Pediatric Ophthalmology

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Pediatric Ophthalmology

Dr. P.K. Mukherjee
MS

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

NEW AGE

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

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

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

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

(xi)

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CHAPTER

1

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

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

2 The development of lens has two distinct stages :

PEDIATRIC OPHTHALMOLOGY

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

OUTLINE OF DEVELOPMENT OF THE EYE

3

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 Bownan’s 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.

The mesoderm gives rise to strom of iris. It develops from the lips of the optic cup.4 The Development of Extra Ocular Muscles13 PEDIATRIC OPHTHALMOLOGY All voluntary muscles develop from paramedian mesoderm. The levator develops from the dorsomedial aspect of the superior rectus. The ciliary epithelium is neuro ectodermal in origin. The tip of the cup develops into two layered pigment epithelium of the iris. If there is less of pigment the iris takes a blue colour. Its . 3. It is mostly mesodermal except the Bruchs membrane that has both ectodermal and mesodermal origin. The anterior layer is the continuation of outer layer of optic cup while the posterior layer is continuation of inner layer of optic cup. Bruch’s membrane is partly neuro ectodermal and partly mesodermal. It lacks stroma as in iris or musculature of ciliary body. oblique and circular muscles. It develops between the lens and surface ectoderm. and partly from mesoderm. Initially this stretches across the lip of the optic cup. Melanocytes also arise from ectoderm. connective tissue and blood vessels. The pupillary membrane is a transient structure that lateron atrophies and disappears. The Iris. The Choroid. From each will develop muscles that are supplied by different cranial nerves i. epithelium of ciliary body. The nerves grow from brain towards individual muscle mass marked for each nerve. The ciliary muscle develops from paraaxial mesoderm. Some times fine vessels are visible on the iris of a new born. third. It does not have an epithelium like the former two. The Ciliary body. Incomplete absorption of the pupillary membrane is called persistent pupillary membrane.e. The central part of the pupillary membrane is eventually completely absorbed forming the pupil. fourth and sixth. The structures that originate from neuroectoderm are pigment epithelium of iris sphinter and dilator pupilae of iris. The choroid is mostly vascular with connective tissue inbetween. The peripheral part of the pupillary membrane gets vascularised. The extra ocular muscles develop from a common mesodermal mass that is separated as three different groups. The anterior tip of the optic cup develops over the scaffolding of mesodermal stroma. The part of the iris that develops from mesoderm is its stroma. It is formed by the mesodermal tissue surrounding the margin of the optic cup and tunica vasculosa lentis. The Uvea The uvea develops from neuroectoderm and mesoderm. The Iris and ciliary body develop partly from neuroectoderm (that is part of optic cup). The nerve supply to levator passes through the superior rectus mass hence simultaneous congenital under action of both are a common feature. Structure wise choroid differs from iris and ciliary body. The extra ocular muscles are no exception. 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 ciliary muscles become evident by third month and is gradually differentiated into longitudinal. The outer layer is pigmented while the inner layer is non pigmented the ciliary epithelium give rise to 70-75 ciliary processes. 1. 2. The iris is fully pigmented after birth. ciliary muscle. Later the pupillary membrane separates from tunica vesculosa lentis.

The pigment epithelium starts acquiring pigment granules at this stage. The Anterior Chamber The anterior chamber develops as a cleavage in the paraxial mesoderm that lies between the corneal endothelium and iris stroma. The foetal fissure that develops at the under side of the optic vesicle extends in to the stalk also. By third month the mesoderm that forms the connective tissue of optic nerve along with minute capillaries enter the optic nerve. Its presence is noticed at 20mm stage. In few cases the myelination may extend on the surface of the retina as medulated nerve fibers. By seventh month of gestation all the layers of retina are well developed except in macula. then there is a slowing of growth while rest of retina grows in usual pace. The angle of anterior chamber is not formed before 6 month i. the fovea which at birth has only one layer of ganglion cells left.OUTLINE OF DEVELOPMENT OF THE EYE 5 vasculature develops in three stages. Initially there is fast development in area of macula upto third month of life. The Retina The retina develops from both the layers of optic cup. dura. By the time the fetal fissure begins to close the inner layer starts to thicken to form various layers of sensory retina. At sixth month of foetal life it is thicker than rest of the retina. arachnoid and pia develop between third and seventh months.e. The outer covering of the nerve i. Schlemm’s canal starts as venous channel derived from various plexus at the margin of the optic cup. two months after the canal of Schlemm is visible. The Optic Nerve The optic stalk that joins the fore brain and the interior of the optic vesicle is the future optic nerve. In second phase larger tributaries of venae verticosae develops by the third month of gestation.e. Initially it is very shallow but at birth it is fully formed.e. the lamina cribrosa develops late. The Macula Development of the macula differs from rest of the retina. The outer nuclear layer is also single layered. extending towards the lamina and stops short at lamina. 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. Myelination of optic nerve starts at about seventh month of foetal life towards cephalicend. The inner layer gives rise to nine layers of sensory retina while the outer layer that remains single layered gives rise to pigment epithelium. Thus its development is not complete by ninth month.eighth months it starts thinning. To attain full development macula has to wait up to fourth month post natal. The thinning is due to spreading out of ganglion cells from the central part i. By seventh .. Anterior chamber starts as a chink in the centre of this mesoderm and spreads to the periphery. This state of retardation persists upto eighth month then it start growing in the same manner as rest of the retina. Earliest is development of chriocapillaries. . The third phase consists of development of vessels from short ciliary vessels. The axons of the retina and blood vessels pass through this opening.

The hyaloid system 2. The primitive internal carotid gives off two branches that are precursors of long posterior ciliary arteries i.e. lateral and anterior. This passes through the foetal fissure and courses through middle a developing vitreous. The uveal system 3.e. In hyaloid artery the anterior branches start disappearing first. The posterior part of the tunica vasculosa lentis is first to start to atrophy. . 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. The central part of the pupillary membrane is almost devoid of any vessel. The dorsal ophthalmic artery also gives rise to short posterior ciliary arteries.6 Intra Ocular Vasculature PEDIATRIC OPHTHALMOLOGY Intra ocular vasculature can be divided into three components : 1. It has three parts i. Exact stimulus for disappearance of hyaloid system is not well under stood. followed by lateral. (b) The tunica vasculosa lentis. The Uveal Circulation. 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. the former develops to become temporal long posterior ciliary artery while the latter is destined to become medial long posterior ciliary artery. The anterior uvea is supplied by muscular branches of ophthalmic artery which form the anterior ciliary system. stretching from posterior part of the optic vesicle to posterior pole of the lens. A small part may remain unabsorbed in the Cloquet’s canal or may remain attached to the optic nerve head as Bergmeister’s papillae. 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. It forms the pupil as it atrophies. (c) The hyaloid artery is a branch of dorsal ophthalmic artery. the posterior. The uveal circulation becomes evident at very early stage as vessels round the posterior part of the optic vesicle. The anterior tunica is last to atrophy without leaving any trace. 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. Atrophy of the hyaloid system. 1. It supplies blood to the developing lens. 2. dorsal and ventral ophthalmic artery. The peripheral thick part persists as part of iris stroma. It also forms a connecting channel between intra and extra ocular circulation. Sometimes leaving a small part attached to the posterior pole of the lens as Mittendrof ’s spot. It completely engulfs the developing lens and supplies blood to it.

the main mass of the lid is formed by mesoderm while the skin and conjunctiva develop from surface ectoderm. The retinal circulation. The upper end will form the two canaliculi. lacrimal gland and accessory lacrimal glands also develops from surface ectoderm and are associated with development of the lid. its diameter is relatively large as compared to face. the mesoderm condenses outside the optic vesicle in the form of lid fold. puncta and sac. The size of orbit is so small at this stage that its walls are snug with the globe. . Development of Lid. The upper part thickens and dilates to form the lacrimal sac. 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. At about 18mm stage. (a) The lacrimal gland. By 14th week the boundaries of orbit are well differentiated. Conjunctiva and Lacrimal System 1. Development of lacrimal system7. the groove or cleft is converted in to a tube. The Lid. Failure to separate will result in ankyloblepharon of various degrees. Up to 28th week the orbital margin is at the level of developing equator of the globe.OUTLINE OF DEVELOPMENT OF THE EYE 7 3. The floor and lateral walls develop from maxillary mesoderm. The upper lid develops from frontonasal process in two parts i. During third month the central cells of the cord begin to disintegrate and form the nasolacrimal duct. 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. Separation is completed well before birth. The lacrimal passage develops in the groove between the maxillary proces and lateral nasal process. The lid has dual origin. cilia. The medial wall develops from the lateral nasal process. The roof differs little from these walls.e. it develops from the mesoderm covering the forebrain. The Conjunctiva. Exact mode of development of retinal circulation is controversial. The upper and lower lids are fused between third and sixth month then they separate. The ectodermal cords gradually canalise and ramify. while the lower lid develops from maxillary process.e. meibomian gland. 3. Lacrimal gland develops from the superior temporal conjunctival fornix. there after it grows rapidly and the rim occupies more anterior position than the globe. Development of the Orbit The walls of the orbit develop from the mesoderm around the eye. as solid cords of ectodermal cells 8-10 in number which are surrounded by mesoderm that develop into connective tissue of the gland. The conjunctiva. smaller medial and larger lateral part. At birth the orbital rim is almost circular. at the same time a similar cord of ectoderm develops from the nasal cavity. 2. The surface ectoderm gets buried in the mesoderm and progress upwards. The disintegration is patchy in nature but ultimately becomes continuous. the upper and nasal will join each other to form a continuous structure. (b) The Lacrimal Passage. Subsequently the two cords i.

Microphthalmos has been divided in following groups more on clinical features rather than embryological. The defects in one eye need not be the same in other eye. hypoplasia of macula. Cyclopia. Congenital cystic eye ball.e. They have been described as phakia children with aphakic correction. (d) Microphthalmos with cyst. 3. colobomatous cyst and typical coloboma of eye. Maldevelopment of formed eye is Microphthalmos (True nanophthalmos) 1. has normal movement but may be strabismic due to associated high axial hypermetropia.e. Nanophthalmos is a rare congenital condition where a fully developed eye fails to grow like any other eye. Cyclopia. There are various types of glaucoma i.9. The nanophthalmic eyes with glaucoma do not respond well either to medical or surgical treatment. There is one palpebral fissure with two rudimentary lids. They are also known as extreme microphthalmos. In bilateral cases both eyes are smaller than normal but not of equal size. late onset of simple glaucoma. AC is shallow. and lacrimal apparatus. retina disc. They can happen : A.12 (pure microphthalmos). 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.10 The Congenital anomalies of globe are caused due to faulty embryogeneses before the closure of the embryonic fissure. (a) Nanophthalmos11. During development of optic cup i. 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.) C. the later is generally . They can be unilateral or bilateral. and extreme degree of microphthalmos. (c) Complicated microphthalmos. This is caused due to failed growth after the embryonic fissure has closed from end to end. Microphthalmos. the lids do not close. narrow angle glaucoma.e. These children have neonatal death. (e) Microphthalmos associated with systemic syndromes. There is pseudoneuritis. The cornea is smaller. amblyopia. However in clinical practice eye with minimal ocular tissues are also called clinical anophthalmos. (Uvea.8 PEDIATRIC OPHTHALMOLOGY CONGENITAL ANOMALIES OF THE GLOBE8. Generally these eyes do not have any evidence of formed globe. precipitation of glaucoma following mydriasis13. There are no colobomas present in the globe. The sclera is thickened and the vertex veins are narrow11. B. (a) Pure microphthalmos (Nanophthalmos) (b) Colobomatous microphthalmos. The IPA is wide. These children require high hyper meteoric correction may require near correction. the rudimentary cornea and conjunctiva are exposed. Anophthalmos differs from enophthalmos. During formation and development of primary optic vesicle i. In unilateral cases the other eye may be normal and remain so for rest of the life. Anophthalmos. nystagmus. In enophthalmos a fully developed globe is pushed back in the orbit due to secondary causes. it occupies normal position in the orbit. These include all eyes that have size less than normal eye due to congenital cause. 2. There is either one orbit or two maldeveloped fused orbits.

lacrimal gland.OUTLINE OF DEVELOPMENT OF THE EYE 9 associated with Chroroideal effusion syndrome13 that may follow any intra ocular surgery or injury to the globe. In between are the cases where a formed eye is associated with colobomatous cyst. No part of the eye or its adnexa develop from endoderm. (e) Microphthalmos associated with various syndromes. 3. lacrimal passage. Epithelium of all the ocular adnexa i. Bruchs membrane develops from neural ectoderm and mesoderm. (b) Colobomatous microphthalmos. DEVELOPMENT OF OCULAR STRUCTURE FORM EMBRYONIC GERMLAYER 1. While zonules develop from surface ectoderm and mesoderm. These are due to (i) Failure of development of primary optic vesicle. The eye along with its adnex develop from ectoderm and mesoderm. iridocorneal defects. The Lens B. Is a term used to denote a congenitally small eye that develops cataract. epithelium of ciliary body. sphinter and dilator muscle of iris. These eyes have multiple colobomatous defects at the site of fusion of embryonal fissure. melanoeytes. meibomian gland. pigment epithelium of iris. BUPHTHALMOS This condition is just reverse of microphthalmos it is a large eye associated with various types of congenital glaucoma. colobomatous microphthalmos is a congenitally small eye that have associated failure of embryonic fissure. 2. (d) Microphthalmos with cyst. retina and vitreous. the coloboma may range from a small notch in iris to coloboma extending up to optic nerve. (ii) Arrest of development after the primary optic vesicle has formed. mostly in uvea and or retina. 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. These eyes are small in all dimensions. (c) Complicated microphthalmos. Corneal Epithelium C. retinal pigment epithelium. . conjunctiva. glands of Zies and Moll. In case only retinal tissue protrudes through the embryonic cleft a cystic eye ball with coloboma results. There is a long list of conditions that are associated with microphthalmos most of which are cranio facial or mandibulo facial anomalies. The surface ectoderm gives rise to : A. defects in iris.e. neural part of optic nerve. The neural ectoderm gives rise to Sensory retina. The Bruch’s membrane and the tertiary vitreous (zonule) have duel origin.

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

Age of onset B. The diseases have been classified variously according to: A. It is essential to keep in mind that a child’s eye is not a condensed form of an adult eye. the patients have been divided into following age groups: A. Neonates and infants B. Neonatal C. 11 . Between one year to five years C. Tissue involved and D.CHAPTER 2 Examination of the Eyes in Pediatric Patients1. Five to ten years D.5 GENERAL PRINCIPLE The examination of the eyes in children basically does not differ much from that of adult eyes. Otherwise ocular disorders can also be categorised chronologically. Chronology of disorder C. The principles of the tests. Between one to five year D.2. the disorder can belong to the following groups and each may have distinct clinical presentations and require specific examination: A. According to age of onset. Clinical Presentation.4. Whereas some of the occult lesions may be present in infancy but become obvious only at later age. For purpose of examination. It should be remembered that some of the disease may be obvious in lower age group and spill over to higher age groups. Above ten years. clinical approach and interpretation of each test is almost the same in all ages with little modification. Above five year.3. Intra uterine B.

followed by various drugs taken in first trimester. Genetic 2. syphilis. Neoplastic (rare). radiation. fibroids and umbilical cord are common mechanical causes of ocular deformity. B. drugs. 4. many of them are genetic in nature. The congenital infections that cause ocular morbidity but do not manifest always at birth are rubella. These infections are gonorrhoea. limbal dermoids. deformed uterus. Transplacental. inclusion conjunctivitis and other bacterial infections. Nutritionational. The infection can be acquired during delivery or soon after. dysgenesis of anterior chamber. herpes simplex. Traumatic 5. C. Intra uterine factors that produce ocular malformation can be: 1. attempted abortion in early pregnancy also cause ocular disorders. 3. The genetic factors can be: (a) Inherited genetic defect. total or partial . microcornea. (d) Effect of exogenous factors like. toxoplasmosis and syphilis. Some of them are life threatening. In developing countries dietary deficiencies have a considerable influence on ocular organogenesis. Anophthalmos. 6. Effect of pre maturity . polycoria. Intra uterine PEDIATRIC OPHTHALMOLOGY The intra uterine ocular disorders produce mild to sever deformity and visual loss. toxoplasmosis. diet. are generally bilateral. The transplacental factors causing ocular defects are mostly infection. Infection. bluesclera. The same is true of congenital infections. NEONATAL CAUSES OF OCULAR DISORDERS CAN BE A. coloboma of uvea. they can involve a single structure or may involve more than one structure in various combinations themselves or their aftermath common among them are. The after effects of such infection may cause ocular morbidity which may be noticed later. aniridia. (c) Chromosomal aberration. Uterine bands.12 A. Maldevelopmental causes of ocular disorders are varied. Mechanical. microphthalmos. Mal developmental. (b) Genetic mutation. The common infections that cause ocular morbidly are Rubella. Intra uterine traumas in the form of amniocentesis. chlamydia. cytomegalo virus diseases.

. associated or secondary congenital glaucoma. Orbital tumours H. Dietary Deficiency B. Various craniofacial and mandibulo facial syndromes. CAUSES OF OCULAR DISORDERS SEEN IN CHILDREN BETWEEN FIVE TO TEN YEARS CONSIST OF A. Inborn errors of metabolism C. Trauma 5. Neonatal infection. squint and amblyopia. Boys in this age group should be tested for colour defect to plan their future occupational training. Developmental anomalies. Errors of refraction D. The macula may be hypoplastic or may have coloboma. 3. Intrauterine infection 2. Strabismus E. Sequel of 1. Residual or continued effect of above B. Glaucoma F. persistent primary hyperplastic vitreous. detachment coloboma. 4. ectopia lentis. Prematurety causes retrolental fibroplasia. congenital myopia. so are allergies C. and myopia of prematurety.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 13 cataract. Allergy — — J. various types of squint. Infection — — Endogenous Exogenous Local Systemic I. More children turn up with errors of refraction. Intraocular tumours G. primary . Infections become common. The retina may show congenital folds. Trauma in this age group is frequent D. non cataractous white reflex in pupillary area. CAUSES MET BETWEEN ONE TO FIVE YEARS OF AGE ARE A. Autoimmune disease K. Ptosis. Degeneration and dystrophies.

The movements of the eyes are bizarre. contrary to general believe there is no spurt in axial length of an emmetropic eye at puberty.8 mm as compared 7. . C. the eye reaches its adults size by 14 years of age. The orbits are smaller. The Vision. The cornea is flatter when examined on kertometer. 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 1.5mm in diameter as compared to 23 to 24mm of an adult eye. The space between the globe and orbital wall is so less that the orbit seems to sit snugly round the globe. D. G.5 mm to 17. 2. Movement of two eyes are independent and non conjugate. The newly acquired organisms are generally non pathological. 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. the conjunctiva is sterile at birth that gets contaminated within few days. The Cornea has mild haze that clears within few days. Its curvature is uniform.0 mm of adult. The volume of eye ball is 2. As the IPA is narrow in new born there is a false impression of cornea being large. 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.5 mm while that of adult cornea is little less than 12mm. E. however there is no clinical method to measure accommodation and convergence. The Conjunctiva is well developed. However the blink mechanism is absent and develops by 7th or 8th week. Parts of this in neutralised by reduction of curvature mostly of lens and partly of cornea4. B. The eyes are smaller in size as compared to that of adults. it has been estimated to be about 1/607 the new born child has good accommodation.14 PEDIATRIC OPHTHALMOLOGY EXAMINATION OF EYES IN NEW BORN Before embarking on examination of eyes of neonate it is essential to remember features of new born eye. F. A. The new born eyes as sensory system are better developed than expected when weighed against its size. they can fix a strong stimulus for a short while and then give up. the width is less as compared to cornea hence the cornea looks larger and may be mistaken as abnormal. the goblet cells are functioning. it is difficult to evert the lids. The diameter of new born cornea is 10. The new born sleeps for almost 18 hours a day so most of the time the eyes are closed. The fixation reflex is present but poor. None the orbital contents are visible or palpable. By the age of 3 years the eye is about 23 mm in diameters. The inter palpebral fissures are 18 mm in length. The eye becomes myopic. The Cornea is relatively large when compared to adult cornea. However this growth is far less when compared to axial length of the globe which grows from 18mm at birth to 24mm at maturity. shallower and round. giving spherical look to the cornea. Hence threatening gesture to find out presence or absence or vision is futile. The eye ball of a new born is 16. The vision of a new born is less as compared to a child of three years but is not very poor.

The lens is more spherical than adult lens. the optic disc is pale. K. 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. The dilator muscles are poorly developed. or seemingly abnormal cases. its average diameter is 6mm in comparison to 9mm of an adult lens. Nurses and midwives attending neonates can also be taught to examine a neonate’s eyes and refer for further examination for abnormal findings. The pupil can be safely dilated without rise of IOP. The Lens is fully developed and functional. . the macula is not fully developed. a neonate wakes only when hungry or is ill at ease. The fundus The fundus is visible. is shifted nasally and down. Its depth is normal or slightly less than adult depth. The examination is done either by attending neonatologist or resident of pediatric ophthalmology posted in nursery and well baby clinic. P. M. Accommodation and Convergence can not be tested clinically due to lack of fixation. Accommodation is well developed by 4 months. The movements are not co-ordinated and can not be tested precisely. The new born cries but does not weep.. The contents are clear. The examiner should be able to differentiate normal parameters of signs from the abnormals. The constrictor muscles are relatively stronger hence it is difficult to dilate the pupil. the angle is wide. An infants eyes are required to be examined under following circumstances A. The retinal periphery is also pale. The anterior chamber is fully formed. the colour is paler than normal. J. The lens grows in size throughout the life. It takes three to four years for them to adequately develop. it continues to grow postnatal. Many a times a casual remark by the examiner may send a wave of panic in the parents least the baby may be blind. As a part of routine examination of all new borns. I. There may be a few blood vessels on the iris surface. The pupil is smaller than adult. It is circular and reacts to bright light both directly and indirectly. It is difficult to elicit correct visual response in an infant. The foveal reflex is absent.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 15 H. this is due to lack of reflex tearing that develops after four to six weeks. The Lacrimal Glands are not fully developed at birth. The broader nose bridge of a new born gives an appearance of pseudo convergent squint. EXAMINATION OF THE EYES OF NEONATES AND INFANTS Examination of the eyes of a neonate is difficult because a neonate sleeps almost eighteen hours of a day the waking period is in short spells and most irregular. O. The Lacrimal Sac is fully developed at birth and its lumen is patent . The iris is light coloured due to scanty pigments. L. The protocols followed in various hospitals differ greatly. Psychic tearing takes about six to seven months to develop. The Lacrimal Passages are canalised at birth.17 N. Q.

corneal haze.T.D. large coloboma of the lid. pupillary abnormalities white reflex in the pupil and refers for detail examination and opinion. Family history of (a) Lamellar cataract (b) Albinism (c) Nystagmus 2. The vision of neonate improves very fast6 from 1/60 to 2/60 at 1 month and 6/60 at four months. VISION TESTING IN A NEW BORN One of most difficult tasks in ophthalmology is to give opinion regarding exact quantitative vision present in each eye of a new born. Optokinetic target. (i) Difficult labour fetal distress. With 1/60 vision the child is able to fix a face moving within one meter.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. microphthalmos. The neonatiologist has noticed some gross abnormality i. (c) Some drugs given in the first trimester that cause congenital anomalies. intensive resuscitation for above causes. D.L)7. (j) A critically ill child who may have congenital toxoplasmosis. 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. faciomandibular abnormality. Catford drum test. hypoglycaemia. There is 1. Signs of infection in the eyes : Edema of lids. unilateral gross ptosis. These are associated with bizarre movements. Maternal history of (a) Suspected rubella in first trimester (b) S. herpes simplex galactosemia. specially injury by forceps.16 PEDIATRIC OPHTHALMOLOGY B.P.e. The above abnormalities are gross enough to be noticed by parents or even a village midwife. proptosis. (d) Diabetes under treatment. neonatal apnoea. craniofacialdysostosis. C. . A rough estimation of qualitative vision present is not difficult to express. visual evoked occipital potential (VEP) and forced choice preferential looking (F. purulent or mucopurulent discharge. cryptophthalmos.e. These visual standards are based on various complicated methods by expensive instruments on small samples i. birth trauma. anophthalmos. (e) Myasthenia in mother (f) Consanguinity (g) Prematurity (h) These babies may or may not have been on prolonged oxygen.

This must be repeated several times least some of the bizarre movements may be mistaken for fixation. Ocular 2. and other examined. It is said that most suitable infant to examine for vision is an awake. 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. 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. both eyes are examined in the similar way separately one after the other. (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 . alter and hungry baby. visible causes are excluded. C. For that one eye is closed. This gives a definite clue of vision being present in at least in one eye but can not indicate which eye. A.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 17 these movements disappear with development of clear fixation. Visual acuity in a new born is determined by optically elicited movements. (l) Tay’sachs disease. (j) Recessive opticatrophy (k) Leber’s amaurosis. The incidence of blindness in neonates is very low if. The condition that result in bilateral sub normal vision can be 1. The first step is to observe if the child tries to look and fix a light when switched on in a semidarkned room. More than one types of movements are common in between the fixations. Each eye should be tested separately. This indicates most primitive form of vision that can be elicited clinically.

5mm at birth any cornea larger than 12mm is abnormal may be buphthalmic. If the pupil is small and it is difficult to appreciate pupillary reaction a hand held slit lamp may be used.P. . G. When in doubt about diameter of the cornea it is better measured. Examination of face for craniofacial and mandibulo facial anomalies. E. Anophthalmos. Face. Ophthalmianeonatorum does not develop within first twenty four hours.18 (f) Cortical blindness (g) Sagital mild line defects. Blepharophimosis that is frequently seen with ptosis and epicanthus and causes shortening of length of I. hemangioma. The light used should be small well focused and bright. Lagophthalmos is far rarer than ptosis. Neonatal nasolacrimal duct obstruction when present is asymptomatic and non elicitable. Most important cause of large cornea is buphthalmos that requires early referral. Palpebral fissure of new born is smaller than adult. cystic eye ball. D. Other anomalies present at birth that draw attention are : epicanthus and ankyloblepharon. proptosis. F. Orbit. It is narrow in ptosis wide palpebral fissure is seen in lagophthalmos. congenital miosis or persistent pupillary membrane. PEDIATRIC OPHTHALMOLOGY Half of the cases of diminished vision in children are genetic in origin.17 EXAMINATION OF PUPILLARY REACTION TO LIGHT This is most reliable test to determine presence of vision except in cortical blindness. The test is best performed in a semi darkned room because the infants pupil are smaller than normal and constricts in presece of bright light in the room. hemangioma. Lid. B.A. the other cause is megalocornea. In the semi dark room the pupil comes to a state of semidilatation that reacts briskly.A.e. Only anomaly visible in a neonate is congenital mucocele of lacrimal sac. The lids are examined for Ptosis. Unilateral ptosis draws attention earlier than bilateral ptosis. Most reliable measuremnent is taken by a corneal calliper. Lacrimal system. albinism.P. proptosis and rarely in buphthalmos. The cornea of a new born is 10-10. coloboma. indirect and swing light responses are noted. The Cornea Even a normal cornea in a new born looks large because of narrow I. Epicanthus also gives a false impression of pseudo convergent squint that may not be appreciated at birth but becomes obvious after few months. Small sluggish irregular pupil are indication of inflammation. virus and chlamydia. OTHER EXTERNAL EXAMINATIONS CONSISTS A. direct. There is apparent shorting in length in epicanthus. C. microphthalmos. All forms of light reflexes i. a transparent scale may be usedfor rough estimation. 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.

Bilateral small cornea is frequent.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 19 Microcornea is frequent . The angle is wide and pupil can be dilated with out rise of I. Other condition being. J. It is difficult to examine the cornea and conjunctiva due to tightness of the lids. more common than megalocornea. Peter’s anomaly. Uvea. and small palpebral fissure. There is no condition in new born eyes where eversion of the upper lid is needed. Corneal haze Most of the cornea in new borns are relatively hazy. Peter’s anomaly and coloboma of iris. coloboma of the iris and ciliary body. which causes irregular shallowing of A. Congenital aphakia is a clinical curiosity. size position and number. The anterior chamber of new born is fully developed but shallow.P. 1. Other signs that should be looked for are: 1. Commonest anomaly of anterior chamber is mesodermal dysgenesis of Reigers. The iris of new born is lighter in colour. A deep chamber is seen in buphthalmos and megalocornea. Anterior Chamber. Rarely a lid retractor may be required to see the upper limbus and upper bulbar conjunctiva. Rupture in Descemet’s membranes. it may be associated with microphthalmous and coloboma of the uvea all three may be present singly in an eye or in various combinations. Presence of few blood vessels is common and non pathological. if at all necessary. . Commonest cause of irregular pupil is persistent pupillary membrane. An infant size lid retractor should be used. anisocoria and cryptocoria.O. Pupil. The pupil is examined for it shape. Sub luxation of lens is not appreciated unless carefully looked for. Other causes are mesodermal dysgenesis. H. Some of the pathological cause of corneal haze are : (a) Buphthalmos (b) Rupture in Descemet’s membrane (c) Dysgenesis of anterior chamber (d) Mucopoly saccharidosis. Frank corneal opacity. Common congenital anomalies are persistent pupillary membrane. 2. Rupture in Descemet’s membrane is mostly traumatic due to faulty instrumentation during delivery. Posterior uvea is examined by direct and indirect ophthalmoscope with dilated pupil. Limbal dermoid. Corneal haze. Aniridia is a rare anomaly but is a vision threatening condition that may have Wilm’s tumour in the abdomen. 4. Pupil of a new born child is relatively moitic due to stronger constrictors than dilators of pupil. I.C. It is difficult to dilate the pupil of new born due to same cause. which become bright in few days. 3.

EXAMINATION OF EYES IN FIRST YEAR OF A CHILD Many of the difficulties in examination of a new born child’s eye gradually pass away giving way to easy examination and better interpretation. unilateral or bilateral is not a good sign and requires prompt investigation. blood vessels. This should not be confused with pathological sunset appearance of the globe. disc. The non pathological lid retraction may persist for four to six months. sub luxation is difficult to elicit. It requires little practice with dilated pupil and knowledge to differentiate between normal and abnormal findings in optic nerve. Squint. Examination of fundus in born. macula and periphery. Commonest anomaly of interest is presence of cataract. Proptosis. The fundus is examined for transparency of media. N.5% phenylpherine with 1% cyclopentolate11. Examination of fundus by both direct and indirect ophthalmoscope is not difficult. Proptosis. Remnants of hyloid system are frequent. Squint is difficult to confirm due to bizarre movements unless there is gross lesion like Mobius syndrome which is other wise very rare. One drop is instilled in each eye twice at an interval of 3 minutes and fundus examined after 45 minute to one hour. Drug used to dilate pupil of new born There are many drugs employed to dilate the pupil of a new born. O. A cataract covering whole of pupillary area requires urgent referral. Commonest combination of drugs is 2. Some ophthalmologists have enough patience to examine the fundus in between involuntary the closure of lids. Up rolling of the eye due to Bell’s protective reflex. Atropine is better avoided due to its possible side effects. especially retinoblastoma from cataract. If there is clear zone then the pupil can be kept dilated for light rays to reach the macula till definite treatment is instituted. Nystagmus. Lens. Absence of lens is extremely rare in new born. 2. . A small pale disc surrounded by yellow halo is suggestive of hypoplasia of disc. Though it is very common to confuse non coordinated movements with nystagmus. M. Each ophthalmologist has his or her drug of choice that may be single drug or a combination of two drugs. the fovea is not bright the disc is pale enough to be confused as atrophic. Non pathological bilateral lid retraction and down ward movement of globe when the illumination in the room is reduced. This may be circumvented by use of neonate size of eye speculum. one sympathomimetic and other parasympatholytic. The arteries are thin. Pupil should be widely dilated to see if there is any clear zone between the outer border of opacity and dilated pupil. L. It is very essential to be able to differentiate between non lenticular causes of white reflex.20 PEDIATRIC OPHTHALMOLOGY K. macula and retinal periphery. Two common phenomena observed during fundus examination are 1. The neonates tolerate presence of speculum well. Enophthalmos in neomates and infants is rare. Nystagmus does not develop before two months. P.

by comparing it with older siblings or children of the same age. prenatal asphyxia. 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. CT and MRI of ocular structures and brain. While recording vision the approximate near vision also be noted in preverbal child. Vision should be considered normal in a child under one year if the media are clear. Presence of pupillary reaction to light is not equivalent to visual ability. Recording of vision The vision generally ranges between 6/60 to 6/36 i. CT and MRI to evaluate prognosis. there is no squint or nystagmus. optic nerve hypolplasia congenital cone dystrophy and stationary night blindness7. persisting staring and inattention to object10 are ominous signs sometimes parents may be able to state if the child’s vision is subnormal or not. Nothing is worse than informing the parents that the child is blind or normal. However nystagmus is absent in cortical blindness. while examining each eye separately it is better to examine the supposed to be good eye first than the suspected faulty eye. Prematurity. Presence of squint. nystagmus. Electrophysiological investigations-electro-encephalogram. 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. There are some children who have delayed visual maturation (DVM)7 and behave like a child with sub normal vision. 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. low birth weight.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 21 A. electro-retinogram and visually provoked response. pupil are central circular and react briskly to direct and indirect light. that turns out to be opposite later. 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. fundus is normal with normal milestones. the child can recognise faces by sight.e.11 In cortical blindness the pupillary reaction is present. commonest cause in children being trauma. The attendant should be asked not to prompt the child when the vision is being recorded. This is a very difficult question to answer. convulsion congenital anomalies of brain may have occipital lesions which requires. 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.12 All children suspected to be blind should also under go : 1. the child may start crying and try to remove the occluder. However vision can not be tested with usual optotypes. It is good practice to let one of the attendants preferably one of the parents present during examination of the vision. there is no error of refraction. If the . This is the age when the children are brought by anxious parents with a definite question “ Does this child has vision ?”. this is due to the fact that the lesions is in optical cortex. 2. develops preference for toys and picks up particles from the ground.

It is better not to darken the room. tilting or abnormal chin position in various combinations point towards possibility of paralytic squint (for details see examination of strabismus) E. 2. C. It is well developed by four months. 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. Even a child of one year may have a raised eyebrow in case of ptosis. It takes steriopis to reach adult level by five to six years of age. coloboma of lid margin and distichiasis. The parents may not like this due to preformed idea that the wrong eye is being examined. B. The eyebrows have scanty.22 PEDIATRIC OPHTHALMOLOGY child has glasses the power of the glasses should be noted and vision in each eye is recorded as with glasses. Diminished vision in one eye generally goes unnoticed by the parents unless associated with other causes like squint. Raised eyebrow with furrows in forehead denotes ptosis. The chin may be elevated . Persistent abnormal head posture like head turning. fine hair normally and the eyebrows are symmetrically arched on both sides. corneal opacity or white reflex in pupillary area. It is well developed by four months though it is considered to be present by second month. The points to be noted in diffuse light are 1. Is the child comfortable. All children with skull larger or smaller than normal should get paediatric consultation. this scares the child it should be semi lit. fusion and convergence17 Accomodation is present at one month but is relatively poor. Look for symmetry of face. the child may open the eye if the room is darkened sufficiently. D. A child with searching movement. It is generally seen with epicanthus and ptosis. Unilateral ptosis attracts more attention than bilateral ptosis in children. Epicanthic fold gives a false impression of pseudo convergent squint while telecanthus gives an impression of divergence. Accommodation. and skull : Asymmetry of face is generally seen in cranio facial anomalies. Fusion starts at two months. Flattening of eyebrows. The lids are examined for hemangioma. The other eye should also get same attention as the diseased eye even when the lesion is suspected to be unilateral. The first step is to observe the child in normally lit room from a distance of about a meter without touching the child. 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. F. In blepharophimosis the horizontal length of palpebral fissure is reduced. 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 . absence of forehead crease and wide IPA denotes lagophthalmos. The parents at this stage should be explained the purpose of examining both eyes. Narrow palpebral fissure is seen in various types of ptosis and pseudo ptosis. most probably does not have vision in either eye. Fusional convergence develops by six month.

(b) Virus. Chemosis with discharge is seen in acute bacterial conjunctivitis. Steven’s Johnson syndrome. Papillae are generally not seen under one year of age. Whooping cough used to be a common cause of subconjunctival . Other cause of membrane over conjunctiva are : (a) Bacterial. independent of immunisation status of the child. (c) Toxic. In children under one month other chlamydia infections are possibile. Follicles are very common. Children brought with complains of recent unilateral watering are rarely due to nasolacrimal duct obstruction. Streptococcus. It is better if a drop of anaesthesia is used before applying the lid retractor. pneumococcus. In such cases an infant size lid retractor may be required sometimes two retractors may have to be applied. Most important condition that requires exclusion is buphthalmos. lower fornix and bulbar conjunctiva do not pose much problem so long this is not associated with edema of the lid and blepharospasm. 5. while using lid retractor care should be taken not to injure the cornea. infectious mononucleosis. staphylococcus. cornea may show Haab’s lines. Chemosis of conjunctiva without mucopurulent discharge is rarely infectious most probably it is due to a hay fever like conjunctivitis. (d) Chemicals (e) Drug allergy. meningococcus. A positive regurgitation test is always confirmatory. Where the cornea is generally larger than 13mm the AC is deep. 2. 1. A congenital coloboma of lower lid which is generally seen at the outer one third may also cause epiphora.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 23 called as neonatal nasolacrimal duct obstruction because inflammation of sac is secondary to congenital obstruction. Koch Weeks bacillus and Ecoli. 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. Presence of membrane over the conjunctiva should be considered to be as diptheritic and treated as such unless proved otherwise. Examination of conjunctiva of lower tarsal conjunctiva. 4. primary secondary or associated. phlycten or exogenous-Spring Catarrh is rare are under one year but can not be ruled out. G. In absence of positive regurgitation the condition of lower puncta for its presence and size should be verified. The child’s head and body below the shoulder should be immobilised by the assistant it is better to take a conjunctival smear at this stage. mere presence of follicle is of no consequence.e. AC and pupil are also examined along the conjunctiva at this stage. herpes simplex. buphthalmos or conjunctivitis commonest cause is corneal abrasion that is confirmed by positive fluorescein stain. Conjunctiva is examined under focal illumination. One of the dictums to be remembered is “Unilateral redness of eye is seldom conjunctivitis” Chronic allergic conjunctivitis both endogenous i. However in endemic area trachoma should be ruled out. The cornea. 3. Epidemic kerto conjunctivitis.

spring catarrh and hemangioma. injury during forceps delivery. corneal abrasion. viral and chemical conjunctivitis. Transparency of cornea.e. 7. Even today it is seen in non immunised children in developing countries. (c) Glaucoma : Acute and chronic congestive. 9. The child usually gets a prophylactic dose of 50. Out of all . mothers milk contains sufficient vitamin. Examination of Cornea Consists of examination of transparency. The haze passes off within a few days to few weeks. size. Mucopurulent discharge is seen in other bacterial infection. Episcleral congestion is rarely seen in children when present they are mostly due to buphthalmos or raised episcleral pressure due to an orbital mass. Conjunctival Xeroxis is a common feature and ominous sign of vitamin A deficiency. Faints blue hue is universal in children due to thin sclera through which the underlying uvea shines. Haabs striations are horizontal may have whirl like appearance while injury by forceps are generally vertical. Conjunctival redness is common in all types of conjunctivitis mostly infective.000 of vitamin A at six months. (c) In cases of sub conjunctival haemorrhage where its outer limit is not visible child should be examined for possibility of head injury.Subconjunctival haemorrhage. This requires differentiation from another common cause i. It can be localised in:. Colour Changes in conjunctiva are common. 6. Generalised congestion specially bilateral is seen in infective conjunctivitis. Mild bilateral corneal haze is universal in all full term babies and more common in pre term. A for the child for first six month. leukaemia and purpura are common causes of subconjunctival haemorrhages. Bitots spots generally do not develop during first year because if a child is breast fed. Circumciliary congestion or circum corneal flush is seen in (a) Keratitis. Yellow discoloration is seen in jaundice. encroachment over the cornea. H. associated with enlargement of cornea.24 PEDIATRIC OPHTHALMOLOGY haemorrhage in children before universal immunisation came in to vouge. 10. Presence of dense opacities requires attention and management. The cornea is uniformly hazy in congenital glaucoma both. vascularisation and deposits on both surfaces. 8. A pale conjunctiva is indicative of anaemia. corneal ulcer. corneal edema and rupture in Descemets membrane that may result in Haabs lines. The congestion is most marked in the periphery in infective conjunctivitis. Watery discharge is seen in allergic. Conjunctival discharge : Purulent discharge is mostly due to gonococci infection or sever other bacterial infection. sensation. 11. 1. patches of black flat spots are due to congenital melanosis. rarely phlycten. curvature. blue conjunctiva denotes cyanosis. (b) Uveitis : Anterior uveitis and panuveitis. (b) Petechial haemorrhages are seen in epidemic haemorrhagic conjunctivitis and pneunococcal conjunctivitis. abrasion and laceration of conjunctiva. shape. primary or secondary that is generally a bilateral diseases.

In sever drought the mother’s vitamin A store is depleted during gestation and remains low thereafter. examination of angle and evaluation of optic nerve head.P. The child suffers from measles. Dermoid14 is more common than the former. sever malnutrition in mother. Peripheral localised opacities are seen sclerocornea and limbal dermoid. 2. In still rarer occasions the whole of the cornea may be as opaque as sclera. However there are circumstances where the child develops signs of vitamin A Deficiency disease (VAD). In case of Peter’s anomaly the opacity is generally localised centrally with clear periphery frequently associated with other malformations of anterior segment.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 25 cases of corneal clouding congenital glaucoma due to what ever cause requires immediate attention to save the sight. 4. In a new born child the cornea looks large because of smaller I. microphthalmos and cornea plana. Increased corneal curvature suggestive of keratoconess that is extremely rare in first year. Kerato malacia : may be seen as bilateral haziness without circum corneal congestion is developing countries. Other causes of uniform corneal haze are relatively rare. 3. Corneal curvature. the cornea is enlarged in buphthalmos and megalocornea. and microcornea. various mucopoly saccharidoses. Any cornea larger than 12 mm should be considered to be buphthalmic unless proved otherwise by measurement of I. Enchromement over cornea. or the child has been abandoned and children of orphanage. 5. Any cornea larger than 13 mm is definitely buphthalmic. In case of positive history of buphthalmos in family and suspected rubella syndrome the corneal diameter should be measured by corneal calliper. 2. mucolipidoses and cystinosis13. . 1. In congenital rubella syndrome the opacity is relatively dens but clears in few weeks. Distortion of shape of cornea is seen in microcornea microphthalmos and phthisis. The corneal periphery may be as opaque as sclera in a rare instance of sclerocornea. or chronic diarrhoea. 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.A.P. they generally have few hairs growing over the growth. The former two are generally associated with gross incorrectable vision loss. Flattening of cornea is more common and is seen in microcornea. Interstitial keratities is seen after five years of age. The child is deprived of mothers milk due to death of mother. Phthisis which is rare in first year of life may also present with flat cornea. Size of cornea. These are . The peripheral opacity may be vascularised. where the opacity extends well into the cornea from the sclera obliterating the limbus. 3. they are congenital hereditary endothelial dystrophy.O. Bilateral large corneas are missed by parents unless associated with watering but unilateral enlarged cornea draws attention early even without tearing. The commonest site being the limbus where a dry raised circular plaque of fibro fatty tissue is seen astride the limbus. Shape of cornea. Small cornea is seen in microphthalmos. congenital hereditary stromal dystrophy. However they may develop in the middle of the cornea as well. The former require urgent management.

0. should undergo measurement of 10P under general anaesthesia. A very few infants may have iris clip IOL16 as part of management of congenital cataract and have relatively deep A. is almost normal. history of buphthalmos in siblings and abnormal tissue in A. in infants who have undergone lensectomy without IOL. cortical material. Measuring intra ocular pressure. Sclera is devoid of any inflammatory process under one year of age. Sensation of cornea. It is very difficult to detect mild to moderate turbidity under one year of age. without any visible suspended particle.C. All infants with large cornea.L. 4. In P. K. Common cause of white material in A. 3. Vascularisation of cornea always denotes pathology either in conjunctiva or in cornea. Sclera is not a tissue to have many findings in a child under one year.C. L. Vascularisation of cornea. 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. Deep AC is seen in buphthalmos. The only cause of deep vascularisation in children between 3-5 years is interstitial keratitis. 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. There is a very rare condition where corneal sensation is absent from birth called congenital dysautonomia. Presence of blood is more common in this age group which is mostly traumatic than pus. ofcourse surgical aphakia gives a deep A.C. Congenital aphakia is a clinical curiosity only a few cases of spontaneous absorption of lens have been reported in first year. cornea plana.26 PEDIATRIC OPHTHALMOLOGY 6. The sclera of an infant is relatively thin it is not as opaque as adult sclera but not translucent also. the depth of A. Causes of superficial vascularisation are infective and allergic kerotoconjunctivitis. sub-conjunctival patches of black pigmentation are very common and may cause worry in parents. should be considered to be pus unless proved otherwise. Examination of anterior chamber : 1. Corneal sensitivity can be elicited from birth. I. Peters anomaly. is seen in micro cornea. Examination of Sclera.C. Deposits on the cornea are mostly inflammatory. Shallow A.C. Conjenital ectasia may be seen rarely more common cause of ectasia is buphthalmos. Presence of any white material in A. It is reduced over the opacities. The sclera develops yellowish tinge in jaundice. however the iris becomes pretty dark by one year if not . keratoglobus and congenital axial myopia. pseudohypopyon.C.C. Contents of anterior chamber Normal content of AC is aqueous humour that is crystal clear. I. J. are: hypopyon. mesodermal dysgenesis of anterior chamber. microphthalmos.C. Difference in depth of AC in two eyes is abnormal so in presence of mesodermal tissue. Examination of iris. 7. Vascularisation of cornea is rare under one year of age. keratanisation and herpes simplex keratities. 2. Iris of a new born has lighter colour than what is expected to be at adult life due to lack of pigment. The former produces supefecial vascularisation. The corneal vascularisation can be superficial or deep. 8.

All irregularity of pupil are not pathological only irregularity due to inflammation needs treatment. Pupil is examined for 1. It disappears with age. 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. An iris that is abnormally light with pink pupil and trans illuminates in a fair child is due to albinism. Congenital coloboma of the iris is generally situated in lower pole of pupil. 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. Number. 1.P. However there may be corectopia where a pupil is shifted away from its normal place a part of multiple ocular anomalies. size. 3. Pupil reacting briskly to direct and indirect light stimulus is confirmed sign of intact afferent and efferent precortical visual path. Presence of congenital holes in iris other than pupil is called polycoria that generally lacks iris sphinters and called pseudopolycoria. Shape. 3. mesodermal dysgenesis of A. 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.C. is suggestive of . The constrictor muscles are better developed than sympathetic. Other causes are coloboma of iris. 2. shape 4. Single Pupil with slight nasal and medial shift is normal. Number. 4. may be localised to iris only or may be part of more extensive colobomatous anomaly of uvea. Persistent pupillary member is the commonest cause of irregular pupil under one year of age. pseudopolycoria. M. True polycoria is rarer. pupillary reaction. Cryptocoria is congenital absence of pupil . 5. Unilateral small pupil with narrow I. Size. Position. Presence of few blood vessels in first two months are no consequence they also disappear with in few months.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 27 as dark as adult.. colour and 6. The coloboma of iris may be present as isolated anomaly of the eye or may be seen in microcornea and microphthalmos.A. these extra pupil have independent constrictor and dilator muscles and react to light independent of main pupil. 2. Examination of pupil Examination of pupil alone gives much more information regarding presence of many anomalies than any other examination. Normal pupil is circular. 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. position.

H. 5. 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. this allows sufficient light rays to reach the macula for its development and prevention of amblyopia. A child with lesion of visual cortex presents as cortical blindness where the pupil are of same size and react well. Vitreous is examined for presence of P. 7. familial exudative vitreo retinopathy. In principle and practice there is no difference in examination of vision in adults and children. Large pupils are seen in congenital myopia and buphthalmos. Dilatation of pupil requires more time due to strong constrictor.. 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. Bilateral irideplegia 2. Slight difference in size is common. 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.28 PEDIATRIC OPHTHALMOLOGY Congenital Horner’s Syndrome. Where it is important to find out which pupil is abnormal.P. Difference in size is called anisocorca. Colour of Pupil. vitreous haemorrhage. Pupil of a child under one year should be examined in diffuse light. Recording of distant vision in a child. retinopathy of prematurity. A lens with clear periphery may be left with dilated pupil. A brisk direct and consensual light reflex denotes intact afferent and efferent neurological precortical path. 8. Best method to examine vitreous and rest of the fundus is by a binocular indirect ophthalmoscope using a condensing lens that give magnification. remnants of hyoid system.V. Lens of an infant is examined mainly for its transparency. The pupil looks dark even in the most pigmented iris when seen with a torch. MarcusGunn pupil is seen in unilateral complete lesion of optic nerve. Examination of pupillary light reflex. On careful examination the normal lens produces two miniature images the third and fourth Purkinje images. Bilateral fixed dilated pupil are due to 1. However in all cases of large pupil history of instillation of mydriatic or cycloglegic should be excluded. Sub luxation and dislocation of lens is less common under one year and when present becomes obvious only on maximum pupillary dilatation. Causes of white reflex in pupillary area (see chapter on Len and Retinoblastom). Absence of third and fourth are seen in an opaque membrane over the anterior lens capsule and absence of lens (aphakia). The difficulty arises in reaction of the child to . steriopsis and moderate field with maximum dilatation. Examination of lens. Examination of vitreous. congenital internal ophthalmoplegia is very rare. 6. Bilateral sever optic neuropathy. A white reflex in pupillary area is always pathological.

attendants and teachers is also desired for correct interpretation. Optokinetic drum of Harcourt. Co-operation of parents. Acute and painful loss of vision brings a child for examination early. Recording of vision above five years is similar to that in adults with little modification. Most often children with poor vision are brought with squint. Nystagmus denotes bilateral diminished vision. 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. a shy child may not co-operate well to record vision. Various methods to examine distant vision are17. nystagmus.18.19 : 1. Vision is a sensory phenomenon for this co-operation of the child is most important pre requisite. Between 1-2 years 4. Unilateral loss of vision may go undetected for longer time unless the child develops squint or accidentally closes the better eye. 4. Examination of vision of 1. than others and resents being shifted away. A very common observation is that the child prefers to sit nearer the T. indicate poor vision at least is one eye. the child may fail to copy from the black board.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 29 method of examination. Between 3-5 years 6. Recording of vision under one year of age has been delt with earlier. Patience. New born 2. Between 2-3 years 5. Sixty parent of afferent input to the central nervous system for development of higher centres come from vision of the child. Teller acuity card . attention of examiner and practice. Presence of squint. 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. Visually evoked potential 5. A child does not complain of gradual loss of vision unless it is discovered by the parents at home. Presence of unilateral squint invariably means diminished vision in the squinting eye. Under One year 3. Recording of vision in children can be grouped into following categories. A teacher may complain of poor attention of the child.V. Above 5 year. Preferential looking test. Catford drum 3. Recording of vision between 1-2 years of age Recording of vision under two years is slightly easier than under one year. A child with searching movement most probably does not have vision in either eye. 2. Closure of the better eye is resented by the child who may start crying and try to remove the obstruction. Preference of fixation by one eye means diminished vision in other eye.

some times more than one test has to be used. This the child finds interesting and worth repeating. The child’s hand is guided to locate the candy and then to mouth. Worth ivory ball test. 17. Sheridan ball test. Rotation of the disc at a distance of 60 cm evokes pendular movement and not nystagmus as seen in O. 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. 18. The child identifies an incentive i. Cardiff acuity card. The tests that do not depend upon preferential looking (a) Boeck candy bead test. candy beads of gradually decreasing size at 40cm. OKNOVIS17 7. (d) Cardiff acuity cards7.5 to 15mm in diameter representing vision between 6/6 and 2/60.30 6.T. The test gives a rough estimate of vision. (a) Catford drum test In this test cylindrical drum is replaced by a circular disc with dots ranging between 0. coloured pictures on the revolving drum are used. Boeck candy bead test 8. PEDIATRIC OPHTHALMOLOGY None of the above tests is accurate. (c) OKNOVIS17 This new technique is based on the principle of arresting an elicited optokinetic nyslagmus by introducing optotypes of various sizes. (b) Teller acuity card10. Rarely children as young as 18 months have responded to Snellen’s optotypes10 Optokinetic drum test. They are broadly divided into two groups: 1. visual evoked potentials may be required. Once nystagmus is elicited optotypes of different sizes are introduced to arrest nystagmus. 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. The test is done at 60cm to elicit optokinetic nystagmus. Some new tests have been evolved for better results. preferential looking test and visually evoked potential and their modifications are time honoured tests.e.K. This test is modification of preferential looking test. Where alternate black and white strips evoke nystagmus when the drum is activated in front of the child’s eye from a close quarter. That depend upon the preferential looking 2. This test takes less time and simple to perform. . That does not depend upon preferential looking. The principle of these test are similar to optokinetic drum. The test depends upon preferential fixation and consists of a series cards depending on principle of vanishing optotypes. Hence preferential looking test are no more required but in cases of gross visual loss or suspected absence of vision. (b) Other tests used are : Marble game test. 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 instrument is a hand held revolving drum that rotates at speed of 12 revolutions per minutes. The results are obtained in cycles which can be converted to Snellen’s equivalent.

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. Examiner may have to revert to methods employed to examine children between two to three years in these rural children. Rural children in developing countries who constitute 80% of paediatric population are difficult to evaluate visually due to illiteracy. Commonly used optotypes are : 1. While recording of vision following steps are useful to gain the confidence of the child and his co-operation. Snellen’s E chart 3. Sjogren Hand or Arrow 6. by three years the child can recognise shapes hence can be tested on optotypes. he can be tested on usual Snellen’s chart at six meters. Addition of A and U make it seven letters. 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. numbers. HOTV test (a) Lipman 4 letters ( It consists of four letters HOTV arranged in a circle) (b) Sneridan 5 letters. they can be used at 6mts or three meters with a mirror. STYCAR Test (Snellen’s test for young children and retarded) 4. Commonly used methods are : All the test objects are various types of optotypes. Snellen’s chart commonly used is in English but may be in vernacular as well. There should be no difficulty in recording vision in a child if he is literate. Dot visual acuity test 6.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 31 Commonly used methods are various types of optotypes that may be 1. The last three tests are crude tests and meant for children in second year. 2. dimension and colour. Record vision in normally lighted room. . 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. Landolts broken C 3. 1. Coin test 7. But a non literate child has to be tested on other charts. what he is expected to tell. HOTV Test 5. recognise shape. X is added to HOTV. 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. Miniature toy test. Snellen’s chart 2. Landots broken C 5. Snellen’s tumbling E test 4.

6. Cycloplegia (a) Neurological-internal ophthalmoplegia (b) Drug induced cycloplegia 4.G. Arabic and some other letters.P and Peterson R. third edition p-251-255. 5. preferably mother to be present near the child but forbid her to prompt the child. Little Brown. 3.B. A child may turn the head to see with the better eye. Nystagmus 3. . Examination of colour vision. REFERENCES 1. Incorrectable distant vision 2. Saunders Company. 4. First note the vision with both eyes open. edited by Scheie H. Boger W.A. 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. Other causes of diminished near vision are 1. edited by Deborah Pavan langston..32 PEDIATRIC OPHTHALMOLOGY 2. and Albert D.M. 7. 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. 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. Normal eye in infancy and childhood in Text book of ophthalmology.B. Some children generally above five years of age can memorise the whole of the Snellen’s 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. After 15 years of age the field examination is done as in adults. Between 10-15 years automatic perimeters may be helpful. It is very difficult to elicit field defect under 10 years of age. Allow one of the parents or grand parents.. While examining one eye the other eye should be occluded completely and watch that child does not peek over the occluder. Because a child with gross visual loss in one eye will resent closure of better eye and may refuse to be tested. 2. Examination of near vision in children. Malingering. If a child complains of diminished near vision the first test is to exclude hypermetropia by cycloplegic refraction. Paediatric Ophthalmology in Manual of Ocular Diagnosis and Therapy. . Shaffer D. Keep the head of the child in primary position. W. 9th edition p-279-280. Philadelphia 1977.

R.T. Lange Medical Publication Singapore 1983. The preliminary examination and assessment of vision in Strabismus simplified. p-1-25. Ophthalmologic examination of the infant. Singh D. Masson New York 1977. Hanrubia.K. Chronology of Development in System of Ophthalmology. New Delhi 2001. Nickel BL. p-2. Edited by smith J.. Edited by Harley M. Butterworth. In textbook of ophthalmology. Study of Congenital nystagmus Br Jr.EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 33 3. Asbury T. 17. Ltd. Duke Elder S. Yee R. Mc Dermott M. 16. W. . Edited by van Heuven WAJ and Zwaan J.D. Safir A. Saunders Company.1-2. Henry Kimpton London 1963. Ramanjit Shihota and Radhika Tandon. Harcourt Brace and Company 1992.R. Visual acuity contrast sensitivity and test for potential vision in Theory and practice of optics and refraction. . Evaluation of Visual Function in Neonate and Infant in Paediatric Ophthalmology Vol. Philadelphia 1977. Harcourt Brace and Company 1992. and Albert D. 4. p-128-129. 11. Duke Elder S. Khurana A.M. OPL 64 : 926-930. Does this baby see in Decision making in Ophthalmology. Henry Kimpton. 1983.. Hoyt. Diamond G. Paediatric Cataract CME series All India ophtlamological society. Billson F. 13. Y. 10. 5. Zwaan J. Merker C. Corneal hypesthesia in Decision making in ophthalmology. 9th edition p-279-280. Harcourt Brace 1992. III part 1. Uncorrectable poor vision in a child in Decision making in ophthalmology . Page 820. Zwaan J. and Yanoff M. .M. . . 7. Heinemann Oxford 2003. . C. Vol. 12. Modern Publishers. Vaughan D. Cloudy cornea in neonate in Decision making in ophthalmology.. Normal eye in infancy and childhood in Text book of Ophthalmology.T. III.T. 9. New Delhi 1999. 18. System of ophthalmology. Part-II. . and Cook C. .B. Edited by Podos S.6 Mosby London 1993. 14. 6. 10th edition p-279-285. Harcourt Brace and Company 1992.T. W B Saunders Company Philadelphia 1983. edited by van Heuven WAJ and Zwaan J T P-174.G. 8. Goldhammer Y. survophth 26 : 177-189. . Flyon J. 1980. Edited by van Heuven WAJ and Zwaan J. . p-93-97.L. 19. . p-291-312. Evaluating Vision in preverbal and preliterate infants. Edited by van Heuven WAJ and Zwaan J. Special subjects of paediatric interest in General ophthalmology. Sharma P. . edited by Scheie H.. Balon. 15. London 1964. Paradoxical pupillary reaction in Neuroophthalmology update. .D. Assesment of visual function in Parsons diseases of the eye.T.S.. p-138-139. 19th edition. first edition p-31-51 BI Churchill Livingstone Pvt. P-39-42. . 1st Edition p-55-62. Vol. Vol-5. I. p-124-125.L. second edition. Zwaan J.T.A.W. .T. ..

CHAPTER 3 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. E. and malignancy common to both. The lower lid extends from the inferior orbital margin to lid margin just reaching the lower limbus. The disorders of the lid can be congenital or acquired. covering the upper 2 mm of cornea.3 A. allergy. C. Trauma is common disorder in all ages.2. Besides protection. B. chief function of which is protective. Spread the tear over the ocular surface. inflammation. muscles. the lids are so tight that they cannot be lifted off the globe or everted with ease. A. Regulate entry of light in the pupil 2. ANATOMY OF THE LIDS1. Lids are two multiple layered folds. The acquired conditions outnumber the congenital anomalies. The lids indirectly help to drain the tear from the conjunctiva and propel it down the nasolacrimal duct. motor and sensory nerves and abundant blood supply. The lids consist of an assorted number of tissues:. vascular and neurological disorders besides usual infection. It spreads from the eyebrow above to lid margin below and from lateral wall of nose to the zygomatic bone laterally. 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. fluids and gases. 5 mm at birth. In adults it is about 30mm in length in contrast to 18. the lids have two more functions and they are: 1. D. The normal lids in adults can be lifted off the globe and everted without any difficult but in children. Benign growths are as common as in adults. Malignant growth of the lid in childhood is almost non-existent. The junction of the conjunctiva and the skin form the lid margin hence the lids are seats of dermatological. D. The width of 34 . C. B.the skin with its glands.The space between the two lids is called inter palpebral aperture (IPA) or Palpebral fissure. The upper lid is larger than the lower one. They protect the eye from external foreign bodies.

The two lacrimal puncta initiate the lacrimal drainage. Skin B. The layers of the lids are A.S. they are constantly in touch with the globe and initiate the lacrimal drainage. the medial and lateral. The fundus of the lacrimal sac is enclosed in between these two5. and lateral fibers from the tarsal plate. It splits into two leaves. 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. H. upper and lower puncta. This structure represents the nictitating membrane of lower vertebrates with out any function but is invariably involved in pathological process of the conjunctiva. THE LAYERS OF THE LID The lid is multi-layered structure of both ecto and mesodermal origin. They are circular or oval about 1mm wide in normal eye.DISORDER OF LID IN CHILDREN 35 IPA in adult is 9 mm while at birth it is 8 mm.P. The fibrous layer : 1. I. The corresponding medial junction is called medial canthus that is rounded medially. The tarsalplate 2. It is a mound of conjunctiva with small hair along with their glands. It also contains goblet cells. It is highly vascular. The other function of the ligament is to keep the lids stretched horizontally. It is palpable as a horizontal cord under the skin when the lateral canthus is pulled laterally. They are situated 2mm lateral to the medial canthus in the inter marginal strip. It lies anterior to the lacrimal sac. The caruncle represents the vestigial third lid. The IPA is a conch shaped space bounded by the two lids. The plica semilunaries4 is a vertical arcuate fold of conjunctiva with concavity towards the cornea. levator palpebral superior 3. The epithelium is thickened. The lids join each other laterally in acute angle and the junction is called lateral canthus. F. The lacus lacrimalis is a shallow depression in the conjunctival surface of the lower lid in the medial side. There are two canthal ligaments. G. The structures in the medial canthus are caruncle. an anterior leaf and a relatively smaller posterior leaf. one in each lid. The orbital septum . The orbicularis 2. J. semi lunar fold and lacus lacrimalis. It has no definite function. to it is attached the lateral horn of L. to this is attached medial horn of the levator and fibrous extension of the tarsal plate. Muller’s muscle. it does not have skin of the lid. The lateral canthal ligament is less developed than the medial. It contains some plain muscles2. E. Layer of muscles : 1. rich in nerve supply and lymphatic. C.

The Muller’s Muscle8. There are only downy hair with their glands scattered thinly throughout. The levator palpebral superior. With age it may become loose enough to hang down at old age. It encircles the palpebral fissure like a sphincter and closes the lid. This in contrast to the levator and orbicularis is a non striated muscle. Lid margin. It is very loose. It is direct antagonist of orbicularis. There is no subcutaneous fat. It has two parts-the orbital and the palpebral. It reaches the lid in a wide aponeurosis that is divided in three slips. It is a direct antagonist of levator. The skin of a newborn is tighter than adult. The orbital part surrounds the orbital margin. Due to laxity of the skin. In fracture of paranasal sinuses. 3. the perceptal and pretarsal. some fibres blending with the anterior surface of tarsal plate. only due to firm attachment of the skin to the periostium6. This striated muscle.e. PEDIATRIC OPHTHALMOLOGY A. when present is confined to the lids. D. These horizontal lines are caused by cutaneous attachment of levator palpabral superior. The lateral horn of the aponeurosis is attached to lateral orbital tubercle and lateral canthal ligament. 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. 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. 2. 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. The conjunctiva—Tarsal conjunctiva E. B. Layer of Muscles 1.36 3. The canthal ligament’s 4. cheek and the forehead. (a) The main slip is attached to the superior border of the tarsalplate. They are obliterated in ptosis and edema of lid. The muscle originates from the under surface of lesser wing of sphenoid above and in front of the optic foramen. (b) Some fibers anterior to this pass through the fibers of the orbicularis and get inserted in the skin to form horizontal skin creases. pus and other effusion can accumulate under the skin. deep in the . air can get under the skin causing emphysema. Part of the aponeurosis blends with Whithnall ligament that acts as check ligament of the levator. it can be lifted off the subcutaneous tissue. The orbicularis oculi is a horizontally placed striated muscle supplied by seventh nerve. The Whitnall ligament. The palpebral part has two smaller divisions i. (c) The posterior most fibers are attached to the upper fornix to give it its depth. The Accumulation of fluid . The skin of the lid is thin and delicate but rich in blood supply and sensory nerves. Paralysis of orbicularis results in lagophthalmos. large amount of fluid like blood. Its function is to lift the upper lid and keep lifted. It travels between the roof of the orbit above and superior rectus below. The medial horn gets inserted in medial canthal ligament and frontomaxillary suture. supplied by upper division of the third cranial nerve is the main elevator of the upper lid. The palpebral part spills over like a sheet in the temple.

The septum prevents orbital fat to herniate in the lid. It augments the action of L. The tarsal plate. It is not a very rigid structure. The upper tarsal plate is larger than lower tarsus in all dimensions. by 2mm. It is supplied by cervical sympathetic. It is an accessory elevator of lid its paralysis causes mild ptosis of upper lid.DISORDER OF LID IN CHILDREN 37 main aponeurosis. The meibomian glands are visible through the conjunctiva. The lower border of the upper tarsus is slightly concave. It is about 30mm long. This structure is also known as palpebral fascia. Medially it is attached posterior to the fossa for lacrimal sac and laterally to the orbital tubercle. A similar but less developed muscle arises from the inferior rectus and gets attached to the lower border of tarsus of lower lid. The septum orbitale along with tarsal plate form a vertical barrier that separates the orbit from the lid. They extends from canthus to canthus in each lid are about 3 mm in thickness. The orbital septum. 2. Its paralysis causes elevation of the lower lid margin and the condition is called upside down ptosis. 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. 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. It ripples with movement of the globe. The lid margin The lid margins form the borders of palpebral fissure. The tarsal glands are visible through the normal tarsal conjunctiva as light streaks. The tarsal plate along with orbiculris that is loosely attached to its anterior surface. It is a sheet of fibrous tissue of variable thickness. follicle.P. 3. This is a fibrous band that runs horizontally above the aponeurosis of levator palpebral superior from Whitnall tubercle on the zygomatic bone. The normal tarsal conjunctiva is translucent over the tarsal plate. It limits the infective process to reach the lid from orbit and vice versa. so long it is translucent. D. form a strong protective layer when the lids are shut. The tarsal conjunctiva cannot be lifted off the tarsus. its posterior border is sharp and must remain constantly in touch with the globe for proper spread and drainage of tear. It is pushed posteriorly by effusion in lid and forward by same pathology behind. The Whitnall ligament9. It is one of the important landmarks in ptosis surgery. The tarsal conjunctiva The tarsal conjunctiva lines the tarsi. The anterior border . It originates from the periorbita of the orbital margin and gets attached to the peripheral margin of the tarsal plates. The inner surface of the tarsal plate is lined by tarsal conjunctiva. The duct become obscure due to edema. 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. See above. C. papillae and scar in the tarsal conjunctiva. It is pierced by aponeurosis of levator in the upper lid and fibers from interior rectus in the lower lid. E. form the inter marginal strip of the lid margin to the fornices in each eye. 9 to 12mm in height and 1mm thick. 4. The Fibrous Layer 1. Canthal ligaments. The upper tarsus has about 25 glands while the lower has only 12 to 15 glands. Either side of the tarsal plate is anchored to the corresponding canthal ligament. The upper tarsal glands are longer and have wider lumen than lower glands. It gives attachment to levator aponeurosis.S.

unilateral congenital anomaly is no exception. The arteries of the lid ultimately form two arterial arcades in each lid.39. THE BLOOD SUPPLY The lids have very rich blood supply this is the cause of rapid healing of lid wounds. the nerve to Muller’s muscles is via sympathetic. in the pretarsal and retrotarsal. cavernous sinus and pterygoid plexus. one above the tarsal plate and other near the lid margin. The lids get their arterial supply from both internal and external carotid arteries. an anomalous lid. The glands of Zeis have no lumen they produce lipid. The orifices of the meibomian glands open on the lid margin behind the lashes. At the base of the lashes open the modified sebaceous glands the glands of Moll and Zeis. However. if it covers the pupil may result in . Children with congenital lid anomalies are brought to physician generally for cosmetic blemish or exposure of the globe. THE NERVE SUPPLY The motor supply is from facial and oculomotor. A common combination is congenital anomalies of lid with cranio facial and mandibulo facial deformity.38 PEDIATRIC OPHTHALMOLOGY is rounded. which drain in external juglar. the sensory is from trigeminal. Those from medial side drain in sub mandibullar group of nodes. the anterior containing skin and orbicularis and the posterior containing tarsus and conjunctiva along this line.41 A. CONGENITAL ANOMALIES OF THE LIDS38.e. Congenital anomalies of the lids are generally bilateral and symmetrical. the lashes arise from the anterior border in two or three rows. The congenital anomalies may be confined to the lid or may be associated with other anomalies of globe and systemic anomalies. There is a faint grey line along the lid margin. The blood supply is broadly divided in superficial system and deeper system the former is again divided into facial and orbital groups. The lid can be surgically split into two halves. The upper lid being larger has more and longer lashes than lower lid. THE LYMPHATIC DRAINAGE The lymphatic from lateral half drain into pre auricular nodes. THE VENOUS DRAINAGE The lids have two types of venous drainage i.40. 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 glands of Moll are modified apocrine sweat glands that pour the secretion in the lash follicle.

may present as a single skin tag or there may be four or five strands. Deformity of the interpalpebral aperture (a) Telecanthus (b) Epicanthus (c) Blepharo phimosis (d) Euriblepharon 3. Congenital anomalies of lid can be : 1. Franceschetti syndrome. they by themselves are almost symptom less. Medial strands are less common.e. It is not uncommon to find other congenital anomalies in the same eye i. 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.DISORDER OF LID IN CHILDREN 39 amblyopia that requires early management. TreacherCollin. Deformity of lid margin (a) Coloboma (b) Ankyloplepharon (c) Distichiasis (d) Entropion (e) Ectopion 2. B. Lower lid colobomas are wider and shallower. Coloboma of upper lid is generally unilateral. coloboma of the pupil. These are called ankyloblepharon filiformis adnatum. Large exposure of the globe due to coloboma of upper lid may also require urgent treatment. (b) Ankyloblepharon. these strands are called ankyloblepharon. Disorders of the lid skin (a) Blepharochalasis (b) Epiblepharon 5. They are a constant feature of mandibulo facial syndrome i. The separation is complete at the time of birth. central are least . Deformity of Lid Margin (a) Coloboma of the lid.e. Congenital Ptosis and allied conditions 4. The lower lid colobomas are seen at the lateral third of lower lid. a large rectangular defect may develop in the middle of the lid. Minor and rare deformities 1. They are more common on the lateral side. The colobomas generally have a broad base at the lid margin. they are not so well demarcated as coloboma of the upper lip and may be missed unless carefully looked. Some times few strands of skin may extend upon the inter palpebral aperture attached to the other lid. corneal opacity. The separation starts from the middle and spreads on each side. The lids are fused between third and sixth month intra uterine life and start separating with development of cilia and glands of the lid. exposing upper part of the globe and conjunctiva. Dermoid or dermolipoma may be present in Goldenhars Syndrome. However. it may involve the upper punctum and canaliculus. microphthalmos.

Congenital ectropion is also rare. Telecanthus is lateral displacement of medial canthi due to abnormal lengthening of medial canthal ligament . 2. It is generally bilateral. Ptosis with epicantus is a common presentation Blepharophimosis. It is a consistent finding in Down’s syndrome Epicanthus is seen as a vertical semilunar fold of skin on the medial side of the palpebral aperture with concavety towards the cornea. Epicanthus tarsalis. There is an intact inter marginal strip between normal lashes and distichiasis. it can be : Epicanthus supraciliary when the skin folds starts just below the eyebrow. This bilateral symmetrical congenital anomaly is very common in otherwise normal infants and disappear with age. (e) Ectropion. it is more common on the lateral side. . In telecanthus this increases proportionately. It is not uncommon to confuse them with cicatricial trichiasis in adults. It is a distinct feature of oriental races. All the lids may be involved. here the lid fold starts at the level of upper border of tarsus. Isolated telecanthus does not produce any symptom and does not require any treatment. telecanthus. epicanthus inverses or ptosis. they may be confused with epiblepharon. Epicanthus inverses are same as any epicanthus only difference being that skin folds starts from the crease of the lower lid. frequently seen with epicanthicfold. It may be present as an isolated defect or may be associated with blepharophimosis. the children are brought for cosmetic blemish. Treatment consists of repeated epilation when they are a few other wise plastic procedures are required to turn the lashed forwards. The strands do not contain any other tissue except skin and few capillaries. telecanthus and epicanthus inversus represent Komoto’s Tetrad42. (b) Epicanthus. In most of the children the epicanthus disappears with age. The distichiatic lashes grow posteriorly and rub against the cornea and conjunctiva. Congenital entropion lids are very rare. It is also seen in Waardenburgs syndrome. Normal inter canthal distance is generally half of inter pupillary distance. (d) Entropion. According to its extent. (c) Distichiasis. True telecanthus must be differentiated from hypertelorism which is increased distance between two orbits Telecanthus may be associated with epicanthus. In cicatricial trichiasis. They are more common in females. the in turned lashes arise from the anterior margin of the lid. Large epicanthus fold causes pseudo convergent squint that disappears on abliterating the epicanthal fold. Simple epicanthus is almost symptom free. Epicanthus palpebralis. 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.40 PEDIATRIC OPHTHALMOLOGY common. Deformity of the Interpalpebral Aperture (a) Telecanthus. ptosis. here the lid fold starts at the level of normal lid fold this is the commonest type of epicanthus. The strands can be cut without difficulty soon after birth. distichiasis and trichiasis. This is due to vertical shortening of the lid skin following change in subcutaneous tissue.

This is generally familial bilateral condition where there is atrophy of the dermis of the upper lid skin that hangs loose. Simple blepharophimosis is rare. It is generally associated with epicanthus inversus. Minor and Rare Deformities (a) Ablepharon (Cryptophthalmia).DISORDER OF LID IN CHILDREN 41 Differential diagnosis consists of epiblebharon where there is no superior palpebral fold but there may be trichiasis. congenital coloboma of lower lid. The orbital fat may protrude in to the overhanging skin. See page 43. 5. Rarely there may be trichiasis. It is corrected for cosmetic reasons by oculoplasitc repair. (d) Euriblepharon (Euryblepharon). There may be amblyopia due to associated ptosis. 3. lagophthalmos. Congenital ptosis is commonest congenital anomaly of the lid for which a child may be brought. covering the pupil. Epicanthus may be associated with punctal stenosis. specially seeking treatment for cosmetic defect. Blepharophimosis may give impression of pseudo convergent squint. When the patient sleeps a part of the lower bulbar conjunctiva may be visible. Disorders of the lid skin (a) Blepharochalasis. as the neonate keeps the eyes closed most of the time and normal palpebral fissure is very narrow as compared to adults. (c) Blepharophimosis . This condition should not be confused with other causes of enlargement of IPA i. There is mild lagophthalmos. In this case the vertical distance of the lid is short both lids. CONGENITAL PTOSIS AND ALLIED CONDITIONS A. This is always bilateral and symmetrical. The skin of the face passes over a deformed eyeball to the cheek without inter-palpebral aperture. In this case there is uniform enlargement of interpalpebral fissure. It may be present at birth but not much attention is given to ptosis. (b) Microblepharon. Congenital Ptosis and Allied Disorders. (c) Mongoloid obliquity of lid is up and outward obliquity of the outer canthus. 4. (d) Anti mongoloid Obliquity is downward displacement of outer canthus. lower epiblepharon is more common. There is no defect in muscles. The juvenile form starts between seven to twenty years with episodic attacks of lid swelling (b) Epiblepharon. The child keeps the chin raised and there are prominent furrows on the forehead to compensate associated ptosis. It may be associated with congenital entropion of lower lid.e. However by three months of . telecanthus and ptosis. there is displacement of outer canthus laterally. proptosis. 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. ectropion. It can be congenital or juvenile. multiple surgeries may be required. This is congenital reduction of length and width of interpalpebral fissure. may be involved. A very rare condition in which there is failure of lid folds to develop in to lid. This is a constant feature of monogolism.

Simple congenital ptosis may be unilateral or bilateral. C. The lid covering the pupillary area 2. In very small number of cases. E. These changes are due to dystrophic changes in L. congenital Horner’s Syndrome is seen. Superior rectus and inferior oblique under action. B. 3. 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. accommodation and pupillary reaction. Involvement of other extra ocular muscle may be central in origin. Congenital ptosis with superior rectus under action is frequent. In orders to have better vision the child uses frontalis. blepharophimosis D. 2. However congenital mal-development of third nerve nucleus or its trunk may be a contributory factor. 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.P. It is not only the elevation that is subnormal downward movement of the lid is also defective (lid lag). Congenital ptosis can be simple unassociated with other extra ocular muscle palsy or may be associated with 1.. Most of the congenital ptosis are due to mal-development of the levator palpebral superior. Associated strabismus. Other extra ocular muscle palsy. 3. They are more marked in bilateral cases. Many a times a child may reach second decade not being aware of ptosis in mild cases. so may more rare myotonia. 4. Myogenic cause especially neonatal myasthenia may present as ptosis. This procedure is utilised to measure levator function.42 PEDIATRIC OPHTHALMOLOGY age the parents become aware of ptosis especially if it is unilateral. Synkinetic movements of the lid. Though vision is unaffected amblyopia may develop due to 1. Generally levators function is moderate to mild in congenital ptosis and remains unaffected throughout life. Associated myopic astigmatism. A miotic pupil with ptosis points towards Horner’s syndrome. 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. Action of the frontalis can be obliterated by pressing this muscle against the frontal bone. Paradoxical movement of the lid is generally . Raising of chin and arching of eye brow develop with age. Lid lag is not seen in any other ptosis except congenital ptosis. F. which elevates the lid slightly. Both the sexes are equally affected. Congenital ptosis has strong hereditary tendency. Superior rectus palsy. Throwing the head back (chin up) helps to compensate lowered position of lid.S. It can be associated with other deformities of the lid like epicanthus. Generally there is normal vision. 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.

Change of colour. New growth 5. This is seen with congenital ptosis in infancy. Raised position of the lid is not maintained if the mouth is kept open. Synkinetic movement of lid with ptosis (Jaw winking). The paradoxical movement persists for rest of life. Injury 4. with mouth closed there is mild ptosis as the patient opens the mouth the lid drifts upwards. ptosis increases. radiation. Sometimes forward protrusion of the jaw may produce same phenomenon Marcus Gunn phenomenon is seen only on sucking or chewing. (a) The Inflammatory Causes of the Lids may be (i) Infection of the lids. Infection 2. 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. It may be present without ptosis as well 1. Vascular 6. 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 . it is not seen on smiling or coughing. looking down. Non Specific 1. chemical. If the jaw is moved towards the side of effected eye. Specific like 1. B. Ecchymosis 3. The mother notices abnormal movement of the upper lid in an act of sucking. This is the commonest congenital paradoxical movement of the lid. ACQUIRED DISORDERS OF THE LIDS IN CHILDREN Acquired disorders of the lids in the children can be A. It is always unilateral. Neurological. Non Specific like as 1. thermal. Inflammation 3. In primary position. 2. Synkinetic movements of the lids. The cause of this phenomenon is congenital misdirection of some of the fibers of trigeminal that supply the pterygoid muscle. . Edema 2. to the oculomotor nerve.Blunt.e. Marcus Gunn phenomenon. A.DISORDER OF LID IN CHILDREN 43 present with congenital ptosis. Edema.

Hence called Black eye. Allergic edema may subside with oral and rarely injectable antihistamine. It may involve any or all the lids. Treatment of lid edema is essentially treatment of it’s cause. or along the sheath of the extra ocular muscles.44 ● ● ● ● PEDIATRIC OPHTHALMOLOGY Bacterial-lid abscess. the colour of lid changes from red to bluish green to black. hypoproteinaemia. Acute angioneurotic edema. giving rise to Ecchymosis.the child may get up with edema of the lids that pass off within few hours. hence trauma easily causes extravasations of blood in the lids due to laxity of lid skin. manifesting only in a particular time of the day. Fungal. haemophilia. scalp. Viral-herpeszoster. In injury to scalp.local or systemic and hot fomentation. (ii) Infection of globe ● ● ● ● ● (iii) Orbital cellulitis. chickenpox. cellulitis lid. 2. (b) Blood dyscrasia—Purpura. Ecchymosis of the Lid. Steroids are indicated in angioneurotic edema. (iv) Cavernous sinus thrombosis. Presence of ecchymosis may be frightening but . The causes of black eyes are: (a) Blunt injury to the lid. Treatment of edema. myxedema and thyrotoxicosis. atopic dermitis. stye. insect bite. The lids are very vascular. dermatitis medicamentosa. leukaemia. The edema may be transient. 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. Ecchymosis of the lid may be localised to lid only. (c) Systemic conditions that produce edema of lids are acute and chronic nephritis. orbit. Edema may be acute or chronic. muscle plane. (v) Proptosis (b) Allergic. The ecchymotic lid is swollen and bluish green in colour. measles. Blood accumulates in the subcutaneous space. Severe uveitis Endophthalmitis Purulent conjunctivitis Panophthalmitis Epidermic conjunctivitis. As time passes. middle cranial fossa. The local infective causes are treated by antibiotic . Parasitic. (c) Neuroblastoma. molluscum. 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. In infants it may have a red discoloration. It may be associated with sub conjunctival haemorrhage with or without rupture of the globe. herpessimplex. This is generally due to systemic causes requiring pediatric consultation.

atopic. seborrhic. It is always associated with chronic blepharo . Skin and subcutaneous tissues of the lid.2.3. which are easily removed and on removal there are no ulcers or hemorrhagic spots that are common in ulcerative blepharitis. Inflammation of the lid margin is called Blepharitis1. C. They are best rubbed off after being moistened by warm water for few minutes. B. and allergic. However after few hours cold compress does not help. The Causes of blepharitis are bacterial. Involvement of upper lid is more extensive than lower lid. Hypopigmentation is seen following injury burn and vitiligo. Blepharitis is broadly divided into squamous. However some of the birthmarks are permanent like naevus. The cursts are difficult to remove.e. Seborrhic blepharitis may produce mild chronic conjunctivitis and punctate keratitis in lower part of cornea.DISORDER OF LID IN CHILDREN 45 not always vision threatening and most of the time disappears without treatment. involves both the lids on both sides. Generally the colour of the lids is similar to that of face and forehead.4. Treatment of ecchymosis. There are yellow crusts at the base of the lashes that may extend over the rounded anterior lid margin and the skin. It may be simple staphylococcal or it may be a combination of seborrhoea and staphylococcal infection. The squamous (seborrhic) blepharitis This is basically disorders of gland of Zeis and is associated with seborrhic dermatitis of eyebrow. and small ulcers are seen. The condition is mild. produces more complication and is difficult to eradicate. madarosis and trichiasis. The former is mostly seborrhic in nature while later is chronic infection by staphylococci or due to its toxins. blood dyscrasia or neuroblastoma or traumatic optic neuropathy. parasitic. without wetting them. Ulcerative blepharitis This is more troublesome. It takes about seven to ten days for simple ecchymosis to disappear. Birthmarks may last for first few years and gradually disappear. scalp and nasolabial fold. On long run there may be tylosis. The lid margin (Blepharitis). the underlying skin is found to be hyperaemic. ulcerative (staphylococcal) and parasitic. As a first aid cold compress may reduce the swelling. This makes it difficult to treat. On removal of the crusts. Some times there may be localised colour changes due to ecchymosis which is temporary and passes off. INFLAMMATION OF THE LID Inflammatory process may involve : A. The lid margin has dry scales or greasy material. Sturge Weber syndrome and neuro fibromatosis. However ecchymosis of any kind may be just a superficial sign of a deeper malady i. Glands of the lid margin. Colour change of the lid. It generally starts after 3 years of age. fracture base of skull. 3. Echymosis of lid per se does not require any treatment. Analgesics reduce pain and inflammation.

If children do not respond to local treatment systemic antibiotic may be required for seven to ten days. 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. combs hairbrushes should be washed in boiling water to avoid . Treatment of seborrhic blepharitis consists of management of systemic seborrhoea i.46 conjunctivis and may produce superficial punctate keratitis.15 Common parasitic involvement of the lid margin is pediculosis. conjunctiva and lower cornea. Anti pediculosis shampoo after proper dilution may be applied to the lid margin. p. They are visible on naked eye when many.e. bedding. use of suitable antidandruff shampoo for the scalp and eyebrow. (ii) Follow up. Treatment of ulcerative blepharitis is prolonged. This should precede application of any ophthalmic eye ointment in greasy base. Treatment. The cloths. The whole procedure is repeated at least three times a day. (iv) Associated errors of refraction. The children should be taught proper hygiene of the face and hands. 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. The treatment is mainly treatment of systemic pediculosis in all the contacts. but may require magnification when few. PEDIATRIC OPHTHALMOLOGY Management of blepharitis is difficult. In co-operative and older children the parasites may be picked up with epilation forceps or destroyed by direct application of cryo. Personal hygiene is the key word in management of pediculosis and phthiriasis. premethrine 1%. for months. pubis. while they get the other two types from their infected parents. regular cleaning of the face. frequent stye. madarosis. servants or baby sitters. keratitis and epiphora. 20% fluorescein sodium have been used with good result. steroid with antibiotic ointment may be rubbed on the lid margin—three times a day and gradually tapered off. to prevent secondary infection. There are three types of these arthropods that involve the lid margin. (iii) Personal hygiene. The oily base acts as mechanical repellent for aqueous shampoo which other wise may get into the conjunctiva causing chemical conjunctivitis. Parasitic blepharitis Pediculosis and phthiriasis of lids14. same antibiotic is instilled in the conjunctival sac at bed time. Complications of ulcerative blepharitis consist of—Tylosis. They are pediculosis-corporis. redness and secondary infection of the lid margin. trichiasis. The drugs commonly used as shampoo are benzene hexachloride. (ii) Broad Spectrum antibiotic is rubbed on the lid margin. chronic conjunctivis. The parasites produce chronic itching. Numbers of parasites vary from a few to numerous. The child gets the infection due to p. (i) Local Treatment. Should be corrected after proper retinoscopy under cycloplegia. (Lindane) 1%. capitis and p. capitis from siblings or playmates. Local instillation of broad-spectrum antibiotic drops.

In such children long acting sulpha or erythromycin in consultation with pediatrician should be given. Preauricular lymph nodes are always enlarged that may be tender. Associated conjunctivitis is frequent. Disorders of glands of lid margin 1. There is diffuse swelling of the lid on either side of the stye. hence styes are more frequent and more numerous in upper lid. There may be multiple crops of styes. Both lids may be affected. The position of an early stye in the lid can be pin pointed by moving the finger on the lid margin. pus is epilated. The stye is an acute staphylococcal infection of gland of Zeis and Moll. . The patient winces as the exploring finger passes over the stye. Styes of outer canthi are most painful. Treatment of Stye The treatment is similar to treatment of a boil that consists of local dry hot fomentation. Each lid may have single to multiple styes. Repeated attacks of styes are very common.DISORDER OF LID IN CHILDREN 47 re-infection in carriers and infected children. Proper lid hygiene should be maintained. Other predisposing factors are uncorrected errors of refraction that leads to asthenoipa. Other predisposing factors are chronic seborrhic and staphylococcal blepharitis . Tendency of stye runs in families. A child with asthenopia has tendency to rub the eyes with fingers frequently. With above treatment either the stye subsides by evacuation of pus or the lash round which the stye has developed. systemic analgesic. The upper lid has more glands of Zeis and Moll. The contacts should also be treated vigorously and repeatedly for pediculosis. it should be corrected by appropriate glasses following refaction under cycloplegia. It is a painful condition that begins as tender red spot on the lid margin. (ii) Prevention of recurrence. Styes situated near the canthi are more painful than in the middle of the lid. They are very common between five years to fifteen years. Management of Stye Management of stye consists of (i) Treatment of stye. However doxycyclin is not preferred in children under eight years as it may cause permanent staining of the teeth. B. This introduces infection in the lid margin resulting in repeated styes that disappear with correction of errors of refraction. In chronic and recurrent stye doxycyclin 100mg daily for ten days gives relief. Prevention of recurrence As error of refraction is one of the important predisposing factors. Development of stye after measles is very common. Stye (Hordeolum externum). There may be a single stye that may be cured spontaneously not to appear again. Boys and girls are equally affected. (iii) Management of complications. anti-inflammatory drug and systemic antibiotic. Local antibiotic eye ointment at bedtime prevents matting of lid margins at night. Stye itself can induce slight astigmation hence refraction should be under taken only when the child is free from stye. Cleaning the lid margins and application of broad spectrum antibiotic eye ointment at night for at least two months after the stye has subsided. All lids may be affected at the same times.

systemic analgesic and anti-inflammatory drugs. 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. The chalazion is well circumscribed and smooth without a true capsule of a cyst. This results in stagnation of oily meibomian discharge in the acini of the glands. preauricular glands are not enlarged. 2.e. neither painful nor tender. giving rise to a sessile papillomatous granuloma. On evertion of the lid the conjunctiva over the chalazion shows a bluish coloration. Small chalazion may resolve without any treatment or simply by frequent hot fomentation. It differs from stye in following points: It is chronic granuloma. Sometimes chalazion may be associated with stye as well.48 PEDIATRIC OPHTHALMOLOGY Cutting of consumption of sweets like candies.22 Many of the chalazia resolve without treatment. In contrast to stye. No attempt should be made to drain the acute chalazion unless pain and . If it does not resolve by it self-following changes may be seen:. any or all lids may be involved separately or simultaneously. In children chalazia should be incised under general anaesthesia. (b) It may less frequently rupture on the skin surface. Otherwise chalazion share same epidimiology as stye i. systemic broad-spectrum antibiotic. There may be multiple chalazia in the same lid or in all the lids. (iii) The chalazion gets secondarily infected and becomes severely painful. The skin over the chalazion can be moved. There may be repeated crops of chalazia. Chalazion is a selflimiting inflammation. This pent up lipid acts as an irritant that result in a granuloma with giant cell without caceasion. chocolates and supplementation of food with multi vitamin orally helps in reduction of recurrence. Intra lesion injection of steroid: 10mg of triamcinolone acetonide is injected in the chalazion under local anaesthesia from conjunctival surface. (i) The growth increases in size and the central core may liquefy forming a tarsal cyst that may cause pseudo ptosis and induce astigmatism. Chalazion. it is a disorder of children and young adults and it is equally common in both sexes. chalazion is a chronic granulomatous inflammation of meibomian glands. The chalazion is heralded by changes in the duct of the meibomian gland which are obstructed due to infection spreading from the lid margin. A patient may require two to three such shots. It is called Hordeolum internum. (ii) It bursts on (a) Most commonly on the conjunctival surface. Management of chalazia21. (c) Rarely it may protrude through the opening of meibomian orifice. Hordeolum internum requires more energetic treatment that consists of dry hot fomentation. Chalazia in children never undergo neoplastic change. Though meibomian cell carcinoma after 40 years may initially present as chalazia. Children should be instructed to keep the hands clean and nails trimmed. It is more common with uncorrected errors of refraction.

C. (a) Chicken pox (Varicella)16. may produce superficial keratitis pseudo dendretic keratitis and disciformkeraitis. Antiviral drugs have not proved to be effective. bacterial and fungal. Herpes zoster is classically unilateral others are bilateral all the above conditions have or may have systemic involvement. Prevention is similar to that of stye i. long term local application of broad spectrum antibiotic on the lid margin. may develop in conjunctiva simulating phlycten. 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. There may be ulceration or vesicles on the conjunctiva. The child should get full dose of cycloplegia. correction of errors of refraction.DISORDER OF LID IN CHILDREN 49 inflammation has subsided. Ocular management. (b) Measles (Rubeola. 1. Conjunctivial involvement is universal. the vesicles can be seen on the lid margin as well. There is no known method to immunise the child against chicken pox. Antibiotics are used to prevent secondary bacterial infection. face and hands. Measles is very common among non-immunised children. these are slightly raised and red. hygiene of lids. if needed atropine may be used. it starts as hyperaemia with vesicle formation. it starts as . Rhinosporidiosis of tarsal conjunctiva. there is no specific systemic treatment. may involve the conjunctiva. Differential diagnosis of burst chalazion consist of : Foreign body granuloma.24. They can be viral. Cutaneous and subcutaneous inflammation of the lids All infective processes that involve the skin of the face and forehead can affect the lid.e. They may be separate or merge with each other. as well as cornea. The lid it is involved as part of generalised hyperaemic rashes that become papules. It mainly affects the respiratory tract and conjunctiva. Treatment. The child should be observed for possibility of pneumonia or encephalitis. Morbilli)23. Immunoglobulins have favourable result when started with in three days of onset of skin rashes. If there is involvement of nasolacrimal duct it may lead to chronic dacryocystitis4. Frank ulcer is not uncommon. Haemangioma.18 is very common exanthematous disorder of the lid caused by varicella zoster virus. which subside within 10 to 12 days leaving no scar. Consists of relief of photophobia and watering due to corneal involvement. Papilloma.

frank corneal ulcer develop in malnourished children. Management Treatment is symptomatic. Keratitis and follicular conjunctivitis are toxic in nature. cellulitis lid. however it is less common in children. watering and photophobia due to pseudo dendritic ulcer. malnutrition and hypovitaminosis A. 2. pain less. Treatment. Some times they are seen on conjunctiva or cornea. Bland lotion to prevent sticking of eyelids. It is known to affect all parts of the eye and adnexa except the lens.50 PEDIATRIC OPHTHALMOLOGY catarrhal conjunctivitis that may be superimposed by secondary infection. multiple styes and blepharitis. which are umblicated.19. There are various stages of epithelial keratopathy. They are not as numerous as eruptions of chicken pox. Koplic spots develop on the conjunctiva. the interior of the punctured lesion is curetted by tincture iodine or weak solution of carbolic. 4. number may vary from few to few dozens all over the body. may be the pain is less sever in children. Compulsory immunisation is essential not only to save the eye but also life of the child. Children tolerate pain of herpes zoster better than adults. Herpeszoster ophthalmicus is an acute eruptive condition of the lid caused by zoster varicella virus that causes chicken pox otherwise. corneal involvment is common causing blepharospasm. The disease is self-limiting but may last for weeks. There is no known immunisation. 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. pannus formation and sub epithelial infiltration. not associated with fever or any other systemic manifestation. May be seen on the lid or lid margin or both. Herpeszoster ophthalmicus may be mild or may be very severe. Administration of additional does of water-soluble vitamin A 50. Children are not known to suffer from secondary zoster that is seen in immuno compromised adults. In herpes zoster ophthalmicus the infection lies dormant in posterior root ganglion of .U. Cycloplegie if cornea is involved. The lesions may appear any where on the body as dome shaped. There may be edema of the lid. The lesion can be treated with cryo as well. or small pox. This is a chronic self-limited virus disease of skin caused by poxvirus. It is contagious. 1. 3.000 I.20. Corneal involvement causes redness. When the lesion is near the puncta it may block the puncta causing epiphora. Blindness due to measles can be as high as 1% of all children affected mostly due to bacterial infection. They are best treated by puncturing each lesion either by needle or electrocautery. Corneal involvement may require broad spectrum anti biotic drops and cycloplegia. (d) Herpeszoster Ophthalmicus18. Broad spectrum antibiotic drops for conjunctivitis 5. translucent vesicles. there may be sub conjunctival haemorrhage. anti viral drugs are of not of much use.daily for four days orally. (c) Molluscum contagiosum. Herpes zoster ophthalmicus can occur at any age. It is morphologically similar to herpes simplex virus but differs in antigen.

Scar of the lid margin may cause notching of lid margin or trichiasis. .DISORDER OF LID IN CHILDREN 51 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. The crust fall in due course leaving shallow scars that may disappear in time. its severity is not directly proportionate to lid involvement. The vesicles are as a rule always unilateral they do to cross the mid line however associated edema of the forehead. Corneal involvement is varied it may be :Epithelial punctate infiltrates that stain better with rosebengal. It may happen without involvement of nasociliary nerve. Herpes zoster ophthalmicus starts as hyperaemic rash over the fore head and skin of the lid or lid margin. In initial stages herpes zoster of lid may be confused as allergic edema. server corneal involvement may be seen with mild lid involvement. However cornea may be involved without involvement of nasociliary nerve. (ii) Involvement of the globe ● Corneal involvement. lid and face may spill over the mid line giving a false impression of allergic edema or bacterial cellulitis. 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. Involvement of cornea is very common. insect bite. May be associated with mild fever and malaise. Preauricular lymph nodes are enlarged. This is almost sure indication of corneal involvement. Numular keratitis is known to change density from time to time without treatment. The eruptions are initially macular and soon converted into vesicles similar to chicken pox. If the lesion was deep it may leave permanent scar on the forehead. The vesicle may develop along the distribution of one or all the branches of first division of fifth nerve. This is called Hutchinson sign. stye or bacterial cellulitis Conjunctiva may show vesicles. Like chicken pox the vesicles take ten days to dry and crust. Involvement of maxillary division is rare. It is very common to develop secondary bacterial conjunctivitis. If the vesicles are present on the tip of the nose it means that nasocillary nerve is also involved. 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. Generally facial pain precedes appearance of rashes. lid or lateral side of the nose. chemosis and subconjunctival haemorrhage.

Loss of sensation may last for months. trichiasis is common. Delayed lid Changes. Children tolerate pain better.52 ● PEDIATRIC OPHTHALMOLOGY Scleral involvement. However it should be given along with systemic antiviral. Chronic anterior uveitis and secondary glaucoma may predispose cataract formation. Definitely reduce pain inflammation and scaring. Commonest being third nerve but any or all the extra ocular muscles may be affected due to involvement of their nerve supply. Secondary Glaucoma is due to associated trabeculitis and clogging of trabecular meshwork by inflammatory cells. Its role in zoster uveitis is not established. fortunately it is milder and short-lived in children. Anterior uvea may be involved either as primary inflammatory process of or secondary to keratitis. prevention of secondary bacterial infection. Scleritis is less common than keratitis it may be missed due to lid edema and conjunctivitis. however water soluble non-steroidal anti flamatory drugs helps to over come pain. Children tolerate post herpetic neuralgia better than adults. control of virimia. It is claimed to reduce post herpetic neuralgia. associated inflammation. Antiviral drugs reduce lid inflammation pain and shorten duration of disease but does not affect keratitis. Analgesia. management of uveokeratitis and its squeals. least secondary bacterial infection gets an upper hand in an already anaesthetic cornea. Mucus secreting conjunctivitis. Systemic steroids. Bland lotion or local antiviral ointment is prescribed for lid vesicle. There may be chroiditis as well as chorioretinitis. Antiviral. Optic neuritis is rare complications. Local steroids are required for uveo keratitis. atropthic patches on the iris that transilluminate and causes irregular semidilated pupil is also caused due to is ischemic necrosis of iris. Uveitis. neurotrophic perforation of cornea. there may be notching of the lid margin. Local antiviral drops or ointments are given along with local steroid in uveo keralitis. Such atrophic patches are not seen in herpes simplex. Management Management consists of reducing pain. herpes oticus (Ramsay Hunt Syndrome)18 herpes of palate. diminished corneal sensation start from very early phase of corneal involvement. corneal mucus plaque. Post herpetic complication Post herpatic neuralgia is a common feature. recurrent episcleritis and scleritis. Scleritis is less common in children. Forty percent of eyes with herpes zoster develop non-granulomatous iridocyctitis. generally recovers within a few months but in some cases it may last for years leading to neuro tropic keratitis requiring tarsorrhaphy. Neuro tropic keratitis. the eyes receiving local steroids should be stained frequently. . It may be mild with few kps or may have blood tinged hypopyon due to is ischemic necrosis of iris. ● ● (iii) Neurological lesions may cause encephalitis and cranial nerve palsy. There may be hypo pigmentation or hyper pigmentation with scaring of the skin. edema and inflammation. Other changes. which is also associated with keratouveitis.

Recurrent ocular infection. multiple styes. If it is known that the mother has genital herpes simplex it is better to deliver the child by caesarean section. There are two types of herpes simplex i. Lid abscess starts as diffuse swelling of the lid. The vesicles rapidly develop crust and disappear without scaring. which is due to type two herpes simplex virus transmitted via birth canal of infected mother. It may obliterate the inter palpebral fissure. It may be associated with acute folicular conjunctivitis. 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. It may start in the hair follicle and result as a boil or may be extensive to cause cellulitis. Skin of the lid. Systemic broad-spectrum antibiotic preferably by injection. Sever form of herpes simplex requires pediatric consultation for management. As the skin of the lid is thin. type one that involve the body above the waist and type two that cause lesions below the waist more commonly known as genital herpes. infected chalazion. pus may point at a dependent part. retrobulbar haemorrhage retro ocular mass. Bacterial infection of the skin of the lid Boil. Hot fomation. is prone to get infected. pre-eruptive stage of herpes zoster. Herpes simplex infection is very common infection caused by virus: Herpes hominis. 4. 3. Gradually the skin becomes lax and fluctuation develops. Haemophilus is an important cause of lid abscess in children.DISORDER OF LID IN CHILDREN 53 (e) Herpes simplex25. The vesicles may be bilateral in contrast to zoster. Herpes simplex can occur as: Neonatal infection. cellulitis and abscess of lids. Neo natal herpes simplex may present as few vesicles on the lid or conjunctivitis in the neonate. which is unilateral. 2. lots of exudates and pus can accumulate under this and ultimately result in abscess formation. Primary ocular infection: commonest age to get ocular infection is between six months to six years. Initially the lid is tense and red. Half the patients develop harpes simplex keratitis in the form of fine or coarse epithelial keratitis most of the corneal lesions heal without scar. Treatment consists of 1. The lids develop cluster of vesicles without predilection for any particular nerve. rhabdomyosarcoma.e. which is a sexually transmitted disease. 2. It is more common in hot seasons. like any other exposed part of the body. anti-inflammatory drugs to reduce pain and inflammation. Neonatal herpes simplex is life threatening condition when server. . Analgesic. Differential diagnosis consists of cavernous sinus thrombosis. very lax and very vascular. If pus points or fluctuation develops. Commonest organism is staphylococcus. cellulits lid and lid abscess are preseptal hence the globe is neither involved nor proptosis is produced. pus should be drained by a stab incision. Sometimes it may progress to disciform keratits. That may be few months to years Recurrent herpes simplex involves cornea and uvea and not the lids.

(c) Pseudo cancerous. Benign 2. Benign (a) Papilloma. JUVENILE XATHOGRANULOMA . (b) Squamous cell hyperplasia. they can be benign or malignant. B. (e) Seborrhic keratosis. Neural tumours : NEUROFIBROMATOSIS F. Malignant (a) Basal cell carcinoma (b) Squamous carcinoma B. Malignant C. Those involving deeper layers of lid. NAEVUS FLAMMEUS 3. Precancerous (a) Active keratosis (b) Intra epithelial epithelioma. CAVERNOUS HAEMANGIOMA 4. D. Others 1. Other classification is according to structure of the lid (in all ages). Melanocytic 1. LYMPHANGIOMA 2.54 PEDIATRIC OPHTHALMOLOGY TUMOURS OF LID IN PAEDIATRIC AGE GROUP26. STURGE-WEBER SYNDROME E. 3.27. CAPILLARY HAEMANGIOMA 2. (c) XERODERMA PIGMENTOSUM. A. (f) Inverted follicular keratosis.28 Tumours of the lid in all ages could be both congenital as well as acquired. (d) Kerato acanthoma. 2. All lid tumours are difficult to manage satisfactorily. Vascular 1. Epithelial 1. Tumours arising from glands of lid Meibomian cell carcinoma. Those involving superficial layers of lid. They have been classified as A.

Some are self-limiting. or globe. 1.30 Phakomas are a group of tumours. PHAKOMAS A. Skin and lid changes. . they may be localised to the lid or may involve orbit. in neurofibromatosis 1. It can be inherited as autosomal dominant trait. These are slightly raised hyper pigmented spots of variable size i. Tuberous sclerosis (Bournville’s disease) E. Arteriovenous malformation of retina and brain (Wybern Mason syndrome) F. Except for neurofibroma and xeroderma pigmentosa none show malignant changes in children. NF II cause bilateral acoustic neuroma besides usual features of neurofibroma. They become marked at puberty. Ataxia talengectasia (Louis Bar Syndrome) G. These sporadic cases may pass the gene to the next generation. It may be as common as one in every three thousand live births.DISORDER OF LID IN CHILDREN 55 Condition in capital letters mentioned above are found under 15 years of age and continue to persist in later age group if not treated. Spontaneous mutation may lead to sporadic cases. In fact they are congenital anomalies that result in tumour like malformations. All of them are Hamartomas i. skin brain (Rendu-Osler-Weber syndrome) A.e. may be present at birth or manifest later. type I and II commonly known as NF I and NF II. Neurofibromatosis Neurofibromatosis is one of the most common congenital phacomas of the lids. Skin and lids changes 2. The changes may be seen any where on the body as : (a) Caféaulait spot. Cavernous haemangioma of retina.e. tumours arising from the tissue components that are normally found at the involved site. The sporadic cases can pass the gene to the next generation. without sensory loss. Involvement of nervous system 4. which are congenital in nature. Ocular changes other than lid changes 3. Visceral changes. The changes may be present at birth but generally develop by five years of age. Angio matosis retinae (von Hippel-Lindus disease) D. The following components may be seen alone or in various combinations. Some of them may be congenital others develop at puberty. may have intra cranial extension or systemic involvement. Neurobibromatosis (von Reckling Hausen’s disease) B. Encephalo facial angiomatosis (Sturge-Weber syndrome) C. Genetically and clinically there are two types of neurofibroma i. All the other tumours are not seen in children. PHACOMATOSIS29. pin point to large areas of pigmentation. Either of the parents or both may show clinical evidence of disorder or may have subtle changes. they may cross over the midline six hyper pigmented spots or more than five millimetres is significant. They are seen in all races and equal in boys and girls.e. They have strong hereditary predisposition some of them may be sporadic.

Otherwise there may be proptosis due to retro bulbar mass with enlargement of orbit. In fact all parts of the eye except the lens may be involved. There may be changes in the bony spine. Congenital opaque nerve fibers are more common in persons who have evidence of neurofibromatosis. Visceral involvement. Treatment is mostly symptomatic minor defects can be treated with plastic reconstruction. The bulbar conjunctiva and lower formix may show small nodules between 1mm to 3mm. (d) Optic nerve. There may be hypertrophy of the skin of the face on rare occasion the condition may be bilateral. Otherwise multiple tortuous nerves may be visible on the conjunctiva. 2. meningies. 4. Any of the viscera can be involved in neurofibroma. There may be gonadal changes. (a) Conjunctiva. These are firm non-tender nodules. It is most commonly seen in the lid. 3. It is termed as associated congenital glaucoma. cranial nerves specially bilateral acoustic neuroma. Spinal nerves may be involved with involvement of spinal cord. Nervous system involvement. (g) Orbit. the inter palpebral fissure is obliterated initially the lid acquires a S shaped Curve gradually the lid margin develops a convexity down wards. Generally there are multiple neurofibromatous growths in the brain. Prominent corneal nerves may be visible on bio microscopy. which has all the features of buphthalmos that may be obscured by pseudo ptoisis. It may be large enough to be called elephantiasis neuro fibromatosa. These are pedunculated mass of various sizes any where on the skin including lid. (c) Uvea. (b) Cornea. Management There is no known treatment to eradicate neurofibromatosis. (f) Glaucoma is very common with neurofibroma of the lid. These are most disfiguring growths that are due to involvement of multiple superficial nerves which on palpation feel like a bag of worms.56 PEDIATRIC OPHTHALMOLOGY (b) Fibroma molluscum. The pathology may be due to presence of neuro fibroma at the angle. 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. Even autonomic nervous system may be involved. fibro vascular closure of angle or due to forward displacement of lens iris diaphragm due to growth of ciliary body. Pheochromocytoma has been reported more commonly in neurofibroma. If possible amblyopia should . Treatment of glaucoma in neurofibroma is unsatisfactory. Palpebral conjunctiva may be involved as part of involvement of the upper lid. (e) Retina is less commonly involved. the margin may over hang the lower lid. Exact mechanism of glaucoma is not well understood. (c) Plexiform neuro fibroma. Chloroid and ciliary body may show localised thickening of the nerve fibers. Both neuro ectodermal as well as mesodermal structure are affected. Bony faults in the sphenoid may lead to pulsating exophthalmos. Ocular involvement. the whole of the lid is involved and thickened including conjunctiva. Commonly involved uveal tissue is iris that may have multiple hyper-pigmented nodules similar to nevi.

both boys and girls are equally affected. Including enlargement of globe. There may be atrophy of ipsilateral cerebral and cerebellar cortex. choroidal haemangioma and associated glaucoma. There may be contra lateral hemipersis. Skin and lid involvement. It has following components. There may be non rhegmatogenous retinal detachment that may lead to secondary glaucoma in an eye that is already predisposed to glaucoma. The most widely accepted theory of raised intra ocular tension is increased episcleral pressure. 4. It is generally unilateral but in ten percent of cases it can be bilateral. 1. 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. large cornea.DISORDER OF LID IN CHILDREN 57 be treated. A clinical feature of glaucoma is similar to congenital buphthalmos. 3. the globe too has angiomatous malformation mostly in the uvea that result into heterochromia of iris. 1. Central nervous involvement. Encephalo trigeminal syndrome (Sturge-Weber syndrome) This congenital hamartomous anomaly differs form other phacomatoses by not being hereditary. have orange hue may be mistaken as amelanotic melanoma of choriod. 2. The facial angiomatosis that frequently involves both lids may be localised or have extensive involvement of not only facial skin but also the trunk. Angiomatosis retina .C. peripheral anterior synechea. The hemangioma due to its proximity to macula may cause cystoid macular edema. normal or deep A. which is difficult of treat medically. however if the medicines fail to reduce pressure below episcleral pressure these patients reqire surgical treatment. 2. Glaucoma may be managed medically or surgically. The angioma cause Jacksonian epileptoform attack on the contra lateral side. Homonymous hemianopia is frequent. Various degree of mental retardation is also known to take place. Skin involvement is present at birth as port wine stain. Ocular involvement. C. Sturge Weber syndrome does not cause ptosis which is a frequent feature of neurofibroma. Trabeculectomy may help. The choroidal haemangioma is generally single. Ocular involvement. Stretching of the globe may sub luxate the lens. It is seen in all races. The size and shape of the cutaneous angioma does not change with age. Sturge Weber syndrome glaucoma can be controlled by medical treatment. Other probable causes are malformation of angle. Glaucoma About 30% of eyes in Sturge Weber syndrome develop glaucoma by second year. Some times only dilated conjunctival vessels can be seen in the epsilateral side. Besides lid. large and situated in the posterior pole on the temporal side of the disc. Intra cranial haemangioma of the tapeto meninges on the same side as that of facial angioma is a constant feature. Visceral and other involvement. Generally there is hemiatrophy of the face on the affected side. The haemanagiomas have diffuse out line. B. Common complications of glaucoma surgery are intra operative hyphaema and large chroidal effusion in post-operative period. 3. they are raised. Central nervous system involvement.

In the eye it involves retina mostly and optic nerve head less commonly. Cutaneous.T. It is a autosomal dominant disorder. Cerebral. of different sizes. Ocular. Ocular Involvement. The angiomas are progressive and keep on increasing in size and number. kidney. All children with suspected angiomatosis retinae should undergo complete systemic examination including ultra sonography C. Almost all parts of the brain and spinal cord may develop haemangioblastomas. The endophytic tumours may bleed leading to vitreous band formation and traction detachment. Visceral. Argon laser therapy should have large spot.e. Management Treatment consists of obliterating the retinal angiomas as early as possible. There are mainly three locations where these angiomas are seen. This can be achieved by argon laser. peripapillary and papillary. low intensity and long duration. The angiomas are generally multiple. The ocular lesions are bilateral may be a symmetrical the components are : 1. Central nervous system involvement occurs after ocular symptoms. multiple treatment sessions may be required. As the angiomas grow. rhegmatogenous retinal detachment may lead to secondary glaucoma. sub retinal fluid and exudates accumulate round the lesion. Systemic involvement. it is generally diagnosed in first decade either due to diminished vision or on routine examination. Larger angiomas are round elevated bright red mass that have a feeding artery and draining vein both are dilated. Tuberous sclerosis (Bournville’s disease) This is a congenital hamartoma that differs from neurofibromatosis and Sturge Weber syndrome. The peripheral lesion arise as endophytum i. scan and MRI. Even after treatment recurrence in the same quadrant is possible. tortuous and of same caliber. peripheral. Similar growth can be seen in liver. they arise from inner retinal layer while peripapillary lesions arise from outer retinal layers and are diffuse. It is associated with haemangioblastoma of central nervous system and viscera. and pancreas. Retinal involvement starts first. 2.58 PEDIATRIC OPHTHALMOLOGY Angiomatosis of retina is a hereditary hamartomatous disorder of retinal vasculature and optic nerve. D. other parts of the globe orbit or adnexa are not involved. The exudate may accumulate under the macula forming a macular star and loss of central vision. Argon laser is better for posterior polar lesion while cryo is more effective in peripheral lesions. The optic nerve angioimas when situated in the substance of the optic nerve head may present as disc swelling. It is a hereditary condition all races and both sexes are equally affected. The early angiomas are small but have evidence of arterio venous shunt on fluorescein angiography. 4. It does not involve any ocular structure except retina and optic nerve. cryo or penetrating diathermy. The Cutaneous involvement. The disc angiomas are similar but do not develop arterio venous shunt. The Cutaneous involvement is not present at birth but . 3. Recurrent haemorrhage. The children do not live beyond second decade. It is a fatal condition.

Ocular involvement consists of hamartoma of retina that may be single or multiple generally raised and globular or may be flat. It may affect siblings. pigmented patches. that does not involve sebaceous glands. cerebellar or medullary may be seen in ventricles. benign multiple hyperpigmented growth ending in development of various types of diffuse malignant growths that may cause death in adolescence. Ocular structures commonly involved are: lids. Visceral Involvement Rounded hamartomas can be seen in almost all organs including heart. Lids contain various stages of growth ranging from erythema. hyperpigmented nodules to malignant growth. 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. Fibromas may be seen under or by the side of the nails. This is followed by so called adenoma sebaceum. PRE CANCEROUS EPITHELIAL LID TUMOUR IN CHILDREN Xerodermapigmentosum31. It may involve the spinal cord.e. It is due to hypersensitivity to ultraviolet light between 320 nm to 340 nm. The nodules increase size at puberty. are in fact angiofibromas histologically. 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.32. formation of pterygia and symblepharon.35 Xeroderma pigmentosum is relatively rare precancerous disorder of ectodermal and mesodermal structure with prominent extra ocular involvement. dilated vessels are common over the nodules. atrophy of the lid. The skin lesion undergo a chain of changes i. The lesions get calcified and are visible on x-ray. papillomas dryness of conjunctiva. ectropion of lower lid is common. It may be cerebral. which may be the cause of death. conjunctiva and cornea. development of fine vessels on skin lesions. Central nervous involvement is wide spread in the brain. The Conjunctival involvements include nodular growths near limbus. The cutaneous signs are : Erythema in all exposed parts of the skin. Both eyes are involved.DISORDER OF LID IN CHILDREN 59 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. Various types of malignanies noted are epithelioma. Central nervous involvement. basal cell carcinoma. There is no known treatment for this condition. It is an autosomal recessive disease where both the sexes are involved equally. convulsion and mental retardation. There is atrophy of skin of the lid. Ocular involvement. The disease is first noticed by second year of life when the child shows signs of intolerance to sunlight. erythema bullae formation. They may cause raised intracranial tension. Corneal involvement is early and may be first to draw attention of the parents due to . Optic nerve changes are not hemartomatous they are due to drusen that is very common with tuberous sclerosis. In fact any part of the brain and spinal cord may be involved. Intraocular structures are not involved. even malignant melanoma.

Management of xerodermapigmentosum is almost hopeless due to high mortality.60 PEDIATRIC OPHTHALMOLOGY photophobia. or may be delayed by another two to three years. better given in single dose alternate day. 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. Injection of depot steroid in the lesion is claimed to be as effective as oral treatment but less toxic. The vessels regress leaving a permanent opacity. There is no way to prevent progress of disease. Capillary haemangioma B. Capillary Haemanagioma34. 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. If the growth is large it may require treatment. They have a typical evolution. They may be present at birth. The growth by itself can cause astigmatism. use sun glasses with side protection. cover the feet by fully covered shoes. lacrimation and redness of the eyes. Malignant growths require separate. titanium oxide and para-amino benzoic acid are smeared over the exposed parts. HAEMANGIOMAS OF THE LID Haemangiomas of the lid are commonest benign tumours of the lid. Medical treatment consists of oral prednisone 2mg / kg / day.35 Capillary haemangiomas are commonest types of congenital tumours of the lid. If the growth is large it may cause mechanical ptosis producing deprivation amblyopia. anisometropia and result in amblyopia. Parents are instructed to keep the children away from direct sun-rays. As most of the tumours regress spontaneously. However in some cases reduction in dose may halt regression. so they do not require any treatment except reassurance to the parents who themselves may had such lesions in their infancy. Whenever the child is on systemic steroid its benefit should be weighed against side effects. They are of three types : A. they appear as strawberries of irregular raised mass that do not have formed capsule on histology. specialised treatment. Other form of corneal involvement is extension of conjunctival neoplasm over the cornea. The growths are more common on upper lid on the medial side than lower lid. Whole of the cornea may be involved. Only few will remain visible after five years. Corneal involvement may start as early as two years in severe cases. Corneal involvement starts as superficial vascularisation of lower part causing intense lacrimation and photophobia. Treatment is palliative aimed to give maximal relief from sensitivity to ultra violet rays. Cavernous haemangioma C. Gradually whole of the cornea may become opaque. keep long hair. Nevusflammeus A. All the parts of the body are covered by long sleeved shirts and trousers. they are seen one in every 200 live births. then the dose can be tapered. most children show significant regression within ten days. Skin protective lotion containing zinc oxide. Other modes of management .

Intra ocular lesions are seen in iris and ciliary body. However proper protective shields should be used during radiation to protect the globe and periorbital tissue. Lagophthalmos36 Inability to close the lids fully is called lagophthalmos. Lagophthalmos B. They are mostly orbital. Irradiation. Cavernous Haemangioma Cavernous haemangiomas are far less common than capillary haemangiomas in children. THE NEUROLOGICAL DISORDERS OF LID The two most common neurological disorders of lids in children are A. JUVENILE XANTHOGRANULOMA This dermatological condition of infancy and childhood is of importance not for its involvement of lid or skin but it causes spontaneous uniocular recurrent hyphaema due to involvement of iris. Neurogenic cause of lagophthalmos is paralysis of motor supply to orbicularis oculi. benign tumours that show spontaneous regression. argon NdYAG and carbon dioxide laser have been tried without uniform result. The skin lesions are multiple yellowish. Page 73-76. It may get calcified. It has a purple blue hue hence the name port wine stain. Surgery. Ortho voltage radiation of 200 rads may regress the growth in two weeks. are more common in females. Lagophthalmos can be due to 1. as pigmented single lesion or may have a salmon colour. Various types of surgical procedures are available but may not be very effective. It too has tendency for spontaneous regression. It does not require any treatment except cosmetic in fair skinned children when it is too large. various types of lasers i. single and well capsulated. in an attempt to close the lids the eye ball rolls up due to protective mechanism of Bell’s phenomenon. B. 1. Nevusflammeus This is flat cutaneous lesion of the lid that does not blench on pressure. B. Neurogenic cause or 2. It may be seen separately or in association with Sturge Weber syndrome. Neurological ptosis See Chapter 4. . They do not have prominent arterial supply which is present in capillary haemangioma. that manifest at later years of adolescence. Mechanical cause. C. Hyphaema is self-limiting but may cause secondary glaucoma. The lesion is mostly peripheral in the facial canal or its peripheral branches.e. Neurogenic.DISORDER OF LID IN CHILDREN 61 Laser.

common cause in children are head injury. photophobia. ectropion of the lower lid. Vestibular nystagmus that can not be elicited by caloric test. Initially the inner margin just fails to remain in contact with the globe later it gets everted. even when lid closure is achieved the lids gradually separate to expose the anterior part of the globe. Reflex watering is produced due to exposure of the cornea and conjunctiva that result in corneal abrasion and ulceration. (c) Widening of the interpalpebral fissure.62 PEDIATRIC OPHTHALMOLOGY Supranuclear lesion of seventh nerve produces total contralateral weakness of lower two third of the face with slight weakness of orbicularis. Palpebral causes are Large coloboma of the lids. The cause of redness of the eye in is exposure conjunctivitis and circumciliary especially in the lower lid. It does not require tarsorrhaphy. The mechanical cause may be in the orbit or in the lids. Two mechanisms are involved in watering of the eye in lagophthalmos. Leprosy is a common cause of bilateral lagophthalmos in adults. which is frequently required for peripheral seventh nerve lesion. which in turn leads to circumciliary congestion. Symptoms of lagophthalmos are watering. deafness. pain. It takes minimum 10-15 year for leprotic lagophthalmos to develop hence it is not seen in children. Mobius syndrome that consists of bilateral sixth and seventh nerve palsy. (b) Flattening of the eyebrow. If the evertion is much the tarsal conjunctiva also gets everted and becomes keratanised. Improper closure of the lid. anterior staphyloma or large ciliary staphylomas do not obstruct closure. redness of the eye. palatal or lingual palsy. Neurogenic lagophthalmos is generally unilateral but can be bilateral. early tear film break-up all in combination or alone produce epiphora. (e) Ectropion of the lower lid. Corneal sensation remains normal so long trigeminal is not involved. (d) Weakness of the orbicularis. they are mostly seen following chemical and thermal burns of the lids. 1. (f) Conjunctival congestion. Weakness of orbicularis in supra nuclear seventh nerve palsy may be so mild as to go unnoticed. cicatricial ectropion of the lids. The orbital causes are: Proptosis and exophthalmos. Buphthalmos. mostly upper lid. inability to close the eye. Facial diplegia37 is almost always central in origin. absent fingers and toes. Once there is loss of sensation the condition becomes grave. The child without corneal sensation does not com- . Mechanical causes of lagophthalmos prevent the lids to close over the globe. Signs of lagophthalmos consist of (a) Loss of horizontal folds in the fore head. syndactyly. Rosenthal Malcarson37 syndrome is a rare condition where there is bilateral infra nuclear facial palsy with furrowed tongue. 2. (g) Exposure keratitis. dry and ulcerated. 2. polydactyly.

In case of corneal ulcer. In infants patching of the eye may lead to entropion of short duration that passes off with removal of pad. Trichiasis Most of the conditions mentioned above except entropion. hypopyon tarsorrhaphy is recommended to save the cornea from perforating. Stye 3. This may lead to ulceration. perforation and blindness. Children do not develop trachomatous entropion because it takes minimum 1520 years to develop trachomatous entropion. Management Management of lagophthalmos depends on severity and duration. Blepharitis 2. ectropion and trichiasis has already been discussed. Chalazion C. Entropion 2. Trichiasis 4. Congenital ectropion is rarer than congenital entropion. Antibiotic ointments are given during sleep. Ectropion 3. Lagophthalmos of mild degree and short duration does not require much attention except to keep the cornea protected by bland lubricant or artificial tear. Bells palsy is produced secondary to edema of the facial nerve in the facial canal due to uncertain cause is treated by systemic steroids. a congenital entropion may be associated with epicanthic fold. Cicatricial entropian is seen in children following acid alkali burn or post-inflammatory conditions like Steven’s Johnson syndrome membranous conjunctivitis. DISORDERS OF LIDMARGIN IN CHILDREN Important disorders of lid margin in children consist of: A. Entropion in children is rare. Congenital 1. Post inflammatory and traumatic 1. Entropion B. Inflammatory 1. Entropion. 2.DISORDER OF LID IN CHILDREN 63 plain of pain and watering which he would have done in presence of sensation. Ectropion. Ankyloblepharon 3. Ectropion in chil- . Coloboma of the lid margin. Distichiasis 5.

Nema H. Ahmed E.V. vol. Comparative bacteriology of chronic blephariti B. and Asbury.H. Philadelphia 2000. first Indian edition.B. W. Cryo therapy in phthiriasis palpebrum Am.C.. Blepharitis in Decision making in ophthalmology. .. Jay Pee Brothers. Dougherty J.B.C. and Albert D.C. Eye lids in Anatomy of the Eye p-35-43.K. . Lacrimal diseases in Text book of Ophthalmology. Anatomy of the eyelids in A Text book of ophthalmology. Oxford University Press. . 6. and Wyber K. Oph 83: 906-907 1997. Duke Elder. Khamar B. Philadelphia 2000. edited by vanHeuven WAJ and Zwann J. p-24. and Roy F.P. McCulley J.O. Donna Brown. Banumathy S.C. Jain P. 13. Yanoff M.P. Jay Pee Brothers. 1st edition p-331.P. .K. Saunders Comp. REFERENCE 1. Natchiar.J. Mukherjee P. C. 16. Vol. I. Scheie H. . Inflammatory conditions of the lids in Modern Ophthalmology. Mishra R.J. W. Harcourt Brace 1998.B. Exanthemata : A caus of chronic dacryo cystitis . Blepharo Conjunctivitis in Current ocular therapy 5th edition p-421-422. . K. Mayuri Khamar Trivedi N. 12.G. Lids and lacrimal apparatus in General Ophthalmology 10th edition p-43-44. 2nd edition p-4-6.M. Held K.. California 1983.5. Disease of eye lids in Ophthalmology Principle and practice 1st edition p-5 Current Books International Calcutta 1995.. Disease of the orbit in Ophthalmology Principle and practice . D. .M.B Saunders Comp. Philadelphia 1977. T. Calcutta 1995.M. . p-7. . Current Books International. L. . 5. Calcutta 1993. The lids in Anatomy of the eye and its adnexa 1st edition.64 PEDIATRIC OPHTHALMOLOGY 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.T. J.. 1st edition p-9-12. 4. edited by Fraunfelder F. Kincaid Marilyn. Vaughan. and Usha Vyas . Saunders Company. 11. Anatomy of the human eye in Text Book of Ophthalmology 9th edition p-50. 8. 9. .S.C. edited by Dutta L. edited by Fraunfelder F. Pediculosis and phthiriasis in Current Ocular Therapy 5th edition p-100. II p-503-537 1st edition. 14. and Roy FH. and McCulley J. p-61-67. 7. Awan.. 10. 15. 68:524-528 1984. Buftam F. . . 2.M. Arvind Eye Hospital & Postgraduate Institute of Ophthalmology. System of ophthalmology Vol.. IV. London 1961. edited by G. Lange Medical Publication. Henry Kimpton. S. 3. Madurai. Dutta L. .T. New Delhi 1991. McInnes J. New Delhi 2000. edited by Podos S. W. Gower Medical Publication. London 1993. Dutta..

. and Asduria G.A.B. Calcutta 1993. 18. Dekker N.N. Rao V. .J. and Asdourian G. First Indian edition. Kanski J. 32. W. . Corneal Hypesthesia in Decision making in ophthalmology. T. 26.. W. Philadelphia 2000. Blackwell Scientific Publication. Inter-lesional Corticosteriod treatment of chalazia. 21. and Roy F. 27.K. Oxford 1963. and Ashton N.. Marsh R. Melanosis. 52: 1-120 1981. M.A.A.B. Edited by Gittinger J. 23. and Roy F. Viduarri. edited by Fraunfelder F. Glacucoma in Clinical Ophthalmology. W. Oculocutaneous manifestation of xeroderma pigmentosum. The Child Measles and the eyes. Rubeola in Current ocular therapy edited by Fraunfelder F. U. Saunders Comp. 17. Non-surgical therapy of chalazion Am. . 18-339-340 1986. Xeroderma Pigmentosum Cutaneous. Ahmed E.S. in 830 published cases. Cornea is measles Doc. Boston 1998.B.. p-83-82. J. 19.P. 25.DISORDER OF LID IN CHILDREN 65 in children I.W. Ophthal 78: 295-297 1994. .. . Gittenger J. Disease of lids in A Text Book of Ophthalmology 1st edition.K. Vericella and herpeszoster in Current Ocular therapy 5th Edition.M. Harcourt Brace 1998.J. . Agrawal K. 31.W. Ann. and Nilawar D. London 1989.M. Cornea and Sclera in Manual of clinical problem in ophthalmology edited by Guttinger J. Paediatric Ophthalmology in Manual of ocular diagnosis and therapy 3rd edition p-274-276 edited by Deborah Pavan Langston Little Brown. Oph 17.. . New Delhi 1997. 1st Edition p-46-47.. .H. WHO . Lee M. J.P. p-69. Kraemer K. 2nd edition p-228-230. Oph. Boger W. Phildelphia 2000. 33. Jacob. Lids and Adenexa in Manual of Clinical Problems in Ophthalmology. . Saunders Company Philadelphia 2000. p-174. Geneva 1993. Ratnakar K.H. Xeroderma Pigmentosum in current ocular therapy 5th edition p-136-137. Oph. McDermott M. Jay Pee Brothers. 1st edition p-5-7 Little Brown and Co. Boston 1998.W.. Pathology of eye lids and adnexa in Pathology the Eye and orbit.M. Little Brown and Co. 29. Scotto.S. . 27-30 1969. Freedman J.T. and Peterson R. 30. .. 20. Arch dermt 123: 241-250 1987.V. Green C. Edited by vanHeuven W.J.J. Dua H. . 1st edition p-175-187. Oxford University Press. naevi and melanomas of the Conjunctiva and lids in Ocular Pathology 1st edition p-85-101. .M. . 94: 424425 1982. Saunders Comp. Opl. W.W. Srinivasn R. Buther Worth.A. and Roy H. .H. and Zwann. Dekker N. edited by Fraun felder F.H. 24. .L. ocular and neurological abnormalities.M.. Gittenger J. 22. 28.H.F.

Disorder of the lid in Paediatric ophthalmology. Philadelphia 1983. Epicanthus in current ocular therapy. III.D.B. .F.H.V. T. .B. 38. Shaffer D. and Roy F.M. Daily R. Haik. Karcioglu Z. W. Mukherjee P.B. Saunders Company... Saunders Company Philadelphia 2002. Pechous B. 2nd edition p-412-419. New Delhi 1991. . Gordon R. 39. Edited by Fraunfelder F. W. .. Congenital Anomalies of eye and its adnexa in Anatomy of the eye and its adnexa. Abnormalities of Lid and Lacrimal apparatus in Text Ophthalmology. Jay Pee Brothers.G. . Surv. 37. Flanagan J. Oph. Jr.M. and Saunders D. Nema H. Anomalies of the eye lids in System of Ophthalmology Vol. Duke Elder S. A Case of acquired facial diplegia.H and Albert D. 36. 41. 9th edition p-287-293.. edited by Harley R.. 2nd edition p-162-165. WB Saunders Comp. 1st edition p-827-871. Henry Kimpton.C. 40.C.K. 42. London 1964. 38: 339-426 1994. edited by Scheie G.66 PEDIATRIC OPHTHALMOLOGY 34. 35. part II. Oph. and Roy H. macular oedema and lingua plicata (Melkerson-Rosenthal syndrome) Ind. W. Shannon G. Lobee C.B.. . Successful treatment of eye lid hemangioma with prednisone.A. Jobe Richard. Lagophthalmos in Current Ocular Therapy 5th edition p-436-438. . Philadelphia 1977. P.C. and Dongre R. . DeVenecia G. 21-36-39 1973. 5th edition p-430-431.M.P. .A. Archoph 94: 98-102 1970. . II.B.. Capillary Hemangioma. Edited by Fraunfelder F. Vol. Philadelphia 2000.T..A. Saunders Comp.

Ptosis and lid retractions are not vision threatening but lagophthalmos is potentially vision threatening.CHAPTER 4 Motility Disorders of the Lids Main function of the lids is to protect the globe from external trauma. It is directly antagonist of levator palpebral. 67 . The third group may be associated with narrowing or widening of IPA.3. The levator palpebral superior is the main elevator of the upper lid and the Muller’s muscle is the accessory elevator of the lid. Two of them : the levator palpebral superior and orbicularis are striated muscle supplied by third and seventh cranial nerve respectively with opposite actions. 2. That narrow the IPA. The orbicularis is the muscle that closes the lid. by an almost impermeable wall of lids. the later group consists of lid retraction and lagophthalmos. Ptosis1. 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. Associated with synkinetic movement of lids. In life a delicate balance exists between the two muscles and the eyes can be closed or opened at will and reflexly. it is called blepharoptosis or simply ptosis. 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. That widen the IPA 3. This is brought about by various reflexes that shut the eye behind.2.8 Normal upper lid covers 2 mm of the upper cornea. The third muscle— the Muller’s muscle is plane muscle supplied by cervical sympathetic. There is no well formed depressor of the lower lid however in paralysis of Muller’s muscle the lower lid is raised from its normal position upwards. The first group consists of ptosis and blepharospasm. The motility disorders of the lids are1 1. If it droops from this position.

There are many condition of the lid. Causes of pseudo ptosis can be— 1.4 Traditionally ptosis has been divided into two groups: 1. Orbit 4. blinding even life threatening. cosmetic blemish and possibility of developments of amblyopia. globe or orbit that may mimic ptosis and are grouped as pseudo ptosis. It is of utmost importance that all drooping upper lids do not constitute true ptosis. .only levator muscle is involved.68 PEDIATRIC OPHTHALMOLOGY Ptosis in children is mostly congenital in origin and less commonly acquired.2. size and position of the eyeball in relation to orbit. Congenital ptosis 2. However acquired ptosis in children have an added factor of systemic neurological involvement that may be crippling. Globe — 3. 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 Duane’s retraction syndrome — Hypotropia of contra lateral side — Hypertropia of ipsilateral side 2. 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. Acquired ptosis Other widely used classification is based on anatomical structures involved— 1. In adults the incidence of congenital to acquired ptosis is reversed. Ptosis in children have two pressing factors for management i. 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. Miscellaneous — — Classification of ptosis1. Congenital (i) Simple congenital .e.

It accounts for about sixty percent of all ptosis in childhood. There are various modes of presentation1 1. (c) Along with other congenital anomalies of lids. Birth trauma (i) To LPS (ii) Third nerve (iii) Cervical sympathetic . 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— Horner’s syndrome (iii) Myogenic— (a) Myasthenia (b) Muscular dystrophies (c) Chronic progressive ophthalmoplegia and it’s 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. 2. (d) Ptosis with synkinetic movements. Due to congenital fault in (i) Levator alone (ii) Levator Superior rectus complex (iii) Third nerve 2.MOTILITY DISORDERS OF THE LIDS 69 (ii) Complicated congenital ptosis (a) Other muscles along with LPS are involved. (b) Third nerve or sympathetic nerve involved.

It is due to lack of peripheral differentiation or aplasia of muscle. The other extra ocular muscles including orbicularis are normal. unless the lids are lifted. The cause of maldevelopment of levator is not well understood. In congenital ptosis some action of levator is always present hence most of them are called incomplete. the parents may notice its presence at birth. The appearance of bilateral ptosis is characteristic. The commonest association is involvement of superior rectus on the same side. Unknown trauma to the levator. Dystrophy or degeneration of levator.5 Congenital ptosis associated with defects in other extraocular muscles These are generally put under heading of complicated congenital ptosis. However associated error of refraction. Of course in cases of rare instances of severe ptosis. The condition may be so small that it goes unnoticed unless the child looks up or develops abnormal head posture. Boys and girls are involved equally. The ptosis is said to be complete when there is no action of the levator. In a normal child if the upper lid is everted and child looks up. Child with unilateral ptosis may raise the eyebrow on the same side only. In severe cases the pupil may be obscured with severe loss of vision. It is inherited as autosomal dominant trait. which is more often anisometropic than deprivation. unwrinkled. The vision is normal. the lid is automatically corrected but not in congenital ptosis where most of the action of levator is absent. 2. The lid droops down over the cornea.70 PEDIATRIC OPHTHALMOLOGY Ptosis due to congenital fault in levator palpebral superior only (simple ptosis) This type comprises of 75%-80% of all congenital ptosis. The forehead furrow and the head tilt are less marked in unilateral ptosis. The forehead skin is thrown in horizontal furrows. The head is tilted back. The striated muscle fibres are replaced by fibro fatty tissue. The other cause of diminished vision is amblyopia. which is mostly astigmatism and causes subnormal vision. Various suggestions given are: 1. 4. . 1. The bilateral cases are generally symmetrical. The tarsal fold is generally absent. Congenital simple ptosis is non progressive and stationary unless corrected surgically. the drooping varies in different cases. depending upon available levator function. Abnormal development of levator. moderate or severe. The inter palpebral fissure is narrow with some action of levator. Pupillary reaction and accommodation are normal. There may be many members of the family to have congenital ptosis including the siblings. It is generally present at birth but most often missed because a newborn keeps his eyes closed for eighteen to twenty hours. This is also known as simple ptosis. 3. the eyebrows are arched up. Prolonged amblyopia may result in strabismus.1 It is unilateral in seventy five percent of cases. The skin of the lid is smooth. 5. Head tilt and arching of the eyebrows are acquired late when the child realises that by doing so he has better vision. Unilateral ptosis draws attention early than bilateral. it may be mild.

3. Bell’s phenomenon may be minimally affected or absent. Other extraocular muscles have normal movement. Treatment— The definitive treatment is surgical correction of each deformity in various sittings. Some may have just epicanthus inversus and ptosis. In blepharophimosis the inter palpebral fissure is shortened in length as well as width.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. There are no synkinetic movements. equally seen in boys and girls.1 5. Rarely neonatal myasthenia may present as congenital ptosis.MOTILITY DISORDERS OF THE LIDS 71 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.e. coloboma of optic nerve head6. Presence of diplopia on lifting. 3% to 6% of children with congenital ptosis have blepharophimosis. 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. The nerve supply to the levator i. Congenital ptosis associated with under action of cervical sympathetic. Hence involvement of both the muscles is a common feature. The condition is bilateral. In telecanthus the inter canthal distance is increased. 2. punctal displacement. The epicanthus inversus is a fold of skin on the medial side of the medial canthus extending from lower lid upward.6 Blepharophimosis syndrome The tetrad is an inherited disorder. It is also not associated with changes in pupillary reaction or accommodation. It may be associated with under action of inferior oblique. Involvement of superior rectus does not produce expected diplopia. Other anomalies found are squint. the lid denotes involvement of extraocular muscles other than superior rectus alone. The surgical treatment can be deferred up to age of five years in absence of amblyopia. This is rare and mostly due to traction trauma to the brachial plexes during delivery. . Rarely there may be congenital anomaly of other cranial nerves supplying the extra ocular muscles. All the features may not be present in all the cases all the time. The child keeps the head tilted back. upper division of third nerve also supplies the superior rectus and is also likely to share common dystrophic changes. 4. There may occasionally be an elevator palsy. The medial canthal deformity is corrected six to twelve months before the ptosis surgery.

moving the jaw to contra lateral side. Jaw winking is present in four to six percent of all congenital ptosis. before that any amblyopia or anisometropia present may be corrected. . 4.72 PEDIATRIC OPHTHALMOLOGY Congenital ptosis associated with synkinetic movements of the lids The synkinetic movements can be— 1. Fibres of inferior rectus may be directed to the pupil. 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.7 Congenital ptosis associated with abnormal ocular movements1. In inverse jaw winking.10 The ipsilateral eye is generally ptotic with jaw winking. Treatment consists of surgical correction. Lifting of upper lid in attempted adduction. Jaw movement 2. Paralytic ptosis Paralytic ptosis can be due to 1. projecting the jaw forwards or opening the mouth.3.ptosis and jaw winking present simultaneously. Involvement of cervical sympathetic. the lid may elevate on closing the mouth or clinching the mouth. 2. Paradoxical retraction of upper lid on the affected side in congenital paralysis of sixth nerve.9 Fibres destined for medial rectus are directed to superior rectus or inferior oblique. Lid retraction on occlusion of contra lateral eye.e. Involvement of third nerve. The former is caused due to contraction of external pterygoid muscle and latter is due to contraction of internal pterygoid muscle. Later the parents notice that there is involuntary spasmodic jerky retraction of upper lid with certain movements of the lower jaw i. may be seen in several members of the family. Surgical treatment is definitive treatment that requires correction of two separate features . Hence many combination are possible. 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. 3. Raising of lid in downward gaze (Pseudo Graefe sign). The child may learn to suppress it later to overcome embarrassment. It is also known to become less severe as the child grows. Ocular movements 1. 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. Some of the common combinations are 1.8. The syndrome of jaw winking is due to congenital misdirection of fifth nerve to the levator.6 Jaw winking is more common than inverse jaw winking. It is mostly sporadic. 2. Ptosis with jaw movements is also known as Marcus Gunn jaw winking and inverse Marcus Gunn jaw winking.

Trunk may be involved either in cavernous sinus or orbit. However third nerve involvement in which ptosis is an outstanding feature may be the only sign to begin with. and Webers syndrome. Treatment should be— 1. The third neuron spreads from superior cervical ganglion to the eye via nasociliary and long ciliary nerves. cavernous sinus disease. this may be followed by serious central nervous involvement that may not only be vision threatening but crippling or life threatening. 3.12 Sympathetic ptosis occurs due to involvement of oculosympathetic chain anywhere from hypothalmus to eye causing in a number of signs that constitute Horner’s syndrome. Surgical intervention is deferred for about six months after ptosis has been stabilised. diabetes. Due to its proximity to fourth nerve nucleus.MOTILITY DISORDERS OF THE LIDS 73 1. The first neuron extends from posterior hypothalmus to cilio spinal centre of Budge between C8 and T2. vascular lesions and neoplasm are more common than infection. Amblyopia is treated by standard method. Supra nuclear lesions cause mild ptosis with subnormal gaze palsy and miosis. 2. enlarged lymph nodes in neck. Benedicts syndrome. In adults. It consists of bilateral. It can be congenital or acquired. It can either be congenital or acquired. Sympathetic ptosis (Horner’s syndrome)11. May be isolated. .13 1. inflammation in orbit can cause Horner’s syndrome.e. The acquired causes in children are birth trauma mostly to the brachial plexus associated with Klumke’s paralysis. fourth nerve involvement is also common. 3. Ptosis due to oculomotor palsy is far more common in adults than in children. Directed towards the cause. The second neuron extends from centre of Budge to superior cervical ganglion in the neck. Oculomotor ptosis can be due to— Nuclear. In children infection is more frequent cause of paralytic ptosis than others. Incidence of Horner’s syndrome is less common in children than in adults. Mid brain lesions are associated with signs of dorsal or ventral fascicular lesion i. may precede palsies of other muscles supplied by third nerve. For diagnostic purposes the oculo sympathetic is divided into three anatomic part consisting of three neuron. The nuclear lesions are rare. basilar and peripheral involvement. Besides birth trauma accidental injury to neck. Some authors believe that congenital Horner’s syndrome is nothing but a result of birth trauma. 2. Ptosis in third nerve involvement. Congenital ptosis due to third nerve palsy is less common than congenital ptosis due to levator dysplasia. facicular. symmetric ptosis with superior rectus under-action.

The pupil does not dilate with 4% cocaine. Narrowing of inter palpebral aperture: This is due to combined effect of ptosis and upside ptosis. 7. Increased accommodation. Dilated facial vessels. 2.Congenital miosis. healed iridocyclitis. Transient features The permanent features are: 1. 6. The eye looks small due to ptosis and upside ptosis. Treatment There is no known treatment for Horner’s syndrome. 5. Ptosis PEDIATRIC OPHTHALMOLOGY (i) Ptosis is mild. Reduced intraocular pressure. (ii) The lid folds are retained. 4. It is best observed in dim light. 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. Attention should be directed to the cause of Horner’s syndrome. not more than 2 mm to 3 mm due to paralysis of Muller’s muscle. (iv) The action of LPS and superior rectus are normal. The iris on affected side is lighter.74 The features of Horner’s syndrome consists of— 1. 3. 3. which are confirmed by 1. There is no change in convergence and light pupillary reaction. Heterochromia of the iris on the affected side.13 The transient signs 1. Enophthalmos: This is more of a pseudo enophthalmos than true. Permanent signs and 2. Differential diagnosis consists of all causes of unilateral small pupil .5 mm to 1. The pupil will dilate with hydroxyamphetamine 1% (Paredrine) in pre ganglionic lesion but not in post ganglionic lesion. 2. (iii) There is no lid lag on upward gaze.00 mm and not pin point. Pharmacological test (i) Cocaine test (ii) Paredrin test . 4. This is characteristic of congenital Horner’s syndrome in children. traumatic miosis. Dilatation of conjunctival vessels. Anhydrosis of the affected side of the face. The normal lower lid that just touches the lower limbus covers the lower 1 mm of the cornea. Miosis: The pupil on the affected side is smaller by 0.

2. Diplopia. It is not a hereditary disease but may be seen in members of the same family. However childhood myasthenia is more common in girls i. It can affect any age from birth to late seventh decade but common age group is between 20-40 years.MOTILITY DISORDERS OF THE LIDS 75 2. Myasthenia is more common in women than in men in a ratio of 2:1. 1. Myasthenia is a common cause of acquired ptosis and diplopia in all ages without other neurological involvement. It is generally minimal or absent on waking up and gradually increases during active hours becoming maximum in the evening. girls to boys ratio is 6:1. It is of two types: 1. Fault at myoneural junction. Drooping changes not only in days but may be in hours.e. Ocular symptoms of myasthenia gravis Two main symptoms are 1. Systemic About 90% of patients with myasthenia have predominantly ocular involvement that may precede systemic involvement in 75% of cases. . About six percent of cases may develop dysthroid oculopathy later. The other eye follows soon. This unknown substance19 has curare like action that renders the muscle hypoactive and fatigued. 80% of cases with ocular involvement will be gradually converted into systemic involvement in two years14. Ptosis: Drooping of upper lid in myasthenia is variable though it is bilateral.16.17 Myasthenia can be progressive or remittent. Acetyl choline. Ptosis and 2. There may be concurrent or one may follow one another. Both are more common in adult but can be seen in children and may be congenital or neonatal. In bilateral ptosis drooping is systemic. x-ray chest 3. Fault in myoneural junction Myasthenia gravis15. which is a pharmacological transmitter at neuro muscular junction is destroyed fast by an unknown substance present at the neuro muscular junction. It is presumed to be an autoimmune disorder. CT and MRI 4. which may be present at birth. Ocular and 2.17: Myasthenia gravis is an auto immune disorder that produces extra ocular muscle hypofunction without involving intrinsic muscle. It may shift from one eye to the other eye. Neurological evaluation Neuro muscular (Myogenic) ptosis There are two types of myogenic ptosis: 1.21 Use of anticholine estrase drugs rectify this process. There is reduction of acetylcholine receptors at motor end plate20.15 Chances of conversion to systemic myasthenia after two years is less common. may begin in one eye. Exact etiology of myasthenia is not known.3 Infants born to myasthenic mothers are prone to develop neonatal myasthenia. this forms the basis of medial treatment of myasthenia. Myopathies and muscle dystrophies.

The conditions that constitute differential diagnosis are: — Chronic progressive external ophthalmoplegia and its variations. Transient neonatal myasthenia seen in children born to mothers who have the disease. Improvement of ptosis following Tensilon test. Under action of orbicularis is almost constant feature in myasthenic ptosis. Diplopia Diplopia is one of the most common and sometimes first symptom experienced by the patient. Extraocular muscle involvement does not follow any fixed pattern. most probably due to involvement of bulbar muscles. diplopia with orbicularis weakness and normal pupil should be suspected to have myasthenia unless proved otherwise. This sends the upper lid higher than normal. The presentation is similar to adult myasthenia. Squint Some patient may notice squint. 4. 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. Myasthenia of childhood differs from adult myasthenia. On removal of the ice. Muscle imbalance may simulate inter nuclear ophthalmoplegia or central gaze palsy. the lid is markedly elevated which falls to ptotic level in 15 seconds. laryngeal muscle. It is more often vertical than horizontal. 3. Muller’s muscle is never affected. Persistent neonatal myasthenia: This is similar to transient myasthenia but mothers do not have the disease. Cold test: An ice cube is applied to the ptotic eye for about two minutes. 5. 2. 6. There are three types of myasthenia in children 1. Patient may present with heterophoria. . Common are the small muscles of face. Differential diagnosis All cases of variable ptosis. Any of the skeletal muscles can be affected. the ptosis becomes more. It becomes worse in the evening.17 Involvement of thymus is common. Lid fatigue: With sustained upward gaze. Therapeutic trial by oral prostigmine and pyridostigmine shows improvement in ptosis and reduction of diplopia.76 Variability is demonstrated by following tests: PEDIATRIC OPHTHALMOLOGY 1. 2. muscles of mastification. Commonest being exophoria. Juvenile myasthenia sets in after ten years of age. 7. only to fall to ptotic position.16 There may be nystagmoid movements. Extra ocular muscles to be involved other than levator is orbicularis. If the contra lateral lid is lifted mechanically there is enhanced ptosis in ipsilateral side. It varies in type and extent. 3.

Its effect starts with in 10-30 minutes following oral administration and lasts for three to four hours with peak in between. and observe for any adverse reaction or improvement. Correct dose is adjusted empirically in consultation with pediatrician. Surgical correction should not be done unless there is no recurrence for one year. The response in 2 and 3 are not seen in true myasthenia. 2.V. II. The side effects of the drug are colic. CT and MRI of mediastinum may show thymoma. In adverse reaction. Management Myasthenia is a self limiting disease. Inject 2 mg I. 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.MOTILITY DISORDERS OF THE LIDS 77 — 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. An improvement. abandon the test and consider therapeutic trial with oral prostigmine. (adjust dose in children). diarrhoea. x-ray chest. III. Worsening: Small tropia becomes large tropia. facial expression and diplopia. 4. Antibody to anti actycholine is present in 90% cases. Fill a syringe with 10 mg Tensilon. Medical treatment Medical treatment consists of oral a anticholineesterase drugs given in divided doses. 3. ptosis and squint are stable for at least one year. Inject 0. the test is conclusive that may be further confirmed by injecting remaining 8 mg.23 Three types of responses are possible: 1. Most of children in this group will have some residual ptosis and or manifest muscle imbalance that requires surgical correction later. 2. To do this test first precaution is to have a resuscitation trolley ready and handy. Reversal: Squint shifts to the other eye.V. Most commonly used drug is pyridostigmine. 3. In case of improvement in ptosis. Pharmacological test—Tensilon test.3 mg atropine I.22 1. Hence the drug has to be repeated every four to six hours. 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. It takes two to three days for prostigmine to be effective. Other drug used is prostigmine which is less effective than pyridostigmine.

In case of acquired ptosis attention should be diverted to find out the primary cause and its management. The internal ocular muscles are spared. It is associated with mutation in mitochondrial DNA. As diplopia is fleeting and changes direction too often many a times prisms may be of little use. It takes few months to be effective. Cyclosporine and cyclophosphamide are other immunosuppressive drugs used in selected cases. The first sign that develops after ten years of age is gradually developing ptosis. Evaluation of a case of ptosis for surgery Definite treatment of congenital ptosis is surgery. Other drug used in patient is refractory to anticholine esterase drugs is azathioprine. In case of acquired ptosis surgical intervention should be undertaken only when the ptosis has been stable for at least one year. Ptosis crutches have limited value. The ptosis is bilateral and symmetrical. The child throws the head back to get clearer vision and forehead shows prominent frontal creases. oral steroid are started. So long only extraocular muscles are involved.24A In fifty percent of cases the disease is hereditary. It is mandatory to evaluate each patient separately. It begins as single muscle involvement resulting in diplopia. Each disorder has to be treated individually. the condition is considered as ocular myopathy. Thymectomy have been claimed to give good result in refractory cases in selected few. initially a single daily dose is given till the desired effect has been reached.78 PEDIATRIC OPHTHALMOLOGY If the patient fails to show desired therapeutic dose. Common heads under which a ptotic child is evaluated are: 1. 2. . Prisms may be given to minimize diplopia.17 Ancillary ocular methods 1. Involvement of muscles is ill defined. As more muscles get involved the eye becomes almost immobile and diplopia disappears. The eye may be patched to give same effect. which progresses to become complete. There is no known specific treatment. It is a bilateral disease of slow progress. History. The child learns by experience to close one eye to avoid diplopia. Sometimes each eye may require individual evaluation. Greatest drawback with the drug is its hemato and hepato toxicity. However levator resection gives less correction in congenital ptosis. then the schedule is changed to every alternate day and gradually reduced. 3. Generally it manifests between second and fourth decade. The condition may be associated with retinal pigment degeneration and heart block. Chronic progressive external ophthalmoplegia25 The condition may be seen in children as well as adults. Ophthalmoplegia develops years after ptosis. Congenital ptosis requires more correction than acquired. Find out if ptosis is congenital or acquired. If other muscles get involved it is called ophthalmoplegia plus. Occasionally there may be involvement of pharyngeal muscle.

Presence of Bell’s phenomenon. this is between 12 mm-15 mm. In absence of Bell’s phenomenon. (ii) Presence of epicanthic fold and blepharophimosis which may require separate surgeries. which may be considered worse than cosmetic ptosis by the patient. (iii) Absence of orbicularis action goes more in favour of myasthenia.e. In normal eye. 2. If excrusion is more than 8 mm. This unmasks presence of pseudo ptosis. (iv) Presence of synkinetic movements require multiple surgeries. 3. Bell’s phenomenon is one of the most important defence mechanisms of the eye. the lid will droop down. mild. (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. 5. Examination of the lids (i) Exclude pseudo ptosis from true ptosis. if the upper lid is raised the cornea becomes unprotected and exposed to external injuries. the condition is designated as 2 mm ptosis and the ptosis is considered to be mild ptosis. 4 mm from upper limbus leaving on 7 mm of uncovered cornea. In unilateral ptosis the other eye acts as control. If the upper lid droops by 2 mm from usual position i. In presence of ocular palsy. 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. This leaves 9 mm of cornea not covered by lid.e. 4. More is the excrusion better is the action of LPS. It is lost in paralysis of the superior rectus. correction of ptosis will result in intractable diplopia.MOTILITY DISORDERS OF THE LIDS 79 2. Exclude vertical tropia by cover test. Examination of the eye 1. Absence of Bell’s phenomenon is an absolute contra indication for any type of ptosis surgery. The average vertical diameter of cornea is 11 mm. however some of the pseudo ptosis may require surgical intervention. which means that the levator if strengthened surgically will eliminate ptosis. . it is called good excrusion while fair excrusion means5-7 mm and less than 4 mm is poor. (ii) If the action of LPS is poor. The choice of type of surgery to be undertaken partly depends upon degree of ptosis i. The function of levator is assessed by noting the excursion of upper lid from downward gaze to maximum up gaze. In normal eye the upper lid covers upper 2 mm of the cornea in primary gaze. moderate or severe and amount of levator function available. Similarly ptosis of 3 mm is called moderate and more than 4 mm is called severe ptosis. Vision—Visual acquity is evaluated to diagnosis presence of amblyopia that requires energetic anti amblyopic treatment. Action of extraocular muscles—Under action of extraocular muscles result in diplopia that is generally not felt if the upper lid covers the pupil.

Bilateral lid retraction is generally symmetric. Sensation of cornea—Absence of corneal sensation is absolute contra indication for ptosis surgery. 3. Sympathetic lid retraction presents with feature opposite of sympathetic ptosis. It consists of widening of IPA. In unilateral cases the condition may remain unilateral or the other eye may be involved later. . Tear film status—Poor tear film status becomes worse following ptosis surgery. dilated pupil. Children: Children suffering from progressive loss of vision have upper lid retraction as an effort to see better. Commonest cause of lid retraction is dysthyroid myopathy due to sympathetic over action of Muller’s muscle. Prolonged use of steroids are also known to cause lid retraction. sweating on the affected side and lowered temperature. Visible bare sclera above and below is seen in exophthalmos and proptosis. Prostigmine and tensilon when given as injection cause improvement of ptosis and sometimes the lid may be retracted. The effect passes off with in hours. 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. Other causes of lid retraction are sympathetic over action and dorsal mid brain lesion.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. Pathological lid retraction Lid retraction can either be unilateral or bilateral. Pharmacological causes of lid retraction 1. Lid retraction is dysthyroid myopathy is generally bilateral and symmetrical. Lid retraction25. enhanced lid crease. 7.80 PEDIATRIC OPHTHALMOLOGY 6. This is generally associated with lid lag. 4. 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. Exposure of a strip of sclera below the lower limbus may be seen in case of sympathetic over activity along with upper lid retraction. 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. mild exophthalmos. Infants born to mothers with hyperthyrodism show retraction for two to three weeks. Lid retraction can be seen in some physiological states also specially in infants and children.26 In unilateral ptosis the contra lateral lid may show retraction when the ipsilateral lid is forced to elevate. This is enhanced by instillation of sympathomimetic drugs and reduced by instillation of sympatholytic drugs. 2. 2. 1.

There may be associated retraction nystagmus. Other causes of bilateral lagophthalmos (facial diplegia) are— Causes of bilateral lagophthalmos have to be central. burn. — Extreme degree of symblepharon. Lagophthalmos is generally unilateral due to peripheral involvement of seventh nerve. — 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. A strip of the IPA remains open during sleep. Nocturnal lagophthalmos is a mild form of bilateral lagophthalmos. the lesion may be anywhere from its nucleus to extreme periphery in the orbit. Treatment of lid retraction consists of recession of LPS along with Muller’s muscle. dissociation of light near reflex. Children do not suffer from lagophthalmos due to leprosy. trauma accidental or surgical. Lids may be caught in scar of the lid following infection. there is conjugate up gaze paralysis of convergence. Hence it is but natural that affection of facial nerve will lead to lagophthalmos. These are— — Large coloboma of the upper lid. which becomes obvious when the child sleeps.29 — Brain stem trauma30 — Brain stem glioma30 . Bell’s palsy or trauma.MOTILITY DISORDERS OF THE LIDS 81 Dorsal mid brain lesion is called Collier’s sign that consists of bilateral lid retraction. Bilateral exophthalmos is mostly central in origin. On examination there is mild weakness of orbicularis. Bell’s phenomenon is intact. Besides neurological causes there are other causes that are generally put under mechanical causes that prevent the lids to close. — Scar on the skin surface of the lids. i. They are— — Mobius syndrome30 — Melkerrson Rosenthal syndrome28. Lagophthalmos In this condition there is either difficulty in closing the eye or there is complete absence of lid closure.e. Closure of lids is brought about by the orbicularis muscle that is supplied by facial nerve. An exception being leprosy which is the foremost cause of bilateral lagophthalmos in adults due to peripheral involvement. Over correction of ptosis is a common cause of unwanted lid retraction.

The signs of Bell’s palsy: 1. Such cases should be investigated for causes other than viral infection. however there may be some cases where there is not complete recovery. there is paralytic ectropion of lower lid with watering from the eye. the eyeball roles up due to intact Bell’s phenomenon. The lesion causes swelling of the nerve in a narrow canal. — A lesion at the pons involves sixth nerve nucleus causing ipsilateral lateral rectus palsy. Lagophthalmos . The symptoms consist of inability to close the eye on the affected side and watering. Bell’s palsy is an acute unilateral facial palsy involving the facial nerve in the facial canal. Common causes for such lesions are fracture base of the skull and basal meningitis. It is known to recur in 3% to 10% cases rarely on the same side. loss of conjugate gaze toward the same side and hemiplegia of the contra lateral side i.e. However lower part of the cornea remains exposed in spite of intact Bell’s phenomenon and develops exposure keratitis that may be converted to ulceration on long run. This may be preceded by pain in ear and over the mastoid with hyperacusis. 2. 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. Geniculate ganglion lesion causes Ramsay Hunt syndrome. Foville’s syndrome. Commonest type of facial palsy in all ages is Bell’s palsy. loss of taste in anterior two third of the tongue and diminished tearing. 4. Commonsest cause is viral infection though auto immune causes can not be excluded. 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.The inter palpebral fissure is wide. The patient is unable to close the eye. A basal lesion involving roots of seventh nerve also affects eighth nerve causing loss of hearing.82 — Lyme disease31 — Guillain Barre syndrome — Closed head injury — Myasthenia Neurological causes of unilateral lagophthalmos— PEDIATRIC OPHTHALMOLOGY — Supra nuclear and nuclear lesions does not involve orbicularis in a peripheral lesion. Cerebropontine angle lesions causes ipsilateral facial weakness and hyperacusia. The condition is self limiting clearing in two to three weeks without any residual effect. . In an attempt to close the eye. 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. 3.

London.D.H. 2000. nerves from fifth to twelfth are known to be affected.N. : Ptosis in Current ocular therapy. Vol. III. London.MOTILITY DISORDERS OF THE LIDS 83 5. Management of anatomical defects 3. Lingua plicata is most probably congenital in origin. p-447-449. Conjunctivitis and keratitis are treated with frequent antobiotic drops and lubricants by day and antibiotic ointment by night. Jay Pee Brothers. Kahn N. Corneal sensation and other cranial nerve are normal. Second edition.D. P. 3. Management of lagophthalmos The management depends upon the cause of lagophthalmos. keratonisation of conjunctiva may develop. However associated conjunctivitis. Philadelphia.. Seventeenth edition.T. 497-502. 2. There is inability to abduct the eyes. 807-814.H. Edited by Harley R. . p-316. and Roy F. p. 1984..J.29 consist of peripheral facial diplegia. p. Edited be Saxena S. : Congenital anomalies in mobility of lids in System of ophthalmology. Martyn : Abnormalities of lid function in Pediatric ophthalmology.. Churchill Livingstone. The child has unilateral or bilateral esotropia. In few days microbial conjunctivitis develops. 1986. Melkerrson Rosenthal syndrome28. Vemuganti. Bell’s palsy does not require any specific treatment as it is self limiting. Duke Elder S. Henry Kimpton. Naik M. WB Saunders Company. Mobius syndrome—This is a multiple cranial nerve palsy of congenital origin. Edited by Fraunfelder F.. Miller S. 2. Management of central causes. Philadelphia.G. Lois J. Management of Bell’s palsy 2. It can be divided into: 1. Commonest combination is sixth nerve with facial diplegia. puffiness of face and lingua plicata. 2003. Vol. 1964. If Bell’s palsy does not subside.. 4. part 2. keratitis and lagophthalmos requires attention. 5. WB Saunders Company. Fifth edition. 887-905. The central causes require more elaborate investigation like CT and MRI and treatment is directed towards primary cause. New Delhi. Anatomical defects like scars and coloboma are treated by appropriate oculoplastic surgery. Honavar S. : Congenital ptosis in Parson’s diseases of the eye. 6. It manifests in childhood. Chandrashekhar G. If lagophthalmos does not improve with in a fortnight temporary lateral tarsorrhaphy may be required. First edition. Geeta K. : Ptosis in clinical practice in ophthalmology. REFERENCES 1.

Edited by Fraunfelder FT and Roy F.J. p-881-882. and Roy F. p-227-228. and Kline L..J. Harcort Brace. Philadelphia.. 20.H.C. Second edition. Berry S.R. 1989. WB Saunders Company.. II. 13. 21. 1989. Kanski J. Henry Kimpton.B. and Kline L. 1989. Third edition. Edited by Fraunfelder F. p-477478.. 1983. : Horner’s syndrome in Clinical ophthalmology.M. and Nancy J. 1998. p-221-222.J. 23. : Blepharophimosis in Current ocular therapy.K. Third edition. 1981. London. Fiona Rowe : Ptosis and pupils in Clinical Orthoptics. 40.L. p-898-905. 2000.84 PEDIATRIC OPHTHALMOLOGY 6. and Kline L. : Myasthenia gravis in Current ocular therapy. 1982. Butterworth International. Vol. and Dutta N. : Myasthenia in Current ocular therapy. 1964. Edited by Fraunfelder F. Harley R. : Myasthenia gravis in Clinical Ophthalmology. Martyn : Myasthenia Gravis in Pediatric ophthalmology.T. Third edition. 2000. Jain. 15. 11. 1956. Jay Pee Brothers. 1989.. First edition. Blackwell Science. Seth : Prakash BJO. London. WB Saunders Company. New Delhi. 22. 12. Bajan Das F.B. and Roy F. Kanski J. 1997. 10. London. p-905-907.J. Fifth edition. New Delhi. p-164-171. Newman : Chronic progressive external ophthalmoplegia in Current ocular therapy. : Ptosis. : Myasthenia gravis in Modern Ophthalmology. WB Saunders Company.. Second edition.T. Vol. 1989. WB Saunders and Company. p-221-222. Kanski J. London. Third edition. St. London.R. Roy F. Second edition. WB Saunders Company. Beard C. Lois J. : Myasthenia gravis in The eye in systemic disease. Bajan Das F. : Oculo sympathetic defect in Neurophthalmology.. Miller N. Fiona Rowe : Myasthenia gravis in Clinical Orthoptics. : The Tensilon test in Neuro-ophthalmology. : Myasthenia and ocular myopathies in Neurophthalmology. 9. First edition. Kerrison J. Fifth edition.B. p-268-273.J. : Drooping of upper lid in Decision making in ophthalmology. p-422-423. 18. 1997. p-5658.B. : Myasthenia and ocular myopathies in Neurophthalmology. Oxford. Glasser J. Second Vol. Philadelphia. Dutta L. Jay Pee Brothers. : Paradoxical movements of the lids in System of Ophthalmology. Philadelphia. Second edition. Glasser J. Harper and Row. Blackwell Science Ltd. p-177.T. 24A. Harper and Row.D. Duke Elders S.H.. 7. Second edition. Oxford. 2000. p-141-145. p-474475. CV Mosby. Louis. Butter Worth Heinemann. Fifth edition. p-113-115. p-68. New Delhi.B. 14. Philadelphia.. Fifth edition. : Horner’s syndrome in Neurophthalmology..S.J. Part 2. p-588. Ed. Butterworth.S. JayPee Brothers. 1982. . New Delhi. Slonim C. III. Edited by Heuven WAJ and Zwaan J. 2000. First Indian edition. Bajan Das F. Miller N. 19. p-121-123. Philadelphia.. Carter John E. Jay Pee Brothers.H. 17.H. Piest K. 24. Edited by Fraunfelder F. p-208-210. p-177-179. 8. 2000. 16.T.

Jr. London.. 1987. : Melkerrson Rosenthal Syndrome in System of ophthalmology.K. XIII. and Roy F. Edited by Duke Elder S. Hedges T. 2000. WB Saunders Company. and Roy F. Edited by Peyman G. p-205-206. . First Indian edition. Fifth edition. p-1965-1967. Edited by Fraunfelder F. III..T.H. 1974. London. Bajandas F..J..T. and Dongre R.MOTILITY DISORDERS OF THE LIDS 85 25. Henry Kimpton. 1982. macular edema and lingua plicata. Jay Pee Brothers. 2000.A. : Bells palsy in Current ocular therapy. Third edition.H. Vol.S. 30. and Macfaul P. : Facial diplegia in Neurophthalmology.A. p-153. Jay Pee Brothers. 1989. Oph.C. 31.B.R. New Delhi. : A case of acquired facial diplegia. Rosenberg M. Glaser J. Ind. 29. : Ocular myopathy in Principle and practice of ophthalmology. : Lid refraction in Neurophthalmology.R. Harper and Row. Meyer D.A. New Delhi. Mukherjee P. Sanders D. WB Saunders Company.. Philadelphia. : Lid retraction in Current ocular therapy. Kline L.F. Fifth edition.. and Hedge5 T. Edited by Fraunfelder F. 27. 21 : 36-39. p-439-440. Duke Elder S. 28.R. 26. p-36-37. Philadelphia.R. p-21. Vol. and Goldberg M.

The lacrimal gland is divided 86 . D.2.4 Lacrimal apparatus consists of three parts : A. The drainage system consists of 1.1.3. 4. Tear spreading system consists of 1.2.4 E. lids 2. Lacrimal gland proper and 2. across the conjunctive and cornea to drain out of the eye and C. 5.lacrimal duct (a) The lacrimal glands The lacrimal glands are the main source of tear. one for each eye.CHAPTER 5 Disorders of Lacrimal System in Children LACRIMAL APPARATUS1. The lacrimal gland proper is situated in the fossa for lacrimal gland on the superiotemporal aspect of the orbit behind the septum orbitale. Tear spreading systems : that transports tear from gland. The lacrimal glands are divided into two groups: 1. Naso. Canaliculus one in each lid 3. The drainage system. Common canaliculus one for each eye. F. That produces tears. lacrimal gland proper. 2.3. 2. conjunctiva. Tear forming parts consist of 1. Lacrimal sac. Accessory glands. Accessory Lacrimal glands. 1. B. Puncta one in each lid.

2. the lacrimal gland. the periostium lies between the gland and the orbital bone. chronic inflammation i. The medial end reaches. (f) Infra orbital glands. In the posterior part the two lobes are indistinguishable. acute infection. The para sympathetic fibres originate in the lacrimal nucleus in the pons and reach the lacrimal gland in the lacrimal nerve2. Histologically. The Accessory lacrimal glands. by mumps. 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. There are about 1012 lacrimal ducts that originate in the orbital part and travel through the palpebral part. The sympathetic fibres reach the lacrimal gland via sheath of carotid through ophthalmic artery. These glands are situated in the conjunctiva. Superiorily. 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. Blood Supply The lacrimal gland is supplied by lacrimal artery and some times by infraorbital part of the maxillary artery. Hence. The venous drainage is through the lacrimal vein that travels back in the superior ophthalmic vein4. Mikulicz’s syndrome and growth like adenoma. As the orbital part lies behind the orbital septum. The lacrimal ducts from both the lobes open in the conjuctival sac 4 to 5mm above the tarsal plate. Role of sympathetic nerve in tear production is uncertain.e. They are : (a) Goblet cells of conjunctiva. The lymphatics drain in preauricular and submandibular glands. They are very small and contribute small amount of fluid mostly mucin to the tear film. the inferior surface has lateral part of the levator with its extension and lateral rectus under it. afferent and efferent. it has a superior and an interior surface. The main lacrimal gland . removal of palpebral part alone will produce the effect of total removal as far as tears are concerned. an anterior and a posterior border and two ends.e.e. it is an exocrine tuboalveolar gland that resembles the salivary gland and share similar pathological process i.e. measles. the levator muscle and the lateral end reaches the lateral rectus. the lateral and medial. The tear production mostly controlled by parasympathetic4a.DISORDERS OF LACRIMAL SYSTEM IN CHILDREN 87 into two unequal parts i. The former is via lacrimal nerve. (e) Glands in the plica and caruncle. trigeminal.4a 4a. The nerve supply to the lacrimal gland is divided broadly in to two parts i. The efferent consist of both sympathetic and parasympathetic nerves. The division is limited to the front part of the gland. it is normally neither visible nor palpable. (b) Krause’s glands in the fornices (c) Glands of Wolfring near the tarsal plate. (d) Glands of Manz in the bulbar conjunctiva. The posterior border rests on the orbital fat. The palpebral part is about one third of the orbital part2. This part is sometimes visible when the lid is everted and the patient looks down and it may be mistaken as a growth. It lies just above the lateral part of the upper fornix. There are six to eight lacrimal ducts that originate in the lower lobe. which is a branch of.

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

Anteriorly. behind the sac lies the lacrimal part of the orbicular is and posterior limb of medial canthal ligament.DISORDERS OF LACRIMAL SYSTEM IN CHILDREN 89 part of the orbital rim. The fossa has two ridges called lacrimal crests. This is an important landmark. the sac swellings are more marked under the medial canthal ligament. The ethmoidal cells and medial meatus form the medial relation of the sac. The incision should be with in 3mm of the medial canthus. There is no strict anatomical landmark to differentiate the three parts. The angular vein runs vertically 8mm medial to the medial canthus. The part that joins the nasolacrimal duct is called the neck. The directions of the naso lacrimal duct be taken into consideration while probing in children least a false passage is formed. Between the lacrimal fascia and the sac is a plexus of veins. a tributary to this descends with in 3mm of the sac. The nasolacrimal canal is formed by maxilla. in praorbital artery angular artery and spheno palatine artery. back wards and laterally.5mm) part and a smaller intra mural part. one formed by maxilla is known as anterior lacrimal crest the other is called posterior lacrimal crest. the anterior limb of the medial palpebral ligament crosses the sac mid way between the fundus and neck above the larimial fascia 4a. 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. An oval swelling above the medial canthal ligament is more likely to be unrelated to the lacrimal sac. The lacrimal passage gets its blood supply from superior and inferior branches of the ophthal micartery. goblet cells and some columnar cells resembling respiratory columnar cells line the inner wall of the sac. The duct has a larger in introseous (12. by 15mm it sends two columns in to the lid to form the canaliculi. The lacrimal sac develops as a solid cord of ectoderm at 10mm stage. The intramural part open under inferior turbinate that is 30-40mm form the anterior nares in adults and 20mm in children. They are ectodermal in origin embedded in mesoderm. The NLD is surrounded by rich vascular plexus that is similar to seen in interior turbinate. The incision to expose the sac should be concentric with the fibres of the orbicularis. The upper dome shaped part of the sac is called its fundus while most of the part is called body. on the lateral side is the caruncle and palpebral part of the orbicularis. 5. The resiratory type of columnar cells are more in NLD than in sac. lacrimal bone and inferior nasal concha. and nasal veins. The naso lacrimal duct is down ward extension of the lacrimal sac in a bonny canal. It’s about 18mm in length. The lower canaliculius separates a part of the lid to form the caruncle. The medial palpebral ligament is felt as a horizontal band medial to the medial canthus when the lateral canthus is pulled laterally. The frontal process of maxilla and lacrimal crest forms the fossa. It extends from the sac to the interior turbinate of the nose. The lacrimal sac and naso lacrimal duct develop beneath a furrow formed by the lateral nasal process and maxillary process. The sac is surrounded by the lacrimal fascia that is formed by splitting of the periorbita. It is directed down words. The lymphalies drain in submandibular and deep cervical glands. At 15mm stage the solid cord descend down to form the nasolacrimal duct that is met by similar structure . For ideal exposure the incision should begin 3mm above the medial palpabral ligaments. while exposing the sac. Naso lacrimalduct. Development of the lacrimal drainage system The lacrimal drainage passages have a common origin. Non-kertanised squamous cells. The venous drainage is via angular intra orbital. As the sac has less space to distend at the fundus.

Common congenital anomalies are9 A. primary buththalmos. Congenital fistula of the sac. By birth the duct should be fully canalised. 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. Diagnosis is straightforward an infant develops epiphora after 3 weeks with or without discharge and positive regurgitation. Atrasia of the inferior osteum by a membrane is very common cause of obstruction. A. there may be few insignificant blocks that get cleared by first month post partum. Generally there is single obstruction at the lower end. after birth because the lacrimal gland does not start secreting adequality before third week. At this stage pressure over the sac may result in positive regurgitation. Diagnosis. CONGENITAL ANOMALIES IS LACRIMAL DRAINAGE SYSTEM All the structures from lacrimal puncta to opening of nasolacrimal duct in the inferior meatus may be effected by developmental anomalies singly or in combination. 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 effect of nasolacrimal duct block is not visible until three to four weeks. . The duct gradually gets canalised from both the ends but predominantly from upper end. It is found in about 6% of new born8. 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. The canaliculi and puncta become patent after the two lids have separated. 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. In few months the sac gets distended and there is copious reflux following pressure over the sac. However there may be more than one block. After few days the conjunctiva gets inflamed and there is mucopurulent discharge. in about 10% of infants. conditions that may be mistaken as congenital nasolacrimal duct obstruction (congenital dacryocystitis. A typical presentation is watery discharge in a congested eye with out photophobia. or neonatal dacryocystitis) are ophthalmic neonatorum. The solid cord starts canalisation from both the ocular and nasal ends5 in segments. D. Atresia of puncta and canaliculi. The blockage is caused by epithelial debries. Congenital block of nasolacrimal duct B. Congenital mucocele of the sac C. Congenital coloboma of the lower lid is a common cause of ipiphora with negative regurgitation test.90 PEDIATRIC OPHTHALMOLOGY arising from the primitive nasal cord7. retino blastoma. Rest of the eye is not involved in congenital nasolacrimal duct block but becomes prone for recurrent infection. corneal abrasion. However in about six percent of cases the canalisation is not complete.

and fistula of the sac. ofcourse the tension is high. If repeated probing fails than the condition should be treated either by conventional dacryo cysto rhinostomy. mild corneal abration in presence of infected sac may result in to disastrous keratitis and corneal ulceration. removing the discharge. Sooner the massage is started better is the result generally 95% of the cases will be cured by massage and local antibiotic. However sometimes repeated probing may be required. This give relief in 95-98% of cases. Success of this method depends upon proper compliance. A chronically infected sac may lead to acute dacryocystites. However this process may not be helpful (1) It started after three weeks of on set of eiphora. The sac is pressed with the pulp of the index finger. For a successful result the probe should be directed downwards. In that case probing of the nasolacrimal duct under general anaesthesia through upper puncta is indicated. 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. Corneal injuries are generally unilateral without mucopurulent discharge and stain with fluoreseein. The mother is taught this simple method by repeated demonstration and is asked to perform the same in presence of the doctor. congenital glaucoma is generally associated with photophobia and the cornea is large. laterally and backwards10. The process of cleaning the hands pressing the sac. Complication of Naso Lacrimal Duct Obstruction Commonest organism that is isolated from infected sac is pneumococcus which is a potential danger to the cornea. She is instructed to trim finger nails and keep the hands clean. A single probing done properly results in permanent cure. instilation of antibiotic should be under gone for five to six times a day for six week. the discharge changes to watery from mucopurulent. The application of pressure is generally called massaging of the sac. repetition and continuation of the process for five to six weeks. The treatment consists of opening of the block by external pressure over the sac and treatment of associated infection. Infact she should be asked to wash her hands with soap and water every time before she massages the sac.CR. or endoscopic D. 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. 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 term congenital dacryocystitis is not very appropriate because inflammation of the sac is secondary to duct obstruction. which dislodges the epithelial derbies. . After few days of repeated pressure application on the sac. It is generally bilateral and there is copious mucopuralent or purulent discharge without regurgitation. Incubation of nasolacrimal duct is another alternative. Relief of the obstruction is achieved by application of gentle and firm pressure over the sac to force the fluid down the nasolacrimal duct.DISORDERS OF LACRIMAL SYSTEM IN CHILDREN 91 Ophthalmianeonatorum becomes obvious with in first week. Management Management of the condition is simple provided the treatment is initiated before twelve weeks. (2) There is atrasia of boney canal (3) There is a nasal cause (4) Improper method.

The conditions waxes and wanes size and may altogether disappear following gentle pressure over the sac12. INFLAMMATION OF LACRIMAL SAC (DACRYOCYSTITIS) IN CHILDREN Inflammation of lacrimal sac in children is rarer than seen in adults.92 B.11 PEDIATRIC OPHTHALMOLOGY This is a rare condition present at birth as a bluish diffuse swelling medial to the medial canthus. The condition may be confused with a haemangioma. Submandibular and preauricular glands are enlarged. The diagnosis is confirmed by injection a coloured fluid in the sac through inferior puncta. The dye is seen to trickle through the opening. Symptoms of acute dacryocystitis consist of painful. diffuse swelling between the lateral side of nose and medial canthus which may develop fluctuation and pus forming at most dependent part. C. It may occasionally be bilateral. The condition is caused by simultaneous non canalisation of both the canaliculi and nasolacrimal duct. Management of acute dacryocystitis is systemic broad spectrum antibiotic (common organisms are H. treatment consists of removal of the fistulous tract and closure of gap in the sac and skin. Congenital Mucocele (Dacryocystocele)4a. Another type develop in presence of chronic bacterial dacryocystitis without congenital block in old children.T. may be done. The opening of the fistila may be so small that it may not be noticed. Symptoms are epiphora without infection. 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. influenza and pneumococcous) oral or injection. One of the theories put forward is failure of embryonic tissue to close fully14.13 otherwise. Acute dacryocystitis Acute dacryocystitis is generally pericystic cellulilis secondary to chronic infection of the sac. pink. Otherwise there is constant flow of tear form opening . The punctum may be altogether absent or may be rudimentary. Condition may subside only to recur or may form a fistula. 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 sac may have either acute dacryocystites or chronic dacryocystitis. oral . Probing under general anaesthesia is indicated other condition that may be confused with is meningocele in infants for which suitable tests including C. The exact mode of development of the condition is not well under stood. Acute dacryocyititis in children develop when a congenital nasolacrimal duct block has not been relieved. D. This can be sometimes bilateral. A. 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 . this is generally seen under 5 years of age. hot fomentation.

Indonesia. Lastly rhinosporidiosis is a common cause of chronic inflammation of sac in children in endemic area. It may follow chicken pox15 measels. mucocele. These area have high rain fall. Chronic dacryocystitis in children Chronic dacryocystitis in children is less common than adults. regurgitation of pus from the puncta on pressure over the sac.DISORDERS OF LACRIMAL SYSTEM IN CHILDREN 93 analgestic. Generally the condition is unilateral. The Signs are mucoid discharge near the medial canthus. Local antibiotics are not effective. The wound is allowed to heal from beneath. Jones I and II test 4. Lacrimal scintilography31 RHINOSPORIDIOSIS OF SAC16 Chronic dacryocystitis of the sac due to rhinosporidiosis is rarely seen outside endemic area of rhinosporidiosis. Philipines. Tamilnadu. if the pus points it is drained by stab incision and pus expressed out. The mucopurulent discharge may come from any or both puncta. Orissa. humid and warm climate as in Kerala. 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. external or endoscopic DCR is performed. diffuse painless swelling (mucocele) under the medial canthal ligament. When the persons share the same pond for bathing with infected water . Positive regurgitation test is a due to nasolacrimal duct obstruction which is confirmed by 1. India from where large number of cases17. Sri Lanka. Other possibility is mid fascial trauma with fracture bony nasolacrimal duct. Persistent absence of regurgitation from any puncta in presence of mucocele denotes corresponding canalicular block. When acute infection subsides.16 The symptoms consist of epiphora with or without conjunctivitis. as well ascends from nasopharynx. Lacrimal Syringing 3.18 have been reported too have pockets where the disease is prevalent. Taste test 2. B. Dacryocystography 5. West Bengal and Chhattisgarh. positive regurgitation test. Syringing and probing is contra indicated. The disease is caused by an organism Rhinosporidium Seebri Exact mode of spread of infection is not known. In children chronic dacryocystitis is caused due to unrelieved partial stenosis of nasolacrimal duct . where infection travels down from the conjunctiva. The disease is most prevalent in tropical and subtropical countries like India. A widely put forward theory is what the patients get the infection from infected cattle. coastal Andhra. The wound is dressed with magsulf and glycerine dipped ribbon gauge.

Associated conjunctivitis should be treated with local antibiotic drops. Primary where either the sac or conjunctiva is involve without nasal involvement.9 Congenital anomalies to lacrimal gland are far rarer than that of lacrimal passage. Management There is no specific systemic or local medial treatment. Appearance of the lacrimal sac is similar in both the types. skin over the swelling has an orange peel appearance. . Generally there is bleeding from the nose in case of associated nasal involvement. Which disappears by firm pressure. A. Absence of the lacrimal gland B. Though there is associated chronic dacryocystitis. Common anomalies of lacrimal gland are A. bilateral lesions are infrequent. There are two types of ocular involvement . B. following syregning the swelling increases in size. the swelling is non tender. It is rarely seen in other the members of the same family. All persons in the same locality do not get the same disease. There is a defuse pain less swelling over the sac. eiphora is not a dominant symptom. the water borne spores get lodged in the anatomical recesses like fornices. Aberrant lacrimal gland D. Crocodile tear. plica. Definite treatment is surgical removal of the sac. Selective involvement of a few persons in the same locality with similar life style point towards an immune mediated mechanism. Incubation period of the disease is not known.94 PEDIATRIC OPHTHALMOLOGY buffalo. It part of the tissue is left behind the condition is bound to recur. Here the infection spreads to the sac from the nose via nasolacrimal duct and conjunctiva is generally spared. Most probably the infected child has a pre existing total or partial block. Lymph nodes are not involved. The organism derives its name from the fact that it was first described as a nasal lesion. CONGENITAL ANOMALIES OF LACRIMAL GLAND1. nasolacrimal duct. Alacrima. Complete removal of sac ensures freedom from the symptoms and recurrence. 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. The condition is generally unilateral there is no regurgitation. simultaneous involvement of sac and conjunctiva is also rare. The nasolacrimal duct is either open or partially blocked. Fistula of lacrimal gland E. C. Nasal lesion happens to be most common involvement. Congenital cyst of lacrimal gland F. It is but natural that the lacrimal gland will be absent in absence of the conjunctiva as in cryptopthalomus. Precaution should be taken to remove whole of the sac without repture. Lacrimal gland is present in anophthalmos where the eye ball is absent but conjunctiva is present. A. Secondary.

Day syndrome. As the opening. . Riley—Day syndrome1. vascularisation. C. feeding difficulty. Alacrima In this condition there is partial or total absence of tear production in presence of lacrimal gland and its ducts. excess of sweting. The parents may notice that though the child has redness of the eye and intolerance to light. Aberrant lacrimal gland Aberrant lacrimal glands other than accessory lacrimal gland are found at any location under the conjunctiva. they may occasionally be associated with malformation of outer canthus or with congenital cyst of lacrimal gland . this may cause a ptosis. exact mechanism is not known most widely accepted theory is that there is lack of neural communication between the gland and the lacrimal nucleus . The children are less sensitive to pain. loss of corneal sensation. E. decreased tenden reflexes is common. It has wide spread systemic features which include. 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. D. It may be present at birth or may become obvious later. In this condition there is unilateral tearing during mastication. There is no specific treatment management is symptomatic ocular treatment consists of local tear substitute drops by day and ointment by night. It may be seen at limbus and mistaken for limbal dermoid. F. emotional out burst.19 Is a rare disorder mostly seen in Jewish children caused by sever depletion of parasympathomimetic neurones in spheno palatine ganglion.DISORDERS OF LACRIMAL SYSTEM IN CHILDREN 95 B. Crocodile Tear4a. absence of basic and reflex tear production. Treatment consists of removal of the tract and closure of the communication with the gland. absence of emotional tear production. corneal opacity. The tract is common site for recurrent infection. The opening is generally associated with a tuft of hair around it. The pupil constricts with very weak solution of pilocarpine (0. communicates with the lacrimal gland. S shaped curve in the upper lid and proptosis. commonest site is outer half of conjunctiva. Episodes of systemic hypertension. Fistula of lacrimal gland Lacrimal gland fishila are seen on outer side of the upper lid above the tarsal plate. cyclic vomiting muscular hypotony. Treatment consists of removal by orbitotomy if there is moderate to server proptosis. It is generally a bilateral condition. Ocular symptoms consist of alacrima. it is obvious that tear should flow out from the opening. the eyes do not water as is expected and has only thick mucus discharge.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. It may be associated with multiple cranial nerve anomaly specially sixth and seventh or may be part of congenital autonomic dysfunction known as Riley. Others feel that the lacrimal gland itself is hypoplastic. local anti biotic. More common is aquired paradoxical tearing following seventh nerve palsy due to aberrant degeneration. The flow is enhanced when there is stimulation of fifth nerve. It can even be seen on iris and choroid. On investigation both basic and reflex tearing are absent .0625%).

Discharging sinus 4. It is generally unilateral. Trauma : Dacryoadinitis. More common than above are : 1. Commonest virus is mumps followed by infective mononucleosis rarely by herpes zoster. ACUTE DACRYOADENITIS4a. It commonly . mild proptosis. ptosis. THE LACRIMAL GLAND DISEASES IN CHILDREN Disorders of lacrimal gland can be : A. The viruses infect the lacrimal glands more commonly than bacteria.96 PEDIATRIC OPHTHALMOLOGY Systemic condition that produce unilateral tearing due to gusto lacrimal reflex are : brain stem lesions. Ptosis. Congenital (very few) B.4b It is seen between five years to late teens. The disease starts with chills rigor and pain in the superio temporal aspect of orbit. Proptosis 7. Slow progressive swelling of lacrimal gland (chronic dacryodenitis) 3. Ocular surface disorder. Excessive production of tear—Lacrimation 2. Painful swelling of the main lacrimal gland. The symptoms of lacrimal gland disorders are : 1. 6. Neoplasia 4. Infective 2. 5. 2. Acute or chronic : Sarcoidosis. Acquired. Lacrimation. The acquired disorders can be : 1. Parotids and other salivary glands are invariably attacked along with lacrimal gland. (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. S shaped curvature of upper lid. The disease is caused by mumps virus single attack gives life long immunity. Scanty production of tear—Ocular surface disorders. Conjunctival congestion and rarely restricted movements and optic neuritis. S Shaped Curve of upper lid 8. Ipsilateral sixth nerve palsy is common in congenital crocodile tear. B. Inflammatory 3. There is a tense swelling over the lacrimal gland. infact any part of the eye except lens and vitreous can be involved by the disease.

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

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

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

98 B. Psychogenic

PEDIATRIC OPHTHALMOLOGY

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

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

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

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

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

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

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

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

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

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

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

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

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

DRY EYE SYNDROME IN CHILDREN IS BROUGHT ABOUT BY
A. Congenital absence of lacrimal gland, meibomian glands and congenital alacrima (Riley Day syndrome ) B. Traumatic—Loss 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. Steven’s 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 Sjogren’s 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, Steven’s Johnson syndrome or Riley Day syndrome. Trachoma rarely produces dry eye in children as it takes ten to twenty years to develop conjunctival scaring. Lagophthalmos is generally associated with lacrimation due to exposure keratitis and conjunctivitis. Inability to close the lid leads to poor resurfacing of the tear film. In neurotropic keratitis loss of corneal sensation results in less frequent blinking this also leads to poor tear spread. The symptoms are unexplained redness, foreign body sensation muciod discharge with relative scanty lacrimation, blurring of vision, fluctuation of vision. Diagnosis of dry eye History. Detailed history of trauma, adverse drug reaction, hypovitaminosis A should be elicited Examination Lid for coloboma and orbicularis palsy.

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Conjunctiva for evidence of vitamin A deficiency i.e. xerosis, Bitot’spot. 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 stability—Tear film break up time 2. Tear quantity—Schirmer 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. Schirmer’s Test 1a. This measures total quantity of aqueous tear i.e. basic and reflex. To perform the test a specially designed filter paper of Whatman 41 grade, 5mm wide and 35 mm long is used. The paper strip is folded at one end five millimetre from the end. The patient is seated in a dimly lit room, the strip is inserted behind the lower lid on the outer side of the IPA. In such a way that the folding line rests on the inner margin of the lid and remaining 30 mm hangs out side the lid. No anesthetic is used hence irritation of the paper will initiate the reflex tearing along with basal tearing. The patient is permitted to blink but not to squeeze the lids. The tear is

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drawn over the filter paper by capillary action and spreads to the other end. After five minutes the paper is removed and length of the paper wetted by tear is measured. In a normal eye this should be about 10 mm to 30 m wetting. Less than 10 mm is abnormal. If the strip is moist beyond 30 mm in lessthan five minutes it denotes either hyper secretion (lacrimation) or over flow of normal tear secretion. In both the condition dry eye status is excluded. The test should be postponed by half an hour if the lid has been manipulated. Schirmer’s 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 Steven’s Johnson’s 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. Steven’s Johnson’s syndrome is an unfortunate non curable disorder. Once a dry eye status has set in the first line of treatment consists of :1. Replacement of tear by a tear substitute that can either be cellulose derivative or polyvinyl alcohol. 2. Mucolytic drugs to remove excess of mucin 3. Reduction of tear drainage 4. Conservation of tear already available. The tear substitutes generally used are available as drops, ointment, gel or in the form of slow release inserts. They may have to be applied almost hourly when the child is awake. Bandage contact lenses along with tear substitutes are claimed to give more comfort to the child when it is possible to insert and retain a bandage lens.

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

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

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

15. Mukherjee P.K., Jain P.C., Mishra, R.K. ; Exenthemata ; A caus of chronicdacryocystitis in children IJO 17-27-30, 1969. 16. Mukharjee P.K. ; Rhinosporidiosis in current ocular therapy, fifth edition, edited by Fraunfelder F.T. and Roy F.H. pp-66-67, W.B. Saunders Company, Philadelphia 2000. 17. Mukharjee P.K. Shukla I.M. Deshpande and M. Praveena Kher ; Rhinosporidiosis of the lacrimalsac Ind. Jr. Oph. 30:513-514, 1982. 18. Shukla IM mukharjee P.K. Shushma Verma ; Primary rhinospori diosis of the eye int congress of oph Acta XXVI , Vol. 2, 864, 1982. 19. Axelord F.B, Soloman J.M. and D’Amico R. ; Familial dysautonomia unclassified diseases or conditions in Current Ocular therapy fifth edition p-285-287, edited by Fraunfelder F.T. and Roy F.H., Saunders Company, Philadelphia 2000. 19. (a) Hornblass A. ; Asimple test of lacrimal obstruction Arch OPH 90: 435-436, 1973. 19. (b) Hornblass A Ingris T.M. ; Lacrimal function tests. Arch oph. 97 : 1656-1679. 20. Kanski J.J. ; The lacrimal system in Clinical ophthalmology, second edition p-54-56 Butter Worth, London 1989. 21. Swartz N.G., Cohen M.S. ; Tearing and the lacrimal system in Ophthalmology Secrets , Edited by Vander J.F. and Gault J.A. , First Indian edition pp-226-227, Jay Pee Brothers, New Delhi 1998. 22. Piest K.L. and Walton M.A. ; Epiphora in Decision making in Ophthalmology, first Indian edition, P-82, edited by Van Heuven W.A.J. and Zwaan J.T., Harcour Brace and Company, Singapore 1998. 23. Scheie H.G. , Albert D.M. ; Ophthalmic raiodlogy. Im Text book of ophthalmology, Ninth edition P-254-256, W.B. Saunders Company 1977. 23. (a) Miller S.J.H. ; Diseases of lacrimal apparatus in Parsons daises of the eye, Seventieth edition p-327, Churchill Livingstone, London 1984. 23. (b) Chavis, R.K., Welham A.N., Maisey M. ; Quantitative lacrimal scientillography Archoph 96; 2066-2068, 1978. 24. Kanski J.J. ; The dry eye in Clinical Ophthalmology, Second edition p-46-52, Butter Worth International, edition 1989. 25. Daughman D. ; corneal Physiology in Principle and practice of Ophthal mology, first Indian Edition, Edited by Peyman. G.A. Sander D.R. and Goldberg M.F. p-356-358, Jay Pee Brothers, New Delhi 1987.

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

CHAPTER

6

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

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APPLIED ANATOMY OF CONJUNCTIVA1,2
For purpose of description, conjunctiva has been divided into three anatomical parts without any line of demarcation between two adjacent parts. They are : 1. Palpebral 2. Bulbar 3. Fornix 1. The palpebral part is divided into (a) Marginal, (b) Tarsal and (c) Orbital. The marginal conjunctiva. The marginal part is a transitional zone between the skin of the lid on the outer side and conjunctiva proper. It stretches from middle of the lid margin to sulcus subtarsalis on the posterior surface of the lid. The sulcus sub-tarsalis is a groove running all along the tarsal conjunctiva, 2mm from the sharp inner border of the lid. It is more developed in the upper lid than in the lower lid and is a favoured spot for small foreign bodies to get lodged. The two lacrimal puncta open on the marginal conjunctiva. The tarsal conjunctiva extends from the sub tarsal groove to the border of tarsus. A normal tarsal conjunctiva is transparent enough for the tarsal glands and blood vessels to be visible as light coloured streaks through the tarsal conjunctiva. The transparency of tarsal conjunctiva is lost in edema, hyperemia and scarring. The tarsal conjunctiva is attached to the underlying tarsal plate so firmly that it can neither be lifted off the tarsal plate nor can fluid accumulate under it. The orbital part of the palpebral conjunctiva is an ill defined area of loose conjunctiva extending from upper border of tarsal plate and blends with fornix. The Muller’s 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 Tenon’s capsule up to insertion of four recti. It is almost transparent, the white sclera is visible through normal bulbar conjunctiva. The conjunctival vessels are also visible in normal conjunctiva. With increase in number of vessels in the conjunctiva, the white conjunctiva become hyperemic. The bulbar conjunctiva is very loose 2-3 mm from the limbus and this laxity is used for sub conjunctival injection. A large amount of fluid can accumulate under this bulbar conjunctiva. The bulbar conjunctiva is transparent, moist and glistening in normal conditions. It is non keratanised but gets keratanised if exposed for long time as in lagophthalmos, proptosis, exophthalmos, ectropion of lower lid. It also gets keratanised in Vitamin A deficiency. The limbus3, 4, 5. The limbus is an important surgical landmark of the eye. It is an ill defined arcuate zone that is junction of cornea on one side and conjunctiva and sclera on the other side. Besides having surgical importance the limbus has two more functions. 1. It supplies stem cells for corneal epithelium, 2. The limbal plexus are the sole sources of blood supply to the corneal periphery.

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The limbus is a ring shaped zone widest superiorly and narrow on the side. The limbus has three lines that divide it into two surgical zones. These lines are : 1. Anterior limbal border, 2. Posterior limbal border, 3. Mid limbal line. The anterior limbal line represents the termination of conjunctiva and Tenons capsule at the corneal periphery, this overlies the termination of Bowman’s membrane. The posterior limbal line lies over the scleral spur 2mm away, concentric with anterior limbal line. It’s 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 Schwalbe’s 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, Krause’s 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 12’O clock i.e. roughly 13 mm from the superior limbus. It gradually becomes shallow on either side. Few slips of levator palpebral superior are attached to the deeper surface of the conjunctiva to give it its depth. It is a common site for foreign bodies to be lost causing intractable conjunctival discharge. It is the only part of the conjunctiva that requires double eversion to examine.

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The inferior fornix is smaller than superior fornix. It is located near the inferior orbital margin, its maximum depth is about 8mm at six o’ clock position. The lateral fornix is more ill defined than the former two, it extends 14mm from equator on the lateral side. The medial fornix is fully occupied by caruncle and plica semilunaris. The fornices have Krause’s 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 Krause’s 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 Krause’s glands but far less in number. There are about 3-5 glands mostly in the upper palpebral conjunctiva at the upper border of superior tarsus. Henles gland. Like goblet cells these also are not true glands. They are folds of mucous membrane between the tarsal conjunctiva and fornix. Blood supply of the conjunctiva6. Conjunctiva is very rich in blood supply. This makes large conjunctival wounds to heal fast. The conjunctiva is supplied by palpebral branches

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

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may be found to extend inside the eye in the vicinity of iris and ciliary body. About one third case of limbal dermoids are associated with systemic syndromes out of which Goldenhar Gorlin syndrome is the commonest. Treatment—Small 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, Goldenhar’s syndrome, optic nerve anomaly and macular hypoplasia. Congenital telangiectasis of conjunctiva. This is a hereditary anomaly of mucous membrane and skin. Out of the mucous membrane the conjunctiva is very commonly effected. It may be discovered at birth or infancy. Commonest age group being late adolescence and early adulthood. It may be associated with telangiectasia of retina. It may be seen anywhere on the conjunctiva but common site is bulbar and lower palpebral conjunctiva where it may be present as a star shaped bunch of vessels or a mass of vessels. As similar changes are seen in their mucous membranes the patient may have epistaxis, haematuria. Traumatic conjunctival bleeding is common. Small a telangiectasia may be obliterated by cryo.

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

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

10. Cause

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

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2. Orbit—Proptosis, 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 allergy—As seen in seasonal allergic conjunctivitis. (b) Endogenous allergen may produce picture similar to angio neurotic oedema. 2. Inflammation (a) Hyper acute conjunctivitis—Ophthalmia neonatorum, (b) Panophthalmitis,

118 (c) Endophthalmitis, (d) Cellulitis—Orbit, lids (e) Cavernous sinus thrombosis.

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

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

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

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

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

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

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Viruses responsible for conjunctivitis in children are : Herpes simplex, herpes zoster, measles, chicken pox, pharyngo conjunctival fever (adeno virus 3 and 7), epidemic kerato conjunctivitis (adeno virus 8 and 19), acute haemorrhagic conjunctivitis, molluscumcontagiosum. CONJUNCTIVITIS Conjunctivitis in children differ from those in adult. Some of the conjunctival infections like ophthalmia neonatorum and diphtheretic conjunctivitis are not only vision threatening, they can be life threatening also. Best classification of conjunctivitis would be on the basis of etiology. However some of the organisms produce identical clinical picture and are amenable to same treatment, hence for the sake of convenience infectious conjunctivitis will be discussed according to their mode of presentation i.e. Acute, sub-acute, or chronic under two broad heads i.e. Bacterial and Viral. Rest of the conjunctivitis would be discussed under individual heads. Bacterial conjunctivitis According to mode of onset, acute infectious conjunctivitis can be—Catarrhal, 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 Gram’s stain examination, culture and sensitivity. A smear stained with gram’s stain will

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render it possible to find out if the organism is cocci or bacilli, is it gram positive or negative, is it intra cellular or extra cellular and accordingly antibiotic can be started. However when facility of smear examination is not available, clinical judgement should be utilised to choose antibiotic for local instillation. 1. Commonly used antibiotics are : Chloramphenicol 0.3-0.5% solution and 1% ointment ; Gentamycin 0.3% drops ; Framycetin 0.5% drop ; Tobramycin 0.5% drops—ciprofloxacein 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 are—Associated allergy, smoke, dust, uncorrected errors of refraction, under nourishment etc. The conjunctiva on casual inspection may look normal but on pulling the lower lid down the fornices are congested. The discharge is mild, the lids rarely stick in the morning. Chronic blepharitis is very common in staphylococcal conjunctivitis and ulceration in the lower part of cornea, fortunately it is less common in children. Differential diagnosis of chronic bacterial conjunctivitis includes Trachoma, chronic follicular conjunctivitis, seasonal allergic conjunctivitis. Treatment. Treatment consists of complete elimination of causative organism and prevention of recurrence. For complete elimination it is necessary to find out the causative organism and the antibiotic to which it is sensitive. Both drops and ointments are used for weeks. Special attention should be paid to lid hygiene, correction of error of refraction and malnutrition.

SOME ACUTE BACERIAL CONJUNCTIVITIS IN CHILDREN
Membranous and pseudo membranes conjunctivitis. These two types of conjunctivitis have acute onset and are bilateral. Mode of clinical presentation in the two forms is

Membranous conjunctivitis is more severe form of the two. Sometimes it can occur in immunised children in a milder form. No attempt should be made to peel off the membrane as its bleeds profusely and leave a raw area. 2. Extra ocular muscle palsy is due to involvement of third. It is generally associated with diphtheretic membrane of nasopharynx. (4) Corneal involvement. All cases of membrane formation on conjunctiva true or pseudo must be treated as diphtheretic unless proved otherwise. Diphtheretic conjunctivitis9. Involvement of the cornea occurs due to two factors : (1) The bacteria can invade intact epithelium without trauma and cause corneal ulceration and perforation. The lid can not be separated or everted. Corneal complication. Cicatrisation. hard and temperature is raised. Though many organisms produce membranous conjunctivitis. The toxin is not neutralised by antibiotic. This form of conjunctivitis was very common when immunisation against diphtheria was not available. Management : 1. Changes in conjunctiva go along with the changes in the lid. Generally bilateral. They are tender. This follows as the membrane heals and shrinks. Clinically diphtheretic conjunctivitis can be divided into (1) Stage of infiltration. A true membrane develops over both the palpebral and tarsal conjunctiva. 2. In severe cases the membrane may slough off leaving a raw bleeding surface. Membrane formation. 4. The pre auricular lymph nodes are enlarged. trichiasis and rarely entropion. Diphtheric conjunctivitis may present as pseudo membranous conjunctivitis as well but incidence of pseudo conjunctivitis is less than true membranous conjunctivitis indiptheria. However it should be remembered that there are other causes of membranous conjunctivitis that may mimic as diphtheria conjunctivitis. diphtheretic conjunctivitis is more important than others due to its systemic complication.DISORDERS OF CONJUNCTIVA IN CHILDREN 125 almost same. symblepharon.10. Stage of infiltration. 5. fourth and sixth cranial nerves either as isolated palsy or in combination. Systemic antibiotic only reduces number of bacteria thus diminishing the source of toxin production. (5) Extra ocular muscle palsy. (3) Stage of cicatrisation. Antitoxin is administered systemically and locally in consultation with physician. As diphtheria produces a potent toxin that can be neutralised by antitoxin only before it gets fixed to the tissues. It still occurs in non immunised children. 3. (2) The corneal nutrition suffers due to widespread thrombosis of peri limbal blood vessels. The drugs commonly . The resulting scar may produce xerophthalmia. 3. It also eliminates other bacteria that are seen frequently in diphtheretic conjunctivitis. (2) Stage of membrane formation. There can be paralysis of convergence or divergence. board like rigidity is classical of diphtheretic conjunctivitis. 1. In fact the changes in lid are mostly due to changes in tarsal conjunctiva. the lids are swollen. It used to occur in epidemics. There may be paralysis of accommodation and iridoplegia. Commonest age to develop diphtheretic conjunctivitis is between 2 years to 8 years.

Erythromycin and ointment along with antitoxin. Local treatment consists of : 1.13 Ophthalmia neonatorum or conjunctivitis in new born is a serious infection of new born. Out of the above three. Cycloplegic should be used least keratitis develops. Etiology comprises of three groups of causative agents i. The condition is potentially vision threatening if not managed in time. Bacteria. viruses and chemical.12.000 to 100. 2. Once corneal involvement is noticed it should be treated vigorously.000 IU for 24 to 48 hours. Any conjunctivitis developing within first month of life is called ophthalmia neonatorum.126 PEDIATRIC OPHTHALMOLOGY used are . incidence of gonococcal ophthalmia neonatorum has come down considerably. All Membrane ++ – – ++ + – – – + – – – + + + + + + + + + + – + Pseudo membrane + + + + + + + . the bacterial conjunctivitis in neonate is most common. clindamycin and tetracyclin.e. However it remains an ocular emergency. Erythromycin in dose of 25 mg to 50/kg for days. Local administration of antitoxin in the conjunctival sac between 10. With better health care and antenatal screening of the mother.000 IU BD for ten days. Local instillation of fresh solution of Penicillin. 3. Other drugs used are ampicillin. 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. Other causes of membranous and pseudo membranous conjunctivitis : Cause Streptococci E Coli H. Previously Gonococcus14 was considered to be commonest organism to cause ophthalmia neonatorum. It is a notifiable disease.Penicillin G 600.

Out of all bacteria. Gonococcus is one of the few organisms that can pass through intact corneal epithelium. It is far milder than bacterial conjunctivitis. Incidence of gonococcal conjunctivitis in neonates has shown a gradual decline due to better maternal and child care and use of prophylactic anti microbial. The lids are swollen. watery discharge. Corneal involvement is most serious vision threatening findings. If the mother has active lesion of genital passage a caesarean sections saves the child from getting conjunctivitis. diffuse or dendritic keratitis. Otherwise a small perforation may heal by formation of central leucoma. Herpes simplex conjunctivitis is also acquired from infected birth canal. 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. gonococcus has most severe form of infection and has shortest incubation period of 23 days. pseudomonas. rigid and may stick together. Incidence of neonatal conjunctivitis by Chlamydia and herpes simplex is showing an upward surge. haemophilus3. basophils. Once perforation has occurred endophthalmitis and panophthalmitis is very common. Source of infection is birth canal. may protrude through the lids. leucoma adherent and anterior pallor cataract. It develops after 10 days as mild conjunctivitis. pneunococcus.e. Loss of lustre of cornea is a bad sign. formation of true or pseudo membrane.2 Bacteria other than gonococci that produce ophthalmia neonatorum are : Staphylococcus. This should be sufficient to start anti gonococcal treatment but .e.DISORDERS OF CONJUNCTIVA IN CHILDREN 127 purulent conjunctivitis in neonate must be considered as gonococcal unless proved otherwise. All cases of mucopurulent bilateral conjunctivitis should be considered to be due to gonococcus unless proved to be otherwise. It never perforates. Presence of gonococcus is confirmed by Gram’s 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. Non bacterial micro organism that produce ophthalmia neonatorum are Chlamydia trachomatous and herpes simplex. in fact any bacteria that may find its way to conjunctiva of new born can cause purulent conjunctivitis. Even if there is no perforation gonococcal uveitis is very common. There may be blood discharge. 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. 4-5 days and are generally due to secondary infection after birth. they have longer incubation period i. It also gives nature of intra cellular or extra cellular inflammatory cells i. Chlamydia caused inclusion conjunctivitis. Neonatal conjunctivitis by other bacteria are milder than gonococcal. There may be associated skin vesicles. Gram negative intracellular diplococci and polymorpho nuclear neutrophils indicate gonococcal infection. pseudomonas infection is more common in prematures. lymphocytes and inclusion bodies. and streptococcus. The eye soon develops nystagmus. Diagnosis of ophthalmia neonatorum specially bacterial is not difficult. There may be corneal infiltration. Corneal involvement is milder in the form of micro pannus that may cause permanent scarring. polymorph. takes five to seven days to develop. The disorder is noticed only after there is out pouring of muco purulent discharge from the eye. central corneal ulcer which may perforate in very short period. On separation of the lids that may require use of lid retractor the conjunctiva is chemosed.

1% Tetracyclin. within 24 hours and is mild and self limiting.3% Ciprofloxicin. Then every two hourly.3% gentamycin. 0. Any of the following antibiotics can be used as drops or ointment— 0. Crede’s 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. It reduces severity of the condition.16. Management of clinically established gonococcal conjunctivitis13. edema of lids discharge and redness of surrounding skin. Irrigation with balanced solution have doubtful efficacy. In suspected chlamydia infection of the mother 10% sulpha cetamide may be added to any of the above. This chemical conjunctivitis develops earlier than any other conjunctivitis i. This includes (a) Antenatal screening of mother for evidence of gonococcal. (ii) Povidon—Iodine 2. If found to be infected. 0.128 PEDIATRIC OPHTHALMOLOGY to find out if the stain is penicillin resistance or not and its sensitivity to other antibiotics. then every ten minutes for six instillation’s. 16 in neonates comprise of : 1.5% has been claimed to be better than AGNO3 without side effects of latter. every five minutes for six times. gentamycin 0. (iv) If the mother is known to suffer from gonococcal infection. The eyes of new born should be cleaned by two separate wet cotton swabs. one for each eye from lateral to medial side. culture and sensitivity test must be done because resistant stain may prove to be life threatening. (2) Treatment of various microbial ophthalmia in new born. Cleaning of ‘muco purulent discharge frequently using separate swab for each eye and each cleaning. Management consists of—(1) Prophylaxis.3% Ciprofloxicin. Crede’s prophylaxis does not always eliminate possibility of ophthalmia neonatorum. 1. the neonates are at high risk of developing ophthalmia neonatorum.e.5% may be put to reduce stickiness of lids. proper treatment should be initiated.5% erythromycin.000 unit per ml. Causes redness of conjunctiva. chlamydia and herpes simplex of genital tract. Instillation of fresh aqueous solution of crystalline penicillin G. In case of herpes simplex of birth canal an elective caesarean section may be done. Prophylaxis15.3% or any ophthalmic drop that is effective against gonococci may . Other drugs like 0.000 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. (b) Complete antisepsis and asepsis should be followed during and after delivery. 2. then one hourly for six instillation. As ointment of penicillin is no more available erythromycin 0. Such infants should get single intra muscular injection of 50. (c) Prophylactic anti microbial drop : (i) 1% silver nitrate drop (Cred’s 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. (iii) Antibiotic. in strength of 100.

They prefer systemic administration of systemic crystalline penicillin twice a day for seven days or cefotaxime in neo natal dose in consultation with neonatologist. Treatment of other bacterial conjunctivitis : (a) Pseudomonas conjunctivitis requires as prompt treatment as gonococcus specially in pre mature infants—Topical gentamycin or tobramycin in strength of 0. membranous or pseudo membranous conjunctivitis. It is . Atropine drops are better avoided as it is likely to be absorbed from nasolacrimal passage and throat in amount sufficient to produce systemic toxicity.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. In recent years hyper acute conjunctivitis similar to ophthalmia neonatorum has been reported from various parts of the world. both are gram negative cocci. rhinitis and pneunonitis. but can be found as a symptomatic strain in conjunctival sac. It is mostly seen in children as acute muco purulent conjunctivitis. The organism belongs to family Neissericeae.DISORDERS OF CONJUNCTIVA IN CHILDREN 129 be used in the same frequency. Recent studies have started doubting need and efficacy of local antibiotic drops. The acinetobactor fortunately does not cause systemic infection but is penicillin resistant.5% erythromycin every 2 hourly. 3. Pneumococcal conjunctivitis Pneumococci are generally present in respiratory system.e. It is best treated by local 0. If cornea shows any evidence of involvement 0. Treatment comprises of instillation of 10% sulpha-cetamide drop and erythromycin 0. (d) Coliform organisms can be treated with gentamycin drops. (f ) Chalmydia conjunctivitis in neonate has become more common than in the past.3% as in case of gonococcus with ointment of the same antibiotic three to four times recommended. It is milder than bacterial infection but may have long term local as well as systemic effect i. It can cause ophthalmia neonatorum. It can involve the conjunctiva independent of systemic involvement. Treatment with systemic anti viral drugs is needed in consultation with neo-natologist. untreated cases may be fatal in as many as 50% of cases. (e) Acineto bactor17. (c) Conjunctivitis with other gram positive cocci and diplococci is treated with 0. It gradually produces systemic infection with ocular involvement and is a common cause of hypopyon.5% atropine ointment is put to treat and/or prevent associated uveitis.3% ciprofloxacein or 0. Presence of skin vesicles may point towards diagnosis of herpes simplex. It is difficult to differentiate it on gram’s stain from gonococcus. It is one of the commonest organisms that cause chronic dacryocystitis. Diagnosis of chlamydial conjunctivitis is confirmed by Giemsa stain that shows besophilic cytoplasmic inclusion bodies. Herpes virus—conjunctivitis is uncommon but it is potentially vision and life threatening infection in neonate. (b) Haemophilus is best treated either by local gentamycin drops or 10-15 percent sulphacetamide drops.3% Tobramycin. with patecheal haemorrhage. otitis.

Erythromycin 0. 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. Inferior corneal infiltration is common. Acute (i) Herpes simplex. 1. Management is similar to any other bacterial conjunctivitis. that can cause patchy sub conjunctival haemorrhage. sulpha cetamide. Haemophilus influenzae18 and H.000 units per cc can also be given as hourly drop. Systemic antibodies do not seem to have any advantage over local instillation. 4. Conjunctiva is involved in children as primary infection mostly by type I but occasionally by type II virus as well. Cefazoline 50mg/cc may be given in place of penicillin. can have serious systemic involvement and peri orbital cellutitus.000 unit per cc every hourly during day and every four hourly by night. (3) Teenage. Fortified aqueous penicillin G. 2. Neonatal herpes simplex conjunctivitis can be congenital or acquired. and orbital cellulitis Treatment Local penicillin drop. with acute onset that lasts for about 10 days. 3. aegyptius.e. rare and associated with severe systemic involvement hence most of the time fatal. acute mucopurulent conjunctivitis membrane or pseudo membrane Keratitis Gas in AC Keratitis Erythromycin. . Commonly used antibiotics are : 1. till the acute symptoms subside.5% may be given at night as ointment. bacitricin cefazolin Chlormphenicol Gentamycin. erythromycin drop. Corneal involvement consists of a central ulcer with or without hypopyon. However every child suspected to have pneumococcal conjunctivitis should have a pediatric consultation for possible systemic involvement. In acquired form the neonate gets infection during passage through birth canal. All cases of suspected corneal involvement should be treated as emergency with atropine. vigorous antibiotic instillation and beta blockers. (1) Neonates. (2) Between six months to 5 years. Coli Hyperemia. Becitricin 10. bacitricin.130 PEDIATRIC OPHTHALMOLOGY spread by hand to hand contact in children. The organism is best grown on chocolate agar medium. Koch-Weeks bacteria are common causes of conjunctivitis in children. chemosis. solution containing 100. Tobramycin Other feature Keratitis. The former is. Viral conjunctivitis Viral conjunctivitis in children can be (1) Acute or (2) Chronic. There are three age groups in which herpes simplex involves a pediatric patients i.

Tender pre auricular nodes develop in early stage of disease. In children it is associated with sore throat. Conjunctivitis is muco purulent always associated with vesicle formation on the lid. pre auricular non tender lymphadenopathy. recur as herpetic keratitis later. It is less common in children but children are not immune. It develops as follicular conjunctivitis (after three months of age) with lymphadenopathy. lid margin. There may be secondary bacterial infection. forehead. Other sero types may also cause the disease. the eye is congested. It is generally unilateral. As herpes simplex conjunctivitis is a primary infection. As it is a self limiting disease. pharyngitis. It is always unilateral. Treatment is non specific and symptomatic. It is caused by adeno virus 8 and 19. Treatment. This contagious conjunctivitis is common in children. In severe cases pseudo membrane develops. incubation period is 3 to 12 days. Conjunctivitis is . Corneal involvement require local use of anti viral drugs along with cycloplegic. Local antibiotics are prescribed to prevent secondary bacterial infection. It has incubation period of 5 to 12 days. One eye is first involved and more severe than the other eye. the virus may pass into the trigeminal ganglion and remain dormant. It sometimes develops following bath in a contaminated swimming pool. it lasts for two to three weeks. Conjunctivitis is self limiting and resolves in a week. Conjunctivitis in pharyngo conjunctival fever is very common in children. The teenager gets it by kissing an infected person and rarely as sexually transmitted disease.DISORDERS OF CONJUNCTIVA IN CHILDREN 131 The child between 3 to 5 years gets infection from infected adults. There may be pseudo membrane formation. fever and diarrhoea. Conjunctivitis does not require any specific treatment. Treatment. The conjunctivitis to beginwith is papillary. Incubation period is 5 to 12 days. Treatment is directed towards the disease in toto. Mucopurulent discharge denotes superimposed bacterial infection. There may be actual vescicles on the conjunctiva. There is watery discharge. conjunctival and corneal involvement in various combinations. there may be follicle formation. The skin lesion consists of vesicles over the skin of the lid. generally unilateral may become bilateral generally there is a watery discharge. It is difficult to diagnose clinically. (ii) Herpes zoster conjunctivitis. Cornea may be involved in the form of superficial fine epithelial keratitis. (iv) Epidemic kerato conjunctivitis. Sometimes adeno virus 4 may also cause pharyngo conjunctival fever and conjunctivitis. Treatment. Herpes simplex conjunctivitis irrespective of age of onset have cutaneous. Conjunctivitis is self limiting lasting for seven to fifteen days. The vesicles may cross over the mid line. The disease has more serious corneal and lid involvement than herpes simplex. The conjunctivitis is follicular in nature. The corneal involvement starts as small epithelial lesions that join together to form typical dendritic keratitis. Steroids are contra indicated. Herpes zoster conjunctivitis is caused by vericella zoster virus. It is a part of syndrome consisting of fever. Onset of photophobia denotes corneal involvement. The causative viruses are adeno virus 3 and 7. However corneal infiltrates may persist for a month. The lids are generally edematous. it does not require any specific treatment. The infection is self limited. (iii) Pharyngo conjunctival fever.

12 to 48 hours. umblicated vesicle may be seen. The virus is transmitted by close person to person contact and by fomites. Occasionally it may be spread by contaminated instruments. It is caused by entero virus type70. phlycten. No anti-viral drugs are required.e. The disease is self limiting lasting for seven to ten days. common linens or ophthalmic instruments used to examine infected eye. Mumps—Papillary or follicular conjunctivitis. Measles—Koplik’s spot. Pre auricular lymphadenopathy is a common feature. In absence of corneal involvement weak solution of steroid drops may shorten duration. The patechea disappear early. Recurrence in the same patient has been observed. Molluscum contagiosum—Chronic unilateral conjunctivitis. Occasionally there may be uveitis. It is a misconception that dark glasses prevent spread. follicular sub conjunctival haemorrhage. Dark glasses help in reducing photophobia. Chlamydia infection in children19 Chlamydia (formerly known as Bedsonia) are a group of micro-organism in between bacteria and viruses. sub conjunctival haemorrhage. it presents as sub epithelial opacities that may appear after conjunctivitis subsides and may persist for months.e. Non steroidal anti inflammatory drugs are given to relieve pain. vescicle formation. and body ache. Broad spectrum antibiotics are given to prevent and treat associated secondary bacterial infection. There is no specific treatment known.132 PEDIATRIC OPHTHALMOLOGY follicular in nature. There may be lymphadenopathy. The disease does not give immunity. water. Treatment is symptomatic. It has an acute onset first in one eye with in few hours the other eye gets involved. 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. The incubation period is short i. Other viral infections that produce conjunctivitis in children : Viral—Conjunctival lesions. The disease spreads among the school children in epidemics. Influenza—Catarrhal conjunctivitis. there may be papillary reaction and pseudo membrane formation. Sometimes there may be chemosis of conjunctiva and follicle formation. Conjunctivitis may be associated with fever. Corneal involvement is common. Treatment is directed towards symptoms. Treatment. Chicken pox—Muco purulent conjunctivitis. The symptoms are intense redness of conjunctiva with copious watery discharge. malaise. The epidemics keep on appearing at regular intervals mostly in the months of late June and early July. pannus formation. large haemorrhage take ten to fifteen days to clear. schools etc. These spots may join together and form a large sub-conjunctival haemorrhage. lotions or drops following ocular examination. (v) Acute haemorrhage conjunctivitis. It is most commonly seen in crowded localities i. This acute conjunctivitis was first noticed in 1969 and has been known by various eponyms. subconjunctival haemorrhage. motor paralysis of lower limbs have been reported. They are intracellular organisms that are difficult to culture. Onset of corneal involvement in form of epithelial keratitis heralds photophobia. Their . follicle formation. lid edema and pain ranging from mild discomfort to severe pain.

It is mostly acquired in childhood. The incubation period is longer than gonococcal ophthalmia i. cervicitis in adults and secondary conjunctivitis in adults which resembles trachoma in many aspects but is milder than trachoma. Infants with inclusion conjunctivitis may develop pneumonitis and gastro-enteritis in first six months of life. Common chlamydiae are : 1. 3.e. It is not prevented by usual Crede's prophylaxis but can be prevented by tetracyclin or erythromycin. C psittacosis. Trachomatis is gram negative organism that is sensitive to sulphonamide. . The trachoma does not reach the cicatricial stage in children as it takes more than decade to develop cicatricial stage. They multiply by binary fission. There is lacrimation and serous discharge. SURMA and flies play an important part in spread of trachoma in children. No race is immune. dusty climate. Hence drugs are to be administered for long time. Neonate. The former two produce kerato conjunctivitis in children. 5 to 12 days. Young adults. A. it is a sexually transmitted disease. Uncomplicated trachoma in children do not cause blindness. In children. Inclusion conjunctivitis effects patient in two distinct age groups : 1. C. Ba. Para trachoma causes urethritis. Neonatal chlamydiae disease is acquired by new born during birth via infected birth canal of the mother. Lympho granuloma venerum. Ba and C. However. It is a bilateral condition. 22 Trachoma is a chronic kerato conjunctivitis due to C Trachomatous sero type A. It produces nongonococcal ophthalmia neonatorum. regular cleaning of face. C pneumonae and 2. the lids are swollen. C. it is almost exclusively human pathogen.DISORDERS OF CONJUNCTIVA IN CHILDREN 133 intracellular nature gives them partial immunity against drugs. 2. 21. The disease is transmitted from person to person by fomites. boys and girls are equally affected.e. Trachoma is a disease of dry. Psittacosis former is more common cause of ocular manifestation while latter produces more systemic infection. In adults women have more serious involvement than men. associated malnutrition and xerophthalmia may lead to corneal ulceration. The children get trachoma from infected family members. most of adults acquire trachoma in childhood. Out of C Trachomatis and C. C that produce trachoma and D to K that produce inclusion conjunctivitis also called paratrachoma.29 Clinical feature of trachoma Clinical features of trachoma in children differ from those in adults. perforation and loss of eye in children. B. however secondary bacterial infection. Trachoma20. Contaminated KAJAL. C. The organism is also known as TRIC where TR stands for trachoma and IC stands for inclusion conjunctivitis. B. It is commonest preventable infectious disease of eye that may otherwise lead to blindness in adults. Improvement of health care and awareness of cleanliness has drastically reduced incidence of disease in areas where it used to be endemic. availability of free flowing water and ownership of flush latrine in the family reduce spread of trachoma. It has been noticed that reduction in fly population. Untreated cases heal in four to five months but may persist for years causing chronic follicular conjunctivitis and pannus formation. and playmates. It is a disease of poor children with poor hygiene. Trachomatis. C. 4. Trochomatis has many types i.

(4) Healed trachoma. 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. Stage III : Follicles and scarring at upper tarsal conjunctiva. papillae formation. McCallau23. Papillary hypertrophy follicles or Herbert’s pits at limbus. disease no longer infectious. Established florid trachoma mature follicles. redness of the eye. which have great prognostic value. Clinical feature Code Tr D Tr D Tr I Trachoma II Tr II Trachoma Stage III Tr III Trachoma Stage IV Tr IV . pre auricular nodes may be enlarged. Cicatrising trachoma. Stage IV : Healed trachoma. Signs. (b) : Papillary hypertrophy masking follicular hypertrophy.134 PEDIATRIC OPHTHALMOLOGY Incubation period of trachoma is short i. Symptoms are so common in endemic areas that it is taken as natural phenomenon. seven days (range 5 to 12 days) It begins as mild conjunctivitis similar to bacterial conjunctivitis and indistinguishable form it. Conjunctival congestion. Symptoms are mild in children unless secondary bacterial infection is superimposed. moderate swelling of the lids. The classification takes into consideration only conjunctival changes. WHO 198724 adopted a better classification. (2) Established trachoma (3) Cicatrising trachoma. Scars present. No weightage is given to keratopathy and . Symptoms. No inflammation. Conjunctival scar. Some of the signs of stage I and II. discharge. No sign of inflammation and late scarring of tarsal conjunctiva. Smear from conjunctival sac in a case of pure trachoma does not grow bacteria on culture.e. superficial keratitis in the upper part and mild vascularisation. Various stages are : Stage I : Immature follicles in upper palpebral 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. Symptoms consist of watering. Stage II (a) : Prominent follicular hypertrophy. 23A classified the conjunctival signs into following grades : (1) Incipient trachoma. follicular hypertrophy mostly in the fornices and upper part of tarsal conjunctiva.

CO—Corneal opacity—Easily visible corneal opacity over the pupil. Vascularization of cornea is superficial in nature. Vacularization. Trachomatous pannus is said to be : (i) Progressive when cellular infiltration extends beyond the terminal end of the newly formed blood vessels. later the Bowman’s membrane may be destroyed. Presence of easily visible scarring in upper conjunctiva. Ba and C with secondary bacterial infection superimposed. The cellular infiltration is lymphoid in nature with treatment the vessels disappear leaving a clear cornea. 26. Epithelial and sub epithelial punctate keratitis. In early stage it is visible only on slit lamp examination. Sub epithelial punctate keratitis are large enough to be visible without magnification. 27 : TF—Topical antibiotic T1—Topical and systemic antibiotic TT—Surgery Corneal changes in trachoma Corneal changes in trachoma are early to appear. Presence of five or more follicles of 0. At least on eye lash rubs on the eye ball. They can be due to (1) chlamydia infection of corneal epithelium.5 mm or larger is significant. (2) Lid changes leading to secondary changes in cornea (Not generally seen in children). Flies in unhygienic condition where free flowing water is not available is most important factor in causing blinding trachoma. It consists of epithelial invasion of conjunctival vessels with diffuse infiltration and epithelial punctate keratopathy. WHO recommended for management of trachoma25. TS TT and CO due to trachoma are not seen in children. Initially the vessels lie between epithelium and Bowman’s membrane.DISEASE OF CONJUNCTIVA IN CHILDREN 135 Blinding trachoma is seen in endemic areas. The corneal changes are : 1. Trachoma in any stage does not produce perforation unless bacterial infection is superimposed. TS—Trachomatous scarring. only obliterated vessels may be visible on higher . these are small in size seen generally in the upper part of the cornea. B. The pannus of trachoma is mostly seen in the upper part of cornea associated with cloudiness of cornea. TI—Trachomatous inflammation—Intense. it can occur at any part. WHO grading of trachoma as guidelines for treatment : TF—Trachomatous inflammation—Follicular seen in children. in upper tarsal conjunctiva. However. TT—Trachomatous trichiasis. (ii) Regressive when the ends of the vessels extend beyond the cellular infiltration. infection is caused by sero type A. 2. pronounced inflammatory thickening of tarsal conjunctiva that obscure more than 50% of deep tarsal vessels. Severity of pannus formation depends upon duration and severity of infection.

2. Epithelial keratitis. Trachoma on McCoy cells is said to be gold standard but is very difficult and costly. Trachoma is one of major cause of blindness yet it is one of the most treatable and preventable disease. edema and follicle formation are common changes in trachoma. palpebral type of spring catarrh. Presence of basophilic intra cytoplasmic inclusion bodies in epithelial cells are diagnostic. (b) Awareness about trachoma. 3. riboflavin deficiency. Trachomatous pannus. Papillary hypertrophy. Culture of C. Conjunctival smear for inclusion bodies.136 PEDIATRIC OPHTHALMOLOGY magnification. Follicles in tarsal conjunctiva and/or limbus. chronic conjunctivitis. dry eye syndrome. multiple phlycten. Follicle formation. Characteristic signs of trachoma in children : 1. 3. 3. The follicles are generally small and transient. Presence of immuno fluorescent monoclonal antibodies is specific. Diagnosis of trachoma Diagnosis of trachoma in endemic area is easy clinically. 2. Limbal changes in trachoma Hyperemia. disappear without any trace but larger follicles stay for longer time and when rupture leave depressed areas called Herbert’s pits which are generally not seen in children. Management of trachoma in children : Prophylaxis : (a) Chemo prophylaxis. Common conditions are : Acute and chronic follicular conjunctivitis. Chemo prophylaxis All children in endemic area should be put on any of the local anti-trachoma chemo therapeutic drugs : . Differential diagnosis of trachoma in children : Consist of all case that produce following bilateral signs either alone or in combination. Early trachoma and trachoma outside endemic area present difficulty in diagnosis because there are other causes that produce similar signs. Combination of trachomatous pannus with any of above two is pathognomic in established trachoma. 2. 1. healed interstitial keratitis. Prophylaxis. A permanent haze results with destruction of Bowman’s membrane. Superficial vascularisation. Diagnosis of early trachoma 1.

WHO schedule for management of trachoma in endemic area consist of SAFE method. Sulpha cetamide drops 10% to 20% 4 times a day in both eyes is prescribed for six weeks. Trachoma is sensitive to both sulpha and many broad spectrum antibiotics. Then a gap of six weeks is given and the regime is repeated. 3. Local chemotherapeutic drugs (TF Stage) : 1. 1% Tetracyclin. Azithromycin – 250 mg of single dose is said to be as effective as 1% tetracyclin TDS for six weeks. 3. Chloramphenicol 0. Sulpha methoxazole 30mg/kg in divided doses for 3 weeks along with local antibiotic drops. Systemic Tetracyclin and doxycycline should not be used in children under eight years of age.29 Treatment of trachoma C. Environment. Doxycycline – 1. It is emphasised that trachoma is : — Preventable and curable disease. 4. 29. 2. Erythromycin – 30 mg/kg in divided dose for 3 weeks. 5. 1% chloramphenicol ointment. ciprofloxin 0. ciprofloxin eye ointment have also been found to be effective. Rifampin eye ointment. Tetracyclin 1% oxy tetracyclin 1%.5 mg/kg single dose for 15 days. 30. Vector control. Tetracyclin – 15mg/kg in divided dose along with local antibiotics for 3 weeks not given under eight year of age. 31.5% Erythromycin ointment twice a day for six weeks.5% drop or Ciprofloxin 0. oxy tetracyclin ointment. — Preventing use of KAJAL and SURMA from common container. ownership of septic latrine in the family/ community reduces incidence of trachoma. Trachoma awareness28. 0. where S = stands for surgery A = stands for local and systemic antibiotic .3% ointment three times a day alone or at the bed time along with local sulpha or antibiotic drops for six weeks. Chlortetracyclin 1%. Fly is commonest vector that disseminate trachoma. If fly density can be reduced in the community trachoma can be eliminated. 2. 3.3% drop 4 times a day for six weeks. Availability of running water. 2. Oral antibiotic agents—In TT stage : 1. As the organism is obligatory intracellular in nature prolonged treatment in effective dose is required. — Face wash—Regular face wash with running water greatly reduces incidence of trachoma.DISEASE OF CONJUNCTIVA IN CHILDREN 137 1. In the mean time all the other members of community who have active trachoma are treated for trachoma.

1. The reaction is type one allergy . The allergen responsible for immediate (humoral) hypersensitivity are exogenous. Polymers are also capable of producing allergic conjunctivitis. Most accepted theory regarding allergic conjunctivitis is that exogenous allergens disrupt the mast cell membrane and cause degeneration. In under developed countries its presentation and outcome may be over shadowed by prevailing infective conjunctivitis and vitamin A deficiency. hay. disposal of human waste. moulds or of animal origin i.138 F = Face wash (personal hygiene) PEDIATRIC OPHTHALMOLOGY E = Environmental changes (availability of running water. race. Those responsible for delayed (cellular) hypersensitivity are endogenous. Immediate hypersensitivity (humoral). fur. dander. edema. inflammation and exudation. cosmetics. Factors that make conjunctiva vulnerable to allergic reaction are : (i) Conjunctiva is most exposed mucous membrane of the body. Delayed hypersensitivity (cellular) or 3. vector control) Surgery is not required in children. No race. husk. duration vary from few minutes to few years. 2. Food and additives are known to produce allergic conjunctivitis. wool. genetic predisposition and reaction to allergen. Many a times it is impossible to find out the allergen. drugs. age. These factors are—Geography. dust. Allergic conjunctivitis may be seen both in atopic and non atopic patients. (v) It is constantly bombarded with exogenous allergen. pollen. sex or age is immune. plastics. (iv) It is rich in lymphatic. Its frequency in children may surpass infective conjunctivitis in developed countries. various prostaglandins. (iii) It is very loose. 34 Allergic conjunctivitis is influenced by many factors which may work separately or in various combinations.e. The chemicals released in this process are—histamine.e. mites. Allergic conjunctivitis in children32 Allergic conjunctivitis is almost pandemic ocular disorder. The exogenous allergen are organic matters either of vegetable origin i. sex. Clinical presentation33. climate. (ii) It is rich in blood supply. Exact mechanism of allergic conjunctivitis is not well understood. Presenting features of allergic conjunctivitis are variable. Allergic conjunctivitis are generally bilateral. may be asymmetrical. leucotriens. Auto immune. All allergic conjunctivitis are—self limiting. releasing a host of chemicals that cause—vasodilatation. 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.

Local steroids are not necessary. hay etc. sore throat. Condition is invariably bilateral with mild itching and slight ropy discharge. In milder cases there is preponderance of mast cells. There is no anterior chamber reaction. Cornea is bright. Acute allergic conjunctivitis. vision may be blurred due to over hanging chemosed conjunctiva.DISEASE OF CONJUNCTIVA IN CHILDREN 139 and IgE mediated. The conjunctiva may be so edematous as to protrude through the inter palpebral fissure. Immediate (humoral) hypersensitivity33 (a) Acute allergic conjunctivitis. It is due to large inoculum of exogenous allergen. less troublesome and easy to manage than latter three which are less frequent but difficult to manage. It is very common among children. Delayed hypersensitivity (cellular) 3. It can manifest as early as first year of life. Symptoms coincide with release of allergen in the atmosphere (pollen. Acute allergic conjunctivitis is equivalent to urticaria of the skin. (b) Seasonal allergic conjunctivitis. The symptoms pass off within half an hour to one hour without leaving any trace. (f) Atopic kerato conjunctivitis. hence may be associated with rhinitis. Treatment. Muco purulent discharge. corneal involvement and lymphadenopathy point towards anti microbial conjunctivitis. Cornea remains unaffected. dry eye syndrome which are not seasonal nor produce itching. They have seasonal predilection. The patient complains of sudden onset of copious watering from the eye which may be unilateral or bilateral.) Main symptoms are itching. The lids are swollen. There is no lymphadenopathy. The cornea forms the floor of the crater (well) of the chemosed conjunctiva. (d) Vernal kerato conjunctivitis. 2. Conjunctival scrapping show eosinophils. Best is to avoid the allergen if it is known. 1. otherwise local vasoconstrictors. All are caused due to exogenous allergen. (g) Giant papillary conjunctivitis. The first three are more common. 2. Conjunctival reaction consists of mild to moderate papillae or follicle formation. Seasonal allergic conjunctivitis. 1. There is no AC reaction. In severe cases there is increase in T cell lymphocytes. redness. watering. There is no lymphadenopathy. Mast cell inhibitors have no role. recurrence are common. no follicles are seen. Classification of allergic conjunctivitis is difficult. Fast developing chemosis of congested conjunctiva. Cold compress applied within few minutes may give relief. . Seasonal allergic conjunctivitis is equivalent to hay fever. (c) Perennial allergic conjunctivitis. The condition is to be differentiated from trachoma. following classification may fulfil many of the clinical criteria and possible mode of management. However. Auto immune disease (a) Cicatricial pemphigoid (seen in elders) (b) Erythema multi formis (Stevens Johnson syndrome) minor and major. There may be moderate papillary reaction. astringent and short acting systemic antihistamine tablets are sufficient.

Vernal (spring) kerato conjunctivitis34. It has been reported as early as first year of life. animal dander. It is more common in summer in tropical and sub-tropical countries. Low dose of non sedative antihistamine tablets give additional relief. duration is shorter. The condition may be confused with trachoma. In spite of its name it need not manifest in spring always. redness and watering. The post puberty cases are less in number. It is a bilateral condition. Steroids are best avoided. after which it may automatically heal. animal or chemical in nature like house dust mite.5% drops three times or Flurbiprophen 0. It is best used as prophylactic in between the attacks three to four times a day. In girls the condition is milder. Mast cell inhibitors are almost devoid of any side effect.e. Seasonal allergic conjunctivitis have definite seasonal predilection which lacks in perennial conjunctivitis. The disorder is seen in two age groups i. It has remission and exacerbations. mast cell inhibitor specially long acting Nedocromil 2% two times a day are effective. 35. may be a symmetrical specially when it develops first time. Cornea is never involved. two to three years. (ii) Signs comprise of conjunctival congestion.e. and post puberty. (v) Steroids are best avoided. The symptoms are : (i) Itching. (ii) Instillation of combination of astringent.03% drops three times a day. It is more common in coloured races and in warmer climate. generally not seen in females. dry eye syndrome or angular conjunctivitis. More common age is between six years to sixteen years. It is more common in boys who outnumber girls in ratio of six to one. In milder cases local anti histamine drops in combination with long acting vaso constrictor are sufficient. The allergen in perennial allergic conjunctivitis is present in the environment throughout the year which could be vegetable. Perennial allergic conjunctivitis As the name suggests the condition lingers throughout the year without remission or exacerbation. follicle formation and papillary reaction. There . Some authors consider this as variation of atopic kerato conjunctivitis. (iv) NSAID like ketorolac tromethamine 0. 36 It is most troublesome allergic conjunctivitis in children. It is a self limiting disease lasting for six to ten years. pre puberty which forms the bulk of patients. In the past perennial and seasonal allergic conjunctivitis were grouped under single category of chronic allergic conjunctivitis. vasoconstrictor. food allergen and locally used drugs. 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. (iii) Local mast cell inhibitor – 2% NedoCromil two times a day is better than 4% sodium cromoglycate BD 2% cromoglycate has less therapeutic value.140 PEDIATRIC OPHTHALMOLOGY Management of seasonal allergic conjunctivitis consists of : (i) Oral non sedative antihistamine. antihistamine several times a day. are less severe and last for shorter i. Management consists of removal of offending allergen which is not always possible. Local NSAID.

Besides discomfort itching becomes social embarrassment to the child. The corneal changes in spring catarrh comprise of following. Common in black races. The papillae are not seen in the lower lid or fornices. Itching is associated with redness that becomes more intense following itching. Symptoms. Sometimes more than one sibling may be affected. There is a tenacious ropy discharge which is alkaline in nature. Itching and watering leads to excoriation of the outer canthus. photophobia and diminished vision. In the bulbar form there are mucoid confluent nodular papillae at the limbus. 3. there is a ropy discharge. In between adjacent papillae there are deep furrows that zigzag in between the papillae. 2. Epithelial micro erosion generally seen in the upper part of cornea 1mm inside the limbus. With treatment the papillae shrink in size both in height and width. This is most common. Superficial vascularisation. Atopic dermatitis is a frequent dermatological disorder in children with spring catarrh. 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. 3. . Palpebral (tarsal).DISEASE OF CONJUNCTIVA IN CHILDREN 141 may be a positive history of vernal catarrh in the parents. There may be watering from the eyes. Mixed. Most commonly at the upper part. They are called shield or amaeboid ulcers seen in the upper part of the cornea. they may be present as isolated lesion or in various combination : 1. Topographically there are three types of vernal kerato conjunctivitis : 1. The discharge which fills the gap between the papillae. these nodules are generally seen in segments but may encircle whole of the cornea. The flat topped papillae are polygonal in shape. The skin over the lid may be darker than the surrounding area. Least common but most difficult to treat. The corneal changes in spring catarrh are the cause of lacrimation. There may be pseudo membrane over the papillae (Maxwell Lyons sign)35 in untreated cases. After sometime a pseudo ptosis develops which gives sleepy look to the child. The surface loose its roughness and becomes velvety. 2. Bulbar (limbal). Plaque over the macro erosion. Commonest symptom is intense itching that may last throughout the day. The discharge can be pulled out in form of fine threads. the appearance is called milk spilled over cobble stone as the large papillae have appearance of cobble stone. Epithelial macro erosion—There is large area of epithelial loss leading to sterile ulcer formation due to their shape. 4. 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. This causes early and more corneal involvement is more. In untreated cases there may be formation of Horner-Tranta’s spot35 which are discrete white spots at the limbus.

In under developed countries. Drawback of suspension is that the . acnerosacea. is self limiting and by itself does not produce blindness but blindness can set in by indiscriminate use of steroids that gives prompt relief. 2. Sodium cromoglycate 2%-4% three to four times a day gives comfort to the child. 4. Frequent rubbing of the eye has also been blamed for development of keratoconus. 2.1% may be used above twelve years of age. Nedocromil sodium 2% BD has better effect that 4% sodium cromoglycate. Systemic aspirin. Pseudo arcus. 1. dry eye status. bilateral. Cromoglycates are mast cell inhibitors have almost no side effect. chronic follicular conjunctivitis. Flurbiprophen 0. trachoma. is chronic and requires prolonged treatment. drug induced conjunctivitis. If these fails mast cell inhibitors are added to above treatment. They lack side effects of steroid are well tolerated. nemesulide have been used without uniform result.03% two to three times a day. Local steroids are used as clear solution. Keratoconus has been observed more frequently with vernal kerato conjunctivitis. 38. Sub epithelial scarring.5% two to three times a day. 39 Management of vernal kerato conjunctivitis has special significance as it is seen mostly in children. In spite of all drawbacks local steroids have remained most effective treatment of choice. indomethacin. Differential diagnosis. Parents should be persuaded to use nonspecific astringents vasoconstrictor and antihistamine drops in various combinations as local drops rather prompt relief giving steroids. PEDIATRIC OPHTHALMOLOGY 7. 4. Generally there is no problem in diagnosis of vernal kerato conjunctivitis due to its typical clinical feature of chronic. blepharo conjunctivitis. Parents should be informed that the condition is recurrent. Ibuprofen. 3. Ketorolac tromethamine 0. 6. This is segmental in nature. Diclofenac sodium 0. The exact cause of keratoconus is not understood. Common conditions that mimic as vernal conjunctivitis are—Other type of allergic conjunctivitis. They have reduced use and dependence on steroid. Dark glasses. Frequent cold compress. Management35. may be a genetic feature.142 5. runs a prolonged course. Children with vernal conjunctivitis try to skip school as perpetual redness of eyes and constant itching is not socially acceptable to the children. 3. Drugs used are : 1. clinical features may be overshadowed by infective conjunctivitis and xerophthalmia. 5. papillary reaction in a male child intense periodic itching with remission and exacerbation that responds dramatically to local steroid drops. Chromoglycate sodium in two percent strength can be used as prophylaxis in between exacerbation. Non steroidal anti inflammatory drugs have been used with good result.

On . keratoconus. Commonly used steroids used are Dexamethasone/Betamethasone 0. hyperemia.vision. (iv) Cyclosporine 2% in castor oil may be used in those cases that do not respond to steroids. mostly seen in atopic adults but children in second decade may be affected. NSAID. All children under topical steroid should be under constant supervision of ophthalmologist who should monitor .DISEASE OF CONJUNCTIVA IN CHILDREN 143 suspended particles get lodged in between the papillae and act as irritant. (iii) Tear film substitutes are given to manage ocular surface disorder that is too common. Addition of mast cell inhibitors reduces requirement of steroid without jeopardising result. sodium nedocromil. itching.1% In severe cases any of the above drugs is instilled in conjunctival sac every two hourly for first twenty four hours. (v) Shaving of papillae. rhinitis. papillae formation. Ocular manifestation consist of blepharitis. After a month the child may be comfortable in once a day instillation. (ii) Weak solution of acetic acid to change pH of tear from alkaline to acidic. Atopic kerato conjunctivitis Fortunately this condition is rare. The lens should be examined for iatrogenic cataract. Some cases may require topical cyclosporin in oil two times a day.5% Fluorometholone 0. By 1977 a syndrome consisting of contact lens intolerance. error of refraction. Management consists of local low dose of steroid. Giant papillary conjunctivitis41. antecubital fold. intra ocular pressure and any change in the disc. then frequency is gradually reduced over weeks. may have to be used in severe corneal involvement under strict supervision. (vii) Rarely low dose systemic steroid for a short period. cryo application to the papillae have not found to be very effective. sodium cromoglycate. blurred vision has emerged. 43 The malady was first reported in 1974. It has more systemic symptoms of atopic disease than ocular symptoms. The principle of local steroid therapy is to use weakest solution used least frequently should give maximum relief without side effect. mucus discharge. corneal epithelial defect. These cases are generally associated with atopia. 8.1% Prednisolone—0. 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. 42. (vi) Plano T contact lenses may be helpful in persistent superficial keratitis. Systemic signs consist of eczema in the neck.125% to 0. asthma. corneal plaque and atopic cataract.

Most of the time it is coincidental. Use of preservative free contact lens solution. Typical conjunctival phlycten develops one to two millimetres away from limbus as a pale vescicle that form a nodule later44. Other possibility is that due to infection conjunctival epithelium gets sensitised and a phlycten ensues.144 PEDIATRIC OPHTHALMOLOGY examination large papillae that are larger than 3mm across are seen in upper tarsal conjunctiva. Rarely it can be idiopathic as well. multiple embedded foreign bodies. After ten years frequency becomes less. 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. ascariasis. Besides contact lens giant papillary conjunctivitis is also encountered in protruding mono filament sutures. The initial size may be that of a pin head. Extendedwear lenses produced clinical features. Larger the lens more are the symptoms. proper contact lens hygiene. Management comprises of discontinuation of CL. Local NSAID and cromoglycates give sufficient relief. conjunctival vessels do not pass over the nodule. It is not associated with atopia. coccidomycosis. However there may be crops of lesions one after another. strepto coccus. Tubercular protein is commonest causative factor.45. candida. Common age to develop phlycten is between three to ten years. Phlyctens are self limiting in nature. Other frequent part to be involved is cornea either primarily or due to spread from conjunctiva. It has been observed on the bulbar conjunctiva as well as tarsal conjunctiva and inter marginal strip.e. change of CL polymer and design. They resolve in ten to fifteen days. lympho granuloma. Girls are effected more than boys. Acute infective conjunctivitis may facilitate transfer of large amount of tubercular . Perilimbal conjunctiva is the commonest site to develop phlyctenular conjunctivitis. Phlyctenular kerato conjunctivitis44. Role of infective conjunctivitis in production of phlycten is not clear. only phlycten without muco purulent conjunctivitis or phlycten developing in infective conjunctivitis. It can affect any age both sexes are equally effected . gonococcus. 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. It was the preservative and protein deposits on the lens that were the causative factors. It is a bilateral disease. The conjunctiva round the nodule is congested. Two types of presentation are encountered i. malnutrition is frequent and lack of public health facility common. artificial eye. kerato prosthesis. After fifteen it becomes very rare. cyno acrylate glue. corneal scleral shell. On long run tops of papillae ulcerate and stain with fluorescein. The allergic response is attributed to lipid fraction of tubercular antigen. steroids are best avoided. or separately. The symptoms diminished on discontinuation of contact lens. ocular prosthesis. scleral buckle. Other causative organisms that can produce phlycten are 46 staphylococcus. Severity depends produce more encounter in on size of contact lens. both eyes may be involved simultaneously. more commonly in under developed countries where tuberculosis is endemic. never to recur on the same spot. soon it enlarges to 1 mm to 2 mm. However it was realised that contact lens was not the offending agent.48 This is very common allergic conjunctivitis seen world-wide.

follicles blepharitis. Acute allergic Any Both 2. Contact lens -Dopreservative Atopic kerato conjuctivitis Any Both Atopia -Do- -Do- 6. follicle Antihistamine Mastcellinhibitors -DoMast cell inhibitors Sub acute Itching. 4% sodium cromo glycali. CL—Contact lens. Vernal kerato conjunctivitis Under 20 years Males more 5. corneal involvement. Seasonal allergic.Comparison between various types of allergic conjunctivitises : Sl. MCS—mast cell stabilizer. Anti histamines. hyperemia Mast cellinhibitors antihistamines Mast cell inhibitors Acute Itching watering chemosis Antihistamine weak steroid Mast cellinhibitors Preventive Type Age Sex Allergen On set. NSAID home made contact lens solution AH = Antihistamine. Giant papillary conjunctivitis Any Both Contact lens hygine. Giant papillae upper tarsus.No. Symptoms Treatment 1. Curative Pollen Variable Constantly present Pollen -DoItching.Any conjunctivitis Both 3. itching pseudo ptosis. NDAID Chronic Itching. Contact lens remove contact hygine Do not use lens. 145 . Itching. Mast cell inhibitor steroid cyclosporin Steroids. Perennial allergic -Doconjunctivitis -Do- DISEASE OF CONJUNCTIVA IN CHILDREN 4. ropy discharge-papillae.

An opacity due to marginal phlycten has a typical dome shaped configuration with base at the limbus and convexity towards the pupil. Once it stops shifting it starts healing. Symptoms. This band represents the place of leash of vessels that followed the ulcer. Fascicular ulcer is a typical corneal presentation of phlycten. The superficial vascularisation may develop anywhere on the cornea even all around the cornea and called phlyctenular pannus.146 PEDIATRIC OPHTHALMOLOGY protein to the limbus that results in formation of phlycten. associated bacterial conjunctivitis.e. Symptoms of phlyctenular kerato conjunctivitis depend upon number of phlycten. The vessels develop in this furrow. However if the ulcer is deep it destroys the Bowman’s membrane and a permanent opacity is formed.e. The phlycten sits astride the limbus i. The vascularisation ultimately dies down leaving no scar. Corneal involvement in phlycten can be of following types : Cornea may be involved as (1) Primary lesion (2) The lesion may spread from conjunctiva. The final location of the ulcer where it may become stationary is uncertain. The ulcerative form may be (1) Marginal. Third rare possibility is small pin point superficial ulcers all over the cornea. leaving a superficial opacity followed by fainter band shaped opacity extending upto limbus. Other form of involvement in phlycten is when it develops at the limbus. slight irritation . The vessels are typically straight and do not branch. It is generally preceded by marginal or keratoconjunctival phlycten. There are two main types of corneal involvement i. has a tendency to move towards centre followed by a leash of superficial vessels. There may be single or multiple marginal phlyctens. (2) Fascicular. nor does it produce anterior chamber reaction. Simple conjunctival phlyctens do not produce much symptom except localised redness in the eye. 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. Marginal corneal phlycten is generally due to a phlycten developing at the limbus. and involvement of cornea. half on conjunctiva. (1) Ulcerative (non vasularised) and (2) infiltrative (vascularisation)47. The ulcer starts on the corneal periphery. 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. The fascicular ulcer does not perforate. half on cornea. More than one may coalesce to form a sectorial ulcer. In a more severe case of longer duration there may be deep vascularisation which leaves permanent mark. Like any phlycten in due course it ulcerates and heals with out any scar. Though tuberculosis has been blamed as major cause of phlycten. Corneal involvement is more when tubercular protein is causative factor. Tubercle bacilli have never been isolated from the conjunctiva in phlyctenular keratoconjunctivitis. Infiltrative type of corneal phlycten may produce two types of vascularisation : First more common superficial vascularisation and a less frequent deep vascularisation. It differs from trachomatous pannus in the sense that latter has predilection for upper cornea. The histological changes do not show tubercle formation it resembles more or less of a micro abscess. There may be more than one fascicular ulcer in the same cornea. The fascicular ulcer while shifting from periphery towards pupil digs deep in the substance of cornea creating a shallow furrow.

In case of staphylococcal allergy lid hygiene along with systemic anti staphylococcal antibiotic may be required. photophobia. Associated infective conjunctivitis invariably overshadows simple phlycten that may be missed altogether or it may become obvious when conjunctivitis has subsided. 2. Phlycten with infective conjunctivitis is treated as any catarrhal conjunctivitis along with local steroids under supervision. Management 1. There is no itching either. This reduces symptoms promptly or FML 0. Corneal complications consist of peripheral dome shaped opacity. BCG does not prevent a child from developing phlycten. mostly cutaneous and mucous membrane involvement. Keratitis due to tubercular antigen though produce severe symptoms responds better than in cases of staphylococcal antigen induced keratitis. interstitial keratitis. Erythema multiforme49.e. Children suffering from intestinal helminths should get a course of anti helminth. 50 Though this disorder is classified along with allergic conjunctivitis. It is not a true allergic conjunctivitis. Conjunctival phlycten is devoid of any complication. 51 It differs from both endogenous and exogenous allergic conjunctivitis by not producing . 4. A phlyctenular kerato conjunctivitis produces severe photophobia in children. 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. Symptoms become more severe as cornea gets involved. It has profound systemic manifest.1% every one to two hourly till symptom subsides then the frequency is reduced. Vision is greatly reduced if the ulcer is positioned against the pupil. Complication. Albendazole is an effective drug.DISEASE OF CONJUNCTIVA IN CHILDREN 147 and watering. 5. This is given in form of chewable tablet in single dose. Fascicular ulcer is associated with severe blepharospasm. Salzmann’s nodular dystrophy46. lacrimation and diminished vision. Simple phlycten without infective conjunctivitis—Local weak solution of steroid i. Differential diagnosis of phlycten comprise of nodules at limbus. dexamethasone 0. 3. 6. Most acceptable theory is an auto immune disease.1% 4 hourly for first day. Children above 2 years should get 400 mg single dose to be repeated as required. If the child is found to be tubercular a full course of anti-tubercular treatment should be ensured. It is a disease of children . The exact mechanism is not known. spring catarrh. trachoma. corneal vascularisation and various grades of corneal opacities that may diminish distant vision leading to squint and amblyopia. follicles or nodule. If cornea is involved atropine is added to steroid to reduce ciliary spasm. this gives prompt relief. Presence of itching with nodule at limbus points towards limbal spring catarrh. Though phlycten is an allergic manifestation it does not produce itching.papillae. In under developed countries all children with phlycten should be investigated for both pulmonary as well as extra pulmonary tuberculosis.

Erythema multi form minor may pass off without any complication. Management. Shrinking of conjunctiva leads to trichiasis. keratanisation. The lesions are generally symmetrical. Any part of the mucous membrane may be involved. The lesion soon becomes papular. The follicles are small. The muco cutaneous involvement consists of erythematous macules on dorsal hand and feet. This is not a true conjunctivitis. entropion. bilateral. Vesciculo bullus stage 3. 3. Complications. (ii) Respiratory infection with mycoplasma pneumonae. Infection. Drugs. However Steven’s Johnson has far reaching vision threatening complication like xerosis of conjunctiva and cornea. Due to vasculitis part of the conjunctiva gets infarcted. tarsal conjunctiva is not much involved. seen mostly in lower fornix. It is bilateral. sore throat.148 PEDIATRIC OPHTHALMOLOGY and young adults. There may be vescicle formation. This stage lasts for seven to fifteen days. Shedding of these membranes leave raw areas in the conjunctiva. Healing starts within a few days and heals completely. When two such raw areas come in contact with each other adhesion develop. obliteration of lacrimal ducts. (i) Recurrent infection by herpes simplex type I and II. There is hardly any discharge. The disease has three stages : 1. There is no effective treatment of ocular involvement. generally symmetrical. Local steroid may minimise vasculitis and prevent conjunctival infraction which in turn reduces formation of pseudo membrane. It is a benign. Idiopathic. fever. It is to be differentiated from other causes of follicles and papillae formation . exterior surface of forearm and legs. There are two types i. non-infective follicular hypertrophy in children.e. Cornea is never involved. vascularisation and opacification of cornea. Conjunctivitis of unknown causes in children : 1. Recurrence has been reported if the child is exposed to same precipitating factors. Complication. There is no itching. it can often be fatal. Prodromal 2. Top of the list is taken by sulpha drugs including acetazolamide others are penicillin’s. salicylates. 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. Etiological factors that can culminate into this troublesome disease are : 1. joint pain and swelling. phenylbutazone barbiturates. Folliculosis. Many drugs that are routinely used for day to day management of various symptoms can cause Stevens Johnson syndrome. starts as muco purulent conjunctivitis that may become purulent. In second stage. The pro dromal stage consist of malaise. keratanisation of cornea. That result in formation of symblepharon. The ocular lesions Lesions of the eyes seen in 50% of cases. (1) erythema multiform minor (2) Erythema multi forme major (Stevens Johnson syndrome) The latter is not only bilateral sight threatening. There may be formation of pseudo membrane or true membrane. 2. which become bullus.

There are some follicles which may be associated with itching and confused with non drug induced allergic conjunctivitis. 53 (Oculo sporidiosis) Rhinosporidiosis is caused by fungus Rhinosporidium Seebri. This is rare type of conjunctivitis seen in children may be seen soon after birth or develop any time even in adults. There is no satisfactory treatment. 2. 4. 3. The disease has wide distribution mostly in tropics with moderately to heavy annual rainfall and high humidity. atropine. 3. 7. It is more common in girls.conjunctiva. Almost all mucous membranes of the body are known to be involved including conjunctiva and lacrimal sac. Exact cause of this condition is not known. It is a non specific conjunctivitis following prolonged administration of host of commonly used local drops that include . Ligenous conjunctiva. Ligneous conjunctivitis. lesions of skin and bone have also been reported. 5. Chronic follicular conjunctivitis is an infective conjunctivitis of under nourished children. home atropine. Common cause in children are : 1. idoxuridine. Treatment consists of stopping the offending drug and replacement by nontoxic drug. neomycin. ophthalmia nodosum (caterpillar hair). 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. However chronic infective process and hypersensitivity are two important factors. Rhinosporidiosis52. Coccidomycosis. . is generally bilateral but asymmetrical. Foreign body. Ocular structures involved in rhinosporidiosis are . The spores of the organism get implanted in the mucous membrane and develop into sporangia. Burst chalazia. Conjunctival involvement is generally primary.000 spores. gentamycin sulpha cetamide. 2.DISEASE OF CONJUNCTIVA IN CHILDREN 149 like chronic follicular conjunctivitis. There may be formation of granuloma on the tarsal conjunctiva. It is generally associated with dermatitis of the lid margin and excoriation of the outer angle. The follicles are numerous both upper and lower tarsal conjunctiva are involved. In spite of treatment follicles may persist for few days to weeks. Iatrogenic conjunctivitis (Drug induced). Ocular involvement is seen mostly in children in endemic areas. Most important feature is formation of membrane on the tarsal conjunctiva that gives it a hard feeling. Weak solution of steroid. In secondary rhinosporidiosis of the eye the disease spreads from the nasopharynx to the sac. There may be minimal discharge. 6. The condition does not require any treatment except reassurance to the parents. Initially the condition may be confused with membranous or pseudo membranous conjunctivitis and StevensJohnson syndrome. Candida. Granulomas of conjunctiva Conjunctival granuloma are seen in chronically inflamed conjunctiva. a mature sporangium may contain as many as 16. trachoma and spring catarrh. lacrimal sac and sclera. Combined involvement of sac and conjunctiva is very rare. Tuberculosis. Rhinosporidiosis. This is also known as oculosporidiosis. It is a bilateral condition.pilocarpine. 8.

The growth is . 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.150 PEDIATRIC OPHTHALMOLOGY Typical conjunctival lesion is a gradually developing painless granuloma in the conjunctiva. While those arising from fornices have long thin pedicle with finger like projection. They are fragile. The lesions bleed on slight trauma. The staphyloma is located anterior to the equator. Common lesions that form differential diagnosis are burst chalazion. Sometimes free spores are demonstrated in the tears of affected child.54 is more common than conjunctival involvement. (See page 93-94 as well) Scleral involvement is most rare. The growths in the tarsal conjunctiva are flat and sessile. However reverse is also true i. may spread under the lower lid. papilloma. that presents as a diffuse non tender swelling on the medial side of the eye mostly below the medial palpebral ligament. bleed on slight manipulation like everting the lid. have rough surface dotted with samolina (sujee) like white dots that represent sporangia. The conjunctival lesions can be removed under topical anaesthesia. without any involvement of nasopharynx or as a secondary spread from nose and throat. If needed short term general anaesthesia may be administered. The sac can be involved either as primary lesion i.e. Bilateral involvement is rare. haemangioma. Management. chicken pox or measles are contributory factors in endemic area. Bulbar conjunctiva is rarely involved. Symptoms. Occasionally it is visible on indirect ophthalmoscope. This generally happens following syringing. Bilateral conjunctival involvement is rare.53. The lesion is more common among boys between 5 to 15 years. Partial congenital obstruction of nasolacrimal duct. Regurgitation test is negative. Other useful diagnostic method is to examine small piece of tissue dipped in saline under high power that show sporangia. The lesions are non tender and painless. who have habit of taking bath in a pool shared by cattle which are invariably infected. foreign body granuloma. Temperature over the swelling is not raised. Generally there is only one granuloma in an eye. above conditions may be mistaken as rhinosporidiosis in endemic area. There is no known drug that is effective against rhinosporidiosis. does not change in size on coughing or sneezing. Exact mechanism of sac involvement is not clear specially as a primary lesion. Commonly involved are fornices and tarsal conjunctiva. it may cause lacrimation. Involvement of lacrimal sac in rhinosporidiosis52. those at limbus or bulbar conjunctiva are round. Main symptoms are bleeding in the conjunctiva and visible red mass. Erosion of surrounding bone is common in long standing cases. Diagnosis of conjunctival rhinosporidiosis is simple in endemic areas. complete block of nasolacrimal duct is rare.e. Surgery is the treatment of choice. 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. The swelling is compressible but non-reducible. All the lesions are bright red in colour. The size of the growth varies from few millimetres to few centimetres. If sac is secondarily infected it presents as cellulitis. It is always associated with a conjunctival growth that hangs over the scleral lesion which is in the form of a staphyloma. Once the spore reaches the sac it forms a nidus over which the growths develops. Diagnosis is best confirmed by histopathology of the excised tissue. If the growth hangs over the cornea. The skin over the swelling has orange peel appearance. Diagnosis.

parasitic infection and adhesion between two layers of conjunctiva. They may develop alone in the conjunctiva or may be seen with haemangioma of lids. Dermolipoma. retained conjunctival foreign body. 2. These are fairly common congenital benign tumours. Dermoid. Traumatic cysts are mostly epithelial implantation cyst due to trapping of epithelium under the mucosa. Conjunctival involvement in neuro fibromatosis is less common than in Sturge Weber syndrome. Management of rhinosporidiosis of sac is less satisfactory. traumatic and non-traumatic. congenital anomaly. coenurosis. Intraocular pressure of the eye is kept low either by paracentesis or IV mannitol and maintained low by oral diamox and betablocker drops. Cysts can be grouped into two broad groups i. There are two types of choriostoma : 1.e. Unless meticulous care is taken to remove every bit of tissue involved recurrence is the rule. retention.DISEASE OF CONJUNCTIVA IN CHILDREN 151 grasped with flat. (See page 61-62) Cysts of the conjunctiva Various types of conjunctival cysts are : traumatic. For scleral staphyloma best results are obtained by applying cryo all round the staphyloma. The growth of the sac can also be removed by nasal endoscopic surgery. lymphatic or developmental. They are seen following wounds of conjunctiva. It is essentially complete removal by surgery. Conjunctiva is never involved alone. echynoccosis. While removing the sac the nasal growth should also be taken care of simultaneously. They are generally . In scleral involvement the conjunctival growth is removed by usual method. . choriostomas. These benign tumours are not true tumours. Even without ligature the bleeding can be stopped by firm pressure. The scleral bulge is indented with scleral explant of suitable size. Small growth can be treated with cryo. cysticercosis. Surprisingly there is no recurrence in conjunctiva once the growth has been removed surgically even if the child lives in the same environment. Choriostomas. Haemangiomas of conjunctiva. it is always associated with lid involvement and many have associated systemic involvement. The pedicle is cut with sharp scissors. squint surgery. Common causes of cysts in conjunctiva are : trauma. haemangiomas and xeroderma pigmentosa. Hamartomas that have congenital involvement are : Neuro fibromatosis (von Reckling Hausen’s disease and Enccphalo trigeminal angiomatosis (Sturge Weber Syndrome). They can be parasitic in nature i. blunt forceps and pulled away from the conjunctiva or a ligature may be tied round the pedicle. Tumours of conjunctiva Tumours of conjunctiva in children are rare.e. 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.Hamartomas.

Common parasitic conjunctivitis seen in children are : Cysticercosis. Some of parasitic conjunctivitis are very rare and may not be seen in children. Most of conjunctival lesions are located near the medial canthus. The cyst is painless. Management. Retention cysts These generally develop in glands of Krause following chronic inflammation or trauma. The child may not be aware of small cysts.e. have ocular involvement. Aim should be to remove the cyst without rupture. 59 is a systemic parasitic infection. retention cysts and lymphatic cysts. A large cyst may cause astigmatism or may cause a diffuse swelling in the lid. Humans harbour the parasite in two forms i. (1) Definite host form of adult tapeworm or (2) Intermediate host. Large cysts are removed for cosmetic purpose and to reduce astigmatism. Cysticercosis is caused by Cysticercus cellulose the larval stage of pork worm taenia solium. Latter mode of infection is more common than in former. The ocular involvement can be (1) less vision threatening. extra ocular manifestation in the form of conjunctival or orbital cyst. About forty percent of persons infested by the parasite. It may migrate along the extra ocular muscle . Small cysts do not require any treatment.57 The swellings are of variable size generally fixed to episcleral tissue or the sclera. Other method of infection is consuming food and water contaminated with ova of the parasite. Bilateral and multiple unilateral involvement are rare. Parasites range from protozoa to helminth. However right eye is not immune. Left eye is more frequently effected than right eye. Humans get infected by consuming under cooked pork that contains viable cysticersis which mature in human intestine. 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. Initially the conjunctiva moves freely over the translucent oval swelling. echinococcosis. They may be discovered during routine examination of the eye. coneurosis. This is the mode of infection in strictly vegetarians and those persons who shun pork due to religious reasons. Parasitic conjunctivitis Parasitic infestation of conjunctiva is far less common than bacterial or viral infection. Rarely arthropods may invade the conjunctiva. (See Chapter Ocular Manifestation of Systemic Diseases) Cysticercosis56. There may be multiple translucent cysts. thelaziasis. Symptoms depends on size and position of the cyst. The embryo reaches the eye via ophthalmic artery. and become adult worm. Some of the parasitic conjunctivitis are seen only in endemic areas (river blindness). Parents may become aware of large cysts. Pig is the usual and natural intermediate host. They are generally seen in the upper part of the conjunctiva. onchocerciasis. harbouring larval stage.152 PEDIATRIC OPHTHALMOLOGY Non-traumatic cysts are : Dermoids. parasitic cyst. Parasitic infection of conjunctiva is generally seen in tropical and sub-tropical regions. The ducts of the glands are obstructed resulting in formation of cysts. (2) More severe and blinding intraocular involvement.

Role of antihelminth as cure of conjunctival cyst is not confirmed. Echinococcosis62 Echinococcosis is systemic parasitic infection caused by larva of T. Differential diagnosis consist of other parasitic cyst. Perforation of the retina by cysticareus results a free floating cyst in the vitreous. Prophylaxis. liver and lungs are most frequent sites. However increased eosinophils in differential count and presence of ova in stool is highly suggestive. chemosis of conjunctiva and lid. Prophylaxis consists of proper cleanliness and food hygiene. Human is an intermediate host who gets infected following ingestion of contaminated food. vitreous and anterior chamber. Man and other animals can get infected from affected dogs. Diagnosis is confirmed by histopathology after removal. Adults get infected by ingestion of contaminated food. Rupture causes severe conjunctivitis. 2. The embryo develops in to an expanding cyst in the choroid that lifts the retina to produce exudative retinal detachment. orbit. This consists of regular de-worming of the pet and the child. Humans act rarely as intermediate host which are generally sheep and goats. Coenurosis This is systemic and ocular involvement of an intermediate host by larval stage of dog tapeworm multiceps. The cyst may occasionally extrude through the conjunctiva. Ocular involvement consist of—Cyst in sub-retinal space. Avoiding green raw vegetables. raw vegetables should be avoided least they are contaminated. Intra ocular involvement results when the embryo reaches the interior of the eye via posterior ciliary artery. No part of the body is immune to echinoccosis however. Death of the larvae also causes endophthalmitis severe enough to cause loss of eye. there is no recurrence. The cyst on rupture produces larval endophthalmitis both large intra vitreal cyst and endophthalmitis cause white reflex in pupillary area. Following successful removal. Other parts of the eye that may be invaded by cysticercosis are lids. and anterior chamber. Management consists of prevention of infection in children. Conjunctival cyst formation is less common than seen in cysticercosis. Children get infected directly by ova of the parasite from soil.DISEASE OF CONJUNCTIVA IN CHILDREN 153 from behind to be visible on the conjunctiva and extruded56. Surgical treatment consists of complete removal of cyst without rupture. Surgical removal of the cyst. Definitive treatment is removal of the cyst surgically without rupture. vitreous. retina. Orbital cysts cause proptosis. Echinococci a helminth found in intestine of dogs. If the cyst ruptures it causes localised conjunctivitis. 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. Management consists of 1. Pork should be well cooked. vegetable or fur of dogs deposited in the conjunctival sac and develop cyst similar to cysticercosis. 60. Cyst when present in the conjunctiva may be attached to one of the recti. . Health authorities should see that infected pigs are not slaughtered for consumption.

Thelaziasis63. Duke Elder S. In humans mostly outer eye is involved. London. Diethyl carbamazine 50 mg three times for 14 days may prove effective. Live worm have been removed from anterior chamber.. itching. lid and lacrimal passage. Live worm can be found in the conjunctival sac. The flies lay eggs in the conjunctiva where the eggs hatch as larvae (maggots). REFERENCES 1. Common being thelazia callipaeda and thelazia californiensis. vitreous or orbit. There is no known medical treatment. Henry Kimptom. Associated uveitis is treated by usual local cycloplegic and steroid. . : General consideration in conjunctival diseases in system of ophthalmology. On examination small elongated white larvae are seen crawling in the conjunctival sac. 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. Larval conjunctivitis. Worm from AC should be removed surgically. There may be associated marginal keratitis. The maggot infestation is seen mostly in emaciated children. Ocular myiasis Ocular myiasis or larval conjunctivitis is caused by deposition of eggs of some of the flies.154 PEDIATRIC OPHTHALMOLOGY All the parts of the eye can be infected. 64 Thelaziasis is a nematode infection of ocular tissue mostly in animals. 61 Even common housefly can cause ocular myiasis. Occasionally the bones may be eaten away and meninges exposed. hyperemia and chemosis of conjunctiva. VIII. Vol. The larvae penetrate deep under the conjunctiva and skin into the orbit. Destructive myiasis. Three species of flies have been identified to cause myiasis. 2. burning and lacrimation. In adults it can involve sub retinal space. Unless the larvae are eradicated promptly they may cause death. Attempt should be made to remove the larvae manually as soon as detected. When surgical removal is not possible. There are about ten species of thelazia that have been reported to infect conjunctiva. Unlike other nematodes thelazia does not cause cyst in the ocular tissue. p-4 Edited by Duke Elder S. There is redness. There are two types of conjunctival infection by larvae : 1. In children subconjunctival cyst is most common presentation. Intra ocular involvement have been reported. surgical removal is best option. Use of mosquito net to prevent fly from depositing eggs on the conjunctiva. Parts involved are conjunctiva. Part 1. Involvement of human eye is rare.63 Management of conjunctival involvement is manual removal of worm from conjunctival sac after it has been immobilised by local anaesthetic agent. Management consists of maintaining proper cleanliness in emaciated child. 1965. It is the larvae that are the cause of conjunctival inflammation. The flies are attracted to the conjunctival and nasal discharge of the filthy child. Intraocular penetration may result through an abraded cornea or sclera.

5. Brunham R. Chandler J. Nema H. Goldberg M.. Ophthalmia neonatorum. Jay Pee Brothers. Usha H.. Isada C. I. Khamar B. 12.M. p-38-42 Edited by Dutta L. and Thoft R.. : Ophthalmia neonatorum. 9. Third edition p-119.M. 1047-1048. Gipson : Anatomy of the conjunctiva and cornea in The cornea. American academy of pediatrics committee on drugs : Prophylaxis and Treatment of neonatal gonococcal infection.. Philadelphia 1983. Burd Eileen M. Saunders Company. Philadelphia 1995.S.A. 4. New Eng. W. and Roy F. Edited by Samolin G. Edited by Harley R. Edited by Fraunfelder F.H. p-547-548. : Development of ocular adnexa in Anatomy of the eye and its adnexa..T. Third edition. Fifth edition p-22-24. Edited by Fraunfelder F. 10. Lippincot Williams and Wilkinsons. 7.C. p-532-534. Berger B. 17. WB Saunders Company. Wilson F..B. I..R. 14. 15. : Ophthalmia neonatorum. Lippincot Williams and Wilkins. Philadelphia 1994... First edition. Jay Pee Brothers. 1987.W. Basak S. 1983. I.. 8.C. New Delhi. Saunders Company.K. Vol. Vyas.T. and Mayuri Khamar : Ophthalmia neonatorum in Modern Ophthalmology. . and Usha Vayas : Disease of conjunctiva in Modern Ophthalmology. Saunders Company Philadelphia 2000.B. Vol. p-138-139.A.B.A.. Jay Pee Brothers. Paediatrics 65. Williams and Willkins. 11. Naamara W. M. : Gonococcal ocular disease in Current ocular therapy. New Delhi 2000. : Surgical anatomy of the limbus in Principle and practice of ophthalmology. 1986.V. p-13-17 Edited by Smolin G. New Delhi 1994.T. Fourth edition p-3-4. 3. : Single dose therapy of gonococcal. : Congenital anomalies of cornea and conjunctiva in The Cornea. Edited by Dutta L. Wand M. Philadelphia 1994. : Bacterial keratitis and conjunctivitis in The Cornea. Second edition. 40:715-725. 13. : Diphtheria in Current ocular therapy. : The limbus in Essentials of ophthalmology. Laga M. 6. second edition. Frindly D. Trivedi N. Meisler D. Edition third. Vol. 1999. Edition 2.H. p-19-21.C. 16. O’Hara M. New Delhi 1991. 30:1033-1042. Pediatric clinic of North America. Current Books International. Khamar B.DISEASE OF CONJUNCTIVA IN CHILDREN 155 2... and Thoft R. and Thoft R. Jr.B.R.A. Lippincot.F.. Calcutta. Ilene K.M. p-480-483. and Roy F. W. Fifth edition p-28-31. Philadelphia 2000.H. Pediatric clinic of North America. 1993. Sonders D. Jay Pee Brother. and Roy F. Edited by Smolin G. W. Mayuri Khamar. Edited by Fraunfelder F. First Indian edition Edited by Peyman G.D.. Second edition p-5-6.. 315 : 1382-1385. Owaring : Neonatal corneal opacities in Pediatric Ophthalmology. : Acine bactor : In current ocular therapy. Trivedi N. Laibon P. Philadelphia 1994. 1980.

. p-76-78. Third edition. W.K. Dawson C. 19:205217. Third edition.T. Taylor and Taylor H. 34A. Philadelphia 2000. B. Philadelphia 2000. 1994.H.F. WHO/93. Geneva..P. 26.D. Lippincot. Sadek A. Jones B. 23A. Lane S. WHO : Primary care level management of trachoma. 28. 34. 1984. Whitcher J. : Ocular allergy. 15:369.B. Thylefors B. p-281-282. Tanzania.K. Duke Elder S. 1994. 7:17-18.S.. p-61-63. : The epidemiology of trachoma.T. : Trachoma control : Community health. : System of Ophthalmology. : Allergic conjunctivitis. Edited by Fraunfelder F. 1988.H. : Diagnosis of allergic conjunctivitis. Harcourt Brace and Company Asia Pvt. et al. Oph. Edited by Fraunfelder F. 1994.A.156 PEDIATRIC OPHTHALMOLOGY 18.N.. 22.33. : New hope for trachoma control. 27. 29. 294-301. 1995. VIII Part I. 7:18-20. Edited by Smolin G. 7:28-30. J. : Impact of face washing on trachoma in Kongwa. West S. West S. 28 : 261. London 1965. p-282-292. Philadelphia 1994. WB Saunders Company.T. Friedlacnder M.56. 31.H. 21. Fifth edition.F. Munoz B. Kelly J. and Thoft R. Chlamydia : Keratitis and conjunctivitis in The Cornea. Trachoma S. 1991. Little Brown. Clinic.C. and Roy F. p-341-343. Courtright P. Victoria Fransis : Achieving community support for trachoma control. Trachoma : The forgotten cause of blindness. Fifth edition. 1988. Viswalingam N. Deborah Pavon-Langston.J. : Cornea and external diseases in Manual of ocular diagnosis and therapy. International Oph.. Tylor HR : A simple system for assessment of trachoma and its complications. et al. Edited by Fraunfelder F. 1983.R. Denise Mabey and Mabey D. 19. Fourth edition. : Trachoma in Current ocular therapy.R. Bull WHO 65. Foulks G.. 1931. WB Saunders Company.T.. Munoz B. 32... : Vernal kerato conjunctivitis in Current ocular therapy. International Oph.. Clinic. 477-483. Henry Kimpton. : Trachoma in Current ocular therapy. West S. First Indian edition p-18-19.. Darougas S. Br. Courtright P. Ltd. Okumoto M. Community Eye Health. : Itchy eye in Decision making in ophthalmology. Williams and Wilkins. Jr. 30.. p-71. Geneva WHO/PBL 96. McCallan A. Roy F. Kathryn I. Rev.O. Friedlaender M. 102-1190-1195. World Health Organisation : Future approach to trachoma control. 23.H. 25. 35. 20.75:322-325. Sheppard.R. Vol. Philadelphia 1995. Arch Oph.. . Held K. 24.. 33. : Latrine ownership as a protective factor in inflammatory trachoma in Egypt.. 28. A report of global scientific meeting. Epidemiol.H. Lancet 345:155-158. and Roy H. Community eye health.. Edited by Hunven WAJ and Zivaan J. Donshik P. Douglas S.. Saunders Company. 1988. 1997.

Edited by Fraunfelder F. Vol. Phildelphia 2000.5% solution for treatment of vernal kerato conjunctivitis. 43. Felberg N. : Phlyctenulosis in Current ocular therapy. : Phlyctenular kerato conjunctivitis in Manual of ocular diagnosis and therapy. p-509-572. clinic. and Hampton Roy F. : Giant paipllary conjunctivitis in Contact lens wearer. Oph.M. WB Saunders Company. Edited by Fraunfelder F. 38. Praveena Kher : Rhinosporidiosis of the lacrimal sac. p-145-146. Dohlman C.T. : Phlyctenular kerato conjunctivitis in System of Ophthalmology.. 1988.B. Fourth edition.D.T. Vol.D. p-373-374.. Part one. Michelson J. Current Books International. WB Saunders Company. 37.K. Op. Ross R. 28:303-307. Philadelphia 1983. 1996. Shukla I. 45 : 177-180. and Fraunfelder F. 39. : Giant papillary conjunctivitis with ocular prostheses. Clinic. Deshpande M. Oph.T..B.. Edited by Harley R. Fifth edition. Allen Smith M. Int. Hamptom Roy F. Oph Clinic.W. 1977. 28:290-292.DISEASE OF CONJUNCTIVA IN CHILDREN 157 36. WB Saunders Company.F.. Philadelphia 2000. WB Saunders Company Philadelphia 1995. Allan Smith M. : Stevens-Johson Syndrome in Pediatric ophthalmology... p-105. : Vernal kerato conjunctivitis. 48. : Erythem multiformis major in Current ocular therapy.T. Buckley R. 309-316. second edition p-1178. Little Brown. 45. 44. : Phlyctenulosis in Current ocular therapy.R. Int. Harper and Row. 1988. W. 46. Dutta L. 28. Allen Smith M. 47.R. . AJO 83:697708. Saunders Company. 53. Third edition. Calcutta. Deborah Pavan Langston and Foulks G.R. : Giant papillary conjunctivitis.B.C. Philadelphia 1986. Bajarat A. Tabbara K. Srinivasan B..T. 42. Edited by Fraunfelder F. Mukherjee PK : Rhinosporidiosis in Current ocular therapy. p-35.. Gupta R. : Vernal kerato conjunctivitis. Int. First edition.D.M.. 51. Fourth edition p-476-477. Jr. 1997.N. Edited by Fraunfelder F. 50. Ind.M.. Vol. Ram J. 40. Duke Elder S. 1982. Sharma A. 301 : 513-514. Wagoner M.. Fraunfelder F. 85. Hennawi M. Fifth edition. : Topical ketrolac 0. Clinic. Phlyctenular kerato conjunctivitis in Clinical ophthalmology. : Vernal kerato conjunctivitis in current ocular therapy.. Colby K. 1979. and Gupta A. 36:15-20.M. p-66-67. Fifth edition. W. Oph. Philadelphia 2000. Christiansen Sandra : Evaluation and treatment of the allergic patient. 41. Ind. VIII.T. Philadelphia 1995. 2. London 1965. : Erythema multiformis major in Ophthalmology principle and practice. 97 : 892-895. 1995.Ph. Henry Kimpton. 52. IV 8:1-6. Arch Oph.. Edited by Fraunfelder F. 49. Mukherjee P. Int. and Hamptom Roy F.. and Hampton Roy F. p-461-471.T. Vol. Saunders Company. and Hampton Roy F. Jr. 1988.

Int. Op. Ind. Fifth edition. 64. Topilov H. W. W..D.F. Bansal R. Br. Fifth edition.K. Jr.. 1975. 62. Gupta A. 55. W. Edited by Fraunfelder F. 565-568. Agrawal S.. .N.. Sushma Verma : Primary rhinosporidiosis of the eye. : Subconjunctival twin cysticercosis. Lamba P. : Rhinosporidiosis granuloma of conjunctiva with scleral ectasia. J. Mukherjee P. Oph.B. Ind. Saunders and Company..B. 66. : Systemic protozoan and metazoan diseases in Pediatric ophthalmology. Edited by Fraunfelder F. Philadelphia 1983. Sushma Verma. Oph. 25 : 41-42. Echinococcsis : In Current ocular therapy. 864 : 1982. : Intraocular thelazia—A case report. Philadelphia 2000. Raina U. Am Jr. Philadelphia 2000. Vol. p-92-94. II. Chaudhari S.K. Ganapathy M. 60.. Harley R. Saunders Company. 1968 59. Fifth edition. Saunders Company. p-95-96. Congress of Ophthalmology. 23 : 2829.. Mukherjee PK : Thelaziasis in Current ocular therapy.T. Oph. 56.158 PEDIATRIC OPHTHALMOLOGY 54. 54. Lerners S. p-100-101 Edited by Fraunfelder F. 57. Shukla IM.. Philadelphia 2000. Taneja S.K. p-1168. Mukherjee P. : Spontaneous extrusion of extra ocular Cysticercus cyst. 1970. 2. Lamba P.B.K. and Hampton Roy F. W. Second edition. : Cysticercosis in current ocular therapy. : AmJr. 1978.A.K.A..R. Malik S.K. Oph 124 : 438-441. ACTA XXVI Vol.A.B.H. Agrawal S. and Hampton Roy F. and Hamptom Roy F. Mukherjee P. Jr. Shukla K.K.. p-931-937. Saunders Company.. 1996.W. 63. 61.T.

Corneal thickness is 0. 3. Corneal diameter is not equal in horizontal and vertical meridian. Posterior corneal surface is more curved than the anterior surface. 4. Cornea occupies anterior 1. 7.0 mm that is called optical zone and is almost spherical. The epithelium acts as effective barrier to 159 2. Stroma 5.7 mm is called megalocornea while smaller than 10 mm is called microcornea. it increases to 0. Cornea forms roughly one third of the outer coat. The cornea comprises of following distinct anatomical layers6. Corneal layers. Had the curvature been equal on both surfaces. Endothelium 1.3 sq. Descemet’s membrane . Horizontal diameter is longer i.52 mm in the centre. Cornea of a new born is slightly hazy that clears within few days.e. The bulge is 2.cm5 of the eyeball. Its main functions are optical and protective. The epithelium is relatively impermeable to water soluble substances.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 cornea of a new born looks larger because of narrow interpalpebral fissure. In new born cornea is flatter than in adult and its curvature is more in the periphery. This makes cornea thinnest at the centre and thicker at the periphery.6 mm. 11. Mean corneal diameter at birth is 10 mm. Though on casual look cornea seems circular. The anterior epithelial surface has microvilli that anchor the tear film to the corneal surface. 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.7 : 1. the corneal thickness would have been uniform throughout its length and width.e. 2. The corneal epithelium is continuous with conjunctival epithelium.70 mm in the periphery. Epithelium 3. As cornea is more curved i.CHAPTER 7 Disorders of Cornea in Children Anatomy of cornea1. in fact it is not so. The epithelium. it increases rapidly to reach almost adult size by the end of the first year.e. It sits over the anterior scleral foramen like shining crystal clear watch glass with convexity anteriorly.7 mm from the plane of the limbus.6 mm than vertical diameter i. Bowman’s membrane 4. The central 4. Corneal diameter more than 12. making it easy for infection to travel from conjunctiva to cornea. It is transparent. Cornea is the anterior most part of the outer coat of the eyeball. Corneal curvature is not equal on both the surfaces. The corneal surface gradually flattens on the periphery in adults. Central corneal thinness makes cornea more prone for perforation than periphery. 10. This difference of one milliter makes the cornea horizontally oval.

380 nm to 760 nm. 1. The energy to the cornea is supplied by metabolism of glucose in the form of adenosine triphosphate (A. 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. touch temperature and pressure are converted to pain sensation. It is continuous with endothelium of the iris. It is formed by condensation of anterior layer of stroma. 4. Optical. The Bowman’s membrane is not a true membrane. The aqueous supplies nutrition to the endothelium. There are three metabolic pathways . diptheria. Both these are met with due to a smooth epithelial surface.). The corneal epithelium can be rubbed or pealed off with ease. 14 Cornea is highly metabolic tissues. Damage to endothelium leads to edema of the cornea. keeping it transparent and bright. It requires constant supply of oxygen and energy. 14 The two functions of the cornea are—1. It is one millimetre shorter than rest of the cornea in all directions. toxin and dystrophy. relative dehydration. the oxygen permeates through the vessels of tarsal conjunctiva. 3. Protective. 12.P. The nerve supply of the cornea is by trigeminal nerve via ciliary nerve. The corneal epithelium is most mitotically active. 5. The nerve fibers enter the stroma from sclera. The damage can be brought about by trauma. The stroma forms 90% bulk of the cornea and is continuous with the sclera.e. Corneal endothelium is single layered structure that is metabolically very active. The epithelium can metabolise glucose both aerobically and anaerobically. Most of the metabolism takes place in the epithelium and endothelium. Thus most of the micro-organism can not invade the cornea so long as the epithelium is intact. Bowman’s membrane is easily destroyed and does not degenerate. meningococcus and C. The cornea is very sensitive to pain. 12. thus healing of the epithelium is very fast.(2) from the perilimbal blood vessels (3) from the tarsal conjunctival vessels when the lids are closed.T. Nutrition of Cornea11. The oxygen is derived (1) mainly from the atmospheric air dissolved in tear film. Other nutrients are supplied by the aqueous from behind. and conjunctiva. It is tough and can withstand considerable amount of raised intraocular pressure and offers resistance to infection. The Descemets membrane is the basal membrane of corneal endothelium.160 PEDIATRIC OPHTHALMOLOGY many micro-organism. 13. inflamation. The organisms that can bypass the epithelial barrier are gonococcus. For light to travel uninterrupted with out scatter.e. Its thickness increases with age. all sensation i. constant supply of nutrient. the cornea must have (a) An optically regular surface and (b) A transparent medium. Physiology of The Cornea11. Optical. 2. During sleep. and 2. The shortness of the Bowman’s membrane is because its development is completed before full development of rest of the cornea. The cornea transmit cent percent of white light i. The endothelium maintains deturgescence of the cornea. 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.

The single layered endothelium is arranged in a regular fashion so are the stromal collagen fibrils. Deturgscence of cornea 3. The epithelial cells which are exposed to atmosphere for long time remain non keratanised in normal eye. This is the stage when corneo scleral junction is also well defined. They are 1. Accumulation of fluid causes the stroma to lose its transparency. The Bowman’s membrane is recognisable at 100 mm (4 months). The epithelium is ten times thicker than endothelium yet metabolic requirement of endothelium is more than epithelium. Common congenital anomalies of cornea are : Microcornea Megalocornea Cornea plana . 10. The normal cornea should remain in a state of relative dehydration.DISORDERS OF CORNEA IN CHILDREN 161 involved in metabolism of cornea. Endothelium cells are first to be differentiated at 12 mm (5 weeks) stage. It helps to maintain the structural integrity of the globe. Anatomical 2. The Descemet’s membrane is visible at 12 weeks. Corneal transparency depends upon following factors : 1. need not be symmetrical in bilateral cases. The anterior surface has more important role as refractive surface than posterior surface. Intraocular Pressure . The tear film obliterates whatever irregularity the epithelial surfaces may have in normal eye to give a better optical surface. The former gives rise to corneal epithelium while rest of cornea is mesodermal in origin. The anatomical factors are : Absence of vessels in the centre of the cornea. 8. As the lens vescicle separates from the surface ectoderm a space develops. Intraocular pressure 4. Development of cornea6.38. 9. Loss of endothelial cells is result of embibement of fluid in stroma and epithelial edema. There are no pigments in the cornea. Uniform refractive index of various layers. Refractive index. The cornea is most important refractive surface of the eye. Glycolysis 65% (2) Kerb-cycle (3) Hexose monophosphate shunt. the epithelium is multi-layered but the cells are arranged in a regular fashion. Deturgscence of cornea.Acute rise of intraocular pressure causes derangement of fluid transport across the stroma and causes stromal edema and loss of transparency. Congenital anomalies of the cornea. Cornea develops from two sources. They may be unilateral or bilateral. This space is invaded by mesoderm that gives rise to all corneal layers except the epithelium. The refractive index of cornea is 1. The cornea protects the eye from most of organism and cuts off ultra violet rays as well. Congenital anomalies of the cornea may be localised to the cornea only or associated with other anomalies of the globe. surface ectoderm and mesoderm. 7. 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.

Astigmatism with the rule 5. In about one fifth of cases there may be glaucoma due to anomalies of the angle. 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. Anterior chamber is deep. No disc change 10. hence the term anterior microphthalmia is also used to denote the same condition. 16. Slight functional defect Buphthalmos 35% cases are unilateral.e. Two common complications met with the condition are glaucoma and cataract. 9. It should not be confused with another congenital anomaly of the globe i. condition is bilateral. As the diameter is more there is increase in circumference of the cornea.28. Sometimes it is difficult to differentiate between pure micro-cornea and microphthalmos especially if there are other associated anomalies of the globe like coloboma. Cornea clear and bright 7. Corneal curvature is generally normal but may be increased with myopic astigmatism. 17. Term microcornea is used to denote a condition where the corneal diameter is less than 10 mm in otherwise normal eye. The recti are inserted more anteriorly than normal eyes. The corneal diameter varies from 13 mm to 16 mm. which is “astigmatism with the rule”. Angle-normal or may have minor congenital anomalies. mesodermal tissue in angle of anterior chamber. Tension-Normal 6. There may be both phacodonesis as well as iridodonesis. All microphthalmos eyes have small cornea but all eyes with micro cornea need not be microphthalmic. Corneal curvature is flat. In bilateral cases involvement may not be symmetrical in two eyes. nanophtalmos where the eye is small in all meridian but is normal in function. resulting into hypermetropia. Megalocornea. Main pathology is gross malformed angle. Bilateral. symmetrical 2. The lens may be large. progressive vision threatening buphthalmos and keratoglobus that is almost symptomless. Megalocornea 1. In absence of myopia vision is unaffected. Progressive Boys to girls—ratio is 5 : 3 Astigmatism against the rule. . No lacrimation 8. The condition must be differentiated from more serious. Tension raised Cornea cloudy due to rupture in Descemets membrane Lacrimation prominent feature.162 Keratoglobus Keratoconus26.29 Anterior embryotoxon Sclero cornea Corneal dystrophies PEDIATRIC OPHTHALMOLOGY Microcornea5.27. This condition is rarer than microcornea and microphthalmos. Exclusively seen in boys 4. microphakia. non progressive where anterior segment of the eye is larger than normal. Cupping prominent Gross functional defect. Non progressive 3. In these cases whole of the anterior segment is small.

23. The sclera may also be thinned. Ehler-Danols syndrome. retinitis pigmentosa.atopic dermatitis. then either the progress comes to standstill or is very slow. Some authors classify it as congenital disorder presuming that there is failure of mesoderm from periphery to centre. Anterior chamber is shallow. It should also be differentiated from pellucid degeneration14.27 Keratoconus is non hereditary in 95% of cases. microphthalmos. anterior polar cataract. One eye is generally more involved than the other. ectopia lentis. atrophic bulbae and nanophtalmos. Differential diagnosis consists of micro-cornea.15 of cornea that is generally seen after 30 years of age and has a toric bulge in the lower part of the cornea. Generally puberty heralds a year or two of rapid progression. anirida. Keratoconus generally presents as isolated ocular disorder. some ocular disorders are more frequently associated with keratoconus than others. In rare instance the disease has been described as unilateral. There may be diffuse opacities in the cornea. The eyes are generally hypermetropic. however. Keratoconus (Conical cornea)18. Due to flattening of cornea there is impression of ptosis. phthisis. Tension and angle remain normal throughout. In this rare condition corneal curvature is reduced. Keratoglobus. Differential diagnosis include all cases of enlarged cornea and corneal causes of deep anterior chamber. Others have put keratoconus under degeneration27 while most put it under dystrophy. resulting into weakness. Causes of poor vision is curvature hypermetropia and corneal opacity. In keratoglobus the cornea is thinner than normal almost throughout. Sometimes cornea itself is small. mitral valve prolapse. Systemic conditions that are frequently associated . Sclera may encroach over the cornea giving an impression of small cornea.7 Increase in keratoconus has been noted up to fourth and fifth decade. There may be associated glaucoma. no race is immune. Keratoconus has world-wide distribution. Proponents of theory of congenital anomaly feel that subtle changes are present years before the disorder becomes manifest. 22. The cornea becomes globular in contrast to keratoconus where cornea is conical. Repeated rubbing of eye has also been thought to be a contributory factor. In extreme cases cornea may be flat generally associated with decreased scleral curvature. 24. In remaining it can either be autosomal dominant or recessive. The disease is said to be more common in girls. Down syndrome Marfan’s syndrome. Other members of the family may show keratoconus or irregular myopic astigmatism. Keratoconus is a non inflammatory ectasia of central cornea due to obscure causes. 21. Though contact lenses are prescribed as treatment of keratoconus they may occasionally worsen it. The condition is stationary and symptomless cases are detected on routine examination. 20. . The disease is bilateral and progressive. neurofibromatosis. 25. 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 symptoms start round ten years and progress for eight to ten years. in such cases the other eye shows irregular myopic astigmatism without corneal thinning and ectasia. It is worth noting that most of the pathological changes are seen in corneal structure that are mesodermal in origin. These are—Spring catarrah. blue sclera. Keratoconus is suspected to be due to a kerato softening enzyme14.DISORDERS OF CORNEA IN CHILDREN 163 Cornea plana. Lebers familial amaurosis. This is a relatively rare bilateral condition where corneal diameter is normal but the corneal curvature is increased.

surrounded by a dark rim and then a pink ring up to periphery. glare. Corneal mapping through photo keratoscope detect very early and subtle changes.164 PEDIATRIC OPHTHALMOLOGY Clinical features consist of diminished distant vision which is progressive.5 mm to 5 mm. The retinoscopy shows scissors movements and the axis is generally oblique. In case of keratoconus. Klein keratoscope. Computerised corneal topography is more informative than keratometery. it leaves some degree of stromal scarring. Vary according to severity of the case : In early stages the corneal bulge is minimal and can not be detected on routine examination. In keratoconus this may increase to 4. In 50% of eyes an incomplete yellow green ring is visible at the base of the cone. however. On keratoscopy and keratometry the findings are exaggerated. the apex of the cornea produces a notch in the lower lid (Munson’s sign). Slit lamp biomicroscopy shows fine vertical lines at the level of deep stroma and Descemet’s membrane. it is shifted downwards and medially. On slit lamp biomicroscopy with a bright narrow beam. the opposite limbus lightsup in a circular fashion. The stage of corneal hydrop lasts for 6-10 weeks and clears with healing of the rupture. uniocular diplopia. Generally the distance of corneal vertex to plane of limbus is 2. Signs. The intermediate dark rim represents the base of the cone where there is near total internal reflection. Retinoscopy with plane mirror shows a central dark spot. In all sporadic cases of increasing myopic astigmatism. leading to photophobia and lacrimation. Sometimes astigmatism is irregular which is confirmed by Placido disc. This is due to deposition of hemosiderin superficial to Bowman’s membrane. Keratometer may fail to measure the cone fully and may require additional plus lenses in the keratometer to measure the curvature completely. and keratometery. if a narrow beam of light is thrown at the corneoscleral junction from lateral side. Retinoscopy reveals compound myopic astigmatism with oblique axis. surrounded by a zone of pink retinoscopic glow. the central part looks thin while peripheral cornea has uniform. the cornea looks conical on profile with deep anterior chamber. the lines disappear on pressure over the globe. In normal cornea. the light reflex on the other side is conical. photophobia. keratoconus should always be kept in mind. corresponding to apex of the cone. lacrimation and rapid fall of vision. The whole of the cornea becomes opaque. Sometimes prominent corneal nerves are also visible. The apex may show some opacity. Sometimes there may be rupture in Bowman’s membrane as well. . Hydrops of keratoconus denotes acute edema of the cornea due to rupture of Descemet’s membrane resulting into imbibement of aqueous through endothelium. The apex of the cone is not opposite the centre of pupil. Late cases do not present any difficulty in diagnosis.5 mm.28 If the patient is asked to look down. On oblique illumination. In advanced cases there may be rupture in Descemet’s membrane. associated with pain. normal thickness.

size. Common corneal disorders seen in children are : 1. Local instillation of hypertonic solutions. Dystrophies Malignancies of cornea are not known. lamelar keratoplasty and epikeratoplasty have not proved better than penetrating keratoplasty. however a growth of conjunctiva at limbus may invade the cornea. Generally steroids are not required. which is a definitive surgical treatment for keratoconus. sometimes contact lenses have been reported to worsen keratoconus. Diminished sensation 4. In moderate cases where spectacles do not give satisfactory visual improvement contact lenses are prescribed initially. Penetrating keratoplasty with a large donar button have very good prognosis. Congenital anomalies 3. patching or use of a bandage lens may reduce discomfort. Infection 5. it is otherwise very rare. thickness 2. Loss of transparency—Opacity. Management of keratoconus29 1. Initially fairly good vision is attained by prescription of spectacles which require frequent changes. Common Corneal Disorders Seen in Children Children are prone to suffer from various corneal disorders. surface. Trauma 4. 2. 3. 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. Some Pathological Signs of Corneal Disorder Seen in Children are Disorders of cornea produce following changes in cornea : 1. deposits. Morphology—Shape. Ulceration—its sequel and complication. edema. Inflammation 6. Degeneration 7. Nutritional 7. Other cause extensive visual damage that may be permanent even with best treatment. curvature. Xerosis 6. Acute hydrops may require lowering of tension by systemic acetazolamide and betablocker. Local non-steroidal anti-inflammation drugs along with cycloplegic give relief from pain and photophobia. 5. induced astigmatism may require correction by contact lens. Thermo keratoplasty. Dystrophies 2. Degeneration .DISORDERS OF CORNEA IN CHILDREN 165 Though perforation in high degree of keratoconus has been reported. 3. Some are very mild and may not reduce vision. however. Many of the corneal disorders are preventable. Generally such growths are seen only in adults except the limbal dermoid that may be present at birth or may manifest later. However. vascularisation. Some may reduce vision marginally that pass off with treatment.

Corneal surface. 2. There may be an apparent change in corneal shape with encroachment of dermoid or limbal papillae of spring catarrah. the curvature may be increased irregularly. megalocornea and keratoglobus. 3. The cornea becomes horizontally oval in soft eyes i. foreign bodies. band keratopathy. bulbus keratopathy. pterygium.e. Rarely the cornea may be oval vertically or horizontally as a congenital anomaly. adherent leucoma. corneal staphyloma. Size of cornea : 1. Corneal curvature is increased in—Keratoconus. keratometer and photo keratoscope. Myopic eyes have larger cornea than hypermetropic eyes. Cornea looks circular on casual look. Normal cornea has a smooth. A cornea smaller than 10 mm is called microcornea. Large cornea. corneal curvature can be appreciated by 1. Curvature. Asking the patient to look down. corneal scar. phthisis. In case of partial corneal staphyloma. while those larger than 13 mm are known as megalocornea. deposits on cornea i. keratoconus. growth at the limbus (Dermoid). Other causes are—Keratectasia. Curvature of cornea—In normal life. keratometer. In fact the measurement in children should be taken under general anaesthesia along with intraocular tension. Small corneas are seen as congenital anomalies like microcornea. 3. Other causes of small cornea are perforated globe. Small cornea.166 Morphological changes in cornea : PEDIATRIC OPHTHALMOLOGY Shape. the apex of the cornea causes a notch in the middle of the lower lid when patient looks down (Munson’s sign). Looking at the depth of AC. computerised corneal topography. shinning surface that acts as a convex mirror forming miniature. Accurate measurement of cornea is done by—keratoscope. phthisis bulbae and atrophic bulbae. it is elliptical in shape when seen from front. corneal plaque. Causes of irregular corneal surface are—Corneal ulcer. In pellucid degeneration of cornea—there is a localised bulge in the lower part. In case of increased curvature. In micro-cornea it may be as small as 3-4 mm. . microphthalmos and nanophtalmos. Looking from temporal side when the patient looks at a distant object.e. Its vertical diameter is less than horizontal diameter. 2. It develops in third decade. virtual erect image of the object in front of the cornea without any distortion or haze. Cornea larger than 13 mm is seen in buphthalmos. Cornea with normal curvature does not produce any localised dent in the lower lid. keratoplasty. however. buphthalmos and keratoglobus. Irregularity of the corneal surface can be observed by window reflex keratoscope. perforated globe due to pressure of lids. In extreme degree of softness it becomes quadrilateral. In older children local anaesthesia is sufficient. The difference becomes obvious when measured by a corneal calliper.

Epithelial edema. growth of conjunctiva or conjunctival tumour spreading over cornea. and stromal edema. Cornea is not uniform in its thickness. their uniform arrangement and compactness. epithelial down growth. Vascularisation of cornea both superficial and deep Changes in corneal thickness. incarceration of uvea. Corneal edema. Transparent corneal nerves and a 5. number and depth varies according to causative factors and duration. Loss of transparency of cornea is called corneal opacity that can be unilateral or bilateral. 3.2. infection or toxin has more serious effect than epithelial damage. inflammation or infiltration. infection.5 They are : 1. It is thicker at the periphery and thinnest in the centre. size. Deposits on the cornea 4. Changes in Corneal Transparency Main function of cornea is optical. it causes localised swelling of epithelium and is transient causing diminished vision for short time. Other causes are superficial and deep vascularisation. Derangement of any of the above factor alone or in combination can cause loss of corneal transparency that may be short lived or permanent. corneal ulcer. 3. loss of corneal epithelium i. Desmetocele corneal ulcer. Uniform size of stromal cells. 2. Reduced thickness is seen in keratoconus. Virtual absence of vascularisation. 2. Shape. 2. Increase in corneal thickness is seen in : 1. Though endothelium and epithelium both contribute in maintaining corneal deturgescence. Uniform and regular arrangement of epithelial cells. dystrophies. deposits on the cornea. The epithelium regenerates quickly thus damage to epithelium is short. due to trauma. Injury to endothelium by trauma. degeneration.e. 4. 5.DISORDERS OF CORNEA IN CHILDREN 167 Corneal brightness is diminished in : 1. Deturgescence of cornea is a state of relative hydration of corneal tissue that is maintained by Na+ – K+ cell pump of endothelium and epithelium. former has more pronounced effect than the later. Constant state of deturgescence. raised intraocular tension. It is more marked if endothelium is damaged than epithelium. Clinically thickness is measured by pachymeter. buphthalmos. Commonest cause of loss of transparency is replacement of transparent keratocytes by irregularly arranged opaque fibrocyte. Corneal opacities 3. Some . For this it has to remain in a state of utmost transparency that is maintained by multiple factors. Integrity of epithelial cells depend on (1) Tear film and (2) Available oxygen. The other causes of increased corneal thickness is congenital hereditary endothelial dystrophy. trauma.

corneal dystrophies. phlyctenular keratitis. number or depth. stroma or may involve both. Causes of bilateral corneal opacities in children (on the basis of age of onset) : 1. band keratopathy. Some of the opacities may increase in size. Corneal edema may be localised in epithelium. cornea must remain in a state of relative hydration. Epithelial edema presents as diminished vision specially on awakening. The congenital causes of corneal opacity includes—sclerocornea. Five year and above — — — — — Congenital Ill managed ophthalmia neonatorum Xerophthalmia. Neonate 3. trauma. malnutrition. The child may remain permanently visually challenged. congenital glaucoma. In well formed bullae blinking causes foreign body sensation that becomes more marked if the bullae ruptures. First year 4. bacterial corneal ulcer. New born 2. Rarely corneal opacities can be congenital. allergy. The corneal epithelium and endothelium regulate passage of fluid and ions. infection. mucopolysccharidosis and mucolipidosis. Birth trauma 3.168 PEDIATRIC OPHTHALMOLOGY of the opacities are permanent. 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. inflammation. Deranged fluid transport across the cornea causes corneal edema. Causes of Corneal Opacities in Children Corneal opacities in children can be caused by any of the following separately or in combination—Congenital. Two to five years 5. interstitial keratitis. Peripheral opacities with clear pupillary area generally have only cosmetic effect. Congenital corneal opacities : Cornea of new born is mildly hazy. Central corneal opacities cause more visual loss. xerophthalmia. Infantile glaucoma. Accumulation of fluid in cornea causes it to swell up. 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. The haze gradually disappears in next two to three days. There may be . other may disappear with treatment or spontaneously. Intrauterine infection 2. If cloudiness persists following causes should be excluded : 1. Corneal opacities in children have far reaching consequences not only regarding vision but also mental development. trauma. Corneal Edema To maintain optical clarity. interstitial keratitis Trauma. congenital glaucoma Xerophthalmia. bacterial corneal ulcer. limbal dermoid. spring catarrah. Thus corneal opacities are one of the most common but neglected cause of amblyopia and squint when unilateral and nystagmus when bilateral. keratoconus. degeneration and dystrophies.

allergy and degeneration. Superficial—mostly epithelial 3. Presence of corneal vascularisation is always pathological. Growth of vessels in cornea is blocked by compactness of corneal tissue and anti vascular chemical factors present in the cornea. Iris incarceration 11. 2. vessels start growing into the substance of cornea both in epithelium and stroma. hypo vitaminosis B. 1. Avascularity of cornea plays a very important role in keeping cornea bright and transparent. Herpctic disciform keratitis 7. cornea. For a sharp retinal image all factors remaining within normal limits cornea must remain transparent. redness and photophobia. chemical burn. 3. Factors that encourage corneal vascularisation are—trauma. Vessels once formed in cornea never disappear. The anti vascular factor may be overwhelmed by a vasoformative factor in newly formed vessels. 30. Common causes of corneal edema are : 1. 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.e. 4. 31 Out of all refractive media i. aqueous. 5. It results in haziness of stroma and thickening of cornea. Aphakia (a) Due to vitreous in AC (b) Toxicity of irrigating fluid (c) Trauma during manipulation of IOL (d) Silicone oil in AC. cornea has maximum refractive power. hypoxia. persistent raised tension. Chronic uveitis 9. Corneal vascularisation can be put into three groups depending upon the layer at which they develop i. rupture of Descemet’s membrane. penetrating keratoplasty.DISORDERS OF CORNEA IN CHILDREN 169 associated pain. Acute rise of tension. Corneal staphyloma (Anterior staphyloma). they may get obliterated leaving tell a tale sign of ghost vessels which are more prominent in deep vascularisation. placement of glaucoma valve. Once the compactness of cornea is lost. lens and vitreous. infection. inflammation. Trauma to epithelium—Ill fitting contact lens. Corneal neovascularisation15. Keratocele (Descemetocele) 10. Interstitial—mostly stromal . Keratoconus—acute hydrops 6. Trauma to endothelium—accidental or surgical during IOL implant. Stromal edema causes less loss of vision and less symptoms. Retro corneal 2. Fuchs dystrophy 8.e. Ruptured bullae may result into anterior chamber reaction and formation of sterile hypopyon or may lead to bacterial corneal ulcer.

There are three clinical stages of superficial corneal vascularisation i. They have very characteristic feature : 1.e. other things remaining the same. Rarely there may be associated superficial vascularisation at the level of epithelium giving an epaulet appearance to the peripheral cornea. Cellular infiltration is followed by development of tufts of new vessels. trauma etc. The vessel seem to disappear at limbus. Micropannus30 extend only one to two millimeter beyond the normal limbal arcade. It is seen in trachoma. herpetickeratitis. This is due to extension of conjunctival vessels into the cornea in front of Bowman’s membrane. 9. 2. Micro pannus. It can be sectorial or may involve whole of corneal periphery. rosaceakeratitis. 4. Pannus is a fibro vascular proliferation that extend up to the cornea. it is essentially a defensive process against infection. Gross pannus. When severe. In the progressive stage. they give a pink hue to the cornea known as Hutchinson’s salmon patch. (2) Stationary stage. They are generally sectorial in distribution. and uveitis. They are generally straight and parallel to each other. 5. atopickerato conjunctivitis. Branch at an acute angle from the main branch giving a bottlebrush appearance. riboflavin deficiency. chronic blepharitis. Gross pannus extend more than 2 mm beyond normal limbal vessels. 7. It may destroy the Bowman’s membrane causing corneal haze. 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. 8. severe blepharoconjunctivitis. blood vessels grow towards the centre of the cornea. phlyctenulosis. corneal graft rejection. According to its extent in the cornea it is divided into 1. . They may be parallel to each other. toxin. It is always preceded by infiltration of corneal epithelium by polymorphonuclear and mononuclear cells. They do not anastomose with each other. In regressive stage the zone of infiltration is between the tip of the blood vessels and the limbus. contact lens wearer. 6. (3) Regressive stage. Milder form of chlamydia kerato conjunctivitis. 3. (1) Progressive stage.170 PEDIATRIC OPHTHALMOLOGY Superficial vascularisation of cornea is commonest form of vascularisation. Deep vascularisation of cornea In this condition anterior ciliary blood vessels invade the cornea at the level of stroma. It is most commonly seen is contact lens wearer. Superficial vascularisation can be seen as—micropannus or as gross pannus. vernal catarrh. They are always associated with deep keratitis. A zone of cellular infiltration extends beyond the tip of the vessel terminal. 2. The vessels grow in the particular plane and remain in the same plane.

number and position. Opaque lines that represent healed breaks in Descemet’s membrane. 2. An intact corneal sensation is essential for health of cornea specially the epithelium. Recent use of local anaesthetic agent. Other causes are prolonged uveitis with hypotony.e. When the inflammation is under control the vessels obliterate leaving a track of ghost vessels that last for rest of the life. 2. diabetic neovascularisation or thrombosis of retinal vein. Keratic precipitate (see diseases of uvea) Loss of corneal sensation. Inter corneal vascularisation. tuberculosis. The cornea stretches either due to thinning as seen in keratoconus and buphthalmos. Rupture in Descemet’s membrane due to trauma are generally central and vertical. Ruptures due to elevated IOP are either horizontal or parallel to limbus. They may be linear. Use of contact lens. and conjunctival epithelial down growth. . With ophthalmoscope they stand out as dark double lined areas in red background. This happens rarely when limbal vessels grow under the partial thickness wound of the cornea either accidentally or following large lamellar graft. herpes simplex. They may also grow from conjunctiva following—penetrating injury at the limbus. curvilinear or whirl like. lacrimation and photophobia. Their presence in buphthalmos is known as Haab’s line which are curvilinear. If the eyeball is compressed suddenly like in birth injury or concussion injury to the globe. It is of diagnostic significance not only for local corneal diseases but also neuro ophthalmic diagnosis. There is only one sensation in cornea i. Folds in Descemets membrane are more common than tears. Deep vascularisation gives a ground glass appearance to the cornea. 33. both blunt as well as incised wound. The area between the two lines also give a red glow. Cornea is very richly supplied by sensory nerve endings of trigeminal nerve. 34 : Descemets membrane is likely to rupture : 1. leprosy. Some of the dystrophies may look similar to Haab’s striae. penetrating keratoplasty. pain. With rupture of Descemet’s membrane there is a sudden influx of fluid into the stroma leading to stromal edema.DISORDERS OF CORNEA IN CHILDREN 171 10. They can be caused by trauma. Retrocorneal vascularisation. size. This type of vascularisation is mostly seen when blood vessels from uvea develop on the endothelium of the cornea either due to chronic uveitis. Rupture of Descemet’s 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 Descemet’s membrane. Causes of deep vascularisation are—Congenital syphilis. Rupture and folds in Descemets membrane32. chemical burn. 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. 11. The tears differ in shape. Causes of diminished corneal sensation are : 1. Corneal sensation may be marginally reduced or may be totally absent.

leprosy. Buphthalmos 6. Punctate keratitis stain irregularly and poorly with fluorescein but better with rose bengal. corneal scar. leprosy. Superficial keratitis can be diffuse or punctate.Keratopathy. shape and distribution of superficial punctate keratitis vary depending upon severity and duration of the 2. corneal vascularisation. complications are more in children. Keratitis in infancy may be responsible for nystagmus. Parenchymatous keratitis .172 3. Keratitis with loss of epithelium is called corneal ulcer. herpes zoster. Repair of corneal wound. (ii) Chronic superficial keratitis is seen mostly in trachoma. Deep keratitis 1. Keratitis in general has been described under various heading. Herpes simplex. Cerbropontine angle tumour 10. Though it too has its short comings. Hence keratitis requires prompt diagnosis and efficient management. A lesion may stain partly with fluorescein and partly with rose bengal depending upon level of the lesion. A large group of cases which are not caused by microbes are grouped under a loose term . Vitamin A deficiency 8. Chronic superficial keratitis is characterised by pannus formation. mollouscum contagiosum. Keratitis in children basically does not differ from those seen in adults. The unstained lesion’s have a whitish appearance. tattoo 9. Trigeminal neuritis Xerosis of cornea (see xerophthalmia) Corneal Ulcer in Children PEDIATRIC OPHTHALMOLOGY Inflammation of cornea secondary to micro-organisms is called keratitis. However most accepted classification by Duke Elder 1565 on the basis of etiology still remains most widely accepted classification. keratoplasty 7.e. however. band keratopathy. (a) Diffuse superficial keratitis can be (i) Acute superficial keratitis as seen in acute bacterial and viral conjunctivitis that may result in corneal ulcer. Deposits on cornea i. The classification is as follows Keratitis35 : 1. Corneal edema. Superficial keratitis has been divided into (a) Diffuse and punctate keratitis (b) Corneal ulcer (c) Degenerative superficial keratitis. Superficial keratitis 3. The size. 5. Most of keratitis in spite of good management leave corneal opacity that may produce amblyopia and squint. phlycten. 4.

Vitamin A deficiency disease (VAD) 6. sex or race is immune to corneal ulcer. trachoma. raised intraocular tension. the lids should be examined for misdirected lash. The probable primary causes should be treated with appropriate antibiotic. vernal conjunctivitis. However some of the processes like epithelial keratitis may be limited to epithelium. Intact protective mechanism saves cornea from trauma. 4. corneal edema. They may be primary corneal or secondary to conjunctival or lid lesions. However. ectropion of lid. 3. trichiasis. may be a mild abrasion or the traumatic factor may be persistent like ill fitted contact lens.DISORDERS OF CORNEA IN CHILDREN 173 lesion. they are more common in second to fourth decade. 2. ultra violet light. Sometimes they are seen with various type of skin lesions. If there is photophobia and anterior chamber reaction. 37 Corneal ulcer is an inflammatory process (infective or sterile) where there is an epithelial defect with some loss of stroma. General consideration : 1. short acting cycloplegic like home atropine 2%. Corneal ulcers are generally uniocular unless associated with systemic diseases like— Vitamin A deficiency. Though the conjunctiva is never sterile except first few hours after birth. Indiscriminate use of antibiotics and steroids. proptosis. inclusion conjunctivitis. coryne bacterium diptheriae. entropion. Failure in protective mechanism anatomical or neurological predispose corneal ulceration (a) Coloboma of the lid. entropion of lid. loss of sensation. They may be localised in epithelium. ophthalmia neonatorum. may be sub-epithelial. however. 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. Corneal Ulcer35. 5. The epithelial defect stains with fluorescein. repeated use of local anaesthetic agent. ill fitting contact lens. foreign body on the cornea or tarsal plate. Epithelial break is generally due to trauma. No age. concretion. leprosy. Management of superficial keratitis. blast injury. there may be a combination of both forms in the same eye. There is no specific treatment. herpes simplex and zoster. trichiasis. pad and bandage at bed time gives early relief. . blepharitis or foreign body in tarsal plate especially in unilateral cases. A breach in the epithelium is a pre-requisite for all infective corneal ulcers exceptgonococcus. 36. juvenile rheumatoid arthritis. Common conditions associated with punctate keratitis are : Staphylococcal blepharoconjunctivitis. chemical burn. molluscum. Infective corneal ulcer is generally associated with anterior chamber reaction. meningococcus. (b) Lagophthalmos (c) Loss of sensation (d) Abnormal tear film. Infectious corneal ulcers are generally associated with stromal infiltration.

the Descemet’s membrane gives way and a perforation of cornea results with resultant loss of AC. Over all result is formation of a corneal opacity depending upon the depth of the opacity it can be . If proper anti-microbial drugs are used at this stage. organism reach cornea via sensory nerve ending. Healing is initiated by natural defence mechanism of host cornea. If the anterior chamber reaction is severe. Progressive infiltration. circumciliary congestion disproportionate to size of the ulcer. Invasion by micro-organism. 4. intraocular contents including lens may be expelled through the perforation. in case of acute bacterial conjunctivitis. Peripheral ulcers are generally non infective. further progress can be averted or minimised resulting into clear cornea. 3. Once micro-organism has reached traumatised epithelium. 7. 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.nebular. Deep and large ulcers heal with treatment. chronic dacryocystitis. hypopyon may develop. watering. The ulcer deepens with infiltration and edema of the stroma. Prolonged use of steroid. 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. Organisms also reach cornea by direct continuity of conjunctiva. there may be associated superficial vascularisation. softening of eye and plugging of perforation by iris. 8.e. Failure of regression. Central corneal ulcers are generally due to exogenous infection. Organism generally get access to cornea following trauma. Commonest site for perforation is centre of the cornea which is thinnest. The fibrocytes are opaque and do not confirm with normal corneal architecture. The infected epithelium is necrosed and sequesteredoff with stromal infiltration. gonococci meningococci and diphtheria however may invade intact epithelium. there will be a leucoma adherent. photophobia. Pathogenesis of corneal ulcer35 Evolution of infective corneal ulcer has following stages : 1. If the perforation is large. a corneal fistula results. If the perforation is small but is not closed by iris. This is enhanced by treatment. At this stage anterior uveitis develops due to toxins liberated by the organism which percolate to endothelium and spreads to uvea resulting into pain. If iris is incarcerated. Cornea subjected to constant inoculation by micro-organism i. If . Small and superficial ulcers may heal in due course with resultant corneal opacity without treatment. 2. If intraocular pressure rises suddenly or remains raised for considerable time. The ulcer may spread all around towards the periphery and or it may deepen to reach the Descemet’s membrane which is tougher than other structures of cornea hence is not destroyed with ease. macular or leucomatous. In herpes simplex and zoster.174 PEDIATRIC OPHTHALMOLOGY (b) Systemic. Regression. resistant organisms AIDS. Active ulceration. blepharoconjunctivitis. The Descemet’s membrane bulges through the defective stroma even with normal IOP and forms a keratocele or Descemetocele. Healing starts with laying down of fibrocytes in place of normal keratocytes.

In infants trauma by own nails is very common. Fungal ulcers take weeks to manifest. Lacrimation and foreign body sensation. complicated cataract. Symptoms : 1. The surrounding area is edematous and infiltrated by inflammatory cells. Generally there is single central ulcer. Koch-Weeks bacilli produce severe pain. endophthalmitis. vision diminishes fast. it may be so trivial that the child may not be able to recollect. the ulcer is bound to perforate. large sloughing ulcers and neurotropic ulcers. cornealfistula. With progression of ulcer. Corneal sensation is diminished in viral ulcers. phthisis. it means perforation. . Breach in epithelium is brought about by trauma. 5. pseudomonas.Visual status depends upon position of ulcer itself. 3. 38. Signs. Spasm of the ciliary body is the foremost cause of pain. anterior staphyloma. and area of surrounding infiltration. 7. pseudomonas have short incubation period. 37. Trauma is generally mechanical. Late increase of pain is due to rise of intraocular pressure. 4. Central ulcers produce more visual loss while peripheral ulcers may not hamper vision at all or visual loss is minimal. this is associated with photophobia due to irritation or trigeminal nerve. The ulcer starts as an area of greyish infiltration that is lustureless. Circumcorneal congestion is prominent sign of anterior uveal inflammation that follows deepening of the ulcer and liberation of toxin. Most of the bacterial corneal ulcers are associated with mucopurulent conjunctivitis. meningococcus and diphtheria. In severe ulcer hypopyon may become evident. Depending upon type and virulence of offending organism ulcer may develop within hours to days. severity of trauma is variable. Pneunococcus. The edge of the ulcer stains brighter than floor. 2. pano-phthalmitis. Vision . peripheral ulcers may be multiple. These are the two early symptoms. Other cause of pain is stimulation of corneal nerve endings. 6. anteriorpolarcataract. Corneal nerve endings become numb with progress of ulcer. Foreign body sensation becomes worse and is converted into dull ache. Redness of eye is due to circumciliary congestion. The surrounding edematous and infiltration takes a yellowish green stain. Biomicroscopy shows anterior chamber reaction with flare and cells. The ulcer stains bright green with fluorescein. resulting into—leucoma adherent. Clinical features of corneal ulcer35.DISORDERS OF CORNEA IN CHILDREN 175 progression of ulcers is not checked. Sudden relief of pain is an ominous symptom. pneumococcal. there is distortion of windows reflex over the ulcer. Blepharo-spasm is a prominent feature in children. Sometimes there may be more than one offending organism responsible for corneal ulcer. In case of hypothesia of cornea that is found in herpes simplex and neurotropic ulcers patient may not complain of pain. For a corneal ulcer to develop breach in epithelium is a prerequisite except in gonococcus.

Only drawback with rose bengal is its sting. Chemically rose bengal is similar to fluorescein and is water soluble. large lesions are visible with ordinary flash light. epithelial edema. demonstration of leaking wound.e. For finer details an optical section of cornea may be drawn. dead and damaged cells. Jones I and II test. Purpose of staining is to demonstrate presence of a stainable lesion i. 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. One is frontal and other is vertical section of anterior segment as seen on slit lamp. applanation tonometry. Rose bengal causes severe stinging sensation of the conjunctiva. It is used along with fluorescein in many superficial corneal lesion. It is used either as one percent autoclaved solution or as 1% stain impregnated. Fluorescein is notorious to harbour pseudomonas. The patient should be informed about this phenomenon which is otherwise harmless. Other purpose is to know the extent of ulcer. Extra corneal examination uses are fluorescein angiography. however. It is water soluble. pre sterilised. Rose Bengal. Rose bengal is used to stain mucous. Corneal lesions are drawn in two views. dead and damaged cells. its progress or healing. It does not share infectious tendency of fluorescein. It stains epithelial defect. it stains tear film also but not the dry spot. Confirmation of diagnosis—History. Corneal lesions are stained by vital stains. mucous. Rose bengal stains filament of epithelium in filamentary keratopathy while base of the filament stains with fluorescein. ulceration. Dry crystals of both the chemicals are brown in colour. Like colour coded drawing of fundus for retinal lesions. (ii) Specific treatment .176 PEDIATRIC OPHTHALMOLOGY Staining of corneal lesions. disposable strip. clinical examination and stain give definite clue to causative factor. Lesions of cornea are documented by drawing with international colour code. xylocaine can cause epithelial changes that may stain with rose bengal. Rose bengal stains peripheral viral laden cells and fluorescein will stain floor of the ulcer in herpatic ulcer. Fluorescein staining should be performed after testing corneal sensation and before instilling local anaesthetic agent. Two commonly used stains are Fluorescein and Rose Bengal. hence washable. Other uses of fluorescein are contact lens fitting. abrasion. Prior instillation of local anaesthetic abolishes sting of rose bengal. desquamation. Documentation of corneal lesions. While using fluorescein its sterility should be guaranteed. Fluorescein remains extracellular.e. tear film study. small and faint lesions are best seen under cobalt blue filter with magnification. single use. evaluation of patency nasolacrimal duct i. Proparacine hydrochloride 1% aqueous drops is the only recommended local anaesthetic that does not interfere with rose bengal stain. Management of infective corneal ulcer : Management consists of : (i) Confirmation of diagnosis (iii) Ancillary treatment. Fluorescein does not cause irritation of cornea or conjunctiva.

dry surface that has zone of infiltration all round. In most of the cases there is history of trauma by organic material. and stains with Giemsa stain. Recurrent ulcers . Bacterial corneal ulcers are likely to develop hypopyon more frequently. Characteristics of viral ulcer. the chances are that it is a bacterial ulcer. 2. Like bacterial ulcers. If an aggressive ulcer develops following corneal injury within twenty four hours. They promptly stain with fluorescein.DISORDERS OF CORNEA IN CHILDREN 177 Infective ulcers can be caused by—bacteria. Most of the bacterial ulcers are associated with mucopurulent conjunctivitis. Sensation over clear cornea is generally normal. fungal ulcers are also central in nature. It is very common for viral and fungal ulcers to be contaminated by bacteria. Generally central. Characteristics of bacterial corneal ulcer : They are : 1. indolent. Fungal corneal ulcer has a few typical features42 : 1. 5. Primary herpes simplex is disease of infants and children. There is pronounced anterior chamber reaction that results in formation of endothelial plaque and thick almost organised hypopyon. Each group of organism have specific characteristics. 4. simplex type I. pseudomonas.e. However pneumococci. even when cornea is infected by bacteria on the periphery due to some ill understood phenomenon ulcer starts in the centre. Corneal biopsy may be needed some time. Bacterial ulcer may be caused by single organism or there may be mixed infection. or Gomori methamine silver stain. that can be cultured and sensitivity of the organism to antibiotic can be determined. 7. P. Scrapping from the ulcer show fungus in 10% KOH solution. Characteristics of fungal ulcer37. Scrapping from fresh and untreated ulcer give positive smear. Reverse is also possible but rare. type II and zoster virus are of clinical significance as far as corneal ulceration is concerned.S. 2. 6. streptococcus and staphylococci are more common causative factors than others. Most of the bacteria are capable of producing hypoypon. 3. fungi and viruses. 40. It takes seven to fifteen days for a fungal ulcer to develop following injury. Fungi are cultured at room temperature on blood agar and Sabouraud medium. Almost all viruses that invade conjunctiva also involve cornea and produce various types of superficial keratitis only herpes group of viruses i. Exposure keratitis. 3. Bacterial ulcers are generally associated with severe pain especially in pneumococcal ulcers. History of prolonged and indiscriminate use of local and systemic steroid is second most predisposition.A. edematous cornea are also likely to develop fungal ulcer. They develop in short time following injury and spread fast. there are smaller satellite lesions away from the main ulcer. 5. 8. 4. It is central. anaesthetic cornea. yellowishwhite ulcer with dull. they are more indolent. 41.

(2) circumferential . In later stage with disquamation of epithelium fluorescein stain becomes prominent. both can be superficial. Opacity developing in infancy may cause nystagmus. Diminished vision is not only due to obstruction of light to pass through pupil but also due to accompanied irregular astigmatism. Even successful treatment leaves residual central opacity that causes diminished vision. sometimes diminished corneal sensation. 44. Smaller ulcers may require cobalt blue filter to become visible many viral ulcers are bilateral. The ulcer is unilateral. Marginal corneal ulcers are as common as central ulcers. Circumferential ulcers have tendency to develop heavy superficial vascularisation. Peripheral (marginal) corneal ulcers35. The ulcers are small and numerous. Toxin 4. Etiological factors are generally systemic. As they develop in front of pupil. Corneal ulcers can be central or peripheral. Morphological distribution of corneal ulcer. deep. There is no anterior chamber reaction.may form a ring ulcer. photophobia. infective or non infective. However incidence of peripheral ulcer is less in children than adults. phlycten. diminished vision is early and marked that becomes worse with extension of ulcer. Corneal infiltrates and epithelial defect are minimal or absent. rosacea. Other symptoms are mild redness and pain. Vision is diminished if the lesion is central. Central corneal ulcers are mostly infective. fungi and viruses. 43. no hypopyon. Marginal ulcers in children do not perforate unless secondary bacterial infection is superimposed. corneal sensation is normal. The other symptoms of viral keratitis are foreign body sensation. trachoma. Characteristics of sterile corneal ulcer.178 PEDIATRIC OPHTHALMOLOGY are seen in adults. mostly bilateral. staphylococcal blepharoconjunctivitis. may be caused by bacteria. These ulcers are generally peripheral. Most characteristic sign of herpetic ulcer is diminish corneal sensation. Initially the ulcer stains poorly with fluorescein and better with rose bengal. Initially there is hardly any circumciliary congestion. They are less dramatic than central ulcers and have a more benign course. They stain with fluorescein. Causes of their being superficial and non perforating is better nutrition on the periphery of cornea and enhanced thickness of corneal periphery. Metabolic Marginal ulcers according to their position can be (1) sectorial. Diminished vision following central corneal ulcer puts child at an added disadvantage of intractable amblyopia and squint. Allergic—phlycten 3. 2. Circumferential ulcers generally result due to coalescence of more than one peripheral ulcer. relatively quiet. generally central and dendritic in shape without much redness and pain.e. diminished vision. without circumcorneal congestion. Causes of peripheral corneal ulcers are : 1. . lacrimation. Extension of conjunctival disease in the cornea i. Central corneal ulcers42. hence draw less and late attention than central ulcers. Herpetic ulcer does not develop hypopyon unless secondarily infected by bacteria.

Another popular combination is combination of neomycin with chloramphenicol.3%. raised intraocular tension. they have slow absorption. Commonly used fortified solutions used for corneal ulcer are : Gentamycin 14 mg/ml. one drop each hour in the lower fornix.35% + Polymyxine B. Cephazoline 33 mg/ml. sparfloxacin 0.3%. They need not be applied frequently. Sometimes a combination of antibiotics that have synergistic effect is used.%-0. Culture of virus is done only in few selected and advanced centres. More than one drop at a time is not retained in the conjunctiva and flows out without additional therapeutic advantage.e. Diabetes is generally not a problem in children but should be kept in mind if it is associated with symptoms of juvenile diabetes. Generally a long acting antibiotic that is effective against both gram positive and negative organisms is used as aqueous drops. fortified antibiotic solutions are instilled instead of ordinary drops.3%. Malnutrition.3%.3%. loss of sensation. lagophthalmos. Specific treatment : 1. Chloramphenicol 03. Once it has been established that the ulcer is infective and its causative organism identified. slow drainage from the conjunctival sac. However it is not always practical to get smear. culture and sensitivity done. Framycetin 0.5%. . A common combination are Neomycin 0. In adults and older children subconjunctival injection may be given. 10000 u and Bacitracin 500u. Newer antibiotics are being added frequently replacing older antibiotics. Ciprofloxicin 0. Commonly used antibiotic in infective corneal ulcer are Erythromycin 0. Advantage of ophthalmic ointments are : They act as lubricants. Local antibiotics. Ofloxacin 0. Fungal culture takes as much as a fortnight to be informative. They prevent sticking of the lids. Anti microbial drugs. specific anti microbial drops in suitable strength and frequency will eliminate the organism and enhance healing. Most practical method is to decide an antibiotic according to clinical feature. measles. Gentamycin 0.5%. chronic dacryocystitis. Frequency depends upon severity of the condition. Tobramycin 0. Systemic diseases. Tobracin 15 mg/ml Ophthalmic ointment is used at bed time either with or without bandage.DISORDERS OF CORNEA IN CHILDREN 179 Identification of specific causative organism is done by : (a) Corneal scrapping and examination of stained slide. vitamin A deficiency. lid deformity. result of sensitivity to antibiotic for bacterial infection takes minimum 24 hours to 48 hours. (b) Culture of organism (c) Sensitivity of organism to antibiotic. Identification of other ocular diseases that may influence treatment of corneal ulcer i. Whenever it is not possible to administer subconjunctival injection. These drugs are instilled as drops.5%.

in inflamed eye more than one instillation is required. Commonly used cycloplegics in corneal ulcer are : (i) Atropine sulphate. analgesics or anti microbial. One drop of one percent atropine contain more than therapeutic dose. It is less powerful cycloplegic than atropine. .e. While prescribing atropine to children the parents should be appraised of the side effect of atropine i. Cycloplegics have no action on cornea. they are not anti-inflammatory. Hence they are either given along with local instillation or at bed time with a pad and bandage. Its action last for more than ten days following single instillation. Bacterial and fungal corneal ulcers are generally associated with mucopurulent or purulent discharge. As such pure mydriatics have no role in management of corneal ulcer. they dilate the pupil and break posterior synechea. As all cycloplegics are strong mydriatic also. If necessary short acting cycloplegic may be added to atropine to enhance effect of atropine. It is generally used in cases of superficial small ulcers. Atropine should be used with caution in children. Commonly used subconjunctival injections are : Penicillin—1 million unit/ml Gentamycin—20 mg-40 mg/ml Cephazoline—100 mg-125 mg /ml Tobramycin—20 mg-40 mg/ml Subconjunctival injection of antibiotic gives a high but short lived peak of antibiotic concentration at the site of injection. 3. (For anti fungal and anti-viral drugs used in corneal ulcer see under specific ulcers) 2.180 PEDIATRIC OPHTHALMOLOGY Subconjunctival injection. however. It is better to avoid atropine as drop in children. and dryness of mouth which are not serious and can be managed by systemic antipyretic and plenty of fluids orally. Its side effects are less than atropine so it can be used frequently. In no case should atropine be used more than twice in twenty four hours. Atropine is most potent cycloplegic and prompt mydriatic. Cycloplegics relive spasm of ciliary body. Removal of discharge. Dilated pupil makes the child uncomfortable due to glare. flushed face. Cycloplegics should be used in all corneal ulcers with anterior chamber reaction. Irrigation of the conjunctival sac has no added advantage. Efforts should be made to remove the discharge from the conjunctival sac and lid margin with sterile wet swab. Generally subconjunctival injections are given once a day for five to seven days. Prolonged use of atropine may cause allergic dermatitis. Main function of cycloplegic in corneal ulcer is to prevent and treat associated anterior uveitis that is main cause of pain and photophobia. Its action does not last more than 48 hours. fever. thus reduce pain indirectly. Systemic antibiotics do not have any added advantage over local antibiotics. However in children who develop allergy to atropine are also allergic to home atropine. On the contrary it washes away tear lysozyme which has anti microbial property. Cycloplegia. (ii) Home atropine hydrobromide 2%.

Destruction of ulcer : (i) Cauterisation of ulcer. Bacteria produce hypopyon earlier than fungi. However pad and bandage raise the temperature of the conjunctiva which encourages proliferation of micro organism. occasional mononeuclear cells. It contains mostly polymorphonuclear leucocytes. 6. it only reduces movement of lid that helps epithelisation of ulcer. Pad and bandage. Development of pus is preceded by severe anterior chamber reaction. Amount of hypopyon is directly proportionate to virulence of organism. Management of hypopyon corneal ulcer. The pus gets infected following perforation of ulcer. it assumes a horizontal level at the upper part. Bandage contact lenses are good substitute for usual pad and bandage. others cause minimal hypopyon. In case of non healing corneal ulcer. As pus is heavier than aqueous. Though hypopyon is thicker than aqueous yet it freely moves unless it gets organised as is seen in fungal ulcer. it may be occasionally tinted with blood.DISORDERS OF CORNEA IN CHILDREN 181 (iii) Cyclopentolate hydrochloride. In bacterial corneal ulcer. trichlor acetic acid. 5. can be used frequently. application of pad is contra indicated in presence of copious discharge. (ii) Physical methods of destruction of ulcers are Heat cautery. and is yellowish in fungus. 4. Presence of pus in viral keratitis denotes presence of secondary bacterial infection. penetrating keratoplasty is indicated. The pus is due to out pouring of exudates from iris and ciliary body. It is a prominent feature in central corneal ulcers and penetrating injury. This is used as 1% drop. Presence of pus cells in the anterior chamber gives it characteristic white colour. debridement of the ulcer is done under local anaesthesia and edges are cauterised by pure carbolic with caution. macro-phages and fibrin. The aqueous becomes plasmoid and fibrinous that entangles leucocytes. Pad and bandage prevent movement of lid over the ulcer this stabilises the epithelial growth during healing. Some organisms characteristically produce severe hypopyon. Virus by themselves do not produce hypopyon. cryo and laser. however the pus is tinged green in pseudomonas infection. All bacteria are capable of producing hypopyon. Hypopyon in fungal ulcer is generally infected because fungus can penetrate intact Descemet’s membrane. hypopyon settles down at the most dependent part of anterior chamber. Hypopyon is acculumation of sterile pus in anterior chamber. hence. Hypopyon corneal ulcer denotes presence of severe anterior uveitis due to corneal ulcer. Tropicamide 1% is very short acting unless used repeatedly has hardly any beneficial effect in corneal ulcer. the pus remains sterile so long as Descemet’s membrane is intact. 7. It does not abolish movement of eye. has action faster than home atropine but action lasts for twenty four hours to forty eight hours. If ulcer fails to respond to usual treatment within a week. The pus in bacterial corneal ulcer is worsened by absorption of toxin liberated by causative organism. Some organisms produce early . Other drugs used to cauterise the ulcer are 7% alcoholic solution of iodine.

mild. Atropinisation should be done under supervision of trained person. streptococci pyogenes. it is associated with severe anterior chamber reaction. Organisms that produce severe hypopyon are—pneumococci. Atropine drops are better avoided because an illiterate attendant may instil atropine frequently in place of antibiotic without anticipating serious complication.182 PEDIATRIC OPHTHALMOLOGY and severe hypopyon. Subconjunctival injection in children is difficult to administer. Outline of treatment of hypopyon—Corneal ulcer should be as follows : 1. The ulcer has multiple satellite lesion and an endothelial plaque under the original ulcer. Affective and safe atropinisation. Management of hypopyon corneal ulcer is basically the same as any infective ulcer with severe uveitis. 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. Corneal scrapping : (i) Examine part of scrapped material after staining with Gram’s stain. . does not move freely with change in position of the eye. The pus is generally thick. In children sub-conjunctival injection and cauterisation should be done under short acting general anaesthesia or good sedation. light is thrown from the above in front of the iris. 4. 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. 5. It is better to hospitalise the patient and start vigorous antibiotic drop instillation. moderate. Bandage lenses and occuserts are good alternative when available and possible. 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. gonococcus. All cases of hypopyon should receive atropine sulphate 1% as an ointment. pseudomonas. (ii) Inoculate part of the material to grow bacteria or fungi. 10% KOH to identify bacteria and fungus and find out if the characteristics present are in favour of bacteria or fungi. Hospitalise the child. (iii) Identify organism and test for sensitivity to antibiotic. Hence frequent local instillation of suitable fortified antibiotic is most important part of treatment. If possible give sub-conjunctival injection of the same antibiotic. yellowish in colour and infected with fungal element. Hypopyon in fungal corneal ulcer takes more time to develop. replace initial antibiotic with sensitive antibiotic as fortified drops. is fluffy. Hypopyon corneal ulcer should be treated as an emergency. making it difficult to be visualised. half of AC or full of AC. Once best possible antibiotic has been identified. 3. Giemsa stain. 2. Extent of hypopyon is measured by its height from bottom of anterior chamber in millimeters or roughly as trace. In recumbent position the pus spreads all over the iris or may pass through the pupil.

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

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

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3. If necessary, intra cameral antibiotics can be injected. 4. Following paracentesis, there is out pouring of fresh aqueous that has healing property. 4. Strengthening the thinned cornea by cynoacrylate substance when the ulcer is small. 5. Keratoplasty. In selected cases with rim of healthy cornea all round, penetrating keratoplasty may be tried. One of the advantages of penetrating keratoplasty is that it removes the infected part of the cornea, which harbours micro-organism, hypopyon is drained through the wound, AC can be washed with balanced salt solution antibiotics can be instilled directly in AC. Chances of dens central corneal opacity are reduced. Complications of corneal ulcer. Aim of treatment of corneal ulcer is to eliminate infection and give a clear cornea. Corneal opacity is almost unavoidable even with best treatment unless it involves the epithelium only. The size and depth of opacity varies with extent of ulcer and promptness of treatment. Ulcers anterior to the Bowman’s 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 Bowman’s 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 Bowman’s membrane. Small nebular opacities may be missed on oblique illumination but are best visualised on slit lamp biomicroscopy. On retinoscopy they cause irregular reflex. Though they do not cause much visual loss diminished vision due to nebulae are sufficient to cause amblyopia.

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Macular opacities are denser than nebulae and visualised by ordinary flashlight. They are due to destruction of Bowman’s 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 Descemet’s 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 Descemet’s membrane. Small corneal perforation is plugged by a knuckle of iris to form an adherent leucoma. A large perforation is not immediately sealed. As has been discussed earlier, a perforation leads to draining of aqueous, flattening of cornea, softening of eyeball, miosis and shift of iris lens diaphragm forwards. This forwarded shifted iris blocks the perforation and the constricted pupil is closed by exudate that acts as a scaffolding over which fibrocytes from stroma and epithelium develop to form a white scar or pseudocornea of uneven thickness which is very thin and plastered with iris on posterior surface at places the whole of the scar gets ectetic and bulges forward. This ectatic cicatrix with iris incarcerated is called corneal or anterior staphyloma. It can engulf whole of cornea and called total anterior staphyloma or may be partial. The anterior staphyloma may be vascularised, may have facets. There is always associated secondary glaucoma, which produces loss of vision which is very common. A large staphyloma may bulge through inter palpebral fissure. Generally the staphylomatous eyes are divergent and have nystagmus. Commonly they are not painful, uncontrolled glaucoma or break down of scar make them painful. In unfortunate children anterior staphyloma can be bilateral. Small pox used to be commonest

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

188 Complication of corneal fistula are :

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1. Endophthalmitis, panophthalmitis due to introduction of infection. 2. Phthisis due to persistent hypotony. 3. Epithelial down growth. Summary of perforation of corneal ulcer : 1. Small perforation with mobile iris. Adherent leucoma. 2. Moderate perforation with miotic pupil and mobile iris—corneal 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 iris—anterior 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. Bowman’s membrane and Descemets membrane once destroyed do not regain transparency. 2. Edema of cornea. It could be epithelial, stromal, endothelial or combined. 3. Inflammatory infiltration. 4. Vascularisation. Superficial or deep vascularisation always leave scar of obliterated vessels. 5. Degeneration of cornea. Band keratopathy is more common in children than in adults. 6. Dystropies of cornea. Many of corneal dystrophies manifest in childhood. 7. Deposits on cornea. Tattoo, corneal mucus plaque.

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

190 4. Painted contact lenses mask the corneal opacity.

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

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

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graded. The pneumococci is known to become resistant to antibiotics specially penicillin. Generally systemic antibiotics are not recommended unless patient has systemic involvement. Systemic antibiotic should be administered in full dose in consultation with physician. All cases of pneumococcal keratitis threatening to perforate should also get systemic antibiotic. Usually available commercial ophthalmic drops are not sufficient for management of suppurative keratitis. Fortified aqueous drops should be administered frequently. Frequency may be as often as every five minutes for six times followed by every ten minutes for another six times. This is followed by hourly instillation during day and two hourly at night. Commonly used fortified drops are : Penicillin G—100,000 1u/cc Bacitracin—10,000 IU/cc Cefazolin—50 mg/c.c. Other antibiotics used are erythromycin, vancomycin. Ciprofloxacin is less effective. If topical antibiotic drops are not effective or there is a moderate hypopyon concurrent sub-conjunctival injection of either 0.5 to 1.0 milli units of penicillin or 50mg to 100mg of cefazolin BD may evert further extension. As there is always associated severe uveitis, use of atropine is one of the most important steps in suppurative keratitis. Atropine is administered as one percent ointment two times a day. It is better to avoid atropine as drops in children because there is always danger of atropine drop being instilled in dose more than therapeutically permitted. The eye should be bandaged at night only if there is no discharge. Pseudomonas keratitis46, 48, 51. Pseudomonas aeruginosa causes one of the most serious central corneal ulcers and is one of the most common causes of perforating corneal ulcer. The organism is an opportunistic, gram negative, mobile, obligate aerobe that produces a proteolytic enzyme which acts on stroma and is responsible for early perforation. About 5% of population pass pseudomonas in stool and yet be asymptomatic. Common mode of infection are - instillation of contaminated fluorescein or anaesthetic drops in hospitals or by contaminated contact lenses specially extended wear. It has also been cultured from contact lens solutions. Pseudomonas has a short incubation period. Otherwise it may follow mild trauma. Pseudomonas corneal ulcer starts as a small central area of infiltration following trivial injury and spreads in all directions. Occasionally it may start in the periphery. The ulcer spreads rapidly, is always associated with severe pain. Hypopyon develops fast and increases with spread of ulcer. Hypopyon has greenish blue hue due to pigment produced by the organism. Pseudomonas ulcer may spread so fast as to cause large central perforation within twenty four hours. Ulcer is always associated with mucopurulent conjunctivitis that may have satellite conjunctival abscess. Peripheral ulcers may spread into the sclera as tunnel lesions. All fast spreading corneal ulcers in contact lens users should be considered to be due to pseudomonas unless proved otherwise by culture. Management of pseudomonas ulcer does not differ from any other central ulcer. When pseudomonas is suspected but its presence has not been confirmed a combination of fortified cepfazolin and tobramycin should be used as drops very frequently. Once

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

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

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

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

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

198 Commonly used cycloplegics are :

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

Herpes zoster gives life long immunity. (i) First antiviral drug of choice should be trifluridine locally whenever available or acyclovir locally supplemented by oral acyclovir. they still hold the fort when done carefully. Idoxuridine and vidarabine have no role in stromal involvement. instillation of cycloplegic drugs relieve discomfort. 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. food allergen. It is claimed that if trigger mechanism like fever. Cycloplegics are neither antiviral nor anti inflammatory analgesic. Chemical cautery by 7% alchoholic solution of iodine and carbolic was standard treatment before advent of antiviral drugs. (ii) Debridement.DISEASES OF CORNEA IN CHILDREN 199 attack and subsequent recurrence is variable even gaps between attacks of two episodes of recurrence is not definite. Whenever steroids are deemed necessary it should be under cover of effective antiviral drug. Metaherpatic keratitis. ultra violet exposure can be avoided. . Now they have been given up. Herpes zoster virus involves single dermatome HSV has irregular distribution. Whenever there is associated lacrimation and photophobia. 7. 5. Idoxuridine and vidarabine are effective against epithelial lesions only idoxuridine is most kerato toxic. pad and bandage. Their main function is to treat associated anterior uveitis. debridement may be followed by local instillation of trifluridine or acyclovir. 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. lubricants. Dendritic and Geographic ulcer. Simple debridement is an effective treatment for epithelial keratitis. (iii) Cycloplegic. Stromal HSV infection is always associated with uveitis making use of cycloplegic almost mandatory. Herpes zoster infection does not cross the midline. The herpes zoster virus is similar to herpes simplex virus in structure but different antigenically and clinically. Stromal involvement. Stromal involvement both necrotising or non necrotising are most difficult to manage. least frequently for shortest possible period. However when antiviral drugs are not available. 6.e. Use of steroid is one of the trickiest decisions. In herpes zoster there is a single recurrence that may have a prolonged course while more than one recurrences are common in HSV infection. 200 to 400 mg five times a day for five to ten days. vericella (chicken pox) and herpes zoster (shingles). bandage contact lens help to epithelise the effected cornea. recurrence is averted. Both viruses have primary infection followed by a period of latency and a recurrence. stress. 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. Number of recurrence is also uncertain.e. Trifluridne and acyclovir are effective in stromal herpes. Besides antiviral drops. well supervised and with weakest strength.

However there may be corneal involvement without involvement of nasociliary nerve. which may involve other cranial nerves as well. 2. may last for years associated with post herpetic neuralgia. Initially the lesions are maculo papular which become pustular within few days. In severe viremia encephalitis is possible. Involvement of maxillary division produces vesicular eruption in the lower lid. which are infective to others and can cause chicken pox in children. Kerato conjunctival. There may be ptosis due to various factors i. Cutaneous. : 1. Any of the branches of ophthalmic division may be involved but common involvements are supra orbital. True ptosis is due to involvement of upper division of third nerve. The crusts are loaded with live viruses. while herpes zoster infection is more common in adults. As the scabs fall of the edema subsides with punched out scar marks that can either be hyperpigmented or hypopigmented. There is generally mild pain in the distribution of dermatome where vescicles develop in due course.200 PEDIATRIC OPHTHALMOLOGY Herpes zoster virus infection differs from vericella in following ways. 5. Uveal. forehead and scalp are most commonly involved. It can produce madarosis. nasociliary and lacrimal. that rupture to form crust. Vericella is very common. 2. Cutaneous. Edema of the lids may obliterate the interpalpebral fissure due to mechanical pseudo ptosis. Others. The papulo macular stage is associated with intense edema that may spill over the mid line but not the rashes. It is rare in adults. Pseudo ptosis is generally due to inflammatory edema. Some degree of loss of sensation is always present. reverse is also possible. however children are not immune. In rare cases it may produce contra lateral hemiplegia. Commonest cause of paralysis of orbicularis is Ramsay Hunt syndrome where vescicles develops in the external auditory canal. Involvement of cranial nerves other than ophthalmic is mostly immunological rather than infectious in nature. ectropion or entropion. Scleral. Skin of the lids. 1. Generally orbiculosis is not involved in herpes zoster ophthalmicus. The second and third divisions may also be involved. 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 (Hutchinson’s sign). which is otherwise rare. Only ocular involvement without cutaneous manifestation has not been observed. The crusts gradually fall off leaving depressed marks similar to chicken pox. Vescicles on the lid margin may heal by producing notch in the lid margin.e. Ocular involvement in herpes zoster ophthalmicus can be divided under following heads : 1. Ocular involvement in herpes zoster is known as herpes zoster opthalmicus that has severe vision threatening ocular involvement with neurological defect. trichiasis. However rarely orbicularis palsy may develop independent of Ramsay Hunt . 4. supra trochlear. 3. (See Chapter 22 as well) Though involvement of first division of trigeminal is commonest manifestation. self limiting disease with predominant skin involvement and less pronounced ocular involvement in children. There may be localised involvement of any of the branches of ophthalmic division without ocular involvement.

It is associated with anterior uveitis and stromal infiltration. however. which may respond to local antiviral drugs. by this time the rashes have crusted and may have fallen i. 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. Conjunctival sensation is either lost or diminished. 2. stain with rose bengal and contain live viruses. 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. Mucopurulent conjunctivitis becomes worse with obliteration of I. they are transient. may be missed due to swollen lid and inflamed conjunctiva. To begin with iridocyclitis is mild with fine KP and few cells and flare. Uveitis. (i) Hypoethesia of cornea. It may be vascularised and cause lipid degeneration. 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. 2. Rarely there may be vescicles on the conjunctiva itself. Conjunctival involvement become obvious within first few days as mucopurulent conjunctivitis. Disciform keratitis are associated with thickening of stroma and anterior uveitis. Scleral. They stain with rose bengal. They are generally multiple. three weeks after appearance of rashes. This is invariably accompanied with corneal involvement. are seen between fourth to sixth day. Kerato conjunctival involvement : 1. Corneal involvement.DISEASES OF CORNEA IN CHILDREN 201 syndrome. some of them may coalesce to form lesions similar to dendrites. Earliest infective manifestation is punctate lesions in epithelium. Episcleritis and scleritis are very common in herpes zoster ophthalmicus in first week following onset of rashes.e. Scleritis may cause sclerokeratitis and sclero uveitis 4. 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. Iridocyclitis in herpes zoster is a non granulomatous in nature. Involvement of nasociliary nerve produces development of vescicle on the lateral side of nose and tip of nose. Due to loss of sensation patient does not complain of pain that may lead to secondary bacterial infection resulting in perforation. They are transient in nature. Loss of sensation leads to formation of trophic ulcer.P. mostly on the periphery. Dendrites of herpes zoster are smaller than dendrites of herpes simplex.A. This is always associated with vescicles of the lid. The plaque can be removed with ease. They appear within forty-eight hours of onset of skin lesion. Uveitis in herpes zoster ophthalmicus is limited to anterior uvea causing acute iridocyclitis within fortnight. effect of which could last for years and may cause permanent damage. . (ii) Keratitis. Corneal involvement is one of the most serious complications of herpes zoster ophthalmicus. 3.

3. Loss of corneal sensation is a perpetual problem that requires frequent instillation of broad spectrum antibiotic. Combination of antibiotic and steroid is recommended at the crusting stage. On the distribution of the dermatome involved leading to anaesthesia of cornea. Role of local antiviral drugs to combat conjunctivitis is not established. It is very severe in old age. Systemic corticosteroid under the umbrella of systemic antiviral drugs reduces lid edema. Local antiviral drugs are of doubtful value. 1. mild analgesic given orally is sufficient. Neurological involvement in herpes zoster ophthalmicus : (i) Commonest neurological sign of herpes zoster ophthalmicus is loss of sensation. 2. Management of herpes zoster ophthalmicus in children does not differ from management in adults. the swollon trabecular meshwork and inflammatory debris may cause obstruction of the drainage. Children have advantage of less frequent post herpetic neuralgia which is very mild. cause secondary infection and deep scar formation.202 PEDIATRIC OPHTHALMOLOGY 5. Anaesthesia masks pain of corneal and uveal involvement. This reduces viremia and enhances healing. As pain is not a very prominent feature of herpes zoster ophthalmicus in children. It is called anaesthesia dolorosa. Active immunisation against vericella has been recommended to prevent herpes zoster but its efficacy has not been proved beyond doubt. Acyclovir should be started within 48 hours of onset of rashes. If . Conjunctival lesions are treated by local broad spectrum antibiotics instilled in the conjunctiva. The patient must be put on steroid for months. Acyclovir does not have much effect on corneal lesions or postherpeticneuralgia but is known to diminish uveal involvement. long acting cycloplegic. Steroid in weakest possible strength is recommended. Fortunately it is rare and milder in children. A common complication of herpes zoster uveitis is secondary glaucoma that may be overshadowed by keratitis and missed altogether unless sought specifically. Skin lesions are best treated with oral acyclovir in dose of 50-60 mg/kg/day divided in five doses. They help to treat stromal involvement and uveitis. Corneal involvement in herpes zoster is a clinical riddle. resulting in scar formation. and deep scar. (ii) The next bothersome symptom is post herpetic neuralgia that may persist for months to years. Management of herpes zoster ophthalmicus58. It is caused due to associated trabeculitis. Encephalitis is a rare manifestation of herpes zoster ophthalmicus. In the vesicular stage combination of calamine with talcum powder or starch should be avoided as it masks the changes in the vescicles. Exact cause of post herpetic neuralgia in absence of sensation is not well understood. Local skin ointment of acyclovir is applied in the vesicular stage. Children can get as much as 400 mg acyclovir five times a day for 7 days. Steroids also helps in reducing post herpetic neuralgia. Local steroids must be given under strict medical supervision. conjunctiva and skin resulting in delayed healing and may lead to trophic ulcer. (iii) Other neurological involvement’s are—optic neuritis. cranial nerve palsy and contra lateral hemiplegia that may take few months to develop.

Sub-epithelial involvement is less frequent. The satellite ulcer may join to form a ring called Wessely ring. The injury is caused by foreign body of vegetable origin. 4. It is more common in hot and humid climate than in cold and dry climate. blurring of vision and pain. The conjunctiva is severely inflamed. It is generally unilateral and central following history of injury. dry eye. hypopyon and conjunctival congestion. (See Chapter 22 also) Following trauma with vegetable matter. Severity of infection depends upon size of inoculum and immuno competence of the cornea. 64 Fungal infection of cornea is less frequent than bacterial or viral keratitis. Common fillamentous fungi that cause corneal ulcer are aspergillus. Fungal keratitis is a suppurative. Adeno virus keratitis in children62 Almost all adeno viruses that cause conjunctivitis in children involve cornea to some degree. other conditions that predispose fungal keratitis are situations where corneal defence mechanism is compromised i. stromal herpetic keratitis. Optic neuritis. fusarium.DISEASES OF CORNEA IN CHILDREN 203 necessary. After a gap of ten to fifteen days acute fungal keratitis becomes manifest causing lacrimation. Filamentous and 2. indolent corneal ulcer with severe anterior chamber reaction. Non filamentous. shape and size. Besides trauma. There may be a epithelial plaque formation. paralysis of orbicularis.e. raised dry rough lesion with raised margin develops. vitamin A deficiency. Loss of corneal sensation is a contra indication for keratoplasty and contact lens. Corneal involvement in adeno viral infection is less common and less troublesome than seen in herpes simplex. tarsoraphy must be performed. 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. Fungal keratitis42. 63. Systemic steroid helps in shortening the course. The ulcer may extend in to surrounding sclera. Sub epithelial and stromal feathery extension may develop. Corneal involvement are less marked in children than in adults. first a sub epithelial gray white lesion develops at the site of trauma. loss of sensation. Fungi that cause corneal ulcer can be divided into two main groups i. The appearance is characteristic. . It takes more than ten to fifteen days for a fungal corneal ulcer to develop following trauma by vegetable material. transient and leave no scar. 1. photophobia. Use of steroid in such situation makes the condition worse. They are generally epithelial in nature and present as fine superficial keratitis that stain brightly with fluorescein. At the end of the feathery extension multiple satellite lesions may develop which vary in number. Their appearance is similar to original central ulcer. prolonged local or systemic steroid therapy. the initial breach in epithelium heals as simple corneal ulcer. There is an initial corneal abrasion that either heals spontaneously or is treated as bacterial corneal ulcer generally with antibiotic drops. The epithelium is raised over this and a dirty white. cephalosporium.e. cranial nerve palsy are self limiting. There is intense anterior chamber reaction with thick yellowish white hypopyon and severe anterior uveitis.

. Examination of corneal scrapping : 1. Laboratory finding consists of : 1. Appearance of ulcer 4. 3. 4. 2. 10% KOH solution is used most frequently to visualise septed fungal hyphae directly under microscope. Staining — Grams stain Giemsa stain Gomori methamine silver stain Lactophenol cotton blue66 Caleoflour white67 Culture68 — Sabouraud’s medium Blood agar Brain heart infusion 1. Management and complication of all fungal corneal ulcers is almost the same. it may take as long as fifteen to twenty days to show positive result. In KOH solution65 2. History 3. Unlike bacterial ulcers there are no characteristic features that can help clinically to differentiate various filamentous ulcers. Failure to diagnose. Management of fungal keratitis : Management of fungal corneal ulcer is one of the most frustrating forms of treatment due to : 1.204 PEDIATRIC OPHTHALMOLOGY It is not always possible to differentiate non filamentous fungal ulcer from filamentous ulcer. and phthisis. Non availability of broad spectrum antifungal antibiotic. Other common complications are secondary glaucoma. it invariably perforates and the eye is lost. it fails to stain filamentous fungi. Giemsa stain is more useful to define filamentous fungus. Gram stain is useful to visualise candida. 3. If a fungal ulcer is not treated in time. Even if the ulcer does not perforate. Frequent serious complications. It is a reliable test. it leaves a dense central opacity that compromises vision to great extend. Diagnosis : Diagnosis of fungal keratitis depends on : 1. Its clinical course and 2. 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. Gomori stain provide better fungal cytology. 2. complicated cataract. Culture is done at room temperature.

If stain confirms presence of yeast (candida) nystatin 50. . econazole 3. ketoconazole. Debridement may be followed by chemical cautery. Too enthusiastic cauterisation may enhance perforation hence it should be done carefully under magnification. Local betablockers and oral acetazolamide keeps IOP low and lowers incidence of perforation. The keratitis may be self limiting. They are caused by allergy. Amphotericin B. Most of them respond to local steroid. It may be limited to conjunctiva only. natamycin 2. Cortico steroids have no role in management of fungal keratitis Surgical treatment. Polyenes. 3. miconazole.1% to 0. Phlyctenular kerato conjunctivitis is very common in developing countries. Good debridement if necessary under general anaesthesia helps to remove infected material. Out of many marginal keratitis only phlyctenulosis of cornea is commonly seen in older children. In case of confirmed filamentous fungal ulcer. Imidazoles. Imidazoles. 2. They are : 1. Clotrimazole. Non infective causes outnumber infective causes. Perforation is due to secondary bacterial invasion. may involve limbus effecting both cornea and conjunctiva or may primarily develop in the cornea. Antifungal treatment should be continued for at least ten days before any improvement occurs. Pyrimidines 1. Anterior chamber reaction is either absent or minimal. Penetrating keratoplasty shows better results. Pyrimidine. 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. Some times marginal (catarrhal) ulcer may be seen in older children who may also be effected by autoimmune diseases like rheumatoid arthritis. flucytosine 1% and amphotericin B 0. They are generally bilateral. Peripheral keratitis70 Peripheral keratitis may result due to many local and systemic condition both infective as well as non infective.DISEASES OF CORNEA IN CHILDREN 205 Antifungal drugs69 : Three groups of antifungal drops are available. Local broad spectrum antibiotic drops are given to prevent secondary bacterial infection. Phlyctenulosis of cornea. Flucytocine In all cases of suspected fungal keratitis. However the donor cornea itself may get infected in course of time. or auto immune disorders. nystatin. Peripheral ulcers by themselves do not perforate. in spite of a benign course they are more painful than infective keratitis. ketaconazole or clotrimazole may be given hourly. miconazole.2% should be given every hourly. Lamellar keratoplasty fails to eliminate the infection.000 IU. initial treatment is instillation of natamycin 5% drop every hourly by day. Polyenes.

It can be diffuse or central. malaria. There may be involvement of retina and choroid that result in to salt pepper appearance. The epithelium and endothelium getting involved later. The disease is self limiting. The clinical picture has been divided into : 1. interstitial keratitis manifesting between 5 to 15 years of age. 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. Florid and . Anterior uveitis is early and prominent feature. tuberculosis and leprosy. onchocerciasis. blepharospasm. The child is infected in utro between first and second trimester. which is an important marginal keratitis is not seen in children. it responds well to steroids but not to pure anti syphiletic treatment. In late stages all the layers of cornea get effected. This is the stage when the symptoms are in peak. Florid stage—Progressive stage is followed by florid stage that lasts for two to three months. 72. Other causes are . 73. 1.Lymphogranuloma venereum. Conversely trapenoma has not been demonstrated in cornea in interstitial keratitis. It is expected that vessels in cornea should have bright red colour. Trapenoma has been isolated from syphilitic foetus and new born without evidence of interstitial keratitis. 74. Changes in iris are masked by corneal haze. This is the time when uveitis develops mostly in the form of acute iridocyclitis. Causes of haziness are due to changes in all the layers of cornea i. which is wrinkled. 3. Progressive stage lasts for two to three weeks following clinical symptom of lacrimation.Vernal kerato conjunctivitis and rheumatoid arthtitis. Vascularisation spread from all sides. Primarily interstitial keratitis is a disease of stroma. photophobia. In children commonest type of interstitial keratitis is due to syphilis in the form of congenital syphilis or hereditary syphilis.206 PEDIATRIC OPHTHALMOLOGY Other causes of peripheral keratitis are . bedewing of epithelium. Progressive . 2. Interstitial keratitis is a chronic disease of cornea with acute onset and protracted course. These changes when added result into a hazy cornea (ground glass appearance). herpes simplex. In children interstitial keratitis is generally diffuse and bilateral. Moorens ulcer. due to corneal haze the redness is toned down to a peculiar pink colour known as Salmon red colour. Interstitial keratitis has a long latent period of five to ten years following intrauterine infection.e. The Bowman’s membrane is wavy while deep vascularisation is . cellular infiltration round the newly formed vessels in front of the Descemet’s membrane. The cornea develops heavy deep vascularisation that gives a red hue to the cornea. The other eye getting involved few days after the first. Parenchymatous (Interstitial) Keratitis in Children71. from epithelium to endothelium with keratic precipitates. 2. Regressive stages. Uveal involvement is so pronounced that it is thought that basic pathology starts in the uvea and cornea gets involved secondarily. It is due to an antigen antibody reaction to spirochete trapenoma pallidum. Exact pathology of interstitial keratitis is not well understood. There may be anterior choroiditis as well. Other change seen in cornea on biomicroscopy are.

Otherwise stigmata of congenital syphilis i. Rhagades at angle of mouth. Once acute phase has subsided subconjunctival depot steroid can be given at an interval of 15 days. Dental changes—Hutchinson’s teeth The permanent upper central incisors are peg shaped reduced in length and breadth. Hutchinson’s teeth and deafness constitute Hutchinson’s triade. 5. Interstitial keratitis is self limiting disease which starts improving within two to three months. 4. Nose—Saddle shaped depression. Forehead—shows frontal eminence. Process of regression starts as the advancing vessel meet in the centre. Treatment—72. saddle nose.DISEASES OF CORNEA IN CHILDREN 207 developing in the stroma. 2. Systemic involvement in congenital syphilis : 1. 2. Positive serological test in mother and child. frontal bossing. Regressive stage—After four to five weeks. 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. photophobia and diminished vision. 3. anterior bowing of shin. 3. improvement in vision.e. buphthalmos. Other changes are—Retarded physical and mental growth. Superficial vascularisation may be superimposed in the epithelium on the periphery resulting into a crecentic pile of blood vessels known as epaulet. Laboratory investigations72 include 1. Elevated cell or protein in CSF without any other cause. Diagnosis. inflammation suddenly subsides with clearing of the cornea. if possibility of interstitial keratitis is kept in mind especially in a child borne to known syphilitic mother. Hutchinson’s teeth. Differential diagnosis consists of—All causes of hazy cornea i. 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. Once the cornea is fully vascularised. trachoma and congenital corneal haze. 6. deafness with severely vascularised cornea leads to diagnosis. Clutton’s joint. pain and photophobia. The condition is very troublesome to the child who has intense lacrimation.e. Local steroids reduce this period of ocular morbidity to few weeks. Interstitialkeratitis. 3. reduction of lacrimation. Reactive CSF-VDRL. Changes in ear—permanent deafness. cardio vascular disorders. 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. To treat associated uveitis that is always . Diagnosis is clinically simple. stage of regression starts which lasts for two to three years. They have a notch on the cutting edge.

There are eight species of acanthamoebae that cause corneal ulcer. Children on prolonged local steroids should be closely monitored for rise of tension. Only sympathomimetic drug like phenyl pherine has no role in management of uveitis. Use of non standard contact lens solutions specially home made. This initial stage mimic herpes simplex or bacterial keratitis and is confused as such. atropine sulphate is used as one percent drop two times a day under supervision along with local steroids. non-suppurative. limbitis and scleritis. Keratitis produced by acanthamoebae is indolent. The condition can be put in following three stages : Stage I. Swimming in a contaminated pool.e. 78. Stage II. soil. resistant to antibiotic. pseudo dendrite and epithelial defect. 1% cyclopentolate hydrochloride or 1% tropicamide. Desmetocele.e. Use of any type of contact lens 2. home made contact lens solutions. Besides throat and pharynx the organism is commonly found in fresh water. This consists of anterior stromal ring. disciform keratitis. trophozoite and cyst. it remains a relatively rare disease. antiviral and antifungal drugs. . Once acute phase has subsided atropine can be replaced by any of the following short action cycloplegic i. Diagnosis77. Trauma by vegetable matter. incidence of which is increasing. iritis. Pain is out of proportionate to the size of corneal involvement. 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. The changes consist of one or combination of many punctate lesions.208 PEDIATRIC OPHTHALMOLOGY present and is a dominant feature cycloplegic drugs are used in acute phase. 3. 75. redness and photophobia. 2% home atropine hydrobromide. steroid induced cataract and secondary infection. Corneal lesions may be as mild as epidemic kerato conjunctivitis or as severe as Descemtocele or perforation. 79. All the stages are very painful. Stage III. Diagnosis is generally missed. It thrives on gram negative bacteria and cynobacteria at 25°–35°C temperature. 76 Acanthamoebae keratitis was unknown three decades earlier because the organism. It is being reported more commonly among non contact lens users following trauma. hypopyon and sometimes hyphaema. Symptoms at this stage are foreign body sensation. It only produces immediate blanching of conjunctival vessels giving a false impression of white eye. However. 4. The organism is found in two forms i. It is difficult to culture. severe disciform keratitis. Predisposing factors include : 1. all types of contact lenses. which is found normally in human throat and pharynx was thought to be non-infective as far as eyes were concerned. Acanthamoebae keratitis38. formation of double ring. scleritis. Corneal abscess.

3. The scrapped material should be stained with hematoxylin and eosin. Diamidine 1. Propamindine 0. viral and fungal ulcer. Prevention by proper contact lens storage and cleaning.e. lactophenol cotton blue and celluflour white.1% as solution to be instilled for 2 days. Role of local steroids is controversial and better avoided. Cysts have a typical polygonal double walled appearance. It is difficult to culture the organism. It requires constant and prolonged treatment with specific drug. Penetrating keratoplasty seems to be the ultimate method to salvage vision and save the eyeball. the material may be inoculated on blood agar. followed by six times a day for 3 months. Treatment of kerato acanthameba is difficult. Giemsa stain. 3. Instillation of anti acanthamebial drugs. Hexamidine 0.Coli. Penetrating keratoplasty. however. 4.02% solution hourly for first three days then alternate hourly for 2 to 3 weeks. uveitis and secondary glaucoma. Gram’s stain. (ii) Repeated debridement (iii) Local beta blockers to keep the tension low. Anti acanthamebic drugs are : 1. Management consists of76 : 1. The material can be examined after immuno fluorescent stain. Management. Antibiotics—Neomycin 5. The infected corneal button removed from the patient should be subjected to histopathological identification of the organism.1% 2. . atropine sulphate 1% drop two times a day (in children atropine should be used under supervision). 2. Antifungal A. 4. non nutrient agar79a or chocolate agar that has been overlaid with killed E. Local (i) Ketaconazole (ii) Clotrimazole 1% drop B. The frequency is reduced over months. Usual treatment of corneal ulcer. Systemic (i) Ketaconazole (ii) Fluconazole Usual treatment of non healing corneal ulcers consists of : (i) Strong cycloplegic i.DISEASES OF CORNEA IN CHILDREN 209 All suspicious cases of kerato acanthamebae should undergo examination of corneal scrapping to exclude bacterial. Unfortunately herpes simplex keratitis is very common with acanthameba keratitis. result of which may be frustrating. 2.0 mg/drops to be instilled 2 hourly. Biguanides—Polymeric-polyhexa methylene biguanide 0.

D. dystrophic cornea do not get vascularised. Lamellar corneal graft or penetrating keratoplasty or excimer may help to remove the opacities and improve vision. A. Generally there are multiple opacities. They are hereditary in nature of varied mode of transmission. 82 Corneal dystrophies are a group of bilateral. Affecting anterior membrane (i) Affecting epithelium (ii) Affecting Bowman’s membrane (iii) They involve superficial stroma. Anterior corneal dystrophies Commonly seen in children : 1. B. this causes tear film abnormality. There is no known medical treatment for corneal dystrophy. The disorder progresses very little. It has been reported under one year also. however autosomal dominant trait is most common type of inheritance. Recurrence of disease in grafted cornea is known. only few extend to the periphery. generally seen between 3 to 4 years of age. Affecting the posterior membrane i. Exact causes of corneal dystrophies are not known. Descemet’s membrane and endothelium. Use of home made solution should be a taboo. Hereditary juvenile epithelial dystrophy (Meesmann’s). Some of them may be present at birth. Combined Following are a few common dystrophies that are seen in children.210 PEDIATRIC OPHTHALMOLOGY All persons using contact lens should be warned about possibility of contamination by various organism. Corneal dystrophies generally involve central cornea. . If vision is reduced sufficiently to hamper patient’s routine then only lamellar keratoplasty is recommended. loss of vision depends upon position of the opacity in relation to the pupil and density of the opacities. There is no evidence of any other ocular disease in the affected eye. Corneal dystrophies are best classified according to topographic location of the dystrophy. If corneal erosion occurs. Some of the dystrophies have loss of corneal sensation many of them develop recurrent erosion of corneal epithelium.e. All cornea’s 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. In the inter palpebral fissure corneal sensation is reduced. Classification. inflammation and malnutrition are very common. which is rare. Visual acuity is not much reduced the lesions are best seen on retro illumination as micro vescicles in the deeper layers of epithelium. Corneal dystrophies80. They may be seen at any age but most of them start in childhood. The epithelial micro vescicles may raise the epithelium. They should be instructed to use standard cleaning solution. There may be irregular astigmatism. bandage contact lenses may be helpful. non-inflammatory disorder of cornea. This is an autosomal dominant disorder. 81. 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. Affecting stroma C.

Soft contact lens also helps to reduce irregular astigmatism. Loss of vision is severe as compared to other anterior dystrophies. There are two types of this Bowman’s membrane dystrophy i. Patching of the eye at bed time gives much relief to the patient. This can also be done by YAG laser. loss of vision varies between slight haze to severe loss due to total opacification of the cornea. hyperosmotic agents and antibiotic agent. (3) There is vascularisation secondary to recurrent erosion. The opacities are lattice in nature. It starts in infancy. Corneal sensitivity is sub-normal. Corneal sensation remains normal. In childhood vision is good and does not require treatment. photo therapeutic keratectomy may help in removal of opacities. The epithelial surface is irregular leading to irregular astigmatism. The eye may require patching. Granular corneal dystrophy (Groenouw’s I). Recurrent corneal erosion in third and fourth decade is treated with artificial tear. 4. Excimer laser. Excimer laser is also used in case of recurrent corneal erosion. In childhood vision is good that gradually diminish over years. (1) Classical less common variety and (2) More frequent type II. Treatment depends upon degree of visual loss. Management consist of local instillation of lubricating drop and ointment. This is autosomal dominant dystrophy not seen before ten years of age. The condition has autosomal inheritance. This condition is less common than previous two conditions. Corneal sensation is reduced. which is sometimes considered as variant of Messmann’s dystrophy.e. penetrating keratoplasty is preferred. Reis-Bucklers dystrophy. Histochemically the opacities stain with Congo red.DISEASES OF CORNEA IN CHILDREN 211 2. There is gradual increase in corneal opacification that extend from limbus to limbus. 2. Common age of onset is three to five years. Stroma in . Inheritance is autosomal dominant. The lesions may lead to central corneal opacification. Anterior stromal puncture is generally done by fine needle under magnification. Excimer laser photo therapeutic keratoplasty helps to ablate the epithelium. This gives much respite from recurrent corneal erosion. Lattice dystrophy is an autosomal dominant disorder that manifest at the end of first decade or early. The branching opacities may be confused with corneal nerve. Stromal Dystrophies in Children 1. lubricating ointment. This is an autosomal dominant dystrophy. As the lesion is deep. 3. starts in the first decade of life in the central cornea as white granules. The donor cornea may develop opacities after ten years. which can also be achieved by bandage contact lens. Corneal sensation is good initially but gets reduced by third decade. On slit lamp examination the corneal nerves are found to be thickened corneal opacity may require lamellar keratoplasty. Erosion is common. Lamellar or penetrating corneal graft is required if—(1) Vision is much hampered. There is gradual loss of vision. anterior stromal puncture along with bandage corneal lens may help epithelisation of erosion. Lattice dystrophy (Biber-Haab). (2) Lesion has reached anterior stroma. Hereditary anterior membrane dystrophy (Grayson-Wilbrandt). There may be recurrent corneal erosion. In both Messmann’s dystrophy and Stocker and Holt dystrophy. soft contact lens. Hereditary epithelial dystrophy (Stocker and Holt). In severe visual loss lamellar keratoplasty is advocated.

D. congenital corneal edema. diagnosed on routine examination. The needles may come together to form a disc shaped opacity. One unique feature of CHED is increased thickness of cornea. Endothelial dystrophies are only conditions that may have other congenital anomalies of anterior segment. Other associated congenital anomalies should be treated individually. In suitable cases contact lens may help in improvement of vision. It starts in the centre of cornea as needle shaped crystals. No treatment is warranted in childhood. The condition is almost symptomless in childhood. 2. Posterior polymorphus dystrophy. lrisatrophy. They are : 1. This dystrophy is seen under ten years of age. Treatment. There are two forms 1. There is no vascularisation. Definite treatment is keratoplasty in third and fourth decade. It is mostly autosomal in nature. It has been reported at birth. Penetrating keratoplasty may be required after forty years of age. Autosomal dominant.M. Crystalline dystrophy (Schnyder’s). Children do not require any specific treatment. This condition is mostly seen at birth. Congenital hereditary endothelial dystrophy. There are two condition that are seen in childhood. Autosomal recessive. 2. This is present at birth but non symptomatic. Symptoms manifest after fourth decade as irritation photophobia and diminished vision. It is autosomal dominant in inheritance. The condition becomes stationary after third decade.212 PEDIATRIC OPHTHALMOLOGY between the granules is clear hence initially there is no visual disturbance. 3. Posterior Dystrophies in Children Cornea deeper to stroma is less frequently involved in dystrophic conditions in childhood. This dystrophy manifests before ten years. Congenital hereditary endothelial dystrophy (CHED). Most of the children do not require any treatment. . No treatment is required.). when not associated with other anomalies of anterior segment like lridocorneal dysgenesis. progresses slowly but is symptomatic. Corneal sensation is normal. This differs from most of the corneal dystrophies in being autosomal recessive. Posterior polymorphous dystrophy (P. This presents in early childhood. peripheral synechiae. may be present in infants. the condition is almost symptomless. Corneal sensation is normal. correctopia.P. may be present at birth. rarely it may be recessive. does not cause visual impairment. By third decade there is troublesome corneal erosion. Vision may be between 20/40 to CF. Macular dystrophy (Groenouw’s II). 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.

K. CL.DISEASES OF CORNEA IN CHILDREN 213 COMPARATIVE FEATURE OF CORNEAL DYSTROPHY IN CHILDREN Dystrophy Age of onset Heredity Visual loss Corneal sensation Erosion Complication Treatment Anterior Dystrophy Meesmann’s 3-4 yrs Autosomal dominant Mild to moderate Slight reduction ± ± Nil. CL Stocker-Holt Infancy Autosomal dominant Autosomal dominant Mild to severe Severe 6/60 or less Reduced ± Reis-Buckler 3-4 years Reduced Recurrent GraysomWilbrandt 10 yrs Autosomal dominant Gradual loss Normal ± ± Stromal dystrophies Groenouw’s I Lattice Groenouw’s II Macular Granular 10 yrs <10 yrs 10 yrs Autosomal dominant Recessive Autosomal dominant Moderate Severe Mild Reduced ± N ± ± ++ ++ ± Corneal opacity PK Corneal opacity PK Corneal vascularisation Nil Nil Schnyder Infancy Autosomal dominant Mild Normal Nil Posterior dystrophy Posterior polymorphus corneal dystrophy Congenital Autsomal dominant/ recessive Mild N Nil May develop Glaucoma Endothelial Decompensation Symptomatic .B.. Not required. BCL may help in erosion ± Irregular astigmatism L.. BCL Corneal opacity P.K. P.

(See page 248-249 as well) Band keratopathy can be divided into : 1. Primary (rare) 2. congenital anomalies are common predisposing factors. uveitis etc. penetrating injury. Band keratopathy develops in the inter palpebral fissure. it is reported to manifest within two to three weeks following alkali burn. uveitis or systemic disorder like abnormal calcium metabolism. Gradually opacities from each end meet to cover the cornea. Corneal degenerations83.e. 86 PEDIATRIC OPHTHALMOLOGY Degeneration’s of cornea are far more common than dystrophies. Such holes correspond to corneal nerves. pupillary area is last to be affected. Bilateral degeneration are secondary either to bilateral ocular disorder like trauma. Band keratopathy84. Corneal sensation is not disturbed. They are called holes. so is its predilection for exposed part of cornea. The endothelium is not involved. However in rare instance it may spread from centre to periphery. The outer edge of the opacity is sharply defined while the inner edge is irregular and serrated. The opacity is caused by extra cellular deposition of non crystalline salts of calcium carbonate and phosphate at the level of Bowman’s membrane and superficial stroma. It extends across the cornea in a band shaped opacity which starts from periphery and spread towards the centre. The band keratopathy itself does not produce any symptom initially. Exact cause of deposition of calcium at the level of Bowman’s membrane in band keratopathy is not well understood. Secondary to ocular cause B. Band keratopathy secondary to ocular disorder is commonest form of corneal degeneration seen in children. Secondary to systemic cause. They are not genetically predisposed. . It takes few months to years to be symptomatic. In case of band keratopathy secondary to ocular disorders. Commonest type of corneal degeneration seen in children is Band keratopathy. Anterior chamber reaction when present is legacy of primary disorder i. Chronic uveitis. They can start at any age. chemical injuries. 85. Though it takes months to years to develop band keratopathy. There are few areas of deficiency in the opacity. The epithelium shows no change except that it may be irregular due to underlying deposits. serum calcium is within normal range.214 BCL = Bandage Contact Lens CL = Contact Lens LK = Lamellar Keratoplasty PK = Penitrating Keratoplasty PB = Pad and Bandage CO = Corneal Opacity. chronic keratitis. They can be unilateral. The opacity starts as a linear band one millimetre inside the limbus on either side. Secondary A.

Primary band keratopathy. sodium chordrontin sulphate. 90. Use of a chelating agent 2. hereditary. reduction in serum calcium sometimes clear the cornea. The calcium deposit is next scrapped off. Keratoplasty Treatment is indicated only in symptomatic cases. These eyes generally become blind in early life. In presence of active inflammatory lesion or reactivation of lesion. This haze clears within few days without any treatment and is generally not noticed . It has been observed in failed keratoplasty. The aim of chelation is to remove the calcium without scarring of the stroma. Enthusiastic supply of vitamin D in children should be discouraged. The success depends upon presence or absence of effect of primary disorder. phenyl mercuric nitrate.5% EDTA fails to produce desired effect the strength of the solution is raised to 1% or 1. Once the cornea and conjunctiva have been anaesthetised the epithelium is removed by scrapping with any suitable method. glaucoma and trauma reduce the incidence. the stroma preferably under microscope. Use of chelating agent.5% solution of EDTA. In band keratopathy secondary to systemic hyper calcimia. This is rarest form of band keratopathy. Once all the deposits have been removed. However properly managed uveitis. Cornea of a new born is hazier than normal. There is no associated ocular disease.DISEASES OF CORNEA IN CHILDREN 215 Band keratopathy has been reported following use of silicone oil. There are two modes of treatment for band keratopathy 1. Method consists of anaesthetising the cornea with local anaesthetic agent. In latter the calcium is intra cellular and there is an alteration in calcium and phosphorous metabolism. the eye is patched with cycloplegic and local antibiotic drops. Anterior segment ischaemia produces early band keratopathy. Band keratoplasty secondary to systemic disorders are less common than the former.e. sarcoidosis. The condition is X linked recessive trait. the calcium deposit may start again. sodium hyaluronate.5%. Systemic conditions that cause band keratopathy are—Vitamin D toxicity. A lamellar keratectomy or excimer laser photo therapeutic keratoplasty are better options than chelation. a to-and-from movement helps to remove the deposits better. Topography of the opacity is similar in both conditions. hyper parathyroidism. Similarly discontinuation of vitamin D. Treatment of band keratopathy87. This is applied on the exposed calcium deposit. Corneal opacity in new born and infants88. Is it a degeneration or dystrophy ? as there is overlaping of characteristics of both conditions. Treatment of band keratopathy will restore vision only if the cause of defective vision is corneal. A Week-cel sponge is soaked in 0. retina may also be involved. There is lack of unanimity regarding its nature i. present at birth or early childhood. In children the procedure should be done under general anaesthesia. Band keratoplasty secondary to systemic disorder. Drawback with excimer laser treatment is that it can cause post operative undesirable hypermetropia. choroid. chronic renal failure. milk alkali syndrome. If 0. There is no known prophylaxis. uremia. Besides cornea other parts of eye like lens.

Interstitial keratitis is a common cause of bilateral corneal haze but seen only after third year of life with redness. Normal tear has three layers of different chemical composition and each has specific purpose. that is generally preceded by xerophthalmia. It is sandwiched between lipid and mucin layer. the mucin layer anchors the aqueous layer to the cornea and the . However a child may be born with corneal haze sufficient to be called opacity. However term xerophthalmia93 is generally used to denote dryness of conjunctiva and cornea due to vitamin A deficiency. A child may have bright and clear cornea at birth but later becomes hazy. Tear in Descemet’s membrane 3.216 PEDIATRIC OPHTHALMOLOGY by mother or nurse unless looked for. Commonest cause of unilateral haze is birth trauma. 93 Logically the term xerophthalmia should include all the conditions that produce non wetability leading to dryness of ocular surface i. Xerophthalmia and Keratomalacia91. Congenital hereditary. 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.e. cornea and conjunctiva. Corneal haze in new born may be unilateral or bilateral. Tear is essential to keep the cornea and the conjunctiva bright and moist. Endothelial defect Mucopolysaccharidosis Anterior chamber cleavage syndrome 1. Congenital hereditary endothelial defect (CHED) 2. The layers are—Lipid. stromal dystrophies 7. Congenital rubella 3. 2. Posterior polymorphs dystrophy 3. Aqueous layer is voluminous when compared to other layers. Lid anomaly (Neurotrophic) 4. Corneal 2. Commonest cause is buphthalmos. Buphthalmos 1. Dermoid 1. Sclero cornea of various degrees. Ulcer (keratitis) 1. Birth trauma 2. Buphthalmos that is more advanced in one eye may also produce unilateral hazy cornea. Common causes of corneal opacity are : 1. Metabolic 5. Deficiency of any layer will lead to tear film abnormality. aqueous and mucin. Posterior corneal defect 6. Keratomalacia93 denotes softening and aseptic and necrosis of cornea due to deficiency of vitamin A. The lipid layer prevents excess evaporation of aqueous layer and gives a shinning surface while. Limbal The above conditions are acronym as STUMPD89 with beginning alphabet of each cause. Herpes simplex 2. photophobia and lacrimation. 92.

Retinol combines with protein in rods and act as light stimulus. that is of animal origin found in fish. the conjunctiva is thrown into three to four vertical folds in the intra palpebral aperture. Prexerosis. yellow fruits. There is degeneration of Bowman’s membrane infiltration of stroma by inflammatory cells and fluid. Increased loss or 3. duration. Lack of mucin hasten tear break up and delayed bridging of the gap. Reduced storage in liver. which is reservoir of vitamin A. There is increased pigment deposit on the conjunctiva that appears as dusty white patch. Reduced consumption 2. Retinol besides maintaining health of epithelial cells of conjunctiva also plays an essential role in night vision. Deficiency of vitamin A responds quickly to administration of water soluble vitamin A by intra muscular injection within twenty to twenty four hours. associated secondary infection of the eye and systemic infective diseases like pneumonitis. The retinol stored in liver is released in blood stream as retinol binding protein. gastro enteritis. It is found mostly in green leafy vegetables. Conjunctival xerosis. Normally the conjunctiva is not thrown into vertical folds when the eye is rotated but in case of dry conjunctiva. Night blindness sets in when serum vitamin A level falls below 50 IU/L. Perforation. An infant requires five times more than an adult. meat. lack of mucin causes abnormal break up time resulting in formation of large dry spot on the ocular surface. hence systemic protein deficiency may precipitate xerophthalmia if it is not corrected. 5. 3.DISEASES OF CORNEA IN CHILDREN 217 conjunctiva. exciting the optic nerve in scotopic vision. In hypo vitaminosis A there is hyposecretin from goblet cells of conjunctiva. Most commonly used classification is that given by WHO94 that divides xerophthalmic signs into two broad groups i. Retinol is fat soluble vitamin A. This may happen in : 1. Clinical features. Keratomalasia. Each group has been divided into three sub groups : . Corneal xerosis. 4. It is involved in breakdown and resynthesis of rhodopsin. The aqueous and lipid layers are unaffected. Vitamin A is essential for maintenance of conjunctival goblet cells that secrete mucous. primary and secondary signs. The exact mechanism of dryness of cornea is not understood. Carotenes are precursors of vitamin A. Xerophthalmia has been classified variously91 1. There is thickening and loss of elasticity of conjunctiva. Clinical features depend upon severity. 2. 4. red palm oil. it may be keratinised which leads to opacification of conjunctiva. 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. poultry and milk. Vitamin A is involved in photo chemistry of night vision. Daily requirement of vitamin A varies with age. The carotenes are converted to retinol in the small intestine from where it is absorbed and transported to liver. measles etc.e.

vice versa is rare. The triangle has its base towards the cornea and apex towards outer canthus. Dryness of conjunctiva 3. Bitot’s spot. The foam is caused by action of xerosis bacilli. The stroma is involved following break in epithelium. Conjunctival and corneal 2. 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. Night blindness. surrounded by hazy or opaque cornea. These early changes are reversible and respond promptly to systemic administration of vitamin A. tear film break up time is less than 10 seconds.218 Primary signs96 X X X X 1A 1B 2 3A 3B XN XF XS Conjunctival xerosis Bitots spot with conjunctival xerosis Corneal xerosis Corneal ulcer with xerosis Keratomalacia Night blindness Xerophthalmic fundus Xerophthalmia scar PEDIATRIC OPHTHALMOLOGY Secondary signs Where X stands for xerosis Symptoms of xerophthalmia can be related to 1. abnormal tear break up is due to changes in corneal epithelium. Tear production and composition of tear is within normal limits. Conjunctival—Vertical folds on looking laterally 2. Cornea develops dry spots. Bitot’s spot is not specific of vitamin A deficiency. the foamy substance can be removed with ease only to reappear. Bitot’s spot due to vitamin A deficiency disappears after sometimes but idiopathic Bitot’s spot does not respond to administration of vitamin A and is seen in older children and adults. There is minimal congestion and hardly any pain. The surface of the triangle is foamy. Bitot’s spot94. If the condition is not arrested at this stage it proceeds to corneal ulceration which is generally central. The stroma is involved more easily and severely in vitamin A deficiency . It can be idiopathic as well. 2. Clinical signs 1. One may precede the other or may be found together. Pigmentation on dry conjunctiva 4. Peculiarity of these ulcers is that they are aseptic in nature. The surface of Bitot’s spot does not wet with tear. The cornea is lusturless. Corneal signs. There are instances where the eye may advance into conjunctival and corneal xerosis without night blindness. Kajal and Surma applied to lid margin do not stain normal conjunctiva but the surface of the Bitot’s spot and dry conjunctiva stains with Kajal and Surma.

Night blindness due to vitamin A deficiency is of acute onset. The first dose may be delayed up to nine months and given along with measles vaccine. Night blindness due to vitamin A deficiency responds to systemic vitamin A therapy quickly. It is very common following attack of measles.000 unit of vitamin A concentrate. one eye may be more advanced than the other. Every child between six months to six years should get an oral dose of 200. . However administration of vitamin A along with management of measles has reduced mortality in measles. Scar following keratomalacia is generally bilateral. Therapeutics Prophylaxis98. Management of xerophthalmia97. Night blindness. hence soon after birth the child should be put to breast of the mother and allowed to take the collustrum. In developing countries with eradication of small pox. Prophtlaxis 2. government hospitals. 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. The child is so incapacitated that he may not locate his food in the plate placed in front of him. If the child gets sufficient amount of mothers milk and thrives on it. This has been termed as chicken blindness. there is no need of any supplementation of vitamin A. Under National Programme of Prevention of Xerophthalmia. Perforation with protrusion of intraocular tissue is inevitable. Keratomalacia. This has not been produced in experimental animals. The child prefers to sit in one place and avoid moving. It is invariably associated with protein caloric malnutrition. malaria.DISEASES OF CORNEA IN CHILDREN 219 than seen in normal eye when epithelium is damaged. mal-absorption syndrome. Prophylaxis in mother and new born. It is not clear if vitamin A deficiency precipitates attack of measles or vice a versa. Vitamin A prophtlaxis between 6 months to 6 years. Vitamin A is stored in liver and secreted in mother’s milk in sufficient quantity to prevent deficiency in infants. 98 Management consists of : 1. worm infection. Hence it is of utmost importance that mother has good storage of vitamin A during pregnancy and lactation. At this stage vigorous local and systemic treatment may avert perforation but complete recovery with clear cornea is never achieved. The central part is covered by a white gelatinous material due to collagenase enzyme. From the age of five months solid food should be started in consultation with pediatrician. yellow fruits and vegetables to cereals and pulses. adding green leafy vegetables. This is advanced stage of xerophthalmia. The child should be breast fed as long as there is sufficient mother’s milk for the child. The colostrum is rich in vitamin A. vitamin A is distributed free from primary health centres. Here the cornea becomes soft and virtually melts away within twenty to twenty four hours. If top feed is to be started bovine milk is preferred over formula milk. diarrhoea. Xerophthalmia due to vitamin A deficiency is never isolated. keratomalacia has become foremost cause of bilateral corneal staphyloma in children.

000 IU of oral vitamin. If the child is unable to take oral feed or has vomiting. Children and young adults should be encouraged to take vitamin rich food as part of daily diet in the form of green vegetables.000 IU orally. There should be adequate supply of other vitamins and minerals specially the trace elements. 100.000 IU orally 3. Atropine—In all cases of corneal involvement irrespective of actual ulceration atropine is used as 1% ointment two times a day.000 IU oral after seven days or (2) Repeat 100. After seven days—200.220 PEDIATRIC OPHTHALMOLOGY Fortification of food with naturally occurring vitamin A.000 IU of vitamin A followed by (1) 200. Immediate upon diagnosis . Local retinol—Tretinoin 0. 2. C.1% as drops in oil three times a day is claimed to retard progress of corneal involvement. Ocular therapy A. Keratomalacia WHO recommendation : 1. Thus one drop in each eye reaches almost lower level of toxicity especially when the child is under weight. Next day—200.000 IU orally. Daily requirement of vitamin in normal individual : 1. Local artificial tear may help in preventing the cornea from drying.200.000 IU of water soluble vitamin A is given as injection. The child’s total protein and calories should be calculated and supplied because vitamin A deficiency does not occur in isolation. Intra muscular injection of water soluble 100.000 IU every six months up to six years of age to prevent recurrence. Atropine as drop is better avoided in children for one drop of atropine contains more than therapeutic dose of atropine. Xerosis—200. School children—2250 IU Management of clinical xerophthalmia : Night blindness99. . 5. Local tear substitutes—Though aqueous part of tear film is not reduced yet it fails to wet the cornea and conjunctiva due to changes in epithelium. 4. yellow fruits. yellow tubers. B. Children less than 8 kg should receive half the above dose and under 4 kg quarter of the above dose. Pregnancy and lactation—3000-3500 IU (300 mg to 750 mg) 2. D. Local instillation of antibiotic drops every 2 hourly and ointment during sleep to prevent sticking of lids.000 IU intra muscular after seven days. 0-4 years—1000 to 1200 IU 3. Repeat 200.

2002. Third edition p-3-25. Duke Elder S. Sanders D. p-165. First edition. Lippincot Williams and Wilkins. proptosis. and Thoft R. 2003. Modern Publishers. London. Lippincott Williams and Wilkins. Ilene K. 1963.A. cornea and limbus in The cornea. 1963. Edited by Smolin G. First edition. edited by Pyeman G. Khurana A.A. 5. : Applied anatomy of the eye and adnexa in Modern ophthalmology. : Development of eye and its adnexa in Anatomy of the eye and its adnexa.A. New Delhi. Second volume. p-20-21. 2000. Oxford. 12. Nineteenth edition. 2000. Second edition. Vol. : Physiology of the eye in Essentials of ophthalmology. Ramanjeet Shihota and Radhika Tandon : Metabolism of ocular tissues in Parson’s diseases of the eye. 11. New Delhi. London. Edited by Smolin G. Vol. III. 1991. 2000. Jay Pee Brothers. p-105-1056. Second edition.V. : System of ophthalmology. Nema H. Part II.. p-360. p-24-25. : Diseases of the cornea in Ophthalmology. 14.R. New Delhi. .. lagophthalmos. : Development of cornea and sclera in Anatomy of the eye.368. Madurai. Geroski D. Current Books International. acid alkali burn. 8.. and Ubels J. 3.P. Third edition.R. 1991. 4. 6. Sander D. New Age International. ectropion of lid. p-28-32. Duke Elder S. : Corneal transparency in The Cornea. Basak S.S. Second edition. p-137.. 1st edition. Edelhauser. Philadelphia. 9.K.L. Lippincot Williams and Wilkins.C. p-164-169. Second edition. Philadelphia. Deborah Pavan Langston : Cornea and external eye disease in Manual of ocular diagnosis and therapy. Nema H.H. Riley Day syndrome. p-18-19. Meharotra A.A. New Delhi..F. 7. and Thoft R. First Indian edition. p-3-9. Edited by Pyeman G. Jay Pee Brothers. Calcutta. 2. : The cornea and limbus in Anatomy of the eye and its adnexa. 1999. 1987. REFERENCES 1. : Corneal physiology in Principle and practice of ophthalmology. Part I. : Anatomy and embryology in Principle and practice of ophthalmology. Banumathy S. Henry Kimpton. Sharma P. Henry Kimpton. and Goldberg M. 15. Edited by Dutta L. New Delhi. p-114-115.DISEASES OF CORNEA IN CHILDREN 221 Other causes of xerosis.F. : System of ophthalmology. Steven-Johnson’s syndrome. Doughman D. Butterworth Heineman. H. Jay Pee Brothers. Coloboma of lids. Fifth edition 67-68.F..K. Nancy Anderson Hamming and Apple D. Jay Pee Brothers. 1994. 1987. Arvind Eye Hospital.V. 13. New Delhi. First Indian edition. : Cornea and its diseases in Essentials of ophthalmology. Gipson : Anatomy of the conjunctiva. Jay Pee Brothers. 10. III. 1994. p-129. New Delhi. and Goldberg M.

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Philadelphia. Duke Elder S. New Delhi. Singapore.T. Jay Pee Brothers. and Yee R. 1965. New Delhi. : Fungal keratitis in Current ocular therapy. and Roy H.. I. 1995. and Roy H.P.J.T. : Inflammation of the cornea in System of ophthalmology. 1987. Fourth edition. Vol. Edited by Duke Elder S.T. 48. Fifth edition. 2000. 40. p-152-153. 38.W. p-360-361. 1965.G. Ltd.. 1998. Part II.C. Jay Pee Brothers. 46. W. New edition p-207-216.N.A.DISEASES OF CORNEA IN CHILDREN 223 34.T. 1993. and Roy F. Saunders and Company. Edited by Duke Elder S.B. p-58-59. Coster D. Vastine David : Corneal ulceration : Classification. Saunders Company. Harcort Brace. Saunders Company.B. Fourth edition. 1998.T. Duke Elder S. Ostler H. Henry Kimpton. Lippincot William and Wilkins. Dhanda R. and Roy F.F. and Leigh A. Edited by Heuven WAJ and Zwaan J. Vol. 45. Galgotia Publications Pvt.G.R. Roberson M. signs and symptoms in Principle and practice of ophthalmology. p-492-493. New Delhi. VIII. : Corneal scar in System of ophthalmology. Philadelphia. O’Day D. Second edition. Philadelphia. Edited by Fraunfelder F. London. Henry Kimpton. Fifth edition. First Indian edition.F. Philadelphia. and Leigh A. 35. Galgotia Publication Pvt. Ltd. : Infected corneal ulcer in A Text Book of Ophthalmology.T. 36. Henry Kimpton.H. 44. Dhanda R. Singapore. Yee R. : Marginal corneal ulcer in Decision making in ophthalmology. London. p-345-346. p-154-155. Edited by Heuven WAJ and Zwaan J. W.B. Philadelphia. : Central corneal ulcer in System of Ophthalmology. Whitcher J. O’Day D. W.R. London. edited by Faunfelder F. Duke Elder S. p-777-800.B. Part II. p-198. 41. Harcort Brace. Saunders Company.T. p-258-264. : Fungal keratitis in Current ocular therapy. Sander D. Reddy M. Vol.P. edited by Fraunfelder F. 1994.. : Corneal scars in A Text Book of Ophthalmology.C. Shields M.M. New First edition. Fifth edition. VIII. Liegner J.B. 2000. and Eileen M : Burd Fungal keratitis and conjunctivitis in The Cornea. and Kalever V. Philadelphia.B. 8. Edited by Smolin G. 39. Edited by Peyman G. Part II.B. Edited by Dutta L. 1993. 1998. 1st Indian edition. . and Goldberg M. Vol. Third edition. I. p-639. Edited by Fraunfelder F. p-285-294.. 42.P. 2000.F. Williams and Wilkins. 1967. p-729-860.J. New Delhi..W. : Central corneal ulcer in Decision making in ophthalmology. : Pneumococcus in Current ocular therapy.M. W. 43. : Bacterial corneal ulcer in Current ocular therapy. p-229-252. Savitri Sharma and Rao G. p-200-214. First Indian edition. and Cheng C. 47. Edited by Duke Elder S. 2000.H. and Thoft RA. 37. and Kalever V. and Cevallos V. and Leigh A. : Corneal ulcer in Modern ophthalmology. and Badenoch P. : Congenital glaucoma in Text Book of glaucoma. Vol.

: Fungal keratitis in Current ocular therapy. Edited by Samolin G. Edited by Smolin G. 45 : 203-210. 1998. Third edition. Usha Gopinath.K. Jay Pee Brothers. : Penicillin resistant streptococcus pneumonae keratitis. Whitcher J. Jr. W. 65. Thoft R. p-99-101. 63. P. 64.T. 61.. Savitri Sharma. : Adenoviral ocular infection in The Cornea. New Delhi. Deborah Pavan Langston : Herpes simplex virus kerato conjunctivitis and iritis in Manual of ocular diagnosis and therapy. 62. Jr. 1994. 2000. : Herpes Simplex Keratitis. and Thoft. and Margolis T. Butterworth. 53. p-183-208. First edition..D. Lippincot Williams and Wilkins. Saunders Company. Malouf D. 1989. and Tullo A.B. and Thoft R. 56. 31-35. Cornea 15 : 99-100. : Dendriticular in Clinical ophthalmology. Oph. Forster R. : Management of herpes simplex keratitis in Diagnosis and management of ocular inflammation. Edited by Harley R. Third edition. 1999. : Diseases of the Cornea in Paediatric Ophthalmology. Vol. p-490-498..R. Edited by Gittinger J. : Ophthalmic antiviral chemotherapy.P. Saunders Company. Philadelphia. p-215-225.W. Fifth edition.A.K. Laibson P. Little Brown and Company. 1983. W. cifazolin and tobramycin in Bacterial keratitis. 58. Ind. p-54-62. Second edition.P. 1988. Ind. Philadelphia. Paed. and Clin. 57. Oph. p-89-92.C. Philadelphia. and Asdourian G. Edited by Fraunfelder F.W.S.K. 60. Edited by Jain M. 54. Lippincot Williams and Wilkins. Philadelphia.T. Fifth edition. Jr. Mehta P. 1997. Wilkins J. and Roy H. Philadelphia.. Edited by Jain M. 1994. Oph. : Herpes simplex keratitis in Manual of clinical problems in ophthalmology. Saunders Company. 31:332-335. 1996. An overview. 1995. Lippincot Williams and Wilkins. London. Oph. and Oates R. Second edition.. : Fungal keratitis and conjunctivitis in The Cornea. Ind. p-239251.J. health. Gittinger J.B. p-360-361. Edited by Samolin G. Agrawal W. : Herpes simplex virus infection in neonate.. Efficacy of ofloxacin vs. 51.B.A. and Waring G. : Bacterial keratitis study research group. Arch. Jr.P. W. Missotten L.S. Philadelphia. Laibson P. Silverberg M. 50. Garg P. 52. p-63-65.R. and Roy F.. . Suresh P. New Delhi. 55.224 PEDIATRIC OPHTHALMOLOGY 49. Op.F. 2002. and Rao Gullapalli N. p-61-63. 113:1257-1265. Kanski J. 1990.J.J. Boston.B. O’Day D.A.. 1994.. Athmanathan S. and Naduvilath T. Jay Pee Brothers.P. Mudgal P.R.M. p-46-47. I. 1990.R. 2000. : Early diagnosis of mycotic keratitis predictive value of potassium hydroxide preparation.. 59. Philadelphia. Lippincot Williams and Wilkins. : Antiviral agents in Diagnosis and management of ocular inflammations.H. 47 : 155-165. Edited by Fraunfelder F. Gorden J. Deborah Pavan Langston : Herpetic infection in The Cornea. 1995. : Pseudomonas aeruginosa : In Current ocular therapy. Third edition. Second edition. Fifth edition. O’Brien T.

Arffa R. 1985. VIII. Second edition. Annl. Edited by Smolin G. : Acanthameoba keratitis.. Part II.A. 1995.B. Jr. Ind. p-28-32. Part II. Robin J. Diag. W. Dart J. 100: 719-723. and Leigh A. Edited by Fraunfelder F. Srinivasan M. Edited by Smolin G. and Sachdeva M.K. 1990. Vol. 1990. Sandramoulis. New Delhi. 193-199. Philadelphia.H. 1993. B. W. Duke Elder S. Surv. and Kaufmann H. 1994.. Wilhelmus K.C. and Leigh A. 2002. and Roy F. and Dutta L.K.T. p-502-506. 72.P. Saunders Company.K. 79. Angra S. 1992. Edited by Duke Elder S. 68. p-815-830. : System of ophthalmology. Kimpton.. Henry Kimpton. and Thoft R. Edited by Harley R.C. p-89-90. I.. Acanthamoeba keratitis in The Cornea.E. p-2-5. New Delhi. Edited by Fraunfelder F. Stuphin J. and Thoft R. Kirupa Shankar M. : Clinical features of acanthamoeba keratitis. 1983. and Roy F.R. comparative evaluation of direct microscopy and culture results..DISEASES OF CORNEA IN CHILDREN 225 66. Philadelphia. and Dunn J. . Barua C. 1991. 8.S. Saunders Company. Third edition p-144-149.. 75.B. 69. Fifth edition. 1994..A.G. : Peripheral corneal ulcers in Modern Ophthalmology. 77. : Ocular syphilis. Vol. p-238-241. 82. 14 : 219-224.K.D. New Delhi.R. Vol.H.H. p-262-266. and Hyndiuk R.. Edited by Dutta L. and Pandey S. : Acquired and congenital syphilis in Current ocular therapy.P. Jain M.P. Ophth. Seal’s Textbook of ophthalmology 5th Ed. 79. and Waring G. Jay Pee Brothers. Oph. microbio. 67.O. Third edition. Duke Elder S. 80.S. 1965. I. 76. Avni I. : Laboratory diagnosis of keratomycosis. and George C. 70. Philadelphia. Jay Pee Brothers.G.E. Vajpayce R. 81. Edited by Duke Elder S. London. 2000. Henry. : Acanthamebiasis in Current ocular therapy.T. Laibson P. and Hamed L. : Use of lactophenol cotton blue mount of corneal scrapping as an aid to the diagnosis of mycotic keratitis. 1994. current book int Kolkata. London. Vol. Lippincot Williams and Wilkins.J. Honavar S. Jay Pee Brothers. I. 2000.G. Second edition. Philadelphia.C.B.A..M. Lippincot Williams and Wilkins.E. Margo C. Jr. 8 : 1. 2000. 73.G. 2000. : Corneal dystrophies in System of ophthalmology. Shalaby A. : Keratomycosis and antifungal drugs in Diagnosis and management of ocular inflammation. Fifth edition.R. Second edition. Am. Kuriacose T.A. : Calco flurol ink potassium hydroxide preparation for identifying fungi. 38:50-56. Oph. second edition.A. and Chabra V.. Sharma S. p-162-163. Thomas P. 74. Ogawa G. 25 : 68-71. Community eye health. 1965. 309:273.M. 71. Oph. : Acanth amoeba keratitis in G. Singh S.K.. Seal S. Ram J.K. : Diagnosis of acanthamoeba keratitis. Vol. p-921-932. 37 : 207-220. : Non ulcerative keratitis in The Cornea. : Corneal dystrophies and degeneration in Pediatric Ophthalmology.N. Aschibashiy. Philadelphia. : Corneal degenerations and dystrophies in Modern Ophthalmology.S. and Maharajan V. 78.

Edited by Duke Elder S. Part II. London.R. : Morphological and pathological response of cornea and conjunctiva diseases in The Cornea.B. 1965. Tran D. Zwaan J. and Asbury T. Philadelphia. Henry Kimpton. First edition. 94.V.F. 1982. : Corneal and conjunctival calcification in Current ocular therapy. W.J. WHO : Vitamin A deficiency and xerophthalmia. 1965. Edited by Peyman G. p-128-129. 2000. Philadelphia 2002. Liabson P.. Doughman D. Lancet 1:1169173. 88. p-414-419. Jay Pee Brothers. p-1115-1125. : Corneal ulcer due to vitamin A deficiency in General Ophthalmology. I. p-288. : Hypovitaminus A in Current ocular therapy. 95. p-114-116. 1983.M. 2000. Philadelphia.D.. 98. Fifth edition. : Corneal dystrophies and degeneration in The Cornea.. Saunders Company.S. 1983. New Delhi. Fifth edition. 93. : Xerophthalmia in Sight and life manual of vitamin A deficiency. Third edition. Philadelphia. London. Tenth edition. Peterson R. Second edition. : Xerophthalmia in Essentials of ophthalmology. Lippincot. Philadelphia. Duke Elder S.O. Kenyon K. 87. Edited by Vantteuven W. Edited by Fraunfelder F. Basel. and Boger W. Edited by Smolin G. : Pediatric ophthalmology in Manual of ocular diagnosis and therapy.Brart D..A. p-97. VIII. California. 590. Lippincot Williams and Wilkins. I Edition II.AJ and Zwaan J. Henry Kimpton.. 1976. W. 1994.R. Oxford University Press. and Frigg M. p-893-902. p-329-330. Vol. p-51-62. and Chaves H. Vol. VIII.. O. Sommer A. Sommer A. Third edition. and Schanzlin D.G. 2000. and Waring G. 90. . Modern Publishers.P. W. 86. 1994. and Leigh A. 1993. Thoft R. p-71-78. Saunders Company. Philadelphia. and Gartry D. p-456-490. 84.A.T. p-499-508.. Saunders Company. First Indian edition. New Delhi. Lippincot Williams and Wilkins. Duke Elder S. 2001. Sharma P. Samolin G. Edited by Harley R.A. H. Edited by Deborah Pavan Langston. : Diseases of the cornea in Pediatric ophthalmology. Vol. : Cloudy cornea in a neonate in Decision making in ophthalmology. p-107-108. 92. Edited by Duke Elder S. and Thoft R. Part 2. : Impact of vitamin A supplemention of childhood mortality. 96. Williams and Wilkins.T.226 PEDIATRIC OPHTHALMOLOGY 83. Vol.B. Edited by Samolin G.R. : Treatment of band keratopathy by excimer laser photo therapeutic keratectomy. and Goldberg M.. 1987.P. Published by Task Force Sight and Life.D. and Roy. : System of ophthalmology. 91. McLaren D. : The cornea in Principle and practice of ophthalmology. 89. First Indian edition. : Xerophthalmia and keratomalacia in Nutritional Blindness. 1992. Leigh A.G. 99. 77 : 702-708. Sander D. Technical report No.S. Harcort Brace. Lange Medical Publication. Fifth edition. : Band shaped keratopathy in System of ophthalmology.B. Vaughan D. B.S.A.O.J. 85. Sommer A.J. 97.

The ciliary zone is a wider zone that extends from collarette to the root of the iris. It is divided into two zones by a circular raised irregular lines called the collarette i.1 1. It is divided into three parts. It is 12 mm. The iris divides the aqueous chamber into anterior and posterior chambers. It is relatively rough due to the presence of series of radial elevated ridges that 227 . It’s thickness is maximum at the collarette. all round with a circular hole in the center the pupil. darker is the iris. Thickness of the iris is not uniform. Some of the stromal vessels are visible in the iris of the new born. More the pigment. is lined by a thin zone of pigment called iris ruff. which disappear after a few weeks. Pupillary zone that is 1.2 Heterochromia is generally congenital in nature. In albinism the iris is generally bluish. Ciliary body 3.e. It is practically a diaphragm of blood vessels and unstripped muscle fibres. from root to root. It is coronal to the cornea but not parallel to it. Colour of the iris of the new born is lighter than that of adults. Difference in the colour of the iris in two eyes is called heterochromia. Choroid. The anterior surface of the iris is rough. Iris 2. but also from person to person. 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. then its thickness gradually diminishes towards the pupil in a rounded fashion. It may vary in two eyes or in the same eye. The iris Iris is the most anterior part of the uvea that is visible on oblique illumination.5 mm. The colour of the iris not only varies from race to race. almost translucent that transilluminates with reflected light. in diameter. held together by loose stroma. 1. Colour of the iris is due to the presence of pigments in the iris. The pupillary zone contains the sphincter pupillae muscle that is a smooth muscle and originates from the neuroectoderm and is supplied by parasympathetic nerve. It is thinnest at its insertion (root) to the middle of the anterior border of the ciliary body. which is in fact continuation of the posterior pigment layer. All the three parts develop from neuro ectoderm and mesoderm. wide from the collarette to pupillary margin.CHAPTER 8 Disorders of the Uvea in Children Uvea is the middle vascular pigmented coat of the eye ball. 2.

lymphocytes and mast cells. it is innervated by cervical sympathetic nerve. 3. Posterior epithelium. The dilator pupillae arises from the anterior epithelial layer. 2. The Anterior limiting layer is a modification of superficial layer of stroma1. The unstratified muscles are: 1. Direction of the fibres in this zone is radial. Some authors consider that what was thought to be endothelium of the iris is in fact a layer of condensation of anterior’s limiting layer. 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. Now it is presumed to be distributed in patches. 2. oculomotor and sympathetic. it comes in close proximity of the posterior pigment epithelium. (ii) Stroma proper.5. The dilator pupillae lies in the ciliary zone of the iris. A part of it represents outer wall of the optic cup. The detachment of the iris from the root is called iridodialysis.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. (i) Anterior limiting layer. Microscopically the iris is divided into: 1. The nerves present in the stroma are—branches of trigeminal. The vessels in the iris are nonfenestrated. is flatter than the anterior surface.228 PEDIATRIC OPHTHALMOLOGY represents radial blood vessels in the stroma. unstratified muscles. width that encircles the papillary border. It is the forward extension of pigment epithelium of retina and ciliary body and is commonly referred to anterior epithelium. The posterior surface though not very smooth. In blue coloured iris there is less pigment and in dark coloured iris there is more pigment in this layer. Endothelium. Nerve endings and capillaries are present in this layer. The endothelium. it is a narrow strip of about 1 mm. fibroblasts. The pupil continues to constrict even in presence of iridectomy. hence not suitable for ordinary fluorescein angiography. (ii) Stroma proper. Amount of pigment and the thickness of this layer is responsible for the colour of the iris. Stroma. fibroblasts and collagen fibres.5 This layer consists of melanocytes. This is the place where neovascularisation of iris begins. The stroma has been divided into: (i) Anterior limiting layer. Traditionally it was thought to be an extension of the cells that line the posterior layer of cornea. The presence of endothelium is controversial4. 1. The posterior surface is dark brown or black due to the pigment in the epithelium. . The function of the muscle is to constrict the pupil. The peripheral crypts communicate with the anterior chamber. The posterior surface rests on the anterior surface of intact lens and glides smoothly over the lens capsule. Besides the ridges there are scattered depressions that are formed due to lack of superficial layers of the iris. nerves. The sphincter pupillae is present in the pupillary zone. 3. It is supplied by the parasympathetic fibres of the third nerve. 2. The endothelial layer is absent over the crypts. Stroma proper consists of blood vessels. Its function is to dilate the pupil. it lies in the posterior part of the stroma. it is more bulky than the sphincter. It is two layered. Posterior epithelium.

while those that dilate are called mydriatics. The ciliary body is empirically divided into two parts: 1. to ora serrata posteriorly. away from the limbus. which is of no clinical significance. In section it looks like an isosceles triangle. An appreciable flicker of pupil is called hippus. . Pars plana. The pigment epithelium acts as blood aqueous barrier besides preventing light from entering the eye. The drugs that constrict the pupil are called miotics.e. A deficiency in iris is called coloboma that can be congenital or acquired. Presence of more than one pupil in each eye is called polycoria. 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. It acts as communication between anterior and posterior chamber through which aqueous passes from the posterior chamber to the anterior chamber. The outer edge of the base is attached to the scleral spur. dysgenesis of anterior chamber or may be acquired following trauma which is mostly surgical or may be accidental. The ciliary body This is the middle part of the uvea. Generally the size of the pupil is equal in the two eyes. In some chronic uveitis.5 mm. It’s colour is brownish black. The posterior part forms the boundary of the posterior chamber. while larger than the normal is called mydriatic pupil. the base of which points towards the pupil.DISORDERS OF THE UVEA IN CHILDREN 229 The posterior epithelium is the anterior extension of non pigmented epithelium of ciliary body and merges with the anterior epithelial layer. It is circular in shape. It extends from scleral spur anteriorly. From the middle of the base arises the iris dividing the base into two parts. The size of a normal adult pupil is 2 mm. 1. The function of the pupil is to control the light entering the eye. Pupil in true polycoria should have independent sphincter and dilator muscles. both blunt and penetrating. This is called ruff of the iris. This part is visible on gonioscopy only. The ciliary body is highly vascular and bleeds profusely on trauma. The pupil The pupil is not a tissue. this is generally bilateral and congenital. Such pupil are capable of reacting to light and accommodation to a limited extent. Normally the pupil is never stationary and its size keeps on changing within a normal range. with change of intensity of light and accommodation. Pars plicata and 2. there may be loss of iris tissue resulting in holes in the iris. it is a deficiency in the iris. Pseudopolycoria can be seen as congenital anomaly i. to 4 mm. Pupil smaller than normal is called miotic pupil. It is very common for the coloboma of the iris to extend up to pupil resulting in a vertically enlarged key hole shaped pupil. It encircles the interior of the sclera. It is smaller in new born and in old age. Corectopia is a term used to denote the position of the pupil other than normal. which is a congenital condition. the anterior part forming the angle of the anterior chamber. Difference in the sizes of the pupil in two eyes is called anisocoria.

x 2. It extends from optic nerve to the ora serrata between the sclera on the outer side and retina on the inner side. 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 Pars plicata is anterior 2 mm. the other function of the ciliary process is to secrete aqueous. The pars plana is thought to secrete mucopolysaccharides of the vitreous. The vascular layer of the pars plana is similar to that of choroid without choriocapillaries. The ciliary processes are made up of capillaries covered by two layers of ciliary epithelium. This space is traversed by ciliary vessels and nerves. Pars plicata. This is the thickest part of the uvea. ciliary muscles. Its thickness is not uniform throughout. The muscles are nonstriated muscles that has three parts. 2. in size. The lens and the zonules together form a barrier that separates the vitreous chamber from the aqueous chamber. The most important are the longitudinal fibres. Contraction of the ciliary muscles open up the angle of the anterior chamber to facilitate aqueous out flow. of the ciliary body. From the tips of these processes arise the zonules of the lens. The suprachoroidal space extends anteriorly under the ciliary body as supraciliary space. 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.230 PEDIATRIC OPHTHALMOLOGY 1. These are known as ciliary processes. that are responsible for accommodation. the ciliary muscles take a major part in accommodation.00 mm. of the ciliary body. The sensory supply is via trigeminal. . Sympathetic supply has lesser role to play in the ciliary body. The exact function of the ciliary muscles are not well understood. 3. The ciliary muscles form the main bulk of the ciliary body. of pars plana. The choroid can not be separated at the exit of the vertex veins and round the disc due to firm attachment. in finger like processes. Besides acting as attachment of zonules. their length depends on tightness of the ciliary muscles3.25 mm. in thickness. and anteriorly 0. The ciliary epithelium acts a blood aqueous barrier. The separation is more pronounced anteriorly resulting in a ciliochoroidal detachment following trauma and inflammation. 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. There is a potential space in between the sclera and the choroid called suprachoroidal space that is liable to distended by fluid. 2.1 mm. The inner surface of para plicata is divided into 70 to 80 ridges or plications. Most of the bulk of the ciliary muscle is located in the anterior two-third of the ciliary body. The vitreous base extends from retinal periphery up to 2 mm. The exit of the vortex veins near the equator. It comprises of ciliary stroma. they extend towards the lens. at the posterior pole it is 0. The non-pigment epithelium secretes aqueous while the pigment epithelium continues as retinal pigment epithelium. They do not have any stroma or muscles. The middle part is formed by the radial fibres. While the innermost are the circular fibres. It is continuous with ora serrata anteriorly with loose attachment to the 1. The nerve supply of the ciliary body is mostly by parasympathetic nerve. Each of them is 0. Pars plana. The sclera.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.5 mm. The pars plana is posterior 6 to 7 mm. blood vessels and nerves. however. Its attachment to most of the sclera is loose. round the disc at posteriorly.

The functions of the choroids is to supply blood to the outer retina. The choriocapillaries are denser and relatively larger under the macula. In high magnification. it has five layers. Bruch’s membrane. Vascular layer 3. The blood supply to the eye ball is from the ophthalmic artery which gives two independent system of blood supply. the two . there are no valves in choroidal veins7. The Bruchi’s membrane is an acellular thin structure that develops from neuro ectoderm as well as uvea. The choroid does not have any motor function. (i) The layer of large vessel (ii) The layer of small vessels (iii) The choriocapillaries. The choriocapillaries end at ora and rest of the vessels continue in ciliary body. melanocytes and fibrocytes like iris and ciliary body. The vascular layer forms the main bulk of the choroid. The choroid is said to act like a lymph node following inflammation. It is not influenced by parasympathetic nerve.DISORDERS OF THE UVEA IN CHILDREN 231 Histologically the choroid is divided into: 1. vascularity is maximum in the choroid and least in the iris. The main function of the choriocapillaries is to supply nutrients to the outer part of the retina. The retinal system and the ciliary system. There is no muscle in the choroids. 3. It is thickest around the optic disc. It consists of three layers of blood vessels i. The size of the blood vessels diminish in size from outer surface to the inner surface. Sympathetic nerve supply regulates choroidal circulation. they supply a globular area rather a sector. and is attached to the inner surface of the sclera. The endothelium is fenestrated in contrast to iris vessels which are non-fenestrated.e. Each vortex system unites to form an ampula that drains obliquely through a scleral canal. The outer layer is mostly venous in nature. The lower vortex veins are formed by union of choroidal vessels 3 mm behind the equator. Supera choroidal lamina 2. The veins unite to form vortex veins which are four in numbers. The suprachoroidal lamina contains melanocytes. Suprachoroidal lamina. The choriocapillaries are the largest capillaries in the body. They lie in between the layers of choroidal vessels and Bruch’s membrane. 1. The choriocapillaries do not anastamose freely. Other functions are to keep the interior of the globe dark. fibrocytes and thin fibres. 2. one in each quadrant. Bruchi’s membrane. The Bruchi’s membrane forms the blood retinal barrier and acts a filter for metabolic exchange between choriocapillaries and pigment epithelium of the retina8. The spaces in between the vessel is occupied by strands of stroma. Choriocapillaries. This layer is attached to the choroid on its outer surface. All the vortex veins drain into ophthalmic veins. Blood supply of uvea4. They are channels lined by endothelium in which the choroidal arterioles end. 7 Uvea is a vascular structure. to regulate the temperature of the eye and enhance the outflow of aqueous through uveoscleral channel.

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. they anastomose with the tiny long posterior ciliary arteries to form the major arterial circle. They are more in number on the lateral side to give more blood to the macula. 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. 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. On reaching the choroid these vessels branch profusely.232 PEDIATRIC OPHTHALMOLOGY generally do not anastomose. These vessels travel forward to reach the posterior part of the globe. Venous drainage of uvea is mostly by vortex veins. The two branches give off multiple smaller twigs. At this level. There are two type of vessels in the choroid. A poorly developed ciliary system. 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. They divide into two branches on entering the ciliary body. The former supplies the inner layer of the retina while the latter supplies the outer layer of the retina. The choroid drains via vortex veins exclusively. 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. 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. The ciliary circulation consists of—a posterior ciliary system consisting of long and short posterior ciliary arteries and complex of anterior ciliary arteries. 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. 11 The uvea as a whole has bipartite development. The iris and the ciliary body are mostly ectodermal in origin except the stroma and the blood vessels. Development of the uvea9. There are no formed lymphatics in the uvea. the dilator pupillae and melanocytes. The long posterior ciliary arteries are two in number. which travel forward encircling the optic nerve all around. uvea and extra ocular muscles. the sphincter. 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 venous drainage is more developed than the posterior venous drainage. The ectodermal structure in the iris are the two layers of epithelium. The iris and ciliary body have two systems of venous drainage-1. They arise from the opthalmic artery in the orbit. either by a common trunk or two separate trunks. 10 to 12 recurrent branches go backward to supply the anterior choroid. . The choroid is dominantly mesodermal except the Bruch’s membrane which is partly mesodermal and partly ectodermal. A well developed vortex system and 2. 10.

The choroid develops from mesodermal mass at about 6 mm. Involvement of the iris in coloboma varies from a small notch at the pupillary border to extensive . A network of capillaries that surround the optic cup develop into choroid. The mesodermal structures in iris and ciliary body are—the iris stroma. The mesoderm that lies in front of the developing iris epithelium forms the stroma of the iris. can be complete or incomplete. The closure is completed by 18 mm. 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. The embryonic fissure starts closing at 10-11 mm. The Bruch’s membrane is secreted by neural epithelial layer. ciliary body and choroids in various combination.e. Close proximity of iris and ciliary body and choroids to retina may involve them as well.e. well before the development of the iris. Closure of the embryonic fissure (choroidal fissure) and atrophy of tunica vasculosa lentis. A coloboma is said to be complete when all the layers of iris. 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. Congenital anomalies of the uvea Congenital anomalies may involve all the three parts of uvea or individually iris.DISORDERS OF THE UVEA IN CHILDREN 233 The epithelium of the ciliary body and the ciliary processes are also ectodermal in origin. The edges of the optic cup grow in front of the lens as a double row of epithelium behind the mesoderm. A coloboma is called typical when it develops at the site of the closure of foetal fissure i. Coloboma of the iris Coloboma of the iris may be typical or atypical. they develops from the non-pigmented iris epithelium surrounding the pupil. inferior nasal part of the uvea and atypical when coloboma is located in place away from foetal fissure. colobomas are generally hereditary. By third month large and medium choroidal vessels develop. stage at the middle of the fissure and spreads in both directions. The growth of the iris is influenced by two factors. the anterior end fuses later than the posterior end. The iris and the ciliary body develop from the anterior lip of the optic cup. blood vessels and ciliary muscles. The two layers of the iris epithelium become pigmented in ciliary body. The dilator muscles develop from the non pigmented layer near the root. Frequently the congenital anomalies of uvea are due to faulty closure of the foetal fissure resulting into colomboma of the uvea of various types. The coloboma may be unilateral or bilateral when present in both the eyes they are generally symmetrical. 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. involve both sexes equally. The vortex veins also develop at the same time. i. Non closure of the foetal fissure from end to end leads to formation of colobomatous cystic eye ball. ciliary body or choroids are involved and incomplete where instead of full thickness involvement only partial thickness is underdeveloped. 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.

is called clinical anisocoria. The pupil is inverted pear shaped or key hole like. The eye may be of normal size but most of the times it is smaller than normal. The two pupil generally do not have symmetrical displacement. Congenital microcoria or miotic pupil is due to faulty development of sphincter pupillae. Aniridia13. In about 50 percent of cases. Vision varies from almost normal to severe loss depending upon the degree of involvement of intraocular structures. No specific treatment is required for coloboma of iris. The cornea shows peripheral fine pannus. 14 Tough the term aniridia should mean total absence of iris. The macula is hypo plastic under development of macula is the cause of poor vision and nystagmus. The defect is generally circular. The zonules are visible all round and the ciliary process are also seen. in distant gaze. the lens generally subluxates away from the decentred pupil. presence of error of refraction should be managed as and when present to prevent amblyopia. The typical coloboma is situated at inferionasal part of the iris.Normal pupil is central. The lower end of the lens is visible with its zonules. These pupil have their own sphincter and dilator muscles. There may be localized peripheral lenticular opacity in the lens behind the coloboma. however. a bluish streak is seen through the conjunctiva. These strands are generally detected on 360 degrees gonioscopy. If the coloboma extends into the ciliary body. It is generally bilateral. . Even in most advance cases. Anomalies of position. Anisocoria When the size of two pupil are different the condition called anisocoria. Sporadic. Psudo polycoria denotes full thickness defects in the substance of iris. bridging the gap. there are always some strands of iris present at the roots. There are two types of inheritance. It extends from pupillary margin up to the root of the iris. 13A. circular with slight nasal shift. may extend in the ciliary body. 1. The two pupils are never of the same size. size and shape of pupil . A pupil is said to be microcoria if its diameter is less than 2 mm. sensory nystagmus. These pupil does not react to light or accommodation independent of usual pupil.234 PEDIATRIC OPHTHALMOLOGY involvement of the uvea from pupillary border to the optic nerve. The corectopia is generally associated with ectopic lens. Besides poor development of the iris the eye has other signs as well. they obstruct the trabecular mesh work in later life. A similar hole near ciliary body is called iridodiastasis12. Position of both the pupil is identical in two eyes. A change of more than 2 mm. looking like additional pupil. If the pupil is shifted from its normal position it is called ectopia pupil or corectopia . The pupil is generally slit like in presence of light but becomes almost circular in dim light. A coloboma is called bridge coloboma when a strand of iris tissue spans over a coloboma. Dyscoria is a rare condition where there is congenital abnormality in shape of pupil other than the coloboma. the lens shows various degrees of opacities on slit lamp examination. in clinical practice it is not so.13 The condition is present at birth and is bilateral. If the ciliary body is involved there may be absence of zonules at the site adjacent to the coloboma. the pupil is almost as large as the cornea. Such pupil do not have sphincter or dilator muscles or pigments on its border. Dominant 2. The eye have variable degrees of errors of refraction. this tissue is mesodermal remnant of pupillary membrane.

The child may require low vision aid and trained as visually handicapped. The shreds move freely with the movement of the pupil without restricting it. The condition does not hamper vision or cause any complication hence does not require any treatment. All sporadic cases of aniridia should undergo abdominal examination and to examination by ultrasonography on first presentation and repeated yearly for next few years. for the first six month of foetal life and then disappears. Persistent pupillary membrane14. However a typical coloboma may be seen at other parts. The strands vary in number and length. There may be other causes of glaucoma than simple obstruction of trabecular mesh work.13 Half of the sporadic cases may develop Wilm’s tumor. they are very rare in old age. It does not require any treatment. Occasionally it can be seen while doing goneoscopy or ultrasonography. They represent anterior part of tunica vasculosa lentis that supplies nutrition to the lens in foetal life. They normally distort pupillary shape but may be mistaken as posterior synechea which are at pupillary margin. 16 It is an associate anomaly that looks like anomaly of the iris. Sometimes it gets attached to the anterior lens capsules where a small opacity may be present. Coloboma of the ciliary body is most commonly seen in the microphthalmic eyes. Coloboma of ciliary body The isolated congenital coloboma of the ciliary body alone is infrequent. poor vision should be corrected as far as possible and glaucoma controlled as much as possible.DISORDERS OF THE UVEA IN CHILDREN 235 Secondary glaucoma develops in the second and third decade of life. most of which disappears by first year. The strands may float freely in the aqueous with one end attachment to the collarette. It persists for few years and then gets absorbed. It may span the pupil and get attached to the opposite collarette or may cross the pupil in segments. Colobomata are visualised on indirect opthalmoscopy with scleral indentation. It is generally associated with coloboma of the iris. Commonest congenital anomaly of the ciliary body is typical coloboma due to non-fusion of the foetal fissure in six O’ clock position. Development of pigment is a post natal feature of the shreds. Presence of Wilm’s tumor and aniridia is known as Miller’s syndrome. 15. surgery fails to reach the desired goals unless prophylactic goniotomy is done before onset of glaucoma. It may get attached to the posterior surface of the cornea with a faint opacity. Failure of complete disappearance results in shred of mesodermal tissue and obliterated blood vessels that are attached to the collarette. . may be associated with the coloboma of choroids. The glaucoma is difficult to manage and the condition does not respond to medical treatment. Persistent pupillary membrane are more common than coloboma of the uvea. Persistent pupillary membrane may be unilateral or bilateral and equal in genders. most probably due to obstruction of trabeculum by iris tags. Persistent pupillary membrane is not a true congenital anomaly of the iris.15 Management is difficult. It has been reported to be present in 96% of new born. They may be gross enough to be seen by oblique illumination without magnification or may be fine enough to be seen by slit lamp. It may be associated with subluxation of the lens due to non-development of zonules at the site of the ciliary coloboma.

The edges are pigmented. sometimes these may be irregular. Generally a choroidal coloboma has a parabolic appearance with its broader end towards the ciliary body and rounded head towards the optic nerve. 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. Oculo cutaneous and 2. 1. The sclera may be ectatic. Ocular. The coloboma when present are bilateral in two third of the cases.236 Coloboma of choroids PEDIATRIC OPHTHALMOLOGY Coloboma of choroids can be typical when situated at the site of foetal fissure or atypical when at other than foetal fissure. The periphery of large coloboma is seen by indirect opthalmoscope that may show up scleral ectasia. it may be very small or larger than the optic nerve. The other group known as tyrosinase positive. have some feature in milder degree of oculcutaneous albinism. It is generally bilateral. The scleral bed is generally depressed. the edges are clear cut. The colour of the coloboma is white due to exposed sclera underneath. tyrosinase positive and tyrosinase negative. Outer margin of the small coloboma is visible with direct opthalmoscopy. There is corresponding negative scotoma. Clinically albinism has been broadly divided into two types. There may be more than one such patch in the line of closure of the fissure. Ultrasonography may also show choroidal defect and scleral ectasia. Oculo cutaneous albinism have extensive systemic as well as ocular involvement. Diagnosis is confirmed by direct and indirect opthomoscopy. The retinal vessels are seen to traverse over this coloboma. in extreme cases it may be ectatic.e. Vision is greatly reduced leading to nystagmus. 3. A large coloboma may give rise to grey reflex on retinoscope. The former can be divided in biochemically into two sub groups i. it is usually autosomal recessive inheritance. The colobomas are punched out horizontally oval areas with clumps of pigments on the periphery and a white floor representing sclera. The diagnosis is straight forward. 1. 2. The area is devoid of choroidal and retinal tissues. 4. A macular coloboma may be mistaken as part of congenital toxoplasmosis. a patch of retinal tissue may cross the coloboma from side to side. In rare instances of bridge coloboma. Sometimes the edges of the coloboma may reach very near the macula but not involve it. rarely abnormal retinal vessel may traverse the gap. It has also been thought to be dysplasia of macula. The former is more common. The retina is absent over the coloboma so it is better to call it a retino choroidal coloboma. when holes may develop at the edge of the coloboma. An X-ray study is done to exclude intera cranial calcification which is seen in congenital toxoplamosis. One of the complications besides sub normal vision is development of rhegmatogenous retinal detachment. . squint and amblyopia. Vision is generally poor. Albinism18 Albinism is hereditary disorder due to abnormal metabolism tyrosine hydroxylase resulting in complete or partial absence of pigment in the body. Coloboma of macula Coloboma of the macula may be considered as modified form of atypical choroidal coloboma. On retinoscopy a white reflex in the lower part amidst a pink glow is seen.

Choroideremia 3. The iris may transilluminate at places but there are no holes in the iris. Choroidal sclerosis. In ocular albinism the lids. There is nystagmus and squint. Management of albinism is difficult. Complicated heterochromia is seen in Fuch’s heterochromic cyclitis. Some of the systemic anomalies associated with heterochromia of iris are : Waardenburg syndrome.e. Hair all over the body are light coloured including eye brows and eye lashes.DISORDERS OF THE UVEA IN CHILDREN 237 The ocular albinism is generally X-linked and autosomal recessive in inheritance. There are many types of heterochromia. Other types may require ocular treatment i. Sympathetic heterochromia is seen commonly with Horner’s syndrome. It can be unilateral. In these cases decussation of optic fibers at optic chiasma has also been found to be defective. Fortunately they are rare. This involvement is limited to the globe only. Contact lenses are not suitable due to associated nystagmus. 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. Unilateral heterochromia of iris is called heterochromia iridum. Gyrate atrophy of choroid 2. The oculocutaneous albinism produces fair coloured skin that is sensitive to light. hypermetropia is more common than other types of refractive error. use of tinted glasses reduce glare. The eyes with undeveloped macula have congenital colour blindness. Systemic conditions require multi system work up and management. Simple heterochromia is symptomless and does not require any treatment. inflammation and new growth. full correction should be given to salvage as much of vision as possible. 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. that is one eye has lighter iris than the other. The exact cause of congenital heterochromia is not known. The vision is generally greatly reduced. Heterochromia of the uvea Heterochromia of the uvea is confined to the iris.e. but neither preventable nor treatable. Romber’s syndrome and various types of status dysraphicus. . Simple heterochromia is common without any other ocular or systemic involvement. however.19 The eyes generally have various types of errors of refraction. lashes and eye brows are normal. Fuch’s heterochromia cyclitis. Vision can rarely be improved. Albino children may require low vision aids to pursue studies. The iris with lighter shade is abnormal. They are 1. Acquired difference is generally due to trauma. with developed macula and without properly differentiated fovea. 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. Asymptomatic female carrier of ocular albinism may also have iris trans illumination. The eyes with different colours are called heterochromia iridus.

No age sex or ethnic group is spared. There is no specific treatment. Ultimately the patient has to be rehabilitated as visually challenged. Choroideremia This is a bilateral progressive disease. Choroidal sclerosis Choroidal sclerosis has two forms: 1. Constant supplementation of diet with pyridoxine (B6) is supposed to delay the progress. The disease progresses to legal blindness in ten to twenty years. The juvenile form is most probably congenital in nature and manifest round about fifteen years of age. Commonest age group inflicted by uveitis is between second to sixth decade after which incidence of uveitis diminishes rapidly. Secondary cataract is common EOG and ERG are sub normal. It is the commonest disorder of the uvea. Low vision aid and rehabilitation are the only paliatives available. Macular involvement is late either as macular edema or a patch may cover the macula. retina. Uveitis in children may range between mild self limiting anterior uveitis to sever . Low protein diet is also recommended. However. seen only in males. 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. it can be seen at any age in both the sexes.238 PEDIATRIC OPHTHALMOLOGY One of the suspected causes of Fuch’s heterochromic cyclitis is congenital in origin. The central vision is poor. There is no known treatment. The two features common among the two conditions are night blindness and ring scotoma in the peripheral field. There is no specific treatment. The disease is caused due to atrophy of choroid and retinal pigment epithelial. It is associated with raised level of ornithine in plasma. The adult form is probably is degenerative process. About five percentage of all uveitis is seen in pediatric age group. Low vision aids and mobility support may help. The condition is heriditory bilateral presenting in the first decade as progressive night blindness. The lesion begins on the macula that looks similar to patch of choroiditis. some consider it be inflammatory or degenerative and do not put in category of congenital anomalies. CSF and urine. Central vision is retained till late. they merge with each other to form a larger patch and girdle the mid periphery. aqueous. The fundus has no resemblance to retinitis pigmentosa. Due to proximity of lens. Uveitis in children General consideration The term uveitis means inflammation of any part of the uvea. these parts are invariably drawn into the clinical manifestation of uveitis as well as complication. The lesions start in mid periphery as oval patches with scalooped border. Adult and 2. women are carriers who show mild form of the disease. vitreous and optic nerve to the uvea. There is progressive atrophy of choroid as well as retina. Choroid and retina are absent over the patch but the retinal blood vessels remain normal for long time and then attenuate. It can be unilateral or bilateral. 10% cases are bilateral. Juvenile. Gyrate atrophy of choroid This is an inborn error of aminoacid metabolism. The size and shape of the lesion vary.

In case of interstitial keratitis the inflammation starts in the uvea and spreads to the cornea. that may involve the whole of choroid. Various classifications have been put forward depending upon 1. 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. (iii) Posterior uveitis. This rises to fifty percent in uveitis in children.e.e. syphilis. 3. Chronic III. 1. Parts of the uvea involved. not extending beyond sclera. This is inflammation localized to iris and or ciliary body. Pathology. Some of the uveitis in children are due to systemic infection and are bilateral i. Recurrent IV. may be near the disc or involve the periphery. (xi) Sclero uveitis is involvement of uvea along with sclera. Masquerade syndromes. uveitis can be I. 6. (vii) Keratouveitis. toxoplasmosis. Associated with various systemic manifestation. Panophthalmitis is the severest form of uveitis involving all coats of eye and periocular tissue. In this sight threatening condition there is pan uveitis with involvement of vitreous. Onset (Chronological) On the basis of mode of onset. . Small areas of inflammation are scattered all over the fundus behind the equator. (viii) Juxta papillary22. Undetected uveitis in children may result in permanent sub normal vision leading to strabismus and amblyopia. 5. Acute II. All the parts of uvea are involved as in sympathetic ophthalmia. tuberculosis. Etiology. 2. There is no satisfactory way to classify uveitis. The lesion is a choroidal inflammatory patch near the disc producing sector shaped lesion and corresponding field defect. (ix) Disseminated choroiditis. Acute exacerbation on chronic. 2. (vi) Panophthalmitis. Due to lack of anatomical barrier between iris and ciliary body the inflammation may involve both resulting in to iridocyclitis. (x) Central choroiditis. It is located in the posterior pole involving the macula. (ii) Intermediate uveitis.DISORDERS OF THE UVEA IN CHILDREN 239 vision threatening endophthalmitis involving all parts of uvea as well vitreous. Anatomical classification (i) Anterior uveitis. Inflammation limited to iris is called iritis while that involving ciliary body (pars plicata) is known as cyclitis. (v) Endophthalmitis. The part involved is mostly choroid. In one third of all uveitis cases no definite cause can be pinpointed as causative factor. Here the process involves posterior part of the ciliary body (parsplana) peripheral choroid and retina and called as parsplanitis. It is a blinding condition. may be localized to macula. 4. Mode of onset and duration. toxoplasmosis. (iv) Pan uveitis. 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.

malignant tumor of eye. . tuberculosis. HLA typing is now considered an important diagnostic test in uveitis. but not fatal and must be differentiated for more serious retinoblastoma which is both sight threatening as well as life threatening. retained intra ocular foreign bodies specially pure copper.25 It generally produces bilateral congenital or acquired posterior uveitis. (b) Exogenous microbial uveitis is due to direct entry of pathogenic organism. Both present with white reflex in pupillary area. gonococcus and pneumococcus. measles and mumps. Etiological PEDIATRIC OPHTHALMOLOGY 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. anterior segment ischaemia following squint surgery. accidental and surgical or spread from outer coat of the eye mostly as a result perforating corneal ulcer. (ii) Auto immune disease. following perforation of globe. Lens protein sensatization cause phacogenic uveitis. sarcoidosis. (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. iritis glaucomatosa. syphilis.e. Vogt Koyanagi Harada disease. rheumatoid arthritis. Out of many protozoan infection that produces uveitis is Toxoplasma gondi. fungi. herpes zostor Epistein Barr virus. Reiter’s syndrome.240 3. They can produce acute or chronic lesions.24 Fungi that produce uveitis are candida. rubella. pulse less disease also produces uveitis but all are not seen in children.e. These are juvenile rheumatoid arthritis. Eiologically uveitis can be divided into : (i) Microbial (a) Endogenous. The bacilli generally produce chronic uveitis i. Viruses that cause uveitis are herpes simplex. (b) Exogenous. Acute lesions are generally produced by cocci i. All bacteria are capable of producing some type of uveitis or other if they get access to the uvea. protozoa and nematodes. leprosy23. ankylosing spondylitis. long standing retinal detachment. Auto immune disorders that generally have systemic manifestation form the largest group of diseases that produce both acute and chronic uveitis. Fuch’s heterochromatic iritis.24 Sympathetic ophthalmia is presumed to be due to hypersensitivity to uveal pigment. fusarium. Behcet disease. (iii) Other non specific uveitis are—Pars planitis. influenza. Toxocara canis and catis are two round worms of dogs and cats that produce unilateral posterior uveitis in children that is always vision threatening. They are generally associated with certain specific HLA type. Other nematodes that cause uveitis are cysticerosis and onchocercisasis. viruses. The organism can be bacteria.

Pathological classification The pathological classification most widely used was suggested by Woods in 194727. Vitreous does not show cells. Complications and sequaelae may change this clinical picture entirely. . The onset is generally insidious without much pain. the keratic precipitates are large mutton fat in nature. looks like granulomatous. The condition has long slow course. it present as granulomatous uvietis with nodule formation. granulomatous and non-granulomatous. Division of uveitis into two classes of granulomatous and non granulomatous is not always clinically possible. In late cases peripheral anterior synechia are seen. KPs are generally small and numerous. It is more commonly a bilateral involvement. In late stages there may be peripheral anterior synchia. that divides uveitis into two groups i.28. The non-granulomatous uveitis has an acute painful onset. 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. There is a proliferation of tissue. Posterior synechia are small and narrow. broad and difficult to break. The iris develops nodules. generally uniocular to begin-with. Symptoms of posterior uveitis may differ from those of anterior uveitis in such an extent that they may look like two separate entities. on the basis of histopathology presentation without sharp line of demarcation in clinical presentation. Some of the examples are—uveitis due to leptospirosis should cause a granulomatous uveitis but in fact it presents as a non granulomatous uveitis. There is general actual invasion of the uvea by organism. Similarly sympathetic uveitis also presents as granulomatous lesion with epithelial cells and giant cells when it is supposed to be an allergic reaction.e. There may just be blurring of vision without pain or redness as seen in pars planitis. Logically it should produce non granulomatous uveitis but in most of the eye. Clinical features of uveitis in general Symptoms and signs in uveitis greatly differ in different types. Hypopyon is unusual. The posterior synechia are thick. aqueous flare is relatively low with scanty cells. Similarly lens induced uveitis is expected to be an allergic reaction to lens protein and should behave like non granulomatous which on slit lamp. mostly self limiting. There may be fibrinous exudation and formation of hypopyon. There is marked circumciliary congestion. The proliferation of tissue due to microbial invasion depends upon the virulence of the organism and the state of immunity of the tissue. An episode lasts for four to six weeks without treatment. There may be ciliary tenderness. This may involve all parts but anterior uveitis is more common. white iritis in girls and Fuch’s heterochromic iritis to severe loss of vision with pain as in endophthalmitis. pupil is miotic. Anterior chamber reaction is marked with flare and cells. The granulomatous uveitis generally involves all the part of uvea with predilection for choroids. 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. other eye gets involved later. break with ease. the vitreous shows marked flare and cells. Sarcoidosis is a non infective multi systemic disorder. Iris module are generally not formed.DISORDERS OF THE UVEA IN CHILDREN 241 4.

few KPs may be deposited in an irregular fashion. Signs of anterior uveitis 1. 3. The number. Moreover the signs and symptoms differ greatly between acute and chronic uveitis. The congestion fades towards periphery. exudates in the papillary area. i. 2. sometime there may be papillitis result in diminished vision. develops after three four days. turbid aqueous. Generally sudden and unilateral but may occasionally be bilateral. 3. This pain is worst at night. Circum corneal congestion. to visualise them in details they should be examined with good illumination of slit lamp. Simultaneous onset is extremely rare. Redness. size and distribution depend up on severity and duration of inflammation. 5. macrophases. Fine KPs are generally dusted all over the endothelium. small constricted pupil.242 PEDIATRIC OPHTHALMOLOGY It is better to divide clinical features related to following types of uveitis. Symptoms of acute anterior uveitis 1. Keratic precipitates: Keratic precipitates are deposition of cells. The circum ciliary congestion does not blanch with weak solution of vasoconstrictor. 4. The eye may present as red eye due to circum ciliary congestion caused by dilated anterior ciliary vessels. However. 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. 2. uveal pigments or RBC on the endothelium of cornea.e. 2. posterior uveitis. Redness around the cornea starts as a pink hue that becomes gradually darker red. . Mild edema of the lids : Exact cause of such edema is not well understood. The pain ranges from dull ache to throbbing pain. secondary glaucoma.27 The large keratic precipitates may be visible on bright oblique illumination with corneal loupe. deposition of precipitates on the posterior surface of cornea. 3. anterior uveitis. radiating along the distribution of ophthalmic branch of fifth nerve on the same side. Onset. shape. pan uveitis in separate groups. Pain. 4. Tenderness of the globe is indicative of ciliary involvement. or from the beginning in a case of recurrent attack. Large to medium sized keratic precipitates are generally deposited in a triangular fashion with apex up towards the pupil. Diminished vision. intermediate uveitis. spasm of ciliary body resulting in the myopia. those belonging to 1. Complications like glaucoma or cataract may overshadow the primary features of uveitis. anterior vitreous cells and flares. Diminished vision if at all present. Lacrimation and photophobia are common symptoms due to irritation of sensory supply to anterior uvea. Pain may be the first symptoms of acute anterior uveitis. Causes of diminished vision are—Edema of corneal epithelium. They are more marked in children. Recurrent anterior uveitis is commonly associated with macular edema. Initially patient may not have diminished vision.

Aqueous flare. The apex of the triangle is upwards. As inflammation subsidies they become hylanised and fade in colour. these borders become crenated. Old KPs. Mutton fat KP. they may at times be pigmented. Red KPs are seen in uveitis that cause hyphaema i. Increased turbidity of aqueous is due to suspended particles : cells fibrin. 4. slit. There may be as many as forty to fifty in one eye. They may be seen on the iris. larger KPs occupy lower position.e. endothelial dusting. 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. They are scattered all over with predilection for lower part. ciliary body. Pigmented KPs. they are replaced by lymphocytes. In inflammation there is outpouring of protein from the anterior uvea resulting into plasmoid aqueous giving it a turbid appearance. sarcoidosis. Flare in not always a sign of active uveitis. pigmented KP and red KP. Larger KPs may reach upto 1 mm.DISORDERS OF THE UVEA IN CHILDREN 243 Various types of keratic precipitates according to their size. they have a sandy white colour. colour and characteristic are: large mutton fat KP. They are generally seen in so called granulomatous uveitis but may be seen in recurrent non granulomatous uveitis also. trabeculum lens. medium KP. plasma cells and macrophages. suspensory ligament and anterior vitreous face. These are seen in chronic inflammation of long duration. protein. as days pass. More are the cells. KPs shrink in size. Normal aqueous is colourless. They are due to entanglement of iris pigment in white KPs or the iris pigment has been engulfed by the cells. Small and medium sized KPs are due to lymphocytes and plasma cells. herpes simplex and herpes zoster. They are seen in nongranulomatous anterior uveitis both acute and chronic. They are called mutton fat KPs due to their greasy lardaceous appearance. sympathetic ophthalmia. The cells may circulate in the aqueous by convection current or settle on the endothelium. in size. 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. Endothelial dusting is caused by very small KPs that may run into hundreds. endophthalmitis anaphylectica. toxoplasmosis. 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. Anterior chamber reaction. their number varies between ten to twenty. RBC etc. Flare is again graded between 0 to 4+.27 Initially the cells are poly-morphonuclear. fade in colour. In aqueous cells originate in the iris and ciliary body due to active migration from uvea to aqueous humor. clear fluid. The cells are examined by the slit lamp with maximum illumination and magnification by a 3 mm. The cells. As time passes. commonly seen in acute nongranulomatous anterior uveitis and recurrence of such conditions.. These are largest KPs arranged in a broad based triangular distribution. more is the involvement of ciliary body. their borders are clear cut. they have ground glass appearance and may be pigmented. Clearer details denote less flare and total obscuration is maximum flare. this phenomena is called aqueous flare. it can be seen due to . Mutton fat KPs primarily consists of macrophages and clusters of epithelial cells. Common causes being—Tuberculosis. × 1 mm.

Pupil. The iris looses its roughness. Presence of fibrinous aqueous is called plastic iritis which is a form of severe iritis. In fibrinous iridocyclitis a membrane similar to occlusio pupil develops behind the posterior capsule and is called posterior cyclitic membrane. varying in number and size. later the iris gets adhered to it which on dilatation leave fragments of iris pigment on the lens. Fibrenous exudates in the AC. 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. In early stages that lens is clear. Accumulation of pus in AC is called hypopyon that is sterile. Long standing posterior synechia are the site where future anterior capsular opacities may develop. 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. In severe cases of uveitis there is down powering of WBC. This gives the pupil a scalooped or festooned appearance. In case of recurrence complicated cataract develops starting from posterior capsule which on slit lamp give a polychromatic lusture.244 PEDIATRIC OPHTHALMOLOGY leak of damaged vessels without iridocyclitis. With repeated use of mydriatic. A swollen peripheral iris may come in contact with posterior surfaces of cornea resulting in peripheral anterior synechia. the contours of the pits and collarettes are lost. but dilates inbetween the synechia. Changes in vitreous are common specially when ciliary body in inflamed. Initially the pupil does not dilate at the site of synechia. In severe anterior uveitis the cells may get enmeshed in fibrinous aqueous and form a sheet over the iris surface and lens capsule. Nodules on iris are late phenomenon mostly seen in recurrent and chronic uveitis. they require stronger mydriatic cum cycloplegia. 7. relatively stronger sphincter muscles and irritative action of toxins on the nerve ending. The anterior vitreous can be seen by a bright beam of slit lamp. The iris gets fluid laden due to dilation of radial vessel and cellular infiltration of the stroma. but do not break. infiltration of stroma by cells. Causes of miosis are engorged radial vessels. Pus in AC. 6. 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. Vitreous involvement is directly proportionate to severity of inflammation. 8. It is miotic which on careful examination looks irregular that reacts poorly to light. As the iris gets fluid filled and increases in thickness it spreads towards the centre causing shrinkage in pupillary diameter. In long standing and recurrent cases anterior capsular cataract develops at the site of posterior synechia. If the synechia persists for few weeks. Vitreous. Iris. To diagnose uveitis a combination of cells and flare must be present. the changes are in content and structure. coagulated exudates and fibrin. Deposition of exudates that obscures pupillary area is called occlusio pupil with loss of third Purkinje image. The changes can be in the form of opacities that are due to cells. 5. In untreated cases posterior synechia may spread all along the circumference and bind down the pupillary margin all around. Presence of cells in the anterior vitreous is seen in anterior uveites while that in the posterior . that due to their weight gravitate at the bottom of AC. Lens.

There are very few cells in anterior chamber. C. There may be fine KPs in the lower part. posterior capsular opacification and vitreous debris. Shrinkage of perivascular membrane may lead to traction detachment. The macular edema is a self limiting feature but may leave permanent pigmentation. Lowering of intraocular pressure is more pronounced in cyclitis due to lowered secretion in ciliary process. Signs of intermediate uveitis There is hardly any sign visible on oblique illumination the eye is white in most of the cases. Children complaining of floaters in eye should not be dismissed as simple floaters unless proved other wise. It may be localized either in posterior pole or periphery. perivasculitis and vitritis. In 70% cases it is bilateral. this is more common in cyclitis. scattered all over the choroids. The retinal changes can be diffuse. Diffuse retinal edema may be present in anterior uveitis. the posterior vitreous is clear. 31 Intermediate uveitis is also known as pars planitis and chronic cyclitis. In case of a small rigid pupil fundus may not be visible. 9. Fundus changes A. The real diagnosis lies in the peripheral fundus examined by indirect opthalmoscope and scleral dentation that shows peripheral retinitis. Intraocular pressure. The symptoms and signs depend upon the above factors. . In case of cyclitis cell density in anterior vitreous is more than in anterior chamber. The media may look hazy due to presence of KPs. Macular changes are seen mostly as cystoid macular edema (CME). peripheral anterior synechia. Symptoms of Intermediate Uveities24. 10. A comparison between the cell count in anterior chamber and posterior chamber gives a rough idea differentiating involvement of iris and ciliary body. Intraocular pressure changes are variable. best seen by Goldman three mirror lens or Volks + 68 to + 90D and fluorescein angiography. in case of iritis the finding is reversed. diminished or foggy vision that may worsen over years without any attributable cause. The macula gets involved in late stages in the cystoid macular edema. the iris is normal. Posterior sub capsular cataract is a common late feature. 27. while in most of the eyes it remains normal. Peripheral changes are seen in pars planitis. 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. Pars planitis is a disease of children and young adults. in some cases it may become abnormal in initial few days. Retina and optic nerve are frequently involved. fibrin in pupillary area. Intraocular pressure rise in acute anterior uveitis is due to plasmoid aqueous and cells obstructing the trabecular meshwork. especially in children.DISORDERS OF THE UVEA IN CHILDREN 245 vitreous is due to inflammation of choroid. B. Symptoms of posterior uveitis Posterior uveitis can be acute or chronic. trabeculitis. The anterior vitreous shows more cells than in aqueous. may be diffuse. so is the pupil. peripheral or macular. 30. The disc may be blurred due to optic neuritis. Rarely there may be papillitis. The symptoms are floaters.

Vitreous changes are very prominent in acute lesions of the choroids. photophobia or lacrimation. medium field. border of the lesion is well defined. An unilateral lesion of central choroiditis may present as strabismus and amblyopia in a child. redness and ciliart tenderness are also absent. Acute posterior uveitis with muscular involvement have deterioration of vision that may be preceded by metamorphopsia. In a case of acute choroiditis. redness. optic nerve head. In case of endophthalmitis there may be severe vitreitis with accumulation of exudates. Zero being clear is view of nerve fibre striation and four plus stands for obscuration of optic nerve head. Lacrimation. They may be fine coarse.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. seeing of floaters. Signs of posterior uveitis are better divided into 1. sudden loss of red reflex denotes onset of endophthalmitis. Symptoms of chronic posterior uveitis The symptoms are same as in acute posterior uveitis i. Peripheral lesions do not produce much of visual symptoms.e. vitreous flare and posterior vitreous detachment. A large area in the posterior pole may give grayish reflex and retinoscopy that disappears from retinoscopy field on movement of the eyeball. scotomas and diminished vision persists. candidiasis and sarcoidosis32. papillitis. examined by + 20 D lens. Acute lesion of choroids is a patch of choroiditis seen as an area over which retina is invariably involved. In case of congenital toxoplasmosis loss of vision may be detected only when the child is brought as a case of squint. Acute and 2. Diminished vision : Diminished vision is a prominent feature of posterior uveitis specially if the lesion involves the macula. vasculitis.33 Visibility of three fundus landmarks i. retinal blood vessels and retinal nerve layer are noted and graded between 0 to 4+. vitreous haze.e. scar or the macula. Signs of posterior uveitis are mostly localized in the posterior pole. however. . photophobia. Colour of the area involved is yellowish or grayish. The vitreous opacities are graded by number of cells counted in a field by direct opthalmoscope and graded between 0 to 4 +.32 Other changes noted on fundus examination are retinitis. with diminished illumination. The vitreous opacities may vary in shape and size. the edema may not obscure the retinal vessel. They are in the form of vitreous floaters. Fine opacities are due to inflammatory cells. Causes of diminished vision in posterior uveitis consists of simple macular edema. However. pigmentation of the macula. Chronic. lenticular opacity and secondary glaucoma. maculopapillar bundle and optic nerve. cystoid macular edema. followed by indirect opthalmoscopy with scleral indentation. Large opacities on the periphery and snow ball opacities are seen commonly in pars planitis. papillitis and exudative retinal detachment. They are mostly associated with floaters in the field of vision. whole of the fundus should be scanned with a direct ophthalmoscope as far as possible. The posterior polar lesions are best seen with slit lamp. there is no pain.246 PEDIATRIC OPHTHALMOLOGY Pain : In contrast to anterior uveitis pain is almost always absent unless associated with anterior segment involvement specially in the form of secondary glaucoma. large or stringy.

other may develop satellite lesion adjacent to healed or healing lesion or there may be development of new lesions independent of original lesion. some haze may persist. The floor of the patch is white due visible sclera over which the choroid has been destroyed. traction detachment. Complication in acute anterior uveitis: Common complications are secondary glaucoma.DISORDERS OF THE UVEA IN CHILDREN 247 Causes of diminished vision in chronic posterior uveitis are Complicated cataract mostly posterior capsular opacification with polychromatic lusture. post neuritic optic atrophy. 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. there may be clumps of choroidal pigment away from the patch. Vitreous floaters which are hallmark of acute posterior uveitis subsides to a great extent. however. they are—Obliteration of the trabecular mesh work by swelling of the meshwork in the form of trabeculitis. Large areas of the choroiditis may result into a scotoma that becomes negative with passage of time. . Cystoid macular edema. secondary glaucoma and traction detachment. extensive contact of iris over the lens without the formation of actual synechia. periphlebitis. In some cases hypotony persists. scar over the macula and maculopapillar bundle. Obstruction of aqueous flow from posterior to anterior chambers is caused due to ring synechia. sub retinal neovascularisation. shallowing of AC resulting in increased iridocorneal contact that ends in formation of peripheral anterior synechia. The retina generally disappears over the healed patch but the vessels persist. Causes of secondary open angle glaucoma in acute anterior uveitis are many that may act separately or in combination. hypopyon and hyphaema. complete stoppage of aqueous production never takes place. The hypotony presents as flattering of cornea. membrane formation over macula and neovascularisation. massive deposit of KPs on the endothelium. The tension may be as low as 6 mm to 7 mm. macular hole. papillitis. These already narrowed channel may further be clogged by fibrinous aqueous. macular scar. Posterior vitreous detachment is more common with development of traction bands. vitreous bands. aqueous flow can be hampered due to small pupil. macular pigmentation. This is popularly known as ciliary shutdown. cystoid macular edema. Hypotony is general far less frequent than seen in acute anterior uveitis. Intraocular tension may be normal or raised. Other fundus changes consists of sheathing of blood vessels. changes in refraction. Some of the lesions get healed. A healed patch of choroiditis has an irregular margin with clumps of uveal pigment on the periphery. vitreous haze. Signs of chronic posterior uveitis are same as seen in acute choroiditis with changes due to passage of time. 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. hypotony. Hypotony In initial phase of acute iridocyclitis aqueous secretion by ciliary body is diminished more is the inflammation lower is the tension.

On long run the epithelium may become irregular and stains with fluorescein giving a fake impression of inflammatory keratitis.248 Corneal complications PEDIATRIC OPHTHALMOLOGY Transient epithelial edema. This is transient and reversed by use of cycloplegic. Cystoid macular edema and papillitis are common feature of loss of vision. Complication of chronic anterior uveitis These complications develop after days or months of unchecked uveal inflammation. There seems to be an unanimity in the theory that it is a degenerative condition. It is sterile in nature. Vitreous changes. Iris and pupillary changes. other hypothesis put forward are that its deposition is influenced by actinic rays. The exact cause of band keratopathy is not known. Hyphaema in acute uveitis is seen in cases of gonococcal uveitis and uveitis due to herpes simplex and zoster. A school going child following bilateral use of atropine may require near correction for the period of treatment. 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. They can be grouped in following categories : 1. It is due to the deposition of calcium carbonate and calcium phosphate in the Bowman’s membrane and anterior stroma. Keratopathy. exact causes of these are not well understood most probably they are toxic in nature. Loss of accommodation persists for two weeks following stoppage of atropine. The calcium deposited in band keratopathy is extra cellular. 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. Cycloplegic itself can cause iatrogenic loss of accommodation and unmasking of facultative hypermetropia. Hypopyon : Accumulation of pus in anterior chamber is common in many bacterial uveitis. 3. massive deposition of precipitates on the endothelium that may itself lead to stromal and epithelial haze. Generally there is grave lose of vision however there may be sufficient salvageable vision. 2. Lenticular changes. 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. The epithelium in the initial stage is normal with intact corneal sensation. Altered aqueous dynamics. It is most commonly seen in pneumococcal inflammation. 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 Behcet’s disease. Myopia is induced due to irritative spasm of ciliary body. Fundus changes. 5. Amblyopia and squint in children. 7. plasmoid aqueous and edema of cornea. 6. resulting into a chemical change between cornea and air causing . 4.

There are two possible modes of treatment. Iris nodules : There are many types of nodules that develop on the iris which fade away with treatment. Common nodules are Keoppe nodule. Signs Signs consists of horizontally placed sub-epithelial calcarious opacity with holes in between. it may take one to two years for the two ends to meet in front of the pupil. 1. not exceeding more than fifteen in each eye. This should remove all the deposits. A rare type begins in the center and spreads towards the periphery. They are seen on the pupillary margin and may project in the pupil. trauma. The calcarious deposits is rubbed with sodium EDTA 0. 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. May be slightly below the center of the cornea. some take just few days to disappear. Common iris changes are formation of nodules. The calcium crystals can be scrapped off the Bowman’s membrane leaving a clear stroma. these deficiencies represents the entry of the corneal nerves. Recently excimer photo keratotomy has given encouraging results. Sometimes there may be recurrent redness. the eye is bandaged with antibiotic and cycloplegic till the cornea is reepithelised. however. vary in number. 32. 39 Keoppe nodules are equivalent of mutton fat KPs. Generally posterior synechia develops at the site where this nodule comes in contact with the lens capsules. They develop from ectodermal tissue. glaucoma. The overlying epithelial is not invaded for a long time but ultimately the crystals protrode through the epithelium making it susceptible to infection. The progress is very slow.35 Children are more capable of mobilizing calcium than adults. failed keratoplasty.27 The corneal hemidesmosome may act as a lattice over which calcium crystals are built. posterior synechia. absolute glaucoma.5% for about 10 minutes.38 Iris changes in chronic anterior uveitis are generally associated with changes in pupil. Medical treatment is an outdoor procedure where under local anaesthesia the epithelium is scrapped off the band. lepromatous nodules and nodules of sarcoid. anterior segment ischaemia. tubercular. The band is white colour with a few deficiencies in between in circular fashion. It is presumed that an early and satisfactory treatment of underlying ocular pathology wards-off band keratopathy. Diminished vision is commonest symptoms which the patient attributes to underlying ocular disease. Busacca’s nodule. They are accumulation of large epitheloid cells and lymphocytes. Lamellar corneal graft and 2. Ocular causes of band keratopathy are : chronic anterior and pan uveitis in children.27. they are metwith in both types of uveitis. There is always signs of primary disease i. . syphilitic. peripheral anterior synechia. iris atrophy. Medical treatment. The band is wider in the mid cornea. others may linger for longer duration.e. chronic uveitis. if it fails strength of EDTA is increased to 1% and rubbed for 20 minutes. In late stages the cracks appears in the band.DISORDERS OF THE UVEA IN CHILDREN 249 a low CO2 and low pH in the cornea. They were thought to be diagnostic feature of granulomatous uveitis. Management of band karatpathy is difficult. increased translucency of iris and neovascularisation. watering and photophobia. chronic uveitis in young adults phthis bulbae.37 Symptoms Band keratopathy is almost symptomless except gradual diminished vision.

If the posterior synechia involves the whole periphery of the pupillary border it is called seclusio pupillae. They are generally located near the pupillary margin. Some of them may be benign not requiring any treatment. The nodules are generally hyperemic. Hyperemia of iris in syphilitic iridocyclitis is known as roseola. There are many conditions that may mimic iris nodule. They may be seen on the pupillary margin or in the stroma of the iris. Like other nodules the syphilitic nodules disappear with treatment.250 PEDIATRIC OPHTHALMOLOGY Busacca’s nodules : These nodules are less common. 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. They take weeks to months to disappear. The vessels form a lattice like pattern over the nodule. sometimes leaving a patch of atrophy behind.e. Peripheral anterior synechia may result even in absence of iris bombe i. An annular posterior synechia prevents the posterior chamber from communicating with the anterior chamber via pupil. Sacroid nodule : Iritis is most frequent ocular manifestation of sarcoidosis. They are more common in the pupillary margin. In rare instances one of the nodules may be converted in to a gumma. They are grayish yellow in colour some times new vessels develop round the base of the nodule. only one eye develops nodules and can be considered as papule. 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 periphery of the iris may touch posterior surface of the cornea resulting into peripheral anterior synechia. These pupil take a festooned appearance. Large single nodule has predilection for iris root. they are not diagnostic of any specific disease. Posterior synechia in chronic uveitis are generally broad and difficult to break when dilated with strong cycloplegic. Incidence of nodules is greatly reduced by systemic chemotherapy. The tubucular nodules are scattered throughout the iris and ciliary body mostly in the stroma. resulting in forward bowing of the iris causing the anterior chamber to be deep in the center and shallow at the maximum bulge. . It is directly related to amount of fibrin present in the aqueous. fewer and smaller than Koeppe’s nodule. size and number vary. They may get hyalinised on long run. Prolonged dilatation of pupil and nodules on the periphery may obstruct an anatomically narrow angle causing rise of tension. 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. They develop from mesodermal tissue at the periphery of the iris. Syphilitic nodules40 : The syphilitic nodules are unilateral even in case of bilateral involvement. to 1. However they may be allergic in nature.5 mm. around the collarette. The nodules of sarcoid look similar to that of tubercular nodule. such condition is called iris bombe. due to prolonged contact between edematous iris and corneal endothelium at the periphery. they vary in size from 1 mm. Tubercular nodule : Prior to the advent of anti tubercular drugs tuberculosis was thought to be commonest cause of endogenous both anterior and posterior uveitis. They are more hyperemic and vascularised than tubercular nodules. They are generally initiated by Koeppe nodules. They disappear with systematic chemotherapy and local steroids. Long standing posterior synechia may start anterior capsular opacification.

Altered aqueous dynamics in chronic uveitis: Glaucoma. it is seen in some chronic anterior uveitis.29. These patches may have sectorial removal of pigment. Areas of atrophic patch of full thickness transmit reflected light of retinoscope and retro illumination. generally at the site of previous nodule. Fluorescein angiography of iris in healed herpes zoster show occuluded iris vessels at the site of atrophy. are central vein thrombosis. Neovascularisation of iris is a rare phenomenon in iridocyclites. The outflow is also hampered due to fibrosis and hyalin deposits in the mesh work. this condition is known as occlusio pupillae. Formation and flow of aqueous may be normal. It should be differentiated from other causes of neo vascularisation of iris and ciliary body which in adults. Chronic swelling of ciliary body can cause forward rotation of lens iris resulting in angle closure glaucoma. sickle cell retinopathy. New vessels lead to formation of small patches of haemorrhage or may cause frank hyphaema. Long term use of cortico steroid locally or systemically in genetically predisposed eyes is a common cause of rise of intra ocular tension. These are areas of hyalinised scars from where pigment is removed.DISORDERS OF THE UVEA IN CHILDREN 251 The exudates may be deposited in front of the lens as a white membrane in the pupillary area. They are—Inflammatory edema of trabecular meshwork that reduce the diameter of the pore. A seclusio pupil may cause not only pupillary block but also angle block. iridotomies. this accentuates already distorted pupil. which may prove to be the important missed cause of loss of vision. making it difficult for the plasmoid aqueous to pass through. In herpes vesicles develops on iris which on healing leaves white areas on the iris. In case of anatomically narrow angle swollen peripheral iris may block the entrance to trabecular meshwork. There is a rise of intra ocular pressure. which may act separately or in combination. this is worsened by the use of long acting cycloplegic in anterior uveitis of long duration. maintaining normal intra ocular pressure in majority of cases of chronic anterior uveitis. The membrane may be dusted by iris pigment obscuring the third Purkinge image making it difficult to evaluate transparency of the lens. Causes of secondary glaucoma are multiple. It can be seen anywhere but is more common at the collarette. There may be neovascularisation of angle. Peripheral anterior synechia may plaster the face of the meshwork to form a pseudo angle in front of the original angle. 32 Rubeosis of iris is due to chronic iridocyclitis. The pupil adjacent to the patches of iris atrophy does not react to light or reacts weakly. 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. Glaucoma thus developed is generally chronic secondary open angle. essential iris atrphy and polycoria. which generally comes down following stoppage of the steroid. In Fuch’s heterochromic iridocyclitis patches of atrophy are most marked at the pupillary margin. Miotics are contra indicated in all . diabetic retinopathy. This is a non-inflammatory process. Iris atrophy In some types of anterior uveitis especially in herpes zoster and Fuch’s heterochromic iritis patches of atrophy develop. and Coats disease. Other condition that allows passage of reflected light through iris areiridectomics.

this hypotony persists for a long time or may develop after few weeks. these do not progress. A narrow inter palpebral fissure with reduced corneal curvature and shallow anterior chamber should arouse suspicion of hypotony. In prolonged cyclitis the zonules may give way resulting in subluxation or complete dislocation either spontaneously or with minimal trauma. after a few days aqueous production is resumed and intra ocular tension returns to normal or may rise to cause secondary glaucoma. This is a self limiting condition. It is common is patient with juvenile rheumatoid arthritis. Glaucoma should be suspected in all cases of anterior uveitis.5 mm is well tolerated by the eyes without any immediate deleterious effect. ultimately whole of the lens is opacified. the nucleus becomes yellowish. If the patient is on oral carbonic anhydrase inhibitor or local anti glaucoma drops they are withdrawn. Deposition of exudates in occulsio pupilli may be confused as opacity in the lens without a true cataract formation. Prolonged hypotony over months may results in total ciliary shutdown and result in phthisis bulbae. The cortical fibre in front of this may also be involved. Lenticular changes in chronic anterior uveitis Lenticular complications are common aftermath of chronic uveitis of all types. reactivate chronic uveitis and results in to pupillary block glaucoma. alpha agonist. posterior. Latanoprost which is an anti prostglandin drug that increases uveo scleral out flow is better avoided because it can reactivate uveitis. Cataract formation is more common in cyclitis than in posterior uveitis. The vitreous may develop floaters and liquefaction. Hypotony Reduction in intra ocular tension is common in initial stages of acute anterior uveitis due to hypo secretion of aqueous. intermediate and pan uveitis. Chorioretinitis and vasculitis may result in formation of vitreous bands . it is mostly due to altered bio-chemistry of aqueous. Short term systemic carbonic anhydrase inhibitors may be used to bring down the tension fast. The anterior capsule also develops sub capsular opacity later. The tension should be brought down by the use of betablockers. 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. hence it should be a practice to measure intra ocular pressure not only on first visit but also on subsequent visits also. It takes months to years for lenticular opacity to develop.252 PEDIATRIC OPHTHALMOLOGY types of uveitis as they worsens acute iridocyclitis. There is no specific treatment cycloplegic and steroid are continued. 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. however some times. Intra ocular tension as low as 6. A reduction of vision with normal macula and no evidence of papillitis should always warn about possibility of glaucoma. carbonic anhydrase inhibitors. Patients not responding to medical therapy are subject to standard glaucoma surgery. specially if the patient is on the steroid or cycloplegic. Broad posterior synechias that fail to break develop opacities under the synechia and just ahead of the synechia. The opacity is mostly posterior subcapsular in the form of polychromatic lusture. Cause of cataract in uveitis is not well understood. vitreous or lens in various proportion. anterior.

parsplanitis. vitreous detachment and liquefaction. copious discharge. phthisis. probable cause of uveitis. bacteria are the commonest causative factors. peripheral vaseulitis and chronic papillitis or post neuritic optic atrophy. proptosis. . exudative retinal detachment. Pan uveitis Involvement of all the parts of uvea in an inflammatory process24 is called pan uveitis. Fundus changes consists of : Macular edema. peripheral retinal degeneration. Otherwise it may be frustrating. part of the uvea involved. Management of uveitis Management of uveitis may be very simple with recovery without recurrence or complications. Commonest parasite causing pan uveitis is toxoplasmosis. Aids. neovascularisation. hypotony. The course tends to be chronic with fair prognosis. Posterior vitreous detachment is more common than detachment from the base. glaucoma. it may be infective or sterile.DISORDERS OF THE UVEA IN CHILDREN 253 leading to rhegmatogenous retinal detachment. Treatment depends upon its onset. however. Panophthalmitis Panophthalmitis is an acute superative inflammation of eye as well as periocular structures basically it is an endophthalmitis that crosses the scleral barrier. changes of conjunctivitis or sloughed hazy cornea. duration and treatment undertaken. massive hypopyon and restricted movement. syphilis. retina and optic nerve. Complications of posterior uveitis consists of loss of vision. sarcoidosis and Behcet’s disease. Clinical presentation in the eye vary according to etiology. Among infective organism. Commonest source of infection is penetrating injury either accidental or surgical. Vogt Koyanagi Harada syndrome. Epistein-Barr virus. it can have both acute and chronic onset with or without recurrence depending upon the etiology. brucellosis and lyme disease. one eye may be involved earlier than the other. Endophthalmitis Endophthalmitis is a severe form of pan uveitis with involvement of vitreous. The signs and symptoms of pan uveitis are a combination of both anterior and posterior uveitis. It can be bacterial or viral. scattered scotomas in the field. The organism may reach the eye via the blood stream. long drawn with complications. Viral endophthalmitis is unknown. Common bacteria are tuberculosis. The viruses involved in pan uveitis are herpes zoster and simplex. optic neuritis and optic atrophy. With profound loss of vision. All the parts may not be equally involved. pain. Generally pan uveitis is discussed under chronic uveitis. rubella. rubeolla. which invariably produces acute endophthalmitis while fungal endophthalmitis develops late and progresses slowly. severity. tolerance of the patient towards therapy and compliance. may spread from sloughing corneal ulcer. The inflammation is always intra ocular. It is generally unilateral. macular pigmentation papillitis. traction detachement complicated cataract. It is generally bilateral. Patches of chroiditis and chorioretinitis may be present. edema of lids. associated complication. Conditions that have uncertain etiology are—Sympathelic ophthalmia. Endophthalmitis can be acute or chronic.

Atropine is strongest of all cycloplegic. photophobia. 2. Specific antibiotic for tuberculosis. Instillation of cycloplegic also causes much needed mydriasis that keeps the pupil dilated. 4. Bring inflammation under control. breaks posterior synechia. Some times increase in concentration may be required to produce desired therapeutic results. this means that two drops contain 1. Non specific can be 1. 3. it is an alkaloid. requiring frequent use for long period. syphilis. Action of these drugs are not neutralized by miotics in therapeutic dose. Cycloplegics are metabolized faster in inflamed uvea. Cycloplegics are used as drops. Short acting—Home atropine.2 mg and such two drops when absorbed through conjunctiva and nasolacrimal duct will produce toxicity. As drops they may be used singly or in combination of two cycloplegics or a cycloplegic with mydriatic. atropine. Drops should be avoided because each moderate sized drop contains 0. prevents post synechia. Cycloplegics : Purpose of cycloplegic is to relieve spasm of ciliary body which is a major cause of pain in and around the eye. cortico steroid.6 mg. once the pupil is dilated it reduces glare. Absorption through .e. Dry fomentation is better as it is convenient to apply. toxoplasmosis etc. Commonly used concentration is half to one percent as ointment and one to two percent as drops. they may be added to cycloplegics to enchance mydiratic effect of the later. lacrimation. Reduce discomfort i. Prevent and treat complication as and when they arise. Atropine is used as sulphate either as ointment or drops. Out of all cycloplegics only atropine is available in ointment and injectable form. ointment and subconjunctival injections. Physical : Hot fomentation. pain. immuno suppressive drugs. cyclopentolate and tropicamide. 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. of atropine.e. Similarly each drop of two percent will contain 1. Eliminate infective process. non steroidal anti inflammatory drug. 2. The cyloplegics have local as well as systemic action especially with long acting drug i. Medical treatment of acute anterior uveitis consists of Cycloplegic. cyclopentolate with phenyle pherine. Treatment of acute uveitis is divided into non-specific and specific treatment. 2. hence their action passes off faster in uveitis and require more frequent instillation. Concentration of more than two percent is hazardous in children. The eye is fomented for three to five minutes at a time for two to three times a day.254 PEDIATRIC OPHTHALMOLOGY Aim of the treatment for acute anterior uveitis is to 1. This gives some relief from pain in the eye. which is within therapeutic limit. Cycloplegics are parasympatholytic agents they are of two types : 1. Commonest combination is that of tropicamide. Dark glasses : In initial stages it helps in reducing photophobia. when detected. Mydriatics themselves do not have any therapeutic value in treatment of uveitis.2 mg that is more than upper limit of safety. All cycloplegics are mydriatics. Long acting—Atropine.

Sympathomimetic drugs have no role in uveitis except as adjuvant to cycloplegics. Once synechia are broken maintain mydriasis with other cycloplegic or combination of cycloplegic and mydriatic drugs. which is not responsive to local miotics. epinephrine and procaine/xylocaine. the pupil will most probably not dilate with subconjunctival injection of mydricane that is a combination of atropine. every half an hour for four to five instillation. . rise of body temperature. beta blockers and alpha agonist. Fortunately this passes off within a few days after stopping the drug. the drug should be withdrawn and replaced by home atropine hydrobromide 2% drops or cyclopentolate one percent. Exact mode of action of steroid is not well understood. Steroid are very cost effective provided they are administered as per norms otherwise they can be potentially dangerous both locally and or systemic. To summarise Avoid atropine drops in children. Similarly cyclo pentolate is known to aggravate iritis. rather than increasing strength and frequency of drops in children. Latanoprost and cyclopentolate may aggravate uveitis. most widely accepted theory is that they act as potent anti inflammatory agent. unmasking of facultative hypermetropia. In case of glaucoma control tension by acetozolamide. They do not change the basic pathology. Inform parents about possible side effect of atropine. Atropine is one of the local drugs that is frequent cause of dermatitis medicamentosa. in eyes with anatomically narrow angle atropine can precipitate angle closure glaucoma. Cortico steroids Most commonly used drugs in uveitis is steroid. If pupil does not dilate with BD dose. In case there is rise of tension it should be brought down by systemic acetazolamide. it is better to add five percent phenyl pherine. In case of allergy to atropine. Fortunately angle closure glaucoma following mydriasis is rare in children. Common side effect of atropine are—Glare. Miotics are contra indicated in uveitis and do not counteract cycloplegic action. constipation. Steroids are effective in all types of uveitis. loss of accommodation. dryness of mouth. However many of the patients allergic to atropine are also allergic to home atropine. local beta blockers or alpha agonist. flushing of face. The steroids protect the tissue from the onslaught of inflammation till the immune system is capable enough to overcome inflammation by reducing phagocytes. are easy to be administered. thirst. They also do not counteract drug induced mydriatic in therapeutic dose. They may release uveal pigment in aqueous that may be mistaken as flare.DISORDERS OF THE UVEA IN CHILDREN 255 nasolacrimal duct can be minimized if the punctum is obliterated by finger for one minute just following instillation. The patient may require local steroid ointment to treat allergic blepharitis and dermatitis of the lids. evaluate and adjust their dose. If pupil does not dilate with atropine BD or combination of tropicamide / cyclopentolate / home atropine with phenylpherine.

Daily single dose or alternative dose reduces adrenalin suppression. they form an oily layer over cornea that reduces vision and interferes with examination by slit lamp or opthalmoscope. Retrobulbar injection is given less frequently mostly for optic nerve involvement and causes less rise of IOP. 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. anterior and posterior subTenon and retrobulbar. often enough and long enough. The above dictum is true for systemic and local steroids. The drops are available as clear solution or suspension. SubTenon and retrobulbar injections are given for lesions behind the lens and suspensory ligament. Injectable solutions are also available in soluble state or suspension form. Subconjunctival and subtenon injections are absorbed by conjunctiva which passes it over the aqueous or via sclera. per day. Commonly used IV steroid are methylprednisone and ACTH. The commonest drug used for daily and alternate day regime is prednisolone in a dose of 1 mg to 1. long acting and short acting. The drops can be clear solution or suspension. 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. cataract is also less frequent following retrobulbar injection. As steroid has no effect on micro organism they should be used under umbrella of anti microbial drugs other wise the condition may worsen. dexamethasone or betamethasone. Steroids can be divided into two groups i. which are absorbed quickly requiring repeated injection every day or alternate day till the inflammation has been brought under control. soon enough. They are not to be used in acute states. tablets and injectable forms. Both short and long acting drug can be used. They may be give intra muscular or IV as well. The clear solution have better corneal penetration hence are preferred over suspension. Depot injections are suitable for chronic cases. In an adult this may be as high as 60 mg to 120 mg.256 PEDIATRIC OPHTHALMOLOGY fibrosis and neovascularisation24.5 mg per kg. The divided dose is more effective but produce more side effects. The tablets can be given daily or alternate day or in pulse form.e. Injectable steroids can be given as sub conjunctival. A part of subconjunctival injection is bound to absorbed by systemic circulation. Ointments are poorly absorbed. The steroid should never be stopped abruptly. they form depot from which the drug is released. The injection may be repeated every fortnight. General principles of use of steroid are : Use enough. The advantage of ointment is that it has prolonged effect hence can be used during sleep. Children are put on steroid in consultation with pediatrician. Both groups are available as local drops and ointments. They are generally tapered over weeks or months. 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 . The suspensions are absorbed slowly from the site. The daily dose can be given in divided dose of TDS or QID or as a single dose in the morning just before breakfast. If the condition requires prolonged use of steroid they may be given every month. The solution are generally short acting i. hence they cause less side effects. Local steroids Local steroids are available as ointment as well as drops.e. The frequency of instillation depend upon severity of uveitis.

They are also used in cystoid macular edema. They are used to enhance preoperative mydriasis and maintain intraoperative mydriasis. Reactivation of dormant infective process. Patients on systemic steroids require less local steroid. moderate mydriasis. cytomegalo virus infection. and reduce aqueous flare. diclofenac sodium ad potassium. It has been observed that eye under steroid therapy requires less cycloplegic to dilate the pupil. worsens existing diabetes. They also antagonise vasodilatation. ointments. Systematic side effects produce by NSAID are—hyper acidity and peptic ulceration. toxoplasmosis. their simultaneous use with steroid results in lowering requirement of steroid. as expected such potent drugs have far reaching side effects both local as well as systemic. corneal staining. Side effect of steroids: Steroids are most potent anti inflammatory agents with a very wide spectrum utility. indomethacine and recent drugs like ibuprofen. lysosmal enzyme. edema. rise of intra ocular pressure. They can be diluted as much as one tenth. hypertension. nimesulide. herpetic disease. 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. They hardly produce any ocular side effect. They do not replace steroid altogether. Systemic use produce all above side effects along with following in various combination : Precipitate latent diabetes in steroid responders. They include age-old analgesic like salicylate. The patient should follow the instructions and agree for adequate follow-up and have a knowledge of side effects of antimetabolites. lymphokinase. The steroids can be tapered not only in frequency but also in strength. salt retention leading to weight gain. personality change. Systemic allergy has been reported. They also block polypeptide of kinin system. Local steroids are capable of producing corneal complication and rise of intraocular tension like systemic used steroid. Newer drugs are being added frequently claming to be more effective and less toxic. Local and periocular use of steroids produce less side effects but are not altogether free from them. Cushing syndrome. They block the synthesis of enzyme prostaglandinsynthatase and cycloxygenase. All NASID are potent analgesic and moderate antipyretic. They can be used to prevent recurrence for long time. They antagonise prostaglandin induced miosis which may otherwise be atropine resistant. ketarolac etc. Local drops are usually used twice a day.DISORDERS OF THE UVEA IN CHILDREN 257 day. oral tablets and injection. Non steroidal anti inflammatory drugs: These drugs are mostly prostaglandin inhibitors. There is potassium loss. not loosing much effectiveness when used for long time. Such prophylactic use of antiviral and antifungal drugs have doubtful role. acne and poor healing. It is better to use a broad spectrum antibiotic drop along with local or systematic steroid to ward off possible secondary infection. ptosis. Their use is restricted to mild to moderate anterior uveitis. generilsed weakness. There should be no contra indication of their use like tuberculosis. they may even produce systemic side effect to less extent when used for a long time. They are available as local drops. sleep disturbance. posterior subcapsular opacity and ptosis. Local complication include delayed healing of both post traumatic and infective process. . fungal infecton. aspirin.

average being 5%.258 PEDIATRIC OPHTHALMOLOGY The drug should always be prescribed under supervision of oncologist. Hence side effects encountered in ophthalmic use are fewer in comparison to malignancy. All of them produce cytopenaea and liver toxicity. leprosy (rare in children). uncertain etiology. These figures are definitely less when compared to adult uveitis. some are seen less commonly in children. 4. Combination of steroid with cyclosporin reduces total dose of cyclosporin which is available as drops. aids. Other organism causing uveitis are pneumococcus. 42. juvenile spondylitis. methotrexate. The dose used in ophthalmology is comparatively low. herpessimplex herpeszoster. Connective tissue diseases effecting joints43 : Juvenile rheumatoid arthritis. herpes simplex syphilis. Prognois is poor due to chronicity. Their benefit should always be weighed against their potential dangers. poor response and poor compliance. toxoplasmosis. It can be congenital or acquired. 2. inflammatory bowel disease. late diagnosis. it is important not to miss uveitis in children because it can be bilaterally sight threatening. Unilateral chronic uveitis (white uveitis syndrome) may go undetected. Infective : Generally chronic but may be acute. Baheet’s disease and serpiginous choroditis. One third of childhood uveitis have undetermined etiology. Specific type of uveitis in children: While pediatric population comprises of 15% of general population. Endogenous uveitis is commonest form of childhood uveitis. juvenile Reiters syndrome. . retinal vasculitis. various fungi. Cyclosporine does not come under true antimetabolite. 3. Cytotoxic drugs are not available as drops or ointment commonly used drugs are cyclophosphamide. propioni bacterium. toxocara. 6. Girls are effected more than boys in a ratio of 6:4 except sympathetic uveitis where boys outnumber girls. Incidence of bilateral uveitis in children is more than adult uveitis. They are mostly used in sympathetic ophthalmia. They may be due to tuberculosis. They may be used in parsplanitis. however. Acute anterior uveitis is less frequent than chronic recurrent uveitis. Some of the peculiarities of pediatric uveitis are : 1. 5. syphilis. cyclosporin. staphylococcus.Harada disease. it is basically an antibiotic. lyme disease. gonococcal. incidence of pedriatric uveitis ranges between 2 to 10 percent27. azathioprine. Though all adult form of uveitis can inflict patients under fifteen. chronic cyclitis. 8. leptospirosis. chlorambucil. bacillus cereus. 2. streptococcal. Acquired— 1. Pediatric uveitis can be: Congenital—Toxoplasmosis. Their role in childhood paraplanitis is questionable. 7. rubella. Vogt-Koyanagi.

however. asexually and form a protective cyst within the cells where the cyst remains protected from the immune system hence dormant occasionally. The congenital lesions have predilection for brain and retina. Masquerade syndromes : These disorders are mostly non inflammatory in nature diagnosed as chronic idiopathetic uveitis. Toxoplamosis uveitis Toxoplasmosis has emerged as commonest cause of posterior uveitis. Thus only one of the pregnancies is at risk. intraocular foreign body. medulo epithelioma. All except Fuch’s heterochronic iritis generally produce bilateral chronic pan uveitis. A mother who is sero positive for toxoplasma before first pregnancy will not infect the foetus nor the subsequent foetuses. acquired that is very mild and self limiting does not require any treatment and gives immunity to reinfection. half cooked meat. Fuch’s heterochronic uveitis. a rising titer is indicative of recurrence that is very common. . 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. So long as the organism is intracellular it does not give a positive serological test. sensitization to lens protein following rupture of lens capsule. 5. It is caused by protozoa. However a pregnant mother who is infected during pregnancy can transmit the infection to the growing foetus. retained in intraocular foreign body. Coats disease. It is also transmitted by way of inhalation. Other causes are ischeamic ocular inflammation following squint and retinal surgery. All these are very infrequently seen in children. toxoplasma gondi that is an obligatory. Toxoplasmosis occurs in two forms i. However now it is known that these lesions can be acquired also. the patches of which get calcified in long term. From the lymph nodes they get circulated in regional circulation44. through blood transfusion. Common examples are—Retinoblastoma leukemia. 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.e.Toxoplasma can be transmitted via unpasteurised milk. They can be life threatening malignant lesions or non malignant lesions in children. skin wounds and transplants. Scattered smaller patches away from the posterior pole without visual symptoms. peripheral retinal detachment. There is no prophylaxis against toxoplasma. In the brain it produces encephalitis. but recurrence due to rupture of toxoplasma cyst is possible. Titer of serological test is not related to ocular involvement.DISORDERS OF THE UVEA IN CHILDREN 259 3. In eye the lesion are patches of retinochoroiditis in the posterior pole on the macula. Once it reaches human intestine it multiplies there and pass to the regional lymph nodes. Cat is the first definite host and humans are the intermediate hosts. 45 In intermediate host toxoplasma multiplies within. 4. Trauma : Sympathetic ophthalmia. The foetus is infected via plancental blood or by direct extension from a dormant lesion on the uterine wall. close enough to the macula to cause loss of vision or juxta papillary. intra cellular parasite. Pars planitis is common in children that produce intermediate uveitis. Congenital toxoplasmosis Once it was thought that all ocular and brain lesions of toxoplasmosis were congenital and consequently lesion as recurrences. Behcet’s syndrome. these lesions are detected only on routine fundus examination by indirect ophthalmoscope. Others : Vogt Koyanagi . sarcoidosis.Harada syndrome.

The healed patch is not noticed unless looked for particularly. Complications include cataract. Some times there may be nystagmus unilateral cases are generally amblyopic. 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. Meat should be well cooked. Management There is no prophylaxis for toxoplasmosis. A child with congenital toxoplasmosis has three Cs representing Chorioretinitis. Posterior vitreous detachment is common. mental retardation. there is microcephally and mental retardation. opportunistic systemic infection in immuno compromised individual. tubercular choroiditis. There is no treatment for infected cats as cats may be asymptomatic. Once diagnosis has been confirmed the treatment is by anti-toxoplasmosis chemotherapy. microcephaly and squint.45 2. subretinalneovascularisaton. In severely infected child. . 3.260 PEDIATRIC OPHTHALMOLOGY All lesions are generally bilateral : The lesions starting at early pregnancy may heal and the child born with a patch of healed choroiditis. Some times the lesions may be punctate. Other investigation are (1) serological test for serum antitoxoplasma antibody. Any titer of serum antibody is significant. The blood vessels pass over the patch. exudative retinal detachment45 Vitreous haemorrhage. A healed patch is oval in shape may be larger than disc. On examination a typical patches of central choroiditis is visible. has patches of pigment on the periphery. herpes simplex chorio retinitis. There are no cells in the vitreous or aqueous. the retina is edematous. Differential diagnosis consist of congenital coloboma of macula. IgG and 3 IgM titer . However. CT and MRI may show small lesions in the cortico medulary region. Best way is to look after personal hygiene. vitreous may have cells. cytomegalo virus infection. The center of the patch is white. They are more common in teen age girls 24. Children with ocular lesion are generally diagnosed when they are examined for infentile strabismus which is invariably estropia or diminished vision. Calcification and Convulsion. nystagmus. the child may have only ocular lesions.42 Recurrences generally occur either on the periphery of the healed lesions or as satellite lesion adjacent. positive IgM is indicative of recent infection. On X-ray there is invariably intracranial calcification. A child infected late in pregnancy has active lesion in the posterior pole with fluffy appearance.A positive IgG may be present without ocular involvement. Recurrence is seen between ten years and forty years. retinoblastoma. scattered on wide area of retina without much vitreous reaction. There are no pigments on the periphery. avoid raw vegetables. Diagnosis : Diagnosis of congenital toxoplasmosis is straight forward when the child is brought with systemic symptoms of convulsion. Elisa test. All neonates with convulsions should undergo fundus examination and X-ray skull. glaucoma. Other involvement’s are . cortico steroid. photo coagulation of peripheral lesions and management of uveitis.papillitis and vasculitis. the detached vitreous is dusted with vitreous precipitates similar to Kps.

49 In children herpes simplex uveitis is generally secondary to herpetic keratitis but can be due to systemic involvement as well. Rubella uveitis Rubella uveitis in children is part of congenital rubella and over shadowed by congenital cataract and glaucoma. minocylin and atovaquone. Other common age groups to be affected is between 6 months to five years. clindamycin. Pyrimethamine can cause marrow suppression. It may occasionally be life threatening. cells. Sulphadiazine alone. The chemotherapy should be continued for weeks.DISORDERS OF THE UVEA IN CHILDREN 261 Chemotherapy Drugs available for treatment are—Pyrimethamine. flares. 42. tetracycline. 5 mg. It can cause non granulomatous anterior uveitis with or without evidence of cutaneous or corneal involvement. For active lesion a combination of pyrimethamine triple sulpha with systemic steroid is the best combination available. The neonate gets the infection during passage through birth canal and lesion develops between two weeks to 18 months after. Eye with multiple recurrence and vitreous haemorrhage may require vetrectomy. spiromycin. It used to . pupil is generally small and dilates poorly with atropine. of leucovorin (folinic) acid is given twice a week. vancomycin. Herpes simplex uveitis24. Systemic anti viral drugs should be administered in consultation with pediatrician. sulpha methoxazole with trimethoprim. It can inflict any age of both sexes. Syphilitic uveitis in pediatric age group Syphilis has been called greatest imitator in clinical medicine. In seventy percent of cases it produces non progressive chorioretinitis giving a pepper and salt fundus appearance with good central vision and normal ERG. It can also cause chorioretinitis and retinitis in older children. and photophobia. The condition is painful with intense lacrimation and photophobia with evidence of active keratitis or stromal keratitis. Steroid alone may suppress an acute attack for some times only to flare up later. triple sulpha. The dose should be administered in consultation with pediatrician who should also monitor for side effects. but chemotherapy may prevent recurrence. posterior synechia and occasional hypopyon. however. nausea and leucopenia. It is mostly produced by type II herpes simplex virus. Other causes of pepper salt fundus should be excluded. Both dilator and sphincter muscle may be involved. three times a day and weakest possible steroids under supervision if cornea is not involved. Management is by way of cycloplegic for lacrimation. type I can also cause cutaneous lesions that are self limiting. there may be iritis or iridocyclitis. Pregnant mothers who test positive should be given full course of treatment in consultation with gynecologist and neonatologist. the steroid is generally tapered after seven to ten days. Once the lesion has healed no treatment is required. The iris stroma is severely damaged leading to partial hole formation that trans-illuminates reflected ray. Hence it is necessary to get weekly WBC count to overcome the side effects. There is no specific treatment. congenital rubella can be prevented only if the mother has been immunized in childhood. In some percentage of cases. Virus may migrate to trigeminal ganglion only to be reactivated with reduced resistance. all ethnic groups and involve any part of body in various combinations.

however. Non treponemal test V. Corneal endothelium may be damaged by KPs and secondary guttatae may develop.24 There are multiple synechia resulting into miosis which does not respond to atropine.L. Finding of the uveitis may only be visible after the cornea has cleared. corneal reaction may be severe enough to obstruct examination of the iris and anterior chamber. salt and pepper spots may be confused as retinitis pigmentosa.Micro hemo agglutination test. Difficulty arises when uveitis in children is suspected to be due to secondary or tertiary stage. Interstitial keratitis related anterior uveitis is easy to diagnose and responds well to treatment.262 PEDIATRIC OPHTHALMOLOGY be a major cause of uveitis both acute and chronic before present era of antisyphilitic treatment came into vogue. retinal phlebitis. Diagnosis of syphilitic uveitis is often missed as it is generally thought that syphilitic uveitis is only seen in adults. The congenital syphilis in initial years are comparable with secondary syphilis becoming tertiary.39 Posterior segment involvement results in salt and pepper spots of chorioretinitis. chorioretinitis. (4) may be acquired in teens. hyaline strands may project in anterior chamber. if not treated. acquired syphilis can also be seen in pediatric age group due to frequent child abuse and precocity.30 Other syphilitic lesions include chronic iridocylitis. The diagnosis is confirmed by variety of serological tests. The iris may be atrophic.45 Pupillary changes can be due to involvement of iris stroma or part of neurosyphilis.45. They can be (1) Nontreponemal—VDRL and rapid plasma regain (2) Specific treponemal—FTA . There is no visual loss. It is generally bilateral and peripheral may involve posterior pole or a quadrant. however. In children commonest form of uveal involvement is associated with congenital syphilis. flare and cells in anterior chamber are common. vitreous opacity. In later stages. The laboratory investigations in syphilis are same for all ages. Treatment Treatment of syphilitic uveitis with interstitial keratitis does not pose much difficulty. Acute iritis can start as network of fine capillaries. vascular papules and nodules. (2) active at birth. In all such cases it should be confirmed by FTA-ABS and treated as acquired syphilis in consultation with pedriatician. It can be acute (1) Associated with interstitial keratitis (2) Without involvement of cornea (a) Acute iritis (b) Diffuse chorioretinitis.24 There is no primary stage in congenital syphilis.D.Flourescent treponemal antibody absorption MH4TP .47 Hence uveal involvement in children may show features of secondary or tertiary syphilis. . Uveitis in interstitial keratitis preceeds corneal involvement. becomes negative in late stages even with or without treatment but FTA-ABS never become negative.R. angle may get hyalinised resulting in glaucoma. Uveal involvement in children may be (1) congenital and quiet at birth.ABS . (3) may become active at teens.

atropine may be replaced by 1% cyclopentolate. Topical steroids are administered as per severity. In children. Presumptions In case of presumed uveal tuberculosis—the child has raised ESR and lymphocytosis. A self limiting acute iritis develops following cutaneous injection of tuberculin. drops may be used in older children with caution. This is non granulomatous and without synechia. Diagnosis Diagnosis of uveal tuberculosis is one of baffling problems. aqueous flare and cells in anterior chamber. It is done by exclusion. Vitritis is common. The sensitization depends on systemic resistance and host immunity. Macular involvement leads to early and permanent loss of central vision. Steroids drops may have to be used for months and the child monitored constantly for possibility of side effects. 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. In older children peribulbar steroid can be given in recalcitrant cases. Posterior uveitis is less common in children than in adults.DISORDERS OF THE UVEA IN CHILDREN 263 Local treatment consist of atropine ointment under five. 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. presumption and confirmation. A healed macular involvement may be confused as congenital coloboma of macula or toxoplasmic chorioretinitis. there may be papillitis. Common presentations are : (1) Miliary tubercle in the choroid secondary to tuberculous meningitis (2) Circumscribed lesion in the posterior pole (3) Involving the macula. Surprisingly involvement of uvea is rare along with active pulmonary lesion. Tuberculosis should be differentiated from other forms of granulmatous uveitis like toxoplasmosis. Uveal involvement is generally hematogenous. either as solitary or miliary tuberculosis. and sarcodosis. following primary infection a fast developing hypersensitivity takes place due to low or absent immunity. broad posterior synechia. It is possible to get either anterior or posterior uveitis separately or together to results in a pan uveitis. The uveitis does not respond favourably to local cycloplegic and steroids or becomes worse. syphilis. Once desired mydriasis has been achieved. Inflammatory and destructive process is due to hyper sensitivity while healing is due to host immunity and host resistance. If there is significant . edema of iris and ciliary body. As anterior and posterior uvea have different blood supply. Uveal involvement is generally granulomatous with mutton fat KP. to begin with its can be as often as hourly to be reduced to three times a day. Oral steroids are not required. Formation of Koeppe nodules at the pupillary margin. exdates over the anterior lens capsule. Tuberculoma is now a days extremely rare. Formation of tubercular nodule all over the uvea. the involvement is independent of each other but can be concurrent. 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.

(This generally comes out to be negative. the puppies get infected via two sources—1. Once the pupil has been dilated atropine may be replaced by cyclopentolate or a combination of cyclopentolate with phenylephenine. If this test is negative 25(TU) is used for confirmation. However. They only hatch in the intestine of either dogs or human being. DLC and ESR. The systemic infection of by toxocara has two distinct stages. IgG and IgM. hence they are not excreted in the feces of human being. Involvement of eye is commonly referred to as ocular toxocariasis. The larvae do not mature to become mature worm in human beings. brain. from where they are carried to distant organs including eye. .57E Confirmatory investigations : (1) Routine TLC. nor they return to the gut.50 The puppies that are most infectious. Any reaction even erythema is suggestive of tubercular origin.264 PEDIATRIC OPHTHALMOLOGY improvement other systemic anti tubercular antibiotic agents are added in consultation with pediatrician. The second stage of infection develops in the intestine of a toddler who may ingest either the soil or food contaminated with by ova. lungs. The child should get minimum three drugs for systemic anti tubercular chemotherapy for 6 to 9 months. muscle and eye. (2) X-ray chest to exclude pulmonary involvement is undertaken. Management of tubercular uveitis Management of tubercular uveitis consists of local treatment by cycloplegic and steroids. Purified protein derivative is used.42 In both instances this ova hatches in the small intestine to form larvae. Toxocara infection of uvea (See page 489 also) Toxocarasis is a nematod infection. The larva penetrates the intestinal wall and is taken up by systemic circulation to reach distant organs. The eggs can survive in the soil for years without hatching. a positive test is seen in children who have received BCG as a part of immunization or suffered from tuberculosis in the past. The ova form larvae in the intestine that penetrate the intestinal wall. Some larvae reach the trachea and are coughed up. Local steroids have to be used for long time than other non infectious uveitis and gradually tapered off over months. It is caused due to ingestion of embryonated ova of an intestinal parasite—Toxocara canis round worm of the dogs. The coughed larvae is reswallowed to reach the intestine and get converted into an adult worm. that are excreted in feces of the dog. Polymerase chain reaction is very sensitive in pulmonary tuberculosis and less sensitive in extra pulmonary tuberculosis. Posterior synechia of tubercular iridocyclitis are difficult to break hence they require prolonged use of strong cycloplegic like atropine used with usual caution. (1) Stage of viscereal larva migrans. Other test : Anti tubercular immunoglobin IgA. Skin test (Mantoux test). a more common way is ingestion of infected food and 2. Less common by trans placental infection.000 eggs per day. First 5(TU) of PPDT is used as interdermal injection and reaction is red after 48 hours. It is a c ommon cause of chronic posterior uveitis.) (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. (2) Distant spread to liver.51 A female adult worm produces 200.

Children are mostly brought with strabismus. may be discovered in adults by chance. leucocytosis. cough. may be surrounded by hard exudates. diffuse unilateral sub acute neuro retinitis (DUSN)50.51 There is a latent period of few years between visceral larval migrans and ocular toxocariasis. flare. exact cause of this preference is not known.51. There may be vitreous bands extending from the lesion to the macula.DISORDERS OF THE UVEA IN CHILDREN 265 Visceral larva migrance is characterized by malaise. 1. Retinal vessels disappears in the lesion. raised yellowish white area. fever. This type of lesion is generally seen in younger children who are brought with either diminished vision squint or white reflex in pupillary area. Peripheral retinal granuloma. Ocular toxocariasis is always unilateral condition. 2. disc or both causing traction displacement of macula that may present as pseudo squint. eosionphilia and cutaneous lesion without ocular manifestation. On examination the eye is generally white with minimum cells and flare in aqueous. Less common presentations are—Exudative retinal detachment. or extensive tractional retinal detachment.5 years. optic neuritis unilateral pars planatis. Peripheral granuloma 3. Tractional or rhegmatisgenous retinal detachments are common. There may be involvement of anterior segment with cells. There may be peri retinal gliosis with traction lines. As the lesion is peripheral vision remains unaffected unless macula is involved. even hypopyon. Average age of a child with visceral larval migrans is two years while ocular toxocariasis is seen between 18 months to 18 years. The vitreous is infiltrated with cells. All races are equally affected. hepato spleenomegaly. Once this larvae reache the eye. This is generally associated with parsplanitis. Its incidence in under developed countries must be more than reported. Chronic endophthalmitis. average being 7. more rarely with a white reflex in pupillary area. Chronic endophthalmitis. The peripheral lesion is a solitary mass associated with extensive peripheral gliosis. It is rarely seen with systemic involvement. diminished vision or rarely white reflexes in pupillary area. Ocular toxocariasis generally produce one of the following types of lesions. macular edema and cataract further worsens the vision. The vitreous band may cause retinal breaks that lead to localized. seen above 6 years of age. . there may subretinal haemorrhage with serous retinal detachment. The lesion is one or two disc diameter. Anterior segment signs and symptoms are minimal but not altogether absent. it can produce various types of posterior uveitis. Solitary posterior polar granuloma is most common type of lesion that causes diminished vision due to involvement of macula. Solitary posterior polar granuloma in the macular region or between the macula and nerve head. posterior synechia. 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. diffuse chorioretinitis. In late cases a cyclitic membrane may develop. Ocular toxocariasis is a late feature of systemic involvement. Vision is grossly diminished and it becomes difficult to visualize the fundus. 51. It is mostly seen in age group 6 to 14 years. seen mostly in male. 52 A typical lesion is posteriorly placed.

Corticosteroids : Corticosteroids can be used in conjunction with anti helminths or alone. Vitreous surgery not only removes all inflammatory process and antigen but also vitreous bands and helps in placing of scleral buckle. however. Anti helminths may cause increased inflammatory reaction due to deaths of the organism. regular deworming of puppies from second week has been recommended. A titer of 1:8 in serum is diagnostic. toxocariasis does not show intraocular calcification on X-ray and ultrasonography. Diagnosis Diagnosis of ocular toxocariasis is always presumptive. 2. (A) Medical I. Prophylaxis. Anterior chamber aspiration does not show tumour cells but gives polymerase chain reaction to toxocara antigens. Most reliable test is ELISA test to detect toxocara antibodies in serum. sometimes serum may give negative results while aqueous/vitreous give positive results that is reliable. Cryo freezing24: When laser is not available trans scleral cryo coagulation of peripheral lesion may be attempted under direct vision. They can be used orally or by way of periocular injection. It is virtually impossible to deworm stray puppies that are the main source of contamination of soil. however. All cases of unilateral granuloma in a white eye should be suspected to have ocular toxocariasis unless proved otherwise. Therapeutic. so are the skin changes of viscerallarval migrans. Anti helminths whenever administered should be given under umbrella of full dose of cortico steroids in uveal toxocariasis. it is extremely rare to get bilateral toxocariasis. The material removed by vitrectomy may be used for confirm diagnosis. X-ray orbit. Contact with infected puppies is so common in general population that history of contact with puppies is of hardly any use. Vitrectomy may be used to remove posterior cyclitic membrane as well. Role of routine deworming of child may not be sufficient to expel the larvae. aqueous or vitreous. should be tried only when the nematode is away from posterior pole. Laser photo coagulation51 of live nematod when detected. . Other disorders that form differential diagnosis are : Coats disease. persistent hyper plastic primary vitreous. Anterior segment reaction is managed by local cycloplegies and steroids. CT and MRI are negative for intraocular calcification. retinopathy of prematurity. However. pica cannot be stopped in toddlers who are most susceptible. Differential count may show eosinophilia. II. Management 1. ocular USG. other forms of endophthalmitis. (B) Surgical method. There role has been questioned. There are no sure way to prevention except personal hygiene and non consumption of uncontaminated food. Anti helminths : Though broad spectrum anti helminths are routinely prescribed in children suspected to be suffering from toxocariasis. However.266 Differential diagnosis PEDIATRIC OPHTHALMOLOGY Most commonly mistaken diagnosis is retinoblastoma which cannot always be differentiated clinically.

Roth’s spot. Children who receive blood transfusion regularly due to various blood disorders are also at high risk. It is mostly seen in males. AIDS related opportunistic organism and malignancies. It involves lids more often than conjunctiva. 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. retinal vasculitis.DISORDERS OF THE UVEA IN CHILDREN 267 Acquired immuno deficiency syndrome and uveitis Acquired immuno deficiency syndrome is an infectious disease with world wide distribution. Diagnosis A person is said to have AIDS if following criteria are present. expectancy is less than two years. adeno virus. which may result into blindness. It has only RNA in its structure. it is seen in 35% of with HIV infection. 50% to 75% of AIDS patients will ultimately develop ocular lesion. it uses patient’s cellular reproductive system to manufacture DNA for multiplication. fungi. both sexes are affected. The HIV virus is a retrovirus known as human immuno deficiency virus type I. microaneurysm. Other ocular manifestations may involve any or many parts of the eye from simple follicular conjunctivitis to severe retinal necrosis leading to bilateral blindness.24 3. The disease is bilateral due to haemotogenous spread. The tumor is of endothelial origin 54. papillitis and ischemic maculopathy. Epstein Barr virus.53 . AIDS itself or 2. herpes zoster. cytomelagic virus. AIDS by itself can clog this micro vasculature by deposition of immune complexes or 4. Commonest type of ocular infection is CMV retinitis that cause multiple cottonwool spots. Once Kaposi sarcoma develops life. about 90 percent will develop AIDS which is uniformally fatal over the years. Though this is malignancy of adults it occurs in teens in patients with AIDS as well. These opportunistic organism can be bacteria—mycobacteria. It is considered to be an indicator that the patient has reached the last stage of primary disease55. herpes simplex. There may be many fold increase in virulence of infective organism in the body. The lesions are multicentric.53 Fifty percent of HIV positive persons are transient non infectious. Some of the microorganisms have greater predilection for AIDS patients. No age is immune. Kaposi sarcoma is commonest form of malignancy met in patients with AIDS. retinal haemorrhage. There is a gap of few years when an HIV infected person is converted to AIDS.53 It is seen in patients who have CD4 count less than 50 cells/mm3. Number of children affected by AIDS is increasing. 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. The HIV virus targets the CD4 lymphocytes and destroy it. However. Extremities are involved earlier than periocular structures. They include keratitis. AIDS related complex. The uveal lesion may be a nongranulomatous pan uveitis due to HIV or due to herpes simplex or herpes zoster. toxoplasma and pneumocystis. Ocular manifestation may be either due to 1. acute retinal necrosis or retinal detachment. orbit is involved rarely.53 CMV retinitis is cell CD4 dependent. In older children HIV can be acquired due to use of contaminated needle especially in drug users.

It is more frequent is girls. knee happens to be most commonly involved. Management AIDS is uniformly fatal in spite of treatment. lymphadenopathy. Antinuclear antibodies is positive in 75% of cases while 95% of patients have positive HLA B-27. 1. Unequivocal evidence of HIV I infection confirmed by ELISA and Western blot test. 4. hepato spleenomegaly and maculo papular rash. 2. Evidence of systemic infection by at least one opportunistic organism. Posterior segment treatment consists of systemic anti HIV drugs and surgical treatment for retinal detachment with uniformly poor prognosis. 29. arthritis with bowel disease. CD4 cells count less than 200 cells/mm3. There may be pericarditis. This type affects four or more than five joints during first to three months. Knee joint is mostly involved followed by wrist and ankle. unilateral that may be recurrent in the same eye or may alternate between the two eyes. Arthritis may last for many years resulting in permanent joint deformity. 3. Ratio of girls to boys is roughly 3:2. 42. Predominantly systemic involvement with minimum uveal involvement. Malignancy do not require separate treatment however concurrent anti cancer drugs may be required. 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. Stills disease. The condition is generally symmetrical. antinuclear antibodies and negative for rheumatoid factor. juvenile spondylitis. 2. It manifests with intermittent fever. Juvenile rheumatoid arthritis24. Number of joints involved in first three months is four or less. management is by multiple HIV drugs given systemically.About 5% of children with JRA come in this group. Some of the characteristic features of arthritic uveitis are—They are generally acute. Pauci articular involvement with more uveal involvement . involvement is asymmetric. According to clinical features in first three months the disease has been divided into three sub groups. Presence of Kaposi sarcoma. 43 It is the commonest form of arthritis seen in children between 2 to 16 years of age. juvenile Reiters syndrome. Ocular treatment for anterior uveitis is by local steroids and cycloplegic. posterior synechia. Polyarticular involvement with moderate uveal involvement. Once the disease sets-in. peak being 2 to 4 years.268 PEDIATRIC OPHTHALMOLOGY 1. . It is a blinding disease in 60% of patients but it is fully preventable disease. Ocular management is paliative. 3. Common joint disorders associated with uveitis in children are : Juvenile rheumatoid arthritis (JRA). it is sero negative. Hypopyon is common.

positive ANA and HLA B-27 but negative serology for rheumatoid factor. Children should not be put on atropine for long . 1. Child should undergo assessment of errors of refraction and examination of fundus and detailed slit lamp biomicroscopy. Management Management depends upon severity and duration of disease. Arthritis usually precedes ocular involvement by several years. are more prone to develop complications. Uveitis in juvenile rheumatoid arthritis Uveitis in ninety percent of cases is mostly anterior uveitis. Moderately severe lasting more than for four months with acute exacerbation. Moderate attack lasting more than 4 months with +2 to +4 aqueous cells. The pausi articular involvement as idiopathic anterior uveitis in adolescent. presence of complications are managed as and when they arises. Mild with +1 cells and flares. Diagnosis is not difficult with history of joint involvement in child with non granulomatous uveitis. cataract secondary glaucoma and macular edema. respond poorly to treatment. occasionally reverse may be true. The onset is so insidious without any discomfort that it is called silent uveitis. 4. There are two groups of patients who have uveitis. 2. 3. hence the child should be under surveillance for at least 6 to 7 years following development of arthritis. The condition may be diagnosed not due to redness. Examination should include recording of acuity of vision. 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. In initial stages the eye should be atropinised to break the posterior synechia. measurement of intraocular tension both for glaucoma and hypotony.DISORDERS OF THE UVEA IN CHILDREN 269 Twenty percent of children develop uveitis. It is non granulomatous in nature. Mild to moderate cases can be controlled by cycloplegic and local steroid drops. Differential diagnosis consists of idiopathic iritis. It is bilateral in 70%-80% of cases and recurrent. Silent white uveitis seen mostly in girls between 4 to 16 years of age. infective iritis and sarcoid in children. Severity of the disease can be 1. Somehow hypotony is also a common feature. The complications are potential threat to vision. pan uveal involvement is extremely rare so is involvement of vitreous and choroids. 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. Severe—attack lasts for years in spite of treatment. The ocular manifests may continue despite treatment and apparent cure of joint involvement leading to frequent complications of band keratopathy. 2. Children with iridocyclitis in pausi articular disease should be examined every three months and cases of iris with positive ANA more frequently.

band keratopathy. Cataract is managed by surgery for which a quiet eye is a prerequisite. They are also better avoided under ten years of age. Aphakia may be managed either by contact lens. Complications are frequent both due to disease itself and sometimes due to prolonged use of steroids. steroid induced or both. Surgery should be followed by use of steroid in sufficient dose and frequency for long time. If at all it has to be given it should be prescribed in consultation and under supervision of pediatrician. secondary glaucoma. Dose of immuno suppressive drugs for uveitis is less than that for malignancy. Next line of medicines are steroids in the form of drops or periocular injection. may be avoided as far as possible. So far no ocular side effect has been reported following local administration of NSAID. A constant watch on side effects especially haemotological must be kept. amblyopia and hyptony. In smaller children contact lens fitting is really difficult though not impossible. Strength of steroid should be minimum with maximum benefit. Non steroidal anti-inflammatory drops are generally prescribed as maintenance therapy for long time. The eye should be white without flare for at least three month before surgery. Systemic non steroidal anti inflammatory drugs are better avoided in children. failure is common. Drops may be required hourly for first few days in acute stages. Thick posterior capsular membrane formation is very common that may require repeated manipulation. A school going child under atropine require bifocal for near work. All surgeries should be done under general anaesthesia with endo tracheal intubation. Miotics and prostaglandin analogs are contra indicated. Ocular side effects of local steroids should be weighed against its advantages and its dose adjusted accordingly. Role of intra ocular implant is unsatisfactory.270 PEDIATRIC OPHTHALMOLOGY times as it may induce atropine induced amblyopia. Secondary post inflammatory glaucoma respond poorly to anti glaucoma drugs. Older children are given steroid as in adults. A child can not be put on oral carbonic anhydrase inhibitor for a long time due to systemic side effect. Chronic cases not responding to local steroids or intolerant to systemic steroids are put on systemic immune suppressives drugs in consulation with oncologist. Periocular steroids are given in children in teens. Glaucoma Possibility of glaucoma should always be kept in mind. For mydriasis induced glare dark glasses are prescribed. otherwise instillation four times a day is sufficient. Surgery also does not give satisfactory results. with spectacle reading correction or bifocal. Once the inflammation has been reduced. it can be due to inflammatory process and its aftermath. a short acting cycloplegic like tropicamide at bed time gives sufficient accommodation during day to go to school. Common complications are cataract. younger children do not cooperate for periocular injection. . Band keratopathy is managed initially by EDTA otherwise lamellar keratoplasty or excimer photo therapeutic keratectomy may be tried. An unnecessary delay in cataract surgery may lead to amblyopia. Systemic steroids should be avoided under ten years of age due to its systemic side effect.

Management is with cycloplegic local and periocular steroids. Dysentery is treated by combination of trimethoprim and sulpha methoxazol or ciprofloxacin. non granulomatous. non granulomatous. In children the disease is mostly seen in peripheral joints of lower limb. Uveitis may be proceeded. In more severe cases there may be frank hypopyon. recurring. However it is associated more commonly with anterior uveitis. . parts of it may be infective. anisometropia following unilateral lens extraction. may be simultaneous or may follow diagnosis of spondylitis. Treatment comprises of local treatment of mucopurulent conjunctivitis by frequent antibiotic drops. Ocular manifestations are mainly anterior uveitis that is acute. fine dusty or small KPs cells and flares are generally in the range of 3+ and 4+. Urethritis is non gonococcal and treated with erythromycin. unilateral. Diagnosis Unilateral acute iridocyclitis in second decade in a boy with pain in peripheral joint.57C Since than it is being referred as such. uveitis is generally unilateral. Reiters syndrome is a rare disease of unknown origin. undetected glaucoma. Conjunectivitis when present is generally bilateral and mucopurulent. Uveitis is treated with cycloplegic and steroids. About 25% children suffering from ankylosing spondylitis develops anterior uveitis. CME and band keratopalthy are less common than in juvenile rheumatoid arthritis. Pars planitis or intermediate uveitis57A. Arthritis is poly articular and asymmetrical. conjunctivitis and arthritis. The disease has positive HLA B 27 in 80 to 90 percent patients. Cataract. macular edema.57B Pars planitis is a common chronic bilateral uveitis that is either not diagnosed till late or misdiagnosed. It is sero negative for rheumatoid factor. Recurrence may vary between every month to one year. posterior synechia results in iris bombe and secondary glaucoma. An attack of anterior uveitis generally lasts for 4 to 6 weeks. keratitis than conjunctivitis. 50% uveitis with positive HLA B 27 suffer from ankylosing spondylitis. hence it has been called by various names since 1908. About 80% children with uveitis will develop bilateral disease that is rarely simultaneous. Failure to use contact lens and unsatisfactory I0L transplant.56 It is a chronic inflammatory disease of unknown origin that involves mostly the axial spine in adults. (urethritis and dysentery) it was originally described as triad of urethritis.DISORDERS OF THE UVEA IN CHILDREN 271 Amblyopia is a tumbling block that is produced due to prolonged use of atropine.45. When Fuch’s called it cyclitis. positive HLA-B 27 should undergo X-ray of sacro iliac joint for possibility of sacroilitis. It is more common in adolescent boys than girls. The Internaational uveitis study group has finally labelled it as intermediate uveitis in 1987. Its exact etiology is not known. Uveitis in ankylosing spondylitis42. It has variable clinical presentation. Signs Consists of circum corneal congestion. opacities in media.

Most important finding on examination of peripheral retina are snowball opacities and snow banking mostly in the inferior quadrant. The course is variable.idiopathic iridocylitis. 61 Sympathetic ophthalmia is a bilateral potential blinding disorder. Both boys and girls are affected equally. 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. It is said to be rare disease in advanced countries and urban areas of developing countries but it’s incidence in under developed countries must be more than reported. To begin with it is almost symptomless. Local and peripbulbar steroids are required if vision falls below 6/18. There are reports that it may run in families. It is a disease of young. the earliest symptoms that may attract child’s attention is floaters in the affected eye or mild diminished vision. presence of snowball makes it imperative to exclude sarcoid by X-ray chest. Some children may have the disease for years with or without loss of vision. Fundus fluorescein angiography is the diagnostic of CMC and periphlebitis. The anterior vitreous shows flares. However some patients may have rise of intraocular pressure due to spilling over of the pathology in the angle. cells and strands. Immuno suppressive drugs may be required.Posterior subcapsular cataract and cystoid macular edema. Hypotony is common.57D Sometimes there may be few KPs and peripheral corneal edema. Cystoid macular edema is a late feature and major cause of diminished vision.272 PEDIATRIC OPHTHALMOLOGY Pars planitis is a disease of pars plana of ciliary body adjoining choroid. Sympathetic ophthalmia57. 60. There is no HLA phenotypes. peripheral retina mostly peripheral retinal vessels. Children not improving with local peribulbar steroids should be put on systemic steroid for long time with usual precaution. gallium scan and raised serum calcium. Careful examination of retinal periphery. adults and children. Floaters are generally not taken very seriously by the parents or treating general physician. On slit lamp examination there are cells and flares is aqueous. The vision may be within normal limits initially. There may be new vessel formation near the snow bank that may be source of haemorrhage58. others may have choroidal effusion and serous retinal detachment. Differential diagnosis consists of . Examination of peripheral retina with indirect opthalmoscope and scleral indentation shows evidence of peri artrititis and periphlebitis. Management Almost eighty percent of children do not require any treatment. Common cause of diminished vision are. Absence of joint systems rule out juvenile rheumatoid arthritis associated with uveitis. Peripheral granuloma of toxocara is excluded by negative serology test. Rarely surgical intervention like lensectomy or vitrectomy is required. In large series it has been reported to be as common as 10 to 15 percent of all uveitis cases observed. By the time the next symptoms of diminished vision occurs the disease has progressed far. It is a bilateral chronic pan uveitis . Cycloplegic do not have any added advantage.

The chorio capillaries are spared. any glaucoma operation. The anterior chamber may show 2+ to 3+ cells and flare. The eye that is injured is called exciting eye and the eye that develops chronic pan uveitis is called sympathizing eye. Clinical features Clinical features are variable. number of boys developing sympathetic uveitis is more than girls. retinal detachment surgeries with drainage of sub retinal fluid. all eyes with penetrating injury do not develop sympathetic uveitis. rupture of the globe anywhere with incarceration of any intra ocular structure can cause sympathetic ophthalmia. The cornea has various shapes of KPs. The disease generally develops in iris but may start anywhere in the uvea and it is bound to become panuveal. The iris is greatly thickened. There may be both Koeppe and . It is most commonly seen following penetrating injury within five mm. There is thickening of all the parts of uvea. However. 66. The immune reaction results in chronic granulomatous lesion that forms a giant cell similar to tuberculosis without caseation. About 60% of the cases are seen following accidental penetrating wound. First sign of sympathetic uveitis is flare the retrolental space. however. age or sex is immune. Generally they are large mutton flat in nature but may be fine dusty. Some may develop it within few days. It spans between 5 days to 66 years. Etiology of the condition is not well understood. They are common in the lower mid periphery. The eye at this stage may not be congested. The pupil is miotic and irregular due to strong posterior synechia. there is sufficient evidence to consider auto immunity as prime factor. Clinical presentation depends upon inflammatory process which in turn is perhaps genetically predisposed. 61. Common surgical procedures are—lens extraction. However. About 30% are seen following surgical manipulation in which the globe is penetrated. lacrimation and blurring of vision. others may not be effected for decades after initial injury. The injured eye has evidence of penetrating injury and its complication that may mask the presence of indolent plastic iridocyclitis.DISORDERS OF THE UVEA IN CHILDREN 273 with acute exacerbation and recurrences. loss of accommodation. iris inclusion surgeries for glaucoma were notorious to produce sympathetic ophthalmia. specially the choroid. Anti retinal antibodies have been found in some cases24. This is perhaps due to the fact that boys are more prone to injury than girls. they are not diagnosed of sympathetic uveitis. No race. it has been reported following perforation of corneal ulcer. Children are affected more than adults. Interval between injury in exciting eye and development of sympathetic uveitis varies. Even laser photo coagulation have been reported to cause sympathetic ophthalmia. 70% of cases develop within three months and 90% within first year. however. it has been reported following blunt injury. of limbus with incarceration of uvea. The reaction is delayed type-four hypersensitivity to uveal antigens located in retinal pigment epithelium and melanocytes. The early symptoms of uveitis in the sympathizing eye are heralded by photophobia. However. Small yellowish white spots called Delen Fuch’s nodule are seen scattered in the choroid. It is said that sympathetic ophlhalmia does not develop if the injured eye has developed suppuration.

The other eye is examined regularly. Fluorescein angiography may initially show areas of hypo fluorescence due to masking of choroid by Delen Fuch’s nodule followed by hyper fluorescence due to the leak of the dye. . they may be vitiligo. Onset of inflammation in sympathetising eye generally worsens. Diagnosis is mostly clinical based on 1. Surgical treatment is restricted to injured eye that consists of management of (1) Injury and (2) Enucleation of unmanageable injury. 4. The uvea should be dis-engaged from the wound and excised by standard procedure. 3. 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. retinal vasculitis and serous retinal detachment. retinal detachment. Extra ocular manifestation are rare. the eyes are generally soft. under microscope with use of viscoelastic substance. Ultrasonography shows thickening of choroids. Management Correct and early diagnosis helps to restore vision better and prevent permanent loss of vision. Sometime the injured lens may cause lens related uveitis.274 PEDIATRIC OPHTHALMOLOGY Busacca’s nodule present. The lens matter should be removed and if needed anterior vitrectomy done. Management of sympathetic ophthalmia can be divided in the two groups (1) Surgical and (2) Medical. Sometimes the exciting eye may have better vision than the sympathetising eye. This does not involve the other eye and can be managed-well by removal of lens material. band keratopathy. Sometimes the exciting eye may have better vision than sympathizing eye. the signs and symptoms in the exciting eye. if necessary. a sub conjunctival injection of broad spectrum antibiotic with steroid is given along with atropine. alopecia and polycytosis of CSF. The eye is kept under observation for evidence of uveitis. poliosis. that has either not been repaired or repaired without freeing of uvea from the wound. Role of enucleation after onset of sympathetic opthalmia is doubtful. papillitis. The posterior segment involvement results in the posterior vitreous cells. All penetrating injuries in children should be repaired under general anaesthesia. phthises and blindness. evisceration does not serve this purpose hence it is not recommended. 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. secondary glaucoma. The purpose of enucleation is to remove the uvea in toto. Clinical evidence of anterior uveitis or choroiditis. The anterior segment should be reconstructed as far as possible. 2. History of trauma. Common complicationa in untreated cases are—Complicated cataract. Enucleation If the injured eye is not salvaged it is better to enucleate it within the first ten days of injury.

Azothioprine 1 to 2 mg/kg day oral 3. Cortico steroids are the main stay of management of sympathetic ophthalmitis. Initially steroid may be required as frequently as hourly which is generally tapered to two times a day. Methotrexate 5 to 30 mg/week 4. Immunosuppressive drugs. It may be started with divided dose and switched over to alternate day dosage and gradually reduced by 5 mg. poliosis and alopecia. Immunosuppressive drugs are prescribed when steroids are ineffective or producing local and systemic side effects. 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. They are 1.DISORDERS OF THE UVEA IN CHILDREN 275 Medical treatment is usual treatment of non infective granulomatous uveitis that can be local and systemic. Commonly used immunosuppressive drugs are : 1. Long acting steroids and 2. It can be given in divided doses. vomiting. a week and later maintained either on 5 to 10 mg. There is no exciting eye or sympathizing eye in Vogt Koyangi Harada disease. Most commonly used steroid is prednisolone in a dose of 1 to 1. neck stiffness and periorbital pain. 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. Cyclophosphamide 1 to 2 mg/kg/day Immunosuppressive drugs can be given alone or in combination with steroid. vertigo. Local treatment consists of judicious use of atropine and local steroid drops. 64. It is rarer than sympathetic ophthalmia in children. 63. If steroid drops do not give relief within 48 hours peribulbar injection of steroid is advocated. Both the conditions produce chronic panuveitis.5 mg/kg/day. The syndrome has (1) Prodromal stage that may be mild and consisting of headache. . Vogt Koyanagi Harada Syndrome24. a day or 10 to 20 mg. 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. Cyclosporin A 5 mg/kg/day oral 2. They should always be prescribed in consultation with oncologist. alternate day for months with usual precaution. which are : (i) Skin changes in the form of vitiligo. (2) Systemic features generally precede ocular features. daily single dose or alternate day.

It is a predominantly uniocular disease. myopathy. on slit lamp examination small non pigmented . Management is systemic steroids. cranial neuropathy. The eye is generally white. strong and broad posterior synechia. local. Sarcoid uveitis It is a disease of adults but may be seen in children as well. On examination. 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. Complications consists of complicated cataract. secondary glaucoma band keratopathy. 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. vitreous haemorrahage. The auditory symptoms lasts only few months. (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. Only 10% cases are bilateral. meningism. skin. anterior chamber reaction. (iii) Auditory features are tinitus. Management is similar to any chronic non-infective granulomatous uveitis. uvea etc. neck stiffness. There may be papilitis. The iris with light colour is the affected eye. Uveitis in sarcoidosis can be acute or chronic both granulomatous and non-granulomatous lesion are possible.276 PEDIATRIC OPHTHALMOLOGY (ii) Neurological signs and symptoms are headache. Fuch’s heterochronic iriodcyclitis It is commonly presumed that Fuch’s heterochromic iridocyclitis is a disease confined to adults only but it is known to be present in all ages. photophobia. Diagnosis is confirmed by histopathological examination of skin or conjunctival nodule. edema of iris. Sarcoid is a systemic disorder of unknown cause of most probably T-cells mediated immunity that involve almost all organs of the body. lacrimation. It is generally asymptomatic. cycloplegic. CSF lymphocytosis. Attention may be drawn to uniocular light colour of iris. redness. encephalopathy. All parts of the eye may be affected. in all ethnic groups and both sexes. phthises. Area of retinal edema and detachment show hypo fluorescence. dull pain. there is ciliary flush. daily single dose or alternate day single dose. Involvement of macula results in metamorphopsia. diminished near vision. vertigo and deafness. pars planitis with typical snow bank appearance and candle wax lesion in retina. (5) Immunosuppressive agents are effective in recurrence and helps reduce dose of steroids. Common posterior segment involvement can be vitritis. Areas of choroidal ischeamia are better shown by indocyanine green. mild mutton fat KPs. (3) Uveitis phase : This can be divided into two groups : (i) Anterior segment involvement or Vogt Koyanagi form . local and periocular steroids. Both anterior and posterior segments may be involved. the iris nodule and atrophy of iris stroma.This consists mostly of granulomatous uveitis that begins with blurring of vision.e. Fluorescein angiography shows multiple area of hypo fluorescence.

some times immuno suppressive may be required. Lens induced uveitis So long as the lens is inside intact capsules it does not produced any reaction either allergy or chemical. Complete removal cortical material in the first eye. Exact etiology of the disease is not known. The condition may produce acute or chronic anterior uveitis and occasional glaucoma. joint pain. if the lens ruptures accidentally or during surgery even years after the original incident. It presents as hazy cornea due to edema and folds in Descemet’s membrane. retinal exudates and haemorrhage. Prolong systemic steroids results in poor visual outcome. Anterior segment ischeamia and uveitis Anterior segment ischemia is mostly an iatrogenic disease following strabismus and retinal surgery. Treatment consists of local cycloplegic. Most of the cases do not require any treatment unless cataract develops. the second eyes get effected in similar ways i. This may be confused with sympathetic uveitis. There may be cystoid macular edema. skin lesions. If not attended to it may lead to iris atrophy causing distorted pupil and complicated cataract. There are two types of ocular involvement. Treatment is unsatisfactory. If lens matter is left. Cycloplegies are not required. it may result into second type of lens induced uveitis i. photophobia. 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. Some cases may require local steroids. there may be vitreous cells. steroid. Flourescein angiography of iris shows well demarcated areas of non . 2. it can occur in children. 66. 1. In children hyper mature lens may result either in traumatic cataract following blunt injury or in unattended congenital cataract. 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. Leak of lens material causes two types of reaction. It’s share as causative factor of uveitis in children is very less. Mostly posterior segment involvement with systemic involvement that includes recurrent aphthous ulcer of mouth and genitals. cataract and glaucoma. is more common among Japanese. beta blockers. endophthalmitis phacoanaphylactica.e.68 The disorder starts within 24 hours of following surgery with pain. Mostly anterior segment involvement. (II) Type two hyper lens induced hyper sensitivity. There may be sudden development of hyppyon in iridocyclitis. Local treatment consists of cycloplegic and steroids.e. There may be iris nodules but no posterior synechia are seen. HLAB-51 is closely associated with it. keratic precipitates. 67 The disease is seen world over as rare condition. Behcets disease42.DISORDERS OF THE UVEA IN CHILDREN 277 keratic precipitates are seen. Uveitis subsides with complete removal of lens matter. This is more common in adults and incidence rises with age. Anterior segment ischeamia may also develop in sickle cell haemoglobinopathy. however. It has a protracted course with good prognosis. (I) A chemical reaction due to leak of lens protein through non ruptured lens capsule in hyper mature cataract. lacrimation. aqueous flare and cells. systemic steroids are given only in cystoid macular edema for short period. After uveitis subsides in the first eye.

Bacteria or fungi reach the eye from a primary site due to hematogenous spread.e perforated corneal ulcer.69 Management consists of intensive local steroids and use of cycloplegic. vitrectomy. sex or race is immune. 3. paracentesis. Virulence of the causative organism. physical or chemical reaction. . In all the cases. mechanical capsulotomy. It is defined as inflammation of intraocular tissues due to infection by bacteria.e. 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. The inflammatory process from choroid spreads to ciliary body and iris resulting in purulent pan uveitis. fluid gas exchange. in case of micro organism or setting an immune reaction or release of chemicals. Clinical presentation depends upon—1. lung. Surgical Commonest surgery that results in endophthalmitis is lens extraction of any type with or without IOL. Clinically there are two main types of endophathalmitis. vitrectomy. periocular or peribulbar injection. Treatment received. (2) Surgical.70 It is generally acute that can become chronic or may have chronic onset. endogenous endophthalmitis is more common in children in developing countries due to prevailing malnutrition. Viruses are not known to cause endophthalmitis. this happens following inadvertent perforation of globe during squint and retinal surgery. vasculitis or neoplasm can also cause it. fungi and parasite (toxocara).278 PEDIATRIC OPHTHALMOLOGY perfusion. The trauma can be (1) accidental with or without retained foreign body. trabeculectomy. Immune reaction. Endogenous endophthalmitis in children Endogenous endophthalmitis in children is due to spread of micro organism from primary site that can be abdomen. Rarely extra ocular surgeries may produce endophthalmitis. Exogenous endophthalmitis is caused due to direct entry of bacteria or fungi inside the eye following trauma or spread from neighbouring structure i. iris inclusion procedures. 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. however. iridectomy. Chemical composition of the offending causative factor. infected teeth. penetrating keratoplasty. middle ear. Ultimately whole of the vitreous is involved in abscess formation. the condition can be avoided. Endophthalmitis Endophthalmitis is one of the most dreaded catastrophes in ophthalmology. Endogeneous (metastatic) end opthalmitis—This is caused by micro organism. Other surgeries include all intraocular surgeries i. The second path is commonly seen in exogenous endophthalmitis. 1. para nasal sinus. xerophthalmia and systemic pyogenic disease and unattended or poorly managed trauma. The basic pathology is lodgement of the offfending organism and multiplication of it. It is presumed that if only two muscles are disinserted at a time. retained intraocular foreign body. It is generally unilateral but can be bilateral in endogenous type. While the first route is generally seen in endogenous endophthalmitis. 2. bones and joints. 2. The lens is not capable of inflammation hence it is spared. 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. No age.

Presenting features of bacterial endophthalmitis differ from fungal in many ways. Bacterial 2. For the same reason vitrectomy is less effective in endogenous endophthalmitis. The symtoms are more indolent as compared to Aspergillus which is more severe. under radiation therapy or have immuno compromised status. Endogenous endophthalmitis is more common in children who are malnourished. 3. Systemic steroids help in controlling inflammation in bacterial endophthalmitis but are contra indicated in fungal endophthalmitis. Pure copper produces more severe endophthalmitis than copper alloys. sometimes inert foreign bodies can also cause sterile endophthalmitis. have been on systemic antibiotic or steroids for long time. Exact percentage of post operative endophthalmitis is just a guess. Causative organisms can either be bacteria or fungus or parasite. painful and associated with hypopyon. All of them can follow surgery or be accidental. have under gone extensive abdominal surgery. 2. about 16% of all white reflexes in pupillary area are caused by toxocara. Candida is more frequent than aspergillus. Endogenous endophthalmitis is more effectively treated by parental antibiotic. Parasitic endophthalmitis is far more common than expected. Fungi are considered to be more common causative organism than bacteria. Bacterial exogenous endophthalmitis is commonest form of endophthalmitis. Fungal 3. which is less common in children whose accidental wounds with or without retained foreign bodies cause more inflammatory reaction. Undiagnosed nasolacrimal duct obstruction. 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. In adult common type of exogenous endophthalmitis is post surgical. It has been reported to be in the range of 0. Pure copper causes severest form of endophthalmitis. Thus exogenous endophthalmitis can be 1. However. retinal haemorrhage. As the lesion starts and remains under the internal limiting membrane. visco-elastic. .DISORDERS OF THE UVEA IN CHILDREN 279 heart. lacrimal sac etc. Exogenous endophthalmitis Exogenous endophthalmitis is three times more common than endogenous endophthalmitis. As the infection is hematogenous it starts in posterior uvea. Saprophytes from the lid and conjunctiva that become opportunistic. Candidal infection starts in the choroids as multifocal round lesions that spread to cause perivasculitis. Break down of asepsis and antisepsis protocol is the commonest cause of post surgical endophthalmitis. Contaminated instrument.71 Many fungi have been isolated as causative factor but common are Candida and Aspergillus. Local antibiotic drops are prescribed to combat secondary corneal and conjunctival infection while combination of local cycloplegic and steroids are given for anterior uveitis. may lead retinal necrosis. frequently it can be bilateral. chronically ill. However. Endogenous endophthalmitis generally has an indolent course that may be initially masked by more serious systemic condition. Sterile. in developing countries bacterial metastatic endophthalmitis is equally common if not more than fungal endophthalmitis. It is more common following secondary I0L than primary I0L.2%. Retained organic foreign bodies more inflammatory than inorganic. Organism get access into intraocular structure via—1. skin. The anterior uveal inflammation lags behind in contrast to exogenous endophthalmitis where anterior segment inflammation is more pronounced than posterior.05% to 0. In penetrating keratoplasty it is perhaps the micro organism in donor cornea that is responsible for the infection.72 Post surgical endophthalmitis is more common than accidental. irritating fluid.

The symptoms consist of chronic reduced vision. Thus masking the exact pathology resulting into delay in treatment. Propioni bacterium acne. the conjunctival congestion is prominent. On examination. streptococci and staphylococcus aureus. At this stage ultra sonography may clinch the diagnosis. Late onset post surgical endophthalmitis This is generally due to otherwise non virulent commonsels. Exudative membrane over the I0L or vitreous face. 4. Delayed between seven to tens days mostly caused by staphylococcus epidermis and coagulase negative cocci. their may be chemosis of conjunctiva.71 Gram negative organism are more virulent. Marked reduction of vision and loss of fundal glow are two ominous signs. On slit lamp examination there are large KPs with 2+ to 3+ flare and cells. All types of bacteria both gram positive or negative are capable of causing post operative exogenous endophthalmitis. always bacterial. Symptoms and signs of acute post surgical endophthalmitis are—Pain more than usually expected pains of surgery.Acute within first forty eight hours following surgery. gram positive strains are responsible for 90 to 95% of endophthalmitis. caused by gram negative bacteria and gram positive cocci i. small pupil if cycloplegics have not been used. In children streptococci are more common causative organism than staphylococci which is a major cause of endophthalmitis in adults. On examination all signs of persistent uveitis are present that are initially responsive to steroids.280 PEDIATRIC OPHTHALMOLOGY There are three forms of endophthalmitis according to the onset of the disease 1. However. 2. spreads fast and produce endotoxin. The fundal glow is either very faint or absent on retinoscopy and direct ophthalmoscopy. commonest organisms being various fungi. Loss of ocular movement and onset of proptosis denotes panophthalmitis. Remote infected bleb. edema of iris. 3. Lower angle of AC should be carefully scrutinized for small hypopyon that changes its level and position with the eyeball. An indirect ophthamoscope may circumviate the exudates in the vitreous and allow visualization of the fundus behind.e. retrolenticular flare and cells in phakic eye. hazy cornea. there are signs of recurring pan uveitis with loss of fundal glow or a white reflex in pupillary . Intense flare and cells may have fibrinous aqueous or frank hypopyon. The eye remains congested with mild to moderate pain and vision that was good is lost in days. drop of vision below anticipated level. Fundus when visible may show retinal hemorrhages and exudates. Delayed post operative endophthalmitis undergoes same pathological process as acute but in a milder form. which is an anaerobic gram positive commensal is becoming more frequent cause of chronic post surgical endophthalmitis. acne and staphylococcus epidermis. photophobia. lacrimation. the lids are swollen. aqueous may have fibrous strands. Late—after four to six weeks. There is generally exudates in the pupillary area. prolapsed uvea. P. There may be exudates behind the posterior capsule. Generally there is formation of vitreous abscess. On examination. Non reacting. 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.

Post traumatic endophthalmitis About 5% of eyes sustaining accidental penetrating injury develop endophthalmitis. Vitreous may show snowball and fluffy opacities that may mistaken as cortical matter. The inflammation is caused due to chemical insult of irrigating agents. latches of the door. They are more common in rural set up and in boys. most of these eyes have generally received usual high doses of steroids. as it has relatively less symptoms than bacterial endophthalmitis. Most of the time they are uniocular. immune status of the eye and treatment received. Late onset of endophthalmitis is generally seen in fungal infection.71 Fulminating endophthalmitis develops in short period when infected by B Cereus the organism that has never been isolated in post surgical endophthalmitis. twigs and leaves. Post operative fungal infection is more often missed than diagnosed. Generally there is a fixed thick hypopyon and rise of intraocular pressure. non responding cases may require vitrectomy. The presentation is similar to post surgical endophthalmitis. The child invariably does not complain of lowering of vision. Diagnosis of post traumatic traumatic endophthalmitis is not difficult. lens matter. Too much manipulation of anterior uvea. In fact any relatively sharp object cause penetrating injury. Inadvertent intraocular injection of xylocain during peribulbar injection and antibodies / steroid during periocular injection talc from the surgical gloves. Onset may be as short as 1-2 days or as long as 2 to 3 months depending upon the virulence of the organism. retinal detachment. The condition has to be kept in mind to be correctly diagnosed. Intraocular tesion is variable. Sterile post operative endophthalmitis24. . vitreous haemorrhage. protruding nails from walls. even the eye may be relatively white.DISORDERS OF THE UVEA IN CHILDREN 281 area. 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. vitreous disturbance. residue from chemicals sterilizers. cotton swabs. Ultrasonography at this stage helps to clinch the diagnosis and differentiate from other condition that may mimic endophthalmitis. It takes several weeks to months for fungal endophthalmitis to develop. The exact features may be masked.Bow and arrow. Common causes of perforating injuries in children are . monomers on the I0L. less frequent than infective endophthalmitis.71 is mostly iatrogenic in nature. sling shot (gulel). The overall profile of the organism isolated from post traumatic endophthalmitis are similar to post surgical endophthalmitis. The eyes are generally soft. suture material are some of the frequent causes of sterile post operative endophthalmitis. they may have retained intraocular foreign body. Ophthalmic ultrasonography gives information like position of lens. The difference being disorganization of the globe due to involvement of multiple ocular structures. Sterile endophthalmitis have an acute or delayed onset and respond fairly well to medical treatment. presence of foreign body. size of the inoculum. tip cat (gilli). Rise of tension results in unexplained pain that makes the child restless. Accidental trauma has been found to be responsible for about one third of all cases of exogenous endophthalmitis. dust contaminated fluid etc. X-ray orbit is required to exclude presence of radio opaque foreign body.

Systemic antifungal 5. Systemic steroid under umbrella of systemic antibiotic 4. Panophthalmitis This is acute purulent infection of all the structures of the eye including Tenon’s capsule. dislocated lens nucleus. retinal detachment. The condition is to be differentiated from orbital cellulitis which generally does not involve the globe and vision is retained. Systemic antibiotic 3. 2. The main role of antibiotics in panophthalmitis is to prevent septicemia. fever. headache. Vitrectomy II. Initially the cornea is steamy but later on develops purulent keratitis and sloughs. It is sometimes difficult to differentiate between severe endophthalmitis and early panophthalmitis. Once the endophthalmitis develops. Endogenous 1. Prophylactic use of broad spectrum antibodies is debatable. In post operative eyes pus may exude through the section. Treatment of panophthalmitis is one of the most frustrating things. It may take few days for panophthalmitis to develop following penetrating injury surgical or otherwise. The globe is immobile and proptosed. the lens may dislocate. The eye when untreated goes into phthisis after a prolonged course. it should be treated as any endophthalmitis. 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. unresolved haemorrhage. the conjunctiva is chemosed and hyperemic. Pus trickles through the lids. extraocular muscles and orbital tissues. Construction of anterior segment. Visual recovery is almost impossible. . The interpalpebral fissure is obliterated. Differential diagnosis of post surgical and post traumatic endophthalmitis consists of endophthalmitis phaco anaphylactica. all pus forming bacteria can cause pan opthalmitis. Broad spectrum antibiotic may suppress the infection. Sometimes the period may be few hours only and starts with malaise. ciliochoroidal detachment. Absence of perception of light is the rule. large fragments of cortical material. the anterior chamber is full of pus. Role of vitrectomy is not yet clear. The whole of the uvea is smothered with pus and the vitreous is almost a bag of pus. Supportive treatment by cycloplegic and steroid locally. Xerophthalmic children and eyes with lagophthalmos are more likely to develop panophthalmitis. meningitis and cavernous sinus thrombosis. Absence of perception of light and immobile globe is more in favour of panopthalmitis than endophthalmitis. Management of endophthalmitis I. On examination the lids are swollen red and hot. Details of this structure beyond the hypopyon is not visible. The organism reach the exterior of the globe following exogenous endophthalmititis which generally follow intraocular surgery or accidental penetrating wound. Panopthalmitis is generally bacterial in nature. It may also result following perforation of a sloughing corneal ulcer.282 PEDIATRIC OPHTHALMOLOGY Management consists of prompt repair of wounds as per standard method. pain in and around the eye.

Differential diagnosis consists of all causes of white reflex in pupillary area. Benign tumor of the iris are hemangioma. heterochromia and glaucoma. 74 New growths of uvea are rare in children. All cases of uveitis in children that improves with cycloplegic and steroid and recur should be suspected to be masquerade syndrome. Pseudo hypopyon may disappear and uveitis subside with local steroid and cycloplegic only to reappear. Serum and aqueous LDH may be raised. Other less common conditions are retinitis pigmemtosa. CT of orbit and MRI. However. photophobia which on examination shows a posteriorly placed growth or may come with white reflex in pupillary area. others may be non malignant growth or non inflammatory conditions. nodules on iris. may be associated with angioma of the lid and cause secondary angle closure glaucoma. KP flare cells in AC and accumulation of pus like fluid in AC (pseudo hypopyon) which is infact accumulation of tumor cells. when present they are generally benign or locally invasive. Fundus examination may show Roth’s spot. redness. X-ray chest and orbit. Management depends upon causative factor. The child is generally ill and pale. tension is invariably raised. . While juvenile xantho granuloma is a benign dermatological condition that causes spontaneous hyphaema in children. Aqueous tap may reveal tumor cells. but may be seen as early as first year of life. Some of them are malignant and potentially life threatening. New growth of uvea in children24. Examination of blood gives clue to presence of leukemia. cells in vitreous. 51.DISORDERS OF THE UVEA IN CHILDREN 283 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. DLC. TLC. malignant melanoma of iris is more common in children than adults. ESR. posterior synechia. Medulo epithilioma is a locally invasive tumor. They do not require any treatment. Intra ocular foreign bodies and peripheral retinal degeneration are two common non neoplatic condition that may present as uveitis. Neuro fibroma of iris is seen as nodule on the iris in association with generalized neurofibromatosis. All the above conditions may cause aqueous flare. cells in AC. They are seen mostly under 15 years of age in both the sexes. congestion pain. Such occurrences are extremely rare. malignant melanoma which is malignant growth of sixth decade can sometimes be seen in second decade. posterior synechia. X-rays shows intera ocular calcification ophthalmic ultrasonography may outline the tumor and intraocular calcification. Investigation includes : Routine Hb%. ultrasonography. Retinoblastoma is generally seen is children under five years of age with strabismus. Leukemia : Acute myeloid leukemia may present as anterior uveitis in children. 42. However. Spontaneous hypheama. may be malignant. Two common malignant disorders are retinoblastoma and myelogenous leukemia. multiple sclerosis and post vaccination status.

Presenting features depend upon site. unless they start growing in size or there is change in pigmentation. retina or optic nerve. Role of chemotherapy and radiation are not well established. secondary glaucoma and uveitis. The condition of self limiting. only occurring when the growth becomes extra ocular. However. There may be visceral involvement.284 PEDIATRIC OPHTHALMOLOGY Nevi are common tumors of the iris that make their presence felt just before puberty. which are common features of retinoblastoma as well. Both types can be benign or malignant. brain tissue etc. Medullo epithelioma may present as white reflex in pupillary area. however. They do not require any treatment. commonest age of presentation is between two to four years. Juvenile xanthogranuloma73 is a benign dermatological tumor seen at the age of one year. The former besides ciliary epithelium contains hyaline. pain and redness of the eye. One third of the medulla epitheliomas are malignant. They lesions may involve all parts of the eye. Metastasis is rare. Ocular involvement are—Iris nodules. It may only be detected accidentally when the child presents with diminished vision and squint. whitish reflex in the pupillary area.76 There are two types of medullo epithelioma . Eyes with large tumors are best enucleated. cartilage. Diktyoma75: This is a rare tumor. Treatment There is no definite treatment77 for small growth of iris and cilliary body be cured by wide iridectomy or irido cyclectomy. mostly arising from ciliary body but may arise from iris. Hyphema and uveitis are treated with local steroids. spontaneous hyphema. incidence of bilateral retinoblastoma is common presenting feature which is extremely rare in medullo epithelioma. unilateral growth without extra ocular involvement. All cases should be examined with indirect ophthalmoscope. On histology it shows rosettes similar to retinoblastoma. The term diktyoma is derived from its lace like appearance when it invades the iris. absence of intra ocular calcification should arouse suspicion of medullo epithelioma. buphthalmos and late proptosis. Ocular lesions are generally associated with cutaneous lesions. There is no known genetic predisposition or known family history. secondary glaucoma and proptosis. X-ray. The skin lesions are yellow multiple nodules. which may be sign of malignant change. Otherwise common presenting features include diminished vision. duration and location of the growth. rhabdomyocytes. It is a congenital growth. USG may help to differentiate medullo epithelioma from other disorders. CT. It is unilateral slowly progressive may remain confined to globe or may become locally invasive.The teratoid and the non teratoid. Diagnosis is difficult. . It is also known as medullo epithelioma because it was thought to arise from medullary epithelium of fore brain. MRI. The tumor is very often mistaken as retinoblastoma because retinoblastoma is commonest ocular growth in the same age group.

New Delhi.K. III. 2. 1963. Nancy Anderson Hamming. 52. Jogi Renu : Basic ophthalmology. 3. Banumathy S. Henry Kimpton London. 1963. p. 8. Duke Elder’s : System of ophthalmology Vol. 1. Duke Elders S. Vol. 10-11. Arvind Eye Hospital. 559. First edition. 1983.. WB Saunders Company. Duke Elder’s : System of ophthalmology Vol. 13. London. Maduri. : Disease of uveal tract in Modern ophthalmology.J. p. 10. Second edition. Asbury T. First Indian edition. p. p. Jay Pee Brothers. Vol. : Anatomy and embryology of eye in Principle and practice of ophthalmology. Vaughan D. Brothers. 14. 775. p. p. London. 17th edition. 1st edition. Jay Pee Brothers. p. Vol. p. p. 5.DISORDERS OF THE UVEA IN CHILDREN 285 REFERENCES 1. 2000. III part I. 2. : System of ophthalmology. 2nd edition. 4. 167-184. Miller S. 2000.. Ajeet Publication New Delhi. 573. and Roy F. First edition. New Delhi. . 1st edition p. 2nd edition p. 18. Duke Elder’s : System of ophthalmology. 17. 152-156. 16. 6. London. 37-39. 11. Walton D. Churchill Livingstone. : Parson’s disease of the eye. 1963 6. Jay Pee. 24. 1993. : Albinism in Modern ophthalmology. 1994. Biswas J. Sorsby A. Apple D. 148. 2. III. Ist edition.S. Khurana A.V. 1984. Part I. 1984.J. 7.V. New Delhi. : Anatomy of the eye. Vol. Nema H.H. III Part II. Second edition. Vol. Henry Kimpton London 1963. 19. 151. : Aniridia in Current ocular therapy. 5th edition. p. 9. Churchill Livingstone. 1st edition Henry Kimpton London 1963. Ahmed E : A text book of ophthalmology. III. Vol. pp 33-44. Oxford University Press. 1991. : Anatomy of the eye and its adnexa. Lange Medical Publication. pp. 508-509. : Parson’s disease of the eye. California. p. 1963. Henry Kimpton. New Age International (P) Limited. London. 2000. Henry Kimpton. 290. p. New Delhi. p-150. Third edition. 19 to 30.H.H. : Ophthalmology. Nema H. Deborah Pavan Langston : Pediatric ophthalmology in Manual of ocular diagnosis and therapy. p. 1987. Philadelphia. Edited by Fraunfelder F. Calcutta. 36-40 1st edition. 17th edition. Duke Elder’s : System of ophthalmology. 15. p. Jay Pee Brothers. Butterworth London.T. Miller S. 12. New Delhi 1991. : Anatomy of the eye and its adnexa. : General Opthalmology tenth edition. 158 to 162. P.

22. 25. Kimura S. Debora Pawan Langston : Uveal tract iris ciliary body and choroids in Manual of ocular diagnosis and therapy. first edition. 31. Jay Pee Brothers. 32. : Uveitis in Principle and practice of ophthalmology Vol. : Disease of the uveal tract in A text book of ophthalmology. Modern Publishers. 37. and Nolan S. New Delhi 2000. 1998. III part II p. : Disease of the uveal tract in Parson’s disease of the eyes. Churchil Livingstone London. 183-184. London. 156-175. edited by Sorsby A. 227. Modern Publication. Hogan M. First edition p. 893-902. 34. 571-580. Biswas J. Kanski J. J. : Signs and Symptoms of uveities I. Arch Oph 81: 264. : et al. 3rd edition. Williams and Willkino.W. 157. London. London 1964 29. p. Calcutta. Anterior uveitis AJO 47-155. 1. : Experimental band Keratopathy. Wood A. First edition. Vol. Edited by Peyman G A. Gittinger J.H. First edition. : Endogenous inflammation of the uveal tract. 155. p. 21. 1959. Edited by Dutta L C. p.286 PEDIATRIC OPHTHALMOLOGY 20. New Delhi. Kanski J. Nussenblat R. Duke Elders in Systems of ophthalmology. 36. 35. : Asdorian G K—Toxoplamosis in Manual of clinical problems in ophthalmology. 2.. Barua C. Carolene R Baumal : Granulomatous uveitis in ophthalmology secretes.B. Vol 4.. Standardisation of vitreal inflammatory activity in intermediate and posterior uveitis. Second edition. 1985. First Indian edition. Jay Pee Brothers New Delhi. 1984 24. p.C. Butterworth.A. p. Opthalmology. London. 147. 2nd edition. 92-467-471. 258. 2000. Oxford University Press.C. 1554-1566. and Thygeson P. p. Butterworth. Sharma P. Boston. edited by Dutta L C. : Uveitis in Modern Opthalmology. 23. p. p.H. Miller S. : Heriditory disorders of the retina and choroids in Clinical ophthalmology. second edition p.J. p. . 2000. 2. Jay Pee Brothers. : Essentials of ophthalmology. Doughman D. 1969. 1963. First Indian edition. : Intermediate uveitis (Pars Planitis) in. and Dutta L. : Uveitis in Clinical ophthalmology.K. 636-686. Vol. 33. Henry Kimpton. 2000. : Olson G. edited by Vander JF and Gault JA.S. 174-213. 1989. 385-386. Coles R. 27. New Delhi.J. p. 136-177. Butterworth. : Uvea and its diseases in Essentials of ophthalmology. Sharma P. 28. Seventeenth edition. Second edition. Ahmed E. Baltimore 1961 30. 26. 1993. Tesseler M. 1998.J. Jay Pee Brothers New Delhi. p. First edition. : Corneal degeneration and dystrophies in Modern Opthalmology Vol. Sanders D and Goldberg FM. New Delhi 1987. Little Brown and Co. 1989.J.

and Albert.T. and Randall J. 1998. Jay Pee Brothers. and Asdourian G.T. 50. and Wagle A.C. : Uveitis in Current ocular therapy. Gupta V. Roy F. 54. Wood A. second edition. Boston. Rosenbaum J. edited by Dutta L. Calcutta. Vol.J. p. edited by Franfelder F. Dutta L. Pamela S. Gittinger J. 78-81. edited by Scheic H. : Corneal and conjunctival calcification in Current of ocular therapy. Current Book International.. p. : Ophthalmology : Principles and practice. edition 5. and Ocular tuberculosis. p... 5th edition page 329-330 edited by Fraunfelder F. 1st edition. Boston 1998. D M. 46-47.C.. Philadelphia 1983.H. Helm C. B. Saunders Company Philadelphia. Shaffer D B : Paediatric ophthalmology in Text books of ophthalmology. W.L. II. first edition. Ed 5. 580-585.P..K. 566-571. edited by Sorsby A.G. : Treatment of band keratopathy by excimer photo keratectomy BJO 77: 702-708 1993.C. Vol. : Acquired and congenital syphilis in Current therapy.T. first edition. 2. and George R. edition 2. and Gupta A.. edited by Harley R. Saunders Company Philadelphia..B. 41. Randall J. Vol II. edited by Gittinger J. Saunders Company. Tran D. Igerscheimer J.T. edition second. and Randall J. p. : Toxocariasis in Manual of Clinical Problems in Ophthalmology. 39. and Schanzlin D. : Chronic bacterial infection: Ocular tuberculosis in Modern ophthalmology. Saunders Company. London.B.K. Edited by Fraunfelder F. 52.. Little Brown. : Disorders of the uveal tract in Paediatric Ophthalmology.DISORDERS OF THE UVEA IN CHILDREN 287 38. 116-153.W.B. Butterworth. Shalaby I. Survey ophthalmology 38 : 229256 1993 51. 42. 5th edition p. Vol. edited by Dutta L. 49. Knox D.H. and Asdaurion G. 2000. 121-122. Jay Pee Brothers New Delhi 2000. Little Brown and Co.. : Holland G. p.H. 40.T. 97-98. I. 46. 2000.W. 1977.. New Delhi. p. W.B. WB Saunders company Philadelphia.. edited by Gittinger J. : Anterior uveitis and arthritis in Modern Ophthalmology. O’Brat DPS.. Vol.C. Vaishali Gupta and Gupta A : Ocular toxoplasmosis in Modern Ophthalmology. W. fifth edition. W.D.K. and Dunn J. p... 9th Edition. 43. 2000. : Pediatric posterior uveitis in Modern Ophthalmology. 2-5.W. p. : Herpes simplex keratitis in Manual of clinical problems in ophthalmology. 47. edition two. Asdourian G. 2.J. 149-151.A.N. 561-565. 53. W. Gillespie S. II. 217-219 Butterworth London 1963.. 519-521. Edited by Dutta L. p. : Ocular toxocariasis in Current ocular therapy.H.K. Philadelphia. New Delhi 2000. W. p. Jay Pee Brothers. Randell J. Roy F. : Spirochaetal infection-Aquired syphilis in Modern Opthalmology Vol. edited by Fraunfelder F.B. 48. Roy F. Chavis : Tuberculosis in Current ocular therapy.B. p.H. . 2000. and Roy F. Saunders Company Philadelphia 2000. 44. Gantry D S et al.H..T. Roy F. 1963. Biswas J.. edited by Fraunfelder F. and Randel J.C. 45. Saunders Company Philadelphia 2000. first edition.

Louis 1966 62. Roy F. Biswas J. 59.. 3.T. and Spalton D. 66.. Roy F. and Randall J. WB Saunders Company.T. First edition. : Pars planitis in Current ocular therapy.A.S. 60.H. 442-450.A. Mosby 1994. Vol. 103-234.T. 2000. edited by Fraunfelder F. 241. : The uvea in Text book of ophthalmology. 2000. 267-273. AJO. Saunders Company. 1995. edition 5. first edition. Vol 2. Philadelphia. 9. p. 65. p.. 2. p. edited by Fraunfelder F. Editor Ryan NR. 1992 63. 169-170. 558-593.M. W. p. 561. p. Faunfelders F. 61.. page 293-295..J. Duker J. Rao N. Hampton Company. Vrabee T..J. Editor Podos S.H. 58. : Sympathetic ophthalmia in Retina.N. edited by Dutta L. Kanski J. Forster D.J.F. and Baldassano V. and Randall J. New Delhi. Duke Elder S. : Sympthathetic ophthalmic in Current ocular therapy.598.C. 57A. Oku H. : Common Panuveitic entities in Modern Opthalmology. Edition fifth.R. Butterworth. 2nd edition.B. : Injuries in Modern ophthalmology. Butterworth. Edition 5. : Vogt Koyangi Harada Syndrome in Current Ocular Therapy. Philadelphia 2000.T. Second edition. : Rare idiopathic specific uveitis syndrome in Clinical ophthalmology. edited by Fraunfelder F. Roper Hall M. 57D. II.. Roy F.A.B.. Edition 5. Vol.. p.S. W. 64. Roy FH and Randall J. 320-322. Biswas J. Vogt Koyangi : Harada Syndrome. Moorthy R. : International uveitis study group : Recommendations for evaluation of intraocular inflammation diseases. 517-519. edited by Fraunfelder F. Mosby. p. and Rao N. p.S.. New Delhi 2000. Mosby. Saunders Company. . Saunders Company... edition II.288 PEDIATRIC OPHTHALMOLOGY 55. Yanoff M. Roy F. Philadelphia. Rao N. W. Jay Pee Brothers New Delhi 1998. : Kaposi sarcoma in Current ocular therapy. edited by Dutta L. St. edited by Duke Elder S. Sur Opthal 39 : 265-292.. 528-529. 56. : Disease of the uveal tract in Systems of ophthalmology. 57E.T. 62A Rao N. p. edited by Fraunfelder F. 571-579.H. 5-7. Vol. Vol. Lippincott..T. W.B..B. p. Saunders Company Philadelphia 2000. Deborah Pavan Langstone : Uveal tract in Manual of ocular diagnosis and therapy.D. 57C. : Ocular manifestation of AIDS in Ophthalmology Secrets. edited by Harley R. 2000.T. Inomata H. A. Spitzberg D. Moorthy R. Jay Pee Brothers. 57B. Block-Michele and Nussen Blat B.J. 1987. London 1964. 1989.C. Knox D. p. 57. Jay Pee Brothers. edited by Fraunfelder F.H.. Philadelphia.S. 5th edition. 1983. : Acquired immune deficiency syndrome in Current ocular therapy.. Second edition. 1729 35. : Disorders of uveal tract in Pediatric ophthalmology. London. and Randall J. 191-196. Saunders Company..B. Philadelphia 2000. p. and Perkin E. page 555-556. Dunn J P : Ankylosing spondylitis and Reiters disease in Current ocular therapy.H. fifth edition. Philadelphia 2000. W. Third edition. : Intermediate uveitis in odern ophthalmology.

Kottow M. Sanders D.H.S. Roy F. 75.. S.E. : Essentials of Opthalmology. 70. Vol. 1998. 76. p.J. 2000. W. Kone Paul I.R..B. Vol.. 9. Hunyor A. 5th edition. 1987. Jay Pee Brothers. Wheeler D. 1181-1182. and Roberston J. and Randall J. p. Sander D. 2. 71.E.T.H. WB Saunders Company Philadelphia. p.T. Saunder Company Philadelphia 2000. 239-241.P. Roy F. 235. Yurdkul S. Zimmerman L.. edited by Fraunfelder F. 69. p. Vol. edited by Fraunfelder F. 2. W. 72: 1039. Edition 5. Edition-5. 721-725. edited by Duke Elders.H. CME Series4. Saunder Company. 1971. New Delhi. : Juvenile Xanthogranuloma in Current Ocular Therapy.. first edition. edited by Peyman G. J. New Delhi 2000. Jay Pee Brothers.. edited by Peyman G.E. p.P.. 73. 134. 78.F.. Roy F.K. 74. W. and Bahabri.T.F. 77. Edited by Fraunfelder F. Am. 249.. and Tewari H. 1st Indian Edition. : A critical reappraisal in light of new observation and current concept of embryopic tumors. Verma L. : Bacterial Endothalmitis in Current Ocular Therapy. II. New Delhi 2000.H. Saunder Company.B.A. New Delhi.C. J. : Disease of the uveal tract in Systems of Opthalmology.A. edition-5. 925. : Medullo epithelioma in Current Ocular Therapy. Venkatesh P. edited by Dutta L.. 72. Masuda K : Behcet’s disease in Current Ocular Therapy. and Pulkin J. Vol. Apple D.. p.T. Philadelphia 2000..T. All India Opthalmology Society.DISORDERS OF THE UVEA IN CHILDREN 289 67. p. Jay Pee Brothers. . 160-161.H.B. Edited by Fraunfelder F. 456-460. O’ Keef M. : Fluorescein angiography of the anterior segment in Principles and Practice of Opthalmology. CBS Publishers and Distributors. Duke Elders and Perkins E. : Clinical features of Behcet’s disease. first edition.. Randall J.R.A... Philadelphia 2000. 2000. Randall J. Henry Kimpton London 1966. Agrawal L.Pedia. Vinita Singh : Surgical treatment of Strabismus in Modern Ophthalmology. : Management of Endothalmitis.. Oph. 68. : Leucokoria in Principles and practice of ophthalmology. Randall J. and Goldberg M. and Goldberg M. Roy F.

3.4 The lens is suspended between the iris and the vitreous. The posterior surface of iris is in contact with the anterior surface of the lens. There are only three possible disorders that can afflict the lens they are A. a large vitreous chamber behind and a smaller aqueous chamber in front. For its nutrition it depends exclusively on aqueous humour in postnatal period. Disorders of lens are met from intrauterine life to ripe old age.0 mm. Change in shape C. The iris glides smoothly over the anterior surface of lens in presence of light.50 mm to 4. The anterior and posterior surface differ in curvature. to focus light on the retina. Average diameter of the adult lens is 9. at birth it is 6. 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.CHAPTER 9 Disorders of Lens in Children GENERAL PRINCIPLE Lens is a unique tissue in the body.50 mm. The lens is a disc shaped structure that looks circular when looked from front and back and elliptical when seen from the sides. The curvatures of the lens is variable.00 mm in adults. It is solely ectodermal in origin. ANATOMY OF LENS1. It has only one function i. The pupil is in front of the central point of the anterior surface around the anterio posterior axis of the lens. Change in transparency B. its maximum anterior posterior thickness is 3.00 mm to 6. In accommodation the curvature increases. inflammation and degeneration or dystrophic. It is avascular.e. It is more curved in infancy and child hood. accommodation and convergence. No new growth is known to develop in the lens. Displacement.2. The lens along with its suspensory ligaments divides the interior of the eye into two chambers. It is not capable of getting infection. 290 . does not have any nerves hence lens is devoid of any sensation.

So long the colour of the lens does not interfere with vision it should not be called cataract. The capsule B. These are ultimately converted to embryonic nucleus. There are two types of lens fibbers (1) Primary lens fibres that develop from the posterior layer of the lens vesicle. In infancy and childhood there is an ill-defined adhesion between the posterior capsule and anterior vitreous fossa called capsulo hyaloid adhesion. the posterior capsule rests in this fossa. . Lens fibres 1. There are no cells over the posterior capsule. These cells form the lens fibbers. The lens epithelium is limited in an area between the equator and under the anterior lens capsule. The structure commonly referred to as capsule of the lens is not a true capsule. Anatomically the lens has following parts A. The central part of the anterior surface is called anterior pole while corresponding posterior point is called posterior pole. Nucleus. This uneven thickness is due to metabolism of epithelial cells and amount of direct stress exerted on it. It envelops the lens from all sides its thickness is not uniform. The posterior capsule is devoid of any epithelium. It is a homogenous layer without any cells. 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. repeated needing put undue traction on the vitreous that is transmitted to the retinal periphery resulting in traction detachment. extensive. Epithelium C. This adhesion gradually disappears by fifth decade. The capsule has no healing capability of its own. The future growth of the lens depends on the integrity of these epithelial cells. Cortex 2.DISORDERS OF LENS IN CHILDREN 291 The two surfaces merge into each other in a rounded equator. It is the basement membrane of the under lying. thinner at the anterior pole and thinnest in the posterior pole. The lens epithelium is metabolically most active part of lens. epithelium. opacities on the periphery do not cause any visual change. The shape of epithelium varies from place to place. in the central part they are polygonal. C. at equator they are columnar and gradually become conical with apex towards the centre of the lens. It is thickest at the equator. it is supported by the vitreous behind. The normal lens is for all practical purpose crystal clear in childhood. in prequatioreal area they are cuboidal. does not bulge much in accommodation and force directed by contraction of zonules is minimal. D. As age advances the colour becomes yellowish white that gives a false impression of cataract after fifty years of age. In children any traction on the lens like attempted intracapsular lens extraction. There is a small depression in the anterior vitreous called patelar fossa. The imaginary line joining the two is called axis of the 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. B. Zonule A.

(a) The embryonic nucleus is the innermost and earliest to come into existence it is formed by primary lens fibre. So child has more cortex and small nucleus while a person in sixth decade has larger nucleus and scanty cortex. There is no histological demarcation between the cortex and the nucleus. middle and posterior attaching to anterior. The nucleus not only enlarges in size it gets sclerosed as the person ages. The zonules are infact tertiary vitreous. The lens fiber that are not converted into nucleus are known as cortex. nucleus of present day is the cortex of yesterday5. The oldest fiber are deepest. they do not meet at a point. 2. middle and posterior part of equator respectively. the number of fiber keep on increasing throughout the life without much gain in size of the lens. (b) The foetal nucleus develops from the secondary lens fibber over the embryonic nucleus. their only purpose is to keep the lens in the papillary area and help change its curvature during accommodation. There are two sutures the anterior one is an erect Y while the posterior one is inverted Y.292 PEDIATRIC OPHTHALMOLOGY (2) Secondary lens fibres are derived from the equatorial part of the lens epithelium2. the adult nucleus develops there after. Size of adult nucleus keeps on increasing without much change in overall dimension of the lens. The central fibers are the oldest and peripheral are most recent. it is mere condensation of centrally pushed lens fiber. as the posterior cells elongate the lumen becomes crescent shaped and becomes completely-extinct. Lens suture. The posterior cells elongate to obliterate the space. initially there is a circular empty space surrounded by single layer of cuboidal cells. The peripheral soft fibers are called Cortex while relatively hard fibers are called Nucleus. There are three groups of zonule. The nucleus of the lens is not a separate entity. The sutures start forming in the first two months and seen in foetal life. The secondary lens fibers keep multiplying through out the life. The fiber in the same level meet in a peculiar fashion forming a Y shaped figure called. 1. The nucleus of the lens. The secondary fiber are not long enough to span the lens pole to pole. They are (a) Embryonic (b) Foetal (c) Infantile (d) Adult. (c) The infantile and adult nuclei are formed after birth. They extend from the apices of the ciliary process to the capsule. . It may be said. The older fibers are pushed towards centre and are converted in to the nucleus. The advantage of these sutures are that they give an elliptical shape and better optical property to the lens. The anterior. The infantile nucleus is completed before puberty. (d) The zonules are entirely different from the lens. According to period of development the nucleus has been assigned various names. in life they are pin point in size. The size of the embryonic and foetal nuclei do not change. developmentally and functionally.

The lens contains 65% water and 35% protein. Ninety percent of protein is water soluble. There is difference in development of cell forming the anterior and posterior walls of the lens vesicle. infantile and adult nucleus. formation of new lens fibber is shifted to the equatorial region . 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. 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. These fibber are called secondary lens fiber. the capsule envelops the growing lens fully. The posterior zonule may extent from oraseratta to equator of the lens. By fifth month it is fully atrophied. DEVELOPMENT OF THE LENS The lens is ectodermal in origin. 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. they are devided into three types alpha . The lens capsule that is a true basement membrane starts forming and by 13mm stage. PHYSIOLOGY OF THE LENS8.DISORDERS OF LENS IN CHILDREN 293 The anterior zonule extends for a few millimetres on the anterior lens capsule. the mouth of which gradually narrows and by 8mm stage it closes completely and separates from the surface ectoderm. it covers the developing posterior capsule and the equator. The postnatal lens is avascular and gets its nutrition solely form aqueous.9. The tunica vasculosa lentis. Its presence becomes evident at 4mm stage7.10 Lens is avascular. The . depends upon aqueous for its nutrition. iris and AC. This contact between two surfaces triggers thickening in the surface ectoderm called the lensplate. After formation of embryonic nucleus. it has two parts. 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 fibber meeting at the centre of the core form the anterior and posterior Y sutures. The tunica vasculosa lentis starts regressing even before the whole of the lens has formed. Developmentally. It has both anaerobic and aerobic metabolism. beta and gamma crystalline and remaining portion is insoluble albunoid. As more and more fibers are formed at the equator. the highest concentration of protein in any tissue. when the optic vesicle comes in contact with the surface ectoderm. As the fibers are formed the nuclei of elongated cells disappear this is one of the causes for clarity of the lens. 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 earlier fiber are pushed centrally and compressed to form various nuclei : the foetal . 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 anterior vasculosa lentis and posterior vasculosa lentis. The surface ectoderm closes to form the subsequent corneal epithelium.

The amount of ascorbic acid and glutathion decrease with age and cataract formation. The congenital anomalies may be : 1. They are in the form of opacities. A. Loss of transparency 2. Many a times it is difficult to differentiate between the two. B. the lens fibers laid before or after the stoppage of cataractogenesies remain clear. others are detected late due to associated diminished vision. The transparency of the lens depends greatly on its metabolism and physiochemical status of protein. The lens protein is organ specific and not species specific i. Anomalies that involve embryonic and foetal nuclei are true congenital and others are called developmental defects. The lens has a good concentration of ascorbic acid which is synthesised by the lens and ciliary epithelium. In cataract almost all the protein is converted into insoluble albunoid. Congenital loss of transparency. The congenital opacities are generally central. Some of the congenital anomalies are obvious at birth. Associated with systemic disorders 4. Various combination of above three. The cataracts in childhood are frequently stationary because the cataractogenic factor effects a particular zone of developing lens. Congenital anomalies can be 1. Change in position 4. It also contains glutathion both in oxidised and reduced form. are generally soft. 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. Combination of all above B. . Loss of transparency complete or partial is clinically known as congenital or developmental cataract.e.294 PEDIATRIC OPHTHALMOLOGY concentration of insoluble protein increases with age. dens and may be large enough to cover the entire papillary area and. CONGENITAL ANOMALIES OF THE LENS Congenital anomalies of the lens are common but all need not be treated as many are asymptomatic. Congenital anomalies can be : 1. squint and nystagmus. The opacities that involve embryonal and foetal nuclei are called congenital while rest excluding traumatic are developmental. Limited to Lens 2. Change in shape of the lens 3. Congenital and developmental cataracts are often bilateral but can be unilateral. 25% to 30% of cataracts are hereditary how ever some time they may be sporadic. Associated with other anomalies of the eye 3.

12 Birth Trauma Placental haemorrhage leading to poor oxygen supply to foetus. steroids. Toxoplasmosis is yet another cause of congenital anomaly if acquired during late months of pregnancy maternal syphilis can also cause congenital cataract. Lenticular Congenital cataract can be either nuclear or cortical. A broad classificais dividing these cataracts into the broad categories as :— A.12. It is frequent for nuclear cataract to become total cataract. The capsulo lenticular cataracts are— 1. universally accepted classification. These are generally sporadic11. Maternal radiation during pregnancy. .e.15 due to genetically determined chromosomal anomaly. CLASSIFICATION OF CONGENITAL AND DEVELOPMENTAL CATARACT tion16 It is difficult to have a uniform. Capsulo lenticular (Capsulo cortical) B. Posterior capsular—Mittendorf dot. posterior lenticonous.DISORDERS OF LENS IN CHILDREN 295 Etiology Etiology of congenital and developmental cataracts are not well understood it can be: (i) Idiopathic in 40 % to 50 % of cases. It is not uncommon for both to exist in the same eye. mumps influenza. rubeola.13. Metabolic disorders (i) Foetal (ii) Infantile ● ● ● ● (iv) Foetal ● ● ● ● ● ● ● Various syndromes Prematurity Persistence of anterior hyloid system Intra ocular tumours.14. Endocrine disturbance.13 Maternal malnutrition specially fat soluble vitamins. thalidomide. (ii) Heredity one third of all congenital and developmental cataracts are hereditary. (iii) Maternal ● Maternal infection during pregnancy commonest virus resulting in cataract is Rubella. Anterior polar and anterior capsular cataract. A. 2. Drugs : many of the drugs if taken during first trimester result in foetal cataract i. others are cytomegalo inclusion virus. Posterior polar cataract. 3.

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. The Capsulo lenticular cataracts 1. prolonged contact between anterior lens capsule and cornea. Disciform 2. loss of A. spear shaped cataract.C..296 B. Posterior polar and posterior capsular cataracts. These are generally bilateral and associated with nystagmus. This is formed due to incomplete absorption of anterior part of the hyaloid artery that supplies nutrition to the lens during . The lenticular cataracts are— 1. The anterior polar cataract may be associated with persistent pupillary membrane or central corneal opacity. Sutural 3. (b) Acquired. Zonular (a) Lamellar (b) Total Cataract (c) Cataracta central pulverulenta. They are generally small slightly raised plaque. Others—Corkscrew cataract. (d) Membrenous 2. Sometimes there may be localised opacification of anterior lens capsule resulting into true anterior capsular cataract. Posterior polar and posterior capsular opacities are as common as their anterior counterpart. blue dot cataract. A. There are two forms of posterior polar cataracts. Coraliform 3. This occures frequently in badly managed ophthalmia neonatorum resulting in central perforation. the size of the reduplication cataract is either same as the base of the pyramidal cataract or larger. Coronary PEDIATRIC OPHTHALMOLOGY 4. Occasionally the two opacities may be joined by a strand of opacity traversing the clear zone in between. Anterior polar cataracts are common but not detected unless specifically looked for under magnification. Anterior Polar and Anterior Capsular Cataract. one commonly known as Mittendorf dot. Sometimes these are described separately but as their clinical presentation. The opacities are stationary and do not cause much visual disturbance. hence do not need any treatment. Axial According to shape the catracts can be :— 1. More frequent are reduplication or imprint cataract where a second opacity develops little below the anterior polar cataract with a clear zone in between. 2. Christmas tree cataract. Sometimes there may be a third imprint still posteriorly. pathogenesis and management are similar they are being discussed in one group. 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.

Congenital nuclear cataract.C. On examination the opacity may cover whole of the pupillary area.L. non progressive with out much visual loss. how ever it is generally discovered and diagnosed during routine retinoscopy or direct ophthalmoscopy. Translucent lenses with good vision need no treatment. The condition is not known to involve the nucleus16. 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. CATARACTS INVOLVING THE NUCLEUS AND DEEPER LAYERS OF CORTEX Most of these cataracts are developmental and have been put under category of lenticular developmental cataract2. the lamellae may be single or multiple. symmetrical and stationary. The sporadic cataracts are generally due to derangement of calcium metabolism in the mother with vitamin “D” deficiency during growth of the lens. These are dens. the opacity develops in a layer that corresponds with maximum level of noxious substance. 2. on dilatation a clear zone of the cortex is visible both on retinoscopy and ophthalmoscopy. It does not require any treatment. 1. If the opacity is dense enough it causes diminished vision. boys are more effected than girls. it may be prenatal or postnatal. This type may be confined to the embryonic nucleus due to changes at 3 months gestation. Most of the lamellar cataracts are hereditary. The fibres laid before and after this cataractogenesis are clear. Some times a small stalk of vessel may remain attached to the lens. Otherwise the treatment is removal of opacity by standard microsurgical method preferably with P.75 mm it is most probably prenatal otherwise post natal. some may benefit with dilatation of the pupil. transmitted as autosomal dominant trait may be present in parents or in siblings. On the surface of the opacity many ridges are visible on oblique illumination. Lamellar cataract is commonest congenital cataract. The opacity may be fully opaque or may be translucent. The opacity inside and out side the affected nucleus are clear. The commonest in the group is lamellar or perinuclear also refereed as zonular cataract.e. it amount for about 50% of all developmental cataracts. Treatment depends up on size and density of the cataract.DISORDERS OF LENS IN CHILDREN 297 first three months of gestation. 5. This condition is stationary and cause visual disturbance due to its proximity to the nodal point of the eye. It is generally bilateral. powdery hence called pulverulent cataract which are generally bilateral.I.O.16. . It most commonly develops in foetal and infantile nuclei in lamellar formation. Actual size varies according to on set of cataractogenesis and level of lens fibres involved. 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. and amblyopia it should be operated by any standard microsurgical procedure. 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.

These are discrete opacities in the Y sutures of the embryonic nuclei. A type of sutural cataract seen in premature children are known to fade by third month post natal19. The systemic manifestations in infected mother are mild and passed off as common cold with enlargement of occipital lymph glands. The two capsules come close to each other with some opaque lens fibres in between without nucleus. One or both the sutures may be involved but need not be symmetrical. The rubella cataract is central dense opacity to begin with. main being eyes. There is no antiviral agent known to be effective against rubella virus. do not cause any complication hence do not require any treatment. Hence in all cases of congenital membranous cataracts possibility of rubella should be kept in mind. others may be visible with bright oblique illumination. 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. (c) Membranous Cataract. and can be prevented by prophylactic vaccine given in school going age. ear and heart. (b) Blue dot Cataract. some of them visible only with slit lamp. which becomes pearly and spreads to cover whole of the lens and may loose fluid later to become membranous.298 PEDIATRIC OPHTHALMOLOGY The other type is total nuclear cataract that may involve infantile. 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. (Coralliform) and anterior axial cataract. 3. This is very common congenital anomaly of lens. The virus remains alive in the lens for months. The opacities are scattered in the centre of the lens. The condition is bilateral. symmetrical and stationary may hamper vision. They are generally bilateral and stationary. The rubella virus belongs to a group of toga viruses and has single antigenic type21. The infected mother has 50% chances of having child with congenital anomalies half of them will have ocular involvement12. Other cataracts (a) Sutural Cataracts. They result due to absorption of soft cortical matter in congenital cataract of long standing spontaneously or following trauma . foetal or embryonic nuclei. In utero the virus causes cells to have increased doubling time and short survival time. The opacities of various sizes. They generally do not require any surgery but thick membrane may be needled folled by standard correction of aphakia. The opacities do not hamper vision. . They are not really coloured blue. The virus passes to the developing foetus via placenta and affects organogenesis of multiple organs. The growing lens develops areas of necrosis that loose transparency. These are not separate type of cataracts. Other variants are : Floriform. they are white but due dispersal of violet light20 they look blue. RUBELLA CATARACT Rubella cataract is caused by systemic infection of the mother by rubella virus in first trimester. The virus is found all over the world with pockets of endemic areas.

2. galactose itself is converted into glucose. Hypoglycaemia C. A. Bone lesions. Congenital heart disease. 3. In galactocemia . hemolytic anaemia and occasional central nervous system involvement are infrequent systemic manifestation. rubella retinopathy. Galactosemia Galactosemia is one of the inborn errors of metabolism that causes developmental cataract. thrombocyotopaenic purpura. The other source being endogendus in form of neurolipids in small quantity. Hepato lenticular degeneration (Wilson’s disease) F. iris hypoplasia. hepatitis. prematurety and mental retardation making the management more difficult. The lactose is hydrolyzed in to glucose and galactose in the intestine. CONGENITAL AND DEVELOPMENTAL CATARACTS DUE TO INBORN ERRORS OF METABOLISM CONSIST OF A. Congenital glaucoma is a common feature with congenital rubella cataract but is independent of pathological process of cataract formation. Homocytinuriaand other amino aciduria (Low’s Syndrome) E. squint and nystagmus (d) Associate glaucoma requires separate management. Only definitive treatment of congenital cataract of (a) Surgical removal of Cataract (b) Management of aphakia (c) Prevention and treatment of amblyopia. Management of rubella Cataract 1. There is no known medical treatment for the infected mother or child. transient corneal haze. Galactosaemia B. Hypoparathyroidisionm.DISORDERS OF LENS IN CHILDREN 299 Congenital rubella cataract is part of a syndrome of triad that consist of A. The neurosensory hearing loss is not always present at birth but may develop in preschool age. Diabetes mellitus D. D. If rubella vaccination is made part of universal immunisation the scourge of rubella syndrome with its morbidity and mortality can be abolished altogether. Neurosensory hearing loos C. Besides cataract there are other congenital anomalies of the eye they are : Microphthalmos.. Galactose is not available separately as source of food. Congenital rubella is known to cause still birth. The systemic manifestation besides heart disease and neurosensory hearing loss are many. It is a metabolic product of lactose which is an important carbohydrate content of mammalian milk. Prophylaxis in mother : Rubella is fully preventable disease following rubella vaccination between 12 to 14 years of age. Congenital cataract B.

Commonest being transferees deficiency the epimerase deficiency is extremely rare. (g) Hepatic function test. The systemic changes are serious and even life threatening they consists of vomiting. The opacity spreads rapidly to cover whole of the lens within few months. diarrhoea hepatospleenomegaly. dehydration and hepatio spleenomegaly (b) Examination of urine for reducing substances. The infant is healthy.300 PEDIATRIC OPHTHALMOLOGY this conversion of galactose to glucose is hampered due to deficiency of one of the three enzymes required to complete the metabolic cycle. other specific tests consists of (d) Estimation of urinary and serum galactose. The opacity may initially look like a zonular opacity with clear zone all round. (e) Assay for uridyltranasferase in peripheral red cells. The urine is subjected to chromatography for specific band denoting galactosemia. Management. 2. failure to gain weight. This may retard or reverse the opacity. show refractile opacities in the centre of the lens. The cataracts are central and bilateral. and opacification of the lens. the cataract develops in first few years of life. their natural history is similar to transferase deficiency cataract except that kinase deficiency cataract is slower than the former. If opacity persists it is managed by any of the standard procedure. hepatic and renal dysfunction. Glactokinase deficiency galactosemia is rarer than the former it is more benign26 has no systemic changes. (f) Urine examination for albumin and aminoacids. anaemia. As soon as it is conformed that the child has galactosemia all mammalian milk products are withdrawn from the diet. galactokinase and epimerase. The progress of the opacity can be stopped or altogether reversed if milk and milk products are fully eliminated from the infants feeds. 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. (c) If reducing substances are present the urine should be examined for osazone crystals which are absent in galactosemia to exclude diabetes. The three enzymes involved in galactose metabolism are Transferase. 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. cirrhosis. The first change may be posterior lenticonous that on examination under slit lamp within few days of birth. mental retardation. vomiting. 1. . Concentric refractile rings may develop round the central opacity. Diagnosis of galactose cataract consist of (a) Clinical examination of the new born with diarrhoea.

The lens is microphakic. lamellar. 5. C. Deprivation of sugar for first forty eight hours also result in hypoglycaemia. The edges of the lens are not visible even with maximum mydriasis. 6. Generally an infant of a diabetic mother without biochemical control of hyperglycaemia. Lowes syndrome. Diabetic cataract Diabetic cataracts are not seen in new born they are generally met with in juvenile diabetic’s. these children have mental retardation. The infantile . Fabry’s Disease. Hypoglycaemic cataract Hypoglycaemic Cataract in infant is a cataract developing in a hypoglycaemic infant or child. these cataracts are bilateral. 3. 2. 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. Manosidosis. Progress of cataract can be prevented by treatment of hypoparathyroidism.28 1. To begin with they are snow flake like opacities under the capsule. Lenticular changes cause anterior capsular sunflower cataract. incidence of hypoglycaemia is more in premature infants. Also called oculo cerebro renal syndrome effect mostly boys. Cataract of childhood in miscellaneous errors of metabolism26. The accumulated glucose is converted into sugar alcohol which imbibes water and over hydrates the lens . Homocystinurea (see ectopia lentis) CHANGE IN SIZE OF THE LENS Microspherophakia Normal diameter of an adult lens is 9 mm and that of new born is 5. Lens opacities may be capsular. Some neonates may be hypoglycaemic without mother being diabetic. Initially these cataracts are reversible if water electrolyte and metabolic disorders are corrected within first two or three days. nuclear or total. 4. Spots like opacities in the posterior capsule is caused due to deficiency of α mannosidase.27.DISORDERS OF LENS IN CHILDREN 301 B. D. repeated diarrhoea is another cause of hypoglycaemia all these condition may precipitate hypoglycaemic cataract which is reversible if treated well in time.75 mm. Management of diabetic cataract is like any other cataract of childhood done with proper 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. Hypoglycaemia in new born can result due to various causes in the infant. It results in spoke like opacities in cortex without disturbance of vision. Hypoparathyroidism. Wilson’s disease (Hepatolenticular degeneration) This is due to inborn error of copper metabolism. Caused due to lac to α galactosidase. Repeated attacks of hypoglycaemia lead to lamellar cataract by 1 to 2 years of age. diabetic cataract has been seen in children as young as one year. Cause congenital cataract associated with congenital glaucoma. It is an acute cataract due to accumulation of glucose in the lens.

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 exact cause of the deforming is not known. The cornea is of normal size so is the globe. CONGENITAL ANOMALIES OF SHAPE OF THE LENS There are two types of anomalies of shape of the lens due to congenital cause. Generally there are no other congenital anomalies of the globe. The anterior chamber may be deep on the periphery with mild iridodonesis the real diagnosis is made on dilatation of the pupil. may prevent rise of tension. With a widely dilated pupil the periphery of the lens gives a crescent like reflex. The child is brought due to diminished distant as well as reading difficulty. The anterior posterior diameter of the lens is 25% more than normal. resulting is subluxation which is common. The condition is bilateral. Lenticonus. A..31 B. It is presumed that it results due to arrest of development of lens during 5th or 6th month of gestation. Reduced equatorial diameter and increased thickness of the lens gives it an appearances of a spherical lens with small diameter hence the name microspherophakia. use of mydriatic is better avoided because the lens may subluxate in the A. A peculiar phenomenon called glaucoma inverse results if the pupil is constricted either due to prolonged exposure to bright light or use of miotic.75 as against 9mm of an adult lens. The spherical lens obstructs the miotic pupil resulting in pupillary block and rise of tension. Besides correction of index myopia a constant watch should be kept on possibility of pupillary block. The lens may occasionally come in the anterior chamber.C. Management of the condition is difficult due not only to index myopia. however it can be eccentric or peripheral. 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. On retinoscopy the central part shows high myopia with or without astigmatism. Prophylactic iridectomy or iridotomy. The tension falls with mydriasis. The terms lenticonous and lentiglobus are used as interchangable terms. Coloboma of the lens. A. There is a very rare condition where the posterior cortex develops a local bulge with in the confines of the capsule32. 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. There may be associated angle closure. Lenticonous is a condition where the poles of the lenticular capsule bulge out side the confines of the capsule. The condition may have only ocular component or may be associated with systemic anomalies resulting in Weill-Marchesani syndrome.302 PEDIATRIC OPHTHALMOLOGY lens gradually increases to adult size in the second decade. Cataract is common and should be managed like any other cataractus ectopia lentis. poor accommodation but also secondary pupillary block glaucoma. The cone is situated within 5mm of the posterior pole. Posterior lenticonous is more common. .

When the zonules are weak alround. In what is called coloboma of lens. On slit lamp examination zonules are generally absent at the site of coloboma. a site common for uveal coloboma. The cause of diminished vision is curvature myopia. As the area of coloboma is deprived of normal pull of the zonule. there is no actual loss of substance of the lens matter as the name suggests. the lens is generally partially cataractous. If the media is dense and the bulge is not visible on slit lamp. Management. There may be a history of lenticonous in the family lenticonous may be associated with many systemic congenital anomalies. adjacent to an area of coloboma. Coloboma of the lens does not require any specific treatment. The lens shows progressive opacity in the posterior cortex in the cone and around the cone. There is flattening of the circumference. Coloboma of the lens is usually unilateral. scissors movement are common. amblyoipia is common. If there is not much of visual loss. The condition may not be diagnosed unless the child is brought for improvement of vision or correction of squint which is common.DISORDERS OF LENS IN CHILDREN 303 The anterior lenticonous are bilateral while posterior lenticonous are unilateral. It may involve the cortex or adult nucleus and not embryonic or foetal nuclei. it is thicker and the periphery is more rounded than normal lens. B. On retinoscopy there is central myopic and peripheral hypermetropic refraction. One of the complications of colobomatous lens is subluxation. The exact cause is not known. Colobama of the lens Coloboma of lens depends upon development and integrity of the zonules. Occasionally there may be a dent in the lower equator34. U S G may delineate the bulge. On examination there is an oil drop appearances. they result in . Dense opacities can be managed either by limbal or pars plan route with posterior capsulotomy anterior vitrectomy and IOL. A notch in the lens is very rare. The anomalies can be 1. 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. with dilated pupil The change in curvature is visible on slit lamp. However danger of amblyopia is always present that should be managed as per standard methods. The coloboma is generally not visible if the pupil is circular and requires myriasis to be visible. CONGENITAL ANOMALIES OF ZONULE Congenital anomalies of zonules are not uncommon but they are generally not discussed as separate entity simply because the result of such anomaly are reflected as anomalies of the vitreous.33 The lens generally has curvature myopia with astigmatism and poor power of accommodation. contact lens and mydriatics my help. 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. Changes incurvature of lens is seen equally in both sexes. spectacles. Generally it is situated at down and in position.

Transparency of the subluxated lens 4. . Unilateral ectopia is rarely congenital it is mostly due to trauma or other ocular causes like chronic uveits . (c) The lens is behind the iris. 2. Area of the lens present in the pupil.one phakic which is generally myopic and the other area aphakic that is hypermetropic. When the defect is localised the result is a coloboma of the lens. A lens is sub luxated when some of the zonules are still attached to the lens capsule. 1.304 PEDIATRIC OPHTHALMOLOGY speherophakia. and systemic cause of ectopia lentis ruled out. Ectopia lentis Next to cataract. retinal detachment. It may be mild enough to be missed unless examined with slit lamp under complete mydriasis. Tilt of the lens. displacement of lens is most common congenital anomaly of the lens. In all cases of unilateral ectopia lentis the other eye should be carefully examined for subtle signs of ectopia. if the zonules are lacking all-round the lens is dislocated. B. Symptoms of ectopia lentis depend mostly on 1. 6. Complications like amblyopia. CONGENITAL ANOMALIES IN THE POSITION OF THE LENS A. 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. A lens is said to be ectopic when (a) Its centre is not in the anterio posterior axis of the eye. Associated error of refraction and 5. (e) The edge of the lens in the pupillary area dividing the pupil into two parts:. 2. 3. (d) Partly visible in the pupil. 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. Associated systemic condition. (b) Some of the zonule are still attached to the lens. 2. glaucoma. 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.

Complication (a) Raised intra ocular tension (b) Myopic degeneration (c) Rhegmatogenus retinal detachment 12. depending upon other associated and anomalies. one aphakic and other phakic causes moderate visual symptoms of unilateral diplopia and fleeting visual disturbances. 2. Bilateral moderate subluxation may cause polyopia. 9. 8. A lens that divides the pupil in two halves. the phakic area is myopic whereas the aphakic area is hypermetropic. Uniformly or partially deep A. Diminished near vision 3. 4. 7 On fundus examination—doubling of the fundus i. The symptoms can be divided into Mild. . retinal detachment etc. 6. The edge of the lens may give a crescent shaped reflection on retinoscopy. Occasionally there may be diminished vision due to errors of refraction independent of subluxation. Associated anomalies (a) Mesodermal dysgenesis of anterior chamber (b) Megalocornea. A lens that has subluxated but its edge lies in between the pupillary margin and iris root may not produce any visual symptom.e. 1. Pupillary shape and size are variable. a large area seen through the phakic myopic area and a small area of fundus seen through hypermetropic area. 3. The aphakic part which is hypermetropic and has both diminished distant as well as near vision. 2. myopic astigmatism. 10. Ectopia lentis with associated complication have various grades of visual loss due not only to ectopia but also associated lesions like glaucoma. The eye invariably has strabismus in unilateral cases and also when the refraction is grossly different in both the eyes. gross and associated complications. (See below) D. moderate.e. Signs of Ectopia lentis consist of 1.C. or due to subluxation i. may have normal accommodation of the age. 4. The refraction may not be symmetrical and equal in both the eyes. The phakic part is myopic or myopic astigmatism with accommodation. On slit lamp examination the zonules may be visible in the aphakic area and vitreous may herniate though the aphakic area. Generalised or localised tremulousness of iris 5. Diminished distant vision that may or may not be fully corrected by glasses or contact lens. 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.DISORDERS OF LENS IN CHILDREN 305 C. 11.

Danlos syndrome (j) Mandibulo facial dysostosis. (b) Homocytinuria. Associated systemic anomalies (a) Marfan’s syndrome. the normal pupil is replaced by a slit like or oval opening complete with constrictor and dilator muscles. The angle of anterior chamber is clear. They generally have good distant as well as near vision for many years but may develop anterior dislocation. the pupil is shifted opposite the shift of the lens i. the pupil is shifted down and in. . glaucoma. 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.306 (c) Cornea plana (d) Persistent primary hyperplastic vitreous (e) Retinitis pigmentosa (f) Congenital glaucoma. transmitted as autosomal dominant inheritence. if the lens is displaced up and out .35 It may rarely be sporadic. PEDIATRIC OPHTHALMOLOGY SIMPLE ECTOPIA LENTIS Simple ectopia lentis represents isolated ectopia without any other ocular or systemic disorder. 13. (g) Keratoconus (h) Ectopic pupil (i) Aniridia (j) Ptosis. is bilateral symmetrical. ECTOPIA LENTIS et PUPILLAE37 This is transmitted as autosomal recessive inheritance. The edge of the pupil generally bisects the pupillary slit. cataract or retinal detachment36. It is generally familial.e. the lens is subluxated up and out. (k) Sturge Weber syndrome. The condition may be considered as mildest form mesodermal dysgenesis. (c) Weill Marchesani syndrome (d) Reiger’s anomaly (e) Crouzon’s syndrome (f) Oxycephaly (g) Hyperlysinemia (h) Sulphite oxidise deficiency (i) Ehlers .

the lens rarely shows a scalloped lower border or a notch. The pupil retains normal light and near reflex. On retinoscopy the phakic area generally gives a compound myopic astigmatism while the aphakic portion has hypermetropic refraction generally less than +10D. 3. Common predisposing factor being trivial blunt injury. The lens is generally clear but may have localised opacification at the lower equator. 2. The fundus seen from the phakic area is generally larger while seen through the aphakic area is smaller than emmetropic fundus. Iris. the aphakic portion covers lower half of the undilated pupil. Skeletal C. The subluxation is gradual but does not progress much. The pupil is smaller than normal due to poor development of dilator fibbers. Gonioscopy shows wide angle with multiple changes that include ill defined Schwalbe’s line. subluxation may occur at any age. On ophthalmoscopy duplication of fundus is seen. The lens. 8. The surface of the iris is relatively smooth. Though the condition beings in infancy its full blown presentation is felt in teens. 5. The changes are bilateral almost symmetrical. deeper over the aphakic part and shallow or normal over the phakic part. 6. It has been reported to run in generations and common among the siblings. Miscellaneous A. The anterior chamber is irregularly deep. 4. The subluxated lens gives a crescentic dark reflex on oblique illumination due to reflection of light from rounded edge of the clear lens. frequent and prominent ocular changes are seen in the lens.DISORDERS OF LENS IN CHILDREN 307 MARFAN’S SYNDROME38.C.e. The eyes commonly have axial myopia with corresponding myopic fundus changes.39. The lens is subluxated upwards in both eyes. Ocular Most important. 1. . Cardiovascular D. all of which need not be present simultaneously:– A. Generally the lens is slightly smaller in diameter it may be microspherophakic. the child has difficulty in near work. The lower iris is generally tremulous. they are mirror images of each other. Occasionally the subluxation may increase and the lens may even dislocate apparently without any reason. Angle of A. The pupil is resistant to usual mydriatic and cycloplegic. It is seen equally in boys and girls with world wide distribution. occasionally there is phacodonesis. The syndrome has following component. On slit lamp examination the zonules are visible some of them may be broken. 7.40 Marfan’s syndrome is an autosomal dominant disorder of mesodermal tissue with multi systemic involvement. The disorder does not have any biochemical deficiency that may be linked to the anomaly. Ocular B. It is usual for patient to have normal accommodation for the age but if the subluxation is more i. Pupil.

The chest is long and narrow. 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. the child will benefit by minus glasses with constricted pupil or aphakic correction with dilated pupil. When error of refraction is present it is generally axial myopia. The thumb can be extended back wards to touch the radial border of the forearm. the skin is loose without subcutaneous fat. Occasionally there may be hemivertebrae. . Cardiac changes Cardiac changes begin with dilatation of aorticroot. the fingers are long. malformation of ears. spidery hence called arachnodyctalous. and spinabifeda. rarely their may be ptosis. 10. weakness in abdominal muscles predispose hernia formation. fuciform aneurysm of aorta is common and the aneurysm may show dissecting changes. over lap each other. keratoconous or even cornea plana. E. 11. 12. The cornea is normal but may show megalo cornea. With a large aphakic area in the pupil it should be decided if. The tip of the thumb passes beyond the ulnar border of the palm when flexed. rigid central pupil. Management Management depends upon severity and duration of the condition it consists of:Improvement of vision 1. C. Aphakic power is considerably counter balanced by presence of axial myopia hence it is invariably less than + 10D.308 PEDIATRIC OPHTHALMOLOGY 9. The metacarpals and phalanges are long and thin. flat foot. most striking are long slender limbs. B.Q and there is no biochemical changes. The sclera is thin and stretched may have small staphylomas. Skeletal Changes Skeletal Changes are prominent and may be the first cause to bring the child to the physician. Cornea. The children have normal I. leading to aortic insufficiency. The head is often dolicocephalous with high arched palet. because the aphakic is exclude from the pupil due to small. If there is gross difference in refraction between the two eyes there may be squint and ambloyopia.The spine shows scolio kyphosis. the span of out stretched arms is more than the height of the child. the sternum shows pactus deformity. Other changes consists of Winged scapulae. Error of Refraction. The child is tall and thin for age. The large joints are prone for subluxation. Sclera. 3. contracture of joints. 2. D.The muscle are hypoplastic. The thumb and index finger of one hand when wrapped round the wrist of the other hand. Some children may not have much visual disturbance inspite of subluxation if there is no error of refraction. pulmonary and renal anomalies.

Lens. gives positive bio chemical test for uninary homocystine.e. However the child may show signs of delayed physical milestone from early age. methionine metabolism42. The options open are. It’s skeletal and ocular features have many similarities with that of Marfan’s syndrome. The accommodation is poor or absent. The retinal detachment can be as a part of axial myopia or vitreous disturbance. The lens should not be tempered with unless it is : Cataractous. The condition has following components: A. This is initially attributed to illhealth and diminished vision. (c) Aphakia is managed by spectacle or contact lens.Homocytiruria is associated with mental retardation. 5. Skeletal C. Positive biochemical test. The clinical signs are not noticed at birth. It may require extensive surgical procedures. A. synthase that helps to convert methionine to cystine . The subluxation of lens becomes apparent by tenth year.DISORDERS OF LENS IN CHILDREN 309 4. The condition manifests itself by eight to ten years. Homocystinuria is one of congenital errors of metabolism due to deficiency of more than one enzyme i. dislocated or causing glaucoma. 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. As the zonules are broken which may be matted . The syndrome is caused mostly due to lack of cystathionine B. HOMOCYSTINURIA Homocystinuria is second most common cause of bilateral subluxated lens. The main causes of the clinical features are excess of methionine (an essential amino acid) and homocystine in blood. Ocular B. Cardiovascular E. The result is rise of methionine and homocystine in body fluid. The mental retardation becomes obvious as the child goes to school. Bilateral progressive subluxation of lens that has the tendency to fully dislocate. The patients excrete large amount of homocystine in urine. The ocular changes comprise of 1. The disorder is equally common in boys and girls. Mental D.iris clip lens. (a) The lens is generally removed through limbal route. lens in the sulcus or sclera fixated lens all which has their advantages and disadvantages. (b) The posteriorly dislocated lens is removed through parsplana with extensive vitrectomy. It is not possible to put an IOL in the bag in subluxated lens following phacofragmentation. Important differentiating point are:.

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. Retina. The iris is generally tremulous over the aphakic part. 5.310 PEDIATRIC OPHTHALMOLOGY over the lens surface39. Cardio vascular changes consist of Various congenital anomalies of heart. The child with homocystinura generally does not live beyond third decade. . The limbs are long. There is high incidence of retinal detachment mostly due to myopia. Vision. Most of the children have diminished distant vision due to: (a) Error of refraction (b) Glaucoma (c) Squint (d) Amblyopia B. E.C. Joints are prone for subluxation. Sclera.May develops staphyloma due to associated buphthalmous. The spine shows kyphosis and scoliosis. and the skin is dry. Feet are flat with floppy gait. thus the aphakic power is generally less than +10 D. D. All these factors make these children very poor surgical risk. 3. 4. the check bones are flat. the fingers are spidery (arachnodactyly). C. with malarflush. If the lens dislocates in vitreous it may start phacogenic uveitis and glaucoma or may remain silent. Thromobo embolic episodes are common leading to-cerebrovascular accidents. The Sclera may be stretched and thin . 44 Cyanide sodium nitropruside test is a good secreting test but not conformatory due to high rate of false positive results. The lens may develop lamellar opacification. Biochemical test43. They have high rate of death during general anaesthesia. The skeletal changes are The skin is light coloured. The aphakia produced due to subluxation or dislocation compensates pre existing axial myopia. 2. Myocardial infarction in early adulthood and pulmonary embolism are frequent. Osteoporosis is common leading to fractures. However aphakic detachment is very common following lens exaction. Iris. . The mental changes consist of Low IQ and other psycho somatic disorders are common. Hernia is common due to under developed abdominal muscles. Occasionally there may be aniridia which makes diagnosis more difficult. However some of the children may have normal intelligence. The shape of the lens may be small and spherical.

45b. Ocular Management is similar to any other ectopia lentis. The only common feature of Weill Marchesani syndrome with the former two is ectopia lentis. It is an inherited disorder of mesoderm. Bacterial contamination of urine of normal child may also give false positive result. Mentally retarded children may have to be put in institutions meant for mentally and visually challenged. This gives bright red colour which is common both to homocystine and cystine present in urine.45c This is the third of the three common disorders that cause ectopia lentis with systemic involvement. The shape of the lens is not apparent unless the pupil is dilated. F. The zonules are visible on slit lamp examination. (2) Increased risk of thromboembolic phenomenon that makes them poor surgical risk. WEILL—MARCHESANI SYNDROME45a. The lens may completely . Non ocular A.DISORDERS OF LENS IN CHILDREN 311 The test consists of: Adding two ml of 5% sodium cyanide to 5ml of fresh and acidified urine. Microspherophakia where the lens is small in equatorial diameter but has greater than normal anterio posterior diameter. The spherophakia results in index myopia. The lens has tendency to move anteriorly rather than upward or downward. (3) Only positive point in homocystinuria is that the condition can be helped by : (a) Restricting dietary methonine and supplementation of oral cystine42. It may have both types of inheritance i. Ocular B. (b) High doses of vitamin B6 (Pyridoxine). The block is enhanced by use of miotics. The dose recommended is 600 mg to 1200 mg daily and folic acid orally. F. This mixture is allowed to stand for ten minutes. This results into a ball and socket pupillary block. Management Management of homocystinuric child is more complicated due to : (1) Associated mental changes which may hinder visual rehabilitation. It is rarer than Marfan’s syndrome and homocystinuria both of which have many common features.C. (c) Those individuals not responding to pyridoxine may be put on Betaine that reverses degradation of methonine. with dilated pupil the lens has a circular bright crescent on the periphery. autosomal dominant or recessive. is shallow and tremulousness of iris is better seen on the periphery. Occasionally the A. XVII. The Ocular features are 1. G. To confirm the diagnosis of homocystinuria electrophoresis is required. The components of the disorder are A. then two to four drops of sodium nitropruside is mixed to the previous solution.e.

B. Non ocular signs The non ocular signs are striking. Comparison between Marfan. The patients have a short stubby stature. Prevention of glaucoma by doing prophylactic surgical peripheral iridectomy or laser iridotomy. Management Consists of 1.C. 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.312 PEDIATRIC OPHTHALMOLOGY dislocate in anterior chamber with its resultant complications.C. by iridectomy or laser iridotomy. . the hands and feet have squarish shape. 4. The tension is relived flowing maydriasis or establishing a path between A. There is no fixed pattern of cardiac disorder. life expectancy is better than in homocystinuria and no specific biochemical change have been attributed to the conditions. homocystinuria and Weill-Marchesani syndrome. 2. Secondary pupillary block glaucoma is most troublesome complication. Removal of dislocated lens. 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. and narrowing of angle which itself may have strands of mesodermal tissue. well developed subcutaneous fat. 3. The thorax looks larger as compared to the body. there is no mental retardation. and the joints are prominent and stiff. Correction of error of refraction. 2. They have brachycephly. loss of pattern iridodonesis. arms and legs are short. muscles are hypertrophied. resulting in shallowing of A. Their maximum height seldom exceeds five feet and many are considered to be dwarfs and investigated as such. the children have assorted cardiac anomalies. They have tendency to develop carpal tunnel syndrome. Posterior dislocation is less commaon. Management of lenticular opacity by standard microsurgical procedure.C. and P.

myopic astigmatism Retained Retinal detachment rhegmatogenous or traction Deep in upper part shallow in lower part Non-specific 313 Irregular. phacogenic or due to mesodermal change in angle Axial myopia. Deep in lower part shallow in upper part Pupillary block. 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 . Slender Dolicocephalic Scoliosis. 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.C. 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. myopic astigmatism. kyphosis Long slender Poorly developed Hypoplastic Long thin Arachno dactyly Loose. due to pupillary block Inverseglaucoma Glaucoma Refraction Accommodation Retina Axial myopia. iris may bulge in A.DISORDERS OF LENS IN CHILDREN A.C.

Aniridia E. thinning of cornea.314 PEDIATRIC OPHTHALMOLOGY OTHER SYNDROMES WITH ECTOPIA LENTIS A. is caused due to inherent defect in collagen. kertoconous. These children have low IQ and have retardation of growth and laxicity of joints there is no specific treatments. megalocornea. Keratoglobus D. Hyperlysinemia C. Reigers anomaly F. (Generally unilateral rarely bilateral. Ehlers Danlos syndrome B. Angioid streak is a common feature in fundus. blue sclera. Stretching of cornea and sclera predispose laceration of eyeball. The children have short life span and mental retardation there is bilateral dislocation of lens. Buphthalmos : Primary or Secondary. The other systemic presentation is hypermobility of joints. Adult cataracts have excellent visual . may be noticed in infancy.) PAEDIATRIC CATARACT Incidence and prevalence of paediatric cataract is far less than adult cataract however cataract in childhood has far-reaching consequences. Blunt trauma. There is no biochemical defect. The ocular features are Sub laxation of lens in both eyes. may have ophthalmoplegia. OCULAR CAUSES OF ECTOPIA WITH OUT SYSTEMIC INVOLVEMENT A. No specific treatment is known. High myopia B. causing extensive ecchymosis and hematoma. Hyperlysinemia45. Ruptures in Bruch’s membrane and stretching of retina result in retinal detachment. microcornea. Sulphite oxidase deficiency A. There is no specific treatment. Sulphite oxidase deficiency In the broad sense this is derangement of cystine metabolism due to sulphite oxidase deficiency. Uvitis G. This is an inborn error of metabolism due to lysinedehydrogenase deficiency. Its two systemic findings are hyper elasticity of the skin that ruptures on slightest trauma. microspherophakia. Ehlers Danlos syndrome is a rare disease generally seen in adults but becomes apparent in childhood. Its ocular manifestations include ectopia lentis. intelligence and life span is normal. C.

and correction of maternal malnutrition.DISORDERS OF LENS IN CHILDREN 315 result following lens extraction. Some frequent syndromes are Lowe’s syndrome. in such cases the other eye must be examined in detail for subtle signs of cataract. Congenital and developmental cataracts have a strong heredity. parathyroid disorder and malnutrition. 4. Position of the opacity. . Symptoms of congenital and developmental cataract46. They may go unnoticed if the other eye has good vision. Unilateral cataracts. Opacities larger than normal pupil cause more visual loss. All opacities in the lens do not cause visual disturbance and may go unnoticed till later life. In another group of children. many of the children present as unilateral cataract. However sporadic cases are also common. Denser opacities cause more loss of vision than translucent opacities. 3. homocystinuria and other aminoaciduria. 5. Childhood cataract surgery has poorer visual prognosis and more complications. Trauma and intraocular diseases are common causes of unilateral cataracts. incidence is higher in developing countries where hardly any prophylactic measures are taken for maternal rubella. the mother is normal but the child has various errors of metabolism i. 2. Congenital cataract is a leading cause of blindness in children about 20% of blindness in children is due to congenital cataracts46. 48 Symptoms depend on 1. A.e. Though most of the congenital cataracts are bilateral. B. Congenital cataract may be the only anomaly or else it may be associated with other maldevelopments of the eye. In developing countries trauma is a major cause of cataract in children. There are numerous syndromes associated with congenital cataract. 3. other causes can be maternal diabetes. galactocimia. Size of the opacity. Commonest cause being maternal rubella in first trimester. Visual loss is more in central cataracts than peripheral. Alport’s syndrome and Turner’s syndrome. Density of the opacities. The cause of congenital cataracts can be 1. 2. Number of the opacities. In one third of the cases of cataract no cause can be detected. 4. 5. same is not true with childhood cataract. only few require treatment. hypoglycaemia. these eyes develop amblyopia and squint more frequently. only to be discovered on routine examination. The matter is worsened when pupil constricts in bright light or the child has moitic pupil due to any cause. Unilateral cataracts have poorer prognosis as compared to bilateral cases. opacity near the nodal point is likely to cause more visual disturbance than one away from it. It is estimated that congenital cataract is found roughly one in every 250 live births46 Very few have clinical symptoms. Scattered small opacities hardly produce any symptom but nuclear opacity with posterior capsular opacification cause more visual loss than any one of them. incidence also increases due to consanguinity47.

so the child is brought to get it excluded. (c) There is positive history of developmental cataract in the family. Even children with normal IQ may fail to attain expected academic grades due to poor vision. Unilateral dense cataracts draw attention earlier than posterior cortical and posterior polar cataracts. Microphthalmos. 5. They are fist brought with squint. persistent hyaloid system. 4. photo phobia or redness of eye. retrolental fibbroplasia. Associated with symptoms of other systemic syndromes. 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. Congenital cataracts that are apparent before three months of age cause intractable nystagmus. midwife or the attending neonatologist. 9. The child with unilateral cases may close the effected eye to wardoff the glare. 3. Peripheral and posterior opacities are missed initially. persistan primary hyperplastic vitreous. UNILATERAL CATARACT Unilateral congenital and developmental cataracts are unique in many ways: A. Unilateral cataracts are detected late because the child carries out his routine with better vision in the other eye. (b) The child is brought with nystagmus or squint. nystagmus that dates back to first three months. . 1. Congenital cataract per se does not cause pain. Sick children who fail to thrive are likely to have metabolic cataract or rubella cataract. 30% of idiopathic congenital and developmental cataracts are unilateral. (ii) Faint. squint or diminished vision in various combinations. Some of the heredity cataracts are also unilateral. 8. 7. B. Some children are brought with glare. Associated ocular malformation. Physical under development is a frequent associated feature of congenital cataract. Frank mental retardation is common in homocystinuria.316 PEDIATRIC OPHTHALMOLOGY 6. 10. Reigers anomaly have poorer vision and prognosis. 7. It is only with the dilation of pupil the cataract becomes obvious. Some of the children are referred by paediatricians who have diagnosed a syndrome that is likely to have cataract. Age of on set. C. Such an infant is brought to ophthalmonologist by parents with the suspicion that the child does not have expected vision. 6. Older Children are brought with white reflex. If these symptome are present the child should be investigated for retinoblastoma. Presenting Features of Congenital and Developmental Cataract Various age groups have different presenting features. 2.

All white reflexes in pupillary area in children are not congenital cataracts. (See differential diagnosis of white reflex in pupillary area. All neonates who have hepato spleenomegaly. rubella and toxoplasmosis.I. Unilateral traumatic cataracts have better vision following cataract surgery than congenital and developmental cataract. WORK UP IN A CASE OF PAEDIATRIC CATARACT A. Generally they are brought with strabismus. retinal detachment etc. B. The traditional midwives and nurses can be taught to screen neonates for lenticular opacities. As infants can not be examined on usual slit lamp they should be examined either with hand held slit lamp or under operating microscope.DISORDERS OF LENS IN CHILDREN 317 C. J. Children with unilateral cataract seek medical help later than bilateral cases. I. Post operative visual prognosis in unilateral congenital or developmental cataract is always poorer than bilateral cases.L than conventional extra capsular cataract operation49 with spectacle or contact lens. Positive family history of developmental cataract in parents. F. D. All cases of unilateral congenital and developmental cataracts should be encouraged to have P.) E. . abnormal heart sound and fail to thrive should be examined for possibility of galactocemia. uveitis. H. siblings and first cousins should alert the physician for possibility of lamellar cataract. neonatologist for evidence of dense lenticular opacity. In case of obvious cataract in one eye the other eye should be examined for of subtle signs of cataract. the lens should be removed to improve peripheral field on that side. Urine examination for reducing substance will exclude galactosaemia while torch test28 is helpful for rubella and toxoplasmosis. Developmental cataracts that have relatively clear lens for first few years like posterior lenticonous have good visual prognosis. It should be kept in mind that all non lenticular opacities need not be retinoblastomas. All cases of uniocular cataract should be examined in details for evidence of occult or forgotten trauma. Examination of an eye with congenital cataract 1. G.O. D.C. Binocular loupes give very low magnification. Eyes of all new born children should be examined routinely by attending obstetrician. 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. C. Cataract should not be confused with retinoblastoma and vice versa. E. Galactokinase deficiency is seen in otherwise healthy children with congenital cataract. In absence of these an examination with uniocular loupe with a bright torch is good enough.

5% to 1% is most suited for children and infants. is most likely to be a buphthalmic eye where examination of tension under generally anaesthesia is a prerequisite. The posterior segment is examined for presence of remnants of hyaloid system. 7. If no clear zone is visible between the lens and iris.C and brisk pupillary reaction generally has normal. 9. (b) Atropine should be used with usual caution to avoid side effects. The choice of mydriasis is as follows: (a) Tropicamide 0. MANAGEMENT OF PAEDIATRIC CATARACT A. Pupillary light reflex in infants is poor due to strong constrictor muscle.Scan can help to find out posterior capsular defect that is common in many congenital cataracts. It can be seen well under magnification.5% is also a suitable cycloplegic. clear A. 4. 3. It is contra indicated in Down syndrome. A widely dilated pupil delineates outer border of the opacity. Next step should be examination of anterior segment under full mydriasis. 8.5% drop along with 2% home atropine. retrolental fibroplasia and other fundus changes. It is very important to explain the procedure and its utility to the parents. Anterior chamber is examined for other malformation of anterior segment and evidence of other diseases. Poor dilatation of pupil means a rigid pupil that may cause difficulty during subsequent surgery. A large cornea with haze.318 PEDIATRIC OPHTHALMOLOGY 2. foreign body. An eye with bright cornea of normal size. surgery can be postponed and child is kept on mydriatic. exudates.T. associated detachment. The macula is specially examined for evidence of central choroiditis in case of suspected toxoplasmosis.Scan delineates vitreouscortexmix. In lamellar cataract the periphery is clear. in case of bilateral dense opacities and all unilateral cataracts. All children posted for surgery should under go a pre anaesthetic check. intraocular tension. (d) Phenylpherine is generally used as 2. Fundus can be examined through this clear zone both by direct and indirect ophthalmoscope. So are the eyes with mesodermal abnormalities. haemorrhage. 5. If the eye has a large clear lens on the periphery. deep A. B. especially. 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. (c) Cydopentolate 0.C. In traumatic cataracts. Management of paediatric cataract is difficult and frustrating because of many factors some of them are: . 10. prompt surgery is indicated. B. In cases where fundus can not be seen with dilated pupil USG of the eye and some time C. A preoperative check up by neonatologist may be ordered by an anaesthetist. may have to be under taken. 6.

The eye of an infant is small and changes rapidly with age. Cataracts in childhood may be associated with anomalies of other intra ocular structure that hamper improvement of vision. 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. Various modes of management available for paediatric cataracts 1. (c) Trauma as a cause of cataract requires involvement of parents. (b) In galactosemia. 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. Genetic counselling is not always possible. 2.These children under atropine may be provided with near correction. If every girl in school going age receives a shot of rubella prophylaxy as part of national immunisation. Atropine has a draw back that it abolishes accommodation that is an important factor for learning. loss of peripheral field and appearance may be some of the hindering factors. Definitive treatment is surgery which requires high degrees of skill and efficiency. There is not medical treatment for cataract60. 6. 5. removal of milk products from the child’s diet will not only remove the possibility of child developing cataract but may improve transparency of the lens. The child has a long life to live that includes education. Non-Surgical This depends on presence of useful clear zone between the pupillary border and periphery of the opacity. Dark glasses during day may also serve in limited way. 4. Nystagmus and amblyopia develop fast in infancy and child hood. B. the danger of rubella cataract can be wiped out altogether. .DISORDERS OF LENS IN CHILDREN 319 1. 3. tipcats. diminished accommodation. 9. vocational training. arrows. Children should be explained about possibility of blindness by sharp objects like spears. and catapult. All the opacities are not of uniform nature as regards density and position hence a uniform protocol can not be advised for all cases. choice of profession where diminished vision. 7. 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. in children . 2. The children should be taught to sit with back towards the light and light falling on the books. teacher and social workers. 8. 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. 10. Prophylaxis (a) Prophylaxis is limited to rubella cataract and to some extant galactosemic cataract. Scope of prophylaxis is limited to rubella. Cataracts may be associated with larger systemic syndromes including errors of metabolism.

52 and at four years it is 22 mm. prevent amblyopia and maintain fusion as far as possible. Till few years ago it was thought that intra ocular lens be implanted only after 2 years of age50 Now a days. The child should be watched for evidence of increase in size of opacity. If the opacity is stationary the eye can be atropinised and bifocal prescribed when the child starts schooling.8mm 3. . The most ideal choice will be to use an IOL that will make the eye slightly myopic. 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. spectacles or epikeratophakia are some of the alternatives available.49. If the discs in both eyes are visible.5mm 7. elevation and size. The last method has been given up in favour of secondary implant. with an IOL of 32D will make it myopic by 8 to 10D. It is better not to operate both the eye at the same time. are of normal colour. The aims of surgery are to preserve vision. otherwise a simple aphakia is left to be corrected by. 5. For accurate reading this has to be done under general anaesthesia which by it self is a highly specialised discipline. which. Unilateral cataracts of all densities have uniformly poor visual prognosis.2mm 3. 7.5mm Adult 23.8ml 6. 8. At 2 years it is about 20mm51. The other difficulty faced is difficulty in calculating IOL power in children below 2year of age. 6.1ml 9. This amount of myopia reduces the distant vision greatly.00mm 2. contact lens. The child’s axial length will reach its adult size by seven years.00mm 10. and epikeratophakia.5mm 2. Comparison between various dimensions of a new born and adult eye is given below : Feature Axial length Volume Lens diameter Corneal diameter A. 2.1mm 11. This will give a comfortable near vision as well.O.C. depth New Born 16. 4. 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. both maculae are visible with out any abnormality chances are that vision in both eyes are equal. An opacity larger than 3 mm requires removal.5mm) emmetropic. Some of the anatomical difficulties in implanting IOL in children are 1. An IOL of 32D will be required to make an eye of a new born (axial length 16.I. implanting a lens within 48 hrs of birth is considered safe47. In such cases Lensectomy followed by contact lens. IOL is with held only when its placement is not possible. IOL in Children Older children can tolerate P. Surgery PEDIATRIC OPHTHALMOLOGY Surgery is the definitive treatment for all paediatric cataracts. Next difficulty is to decide when to implant an IOL. In bilateral cases lensectomy is followed by IOL implant. The eye ball is small in all dimensions.L as much as adults. Lensectomy with IOL is the standard procedure for most of paediatric cataracts.C. Children with dense opacities. spectacles.320 3.

This negating the optical advantage of spectacle.IOL. 4. They are given near correction in the form of spectacles. (c) Combination. easy to manage. frequent displacement of lens and expulsion of the contact lens. bifocals may suffice for near vision. Aphakia in children is managed by (a) Spectacles (b) Contact lens (c) Secondary implant (d) Epikaratophakia (e) Combination of more than one method. Once the child can manage the contact lenses gas permeable lenses may be prescribed. have a tendency to look over the frame. 11. strong blink reflex. Spectacles in children are generally prescribed in all ages more for economic reasons than its optical efficiency in developing countries. Many of the pediatric aphakics need secondary lens implant. Up to age of six daily wear or extended wear contact lenses may be tried. There may be traumatic dislocations or spontaneous dislocation in buphthalmos. Other methods of management of aphakia in children Commonest cause of aphakia in children is surgical removal of lens. In majority of cases P. Presence of nystagmus is always a very poor prognostic factor. Other causes are spontaneous absorption of a congenital cataract due to posterior leak or absorption following penetrating wound. (a) Spectacles. 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. amblyopia must be treated vigorously. Next problem is to decide which type of lens should be implanted. Contact lenses are best alternative to IOL. Hard contact lenses are difficult to insert due to tightness of the lid. Of course there is a school of thought that considers the infants eye to be too small and too immature to put a P. Its advantages are: It is cheap.C. 10. others prefer to fit extended bear lens after 10 days.C. It generally develops by two month in case of congenital cataract. if the posterior capsule is intact.IOL because except an iris claw lens all lenses are over sized for the placement and fixation14. If there are evidences suggestive of asymmetry in vision. some surgeons fit contact lens just after the lensectomy.C.DISORDERS OF LENS IN CHILDREN 321 9. Contact lenses do not give use full near vision. An iris claw lens may be put even in a partially absent posterior capsule. megalocarnea or chronic uveitis. It is better to leave an eye aphakic rather than pseudophakie with A. A. (b) Contact lenses49.IOL are put with satisfactory results. gives fairly good distant vision.47 and recommend an iris claw lens with maximum width of 6mm to 7 mm with optics of 4mm. If power is equal in both eyes chances of amblyopia is reduced. . All the parents can not be taught to insert them. IOL are out of question because of high rate of complication.C. Some time spectacles may be required to correct residual power over contact lens or IOL.

Their numbers vary from single to many.O. They are generally seen in the pupillary area. Placement of IOL and type of IOL.O the second. B. Management of post operative uveitis. Amount of cortical matter left.L. Most of the children will develop PCO with in one year. micro surgical extra capsular cataract extraction with or without IOL phacoemulsification. Almost all eyes develop P. is by far denser in children than in adults. Incidences of after cataract depends upon : 1.C. 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. Common symptoms of after cataract are 1. accidental or spontaneous. Mechanism of after Cataract Formation The posterior capsule of lens is devoid of epithelium. Elschnig’s pearl and the last Soemmerring ring. Rarely injury to lens during vitrectomy may also result in after cataract formation. yet some of the fibres are left behind. The size varies from pin head to 2–3 mm in diameter. . The after cataracts besides lens fibbers also contain pigments. may proliferate as globular bodies or may be entrapped between the anterior and posterior capsule. following rupture of lens capsule that may be surgical. The first is called simple P. Non improvement of vision 3.C. 5.O. exududate and blood in the initial stages. Common surgical procedures that result in after cataract are extra capsular cataract extraction. It is a major cause of gradual lowering of vision following uneventful I. Age of the patient : almost all aphakic children following extra capsular lens extraction develop PCO. Associated pre existing uveitis 6. opacification of posterior capsule with proliferation of lens fibers. 4. White reflex in pupil which may be faint enough to be seen with magnification or denser that is visible in diffused light 2. P. 3. C.O. these opaque fibbers may spread over the posterior capsule. 2. 54. needling aspiration of soft cataract. Frequent change of refraction. Elschnig’s pearls are formed due to proliferation and migration of epithelial cells to form a fish spawn like small white translucent bodies. 55 A.C. Dilatation of pupil may expose more. It is the after math of rupture of lenticular capsule with partial absorption of cortex. that continue to proliferate as opaque fibres. After cataract is not a true cataract.C.O. Duration following lens extraction : may develop soon after if coxtex has not been removed well. In milder form it is also known as posterior capsular opacification or simply P. In an extra capsular cataract extraction following capsulotomy as much of cortex as possible is removed.322 PEDIATRIC OPHTHALMOLOGY AFTER CATARACT53. implant.

O. Treatment 1. There may be gaps in between.O. The post operative uveitis should be managed promptly.C. Most important thing is to remove as much of cortical matter as possible during the initial surgery.C. F.O. In dense after cataracts the parents complain that cataract has not been cleaned well or has developed again. The ring may occasionally be dislocated in the anterior chamber. use of biconvex IOL and use of heparin coated IOL.C.O than canopener capsulorrhexis. Once the after cataract has developed and is dense enough to cause reduction in vision it should be cut either surgically or by laser. This is formed due to adhesion of anterior capsule to posterior capsule entrapping cortex as well as equatorial epithelial cells. age of the patient. IOL causes more opacity than with P. . large capsulorrhexis. The ring is generally hidden behind the iris and visible only with mydriasis. 2. and associated uveitis. D. time lapse after surgery. Continuous curvilinear capsulorrhexis cause less P.DISORDERS OF LENS IN CHILDREN 323 Soemmerring ring is doughnut shaped ring opacity. Clinical Features of aftercataract depend upon Location of opacity. Best treatment of after cataract is its prevention or reduction in its density. and examined for amblyopia strabismus and nystagmus. Pre School children do not complain of diminished vision.C. Lens extraction with out P. that can be prevented by posterior capsulorrhexis with anterior vitrectomy.C. Soemmerring ring and posterior capsular traction lines. E. Occasionally the posterior capsule develops wrinkles this causes. Incomplete capsulotomy as was fashion in traditional operation for congenital and traumatic cataract resulted in thicker after cataract than present day microsurgical capsulorrhexis.C.IOL. This generally happens if the child is less than two years of age. Posterior capsulorrhexis is done routinely with or without anterior vitrectomy in children to reduce P. they have to be tested for vision. the space inside the ring has fainter P. Other features observed are Elschnig’s pearls. A biconvex IOL or a lens with posterior convexity also lessens P. On set of nystagmus in infants following surgery means that vision has not improved.C. stretch lines on the posterior capsule resulting in fluctuating vision and glare. 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. density of opacity.O. It must be remembered that cent percent children develop some degree of P. When examined under mydriasis with slit lamp shows various grades of opacity ranging from translucent to dense white membrane. Signs consist of white reflex in pupillary area. Heparin coated IOL or heparin used in irrigating fluid diminishes chance of after cataract. 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.

the observed eye should also be able to see.324 PEDIATRIC OPHTHALMOLOGY 3.D. but it is common to find iris incarcerated in the wound. Cystoid macular oedema or worsening of cystroid macular oedema 2. to facilitate intra-ocular examination and other manipulation. YAG is the treatment of choice. Injuries commonly associated with traumatic cataract are: Penetrating injury with or without retained intra ocular foreign body. blunt injury. Domestic injuries are commonest cause in younger children while sports injuries are more in older children. They are less common in first three years of age. Ninety percent of injuries are avoidable. Most of them are unilateral however blast injury. glare. (vi) Unwilling parents of the child and uncooperative child. 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. amblyopia. radiation can cause bilateral traumatic cataract. Rhegmatogenous retinal detachment. (v) Peripheral retinal degeneration. Posterior segment and retinal involvement remain major cause non improvement of vision. Transient rise of IOP 3. Laser capsulotomy by N. corneo scleral or scleral injuries. electric shock. TRAUMATIC CATARACT IN CHILDREN General Consideration Trauma accounts for 80% of cataract in paediatric age group. because if observer can see the fundus. . (a) The indications of laser capsulotomy are diminished vision. Penitrating injuries do not involve lens in isolation they are associated with corneal. electrical injury and radiation. Otherwise other causes of non improvement of vision such as cystoid macular oedema. chemical injury. otherwise injury to iris and or ciliary body is very common. incidence gradually rises as the child grows. cracker injury and chemical injuries. retinal detachment. (b) Relative contra indications consist of (i) Clear view of fundus by direct ophthalmoscope through the centre of the pupil. vitreous bands and chronic endophthalmitis should be looked for (ii) Central corneal opacity (iii) Corneal oedema (v) Already existing glaucoma and cystoid macular oedema. Penetrating injuries passing through pupil generally have centre corneal scar and injury to the lens with out injury to iris. (vii) Glass I0L The laser capsulotomy is done either in normal or semidilated pupil Complications of laser capsulotomy 1. monocular diplopia.

3. some times the cortical matter gets mixed with vitreous. discrete opacities that may fade with time. 8. It may be permanent and stationary or may spread over the years. Localised as discrete opacities. Early rosette cataract develops with in few days of blunt injury. If the capsule ruptures and the opaque cortical matter spills out of the capsular bag it may fill the entire anterior chamber. Late rosette cataract develops in the posterior cortex one to two years after the injury that may have been trivial and forgotten. 2. Rarely may be total. It can occur early or late. sharp long thorns etc. 2. 4. These injuries are generally caused by sewing and knitting needles. chroidal repute or retinal detachment. punctate spots. rosette shaped or scattered subepithelial . Both contusion and concussion can cause traumatic cataract. There may be associated hyphema. shallowing anterior chamber and glaucoma if the cortical matter does not leak. This hydrates the cortex. Blunt injury may cause small. if the capsular wound heals quickly. IOFB are common. 7. which converts soluble protein into insoluble protein and leads to ultimate opacification Opacities may be: 1. Rosette cataracts Rosette cataracts are common in blunt injuries. Lenticular changes following blunt injury are less dramatic than penetrating injuries. nibs. 5. 3.DISORDERS OF LENS IN CHILDREN 325 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. The uvea may suffer the effect of blunt injury along with lenticular changes that always hampers future vision. There may be irido dialysis. faint. There is a clear zone between the opacity and the equator of the lens. pins. each line has feathery appearance due to finer linear opacities radiating from its trunk giving a star like appearance. With a watertight wound the tension may rise resulting into secondary glaucoma. These small opacities are generally stationary. static or progressive. These closed globe injuries do not cause incarceration of the uvea. In such conditions vitreous may herniate through the aphakic area. 6. Large penetrating injuries cause 1. It develops in the posterior cortex as opaque lines radiating from a central point. 9. Transient. For traumatic cataract to develop there should be some damage to any part of lens capsule that allows aqueous to reach the cortex. Total opacification in short time that may cause swelling of the lens. If the external wound still leaks the eye remains soft. If there is associated zonulolysis : sub luxation or dislocation of cataractous lens. Prolapse of uvea is common.

choroidal rupture. D. retained intraocular foreign body. It has far-reaching medico legal value. All of them may have adverse effect on visual improvement of vision. In infants. localised opacities away from pupil. E. However USG may not resolve foreign bodies . 4. The deposits on the lens capsule correspond to constricted pupil. subluxation of lens. Ocular ultrasonography C. Late rosette cataracts cause gradual loss of vision after months. 9. and posterior perforation of globe. The first and fore most investigation is careful history taking. lacrimation. type and extant of lenticular opacity. rupture of sphinter. vitreous haemorrhage etc. I.F. These are transient and may be confused with post inflammatory pigment on the lens capsule. In opaque media when fundus is not visible. the first investigation ordered should be x-ray of the orbit. No appreciable visual loss is seen in Vossius ring. retinal haemorrhage.B.I are useful to visualise small. slit lamp should be done when ever possible. choriodal haemorrhage. vitreous haemorrhage. Unexplained pain in a closed globe with cataract denotes rise of intra ocular tension. They will give information regarding status of posterior chapsule associated uveitis. 2. it should be localised. 5. punctate opacities. direct and indirect ophthalmoscope. endophthalmitis. Symptoms of traumatic cataract may be 1. twigs may present as endophthalmities. iridodialysis. Berlin’s oedema. choroidal tear. Examination of media by oblique illumination. 6.R. cystoid macular oedema. with or without rise of tension. Other uveal injuries associated with concussion and contusion cataract are: Hyphema. using any of the standard method and foreign body extracted. The child may altogether withhold history of blunt injury. 2. Examination of vision has not only diagnostic and prognostic value.T and M. Acute following penetrating injury in the form of loss of vision. Careful examination of vision in both eyes should be under taken in all cases of injury. retinal detachment. If the x-ray shows retained intraocular foreign body. retinal detachment and Berlins oedema. Injury with organic matter like thorns. 3. 7. state of vitreous. Concussion cataract may cause diminished vision after days or months. Diminished vision weeks after blunt injury is seen in early rosette concussion cataract. Investigation 1. 3. photophobia. retinal detachment. Faulty projection should warn about possibility of large retinal detachment. 4. A penetrating wound should always arouse suspension of intra ocular foreign body. In case of penetrating injury the child can not hide the history of injury but may not come out with the truth about the circumstances leading to injury and the offending object causing injury. recession of angle of anterior chamber. They fade out over months some times leaving behind faint subepithelial opacities. pupillary reaction and projection of light gives a gross assessment of vision. 8.326 PEDIATRIC OPHTHALMOLOGY Vossius ring is not a cataract but is a common effect of blunt closed globe injury where black pigments are imprinted on the anterior lens capsule in a circular fashion. retinoscope.O.

2. 3. For such a small foreign body CT is more suited as it can detect bodies as small as 1. Surgery of cataract.I. 4. in children. retina in various combinations. OTHER FORMS OF CATARACTS IN PAEDIATRIC AGE GROUP A. Difficulty in placement of IOL. Longer and stronger the steroids used more are the chances of development of the cataract. Management of other injuries.C. Presence of central corneal opacity always jeopardises visual improvements.O. The management of traumatic cataract in children 1. Most of the time it is not possible to put a PCIOL in such cases. and duration can arrest progress of cataract formation but does not reverse the process. Reduction in concentration.06 mm3. anterior vitrectomy and primary P. Management Management of traumatic cataract in children does not differ basically from adult traumatic cataract but there are some special features that need to taken into consideration. These injuries are caused due to penetrating injuries. corneo scleral injuries along with damage to uvea. Two common vision threatening conditions are cataract and glaucoma. 3. Glass fragments are visible only when they are as large as 1. frequency. Exact duration and strength that causes cataract is not known. It is easier said than done.DISORDERS OF LENS IN CHILDREN 327 less than 2 mm. Children are more prone to develop steroid induced cataract than adults.5mm3. which are : 1. The teachers and coaches should be instructed to encourage the children to use standard protective gears when ever required in sports and games.O. It may require vitrectomy. vitreous. perhaps it is influenced by genetically determined factor. These are managed by lensectomy. Difficulty in management of resultant aphakia in traumatic cataract. Parents should see to it that children do not play with pointed objects. Development of amblyopia and squint. Traumatic cataract is very often complicated by corneal. . Prevention: The best management is prevention.MRI is generally not used in suspected metallic foreign bodies because movement of metallic foreign bodies during MRI can result in MRI induced blindness56.C.L implantation. Frequent development of P. 2. If the lens is already opaque it should be removed along with repair of above injuries as primary process with reconstruction of anterior segment. Simple traumatic cataracts are generally due to blunt injuries or small penetrating injuries of the lens with other tissue intact. Steroid induced cataract Steroids when used for long time locally or systemic have profound side effects.

Replace steroid by non steroidal anti-inflammatory drugs when ever possible. Steroid induced cataract has been reported following use of skin ointment. iridocyclitis due to juvenile rheumatoid arthritis. 2. How ever it does not explain why steroid induced cataracts always start in posterior cortex.I. 3. It never develops following use of steroid for short duration even in high dose like sub conjuctival injection. Common symptoms are : Glare. glaucoma and amblyopia. Most widely accepted theory is that steroids act on the lens epithelium that is responsible for hydration of lens fibers. The opacity being near the nodal point causes much visual disturbances for its size.58.C. Generally these children have sever skin and multisystemic involvement that may over shadow the ocular manifestation which becomes apparent only if the child survives and acute systemic effects of electric shock has subsided. It may be in the form of circular dense disc surrounded by lighter areas of opacification. it is essential that all children who have been on steroid on long term should under go tests for raised intraocular pressure as well. post operative and post traumatic status. Surgical intervention when required with P. diminished distant vision. both in anterior and posterior cortex. streaks. There are various types of opacities in electric cataract. astringent. Electric cataract57. anti histamine etc. . keratoplasty and sympathetic ophthalmitis. The Electric cataract is generally bilateral.L and management of amblyopia. steps should be taken to: 1. Less common cataracts in children 1. Examine both eyes periodically for diminished vision. how ever it may be denser on the side affected more severely than the other. Exact mechanism of production of cataract by steroids is not known. and inhalers containing steroids of various strength and for a long time. In these cases steroid induced cataract may have to be taken as inevitable therapeutic risk and dealt with accordingly. 4. 5. Manage spring catarrh by weakest solution of steroid. nasal drops. Management. Exact time taken for cataract to develop is not known. interstitial keratitis. Out of all the conditions listed above spring catarrh is the most offending condition that causes steroid induced cataract in children because the parents are not explained the regime to follow who allow the children to use strongest possible steroid for quick relief. It is frequent to find associated glaucoma that may contribute to further loss of vision. diminished vision in bright light. Reduce the strength and frequency of steroid to a level that gives best therapeutic response without causing cataract. Hence. difficulty in near vision. They can be vacuoles. mastcell stabiliser. The child is likely to develop cataract when struck by electric current or lightening of more than 200 volts that passes through the head. The opacity develops as a posterior sub capsular opacity that is denser in the axis of the lens. As cataract is a common feature of steroid use.O. Generally the complete lens gets opacified rapidly.328 PEDIATRIC OPHTHALMOLOGY Conditions where steroids are used for long time and most of the time in both the eyes are-vernal catarrh.

The sun flower cataracts involve both anterior and posterior cortex and sub capsular area as yellowish dust in the centre of the lens with radiating yellowish petals pointing towards the periphery. If there are only few vacuoles present in anterior cortex with in first few months chances are that no significant opacities will develop. The overall effect of dissemination depends upon ferrous content of the foreign body. 2. the X-ray reveals its position unless it is very small CT and USG may show such bodies. The earliest change is deposition of iron in the anterior sub capsular area of lens. Management depends on correct diagnosis of retained iron foreign bodies. Occasionally a small particle may get embedded in the avascular lens through the pupil and remain localised as a small dot of lenticular opacity never progressing to full blown cataract. it is generally an alloy of iron. vitrectomy. The metal is deposited as brown rings resembling rust ring in the mid periphery. The iris also develops brown iron deposits. It is always associated with penetrating wound. after the acute multisystemic signs and symptoms have subsided. Cataract is to be managed as any other cataract following blunt injury. Management. MRI is contra indicated in metal foreign bodies. There may be hole in the iris through which the foreign body has passed. Diagnosis is based on history of penetrating injury on examination an external wound of entry is always present. chronic non granulomatous panuveitis and sun flower cataract. more sever and faster the reaction.Endophthalmitis that is supurative in nature. The metal is disseminated through out the tissues of the eye by electrolytic dissociation and gets combined with cellular protein that destroys the cells causing atrophy of the cells. The lens subsequently develops larger opacities. lens extraction and management of glaucoma. Glaucoma is common. The copper is mostly deposited on continuous membrane like lens capsule and corneal periphery59 various manifestations of intra ocular copper foreign body are . Brown pigment on lens periphery and iris should arouse suspicion of iron as foreign body. More the copper in the foreign body. It is rare in children. dyschromatopsia and diminished vision. In all cases of suspected intra ocular iron foreign bodies. The retina develops degenerative changes similar to pigmentary degeneration causing diminished night vision. This is due to electrical dissociation of retained intra ocular copper that does not combine with the cells. The elemental iron is converted into its ferrous and ferric compounds. Large foreign bodies are removed after proper . Cataract in siderosis59 Iron is one of the commonest forms of intraocular foreign bodies in adults it is rare in children. Very small inter lenticular bodies do not require removal but the eye is kept under observation for possibility of development of siderosis on long run.DISORDERS OF LENS IN CHILDREN 329 It may some times take months and years to develop cataract. Copper also gets deposited on corneal periphery as Kayser-Fleischer ring. Fully developed sun flower cataract is visible with on ordinary oblique illumination. The reaction depends upon copper content of retained foreign body. It the media is clear the foreign body can be seen with direct and indirect ophthalmoscope. It consists of removal of foreign body. Inter lenticular particle may be visible. Management. C. Sunflower Cataract59. It is rarely elemental iron.

Natchiar P-59-60 published by Arvind Eye hospital and post graduate Institution. 3. 1987.31–35.S. Commonly opacification starts in the posterior capsule. System of Ophthalmology. .C. Maduri. 2.P. . . Cataract in Lowe’s syndrome Lowe’s syndrome consists of mental retardation. Lenticular features consist of fine dots beneath the capsule that may have polychromatic lusture. New Delhi.P. 2nd Edition pp. . albumen in urine. Henry Kimpton. Banumathy S. and Nema. It is multi factorial. New Delhi. and Goldberg M. 1991. No surgical intervention should be undertaken unless the eye is quiet for at least six weak. Nancy Anderson Hamming. There is progressive loss of facial expression and ptosis. Sometimes broad posterior synechiea which are difficult to break cause anterior sub capsular opacities that are generally stationary.. Later involving posterior cortex and ultimately covering whole of the lens.F. 3–12 First India Edition. 1961.I pp. 133. Nema. Exact cause of cataract formation is not known. Management consist of early and proper treatment of uveitis and steroid should be used in its minimum possible strength and duration. REFERENCES 1. Associated glaucoma may worsen the prognosis. Removal of iron foreign body reduces chance of siderosis. Essentials of Ophthalmology. Edited by Peyman G. Apple D. 2000. H. New Delhi. Ocular manifestation consists of bilateral nuclear cataract that may be microspherophekic. The lens. 4.R. The condition is generally seen in adolescents. Anatomy of the Eye and Adnexa. First Edition pp.V. Cataract secondary to ocular inflammation Cataract is a common complication of chronic uveitis of long duration. London. The stelate appearance of such opacities are called Christmas tree cataract. Singh V. Vol. Duke Elder. convulsion. 5. which form the sheet anchor of the treatment of chronic uveitis. diabetes. The surgery should be followed by prolonged instillation of steroids as trauma of surgery results in flare up of uveitis..N. This increased tone may be associated with progressive weakness of the muscles. and Wyber K.311–338. Anatomy and embryology of the eye in Principle and Practice of ophthal mology. Jay Pee Brothers. Anatomy of the eye Edited by G.A. The two common offending factors are the toxic effect of inflammation on the lenticular metabolism and Corticosteroids. . II : Anatomy of the visual system 1st Edition pp. if opaque is removed by any of the standard procedures. Cataract in myotonic dystrophy Myotonic dystrophy is a heredofamilial disorder where muscles have increased tone that is difficult to relax. Sharma P. Sander D.330 PEDIATRIC OPHTHALMOLOGY localisation through vitrectomy. Modern Publishers. renal stone formation. Vol. Jay Pee Brothers.

119–120.F jay Pee Brother . System of ophthal mology. Congenital and Infantile Cataracts in Current Ocular Therapy Fifth Edition. Current Book International Kolkata. . 75–76 Little Brown and Co Boston 1998. . Epstein R. 11. and Paterson R. III. Adler F.H. 171–173 Churchill Living stone.P. . 15. Modern Publishers New Delhi 2000. 14. . Sixth Edition. Edited By Fraunfelder F. London 1984. Dobree J. and Albert D. Sharma P. System of Ophthalmology Vol.G. Third Edition.M. WB Saunders Company Philadelphia 2000. 13.A.M.. Miller SJH . London. Henry Kimpton London 1564. 9. Therapy Fifth Edition. . 136-.A. 598–618. Saunders Comp. Ahmed E. 68–69 W B Saunders Company. Galactosemic Cataract Am. Peyman. 25. The lens cataract and its management in Principle and practice of ophthalmology. Galactocemia in Current Ocular Therapy Edition 5th . 8. Scheic H. Berger B. Cataract in Modern Ophthal Mology Vol-4. Part-II. and Roy F. P-538-541 W. .T. First Edition Current Book Inter national Calcutta 1995. 23. Singh D. Embryology of the human eye in Text book of ophthalmology. pp. Beger W.R.V. 81–82 Wb Saunders Company. Physiology of the crystalline lens in A Text book of ophthalmology . Philadelphia. Rebella in Current Ocular. Surgery 23: 601-604(99). Henry Kimpton.A Sauders D. Dutta L. Congenital Cataracts in Manual of clinical problems in Ophthalmology Editors Gittinger J. First Edition. Goldberg M. Physiology of the eye. . . Mos by Co St. 10.C. 51–52 Oxford University Press Kolkata.Oph Oph 50:115 1960. . 19. . Edited By Deborah Pavan Langston . Duke Elders . 18.L. . 21. 7. . 688–760.-III. Sorsby. First Edition pp.H. Duke Elder S. Paediatric Ophthalmology in Manual of ocular diagnosis and therapy. Philaled phia 2000. Edited by Fraun felder F. Diseases of the lens in Ophthalmology second edition pp. G.A. 1977. pp. 16. . New Delhi 1987. .C. New Delhi 2000. 17. Essentials of Ophthalmology first edition p. 22.W.C. Butter work London 1964.B. Gittinger J. 183–212 New age international. Epidemiology of Cataract in childhood.DISORDERS OF LENS IN CHILDREN 331 6. Alcon D. 1995. Editors. 1739–1741 I. . 24. . and Roy F.H. Khurana A. . Cordes F.M. 1968. Inborn metabolic disorders and the eye in Principles and practice of ophthalmology Vol. Bonuik. pp. . The Lens in Parsons diseases of the eye 17th edition pp. Ninth Edition. Edited By Fraun felden FT and Roy F. Edited by Peyman G.F. Louis. First Edition Page 226. Edited by A. First Edition. The physiology of the eye and of vision.K.. A global Perceptive. V. pp. Edited Moses R. Dutta L.M. 20. . Ophthalmology Principles and Practice. Foster A Gilbert C. Vol. First Indian Edition pp. pp. 12. . Jr Cat and Ref.T. . C. Rahi F. 1975.W.1993. First Edition pp.B. 4th.J.K. Blue Dot Cataract in Ophthalmology Principle and Practice. and Asdourian G.

Anomalies of the lens in system of ophthalmology Vol.E. . Duke Elders . Developmental anomalies of the eye. 40. Indian Edition pp. Vol. Boston 1998.A. Hayasaka. . etal . Jay Pee brother . Homocystinuria in Current ocular therapy . 988–1012. 32. Second Edition pp. fifth edition edited by Fraunfelder FT and Roy F.II. Ectopia lentis in Manual of clinical problems in ophthal mology. 230 1985. 234–258. . New Delhi 1987. 1746–1754. 549–582. Second Edition. Edited by Harley R. 542–543.D Second Edition pp.K. Gittinger J. 30.W. In Principles and practice of Ophthalmology Vol. Maumene IHEctopia Lentis. Ida Caroline Mann . New age international (p) Ltd. Simon J.F First. Pediatric 40 : 586 : 1967.W. First Edition.M. 41. Kansky J. fifth edition. 39. Familial simple ectopia lentis. 42. .D. Clinical Ophthalmology .H. Epistine RL . Duke Elder S. 688–710. Tadeka H. edited by Fraunfelder FT and Roy F. Ocular manifestation of Marfan syndrome and homocystinuria. WB saunders company Philadelphia 1983. pp. 1102–1112. . Sander D.H and Hiles D. WB Saunder Company Philadelphia 2000. III Edited by Peyman G. Butter Worth London 1989. 543–544. 31. III. Oph 75 : 405 : 1973. Henry Kimpton London 1964. A case study . First Edition pp. Duke Elders . I.M. 43. 78–80 little Brown and company. Lenticonous and lengiglobus. Speath G L Barber G. Part-II. Edited by Gittenger and Asdourian G.J. Congenital anomalies of the lens in Ophthalmology Second Edition. Second Edition pp.D. Khurana A. WB saunders company Philadelphia. In born metabolic disorders of the eyes. AsanoY. J. . 27.R. New Delhi 2001. J Pediatric ophth and strab 22 : 227. III. Prevalence of homocystimuria among mentally retarded evaluation of specific screening test. . pp. . 28. and Jensen A. Ocular Changes in endocrine disease. Nelson L.J. 110–111. 694–675.K. 37. Surv Oph 27:143. Digeorge A. 38. 35. . M.1982. S.. Abnormalities in size of lens in system of ophthalmology Vol. Casper D. WB Saunders Company Philadelphia 1983. Cross HE . in Pediatric ophthalmology. Cross H.S. 33. Henry Kimplon London 1964. Guemes A and Elias Traboulsi . Am.A.M pp. . Edited by Harley R. . Calhoun J.B. 29.332 PEDIATRIC OPHTHALMOLOGY 26. Bloch R. Oph 88:381 1979. Goldberg.W. Vol. Guemes A and Elias Iraboulsi .S. pp. Cataract and Intraocular lens implant in Pediatric ophthalmology Vol. 34. 44. p. in current ocular therapy. Hunry Kimpton London 1964. Multiple Syndrome in System of ophthalmology . 212. Lens subluxation in homocystinuria Acta Oph 62-425-431 1984. 36. London 1957. pp. Part-II . . . Ectopia Lentis et pupillae. Microspherophakia in Current ocular therapy fifth edition edited by fraunfelder FT and Roy F.Am. III part-II first edition pp.

S. Edited by Fraunfelder FT. Singapore. Har court. . Haywood M . . J.M. Woody NC . Miller S.C.Wang XH . 53. Injuries to the eye in Parsoris Diseases of the eye. p.K.R.-I edited by LE Dutta pp.V. 54. Weill marchesani Syndrome in current oculartherapy Fifth edition edited by Fracenfeldes. Pathogeneses and management of posterior capsule opacification in Modern ophthalmology edited by Dutta L. Dutta L. 400–404. New Delhi. Published by All INDIA OPHTHALMOLOGICAL SOCIETY. . Edited by Dutta L. First Indian Edition. Walton D. Fraunfelder F. 55.E. complication and their management in Modern ophthalmology Vol. .A. Roy F.K. Jay Pee Brothers. Paediatric Cataract in CME Series. 372–378. .W. The syndrome of Marchessani Br J. Congenital cataract and other congenital abnormalities of the crystalline lens in Modern ophthal mology Vol. 10L in Children in 10L secrets . Mehta K. Jay Pee Brothers . 272. WS Saunders Company Philadelphia 2000.F. Harcourt Brace & Comp. 48. F. Dutta N.S. Infantile cataract surgery : Current techniques. 45a. Smith T. Neena Mittal Dinesh . Ocular manifestation of familial hyperlysinemia. pp. First Edition Kay Pee Brothers. 17th Edition pp. 378–384.M.A. 51. Roy F. . Current book international Calcutta 1995 58. Posterior capsulor rehexis with optic capture in paediatric cataract and intra ocular lens surery ophthalmology 103 : 175 -187 1996.T. pp.. Edited by VanHeuven .C.. Cataract in children : Diagnosis in Decision making in ophthalmology. I Edition . . First Edition pp. Sharma Y. Lens Implantation in children in Modern ophthal mology Vol. Diseases of the crystalline lens in Ophthalmology principle and practice. 47.C. Apple D. Singh D.K.T. First Indian Edition 1998 pp. Saunders Company Philadelphia 2000. 45c.C. 249–250 Churchill Livingstone. 52. 535–538 W. . . 56. J.R.WB Saunders company Philadelphia 2000. Jay Pee Brothers . 2000. Dutta L. 130–131.M.H. oph 45: 377-381. New Delhi 1992. .C. . 46.T. New Delhi 2000. pp. After Cataract in Current ocular therapy Ed. First Edition pp.W. 57. J and Zwaan J. 50. Fifth Edited by Fraunfelder F.A. Weill Marchesani Syndrome with bilateral angle closure glaucoma. Pedioph Strab 22:129-132. Allinson R. Vol. New Delhi 2000. Chrous G.J and Znaan J. 58–59.2.J. Jones R. I Edited by Dutta L. and Roy F. 45b. Wilson M. . and Lina M Marouf. New Delhi. Gimbal H. Jagat Ram Pandey S. I. Zwann.M pp.. 1985. Bluestin E. Brace and company Asia Ltd. New Delhi 2000. 224–243.M. . pp.T. . Electric Injury in Current ocular Therapy Fifth Edition. Holland M. W. 49. Wright K. Intra ocular lenses for Pediatric implant. Eye Trauma in Decision making in ophthalmology edited by Heuven W. pp. 308–312. J-cat reft sur 20 : 584-591 1994. . Biomaterials design and sizing.T.DISORDERS OF LENS IN CHILDREN 333 45. London 1984. Dada V. Tran Am Acc of oph and otolyr 75 : 355 1971.C.J. 59. 179. 1961. Jay Pee Bro. 19–24.G. Singapore 1998.

It is mostly muco poly saccaharide with few collagen fibres. 3.4 334 . B. They are 70-75 in number. 1. The epithelium is two layered. Stroma. They are similar to choroidal vessels in nature without choricapillaries. The non pigmented and pigmented layers of the ciliary process are firmly attached to each other and do not detach from each other like in retina. Structures related to drainage of aqueous. Epithelium. Structure related to aqueous production. The stroma. The endothelium of these vessels are thin and fenestreted4. The stroma of ciliary processes is very thin. They have following layers : 1. Each ciliary process has a tuft of blood vessel in the central core. Ciliary processes1. These are the sites of production of aqueous humour. They arise from pars ciliaris of the ciliary body and project towards the centre of the eye ball all around. This is the inner layer of ciliary process. It is continuation of non pigmented layer of the retina. It extends from ora serrata to the root of the iris. Tuft of blood vessels. C. Tuft of blood vessels. (b) Non pigmented epithelium. 2. It is also neuroectodermal in origin. It extends from ora serrata to the iris. (a) Pigmented epithelium2 which is neuro ectodermal in origin and is forward continuation of pigment epithelium of the retina. D. It is a single layer of cuboidal cells. Structures related to circulation of aqueous. it surrounds the blood vessels and separates them from the surface epithelium. The limbus A.CHAPTER 10 Glaucoma in Children Anatomy of the structures related to glaucoma : The structures of the eye related to glaucoma can be divided into : A. It is the outer layer of the ciliary process.3 2. 3.

This is called scleral sulcus. The ciliary body is attached to the scleral spur. 2. The angle of the anterior chamber. At the extreme periphery of the cornea on its inner side of the anterior chamber directly under the limbus is a depression that runs all round the angle of the anterior chamber. swollen lens. It is shallow in micro-ophthalmos. (b) The anterior chamber is a convex space with convexity forward. The convex surface is bounded by the posterior surface of the cornea. anterior plane of zonule and anterior part of the ciliary process. The junction of the anterior and posterior surfaces is the angle of the anterior chamber. cornea plana. The angle formed by the posterior surface with the anterior surface is almost 45°. the iris and the anterior lens capsule. Structures related to drainage of aqueous C. its depth is maximum at the centre and gradually diminishes on the periphery where the two surfaces meet. The endothelium is absent in crypts of the iris also. Being a plano convex space. The angle of anterior chamber. The structures related to circulation of aqueous are : 1. A sieve like structure bridges the scleral sulcus and is called trabecular meshwork. In normal eye the angle is not blocked even with maximum mydriasis. dysgenesis of anterior chamber. narrow angle glaucoma. buphthalmos. Posteriorly it is bound by periphery of anterior lens capsule. The flat posterior surface is formed by a chink of anterior surface of ciliary body.A large vitreous chamber and a smaller aqueous chamber. The Trabecular mesh work. keratoconus. Structures related to circulation of aqueous. micro-cornea. subluxation of lens and aphakia. The aqueous is produced from the ciliary processes and poured in posterior chamber from there it passes through the pupil in to the anterior chamber where it circulates and leaves the anterior chamber via angle of anterior chamber. It is shallow in new born.GLAUCOMA IN CHILDREN 335 B. The aqueous chambers. . The anterior chamber is lined by endothelium except on the posterior surface formed by lens capsule. It is deep in myopia. The line where the trabecular meshwork is attached to the corneal periphery is called Schwalbe’s line which is the thickened end of Descemet’s membrane. zonules and the lens divide the eyeball in two unequal chambers . Depth of the anterior chamber is genetically determined. The meshwork extends from the scleral spur to periphery of the cornea. It forms an important landmark in gonioscopy. 1. The aqueous chambers of the eye. The posterior lip of the sulcus is sharp and is called the scleral spur. The ciliary body. The aqueous chamber is again divided into two unequal parts by the iris into : (a) A smaller posterior chamber (b) A larger anterior chamber (a) The posterior chamber is a space bounded anteriorly by the posterior surface of the iris. hypotony and intraocular tumour pushing from behind. megalo cornea. The trabecular meshwork converts the scleral sulcus into canal of irregular calibre called Schlemm’s canal. attaining adult depth after two years.

e. The tributaries may merge with the main channel at places. An imaginary line between the anterior and posterior limbal border is called mid limbal line that lies over the Schwalbe’s line. the outer wall is formed by canal of Schelemm’s. It overlies the termination of Bowman’s membrane. the surface ectoderm is converted into corneal epithelium. D. the apex of which lies at the Schwalbe’s line. The space beyond the mid limbal line upto posterior limbal line is called posterior limbal zone. The episcleral veins drain into anterior ciliary veins. is joined to episcleral and conjunctival veins.336 PEDIATRIC OPHTHALMOLOGY The trabecular meshwork is a collection of tissues that on section looks more or less like a triangle. The canal of Schlemm develops at about nine weeks6 as a vascular channel at the level of recess of angle and gradually moves anteriorly in relation to the iris. It has irregular calibre.7 This space remains very narrow as a cleft up to fifth month antenatal and then develops rapidly with the growth of the anterior segment. A wave of mesoderm invades the space between the corneal epithelium and the lens to develop stroma of the cornea. the inner wall faces the anterior chamber. The development of anterior chamber. The space between the iris mesoderm and the corneal stroma is anlage of the anterior chamber. The mid limbal line is the junction of blue corneal limbus to the scleral limbus. The meshwork as per histological picture is divided into8 Corneoscleral meshwork. The uveal meshwork is adjacent to canal of Schlemm hence called juxta canalicular meshwork. and endothelial meshwork. The presence of anterior chamber becomes evident at an early stage of embryo genesis i. On the outer side the canal-of-Schlemm. at places it breaks into tributaries and becomes multi channelled. The circles are formed by anterior limbal border. As the lens separates from the surface ectoderm.9 The drainage system becomes functional just before birth. uveal meshwork. The anterior limbal zone is narrowest at the horizontal plane of cornea and widest at superior limbus. This is a venous channel that runs all round the angle of the anterior chamber in the sclera. The canal of Schlemm. The limbus does not form a ring of uniform width all round the cornea. The angle of the anterior chamber develops two months after the canal of Schlemm. The space between the anterior limbal border and mid limbal line is called anterior limbal zone. It is an area 1 mm wide all round the cornea representing the junction of cornea on one hand. episclera and sclera on the other hand. . The limbus. iris and iris stroma. The posterior limbal border lies approximately over the scleral spur.8 The trabecular meshwork develops from vascular mesoderm originating from the margin of the optic cup. the conjunctival veins drain into palpebral and angular veins. At most of the places it is a single endothelium lined channel. From surgical point of view it has been divided into two circles and two zones in between5. endothelium of cornea. and conjunctiva. that is insertion of conjunctiva and Tenon’s capsule in the cornea. 20 mm (7 weeks)6. The limbus is most important anatomical landmark for glaucoma surgery.6 At birth the canal of Schlemm and the trabeculum both lie at the angle of the anterior chamber. The base is formed by scleral spur and ciliary body.

may be associated with glaucoma and hazy cornea and is bilateral. is most commonly known as essential iris atrophy14 and also as Cogan Reese syndrome or Chandler syndrome due to overlapping signs however some authors divide them as different entities. The condition should be differentiated from posterior polymorphic corneal dystrophy which may be seen in children.GLAUCOMA IN CHILDREN 337 Congenital anomalies of anterior chamber. . the trabecular tissue is mostly involved and cornea is either spared or effected minimally. heterochromia of iris. In trabeculodysgenesis. The disease starts in childhood and is detected during routine eye examination. Glaucoma in the form of primary congenital glaucoma is the most important clinical finding. The progress of the disease is slow. No associated systemic disorders have been reported with the condition. only 50% of eyes require antiglaucoma treatment for raised intraocular pressure that range between moderate and severe. pseudo polycoria. The iris is wider in the sector where the holes develop. shifting of pupil away from the holes towards the peripheral anterior synechea. full thickness hole in the iris. This is non-hereditary more common in females. it is unilateral mostly. iris and angle of anterior chamber in various combinations. The abnormal endothelial cells also cause corneal decompensation resulting in corneal edema. there may be nodules on the iris.18. The ICE cells migrate to the trabecular meshwork and cause mechanical obstructing resulting in glaucoma. the term iridocorneal defect is more appropriate. Majority of glaucomas in children are either congenital or developmental hence it is necessary to know the common congenital anomalies of anterior chamber where most of the lesions lie. The ICE cells are pathognomic and are supposed to be the cause of endothelial damage. Irido corneal dysgenesis13. It is but natural that congenital anomalies of anterior chamber may involve cornea. B. The ocular findings are : Peripheral anterior synechea. Roughly anomalies of anterior chamber can be divided into following groups : A. Corneal edema is independent of raised intraocular tension. Iridocorneal dysgenesis. The vision is generally poor due to corneal edema and glaucoma. Endothelial defect being a prominent feature. the cells are known as iridocorneal endothelial cells (ICE)16. The iris is atrophic at places. Mesodermal dysgenesis (anterior chamber cleavage syndromes) C. Specular microscopy reveal peculiar cells in the endothelium. The anterior chamber develops as a chink in the mesoderm between the corneal stroma and iris stroma. Common age for it to become symptomatic is third and fourth decade.17. ectropion of uvea. The exact etiology of the condition is not known15. rarely the other eye may be involved. This is a rare condition. A. Trabeculodysgenesis In the first two groups the cornea is frequently involved and glaucoma is a common feature that manifests in childhood. A thin transparent membrane is seen over the iris surface.

iris. The corneal change is represented by posterior embryotoxon. The Schwalbe’s line is prominent and shifted centrally. Riegers anomaly represents gross anomaly of the structures that arise from mesoderm. Ten to thirty percent of normal eyes show posterior embryotoxon without any other symptom21 except cosmetic blemish. dyscoria. There is hypoplasia of maxilla. lenticular changes are secondary to glaucoma. The lens is not primarily involved. B. Axenfeld anomaly 3. angle and trabecular mesh work are normal. Posterior embryotoxon—18. Mesodermal dysgenesis (Anterior chamber cleavage syndrome) Mesodermal dysgenesis consists of : 1. Severe rise of tension in infancy causes typical buphthalmos. This is the mildest form of mesodermal dysgenesis. Rise of intraocular tension may go unnoticed unless looked for specifically. Initially medical treatment is tried if it fails. hypodontia and micro-dontia. The trabecular meshwork itself is normal. Persistent hyaloid vasculature. sporadic cases are also known. Trabeculectomy is the best surgical choice. In this condition strands of peripheral iris tissue are attached to the thickened Schwalbe’s line. iris and trabeculum in various combinations. Posterior embryotoxon 2. . Riegers anomaly22. 19. The condition becomes obvious in childhood due to prominent posterior embryotoxon. dysplasia of retina. The peripheral iris is attached to the thickened Schwalbe’s line as slender or broad adhensions. Peters anomaly 1. It is formed by splitting of Descemets membrane into two leaves that contains collagenous tissue that is nearer to trabecular meshwork than cornea. Its appearance is similar to arcus juvenilis that develops at the end of the Bownan’s membrane. 3. Next alternative is Molteno valve. Glaucoma need not be present in all cases because angle remains unaffected in most of the eyes.338 PEDIATRIC OPHTHALMOLOGY Treatment is directed towards glaucoma. pseudocoria or slit like pupil. The condition does not require any treatment. The iris and pupil are also normal. partial or total absence of choroidal and retinal pigment epithelium. The trabeculectomy hole may be blocked by ICE cells. rest of the cornea. Axenfeld’s anomaly. The condition is bilateral without any predilection for sex. Glaucoma is not met with in childhood. 23. It is a feature of late adolescence or adulthood. These adhesion may be in patches or may encircle the whole of the angle. Common inheritance is autosomal.20 This anomaly is present in all forms of mesodermal dysgenesis. The thickened Schwalbe’s line projects in the AC as a ridge all around 1 mm inside the limbus or it may be fragmented. 2. Glaucoma when present is difficult to manage. There may be positive transillumination of the iris. Iris shows hypoplasia of stroma with full or partial thickness holes that may be present as deformity of the pupil in the form of corectopia. Riegers anomaly 4. Other less common findings are23A. The ocular features are bilateral but not symmetrical. It involves cornea. The iridocorneal adhesion is the cause of glaucoma that is seen in half of the cases. This condition may be considered to be more advanced form of posterior embryotoxon. Skeletal changes are sometimes seen in Riegers anomaly limited to face and teeth.

The lens soon becomes opaque. C. seen equally among boys and girls. The other postulates that there is arrest of maturation. Irido corneal dysgenesis Age Mesodermal dysgenesis Symptoms develop in second and third Symptoms develop late but can manidecade. 26. 25.GLAUCOMA IN CHILDREN 339 Once glaucoma develops the aim is to keep the tension low medically if that fails surgical intervention may be required. It is a bilateral condition. The iris is attached to the cornea all around the defect. It can be divided into following groups : (a) Type I—associated with posterior embryotoxon. The inheritance is heterogeneous but may be autosomal recessive. It is present at birth. Peter’s anomaly— 24. The meridional fibres are inserted either in the trabecular meshwork or in Schwalbe’s line. (b) Type II—No evidence of Axenfeld or Riegers anomaly. It has combined picture of Axenfelds and Riegers anomaly with central corneal defect in the form of central facet at the level of Descemets membrane. if looked for. This is eponymed as Barkan’s membrane. more common in boys. (c) Type III—The findings are similar to type II. Glaucoma is seen in fifty percent of cases. anterior chamber and iris are developmentally normal. more than one sibling may be effected. Only feature is central iridocorneal adhesion as in type one. The lens is in place. rest of the angle. The endothelium is absent in the facet. 28. present at birth. leading to stromal opacity. More in females Non hereditary Equal in both sexes Polygenic inheritance Sex Inheritance . can be discovered in childhood fest in childhood. sclerocornea. Trabeculo dysgenesis27. As the trabecular meshwork is plastered with mesodermal tissue. 4. the first believes that there is an anatomical mal-developmental. has autosomal recessive inheritance. There are two schools of thought. This condition is rarest among all mesodermal dysgenesises. There is an incomplete cleavage of uveal periphery from the corneoscleral wall. cornea plana. The most striking feature is a central corneal defect. Other anomalies include microphthalmos. Many authors have doubt about its presence. it is familial. The anterior chamber is slit like in between the central adhesion and peripheral iridocorneal synechea. In this condition the developmental anomaly lies in the trabecular meshwork. The anterior chamber is shallow. 29. Comparison between iridocorneal dysgenesis and mesodermal dysgenesis. the aqueous fails to reach the canal of Schlemm resulting into rise of intra ocular tension that causes primary infentile glaucoma or buphthalmos with its consequences. Gonioscopically the angle is wide but the trabecular meshwork is not clearly visible due to a thin membrane like structure on the surface of the trabeculum. vitreo retinal defects. The exact mechanism of the anomaly is not well understood. The anterior lens capsule is adhered to central corneal defect.

The refractive index of aqueous is 1. The composition of aqueous humour : The aqueous humour differs greatly from plasma from which it seems to be derived by the way of its low. The albumin globulin. Provide nutrition to avascular structures of the eye i. Secretion The plasma passes through three tissue layer that allow fluid with specific quality to pass through it. C. Poor Aqueous humour A. 3. The ascorbate content is very high. corectopia and ectropion of uvea. Ultra filtration 3. 4. The functions of aqueous humour are to : 1. dental and skeletal abnormality. The composition of aqueous in phakic and aphakic eyes are the same proving thereby that lens does not participate in formation of aqueous. Formation of aqueous humour. D. There are three theories of production of aqueous in the ciliary processes. The exact time it starts forming is not establishing but must be before birth because the aqueous drainage system is ready to function before birth6. They are : The wall of the capillary. The human aqueous contains only IgG and not IgA and IgM. and slightly hypertonic. strands of iris may have corneal opacity at a place touching cornea. There is hardly any change in the composition of aqueous in anterior and posterior chamber. Diffusion 2. ratio is same as in plasma. Give and maintain an optimal intraocular pressure compatible with vision. The aqueous humour is derived from the plasma in the capillary network of the ciliary process. Nil Poor May have facial. the cornea and lens. protein. 1. the stroma of the ciliary process and the . bicarbonate and glucose. B. shifted towards peripheral Distorted may have pseudo polycoria. Present. Corneal edema Pupil Systemic involvement Visual prognosis Distorted. The aqueous humour is acidic. where iris touches the cornea.33710 which is less than both the cornea and lens. anterior synechea. independent of glaucoma Glaucoma is the cause of corneal edema. This is a crystal clear fluid that fills both the anterior and posterior chamber. Act as an important optical medium.e. chlorides and lactic acid have higher concentration. sodium. but Posterior embryotoxon.340 Laterality Corneal involvement Generally unilateral PEDIATRIC OPHTHALMOLOGY Generally bilateral Mostly due to endothelial damage. 2. Remove waste products of metabolism.

The aqueous is produced in the ciliary processes that passes successively through posterior chamber. It shows a diurnal variation. It is not uniform throughout the day and night. It is hardly influenced by age or sex. E. The other is Draeger tonometer.e. Rate of outflow. Applanation tonometer The applanation tonometer is more accurate than indentation as it is not influenced by scleral rigidity. Other used applanation tonometers are : A. It seems all the processes take part in different proportion. Normal intraocular pressure ranges between 15-20 mm of Hg.Hg.20 ml/mt/mm Hg. The pneuno tonometer B. the cornea and the sclera. The normal range in adults is 2. The advantages of indentation tonometer are that it is very cheap. Indentation tonometer 2. Out of all applanation tonometers most widely used tonometer is Goldmann’s tonometer. None of the above process fulfil all the conditions to be fit into one process. The facility of outflow is measured by a non invasive method called tonography. Measurement of intraocular pressure .Intraocular pressure is the lateral pressure exerted by intraocular contents on the outer coats of the eye i. This mode of outflow that is about 85% of total outflow is called conventional outflow. In most of the eyes the tension is maximum in the morning however some persons may show an evening rise. pupil and anterior chamber where the aqueous circulates between the cornea and the iris by convection current. The outflow canbe increased by applying pressure over the globe and by topical drugs. The rate of aqueous production is called in flow of aqueous. The aqueous leaves the anterior chamber via trabecular meshwork. . The air puff non contact tonometer. The normal value is about 0. rest is drained by what is known as unconventional outflow.8 to 4. F. It shows a physiological variation of 2 to 3 mm between the highest and lowest pressure during the days and is called diurnal variation. The unconventional path comprises of uveoscleral and uveovertex pathway. To maintain intraocular pressure within normal range. The outflow of facility is expressed in ml/min/mm. It is measured in micro litres per minute (ml/min). There is no unanimity regarding presence of uveo vertex path.3 ml/mt The production is least in the night12. Intraocular pressure : A. The most important out of the three is secretion. The applanation tonometer can be mounted on a slit lamp or may be handheld (Perkins). There are two clinical methods of clinical methods of measuring it. A biphasic rise is also possible. The inflow increases following drinking of large volume of water and is decreased by ingestion of many locally and systemic acting drugs. easy to handle and gives fairly accurate reading. Presence of uevoscleral channel is well etablished. 1.GLAUCOMA IN CHILDREN 341 ciliary epithelium. there should be an equilibrium between inflow and outflow of aqueous. canal of Schlemm and is finally picked up by episcleral vessels. B. Rate of formation of aqueous humour.

This is not visible in all eyes. It looks like a translucent circular band. a non-functioning anterior part and a functioning posterior part. This represents the posterior sharp edge of the scleral sulcus. The rays arising from the angle of anterior chamber do not leave the eye. To see it. The critical angle of the rays is more than 46° hence they are reflected back. When visible it looks like a darker line in the lower trabeculum. The colour is grey to dark brown. Indirect gonioscope The direct gonio-lens has an anterior curvature that does not allow the critical angle to be reached hence the rays are refracted at goniolens air interface. exfoliations. The angle of anterior chamber has been called the cockpit of glaucoma. In case of indirect . new vessels. special optical devices called gonioscopes are employed. F. uveitis. The gonioscopic anatomy of the anterior chamber has following visible landmarks from peripheral iris to peripheral cornea. The device is called gonioscope. The trabecular meshwork. The Schlemm’s canal. This difficulty is overcome by eliminating air corneal interface and allowing the rays to pass a contact lens making the angle visible. D. The width of ciliary body is wider in myopia and narrower in hypermetropia. Common causes of increased pigmentation are acute and chronic glaucoma. The angle recess is the term used to denote dipping of iris in the ciliary body as it inserts in the ciliary body. There are two types of gonioscopes 1. Schwalbe’s line. Direct gonioscope 2. This is the termination of Descemets membrane and represents the anterior limit of the trabecular meshwork. trauma. it is very prominent and shifted anteriorly. B. However blood can also be seen in hypotony with normal episcleral pressure. The ciliary body is attached little behind the posterior attachment of trabecular meshwork. They are reflected back hence are not visible because the rays arising from angle pass through aqueous and cornea into air which has refractive index less than the former two. Scleral spur. This is the part of the sclera to which the ciliary body is inserted. It has two distinct parts. This is the part of the ciliary body visible from the insertion of the iris to the ciliary body to the insertion of the ciliary body in the scleral spur. Gonioscopy. Ciliary body.342 PEDIATRIC OPHTHALMOLOGY Gonioscopic anatomy of angle of anterior chamber. On gonioscope. The angle of anterior chamber is examined for presence of pigment. If the episcleral pressure is higher than intraocular pressure blood may be seen in Schlemm’s canal. C. A. it looks as a prominent white line in front of ciliary band and behind the trabecular meshwork. The trabecular meshwork is porous structure that stretches across the angle of anterior chamber. In case of posterior embryotoxon. E. synechia and foreign body. bridging it between the scleral spur and Schwalbe’s line. Gonioscopy is a non invasive clinical method of visualising the angle of the anterior chamber. It is not visible in normal eye with oblique illumination.

B.30 Most of the cases are symptomatic hence seek medical advice early unless it is unilateral and mild. Some of them are : A. Those due to developmental anomalies in the eyes.GLAUCOMA IN CHILDREN 343 gonioscope. A large number of gonioscopes in both the categories are available.31. C. There are many ways to classify them. Childhood Glaucoma Roughly two percent of population above forty years suffer from some type of glaucoma or other. Prevalence of childhood glaucoma is far less than adult glaucoma hence chances of missing it are more. Secondary congenital buphthalmos All the eyes under three years of age that have raised intraocular tension will go into buphthalmos irrespective of cause. The incidence rises with age and by eighty years the percentage reaches an alarming figure of five percent. Fortunately. mostly chronic symptomsless primary open angle glaucoma. (a) Anomaly in the angle only. awareness of adut prevalence of glaucoma is quite high nowadays and more and more persons are benefited by early detection and proper treatment. Developmental buphthalmos (a) Simple buphthalmos (b) Associated buphthalmos 2. There is no unanimity regarding classification of glaucoma. each with specific design and purpose. If the condition is not treated. . Those developing after two years but before puberty. (b) Multiple anomalies of anterior segment including angle. According to involvement of ocular structures 1. both the groups can have wide or narrow angle. 3. The group A is designated as congenital or developmental glaucoma. Classification of glaucoma in children. According to age of onset 1. 2. Developing in early childhood. 2. Childhood glaucoma differs from adult glaucoma in many ways. Secondary to causes not related to developmental anomalies of the eye. The reflecting angle is almost 90° to the lens air interface hence can be picked up by slit lamp or an operating microscope. Those manifesting within first two years of age. This divides congenital glaucoma’s into : 1. permanent visual damage is inevitable. The most widely accepted classification that fulfils almost all criterions is one given by Duke Elder in 196430. Loss of vision is fast and gross. The group B consists of conditions that constitute a heterogeneous group of glaucoma called secondary glaucoma. it has a contact lens and either a reflecting mirror or a prism.

rest of the eye does not suffer from any other congenital anomaly. In true congenital glaucoma rise of intraocular tension and its consequences are present in intrauterine life. 36.344 Simple buphthalmos PEDIATRIC OPHTHALMOLOGY General consideration—35. The most spectacular changes are seen in cornea. It may be present in the siblings.0 mm. at two years it should not exceed 12 mm and enlargement virtually comes to stand still. It embraces many diverse conditions. These are the cases where instead of complete shutdown of flow of aqueous. The lines produced by rupture of Descemets membrane in primary buphthalmos are known as Haab’s striations.5 mm to 11. The cornea stops enlarging after three years of age so enlargement of the globe is either arrested or minimal by three years. As the cornea stretches. The term buphthalmos is not a specific clinical entity. initially the corneal curvature is increased. The size of the cornea depends on range of intraocular tension and duration of raised intraocular tension. There may be more than one rupture in the same cornea. the Descemets membrane gives away giving rise to striated opacities that assume various forms. Stretching of the cornea. by one year it varies between 11. Primary buphthalmos is relatively uncommon disorder. The eye in primary buphthalmos : 1. The term buphthalmos will be used in the text to denote primary developmental glaucoma as a general practice rather than its accuracy.34 In advanced cases of buphthalmos diameter as high as 18 mm have been reported. At birth the diameter of the normal cornea is 10. In sixty percent cases it is bilateral which need not be symmetrical. Enlargement of cornea. 2. It is inherited as autosomal recessive trait. the rupture in the centre looks like a horizontal line while ruptures on the periphery are concentric with limbus. They start as fine branched striae in the central part. The only common feature among them is enlargement of the globe secondary to raised intraocular tension. or may manifest in first year as infantile glaucoma. On rare instances the disease may have still late presentation. Buphthalmos is seen not only in congenital glaucoma but in all conditions that cause rise of intraocular tension under three years of age. which is enlarged in all directions. . The ratio between boys to girls is 3:2. the child does not have any other extra ocular or systemic congenital anomaly. the flow is slower than normal and rise of tension late and slow. The peripheral ruptures are more common in lower part of the cornea. There is a relative flattening in the centre of cornea. Stretching of the cornea in all meridians cause thinning of the cornea that alters the curvature of the cornea.5 mm and 12. The disease may either be present at birth as true congenital glaucoma. The enhanced corneal curvature is not uniform. to be specific eye of an Ox. The disorder is caused due to trabecular dysgenesis of angle of anterior chamber32. 33. Some of the older members of the family may have early onset of chronic simple glaucoma. Very high tension cause enlargement quickly.0 mm. Corneal diameter more than 13 mm at any age and more than 12 mm at 1 year should be seen with suspicion and investigated for buphthalmos. Relatively low tension takes more time to reach the same dimension. The eye ball is enlarged and looks more or less like a bovine eye. Due to some ill understood causes it is more common in boys.

specially following forceps delivery. 3. The stretching is not limited to the cornea.GLAUCOMA IN CHILDREN 345 They may be present in other forms of congenital or developmental glaucoma as well. thinning of iris. however large pupil can predispose anterior dislocation. due to high intraocular pressure in an immature eye. Stretching is most marked in pre-equatorial area than posterior sclera. The ciliary body that is attached to the scleral spur. 8. choroids and retina do not show any significant change. 6. Anterior uvea. Due to unequal stretching of the globe instead of becoming an enlarged sphere it becomes more or less like an egg. A strained suspensory ligament may give way. As the sclera moves more peripherally. lacrimation and photophobia. relative flattening in the centre of the cornea lead to irregular astigmatism. The lines become opaque and more prominent with time. is shifted peripherally resulting in stretching of the zonules and the iris. The vitreous. Anterior chamber. 7. 5. The ruptured Descemets membrane allows aqueous to permeate into the stroma and cause clouding of corneal epithelium. 4. After sometimes the clear lens or lens with variable degree of opacity may dislocate. The stretched iris becomes thin and the pupil becomes larger than normal. 4. The vertical diameter is least effected. 2. the suspensory ligament becomes tight and flatten the lens. 5. commonest direction of dislocation is posteriorly. Over all enlargement of globe specially in anterio posterior axis gives an appearance of pseudo proptosis. It is most pronounced in anterior posterior diameter followed by horizontal diameter. whole of the globe undergoes stretching and enlargement. 9. Stretched sclera causes an increase in limbal diameter in all meridians. The Haab’s striations may become whirl like sometimes. The stretching of sclera is not uniform in all directions. Other cause of large pupil in buphthalmos of course is optic nerve change and tension related iridoplegia in late stages. Lens. 3. The stretching. A clear deep anterior chamber with large cloudy cornea in a child should be investigated for primary buphthalmos. The other effect of enlargement of outer coat of the eye is obliteration of the corneoscleral sulcus that brings about significant change in the surgical anatomy of the limbus resulting in accidental placing of sclerotomy too posterior during surgery5. The changes are mostly due to stretching of the sclera and backwards shifting of lamina cribrosa. In long standing cases there may be formation of equatorial or ciliary staphyloma. resulting in subluxation or dislocation of the lens. The effect of increased corneal curvature. Optic disc. and flattening of lens is deepening of anterior chamber. The changes in optic nerve in primary buphthalmos are prominent. development of opacity in the cornea. The Haab’s striations must be differentiated from rupture in Descemet’s membrane due to birth trauma. clouding. Such ruptures are vertical. . Flattening of lens and sometimes subluxation of lens leads to tremulousness of lens. Stretching of sclera : 1.

The angle recess is very narrow. The examiner should have a good knowledge of gonioscopic appearance of angle of anterior chamber in neonate and infants that differs from that of an adult angle. Changes in optic nerve head. D. 10. the optic nerve changes shift towards normal. . The main diagnostic feature of primary buphthalmos lies in the angle of anterior chamber. Corneal opacity. The angle of a new born has following features : 1. C. It can be done by placing the child on its back. 2. Intraocular tension. the eyes are moderately myopic. due to : 1. Nystagmus. 360° of an angle is visible at a given time. may even require general anaesthesia. 11. This myopia is partially neutralised due to flattening of central cornea. 3. Neonate need not be given general anaesthesia to do direct gonioscopy but a child above three months may not co-operate without sedation. The gonioscopic examination in case of primary buphthalmos is done by direct gonioscope of Koeppe or its modification. Error of refraction.346 PEDIATRIC OPHTHALMOLOGY The optic nerve changes are reversible. 6. 2. E. 4.33. The peripheral iris and blood vessels produce a scalloped appearance.37.. However after three years when anterior segment enlargement stops. Intraocular tension is generally above 40 mm Hg. the epithelium should be scrapped either mechanically by the blunt side of a cataract knife or chemically by rubbing the cornea with swab soaked in alcohol32. Main diagnostic features in primary buphthalmos : A. 5. Myopia is invariably associated with irregular astigmatism. 38 4. The angle should be examined by a suitable gonioscope in every eye that has large. B. the optic nerve changes become accentuated and progress. Vision in an eye with primary buphthalmos is always subnormal. 3. Both the eyes can be examined simultaneously by using two gonioscopes and the changes compared in two eyes. It may be as high as 80 mm Hg. If the intraocular tension is brought low and kept low. cloudy cornea with deep and clear anterior chamber. 4. 12. Instillation of 5% hyper tonic sodium chloride drop few times may also clear epithelial haze. flattening of lens and posterior shift of lens. 3. An additional advantage of Koeppe’s gonioscopy is that direct ophthalmoscopy can be done over the gonioscope because it gives extra magnification. The trabecular sheets are translucent. The anterior part of the ciliary body and its insertion in the scleral spur is seen clearly. Error of refraction. The advantages of direct gonioscopy are : 1. Due to increased axial length. 2. If the cornea is hazy due to epithelial edema. when the child presents for the first time. Amblyopia. Vision. 5. It can be done either by handheld microscope with suitable illumination or operating microscope. Lenticular changes.

it may vary between 40 mm to 80 mm Hg depending upon severity of trabecular block. C. The child may open the eyes at night due to reduced intensity of light and then it is realised that the child has poor vision. Gonioscopic findings34. require thorough examination of a new born. 4. in first cousins. The peripheral iris is inserted in the line of Schwalbe. Similarly children and grand children of persons who had early onset of wide angle glaucoma should also be suspected to have primary buphthalmos and investigated. The angle is wide open. Some of the anaesthetic agents themselves alter intraocular tension. A positive history in siblings. The other eye should also be examined even when it is apparently normal. Unilateral cases draw early attention of the mother to relatively prominent eye. chances of missing the condition in very high specially in bilateral cases with mild to moderate manifestation.39. Perkin’s handheld applanation tonometer or tonopen. keeps both the eye closed and moves the face away from light. the anterior chamber is of normal depth before corneal stretching and enlargement ensue. 3. obstetrician and attending nurse. Unless looked for carefully.35. 2.40 Tension recorded in a struggling child is not good enough. while ketamine elevates the tension.36 : 1. D. 5. other symptoms that point towards possibility of buphthalmos are: lacrimation. it need not be symmetrical in two eyes. The scleral spur is not well visible. provided there is enough awareness among the attending neonatologist. B. duration and therapeutic measures undertaken. Measurement of Intraocular tension—Intraocular tension is primary buphthalmos is always raised. Allowance should be made for general anaesthesia induced rise of tension.GLAUCOMA IN CHILDREN 347 F. G. On the inner surface of trabecular meshwork a translucent membrane called Barkan’s membrane may be present. and large cloudy cornea. Diagnosis of full blown case of primary buphthalmos is not difficult. Diagnosis A. Gonioscopic findings are not uniformly spread all over the angle. Besides prominent eye/eyes. The tension is measured either by Schiotz’s tonometer. 6. In children over three years tension should be recorded under general anaesthesia under a competent anaesthetist well versed in paediatric anaesthesia.34 7. with rise of tension and passage of time the anterior chamber deepens. . There is no angle recess. 8. halothene and barbiturates lower tension. In primary buphthalmos. Digital recording of tension should always be avoided. The child with buphthalmos has poor appetite and the child is withdrawn. The child with bilateral disease prefers to be in dark place. the symptoms become more pronounced. As the child grows.38. In unilateral cases the child keeps the effected eye closed. blepharospasm. photophobia.

The discharge is watery. gonioscopy and evaluation of optic nerve head. Regurgitation test is negative. Management of primary buphthalmos. E. cornea AC and pupil are within normal range. AC and pupil are normal. retinopathy of prematurity. The diagnosis is confirmed by measuring intraocular tension. Endothelial dystrophy 2.348 PEDIATRIC OPHTHALMOLOGY E. C. Prominent eyeball. Mucopoly saccharoidosis 3. corneal diameter. Interstitial keratitis comes into differential diagnosis only if a child with lacrimation and photophobia present after three years of age. Other causes of secondary and associated glaucoma in children should be excluded. Differential diagnosis of primary buphthalmos consists of all cases with A. has mucoid or mucopurulent discharge may have positive regurgitation test. Photophobia and blepharospasm C. Ophthalmia neonatorum develops within first three to four days. The cornea in interstitial keratitis has uniform haze due to deep vascularisation. Watering should not be mistaken as congenital nasolacrimal duct obstruction. CT 4. 2. The cornea is bright. True proptosis due to a retro bulbar mass and craniocynostosis and congenital myopia. The congenital nasolacrimal duct obstruction generally manifests after three weeks of birth. Mucolipidosis D. Prominent eyeball in a neonate. the lids are moderately edematous. Ultrasonography 2. Cloudy cornea : Cloudy cornea may be due to 1. 1. It is invariably bilateral. anterior chamber depth is normal and pupil of normal size and reaction. B. Clinically and by specialised investigation like : 1. The conditions that require special attention are retinoblastoma. ophthalmia neonatorum or corneal abrasion. A.R.42.I. Photophobia and blepharospasm are generally due to corneal abrasion. Medical treatment is employed for temporary reduction of . 3. B. The definitive treatment of primary congenital glaucoma is surgery. M. The abrasion stains with fluorescein. persistent primary hyper plastic vitreous41. discharge is purulent. Cloudy cornea D. Corneal abrasion are mostly due to birth trauma. causes of prominent eyeball in neonate may be : 1. otherwise cornea. Watering of the eye. neonatal keratitis and anterior uveitis. X-ray skull and orbit 3.

42 They are : (a) (i) Goniotomy through operation gonioscope. Medical B. Ancillary treatment consists of management of 1. This is a less accurate method of opening the trabecular meshwork.GLAUCOMA IN CHILDREN 349 intraocular pressure till the facility of surgery becomes available. The pupil is constricted by local pilocarpine. It is less effective after three years. The goniotomy knife is passed almost . (b) (i) External trabeculotomy (ii) Trabeculectomy (iii) Trabeculotomy combined with trabeculectomy. In this procedure a fine specially designed goniotome is introduced in the anterior chamber via a peripheral corneal stab incision under operating gonioscope and operating microscope. 35. (c) Draining devices (d) (i) Laser (ii) Cryo (iii) Ultrasound (a) (i) Goniotomy. 4. Prevention and management of amblyopia. 37. The success rate falls with increasing age. This is done in presence of cloudy cornea. some surgeons prefer to do bilateral surgery. Surgery consists of various microsurgical procedures. Some eyes may require repeat surgery. (ii) Direct goniotomy. (ii) Direct goniotomy in cloudy cornea. 41. Prior to surgery. Specific 1. The superficial layers of one-fourth circumference of trabecular meshwork are cut just below the Schwalbe’s line. Rehabilitation. 1. 3. sometime medical treatment may have to be re-introduced after surgery to control residual glaucoma. 2. (iii) Gonio puncture. Treatment of primary buphthalmos is divided into : A. Low vision aid.34. intraocular pressure is lowered to a safe limit by oral acetazolamide 15 mg/kg in divided doses. It can be done between one month to two years with excellent result. It is a very safe procedure in competent hands. Associated errors of refraction. Surgery 2. Goniotomy is the preferred surgical procedure for all buphthalmic eyes where surgery is indicated. Laser 3. It is claimed that only complication during goniotomy is anaesthesia induced. This is a highly skilled microsurgical procedure done through a suitable operating gonioscope under general anaesthesia. 40. This type of goniotomy is not possible in cloudy cornea.

loss of vitreous. The difficulty arises due to changed anatomy of the surgical limbus. One end of which is introduced in the AC and the other end is anchored under the subconjunctival tissue. (b) (i) External trabeculotomy is a procedure where the conjunctiva is incised and reflected to expose the sclera near the limbus. have one-way flow from anterior chamber to sub-Tenon’s space.43 C. The advantage of the method is that it can be done in presence of hazy cornea. the first two are of historical importance and are no more in use the devices are : (a) Seton. These devices are associated with high risk of intraoperative and post operative complications. They are generally used in advanced glaucoma or where previous surgeries have failed. (c) The valves. This is also uncontrolled. once the tension falls below a critical level the flow stops. cyclodialysis which were done in past have been given up in favour of trabeculectomy. trabeculectomy with use of anti metabolities is said to give better results. Thermal sclerotomy. Common complications of these devices are : — Perforation of thin sclera. The superficial meshwork is cut as in classical goniotomy. The canal is divided. the lens may be injured during the procedure. This is a tubular structure one end of which rests in the anterior chamber.D. (ii) Trabeculectomy. This is a solid rod. it is difficult to identify the canal of Schlemm. They are used only in eyes where other surgical procedures have failed. A sclerotomy wound may be inadvertently placed too posteriorly. — Injury to the ciliary body . The procedure is less effective than goniotomy.)44. operating gonioscope or trabeculotome. This method is less effective and produces more complications. Trabeculectomy is more successful in moderately advanced cases does not require special device like goniotome. Once whole length of the trabeculotome has gone inside. Artificial drainage device44 or aqueous drainage device (A. It is not suitable for advanced case where due to altered surgical anatomy of the limbus. A vertical scletotomy is done to reach the canal of Schlemm. the two cut ends are identified. The fluid passes through the lumen passively. iris inclusion surgery. (c) Valves. These are miniature artificial drainage devices that45 are transplanted under the Tenon’s capsule to bypass the trabecular meshwork and canal of Schlemm. (b) Shunts. The flow is regulated. the trabeculotome is swept in the anterior chamber ripping open a passage in the trabecular meshwork. resulting in injury to the ciliary body. A specially designed trabeculotome is passed in the canal. There are three types of glaucoma devices. These are tubular structures similar to shunts. The drainage is uncontrolled. (b) Shunt. corneo scleral trephine.D. The aqueous drains by the side of rod by capillary action. These devices are seldom used as primary procedure. (a) Seton.350 PEDIATRIC OPHTHALMOLOGY blindly across the anterior chamber to reach the trabecular meshwork on the opposite side.

In uniocular cases amblyopia sets in early and if not managed becomes permanent. mostly astigmatism. Correction of existing error of refraction. Acetazolamide and IV Mannitol is seldom required. Laser is also used to make extra iridotomies if need arises. Amblyopia squint and nystagmus are common both in treated as well as untreated eyes. B.GLAUCOMA IN CHILDREN 351 — Vitreous loss — Endothelial damage during insertion — Intra operative hyphaema — Expulsive haemorrhages — Persistent shallow AC — Corneal decompensation — Tube touching the iris — Moderate to severe uveitis — Closure of the lumen — Extrusion of the device Laser in buphthalmos. irregular astigmatism. In all cases of buphthalmos occult retinoblastoma should be suspected and excluded. Medical treatment. 1. It is claimed that pilocarpine may cause paradoxical rise of tension due to collapse of trabecular meshwork because of high attachment of uvea in posterior meshwork42. Medical treatment have limited role in management of primary buphthalmos. optic nerve changes. (a). Argon or Nd YAG is also used to open the clogged end of aqueous drainage device. vision with best corrected glasses is rarely normal due to corneal opacity. There may be a change in refractive power following surgery. The child must be examined with an indirect ophthalmoscope and if needed an ultrasound may be ordered. (b). Miotics. The buphthalmic eyes are generally myopic with astigmatism. Utilising salvageable vision by low vision aids. the drug used widely in adult wide angle glaucoma is only used to constrict the pupil before glaucoma surgery or iridotomy. Either transscleral or through pupil46. Persistent lacrimation and photophobia may be seen even when tension has been brought under control. alpha agonist should be used in consultation with paediatrician. 2. otherwise laser is mostly used as cyclo destructive procedure. Either the glaucoma is mistaken as simple epiphora or conjunctivitis or these conditions are mixed up as glaucoma and the child exposed to unnecessary investigation. residual glaucoma and amblyopia. . Q switched Nd-YAG has been used to produce laser goniotomy. The later is a better option. This is divided into two main parts : 1. Transscleral Diode or Nd-YAG is used to ablate ciliary body as cyclo destructive procedure. First and foremost complication is missed diagnosis. Complication in buphthalmic eyes : A. C. Beta blockers. Ancilliary treatment.

B. Congenital anomalies of posterior segment (a) Persistent primary hyperplastic vitreous (b) Retrolental fibroplasia 6. Mesodermal dysgenesis (see page 338) 3. Congenital uveitis 2. Some of the causes are : 1. Large equatorial staphylomas are seen in untreated or poorly managed cases. A heterogeneous group of congenital anomalies of eye with or without systemic manifestation produce glaucoma in children. Miscellaneous (a) Lowe’s syndrome (b) Nevus of ota (c) Mucopolysaccharidosis . Associated causes of congenital glaucoma. In contrast to primary congenital glaucoma where the pathology lies in the trabecular meshwork with normal cornea and iris. The buphthalmic eyes generally have high axial myopia hence they are prone for retinal detachment. Congenital anomalies of globe (a) Microphthalmos (b) Nanophthalmos 5. The other group consists of few primary conditions that cause secondary glaucoma. The associate glaucoma’s may or may not have abnormality in the trabecular meshwork. They are : A. Phacomatosis (a) Struge Weber syndrome (b) Neurofibromatosis 7. Congenital keratitis 3. E. Iridocorneal dysgenesis (see page 337) 2.352 PEDIATRIC OPHTHALMOLOGY D. warranting removal of eye. The associated causes of congenital glaucoma is long. Congenital anomalies of uvea : (a) Aniridia (b) Congenital ectropion of uvea 4. Intrauterine trauma 4. F. Associated and secondary congenital glaucomas32. Neoplasm A. These are : 1. Subluxation and dislocation of clear or cataracts lens are occasionally seen. The thin sclera is prone for rupture that may lead to bleeding.

46 Glaucoma in older children. Management of glaucoma is difficult. The systemic involvement consists of mental retardation. 2. iris atrophy. The pigments obscure scleral spur and ciliary band. Late manifestation of congenital glaucoma 2. unilateral condition. microphthalmos. in this condition iris pigments are present in anterior stroma. Glaucoma is caused due to maldevelopment of angle of anterior chamber. glaucoma in Lowe’s syndrome and nevus of ota. Nevus of ota. may be hereditary or non-hereditary. Trauma 3. This is non progrgessive. Glaucoma develops in childhood or during puberty. Juvenile glaucomas form a large group of conditions of diverse cause that results in rise of intraocular tension. Tumour related . The condition may be associated with neurofibromatosis. Glaucoma in children fall in following broad groups : A. B. The pupil is circular. The ocular manifestation are bilateral congenital cataract. and conjunctiva. In normal eyes there is no pigment in the stroma. True congenital glaucoma. Congenital ectropion of uvea47. Juvenile glaucoma1 seen over three years of age. These are either : 1. Besides melanosis bulbae a small percent of cases develop glaucoma on the side of malanosis. Secondary glaucoma (a) Wide angle (b) Narrow angle Common causes of junevile glaucoma are : 1. C. Uveitis 4. Steroid induced 5. Cataract and glaucoma are independent of each other. In this condition. there is deposition of melanocytes in the skin. 3. the trabeculum may be fragmented. Post surgical status 6. Early onset of adult chronic simple glaucoma. diagnosed between birth and first three months. Glaucoma in Lowe’s syndrome. Female carriers have cortical lenticular opacity. renal ricket.GLAUCOMA IN CHILDREN 353 Most of the common conditions have been discussed in various chapters except congenital ectropion of uvea. aminoaciduria and hypotony48. This is generally an isolated congenital anomaly or may be associated with nevus of skin on the distribution of second and third division of the trigeminal. Phacogenic 7.30. glaucoma. Glaucoma is bilateral in one third of cases. Associated congenital glaucoma29. miosis. goniotomy may help. Lowe’s syndrome is a systemic disorder that involves not only eyes but multiple organs. Infentile glaucoma1 is between three months to three years. react well to light and accommodation.

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

lensectomy in congenital and traumatic cataracts. If medical treatment fails or the child does not comply with the prescribed regime. Glaucoma following ocular surgery. Associated infection. Uveitis is treated by steroids and cycloplegic. Both types of surgical wounds i. inspite of best surgical management of penetrating injury. Primary repair of cornea. An associated retained intraocular foreign body may contribute to rise of tension separately or with uveitis. removal of foreign body. inflammation may also worsen the condition. scleral and corenoscleral wound with removal of incarcerated uvea. Laser may be used to do an iridotomy or iridoplasty. carbonic anhydrase inhibitors or systemic carbonic anhydrous inhibitors for short period. There are two methods of repairing the penetration wound : 1. Miotics and latanoprost are contra indicated. scleral buckling and reformation of anterior chamber with or without intraocular implant after the primary wound has healed well.GLAUCOMA IN CHILDREN 355 chamber or block of angle. Repairing of the corneal. the eye may be put on medical therapy of glaucoma by way of local beta blockers. tissue disruption that is already present. lens and vitreous. From the conditions mentioned above it is clear that surgery on lens is a common cause of post operative glaucoma. minimise uveitis by use of local and systemic steroids. It can be seen in all ages but more common in children. aspiration. Angle closure may worsen pupillary block and vice versa. Laser trabeculoplasty generally fails. alpha agonist. best alternative is to undertake any of standard anti-glaucoma surgeries. Best management of glaucoma in penetrating injury is to minimise tissue disruption rectify. antibiotics and cycloplegics. even a surgery of short duration and minimal handling like needling. Common intraocular surgeries that are associated with glaucoma are : — Needling. In some cases an aqueous drainage device may be the last alternative. This is followed by well planned extensive surgery that include lensectomy. The next important step is to reform the anterior chamber. this prevents closure of trabecular meshwork. — Pupillary block in aphakia and pseudoaphakia — Repair of penetrating injury — Vitrectomy — Penetrating keratoplasty Closed globe surgeries that cause post operative rise of tension are : Scleral bucking NdYAG posterior capsulotomy Glaucoma following lens surgery. 2. open globe and close globe wounds may lead to secondary glaucoma. scleral wound and excision of incarcerated uvea. various grades of vitrectomies. A non incarcerated lens may too have to be removed by lensectomy along with anterior vitrectomy. Any retained intraocular foreign body is also removed while doing extensive vitrectomy. Management of glaucoma in penetrating injury.e. aspiration is fraught with rise of tension51 that may be seen between 6% and 24% . Once the glaucoma sets-in.

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

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357

Uveitis glaucoma hyphaema (UGH). This condition was very common in era of anterior chamber IOL. The AC IOL caused, repeated trauma to the iris that resulted in acute non granulomatous uveitis after months of surgery. It presented as haemorrhagic uveitis with rise of tension. Many factors like 10L itself, ischamia of iris, pressure necrosis of iris, chaffing of iris trigger