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Pediatric Ophthalmology


Pediatric Ophthalmology

Dr. P.K. Mukherjee


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



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

ISBN : 978-81-224-2310-5


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


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


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


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




Outline of Development of the Eye

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 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 :


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.


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


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


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


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.


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.



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


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.

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


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.


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.


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

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


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.


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



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.


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.

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.




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.



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 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.



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.


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,



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.


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


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.


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.



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.



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.


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



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



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



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



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



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.



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



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



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



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.


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.



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.



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.

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.



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.


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.


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



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 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.


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



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



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


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


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



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



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.



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.


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



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



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 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.



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



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.


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.


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



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.



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



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



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



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.



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.



(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.




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



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.

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.



(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



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



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



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.


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



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 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



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.

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 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.



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-



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.


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-



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.

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



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.



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.


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



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.



(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



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.



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.



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.



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


(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



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:


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.



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.



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.



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.



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.



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


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.



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.

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.



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.



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.


Disorders of Lacrimal System in Children

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



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



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 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



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



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.


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.



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


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 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



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

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



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 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.



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.



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.


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.

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



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

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


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.

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.



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



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.



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.



(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.


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.



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.


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.



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.


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.



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



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.



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.

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.



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.



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.


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




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.



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.



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



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



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.


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



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.



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.


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.



(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



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.



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.



(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.

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.



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



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.


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



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



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 + + + + + + +



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



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



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



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.



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



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



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.



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


Trachoma Stage III


Trachoma Stage IV




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



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 :



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)


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



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.



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



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.


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



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



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





On set.




Acute allergic




Seasonal allergic- Any conjunctivitis



Perennial allergic -Doconjunctivitis




Vernal kerato conjunctivitis

Under 20 years

Males more

5. Contact lens -Dopreservative

Atopic kerato conjuctivitis







Giant papillary conjunctivitis



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.




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



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



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



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.



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



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.



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



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.



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.

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.



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.



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.



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.



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.


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



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



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


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.



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.



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.



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 :


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.



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



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



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



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.



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


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



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.



(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



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.



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



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



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.



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.



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.



(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



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.



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.



(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.



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.



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



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 :


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.



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.


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.



(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



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



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.



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.



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



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.



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 :


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



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.



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



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.



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



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.



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.



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.



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



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



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.



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.



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.



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



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.




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



Autosomal dominant Autosomal dominant

Mild to severe Severe 6/60 or less



3-4 years




10 yrs

Autosomal dominant

Gradual loss


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



Autosomal dominant




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


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.



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



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



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


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



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.



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.



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

1. Nema H.V. : The cornea and limbus in Anatomy of the eye and its adnexa. Second edition, p-3-9, Jay Pee Brothers, New Delhi, 1991. 2. Doughman D. : Corneal physiology in Principle and practice of ophthalmology. Edited by Pyeman G.A., Sander D.R. and Goldberg M.F. First Indian edition. p-360.368. Jay Pee Brothers, New Delhi, 1987. 3. Meharotra A.S. : Applied anatomy of the eye and adnexa in Modern ophthalmology. Second volume. 1st edition. p-105-1056. Edited by Dutta L.C., Jay Pee Brothers, New Delhi, 2000. 4. Ilene K. Gipson : Anatomy of the conjunctiva, cornea and limbus in The cornea. Third edition p-3-25. Edited by Smolin G. and Thoft R.A., Lippincot Williams and Wilkins, Philadelphia, 1994. 5. Deborah Pavan Langston : Cornea and external eye disease in Manual of ocular diagnosis and therapy. Fifth edition 67-68. Lippincott Williams and Wilkins, Philadelphia, 2002. 6. Duke Elder S. : System of ophthalmology. Vol. III, Part I. First edition. p-137, Henry Kimpton, London, 1963. 7. Nancy Anderson Hamming and Apple D. : Anatomy and embryology in Principle and practice of ophthalmology. First Indian edition, edited by Pyeman G.A., Sanders D.R. and Goldberg M.F. p-18-19, Jay Pee Brothers, New Delhi, 1987. 8. Banumathy S.P. : Development of cornea and sclera in Anatomy of the eye. p-165, Arvind Eye Hospital, Madurai. 9. Nema H.V. : Development of eye and its adnexa in Anatomy of the eye and its adnexa. Second edition. p-129, Jay Pee Brothers, New Delhi, 1991. 10. Duke Elder S. : System of ophthalmology. Vol. III, Part II. p-164-169, Henry Kimpton, London, 1963. 11. Basak S.K. : Physiology of the eye in Essentials of ophthalmology. Second edition. p-24-25, Current Books International, Calcutta, 1999. 12. Khurana A.K. : Diseases of the cornea in Ophthalmology. Second edition. p-114-115, New Age International, New Delhi, 2000. 13. Sharma P. : Cornea and its diseases in Essentials of ophthalmology. First edition. Modern Publishers, New Delhi, 2000. 14. Ramanjeet Shihota and Radhika Tandon : Metabolism of ocular tissues in Parsons diseases of the eye. Nineteenth edition, p-20-21, Butterworth Heineman, Oxford, 2003. 15. Edelhauser, H.F., Geroski D.H. and Ubels J.L. : Corneal transparency in The Cornea. Third edition, p-28-32. Edited by Smolin G. and Thoft R.A., Lippincot Williams and Wilkins, 1994.



16. Wilson F.M. : Congenital anomalies of cornea and conjunctiva in The Cornea. Third edition, edited by Smolin G. and Thoft R.A. p-535-550, Lippincot Williams and Wilkins, Philadelphia, 1994. 17. Duke Elder S. : Symptom of ophthalmology. Vol. III, part II. Edited by Duke Elder S. p-497-525, Henry Kimpton, London, 1964. 18. Nema H.V. : Anomalies of cornea in Anatomy of the eye and its adnexa. Second edition. p-145-146, Jay Pee Brothers, New Delhi, 1991. 19. Rabinowitch V.S. and Rasheed K. : Pellucid marginal degeneration in Current ocular therapy. Fifth edition. Edited by Fraunfelder F.T., Roy F.H. p-371-372, W.B. Saunders Company, Philadelphia, 2000. 20. Kanski J.J. : Pellucid marginal degeneration in Clinical ophthalmology. Second edition p-119, Butter Worth, London, 1987. 21. Kennedy R.H., Bourne W.M. and Kyer J.A. : A 48 year clinical and epidemiologic study of keratoconus. Am.Jr.Oph. : 101, 267-273, 1986. 22. Nesburn A.B. and Kenney M.C. : Keratoconus in Current ocular therapy. edition 5. Edited by Fraunfelder F.T. and Roy F.H. p-364-365, W.B. Saunders Company, Philadelphia, 2000. 23. Nagy K.A. and Abel R. : Keratoconus in Current ocular therapy. Edition 4. Edited by Fraunfelder F.H. and Roy F.H. p-501-502, W.B. Saunders Company, Philadelphia, 1995. 24. Duke Elder S. : System of ophthalmology. Vol. III, Part II. First edition. p-964-974, Henry Kimpton, London, 1964. 25. Duke Elder S. : System of ophthalmology. Vol. III, Part II. First edition. p-509, Henry Kimpton, London, 1964. 26. Raber I. : Keratoconus in Ophthalmic secrets. Edited by Vander J.E. and Gault J.A. First Indian edition p-95-101, Jay Pee Brothers, New Delhi, 1998. 27. Samolin G. : Corneal dystrophies and degeneration in Cornea. Third edition. Edited by Samolin G. and Thoft R.A. p-522-524, Lippincot Williams and Wilkins, Philadelphia, 1994. 28. Rizutti A.B. : Diagnostic illumination test for keratoconus. Am. Jr. Oph. 70 : 141, 1970. 29. Tuft S.J., Moodaley L.C. and Gregory W.M. : Prognostic factors for progression of keratoconus. Ophthalmology 1012 : 439, 1994. 30. Stone Chiper K.G. : Corneal neovascularisation in Current ocular therapy. Fourth edition. Edited by Fraunfelder F.T. and Roy F.H. p-484-485, W.B. Saunders Company, Philadelphia, 1995. 31. Duke Elder S. : System of ophthalmology. Vol. VIII, Part II. p-676-691, Henry Kimpton, 1964. 32. Sridhar Rao B. : Congenital glaucoma in Modern ophthalmology. Part I, Second edition. p-454, Edited by Dutta L.C., Jay Pee Brothers, New Delhi, 2000. 33. Kanski J.J. : Congenital glaucoma in Clinical ophthalmology. Second edition. p-223224, Butterworth, London, 1989.



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.



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.



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.



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.


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




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.



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.



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.



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



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.



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



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.



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.

236 Coloboma of choroids


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.



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.



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



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.

240 3. Etiological


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.



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.



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.



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



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



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.



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.



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.

248 Corneal complications


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



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.



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.



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



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



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.



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



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,



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



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.



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.



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.



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.



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



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.



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



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.



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.

266 Differential diagnosis


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.



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



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.



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



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.



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.



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



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



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.



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.



(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



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



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,



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.



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



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.



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.



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.



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.



1. Miller S.J.H. : Parsons disease of the eye, 17th edition, p. 151, Churchill Livingstone, London, 1984. 2. Nema H.V. : Anatomy of the eye and its adnexa, 2nd edition p. 19 to 30, Jay Pee Brothers, New Delhi, 1991. 3. Jogi Renu : Basic ophthalmology, 1st edition, p. 148, Ajeet Publication New Delhi, 1994. 4. Miller S.J.H. : Parsons disease of the eye, 17th edition, p. 6, Churchill Livingstone, London, 1984. 5. Duke Elders S. : System of ophthalmology, Vol. III, p. 167-184, Henry Kimpton, London, 1963 6. Khurana A.K. : Ophthalmology, 2nd edition, p. 152-156, New Age International (P) Limited, 2000, New Delhi. 7. Nancy Anderson Hamming, Apple D. : Anatomy and embryology of eye in Principle and practice of ophthalmology, Vol. 1, First Indian edition, pp 33-44, Jay Pee, Brothers, New Delhi, 1987. 8. Banumathy S. P. : Anatomy of the eye, 1st edition p. 52, Arvind Eye Hospital, Maduri. 9. Ahmed E : A text book of ophthalmology, First edition, p. 24, Oxford University Press, Calcutta, 1993. 10. Duke Elders : System of ophthalmology, Vol. III, Part I, pp. 158 to 162, 1st edition Henry Kimpton London 1963. 11. Vaughan D., Asbury T. : General Opthalmology tenth edition, p. 10-11, Lange Medical Publication, California, 1983. 12. Duke Elders : System of ophthalmology Vol. III part I, p. 36-40 1st edition. Henry Kimpton London 1963. 13. Duke Elders : System of ophthalmology Vol. III Part II, p. 573, First edition. Henry Kimpton London, 1963. 14. Walton D.S. : Aniridia in Current ocular therapy, 5th edition, p. 508-509, Edited by Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadelphia, 2000. 15. Deborah Pavan Langston : Pediatric ophthalmology in Manual of ocular diagnosis and therapy. Third edition, p. 290. 16. Duke Elders : System of ophthalmology, Vol. III, p. 775, Ist edition, Henry Kimpton, London, 1963. 17. Nema H.V. : Anatomy of the eye and its adnexa, Second edition, p-150, Jay Pee Brothers, New Delhi 1991. 18. Biswas J. : Disease of uveal tract in Modern ophthalmology, Vol. 2, Second edition, p. 559, Jay Pee Brothers, New Delhi, 2000. 19. Sorsby A. : Albinism in Modern ophthalmology, Vol. 2, p. 37-39, Butterworth London, 1963.



20. Kanski J.J. : Heriditory disorders of the retina and choroids in Clinical ophthalmology, 2nd edition, p. 385-386, Butterworth, London, 1989. 21. Sharma P. : Essentials of ophthalmology, first edition, p. 155, Modern Publication, New Delhi, 2000. 22. Ahmed E. : Disease of the uveal tract in A text book of ophthalmology. First edition, p. 227, Oxford University Press, Calcutta, 1993. 23. Miller S.J.H. : Disease of the uveal tract in Parsons disease of the eyes. Seventeenth edition, p. 157, Churchil Livingstone London, 1984 24. Carolene R Baumal : Granulomatous uveitis in ophthalmology secretes. edited by Vander JF and Gault JA. First Indian edition, p. 258, Jay Pee Brothers New Delhi, 1998. 25. Debora Pawan Langston : Uveal tract iris ciliary body and choroids in Manual of ocular diagnosis and therapy, 3rd edition, p. 174-213. 26. Gittinger J.W. : Asdorian G KToxoplamosis in Manual of clinical problems in ophthalmology. First edition, p. 147, Little Brown and Co., Boston, 1998. 27. Sharma P. : Uvea and its diseases in Essentials of ophthalmology. First edition, p. 156-175, Modern Publishers, New Delhi, 2000. 28. Coles R.S. : Uveitis in Modern Opthalmology, Vol 4, edited by Sorsby A. First edition p. 636-686. Butterworth, London 1964 29. Wood A.C. : Endogenous inflammation of the uveal tract, Williams and Willkino, Baltimore 1961 30. Hogan M.H., Kimura S. J. and Thygeson P. : Signs and Symptoms of uveities I. Anterior uveitis AJO 47-155, 1959. 31. Tesseler M. : Uveitis in Principle and practice of ophthalmology Vol. 2, First Indian edition, Edited by Peyman G A, Sanders D and Goldberg FM, p. 1554-1566, Jay Pee Brothers, New Delhi 1987. 32. Biswas J. : Intermediate uveitis (Pars Planitis) in, Vol. 2, Second edition, Edited by Dutta L C, p. 571-580, Jay Pee Brothers, New Delhi 2000. 33. Kanski J.J. : Uveitis in Clinical ophthalmology, second edition p. 136-177, Butterworth, London, 1989. 34. Nussenblat R.B. : et al. Standardisation of vitreal inflammatory activity in intermediate and posterior uveitis. Opthalmology, 92-467-471, 1985. 35. Duke Elders in Systems of ophthalmology, Vol. III part II p. 893-902, Henry Kimpton, London, 1963. 36. Doughman D.J. : Olson G.A. and Nolan S. : Experimental band Keratopathy, Arch Oph 81: 264; 1969. 37. Barua C.K. and Dutta L.C. : Corneal degeneration and dystrophies in Modern Opthalmology Vol. 1, Second edition, edited by Dutta L C, p. 183-184, Jay Pee Brothers New Delhi, 2000.



38. Tran D.B. and Schanzlin D.J. : Corneal and conjunctival calcification in Current of ocular therapy. 5th edition page 329-330 edited by Fraunfelder F.T., Roy F.H., Randall J., W. B. Saunders Company, Philadelphia, 2000. 39. OBrat DPS, Gantry D S et al. : Treatment of band keratopathy by excimer photo keratectomy BJO 77: 702-708 1993. 40. Igerscheimer J. : Spirochaetal infection-Aquired syphilis in Modern Opthalmology Vol. II, edition second, p. 217-219 Butterworth London 1963. 41. Dutta L.C. : Ophthalmology : Principles and practice, 1st edition, p. 121-122, Current Book International, Calcutta. 42. Rosenbaum J.T. and George R.K. : Uveitis in Current ocular therapy, 5th edition p. 519-521. edited by Franfelder F.T. and Roy F.H. and Randall J., W.B. Saunders Company Philadelphia, 2000. 43. Knox D.L. : Disorders of the uveal tract in Paediatric Ophthalmology, Vol. I, second edition, edited by Harley R.D., W.B. Saunders Company, Philadelphia 1983. 44. Biswas J. and Wagle A. : Anterior uveitis and arthritis in Modern Ophthalmology. Vol. 2, Edited by Dutta L.C., p. 566-571, Jay Pee Brothers, New Delhi, 2000. 45. Vaishali Gupta and Gupta A : Ocular toxoplasmosis in Modern Ophthalmology, Vol. 2, edition 2, edited by Dutta L.C. p. 580-585, Jay Pee Brothers New Delhi 2000. 46. Shaffer D B : Paediatric ophthalmology in Text books of ophthalmology. 9th Edition, edited by Scheic H.G. and Albert, D M, WB Saunders company Philadelphia, 1977. 47. Shalaby I.A. and Dunn J.P. : Acquired and congenital syphilis in Current therapy. Ed 5, Edited by Fraunfelder F.T., Roy F.H., Randell J., p. 2-5, W.B. Saunders Company Philadelphia, 2000. 48. Wood A.C. : Chronic bacterial infection: Ocular tuberculosis in Modern ophthalmology. Vol II, first edition, edited by Sorsby A., p. 116-153, Butterworth, London, 1963. 49. Pamela S. Chavis : Tuberculosis in Current ocular therapy, fifth edition, edited by Fraunfelder F.T., Roy F.H. and Randel J., p. 78-81, W.B. Saunders Company Philadelphia 2000. 50. Helm C.J. : Holland G.N. and Ocular tuberculosis, Survey ophthalmology 38 : 229256 1993 51. Gittinger J.W. : Herpes simplex keratitis in Manual of clinical problems in ophthalmology, first edition, edited by Gittinger J.W. and Asdaurion G.K., p. 46-47, Little Brown and Co. Boston, 1998. 52. Gillespie S.H. : Ocular toxocariasis in Current ocular therapy, edition 5, p. 97-98, edited by Fraunfelder F.T., Roy F.H. and Randall J., W.B. Saunders Company Philadelphia 2000. 53. Gupta V. and Gupta A. : Pediatric posterior uveitis in Modern Ophthalmology. Vol. II, edition two, edited by Dutta L.C., p. 561-565, Jay Pee Brothers, New Delhi 2000. 54. Asdourian G.K. : Toxocariasis in Manual of Clinical Problems in Ophthalmology, first edition, edited by Gittinger J.W. and Asdourian G.K., p. 149-151, Little Brown, Boston 1998.



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.



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.


Disorders of Lens in Children

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.

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.




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.



(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.



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.


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.


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



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 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.



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.


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


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



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.


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.



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 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.



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.


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



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.



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)


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



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.


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.



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 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



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.


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.



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.



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.


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.



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.



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.



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 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



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.



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.


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



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

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


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




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.


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

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



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.



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 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.



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.


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.



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.


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



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.

320 3. Surgery


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.



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.




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.



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.



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.


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.



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 subepitheli