cause as far as possible by neuro imaging, CSF examination, X-ray, chest X-Ray, PNS X-ray orbit. Thirty percent cases are idiopathic and recover without treatment. In idiopathic optic neuritis recovery of vision following initial abrupt loss of vision take few weeks and complete recovery may take as much as 6-8 months. If there is no improvement in recovery phase, the child should be investigated for some other cause.

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Cortico steroids are given only to reduce inflammatory process and hasten recovery. They do not cure neuritis, nor do they influence ultimate recovery. They do not reduce chance of relapse.

Some Specific Type of Optic Neuritis


1. Post vaccination optic neuritis. One of the untoward side effects of prophylactic vaccination against host of diseases, both viral and bacterial diseases, are delayed optic neuritis which is more frequent in antiviral vaccination than bacterial immunisation. It develops two to three weeks after vaccination, is generally bilateral and self limiting. Common prophylaxises that are known to cause optic neuritis are - Hepatitis B, rabies, measles, mums, rubella, and tetanus toxoid. 2. Neuro neuritis. Commonest cause of neuro retinitis in children is toxoplasmosis. Other systemic causes like syphilis, tuberculosis which are common in adults are generally do not cause neuro retinitis in children. 3. Retro bulbar neuritis. Acute retrobulbar neuritis is common in older children due to acute sinusitis. Chronic retrobulbar optic neuritis which is also known as toxic amblyopia is unknown in children. Retrobulbar neuritis may not show signs of inflammation on optic nerve head. 4. Drug induced optic neuritis. Drug induced optic neuritis is common following administration of anti tubercular drugs i.e. streptomycin, isoniazide and ethambutol, another drug used commonly in children is chloroquine. Fortunately chloramphenicol is no more used as routine drug for typhoid hence incidence of chloremphenicol induced optic neuritis is rare. Drugs may involve any part of the optic nerve. Retro bulbar involvement is more common. Management consists of withdrawal of the drug whenever possible. In some instances vision may not return to normal even after withdrawal of offending drug due to irreversible neuronal damage. In such cases other possibilities should be excluded by complete physical examination, CT and MRI. Demyelinating diseases. Demyelinating diseases are common systemic causes of visuocortical tract involvement that may spread from disc to lateral geniculate body and even the optic radiation56. The common demyelinating diseases that involve optic nerves are53 1. Multiple sclerosis 2. Devics neuromyelitis 3. Schilders disease 4. Acute disseminated encephalomyelitis 5. Krabbes disease. Multiple sclerosis54, 55 is a very prominent cause of optic neuritis in adults. It is not a frequent cause of optic neuritis in children. One third of all patients with multiple sclerosis develop optic neuritis, sometimes or other in the course of disease that may last for 20 to 30 years. About thirty percent of acute idiopathic optic neuritis develop multiple sclerosis. In

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fifteen percent of cases optic neuritis is presenting sign of multiple sclerosis. Spinal cord is the commonest site of involvement. Visual loss is unilateral with deep central scotoma and marked colour defect. Diminished nerve conduction is the cause of visual impairment. There may be an afferent pupillary defect. There is pain on movement of the eyeball. There may be involvement of any of the cranial nerve that supply the extra ocular muscles in various combination. Muscles may be involved in inter nuclear ophthalmoplegia Uhthoff sign is common, so is Pulfrich phenomenon in which the patient has better vision in dim light. Devics neuromyelitis optica. This is seen in children and young adults but progresses well into pre senile age. It involves boys and girls equally. It is an acute disease that starts as acute, bilateral loss of vision, which need not be equal in two eyes. Myelitis, which is a constant feature may follow neuritis or precede it. It involves the spinal cord, and the optic nerve. Other cranial nerves are spared. It may involve sympathetic chain as well. Visual field defects depend upon presence of demyelination in the optic nerve. Myelitis results in paraplegia. It is generally acute in nature and profound. Most patients show considerable improvement in motor function, some residual effect is universal. Some visual recovery is the rule. Recurrence of neuritis and myelitis is common. It should be differentiated from other causes of bilateral optic neuritis. Fundus changes depend upon part of the optic nerve involvement and its severity. It may range between normal disc when the lesion is far back in the nerve, to frank swelling when the lesion extends in the pre lamilar part. Schilders disease56. This is a genetically determined in born error of metabolism where there is primary degeneration of adrenal gland. The disorder belongs to a group of disorders known as leukodystrophies. The condition has profound neurological deficiencies besides loss of vision. Loss of vision is due to neuritis that is followed by atrophy of optic nerve. There may be cortical blindness. It is associated with hearing loss, slurred speech, mental retardation, ataxia, ophthalmoplegia and nystagmus. There is no known treatment. Krabbes disease56. This is also a leukodystrophy. There is relentless demyelination of not only the optic nerve but also that of cerebrum. There is mental retardation, diffuse sensory loss and motor loss. Blindness is very common not only due to involvement of optic nerve but also optic radiation. There is no specific treatment. Comparison between optic neuritis, papilledema and pseudo neuritis. Many a times it is difficult to differentiate between fully developed optic neuritis and early papilledema. Same is true between pseudo neuritis (pseudo papilledema) and early papilledema ophthalmoscopically. Other clinical features and investigations have to be gone through when in doubt.

DISORDERS OF OPTIC NERVE IN CHILDREN Signs and symptoms Age Sex Onset Bilaterality Vision Cause of visual loss Papillitis Any, rare after 50 years Both Acute Generally unilateral Rapid fall of vision Early conduction defect Papilledema Pseudo neuritis

531

Any rare before the su- Any tures close Both Slow Bilateral Both Present from birth Bilateral, may be unilateral

Good, fall of vision is late Normal, may have error of refraction. Involvement of macula Nil and late conduction defect Transient loss of vision Nil

Other visual defects Field change

Defective colour sense, stereopsis and contrast Central or centrocecal, may have nerve fibre defect May show afferent defect

Enlargement of the Nil blind spot initially normal. Peripheral constriction late. Pupillary reaction ab- Nil sent if third nerve is involved or optic atrophy has set in Signs of raised intra cra- Nil nial tesion Swollen disc more than 3D. Absent venous pulsation. Edema of macula. Macular star. May have flame shaped haemorrhages. Small hyperemic disc not more than 3D. No haemorrhage, no exudate.

Pupil

Other neurological as- Generally none sociation Fundus Variable degree of disc swelling not more than 3D. May be normal initially, blurring of disc margin, few flame shaped haemorrhages, macular star absent, vitreous haze over the disc Non specific, Minimal leakage of dye. No specific change. Shows plaque demyelination in

Fluorescein angiography CT MRI

Vertical pool of dye due Normal to leakage. May show intracranial No change lesion Intra cranial lesion. Normal

532 Causes of unilateral disc swelling in children : 1. Ocular57 : Papillitis Drusen of optic nerve head Ocular hypotony Severe intraocular inflammation Neuro retinitis Pseudo neuritis Optic nerve glioma Orbital cellulitis 2. Intracranial : Foster Kennedy syndrome

PEDIATRIC OPHTHALMOLOGY

Unilateral high myopia with raised intra cranial tension. The normal eye develops disc swelling. Cavernous sinus thrombosis 3. Pseudo tumour ceribri (initial stage) Sub arachnoid haemorrhage.

Optic Atrophy
Optic atrophy. Optic atrophy is not a disease. It is the end result of many diseases, where the optic nerve is virtually a scar with loss of its function. It has been defined in various ways, the sum total of which says that optic atrophy occurs due to degeneration of axons in the anterior visual pathway. Anatomically it has been divided into : 1. Ascending optic atrophy where the pathology spreads from the retinal ganglion cells towards lateral geniculate body. 2. Descending optic atrophyHere the degeneration spreads from lateral geniculate body to optic nerve. Other classifications are : On the basis of ophthalmoscopic appearance : 1. Primary (simple) optic atrophy 2. Secondary optic atrophy Post neuritic Post papilledematous 3. Consecutive optic atrophy 4. Cavernous optic atrophy 5. Glaucomatous optic atrophy 6. Temporal pallor. 7. Bow tie pallor

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Etiological classification The causes of optic atrophy in children can be : 1. Heredo familial 2. Post papilledematous 3. Post neuritic 4. Trauma 5. Consecutive 6. Toxic 7. Nutritional 8. Metabolic (Juvenile diabetes mellitus, mucopolysaccharidosis) Primary optic atrophy. In this type of optic atrophy no visible cause that may involve the optic disc i.e. disease of choroid, retina, or optic nerve (optic neuritis) is detectable. There is no evidence of raised intra cranial tension. The lesion is in the second visual neuron proximal to disc.30 This is due to degeneration of optic nerve fibres with minimal glial proliferation without any mesenchymal reaction.31 The myeline sheath and axis cylinders disappears, there is shrinkage of optic nerve. At the disc there is reduction in nerve tissue, this results in increase in width and depth of optic cup and exposes the holes in lamina cribrosa. The optic cup becomes pale. The cause of whitening was thought to be paucity of capillaries on the disc head.30 Now it is thought to be due to impaired transmission of light into the substance of the disc. This is brought about due to absence of axonal fibres, which are responsible for transmission of light32. Commonest cause of primary optic atrophy in adults is tabes dorsalis which is not seen in children. In children the cause of primary optic atrophy are - hydrocephaly, toxic, retrobulbar neuritis, traumatic opticneuropathy, heredo familial optic neuropathy. The clinical presentation consists of visual, pupillary change and pallor of disc. Visual symptoms consist of diminished distant vision, central scotoma, colour deficiency, reduced contrast sensitivity and stereopsis. Pupillary changes consist of large pupil and afferent pupillary change. In bilateral primary optic atrophy both pupil are large and sluggish. Bilateral immobile pupil is sign of gross loss of vision. Fundus change. The media is clear. The margin of the disc is clear cut. The colour of disc has been called by various adjectives i.e. chalk white, marble white or paper white. The number of vessels on the disc is less than ten.33 However on fluorescein angiography, the number of capillaries are more than visible on ophthalmoscope but atrophic, no leak or pooling is seen. The cup is deeper than normal physiological cup and may be mistaken as glaucomatous. The width of the cup is also more than normal. The lamina cribrosa is visible as bluish dots. The vessels have normal calibre and do not show any sheathing. The peripapillary retina and choroid are normal. The primary optic atrophy is an example of descending optic atrophy where the lesion may be anywhere from retrobulbar part of optic nerve to the optic radiation.33

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Secondary optic nerve. This is more common than primary optic atrophy in children. This is always preceded by swelling of the disc either due to inflammation, which is associated with active congestion or with passive congestion as in papilledema or uniocular disc edema. The atrophy is also a degenerative process of the optic nerve, have more reactive elements than primary atrophy. The degenerative process is obscured by proliferation of astrocytes, glial tissue and to some extent blood vessels. It is more common than primary optic atrophy in all ages. The condition is equally common following either papillitis or papilledema. In children secondary optic atrophy is usually bilateral. The symptoms of secondary optic atrophy are similar to that of primary optic atrophy, only difference is that the former is preceded by symptoms of either papillitis or papilledema. The signs are also same as far as field changes and pupillary reaction is concerned. The main difference lies in fundus picture. The fundus picture. The media is not affected. The margin of the disc is blurred. The disc may be raised in spite of onset of atrophy. In fully developed secondary optic atrophy, the swelling subsides but never regresses in the disc, thus the physiological cup is always obliterated and lamina is not visible. The vessels always show some degree of sheathing. The retina and choroid are not affected. The disc has dirty yellow colour. It is not possible to differentiate between post papilledematous and post papillitis optic atrophy. The field when possible to chart shows enlargement of blind spot. Onset of atrophy is heralded by gradual constriction of peripheral field, ultimately only a central island of vision may be left. Consecutive optic atrophy. This is an ascending type of optic atrophy, which is essentially secondary to disease of retina, choroid or both. It may be unilateral or bilateral. The fundus picture comprises of : The disc is pale but colour is waxy white. The disc margin is almost normal but not as clear cut as in primary optic atrophy. The cup is maintained unless there was a concurrent involvement of optic nerve as seen in neuroretinitis. The calibre of the blood vessels depend upon their involvement in primary chorioretinal lesion. They are attenuated in retinal abiotrophy i.e. retinitis pigmentosa. The surrounding fundus shows evidence of chorioretinal scar. In case of a macular lesion, the atrophy is generally confined to temporal side. In case of patch of choroiditis adjacent to the disc, the part of the disc near the lesion is atrophied so is in case of choroidal rupture near the disc. Commonest cause of consecutive optic atrophy is retinitis pigmentosa. Other causes are diffuse chorioretinitis, toxic retinopathy, extensive photocoagulation.33 The symptoms of consecutive optic atrophy is overshadowed by symptoms of primary chorioretinal pathology.

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Cavernous optic atrophy. This type of optic atrophy is rare in children. There is mucoid degeneration of the optic nerve head. There is no proliferation of glial tissue. On histology empty spaces are formed in between the nerve fibres which are filled with mucoid material.32 The fundus picture is in between primary optic atrophy and glaucomatous optic atrophy, there is prominent pale cup in pale nerve with nasal shift of blood vessels. Temporal pallor. This is partial optic atrophy due to involvement of papillo macular bundle. Commonest cause is a lesion either in the macula or in between the macula and the disc. It could be a congenital anomaly of macula i.e. macular coloboma, toxoplasmal chorioretinitis or rupture of choroid between the macula and disc. Laser burns too near the disc on temporal side. Other causes are toxic neuritis, old retro bulbar neuritis, heredofamilial optic atrophies, Lebers optic atrophy, Behrs optic atrophy, Kjers optic atrophy. Some specific types of optic atrophy : Traumatic optic neuropathy. Trauma to optic nerve direct or indirect is the cause of traumatic optic atrophy. The injury can be a closed head trauma, an injury to the frontal bone, or a trivial injury on the eyebrow. The optic nerve can be injured anywhere from chiasma to entry of nerve in the sclera. It can be avulsed if the eyeball is caught in a curved object and pulled anteriorly besides avulsion such injuries can stretch the optic nerve, can cause haemorrhage in the sheath. The optic nerve may be severed by a bullet injury, fragment of granade, or by a stab injury in the orbit. The intraorbital part may be injured during retrobulbar injection, or during facio orbital surgery. In avulsion of optic nerve, there is sudden loss of vision with severe pain, may be associated with mild proptosis and restricted movement. On ophthalmoscopy there are large blotches of haemorrhages round the disc. Gradually as the haemorrhages clear, a pale disc almost devoid of any vessels becomes visible. The resultant atrophy is primary optic atrophy in nature. Injury behind the entry of central retinal vessels and optic canal does not produce any change on fundus examination initially. It takes four to five weeks for the change to appear in the form of primary optic atrophy. The intracanalicular part of the nerve is most likely to be injured in closed head injury. The mechanism of damage to intra canalicular part of optic nerve is : Rotational and shearing force that push the frontal lobe, pull the nerve back. The shearing force is transmitted to immobile optic nerve in the canal. Accumulation of blood or edema may throttle the blood flow of the optic nerve. Bone fragments from the fractured canal may get lodged in the substance of the nerve or may press the nerve from outside. Traumatic optic neuropathy is a common accident in children, more frequent in boys. It is commonly seen following a cricket ball injury to the lateral side of the eyebrow, in fact any hard object hitting the frontal bone is likely to cause traumatic optic neuropathy. It is generally unilateral. Bilateral traumatic optic neuropathy is seen in closed head injury that damages the chiasma. The symptoms may be acute loss of vision if the injury is with in 10 mm of the posterior scleral canal and is generally profound. Vision may be reduced

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to perception of light, which may be lost altogether. Loss of vision following injury to posterior part of the optic nerve is variable between moderate loss to loss of perception. In uniocular profound loss of vision there may be afferent pupillary defect. Diagnosis of traumatic optic neuropathy is generally delayed due to overwhelming signs and symptoms of head injury. Diminished unilateral loss of vision following frontal head injury with afferent pupil on the same side is a strong point in favour of traumatic optic neuropathy that is confirmed by - fundus examination, field charting, CT and MRI. CT is superior to MRI in outlining fracture of bones. MRI gives better image of soft tissue damage. MRI is contra indicated if there is suspicion of intra orbital or intra cranial metallic foreign body that should be excluded by plain X-ray first. Treatment consists of : 1. Management of head and cranio facial injury. This should get priority over ocular treatment because these can be life threatening to the child. 2. Ocular therapy should be started as soon as the child is stable. Ocular treatment is : 1. Medical. This consists of heavy dose of I.V. steroid. The drug of choice is methyl prednisolone as 30 mg/kg35 as loading dose followed by 5.4 mg/kg/hr for 48 hours to 72 hours or dexamethasone 3-5 mg/kg/day is given if vision improves. Methyl prednisolone is continued for 2-3 days and then replaced by oral prednisolone in tapering dose. If there is deterioration of vision while on oral steroid, I.V. methyl prednisolone is restarted. If there is no improvement in first 72 hours following initial start of I.V. methyl prednisolone, surgery should be considered which consists of fenestration of optic nerve sheath or removal of roof of optic canal via trans nasal or trans ethmoidal route by endoscope.

Heredo Familial Optic Neuropathy36, 37, 38


Heredo familial optic neuropathies comprise of a group of hereditary degeneration of optic nerve that manifest under 15 years of age. The causative factor may be present at birth but the condition manifests late. They are generally bilateral, most of the clinical presentation in two eyes is similar. They progress gradually, may terminate in complete blindness, may have only ocular manifestation or may be multi-systemic in nature. Heredo familial optic degeneration can belong to following groups : 1. Optic abiotrophies 2. Associated with retinal dystrophies 3. Storage diseases. The common conditions are39 1. Lebers optic neuropathy 2. Kjers dominant juvenile neuropathy 3. Behrs recessive neuropathy 4. Optic neuropathy in juvenile diabetics.

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Lebers optic atrophy40. This is a heredo familial optic neuropathy also known as Lebers disease. This is not to be confused with Lebers congenital amaurosis which is a congenital retinal pigment degeneration and not a neuropathy. This disease is seen between 5 years to 30 years of age, commonest manifestation is at onset of puberty. Ninety percent affected are boys. The disease is passed on to children of carrier women who may or may not have the disease. The affected males do not pass the disease to their children including daughters. The affected child may have fully developed disease in his sibling or younger siblings may develop the disease later. Besides brothers there may be positive evidence of disease in maternal uncles. Most of the sisters of the affected child may be carriers. Female carrier do not have affected father. The social aspect of the inheritance is that it is not the affected man who spreads the pathological gene but the unaffected sister. The biogenetic defect of the disease lies in the mitochondrial DNA of the mother due to mutation. Several different mutations have been identified.41 There is defect in : Oxydative phosphorylation Cyanide metabolism Reduced zinc in plasma The disease starts as rapid fall of vision in both eyes. Occasionally one eye may be involved earlier than the other. Initial fall may be up to 6/60 or less. The initial fall may continue in slower rate and stops at lowest level then may start improving. Improvement is never beyond 6/24. There is partial or total loss of colour sense, stereopsis and contrast. There is a large dense absolute central or centrocecal scotoma which is surrounded by relative scotoma or a relative scotoma may become denser and absolute. On fundus examination, the disc may look normal or may be swollen. The disc develops secondary optic atrophy. The basic pathology is retrobulbar. The commonest fibres to be affected are papillo macular hence commonest type of optic atrophy is temporal pallor to begin with which can spread all over the disc. Peripapillary telangiectasia and micro vasculo angiopathy is common that disappears in late stages. Rest of the fundus is normal. There are no exudates or haemorrhages as seen in optic neuritis. The disease can be divided into following stages : 1. AsymptomaticThese are generally the siblings and carriers. They may show peripapillary telangiectasia and tortuosity of large vessels. 2. Stage of rapid fall of vision. 3. Stage of slow fall of vision and slight improvement of vision. 4. Stage of optic atrophy with some vision. 5. Blindness. Treatment. There is no specific treatment. In stage of neuritis, systemic steroids have been used without much success. Commonly used drugs are : 1. High doses of hydroxycobalamin given intra muscular. 2. Oral cystine. 3. Oral zinc.

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Fenestration of optic nerve sheath too has not shown good results. The children with non improvable vision should be given low vision aids and treated as partially visioned children. They should be encouraged to stay in schools with normal children and encouraged to learn profession that is compatible with low vision. Kjers juvenile optic neuropathy. This condition is less common than Lebers optic neuropathy but commonly diagnosed as Lebers because of its bilaterality and persistent visual loss. There is a strong family history. The disorder starts between 4 to 8 years. Vision is never less than 6/60. Never goes to loss of perception of light. There is acquired blue yellow dyschromatopsia. There is a central or centrocecal scotoma. Peripheral fields are normal. The optic nerve shows mostly temporal pallor with mild excavation of cup. There is no nystagmus. No treatment is effective. Low vision aid may help the child. Behrs recessive optic neuropathy. There are two types of recessive optic neuropathy, one is called simple and the other complicated. The simple starts in neonates hence also wrongly called congenital neuropathy. Though the disease starts in infancy, it is missed unless the eyes are examined with special reference to vision and changes in the disc. Generally the children are brought for examination round about school going age i.e. 3-4 years. Visual loss is severe that may be as low as H.M. Nystagmus is common which is generally associated with achromatopsia otherwise severe dyschromatopsia is common. However vision remains stationary. Consanguinity is common.38 There is no known treatment. Complicated. Commonest age of presentation is between one to nine years. Loss of vision is moderate. Children never go blind. In about half the children there is nystagmus and strabismus, there is moderate loss of colour vision. Disc shows temporal pallor. The vision is generally stable at 6/60-6/36. No treatment is known. Optic neuropathy in juvenile diabetics. This has a recessive form of inheritance. There is severe loss of vision that may be as low as counting fingers. It is a progressive condition. There is severe colour defect. Nystagmus is absent, there may be hearing loss. The disc has marked pallor. No treatment is available for optic neuropathy. Congenital optic atrophy42, 43. These rare conditions are generally confused with heredo-familial degeneration of optic neuropathy. The heredo familial neuropathies are not present at birth. There are two types of congenital optic atrophy i.e. dominant and recessive. The loss of vision is not severe and stable in spite of dead white colour of disc, and excavation. The whole of the disc is involved. Nystagmus is common. There may be other members of the family who have uncorrected visual defect from childhood. There may be dyschromatopsia mostly for blue colour. Gray (grey) pseudo optic atrophy37. This rare condition is seen in premature children, who are born with very poor vision that improves with in few months to normal level. On examination, the pupil are dilated and fixed. The colour of the disc is grey. The condition is thought to be delayed myelination of the disc.42

Tumours of Optic Nerve44, 49


Tumours of optic nerve are far less common than that of retina. The tumours of optic nerve can be seen any where from optic nerve head to chiasma.

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They can be grouped broadly into two groups45 1. EctodermalAnterior visual pathway gliomas 2. MesodermalMeningioma. The former is mostly seen in children and rarely in adults while glioma of the optic nerve is a benign or marginally malignant tumour in children. Its counterpart in adults is highly malignant. Meningiomas are not met with in children. The optic nerve glioma. It is a slow progressing mostly unilateral painless growth of substance of optic nerve. On rare occasions it can be bilateral. It can be divided into two types according to its clinical presentation i.e. glioma of the optic nerve and glioma of the chiasma. Glioma of optic nerve can arise anywhere in its orbital part. Posteriorly located tumours are more common than anteriorly located tumours. Occasionally the tumour may start in the anterior part and spread like a fusiform growth all along its length. Posteriorly placed gliomas are more likely to invade the optic canal. Similarly a glioma of chiasma may spread towards the orbit. The tumour is said to be congenital hamartoma, which does not manifest before 4 years of age. Its association along with neurofibromatosis is seen in 20-30% of cases. Hence in all cases of neurofibromatosis the optic nerve function should be evaluated that should include, vision, pupillary reaction, fundus examination, CT and MRI. A child with caf au lait spot anywhere on the body with diminished vision should be examined for presence of proptosis and visual function. Neurofibromatosis type I46 is more likely to be associated with glioma of optic nerve. Pathologically glioma arises from neuroglia of the optic nerve. Histopathologically the tumour is an astrocytoma. It is generally called juvenile pilocytic astrocytoma47, 49 due to thread like appearance of astrocytes in the tumour. There are two known patterns of the tumour : 1. Tumour related to neurofibroma are circumferential, perineural tumour, proliferating in subarachnoid space. 2. Intraneural tumour is not related to neurofibroma.48 Presentation. Common age is between 4 to 8 years. It may be diagnosed as late as end of second decade if the growth is small and slowly progressively. There are two modes of presentation : 1. Proptosis with diminished vision. 2. Strabismus with diminished vision. The later happens when diminished unilateral vision has not been noticed because of its gradual painless nature. Such children are brought mainly for squint and found to have glioma. Proptosis in case of glioma of optic nerve is axial, of gradual progress. Degree of proptosis depends upon position of growth, a smaller growth near the orbital apex causes more proptosis than a larger growth just behind the globe. The growth is always intraconal. It takes one to three months for a growth to cause proptosis in younger patients50. The proptosis is non tender, non pulsative and non compressible, there is no bruit present and the proptosis does

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not increase either by lowering the head or Valselva procedure. Generally examiners finger can be insinuated all round the globe and the orbit. The movements are normal or may be minimally reduced. Strabismus is due to loss of vision. Occasionally there is rapid progression of proptosis due to mucinous degeneration in the tumour and not true increase in number of cells. There is slow and uniform enlargement of orbit. Rarely in extreme degree of unattended proptosis, cornea may be abraded and develop ulcer. Vision. Loss of vision is a common feature of optic nerve glioma and is variable ranging between loss of few lines on Snellens chart to loss of perception depending upon number of visual fibres involved. Generally visual loss is early that deteriorates slowly, proptosis follows visual loss. Generally there is central or centrocecal field defect. Loss of vision is due to resultant optic atrophy. Anteriorly placed tumour cause post papilledematous optic atrophy while posteriorly placed and chiasmal lesions produce primary optic atrophy. Pupillary reaction also depends upon involvement of afferent pupillary fibres. Poor vision is associated with afferent pupillary reaction. Diagnosis of optic nerve glioma is not difficult in cases of moderate glioma which cause unilateral loss of vision with proptosis in a child under ten years of age. Investigation 1. X-ray : (a) X-ray of orbit shows uniform enlargement of orbit behind the orbital rim without any intraocular or intra orbital calcification. (b) X-ray optic foramen (Rhese view) shows circular enlargement of optic foramen with intact margin due to slow growth of the tumour. If the posterior end of the glioma has not extended in to the optic canal the x-ray of the foramen is unaltered and equal on both sides. In early cases X ray of both the foramen should be taken for comparison. When the growth extends in the optic canal, it too is enlarged most of the time in a funnel shape i.e. wider anteriorly. Size of the foramen is reduced following removal of growth. 2. Ultrasonography of orbit shows uniform fusiform or secular growth inside the cone of the eye. 3. CT and MRI are useful in early cases. 4. Fine needle biopsy has not proved to be more useful than CT, MRI or X-ray. Management. Management of optic nerve glioma is controversial. There is no medical treatment available. The main argument revolves round the question if the growth should be left as such or be surgically removed.

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Points against surgical intervention are : 1. It is slow progressive benign growth that is self limiting and after some time stops growing. 2. By removing the growth, the vision can not be restored. 3. The growth has to be removed either by a lateral orbitotomy, craniotomy or endoscopically via trans nasal, trans ethmoidal route that requires help of neurosurgeon and ENT surgeon. Only point in favour of surgical intervention is cosmetic. To a growing child, a large proptosed strabismic eye is not cosmetically acceptable. Surgical options available are : 1. Tarsorrhaphy if cornea is compromised before definitive surgical procedure. 2. Removal of the growth along with the eye if the exposed cornea has sloughed and perforated. 3. Removal of the growth leaving blood vessels either via orbitotomy, craniotomy or by transnasal and trans ethmoidal endoscopic surgery. Radio therapy is not of much use unless the growth is malignant, which is rare in children. Chiasmal glioma50, 51. Chiasmal gliomas are more common than isolated orbital glioma but are not diagnosed as early as orbital glioma for obvious reason that there is no proptosis which is a hallmark of optic nerve glioma and unilateral loss of vision in child may go unnoticed unless the child develops squint or nystagmus. The chiasmal gliomas have same pathological features as optic nerve glioma except that they are more likely to be invasive than optic nerve glioma. Chiasmal gliomas may produce bilateral loss of vision due to spread to the contra lateral side of the midline. Chiasmal glioma may extend : 1. In the optic canal and become intraorbital that results in proptosis. 2. Extend into the third ventricle and cause internal hydrocephalus, raised intracranial pressure, and hypothalamic signs and symptoms of precocious puberty, obesity, somnolence, dwarfism and diabetes insipidus. There may be involvement of pituitary as well. The presenting features are : Loss of vision, initially unilateral that may become bilateral. Vision may vary between mild to severe. In late cases there may be loss of perception as well. Squint and nystagmus are common. Afferent pupillary reaction is seen in unilateral involvement. In bilateral involvement both pupil are large and sluggish.

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Field changes of chiasmal glioma are variable, so long the growth has not crossed the mid line, the field changes are same as in optic nerve glioma. The other eye may also show unilateral field change of optic nerve type i.e. central or centrocecal scotoma. Bi-temporal hemianopia, which is expected in chiasmal lesion is absent in chiasmal glioma. X-rayOptic canal : Widening of optic canal means intra orbital extension. X-ray skull lateral view shows change in sella that appears either as Jor W shaped sella without supra sellar calcification. Management of chiasmal glioma is difficult more so when there is extension in the surrounding structure. Palliative radio therapy may arrest the growth. Tumours of optic nerve head : Growth of optic nerve head may be benign or malignant. Benign growths are : Drusen of optic nerve that may be intra neural and non visible ophthalmoscopy or may protrude in the vitreous as irregular grey mass. Other such growths are : neurofibromatosis and tuberous sclerosis. Another rare growth is benign melanocytoma which is not common in children. Remnants of hyaloid system may be mistaken as tumours arising from the disc. A malignant tumour that invades optic nerve head in children commonly is retinoblastoma. It is always associated with visible retinoblastoma and means extra ocular spread. Similarly neuroblastoma may occasionally invade the optic nerve head45 so can leukaemia.

REFERENCE
1. Nancy Anderson, Apple D. ; Optic nerve in Principles and practice of ophthalmology. Vol. I. p-5962, Edited by Peyman G.A., Sanders D.R. and Goldberg M.F. Jay Pee Brothers, New Delhi, 1987. 2. Duke Elder S. ; Optic nerve in System of ophthalmology. Vol. II. p-273294, Henry Kimpton, London, 1961. 3. Banumathy S.P. ; Optic nerve in Anatomy of the eye. p-6873, Edited by Natchair G. Arvind Eye Hospital, Maduari. 4. Nema H.V. ; The optic nerve in Anatomy of the eye and its adnexa. Second edition. p-8590, Jay Pee Brothers, New Delhi, 1991. 5. Shields M.B. ; Optic nerve head and peripapillary retina in Text Book of glaucoma. Fourth edition, p-7277, Williams and Wilkins, Philadelphia, 1997. 6. Anderson D.R., Braveman S. ; Re-evaluation of the optic disc vasculature. Am.J.Op. 82:165, 1976. 7. Nancy Anderson Hamming and Apple D. ; Anatomy and embryology of the eye in Principles and practice of ophthalmology. Vol. I. p-1314, Edited by Peyman G.A., Sanders D.R. and Goldberg M.F. Jay Pee Brothers, New Delhi, 1987.

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8. Duke Elder S. and Cook C. ; Embryology of the eye in System of ophthalmology. Vol. III, part I. p-109118. Henry Kimpton, London, 1963. 9. Dutta L.C. ; Congenital anomalies of the retina in Ophthalmology principles and practice. First edition. p-151153, Current Book International, Calcutta, 1995. 10. Kanski J.J. ; Congenital optic disc anomalies in Clinical ophthalmology. Second edition. p-451455, Butterworth, London, 1989. 11. Duke Elder S. ; Anomalies of optic disc and nerve in System of ophthalmology. Vol. II. p-668687, Henry Kimpton, London, 1964. 12. Shirly H. Wray ; Congenital optic disc anomalies in Manual of ocular diagnosis and therapeutics. Third edition. Edited by Deborah Pavan Langston, p-341342, Littlebrown. 13. Duke Elder S. ; Medulated nerve fibres in System of ophthalmology. Vol. II. p-646 650, Henry Kimpton, London, 1964. 14. Radhika Tandon, Verma L. ; Optic disc pit in Clinical practice in ophthalmology. First edition. Edited by Saxena S., p-494, Jay Pee Brothers, New Delhi, 2003. 15. Shields J.A., Stephen R.E. and Sarin L.K. ; Morning glory syndrome in Pediatric ophthalmology. Vol. II. Second edition. Edited by Harley R.D., p-1152, WB Saunders Company, Philadelphia, 1983. 16. Mukherjee P.K. ; Congenital pit of the optic nerve in Current ocular therapy. Fifth edition. Edited by Fraunsfelder F.T. and Roy F.H., p-558, WB Sanuders Company, Philadelphia, 2000. 17. Glaser J.S. ; Optic disc - congenital anomalies of shape and size in Neuro ophthalmology. p-7374, Harper and Row, London, 1978. 18. Martyn L.J. ; Optic nerve hypoplasia in Pediatric ophthalmology. Vol. II. Second edition. Edited by Harley R.H. p-831, WB Saunders Company, Philadelphia, 1983. 19. Levine R.A., Rosenberg, M.A. and Rabb M.A. ; Drusen in Principle and practice of ophthalmology. Vol. III. Edited by Peyman G.A., Sanders D.R. and Goldberg M.P. p-21302132, Jay Pee Brothers, New Delhi, 1987. 20. Dutta L.C. ; Papilledema in Modern ophthalmology. Vol. II. Second edition. p-977981, Jay Pee Brothers, New Delhi, 2000. 21. Shirley H. Wray ; Papilledema in Manual of ocular diagnosis and therapy. Third edition. Edited by Deborah Pavan Langston. p-343, Little Brown. 22. Kuhn F., Halda, T. ; Optic nerve in Optic differential diagnosis. Edited by Roy, H. p-618619, Williams and Wilkins. Baltimore, 1996. 23. Manikavasagan T. ; Pathogenesis of papilledema. Recent concept in Neuro ophthalmology. Edited by Natchiar G. p-1101 to 11-04, Arvind Eye Hospital, Maduri. 24. Kanski J.J. ; Papilledema in Clinical ophthalmology. Second edition. p-447448, Butterworth, London, 1989. 25. Badrinath S.S. ; Papilledema in Sankar Netralaya Clinical practice and pattern in ophthalmology. First edition. p-288-289, Jay Pee Brothers, New Delhi, 2004. 26. Sundarajan S. ; Optic neuritis in Neuro ophthalmology. Edited by Natchiar G. p-12.112.8, Arvind Eye Hospital, Maduri.

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27. Horton J.C. ; Disorders of the eye in Harissons principles of internal medicine. 14th edition. Vol. I. p-159172, McGraw Hill Book Company, 1988. 28. Brain W.R. ; Lesion of optic nerve in Diseases of nervous system. 6th edition, p-125 132, Oxford University Press, London, 1962. 29. Bajandas F.J. and Kline L.B. ; Pale optic disc in Neuro ophthalmology review manual. Third edition. p-135140, Jay Pee Brothers, New Delhi, 1989. 30. Miller S.J.H. ; Degeneration of the optic nerve : optic atrophy in Parsons diseases of the eye. Seventh edition. p-229230, Churchill Livingstone, 1984. 31. Lois J. Marthyn ; Opticatrophy in Pediatric ophthlmology. Vol. II. Second edition. Edited by Harley RD. p-817820, W.B. Saunders Company, Philadelphia, 1983. 32. Levine R.A., Rosenberg M.A., Rabb M.F. ; Optic atrophy in Principles and practice of ophthalmology. Vol. III. p-21252129, First edition. Jay Pee Brothers, New Delhi, 1989. 33. Hebber K.G. ; Optic atrophy in Neuro ophthalmology. Ist edition. Edited by Natchair. p-13.113.12, Arvind Eye Hospital, Maduri. 34. Dutta L.C. ; Optic neuritis : Optic neuropathy in Modern ophthalmology. Vol. II. Second edition. p-956962, Jay Pee Brothers, New Delhi, 2000. 35. Miller M.R. ; Traumatic optic neuropathy in Current ocular therapy. Edition V. p-567568, Edited by Fraunfelder F.T. and Hampton Roy F., WB Saunders Company, Philadelphia, 2002. 36. Sorsby A. ; Maldevelopment defectsLebers disease in Modern Ophthalmology. Vol. III. First edition. p-311319, Butterworth, London, 1964. 37. Lois Martyn ; Lebers hereditary optic atrophy in Pediatric ophthalmology. Vol. II. Second edition. p-818820, W.B. Saunders Company, Philadelphia, 1983. 38. Glasers J.S. ; Heredo degenerative optic atrophies in Neuro ophthalmology. p-93 100, Harperz Row, New York, 1977. 39. Bajandas F.J. and Kline L.B. ; Pale optic disc - optic atrophy in Neuro ophthalmology. Review manual. Third edition. p-139, Jay Pee Brothers, New Delhi, 1989. 40. Gittinger J.W. ; Lebers optic atrophy in Manual of clinical problems in ophthalmology. First edition. p-167168, Edited by Gittinger J. and Asdourian G.K. Little Brown and Co., Boston, 1998. 41. Seal S.K. ; Retrobulbar neuritis in G.N. Seals Text Book of Ophthalmology. Fifth edition. p-340341, Current Book International, Kolkota, 2002. 42. Duke Elder S. ; Congenital optic atrophy in System of ophthalmology. Vol. III, part II. p-683684, Henry Kimpton, London, 1964. 43. Sorsby A. ; Congenital optic atrophy in Modern ophthalmology. Vol. III. p-264-265, Butterworth, London, 1964. 44. Green C.H. ; Orbital gliomas in Ocular pathology. First edition. p-181-183, Blackwell. Scientific publication, Oxford, 1963. 45. S.J.H. Miller ; Primary tumours of optic nerve and its sheath in Parsons diseases of the eye. Seventh edition. p-335337, Churchill Livingstone, London, 1984.

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46. Hoyt W.F., Baghdassarian S.A. ; Optic glioma of childhood; natural history and rationale of conservative treatment. BJO 53:793-798, 1969. 47. Levine R.A., Rosenberg M.A., Rabb M.F. ; Glioma in Principles and practice of ophthalmology. Vol. III. p-21362142, Edited by Peyman G.A., Sander D.R. and Goldberg M.F. First Indian edition. Jay Pee Brothers, New Delhi, 1987. 48. Lois J. Martyn ; Optic glioma in Pediatric ophthalmology. Vol. II, Second edition. Edited by Harley R.D. p-839841, W.B. Saunders Company, Philadelphia, 1983. 49. Nicholson D.H. and Green W.R. ; Glioma in Pediatric ophthalmology. Vol. II. Second edition. Edited by Harley R.D. p-12591262, W.B. Saunders Company, Philadelphia, 1983. 50. Natrajan M. ; Tumours of the optic nerve in Neuro ophthalmology. Edited by Natchiar. p-14.114.10, Arvind Eye Hospital, Maduri. 51. Glaser J.S. ; Optic gliomas in Neuro ophthalmology. p-116120, Harper and Row, London. 52. Radhika Tandon and Verma L. ; Optic neuritis in children in Clinical practice in ophthalmology. First edition. p-480, Edited by Saxena S. Jay Pee Brothers, New Delhi, 2003. 53. Scheie H.G., Albert D.M. ; Optic neuritis in Text Book of ophthalmology. 9th edition. p-39, W.B. Saunders Company, Philadelphia, 1977. 54. Dutta L.C. ; Multiple sclerosis in Modern ophthalmology. Vol. II. Edition II. p-958 960, Jay Pee Brothers, New Delhi, 2000. 55. Radhika Tandon and Verma L. ; Optic nerve disorders in Clinical practice in ophthalmology. Edited by Saxena. S., First edition. p-477495, Jay Pee Brothers, New Delhi, 2003. 56. Levine R.A., Rosenberg, M.A. and Rabb M.F. ; Degenerative optic neuritis in Principles and practice of ophthalmology. Vol. III. First Indian edition. p-21152129, Edited by Peyman G.A., Sanders D.R., Goldberg M.F. Jay Pee Brothers, New Delhi, 1987. 57. Miller S.J.H. ; Diseases of the optic nerve in Parsons diseases of the eye. Nineteenth edition. p-224, Churchill Livingstone, London, 1984.

CHAPTER

15

Errors of Refraction in Children


General consideration1, 2, 3, 4 A normally developed eye (by 5 to 6 years of age) acts as a convex lens of +60D. This power is divided into two major components i.e. the corneal and lenticular. The converging power of cornea is +43D. This leaves the lens with +17D of power, normal aqueous and vitreous contribute a negligible converging power. In an eye with normal refractive (diopteric) power, parallel rays are brought to focus on the retina with accommodation at rest, the parallel rays actually form a circle of least diffusion4. This refractive status is called emmetropia. An emmetropic eye has clear image of a distant object without internal adjustment of its optics.6 In contrast to this, if all parallel rays are not brought to focus on the retina in all the meridian with accommodation at rest, the condition is called ametropia. Almost all emmetropic eyes have an axial length of 24 mm, any change in its length should produce ametropia. However a larger eye can be emmetropic if its total optical power is stronger.8 An absolutely emmetropic eye is extremely rare, most of the eyes have some degree of ametropia. A full term normal child at birth is about +2 to +3D hypermetropic. This is due to shorter axial length of the new borns eyeball. As the child grows, this power is neutralised by corresponding lengthening of the eyeball up to 5-7 years when all eyes should become emmetropic. However, if the increase in length does not stop at this point, the eye becomes myopic and if the eye fails to reach the emmetropic length (24 mm) the eye becomes hypermetropic. The ametropia eye can be : 1. Myopic 2. Hypermetropic 3. Astigmatic Anisometropia is a term used when the diopteric power in both eyes are not the same. Possible causes of ametropia4 are: 1. Axial. In this condition, the retina is either in front or behind the circle of least diffusion. If the focus is behind the retina, the condition is called hypermetropia and if the image is formed in front of the retina, the condition is called myopia. Axial astigmatism is very rare. 1 mm change in axial length leads to ametropia of 3D. 546

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2. Curvature. If the curvature of the cornea is uniformly less than normal, the eye develops curvature hypermetropia, similarly reduction in lenticular curvature also results in curvature hypermetropia. Increase in corneal or lenticular curvature results in curvature myopia. 1 mm change in corneal curvature leads to 6-7D ametropia. If the corneal or lenticular curvatures are not equal in all meridian, there will be an ametropia called astigmatism. 3. Index. If the refractive index of optical system is low, it will result in hypermetropia and high refractive index will result in myopia. 4. Relative position of lens. A forward shift of lens causes myopia, backward shift results in hypermetropia. An absence of lens from the pupillary area results in aphakia, which is an example of high hypermetropia. An obliquely placed lens causes astigmatism.

MYOPIA
Myopia is the commonest cause of gradually developing painless diminished vision in pediatric age group. It is equally common among boys and girls. It has strong heredity. Fifty percent of myopic children have myopia in both the parents, about a quarter of myopic children have one parent with myopia, and rest are sporadic. Both the hereditary and sporadic cases can transmit myopia in the next generation. It is common to have history of myopia in grand parents from maternal or paternal side. The first cousins too may be myopes. It is very common to have myopia in all or some of the siblings. Myopia invariably is a bilateral disorder that manifests simultaneously in both eyes that need not be equal, generally difference in two eyes is not much. In simple myopia rest of the eye is generally as normal as any non myopic eye. Myopia is the error of refraction in which rays from far point, real or apparent (myopic far point) are focussed in front of photo sensitive layer of retina when accommodation is at rest.1, 2, 3 Myopia is also known as short sightedness or near sightedness as the patient finds it difficult to see beyond a certain distance that may be very short.8 The word myopia represents the phrase I shut the eye in Greek. This is because a myopic child narrows the inter-palpebral fissure so much as to make it like a slit. This gives a pin hole effect that improves vision by few lines on Snellens chart. Loss of vision in myopia is so gradual that the child fails to notice its onset and progress. Sometimes the child considers visual status as normal and is resigned to it. Classification of myopia. Myopia has been classified in various ways, none of the classifications have universal acceptance. It could be primary or secondary. Primary could be congenital or developmental. In secondary myopia some detectable cause other than increased axial length of the eyeball is present.

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near the eye gives slight enlargement of the image. These two phenomenon makes the child to keep the reading material close to the eyes. Myopic eyes are prone for peripheralretinal and vitreous degeneration that make myopic eyes more prone for retinal detachment. Congenital myopia. This is a rare form of myopia. The child is born with eyes longer than 24 mm, may be as long as 27 to 28 mm. It is mostly unilateral, occasionally both eyes may be involved. The condition is not diagnosed at birth. It may take as much as two to three years for parents to be aware of the condition. Unilateral larger eye, squint or nystagmus may be the early signs to draw attention of the parents. Generally the child is brought for unilateral squint and on examination presence of myopia is revealed, which is generally 10 diopter and generally does not progress.9 Congenital myopia is more common in pre-term babies. It may be associated with other congenital anomalies of the globe i.e. microphthalmos, aniridia, megalocornea, cataract and retinal separation. Congenital myopia develop all the complication that are seen in simple myopia i.e. peripheral retinal degeneration and retinal degeneration. Management consists of early detection of myopia and optical correction either by spectacles or contact lenses after refraction under cycloplegia. Contact lenses are generally prescribed when the child is grown enough to manage the contact lenses. However, extended wear lenses may be prescribed in younger children with instruction to parents regarding its management. Amblyopia when present should be treated as per standard method. Surgical intervention is not indicated before eighteen years of age. Simple myopia10. This is the commonest form of myopia met in children. It is also known as developmental myopia. The exact etiology is not known. It is considered to be derangement of physiology of the developing eye. It is inherited as autosomal dominant. However, some children may have autosomal recessive inheritance. It is equally distributed among various races. Boys and girls are equally effected. There is a strong hereditary tendency. Simple myopia is common among siblings. Commonest type of simple myopia is axial myopia, where the eye is too long for its refractive status. Role of excessive near work has been unequivocal, so is the role of diet and general health. Simple myopia is generally progressive but not pathological any progress is mild to moderate, about one third children have stationary myopia, only 15% have progressive increase in power. Even in progressive form, the vision can be corrected to normal with change of lens. The children at birth are not myopic. Simple myopia is generally detected between five to seven years of age and continues to grow up to late teens. Hence called school myopia which may start as 1.00 D or less but does not exceed 5D or 8D when fully developed. There is no known method to stop the growth. Associated astigmatism is common. Another type of myopia that develops after 15 years of age and progresses into second decade is called college myopia. It is a low grade of myopia which rarely exceeds 2D to 2.5D diopter and difference between the initial power and final power is seldom more than one diopter.

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As the punctum remotum of a myopic eye is always finite, the myopic eye need not accommodate. Accommodation worsens the vision. Hence accommodation in simple myopic eye remains unused. Convergence is within normal range, or may be in excess. The results in dessociation between accommodation and convergence leading to exophoria which may break into exotropia.10 However excess convergence may lead to esotropia as well. A negative angle alpha in myopia leads to pseudo convergent squint. Symptoms. Most frequent and striking symptom is diminished distant vision. A child may have normal distance vision at a given time, which may rapidly deteriorate to 6/24 to 6/36 with in one year. These children may not complain of diminished vision as their vision is good enough for reading, writing and indoor activities. They find it difficult to see the writings on the black board in the school, or read the sign boards. These children do not see the television from usual distance. They have tendency to move nearer to the TV set for better view. These children prefer to keep books closer to eyes than other children. As the child has poor distant vision that he takes for granted, the child concentrates on near work like reading, writing and other indoor activities with less interest in outdoor sports. They become introvert. They generally do well in studies. Other symptoms are glare, and asthenopia. Asthenopia is more common in low myopia associated with astigmatism. Signs. A eye with low degree of myopia is difficult to differentiate from emmetropic eye. The child may be termed as squinting by parents. The child actually may narrow the inter palpebral fissure for better vision. This phenomena is mistaken as squint by parents. However eyes with more than three diopters may look larger than normal eye due to axial increase in length. Higher degree of myopia may present with pseudoproptosis. The axial enlargement is confined to posterior sclera. The cornea is relatively larger and anterior chamber is deeper than normal. The pupil is larger than normal, may look sluggish on torch light examination but brisk on slit lamp. Fundus is within normal range. After few years temporal crescent and peripheral retinal changes in the form of lattice degeneration may take place putting the child on higher risk of retinal detachment. Diagnosis. Diagnosis is simple on the basis of diminished distant vision, good near vision, positive family history and improvement of vision by three four lines with PH. The diagnosis should be confirmed by retinoscopy under cycloplegia. Subjective test with minus glasses that improve should not be relied as that fails to unmask pseudomyopia. Management of simple myopia. The aim of treatment is to bring the image back on the retina from its forward position. This is achieved by placing concave glasses in front of the eye either as spectacles or contact lens. The next alternative available is a group of surgeries that flatten the cornea. These surgeries are not indicted in children. In an attempt to form the image on the retina, the aim should be to give minimum minus power that gives maximum and comfortable vision. In children under 7 to 8 years, with low to moderate degree of myopia (up to 6D), myopia is fully corrected by spectacle. The child is instructed to use the glasses constantly.

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This gives a bright image on retina for maximum waking hours. This prevents development of squint and helps develop normal accommodation convergence reflex. A fully corrected myopic child does not find it difficult to perform near work with distant correction. The child can read smallest letter without glasses by bringing the reading material near the eye, hence develops a habit of reading without glasses. The child should be encouraged to use the glasses for both distant and near work to develop a proper reading habit. The spectacles are the most practical form of device to correct myopia, they are easy to handle. A child as young as one year can be taught to wear them, the spectacles give good and comfortable vision. They are cheap and the power can be changed with ease. The disadvantages of spectacle correction of myopia consist of minification of image. A child who was accustomed to larger image finds a smaller image unacceptable and too bright. The other disadvantage is unacceptability of glasses in teens for cosmetic purpose. The field is also restricted. The next alternative is to prescribe contact lenses. They are not suitable for children under ten years who can not manage to wear and remove them. The first advantage of contact lens is its invisibility that removes the cosmetic blemish of spectacle. Other advantages are optical. They give larger image, improved field all around, aniseikoina can be minimised when present. Greatest disadvantage of contact lens is childs inability to handle the contact lens and maintain its sterility. They are expensive and may require frequent change.7 Pathological myopia. This type of myopia is also known as progressive or degenerative myopia. It begins as simple myopia but increases relentlessly and always associated with extensive vitreoretinal degeneration. Macular involvement is common. The refraction may increase by -3D to -4D every year in contrast to simple myopia that increases by 1D to 1.5D yearly and get stabilised before end of first decade. In small percentage of cases of pathological myopia, the increment in power may extend up to 30 years of age and final may be as high as 20D to 25D. The exact cause of pathological myopia is not well understood. Heredity seems to play a definite role. It runs in families11, female are more prone to develop pathological myopia. It is more common in Asians and rare Africans. Symptoms. Most prominent symptom is diminished distant vision, which is very poor. But the child can read and write, in fact can do fine work without difficulty from very close quarter. Next common symptom is seeing fine black spots in front of eyes. The spots may range from single to multiple, mostly in straight or curved lines. These opacities move with movement of the eyes. They are called muscae-volitantes. They are due to degeneration of vitreous. They do not require any treatment. Sudden increases in number of black spots and enlargement in size of spots require examination of fundus by indirect ophthalmoscope to detect presence of retinal detachment. Children with pathological myopia have poor dark adaptation which they may call night blindness. This is due to extensive peripheral chorioretinal degeneration. Squint mostly esotropia is common in pathological myopia due to excess of convergence and under action of lateral rectus.

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Sudden profound loss of vision, photopsia, loss of field always means myopic retinal detachment. Other cause of sudden painless loss of vision are haemorrhage in front of the macula or large vitreous haemorrhage. Signs. The signs are similar to those seen in simple myopia. However prominence of eyeball is more due to stretching of the globe mostly beyond the equator giving an impression of pseudo proptosis. Cornea is larger but not as large as in megalocornea or buphthalmos. Any cornea larger than 12 mm in diameter should be investigated to rule out buphthalmos. AC is deep and pupil larger and sluggish. On retinoscopy the retinal glow looks dull and moves slowly against the movement of the retinoscope, as more and more minus lenses are added, the glow looks brighter. A concave mirror retinoscope is helpful in retinoscopy in progressive myopia. The refraction may be between 10-20D. Large vitreous floaters may be visible as black spots against pink background. White reflex amidst pink glow is due to large patches of choroidal degeneration. Absence of glow in presence of normal anterior segment is caused due to large retinal detachment or vitreous haemorrhage. Fundus examination. Most striking features of pathological myopia is seen with ophthalmoscope. Direct opthalmoscope gives out findings only round the posterior pole. To have a good view of whole of the retina of every fundus should be examined by indirect ophthalmoscope under full mydiasis. The fundus changes are seen on optic disc, macula, choroid and retina in various combination. The fundus changes clinically can be divided into early and late changes. The fundus changes increase with duration of myopia and not always directly proportional to degree of myopia. The early changes develop in first and second decade12 consisting of diffuse atrophic changes in the retina and choroid in the posterior pole, which include pale retina through which prominent choroidal vessels are seen. The retina gradually gets atrophic, soon a myopic crescent develops mostly on temporal side of the disc extending from upper pole of the disc to lower pole with widest area at temporal side. Occasionally a nasal crescent may also develop. A complete ring is rare. The crescent is more pale, almost atrophic near the disc and pink on temporal side that is dotted with choroidal pigment. The crescent gradually becomes more extensive and spreading towards macula. The nasal margin may overhang, the optic disc resulting in distortion of retinal vessels. The optic nerve seems to enter the globe obliquely. The macula in early stages is normal. The retinal periphery may be normal or may show minimal degenerative changes. The late changes are : Optic disc is pale and large. The largeness of the disc is not real, it is optical. The disc has prominent myopic crescent. Peripapillary crescent is more frequent in late stages. There may be super traction of retina over the disc. The cup is proportionately large but cup disc ratio is within normal limits. Choroid. In early stagesThe choroidal vessels are visible through the pale retina. In late stages there is atrophy of chroiocapillaries and retinal pigment epithelium. In still late

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stages there is total disappearance of choroidal tissue with large patches of atrophy. The white sclera shines through the atrophic, choroid and retina. There is heaping of pigment on the periphery similar to be seen in healed patch of choroiditis. The choroidal changes do not extend in anterior segment. The macula. The macula develops a dark red circular patch surrounded by a pale ring. This is called Foster-Fuchs spot of Fuchs fleck. This is due to choroidal thrombosis13 and choroidal neovascularisation.14 Bruchs membrane develops breaks due to over-stretching. These breaks look like fine yellow irregular lines that may branch. They are more common in young myopes. They are called lacquer cracks. Presence of these breaks in Bruchs membrane carry poor prognosis as they may cause necovascularisation and haemorrhage.14 Peripheral retinal degeneration in the form of lattice, snail-track or cystoid degeneration are common. They themselves are not dangerous but may result in retinal hole formation leading to rhegmatogenous retinal detachment which are more common in myopia than emmetropia or hypermetropia. Trauma to pathological myopic eye is more likely to cause retinal detachment than in other eyes. Posterior staphyloma is unique to pathological myopia. It is due to thinning and stretching of sclera in the posterior pole. The thin sclera becomes ectatic and lined by choroid with all the layers of retina along with blood vessels. The blood vessels dip in the ectatic area. On retinoscopy, the posterior staphyloma has more minus power than the surrounding area. Direct ophthalmoscopy requires more minus addition to see the floor of the staphyloma, which is better seen by indirect ophthalmoscope. Ultrasonography also shows up its presence, depth and extent. Vitreous changes are very common. They are degenerative that include posterior vitreous detachment, formation of large vitreous opacities, liquefaction of vitreous. Like all degenerative changes in eye, the changes in vitreous are also not directly proportional to myopic changes. Peripheral fields also shows constriction. Management. Management of pathological myopia is same as in simple myopia except that pathological myopia does not improve to 6/6 with best correction. 6/12 or 6/18 may be the best possible correction in pathological myopia. The second problem is more frequent change in power, the yearly change may be as much as 3D to 4D. It is better to under correct high myopia by -1 to -2D for more comfortable distant as well as near vision than better and uncomfortable distant vision. With increase in power, the image becomes smaller and smaller that adds to discomfort to the child. As pathological myopia have large and sluggish pupil, they are more prone to develop glare, which can be reduced by prescribing tinted glasses. While prescribing spectacles to children it is better to inform their parents that glasses are being given to improve distant vision. Use of glasses is neither going to stop progress of myopia nor reduce the power of myopia. It should also be impressed that the glasses are not specific for reading but should be used for near work to keep balance of accommodation and convergence.

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The next alternative is contact lens. Contact lenses are preferred in high myopia because they do not cause minification of images as seen in spectacles. They reduce peripheral distortion that is common with high myopia glasses. Peripheral field also improves with contact lens. Moreover contact lenses are cosmetically more acceptable than spectacles. The disadvantages of contact lens are their high cost, frequent change, difficulty in handling by children and possibility of non tolerance of contact lenses. Previously, it was thought that if hard contact lenses, that were flatter than corneal curvature, if worn for sometime will flatten the cornea and reduce the power. This hypothesis is known as orthokeratology.15 The philosophy behind the procedure has not been found to be acceptable. Surgical procedures for correction of myopia : Many surgical procedures have been developed to correct myopia. They can be broadly divided into following groups : 1. Surgery on cornea 2. Surgery on lens 3. Intraocular lens implant 4. Posterior scleral support Common surgical procedures on cornea are : 1. Radial keratotomy (RK) 2. Photo refractive keratectomy (PRK) 3. Lasik (Laser in situ keratomileusis)16 4. Lasek (Laser sub epithelial keratomileusis) 5. Intracorneal ring Surgical procedures on lens : 1. Clear lens extraction 2. Phakic intraocular lenses (i) Posterior chamber lens (ii) Angle supported lens (iii) Iris supported lens Of all the surgical procedures mentioned above, only clear lens removal and phakic intraocular lenses are suitable for myopia in older children. Rests of the surgeries are at present considered to be contra indicated. They are suitable only after myopia has stabilised which occurs round about twenty-one years of age. Other forms of myopia : 1. Curvature myopiaCornealKeratoconus LenticularLenticonus 2. Index myopia. This is more common in adults beyond fourth decade as nuclear sclerosis. However any condition that increases refractive index of lens in children can also result in myopia i.e. diabetes, concussion injury.

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Similarly stromal edema of the cornea can also produce some degree of myopia. 3. Positional myopia. This infrequent condition occurs due to forward displacement of lens which by itself is rare. 4. Pseudophakic myopia. This is common following IOL implant in congenital cataract where the power of the IOL may be too strong for the eye. 5. Pseudomyopia. This is common in hypermetropic children who accommodate too much and make the eye myopic. Pseudomyopia may occur due to spasm of accommodation that may follow blunt injury to the globe, iridocyclitis or due to instillation of miotics. 6. Myopia of prematurity23. This type of myopia is commonly seen in children with birth weight of 1250 gm or less (a birth weight that predisposed retinopathy of prematurity). There is a fluctuation of myopia that may range between 10D to 20D. The myopia is reduced to 2 to 6D by 6 months and is altogether disappears by 1 year. The cause of the condition is not known. 7. Drug induced myopia. Some of the drugs taken orally can cause transient low degree of myopia. Commonest example is acetazolamide induced myopia. 8. Space myopia 9. Night myopia The last two are not seen in children.

Hypermetropia (Hyperopia)
Hypermetropia is commonest form of error of refraction seen in children under five Most of them are symptom less. The condition draws attention less frequently than myopia where diminished vision is poor enough to draw attention of the child or relatives. years.3 Hypermetropia is also called farsightedness, which is irrelevant8 as the children may sometimes have diminished distant vision as well, in contrast to myopia where the children always have poor distant vision but good near vision. Hypermetropia is a state of refraction where parallel rays are brought to focus behind the photo sensitive layer of the retina and the image formed on the retina is blurred circle when accommodation is at rest.3 At birth almost all eyes are hypermetropic by 2 to 3 diopters which gradually gets neutralised due to growth of the eyeball and theoretically the eye should be emmetrope at puberty, but in practice only half of the eyes reach a state of emmetropia. About 90 percent of these children who do not reach emmetropia remain hypermetropic at five years of age. This percentage is reduced to 50 percent by sixteen years. Hypermetropia is considered to be a state of underdeveloped eye. The eye is shorter than emmetropic eye. As a rule the shortening is not too much in contrast to myopia where the lengthening is generally in higher order. In hypermetropia, the shortening is rarely more than 2 mm, hence it is unusual to have more than 6D of hypermetropia in case of simple hypermetropia.

556 Etiologically hypermetropia can be : 1. Axial 2. Curvature 3. Index 4. Displacement of lens

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Out of all, the axial hypermetropia is the commonest form. This can be part of a shortened length or the sclera may be pushed forward by a retrobulbar mass or the detached retina may come forward. The later two are pathological causes of hypermetropia. Other causes of short eyeball are microphthalmos and nanophthalmos. Curvature hypermetropia occurs when curvature of either cornea or lens is smaller than normal. Reduced curvature of cornea is more probable cause of curvature hypermetropia than lens. A flattening of curvature by 1 mm results in hypermetropia of 6 diopter. Astigmatism is more common in curvature hypermetropia than axial hypermetropia. The causes of flattening of cornea are : Cornea plana, micro cornea, microphthalmos, post trauma and post surgical status. The lens is flattened in buphthalmos. Index hypermetropia is least common cause of hypermetropia in children. This is commonly seen in diabetic children under treatment. Displacement of lens posteriorly causes hypermetropia that could be congenital or following trauma. Absence of lens from pupillary area (aphakia) causes highest amount of hypermetropia. Optical status of a hypermetropic eye. The parallel rays are focussed behind the retina. The rays arising from the retina going out of the eye are divergent and seem to meet behind the globe. As the retina is nearer the nodal point, the hypermetropic image is smaller than emmetropic. Accommodation in hypermetropia. Accommodation plays an important role in correcting part of hypermetropia as against myopia where accommodation worsens the myopia. A child has stronger accommodation than an adult. Hence a child is in advantageous position in correcting hypermetropia without glasses than an adult. According to available accommodation, hypermetropia can be divided into two main groups i.e. 1. Latent hypermetropia and 2. Manifest hypermetropia. The sum total of the two is called total hypermetropia. Latent hypermetropia is that part of hypermetropia that is corrected by physiological tone of ciliary muscles. This ranges between 0.5D to 1.0D. This is measurable only following paralysis of accommodation by cycloplegia. Latent hypermetropia is the difference between total hypermetropia and manifest hypermetropia. The manifest hypermetropia is divided into : 1. Faculatative hypermetropia and 2. Absolute hypermetropia.

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1. Facultative hypermetropia. This is corrected by available accommodation over and above the latent hypermetropia. Most of the children have more facultative hypermetropia and less absolute hypermetropia. With age amount of facultative hypermetropia gets reduced and replaced by absolute hypermetropia. 2. Absolute hypermetropia is that part of manifest hypermetropia that is not corrected by accommodation. Symptoms of hypermetropia. Symptoms of hypermetropia depends upon amount of accommodation available. 1. No symptoms. Children with low degree of hypermetropia may have no symptoms and hypermetropia may be unmasked only when cycloplegic is used for examination of fundus without defective vision. 2. Asthenopia. The next stage is when moderate hypermetropia is fully corrected by accommodation, resulting in normal or near normal distant vision. This accommodation has to be exerted throughout the day to maintain normal vision. This sustained action of ciliary body leads to asthenopic symptoms of 1. Headache that is generally frontal. 2. Tiredness of the eye 3. Mild photophobia and watering. The asthenopic symptoms worsen by evening or after prolong near work. These children generally complain of headache after coming from school and do not complain on holidays. 3. Asthenopia with diminished vision. When the hypermetropia is not corrected by accommodation, the child complains of diminished vision, first in the form of difficulty in near work and then diminished distant work. The latter is less frequent in healthy children. 4. Defective vision. If hypermetropia is high between 4D to 6D, the patient generally gives up accommodation and absolute hypermetropia is precipitated and patient complains of diminished near vision in pre-presbyopic age and diminished distant vision. 5. Pseudo myopia. A child may over accommodate to achieve good distant vision. In this effort the child may overshoot the emmetropia and become myopic, which again causes diminished distant vision that may be wrongly corrected subjectively by minus glasses worsening the condition. Thus all children should get their glasses only after refraction under cycloplegia. 6. Squint sensation. Some children may complain of squinting without diplopia, in absence of obvious squint, however, there may be esophoria. 7. Squint. A child presents with esotropia and is found to have high hypermetropia. 8. Recurrent blepharitis, stye and chalazion. 9. Some children may hold the book too close to the face and may be mistaken as myopic. The phenomenon of keeping books near the eyes give larger retinal image that compensates for poor vision.

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10. The children may have other ocular deformity like microphthalmos, microcornea or nanophthalmos. Signs : 1. Similar to low grade of myopia, an eye with low hypermetropia looks as normal as emmetropic eye. An eye with moderate to high hypermetropia is typically a small eye not only in anterio posterior diameter but in all directions.3 The cornea is smaller than emmetropia and the anterior chamber is shallower. The pupil is also small. In case of pathological hypermetropia coloboma of uvea is not an uncommon finding. 2. The face of the child on close inspection may not be symmetrical. Hypermetropia is generally more on the asymmetrical side and is associated with astigmatism. 3. Pseudo divergent squint. This is due to large positive angle alpha. (kappa) 4. Esotropia. This is due to high hypermetropia i.e. between +4D to +7D. And mostly is an accommodative esotropia. 5. Fundus. Overall size of the fundus is smaller than the emmetropic eye with small disc, which resembles optic neuritis without visual symptoms. This is called pseudo neuritis. Generally fundus background has a peculiar sheen that is called shot silk appearance. The blood vessels reflect more light than emmetropia. The macula is generally situated away from the disc. Management of hypermetropia. Management of hypermetropia like myopia is optical or surgical. Surgical treatment is not indicated in children. Optical treatment consists of prescription of spectacles or contact lens, which depend on available accommodation. There is no rule of thumb according to which power of the lenses can be determined. A rough method is 1. Children below six years. Some accommodation for distance is physiological at this age and the child may not be aware of presence of hypermetropia. These children are asymptomatic and need no treatment. If there is a family history of hypermetropia, these children should be refracted under cycloplegia. Treatment is called for if there is evidence of asthenopia or muscle imbalance. 2. In school going children. Who requires prolonged near work, require correction even of smaller degree. 3. Children with accommodative squint and diminished vision should be corrected. If the error of refraction is more than +3D, the child should be given glasses for constant use. Glasses less than +3D may be worn for near work only. In children under six years, the power of glasses should be +1.5D to +2.0D less than the objective finding. In children above six years, full correction with good vision should be prescribed.

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As the child grows, some of axial hypermetropia gets reduced and the refraction may swing towards emmetropia. In rarer instances the accommodation may worsen with corresponding increase of hypermetropia in children in second decade. Keeping in mind the above factors, all hypermetropic children should get yearly ophthalmic check and undergo corresponding adjustment. Contact lenses in hypermetropia. In principle contact lenses can correct any type of hypermetropia. It is better to prescribe contact lenses in hypermetropic children after hypermetropia has stabilised. Other indications are anisometropia and uniocular hypermetropia. Surgical management of hypermetropia. Refractive surgery for hypermetropia are not as rewarding as in myopia. Following surgical procedures are available. 1. Hexagonal keratotomy 2. Photo refractive keratectomy 3. Lasik 4. Laser thermal keratoplasty 5. Phakic intraocular lens Like surgical treatment of myopia, the above procedures are not indicated in children. Other types of hypermetropia seen in children are : 1. Aphakia 2. Pseudophakia 3. Over corrected myopia. Aphakia : Aphakia in children is less common than seen in adults. Aphakia literally means absence of lens from the eye. From clinical point of view aphakia means absence of lens from pupil, complete or partial. Aphakia is an example of extreme form of hypermetropia where there is no accommodation. Hence aphakia is a state of absolute hypermetropia which equals total hypermetropia. Any amount of cycloplegic does not increase hypermetropia in aphakia. Causes of aphakia in children are : 1. Congenital. This is one of the rare congenital anomalies, where either the lens vescicle has not formed (primary aphakia) or the partially formed lens has been absorbed in intrauterine life (secondary aphakia)17. 2. Trauma : (i) Surgical. This follows surgery on congenital, developmental or traumatic cataract in children. (ii) Accidental trauma. Spontaneous absorption of cortical matter following injury to the lens. (iii) Traumatic extrusion of the lens from the globe, generally the lens becomes subconjunctival and forms a phacocele.

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3. Displacement. This can either be congenital or may be traumatic. Sometimes the lens may be displaced in congenital glaucoma or aniridia. Posterior dislocation is more common than anterior dislocation. An aphakia is said to be complete when whole of lens i.e. capsule, cortex nucleus are absent from the pupillary area. It is said to be partial when a part of the pupil is devoid of the lens. Optics of aphakia. Though aphakia is a high degree of hypermetropia, its optic differs greatly from phakic hypermetropia. The first and foremost component of aphakia is absence of lens from optical system and equally important part is total absence of accommodation. 1. The total refractive power of the eye is reduced to +43+44D from +60D of normal emmetropic eye with only one refractive element i.e. cornea. 2. The eye becomes highly hypermetropic without accommodation. 3. The anterior focal point shifts to 23.2 mm from usual 17.05 mm in front of the cornea. 4. The posterior focal point shifts to 31 mm, which is 7 mm more than the normal anterio posterior length of a normal eyeball. 5. The two principal points are almost on the anterior surface of the cornea. 6. The two nodal points almost merge with each other and are 7.75 mm behind the anterior surface of the cornea. Thus the nodal point moves forward in absence of lens. The image is formed away from the nodal point, hence it is smaller than in emmetropic eye. The image gets magnified when aphakia is corrected by plus lenses, may be in spectacle or as contact lens. Magnification by spectacle is about 30% to 40% more than that of emmetropic eye. The corresponding magnification range between 5% to 12% in contact lens. If an IOL is put in the posterior chamber, the magnification is abolished altogether. Symptoms of aphakia. Symptoms of aphakia consists of diminished distant vision and near vision. Uncorrected distant vision in aphakia is generally 1/60-2/60. The child can not read the usual prints in books due to total absence of accommodation. Signs of aphakia : 1. Scar : (a) At the limbus if a small incision lens extraction has been done. (b) A small scar inside the limbus was a common site when needling was a popular mode of lens extraction in children. (c) A small opacity in the cornea away from the limbus is seen in case of penetrating injury by pointed objects like needle, thorn, pin etc. (d) An extensive corneoscleral scar is seen in corneoscleral penetrating injury. (e) Phaco emulsification done in older children may have barely visible scar away from the limbus. (f ) Scar is absent in blunt injury, buphthalmos, Marfans syndrome. 2. Anterior chamber is deeper than normal.

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3. The iris is tremulous. 4. An iridectomy may be present. 5. Pupil (a) colour : (i) Jet black in case of posterior dislocation of lens due to any cause. (ii) After cataract is common following needling, needling and aspiration, and spontaneous absorption of cortical matter following penetrating injury. In children posterior capsular opacification is almost universal even following micro-surgical lens extraction. (b) Position. A shifted pupil denotes incarceration of iris in the section. It is more common in accidental aphakia than in surgical aphakia. 5. Refraction. Refraction in adults shows compound hypermetropic astigmatism against the rule following conventional intracapsular or extra capsular cataract extraction. In children because the scar is very small, the refraction is generally confined to simple hypermetropia. 6. Loss of accommodation. There is total loss of accommodation. 7. Fundus examination shows small hypermetropic disc. 8. In all cases of aphakia following trauma or cases where mechanical capsulotomy has been done, the fundus should be examined by indirect ophthalmoscope for evidence of holes or traction bands. 9. Muscle imbalance and amblyopia are common in uniocular aphakia. Management. Management of aphakia in children, in principle does not differ from adult aphakia that is to correct hypermetropia by convex lenses. Cylindrical errors should also be taken into consideration. Additional plus lenses are required for near vision. The various options available are : 1. Spectacle 2. Contact lens 3. Intraocular lens 4. Refractive surgery. Spectacles are suitable in bilateral aphakia in children. They are not tolerated in uniocular aphakia due to anisometropia and aniseikonia that leads to intractable diplopia and child prefers single vision without aphakic correction. This in turn leads to development of squint and amblyopia. An aphakic child may tolerate spectacle correction if the other eye has no vision, poor vision, has deep suppression or is divergent. Even in case of binocular aphakic correction with spectacles, the child has : 1. Spherical aberration leading to pin cushion distortion. The straight lines look curved, the whole scenario consist of paraboles which keep on changing with movement of the eyes. 2. Prismatic aberration causing a roving ring scotoma between 5065 all round.

562 3. Overall restriction of peripheral field.

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4. Absent or poor near vision. A child is less likely to tolerate bifocal so he requires thicker near glasses. 5. Cosmetic blemish. A child especially in teens does not like using thick heavy glasses that give an enlarged view of the eye. 6. Sliding of the spectacles on the nose. This increases the effective power of the plus lens which may help the child in near work but blurs distant vision and increases cosmetic blemish. Few of the advantages of spectacle correction are that they are cheap, can be replaced with ease, do not require specialised handling. Contact lens. Contact lens correction is better option than spectacle correction especially in uniocular aphakia because : 1. Magnification by contact lens is only 5% to 10% in contrast to 30% by spectacle correction. 2. The field of vision is better, the roving scotoma is abolished. 3. Prismatic aberration is almost abolished. 4. The cosmetic blemish is eliminated. It has been seen that small children can also tolerate contact lenses well. Intraocular lenses are the best possible answer to aphakic correction as they are devoid of all the aberrations met with spectacle and contact lens correction. Most advantageous property being complete elimination of magnification, which by itself abolishes aniseikonia when placed in posterior chamber. Intraocular lenses are generally used as primary procedure for cataract in children but can be used as secondary procedure where the eye was left aphakic following lens extraction. Refractive surgeries are not suitable aphakic children. Pseudophakia. Pseudophakia is the best method of correcting aphakia. In this the lens is removed partially or totally and supplemented by an artificial lens. The intraocular implants are made up of polymethacrylate (PAMA), silicon, acrylic or hydrogel. The first is a rigid, hard lens while the others are malleable. The IOL can be implanted as a primary procedure i.e. removal of lens with simultaneous implantation of lens or as a secondary procedure where the lens is removed in first session and IOL implantation later. According to position of lens, the implant can be in the : 1. Anterior chamber 2. Iris fixed 3. Posterior chamberIn the bag In the sulcus 4. Scleral fixed.

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Anterior chamber lenses have been given up as they are prone for immediate and delayed complication and are implanted following intracapsular lens extraction mostly, which is not possible in children. Iris fixated lens can either be fixed in the pupil or on the surface of iris. Posterior chamber IOL in the bag is the most popular and suitable lenses in children. IOL in sulcus has also been abandoned in favour of IOL in bag. Scleral fixed lens require high degree of skill and are used in subluxated and dislocated lenses. The IOLs mostly are unifocal that require additional near correction unless the child is left slightly myopic. In contrast to this, there are multifocals that have power to focus distant as well as near objects. Role of multifocals in pediatric aphakia has not been evaluated well. If the power of IOL has been calculated accurately and lens placed well, the IOL should make the eye emmetropic but this is not always possible. Some residual error is almost universal. The post operative corneal astigmatism is not corrected by best calculated IOL. The IOL reduces aniseikonia to well tolerated limit. Thus reducing incidence of diplopia. However most of the IOLs leave some loss of stereopsis. Some of the children may require spectacle correction even after IOL mostly to neutralise astigmatism.

Diminished Near Vision in Children


Diminished near vision is universal in emmetropic adult after forty years of age. Children due to their reserve of accommodation do not have difficulty in finest near work. The condition where the child has difficulty in near vision are : 1. TherapeuticAccidental or therapeutic use of strong cycloplegic. 2. Neurological (a) Internal ophthalmoplegia (b) Third nerve palsy 3. Refractive (a) Uncorrected or under corrected hypermetropia (b) Over corrected myopia (c) High astigmatism (d) Aphakia (e) Pseudophakia 4. Disparity between accommodation and conversions. 5. Nystagmus

Astigmatism
This is a state of refraction where a point focus of light is not formed on the retina with or without accommodation. Instead of one focal point there are two focal lines. The distance

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between the two focal lines is known as focal interval that represents the astigmatic power of the eye. The aim of treatment is to abolish this distance by optical methods i.e. spectacle, contact lens or surgery so that the two focal lines merge with each other, and a pinpoint focus is formed on the retina. In between the two focal lines is the circle of least diffusion which represents the spherical power. A perfect stigmatic eye is an exception. All eyes have some minor degree of astigmatism without any symptoms. The cornea of a new born is almost spherical18, with age slight astigmatism is natural due to pressure of the lids on the cornea. The vertical meridian is steeper hence slightly more myopic than flatter horizontal meridian, which remains emmetropic. This is known as direct astigmatism or astigmatism with the rule. In contrast to this, where horizontal curvature steeper, the condition is called indirect astigmatism or astigmatism against the rule. Types of astigmatism : 1. According to site of involvement Corneal Lenticular Retinal 2. According to axis of astigmatism 3. According to position of the image. 4. Clinical classification Regular astigmatism Irregular astigmatism According to site : Corneal astigmatism is the commonest form of astigmatism both regular and irregular. Lenticular astigmatism : 1. Decentered lens 2. Different curvature in different meridian i.e. lenticonus, subluxation, coloboma of lens. 3. Difference in refractive index i.e. early cataract. Retinal astigmatism. This is seen generally with retrobulbar mass or oblique placement of macula.19 According to axis of image : Regular astigmatism 1. The two meridians are at right angles to each other, one of them is horizontal and the other is vertical. This is the commonest type of astigmatism. 2. The two axises are at right angles to each other but not horizontal and vertical, the condition is called oblique astigmatism. The axises are off the horizontal or vertical by 20 or more. The axis in one eye is mirror image of the other eye i.e 80 and 110 or 20 and 160. 3. The difference between the two meridians is less than 90, the condition is called bioblique astigmatism.

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Irregular astigmatism is that astigmatism where astigmatism in the principal meridian vary. This is caused due to corneal irregularity like corneal scar, keratectasia, keratoconus, pterygium, limbal dermoid, post keratoplasty. According to position of image in relation to retina. The astigmatism can be simple, compound or mixed. Simple astigmatism is that astigmatism in which one meridian is emmetropic, the other is either myopic or hypermetropic. The former is called simple myopic astigmatism, while the latter is called simple hypermetropic astigmatism. Each is corrected by suitable single cylinder of appropriate sign, axis and power. Compound astigmatism is that astigmatism where both the meridians are either myopic or hypermetropic and known as compound myopic or compound hypermetropic astigmatism respectively. They are corrected by sphere and cylinder of same sign i.e. compound myopic astigmatism is corrected by myopic sphere with myopic cylinder. Mixed astigmatism is that astigmatism where two meridians have different signs i.e. one is myopic, the other is hypermetropic or vice-versa. In mixed astigmatism the power of the cylinder is always more than the sphere. They are most difficult to correct. They are very common following IOL implant. Symptoms of astigmatism : 1. Small degree of astigmatism may be symptomless. 2. Diminished distant visionAs the eye fails to form a sharp image on the retina, the visual loss is considerable. However sometimes high astigmatism may not cause corresponding degree of loss of vision i.e. a child with 6/12 vision may require just 0.75 to 1.00 Dsp to bring the vision to 6/6. But an astigmatic may require as much as 2 to 2.5 Dcyl to attain same vision. 3. Difficulty in focusing near objectsAs accommodation fails to correct astigmatism, the near vision is never comfortable for an astigmatic. 4. Head turning and tiltingThe child may turn or tilt the head to compensate for the axis of astigmatism. 5. Narrowing of interpalpebral fissureThe child tries to produce a pinhole effect to improve the vision. This attempt is less effective than seen in spherical ametropia. 6. Muscle imbalance is more common in astigmatism than in other errors of refraction. 7. Asthenopia and asthenopia related symptoms are more common in astigmatism of low grade. They are : Watering, redness of eye, recurrent blepharitis, stye, chalazion, frontal headache. 8. Muscle imbalance. Signs of astigmatism : 1. Externally the eyes may not look abnormal. In case of compound high myopic astigmatism the eye may look large, may similarly look small in case of compound hypermetropic astigmatism. Recurrent stye, chalazion, blepharitis, presence of haemangioma or neurofibroma on the lid should arouse suspicion of astigmatism.

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2. Placido disc may show irregularity in the circles, in irregular astigmatism and crowding and elliptical shape in high regular astigmatism. 3. Computerised keratoscopy shows irregularity in the corneal surface. 4. Refraction always shows difference in refraction in two principal meridians. 5. Keratometry shows difference in power in different meridian and their axises. 6. On fundus examination the optic disc looks oval. Vertically oval disc is more common than horizontal. 7. An astigmatic child when examined on E chart may not be able to tell the direction of the arms of E. He may be able to tell the direction in vertical direction but fumble in horizontal direction and vice versa. 8. On astigmatic fan the child may see some lines clearly and not those at right angles to it. 9. Vision with pinhole does not improve satisfactorily in astigmatism. A child with -2D myope will improve to 6/6 with a pinhole but a child with -1Dcyl may improve only to 6/12. Management 1. No treatmentIf there is no visual loss or there are no symptoms of eye strain, asthenopia or muscle imbalance, no treatment is required. The child is examined once every year and his retinoscopy is done under cycloplegia. 2. Prescription of glasses : 1. Simple astigmatism is corrected by single cylinder of appropriate power and sign at proper axis. 2. Compound astigmatism is corrected by prescribing sphere and cylinder of same sign. 3. Mixed astigmatism is corrected by sphere and cylinder of opposite sign. As a rule every attempt should be made to correct cylindrical defect fully.18 The spherical correction should be done on usual lines of spherical ametropia. While correcting astigmatism utmost attention should be given to correct axis of the cylinder. A wrongly placed cylinder is more troublesome than without one. In mixed astigmatism the combination that is most comfortable should be prescribed i.e. minus sphere with plus cylinder at right angles or plus cylinder with minus cylinder at horizontal axis. The power and axis of the cylinder can be checked by : 1. Auto refractionThese are good for non-verbal children who can not be tested subjectively. However refraction under cycloplegia should get proper weightage. 2. KeratometryThis is more important in contact lens fitting than spectacle. This measure the curvature of a anterior corneal surface only 3 mm wide. It measures the astigmatism of the front surface of the cornea at two points about 1.25 mm on either centre side of the cornea.

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3. Foggin 4. Astigmatic fan The last two are not suitable for small children. 5. Jacksons cross cylinder. This is the best and most accurate method to verify power of cylinder and axis of cylinder. The spectacles given for astigmatism should have proper fitting otherwise the axis may change giving poor vision and discomfort. Contact lens. Contact lenses are prescribed only when the child can manage the contact lens himself. Though small degree of regular astigmatism are well corrected by contact lens, it is better to go for spectacle correction because it is cheap, easily replaceable, and require no maintenance. Contact lenses are more suitable for high astigmatism, bi-oblique astigmatism and irregular astigmatism. Spherical contact lenses fail to correct astigmatism more than 2 to 3 Dcyl. For high cylindrical error toric contact lenses are best suited. A toric lens20 should have full corneal coverage and good centering. The lens should have good movement with each blink, the up and down gaze. The lens should give vision comparable to spectacle vision if not better. The toric contact lenses are difficult to fit.21, 22

Anisometropia
When the refractive power of two eyes is different, it is called anisometropia in contrast to isometropia where refraction in two eyes is equal. Small degree of anisometropia is more or less the rule. Perfect isometropia is exception like perfect emmetropia and stigmatic eye. Anisometropia is more common in astigmatism, may be congenital. A difference in refraction in two eyes results in difference in retinal size in two eyes. One diopter of difference results in 2% difference in size of image. In myopia the size is reduced while in hypermetropia the size is magnified. A difference in 2.5D between two eyes will result in 5% difference in size of image. Difference in size of two retinal images is called aniseikonia. A difference in size up to 5% between the two eyes is the limit of tolerance beyond which diplopia results. A difference of 2.5D or more is called clinical anisometropia. Anisometropia upto 2.5D is well tolerated, of course some may not tolerate even a smaller difference. However, some individuals may tolerate difference upto 4D. Etiology of anisometropia. Anisometropia can be congenital or acquired. Congenital anisometropia is generally hereditary and due to differential growth of two eyes. Acquired anisometropia. Commonest cause of anisometropia is uniocular aphakia. Second cause that is being seen more often is wrong calculation of IOL power. Other such cause is over or under correction following refractive surgery and uniocular keratoplasty. Types of anisometropia : 1. Simple Spherical Astigmatic 2. Compound Spherical Astigmatic

568 3. Mixed Spherical Astigmatic

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Simple anisometropia. In this condition one eye is emmetropic, the other eye is either myopic or hypermetropic. Compound anisometropia. Both eyes have same type of error of refraction i.e. myopia or hypermetropia but power in one eye is more than the other. Mixed anisometropia or Anti metropia. In this, one eye is myopic, the other eye is hypermetropic. In simple astigmatic anisometropia one eye is emmetropic, the other has simple astigmatism. In compound astigmatic anisometropia both the eyes are astigmatic but at different degree. In mixed astigmatic anisometropia one eye has myopic astigmatism, the other eye has hypermetropic astigmatism. Symptoms of anisometropia. Symptoms of anisometropia depends upon difference between the refractive status in two eyes. Visual : 1. Binocular single vision. This is present when the difference between the two eyes is not more than 2.5D to 3.0D. 2. Uniocular vision. This happens when the difference in power in two eyes exceeds 4D. Generally one of the eye is emmetropic or has less power than the fellow eye. The eye with higher power is generally suppressed, develops and becomes amblyopic and starts squinting. 3. Alternate vision. This happens when one eye is either emmetropic or has low error of refraction and the other eye has myopia more than 2D. The patient uses the myopic eye for near vision and the other eye for distant vision. These patients may not know about their visual short coming and are comfortable. They do not seek optical aid. Such children are diagnosed on routine school survey or if they wish to join some project that requires binocular vision. Muscle imbalance. The child may be brought with squint and discovered to have anisometropia and many a times amblyopia. Anisometropia may cause both eso or exodeviation but concomitant convergent squint is more common. Diagnosis. Diagnosis of anisometropia is simple by doing retinoscopy which should be done under complete cycloplegia. Otherwise, it can be done by Friend test or Worth four dot test. The Friend test. This is a simple test. It consists of a panel generally incorporated at the bottom of usual Snellens drum. In the chart the letter FIN are written in green and RED are written in red. The size of all the letters are same. The background is illuminated. The size of the letters correspond to size of 6/18 or 6/12. The child sits at a distance of six metres with red green glasses in the trial set or may wear a diplopi goggles. The red glass is kept in front of right eye as convention and not for any clinical significance. The child is asked to read the line

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keeping both the eyes open. The right eye can see only red letters through the red glass and the left eye only green letters through the green glass if the patient has binocular single vision the child will be able to read the full line as FRIEND. If the child has alternate vision, the child will read either FIN or RED at a given time. The child with uniocular vision will read either FIN or RED depending on which eye has better vision. Worth four dot test. This panel is also incorporated at the bottom of usual Snellens vision drum. It has four dots of the size of 6/18 arranged in a diamond shape. The spots have following colours - one white, one red and two greens. The child sits at a distance of six metres with red glass in front of the right eye and green glass in front of the left eye and asked to count the number of spots and tell their number with both eyes open. Before starting the test, it is seen if the child has manifest squint or not. Interpretation : 1. In arthophoria, if the child sees all the four dots and tells their number correctly that matches with the panel, the child has normal binocular single vision. 2. If the child has manifest squint and the child sees all the four dots as in the panel, the child has abnormal retinal correspondence. 3. If the child sees only two red lights, the left eye is suppressed. 4. If the child sees five lights i.e. two red and three greens, the child has diplopia which otherwise means fairly good vision in each eye. Management : 1. Spectacles. Spectacles are tolerated well if the difference of power in two eyes is not more than 2.5D. Some patients may tolerate difference up to 4D. Usually the eye with more power is under corrected. In children care should be taken to see that the child sees through the centre of the glasses and not above the glasses and uses glasses constantly. 2. Contact lenses. The contact lenses are better option than spectacles because the change in size of the image is less. The child has no chance to look above the lens and the lens moves with the movement of the eye. 3. Intraocular lens implant. This is most suitable form of treatment in uniocular aphakia. All uniocular lens extraction in children should have IOL implanted as primary procedure if not contra indicated. In cases of old uniocular aphakia, secondary IOL is a safe procedure. 4. Clear lens extraction may be tried in case of uniocular high myopia. High myopes may also have phakic IOL in presence of clear lens. 5. Refractive surgeries are not indicated in children.

Aniseikonia
An aniseikonia is a rare disturbance of binocular vision. Prevalence of the condition is more than the numbers diagnosed. It is defined as a condition where images presented to cortex from two retinae are of unequal shape or size or both.

570 Causes of aniseikenia are

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1. Optical. This is the comonest and most easily diagnosed type of aniseikonia where due to high anisometropia, images of two different sizes are formed on the retina and presented as such to the cortex. This can be congenital or acquired. The acquired cause consist of difference in power, thickness of the lens and cylinders in the lens of the spectacle prescribed. 2. Retinal 1. Shift of the retinal element towards the nodal point. 2. Separation of retinal element due to edema of the retina. Clinical types of aniseikonia 1. Aniseikonia is said to be symmetrical when there is difference in size of the two images. The difference can be overall i.e. in all meridians or meridinal when the difference is greater in one meridian and compound when both types i.e. overall and meridinal are present. 2. Asymmetrical when there is difference in shape of the two images. It could be regular, pin cushion, barrel type or irregular. In regular type, there is progressive increase or decrease in size across the field. Symptoms of aniseikonia vary from patient to patient. Some of the patients may not be able to express the correct symptom except diplopia. Other symptoms are asthenopia. In some patients there may be faulty stereopsis. There may be disturbances of spatial orientation in the form of objects on right side of the field looking farther away than objects of same size and distance on the left side. A flat surface slants down on the right side and up on the left. The square appears rectangular and circles seem to be elliptical. Diagnosis. Diagnosis of aniseikonia is difficult, however, a rough estimate can be made on the basis of anisometropia present, 1% change in size per dioptre is most accepted estimate. Treatment. Optical causes of aniseikonia are best treated by contact lens. Best treatment for unilateral aphakia is IOL implant either as primary procedure or secondary procedure. Treatment of retinal aniseikonia is directed towards treatment of retinal condition mostly medically.

Asthenopia
Asthenopia is one of common symptoms, which the patient may not be able to express precisely. It is defined as uncomfortable vision that may cause unexplained pain and irritation in the eyes, fatigue, after near-work, heaviness of lids, sleepy feeling. Causes of asthenopia include 1. Refractive error. 2. Muscle imbalance 3. Accommodation and convergence insufficiency.

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Errors of refraction. Uncorrected errors of refraction are commonest cause of asthenopia. High errors of refraction rarely cause asthenopia because the child is resigned to blurred vision and does not try to get a clear vision. Smaller errors of refraction cause more asthenopia. Astigmatic eyes are more likely to develop asthenopia than spherical errors. Horizontal axis and oblique axises cause more trouble than other meridians. Under corrected or over corrected errors are more likely to cause asthenopia. In myopia asthenopia develops more for near work because myope requires more convergence for clear view without or with least accommodation. The child has to depend on increased fusional convergence. Some myopic patients who had no asthenopia before correction may develop asthenopia following correction. A hypermetropic has to use accommodation more than emmetrope for near as well as distance, putting constant strain on ciliary muscle. Hypermetropic astigmatism causes more problem because hypermetropic tries to get clear vision by accommodating, as accommodation is equal in all meridians the astigmatism is worsened in opposite meridian. In anisometropia, the blurring of vision is unequal in two eyes and accommodation which acts equally in both eyes may worsen in one of the eyes. Muscle imbalance. Asthenopia is more common in phorias than in tropias. The child constantly tries to keep the eye in orthophoric state and a discrepancy between amplitude of motor fusion and deviation results in asthenopia. Vertical deviations cause more asthenopia. Accommodation convergence insufficiency. It can either be deficiency of accommodation or convergence. Some patients with good vision with low degree of errors of refraction may be unable to accommodate without convergence. Convergence insufficiency is seen in uncorrected myopes or hypermetropes corrected for first time. Otherwise they are orthophoric for distance. Symptoms. The symptoms of asthenopia may be ocular or non ocular. Ocular symptoms can be visual or non visual. The visual symptoms are intermittent. They are seen following prolonged non accustomed visual effort or general dibility. There is slight fall in vision. Non visual symptoms are tired eyes, aching eyes and bleary eyes. The ocular pain is described variously as deep seated, boring or dull. Chronic conjunctivitis, blepharitis, styes, chalazia are common. Commonest non ocular symptom is headache. It is very common for general physicians to refer all cases of headache to ophthalmologist as asthenopia. On examination most of them may turn out to be non asthenopic. Headache in asthenopia does not follow any fixed pattern. It may be referred as browache, frontal, temporal or occipital in location. Most probable explanation for headache in asthenopia is pain referred from the ciliary body. Management of asthenopia. Management of asthenopia is frustrating. It is directed to cause of asthenopia. The examination that should precede management are : 1. Recording of distant and near vision.

572 2. Checking near point of accommodation. 3. Checking near point of convergence. 4. Assessment of muscle imbalance. 5. Refraction under cycloplegia.

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Refraction should be followed by prescription of glasses that are most comfortable even with slightly reduced vision. Axis of astigmatism should be checked meticulously. Orthoptic treatment or orthoptic exercise in the form of - adduction exercise are given for convergence insufficiency. PrismsBase in prisms also give good result in convergence insufficiency.

REFERENCES
1. Duke Elder S. ; Ophthalmic optics and refraction in System of ophthalmology. Vol. V, C.V. Mosby, St. Louis, 1970. 2. Duke Elder S ; The foundation in ophthalmology. System of ophthalmology. VII, Henry Kimpton, London, 1962. 3. Primrose J. ; Anomalies of refraction and accommodation in Modern Ophthalmology. Vol. III, Edited by Sorsby A. Butterworth, London, 1964. 4. Agrawal L.P. ; Principles of optics and refraction. Second edition. CBS Publishers and distributors, New Delhi, 1979. 5. Singhal N.C. ; Principles and practice of refraction and optics. JayPee Brothers, New Delhi, 1996. 6. Khurana A.K. ; Theories and practice of optics and refraction. B.I. Churchill, Livingstone, New Delhi, 2001. 7. Sharma P. ; Myopia in Essentials of ophthalmology. First edition p-2124, Modern Publishers, New Delhi, 2000. 8. Debhorah Pavan Langston ; Refractive errors and clinical optics in Manual of ocular diagnosis and therapy. Third edition, p-361-388, Lippincot William and Wilkins, Philadelphia, 2002. 9. Khurana A.K. ; Congenital myopia in Opthalmology. Second edition, p-56, New Age International, New Delhi, 2000. 10. Agrawal L.P. ; Myopia in Essentials of Ophthalmology. First edition, p-469473, CBS Publication, New Delhi, 2000. 11. Basak S.K. ; Myopia in Essentials of ophthalmology. Second edition, p-5660, Current Book International, Calcutta, 1999. 12. D.S.T. Clair Roberts ; Fundus changes in myopia in Modern ophthalmology. Vol. 4, p-761762, Edited by Sorsby A. Butterworth, London, 1964. 13. Miller S.J.H. ; Myopic choroido retinal degeneration in Parsons diseases of the eye. Seventeenth edition, p-165166, Churchill Livingstone, London, 1984.

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14. Kanski J.J. ; Myopic maculopathy in Clinical ophthalmology. Second edition, p-360, Butterworth London, 1989. 15. George E. Garcia and Deborah Pavan Langston ; Orthokeratology in Manual of ocular diagnosis and therapy. Third edition, edited by Deborah Pavan Langston, p-387, Lippincot, William and Wilkins, Philadelphia, 2002. 16. Boyd S. Lasek ; Laser subepithelial keratomileusis. Highlights of ophthalmology. Vol. 30, p-1517, 2002. 17. Duke Elder S. ; Congenital aphakia in System of ophthalmology. Vol. III, Part I, p-688, Henry Kimpton, London, 1964. 18. Duke Elder S. ; Astigmatism in Duke Elders Practice of refraction. Ninth edition, p-5255, BI Chirchill Livingston, New Delhi, 1989. 19. Khurana A.K. ; Astigmatism in Theory and practice of optics and refraction. First edition, p-70, Bi Churchill Livingston, New Delhi, 2001. 20. Phyllis L. Rakow ; Soft lenses and astigmatic eye in Contact lenses. First edition, p-4961, Jay Pee Brothers, New Delhi, 1989. 21. C.Y. Khoo ; 101 Questions and answers about contact lenses. P.G. Publication, New Delhi, 1985. 22. Hunter D.G. and West. C.E. ; Contact lenses in Last minute optics. First Indian edition, p-72, Jay Pee Brothers, New Delhi, 1997. 23. Schaffer D.B. ; Prematurity as related to ocular abnormalities in Text Book of Ophthalmology. 9th edition, edited by Scheie H.G. and Albert D.M., p-323, W.B. Sanuders Company, Philadelphia, 1977.

CHAPTER

16

Symptomatic Disturbance of Vision in Children1, 2, 3


Commonest visual symptom in all ages is diminished vision either for distance, near or both. Causes of which can be traced mostly to the eye itself and considered to be peripheral. Besides these there are a host of complaints which may or may not have diminished vision, have systemic origin, mostly in neuro-visual paths. Many of them are suffixed with term blindness i.e. night blindness, colour blindness. These terms do not strictly meet the criteria laid down in definition of blindness. They are used clinically more as habit than academic accuracy. They are : 1. Night blindness (nyctalopia) 3. Chromatopsia 5. Colour blindness 7. Dyslexia 9. Visual hallucination 11. Amaurosis 2. Day blindness 4. Metamorphopsia 6. Visual aphasia4 8. Agnosia 10. Scotomas 12. Amblyopia

Out of all the conditions in the long list of disturbances mentioned above night blindness is the most important because acquired night blindness due to vitamin A deficiency is a major cause of preventable and curable blindness in children in developing and under developed countries where more than half of blind children are found. Night blindness is a state of diminished vision in reduced illumination (scotopic vision) with prolonged dark adaptation and abnormal ERG. In extreme degree the child finds it difficult to locate his food placed in front of him after dusk or even in a darker room during day, with no complain in bright light. Night blindness can be : 1. Congenital 3. Acquired Congenital night blindness is far rarer than acquired night blindness. It can have normal fundus as in congenital stationary night blindness or with fundus changes as in Oguchis disease and fundus albipuntatus. 574 2. Developmental

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Congenital stationary night blindness. They have early onset of diminished vision that may be moderate or crippling. The children have normal field when tested in normal illumination. Their colour sense is impaired. The fundi are normal except myopic changes due to associated moderate myopia. Myopia is common accompaniment in stationary congenital night blindness. The condition does not progress much. It is bilateral. The condition is genetic, there are three types of genetic presentation i.e. Recessive either X linked or autosomal and autosomal dominant. Children with autosomal variety have normal vision while other two have diminished distant vision due to associated myopia. Children with diminished vision may have strabismus and or nystagmus. The children have abnormal ERG and EOG. There is no known treatment. Oguchis disease. This is a very rare congenital anomaly of rods, mostly seen in Japanese. It is an autosomal recessive disorder with high incidence of consanguinity. The main symptom is night blindness (nyctalopia) followed by diminished distant vision which is moderate, mostly due to myopia. The condition is non progressive. The diagnosis lies in the fundus picture. The fundus has a yellowish gray phosphorescent sheen2 that spreads from disc to periphery. Occasionally only peripheral sheen is also met with. The choroidal vessels are not visible. It is difficult to differentiate between the retinal arteries and veins as they have almost same dark colour. The confirmatory feature is Mizuo sign or Mizuo phenomenon that consists of reversal of fundus finding from yellowish gray sheen to normal fundus picture if the eyes are kept closed for 2 to 3 hours. Then the retina returns to its yellowish gray colour after exposure to normal light in half an hour to one hour. Colour vision is not affected. ERG shows only scotopic a wave, b wave is extinct, EOG is normal. There is no known treatment. Fundus albipunctatus5 . This is a condition of congenital night blindness of autosomal recessive type. The condition is stationary in nature. There is a progressive type which is a tapeto retinal dystrophy and is called retinitis punctata albescens. Both the conditions have uniform sized white or yellow white dots scattered all over the fundus. The dot may increase in number and area but do not involve the macula. The retinal vessels are of normal size. The disc does not undergo atrophic changes that is common in true retinal dystrophies. The peripheral fields are within normal limits. The ERG results are variable. EOG is normal. There is no known treatment. Developmental causes of night blindness. This comprises a number of conditions of diverse etiology and pathology with common features of night blindness, constricted field and fundus changes in the form of pigmentation. The condition may be localised to eyes only or may be associated with number of systemic condition. All of them are genetically determined conditions, are most probably present at birth but clinically manifest after few years hence they are put in category of developmental disorders.

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They are all abiotrophies. They can be put into two broad groups according to parts of the eye involved : 1. A large number of conditions that are retinal in origin. 2. A smaller number of uveal dystrophies. Though some of them are called degeneration, they are in fact dystrophies. The retinal conditions are also known as tapetoretinal degeneration. Some of the terms like retinitis pigmentosa may suggest an inflammatory etiology. They are non infective or inflammatory. The retinopathies may be confined to the eye only like in primary retinitis pigmentosa or may have systemic involvement like Laurence Moon Biedl Bardet Syndrome. The number of systemic conditions with night blindness is a long one. See page. 433439 also. Acquired night blindness Acquired night blindness in children is a serious ophthalmic problem in developing countries, which if not managed may lead to permanent loss of vision. The commonest cause is vitamin A deficiency either due to short supply in malnutrition, high depletion in measles or poor absorption in diarrhoea. It may be seen in many children in the same family. It is fully preventable as well as curable in early stages. It is generally associated with xerophthalmia, which may precede onset of night blindness, may accompany night blindness. In lesser number of children, night blindness may be the first symptom that develops over few weeks. This is called chicken blindness because the child fumbles in diminished light. Other causes of acquired night blindness in children are less common. They are high myopia with extensive chorioretinal degeneration, peripheral cataract and juvenile glaucoma. Day blindness. This is less common than night blindness and not serious enough to cause blindness. Nonetheless there is a troublesome symptom that comprises of constriction of pupil resulting in lowering of vision. The lesion is either in front of the pupil i.e. dense central corneal opacity or behind the pupil as in central nuclear or posterior polar cataract. Large macular lesion also causes diminished vision in bright light. Day blindness should not be confused with glare and photophobia. Glare is intolerance to bright light without loss of vision as seen in - Coloboma of iris, congenital or surgical, aniridia, albinism, myopia and following mydriasis either therapeutic or neurologic. In photophobia the child involuntarily shuts the lid in presence of light or may turn the head away from the light, may bury the face under the pillow. This is due to irrigation of trigeminal nerve. It has not been conclusively proved if photophobia can be present in absence of light perception. Common causes of photophobia in children are - Interstitial keratitis, corneal abrasion, corneal ulcer, phlyctenular kerato conjunctivitis, fascicular ulcer, buphthalmos, hydrops of cornea, acute iridocyclitis, meningitis and encephalitis. It is also seen in congenital achromatopsia. Photopsia. This represents seeing flashes of light. This is due to irritation of visual elements of the retina. This is a serious symptom that is almost always a warning to development of retinal detachment especially in myopic children and eyes that have sustained

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injury. Cases of photopsia should not be dismissed casually. All children complaining of flashes of light should undergo complete ocular examination, which should include indirect ophthalmoscopy with scleral indentation. Rarely cerebral irritation may also cause flashes of light. Chromatopsia. In this condition, the persons gets a coloured hue on a light coloured object i.e. white washed wall, white cloth etc. This is mostly due to faulty absorption of a particular colour due to changes in the lens or may be drug induced. Erythropsia (Seeing red). This is common following lens extraction in first few days. Occasionally a child with fresh thin blood film in front of the pupil or a fresh vitreous haemorrhage can complain of red coloured vision. It also occurs in snow blindness. Cyanopsia (seeing blue). This is also a common complain after lens extraction. Xanthopsia (seeing yellow). Commonest cause is jaundice. There is a big list of drugs that cause xanthopia. Changes in the form of objects (Metamorphopsia) : Micropsia. When the things took smaller than their actual size. This is due to crowding of cones. In contrast to this, minification is smaller image formation on retina by minus glasses as is seen in myopic correction. Macropsia. When the things look larger than its actual size due to separation of cones. Magnification like minification is an optical phenomenon where the image of the object real or virtual look larger than its actual size as in hypermetropic correction, aphakic correction, use of magnifiers. Metamorphopsia is distorted shape, this is best appreciated on Amsler grid. Micropsia, macropsia and metamorpopsia are signs of macular lesion and retinal detachment.

Colour Blindness
Human eye have a highly developed colour sense. Most of the animals and birds have poor colour sense. A species nearer the human beings has better colour sense than those away from human beings. None of the animals have colour sense as good as human. Human eyes can perceive about 200 colours between 380-760 nm. The visible spectrum ranges between 397 nm to 723 nm roughly between 400-725 nm.5 The visible spectrum is called white light. Ultra violet has shortest wave length while infrared has longest wave length. Colour vision is mediated through cones and is transmitted via optic pathway like white light. Hence defect in retina specially in macula and disorders of anterior optic pathway lead to colour defect. Red, green and blue are the three primary colours. Others are made up of various combinations. There are three types of cones responsible for red, green and blue colour sense. The cones contain three different types of pigments one for red, the other for green and last for blue.

578 Colour defect can occur due to :

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1. Absence of all cones responsible for colour vision i.e. achromatopsia. 2. All the three cone pigments are present but one of them is abnormal and the condition is called anomalous trichromacy. They are divided into (i) ProtanomalousWhen there is abnormal red sensitive cone. (ii) Deuteranomalous have abnormal green sensitive cone. (iii) Tritanomalous have abnormal blue pigment. Former two are common, the last one is rare. Distribution of pigment sensitive cones is not uniform, the central part is devoid of blue sensitive cones. Trichromatic vision extends up to 30 round the point of fixation. Total absence of colour sensitive cone is suffixed with anopia that is protanopia deuteranopia and tritanopia respectively for red, green and blue blindness.8 3. Lesions of optic pathway also leads to acquired colour defect. 4. Changes in colour absorption by lens causes minor colour defect. Sensitivity to colour depends on many factors either in isolation or in combination. They areBrightness, hue this describes the name of the colour that the person sees, it refers to dominant wave length saturation. This is also called purity of colour and represent amount of white light present, lesser the white element purer is the colour. Classification of colour blindness : There are various methods to classify colour blindness : According to age of onset : 1. Congenital 1. Monochromatism (i) Rod monochromatism (ii) Cone monochromatism 2. Dichromatism (i) Protanopia (ii) Deuteranopia (iii) Tritanopia 3. Anomalous trichromatism (i) Protanomaly (ii) Deuteranomaly (iii) Tritanomaly Colour blindness is very common among boys, about 8% boys suffer from some type of colour blindness or others. It is very rare in girls. Women are as often affected by acquired coloured blindness as men. In congenital colour blindness women are the carriers but 2. Acquired According to type of cones involved9

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do not suffer from colour blindness. This is a classic example of x linked inheritance in red green blindness.10 As colour blindness is a dominantly male affliction without deterioration or improvement of vision in otherwise normal boys and is a bilaterally symmetrical in both eyes, all boys during their entry in primary school should be screened for presence of colour defect that may be mild to moderate. Total colour blindness where everything looks gray is very rare with normal vision. Screening is necessary not for treatment but for planning the future profession of the boys. Persons with colour defect are not suitable for many jobs. It is surprising that a colour blind does not find it difficult with traffic lights. This is most probably due to brightness of the traffic light and his experience. The boy learns to call leaves green and rose red by experience but when given to match different hues they fumble or fail. Hence ability of a boy to name a particular colour correctly does not exclude colour defect. To determine for colour vision there are various tests. None of them are perfect, some are simple and good for screening incidence of colour blindness and its probable type. They are : 1. Pseudo isochromatic11 (polychromatic) charts (i) Ishihara plates (only good for red-green deficiency) (ii) Stilling plates (iii) Hardy-Rand-Ritler plates (Useful in blue blindness) 2. Lantern test (Edridge green lantern)This is widely used test in railways and airways. It examines ability for a person to differentiate red from green signal under various conditions of brightness and size from a standard distance. 3. Fransworth hue discrimination test (i) Frans worth D-15 test. (ii) Frans worth Munsell 100 hue test. 4. Nagels anomaloscopeThis fails to detect blue blindness. 5. Holmgrens wool test is a complicated time consuming test. 6. Inter Society Colour Council aptitude testIt has too many negative results. 7. Sloan achromatopsia testThis is useful in total colour blindness.

Common Clinical Types of Colour Blindness


1. Achromatopsia. This is an autosomal recessive condition. The children have poor vision, sensory nystagmus, photophobia and total colour blindness. They have either absent or have marked abnormal cone function. To them all colours are various shades of gray which is confirmed by Sloan achromatopsia test. Photophobia in achromatopsia is not a true photophobia seen commonly in children due to irritation of trigeminal nerve. These children avoid bright light to keep the rods dark adapted12 to use them for better vision.

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All children with nystagmus and poor vision should be examined for achromatopsia. Similarly all children with photophobia without evidence of irritation of fifth nerve should also be tested for achromatopsia. There is no treatment, however extra dark glasses with side covers give some improvement in vision. Any error of refraction should be corrected. Low vision aids do not have much value. The children have to be brought up as visually challenged. 2. Other congenital colour defects. These defects are present at birth but detected later. The child and his parents may not be aware of the defect unless pointed out. They have generally normal vision with normal eyes, do not suffer from any neurological deficit, lead a normal life. They generally have anomalous pigments in the cones. There is no known treatment. Acquired colour defect : The causes can be : 1. Lenticular (not seen in children) 2. Retinal Macular Pan retinal photo coagulation 3. Optic pathway Retinal : 1. Lesions of outer layers of retina give rise to rarer form of blue-yellow defect. 2. Lesions of inner layer give rise to red green defect. The conditions that cause retinal colour defect are - pan photo coagulation, macular degeneration, high myopia and chorioretinitis. Optic pathway. Optic pathway not only carries visual impulses but also colour sense to the central nervous system. Hence it is but natural that lesions of optic pathway should produce colour defect. In lesions of optic nerve, the colour sense is not totally abolished, it seems to be washed out13. Reduced colour sense is so consistent with lesion of optic nerve that diminished vision with normal colour senses excludes possibility of optic nerve lesion. In lesions of optic nerve, the coloured object when centrally fixed has duller colour than when shifted to periphery due to central relative scotoma. Colour vision is diminished even before central vision is affected.14 Colour field may be lost in not only in central field but also in peripheral field also. Inability to recognise colours when no colour blindness is present is called colour agnosia. Inability to recall the name of colour is called amenestic colour aphasia.14 Visual aphasia3 (word blindness). In this condition, the child has normal vision and colour sense but can not recognise words, letters or colours. The child can not copy written letters but can write spontaneously. It is generally seen in involvement of optic radiation. Agraphia is inability to express meaning in written language while alexia means inability to read. Dyslexia15. Dyslexia is more serious and common disorder in children than alexia. In alexia the child can not comprehend written words. Incidence of dyslexia in developed countries is as high as 4 to 6 percent. No data is available for developing countries, most probably

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because its presence is not appreciated and the child is considered to be dull. In fact dyslexic children have normal or higher intelligence than other children. The condition is thought to be central in origin, exact location of the lesion is not known. It affects both the sexes equally. There may be positive history of learning difficulty in members of the same family. The condition is congenital but detected only when the child is admitted to school. The child has difficulty in relating letters and groups of letters with appropriate sound15. The condition is permanent. Diagnosis is not simple. It requires specially trained, multi disciplinary team to diagnose and train the child to lead as normal life as possible. Ophthalmologist role is to exclude other causes of reading difficulty i.e. error of refraction, muscle imbalance, bilateral amblyopia and treat them as far as possible. Agnosia. Visual agnosia is a condition where a person can not recognise an object by sight but can recognise the object by touch or sound. This is caused due to lesions of Broadmanns area 18.16 Visual hallucination. This is a condition where the patient complains of seeing an object that is not present. There are two types of hallucinations : (i) Formed hallucination where the objects claimed to be seen are identifiable objects. (ii) Unformed hallucination consists of lights, colours or amorphous images. There may be a mixture of both. Many visual hallucination may be associated with auditory hallucination in psychological causes. Many of the drugs are known to produce visual hallucination. Scotomas. Scotomas are defects in field of vision. They can be central i.e. round 30 of point of fixation or peripheral beyond 30. They are called absolute if the size and densities of scotoma does not change with size, colour of target and illumination. They are called relative if the size and density of scotoma changes with size and colour of target and illumination. A scotoma is said to be steep if the margins of the field defect do not change with change in size of object. If the margins of the scotoma is different with different targets then the scotoma is called sloping. A congruous field defect refers to similarity of scotoma to the contra lateral scotoma when they are superimposed on each other, they should match in outline. The scotomas can be : 1. Choroioretinal 2. Retino neural 3. Optic nerve type 4. Chiasmal 5. Optic tract type 6. Geniculate

582 7. Optic radiationTemporal, perital 8. Cortical

PEDIATRIC OPHTHALMOLOGY

Chorioretinal scotomas can be isolated or multiple without any fixed pattern. They do not respect either and horizontal raphe of retina or vertical line of chiasmal lesion. They may be unilateral or bilateral. Retino-neural scotomas can be : Central Centrocecal or Paracentral. They are due to involvement of papillo macular fibres. A scotoma is said to be central when it covers the point of fixation. When the central scotoma extends in to the blind spot it is called centrocecal scotoma. If some of the papilomacular fibres other then macular are involved, the scotoma is called para central. This does not involve either the point of fixation or blind spot. The lesion when involve, the nerve fibre bundle cause : Bjerrums scotoma Seidel scotoma Roennes step The two Bjerrums scotoma may join to form an annular scotoma with Rennes step. These scotomas are monocular. The involvement of the other eye is independent of first eye. Scotomas due to lesions of optic nerve produce central scotoma that may be relative or absolute. They are unilateral unless the contra lateral nerve is also involved. The scotomas of optic nerve are associated with : Diminished vision Diminished colour sense Diminished contrast sensitivity and Afferent pupillary defect. If the lesion is nearer the chiasma, it will produce ipsilateral diminished vision and central scotoma with absolute or relative contra lateral hemianopia. Scotomas due to chiasmal lesions : The scotoma contains uncrossed temporal fibre of ipsilateral eye and crossed nasal fibres of contra lateral eye. The ratio of crossed to uncrossed fibres is 3:217. Crossing of nasal fibres is not straight, the inferior nasal fibres form a curve that encroaches the posterior end of the contra lateral optic nerve. The superior nasal fibre similarly form a bend into the anterior end of ipsilateral optic tract. These arrangements result in various types of scotomas due to location of the lesion, size of the lesion and number of lesions. 1. The anterior chiasmal lesion on one side only produces ipsilateral central scotoma and contra lateral hemianopia.

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2. Lesions posterior chiasma will produce homonymous hemianopia i.e. temporal loss on the one side and nasal loss on the contra lateral side. The defects are incongruous. 3. Lesions pressing the mid chiasma - This is the commonest type of field defect produced by sellar or suprasellar lesions. It is a heteronymous field defect. It produces bitemporal hemianopia. The bitemporal hemianopia may start in one eye in the superior temporal quadrant and gradually increasing towards inferior temporal quadrant. In the right eye, the progress is clockwise, in the left eye it is anticlockwise. Initially the vertical line is respected i.e. the field change does not spread towards the nasal side. Once the whole of the inferior temporal quadrant has been lost, the field encroaches beyond the midline. If the lesion is small enough to involve the junction of crossing of macular fibres, there will be small central bitemporal hemianopia more for red object than for white.18 Lesions of chiasma do not cause afferent pupillary defect. Commonest cause of mid chiasmal lesions is pituitary tumour, other causes that cause bitemporal hemianopia are hydrocephalus, inflammation, infection, craniophryngioma, glioma of optic nerve or chiasma. 4. Binasal hemianopias are rare, are not met with in children. It requires two lesions pressing upon the chiasma from two temporal sides. Scotomas due to optic tract lesions. The optic tract on each side contain ipsilateral uncrossed temporal fibres and crossed nasal fibres from the contra lateral side. Hence they produce homonymous hemianopia and the field is split through the fixation19. They may be associated with afferent pupillary defect, the lesions are incongruous. Posteriorly placed lesions produce more congruity20, vision is generally good. Scotomas due to geniculate lesions. Lesions of lateral geniculate body are rare. As the lateral geniculate body is relatively small area, and contains predominantly macular fibres, the lesion is incongruous homonymous hemianopia. The scotomas are sectorial, the apex points towards the point of fixation. Good vision is possible. Scotomas due to lesions of optic radiation. After synapsing in the lateral geniculate body, the visual pathway fans out in the brain substance. There are mainly two types of scotomas : 1. Due to lesions of temporal lobe (Meyers loop). The scotoma is called pie in the sky due to its shape which is sectorial with apex towards point of fixation in the superior quadrant. The scotoma is homonymous and respect the midline. Small vascular lesions generally cause quadraniopia. Large lesions cause hemianopia. The lesions are due to involvement of anterior inferior fibres. Good vision is rule. The pupillary response is unaffected. 2. Due to lesions in parietal lobe. The lesions involve superior fibres of optic radiation produce quadranopia in lower quadrant and called pie on the floor. The scotoma are homonymous and congruous. There is good vision with normal pupillary reaction. Scotomas due to lesions of visual cortex. The fibres from the optic radiation synapse in calcarine fissure in occipital lobe are mostly macular that is located at the top of the occipital

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cortex. This area has dual blood supply from middle cerebral and posterior cerebral arteries. The scotomas are congruous, homonymous and hemianopic. The scotomas may involve macula or macula may be spared. The scotomas respect vertical meridian can respect horizontal meridian as well. Sometimes a temporal crescent may be present. Probable causes of macular sparing are : 1. Dual blood supply to the occipital cortex. 2. The macular area is spread over a large area hence it is possible that enough macular fibres are spared to retain macular vision. 3. It is presumed that there is double innervation of macular area. Some specific type of field defects : 1. Enlargement of blind spot is not always diagnostic. It is seen in : (i) papilledema (ii) Chronic simple glaucoma (iii) Opaque nerve fibres (iv) Drusen of optic nerve (v) Coloboma of optic disk (vi) Peripapillary edema of retina (vii) Juxtapapillary chroiditis. 2. Constriction of peripheral field : (i) Onset of optic atrophyPost papilledematous or post neurotic. (ii) Retinitis pigmentosa (iii) Advanced glaucoma (iv) Hysteria (v) Malingeing 3. Altitudinal field defect : (i) Acute ischaemic optic neuropathy (ii) Occlusion of central retinal artery branch (iii) Trauma to superior occipital lobe (iv) Optic tract syndrome Cortical blindness. In these cases the patient is blind but denies blindness. The pupillary reaction is normal. There are no fundal findings that can be attributed to blindness. The lesion is bilateral, occipito cortical. Bilateral homonymous hemianopia. These scotomas are difficult to chart, have poor prognosis. Vision may not improve beyond hand movement, is caused due to ischaemic lesion of both the occipital cortexes. There may be some gap between involvement of two cortexes.

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Amblyopia22, 23, 24, 25, 26


Amblyopia is a common cause of diminished vision in children which if not treated early results in permanent loss of vision generally in one eye but on occasions it may be bilateral. It has been defined variously from time to time. Most accepted description states it to be a symptomatic disturbance of vision in children due to deprivation of vision or abnormal binocular interaction.24 The cause of which can not be explained by presence of disorders of ocular media or visual pathway. When amblyopia is present without any detectable cause, is called pure amblyopia.23 Prevalence of amblyopia ranges between 0.5 to 3.5 percent in school going children.25 It is seen equally in boys and girls. No race is immune. There is no fixed hereditary factor, however, the predisposing causes like error of refraction or concomitant squint may have genetic background. Amblyopia develops due to failure of visual pathway development, which may start at birth or soon after. Development of amblyopia after full maturation of visual pathway is rare. Onset after six to seven years is almost unknown. For development of visual pathway it is essential to have a clearly formed image on the macula. The macular development is stretched up to 6 to 7 years post natal. Any derangement of formation of vision during this period is bound to cause amblyopia. Amblyopia developing early is more difficult to manage than those developing later. Anything that prevents formation of equally clear image on both the maculae i.e. physical obstruction like ptosis, corneal or lenticular opacity, anisometropia or squint will result in amblyopia. Characteristics of amblyopic eyes : 1. Amblyopia starts in the critical period of development of visual pathway. 2. It is seen in children under six to seven years of age. 3. If amblyopia is not treated before eight years of age it becomes permanent. 4. Amblyopia is mostly uniocular disorder however in a very small percentage of cases it can be bilateral. 5. It is not possible for the parents to know that the child is amblyopic unless the child undergoes examination of the vision. This happens more often in children who have amblyopia in spite of straight eyes. 6. In contrast to this a child may be brought with squint and found to have amblyopia. This is also known as amblyopia with squint. 7. Diminished distant vision - The amblyopic eye always has poorer vision than the normal fellow eye. A difference of two lines on Snellens chart after best correction is diagnostic. 8. Vision in amblyopic eye may be as low as perception of light in congenital squint. Generally vision in amblyopic eye ranges between 6/12-6/18. A child may have 6/36 vision before starting treatment and end up with 6/12 vision. This eye will still be considered to be amblyopic.

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9. Crowding phenomenon. An amblyopic eye has better vision when single optotypes are shown but when letters of same size in a line are shown, the child is unable to read them. Greater the difference between the single letter vision and linear vision, poorer is the prognosis. 10. Vision with neutral density filter. In non amblyopic eye if neutral density filter is put in front of the eye, the vision is reduced by one to two lines but not in amblyopic eye when neutral density filter of increasing strength is put in front of the eye the vision either remains same or even improves.27 In case of organic amblyopia i.e. toxic amblyopia vision is markedly reduced with neutral density filter.26 The explanation for the phenomenon isThe scotopic and mesopic vision of an amblyopic eye is equal to non amblyopic eye but less in photopic vision. Placing a neutral density film simulates scotopic vision, thus it is not affected. 11. Colour vision and dark adaptation are normal in amblyopia. 12. Contrast sensitivity, grating acuity, Vernier acuity and spatial localisation are lowered. 13. Visual evoked response to pattern stimuli is poorer in amblyopic eye. 14. There may be milder degree of relative afferent pupillary response. 15. The amblyopic eye may have mobility defect in the form of pursuit, fixation and saccades. 16. There may be latent nystagmus. 17. There may be poor accommodation. 18. Severe degree of amblyopia may have eccentric fixation. Classification of amblyopia. There is no unanimity in classification of amblyopia. Various classification have been done on the basis of presumed physiopathology of the condition. Many of the terms used in the past are either avoided22 or altogether discarded. Various terms used in amblyopia are : 1. Strabismic amblyopia. Amblyopia due to abnormal interaction between two eyes due to squint. 2. Anisometropic amblyopia. Amblyopia due to uncorrected unequal refraction into eye. 3. Stimulus deprivation amblyopia is caused due to blurring of retinal image due to opacity in the media, i.e. congenital or traumatic cataract, corneal opacity, ptosis. It was formerly called amblyopia exanopsia. The term is no more in vogue22, 24. 4. Ametropic amblyopia is caused due to high uncorrected error of refraction. 5. Meridional amblyopia due to high astigmatism in a particular meridian. 6. Amblyopia of arrest due to arrest of development of vision. 7. Amblyopia of extinction due to secondary loss of vision. The last two terms are also not used any more. The toxic amblyopia, nutritional amblyopia and amblyopia in various neuropathies like retrobulbar neuritis do not fit into the definition of amblyopia hence they are not included in this chapter.

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One of the widely used classification is to divide amblyopia into : (1) Organic amblyopia and (2) Functional amblyopia. The latter is used to denote psychological disturbance and does not fulfil the criteria of amblyopia. Organic amblyopia is also known as developmental amblyopia and can be unilateral or less commonly bilateral. The bilateral developmental amblyopia is generally stimulus deprivation amblyopia and the causes are - Bilateral cataract of equal density, high uncorrected hypermetropia and motor nystagmus. The uniocular developmental amblyopia can be strabismic or anisometropic separately or in combination. For practical purposes the best way is to classify amblyopia is amblyopia with squint and amblyopia without squint. The latter is called straight eye amblyopia. Amblyopia with squint : Characteristics : 1. The child constantly uses one eye for fixation. The deviating eye becomes amblyopic, patients with alternate fixation do not develop amblyopia. 2. Esotropes develop more amblyopia than exotropes. Congenital esotropes do not develop amblyopia due to cross fixation. 3. Hypertropes generally do not develop amblyopia as they manage to maintain fusion by abnormal head posture.26 4. Micro strabismus causes more amblyopia when compared to large degree squint27. 5. Strabismus causes more amblyopia than anisometropia. 6. Strabismic amblyopia is caused due to suppression of image in the squinting eye. 7. Amblyopia with squint report for checking earlier than straight eye amblyopia, not because they are aware of diminished vision but for cosmetic blemish of squint. 8. Degree of amblyopia is influenced by duration of squint rather than age of onset of squint. 9. Earlier the squint develops, deeper is the amblyopia and more difficult to treat. Thus in case of congenital squint where inhibition is present at birth, the vision in the amblyopic eye will not improve beyond perception of light.25 10. The suppression is at the level of cortex.27 11. The condition is less amenable to treatment than straight eye amblyopia. 12. No correlation exists between severity of amblyopia and angle of squint. Micro tropias are known to cause severe amblyopia. Straight eye amblyopia : Characteristics : 1. Error pf refraction : (i) High degree of hypermetropia in one eye, the amblyopic eye called anisometropic amblyopia. (ii) Both eyes have equal errors of refraction mostly of high degree. (iii) No significant error of refraction.

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2. Amblyopia is obligatory i.e. suppression is present when the eye squints and continues during forced fixation28. 3. The condition goes unnoticed because the child is unaware of less vision in one eye and the parents do not notice because there are no external signs. 4. The eyes have fairly good binocular function. 5. The steriopsis is good. 6. Chances of eccentric fixation is minimal or absent. 7. The blurred image in the amblyopic eye acts as hindrance to binocular vision. 8. Only rough correlation exists between degree of anisometropia and depth of amblyopia. 9. It is possible to have amblyopia in both eyes. 10. The condition has better chance of improvement following treatment. Diagnosis. Diagnosis of amblyopia in squint is easy. All squinting eyes should be examined for possibility of amblyopia. Strabismic children have 15 times more chances of developing amblyopia than non strabismic children. Squint is responsible for one third case of amblyopia. The diagnosis depends on : 1. Recording of vision. 2. Crowding phenomenon. 3. Unchanged or improved vision with neutral density filter. Treatment : 1. Management of amblyopia is rewarding (i) If diagnosed early, (ii) If treatment is initiated early, (iii) If treatment is continued for sufficient time and maintained for sufficient time after vision has improved and come to equal level in both eyes. All above factors depends upon compliance of the patient and co-operation of parents and teachers. Management begins withRefraction under cycloplegia followed by prescription of best possible power in both eyes which gives the child a comfortable vision according to standard protocol of prescription of glasses for children with special attention to astigmatism. Any opacity in the media should be removed, so should be any physical obstruction like ptosis, tumours of lid. This is followed by occlusion of sound eye to begin with. Occlusion is a time honoured method of treatment for amblyopia in children. Occlusion therapy should take into consideration following parameters : 1. When to start occlusion. 2. Gap between two visits.

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3. Type of occluder to be used. 4. How long should occlusion be continued. 5. Should the better eye be occluded and how long. Occlusion should be started as soon as amblyopia has been confirmed. Occlusion after six years of age do not have much impact on amblyopia though it has been observed to give fairly good result up to age of fifteen.23 The occlusion should be complete and constant to begin with. There are various types of occluders. The best is a skin patch attached to the forehead and check by an adhesive tape. This occludes the eye fully. The child has no chance to peep over the patch which is always possible with spectacle occluders i.e. Doynes occluder or ground glass occluders. These are not favoured because the child has tendency to peep over the occluders. In a co-operative child an opaque contact lens may give equally good result like a patch especially if the child is already using contact lens. 1. Minimum amount of occlusion required per day is not known. 2. Constant occlusion for a period calculated as per age of the child gives good results. 3. 75% waking hours occlusion also gives fairly good result and has the advantage of not being amblyogenic to the better eye. 4. Occlusion for less than 50% waking hours are not likely to be of much help. Calculation of duration of occlusion. The commonly used formula is one week for each year of age in between the visits. Younger the child better is the result of the treatment. Occlusion of better eye is called conventional occlusion that is effective irrespective of fixation pattern of fixing eye. Occlusion of amblyopic eye is called inverse occlusion. A rough estimate of occlusion as per age of the child is as follows26 Age 0-1 year 1-3 years 4-6 years More than 6 years Conventional occlusion Three days Four days Six days Seven days Inverse occlusion One day One day One day Nil

As far as possible occlusion should be full time i.e. during all waking hours. Part time occlusion means that the eye is occluded only for a few hours. During rest of the time both the eyes are open which goes against the principle of total occlusion. Occlusion therapy may be accompanied by enhanced near vision work like tracing picture, joining dots, hand video games. Follow up. The child should be followed up at the frequency of 1 week per year of age, i.e. a child aged one should be examined every week while a child of three years is examined after three weeks of occlusion. What to look for on follow up visits : 1. On every visit childs vision in both eyes must be recorded separately with correction when needed on the same Snellens chart in the same setting always. 2. The fixation pattern of the amblyopic eye should be noted on every visit.

590 How long should occlusion be continued ? Occlusion is continued till : 1. Vision in amblyopic eye is as good as in normal eye. 2. Fixation becomes alternating.

PEDIATRIC OPHTHALMOLOGY

3. There is no improvement of vision following occlusion for three months. 4. Once vision has been equalised or the child has developed alternating. Full time occlusion can be stopped and the dominant eye is occluded for part of the waking hours. This part time occlusion is continued up to age of eight years after which chances of improvement are less. Visual improvement is generally better in straight eye amblyopia than amblyopia with squint. Anisometropic amblyopia may require occlusion in addition to correction of refraction. Amblyopia is known to recur following completion of occlusion. So all children with amblyopia should be examined frequently and as soon as there is recurrence, occlusion is started as was done on the first occasion. Occlusion of sound eye is contra indicated in amblyopia with eccentric fixation. CAM vision stimulator. The normal eye is occluded for 7 minutes. The child uses the amblyopic eye to draw patterns on the transparent cover placed over the stimulator that has slow rotation, high contrast grating with sharp edges. The child may be exposed to the stimulator 10 to 20 times at a stretch. This method is not superior to conventional occlusion. Complication of occlusion. Occlusion is a safe, simple and sound method for treatment of amblyopia provided it is done properly in selected children for sufficiently long time. The co-operation of the child and parent are some of the prerequisites.30 They are : 1. Occlusion amblyopia in better eye. 2. Development of new strabismus. 3. Worsening of pre-existing strabismus 4. Intractable diplopia 5. DVD 6. Allergic reaction to occluder 7. Infection may develop in occluded eye. Pharmacological defocusing (Penalisation). This may be considered as a modified type of occlusion without actually covering the eye. This uses cycloplegic drug and spectacle in various combination to change the accommodation. The vision is blurred in better eye and forcing the amblyopic eye to see. The best results are obtained in amblyopia of moderate depth in moderate hypermetropia. The amblyopic eye is given full correction. The better eye is denied spectacle correction. One drop of one percent atropine is instilled in the better eye once a day. Follow up and duration is similar to that of occlusion. It is recommended if the child does not tolerate occlusion or peeks over the occlusion. It takes more time for improvement than occlusion. Surgery. Squint surgery is performed after amblyopia has been treated. However ptosis, haemangioma of lid, cataract are operated before starting occlusion.

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Medical treatment. Levodopa has been tried in small groups of amblyopic children with improvement of vision in both amblyopic and non amblyopic eye without any side effect.

REFERENCES
1. Miller S.J.H. ; Symptomatic disturbance of vision in Parsons disease of the eye. Seventeenth edition. p-231236, Churchill Livingston, London, 1984. 2. Marmor M.F. ; Clinical physiology of the retina in Principles and practice of ophthalmology. Vol. II. p-868887, Edited by Peyman G.A., Sanders D.R., Goldberg M.F. First Indian edition. Jay Pee Brothers, New Delhi, 1987. 3. Brain R.W. ; Visual fibres and visual fields in Diseases of nervous system. Sixth edition. p-4958, Oxford Medical Publication, London, 1962. 4. Brain R.W. ; Aphasia in Diseases of nervous system. Sixth edition. p-8490, Oxford Medical Publication, London, 1962. 5. Shaffer D.B. ; Fundus albipunctatus in Text Book of ophthalmology. Edited by Scheie H.G. and Albert D.M. Ninth edition. p-307, W.B. Saunders Company, Philadelphia, 1977. 6. Agarwal L.P. ; Properties of light in Principles of optics and refraction. Second edition. p-34, C.B.S. Publishers, New Delhi, 1979. 7. Majji A.B. ; Sharma Y.R., Rajashekhar Y.L. and Nath R. Colour vision and colour blindness in Modern ophthalmology. Vol. II. Second edition. p-10321036, Edited by Dutta L.C. Jay Pee Brothers, New Delhi, 2000. 8. Speath G.L., Auerbach V.H. ; Cone vision in Pediatric ophthalmology. Vol. II, second edition. p-10611064, Edited by Harley R.D. WB Saunders Company, Philadelphia, 1983. 9. Seal S.K. ; Symptomatic disturbance of visual function in G.N. Seals Text Book of Ophthalmology. Fifth edition. p-352353, Current Book International, Kolkata, 2002. 10. Boger W.P., Peterson R.A. ; Pediatric ophthalmology in Manual of ocular diagnosis and therapy. Third edition. p-262263, Edited by Deborah Pavan Langston. Lippincot Williams and Wilkins, Philadelphia, 1991. 11. Duke Elder S. ; Colour sense in System of Ophthalmology. Vol. VII. p-380383, Henry Kimpton, London, 1962. 12. Boger W.P. and Peterson R.A. ; Achromatopsia in Manual of ocular diagnosis and therapy. Third edition. p-273274, Edited by Deborah Pavan Langston. Lippincot Williams and Wilkins, Philadelphia, 1991. 13. Kanski J.J. ; Neurophthalmology in Clinical ophthalmology. Second edition. p-441, Butterworth, London, 1989. 14. Martyn Lois J. ; Colour vision disorder in Pediatric ophthalmology. Vol. II. Second edition. p-781, Edited by Harley R.D. WB Saunders Company, Philadelphia, 1983. 15. Keys M.P. ; Dyslexia in Current ocular therapy. Fifth edition. p-213214, Edited by Fraunfelder F.T., Roy F.H. W.B. Saunders Company, Philadelphia, 2000.

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16. Roy H.F. ; Visual agnosia in Ocular differential diagnosis. Third edition. p-453, Jay Pee Brothers, New Delhi, 1984. 17. Shirley H. Wray. ; The visual fields in Manual of ocular diagnosis and therapy. Third edition. p-329, Edited by Deborah Pavan Langston. Lippincot. Williams and Eilkins, Philadelphia, 1991. 18. Rosenberg M.A. ; Topographic diagnosis of field defect in Principles and practice of ophthalmology. Vol. III. First Indian edition. p-19361943, Edited by Peyman G.A., Sanders D.R. and Goldberg M.F. Jay Pee Brothers, New Delhi, 1987. 19. Norma Graber. Matching field defect to pathology in Visual field examination. First Indian edition. p-18-33, Jay Pee Brothers, New Delhi, 1989. 20. Bajandas F.J., Kline L.B. ; Visual field in Neurophthalmology review manual. Third edition. p-142, Jay Pee Brothers, New Delhi, 1989. 21. Rabinowicz M. ; Amblyopia in Pediatric ophthalmology. Vol. I, edition second. p-293341, Edited by Harley R.D. WB Saunders Company, Philadelphia, 1983. 22. Eggers H.M. ; Amblyopia in Text Book of Ophthalmology. First edition. p-13.1 13.16, Edited by Podos S.M. and Yanoff M. Gower Medical Publication, London, 1993. 23. Rohatgi J.N. ; Amblyopia in Modern ophthalmology. Vol. II. Second edition. p-875878, Edited by Dutta L.C. Jay Pee Brothers, New Delhi, 2000. 24. Sharma P. ; Amblyopia in Strabismus simplified. First edition. p-4149, Modern Publishers, New Delhi, 1999. 25. Rohatgi J.N. ; Amblyopia in Squint basic and clinical aspect. First edition. p-8091, CBS Publication, New Delhi, 2003. 26. Mohan K. ; Amblyopia in Clinical practice in ophthalmology. First edition. p-529533, Edited by Saxena S. Jay Pee Brothers, 2003. 27. Seal S.K. Amblyopia in GN Seals Text Book of Ophthalmology. Fifth edition. p-349351, Current Book International, Kolkata, 2002. 28. Boger W.P. and Peterson R.A. ; Pediatric ophthalmology in Manual of ocular diagnosis and therapy. Third edition. p-307, Edited by Deborah Pavan Langston. Lippincot. Williams and Wilkins, philadelphia, 1991. 29. Lyle T.K. and Wyber K.C. ; Adaptation to development of ocular deviation in Lyle and Jacsons practical orthoptics. Fifth edition. p-82, First Indian edition. Jay Pee Brothers, New Delhi, 1994. 30. Keech R.V. ; Functional amblyopia in Current ocular therapy. Fifth edition. p-214 216, Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000. 31. Fiona J. Rowe. ; Amblyopia in Clinical orthoptics. First edition. p-131135, Blackwell Science Ltd., London, 1997.

CHAPTER

17

Disorders of Extra Ocular Muscles and Paralytic Squint in Children


General information about extra ocular muscle There are six muscles attached to each eye, to move the eye in various directions. The movements are either voluntary or reflex. The muscles are striated in nature and are supplied by cranial nerves - Three, four and six for motor function. The sensory supply to all of them are through fifth nerve, they are not innervated by autonomic nerves. The six extra ocular muscles (EOM) areFour recti i.e. the superior rectus (SR), the lateral rectus (LR), the medial rectus (MR) and the inferior rectus (IR). The four recti muscles combine to exert a retracting influence also on the globe1 because they originate from the apex of the orbit and are inserted in front of the equator of the globe. The two oblique muscles are the superior oblique (S.O.) and the inferior oblique (I.O.), the functional origin of both are in the rim of the orbit in front of the eye ball and are inserted behind the equator of the globe. The two obliques combine to give a protracting influence on the globe.1 The six muscles can be divided into two main groupsHorizontally acting muscles i.e. the medial and lateral recti. They have only primary action of moving the eye in horizontal plane. They are inserted in front of the equator in a straight line. Their muscle plane coincides with visual axis hence they have only horizontal action. Cyclo vertically acting muscles They are four in numbers, two recti i.e. the superior and inferior recti both are supplied by single cranial nerve i.e. oculomotor and both are adductors. The remaining two muscles are the two obliques i.e. the superior and inferior obliques, both have functional origin in front of the globe and are abductors of the globe. Unlike the vertically acting recti, they have different motor supply. The superior oblique is supplied by the trochlear nerve while the inferior oblique is supplied by the lower division of the oculomotor nerve. The muscle plane of cyclo vertical muscles do not coincide with visual axis in primary position. The extra ocular muscles contain two types of fibers.2 The slow fibres and the fast fibres. The former are on the exterior surface of the muscles and produce graded contracture of the muscles. The latter are situated on the interior surface of the muscles and are concerned with rapid movements. 593

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The ocular movements can be divided into four broad groups3The saccades, the smooth pursuit movements, the vergence and vestibulo ocular movements. The saccades are rapid conjugate eye movements, they are voluntary movement due to quick burst of activity. They jump from one fixation to a new object of regard.4 They are present at birth.3 The smooth pursuits are involuntary movements that follow a slow moving object. They are also conjugate movements. The vergence5When the eye movements are disjugate (disjunctive), the movements are called vergence. The eyes move in opposite direction to allow both fovea to maintain fixation at the same time even when the object moves near or farther away, the former is called convergence and the other is called divergence. The advantage of this bifoveal fixation is binocular single vision and stereopsis.4 The vestibulo ocular movements3 These movements are related to maintaining eye position when there is an altered head position or body posture. The ability of the human eyes to move in various directions is immense, the human eyes can have almost 180 of binocular field. The eyes can be moved 50 in each direction from the primary position. Various position of the eye (Gaze) 1. Primary position. When the eyes fix an object at 20 feet straight ahead with the head straight. 2. Secondary position consists of right gaze, left gaze, straight up and straight down gaze. 3. Tertiary position. There are four oblique position of the eye. 4. The cardinal position. They are six in numbers brought about by one muscle and its yoke muscle. Agonist and antagonist muscles These are descriptive terms used to denote the action of an extra ocular muscle. An agonist is the prime mover for a required direction. An antagonist of the same eye acts directly against the agonist. If right lateral rectus is agonist for right lateral gaze, the medial rectus is its direct antagonist in the same eye. Synergist muscle A muscle in the same eye that enhances the action of a particular muscle to achieve a particular action is called synergist. In fact both of them are synergist to each other. For example superior rectus is synergist to inferior oblique for elevation. It is synergist to superior oblique for intortion. Adduction is enhanced for medial and inferior rectus. Yoke muscles. These are pairs of muscles one from each eye that work together in binocular movement in one direction (version).

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Duction is movement of one eye i.e. abduction moving laterally, adduction moving medially. Sursum duction (elevation)7.8 is upward movement either in primary position or when the eye is either adducted or abducted. Deorsum duction is downward movement (depression or infraduction). This can be in primary position or secondary position of adduction or abduction. Similarly there can be in cycloduction when the twelve Oclock meridian dips nasally or excycloduction when twelve Oclock meridian dips temporally. Versions. These are binocular movements in the same direction i.e. conjugate movement. Versions are noted in three diagnostic directions. Right sided binocular movements are called dextroversion, levo version denotes left binocular movements, dextro elevation and dextro depressions represent right and up, left and down movement respectively. Sursum version means moving both eyes up. Deorsum version is moving both eyes down. The binocular torsional movements are Dextro cycloversion when the right eye extorts and left eye intorts at the same time. Levo cyclo version similarly denotes right intorsion and left extorsion. Advantage of testing version is that paresis that may be missed on uniocular movement can be diagnosed on binocular testing. Vergences Binocular movements in opposite directions are called vergences. They areConvergence, when both eyes move inwards and divergence when both eyes move outwardly. Laws governing ocular movements They are Herings law of equal innervation and Sherringtons law of reciprocal innervation. Herings law of equal innervation means that the synergists of one eye gets equal and simultaenous innervation for any movement, e.g. amount of innervation of the right medial rectus is exactly the same as that of left lateral rectus and inhibitory responses to the right lateral rectus is exactly the same as that of left medial rectus. Sherringtons law states that increased action of an extra ocular muscle associated with diminished contraction of its antagonist for example during convergence there is increased contraction of both medial recti with reduced contractibility of both lateral recti. Neural control of ocular movements Binocular movements of the eye consists of a delicate control of many systems and not confined to motor nerve supply to the muscles only. They consist of two independent groups of movement i.e. version and vergence7, both are binocular movements. The versions are conjugate movements while vergence are disjugate movements. Depending on input, the ocular movements can be classified into fast eye movement, slow eye movement and vergence eye movement.

596 The fast eye movements : 1. Saccade 2. Quick phase of nystagmus.

PEDIATRIC OPHTHALMOLOGY

The fast eye movements bring the object of interest on the fovea. The slow eye movements : Smooth pursuit Optokinetic Vestibular Vergence The slow eye movements hold the image of the object of interest on the macula. In vergence the eyes move in opposite direction i.e. converge or diverge so that images of single object are placed on both foveae.8 The fast eye movements (saccade) are brought about by fronto mesencephalic path ways and superior colliculus. The quick phases of nystagmus is brought about by para median reticular formation in mid brain and pons. The slow eye movements (smooth pursuits) are brought about by parieto-occipial temporal pathway. The vestibular slow eye movements are brought about by labyrinthine-pontine-pathway.8 Anatomy of extra ocular muscles The horizontal muscles The medial rectus muscle This is a straight muscle, the plane of the muscle coincides with the visual axis of the eye. It originates from the apex of the orbit from the medial side of circle of Zinn. It is 41 mm (actually 40.8 mm) in length. It passes forward between the medial wall of the orbit and the globe with in the horizontal meridian of the eyeball. The anterior 3.7 mm to 4.5 mm (mean 4 mm) is its tendon that is 10.3 mm wide. It inserts in the sclera in vertical linear fashion 5.5 mm away from the limbus. Its insertion is nearest to the limbus when compared to other extra ocular muscles insertion. It is innervated by lower division of third cranial nerve that pierces the muscle from its ocular surface. It gets its blood supply by two twigs of anterior ciliary artery. The venous drainage is through the corresponding veins of the same name that drains in the superior and inferior opthalmic vein. The sensory supply is through the trigeminal. It has only one action in horizontal plane that is adduction. Adduction is better in slightly lowered gaze, this helps in converging the eye to near work. Under action of this muscle causes exotropia. Its arc of action with the globe is 7 mm. Near the apex of the orbit, the dura of the optic nerve is closely related to the muscle. The lateral rectus The lateral rectus arises from the apex of the orbit mostly from the lateral part of the circle of Zinn and partly from spina lateralis recti which is a spike in the greater wing of the sphenoid. Its muscle plane coincides with the visual axis so it has only one horizontal action

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i.e. abduction. Its average length is 41 mm. It passes between the lateral orbital wall and the globe. Its tendon is about 9 mm in length and 9.2 mm wide. It gets inserted in front of the equator 7 mm from the limbus in a vertical line. It is innervated by abducent nerve that pierces the muscle from the inner surface. It gets its blood supply from a single anterior ciliary artery, all other recti get their blood supply by two anterior ciliary arteries. The anterior ciliary arteries do not supply any of the obliques. Its venous supply is via the veins of the same name that drain in the ophthalmic vein. Its sensory supply is through trigeminal. The action of lateral rectus is best for distant fixation in slight up gaze. The upper border of the lateral rectus almost reaches the lower border of the lacrimal gland along with lacrimal artery in the anterior part of the orbit. The tendon of the inferior oblique lies between the lateral rectus and the globe at its insertion. The cyclo vertical muscles These are two recti, the superior and inferior rectus and two obliques - the superior and the inferior obliques. The actions of the cyclo verticals are more in number and more complex in nature. They have all the three actions i.e. primary, secondary and tertiary in different gaze. Their long axes do not coincides with the visual axis in primary position. The two cyclovertical recti are adductors while the two obliques are abductors. The superior cyclo verticals, the S.R. and S.O. are intortors. The two inferior cyclo verticals are extortors. The primary action of all the cyclo vertical muscles is best in abduction. The obliques have effective origin in front of the globe and insertion behind the equator. The recti originate from the apex of the orbit and are inserted anterior to the equator. The cyclo vertical recti The superior rectus (S.R.) The superior rectus originates from the upper part of the circle of Zinn, lateral side of optic foramen and sheath of the optic nerve, it travels forward under the roof of the orbit. Its average length is 42 mm. Its tendon is 5.5 mm in length and is 10.5 mm wide at the insertion, which is 7.7 to 7.8 mm away from the limbus. The tendon does not insert in a straight line but has a mild convexity forward. The superior rectus lies under the levator and above the superior oblique. Its insertion is farthest from the insertion of any other rectus. In primary position, the long axis of the superior rectus form an angle of 23 with the visual axis. The axis of the muscle coincides with visual axis only when the eye is abducted 23. At this position, if the superior rectus contracts, the eyeball will be elevated without any torsion. Elevation is the primary action of the superior rectus. Ability to elevate the globe increases with abduction and decreases in adduction. For intortion, the eyeball rotates to 67 medially, it is no more an elevator but is an intortor which is secondary action of superior rectus. Adduction is the tertiary action of the superior rectus. Thus superior rectus is an elevator, intortor and adductor of the globe. It is supplied by the upper division of the third nerve. It gets its blood supply by two anterior ciliary arteries. It has its corresponding venous drainage. Its sensory supply is through the trigeminal. One of its protective reflex action is Bells phenomenon. The superior rectus and inferior rectus work together as adductor but oppose each other as cyclo vertical muscles.

598 Inferior rectus (I.R.)

PEDIATRIC OPHTHALMOLOGY

Inferior rectus originates from the circle of Zinn. It travels slightly down and anteriorly under the globe and inserts in the sclera slightly temporal to its origin, 6 mm from the limbus in a convex fashion with convexity anteriorly. Its arc of the contact with the globe is 6.5 mm. Its length is 40 mm. It has a tendon 5.0 mm long, the width of the tendon is 10.0 mm at insertion. The inferior oblique passes under it at the anterior part. The inferior rectus is intimate with inferior oblique and the lower lid muscle. It merges with the ligament of Lockwood to give support to the globe from below. Its direction from its origin to insertion is similar to that of superior rectus. Its long axis forms an angle of 23 with the visual axis when the eyeball is in primary position. When the eyeball is abducted 23, the axis of the muscle and visual axis coincide at this position, it is a depressor of the globe that increases with abduction and decreases in adduction. The primary action of the inferior rectus is depression. Its secondary action is extorsion, which is best when the eye is adducted by 67. The tertiary action of inferior rectus is adduction. Thus the inferior rectus is a depressior, extortor and adductor. The oblique muscle The two obliques, the superior and inferior obliques are unique in their arrangement in relation to the globe and visual axis. The effective origins of both the muscles are in front of the globe from the medial side of the rim of the orbit, both get inserted behind the equator farther away from the farthest insertion of any rectus i.e. 14 mm and 17 mm for SO and IO respectively. Their main function is to keep the upper pole of the limbus at 12 Oclock position irrespective of the tilt of the head. They have a forward pulling effect on the globe, which is counteracted by backward pull of the recti. Hence in case of paralysis of third nerve with sixth nerve the eyeball is slightly proptosed. Both of them are abductors. The superior oblique The superior oblique has the distinction of having longest muscle belly, and tendon. The muscle is divided into a long and a short part. The shorter part is the effective part of the muscle. It is also a muscle that is innervated by single cranial nerve, a distinction shared by lateral rectus that too has individual nerve supply. The muscle is supplied by contra lateral nucleus of fourth nerve. Due to its peculiar position, the muscle is most susceptible to trauma. Congenital anomaly of superior oblique is very common. The superior oblique takes origin from the periosteum of the body of sphenoid little superior and medial to the optic foramen. The muscular part 40 mm in length runs anteriorly and nasally and upwards up to the trochlea at the superio medial aspect of the rim of the orbit where it passes over the pully and is converted almost fully to its tendon that is 20 mm in length. The tendon runs backwards, downwards and laterally below the superior rectus. The tendon is inserted in the sclera in a wide insertion behind the equator in the posterio lateral quadrant. The muscular part is called direct part while the tendon is called the reflected part. The attachment of the muscle to the trochlea is called its effective origin. The temporal side of the muscle is inserted nearer

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the limbus than the medial end. The temporal end is inserted about 14 mm from the limbus, the medial end is inserted about 19 mm from the limbus. The insertion is rounded with convexity away from the limbus. At the insertion, the posterior border is very near the vertex vein. The effective muscle plane is formed by the reflected part of the muscle, which forms an angle of 54 with the visual axis in primary position. When the muscle contracts from this position, the eyeball intorts. The primary action of superior oblique is intorsion. When the globe is adducted 54, the muscle plane coincides with visual axis and when the muscle contracts the eyeball is depressed, the secondary action is depression while the tertiary action is abduction. The muscle is supplied by fourth nerve that enters the orbit outside the circle of Zinn and enters the muscle belly from the orbital surfaces. All other extra ocular muscles get their nerve supply from the ocular surface. As the muscle lies outside the muscle cone, its motor nerve enters the orbit outside the circle of Zinn and the motor supply is from outside, the muscle is least affected by retro bulbar anaesthesia but well anathesized by peribulbar injection. Test for fourth nerve in presence of third nerve palsy. Involvement of superior oblique in presence of third nerve palsy is an important neuro ophthalmic test. In complete paralysis of third nerve, the eye can not be adducted, the vertical action of SO which is best in adduction can not be tested. The eye is already abducted which is best position for intorsion so if the patient attempts to look down the eyeball intorts. Blood supply. The muscle gets it blood supply from the superior muscular branch of the ophthalmic artery. The inferior oblique The inferior oblique too has few peculiarities. It is the shortest extra ocular muscle 37 mm. It has almost no tendon (only 1 mm). It is only extra ocular muscle that have true anterior origin from posterior lacrimal crest of the inferio medial part of rim of the orbit. Its insertion is very close to the fovea. After origin the muscle passes backwards and laterally between the floor of the orbit and inferior rectus with in the Lockwoods ligament. It passes under the globe and gets inserted 17 mm from the limbus, 1 mm anterior and below the fovea. The anterior end of the insertion lies in the same plane as lower end of insertion of the lateral rectus. The insertion of the muscle is covered by the lateral rectus. The action of the inferior oblique is as complex as superior oblique. Its primary action is extortion, which is best in abduction. The secondary action is elevation and tertiary action is abduction. Its motor supply is through the inferior division of the third nerve. Its blood supply is through the inferior orbital artery and medial muscular branch of ophthalmic artery. The muscle is most likely to be injured in fracture floor of the orbit.

600 Spiral of Tillaux

PEDIATRIC OPHTHALMOLOGY

The attachment of the extra ocular muscles is not the same for all the recti, the distance vary from muscle to muscle. The medial rectus is inserted nearest to the limbus followed by interior and lateral rectus. The superior rectus is farthest from the limbus, the curved imaginary line joining the insertion of the recti form a spiral called spiral of Tillaux. Its knowledge helps in finding the attachment of the muscles during squint surgery. Summary of action of extra ocular muscles All recti are Adductors except lateral. The Superiors are Intortors (SR and SO). The Inferiors are Extortors (IR and IO). The mnemonic for cyclo vertical muscles is SIN RAD. Where S In R AD stands for Superior stands for Inferior stands for Recti Stand for Adductor

Thus superiors are Intortors. The recti are adductor, this leaves the obliques as abductors and inferiors as extortors. Muscle actions Muscle 1. Medial rectus 2. Lateral rectus 3. Superior rectus 4. Inferior rectus 5. Superior oblique 6. Inferior oblique Primary Adduction Abduction Elevation Depression Intorsion Extorsion Secondary Nil Nil Intorsion Extorsion Depression Elevation Tertiary Nil Nil Adduction Adduction Abduction Abduction

Development of extra ocular muscles12,13,14 All voluntary muscles in the body develop from para-axial mesoderm. Ocular muscles are no exception. Presence of extra ocular muscles is visible as early as four weeks of gestation. The para axial mesoderm first forms a common pre muscle mass that condensates into two groups i.e. the pre mandibular and the maxillo mandibular condensation. The first is supplied by oculo motor. From the first condensation develop all the muscles supplied by the third nerve. The remaining two develop from maxillo mandibular condensation which again divides into two groups called second head somite and third head somite. From the former develops the superior oblique. The lateral rectus develops from the latter. The three muscle masses are destined to be supplied by three different cranial nerves. The oculomotor reaches the pre mandibular mass while the trocheal supplies the superior oblique developing from the second head somite and the abducent supplies the lateral rectus developing from the third head somite. The insertion of the muscles develop separately from the scleral condensation near the limbus. The muscle masses differentiate from behind forward hence the origin of the muscle is more constant than the insertion. The levator is the last to differentiate from the superior

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rectus. As the two muscles remain undifferentiated for long time and have a common motor supply i.e. upper division of third nerve that terminates in the levator after piercing the superior rectus. The congenital anomalies of levator are mostly associated with congenital anomalies of superior rectus. The medial rectus is the first muscle to differentiate. The third nerve reaches its designated muscle mass roughly at 7-8 mm stage while the abducent reaches the lateral rectus at 8-9 mm and the trochlear reaches the superior oblique at 10-12 mm stage. Development of oculomotor nerves12 The nerves concerned with movement of the eye develop from the neuroblast nearest to the cranial part of the neural tube. The nucleus of the third nerve is first to appear and the abducent the last. The fourth nerve reaches its target muscle last, its nucleus develops in between development of III and VI nucleus. The nerves start developing from the cranial end and terminate in the ocular end. Anatomy of the nerves involved in ocular movement Three cranial nerves i.e. the oculo motor, trochlear and abducent nerves are directly involved in ocular movements. Other neural pathway involved indirectly with ocular movements are8 : Fronto mesencephalic path Parieto-occipital temporal mesencephalic path Occipito mesencephalic path Para median pontine reticular formation Labyrinthine pontine path. The oculomotor nerve This is the third cranial nerve, it is the only nerve that supplies more than one extra ocular muscles, and intra ocular muscles. It has largest nucleus situated in the mid brain at the level of superior colliculum. It is divided into paired and unpaired nuclei. The paired nuclei are again divided into three sub-groups and supply the ipsilateral, medial and inferior rectus and inferior oblique individually. The unpaired nuclei are motor cell, pool of superior rectus, Perlias nucleus, caudal central nucleus for levator. The Perlias nucleus is supposed to be the center of convergence. The pre-ganglionic parasympathetic fibres originate from the Edinger Westphal nucleus. This nucleus also contains fibres for accommodation and light reflex. The nucleus of the four nerves is below the nucleus for inferior rectus. The superior rectus and superior oblique muscle have their motor pool in the contra lateral side. Connections of the third nerve nucleus15 The third nerve nucleus is connected with following tract and nuclei : 1. Cortico nuclear tract of both sides. 2. Visual cortex through tecto bulbar tract.

602 3. Pretectal nuclei of both sides.

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4. Nuclei of fourth, sixth and eighth nerve through medial longitudinal fibres. The nuclear mass is supplied by posterior cerebral artery, its branches. For ease of description, course of all nerves that supply extra ocular muscles have been divided into : 1. Fascicular, 2. Basilar, 3. Intra cavernous, and 4. Orbital parts. This helps to understand the relation of various structures that come on the way of the nerves. The lesions of these structures produce definite clinical features that help to pin-point the exact location of the lesion and many a times the nature of the lesion. 1. The fascicular part. The fibres of the third nerve travel anteriorly in the substance of the mid brain to pass through the red nucleus and the medial aspects of the cerebral peduncle. The fascicles join to form the two trunk of the third nerve on either side of the mid line and emerge in the inter peduncular space (fossa) in front of the mid brain. Involvement of fascicles at this level produces a symmetric lesions of the muscles supplied by individual nuclei. 2. The basilar part. As the oculomotor nerve travels forward in the inter peduncular space. The nerve passes through the sub arachnoid space, and passes under the posterior cerebral artery and above the superior cerebraller artery. The nerve passes parallel to the posterior communicating artery. The fourth nerve also passes in between the posterior cerebral and superior cerebellar artery, lateral to the third nerve. It is clear that in the inter peduncular space the nerve is not related to any nerve, tract or cranial nerve hence lesion in this area do not have any systemic neurological deficits only defect produced is in the ocular muscles and the lesions are mostly vascular. It is the commonest site for isolated third nerve involvement. 3. Inter cavernous part. At the anterior aspect of the inter peduncular space, the nerve passes between the posterior clinoid and the free edge of the tentorium to pierce the dura to enter the cavernous sinus. Initially it lies near the roof of cavernous sinus and then in the lateral wall of the sinus. The nerve throughout its course in the lateral wall of the cavernous sinus lies above the fourth nerve, ophthalmic and maxillary nerves. The fourth nerve crosses the third nerve to be superior and medial to the oculomotor nerve before the third nerve leaves the cavernous sinus to enter the orbit, however, the ophthalmic and maxillary division of the fifth nerve remain inferior to the third nerve through out its inter cavernous course. At the anterior end of the cavernous sinus, the third divides in to two division i.e. a smaller superior division that supplies the levator and the superior rectus and a larger inferior division that supplies the medial rectus, inferior rectus and inferior oblique. The branch to inferior oblique also carries the parasympathetic fibres to the ciliary ganglion. 4. Intra orbital part. The two divisions enter the orbit through the superior orbital fissure inside the circle of Zinn.

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The superior division passes over the optic nerve for some distance and pierces the superior rectus from its ocular surface. Some fibres travel superiorly to supply the levator palpebral from below. The inferior division enters the inferior rectus, medial rectus and inferior oblique from the ocular surfaces. The trochlear nerve15,16,17 Trochlear nerve is an unique cranial nerve. The special features of the nerve are : 1. It is the nerve with longest intra cranial course. 2. It is the thinnest cranial nerve. 3. It is the only cranial nerve that emerges from the brain on the dorsal side. 4. It is the only cranial nerve that decussates completely. 5. Its nucleus supplies single extra ocular muscle i.e. superior oblique on the contral lateral side. 6. Its fascicles are so small and so close to the nucleus that its not possible to differentiate between a nuclear and a fascicular lesion. 7. It enters the orbit through the superior orbital fissure outside the circle of Zinn. 8. It innervates the superior oblique from the orbital surface. The nucleus of the fourth nerve The fourth nerve has a single nucleus on the either side of the mid line just caudal to the third nerve nucleus in the grey matter of aqueduct at the level of inferior colliculus. The nucleus is so close to the third nucleus that it seems to be continuous with it. The nucleus of the fourth nerve innervates contra lateral superior oblique. Fascicular part The fibres of the fourth nerve bend round the aqueduct and decussate completely in the anterior medullary velum and leave the brain stem on the dorsal surface. Thus fourth nerve is the only cranial nerve that decussates fully and emerges on the dorsal side of the brain stem. The fascicles are so small and so near the fourth nerve nucleus that it is impossible to differentiate between nuclear and fascicular lesions. The lesions in the mid brain are best called fasciculo nuclear lesion. A small lesion dorsal to the aqueduct in the mid line produces bilateral fourth nerve palsy. The basilar part The fourth nerve curves round the brain stem from behind to have an anterior route to reach the superior oblique. In the basilar part its course is similar to that of third nerve. It passes between the posterior cerebral and superior cerebellar arteries lateral to third nerve away from the posterior communicating artery. Hence it is not involved in aneurysm of posterior communicating artery which is a common cause of isolated third nerve palsy. In the basilar part it does not come in relation to any other cranial nerve or nerve tract hence lesion at this level produce isolated fourth nerve palsy. The commonest cause of involve-

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ment of fourth nerve in the basilar part is head injury by which the nerve is pressed against the free edge of tentorium. The same traumatic force when directed posteriorly may cause bilateral fourth nerve palsy in the anterior medullary velum causing bilateral fourth nerve palsy. Cavernous part After leaving the basilar part the fourth nerve pierces the dura to enter the cavernous sinus lateral and inferior to the third nerve in the wall of the cavernous sinus. The fourth nerve changes its course upwards in the anterior part of the cavernous sinus and becomes superior to the third nerve to enter the orbit by the superior orbital fissure at a higher level than third and sixth nerve outside the circle of Zinn. The orbital part The fourth nerve supplies the belly of the superior oblique from its orbital surface. The third and sixth nerve enter the other extra ocular muscles from their bulbar surfaces. The abducent nerve (abducens nerve)16,18 The nucleus. The abducent or the sixth cranial nerve takes origin from its nucleus that is situated in the mid pons under the floor of the fourth ventricle. The fascicular part. The fascicles of the seventh nerve encircle the nucleus of the sixth nerve at this level making it impossible for an isolated sixth nerve palsy due to a nuclear lesion. The medial longitudinal fibres pass medial to the sixth nerve nucleus. There after the fibres of the sixth nerve pass through the substance of the pons with superior-olivarynucleus on the lateral and pyramidical tract on the medial side. Basilar part. Each abducent nerve comes out of the brain stem at the lower border of the pons at the ponto medullary junction one centimeter from the mid-line. The nerve goes up on the anterior face of the pons for a short distance only to be crossed by anterior inferior cerebellar artery. The sixth nerve pierces the dura 2 cm below the posterior clinoid process and passes above the inferior petrosal sinus beneath the Grubers (petro clinoid) ligament to enter the cavernous sinus. The cavernous part In the cavernous sinus unlike other cranial nerves that lie in the lateral wall of the sinus the sixth nerve lies in the substance of the cavernous sinus between the internal carotid and the wall of the sinus. The orbital part The sixth nerve enters the orbit through the superior orbital fissure within the circle of Zinn. It supplies only the ipsilateral lateral rectus from its orbital side. Clinical features of lesions at various levels of cranial nerves : The lesions can be divided into : 1. Nuclear 2. Infra nuclear

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(a) Fascicular (b) Basilar (c) Cavernous (d) Orbital Characteristics of oculomotor nerve palsy20,21 Nuclear. Nuclear lesions are rarer than lesions at other locations. A nuclear lesion should have following essential features : 1. Bilateral superior rectus palsy with bilateral ptosis. 2. Bilateral ptosis 3. Unilateral third nerve with contra lateral superior rectus and levator involvement. 4. Bilateral total third nerve palsy 5. Bilateral external ophthalmoplegia 6. Bilateral internal ophthalmoplegia. The cause of such selective involvement lies in the fact that : 1. Each superior rectus is innervated by contra lateral third nerve nucleus. 2. Both leavtors are supplied by one central caudal nucleus. 3. Large lesion may involve all the nuclei and cause bilateral third nerve palsy. Webino. Wall eyed bilateral inter nuclear ophthalmoplegia is associated with bilateral exotropia with absent convergence due to involvement of both medial rectus sub nuclei and medial longitudinal fascicular (not seen in children). Inter nuclear ophthalmoplegia22,23 is caused due to lesion of medial longitudinal fasciculus (MLF) that block the impulse from contra lateral para median pontine reticular formation (P.P.R.F.) to ipsilateral third nuclei. The lesion is in between the nucleus of third and fourth nerve. Its clinical features consist of - diminished adduction, adduction nystagmus, may have absent convergence (not seen in children). One and half syndrome22,23 There is a combination of horizontal gaze palsy on one side with inter nuclear ophthalmoplegia (INO) due to lesion of P.P.R.F. and M.L.F. in the pons. Horizontal gaze palsy constitute the One of the syndrome and INO is the remaining half, both together constituting one and half syndrome (Not seen in children). Fascicular lesion24 The fascicular lesions of third nerve are associated with involvement of either red nucleus, cortico spinal tract or superior cerebellar peduncle depending upon the position of the lesion. The various syndrome are : 1. Webers syndrome. Third nerve palsy on one side and contra lateral hemiplegia due to lesion involving third nerve fascicle. 2. Claudes syndrome. Ipsilateral third nerve palsy with contra lateral ataxia/tremor due to involvement of third nerve fascicle and the red nucleus.

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3. Benedicts syndrome. Third nerve palsy with contra lateral hemiplegia and tremors due to lesion involving third nerve fascicle, red nucleus and cortico spinal tract. 4. Nathnagels syndrome. Ipsilateral third nerve palsy, cerebellar ataxia on the same side due to involvement of third nerve fascicle and superior cerebellar peduncle. Basilar lesions The basilar parts of the third nerve is in close relation with three vessels without any other cranial nerve or tract in the vicinity. The basilar lesions are mostly vascular and isolated. The third nerve trunk passes between the posterior cerebral and superior cerebellar artery near the trunk of the basilar artery, hence the nerve may be compressed by aneurysm of these vessels or that of posterior communicating artery, which runs parallel to the third nerve trunk in the inter peduncular (basilar) part. These lesions generally are painful and involve the pupil. The basilar part of the third nerve is involved in uncal herniation at this level, which can be brought about due to an extra dural hematoma that presses the nerve trunk over the tentorial edge. It manifests as total third nerve palsy with fixed dilated pupil (Hutchinsons pupil). Traumatic lesion of third nerve Traumatic paralysis of third nerve is less common than that of fourth nerve. It is mostly seen in closed head injury. The common sites for involvement of third nerve in closed head injury is extends between its exit from the ponto medullary junction to its entry in the cavernous sinus. The nerve may be avulsed near the brain stem. It may be forced down over the tentorial edge, there may be hemorrhage in the nerve, or there may be contusion of the nerve during its passage in the inter peduncular space. Such lesions are generally associated with fracture base of skull, loss of consciousness, total third nerve palsy and Hutchinsons pupil. Cavernous sinus lesions In the cavernous sinus, the third nerve lies in the lateral wall of the sinus with fourth nerve and branches of the fifth nerve. The fourth nerve initially lies inferior to the third nerve but before it leaves the sinus it takes an upward course. The sixth nerve lies in the substance of the sinus. The oculosympathetic accompanies the internal carotid. Hence the lesion of the cavernous sinus does not produce isolated lesion of any single nerve, it generally involves all the nerves along with sympathetic. The lesions are generally partial. Involvement of fifth nerve is the cause of pain along with ophthalmoplegia. In some instances there may be loss of sensation on the distribution of fifth nerve. Generally pupil is of normal size. This is explained on the basis of two diagonally opposite mechanism acting simultaneously. The paralysis of third nerve should cause dilatation of pupil, which is counteracted by superimposed miosis of associated Horners syndrome. Aberrant regeneration of third nerve is common. Orbital lesions The third nerve divides into two parts just before entering the orbit, the upper supplies the levator, and superior rectus, the inferior supplies the inferior rectus and inferior oblique and the medial rectus. It also contains parasympathetic fibres. The orbital lesions are generally partial, associated with involvement of fourth and sixth nerve. Sixth nerve under action is

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more common than fourth nerve under action. It is generally associated with mild proptosis. Pupil may be involved in lesions of lower division. Painful ophthalmoplegia. Painful ophthalmoplegia may be confined to third nerve palsy or with multiple nerve palsies. The causes of painful third nerve palsy are : 1. Posterior communicating artery aneurysm. 2. Basilar artery aneurysm 3. Aneurysm of intra cavernous carotid 4. Diabetic oculomotor palsy 5. Ophthalmoplegic migraine Painful ophthalmoplegia involving more than one nerve can be due to any of the following : 1. Cavernous sinus thrombosis 2. Superior orbital fissure syndrome 3. Gradenigos syndrome 4. Pseudo tumours of the orbit 5. Metastatic tumours 6. Lymphoma 7. Naso pharyngeal carcinoma 8. Sinusitis 9. Para sellar growths Tolosa Hunt syndrome25 This is a chronic non specific granulomatous involvement of anterior cavernous sinus and/or superior orbital fissure in isolation or combination. This is also called superior orbital fissure syndrome or anterior cavernous sinus syndrome. A localised lesion may involve the apex of the orbit and is called as orbital apex syndrome. One of the important features of which is diminished vision due to involvement of optic nerve. The Tolosa Hunt syndrome may start as an acute episode of unilateral boring pain with diplopia and mild proptosis that may become chronic. Spontaneous recovery is known. Recurrence is common even with treatment. The muscle involvement is varied, may begin with involvement of one muscle only to be followed by involvement of other muscles. The ocular palsy is partial. Pupillary involvement is variable. It may be normal, dilated or constricted depending upon the part of the third nerve involved and its effect being counteracted by sympathetic system. As the first division of the fifth nerve is affected, there may be hypothesia or anaesthesia on the distribution of the fifth nerve. ESR is always raised. The condition shows improvement following administration of systemic steroids that has to be used for months. Most probable cause is an auto immune disturbance resulting in formation of non specific granuloma of the orbit or cavernous sinus. In adults it is generally unilateral but in children it may be bilateral.

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The diagnosis is by exclusion supported by x-ray, CT, MRI, USG and fine needle biopsy. Ophthalmoplegic migraine It is a frequent but missed cause of headache in children. It could be the beginning of migraine in adulthood. Generally there is history of migraine in the family. A typical episode comprises of pain in or around the eye on one side. The eye is non congested, vision is unaffected. There is associated nausea or actual vomiting. There is diplopia due to involvement of extra ocular muscles. Commonest group of muscles to be involved are those supplied by the third nerve. Other muscles may also be involved, the pupillary changes are not constant. The headache generally subsides with onset of ophthalmoplegia. Ophthalmoplegia generally subsides and clears with in one month, may not leave any residual sign or the recovery may be partial. Recurrence is common. There are no other neurological signs. The optic nerve is normal. Exact cause of the condition is not known. Most widely put forward cause is dilated edematous internal carotid pressing the third nerve. Cyclic oculomotor palsy26 This is an unilateral condition most probably of congenital origin, birth trauma may be a contributory factor. It may be noticed in early childhood. There are two phases : 1. A paralytic phase consisting of partial third nerve palsy resulting in ptosis, mydriasis, diminished accommodation, the eye is generally abducted. 2. Spastic phase. This begins with lid twitching, elevation of the lid, miosis and spasm of accommodation and the eye may be adducted. The cycles last for less than a minute. Exact cause is not known. Aberrant regeneration of third nerve27 The two cranial nerves that can develop aberrant degeneration (misdirection) are third and seventh nerve. (The seventh nerve aberrant regeneration results in crocodile tear and facial sweating on movement of the jaw.) The oculomotor aberrant regeneration follows injury to the third nerve trauma, aneurysm or neoplasia. There is abnormal sprouting of axons from the affected nerve that are misdirected to areas for which they are not destined. It takes three to four months for aberrant regeneration to take place. It is generally unilateral resulting into paradoxic movements. There are three possible variations of paradoxic movements : 1. Elevation of lid on attempted movement of inferior rectus and medial rectus (Pseudo Grafes sign). 2. Adduction or retraction of globe on attempted downwards or up gaze (Inverse Duanes syndrome). 3. Light near dissociation on attempted convergence (Pseudo Argyll Robertson pupil). There are two forms of aberrant regeneration of the third nerve : 1. Primary without previous history of acute oculomotor palsy. Third nerve weakness develops gradually along with aberrant regeneration. 2. Secondary. Always follows acute non ischeamic oculomotor palsy during recovery.

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The exact cause is not known. No medical treatment is known to be effective. Surgical intervention should be attempted after minimum six months of onset of aberrant regeneration that comprises of correction of ptosis and multiple muscle surgeries. Double elevator palsy28,29,30 This is a congenital anomaly of elevators of eye. It is a unilateral condition where elevation of the eye is subnormal in abduction or adduction. Most probable cause is hypoplasia of elevator nucleus. There is true or pseudo ptosis of affected eye. The eye is hypotropic in primary position. Bells phenomenon is present. Hypo deviation may be manifest or latent. Chin is elevated. Elevated chin with hypotropia means absence of amblyopia, which is otherwise common. There are three possible types of double elevator palsy 1. Prominent inferior rectus and normal superior rectus saccade 2. Weakness of elevation negative forced duction test, diminished superior rectus saccade. 3. Combination of both Differential diagnosis consist of 1. Browns syndrome 2. Congenital fibrosis of inferior rectus 3. General fibrosis syndrome 4. Blow out fracture 5. Dysthyroid oculopathy (not seen in children) 6. Anomalous superior and inferior rectus muscle insertion. Management Surgery is the only definitive treatment indicated in large vertical deviation or abnormal head posture. Surgery consists of recession of inferior rectus and transposition of lateral and medial recti to superior rectus. Double depressor palsy This is rarer than former. It is a congenital anomaly that has paralysis of inferior rectus and superior oblique in the same eye. In primary position the affected eye is hypertropic. Complete third nerve palsy The eye is abducted, slightly depressed and intorted. There is associated ptosis, there is mild proptosis. All the muscles supplied by this nerve show no movement or very little movement. The pupil is dilated not reacting to light and accommodation, the eye does not converge. Common causes of complete third nerve palsy in children are head injury, and raised intra cranial pressure. Paralysis of individual muscles supplied by third nerve. Superior rectus palsy Superior rectus palsy is invariably associated with paralysis of levator palpebral superior. Isolated superior rectus palsy is very rare. Common cause is congenital either in the muscle

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or in its nerve supply. In primary position the affected eye is hypotropic. The Bells phenomenon is absent. When the paretic eye fixes the sound eye becomes hypertropic due to over action of inferior oblique of the sound eye. The chin is elevated and head is tilted towards the sound side. Medial rectus palsy Isolated medial rectus palsy is very uncommon. The paretic eye is abducted due to unopposed action of lateral rectus on the same side. There is no change in position of chin or tilt of the head. Inferior rectus palsy Commonest cause of this rare palsy is congenital. Commonest acquired cause is fracture floor of the orbit. The depression of the involved eye is restricted along with the superior oblique of the contra lateral eye. The ipsilateral superior rectus is over active. The chin is depressed, the face is turned to the same side. Inferior oblique palsy The condition is rare. The affected eye is hypotropic in primary position. When fixation is shifted to sound eye, it becomes hypertropic. When the eyes are moved up and out, there is restriction of conjugate movement. Characteristics of fourth nerve palsy Fourth nerve under action is a common cause of vertical squint and compensatory head posture. It is however less common than third or sixth nerve palsy in children. In children two major causes of fourth nerve palsy are congenital anomaly and trauma. However infection, inflammation and neoplasm have their share towards fourth nerve palsy. Ischaemic lesion like diabetes, hypertension, arterio sclerosis which are major causes of trochlear nerve palsy in adults are not seen in children. Congenital fourth nerve palsy It may not be obvious unless it is severe, which presents as hypertropia in primary position. In moderate form the child may not complain of diplopia but may have compensatory head posture which gradually disappears when the palsy become concomitant, which is common. Hence old photographs may show abnormal head posture i.e. head tilted to the opposite shoulder. The anomaly may be seen in other members of the family. An old family photograph may show abnormal head posture in other members of the family as well. The child may read with one eye closed to avoid diplopia. Facial asymmetry is common, the antagonist may show contracture, difference between primary and secondary deviation is absent due to spread of comitance. There may be suppression in the affected eye that nullifies diplopia. Other neurological defects are generally not seen. Effect of congenital palsy : 1. The palsy may be constant at birth and remain so. 2. The squint is small and the child may control it without compensatory head posture. 3. The squint manifests in particular gaze with binocular vision. 4. It may develop latent squint.

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5. It may develop intermittent squint. 6. Intermittent squint becomes constant Unilateral fourth nerve palsy This is more common than bilateral. It can be congenital or acquired. It presents as ipsilateral hypertropia in primary position that increases when the eye is moved medially and increases more when the head is tilted on the same side (Park-Bielschowskys test). The above features are true for recent fourth nerve palsy where the child may complain of vertical diplopia and parent notice abnormal head posture. With passage of time there is spread of comitance and diplopia is minimised or abolished due to suppression. Associated exotropia is best measured by double Maddox rod test or Bagolinies striated glass 32. It can be demonstrated on synoptophore with torsion slides. Exccyclotropia due to uniocular superior oblique palsy is about 7. The child may tolerate up to 4 of excyclotropia. Occasionally the contra lateral eye may show hypotropia when the child fixes with the paralysed eye. Acquired superior oblique palsy should be differentiated from congenital palsy because an acquired palsy may be associated with other neurological findings. Second important point to remember is that the superior oblique may be absent in congenital palsy, which becomes obvious only during surgery. Bilateral fourth nerve palsy Recent unilateral superior oblique palsy is less difficult to diagnose than bilateral palsy. Trauma is a major cause of bilateral palsy than congenital anomaly which is otherwise an equally important cause of bilateral superior oblique palsy. Asymmetry in signs is common in bilateral palsy. In symmetrical bilateral case, there is either no vertical deviation or very little vertical deviation in primary position. The child may be orthophoric or show mild exo or eso deviation. The esotropic eye generally shows V pattern. There is left hypertropia on right gaze and right hypertropia on left gaze. Excyclotropia may be as much as 25-30. It increases in down gaze. Diplopia is present in all position of gaze except up gaze. Bielschowsky head tilt test is positive on both sides. Occasionally the cases may present as unilateral palsy, may develop highly asymmetric palsy in the other eye. Both the contra lateral synergists i.e. both inferior recti show over action. Asymmetric palsy develops ocular torticolis more often than symmetric palsy. Neurological characteristics of fourth nerve palsy Traditionally all cranial nerve palsies have been put into nuclear, fascicular, basilar, cavernous and orbital types except the fourth nerve because 1. The fourth nerve nucleus and its fascicles are so close to each other making it impossible to differentiate between nuclear and fascicular vision.

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2. There are no structure in the viscinity that may cause other neurological signs as seen with third and sixth nerve. 3. Only structure that passes through the mid brain adjacent to fourth nerve fascicles is ocular sympathetic. The lesions are best put as nuclear-fascicular lesion.34 Nuclear-fascicular lesion produce contra lateral superior oblique palsy, may be associated with contra lateral Horners syndrome. A lesion in the anterior medullary velum will involve both the fourth nerves causing bilateral superior oblique palsy. In children commonest cause of which is head injury. The basilar part of the fourth nerve winds round the brain stem and passes between the posterior cerebral and superior cerebellar arteries lateral to the third nerve and then passes forward to enter the cavernous sinus. At this location it gets involved in head injury. The nerve is pressed against the tentorium and damaged causing palsy of superior oblique on the same side without any other localising sign. Cavernous sinus lesion The fourth nerve is generally involved along with third and sixth nerve in various combinations along with fifth and oculo sympathetic in the cavernous sinus. Orbital lesionsOrbital lesions can be of two types : 1. Involving the nerve along with third and sixth nerve. 2. Injury to the muscle itself, or trochlea of the muscle. Such injuries involves the orbital rim in the superio medial aspect. Causes of fourth nerve palsy in children In adults the cause of fourth nerve palsy are mostly vascular. They produce ischaemia, bleeding or compression by aneurysm. Other cause is neoplasm. In contrast to this, the causes in children are rarely vascular. They are mostly congenital, traumatic, infective. In one third cases no cause can be found out. Other causes of vertical squint Vertical squints are less common than horizontal squint. Small degree vertical squint may be missed due to development of compensatory head posture. However in later life there may be decompensation and the squint may manifest with diplopia and obvious deviation. There are some conditions that may look vertical deviation when actual deviation is absent. The binocular vision is intact. These are called pseudo vertical squints. The causes of pseudo vertical squints are : 1. Asymmetry of orbit. 2. Cranio facial dysostosis 3. Orbital growth pushing the globe vertically. 5. Ectopic macula 6. Vertical angle kappa. The true vertical squint can be latent or manifest. It can be concomitant or paralytic. In paralytic vertical squint, spread of comitance is common.

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Causes of non comitant vertical squint These are due to involvement of cyclovertical muscles. Involvement of muscle may be restricted to one muscle in one eye, to involvement of multiple muscles in both eyes in various combinations. The non concomitant vertical squint can be neurological or restrictive. The neurological causes may be : Supra nuclear Nuclear Infra nuclear The supra nuclear lesions are :

Vertical gaze palsy Skew deviation Double elevator palsy Parinauds syndrome

The nuclear and infra-nuclear lesion have been discussed in lesions of third, fourth and sixth nerve. The other lesions are :

Dissociated vertical deviation Brown syndrome Inferior oblique over action Superior oblique over action Inferior rectus muscle paresis Superior oblique myokymia

The restrictive causes of vertical squint are : 1. Fracture floor of the orbit. 2. Myasthenia gravis 3. Dysthyroid ophthalmopathy 4. General fibrosis syndrome 5. Progressive external ophthalmoplegia Vertical gaze palsy35 The vertical gaze movements are under bilateral cerebral and brain stem centres. Transient tonic vertical deviation downwards is seen in neonates which does not indicate any neurological defect and does not require investigation. Another vertical gaze palsy seen in children is setting sun sign in infantile hydrocephalus. This is associated with retraction of the lid. The condition is serious but reversible and is treated by ventricular decompression by standard neurosurgical procedure. The condition consists of inability to move the eye vertically up or down. There are three types of vertical gaze palsies :

614 1. The most common, inability in up gaze 2. Difficulty in both up and down gaze 3. Least common difficulty in down gaze.

PEDIATRIC OPHTHALMOLOGY

The condition may be associated with light near dissociation, rigid pupil correctopia, dyscoria, spasm of accommodation, lid retraction or ptosis, convergence palsy, vertical nystagmus. Common causes of vertical gaze palsy in children are : Stenosis of aqueduct, hydrocephalus. Tumors of the third ventricle, mid brain and pineal body.35 Skew deviation36,37 In this supra nuclear defect, there is divergence of the eyes in vertical plane which is large. It may vary in different position of gaze. The deviation is more in uniocular lesion. There is no restriction on forced duction test. No single muscle could be pin pointed as cause of skew deviation on Park Bielschowsky three step procedure. The condition may be associated with inter nuclear ophthalmoplegia. Commonest cause is a posterior fossa lesion that is confirmed by CT and MRI. Other causes are vestibular or cerebellar. It may develop in congenital esotropia for which no cause is detectable. The pathology is generally ipsilateral to the involved eye. Parinauds syndrome This is an acquired lesion. It is bilateral, there is paresis of up gaze. In an attempt to look up, the eyes converge. The pupils are semi dilated, there is light near dissociation. There may be lid retraction on down gaze. This is seen more commonly in children with setting sun sign of hydrocephalus. Other common cause is tumor of pineal body. Dissociated vertical deviation (DVD)42 Dissociated vertical deviation is a neuro-muscular anomaly of obscure nature. It is known by many other names also. Common among them are : dissociated hyper deviation, alternate sursum-duction, alternate hyper phoria.38 As the last term indicates, it is basically a latent squint that manifests under cover, however, in some instances when the child is tired or day dreaming, one of the eyes may deviate upwards. It is a binocular disorder with variable asymmetry in two eyes. The difference may be so much that one eye seems to divert all the time and the other looks normal. It can be an isolated feature without any squint. Its association with strabismus is more frequent than without. When it presents as manifest deviation, it can be intermittent or constant. It can be seen with any types of squint but commonest squint associated with dissociated vertical deviation is infantile esotropia. It is more common with non accommodative esotropia. It is rare if squint is acquired after eighteen months39. In case of infantile squint, it takes about two years for DVD to set in. It is seen in about 40-50% of cases of infantile esotropia. Another group of children who develop DVD are those who have undergone successful surgical correction for infantile esotropia. In such cases the anomaly develops two years following initial surgery. It is better if the parents are informed about its possibility at the time of first surgery.

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The other common features are : 1. Development of manifest nystagmus that may become latent. 2. It is possible to have binocular single vision with DVD. 3. Extorsion of the deviated eye. 4. The eye intorts as the child looks down. 5. Cover test. The eye under cover deviates slowly up. The drift is more on prolonged cover. It is more for distant than near. Once the cover is removed, the eye gradually comes down and may over shoot the normal position and become hypotropic. 6. Measurement of deviation is made by using base down prism in front of the deviating eye. The occluder is shifted to the other eye, which starts deviating and base down prism is added to measure the deviation. 7. The Bielschowsky phenomenon. Besides DVD, the other condition where an eye deviates upwards under cover is alternation hyper phoria. The two conditions are differentiated by positive Bielschowsky phenomenon or Dark wedge test. Any of the eyes is occuluded by a translucent occluder (Spielmann occluder) through which the movement of the eye can be seen behind the occluder. The other eye fixes a spot light at distance. A Sbiza bar is placed before the fixing eye and density of the filter is gradually increased. The eye under Spielmann occluder is noticed to drift down and pass below the mid line if DVD is present. Now the filter density is gradually reduced and the eye under cover is seen to elevate once again. This phenomenon is absent in alternating hypertropia. Sbiza bar (Bagolini filter). This instrument consist of a series of red filters of the density (photo metric neutral density filter). The end has lightest red colour, the other end has darkest colour. The instrument is generally used to measure depth of suppression in amblyopia for which the test is done at one third of a meter in contrast to Bielschiwsky phenomenon where the test is done at six metres. The bar is placed before the fixing eye in increasing density until diplopia develops. The strength of the density gives the depth of suppression40,41. 8. DVD may be associated with A and V pattern. 9. There may be associated oblique muscle over action. Differential diagnosis consists of all conditions that cause upward shift of eye in primary position. They are : 1. Alternate hyper phoria 2. Inferior oblique over action 3. Superior oblique over action 4. Inferior rectus palsy 5. Skew deviation Treatment Treatment is correction of error of refraction and amblyopia when present. Surgery is indicated if vertical deviation occurs spontaneously so frequently that it causes cosmetic blemish. The alternatives available are :

616 I. Weakening of superior rectus 1. Recession of superior rectus 7-8 mm 2. Retro equatorial myopexy (Faden operation). II. Strengthening of inferior rectus (resection). III. Total anterio positioning of inferior oblique. Inferior oblique over action

PEDIATRIC OPHTHALMOLOGY

Inferior oblique over action is common in infantile esotropia where it develops between one to six years of age. However it may develop in exotropia less commonly. Generally there is no hypertropia in primary gaze. Hyper deviation is best seen in adduction. Secondary hypertropia is seen in under action of antagonist superior oblique. There may be pseudo paresis of contra lateral superior rectus. Associated V phenomenon is common. Latent nystagmus is frequent. Negative Bielschowsky phenomenon differentiates it from DVD. Cause may be mechanical or innervational. Treatment consists of weakening of inferior oblique either by recession or myectomy. Superior oblique over action Superior oblique over action can be primary or secondary. It can be unilateral or bilateral. In bilateral cases it can be asymmetric enough to seem to be unilateral. All bilateral cases are primary. In unilateral cases, a vertical deviation is common in primary position. The side of over action causes hypotropia. It may be associated with A pattern. In case of bilateral over action of superior oblique bilateral, weakening of SO is indicated. Inferior rectus muscles palsy Palsy of inferior rectus is rare. It can be congenital due to mal-development of the muscle or its faulty insertion. Trauma is more common. Commonest type of injury is fracture floor of the orbit with or without entrapment of the muscle. The muscle may be injured at the time of original injury or may be accidentally injured during repair of fracture floor of the orbit. If the muscle is not entrapped, the eye become hypertropic in primary position. In entrapment of the muscle the eyeball may fail to elevate and hypotropia results. It is commonly associated with reduced down and out movement due to inter action of contra lateral superior oblique. The compensatory head posture comprises of face turned towards the paretic side, chin depressed and head tilted on the same side. Treatment consists of strengthening the muscle by resection of involved muscle and recession of ipsilateral superior rectus. Brown syndrome (superior oblique sheath syndrome) : Brown syndrome is one of the common conditions of abnormal head posture in children. It is mostly congenital but can be acquired, it may be intermittent or constant. It is bilateral in 10% of cases. Congenital Brown syndrome may spontaneously resolve over years. Post inflammatory Browns syndrome also resolve with treatment. Probable congenital causes of Brown syndrome are : 1. Short anterior sheath of superior oblique. 2. Inelastic superior oblique tendon

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3. Defective innervation of both the obliques. 4. Thickened muscle not moving freely over the trochlea. 5. Anomalous insertion of superior oblique. The acquired causes are 1. Trauma. Trauma, accidental or surgical is the commonest acquired cause of Brown syndrome. The traumas that result in the syndrome are - direct trauma to the trochlea either blunt or penetration. There may be post traumatic scar formation round the trochlea, hematoma or blood cyst formation in the muscle. Orbital floor fracture with entrapment of inferior oblique muscle. The surgical traumas responsible areexcessive tucking of superior oblique, ptosis correction, injury to superior oblique or entrapment during scleral buckling. The next common cause is rheumatoid conditions involving the trochlea. Frontal sinusitis and frontal osteoma may also cause the disorder. The presenting symptoms and signs consists of In mild cases, the disorders may go unnoticed as there is no deviation in primary position. Otherwise sometimes parent notice abnormal head posture and defective eye movements. Vision may be diminished and amblyopia is common if squint is present. The chin is slightly elevated, the head is tilted towards the affected side and face turned away from the affected side. On cover test mild to moderate latent hypo deviation may be noticed. The affected eye fails to elevate is adduction. There may be down shoot of the eye in adduction and there may be widening of the inter palpebral fissure in attempted adduction. Elevation capacity increases as the eye is abducted. There is over action of contra lateral synergist. AV phenomenon is common when squint is manifest. There is positive forced duction test. Browns syndrome has been put in three grades i.e. mild, moderate and severe. In mild form, there is no hypotropia in primary gaze, there is no down shoot on adduction. Moderate form also does not show hypotropia in primary gaze but down shoot is demonstrated on adduction. Severe form has hypotropia in primary gaze, down shoot in adduction and abnormal head posture. The mild and moderate forms go unnoticed as there is no manifest squint, minimal abnormal head posture. They constitute about sixty percent of all cases. Binocular single vision is present in primary position and lower field. Management Any error of refraction is treated following refraction under cycloplegia. Amblyopia when present should be treated vigorously.

618 Prisms help only in small degree deviations.

PEDIATRIC OPHTHALMOLOGY

Steroids may be needed if there is element of rheumatoid disease. Surgery is rarely indicated as many show - spontaneous improvement, no manifest strabismus and minimum head tilt. Surgery is indicated in Diplopia in primary position, Marked abnormal head posture, Decompensation of squint Surgery is not very rewarding as it rarely corrects the ocular rotation. Surgery consist of stripping of superior oblique tendon sheath, removal of adhesion round the trochlea and recession of contra lateral superior rectus. Superior oblique myokymia This is an ocular dyskinesia not seen in children. The exact cause is not known, there is periodic vertical and torsional oscillopsia each lasting for few minutes. There is no definite treatment for the condition. Characteristics of abducent nerve palsy42,43 The sixth nerve supplies only lateral rectus on ipsilateral side. The lateral rectus is involved in one horizontal movement i.e. abduction. The sixth nerve has a long intra cranial course second only to fourth nerve. Lesions extending from its nucleus to its end in the muscle may involve lateral rectus. There are two types of lesions of sixth nerve i.e. those (1) That have other neurological signs in pons and (2) Those that cause isolated sixth nerve palsy. Isolated sixth nerve palsies are non specific and non localising. An isolated sixth nerve palsy is never nuclear. The signs and symptoms of isolated sixth nerve palsy produce : 1. Convergent squint. 2. Head turned towards the affected muscle. 3. No change in position of chin or tilting of head. 4. There is limitation of lateral movement. 5. Homonomous horizontal diplopia that becomes worse on looking towards the involved side. 6. Bilateral paralysis of sixth nerve produces diplopia in all gazes. 7. Paralysis of lateral rectus is frequent in children. It may be unilateral or bilateral. Common causes of sixth nerve palsy in children are Congenital Trauma Infection

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Inflammation Demyelination Vascular lesions like haemorrhage, aneursym, ischaemia are rare in children but are major cause of sixth nerve palsy in adults. Neoplasm of mid brain are also major causes of sixth nerve palsy in children. Majority of congenital cases are bilateral. The common congenital causes are : 1. Developmental anomaly of lateral rectus 2. Maternal infection 3. Birth trauma (common) 4. Hereditary. In new borns, a transient lateral rectus palsy due to viral infection is seen occasionally developing one to three weeks after non specific upper respiratory tract infection. Isolated sixth nerve palsy in children without neurological features of headache, vomiting, papilledema, ataxia is caused due to : 1. 2. 3. 4. Middle ear infection Post viral status Sinusitis Raised intra cranial pressure

Topographic location of sixth nerve lesion Nuclear lesion An isolated sixth nerve palsy can never be nuclear. A nuclear lesion is associated with horizontal gaze palsy towards the side of the lesion due to involvement of pontine paramedian reticular formation. Involvement of seventh nerve loop round the sixth nerve nucleus causes ipsilateral facial palsy. Involvement of medial longitudinal fascicles cause ipsilateral inter nuclear ophthalmoplegia. Involvement of oculo sympathetic will cause ipsilateral Horners syndrome. Fascicular lesion (Brain stem lesion or anterior inferior cerebellar artery syndrome) : This can cause three types of lesions : 1. Dorsal pons lesionMillard Gubler syndrome 2. Dorso lateral pons lesionFovilles syndrome 3. Combination of the twoRaymonds syndrome. Millard Gubellers syndrome consists of 1. Sixth nerve palsy 2. Ipsilateral seventh nerve palsy. 3. Contra lateral hemi paresis due to involvement of pyramidal tract. Fovilles syndrome consists of : 1. Horizontal conjugate gaze palsy due to involvement of P.P.R.F. 2. Ipsilateral fifth, seventh and eighth nerve palsy 3. Ipsilateral Horners syndrome.

620 Raymonds syndrome consists of : 1. Ipsilateral sixth nerve palsy 2. Contra lateral hemiparesis.

PEDIATRIC OPHTHALMOLOGY

The above lesions are generally seen in elderly persons with micro vascular lesion of diabetes, hypertension, athero sclerosis and not in children. Moreover such patients are seriously ill and first seen by neuro physician. Basillar lesion The three common causes of sixth nerve palsy in children at this level are : 1. Raised intra cranial pressure 2. Gradenigo syndrome 3. Trauma Other causes like acoustic neuroma and nasopharyngeal tumors are seen in elders. 1. Raised intra cranial pressure. Raised intra cranial pressure pushes the brain stem down pulling the sixth nerve on each side. It stretches the sixth nerve in the Dorellos canal over the apex of the petrous bone. This produces bilateral sixth nerve palsy without any localising sign. The causes are posterior fossa tumor and pseudo tumor ceribri. Gradenigos syndrome This is a common cause of unilateral sixth nerve palsy in children in developing countries. This is secondary to otitis media which leads to infection of petrous bone and extra dural abscess formation that comes in close proximity of the sixth nerve. The syndrome consists of sixth nerve palsy, facial palsy, diminished hearing, facial pain in the distribution of first division of fifth nerve. Battles sign. This consists of bilateral sixth and seventh nerve palsy, bleeding or leak of CSF from external ear and ecchymosis over the mastoid. The cause of the condition is closed head injury. The skull is compressed in horizontal diameter, there is transverse fracture of the temporal bone or base of the skull. Differential diagnosis of sixth nerve palsy Differential diagnosis of sixth nerve palsy consists of many unrelated non neurological conditions that produce more or less degree of mechanical restriction of lateral rectus. The conditions can be congenital or acquired. They are : 1. Mobius syndrome 2. Duanes retraction syndrome 3. Fractured medial wall of the orbit 4. Strabismus fixus 5. General fibrosis syndrome 6. Alternate day esotropia 7. Conjunctival shortening syndrome 8. Spasm of near reflex

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9. Break in fusion of congenital esophoria 10. Infantile esotropia 11. Nystagmus blockage syndrome Besides these myasthenia and thyroid myopathy are two systemic conditions that may masquerade any motor palsy. Mobius syndrome44,45 This is a multi systemic congenital anomaly where bilateral sixth nerve palsy with bilateral seventh nerve palsy are the two constant features. The Bells phenomenon is retained. The eyes can not be moved in horizontal direction, both duction and version are equally affected. Vertical movements are retained. There are no pupillary changes. No vestibular nystagmus can be demonstrated by caloric or rotatory tests. Occasionally there may be esotropia. Rarely there may be exotropia, A and V pattern is common. Involvement of other nerves from fifth to twelfth have been reported.45 Most probable cause is aplasia or hypoplasia of nuclei of various cranial nerves. Other associated deformities are - Deafness, deformed tongue, dental defects, skeletal defects in hands, feet. General hypotonia of muscles, especially pectoral muscle, variable mental retardation. Management Associated error of refraction and amblyopia when present should be treated by standard method. Lateral tarsorrhaphy may be required in presence of seventh nerve palsy. Results of squint surgery are not very good. Maximum recession of medial rectus may be done. Duanes retraction syndrome Duanes retraction syndrome is a common motility disorders seen in children. The condition is congenital and sporadic. Though it is present at birth, it is generally not diagnosed before three years of age. The child is generally brought for squint or abnormal movements of the eye and lids. It is generally unilateral but can be bilateral. When unilateral the left eye is affected more often than right. It is more common in girls. The essential features are : Narrowing of the inter palpebral fissure on adduction. Widening of IPA on abduction. Retraction of globe on adduction. Upshoot of the eyeball on adduction. The eye may be orthophoric, esotropic or exotropic. A-V pattern is common. There are many classification of the condition based on clinical presentation and electro myographic findings. Clinically there are three types : 1. Narrowing of IPA and retraction of globe with limited abduction, this is the commonest clinical type.

622

PEDIATRIC OPHTHALMOLOGY

2. Other things remaining same as in type I, there is limited adduction. 3. Both adduction and abduction are limited. This is the rarest type. The exact cause of Duanes retraction syndrome is not known. Many congenital anomalies have been blamed for the condition alone or in combination. They are : 1. Mechanical, 2. Neural The mechanical factors are : 1. Fibrotic, thin, non elastic lateral rectus 2. Relatively posterior insertion of medial rectus 3. Tight medial rectus acting as band 4. Abnormal attachment of lateral rectus 5. Muscles bound to the orbital wall 6. Abnormal attachment of medial rectus at its origin near the orbital apex 7. Fibrosis or atrophy of lateral rectus The neural anomalies can be : 1. Absent sixth nerve nucleus 2. Poorly formed abducent trunk 3. The lateral rectus is partially supplied by third nerve 4. The lateral rectus is supplied by third nerve 5. Simultaneous contraction of medial and lateral rectus in an attempt to adduct with retraction of globe The other classification is based on electro physiological changes in extra ocular muscle48 1. Paradoxical innervation of lateral rectus getting maximum impulses on adduction and diminished impulse abduction. This is the commonest type of Duanes retraction syndrome. 2. Lateral rectus gets maximum impulse on abduction with normal medial rectus. 3. Innervation of both lateral or medial rectus in primary as well as adduction or abduction. Duanes retraction syndrome may be just an ocular anomaly which is very common. Involvement of other systems are not rare. The ocular changes commonly seen with Duanes retraction syndrome are microphthalmos, lid defects, crocodile tear, heterochromia of iris, lenticular opacities, dermoids, coloboma of choroid. The auricular changes include - changes in external ear, external auditory meatus and defects in vestibular canals. The skeletal system changes consists of changes in palate and vertebrae, hand and feet. The neural changes - mal-development of third, fourth and sixth nerve.

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Variants of Duanes retraction syndrome47 1. The retraction of globe is so minimal that the condition is called Duanes retraction syndrome without retraction. 2. Similarly there may be very little narrowing of palpebral aperture. 3. There may be retraction of upper lid. 4. There may be hypotropia or hypertropia in primary position. The term pseudo retraction syndrome is used to denote entrapment of medial rectus in fracture of medial orbital wall. There is retraction of globe in attempted abduction. Management Management consist of correction of any error of refraction when present, prevention and treatment of amblyopia. Some children have no visible change in primary position and maintain good binocularity with slight change in head posture. Surgery There are many types of surgeries performed to treat the condition. Each case seems to be requiring separate method. Multiple bilateral surgeries are required to strengthen the lateral rectus. Faden procedure may be used to correct abnormal head posture. This prevents up and down shoot with stabilising effect on horizontal muscles. Commonest type of surgery advocated is bilateral medial rectus recession. Strabismus fixus This is a congenital condition of large bilateral esotropia due to anchoring of extra ocular muscles. The medial rectus is inelastic and replaced by a fibrous band, which is shorter than usual length of medial rectus. The muscle is inserted in a larger than normal area. Occasionally the lateral rectus and vertical muscles may also be affected. The eye is fixed in adduction. Forced duction test is positive. In an attempt to adduct, the globe retracts. General fibrosis syndrome is more severe form of strabismus fixus. All the extra ocular muscles are fibrosed. There may be adhesion between Tenons capsule and the globe. Alternate day esotropia (Intermittent cyclic esotropia) This is an unusual form of squint that has rhythmic cycle of esotropia for twenty four hours followed by orthophoria for next twenty four hours. Making the squint to manifest every alternate day, such rhythm cycle may last for six months to one year, by then the esotropia becomes constant. Treatment is required when esotropia becomes constant and most commonly practiced surgery is bimedical recession. Conjunctival shortening syndrome This form of restrictive tropia is seen when conjunctiva is kept contracted for months as is seen in third nerve palsy, sixth nerve palsy, long standing concomitant squint or following trauma, surgery where conjunctiva has not been recessed well at the time of muscle surgery. The condition is also met with in thyroid eye disease. There is limited ocular movement, forced duction test is positive.

624 Progressive external ophthalmoplegia

PEDIATRIC OPHTHALMOLOGY

This is a rare chronic multi systemic disorder generally seen in second to fourth decade. Infantile and juvenile are also known.48 The exact cause of the disease is not known. It is said to be associated with micro chondrian mutation.49 Others consider it to be an abiotrophy. It is a bilateral progressive disease that beigns with bilateral ptosis which is generally equal on both sides, gradually other extra ocular muscles also get involved. There is no fix pattern of involvement of the muscles, however, down gaze is unaffected for long time. At the end all the muscles loose function and the eye has a fixed position. The orbularis may also be involved. The iris and ciliary body are spared. KearnsSayer syndrome is a triad of external ophthalmoplegia, retinal pigment degeneration and complete heart block. Ophthalmploegia plus generally develops in childhood, there is no family history, CSF protein is elevated. The children have short stature, may have subnormal intelligence and hypogonadism. The condition has to be differentiate from myasthenia, thyroid eye disease, orbital pseudo tumor, myotonic dystrophy, progressive supra nuclear palsy. There is no known specific treatment. Differential diagnosis of abducent palsy in children Diagnosis of recent lateral palsy is not difficult. However, occasionally the child with esotropia may fail to abduct the involved eye in conditions other than sixth nerve palsy i.e. infantile esotropia. In such situation of simulated sixth nerve palsy it is essential to find out if the lateral rectus in question is really paralytic. The best way to demonstrate action of lateral rectus in such conditions is to patch the straight eye for few hours. This will move the eye laterally in cases of pseudo paralysis of lateral rectus but not in paralysed lateral rectus. The only exception is entrapment of medial rectus in fracture of the medial wall of the orbit, which is confirmed by forced duction test. The child with lateral rectus palsy has his face turned towards paralysed muscle to avoid diplopia. The other advantage of face turning is increased temporal field on the side of the paralysed lateral rectus. The child with prolonged lateral rectus palsy may cross fix with the paralysed eye like in infantile esotropia. The other test is Dolls eye movement which is negative in lateral rectus palsy. Other conditions that simulate like lateral rectus palsy areDuannes retraction syndrome, Mobieus syndrome, congenital fibrosis syndrome, pseudo tumor orbit, myasthenia. Investigation generally done to clinch the diagnosis of lateral rectus areUltrasonography of the orbit, CT, MRI, in cases of suspected myasthenia Tensilon test is positive. Forced duction test helps in finding out entrapment of the muscle and fibrosis syndrome. Long term effect of extra ocular muscle palsy 1. Presentation of acute ocular palsy greatly differ from chronic palsies 2. Some cases may present with palsy of one particular muscle only to involve other muscles later. This happens frequently in thyroid eye disease and myasthenia.

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3. Following are the changes that are seen in other muscle/muscles when one muscle is involved : (a) Over action of ipsilateral antagonist (b) On long term, the ipsilateral antagonist may end up in contracture (c) Secondary inhibition of contra lateral antagonist (under action) (d) By the time secondary changes (sequel) take place, the other muscles get primarily involved. (e) Compensatory head posture - The child may overcome diplopia by keeping the head in abnormal position. (f) Suppression and amblyopia are common in children with manifest squint.

REFERENCES
1. Lyle K.T. and Wybar K.C. ; The extrinsic ocular muscles and related fascia in Lyle and Jacksons Practical Orthoptics in treatment of squint. First Indian edition. p-8 14, Jay Pee Brothers, New Delhi, 1994. 2. Rowe Fiona ; Extra ocular muscles anomaly and innervation in Clinical orthoptics. First edition. p-312, Blackwell Science Limited, London, 1997. 3. Rosenberg, M.A. ; Ocular motor system in Principle and practice of ophthalmology. Vol. III, edited by Peyman G.A., Sander D.R. and Goldberg M.F. First Indian edition. p-19511954, Jay Pee Brothers, New Delhi, 1987. 4. Virginia Carlson Hansen ; Extra ocular muscles anatomy and function in Ocular motility. First Indian edition. p-111, Jay Pee Brothers, New Delhi, 1989. 5. Datta H. ; Anatomy of extra ocular muscle and their fascia in Strabismus. First edition. p-10, Jay Pee Brothers, New Delhi, 2004. 6. Sharma, P. ; An overview of strabismology in strabismus simplified. First edition. p-18, Modern Publishers, New Delhi, 1989. 7. Glasser, J.S. ; Eye movements characteristics and recording techniques in Neurophthalmology. p-187198, Harper and Row, London, 1978. 8. Bajandas, F.J., Kline, L.B. ; Supra nuclear and inter nuclear gaze pathway in Neurophthalmology review manual. Third edition. p-4365, Jay Pee Brothers, New Delhi, 1989. 9. Duke Elder S. and Wybar, K.C. ; Extrinsic muscles of the eye in System of ophthalmology. Vol. II. First edition. p-414463, Henry Kimpton, London, 1961. 10. Nema, H.V. ; Extra ocular muscles in Anatomy of the eye and its adnexa. Second edition, p-7784, Jay Pee Brothers, New Delhi, 1991. 11. Banumathy, S.P. ; Muscle in orbit in Anatomy of the eye. Ist edition. p-2332, Arvind eye hospital, Madurai. 12. Duke Elder S. and Cook, C. ; Extrinsic ocular muscles in System of ophthalmology. Vol. III, part I, p-220230, Henry Kimpton, London, 1969.

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13. Nema, H.V. ; Development of the eye and its adnexa in Anatomy of the eye and its adnexa. Second edition. p-139142, Jay Pee Brothers, New Delhi, 1991. 14. Banumathy, S.P. ; Development of extrinsic muscles of orbit in Anatomy of the eye. First edition. p-167, Arvind eye hospital, Maduri. 15. Nema, H.V. ; The oculomotor nerve in Anatomy of the eye and its adnexa. Second edition. p-108112, Jay Pee Brothers, New Delhi, 1991. 16. Duke Elder S., Wybar K.C. ; Efferent ocular motor pathways in System of ophthalmology. Vol. II. p-701718, Henry Kimpton, London, 1961. 17. Banumathy, S.P. ; Trochlear nerve in Anatomy of the eye. p-115118, Arvind eye hospital, Maduri. 18. Banumathy, S.P. ; Abducent nerve in Anatomy of the eye. p-119123, Arvind eye hospital, Maduri. 19. Nema, H.V. ; The abducent nerve in Anatomy of the eye and its adnexa. Second edition. p-119121, Jay Pee Brothers, New Delhi, 1991. 20. Glasser, J.S. ; Oculomotor palsies in Neurophhalmology. p-255260, Harper and Row, London, 1978. 21. Bajandas, F.J., Kline L.B. ; Seven syndromes of third nerve in Neurophthalmology review manual. Third edition. p-8794, Jay Pee Brothers, New Delhi, 1989. 22. Bajandas, F.J. and Kline L.B. ; One and half syndrome in Neurophthalmology review manual. Third edition. p-6364, Jay Pee Brothers, New Delhi, 1989. 23. Rowe, Fiona ; Inter nuclear and supra nuclear disorders in Clinical orthoptics. First edition. p-238244, Blackwell Science Ltd., London, 1997. 24. Natchair, G. ; Syndrome of neurophthalmic significance in Neurophthalmology, a manual for post graduate students, p-27.127-6, Arvind eye hospital, Maduri. 25. Shult, WT ; Tolosa Hunt syndrome in Current ocular therapy. Fifth edition. p-223 225, Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000. 26. Bruin H.M. and Vanallen, M.W. ; Cyclic oculomotor paralysis. AJO 55 : 529, 1963. 27. Bajandas F.J. and Kline LB ; Aberrant regeneration of third nerve in Neurophthalmology review manual. Third edition, p-93-94, Jay Pee Brothers, New Delhi, 1985. 28. Sharma, P. ; Differential diagnosis of cyclo vertical strabismus in strabismus simplified. First edition. p-152153, Modern Publishers, New Delhi, 1999. 29. Janice A. Gault ; Miscellaneous ocular deviations in Ophthalmology secrets. First Indian edition. Edited by Vander J.F. and Gault J.A. p-193, Jay Pee Brothers, 1998. 30. Rohatgi J.N. ; Acquired paralytic squint in Squint basic and clinical aspect. First edition. p-197201, C.B.S. Publishers, New Delhi, 2003. 31. Gittinger J.W. ; Fourth nerve palsy in Manual of clinical problems in ophthalmology. First edition. Edited by Gittinger J.W. and Asdourian G.K. p-177178, Little Brown, Boston, 1998.

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32. Dutta H. ; Superior oblique palsy in Strabismus. First edition. p-104109, Jay Pee Brothers, New Delhi, 2004. 33. Lyle T.K. and Wybar KC : Paralytic strabismus in Lyle and Jacksons practical orthoptics. First Indian edition. p-528575, Jay Pee Brothers, New Delhi, 1994. 34. Bajandas F.J. and Kline L.B. ; Five syndromes of fourth nerve in Neurophthalmology review manual. First Indian edition, p-97105, Jay Pee Brothers, 1989. 35. Lois J. Martyn ; Vertical gaze palsy in Pediatric ophthalmology. Vol. II, Second edition, p-798800, Edited by Harly RD, WB Saunders Company, Philadelphia, 1983. 36. Branett J., Dieterich M. ; Skew deviation with ocular torsion. A Vestibular brain stem sign of topographic diagnostic value. Annals of neurology 33 : 528, 1993. 37. Keane J.R. ; ocular skew deviation. Arch. Neurology 32-185, 1975. 38. Lyle T.K. and Wybar KC ; Alternating hypertopia in Lyle and Jacksons practical orthoptics. First Indian edition, p-341343, Jay Pee Brothers, New Delhi, 1994. 39. Folk R. Eugene ; Alternating hyper deviation in Principle and practice of ophthalmology. Vol. III, Ist Indian edition, p-18581860, Edited by Peyman G.A., Sanders DR and Goldberg MR. Jay Pee Brothers, New Delhi, 1987. 40. Rowe Fiona ; Bielschowsky phenomenon (Dark wedge test) on Clinical orthoptics. First edition, p-68, Blackwell Science, London, 1997. 41. Jampolsky, A. ; Surgical leashes and reverse leash in Strabismus surgical management in symposium on strabismus. Transection of New Orleans Academy of Ophthalmology, Mosby, St. Louis, 1978. 42. Lois J. Martyn ; Abducence palsies in Pediatric ophthalmology. Vol. II, Second edition, p-795, Edited by Harley RD, WB Saunders Company, Philadelphia, 1983. 43. Glasser, J.S. ; Abducence palsies in Neurophthalmology. p-248253, Harper and Row, London, 1978. 44. Duke Elder S. ; Congenital oculo facial palsies in System of ophthalmology. Vol. III, part 2, p-10311032, Henry Kimpton, London, 1964. 45. Janice A. Glout ; Miscellaneous ocular deviation in Ophthalmology secrets. First Indian edition, p-194, Edited by Vander J.F. and Gault J.A. Jay Pee Brothers, New Delhi, 1998. 46. Huber A. ; Electro physiology of the retraction syndrome. B.J.O. 58:293, 1974. 47. Sharma P. ; Restrictive strabismus in Strabismus simplified. First edition, p-136 141, Modern Publishers, New Delhi, 1999. 48. Kerrison J.B. and Nancy Newman : Chronic progressive external ophthalmoplegia in Current ocular therapy. Fifth edition, p-208210, Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000. 49. Moraces C.T., DiMauros ; Mito chrondrial DNA deletion in Progressive external ophthalmoplegia. New Eng. Jr. Med. 320, 1293-1299, 1989.

CHAPTER

18

Nystagmus1, 3
Nystagmus is a clinical sign. It is not a diagnosis, is a disturbance of ocular posture caused due to long list of lesions that can be ocular or non ocular (central). The exact mode of development of nystagmus is not well understood. It is fully coordinated function with reciprocal innervation. In normal steady fixation, the eyes are motionless. The steady fixation is maintained by afferent path, efferent path and intracerebral components.1 Deficiency in any of them will result in involuntary movement of eyes. These involuntary movements are grouped as nystagmus. Ninety percent of nystagmuses are caused due to afferent defect in poor vision, rest are caused due to efferent defect in ocular motor disturbance.2 However there are occasions when nystagmus can be physiological that can be elicited by appropriate stimuli.3 Nystagmus can be congenital (infantile) or acquired. The latter can be physiological that lasts for short time, only during the body is exposed to appropriate stimuli or it can be pathological. Congenital nystagmus is always pathological. Some terminologies3 used in describing nystagmus areMorphology, plane, amplitude, rate, direction, and grade. Morphologically nystagmus can bependular nystagmus4, jerky nystagmus and mixed. Pendular nystagmus has oscillation of equal speed and amplitude on each side like a swing of a clock-pendulum. They are generally present in primary position, they can be seen in any plane i.e. horizontal, vertical or rotatory. Horizontal pendulum nystagmus is the commonest. It is generally seen with sensory deprivation causing diminished central vision5 due to congenital lesion i.e. macular hypoplasia, achromatopsia, Lebers congenital amaurosis, optic nerve hypoplasia or conditions acquired early in infancy like congenital and developmental cataract, high errors of refraction, corneal opacities. It is seen as hereditary pendular nystagmus that runs in the families without other ocular involvement and last for rest of life.3 It is also seen in spasmus nutans. Other causes of pendular nystagmus are miners nystagmus, acquired unilateral nystagmus, lesions of brain stem, and drug toxicity. The horizontal pendular nystagmus is a slow smooth to and fro movement that is reduced on convergence. It persists in dim light but disappears on closure of eyes. It is associated with diminished vision. 628

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Jerk nystagmus. This type of nystagmus is biphasic, it has a slow phase in one direction and a rapid phase on the opposite direction. Jerk nystagmus is customarily named after the direction of rapid phase. The slow phase is the fundamental phase, while the rapid phase is a compensatory phase.6 The slow phase is pursuit phase while the rapid phase is saccadic phase.7 Common causes of jerk nystagmus can be congenital or acquired. They areOpto kinetic nystagmus, end point nystagmus, vestibular nystagmus, defect in fixation, latent nystagmus, congenital jerk nystagmus, gaze paretic. Mixed nystagmus. This is a rarer than the previous two. In this condition there is pendular nystagmus in primary position and jerk nystagmus in deviated eyes. Null point (zone) in nystagmus is a point at which the oscillations are either minimal or absent. It is situated mid way between levo version and dextro version. The child tries to keep the eye in such a position that it is nearest to the primary gaze. If the null point falls outside the primary gaze, the child will change the head posture to maintain the gaze in a position that has least oscillation. Plane of nystagmus can be horizontal (commonest), vertical, torsional, oblique or mixed. Amplitude. This is a measurement of excrusion of the eyeball in degree during oscillation. It can be fine, medium or coarse. A fine amplitude has an excrusion between 5-15 degrees, excrusion more than 15 degrees is called coarse. Rate (frequency)8 of nystagmus gives the number of oscillation in a given time. It is roughly divided into rapid and slow. It is given in cycles per second or Hertz, slow is 1-2 Hz, while 5 or more Hz is called rapid or fast. Relation between amplitude and frequency. Generally the rate varies with amplitude, faster the rate, finer the amplitude and vice versa.3 Intensity of nystagmus is calculated by multiplying amplitude with frequency. Grades of nystagmus. Alexanders law states that amplitude of jerk nystagmus is largest in the gaze of the direction of fast component. On the basis of this, nystagmus has been put in to three grades3, 8 First degree. Nystagmus is present only in the direction of fast component. Second degree. Nystagmus present even in primary gaze. Third degree. Nystagmus is present in both the above two positions. Classification of nystagmus. Classification of nystagmus is one of the most difficult tasks, because there are about forty types of nystagmus and all of them do not fall in to a simple classification. Some of the useful classifications are : I. Congenital ; or Acquired II. As there is overlap of characteristics in many fields, the above classification is not very suitable clinically. The other classification can be 1. Ocular (sensory deprivation) or 2. Non ocular (motor imbalance).

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The term ocular nystagmus denotes that the cause is in the eye i.e. macular hypoplasia, macular scar, optic nerve hypoplasia, Leber congenital amaurosia, congenital cataract. While extra ocular nystagmus is caused byvestibular, brain stem, cerebellum or spinal cord disorder.9 Ocular nystagmus follows the rule of 2-4-64, which states : 1. If the child has poor vision before 2 years of age, he will always develop nystagmus. 2. Between 2 to 6 years some children will develop nystagmus if central vision is lost. Some will not develop nystagmus. 3. Ocular nystagmus does not develop after 6 years of age with loss of central vision. Some of the nystagmuses are called physiological. They can be produced artificially by appropriate stimuli, these last only during the presence of the stimulus. They are : 1. Optokinetic nystagmus 2. Rail road nystagmus 3. Evoked vestibular nystagmus 4. Voluntary nystagmus. The remaining types are put under category of pathological nystagmus. They are sub-divided into : 1. Ocular type of fixation nystagmus 2. Neurological type of fixation nystagmus The former group includes : 1. Congenital jerk nystagmus 2. Hereditary pendular nystagmus 3. Latent nystagmus 4. Latent (manifest latent) nystagmus6 5. Pendular nystagmus of subnormal vision. 6. Spasmus nutans 7. Miners nystagmus 8. Acquired unilateral nystagmus The second group consists of : 1. Nystagmus of neurologic origin (brain lesions i.e. chiasma, posterior fossa, brain stem). They are : (i) Fixational nystagmus (ii) See-saw nystagmus. 2. Defects in gaze mechanism (i) Gaze paretic nystagmus (ii) Toxic nystagmus (iii) Inter nuclear ophthalmoplegia

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3. Nystagmus of vestibular defect (i) Nystagmus of peripheral vestibular disorder (ii) Nystagmus of central vestibular disorder. A comprehensive classification given by Lyle and Wybar9 is as follows : 1. Ocular fixation nystagmus (a) Physiological (i) Deviational nytagmus (ii) Optokinetic nystagmus (iii) Latent nystagmus (b) Pathological (i) Blindness (ii) Defect in central vision (iii) Spasmus nutans (iv) Miners nystagmus 2. Labyrinthine and vestibular nystagmus (i) Labyrinthine (ii) Lesions of vestibular nerve 3. Nystagmus of central origin (i) Brain stem lesion (ii) Cerebellar lesion (iii) Spinal lesion 4. Congenital idiopathic 5. Voluntary and hysterical Symptoms of Nystagmus 1. The child is unaware of nystagmus especially if it is congenital or acquired in early childhood. The parent may observe presence of nystagmus and seek help. There may be history of nystagmus in the family, without any other sign of ocular involvement. 2. Diminished central visionThe vision is poor, there is no correlation between degree of error of refraction and degree of nystagmus. However there may be associated errors of refraction. It is the diminished central vision that is the cause of nystagmus and not the other way. 3. A child may keep the objects very near to see clearly. This induces convergence which in turn reduces nystagmus. 4. Changed head postureIn order to use the null zone, the child may assume abnormal head turn. In vertical nystagmus the child may have abnormal chin position, there may be head tilt.

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5. Nystagmic child need not be blind. There should be some vision to develop nystagmus.10 6. SquintVarious types of squint are possible with nystagmus. 7. Other common symptoms consists of photophobia, glare, albinism, achromatopsia. 8. Neurological nystagmus may have symptoms of central nervous involvement i.e. tinitus, hearing loss, vertigo, hemianopia, see-saw nystagmus, head nodding, torticolis. Diagnosis. Diagnosis of nystagmus is not difficult, large amplitude nystagmus are observed by parents. Important factor in diagnosis is to find out if nystagmus is congenital, acquired in infancy or has developed after six years. Nystagmus developing after six years is of central, vestibular or toxic origin. Fine nystagmus may require examination either by slit lamp or by direct ophthalmoscope. Various Types of Nystagmus Physiological nystagmus 1. Optokinetic (opticokinetic)11 nystagmus. This happens when a person gazes at a succession of objects moving past in the field of gaze in one direction. The eye follows one particular object slowly to the limit of comfortable conjugate gaze. After this the object of interest disappears from the field of gaze, the pursuit is given up and the eyes move rapidly to the opposite direction to fix another object of interest and repeat the same. In quick succession resulting in to a jerk nystagmus. The commonest example of this is rail road or train nystagmus. Where a person looking out picks up a part of floating scenario and follows involuntarily till it disappears from the field of gaze and then fixes next object of interest. The fixing movement is towards the direction of the train. The person is not aware of the nystagmus but a person sitting opposite can see the nystagmus. The same effect can be produced under specific condition by moving an optico kinetic drum (Catford drum) which has alternating white and black strips of same width. The contrast between the white and black strips should be the same all through. When the drum is moved in front of the child, the eyes develop nystagmus, similar to rail road nystagmus and observed by the examiner. Presence of nystagmus means presence of vision, in a child suspected to be blind, absence of opto kinetic nystagmus is diagnostic confirmation of blindness unless the child has serious central nervous system defect including mental deficiency. The other use of opto kinetic drum is to detect malingering as well. Opto kinetic drum rotated vertically will produce vertical nystagmus. 2. Vestibular evoked nystagmus. This is a jerk nystagmus that can be produced by various methods. Commonly used methods are : 1. Displacement of the endolymph in the semi circular canal. 2. Acceleration and deacceleration of the body. If cold water is injected in the right ear, a jerk nystagmus will be produced on the left side and irrigation of the ear by warm water will produce nystagmus on the same side, the mnemonic is COWS i.e. cold opposite warm same side.

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Pure horizontal nystagmus can be produced by rotating the upright body with head flexed at 30. A vertical nystagmus can be produced by rotating the upright body in same manner with head tilted towards either shoulder. 3. End point nystagmus. This is ill sustained jerk nystagmus that develops on extreme lateral gaze, when the object of interest is outside the binocular field of vision. It does not occur in vertical gaze. It is more marked in abduction. The nystagmus consists of ten to fifteen beats and occurs on the side of the gaze. 4. Voluntary nystagmus. Some persons can induce pendular nystagmus at will for very brief period, best during convergence. Children learn sooner than adults. Many number of the family may have the skill to produce voluntary nystagmus. Pathological nystagmus : 1. Latent nystagmus. When both the eyes are open and straight, there is no nystagmus as soon as any of the eyes is covered both the eyes go for jerk nystagmus towards the uncovered eye. The vision in the uncovered eye also diminishes. It is a congenital condition, bilateral, mostly seen in esotropia and in hypertropia. If both eyes are covered the nystagmus disappears. The condition lasts for whole life9. If the weaker of the two eyes is occluded the nystagmus is finer, the nystagmus is also reduced and vision improved if the eye under observation is adducted. A nystagmus is termed manifest latent nystagmus when there is manifest nystagmus that becomes more severe if the dominant eye is covered.8 2. Manifest nystagmus. This is that type of nystagmus that is present when both eyes are uncovered and does not increase following covering of either eye. It is bilateral, conjugate mostly horizontal. It may have a mixture of both pendular and jerk nystagmus. These children have better near vision. The nystagmus is reduced on convergence. Generally there is abnormal head posture to bring the eyes in null zone. It disappears during sleep. 3. Nystagmus blockage syndrome. It is common for infantile esotrope children to have either manifest nystagmus or manifest latent nystagmus. On occasions on adduction, the nystagmus is reduced and vision improves. Nystagmus is otherwise present when the eyes are straight. This is called nystagmus blockage syndrome. The nystagmus in primary gaze is horizontal and increases on abduction. They may have cross fixation. They try to see either by converging both eyes or changing the head posture towards the adducted eye. 4. Miners nystagmus. Obviously this is not seen in children. This develops in miners after years of work underground in dim illumination. The nystagmus is mostly horizontal but can be oblique as well. It is mostly seen in up gaze. 5. Spasmus nutans3, 12, 13. Spasmus nutans consists a triad of nystagmus, head nodding and troticollis in infants. Its cause is unknown. It is a benign condition, does not denote any disease, lasts for only a few months and disappears leaving no untoward affect. However sometimes intracranial lesion may produce signs similar to spasmus nutans. The nystagmus is pendular, very fine, rapid. It is horizontal in plane, the nystagmus varies in different gazes. It is asymmetric. The head nodding is not uniform and irregular in rhythm, may be horizontal, vertical or both. Torticollis begins with nystagmus and passes off with it. The condition does not require any treatment.

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6. Hereditary pendular nystagmus. It is a congenital nystagmus that may be seen in other members of the family. It lasts for life, horizontal in nature that remains horizontal even on up gaze. However the nystagmus may become jerk nystagmus on lateral gaze. 7. Nystagmus secondary to diminished vision. Clinical presentation is similar to the hereditary pendular nystagmus of congenital origin. The difference is that vision becomes grossly defective in first year of life. The common causes that result in this condition are congenital optic atrophy, macular lesion, achromatopsia, congenital or developmental cataract, high error of refraction. There is no family history, the condition follows the genetic pattern of the primary cause of defective vision. 8. Congenital jerk nystagmus. There is horizontal jerk nystagmus, which is coarse. The vision is poor but best in null zone. There is abnormal head posture to improve vision. Some neurological conditions that produce nystagmus
Disorders 1. 2. 3. 4. 5. 6. Posterior fossa disease Demyelination of mid brain Chiasmal lesion Gaze paresis Inter nuclear ophthalmoplegia Vestibular lesion Type of nystagmus Acquired fixtaional nystagmus Acquired fixtaional nystagmus. Not common in children. See saw nystagmus Jerk nystagmus, jerk opposite to side of lesion Jerk nystagmus on lateral gaze Horizontal rotary jerk, may be of constant direction in peripheral lesion. In central lesion, the direction may change, may become vertical on up gaze.

See Saw nystagmus. In contrast to nystagmus described earlier, which were conjugate, this is a disjunct nystagmus. In this as one eye elevates and the other eye depresses. It is often associated with chiasmal lesion producing chiasmal type of field defect. Down beat nystagmus13. This consists of downwards jerking movements on downwards gaze but can happen in any head posture. Nystagmus increases in amplitude with gaze down and out. It is found in lesions of spinomedullary junction of congenital origin. Upbeat nystagmus. It may be of congenital origin, drug induced or posterior fossa lesion. In primary position the fast component is upwards. Management of nystagmus. Management of nystagmus is most frustrating both for the ophthalmologist and the child. Poor vision of nystagmus rarely responds well with optical correction. Medical treatment for oscillation of the eye has limited role in selected cases. Surgical intervention may correct associated squint and reduce oscillation to some extent which in turn increases foveation time and increased distant vision.

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Various optical options available are : 1. Prescription of glasses after cycloplegic refraction. 2. Adding concave glasses to distant correction, induces artificial accommodation that is accompanied with secondary convergence. This induced convergence diminishes amplitude and rate of nystagmus thus enhancing vision. 3. Similarly prism may be given to strengthen convergence. 4. Contact lenses have been reported to have reduced amplitude and frequency. Medical treatment. Many drugs have been used to treat nystagmus. They are - Baclofen, gabapentin, clonazepam, valproate, and carbamazepine.2 Retrobulbar injection of botulin toxin A (Botox) has been found to abolish nystagmus for short period. Surgical treatment consists of Anderson or Kesten baum procedures. The principle of which is to move the eye to the null zone. Recession of all the recti have also been advocated.

REFERENCES
1. Lyle T.K. and Wybar K.C. ; Nystagmus in Practical orthoptics in the treatment of squint. First Indian edition. p-592600, Jay Pee Brothers, New Delhi, 1994. 2. Wheeler D.T. ; Nystagmus in Current ocular therapy. Fifth edition. p-407410, Edited by Fraunfelder F.T. and Roy H.F. WB Saunders Company, Philadelphia, 2000. 3. Martyn Lois J. ; Nustagmus in Pediatric ophthalmology. Vol. II. Second edition. p-800807, Edited by Harley R.D. WB Saunders Company, Philadelphia, 1983. 4. Kanski J.J. ; Nystagmus in Clinical ophthalmology. Second edition. p-475477, Butter worth, London, 1989. 5. Deborah Pavan Langston. ; Nystagmus in Manual of ocular diagnosis and therapy. Third edition. p-323325, Lippincot Williams and Wilkins, Philadelphia. 6. Rohatgi J.N. ; Nystagmus in Squint basic and clinical aspect. First edition. p-211 218, CBS Publishers and Distributors, New Delhi, 2003 7. Seal S.K. ; Nystagmus in G.N. Seals Text Book of ophthalmology. Fifth edition. p-404405, Current Book International, Kolkata, 2002. 8. Sharma P. ; Nystagmus in Strabismus simplified. First edition. p-155163, Modern Publishers, New Delhi, 1999. 9. Lyle T.K. and Wybar K.C. ; Nystagmus in Practical orthoptics in the treatment of squint. First Indian edition. p-595, Jay Pee Brothers, New Delhi, 1994. 10. Reinecke R.D. ; Nystagmus in Ophthalmology secrets. First Indian edition. p-202 205, Edited by Vander J.F. and Gault J.A. Jay Pee Brothers, New Delhi, 1998.

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11. Rosenberg M.A. ; Nystagmus in Principles and practice of ophthalmology. Vol. III, p-19711974, Edited by Peyman G.A., Sanders D.R. and Goldberg M.F. First Indian edition. JayPee Brothers, New Delhi, 1987. 12. Glaser J.S. ; Nystagmus and related ocular oscillation in Neuro ophthalmology. p-221-236, Harper and Row Publishers, London, 1978. 13. Gittinger J.W. ; Down beat nystagmus in Manual of clinical problems in ophthalmology. First edition. p-182184, Edited by Gittenger J.W. and Asdourian G.K. Littlebrown and Co., Boston, 1998.

CHAPTER

19

Non Paralytic Squint in Children


Binocular vision1 Binocular single vision gives animals of higher order, a better view. Binocular single vision is a pre-requisite for larger field of vision, stereopsis and better visibility. For this both the eyes should have a well coordinated movement and a sharp image formed on the two foveae. The binocular single vision is not present at birth, it is an acquired faculty. It starts developing by six weeks of age. The child starts following a bright light for a short distance. This is the beginning of fixation reflex. The refixation reflex takes some more time to develop and develops by four to six months of age. By the age of six years, the fovea develops fully and the child has almost 6/6 vision in each eye with binocular single vision and stereopsis if the eyes are straight. Fixation reflex The two eyes are inter-related anatomically and physiologically. The physiological bond is enforced via various reflexes, fixation reflex is one of them.2 Fixation reflex is defined as an involuntary reflex which adjusts the eyes in such a way that the image of object of regard fall on the foveae at the same time and the image of moving objects are retained on the foveae. The re-fixation reflex brings the eyes to the original point of fixation. This could be active or passive. Fusion reflex is an involuntary reflex that is controlled by a fusional centre in the occipital cortex. This reflex adjusts the eyes in such a fashion that binocular single vision is produced and maintained. To have a single binocular vision, it is essential that the two images fall on corresponding points of the two retinae and fused as one. The retinal points that do not correspond are called disparate points. If an image is formed on disparate points, the eye will not have binocular single vision and will result in binocular diplopia.

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638 Horoptor

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Horoptor is an imaginary surface in space. All points lying on the horopter curve will stimulate corresponding retinal element and will be seen as one. For every retinal point in one eye, there is a limited area around the corresponding point in the other eye. Stimulation of this area along with original point can also result in binocular single vision. This limited area is called Panums fusional area. Objects in front and behind the Panums area cause diplopia. Projection is defined as interpretation of the position of an object in the space due to stimulation of retinal element. The foveal projection is straight ahead. A point in temporal retina is projected in the nasal field and vice-versa. Prerequisite of binocular single vision : 1. Reasonably clear media in both eyes. 2. Accurate co-ordination between the two eyes in all directions of gaze 3. Ability of the brain to cause fusion of two slightly different images. Grades of binocular vision are : 1. Simultaneous perception 2. Binocular fusion 3. Stereopsis Advantages of binocular single vision are : 1. Greatest advantage of binocular single vision is stereopsis or perception of depth. 2. Binocular field of vision is larger than uniocular field. 3. Due to overlapping of the uniocular fields, defect in one field is masked by the other field. 4. Binocular single vision is greater than uniocular vision. Lack of binocular single vision results in double vision.3 This has two components : 1. Confusion 2. Diplopia 1. Confusion. A patient without squint has a correspondence between the two foveae. The brain superimposes and fuses the two images provided they are similar enough. In case of a manifest squint fovea of fixing eye sees the object of regard while the squinting eye sees something other than the object of regard. The brain in an effort to superimpose these two dissimilar images create a state of confusion. This confusion is the result of corresponding retinal points being stimulated by two different objects. Confusion can not be explained well by the patient. It is very rare for a patient to describe two different images superimposed over one another. Confusion is soon replaced by binocular diplopia. 2. Diplopia is the result of one object stimulating non corresponding retinal points i.e. fovea of fixing eye and non foveal point in the deviating eye. Pathological binocular diplopia develops only in presence of manifest squint. Esotropia cause uncrossed (homonymous)

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diplopia while exotropia cause crossed (heteronymous) diplopia. Direction of diplopia depends on involvement of particular muscle or group of muscles. It is most marked in acute paralytic squint. Diplopia can be horizontal, vertical or torsional. Diplopia is generally associated with paralytic squint, though there is misalignment of eyes in concomitant squint as well, it is not associated with diplopia because of the following mechanism : 1. Anomalous retinal correspondence 2. Suppression 3. Amblyopia Anomalous retinal correspondence (abnormal retinal correspondence ARC)4 Normal retinal correspondence is a state where fovea of one eye corresponds with fovea of the fellow eye, nasal retina of one eye corresponds with temporal retina of the fellow eye, the two images are fused together to give a binocular single vision. Once normal retinal correspondence has been well established up to adulthood, it is not possible for ARC to develop hence ARC is seen in children. Abnormal retinal correspondence Abnormal retinal correspondence is an attempt to restore binocular single vision as far as possible. The binocular single vision may not be real but may resemble it. ARC is an adaptation in squint to avoid diplopia and confusion. It is not an anatomical change in the retina, it is a cortical adaptation. ARC may lead to formation of pseudo macula.2 It coexist with some suppression. Amblyopia may be present in ARC. It has various degrees of severity. ARC develops more commonly and is more severe when the child is small, with smaller degree of squint, longer the duration of squint more severe is the ARC. It is more common in infantile esotropia, less common is exotropia and least in vertical squint.1 Clinically there are two types of abnormal retinal correspondence : 1. Harmonious 2. Unharmonious In harmonious ARC, angle of anomaly is equal to angle of squint i.e. subjective angle of squint is zero. In unharmonious ARC, the subjective angle of squint is less than objective angle. Objective angle of squint is the actual measurement of deviation. This is measured on synoptophore. The patient sits on the synoptophore with chin on chin rest and head touching the head band. The inter pupillary distance is adjusted. The fixing eye fixes the slide in the tube. The non-fixing eye deviates. The examiner moves the tubes of the synoptophore so that the corneal reflexes produced by the illumination from the tubes are located in the centre of the cornea. The examiner than does alternate cover test by alternatingly switching off the lights in each tube and moving the tube until there is no movement in either eye. Angle between the two eyes gives the objective angle of deviation.

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In the other method the slide of lion is put in front of the fixing eye and the cage before the deviating eye, the tube in front of the fixing eye is set at zero. The other tube is moved by the examiner till the corneal reflex is brought to the centre of the deviating eye. At this point, the patient can see the lion and the cage simultaneously but can not superimpose. This gives the objective angle. To measure subjective angle the patient moves the tube to put the lion inside the cage and the corneal reflex is in the centre of the squinting eye. The difference between the objective and subjective angle is the angle of anomaly. In normal retinal correspondence (NRC) the two angles are equal. In harmonious ARC, the subjective angle is zero. Various methods available to diagnose ARC4 1. Bagolini glasses (striations) 2. Worth four dot test 3. Synoptophore 4. Titmus fly test 5. After image test 6. Vertical prism test 7. Binocular convergence test Abnormal retinal correspondence does not develop in all cases of squint always. The conditions where ARC may develop are : 1. Small angle of non paralytic esotropia 2. Infantile esotropia 3. Esotropia developing under three years of age 4. Congenital alternating esotropia 5. Residue in post operative status Conditions where ARC may not develop : 1. Concomitant divergent squint 2. Concomitant convergent squint after 15 years 3. Esotropia with large degree of deviation Management of ARC It is not always possible to treat ARC. It may be so mild that it is not worth the trouble to treat it. In early stages occlusion of the sound eye may help. In cases of alternate squint, alternate occlusion should be tried in children. Orthophoric treatment may be required. Suppression Suppression is an in-built central faculty that develops in young children with manifest squint. It is meant to ward off diplopia. It develops in the squinting eye that gets a blurred image. The brain learns to ignore the blurred image. It is a binocular feature, disappears if the fixing eye is closed. Suppression is mostly uniocular but may be alternating. Suppression is

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not limited to the fovea. There are larger areas where suppression is present. These areas are called suppression scotomas. They are seen both in esotropia as well as exotropia. Esotropic suppression scotomas extends nasally while the exotropic suppression scotoma spread towards temporal side. There are two types of suppression : 1. Facultative 2. Obligatory 1. Facultative suppression. It is that type of suppression that develops in the eye when it deviates. The moment the eye takes up fixation, the suppression disappears. This is met with in alternating deviation. 2. Obligatory suppression. In this, suppression is present even when the squinting eye takes up fixation. It is most commonly seen in monocular esotropia. In such cases, in initial stages the suppression is facultative which becomes obligatory later. Suppression is diagnosed by : 1. Worth four dot test 2. Red glass test 3. Bagolinis glasses Eccentric fixation This is a uniocular feature in which an object is fixed by a retinal point other than fovea without change in the principle visual direction. It develops in long standing esotropia and microtropia. There are various types of eccentric fixation according to location of the retinal point that acts as eccentric point as per findings of visuscope.5 Visuscope is a modified direct ophthalmoscope that has an in-built star which can be projected on the retina. The instrument is used as any other direct ophthalmoscope with dilated pupil. To do the test one eye is closed. The beam of the visuscope is focussed on the retina, this casts a shadow of the star on the retina. The patient is asked to fix the star. In normal eye, the star will be superimposed on the macula but not in eyes with eccentric fixation. As per position of the star, the eccentric fixation can be classified asPara foveal, para macular, para central, centrocecal. In erratic fixation, the star seems to jump from one place to another near the macula. Squint In normal person the visual axes of the two eyes are parallel in primary position and all direction of gaze except convergence and divergence, there too the angle of convergence or divergence of one eye is equal to that of the fellow eye. This ideal condition is called orthophoria. The position of rest in a normal person is slight divergence. Orthophoria like many physiological conditions is exception than rule. All such non orthophoric need not have manifest deviation. In contrast to this, a person may be orthophoric but looks to have squint due to anatomic configuration of the eye, lids or orbit. Such conditions are called pseudo squint.

642 Causes of pseudo squint are :

PEDIATRIC OPHTHALMOLOGY

1. Prominent epicanthic foldThis gives an appearance of convergent squint. 2. HypertelorismThis gives an appearance of divergent squint. 3. Facial asymmetry results in pseudo vertical squint. 4. Hypermetropia due to large positive angle kappa gives an appearance of apparent divergent squint. 5. Myopia due to large negative angle kappa gives an appearance of convergent squint. An apparent convergent squint worsens true convergent squint and neutralises divergence squint. Similarly an apparent divergent squint worsens appearance of divergence. In pseudo convergent squint, obliteration of epicanthic fold abolishes squint. In pseudo squint, the corneal light reflex is always in the centre of the cornea. The eyes do not move under cover test or alternate cover test. Pseudo convergent squint gradually disappears as the child grows and epicanthic fold fades away. But this is not true for hypertelorism that is a permanent feature. Pseudo squint due to errors of refraction may disappear with correction. It is a wrong notion that a true manifest squint will disappear as the child grows. In fact with age, the child adapts a compensatory head posture that may mask the squint to a casual observer. A high degree of fusion is required to keep the eyes in near orthophoric condition. A breakdown of fusion will cause one of the eyes to deviate. This is called strabismus or squint and is defined as a state when only one of the visual axes is directed towards the point of fixation and the other deviates away. There are two possibilities : In one condition, the fusion is maintained under strain and as soon as the fusion is broken, the eyes deviates only to return to normal position when the fusion is restored. This state is called heterophoria or latent squint. In the other type of squint, the fusion is already broken down or is absent resulting into a visible deviation of eye. This is called heterotropia. In between the two are the conditions that cause intermittent heterotropia. The deviation may not be equal for near and distances. It may vary during the day, or may be present from time to time. Classification of squint Varieties of squints are caused due to diverse causes with great variation in signs and symptoms making it impossible to have an uniform classification of squint. Most practical is to divide ocular deviation into : 1. Latent : (apparent) heterophoria 2. Manifest : heterotropia In case of manifest squint, it is essential to find out if the deviation is 1. Pseudo squint or 2. Real squint

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Pseudo squint does not require any specific treatment and does not develop sequel of manifest squint. Once it has been decided that the child really has a manifest squint, the next step is to decide if the squint is 1. Concomitant 2. Non comitant Non comitant is further divided into 1. Paralytic 2. Restrictive (obstructive) A flow chart given below is helpful
Eyes

Orthophoric (Rare) Latent

Non orthophoric

Intermittent

Manifest

Pseudo squint (Never latent) Concomitant

Real squint

Noncomitant

Paralytic

Restrictive

Primary change (Acute)

Secondary (Late)

Latent squint (Heterophoria) Heterophoria is that binocular state of ocular balance where the two eyes are kept in physiological alignment (orthophoric) for distance as well as near and in all direction of gaze so long the child is able to maintain binocular fusion. Once the fusion has been broken, any of the eyes will deviate only to come to near orthophoric position when the fusion has been restored. Fusion can be broken in following conditions : 1. One eye is occludedCover test 2. Dissimilar images fall on two foveaeMaddox rod test

644 3. The two images have different colourRed glass test

PEDIATRIC OPHTHALMOLOGY

4. Separate images are being projected on two foveaeMaddox wing test Etiology of heterophoria is not well understood. Some of the following conditions may cause heterophoria singly or in combination. 1. Uncorrected error of refraction : (a) Hypermetropia is more associated with esophoria than myopia. (b) Myopia is more associated with exophoria than hypermetropia. 2. Muscular anomaly : (a) Faulty origin (b) Faulty insertion (c) Poorly developed muscle 3. Abnormality of the orbit 4. Defective innervation of extra ocular muscles, muscles in each eye getting different tonic innervation. 5. Debilitating disease 6. Fatigue 7. Drugs, toxins, psychomatic disorder 8. AgeChildren are more likely to develop esophoria. 9. Heredity Types of heterophoria Heterophoria is not a manifest condition, neither the child nor the parents are aware of it so long the fusion is maintained. Its presence is elicited by cover test and its extent is evaluated by various tests. Maddox rod, Maddox wing, red glass test on Maddox tangent, prism test, synoptophore etc. As per direction of the eye when fusion is broken i.e. cover test. Heterophoria can be : 1. Esophoria. The eye deviates nasally under cover and move temporally to take up orthophoric position when cover is removed. 2. Exophoria. When the eye deviates out under cover and moves inward when cover is removed. 3. Hyper phoria/hypo phoria. One eye has a tendency to turn upwards in relation to the other and called hyper phoric. Logically there is a possibility that one eye can deviate down in relation to the other eye and should be termed as hypo phoria but in practice the term has been given up in favour of right hyperphoria or left hyper phoria. Here it is worth noting that esophorias and exophorias are not specified as right or left phoria. In horizontal phorias the amount of deviation in each eye is the same for a given distance but may change in relation to distant or near fixation.

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Cyclophoria This is the rarest form of phoria. In this there is tendency for one eye to wheel rotate in relation to the other. If the upper pole of the eye in question dips inwards, it is called incyclophoria and when it dips outward it is called excyclophoria. There is no condition when the two eyes deviate in different direction under cover. Diagnosis of heterophoria Cover test This simple test which does not require any special instrument denotes presence and type of heterophoria. The child is made to look at a distant object, than any of the eyes is covered and the other is made to fix. After a few seconds the occluder is removed and the movement of the eye is noted. Movement in any direction denotes presence of heterophoria. If the eye moves out to take up fixation means that the eye has moved in under cover, in a convergent position and the condition is called esophoria. If the eye moves in, it is called exophoria. If the eye moves down, it is hyperphoria. If the upper pole dips medially, it is incyclophoria, if the upper pole moves out it is excyclophoria. The test is repeated for near. It is better to do the test with correcting glasses also, if phoria is still present with correction, the exact number that abolishes deviation should be noted. If movement still persists with best correction, other method of treatment should be considered. Once the direction of deviation has been known by cover test, the next step is to find out the degree of deviation that can be done by : 1. Prism and cover test 2. Maddox rod test 3. Red glass test 4. Synoptophore test 5. Prism dissociation test 6. Maddox wing test 7. Double Maddox rod test 8. Maddox double prism Measurement of phoria for distance 1. Prism and cover test. Prism of increasing strength with apex towards the deviation is placed in front of one eye. The child fixes with the other eye. Cover uncover test is performed till all the movements in the eye under cover are abolished. This gives the amount of deviation in prism diopters. This test is equally useful in both heterophoria and heterotropia. 2. Maddox rod test. The child is made to sit in a semi darkened room 6 meters away and in front of the Maddox tangent. The Maddox rod is traditionally put in front of the right eye and position of the red streak noted on the scale, both horizontal and vertical. In orthophoria, the streak passes through the central light of the scale, otherwise it passes away from the

646

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central light, the distance of streak from the central light is directly read from the scale inscribed on the Maddox tangent. The test fails to differentiate between phoria and tropia. 3. Red glass test. This is similar to Maddox rod test. In this test, instead of a Maddox rod, a red glass is used. The procedure is the same as in Maddox rod test. If the vision is unequal, the glass is put in front of the better eye to prevent suppression of the poorer eye. The red glass should be dark enough so that the patient sees only central light of the Maddox scale. This test fails to differentiate between phoria and tropia. 4. Synoptophore test. Synoptophore is a versatile instrument that not only give degree of deviation but also can be used for cover test, alternate cover test, degree of deviation, measure range of fusion, suppression and retinal correspondence. It can also be used for orthoptic treatment for both phorias and tropias. 5. Prism dissociation test. This is less frequently used test in which a 6 Pd prism, base down is held before one eye and a rotary prism is held in front of the other eye. In presence of horizontal phoria, the patient will have both vertical and horizontal diplopia. The rotary prism is rotated to align the images horizontally one above the other. The power of the prism that causes horizontal alignment is the degree of heterophoria. Measurement of phoria for near Phorias for near can be measured roughly by cover test, prism and cover test, but the most accurate measurement is obtained by Maddox wing. It not only gives information about horizontal or vertical phoria but also measures cyclophoria for near. Detection and measurement of cyclophoria Careful observation during cover test gives a rough idea about cyclophoria. It is better measured by : 1. Maddox wing 2. Double Maddox rod, one usual red and other white 3. Maddox double prism Double Maddox rod test In this test a red Maddox rod and a white Maddox rod is put in front of each eye, in the trial frame with their axes at 90. This results into two horizontal lines one red and other white. The patient fixes a white bulb at six metres. If there is no cyclophoria, both the lines will be parallel to floor. If they are not parallel, the glass causing oblique image is moved to make the streak parallel. The axis of rotation gives the amount of cyclo deviation. Maddox double prism test Maddox double prism consists of two prism each 4 prism diopter in strength mounted on a lens ring in such a way that they are base to base with apex away. The double prism is put in the trial frame in front of one eye, the other eye is left uncovered without any lens or prism. The patient is given a white sheet of paper which has a line drawn in the middle. The patient keeps the paper at usual reading distance in such a way that the black line is horizontal. The two prisms in the trial lens separate the line away from each eye. Thus a patient without cyclophoria will see three parallel lines. The central line is the line seen by the eye without the

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prism. The other two lines one above and one below the central line are formed by the two prisms. If the central line is oblique to the two parallel lines, the patient has cyclophoria or cyclotropia. Symptoms of heterophoria6 Most of the time the child is not aware of heterophoria and has no symptoms. The child is generally able to overcome the deviation without much effort. The parent may notice the deviation occasionally when the child is either daydreaming or fatigued. So long the fusion is well maintained, the phoria is said to be compensated. The symptoms arise only in decompensated phorias. Factors that precipitate decompensation are : 1. Inadequate fusional reserve 2. General debility 3. Prolonged near work Horizontal phorias are least disturbing, symptoms are more in vertical phorias and most troublesome in cyclophoria. Common symptoms are : 1. Asthenopia and asthenopia related : (a) Burning (b) Redness (c) Itching (d) Stye, chalazion 2. Visual (a) Blurring of vision (b) Running of letters (c) Intermittent diplopia 3. OthersDizziness, vertigo, nausea Esophoria Here the eyes move in on dissociation. It is more common in children. Hypermetropic children have more tendency to develop esophoria than emmetropic or myopic. Type of esophoria 1. Convergence excess typeHere esophoria is more for near than for distance. 2. Divergence weaknessHere esophoria is greater for distance than for near. 3. In between the above two there is not much change in near or distant deviation. Management 1. First and foremost treatment is correction of any error of refraction under cycloplegia and prescription of glasses for constant use.

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2. Orthoptic treatment. The fusion is the factor that keeps the eyes in alignment in heterophoria. When the eye becomes decompensated, improvement of fusional divergence (relative negative convergence) is desired. They consist of (i) Divergence exercises on synoptophore (ii) Home stereogram exercise (iii) Bar reading in case of convergence excess. 3. Prism may sometimes reduce symptoms. Children using glasses accept prisms better than those who do not use spectacles. Fresnel prism may be pasted over the spectacle glasses for temporary use. 4. Surgery is seldom required. Exophoria Here the eyes diverge on dissociation. Types of exophoria 1. Convergence weaknessHere exophoria is greater for near than distance. 2. Divergence excessHere exophoria is greater for distance than near. 3. In between the twoThere is not much difference between near and distant deviation. Exophoria should be differentiated from intermittent exotropia. Exophoria rarely measures more than 20 prism dioptres while in case of intermittent exotropia the measurement is more. Intermittent exotropia develop suppression hence they do not develop diplopia and become manifest. Management In children treatment is required only if the eye has tendency to become exotropic. Here also first step is to correct any error of refraction under cycloplegia followed by orthoptic treatment. Hyperphoria This is a vertical phoria, less common than horizontal phorias. However a combination of vertical phoria associated with horizontal phoria is common. As vertical fusional amplitude is relatively less, asthenopic symptoms are more in vertical phoria. Even a deviation of 2-4 prism diopters produces sufficient symptoms. The treatment is prescription of prism with base down in case of hyperphoria and base up in case of hypophoria. Strength of the prism is equally divided in two eyes. It is better only to neutralize one third to half of deviation by prism7. Surgery is indicated in large deviation with paretic element. Dissociated vertical divergence (DVD) is also a type of hyper deviation and called double hyperphoria. Cyclophoria This is rarest form of latent squint with profound symptoms and is most missed type of phoria unless specifically looked for and eye is examined by Maddox wing, Maddox double rod,

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and Maddox double prism. In this case the eyes have tendency to rotate along anterio-posterior axis. Excyclophoria is more common than incyclophoria. It is generally associated with vertical phoria. Treatment is directed toward treatment of vertical phoria. Manifest squint (Heterotropia) Heterotropia is that state of muscle imbalance in which one of the eyes deviates in relation to the other eye. This can be : 1. Concomitant squint 2. Incominant (non comitant) squint (a) Paralytic squint (b) Restrictive (c) Kinetic8 Kinetic strabismus is a rare, often missed clinical condition, is due to irritative intracranial pathology resulting in unequal stimulation of nerves, nerve centres or muscles. Concomitant squint Characteristics of concomitant squint : 1. It is a visible diviation deviation. 2. The deviation is present most of the time. 3. The deviation may vary for distance and near. 4. The object is fixed by one eye at a time. 5. The fixation may alternate between two eyes. 6. The angle of squint remains constant in all directions of gaze. 7. The ocular movements are normal in all direction. 8. The squint may be mono-ocular or alternate. Etiology of concomitant squint are the same as in latent except that there is a permanent break down of the fusion. Any eye can have concomitant squint 1. As primary disorderThe deviation starts without being latent. 2. Latent squint getting so much decompensated that the eyes fail to revert to orthophoria. Phoria Intermittent tropia Constant tropia 3. Spread of comitance in case of congenital squint. Duane9 has divided squint into two basic types : Near dysfunctionMedial rectus dysfunction Distance dysfunction lateral rectus dysfunction. This is based on the fact that medial recti are responsible for near and convergence and lateral recti are responsible for distance and divergence. The etiological factors are : 1. CongenitalMal-origin or insertion of one or more extra ocular muscles. There may be under development of muscle/muscles.

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2. Defective vision in one eye due to any cause i.e. opacity in media, optic atrophy, macular lesion, ptosis, error of refraction. 3. Defective vision in both eyesMore in one than the other, the eye with less vision is more likely to squint. 4. Dissociation between accommodation and convergence - This is one of the most common causes of squint in children. (a) HypermetropiaIn moderate degree of hypermetropia, the eye accommodates more to see clearly. This stimulates convergence as well as produces convergent squint. (b) MyopiaAn acquired myopic eye does not require accommodation for clear vision hence there is reduced convergence and the eye deviates out. A child with congenital myopia has a very short far point, and can see only near object. To see near things he has to converge more than an emmetrope of same age, this results in convergent squint. 5. Deficient fusional power 6. Secondary change in paretic muscle A child born with paralysis of one of the muscles, may recover over a period of time, but its direct antagonist develops contracture and under action of muscle that was limited to one gaze only, spreading to all directions of gaze. 7. HeredityAbout 10% of squint have family history. Types of heterotropia Heterotropia can be unilateral (monocular) or alternate. Unilateral heterotropia is more common than alternate. A squint is said to be unilateral when one the eyes squints constantly and the other eye fixes. If the fixing eye is covered, the squinting eye takes up the fixation and the fixing eye deviates under cover. In both eyes the angle of squint is equal. As soon as the cover is removed the previously covered eye takes up fixation and the other eye squints. In alternate squint when the fixing eye is covered, the squinting eye deviates under cover and remains deviated when the cover is removed. The angle of deviation is equal in both eyes. The vision in both eyes is almost the same and loss of vision is not much. Generally both the eyes are emmetropic. Even if there is any error of refraction present, it is of low grade. Alternate esotropia is more common than exotropia. The alternating eyes lack all the grade of vision. According to direction of deviation, the eye can be esotropic, exotropic, hypertropic or rarely cyclotropic. Diagnosis of heterotropia Diagnosis of well established moderate manifest squint is simple. In fact the parents bring the child for complaints of squint. The child is generally unaware of squint. Once the child becomes aware especially the teenagers, they try to hide the cosmetic blemish keeping a downward gaze.

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1. Examination of distant vision should get preference over all other examination and whenever there is diminished vision in any of the two eyes, the cause of the diminished vision should be determined by refraction, examination of anterior segment and fundus examination. Diminished vision in the squinting is common but the other eye too may have diminished vision due to same error of refraction or different errors of refraction may exist between the two eyes. There may be difference in vision in two eyes due to other causes. 2. The next step is to find out if the squint is real or pseudo squint. 3. Once pseudo squint has been ruled out, the next step is to exclude possibility of paralytic squint.
Paralytic (incomitant) Age Onset Symptoms Diplopia Any Sudden (a) Diplopia is a major symptom. (b) Diplopia can be reduced by change in head posture. (c) Diplopia disappears with closure of any eye. (d) Diplopia is most marked in recent cases that passes off with time False projection Diminished vision The child can not locate the object No false projection in the space. Generally there is no reduction of vision that can be related to squint. Nausea, vertigo due to confusion Symptoms of primary cause may be present Other neurological deficit may be present Ocular movements are absent or restricted in the direction of action of paralysed muscle There is change in angle of squint in various direction of gazes. Secondary deviation is greater than primary deviation. The squinting eye may have diminished vision Not Present NIL NIL Movements are normal Any Usually gradual Generally there is no diplopia Concomitant

Systemic symptoms

1. 2. 3.

Movement

Angle of squint

1. 2.

Angle of squint is constant in all direction. Secondary and primary deviation are equal.

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Afferent path and nerve centres Efferent path ARC Amblyopia Cyclovertical deviation Intact Faulty Generally absent May not develop if the squint disappear

PEDIATRIC OPHTHALMOLOGY Faulty Normal Common Common Less common

More common

4. The above test leaves with only diagnosis i.e. concomitant squint that should be examined under following heads : Cover test This is most important but often neglected test. It differentiated between (I) Pseudo and true squint, (II) Uniocular and alternate squint and (III) Paralytic and non paralytic squint. It should be done for distance and near. Measurement of angle of squint (i) Hirschberg test (Corneal reflex test). The child keeps both eyes open and fixes a distant object with normal head posture. A bright pin-point light is directed on the squinting eye from 33 cm distance and position of the image of the light is noted. In a normal eye, it should be centred over the pupil. In squinting eye, the image is formed opposite the direction of squint i.e. if the eye is deviated medially the reflex will form on temporal side of the pupil. Each 1 mm decentering denotes 7 or 14 PD of squint. Besides manifest squint there are some causes of decentred corneal reflex. They are : 1. Angel kappaA positive angle kappa may look like an exodeviation and a negative angle kappa may look like esodeviation. 2. In case of eccentric fixation in an amblyopic eye the light reflex remains decentred upon covering the second eye. 3. Ectopic macula The corneal light reflex test is done for near also. (ii) Krimsky test (Prism reflex test). This is a better test than Hirshberg test, but Hirshberg test is only the first part of this test. In this test prism of increasing power are placed before the eye until the light reflex is centred in the deviating eye. The child should be able to fix a light with the dominant eye. It is not influenced by eccentric fixation. However there may be a confusion in presence of large angle kappa. The observer should place himself exactly in front of the child to avoid parallax from the prism. The test is suitable even in children with poor vision in one eye. (iii) Perimeter test. This is good only for grown up children. The child sits on a Listers perimeter with chin on the chin rest and fixes the central white dot. The other eye is allowed

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to deviate. A torch light is moved on the arc of the perimeter from periphery to the centre until the light reflex is centred on the deviating eye. The position of the torch is read on the arc of the perimeter. This gives the angle of squint in degree, which is half of prism dioptre. (iv) The synoptophore test. This is the most accurate method to measure angle of squint for distance and near in all direction of gaze, objective and subjective angle, angle of anomaly. The synoptophore also gives away presence of ARC and its type, grades of vision, measurement of range of fusion after image testing and AC/A. It can be used for therapeutic use also in : 1. Amblyopia treatment with Haidinger brush. 2. Management of suppression 3. Treatment of ARC 4. Fusional exercises In manifest squint, simultaneous macular perception is absent. There may be a suppression scotoma. The size and position of which varies according to type of squint. In esotropia, a small suppression scotoma is seen on nasal side of the disc, may involve macula. In exotropia, a large suppression scotoma is seen on temporal half of the retina including the fovea. In uniocular squint, the scotoma is constant in the squinting eye, may lead to strabismic amblyopia. In alternate squint the scotoma alternates between the two eyes. Binocular fusion may be absent in concomitant squint so is the stereopsis. The stereopsis can be tested on various types of stereoscopes. Test for suppression : (i) Worth four dot test (ii) Synoptophore (iii) Four dioptre prism test (iv) Bagolini striated glass test (v) Red glass test Test for abnormal retinal correspondence 1. Bagolini striated glass test 2. Synoptophore 3. After image test Horizontal concomitant squint Classically horizontal squints are divided in 1. Simple horizontal squint (a) Esotropia (b) Exotropia 2. Horizontal squint associated with vertical deviation (a) Esotropia with (i) Inferior oblique over action (ii) Dissociated vertical deviation (iii) AV pattern (iv) Superior rectus under action (b) Exotropia (i) AV pattern

654 Esotropia

PEDIATRIC OPHTHALMOLOGY

Out of many causes of esodeviation in children, esotropia is commonest. It could be congenital or acquired. It can be unilateral or alternate. Deviation may vary for distance and near. Other causes of esodeviation are10
Esodeviation

Esophoria

Esotroria

Pseudo esotropia

True esotropia

Congenital

Congenital esotropia

Congenital syndrome 1. Mobius 2. Duanes 3. Strabismus fixus 4. Congenital lateral rectus palsy

Acquired 1. Infantile esotropia 2. Accommodative esotropia 3. Non accommodative esotropia 4. Consecutive esotropia 5. Lateral rectus palsy 6. Entrapment of medial rectus 7. Microtropia 8. AV pattern 9. Cyclic esotropia 10. Uniocular loss of vision
11

Clinically esotropia has also been classified according to its relation with accommodation, on the basis of this esotropia can be
Esotropia

Non accommodative I. Essential (a) Infantile (Congenital) (b) Acquired (c) Late on set Essential non accommodative esotropia can be (i) Convergence excess (ii) Divergence deficiency (iii) Basic II. Acute concomitant III. Consecutive esotropia IV. Microtropia V. Cyclic esotropia

Partial accommodative

Accommodative

Refractive

Non refractive

More accommodation

Less accommodation

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Congenital (Essential infantile)12,13 esotropia This is an important type of esotropia seen under six months of age. The importance lies in the fact that it causes permanent loss of binocular vision if not treated in time and the next important factor is that many a times early onset retinoblastoma may present as esotropia in an infant. Hence all children with real esotropia should be examined by ophthalmologist. Characteristics of infantile esotropia14 1. It is rarely present at birth. It develops between two to six months. 2. It is not possible to predict which child will develop esotropia. 3. It can be hereditary. 4. It is uniocular, may show alternate fixation. 5. Angle of squint is always large i.e. 30-40 PD. 6. The deviation is equal for far and near. 7. The child has a tendency to cross fixate i.e. the child fixes the left field with right eye and right field with left eye. This sometimes gives an impression of bilateral lateral rectus palsy. In fact inspite of being non paralytic squint, adduction is stronger than abduction in infantile esotropia. Abduction can be demonstrated by : (i) The fixing eye is covered, an object of interest - a feeding bottle which is the most interesting object to an infant is moved in front of the squinting eye from medial to lateral. The chances are that the child will follow it to full abduction position. (ii) If this fails the fixing eye is occluded for few hours. The child may develop abduction or it may take two to three days of occlusion of the fixing eye. (iii) A feeding bottle is kept straight ahead of the child, both eyes are kept open. The child is allowed to fix it, then as the child fixes it, obviously with the fixing eye the head of the child is rotated quickly in the vertical axis i.e. to the right and then to the left. This will invariably demonstrate presence of abduction. (iv) Stimulating oculo vestibular reflex - The child is held by the examiner at an arms length, face to face. The examiner rotates himself by 360 holding the child in the same position. The child will be observed to have conjugate deviation. The rotation is than repeated in the opposite direction. 8. Dissociated vertical squint is common. It generally develops by two years. It is a bilateral condition. The eyes deviates up and rotates outward under cover. As soon as the cover is removed the eye drift down and rotated medially. DVD may develop long after successful surgery as well. 9. Inferior oblique over action - There is decrease of convergence in up gaze showing V pattern (This is absent in DVD). The condition is unilateral to begin with but may become bilateral. It is also a common feature after successful surgery and the parents should be informed and forewarned about this. Fortunately most of the parents fail to notice it. When present it requires a separate surgery. 10. Unilateral superior oblique paresis15 may be present. The incidence is less than inferior oblique over action and DVD.

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11. Nystagmus16 may be latent or manifest, resulting in abnormal head postureTo locate null point of nystagmus, to get better vision, convergence may be utilised to reduce nystagmus. 12. Vision is generally good. Amblyopia is common but may not develop in an alternator. 13. Refractive errorThe child has low degree of hypermetropia. Refraction should always be tested under atropine. 14. There is no neurological defect. Differential diagnosis Differential diagnosis consists of accommodative squint, unilateral abducent palsy, Duanes retraction syndrome, high AC/A ratio, nystagmus blocking syndrome, unilateral blind eye, incomplete Mobius syndrome, albinism, cerebral palsy. Variants 1. Congenital esotropia syndrome consists of esotropia with other muscle malfunction i.e. SO over action, DVD, V syndrome etc. 2. Ciancia syndrome consists of (a) Esotropia (b) Bilateral abduction defect 3. Manifest nystagmus that is latent. 4. Abnormal head posture 5. Langs syndrome. This consists of : (a) Torticollis (b) DVD (c) Nystagmus (d) Excyclodeviation (e) Early onset esotropia Management Management is essentially surgical. However before embarking upon surgery : 1. Any error of refraction should be corrected. Commonest error of refraction is low grade of hypermetropia. Refraction should always be done under atropine with usual precaution. If error of refraction turns out to be more than +3D, an accommodative factor should be considered. It is better to make the child slightly myopic. 2. Management of amblyopia by standard method. I. ConventionalFull time complete occlusion by eye pad of the fixing eye. The duration should be 1 day for every year between two years to six years. Upto two years 2 days of occlusion is done. II. Alternate occlusionOne day of occlusion of amblyopic eye i.e. in a three year child, the fixing eye is occluded for 3 days and on the fourth day the amblyopic eye is similarly occluded. In a four year child, it should be four to one and in a five year child, it should be five to one.

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Occlusion should be continued : (i) Until vision has been equalised in both eyes. (ii) Optimal vision for the eye has been achieved. Sometimes an eye has to be occluded for three to six months. (iii) There is no further improvement of vision in spite of sufficient occlusion. Frequency of follow up depends upon age of the child. Younger the child more frequent should be the follow up. 6 months 1 year 2 years Every 3 days Every week Every two weeks

The visual acuity should be checked on every visit. 3. Miotics. Some children may respond to local miotics. Miotics should be used only when some binocularity has been reached. However children with non accommodative esotropia respond poorly with miotic. Commonly used miotic is phospholine iodide 0.03% to 0.125% once a day. If surgery is required, the anesthetist should be told about use of miotic during pre anaesthetic check up. The miotics are stopped for two three weeks17 before general anaesthesia is administered especially if succinyl choline is to be used to intubate the child, otherwise other muscle relaxtants that do not depend upon body choline esterase may be used.18 4. Surgery is the definitive treatment of congenital esotropia. Sooner it is done better are the results, but vision should be improved, alternation should be achieved and amblyopia should be managed before surgery.19 Chances of development of DVD and inferior oblique over action after 2 years are positive hindrances. The parents should be told about possibility of binocular surgery and repeat surgery before planning the surgery. Basically there are two groups of surgeries for congenital esotropia : (1) Bilateral equal recession of medial rectus (2) Recession one medial rectus with re-section of lateral rectus on the same eye. 5. Botulinum toxin (Botox) injection in the medial rectus - The injection is given under general anaesthesia under electromyographic control in the medial rectus. A common needle used to inject botox also acts as electrode for EMG. Effect of injection starts within twenty four to forty eight hours and lasts for two to three weeks after which the injection may be repeated. The injected muscle is paralysed and is said to be elongated, while its antagonist contracts. Drawbacks are cost of injection, its unpredictability and systemic toxicity. It should be given only by a person especially trained. Other uses of botox in ophthalmology are : 1. Paralytic sqint (single muscle) injection is made in the direct antagonist 2. Cyclic esotropia 3. Thyroid ophthalmopathy 4. Ptosis

658 5. Essential blepharo spasm 6. Exotropia20 Acquired esotropia

PEDIATRIC OPHTHALMOLOGY

Acquired non paralytic esotropia is commonest type of esodeviation seen in children. They have been classified in different ways 1. On clinical presentation 2. Etiological basis The second seems to be better than the first. It divides acquired esotropia into (i) Primary (ii) Secondary and consecutive (They are far less in number) The primary or the common variety is divided into (a) Accommodative (b) Non accommodative (a) Accommodative esotropia can be fully accommodative with normal Ac/A or high Ac/A ratio. However many of the accommodational esotropias may be partially accommodative. (b) Non accommodative esotropia can be Basic type Convergence excess type Esotropia in myopia Microtropia Noorden21 1990 gave a comprehensive classification
Acquired esotropia

Accommodative

Non accommodative

Secondary

Consecutive

Fully accommodative

Partially accommodative

Uniocular loss Surgical over of vision in a child correction of divergent squint Convergence Myopic Excess

Basic

Accommodative esotropia Accommodative esotropia is one of the commonest forms of ocular deviation seen in children. Its diagnosis is simple and treatment is effective. This is a type of manifest squint that can be treated mostly by optical methods. However those who present late may have to undergo surgery and anti amblyopic treatment.

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Characteristics of fully accommodative esotropia 1. Age of onsetIt is seen between six month to six years. Average age of presentation being 2 years. 2. HeredityThe condition has strong hereditary background. It may be present in parents, near relatives or in siblings. 3. DeviationOcular deviation is (i) Uniocular (ii) Variable greater for near than distance (iii) IntermittencyThe child may have intermittent esotropia that may become constant. (iv) Angle of deviation is not very large, it ranges between 20PD to 45 PD. (v) The child does not alternate. (vi) There is no cross fixation. 4. Amblyopia is common but not deep. 5. Error of refraction is typically hypermetropic ranging between +4D and +7D. The squinting eye is more hypermetropic than the other. 6. AC/A is normal i.e. between 3D-5D. 7. Near distance relationship is either normal or when different it is not more than 10 PD. 8. The deviation is fully corrected by spectacles and the eyes remain straight, hence do not require surgery. 9. Binocularity is present due to anomalous retinal correspondence. 10. Children with higher hypermetropia more than 8D generally do not develop accommodative squint.22 They have bilateral amblyopia. Most probably they give up accommodation. These children do not improve with glasses.23 11. Spontaneous recovery with reduction of hypermetropia is possible, the condition may pass into partially accommodative or non accommodative squint.24 12. There may be associated DVD or inferior oblique over action. 13. Binocular single vision is possible for far and near when hypermetropia is fully corrected. Etiology of accommodative esotropia There are two factors that lead to accommodative esotropia i.e. 1. Uncorrected hypermetropia 2. Abnormally high Ac/A ratio. 1. Hypermetropia. An emmetropic child needs no accommodation for distance vision, a myopic eye becomes worse by accommodation. It is only hypermetropia that is relieved by accommodation. These children with accommodative esotropia generally have an uncorrected hypermetropia of moderate degree. The child uses excessive accommodation to overcome this

660

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hypermetropia. This by itself does not explain the complete mechanism. The second part of the mechanism is in sufficiency of fusional divergence. So long fusional divergence is sufficient, the child does not develop manifest squint. An occassional esophoria may result. This may lead to intermittent esotropia. Once the fusional divergence breaks down esotropia results. 2. Abnormally high Ac/A ratio. Normal Ac/A ratio is 3D-5D. It can be measured by following methods25 : 1. Heterophoria method 2. Gradient method 3. Graphic method. Management of accommodative esotropia Optical A. The first line of management is prescription of hypermetropic correction for constant use with correction of associated astigmatism. Glasses should be prescribed after refraction under 1% atropine only. Children under four years are given full cycloplegic correction. If a child older than four years is prescribed full cycloplegic correction, the child may not tolerate it. They are generally given as much plus lens possible that keeps the eyes straight and vision clear. The power of the plus lenses may be required to be reduced over years. B. Fully hypermetropic correction for distance generally keeps the eyes straight for distance as well as near. In some children there is some esodeviation for near. In such situation the first step is to see if the distant correction has been fully corrected or not. This should follow repeat refraction under atropine. If it is found that the child has been given full correction at the first instance, the second step is to give bifocals. The near power should be just sufficient to keep the near axis straight. The best bifocals for children are executive type where the junction of far and near correction pass across the pupil. Miotics The next alternative is use of miotic. All children do not respond equally well to miotic therapy. About 10% may not improve, some may show poorer result than bifocals. The beneficial effect of miotics should be weighed against the local and systemic side effects of miotics. Orthoptic treatment Orthoptic treatment is recommended in older children with hypermetropia less than +3D. Surgery Surgery is not required for horizontal deviation but DVD and inferior oblique over action requires surgical management. Accommodative esotropia with high Ac/A In case of fully accommodative esotropia Ac/A is within normal limits. There are some children who have high Ac/A. They have more esodeviation for near than distance. Some may

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not even squint for distance.26 They may have various types of error of refraction that range between low hypermetropia to mild myopia, even emmetropia in some cases. Chances of high Ac/A are more when cycloplegic refraction shows low hypermetropia, myopia or emmetropia. High hypermetropes have more or less normal Ac/A. The condition is not fully corrected by glasses, some esotropia is left uncorrected. Management consists of correction of any error of refraction and improvement of vision for distance. Adding plus lenses for near deviation either as bifocal or clipon near correction. While adding near correction minimal addition that corrects near deviation is prescribed. The bifocal should be executive type, the upper border of the near correction should divide the pupil in two equal parts horizontally. Non correction of near deviation by bifocals is strong indication of non accommodative factor that can be corrected by surgery only. Miotics can also be given for near esodeviation. Partial occlusion and orthoptic exercises help to overcome amblyopia and help increase amplitude of divergence fusion. Comparison between infantile esotropia and accommodative esotropia
Infantile esotropia Age of onset Angle of deviation Laterality Alteration Cross fixation Refraction Restriction of abduction Intermittency Deviation Vertical deviation Nystagmus Amblyopia Management Before six months 35 PD-50 PD Uniocular Very common Common Moderate Hypermetropia does not exceed +2D Common but no under action of lateral rectus Absent Equal for far and near DVD and inferior oblique over action common Very common Common, Alternators do not develop Surgery is the treatment of choice Accommodative esotropia Average 30 months Average 20 PD Uniocular Nil Nil +4D to +7D is common No restriction of abduction May be present as intermittent esotropia Esotropia slightly more for near DVD and IO over action may be present Absent May be present but not deep Glasses will correct esotropia for far and near

Partially accommodative esotropia Partially accommodative esotropia is a mixture of accommodative as well as non accommodative esotropia. There is always some esodeviation left after full spectacle correction of hypermetropia even with bifocals and/or miotics.

662 There are two possible explanation for the condition :

PEDIATRIC OPHTHALMOLOGY

1. The child was initially an infantile esotrope to which accommodative element has been added as the child ages. There may be increase in hypermetropia. Final hypermetropic refraction is more than initial hypermetropia. 2. The child had accommodative esotropia to which non accommodative element has been added due to decompensation. Generally there is hypertrophy of medial rectus or conjunctiva on the medial side. The residual esodeviation is constant, it is same for near and distance. It is associated with amblyopia. Treatment27 Treatment consists of surgery for esodeviation and continuation of glasses post operatively. Non accommodative acquired esotropias There are three types of these esotropias28 1. Basic type 2. Convergence excess 3. Esotropia in myopia 1. Basic type non accommodative acquired esotropias : Characteristics : 1. This generally manifests after 6 months of age and limited to childhood. 2. It is unilateral. 3. Vision is less in squinting eye. 4. There is hardly any error of refraction. 5. There is no accommodative factor. 6. Deviation is almost same for distance and near. 7. To begin with, the deviation is small that may become very large with age. 8. Being monocular, the squinting eye develops amblyopia which requires proper management. 9. Glasses or miotics do not abolish squint. However associated error of refraction should be treated when present. 10. Surgical treatment is definitive treatment. Commonest surgery is bi-medial recession with conjunctival recession. 2. Convergence excess esotropia : Characteristics : 1. Seen between 2 to 3 years of age 2. Deviation for distance is minimal 3. Near deviation is between 20 to 40. 4. The child may have low grade hypermetropia or may be emmetropic. 5. There is no accommodative factor responsible for deviation. 6. Surgery is the treatment of choice.

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Other esotropias : 1. Microtropia 2. Esotropia in myopia 3. Cyclic esotropia 4. Nystagmus blocking esotropia 5. Consecutive esotropia 6. Secondary esotropia 7. Esotropia in DVD and inferior oblique over action 8. Acute esotropia 1. Microtropia29,30,31. Commonest form of presentation is small degree of esotropia, occasionally it may present as exotropia, hypertropia and rarely as orthophoric, hence it is also called micro-strabismus. The fixation is restricted to non squinting eye, hence it is called mono fixation syndrome. Characteristics : 1. It is a permanent condition. 2. It may follow Congenital or infantile esotropia Surgical correction of above 3. Angle of deviation is small8 to 10 PD. 4. Mild amblyopia is common even in spite of small angle of deviation. 5. Central fusion is absent. 6. There is facultative macular scotoma that is made obvious on Worth four dot test and Bagolinies test. 7. Cover uncover may show small esotropia that increases during prism cover test. 8. Sometime 4 prism diopter base out test may be positive. 9. The condition is more common in anisometropic amblyopia. 10. There is para foveal fixation, peripheral fusion. 11. Harmonious ARC. 12. Almost normal stereo acuity. Management : Mono-fixation syndrome can be treated by orthoptic treatment or surgery. Microtropia persists even after orthoptic treatment, it may strength peripheral fusion. 2. Esotropia in myopia. Myopic children have good near vision and poor distant vision. In small children onset and progress of myopia is so gradual that the child does not realize his poor distant vision unless pointed out. In moderate uncorrected myopia, the near vision is either within near point or near to it. Accommodation worsens myopia. The child is only left with one option i.e. to converge to have clear vision. The child may altogether give up any effort for clear distant binocularity, retain binocularity for near and convergent squint. In older children who have acquired myopia, fusional deficiency of divergence enhances convergence and esotropia.

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The treatment consists of correction of myopia and surgical correction of esodeviation. 3. Cyclic esotropia32,33. This is an uncommon esotropia of unexplained origin. Generally seen in children between three to four years. It is most often mistaken as intermittent esotropia. There is a fixed cycle of esotropia and orthophoria appearing with a fixed gap that ranges between 24 hours to 96 hours. Typical presentation is that the child has a large angle esotropia for a day or two followed by a gap of same duration of orthophoria (non squinting days). Such episodic attacks last for few months to two years before the condition becomes constant for all days. Initially there is no amblyopia, amblyopia develops when the squint becomes constant. See chapter 17 page 608. Treatment consists of treatment of amblyopia when present and surgical correction. 4. Nystagmus blocking esotropia. Children with congenital esotropia are known to have nystagmus either as manifest nystagmus or manifest latent nystagmus independent of esotropia. In some children who have nystagmus in straight eye, the nystagmus disappears if the eye is adducted. This is called nystagmus blocking esotropia. This is often confused as infantile esotropia with nystagmus. The treatment is recession of both medial recti with myopexy behind the equator. See page 633. 5. Consecutive esotropia. It is common for a child to develop small degree esotropia following surgery for exodeviation. This is transient and associated with diplopia. However if esotropia persists after few weeks, it should be evaluated and treated. Possibility of amblyopia should be kept in mind and managed.

EXOTROPIA34
Exotropia is manifest deviation of any of the eyes outward. Normal position of the eye at rest is slight exodeviation. This is so small that it is taken as physiological and the eye is considered to be orthophoric. General features of exotropia1. Exotropia can be congenital or acquired. 2. It can be uniocular or alternate. 3. To begin with most of the exotropias are exophoric. However an eye can be exotropic without going through the phase of exophoria. 4. It can be equal for far and near, it can be greater for distance and lesser for near or vice versa. 5. The angle of deviation may increase with passage of time. 6. Exotropias are generally large degree of deviation. 7. Associated errors of refraction are generally of small degree and there is no predilection for any specific type of error of refraction. 8. Suppression is common but amblyopia is less frequent. 9. An exotropic eye generally have poor adduction that may be diagnosed as medial rectus paresis. In such cases adduction can be demonstrated by patching the other eye for few hours. 10. Exotropia is more common among girls. There may be a hereditary basis.

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There are various modes of development of exotropia. Commonest mode is as follows : OrthophoriaExophoriaIntermittent exotropiaConstant exotropiaIncrease in exotropia. The other less frequent possibility is esotropia passing into straight eye and then exotropia. The third possibility is consecutive exotropia where there is surgical over correction of esotropia. Lastly, secondary exotropia develops in eyes that have long standing poor vision with amblyopia in one eye due to - anisometropia, uncorrected unilateral aphakia, unilateral cataract, corneal opacity, macular scar, optic atrophy. It has been observed that uniocular loss of vision under five years of age may produce both esotropia or exotropia. Esotropia is more common. After five years chances of exotropia is far more than esotropia.35 Various combinations of exotropia
Distance 1. Exotropia 2. Intermittent exotropia 3. Exotropia 4. Exotropia 5. Exotropia Orthophoria Orthophoria or exophoria for near Exotropia Intermittent exotropia Exotropia Near Nil Symptoms for distance Suppression scotoma for distance Binocular vision for near Loss of binocular vision Symptoms

Classification of exotropia
Exotropia Pseudo exotropia True exotropia Congenital Primary Acquired Secondary Consecutive Paralytic Restrictive

Divergent excess

Convergent weakness

Mixed Motor obstacle Orbital anomaly Defective Cyclovertical convergence deviation

Sensory obstacle Uniocular loss of vision Uniocular error of refraction

666 An exotropia is called essential when it is 1. Primary concomitant 2. There is no binocularity 3. There is no neurological defect 4. There is no anatomical defect

PEDIATRIC OPHTHALMOLOGY

5. There is no detectable cause of diminished vision i.e. opacities in media, defect in conduction, unilateral high error of refraction. Pseudo exotropia In this case the eye/eyes seem to diverge due to any of the following causesTelecanthus, positive angle kappa, temporal drag of the macula as in retinopathy of prematurity. On Hirschberg test, the corneal light reflex is central. The cover test does not show any deviation. Pseudo exotropia unlike pseudo esotropia does not disappear with age. Congenital exotropia33,35,36 Incidence of congenital exotropia is less than congenital esotropia. The exotropia becomes obvious by two months of age. The angle of deviation is constant for distance as well as near. The deviation ranges between 40 to 80 PD. There is no cross fixation. The child has homonymous fixation i.e. the child fixes right field by right eye and left field by left eye. This increases temporal field of vision. Alternation is common hence amblyopia is less frequent. DVD and nystagmus may be seen in some cases. Error of refraction when present are of small degree. Management The aim of the treatment is to bring the alignment within 10 PD before two years. Surgery is the treatment of choice. However if there is error of refraction more than 3D hypermetropia or 2D myopia, it should be corrected before surgery. Primary acquired exotropia Intermittent exotropia37 Intermittent exotropia is the commonest type of exotropia and second most common squint in children. The child has exotropia especially when the child is tired or daydreaming. A child may be brought with history of exotropia but on initial examination he may seem to be orthophoric and requires repeated examination in the same sitting to unmask the deviation. Sometimes an intermittent exotropia may resolve spontaneously.37 In about 75% cases the deviation does not change over years but in a small group of cases the deviation may increase. Generally the condition becomes manifest by four to five years of age but not before two years. The child generally has good vision or small error of refraction with normal binocularity in primary position. When the eyes are straight there is good stereopsis, bifoveal fusion without suppression. Facultative suppression develops only when the eye deviates. This prevents diplopia.

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The child himself may not be aware of deviation, looking at a mirror corrects the deviation. However presence of exotropia may become obvious in a photograph. Some children may complain of blurred vision and/or asthenopia. The parents may complain that the child closes one eye in bright light. This phenomenon may persist even after surgical correction. Both A and V pattern are met with, however V pattern is more common in intermittent exotropia. DVD and nystagmus are less frequent than in esotropia. Clinically it can be divided into : 1. Divergence excess exotropia 2. Convergence deficient exotropia 3. Basic exotropia 4. Pseudo divergence Divergence excess type of exotropia The exotropia is more when measured for distance. It is better to measure distant deviation when the child is fixing beyond usual six meters. The difference between far and near is more than 15 PD. The near deviation may occasionally be exophoria. If the accommodation is relaxed by putting +3.00D sph. in front of each eye and near distance is remeasured, there is generally no difference from the original deviation. Convergence deficient exotropiaThe near exotropia is more than distant exotropia at least by 15 PD with full correction. It may be present with diminished accommodation as in myopes who need not accommodate or rarely in hypermetropes more than 6 Dsph who give up accommodation. They may have low Ac/A ratio. Basic exotropia Here exotropia for far and near are equal or with in 15 PD. Pseudo divergence (Simulated divergence excess) The distant exotropia is greater by 15 PD than near as in divergence excess type when examined for first time. This difference decreases to less than 10 PD after half an hours patching of one eye. The measurement should be taken immediately before fusion develops. The other diagnostic test is to measure near deviation with +3.00 Dsph in front of both eyes. The near deviation becomes as much as or greater than distant deviation. The causes of this phenomenon is not understood. Management Treatment is indicated only if the intermittency is replaced by constant exotropia. The mode of management depends upon age of the patient and severity of symptoms. Treatment consist of 1. Optical (a) Glasses for any significant error of refraction. Myopia is fully corrected and hypermetropia is under corrected.

668

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(b) PrismBase in prisms are given for short time before or after surgery. They give better results in small deviation and have hardly any effect on large degree of exotropia. 2. Orthoptics are given in convergence deficient exotropia to strength convergence and eradicate suppression. 3. SurgicalSurgery is the definitive treatment. It is better to operate round about four years of age. Chances of suppression increase after four years and visual prognosis is not good. In small degree squint orthoptics is preferred over surgery. The aim of surgery is to produce small degree of esotropia. In divergence excessRecession of both lateral recti is done to clear distant exotropia. This results in slight esotropia. In basic exotropiarecession of lateral rectus with resection of medial rectus is required. If there is recurrence of exotropia, it should be corrected by base in prism, myopia is over corrected, hypermetropia is under corrected by one dioptre. Addition of 1D stimulates accommodative convergence. Consecutive exotropia This develops following over correction of esotropia especially when esotropia was variable, and partially accommodative. This is common in esotropic eyes with uniocular high hypermetropia. Time lapse between surgery and development of consecutive esotropia is variable. It may develop soon after surgery or may develop months to years after surgery. Sometimes consecutive exotropia develops spontaneously in an esotropic child with amblyopia. Management consists of under correction of hypermetropia, prescription of base in prisms or re surgery for cosmetic purpose. Secondary exotropia This is generally sensory exotropia due to long standing visual loss in one eye. The causes may be - amblyopia, anisometropia, either as high error of refraction in one eye or uncorrected uniocular aphakia, corneal opacity, unilateral dense cataract, maculopathies or optic neuropathies. If diminished vision sets in after five years of age, exotropia is the rule. Before five years the deviation can be either eso or exo. Mechanical factors like scarring of medial rectus following accidental or surgical trauma or paralysis of adductors mostlymedial rectus pareses can cause secondary exotropia. Convergence paralysis This is an acquired form of near esotropia due to CNS involvement as in Parinauds syndrome. Convergence paralysis is associated with palsy of up gaze. It can be seen in encephalitis. In adults it may be caused by multiple sclerosis or neurosyphilis. On examination the convergence is greatly reduced with intact accommodation and miosis. Fusional convergence is invariably poor. Management consists of treatment of primary cause and prisms.

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A-V syndrome38,39 It is a common observation that the eyes diverge slightly on vertical gaze and is considered to be physiological in nature. However in some cases of squint both eso or exodeviation, the divergence is prominent not only on up gaze but also in down gaze. In some cases there is no deviation in straight gaze but have exotropia on up gaze and equal amount of exotropia in down gaze. Similarly still less number of children may show exotropia only on up or down gaze. The conditions have been named after alphabets A, V, X or lambda (inverted y ) due to their imaginary symmetry with these letters. Out of above five condition, the patterns A and V are most common, about 20% of all horizontals squints are associated with A or V pattern. The exact cause of the condition is not known. There are three schools of thoughts for three different causative factor i.e. 1. Horizontal school 2. Vertical school 3. Oblique school The horizontal school of thinking is based on the assumption that the fault lies in horizontally acting muscles only. The vertical school of thought is built on presumption that the A and V patterns are due to symmetrical underaction of vertically acting muscles. The third group believes that both the horizontal and vertically acting muscle are at fault. As there is no single opinion as far as cause of the condition, the mode treatment also vary widely. Classification
Phenomenon A A esotropia/ phoria A exotropia/ phoria V esotropia/ phoria V X Y l

Exotropia Vexotropia/ phoria

Exotropia

Exotropia

The following figures roughly explain A and V pattern :


Exotropia more on up gaze Esotropia more on up gaze

Esotropia more on down gaze

Exotropia more on down gaze

670 Presentation

PEDIATRIC OPHTHALMOLOGY

A child may not be aware of these patterns even in presence of manifest squint and the symptoms are attributed only to horizontal deviation. Sometime A exotropia or V esotropia cause discomfort for near work. A esotropic generally has a chin up position while A exotropic keeps the chin down. The former may have mongoloid slant of inter palpebral fissure. The later has anti mongoloid slant. Diagnosis A case of V esotropia may be mistaken as convergence excess due to accommodation and a V exotropia may be mistaken as divergence excess. To avoid this the measurement should be taken not only on straight gaze but also at 25 up and 25 down gaze and deviation measured by prism bar and cover test. The measurements should be done with full correction whenever needed. In V pattern the difference should be at least 15 PD and for A pattern 10 PD difference is sufficient for diagnosis. A-V syndrome may be associated with dysfunction of obliques, DVD or cranio facial anomalies. Treatment of AV syndrome consists of management of error of refraction, elimination of amblyopia, correction of horizontal deviation by surgery with appropriate modification of placing of horizontal insertion or modification of action of obliques. For A esotropia Without oblique dysfunctionBilateral recession of medial rectus with upward shift of the insertion or recession of medial rectus with shift and resection of lateral rectus with shift downwards. If there is associated superior oblique over action either both medial recti are recessed or recession of one medial rectus and resection of lateral rectus. Both the above procedures are done with weakening of superior oblique. A exotropia with normal oblique action - Bilateral recession of lateral rectus with down shift. A exotropia with superior oblique over action is treated by recessing both lateral recti and weakening superior oblique. V esotropiaRecession of medial rectus with downward shift of insertion. V ExotropiaRecession of lateral rectus shift upward. Oblique may have to be tackled along with horizontals. Care should be taken not to operate more than three muscles in the same eye in single sitting to avoid ischemia of anterior segment.

REFERENCES
1. Lyle T.K., Wybar K.C. ; Development of binocular vision in Lyle and Jacksons Practical Orthoptics in the treatment of squint. First Indian edition, p-4759, Jay Pee brothers, New Delhi, 1994.

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2. Dutta L.C. ; Physiological binocular vision in Ophthalmology principle and practice. First edition, p-533536, Current Book International, Calcutta, 1995. 3. Dutta H. ; Abnormalities of binocular vision in Strabismus. First edition, p-2122, Jay Pee Brothers, New Delhi, 2004. 4. Rohatgi J.N. ; Abnormal retinal correspondence in Squint basic and clinical aspect. p-7377, CBS Publishers, New Delhi, 2003. 5. Agarwal L.P. ; Viscuoscope in Optics and refraction. Second edition, p-209210, CBS Publishers, New Delhi, 1979. 6. Agarwal L.P. ; Heterophoria in Essentials of Ophthalmology. First edition, p-393 398, CBS Publishers, New Delhi, 2000. 7. Vaughan D. and Asbury T. ; Hyperphoria in General ophthalmology. Tenth edition, p-192193, Lange Medical Publication, California, 1983. 8. Miller S.J.H. ; Kinetic strabismus in Parsons disease of the eye. Seventeenth edition, p-289, Churchill Livingstone, London, 1984. 9. Virginia Carlson Hansen ; Duanes classification of horizontal strabismus in Ocular motility. First Indian edition, p-35, Jay Pee Brothers, New Delhi, 1989. 10. Manley D.R. ; Classification of esodeviation in Pediatric ophthalmology. Vol. I, second edition, p-227228, Edited by Harley R.D., WB Saunders Company, Philadelphia, 1983. 11. Lang J. ; Microtropia Arch. Ophthal. 81: 758, 1969. 12. Buckley E.G. ; Congenital esotropia in Current Ocular therapy. Fifth edition, p-391392, Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000. 13. Diamond G.R. ; Congenital esotropia in Text book of ophthalmology. Vol. 5, p-8.1, Edited by Podos S.M. and Yanoff M., Mosby, London, 1991. 14. Manley D.R. : Congenital esotropia in Pediatric ophthalmology. Vol. I, Second edition, p-228229, Edited by Harley RD, WB Saunders Company Philadelphia, 1983. 15. Dutta H. ; Esotropia in Strabismus. First edition, p-7476, Jay Pee Brothers, New Delhi, 2004. 16. Fiona Rowe ; Congenital/infantile esotropia in Clinical orthoptics. First edition, p-99100, Blackwell Science Ltd., Oxford, 1997. 17. Deborah Pavan Langston : Accommodative esodeviation in Manual of ocular diagnosis and therapy. Third edition, p-310311, Lippincot Williams and Wilkins, Philadelphia, 1991. 18. Dy Santiago Alvina Pauline ; Rosen Baum A.L., Esotropia, High accommodative convergence to accommodation in Current ocular therapy. Fifth edition, p-400401, Edited by Fraunfelder FT and Roy FH, WB Saunders Company, Philadelphia, 2000. 19. Ing MR ; Early surgical alignment for congenital esotropia. Tr. Am. Oph. Soc. 79:625663, 1981.

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20. Eggers H.M. : Non surgical treatment exotropia in Text book of ophthalmology strabismus and pediatric ophthalmology Vol. 5. Vol. 5, p-77, edited by Podos S.M. and Yanoff Mosby, London, 1991. 21. Noorden GK ; Binocular vision and ocular motility. 4th edition, CV Mosby, St. Louis 1990. 22. Sharma P. ; Accommodative esotropia in Strabismus simplified. First edition, p-93, Modern Publishers, New Delhi, 1999. 23. Eugene R. Folk : Accommodative esotropia in Principles and practice of ophthalmology. Vol. III, First Indian edition, p-6-27, Edited by Peyman GM, Sanders DR and Goldberg MF, Jay Pee Brothers, New Delhi, 1987. 24. OHalloran H.S. ; Accommodative esotropia in Current ocular therapy. Fifth edition, p-386387, Edited by Fraunfelder FT and Roy FH, WB Saunders Company, Philadelphia, 2000. 25. Fiona Rowe : Method of measurements of Ac/A ratio in Clinical Orthoptics. First edition, p-58, Black Science, Oxford, 1997. 26. Mohan K. ; Non refractive esotropia in Clinical practice in ophthalmology. First edition, p-536, Jay Pee Brothers, New Delhi, 2003. 27. Kenan J.M., Willshaw H.E. ; The outcome of strabismus surgery in childhood esotropic, Eye : 7:341-345, 1993. 28. Rohatgi J.N. ; Concomitant convergent squint in Squint basic and clinical aspect. First edition, p-142145, CBS Publishers, New Delhi, 2003. 29. Park M.M. ; Mono fixation syndrome. Tr. Am. Oph. Soc. 67:609-657, 1969. 30. Lang J. ; Microtropia. Arch. Oph. 81 : 758, 1969. 31. Wilson M.E. ; Mono fixation syndrome in Current ocular therapy. Fifth edition, p-406407, Edited by Fraunfelder FT, Roy FH, WB Saunders Company, Philadelphia, 2000. 32. Burian H.L.M. ; Cyclic esotropia in strabismus ophthalmic symposium, edited by H. Allen C.V., Mosby, St. Louis, 1958. 33. Manley D.R. : Cyclic esotropia in Pediatric ophthalmology. Vol. 1, second edition, p-233240, Edited by Harley RD, WB Saunders Company, Philadelphia, 1983. 34. Burian H.L.M. ; Exodeviation, their classification, diagnosis and treatment. AJO. 62 : 1161-1166, 1966. 35. Eggers H.M. ; Exotropia in strabismus and pediatric ophthalmology. Text book of ophthalmology. Vol. 5, First edition, p-717.13, Edited by Podos SM and Yanoff M., Mosby, London, 1991. 36. Moore, S., Cohen R.L. ; Congenital exotropia, AJO 35 : 68-70, 1985. 37. Steinkuller P.G. ; Intermittent exotropia in Current ocular therapy. Fifth edition, p-404405, Edited by Fraunfelder F.T., Roy F.H. WB Saunders Company, Philadelphia, 2000. 38. Rohatgi J.N. ; A and V syndrome in Squint Basic and Clinical aspect. First edition, p-164173, CBS Publishers, New Delhi, 2003. 39. Lyle, T.K. ; A and V phenomenon pattern or syndrome in Practical orthoptics in the treatment of squint. First Indian edition, p-397405, Jay Pee Brothers, New Delhi, 1994.

CHAPTER

20

Disorders of Orbit in Children


Orbits are two spaces surrounded by craniofacial bones on all sides except in front, they are roughly pyramidal in shape with base anteriorly and apex posteriorly, unlike pyramids all the four sides the walls of orbit are not equal in length nor their inclination to each uniform. The medial wall is shortest. The medial wall of two sides are more or less parallel to each other, distance between two medial walls is 25 mm. This is called inter orbital distance, the lateral walls are inclined to medial wall at an angle of 45. The two lateral walls if extended backward form an angle of 90 with each other. The point where the four walls of orbit meet is known as apex of orbit, which is posterior-most part of orbit. The orbits lie under the anterior cranial fossa and above the maxillary sinuses. The medial wall is mostly formed by the ethmoid and sphenoid that separate the orbit from the nasal cavity. The lateral wall separates the orbit from middle cranial fossa and temporalis muscle. The anterior border of the orbit is not circular in adults. In children they are almost circular. The base of the orbit is not the widest part of the orbit, the widest part of the orbit lies behind the orbital rim.1 It corresponds to the maximum diameter of the globe i.e. the equator. The orbit is formed by seven bones of different sizes. They are frontal, zygomatic, maxilla, lacrimal, palatine, ethmoid and sphenoid. The orbit is said to have an apex, a roof, a lateral wall, a floor, a medial wall and rim. The rim is formed by frontal, maxillary and zygomatic bones. The rim has been divided into four parts - superior, lateral, inferior and medial corresponding to roof, lateral wall, floor and medial wall respectively. The superior rim has two notches, the supra orbital notch and supra trochlear notch. They transmit supra orbital and supra trochlear nerves and vessels. The lateral orbital margin is strongest part of the orbit, the infra orbital margin transmits infra orbital nerve. In fracture of the orbital rim, this nerve is invariably injured resulting in hyposthesia of the skin lateral to the nose. The medial margin has fossa for lacrimal sac in the inferior medial aspect. It is formed between the anterior and posterior lacrimal crests. The lower part of the fossa narrows to form canal for nasolacrimal duct. The roof of the orbit is formed by orbital plate of frontal and lesser wing of sphenoid. On the anterio lateral part is fossa for lacrimal gland and medially there is trochlear fossa. The apex of the orbit lies in the posterior most part of the roof. The frontal lobe of the brain and its meninges are above the roof while the periorbita LPS, RS, SO, lacrimal gland, 673

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frontal and trochlear nerves lie under the roof. The roof is not thick and strong. Defect in the roof can cause herniation of the brain and meninges in the orbit either as a congenital anomaly or following trauma. The medial wall is mostly formed by ethmoid and sphenoid, which are very thin. They are the weakest parts in the orbit and liable for fracture. The thin wall of the ethmoid can transmit infection from the ethmoidal sinuses, which is the most common cause of orbital cellulitis in children. The frontal process of maxilla and lacrimal bone form a small part of medial wall, the maxilla forms the anterior lacrimal crest while the posterior lacrimal crest is formed by lacrimal bone. The anterior lacrimal crest is palpable in normal individuals. The ethmoidal sinuses and middle meatus form the medial relation while the superior oblique medial rectus, terminal part of the ophthalmic artery and nasociliary nerve pass over the inner surface. The floor of the orbit is formed by maxilla, zygomatic and palatine bones. The maxillary part is very thin and common site for blow out fracture of orbit. The contents of the orbit can get herniated in the maxilla following blow out fracture. The floor is separated from the lateral wall by infra orbital fissure, the part anterior to this fissure is infra orbital groove. The anterior most part is converted into infra orbital canal that opens in infra orbital foramen. The inferior oblique takes origin from a rough area on the anterio medial part of the floor and traverses back. This is the only extra ocular muscle that has its origin in front of the globe. The inferior rectus, the inferior oblique, the nerve to inferior oblique lie above the periorbita. Under the floor lies the maxillary antrum and palatine air cells. The lateral wall of the orbit is formed by the zygomatic bone anteriorly and greater wing of sphenoid behind. The greater wing of sphenoid separates the superior orbital and inferior orbital fissures. The lateral wall separates temporal fossa laterally and middle cranial fossa posteriorly from the periorbita, lateral rectus, lacrimal nerve vessels and zygomatic nerve. The orbit is lined by the periostium of the orbit, called the periorbita on its inner surface. The periorbita extends from the apex of the orbit to the rim of the orbit where it merges with the periostium of face and forehead. The periorbita is loosely attached on the inner surface of the orbit and a potential space lies between the bone and the periorbita, which can accumulate fluid resulting in periorbital abscess. The periorbita is attached firmly to the apex of the orbit, round the optic foramen where it merges with dura of optic nerve, at the margins of the superior and inferior orbital fissure. At the posterior lacrimal crest, it splits into two parts that cover the lacrimal sac as lacrimal fascia. It is also firmly anchored to the sutures of all the bones of orbit and borders of various foramena. The orbital septum The orbital septum is a sheet of strong elastic tissue that prevents the contents of the orbit to come out into the lid. In fact it divides orbit into two parts, a smaller anterior part and a larger posterior part. The orbital septum prevents spread of infection from the anterior part to the orbit and vice a versa. The septum originates from the periostium of the orbital margin. In the upper part, it terminates 10-15 mm above the upper tarsus and gets inserted in the aponeurosis of levator. In the lower lid, it originates from the periostium of the inferior orbital rim, goes up tobe inserted in the capsulopalpebral ligament of the lower lid. The

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capsulopalpebral ligament is anatomic equivalent of levator aponeurosis in the lower lid. The septum terminates 6mm below the lower tarsus. Surgical spaces of the orbit3,4 The orbit is divided into four unequal surgical spaces by various membrane like structure. They are : 1. Periorbital 2. Peripheral 3. Muscle cone and 4. Tenons space The spaces help in localising infection, inflammation and neoplasm in a restricted zone. Involvement of each space has its own clinical presentation that differentiate one from the other, however there may be some overlap. 1. Periorbital space. This is a potential space between the periorbita and the bones of the orbit. The periorbita can be surgically lifted with ease except at its attachment at various sutures, edge of the fissures and optic foramen. Pus, blood or tumours lift the periorbita, pushing the globe to the opposite side. The tumours may remain in this space for considerable time without breaking the periorbita. Common growth that are seen in this space are tumours of the bone of the orbit, leukaemia deposits, dermoids, meningiomas and mucocele arising from various paranasal sinuses. This is a frequent site for accumulation of blood. The growths of this space are diagnosed by direction of displacement of globe, X-ray or CT. While surgically opening the space, care should be taken not to spill the infection to other surgical spaces. 2. Peripheral space. This space lies between the periorbital space and the muscle cone. It is bounded anteriorly by the septum orbitale, tarsal plates, tarsal ligaments and levator aponeurosis. In the posterior part, it merges with the central space. It contains lacrimal gland, superior and inferior oblique muscles, and levator palpebral muscle. The frontal, lacrimal and ethmoidal nerves pass through this space. The superior and inferior ophthalmic veins lie in this space. The position of superior ophthalmic vein is very constant hence it is used for orbital venography, which differentiates intra conal lesions from extra conal lesion. Space occupying lesions of this space cause displacement of the globe to the opposite side and proptosis, which are best seen on CT and explored either via anterior or lateral orbitotomy. Posterior placed lesions are best removed by transnasalendoscope. Exudation from this space may permiate through the septum orbital and cause edema of the lids. 3. Central space. There is a conical spaces with apex towards the apex of the orbit, the wall of this space are formed by the four recti and their inter muscles attachments. The inter muscular septa is deficient at the posterior part of the space hence the apex of the cone merges with peripheral space. The optic nerve traverses the cone from its apex to the back of the globe along with its covering. The long and short posterior ciliary nerves and vessels, the ciliary ganglion, the two divisions of third nerve; the sixth nerve, the nasociliary nerve, ophthalmic artery, lie in this space. These structures are embedded in the orbital fat. Growths arising from any of these structures, neoplastic deposits, pus, blood or foreign body cause an

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axial proptosis. Commonest cause of axial proptosis in children is optic nerve glioma. The growths are explored through a lateral orbitotomy. Posteriorly placed growths are removed endoscopically or by transcranial route. The Tenons capsule and Tenons space 4. Tenons capsule or fascia bulbi is a thin membrane that covers the globe from the limbus upto optic nerve. Anteriorly it is deeper to the conjunctiva and merges with the subconjunctival tissue to form a strong attachment, posteriorly it merges with the dura of the optic nerve. The sclera lies within the capsule. With the movement of the eye, the Tenons capsule also moves. Posteriorly the orbital fat is in contact with the capsule, the posterior part is pierced by the optic nerve, ciliary vessels and nerves. The venae verticosae pierce the capsule behind the equator. At the insertion of the extraocular muscle, the Tenons capsule assumes a tube like shape in which the muscles pass like hand in the glove. The Tenons capsule sends few extensions beyond the muscle. The expansion of the capsule from the lateral rectus is attached to the Whitnall ligament and is called lateral check ligament of the eye. Similarly an extension from the medial rectus is attached to the lacrimal crest and called the medial check ligament. The function of these check ligaments is to stabilise the globe and prevent excessive movement of the globe. The expansion from the sheath of the medial rectus is joined to the levator palpebral superior and co-ordinate upward movement of the globe and the lid. It is essential to cut these extensions during ptosis surgery otherwise the eye will be rotated down resulting in to hypotropia. In case of surgery of the recti, the capsule should not be cut beyond 10mm from the insertion otherwise orbital fat will herniate. Similarly an extension from the inferior rectus is attached to the capsulopalpebral fascia of lower lid. The expansion from the superior oblique is attached to the trochlea, that of inferior oblique is attached to the lateral part of the floor. Expansions from the lateral and medial recti are joined by the extension from the two oblique to form a sling on which the eyeball rests and this is called Lockwood ligament.3 The Tenons space is a potential space between the Tenons capsule and the outer wall of the globe. The space is traversed by the insertion of the extraocular muscles, the ciliary nerves and vessels the venae verticosae. Fluid accumulated in the posterior part of the space cause proptosis. The fluid accumulated in the Tenons space is drained through the conjunctiva. This space is also utilised to give anterior and posterior Tenon injection of antibiotic, steroids and anaesthesia. Fissures of the orbit The orbit has two elongated fissures i.e. the superior orbital or sphenoidal fissure and inferior orbital fissure, the former is larger and transmit more anatomical structures to and fro the orbit. The superior orbital fissure This is an elongated inverted comma like defect between the lateral wall and the roof of the orbit. It is a gap between the greater and lesser wing of the sphenoid, wider medially and inferiorly and narrow in superio lateral aspect. It is about 22 mm in length. The lateral most point of the fissure is about 35 mm from the frontozygomatic suture of the rim of the orbit. It

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is lateral to the optic foramen. A tendenous ring called annulus of Zinn that divided the fissure into superior, central and inferior part forms the common origin for all the recti except the lateral rectus. The lateral rectus originates slightly lateral to this ring from a boney prominence called the spine for the lateral rectus. The fissure communicates with the middle cranial fossa posteriority. The upper part transmits trochlear, frontal and lacrimal nerves, superior ophthalmic vein and recurrent lacrimal artery. Through the middle part pass the superior division of third nerve, the nasociliary nerve is the sympathetic connection to ciliary ganglion, inferior divisions of third nerve and abducent nerve. The inferior ophthalmic vein is the only structure that passes through the lower part of the fissure. The optic nerve and ophthalmic artery enter the orbit through the fibrous ring outside the superior orbital fissure. The inferior orbital fissure The inferior orbital fissure lies between the lateral wall and floor of the orbit. It is separated from the superior orbital fissure by a thin piece of bone. The inferio orbital fissure transmits veins from the lower part of the orbit to join the pterygoid plexus, the zygomatic nerve and intra orbital nerve. Development of the orbit Development of orbit is a complicated process involving bones of orbit, paranasal sinuses, contents of the orbit and brain. By 3 months of intrauterine like boundaries of the orbit are apparent but not complete.6 The walls develop along the development of the face and are well grown by fourth month. The bones are mesodermal in origin that develop around the developing eyeball. The floor and lateral walls develop to form the maxillary process, the roof develops from paraaxial mesoderm forming capsule covering brain. The medial wall develops from lateral nasal process. The developing eye does not influence development of walls of orbit. However size of the orbit depends on the size of the eyeball as size of cranium depends on size of the brain. (See page 7 also) The extra ocular muscles develop from the paraxial mesoderm, the six muscles are supplied by three cranial nerves. The mesodermal masses are arranged into three groups, each supplied by one cranial nerve. The cranial nerves grow from the brain into specified mesodermal mass. The oculomotor starts developing earliest and trochlear last. (See page 4 also) The orbit at birth Orbits are fully developed at birth but are larger in proportion to the face when compared to adult face. The orbital walls are nearer to the globe. The volume of the orbit is small in infancy that makes the children more prone to develop proptosis. The orbits are placed nearer to each other, with age they shift laterally to assume normal adult position. The intraocular contents are well developed, any defect in development of extraocular muscles lead to strabismus. Some of them may be congenital others develop in childhood. The orbits of the children are placed nearer to each other than adults, with age of orbits move laterally. The depth of the orbit is shallower in children. Volume of average adult orbit is 30 ml, the volume of average adult eye ball is 7.1 ml, giving a ratio of 4.5 : 1. The volume of eyeball of an infant is 2.8 ml and volume of orbit is about 13 ml. The orbital walls

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are very close to the eyeball at birth. The size of eyeball in children is an important factor in growth of the orbit. Smaller or absent eyeball retard the growth of orbit in children giving asymmetrical orbit. Bilateral microphthalmia or anophthalmos result in smaller orbit on both sides. Large eyeball i.e. congenital myopia, buphthalmos, retinoblastoma cause enlargement of the orbit. So do other intra orbital growths in children. Congenital anomalies of orbit5 The common congenital anomalies are : 1. Hypertelorism 2. Hypotelorism 3. Shallow orbit 4. Orbital meningocele 5. Orbital encephalocele 6. Orbital and periorbital dermoids 7. Plexiform neurofibroma 8. Sturge Weber syndrome 9. Orbital teratoma 10. Cranio facial dysostosis. Hypertelorism6,7,8 Hypertelorism is a condition where the intra orbital distance is more than 33 mm in adult and correspondingly less in children. It may be isolated defect but generally associated with other deformities of face and skull. It is inherited as autosomal recessive or dominant trait, sporadic cases are also known, there may be family history. The clinical features are broadening of nose bridge, depressed nose bridge, prominent forehead, exotropia, optic atrophy, astigmatism, displacement of lacrimal puncta laterally, ptosis, inverse epicanthus. Hypertelorism is common in Crouzons and Aperts syndrome. Generally the condition is bilaterally symmetrical, however it may be asymmetrical and does not require treatment. Hypotelorism This is a very rare condition where intra orbital distance is reduced. The eyes are set nearer as in Hallermann-Streiff syndrome. No treatment is required. Shallow orbits - Shallow orbits are very common in craniofacial dysostosis resulting into proptosis. Meningo encephalocele orbit8,9 During the development of the skull and orbit, the covering of the brain/ brain substance with its covering may herniate through a bony defect in the orbit. The common sites for such herniation are root of the nose and superio nasal angle of the orbit. When only the meninges herniate, this is called meningocele. This is rarer than encephalocele. The meningocele presents as round, soft, pulsatile growth that may have cough impulses. It is generally noticed at birth or soon after. The encephalocele commonly develop at root of the nose, may remain localised

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or spread to the orbit. Ocular involvements depend on position and size of the encephalocele. It can cause lateral displacement of the globe, proptosis. Squint, may be associated with microphtlamia. The systemic findings include mental deficiency, hydrocephaly, spina bifida, harelip and cleft palate. Treatment is surgical removal of the growth and repair of the defect in the orbital bone. A small meningocele or encephalocele near the medial canthus may be confused as congenital mucocele in infant and encysted mucocele in adult. The difference in two conditions is settled on CT and MRI. Orbital and periorbital dermoids Dermoids are choriostomas, which means that they are benign growths, which arise from tissues that are not normally found in the site of the origin of the growth i.e. skin and sebaceous glands in case of orbital dermoids, epidermoids and teratomas. These growths are due to sequestration of primitive ectoderm in case of epidermoid and dermoid while teratomas contain more than one germinal layer5, i.e. cartilage, bone, teeth etc. Dermoids result when deeper layer of skin containing sweat glands and sebaceous glands, hair follicles are incarcerated. Epidermoids contain only superficial layers of skin. It is not always possible to differentiate between the dermoid and epidermoid. The choriostomas generally arise at the suture lines of the orbit. Commonest site being superio temporal followed by superio medial in front of the orbitalseptum, sometimes they may arise from greater wing of sphenoid and cause proptosis. The dermoids arising on the globe are called limbal dermoids. Twenty five percent of dermoid anterior to the orbital septum are noticed at birth or soon after as palpable, non-tender, well circumscribed growth with rubbery consistency. They may be movable or immobile, generally cystic. On rupture they produce pultaceous material. The deeper dermoids are discovered in the first two decades, they may not produce proptosis and are palpable only if placed anteriorly. Posteriorly placed dermoids are seen in third decade and cause proptosis. The epidermoids are solid growths in contrast to dermoids that are cystic. Both produce sharply demarcated bone defect with well defined margin and sometime sclerotic bone changes. Occasionally the dermoid can be dumb-bell shaped. The radiology of deep orbital dermoids have classical cystic appearance with well defined wall. CT and MRI give better results. Treatment consist of surgical removal. Anteriorly placed cysts present no problem in removal. However, they should be differentiated from meningo encephalocele prior to surgery. The posterior orbital dermoids are best removed by lateral orbitotomy or craniotomy. Orbital teratomas5 Orbital teratomas are uncommon, they may contain all the three germinal layers. Occasionally a miniature second foetus may be present in the orbit. Generally these are small at birth but grow fast enough to produce severe proptosis and exposure, with loss of vision. Treatment consists of complete surgical removal. Cranio synostosis (Craniostenosis, craniofacial dysostosis) oxycephaly (Tower skull) Oxycephaly is a common form of craniostenosis with ocular involvement. In this case there is premature closure of coronary and lambdoid sutures that results in growth of the skull vertically and arrested growth in anterio posterior and lateral planes before the brain growth is complete.1,2,8

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Growth of brain is an essential factor in growth of skull like growth of eyeball influences growth of orbit, if the brain stops growing the skull remains small even with normal sutures resulting in microcephaly. The frontal and perital bones grow steeply and vertically. It is more common in boys. It is generally present at birth or noticed soon after birth. Orbital involvement is roughly symmetrical. Due to premature closure of the sutures, the orbit fails to develop in all directions, resulting in shallow orbit that is too small to house growing globe. The globe is generally normal, the effect on the globe is secondary to shallow orbit. The shallowness of the orbit is caused by medial and forward rotation of greater wing of sphenoid. The pituitary fossa is also small. 5 Most striking ocular feature is gradually increasing bilateral proptosis. In advanced degree of proptosis, the lids fail to cover the cornea and cause exposure keratitis leading to ulceration and perforation. In extreme proptosis, the globe may subluxate. The first dislocation is very frightening to the child and the parents who learn to reposit the globe themselves later. Proptosis is very often associated with divergent squint, nystagmus and other motility disorders. There may be associated developmental cataract. Commonest symptom next to cosmetic blemish is diminished vision that is brought about by corneal changes, change in optic nerve. Optic nerve changes are caused by narrowness of optic canal leading to primary optic atrophy or post papilledematous atrophy due to raised intra cranial tension. Raised intra cranial tension leads to separation of non-fused sutures, thinning of the cranial bones and digital impression on the cranium. The vertical growth of the skull stops with completion of brain growth round about seven to eight years of age. There is no known treatment except craniotomy under six months of age. The exposed cornea should be kept moist by lubricants and antibiotic ointment. Tarsorrhaphy may be required in moderate cases and cases of frequent subluxation of globe. The tarsorrhaphy usually is ineffective in severe cases. Other forms of congenital deformities of skull are5 1. Scaphocephaly (Scaphoid head)Long narrow head due to premature union of sagital sutures. 2. BrachycephalyThe skull is short and broad due to fusion of coronal suture, may be considered as variation of oxycephaly. 3. DolichocephalyA prominent frontal region. 4. Trigonocephaly (Triangular head)There is under development of frontal region of skull with over development of posterior part of the head. This results in close set orbits and optic atrophy. 5. Microcephaly (Microencephaly)Overall dimension of the skull is smaller than normal, the primary fault lies in the smallness of the brain. There is premature fusion of all the sutures and closure of the fontanelle, neurological defects which include mental retardation, idiocy, paraplegia, diplegia. Epilepsy is common. The condition may be noticed at birth or soon after. The orbits are deformed. The child may not only be blind but deaf and dumb. Multiple ocular defects are met with. 6. Hydrocephalus - In this condition there is normal production of CSF with obstruction in its flow. There are three possible sites where the obstruction may develop.

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(i) Sylvius aqueduct (ii) Foramen of Magendie (iii) Foramen magnum. Obstructed CSF circulation results in accumulation of CSF in ventricles, which get dilated, pressing the overlying brain, and thinning of the skull resulting into enlargement of the head in all direction with normal face. The sutures are wide open. The face looks triangular in relation to monstrous head, which the child may not be able to hold. The skin over the forehead is stretched, dragging the upper lid leading to lagophthalmos and lid retraction. The lower lid is similarly pulled up and covers the lower part of the cornea compensating the up drawn upper lid. The eyes are pushed down, giving a setting sun appearance. There may be associated proptosis, paralytic squint. In spite of accumulation of CSF, papilledema is rare and primary optic atrophy is more common. The condition may be noticed on ultrasonography before birth, may cause obstructed labour or may be noticed soon after the birth. Some of the predisposing factors are high maternal age, Rh incompatibility, maternal rubella and hydramnios. Crouzons Syndrome5,6,13 This craniofacial dysostosis has important ocular manifestation. This is an autosomal dominant trait seen more commonly in boys. It is a bilateral condition that may be suspected at birth or within few months. The child has dolicocephaly and frog like face. Bilateral exophthalmos is commonest feature caused by shallow orbit with normal sized eyeball. There is downward slant of outer canthus. Other findings are exotropia with tendency towards V pattern. Paralysis of extra ocular muscles, nystagmus, under developed maxilla resulting small upper jaw and large lower jaw, hypertelorism and optic atrophy without papilledema. X-ray shows sutural closure with increased digital marking. There may be hearing loss and various degrees of mental retardation. Management consist of protection of cornea from exposure keratitis that may end up in perforation. Early craniotomy have been advocated to arrest the progress of disorder. Aperts syndrome (Acrocephalo syndactylia) This is a combination of oxycephaly with fusion of fingers of both extremities of variable nature. It may run in families in various combinations and inheritance is autosomal dominant however sporadic cases are frequent. Increased maternal age is a contributing factor like in Crouzons disease, the orbits are widely separated and shallow resulting in to hypertelorism, proptosis and anti mongoloid slant of palpebral fissure. The forehead is prominent, maxilla is hypoplastic with high arched palate. The ears are low set. The middle third of the face is more involved than the lower two third, causing crowding of the teeth, parrot shaped nose. Oculo motor disorders are more common, commonest being exotropia with V pattern followed by vertical squint that is brought about by displacement of trochlea at the superior orbital margin. There may be over action of inferior oblique. Due to pushing forward of the globe, there is stretching of extra ocular muscles, resulting in various degree of external ophthalmoplegia. Diminished vision is brought about by corneal changes, astigmatism, amblyopia and optic atrophy. Generally intra cranial tension is normal. Occasionally papilledema has been reported. Mental retardation is common so are cardiac and visceral malformation.

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Management consists of protection of cornea by lubricant, antibiotic. Tarsorrhaphy may give temporary respite, early craniotomy may arrest the progress of the disease. First and second branchial arch syndromes5,6,8,14 Abnormal development of first and second branchial arches lead to a group of congenital disorders of face and eye, that are called mandibulo facial dysostosis without proptosis. Common syndromes are 1. Franceschettis syndrome 2. Pierre Robin syndrome 3. Goldenhars syndrome 4. Hallermann Streiff syndrome 5. Waarden burg syndrome Franceschettis Franceschettis syndrome (also known as Treacher Collins syndrome when the ears are not deformed)The syndrome is bilateral, symmetrical. There is no gender predilection. No race is immune. It has autosomal dominant inheritance. Sporadic cases are not uncommon. Severity increases with successive generations. Claims of unilateral cases are doubted. Increased parental age increases chances of the syndrome. Both upper and lower facial bones are involved, resulting in to hypoplasia of maxilla and mandible. The general features are striking with macrostomia, micrognathia, abnormal hair line, deformity of external and middle ear, hearing loss, sinus between the ear and angle of mouth, coloboma of the lip, high arched palate and abnormality of dentition has been seen. The ocular manifestation consists of normal globe with fairly good vision. The orbit is egg shaped, most of the anomalies are located in the lower lid with changes in the lateral canthal ligament and lower lid margin. The change in lower lid contains various types of colobomas of different length and depth. The minimal change is a S-shaped curve in the outer third of a lower lid. There may be a full thickness defect with loss of lashes and tarsus. Sometimes a large coloboma may cause deficiency of outer third. There may be trichiasis, distichiasis. The orbicularis is also under developed. All these lead to abnormal tear film, corneal irritation and corneal exposure. Various types of strabismus are possible. There is always some degree of astigmatism. Squint, astigmatism and corneal opacity lead to amblyopia, which is the main cause of defective vision. Optic nerve does not show any atrophy or papilledema. Intelligence and growth of the child is good. The condition can be noticed at birth if looked-for carefully, especially if there is a positive family history or it becomes evident by first year. Presence of syndrome can be seen on intra uterine ultrasonography. The ocular changes do not require any treatment except correction of error of refraction, management of amblyopia and protection of cornea. Facial deformities are managed by plastic surgeons. The children are prone to repeated respiratory problems due to narrow upper respiratory passage, which may cause difficulty in anaesthesia. Pierre Robin syndrome8,15 This syndrome has fewer ocular features. Main features are micrognathia. U Shaped cleft palate without cleft lip, glossoptosis, bird like face, dysphasia, difficulty in feeding lead-

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ing to failure of development. Ocular findings are microphthalmia, high myopia, glaucoma, and retinal degeneration. Goldenhars syndrome (Oculo auriculo vertibral syndrome)5,16 This syndrome is sporadic, slightly more in boys. Right side is more commonly involved than the left. The triad consists of ocular changes, changes in external ear and changes in cervical vertebra. Heart and central nervous system may also be effected. There is hypoplasia of temporo mandibular and pterygo mandibular complex16A. 1. The ocular components arecoloboma of upper lid, dermoid or dermolipoma, nasolacrimal duct obstruction, reduced corneal sensation, squint, microphthalmia, persistent pupillary membrane. 2. The auricular components consists ofPre auricular appendages, distorted pinna, absent auditory meatus. 3. Vertebral changes areFusion of cervical vertebra, hemivertebrae, occipitalisation of atlas. The condition can be diagnosed by ante natal ultrasonography. Post natalit can be diagnosed at birth due to limbal dermoid, coloboma of lid and auricular appendages. The limbal dermoids are met within three fourth of cases, out of which about 25% are bilateral. The dermoids are uniformly situated at lower temporal area. Management of the cases is multidisciplinary. Ocular treatment consists of repair of upper lid coloboma and removal of limbal dermoid. The coloboma and dermoid can cause permanent corneal scarring and astigmatism. Hallerman Streiff syndrome This relatively rare syndrome, which is sporadic, consists of a parrot beak nose, loss of eyebrow and lashes, microphthalmia, congenital cataract, micrognathia, short stature. There is an antimongoloid slant of the inter palpebral fissure. Hypotelorism, nystagmus and squint are some of the other ocular features. Vision is generally poor and does not improve with glasses, may be due to corneal or lenticular changes, cataract is generally bilateral and may become mature and soft, predisposing lens induced uveitis and glaucoma. Systemic conditions are infection of respiratory system, difficulty in breathing. Management consists of lensectomy either with posterior chamber implant, failing which aphakia is corrected by contact lens or spectacle. Waardenberg syndrome (White forelock syndrome)32 The syndrome is autosomal dominant32A in nature. There is a defect in melanocyte, which runs in the families. The syndrome consists of band of grey forelock, fusion of prominent eyebrows, broad nose bridge, displacement of medial canthus along with lacrimal puncta. The puncta may be shifted as much as the lower part of cornea, there is heterochromia of the iris, albinism and unilateral deafness. The condition is compatible with normal life except occasional aganglionic megalocon. Visual loss is minimal. Ocular conditions do not require much attention. However deafness requires early management.

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The orbits are surrounded by paraorbital air sinuses from all sides except anteriorly and laterally, the brain with its coverings lies above the roof of the orbit, the optic foramen, and superior orbital fissure communicate with the brain, the ophthalmic veins, the vena verticosae open in the cavernous sinus. The orbit besides the globe, contains muscles, vessels, nerves, fascia, meninges and fat. Hence the orbits are seats of many diverse disorders that spread between intra uterine life to ripe old age. The symptoms of orbital disorder can be broadly divided into two groups : 1. Forward propulsion of the globethe proptosis/exophthalmos 2. Retraction of globeEnophthalmos All other symptoms are sequelae or complications of these two symptoms. Incidentally proptosis (exophthalmos) and enophthalmos themselves are signs of other clinical importance as well. Signs and symptoms of proptosis are : 1. Pain 2. Conjunctival congestion and chemosis 3. Diminished vision 4. Ptosis 5. Lagophthalmos 6. Squint 7. Diplopia 8. Exposure keratitis 9. Loss of corneal sensation 10. Rise of intraocular tension 11. Rise of intra orbital tension 12. Marcus Gunn pupil 13. papilledema 14. Optic atrophy 15. Central vein stasis 16. Central retinal artery occlusion 17. Choroidal fold 18. Change in refraction 19. Pressure changes in the bones 20. Systemic involvement All the signs and symptoms need not be present in all eyes at the same time. They may be present in various combinations. There is no fixed chronological order, some signs and symptoms may be of acute onset, others may be slow in progression. 1. Pain - Children have greater threshold of pain, a non-verbal child may cry in discomfort. Gradually progressing proptosis is generally devoid of pain. Some of the painful proptosis are :

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Accumulation of fluid under periorbita i.e. periorbital abscess or hematoma. Acute orbital cellulitis and abscess. Cavernous sinus thrombosis. Exposure of cornea is always associated with some degree of pain. 2. Conjunctival congestion and chemosis are very common when the child is unable to close the eye. Common causes of chemosis of conjunctiva are orbital cellulitis, panophthalmitis, almost all cases of proptosis have some degree of conjunctival congestion. 3. Diminished vision in proptosis can be due to following : (i) Error of refraction (ii) Corneal changes (iii) Fundus changes (iv) Squint (v) Amblyopia (i) Errors of refraction are brought about by compression of the globe at various sites. Pressure on the cornea by the lids produce astigmatism, pushing of the retina from behind resulting in to hypermetropia, distortion of the globe due to pressure from the orbital side produces irregular astigmatism. (ii) Amblyopia is caused by : (a) Over hanging lid over the cornea, corneal opacity cause deprivation amblyopia. (b) AstigmatismCommon causes of astigmatism areCapillary haemangioma, neuro fibromatosis, dermoids, epidermoids and dermolipoma. (iii) Changes in cornea results from exposure keratitis due to lagophthalmos. (iv) Fundus changes consist of optic nerve changes i.e. optic atrophy, venous congestion, rarely central retinal artery occlusion, retinal haemorrhages and fold in the choroid. 4. Ptosis is not a very consistent feature it can be : (i) Mechanical Neurofibroma Capillary haemangioma Orbital cellulitis (ii) Paralytic as part of - Paralysis of third nerve, isolated paralysis of third nerve is rare in proptosis. It may be an early manifestation of wide range of paralysis of other extra ocular muscle due to involvement of third, fourth and sixth nerve, may be associated with corneal hyposensation. 5. Lagophthalmos (Inability to close the eyes)It can be mechanical due to extreme degree of proptosis which does not allow the two lids to meet or due to paralysis of orbicularis. 6. Squint - Ocular deviation can be brought about by paralysis of extra ocular muscles, mechanical restriction of extra ocular muscle movement. Concomitant squint is generally associated with errors of refraction, opacity in media and optic nerve changes.

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7. Exposure keratitisExposure of the cornea is one of the most unpleasant complications that may lead to sloughing and perforation of cornea. It is caused due to inability to close the lids and diminished corneal sensation, any of these alone or in combination with infection cause corneal involvement. Post keratitis corneal opacity is a cause of persistent diminished vision. 8. Loss of corneal sensation is met in cavernous sinus thrombosis, superior orbital fissure syndrome and pseudo tumour of the orbit where fifth nerve is involved along with other cranial nerves. 9. Rise of intra ocular tensionGlaucoma in proptosis is mostly due to extra ocular causes i.e. increased episcleral pressure, pressure of the globe from outside and restrictive squint. 10. Rise of intra orbital pressureThere is no known method to measure intra orbital pressure but clinically this happens if there is an expanding orbital lesion with intact orbital septum. 11. Marcus Gunn pupilPresence of Marcus Gunn pupillary reaction denotes involvement of optic nerve resulting in conduction defect in unilateral proptosis. 12. PapilledemaSwelling of optic disc denotes either raised intra cranial pressure, which is common in congenital anomalies of skull, extension of growth in to the cranial cavity or compression of central retinal vein. 13. Optic atrophyBoth primary as well as post papilledematous optic atrophy are possible. The first is seen in glioma of optic nerve while the second is noted as end result of papilledema. 14. Central vein stasis results in dilatation of retinal veins, brought about by many expanding lesions of the orbit. However full blown central vein thrombosis has not been observed. 15. Central retinal artery obstruction and optociliary shunts are rare phenomenon. 16. Change in refraction - Refractive errors are induced due to change in curvature of the cornea or due to pushing of posterior pole by a retrobulbar mass. Paralysis of third nerve leads to loss of accommodation. 17. Pressure changes in the orbital bones are evident on X-ray. 18. Some of the orbital disease may be associated with systemic disorders i.e. Storage disease, leukaemia, parasites, neuroblastoma, Ewings sarcoma, thyroid eye disease and secondaries. Evaluation of a case of proptosis Orbit is a Pandoras box that contains so many tissues. Involvement by diverse etiology and presentation make it difficult to pinpoint the exact nature of the disorder. Hence proptosis requires meticulous evaluation under following heads. 1. History 2. Ocular examination 3. Systemic examination

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4. Investigation 5. Therapeutic trial History Detailed history is one of the most important clinical methods in evaluation of proptosis. The child may be non-verbal, or may not co-operate. In such cases parents should be quizzed about the disorder, children have a tendency to hide history of trauma, they have greater threshold for pain. A child who has good sleep without analgesic and sedation most probably has mild or no pain. History should comprise of : 1. Age of onset 2. Mode of onset 3. Duration 4. Recurrence 5. Trauma 6. Pain 7. Diminished vision 8. Diplopia 1. Age of onset. Proptosis can be present at birth and the causes are invariably congenital. They are craniostenosis, meningocele, meningoencephalocele, and microphthalmia with congenital cyst. In infancy above conditions continue to be the cause of proptosis and may cause progression of proptosis. To this list are added following benign growth in first decade. Dermoid, epidermoid, haemangioma, neurofibroma. Lymphangiomas are generally seen after first decade. Average age for optic nerve glioma is seven years. Intra orbital meningiomas too manifest in early part of second decade. It must be kept in mind that all the above growths have congenital background. The malignant growths that cause proptosis in infancy and childhood belong to two distinct groups i.e. primary malignant tumours and secondaries. Commonest primary malignant orbital tumour in childhood is rhabdomyosarcoma followed by Burkitts lymphoma, Common secondaries are neuroblastoma, Ewing sarcoma, Wilms tumours leukaemias. Orbital retinoblastoma have a place of its own though orbital extension of retinoblastoma is a late feature that is preceded by latent stage and stage of secondary glaucoma. Sometimes there may be extension of retinoblastoma before stage of secondary glaucoma. Less common causes of proptosis are orbital varix and fibrous dysplasia. Though the list of new growths is long and impressive, infections are the foremost cause of proptosis in children. They areOrbital cellulitis, orbital abscess, cavernous sinus thrombosis, panophthalmitis, retained intra orbital foreign bodies. Orbital pseudo tumours manifest in late childhood, occasionally retro bulbar haemorrhage or emphysema may result in proptosis in children mostly due to trauma.

688 To summarise, causes of proptosis in childhood are17,18 I. Congenital

PEDIATRIC OPHTHALMOLOGY

1. Craniostenosis 2. Meningocele 3. Meningoencephalocele 4. Encephalocele 5. Microphthalmia with cyst 6. Dermoid 7. Teratoma

II. Traumatic

1. Retrobulbar haemorrhage 2. Emphysema 3. Retained orbital foreign body.

III. Infective 1. Acute (a) Orbital cellulitis (b) Orbital and periorbital abscess (c) Cavernous sinus thrombosis (d) Panophthalmitis 2. Chronic (a) Pseudo tumour orbit (b) Intraorbital parasitic cyst IV. Neoplasm 1. Benign (a) Dermoid and epidermoid (b) Capillary haemangioma (c) Neurofibroma (d) Lymphangioma (e) Optic nerve glioma (f) Orbital varices (g) Fibrous dysplasia (h) Intraorbital meningioma 2. Malignant (a) Primary Rhabdomyosarcoma and Burkitts lymphoma (b) Secondary Neuroblastoma, orbital retinoblastoma, Ewings sarcoma Wilms tumour leukaemia.

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V. MiscellaneousHand Schuller Christian disease, juvenile xantho granuloma, eosinophilic granuloma, sinus histocytosis. Mode of onset Acute onset and increase in proptosis is seen in orbital cellulitis, panophthalmitis, cavernous sinus thrombosis and retro bulbar hae-morrhage. Rhabdomyosarcoma, neuroblastic and leukeamic deposits have moderate to fast progression. DurationLong duration with minimal symptoms are seen in congenital causes and benign growths. RecurrenceRecurrence is common following treatment of rhabdomyosarcoma, neuroblastoma, leukaemia, retinoblastoma. Retinoblastoma treated by radiation are known to develop second non retinoblastoma malignancy. Trauma Frank trauma with laceration of lid, fracture base of the skull and orbit cause accumulation of blood in retro bulbar space. Accumulation of air results in orbital emphysema, which denotes fracture of paranasal sinuses. Retained intra orbital foreign bodies not only cause retro bulbar hematoma but also cause orbital cellulitis if the foreign body is infected and are known to develop foreign body granuloma. Pain Pain is seen in acute infective lesions like orbital cellulitis, orbital abscess, cavernous sinus thrombosis or any fast expanding mass. The pain suddenly subsides if the fluid spontaneously discharges outside the orbit via conjunctiva or in to the lid after perforating the orbital septum. Corneal involvement is a common cause of pain, lacrimation and photophobia. In case of unexplained pain, intraocular tension should be measured by tonometer. A non contact tonometer is preferred over contact tonometer. Diminished vision Loss of vision followed by proptosis is pathognomic of optic nerve glioma in children. However the child may not be aware of such painless loss and may not complain. Loss of vision with pain is due to corneal involvement. Diplopia Diplopia denotes early stage of paralytic squint or restrictive strabismus, which may subside due to loss of vision, or onset of deep amblyopia. Ocular examination VisionRecording of vision should precede all other examinations. In a non-verbal child clear, bright cornea, normal anterior chamber, brisk direct and indirect pupillary reaction, clear media, absent error of refraction and normal fundus invariably denotes good vision except in case of deep amblyopia and cortical blindness. In a slow progressive proptosis, vision is generally good and remains so for sometime. In glioma of optic nerve, visual loss is followed by proptosis, sudden painful loss of vision means

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corneal involvement. Sudden painless loss of vision is due to fundus changes that may be optic neuropathy, optic atrophy, haemorrhage in the retina or vitreous, vascular obstruction, choroidal fold or hypermetropia due to growing mass behind the globe that pushes the retina forward. Pupillary reactionA Marcus Gunn phenomenon denotes optic nerve compression. Cavernous sinus thrombosis and superior orbital fissure syndrome may result in dilated sluggish pupil. InspectionA well established proptosis is not difficult to diagnose. An early proptosis requires proper examination. In such case most important is to determine if the proptosis is real or apparent (pseudo proptosis). Pseudo proptosis is that condition where there is no pathology in the orbit. The pathology is either in the lid or eyeball. Sometimes the pathology lies in the contra lateral eye in the form of enophthalmos. The causes in the lid that mimics proptosis areLid retraction and lagophthalmos in the ipsilateral eye or ptosis, pseudo ptosis of the other eye. Ocular conditions that may be mistaken as proptosis arePathological myopia, buphthalmos, large ciliary staphyloma, in the eye under examination. Contra lateral microphthalmos, phthisis or enophthalmos are also sometimes mistaken as proptosis of the ipsilateral eye. Next step involves finding out laterality of the proptosis i.e. is at unilateral or bilateral. If bilateral, is it equal in both eyes or advanced in one eye. Causes of unilateral proptosis in children are : Orbital cellulitis, orbital and peri-orbital abscess, early stage of cavernous sinus thrombosis, rhabdomyosarcoma, pseudo tumours, neurofibroma, capillary haemangioma, lymphangioma, dermoids, retro bulbar haemorrhage, rarely congenital. Causes of bilateral proptosis in children are : 1. Mostly systemic condition 2. Congenital anomalies of the orbit Systemic conditions that cause bilateral proptosis areneuroblastoma, leukaemia, bilateral retinoblastoma, second malignancy following radiation for retinoblastoma, fully developed cavernous sinus thrombosis. Measurement of proptosis Relative position of cornea in relation to orbital margin gives clue regarding proptosis of ipsilateral eye, enophthalmos of the other eye, extent of proptosis when present and its progression. This can be done clinically by noting the position of corneal apex in relation to superior orbital margin. It can be conveniently done in a co-operative child. The examiner stands behind the child, asking the child to tilt the head backwards and fix at a distant object keeping both eyes open and noting the position of the cornea in relation to superior orbital rim. Normal corneal apex is generally not visible beyond the superior orbital margin. A visible cornea beyond orbital rim denotes proptosis. A false impression is given by high myopia. 3D myopia is equal to 1mm enlargement of globe in anterio posterior diameter. The other method consists of actual measurement of distance between the corneal apex and the lateral orbital

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margin. There are various types of exophthalmometer (Proptometers) to measure this distance, they are - Optical and mechanical. The optical exophthalmometer is more popular and widely used than mechanical. They are Leuddes transparent scale and Hertels mirror proptometer. Normal exophthalmic measurement vary between a wide range of 10 to 20 mm, average is taken as 15 mm. Any reading higher than 21 mm is considered as definite proptosis. The other criteria is any difference of more than 2 mm between the two eyes provided the other eye is normal. An enophthalmic contra lateral eye gives a false positive impression. CT pictures can also be useful in evaluating extent of proptosis. Inspection 1. Position of upper lid. Normal lid covers upper one fifth of the cornea and lower lid just touches the lower limbus. No strip of sclera is visible either above or below the cornea. Visible sclera above, below or both denotes proptosis or lid retraction. Generally lid retraction is associated with lid lag. Lagophthalmos gives an impression of pseudo proptosis. All cases of lid retraction with or without clearcut proptosis should be investigated for dysthyroid eye disease. Incidence of dysthyroid orbitopathy is far less in children than seen in adults. 2. Direction of proptosis. Abnormal position of globe gives clue to location of orbital pathology in relation to globe. The globe may be displaced : 1. Centrally (axially) or 2. EccentricallyEccentric proptosis may be unidirectional or otherwise. The causes of axial proptosis lies in the muscle cone. Commonest causes in children are being glioma of optic nerve. Other causes are foreign body in the muscle cone, orbital varices, pus localised in muscle cone. A growth from the medial wall displaces eye laterally. Pathology from the floor pushes the eyeball up. However the displacement always does not follow such simple rule. A growth on the superio medial aspect pushes the eyeball forward, downward and laterally. Similarly a growth from superio temporal orbit shifts the globe down, out and medially. 3. Visible pulsation. Sometimes the proptosed eye may have pulsation synchronised with heart beat. Gross pulsation is evident without magnification. However slit lamp examination may show very mild pulsation that later becomes evident without biomicroscope. Such pulsations are more common in adults, however pulsations are met with in neuro fibromatosis when associated with defect in sphenoid, fibrous dysplasia and rarely in post traumatic carotico cavernous fistula. 4. Cough impulse is visible and palpebal in cases of encephalocele and meningo encephalocele. 5. Palpation (i) Palpation of the orbital marginNormal orbital margin is smooth without any notch or protuberance except superior orbital notch and the trochlea occasionally. The medial palpebral ligament is palpable as a horizontal band, this is also a normal feature.

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Palpation or orbital margin may reveal irregularity, bone formation or bone dehiscence. Growth arising from bony sutures i.e. dermoids can not be palpated separately from orbital margin. (ii) Palpation of orbital massAnteriorly placed growths are easily palpable. While palpating a mass, if a finger can be insinuated between the mass and the orbital wall, the growths is not arising from the orbital wall or the rim. The finger can not be insinuated between the growth and the wall in case of encephalocele, meningocele, dermoids, mucocele from the paranasal sinuses and lacrimal gland growth. Lacrimal gland tumours are not met within children. Compressibility of the proptosis The child is asked to close the lids and the mass is pushed back through the closed lid by first three fingers of the examiner. This should be avoided when tenderness of globe is present. A compressible mass means a soft or vascular growth, solid tumours cause retro bulbar resistance. Valselva maneuver, juglar vein compression and head bending None of the above procedures change the position of the eyeball in any proptosis unless it is caused by vascular anomaly like capillary haemangioma and congenital varices. In head bending test, the child is asked to bend the head and trunk forward for about a minute and then come to normal position. The position of the globe is measured, an increase in proptosis is considered positive denoting a vascular tumour. Forced duction test20 This test, when performed properly differentiates obstructive squint due to fibrotic contracture from neurological defects. In a co-operative child this can be done as outpatient procedure under local anaesthesia, otherwise it should be done under short term general anaesthesia. The test consists of : 1. Anaesthetise the conjunctiva by instilling local anaesthetic agent in both the eyes for two to three times. If the child feels pain, a cotton swab dipped in same anaesthetic solution may be left at the insertion of the muscle to be tested for three to four minutes. 2. Ask the patient to look in the direction of limited movement. 3. Grasp the insertion of the muscle to be tested with a conjunctival fixation forceps, move the globe passively in the direction of limited movement. 4. The procedure is repeated in the other eye and findings are compared. A test is considered to be positive if the eye can not be moved passively in the direction of limited duction and the cause is fibrotic contracture of the muscle or entrapment of the muscle in a fracture. If the eye can be moved passively in the direction of limited action, the test is called negative and the cause of limitation is neurological. 5. While performing the test, care should be taken not to push the globe backward deep in the orbit otherwise there will be no limitation in presence of fibrotic band.

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In children the test is frequently used in case of superior oblique sheath syndrome, Duanes retraction syndrome, entrapment of muscle in fracture orbit or in case of strabismus fixus. The test is most frequently used in adults in case of thyroid myopathy. When the child does not allow forced duction test under local anaesthesia, a less painful test is differential intra ocular pressure test. Intraocular pressure is noted in usual manner in primary gaze. The patient is asked to look toward the field of action of the muscle under question and tension is repeated. A rise of 6 mm denotes fibrosis or entrapment of the muscle. No rise of tension or rise less than 6 mm is suggestive of neurological cause. Special investigation in proptosis They consist of : 1. Orbital imaging A. Non invasive (a) Plain x ray (b) CT (c) MRI (d) Ultrasonography (e) Venography (f) Arteriography B. Invasive (a) Fine needle aspiration (b) Biopsy (c) Surgical excision and histopathological examination 2. Rhinological consultation 3. Blood picture 4. Blood chemistry 5. Systemic examination Radiology of orbit in relation to proptosis Posterio anterior view without angulation of head or beam, has very limited value in evaluation of orbital lesions. The petrous part of the temporal bone and the para nasal sinuses get superimposed on the soft fissure of the orbit and obscure their view. As most of the anatomic landmarks form various degree of angulation in anterio posterior axis, they are required to be positioned in such a way that they do not interferes with the structure of radiological interest. Commonly used X-ray views are : 1. Caldwell view (a) View with central X-ray tilted 25 downward (b) Hypoangulated viewCentral X-ray is tilted less than 15 2. Waters view 3. Lateral view

694 4. X-ray for optic canal and foramen (i) Rhese view (ii) Ruggiero 5. Axial basal view 6. Submento vertex view 7. Townes view.

PEDIATRIC OPHTHALMOLOGY

1. Caldwell view. In this view the patient lies face down, the forehead and the nose touching the X-ray table with X-ray tilted downward. (a) at 25 and (b) 15. The advantage of this projection is that the petrous part of temporal bone is shifted down so that the orbital rim, roof, greater wing of sphenoid, lesser wing of sphenoid, the superior orbital fissure are clearly visible. The foramen rotundum is shifted below the lower rim of the orbit. The greater wing forms a larger part of lateral wall, the lesser wing of sphenoid is seen near the medial wall. The superior orbital fissure is seen in between the two wings. It also shows frontal sinuses, sphenoid ridge, floor of sellaturcia and innominate or oblique orbital line. An angulation less than 15 gives better view of some of the above structures. 2. Waters view. This is also a posterio anterio projection. The patient lies prone on the table with the chin resting on the table and nose lifted 4 cm off the table. This makes the canthomental line form an angle of 37 to the central X-ray, which is at right angles to the Xray plate. It gives a clear view of maxillary, frontal and ethmoidal sinuses, the lateral wall, zygomatic arch and inferior orbital rim. This is useful when the pathology is in the sinuses and fracture of the orbit is suspected. 3. Lateral view The X-ray plate is kept parallel to the sagital plane, the central ray is at right angle to this plane that passes 2.5 cm in front and 2 cm above the external auditory meatus. This projection shows anterior and posterior clinoid process. The sellaturcia, sphenoid sinuses, intra orbital canal. Part of the floor of the orbit is also visible. The projection is used to localise intraocular, intra orbital foreign bodies, calcification and pathology in sella. 4. Ray of optic canal and foramen (i) Rhese view. This requires two separate exposures, one for each orbit. This is used to visualise the optic foramen and optic canal. The patient lies prone on the table with zygoma, nose and chin on the table. The mid sagittal plane of the skull makes an angle of 53 with the film. The central ray is projected posterio anteriorly with 12 angulation towards feet. (ii) Ruggiero view21. These projections show optic canal, optic foramen, ethmoid cells, lesser wing of sphenoid and superior orbital fissure. 5. Axial basal view shows ethmoid and sphenoid air cells. 6. Submento vortex view is taken on a supine patient. This view shows base of the middle cranial fossa, the maxillary sinus, and the nasal cavity.

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7. Towens view shows infra orbital fissure. This view is exposed when the patient is supine and the X-ray is projected in anterio posterior direction. Points to be noted of a X-ray of orbit : The radiologist should see the patient and the ophthalmologist see the X-ray. Single projection may not be sufficient to give all the required information. X-ray of both the orbits should be taken, this helps in comparing changes in two orbits especially in unilateral proptosis and a systemic bilateral proptosis. 1. Intraorbital distance. The intra orbital distance refers to distance between right and left junction of frontal process of maxilla and maxillary process of frontal bone. The two medial walls of the orbit are parallel to each other and the distance between the two varies between 12.5 mm in child of one year to 25 to 28 mm at 12 years of age. If the orbits are set apart, the condition results in hypertelorism. The cause of which are Crouzons disease, Aperts syndrome, fibrous dysplasia, osteogenesis imperfecta, encephalocele. The intraorbital distance may be as much as 30 to 40 mm in adult.22 Hypotelorism When the intra orbital distance is less than expected normal value of a given age. This is seen in Downs syndrome, trigonocephaly and craniofacial anomalies. Premature closure of cranial sutures This is associated with deformity of skull, expansion of the skull at the right angles to the closure of suture, shallowing of the orbit, rotation of wall of the orbit and increased digital marking commonly seen in craniostenosis. Widening of cranial suture Normally a cranial sutures should close at 18 months of age. However if the intra cranial tension rises before the sutures are closed, the skull expands due to increased intra cranial pressure widening the cranial sutures, in such cases no digital markings are seen on the bones of the skull. However, if there is rise of intra cranial pressure after the sutures are closed, X-ray evidence of raised intra cranial tension will be observed. If some of the sutures are closed, remaining sutures will open up; the example is craniostenosis. Enlargement of the orbits Size of the normal eyeball is essential for the growth of the orbit. In most of the cases of proptosis the size of the eyeball is within normal limits, other orbital structures force the orbit to expand. However a large eyeball itself can cause enlargement of the orbit. The enlargement of the orbit can be unilateral and bilateral. Unilateral enlargement is more rapid in children. The orbit of a child takes two to three months to show enlargement while similar enlargement in adults take 1 to 2 years. Unilateral enlargement can be symmetrical or asymmetrical. Unilateral buphthalmos either primary or secondary produce enlargement of orbit. Retinoblastoma produces more and frequent enlargement than primary buphthalmos, other causes of unilateral symmetrical enlargement are due to intra conal lesions i.e. optic nerve glioma, neuro fibromatosis, haemangioma, extra ocular retinoblastoma inside the muscle cone and intra conal pseudo tumour. Causes of asymmetrical enlargement of orbit

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are - haemangioma, extra conal retinoblastoma, neurofibroma, dermoid, rhabdomyosarcoma, orbital dysplasia, microphthalmos with cyst, congenital teratoma. Diminished size of orbit Orbital size may be smaller than normal either due to small or absent eyeball, a growth encroaching from orbital wall or craniostenosis. The common causes of small orbit areAnophthalmos, microphthalmos, early enucleation of the eyeball, post radiation. Mucocele of the frontal sinus and growth from maxillary sinus (rare in children) also reduce size of the orbit. Changes in shape of orbit Orbital shape can be deformed as part of craniostenosis, orbital dysplasia and dermoids. Changes in the bone The changes in the orbital bones can be : 1. Increased density 2. Decreased density 3. Destruction of bone 4. Fracture of the orbit 1. Density of bone is increased in osteoblastic metastases, chronic periostitis, fibrous dysplasia. 2. Decreased bone density is seen in dermoid. 3. Dehiscence of bone is seen in neuro fibromatosis, mucocele and fracture of roof and floor. Calcification in the orbit Intra orbital calcification may be confined to intra ocular structure, extra ocular structure or both. In posterior anterio view, intra cranial calcification like cranio pharyngioma, congenital toxoplasmosis, Sturge Weber syndrome may be superimposed on orbit, however a lateral exposure separates intra orbital calcification from intra cranial calcification. Causes of intraocular calcification areRetinoblastoma, parasitic cysts, choroidal bone formation, complicated cataract, retrolental fibroplasia, phthisis blubae. Extra ocular calcification is seen in orbital retinoblastoma, orbital varix, optic nerve glioma, parasitic cysts, haemangioma, neurofibroma. Dermoids get calcified occasionally. Changes in superior orbital fissureThe superior orbital fissure widening is more common than its narrowing which is brought about by encroachment of growth over the fissure and as a congenital malformation. Widening of the superior orbital fissure is generally seen in adults as part of intra clinoidal aneurysm. Intra cavernous aneurysm, carotico cavernous fistula, extension of intra orbital growth backwards or spread of growth from cranium. Sometimes neurofibroma and pseudo tumours of orbit may also widen superior orbital fissure.

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Changes in optic canal and foramen Usual diameter of optic canal varies between 4.4 to 6 mm in adults. At birth the canals are narrower, reaching the adult size by 5 years of age, diameter greater than 7 mm is always pathological. Generally diameters of both the canals are more or less similar. Any difference of more than 1 mm between the two canals is also pathological. For comparison both the canals should have identical X-rays preferably taken on the same plate. The enlargement may be concentric or irregular. Uniform concentric uniocular enlargement is seen in optic nerve glioma. Other causes in children are neuro fibromatosis, intra neural extension of retinoblastoma. In children neurofibroma and retinoblastoma cause uniform irregular enlargement. Computed tomography23 Computed tomography has emerged as an important tool in orbital imaging. It is safe with minimal radiation hazard, which is as less as in X-ray. It is a non invasive outdoor procedure which uses computer assistance. X-ray Tomography is axial as well as coronal in section. A combination of axial and coronal section when programmed enables a lesion to be seen in three dimensions. Its use in orbit is based on ability in differentiating between relative tissue densities. Retrobulbar fat is in plenty in orbit and surrounds many structures. The retrobulbar fat is hypo dense on X-ray in relation to other structure, hence a good contrast is ensured. The bones have maximum density, the orbital fat and vitreous have least density, other structures have intermediate densities. The vascular structures are best visualised by using intravenous iodine containing contrast dye.24 Besides orbit the intra cranial and paraorbital structures are also well delineated in the same exposure. This enables to visualise growths extending from the orbit into the cranial cavity and vice versa. It also demonstrates other intra cranial lesions not directly related to the orbital lesion. The structures well visualised are the walls of the orbit, optic canal, optic nerve, extra ocular muscles, globe, intra ocular mass. Intra ocular, orbital and cranial foreign bodies. It is very sensitive in detecting orbital, intra ocular, and cranial calcification. Fractures are well demonstrated. The superior orbital fissure is better visualised in contrast to inferior orbital fissure. The sclera is demarcated clearly. It is not possible to differentiate the layers of the globe separately in normal eye. However anterior chamber, lens and vitreous chambers are seen separately. Generally 4-5 mm sections are taken routinely from vertex to base of the skull, parallel to the optic nerve. The coronal plane is parallel to equator of the globe similar to Caldwell view of X-ray. The computerised tomography can not differentiate between pathological and normal tissue of same density. The walls of the orbit are clearly seen in both axial as well as coronal slices. The roof and floor are seen better in coronal section so are the superior, medial and lateral recti. The levator palpebral superior is not very clearly seen. All these muscles are seen as elliptical masses in coronal section, in axial view they have fusiform shape. The optic canal, intra orbital part of the optic nerve, sometimes its meningeal covering are seen in axial view. The medial and lateral recti are seen as dense masses extending from equator of the eyeball to the apex of the orbit. Computed tomography is ordered in children to evaluate orbital soft tissue mass, intra ocular and intra cranial calcification. Evaluation of trauma, intra orbital and intra cranial foreign bodies.

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Common intra orbital lesion where CT is used are24 : 1. Retinoblastoma of all stages, post radiation status, recurrence and intra cranial extension. 2. Rhabdomyosarcoma 3. Capillary haemangioma is best seen in contrast, may show calcification. 4. Optic nerve glioma with or without neurofibroma 5. Dermoids and teratomas 6. Pseudo tumours 7. Orbital cellulitis CT may be a good diagnostic tool in optic neuritis. Moderate and symmetric enlargement is seen in raised intra cranial pressure. In children dermoid cyst, rhabdomyosarcoma, optic nerve glioma, lymphangiomas give well circumscribed and sharply defined appearance. Capillary hemanioma, pseudo tumours, plexiform neuroma, leukaemia give ill defined view.25 Non ocular causes of loss of vision in one eyeoptic neuritis, uniocular papilledema, ophthalmoplegia of unexplained origin. Conformation of ultrasonic findings are some other indications of CT of orbit. Magnetic Resonance Imaging (MRI) Magnetic resonance imaging is a non invasive method of imaging that does not use ionising radiation used in common X-ray and computed tonography. The principle of MRI is that thin anatomic section similar to CT are viewed by exposing the patient to an intense magnetic field26. Besides magnetic field, a radio frequency wave and an atomic nuclei possessing an electric charge should also be available. Hydrogen atoms is used to generate MR signals because of its abundance in the body. The magnetic field is measured in a unit called Tesla (T). Most of the MRIs uses 0.1 to 1.5 T of magnetic field. The process is not hampered by bone. It can very well differentiate between normal and abnormal tissue due to its high resolution. The metabolic profile of the tissue is also obtained. Indications of MRI are ocular, orbital and neuro ophthalmic. The ocular indication consists of ocular trauma, suspected nonferro magnetic intra ocular foreign bodies, growths obstructed by opacity in media. In such cases first choice should be ultrasonography but if ultrasonography findings are equivocal, the eye should be subjected to CT and MRI. Latter gives better view due to excellent resolution. The MRI can differentiate between retinoblastoma and intra ocular haemorrhage, exudates, Coats disease, choroidal efflusion and toxocariasis. Papilledema, optic neuritis, lens, vitreous and sclera do not normally enhance on post contrast MR.29 Orbital indication areProptosis of any kind, orbital cellulitis, pseudo tumours, neoplastic growths. Haemangiomas are well visualised in dye contrasted MRI. Magnetic resonance imaging is better than CT in evaluation of intra canalicular lesions. Orbital fat and optic nerve sheath do not enhance. MRI does not image bones well. MRI is contra indicated in suspected fero-magnetic foreign bodies. It should only be performed when such foreign bodies have been excluded by CT. MRI induced blindness in presence of metallic foreign body has been reported.29A

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Neuro ophthalmic indications consists of : Optic neuropathy of unknown origin, unexplained cranial nerve lesions, multiple sclerosis, paralytic squint, visual field defects not related to optic neuropathy. Lesions of sellaturcica, cavernous sinus and optic chiasma. It is widely used in suspected extension of retinoblastoma in the intra canalicular part of optic nerve and chiasma. Some of the important indication of MRI in children areevaluation of proptosis of sudden origin, suspected optic nerve sheath - complex lesions, organic foreign bodies, retinoblastoma with extra ocular extension. In children dermoid, rhabdomyosarcoma and glioma of optic nerve give well circumscribed image while capillary haemangioma, pseudo tumour, plexiform neuroma and leukaemia give ill defined image. The child may have to be sedated if not cooperative. Advantages of MRI are : 1. The process is devoid of radiation hence it is safer than CT and X-ray. The child can be exposed to MRI for long period without any harm. 2. The procedure is not hampered by boney orbit. 3. Soft tissue image is seen better. Ultrasonography with special reference to orbital diseases in children General Ultrasonography is one very important non invasive, non traumatic method. It does not use ionic radiation or magnetic field as used in X-ray, CT or MRI. Ocular ultrasonography does not have any side effect, can be used very frequently. It can be done as office procedure, bed side examination or in the operation theatre as this is a small handy instrument. It can be used in children with ease. However a non co-operative child should be suitably sedated by a trained anaesthetist. Ultrasound Speed of sound in vacuum is far less than that of light which is 3x1010 cm/sec or 186,000 mile/second. Ultrasound is produced in the transducer mechanically at a rate of 20,000 cycle per second (20 kHz). This is not audible to human ears. Diagnostic ophthalmic ultrasonography uses 10 omega hertz (10Mhz). Ultrasonography of other parts of the body use 2.5 to 5 MHz. Higher the frequency of ultra sound, lower is the penetration. Thus, to measure corneal thickness or depth of AC 12-15 MHz are used while that for globe is 10 MHz and orbit is 7 MHz. The ultrasound behaves more or less like light wave, it is reflected, absorbed and refracted. Maximum ultrasonic waves are reflected when it falls at right angles. Ultrasound can also disrupt a target tissue and generate heat, the later two are used in treatment and not diagnosis. Basically a diagnostic system consist of (1) a tranducer, (2) a black box that detects the reflected beam and prepare for display, (3) Display. The transducer (The probe) This part of the system is most important and has been called the heart of the system. It is both a transmitter as well as a receiver. The transducer is made up of either quartz crystal or a ceramic plate, which send out sound wave when charged electrically. The same crystal picks up the returning waves and prepare it for visual display and photography. The

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mechanism is called piezoelectric effect. This converts one form of energy to another form, in ultrasound it converts electrical energy to sound energy, which is again converted to visible electric form. The transducer of A scan is called stationary because there is no moving part inside the probe. Though the probe itself is moved by the examiner to scan various parts of the eye. In the transducer of B. Scan, the crystal inside the probe moves to and from. The pulse is focussed and directed into the ocular tissue. The pulse after striking the tissue travels back to the probe. This process is repeated may times each second. The distance of the target tissue can be calculated by noting the time to travel the echo from the tissue, if the velocity of the sound is known. In A scan this is processed by a built in computer that automatically measure the distance and displays on the screen, which can be photographed for future reference. The resolution depends upon the frequency. Higher is the frequency, greater is the resolution but as frequency increases penetration decreases.30 The electronically amplified sound wave signal received by the probe is called its sensitivity or gain. When the sensitivity is more, the spikes of A mode are taller and the dots of B mode are brighter. Other factors on which the height of A Scan and brightness of B. Scan depend are angle of sound beams when it strikes the tissue, relative difference between the two tissues, the size and shape of the inter-face. Sometimes some echoes are noted during ultrasonography, which are not produced by anatomical structures, these are called artifacts. Commonest artifact is an air bubble either in the probe itself, in the coupling medium used during ultrasonography or in the eye. Air bubble can get into the eye either by accident as in penetrating injury. It can be produced by gas forming organism or may be introduced deliberately as part of surgery. Intra ocular foreign bodies sometimes bounce back and forth waves inside the eye causing a chain of echoes behind the foreign body. This phenomenon is called reverbation. The foreign bodies that produce such artifacts are - intraocular lens, glass particles or steel pieces. Air is a potent reflector of sound in case of a penetrating injury. It is often difficult to differentiate echo produced by retained intra ocular foreign body and air bubble.31 Modes of display30 There are two modes of display on screen that can be photographed as well. They are A and B modes also called A Scan and B Scan respectively. A Scan. A scan is a simple instrument but findings are more difficult to interpret. This mode is called time amplitude. The picture produced is unidimensional. The spike has a base line and a vertical deviation. The spike is a triangular configuration, base of which is formed by the base line and the point where two slanting arms meet is the apex. The distances between the two arms on the base line is called width of the spike. The left arm is called ascending limb while the other arm is called descending arm. The height of each vertical deflection is proportional to intensity of the echo. The base line represents the distance and depends on the time required for the sound to reach the target, to be bounced back to reach the probe.

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A Scan is used to find out axial length of eyeball, AC depth and corneal thickness. It is most widely used to calculate power of the intra ocular lens. However intra ocular growths, intraocular foreign bodies and retinal detachment can also be seen by A mode. The difference between anterior corneal echo to retinal echo in a normal eye is the axial length of the eye. The cornea gives two spikes, one from the anterior surface and other from the posterior surface as the cornea is relatively thinner than other structures i.e. lens, IOL. The distance between the two is very small. The lens when clear also gives two sharp spikes, one from the anterior surface and other from the posterior surface in case of homogenous lens. In case of nuclear sclerosis, two additional echoes represent anterior and posterior surface of nucleus. In an aphakic eye, the corneal spike is followed by the retinal spike, however, if the posterior capsule has been left it gives one additional spike. Occasionally the aqueous, vitreous interface may also give a spike. A clear vitreous is echo free and gives widest gap between the two peaks. Floaters, haemorrhages, endophthalmitis, foreign bodies produce echo inside vitreous. A high sharp echo from the retina denotes a normal retina. To measure the axial length, the beam should pass through the centre of cornea and macula. A posterior staphyloma can also be seen on A Scan. B Scan. This is a two dimensional display that is shown as dots of various brightness. Clinical interpretation depends on the brightness of the dot. A structure that produces a tall spike on A Scan will give bright picture on B Scan as well. The B Scan is said to reveal not only geometry of the structure but also its geography in the form of shape, size and position. It gives the information in all quadrant as the probe is moved all over the globe including the axial length. However for power calculation, A Scan should only be used. It is more useful than A Scan in diagnosis of vitreous opacity, vitreous haemorrhage, retinal detachment, intra ocular tumour, intra ocular calcification. B Scan is used to detect path of intraocular foreign body and occult rupture of the globe. Following conditions show calcification on ultrasonography(All ages) Retinoblastoma, optic nerve sheathmeningioma, osteoma, choroid, haemangiomachoroid, toxocara, granuloma, chronic retinal detachment, drusen of optic nerve head, phthisis bulbae, intumescent and hyper mature cataract asteroid bodies in the vitreous. Orbital inflammatory disorders in children Orbit in children may have acute or chronic inflammation of contents of the orbit, periorbita or bones. Acute orbital inflammation is generally unilateral. Anatomically infections of orbit may be preseptal or orbital. A useful classification of orbital infection based on anatomical spaces is 1. Preseptal or periorbital 2. Orbital cellulitis 3. Orbital abscess 4. Periorbital abscess 5. Cavernous sinus thrombosis Preseptal orbital cellulitis (cellulitis lid) is very common, easy to diagnose and treat unilateral inflammation limited by skin anteriorly and orbital septum behind. Generally the

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orbital contents are not involved. Pre auricular and sub mandibular glands are enlarged. Common organism that produce preseptal cellulitis are streptococcus, staphylococcus, pneumococcus and haemophilus influenzae. The last organism is more common in children below 3-4 years. It is associated with systemic involvement especially respiratory tract. It can prove to be both sight and life threatening. Predisposing factors in preseptal cellulitis areinjury, insect bite, stye, acute sinusitis. The symptoms are mild, may have fever and malaise. It is more frequent in upper half than lower. Clinical pictures depend upon duration and severity of the condition. In a fully established case, the lid is swollen, hyperemic and tense, obliterating in palpebral fissure. It is impossible to evert the lid. In case of H. influenzae, the skin has a bluish pink tinge. There may be tender points on the lid margin denoting a stye. The swelling may spill over the midline, causing edema of the other side without infection. The pre auricular and sub mandibular lymph nodes are enlarged. Most of the time lid retractors are required to examine the eyeball. The eye is generally non congested, the cornea is bright and papillary reaction is normal, movements are not restricted and the vision is not reduced. Treatment consist ofHot fomentation, oral analgesic oral antibiotics in divided doses. Complications are formation of a lid abscess and rarely cavernous sinus thrombosis. If a lid abscess develops fluctuation it should be drained through a horizontal incision and the pocket of pus broken. The wound may be packed with cotton gauze impregnated with antiseptic for twenty four hours. Periorbital cellulitis This is generally periostitis, there may be bony involvement9.36, 37. It may be peripheral, inflicting the orbital margin or may develop deep in the orbit near the apex of the orbit. The infection reaches the periostium either via veins that drain the paranasal sinuses or due to direct spread from the paper-thin walls of the sphenoid and ethmoidal sinuses. It may result following compound fracture of the orbital rim or due to penetrating injury. There are two distinct types : 1. An acute stage due to strepto, staphyloor pneumococci or haemophilus influenza. This presents as tense swelling due to accumulation of pus between the rigid bony wall and periostmus. This is a painful condition, may push the eyeball to the opposite side, cause mild proptosis, is generally confused with acute orbital cellulitis. The diagnosis is confirmed by CT and MRI. The condition may subside following administration of broad spectrum antibiotic without any residual effect or the pus may find its way to the orbital margin, conjunctival fornix, inside the orbit or even the cranium. This may lead to a sinus formation. 2. The deep type of periostitis is associated with proptosis, extra ocular muscle palsy and optic nerve involvement generally mistaken for cavernous sinus thrombosis. The chronic variety is generally seen in the orbital margin due to trauma, tuberculosis, unresolved acute peri orbititis. It presents with hard tender swelling on the orbital margin that is fixed to the bone. There may be an external sinus or a fistula. Sinus formation is more common with tuberculosis.

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Treatment of the chronic tubercular lesion is treatment of the primary cause. Other causes require long term broad-spectrum antibiotic, drainage of the abscess and curettage of the sinus. Orbital cellulitis32,33 Orbital cellulitis is common in children. It may be seen at any age but common at about five years.38 It is an acute purulent infection of contents of orbit that may involve more than one structure. It is a potentially sight threatening and sometimes may lead to loss of life due to involvement of central nervous system and septicimia.35 It is an unilateral condition caused by strepto, staphylo and pneumococci. It is generally single organism infection. H. influenza has emerged as a main causative organism especially in children under three years of age. The commonest route for organism to reach the orbit is via paranasal sinuses. All paranasal sinuses can cause orbital cellulitis, however, ethmoidal sinusitis is the most frequent source.39 Other common route is vascular spread from the sinuses, yet another mode of spread from the sinuses is dehiscence of the orbital wall of the sinus or its fracture. Generalised septicimia in a malnourished child and infection following penetrating injury can also introduce infection from outside. Occasionally septic teeth or acute dacryocystitis has also been observed to cause orbital cellulitis. The infection is always acute. It starts with symptoms of fever, malaise, pain in the periorbital region and headache. The lids are swollen, in case of H. influenza the lid has a bluish red hue. The lid itself is not primarily involved as the infections start in the orbit and is localised behind the orbital septum. Accumulation of pus, inflammatory substances and edema in a closed compartment cause sever pain, the eyeball is proptosed. As ethmoid and sphenoid are common source of primary infection. The eyeball is proptosed forward and laterally. The movements are restricted either due to edema of the muscles or paralysis of extra ocular muscle due to cranial nerve involvement. The conjunctiva is chemosed, however, the cornea is bright and AC does not show any reaction. Evidence of anterior chamber reaction denote uveal involvement. Dilated pupil is common with third nerve involvement. Fast deterioration of vision should warn about involvement of optic nerve that may be optic neuritis, central artery occlusion or central vein thrombosis. Investigation show : 1. Leucocytosis 2. X-ray orbit shows a hazy orbit with hazy paranasal sinuses 3. CT and MRI shows fluid and air in the sinuses 4. Ultrasonography helps to differentiate other conditions that cause fast growing proptosis in a child. Swabs from conjunctiva or nasopharynx are seldom conclusive. Differential diagnosis consist of cellulitis lid, cavernous sinus thrombosis, rhabdomyosarcoma, leukimic deposits, orbital deposit of neuroblastoma, retained intra orbital foreign body. Complications may vary from discharging sinus on the orbital margin to meningitis, brain abscess, cavernous sinus thrombosis. If the cornea is exposed due to proptosis, cornea may develop ulceration that may slough and perforate. Occasionally tenonitis may develop.

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As the condition is potentially life and sight threatening efforts should be made to save both in consultation with ENT specialist and pediatrician. Treatment of choice is parentral administration of ampicillin in divided doses. If there is chance of penicillinase producing organism, the antibiotic should be methacellin. Other antibiotic generally used arenaficillin or oxacillin41. With intense antibiotic administration the condition should subside in five to seven days. If the condition does not subside CT should be repeated to locate the pus and drain it. The abscess is drained through an area that shows maximal fluctuation near the lid margin avoiding trochlea, superior oblique and lacrimal gland. Proptosis, chemosis of conjunctiva and lateral rectus palsy of the contra lateral eye strongly suggest cavernous sinus thrombosis. Orbital and sub periosteal abscess38,42 Primary abscess of orbital and periostial space is extremely rare. It is generally secondary to non resolving orbital and periosteal cellulitis. Accumulation of pus among the contents of orbit is known as orbital abscess and pus under periostium is called as sub periosteal abscess. The organisms are same as that cause orbital cellulitis and sinuses are generally the source of infection. There is always some proptosis depending upon the size and location of abscess. Periosteal abscess generally leads to eccentric proptosis. An orbital abscess in the vicinity of optic nerve can cause purulent optic neuritis and retinal vascular obstruction. If the lids fail to protect the cornea, there may be exposure keratitis, ulcer and perforation. Diagnosis is conformed by CT and MRI. Treatment consists of change of antibiotic and its dose. If there is palpable fluctuation, the abscess should be drained through the maximum area of fluctuation. Posteriorly placed abscess are best drained via ethmoid. Cavernous Sinus Thrombosis To understand the pathology of cavernous sinus, it is essential to know general outline of applied anatomy of cavernous sinus. The cavernous sinuses are situated on either side of the sphenoid bone. The body of the sphenoid lies between the right and left halves of cavernous sinus. The sellaturcia lies above the sphenoidal sinus. The cavernous sinus starts at the end of the superior orbital fissure and ends posteriorly near the apex of petrous bone. The Gasserian ganglion comes in close proximity of the posterior end of the sinus. The sinus is formed by splitting of the dura and is traversed by trabeculae that gives it a spongy look. The sinuses of each side communicate with the sinuses of other side by transever sinuses of cavernous sinus. The walls of the trabecular space are rough, making is vulnerable for thrombus formation. Each cavernous sinus is formed by superior ophthalmic vein, central retinal vein, part of inferior vein and spheno palatine sinus. Thus, the cavernous sinus is the reservoir of venous drainage from scalp, face, orbit and most part of the cerebrum. The superior and inferior petrosal sinuses drain backwards. The petrosal sinuses drain into sigmoid sinus that finally drain into juglar vein. The tributaries of cavernous sinus are valve less and are prone for infection to travel both ways.42

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The contents of the cavernous sinus are internal carotid artery along with sympathetic plexus. The sixth nerve lies inferio lateral to the artery in the substance of the sinus making it more vulnerable than other cranial nerves. The other nerves lies in the lateral wall of the sinus in the posterior part. The third nerve is superior most followed by fourth and two divisions of fifth nerve. In the anterior part of the sinus, the fourth nerve changes its course to be superior to the third nerve. However this superior course of the fourth nerve is very short.42 Infection of the cavernous sinus is basically thrombo phlebitis which used to be almost cent percent fatal in pre antibiotic era. Even today it is common in malnourished and chronically ill children and remains sight threatening and life threatening condition. The cavernous sinuses can get infected as part of acute bacterial inflammation or by chronic granuloma. The former is generally refined to a cavernous sinus thrombosis while the later is known as anterior cavernous or superior orbital fissure syndrome. A third kind is known as a septic thrombosis that results following head injury and neuro surgical manipulation.44 Diagnosis of cavernous sinus thrombosis is straight forward when there is tenderness over the mastoid, proptosis, in 50% cases proptosis is bilateral, restricted movement of the globe, contra lateral sixth nerve palsy. Only condition that mimic cavernous sinus thrombosis is orbital cellulitis which is invariably unilateral. Presence of orbital cellulitis can be confirmed by CT and MRI. X-ray skull has no role in diagnosis of cavernous sinus thrombosis. Management of cavernous sinus thrombosis is a medical emergency. The child should be hospitalised and put on heavy dos of parental antibiotic. The cornea should be protected by antibiotic ointment and lubricants. Pseudo tumour orbit Pseudo tumour orbit is the cause of proptosis in ten percent of all cases. It is generally seen in adult male in fourth and fifth decade. In adults, it is generally unilateral. About one tenth of proved cases of pseudo tumour of orbit however belong to the pediatric age group, where it is invariably bilateral.47 Early diagnosis of this benign growth in children is required because it mimics rhabdomyosarcoma, metastatic neuroblastoma and leukaemic infiltration, all of which pose a threat to the life of a child and mistaken diagnosis of pseudo tumour in a case of malignancy can cause death of the child. The condition has been defined as an idiopathic localised inflammatory disease47. The disease acquires its name from the fact that it masquarades as neoplasm with a histopathological picture of inflammatory granuloma. The exact etiology is not known, the condition is perhaps multifactorial in which auto immune responses to antigens has been implicated.48 Vasculitis, myositis granuloma, thickening of tissue, lymphatic infiltration are common.9 The disease generally has a torpid course, it may have an acute onset or may linger for long time causing extensive fibrosis leading to a condition called frozen orbit. Children may show variable systemic symptoms. The characteristic presentation is a slow growing proptosis which may be axial when the growth is intra conal or eccentric when it is extra conal. The growth may involve any part of the orbit from the lacrimal gland to apex of orbit. It may even invade anterior part of the

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cavernous sinus. The proptosis is irreducible and does not change with posture or valselva manoeuvre. The mass is palpable between the orbit and the globe when it is anteriorly placed. The extra ocular muscle are involved in various combinations causing tropia resulting in diplopia or complete immobilisation of the globe. The conjunctiva is generally congested and chemosed. Corneal anaesthesia is a common feature due to involvement of fifth nerve otherwise cornea is bright and transparent unless it protrudes beyond the lid. In such cases, there may be exposure keratitis. As multiple structures are involved, the symptoms are variable. Initial symptoms are redness of eye, headache and diplopia. The parents may complain of gradually developing enlargement of the eye, which turns out to be a proptosis. Pain in the distribution of fifth nerve is common. Squint and diplopia are invariably present, loss of the vision should draw attention to optic neuritis, a growth behind the globe pushes the posterior pole forwards leading to hypermetropia. Diagnosis is by exclusion, confirmed by X-ray orbit + ultrasonography, CT, MRI, fine needle biopsy and excessional biopsy. A therapeutic trial by oral steroid is advocated in adults. It is avoided in children. The first step is to give proper attention to possibility of fatal conditions i.e. rhabdhomyosarcoma, leukaemia and metastatic neuroblastoma. Foreign body granuloma, unresolved orbital cellulitis and specific granuloma of the orbit are rare but possible differential diagnosis. X-ray orbit may also show enlargement of the orbit.49,50 A high resolution CT is more helpful because it provides more information about periorbital structures as well.42 A Scan has no value in this condition. On B Scan a characteristic irregular mass in retro bulbar space is visualised. Other findings on CT are orbital myositisthickening of the muscle, lateral and inferior recti are more involved, the insertion of the muscles also thickened. The posterior sclera and choroid may also show thickening.48 MRI is more sensitive and help to localise the small growth more precisely. Fine needle biopsy unless done by a person who is well versed with the procedure may be futile. Even in case of positive specimen, histopathologist may only be able to see if it is a malignancy or not but not the exact nature of the growth. Excisional biopsy under general anaesthesia through anterior orbitotomy or trans ethmoidal approach gives the best result to pinpoint the diagnosis. Management Most important part of the management is to be sure that the case under consideration is not malignant. Rhabdomuosarcoma, which is the commonest malignant tumour of the orbit in children may present with feature of inflammation and may show initial regression with systemic steroid. There is no specific treatment for pseudo tumour. The tumours completely regress under full dose of steroids orally over few months. Inter lesional steroids also give some relief but is difficult to administer in a child. While the child is under steroid careful watch should be kept on possible ocular and systemic side effects. The drug should be tappered over weeks. Other methods used areradiation and cytotoxic drugs in case of steroid resistant cases. Radiation consists of 15-25 Gy given in consultation with radiologist in divided dose. Cytotoxic drugs commonly used are cyclophosphamide, chlorumbucil, metho trex ate, cyclosporin

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separately or in combination in collaboration with pediatric oncologist and physician. Complications of pseudo tumours arerecurrence, poor response to steroids, strabismus, amblyopia, exposure keratitis, optic neuritis, papilledema, frozen orbit, permanent diminished vision. Tenonitis9,37 This is inflammation of Tenons capsule. It is rare in children except as complication of orbital cellulitis. Besides orbital cellulitis, severe scleritis and posterior uveitis can cause tenonitis in adults. The condition is painful, the eye is axially proptosed. There may be diplopia with restriction of ocular movement without paralysis of extra ocular muscles. There may be associated optic neuritis. Diagnosis is confirmed by CT and MRI. Management in children is meticulous treatment of orbital cellulitis. Common orbital tumours in children Tumours in children can be primary or secondary or extension from other structures (retinoblastoma). The secondary tumours are always malignant. They may metastasise from a distant organ i.e. neuroblastoma, Ewings sarcoma, Wilms tumour or leukaemia. The primary tumours are mostly benign. The benign tumours inflicting orbit in children areNeurofibroma, capillary haemangioma, dermoids, teratomas, optic nerve glioma. Commonest type of primary malignant tumour of orbit in children is rhabdomyosarcoma. Rhabdomyosarcoma54, 55 Rhabdomyosarcoma may develop at any age even at birth. In children it is the most common primary malignant tumour of the orbit. Average age of presentation is seven years. In adults it spares the orbit and develops in skeletal muscles. Surprisingly in children it does not develop from extra ocular muscles as it was thought to be in the past.45 However rhabdomyosarcoma may present as a mass in the lid.42 No race is immune, there is no predilection for right or left eye. It is unilateral. Boys have slightly more predilection. It is highly malignant and cent percent fatal when not treated. Mortality level has been considerably reduced due to radio therapy and chemo-therapy. It develops from pluri potential embryonal mesoderm which commonly differentiates to form rhabdomyoblast as seen in foetus.52 The growth may originate in the ethmoid and extend in the orbit. It may develop as second malignancy in case of retinoblastoma. Histopathologically there are three types of rhabdomyosarcoma1. The embryonal, 2. Aluelar and 3. pleomorphic. The first is seen in about 70% of cases, the last is least common. The tumour may arise anywhere in the orbit but commonest site is superio nasal quadrant. It starts as a painless rapidly growing mass that pushes the globe towards the opposite direction. However it may present with inflammatory signs and mistaken as orbital cellulitis or vice versa. The mass can be palpated by insinuating a finger between the globe and the orbital wall. The growth is non compressible. It does not invade the globe, which may otherwise be damaged and lost due to proptosis, exposure keratitis. The tumour may extend into surrounding structures by direct invasion. Distant metastasis is via blood stream. Lymphatic spread occurs when lid, conjunctiva and para orbital tissues are involved. In such case preauricular submandibular and neck glands may be involved.

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Differential diagnosis consists of orbital cellulitis, orbital neuroblastoma and retinoblastoma, leukaemic deposits, infected dermoids, Burkitts sarcoma, pseudo tumour. As the tumour is relatively fast growing and fatal, it should be treated early. Diagnosis is confirmed by X-ray that may or may not show boney erosion and enlargement of the orbit. CT and MRI are more definitive to assess the location, size of the tumour and plan treatment. Presence of bone erosion carries less favourable prognosis. Excisional biopsy is better avoided. Fine needle biopsy is not always reliable. Management consists of : 1. Treatment directed towards the local growth. 2. Treatment of distant metastasis 3. Handling of local complicationsOnce upon a time debulking and excentration were only treatment available. At present exentration has been given up. Sometime debulking is used to be followed by chemotherapy and radiotherapy. The tumour responds very well to combined chemotherapy and radiotherapy in which the survival rate is as high as 90% when the tumour is confined to orbit. With extra orbital extension this drops to 65%. The tumour is fatal within 18 months if not treated. Death is due to multiple organ failure following distant mertastasis. Many chemotherapeutic drugs have been used from time to time with variable results. They areandriamycin, vincristine, cyclo phosphamide and actinomycin D (dactinomycin). A combination of vincristine, actinomycin D and cyclophosphamide (VAC) is most commonly used as adjunct to radiotherapy. Radiotherapy consists of external beam radiation directed to the orbit. Total dose ranges between 4000 cGy to 6000 cGY given over four to six weeks protecting the cornea and lens. Side effects of radiation aremadarosis, dry eye, cataract, retinopathy of radiation. In children with diminished orbital volume, the size of the orbit may stop growing.51 Common secondaries that involve orbit in children are : Neuroblastoma Leukaemia Retinoblastoma Ewings sarcoma

Wilms tumour

Burkitts lymphoma Out of the above retinoblastoma and Burkitts lymphoma are not true metastatic tumour of orbit. They invade the orbit from primary source i.e. from the eyeball in case of retinoblastoma and from the maxilla in case of Burkits lymphoma. Neuroblastoma Neuroblastoma is a common malignancy of infants and children. It develops from embryonal neuroblast generally from adrenaline gland and sympathetic chain. However in 3% cases it develops from sympathetic plexus in orbit. Most of the neuroblastomas develop under seven years of age. The primary develops either in abdominal or thoracic segment of

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the sympathetic chain. It may develop in the cervical sympathetic as well. Fifty percent of children who develop neuroblastoma are under two years of age. 54 The metastatic neuroblastoma rarely show rossette formation which is seen mostly in primary site. There are two types of neuroblastoma i.e. (1) that has predilection for orbits known as Hutchinson type; (2) that metastasise in liver known as Pepper type. The metastasis is mostly blood borne, which develop in the orbital bones and from which it spreads to the orbital54 contents. Metastatic neuroblastoma is generally bilateral, which is detected few months after the primary has been discovered. However proptosis may be the first sign of neuroblastoma and generally attributed to a doubtful trauma because of one of the clinical feature is ecchymosis of the lid. Commonest presentation is fast growing proptosis in a child with a lump in abdomen or a para vertebral shadow on X-ray. The lids are swollen and ecchymotic. Forty percent of children with neuroblastoma develop orbital metastasis.53 The mass not only invades the orbit but may form a lump in the cheek, temple. Zygomatic bone is the commonest bone to be involved. Neuroblastoma should be differentiated from orbital cellulitis, infected dermoid, black eye, retrobulbar haemorrhage, rhabdomyosarcoma, Burkitts lymphoma and leukaemia. orbital retinoblastoma is diagnosed with ease due to presence of white reflex in pupillary area. Diagnosis is confirmed by CT, MRI, X-ray chest, ultrasonography of abdomen. A body scan may be required to locate the primary, fine needle biopsy may be helpful. Treatment consist of radiation and systemic chemotherapy. In radiation external beam photon is directed to the orbit. Total dose may vary 1500 to 3000 cGy in divided doses. Chemotherapy in neuroblastoma gives long term regression in about one third cases. However it should be remembered that radiation and chemotherapy are only paliative. Ewings sarcoma55,56,57 This is less common metastatic tumour of the orbit in children than neuroblastoma. Common age is first and second decade. The tumour has been observed more frequently in second decade. In contrast to neuroblastoma this tumour has para sympathetic origin. It generally develops in bones and from there it spreads to orbit. It may develop as second tumour following radiation in children who have undergone treatment for retinoblastoma. It presents as proptosis with or without visual loss. It must be differentiated from rhabdomyosarcoma, neuroblastoma and other metastatic tumours of the orbit in children. Treatment consists of chemotherapy and radiotherapy. Burkitts lymphoma (Lympho sarcoma) is a relatively uncommon tumour, no race or gender is immune to it. However, it is more common among coloured Africans. Common age is three to seven years with affinity for orbit. It has been postulated that this tumour has a viral etiology. The tumour starts in maxilla and erodes the floor of the orbit. It does not invade the eyeball. It starts as a painless swelling over the maxilla, spread into the lower lid and conjunctiva. The proptosis is rapid in progress. It may be bilateral. There may be involvement of central nervous system and invariably leads to death of the child. Some of the children58,59 may have abdominal mass. Proptosis may cause exposure keratitis with sloughing of cornea and perforation. The lymphoma responds to chemotherapy and radiation. Commonly used cytotoxic drugs are cyclophosphamide, methotrexate and vincristine. Relapse are common and fatal.

710 Optic nerve glioma61,62

PEDIATRIC OPHTHALMOLOGY

Glioma of the orbital part of optic nerve is a manifestation of congenital hamartoma of the anterior visual path. It is a benign slow growing tumour of ectodermal origin. Commonly associated with neurofibreomatosis.45 Topographically gliomas of visual path are divided into two parts : 1. Orbital, 2. Cranial. 1. Glioma of the orbital part is again divided into two groups according to its potential malignancy : (i) Benign glioma of childhod and (ii) Malignant glioblastoma of adult. The benign glioma of childhood arises from the supportive tissue of the optic nerve i.e. astrocytes and oligodocytes. The tumour is generally formed predominantly by one type of these cells and has been divided into astrocytomas and oligodendro glioma according to predominance of type of cells. Commonly found cells are pilocytic astrocytes. The incidence of glioma of optic nerve falls gradually with age. Though the growth is designated as congenital hamartoma, its common age is around seven years. The incidence falls sharply from first to second decade, however, when found in adults it is malignant in nature. The optic nerve glioma is an unilateral slow growing tumour that starts near the optic canal and spreads towards the globe in a fusiform shape. It is an intrinsic tumour of optic nerve and does not involve the sheath of the nerve. The growth is generally not palpable. Optic nerve glioma itself is painless swelling but the eye may become painful due to severe proptosis. The proptosis is axial and slowly progressive over years. It is non pulsative, irreducible, is not influenced by change in the position of the eye or straining. After years the lids may fail to protect the cornea, resulting in exposure keratitis, corneal ulcer and perforation. Reduction of vision is common but some vision may be retained for years, which is eventually lost due to optic atrophy. In later stages the growth presses on the posterior pole of the globe and results in axial hypermetropia. Unilateral diminished vision in childhood leads to strabismus and amblyopia. The squint is always non paralytic. Other clinical findings are presence of frank neurofibromatosis in about 25% of cases or presence of neurofibroma in close relatives. Suspicion of neurofibromatosis amy be aroused by presence of caf au lait in absence of obvious neurofibroma. A child with : 1. Unilateral painless diminished vision 2. Slow proptosis 3. With caf au lait anywhere in the body is a case of optic glioma that can be confirmed by X-ray, CT and MRI. Biopsy of the growth is seldom indicated. Sometimes the iris may show neurofibromatosis nodule (Lisch nodule). otherwise anterior segment has no findings that can be attributed to glioma except afferent pupillary reaction. Fundus may show papilledema, post papilledematous optic atrophy and even primary atrophy. The posterior pole shows retinal striation.

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X-ray of orbit and optic foramen are most important diagnostic investigation. On plain X-ray orbit : (1) There may be uniform enlargement of the orbit on the side of the growth. (2) Most characteristic finding is seen on X-ray examination of optic foramen by Rhese view. (i) Both the optic foramena should be examined at the same time. (ii) The diameter of normal optic foramen is almost constant i.e. 6.5 mm. (iii) Any foramen larger than 7 mm is sure evidence of enlargement. (iv) When both foramena are compared, a difference of more than 1 mm between the two is also diagnostic. (v) The enlargement is uniform without erosion showing slow expansion of the growth. (vi) The edge gives a polished sclerosed appearance. (vii) The optic canal is generally not enlarged as the tumour rarely invades the canal, which is more common in retinoblastoma. (viii) The sella may also show some changes in the form of J. shaped sella instead of usual round sella. Changes in sella are more common in glioma of optic chiasma. Ultrasonography and MRI show a well circumscribed, well delineated, fusiform growth. Chiasma glioma63 does not differ in histopathology. However it may extend in to the third ventricle or hypothalamus or may spread backwards in the optic tract. Involvement of chiasma does not produce typical bitemporal field changes. The field changes are generally stable as the growth is slow in expansion. The field changes are bizarre.64 They are more like optic nerve field changes than chiasma. Invasion of third ventricle leads to hypothalamic syndrome consisting of obesity, diabetic insipidus, precociuspuberty, dwarfism, pan hypopituitarism. Management As the growth is slow progressive, unilateral, non malignant and non fatal, there is no urgency in instituting definite treatment unless the cornea is at risk. Corneal involvement should be managed by - antibiotic and cycloplegic. If necessary the eye should be patched. In case of poor response to patching a median tarsorrhaphy is advised. Surgery is indicated for cosmetic reason and protection of cornea. In the past, the eye was enucleated with as much part of glioma as possible. This procedure is seldom practised. The surgical procedure is to remove as much of tumour tissue as possible either through an orbital or transcranial route leaving the eyeball intact. Radiation is indicated only when there is a tendency towards malignancy. Leukaemia deposit in orbit in children Lymphoblastic leukaemia is the commonest type of leukaemia that involves eye. It has both intra ocular as well as orbital manifestation. Myeogenous and monocytic leukaemia has less common ocular presentation. Orbital deposits are less common than intra ocular lesion.

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The child is generally anaemic and has thrombocytopenia that may result in bleeding form gums and cause epistaxis. The deposits are bilateral. The optic nerve may be infiltrated leading to loss of vision. The lymphoblastic leukaemia have good prognosis with modern chemotherapy. The proptosis regresses with chemotherapy combined with radiation. Capillary haemangioma of orbit Capillary haemangiomas are non neoplastic growths. They are hamartomas. Capillary haemangioma may be present in orbit, in the lid, on the skin of the face. There may be a combination of orbital and lid tumour. Some of the orbital tumours may be mistaken for lid tumours due to purple blue coloration of the skin of the lid. Histopathologically, they are blood filled endothelial channels without a true capsule. The growth is general diffuse but may be well localised. Most of the capillary haemangiomas are congenital but not recognised at birth. The presence of tumour becomes evident within first few months of birth when the growth increases in size while the child strains. The growth is compressible without bruit. The proptosis is unilateral and non tender. The growth is generally situated in the anterior orbit in the superio nasal quadrant. The tumour has a very predictable mode of evolution that is(1) Stage of rapid growth, (2) Stage of stability and (3) Stage of regression. The stage of growth is generally limited to the first year, stage of stability lasts for one to two or three years followed by regression by five years. The tumours situated deep in orbit are less common and difficult to diagnose. Capillary haemangioma may be associated with strawberry patches on the lid or skin. Sometimes dilated vessels may be visible in the fornices. Besides cosmetic blemish the common complications are associated myopia, astigmatism and amblyopia. Diagnosis is straight forward in case of growth in anterior orbit by : 1. Careful history 2. Examination of earlier photographs that may reveal proptosis not noticed by parents. 3. Presence of strawberry growth elsewhere. 4. X-ray orbit may show diffuse enlargement of orbit. 5. B scans show diffuse irregular mass. 6. CT and MRI give an ill defined appearance. 7. Contrast CT delineates the endothelial lined channel along with its feeding vessels. Differential diagnosis consist oforbital dermoid, orbital cellulitis, rhabdomyosarcoma, lymphangioma. Rhabdomyosarcoma has rapid progress without stage of stabilisation and commonly seen at about seven years of age while lymphangioma which is also a hamartoma is seen in teens with sudden enlargement due to haemorrhage in the growth that causes chocolate cyst formation. Management is divided into two groups 1. Management of error of refraction and amblyopia. Amblyopia may be due to uncorrected error of refraction or deprivation due to edema of lid or a growth in the lid.

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2. Management of the tumourAs the growth is self-limiting regresses without interference. Most of them do not require any treatment however if there is evidence of corneal exposure or compression of the optic nerve medial treatment with steroids and interferon is indicated, both of them are always associated with possible side effects, that should be monitored regularly in consultation with pediatrician. Steroid can be given either (1) orally or (2) intra lesional injection. Oral steroid is generally given in the form of prednisolone 1 to 2 mg/kg/day or 2 to 4 mg /kg/alternate day. Shrinkage of tumour starts within two weeks but there is high incidence of rebound growth. Intra lesional injection consist of 40 mg of triamcinolone with 6 mg of betathethasone given in the growth under general anaesthesia and repeated after 8 weeks. Orbital varices Orbital varices are commonest cause of intermittent unilateral proptosis. The orbital varices are part of vascular malformation of the orbit, which can be arterial, venous or arterio venous42. The venous formation are known as orbital varices. They are pathological dilatation of pre-existing venous channel. The commonest form is simple or primary varix that causes unilateral, intermittent, non pulsatile proptosis in a child. Proptosis may be present at birth and may be overlooked. The proptosis may disappear when the child is lying flat only to appear when the venous pressure is increased as in crying, sneezing, straining or pressure on the jugular vein. The proptosis becomes more when the child lowers the head. It may be associated with prominent vessels on the conjunctiva, lid or scalp on the same side.65 Besides congenital malformation orbital varices can be seen following orbital trauma where the superior orbital veins is torn. Some people presume that congenital varices are a variation of capillary haemangioma and lymphangioma, which is slow progressive, non intermittent and stationary. There may be dilatation of orbital veins secondary to intracranial AV malformation, generally in adults. The primary varices keep on growing at slow pace till the child is in teens when the progress stops. The plain X-ray picture is characteristic which shows (1) Enlargement the orbit, (2) Presence of round pheboliths, (3) There is a prominent vascular markings in the frontal bone. Venography may show either localised dilatation or a system of diffuse abnormal channels throughout the orbit. Ultrasonography shows dilatation of superior ophthalmic vein that may increase in size if venous pressure is raised. MRI gives better result as compared to CT. Orbital varices rarely cause visual disturbance or strabismus. Only complain is cosmetic. No specific treatment is required. Histocytosis Histocytosis is a systemic disease that affect children predominantly in various combinations of ocular and non ocular clinical presentation. Previously they were known by various names i.e. Histocytosis, Lettercrswie disease, Hand Shuller Christian disease and eosinophilic granuloma67. Since 1987, they have been brought under only one name that is Langerhans cell cytosis.66 The Langerhans cells do not have any definite function. They are derived from bone marrow. They are basically monocytes and present in various tissues in the body. The disorder

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histocytosis seems to be an auto immune disease that responds to systemic steroid as well as anti cancer drugs and radiation. The disease may start in infancy or early childhood. Common orbital manifestations can be unifocal, eosinophilic granuloma of the bones of the orbit. However bones involvement sometimes can be at more than one site. Multi focal involvement have multiple organ involvement, with bilateral proptosis, involvement of orbital bone and diabetes insipidus. Involvement of posterior pituitary is the cause of diabetes insipidus. There may be multiple intra ocular and periocular involvement. X-ray of the orbit shows lytic lesion of bone. Treatment consists of systemic steroid, vincristine, vinblastin, antibiotic and radiation. Some probable features and probable cause of proptosis in children
Features 1. Pale child, moderately ill, bleeding from gums, thrombocytopaenia and bilateral proptosis. Palpable abdominal mass, radiologically visible thorasic mass, hepatomegaly, ecchymosis of lid, unilateral proptosis. Unilateral fast growing proptosis, fever toxic child, X-ray showing sinusitis. Fast growing palpable mass, unilateral proptosis. Proptosis with white reflex. Unilateral axial proptosis with early visual loss. Bilateral proptosis, fever, toximic, child cranial nerve palsy. Unilateral axial proptosis with caf au lait on the body. Port wine stain on the lid, forehead or anywhere with proptosis. Bilateral proptosis with deformity of skull and face. Intermittent proptosis that increases with increased venous pressure. Unilateral proptosis, pain, fever, hazy cornea. Loss of vision. Bilateral proptosis, diabetes insipidus, involvement of boney orbit. Probable Cause 1. Leukaemia

2.

2. Metastatic neuroblastoma

3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13.

3. Orbital cellulitis 4. Rhabdomyosarcoma 5. Retinoblastoma 6. Glioma of optic nerve 7. Cavernous sinus thrombosis 8. Optic nerve glioma with neurofibroma. 9. Capillary haemangioma 10. Congenital anomaly of skull and face. 11. Orbital varix 12. Panophthalmitis 13. Histocytosis (Hand-Schuller Christian disease)

Orbital compartment syndrome67 This consist of a group of condition that cause acute rise of intra orbital pressure that result in elevation of intra ocular pressure, obliteration of central retinal artery and diminished ocular perfusion. The causes could beHaemorrhage, emphysema, profound edema of retro

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ocular structure due to trauma infection or inflammation like orbital cellulitis or expanding orbital abscess and namely pseudo tumours. The clinical features areDiminished vision, restricted movement of globe, afferent pupil, proptosis and raised IOP tight lids. Management This should be treated as emergency and treatment consist of lateral canthotomy and cantholysis. Reduction of IOP by IV Mannitol oral glycerine, broad spectrum systemic antibiotic and systemic steroids.

REFERENCES
1. Nema H.V. ; The orbit in Anatomy of the eye and its adnexa. 2nd edition. p-5260, Jay Pee Brothers. Medical Publishers, New Delhi, 1991. 2. Singh D. ; Pediatric cataract. CME series. All India Ophthalmological Society, New Delhi. 3. Duke Elder S. ; The orbit (connective tissueplanes and spaces) in System of Ophthamology. Vol. II. p-465466, Henry Kimpton, London, 1961. 4. Khurana A.K. ; Diseases of orbit in Modern Ophthalmology. Edited by Dutta L.C. 1st Ed. p-8587, Jay Pee Brothers, New Delhi, 1994. 5. Duke Elder S. ; Anomalies of the orbit and its contents in System of Ophthalmology. Vol. III, Part II. p-9421072, First edition, Henry Kimpton, London, 1964. 6. Marilyn Miller, Pruzansky S. ; Cranio facial anomalies in Principle and practice of ophthalmology. Edited by Peyman G.A., Sander D.R. and Goldberg M.F. Vol. III, p-23552417, 1st Indian edition, Jay Pee Brothers, New Delhi, 1987. 7. Harrison A.R. ; Orbital hypertelorism in Current Ocular Therapy. Ed. 5, Edited by Fraunfelder F.T., Roy F.H. p-189190, W.B. Saunders Company, philadelphia, 2000. 8. Schaffer D.B. ; Pediatric Ophthalmology in Text Book of Ophthalmology. 9th edition. Edited by Scheie H.C. and Albert D.M. p-283286, W.B. Saunders Company, Philadelphia, 1977. 8A. Nicholson D.H., Green W.R. ; Tumours of the eye, lid and orbit in Pediatric ophthalmology. Vol. II. Edited by Harley R.D. Second edition. p-248249, W.B. Saunders Company, Philadelphia, 1983. 9. Ahmed E. ; Text Book of Ophthalmology. 1st edition. p-130137, Oxford University Press, Calcutta, 1993. 10. Boger W.P., Peterson R.A. ; Pediatric Ophthalmology in Manual of ocular diagnosis and therapy. 3rd edition. Edited by Deborah Pavan Langston. p-275, Littlebrown. 11. Robb R.M. ; Dermoid in Current ocular therapy. Edition 5. Edited by Fraunfelder F.T., Roy F.H. p-234235, W.B. Saunders Company, Philadelphia, 2000. 12. Vaughan D., Asbury T. ; General Ophthalmology. 9th ed. p-243244, Lange Medical Publication, California, 1980.

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13. Vaughan D. and Asbury T. ; General Ophthalmology. 10th edition. p-229282, Lange Medical Publication, California, 1983. 14. Maya Eibschitz and Delmonte M.A. ; Mandibulo facial dysostosis in Current ocular therapy. Ed. 5. Edited by Fraunfelder F.T., Roy H.F. p-185188, W.B. Saunders Company, Philadelphia, 2000. 15. Christiansen S.P. ; Robin Sequence : in Current ocular therapy. Ed. 5. Edited by Fraunfelder F.T., Roy H.F. p-192193, W.B. Saunders Company, Philadelphia, 2000. 16. Fernando H., Murillo Lopez ; Oculo auriculo vertebral dysplasia in Current ocular therapy. Ed. 5. p-188189, Edited by Fraunfelder F.T., Roy H.F. WB Saunders Company, Philadelphia, 2000. 16A. Sargent R.A. and Ousterhout D.K. ; Ocular manifestation of skeletal diseases in Pediatric Ophthalmology. Vol. II, Second edition. p-10411042, Edited by Harley RD. W.B. Saunders Company, Philadelphia, 1983. 17. Khurana A.K., Ahluwalia B.K. ; Investigations of orbital space occupying lesion in Modern Ophthalmology. Ed. Dutta L.C. p-89102, 1st edition. Jay Pee Brothers, New Delhi, 1994. 18. Arthur S. Grove Jr. ; Orbital disorders in Manual of ocular diagnosis and therapy. 4th edition. Edited by Deborah Pavan Langston. p-5765, Little Brown 19. OConnor P.S. ; Ancillary clinical procedures in Neurophthalmology. Review Manual. 3rd edition. p-178, Edited by Bajandas F.T. and Kline L.B. Jay Pee Brothers, New Delhi, 1989. 20. Kanski J.J. ; The orbit in Clinical ophthalmology. Second ed. p-25, Butter Worth, London, 1989. 21. Dutta H., Mandal D. and Chakravarty A. ; Clinical Methods in Ophthalmology. 1st edition. p-146, Jay Pee Brothers, New Delhi, 1995. 22. Harrison A.R. ; Orbital hypertelorism in Current ocular therapy. 5th edition. Edited by Fraunfelder F.T. and Roy F.H. p-189190, W.B. Saunders Company, Philadelphia, 2000. 23. Grawler J., Saunders M.D., Bull J.W.D., du Bouley G., Marshall J. ; Computer assisted tomography in Orbital disease. BJO 58:571, 1974. 24. Rao VRV and Ravimandalam K. ; Computerised tomography in Neurophthalmology a manual for post graduates. Edited by Natchair G. P. 25.01 to 25.19. Arvind Eye Hospital, Madurai. 25. Buerger D.G. ; Proptosis in Ophthalmology secrets. p-235, Edited by Vander J.F. and Gaulet J.A. Jay Pee Brothers, New Delhi, 1998. 26. Dutta H., Mandal D. and Chakarvarty A. ; Clinical methods in Ophthalmology. p-2021, Jay Pee Brothers, New Delhi, 1995. 27. Deborah Pavan Langston : Ocular examination and diagnostic tests in Manual of ocular diagnosis and therapy. p-27, Edited by Deborah Pavan Langston, Little Brown. 28. DePotter P. ; Orbital imaging in Ophthalmology secrets. p-232, Edited by Vander J.F. and Gault J.A. Jay Pee Brothers, New Delhi, 1998.

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29. Haywood M. and Lina M. Marouf ; Eye trauma in Decision making in Ophthalmology. First Indian edition. p-58, Edited by Heuven WAJ and Zwaan J.T., Jay Pee Brothers, New Delhi, 1998. 30. Diamond G.J., Ossoinig KC, Fisher Y.L. : Ultrasonography in Principles and practice of Ophthalmology. Ed. Peyman G.M., Sanders D.R. and Goldberg M.F. p-14021487, Jay Pee Brothers, New Delhi, 1987. 31. Cynthia J. Kendall ; Ophthalmic echography. First Indian edition. p-19, Jay Pee Brothers, New Delhi, 1991. 32. Bergin D.J., Wright J.E. ; Orbital cellulitis. BJO 70:174-178, 1986. 32A. Leyden JJ ; Ocular changes in skin disorders in Pediatric ophthalmology. Vol. II, Ed. 2, p-10241025, Edited by Harley R.D. WB Saunders Company, Philadelphia, 1983. 33. Eustis H.S., Armstrong D.C., Buncic J.R. ; Staging of orbital cellulitis in children. J.Paed.Oph. and Strab. 23:246-251, 1986. 34. Spire J.R., Smith R.J.H. ; Bacterial infection of the orbital and periorbital soft tissue in children. Laryngoscope. 96:763-767, 1986. 35. Boger W.P. and Peterson R.A. ; Pediatric Ophthalmology in Manual of ocular diagnosis and therapy. 3rd edition. p-260, Edited by Deborah Pavan Langston, Little Brown. 36. Dhanda R.P. and Kalevar V. ; A Text Book of Clinical Ophthalmology. p-588589, Galgotia Publication Pvt. Ltd., New Delhi, 1993. 37. Agarwal L.P. ; Essentials of Ophthalmology. p-116120, 1st edition. CBS Publishers and Distributors, New Delhi, 2000. 38. Maus M. ; Orbital inflammations in Ophthalmology secrets. p-244247, Edited by Vander J.F. and Gault J.A. First Indian edition. Published by Jay Pee Brothers, New Delhi, 1998. 39. Ahemed E. ; A Text Book of Ophthalmology. Ist edition, p-129, Oxford University Press, Calcutta, 1993. 40. Kanski J.J. ; The orbit in Clinical ophthalmology. Second edition, p-3640, Butterworth, London, 1989. 41. Catherine E. Durboraw, Stasior G.O., Krohel G.B. ; Orbital cellulitis and abscess in Current ocular therapy. Ed. 5, p-577579, Edited by Fraunfelder F.T., Roy F.H. WB Saunders Company, 2000. 42. Gitternger J.W. ; Orbit in Manual of clinical problems in ophthalmology. Edited by Gittinger J.W. and Asdourian G.K. First edition, p-1740, Little Brown and Company, Boston, 1988. 43. Duke Elder S. ; System of ophthalmology. Vol. II, p-474478, Henry Kimpton, London, 1960. 44. Melen Onur and Klein J.W. ; Ophthalmic manifestation of vascular diseases of the brain in Principle and practice of ophthalmology. Vol. III, p-2064, Edited by Pyeman G.A., Sander D.R. and Goldberg M.F. First Indian edition. Jay Pee Brothers, New Delhi, 1987. 45. Miller S.J.H. : Parsons diseases of the eye. Seventeenth edition, p-332335, Churchill Livingstone, London, 1984.

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46. Glasser J.S. ; Neurophthalmology. p-258, Harper and Row, London, 1982. 47. Butten C.L., Young B.R. : Chronic orbital myositis. Arch. Oph. 100 : 1749-1751, 1982. 48. Ratnakar K.S. ; Pathology of orbital space occupying lesion in Pathology of the eye and orbit. First edition, p-204205, Jay Pee Brothers, New Delhi, 1997. 49. Scheie, H.G. and Albert D.M. : Ophthalmic radiology in Text Book of Ophthalmology. p252253, 9th edition, W.B. Saunders Company, philadelphia, 1977. 50. Scheie H.G. and Albert D.M. : Medical ophthalmology in Text book of ophthalmology. p454455, 9th edition, WB Saunders Company, Philadelphia, 1977. 51. Carol L. Shields and Sheilds J.A. ; Orbital rhabdomyosarcoma in Current ocular therapy. Edition fifth, p-256258, Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, philadelphia, 2000. 52. Levin R.A. ; Orbital tumors in Principle and practice of ophthalmology. Vol. III, p-21612165, First Indian edition, edited by Peyman G.A., Sander D.R. and Goldberg MF. Jay Pee Brothers, New Delhi, 1987. 53. Kanski J.J. ; The orbit in Clinical ophthalmology. Second edition, p-41, Butterworth, London, 1989. 54. Levin R.A. ; Orbital tumors in Principle and practice of ophthalmology. Vol. III, p-215657, First Indian edition, edited by Pyeman G.A., Sander D.R. and Goldberg M.F. Jay Pee Brothers, New Delhi, 1987. 55. Fraunfelder F.T. ; Ewings sarcoma in Current ocular therapy. Edition fifth, Edited by Fraunfelder F.T. and Roy F.H. p-235236, WB Saunders Company, Philadelphia, 2000. 56. Jakobicc F.A. Root man J., Jones I.S. ; Secondary and metastatic tumors of orbit in Clinical opthalmology. Vol. II, Edited by Duane T.D., p-167, Harper and Row, 1982. 57. Khurana A.K., Ahluwalia B.K. and Gupta S. ; Bilateral proptosis due to metastatic Ewings sarcoma. Ind.Jr.Oph. 40 : 15-17, 1992. 58. Karp, L.A., Zimmerman L.E., Payne T. ; Intra ocular involvement in Burkitts lymphoma. Arch. Oph. 85 : 295, 1971. 59. Feman S.S., Niwayama G., Hapler R.S., Foos R.Y. ; Burkitts tumor with intra ocular involvement. Surv. Oph. 14 : 106, 1969. 60. Vyas P. and Chandrashekhar G. ; Orbital lymphoma and allied conditions in Modern ophthalmology. Edited by Dutta L.C. First edition, p-124, Jay Pee Brothers, New Delhi, 1994. 61. Natrajan M. ; Tumors of optic nerve in Neurophthalmology. First edition, p-14.114.14, Arvind eye hospital, maduri. 62. Glaser J.S. : Neurophthalmology. p-116117, Harper and Row, 1982. 63. Boger, P.W., Peterson R.A. ; Paediatric ophthalmology in Manual of ocular diagnosis and therapy. Edited by Deborah Pavan Langston, p-283284, Little Brown. 64. Melen O. ; Ophthalmic manifestation of brain tumors in Principle and practice of ophthalmology. Edited by Peyman G.A., Sander D.R., Goldberg M.F. Ist Indian edition, p20122015, Jay Pee Brothers, New Delhi, 1987.

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65. Scheie H.G. and Albert D.M. ; Ophthalmic radiology in Text book of ophthalmology. 9th edition, p-147148, WB Saunders Company, Philadelphia, 2000. 66. Apple D.J., Pandey S.R., Gomez M.E. ; Langerhans cell histocytosis in Current ocular therapy. 5th edition, p-289290. Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000. 67. Rubin P.A.D. ; Orbital disorders in Manual of ocular diagnosis and therapy. 5th edition, p-5666, Edited by Deborah Pavan Langston, Lippincot Williams and Wilkins, philadelphia, 2002.

CHAPTER

21

Disorders of the Sclera in Children


Disorders of the Sclera The Sclera is the largest part of the outer coat of the eye, also the largest visible part of the eye and forms the white of the eye. It constitutes the posterior five-sixth of the outer coat of the eyeball. It extends from the limbus anteriorly to optic nerve posteriorly. It is opaque and white. It is tough. All the vessels that supply the uvea pass through the sclera without giving any specific branches to it. It gets some blood supply at the limbus. The sclera gets its nutrition from the episcleral vessels on the outer and choroid on the inner side. Absence of vessels in the substance of the sclera makes sclera less favourable site for infection, degeneration and new growths. Number of diseases that inflict sclera in all ages is not very long. Disorders of sclera in children is relatively rare except changes in colour, dehiscence and trauma. The sclera forms a hollow sphere of 22 mm diameter when fully developed. It has two large openings and three sets of smaller apertures in its coat. The anterior opening is called anterior scleral foramen it corresponds to the limbus and is 11mm in diameter while the posterior scleral foramen is the circular opening through which the optic nerve passes. The posterior scleral opening is bridged by lamina cribrosa. The opening for the optic nerve is situated 2 mm nasal to the posterior pole of the globe. The other apertures: 1. For passage of long and short posterior ciliary arteries and ciliary nerve. 2. For exist of venaeverticosae. They are four in numbers. One in each superior and inferior temporal or nasal quadrant, just behind the equator at 1, 5, 7 and 11 oclock. Sometimes there may be more than four vertex veins. The distance of vertex vein from limbus is not constant. The venaverticosae have an oblique passage through the sclera. 3. The anterior apertures near the limbus are for passage of anterior ciliary vessels. Thickness of the sclera is not uniform through out. It is thickest round the optic nerve i.e. 1.00 mm. It is about 0.75 mm at limbus and thinnest at the attachment of the four recti i.e. 0.3 mm. The sclera is covered intimately by the Tenons capsule. The capsule is attached to the sclera 1 mm to 2 mm away from the limbus and covers it snugly up to optic nerve where it blends with the covering of the optic nerve. There are some fine trabeculae, which keep the Tenons capsule anchored to the sclera. The space between the sclera and the Tenons capsule 720

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is a potential space without much clinical importance except that antibiotics and steroids can be injected in this space for better penetration. The Tenons capsule acts as synovial-membrane for the sclera. The visible part of the sclera is covered by bulbar conjunctiva, which can also be lifted off the sclera by fluid injected in the sub conjunctival space. In normal eyes the vessels seen over the sclera are conjunctival in nature and can be moved with conjunctiva. There are only a few visible episcleral vessels in normal eye, in chronically inflamed eyes and absolute glaucoma, prominent episcleral vessels can be visible. Attachment of the extra ocular muscles All extra ocular muscles are inserted in the sclera. The four recti are attached in front of the equator and the two obliques behind the equator. The distance of attachment of the recti are not equal from the limbus. The medial rectus is attached 5.5 mm away from the limbus. The inferior rectus is attached 6.5 mm away from limbus and the lateral and superior rectus 7.00 mm and 8.1 mm respectively. The tendons of the extra ocular muscle get mingled with the sclera very intimately. The inferior oblique has the shortest tendon i.e. 1 mm. Attachments of recti are on the thinnest part of the sclera. Care should be taken not to nick the sclera during detaching these muscles or passing sutures during strabismus surgery. Weak spots in the sclera The sclera is not of uniform thickness, it is precariously thin at the equator. The sclera has multiple apertures through which vessels and nerves pass. These are also potentially weak spots. Besides these anatomical weak spots the sclera may be thinned at other place following injury and inflammation. Thus the weak spots on the sclera are - Corneo scleral junction, equator, over the ciliary body and posterior pole. Colour of the sclera The colour of the adult sclera is white. At birth it has a bluish tinge due to choroid shinning under thin sclera. The sclera is thin in childhood, by five to seven years it becomes as white and as thick as adult sclera. Histologically sclera is divided into:1 1. Episclera 2. Scleraproper 3. Lamina fusca. The episclera is an ill defined layer of connective tissue that lies between the Tenons capsule and the sclera. It is loosely attached to both. It is thin at the equator and under the bulbar conjunction and thick round the optic nerve. It is less vascular than conjunctiva but more vascular than sclera proper, is more likely to be inflamed than sclera. The sclera proper consist of large bundles of collagen, packed densely. The superficial fibers are larger than the deeper. The space in between the fibers is filled by mucopolysaccharides. The fibers round the limbus and optic nerve are concentric with these structures, the fibers in between are arranged irregularly.

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Lamina FuscaIt is a thin ill defined layer that contains melanocyte on the inner side of the sclera. Functions of the sclera The main function of the sclera is to protect intra ocular structures, to maintain the shape of the globe and to keep the retina in optically correct position. For sharp focus it is essential that the interior of the eye remains in darkness, which is brought about by small mobile pupil, pigment in uvea and opaque sclera. The opacity of the sclera is due to irregular arrangement of its large fibres and low water content. Development of sclera2 Developmentally sclera is mesodermal. It develops from condensation of mesodermal tissue round the optic cup. The anterior part develops early, to which the developing recti are attached. The limbus is placed well behind its normal position initially, the limbus gradually moves anteriorly. By 12 weeks the posterior sclera develops round the optic nerve and a well formed posterior pole becomes obvious. The lamina also develops at this time. The scleral spur develops by 12th week. The Tenons capsule develops along with the eyeball. Surprisingly the shape and configuration of the eyeball are least influenced by sclera. These are regulated by developing retina3. (See page 3) Congenital anomalies of the sclera (See page 8-9) The congenital anomalies of the sclera are few and are mostly due to defect in: 1. Development of optic cup and its further progress 2. Failure of development of mesodermal tissue round the optic vesicle resulting into malformation of globe with (a) Intact scleraMicrophthalmos (b) With scleral defect i.e. microphthalmos with cyst and cystic eyeball. The other congenital anomalies of sclera are: 1. Blue sclera 2. Inter scleral nerve loop 3. Nanophthalmos 4. Sclerocornea 5. Congenital staphyloma Blue sclera Sclera of the new borne is more translucent than adult. The sclera of a new borne is thinner than adult sclera and number of scleral fibres are also less. With age both increases and the sclera becomes white. The colour of a blue sclera is in fact a blue tinge due to the uvea shining through. In some cases, the sclera fails to attain normal white colour and remains blue, without any other ocular anomaly and does not predispose any disease. Sometimes blue sclera is associated with systemic anomalies. Commonest combination is blue sclera with brittle bone in osteogenesis imperfecta with deafness.

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The other condition associated with blue sclera is Ehlers-Danlos syndrome. This is generally an autosomal dominant disorder; occasionally it can be autosomal recessive as well. Most striking feature of the condition is hyper elasticity of the skin due to loss of collagen tissue in the dermis. The skin of the upper lid can be pulled as far as the check. The ligaments of the joints are also lax leading to hyper extensibility of joints with frequent subluxation of joints. The other possible ocular components are blue sclera, keratoconus, micro cornea, subluxation of lens, angioid streak, may have choroidal haemorrhage. Inter scleral nerve loop3 In this case, a branch of long ciliary nerve changes its course to enter the substance of the sclera and emerges on the scleral surface under the conjunctiva as a tender nodule. It is generally appears on the nasal side, but has been reported from other sites also. It is generally a grey nodule but sometimes, it is surrounded by choroidal pigments. The cause of the anomaly is not known. It does not require any treatment. Nanophthalmos This is generally sporadic in nature, does not follow any fixed hereditary pattern. It can be unilateral or bilateral. The development of the globe is arrested in all dimension after the foetal fissure has fused leading to a small globe even smaller than a globe of a new born and remain small throughout the life. There are no other anomalies in the globe. The lens continues to grow as in other eyes. The result of small globe manifests as narrow inter palpebral aperture and deep set eyes. The distant vision is poor, occasionally with poor near vision in childhood, brought about by axial hypermetropia. The hypermetropia is always more than + 10 D. The child may have nystagmus; squint is common. Other cause of diminished vision is hypoplasia of macula. The eyes have tendency to develop simple glaucoma. Commonest complication of the condition is uveal effusion syndrome6 following penetrating injury accidental or surgical. Sclerocornea This is a combined anomaly of cornea and sclera, generally discussed under congenital anomaly of cornea along with corneaplana. The cornea and sclera blend into each other without trace of limbus. The condition may be localised on the peripheral cornea or may extend well into the centre of cornea. Congenital staphyloma Congenital staphyloma is congenital dehiscenes of sclera with bulging of the scleral coat that is lined either by ciliary body or choroid. The congenital scleral staphylomas are equatorial and posterior. Ciliary staphylomas are rarely congenital. About 6% to 14% of eyes have staphylomas5 without symptoms and are discovered during strabismus or retinal surgery. Posterior staphylomas develop in eyes with myopia more than 8 D. All high myopic eyes do not develop posterior staphyloma. Posterior staphylomas develop in the posterior pole. It may involve the optic nerve head also. These eyes are generally large eyes with pseudo proptosis. The distant vision is poor and not corrected fully by glasses. On retinoscopy the staphyloma shows more myopia than rest of the globe. Direct ophthalmoscope requires more

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minus lenses to focus the bottom of the staphyloma. The retinal vessels seem to disappear in the wall of the excavated staphyloma. A posterior staphyloma is best visualised with indirect ophthalmoscope. In presence of hazy media, presence of posterior staphyloma is confirmed by B scan ultrasonography. It is also well delineated by C.T. scan. Other cause of scleral staphyloma in children is buphthalmos7, which produces ciliary as well as equatorial staphylomas. Ciliary staphylomas are more common. Raised intra ocular tension does not produce posterior staphyloma. Intercalary staphylomas are least common in children. They are seen following badly repaired wounds, accidental or surgical at the limbus where the root of the iris in incarcerated in the dehiscenced wound. Scleral staphyloma are occasionally seen in older children in cases of oculo sporidiosis in endemic areas8. Infection and inflamation of the sclera do not develop in children. However inflamation of episclera i.e. episcleritis may occur in older children suffering from collagen disease. It is generally confused with phlycten of the conjunctiva, which is very common in children.

REFERENCES
1. Nema H.V., Singh V.P. and Nema N. ; The sclera in Anatomy of the eye and its adnexa. Second edition, p-1012, Jay Pee Brothers, New Delhi 1991. 2. Nema H.V., Singh V.P. and Nema N. ; Development of the eye and its adnexa in Anatomy of the eye and its adnexa. Second edition, p-129, Jay Pee brothers, New Delhi 1991. 3. Duke Elder S., Cook C. ; System of ophthalmology. Vol. III, Part II, p-162167, Henry Kimpton, London 1963. 4. Duke Elder S. Wybar K.C. ; The nerves of the eye in system of ophthalmology. Vol I, p-385, Henry Kimpton, London 1961. 5. Faris. B.M. and Freeman H.M. ; Scleral staphytoma and dehiscences in Current ocular therapy. Fifth edition, p-336337, Edited by Fraunfelder F.T. and Roy. F.H., W.B. Saunders Company, Philadelphia 2000. 6. Dutta L.C. ; Uveal effusion syndrome in Ophthalmology Principle and practice. First edition, p-122123, Current book international, Calcutta, 1995. 7. Duke Elders S. Leigh A.G. ; Disease of the sclera in system of ophthalmology. Vol. VIII, Part 2, p-9981003, Henry Kimpton, London 1965. 8. Mukherjee P.K. ; Rhino spordiosis in Current ocular therapy. Fifth edition, p-6667, Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Philadelphia 2000.

CHAPTER

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Ocular Manifestation of Systemic Infection in Children


The eyes are involved in many disorders of the body that may be present at birth as Congenital disorders: (i) Anatomical or functional disorder (ii) Infection (iii) Errors of metabolism (iv) Central nervous system disorders. Or be Acquired later, as infectious, allergic, auto immune, metabolic, degenerative or dystrophic disorder. It is common for malignancy of the eye to metastasise to distant organs, the reverse though rare is also possible. Ocular manifestation of infectious disease of the eyes: Infectious disease can be caused by: 1. Bacteria (i) Bacilli (ii) Cocci 2. Viruses 3. Fungi 4. Protozoa 5. Helminths 6. Myiasis (larva) 7. Arthopods Ocular manifestation of systemic bacterial disease1,2,3,4 (The following are short descriptions of systemic manifestation of infections that outline important systemic signs and symptoms, followed by ocular manifestation.) Bacterial ocular infection can either be acute or chronic. Acute infection is generally due to spread from neighbouring structure, or hematological spread. Acute systemic bacterial infection may release bacterial toxin as well.

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Chronic bacterial diseases are generally associated with granulomatous ocular disease or can cause allergic reaction to bacterial protein. Various systemic bacterial diseases that have ocular involvement are arranged in alphabetical order. All of which are not equally common. Some may have minimal ocular ocular morbidity. Others may have widespread ocular involvement. Most of them respond to specific antibiotics. Acinetobacter (Herellea Vaginicola) This is an opportunistic organism, present widely in water and soil. The organism belongs to family Neisseriaceae. It is an aerobic, gram-negative cocobacilli, infects children with compromised immunity. Systemic involvement Multi systemic infectionPneumonia, meningitis, urinary tract infection, and septicemia. Ocular involvement is less commonThey include: Blepharitis, purulent conjunctivitis, superficial punctate keratitis, sloughing corneal ulcer, and endophthalmitis, mostly following traumaaccidental or surgical. Management consists of correct diagnosis by culture. It is sensitive to many antibiotics, both local as well as systemic, common antibiotics used areTrimethoprimesulpha methoxazole, aminoglycocides, fluroquinolones. Actino mycosis This was previously thought to be a fungus infection, now it is classified as mycobacterium. This is an anaerobic organism. The organism is of low virulence with chronic course, does not cause impairment of vision. Systemic involvementCutaneous especially skin of face and neck, teeth and gums. Ocular involvement is generally unilateral, canaliculitis of lower lid leading to epiphora, swelling over the canaliculi. On pressing the inflamed canaliculus, there is regurgitation of yellow creamy material, which on microscopy contains concretion, like sulphur granules. Rarely there may be chronic dacryoadenitis. Management consists of systemic tetracycline, cephalosporin or penicillin. Local antibiotics are tetracycline ointment, or aminoglycocide drops frequently. Bacillus cereus The organism B. cereus belongs to genus bacillus that also contains bacillus anthracis or anthrax bacillus, which has acquired notoriety as a tool of biological warfare with lethal effect. Bacillus cereus is commonly found in water, soil, air and dust. It is highly virulent, generally resistant to penicillin and cephalosporins. It is aerobic, gram-positive, spore forming. Systemic involvement is relatively rare, seen in drug addicts who use infected needles. It reaches the eye as hematogenous spread due to contaminated intravenous injection or more commonly due to penetrating injury, mostly accidental rarely surgical. Ocular involvement is fast developing endophthalmitis often leading to panophthalmitis and loss of the eye.

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Commonest cause of post operative endophthalmitis is due to failure to destroy B Cereus by usual autoclaving because of its spore bearing character. The endophthalmitis develops within twenty-four hours, may have fever and raised WBC Count. The severity is directly proportionate to the capacity of the organism to produce exotoxin. The outcome of the condition is devastating visual loss. Management is an ocular emergency, fast developing endophthalmitis with rapid fall of vision, fever, leucocytosis points toward possibility of B Cereus infection that is conformed by AC or vitreous tap, and positive culture of organism. IV ciprofloxacilin 400 mg BD has been reported to give good result. Intra vitreous injection of vancomycin, gentamycin and clindamycin give better result than intra venous antibiotics. Early vitrectomy may salvage some vision. Brucellosis Brucellosis is mostly a zoonosis i.e. a disease that spreads from animal to man. The disease spreads following consumption of unpasteurised milk and milk products or direct contact with infected animals. The organism is a gram negative, intra cellular, non-motile, aerobic organism. There are four types of organisms that may infect man. The animals transmitting the disease may be any of the following: dogs, cattle, horses, sheep, goats and pigs. Systemic involvement Intermittent fever, headache, chills, joint pain, weight loss, speenogegaly and generalised lymphadenopathy. Other organs can also be involved. Ocular involvement In milder form it can cause swelling of lid, conjunctivitis, nummular keratitis. In sever form it causes sever anterior uveites, retinal haemorrhage, retinal edema, endophthalmitis, optic neuritis, extra ocular muscle palsy. Management The organism is sensitive to many antibiotics i.e. tetracycline, doxycycline, trimethoprinsulphamethoxazole, streptomycin and rifampin. It requires four to eight weeks continuous medication to eradicate the organism. Clostridium perfringes This is commonly known as gas gangrene organism that has dangerous systemic involvement, mostly involving large muscles. The organism is opportunistic, gram positive bacillus. It is an anaerobic gas forming organism. The systemic and ocular diseases are of acute onset, starting within twenty four hours of onset of infection. It can produceIntestinal disorder, suppurative deep tissue infection, skin and soft tissue infection.

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Ocular involvement includes sever uveitis with coffee coloured hypopyon, endophthalmitis, panophthalmitis, orbital cellulitis. Edema of the lids, chemosis of conjunctiva, sever ocular pain, fever, loss of vision, acute rise of intra ocular pressure are the hallmarks of the disease. Management In all cases of gas gangrene, initial treatment should be directed towards systemic manifestation of shock, haemolytic anaemia, fever by IV injection of gas gangrene antitoxin 50,000 units every six hourly for first two days. Hyperbaric oxygen when available may be useful. Antibiotics to which the organism is sensitive arePenicillin G. sodium, cefazoline, chloramphenical, amoxicillin, cefotoxamime. Management of ocular involvement is more difficult. Hyperbaric oxygen therapy seems to have some beneficial effect. Local antibiotic drops do not have any therapeutic effect. Sub conjunctival injection is better alternative. Intra vitreous route is the best mode of administration of antibiotic. Early vitrectomy removes highly toxic necrotic material, improving visual prognosis. Clostridium tetani Tetanus is a severe neuromuscular disease, it is one of the top ten causes of death in children in under developed countries where there is lack of universal immunisation. It can infect a person at any age. Clostridium tetani is found as spores in soil, dust, animal and human faeces. Symptomless humans can carry tetanus spores in gastro intestinal tract in about ten percent of population in non-immunised community. The organism is gram positive, anaerobic, motile spore wearing organism. The spore is terminal and colourless. The organism produces a neurotoxin called tetanospasmin, which causes muscle spasm. The disease is caused following injury on any part of the body with introduction of organism. The incubation period varies between two hours to several months. Short incubation period is associated with more severe infection. Distance of the injury from the central nervous system is important, nearer the wound to CNS more serious is the condition. Clinically there are four forms of tetanusNeonatal, cephalic, generalised and localised. Systemic involvement is wide spread neuromuscular involvement. Typical findings areLock jaw (trismus), risus-sardonicus, restlessness, irritability, stiffness, cramps, dysphagia and arched back. Ocular involvementOcular involvements are due to neuromuscular involvements and their sequel. They areBilateral blepharo spasm, paralysis of all the cranial nerves from third to twelfth resulting into ptosis, external ophthalmoplegia, internal ophthalmoplegia, total ophthalmoplegia, facial palsy, supra nuclear palsy, corneal exposure, conjunctival congestion, corneal ulcer, conjunctivitis.

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Management5 Tetanus is one of the most fatal diseases, which is fully preventable following proper immunisation. All expectant mothers should get two shots of tetanus toxoid irrespective of immunisation status. All children should get usual DPT as per national programme of universal immunisation. The child must get a booster dose by seventh year and repeated every ten years or following tetanus prone injuries that could be systemic or ocular. The systemic tetanus prone injuries are unattended wound of more than six hours, with depth more than 1 cm, all road traffic accidents, sports injuries, burns where the wound is contaminated by dirt. Tetanus prone ocular injuries are - Corneal and scleral perforation, lacerated wounds of lids, peri orbital structures. Non immunised children with tetanus prone injury should get injection of human tetanus immunoglobulin. Treatment of actual disease is mostly symptomatic and supportive: 1. The child should be admitted in specialised wards the child is put on life supportive measure with sedation under supervision of expert trained person dealing with such emergencies. 2. Antibiotics are given to prevent secondary infection. Common antibiotics are Penicillin G, tetracycline, erythromycin, metronidazole. The child should get an extra dose of tetanus toxoid and human tetanus immunoglobulin. Clostridium botulinum Botulism is a paralytic disorder that begins with cranial nerves and spreads downwards, is a fatal disease. Main types of botulism are - Infantile botulism, wound related botulism and food related botulism. C. Botulinum is gram positive that forms sub terminal spore, anaerobic in nature, found in soil and seawater. It produces most potent bacterial toxin. Incubation period is short i.e. 18 to 36 hrs in food related botulism and may be as long as 10 days in wound related botulism. Clinical picture in both is almost same. The systemic involvement includes descending paralysis beginning with ptosis, going through diplopia, dysphagia, dysarthria, may involve neck and thorax. Other symptoms are that of food poisoning i.e. nausea, vomiting, abdominal pain, paralytic ileus, urinary retention. Ocular involvement consists of bilateral ptosis, diplopia, fixed dilated pupil, and cycloplegia. Management The child should be hospitalised in food related botulism. Trivalent antitoxin is administered, intestinal antibiotics are given to reduce the organism present in the gut. Corynebacterium diphtheriae This is an acute, life threatening disease with extensive ocular involvement. Healthy asymptomatic carriers may be responsible for spread of infection in non-immunised community. The

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disease is fully preventable except in a few individuals who may be infected by the disease in spite of proper immunisation. The effect of disease in such cases in mild and nonfatal. The organism is a surface saprophyte affecting naso pharyngeal area. It is gram positive, club shaped, non motile, pleomorphic organism. It produces a strong toxin that affects heart, central nervous system, cranial nerves, and eyes. Respiratory obstruction is caused due to accumulation of necrotic exudate consisting of leucocytes, RBC and respiratory exudates. Systemic involvement comprises of fever, chills, cutaneous diptheria, myocarditis, and polyneuritis. Respiratory tract involvement includes sore throat, rhinorrhea, hoarseness, dysphagia, cough, and pneumonia. Ocular involvements are mostly extra ocular. The commonest involvement is conjunctival that start as congestion proceeding tocatarrhal conjunctivitis, purulent conjunctivitis, membranous and pseudo membranous conjunctivitis. There may be small sub conjunctival haemorrhage, as the condition proceeds towards membrane formation the lids become swollen and hard due to development of tarsitis and meibomianitis. Corneal involvementSuperficial keratitis, frank corneal ulcer, sloughing corneal ulcer and perforation. Cranial nerve involvement includesPtosis, paralysis of extra ocular muscles, paralysis of accommodation without mydriasis, internal ophthalmoplegia, convergence and divergence paralysis. Optic neuritis is rare. Late complications of conjunctival and corneal involvement lead to trichiasis, entropion, symblepharon, and xerophthalmia. Management The most effective management begins with immunisation of all children against diphtheria along with tetanus and whooping cough. The immunisation needs to be repeated after 3 to 5 years. Once the disease has been diagnosed, the management consists of neutralising the circulating toxin and reducing the bulk of bacteria by antibiotic, along with life supportive measures. Ocular involvement is treated by instillation of anti toxin, local instillation of penicillin G., erythromycin, mydriatic and cycloplegic. Escherichia Coli This is a commensal found in the gastro intestinal tract. Other common portal of entry is urinary tract. The organism is gram negative rod. Systemic involvement cause diarrhoea, dysentery, haemorrhagic colitis, urinary tract infection. It can cause fatal haemolytic- uremic syndrome in children. Ocular involvements are relatively less frequent. It may be as mild as mucopurulent conjunctivitis to as serious as metastatic endophthalmitis. It can also cause pseudo membranous conjunctivitis, keratitis, corneal ulcer, uveitis, gas in AC, hyphema, hypopyon, and endophthalmitis. Management consists of systemic antibiotic. Common antibiotics used areAmpicillin, cephalosporin, nitrofurantoin, trimethoprinsulfamethoxazole, gentamicin, kanamycin, amikacin.

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Ocular management is management of conjunctivitis, corneal ulcer, uveitis and endophthalmitis by standard methods along with management of systemic condition. Hemophilus influenza and Koch-Weeks bacillus Both the organisms belong to the group of hemophilus, have identical staining pattern, clinical presentation and are sensitive to same type of antibiotics. The Koch-Weeks bacillus is also known as H. aegyptius. There are many more strains of hemophilus. Both are pleomorphic, gram-negative cocco-bacilli. Hemophilus is a common bacterial infections in children that cause upper respiratory tract infection, have seasonal variations, seen in small epidemics, may present as pink eyes followed by respiratory infection or vice versa. Systemic involvement consist ofFever, malaise, upper respiratory tract infection, sinusitis, bronchitis. In sever form it causes pneumonia, Otitis media is less common. Severe infection may cause meningitis and bacteremia, septic arthritis. Ocular involvements are common. They are - Rapidly developing mucopurulent conjunctivitis, pseudo membrane formation, edema of the lids with violet colour, keratitis, central ulcer is rare. Conjunctivitis resolves in eight to ten days that may leave conjunctival scar in the tarsal conjunctiva. Phlycten are common after clearing of acute conjunctivitis. Ocular management is instillation of broad spectrum antibiotics like ciprofloxacilin drops frequently with same ointment at bed time along with management of corneal ulcer when present. Mycobacterium tuberculosis Infection by this bacillus is one of the oldest known chronic disease world-wide. It still is a major cause of death in developing countries. It can infect any organ in the body in all ages in both sexes. It is not a hereditary disease but it is common to see more than one person infected by tuberculosis in the same family. Broadly it is divided in to two groups i.e. pulmonary tuberculosis and extra pulmonary tuberculosis. Mycobacterium tuberculosis is a rod shaped, non spore forming, slender, aerobic bacterium. It does not stain with gram stain. It is an acid-fast organism. It spreads by droplet from the infected person who is positive for tuberculosis organism. Systemic involvement - Commonest organs to be infected are the respiratory organs. Common infections are seen in lungs and pleura. It can infect the small intestine, urinary tract, meninges. It can form intra cranial tuberculoma. Bones and joints are also involved. The disease is characterised by tissue destruction and replacement by granulomatous tissue forming typical tubercles, which has central caseation. The severity of the lesion depends on many factors i.e. organ involved, tissue hypersensitivity, immunity and overall resistance power of the individual. Inflammatory process and tissue destruction is mostly due to tissue hypersensitivity while healing is immunity dependent. Ocular involvement Primary ocular involvement is rare and mostly seen in the lids as cutaneous tuberculosis or tubercular conjunctivitis, which may produce a cocks comb like growth in the conjunctiva. Incidence of tuberculoma of the conjunctiva has become very rare.

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It is rare for a case of frank pulmonary tuberculosis to develop tuberculosis of the eye. The common conjunctival lesion is phlyctenular conjunctivitis. Conjunctival ulceration is seen with cutaneous tuberculosis involving the lid margin. Cornea Commonest involvement is phlyctenular kerato conjunctivitis, followed by fascicular ulcer of the cornea. Frank keratitis by tubercular bacilli is very rare; more common is interstitial keratitis. Superficial keratitis is more common in cases of non tubercular myco-bacterial infection. In sclera two common involvements are episcleritis and scleritis. A scleritis nodule near the limbus may cause sclerosing keratitis. Involvement of orbit is rare but may cause mild proptosis. A tubercular sinus may develop on the skin or conjunctival fornix. The conjunctival, corneal and scleral involvements are immunity mediated but orbital involvement is either due to extension from infected sinuses or via blood. Intraocular involvement All vascular intra ocular structures may be involved either as hematogenous spread which is rarer than more common hypersensitivity to the tubercular protein. It can be present as acute mild iritis to severe posterior uveitis. The uveitis is granulomatous in nature with vitritis. Tubercular endophthalmitis has not been reported. The iris may have multiple tubercular nodules in chronic stage. Ciliary body may be involved as part of iridocyclitis or may have large nodule that may be large enough to cause angle closure glaucoma and confused as a neoplasm which can be differentiated only on B scan ultrasonography. Choroidal involvement may be in the form of solitary patch of choroiditis or there may be multiple patches of choroiditis. The choroid may be involved in miliary tuberculosis. Retinal involvement is rare. It is involved as patch of chorioretinitis, periphlebitis is more common than retinitis. Extra ocular muscle palsies are seen in cases of tubercular meningitis and intracranial tuberculoma. Sub tentorial tuberculoma are common in children that my lead to papilledema and post papilledematous optic atrophy. Optic neuritis is less common, however, anti tubercular drug induced optic neuritis is common which is dose related and resolves following reduction in dose of the drug. Management ProphylaxisBCG injection soon after birth saves the child from serious forms of tubercular infection like miliary tuberculosis and meningitis. The best prophylaxis is early detection and complete eradication of infection in the individual.7 Preventive chemotherapy is popular in developed countries. Its place in under developed countries has not been fully evaluated.

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All cases of proved tuberculosis should get appropriate anti tubercular chemotherapy for adequate period. Ocular tuberculosis is managed by 1. Appropriate systemic anti tubercular treatment for adequate period. 2. Local treatment by cycloplegic and local steroid 3. Posterior uveites, neuro retinitis, periphlebitis require systemic steroid for long time that should be always given under cover of systemic anti tubercular chemotherapy. Neisseriae gonorrhoeae6 or gonococcal infection is a world wide health problem, more in developing countries than in developed countries. It is an acute multi systemic disease that can be fatal in new born and infants. It is a cause of bilateral diminished vision in children who had inadequate treatment. Neisseriae gonorrhoeae are intra cellular, gram negative diplococci, seen inside the polymorphonuclear lencocytes. The organism is oxidase and catalase positive. Many strains produce B. lactamase that makes the organism develop resistance to penicillin, which is still considered to be the first line of treatment of gonococcal infection. Chromosomal mutation is yet another cause of penicillin resistance. Humans are the only natural reservoir of the organism. It is a foremost cause of sexually transmitted disease. Though largest number of new cases start in second and third decade, no age, sex or race is immune to the disease. The infection begins as acute inflamation of colummar and cuboidal epithelium. Systemic involvementIn adults it begins mostly as purulent urethritis in males and cervicitis in females. The incubation period is 2 to 5 days. However some persons do not develop acute stage and remain asymptomatic, do not seek treatment and act as reservoir of infection. While the symptomatic patients especially males always seek medical treatment for dysuria, meatal inflamation, local swelling, pain, fever, unilateral epidedymitis, inguinal lymphadenitis. In females the infection starts as endo cervicitis, leading to yellowish discharge. The infection may travel up and cause acute endometritis and salpingitis. Urethritis is common. Other systemic involvements arePolyarthritis, tenosynovitis, pericarditis, endocarditis, toxic hepatitis and rarely meningitis. Gonococcal urethrites is a major cause of stricture urethra in males and cause of pelvic inflammatory disease in females. Ocular inflamation was a major cause of bilateral blindness before advent of chemotherapy. Though the pathogenesis of ocular gonococcus infection is almost the same in all ages, clinical presentation and management differ in different age groups. Ocular manifestations in children can be broadly divided into - Neonatal gonococcal infection and gonococcal infection in pediatric and adolescent. Neonates develop gonococcal infection in two ways: 1. A less frequent intra uterine infection 2. Common direct infection during passage through an infected birth canal. The first is possible if mother develops premature rupture of the membranes. This results in serious septicaemia, pneumonia and meningitis. Ocular involvement is restricted to

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chorioretinitis. This type of infection is not preventable by caesarean section. The second is more common and called as gonococcal ophthalmia neonatorum. Pediatric and adolescent gonococcal ophthalmia is clinically similar to adult gonococcal ophthalmia. It is generally associated with child abuse or assault. However with development of early sexual activity and precociousness, incidence of adolescent ophthalmia is on the rise. Other ocular involvements are: Bilateral central corneal ulcer, gonococcus is one of the few organisms that can pass the intact corneal epithelium causing not only corneal ulcer but also anterior uveitis. Hence all cases of ophthalmia neonatorum should be treated as gonococcal unless proved otherwise. All cases of gonococcal conjunctivitis should also get local atropine in appropriate dose. Perforation of central corneal ulcer may lead to acute endophthalmitis, panophthalmitis and loss of eye. Smaller perforation generally lead to collapse of AC, bringing the lens in contact with the cornea resulting in formation of anterior polar cataract. The perforation generally heals and a central leucoma results. This leads to a triad of central corneal opacity, anterior polar cataract and nystagmus. Gonococcal infection may lead to mild iritis to sever plastic uveitis. Management Management of gonococcal infection in new-born child begins with ante natal examination of mother and if found to be suffering from genital gonococcal infection, she should be treated adequately. Caesarean section may save the child from neonatal infection. All children born of infected mother should also get adequate systemic antibiotic. All neonates irrespective of their infective status should get prophylaxis for ophthalmia neonatorum either as single drop of 2% silver nitrate solution in each eye or 2.5% single drop of povidone iodine in each eye. In absence of above two, any broad spectrum antibiotic may be used as local drop. Neisseriae meningitidis Neisseria meningitidis or meningiococcus is a gram negative diplococcus that perish on exposure to drying and chilling. It is found only in human beings. The natural habitat of the organism is the nasopharynx. Meningococcal infection is mostly seen in children world wide. The infection may be sporadic or there may be localised outbreak, epidemics are no more seen. It spreads either by inhalation of infected droplets or direct oral contact of infected person. Systemic involvement is more common and serious than ocular involvement. The organism has a short incubation period. The clinical manifestations vary from transient bacteremia with good health to irreversible shock, hemorrhagic shock and death. The systemic involvement follows a set pattern of clinical manifestation that begins as upper respiratory infection followed by bacteremia and meningitis. To begin with there is soar throat, rhinorrhea, cough, headache, redness of the eye, with fever, meningeal irritation. Less frequent manifestation are arthritis, pneumonia, sinusitis, otitis media, endocarditis, pericarditis.

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Neurological complications are common they include encephalitis, brain abscess, extra ocular muscle palsy. Ocular involvement Ocular involvements are less frequent and may be overlooked due to more severe systemic manifestation. Ocular involvement is mostly metastatic, however acute conjunctivitis may be due to direct droplet infection or spread from nasopharynx. Meningococcus like gonococcus can pass intact corneal epithelium. Fortunately corneal involvement is rare, there may be pseudo membrane formation over the conjunctiva. Other ocular features arePupillary changes due to involvement of third nerve in basal meningitis. Mydriasis is more common than miosis. Transient extra ocular muscle palsy is common. So is optic neuritis ; there may be papilledema due to raised intra cranial pressure. There may be metastatic uveitis that may be mild or as severe as endophthalmitis. Management is mostly medical by systemic antibiotic for meningitis, pneumonia and endocarditis. Local treatment consists of atropine ointment once a day and broad spectrum antibiotic drops given frequently. Moraxella This group of mild to moderate infectiveness consists of many strains. The same organism is known by more than one name. Previously due to its similarity in morphology and staining characteristic to gonococcus, it was thought to belong to neisseriae. With more study of DNA, it has been put in genus branhamella. It was formerly known as diplococcus of MoraxAxenfeld, Moraxella lacunata, Moraxella catarrhalis or even Neisseria catarrhalis. The organism is a gram negative paired organism that resembles gonococcus due to its bean shaped appearance. It is found in the upper respiratory tract of almost fifty percent asymptomatic children. Systemic involvement includesOtitis media, sinusitis and pneumonia. Ocular manifestations are seen in malnourished, chronically ill children. It causes blepharitis, dermatitis of the outer canthus, chronic angular conjunctivitis, corneal ulcer that may terminate in hypopyon corneal ulcer. The organism is sensitive to fluoroquinolones, erythromycin, and many other antibiotics. The organism produces an enzyme protease that is specifically counteracted by zink sulphate as 0.25 % to 0.5% drops. Pneumococcal infection The organism is better called streptococcus peumoniae due to its tendency to form chains and predilection for respiratory tract. It is one of the major causes of pneumonia in all ages. Children under two years are more prone to get the infection than older children. The natural habitat of the organism is human nasopharynx. It has been isolated from nasopharynx of 5% to 10% of healthy adults and 20% of healthy children. It is also found as commensal of many healthy persons. The organism is a gram positive, capsulated

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diplococcus with short incubation period. Mode of spread is through droplet from infected person. Once the organism gets into the nasopharynx it spreads quickly. The common modes of spread are - Direct spread over the respiratory epithelium mostly in the respiratory tract, middle ear, lacrimal sae and conjunctiva. Conjunctiva gets infected by: 1. Change of organism from commensal to pathogenic state. 2. Spread via lacrimal passage 3. Hematogenous spread 4. Direct infection by droplet. Systemic involvement consists ofBilateral pneumonia, consolidation of lung, otitis media, sinusitis, meningitis, peritonitis, salpingitis, osteomyelitis and pericarditis. Pneumonia and consolidation may lead to empyema. Pneumococcus is known as true ocular pathogen. Ocular involvements are mostly conjunctival. There may be acute, bilateral mucopurulent conjunctivitis with or without petechial haemorrhages. Pseudo membrane formation is common. Conjunctivitis may be associated with superficial keratitis, corneal ulcer, which is generally fast spreading serpiginous ulcer with large hypopyon. There are three ways by which the uvea may get involved: 1 By way of pneumococcal ulcer 2. Spread of toxin from lacrimal sac 3. Metastatic spread from other organs. Uveal involvement may present as endophthalmitis. Chronic dacryocystitis is very common. The lacrimal sac may harbour pneumococci without symptom, may have chronic dacryocystitis. Occasionally there may be acute orbital cellulitis. Management Pediatrician should manage the systemic conditions by antibiotics. Common anti microbials used areB. lactame antibiotics; once penicillin was the first drug choice, now more and more organisms have become resistant to it. Other commonly used drugs areTrimethoprime, cephalosporins, erythromycin, clindamycin, chloramphenicol. The organism is less susceptible to the qunolones. Ocular management Conjunctivitis responds well with local erythromycin ointment, bacitracin drops. Even fresh solution of penicillin, but poorly by qunolones. In all cases of suspected pneumococcal conjunctivitis, cornea should be carefully watched for any evidence of its involvement. The anterior chamber should be examined for AC reaction, cells, flare or even hypopyon. Hypopyon is so common with pneumococcal dacryocystitis that in all cases of hypopyon with or without ulcer the sac should be examined for stagnation or frank infection. Acute dacryocystitis, orbital cellulitis, endophthalmitis require systemic anti pneumococcal antibiotic in appropriate dose for sufficient time. Corneal ulcer is treated as ophthalmic emergency.

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Pseudomonas areuginosa The organism is one of the commonest sources of hospital infection and infects accidental as well surgical wounds frequently. It is ubiquitous, free living, opportunistic, gram negative, small, straight or slightly curved motile organism. It produces a blue green pigment called pyocyanin that colours the pus and exudate making its diagnosis easy. It produces large quantity of proteases that rapidly cause stromal necrosis. It is found in 50% of water, plants, animals and humans. About 5% of symptomless persons have stool infected by the organism. The organism does not produce clinical symptoms in healthy adults. It mostly invades the tissue following trauma accidental or surgical or following invasive procedures. It may involve the skin or mucous membrane primarily, from where it may spread by continuity or metastasis. Out of all infections, ocular infections are most devastating. The systemic involvement consists of: Respiratory infection that may be mild or life threatening, associated with cough, fever, chills, dyspnea, cyanosis. Ear infection is common. Generalised bacterimia, endocarditis, CNS involvement, Bones and joint involvement may be traumatic or non traumatic. Ocular involvement by pseudomonas is a serious problem. It should be treated as ocular emergency. Predisposing factors areContact lenses, soft extended wear contact lens are more likely to be contaminated by pseudomonas; hard contact lenses are no exception. The organism may be adherent to the contact lens. Other source of contact lens contamination is contact lens solutions. Fluorescein drops are notoriously contaminated by pseudomonas. Hence it is better to use sterilised fluorescein strip rather than hospital prepared drops. If use of drops is inevitable, it should be autoclaved frequently and kept away from any possible source of contamination. The organism may be passed from one patient to other via tonometer, gonioscope etc. Clinical presentation of pseudomonas infection consist of redness, pain, discharge, mucopurulent conjunctivitis, foreign body sensation, excess of watering, increased pain herald onset of corneal ulcer. The ulcer is generally central with massive bluish green hypopyon. Peripheral ulcer may be associated with scleritis. The ulcer if not treated progresses fast and leads to perforation that may end up in endophthalmitis and panophthalmitis. Management Pseudomonas is sensitive to many broad spectrum antibiotics. Some of them areTobramycin, ciprofloxacilin, ofloxacilin. Staphylococcus This is one of the most common infection world over. The organism is ubiquitous that commonly develops colonies on the skin and mucous membrane without much symptom or may remain asymptomatic, 15 to 50% of population being the reservoir. They are a public health hazard. They are the commonest organism to cause nosocomial infection. Beside skin and mucous membrane, they are found in water, dust and soil, inanimate objects of the hospital. Many of the persons working in the hospital are carriers of the organism. Contamination of bioprosthesis including IOL, contact lenses, irrigating tubes, irrigating solutions are common by staphylococci. The stain S. aureus is most important human pathogen.

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It is one of the most tenacious organism that is potentially destructive, difficult to destroy by sterilisation and has tendency to develop resistance to antibiotic. The organism is a small, gram positive, non motile, non sproulating that is seen in various forms i.e. single, in chains or in bunches. It is easily grown. The growth is best at 30-37C. It is resistant to desiccation and many disinfectants. They are aerobic or facultative anaerobic. Basically there are two types of organism i.e. coagulase positive and coagulase negative. Out of the two the former is pathogenic and the organism is called staphylococcus aureus, that produces golden coloured colonies. It produces many enzymes and toxins. There are many species of coagulase negative staphylococci. They are known by various names i.e. S. albus, S. epidermis, S. hemolyticus, S. saprophyticus. Coagulase negative staphylococci were thought to be only contaminants in the past, they are now considered as important opportunistic organism. There are no known vaccines, anti toxins or toxoids that can be used as prophylaxis against all strains and species. Systemic involvement - Almost all organs can be infected by staphylococci. Multiple organ involvement is common. It may develop in new born as toxic epidermal necrolysis to ripe old age as pneumonia. It may manifest as simple single boil, carbuncle, erysipelas. Other common systemic involvements are respiratory infection, rhinitis, otitis media, CNS infection, urinary tract infection, osteomyelitis, food poisoning and sepic shock syndrome. Ocular involvement is common due to continuity of ocular surface with skin through lids and nasopharynx via lacrimal passage. Other sources of intra ocular infection are mostly intra operative and post operative. Common ocular lesions areStye, lid abscess, boil on the lid, chronic blepharitis, dacryocystitis, orbital cellulitis due to generalised septicimia, there may be periostitis or orbital abscess. Most common infection is acute or chronic conjunctivitis, other involvement in children is phlycten. Corneal involvement may be peripheral catarrhal keratitis due to staphylococcal toxin and suppurative keratitis. Most dangerous infection is acute or delayed endophthalmitis following any intra ocular surgery, or penetrating injury. S. aureus causes acute endophthalmitis while coagulase negative strain cause delayed and chronic endophthalmitis. Staphylococcal infection can cause anterior non granulomatous uveites, scleritis even dacryoadenitis. Cavernous sinus thrombosis though basically a systemic CNS infection invariably report to the ophthalmologist. Management The infection may be caused by penicillin and methicillin resistant organism. Hence it is essential to find out the cultural character and sensitivity to antibiotic for complete eradication.

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Ocular Blepharitis: Lid hygiene, frequent application of erythromycin 0.5% ointment or bacitracin ointment for weeks. Oral doxycilin 100 mg OD 10 ConjunctivitisSulphacetamide drop, ciprofloxacilin 0.3% drops, frequently. Keratitis- Fortified cefazoline drops. Vancomycin 50 mg/ml in phosphate buffered artificial tear is used in methicillin resistant staphylococci along with cycloplegic, may require sub-conjunctival injection. Endophthalmitis is an emergency that requires early detection and management. Intra vitireal injection of proper antibiotic is the most effective method to combat endophthalmitis. Role of vitrectomy is doubtful. Streptococcus Human respiratory, gastro intestinal and genito urinary tracts commonly have colonies of streptococci. A person may be asymptomatic yet may harbour the organism. It is a major cause of neonatal sepsis, post infective acute rheumatic fever and post streptococcal glomerulonephritis. The enteroccocci cause mostly urinary tract infection while viridans cause endocarditis frequently. The organism is gram positive, oval shaped, non motile, non sporulating. When grown in liquid media, they are seen in pairs or chains. The human pathogenic strains are facultative anaerobes. They are difficult to culture, requiring enriched media. Many strains cause hemolysis round the colonies. This property to causes hemolysis has been utilised to classify them into alpha and beta hemolyticus, when cultured on blood agar. About 20 percent of persons in a population may be asymptomatic carriers who are responsible for food borne infection and nosocomial infection. Systemic involvement All parts of the body can be infected by streptococci. The common conditions are pyoderma, cellulitis, erysipelas, necrotising fasciitis, scarlet fever, pharyangitis, tonsillitis, tonsilar abscess, pneumonia and empyema, streptococcal shock syndrome, neonatal sepsis and meningitis. Ocular manifestation can be direct involvement of the conjunctiva, spreading from the skin, nasopharynx or metastatic. Streptococcal conjunctivitis is generally self-limiting. It can cause membranous or pseudo membranous conjunctivitis that can lead to corneal infiltration, ulceration and even perforation. It can cause ophthalmia neonatorum. Streptococci can cause preseptal cellulitis or frank orbital cellulitis. Preseptal cellulitis is common in children. Endophthalmitis due to streptococci has uniformly poor prognosis. It is worst with S pyogenes and slightly better with S. viridans. Mild to moderate anterior uveitis is also known to be due to streptococci. Management Most of the strains of streptococci are sensitive to penicillin. Alternate drugs include Cephalosporins, erythromycin, clindamycine, tetracycline and chloramphenicol. Enterococci

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are generally resistant to penicillin and cephalosporins. They respond well to vancomycin and aminoglycocides. Spirochaetaceae8, 9 There are many organisms in this group. The most important out of them is treponema pallidum that causes syphilis. The other two are leptospira and Borrelia. The former cause leptospirosis, the latter results in relapsing fever, vincent angina and lyme disease. Syphilis is one of the most widely spread chronic disease that can infect at any age and both the sexes in all races. It can be congenital or acquired. The organism treponema pallidum is a spiral shaped, thin. delicate looking organism that has 6 to 14 spirals along its long axes. It is a motile organism. It is best seen on dark field examination. It can not be cultured invitro. Humans are the only known natural host. The disease is mostly sexually transmitted though it can be acquired following blood transfusion with infected blood. Congenital syphilis is mostly due to transplacental spread from infected mother to the growing foetus generally in the third or fourth month of gestation. However, older children can acquire it following child abuse. In such cases they go through all the stages of adult acquired syphilis i.e. the primary, secondary, latent and tertiary stage. Systemic involvement in congenital syphilis The disease can be transmitted to the foetus any time during pregnancy if the mother is serologically positive. It develops more commonly by fourth month. Mothers in early stage of infection are more likely to infect the foetus than those who had infected period of more than two years. Early treatment of the mother before sixteen week is said to protect the foetus. Abortion and still births are two common modes of termination of congenital syphilis. It is a major cause of neonatal death. The clinical presentation of congenital syphilis in surviving neonates can be divided into three stages: 1. Early features that appear within first two years of age. 2. Late manifestation that develop after two years 3. Residual effect of late manifestation The systemic manifestations differ in infants and older children. InfantsPresence of congenital syphilis is not felt at birth. They become obvious by second to tenth week post natal with hepato spleenomegaly, jaundice, anemia thrombocytopenia, lymphadenopathy. Skin rashes that may be bullae or vesicle, rhinitis, osteo chondritis. The ocular signs are absent at this stage: Older childrenAnterior bowing of shin, periostitis, frontal bossing, Clutton joints, saddle nose, mulberry molars, peg shaped teeth, eighth cranial nerve involvement and ocular manifestation. Ocular manifestations are late manifestation of congenital syphilis that has remained untreated for more than two years.

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Commonest ocular manifestation of congenital syphilis is interstitial keratitis that develops between two to twenty years, is a bilateral condition. It is always associated with sever anterior uveitis that is associated with endothelial edema, leading to generalised edema of the cornea and development of deep vascularisation. The deep vascularisation gives rise to salmon patch appearance, which gradually subsides to leave obliterated corneal vessels as ghost vessels. It takes years for the vessels to regress. A ground glass appearance of the cornea is common. Other features are bilateral chorioretinitis, which gives an appearance of salt and pepper pigmentation also known as pseudo retinitis pigmentosa. Secondary glaucoma is common due to changes in the angle secondary to anterior uveites. The differential diagnosis of interstitial keratitis include other condition of hazy cornea in childrenCorneal birth injury, late corneal injury, congenital glaucoma, corneal endothelialdystrophy, congenitalrubellasyndrome, mucopolysaccharidosis, mucolipidosis. Conditions that give pepper salt appearance of the retina besides congenital syphilis are rubella, cytomegalovirusretinitis, influenza. Neonatal congenital syphilis should be differentiated from - Rubella, cytomegalo virus infection, toxoplasmosis, herpes simplex and erythroblastosis fetalis. Management The condition is preventable if the mother is adequately treated before sixteenth week of gestation. The infected child is treated with aqueous crystalline penicillin in consultation with pediatrician. Ocular condition is treated with atropine and local steroid over months. Leptospirosis Leptospirosis as an organism that belongs to order spirochaetales. Leptospirosis is less frequent disease. It is seen as epidemics in certain parts of tropical and subtropical countries where financially less privileged persons live in unhygienic conditions in close contact the animals. The disease is zoonosis, is spreads commonly from certain animals to humans. Human to human spread is possible but less frequent. Drinking water contaminated by urine of infected animals or human beings is also a mode of spread. Many animals, fish or birds are the reservoir of the organism. The organism is a spirocheate, it is a thin highly motile spiral organism that stain poorly but is clearly visible on dark field examination. Systemic involvement Many animals act as reservoir for the organism. These animals rarely develop the disease. Humans acquire the disease by contact with urine or tissue of infected animals, or by drinking contaminated water. The organism commonly reaches the body through abraded skin or mucous membrane. The incubation period is one to two weeks. The organism can be recovered from blood, CSF, aqueous within twenty four hours.

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The virulence depends on toxin production. Clinically the disease has two stages1. Phase of leptospiracmia 2. Immune phase The first phase consists of influenza like symptoms of pain all over the body, fever, chills, skinrashes, gastrointestinal disturbance, respiratory infection. One of common findings at this stage is conjunctival effusion. The second phase lasts for several weeks. The child may become asymptomatic or may have serious complications like encephalitis, Guillain-Barre syndrome, multiple cranial nerve palsy, peripheral nerve involvement and myocarditis. Ocular manifestations are common and variable. Commonest finding is conjunctival effusion without lacrimation or discharge. If there is discharge, it may contain leptospira. Other ocular manifestations areIridocyclitis that may be mild and self limiting. Rarely may have chronic uveitis. Neovascularisation of iris, hypopyon, mutton fat KPs, broad posterior synechia, complicated cataract and secondary glaucoma. Retinal haemorrhage, choroiditis, chorio retinitis, vitreous membrane have also been reported. Management consists of: Prevention by personal hygiene, food hygiene, drinking potable water and chemo prophylaxis during epidemics by way of 100 mg doxycyclin once a day for a week in adults. The organism is sensitive to ampicillin, erythromycin, doxycyclin, tetracyclin. Severe cases may require injection IV penicillin G. Conjunctivitis does not require any separate treatment if the patient is on systemic antibiotic otherwise erythromycin ointment or fresh penicillin drops may be required. Iridocyclitis is treated by standard method. Lyme disease The disease is spirochetal disease caused by Borreliaburgdorferi. It is a multi systemic disease. It is one of the arthopod related diseases that is spread by bite of tick ixodes. Many mammals are host of the tick. Systemic involvement The disorder involves multiple symptoms. Common areSkin rash. neurological signs consist of Bells palsy, other cranial nerve palsy, meningitis, peripheral neuritis, late presentation are arthritis and peripheral neuropathy. Ocular involvementAny part of the eye may be involved. They can be divided into two groups i.e. secondary to neurological causes and those due to involvement of the eyeball proper. These are mostly immune mediated inflamations. The signs and symptoms of both the groups can run simultaneously. They are facial palsy, paralytic squint, optic neuritis, retrobulbar neuritis, optic atrophy, pseudo tumour cerebri, papilledema, conjunctivitis, symblepharon, keratitis, episcleritis. Intra ocular manifestations areIritis, parsplanitis, and chorioretinitis. The involvement may be unilateral or bilateral. One eye may be involved later than the other may. The lesion can be single or multiple.

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Management The disease mimic many diseases. It is relatively rare hence knowledge of the disease and high degree of suspicion is required. Elisa test and immuno fluorescent assays are the two investigations that may clinch the diagnosis. The organism is sensitive toPenicillin, amoxycillin, erythomycin, roxithromycin, tetracycline and doxycyclin. Ocular management consists of management of Bells palsy, conjunctivitis, keratitis and episcleritis by standard methods. Proteus This is a saprophyte that is found in decaying organic matter, soil, water and GI tract of many animals and human beings. Generally infection by proteus is mild and may be asymptomatic unless they become opportunistic and cause wide spread infection. The organism is gram negative, actively motile, non lactose fermenting. It possesses an enzyme urease, making it a potential urinary tract infective organism. Systemic manifestationCommonest infection is urinary tract infection. The urinary tract acts as portal of entry for other organs. Proteus invades immuno compromised individuals and debilitated individuals. It is a known contaminant of wounds, accidental or surgical and burns. Other systemic manifestations areOtitis media, mastoiditis, lateral sinus thrombosis, meningitis, even brain abscess. Ocular manifestationThough external ocular infections like conjunctivitis, keratitis is known to be caused by proteus, they are mostly due to trauma. However proteus is a potentially blinding organism following penetrating injury that may be accidental or surgical. Rarely it can cause dacryocystitis and scleritis. Management The organism is sensitive to many antibiotics i.e. ampicillin, third generation cephalosporin, all aminoglycocides. Ocular treatment is by fortified gentamycin 14 mg/ml or tobramycin 14mg/ml frequently or by 0.3% ciprofloxacilin or ofloxacilin 0.3% drops hourly. Endophthalmitis following either accidental or surgical is a serious condition requiring intra vitreal injection of gentamycin or amikacin. Vitrectomy may be required. SalmonellosisTyphoid (enteric fever) Typhoid is one of the common acute febrile conditions in all ages. It is very common in children. It is a preventable public health problem that may cause death if not treated. There are many organism in the salmonella group, only few cause human infection. The salmonella typhi the causative organism for typhoid fever is found only in humans. The disease is transmitted by ingestion of contaminated drink or food. The disease also spreads due to person to person contact during acute phase. It can also spread from asymptomatic carriers. Faecal oral contact is commonest mode of spread in all ages, more so in children.

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S. typhi is a gram negative, motile, facultative anaerobe. Incubation period varies between 3 days to 60 days. Systemic involvement is basically enteritis, which is heralded by fever that is prolonged and persistent. There is step like rise of temperature, rose spots all over the body. Relative bradycardia is supposed to be a strong suggestive factor. There may be diarrhoea or constipation. The small intestine is commonly involved. There is hyperplasia of Peyers patches, mucosal ulceration, bleeding and perforation. The child is generally delirious, has severe anorexia and loss of weight. The child may recover from these early phases or may pass into more widespread systemic involvement that includes almost all systems. There may be meningitis, encephalitis, paralysis of cranial nerve, pneumonia, bronchitis, myocarditis, cholecystitis, osteomyelitis, arthritis. The child becomes afebrile with adequate antibiotic in four to seven days. In about 10% cases there is relapse. About 2% to 3% cases develop chronic carrier status and public health hazard. Ocular manifestations are rare, when present are due to hematological spread of the organism. They areLid abscess, dacryoadenitis, hemorrhagic spots on the conjunctiva, keratitis, uveitis, vitreous and retinal haemorrhages, paralysis of extra ocular muscles. Management : ProphylaxisThe disease is preventable if environmental, sanitation, water supply and sewage disposal could be improved. However if all children are supplied with potable water and instructed to follow proper food hygiene, the incidence falls sharply. Other prophylactic method is to give prophylactic oral vaccine and repeated every two years. The first antibiotic found to be effective against typhoid was chloramphenical. The drug though very effective had many untoward side effects. The chloramphenical was followed by a long list of antibiotics that includes-Trimethoprim-sulphamethoxazole, ampicillin, amoxycillin, fluoroquinolones, third generation cephalosporins. Ocular manifestations are treated by standard methods along with systemic antibiotics. Ocular manifestation of systemic virus disease11, 12, 13, 14 Viruses are smallest infective organisms. They are called primitive organisms because they contain only one single nucleic acid either DNA or RNA. The viruses consist of nucleic acid surrounded by one or more proteins. The viruses themselves can not replicate for which they require a host-cell, hence they are called obligate intracellular parasites. Some of the viruses have an outer cover membrane that protects and stabilizes the nucleoid from environment outside and helps the virus to invade host cell. The outer cover, which is protein in nature, is responsible for antigenicity of the virus. On which depends the immunity and diagnostic tests. The antigenecity is usefully utilised to manufacture vaccine. The invading viruses get attached to a special receptor site on the host cell membrane. The viruses are then pagocytosed. The viruses do not possess enzyme required for reproduction and survival. They depend on the host cell for the enzyme, energy and precursor for multiplication. The RNA viruses replicate in the cytoplasm of the host cell. The DNA replicate in the nuclei.

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The RNA viruses that have ocular involvement are Entero virus (Picorna viruses)They includePoliomyelitis, Coxsackie virus and ECHO viruses. Paramyxo virusesMumps, rubella and measles viruses. Human immuno deficiency virus The DNA viruses that have ocular involvement are Pox virusesSmall pox virus (variola) and vaccinia virus. Herpes virusesChicken pox (varicella), herpes simplex, herpes zoster, and cytomegalo viruses. Other virusesMolluscum contagiosum, human papilloma virus and human adeno viruses, Epistein-Barr virus. Poliomyelitis This viral disease has world-wide distribution. Though it has almost been eradicated in developed countries, it remains a major cause of death and physical handicap in developing countries. Its ocular manifestations are few and overshadowed by motor paralysis and deformity. Like any other entero virus, poliovirus is transmitted by fecal-oral route. It has a short incubation period of three to six days. Most of the time it is asymptomatic. Only five percent of children have mild symptoms of fever, malaise, and body ache that is generally passed as influenza or other viral fever. Only one percent develops aseptic meningitis which is the cause of most visible sequel i.e. motor paralysis. The central nervous system involvement has been divided anatomically into- meningeal, encephalic, bulbar, cerebellar and spinal. The bulbar form is most likely to cause ocular involvement in the form of cranial nerve palsy. Commonest extra ocular muscle to be involved is lateral rectus. Involvement of fourth nerve is least common. Some children may develop seventh nerve palsy. Nystagmus is very frequent. Pupillary abnormalities are part of third nerve involvement. Horners syndrome develops rarely. Involvement of optic nerve is the cause of transient visual loss. The ocular involvements are self limiting. Coxsackie virus They cause less ocular manifestation than poliomyelitis. Systemic infection may lead to necrosis of skeletal muscles, tremor, spasticity, flacid paralysis, encephalo myelitis, aseptic meningitis. The ocular involvements are either secondary to involvement of CNS or due to primary infection of conjunctiva and cornea resulting is kerato conjunctivitis, keratitis, phlycten, corneal pannus, pseudo membranous conjunctivitis. ECHO viruses produce ocular manifestation similar to Cox sac kie virus. Mumps Mumps is common acute contagious disease of childhood. Commonest presentation is bilateral, almost symmetric painful swelling of parotid glands. The condition has a

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prodromal stage of fever, body ache, anorexia, and malaise. It gives a life long immunity. Life long immunity is also acquired following immunisation. The virus is a paramyxo virus, which is a RNA virus. It is one of the smaller viruses. It is pleomorphic. The disease spreads as droplet infection via saliva and fomitis. The incubation period is 14-20 days. Systemic involvement After prodromal period that may last for one to two days, the child develops fast growing bilateral swelling, difficulty in swallowing and pain in the ear. It is common to develop similar swelling in the sub mandibular glands. Sub mandibular glands are always associated with parotitis. The swelling of the gland increases for two to three days and then subsides gradually leaving no trace of infection. Other organs to be inflamed are testes, pancreas, ovaries. The orchitis is common in post pubertal patient. About 20% males are effected. The swelling is generally unilateral and painful. Late complication of orchitis is testicular atrophy. As testicular atrophy is unilateral it does not lead to sterility in men. Oophoritis is less common, presents as pain in lower abdomen and does not cause sterility. More serious manifestations are Aseptic meningitis, encephalitis, transverse myelitis, cerebellar ataxia, facial palsy. Ocular involvement Commonest ocular involvement is acute dacryoadenitis, which is bilateral, may cause narrowing of palpebral fissure and mild proptosis. Other ocular involvements are conjunctivitis, keratitis, scleritis, iridocylitis. There may be sub conjunctival haemorrhages and rarely interstitial keratitis. Bilateral optic neuritis is self limiting and has good prognosis. Rarely extra ocular palsy or pupillary abnormality may develop. Management : Prophylaxis An effective mumps vaccine is available that is administered as part of MMR vaccine that includes measles and rubella vaccine. It is generally administered after 12 months of age as subcutaneous injection. It gives life long immunity. TherapeuticThere is no known antiviral drug effective against mumps virus. The management is symptomatic and supportive in the form of analgesic. Role of steroids is not established. Ocular management is symptomatic by standard methods of antibiotic drops, cycloplegics and local steroids. Rubella (German measles) This RNA paramyxo virus causes a serious systemic infection that is fully preventable. It causes multiple crippling congenital anomalies in the foetus if the mother acquires the disease in first trimester. Infection of the mother after fourth month does not cause much damage to

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the foetus but the foetus is not altogether safe. The infection gives a life long immunity, subsequent pregnancies are safe. The disease occurs in endemics in population that have not been immunised. The disease spreads by droplet. The incubation period is 12 to 23 days. The infected droplets cause infection in the respiratory tract which is followed by hematogenic spread. There are two modes of presentation 1. Common mild post natal infection that may go unnoticed. 2. More serious life and vision threatening congenital infection Commonest age to get post natal infection is school going children. This does not mean that other ages are immune. It can occur at any age. Rubella is contagious in all ages even in asymptomatic cases. The disease has a prodromal phase of malaise, fever and anorexia. The actual infection is always associated with suboccipital, postauricular lymphadenopathy, fever and rashes. Congenital rubellaMaternal infection during first trimester is most important cause of severe multiple congenital anomalies in the developing foetus. The foetus is infected transplacentally. The classical signs of congenital rubella is rubella syndrome that includes cataract, heart disease and deafness. Other systemic involvements areLow birth weight, retardation of growth, hepatospleeanomegaly, pneumonia, thrombocytopenia. The common cardiac anomalies arePulmonic stenosis and ductusarteriosus. Other anomalies are dental anomalies, cleftplate, and microcephaly. The ocular manifestations are There are hardly any ocular involvement in acquired rubella except catarrhal conjunctivitis and superficial keratitis. Ocular manifestations of congenital rubella are many. They areMicrophthalmos, central cataract and retinopathy. Less common ocular features are congenital glaucoma, corneal haze, iris hypoplasia, nystagmus and squint. Late complications include progress in retinopathy, subretinal neovascularisation and maculopathy. The rubella cataract is bilateral, almost symmetrical, slightly eccentric, dense, nuclear type. The lens is known to harbour live virus up to two years of age. Hence surgery should be followed by frequent and prolonged use of local steroids and cycloplegic with usual caution. Every effort should be made to aspirate the cortical matter as much as possible. The congenital glaucoma develops later than cataract and is severe in nature. The cause of glaucoma is multifactorial i.e. spherophakia, microphthalmos, anomalies in the angle, secondary to uveitis or post cataract surgery status. Measles (Rubeola)16 Measles is a common acute exanthematous respiratory disease, which is highly contagious resulting in epidemics. The disease is potentially fatal. It is met with all over the world,

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more in under develop countries. The organism belongs to family paramyxo viridae and genus Morbilli virus. There is only one genetic type. Humans are the only natural host. Systemic involvement The disease is mostly seen in children between 6 months to 2 years. The disease spreads by droplet infection. The disease is most contagious from one to two days before the onset of symptoms and remains so for four days after the rashes have appeared. The incubation period is of 10 days. The disease has following stages: 1. The prodromal stage 2. Stage of exenthema 3. Complications In prodromal stageThere is fever, rhinitis, cough, Koplic spots in the buccal mucosa, and conjunctiva. The Koplic spots are blue-white spots against pink background. In stage of exenthema, the body is covered with rashes. They last for 3-4 days and then disappear without leaving any scar. The complications are related to respiratory system, central nervous system and GI tract. It is not uncommon for more than one system to be involved at the same time, which includes laryngitis, pneumonia, otitis media, deafness, encephalitis, gastroenteritis. The ocular involvement The conjunctiva is involved in prodromal stage and continues to be infected through the exanthematous stage. Commonest conjunctival involvement is catarrhal conjunctivitis, Koplic spot, swelling of plica, edema of lids. This is followed by severe photophobia due to epithelial keratitis. The cornea if infected by bacteria at this stage is bound to end in corneal ulcer, which may perforate if the child is malnourished with vitamin A deficiency. Other ocular manifestations are dacryocystitis15 and frequent stye. The neuro ophthalmic complications due to measles are - Encephalopathy, that may lead to optic neuritis, papilledema, or paralytic squint. Measles and vitamin A deficiency Severity of measles is more in malnourished children. Death is more frequent in malnourished child. Children with borderline vitamin A deficiency develop night blindness and xerophthalmia soon. Measles itself can cause protein caloric malnutrition. It also depletes liver store of vitamin A. Management ProphylaxisMeasles is a fully preventable disease by live attenuated measles vaccine. All children between 6 months to 6 years should get oral vitamin-A 1,00,000 IU. Systemic involvementThere is no specific antiviral drugs against measles. Only symptomatic and supportive treatment is required with a close watch on possibility of pneumonia, enchephalopathy and gastroenteritis which require special attention. Ocular involvement is treated by local antibiotic, cycloplegic and non steroidal anti inflammatory drugs.

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Once the child has been diagnosed to have measles, irrespective of his nutritional status, the child should get extra vitamin A. Common practice is to give 50,000 IU under six month, 100000 IU under one year and 200000 IU over one year in two equal divided doses. Human immuno deficiency virus17,18 Human immuno deficiency virus contains RNA but no DNA. The virus uses hosts cellular mechanism to form DNA for its reproduction, depleting the immune system of the host. The human immuno deficiency virus type 1 (HIV 1) causes a multi systemic chronic contagious disease called acquired immuno deficiency syndrome (AIDS). It takes years to develop the systemic and ocular involvement in AIDS. Two third of the patients with AIDS will develop ocular symptoms. The condition is uniformly fatal. Presently available drugs do not change the immune deficiency but act against mostly the opportunistic organisms. The disease is spread by body fluid. It is most commonly transmitted sexually. It can also be transmitted via infected blood products. The mother can pass the infection to the foetus via placenta. The neonate may acquire the disease during passage through infected birth canal. The breast-fed baby may get it from mothers milk. All body fluids including tears, aqueous, or saliva may be source of infection. Older children get infected either through infected blood products, infected needles or following sexual assault. The HIV virus has affinity for CD4+ lymphocytes. The virus cripples the CD4+ cells, resulting in diminished and later deficient host immunity. The diagnosis is confirmed when: 1. There is confirmed diagnosis of HIV I infection. 2. The CD4+ cell count is less than 200 cells/ mm3. 3. There is evidence of atleast one opportunistic infection. 4. There is some AIDS related malignancy. The ocular infections are early to develop. The ocular lesions can be: 1. Retinal micro angiopathy 2. Ocular infection: (i) Intra ocular (ii) Extra ocular. By one or more opportunistic organisms The opportunistic organism can be viral, bacterial or protozoal. More than one opportunistic infection may be present. 3. Uncommon neoplasms 4. Neuro ophthalmic complications Retinal micro angiopathy is commonly known as HIV retinopathy, is the commonest ocular manifestation. The angiopathy is due to direct invasion of endothelium of the retinal vessels. The result is development of cotton wool spots, micro aneurysm, ischemic maculopathy. The lesions are produced by deposition of immune material in the vessels after the endothelium has been invaded by the virus.

750 Ocular infection Intra ocular infectionRetinitis

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Common opportunistic organisms areCytomegalovirus, herpes simplex, herpes zoster, toxoplasma, pneumocystiscarinii, mycobacterium, candida, and cryptococci. Commonest and early intra ocular infection is cytomegalovirusretinitis (CNV). The virus is member of herpes family. The CNV affects neonates and adult immuno compromised patients. Almost one third of AIDS patients develop CNV in under developed countries where triple anti viral therapy is not available. The lesions develop along the retinal vessels due to invasion of the endothelium by the virus. The lesions are white granular dots. Superficial haemorrhage are common. Other findings are - Retinal edema, retinal vasculitis, and full thickness necrosis of the retina. The scattered spots, coalase to form larger area of necrosis that is replaced by gliosis. Posterior hole formations are common, leading to retinal detachment that is difficult to treat. Early symptoms include diminished distant vision, floaters, central or ring shaped scotomas. Treatment of CNV retinitis is difficult and costly. It includes IV ganciclovir for fifteen days followed by oral maintenance dose. Other mode of treatment is called highly active anti retro viral therapy (HART) which is a combination of anti retroviral drug and protease inhibitor. The anti retro viral drugs can also be given intra vitreous. Progressive outer retinal necrosis (PORN) This is rarer opportunistic infection in AIDS. It is caused by herpes zoster and other herpes simplex viruses. It starts as necrotising lesion of the peripheral retina that progresses relentlessly in circumferential fashion without involving retinal vessels. The condition is potentially blinding disease; about 80% of patients with PORN go blind inspite of treatment. Uveitis Multi focal choroiditis is caused by many organisms. The infection may be due to a single organism or a combination of organism like mycobacteriumtuberculosis, atypical mycobacteria, pneumocystitiscarinii, cryptococcus, toxoplasmosis. In contrast to multi focal choroiditis there may be solitary choroiditis due to syphilis which is not an opportunistic organism because those who develop syphilitic choroiditis have CD4+ cell count >200. It generally occurs in patients with neuro-syphilis. The other intra ocular manifestations are non-granulomatous iritis, optic neuritis, retrobulbar neuritis, and papilledema. Extra ocular involvement may be due to infection by opportunistic organism, neoplasm or CNS involvement. They are: Follicular conjunctivitis, keratitis, punctate or geographic that may look like herpes simplex keratitis. Neoplasms associated with AIDS are: Kaposis sarcoma, Burkitts lymphoma, squamous cell carcinoma of the conjunctiva.

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Kaposis sarcoma is very aggressive neoplasm of endothelial cells. About one fifth of persons with AIDS develop Kaposis sarcoma, lesions of the skin of the lids are more common than conjunctiva. Orbital involvement is still rarer. CNS involvement may cause encephalitis, meningitis, papillitis, papilledema, retrobulbar neuritis and extra ocular muscle palsy. Smallpox virus (Variola) Variola was a common, often fatal that left surviving children with life long scarred body and many a times blindness is no more seen. It has been eradicated in the year 1980 as a result of simultaneous global immunisation. It was a common cause of bilateral anterior staphyloma which can still be seen in patients in remote areas, who were borne before 1977, when the last case of small pox was seen. Vaccinia This is also a DNA virus that is closely related to smallpox virus but not identical to it. The two conditions have different presentation, mode of infection and host range. Vaccinia virus is used as prophylaxis against smallpox. Primary systemic vaccinia does not occur but is a distinct possibility. The vaccinia presents as auto inoculation, contact inoculation, complicated scar of vaccination and rarely vaccinia encephalopathy. The lesion following auto inoculation or contact inoculation resembles single blister of small pox. The pastular lesion develops three days after vaccination; it can be anywhere on the body but more common on the face. The ocular involvement is mostly in the lids. Less common are the conjunctival and corneal involvement. There is intense swelling of the lids, the preauricular lymph glands may be enlarged. It takes eight to ten days for the condition to subside. Conjunctival lesion may be a pustule associated with purulent conjunctivitis. Pseudo membrane may form. The cornea is involved secondary to lid lesion. It may be acute or chronic. The former is in the form of superficial punctate keratitis of mild nature that heals without any treatment unless contaminated by bacteria. The chronic form is a severe but rare disciform keratitis. Rarely there may be iridocylitis and peri vasculitis. No specific treatment is required. Herpes viruses This group consists of herpes zoster, varicella (chicken pox), herpes simplex and cytomegalo virus. Varicella (chicken pox). This is very common, benign, highly contagious disease of childhood seen world wide. Unlike small pox there is no universal immunisation against chicken pox. The virus that causes chicken pox causes herpes zoster in adults. However it should be kept in mind that herpes zoster ophthalmicus is seen in pediatric age group as well. The virus, which is more commonly, called varicella zoster virus looks similarly to herpes simplex virus, but differs in chemical properties, antigenically and clinical presentation.

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The chicken pox, which is an acute exanthematous disease spread by droplet infection. The incubation period is of 10-20 days. The patients are infectious two days prior to development of rashes. The patient remains infectious during the vesicle formation and during crusting. The skin lesions are the most visible signs of the disease. The lesions begin as maculopapules, gradually going to vesicle that scab in about ten to fifteen days. The scabs fall leaving no permanent scar. The lesions can be seen on all exposed mucous membranes and skin. The skin lesions are associated with fever. The complications arise if there is secondary bacterial infection superimposed, or the child is immune compromised. Extra cutaneous lesions are seen mostly in CNS in the form of meningeal irritation and ataxia. There may be meningitis, encephalitis, and transverse myelitis. Varicella infection of the respiratory tract in the form of vericella pneumonia is most serious complication. The ocular manifestations areVesicles may form on the lids and conjunctiva. Infection may spread to the lacrimal sac from conjunctiva or through nasopharynx. Chicken pox is a common cause of chronic dacryocystitis in children.15 Corneal involvement can occur at the vesicular stage as punctate keratitis or may develop disciform keratitis late. Herpes zoster13, 19, 20 Herpes zoster is a sporadic disease mostly seen in adults but not an exception in children. Manifestation in children is milder and less painful. The disease in all ages in caused by same virus i.e. vericella herpes zoster virus. It is equally seen in both the sexes. Herpes zoster is a unilateral disease. There is no predication for right or left side of the body. The virus involves one single dermatome at a time. Both cranial nerves and spinal nerves are involved. Simultaneous involvement of cranial and spinal nerve never occurs. The commonest dermatomes involved are T3 to L3. Commonest cranial nerve to be involved is trigeminal. Out of three divisions of fifth nerve, the 1st division is most commonly involved followed by maxillary. The third division is least involved. It is not uncommon to have involvement of only few branches of 1st division, commonest being supra orbital. The involvement of first division of the trigeminal is called herpes zoster ophthalmicus. The infection starts most probably as varicella in the childhood and the organism lies dormant in the dorsal roof ganglion and manifests only when the organism is reactivated. Most of the patients do not have recent history of exposure. It is said that about 2 percent of patients may develop second attack of herpes zoster.19 The typical lesion develops as unilateral vesicular eruption along the dermatome anywhere on the body that may be painful or preceded by pain. Most of the lesions will be followed by post herpetic neuralgia that is more marked in adults. Older the patient greater is the pain, eruption in the distribution of cranial nerves is more painful than those of spinal nerves. The vesicular lesions look very similar to those of varicella. Generally it takes 7 to 10 days for the vesicles to crust and leave hypo pigmented shallow scars. There is hyposthesia along the dematome.

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In herpes zoster ophthalmicus, the posterior roof ganglion involved is trigeminal ganglion. The geniculate ganglion is involved in Ramsay Hunt syndrome that includes Vescicles in the external auditory canal, pain in the ear, loss of taste in the anterior two third of the tongue. Most troublesome part of herpes zoster of spinal nerve is acute neuritis, result in pain, absence of touch sensation. This lasts for months to years. Other complication areMeningeal irritation and granulomatous angitis that may result in contra lateral hemiplegia, transverse myelitis and cranial nerve palsy otherthan the fifth and seventh. Most important systemic complication of vericella herpes zoster virus infection is cutaneous dissemination where the skin lesions last for weeks. Other causes of dissemination are herpes zoster pneumonia and meningo encephalitis. Ocular manifestation of herpes zoster21 is herpes zoster ophthalmicus (see page 52). Some important points are: 1. Ten percent of all cases of herpes zoster infection are herpes zoster ophthalmicus. 2. Fifty percent of cases of herpes zoster ophthalmicus develop serious complication. 3. Ocular involvement is possible if maxillary branch is involved, may involve nasociliary nerve. 4. Lacrimal branch is generally spared. 5. Hutchinsons sign is not a sure sign of corneal involvement. 6. Corneal hyposthesia even anaesthesia may develop and linger for years. 7. Anaesthesia dolorosa is common. 8. Lagophthalmos may develop in Ramsay Hunt syndrome. 9. Other extra ocular muscle palsy is possible which recover spontaneously in few months. 10. In acute stage mucopurulent conjunctivitis is common. 11. Corneal lesions are many. They may start as superficial punctate keratitis or micro dendrites; may become nummular or even disciform lesion may develop. 12. Episcleritis and scleritis are generally over shadowed by skin and conjunctival lesion. 13. Iridocyclitis is common. 14. Secondary glaucoma is frequent. 15. Optic neuritis has been reported frequently. Herpes Simplex virus Herpes simplex (HSV) infection is a world wide health problem that is seen in all ages including neonates, in both the sexes. About 90% of adults are sero positive for herpes simplex virus.

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Herpes simplex virus is a complex DNA virus. There are two types of herpes simplex viruses i.e. type I and type II. The latter is also known as genital virus that can infect the eye. It is acquired sexually and from the genitals spread to other parts of the body after a period of latency followed by reactivation. It can also involve the eye. The type I generally infects the body above waist. The lesions spread by infected saliva. The infection is said to be primary when it develops in a non-immune person through infected secretions. It is generally acquired in early ages. The primary infection can be subclinical or clinical. The infection then travels to the sensory ganglion, serving the area of the primary infection i.e. trigeminal ganglion for HSV1 and spinal ganglion for HSV2. The virus remains inactive in the root ganglion for variable time called latency, then following some trigger mechanism the virus travels down the axon to the target tissue and cause recurrent infection that keeps on recurring with period of apparent cure only to recur at irregular intervals. The incubation period is 6 to 8 days. The infection starts either through mucous membrane or via an abraded skin. The foetus can get the infection via placenta in an infected mother. The neonate gets the infection from the birth canal of the infected mother.24 Systemic involvement depends on the type of the virus, i.e. type 1 or type 2, site of the primary lesion, target tissue, and immune status of the patient. Except for neonates, the herpes simplex virus is not a lethal virus. In immuno compromised especially in patients suffering from AIDS the organism behaves like an opportunistic organism and hasten the death. The systemic involvement consists of: 1. Labial infection, vesicles on the lips or muco-cutaneous junction of the mouth 2. Infection of the upper respiratory passage i.e. pharyngitis, tonsillitis. 3. Upper GI tractgingivitis, stomaititis, infection of hard pallet, tongue, soft pallet. 4. Genital infection 5. Infection of central nervous system 6. Infection of peripheral nervous system 7. Herpetic whitlow Ocular manifestations are many, some of them are benign, others are potentially sight threatening. They are: 1. Vesicles on the lid skin or at the junction of conjunctiva and skin. The vescicles that crust soon and heal without scar or loss of pigment. Sensation is normal. 2. Acute follicular conjunctivitis with enlargement of pre auricular lymph nodes. There may be pseudo membrane formation. 3. KeratitisEpithelial : Half of the children develop superficial punctate keratitis scattered over the cornea. They become coarse within few days. This is followed by dendritic ulcer. The dendritic keratitis begins as coarse epithelial lesions in a linear form, which later develops branching. The branching is on one end, new lesions

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develop at the terminal of the branching. The dendrite heals from one-end and progresses at the other end. The course of events may be one of the following: (i) The dendritic ulcer heals within ten to twenty days with treatment, may even heal without treatment. (ii) The dendritic figure expands in width and a geographic or amaeboid ulcer develops. Rarely the condition may pass into disciform keratitis. (iii) Stromal Necrotic keratitis. This is due to invasion of the stroma by the virus. Some times the epithelium over the lesion may be intact. In majority of cases the epithelial defect persists. (iv) Disciform keratitis. This may appear following dendritic ulcer or even without it. It is a hypersensitive reaction. The disciform lesion is central, oval diffuse. It may be sub-epithelial lesion with stromal thickening surrounded by ring of infiltration. There may be folds in Descemets membrane. Anterior uveitis is common at this stage. The condition takes few weeks to months to clear. 5. Keratitis meta herpetica. This is not caused by viral invasion neither is an immune reaction. It is a persistent defect in the basement membrane. 6. Loss of corneal sensation is common. It starts from the stage of superficial punctate keratitis and lingers for months. 7. Uveitis is a common feature of HSV. It may be associated with keratitis at any stage or happen independent of keratitis. The uveitis is generally acute. That may lead to iris atrophy. 8. Secondary glaucoma is very frequently associated with HSV keratitis and uveitis and may be the cause of severe pain disproportionate to corneal lesion. 9. Superficial and deep vascularisation are common in long standing epithelial and stromal defects. (For details see chapter on cornea) Cytomegalo virus Cytomegalo virus is a DNA virus, morphologically similar to herpes simplex. The exact route of infection is not known. Percentage of persons with positive titer is high. It is estimated that about 4.5% pregnant women excrete virus in urine. The virus is also secreted in breast milk and saliva. Systemic involvement The condition can be congenital or acquired, can be seen in neonates or in adults. Immuno compromised person are very often infected by the virus. The foetus gets the infection from the infected mother. The neonate can be infected during birth via infected birth canal. Congenital CMV infection can be asymptomatic or the neonate may be seriously ill. Common features are under weighed child, microcephaly, hepatospleenomegaly, thrombocytopenia, patechial haemorrhage. There may be intra cranial calcification. There may be asymptomatic chorioretinitis.

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Acquired CMV differs in presentation in immuno competent and immuno compromised. In immuno competent, it presents as mono nucleosis, mostly in sexually active males. Incubation period is 20-60 days. There is fever with chills, body ache, headache, spleenomegaly; there may be pneumonitis. CMV infection in immuno compromised person is a serious infection. It is a multi systemic disease. Ocular manifestations is chorioretinitis. In neonates it is similar to toxoplasmic chorio retinitis. In AIDS, it is bilateral and extensive potentially blinding condition.

Molluscum contagiosum11,22,23
This is a DNA virus in the family of poxviridae. It has limited systemic involvement in normal healthy persons except the skin lesion which are self healing. In immuno deficient, it has prolonged course and the lesions are far more in number than in immuno competent. Its significance has increased since it has been observed to be too frequent in patients with AIDS. The disease involves the skin mostly, however, the mucous membranes are also known to be involved. The exact incubation period is not known. The commonest mode of spread is by direct close contact with the patient. The infection spreads to other parts of the body from the initial site by auto inoculation. Sexual transmission is also known. It is known to spread via swimming pools. The typical lesion can be seen anywhere on the skin. Its number vary from a single lesion to few dozens. The lesion is a translucent or pearly growth few millimeters in diameter, raised, and circular in shape. The most striking feature is umblication in the center of the lesion. A caseous material can be expressed with pressure on the lesion. The expressed material is infectious. The lesions are also known to shed virus. The common sites are face, abdomen, back and groin. It is a disease of childhood and adolescent; there is no immunity following initial infection. The ocular manifestations areSkin of the lids, lid margin, the lesion of lid margin causes chronic follicular conjunctivitis by the viruses shed from the lid lesion. The infection can spread from the conjunctiva to cornea in the form of kerato conjunctivitis. The corneal involvement consists of punctate keratitis, pseudo dendrite, superficial pannus, and corneal ulcer. A limbal nodule may also develop. There may be notching up of the lid margin and scaring of the conjunctiva. There is no specific treatment. The lesion are destroyed mechanically, chemically (tincture iodine or carbolic) by laser or cryo, only a few at a time. However patient with AIDS require intra venous cidofovir. Chlamydiasis Infection by chlamydia is very common. One of its manifestation trachoma is a major cause of preventable and treatable cause of blindness in a large part of the world. (See chapter on conjunctiva and cornea).

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Chlamydias are a group of organism that are placed in between smallest bacteria and largest virus. It has a cell wall similar to bacteria. It possess both RNA and DNA. It is gram negative, obligate intra cellular organism. The organism is sensitive to many commonly used antibacterials but not to anti virals. The organisms of chlamydia group are antigenetically similar. The three species are C trachomatis, C pneumoniae and C psittaci. The last one causes a rare form of respiratory infection that spreads from birds to men. The C pneumoniae is a frequent cause of upper respirator infection and pneumonia in children. The organism C trachomatis is exclusively human pathogen and is the cause of trachoma, sexually transmitted diseases and infection in neonates. Sexually transmitted Chlamydiasis Genital infection is caused by C trachomatis, serovars D and K, generally known as genital trachoma. Systemic involvements are: In males, non-gonococcal urethrites. Common age group is late teens and early twenties. Many of them may be asymptomatic. In males it may cause epididymitis, Reiters syndrome, proctitis. In females it causes mucopurulent cervicitis, pelvic inflammation disease that includes salpingites, endometritis and infertility. Inclusion body conjunctivitis of new born Mucopurulent cervicitis in pregnant mother is the cause of neonatal inclusion conjunctivitis. It causes neonatal trachoma in about ten percent children born to infected mothers. Chlamydial inclusion conjunctivitis in children is replacing gonococci as main cause of ophthalmia neonatorum. Many a times it is difficult to differentiate between the two. Inclusion conjunctivitis develops five to fifteen days after birth while gonococcal infection has shorter incubation period i.e. one to three days. Gonococci stains grams negative. Intra epithelial inclusion cells are seen on Giemsa stain in inclusion conjunctivitis. Inclusion body conjunctivitis in adults The second peak of incidence is seen in late teens and early sexually active adults, following either direct infection of the eye or as finger to eye infection from infected genitals. Incubation period is 5-7 days. The condition presents as mucopurulent conjunctivitis, generally unilateral with enlarged pre auricular glands. May spread to other eye after 5-7 days or more. The eyes may stick at night. There may be small area of superficial vascularisation of cornea. The conjunctivitis dies down over month without treatment, without scarring. Management Prophylaxis- All new born children should get one drop of 2% AgNO3 in each eye. It should be remembered that AgNO3 itself can cause self limiting chemical conjunctivitis. The better way is to instill 2.5% povidon in each eye. In absence of which any broad spectrum antibiotic drop may be used. Once ophthalmia neonatorum has developed, it should be treated by standard method.

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Adult inclusion body conjunctivitis is treated with 20% sulphacetamide drop three times a day along with oxytetracyclin eye ointment at bed time. Severe cases may require oral tetracycline 250 mg QID for three weeks or doxycyclin 100 mg once a day for same period. Lymphogranuloma venerum It is more of a sexually transmitted disease of genital tract and regional lymph nodes than ocular disease, however ocular manifestations are possible, which are generally missed due to lack of suspicion of the condition in children. The children are generally infected following child abuse and may pass among themselves especially in institutions like hostels or shelters for abandoned orphans. The disease is caused by C. trachomatis serovars L1, L2 and L3. The incubation period varies between 3 days to three weeks. The systemic involvement comprise of painless ulcer of genital followed by matted enlarged, inguinallymph nodes that may become tender that suppurate to form a sinus. Ocular involvement consist of: Granulomatous conjunctivitis with non suppurative preauricular lymph node, enlargement sub-mandibular. Sub clavian or neck lymph nodes may be enlarged. The other mode of presentation is unilateral edema of lid, mucopurulent conjunctivitis, keratitis, interstitial keratitis, episcleritis, uveitis and retinal hemorrhages. Diagnosis requires high index of suspicion that should be corroborated with genital lesion. Other systemic viral disease with ocular manifestation Almost, all systemic viral disease have ocular manifestations. Adeno viruses Adeno viruses are common cause of upper respiratory tract infection. They are DNA viruses. Man is the only known host. Incubation period is short i.e. two to ten days. The infection starts as pharyngitis with high fever and sub mandibular enlargement of lymph nodes. The disease is mostly self limiting. The infection commonly spreads from swimming pools. Ocular involvement is mostly acute unilateral mucopurulent conjunctivitis. The other eye may get involved in three to four days. There may be superficial punctate keratitis. No specific treatment is required. However cornea should be protected from secondary bacterial infections which may lead to frank corneal ulcer. The condition does not impart immunity. Influenza Influenza is caused by orthomyxo virus. There are mainly three types i.e. influenza A, B and C. The virus causes acute respiratory tract infection that starts in the upper part and spread to lower respiratory tract, with fever, pain all over the body and weakness. It is pandemic with episodes of out breaks mostly in winters. Though the condition is self limiting it can be fatal if it produces myocarditis, pericarditis or pneumonia. CNS involvement is rare.

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Ocular involvement consists of congestion of the conjunctiva, conjunctivitis, retrobulbar pain, superficial punctate keratitis, dendritic ulcer, dacryoadenitis, anterior uveitis and optic neuritis. There may be difficulty in near work and diplopia when CNS is involved. There is no specific treatment. Epstein-Barr virus25 This is a herpes virus that causes infectious mononucleosis. It has been blamed to be a causative factor in Burkitts lymphoma and nasopharyngeal carcinoma.26 Ocular involvement consists of conjunctivitis, membranous conjunctivitis, superficial punctate and dendritic ulcer, uveitis, chorioretinitis, optic neuritis, paralysis of internal as well as external ocular muscle, and dacryoadenitis. MYCOTIC (FUNGUS) INFECTION Fungi are micro organisms between bacteria and smallest plant. They belong to the phylum thallophyta29. The fungi are said to be plants without leaves, trunk or twigs. They do not contain chlorophyll and can not form carbohydrates. They have multiple ramified filaments called hyphae30,31. A network of hyphae is called mycelium. The fungi develop on living organism or on dead organic matter. They are either saprophytes or parasites. The saprophytic fungi can become pathogenic. Almost all fungi may become facultative pathogen if the environment is changed in their favour that can be brought about by change in the immunity of the host. An immune compromised body is more likely to develop fungal infection than an immuno competent. This has assumed an alarming situation due to rapid spread of AIDS. Prolonged use of antibiotics may eliminate the causative bacteria but encourages a relatively benign fungus to be invasive. This is worsened by use of steroid both local and systemic. General debility, diabetes, drug abuse, chemotherapy and radiation predispose mycoses. The fungi can reproduce sexually or asexually. The spores are the reproductive bodies. There are about 30-35 species of fungi that cause systemic mycoses in humans. All systemic mycoses have ocular involvement. Occasionally fungi may get access to the ocular structure following trauma that may be accidental or surgical. The pathogenic fungi have been put in two broad groups: 1. SchizomycetisNocardia and Actinomycosis (Actinomycosis is no more considered to be a fungus. It has been put between bacteria and higher moulds). 2. Fungi imperfecti (without spores) Most of the fungi that have ocular manifestation belong to this group they cause: 1. Aspergillosis 2. Blastomycosis 3. Coccidioidomycosis 4. Candidiasis 5. Dermatophytosis 6. Histoplasmosis 7. Mucormycosis

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Fungal infection is suspected mostly clinically in predisposed patients. The predisposing factors areAIDS, prolonged use of antibiotics, steroids, immuno suppressive drugs, patient on radiation patients who had organ transplant, drug abusers, general debility, diabetics and trauma. Fungus infections are always chronic in nature. They have long incubation period except candida. Most of the fungi grow slowly in culture media, can not be developed in laboratory animals. They are identified: 1. Visually, by typical lesions on the skin, and mucous membrane. 2. Observing the characters of mycelium. 3. Microscopically by type of spores, hyphae, septae 4. Culture 5. Histologically. All fungal infection result in giant cell granuloma similar to typical tubercle. The fungi can cause immediate hypersensitivity generally by non pathogenic fungus or delayed hypersensitivity by pathogenic fungi. Thus they can cause contact dermatitis, angioneurotic edema, reaction similar to serum sickness, sensitisation and agranulocytosis. Aspergillosis Aspergillosis is caused by a saprophyte fungus which is commonly found in soil, and decaying vegetable matters. It is found all over the world but it is more common in tropical and temperate countries. The systemic manifestation starts with inhalation of spores leading to aspergillosis of respiratory tract and para nasal sinuses from where the disease is disseminated to distant organs like kidney, brain, skin and bones. Ear drum is rarely involved. Ocular involvement. Common ocular involvement are orbital granuloma and corneal ulcer, followed by endophthalmitis that can either be exogenous following penetrating injury or surgery. Less common mode is metastatic endophthalmitis due to embolus of fungus from distant organ in an emaciated critically ill child. Other parts of the eye involved can be chronic granulomatous ulcer in the lid and conjunctiva, canaliculitis and dacryocystitis. Orbital granuloma may spread to lacrimal gland. Intra ocular involvement are uveites, vitreous abscess, retinitis, retinal vasculitis. Intra ocular involvement is mostly endogenous. Blastomycosis Blastomycosis is caused by a dimorphic fungus balsomycisdermatidis, which is found in two phasesMycelial phase at lower temperature and Yeast phases at body temperature.

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Systemic involvement The fungus is found mostly in the soil. The organism is generally inhaled leading to acute pulmonary infection that may be fatal or pass into chronic phase, which is difficult to eradicate. Skin is the next part to be involved. Other organs are bones, genitourinarytract and CNS. The common presentation is agranuloma. Ocular manifestation32, 33 Granuloma of the skin of the lid is the commonest ocular involvement followed by orbital granuloma, granulomatousconjunctivitis, keratitis, rarely uveitis, endophthalmitis and panophthalmitis. Coccidioidomycosis34 Coccidioidomycosis is caused by a saprophyte coccidioidesimmitis. It is a dimorphic fungus that changes with change in environment. In one form it grows as white fluffy mould on culture media. The second form is a spherule in the host. The organism multiplies in the host by small endospores in spherules. The mature spherules on maturity rupture releasing endospores that are transformed to spherules and the cycle is repeated. The infection occurs following inhalation of airborne spherules. Systemic involvement is pulmonary infection. The disease may be asymptomatic and self limiting or may form a cavity in the lung. The fungus may spread to other organs by dissemination. The patients with altered immunity are at a risk of relapse and pulmonary as well as extra pulmonary lesions. Person with AIDS are at greater risk than other immuno compromised. Other systemic lesions are arthritis, erythema multiforme and nodosum. The ocular involvements are due to hematogenous spread from pulmonary lesion or due to hypersensitivity. The ocular manifestation are35 The hematogenous lesions areProgressive chronic pan uveitis, which is granulomatous in nature. The uveal lesions are independent of severity of pulmonary lesion. Other intra ocular lesions areChorioretinitis, vitritis, retinal exudates and haemorrhages. The cornea may show keratitis. Granuloma of the lid, conjunctiva and lacrimal gland have been reported. Candidiasis Systemic and ocular infection by candida are very common world over. There are more than hundred species of candida, out of which candida albicans is the most common pathogen. The candida is a part of the normal flora of mucous membrane, skin, gastro intestinal tract, genito urinary tract and respiratory tract. It is found in conjunctiva and on lid margin. Candida grows profusely in keratitis sicca. It is also present in the soil, water and sometimes in food. It is also a dimorphic fungus that has two phases, the yeast and mycelia. The yeast form is more common. Mode of infection is either due to local inoculation via abrasion or endogenous spread from primary systemic infection.

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Systemic involvement is common in neonates, children and malnourished children. It may be seen as oral thrush of mucous membrane of neonates and infants. Less common is chronic mucocutaneous candidiasis, that may be associated with multiple endocrinal dysfunction that may result in diabetes, Addisons disease, hypoparathyroidism, hypothyroidism. Ovarian dysfunction may be associated with pernicious or iron deficiency anaemia, hepatitis, cystitis, pyelitis, and vaginitis. The ocular manifestations are: Keratitis is commonest form of candidiasis that requires prompt treatment. It may produce pseudo-membranous-conjunctivitis, sometimes purulent conjunctivitis, conjunctival ulcer or phlycten. The lids may have blepharitis, granulomatous canaliculitis. The intra ocular involvement may be in the form of frank endophthalmitis either endogenous or exogenous following penetrating injury, accidental or surgical. Other mode of intra ocular presentation is milder form of choroiditis, retinitis, chorioretinitis, papillitis, peri vasculitis, free floating snow balls in vitreous are almost diagnostic. Dermatophytosis It is a very common mycotic infection of skin commonly known as ringworm infection. It can involve nails. According to part of the skin involved it is called tinea capitis (scalp) T corporis (body), T pedis (athletes foot), T facici (face). No systemic involvement is known. Ocular involvements. Mostly dermatitis of the lids, blepharitis, madarosis, chronic conjunctivitis. Mucormycosis36, 37, 38 Mucormycosis is a serious infection that may be fatal. It is an opportunistic fungus belonging to species rhizopus, class phycomycetis and order mucorale. The organism is found all over the world in soil, water, manure, skin. It may contaminate the food with high sugar content. The non pathogenic strain changes to pathogenic strain in diabetic ketosis, AIDS, organ transplant, lymphomas and leukaemias. The fungus is non-septate with branching hyphae that can be seen in culture and histology. The infection is acquired from the nature but not from person to person contact. Most probably the organism is inhaled or ingested. The common systemic involvements arepara nasal sinuses, orbit, brain, respiratory system, gastro intestinal tract and skin. The disease is considered to begin in the para nasal sinuses and from there it spread to adjacent structures like orbit and brain, trickle down the respiratory tract or GI tract. The main tissue to be involved is vascular, it produce invasive angiopathy that leads to ischemic or hemorrhagic necrosis. Systemic involvement The typical presentation is similar to bacterial sinusitis with fever, headache, running of nose, blood discharge from nose, facial pain, swelling of lids, and over the cheek. Common presentations are involvement of orbit and signs of CNS infection.

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Ocular involvement Commonest form is orbital involvement, most of the time due to extension from para nasal sinuses and less commonly due to injury to the skin of the lid or retained orbital foreign body. The orbital involvement starts as unilateral apical pain, headache, diplopia, and diminished vision. The lids are edematous, the conjunctiva is chemosed. Proptosis is common that may lead to cavernous sinus thrombosis, superior orbital fissure syndrome and orbital apex syndrome. Extension back into cranium leads to clouding of sensation, diminished mental faculty, cerebro vascular accident and multiple cranial nerve palsy. The diagnosis is best confirmed by histopathology of tissue removed. The biopsy or excision is generally not associated with bleeding due to pre existing vasostasis. CT and MRI are two useful diagnostic methods to demonstrate involvement of sinus and intracranial tissue. Nocardiosis Nocardiosis is caused by an organism nocardia. The organism was previously thought to be fungus along with actinomyctes. Now both are classified as higher bacteria, which are found world over in the soil and decaying organic substance. It usually affects debilitated and immuno compromised persons. It is a gram positive, filamentous organism that stain with Grocott-Gomori stain and periodic acid Schiff stain. It grows on most of the media but takes four to six weeks to grow. On agar plate the organism produces a star shaped colony which imparts the name nocardia asteroides to the fungus. They can survive inside the phagocytes. The disease is more common in adults than in children. Males outnumber females in a ratio of 4:1. Systemic involvement is mostly pulmonary as pneumonia, which is sub acute with anorexia, fever, and loss of weight. There may be pleural pain and hemoptysis. The extra pulmonary lesions are due to hematogenous spread to many organ including skin. One of the possible modes of skin involvement is trans-cutaneous inoculation that may produce cellulitis, lympho cutaneous syndrome and mycetoma. The ocular manifestations are keratoconjunctivitis, keratitis, anterior uveitis, chorioretinitis, endophthalmitis, orbital cellulitis and scleritis. Histoplasmosis38 Histoplasmosis is a chronic disease caused by fungus histoplasma capsulatum. The organism is found world over in soil and dropings of bats and birds. However its manifestation in humans is not universal. It is mostly seen in white Americans. It has rarely been reported from Asia or Africa. Though it is a disease that is commonly seen in second to fifth decade, it can infect children as well. The organism grows with ease on Sabourauds media at room temperature. The fungus is dimorphic, it shows both yeast as well as hyphae form. The yeast form is seen in various tissues causing granuloma. The hyphae develops spores at the ends. The disease is spread by liberated spores in the air. Systemic involvement The commonest mode of infection is by inhaling the small spores that on reaching lung develops budding forms. The multiplying organisms produce granuloma that caseates to necrose,

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may get calcified. The lesions mimics tuberculosis in children. The lesions may heal without much treatment and get calcified at the hilum. In some cases the infection may continue to become fatal in children and immuno compromised. The organism can travel to liver and spleen. Ocular manifestations It is not clear if the ocular involvement is really caused by histoplasma at all. The ocular involvement is called presumed ocular histoplasmosis syndrome (POHS) that is confined to posterior segment. The anterior segment is unaffected. The ocular involvement is most probably hematogenous spread from the primary pulmonary focus in childhood which heals to leave multiple chorio retinal scars, which may be activated in adulthood. The ocular syndrome consists of peri papillary chorio retinal scar, punched out inactive chorio retinal scar, hemorrhagic macular lesion. The left eye is involved more frequently39. In two third cases the condition is bilateral. Histoplasmosis is a major cause of choroidal neovascularisation. Rarely there may be a macular scar. The condition remains asymptomatic untill the macula is involved. Macular involvement is late but symptomatic. The common symptoms are metamorphopsia, diminished central vision, and central scotoma. The patient complains that his vision straight ahead is poor. The treatment of choice is laser photo coagulation of extra foveal and juxta foveal lesion following fluorescein angiography. Sporotrichosis It is a rare fungal disease. The fungus is found all over the world on plants and soil, hence it is but natural that those who work with soil and plants are most likely to get infected. It may manifest at any age. The fungus is found in yeast form and grows as budding yeast on media at 37C. Systemic involvement The commonest route of entry of the organism is via cutaneous wound. In a few days a sub cutaneous lesion develops which most of the time is a self healing lesion. It generally does not spread beyond the limb infected initially, though rarely hematogenous spread to lung and brain are known. The commonest skin lesion is sub cutaneous painless diffuse nodule at the site of injury. Other form are lymphangitis, ulcer or nodule formation. Ocular manifestations are rare but all parts of the eye have been reported to be involved from lid to the retina. Exogenous endophthalmitis occurs following penetrating injury.

Parasitic infection40, 41, 42


The systemic parasitic infection can either be due to protozoa or metazoa. The metazoa infection can be due to helminths that can either be cestodes or nematodes. Other organism that may infect eyes and its adnexa are arthropods or by insect larvae. The commonest protozoa that involves the eye is toxopalsma. The other protozan infections are relatively uncommon and are caused by entamoebahistolytica, plasmodia, giardia, Leishmania, and trypanosoma.

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The common helminths areTaenia echinococcus, taenia solium, causing echinococcosis and cysticersosis respectively. The disease coenurosis is caused by taenia multiceps and taenia glomeratus. The helminth toxocara canis causes toxocariasis. The organism onchocerea volvulus causes river blindness. Thelaziasis is caused by the thelazia callipaeda, and thelazia californiensis. Loasis is caused by worm Loa loa also known as African eye worm. Trichinosis is caused by trichinellaspiralis. Less common ocular manifestations are Dracunculosis caused by dracanculus medinensis. Bilharziasis, sparganosis and ganthostomiasis are still less common in eyes. Rarely the eyes are infested by acariasislumbricoidis and ankylostoma duodenale. The arthropods found on ocular adnexa cause pediculosis, phthiriasis and demodicosis. Infections by insect larvae are called myiasis. Toxoplasmosis42, 43, 44, 45, 46, 47 Toxoplasmosis is caused by protozoa toxoplasma gondii that is found universally. The prevalence of the disease may be as high as 50% in adults who are either asymptomatic or have some systematic or only ocular manifestation. The domestic cat is the definitive host. Other mammals are intermediate host that acquire the disease by eating contaminated food or water by faeces of the cats who excrete the parasite. The organism is obligatory intra cellular. The parasite has two phases: 1. Homologous host phase and 2. Heterologous host phase that is seen in humans. Two forms are met with i.e. the pseudo cystic phase and cystic phase. The disease can either be congenital or acquired. The congenital toxoplasmosis. The foetus is infected via transplacental route from already infected mother who is symptomsless but serologically positive. The mother may have acquired the disease during any phase of pregnancy or has been infected years ago; the disease has been dormant and has been activated during pregnancy. The congenital toxoplasmosis43 is a common cause of abortions and still birth. The two organs commonly involved are the brain and the eyes that produce triad of three Cs i.e. convulsion, calcification (intra cranial) and chorioretinitis. Less frequent features are low birth weight, hepatospleenomegaly, purpuric spots, neonatal hypoxia, raised intra cranial pressure, and hydrocephalus. The ocular lesion in congenital toxoplasmosis is generally bilateral central chorioretinitis that may be mistaken as coloboma of the macula. There is a rough guideline regarding possibility of intra uterine infection i.e. 1. If the mother has been infected six months prior to the onset of pregnancy the chance of infection to the foetus is almost nil. 2. If the mother gets infected less than six months before the conception the foetus may be involved. 3. The chances of infection increases as the duration of infection and conception decreases.

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4. If the mother is infected during first trimester the chances of transplacental spread is least but the disease in neonate is most severe. 5. In contrast to this if the mother is infected in last trimester the chances of foetal infection is most but features in new born are mildest. Acquired toxoplasmosis44 Toxoplasmosis is acquired by ingestion of food or water contaminated by faeces of infected cats. The exact incubation period in not known. The common systemic involvements consist of skin, lymph glands, lungs, CNS, and heart. The organism is known to become opportunistic and have a widespread involvement including eyes. The ocular involvement consists of chorioretinitis that is located posteriorly as necrotising retinitis that has yellowish white cotton wool appearance with indistinct margins and the borders are hyperemic. The lesion may be unicentric or multi-centric and heal by scaring that has central white area with irregular pigmentation all round. Satellite lesion may develop little away from the active lesion during its active phase or after it has healed. The acute stage of acquired toxoplasmosis does not produce any ocular changes. The changes are reactivation of either congenital lesion or a dormant acquired lesion. Anterior segment involvement is less common but is possible. Rarely the extra ocular muscles are involved. Involvement of optic nerve, cataract and glaucoma are common. A child with a lesion in first three months has nystagmus, squint and very low vision. An asymptomatic child may have diminished vision and prone to develop reading difficulty. Entamaeba histolytica Infection by entamaeba histolytica is very common in tropics. It is estimated that one fifth of the population either suffers from this parasitosis or is carrier of the disease who are asymptomatic. Commonest systemic involvement is amaebic dysentery, this is followed by amaebic liver abscess and rarely cerebral abscess. Ocular involvements are very few and rare. They do not have any distinguishing clinical features. There are no specific tests to establish the ocular diagnosis. The diagnosis is presumptive that is supported by improvement following systemic administration of anti amaebic chemotherapy. The commonly reported ocular manifestations are40Anterior and posterior uveitis, that may cause hypopyon, retinal periphlebitis and vitreous haemorrhage.41 Acanthameba is a free living amaeba found world over. It is mostly found in contaminated water. Contamination of water by acanthamoeba has become more frequent in last two decades. It causes granulomatous amaebic encephalitis. The organism reaches the brain via blood stream from sinuses, lungs or skin. Persons with AIDS are at higher risk. Commonest ocular involvement is kerato acanthamoeba. Malaria47, 48 It is an infection caused by protozoa plasmodium. There are four types of plasmodia that are responsible for malaria. They arePlasmodium vivax, P ovale, P malariae and P falciparum. The last variety causes most serious form of malaria and can be fatal.

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The disease is acquired and spread by bite of female anopheles mosquito. Malaria is found in all parts of the world, but is more common in tropics and subtropical countries. The incubation period is different in different species of plasmodium. The systemic features are fever, chill, body ache, abdominal discomfort, all of which can be mistaken as viral infection. Besides fever that may be periodic with definite cycle of fever alternating with afebrile period; there is enlargement of spleen, liver is less enlarged. There may be mild jaundice. There may be rashes all over the body with patechial haemorrhage on the skin and other mucous membrane including conjunctiva. Anaemia is very common. It may cause dysentery like symptom. Cerebral malaria is caused by p falciparum. This is a life threatening condition. Other manifestations are hypoglycemia, lactic acidosis, pulmonary edema and renal failure. Ocular manifestations are rare. They can be divided into two groups: 1. Those caused by malaria. 2. Those caused due to toxicity of anti malarial drug. The common ocular involvements areSub conjunctival patechial haemorrhage, superficial corneal ulcer, simulating dendritic ulcer. However patients with malaria are more prone to develop herpes simplex keratitis both superficial and deep. The deep keratitis is generally attributed to be interstitial keratitis due to malaria, which clears following systemic treatment with anti malarial drugs suggesting that plasmodium is capable of producing interstitial keratitis. Other ocular involvements are retinal haemorrhages and optic neuritis. Commonest ocular feature of anti malarial treatment is quinine amblyopia.49 This is a dose related condition. The effect varies from patient to patient. It consists of sudden loss of bilateral central vision with central scotoma, due to optic neuritis that may end in optic atrophy. Other ocular features of quinine toxicity are retinal pigmentepitheliopathy, damage to bipolar and ganglion cells. Giardiasis Giaradiasis is world wide problem. It is caused by protozoa giardialamblia. It is found in small intestine of many mammals including humans. It causes endemic and sometime epidemic form of enterocollitis and diarrhoea. It is spread by drinking polluted water and ingesting contaminated food. Ocular involvements are rare but can cause uveitis, keratitis, chorioretinitis and retinal haemorrhage all of which respond to anti giardial treatment.40 Leishmaniasis50 The disease is seen only in tropical countries. It is caused by a protozoa belonging to genus leishmania. It is a vector borne zoonosis. Humans are incidental host, small mammals are reservoirs. The disease is spread by bite of sand fly Phlebotomus. There are three common types of leisheniasis in humans they areKala azar, cutaneous leishmaniasis and espundia (nasopharyngeal leishmaniasis).

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Out of these Kala-azar is most commonly seen in endemic areas, can be life threatening. This is caused by L. donovani. Incubation period is long that may range between two to six months following residence in endemic area. The symptoms are long-lasting-fever with enormous enlargement of spleen with moderate enlargement of liver. It is more common in HIV infected persons. It is common in first decade of life. The organism can be transmitted by way of blood transfusion. Ocular manifestations are rare in Kala-azar. The children may have retinal haemorrhage, sub conjunctival haemorrhages. The adults can develop retinal vein thrombosis. Dermal leishmaniasis is associated with ulcers on the lid, episcleral nodules near the limbus causing vascularisation of cornea. There may be iritis. Trypansomiasis (Sleeping sickness)51 This disease is confined to certain parts of Africa and South America. In Africa, it is caused by trypansoma gambiense transmitted by bite of tsetse fly, and in South American, it is caused by trypansoma cruzi transmitted by bite of a bug of genera triatoma. Systemic manifestation of African trypansomiasis - Following bite by the fly the organism spreads via blood and lymphatics to lymphatic glands, CSF and brain. After few weeks the patient develops a skin nodule called trypansome chancre. This is followed by fever that may last for weeks. The organism reaches lymph glands and spleen resulting into their enlargement. The episode of fever has an intermitted pattern. After years the patient reaches a stage of sleeping sickness. The patient develops shuffling gate and finds difficulty to walk. The face becomes mask like with drooping swollen lids. The patient goes to sleep even during working hours. In late stages, there are convulsions, paralysis and coma that terminates in death. Ocular manifestations Unilateral allergic edema of the lids, enlargement of pre auricular and sub mandibular glands, bilateral interstitial keratitis and iridocylitis. In terminal months there may be paralysis of extra ocular muscles and papilledema. The condition is often associated with onchocerciasis. South American trypansomiasis is spread by bite of a bug and not fly, is called Chagas disease. It does not cause somnolence. The cutaneous lesion is called Chagoma, which is a localised area of edema with enlargement of draining lymph node. It is associated with fever that lasts for months. There is hepatospleenomegaly. There may be involvement of heart. Death occurs due to meningo encephalitis. Ocular manifestation is unilateral Chagoma of the lid, which is known as Romanas sign. Taenia echinococcous (Hydatidcyst)52, 53 Taenia echinococcus is smallest of all tape-worms that infest humans. It has world wide distribution. There are three types of echinococi that are pathogenic. Each type has specific geographical distribution. Their life cycle is almost identical with slight dissimilar clinical presentation. The various types are echinococcous granulosus, E multi locularis, E oligoarthus

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and E vogeli. Out of this granulosus causes ocular disease. The cyst of E granulosus is unilocular while that of rest are multi locular. Occasionally multi locular cysts can be seen in the orbit. The dog is the definitive host while many mammals including humans are intermediate hosts. Humans can be primary or intermediate host when they directly ingest food contaminated by dog faeces or secondary intermediate host when they eat contaminated meat which is either uncooked or under cooked of primary intermediate host like sheep, goat, cow etc. Children are infected by swallowing the eggs. Embryos escape from the eggs, penetrate the intestinal mucosa and reach the portal circulation from there they may travel to any distant part and develop the cyst. Common sites where the cysts are deposited are liver, lung, and orbit. Other organs are spleen, kidney and brain. The cyst is generally single, grow very slowly without any discomfort. It becomes symptomatic only when it is large enough to press over the adjacent structures or when it rupture and cause anaphylaxis. A typical echinococcous cyst has two layersAn outer pseudo membrane and an inner germinal layer. The outer layer is surrounded by granulomatous reaction. From the germinal layer develop daughter and grand daughter cysts. The cyst of echinococcus is called hydatid cyst. The cyst is filled with clear fluid. Accidental ruptures of cyst gives rise to severe allergic reaction that may be anaphylactic. Ocular manifestation Only 1% of cases of echinococcosis may involve the eyes and its adnexa. Commonest site being the orbit. Next in frequency is sub retinal space from where it may come to vitreous. Rarely it can come into the anterior chamber. Orbital involvement is common in older children and young adults. It is unilateral, generally the cyst is intraconal causing slow, painless, progressive axial proptosis. The diagnosis is by exclusion and with high index of suspicion. Commonly used investigations are X Ray orbit that may show enlargement of the orbit. A small cyst may not produce any change on X ray. Ultrasonography shows cystic growth without internal reflectivity. CT and MRI give more definite picture. Sub retinal and intra vitrial cysts are best seen by indirect ophthalmoscope and confirmed by CT. Cysticercosis54, 55 Cysticercosis is a common parasitic infection all over the world in all ages. The infection is caused by a cestode, taenia solium which is a pork tape worm. Humans are definitive host while pork is an intermediate host and harbouring larval form. Sometimes humans can become intermediate host by ingesting ova of the cestode. The definitive hosts pass the gravid proglotidis of the adults worm in stool, which is ingested by intermediate host the hog or sometimes dogs and other mammals. In the intestine of the intermediate host the embryos are released which pass the intestinal mucosa and reach the systemic circulation and can be deposited in any organ of the body. Commonest tissues to be infected are the striate muscles. The embryos get entangled in small vessels and are converted into larval stage called cysticircus.

770 Humans get infected by two ways:

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1. By eating under cooked pork that is infested by live cysticircus. 2. By eating food contaminated with ova thus becoming an intermediate host. The ovum ultimately is converted to cysticircus. Systemic involvement consists of cysticircosis of brain called neuro cysticercosis, muscles, skin and eyes. Less commonly involved are kidney, liver and spleen. Intestinal cysticercosis is most of the time asymptomatic but may cause vague pain in abdomen, diarrhoea, weight loss. Patient may notice segments of tapeworm in stool. Neuro cysticercosis is most important and dangerous part of the disease. The clinical manifestation depends on size, location and inflammatory reaction caused by the cyst, which includes - Focal seizures and neurological deficits, generalised convulsion, meningitis, headache, vomiting, and raised intracranial tension. Extra ocular muscle palsy and papilledema are some other features. The presence of intracranial lesion is confirmed by CT and MRI. Ocular manifestation Ocular involvements are common. Almost half of the patients with systemic cysticercosis have ocular involvement. All most all parts of the eye, its adnexa and orbit except the lens may be involved. Involvement of left eye in more frequent than right eye54,55. The cyst may develop in the orbit and migrate anteriorly along the recti muscles and present as sub conjunctival cyst. There may be more than one cyst in the conjunctiva, occasionally the cyst may extrude automatically, may cause proptosis, sub retinal cysts may pass into vitreous, small uveal cysts may pass in AC, involvement of optic nerve is possible. Other ocular involvement is severe intra ocular inflamation following death of the organism inside the eye during anti helmenthic chemotherapy or rupture of the cyst during surgical treatment. Toxocariasis47, 56, 57 Infection by nematode toxocara is called toxocariasis. It is a zoonotic disease that spreads from dog to the children. Now it is established that toxocariasis, of cats do not infect children. Puppies are more infective than adult dogs.58 Children are more prone to develop toxocariasis than adults. The age ranges between two years to fifteen years mean being seven years. Due some ill understood cause the disease is more common in boys. Ocular lesions are more common on the left side. It is more common in children who eat dirt habitually. The puppies get infected via transplacental route, during suckling or direct ingesting food infected by eggs. Children get infected by swallowing soil contaminated by eggs. In children the ingested eggs hatch to form larva that penetrate the gut wall to reach systemic circulation, that carries them to distant organs like liver lung, brain and eyes. The passage of the organism from gut to other organs is called visceral larval migrans, in contrast to cutaneous larval migrans that is caused by hookworm of dogs. Besides visceral larval migrans the parasite can cause abdominal pain, anaemia and eosinophilia that may be as high as 90%. The ocular toxocariasis is less common manifestation than systemic.

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Ocular manifestation consist ofPosterior pole granuloma, that when large gives a white reflex in pupillary area and mistaken as retinoblastoma, peripheral granuloma, parsplanitis, diffuse chorioretinitis, exudative retinal detachment and chronic endophthalmitis. Onchocerciasis59, 60 The disease onchocerciasis is a major cause of preventable and treatable infectious cause of blindness in Africa and South America. The disease is caused by nematode onchocerca volvulus and onchocerea caecutiens. The former is found in central Africa while the latter is found in South America. As the maximum number of cases are found near the rivers, the condition is called river blindness. One of the main complications of the disease is blindness hence the worm is also called blinding worm. Man is the definitive host in whom the micro filaria develops while female black fly-simulium is the intermediate host. It is only the female fly that is responsible for spread of the disease. The adult larva develops in the mouth part of the fly. The disease spreads to infected host to non infected host, multiple infections are known. The systemic involvement is mostly cutaneous, less common is involvement of the lymph nodes. Other systems are not known to be involved. The cutaneous involvement begins with intensive itching along with papular rash. Long term infection leads to hypo-pigmentation of the skin, wrinkled and loose skin. Sometimes there may be hyper pigmentation. Onchocercomata is a subcutaneous nodule, which may be palpable, and visible. In African countries they are seen over the scalp. Mild to moderate lympadenopathy is common generally in the inguinal and femoral area. Hydocele is common. The enlarged nodes may be large enough to hang down. Ocular involvement Almost all parts of the eyes except the lens is known to be infected by onchocerciasis. It includes: 1. Edema of lids that is transient. Some times mild proptosis is seen. 2. Conjunctiva becomes thickened and pigmented though free micro filarae are not seen on the conjunctiva, they are demonstrable in fresh biopsy even without staining. Involvement of the cornea is most important aspect of onchocerciasis and the main cause of blindness. It is present in all most all cases and both the cornae are symmetrically involved. The corneal involvements are: Nummular opacity, presence of dead micro filaria, frosted glass appearance of cornea, superficial vascularisation, deposition of calcium, folds in the Descemets membrane. There may be punctate keratitis or sclerosing keratitis, KPs. Anterior chamber may show number of free floating micro filaria, which do not elicit any reaction. The dead organisms are accumulated at the bottom of AC. Uvea may show iritis, distorted pupil, inverted pear pupil, spongy iris called pumic stone iris. Iridocylitis and chorioretinitis is common.

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Optic neuritis and atrophy are also seen though the lens is not invaded by microfilaria. Complicated cataract is secondary to uveitis. Microfilaria may be seen floating in the vitreous. Thelaziasis60, 61 Thelazia is a small nematode, life cycle of which is not well understood as most of the cases have been reported from South East Asia and far east, it is also called oriental worm. It commonly lives in the lacrimal glands and lacrimal passages of many mammals, common being dogs, and horses. There are two species of thelazia i.e. thelazia calipaeda and thelazia californiensis. Cockroaches are suspected to be intermediate host. It is presumed that the disease is spread by common house fly. Systemic infection has not been reported. It is presumed that the flies deposit the eggs of the nematode in the conjunctiva. The source of these eggs is not well understood. The larvae hatch from the eggs and migrate to the lids and lacrimal sac. The larvae may produce conjunctival nodule. The larvae may penetrate the cornea or the sclera to become intra ocular, causing uveitis. The dead worm like most of the nematodes can cause allergic reaction. The worm may be seen as free floating organism in the AC. Loasis47 The disease loasis is caused by nematode Loa-Loa or African eye worm, Central and western African countries form the endemic area. The microfilaria may live for many years in the subcutaneous tissue of an asymptomatic person who have lived in the endemic area. The life cycle is not well understood. The disease is spread from host to host by bite of the mangrove fly of species chrysops. It is the female fly that bites and spreads the disease. The microfilaria are diurnal, they are seen in the peripheral blood between 12.00 noon and 2.00 noon. The exact incubation period in not known. The disease may be seen concurrently with onchocersiasis. The disease is caused by adult worm that creeps under the skin and produce localised nodule called Calabar swelling. Systemic involvement is rare but may cause nephropathy, encephalopathy and cardiomyopathy. Eosinophilia is constant and the count may go as high as 50-60%. The ocular lesions consist of Calabar swelling of the lids. The adultworm may be seen under the conjunctiva. Trichinosis Trichinosis is a disease caused by larva of nematode trichinella spriralis and trichnatis pseudo spiralis. Besides these two, there are many more types of trichinal nematode that are found in many mammals. The parasite is found all over the world but the disease is mostly confined to Western Hemisphere. The disease is spread by eating under cooked food, infected pork or beef. The larva has predilection for striated muscles. The systemic involvement consists of diarrhoea, abdominal pain, nausea, vomiting. The cutaneous involvement includes migration of larva, allergic reaction, maculo papular rash.

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Myocarditis and tachyarrthymias, and encephalopathy are rare. Muscle involvement is common. Ocular involvements are edema of peri orbital tissue, orbicularis and extra ocular muscles palsy, sub conjunctival haemorrhages, retinal haemorrhages. Dracanculosis Dracanculosis is caused by dracanculus medinensis which is the largest thread worm. The worm is found mostly in tropical countries. A crustacean - cyclope is the intermediate host. The cyclope is found in water. Humans acquire the disease by drinking water contaminated by cyclope. The cyclope contains the larvae of the parasite. The larvae penetrate the wall of the stomach and intestine and mature. The male dies, the female lives for years and migrate under the skin mostly the lower extremities or any part that is frequently soaked in water like back and head of water carriers. The gravid larva migrates under the skin, which is habitually wet and form a blister. The blister ruptures, releasing large number of larvae in stagnant water and ingested by cyclope and the cycle goes on. There are no systemic involvement. The ocular involvement consists of local edema, pain and blister in the lid that may rupture and release larvae. An orbital larva may cause mild proptosis. The worm may form a blister on the skin surface to release the larvae. Sometimes the organism may die in the orbit and get calcified even without any symptom. Ocular myiasis61 Ocular myiasis is caused due to deposition of eggs of some flies that generally breed in the nasal cavity and sinuses of sheep. In persons with poor hygiene and debility of any age may attract the offending flies that lay eggs in the conjunctiva, nose or any open and non-healing wound. There are no systemic involvement. Ocular myasis can either be myiasis externa, which is more common and caused by first stage larvae or myiasis interna, which is rarer and caused by second stage larvae. The ocular myiasis externa consists of conjunctival irritation, itching, edema, photophobia. A sticky white membrane is formed in the fornix and many larvae can be seen under this. Sometimes they may be seen in lacrimal sac or canaliculi. The myiasis externa does not erode the tissue. In myiasis interna, the maggots may burrow in the anterior chamber or the vitreous through sclera leading to sever inflammatory reaction. Management consist of picking up the maggots by forceps after they have been immobilised by xylocaine.

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19. Whitley R.J.Varicella zoster virus infection in Harrisons principles of internal medicine. Fourteenth edition, Vol. I, P.1086-1088, McGraw Hill, New York 1998. 20. Gittinger J.W.Herpes zoster ophthalmicus in Manual of clinical problems in ophthalmology. First edition, p 5-7, Edited by Gittinger J.W. and Asdourian G.K., Little Brown and Co., Boston 1998. 21. Marsh R.J.Varicella and Herpes zoster in Current ocular therapy. Edition fifth, P. 83-84. Edited by Fraunfelder FT and Roy FH. W.B. Saunders Company Philadelphia, 2000. 22. Vastine D.Molluscum contagiosum in Principle and practice of ophthalmology. Vol. I, first Indian Edition, P. 341-342, Edited by Peyman G.A., Sander D.R. and Goldberg M.F. Jay Pee Brothers, New Delhi 1987. 23. Tabbara K.F., Hyndiuk R.A.Infection of the eye. Second edition, Little Brown and Co., Boston 1996. 24. Malouf D.J., Oates R.K.Herpes simplex virus infection in the neonates. J. Paed. Child health, 31: 332-335, 1995. 25. Matoba A.Y.Ocular diseases associated with Epstein-Barr virus infection. Surv. Ophth, 35, 145-150, 1980. 26. Deborah Pavan LangstonEpstein-Barr virus in Manual of ocular diagnosis and therapy. Third edition, P. 195. Edited by Deborah Pavan Langston, Little Brown and Co., 1991. 27. Dutta L.C., Dutta N.K. and Barua C.K.Ocular infections of AIDS in Modern ophthalmology. Vol. II, Second edition, P. 1042-1048, Edited by Dutta L.C., Jay Pee Brothers, New Delhi, 2000. 28. Rao N.A.Acquired immuno deficiency Syndrome and its ocular complication. Ind. Jr. Oph., 42: 51-63, 1994. 29. Birge, H.L.Mycotic infection in Modern ophthalmology. Vol. II, First edition, Edited by Sorsby A., P. 253-271, Butterworth London, 1963. 30. Rezende, C.D.Ocular mycosis. XIX, Con Oph., Vol. 2, P. 299, New Delhi, 1962. 31. Ahmed, E.Mycotic infections in A Text book of ophthalmology. First edition, P. 7273, Oxford University Press, Calcutta, 1993. 32. Bartley G.B.Blastomycosis of the eye lid. Ophthalmology 102 : 2020-2023, 1995. 33. Barr, C.C. and Gamel J.W. : Blastomycosis of eye lid, Arch. Oph., 104: 96-97, 1986. 34. Bennett., J.E.Coccidiodimycosis in Harrisons principles of internal medicine. Fourteenth edition, Vol. I, P 1151-1152, McGraw Hills, New York, 1998. 35. Rodenbiker H.T., Ganley, J.P.Ocular coccidiodimycosis. Surv., Oph., 24: 263-290, 1980. 36. Harrisons A.R., WirtschaflerMucormycosis in Current ocular therapy. Fifth edition, P. 48-50, Edited by Fraunfelder FT and Roy FH, W.B. Saunders Company, Philadelphia 2000. 37. Bennett J.E.Mucormycosis in Harrisons principles of internal medicine. Fourteenth edition, Vol. I, P. 1158, McGraw Hills, New York, 1998.

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38. Harley R.D.Mycotic infections in Pediadric ophthalmology. Vol. II, Edition 2, P. 930-931., Edited by Harley R.D. W.B. Saunders Company Philadelphia 1983. 39. Deborah. Pavan Langston.The fungal disease histoplasmosis in Manual of ocular diagnosis and therapy. Third edition, P. 199-200, Edited by Deborah Pavan Langston, Little Brown 1991. 40. Mukherjee R.Ocular manifestation of tropical disease in Modern ophthalmology, Vol. 2, second edition P. 1037-1041. Edited by Dutta L.C., Jay Pee Brothers, New Delhi, 2000. 41. Bhaduri, BNEye complications in Protozoal parasitic disease of the tropics. XIX, Concillum, ophthalmologium Acta I, P. 79, New Delhi, 1962. 42. Palestine A.G.Toxoplasmosis in Current ocular therapy. Fifth edition, P. 101-102. Edited by Fraunfelder FT and Roy F-H. W.B. Saunders Company, Philadelphia 2000. 43. Hogan M.J.Congenital toxoplasmosis in Modern ophthalmology. Vol. II, First edition, P. 19-22, Edited by Sorsby A. Butterworth London 1963. 44. Hogan M.J.Acquired toxoplasmosis in Modern ophthalmology. Vol. 2, First edition, P. 212-213, Edited by Sorsby A. Butterworth London 1963. 45. Perkins S.Ocular toxoplasmosis. B.J.O., 51: 1, 1973. 46. Rathinam S.R. and Namperumalsamy P.Toxoplasmosis in Clinical practice in ophthalmology. First edition, P. 268-269, Edited by Saxena S. Jay Pee Brothers, New Delhi, 2003. 47. Harley R.D.Ocular parasitosis in Pediatric ophthalmology. Vol. 2, Second edition, P. 931-937. Edited by Harley R.D., W.B. Saunders Company, Philadelphia 1983. 48. Biswas J., Fogla R.Ocular malaria. Ophthalmology. 103:, 1471. 1996. 49. Kanski J.J.Toxic maculopathies in Clinical ophthalmology. Second edition, P. 365, Butterworth London, 1987. 50. Berman J.D.Human Leishmaniasis : Clinical diagnosis and chemo therapeutic developments in last 10 years. Clin. Inf. Dis. 24: 684. 1997. 51. Somerset E.J.Trypansomiasis in Modern ophthalmology. Vol. 2, First edition, P. 292-294, Edited by Sorsby A. Butterworth London, 1963. 52. Sun T. Echinococcosis in Pathology and clinical findings of parasitic disease. P. 287-295, Edited by Sun T., Masson, New York 1982. 53. Dutta L.C.Hydatid cyst in Ophthalmology principles and practice. First Edition, P. 369-370, Current Book International, Calcutta 1995. 54. Malik S.R.K., Gupta A.K., Chaudhri S.K. Am J. Oph. 66: 1168, 1968. 55. Mukherjee P.K., Agrawal S. - Sub conjunctival twin cysticercosis. Ind. Jr. Oph. 23: 2829, 1975. 56. Gille Spel S.H.The Spectrum of ocular toxocariasis. Eye: 7: 415,1993. 57. Shields J.A.Ocular toxocariasis : A review. Surv. Oph. 28: 361-381, 1984.

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58. Liv. L.X., Weller P.F.Visceral and ocular larval migrans in Harrisons Priciple and practice of internal medicine. Fourteenth edition, Vol. I, P. 1206-1207, McGraw Hill, New York 1997. 59. World health organisationOnchocerciasis and its control. Report of a WHO expert committee on onchocerciasis control. WHO Tech Report Series 852, 1995. 60. Mukherjee P.K., Verma S., Agrawal S.Intra ocular thelazia. A case report. Ind. Jr. oph. 25: 41-42, 1978. 61. Mukherjee P.Thelaziasis in Current ocular therapy. Fifth edition, P. 100-101, Edited by Fraunfelder FT, Roy F.H. W.B. Saunders Company, Philadelphia 2000. 62. Ridley H.Ocular myiasis or ophthalmo myasis in Modern ophthalmology. Vol. 2, first edition, P. 251-252, Butterworth London 1963.

Index
A
A and V pattern, 615, 621, 667 A-V pattern, 621 A-V syndrome, 669 A.D.D., 350 A.M.R.D., 382 Abducens nerve, 604 Abducent nerve, 511, 597, 600, 601, 604, 677 Abduction, 595, 597, 599, 618 Abductors, 593, 597, 598 Aberrant lacrimal gland, 94, 95 Aberrant regeneration of third nerve, 509, 606, 608 Abiotrophic, 434, 576 Ablate, 211 Ablepharon, 41 Abnormal colour vision, 441 Abnormal fluorescence, 415 Abnormal head posture, 587 Abnormal retinal correspondence, 569, 639 Abnormal tear film status, 173, 374, 682 Abnormality of light reflex, 506 Abnormality of the orbit, 644 Abortion, 740, 765 Abrahams lens, 382, 383 Abrasion, 23 Abscess of lids, 53, 92 Absence of lacrimal gland, 104 Absolute glaucoma, 116, 721 Absolute hypermetropic, 305, 556 AC depth, 417, 701 AC IOL, 357 AC/A, 653 Ac/A ratio, 667 Acanthameba, 766 Acanthamebial drugs, 209 Acanthamoebae keratitis, 208 Acanthocytosis, 440 Acariasislumbricoidis, 765 Accessory elevator, 7, 67 Accessory glands, 88 Accessory glands of Krause and Wolfring, 103 Accessory Lacrimal glands, 7, 86, 87, 98, 112, 113 Accommodation, 15, 70, 292, 502, 503, 546, 548, 557, 586, 601, 608, 668 Accommodation and convergence insufficiency, 570, 571 Accommodation in hypermetropia, 556 Accommodative esotropia, 22, 654, 658, 661 Accommodative esotropia with high Ac/A, 660 Accommodative squint, 558, 656 Acetazolamide, 148, 184, 193, 255, 351, 370, 377, 555 Acetic acid, 143 Acetyl choline, 75 Acetyl cysteine, 143 Acetylcholine, 502, 505 Achromatopsia, 17, 419, 441, 443, 538, 578, 579, 628, 632, 634 Acid alkali burn, 221 Acid mucopoly saccharides, 443, 448, 477 Acid-fast, 731 Acidosis, 377 Acinetobacter, 129, 726 Acnerosacea, 142 Acoustic neuroma, 55, 56, 620 Acquired, 73, 121, 229, 258, 259, 611 Acquired anisometropia, 567 Acquired esotropia, 658 Acquired immuno deficiency syndrome (AIDS), 267, 749 Acquired ptosis, 68 Acquired syphilitic retinitis, 430 Acquired unilateral nystagmus, 628, 630 Acrocephalo syndactylia, 681 Acronym, 381 Acrylic, 562 Actinic rays, 248 Actinomycosis, 126, 482, 708, 726, 759 Action of acetazolamide, 377 Active immunisation, 202 Active uveitis, 188 Actively motile, 743 Acuta glaucoma, 116 Acute allergic conjunctivitis, 24, 91, 139 Acute dacryocystites, 92, 786 Acute disseminated encephalomyelitis, 527, 529 Acute esotropia, 663 Acute hydrops, 169 Acute iridocyclitis, 206, 271, 576 Acute iritis, 262 Acute peri orbititis, 702 Acute retinal necrosis, 267, 432 Acute rheumatic fever, 739 Acute rise of intraocular pressure, 525 Acute serpiginous ulcer, 191 Acute sinusitis, 529, 702 Acyclovir, 197, 198, 199, 202 Adaptation, 639 Addisons disease, 117, 762 Adduction, 72, 595, 596, 597, 598, 605 Adductors, 597 Adeno virus, 131, 203, 758 Adenoma sebaceum, 59, 87 Adenosine triphosphate, 160, 197 Adherent leucoma, 166, 184, 186, 188 Adies pupil, 505, 511 Adrenal gland, 530 Adrenaline, 117, 512, 708 Adrenergic, 374, 505 Adult, 2, 238 Advanced glaucoma, 584 Advantage of paracentesis, 184 Advantages of binocular single vision are, 638 Advantages of direct gonioscopy, 346 Advantages of NdYAG, 383 Aerobic, 726, 727, 731, 738 Aerobic metabolism, 293 Afferent pupil, 715 Afferent pupillary defect, 510, 517, 520, 523, 530, 533, 536, 540, 541, 517 Afferent visual, 510 Afocal lens, 413 African eye worm, 765 After cataract, 323 After image test, 640, 653 Aganglionic megalocon, 683 Age related macular degeneration, 416 AgNO3, 128, 757

778

INDEX
Agnosia, 574, 581 Agonist, 594 Agranulocytosis, 760 Agraphia, 580 AIDS, 174, 253, 430, 749, 754, 756, 759, 760, 761, 762, 766 Air corneal interface, 342 Air puff non contact tonometer, 341 Alacrima, 94, 95 Albendazol, 147, 153, 490 Albinism, 16, 17, 18, 27, 227, 399, 400, 410, 415, 423, 576, 632, 656, 683 Albinism associated with systemic syndromes, 401 Albinos, 395 Albinotic fundus, 237 Albipuntatus, 439, 574 Albumin, 103 Albunoid, 293 Alcaine blue, 105 Alchoholic solution of iodine, 181, 199 Alexanders law, 629 Alexia, 580 Alkali burn, 214 Alkaline, 141 Alkaloid, 373 Allergen, 140, 144 Allergic blepharo conjunctivitis, 103, 116, 374 Allergic chemosis, 118 Allergic conjunctivitis both endogenous, 23, 97, 103, 122, 142, 147 Allergic conjunctivitis in children, 138 Allergic dermatitis, 180 Allergy, 13, 205 Alopecia, 274, 275 Alpha agonist, 371, 372 Alpha agonist, 252, 255, 351, 355, 356, 360, 361, 362, 365, 368, 369, 371, 372 Alports syndrome, 315 Altered aqueous dynamics in chronic uveitis, 251 Alternate, 649 Alternate cover test, 639, 646 Alternate day esotropia, 620, 623 Alternate hyper phoria, 614, 615 Alternate occlusion, 656 Alternate squint, 650 Alternate sursum-duction, 614 Aluelar, 707 Amacrine cells, 393, 396, 399 Amaebic dysentery, 766 Amaebic liver abscess, 766 Amaeboid ulcer, 141, 195, 755 Amaurosis, 442, 507, 520, 574 Ambloyopia screening, 30 Amblyopia, 8, 28, 39, 41, 56, 68, 70, 79, 147, 168, 172, 178, 185, 186, 189, 236, 239, 246, 248, 260, 270, 297, 303, 315, 319, 323, 324, 327, 351, 371, 402, 404, 432, 444, 488, 513, 517, 549, 574, 585, 586, 587, 590 609, 615, 617, 618, 621, 623, 625, 639, 653, 656, 659, 662, 664, 665, 666, 668, 670, 681, 682, 710, 712 Amelanotic melanoma, 57 Amenestic colour aphasia, 580 Ametropic amblyopia, 546, 586 Amikacin, 730, 743 Aminoaciduria, 315 Aminocaproic acid, 364 Aminoglycocides, 184, 726, 740, 743 Amoeboid, 195 Amoxycillin, 728, 743, 744 Amphotericin, 205 Ampicillin, 126, 740, 742, 743, 744 Amplitude of accommodation, 509, 628, 629 Ampula, 231 Amsler grid, 577 Anaemia, 422, 425, 492, 712, 767 Anaerobic, 293, 726, 727, 729 Anaesthesia dolorosa, 171, 202, 607, 753 Anaesthetic agent, 173 Anaesthetic cornea, 177 Analog of guanosine, 197 Anastomosis of retinal vessels, 428 Anatomy of cornea, 159 Anatomy of the nerves involved in ocular movement, 601 Anatomy of the structures related to glaucoma, 334 Anatomy of the vitreous, 456 Andriamycin, 708 Anemia thrombocytopenia, 740 Aneurysm, 409, 415, 608, 612, 619 Aneurysm of intra cavernous carotid, 607 Angel kappa, 652 Angio matosis retinae, 55 Angio neurotic oedema, 117 Angioid streak, 314, 415, 435, 723 Angioma, 283, 409 Angiomatosis retina, 57, 406, 415, 422, 424, 426, 427, 428, 454, 462, 477, 489 Angioneurotic edema, 44, 760 Angiopathy, 537 Angle closure glaucoma, 97, 162, 251, 357, 370 Angle kappa, 642, 652, 666 Angle of anomaly, 640, 653

779
Angle of anterior chamber, 335, 346 Angle of squint, 587, 651 Angle of the anterior chamber, 335, 336 Angle recess, 342 Angular artery, 89 Angular conjunctivitis, 140, 735 Angular intra orbital, 89 Angular veins, 89, 336 Anhydrosis, 74 Animal dander, 140 Aniridia, 12, 17, 19, 163, 234, 306, 310, 314, 352, 401, 517, 549, 560, 576 Aniseikonia, 551, 561, 562, 563, 567, 569 Anisocoria, 19, 229, 234, 502 Anisometropia, 60, 70, 271, 559, 561, 570, 585, 587, 665, 668 Anisometropic amblyopia, 586, 590, 663 Ankyloblepharon, 7, 18, 39, 121 Ankyloblepharon filiformis adnatum, 39 Ankyloplepharon, 39 Ankylosing spondylitis, 240, 248, 271 Ankylostoma duodenale, 765 Annular posterior synechia, 244, 250 Annular scotoma, 582 Annulus of zinn, 511, 677 Anomalies, 18, 234 Anomalies of structures arising from outer layer o, 400, 402 Anomalous retinal correspondence, 639, 659 Anomalous trichromatism, 578 Anopheles mosquito, 767 Anophthalmos, 8, 12, 16, 18, 68, 94, 678, 696 Anopia, 578 Anorexia, 747 Antagonist muscles, 594 Ante cubital vein, 414 Anterior axial cataract, 227, 256, 298 Anterior capsular cataract, 244, 295, 296 Anterior capsular opacities, 244, 250, 323 Anterior cavernous sinus syndrome, 607 Anterior cerebral artery, 512 Anterior chamber, 3, 5, 15, 19, 153, 154, 162, 163, 173, 175, 177, 184, 243, 302, 335, 337, 451, 501, 563, Anterior chiasma syndrome, 508 Anterior choroiditis, 206 Anterior ciliary arteries, 114, 116, 232, 277, 596, 597

780
Anterior ciliary veins, 336 Anterior ciliary vessels, 232, 242, 720 Anterior cranial fossa, 673 Anterior dislocation, 306 Anterior embryotoxon, 162 Anterior inferior cerebellar artery, 604, 619 Anterior junctional lesion, 508 Anterior lacrimal crest, 89, 674 Anterior lens capsule, 339 Anterior lenticonous, 302 Anterior limbal border, 112, 336 Anterior limbal zone, 336 Anterior limiting layer, 228 Anterior lip of the optic cup, 233 Anterior medullary velum, 603, 604, 612 Anterior membrane, 210 Anterior mesoderm, 3 Anterior microphthalmia, 162 Anterior plus, 486 Anterior polar cataract, 127, 163, 175, 188, 189, 291, 295, 296, 734 Anterior scleral foramen, 159, 720 Anterior segment ischaemia, 215, 249 Anterior segment ischeamia and uveitis, 240, 277 Anterior staphyloma, 62, 169, 174, 175, 186, 751 Anterior stromal puncture, 211 Anterior synechea, 57 Anterior uveitis, 24, 116, 174, 180, 181, 196, 239, 201, 242, 268, 269, 271, 274, 348, 361, 376, 741, 755, 759 Anterior vasculosa lentis, 293 Anterior visual pathway, 272, 278, 507, 532, 462 Anterior vitrectomy, 274, 323 Anthrax bacillus, 726 Anti histamine, 328 Anti inflammatory agent, 255 Anti metabolites, 257 Anti metropia, 568 Anti microbial drugs, 179 Anti mongoloid obliquity, 41 Anti mongoloid slant, 670, 681 Anti retinal antibodies, 273 Anti syphilitic treatment, 207 Anti toxin, 730 Anti toxoplasma chemotherapy, 432 Anti toxoplasmo anti bodies, 431 Anti tubercular treatment, 733 Anti vascular chemical factors, 169 Anti viral drugs, 131, 196 Anti-toxoplasmosis chemotherapy, 260 Antibacterial vaccines, 147, 528 Antibodies, 268 Antibody to anti actycholine, 77 Anticholine esterase drugs, 75, 77, 505 Anticholinergic, 103 Antigen antibody reaction, 367 Antiglaucoma filtering surgery, 357 Antihistamine, 103, 139, 140, 142, 362 Antimongoloid slant, 683 Antinuclear, 268 Antioxidants, 198 Antisepsis, 110 Antitoxin, 125, 728 Antitoxoplasma antibody, 260 Antiviral drugs, 49 Antiviral ointment, 52 Antiviral vaccine, 528 Aortic insufficiency, 308 Aorticroot, 308 Aperts syndrome, 678, 681, 695 Aperture, 382 Apex of the orbit, 511, 596, 673 Aphakia, 28, 270, 321, 335, 354, 412, 452, 547, 556, 559, 563, 665, 668 Aphakic correction, 8, 304 Aphthous ulcer, 277 Apical pain, 763 Aplasia, 621 Aplastic anaemia, 119 Apocrine sweat glands, 38 Aponeurosis of levator, 36, 87, 674 Apoptosis, 434 Applanation tonometry, 176, 341 Applied anatomy of the retina, 393 Apraclonidine, 372, 375 Aqueduct stenosis, 509, 521, 603 Aqueous chamber, 98, 160, 216, 230, 290, 335, 501 Aqueous deficiency, 103 Aqueous drainage device, 350, 355, 356 Aqueous flare, 241, 243, 263, 283 Aqueous formation, 375 Aqueous humour, 26, 340 Aqueous layer, 102 Aqueous misdirection glaucoma, 357, 358 Aqueous penicillin G. solution, 130 Aqueous production, 375 Arachnodyctalous, 308, 310 Arachnoid villi, 5, 511, 513, 521 ARC, 639, 653 Arcuate or nerve fibre defect, 514, 518 Arcuate scotoma, 516 Arcus juvenilis, 190, 338

PEDIATRIC OPHTHALMOLOGY
Arden ratio, 420 Area of non perfusion, 407 Argemone mexicana, 369 Argon gas, 61, 382, 383, 385, 487 Argon laser, 58, 383, 408, 493, Argyll robertson pupil, 507, 509 Argyrosis, 117 ARMD, 415 Arrhythmia, 376 Arterial arcades, 38 Arterial phase, 414 Arterio sclerosis, 422 Arterio venous anastomosis, 415, 466, 713 Arterio venous aneurysm, 405, 409 Arterio venous shunt, 58 Arterio-sclerosis, 119 Arteriography, 693 Arteriovenous malformation of retina and brain, 55 Arteritis, 526 Arthophoria, 569 Arthopods, 725, 742 Arthritic uveitis, 268 Arthritis with bowel disease, 268, 271, 742, 744 Arthropods, 46, 152, 154, 764, 765 Artificial drainage device, 350, 700 Artificial eye, 144 Artificial tear, 211 Ascariasis, 144 Ascending limb, 534, 700 Ascending optic atrophy, 532 Ascorbic acid, 294, 457 Aseptic, 218 Aseptic and necrosis of cornea, 216 Aseptic meningitis, 745, 746 Ash-leaf area, 59 Aspergillosis, 759, 760 Aspergillus, 203, 279 Aspirin, 142, 257 Associated buphthalmos, 343 Associated causes of congenital glaucoma, 352 Associated congenital glaucoma, 56 Associated glaucoma, 57 Asteroid hyalitis, 438 Asteroid hyalopathy, 445 Asthenopia, 32, 47, 48, 60, 70, 143, 152, 178, 302, 303, 515, 546, 547, 549, 550, 557, 558, 564, 565, 566, 567, 570, 588, 647, 648, 667, 678, 681, 682, 685, 712 Astringent, 139, 140, 328, 362 Astringents vasoconstrictor, 142 Astrocytoma, 491, 534, 539, 710, Astroid hyalitis, 461

INDEX
Astronomical telescope, 413 Asymmetry of face, 22, 611, 614 Asymmetry of orbit, 612 Asymmetry of the cup, 371 Ataxia talengectasia, 55, 138, 439, 605, 752 Atopic cataract, 143 Atopic conjunctivitis, 120 Atopic dermatitis, 44, 141, 163 Atopic kerato conjunctivitis, 139, 140, 170 Atovaquone, 261, 432 Atrasia of the inferior osteum, 90 Atreosclerotic retinopathy, 434 Atresia of puncta and canaliculus, 90, 92 Atrophic bulbae, 163, 166 Atrophic eye, 68 Atrophic holes, 449 Atrophic maculopathy, 436 Atrophic RPE, 415 Atrophy of choroid, 238 Atrophy of macula, 443 Atrophy of optic nerve, 530 Atrophy of the lid, 59 Atropine drops, 20, 77, 129, 130, 147, 149, 192, 193, 208, 220, 247, 254, 255, 506 Atropine induced amblyopia, 270 Atropine sulphate, 180, 182, 187, 198, 205, 209, 358 Attenuation of blood vessels, 436 Atypical form of retinitis pigmentosa, 233, 437 Atypical mycobacteria, 750 Atypical pigmentary dystrophy, 439 Auricular lymph, 125 Auto florescence, 518 Auto immunity, 75, 82, 138, 139, 147, 205, 240, 273, 276, 607, 714, Auto refraction, 566 Autoclaving, 727 Autoimmune disease, 13 Autoimmune disorder, 75 Autonomic nervous system, 56, 505, 510 Autosomal dominant, 55, 70, 163, 210, 212 Autosomal recessive, 212 AV phenomenon, 617, 653 Avascularity of cornea, 169 Avulsion of optic nerve, 535 Axenfeld anomaly, 338 Axenfelds, 339 Axial basal view, 296, 694 Axial hypermetropia, 8, 556 Axial length, 14, 546 Axial length of eyeball, 701 Axial myopia, 549 Axial neuritis, 526 Axial proptosis, 676, 769 Axialcortex, 291 Axis of the lens, 291 Axons, 397, 532 Axoplasmic flow, 424, 521, 524 Azathioprine, 78, 258 Azithromycin, 137, 432

781
Batten-Mayou disease, 442 Battles sign, 620 BCG does, 147, 198, 732 Beaten bronze appearance, 444 Becitricin, 130 Bedsonia, 133 Behcets disease, 240, 253, 277 Behcets disease, 253 Behcets syndrome, 259 Behrs optic atrophy, 535 Behrs recessive neuropathy, 537 Bells palsy, 81, 82, 83, 742 Bells phenomenon, 14, 61, 71, 79, 81, 82, 597, 609, 610, 621 Bells protective reflex, 20 Benedicts syndrome, 73 Benign glioma, 710 Benign tumour, 478 Benzene hexachloride, 46 Bergemeister papilla, 518 Bergers space, 457 Bergmeisters papillae, 6, 399, 488, 459, 517, 519, 524, Berlins oedema, 326 Bests disease, 444 Beta adrenergic agonists, 372 Beta blockers, 130, 151, 183, 184, 193, 205, 252, 255, 351, 355, 356, 360, 361, 362, 364, 365, 367, 368, 369, 370, 371, 372, 375 Betamethasone, 143, 256, 713 Betaxolol, 376 Bi-medial recession, 662 Bi-oblique astigmatism, 564 Biber-Haab, 211 Biconvex, 323 Bicycle-wheel pattern, 465 Bielschowsky, 611, 616 Bielschowsky phenomenon, 615 Bifoveal fixation, 270, 594, 660 Bifoveal fusion, 666 Big hypopyon, 191 Biguanides, 209 Bilateral amblyopia, 581 Bilateral exophthalmos, 81 Bilateral fourth nerve palsy, 603, 611 Bilateral irideplegia, 28 Bilateral ptosis, 624 Bilateral retinoblastoma, 690 Bilharziasis, 765 Bimatoprost, 372, 376 Binary fission, 133 Binasal hemianopias, 583 Binocular convergence test, 640 Binocular diplopia, 637 Binocular fusion, 638, 653

B
B cereus, 281 B scan ultra sonography, 412, 417, 424, 484, 700 B. lactamase, 733 Bacillus cereus, 258, 726 Bacitracin, 179, 192, 736 Baclofen, 635 Bacteria, fungi, 177 Bacterial conjunctivitis, 174 Bacterial endocarditis, 425 Bacterial exogenous endophthalmitis, 279 Bacterial keratitis, 208 Bacterial retinitis, 430 Bacterial ulcer, 183 Bacteriostatic, 102 Bagolini filter, 615 Bagolini glasses, 640 Bagolinies striated glass, 611, 653, 663 Bagolinis glasses, 641 Baheets disease, 258 Ball and socket pupillary block, 311 Band keratopathy, 166, 168, 172, 188, 190, 214, 248, 269, 270, 271, 274 Bandage lenses, 106, 181, 182, 184, 210, 211 Bar reading, 648 Barbiturates, 148, 347, 506 Barkans membrane, 339, 347 Barrel type, 570 Basal cell carcinoma, 54, 59 Basal meningitis, 82 Basal tearing, 105 Base down prism, 503, 615 Basic and reflex tear, 95 Basic exotropia, 667 Basic or resting tear, 102, 658, 662 Basic type non accommodative acquired esotropias, 662 Basilar artery, 602, 606 Basilar artery aneurysm, 607 Basilar part, 602 Bassen-Kornzweig syndrome (abetalipo proteinemia), 440

782
Binocular indirect ophthalmoscope, 28, 413 Binocular vision, 324, 569, 594, 595, 637 Bio microscope, 105 Biological glue, 448 Biomicroscopy of the posterior segment (Slit lamp, 413 Bioprosthesis, 737 Biphasic, 418 Bipolar cell, 393, 394, 395, 396, 399, 418 Birth injury, 171 Birth trauma, 16, 168, 216 Bitemporal hemianopia, 583 Bitotspot, 24, 105, 218 Bjerrums scotoma, 582 Black ball, 363 Black eye, 44, 709 Black fly-simulium, 771 Black spots, 411, 551 Blast injury, 173, 187, 324 Blastomycosis, 759, 760 Bleb failure, 385 Blepharitis, 45, 50, 104, 143, 173, 194, 198, 557, 565, 571, 726, 735, 738, 739, 762 Blepharo conjunctivitis, 142, 170, 173, 174, 198 Blepharo phimosis, 18, 22, 39, 40, 41, 42, 67, 71, 79 Blepharo spasm, 50, 67, 123, 147, 175, 206, 347, 728 Blepharochalasis, 39, 41 Blind spot, 524, 531, 582, 680 Blinding trachoma, 135 Blinding worm, 771 Blindness, 407 Blink mechanism, 14 Blink reflex, 321 Blocked intubatinon, 101 Blood agar, 177, 204, 209 Blood aqueous barrier, 229, 230, 373 Blood brain barrier, 375 Blood dyscrasia, 44, 119, 463 Blood pH, 487 Blood pyruvate, 369 Blood retinal barrier, 231, 395, 398, 423 Blood staining of cornea, 189, 363 Blood supply of the optic nerve, 512 Blood supply of the retina, 397 Blood supply of uvea, 231 Blood tinged hypopyon, 52 Blood vessels, 233 Blow out fracture, 609, 674 Blue dot cataract, 296, 298 Blue excitation filter, 414 Blue sclera, 163, 314, 722 Blue sclera brittle bone syndrome, 26 Blue-yellow defect, 580 Bluesclera, 12 Bluish green hypopyon, 737 Blunt injury, 324, 423, 560 Blunt trauma, 427 Blurring of disc margin, 523 Blurring of vision, 273 Board like rigidity, 125 Body, 89 Boeck candy bead test, 30 Boil, 53 Bollus IV mannitol, 380 Bone spicule, 154, 437, 466, 679 Bony nasolacrimal duct, 93 Bony orbit, 10 Borrelia, 740 Borreliaburgdorferi, 742 Botox, 635, 657 Botulin toxin A, 506, 635, 657 Bound fluorescein, 414 Bournvilles disease, 55, 58 Bow tie pallor, 532 Bowmans membrane, 3, 112, 135, 146, 159, 160, 161, 170, 185, 186, 187, 188, 210, 214, 217, 249, 336, 338 Brachial plexes, 71, 73, 512 Bradycardia, 312, 376,439, 680, 744 Brain stem, 56, 96, 259, 479, 510, 522, 674, 677, 703, 735, 743, 765 Brain stem glioma, 81 Brain stem lesion, 619 Brain stem trauma, 81 Branchial arch syndromes, 682 Branhamella, 735 Break up time, 217 Bridge coloboma, 234, 236, 515 Brightness, 578 Brimonidine, 372, 373, 375 Brinzolamide, 377 Broad based iridectomy, 379 Broad posterior synechia, 263 Bronchitis, 744 Brown rings, 329 Browns syndrome, 609, 613, 616 Brucellosis, 253, 727 Bruchs membrane, 4, 9, 231, 232, 233, 314, 395, 414, 435, 445, 553 Bulbar, 111, 141 Bulbar conjunctiva is rarely, 56, 110, 144, 150, 721 Bulbar conjunctiva, 111 Bulbar muscles, 76 Bulbus keratopathy, 166 Bulls eye appearance, 168, 441, 444

PEDIATRIC OPHTHALMOLOGY
Buphthalmos, 18, 23, 24, 25, 26, 28, 62, 97, 166, 167, 171, 172, 190, 207, 216, 284, 314, 321, 335, 373, 375, 552, 560, 576, 678, 690, 724 Burkitts lymphoma, 687, 688, 708, 709, 750, 759 Burkitts sarcoma, 708 Burst chalazia, 49, 121, 149, 150 Busaccas nodule, 249, 250 BUT, 105 Button hole iridectomy, 379 By Schiotzs tonometer, 371

C
C pneumoniae, 757 C psittaci, 757 C trachomatis, 757 C. diptheria, 160 C. trachomatis, 758 C.A.I, 372 Cafaulait spot, 55, 539, 710 Calabar swelling, 772 Calcarine fissure, 583 Calcarious opacity, 249 Calcification, 57, 260, 478, 765 Calcium carbonate, 248, 478 Calcium metabolism, 297 Calcium phosphate, 248 Calcium soaps, 461 Calculation of duration of occlusion, 589 Caldwell view, 693 Caleoflour white, 67, 204 Calliper, 25 CAM vision stimulator, 590 Canal of Schlemm, 336, 339, 341, 350 Canalicular block, 93 Canaliculi, 7, 86, 88, 92, 98, 726, 760 Candida albicans, 144, 149, 279, 750, 761 Candida retinitis, 432 Candidiasis, 126, 246, 759, 761 Candle wax lesion, 276 Canopener capsulorrhexis, 323 Canthal ligaments, 36 Cantholysis, 715 Canthomental line, 694 Capillary drop out, 407 Capillary hemangioma, 60, 491, 685, 688, 690, 692, 698, 707, 712 Capillary non perfusion, 409 Capsulated diplococcus, 735 Capsule contraction syndrome, 323 Capsule of the lens, 291 Capsulo cortical, 295 Capsulo hyaloid adhesion, 291 Capsulo lenticular cataracts, 295, 296

INDEX
Capsulopalpebral fascia, 676 Capsulopalpebral ligament, 674 Capsulorrhexis. Continuous curvilinear capsulorrhe, 323 Capsulotomy, 382 Carbachol, 505 Carbamazepine, 635 Carbolic acid, 50, 198, 199, 756 Carbon dioxide laser, 61, 382, 383, 384 Carbonic anhydrase, 183, 457 Carbonic anhydrase inhibitors, 252, 270, 355, 358, 360, 361, 364, 368, 371, 372, 376 Carboplatin, 482 Carbuncle, 738 Cardiac arrhythmia, 374 Cardiff, 30 Cardinal position, 594 Cardio selective, 372 Cardio selective betablockers, 375, 376 Carotene, 217 Carotico cavernous fistula, 691, 697 Carotinoid, 87, 394 Carpal tunnel syndrome, 312 Cartelol, 375 Cartilage, 284, 679 Caruncle, 35, 87, 89, 112, 113 Cataract in Lowes syndrome, 13, 20, 52, 162, 260, 270, 277, 306, 330, 429, 549, 590, 665, 668, 708 Cataract in siderosis, 329 Cataract secondary glaucoma, 269 Cataract secondary to ocular inflammation, 330 Cataracta central pulverulenta, 296 Cataractogenesis, 297 Catarrhal conjunctivitis, 50, 123, 132, 147, 205, 748 Catford drum test, 16, 29, 632 Catis, 240 Causes of aphakia in children, 559 Causes of asthenopia include, 570 Causes of axial proptosis, 691 Causes of bilateral proptosis in children are, 690 Cause of congenital cataracts can be, 315 Causes of conjunctival chemosis are, 117 Causes of cornal opacity, 188 Causes of corneal opacities in children, 168 Causes of deep vascularisation, 171 Causes of diminished vision in retinal detachm, 450 Causes of diminished vision in chronic posterior u, 242, 247 Causes of RAPD, 508 Cauterise, 181, 183 Cavernous haemangioma of retina, 54, 55, 60, 61, 491, 493 Cavernous optic atrophy, 532, 535 Cavernous sinus, 73, 398, 502, 503, 512, 602, 604, 606, 612, 699, 704 CD ratio, 371 CD4 lymphocytes, 267 CD4 plus, 430 CD4+ lymphocytes, 749 Cefazolin, 129, 130, 184, 192, 192, 193, 728 Cefotoxamime, 728 Cell mediated type four allergic reaction, 144 Cellophane maculopathy, 436 Cells, 272, 393 Cells in AC, 283 Cells in the vitreous, 283, 528 Cells in vitreous, 283 Celluflour white, 209 Cellular debris, 138, 360 Cellulitis lid, 44, 50, 51, 53, 97, 118, 703, 739, 763 Cellulose derivative, 106 Cematidine, 198 Central artery occlusion, 703 Central artery of the retina, 458 Central bitemporal hemianopia, 583 Central cataract, 747 Central choroiditis, 239, 318 Central corneal defect, 339 Central corneal opacity, 296 Central corneal ulcer, 127, 178, 190 Central gaze palsy, 76 Central leucoma, 127 Central nervous involvement, 59 Central retinal artery occlusion, 684 Central retinal vein, 398, 511, 521, 704 Central retinal vessels, 479, 510 Central retinitis pigmentosa (Inverse retinitis pi, 438 Central serous chorio retinopathy, 416, 423, 527, 767 Central serous retinopathy, 415, 423, 516 Central space, 675 Central ulcer, 130 Central vein stasis, 684 Central vein thrombosis, 251, 414, 703 Central viral corneal ulcers, 193 Central vision, 396 Centre of budge, 73

783
Centrocecal scotoma, 520, 527, 531, 582, 641 Cephalic, 728 Cephalosporin, 726, 730, 739, 743 Cephalosporins, 203, 736, 744 Cephazoline, 179, 180 Cerbropontine angle tumour, 172 Cerebellar ataxia, 59, 606, 746 Cerebellum, 521 Cerebral abscess, 59, 766 Cerebral malaria, 767 Cerebral palsy, 656 Cerebral peduncle, 602 Cerebro spinal fluid, 479, 511 Cerebropontine angle lesions, 82 Cerebrovascular accidents, 310 Cerebrum, 530 Cervical sympathetic nerve, 37, 67, 71, 72, 228, 502 Cervical vertebra, 683 Cervicitis, 133, 733, 757 Cestodes, 764, 769 Chaffing of iris, 357 Chalazion, 48, 557, 565, 571, 647 Chamber, 382 Chandler syndrome, 337 Changes in corneal thickness, 167 Characteristics of abducent nerve palsy, 618 Characteristics of afferent pupillary defects, 507 Characteristics of amblyopic eyes, 585 Characteristics of an exudative retinal detachment, 455 Characteristics of bacterial corneal, 177 Characteristics of concomitant squint, 649 Characteristics of fourth nerve palsy, 610 Characteristics of fully accommodative esotropia, 659 Characteristics of fungal ulcer, 177 Characteristics of infantile esotropia, 655 Characteristics of oculomotor nerve palsy, 605 Characteristics of sterile corneal ulcer, 178 Characteristics of viral ulcer, 177 Chediak-Higashi syndrome, 402 Chelating agent, 215 Chemical burn, 169, 171, 173 Chemical cautery, 198, 199, 205 Chemical conjunctivitis, 46, 120, 128, 757 Chemical injuries as cause of glaucoma, 214, 324, 368

784
Chemo prophylaxis, 136 Chemosis of conjunctiva, 117, 132, 139, 153, 479 Chemotherapy, 707, 708, 709, 759 Chequer board, 421 Cherry red spots, 425, 435, 442 Chiasma glioma, 56, 401, 510, 535, 541, 711 Chiasmatic hemianopia, 508 Chicken blindness, 219, 576 Chicken pox, 14, 49, 50, 93, 123, 132, 199, 745, 751 Chief causes of central corneal ulcers are, 191 Childhood glaucoma, 343 Chlamydia, 12, 23, 133, 135 Chlamydia kerato conjunctivitis, 170 Chlamydia trachomatous, 127 Chlamydiasis, 756 Chlorambucil, 258 Chloramphenicol, 137, 179, 193, 526, 529, 728,736, 739, 744 Chloramphenicol ophthalmic drops, 100 Chloride, 103 Chloroquine, 440, 526, 529 Chloroquine maculopathy, 438 Chlortetracyclin, 137 Chlorumbucil, 706 Chocolate agar medium, 130, 209 Chocolate cyst, 712 Cholecystitis, 744 Cholesterol crystals, 407, 461, 488 Choline esterase, 657 Cholinergic antagonists, 505, 506 Chorio capillaries, 394, 397, 414 Chorio retinal atrophy, 446 Chorio retinal scar, 415, 429, 448, 457 Chorio retinitis, 421, 423, 427, 431, 449, 742 Choriocapillaries, 231 Choroid, 230 Choriodal hemangioma, 326, 476 Chorioretinal degeneration, 576 Chorioretinal scar, 382, 534 Chorioretinal scotomas, 582 Chorioretinitis, 52, 239, 260, 261, 262, 732, 741, 742, 759, 761, 762, 763, 765, 766, 767, 771 Choriostomas, 114, 151, 679 Choroid, 4, 153, 227, 230, 239, 393, 448, 701, 721 Choroidal bone formation, 696 Choroidal circulation, 416 Choroidal coloboma, 453 Choroidal crescent, 510 Choroidal degeneration, 552 Choroidal detachment, 453 Choroidal dystrophy, 438 Choroidal efflusion, 698 Choroidal fissure, 1, 233 Choroidal fold, 684 Choroidal haemangioma, 57 Choroidal infarct, 416 Choroidal neovascular membrane, 384 Choroidal neovascularisation, 445, 553, 764 Choroidal nevus, 445 Choroidal rupture, 326, 415, 427, 454, 457 Choroidal sclerosis, 237, 238 Choroidal tear, 326 Choroideremia, 237, 419, 423 Choroiditis, 238, 274, 526, 732, 742, 762 Choroio capillaries, 396 Christmas tree cataract, 296, 330 Chroid, 10 Chroidal effusion, 57 Chroidal repute, 325 Chroiditis, 52 Chroiocapillaries, 552 Chromatic aberration, 501 Chromatopsia, 574, 577 Chromosomal aberration, 12 Chromosome, 472 Chronic anterior uveitis, 97 Chronic blepharitis, 124, 170 Chronic congestive, 24 Chronic conjunctivitis, 45 Chronic cyclitis, 245, 258 Chronic dacryo adenitis, 97 Chronic dacryocystitis, 92, 94, 99, 129, 150, 174, 179, 183, 736, 752 Chronic dacryocystitis in children, 93 Chronic dacryodenitis, 96 Chronic endophthalmitis, 265, 324, 489, 771 Chronic follicular conjunctivitis, 142, 149, 756 Chronic glomerulo nephritis, 434 Chronic granulomatous, 48, 273 Chronic granulomatous inflammation, 48 Chronic granulomatous ulcer, 760 Chronic heart failure, 422 Chronic iridocylitis, 262 Chronic keratitis, 214 Chronic kerato conjunctivitis, 133 Chronic pan uveitis, 272, 275, 761 Chronic papillitis, 253 Chronic progressive external ophthalmoplegia, 69, 76, 78

PEDIATRIC OPHTHALMOLOGY
Chronic retinal detachment, 701 Chronic secondary open angle, 251 Chronic simple glaucoma, 344, 436, 437, 584 Chronic uveitis, 169, 214, 321, 453, 462 Chronic uveitis in young adults phthis bulbae, 249 Chroroideal effusion syndrome13, 9 Chrysops, 772 Cicatricial, 133 Cicatricial ectropion, 62 Cicatricial pemphigoid, 139 Cicatricial trichiasis, 40 Cicatrisation, 125, 188 Cicatrising trachoma, 134 Cidofovir, 756 Cilia, 7 Ciliary band, 4, 624, 342 Ciliary block glaucoma, 357, 358 Ciliary body, 4, 181, 227, 284, 342, 393 Ciliary body, 229 Ciliary circulation, 405 Ciliary congestion, 116, 242 Ciliary epithelium, 4, 230, 341, 377, 456, 458 Ciliary ganglion, 502, 510, 511, 675, 677 Ciliary muscles, 10, 230, 233 Ciliary nerve, 160, 720 Ciliary processes, 4, 230, 233, 335, 340, 344, 488 Ciliary shutdown, 247, 359 Ciliary staphylomas, 62, 345, 723 Ciliary stroma, 230 Ciliary system, 231 Ciliary vessels, 230 ciliary zone, 227 Cilio choroidal detachment, 453 Cilio retinal artery, 405, 414 Cilio retinal vessel, 404 Ciliochoroidal detachment, 230, 282 Ciliolenticular block, 358 Ciprofloxacilin, 727, 737, 739, 743 Ciprofloxin, 128, 137, 179, 192, 271, Circinate retinopathy, 405, 424 Circle of least diffusion, 546, 564 Circle of zinn, 596, 598, 599, 602, 603, 604 Circular fibres, 4, 230 Circum corneal congestion, 242, 271 Circum corneal flush, 24 Circumciliary congestion, 24, 62, 174, 175, 188, 241 Cirsoid aneurysm, 422 Classical galactosemia, 300 Classification of allergic conjunctivitis, 139

INDEX
Classification of amblyopia, 586 Classification of exotropia, 665 Classification of glaucoma in children, 343 Classification of myopia, 547 Classification of nystagmus, 629 Classification of squint, 642 Claudes syndrome, 605 Clear lens extraction, 554, 569 Cleft lip, 682 Cleft palate, 679, 682, 747 Clindamycin, 126, 261, 727, 736, 432, 739, Clinical anisocoria, 234 Clinical anisometropia, 567 Clinical feature of trachoma, 133 Clinical features of corneal ulcer, 175 Clinical features of uveitis in general, 241 Clinical presentation of retinhoblastoma, 472 Clinically significant macular edema, 423 Clonazepam, 635 Clonidine, 375 Cloquets canal, 6, 465, 488 Closed fistula, 101 Closed globe injuries, 325 Closed head injury, 535 Clostridium botulinum, 729 Clostridium perfringes, 727 Clostridium tetani, 728 Clotrimazole, 205, 209 Cloudy cornea, 348 Clutton joints, 207, 740 CME, 245, 271 CMV retinitis, 267 CO2 tension, 487 Coagulase negative cocci, 280, 738 Coagulase positive, 738 Coarse vision, 394 Coats disease, 259, 266, 368, 405, 422, 424, 425, 433, 426, 427, 428, 454, 462, 476, 477, 478, 483, 698 Coats of the eyeball, 230 Cobalt blue, 60, 195, 481 Cobalt blue filter, 101, 105, 176, 178 Cobble stone, 446 Cocaine, 74, 506 Cocaine test, 512 Coccidomycosis, 144, 149, 759, 761 Cockaynes disease, 439 Cockroaches, 772 Coenurosis, 153, 765 Coffee coloured hypopyon, 728 Cogan lid twitch, 76 Cogan Reese syndrome, 337 Coin test, 31 Cokaynes syndrome, 440 Cold test, 76 Colic, 77, 374 Colitis, 730 Collagen diseases, 424, 457 Collagen fibres, 228 Collagenase, 219 Collagenous tissue, 338 Collarette, 227, 235 College myopia, 549 Colliers sign, 81 Coloboma, 18, 39, 81, 162, 229, 233, 236, 416, 476, 622, 683 Coloboma of ciliary body, 235 Coloboma of iris, 19, 27, 187, 576 Coloboma of lens, 303, 488, 564 Coloboma of lid margin, 22, 104, 221 Coloboma of lower lid, 98 Coloboma of macula, 236 Coloboma of optic disk, 584 Coloboma of optic nerve head, 71 Coloboma of retina, 423 Coloboma of the choroid, 457 Coloboma of the disc, 516 Coloboma of the iris, 19, 233 Coloboma of the lens, 304 Coloboma of the lid, 16, 39,62, 173 Coloboma of the lip, 682 Coloboma of the lower lid, 101 Coloboma of the macula, 765 Coloboma of the pupil, 39 Coloboma of the uvea, 12, 19, 484, 558 Coloboma of upper lid, 7, 39 Coloboma with abnormal vessels, 410 Colobomas, 682 Colobomatous cystic eye ball, 8, 233 Colobomatous microphthalmos, 8, 9 Coloquets canal, 3, 457 Colostrum, 219 Colour agnosia, 580 Colour blindness, 574, 577 Colour defect, 13, 32, 411, 530, 538 Colour field, 580 Colour of pupil, 28 Colour of the iris, 227 Colour sense, 520, 537, 575, 582 Colour vision, 394, 396, 401, 586 Coloured haloes, 370 Columnar cells, 89 Combergs nyctometer, 418 Combined trabeculotomy and trabeculostomy, 381 Commensal, 280, 730, 735 Common canaliculus, 86, 88 Common causes of exudative retinal detachment, 454

785
Common causes of junevile glaucoma are, 353 Common causes of sixth nerve palsy in children, 618 Common causes of vitreous haemorrhage in children, 462 Common clinical types of colour blindness, 579 Common conditions that do not require prophylaxis, 455 Common conditions where ERG is useful, 419 Common peripheral retinal degeneration, 445 Common secondaries that involve orbit in children, 708 Common side effect of atropine are, 255 Common vascular retinopathies in adults, 434 Comotio retinae, 442 Compensatory phase, 629 Complete cataract, 17 Complete dislocation, 252 Complete third nerve palsy, 609 Complicated cataract, 175, 196, 204, 244, 274, 407, 488, 696 Complicated congenital ptosis, 70 Complicated contract, 453 Complicated heterochromia, 237 Complicated microphthalmos, 8, 9 Complication in acute anterior uveitis, 247 Complication in buphthalmic eyes, 351 Complication of chronic anterior uveitis, 248 Complication of corneal fistula, 188 Complication of naso lacrimal duct obstruction, 91 Complication of occlusion, 590 Complication of rupture, 444 Complications of corneal ulcer, 185 Complications of herpes simplex keratitis, 196 Complications of posterior uveitis, 253 Complications of retinopathy of prematurity, 487 Composition of aqueous humour, 340 Composition of vitreous, 457 Compound astigmatism, 565 Compound hypermetropic, 565 Compound microscope, 413 Compound myopic, 565 Compound myopic astigmatism, 164, 307 Computed tomography, 697

786
Computerised corneal topography, 164, 166 Computerised keratoscopy, 566 Computerised oscilloscope, 418 Concave glasses, 550 Concave mirror retinoscope, 552 Concomitant, 612, 643, 651, 666 Concomitant divergent squint, 640 Concomitant squint, 585, 623, 649, 652 Concretion, 173 Concussion cataract, 325, 326 Concussion injury, 171, 554 Condensing lens, 413 Conditions where ARC may not develop, 640 Conduction defect, 531 Cone and cone-rod dystrophies, 394, 434, 441 Cone monochromatism., 434, 442, 578 Cone pedicle, 396 Cones, 396, 418, 577 Coneurosis, 152 Confusion, 638 Congenial block of nasolacrimal Duct, 90 Congenital, 41, 68, 73, 121, 229, 258, 263, 303, 433, 611 Congenital achromatopsia, 442, 576 Congenital alternating esotropia, 640 Congenital and developmental cataract, 316 Congenital anisometropia, 567 Congenital anomalies, 214, 453, 475 Congenital anomalies of anterior chamber, 337 Congenital anomalies of optic disc are common, 513 Congenital anomalies of orbit, 678 Congenital anomalies of retinal vessels, 399, 404 Congenital anomalies of the cornea, 161 Congenital anomalies of the lens, 294 Congenital anomalies of the macula, 409 Congenital anomalies of the sclera, 722 Congenital anomalies of the uvea, 233 Congenital anomalies of the vitreous, 458 Congenital anomalies of uvea of late onset, 237 Congenital anomaly of retinal vessels of late onse, 405 Congenital aphakia, 19, 26 Congenital axial myopia, 26 Congenital block of nasolacrimal duct, 90 Congenital brown syndrome, 616 Congenital cataract, 301, 321, 353, 407, 475, 484, 630, 683 Congenital coloboma of macula, 260, 263, 431 Congenital coloboma of the disc, 515 Congenital coloboma of the iris, 27 Congenital coloboma of the lower lid, 90 Congenital coloboma of the macula, 431 Congenital colour blindness, 237, 404 Congenital conjunctival lymphadenoma, 114 Congenital corneal opacities, 168, 212 Congenital corneo scleral cysts, 121 Congenital crescent (conus) of the optic disc, 514 Congenital cyst of lacrimal gland, 94, 95 Congenital cystic eyeball, 8, 515 Congenital cytomegalo virus retinitis, 430 Congenital dacryocystitis, 22, 90 Congenital day blindness, 404 Congenital disc swelling, 519 Congenital dysautonomia, 26 Congenital ectopic, 367 Congenital ectropion of uvea, 63, 353 Congenital esotropia, 40, 666 Congenital esotropia syndrome, 656 Congenital exotropia, 666 Congenital fibrosis of inferior rectus, 609 Congenital fibrosis syndrome, 624 Congenital fistula of the lacrimal sac, 90, 92 Congenital folds, 13 Congenital fourth nerve palsy, 610 Congenital glaucoma, 13, 17, 168, 301, 306, 370, 560, 741, 747 Congenital gliosis, 403 Congenital hamartoma, 539, 710 Congenital hereditary endothelial defect, 216 Congenital hereditary endothelial dystrophy, 25, 167, 212 Congenital hereditary stromal dystrophy, 25, 216 Congenital horners syndrome, 28, 42 Congenital internal ophthalmoplegia, 28 Congenital jerk nystagmus, 629, 630, 634 Congenital keratitis, 352 Congenital lymphe-dema, 114

PEDIATRIC OPHTHALMOLOGY
Congenital melanosis, 24, 117, 440 Congenital membranous cataracts, 298 Congenital microcoria, 234 Congenital miosis, 74 Congenital mucocele, 18, 90, 92, 679 Congenital myopia, 13, 28, 348, 549, 678 Congenital nasolacrimal duct block, 92 Congenital nasolacrimal duct obstruction, 91, 348 Congenital night blindness, 404, 442, 574, 575 Congenital nuclear cataract, 297 Congenital nystagmus, 628 Congenital optic atrophy, 634 Congenital pigmentation of the retina, 400 Congenital pit of the optic nerve, 453, 515 Congenital ptosis, 41, 68, 78 Congenital retinal degeneration and dystrophies, 404 Congenital retinal fold, 476 Congenital retinoschisis, 403, 447, 464, 465, 475 Congenital rosette formation, 403 Congenital rubella, 25, 216, 261, 747 Congenital staphyloma, 722, 723 Congenital stationary night blindness, 419, 574 Congenital syphilis, 171, 262, 429, 430, 740 Congenital telangiectasis of conjunctiva, 115 Congenital toxoplasmosis, 236, 246, 454, 696, 765 Congenital tumour, 472 Congenital uveitis, 352 Congenital varices, 692 Congenitial myopia and myopia of pre-maturity, 548 Congo red, 211 Congruous field defect, 581, 584 Conical detachment, 462 Conjugate gaze palsy, 619 Conjugate movement, 595, 610 Conjunctiva, 7, 14, 34, 56, 86, 93, 95, 102, 149, 154, 336, 676, 692, 759 Conjunctiva and sclera, 111 Conjunctival, 152 Conjunctival biopsy, 106 Conjunctival blanching, 375 Conjunctival bleeding, 119 Conjunctival congestion, 116, 134, 203 Conjunctival cysts, 121, 153

INDEX
Conjunctival discharge, 24, 120 Conjunctival effusion, 742 Conjunctival epithelium, 110, 113, 120, 159, 171 Conjunctival follicles, 119 Conjunctival glands, 98, 113 Conjunctival hood flap, 189 Conjunctival impression cytology, 106 Conjunctival infiltration, 479 Conjunctival injection, 116 Conjunctival involvement, 201 Conjunctival membrane, 121 Conjunctival papillae, 120 Conjunctival recession, 662 Conjunctival scrapping, 106 Conjunctival shortening syndrome, 620, 623 Conjunctival smear, 123, 127, 136 Conjunctival ulcer, 762 Conjunctival veins, 336 Conjunctival xerosis, 24, 217 Conjunctivitis, 47, 50, 83, 86, 93, 116, 271, 571, 742, 743, 746 Conjunctivitis in new born, 126 Conjunctivitis of unknown causes in children, 148 Connections of the third nerve nucleus, 601 Consanguinity, 16, 47, 315, 435, 440, 443, 538, 575 Consecutive esotropia, 533, 663, 664 Consecutive optic atrophy, 422, 436, 532, 534 Consensual light reflex, 28 Consolidation of lung, 736 Constant exotropia, 665, 667 Constant occlusion, 589 Constricted peripheral field, 404, 584 Constrictor muscle, 306, 502 Contact dermatitis, 760 Contact lens, 103, 165, 169, 171, 195, 203, 208, 212, 270, 321, 309, 320, 375, 418, 550, 554, 558, 564, 567, 569, 635, 737 Contact lens fitting, 176 Contact lens intolerance, 143 Contact lens solutions, 208, 737 Contact lens wearer, 170 Contagious conjunctivitis, 131 Contaminated fluorescein, 192 Contents of anterior chamber, 26 Contra lateral antagonist, 625 Contra lateral hemiplegia, 202 Contrast, 537 Contrast sensitivity, 526, 533, 582, 586 Controlled radiation, 480 Contusion, 325 Convection current, 341 Conventional dacryocystography, 102 Conventional occlusion, 589 Conventional outflow, 341 Conventional trabeculectomy, 366 Convergence deficient exotropia, 667 Convergence excess esotropia, 662 Convergence paralysis, 668 Convergence reflex, 504 Convergent squint, 410, 618, 642 Convex mirror, 166 Convulsion, 260, 330, 431, 489, 765, 770 Coralliform, 296, 298 Corectopia, 27, 229, 234, 338, 501 Corkscrew cataract, 296 Cornea, 14, 56, 86, 102, 111, 418, 546 Cornea plana, 25, 26, 161, 163, 306, 308, 335, 339, 556 Corneal, 23, 585 Corneal abrasion, 24, 62, 90, 97, 195, 348, 576, 723 Corneal abscess, 208 Corneal antigen, 191 Corneal astigmatism, 563, 564 Corneal biopsy, 177 Corneal calliper, 18, 166 Corneal changes in trachoma, 135, 681 Corneal complication, 125, 248, 257 Corneal curvature, 25, 162 Corneal decompensation, 351 Corneal degenerations, 214 Corneal diameter, 474 Corneal dystrophies, 162, 168, 210, 190 Corneal dystrophies, 168 Corneal edema, 24, 167, 172, 173, 337 Corneal electrode, 420 Corneal endothelium, 160 Corneal epithelium, 2, 9, 110, 159, 293 Corneal fistula results, 174, 175, 187, 188 Corneal graft rejection, 170 Corneal haze, 16, 19, 24, 747 Corneal involvement in phlycten, 146, 201 Corneal light reflex, 642 Corneal loupe, 242 Corneal mucus plaque, 188 Corneal nerves, 56, 164, 175, 211, 214 Corneal oedema, 324 Corneal opacity, 39, 95, 147, 163, 167, 172, 174, 185, 324, 628, 665, 668, 686 Corneal opacity in new born and infants, 215

787
Corneal plaque, 143, 166 Corneal reflexes, 639, 652 Corneal scarring, 166, 172, 196, 565 Corneal scleral shell, 144 Corneal sensation, 42, 210, 175, 195 Corneal staphyloma, 166, 169, 184, 188, 219 Corneal stroma, 10, 336 Corneal surface, 166 Corneal thickness, 701 Corneal toxicity, 197 Corneal transparency, 189 Corneal ulcer, 24, 50, 91, 97, 133, 166, 167, 172, 173, 188, 576, 710, 726, 760 Corneal ulcer in children, 172 Corneal ulcer threatening to perforate, 184 Corneal vascularisation, 147, 172 Corneal xerosis, 217 Corneo scleral junction, 159, 161, 324 Corneo scleral trephine, 350, 381 Corneo scleral meshwork, 336 Corneo scleral sulcus, 345 Corneo scleral trephine, 379 Coronal suture, 680 Coronary, 296 Corporis, 46 Correcting glasses, 645 Correctopia, 212, 614 Corresponding points, 637 Corrugated, 451 Cortex, 2, 292, 457, 587 Cortical adaptation, 639 Cortical blindness, 18, 21, 28, 421, 530, 584, 689 Cortical material, 26, 354 Cortical matter glaucoma, 367 Cortical venous thrombosis, 521 Cortico nuclear tract of both sides, 601 Cortico spinal tract, 605, 606 Cortico steroids, 251, 255, 260, 529 Corticosteroids, 330 Cortisol, 361 Corynebacterium diphtheriae, 122, 173, 729 Cosmetic blemish, 68 Cotton wool appearance, 424 Cotton wool spots, 267, 749 Cover test, 79, 615, 640, 643, 645, 652 COWS, 632 Coxsackie virus, 745 Cracker injury, 324 Cranial cavity, 697 Cranial nerve palsy, 52, 202, 203 Cranial neuritis, 56, 97 Cranial neuropathy, 276

788
Cranio facial anomalies, 38, 670 Cranio facial dysostosis, 16, 612, 678 Cranio facial injury, 536 Cranio pharyngioma, 508, 583, 696 Cranio synostosis, 348, 522, 679, 687, 688 Craniofacial, 13, 18 Craniofacial dysostosis, 678, 679, 681 Craniotomy, 541, 679, 680, 681 Crater, 139 Creds prophylaxis, 128, 133 Criswick-Schepen Syndrome, 465 Critical angle, 342 Crocodile tear, 608, 622 Cross fixation, 587, 633, 655, 659 Crouzons disease, 678, 695 Crouzons syndrome, 306, 681 Crowding phenomenon, 586, 588 Cryo, 58, 181, 349, 455, 480, 756 Cryo application, 143 Cryo coagulation, 409, 456 Cryo freezing, 266 Cryo therapy, 408, 487 Cryo-coagulation, 464 Cryotherpy, 480, 489, 493 Cryptococci, 750 Cryptococcus, 750 Cryptocoria, 19, 27 Cryptophthalmos, 16, 94 Crypts of henle, 103, 228 Crystalline dystrophy (Schnyders), 212 Crystalline penicillin, 128, 129 Crystalline salts of calcium carbonate, 214 CSF examination, 528 CSR, 416 CT, 416 Culture, 179 Cup of the disc, 510 Curvature hypermetropia, 163, 547, 556 Curvature myopia, 303, 547, 554 Curvature of cornea, 166 Cushing syndrome, 257 Cutaneous, 147 Cutaneous albinism, 400 Cutaneous diptheria, 730 Cutaneous erythema, 489 Cutaneous involvement, 58 Cutaneous melanoma, 479 Cutaneous tuberculosis, 731 Cyanide metabolism, 537 Cyanide sodium nitropruside, 310 Cyanoacrylate, 198 Cyanosis, 577, 737 Cycles per second, 629 Cyclic esotropia, 654, 657, 663, 664 Cyclic oculomotor palsy, 608 Cyclitic membrane, 354 Cyclitis, 239, 245, 271, 369 Cyclo cryo, 381 Cyclo destructive procedure, 351, 369, 380 Cyclo phosphamide, 708 Cyclo vertical muscles, 597 Cyclo vertical recti, 597 Cyclo vertically acting muscles, 593 Cycloablation, 384, 385 Cyclodestructive surgeries, 381 Cyclodialysis, 350, 366, 379, 381 Cyclodiathermy, 381 Cycloduction, 595 Cyclope, 773 Cyclopentolate HCL, 198, 208, 254, 263, 264, 269, 506 Cyclopentolate11, 20 Cyclophoria, 645, 648 Cyclophosphamide, 78, 482, 706, 708, 709 Cyclophoto coagulation, 385 Cycloplegia, 32, 46, 47, 49, 549, 618, 647 Cycloplegic, 8, 50, 124, 126, 131, 173, 180, 184, 197, 199, 208, 254, 264, 269, 563, 730 Cyclosporin, 78, 143, 258, 706 Cyclotonia, 373, 505, 650 Cyclovertical muscles, 613 Cycloxygenase, 257 Cydopentolate, 318 Cyno acrylate glue, 144 Cynoacrylate, 185 Cyst, 408 Cystathionine B. synthase, 309 Cystic eye ball, 1, 18, 722 Cystic phase, 765 Cysticercosis, 152, 522, 769 Cysticercus cellulose, 122, 152 Cysticerosis, 240, 765 Cysticircus, 769 Cystine, 42, 311, 537, 762 Cystinuria, 439 Cystoid cicatrix, 122 Cystoid degeneration, 445, 553 Cystoid macular, 272, 324 Cystoid macular edema, 57, 245, 246, 247, 257, 272, 277, 374, 376, 415, 423, 436, 437, 460, 490 Cystoid macular edema and papillitis, 248, 324, 326 Cystonosis, 439 Cysts and parasites, 417 Cysts of the conjunctiva, 151 Cytology of discharge, 121

PEDIATRIC OPHTHALMOLOGY
Cytomegalo inclusion virus, 295 Cytomegalo virus, 72, 260, 433, 751, 755 Cytomegalo virus retinitis, 429, 430, 432, 741, 745, 750 Cytomelagic virus, 267 Cytopenaea, 258 Cytotoxic drug, 191, 195, 258, 706

D
D.N.A. viruses, 193 Dacroyocystography, 102 Dacryo cysto rhinostomy, 91 Dacryoadenitis, 96, 726, 738, 744, 746, 759 Dacryocystectomy, 100, 101 Dacryocystitis, 4, 49, 91, 703, 736, 738, 743, 748, 760 Dacryocystocele, 92 Dacryocystography, 93 Dacryolith, 98 Dactinomycin, 708 Dark adaptation, 411, 436, 441, 442, 443, 551, 574, 586 Dark adaptometry, 412 Dark field examination, 740, 741 Dark wedge test, 615 Daughter cysts, 769 Day blindness, 576 Day vision, 394 Deaf and dumb, 680 Deafness, 276, 621 Debridement, 181, 198, 199, 205 Decentered lens, 564 Deep exudate, 424 Deep keratitis, 94, 170 Deep vascularisation of cornea, 167, 170, 196, 206 Defective colour vision, 441 Defective contrast, 527 Defective stereopsis, 527 Defective vision, 557 Definite host, 152, 769 Deformity of medial turbinate, 101 Degeneration of cornea, 188 Dehiscence of levator, 69 Delayed (cellular) hypersensitivity, 138, 760 Delayed lid changes, 52 Delayed type-four hypersensitivity, 273 Delayed visual maturation, 21 Delen fuchs nodule, 273 Delivery system, 382 Demecarium, 505 Demodicosis, 765 Demonstration of leaking wound, 176

INDEX
Demyelinating diseases, 529 Demyelination, 514, 525, 526, 619 Dendrile, 194, 195, 753 Dendrites of herpes zoster, 201 Dendritic and geographic ulcer, 197, 199, 754 Dendritic keratitis, 127, 131 Dendritic ulcer, 195, 759 Deorsum duction, 595 Deorsum version, 595 Depiveprin, 372, 373, 374 Deposits on cornea, 26, 167, 188 Depot steroid, 60, 275 Depression, 595, 598, 599 Deprivation amblyopia, 60, 685 Depth of amblyopia, 588 Depth of focus, 501 Depth of suppression, 615 Dermal leishmaniasis, 768 Dermatitis medicamentosa, 44, 149, 255 Dermatome, 199, 200, 752 Dermatophytosis, 759, 762 Dermoid, 4, 25, 39, 114, 151, 152, 166, 189, 216, 622, 675, 683, 685, 687, 688, 690, 692, 696, 698, 707 Dermolipomas, 39, 114, 151, 683, 685 Descemet membrane, 3 Descemetocele, 174, 187 Descemets membrane, 112, 159, 161, 164, 174, 181, 186, 188, 210, 335, 338, 342, 344, 366 Descending arm, 700 Descending optic atrophy, 532 Desmetocele corneal ulcer, 167, 208 Detachment coloboma, 13 Detection and measurement of cyclophoria, 646 Deturgscence, 160, 161 Deuteranomalous, 578 Deuteranomaly, 578 Deuteranopia, 578 Development of anterior chamber, 336 Development of chriocapillaries, 5 Development of cornea, 161 Development of extra ocular muscles, 4, 13, 600 Development of lacrimal and accessory lacrimal, 87 Development of lacrimal system 7, 7 Development of lid, 7 Development of macula, 399 Development of oculomotor nerves, 601 Development of optic nerve, 512 Development of orbit, 677 Development of retinal pigment epithelium, 398 Development of sclera, 722 Development of sensory (pars optica) retina, 399 Development of the lacrimal drainage system, 89 Development of the lens, 1 Development of the macula, 5 Development of the orbit, 7 Development of the uvea, 232 Development of vitreous, 458 Developmental amblyopia, 587 Developmental buphthalmos, 343 Developmental cataracts, 294, 315, 401, 475, 680 Developmental causes of night blindness, 575 Developmental glaucoma, 343 Deviational nytagmus, 631 Devics neuro myelitis optica, 526, 527, 530 Devics neuromyelitis, 529 Dewormed, 153 Dexamethasone, 143, 147, 256, 361, 536 Dextro cycloversion, 595 Dextro elevation, 595 Dextro version, 629 Dextroversion, 595 Diabetes, 119, 179, 257, 330, 554, 759, 762 Diabetes insipidus, 541, 714 Diabetes mellitus, 299 Diabetes under treatment, 16 Diabetic cataracts, 301 Diabetic insipidus, 711 Diabetic ketosis, 762 Diabetic neovascularisation, 171 Diabetic oculomotor palsy, 607 Diabetic retinopathy, 414, 424, 426, 434 Diabetics proliferative retinopathy, 462 Diagnosis, 150 Diagnosis of amblyopia, 588 Diagnosis of conjunctival rhinosporidiosis, 150 Diagnosis of fungal keratitis depends, 204 Diagnosis of galactose cataract, 300 Diagnosis of heterophoria, 645 Diagnosis of myasthenia, 77 Diagnosis of trachoma, 136 Diagnostic features in primary buphthalmos, 346 Diagnostic procedure for retinal dystrophy, 440

789
Dialysis, 449 Dialysis of retina, 449 Diameter of cornea, 79 Diamidine, 209 Diaphragma sella, 512 Diarrhoea, 77, 219, 374, 576, 730, 767 Dichromatism, 578 Diclofenac, 257 Diclofenac sodium, 142 Diclorphenamide, 377 Dietary deficiency, 13 Diethyl carbamazine, 154 Differential diagnosis, 49, 153, 615, 624 Differential diagnosis of phlycten, 147 Differential diagnosis of primary buphthalmos, 348 Differential diagnosis of rhegmatogenous retinal, 453 Differential diagnosis of sixth nerve palsy, 620 Differential intra ocular pressure test, 693 Diffuse chorioretinitis, 262, 534 Dihydro phenylalaline, 401 Dihydro sangunarine, 369 Diktyomo, 284, 368 Dilator muscles, 15, 306, 502 Dilator pupillae, 228 Dimer, 384 Diminish corneal sensation, 178 Diminished central vision, 441 Diminished distant vision, 436 Diminished night vision, 329, 404, 411, 436, 465 Diminished peripheral field, 436 Diminished vision, 242, 411 Dimorphic fungus, 761, 763 Diode Laser, 351, 382, 384, 416, 487 Dionin, 190 Diopteric, 546 Diphtheretic conjunctivitis, 123, 125 Diphtheria, 121, 174, 175 Diphtheroid, 110 Dipiveprin, 371 Diplegia, 680 Diplococci, 191 Diplopia, 75, 76, 77, 78, 523, 567, 570, 608, 610, 611, 618, 625, 638, 639, 651, 684, 689, 706, 729, 759, 763 Direct astigmatism, 564 Direct gonioscope of koeppe, 342, 346 Direct goniotomy, 349 Direct opthalmoscope, 246, 412, 552, 553, 632, 723, Direct part, 598 Direct trauma to conjunctiva, 118

790
Disc angiomas, 58 Disc swelling, 521 Disciform, 296 Disciform degeneration, 416 Disciform degeneration of macula, 424, 435 Disciform keratitis, 49, 51, 53, 196, 201, 208, 751, 752, 755 Disinsertion of levator, 69, 449 Disjugate movements, 595 Disjunct nystagmus, 634 Dislocated lens, 417, 453 Dislocated lens nucleus, 282 Dislocation of cataractous lens, 325, 345 Disorders of glands of lid margin, 47 Disparate points, 637 Disseminated choroiditis, 239 Dissociated hyper deviation, 614 Dissociated vertical squint, 613, 614, 648, 653, 655 Dissociation of light near reflex, 506 Distance dysfunction, 649 Distant metastasis, 483 Distichiasis, 22, 39, 40, 682 Diuretics, 103 Diurnal variation, 341 Divergence excess, 648 Divergence excess exotropia, 667 Divergence weakness, 648, 647 Divergent squint, 642, 680 Diverticule, 102 DNA, 744, 749 DNA inhibitor, 197 Documentation of corneal lesions, 176 Dog tapeworm, 153 Dolichocephaly, 680 Dolicocephalous, 308, 681 Dolicocephaly, 681 Dolls eye movement, 624 Dominant drusen of the retina, 445, 435 Dominantly inherited bests vitelli form, 420 Dorellos canal, 620 Dorsal, 6 Dorsal mid brain lesion, 80 Dorsal ophthalmic artery, 458 Dorsal pons, 619 Dorsal roof ganglion, 752 Dorso lateral pons, 619 Dorsum sella, 510 Dorzolamide, 377 Dot visual acuity test, 31 Double depressor palsy, 609 Double elevator palsy, 609, 613 Double eversion, 112 Double hyperphoria, 648 Double Maddox rod, 611, 645, 646 Double ring, 208 Double vision, 638 Doubling of the fundus, 305 Down beat nystagmus, 634 Down syndrome marfans syndrome, 40, 163, 318, 695 Doxorubicin, 482 Doxycyclin, 47, 137, 147, 727, 739, 742, 743, 758 Doynes choroiditis, 424 Doynes occluder, 589 Dracanculosis, 773 Dracanculus medinensis, 765, 773 Dracunculosis, 765 Draeger tonometer, 341 Dragging of retina, 487 Draining devices, 349 Draining vein, 408 Drops, 256 Drug abuse, 759 Drug induced, 149 Drug induced cycloplegia, 32 Drug induced myopia, 555 Drug induced optic neuritis, 529 Drugs, 148 Drusen, 59, 415, 437, 524 Drusen of optic nerve, 426, 427, 445, 517, 519, 524, 532, 542, 584, 701 Drusen of the retina, 423 Dry eye, 103, 120, 136, 139, 140, 142, 183, 481, 708 Dry spot, 105 Duanes retraction syndrome, 68, 621, 624, 656, 693 Duct of the meibomian gland, 48 Duction, 595 Ductusarteriosus, 747 Duplication of fundus, 307 Duplication of optic nerve head, 515 Dura, 5, 511, 513, 674, 676 DVD, 590, 656, 659, 666 Dwarfism, 541, 711 Dysarthria, 729 Dyschromatopsia, 329, 525, 526, 527, 538 Dyscoria, 234, 338, 614 Dysentery, 271, 730 Dysgenesis of anterior chamber, 12, 190, 229, 335 Dyskinesia, 618 Dyslexia, 574, 580 Dysplasia and a plasia of retina, 338, 402, 513, 682, 728, 729, 730 Dysplasia of macula, 236 Dyspnea, 737

PEDIATRIC OPHTHALMOLOGY
Dysproteinemias, 424, 454 Dysthroid oculopathy, 75 Dysthyroid eye disease, 691 Dysthyroid myopathy, 77, 80 Dysthyroid oculopathy, 609 Dysthyroid ophthalmopathy, 613 Dystrophy, 13, 160, 163, 167, 171 Dystropies of cornea, 188 Dysuria, 733 Dysversion, 513

E
E. coli, 126 E. multi locularis, 768 E. oligoarthus, 768 E. vogeli, 769 Eales disease, 426, 427, 433, 454, 462 Early papilledema, 523 Early retinopathy, 485 Early rosette cataract, 325 Early venous phase, 415 Eccentric fixation, 586, 588, 590, 641, 652 Eccentric proptosis, 691 Ecchymosis, 43, 44, 314, 620, 709 Echinococcosis, 152, 153, 765 Echinococcous granulosus, 768 ECHO viruses, 745 Echothiophate, 505 Econazole, 205 Ectasia, 26, 185 Ectatic cicatrix, 186 Ectodermal, 1, 87, 458, 519, 539 Ectopia lentis, 13, 163, 301, 304, 311, 314 Ectopia pupil, 234 Ectopic lens, 234 Ectopic macula, 612, 652 Ectopic pupil, 306, 501 Ectopion, 39 Ectropion, 40, 63, 81, 98, 101, 111, 173, 200, 221, 337, 340 Edema, 16, 37, 43, 111, 143, 188, 212, 422 Edematous cornea, 177 Edinger westphal nucleus, 502, 503, 601 Edridge green lantern, 579 Edrophonium, 505 EDTA, 215 Effect of congenital palsy, 610 Effective origin, 598 Efferent disorder, 511 Efferent path, 628 Efferent pupillomotor defect, 510 Egg yolk, 444 Ehler Danlos, 453

INDEX
Ehler-Danols syndrome, 163, 306, 314, 446, 464, 723 Eight ball hyphaema, 363, 365 Eiphora, 94 El Bayadi lens, 412, 460 Elective caesarean section, 128 Electric cataract, 328 Electric shock, 324 Electro nystagmograph, 420 Electro oculography, 412, 420 Electro retinogram, 412, 418, 441 Electro-encephalogram, 21, 420 Electro-retinogram, 21 Electrocautery, 50 Electromyographic control, 657 Electrophoresis, 103 Electrophysiological investigations, 21 Elephantiasis neuro fibromatosa, 56 Elevation, 595, 597, 599 Elisa test, 260, 266, 268, 433, 489, 743 Ellsworth classification, 480 Elschnigs pearls, 322, 323 Embedded foreign bodies, 121 Embrayonic fissure, 458 Embryonal, 2, 513, 515, 707, 708 Embryonic, 1, 8, 233, 513 Embryonic fissure, 8, 233, 512 Embryonic lens nucleus, 293 Embryonic nucleus, 2, 291, 292 Embryonic pigmentary retinopathy, 100, 400, 429, 430 EMG, 657 Emmetropia, 14, 412, 519, 546, 650 Emphysema, 36, 687, 688, 689, 736 Enccphalo trigeminal angiomatosis, 151 Encephalitis, 49, 52, 97, 98, 200, 202, 259, 510, 522, 526, 576, 668, 735, 742, 744, 746, 748, 752 Encephalo facial angiomatosis, 55 Encephalo trigeminal syndrome, 57 Encephalocele, 678, 688, 691, 692, 695 Encephalopathy, 276, 748 Encircling bands, 456 Encroachment of conjunctiva on cornea, 189 Encysted mucocele, 679 End point nystagmus, 629, 633 Endemic, 133 Endo cervicitis, 733 Endo laser coagulation, 464 Endo laser photo coagulation, 427, 433 Endo-genous bacterial endophthalmitis, 433 Endocarditis, 733, 734, 737 Endogenous, 13, 117, 144 Endogenous endophthalmitis, 278 Endogenous uveitis, 240, 258 Endolymph, 632 Endometritis, 733, 757 Endophthalmities, 44, 118, 127, 175, 184, 188, 239, 241, 243, 246, 248, 253, 266, 278, 323, 326, 329, 417, 428, 430, 459, 461, 481, 483, 489, 526, 701, 726, 727, 728, 730, 731, 734, 736, 737, 738, 739, 743, 760, 762, 763, 764 Endophthalmitis phacoanaphylactica, 277, 282, 294 Endophytic tumours, 477 Endophytum, 58, 478 Endophytum retinoblasoma, 478 Endospores, 385, 761 Endothelial channels, 712 Endothelial decompensation, 97 Endothelial defect, 405 Endothelial dusting, 243 Endothelial dystrophy, 212, 348, 741 Endothelial meshwork, 336 Endothelial plaque, 177, 182 Endothelial pump system, 161 Endothelium, 160, 161 Endothelium of cornea, 10 Endothelium of the iris, 160 Endotoxin, 280 Enlargement of blind spot, 516, 520, 534, 584 Enlargement of cornea, 344 Enlargement of the blind spot, 514, 518 Enlargement of the orbits, 695 Enophthalmos, 8, 20, 68, 74, 512, 684, 690 Entamoebahistolytica, 764, 766 Enteritis, 744 Entero virus, 132, 745 Enteroccocci, 739 Enterocollitis, 767 Entrapment of muscle, 693 Entropion, 39, 63, 116, 121, 125, 148, 173, 200, 730 Entropion in children, 63 Entropion of lid, 173 Enucleated, 284, 274, 696 Enucleated and eviscerated sockets, 68 Enucleation, 274, 696 Environment, 471 Enzyme, 377 EOG, 238, 420, 443, 575 Eosin, 209 Eosinophilic granuloma, 689, 713 Eosinophils, 121 Epaulet, 170, 207

791
Ephidrine, 506 Epiblepharon, 39, 40, 41 Epibulbar congenital tumours, 114 Epicanthic, 22, 39, 40, 79, 642 Epidedymitis, 733 Epidemic conjunctivitis, 119 Epidemic dropsy, 354, 369 Epidemic haemorrhagic conjunctivitis, 24 Epidemic kerato conjunctivitis, 23, 44, 121, 123, 126, 131 Epidermoids, 679, 685, 687, 688 Epididymitis, 757 Epikaratophakia, 320, 321 Epikeratoplasty, 165 Epilate, 184 Epilation forceps, 46 Epilepsy, 680 Epimerase, 300 Epinephrine, 255, 361, 362, 364, 365, 371, 372, 373, 374, 506 Epiphora, 46, 82, 93, 100, 373 Epiretinal membrane, 416 Episclera, 336, 721 Episcleral congestion, 24 Episcleral nodules, 768 Episcleral pressure, 57, 342, 368, 375, 686 Episcleral tissue, 152 Episcleral veins drain, 336 Episcleral vessels, 341, 720, 721 Episcleritis, 201, 732, 742, 753, 758 Epistaxis, 115 Epistein-Barr virus, 253, 745 Epitarsus, 114 Epitaxis, 118 Epithelial cells, 243 Epithelial debries, 90 Epithelial defect, 185 Epithelial down growth, 167, 188, 189 Epithelial implantation cyst, 151 Epithelial keratitis, 53, 131, 132, 195 Epithelial keratopathy, 50 Epithelial macro erosion, 141 Epithelial punctate infiltrates, 51 Epithelial tissue, 87 Epithelioma, 59 Epithelium, 160, 161, 159, 168 Epithelium of ciliary body, 4 Epithora, 98 Epstein-Barr virus, 193, 267, 759 Equator, 291, 394, 473, 721 Equatorial staphylomas, 352, 724 ERG, 238, 419, 442, 443, 574, 575 Erosion, 210, 211 Erosion of cornea, 376 ERP, 419

792
Erratic fixation, 641 Error of refraction, 47, 70, 143, 346, 402, 513, 515, 581, 585, 615, 617, 621, 623, 644, 647, 650, 670 Errors of metabolism, 309 Errors of refraction, 13, 46, 47, 124, 234, 237, 587, 631, 642, 664 Erysipelas, 738, 739 Erythema multi forme major, 148 Erythema multi formis, 121, 126, 139 Erythema multiform minor, 148 Erythematous, 148 Erytholastic glaucoma, 365 Erythroblastosis fetalis, 741 Erythromycin, 47, 128, 129, 130, 133, 137, 147, 179, 192, 193, 271, 729, 730, 735, 736, 739, 742, 743 Erythropsia, 426, 459, 577 Escherichia coli, 644, 647, 730 Esophoria, 557, 621 Esotropia, 83, 431, 444, 474, 550, 551, 557,558, 587, 621, 623, 624, 633, 638, 653, 654 Esotropia in myopia, 658, 662, 663 Esotropic, 621, 650 Espundia, 767 ESR, 607 Essen prognosis Classification, 483 Essential amino acid, 309 Essential blepharo spasm, 658 Essential iris atrophy, 251, 337 Established papilledema, 523 Estropia, 260 Ethambutol, 526, 529 Ethmoid, 673, 694 Ethmoidal sinuses, 511, 674 Ethyl morphine, 190 Etiology, 273 Etiology of accommodative esotropia, 659 Etiology of anisometropia, 567 Etiology of concomitant squint, 649 Etiology of congenital, 295 Eucatropine, 506 Euryblepharon, 39, 41 Evaluation of a case of proptosis, 78, 686 Eviscerated, 481 Evisceration, 274 Evisceration of the eye, 283 Evoked vestibular nystagmus, 630 Ewings sarcoma, 686, 688, 708, 709 Exadative retinal detachment, 454 Examination of anterior chamber, 26 Examination of iris, 26 Examination of lens, 28 Examination of pupillary light reflex, 28 Examination of sclera, 26 Examination of the lids, 79 Examination of vitreous, 28 Exanthematous, 49, 747, 752 Exccyclotropia, 611 Excentration, 708 Excimer, 210, 211, 215, 249, 270, 382, 383 Exciting eye, 273 Exciting filter, 416 Excoriation, 141, 149 Excursion, 79 Excyclodeviation, 656 Excycloduction, 595 Excyclophoria, 611, 645, 649 Exfoliations, 342 Exocrine, 87 Exogenous, 13, 117, 139 Exogenous endophthalmitis, 279 Exogenous microbial uveitis, 240 Exophoria, 76, 550, 644, 648 Exophthal, 117 Exophthalmometer, 691 Exophthalmos, 62, 80, 81, 111, 681, 684 Exophytic tumours, 477 Exophytum retinoblastoma, 478 Exotoxin, 727 Exotropia, 550, 596, 605, 611, 616, 621, 639, 653, 658, 664, 678, 681 Exposure keratitis, 82, 177, 680, 681, 684, 685, 686, 704, 707, 710 Expulsive haemorrhages, 351 Extended wear lenses, 144, 321 Extensive coloboma, 233 External auditory, 82 External beam radiation, 481, 708 External carotid, 38 External filtration procedures, 381 External limiting membrane, 395, 396, 477 External nares, 101 External ophthalmoplegia, 605, 681, 728 External pterygoid muscle, 72 External trabeculotomy, 349, 350 Extortion, 598, 599, 615 Extortors, 597 Extra capsular cataract extraction, 322 Extra conal lesion, 675 Extra dural abscess, 620 Extra dural hematoma, 606 Extra ocular metastasis, 471 Extra ocular muscle involvement, 479 Extra ocular muscle palsy, 420, 702 Extra ocular muscles, 10, 42, 697 Extra ocular retinoblastoma, 695

PEDIATRIC OPHTHALMOLOGY
Extra pulmonary tuberculosis, 147, 731 Exudates, 423, 429 Exudative membrane, 280 Exudative retinal detachment, 246, 260, 265, 276, 453, 771 Exudative retinal vasculopathy, 405 Exudative vitreo retinopathy, 464 Eye, 97 Eye in primary buphthalmos, 344 Eye speculum, 20 Eye with leucoma adherence, 186 Eyebrow, 22, 34 Eyes, 765

F
Fabrys disease, 301 Face wash, 137 Facial, 38 Facial asymmetry, 610 Facial canal, 61, 82 Facial diplegia, 62, 81, 83 Facial nerve, 81 Facial palsy, 98, 619, 728, 742 Facicular, 73 Faciomandibular abnormality, 16 Factors that precipitate decompensation, 647 Faculatative hypermetropia, 556 Facultative anaerobes, 738, 739, 744 Facultative hypermetropia, 248, 255 Facultative suppression, 641, 666 Faden operation, 616 Faden procedure, 623 Failed DCR, 101 Failed keratoplasty, 215, 249 Failed sclerotomy, 385 Failure to identify causative organism, 183 Falciparum, 766 Famciclovir, 197 Familial dysautonomia, 511 Familial exudative vitreo retinopathy, 28, 407, 433, 476, 489 Familial exudative vitreoretinal dystrophy, 435 Familial idiocy, 442 Farbers disease, 425 Farsightedness, 555 Fascia bulbi, 676 Fascicle, 603, 606 Fascicular, 602 Fascicular lesion, 619 Fascicular part, 602 Fascicular ulcer, 146, 170, 576, 732 Fasciculo nuclear, 603 Fast eye movement, 595

INDEX
Fast fibres, 593 Fate of retinal haemorrhages, 427 Fatty acidamide, 376 Faulty projection, 326 Favre goldmann syndrome, 464, 465 Feeding artery, 408 Feldman adaptometer, 418 Fenestrated, 231 Fenestrated walls, 414 Fenestration of optic nerve sheath, 536, 538 Fenestreted, 334 Festooned, 244, 250 Fetal distress, 16 Fever, 747 Fibrenous exudates in the AC, 244 Fibrin, 181 Fibro vascular proliferation, 170 Fibroblastic reaction, 188 Fibroblasts, 228 Fibrocytes, 167, 174, 185, 186, 188 Fibroma molluscum, 56 Fibrosis, 256 Fibrosis of trabecular meshwork, 354 Fibrous dysplasia, 687, 688, 691, 695, 696 Fibrous histocystoma, 479 Field changes, 370 Fifth nerve, 97 Filamentary keratitis, 105, 195 Filamentary keratopathy, 176 Filamentous, 203, 763 Filling defect, 102, 416 FIN, 568 Fine KPs, 242 Fine needle biopsy, 540, 706 Fine pannus, 234 Fine vision, 394 First neuron, 73, 503 Fish spawn, 322 Fissures of the orbit, 676 Fistula, 92 Fistula of lacrimal gland, 94, 95 Fistula of sac, 91, 99 Fixation forceps, 692 Fixation reflex, 16, 637 Fixational nystagmus, 630 Fixed bodies, 461 Fixed thick hypopyon, 281 Flame shaped haemorrhages, 524 Flares, 242, 272 Flash VER, 421 Flashes of light, 411, 446 Flat retinoblastoma, 423 Flecked retina syndromes, 443 Flecks, 444 Fleurettes, 477, 478, 482 Flexner Wintersteiner, 477, 482 Floaters, 245, 246, 252, 446, 450, 462 Floating cyst in the vitreous, 153 Floor of the orbit, 599, 673 Florid stage, 206 Floriform, 298 Flourescein angiography, 58, 176, 245, 251, 274, 276, 277, 394, 405, 407, 409, 412, 424, 426, 444, 523, 764 Fluconazole, 209 Fluid gas exchange, 278 Fluid shifts, 455 Fluorescein, 172, 173, 174, 175, 176, 177, 178, 184, 203, 348, 398 Fluorescein drops, 737 Fluorescein sodium, 46, 101 Fluorescence, 91, 414 Fluoresecin staining, 105 Fluoreseine clearance, 101 Fluorometholone, 143 Fluoroquinolones, 526, 735, 744 Flurbiprophen, 140, 142 Flurometholone, 361 Fluroquinolones, 726 Fluroquinols, 193 FML, 147 Focal interval, 564 Focal point, 563 Foetal, 2 Foetal fissure, 293 Foetal nucleus, 292 Foetus, 754 Foggin, 567 Folds in descemets membrane, 171, 189, 196 Folic acid antagonist, 432 Folicular conjunctivitis, 53 Folinic, 261 Folinic acid, 432 Folk medicines, 190 Follicle, 23, 37, 116, 131, 132, 134, 139, 140, 147 Follicular conjunctivitis, 50, 119, 131, 132, 136, 750 Follicular hypertrophy, 134, 148 Follicular reaction, 203 Follicular conjunctivitis, 194 Folliculosis, 119, 148 Fomites, 132 Fontanelle, 680 Foramen rotundum, 694 Forced choice preferential looking (F.P.L), 16 Forced duction test, 623, 624, 692 Forehead, 418 Foreign body, 149, 173

793
Foreign body granuloma, 49, 150, 689 Foreign body on the cornea, 97 Foreign body sensation, 195 Formation of aqueous humour, 340 Formation of peripheral anterior synechia, 354 Formed hallucination, 581 Fornices, 37, 94, 134, 150, 712 Fornix, 7, 110, 111, 112, 115, 148 Fortified, 130 Fortified antibiotic, 179, 182 Fortified aqueous drops, 192 Fortified cefazoline, 739 Fortified cepfazolin, 192 Fortified drops, 183, 193 Fortified gentamycin, 743 Fossa for lacrimal gland, 37, 86, 673 Fossa for lacrimal sac, 88, 673 Foster Kennedy syndrome, 532 Foster-Fuchs spot, 553 Four dioptre prism test, 653 Four dot iridotomy, 379, 380 Fourth nerve nucleus, 73, 599, 602, 745 Fourth ventricle, 521, 604 Fovea, 393, 394, 599 Foveal avascular zone (FAZ), 394, 415 Foveal projection, 638 Foveal reflex, 15 Foveola, 394 Fovilles syndrome, 82, 619 Fracture base of skull, 45, 118 Fracture floor of orbit, 68, 616 Framycetin, 179 Franceschettis syndrome, 39, 682 Frank corneal opacity, 19 Frans worth D-15 test, 579 Frans worth munsell 100 hue test, 579 Fransworth hue, 579 Free edge of the tentorium, 602 Free fluorescein, 414 Free iodine, 416 Friedreichs ataxia, 527 Friend test, 568 Fronds, 382 Frontal, 114, 673, 677 Frontal occulomotor centre, 504 Frontal process of maxilla, 89 Frontal sinuses, 694 Frontalis, 42 Fronto mesencephalic, 596, 601 Frontomaxillary, 36 Frontozygomatic suture, 676 Frozen orbit, 705 Fuchs coloboma, 515 Fuchs dystrophy, 169

794
Fuchs fleck, 553 Fuchs heterochromatic iritis, 240, 241, 251 Fuchs heterochromic cyclitis, 237 Fuchs heterochronic iriodcyclitis, 276 Fuchs heterochronic uveitis, 259 Fuciform aneurysm, 308 Functional amblyopia, 587 functions of aqueous humour, 340 Functions of the choroids, 231 Functions of the sclera, 722 Fundal glow, 280 Fundamental phase, 629 Fundus, 15, 89, 558 Fundus albipunctatus, 445, 575 Fundus camera, 414 Fundus changes, 245 Fundus changes consists of, 253 Fundus flavimaculatous, 443, 414, 524 Fundus in optic neuritis, 528 Fundus of the lacrimal sac, 35 Fungal culture, 179 Fungal endophthalmitis, 253 Fungal hypopyon, 190 Fungal keratitis, 203 Fungal ulcer, 175, 183, 184 Fungi, 267 Fungus, 759 Fusarium, 203 Fusion, 22, 642 Fusion reflex, 637 Fusional amplitude, 648 Fusional centre, 637 Fusional convergence, 22, 571 Fusional divergence, 648, 660 Fusional reserve, 647 Gas lasers, 383 Gas permeable lenses, 321 Gasserian ganglion, 704 Gastroenteritis, 133, 748 Gaze, 594 Gaze palsy, 619, 630 Gaze paretic nystagmus, 630 Gel, 373 General features of exotropia, 664 General features of optic neuritis, 526 General fibrosis syndrome, 609, 613, 620, 623 General information about ophthalmic laser, 381 Genetic counselling, 472 Genetic mutation, 12 Geniculate ganglion, 82, 753 Genital herpes, 53 Genital trachoma, 757 Genital virus, 754 Gentamycin, 128, 149, 179, 180, 193, 727, 730 Geographic, 195, 197, 755, German measles, 746 Germinal mutation, 472 Ghost cell glaucoma, 363, 365 Ghost vessels, 169, 171, 207, 741 Giant cell, 48, 273 Giant papillary conjunctivitis, 139 Giant tear, 449 Giardia, 764 Giardialamblia, 767 Giardiasis, 767 Giemsa, 129, 757 Giemsa stain, 127, 177, 182, 204, 209 Gingivitis, 754 Gland of Krause, 113, 152 Gland of Wolfring, 113 Glands of Moll, 38 Glands of Zies and Moll, 9, 45, 47, 102, 103 Glare, 164, 324, 550, 553, 576, 632 Glass , 324, 667 Glass particles, 700 Glaucoma, 8, 13, 24, 56, 57, 162, 163, 185, 260, 271, 277, 306, 324, 325, 339, 353, 383, 683 Glaucoma implants, 368 Glaucoma in Lowes syndrome, 353 Glaucoma in non surgical penetrating injury, 354 Glaucoma in older children, 353 Glaucoma inverse, 302, 367 Glaucoma operation, 273 Glaucoma secondary to angle recession, 365 Glaucoma secondary to anterior uveitis, 359

PEDIATRIC OPHTHALMOLOGY
Glaucoma valves, 169, 381 Glaucomatocyclitis crisis, 354 Glaucomatous disc, 370 Glaucomatous neuropathy, 363 Glaucomatous optic atrophy, 508, 532 Glaucomcyclitis crisis, 369 Glial proliferation, 445, 533 Glial tissue, 534 Glioma, 17, 475, 483, 539, 583, 691, 699, 711 Glioneuroma, 491, 492 Gliosis, 429, 524 Globe, 8, 116, 697 Globular detachment, 462 Globulin, 103 Glomerulonephritis, 739 Glossoptosis, 682 Glucose, 100, 103, 160, 299 Glutathion, 294 Glycerine, 93, 358, 364, 378, 715 Glycerol, 372 Glycolipids, 442 Glycolysis, 161 Goats, 153 Goblet cell, Krauses cells, 112 Goblet cells, 14, 35, 87, 89, 103, 110, 113, 217 Golden shower, 461 Goldenhar gorlin syndrome, 115 Goldenhars syndrome, 39, 115, 682, 683 Goldman three mirror, 245 Goldman three-mirror contact lens, 412, 460 Goldmann-favre vitreoretinal dystrophy, 435 Goldmann three mirror gonioscope, 413 Goldmann Weekers adaptometer, 418 Goldmanns tonometer, 341 Gomori methamine silver stain, 177, 204 Gonio lens, 342, 383 Gonio photo coagulation, 385 Gonio puncture, 349 Goniolens air interface, 342 Gonioplasty, 385 Goniopuncture, 381, 385 Gonioscopes, 342, 382 Gonioscopic anatomy of angle of anterior chamber, 342 Gonioscopy, 342 Goniotome, 349 Goniotomy, 349, 361, 378, 379, 380, 381 Gonococcal, 120, 126, 144, 160, 173, 175, 182, 240, 248, 258, 734

G
Gabapentin, 635 Galactocemia, 315, 317 Galactokinase deficiency, 317 Galactosaemia, 299 Galactose, 299 Galactosemic, 319 Gallium scan, 272 Gama camera, 102 Gamma crystalline, 293 Ganciclovir, 750 Ganglion cell, 5, 393, 394, 395,399, 435, 442, 510, 767 Ganglion cell layers, 397 Gangliosidosis, 443 Ganthostomiasis, 765 Gas, 381 Gas gangrene, 727 Gas in AC, 730

INDEX
Gonococci, 126, 174 Gonococci meningococci, 174 Gonorrhoea, 12 Gradenigo syndrome, 607, 620 Grades of binocular vision are, 638 Grades of nystagmus, 629 Gradient method, 660 Gram, 127 Gram negative, 192, 727, 735, 737, 743, 744, 757 Gram negative bacteria, 280 Gram negative diplococci, 733, 734 Gram negative rod, 730 Gram positive, 191, 729, 730, 735, 738, 739, 763 Gram positive cocci, 280 Gram-negative cocobacilli, 726 Grams stain, 123, 127, 182, 204, 209 Granular corneal dystrophy (Groenouws I), 211 Granuloma, 149, 701 Granulomas of conjunctiva, 121, 149 Granulomatous, 241, 263, 276, 726, 732 Granulomatous amaebic encephalitis, 766 Granulomatous angitis, 753 Granulomatous canaliculitis, 762 Granulomatous conjunctivitis, 758, 761 Granulomatous reaction, 769 Granulomatous uveitis, 241, 243, 276 Graphic method, 660 Grating acuity, 586 Gray (grey) pseudo optic atrophy, 538 Gray reflex, 514, 515 Grayson-Wilbrandt, 211 Greater circle of the iris, 228 Greater wing of sphenoid, 596, 674, 679, 680, 694 Green argon, 383 Grey line, 38 Grey reflex, 433, 451 Grocott-Gomori stain, 763 Gross pannus, 170 Ground glass, 171, 206, 273, 589, 741 Grubers (petro clinoid) ligament, 604 Guanethidine, 505 Guillain Barre syndrome, 82, 742 Gumma, 250 Gusto lacrimal reflex, 96 Guttatae, 262 Gylcerol, 378 Gyrate atrophy of choroid, 237, 419 H. influenza, 92, 126 Haabs, 24, 171, 344 Haemangioblastoma, 58 Haemangioma, 49, 92, 116, 119, 150, 565, 687, 695, 696 Haemangioma of lid, 590 Haemangiomachoroid, 701 Haemangiomas, 151, 414, 491 Haematuria, 115 Haemoglobin, 383, 384 Haemolytic anaemia, 728 Haemolytic-uremic syndrome, 730 Haemophilia, 44, 425 Haemophilus, 53, 119, 122, 124,127, 130, 702 Haemorrhages, 123, 126, 422, 428, 429, 460, 518, 552 Haidinger brush, 653 Hair bulb incubation test, 401 Hair follicles, 679 Hallerman Streiff syndrome, 678, 682, 683 Halogen, 384 Halothene, 347 Hamartoma, 55, 59, 151, 368, 399, 408, 409, 415, 425, 491, 493, 518, 712 Hand Schuller Christian, 689, 713 Harada, 259, 276 Hard contact lenses, 321, 554 Hard exudates, 424, 445 Hardy-Rand-Ritler, 579 Harelip, 679 Harmonious, 639 Harpes simplex keratitis, 53 Hay, 23, 139 Head injury, 118 Head light in the fog manner, 260 Head nodding, 632, 633 Headache, 275, 522, 571 Healed iridocyclitis, 74 Healed trachoma, 134 Heart block, 78 Heinzbodies, 365 Helium, 383 Helminth, 152, 764 Helminths, 764 Hemagglutination test, 431 Hemangio blastoma, 408 Hemangioma, 18, 22, 24, 68, 283 Hematoma, 314, 685 Hematoxylin, 209 Hemianopia, 509, 582, 584, 632 Hemiatrophy of the face, 57 Hemidesmosome, 249 Hemiparesis, 620 Hemiplegia, 82, 200, 605, 753

795
Hemivertebrae, 308, 683 Hemoglobinopathy, 454 Hemolytic anaemia, 299 Hemolytic glaucoma, 363, 364 Hemophilus influenza, 731 Hemophthalmos, 427, 464 Hemoptysis, 763 Hemorrhagic macular lesion, 764 Hemosiderin, 164, 364 Hemosiderine, 364 Henkind and seigel scotometer, 418 Henles gland, 113 Heparin coated IOL, 323 Hepatespleenomegaly, 431 Hepatitis, 299, 529, 762 Hepato lenticular degeneration (Wilsons disease), 299 Hepato spleenomegaly, 268, 317, 402, 489, 740, 747, 755, 765 Hepatolenticular degeneration, 301 Herberts, 134, 136 Herberts pits, 136 Hereditary, 210, 477 Hereditary epithelial dystrophy, 211 Hereditary juvenile epithelial dystrophy, 210 Hereditary pendular nystagmus, 630, 634 Heredity in retinoblastoma, 472 Heredo familial, 533 Heredo familial optic neuropathies, 533, 536 Heredofamilial optic atrophies, 535 Herellea vaginicola, 726 Herings law, 595 Heritable retinoblastoma, 471 Herpes , 12, 16, 23, 26, 44, 49, 50, 52, 53, 82, 96, 121, 123, 126, 127, 129, 131, 148, 170, 171, 172, 173, 174, 175, 183, 191, 195, 193, 199, 200, 206, 208, 216, 240, 243, 248, 251, 253, 258, 260, 261, 267, 429, 430, 432, 741, 745, 750, 751, 752, 753, 754 Hertels mirror proptometer, 691 Hertz, 629 Heterochromia, 74, 227, 474, 509, 511, 683 Heterochromia iridis, 27, 227, 237 Heterochromia of iris, 57, 337, 402, 479, 622 Heterochromia of the uvea, 237 Heterologous host phase, 765 Heteronymous, 583, 642, 643, 649, 660 Hexagonal keratotomy, 559 Hexamidine, 209 Hexose monophosphate shunt, 161

H
H. aegyptius, 122, 731

796
High AC/A ratio, 656 High arched palate, 308, 682 High astigmatism, 401, 563 High axial myopia, 352 High errors of refraction, 628 High hypermetropia, 412 High myopia, 461, 487, 576, 683 High-water mark, 451 Higher bacteria, 763 Highly active anti retro viral therapy (HART), 750 Hippus, 229 Hirschberg test, 652, 666 Histamine, 138, 370, 376, 506 Histocytosis, 713 Histoplasma capsulatum, 763 Histoplasmosis, 759 HIV, 430 HIV retinopathy, 749 HLA B-27, 268 Hole in the iris, 201, 337 Holes, 448 Holmgrens wool test, 579 Home atropine, 149, 173, 254, 269, 318, 506 Home atropine hydrobromide, 180, 198, 208 Home stereogram, 648 Homer-Wright rosettes, 477 Homocystinurea, 301, 306, 309, 311, 315, 464 Homocytinuria and other amino aciduria, 299 Homologous host phase, 765 Homonomous horizontal diplopia, 618 Homonymous, 583, 584, 638, 666 Hordeolum externum, 47 Hordeolum internum, 48 Horizontal cells, 396 Horizontal concomitant squint, 653 Horizontal gaze palsy, 605 Horizontal muscles, 596 Horizontal nystagmus, 401 Horizontal phorias, 644 Horizontal school, 669 Horizontally acting muscles, 593 Horner-Trantas spot, 141 Horners pupil, 511 Horners syndrome, 42, 69, 73, 237, 502, 506, 606, 612, 619, 745 Horoptor, 638 Horse shoe, 449 HOTV Test, 31 House dust mite, 140 House fly, 772 Hruby, 412, 413, 460, 518 Hue, 578 Human adeno viruses, 745 Human herpes, 193 Human immuno deficiency virus, 749 Human papilloma virus, 745 Human tetanus immunoglobulin, 729 Humoral, 138 Hurlers disease, 425 Hutchinson sign, 51 Hutchinson type, 709 Hutchinsons pupil, 510, 606 Hutchinsons salmon patch, 170 Hutchinsons sign, 200, 753 Hutchinsons teeth, 207 Hutchinsons triade, 207 Hyaline, 284 Hyaloid artery, 293, 457, 458, 512 Hyaloid capsular attachment, 457 Hyaloid system, 6, 459 Hyaluronic acid, 454, 457 Hydatid cyst, 122, 769 Hydocele, 771 Hydrocephalus, 522, 583, 613, 680, 765 Hydrocephaly, 533, 679 Hydrogel, 562 Hydrophobic, 103 Hydrops of cornea, 576 Hydrops of keratoconus, 164 Hydroxy amphetamine, 74, 506, 512 Hydroxycobalamin, 537 Hyper acute conjunctivitis, 129 Hyper braic oxygen, 484 Hyper fluorescence, 415, 443, 444 Hyper mature cataract, 277, 701 Hyper parathyroidism, 215 Hyper phoria, 644 Hyper vitaminosis A, 522 Hyperacusia, 82 Hyperbaric oxygen, 487, 728 Hyperemia, 111, 422 Hyperemia of conjunctiva, 115 Hyperlysinemia, 45, 306, 314 Hypermetropia, 163, 215, 237, 304, 342, 401, 405, 444, 466, 515, 519, 546, 555, 642, 644, 650, 685 Hypermetropic astigmatism, 519 Hypermetropic eye, 447 Hypermetropic refraction, 307 Hypermetropic shift, 451 Hypernephroma, 408 Hyperosmotic agents, 211, 372, 377 Hyperphoria, 648 Hypersecretory, 370 Hypersensitivity, 138 Hypertelorism, 40, 642, 681, 678, 695 Hypertension, 119, 257, 374, 425 Hypertensive retinopathy, 424, 434

PEDIATRIC OPHTHALMOLOGY
Hyperthyrodism, 80 Hypertropes, 68, 587, 610, 616, 633, 650 Hypertrophic scar, 445 Hyphae, 759, 760, 763 Hyphaem, 61, 208, 243, 247, 251, 248, 325, 326, 354, 384, 730 Hypo fluorescence, 274, 415, 443 Hypo phoria, 644 Hypo plasia of retina, 401 Hypo vitaminosis B, 169 Hypoangulated, 693 Hypodontia, 338 Hypoethesia of cornea, 201 Hypogenitalism, 439 Hypoglycaemia, 16, 299, 315 Hypoglycaemic cataract in infant, 301 Hypoglycemia, 767 Hypogonadism, 624 Hypoparathyroidism, 299, 301, 522, 762 Hypopituitarism, 711 Hypoplasia, 609, 621 Hypoplasia of disc, 20 Hypoplasia of macula, 8, 723 Hypoplasia of maxilla, 338, 682 Hypoplasia of optic nerve, 17 Hypoplasia of the macula, 410 Hypoplasia of the optic disc, 516 Hypoproteinaemia, 44, 118 Hypopyon, 26, 129, 130, 174, 175, 177, 178, 184, 190, 192, 196, 201, 203, 208, 241, 244, 247,248, 253, 265, 268, 271, 280, 730, 735, 736, 742, 766 Hypopyon in fungal corneal ulcer, 181, 182 Hypopyon in fungal ulcer, 181 Hyposthesia, 752 Hyposthetic cornea, 197 Hypotelorism, 678, 683, 695 Hypothalamic syndrome, 711 Hypothalamus, 73, 711 Hypothesia, 607 Hypothesia of cornea, 175 Hypothyroidism, 762 Hypotony, 171, 247, 252, 269, 272, 335, 342, 359, 453, 532, 525 Hypotropia, 68, 611, 616, 617 Hypotropic, 609, 610 Hypovitaminosis, 50, 104, 404 Hypoxia, 169 Hyppyon, 270, 277 Hysteria, 584

I
Iatrogenic, 183, 190, 281, 361, 475

INDEX
Iatrogenic causes of non healing corneal ulcers, 183 Iatrogenic conjunctivitis, 149 Iatrogenic disease, 277 Ibuprofen, 142, 257 Icthyosis, 439 Idiocy, 680 Idiopathic iridocylitis, 272 Idiopathic iritis, 269 Idiopathic optic neuritis, 525 Idoxuridine, 149, 197 Ill fitted contact, 173 Imidazoles, 205 Immature follicles, 134 Immediate hypersensitivity, 760 Immune ring, 196 Immune suppressives drugs, 270 Immuno fluorescent assays, 743 Immuno fluorescent monoclonal antibodies, 136 Immuno fluorescent stain, 209 Immuno suppression, 196 Immuno suppressive drugs, 272, 760 Immunoglobin, 49, 264, 410 Immunosuppression, 191 Immunosuppressive drugs, 78, 275 Imprint cataract, 296 Imuno potentiating drugs, 198 In flow of aqueous, 341 In KOH solution, 204 In posterior chamber, 335 Inborn errors of metabolism, 13 Incarcerated, 186 Incarcerated in the pupil, 367 Incarceration of uvea, 167, 273 Inclusion bodies, 127, 129 Inclusion body conjunctivitis in adults, 757 Inclusion body conjunctivitis of new born, 757 Inclusion conjunctivitis, 12, 127, 173 Incominant, 649, 651 Incongruous, 583 Incongruous homonymous, 509 Incontenentia pigmenti, 476, 480, 484 Increase in corneal thickness, 167 Increased corneal curvature, 25 Increased thickness of cornea, 212 Incubation of nasolacrimal duct, 91 Incubation period, 127, 134 Incyclophoria, 645, 649 Indentation tonometer, 341 Index hypermetropia, 556 Index myopia, 302, 311, 554 Index myopia curvature myopia, 548 Indirect astigmatism, 564 Indirect fluorescent anti body test, 431 Indirect gonioscope, 342 Indirect ophthalmoscope, 150, 245, 246, 408, 412, 413, 451, 551, 552, 553, 724 Indiscriminate use of steroid, 183 Indocyanine green, 276, 416 Indocyanine green angiography, 416 Indolent, 177, 208 Indolent corneal ulcer, 203 Indolent plastic iridocyclitis, 273 Indomethacin, 142, 257, 440 Induced conjunctivitis, 142 Infantile, 2, 442 Infantile botulism, 729 Infantile esotropia, 614, 616, 621, 624, 633, 639, 640, 661, 662, 663 Infantile glaucoma, 168, 344 Infantile nucleus, 292 Infarction in the optic nerve, 523 Infected bleb, 280 Infected chalazion, 53, 97 Infected dermoid, 709 Infection, 198 Infectious mono necleosis, 23, 126, 759 Infective conjunctivitis, 144 Infective iritis, 269 Infective mononucleosis, 96 Infentile glaucoma, 353 Infentile strabismus, 260 Inferior colliculus, 603 Inferior division, 602 Inferior fornix, 112 Inferior meatus, 90, 100 Inferior nasal concha, 89 Inferior oblique, 72, 395, 502, 593, 594, 597, 598, 601, 616, 674, 676, 681 Inferior oblique muscles, 675 Inferior oblique over action, 613, 615, 616, 653, 655 Inferior oblique palsy, 610 Inferior oblique under action, 42 Inferior ophthalmic vein, 398, 414, 677 Inferior orbital artery, 599 Inferior orbital fissure, 676, 677 Inferior orbital margin, 34 Inferior orbital rim, 694 Inferior osteum of the duct, 90 Inferior rectus, 72, 593, 598, 599, 601, 616, 674, 676 Inferior rectus muscle paresis, 613 Inferior rectus muscles palsy, 616 Inferior rectus palsy, 610, 615 Inferior turbinate, 101 Inflamation, 160 Inflammation of retina, 428

797
Inflammatory bowel disease, 258 Inflammatory infiltration, 188 Influenza, 132, 240, 400, 429, 741, 758 Infra orbital canal, 674 Infra orbital fissure, 674 Infra orbital foramen, 674 Infra orbital glands, 87 Infra orbital groove, 674 Infra orbital margin, 673 Infra red range, 416 Infra red sensitive video camera, 416 Infraduction, 595 Infrared, 577 Infundibular, 462 Inherited genetic, 12 Inner barrier, 423 Inner germinal layer, 769 Inner neuroblast, 399 Inner nuclear layer, 395, 396 Inner plexiform layer, 395, 397 Insect bite, 51 Insect larvae, 764 Intensity modulated, 417 Inter cavernous part, 602 Inter ferometer, 416 Inter limiting membrane of the retina, 456 Inter marginal strip, 38, 144 Inter muscles attachments, 675 Inter neural branches, 397 Inter neural part, 397 Inter nuclear ophthalmoplegia, 76, 77, 530, 605, 614, 619, 630, 634 Inter nuncial neurons, 503 Inter orbital distance, 673 Inter palpebral aperture, 34, 74, 127 Inter palpebral fissure, 53, 67, 71, 117, 210, 214, 550 Inter palpebral fissures, 14 Inter palpebral zone, 249 Inter peduncular space, 602 Inter pupillary distance, 639 Inter retinal, 493 Inter retinal haemorrhages, 425 Inter retinal separation, 447 Inter scleral nerve loop, 722, 723 Inter vaginal part, 397 Inter vitreal bleeding, 462 Inter-palpebral aperture, 41 Inter-palpebral fissure, 547 Intera ocular calcification, 283 Intercalary staphylomas, 724 Interferon, 713 Interior turbinate, 89 Intermediate host, 152, 153, 769 Intermediate uveitis, 242, 271 Intermittent cyclic esotropia, 623

798
Intermittent exotropia, 648, 659, 664, 665, 666 Intermittent heterotropia, 642 Internal carotid, 458, 512, 604, 606, 608 Internal carotid artery, 502, 705 Internal filtration procedures, 381 Internal hydrocephalus, 541 Internal limiting layer, 395 Internal limiting membrane, 279, 397 Internal ocular muscles, 78 Internal ophthalmoplegia, 510, 563, 605, 728, 730 Internal pterygoid muscle, 72 Internal temponade, 456 International colour code, 176 Interpalpebral aperture, 105 Interpalpebral fissure, 41, 51, 282 Interstitial, 206 Interstitial keratitis, 25, 26, 136, 147, 168, 190, 196, 216, 239, 262, 328, 348, 576, 732, 741, 746, 758, 767, 768 Interstitial kerato conjunctivitis, 144 Intestinal helminths, 147 Intorsion, 597, 599 Intortors, 597 Intra cameral antibiotics, 185 Intra canalicular part, 511 Intra cavernous, 602 Intra cavernous aneurysm, 697 Intra cellular, 727, 733 Intra clinoidal aneurysm, 696 Intra conal lesions, 675 Intra cranial calcification, 57, 755 Intra cranial extension, 474, 481 Intra epithelial epithelioma, 54 Intra mural part, 89 Intra ocular calcification, 474 Intra ocular foreign bodies, 283, 454, 460 Intra ocular haemorrhage, 698 Intra ocular implant, 270 Intra ocular malignancy, 241 Intra ocular mass, 697 Intra ocular pressure, 143 Intra ocular tension, 474 Intra ocular toxocariasis, 407 Intra ocular tumours, 295, 417 Intra ocular vasculature, 6 Intra operative hyphaema, 57, 351 Intra orbital canal, 694 Intra orbital nerve, 677 Intra retinal cysts, 452 Intra thorasic pressure, 117 Intra uterine factors, 12 Intra uterine ocular disorders, 12 Intra vitreal cyst, 153 Intra vitreal injection, 282 Intra vitreous, 727 Intra-ocular toxocariasis, 433 Intracapsular lens extraction, 291 Intracellular diplococci, 127 Intracerebral bleeding, 522 Intracerebral components, 628 Intraconal, 539, 769 Intraconjunctival bleeding, 118 Intracorneal ring, 554 Intracranial calcification, 260, 431 Intracranial pressure, 510 Intractable diplopia, 590 Intractable nystagmus, 316 Intraneural, 539 Intraocular calcification, 407, 696 Intraocular foreign body, 259 Intraocular growth, 116 Intraocular lens, 700 Intraocular part, 510 Intraocular pressure, 160, 161, 175, 183, 245, 341, 342 Intraocular tension, 346 Intraocular tumours, 13 Intraoperative mydriasis, 257 Intraorbital, 541 Intraorbital distance, 695 Intraorbital meningioma, 688 Intraorbital parasitic cyst, 688 Intraorbital part, 511 Intrauterine infection, 13, 168, 193 Intrauterine trauma, 352 Intravenous ocular hypotensive agents, 378 Introseous, 89 Inverse argyll robertson pupil, 509 Inverse Duanes syndrome, 608 Inverse epicanthic fold, 71, 678 inverse epicanthus, 678 Inverse Marcus Gunn jaw winking, 72 Inverse occlusion, 589 Inverse retinitis pigmentosa, 438 Inverted follicular keratosis, 54 Investigations in papilledema, 524 Iodides, 190, 198 Iodine 125, 198, 481 Iodoxuridine, 196 IOFB, 417 IOL implant, 169 IOL in children, 320 Ipsilateral, 601 Ipsilateral antagonist, 625 Ipsilateral hypertropia, 611 Ipsilateral lateral rectus, 604 Ipsilateral lateral rectus palsy, 82 Ipsilateral sixth nerve palsy, 96

PEDIATRIC OPHTHALMOLOGY
Iridectomics, 251 Iridectomy, 187, 228, 278, 284, 358, 561 Iridencleisis, 379, 381 Iridium 192, 481 Irido cyclectomy, 284 Irido dialysis, 325 Iridocorneal adhesion, 339 Iridocorneal contact, 247 Iridocorneal dysgenesis, 190, 337, 352 Iridocorneal endothelial cells, 337 Iridocyclitis, 201, 228, 239, 247, 261, 269, 326, 328, 366, 376, 502, 555, 732, 742, 746, 751, 753, 768, 772 Iridodiastasis, 234 Iridodonesis, 162, 302 Iridoplegia, 28, 125, 345 Iridotomies, 251 Iridotomy, 302, 359, 380, 381, 382, 385 Iris, 4, 15, 181, 503, 227, 624 Iris atrophy, 201, 249, 251, 277, 353, 755 Iris bombe, 244, 250, 271, 354, 360, 501 Iris border cysts, 374 Iris claw lens, 321 Iris clip IOL16, 26 Iris clip lens, 309 Iris collarette, 27 Iris fixated, 563 Iris hypoplasia, 299, 747 Iris incarceration, 169 Iris inclusion procedures, 278 Iris inclusion surgeries, 273, 350 Iris inclusion surgery, 350 Iris lens diaphragm, 186, 358 Iris mesoderm, 336 Iris neovascularisation, 493 Iris nodules, 249, 277, 284 Iris pigments, 360 Iris ruff, 227 Iris sphinter, 4 Iris stroma, 10, 233 Iritis, 208, 239, 261, 732, 742, 768, 772 Iritis glaucomatosa, 240 Irradiation, 61 Irregular AC, 189 Irregular astigmatism, 114, 168, 189, 210, 211, 345, 346, 351,564, 565, 685 Irregular myopic astigmatism, 163 Irregular pupil, 189 Irritating fluid, 279 Ischaemia, 514 Ischaemic optic neuropathy, 584 Ischamia of iris, 357 Ischemic, coagulative necrosis, 478

INDEX
Ischemic maculopathy, 267, 749 Ischemic necrosis, 201 Ischemic necrosis of iris, 52 Ishihara, 579 Isocoria, 508 Isolated sixth nerve palsy, 618 Isoniazide, 263, 526, 529 Isosorbide, 372, 378 Itching, 141 Keratanised, 111 Keratectasia, 166, 565 Keratic precipitates, 242 Keratitis, 24, 46, 50, 83, 91, 97, 172, 188, 194, 201, 208, 762, 742, 743, 744, 745, 746, 750, 758, 761, 763, 767 Keratitis in children, 206 Keratitis meta herpetica, 198, 755 Keratitis sicca, 761 Kerato acanthameba, 54, 209 Kerato conjunctival involvement, 201 Kerato conjunctivitis, 133, 205, 745, 756 Kerato conjunctivitis sicca, 104 Kerato malacia, 25 Kerato prosthesis, 144 Keratoacanthamoeba, 191 Keratocele, 169, 174, 187 Keratoconess, 25 keratoconjunctivitis, 763 Keratoconus, 142, 143, 162, 163, 166, 167, 168, 169, 171, 306, 308,335, 437, 466, 548, 554, 565, 723 Keratocytes, 167, 174, 185, 188 Keratoglobus, 26, 162, 163, 166, 314, 466, 548 Keratomalacia, 117, 184, 187, 191, 216, 217, 219, 440 Keratometer, 164, 166, 566 Keratonisation of conjunctiva, 83 Keratopathy, 134, 172, 207, 248 Keratopathy in chronic anterior uveitis, 248 Keratoplasty, 166, 172, 185, 189, 197, 203, 212, 215, 328 Keratoscope, 164, 166 Keratouveitis, 52, 239 Kerb-cycle, 161 Kertoconous, 314 Kertometer, 14 Kesten baum procedures, 635 Ketaconazole, 209 Ketamine, 347 Ketarolac, 257 Ketoconazole, 205 Ketorolac tromethamine, 140, 142 Key hole, 186, 189, 229 Khaki coloured pseudo hypopyon, 365 Kinetic, 649 Kinin system, 257 Kjers dominant juvenile neuropathy, 537 Kjers juvenile optic neuropathy, 538 Kjers optic atrophy, 535 Klebsiellapneumonae, 191 Klein keratoscope, 164 Klumkes paralysis, 73 Koch Weeks bacillus, 23 Koch-Weeks, 122, 123, 175, 731 Koeppe, 273 Koeppe lens, 366, 413 Koeppe nodules, 263 Komotos tetrad, 42, 40, 263 Koplic spots, 50, 132, 748 KPs, 271 Krabbes disease, 529, 530 Krauses gland, 87, 113 Krimsky test, 652 Krypton, 383 Krypton laser, 384

799

J
J. shaped sella, 711 Jacksonian epileptoform attack, 57 Jacksons cross cylinder, 567 Japanese, 575 Jaundice, 117, 740 Jaw winking, 43 Jerk nystagmus, 628, 629 Jews, 440 Jones I, 101 Jones I and II test, 93, 101, 176 Joules, 382 Juglar vein, 692, 704 Juvenile, 41, 238, 447 Juvenile diabetes, 179, 301, 426,427, 462, 537 Juvenile diabetes mellitus, 533 Juvenile glaucoma, 353, 370, 576 Juvenile myasthenia, 76 Juvenile or idiopathic retinoschisis, 403 Juvenile pilocytic astrocytoma, 539 Juvenile primary open angle glaucoma, 370 Juvenile Reiters syndrome, 258, 268 Juvenile retino schisis, 426, 435, 447, 465, 484 Juvenile rheumatoid arthritis, 173, 240, 252, 258, 268, 272, 328 Juvenile spondylitis, 258, 268 Juvenile xanthogranuloma, 54, 283, 284, 368, 689 Juxta canalicular meshwork, 336 Juxta papillary, 239, 259 Juxtapapillary chroiditis, 584

L
L. donovani, 768 Labyrinthine pontine path, 596, 601 Laceration of conjunctiva, 24 Lacquer cracks, 553 Lacrimal, 18, 200, 673 Lacrimal apparatus, 86 Lacrimal artery, 87, 114, 597 Lacrimal bone, 89 Lacrimal branches, 114 Lacrimal canule, 100 Lacrimal crest, 89, 973 Lacrimal drainage system, 88 Lacrimal ducts, 87 Lacrimal facia, 88 Lacrimal fistula, 100 Lacrimal gland, 7, 9, 86, 94, 96, 98, 597, 673, 675, 704 Lacrimal gland growth, 15, 692, 772 Lacrimal gland tumours, 692 Lacrimal nerve, 87, 674, 677 Lacrimal nucleus, 87 Lacrimal out flow, 101 Lacrimal passage, 7, 9, 15, 94, 154, 736, 772 Lacrimal puncta, 111 Lacrimal sac, 7, 15, 86, 88, 89, 110, 149, 191 Lacrimal sac in rhinosporidiosis, 150 Lacrimal scintilography, 93, 102 Lacrimal syringing, 93, 100, 102 Lacrimal vein, 87 Lacrimation, 60, 82, 96, 97, 106, 141, 164, 175, 178, 195, 272, 273, 347, 689 Lactic acidosis, 767 Lactoferrin, 102 Lactoferrinassay, 106 Lactophenol cotton blue, 204, 209 Lactose, 299 Lacus lacrimalis, 35, 98 Lagophthalmos, 18, 22, 36, 41, 61,67,

K
Kajal, 117, 133, 218 Kala azar, 767 Kanamycin, 730 Kandori syndrome, 435 Kaposi sarcoma, 267, 750 Kayser-Fleischer ring, 329 Kearns, 624 Keoppe nodule, 249 Keratanisation, 26, 148 Keratanisation of cornea, 148

800
81, 82, 83, 104, 111, 173, 179, 184, 684, 685, 753 Lamelar keratoplasty, 165 Lamellar, 211, 296, 297 Lamellar cataract, 16, 297, 301, 318 Lamellar corneal graft, 249 Lamellar graft, 171 Lamellar keratectomy, 205, 210, 215, 270 Lamina, 3, 534 Lamina cribrosa, 5, 510, 513, 533, 720 Lamina fusca, 721 Landolts broken C, 31 Lanellar, 189 Langerhans cell cytosis, 713 Langs syndrome, 656 Large ciliary staphyloma, 690 Large cloudy cornea, 347 Large penetrating injuries cause, 325 Larva, 432 Larval conjunctivitis, 154 Larval endophthalmitis, 153, 433 Laryngeal muscle, 76 Laryngitis, 748 Lasek, 384, 554 Laser, 61, 181, 189, 323, 349, 455, 464, 756 Laser burns, 535 Laser capsulotomy, 354, 453 Laser cyclo ablation, 381 Laser in buphthalmos, 351 Laser in glaucoma, 381 Laser in situ keratomileusis, 554 Laser iridotomy, 312, 358, 379, 380, 383 Laser phopcoagulation, 266, 273, 354, 409, 433, 447, 487, 489, 516, 764, Laser scarring to reduce, 385 Laser thermal keratoplasty, 559 Laser trabeculoplasty, 355, 356, 359 Lasik, 384, 554, 559 Lasing cavity, 382 Latanoprost, 252, 255, 355, 365, 366, 372, 373, 376 Late onset post surgical endophthalmitis, 280 Late papilledema, 523 Late retinopathy, 485 Late rosette cataract, 325 Late venous phase, 415 Latent hypermetropia, 556 Latent nystagmus, 586, 616, 629, 630, 631 Latent squint, 614, 642, 643 Lateral canthal ligament, 682 Lateral canthotomy, 715 Lateral canthus, 35, 89, 115, 420 Lateral check ligament, 676 Lateral fornix, 112 Lateral geneculate body, 513, 514, 529, 532 Lateral nasal process, 89, 677 Lateral orbital tubercle, 36 Lateral orbitotomy, 541, 676 Lateral rectus, 87, 593, 596, 618, 623, 670, 674, 745 Lateral sinus thrombosis, 522, 743 Lateral tarsorrhaphy, 83, 621 Lateral view, 693 Lateral wall, 602 Lattice, 211, 553 Lattice degeneration, 445, 449, 451, 457, 464, 487, 550 Lattice degeneration of the retina, 446 Lattice dystrophy, 211 Laurence Moon Biedl Bardet Syndrome, 438, 439, 576 Layers of retina, 395 LDH, 283 Learning difficulty, 581 Leber congenital amaurosia, 21, 419, 537, 628, 630 Lebers amaurosis, 17 Lebers disease, 537 Lebers familial amaurosis, 163 Lebers miliary aneurysm, 405, 406, 476, 477 Lebers optic atrophy, 535 Lebers optic neuritis, 526, 527, 537, 538 Legal blindness, 410 Leishmania, 764, 767 Lens, 9, 15, 503, 546 Lens capsule, 2, 235 Lens epithelium, 291 Lens extraction, 273, 315 Lens fibbers, 291 Lens in the sulcus, 309 Lens induced uveitis, 277, 683 Lens iris diaphragm, 56 Lens pit, 2 Lens plate, 1, 2, 293 Lens protein, 277 Lens suture, 292 Lens vesicle, 2, 161, 291, 293 Lensectomy, 28, 272, 320, 488, 683 Lenticonus, 31, 302, 548, 554, 564 Lenticular, 483 Lenticular changes in chronic anterior uveitis, 252 Lenticular metabolism, 330 Lenticular opacity, 585 Lentiglobus, 302 Lepromatous nodules, 249

PEDIATRIC OPHTHALMOLOGY
Leprosy, 62, 81, 121, 171, 172, 173, 206, 240, 258 Leprotic lagophthalmos, 62 Leptospira, 740 Leptospiracmia, 742 Leptospirosis, 258, 741 Lesser circle of the iris, 228 Lesser wing of sphenoid, 36, 673, 694 Lettercrswie disease, 713 Leucokoria, 473, 489, 515 Leucoma, 185, 186, 188 Leucoma adherence, 127, 174, 175, 185, 186, 188 Leucomatous, 174 Leucoria, 483 Leucotriens, 138 Leucovorin, 261 Leuddes transparent scale, 691 Leukaemia, 24, 44, 119, 283, 368, 422, 424, 425, 463, 476, 477, 483, 491, 522, 542, 675, 686, 689, 690, 698, 706, 707, 708, 709, 762 Leukaemia deposit in orbit in children, 711 Leukaemic deposits, 708 Leukaemic infiltration, 705 Leukodystrophies, 530 Levamesol, 198 Levator, 4, 597, 600 Levator aponeurosis, 675 Levator function, 79 Levator palpebral muscle, 675 Levator palpebral superior, 35, 42, 67, 609, 676, 697 Fevator resection, 78 Levo cyclo version, 595 Levo version, 595, 629 Levobunotol, 375 Levodopa, 591 Lid, 44, 86, 153, 154, 702, 738 Lid abscess, 68, 744 Lid deformity, 179 Lid fatigue, 76 Lid hygiene, 147 Lid infiltration, 479 Lid lag, 42, 74, 80 Lid margin, 101 Lid of deformity, 183 Lid retraction, 19, 20, 23, 67, 80, 127, 374, 375, 509, 614, 681, 690 Lid vescicles, 194 Ligament of lockwood, 598 Ligenous conjunctiva, 149 Light adapted trough, 420 Light brightness, 520 Light bulb, 407 Light near dissociation, 509, 511, 614 Light reflex, 18, 81, 503, 601

INDEX
Ligneous conjunctivitis, 126, 149 Limbal, 141 Limbal blood vessels, 160 Limbal changes in trachoma, 136 Limbal dermoid, 12, 19, 25, 121, 165, 168, 190, 565, 679 Limbal papillae, 120, 166 Limbal vasculitis, 196 Limbitis, 208 Limbus, 3, 25, 105, 110, 111, 114, 144, 159, 171, 273, 336, 676, 720 Limbus parallel tears, 449 Lindane, 46 Liner accelerator external beam radiation, 481 Lingua plicata, 83 Lingual palsy, 62 Lipid, 98, 216 Lipid abnormality, 103 Lipid degeneration, 201 Lipid fraction, 144 Lipid layer, 102 Lipid layer of the tear, 37 Lipidosis, 423, 439 Lipofucin, 416, 443, 444 Lipoidal ultra fluid, 102 Lipoprotein, 103 Liquefaction, 252 Liquefaction of vitreous, 449, 553 Liquid, 381 Lisch nodule, 710 Listers perimeter, 652 Live worm, 154 Liver, 153, 769 Loa-Loa, 765, 772 Loasis, 765, 772 Local anaesthetic, 191 Local retinol, 220 Local steroids, 256 Lock jaw, 728 Lockwood ligament, 599, 676 Long acting steroids, 275 Long acting sulpha, 47 Long ciliary nerves, 73, 502, 511 Long posterior ciliary arteries, 232 Long term drift, 376 Long term effect of extra ocular muscle palsy, 624 Longitudinal fibres, 230 Loss of AC, 174 Loss of accommodation, 255, 273 Loss of foveal reflex, 444 Loss of sensation, 179, 183 Louis bar syndrome, 55 Low IQ, 439 Low vision aids, 238, 538 Lower division of the oculomotor nerve, 593 Lower formix, 56, 110, 119 Lower puncta, 35, 88, 100 Lower tarsal conjunctiva, 119 Lowes syndrome, 301, 315, 352 LPS, 74 Lridocorneal dysgenesis, 212 Lrisatrophy, 212 Lubricants, 195, 211 Lubricating drop, 211 Lumbo peritoneal shunt, 525 Lumigan, 372 Lung, 153, 731, 769 Lyme disease, 82, 253, 258, 740, 742 Lymphadenitis, 733 Lymphadenopathy, 123, 131, 132, 194, 268, 402, 740 Lymphangioma, 687, 688, 690, 698, 712 Lymphangitis, 764 Lymphatic, 151 Lymphatic cyst, 122, 152 Lympho granuloma, 144 Lympho granuloma venerum, 133 Lympho sarcoma, 709 Lymphoblastic leukaemia, 711 Lymphocytes, 121, 228, 276 Lymphogranuloma venereum, 206, 758 Lymphoid tissue, 113 Lymphokinase, 257 Lymphoma, 402, 607, 762 Lypophilic, 375 Lysinedehydrogenase, 314 Lysosmal enzyme, 257 Lysosomes, 102, 110, 180, 395 Lysozymeassay, 106

801
Macules, 148 Maculo papular, 200, 268 Maculopapillar bundle, 246, 247 Maculopathies, 668 Maculopathy, 375, 416, 747 Madarosis, 45, 200, 708, 762 Maddox double prism, 645, 646, 649 Maddox rod test, 643, 645 Maddox tangent, 645 Maddox wing, 644, 645, 646, 648 Maggots, 154 Magnetic resonance imaging, 698 Magnification, 560, 562, 577 Magsulf, 93 Main elevator, 67 Main lacrimal gland, 103 Major arterial circle, 232 Major circle of iris, 232 Mal-absorption syndrome, 219 Malaise, 747 Malaria, 189, 206, 219, 766 Malignant epithelial tumours, 87 Malignant glaucoma, 357 Malignant glioblastoma, 710 Malignant hypertension, 425 Malignant melanoma, 59, 283, 382, 415 Malingeing, 32, 421, 584, 632 Malnutrition, 144, 179, 278, 576 Management of accommodative esotropia, 660 Management of acute dacryocystitis, 92 Management of aphakia in children, 561 Management of ARC, 640 Management of chalazia, 48 Management of glaucoma in penetrating injury, 355 Management of glaucoma secondary to total hyphaema, 364 Management of herpes zoster ophthalmicus, 202 Management of hypopyon corneal ulcer, 181 Management of impending perforation, 184 Management of infective corneal ulcer, 176 Management of nystagmus, 634 Management of optic neuritis, 528 Management of primary buphthalmos, 348 Management of retinopathy of prematurity, 487 Management of rhegmatogenous retinal treatment, 455 Management of stye, 47

M
Macro-phages, 181, 242, 243, 364, 367 Macropsia, 577 Macrostomia, 682 Macula, 5, 15, 185, 188, 231, 246, 259, 394, 401, 431, 490 Macular, 174 Macular coloboma, 410, 535 Macular cyst, 416, 442 Macular dystrophy, 212, 420 Macular edema, 242, 253, 269, 271, 416 Macular granuloma, 433 Macular hole, 247, 416, 425, 442, 449 Macular hypoplasia, 115, 628, 630 Macular lesion, 577, 634, 650 Macular opacities, 186 Macular pigmentation papillitis, 253 Macular scar, 247, 630, 665 Macular star, 58, 409, 424, 493

802
Management of trachoma in children, 136 Management of traumatic cataract, 327 Management of uveitis, 253 Managementof xerodermapigmentosum, 60 Mandibulo facial, 13, 18, 38 Mandibulo facial dysostosis, 306, 682 Mangrove fly, 772 Manifest hypermetropia, 556 Manifest latent, 630 Manifest latent nystagmus, 633, 664 Manifest nystagmus, 615, 633, 664 Manifest squint, 569, 638, 640, 649 Mannitol, 151, 351, 358, 364, 525, 372, 715 Mannitol and urea, 378 Manosidosis, 301 Mantoux test, 264 Manz, 87 Marchesani syndrome, 306 Marcus Gunn, 28, 43, 72, 43, 450, 507, 508, 684, 686, 690, Marcus Gunn jaw winking, 72 Marcus Gunn phenomenon, 43, 690 Marcus Gunn pupil, 450, 507, 508, 684, 686 Marfans, 311, 453 Marfans syndrome, 306, 307, 309, 446, 452, 464, 560 Marginal conjunctiva, 111 Marginal corneal phlycten, 146 Marginal keratitis, 123 Marginal corneal ulcers, 178 Masquerade, 241 Masquerade syndrome, 259, 283, 481 Mass reflex, 504 Massaging of the sac, 91 Mast cell inhibitor, 140, 142 Mast cell membrane, 138 Mast cells, 228 Mastcell stabiliser, 328 Mastoid, 620, 705, 743, Mastoiditis, 743 Maternal diabetes, 315, 487 Maternal infection, 295 Maternal malnutrition, 315 Maternal rubella, 315 Maternal syphilis, 295 Maxilla, 89, 673, 674, 709 Maxillary antrum, 674 Maxillary artery, 87 Maxillary division, 200 Maxillary nerves, 602 Maxillary process, 89, 677 Maxillary sinus, 673, 694 Maxillo mandibular condensation, 600 Maxwell Lyons sign, 141 McCoy cells, 134, 136 Measles, 44, 47, 49, 87, 93, 106, 123, 132, 179, 189, 217, 219, 240, 529, 576, 745, 747 Measles vaccine, 219 Measurement of intraocular pressure, 341 Measurement of phoria for near, 646 Measurement of proptosis, 690 Measuring intra ocular pressure, 26 Mebendazol, 153 Mechanical capsulotomy, 278 Mechanical causes, 62, 81 Mechanical ptosis, 60 Medial, 601, 673 Medial canthal ligament, 35, 101 Medial canthus, 35, 88, 92, 112, 152, 420 Medial check ligament, 676 Medial fornix, 112 Medial long posterior ciliary artery, 6 Medial longitudinal fascicles, 605, 619 Medial longitudinal fibres, 604 Medial meatus, 89 Medial palpebral ligament, 89, 150 Medial rectus, 72, 504, 593, 596, 610, 623, 670, 676 Mediastinum, 77 Medical treatment, 60, 249 Medium, 382 Medrysone, 361 Medulated, 514 Medulated nerve fibers, 5 Medulla epithelioma, 480 Medullary, 59 Medullo epithelioma, 259, 283, 284, 476, 483, 491 Medulo blastomas, 478 Meesmanns, 210 Megahertz, 416 Megalo cornea, 18, 25, 159, 161, 162, 166, 305, 308, 314, 321, 335, 549, 552 Megalo papilla, 513 Meibomian, 37, 104 Meibomian cell carcinoma, 54 Meibomian gland, 7, 9, 37, 40, 48, 102, 104, 115 Meibomianitis, 104, 730 Melanin, 383, 384, 395, 416 Melano phores, 112 Melanocyte,4, 228, 278, 401, 683, 722 Melanocytoma, 542 Melanosis bulbae, 353

PEDIATRIC OPHTHALMOLOGY
Melanosomes, 401 Melanotic tumours of conjunctiva, 117 Melkerrson Rosenthal syndrome, 81, 83 Mellar corneal graft, 210 Membrane formation over macula, 247 Membranous cataract, 298, 488 Membranous conjunctivitis, 63, 759 Meninges, 56, 154, 511, 674 Meningiococcus, 23, 160, 173, 175, 734 Meningioma, 276, 539, 675, 687 Meningitis, 97, 522, 526, 576, 703, 726, 731, 736, 742, 743, 744, 752, 770 Meningo encephalitis, 526, 753 Meningo encephalocele, 678, 679, 687, 688, 691 Meningocele, 92, 678, 687, 688, 692 Mental retardation, 309, 330, 402, 439, 681 Mercuro chrome, 101 Meridional amblyopia, 586 Mesenchymal, 485, 533 Mesoderm, 10, 19, 26, 27, 88, 161, 233, 305, 337, 338, 352, 458, 539, 677, 722, Mesopic, 418 Mesopic vision, 586 Metabolic cataract, 316 Metabolism, 160 Metachromatic leuco dystrophy, 443 Metaherpatic keratitis, 195, 199 Metamorphopsia, 246, 276, 411, 423, 450, 516, 574, 577, 764 Metastasis, 278, 473 Metastatic endophthalmitis, 476, 730, 760 Metastatic neuroblastoma, 705, 706 Metastatic retinitis, 476, 483 Metastatic uveitis, 735 Metazoa, 764 Methacholine, 511 Methazolamide, 377 Methicillin, 184, 738 Methionine, 309 Metho trex ate, 706 Methonine, 311 Methotrexate, 258, 482, 709 Methyl prednisolone, 536 Metiprantol, 375 Metronidazole, 526, 729 Meyers loop, 583 Miconazole, 205 Micro aneurysm, 408, 493, 749 Micro chondrian mutation, 624 Micro cornea, 26, 556, 723

INDEX
Micro dendrile, 194 Micro dendritic ulcer, 195 Micro erosion, 141 Micro filarae, 771 Micro pannus, 127, 170 Micro strabismus, 587 Micro surgical extra capsular cataract extraction, 322 Micro-aneurysms, 409 Micro-cornea, 163, 335 Micro-dontia, 338 Micro-ophthalmos, 335 Micro-strabismus, 663 Microaneurysm, 267, 485 Microbial conjunctivitis, 122 Microblepharon, 41 Microcephally, 19, 25, 27, 159, 166, 260, 314, 558, 680, 747, 755, Microdendrites filamentary keratitis, 51 Microfilaria, 772 Micrognathia, 682, 683 Microphakia, 162, 367 Microphthalmia, 235, 403, 487, 678, 683, 687, 688 Microphthalmos, 8, 12, 16, 18, 19, 25, 26, 27, 39, 68, 115, 162, 163, 166, 299, 316, 339, 352, 353, 437, 457, 549, 556, 558, 622, 690, 696, 722, 747 Microphthalmos with cyst, 8, 9, 696, 722 Microphtlamia, 679 Micropsia, 577 Microspherophakia, 29, 301, 302, 307, 311, 314, 330 Microtropia, 641, 654, 658, 663 Microvilli, 103, 159 Microvilli of retinal pigment epithelium, 447 Mid brain, 503, 521, 602 Mid brain lesions, 73 Mid limbal line, 112, 336 Mid pons, 604 Mid venous phase, 415 Middle cerebral, 584 Middle cranial fossa, 512, 673, 674, 677, 694 Middle meatus, 674 Midline facial defects, 513 Migraine, 608 Mikuliczs syndrome, 87 Mild ptosis, 79 Miliary aneurysm, 406 Miliary tubercle, 263 Miliary tubercle of choroids, 263 Miliary tuberculosis, 732 Milk alkali syndrome, 215 Milk spilled over cobble stone, 141 Millard Gubler, 82, 619 Millers syndrome, 235 Mineral oils, 370 Miners nystagmus, 628, 630, 631, 633 Miniature toy test, 31 Minification, 577 Minocylin, 261 Minor circle of iris, 232 Minus lenses, 503 Miosis, 74, 186, 257, 262, 353, 373, 503, 608, 668, 735 Miotic and cyclotonic, 505 Miotic pupil, 229 Miotics, 229, 251, 255, 270, 273, 355, 365, 371, 373, 505, 555, 657, 660, 661 Mirror image, 304, 307 Missed foreign bodies in cornea, 183 Mitochondrial DNA, 537 Mitomycin C, 356, 366 Mitral valve prolapse, 163 Mitten drofs dot, 488 Mittendorf dot, 295, 296 Mittendrof s spot, 6 Mixed astigmatism, 565 Mixed nystagmus, 629 Mizuo phenomenon, 575 Mizuo sign, 575 Mobius syndrome, 20, 62, 81, 83, 620, 621, 624, 656 Moderate, 79 Moll, 47 Mollouscum contagiosum, 50, 123, 132, 172, 745, 756, Molluscum, 44, 49, 173 Molteno valve, 338 Mongoloid obliquity, 41 Mongoloid slant, 670 Mono filament sutures, 144 Mono fixation syndrome, 663 Mono nucleosis, 756 Mono-ocular, 649 Monochromatism, 578 Monocular diplopia, 324 Monocular esotropia, 641 Moorens ulcer, 206 Morax-Axenfeld, 735 Moraxella, 735 Moraxella catarrhalis, 735 Moraxella lacunata, 735 Morbilli, 49 Morbilli virus, 748 Morning glory syndrome, 513, 515 Morphine, 506 Morphological changes in cornea, 166

803
Morphology, 165, 628 Motile, 740, 744 Motor imbalance, 629 Motor nystagmus, 587 Motor paralysis, 745 Movements of the eyes, 14 MRI, 416, 698 MRI induced blindness, 327 Mucin, 87, 98, 216, 217 Mucin deficiency, 103 Mucin layer, 102 Mucinous degeneration, 540 Mucinous discharge, 120 Muco cutaneous, 148 Muco purulent conjunctivitis, 148 Muco purulent discharge, 120, 123 Mucocele, 93, 675, 692, 696 Mucoid degeneration, 535 Mucolipidoses and cystinosis, 13, 25 Mucolipidosis, 168, 348, 741 Mucolytic, 106 Mucopoly saccharidoses, 19, 25, 168, 190, 216, 348, 352, 425, 439, 533, 721, 741 Mucopuralent, 91, 180 Mucopurulent conjunctivitis, 175, 177, 191, 192, 731, 758 Mucopurulent discharge, 24, 90 Mucorale, 762 Mucormycosis, 759, 762 Mucous, 176 Mucous plaque, 201 Mucus discharge, 95 Mucus plaque, 52 Mucus secreting conjunctivitis, 52 Mulberry molars, 740 Muller cells, 396 Mullers, 74 Mullers cell, 418 Mullers fibre, 396, 399 Mullers muscle, 8, 35, 36, 67, 76, 80, 81, 374, 511 Multi focal choroiditis, 750 Multicentric, 479 Multiceps, 153 Multifocals, 563 Multiple phlycten, 136 Multiple sclerosis, 283, 528, 529, 668, 699 Multiple styes, 53 Mumps, 87, 96, 132, 240, 745 Mumps influenza, 295 Mums, 529 Munsons sign, 164, 166 Muscae volitantes, 461, 551 Muscle cone, 511, 599, 675 Muscle dystrophies, 75

804
Muscle imbalance, 558, 570, 581 Muscle plane, 593 Muscles of face, 76 Muscles of mastification, 76 Muscular anomaly, 644 Muscular arteries, 232 Muscular dystrophies, 69 Mustard oil, 369 Mutation in mitochondrial DNA, 78 Mutation of genes, 436 Mutton fat, 241, 243, 249, 263, 742 Myasthenia, 69, 79, 82, 621, 624 Myasthenia gravis, 75, 505, 613 Myasthenia in mother, 16 Myasthenia of childhood, 76 Mycelia, 761 Mycelium, 759, 760 Mycetoma, 763 Mycobacteria, 267, 726, 750 Mycobacterium tuberculosis, 731, 750 Mycoplasma pneumonae, 148 Mydriasis, 257, 366, 374, 375, 552, 608, 735 Mydriatic, 180, 502, 505, 730 Mydriatic pupil, 229 Mydriatic cycloplegic, 503 Mydriatics, 229, 254 Mydricane, 255 Myelinated, 513 Myelinated nerve fibres, 514 Myeline sheath, 533 Myelitis, 97, 530 Myelogenous leukemia, 283 Myiasis, 725, 765 Myiasis externa, 773 Myiasis interna, 773 Myocardial infarction, 310 Myocarditis, 730, 742, 744, 758 Myogenic, 75 Myographic, 621 Myoneural junction, 75 Myopathies, 75, 276 Myopia, 162, 242, 248, 335, 342, 401, 404, 410, 423, 427, 436, 437, 443, 452, 515, 532, 575, 576, 644, 650, 712, 723 Myopia of prematurity, 555 Myopic, 159, 304, 546 Myopic astigmatism, 42, 371 Myopic crescent, 552 Myopic degeneration, 305, 415 Myopicaphakics, 452 Myopicpseudophakics, 452 Myositis, 705, 706 Myotonic dystrophy, 77, 330, 439, 624 Myxedema, 44, 118

PEDIATRIC OPHTHALMOLOGY

N
N. meningitis, 122 N.D. YAG, 324 Naevus, 45 Naefus flammeus, 54 Nagels anomaloscope, 579 Nalidoxic acid, 522 Nanophthalmos, 8, 162, 163, 166, 352, 556, 558722, 723 Nanophthalmos, 8 Nasal, 114 Nasal cavity, 694 Nasal cord, 90 Nasal endoscopic surgery, 151 Nasal mucous, 97, 106 Nasal veins, 89 Naso lacrimal, 100 Naso lacrimalduct, 7, 34, 86, 88, 89 90, 92, 94, 98, 101, 110, 176, 673 Naso pharyngeal carcinoma, 607 Nasociliary, 73, 114, 200 Nasociliary nerve, 51 201, 502, 511, 674, 675, 677, 753 Nasolacrimal duct obstruction, 279 Nasopharyngeal, 620 Nasopharyngeal carcinoma, 759 Nasopharynx, 93, 110, 125, 149, 150 Natamycin, 205 Nathnagels syndrome, 606 National programme of prevention of xerophthalmia, 219 Nausea, 77, 522 Nd-YAG, 61, 382, 383, 385 Nd-YAG cyclophto coagulation, 369 Nd-YAG posterior capsulotomy, 359 Near dysfunction, 649 Near reflex, 503, 504 Near sightedness, 547 Near synkinesis, 503 Near vision, 589 Nebula, 174, 185, 188 Nebular opacities, 185 Neck, 89 Necrosis of the retina, 750 Necrotising, 196 Necrotising retinitis, 429, 766 Necrotising retino choroiditis, 430 Nedocromil, 140 Nedocromil sodium, 142 Needing, 291, 452 Needling aspiration, 322 Neisseria catarrhalis, 735 Neisseria gonorrhoea, 122, 733 Neisseriaceae, 726 Neisseriae meningitidis, 734 Neissericeae, 129 Nematode, 154, 772

Nematode infection, 430 Nematode onchocerca volvulus, 771 Nematode toxocara, 770 Nematodes, 764 Nemesulide, 142 Neo natal herpes simplex, 53 Neo vascularisation of iris, 251 Neodymium, 382 Neodymium Yuttrium Aluminium Garnet, 383 Neomycin, 124, 149, 179, 209 Neon, 383 Neonatal, 728 Neonatal chlamydiae disease, 133 Neonatal conjunctivitis, 127 neonatal dacryocystitis, 90 Neonatal gonococcal infection, 733 Neonatal HSV, 198 Neonatal hypoxia, 765 Neonatal infection, 13 Neonatal keratitis, 348 Neonatal myasthenia, 42, 71, 75 Neonatal nasolacrimal duct obstruction, 23 Neonatal sepsis, 739 Neonatal trachoma, 757 Neonate, 130, 133, 168, 538 Neostigmine, 505 Neovascular glaucoma, 354, 368, 488 Neovascular tuft, 485 Neovascularisation, 228, 247, 249, 251, 256, 357, 368, 406, 415, 422, 433, 474, 479, 488, 742 Nephritis, 44, 425 Nephrotic syndrome, 118 Nerve fibre layer, 395, 397 Nerve fibre layers of the retina, 431 Nerve to inferior oblique, 503 Nervous system involvement, 56 Neural control of dilator pupillae, 502 Neural control of ocular movements, 595 Neural control of constrictor pupillae, 502 Neural crest, 401 Neural ectoderm, 9 Neural fold, 1 Neural groove, 1 Neural plate, 1 Neural tube, 1, 398, 601 Neuritis, 415, 518, 519, 530 Neuro cysticercosis, 770 Neuro ectodermal, 227, 398 Neuro fibroma, 283 Neuro fibromatosis, 45, 151, 685, 691, 695, 696, 697 Neuro imaging, 528

INDEX
Neuro muscular, 75 Neuro neuritis, 529 Neuro retinitis, 421, 423, 428, 525, 532 Neuro sensory deafness, 439 Neuro tropic keratitis, 52, 104, 107 Neuro-syphilis, 750 Neuroblast, 601 Neuroblastoma, 44, 477, 478, 480, 512, 542, 686, 687, 688, 689, 690, 703, 707, 708 Neuroectoderm, 4, 227 Neurofibreomatosis, 54, 163, 283, 352, 539, 542, 710 Neurofibroma, 68, 565, 687, 688, 690, 696, 697, 698, 707 Neurogenic cause of lagophthalmos, 61 Neuroglia, 28, 518, 539, 613 Neurological involvement in herpes zoster ophthal, 202 Neurological characteristics of fourth nerve palsy, 611 Neurological lesions, 52 Neurological-internal ophthalmoplegia, 32 Neuromyelitis optica, 528 Neuropathy, 28 Neuroretinitis, 526, 534 Neurosyphilis, 509, 668 Neurotic, 584 Neurotoxin, 728 Neurotrophic, 104, 175, 184, 216 Neutral density filter, 507, 586, 588 Nevi, 284 Nevus of ota, 352, 353 Nevusflammeus, 60, 61 New born cornea, 159 New born eyes, 14 New vessels, 342 Nictitating membrane, 112 Niemann-pick disease, 425, 442 Night blindness, 218, 401, 436, 440, 439, 465, 551, 574, 575 Night myopia, 555 night vision, 217, 373, 394 Nimesulide, 257 Nitrofurantoin, 730 Nocardia, 759, 763 Nocardia asteroides, 763 Nocardiosis, 760, 763 Nocturnal lagophthalmos, 81 Nodal point, 560 Nodular papillae, 141 Nodule, 147, 241, 249, 474 Nodule formation, 764 Nodule on the iris, 337, 479 Nodules at limbus, 147 Nodules of sarcoid, 249 Non accommodative acquired esotropias, 662 Non accommodative esotropia, 614, 658 Non atopic, 138 Non cataractous white reflex, 13 Non comitant, 643, 649 Non conjugate, 14 Non fenestrated endothelium cells, 398 Non filamentous, 203 Non fluorescent, 424, 425 Non granulomatous, 201, 261, 263, 269, 271, 329, 738 Non healing corneal ulcer, 181 Non keratanised, 161 Non lactose fermenting, 743 Non motile, 738, 739 Non nucleated marginal zone, 399 Non pigmented coloboma, 410 Non pigmented epithelium, 334, 393 Non rhegmatogenous, 448, 453, 492, 515, Non sporulating, 738, 739 Non steroidal anti inflammatory drugs, 142, 257, 362 Non steroidal anti-inflammatory, 362 Non teratoid, 284 Non traumatic phacogenic glaucoma, 366 Non-fenestrated, 228 Non-gonococcal ophthalmia neonatorum, 194 Non-granulomatous, 52, 241, 276, 750 Non-motile, 727 Non-myelinated, 511 Non-necrotising, 196 Non-pigmented ciliary epithelium, 233 Non-septate, 762 Nongonococcal ophthalmia neonatorum, 133 Nongranulomatous pan uveitis, 267 Nontreponemal, 262 Nor adrenaline, 506 Nor epinephrine, 502 Normal macula in fluorescein angiogram, 415 Normal retinal correspondence, 640 Norries disease, 476 Nose bridge, 678 Nosocomial, 737, 839 Notch coloboma, 233 Notching of lid margin, 51 Notching of the cup, 371

805
Notifiable disease, 126 NSAID, 140 Nuclear, 295 Nuclear sclerosis, 701 Nuclear-fascicular lesion, 612 Nuclei, 2 Nucleic acid, 744 Nucleus, 292, 547 Nucleus of the lens, 292 Null point, 629 Null zone, 401, 631 Nummular keratitis, 51, 201, 727 Nutrition of cornea, 160 Nutritional amblyopia, 586 Nyctalopia, 574, 575 Nystagmoid movements, 76 Nystagmus, 8, 16, 20, 21, 29, 32, 168, 172, 178, 185, 186, 189, 234, 236, 237, 260, 316, 319, 323, 402, 410, 420, 439, 441, 442, 465, 513, 517, 538, 541, 549, 563, 575, 580, 596,628, 632, 656, 666, 680, 681, 683, 723, 734, 745, 747, 766 Nystagmus blockage syndrome, 621, 633 Nystagmus blocking esotropia, 656, 663, 664 Nystatin, 205

O
Obesity, 541, 711 Object of regard, 594 Objective angle of deviation, 639 Objective angle of squint, 639 Obligate aerobe, 192 Obligate intra cellular, 744, 757 Obligatory intra cellular, 765 Obligatory suppression, 641 Oblique, 4, 164, 593 Oblique astigmatism, 564 Oblique axis, 164 Oblique illumination, 164, 307, 326 Oblique muscle, 598, 615 Oblique orbital line, 694 Oblique school, 669 Obliteration of canal of Schlemm, 354 Obliteration of lacrimal ducts, 148 Occipital cortex, 17, 504, 637 Occipital lobe, 584 Occipital lymph glands, 298 Occipito cortical, 584 Occipito mesencephalic path, 601 Occiput, 421 Occlusio pupillae, 244, 251, 354 Occlusion, 588 Occlusion amblyopia in better eye, 590

806
Occuloplastic surgeries, 384 Occulsio pupilli, 252, 476 Occult retinoblastoma, 351 Occuserts, 182 Ocular, 236 Ocular albinism, 401, 402 Ocular changes, 307 Ocular emergency, 126 Ocular fixation nystagmus, 631 Ocular hypotensive, 376 Ocular involvement, 56, 57, 58, 59, 149 Ocular management, 49 Ocular manifestation of systemic virus disease, 744 Ocular myiasis, 154, 773 Ocular myopathy, 78 Ocular nystagmus, 630 Ocular pemphigoid, 104, 120 Ocular posture, 628 Ocular prosthesis, 144 Ocular surface, 102 Ocular surface disorder, 96, 376 Ocular symptoms of myasthenia gravis, 75 Ocular torticolis, 611 Ocular toxoariasis, 264, 476, 489 Ocular ultrasonography, 326, 358, 464 Oculo auriculo vertibral syndrome, 683 Oculo cutaneous, 236, 400 Oculo motor, 600, 601 Oculo sporidiosis, 149, 724 Oculo sympathetic, 74, 612 Oculohypotensive, 377 Oculomotor, 38, 43, 228, 510, 511, 593, 600, 601 Oculomotor palsy, 73 Oculomotor ptosis, 73 Oculosporidiosis, 121 Oculosympathetic, 73, 510, 606 Ocuserts, 373 Oedema, 324 Ofloxacilin, 179, 737 Oguchis, 419, 574, 575 Oil drop, 303 Oil dropelet cataract, 300 Ointment, 256, 373 Old retro bulbar neuritis, 535 Oligodendro glioma, 710 Oligodocytes, 710 Onchocerciasis, 152, 206, 768, 771 Onchocercomata, 771 Onchocerea caecutiens, 771 Onchocerea volvulus, 765 Oncotic pressure gradient, 448 One and half syndrome, 605 Oocytes, 259 Oophoritis, 746 Opacification of cornea, 148 Opacity, 315 Opacity in media, 650 Opaque contact lens, 589 Opaque nerve fibers, 56, 399, 423, 476, 513, 514, 584 Open angle glaucoma, 308 Operating gonioscope, 349 Operating microscope, 349, 382 Operculated hole, 449 Ophthal micartery, 89 Ophthalmia neonatorum, 117, 122, 123, 126, 129, 168, 173, 190 Ophthalmia nodosa, 121 Ophthalmia nodosum, 149 Ophthalmianeonatorum, 18 Ophthalmic, 87, 602 Ophthalmic artery, 87, 152, 231, 293, 397, 405, 511, 599, 674, 675, 677 Ophthalmic neonatorum, 90 Ophthalmic veins, 231, 511 Ophthalmoplegia, 78, 314, 698 Ophthalmoplegia plus, 78, 624 Ophthalmoplegic migraine, 607, 608 Ophthalmoscope, 171, 326, 382 Opportunistic, 192, 279, 727, 762, 766 Opportunistic infection, 749 Opportunistic organism, 173, 492, 726 Opportunistic systemic infection, 260 Opthalmic vein, 596 Optic abiotrophies, 536 Optic atrophy, 508, 514, 520, 523, 532, 540, 650, 665, 678, 680, 681, 684, 685, 686, 742, 767 Optic canal, 511, 540, 694, 697 Optic chiasma, 512, 699 Optic cup, 1, 228, 233, 393, 395, 398, 447, 512 Optic disc, 15, 394, 415 Optic foramen, 597, 674, 677, 694 Optic gliomas, 56 Optic nerve, 51, 56, 230, 246, 393, 395, 456, 507, 530, 675, 676, 677, 680, 697, 720 Optic nerve anomaly, 115 Optic nerve glioma, 532, 539, 676, 687, 688, 689, 695, 696, 697, 698, 707 Optic nerve head, 246, 401, 408, 510 Optic nerve hypolplasia congenital cone dystrophy, 21 Optic nerve hypoplasia, 628, 630 Optic nerve sheath-complex, 699 Optic nerve sheathmeningioma, 701

PEDIATRIC OPHTHALMOLOGY
Optic neuritis, 52, 96, 202, 203, 245, 423, 429, 508, 520, 530, 698, 703, 706, 727, 742, 746, 748, 750, 753, 759, 767 Optic neuropathies, 481, 668, 690, 699 Optic pathway, 577 Optic pit, 1 Optic radiation, 504, 529, 530, 580 Optic stalk, 5, 233, 512 Optic tract syndrome, 584 Optic vesicle, 1, 233, 293 Optical, 159, 160 Optical coherence tomography, 412, 416 Optical iridectomy, 189 Optical zone, 159 Optically elicited movements, 17 Opticcup, 293 Optico kinetic drum, 632 Opticokinetic, 632 Optics of aphakia, 560 Opto kinetic nystagmus, 629 Optociliary shunts, 686 Optokinetic, 596, 632 Optokinetic drum, 30 Optokinetic drum of harcourt, 29 Optokinetic nystagmus, 630, 631 Optokinetic target, 16 Optotypes, 21, 586 Ora serrata, 229, 230, 393, 448, 456 Oral acetazolamide, 205, 349, 367 Oral bays, 393 Oral diamox, 151 Oral steroid, 78 Oral thrush, 762 Orange peel appearance, 150 Orbicularis, 35, 42, 67, 76, 79, 685 Orbicularis muscle, 81 Orbicularis oculi, 36 Orbicularis reflex, 504 Orbiculosis, 200 Orbit, 56 Orbits of the children, 677 Orbit at birth, 677 Orbit, 86, 116, 153, 697, 701, 769 Orbital abscess, 687, 690, 701, 704 Orbital and periorbital dermoids, 678, 679 Orbital apex, 539 Orbital apex syndrome, 607, 763 Orbital causes, 62 Orbital cellulitis, 44, 97, 282, 477, 526, 532, 674, 685, 687, 688, 689, 690, 698, 701, 703, 708, 709, 712, 728, 736, 738, 739, 763 Orbital cyst, 152 Orbital dermoid, 712 Orbital dysplasia, 696

INDEX
Orbital encephalocele, 678 Orbital fat, 87, 675, 676 Orbital granuloma, 760 Orbital meningocele, 678 Orbital neuroblastoma, 708 Orbital part, 87, 602 Orbital part of the palpebral, 111 Orbital plate of frontal, 673 Orbital pseudo tumours, 417, 624, 687 Orbital retinoblastoma, 687, 688 Orbital rim, 89, 673, 694 Orbital septum, 35, 37, 87, 674 Orbital teratoma, 678, 678 Orbital tubercle, 37 Orbital tumours, 13 Orbital varices, 688, 691, 713 Orbital varix, 687, 696 Orbital venography, 675 Orbitalseptum, 679 Orbitotomy, 115, 675, 679 Orbits, 14 Orbularis, 624 Orchitis, 746 Organ of corti, 439 Organ transplant, 762 Organic amblyopia, 586, 587 Oriental worm, 772 Ornithine, 238 Ortho voltage radiation, 61 Orthokeratology, 554 Orthomyxo virus, 758 Orthophoria, 571, 572, 611, 621, 623, 641, 646, 648, 660, 661, 664, 665, 668 Orthoptic, 648 Osazone crystals, 300 Oscillopsia, 420, 618 Oscilloscope, 420 Osteo chondritis, 740 Osteoblastic metastases, 696 Osteogeneses, 26 Osteogenesis imperfecta, 695, 722 Osteogenic sarcoma, 479 Osteoma, 701 Osteomyelitis, 736, 744 Otitis, 129 Otitis media, 620, 731, 734, 735, 736, 743, 748 Outer barrier, 423 Outer neuroblast, 399 Outer nuclear layer, 395, 396, 403 Outer nuclear zone, 399 Outer plexiform, 396 Outer plexiform layer, 395, 396, 403 Outflow is within normal limits, 370 Outflow of aqueous, 341 Ovaries, 746 Over correction of ptosis, 81 Oxalosis, 439 Oxidase, 733 Oxycephaly, 306, 679, 681 Oxydative phosphorylation, 537 Oxygen, 102, 160 Oxytetracyclin, 758

807
Papillary, 140 Papillary hypertrophy, 134 Papilledematous, 532 Papillitis, 242, 245, 246, 247, 260, 263, 267, 274, 422, 424, 425, 520, 524, 525, 526, 532, 534, 762 Papillo macular, 526, 537, 582 Papillo macular bundle, 535 Papillo macular fibre, 432 Papilloma, 49, 54, 150 Papillomatous granuloma, 48 Papule, 49, 250 Para central, 582, 641 Para fovea, 394, 641 Para macular, 641 Para median pontine reticular formation, 601, 605 Para median reticular formation, 596 Para sellar growths, 607 Para sympathetic origin, 709 Para-amino benzoic acid, 60 Para-axial mesoderm, 600 Para-sympatholytic drugs, 505 Paraaxial mesoderm, 677 Paracecal, 527 Paracentesis, 151, 184, 278, 364, 380 Paracentral scotoma, 516 Paradoxic movements, 608 Paradoxical glaucoma, 367 Paradoxical reflex, 95 Paradoxical rise of tension, 351 Paralysis of accommodation, 125, 730 Paralysis of convergence, 125 Paralytic, 612 Paralytic ectropion, 82 Paralytic ileus, 729 Paralytic phase, 608 Paralytic squint, 22, 523, 639, 649, 651, 681, 689, 699, 742, 748 Paramyxo viridae, 748 Paramyxo virus, 745, 746 Paranasal sinuses, 677, 689, 692, 703 Paraplegia, 530, 680 Parasellar, 479, 508 Parasites, 45, 759 Parasitic blepharitis, 46 Parasitic conjunctivitis, 152 Parasitic cyst, 152, 453, 477, 696 Parasitic endophthalmitis, 279, 476 Parasitic granuloma, 483 Parasympathetic, 20, 87, 227, 228, 230, 254, 502, 601, 602, Parasympathomimetic, 95, 372, 505 Paratrachoma, 133 Paraxial mesoderm, 6 Paredrin, 74, 506, 512 Parenchymatous, 172, 206

P
P malariae, 766 P ovale, 766 P. acne, 280 P. capitis, 46 P.A.S., 177 P.C. I.O.L., 26 P.H.P.V., 28 P.P.R.O., 619 Pad and bandage, 181 Pagets disease, 439 Pain, 242 Painful ophthalmoplegia, 607 Painted contact lenses, 190 Paired nuclei, 601 Palatal, 62, 622 Palatine, 673 Palatine air cells, 674 Palatine bones, 674 Pallor, 115 Pallor of conjunctiva, 115 Palpebral, 62, 87, 111, 141 Palpebral conjunctiva, 110 Palpebral fascia, 37 Palpebral fissure, 18, 34, 70 Pan fundic lens, 414 Pan retinal photo coagulation, 580 Pan uveitis, 239, 242, 253, 263 Pan uveitis in children, 249 Pancreas, 746 Pannus, 50, 133, 170, 172, 745, 756 Pannus of trachoma, 135 Pano-phthalmitis, 44, 117, 127, 175, 184, 188, 239, 253, 280, 282, 428, 477, 526, 685, 687, 688, 726, 728, 734, 737 Panophthalmitis, 44, 117, 127, 184, 188, 239, 280, 282, 428, 477, 526, 685, 687, 688, 726, 728, 734, 737 Panretinal photo coagulation, 385 Panums fusional area, 638 Panuveitis, 24 Papilitis, 276 Papillae, 23, 37, 116, 139, 141, 147, 510 Papillae formation, 134 Papillaedema, 415, 422, 423, 424, 425, 518, 519, 520, 521,528, 530, 534, 681, 684, 686, 710, 732, 735, 742, 748, 750, 768, 770

808
Parieto-occipial temporal pathway, 596 Parieto-occipital temporal mesencephalic path, 601 Parinauds syndrome, 509, 613, 614, 668 Park Bielschowsky, 611, 614 Parotids, 96, 746 Parrot beak nose, 683 Pars ciliaris, 334 Pars plana, 229, 230, 272 Pars planitis, 240, 241, 245, 246 Pars plicata, 229 Parsplana vitrectomy, 456, 464, 490 Parsplanitis, 239, 253, 258, 265, 433, 454, 461, 463, 476, 742, 771 Partial accommodative, 654 Partial albinism, 402 Partial occlusion, 661 Partially accommodative esotropia, 661 Passive congestion of conjunctiva, 116 Patechea, 119, 211 Patecheal haemorrhage, 123, 129, 755, 767 Patelar fossa, 291, 547 Pathological adhesion of the vitreous, 457 Pathological lid retraction, 80 Pathological myopia, 551, 690 Pathological nystagmus, 633 Pattern reversal VER, 421 Pauci articular, 268 Paving stone, 446 PCO, 453 Peculiarities of pediatric uveitis, 258 Pediatric uveitis, 258 Pediculosis, 46, 176, 765 Pellucid degeneration, 163, 166 Penalisation, 590 Pendular, 633 Pendular nystagmus, 628, 630 Penetrating corneal graft, 211 Penetrating diathermy, 58 Penetrating injuries, 274, 324, 454 Penetrating keratoplasty, 165, 169, 171, 181, 198, 205, 209, 210, 211, 278, 279, 354 Penicillin, 126, 148, 180, 184, 192, 193, 726, 728, 729, 730, 739, 743 Pepper, 439 Pepper and salt, 430, 466 Pepper and salt fundus, 261 Pepper mottling, 429 Pepper type, 709 Perennial allergic conjunctivitis, 139, 140 Perforation, 185 Perforation a globe, 463 Perforation of cornea, 174 Peri artrititis, 272 Peri fovea, 394 Peri foveal arcade, 415 Peri orbital cellutitus, 130 Peri papillary chorio retinal scar, 764 Peri phlebitis, 428 Peri retinal gliosis, 265 Peri retinal membrane, 465 Peri vasculitis, 751, 762 Peribulbar, 599 Peribulbar anaesthesia, 380 Peribulbar injection, 275, 278 Pericarditis, 268, 733, 734, 736, 758 Pericystic cellulilis, 92 Pericystitis, 100 Perilimbal conjunctiva, 144 Perimeter test, 652 Perineural tumour, 539 Perineuritis, 526 Perinuclear, 297 Periocular, 278 Periocular steroids, 270 Periodic acid Schiff stain, 763 Periorbita, 37, 89, 511, 673, 675 Periorbital abscess, 674, 685, 688, 701 Periorbital cellulitis, 702 Periorbital space, 675 Periostitis, 696, 702, 740 Periostium, 87, 674 Peripapillary crescent, 552 Peripapillary edema of retina, 584 Peripbulbar steroids, 272 Peripheral, 178 Peripheral anterior synchia, 241, 244, 247, 249, 250, 337, 340, Peripheral anterior synechiae, 360 Peripheral basal iridectomy, 379, 380 Peripheral button hole iridectomy, 380 Peripheral cataract, 576 Peripheral field of vision, 394, 396 Peripheral gliosis, 265 Peripheral granuloma, 265, 272, 433, 489, 490, 771 Peripheral involvement, 73 Peripheral iridectomy, 312, 356 Peripheral keratitis, 70, 205 Peripheral neuropathy, 439 Peripheral retinal degeneration, 253, 283, 324, 445 Peripheral retinal detachment, 259 Peripheral retinal granuloma, 433 Peripheral space, 675 Peripheral synechiae, 212 Peripheral ulcer, 193

PEDIATRIC OPHTHALMOLOGY
Peripheral uveitis, 454 Peripheral vasculitis, 253, 433 Periphlebitis, 247, 272, 422, 462, 463, 732 Periphlebitis retinae, 426 Peritonitis, 736 Perivasculitis, 245 Perkins, 341 Perkins handheld applanation tonometer, 347 Perlias nucleus, 504, 509 Persistan primary hyperplastic vitreous, 241, 316 Persistence of anterior hyloid system, 295 Persistent corneal haze, 17 Persistent hyaloid artery, 426 Persistent hyaloid system, 316 Persistent hyaloid vasculature, 338 Persistent hyper plastic primary vitreous, 407, 454, 459 Persistent hyper plastic primary vitreous, 266, 453, 475, 476, Persistent neonatal myasthenia, 76 Persistent primary hyper plastic vitreous, 13, 306, 348, 352, 403, 433, 462, 483 Persistent pupillary member, 4, 19, 27, 235, 296, 401, 683 Persistent shallow, 351 Persisting staring, 21 Petechial haemorrhages, 24, 736 Peters, 19 Peters anomaly, 19, 26, 338, 339 Petrosal sinuses drain, 704 Petrous bone, 620, 704 PH, 550 Phaco-morphic, 367 Phacoanaphylactic, 367 Phacoanaphylatic glaucoma, 367 Phacocele, 122, 559 Phacodonesis, 162, 307 Phacoemulsi-fication, 322 Phacogenic, 363 Phacogenic uveitis, 310 Phacolytic, 367 Phacomas, 409, 491 Phacomatosis, 368, 408 Phacotopic, 367 Phagocytes, 255 Phagocytosed, 364 Phagosomes, 395 Phakia children, 8 Phakic, 304 Phakic eye, 244, 357 Phakic intraocular lens, 554, 559 Phakomas, 55

INDEX
Pharmacological causes of lid retraction, 80 Pharmacological defocusing, 590 Pharyngeal muscle, 78 Pharyngitis, 131, 754, 758 Pharyngo conjunctival fever, 126, 128, 131 Pheboliths, 713 Phenothiazine, 103, 440 Phentolamine, 505 Phenyl mercuric nitrate, 215 Phenyl pherine, 208, 255 Phenylbutazone, 148 Phenyle pherine, 20, 80, 254, 318, 506 Pheo chromocytoma, 408 Phithisis, 488 Phlycten, 23, 24, 49, 132, 172, 178, 724, 731, 738, 745, 762 Phlyctenular keratitis, 168, 190 Phlyctenular conjunctivitis, 732 Phlyctenular kerato conjunctivitis, 97, 144, 576, 732 Phlyctenular pannus, 146 Phlyctenulosis, 170, 205 Phlyctenulosis of cornea, 205 Phorias, 571 Phosphate, 214 Phospholine iodide, 657 Phospholipid, 442 Phosphorescent sheen, 575 Photo coagulation, 260, 408, 456, 465, 493 Photo keratoscope, 166 Photo keratoscope detect, 164 Photo metric neutral density filter, 615 Photo radiation, 382 Photo receptor dystrophy, 434 Photo receptors, 393, 395, 477 Photo refractive keratectomy, 554, 559 Photo stress test, 412, 421 Photo sublimation, 382 Photo vaporising, 382 Photocoagulation, 382, 480, 534 Photodisruption, 382 Photographs, 610 Photon, 709 Photophobia, 60, 91, 131, 132, 141, 164, 178, 273, 347, 441, 462, 474, 576, 579, 632, 689 Photophobia in children, 147 Photopic, 417, 418 Photopic ERG, 441 Photopic vision, 396, 586 Photopsia, 411, 450, 459, 552, 576 Photostatic, 510 Phototoxicity, 487 Phthiriasis, 46, 176, 765 Phthisis, 25, 68, 163, 175, 188, 204, 274, 282, 354, 453, 690 Phthisis blubae, 166, 188, 252, 478, 696, 701 Phthisis, perforated globe, 166 Phycomycetis, 762 Phylum thallophyta, 759 Physiology of the cornea, 160 Physostigmine, 505 Pia, 5, 511, 513 Pial vessels, 479 Pica, 266, 489, 490 Picorna viruses, 745 Pie in the sky, 583 Pie on the floor, 583 Pierre Robin syndrome, 682 Pierre-Robinson, 453 Piezo electric crystal, 417 Piezoelectric effect, 700 Pig, 152 Pigment, 342, 436 Pigment deposit, 189 Pigment epithelium, 5, 393, 447 Pigment epithelium of retina, 228 Pigmentary dystrophies, 435 Pigmentation of conjunctiva, 117 Pigmentation of the macula, 246 Pigmented coloboma, 410 Pigmented epithelium, 334 Pigmented epithelium of ciliary body, 229 Pigmented KP, 243 Pilocarpine, 95, 149, 184, 349, 511 Pilocarpine, methacholine, 505 Pilocytic astrocytes, 710 Pin cushion, 561, 570 Pin hole effect, 547 Pineal body, 479 Pineal tumour, 510 Pinealoblastoma, 474 Pinhole, 455, 565, 566 Pituitary, 541 Pituitary fossa, 680 Pituitary tumour, 508, 583 Placenta, 754 Placido, 164 Placido disc, 566 Plane mirror, 164 Plane of nystagmus, 629 Plano T contact lenses, 143 Plaque, 141 Plaque radiation, 481 Plasmodia, 181, 764, 766 Plasmodium vivax, 766 Plasmoid aqueous, 243, 245, 248, 360, 373

809
Plastic iritis, 244 Pleomorphic, 707, 730, 731 Pleura, 731 Plexiform neuro fibroma, 56, 678 Plexiform neuroma, 698 Plica, 87, 94 Plica semilunaris, 112, 113 Plications, 230 Pliocarpine, 373 Plombs, 456 Pluri potential embryonal mesoderm, 707 Plus disease, 486 Plus lenses, 503 Pneumo cocci, 123, 175, 184, 248 Pneumococcal conjunctivitis, 119, 129 Pneumococcal infection, 735 Pneumococcal keratitis, 192 Pneumococcal ulcer, 191 Pneumococcas, 23, 91, 92, 191, 240, 258, 702 Pneumococci, 122, 126, 177, 182 Pneumocystis, 267 Pneumocystiscarinii, 750 Pneumonae, 133 Pneumonia, 49, 526, 726, 730, 731, 736, 744, 747, 748, 758, 763 Pneumonitis, 133, 489 Pneuno tonometer, 341 Pneunococcal conjunctivitis, 24 Pneunococcus, 127, 175 Pneunonitis, 129 Poliomyelitis, 745 Poliosis, 274, 275, 402 Pollen, 139 Poly articular, 271 Polyarthritis, 733 Polyarticular involvement, 268 Polychromatic, 579 Polychromatic lusture, 244, 247, 252 Polycoria, 12, 27, 229, 251, 501 Polycythemia, 117, 408, 422 Polycytosis, 274 Polydactyly, 62, 439 Polyenes, 205 Polygenic inheritance, 339 Polymer, 144 Polymerase chain, 264 Polymeric-polyhexa methylene biguanide, 209 Polymethacrylate (PAMA), 562 Polymorphonuclear cells, 121 Polymorphonuclear leucocytes, 181 Polymyxine, 179 Polyneuritis, 730 Polyopia, 305 Polyp, 101

810
Polypeptide, 257 Polyvinyl alcohol, 106 Pons, 82, 87, 618 Pontine paramedian reticular formation, 619 Ponto medullary junction, 604, 606 Poor accommodation, 302 Poor colour sense, 443 Pork tape worm, 769 Pork worm, 152 Port wine, 57, 61 Posterior ciliary arteries, 232 Positional myopia, 555 Positive HLA B 27, 271 Positive regurgitation, 93 Post ganglion, 502 Post ganglionic lesion, 74 Post herpatic neuralgia, 52 Post herpetic neuralgia, 52, 202, 752 Post keratoplasty, 565 Post neuritic, 532, 533 Post neuritic optic atrophy, 247 Post operative fungal, 281 Post papilledematous, 422, 533, 584 Post papilledematous atrophy, 680 Post papilledematous optic atrophy, 540, 686, 710, 732 Post surgical status, 556 Post trauma, 556 Post traumatic edema, 423 Post vaccination optic neuritis, 529 Post vaccination status, 283, 527 Post vitrectomy glaucoma, 357 Posterior, 256 Posterior capsular cataracts, 296 Posterior capsular opacification, 247, 561 Posterior capsular traction lines, 323 Posterior capsule, 291, 322, 323, 330 Posterior capsulorrhexis, 323 Posterior cerebral, 603, 606, 612 Posterior cerebral arteries, 584, 602 Posterior chamber, 27, 335, 501, 563 Posterior chapsule, 326 Posterior chiasma, 583 Posterior ciliary arteries, 153, 511, 720 Posterior ciliary system, 232 Posterior clinoid, 602, 694 Posterior communicating artery, 602, 603, 606, 607 Posterior conjunctival, 116 Posterior cortex, 330 Posterior cyclitic membrane, 244, 476, 483, 489, 490 Posterior dislocation of lens, 561 Posterior dystrophies in children, 212 Posterior embryotoxon, 338, 340, 242 Posterior epithelium, 228 Posterior epithelium, 229, 614 Posterior lacrimal crest, 89, 599, 674 Posterior lens capsule, 456 Posterior lenticonous, 300, 302 Posterior limbal border, 112, 336 Posterior limbal zone, 336 Posterior mesoderm, 3 Posterior pituitary, 714 Posterior plus, 486 Posterior polar, 295, 296, 437 Posterior polar granuloma, 265 Posterior pole, 291 Posterior polymorphic corneal dystrophy, 337 Posterior polymorphous dystrophy (P.P.M.D.), 212, 216 Posterior root ganglion, 50, 199 Posterior scleral foramen, 720 Posterior staphyloma, 413, 553, 701, 723 Posterior sub capsular cataract, 245, 436 Posterior sub capsular opacification, 257, 328, 437 Posterior synechia, 235, 241, 244, 249, 250, 268, 269, 271, 283, 330 Posterior uveitis, 239, 242, 461, 481, 525, 732 Posterior vasculosa lentis, 293 Posterior vitreous detachment, 246, 247, 253, 260, 449, 451, 452, 456, 462, 553 Postherpeticneuralgia, 202 Potassium, 103 Potassium loss, 257 Povidon, 757 PovidonIodine, 128, 734 Power of IOL, 417 Pox viruses, 745 Poxviridae, 756 Poxvirus, 50 Prasympathomimetic, 77 Pre arterial (Choroidal) phase, 414 Pre auricular, 131, 702 Pre auricular glands, 114 Pre auricular lymphadenopathy, 132, 203 Pre auricular nodes, 38 Pre ganglionic lesion, 74 Pre geniculate, 507 Pre geniculate path, 420 Pre lamilar, 510, 521 Pre mandibular, 600 Pre retinal haemorrhages, 426 Pre senile cataract, 466

PEDIATRIC OPHTHALMOLOGY
Pre vitelli form, 444 Pre-chiasmal conduction, 508 Pre-presbyopic age, 557 Preauricular, 87, 479 Preauricular glands, 92 Preauricular lymph, 51 Preauricular lymph nodes, 47 Precipitates, 196 Precocious puberty, 541, 711 Precorneal tear film, 102 Precortical path, 28 Precortical visual path, 27 Prednisolone, 143, 275, 361, 536, 713 Prednisone, 60, 361 Preferential looking, 29 Prelenticular, 483 Prematurity, 16, 484 Premethrine, 46 Preoperative mydriasis, 257 Prerequisite of binocular single vision, 638 Presenting features, 316 Preseptal, 53, 701 Preseptal cellulitis, 739 Preseptal orbital cellulitis, 701 Pressure necrosis of iris, 357 Presumed ocular histoplasmosis syndrome (POHS), 764 Pretectal nuclei, 602 Pretectal nucleus, 503, 504 Prethresh hold disease, 486 Prexerosis, 217 Primary acquired exotropia, 666 Primary aphakia, 559 Primary atrophy, 710 Primary band keratopathy, 215 Primary buththalmos, 90 Primary colours, 577 Primary congenital buphthatmos, 97 Primary congenital glaucoma, 337 Primary developmental glaucoma, 344 Primary deviation, 651 Primary dye test, 101 Primary herpes simplex, 177 Primary herpes simplex infection, 194 Primary infentile glaucoma, 339 Primary keratitis, 198 Primary lens fibers, 2, 291 Primary neuro sensory dystrophy of the retina, 435 Primary open angle glaucoma, 343, 370 Primary optic atrophy, 535, 540, 681 Primary optic vesicle, 9 Primary position, 594 Primary Vitreous, 2, 3, 458, 487

INDEX
Primary/congenital retinal telangiectasis, 405 Primitive internal carotid, 6 Primitive precursor, 477 Priscoline, 505 Prism, 503, 635, 648, 668 Prism and cover test, 645, 670 Prism diopters, 645, 648, 653 Prism dissociation test, 645, 646 Prism reflex test, 652 Prism test, 78, 618, 644 Pro drug, 197, 362, 375, 376 Probable cause of angle closure glaucoma in uveiti, 360 Probable causes of open angle glaucoma in uveitis, 360 Probable congenital causes of brown syndrome, 616 Probe, 699 Probing, 93 Probing of the nasolacrimal duct, 91 Procaine, 255 Proctitis, 757 Prodromal, 148 Prodromal stage, 275 Proglotidis, 769 Programmed death, 434 Progressive cone dystrophy, 434, 441 Progressive external ophthalmioplegia, 437, 439, 613 Progressive night blindness, 238 Progressive outer retinal necrosis (PORN), 750 Progressive stage, 170, 206 Progressive supra nuclear palsy, 624 Projection, 638 Prolapse of uvea, 280, 325 Proliferative diabetic retinopathy, 454 Propamindine, 209 Proparacaina, 105, 106 Proparacine hydrochloride, 176 Properties of laser, 382 Prophylactic iridectomy, 302 Prophylaxis, 128, 153, 219 Propioni bacterium, 258, 280, 323 Proptometers, 691 Proptosis, 16, 18, 20, 44, 56, 62, 80, 81, 96, 111, 117, 153, 173, 221, 253,474, 479, 539, 607, 609, 675, 676, 677, 678, 679, 681, 684, 699, 702, 707, 709, 710, 715, 763, 770, 771 Prostaglandin, 354, 360, 368, 370 Prostaglandin analogue, 270, 372, 376 Prostaglandin inhibitors, 257 Prostaglandin level, 487 Prostaglandins, 138, 506 Prostaglandinsynthatase, 257 Prostamide sensitive receptors, 376 Prostamides, 372, 376 Prostigmine, 76, 77, 80 Protanomalous, 578 Protanomaly, 578 Protanopia, 578 Protanopia deuteranopia, 578 Proteases, 735, 737 Protein caloric malnutrition, 219, 440 Proteolytic enzyme, 192 Proteus, 124, 743 Protozoa, 152, 259, 764 Pseudo angle, 251, 360 Pseudo angle closure glaucoma, 252 Pseudo angle formation, 250 Pseudo arcus, 142 Pseudo Argyll Robertson pupil, 509, 608 Pseudo bulbar paby, 98 Pseudo cancerous, 54 Pseudo convergent squint, 15, 18, 22, 40, 41, 550, 642 Pseudo cystic phase, 765 Pseudo dendretic keratitis, 49 Pseudo dendrite, 208, 756 Pseudo dendritic ulcer, 50 Pseudo divergence, 667 Pseudo divergent squint, 558 Pseudo enophthalmos, 74 Pseudo exotropia, 665, 666 Pseudo glioma, 483 Pseudo Graefe sign, 72, 608 Pseudo hypopyon, 283, 444 Pseudo isochromatic, 579 Pseudo macula, 639 Pseudo membrane, 121, 127, 131, 148, 735, 736, 751, 769 Pseudo membrane formation, 132, 203, 731, 754 Pseudo membrane over, 141 Pseudo membranes conjunctivitis, 119, 124, 126, 129, 149, 194, 730, 739, 745 Pseudo membranous, 125 Pseudo membranous colitis, 432 Pseudo myopia, 557 Pseudo neuritis, 415, 513, 519, 530, 532, 558 Pseudo papilledema, 513, 519 Pseudo paralysis, 624 Pseudo polycoria, 337, 501 Pseudo polycoria, corectopia, 340 Pseudo proptosis, 345, 552, 690, 723 Pseudo pterygium, 189 Pseudo ptosis, 22, 48, 68, 79, 141, 200, 609, 690 Pseudo retinitis pigmentosa, 741

811
Pseudo retraction syndrome, 623 Pseudo rosettes, 478 Pseudo spiralis, 772 Pseudo squint, 265, 641, 651 Pseudo tumor orbit, 624 Pseudo tumour, 620, 695, 708 Pseudo tumour cerebre, 522, 532, 742, Pseudo tumour, 525, 607, 686, 688, 698, 690, 697, 705, Pseudo vertical squints, 612 Pseudo-angle-formation, 354 Pseudo-membranous-conjunctivitis, 762 Pseudocoria, 186, 338 Pseudoglioma, 475 Pseudohypopyon, 26, 368, 474, 479, 492 Pseudomembranous conjunctivitis, 104 Pseudomonas, 123, 127, 175, 176, 177, 182, 191 Pseudomonas areuginosa, 737 Pseudomonas corneal, 184 Pseudomonas infection, 181 Pseudomonas keratitis, 192 Pseudomonas ulcers, 184 Pseudomyopia, 550, 555 Pseudomyopia night myopia space myopia, 548 Pseudoneuritis, 8, 518, 524 Pseudophakia, 354, 453, 562, 563 Pseudophakic myopia, 555 Pseudopolycoria, 27, 229, 234 Pseudoproptosis, 550 Pseudorosettes, 477 Psittacosis, 133 Psychosensory reflex, 504 Pteroclinoid ligament, 510 Pterygia, 59, 122 Pterygium, 122, 166, 189, 565 Pterygo mandibular complex, 683 Pterygoid, 43 Pterygoid muscle, 43 Pterygoid plexus, 677 Ptosis, 13, 16, 18, 22, 40, 61, 67, 74, 75, 78, 96, 163, 200, 257, 306, 330, 509, 585, 588, 590, 608, 609, 614, 617, 650, 657, 678, 684, 685, 690, 728, 729, 730 Ptosis crutches, 78 Ptosis in childhood, 69 Puberty, 163 Pulfrich phenomenon, 530 Pulmonary edema, 767 Pulmonary tuberculosis, 731 Pulmonic stenosis, 747 Pulsating exophthalmos, 56 Pulse less disease, 240

812
Pulverulent cataract, 297 Pumic stone iris, 772 Puncta, 7, 86, 88, 98 Punctal displacement, 71 Punctal stenosis, 41 Punctate keratitis, 45, 135, 172, 726, 752, 756, 771 Punctate lesions, 201 Punctual block, 107 Punctum, 92 Punctum dilator, 100 Punctum remotum, 550 Pupil, 15, 19, 227, 244, 501 Pupil, 4 Pupil is kinetic indicator of functional state, 502 Pupillary abnormalities, 16 Pupillary block, 302, 312, 354, 357, 360, 361, 363, 373 Pupillary light reflex, 503 Pupillary margin, 227 Pupillary membrane, 4, 6, 234, 293 Pupillary reaction, 70 Pupillary reaction in mid chiasmal lesion, 508 Pupillary zone, 227 Pupillo motor fibres, 503 Pure amblyopia, 585 Pure carbolic, 181 Pure microphthalmos, 8 Purkenjes image, 28, 244, 251, 484 Purpura, 24, 44, 425, 463 Pursuit, 586 Purtschers retinopathy, 426 Purulent, 148 Purulent conjunctivitis, 44, 726, 751, 762 Purulent discharge, 24, 91, 120, 180 Purulent keratitis, 282 Purulent optic neuritis, 704 Purulent pan uveitis, 278 Purulent retinitis, 428 Pus in AC, 244 Pustular, 200 Pyelitis, 762 Pyocyanin, 737 Pyoderma, 739 Pyramidal cataract, 296 Pyramidal tract, 604, 619 Pyridostigmine, 76, 77, 505 Pyridoxine, 238, 311 Pyrimethamine, 261, 432 Pyrimidines, 205 Quadrilateral, 166 Quinine, 440 Quinine amblyopia, 419, 767 Quinone, 401

PEDIATRIC OPHTHALMOLOGY
Red green defect, 580 Red KP, 243 Red nucleus, 602, 605, 606 Reduced ciliary secretion, 354 Reduced thickness, 167 Reduced zinc in plasma, 537 Reduplication, 296 Refixation reflex, 637 Reflected part, 598 Reflex lacrimation, 103 Reflex tearing, 95, 102, 105, 106 Reflex watering, 62 Refractive error, 371, 570, 656 Refractive index, 103, 161, 340, 456 Refsums syndrome, 439 Regular astigmatism, 564 Regurgitation test, 23, 150 Rehegmatiogenous retinal detachment, 488 Reigers anomaly, 306, 314, 316 Reis-Bucklers dystrophy, 211 Reiters syndrome, 240, 271, 757 Relative afferent pupillary defect, 507 Relative afferent pupillary response, 586 Relative negative convergence, 648 Remnants of hyaloid vessels, 462 Remnants of hyloid system, 20, 519 Remnants of hyoid system, 28 Remnants of primary vitreous, 461 Remote injuries, 119 Renal failure, 767 Renal retinopathy, 434 Renal stone, 330 Rendu-Osler-Weber syndrome, 55 Resse ellosworth classification, 483 Restrictive causes of vertical squint, 613 Restrictive strabismus, 689 Retained intra ocular copper, 329 Retained intra ocular foreign body, 278, 324, 326, 687, Retained orbital foreign body, 688 Retention cyst, 122, 152 Retina, 5, 56, 153, 230, 234, 259, 274, 393, 503, 512 Retinal detachment, 403 Retinal abiotrophy, 534 Retinal aneurysm, 427 Retinal angioma, 407 Retinal angiomatosis, 405, 408 Retinal artery, 396, 397 Retinal artery obstruction, 416 Retinal artery occlusion, 422 Retinal astigmatism, 564 Retinal astrocytoma, 492 Retinal band, 433

R
Rabies, 529 Racemose aneurysm, 405 Radial fibres, 230 Radial keratotomy, 554 Radiation, 324, 471, 706, 709, 759 Radiation cataract, 481 Radiation retinopathy, 481 Radiation to the mother, 429 Radio isotope, 102 Radio therapy, 707 Radioactive cobalt plaque, 480 Radiology of orbit, 693 Radiotherapy, 708 Radon, 481 Raeders, 512 Rafsums syndrome, 439 Rail road, 632 Rail road nystagmus, 630 Raised episcleral pressure, 24 Raised intra cranial tension, 173, 179, 680, 770 Raised serum calcium, 272 Ralysis, 132 Ramsay Hunt syndrome, 52, 82, 200, 753 Range of fusion, 646 Rapid phase, 629 Rarely fungi, 97 Rate, 628 Rate of formation of aqueous humour, 341 Raymonds syndrome, 619 Recession of angle of anterior chamber, 326, 363 Reciprocal innervation, 628 Reckling Hausens, 55, 368 Reconstruction of anterior segment, 327 Recording of distant vision, 28 Rectus, 601 Recurrent branch of choroids, 232 Recurrent erosion, 210 Recurrent herpes simplex, 194 Recurrent HSV, 197, 198 Recurrent HSV keratitis, 198 Recurrent lacrimal artery, 677 Recurrent retinal branch, 397 Red blood cells, 364 Red cell glaucoma, 363 Red glass test, 641, 644, 645, 646, 653 Red green colour defect, 443

Q
Q switched Nd-YAG, 351 Quadraniopia, 583

INDEX
Retinal blood vessels, 246 Retinal break, 448, 461 Retinal capillaries, 393, 394 Retinal capillary haemangioma, 478, 492 Retinal cavernous haemangiomas, 405, 409 Retinal circulation, 7 Retinal correspondence, 646 Retinal cysts, 402 Retinal degeneration, 403, 683 Retinal detachment, 36, 57, 122, 253, 267, 274, 282, 300,309, 324, 325, 326, 374, 384, 403, 429, 465, 478, 490, 549, 552 Retinal dialysis, 452 Retinal diseases in children, 410 Retinal drusen, 438 Retinal dysplasia, 410, 475, 480, 484 Retinal dystrophies and degenerations, 434 Retinal dystrophy, 403 Retinal edema, 423, 750 Retinal electrode, 420 Retinal exudates, 277, 761 Retinal folds, 402 Retinal ganglion cells, 532 Retinal haemorrhage, 267, 326, 424, 685, 742, 744, 758, 767, 768 Retinal holes, 446, 487 Retinal hypoxia, 429 Retinal micro aneurysm, 426 Retinal micro angiopathy, 749 Retinal nerve layer, 246 Retinal periphery, 15 Retinal periphlebitis, 457, 766 Retinal phlebitis, 262 Retinal pigment degeneration, 78 Retinal pigment epithelium, 273, 383, 395, 398, 400, 414, 445, 492, 552 Retinal pigmentation, 487 Retinal pigmentepitheliopathy, 767 Retinal plane, 412 Retinal space, 153 Retinal surgery, 277, 354 Retinal system, 231 Retinal tear, 446 Retinal telangiectasis, 405 Retinal thinning, 451 Retinal toxocariasis, 432 Retinal vasculitis, 258, 433, 750, 760 Retinal veins, 398, 768 Retinal vessels, 414 Retinalblastoma, 489 Retinitis, 245, 246, 261, 422, 423, 424, 425, 760, 762 Retinitis albi punctatus, 418 Retinitis pigmentosa, 163, 238, 262, 283, 306, 400, 404, 418, 419, 422, 429, 435, 438, 439, 534, 576, 584 Retinitis proliferance, 427, 463 Retinitis punctata albescens, 438, 575 Retino blastoma, 17, 20, 90, 240, 260, 266, 283, 316, 317, 348, 368, 403, 406, 407, 411, 415, 421, 425, 433, 454, 471, 483, 488, 489, 542, 655, 678, 689, 695, 696, 697, 698, 701, 708, 771 Retino choroidal coloboma, 236 Retino choroiditis, 421, 428, 431, 432, 476 Retino phlebitis, 414 Retino schisis, 423 Retino-neural scotomas, 582 Retinoblastoma leukemia, 259 Retinochoroiditis, 239, 259 Retinocytom, 478 Retinol, 217 Retinol binding protein, 217 Retinoma, 478, 480 Retinopathies, 411, 423 Retinopathy, 424, 747 Retinopathy of pre-maturity, 28, 266, 348, 433, 454, 463, 466, 476, 484, 489, 666 Retinopathy of radiation, 708 Retinoschisis, 403, 446, 447, 451, 453 Retinoscopy, 164, 251, 246, 326, 412, 451, 457, 550, 552, 553, 723 Retraction nystagmus, 81 Retraction of the lid, 613 Retro bulbar anaesthesia, 599 Retro bulbar growths, 525 Retro bulbar haemorrhage, 687, 690 Retro bulbar mass, 348 Retro bulbar neuritis, 508, 529 Retro bulbar space, 706 Retro hyaloid space, 449 Retro illumination, 210, 251 Retro lamilar, 510, 521 Retro lental fibro plasia, 426, 427, 484 Retro lental white reflex, 406 Retro lenticular mass, 474 Retrocorneal vascularisation, 171 Retrolental fibroplasia, 316, 318, 352, 369, 414, 483, 496 Retrolental space, 273 Retrolental white mass, 460, 488 Retrolenticular, 483 Retrolenticular flare, 280 Retrovirus, 267 Reverbation, 700 Reverse coloboma of the disc, 519 Rhabdhomyosarcoma, 53, 471, 477, 687, 688, 690, 696, 698, 699, 703, 705, 706, 707, 709, 712

813
Rhabdomyoblast, 707 Rhabdomyocytes, 284 Rhagades, 207 Rhegmatogenous, 253, 411, 448 Rhegmatogenous detachment, 450 Rhegmatogenous retinal degeneration, 445 Rhegmatogenous retinal detachment, 236, 305, 324, 404, 448, 487, 553 Rhese view, 694 Rheumatoid arthritis, 206, 240 Rheumatoid factor, 268, 271 Rhinitis, 129, 139, 143, 740, 748 Rhinorrhea, 734 Rhinosporidiosis, 49, 93, 119, 149 Rhinosporidiosis of sac, 99, 151 Rhinosporidium seebri, 93, 149 Rhizopus, 762 Rhodopsin, 217 Riboflavin deficiency, 136, 170 Riegers, 339 Riegers anomaly, 338 Rifampin, 137, 727 Riley day syndrome, 95 104, 221, 511 Rim, 673 Ring scotoma, 238, 440 Ring shaped opacity, 196 Ring synechiae, 247, 360 Ring ulcer, 178 Ringworm infection, 762 Rise of intraocular tension, 183 Risus-sardonicus, 728 River blindness, 152, 765, 771 RNA, 744, 749 Rod and rod-cone, 393, 394, 396, 399, 418, 434, 503 Rod monochromatism, 441, 442, 578 Rod spherule, 396 Rod-cone-break, 418 Roennes step, 582 Romanas sign, 768 Rombers syndrome, 237 Roof, 673 Root, 227 Ropy discharge, 120, 139, 141 Rosacea, 178 Rosaceakeratitis, 170 Rose bengal, 51, 172, 176, 178, 195, 201 Rosenthal Malcarson, 62 Roseola, 250 Rosette cataracts, 325, 326 Rosette concussion cataract, 326 Rosettes, 284, 325, 477, 482, 709 Rotary prism, 646 Roths, 267, 283, 425, 428 Round holes, 447

814
Roundworm, 489 Roving ring scotoma, 561 Roxithromycin, 432, 743 RPE, 382, 414 RPE detachment, 415 Rubella, 12, 240, 253, 258, 295, 298, 316, 317, 319, 400, 429, 529, 745, 746, 747 Rubella glaucoma, 429 Rubella retinitis, 429 Rubella retinopathy, 299, 741, 427 Rubella syndrome, 299, 747 Rubella uveitis, 261 Rubeola, 49, 253, 295, 429, Rubeola retinitis (Measles retinitis), 429 Ruby, 383 Ruby laser, 384 Ruff of the iris, 229 Ruggiero, 694 Rule of 2-4-6, 630 Running of letters, 647 Rupture and folds in Descemets membrane, 19, 162, 164, 169, 171, 345 Rupture and splitting of Descemets membrane, 189 Rupture in Bowmans membrane, 164 Rupture in Desmets membrane, 97 Rupture of anterior vitreous faces, 358 Rupture of choroid, 535 Rupture of conjunctival vessels, 119 Rupture of cyst, 444 Rupture of sphinter, 326 Rush disease, 487 Ruthenium 106, 481 Salivary gland, 87, 96 Salivation, 374 Salmon patch, 741 Salmon red colour, 206 Salmonella typhi, 743 Salmonellosis, 743 Salpingitis, 733, 736, 757 Salt and pepper pigmentation, 429, 741 Salt and pepper spots, 262 Salt appearance, 439 Salt pepper, 440 Salt pepper appearance, 206 Salt pepper fundus, 400 Salzmanns nodular dystrophy, 147 Samolina, 150 Sand fly Phlebotomus, 767 Sandhoff disease, 443, 425 Sangunarine, 369 Saprophyte, 110, 279, 743, 759, 760, 761 Sarcoid in children, 269 Sarcoidosis, 96, 215, 240, 243, 246, 250, 253, 259 Satellite, 177, 182 Satellite conjunctival abscess, 192 Satellite epithelial keratitis, 195 Satellite lesion, 203, 431 Saturation, 578 Sayer syndrome, 624 Sbiza bar, 615 Scalooped, 244 Scalp injuries, 118 Scaphocephaly, 680 Scaphoid head, 680 Scar, 37, 415 Scar or the macula, 246 Scarring, 111 Scarring of conjunctiva, 120 Scattering of light, 168 Schilders disease, 509, 526, 527, 529, 530 Schiotzs tonometer, 347 Schirmer, 2, 105, 106 Schirmers test 1a, 105, 106 Schizomycetis, 759 Schlemms canal, 335, 342 School myopia, 549 Schwalbes line, 112, 307, 335, 338, 342 Scissors, 164 Sclera, 8, 10, 14118, 149, 152, 160, 230, 336, 697, 720 Sclera fixated lens, 309 Scleral buckle, 144, 408, 447 Scleral canal, 510, 519 Scleral crescent, 510

PEDIATRIC OPHTHALMOLOGY
Scleral curvature, 163 Scleral dentation, 245 Scleral explant of suitable, 151 Scleral fixed lens, 563 Scleral indentation, 451 Scleral injuries, 324 Scleral involvement, 52, 150, 151 Scleral rigidity, 341 Scleral spur, 3, 112, 229, 335, 342 Scleral staphyloma, 151 Scleral sulcus, 335, 342 Sclerectomy, 381 Scleritis, 52, 97, 196, 201, 208, 732, 737, 738, 743, 746, 753, 763 Sclero cornea, 162 Sclero cornea of various degrees, 216 Sclero uveitis, 20, 239 Sclerocornea, 25, 168, 339, 722, 723 Sclerokeratitis, 201 Sclerosing keratitis, 732, 771 Sclerosis, 527 Sclerotomy, 379, 381 Scolio kyphosis, 308 Scopolamine, 506 Scotoma, 247, 253, 514, 526, 537, 574, 581 Scotopic, 417, 418, 586 Scotopic ERG, 441 Scotopic vision, 396, 574 Scrambled egg, 445 Searching movement, 29 Seasonal allergic conjunctivitis, 117, 139 Sebaceous glands, 98, 679 Seborrhic keratosis, 54 Seclusio pupillae, 250, 251, 354, 501 Second head somite, 600 Second malignancy, 690 Second neoplasm, 481 Second neoplasm in retinoblastoma, 479 Second neuron, 73 Secondary deviation, 651 Secondary angle closure, 354 Secondary aphakia, 559 Secondary congenital buphthalmos, 343 Secondary exotropia, 663, 665, 668 Secondary glaucoma, 52, 186, 188, 196, 202, 204, 235, 242, 247, 270, 271, 274, 325, 343, 380, 407, 429, 473, 487, 493, 755 Secondary implant, 321 Secondary lens fiber, 2, 292, 293 Secondary open angle glaucoma, 361 Secondary optic atrophy, 523, 537 Secondary position, 594

S
S. albus, 738 S. aureus, 737 S. epidermis, 738 S. hemolyticus, 738 S. saprophyticus, 738 Sabouraud medium, 177, 204 Sabourauds media, 763 Sac, 98 Saccade, 586, 596, 594 Saccoidosis, 97 Sacro iliac joint, 271 Sacroid nodule, 250 Sacroilitis, 271 Safe atropinisation, 182 SAFE method, 137 Sagital sutures, 680 Salicylate, 148, 257 Saline, 100

INDEX
Secondary vitreous, 3, 458 Secretion, 340 Sector iridectomy, 380 Sector retinitis pigmentosa, 438 See saw nystagmus, 630, 632, 634 Seen in atopic adults, 143 Seidel scotoma, 582 Seidel test, 176 Seizures, 770 Sella, 542 Sellar, 583 Sellaturcia, 694, 704 Semi circular canal, 632 Semi lunar fold, 35 Senile retinoschisis, 447 Sensation of cornea, 26, 80 Sensitivity done, 179 Sensory deprivation, 629 Sensory nystagmus, 234, 579 Sensory retina, 5, 395, 398, 403, 447 Sepic shock syndrome, 738 Septae, 760 Septed fungal hyphae, 204 Septic arthritis, 731 Septicemia, 424, 726 Septum orbitale, 37, 86, 675 Sero negative, 268 Serous retinal detachment, 265, 274, 416 Serpiginous choroditis, 258 Serpiginous ulcer, 736 Serum potassium, 377 Serum sickness, 760 Sessile, 150 Seton, 350 Setting sun appearance, 681 Setting sun sign, 613, 614 seventh nerve, 604, 745 Severe anemia, 424 Severe ptosis, 79 Shallow orbits, 678, 680 Shape of cornea, 25 Sheath of the optic nerve, 597 Sheathing, 433, 524, 534 Sheathing of blood vessels, 247 Sheep, 153 Sheridan ball test, 30 Sherophakia, 367 Sherringtons law, 595 Shield, 141 Shingles, 199 Short ciliary nerve, 502, 503, 510, 511 Short ciliary vessels, 5 Short posterior ciliary arteries, 6, 232, 414, 512 Short posterior ciliary nerves, 675 Short sightedness, 547 Short term escape, 376 Shot silk appear, 558 Shot silk look, 519 Shrinkage of sclera, 368 Shunt operations, 381 Shunts, 350, 415 Sialidosis, 443 Siblings, 347 Sickle cell anaemia, 425 Sickle cell disease, 364 Sickle cell haemoglobinopathy, 277 Sickle cell retinopathy, 414, 422, 427, 425, 434 Side effect of steroids, 257 Siderosis, 329 Sigmoid sinus, 704 Signs, 175 Signs and symptoms of proptosis are, 684 Signs of anterior uveitis, 242 Signs of aphakia, 560 Signs of Bells palsy, 82 Signs of chronic posterior uveitis, 247 Signs of intermediate uveitis, 245 Signs of papilledema, 523 Signs of posterior uveitis, 246 Signs of retinal detachment, 450 Signs of retinal diseases, 411 Signs of retinal disorders, 421 Signs of retinitis pigmentosa, 436 Signs of vitreous disorders, 459 Signs of vitreous haemorrhage, 463 Silent uveitis, 269 Silicon, 169, 456, 562 Silicon plug, 107 Silicone oil, 215, 357 Silver beaten impression, 521 Silver nitrate drop, 128 Silver nitrate solution, 734 Simple astigmatism, 565 Simple buphthalmos, 343, 344 Simple congenital ptosis, 42 Simple ectopia, 306 Simple follicular conjunctivitis, 120 Simple heterochromia, 237 Simple hypermetropic astigmatism, 565 Simple myopia, 549 Simple myopic astigmatism, 565 Simple P.C.O., 322 Simple ptosis, 70 Simplex, 253 Simply primary retinal dystrophy, 435 Simulated divergence excess, 667

815
Simulating dendritic ulcer, 767 Simultaneous macular perception, 653 Simultaneous perception, 638 Sinus, 703 Sinus histocytosis, 689 Sinusitis, 607, 735, 736 Sinusitis meningitis, 736 Sixth nerve, 82, 675, 705 Sixth nerve palsy, 523 Size of cornea, 25 Size of pupil, 501 Sjogren hand, 31 Sjogrens syndrome, 104 Skeletal changes, 310 Skew deviation, 613, 614, 615 Skin involvement, 57 Sleeping sickness, 768 Sling shot, 281 Slit lamp, 101, 326, 382, 416, 632 Slit lamp biomicroscope, 164, 412, 452 Slit like pupil, 234, 338 Sloan achromatopsia, 579 Sloughing ulcers, 175 Slow eye movement, 595 Slow fibres, 593 Slow phase, 629 Slow release inserts, 106 Small and medium sized KPs, 243 Small penetrating injuries, 325 Small pox, 186, 189, 219 Small pox virus, 745, 751 Smear, 179 Smooth pursuit, 594, 596 Snail track degeneration, 445, 446 Snail-track, 553 Snellens chart, 31, 443, 463, 513, 540, 547, 585, 589 Snellens drum, 568 Snellens optotypes10, 30 Snow balls, 245, 281, 762 Snow bank appearance, 276 Snow banking, 245, 272 Snow flake, 301, 446, 464 Snowball opacities, 272 Sodium, 103 Sodium chordrontin sulphate, 215 Sodium cromoglycate, 140, 142, 143 Sodium EDTA 0.5%, 249 Sodium hyaluronate, 215 Sodium nedocromil, 143 Soemmerring, 322, 323 Soft contact, 211 Soft cotton wool, 524 Soft exudate, 415, 424 Softening of eye, 174

816
Solid detachment, 455 Solid state, 383 Somnolence, 541 Sorsby pseudo inflammatory dystrophy, 435 Source of energy, 382 Space myopia, 555 Sparfloxacin, 179 Sparganosis, 765 Spasm of accommodation, 373, 509, 555, 614 Spasm of puncta, 373 Spasm of the ciliary body, 175 Spasmolytic drugs, 77 Spasmus nutans, 628, 630, 631, 633 Spastic phase, 608 Spatial, 570 Spatial localisation, 586 Special piggy back, 165 Specific retinitis, 428 Specific treatment, 179, 254 Specific treponemal, 262 Specific types of corneal ulcer, 190 Spectacle, 165, 309, 320, 321, 549, 550, 551, 553, 558, 564, 569, 659 Specular microscopy, 337 Speherophakia, 304 Spheno palatine artery, 89 Spheno palatine ganglion, 95 Spheno palatine sinus, 704 Sphenoid, 511, 598, 673, 704 Sphenoid ridge, 694 Sphenoid sinuses, 694 sphenoidal fissure, 676 Spherical aberration, 501 Spherical lens, 302 Spherophakia, 747 Spherule, 761 Sphincter pupillae, 227, 228, 502, 503 Sphincterotomy and iridoplasty, 379, 380 Spider shaped, 437 Spielmann, 615 Spike, 700 Spina bifida, 308, 679 Spina lateralis recti, 596 Spinal cord, 56, 530 Spinal ganglion, 194, 754 Spinal nerves, 56 Spine, 479 Spine for the lateral rectus, 677 Spino ponto cerebellar degeneration, 439 Spinocerebellar ataxia, 440 Spinomedullary junction, 634 Spiral of tillaux, 600 Spirochaetaceae, 740 Spirochete trapenoma pallidum, 206 Spiromycin, 261 Spontaneous hyphema, 284 Spontaneous regression, 478 Spontaneous venous pulsation, 518, 523 Spontaneously regressed tumour, 478 Sporangia, 149 Spore forming, 726 Spores, 149, 728, 759, 760 Sporotrichosis, 760, 764 Spread of comitance, 612 Spring catarrh, 23, 24, 120, 136, 147, 149, 163, 168, 328, 361, 362 Squamous, 45 Squamous carcinoma, 54 Squamous cell carcinoma, 750 Squamous cells, 54, 89 Squint, 13, 20, 21, 29, 71, 76, 122, 147, 168, 172, 178, 185, 186, 189, 236, 237, 248, 315, 316, 327, 432, 437, 487, 513, 517, 541, 549, 551, 557, 585, 621, 632, 641, 649, 679, 683,684, 706, 710, 723, 747, 766 St. Judes hospital classification, 483 Stage of fibro vascular proliferation, 485 Stage of infiltration, 125 Stage of line of demarcation, 485 Stage of ridge formation, 485 Stage of total retinal detachment, 486 Stage of white reflex, 406 Stages of development of retina, 399 Staining of corneal lesions, 176 Standing potential of the eye, 420 Staphyloccous, 110, 191 Staphylococcal blepharo-conjunctivitis, 178, 193 Staphylococci, 123, 126, 177 Staphylococcus, 23, 53, 124, 127, 144, 258, 702, 737 Staphylococcus aureus, 122, 124 Staphylococcus corneal ulcer, 193 Staphylococcus epidermis, 280, 323 Staphyloma, 150, 186, 308 Stargards disease, 435, 443 Stargardts, 443 Stationary cone dystrophy, 434, 441 Stationary night blindness, 21, 420 Stationary stage, 170 Stem cell transplant, 107 Stem cells, 111 Stenopic slits, 455 Stenosis of aqueduct, 614 Stenosis of nasolacrimal duct, 93 Stereopsis, 413, 526, 533, 537, 563, 570, 594, 637, 638, 653, 666

PEDIATRIC OPHTHALMOLOGY
Sterile hypopyon, 169 Sterile post operative endophthalmitis, 281 Sterile pus, 181 Steriopis, 22, 588 Steroid, 174, 360, 733 Steroid drops, 269 Steroid induced cataract, 327 Steroid induced glaucoma in children, 361 Steroid over dose, 522 Steroid responders, 361 Steroids, 80, 191, 264, 607 Steven-Johnsons syndrome, 23, 63, 104, 106, 120, 121, 139, 148, 221, 377 Stevens Johnson, 148, 149 Sticklers syndrome, 446, 453, 464 Stigmata, 207 Stigmatic eye, 564 Still birth, 740, 765 Stilling, 579 Stills disease, 268 Stimulus deprivation amblyopia, 586, 587 Stocker and Holt, 211 Stomaititis, 754 Storage diseases, 536 Strabismic amblyopia, 586, 653 Strabismus, 13, 239, 246, 265, 277, 465, 474, 489, 538, 539, 540, 575, 614, 642, 682, 710 Strabismus fixus, 620, 623, 693 Straight eye amblyopia, 587, 590 Strands of liquid anterior vitreous, 460 Stratum opticum, 397 Strawberries, 60 Strawberry patches, 712 Streaks, 328 Strepto cocci, 123, 126 Strepto coccus, 23, 127, 144, 177, 191, 702, 739 Streptococcal, 258 Streptococci, 126 Streptococci pyogenes, 182 Streptococcus peumoniae, 735 Streptococcus pyogenes, 193 Streptomycin, 526, 529, 727 Stretching of sclera, 345 Stretching of the cornea, 344 Stretching of the zonules and the iris, 345 Stroma, 160 Stroma, 159, 161, 168, 187, 210, 228 Stroma of ciliary processes, 334 Stroma proper, 228

INDEX
Stromal cells, 167 Stromal dystrophies in children, 211 Stromal infiltration, 201 Stromal involvement, 195, 196 Stromal necrosis, 196 Stromal necrotic keratitis, 755 Stromal ring, 208 Stromal thickening, 755 Struge Weber syndrome, 45, 54, 55, 57, 68, 151, 306, 352, 368, 405, 678, 696 STUMPD, 89, 216 Stycar test, 31 Stye, 44, 47, 51, 97, 557, 565, 571, 647, 702, 738, 748 Sub acute, 425 Sub acute bacterial endocarditis, 422, 424, 428 Sub acute infective retinitis (Retinitis of Roth), 428 Sub arachnoid haemorrhage, 532 Sub arachnoid space, 602 Sub capsular opacity, 252 Sub conjunctival, 44 Sub conjunctival cyst, 154, 770 Sub conjunctival haemorrhages, 23, 44, 50, 118, 130, 132, 768 Sub conjunctival injection, 111, 180, 182, 192, Sub conjunctival patechial haemorrhage, 767 Sub dural space, 511 Sub epithelial opacities, 132, 326 Sub hyaloid haemorrhage, 426, 433, 463 Sub laxation, 252, 302, 304, 307, 314, , 325, 345, 365, 367, 564 Sub luxation of lens, 19, 235, 326, 335, 448, 723 Sub mandibular, 38, 92, 114 Sub mandibular glands, 87, 702 Sub mandibular lymphnodes, 479 Sub normal vision, 17 Sub periostial abscess, 477 Sub retinal cysts, 770 Sub retinal fibrosis, 451 Sub retinal fluid, 273, 448, 488, 516 Sub retinal haemorrhage, 367, 427 Sub retinal neovascularisation, 247, 260, 429, 516, 747 Sub retinal space, 769 Sub-arachnoid haemorrhage, 426 Sub-conjunctival antibiotic injection, 184 Subacute sclerosing panencephalitis, 429 Subarachnoid, 479 Subarachnoid haemorrhage, 522 Subarachnoid space, 397, 479 Subdural hematoma, 522 Subdural lesion, 510 Subjective angle, 640, 653 Submento vertex view, 694 Subscapular opacification, 374 Substantia propria, 113 Sub tenon, 256 Subtentorial, 510 Subtle glioma of the disc, 437 Subtotal vitrectomy, 358 Succinyl choline, 657 Sugar alcohol, 301 Sujee, 150 Sulcus subtarsalis, 111 Sulpha, 148 Sulpha cetamide, 128, 137, 149 Sulpha methoxazol, 137, 271 Sulpha methoxazole with trimethoprim, 261 Sulpha-cetamide, 129, 739, 758 Sulphadiazine, 261, 432 Sulphite oxidase deficiency, 306, 314 Sulphonamide, 377, 432 Sulphur granules, 726 Sun flower cataract, 329 Sunny side up egg yolk, 444 Sunset appearance of the globe, 20 Super traction of retina, 552 Superior orbital fissure, 676 Supera choroidal lamina, 231 Superficial corneal ulcer, 767 Superficial exudate, 424, 428 Superficial haemorrhages, 370 Superficial keratitis, 49, 123, 134, 172, 203 Superficial punctate, 751, 754, 758, 759 Superficial stroma, 214 Superficial vascularisation, 170, 174 Superficial vascularisation of cornea, 170 Superior cerebellar, 612 Superior cerebellar arteries, 602, 603, 606 Superior cerebellar peduncle, 605 Superior cervical ganglion, 73, 502 Superior colliculum, 596, 601 Superior fornix, 98, 112 Superior limbus, 112 Superior oblique, 593, 597, 598, 670, 676 Superior oblique medial rectus, 674 Superior oblique myokymia, 613, 618 Superior oblique over action, 613, 615, 616 Superior oblique sheath syndrome, 616, 693

817
Superior ophthalmic vein, 87, 398, 675, 677, 704 Superior orbital fissure, 602, 604, 694, 697, 704 Superior orbital fissure syndrome, 607, 686, 690, 763 Superior orbital notch, 691 Superior orbital veins, 713 Superior rectus, 4, 36, 72, 70, 72, 74, 527, 593, 594, 597, 616 Superior rectus palsy, 609 Superior rectus under action, 653 Superior-olivary-nucleus, 604 Suppression, 587, 610, 625, 639, 640, 646, 664, 666 Suppression scotoma, 641, 653, 665 Supra choroidal space, 502 Supra nuclear, 614 Supra nuclear lesions, 613 Supra nuclear palsy, 728 Supra orbital, 200, 752 Supra orbital notch, 673 Supra trochlear, 200 Supra trochlear notch, 673 Suprachoroidal space, 230 Supraciliary space, 230 Suprasellar, 479, 583 Supratarsal fold, 36 Surface ectoderm, 9, 161, 293 Surface probe, 382 Surface saprophyte, 730 Surgery, 657 Surgery in childhood glaucoma, 378 Surgical anatomy of the limbus, 345 Surgical aphakia, 26 Surgical procedures for correction of myopia, 554 Surgical spaces of the orbit, 675 Surgical treatment, 205 Surma, 117, 133, 218 Sursum duction, 595 Sursum version, 595 Suspensory ligaments, 290 Sustained release acetazolamide, 377 Sutural, 296 Sutural cataracts, 298 Suture lines of the orbit, 679 Suturelysis, 385 Sweat glands, 679 Swinging flash light test, 507 Swollen lens, 335, 354 Symblepharon, 59, 81, 121, 125, 148, 730, 742 Sympathelic ophthalmia, 253 Sympathetic, 38, 87, 228 Sympathetic chain, 530, 708 Sympathetic fibres, 511 Sympathetic heterochromia, 237

818
Sympathetic ophthalmia, 239, 240, 243, 258, 259, 272, 275, 328, 526 sympathetic over action, 80 sympathetic plexus, 708 Sympathetic ptosis, 73 Sympathetic uveitis, 258, 273, 277 Sympathizing eye, 273 Sympatho-mimetics, 372 Sympatholytic drugs, 80 Sympathomimetic, 20 Sympathomimetic drugs, 80, 255, 374 Symptomatology of orbital disorders, 684 Symptoms, 92, 150 Symptoms of acute anterior uveitis, 242 Symptoms of aphakia, 560 Symptoms of astigmatism, 565 Symptoms of chronic posterior uveitis, 246 Symptoms of congenital and developmental cataract, 315 Symptoms of corneal opacities, 189 Symptoms of diseases of vitreous, 459 Symptoms of ectopia lentis depend mostly on, 304 Symptoms of heterophoria, 647 Symptoms of hypermetropia, 557 Symptoms of intermediate uveities, 245 Symptoms of lagophthalmos, 62 Symptoms of optic nerve disorder, 520 Symptoms of papilledema, 522 Symptoms of posterior uveitis, 245 Symptoms of retinal diseases in the children, 411 Symptoms of traumatic cataract may be, 326 Synchisis, 461 Synchisis scintillans, 461 Synchisis senilis, 449 Syndactyly, 62, 439 Syndrome of triad, 299 Syndromes, 13 Synechia, 342 Syneresis, 461 Synergist muscle, 594 Synkinetic, 79 Synkinetic movements, 72 Synkinetic movements of the lid, 42 Synoptophore, 611, 639, 644, 640,648, 653 Synoptophore test, 645, 646, 653 Syphilis, 12, 121, 239, 240, 249, 253, 258, 400, 430, 432, 529, 740, 750 Syphilitic iridocyclitis, 250 Syphilitic nodules, 250 Syphilitic uveitis in pediatric age group, 261 Syrengiomyelitis, 522 Syringing, 93 Systemic acetazolamide, 184 Systemic herpes simplex, 430 Systemic involvement, 58 Systemic steroid, 177

PEDIATRIC OPHTHALMOLOGY
Tear osmolarity, 106 Tear quality, 105 Tear quantity, 105 Tear substitutes, 220 Teeth, 679 Telangiectasia, 114, 119, 404, 537 Telangiectasia of retina, 115 Telangiectasis, 415, 422, 424, 488 Telecanthus, 22, 39, 40, 71, 666 Teller acuity card, 29 Temporal bone, 620 Temporal fossa, 674 Temporal long posterior ciliary artery, 6 Temporal pallor, 532, 535 Temporalis muscle, 673 Tempro sphenoidal lobe, 521 Tenonitis, 703, 707 Tenons capsule, 3, 111, 120, 336, 350, 623, 676, 720 Tenons space, 676 Tenosynovitis, 733 Tensilon, 77, 80, 505 Tensilon test, 76, 77, 624 Tentorium, 612 Teratoid, 284 Teratoma, 679, 688, 696, 698, 707 Tersons syndrome, 426, 463 Tertiary position, 594 Tertiary vitreous, 2, 3, 9, 292, 457, 458 Test for abnormal retinal correspondence, 653 Test for suppression, 653 Testes, 746 Tetanospasmin, 728 Tetanus prone injuries, 729 Tetanus prone ocular injuries, 729 Tetanus toxoid, 529, 729 Tetracyclin, 126, 128, 133, 137, 193, 261, 522, 726, 729, 739, 742, 743 Tetrad, 71 Thalidomide, 295 Thayer Martin medium, 127 Thelazia californiensis, 154, 765, 772 Thelazia calipaeda, 154, 765, 772 Thelaziasis, 152, 154, 765, 772 Therapeutic, 28, 263 Therapeutic contact lens, 195 Thermal sclerotomy, 350, 381 Thermo keratoplasty, 165 Thiabendazole, 490 Thick almost organised hypopyon, 177 Thickening of choroids, 274 Thickness of lens, 417 Thickness of the cornea, 207, 417 Third cranial nerve, 502

T
T. corporis, 762 T. facici, 762 T. helper lymphocytes, 267 T. pedis, 762 T-cell mediated, 276 T-cells mediated immunity, 276 Tabes dorsalis, 533 Tachycardia, 374 Tachyphylaxis, 117, 374 Taenia echinococcous (Hydatidcyst), 768 Taenia echinococcus, 765 Taenia glomeratus, 765 Taenia multiceps, 765 Taenia solium, 152, 765, 769 Talengiectasis, 409 Tape-worms, 768 Tapeto meninges, 57 Tapeto retinal dystrophy, 575 Tapetoretinal degeneration, 576 Tarsal, 141 Tarsal conjunctiva, 36, 37, 111, 117, 120, 134, 144, 150 Tarsal cyst, 48, 68 Tarsal fold, 70 Tarsal glands, 111 Tarsal ligaments, 675 Tarsal plate, 35, 37, 97, 110, 117, 675 Tarsitis, 730 Tarsoraphy, 52, 62, 107, 184, 203, 541, 680, 682, 711 Tarsus, 37, 674 Taste test, 93 Tattoo, 188 Tay Sachs disease, 17, 425, 442 Tear, 88, 448 Tear film, 159, 161, 167, 176 Tear film abnormality, 210, 216 Tear film break, 105 Tear film break up, 62, 218 Tear film stability, 105 Tear film status, 80 Tear film study, 176 Tear film substitute, 143, 198 Tear in Descemets membrane, 216 Tear meniscus, 102

INDEX
Third head somite, 600 Third nerve, 675 Third nerve palsy, 563 Third ventricle, 541, 711 Thread worm, 773 Three Cs, 260 Thresh hold disease, 486 Thrombo phlebitis, 705 Thrombocyotopaenic purpura, 119, 299 Thrombocytopenia, 492, 712, 747, 755 Thromboembolic phenomenon, 311 Thrombophlebitis, 526 Thrombosis of retinal vein, 171 Thromobo embolic episodes, 310 Thygesons keratitis, 195 Thymectomy, 78 Thymidine analog, 197 Thymoma, 77 Thymoxamine, 505 Thyroid eye disease, 623, 624 Thyroid myopathy, 417, 621 Thyroid ophthalmopathy, 657 Thyrotoxicosis, 44, 118 Tick ixodes, 742 Time amplitude, 417, 700 Timolol, 375, 376 Tincture iodine, 50, 756 Tinea capitis, 762 Tinitus, 276, 632 Tip cat, 281 Titanium oxide, 60 Titmus fly test, 640 Tobacco dusting, 451 Tobracin, 179 Tobramycin, 129, 179, 180, 737, 743 Toga viruses, 298 Tolosa Hunt syndrome, 607 Tonography, 341 Tonopen, 347 Tonsillitis, 754 Topographic location of sixth nerve lesion, 619 Torch test, 317, 429 Toric bulge, 163 Toric contact lenses, 567 Torsional, 595 Torticolis, 632, 656 Total afferent pupillary defect, 507 Total anterior staphyloma, 186 Total cataract, 296 Total colour blindness, 579 Total hypermetropia, 556 Total hyphaema, 363 Total internal reflection, 164 Total nuclear cataract, 298 Total ophthalmoplegia, 510, 728 Total retinal detachment, 407 Total third nerve palsy, 510 Towens view, 695 Tower skull, 679 Townes view, 694 Toxic amblyopia, 529, 586 Toxic epidermal necrolysis, 738 Toxic neuritis, 535 Toxic neuropathy, 527 Toxic nystagmus, 630 Toxic retinopathy, 534 Toxic vasculitis, 370 Toximia of pregnancy, 434 Toxin, 160, 167 Toxocara, 258, 278, 279, 431, 701 Toxocara canis, 240, 432, 489, 765 Toxocara canis round worm, 264 Toxocara catis, 489 Toxocara infection of uvea, 264 Toxocariasis, 668, 765, 770 Toxopalsma, 764 Toxoplamosis uveitis, 259 Toxoplasma, 267, 750 Toxoplasma gondi, 240, 259, 765 Toxoplasmal chorioretinitis, 263, 428, 431, 535 Toxoplasmal retinitis, 431 Toxoplasmosis, 12, 16, 239, 243, 253, 258, 295, 317, 318, 410, 430, 431, 433, 529, 741, 750, 765 Trabecular dysgenesis of angle of anterior chamber, 344 Trabecular mesh, 247 Trabecular meshwork, 52, 112, 234, 335, 338, 339, 341, 342, 363, 382 Trabecular meshwork develops, 336 Trabeculectomy, 57, 278, 338, 349, 350, 361, 371, 381 Trabeculitis, 52, 202, 245, 247, 360 Trabeculo dysgenesis, 339 Trabeculodialysis, 337, 361 Trabeculodysgenesis, 337 Trabeculoplasty, 385 Trabeculostomy, 379, 381, 980 Trabeculotome, 350 Trabeculotomy combined with trabeculectomy, 349 Trachoma, 23, 104, 117, 119, 120, 133, 139, 140, 142, 147, 149, 170, 172, 173, 178, 207, 756, 757 Trachoma dubium, 134 Trachomatis, 133 Trachomatous entropion, 63 Trachomatous inflammation, 135 Trachomatous scarring, 135 Trachomatous trichiasis, 135 Traction detachment, 245, 247, 433

819
Traction displacement of macula, 265 Tractional, 453 Tractional retinal detachment, 453, 454 Train nystagmus, 632 Tram track, 57 Trans ethmoidal, 541 Trans nasal, 541 Trans-illumination, 338, 412 Transcleral cyclocryo, 369 Transcranial, 711 Transducer, 417, 699 Transferase galactokinase, 300 Transient corneal haze, 299 Transient myopia, 373, 377 Transient neonatal myasthenia, 76 Transilluminate, 237 Transnasalendoscope, 675 Transplacental, 431, 766 Transplacental factors, 12 Transplacental spread, 430 Transpupillary, 382 Transscleral, 382 Transverse myelitis, 746, 752, 753 Trauma, 13, 160, 188, 249, 425, 462, 508 Trauma to orbit, 118 Traumatic, 151 Traumatic aphakia, 452 Traumatic cataract, 324, 407, 433, 475, 484 Traumatic cysts, 151 Traumatic glaucoma in children, 354 Traumatic maculopathy, 516 Traumatic miosis, 74 Traumatic optic atrophy, 535 Traumatic optic neuropathy, 45, 508, 533, 535 Traumatic paralysis of third nerve, 606 Traumatic phacogenic glaucoma, 366 Treacher collins syndrome, 682 Treacher collin, 39 Treatment of acute uveitis, 254 Treatment of ecchymosis, 45 Tremulouness of iris, 305, 311, 366 Tremulous, 561 Tremulousness of lens, 345 Treponema pallidum, 740 Tretinoin, 220 Triamcinolone, 713 Triamcinolone acetonide is, 48 Triamsonolone, 361 Triangular head, 680 Triatoma, 768 TRIC, 133 Tricarbocyanine, 416

820
Trichiasis, 40, 41, 45, 51, 52, 63, 121, 125, 148, 173, 200, 682, 730 Trichinellaspiralis, 765 Trichinosis, 765 Trichlor acetic acid, 181 Trifluorothymidine, 197 Trifluridine, 197, 198, 199 Trigeminal, 38, 43, 87, 114, 160, 194, 200, 228, 596, 597, 752 Trigeminal ganglion, 131, 753, 754 Trigeminal nerve, 51, 171, 175, 576 Trigeminal neuritis, 172 Trigeminal reflex, 504 Trigger mechanism, 194 Trigonocephaly, 680, 695 Trilateral retinoblastoma, 474, 479, 482 Trimethoprim, 271, 736 Trimethoprimesulpha methoxazole, 726, 727, 730, 744 Triple sulpha, 261, 432 Trismus, 728 Tritanomalous, 578 Tritanomaly, 578 Ptritanopia, 578 Trivalent antitoxin, 729 Trochlea, 593, 598, 601, 612, 617, 676, 677, 691, 704 Trochlear fossa, 673 Trochlear nerve, 603, 674 Trophic, 201 Trophic ulcer, 196, 201 Trophozoite, 208 Tropias, 571 Tropicamide, 181, 208, 254, 318, 506 Troticollis, 633 True congenital glaucoma, 344 True membrane, 121, 123, 148 True membranous conjunctivitis, 125 True polycoria, 27, 229 True proptosis, 348 True ptosis, 68 Trypanosoma, 764 Trypansoma cruzi, 768 Trypansoma gambiense, 768 Trypansome chancre, 768 Trypansomiasis, 768 Tsetse fly, 768 Tubercle bacilli, 146 Tubercles, 249, 731 Tubercular chorio retinitis, 431 Tubercular choroiditis, 260 Tubercular conjunctivitis, 731 Tubercular meningitis, 508, 732 Tubercular nodule, 250, 263, 732 Tubercular protein, 144 Tubercular sinus, 732 Tuberculoma, 263, 522, 731, 732 Tuberculosis, 97, 121, 144, 149, 171, 206, 239, 240, 243, 253, 258, 430, 432, 433, 526, 529, 702, 764 Tuberous sclerosis, 55, 58, 492, 542 Tubular vision, 436 Tucking, 617 Tumescent, 367 Tumour, 19 Tumour node metastasis system, 483 Tumour related glaucoma, 368 Tumours of optic nerve, 538 Tumours of optic nerve head, 542 tumours of the optic nerve, 417 Tunica vasculosa lentis, 4, 6, 233, 235, 293, 457, 458 Tunica vesculosa lentis, 458 Turbid aqueous, 242 Turners syndrome, 315 Two focal lines, 563 Tylosis, 45, 98 Tympanic membrane, 82 Type I diabetes, 434 Type I herpes simplex, 194 Type of esophoria, 647 Type one allergy, 138 Types of anisometropia, 567 Types of astigmatism, 564 Types of exophoria, 648 Types of heterophoria, 644, 650 Typhoid (enteric fever), 743 Typical coloboma of eye, 8 Typical coloboma of uvea, 1 Typical retinitis pigmentosa, 435 Tyrosinase, 401 Tyrosinase negative, 236, 400, 401 Tyrosinase positive, 236, 400, 401 Tyrosine, 400, 401 Tyrosine hydroxylase, 236

PEDIATRIC OPHTHALMOLOGY
Unconventional outflow, 341 Uncorrected errors of refraction, 116 Uncorrected hypermetropia, 32 Under action of inferior oblique, 71 Under action of orbicularis, 76 Unformed hallucination, 581 Unharmonious ARC, 639 Unifocal, 563 Unilateral abducent palsy, 656 Unilateral Argyll Robertson pupil, 509 Unilateral buphthalmos, 695 Unilateral diplopia, 305 Unilateral ectopia lentis, 304 Unilateral facial palsy, 82 Unilateral fourth nerve palsy, 611 Unilateral loss of vision, 29 Unilateral retinitis pigmentosa, 438 Unilateral sub acute neuro retinitis, 265 Uninary homocystine, 309 Uniocular aphakia, 567, 569 Uniocular diplopia, 164 Unoprostane, 376 Unstratified muscles, 228 Untowards effect of epinephrine, 374 Up gaze paralysis, 81 Upbeat nystagmus, 634 Upper fornix, 87, 110 Upper lid, 34 Upside down ptosis, 37, 74, 511 Urea, 103, 372, 378 Urease, 743 Uremia, 215 Urethritis, 133, 271, 733 Urticaria, 139 Uses of ERG, 419 Ushers syndrome, 439 Uthoff phenomenon, 526 Uvea, 4, 56 Uveal circulation, 6 Uveal dystrophies, 576 Uveal effusion syndrome, 723 Uveal meshwork, 336 Uveal pigments, 242 Uveal reaction, 191 Uveal system, 6 Uveascleral outflow, 375 Uveitis, 24, 44, 52, 170, 214, 314, 326, 407, 422, 728, 744, 758, 759, 760, 763, 766, 767 Uveitis glaucoma haemorrhage syndrome, 356 Uveitis glaucoma hyphaema, 357 Uveitis in children, 238 Uveitis in herpes zoster, 201 Uveo retinal coloboma, 515

U
UGH, 357 Uhthoff phenomenon, 527 Ulcer, 45, 62, 195, 216 Ultra filtration, 340 Ultra sonography, 488 Ultra violet, 161, 382, 577 Ultra violet ray, 194 Ultrasonic cycloablation, 381 Ultrasonic pachymetry (Pachometry), 417 Ultrasonography, 274, 281, 407, 412, 416, 452, 455, 474, 553, 699, 769 Ultrasound, 349 Ultraviolet light, 59, 173 Umblicated vesicle, 133 Uncal herniation, 606

INDEX
Uveo scleral out, 252 Uveo-scleral outflow, 366 Uveokeratitis, 52 Uveoscleral, 341 Uveoscleral channel, 231 Uveoscleral outflow, 373, 375, 376 Uveovertex, 341 Uyemuras disease, 444 Venous obstruction, 416 Venous phase, 415 Venous stasis, 524 Ventral ophthalmic artery, 6 Ventricle, 59, 517 Ventricular decompression, 613 Ventriculo peritoneal, 525 VEP, 16 Vergence, 594, 595, 596 Vergence eye movement, 595 Vericella, 199, 200 Vericella herpes zoster virus, 752 Vericella zoster virus, 131 Vernal catarrh, 117, 170, 328 Vernal conjunctivitis, 120, 173 Vernal kerato conjunctivitis, 139, 206 Vernier acuity, 586 Versions, 594, 595 Vertebrae, 622 Vertex vein, 599, 720 Vertical angle kappa, 612 Vertical gaze palsy, 613 Vertical nystagmus, 614 Vertical prism test, 640 Vertical school, 669 Vertical squint, 610, 681 Vertical tropia, 79 Vertigo, 275, 276, 632, 651 Vescicle formation, 148 Vescicles, 200 Vesciculo bullus stage, 148 Vessels of tarsal conjunctiva, 160 Vestibular, 596 Vestibular and cochlear reflex, 504 Vestibular disturbance, 439 Vestibular evoked nystagmus, 632 Vestibular nystagmus, 62, 621, 629 Vestibulo ocular movements, 594 Vetrectomy, 261 Vidarabine, 197 Video cassette recorder, 416 Vincristine, 482, 708, 709 Viral conjunctivitis, 120 Viral infection, 82 Viral infection of the lids are, 49 Viral retinitis in children, 429 Viral ulcers, 175, 183 Viridans, 739 Virtual erect image, 166 Virtual image, 412, 413 Virus, 745 Visceral involvement, 56 Visceral larva migrance, 265, 489, 770 Visco elastic, 279, 356, 364 Viscoelastic substance, 274 Visible pulsation, 691 Vision, 79, 143, 175

821
Vision in optic neuritis, 527 Vision of a new born, 14 Vision of neonate, 16 Visual acquity, 79, 421 Visual aphasia, 574, 580 Visual axis, 593, 596 Visual cells, 395 Visual cortex through tecto bulbar tract, 601 Visual evoked occipital potential, 16 Visual evoked response, 412, 420, 586 Visual hallucination, 574, 581 Visual purple, 393 Visual response, 15 Visually challenged, 168 Visually evoked potential, 29 Visually provoked response, 21 Visuocortical tract, 529 Visuscope, 641 Vital dye staining, 105 Vital stains, 176 Vitamin A, 198, 217, 395 Vitamin A deficiency, 111, 138, 172, 173, 179, 183, 189, 238, 418, 576, 748 Vitamin A deficiency disease (VAD), 173 Vitamin D, 297 Vitamin D toxicity, 215 Vitamin E deficiency, 487 Vitelli form, 444 Vitelli form macular dystrophy, 444 Vitelliform dystrophy, 435 Vitiligo, 45, 274, 275, 402 Vitrectomy, 266, 272, 278, 279, 281, 282, 322, 354, 427, 433, 488, 727, 728, 743 Vitreitis, 246 Vitreo retinal defects, 339 Vitreo retinal degeneration, 464 Vitreo retinal dystrophies, 435 Vitreo retinal pathology, 417 Vitreo-retinal attachment, 457 Vitreous, 10, 153, 230, 244, 305, 393 Vitreous abscess, 278, 280, 760 Vitreous bands, 58, 247, 252, 324, 405, 453 Vitreous base, 230, 456 Vitreous cells, 242, 274 Vitreous chamber, 230, 290, 335 Vitreous changes in chronic anterior uveitis, 252 Vitreous debris, 245 Vitreous degeneration, 461, 549 Vitreous detachment, 446, 461 Vitreous disturbance, 462 Vitreous flare, 246

V
V pattern, 611, 655 V phenomenon, 616 Vaccines, 528 Vaccinia, 745, 751 Vacuoles, 328 Vaginitis, 762 Vagotonic reflex, 504 Valaciclovir, 197 Valproate, 635 Valselva maneuver, 692 Valselva procedure, 540 Valves, 350 Vancomycin, 192, 261, 727, 739, 740 Vanishing optotypes, 30 Variants of a typical retinitis pigmentosa, 437 Variants of Duanes retraction syndrome, 623 Variation of retinitis pigmentosa, 435 Varicella, 49, 429, 745, 751 Varicella Zoster Viral Keratitis, 199 Varicella zoster virus, 49, 193, 751 Variola, 745 Various methods available to diagnose ARC, 640 Various methods employed to lower sudden rise of i, 380 Various uses of laser in glaucoma, 384 Varnished macula, 444 Vasa hyaloidea propria, 6 Vascular causes of white reflex, 477 Vascular occlusion, 416 Vascular retinopathies in children, 434 Vascular retinopathy, 383 Vascularisation, 148, 188 Vascularisation of cornea, 26, 135, 183, 201, 768 Vasculitis, 148, 246, 260, 274, 428, 429, 705 Vasocons-trictors, 118, 139, 140, 242, Vasoformative, 169 Vector control, 137 Venae verticosae, 5, 676, 720 Venography, 693, 713 Venous congestion, 685 Venous drainage of uvea, 232

822
Vitreous floaters, 246, 459, 552 Vitreous haemorrhage, 28, 260, 326, 365, 427, 445, 461, 462, 552, 577, 766 Vitreous haze, 246, 247 Vitreous in AC, 169 Vitreous membrane, 742 Vitreous opacities, 262, 437, 460, 558 Vitreous precipitates, 260 Vitreous seeding, 473, 478, 481 Vitreous volume, 377 Vitrial causes of diminished vision, 460 Vitritis, 245, 263, 432, 459, 489, 732, 761 Vogt Koyanagi, 259 Vogt Koyanagi form, 276 Vogt Koyanagi Harada syndrome, 253, 275 Vogt-Koyanagi-Harada, 240, 258 Volk, 245, 412 Voluntary nystagmus, 630, 633 Von Hippel Lindaus disease, 55, 151, 407, 476, 492 Von Hippel-Lindau, 405 Vortex, 421 Vortex veins, 230, 231, 232, 233 Vossius ring, 326 Wegner granulo-matosis, 526 Weill-Marchesani syndrome, 302 Weiss ring, 450, 462 Wernickes hemianopic pupil, 507, 508 Wessely ring, 203 Western blot test, 268 White forelock syndrome, 683 White granular dots, 750 White iritis in girls, 241 White KP, 196 White light, 577 White reflex, 153, 236, 265, 316, 403, 407, 411, 433, 473, 485, 488, 492 White reflex in pupil, 16, 322, 473 White reflex in pupillary, 28, 283, 284, 317, 323, 473, 475, 771 White uveitis, 269 White uveitis syndrome, 258 White with pressure, 445, 446 White without pressure, 446 Whitening of scleral spur, 366 Whitnall ligament, 9, 36, 37, 87, 676 Whitnall tubercle, 37 WHO, 134 WHO grading, 135 Whooping cough, 23, 119, 730 Wide angle, 307 Wide angle glaucoma, 356, 370 Widening of ciliary band, 366 Width of the inter palpebral fissure, 79 Wilms, 19 Wilms tumor, 235, 687, 688, 707, 708 Wilsons disease, 301 Window reflex keratoscope, 166 Windows reflex, 175 Winged scapulae, 308 Wise lens, 383 Wolfring, 87 Word blindness, 580 Worm infection, 219 Worth four dot test, 568, 640, 641, 653, 663 Worth ivory ball, 30 Wyburn-Mason syndrome, 55, 405, 409

PEDIATRIC OPHTHALMOLOGY

X
X linked retino chisis, 420 X-ray, 326 X-ray of orbit, 540 X-ray optic foramen, 540 Xanthophyll, 382, 383, 384, 394, 415, 416 Xanthopsia, 577 Xenon, 383, 409 Xeroderma pigmentosa, 54, 59, 151 Xerophtalmia secondary, 106 Xerophthalmia, 106, 121, 125, 133, 142, 168, 172, 190, 278, 404, 576, 730, 748 Xerophthalmia and Keratomalacia, 216 Xerophthalmia scar, 218 Xerophthalmic fundus, 218 Xerosis, 105, 148, 165, 216, 440 Xerosis bacilli, 218 Xylocaine, 105, 106, 176, 255, 773

Y
Y sutures, 293 YAG laser, 211 Yeast, 760, 762 Yellow grey barrier filter, 414 Yellow prickly poppy, 369 Yoke muscles, 594 Yto megalo virus, 193

W
Waarden burg syndrome, 40, 402, 423, 682, 683 Waardenburg, 237 Wagner, 453 Wagners disease, 446, 464, 465 Wagners vitreoretinal dystrophy, 435 Wall eyed bilateral inter nuclear ophthalmoplegia, 605 Walls of the orbit, 697 Watch glass, 159 Waters view, 693 Watery discharge, 24, 120 Watts, 382 Waxy pale, 436, 437 Waxy white, 534 Webers syndrome, 73 Webino, 605

Z
Zeis, 38 Zinc, 537 Zinc oxide, 60 Zinc sulphate, 735 Zonular cataract, 297 Zonule, 9, 230, 303, 305, 456, Zonules of the lens, 2, 458 Zoonosis, 727, 741, 767, 770 Zoster, 173, 174 Zoster varicella virus, 50 Zygomatic, 34, 673, 674 Zygomatic arch, 694 Zygomatic bone, 674 Zygomatic nerve, 674, 677

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