Pediatric Ophthamology 2005

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Pediatric Ophthalmology
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NEW AGE INTERNATIONAL (P) LIMITED, PUBLISHES
New Delhi Bangalore Chennai Cochin Guwahati Hyderabad
Jalandhar Kolkata Lucknow Mumbai Ranchi
Pediatric Ophthalmology
Dr. P.K. Mukherjee
MS
Former Professor of Upgraded Department of Ophthamology,
Pt. JNM Medial College, Raipur
Copyright 2005, New Age International (P) Ltd., Publishers
Published by New Age International (P) Ltd., Publishers
All rights reserved.
No part of this ebook may be reproduced in any form, by photostat, microfilm,
xerography, or any other means, or incorporated into any information retrieval
system, electronic or mechanical, without the written permission of the publisher.
All inquiries should be emailed to rights@newagepublishers.com
ISBN : 978-81-224-2310-5
PUBLISHING FOR ONE WORLD
NEW AGE INTERNATIONAL (P) LIMITED, PUBLISHERS
4835/24, Ansari Road, Daryaganj, New Delhi - 110002
Visit us at www.newagepublishers.com
This 8ook is Dedicofed fo
Those chiIdren
whose sighf couId hove been soved
by
fimeIy infervenfion
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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 de-
veloped countries pediatric opthalmology has reached a status of super-speciality. Pediatric
opthalmology is still being managed by general opthalmologist in developing countries. To over-
come enormous volume of ocular morbidity and blindness is children, WHO has included child-
hood 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 em-
bryology of each system, their disease and disorders.
The medical management has been discussed briefly. Emphasis has been given to pre-
ventive aspects. Surgical procedures have only been outlined. The surgical steps and their de-
tails have not been included, for which the students should refer to standard books on ocular
surgery.
The book is meant as a handbook for postgraduates, teachers and reference book for
undergraduates. The book has been written in English that is understood by English speaking
students of third world.
Some portions in the book on cursory glance may seem to be repetitive. This has been
done in intentionally to emphasize the importance of the topics referred to.
Dr. P.K. Mukherjee
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Acknowledgement
I am thankful to a large number of my colleagues and friends who initiated me in writing this
book. The real inspiration behind this book remains my wife Protima, who prodded me to keep
the schedule. My daughters Protibha and Preeti joined their mother in cajoling me. I am thankful
to my daughters for persuading thier spouses Satyadeep and Abir respectively to get involved
in the project and spend endless hours in typing, arranging and rearranging the text in initial
stages.
Padma Shree Dr. A.T. Dabke, Prof. and Head of Department of Pediatrics, Dean Pt.
J.N.M. Medical College Raipur, gave me free access to his personal library. He was always
available to solve any of the pediatric problems that seemed to dodge me. I extend my heartfelt
thanks to him.
My sincere thanks are due to members of Upgraded Department of Ophthalmology, Pt.
J.N.M. Medical College, Raipur, that includes Dr. S.L. Adile, Prof. and H.O.D., Prof. A.K.
Chandrakar, Dr. M.L. Garg, Associate Professor, Dr. Nidhi Pandey, Asst. Professor of Ophthal-
mology, 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 sugges-
tion. 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 PRNCPLE8
1-8
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 gradu-
ally 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 poste-
rior fundus including optic disc is less frequent than coloboma of anterior uvea. Deficiency in
mid fundus is least common.
DEVELOPMENT OF NDVDUAL 8TRUCTURE8
Development of the Lens
The lens is solely ectodermal in origin. The development of the lens begins at an early
stage of 4 mm.
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The development of lens has two distinct stages :
1. A short period when the lens vesicle develops between 4.5 mm to 10 mm.
2. A longer period of development of lens fibers that continues even after the birth of
the child. The lens fibers are laid down in two phases of primary and secondary
fibers.
The cells at the spot where the neuro ectoderm has come in contact with the surface
ectoderm starts proliferating to form lens plate. The cells on each side of the lens plate in-
crease 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 base-
ment 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 transpar-
ency 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 embryo-
nal, foetal, infantile, (formed during last weeks of foetal life up to puberty) adult and cor-
tex. 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 pri-
mary 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 re-
maining part of it gives rise to corneal epithelium. Rest of the cornea is mesodermal. The
mesoderm encroaches in between the surface ectoderm and the lens.
This mesoderm is divided into two distinct parts :
1. Anterior mesoderm that gives rise to stroma and endothelium of the cornea.
2. Posterior mesoderm that gives rise to iris stroma. In the space between the two
layers of mesoderm develops the anterior chamber. The Descemet membrane develops from
endothelium while Bownans membrane develops due to condensation of stroma under the
corneal epithelium.
The Development of Sclera
1,2,4
Sclera is fully mesodermal in origin. It develops due to condensation of paraxial meso-
derm 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 Development of Extra Ocular Muscles
13
All voluntary muscles develop from paramedian mesoderm. The extra ocular muscles
are no exception. The extra ocular muscles develop from a common mesodermal mass that is
separated as three different groups. From each will develop muscles that are supplied by dif-
ferent cranial nerves i.e. third, fourth and sixth. The nerves grow from brain towards indi-
vidual muscle mass marked for each nerve. The individual extra ocular muscle starts differen-
tiating at about 9mm stage and can be identified as separate muscle by 20mm stage except the
levator. The levator develops from the dorsomedial aspect of the superior rectus. The nerve
supply to levator passes through the superior rectus mass hence simultaneous congenital un-
der action of both are a common feature.
The Uvea
The uvea develops from neuroectoderm and mesoderm. The Iris and ciliary body
develop partly from neuroectoderm (that is part of optic cup), and partly from mesoderm.
The structures that originate from neuroectoderm are pigment epithelium of iris sphinter
and dilator pupilae of iris, epithelium of ciliary body. Melanocytes also arise from ectoderm.
Bruchs membrane is partly neuro ectodermal and partly mesodermal.
The mesoderm gives rise to strom of iris, ciliary muscle, connective tissue and blood
vessels.
1. The Iris. The part of the iris that develops from mesoderm is its stroma. It develops
between the lens and surface ectoderm. The anterior tip of the optic cup develops over the
scaffolding of mesodermal stroma. The tip of the cup develops into two layered pigment epi-
thelium of the iris. The anterior layer is the continuation of outer layer of optic cup while the
posterior layer is continuation of inner layer of optic cup.
The pupillary membrane is a transient structure that lateron atrophies and disap-
pears. Initially this stretches across the lip of the optic cup. It is formed by the mesodermal
tissue surrounding the margin of the optic cup and tunica vasculosa lentis. Later the pupillary
membrane separates from tunica vesculosa lentis. The peripheral part of the pupillary mem-
brane gets vascularised. The central part of the pupillary membrane is eventually completely
absorbed forming the pupil. Incomplete absorption of the pupillary membrane is called
persistent pupillary membrane.
The iris is fully pigmented after birth. If there is less of pigment the iris takes a blue
colour. Some times fine vessels are visible on the iris of a new born.
2. The Ciliary body. The ciliary muscle develops from paraaxial mesoderm. The ciliary
muscles become evident by third month and is gradually differentiated into longitudinal,
oblique and circular muscles. The ciliary epithelium is neuro ectodermal in origin. It
develops from the lips of the optic cup. The outer layer is pigmented while the inner layer is
non pigmented the ciliary epithelium give rise to 70-75 ciliary processes.
3. The Choroid. Structure wise choroid differs from iris and ciliary body. It does not
have an epithelium like the former two. It lacks stroma as in iris or musculature of ciliary
body.
It is mostly mesodermal except the Bruchs membrane that has both ectodermal and
mesodermal origin. The choroid is mostly vascular with connective tissue inbetween. Its
vasculature develops in three stages. Earliest is development of chriocapillaries. In second
phase larger tributaries of venae verticosae develops by the third month of gestation.. The
third phase consists of development of vessels from short ciliary vessels.
The Anterior Chamber
The anterior chamber develops as a cleavage in the paraxial mesoderm that lies be-
tween the corneal endothelium and iris stroma. Its presence is noticed at 20mm stage.
Anterior chamber starts as a chink in the centre of this mesoderm and spreads to the
periphery. Initially it is very shallow but at birth it is fully formed. The angle of anterior
chamber is not formed before 6 month i.e. two months after the canal of Schlemm is visible.
Schlemms canal starts as venous channel derived from various plexus at the margin of the
optic cup.
The Retina
The retina develops from both the layers of optic cup. The inner layer gives rise to nine
layers of sensory retina while the outer layer that remains single layered gives rise to pig-
ment epithelium. The space between the two layers is very large in early stage of develop-
ment that gradually shrinks to a potential space at the time of complete development of retina.
By the time the fetal fissure begins to close the inner layer starts to thicken to form various
layers of sensory retina. The pigment epithelium starts acquiring pigment granules at this
stage. By seventh month of gestation all the layers of retina are well developed except in
macula.
The Macula
Development of the macula differs from rest of the retina. Initially there is fast develop-
ment in area of macula upto third month of life, then there is a slowing of growth while rest of
retina grows in usual pace. This state of retardation persists upto eighth month then it start
growing in the same manner as rest of the retina. Thus its development is not complete by
ninth month. To attain full development macula has to wait up to fourth month post natal. At
sixth month of foetal life it is thicker than rest of the retina. By seventh - eighth months it
starts thinning. The thinning is due to spreading out of ganglion cells from the central part i.e.
the fovea which at birth has only one layer of ganglion cells left. The outer nuclear layer is also
single layered.
The Optic Nerve
The optic stalk that joins the fore brain and the interior of the optic vesicle is the future
optic nerve. The foetal fissure that develops at the under side of the optic vesicle extends in to
the stalk also. The axons of the retina and blood vessels pass through this opening. By third
month the mesoderm that forms the connective tissue of optic nerve along with minute capil-
laries enter the optic nerve. The outer covering of the nerve i.e. dura, arachnoid and pia
develop between third and seventh months, the lamina cribrosa develops late. Myelination of
optic nerve starts at about seventh month of foetal life towards cephalicend, extending to-
wards the lamina and stops short at lamina. In few cases the myelination may extend on the
surface of the retina as medulated nerve fibers.
Intra Ocular Vasculature
Intra ocular vasculature can be divided into three components :
1. The hyaloid system
2. The uveal system
3. The retinal circulation
All of these arise from paraxial mesoderm that get into the eye through the fetal
fissure. Besides supplying nutrient to the eye they play an important part in development of
the eye itself.
1. The hyaloid system comprises of :
(a) Vessels of pupillary membrane
(b) Tunica vasculosa lentis
(c) Hyloid artery
(a) The vessels of pupillary membranes are formed by small buds from annualar
vessels. The central part of the pupillary membrane is almost devoid of any vessel. It forms the
pupil as it atrophies. The peripheral thick part persists as part of iris stroma.
(b) The tunica vasculosa lentis. It has three parts i.e. the posterior, lateral and ante-
rior. It completely engulfs the developing lens and supplies blood to it. It also forms a connect-
ing channel between intra and extra ocular circulation.
(c) The hyaloid artery is a branch of dorsal ophthalmic artery. It supplies blood to the
developing lens. This passes through the foetal fissure and courses through middle a develop-
ing vitreous, stretching from posterior part of the optic vesicle to posterior pole of the lens.
Before reaching the lens it divides into smaller branches and called Vasa hyaloidea propria
that anastomose with each other in the primary vitreous and form the posterior part of tunica
vasculosa lentis.
Atrophy of the hyaloid system. Once the hyaloid system has reached its peak of
development and finishes the main task of blood supply to the developing lens it starts to
atrophy and disappears completely. Exact stimulus for disappearance of hyaloid system is not
well under stood. The posterior part of the tunica vasculosa lentis is first to start to atrophy,
followed by lateral. The anterior tunica is last to atrophy without leaving any trace. In hyaloid
artery the anterior branches start disappearing first. Sometimes leaving a small part attached
to the posterior pole of the lens as Mittendrof s spot. A small part may remain unabsorbed
in the Cloquets canal or may remain attached to the optic nerve head as Bergmeisters
papillae.
2. The Uveal Circulation. The uveal circulation becomes evident at very early stage
as vessels round the posterior part of the optic vesicle.
The primitive internal carotid gives off two branches that are precursors of long poste-
rior ciliary arteries i.e. dorsal and ventral ophthalmic artery, the former develops to become
temporal long posterior ciliary artery while the latter is destined to become medial long
posterior ciliary artery. The dorsal ophthalmic artery also gives rise to short posterior
ciliary arteries. The anterior uvea is supplied by muscular branches of ophthalmic artery
which form the anterior ciliary system.
3. The retinal circulation. Exact mode of development of retinal circulation is contro-
versial. Most widely accepted hypothesis is that central retinal artery buds from the posterior
end of hyloid artery at the level of optic cup but does not atrophy like rest of the hyloid system
and then branches off as temporal and nasal branches that divide into superior and inferior
branches.
Development of Lid, Conjunctiva and Lacrimal System
1. The Lid. The lid has dual origin, the main mass of the lid is formed by mesoderm
while the skin and conjunctiva develop from surface ectoderm. At about 18mm stage, the
mesoderm condenses outside the optic vesicle in the form of lid fold. The upper lid develops
from frontonasal process in two parts i.e. smaller medial and larger lateral part, while the
lower lid develops from maxillary process.
Fault in fusion of medial and lateral frontonasal mesoderm results in coloboma of
upper lid that may very from a simple notch at the lid margin to extensive loss of tissue. The
upper and lower lids are fused between third and sixth month then they separate. Separation
is completed well before birth. Failure to separate will result in ankyloblepharon of various
degrees.
2. The Conjunctiva. The conjunctiva, cilia, meibomian gland, lacrimal gland and ac-
cessory lacrimal glands also develops from surface ectoderm and are associated with develop-
ment of the lid.
3. Development of lacrimal system
7
.
(a) The lacrimal gland. Lacrimal gland develops from the superior temporal conjunc-
tival fornix, as solid cords of ectodermal cells 8-10 in number which are surrounded by meso-
derm that develop into connective tissue of the gland. The ectodermal cords gradually canalise
and ramify.
(b) The Lacrimal Passage. The lacrimal passage develops in the groove between the
maxillary proces and lateral nasal process, the groove or cleft is converted in to a tube. The
surface ectoderm gets buried in the mesoderm and progress upwards, at the same time a
similar cord of ectoderm develops from the nasal cavity. The upper end will form the two
canaliculi, puncta and sac. Subsequently the two cords i.e. the upper and nasal will join
each other to form a continuous structure. During third month the central cells of the cord
begin to disintegrate and form the nasolacrimal duct. The disintegration is patchy in nature
but ultimately becomes continuous. The upper part thickens and dilates to form the lacrimal
sac.
Development of the Orbit
The walls of the orbit develop from the mesoderm around the eye. The floor and lateral
walls develop from maxillary mesoderm. The medial wall develops from the lateral nasal proc-
ess. The roof differs little from these walls, it develops from the mesoderm covering the forebrain.
By 14th week the boundaries of orbit are well differentiated. Up to 28th week the orbital
margin is at the level of developing equator of the globe, there after it grows rapidly and the
rim occupies more anterior position than the globe. The size of orbit is so small at this stage
that its walls are snug with the globe. At birth the orbital rim is almost circular, its diameter
is relatively large as compared to face.
CONGENTAL ANOMALE8 OF THE GLOBE
8,9,10
The Congenital anomalies of globe are caused due to faulty embryogeneses before the
closure of the embryonic fissure. They can happen :
A. During formation and development of primary optic vesicle i.e. Cyclopia,
anophthalmos, and extreme degree of microphthalmos.
B. During development of optic cup i.e. Congenital cystic eye ball, colobomatous
cyst and typical coloboma of eye. (Uvea, retina disc.)
C. Maldevelopment of formed eye is Microphthalmos (True nanophthalmos)
1. Cyclopia. This is an extremely rare congenial anomaly that has either a single mid
line eye or two developing eyes with deformity of the forebrain and multiple anomalies of mid
line. There is either one orbit or two maldeveloped fused orbits. There is one palpebral fissure
with two rudimentary lids, and lacrimal apparatus. The IPA is wide, the lids do not close, the
rudimentary cornea and conjunctiva are exposed. These children have neonatal death.
2. Anophthalmos. Strictly speaking term anophthalmos is reserved for a condition
where no ocular tissue is present in the orbit due to non formation of optic vesicle. However in
clinical practice eye with minimal ocular tissues are also called clinical anophthalmos. Gener-
ally these eyes do not have any evidence of formed globe. They are also known as extreme
microphthalmos. Anophthalmos differs from enophthalmos. In enophthalmos a fully devel-
oped globe is pushed back in the orbit due to secondary causes.
3. Microphthalmos. These include all eyes that have size less than normal eye due to
congenital cause. They can be unilateral or bilateral. In bilateral cases both eyes are smaller
than normal but not of equal size. The defects in one eye need not be the same in other eye. In
unilateral cases the other eye may be normal and remain so for rest of the life.
Microphthalmos has been divided in following groups more on clinical features rather than
embryological.
(a) Pure microphthalmos (Nanophthalmos)
(b) Colobomatous microphthalmos.
(c) Complicated microphthalmos.
(d) Microphthalmos with cyst.
(e) Microphthalmos associated with systemic syndromes.
(a) Nanophthalmos
11,12
(pure microphthalmos). Nanophthalmos is a rare congenital
condition where a fully developed eye fails to grow like any other eye. This is caused due to
failed growth after the embryonic fissure has closed from end to end. There are no colobomas
present in the globe, it occupies normal position in the orbit, has normal movement but may be
strabismic due to associated high axial hypermetropia. The cornea is smaller, AC is shallow.
The sclera is thickened and the vertex veins are narrow
11
. There is pseudoneuritis, hypoplasia
of macula, nystagmus, amblyopia. There are various types of glaucoma i.e. late onset of simple
glaucoma, narrow angle glaucoma, precipitation of glaucoma following mydriasis
13
. These
children require high hyper meteoric correction may require near correction. They have been
described as phakia children with aphakic correction. The nanophthalmic eyes with
glaucoma do not respond well either to medical or surgical treatment, the later is generally
associated with Chroroideal effusion syndrome
13
that may follow any intra ocular surgery
or injury to the globe.
(b) Colobomatous microphthalmos. colobomatous microphthalmos is a congenitally
small eye that have associated failure of embryonic fissure. These eyes have multiple
colobomatous defects at the site of fusion of embryonal fissure, mostly in uvea and or retina.
These eyes are small in all dimensions, the coloboma may range from a small notch in iris to
coloboma extending up to optic nerve.
(c) Complicated microphthalmos. Is a term used to denote a congenitally small eye
that develops cataract, iridocorneal defects, defects in iris, retina and vitreous. These are due
to
(i) Failure of development of primary optic vesicle.
(ii) Arrest of development after the primary optic vesicle has formed.
(d) Microphthalmos with cyst. In case only retinal tissue protrudes through the em-
bryonic cleft a cystic eye ball with coloboma results. The colobomatous cyst may have an
almost normal eye with a small indistinguishable cyst or the cyst may be so large that the
small eye is not visible. In between are the cases where a formed eye is associated with
colobomatous cyst.
(e) Microphthalmos associated with various syndromes. There is a long list of
conditions that are associated with microphthalmos most of which are cranio facial or mandibulo
facial anomalies.
BUPHTHALMO8
This condition is just reverse of microphthalmos it is a large eye associated with various
types of congenital glaucoma.
DEVELOPMENT OF OCULAR 8TRUCTURE FORM EMBRYONC
GERMLAYER
1. The eye along with its adnex develop from ectoderm and mesoderm. The Bruchs
membrane and the tertiary vitreous (zonule) have duel origin. Bruchs membrane devel-
ops from neural ectoderm and mesoderm. While zonules develop from surface ectoderm and
mesoderm. No part of the eye or its adnexa develop from endoderm.
2. The surface ectoderm gives rise to :
A. The Lens
B. Corneal Epithelium
C. Epithelium of all the ocular adnexa i.e. conjunctiva, meibomian gland, glands of Zies
and Moll, lacrimal gland, lacrimal passage.
3. The neural ectoderm gives rise to
Sensory retina, retinal pigment epithelium, epithelium of ciliary body, pigment
epithelium of iris, sphinter and dilator muscle of iris, melanoeytes, neural part of optic nerve.
4. The Mesoderm gives rise to :
A. Corneal stroma and endothelium of cornea.
B. Iris stroma, ciliary muscles and chroid
C. Sclera, vitreous and extra ocular muscles.
D. Bony orbit
E. Blood vessels.
REFERENCE8
1. Duke Elder.S. ; System of Ophthalmology, Vol-III, Part-I, First edition, Henry
Kimpton, London, 1964.
2. Mann Ida ; Development of Human Eye, Third Edition, British Medical Association,
London, 1964.
3. Barber A.N. ; Embryology of Human Eye, The C.V. Mosby St. Louis 1955.
4. Kozart D.M. ; Embryology of the human Eye in Text Book of Ophthalmology, Ninth
Edition p79-92, Edited by Schcie H.G., and Albert D.M., W.B. Saunders Company, Lon-
don, 1977.
5. Vaughan D and Asbury T. ; General ophthalmology, Ninth Edition p9-13, Lange
medical publication, California 1980.
6. Hamming Nancy and Apple D. ; Anatomy and embryology of the eye in Principles and
Practice of Ophthalmology, Vol-I, p3-20, First Indian edition. Edited by Peyman
G.A., Sander D.R. and Goldberg M.F. Jay. Pee Brothers, New Delhi, 1987.
7. Buffam F.V. ; Lacrimal diseases in Text book of ophthalmology, Vol-4, Edited by
Podos S.M. p7.1 to 7.3, Gower Medical Publication, London 1993.
8. Nema H.V. Singh V.D and Nema N. ; Congenital anomalies of the eye and its adnexa in
Anatomy of the Eye and its Adnexa. Second edition. p162-165, Jay Pee Brothers,
New Delhi 1991.
9. Duke Elder.S. ; System of Ophthalmology, Vol-III, Part-2, First Edition p415-495,
Henry Kimpton London,1964.
10. Schaffer D.B. ; Abnormalities of the eye as a whole in Text Book of Ophthalmology
Ninth Edition., p209-293, Edited by Scheie H.G. and Albert D.M., W.B. Saundes Com-
pany, Philaddphia 1977.
11. Dutta L.C. ; Uveal effusion syndrome in Ophthalmology, First Edition p-122-123
Current Books International, Kolkota 1995.
12. Shields M.B. ; Nanophthalmos in Text Book of Glaucoma, Fourth Edition, p-280,
William and Wilkins Philadelphia 1999.
CHAPTER 2
Examination of the Eyes in
Pediatric Patients
1,2,3,4,5
GENERAL PRNCPLE
The examination of the eyes in children basically does not differ much from that of adult
eyes. The principles of the tests, clinical approach and interpretation of each test is almost the
same in all ages with little modification.
It is essential to keep in mind that a childs eye is not a condensed form of an adult eye.
For purpose of examination, the patients have been divided into following age groups:
A. Neonates and infants
B. Between one year to five years
C. Five to ten years
D. Above ten years.
Otherwise ocular disorders can also be categorised chronologically. It should be remem-
bered that some of the disease may be obvious in lower age group and spill over to higher age
groups. Whereas some of the occult lesions may be present in infancy but become obvious only
at later age.
The diseases have been classified variously according to:
A. Age of onset
B. Chronology of disorder
C. Tissue involved and
D. Clinical Presentation.
According to age of onset, the disorder can belong to the following groups and each may
have distinct clinical presentations and require specific examination:
A. Intra uterine
B. Neonatal
C. Between one to five year
D. Above five year.
11
A. Intra uterine
The intra uterine ocular disorders produce mild to sever deformity and visual loss, are
generally bilateral, many of them are genetic in nature. Some of them are life threatening.
Intra uterine factors that produce ocular malformation can be:
1. Genetic
2. Transplacental.
3. Mechanical.
4. Traumatic
5. Neoplastic (rare).
6. Nutritionational.
The genetic factors can be:
(a) Inherited genetic defect.
(b) Genetic mutation.
(c) Chromosomal aberration.
(d) Effect of exogenous factors like, drugs, radiation, diet.
The transplacental factors causing ocular defects are mostly infection, followed by
various drugs taken in first trimester. In developing countries dietary deficiencies have a
considerable influence on ocular organogenesis.
The common infections that cause ocular morbidly are Rubella, toxoplasmosis,
syphilis, cytomegalo virus diseases.
Uterine bands, deformed uterus, fibroids and umbilical cord are common mechanical
causes of ocular deformity.
Intra uterine traumas in the form of amniocentesis, attempted abortion in early
pregnancy also cause ocular disorders.
NEONATAL CAU8E8 OF OCULAR D8ORDER8 CAN BE
A. Infection.
B. Mal developmental.
C. Effect of pre maturity .
The infection can be acquired during delivery or soon after. The after effects of such
infection may cause ocular morbidity which may be noticed later. The same is true of congeni-
tal infections. These infections are gonorrhoea, herpes simplex, chlamydia, inclusion
conjunctivitis and other bacterial infections. The congenital infections that cause ocular mor-
bidity but do not manifest always at birth are rubella, toxoplasmosis and syphilis.
Maldevelopmental causes of ocular disorders are varied, they can involve a single
structure or may involve more than one structure in various combinations themselves or their
aftermath common among them are.
Anophthalmos, microphthalmos, microcornea, limbal dermoids, bluesclera,
dysgenesis of anterior chamber, coloboma of uvea, polycoria, aniridia, total or partial
EXAMINATION OF THE EYES IN PEDIATRIC PATIENTS 13
cataract, ectopia lentis, congenital myopia, primary , associated or secondary
congenital glaucoma, persistent primary hyperplastic vitreous.
The retina may show congenital folds, detachment coloboma.
The macula may be hypoplastic or may have coloboma.
Ptosis, various types of squint, non cataractous white reflex in pupillary area. Various
craniofacial and mandibulo facial syndromes.
Prematurety causes retrolental fibroplasia, and myopia of prematurety.
CAU8E8 MET BETWEEN ONE TO FVE YEAR8 OF AGE ARE
A. Sequel of
1. Intrauterine infection
2. Developmental anomalies.
3. Neonatal infection.
4. Trauma
5. Dietary Deficiency
B. Inborn errors of metabolism
C. Errors of refraction
D. Strabismus
E. Glaucoma
F. Intraocular tumours
G. Orbital tumours
H. Allergy Endogenous
Exogenous
I. Autoimmune disease
J. Infection Local
Systemic
K. Degeneration and dystrophies.
CAU8E8 OF OCULAR D8ORDER8 8EEN N CHLDREN BETWEEN
FVE TO TEN YEAR8 CON88T OF
A. Residual or continued effect of above
B. Infections become common, so are allergies
C. Trauma in this age group is frequent
D. More children turn up with errors of refraction, squint and amblyopia.
Boys in this age group should be tested for colour defect to plan their future
occupational training.
EXAMNATON OF EYE8 N NEW BORN
Before embarking on examination of eyes of neonate it is essential to remember fea-
tures of new born eye.
The new born eyes as sensory system are better developed than expected when weighed
against its size. The eyes are smaller in size as compared to that of adults. The eye ball of a
new born is 16.5 mm to 17.5mm in diameter as compared to 23 to 24mm of an adult eye. The
volume of eye ball is 2.8 mm as compared 7.0 mm of adult, the eye reaches its adults size
by 14 years of age. By the age of 3 years the eye is about 23 mm in diameters, contrary to
general believe there is no spurt in axial length of an emmetropic eye at puberty.
The normal eye at birth has axial hypermetropia of about 3 to 4 D this is neutralised by
physiological increase in axial length however if the axial length continues to increase two
things are possible
1. Parts of this in neutralised by reduction of curvature mostly of lens and partly of
cornea
4
.
2. The eye becomes myopic.
A. The Vision. The vision of a new born is less as compared to a child of three years but
is not very poor, it has been estimated to be about 1/60
7
the new born child has good accommo-
dation, however there is no clinical method to measure accommodation and convergence. The
fixation reflex is present but poor.
B. The movements of the eyes are bizarre; they can fix a strong stimulus for a short
while and then give up. Movement of two eyes are independent and non conjugate.
C. The inter palpebral fissures are 18 mm in length, the width is less as compared to
cornea hence the cornea looks larger and may be mistaken as abnormal. The new born sleeps
for almost 18 hours a day so most of the time the eyes are closed. Any attempt to widen the IPA
results is contraction of orbicularis and rolling up of eye ball due to Bells phenomenon which
is well developed. However the blink mechanism is absent and develops by 7th or 8th week.
Hence threatening gesture to find out presence or absence or vision is futile. it is difficult to
evert the lids.
D. The orbits are smaller, shallower and round. The space between the globe and
orbital wall is so less that the orbit seems to sit snugly round the globe. None the orbital
contents are visible or palpable.
E. The Cornea is relatively large when compared to adult cornea. The diameter of new
born cornea is 10.5 mm while that of adult cornea is little less than 12mm. However this
growth is far less when compared to axial length of the globe which grows from 18mm at birth
to 24mm at maturity. As the IPA is narrow in new born there is a false impression of cornea
being large. The cornea is flatter when examined on kertometer. Its curvature is uniform,
giving spherical look to the cornea. The Cornea has mild haze that clears within few days.
F. The Sclera is thin the under lying uvea shines through it so it has a bluish tinge that
passes off within few month and sclera becomes white.
G. The Conjunctiva is well developed, the goblet cells are functioning, the conjunctiva
is sterile at birth that gets contaminated within few days. The newly acquired organisms are
generally non pathological.
H. The Lacrimal Glands are not fully developed at birth. It takes three to four years
for them to adequately develop. The new born cries but does not weep; this is due to lack
of reflex tearing that develops after four to six weeks. Psychic tearing takes about six to seven
months to develop.
I. The Lacrimal Sac is fully developed at birth and its lumen is patent .
J. The Lacrimal Passages are canalised at birth. Even if the nasolacrimal duct is not
patent at birth its block is not evident till end of first month as there is no reflex tearing before
that.
K. The anterior chamber is fully formed. Its depth is normal or slightly less than
adult depth. The contents are clear, the angle is wide. The pupil can be safely dilated without
rise of IOP.
L. The pupil is smaller than adult, is shifted nasally and down. It is circular and reacts
to bright light both directly and indirectly.
M. Accommodation and Convergence can not be tested clinically due to lack of fixa-
tion. Accommodation is well developed by 4 months.
17
N. The iris is light coloured due to scanty pigments. There may be a few blood vessels
on the iris surface.. The dilator muscles are poorly developed. The constrictor muscles are
relatively stronger hence it is difficult to dilate the pupil.
O. The Lens is fully developed and functional. The lens grows in size throughout
the life. The lens is more spherical than adult lens, its average diameter is 6mm in compari-
son to 9mm of an adult lens.
P. The fundus The fundus is visible, the colour is paler than normal, the optic disc is
pale, the macula is not fully developed, it continues to grow postnatal. The foveal reflex is
absent. The retinal periphery is also pale.
Q. The broader nose bridge of a new born gives an appearance of pseudo convergent
squint. The movements are not co-ordinated and can not be tested precisely.
EXAMNATON OF THE EYE8 OF NEONATE8 AND NFANT8
Examination of the eyes of a neonate is difficult because a neonate sleeps almost eight-
een hours of a day the waking period is in short spells and most irregular, a neonate wakes
only when hungry or is ill at ease.
It is difficult to elicit correct visual response in an infant.
The examiner should be able to differentiate normal parameters of signs from the
abnormals. Many a times a casual remark by the examiner may send a wave of panic in the
parents least the baby may be blind.
An infants eyes are required to be examined under following circumstances
A. As a part of routine examination of all new borns. The protocols followed in various
hospitals differ greatly. The examination is done either by attending neonatologist or resident
of pediatric ophthalmology posted in nursery and well baby clinic. Nurses and midwives
attending neonates can also be taught to examine a neonates eyes and refer for further
examination for abnormal findings, or seemingly abnormal cases.
B. The neonatiologist has noticed some gross abnormality i.e. craniofacialdysostosis,
faciomandibular abnormality, cryptophthalmos, anophthalmos, microphthalmos, large coloboma
of the lid, proptosis, unilateral gross ptosis, corneal haze, pupillary abnormalities white re-
flex in the pupil and refers for detail examination and opinion.
C. Signs of infection in the eyes : Edema of lids, purulent or mucopurulent discharge.
The above abnormalities are gross enough to be noticed by parents or even a village midwife.
D. There is
1. Family history of
(a) Lamellar cataract
(b) Albinism
(c) Nystagmus
2. Maternal history of
(a) Suspected rubella in first trimester
(b) S.T.D.
(c) Some drugs given in the first trimester that cause congenital anomalies.
(d) Diabetes under treatment.
(e) Myasthenia in mother
(f) Consanguinity
(g) Prematurity
(h) These babies may or may not have been on prolonged oxygen.
(i) Difficult labour fetal distress, neonatal apnoea, intensive resuscitation for above
causes, birth trauma, specially injury by forceps.
(j) A critically ill child who may have congenital toxoplasmosis, herpes simplex
galactosemia, hypoglycaemia.
V8ON TE8TNG N A NEW BORN
One of most difficult tasks in ophthalmology is to give opinion regarding exact quantita-
tive vision present in each eye of a new born. A rough estimation of qualitative vision present
is not difficult to express.
Vision of a new born is very poor in comparison to adult vision that is attainedby seven
to eight year in other wise normal eye in a healthy child.
The vision of neonate improves very fast
6
from 1/60 to 2/60 at 1 month and 6/60 at four
months. With 1/60 vision the child is able to fix a face moving within one meter. These visual
standards are based on various complicated methods by expensive instruments on small sam-
ples i.e. Optokinetic target, Catford drum test, visual evoked occipital potential
(VEP) and forced choice preferential looking (F.P.L)
7,8
Definite fixation reflex and following reflexes take about six weeks to develop before
that an infant may fix for few seconds and give up. These are associated with bizarre movements,
these movements disappear with development of clear fixation. More than one types of
movements are common in between the fixations.
It is said that most suitable infant to examine for vision is an awake, alter and hun-
gry baby.
Visual acuity in a new born is determined by optically elicited movements.
A. The first step is to observe if the child tries to look and fix a light when switched on in
a semidarkned room. This must be repeated several times least some of the bizarre move-
ments may be mistaken for fixation. Each eye should be tested separately. This indicates most
primitive form of vision that can be elicited clinically.
B. Next step is ability of the child to fix and follow the face of the examiner when brought
within the field of vision of the child with both eyes open. This gives a definite clue of vision
being present in at least in one eye but can not indicate which eye. For that one eye is
closed, and other examined, both eyes are examined in the similar way separately one after
the other.
The incidence of blindness in neonates is very low if, visible causes are excluded.
C. The condition that result in bilateral sub normal vision can be
1. Ocular
2. Systemic
The Ocular causes consist of
(a) Persistent corneal haze
(b) Complete cataract
(c) Congenital glaucoma
(d) Retino blastoma
(e) Aniridia
(f) Hypoplasia of optic nerve
(g) Sever retinal degeneration
(h) Albinism
(i) Achromatopsia.
(j) Recessive opticatrophy
(k) Lebers amaurosis.
(l) Taysachs disease.
(m) Glioma of chiasma
The systemic causes are seen in
(a) Premature children
(b) Full term with low birth weight
(c) Foetal distress
(d) Asphyxiated child
(e) Birth trauma to occipital cortex
(f) Cortical blindness
(g) Sagital mild line defects.
Half of the cases of diminished vision in children are genetic in origin.
17
EXAMNATON OF PUPLLARY REACTON TO LGHT
This is most reliable test to determine presence of vision except in cortical blindness.
The test is best performed in a semi darkned room because the infants pupil are smaller than
normal and constricts in presece of bright light in the room. In the semi dark room the pupil
comes to a state of semidilatation that reacts briskly. The light used should be small well
focused and bright. All forms of light reflexes i.e. direct, indirect and swing light responses
are noted. If the pupil is small and it is difficult to appreciate pupillary reaction a hand held
slit lamp may be used.
Small sluggish irregular pupil are indication of inflammation, congenital miosis or
OTHER EXTERNAL EXAMNATON8 CON88T8
A. Face. Examination of face for craniofacial and mandibulo facial anomalies,
albinism, hemangioma.
B. Orbit. Anophthalmos, microphthalmos, cystic eye ball, proptosis.
C. Lid. The lids are examined for Ptosis, coloboma, hemangioma. Lagophthalmos
is far rarer than ptosis. Unilateral ptosis draws attention earlier than bilateral ptosis. Other
anomalies present at birth that draw attention are : epicanthus and ankyloblepharon.
D. Palpebral fissure of new born is smaller than adult. It is narrow in ptosis wide
palpebral fissure is seen in lagophthalmos, proptosis and rarely in buphthalmos. There is
apparent shorting in length in epicanthus. Epicanthus also gives a false impression of pseudo
convergent squint that may not be appreciated at birth but becomes obvious after few months.
Blepharophimosis that is frequently seen with ptosis and epicanthus and causes shortening of
length of I.P.A.
E. Lacrimal system. Only anomaly visible in a neonate is congenital mucocele of
lacrimal sac. Neonatal nasolacrimal duct obstruction when present is asymptomatic and non
elicitable. Ophthalmianeonatorum does not develop within first twenty four hours.
F. Conjunctiva Only important finding that may be present in conjunctiva is
subconjunctival haemorrhage which is of no consequence and is due to birth trauma the
conjunctival sac at birth is sterile that soon gets infected by bacteria, virus and chlamydia.
G. The Cornea Even a normal cornea in a new born looks large because of narrow
I.P.A. The cornea of a new born is 10-10.5mm at birth any cornea larger than 12mm is
abnormal may be buphthalmic. When in doubt about diameter of the cornea it is better
measured. Most reliable measuremnent is taken by a corneal calliper, a transparent scale
may be usedfor rough estimation. Most important cause of large cornea is buphthalmos that
requires early referral, the other cause is megalocornea.
Microcornea is frequent , more common than megalocornea, it may be associated with
microphthalmous and coloboma of the uvea all three may be present singly in an eye or
in various combinations. Bilateral small cornea is frequent.
Other signs that should be looked for are:
1. Corneal haze.
2. Frank corneal opacity.
3. Limbal dermoid.
4. Rupture in Descemets membranes.
1. Corneal haze
Most of the cornea in new borns are relatively hazy, which become bright in few days.
Some of the pathological cause of corneal haze are :
(a) Buphthalmos
(b) Rupture in Descemets membrane
(c) Dysgenesis of anterior chamber
(d) Mucopoly saccharidosis.
Rupture in Descemets membrane is mostly traumatic due to faulty instrumentation
during delivery.
It is difficult to examine the cornea and conjunctiva due to tightness of the lids, and
small palpebral fissure. There is no condition in new born eyes where eversion of the upper lid
is needed. Rarely a lid retractor may be required to see the upper limbus and upper bulbar
conjunctiva. An infant size lid retractor should be used, if at all necessary.
H. Anterior Chamber. The anterior chamber of new born is fully developed but shal-
low. The angle is wide and pupil can be dilated with out rise of I.O.P.
A deep chamber is seen in buphthalmos and megalocornea. Congenital aphakia is a
clinical curiosity. Sub luxation of lens is not appreciated unless carefully looked for.
Commonest anomaly of anterior chamber is mesodermal dysgenesis of Reigers. Other
condition being, Peters anomaly, which causes irregular shallowing of A.C.
I. Uvea. Common congenital anomalies are persistent pupillary membrane, coloboma
of the iris and ciliary body, anisocoria and cryptocoria.
Aniridia is a rare anomaly but is a vision threatening condition that may have Wilms
tumour in the abdomen.
The iris of new born is lighter in colour. Presence of few blood vessels is common and
non pathological.
Posterior uvea is examined by direct and indirect ophthalmoscope with dilated pupil.
J. Pupil. Pupil of a new born child is relatively moitic due to stronger constrictors than
dilators of pupil. It is difficult to dilate the pupil of new born due to same cause.
The pupil is examined for it shape, size position and number. Commonest cause of
irregular pupil is persistent pupillary membrane. Other causes are mesodermal dysgenesis,
Peters anomaly and coloboma of iris.
K. Lens. Absence of lens is extremely rare in new born, sub luxation is difficult to elicit.
Commonest anomaly of interest is presence of cataract. A cataract covering whole of
pupillary area requires urgent referral. Pupil should be widely dilated to see if there is any
clear zone between the outer border of opacity and dilated pupil. If there is clear zone then the
pupil can be kept dilated for light rays to reach the macula till definite treatment is instituted.
It is very essential to be able to differentiate between non lenticular causes of white
reflex, especially retinoblastoma from cataract.
L. Examination of fundus in born. Examination of fundus by both direct and indi-
rect ophthalmoscope is not difficult. It requires little practice with dilated pupil and knowl-
edge to differentiate between normal and abnormal findings in optic nerve, macula and
retinal periphery.
Drug used to dilate pupil of new born
There are many drugs employed to dilate the pupil of a new born. Each ophthalmologist
has his or her drug of choice that may be single drug or a combination of two drugs; one
sympathomimetic and other parasympatholytic. Atropine is better avoided due to its pos-
sible side effects. Commonest combination of drugs is 2.5% phenylpherine with 1%
cyclopentolate
11
. One drop is instilled in each eye twice at an interval of 3 minutes and fundus
examined after 45 minute to one hour.
Two common phenomena observed during fundus examination are
1. Up rolling of the eye due to Bells protective reflex.
2. Non pathological bilateral lid retraction and down ward movement of globe when the
illumination in the room is reduced. This should not be confused with pathological sunset
appearance of the globe. The non pathological lid retraction may persist for four to six
months.
Some ophthalmologists have enough patience to examine the fundus in between invol-
untary the closure of lids. This may be circumvented by use of neonate size of eye speculum.
The neonates tolerate presence of speculum well.
The fundus is examined for transparency of media, disc, blood vessels, macula and pe-
riphery. Remnants of hyloid system are frequent. The arteries are thin, the fovea is not bright
the disc is pale enough to be confused as atrophic. A small pale disc surrounded by yellow
halo is suggestive of hypoplasia of disc.
M. Squint. Squint is difficult to confirm due to bizarre movements unless there is gross
lesion like Mobius syndrome which is other wise very rare.
N. Nystagmus. Nystagmus does not develop before two months. Though it is very
common to confuse non coordinated movements with nystagmus.
O. Proptosis. Proptosis, unilateral or bilateral is not a good sign and requires prompt
investigation.
P. Enophthalmos in neomates and infants is rare.
EXAMNATON OF EYE8 N FR8T YEAR OF A CHLD
Many of the difficulties in examination of a new born childs eye gradually pass away
giving way to easy examination and better interpretation.
A. Recording of vision
The vision generally ranges between 6/60 to 6/36 i.e. the child can recognise faces by
sight, develops preference for toys and picks up particles from the ground. However vision can
not be tested with usual optotypes. The child follows a moving target first by moving the eyes
then by turning and lilting the head and moves towards an object of interest. Presence of
squint, nystagmus, persisting staring and inattention to object
10
are ominous signs
sometimes parents may be able to state if the childs vision is subnormal or not, by comparing
it with older siblings or children of the same age.
This is the age when the children are brought by anxious parents with a definite ques-
tion Does this child has vision ?. This is a very difficult question to answer. Nothing is
worse than informing the parents that the child is blind or normal, that turns out to be oppo-
site later. There are some children who have delayed visual maturation (DVM)
7
and be-
have like a child with sub normal vision.
Vision should be considered normal in a child under one year if the media are clear,
there is no error of refraction, pupil are central circular and react briskly to direct and indirect
light, there is no squint or nystagmus, fundus is normal with normal milestones.
Presence of pupillary reaction to light is not equivalent to visual ability, it merely gives
an indication that both afferent and efferent paths of light reflex are intact
10
.
Dilatation of pupil in presence of direct light stimulus is seen is Lebers congenital
amaurosis, optic nerve hypolplasia congenital cone dystrophy and stationary night
blindness
7,11
In cortical blindness the pupillary reaction is present, this is due to the fact that the
lesions is in optical cortex, commonest cause in children being trauma. However nystagmus is
absent in cortical blindness.
12
All children suspected to be blind should also under go :
1. Electrophysiological investigations-electro-encephalogram, electro-retinogram and
visually provoked response.
2. CT and MRI of ocular structures and brain.
Prematurity, low birth weight, prenatal asphyxia, convulsion congenital anomalies of
brain may have occipital lesions which requires. CT and MRI to evaluate prognosis.
In grown up children the routine is to record the vision in each eye separately followed
by binocular vision. In children under three examination of vision with both eyes open is noted
first followed by recording of vision in each eye separately, while examining each eye sepa-
rately it is better to examine the supposed to be good eye first than the suspected faulty eye.
This helps gaining confidence of the child. A child who has gross diminished vision in one eye
always resents when the better eye is closed, the child may start crying and try to remove the
occluder. While testing one eye the other eye should be fully occluded if necessary by a cotton
pad and tape otherwise the child may peek over the occluder in the better eye. While recording
vision the approximate near vision also be noted in preverbal child. It is good practice to let
one of the attendants preferably one of the parents present during examination of the vision.
The attendant should be asked not to prompt the child when the vision is being recorded. If the
child has glasses the power of the glasses should be noted and vision in each eye is recorded as
with glasses. It is better not to darken the room, this scares the child it should be semi lit.
B. Accommodation, fusion and convergence
17
Accomodation is present at one
month but is relatively poor. It is well developed by four months. Fusion starts at two months,
Fusional convergence develops by six month. It takes steriopis to reach adult level by five
to six years of age. It is well developed by four months though it is considered to be present by
second month.
Once the vision has been recorded other clinical signs should be noted according to a
strict protocol instead of jumping from one structure to the other. The other eye should also
get same attention as the diseased eye even when the lesion is suspected to be unilateral. The
parents may not like this due to preformed idea that the wrong eye is being examined. The
parents at this stage should be explained the purpose of examining both eyes.
The first step is to observe the child in normally lit room from a distance of about a
meter without touching the child.
C. The points to be noted in diffuse light are
1. Is the child comfortable, does he keep his lids forcefully shut this means photophobia
and blepharosm for the same reason a child may hide his face from any source of bright light,
the child may open the eye if the room is darkened sufficiently. A child with searching move-
ment, most probably does not have vision in either eye. Diminished vision in one eye generally
goes unnoticed by the parents unless associated with other causes like squint, corneal opacity
or white reflex in pupillary area.
2. Look for symmetry of face, and skull : Asymmetry of face is generally seen in cranio
facial anomalies. All children with skull larger or smaller than normal should get paediatric
consultation.
D. The eyebrows have scanty, fine hair normally and the eyebrows are symmetrically
arched on both sides. Raised eyebrow with furrows in forehead denotes ptosis. Even a child of
one year may have a raised eyebrow in case of ptosis. Unilateral ptosis attracts more attention
than bilateral ptosis in children. The chin may be elevated .
Flattening of eyebrows, absence of forehead crease and wide IPA denotes lagoph-
thalmos.
Persistent abnormal head posture like head turning, tilting or abnormal chin position
in various combinations point towards possibility of paralytic squint (for details see ex-
amination of strabismus)
E. The lids are examined for hemangioma, coloboma of lid margin and distichiasis.
Epicanthic fold gives a false impression of pseudo convergent squint while telecanthus
gives an impression of divergence.
Narrow palpebral fissure is seen in various types of ptosis and pseudo ptosis.
In blepharophimosis the horizontal length of palpebral fissure is reduced. It is gener-
ally seen with epicanthus and ptosis.
F. In all cases of watering from the eye of long duration under one year of age the first
condition that needs to be excluded is congenital dacryocystitis which should better be
called as neonatal nasolacrimal duct obstruction because inflammation of sac is secondary
to congenital obstruction. A positive regurgitation test is always confirmatory. In absence
of positive regurgitation the condition of lower puncta for its presence and size should be
verified. A congenital coloboma of lower lid which is generally seen at the outer one third may
also cause epiphora. Most important condition that requires exclusion is buphthalmos,
primary secondary or associated. Where the cornea is generally larger than 13mm the AC is
deep, cornea may show Haabs lines.
Children brought with complains of recent unilateral watering are rarely due to
nasolacrimal duct obstruction, buphthalmos or conjunctivitis commonest cause is corneal
abrasion that is confirmed by positive fluorescein stain.
G. Examination of conjunctiva of lower tarsal conjunctiva, lower fornix and bulbar
conjunctiva do not pose much problem so long this is not associated with edema of the lid and
blepharospasm. In such cases an infant size lid retractor may be required sometimes two
retractors may have to be applied. It is better if a drop of anaesthesia is used before applying
the lid retractor, while using lid retractor care should be taken not to injure the cornea. The
childs head and body below the shoulder should be immobilised by the assistant it is better to
take a conjunctival smear at this stage. The cornea, AC and pupil are also examined along the
conjunctiva at this stage. Conjunctiva is examined under focal illumination.
1. Chemosis of conjunctiva without mucopurulent discharge is rarely infectious most
probably it is due to a hay fever like conjunctivitis.
Chemosis with discharge is seen in acute bacterial conjunctivitis.
2. Presence of membrane over the conjunctiva should be considered to be as diptheritic
and treated as such unless proved otherwise, independent of immunisation status of the child.
Other cause of membrane over conjunctiva are :
(a) Bacterial. Streptococcus, staphylococcus, pneumococcus, meningococcus, Koch Weeks
bacillus and Ecoli.
(b) Virus. Epidemic kerto conjunctivitis, herpes simplex, infectious mononucleosis.
(c) Toxic. Stevens Johnson syndrome.
(d) Chemicals
(e) Drug allergy.
One of the dictums to be remembered is Unilateral redness of eye is seldom con-
junctivitis
Chronic allergic conjunctivitis both endogenous i.e. phlycten or exogenous-Spring Ca-
tarrh is rare are under one year but can not be ruled out.
3. Follicles are very common, mere presence of follicle is of no consequence. However
in endemic area trachoma should be ruled out. In children under one month other chlamydia
infections are possibile.
4. Papillae are generally not seen under one year of age.
5. Subconjunctival haemorrhage can be
(a) Wide spread either due to birth trauma specially due to application of forceps or
due to sever cough in children. Whooping cough used to be a common cause of subconjunctival
haemorrhage in children before universal immunisation came in to vouge. Even today it is
seen in non immunised children in developing countries, leukaemia and purpura are com-
mon causes of subconjunctival haemorrhages.
(b) Petechial haemorrhages are seen in epidemic haemorrhagic conjunctivitis and
pneunococcal conjunctivitis.
(c) In cases of sub conjunctival haemorrhage where its outer limit is not visible child
should be examined for possibility of head injury.
6. Colour Changes in conjunctiva are common. A pale conjunctiva is indicative of
anaemia, blue conjunctiva denotes cyanosis. Yellow discoloration is seen in jaundice, patches
of black flat spots are due to congenital melanosis. Faints blue hue is universal in children due
to thin sclera through which the underlying uvea shines.
7. Conjunctival redness is common in all types of conjunctivitis mostly infective. It
can be localised in:- Subconjunctival haemorrhage, abrasion and laceration of conjunctiva,
rarely phlycten, spring catarrh and hemangioma.
Generalised congestion specially bilateral is seen in infective conjunctivitis. The con-
gestion is most marked in the periphery in infective conjunctivitis.
8. Circumciliary congestion or circum corneal flush is seen in
(a) Keratitis, corneal abrasion, corneal ulcer.
(b) Uveitis : Anterior uveitis and panuveitis.
(c) Glaucoma : Acute and chronic congestive.
9. Episcleral congestion is rarely seen in children when present they are mostly due to
buphthalmos or raised episcleral pressure due to an orbital mass.
10. Conjunctival discharge : Purulent discharge is mostly due to gonococci infection
or sever other bacterial infection. Mucopurulent discharge is seen in other bacterial infec-
tion. Watery discharge is seen in allergic, viral and chemical conjunctivitis.
11. Conjunctival Xeroxis is a common feature and ominous sign of vitamin A deficiency.
Bitots spots generally do not develop during first year because if a child is breast fed, mothers
milk contains sufficient vitamin. A for the child for first six month. The child usually gets a
prophylactic dose of 50,000 of vitamin A at six months.
H. Examination of Cornea
Consists of examination of transparency, size, shape, curvature, encroachment over the
cornea, sensation, vascularisation and deposits on both surfaces.
1. Transparency of cornea. Mild bilateral corneal haze is universal in all full term
babies and more common in pre term. The haze passes off within a few days to few weeks.
Presence of dense opacities requires attention and management.
The cornea is uniformly hazy in congenital glaucoma both, primary or secondary that
is generally a bilateral diseases, associated with enlargement of cornea, corneal edema and
rupture in Descemets membrane that may result in Haabs lines. This requires differentiation
from another common cause i.e. injury during forceps delivery, Haabs striations are horizon-
tal may have whirl like appearance while injury by forceps are generally vertical. Out of all
cases of corneal clouding congenital glaucoma due to what ever cause requires immediate
attention to save the sight.
Other causes of uniform corneal haze are relatively rare, they are congenital hereditary
endothelial dystrophy, congenital hereditary stromal dystrophy, various mucopoly
saccharidoses, mucolipidoses and cystinosis
13
. In congenital rubella syndrome the
opacity is relatively dens but clears in few weeks. Interstitial keratities is seen after five
years of age. Peripheral localised opacities are seen sclerocornea and limbal dermoid. In
case of Peters anomaly the opacity is generally localised centrally with clear periphery
frequently associated with other malformations of anterior segment.
Kerato malacia : may be seen as bilateral haziness without circum corneal congestion
is developing countries, in children born to mothers who suffer from malnutrition and have
not received prophylactic dose of vitamin A in last trimester and the child has not been immu-
nised against measles nor has been administered prophylactic vitamin A. However there are
circumstances where the child develops signs of vitamin A Deficiency disease (VAD). These
are ;
1. In sever drought the mothers vitamin A store is depleted during gestation and re-
mains low thereafter.
2. The child is deprived of mothers milk due to death of mother, sever malnutrition in
mother, or the child has been abandoned and children of orphanage.
3. The child suffers from measles, or chronic diarrhoea.
2. Size of cornea. In a new born child the cornea looks large because of smaller I.P.A.
Bilateral large corneas are missed by parents unless associated with watering but unilateral
enlarged cornea draws attention early even without tearing, the cornea is enlarged in
buphthalmos and megalocornea. The former require urgent management. In case of posi-
tive history of buphthalmos in family and suspected rubella syndrome the corneal diameter
should be measured by corneal calliper. Any cornea larger than 12 mm should be considered to
be buphthalmic unless proved otherwise by measurement of I.O.P. examination of angle and
evaluation of optic nerve head. Any cornea larger than 13 mm is definitely buphthalmic.
Small cornea is seen in microphthalmos, and microcornea.
3. Shape of cornea. Distortion of shape of cornea is seen in microcornea microphthalmos
and phthisis.
4. Corneal curvature. Increased corneal curvature suggestive of keratoconess that is
extremely rare in first year. Flattening of cornea is more common and is seen in microcornea,
microphthalmos and cornea plana. The former two are generally associated with gross
incorrectable vision loss. Phthisis which is rare in first year of life may also present with flat
cornea.
5. Enchromement over cornea. The corneal periphery may be as opaque as sclera in
a rare instance of sclerocornea, where the opacity extends well into the cornea from the
sclera obliterating the limbus. The peripheral opacity may be vascularised. In still rarer occa-
sions the whole of the cornea may be as opaque as sclera. Dermoid
14
is more common than the
former. The commonest site being the limbus where a dry raised circular plaque of fibro fatty
tissue is seen astride the limbus, they generally have few hairs growing over the growth.
However they may develop in the middle of the cornea as well.
6. Sensation of cornea. Corneal sensitivity can be elicited from birth. It is reduced
over the opacities, keratanisation and herpes simplex keratities. There is a very rare condition
where corneal sensation is absent from birth called congenital dysautonomia.
7. Vascularisation of cornea. Vascularisation of cornea is rare under one year of age.
Vascularisation of cornea always denotes pathology either in conjunctiva or in cornea. The
former produces supefecial vascularisation. The corneal vascularisation can be superficial or
deep. Causes of superficial vascularisation are infective and allergic kerotoconjunctivitis. The
only cause of deep vascularisation in children between 3-5 years is interstitial keratitis.
8. Deposits on the cornea are mostly inflammatory.
I. Examination of Sclera. Sclera is not a tissue to have many findings in a child under
one year. The sclera of an infant is relatively thin it is not as opaque as adult sclera but not
translucent also. The uvea under neath gives it a bluish hue that passes off within few months
but not in case of blue sclera brittle bone syndrome in osteogeneses imperfecta.
The sclera develops yellowish tinge in jaundice, sub-conjunctival patches of black
pigmentation are very common and may cause worry in parents.
Sclera is devoid of any inflammatory process under one year of age.
Conjenital ectasia may be seen rarely more common cause of ectasia is buphthalmos.
J. Examination of anterior chamber :
1. An infants anterior chamber is fully formed at birth is of equal depth in both eyes it
reaches adult depth by age of one year AC should be devoid of any thing but aqueous. Differ-
ence in depth of AC in two eyes is abnormal so in presence of mesodermal tissue.
2. Deep AC is seen in buphthalmos, keratoglobus and congenital axial myopia.
Congenital aphakia is a clinical curiosity only a few cases of spontaneous absorption of lens
have been reported in first year, ofcourse surgical aphakia gives a deep A.C. in infants who
have undergone lensectomy without IOL. A very few infants may have iris clip IOL
16
as part of
management of congenital cataract and have relatively deep A.C. In P.C. I.0.L. the depth of
A.C. is almost normal.
3. Shallow A.C. is seen in micro cornea, microphthalmos, cornea plana, mesodermal
dysgenesis of anterior chamber, Peters anomaly.
4. Contents of anterior chamber Normal content of AC is aqueous humour that is
crystal clear, without any visible suspended particle. It is very difficult to detect mild to mod-
erate turbidity under one year of age.
Presence of blood is more common in this age group which is mostly traumatic than
pus. Presence of any white material in A.C. should be considered to be pus unless proved
otherwise. Common cause of white material in A.C. are: hypopyon, pseudohypopyon, cor-
tical material.
K. Measuring intra ocular pressure. All infants with large cornea, history of
buphthalmos in siblings and abnormal tissue in A.C. should undergo measurement of 10P
under general anaesthesia.
L. Examination of iris. Iris of a new born has lighter colour than what is expected to
be at adult life due to lack of pigment, however the iris becomes pretty dark by one year if not
as dark as adult. An iris that is abnormally light with pink pupil and trans illuminates in a fair
child is due to albinism.
Iris of one eye may differ from the other eye and is called heterochromia iridium in
contrast to difference of colour in the same eye that is known as heterochromia iridis which
can be unilateral or bilateral.
Presence of congenital holes in iris other than pupil is called polycoria that generally
lacks iris sphinters and called pseudopolycoria. True polycoria is rarer, these extra pupil
have independent constrictor and dilator muscles and react to light independent of main pupil.
Congenital coloboma of the iris is generally situated in lower pole of pupil, may be
localised to iris only or may be part of more extensive colobomatous anomaly of uvea. The
coloboma of iris may be present as isolated anomaly of the eye or may be seen in microcornea
and microphthalmos. Perhaps most common anomalous finding in the iris is persistent
pupillary membrane where strands of mesodermal tissue originate from the iris collarette
and may pass across the pupil giving an irregular pupil. It disappears with age. Presence of
few blood vessels in first two months are no consequence they also disappear with in few
months.
M. Examination of pupil Examination of pupil alone gives much more information
regarding presence of many anomalies than any other examination. Pupil reacting briskly to
direct and indirect light stimulus is confirmed sign of intact afferent and efferent precortical
visual path.
Pupil is examined for
1. Number,
2. position,
3. shape
4. size,
5. colour and
6. pupillary reaction.
1. Number. In rare instances there may be more than one pupil in the same eye either
as pseudopolycoria which is more frequent than less common true polycoria both the con-
ditions are associated with multiple anomalies of the eye. Cryptocoria is congenital absence
of pupil .
2. Position. Single Pupil with slight nasal and medial shift is normal. However there
may be corectopia where a pupil is shifted away from its normal place a part of multiple
ocular anomalies.
3. Shape. Normal pupil is circular. All irregularity of pupil are not pathological only
irregularity due to inflammation needs treatment. Persistent pupillary member is the com-
monest cause of irregular pupil under one year of age. Other causes are coloboma of iris,
mesodermal dysgenesis of A.C., pseudopolycoria.
4. Size. Pupil of a child under one year is smaller than that of a child of three year due
to parasympathetic over action when compared to sympathetic. The constrictor muscles are
better developed than sympathetic. Unilateral small pupil with narrow I.P.A. is suggestive of
Congenital Horners Syndrome. Pupil of a child under one year should be examined in
diffuse light. Dilatation of pupil requires more time due to strong constrictor. Slight difference
in size is common. Difference in size is called anisocorca. Where it is important to find out
which pupil is abnormal.
Large pupils are seen in congenital myopia and buphthalmos, congenital inter-
nal ophthalmoplegia is very rare. However in all cases of large pupil history of instillation of
mydriatic or cycloglegic should be excluded.
5. Colour of Pupil. The pupil looks dark even in the most pigmented iris when seen
with a torch. On careful examination the normal lens produces two miniature images the third
and fourth Purkinje images. Absence of third and fourth are seen in an opaque membrane
over the anterior lens capsule and absence of lens (aphakia). A white reflex in pupillary area is
always pathological.
Causes of white reflex in pupillary area (see chapter on Len and Retinoblastom).
6. Examination of pupillary light reflex. A brisk direct and consensual light reflex
denotes intact afferent and efferent neurological precortical path. A child with lesion of visual
cortex presents as cortical blindness where the pupil are of same size and react well.
Unilateral dilated sluggish pupil with contralateral normal sized, reacting briskly to
direct and indirect light is due to iridoplegia that may be therapeutic or neurological.
Bilateral fixed dilated pupil are due to
1. Bilateral irideplegia
2. Bilateral sever optic neuropathy.
MarcusGunn pupil is seen in unilateral complete lesion of optic nerve.
7. Examination of lens. Lens of an infant is examined mainly for its transparency.
Most important opacity is an opacity that covers whole of the pupil but may have clear periph-
ery 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 ur-
gent lensectomy with management of aphakia and avoidance of ambloypia. A lens with clear
periphery may be left with dilated pupil, this allows sufficient light rays to reach the macula
for its development and prevention of amblyopia.
Sub luxation and dislocation of lens is less common under one year and when present
becomes obvious only on maximum pupillary dilatation.
Scattered cortical opacities do not cause visual impairment and their presence should
be noted in examination card and parents informed about their non progressive nature with
instruction for yearly follow up.
8. Examination of vitreous. Vitreous is examined for presence of P.H.P.V., remnants
of hyoid system, vitreous haemorrhage, familial exudative vitreo retinopathy,
retinopathy of prematurity. Best method to examine vitreous and rest of the fundus is by a
binocular indirect ophthalmoscope using a condensing lens that give magnification, steriopsis
and moderate field with maximum dilatation.
Recording of distant vision in a child. In principle and practice there is no difference
in examination of vision in adults and children. The difficulty arises in reaction of the child to
method of examination, a shy child may not co-operate well to record vision, an-other child
under the same circumstances may be most co-operative not only in recording of vision but
also examination and manipulation of eyes. Vision is a sensory phenomenon for this co-operation
of the child is most important pre requisite. Patience, practice and attention of the examiner
in recording vision in a child is as important as attention of the child and co-operation of
parents.
Sixty parent of afferent input to the central nervous system for development
of higher centres come from vision of the child.
A child does not complain of gradual loss of vision unless it is discovered by the parents
at home. A very common observation is that the child prefers to sit nearer the T.V. than others
and resents being shifted away. A teacher may complain of poor attention of the child, the
child may fail to copy from the black board. Most often children with poor vision are brought
with squint. Unilateral loss of vision may go undetected for longer time unless the child devel-
ops squint or accidentally closes the better eye.
Acute and painful loss of vision brings a child for examination early. Recording of vision
in children can be grouped into following categories.
Examination of vision of
1. New born
2. Under One year
3. Between 1-2 years
6. Above 5 year.
Recording of vision above five years is similar to that in adults with little modification,
attention of examiner and practice. Co-operation of parents, attendants and teachers is also
desired for correct interpretation.
Recording of vision under one year of age has been delt with earlier.
Recording of vision between 1-2 years of age
Recording of vision under two years is slightly easier than under one year.
Presence of squint, nystagmus, indicate poor vision at least is one eye. Nystagmus de-
notes bilateral diminished vision. Presence of unilateral squint invariably means diminished
vision in the squinting eye. Preference of fixation by one eye means diminished vision in other
eye. Closure of the better eye is resented by the child who may start crying and try to remove
the obstruction. A child with searching movement most probably does not have vision in either
eye. Various methods to examine distant vision are
17,18,19
:
1. Optokinetic drum of Harcourt.
2. Catford drum
3. Preferential looking test.
4. Visually evoked potential
5. Teller acuity card
6. OKNOVIS
17
7. Boeck candy bead test
8. Cardiff acuity card.
None of the above tests is accurate, some times more than one test has to be used.
Rarely children as young as 18 months have responded to Snellens optotypes
10
Optokinetic drum test, preferential looking test and visually evoked potential and their
modifications are time honoured tests. Some new tests have been evolved for better results.
They are broadly divided into two groups:
1. That depend upon the preferential looking
2. That does not depend upon preferential looking.
The principle of these test are similar to optokinetic drum. Where alternate black and
white strips evoke nystagmus when the drum is activated in front of the childs eye from a
close quarter.
(a) Catford drum test In this test cylindrical drum is replaced by a circular disc with
dots ranging between 0.5 to 15mm in diameter representing vision between 6/6 and 2/60. Ro-
tation of the disc at a distance of 60 cm evokes pendular movement and not nystagmus as
seen in O.K.T. The smallest target that starts the pendulular oscillation gives the vision it may
over estimate vision many folds and can not be used in ambloyopia screening.
(b) Teller acuity card
10, 17, 18
. This test is modification of preferential looking test. This
test takes less time and simple to perform. Testing distance varies with age of the child being
tested infants under six month are tested at 36cm while those up to three years are examined
at 55cm. The results are obtained in cycles which can be converted to Snellens equivalent.
(c) OKNOVIS
17
This new technique is based on the principle of arresting an elicited
optokinetic nyslagmus by introducing optotypes of various sizes. The instrument is a hand
held revolving drum that rotates at speed of 12 revolutions per minutes. The test is done at
60cm to elicit optokinetic nystagmus, coloured pictures on the revolving drum are used. Once
nystagmus is elicited optotypes of different sizes are introduced to arrest nystagmus. The test
gives a rough estimate of vision.
(d) Cardiff acuity cards
7
. The test depends upon preferential fixation and consists of
a series cards depending on principle of vanishing optotypes.
The tests that do not depend upon preferential looking
(a) Boeck candy bead test. The child identifies an incentive i.e. candy beads of gradu-
ally decreasing size at 40cm. The childs hand is guided to locate the candy and then to mouth.
This the child finds interesting and worth repeating.
(b) Other tests used are : Marble game test, Worth ivory ball test, Sheridan ball test.
Recording of vision between 2 to 3 years age
By this age the child is verbal has better power of expression some may be conversant
with alphabets and numbers. Hence preferential looking test are no more required but in
cases of gross visual loss or suspected absence of vision, visual evoked potentials may be re-
quired.
Commonly used methods are various types of optotypes that may be
1. Snellens chart
2. Landolts broken C
3. STYCAR Test (Snellens test for young children and retarded)
4. HOTV Test
5. Dot visual acuity test
6. Coin test
7. Miniature toy test.
The last three tests are crude tests and meant for children in second year, by three
years the child can recognise shapes hence can be tested on optotypes.
Recording of vision between 3 to 5 years
By three years most of the urban children start going to some types of schools that teach
them alphabets, numbers, recognise shape, dimension and colour. Rural children in develop-
ing countries who constitute 80% of paediatric population are difficult to evaluate visually due
to illiteracy. Examiner may have to revert to methods employed to examine children between
two to three years in these rural children.
Commonly used methods are : All the test objects are various types of optotypes, the
target of which makes an angle of 5 minutes when kept of 6 meters or 20 feet the testing may
be done at 3 meters with proportionally reduced size of the optotypes.
Commonly used optotypes are :
1. Snellens chart commonly used is in English but may be in vernacular as well.
2. Snellens E chart
3. Snellens tumbling E test
4. Landots broken C
5. Sjogren Hand or Arrow
6. HOTV test
(a) Lipman 4 letters ( It consists of four letters HOTV arranged in a circle)
(b) Sneridan 5 letters. X is added to HOTV. Addition of A and U make it seven letters,
they can be used at 6mts or three meters with a mirror.
There should be no difficulty in recording vision in a child if he is literate, he can be
tested on usual Snellens chart at six meters. But a non literate child has to be tested on other
charts. It is better to have a smaller hand held version of the test type to be shown to the child
at usual reading distance and explain the child, what he is expected to tell. After two or three
trials the child is able to correctly tell the position of break in Landolts chart and direction of
open end of E.
While recording of vision following steps are useful to gain the confidence of the child
and his co-operation.
1. Record vision in normally lighted room.
2. Allow one of the parents or grand parents, preferably mother to be present near the
child but forbid her to prompt the child.
3. First note the vision with both eyes open.
4. It is better to examine eye with better vision first and the other eye later instead of
usual convention of examining the right eye first and left eye later. Because a child with gross
visual loss in one eye will resent closure of better eye and may refuse to be tested.
5. Some children generally above five years of age can memorise the whole of the Snellens
chart while sitting in the examination room in such cases it is better to ask the child to read
from right to left rather then usual left to right barring of course Urdu, Arabic and some other
letters.
6. While examining one eye the other eye should be occluded completely and watch that
child does not peek over the occluder.
7. Keep the head of the child in primary position. A child may turn the head to see with
the better eye.
Examination of near vision in children. Children under fifteen have strong accom-
modation hence their near vision is good and rarely complain of diminished vision children
with high uncorrected hypermetropia may complain of asthenopia and running of letters.
If a child complains of diminished near vision the first test is to exclude hypermetropia
by cycloplegic refraction. Other causes of diminished near vision are
1. Incorrectable distant vision
2. Nystagmus
3. Cycloplegia
(a) Neurological-internal ophthalmoplegia
(b) Drug induced cycloplegia
4. Malingering.
Examination of colour vision. About 8% of boys suffer from some degree of colour
defect which is congenital hence it is very important to note colour vision in boys more for
future planning of their career than done for any clinical purpose
Examination of field. It is very difficult to elicit field defect under 10 years of age.
After 15 years of age the field examination is done as in adults. Between 10-15 years automatic
perimeters may be helpful.
REFERENCE8
1. Boger W.P and Peterson R.A. ; Paediatric Ophthalmology in Manual of Ocular
Diagnosis and Therapy, edited by Deborah Pavan langston, third edition p-251-255,
Little Brown.
2. Shaffer D.B.; Normal eye in infancy and childhood in Text book of ophthalmology,
edited by Scheie H.G. and Albert D.M., 9th edition p-279-280, W.B. Saunders Company,
Philadelphia 1977.
3. Vaughan D., Asbury T.; Special subjects of paediatric interest in General
ophthalmology, 10th edition p-279-285, Lange Medical Publication Singapore 1983.
4. Hoyt. C.S. Nickel BL, Billson F.A.; Ophthalmologic examination of the infant,
survophth 26 : 177-189, 1983.
5. Safir A., Merker C. ; Normal eye in infancy and childhood in Text book of Oph-
thalmology, edited by Scheie H.G. and Albert D.M., 9th edition p-279-280, W.B.
Saunders Company, Philadelphia 1977.
6. Duke Elder S. and Cook C. ; Chronology of Development in System of Ophthalmo-
logy, Vol. III part 1, p-291-312, Henry Kimpton London 1963.
7. Zwaan J.T. ; Does this baby see in Decision making in Ophthalmology, Edited by
van Heuven WAJ and Zwaan J.T. p-124-125, Harcourt Brace 1992.
8. Flyon J.T. ; Evaluation of Visual Function in Neonate and Infant in Paediatric
Ophthalmology Vol. I, second edition, Edited by Harley M.D. p-1-25, W B Saunders
Company Philadelphia 1983.
9. Zwaan J.T. ; Uncorrectable poor vision in a child in Decision making in oph-
thalmology , Edited by van Heuven WAJ and Zwaan J.T. p-138-139, Harcourt Brace
and Company 1992.
10. Diamond G.R. ; Evaluating Vision in preverbal and preliterate infants. In text-
book of ophthalmology, Edited by Podos S.M. and Yanoff M. Vol-5, p-2.1-2.6 Mosby
London 1993.
11. Goldhammer Y. ; Paradoxical pupillary reaction in Neuroophthalmology update,
Edited by smith J.L. P-39-42, Masson New York 1977.
12. Yee R.D. Balon, R.W. Hanrubia. Y. ; Study of Congenital nystagmus Br Jr. OPL 64 :
926-930, 1980.
13. Zwaan J.T. ; Cloudy cornea in neonate in Decision making in ophthalmology,
Edited by van Heuven WAJ and Zwaan J.T. p-128-129, Harcourt Brace and Company
1992.
14. Duke Elder S. ; System of ophthalmology, Vol. III, Part-II, Page 820, Henry Kimpton,
London 1964.
15. Mc Dermott M.L. ; Corneal hypesthesia in Decision making in ophthalmology,
edited by van Heuven WAJ and Zwaan J T P-174, Harcourt Brace and Company 1992.
16. Singh D. ; Paediatric Cataract CME series All India ophtlamological society.
17. Sharma P. ; The preliminary examination and assessment of vision in Strabismus
simplified, 1st Edition p-55-62, Modern Publishers, New Delhi 1999.
18. Khurana A.K. ; Visual acuity contrast sensitivity and test for potential vision in
Theory and practice of optics and refraction, first edition p-31-51 BI Churchill
Livingstone Pvt. Ltd. , New Delhi 2001.
19. Ramanjit Shihota and Radhika Tandon.; Assesment of visual function in Parsons dis-
eases of the eye. 19th edition. p-93-97. Butterworth. Heinemann Oxford 2003.
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.
A. The lids consist of an assorted number of tissues:- the skin with its glands, muscles,
motor and sensory nerves and abundant blood supply.
B. The junction of the conjunctiva and the skin form the lid margin hence the lids are
seats of dermatological, vascular and neurological disorders besides usual infection, inflam-
mation, allergy, and malignancy common to both.
C. The disorders of the lid can be congenital or acquired. The acquired conditions out-
number the congenital anomalies.
D. Malignant growth of the lid in childhood is almost non-existent. Benign growths are
as common as in adults. In fact most of the benign growths of the lid have their origin in
childhood and may remain dormant to become obvious at various stages in adult life.
E. Trauma is common disorder in all ages.
ANATOMY OF THE LD8
1,2,3
A. Lids are two multiple layered folds, chief function of which is protective. They protect
the eye from external foreign bodies, fluids and gases. Besides protection, the lids have two
more functions and they are:
1. Regulate entry of light in the pupil
2. Spread the tear over the ocular surface. The lids indirectly help to drain the tear
from the conjunctiva and propel it down the nasolacrimal duct.
B. The upper lid is larger than the lower one. It spreads from the eyebrow above to lid
margin below and from lateral wall of nose to the zygomatic bone laterally, covering the upper
2 mm of cornea.
C. The lower lid extends from the inferior orbital margin to lid margin just reaching the
lower limbus.
The normal lids in adults can be lifted off the globe and everted without any difficult but
in children, the lids are so tight that they cannot be lifted off the globe or everted with ease.
D.The space between the two lids is called inter palpebral aperture (IPA) or Palpebral
fissure. In adults it is about 30mm in length in contrast to 18. 5 mm at birth. The width of
34
DISORDER OF LID IN CHILDREN 35
IPA in adult is 9 mm while at birth it is 8 mm. The IPA is a conch shaped space bounded by the
two lids. The lids join each other laterally in acute angle and the junction is called lateral
canthus. The corresponding medial junction is called medial canthus that is rounded medi-
ally. The structures in the medial canthus are caruncle, semi lunar fold and lacus
lacrimalis, upper and lower puncta.
E. The caruncle represents the vestigial third lid; it does not have skin of the lid. It
is a mound of conjunctiva with small hair along with their glands. It has no definite function.
F. The plica semilunaries
4
is a vertical arcuate fold of conjunctiva with concavity
towards the cornea. It contains some plain muscles
2
. The epithelium is thickened. It also con-
tains goblet cells. This structure represents the nictitating membrane of lower vertebrates
with out any function but is invariably involved in pathological process of the conjunctiva.
G. The lacus lacrimalis is a shallow depression in the conjunctival surface of the
lower lid in the medial side.
H. The two lacrimal puncta initiate the lacrimal drainage. They are situated 2mm
lateral to the medial canthus in the inter marginal strip, one in each lid. They are circular or
oval about 1mm wide in normal eye, they are constantly in touch with the globe and initiate
the lacrimal drainage.
There are two canthal ligaments, the medial and lateral.
I. The medial canthal ligament or medial canthal tendon is a horizontal fibrous struc-
ture that extends form medial canthus to the medial wall of the orbit, to this is attached
medial horn of the levator and fibrous extension of the tarsal plate. It is palpable as a horizon-
tal cord under the skin when the lateral canthus is pulled laterally. It lies anterior to the
lacrimal sac. It splits into two leaves; an anterior leaf and a relatively smaller posterior leaf.
The fundus of the lacrimal sac is enclosed in between these two
5
. The other function of the
ligament is to keep the lids stretched horizontally.
J. The lateral canthal ligament is less developed than the medial, to it is attached
the lateral horn of L.P.S. and lateral fibers from the tarsal plate.
THE LAYER8 OF THE LD
The lid is multi-layered structure of both ecto and mesodermal origin. It is highly
vascular, rich in nerve supply and lymphatic.
The layers of the lids are
A. Skin
B. Layer of muscles :
1. The orbicularis
2. levator palpebral superior
3. Mullers muscle.
C. The fibrous layer :
1. The tarsalplate
2. The orbital septum
3. The canthal ligaments
4. The Whitnall ligament.
D. The conjunctivaTarsal conjunctiva
E. Lid margin.
A. The skin of the lid is thin and delicate but rich in blood supply and sensory nerves.
It is very loose; it can be lifted off the subcutaneous tissue. There is no subcutaneous fat. There
are only downy hair with their glands scattered thinly throughout. Due to laxity of the skin,
large amount of fluid like blood, pus and other effusion can accumulate under the skin. In
fracture of paranasal sinuses, air can get under the skin causing emphysema. The Accumula-
tion of fluid , when present is confined to the lids, only due to firm attachment of the skin to the
periostium
6
. The skin of a newborn is tighter than adult. With age it may become loose enough
to hang down at old age. The skin is thrown in horizontal lines and most prominent among
them is the supratarsal fold that is used as a land mark during ptosis surgery. These
horizontal lines are caused by cutaneous attachment of levator palpabral superior. They are
obliterated in ptosis and edema of lid.
B. Layer of Muscles
1. The orbicularis oculi is a horizontally placed striated muscle supplied by seventh
nerve. It has two parts-the orbital and the palpebral. The palpebral part spills over like a
sheet in the temple, cheek and the forehead. It encircles the palpebral fissure like a sphincter
and closes the lid. It is a direct antagonist of levator. The palpebral part has two smaller
divisions i.e. the perceptal and pretarsal. They have their origin over the fascia of the lacrimal
sac and their main function is to cause lacrimal fluid to be pumped into the nasolacrimal duct
with each blink. Paralysis of orbicularis results in lagophthalmos. The orbital part surrounds
the orbital margin.
2. The levator palpebral superior. This striated muscle, supplied by upper division
of the third cranial nerve is the main elevator of the upper lid. Its function is to lift the
upper lid and keep lifted. It is direct antagonist of orbicularis.
The muscle originates from the under surface of lesser wing of sphenoid above and in
front of the optic foramen. It travels between the roof of the orbit above and superior rectus
below. It reaches the lid in a wide aponeurosis that is divided in three slips.
(a) The main slip is attached to the superior border of the tarsalplate, some fibres blend-
ing with the anterior surface of tarsal plate.
(b) Some fibers anterior to this pass through the fibers of the orbicularis and get in-
serted in the skin to form horizontal skin creases.
(c) The posterior most fibers are attached to the upper fornix to give it its depth.
The lateral horn of the aponeurosis is attached to lateral orbital tubercle and lateral
canthal ligament. The medial horn gets inserted in medial canthal ligament and frontomaxillary
suture. Part of the aponeurosis blends with Whithnall ligament that acts as check ligament of
the levator.
3. The Mullers Muscle
8
. This in contrast to the levator and orbicularis is a non striated
muscle. It lies under the conjunctiva as an ill defined mass 12 x 15mm that originates from
under surface of levator and gets inserted on the upper border of the tarsal plate, deep in the
main aponeurosis, It is supplied by cervical sympathetic. It is an accessory elevator of lid its
paralysis causes mild ptosis of upper lid. It augments the action of L.P.S. by 2mm. A similar
but less developed muscle arises from the inferior rectus and gets attached to the lower border
of tarsus of lower lid. Its paralysis causes elevation of the lower lid margin and the condition is
called upside down ptosis.
C. The Fibrous Layer
1. The tarsal plate. Each lid has a tarsal plate or tarsus that is responsible for con-
sistency and shape of the lid and gives contour to the lid margin. The tarsal plate along with
orbiculris that is loosely attached to its anterior surface, form a strong protective layer when
the lids are shut. The upper tarsal plate is larger than lower tarsus in all dimensions. It is
about 30mm long; 9 to 12mm in height and 1mm thick. The tarsus are made up of fibrous
tissues in which are embedded the meibomian glands that are arranged parallel to each
other and have branching ducts. The meibomian glands open on the lid margin and these
glands secrete an oily material that contributes maximum to the lipid layer of the tear. The
upper tarsal glands are longer and have wider lumen than lower glands. The upper tarsus has
about 25 glands while the lower has only 12 to 15 glands. The lower border of the upper tarsus
is slightly concave. Either side of the tarsal plate is anchored to the corresponding canthal
ligament. The inner surface of the tarsal plate is lined by tarsal conjunctiva. The tarsal
glands are visible through the normal tarsal conjunctiva as light streaks.
2. The orbital septum. This structure is also known as palpebral fascia. It is a sheet of
fibrous tissue of variable thickness. It is pierced by aponeurosis of levator in the upper lid and
fibers from interior rectus in the lower lid. It originates from the periorbita of the orbital
margin and gets attached to the peripheral margin of the tarsal plates. Medially it is attached
posterior to the fossa for lacrimal sac and laterally to the orbital tubercle. The septum orbitale
along with tarsal plate form a vertical barrier that separates the orbit from the lid. It limits
the infective process to reach the lid from orbit and vice versa. It is not a very rigid structure.
It is pushed posteriorly by effusion in lid and forward by same pathology behind. It ripples
with movement of the globe. The septum prevents orbital fat to herniate in the lid.
3. Canthal ligaments. See above.
4. The Whitnall ligament
9
. This is a fibrous band that runs horizontally above the
aponeurosis of levator palpebral superior from Whitnall tubercle on the zygomatic bone. It
gives attachment to levator aponeurosis. It is one of the important landmarks in ptosis surgery.
D. The tarsal conjunctiva
The tarsal conjunctiva lines the tarsi, form the inter marginal strip of the lid margin to
the fornices in each eye. The normal tarsal conjunctiva is translucent over the tarsal plate.
The meibomian glands are visible through the conjunctiva, so long it is translucent. The duct
become obscure due to edema, follicle, papillae and scar in the tarsal conjunctiva. The
tarsal conjunctiva cannot be lifted off the tarsus.
E. The lid margin
The lid margins form the borders of palpebral fissure. They extends from canthus to
canthus in each lid are about 3 mm in thickness, its posterior border is sharp and must remain
constantly in touch with the globe for proper spread and drainage of tear. The anterior border
is rounded; the lashes arise from the anterior border in two or three rows. The upper lid being
larger has more and longer lashes than lower lid. At the base of the lashes open the modified
sebaceous glands the glands of Moll and Zeis.
The glands of Moll are modified apocrine sweat glands that pour the secretion in the
lash follicle. The glands of Zeis have no lumen they produce lipid.
The skin of the lid and conjunctiva merge with each other on the lid margin hence it has
characteristics of both skin and conjunctiva and called inter marginal strip. The orifices of
the meibomian glands open on the lid margin behind the lashes. There is a faint grey line
along the lid margin. The lid can be surgically split into two halves, the anterior containing
skin and orbicularis and the posterior containing tarsus and conjunctiva along this line.
THE BLOOD 8UPPLY
The lids have very rich blood supply this is the cause of rapid healing of lid wounds. The
lids get their arterial supply from both internal and external carotid arteries. The blood
supply is broadly divided in superficial system and deeper system the former is again
divided into facial and orbital groups. The arteries of the lid ultimately form two arterial
arcades in each lid, one above the tarsal plate and other near the lid margin.
THE VENOU8 DRANAGE
The lids have two types of venous drainage i.e. in the pretarsal and retrotarsal, which
drain in external juglar, cavernous sinus and pterygoid plexus.
THE LYMPHATC DRANAGE
The lymphatic from lateral half drain into pre auricular nodes. Those from medial side
drain in sub mandibullar group of nodes.
THE NERVE 8UPPLY
The motor supply is from facial and oculomotor, the sensory is from trigeminal, the
nerve to Mullers muscles is via sympathetic.
CONGENTAL ANOMALE8 OF THE LD8
38,39,40,41
A. Congenital anomalies of the lids are generally bilateral and symmetrical, unilat-
eral congenital anomaly is no exception. The congenital anomalies may be confined to the lid
or may be associated with other anomalies of globe and systemic anomalies. A common combi-
nation is congenital anomalies of lid with cranio facial and mandibulo facial deformity.
Children with congenital lid anomalies are brought to physician generally for cosmetic blem-
ish or exposure of the globe. However, an anomalous lid, if it covers the pupil may result in
amblyopia that requires early management. Large exposure of the globe due to coloboma of
upper lid may also require urgent treatment.
B. Congenital anomalies of lid can be :
1. Deformity of lid margin
(a) Coloboma
(b) Ankyloplepharon
(c) Distichiasis
(d) Entropion
(e) Ectopion
2. Deformity of the interpalpebral aperture
(a) Telecanthus
(b) Epicanthus
(c) Blepharo phimosis
(d) Euriblepharon
3. Congenital Ptosis and allied conditions
4. Disorders of the lid skin
(a) Blepharochalasis
(b) Epiblepharon
5. Minor and rare deformities
1. Deformity of Lid Margin
(a) Coloboma of the lid. Coloboma of upper lids result due to improper fusion of medial
and frontonasal mesodermal process of face and are situated in the medial one third of the
upper lid in the form of a small notch or as one extending considerably upwards. The colobomas
generally have a broad base at the lid margin; it may involve the upper punctum and canaliculus.
However, a large rectangular defect may develop in the middle of the lid, exposing upper part
of the globe and conjunctiva. Coloboma of upper lid is generally unilateral. It is not uncommon
to find other congenital anomalies in the same eye i.e. corneal opacity, microphthalmos, coloboma
of the pupil. Dermoid or dermolipoma may be present in Goldenhars Syndrome. The lower
lid colobomas are seen at the lateral third of lower lid, they are not so well demarcated as
coloboma of the upper lip and may be missed unless carefully looked. Lower lid colobomas are
wider and shallower; they by themselves are almost symptom less. They are a constant feature
of mandibulo facial syndrome i.e. TreacherCollin, Franceschetti syndrome.
(b) Ankyloblepharon. The lids are fused between third and sixth month intra uterine
life and start separating with development of cilia and glands of the lid. The separation starts
from the middle and spreads on each side. The separation is complete at the time of birth.
Some times few strands of skin may extend upon the inter palpebral aperture attached to the
other lid, these strands are called ankyloblepharon. They are more common on the lateral
side, may present as a single skin tag or there may be four or five strands. These are called
ankyloblepharon filiformis adnatum. Medial strands are less common, central are least
common. The strands do not contain any other tissue except skin and few capillaries. The
strands can be cut without difficulty soon after birth.
(c) Distichiasis. This is a rare congenital condition where orifices of meibomian glands
are replaced by an extra line of cilia in addition to normal three or four that are present at the
anterior rounded border of lid. There is an intact inter marginal strip between normal lashes
and distichiasis. The distichiatic lashes grow posteriorly and rub against the cornea and con-
junctiva. It is not uncommon to confuse them with cicatricial trichiasis in adults. In cicatricial
trichiasis, the in turned lashes arise from the anterior margin of the lid. Treatment consists of
repeated epilation when they are a few other wise plastic procedures are required to turn the
lashed forwards.
(d) Entropion. Congenital entropion lids are very rare. They are more common in fe-
males, frequently seen with epicanthicfold, they may be confused with epiblepharon,
distichiasis and trichiasis.
(e) Ectropion. Congenital ectropion is also rare. This is due to vertical shortening of
the lid skin following change in subcutaneous tissue. All the lids may be involved; it is more
common on the lateral side. It may be present as an isolated defect or may be associated with
blepharophimosis, telecanthus, epicanthus inverses or ptosis.
2. Deformity of the Interpalpebral Aperture
(a) Telecanthus. Telecanthus is lateral displacement of medial canthi due to abnormal
lengthening of medial canthal ligament . It is generally bilateral. Normal inter canthal
distance is generally half of inter pupillary distance. In telecanthus this increases pro-
portionately. True telecanthus must be differentiated from hypertelorism which is increased
distance between two orbits Telecanthus may be associated with epicanthus. It is also seen in
Waardenburgs syndrome. Isolated telecanthus does not produce any symptom and does not
require any treatment.
(b) Epicanthus. This bilateral symmetrical congenital anomaly is very common in other-
wise normal infants and disappear with age. It is a distinct feature of oriental races. It is a
consistent finding in Downs syndrome Epicanthus is seen as a vertical semilunar fold of
skin on the medial side of the palpebral aperture with concavety towards the cornea. Accord-
ing to its extent, it can be :
Epicanthus supraciliary when the skin folds starts just below the eyebrow.
Epicanthus palpebralis, here the lid fold starts at the level of normal lid fold this is
the commonest type of epicanthus.
Epicanthus tarsalis, here the lid fold starts at the level of upper border of tarsus.
Epicanthus inverses are same as any epicanthus only difference being that skin folds
starts from the crease of the lower lid.
Simple epicanthus is almost symptom free. the children are brought for cosmetic blem-
ish. Large epicanthus fold causes pseudo convergent squint that disappears on abliterating
the epicanthal fold. In most of the children the epicanthus disappears with age. Ptosis with
epicantus is a common presentation Blepharophimosis, ptosis, telecanthus and epicanthus
inversus represent Komotos Tetrad
42
.
Differential diagnosis consists of epiblebharon where there is no superior palpebral fold
but there may be trichiasis. Epicanthus may be associated with punctal stenosis. There may
be amblyopia due to associated ptosis. Rarely there may be trichiasis.
(c) Blepharophimosis. This is congenital reduction of length and width of
interpalpebral fissure. This is always bilateral and symmetrical. There is no defect in muscles.
Simple blepharophimosis is rare. It is generally associated with epicanthus inversus,
telecanthus and ptosis. The child keeps the chin raised and there are prominent furrows on
the forehead to compensate associated ptosis. Blepharophimosis may give impression of pseudo
convergent squint. It is corrected for cosmetic reasons by oculoplasitc repair, multiple sur-
geries may be required.
(d) Euriblepharon (Euryblepharon). In this case there is uniform enlargement of
interpalpebral fissure, there is displacement of outer canthus laterally. This condition should
not be confused with other causes of enlargement of IPA i.e. lagophthalmos, proptosis, ectropion,
congenital coloboma of lower lid.
3. Congenital Ptosis and Allied Disorders. See page 43.
4. Disorders of the lid skin
(a) Blepharochalasis. This is generally familial bilateral condition where there is at-
rophy of the dermis of the upper lid skin that hangs loose. The orbital fat may protrude in to
the overhanging skin, covering the pupil. It can be congenital or juvenile. The juvenile
form starts between seven to twenty years with episodic attacks of lid swelling
(b) Epiblepharon. This is a congenital condition where there is a horizontal fold of skin
either in the upper or lower lid that runs parallel to the lid margin, lower epiblepharon is more
common. It may be associated with congenital entropion of lower lid.
5. Minor and Rare Deformities
(a) Ablepharon (Cryptophthalmia). A very rare condition in which there is failure of
lid folds to develop in to lid. The skin of the face passes over a deformed eyeball to the cheek
without inter-palpebral aperture.
(b) Microblepharon. In this case the vertical distance of the lid is short both lids, may
be involved. There is mild lagophthalmos. When the patient sleeps a part of the lower bulbar
conjunctiva may be visible.
(c) Mongoloid obliquity of lid is up and outward obliquity of the outer canthus. This is
a constant feature of monogolism.
(d) Anti mongoloid Obliquity is downward displacement of outer canthus.
CONGENTAL PTO88 AND ALLED CONDTON8
A. Congenital ptosis is commonest congenital anomaly of the lid for which a child may
be brought, specially seeking treatment for cosmetic defect. It may be present at birth but not
much attention is given to ptosis, as the neonate keeps the eyes closed most of the time and
normal palpebral fissure is very narrow as compared to adults. However by three months of
age the parents become aware of ptosis especially if it is unilateral. Many a times a child may
reach second decade not being aware of ptosis in mild cases. Congenital ptosis has strong
hereditary tendency.
B. Most of the congenital ptosis are due to mal-development of the levator palpebral
superior. However congenital mal-development of third nerve nucleus or its trunk may be a
contributory factor. In very small number of cases, congenital Horners Syndrome is seen.
Myogenic cause especially neonatal myasthenia may present as ptosis, so may more rare
myotonia.
C. Congenital ptosis can be simple unassociated with other extra ocular muscle palsy
or may be associated with
1. Superior rectus palsy,
2. Superior rectus and inferior oblique under action,
3. Other extra ocular muscle palsy.
4. It can be associated with other deformities of the lid like epicanthus, blepharo-
phimosis
D. Simple congenital ptosis may be unilateral or bilateral. Both the sexes are
equally affected. Generally there is normal vision, accommodation and pupillary reaction. A
miotic pupil with ptosis points towards Horners syndrome. Generally levators function is
moderate to mild in congenital ptosis and remains unaffected throughout life. It is not only the
elevation that is subnormal downward movement of the lid is also defective (lid lag). These
changes are due to dystrophic changes in L.P.S., Lid lag is not seen in any other ptosis
except congenital ptosis.
Though vision is unaffected amblyopia may develop due to
1. The lid covering the pupillary area
2. Associated myopic astigmatism.
3. Associated strabismus.
In orders to have better vision the child uses frontalis, which elevates the lid slightly.
Action of the frontalis can be obliterated by pressing this muscle against the frontal bone. This
procedure is utilised to measure levator function. The use of frontalis by a child with ptosis
throws the forehead skin into deep horizontal furrows and raises the eyebrow in an arched
form. Throwing the head back (chin up) helps to compensate lowered position of lid. Raising of
chin and arching of eye brow develop with age. They are more marked in bilateral cases.
E. Congenital ptosis with superior rectus under action is frequent. Superior
rectus and levator palpebral superior develop from same mesodermal mass hence identical
dystrophic changes may develop in superior rectus in case of congenital ptosis. Involvement of
other extra ocular muscle may be central in origin. The patient may be unable to elevate the
globe with ptosis hence it is mandatory that in cases of ptosis action of the superior rectus
should also be evaluated. Uniocular and binocular movements of all other extra ocular mus-
cles and orbicularis should also be tested along with corneal sensation this has prognostic
importance in surgery.
F. Synkinetic movements of the lid. Paradoxical movement of the lid is generally
present with congenital ptosis. It may be present without ptosis as well
1. Synkinetic movement of lid with ptosis (Jaw winking), Marcus Gunn phenomenon.
This is the commonest congenital paradoxical movement of the lid. This is seen with congenital
ptosis in infancy. The mother notices abnormal movement of the upper lid in an act of sucking.
The paradoxical movement persists for rest of life. It is always unilateral. In primary
position, with mouth closed there is mild ptosis as the patient opens the mouth the lid drifts
upwards. Raised position of the lid is not maintained if the mouth is kept open. If the jaw is
moved towards the side of effected eye, ptosis increases. Sometimes forward protrusion of the
jaw may produce same phenomenon Marcus Gunn phenomenon is seen only on sucking or
chewing, it is not seen on smiling or coughing. The cause of this phenomenon is congenital
misdirection of some of the fibers of trigeminal that supply the pterygoid muscle, to the
oculomotor nerve.
2. Synkinetic movements of the lids, with out congentical ptosis
(a) Movement of the upper lid with contraction of medial rectus
(b) Movement of the lid on contraction of lateral rectus
(c) Raising of upper lid on contraction of inferior rectus i.e. looking down.
ACOURED D8ORDER8 OF THE LD8 N CHLDREN
Acquired disorders of the lids in the children can be
A. Non Specific like as
1. Edema
2. Ecchymosis
3. Change of colour.
B. Specific like
1. Infection
2. Inflammation
3. Injury
4. New growth
5. Vascular
6. Neurological.
A. Non Specific
1. Edema. Edema of the lids is a common feature of lid due to divers cause that can be :
(a) Inflammatory
(b) Allergic
(c) Systemic
(d) Traumatic - Blunt, thermal, chemical, radiation.
(a) The Inflammatory Causes of the Lids may be
(i) Infection of the lids.
Bacterial-lid abscess, cellulitis lid, stye.
Viral-herpeszoster, herpessimplex, chickenpox, measles, molluscum.
Fungal.
Parasitic.
(ii) Infection of globe
Severe uveitis
Endophthalmitis
Purulent conjunctivitis
Panophthalmitis
Epidermic conjunctivitis.
(iii) Orbital cellulitis.
(iv) Cavernous sinus thrombosis.
(v) Proptosis
(b) Allergic. Acute angioneurotic edema, insect bite, dermatitis medicamentosa, atopic
dermitis.
(c) Systemic conditions that produce edema of lids are acute and chronic nephritis,
hypoproteinaemia, myxedema and thyrotoxicosis.
Edema may be acute or chronic. It may involve any or all the lids. It may be very mild
giving a puffy appearance or may be so pronounced as to obliterate inter palpebral fissure
requiring lid retractor to separate the lids. The edema may be transient, manifesting only in a
particular time of the day.the child may get up with edema of the lids that pass off within few
hours. This is generally due to systemic causes requiring pediatric consultation.
Treatment of edema. Treatment of lid edema is essentially treatment of its cause.
The local infective causes are treated by antibiotic - local or systemic and hot fomentation.
Allergic edema may subside with oral and rarely injectable antihistamine. Steroids are indi-
cated in angioneurotic edema.
2. Ecchymosis of the Lid. The lids are very vascular, hence trauma easily causes
extravasations of blood in the lids due to laxity of lid skin. Blood accumulates in the subcuta-
neous space, giving rise to Ecchymosis. The ecchymotic lid is swollen and bluish green in
colour. In infants it may have a red discoloration. As time passes, the colour of lid changes
from red to bluish green to black. Hence called Black eye. The causes of black eyes are:
(a) Blunt injury to the lid, orbit, scalp, middle cranial fossa.
(b) Blood dyscrasiaPurpura, haemophilia, leukaemia.
(c) Neuroblastoma.
Ecchymosis of the lid may be localised to lid only. It may be associated with sub conjunc-
tival haemorrhage with or without rupture of the globe. In injury to scalp, fracture of orbit or
fracture base of skull ecchymosis appears few hours to twenty four hours after the initial
injury as it takes some time for the blood to trickle down along the fascia, muscle plane, or
along the sheath of the extra ocular muscles. Presence of ecchymosis may be frightening but
not always vision threatening and most of the time disappears without treatment. However
ecchymosis of any kind may be just a superficial sign of a deeper malady i.e. fracture base of
skull, blood dyscrasia or neuroblastoma or traumatic optic neuropathy.
Treatment of ecchymosis. Echymosis of lid per se does not require any treatment. As
a first aid cold compress may reduce the swelling. However after few hours cold compress does
not help. Analgesics reduce pain and inflammation. It takes about seven to ten days for simple
ecchymosis to disappear.
3. Colour change of the lid. Generally the colour of the lids is similar to that of face
and forehead. Some times there may be localised colour changes due to ecchymosis which is
temporary and passes off. Birthmarks may last for first few years and gradually disappear.
However some of the birthmarks are permanent like naevus, Sturge Weber syndrome and
neuro fibromatosis. Hypopigmentation is seen following injury burn and vitiligo.
NFLAMMATON OF THE LD
Inflammatory process may involve :
A. The lid margin (Blepharitis).
B. Glands of the lid margin.
C. Skin and subcutaneous tissues of the lid.
Inflammation of the lid margin is called Blepharitis
1,2,3,4
. The Causes of blepharitis
are bacterial, seborrhic, parasitic, atopic, and allergic.
Blepharitis is broadly divided into squamous, ulcerative (staphylococcal) and para-
sitic. The former is mostly seborrhic in nature while later is chronic infection by staphylococci
or due to its toxins.
The squamous (seborrhic) blepharitis
This is basically disorders of gland of Zeis and is associated with seborrhic dermatitis of
eyebrow, scalp and nasolabial fold. The condition is mild. The lid margin has dry scales or
greasy material, which are easily removed and on removal there are no ulcers or hemorrhagic
spots that are common in ulcerative blepharitis. Seborrhic blepharitis may produce mild
chronic conjunctivitis and punctate keratitis in lower part of cornea.
Ulcerative blepharitis
This is more troublesome, produces more complication and is difficult to eradicate. It
may be simple staphylococcal or it may be a combination of seborrhoea and staphylococcal
infection. This makes it difficult to treat. It generally starts after 3 years of age, involves
both the lids on both sides. Involvement of upper lid is more extensive than lower lid. There
are yellow crusts at the base of the lashes that may extend over the rounded anterior lid
margin and the skin. The cursts are difficult to remove, without wetting them. They are best
rubbed off after being moistened by warm water for few minutes. On removal of the crusts, the
underlying skin is found to be hyperaemic, and small ulcers are seen. On long run there
may be tylosis, madarosis and trichiasis. It is always associated with chronic blepharo
conjunctivis and may produce superficial punctate keratitis.
Management of blepharitis is difficult. Treatment of seborrhic blepharitis consists of
management of systemic seborrhoea i.e. regular cleaning of the face, use of suitable antidandruff
shampoo for the scalp and eyebrow. Local instillation of broad-spectrum antibiotic drops, to
prevent secondary infection, for months.
Treatment of ulcerative blepharitis is prolonged. Strict and energetic regime should
be followed which consist of:
(i) Removal of crusts by cotton swab soaked in lukewarm water that is applied on the
lid margin for 2-3 minutes and the crusts rubbed off with dry cloth.
(ii) Broad Spectrum antibiotic is rubbed on the lid margin. The whole procedure is
repeated at least three times a day, same antibiotic is instilled in the conjunctival sac at bed
time, steroid with antibiotic ointment may be rubbed on the lid marginthree times a day
and gradually tapered off.
If children do not respond to local treatment systemic antibiotic may be required for
seven to ten days.
(iii) Personal hygiene. The children should be taught proper hygiene of the face and
hands.
(iv) Associated errors of refraction. Should be corrected after proper retinoscopy un-
der cycloplegia.
Complications of ulcerative blepharitis consist ofTylosis, madarosis, frequent
stye, trichiasis, chronic conjunctivis, keratitis and epiphora.
Parasitic blepharitis
Pediculosis and phthiriasis of lids
14,15
Common parasitic involvement of the lid margin is pediculosis. There are three types
of these arthropods that involve the lid margin. They are pediculosis-corporis, p. capitis
and p. pubis. The child gets the infection due to p. capitis from siblings or playmates, while
they get the other two types from their infected parents, servants or baby sitters. The lid
margin is infested by these parasites which cling to the skin of the lid margin, they lay nits on
the shaft of the lashes. Numbers of parasites vary from a few to numerous. They are visible on
naked eye when many, but may require magnification when few. The parasites produce chronic
itching, redness and secondary infection of the lid margin, conjunctiva and lower cornea.
Treatment. The treatment is mainly treatment of systemic pediculosis in all the contacts.
(i) Local Treatment. In co-operative and older children the parasites may be picked
up with epilation forceps or destroyed by direct application of cryo. Anti pediculosis shampoo
after proper dilution may be applied to the lid margin. This should precede application of any
ophthalmic eye ointment in greasy base. The oily base acts as mechanical repellent for aque-
ous shampoo which other wise may get into the conjunctiva causing chemical conjunctivitis.
20% fluorescein sodium have been used with good result. The drugs commonly used as sham-
poo are benzene hexachloride. (Lindane) 1%, premethrine 1%.
(ii) Follow up. Personal hygiene is the key word in management of pediculosis and
phthiriasis. The cloths, bedding, combs hairbrushes should be washed in boiling water to avoid
re-infection in carriers and infected children. The contacts should also be treated vigorously
and repeatedly for pediculosis.
B. Disorders of glands of lid margin
1. Stye (Hordeolum externum). The stye is an acute staphylococcal infection of
gland of Zeis and Moll. They are very common between five years to fifteen years. Boys and
girls are equally affected. Tendency of stye runs in families. It is a painful condition that
begins as tender red spot on the lid margin. The position of an early stye in the lid can be pin
pointed by moving the finger on the lid margin. The patient winces as the exploring finger
passes over the stye. There is diffuse swelling of the lid on either side of the stye. Styes situ-
ated near the canthi are more painful than in the middle of the lid. Styes of outer canthi are
most painful. The upper lid has more glands of Zeis and Moll, hence styes are more frequent
and more numerous in upper lid. There may be a single stye that may be cured spontaneously
not to appear again. Repeated attacks of styes are very common. There may be multiple crops
of styes. Both lids may be affected. Each lid may have single to multiple styes. All lids may be
affected at the same times. Associated conjunctivitis is frequent. Preauricular lymph nodes
are always enlarged that may be tender. Development of stye after measles is very common.
Other predisposing factors are uncorrected errors of refraction that leads to asthenoipa. A
child with asthenopia has tendency to rub the eyes with fingers frequently. This introduces
infection in the lid margin resulting in repeated styes that disappear with correction of errors
of refraction. Other predisposing factors are chronic seborrhic and staphylococcal blepharitis .
Management of Stye
Management of stye consists of
(i) Treatment of stye.
(ii) Prevention of recurrence.
(iii) Management of complications.
Treatment of Stye
The treatment is similar to treatment of a boil that consists of local dry hot fomenta-
tion, systemic analgesic, anti-inflammatory drug and systemic antibiotic. Local anti-
biotic eye ointment at bedtime prevents matting of lid margins at night. With above treatment
either the stye subsides by evacuation of pus or the lash round which the stye has developed,
pus is epilated.
Prevention of recurrence
As error of refraction is one of the important predisposing factors, it should be corrected
by appropriate glasses following refaction under cycloplegia. Stye itself can induce slight
astigmation hence refraction should be under taken only when the child is free from stye.
Proper lid hygiene should be maintained. Cleaning the lid margins and application of
broad spectrum antibiotic eye ointment at night for at least two months after the stye has
subsided.
In chronic and recurrent stye doxycyclin 100mg daily for ten days gives relief. How-
ever doxycyclin is not preferred in children under eight years as it may cause permanent
staining of the teeth. In such children long acting sulpha or erythromycin in consultation
with pediatrician should be given.
Cutting of consumption of sweets like candies, chocolates and supplementation of food
with multi vitamin orally helps in reduction of recurrence. Children should be instructed to
keep the hands clean and nails trimmed.
2. Chalazion. In contrast to stye, chalazion is a chronic granulomatous inflamma-
tion of meibomian glands. Otherwise chalazion share same epidimiology as stye i.e. it is a
disorder of children and young adults and it is equally common in both sexes, any or all lids
may be involved separately or simultaneously. There may be repeated crops of chalazia. It is
more common with uncorrected errors of refraction. It differs from stye in following points:
It is chronic granuloma, neither painful nor tender, preauricular glands are not enlarged.
The chalazion is heralded by changes in the duct of the meibomian gland which are
obstructed due to infection spreading from the lid margin. This results in stagnation of oily
meibomian discharge in the acini of the glands. This pent up lipid acts as an irritant that
result in a granuloma with giant cell without caceasion. The chalazion is well circum-
scribed and smooth without a true capsule of a cyst.
The skin over the chalazion can be moved. On evertion of the lid the conjunctiva over
the chalazion shows a bluish coloration.
There may be multiple chalazia in the same lid or in all the lids. Chalazion is a self-
limiting inflammation. Small chalazion may resolve without any treatment or simply by fre-
quent hot fomentation. If it does not resolve by it self-following changes may be seen:.
(i) The growth increases in size and the central core may liquefy forming a tarsal cyst
that may cause pseudo ptosis and induce astigmatism.
(ii) It bursts on
(a) Most commonly on the conjunctival surface, giving rise to a sessile papillomatous
granuloma.
(b) It may less frequently rupture on the skin surface.
(c) Rarely it may protrude through the opening of meibomian orifice. Chalazia in
children never undergo neoplastic change. Though meibomian cell carci-
noma after 40 years may initially present as chalazia.
(iii) The chalazion gets secondarily infected and becomes severely painful. It is called
Hordeolum internum. Sometimes chalazion may be associated with stye as well.
Management of chalazia
21,22
Many of the chalazia resolve without treatment. However if a chalazion persists it re-
quires surgical drainage and curettage of the granulomatous tissue followed by cauterisation
of the wall of the growth by carbolic. In children chalazia should be incised under general
anaesthesia.
Intra lesion injection of steroid: 10mg of triamcinolone acetonide is injected in the
chalazion under local anaesthesia from conjunctival surface. A patient may require two to
three such shots.
Hordeolum internum requires more energetic treatment that consists of dry hot
fomentation, systemic analgesic and anti-inflammatory drugs, systemic broad-spec-
trum antibiotic. No attempt should be made to drain the acute chalazion unless pain and
inflammation has subsided.
Prevention is similar to that of stye i.e. hygiene of lids, face and hands, correction of
errors of refraction, long term local application of broad spectrum antibiotic on the lid margin.
Differential diagnosis of burst chalazion consist of :
Foreign body granuloma.
Papilloma.
Haemangioma.
Rhinosporidiosis of tarsal conjunctiva.
C. Cutaneous and subcutaneous inflammation of the lids
All infective processes that involve the skin of the face and forehead can affect the lid.
They can be viral, bacterial and fungal.
1. Viral infection of the lids are :
(a) Chicken pox
(b) Measles
(c) Molluscum
(d) Herpes zoster
(e) Herpes simplex
All the above conditions cause eruptions on the lid, may involve the conjunctiva, as well
as cornea. Herpes zoster is classically unilateral others are bilateral all the above condi-
tions have or may have systemic involvement.
(a) Chicken pox (Varicella)
16,18
is very common exanthematous disorder of the lid
caused by varicella zoster virus, it starts as hyperaemia with vesicle formation, which subside
within 10 to 12 days leaving no scar, the vesicles can be seen on the lid margin as well, may
develop in conjunctiva simulating phlycten. There may be ulceration or vesicles on the con-
junctiva, may produce superficial keratitis pseudo dendretic keratitis and disciformkeraitis.
Frank ulcer is not uncommon. If there is involvement of nasolacrimal duct it may lead to
chronic dacryocystitis
4
.
Treatment. There is no known method to immunise the child against chicken pox,
there is no specific systemic treatment. The child should be observed for possibility of pneumonia
or encephalitis. Antiviral drugs have not proved to be effective. Immunoglobulins have
favourable result when started with in three days of onset of skin rashes.
Ocular management. Consists of relief of photophobia and watering due to corneal
involvement. Antibiotics are used to prevent secondary bacterial infection. The child should
get full dose of cycloplegia, if needed atropine may be used.
(b) Measles (Rubeola, Morbilli)
23,24
. Measles is very common among non-immunised
children. It mainly affects the respiratory tract and conjunctiva. The lid it is involved as part of
generalised hyperaemic rashes that become papules, these are slightly raised and red. They
may be separate or merge with each other. Conjunctivial involvement is universal; it starts as
catarrhal conjunctivitis that may be superimposed by secondary infection. Koplic spots de-
velop on the conjunctiva, there may be sub conjunctival haemorrhage. corneal involvment is
common causing blepharospasm. There are various stages of epithelial keratopathy, frank
corneal ulcer develop in malnourished children. Blindness due to measles can be as high as
1% of all children affected mostly due to bacterial infection, malnutrition and hypovitaminosis
A. There may be edema of the lid, cellulitis lid, multiple styes and blepharitis.
Management
Treatment is symptomatic. All parents should be encouraged to get children immunised
against measles. It is one of major cause of depletion of vitamin A and not only vision threaten-
ing but potentially fatal.
1. Compulsory immunisation is essential not only to save the eye but also life of the
child.
2. Administration of additional does of water-soluble vitamin A 50,000 I.U.daily for four
days orally.
3. Bland lotion to prevent sticking of eyelids.
4. Broad spectrum antibiotic drops for conjunctivitis
5. Cycloplegie if cornea is involved.
(c) Molluscum contagiosum. This is a chronic self-limited virus disease of skin caused
by poxvirus. The lesions may appear any where on the body as dome shaped, translucent
vesicles, which are umblicated, pain less, not associated with fever or any other systemic
manifestation. They are not as numerous as eruptions of chicken pox, or small pox, number
may vary from few to few dozens all over the body. May be seen on the lid or lid margin or both.
Some times they are seen on conjunctiva or cornea. Corneal involvement causes redness,
watering and photophobia due to pseudo dendritic ulcer, pannus formation and sub epithelial
infiltration. When the lesion is near the puncta it may block the puncta causing epiphora.
Keratitis and follicular conjunctivitis are toxic in nature.
Treatment. The disease is self-limiting but may last for weeks. It is contagious. There
is no known immunisation, anti viral drugs are of not of much use. They are best treated by
puncturing each lesion either by needle or electrocautery, the interior of the punctured lesion
is curetted by tincture iodine or weak solution of carbolic. The lesion can be treated with cryo
as well. Corneal involvement may require broad spectrum anti biotic drops and cycloplegia.
(d) Herpeszoster Ophthalmicus
18,19,20
. Herpeszoster ophthalmicus is an acute erup-
tive condition of the lid caused by zoster varicella virus that causes chicken pox otherwise.
It is morphologically similar to herpes simplex virus but differs in antigen. Herpes zoster
ophthalmicus can occur at any age, however it is less common in children. Children toler-
ate pain of herpes zoster better than adults, may be the pain is less sever in children. Children
are not known to suffer from secondary zoster that is seen in immuno compromised adults.
Herpeszoster ophthalmicus may be mild or may be very severe. It is known to affect all
parts of the eye and adnexa except the lens.
In herpes zoster ophthalmicus the infection lies dormant in posterior root ganglion of
trigeminal nerve following systemic infection either in the form of chicken pox or zoster
itself and then travels down the first division of the fifth nerve to reach the eye and adnexa.
Herpes zoster ophthalmicus starts as hyperaemic rash over the fore head and skin of
the lid or lid margin. May be associated with mild fever and malaise. The eruptions are initially
macular and soon converted into vesicles similar to chicken pox. Generally facial pain
precedes appearance of rashes. The vesicles are as a rule always unilateral they do to cross
the mid line however associated edema of the forehead, lid and face may spill over the mid line
giving a false impression of allergic edema or bacterial cellulitis. Preauricular lymph nodes
are enlarged. Like chicken pox the vesicles take ten days to dry and crust. The crust fall in due
course leaving shallow scars that may disappear in time. If the lesion was deep it may leave
permanent scar on the forehead, lid or lateral side of the nose. Scar of the lid margin may
cause notching of lid margin or trichiasis. If the vesicles are present on the tip of the nose
it means that nasocillary nerve is also involved. This is called Hutchinson sign. This is
almost sure indication of corneal involvement. However cornea may be involved without
involvement of nasociliary nerve.
Lesions of herpes zoster may be divided into
(i) Involvement of lid and conjunctiva
(ii) Involvement of globe
(iii) Neurological lesions
(iv) Post herpetic complications
(i) Lids may show very mild hyperaemia to server vesicular eruption and edema of the
lid obliterating interpalpebral fissure.
The vesicle may develop along the distribution of one or all the branches of first division
of fifth nerve. Involvement of maxillary division is rare. In initial stages herpes zoster of lid
may be confused as allergic edema, insect bite, stye or bacterial cellulitis
Conjunctiva may show vesicles, chemosis and subconjunctival haemorrhage. It is very
common to develop secondary bacterial conjunctivitis.
(ii) Involvement of the globe
Corneal involvement. Involvement of cornea is very common. It may happen
without involvement of nasociliary nerve, its severity is not directly proportion-
ate to lid involvement, server corneal involvement may be seen with mild lid
involvement. Corneal involvement is varied it may be :-
Epithelial punctate infiltrates that stain better with rosebengal, microdendrites
filamentary keratitis
Diminished corneal sensation
Numular keratitis
Disciform keratitis
Corneal involvements do not respond much to anti-viral drugs but respond to
local steroids. Numular keratitis is known to change density from time to time
without treatment.
Scleral involvement. Scleritis is less common than keratitis it may be missed
due to lid edema and conjunctivitis. Scleritis is less common in children.
Uveitis. Anterior uvea may be involved either as primary inflammatory process
of or secondary to keratitis. Forty percent of eyes with herpes zoster develop
non-granulomatous iridocyctitis. It may be mild with few kps or may have
blood tinged hypopyon due to is ischemic necrosis of iris. atropthic patches on
the iris that transilluminate and causes irregular semidilated pupil is also caused
due to is ischemic necrosis of iris. Such atrophic patches are not seen in herpes
simplex, which is also associated with keratouveitis. There may be chroiditis as
well as chorioretinitis.
Secondary Glaucoma is due to associated trabeculitis and clogging of trabecular
meshwork by inflammatory cells. Chronic anterior uveitis and secondary glau-
coma may predispose cataract formation.
(iii) Neurological lesions may cause encephalitis and cranial nerve palsy. Com-
monest being third nerve but any or all the extra ocular muscles may be affected due to in-
volvement of their nerve supply. Optic neuritis is rare complications.
Post herpetic complication
Post herpatic neuralgia is a common feature, fortunately it is milder and short-lived
in children. Children tolerate post herpetic neuralgia better than adults.
Neuro tropic keratitis, diminished corneal sensation start from very early phase of
corneal involvement, generally recovers within a few months but in some cases it may last for
years leading to neuro tropic keratitis requiring tarsorrhaphy.
Delayed lid Changes. There may be hypo pigmentation or hyper pigmentation
with scaring of the skin, trichiasis is common, there may be notching of the lid margin.
Loss of sensation may last for months.
Other changes. Mucus secreting conjunctivitis, corneal mucus plaque, neurotrophic
perforation of cornea, recurrent episcleritis and scleritis, herpes oticus (Ramsay Hunt Syn-
drome)
18
herpes of palate.
Management
Management consists of reducing pain, associated inflammation, control of virimia, pre-
vention of secondary bacterial infection, management of uveokeratitis and its squeals.
Analgesia. Children tolerate pain better, however water soluble non-steroidal anti
flamatory drugs helps to over come pain, edema and inflammation.
Antiviral. Antiviral drugs reduce lid inflammation pain and shorten duration of dis-
ease but does not affect keratitis. Its role in zoster uveitis is not established. It is claimed to
reduce post herpetic neuralgia.
Systemic steroids. Definitely reduce pain inflammation and scaring. However it should
be given along with systemic antiviral.
Local steroids are required for uveo keratitis, the eyes receiving local steroids should
be stained frequently, least secondary bacterial infection gets an upper hand in an already
anaesthetic cornea. Bland lotion or local antiviral ointment is prescribed for lid vesicle. Local
antiviral drops or ointments are given along with local steroid in uveo keralitis.
(e) Herpes simplex
25
. Herpes simplex infection is very common infection caused by
virus: Herpes hominis. There are two types of herpes simplex i.e. type one that involve the
body above the waist and type two that cause lesions below the waist more commonly known
as genital herpes, which is a sexually transmitted disease. Herpes simplex can occur as:
Neonatal infection, which is due to type two herpes simplex virus transmitted via
birth canal of infected mother. If it is known that the mother has genital herpes simplex it is
better to deliver the child by caesarean section. Neo natal herpes simplex may present as few
vesicles on the lid or conjunctivitis in the neonate. Neonatal herpes simplex is life threat-
ening condition when server.
Primary ocular infection: commonest age to get ocular infection is between six months
to six years. The lids develop cluster of vesicles without predilection for any particular nerve.
The vesicles may be bilateral in contrast to zoster, which is unilateral. The vesicles rapidly
develop crust and disappear without scaring. It may be associated with acute folicular con-
junctivitis. Half the patients develop harpes simplex keratitis in the form of fine or coarse
epithelial keratitis most of the corneal lesions heal without scar. Sometimes it may progress
to disciform keratits.
Recurrent ocular infection. After primary infection the virus travels to the trigeminal
ganglion and lies dormant unless a triger mechanisation stimulates the virus to reach the
target organ after a latent period that varies from person to person. That may be few months
to years Recurrent herpes simplex involves cornea and uvea and not the lids. Sever form of
herpes simplex requires pediatric consultation for management.
2. Bacterial infection of the skin of the lid
Boil, cellulitis and abscess of lids. Skin of the lid, like any other exposed part of the
body, is prone to get infected. It may start in the hair follicle and result as a boil or may be
extensive to cause cellulitis. As the skin of the lid is thin, very lax and very vascular, lots of
exudates and pus can accumulate under this and ultimately result in abscess formation.
Commonest organism is staphylococcus. Haemophilus is an important cause of lid abscess
in children. Lid abscess starts as diffuse swelling of the lid. Initially the lid is tense and red. It
may obliterate the inter palpebral fissure. Gradually the skin becomes lax and fluctuation
develops, pus may point at a dependent part. It is more common in hot seasons, cellulits lid
and lid abscess are preseptal hence the globe is neither involved nor proptosis is produced.
Differential diagnosis consists of cavernous sinus thrombosis, retrobulbar haemorrhage
retro ocular mass, rhabdomyosarcoma, multiple styes, infected chalazion, pre-eruptive
stage of herpes zoster.
Treatment consists of
1. Systemic broad-spectrum antibiotic preferably by injection.
2. Hot fomation.
3. Analgesic, anti-inflammatory drugs to reduce pain and inflammation.
4. If pus points or fluctuation develops, pus should be drained by a stab incision.
TUMOUR8 OF LD N PAEDATRC AGE GROUP
26,27,28
Tumours of the lid in all ages could be both congenital as well as acquired; they can
be benign or malignant. All lid tumours are difficult to manage satisfactorily. They have
been classified as
A. Those involving superficial layers of lid.
B. Those involving deeper layers of lid.
Other classification is according to structure of the lid (in all ages).
A. Epithelial
1. Benign
(a) Papilloma.
(b) Squamous cell hyperplasia.
(c) Pseudo cancerous.
(d) Kerato acanthoma.
(e) Seborrhic keratosis.
(f) Inverted follicular keratosis.
2. Precancerous
(a) Active keratosis
(b) Intra epithelial epithelioma.
(c) XERODERMA PIGMENTOSUM.
3. Malignant
(a) Basal cell carcinoma
(b) Squamous carcinoma
B. Melanocytic
1. Benign
2. Malignant
C. Tumours arising from glands of lid
Meibomian cell carcinoma.
D. Vascular
1. CAPILLARY HAEMANGIOMA
2. NAEVUS FLAMMEUS
3. CAVERNOUS HAEMANGIOMA
4. STURGE-WEBER SYNDROME
E. Neural tumours : NEUROFIBROMATOSIS
F. Others
1. LYMPHANGIOMA
2. JUVENILE XATHOGRANULOMA
Condition in capital letters mentioned above are found under 15 years of age and con-
tinue to persist in later age group if not treated. All the other tumours are not seen in children.
Some of them may be congenital others develop at puberty. Some are self-limiting. Except for
neurofibroma and xeroderma pigmentosa none show malignant changes in children.
PHACOMATO88
29,30
Phakomas are a group of tumours, which are congenital in nature, may be present at
birth or manifest later. They have strong hereditary predisposition some of them may be spo-
radic. The sporadic cases can pass the gene to the next generation, they may be localised to the
lid or may involve orbit, or globe, may have intra cranial extension or systemic involvement.
All of them are Hamartomas i.e. tumours arising from the tissue components that are nor-
mally found at the involved site. They are seen in all races and equal in boys and girls. In fact
they are congenital anomalies that result in tumour like malformations.
PHAKOMAS
A. Neurobibromatosis (von Reckling Hausens disease)
B. Encephalo facial angiomatosis (Sturge-Weber syndrome)
C. Angio matosis retinae (von Hippel-Lindus disease)
D. Tuberous sclerosis (Bournvilles disease)
E. Arteriovenous malformation of retina and brain (Wybern Mason syndrome)
F. Ataxia talengectasia (Louis Bar Syndrome)
G. Cavernous haemangioma of retina, skin brain (Rendu-Osler-Weber syndrome)
A. Neurofibromatosis
Neurofibromatosis is one of the most common congenital phacomas of the lids. It may be
as common as one in every three thousand live births. It can be inherited as autosomal
dominant trait. Either of the parents or both may show clinical evidence of disorder or may
have subtle changes. Spontaneous mutation may lead to sporadic cases. These sporadic cases
may pass the gene to the next generation. Genetically and clinically there are two types of
neurofibroma i.e. type I and II commonly known as NF I and NF II.
NF II cause bilateral acoustic neuroma besides usual features of neurofibroma.
The following components may be seen alone or in various combinations, in neuro-
fibromatosis
1. Skin and lids changes
2. Ocular changes other than lid changes
3. Involvement of nervous system
4. Visceral changes.
1. Skin and lid changes. The changes may be present at birth but generally develop
by five years of age. They become marked at puberty. The changes may be seen any where on
the body as :
(a) Cafaulait spot. These are slightly raised hyper pigmented spots of variable size
i.e. pin point to large areas of pigmentation, without sensory loss, they may cross over the mid-
line six hyper pigmented spots or more than five millimetres is significant.
(b) Fibroma molluscum. These are pedunculated mass of various sizes any where on
the skin including lid.
(c) Plexiform neuro fibroma. These are most disfiguring growths that are due to
involvement of multiple superficial nerves which on palpation feel like a bag of worms. It is
most commonly seen in the lid, the whole of the lid is involved and thickened including
conjunctiva, the inter palpebral fissure is obliterated initially the lid acquires a S shaped
Curve gradually the lid margin develops a convexity down wards, the margin may over hang
the lower lid. It may be large enough to be called elephantiasis neuro fibromatosa. There
may be hypertrophy of the skin of the face on rare occasion the condition may be bilateral.
2. Ocular involvement. Both neuro ectodermal as well as mesodermal structure are
affected. In fact all parts of the eye except the lens may be involved.
(a) Conjunctiva. Palpebral conjunctiva may be involved as part of involvement of the
upper lid. The bulbar conjunctiva and lower formix may show small nodules between 1mm to
3mm. These are firm non-tender nodules. Otherwise multiple tortuous nerves may be visible
on the conjunctiva.
(b) Cornea. Prominent corneal nerves may be visible on bio microscopy.
(c) Uvea. Commonly involved uveal tissue is iris that may have multiple hyper-pigmented
nodules similar to nevi. Chloroid and ciliary body may show localised thickening of the nerve
fibers.
(d) Optic nerve. Congenital opaque nerve fibers are more common in persons who
have evidence of neurofibromatosis. One fifth of optic gliomas are seen in neurofibroma.
Neurofibroma may extend into the chiasma and have positive x-ray finding of pre-chiasmal
bony destruction.
(e) Retina is less commonly involved.
(f) Glaucoma is very common with neurofibroma of the lid. Exact mechanism of glau-
coma is not well understood. It is termed as associated congenital glaucoma, which has all
the features of buphthalmos that may be obscured by pseudo ptoisis. The pathology may be
due to presence of neuro fibroma at the angle, fibro vascular closure of angle or due to forward
displacement of lens iris diaphragm due to growth of ciliary body. Treatment of glaucoma in
neurofibroma is unsatisfactory.
(g) Orbit. Bony faults in the sphenoid may lead to pulsating exophthalmos. Other-
wise there may be proptosis due to retro bulbar mass with enlargement of orbit.
3. Nervous system involvement. Generally there are multiple neurofibromatous
growths in the brain, meningies, cranial nerves specially bilateral acoustic neuroma.
Spinal nerves may be involved with involvement of spinal cord. There may be changes in
the bony spine. Even autonomic nervous system may be involved.
4. Visceral involvement. Any of the viscera can be involved in neurofibroma.
Pheochromocytoma has been reported more commonly in neurofibroma. There may be go-
nadal changes.
Management
There is no known treatment to eradicate neurofibromatosis. Treatment is mostly symp-
tomatic minor defects can be treated with plastic reconstruction. If possible amblyopia should
be treated. Glaucoma may be managed medically or surgically.
B. Encephalo trigeminal syndrome (Sturge-Weber syndrome)
This congenital hamartomous anomaly differs form other phacomatoses by not being
hereditary. It is seen in all races, both boys and girls are equally affected. It is generally
unilateral but in ten percent of cases it can be bilateral. It has following components.
1. Skin and lid involvement.
2. Ocular involvement.
3. Central nervous involvement.
4. Visceral and other involvement.
1. Skin involvement is present at birth as port wine stain. The facial angiomatosis
that frequently involves both lids may be localised or have extensive involvement of not only
facial skin but also the trunk. The size and shape of the cutaneous angioma does not change
with age. Some times only dilated conjunctival vessels can be seen in the epsilateral side.
Generally there is hemiatrophy of the face on the affected side. Sturge Weber syndrome does
not cause ptosis which is a frequent feature of neurofibroma.
2. Ocular involvement. Besides lid, the globe too has angiomatous malformation mostly
in the uvea that result into heterochromia of iris, choroidal haemangioma and associ-
ated glaucoma. The choroidal haemangioma is generally single, large and situated in the
posterior pole on the temporal side of the disc. The haemanagiomas have diffuse out line, they
are raised, have orange hue may be mistaken as amelanotic melanoma of choriod. The
hemangioma due to its proximity to macula may cause cystoid macular edema. There may be
non rhegmatogenous retinal detachment that may lead to secondary glaucoma in an eye that
is already predisposed to glaucoma.
Glaucoma About 30% of eyes in Sturge Weber syndrome develop glaucoma by second
year. A clinical feature of glaucoma is similar to congenital buphthalmos. Including enlargement
of globe, large cornea, normal or deep A.C. Stretching of the globe may sub luxate the lens.
The most widely accepted theory of raised intra ocular tension is increased episcleral pressure.
Other probable causes are malformation of angle, peripheral anterior synechea. Sturge Weber
syndrome glaucoma can be controlled by medical treatment, however if the medicines fail to
reduce pressure below episcleral pressure these patients reqire surgical treatment.
Trabeculectomy may help. Common complications of glaucoma surgery are intra operative
hyphaema and large chroidal effusion in post-operative period.
3. Central nervous system involvement. Intra cranial haemangioma of the tapeto
meninges on the same side as that of facial angioma is a constant feature. The angiomas and
underlying cortex develop calcification that shows up as tram track appearance on x-ray of
the skull. Intra cranial calcification develops in half of the cases by second year of life. The
angioma cause Jacksonian epileptoform attack on the contra lateral side, which is difficult of
treat medically. There may be contra lateral hemipersis. Various degree of mental retardation
is also known to take place. There may be atrophy of ipsilateral cerebral and cerebellar cortex.
Homonymous hemianopia is frequent.
C. Angiomatosis retina
Angiomatosis of retina is a hereditary hamartomatous disorder of retinal vasculature
and optic nerve. It is associated with haemangioblastoma of central nervous system and
viscera. In the eye it involves retina mostly and optic nerve head less commonly. It is a
autosomal dominant disorder, other parts of the globe orbit or adnexa are not involved.
Ocular Involvement. Retinal involvement starts first, it is generally diagnosed in
first decade either due to diminished vision or on routine examination. The angiomas are
generally multiple, of different sizes. There are mainly three locations where these angiomas
are seen, peripheral, peripapillary and papillary. The angiomas are progressive and keep
on increasing in size and number. Even after treatment recurrence in the same quadrant is
possible. The peripheral lesion arise as endophytum i.e. they arise from inner retinal layer
while peripapillary lesions arise from outer retinal layers and are diffuse. The early angiomas
are small but have evidence of arterio venous shunt on fluorescein angiography. Larger
angiomas are round elevated bright red mass that have a feeding artery and draining vein
both are dilated, tortuous and of same caliber. The disc angiomas are similar but do not develop
arterio venous shunt. As the angiomas grow. sub retinal fluid and exudates accumulate round
the lesion. The exudate may accumulate under the macula forming a macular star and loss of
central vision. The endophytic tumours may bleed leading to vitreous band formation and
traction detachment. The optic nerve angioimas when situated in the substance of the optic
nerve head may present as disc swelling. Recurrent haemorrhage, rhegmatogenous retinal
detachment may lead to secondary glaucoma.
Systemic involvement. Central nervous system involvement occurs after ocular
symptoms. Almost all parts of the brain and spinal cord may develop haemangioblastomas.
Similar growth can be seen in liver, kidney, and pancreas. All children with suspected
angiomatosis retinae should undergo complete systemic examination including ultra
sonography C.T. scan and MRI.
Management
Treatment consists of obliterating the retinal angiomas as early as possible. This can be
achieved by argon laser, cryo or penetrating diathermy. Argon laser is better for posterior
polar lesion while cryo is more effective in peripheral lesions. Argon laser therapy should have
large spot, low intensity and long duration; multiple treatment sessions may be required.
D. Tuberous sclerosis (Bournvilles disease)
This is a congenital hamartoma that differs from neurofibromatosis and Sturge We-
ber syndrome. It does not involve any ocular structure except retina and optic nerve.
It is a fatal condition. The children do not live beyond second decade. It is a hereditary condi-
tion all races and both sexes are equally affected. The ocular lesions are bilateral may be a
symmetrical the components are :
1. Cutaneous,
2. Cerebral,
3. Ocular,
4. Visceral.
The Cutaneous involvement. The Cutaneous involvement is not present at birth but
becomes visible between two to five years of age as ash-leaf area of depigmented patches on
the trunk and limbs best seen by Woods ultra violet lamp. This is followed by so called
adenoma sebaceum, that does not involve sebaceous glands, are in fact angiofibromas
histologically. Adenoma sebaceum are distributed on the face as wings of butterfly on either
side of the mid line beyond naso labial fold and nose they are multiple small redish brown
nodules. The nodules increase size at puberty, dilated vessels are common over the nodules.
Central nervous involvement. Central nervous involvement is wide spread in the
brain. It may be cerebral; cerebellar or medullary may be seen in ventricles. It may involve the
spinal cord. In fact any part of the brain and spinal cord may be involved. They may cause
raised intracranial tension, convulsion and mental retardation. The lesions get calcified and
are visible on x-ray.
Ocular involvement. Ocular involvement consists of hamartoma of retina that may
be single or multiple generally raised and globular or may be flat. Optic nerve changes are
not hemartomatous they are due to drusen that is very common with tuberous sclerosis.
Visceral Involvement
Rounded hamartomas can be seen in almost all organs including heart, which may be
the cause of death. Fibromas may be seen under or by the side of the nails. There is no known
treatment for this condition.
PRE CANCEROU8 EPTHELAL LD TUMOUR N CHLDREN
Xerodermapigmentosum
31,32,35
Xeroderma pigmentosum is relatively rare precancerous disorder of ectodermal and
mesodermal structure with prominent extra ocular involvement. Intraocular structures
are not involved.
It is an autosomal recessive disease where both the sexes are involved equally. It may
affect siblings. The disease is first noticed by second year of life when the child shows signs of
intolerance to sunlight. It is due to hypersensitivity to ultraviolet light between 320 nm to
340 nm. The cutaneous signs are : Erythema in all exposed parts of the skin. The skin lesion
undergo a chain of changes i.e. erythema bullae formation, development of fine vessels on skin
lesions, atrophy of the lid, benign multiple hyperpigmented growth ending in development
of various types of diffuse malignant growths that may cause death in adolescence. Various
types of malignanies noted are epithelioma, basal cell carcinoma, even malignant
melanoma. Ocular structures commonly involved are: lids, conjunctiva and cornea.
Lids contain various stages of growth ranging from erythema, hyperpigmented nodules
to malignant growth. There is atrophy of skin of the lid; ectropion of lower lid is common.
The Conjunctival involvements include nodular growths near limbus, pigmented
patches, papillomas dryness of conjunctiva, formation of pterygia and symblepharon. Both
eyes are involved, bulbar conjunctiva in the inter palpebral fissure is first to be effected as it is
exposed to ultra violet rays more than other parts of the conjunctiva.
Corneal involvement is early and may be first to draw attention of the parents due to
photophobia, lacrimation and redness of the eyes. Corneal involvement may start as early as
two years in severe cases, or may be delayed by another two to three years. Whole of the
cornea may be involved. Corneal involvement starts as superficial vascularisation of lower
part causing intense lacrimation and photophobia. The vessels regress leaving a permanent
opacity. Gradually whole of the cornea may become opaque. Other form of corneal involve-
ment is extension of conjunctival neoplasm over the cornea.
Management of xerodermapigmentosum is almost hopeless due to high mortality.
There is no way to prevent progress of disease. Treatment is palliative aimed to give maxi-
mal relief from sensitivity to ultra violet rays. Parents are instructed to keep the children
away from direct sun-rays. All the parts of the body are covered by long sleeved shirts and
trousers, cover the feet by fully covered shoes, keep long hair, use sun glasses with side protec-
tion. Skin protective lotion containing zinc oxide, titanium oxide and para-amino benzoic
acid are smeared over the exposed parts. Malignant growths require separate, specialised
treatment.
HAEMANGOMA8 OF THE LD
Haemangiomas of the lid are commonest benign tumours of the lid. They are of three
types :
A. Capillary haemangioma
B. Cavernous haemangioma
C. Nevusflammeus
A. Capillary Haemanagioma
34,35
Capillary haemangiomas are commonest types of congenital tumours of the lid, they are
seen one in every 200 live births. They may be present at birth, as a red spot on the lid and
extend on the forehead generally in the mid line the lesions become prominent when the child
cries. The growths are more common on upper lid on the medial side than lower lid. They
have a typical evolution. They become manifest most commonly after first month and keep
on progressing for six months to one year and then start regressing by third year. Only
few will remain visible after five years, they appear as strawberries of irregular raised mass
that do not have formed capsule on histology. As most of the tumours regress spontaneously,
so they do not require any treatment except reassurance to the parents who themselves may
had such lesions in their infancy. If the growth is large it may cause mechanical ptosis
producing deprivation amblyopia. The growth by itself can cause astigmatism,
anisometropia and result in amblyopia.
If the growth is large it may require treatment.
Medical treatment consists of oral prednisone 2mg / kg / day, better given in single
dose alternate day, most children show significant regression within ten days, then the dose
can be tapered. However in some cases reduction in dose may halt regression. Whenever the
child is on systemic steroid its benefit should be weighed against side effects. Injection of
depot steroid in the lesion is claimed to be as effective as oral treatment but less toxic.
Other modes of management
Laser. various types of lasers i.e. argon NdYAG and carbon dioxide laser have been
tried without uniform result.
Irradiation. Ortho voltage radiation of 200 rads may regress the growth in two weeks.
However proper protective shields should be used during radiation to protect the globe and
periorbital tissue.
Surgery. Various types of surgical procedures are available but may not be very effective.
B. Cavernous Haemangioma
Cavernous haemangiomas are far less common than capillary haemangiomas in chil-
dren. They are mostly orbital, single and well capsulated, are more common in females, that
manifest at later years of adolescence. They do not have prominent arterial supply which is
present in capillary haemangioma. It too has tendency for spontaneous regression. It may get
calcified.
C. Nevusflammeus
This is flat cutaneous lesion of the lid that does not blench on pressure. It has a purple
blue hue hence the name port wine stain. It may be seen separately or in association with
Sturge Weber syndrome. It does not require any treatment except cosmetic in fair skinned
children when it is too large.
JUVENLE XANTHOGRANULOMA
This dermatological condition of infancy and childhood is of importance not for its
involvement of lid or skin but it causes spontaneous uniocular recurrent hyphaema due
to involvement of iris. The skin lesions are multiple yellowish, benign tumours that show
spontaneous regression. Intra ocular lesions are seen in iris and ciliary body, as pigmented
single lesion or may have a salmon colour. Hyphaema is self-limiting but may cause secondary
glaucoma.
THE NEUROLOGCAL D8ORDER8 OF LD
The two most common neurological disorders of lids in children are
A. Neurological ptosis See Chapter 4, Page 73-76.
B. Lagophthalmos
B. Lagophthalmos
36
Inability to close the lids fully is called lagophthalmos, in an attempt to close the lids
the eye ball rolls up due to protective mechanism of Bells phenomenon. Lagophthalmos can be
due to
1. Neurogenic cause or
2. Mechanical cause.
1. Neurogenic. Neurogenic cause of lagophthalmos is paralysis of motor supply to
orbicularis oculi. The lesion is mostly peripheral in the facial canal or its peripheral branches.
Supranuclear lesion of seventh nerve produces total contralateral weakness of lower two third
of the face with slight weakness of orbicularis. Weakness of orbicularis in supra nuclear sev-
enth nerve palsy may be so mild as to go unnoticed. It does not require tarsorrhaphy, which is
frequently required for peripheral seventh nerve lesion.
Neurogenic lagophthalmos is generally unilateral but can be bilateral, Facial diplegia
37
is almost always central in origin, common cause in children are head injury, Mobius
syndrome that consists of bilateral sixth and seventh nerve palsy, palatal or lingual palsy,
deafness, syndactyly, polydactyly, absent fingers and toes, Vestibular nystagmus that can not
be elicited by caloric test.
Rosenthal Malcarson
37
syndrome is a rare condition where there is bilateral infra
nuclear facial palsy with furrowed tongue. Leprosy is a common cause of bilateral
lagophthalmos in adults. It takes minimum 10-15 year for leprotic lagophthalmos to develop
hence it is not seen in children.
2. Mechanical causes of lagophthalmos prevent the lids to close over the globe,
even when lid closure is achieved the lids gradually separate to expose the anterior part of the
globe. The mechanical cause may be in the orbit or in the lids.
The orbital causes are: Proptosis and exophthalmos. Buphthalmos, anterior
staphyloma or large ciliary staphylomas do not obstruct closure.
Palpebral causes are Large coloboma of the lids, mostly upper lid, cicatricial ectropion
of the lids, they are mostly seen following chemical and thermal burns of the lids.
Symptoms of lagophthalmos are watering, inability to close the eye, redness of the
eye, pain, photophobia.
Two mechanisms are involved in watering of the eye in lagophthalmos.
1. Improper closure of the lid, ectropion of the lower lid, early tear film break-up all in
combination or alone produce epiphora.
2. Reflex watering is produced due to exposure of the cornea and conjunctiva that result
in corneal abrasion and ulceration. which in turn leads to circumciliary congestion. The cause
of redness of the eye in is exposure conjunctivitis and circumciliary especially in the lower lid.
Signs of lagophthalmos consist of
(a) Loss of horizontal folds in the fore head.
(b) Flattening of the eyebrow.
(c) Widening of the interpalpebral fissure.
(d) Weakness of the orbicularis.
(e) Ectropion of the lower lid. Initially the inner margin just fails to remain in contact
with the globe later it gets everted. If the evertion is much the tarsal conjunctiva also gets
everted and becomes keratanised, dry and ulcerated.
(f) Conjunctival congestion.
(g) Exposure keratitis.
Corneal sensation remains normal so long trigeminal is not involved. Once there is loss
of sensation the condition becomes grave. The child without corneal sensation does not com-
plain of pain and watering which he would have done in presence of sensation. This may lead
to ulceration, perforation and blindness.
Management
Management of lagophthalmos depends on severity and duration.
Lagophthalmos of mild degree and short duration does not require much attention except
to keep the cornea protected by bland lubricant or artificial tear. Antibiotic ointments
are given during sleep.
Bells palsy is produced secondary to edema of the facial nerve in the facial canal due to
uncertain cause is treated by systemic steroids.
In case of corneal ulcer, hypopyon tarsorrhaphy is recommended to save the cornea
from perforating.
D8ORDER8 OF LDMARGN N CHLDREN
Important disorders of lid margin in children consist of:
A. Congenital
1. Coloboma of the lid margin.
2. Ankyloblepharon
3. Trichiasis
4. Distichiasis
5. Entropion
B. Inflammatory
1. Blepharitis
2. Stye
3. Chalazion
C. Post inflammatory and traumatic
1. Entropion
2. Ectropion
3. Trichiasis
Most of the conditions mentioned above except entropion, ectropion and trichiasis has
already been discussed.
Entropion. Entropion in children is rare; a congenital entropion may be associated
with epicanthic fold. In infants patching of the eye may lead to entropion of short duration that
passes off with removal of pad. Cicatricial entropian is seen in children following acid alkali
burn or post-inflammatory conditions like Stevens Johnson syndrome membranous conjunc-
tivitis. Children do not develop trachomatous entropion because it takes minimum 15-
20 years to develop trachomatous entropion.
Ectropion. Congenital ectropion is rarer than congenital entropion. Ectropion in chil-
dren are mostly traumatic or post inflammatory. The infection in such cases are seen on the
skin surface rarely it is seen in facial palsy and in acid in alkali burns.
REFERENCE
1. Duke Elder. S. and Wyber K.C. ; System of ophthalmology Vol. II p-503-537 1st edi-
tion, Henry Kimpton, London 1961.
2. Nema H.V. ; The lids in Anatomy of the eye and its adnexa 1st edition, p-61-67, Jay
Pee Brothers, New Delhi 1991.
3. Banumathy S.P. ; Eye lids in Anatomy of the Eye p-35-43, edited by G. Natchiar,
Arvind Eye Hospital & Postgraduate Institute of Ophthalmology, Madurai.
4. Ahmed E. ; Anatomy of the eyelids in A Text book of ophthalmology, 1st edition
p-9-12, Oxford University Press, Calcutta 1993.
5. Buftam F.B. ; Lacrimal diseases in Text book of Ophthalmology, Vol. IV, p-7.5, edited
by Podos S.M., Yanoff M., Gower Medical Publication, London 1993.
6. Scheie H.G. and Albert D.M. ; Anatomy of the human eye in Text Book of
Ophthalmology 9th edition p-50, W.B. Saunders Comp., Philadelphia 1977.
7. Vaughan. D. and Asbury. T. ; Lids and lacrimal apparatus in General Ophthalmology
10th edition p-43-44, Lange Medical Publication, California 1983.
8. Dutta. L.C. ; Disease of eye lids in Ophthalmology Principle and practice 1st edi-
tion p-5 Current Books International Calcutta 1995.
9. Dutta L.C. ; Disease of the orbit in Ophthalmology Principle and practice , 1st
edition p-331, Current Books International, Calcutta 1995.
10. Khamar B, Mayuri Khamar Trivedi N. and Usha Vyas ; Inflammatory conditions of the
lids in Modern Ophthalmology, vol. I, 2nd edition p-4-6, edited by Dutta L.C., Jay Pee
Brothers, New Delhi 2000.
11. Dougherty J.M. and McCulley J.P. ; Comparative bacteriology of chronic blephariti B.J.O.
68:524-528 1984.
12. McInnes J., Donna Brown, McCulley J.P. ; Blepharo Conjunctivitis in Current ocular
therapy 5th edition p-421-422, edited by Fraunfelder F.T. and Roy FH, W.B. Saunders
Company, Philadelphia 2000.
13. Held K.S. ; Blepharitis in Decision making in ophthalmology, first Indian edition,
edited by vanHeuven WAJ and Zwann J.T. p-24, Harcourt Brace 1998.
14. Kincaid Marilyn. C. ; Pediculosis and phthiriasis in Current Ocular Therapy
5th edition p-100, edited by Fraunfelder F.M. and Roy F.H., W.B Saunders Comp.,
Philadelphia 2000.
15. Awan, K.J. ; Cryo therapy in phthiriasis palpebrum Am. J. Oph 83: 906-907 1997.
16. Mukherjee P.K. Jain P.C., Mishra R.K. Exanthemata : A caus of chronic dacryo cystitis
in children I.J. Oph 17, 27-30 1969.
17. Dekker N. W.H.M. ; Rubeola in Current ocular therapy edited by Fraunfelder F.M.
and Roy F.H. p-69, W.B. Saunders Comp., Phildelphia 2000.
18. Marsh R.J. ; Vericella and herpeszoster in Current Ocular therapy 5th Edition, ed-
ited by Fraunfelder F.M. and Roy F.H. p-83-82, W.B. Saunders Company Philadelphia
2000.
19. McDermott M.L. ; Corneal Hypesthesia in Decision making in ophthalmology, First
Indian edition, Edited by vanHeuven W.A.J. and Zwann. T., p-174, Harcourt Brace 1998.
20. Gittenger J.W. ; Lids and Adenexa in Manual of Clinical Problems in Ophthalmol-
ogy, Edited by Gittinger J.W. and Asdourian G.K., 1st edition p-5-7 Little Brown and
Co., Boston 1998.
21. Dua H.S. and Nilawar D.V. ; Non-surgical therapy of chalazion Am. J. Opl. 94: 424-
425 1982.
22. Viduarri. U. Jacob.P. ; Inter-lesional Corticosteriod treatment of chalazia. Ann.
Oph. 18-339-340 1986.
23. Dekker N.W.H.M. ; Cornea is measles Doc. Oph. 52: 1-120 1981.
24. WHO ; The Child Measles and the eyes, Geneva 1993.
25. Gittenger J.W. ; Cornea and Sclera in Manual of clinical problem in ophthalmol-
ogy edited by Guttinger J.N. and Asduria G.K. 1st Edition p-46-47, Little Brown and
Co., Boston 1998.
26. Ratnakar K.S. ; Pathology of eye lids and adnexa in Pathology the Eye and orbit, 1st
edition p-175-187, Jay Pee Brothers, New Delhi 1997.
27. Green C.M. and Ashton N.A. ; Melanosis, naevi and melanomas of the Conjunctiva and
lids in Ocular Pathology 1st edition p-85-101, Blackwell Scientific Publication, Ox-
ford 1963.
28. Ahmed E. ; Disease of lids in A Text Book of Ophthalmology 1st edition, Oxford
University Press, Calcutta 1993.
29. Boger W.P. and Peterson R.A. ; Paediatric Ophthalmology in Manual of ocular diag-
nosis and therapy 3rd edition p-274-276 edited by Deborah Pavan Langston Little
Brown.
30. Kanski J.J. ; Glacucoma in Clinical Ophthalmology, 2nd edition p-228-230, Buther
Worth, London 1989.
31. Kraemer K.H., Lee M. M., Scotto. J. ; Xeroderma Pigmentosum Cutaneous, ocular
and neurological abnormalities, in 830 published cases, Arch dermt 123: 241-250
1987.
32. Rao V.A. Srinivasn R., Agrawal K. ; Oculocutaneous manifestation of xeroderma
pigmentosum, Ophthal 78: 295-297 1994.
33. Freedman J. ; Xeroderma Pigmentosum in current ocular therapy 5th edition
p-136-137, edited by Fraun felder F.T. and Roy H.F., W.B. Saunders Comp. Philadel-
phia 2000.
34. Haik.B.G., Karcioglu Z.A., Gordon R.A., Pechous B.P. ; Capillary Hemangioma, Surv.
Oph. 38: 339-426 1994.
35. DeVenecia G., Lobee C.C. ; Successful treatment of eye lid hemangioma with
prednisone, Archoph 94: 98-102 1970.
36. Jobe Richard. P. ; Lagophthalmos in Current Ocular Therapy 5th edition p-436-438,
Edited by Fraunfelder F. T. and Roy H.F., W.B. Saunders Comp. Philadelphia 2000.
37. Mukherjee P.K. and Dongre R.C. ; A Case of acquired facial diplegia, macular
oedema and lingua plicata (Melkerson-Rosenthal syndrome) Ind. Jr. Oph. 21-36-39
1973.
38. Duke Elder S. ; Anomalies of the eye lids in System of Ophthalmology Vol. III, part II,
1st edition p-827-871, Henry Kimpton, London 1964.
39. Shaffer D.B. ; Abnormalities of Lid and Lacrimal apparatus in Text Ophthalmology,
9th edition p-287-293, edited by Scheie G.H and Albert D.M., WB Saunders Comp. Phila-
delphia 1977.
40. Shannon G.M. Flanagan J.C. and Saunders D.H. ; Disorder of the lid in Paediatric
ophthalmology, Vol. II, 2nd edition p-412-419, edited by Harley R.D., W.B. Saunders
41. Nema H.V. ; Congenital Anomalies of eye and its adnexa in Anatomy of the eye and
its adnexa, 2nd edition p-162-165, Jay Pee Brothers, New Delhi 1991.
42. Daily R.A. ; Epicanthus in current ocular therapy, 5th edition p-430-431, Edited by
Fraunfelder F.T. and Roy F.M., W.B. Saunders Company Philadelphia 2002.
Main function of the lids is to protect the globe from external trauma. This is brought
about by various reflexes that shut the eye behind, by an almost impermeable wall of lids.
However for the light to reach the eye the lids have to keep a clear area in front of the pupil in
the form of inter palpebral fissure. There is a delicate balance between keeping the eyes
open and closing it. There are three muscles in the lid that control the movements of the lids.
Two of them : the levator palpebral superior and orbicularis are striated muscle supplied
by third and seventh cranial nerve respectively with opposite actions. The third muscle
the Mullers muscle is plane muscle supplied by cervical sympathetic.
The levator palpebral superior is the main elevator of the upper lid and the Mullers
muscle is the accessory elevator of the lid. There is no well formed depressor of the lower
lid however in paralysis of Mullers muscle the lower lid is raised from its normal position
upwards.
The orbicularis is the muscle that closes the lid. It is directly antagonist of levator
palpebral.
In life a delicate balance exists between the two muscles and the eyes can be closed or
opened at will and reflexly.
The motility disorders of the lids are
1
1. That narrow the IPA.
2. That widen the IPA
3. Associated with synkinetic movement of lids.
The first group consists of ptosis and blepharospasm, the later group consists of lid
retraction and lagophthalmos.
Ptosis and lid retractions are not vision threatening but lagophthalmos is potentially
vision threatening.
The third group may be associated with narrowing or widening of IPA.
Ptosis
1,2,3,8
Normal upper lid covers 2 mm of the upper cornea. If it droops from this position, it is
called blepharoptosis or simply ptosis.
67
CHAPTER 4
Motility Disorders of the Lids
Ptosis in children is mostly congenital in origin and less commonly acquired. In adults
the incidence of congenital to acquired ptosis is reversed.
Ptosis in children have two pressing factors for management i.e. cosmetic blemish
and possibility of developments of amblyopia. However acquired ptosis in children have an
added factor of systemic neurological involvement that may be crippling, blinding even life
threatening.
It is of utmost importance that all drooping upper lids do not constitute true ptosis.
There are many condition of the lid, globe or orbit that may mimic ptosis and are grouped as
pseudo ptosis. Pseudo ptosis can be mechanical when a growth or edema of the upper lid
causes narrowing of the interpalpebral fissure. Otherwise the lid may be normal in the function
and appearance but it seems to droop due to change in shape, size and position of the eyeball
in relation to orbit. Occasionally the lid may be normal with normal inter palpebral aperture
but seems to be drooping because the contra lateral eye has a wider inter palpebral fissure
which is considered to be normal.
Causes of pseudo ptosis can be
1. Lid Edema of lid
Growth of lid Hemangioma
Neurofibroma
Sturge Weber Syndrome
Inflammation Large chalazion
Tarsal cyst
Lid abscess
2. Globe Microphthalmos
Microcornea
Phthisis
Atrophic eye
Enophthalmos
Anophthalmos
Enucleated and eviscerated sockets
3. Orbit Fracture floor of orbit
4. Miscellaneous Duanes retraction syndrome
Hypotropia of contra lateral side
Hypertropia of ipsilateral side
Classification of ptosis
1,2,4
Traditionally ptosis has been divided into two groups:
1. Congenital ptosis
2. Acquired ptosis
Other widely used classification is based on anatomical structures involved
1. Congenital (i) Simple congenital - only levator muscle is involved.
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.
(c) Along with other congenital anomalies of lids.
(d) Ptosis with synkinetic movements.
2. Acquired ptosis (i)Mechanical
(a) Edema lids
(b) Tumours of lid
(ii) Neurogenic
(a) Third nerve palsy
(i) Upper division
(ii) Both divisions
(iii) Trunk
(iv) Nucleus
(b) Along with other cranial nerves.
(c) Cervial sympathetic involvement
Horners syndrome
(iii) Myogenic
(a) Myasthenia
(b) Muscular dystrophies
(c) Chronic progressive ophthalmoplegia and its variations
(iv) Aponeurotic Traumato levator aponeurosis
Disinsertion of levator
Dehiscence of levator
(not seen in children)
(v) Trauma Laceration of IPS
Fracture orbit
Congenital ptosis
This is the commonest form of ptosis seen in children and lingers on to adult life if not
corrected. It accounts for about sixty percent of all ptosis in childhood. There are various
modes of presentation
1
1. Due to congenital fault in
(i) Levator alone
(ii) Levator Superior rectus complex
(iii) Third nerve
2. Birth trauma
(i) To LPS
(ii) Third nerve
(iii) Cervical sympathetic
Ptosis due to congenital fault in levator palpebral superior only (simple ptosis)
This type comprises of 75%-80% of all congenital ptosis. This is also known as simple
ptosis. It is inherited as autosomal dominant trait.
1
It is unilateral in seventy five percent
of cases. The bilateral cases are generally symmetrical. The ptosis is said to be complete
when there is no action of the levator. In congenital ptosis some action of levator is always
present hence most of them are called incomplete. Boys and girls are involved equally.
There may be many members of the family to have congenital ptosis including the siblings.
Congenital simple ptosis is non progressive and stationary unless corrected surgically. It
is generally present at birth but most often missed because a newborn keeps his eyes closed
for eighteen to twenty hours. Of course in cases of rare instances of severe ptosis, the parents
may notice its presence at birth. Unilateral ptosis draws attention early than bilateral. The
condition may be so small that it goes unnoticed unless the child looks up or develops abnormal
head posture.
The appearance of bilateral ptosis is characteristic. The forehead skin is thrown in
horizontal furrows, the eyebrows are arched up. The head is tilted back. Head tilt and
arching of the eyebrows are acquired late when the child realises that by doing so he has better
vision.
The inter palpebral fissure is narrow with some action of levator.
The skin of the lid is smooth, unwrinkled. The tarsal fold is generally absent. The
other extra ocular muscles including orbicularis are normal. Pupillary reaction and accom-
modation are normal. Child with unilateral ptosis may raise the eyebrow on the same side
only. The forehead furrow and the head tilt are less marked in unilateral ptosis.
The lid droops down over the cornea, the drooping varies in different cases, it may be
mild, moderate or severe, depending upon available levator function. In a normal child if
the upper lid is everted and child looks up, the lid is automatically corrected but not in con-
genital ptosis where most of the action of levator is absent.
The vision is normal. However associated error of refraction, which is mostly
astigmatism and causes subnormal vision. In severe cases the pupil may be obscured with
severe loss of vision, unless the lids are lifted. The other cause of diminished vision is amblyopia,
which is more often anisometropic than deprivation. Prolonged amblyopia may result in
strabismus.
The cause of maldevelopment of levator is not well understood. Various suggestions
given are:
1. It is due to lack of peripheral differentiation or aplasia of muscle.
2. Dystrophy or degeneration of levator.
3. Unknown trauma to the levator.
4. Abnormal development of levator.
5. The striated muscle fibres are replaced by fibro fatty tissue.
5
Congenital ptosis associated with defects in other extraocular muscles
These are generally put under heading of complicated congenital ptosis.
1. The commonest association is involvement of superior rectus on the same side.
During developement of the led a superior rectus and levator develop from a common
mesodermal mass and are closely associated with each other until late in development. The
nerve supply to the levator i.e. upper division of third nerve also supplies the superior rectus
and is also likely to share common dystrophic changes. Hence involvement of both the muscles
is a common feature.
Involvement of superior rectus does not produce expected diplopia. It may be asso-
ciated with under action of inferior oblique. There may occasionally be an elevator palsy.
Bells phenomenon may be minimally affected or absent. It is also not associated with changes
in pupillary reaction or accommodation. Other extraocular muscles have normal movement.
There are no synkinetic movements. Presence of diplopia on lifting, the lid denotes involve-
ment of extraocular muscles other than superior rectus alone.
2. Congenital ptosis may be associated with under action of other extra ocular muscles
supplied by third nerve due to developmental anomaly like hypoplasia of the third
nerve nuclei or its trunk.
3. Rarely there may be congenital anomaly of other cranial nerves supplying the extra
ocular muscles.
4. Congenital ptosis associated with under action of cervical sympathetic. This is
rare and mostly due to traction trauma to the brachial plexes during delivery.
1
5. Rarely neonatal myasthenia may present as congenital ptosis.
9
Congenital ptosis associated with other congenital anomalies of lid
These consist of
Ptosis
Inverse epicanthic fold
Blepharophimosis
Telecanthus
The tetrad is called blepharophimosis syndrome.
6
Blepharophimosis syndrome
The tetrad is an inherited disorder. All the features may not be present in all the cases
all the time. 3% to 6% of children with congenital ptosis have blepharophimosis. Some may
have just epicanthus inversus and ptosis.
In blepharophimosis the inter palpebral fissure is shortened in length as well as
width. The epicanthus inversus is a fold of skin on the medial side of the medial canthus
extending from lower lid upward. In telecanthus the inter canthal distance is increased. The
child keeps the head tilted back. Other anomalies found are squint, punctal displacement,
coloboma of optic nerve head
6
. The condition is bilateral, equally seen in boys and girls.
Treatment
The definitive treatment is surgical correction of each deformity in various sittings. The
surgical treatment can be deferred up to age of five years in absence of amblyopia. The me-
dial canthal deformity is corrected six to twelve months before the ptosis surgery.
Congenital ptosis associated with synkinetic movements of the lids
The synkinetic movements can be
1. Jaw movement
2. Ocular movements
1. Ptosis with jaw movements is also known as Marcus Gunn jaw winking and in-
verse Marcus Gunn jaw winking. Jaw winking is present in four to six percent
of all congenital ptosis.
6
Jaw winking is more common than inverse jaw winking. The
former is caused due to contraction of external pterygoid muscle and latter is due
to contraction of internal pterygoid muscle. The syndrome of jaw winking is due
to congenital misdirection of fifth nerve to the levator.
Jaw winking is present at birth and first noticed by the mother who observes that
the position of the upper lid changes when the child suckles. Later the parents notice that
there is involuntary spasmodic jerky retraction of upper lid with certain movements of the
lower jaw i.e. moving the jaw to contra lateral side, projecting the jaw forwards or opening the
mouth.
In inverse jaw winking, the lid may elevate on closing the mouth or clinching the
mouth, the lid may be ptotic or may be normal.
The jaw winking is generally unilateral, present at birth and remains for rest of the
life. The child may learn to suppress it later to overcome embarrassment. It is also known to
become less severe as the child grows. It is mostly sporadic, may be seen in several members of
the family.
Treatment consists of surgical correction, before that any amblyopia or anisometropia
present may be corrected.
Surgical treatment is definitive treatment that requires correction of two separate
features - ptosis and jaw winking present simultaneously. Jaw winking when severe may be
worsened by levator resection so one of the modes of surgery is to release the levator aponeurosis
that worsen the ptosis which is later corrected by frontalis suspension.7
Congenital ptosis associated with abnormal ocular movements
1,3,8,9
Fibres destined for medial rectus are directed to superior rectus or inferior oblique.
Fibres of inferior rectus may be directed to the pupil. Hence many combination are possible.
Some of the common combinations are -
1. Lifting of upper lid in attempted adduction.
2. Paradoxical retraction of upper lid on the affected side in congenital paralysis of sixth
nerve.
3. Raising of lid in downward gaze (Pseudo Graefe sign).
4. Lid retraction on occlusion of contra lateral eye.
10
The ipsilateral eye is generally
ptotic with jaw winking.
Paralytic ptosis
Paralytic ptosis can be due to
1. Involvement of third nerve.
2. Involvement of cervical sympathetic.
1. Ptosis in third nerve involvement. It can either be congenital or acquired.
Congenital ptosis due to third nerve palsy is less common than congenital ptosis due to levator
dysplasia. Ptosis due to oculomotor palsy is far more common in adults than in children. In
adults, diabetes, vascular lesions and neoplasm are more common than infection. In children
infection is more frequent cause of paralytic ptosis than others. However third nerve involve-
ment in which ptosis is an outstanding feature may be the only sign to begin with, this may be
followed by serious central nervous involvement that may not only be vision threatening but
crippling or life threatening.
Oculomotor ptosis can be due to
Nuclear, facicular, basilar and peripheral involvement.
The nuclear lesions are rare. It consists of bilateral, symmetric ptosis with supe-
rior rectus under-action. May be isolated, may precede palsies of other muscles supplied by
third nerve. Due to its proximity to fourth nerve nucleus, fourth nerve involvement is also
common.
Supra nuclear lesions cause mild ptosis with subnormal gaze palsy and miosis.
Mid brain lesions are associated with signs of dorsal or ventral fascicular lesion
i.e. Benedicts syndrome, and Webers syndrome. Trunk may be involved either in cav-
ernous sinus or orbit.
Treatment should be
1. Directed towards the cause.
2. Surgical intervention is deferred for about six months after ptosis has been stabi-
lised.
3. Amblyopia is treated by standard method.
Sympathetic ptosis (Horners syndrome)
11,12
Sympathetic ptosis occurs due to involvement of oculosympathetic chain anywhere
from hypothalmus to eye causing in a number of signs that constitute Horners syndrome.
For diagnostic purposes the oculo sympathetic is divided into three anatomic part consisting
of three neuron.
13
1. The first neuron extends from posterior hypothalmus to cilio spinal centre of Budge
between C8 and T2.
2. The second neuron extends from centre of Budge to superior cervical ganglion in
the neck.
3. The third neuron spreads from superior cervical ganglion to the eye via nasociliary
and long ciliary nerves.
Incidence of Horners syndrome is less common in children than in adults. It can be
congenital or acquired. The acquired causes in children are birth trauma mostly to the
brachial plexus associated with Klumkes paralysis. Some authors believe that congenital
Horners syndrome is nothing but a result of birth trauma. Besides birth trauma accidental
injury to neck, enlarged lymph nodes in neck, cavernous sinus disease, inflamma-
tion in orbit can cause Horners syndrome.
The features of Horners syndrome consists of
1. Permanent signs and
2. Transient features
The permanent features are:
1. Ptosis
(i) Ptosis is mild, not more than 2 mm to 3 mm due to paralysis of Mullers muscle.
(ii) The lid folds are retained.
(iii) There is no lid lag on upward gaze.
(iv) The action of LPS and superior rectus are normal.
2. Upside down ptosis of the lower lid: The retractor muscle of lower lid is also
supplied by oculo sympathetic hence its paralysis results in upward shift of the lower
lid. The normal lower lid that just touches the lower limbus covers the lower 1 mm of
the cornea.
3. Narrowing of inter palpebral aperture: This is due to combined effect of ptosis
and upside ptosis.
4. Enophthalmos: This is more of a pseudo enophthalmos than true. The eye looks
small due to ptosis and upside ptosis.
5. Miosis: The pupil on the affected side is smaller by 0.5 mm to 1.00 mm and not pin
point. It is best observed in dim light. The pupil does not dilate with 4% cocaine.
The pupil will dilate with hydroxyamphetamine 1% (Paredrine) in pre ganglionic
lesion but not in post ganglionic lesion.
6. Anhydrosis of the affected side of the face.
7. Heterochromia of the iris on the affected side. The iris on affected side is lighter.
This is characteristic of congenital Horners syndrome in children.
13
The transient signs
1. Dilatation of conjunctival vessels.
2. Dilated facial vessels.
3. Reduced intraocular pressure.
4. Increased accommodation.
There is no change in convergence and light pupillary reaction.
Differential diagnosis consists of all causes of unilateral small pupil - Congenital miosis,
traumatic miosis, healed iridocyclitis.
Treatment
There is no known treatment for Horners syndrome. Attention should be directed to
the cause of Horners syndrome, which are confirmed by -
1. Pharmacological test
(i) Cocaine test
(ii) Paredrin test
2. x-ray chest
3. CT and MRI
4. Neurological evaluation
Neuro muscular (Myogenic) ptosis
There are two types of myogenic ptosis:
1. Fault at myoneural junction.
2. Myopathies and muscle dystrophies.
Both are more common in adult but can be seen in children and may be congenital
or neonatal.
1. Fault in myoneural junction
Myasthenia gravis
15,16,17
: Myasthenia gravis is an auto immune disorder that pro-
duces extra ocular muscle hypofunction without involving intrinsic muscle. It is of two types:
1. Ocular and 2. Systemic
About 90% of patients with myasthenia have predominantly ocular involvement that
may precede systemic involvement in 75% of cases, 80% of cases with ocular involvement will
be gradually converted into systemic involvement in two years
14,15
Chances of conversion to
systemic myasthenia after two years is less common. It is not a hereditary disease but may be
seen in members of the same family.
3
Infants born to myasthenic mothers are prone to develop
neonatal myasthenia, which may be present at birth. Myasthenia is more common in women
than in men in a ratio of 2:1. However childhood myasthenia is more common in girls i.e. girls
to boys ratio is 6:1.
17
Myasthenia can be progressive or remittent.
Myasthenia is a common cause of acquired ptosis and diplopia in all ages without
other neurological involvement. It can affect any age from birth to late seventh decade but
common age group is between 20-40 years. About six percent of cases may develop dysthroid
oculopathy later.
Exact etiology of myasthenia is not known. It is presumed to be an autoimmune disorder.
Acetyl choline, which is a pharmacological transmitter at neuro muscular junction is destroyed
fast by an unknown substance present at the neuro muscular junction. This unknown
substance
19
has curare like action that renders the muscle hypoactive and fatigued. There is
reduction of acetylcholine receptors at motor end plate
20,21
Use of anticholine estrase drugs
rectify this process, this forms the basis of medial treatment of myasthenia.
Ocular symptoms of myasthenia gravis
Two main symptoms are 1. Ptosis and 2. Diplopia. There may be concurrent or one may
follow one another.
Ptosis: Drooping of upper lid in myasthenia is variable though it is bilateral, may
begin in one eye. The other eye follows soon. In bilateral ptosis drooping is systemic. It may
shift from one eye to the other eye. Drooping changes not only in days but may be in hours. It
is generally minimal or absent on waking up and gradually increases during active hours
becoming maximum in the evening.
Variability is demonstrated by following tests:
1. Cogan lid twitch: The patient is asked to look down for 10-15 seconds and then
asked to fix the eye in primary position quickly. This sends the upper lid higher than
normal, only to fall to ptotic position.
2. Lid fatigue: With sustained upward gaze, the ptosis becomes more.
3. Cold test: An ice cube is applied to the ptotic eye for about two minutes. On removal
of the ice, the lid is markedly elevated which falls to ptotic level in 15 seconds.
4. If the contra lateral lid is lifted mechanically there is enhanced ptosis in ipsilateral
side.
5. Under action of orbicularis is almost constant feature in myasthenic ptosis.
6. Improvement of ptosis following Tensilon test.
7. Therapeutic trial by oral prostigmine and pyridostigmine shows improvement in
ptosis and reduction of diplopia.
Diplopia
Diplopia is one of the most common and sometimes first symptom experienced by the
patient. It varies in type and extent. It is more often vertical than horizontal. It becomes worse
in the evening.
Squint
Some patient may notice squint. Extraocular muscle involvement does not follow any
fixed pattern. Muscle imbalance may simulate inter nuclear ophthalmoplegia or central
gaze palsy.
16
There may be nystagmoid movements. Extra ocular muscles to be involved
other than levator is orbicularis, Mullers muscle is never affected. Patient may present
with heterophoria. Commonest being exophoria. Any of the skeletal muscles can be affected.
Common are the small muscles of face, laryngeal muscle, muscles of mastification,
most probably due to involvement of bulbar muscles.
17
Involvement of thymus is common.
Myasthenia of childhood differs from adult myasthenia. There are three types of
myasthenia in children -
1. Transient neonatal myasthenia seen in children born to mothers who have the
disease.
2. Persistent neonatal myasthenia: This is similar to transient myasthenia but moth-
ers do not have the disease.
3. Juvenile myasthenia sets in after ten years of age. The presentation is similar to
adult myasthenia.
Differential diagnosis
All cases of variable ptosis, diplopia with orbicularis weakness and normal pupil should
be suspected to have myasthenia unless proved otherwise. The conditions that constitute dif-
ferential diagnosis are:
Chronic progressive external ophthalmoplegia and its variations.
Myotonic dystrophy
Inter nuclear ophthalmoplegia
Dysthyroid myopathy.
Diagnosis of myasthenia
Diagnosis of established myasthenia is not difficult however may have to be confirmed
by
I. Pharmacological testTensilon test.
22
1. To do this test first precaution is to have a resuscitation trolley ready and handy.
2. Inject 0.3 mg atropine I.V. (adjust dose in children).
3. Fill a syringe with 10 mg Tensilon.
4. Inject 2 mg I.V. and observe for any adverse reaction or improvement.
In adverse reaction, abandon the test and consider therapeutic trial with oral prostigmine.
In case of improvement in ptosis, facial expression and diplopia, the test is conclusive that may
be further confirmed by injecting remaining 8 mg.
23
Three types of responses are possible:
1. An improvement.
2. Worsening: Small tropia becomes large tropia.
3. Reversal: Squint shifts to the other eye.
The response in 2 and 3 are not seen in true myasthenia.
II. Antibody to anti actycholine is present in 90% cases.
III. x-ray chest, CT and MRI of mediastinum may show thymoma.
Management
Myasthenia is a self limiting disease. In many children it will pass off after one or two
episodes without recurrence. Others will require medical treatment and complete ameliora-
tion may be achieved and child maintained on a maintenance dose for long time. Most of
children in this group will have some residual ptosis and or manifest muscle imbalance that
requires surgical correction later. Surgical correction should not be done unless there is no
recurrence for one year, ptosis and squint are stable for at least one year.
Medical treatment
Medical treatment consists of oral a anticholineesterase drugs given in divided doses.
Most commonly used drug is pyridostigmine. Its effect starts with in 10-30 minutes following
oral administration and lasts for three to four hours with peak in between. Hence the drug
has to be repeated every four to six hours. Correct dose is adjusted empirically in consultation
with pediatrician.
Other drug used is prostigmine which is less effective than pyridostigmine.
It takes two to three days for prostigmine to be effective. The side effects of the drug are
colic, diarrhoea, nausea due to prasympathomimetic action of the drug. These are reduced
if the drug is taken with food or some spasmolytic drugs are taken few minutes after the drug.
If the patient fails to show desired therapeutic dose, oral steroid are started, initially
a single daily dose is given till the desired effect has been reached, then the schedule is changed
to every alternate day and gradually reduced.
Other drug used in patient is refractory to anticholine esterase drugs is azathioprine.
It takes few months to be effective. Greatest drawback with the drug is its hemato and hepato
toxicity.
Cyclosporine and cyclophosphamide are other immunosuppressive drugs used in se-
lected cases.
17
Ancillary ocular methods
1. The child learns by experience to close one eye to avoid diplopia. The eye may be
patched to give same effect.
2. Prisms may be given to minimize diplopia. As diplopia is fleeting and changes direc-
tion too often many a times prisms may be of little use.
3. Ptosis crutches have limited value.
Thymectomy have been claimed to give good result in refractory cases in selected few.
Chronic progressive external ophthalmoplegia
25
The condition may be seen in children as well as adults. Generally it manifests between
second and fourth decade. It is a bilateral disease of slow progress. It is associated with
mutation in mitochondrial DNA.
24A
In fifty percent of cases the disease is hereditary.
The first sign that develops after ten years of age is gradually developing ptosis, which
progresses to become complete. The ptosis is bilateral and symmetrical. Ophthalmoplegia
develops years after ptosis. Involvement of muscles is ill defined. It begins as single muscle
involvement resulting in diplopia. As more muscles get involved the eye becomes almost
immobile and diplopia disappears. The internal ocular muscles are spared. The child throws
the head back to get clearer vision and forehead shows prominent frontal creases.
So long only extraocular muscles are involved, the condition is considered as ocular
myopathy. If other muscles get involved it is called ophthalmoplegia plus.
The condition may be associated with retinal pigment degeneration and heart block.
Occasionally there may be involvement of pharyngeal muscle.
There is no known specific treatment. Each disorder has to be treated individually.
Evaluation of a case of ptosis for surgery
Definite treatment of congenital ptosis is surgery. It is mandatory to evaluate each
patient separately. Sometimes each eye may require individual evaluation. Common heads
under which a ptotic child is evaluated are:
1. History. Find out if ptosis is congenital or acquired. In case of acquired ptosis atten-
tion should be diverted to find out the primary cause and its management. In case of acquired
ptosis surgical intervention should be undertaken only when the ptosis has been stable for at
least one year.
Congenital ptosis requires more correction than acquired. However levator resection
gives less correction in congenital ptosis.
2. Examination of the eye
1. VisionVisual acquity is evaluated to diagnosis presence of amblyopia that requires
energetic anti amblyopic treatment.
2. Examination of the lids
(i) Exclude pseudo ptosis from true ptosis, however some of the pseudo ptosis may
require surgical intervention.
(ii) Presence of epicanthic fold and blepharophimosis which may require separate
surgeries.
(iii) Absence of orbicularis action goes more in favour of myasthenia.
(iv) Presence of synkinetic movements require multiple surgeries.
(v) Width of inter palpebral fissure gives a rough idea about levator function.
Width of the inter palpebral fissure depends upon
(i) Action of LPS present.
(ii) If the action of LPS is poor, the lid will droop down.
The usual method to assess position of the upper lid is to find out level of the lid margin
in relation to upper limbus and comparing this with normal eye. In unilateral ptosis the other
eye acts as control.
In normal eye the upper lid covers upper 2 mm of the cornea in primary gaze. The
average vertical diameter of cornea is 11 mm. This leaves 9 mm of cornea not covered by lid. If
the upper lid droops by 2 mm from usual position i.e. 4 mm from upper limbus leaving on 7 mm
of uncovered cornea, the condition is designated as 2 mm ptosis and the ptosis is considered to
be mild ptosis. Similarly ptosis of 3 mm is called moderate and more than 4 mm is called
severe ptosis.
The choice of type of surgery to be undertaken partly depends upon degree of ptosis i.e.
mild, moderate or severe and amount of levator function available.
The function of levator is assessed by noting the excursion of upper lid from downward
gaze to maximum up gaze. In normal eye, this is between 12 mm-15 mm. If excrusion is more
than 8 mm, it is called good excrusion while fair excrusion means
5-7
mm and less than 4 mm is
poor. More is the excrusion better is the action of LPS, which means that the levator if strength-
ened surgically will eliminate ptosis.
3. Presence of Bells phenomenon. Bells phenomenon is one of the most important
defence mechanisms of the eye. It is lost in paralysis of the superior rectus. In absence of Bells
phenomenon, if the upper lid is raised the cornea becomes unprotected and exposed to exter-
nal injuries. Absence of Bells phenomenon is an absolute contra indication for any type of
ptosis surgery.
4. Exclude vertical tropia by cover test. This unmasks presence of pseudo ptosis.
5. Action of extraocular musclesUnder action of extraocular muscles result in
diplopia that is generally not felt if the upper lid covers the pupil. In presence of ocular palsy,
correction of ptosis will result in intractable diplopia, which may be considered worse than
cosmetic ptosis by the patient.
6. Sensation of corneaAbsence of corneal sensation is absolute contra indica-
tion for ptosis surgery.
7. Tear film statusPoor tear film status becomes worse following ptosis surgery.
Lid retraction
25,26
Normal upper lid covers up to 2 mm of the cornea in primary position and the upper lid
follows the movement of the globe both in up and down gaze.
If the upper lid does not reach the upper limbus or is so placed that a strip of sclera is
visible above the cornea it is called lid retraction. This is generally associated with lid lag.
Exposure of a strip of sclera below the lower limbus may be seen in case of sympathetic over
activity along with upper lid retraction. Visible bare sclera above and below is seen in
exophthalmos and proptosis.
Lid retraction can be seen in some physiological states also specially in infants and
children.
1. In infants: Transient lid retraction is common that pass off within a few weeks.
There may be widening of IPA if the illumination in the room is reduced.
2. Children: Children suffering from progressive loss of vision have upper lid retraction
as an effort to see better.
Pharmacological causes of lid retraction
1. Phenylepherine: In normal person phenylepherine does not cause any change in
lid position in relation to cornea but children with tendency towards sympathetic
over action show retraction of 1 mm to 2 mm following local instillation of
phenylepherine. The effect passes off with in hours.
2. Prostigmine and tensilon when given as injection cause improvement of ptosis and
sometimes the lid may be retracted.
3. Infants born to mothers with hyperthyrodism show retraction for two to three weeks.
4. Prolonged use of steroids are also known to cause lid retraction.
26
In unilateral ptosis the contra lateral lid may show retraction when the ipsilateral lid
is forced to elevate.
Pathological lid retraction
Lid retraction can either be unilateral or bilateral. In unilateral cases the condition may
remain unilateral or the other eye may be involved later. Bilateral lid retraction is generally
symmetric.
Commonest cause of lid retraction is dysthyroid myopathy due to sympathetic over
action of Mullers muscle. This is enhanced by instillation of sympathomimetic drugs and
reduced by instillation of sympatholytic drugs. Lid retraction is dysthyroid myopathy is gener-
ally bilateral and symmetrical. Other causes of lid retraction are sympathetic over action
and dorsal mid brain lesion.
Sympathetic lid retraction presents with feature opposite of sympathetic ptosis. It con-
sists of widening of IPA, enhanced lid crease, dilated pupil, mild exophthalmos, sweating on
the affected side and lowered temperature.
Dorsal mid brain lesion is called Colliers sign that consists of bilateral lid retraction,
there is conjugate up gaze paralysis of convergence, dissociation of light near reflex. There
may be associated retraction nystagmus.
Lids may be caught in scar of the lid following infection, burn, trauma accidental or
surgical. Over correction of ptosis is a common cause of unwanted lid retraction.
Treatment of lid retraction consists of recession of LPS along with Mullers muscle.
Lagophthalmos
In this condition there is either difficulty in closing the eye or there is complete absence
of lid closure. Closure of lids is brought about by the orbicularis muscle that is supplied by
facial nerve. Hence it is but natural that affection of facial nerve will lead to lagophthalmos,
the lesion may be anywhere from its nucleus to extreme periphery in the orbit.
Besides neurological causes there are other causes that are generally put under
mechanical causes that prevent the lids to close.
These are
Large coloboma of the upper lid.
Scar on the skin surface of the lids.
Extreme degree of symblepharon.
Ectropion
Proptosis
Exophthalmos
Over correction of ptosis
Myasthenia is the main myogenic cause of lagophthalmos
In Mobius syndrome there is bilateral facial palsy with bilateral sixth nerve palsy due
to aplasia of their nuclei.
Nocturnal lagophthalmos is a mild form of bilateral lagophthalmos, which becomes
obvious when the child sleeps. A strip of the IPA remains open during sleep. On examination
there is mild weakness of orbicularis. Bells phenomenon is intact.
Lagophthalmos is generally unilateral due to peripheral involvement of seventh nerve,
i.e. Bells palsy or trauma.
Bilateral exophthalmos is mostly central in origin. An exception being leprosy which
is the foremost cause of bilateral lagophthalmos in adults due to peripheral involvement. Chil-
dren do not suffer from lagophthalmos due to leprosy.
Other causes of bilateral lagophthalmos (facial diplegia) are
Causes of bilateral lagophthalmos have to be central. They are
Mobius syndrome
30
Melkerrson Rosenthal syndrome
28,29
Brain stem trauma
30
Brain stem glioma
30
Lyme disease
31
Guillain Barre syndrome
Closed head injury
Myasthenia
Neurological causes of unilateral lagophthalmos
Supra nuclear and nuclear lesions does not involve orbicularis in a peripheral
lesion.
A lesion at the pons involves sixth nerve nucleus causing ipsilateral lateral
rectus palsy, loss of conjugate gaze toward the same side and hemiplegia of the
contra lateral side i.e. Fovilles syndrome.
The same lesion with intact conjugate movement is called Millard-Gubler syndrome.
In children above lesions are due to pontine glioma or degeneration at the level of pons
and not vescular as in adults.
A basal lesion involving roots of seventh nerve also affects eighth nerve causing loss
of hearing, loss of taste in anterior two third of the tongue and diminished tearing. Common
causes for such lesions are fracture base of the skull and basal meningitis.
Cerebropontine angle lesions causes ipsilateral facial weakness and hyperacusia.
Geniculate ganglion lesion causes Ramsay Hunt syndrome. Commonest cause of
which is herpes zoster where vescicles develop in external auditory canal and tympanic mem-
brane. Other lesions being same as lesion of cerebropontine angle tumour.
Commonest type of facial palsy in all ages is Bells palsy.
Bells palsy is an acute unilateral facial palsy involving the facial nerve in the
facial canal. The lesion causes swelling of the nerve in a narrow canal. Commonsest cause is
viral infection though auto immune causes can not be excluded. The symptoms consist of
inability to close the eye on the affected side and watering. This may be preceded by pain in ear
and over the mastoid with hyperacusis.
The condition is self limiting clearing in two to three weeks without any residual effect.
It is known to recur in 3% to 10% cases rarely on the same side, however there may be some
cases where there is not complete recovery. Such cases should be investigated for causes other
than viral infection.
The signs of Bells palsy:
1. Lagophthalmos - The inter palpebral fissure is wide, there is paralytic ectropion of
lower lid with watering from the eye.
2. Watering from the eye is partly epiphora due to dribbling of tears over the lid margin
and is partly lacrimation due to irritation of unprotected conjunctiva and cornea.
3. The patient is unable to close the eye. In an attempt to close the eye, the eyeball roles
up due to intact Bells phenomenon.
4. However lower part of the cornea remains exposed in spite of intact Bells phenom-
enon and develops exposure keratitis that may be converted to ulceration on long
run.
5. In few days microbial conjunctivitis develops. If Bells palsy does not subside,
keratonisation of conjunctiva may develop.
6. Corneal sensation and other cranial nerve are normal.
Management of lagophthalmos
The management depends upon the cause of lagophthalmos.
It can be divided into:
1. Management of Bells palsy
2. Management of anatomical defects
3. Management of central causes.
Bells palsy does not require any specific treatment as it is self limiting. However asso-
ciated conjunctivitis, keratitis and lagophthalmos requires attention. Conjunctivitis and keratitis
are treated with frequent antobiotic drops and lubricants by day and antibiotic ointment by
night. If lagophthalmos does not improve with in a fortnight temporary lateral tarsorrhaphy
may be required.
Anatomical defects like scars and coloboma are treated by appropriate oculoplastic sur-
gery. The central causes require more elaborate investigation like CT and MRI and treatment
is directed towards primary cause.
Melkerrson Rosenthal syndrome
28,29
consist of peripheral facial diplegia, puffiness
of face and lingua plicata. It manifests in childhood. Lingua plicata is most probably congeni-
tal in origin.
Mobius syndromeThis is a multiple cranial nerve palsy of congenital origin, nerves
from fifth to twelfth are known to be affected. Commonest combination is sixth nerve with
facial diplegia. The child has unilateral or bilateral esotropia. There is inability to abduct the
eyes.
REFERENCE8
1. Lois J. Martyn : Abnormalities of lid function in Pediatric ophthalmology. Vol. 2,
Second edition, p. 807-814. Edited by Harley R.D., WB Saunders Company, Philadel-
phia, 1986.
2. Duke Elder S. : Congenital anomalies in mobility of lids in System of ophthalmology.
Vol. III, part 2. P. 887-905, Henry Kimpton, London, 1964.
3. Honavar S.G., Naik M.N., Geeta K. Vemuganti, Chandrashekhar G. : Ptosis in clinical
practice in ophthalmology. First edition. p. 497-502. Edited be Saxena S., Jay Pee
Brothers, New Delhi, 2003.
4. Miller S.J.H. : Congenital ptosis in Parsons diseases of the eye. Seventeenth edi-
tion. p-316, Churchill Livingstone, London, 1984.
5. Kahn N.D. : Ptosis in Current ocular therapy. Fifth edition. p-447-449. Edited by
Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadelphia, 2000.
6. Slonim C.B. : Blepharophimosis in Current ocular therapy. Fifth edition. p-422-423.
Edited by Fraunfelder FT and Roy F.H., WB Saunders Company, Philadelphia, 2000.
7. Beard C. : Ptosis. Third edition. p-113-115. CV Mosby, St. Louis, 1981.
8. Fiona Rowe : Ptosis and pupils in Clinical Orthoptics. First edition, p-164-171.
Blackwell Science, Oxford, 1997.
9. Duke Elders S. : Paradoxical movements of the lids in System of Ophthalmology. Vol.
III. Part 2. p-898-905, Henry Kimpton, London, 1964.
10. Jain, Seth : Prakash BJO, 40, p-588, 1956.
11. Glasser J.S. : Oculo sympathetic defect in Neurophthalmology. p-177-179. Harper
and Row, London, 1982.
12. Kanski J.J. : Horners syndrome in Clinical ophthalmology. Second edition. p-474-
475, Butterworth International, London, 1989.
13. Bajan Das F.J. and Kline L.B. : Horners syndrome in Neurophthalmology. Third
edition. p-121-123, JayPee Brothers, New Delhi, 1989.
14. Lois J. Martyn : Myasthenia Gravis in Pediatric ophthalmology. Vol. II. Second edi-
tion. p-881-882, Ed. Harley R.D., WB Saunders Company, Philadelphia, 1983.
15. Bajan Das F.J. and Kline L.B. : Myasthenia and ocular myopathies in
Neurophthalmology. Third edition. p-141-145, Jay Pee Brothers, New Delhi, 1989.
16. Glasser J.S. : Myasthenia and ocular myopathies in Neurophthalmology. p-268-273,
Harper and Row, London, 1982.
17. Miller N.R. : Myasthenia gravis in Current ocular therapy. Fifth edition. p-221-222.
Edited by Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadelphia, 2000.
18. Piest K.L., Berry S.M., Carter John E. : Drooping of upper lid in Decision making in
ophthalmology. First Indian edition. p-68. Edited by Heuven WAJ and Zwaan J.T.,
Harcort Brace, 1998.
19. Kanski J.J. : Myasthenia gravis in Clinical Ophthalmology. Second edition. p-477-
478, Butterworth, London, 1989.
20. Dutta L.C. and Dutta N.K. : Myasthenia gravis in Modern Ophthalmology. Second
Vol., Second edition. p-905-907, Jay Pee Brothers, New Delhi, 2000.
21. Fiona Rowe : Myasthenia gravis in Clinical Orthoptics. First edition, p-227-228,
Blackwell Science Ltd., Oxford, 1997.
22. Kanski J.J. : Myasthenia gravis in The eye in systemic disease. Second edition. p-56-
58, Butter Worth Heinemann.
23. Bajan Das F.J. and Kline L.B. : The Tensilon test in Neuro-ophthalmology. Third
edition. p-177, Jay Pee Brothers, New Delhi, 1989.
24. Miller N.R. : Myasthenia in Current ocular therapy. Fifth edition. p-221-222. Edited
by Fraunfelder F.T., Roy F.H., WB Saunders Company, Philadelphia, 2000.
24A. Kerrison J.B. and Nancy J. Newman : Chronic progressive external ophthalmoplegia in
Current ocular therapy. Fifth edition. p-208-210. Edited by Fraunfelder F.T. and
Roy F.H., WB Saunders and Company, Philadelphia, 2000.
25. Rosenberg M.A. : Ocular myopathy in Principle and practice of ophthalmology.
Vol. III, First Indian edition. p-1965-1967. Edited by Peyman G.A., Sanders D.R. and
Goldberg M.F., Jay Pee Brothers, New Delhi, 1987.
26. Glaser J.S. : Lid refraction in Neurophthalmology. p-36-37, Harper and Row, Lon-
don, 1982.
27. Meyer D.R. : Lid retraction in Current ocular therapy. Fifth edition. p-439-440. Ed-
ited by Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadelphia, 2000.
28. Duke Elder S. : Melkerrson Rosenthal Syndrome in System of ophthalmology. Vol.
XIII. p-21. Edited by Duke Elder S. and Macfaul P.A., Henry Kimpton, London, 1974.
29. Mukherjee P.K. and Dongre R.C. : A case of acquired facial diplegia, macular edema and
lingua plicata. Ind. Jr. Oph. 21 : 36-39.
30. Bajandas F.J., Kline L.B. : Facial diplegia in Neurophthalmology. Third edition.
p-153, Jay Pee Brothers, New Delhi, 1989.
31. Hedges T.R. and Hedge
5
T.R. : Bells palsy in Current ocular therapy. Fifth edition.
p-205-206. Edited by Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadel-
phia, 2000.
CHAPTER 5
Disorders of Lacrimal
8ystem in Children
LACRMAL APPARATU8
1,2,3,4
Lacrimal apparatus consists of three parts :
A. That produces tears.
B. Tear spreading systems : that transports tear from gland, across the conjunctive and
cornea to drain out of the eye and
C. The drainage system.
D. Tear forming parts consist of
1. lacrimal gland proper, one for each eye.
2. Accessory Lacrimal glands.
1,2,3,4
E. Tear spreading system consists of
1. lids
2. conjunctiva.
F. The drainage system consists of
1. Puncta one in each lid.
2. Canaliculus one in each lid
3. Common canaliculus one for each eye.
4. Lacrimal sac.
5. Naso- lacrimal duct
(a) The lacrimal glands
The lacrimal glands are the main source of tear. The lacrimal glands are divided into
two groups: -
1. Lacrimal gland proper and
2. Accessory glands.
1. The lacrimal gland proper is situated in the fossa for lacrimal gland on the
superiotemporal aspect of the orbit behind the septum orbitale. The lacrimal gland is divided
86
DISORDERS OF LACRIMAL SYSTEM IN CHILDREN 87
into two unequal parts i.e. a larger superior part known as orbital part and a smaller palpebral
part by the lateral expansion of aponeurosis of the levatorpalpebral superior and Whitnall
ligament. The division is limited to the front part of the gland. In the posterior part the two
lobes are indistinguishable. As the orbital part lies behind the orbital septum, it is normally
neither visible nor palpable, it has a superior and an interior surface, an anterior and a poste-
rior border and two ends, the lateral and medial. Superiorily, the periostium lies between the
gland and the orbital bone; the inferior surface has lateral part of the levator with its exten-
sion and lateral rectus under it. The posterior border rests on the orbital fat. The medial end
reaches, the levator muscle and the lateral end reaches the lateral rectus. There are about 10-
12 lacrimal ducts that originate in the orbital part and travel through the palpebral part.
Hence, removal of palpebral part alone will produce the effect of total removal as far as tears
are concerned.
The palpebral part is about one third of the orbital part
2
. It lies just above the lateral
part of the upper fornix. This part is sometimes visible when the lid is everted and the patient
looks down and it may be mistaken as a growth. There are six to eight lacrimal ducts that
originate in the lower lobe. The lacrimal ducts from both the lobes open in the conjuctival sac
4 to 5mm above the tarsal plate.
Histologically, the lacrimal gland, it is an exocrine tuboalveolar gland that resembles
the salivary gland and share similar pathological process i.e. acute infection, by mumps,
measles, chronic inflammation i.e. Mikuliczs syndrome and growth like adenoma.
Blood Supply
The lacrimal gland is supplied by lacrimal artery and some times by infraorbital part of
the maxillary artery. The venous drainage is through the lacrimal vein that travels back in the
superior ophthalmic vein
4
. The lymphatics drain in preauricular and submandibular glands.
The nerve supply to the lacrimal gland is divided broadly in to two parts i.e. afferent
and efferent. The former is via lacrimal nerve, which is a branch of, trigeminal. The efferent
consist of both sympathetic and parasympathetic nerves. The para sympathetic fibres origi-
nate in the lacrimal nucleus in the pons and reach the lacrimal gland in the lacrimal nerve
2,4a
4a. The sympathetic fibres reach the lacrimal gland via sheath of carotid through ophthalmic
artery. Role of sympathetic nerve in tear production is uncertain. The tear production mostly
controlled by parasympathetic
4a
.
2. The Accessory lacrimal glands. These glands are situated in the conjunctiva. They
are very small and contribute small amount of fluid mostly mucin to the tear film. They are :
(a) Goblet cells of conjunctiva,
(b) Krauses glands in the fornices
(c) Glands of Wolfring near the tarsal plate.
(d) Glands of Manz in the bulbar conjunctiva.
(e) Glands in the plica and caruncle.
(f) Infra orbital glands.
Development of lacrimal and accessory lacrimal glands
The lacrimal gland is ectodermal in origin and is the only source of epithelial tissue in
the orbit that is liable to develop malignant epithelial tumours
5
. The main lacrimal gland
develops at the end of second month as eight epithelial buds arising from superio -lateral part
of conjunctiva. The buds get transformed into solid cords that get canalised by third month.
The mesoderm condenses round these. The developing levator divides the gland into palpebral
and orbital part by fifth month. Tear production starts by the first month post natal. The
lacrimal glands are fully developed by third year
6
. The accessory glands develop from the
conjunctiva as ectodermal invagination
7
.
(b) The tear spreading system
It is essential to keep the cornea and the conjunctiva wet through out the life. This is
brought about by constant flow of tear from lacrimal glands. As formation of tear is a constant
process it should also be removed from the eye least there is an over flow of tears. A delicate
balance between production and exit of the tear from the eye exists. This is brought about by
constant evaporation of tears from the cornea and conjunctiva, when the eyes are open and an
intact drainage system that work through out day and night. For a smooth flow of tears from
the superio temporal region to inferionasal quadrant, the lids should have sharp, regular and
continuous margin that remains in contact with the globe and moves freely during blinking.
The tear is spread across the globe not only by smooth movement of lid but also by gravity,
capillary action and surface tension.
(c) The Lacrimal drainage system
1. Puncta. Puncta are two openings situated on the posterior part of each lid margin on
the medial side. They are not visible unless the lid margins are everted. It is easier to see the
lower punctum than the upper punctum especially in children. The puncta are located 6 and
6.5mm from the medial canthus, the upper punctum is nearer the canthus than the lower
punctum, hence the puncta do not over lap each other, when the lids are closed. The punta are
the openings of the canaliculi. The puncta are kept in contact with the globe and glide over the
globe for drainage of the tear. The lower punctum is more important than the upper as far
as drainage of tear is concerned. The lower punctum drains about 75% of total tear.
Damage to lower punctum causes more epiphora than upper.
2. Canaliculi. Each lid has one canaliculus that starts from the punctum and ends in
common canaliculus. Each canaliculus has two parts i.e. a small vertical part and a longer
horizontal part the vertical part is 2mm long while the horizontal part is an 8-8.5mm in length.
The two parts join each other almost at right angle, each canaliculus pierces the lacrimal facia
separately and the two join to form the common canaliculus which opens on the lateral side
of the sac, occasionally the two canaliculi may open separately. The canaliculi lie superficially
in the lid hence they are frequently damaged by wounds of lower lid.
3. Common canaliculus. The two canaliculi join to form common canaliculus before
opening in the lacrimal sac.
4. Lacrimal sac. The lacrimal sac is the reservoir of tears between the canaliculi and
the nasolacrimal duct. The normal sac which is 11-12mm in length, 4 to 5mm in width, and is
flattened anterio posteriorly, the anterio posterior depth is only 2 to 3mm. Thus, it is more
or less like a club that has been flattered anterio posteriorly. Its volume is 20 cu.mm when not
distended. This much of fluid is sufficient to make the sac non visible and non palpable. In
normal condition the sac lies in the fossa for lacrimal sac that is situated in the lower medial
part of the orbital rim. The frontal process of maxilla and lacrimal crest forms the fossa. The
fossa has two ridges called lacrimal crests, one formed by maxilla is known as anterior lacrimal
crest the other is called posterior lacrimal crest. The upper dome shaped part of the sac is
called its fundus while most of the part is called body.
The part that joins the nasolacrimal duct is called the neck. There is no strict anatomi-
cal landmark to differentiate the three parts. The sac is surrounded by the lacrimal fascia that
is formed by splitting of the periorbita. Between the lacrimal fascia and the sac is a plexus of
veins. Non-kertanised squamous cells, goblet cells and some columnar cells resembling respi-
ratory columnar cells line the inner wall of the sac. The ethmoidal cells and medial meatus
form the medial relation of the sac, on the lateral side is the caruncle and palpebral part of the
orbicularis, behind the sac lies the lacrimal part of the orbicular is and posterior limb of medial
canthal ligament. Anteriorly, the anterior limb of the medial palpebral ligament crosses the
sac mid way between the fundus and neck above the larimial fascia 4a. The angular vein
runs vertically 8mm medial to the medial canthus, a tributary to this descends with in 3mm of
the sac. This is an important landmark, while exposing the sac. The incision should be with in
3mm of the medial canthus. The incision to expose the sac should be concentric with the fibres
of the orbicularis. The best landmark to locate the sac is to feel the medial palpebral ligament
first and give a vertical incision over the ligament at right angles to the ligament. For ideal
exposure the incision should begin 3mm above the medial palpabral ligaments. The medial
palpebral ligament is felt as a horizontal band medial to the medial canthus when the lateral
canthus is pulled laterally. As the sac has less space to distend at the fundus, the sac swellings
are more marked under the medial canthal ligament. An oval swelling above the medial canthal
ligament is more likely to be unrelated to the lacrimal sac.
5. Naso lacrimalduct. The naso lacrimal duct is down ward extension of the lacrimal
sac in a bonny canal. It extends from the sac to the interior turbinate of the nose. Its about
18mm in length. It is directed down words. back wards and laterally. The directions of the
naso lacrimal duct be taken into consideration while probing in children least a false passage
is formed. The resiratory type of columnar cells are more in NLD than in sac. The duct has a
larger in introseous (12.5mm) part and a smaller intra mural part. The NLD is surrounded by
rich vascular plexus that is similar to seen in interior turbinate. The nasolacrimal canal is
formed by maxilla, lacrimal bone and inferior nasal concha. The intramural part open under
inferior turbinate that is 30-40mm form the anterior nares in adults and 20mm in children.
The lacrimal passage gets its blood supply from superior and inferior branches of the ophthal
micartery, in praorbital artery angular artery and spheno palatine artery. The venous drain-
age is via angular intra orbital, and nasal veins. The lymphalies drain in submandibular and
deep cervical glands.
Development of the lacrimal drainage system
The lacrimal drainage passages have a common origin. They are ectodermal in origin
embedded in mesoderm. The lacrimal sac and naso lacrimal duct develop beneath a furrow
formed by the lateral nasal process and maxillary process. The lacrimal sac develops as a solid
cord of ectoderm at 10mm stage, by 15mm it sends two columns in to the lid to form the
canaliculi. The lower canaliculius separates a part of the lid to form the caruncle. At 15mm
stage the solid cord descend down to form the nasolacrimal duct that is met by similar structure
arising from the primitive nasal cord
7
. The solid cord starts canalisation from both the ocular
and nasal ends
5
in segments. By birth there is a complete cavity of the sac and patent
nasolacrimal duct however some of the ectodermal derbies may remain in the naso lacrimal
duct causing congenital ductal block, in about 10% of infants. The canaliculi and puncta become
patent after the two lids have separated.
CONGENTAL ANOMALE8 8 LACRMAL DRANAGE 8Y8TEM
All the structures from lacrimal puncta to opening of nasolacrimal duct in the inferior
meatus may be effected by developmental anomalies singly or in combination.
Common congenital anomalies are
9
A. Congenital block of nasolacrimal duct
B. Congenital mucocele of the sac
C. Congenital fistula of the sac.
D. Atresia of puncta and canaliculi.
A. Congenial Block of Nasolacrimal Duct
Congenital block of the nasolacrimal duct is commonest developmental anomaly of the
lacrimal apparatus. It is a very frequent anomaly in a child and commonest anomaly of the
eye and adexa. It is found in about 6% of new born
8
. 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 history
9
commonest site of the obstruction is at the inferior osteum of the duct. The
nasolacrimal duct develops as a solid cord in a groove situated in the frontal process of maxilla
and lacrimal bone beneath the skin. The duct gradually gets canalised from both the ends but
predominantly from upper end. By birth the duct should be fully canalised, there may be few
insignificant blocks that get cleared by first month post partum. However in about six percent
of cases the canalisation is not complete. Generally there is single obstruction at the lower
end. However there may be more than one block. The blockage is caused by epithelial debries.
Atrasia of the inferior osteum by a membrane is very common cause of obstruction.
The effect of nasolacrimal duct block is not visible until three to four weeks, after birth
because the lacrimal gland does not start secreting adequality before third week. A typical
presentation is watery discharge in a congested eye with out photophobia. After few days the
conjunctiva gets inflamed and there is mucopurulent discharge. At this stage pressure over
the sac may result in positive regurgitation. In few months the sac gets distended and there is
copious reflux following pressure over the sac. Rest of the eye is not involved in congenital
nasolacrimal duct block but becomes prone for recurrent infection.
Diagnosis. Diagnosis is straightforward an infant develops epiphora after 3 weeks with
or without discharge and positive regurgitation, conditions that may be mistaken as congeni-
tal nasolacrimal duct obstruction (congenital dacryocystitis, or neonatal dacryocystitis) are
ophthalmic neonatorum, corneal abrasion, primary buththalmos, retino blastoma,
Congenital coloboma of the lower lid is a common cause of ipiphora with negative regur-
gitation test.
Ophthalmianeonatorum becomes obvious with in first week. It is generally bilateral
and there is copious mucopuralent or purulent discharge without regurgitation. Corneal inju-
ries are generally unilateral without mucopurulent discharge and stain with fluoreseein, con-
genital glaucoma is generally associated with photophobia and the cornea is large, ofcourse
the tension is high.
The term congenital dacryocystitis is not very appropriate because inflammation of the
sac is secondary to duct obstruction, the sac is sterile at birth and the inflammation is noticed
after third week post partum hence it will be better to call it as congenital nasolacrimal
duct obstruction.
Management
Management of the condition is simple provided the treatment is initiated before twelve
weeks. The treatment consists of opening of the block by external pressure over the sac and
treatment of associated infection.
Relief of the obstruction is achieved by application of gentle and firm pressure over the
sac to force the fluid down the nasolacrimal duct, which dislodges the epithelial derbies. The
application of pressure is generally called massaging of the sac. This give relief in 95-98% of
cases. The mother is taught this simple method by repeated demonstration and is asked to
perform the same in presence of the doctor. She is instructed to trim finger nails and keep the
hands clean. Infact she should be asked to wash her hands with soap and water every time
before she massages the sac. The sac is pressed with the pulp of the index finger. After the sac
has been pressed the mucopurutent discharge should be removed by wet cotton swab and a
broad spectrum antibiotic drop is instilled. The process of cleaning the hands pressing the sac,
removing the discharge, instilation of antibiotic should be under gone for five to six times a
day for six week.
After few days of repeated pressure application on the sac, the discharge changes to
watery from mucopurulent. The block is often relieved after 3 to 4 weeks but the process
should be continued for an other two to three weeks to keep the passage patent. Success of this
method depends upon proper compliance, repetition and continuation of the process for
five to six weeks. Sooner the massage is started better is the result generally 95% of the cases
will be cured by massage and local antibiotic. However this process may not be helpful (1) It
started after three weeks of on set of eiphora. (2) There is atrasia of boney canal (3) There is a
nasal cause (4) Improper method. In that case probing of the nasolacrimal duct under general
anaesthesia through upper puncta is indicated. A single probing done properly results in per-
manent cure. However sometimes repeated probing may be required. For a successful result
the probe should be directed downwards, laterally and backwards
10
. If repeated probing fails
than the condition should be treated either by conventional dacryo cysto rhinostomy, or
endoscopic D.CR. Incubation of nasolacrimal duct is another alternative.
Complication of Naso Lacrimal Duct Obstruction
Commonest organism that is isolated from infected sac is pneumococcus which is a
potential danger to the cornea, mild corneal abration in presence of infected sac may result in
to disastrous keratitis and corneal ulceration. A chronically infected sac may lead to acute
dacryocystites, and fistula of the sac.
B. Congenital Mucocele (Dacryocystocele)
4a,11
This is a rare condition present at birth as a bluish diffuse swelling medial to the
medial canthus. This can be sometimes bilateral. The condition is caused by simultaneous non
canalisation of both the canaliculi and nasolacrimal duct. The condition may be confused with
a haemangioma. The conditions waxes and wanes size and may altogether disappear following
gentle pressure over the sac
12,13
otherwise. Probing under general anaesthesia is indicated
other condition that may be confused with is meningocele in infants for which suitable tests
including C.T. may be done.
C. Congenital Fistula of the Lacrimal Sac
In this condition of fistulous track develops between the lower part of the sac and the
skin on the side of the nose below the medial palpebral ligument . The opening of the fistila
may be so small that it may not be noticed. Otherwise there is constant flow of tear form
opening . It may occasionally be bilateral. The exact mode of development of the condition is
not well under stood. One of the theories put forward is failure of embryonic tissue to close
fully
14
. The diagnosis is confirmed by injection a coloured fluid in the sac through inferior
puncta. The dye is seen to trickle through the opening, treatment consists of removal of the
fistulous tract and closure of gap in the sac and skin.
D. Atresia of Puncta and Canaliculus
14
This condition is brought about by failure of budding out of upper part of lacrimal pas-
sage into the lid or failure of to canalis the solid mass of cells that eventually become canaliculi.
The punctum may be altogether absent or may be rudimentary. Symptoms are epiphora with-
out infection.
NFLAMMATON OF LACRMAL 8AC {DACRYOCY8TT8} N
CHLDREN
Inflammation of lacrimal sac in children is rarer than seen in adults. The sac may have
either acute dacryocystites or chronic dacryocystitis.
A. Acute dacryocystitis
Acute dacryocystitis is generally pericystic cellulilis secondary to chronic infection
of the sac. It may proceed to form an abscess over the sac that may eventually burst on the skin
to result into a lacrimal fistula that connects the lumen of the sac with the skin out side. Acute
dacryocyititis in children develop when a congenital nasolacrimal duct block has not
been relieved, this is generally seen under 5 years of age. Another type develop in presence
of chronic bacterial dacryocystitis without congenital block in old children.
Symptoms of acute dacryocystitis consist of painful, pink, diffuse swelling between the
lateral side of nose and medial canthus which may develop fluctuation and pus forming at
most dependent part. Submandibular and preauricular glands are enlarged. Condition may
subside only to recur or may form a fistula.
Management of acute dacryocystitis is systemic broad spectrum antibiotic (common
organisms are H. influenza and pneumococcous) oral or injection, hot fomentation, oral
analgestic. Local antibiotics are not effective, if the pus points it is drained by stab incision and
pus expressed out. The wound is dressed with magsulf and glycerine dipped ribbon gauge. The
wound is allowed to heal from beneath. Syringing and probing is contra indicated. When
acute infection subsides, external or endoscopic DCR is performed.
B. Chronic dacryocystitis in children
Chronic dacryocystitis in children is less common than adults. In adults it is caused due
to acquired nasolacrimal duct obstruction mostly in females in third and fourth decade who
have narrow nasolacrimal canal which gets obstructed due to spread of infection from nose
and throat.
In children chronic dacryocystitis is caused due to unrelieved partial stenosis
of nasolacrimal duct . It may follow chicken pox
15
measels, where infection travels down
from the conjunctiva, as well ascends from nasopharynx. Other possibility is mid fascial trauma
with fracture bony nasolacrimal duct. Lastly rhinosporidiosis is a common cause of chronic
inflammation of sac in children in endemic area.
16
The symptoms consist of epiphora with or without conjunctivitis, diffuse painless swell-
ing (mucocele) under the medial canthal ligament, regurgitation of pus from the puncta on
pressure over the sac.
Generally the condition is unilateral.
The Signs are mucoid discharge near the medial canthus, mucocele, positive regurgita-
tion test. The mucopurulent discharge may come from any or both puncta. Persistent absence
of regurgitation from any puncta in presence of mucocele denotes corresponding canalicular
block.
Positive regurgitation test is a due to nasolacrimal duct obstruction which is confirmed
by -
1. Taste test
2. Lacrimal Syringing
3. Jones I and II test
4. Dacryocystography
5. Lacrimal scintilography
31
RHNO8PORDO88 OF 8AC
16
Chronic dacryocystitis of the sac due to rhinosporidiosis is rarely seen outside endemic
area of rhinosporidiosis. The disease is most prevalent in tropical and subtropical countries
like India, Sri Lanka, Indonesia, Philipines. India from where large number of cases
17,18
have
been reported too have pockets where the disease is prevalent. These area have high rain fall,
humid and warm climate as in Kerala, Tamilnadu, coastal Andhra, Orissa, West Bengal and
Chhattisgarh.
The disease is caused by an organism Rhinosporidium Seebri Exact mode of spread
of infection is not known. A widely put forward theory is what the patients get the infection
from infected cattle. When the persons share the same pond for bathing with infected water
buffalo, the water borne spores get lodged in the anatomical recesses like fornices, plica,
nasolacrimal duct. The organism derives its name from the fact that it was first described as
a nasal lesion. Nasal lesion happens to be most common involvement. Incubation period of the
disease is not known. All persons in the same locality do not get the same disease.
It is rarely seen in other the members of the same family, bilateral lesions are infre-
quent, simultaneous involvement of sac and conjunctiva is also rare. Most probably the in-
fected child has a pre existing total or partial block. Selective involvement of a few persons in
the same locality with similar life style point towards an immune mediated mechanism.
There are two types of ocular involvement .
A. Primary where either the sac or conjunctiva is involve without nasal involvement.
B. Secondary. Here the infection spreads to the sac from the nose via nasolacrimal
duct and conjunctiva is generally spared.
Appearance of the lacrimal sac is similar in both the types. There is a defuse pain
less swelling over the sac, the swelling is non tender, skin over the swelling has an orange peel
appearance. Though there is associated chronic dacryocystitis, eiphora is not a dominant symp-
tom. The condition is generally unilateral there is no regurgitation, following syregning the
swelling increases in size. Which disappears by firm pressure. The nasolacrimal duct is either
open or partially blocked. Generally there is bleeding from the nose in case of associated nasal
involvement. Lymph nodes are not involved.
Management There is no specific systemic or local medial treatment. Associated con-
junctivitis should be treated with local antibiotic drops. Definite treatment is surgical removal
of the sac. Precaution should be taken to remove whole of the sac without repture. Complete
removal of sac ensures freedom from the symptoms and recurrence. It part of the tissue is left
behind the condition is bound to recur.
CONGENTAL ANOMALE8 OF LACRMAL GLAND
1,9
Congenital anomalies to lacrimal gland are far rarer than that of lacrimal passage.
Common anomalies of lacrimal gland are -
A. Absence of the lacrimal gland
B. Alacrima.
C. Aberrant lacrimal gland
D. Fistula of lacrimal gland
E. Congenital cyst of lacrimal gland
F. Crocodile tear.
A. Absence of lacrimal gland
The lacrimal gland develops from the conjunctiva of the upper fornix as buds going up
and out from the developing conjunctiva. It is but natural that the lacrimal gland will be
absent in absence of the conjunctiva as in cryptopthalomus. Lacrimal gland is present in
anophthalmos where the eye ball is absent but conjunctiva is present.
B. Alacrima
In this condition there is partial or total absence of tear production in presence of lacrimal
gland and its ducts. It is generally a bilateral condition. The parents may notice that
though the child has redness of the eye and intolerance to light, the eyes do not water as is
expected and has only thick mucus discharge. On investigation both basic and reflex tearing
are absent , exact mechanism is not known most widely accepted theory is that there is lack of
neural communication between the gland and the lacrimal nucleus . Others feel that the lacrimal
gland itself is hypoplastic. It may be associated with multiple cranial nerve anomaly specially
sixth and seventh or may be part of congenital autonomic dysfunction known as Riley- Day
syndrome.
RileyDay syndrome
1,19
Is a rare disorder mostly seen in Jewish children caused by sever depletion of
parasympathomimetic neurones in spheno palatine ganglion. It has wide spread systemic fea-
tures which include, Episodes of systemic hypertension, excess of sweting, cyclic vomiting
muscular hypotony, emotional out burst, feeding difficulty. The children are less sensitive to
pain, decreased tenden reflexes is common. Ocular symptoms consist of alacrima, absence of
emotional tear production, absence of basic and reflex tear production, loss of corneal sensa-
tion, corneal opacity, vascularisation. The pupil constricts with very weak solution of
pilocarpine (0.0625%). There is no specific treatment management is symptomatic ocular
treatment consists of local tear substitute drops by day and ointment by night, local anti biotic.
C. Aberrant lacrimal gland
Aberrant lacrimal glands other than accessory lacrimal gland are found at any location
under the conjunctiva, commonest site is outer half of conjunctiva. It may be seen at limbus
and mistaken for limbal dermoid. It can even be seen on iris and choroid.
D. Fistula of lacrimal gland
Lacrimal gland fishila are seen on outer side of the upper lid above the tarsal plate, they
may occasionally be associated with malformation of outer canthus or with congenital cyst of
lacrimal gland . The opening is generally associated with a tuft of hair around it. As the open-
ing, communicates with the lacrimal gland, it is obvious that tear should flow out from the
opening. The flow is enhanced when there is stimulation of fifth nerve. The tract is common
site for recurrent infection. Treatment consists of removal of the tract and closure of the com-
munication with the gland.
E. Congenital cyst of the lacrimal gland
Congenital cyst of lacrimal gland is seen only in the orbital part of the gland as a tense
swelling under the superio temporal aspect of orbit, this may cause a ptosis, S shaped curve in
the upper lid and proptosis. It may be present at birth or may become obvious later. Treatment
consists of removal by orbitotomy if there is moderate to server proptosis.
F. Crocodile Tear
4a,11
This is a paradoxical reflex feature that is generally unilateral
caused by congenital misdirection of seventh nerve towards lacrimal gland through spheno
palatine ganglion. In this condition there is unilateral tearing during mastication. More common
is aquired paradoxical tearing following seventh nerve palsy due to aberrant degeneration.
Systemic condition that produce unilateral tearing due to gusto lacrimal reflex are : brain
stem lesions, Ipsilateral sixth nerve palsy is common in congenital crocodile tear.
THE LACRMAL GLAND D8EA8E8 N CHLDREN
Disorders of lacrimal gland can be :
A. Congenital (very few)
B. Acquired.
B. The acquired disorders can be :
1. Infective : Dacryoadinitis, Acute or chronic
2. Inflammatory : Sarcoidosis, (Generally not seen in children)
3. Neoplasia : Not seen in children
4. Trauma : Isolated injury to lacrimal gland is rare occurrence it is met
in sharp penetrating injury directed to the gland or is seen
in sever crush injury of skull and face.
More common than above are :
1. Excessive production of tearLacrimation
2. Scanty production of tearOcular surface disorders.
The symptoms of lacrimal gland disorders are :
1. Painful swelling of the main lacrimal gland.
2. Slow progressive swelling of lacrimal gland (chronic dacryodenitis)
3. Discharging sinus
4. Lacrimation.
5. Ocular surface disorder.
6. Proptosis
7. S Shaped Curve of upper lid
8. Ptosis.
ACUTE DACRYOADENT8
4a,4b
It is seen between five years to late teens. It is generally unilateral. The viruses infect
the lacrimal glands more commonly than bacteria. Commonest virus is mumps followed by
infective mononucleosis rarely by herpes zoster. The disease starts with chills rigor and pain
in the superio temporal aspect of orbit. There is a tense swelling over the lacrimal gland, mild
proptosis, ptosis, S shaped curvature of upper lid. Conjunctival congestion and rarely restricted
movements and optic neuritis, infact any part of the eye except lens and vitreous can be in-
volved by the disease.
The disease is caused by mumps virus single attack gives life long immunity. Parotids
and other salivary glands are invariably attacked along with lacrimal gland. It commonly
infects the gonads. Rarely it can cause central nervous system involvement in the form of
meningitis, encephalitis, myelitis and cranial neuritis differential diagnosis consists of stye,
infected chalazion, acute unilateral ineffective conjunctivitis, cellulitis lid and orbital
cellulitis.
Management consist of
Prophy laxis. Alive, attenuated mumps virus vaccine is safe and effective in child hood.
It gives life long immunity. In many countries this has been included in routine vaccination in
childhood.
Curative there is no known anti mumps chemotheraptic agent.
Supportive. Bed rest, analgesic, cool wet compresses, use of steroids is restricted to CNS
involvement. Keratitis, scleritis, uveitis are treated by usual methods.
Chronic dacryo adenitis is infrequent in children. It may be because by tuberculosis,
saccoidosis and rarely fungi.
D8CHARGNG 8NU8E8 OF LACRMAL 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.
LACRMATON
Lacrimation is defined as excessive production of tear. It may be with normal drainage
or obstructed drainage.
It is due to
A. Reflex Stimulation of Fifth nerve either in the Eye or Nasal mucous
B. Psychogenic
C. Supra Neuclear Lesion.
A. Reflex Stimulation of Fifth Nerve can be Brought about by
1. Physical factors. Bright light, cold wind, heat.
2. Chemical. Irritative gases and fumes.
3. Corneal condition that lead to lacrimation are Corneal abrasion, foreign body on
the cornea and tarsal plate, super ficial and deep keralitis, corneal ulcer, phlyctenular kerato
conjunctivitis, rupture in Desmets membrane (buphthalmos ) endothelial decompensation.
4. Uveal conditions that lead to lacrimation are acute and chronic anterior uveitis
5. Acute allergic conjunctivitis.
6. Glaucoma. Primary congenital buphthatmos, secondary buphthalmos, secondary an-
gle closure glaucoma.
7. Reflex irritation of nasalmucosa.
8. Allergic rhinitis, looking at bright light, tickling of nasal mucosa sheezing coughing.
B. Psychogenic
The exact cause of psychogenic tearing is not known however it is the commonest non
pathological cause of tearing in children.
C. Supra Neuclear Lesion
CNS cause include supraneuclear diseases i.e. pseudo bulbar paby, tumours, encephalitis.
EPPHORA
Epithora is defined as over flow of tears in presence of normal tear production it is a
passive phenomenon. Epiphora is produced due to
A. Obstruction in tear drainage apparatus i.e. puncta, canaliculi, sac or nasolacrimal
duct or
B. failure of tear spread i.e. lid deformity like ectropion, cloboma of lower lid, tumors at
the lid margin.
The tear is produced in the lacrimal gland and accessory lacrimal glands in an aqueous
form to these are added mucin from the conjunctival glands and lipid from sebaceous glands
of the lid. The lacrimal secretion is released in the outer corner of the superior fornix, from
there it is propelled towards the puncta by capillary action, surface tension and gravity across
the globe to be collected in a depression in the lower lid called lacus lacrimalis. The puncta,
which are in contact with the globe imbibe the tear, that reaches the sac via canaliculi, and
when the lids are closed and push the tear down the nasolacrimal duct when the lids open.
20
Thus with each blink tear is constantly forced through the drainage passage about seventy
five percent of drainage is through the lower punctum and rest from the upper. Epiphora
becomes worse if reflex tearing is super imposed to it.
C. Causes of Epiphora
Cause of epiphora may be single or multiple extending from lid margin to nasal mucosa.
For proper drainage the inner lid margin should be sharp and in contact with globe
any derangement in this will cause the tear to spill over the lid margin. Lid conditions that
cause epiphora are:- facial palsy, ectropion of lower lid, congenital or acquired
coloboma of lower lid, growth on the lid margin and tylosis.
The puncta may be absent, atrisic or pinpoint. It may be involved in trauma. In ectropion
the puncta generally looses its contact with the globe, commonest canalicular cause of
epiphora is also trauma however it may be blocked following canaliculitis or dacryolith.
Most important cause of epiphora lies in the sac as chronic dacryocystitis either
acquired or congenital. In both instances the sac ifself is patent, the obstruction lies in the
naso lacrimal duct. However chronically inflamed sac can become fibrosed and loose its lumen.
Other cause of epiphora are absence of sac following dacryocystectomy, failure of DCR or
nasolacrimal and lacrimalintubation. Sometimes the opening in the inferior meatus may get
blocked by a nasal growth i.e. polyp.
Tumours of the sac are unknown in children.
Trauma involving fossa for lacrimal sac or nasolacrimal duct may also cause epiphora.
A. Investigation in case of epiphora in children
1. History. Epiphora in infants and children from first month postnatal onwards is due
to neonatal block of nasolacrimal duct. Watering with discharge in a new borne is most
likely to be ophthalmic neonatorum rather that congenital nasolacrimal duct obstruction. In
children in second decade history of chicken pox and measles may be positive. In the same
age group history of bleeding from the nose is found in rhinosporidiosis of sac, history of
facial injury may be associated with nasolacrimal duct obstruction secondary to fracture of
lacrimal canal.
2. Examination should consist of
(a) Regurgitation test
(b) Taste test
(c) Lacrimal syringing
(d) Fluoreseine clearance test
(e) Jones I and II test
(f) Examination of lid margin for ectropion, coloboma of lid margin, growth of lid mar-
gin and tylosis.
(g) Examination of nose
(h) Evidence of scar over the medial canthal ligament
(i) Dacryocystography
(j) Lacrimal scintillography
(k) CT Scan
(a) Regurgitations Test. Normally pressure over the sac does not produce regurgitation
of mucoid or mucopurulent discharge from any or both the puncta, presence of regurgitation
always mean obstruction in the nasolacrimal duct or failure of DCR or intubation. Generally
regurgitation from lower puncta is more than from upper puncta. Regurgitation from lower
puncta with out regurgitation from upper means naso lacrimal duct block with block in the
upper canaliculus. Regurgitation from upper puncta only means block in lower canaliculis
along with nasolacrimal duct block. Absence of regurgitation in case of chronic dacryocystitis
may be due to
(i) Recent evacuated of sac,
(ii) Encysted mucocele of the sac
(iii) Pseudo sac following incomplete removal of sac.
(iv) Patent DCR and intubation
(v) Fibrosed sac,
(vi) Rhinosporidiosis of sac.
(vii) In case of fistula of sac the discharge comes through the opening on the skin, so does
any dye put in conjunctive comes.
(b) Encysted mucocele is a peculiar state of chronic dacryocystitis generally seen in
adult women, however it may be seen in children also, where there is a distended sac, which is
visible as a diffuse oval swelling between the nose and medial canthus. The swelling is tense,
non tender, non reducible without any external sign of inflammation. On exerting pressure
over the sac there is no regurgitation of contents in the conjunctival sac. Generally there is
no epiphora and conjunctiva is non congested, devoid of any discharge. Lacrimal syringing is
not possible. No drug or dye reach the throat following instillation in the conjunctival sac.
As epiphora is invariably absent or very scanty, patient seldom complains about it. The
patient generally presents with a tense painless swelling. Management is dacryocystectomy .
Persistent epiphora that is a common complication of dacryocystectomy is rarely seen follow-
ing 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 Test
19a, 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 drain-
age 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 differ-
entiate 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 dila-
tor under local anaesthesia. A lacrimal syringe is loaded with 2cc of normal saline, lacrimal
canule is attached to the syringe, the tip of the canula is attached to the syringe, and is passed
through the punctum and canaliculus. The plunger of the syringe is pressed slowly.
Following observations are possible
Fluid freely flows into the throat:
(a) Naso lacrimal duct is patent.
(b) DCR or intubation is patent.
Fluid regurgitates through upper puncta and by the side of the lacrimal cancula in
the lower puncta but does not reach the throat this means that there is a block in
NLD but both the canaliculi and puncta are patent.
Fluid does not regurgitate through upper puncta does not reach throat only
regurgitates by the side of lacrimal canulaNaolacrimal duct and upper passage are
blocked.
Fluid flows out of hole on the skinLacrimal fistula with or without block in the
nasolacrimal duct.
Not possible to do syringing either from upper or lower puncta: both the puncta are
blocked.
(e) Fluoreseine Clearance (Disappearance) Test. A drop of 2% fluorescein sodium
is instilled in the conjuctival sac. This stains the tear. The colour of the tear becomes lighter as
the tear drains. In case of normal drainage passage the fluorescine should disappear from the
conjunctiral sac. The presence of fluorescence is examined on a slit lamp with cobalt blue
filter. If no or little stain is present, the drainage passage is normal. If most of the stained fluid
is retained after 10 minutes the drainage passage is not functioning well, may or may not have
blocked nasolacrimal duct.
(f) Jones I (Primary dye test)
20,21,22
. This is a functional test to find lacrimal out flow
under normal physiological condition. In principle it is almost same as fluoresccin disap-
pearance 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 physiologi-
cal test because the dye is pushed down under pressure. A positive test denotes partial block of
the nasolacrimal duct. The test is performed as in Jones I upto instillation of dye then the sac
is irrigated with normal saline by a lacrimal syringe. If the stained saline is recovered from the
nose the test is considered to be positive which means that the dye was held up in the sac due
to partial block in the nasolacrimal duct. Care should be taken to prevent the saline going to
the throat and not coming out of the nose. This can be achieved by lowering the head of the
patient by 45.
A modified secondary dye test comprises of asking the patient to blow the nose on a
white tissue paper when Jones I test is negative and observe for sprinkled stain on the tissue
paper.
(h) Examination of lid margin. The lid margin is examined for any evidence that
cause loss of contact of lower lid margin with the globe through out its length, any loss of
contact will lead to spilling of tears. Common causes are ectropion of the lower lid, coloboma of
the lower lid, a small coloboma due to non repair or faulty repair of lower lid margin leads to
intractable and difficult to manage epiphora, orbicularis palsy.
(i) Evidence of scar over in medial canthal ligament. Scar over the medial canthal
ligament denotes previous surgery that may be simple dacryocystectomy, failed DCR or blocked
intubatinon or a closed fistula.
(j) Examination of nose. As the nasolacrimal duct opens under the inferior turbinate
it may be blocked by nasal polyp, nasal growth or deformity of medial turbinate.
(k) Dacroyocystography. Plain X ray without contrast fails to show any defect in the
sac or naso lacrimal duct. To make them visible it is essential to use a contrast dye, then x-rays
of posterio anterior and lateral views are taken. This is called simple or conventional
dacryocystography. Disadvantage of this procedure is that it fails to demonstrate diverticule
and filling defects. For better visualisation a modified method in which a fine catheter is intro-
duced 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 time
23
. 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 camera
23a,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 syring-
ing 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 8TATU8 N CHLDREN
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 micro-
scopic irregularities on the surface to give a better optical status to cornea, washes away de-
bris, small foreign particles offending micro organism and chemicals, It keeps the cornea cool.
The tear contains lysozyme and lactoferrin
24
which are mild bacteriostatic that helps to
keep infection to minimum. However these enzymes have a limit, beyond which they are inef-
fective 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 Moll
25
.
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 lubri-
cates the surface of the globe to allow the lids to glide smoothly. The lipid layer is removed by
irrigation, frequent instillation of drops or use of local anaesthesia.
Ninety Five percent of aqueous (middle) layer is secreted by main lacrimal gland and
remaining by accessory glands of Krause and Wolfring. This is the thickest layer that dissolves
oxygen and contains lysozyme and lacto ferrin. In fact it is the most important constituent of
tear by virtues of its fluidly and amount which can be increased many folds when need arises.
It acts as a flushing system that washes away debries and micro organisms. One of its main
function is optical.
The main function of the innermost i.e. layer consisting mostly mucin is to anchor the
aqueous layer to the ocular surface. Though the corneal surface looks smooth , in fact it has
microscopic rough texture. The epithelial cells have microvilli that project forward making
the surface uneven. The epithelium contains lipoprotein that make it hydrophobic i.e. , it is
not wetable by aqueous layer . The inner layer is secreted by conjunctival goblet cells, glands
of Manz and crypts of Henle. The mucin contain glycoprotein which are hydrophilic and
turns the coreal epithelium to a wetable surface absence of mucin layer makes the cornea non
wetable and dry.
The tear under normal conditions is formed at a constant rates of about 1.2l/min
26,27
,
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.
ABNORMALTY OF TEAR8 CAN BE BROUGHT UNDER THREE
GROUP8
A. Excessive production with normal out flow (Reflex lacrimation).
B. Over flow of tear with normal production of tear due to block in lacrimal drainage
passage.
C. Sub normal production of tear involve and composition (Tear film abnormality, Dry
eye Syndrome).
Dry eye syndrome can be brought about by.( All ages)
1. Aqueous deficiency
2. Mucin deficiency
3. Lipid abnormality
4. Irregularity of corneal or conjunctival surface
5. Abnormality of lid margin.
Aqueous deficiency tear abnormality is commonest form of dry eye syndrome fol-
lowed 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 film
28,29
. In chronic meibomianitis bacterial lipase hydrolyses the lipid in to fatty
acid that causes disturbance in lipid layer causing dry eye.
DRY EYE 8YNDROME N CHLDREN 8 BROUGHT ABOUT BY
A. Congenital absence of lacrimal gland, meibomian glands and congenital alacrima
(Riley Day syndrome )
B. TraumaticLoss of lid margin, coloboma of lid, inadvertent removal of palpebral
part of lacrimal gland, acid alkali burn.
C. Exposure conjunctivitis and kertanisation of conjunctiva
D. Conjunctival scaring
1. Late stage of trachoma (generally not encountered in first decade) extensive membra-
nous and pseudomembranous conjunctivitis.
2. Stevens Johnson syndrome, ocular pemphigoid, chemical burns mostly alkaline and
radiaton.
E. Nutritional : Hypovitaminosis A
F. Idiopathic
G. Facial palsy
H. Neuro tropic keratitis
I. Kerato conjunctivitis sicca and Sjogrens syndrome which are major causes of dry eye
in adult are not seen in children.
Clinical Presentation of Dry Eye in Children
Symptoms of dry eye develop gradually and are varied in nature. It may be associated
with systemic conditions like vitamin a deficiency, Stevens Johnson syndrome or Riley Day
syndrome. Trachoma rarely produces dry eye in children as it takes ten to twenty years to
develop conjunctival scaring. Lagophthalmos is generally associated with lacrimation due to
exposure keratitis and conjunctivitis. Inability to close the lid leads to poor resurfacing of the
tear film. In neurotropic keratitis loss of corneal sensation results in less frequent blinking
this also leads to poor tear spread.
The symptoms are unexplained redness, foreign body sensation muciod discharge with
relative scanty lacrimation, blurring of vision, fluctuation of vision.
Diagnosis of dry eye
History. Detailed history of trauma, adverse drug reaction, hypovitaminosis A should
be elicited
Examination
Lid for coloboma and orbicularis palsy.
Conjunctiva for evidence of vitamin A deficiency i.e. xerosis, Bitotspot.
Corena for vascularisation, opacity and filamentary keratitis,
Vital dye staining. Rose bengal and fluoresecin staining.
Rose Bengal staining. Rose Bengal is a vital stain similar to fluorescein. It has affinity
for mucus and devitalised epithelium of both cornea and conjunctiva. It also stains corneal
filaments and corneal plaques. Rose Bengal stains conjunctiva in the interpalpebral aperture
in a triangular fashion. The triangle has its base to wards the limbus and apex away. Rose
Bengal is used as 1% aqueous solution either as drop or impregnated over a strip of filter paper
similar to fluorescein strip, Rose Bengal causes intense irritation when used on unanesthetised
conjunctiva and hence not very popular. Xylocain 4% which is usual local anaesthetic agent
used, is contra indicated prior to use of rose Bengal as it devitalises the corneal epithelium,
safest local anaesthetic is proparacaine 0.5%. In contrast to rose Bengal fluorescein remains
extra cellular and does not stain mucus. Alcaine blue is yet another stain that has staining
property of Rose Bengal with less irritation.
Tear Film Examination
30,31
Tear film examination consists of
1. Tear film stabilityTear film break up time
2. Tear quantitySchirmer I and II
3. Tear quality
Tear film break up time
The precorneal tear film seems to be a continuos, permanent layer of tear over the
ocular surface. In fact the tear film breaks down at a fixed and regular interval between the
blinks and develops a so-called dry spot i.e. microscopic area devoid of tear film. This forma-
tion 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 required
30
. The patient is asked to
blink a few times and then keep the eyes open. The time between the last blink and appear-
ance of first dry spot is tear break up time.
Schirmers Test 1a.
This measures total quantity of aqueous tear i.e. basic and reflex. To perform the test
a specially designed filter paper of Whatman 41 grade, 5mm wide and 35 mm long is used. The
paper strip is folded at one end five millimetre from the end. The patient is seated in a dimly lit
room, the strip is inserted behind the lower lid on the outer side of the IPA. In such a way that
the folding line rests on the inner margin of the lid and remaining 30 mm hangs out side the
lid. No anesthetic is used hence irritation of the paper will initiate the reflex tearing along
with basal tearing. The patient is permitted to blink but not to squeeze the lids. The tear is
drawn over the filter paper by capillary action and spreads to the other end. After five minutes
the paper is removed and length of the paper wetted by tear is measured. In a normal eye this
should be about 10 mm to 30 m wetting. Less than 10 mm is abnormal. If the strip is moist
beyond 30 mm in lessthan five minutes it denotes either hyper secretion (lacrimation) or over
flow of normal tear secretion. In both the condition dry eye status is excluded. The test should
be postponed by half an hour if the lid has been manipulated.
Schirmers Test 1b.
Principle of the test is to eliminate reflex tearing by anaesthetising the cornea and
conjunctiva. The method is same as in schirmer 1a. Only difference is that the eye is anaesthe-
tised 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 syn-
drome that is seen in adults are not seen in children. Dry eye status in adults is not vision
threatening. However abnormal tear film status, when present in children are potentially
sight threatening i.e. in Stevens Johnsons syndrome. Xerophtalmia secondary to vita-
min A deficiency and alkaliburn.
Hypovitaminosis A is fully preventable if the child gets prophylactic does of vitamin
A as prescribed for prevention of xerophthalmia under national programme and the child is
immunised against measles. Stevens Johnsons syndrome is an unfortunate non curable dis-
order.
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 com-
fort to the child when it is possible to insert and retain a bandage lens.
Tarsorrhaphy in presence of orbiecularis palsy or neuro tropic kertalitis not only pro-
tects 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 epi-
thelium in Stevens Johnsons syndrome.
REFERENCE8
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, Lon-
don 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 ed-
ited by Fraun felder F.T. and Roy F.H. p-50-51, W.B. Saunder Company, Philadel-
phia 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
9. Duke Elder S. ; Developmental anomalies of the lacrimal apparatus in System of oph-
thalmology, 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
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 pp-
140-141, 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 Vol. III, Part 2 p-936-937, Henry
Kimpton, London 1964.
15. Mukherjee P.K., Jain P.C., Mishra, R.K. ; Exenthemata ; A caus of chronicdacryocystitis
in children IJO 17-27-30, 1969.
16. Mukharjee P.K. ; Rhinosporidiosis in current ocular therapy, fifth edition, edited by
Fraunfelder F.T. and Roy F.H. pp-66-67, W.B. Saunders Company, Philadelphia 2000.
17. Mukharjee P.K. Shukla I.M. Deshpande and M. Praveena Kher ; Rhinosporidiosis of the
lacrimalsac Ind. Jr. Oph. 30:513-514, 1982.
18. Shukla IM mukharjee P.K. Shushma Verma ; Primary rhinospori diosis of the eye int
congress of oph Acta XXVI , Vol. 2, 864, 1982.
19. Axelord F.B, Soloman J.M. and DAmico R. ; Familial dysautonomia unclassified dis-
eases 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 Se-
crets , Edited by Vander J.F. and Gault J.A. , First Indian edition pp-226-227, Jay Pee
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, Sev-
entieth edition p-327, Churchill Livingstone, London 1984.
23. (b) Chavis, R.K., Welham A.N., Maisey M. ; Quantitative lacrimal scientillography
Archoph 96; 2066-2068, 1978.
24. Kanski J.J. ; The dry eye in Clinical Ophthalmology, Second edition p-46-52, Butter
Worth International, edition 1989.
25. Daughman D. ; corneal Physiology in Principle and practice of Ophthal mology,
first Indian Edition, Edited by Peyman. G.A. Sander D.R. and Goldberg M.F. p-356-358,
Jay Pee Brothers, New Delhi 1987.
26. West C. ; Corneal disease in Principle and practice of ophthalmology, first Indian
edition. Edited by Peyman G.A., Sander D.R. and Goldberg M.F. P-447-449, Jay Pee
27. Kirmaini T.H., Daughan D., Asbury J., Siva reddy P. ; in Fundamentals of Ophthal-
mology, 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 Oph-
thalmology, 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 Inter-
national, 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 Con]unctiva 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 conjunc-
tiva 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 unicel-
lular 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
DISORDERS OF CONJUNCTIVA IN CHILDREN 111
APPLED ANATOMY OF CONJUNCTVA
1,2
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 conjunc-
tiva extending from upper border of tarsal plate and blends with fornix.
The Mullers muscle the non striated muscle of the upper lid lies just beneath this
part of conjunctiva.
The bulbar conjunctiva. This part of conjunctiva extends from limbus to the begin-
ning of fornices. It is firmly attached to the limbus. The conjunctival epithelium blends with
the corneal epithelium at the limbus. The deeper part of the bulbar conjunctiva is loosely
attached to Tenons capsule up to insertion of four recti. It is almost transparent, the white
sclera is visible through normal bulbar conjunctiva. The conjunctival vessels are also visible in
normal conjunctiva. With increase in number of vessels in the conjunctiva, the white conjunc-
tiva 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 limbus
3, 4, 5
. The limbus is an important surgical landmark of the eye. It is an ill
defined arcuate zone that is junction of cornea on one side and conjunctiva and sclera on
the other side. Besides having surgical importance the limbus has two more functions.
1. It supplies stem cells for corneal epithelium,
2. The limbal plexus are the sole sources of blood supply to the corneal periphery.
The limbus is a ring shaped zone widest superiorly and narrow on the side. The limbus
has three lines that divide it into two surgical zones.
These lines are :
1. Anterior limbal border,
2. Posterior limbal border,
3. Mid limbal line.
The anterior limbal line represents the termination of conjunctiva and Tenons capsule
at the corneal periphery, this overlies the termination of Bowmans membrane.
The posterior limbal line lies over the scleral spur 2mm away, concentric with anterior
limbal line. Its presence is not well felt under operating microscope. It is best demonstrated
under sclerotic scatter.
3
The mid limbal line lies in between the anterior and posterior limbal line. It is 1 mm
away from the anterior limbal line. It represents the termination of the Descemets mem-
brane and overlies the Schwalbes line, looks blue under microscope and the limbus beyond
it is white.
The limbus contains trabecular meshwork internally.
4
The Caruncle. The caruncle developmentally is a part of the lower lid that gets sepa-
rated 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 me-
dial canthus, has a size of 5mm x 3mm. It is covered by stratified epithelium. As a part of
conjunctiva it has goblet cell, Krauses cells and accessory lacrimal gland. As part of
skin it is endowed by presence of hair follicles and sweat glands. It does not have any
definite function but shares pathological processes common both to the skin and conjunctiva.
The plica semilunaris. This represents the vestigial nictitating membrane of the lower
vertebrates. It is a crescent shaped fold of conjunctiva with concavity towards cornea lateral to
the caruncle in the medial canthus. The lateral border is free with a blind recess underneath
that disappears if the eye ball is moved laterally. Histopathologically it is similar to bulbar
conjunctiva. It also contains melano phores and few non striated muscle fibers. It also does not
have any definite function.
The Fornix
6
The fornix is an irregular annular caul de sac formed by the junction of tarsal and
bulbar conjunctiva. The caruncle and plica semilunaris intrude into it on the medial side. It is
divided into four unequal parts : (1) The superior fornix, (2) The inferior fornix, (3) The lateral
fornix and (4) The medial fornix
The superior fornix is largest and deepest of all conjunctival fornices. It is located at the
level of superior orbital margin. The deepest part is at 12O clock i.e. roughly 13 mm from the
superior limbus. It gradually becomes shallow on either side. Few slips of levator palpebral
superior are attached to the deeper surface of the conjunctiva to give it its depth. It is a com-
mon site for foreign bodies to be lost causing intractable conjunctival discharge. It is the only
part of the conjunctiva that requires double eversion to examine.
The inferior fornix is smaller than superior fornix. It is located near the inferior or-
bital 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 equa-
tor on the lateral side.
The medial fornix is fully occupied by caruncle and plica semilunaris. The fornices
have Krauses gland on the deeper side.
Histology
Microscopically the conjunctiva is broadly divided into two parts
1. The epithelium,
2. Substantia propria.
The conjunctival epithelium is multi layered stratified squamous in nature. It is multi
layered. Number of layers vary at different locations. The substantia propria is a combina-
tion 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 are
2, 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 se-
crete mucin which is the inner most layer of tear film.
The gland of Krause. These are accessory lacrimal glands, they are similar to main
lacrimal gland in structure, in development and function. They secrete aqueous part of tear
film. Glands of Krauses are found in depth of connective tissue in the fornices. The upper
fornix has about 40 glands while the lower fornix has only 6-8 glands.
The gland of Wolfring. These are also accessory lacrimal glands. They are larger than
Krauses glands but far less in number. There are about 3-5 glands mostly in the upper palpebral
conjunctiva at the upper border of superior tarsus.
Henles gland. Like goblet cells these also are not true glands. They are folds of mucous
membrane between the tarsal conjunctiva and fornix.
Blood supply of the conjunctiva
6
. Conjunctiva is very rich in blood supply. This
makes large conjunctival wounds to heal fast. The conjunctiva is supplied by palpebral branches
of nasal and lacrimal artery, anterior ciliary arteries. The conjunctival veins drain in su-
perior 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 conjunc-
tiva 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 lymphe-
dema, 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 mem-
brane.
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 cornea
8
without involving the conjunctiva. When
developing at the limbus they are observed to be astride on the limbus partly on cornea partly
on conjunctiva. They vary in size. They may be very small and missed at birth and infancy. All
dermoids have tendency to increase in size at puberty. They may be large enough to protrude
through inter palpebral fissure. They are circular, raised dirty white coloured, may have
central pigmentation. Conjunctiva over the dermoid can not be moved. The growth itself is
fixed to the sclera. There may be small hairs on the surface of the growth which become
prominent with age. The dermoid never show neoplastic change. Rarely they arise on the
caruncle. Main symptom of dermoid is visible white growth against the black of the eye. It
may be sufficiently large to obstruct the pupil and cause diminished vision. Even when it is
seen partially over the cornea it produces irregular astigmatism. In rare instance a dermoid
may be found to extend inside the eye in the vicinity of iris and ciliary body. About one third
case of limbal dermoids are associated with systemic syndromes out of which Goldenhar
Gorlin syndrome is the commonest.
TreatmentSmall dermoids need no treatment, larger dermoids have to be removed
for cosmetic purpose.
Dermolipoma
This choristoma is less common than dermoid and develop away from the limbus. Com-
monest site being superior temporal aspect of fornix or lateral canthus. It is diffuse, soft in
consistency. It mostly contains fat, other tissues like hair, skin or teeth are not present. The
Conjunctiva can be moved over the growth. It has pale yellow colour. It may extend back in the
orbit. It does not produce any visual change. It is better not to remove the growth unless there
is facility to remove the tumour in toto may be by orbitotomy. Choristomas have been found in
microphthalmos, Goldenhars syndrome, optic nerve anomaly and macular
hypoplasia.
Congenital telangiectasis of conjunctiva. This is a hereditary anomaly of mucous
membrane and skin. Out of the mucous membrane the conjunctiva is very commonly effected.
It may be discovered at birth or infancy. Commonest age group being late adolescence and
early adulthood. It may be associated with telangiectasia of retina. It may be seen anywhere
on the conjunctiva but common site is bulbar and lower palpebral conjunctiva where it may be
present as a star shaped bunch of vessels or a mass of vessels. As similar changes are seen
in their mucous membranes the patient may have epistaxis, haematuria. Traumatic con-
junctival bleeding is common. Small a telangiectasia may be obliterated by cryo.
8GN8 OF PATHOLOGCAL D8ORDER8
Some pathological signs of conjunctival disorders are :
1. Change in colour, Anaemia (Pallor), Hyperemia, Pigmentation
2. Edema, chemosis 3. Haemorrhage 4. Follicle
5. Papilla 6. Xerosis 7. Scaring
8. Discharge 9. Membrane formation 11. Ulcer
12. Cyst 13. Concretion 14. Degeneration
15. Neoplasm
Last three groups are not encountered in paediatric age group.
Pallor of conjunctiva
Conjunctiva is highly vascular, normal conjunctiva is transparent through which the
subconjunctival structures like meibomian glands are visible as bluish streaks. The vessels
are visible as dark red streaks against the white sclera. In anaemia the conjunctiva like other
mucous membranes become pale. Pallor of conjunctiva acts as a rough indication of presence
of systemic anaemia. However conjunctiva can be blanched by instillation of vasoconstrictors.
Conjunctiva may become pale as porcelain in alkali burn.
Hyperemia of conjunctiva. Increased vascularity of conjunctiva is the commonest
sign of conjunctival disorder. It may be caused by abnormality of vessels or development
of abnormal vessels as telangiectasia or haemangioma. Conjunctival vessels may be-
come 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 injec-
tion. 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 al-
lergic. This type of inflammatory hyperemia should be differentiated from ciliary conges-
tion.
Conjunctival congestion Ciliary congestion
1. Blood vessel involved Posterior conjunctival Anterior ciliary
2. Distribution Prominent in fornix, fade Prominent round limbus
towards limbus fade towards fornix
3. Blood flow From periphery to centre From centre to periphery
4. Colour Bright red Light red
5. Effect of weak Instant blanching No or delayed blanching
vasoconstrictor
6. Branching Profuse branching Limited branching
7. Mobility Vessels move with Do not move with
conjunctiva conjunctiva
8. Ciliary tenderness Absent Present
9. Extension Spread over cornea Do not spread over
without interruption at cornea
limbus
10. Cause Disease of conjunctiva Anterior uveitis
Acuta glaucoma
Keratitis
Passive congestion of conjunctiva
Passive congestion of conjunctiva is less frequent in children. They are seen either in
mechanical obstruction of venous flow or rarely due to increased blood viscosity. Mechanical
causes of passive congestion of conjunctiva can be either in the globe, orbit or systemic.
Causes are :
1. Intraocular growth and absolute glaucoma.
2. OrbitProptosis, exophthalmos-Increased intra thorasic pressure, polycythemia.
3. Systemic.
Treatment of hyperemia of conjunctiva is directed towards the cause :
Non inflammatory congestion is treated by instillation of local vasoconstrictors. They
produce immediate whitening of the eye that may last from few minutes to few hours only to
appear again. There may be rebound congestion or tachyphylexis.
Inflammatory congestion is best treated either with antibiotics or anti-inflammatory as
the case may be.
Pigmentation of conjunctiva
Commonest pigmentation of conjunctiva seen in children is staining of conjunctiva and
sclera by bile pigment in various types of jaundice including neonatal jaundice. Pigmentation
of jaundice is bilateral and most marked in the fornix. It does not require any ocular treat-
ment.
Other types of pigmentations are deposits of melanin in conjunctiva as a part of con-
genital 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 con-
junctiva by Kajal or Surma is very common in children in Indian subcontinent.
Chemosis of conjunctiva. Chemosis of conjunctiva is a very common feature of con-
junctival 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 per-
meability 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 ocu-
lar or orbital causes. It is bilateral when the causative factor is systemic. Chemosis of con-
junctiva may be mild giving a glossy appearance to the conjunctiva, moderate when the con-
junctiva 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 con-
gested when the causative factor is inflammatory. Chemosis is least marked in tarsal conjunc-
tiva due to the firm attachment of the conjunctiva to the tarsal plate. The fornices may accu-
mulate 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 hang-
ing chemosed conjunctiva. If the conjunctiva remains exposed for sufficient time it may de-
velop ulcers.
Causes of conjunctival chemosis are :
1. Allergy
(a) Exogenous allergyAs seen in seasonal allergic conjunctivitis.
(b) Endogenous allergen may produce picture similar to angio neurotic oedema.
2. Inflammation
(a) Hyper acute conjunctivitisOphthalmia neonatorum,
(b) Panophthalmitis,
(c) Endophthalmitis,
(d) CellulitisOrbit, lids
(e) Cavernous sinus thrombosis.
3. Systemic condition. Hypoproteinemea, thyrotoxicosis, myxedema, emphysema :
(a) In the head neck area, (b) fracture of paranasal sinuses, hypersensitivity to drugs,
nephrotic syndrome.
Treatment
(1) In mild cases of chemosis without infection or systemic involvement local vasocons-
trictors 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 com-
mon 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 haem-
orrhage in the hidden part of the conjunctiva may go unnoticed.
Subconjunctival haemorrhage is in fact an intraconjunctival bleeding when blood accu-
mulates 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 con-
junctival 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 conjunc-
tival 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 bleed-
ing from the ears.
(d) Remote injuries. Multiple fractures of long bone or crush injury of the chest and
abdomen may produce sub conjunctival haemorrhage generally as streaks in the fornices.
(e) Indirect injury :
(1) Rupture of conjunctival vessels : (i) Sudden recurrent rise of intra thorasic pres-
sure 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 pres-
ence 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 haemor-
rhages generally as patechea which may join to form large patch of sub conjunctival haemor-
rhage. The causes arepneumococcal conjunctivitis, membranous and pseudo mem-
branous 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 sys-
temic 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 lacera-
tion 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 con-
junctival bleeding. It happens when conjunctiva is cut or lacerated or when large sub conjunc-
tival 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 re-
sults 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 spe-
cially if they are in lower fornix or lower tarsal conjunctiva. Their presence in upper tarsal
conjunctiva denote chronic viral infection and trachoma. Presence of follicles along with
conjunctivitis is called follicular conjunctivitis which is very common in children and are
mostly self limiting. Folliculosis can be acute or chronic, in the former they last less than
one month. If follicle persists for more than one month, the condition is called chronic follicular
conjunctivitis. Number, size, position and location of follicles depend upon duration, sever-
ity 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 chil-
dren 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 con-
junctiva or caruncle.
Scarring of conjunctiva
Conjunctiva being highly vascular and lax at mostof the places. Scarring is less common
in bulbar Conjunctiva unless the wound has not been repaired well. To avoid ugly scar in
bulbar Conjunctiva, a lacerated conjunctiva should be repaired in layer i.e. conjunctiva and
Tenons capsule separately. Stitching should approximate the cut ends properly. Scarring in
palpebral conjunctiva is seen best in the tarsal conjunctiva as opaque star shaped areas
that obscure the tarsal gland and its ducts commonly in trachoma. Scarring of fornices is seen
following mechanical trauma, acid, alkali burn, Stevens Johnsons syndrome and ocu-
lar 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 con-
junctiva 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 inflamma-
tion microbial or allergic. Conjunctival discharge contains exudated fluid from dilated ves-
sels. It also contains tear, mucus, pus, micro-organism, inflammatory cells and sometimes
RBC. Conjunctival discharge varies in character in different types of conjunctivitis.
Watery discharge is seen in viral conjunctivitis, and chemical conjunctivitis of short
duration. If there is super added bacterial infection the discharge becomes thick with more
mucus and exudate.
Muco purulent discharge is seen in milder form of bacterial conjunctivitis. Purulent
discharge is characteristics of gonococcal conjunctivitis. Ropy discharge is typical of
spring catarrh. Mucinous discharge is seen in some of the dry eye syndromes.
Cytology of discharge. Identification of cells in discharge have diagnostic significance.
Predominant polymorphonuclear cells in bacterial conjunctivitis. Lymphocytic prepon-
derance 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 bleed-
ing.
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. Pre-
viously 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 chemi-
cal. 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 membra-
nous conjunctiva is xerophthalmia, symblepharon, ankyloblepharon, trichiasis and rarely
entropion.
Granulomas of conjunctiva
Granulomatous lesions of conjunctiva may be due to either exogenous causes or
endogenous causes, latter are more common. Exogenous cause of conjunctival granuloma
are - Embedded foreign bodies, both organic and inorganic. They may be inert or chemically
active. They may be small wooden particles, wings and other parts of insect body, caterpillar
hair (ophthalmia nodosa), hair from cacti, suture material and scleral implants. Endogenous
granulomas are seen in Tuberculosis, leprosy, syphilis, oculosporidiosis, burst chalazia.
Conjunctival ulcers are rare, mild and self limiting. They are seen in tuberculosis,
herpes simplex, Stevens-Johnsons Syndrome, chemical burn.
Cysts of conjunctiva
Cysts of conjunctiva can be part of congenital anomaly or acquired. They may be seen
anywhere on the conjunctiva but commonest sites are the fornices and limbus. They may be
small enough not to be noticed by the patients or large to draw attention, generally they are
painless, non tender, non reducible, translucent, may be punctured only to get filled.
Conjunctival cysts may be : 1. Congenital, 2. Acquired.
1. Congenital cystsThese are rare, they can be cystic form of limbal dermoids or con-
genital corneo scleral cysts.
2. The acquired cysts can be traumatic or non traumatic.
A. Traumatic cysts :
(i) Accidental. Following lacerated conjunctival wound that has not been repaired
properly.
(ii) Surgical wounds in squint, retinal detachment, pterygium which have not been
repaired well.
(iii) Cystoid cicatrix following glaucoma surgery or badly repaired wound at limbus.
(iv) Cyst over iris prolapse.
(v) Conjunctiva covering prolapsed, vitreous and uvea.
(vi) Phacocele. This is seen when there is corneo scleral injury and the lens with intact
capsule is extruded under the conjunctiva. It is not a true cyst.
B. Non-traumatic cyst :
(i) Lymphatic cystThese are generally small cysts anywhere in the conjunctiva without
symptom, do not require treatment.
(ii) Degenerative cystSometimes pterygia undergo cystic degeneration at apex.
(iii) Parasitic cystCysticercus and hydatid cyst.
(iv) Retention cyst.
NFLAMMATON OF CONJUNCTVA
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 limit-
ing.
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 aller-
gic conjunctivitis can be both due to exogenous or endogenous allergen. Microbial (Infec-
tious) 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 bac-
teria 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 conjuncti-
vitis.
3. Haemophilus influenzae produces subacute conjunctivitis.
4. Staphylococcus aureus produces chronic blephroconjunctivitis.
5. Coryy bacterium diphtheriae produces membranous conjunctivitis.
6. Neisseria gonococci and C. diphtheriae may have serious systemic involvement in
children.
7. Many of the non pathogenic bacteria may become pathogenic and cause conjunctivitis.
Viruses responsible for conjunctivitis in children are :
Herpes simplex, herpes zoster, measles, chicken pox, pharyngo conjunctival fever (adeno
virus 3 and 7), epidemic kerato conjunctivitis (adeno virus 8 and 19), acute haemorrhagic
conjunctivitis, molluscumcontagiosum.
CONJUNCTIVITIS
Conjunctivitis in children differ from those in adult. Some of the conjunctival infections
like ophthalmia neonatorum and diphtheretic conjunctivitis are not only vision threat-
ening, they can be life threatening also. Best classification of conjunctivitis would be on the
basis of etiology. However some of the organisms produce identical clinical picture and are
amenable to same treatment, hence for the sake of convenience infectious conjunctivitis will
be discussed according to their mode of presentation i.e. Acute, sub-acute, or chronic under
two broad heads i.e. Bacterial and Viral. Rest of the conjunctivitis would be discussed under
individual heads.
Bacterial conjunctivitis
According to mode of onset, acute infectious conjunctivitis can beCatarrhal, muco
purulent, and membranous.
Catarrhal conjunctivitis is very common among children. They involve both eyes
which are red and there is minimal discharge that is mostly serous in nature. There may be
accumulation of discharge near the inner canthus during sleep. Occasionally the lids may get
glued in the morning. It is a self limiting disease caused by bacteria of low virulence. There
are no corneal complication, no lympadenopathy.
Muco purulent conjunctivitis
This is more severe form of the former. Whole of the conjunctiva is bright red. The
congestion is most marked in the fornices and fade towards cornea. Lids are slightly
oedematous. There is no lymphadenopathy. There is muco purulent discharge with
watering. The discharge may accumulate on the lashes which get matted together. The lids
may get stuck in the morning causing discomfort, warm swabs may be needed to separate
the lids. Generally there is no pain, have mild discomfort. Excess of watering, blepharospasm,
foreign body sensation and occasional pain with onset of congestion round the limbus denotes
corneal involvement that vary from superficial keratitis, marginal keratitis to frank ulcer.
In severe infection there may be formation of pseudo or true membrane. In cases of
pneumococcal infection there may be patecheal haemorrhage.
Mucopurulent conjunctivitis is a contagious disease, transmitted directly by discharge.
The disease is caused by many organisms, generally single organism is responsible for the
disease but mixed infection is not uncommon.
Common organisms that produce muco purulent conjunctivitis are : Staphylococci,
Koch-Weeks bacilli (H. aegypti), pneumo cocci, strepto cocci, rarely pseudomonas. Pneumococci
and haemophilus influenzae are more common in children than other organisms.
They are frequent between three to eight years of age.
Management. Management is to take a conjunctival smear from the lower fornix for
Grams stain examination, culture and sensitivity. A smear stained with grams stain will
render it possible to find out if the organism is cocci or bacilli, is it gram positive or nega-
tive, is it intra cellular or extra cellular and accordingly antibiotic can be started. How-
ever when facility of smear examination is not available, clinical judgement should be utilised
to choose antibiotic for local instillation.
1. Commonly used antibiotics are :
Chloramphenicol 0.3-0.5% solution and 1% ointment ;
Gentamycin 0.3% drops ;
Framycetin 0.5% drop ;
Tobramycin 0.5% dropsciprofloxacein 0.3%;
Sulphacetamide 20% drops.
2. The discharge from the lashes and mucus from the fornices are removed.
3. There is no need to irrigate the conjunctival sac. Any of the above drops are instilled
one drop at a time in the lower fornix four to six times a day for a week. If the lashes have
tendency to stick, same antibiotic in the form of ophthalmic ointment is applied in the lower
fornix. There is no need of systemic antibiotic, however, if cornea is involved more vigorous
treatment is required along with cycloplegic. Neomycin eye drops are better avoided as ten
percent of children are sensitive to neomycin. Similarly combination of two or three drugs
should only be used if culture shows mixed infection and sensitivity to multiple drugs.
Sub-acute and chronic bacterial conjunctivitis
Sub acute conjunctivitis is caused mostly by haemophilus influenzae and less com-
monly 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 conjuncti-
vitis, Half hearted treatment in the form of irregular drops in sub-clinical strength and fre-
quency besides or resistance to the antibiotics are other factors to cause chronic conjunctivitis.
Other contributory factors areAssociated allergy, smoke, dust, uncorrected errors of refrac-
tion, 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 pre-
vention of recurrence. For complete elimination it is necessary to find out the causative organ-
ism 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 malnutri-
tion.
8OME ACUTE BACERAL CONJUNCTVT8 N CHLDREN
Membranous and pseudo membranes conjunctivitis. These two types of conjunc-
tivitis have acute onset and are bilateral. Mode of clinical presentation in the two forms is
almost same. Membranous conjunctivitis is more severe form of the two. Though many organ-
isms produce membranous conjunctivitis, diphtheretic conjunctivitis is more important
than others due to its systemic complication.
Diphtheretic conjunctivitis
9,10
. This form of conjunctivitis was very common when
immunisation against diphtheria was not available. It used to occur in epidemics. It still oc-
curs in non immunised children. Sometimes it can occur in immunised children in a milder
form. However it should be remembered that there are other causes of membranous conjunc-
tivitis that may mimic as diphtheria conjunctivitis.
Commonest age to develop diphtheretic conjunctivitis is between 2 years to 8 years.
Diphtheric conjunctivitis may present as pseudo membranous conjunctivitis as well but inci-
dence of pseudo conjunctivitis is less than true membranous conjunctivitis indiptheria.
Clinically diphtheretic conjunctivitis can be divided into (1) Stage of infiltration, (2)
Stage of membrane formation, (3) Stage of cicatrisation, (4) Corneal involvement, (5) Extra
ocular muscle palsy.
1. Stage of infiltration. Generally bilateral, the lids are swollen, hard and tempera-
ture is raised. They are tender, board like rigidity is classical of diphtheretic conjunctivi-
tis. The lid can not be separated or everted. The pre auricular lymph nodes are enlarged. It is
generally associated with diphtheretic membrane of nasopharynx.
2. Membrane formation. Changes in conjunctiva go along with the changes in the lid.
In fact the changes in lid are mostly due to changes in tarsal conjunctiva. A true membrane
develops over both the palpebral and tarsal conjunctiva. In severe cases the membrane may
slough off leaving a raw bleeding surface. No attempt should be made to peel off the membrane
as its bleeds profusely and leave a raw area.
3. Cicatrisation. This follows as the membrane heals and shrinks. The resulting scar
may produce xerophthalmia, symblepharon, trichiasis and rarely entropion.
4. Corneal complication. Involvement of the cornea occurs due to two factors : (1) The
bacteria can invade intact epithelium without trauma and cause corneal ulceration and perfo-
ration. (2) The corneal nutrition suffers due to widespread thrombosis of peri limbal blood
vessels.
5. Extra ocular muscle palsy is due to involvement of third, fourth and sixth cranial
nerves either as isolated palsy or in combination. There may be paralysis of accommoda-
tion and iridoplegia. There can be paralysis of convergence or divergence.
Management :
1. All cases of membrane formation on conjunctiva true or pseudo must be treated as
diphtheretic unless proved otherwise.
2. As diphtheria produces a potent toxin that can be neutralised by antitoxin only be-
fore it gets fixed to the tissues. Antitoxin is administered systemically and locally in
consultation with physician.
3. The toxin is not neutralised by antibiotic. Systemic antibiotic only reduces number
of bacteria thus diminishing the source of toxin production. It also eliminates other
bacteria that are seen frequently in diphtheretic conjunctivitis. The drugs commonly
used are - Penicillin G 600,000 IU BD for ten days, Erythromycin in dose of 25 mg to
50/kg for days. Other drugs used are ampicillin, clindamycin and tetracyclin.
Local treatment consists of :
1. Local administration of antitoxin in the conjunctival sac between 10,000 to 100,000
IU for 24 to 48 hours.
2. Local instillation of fresh solution of Penicillin, Erythromycin and ointment along
with antitoxin.
3. Cycloplegic should be used least keratitis develops. Once corneal involvement is no-
ticed it should be treated vigorously.
Other causes of membranous and pseudo membranous conjunctivitis :
Cause Membrane Pseudo membrane
Streptococci ++ +
E Coli +
H. Influenzae +
Pneumococci ++ +
Staphylococci + +
Gonococci +
Actinomycosis +
Candidiasis
Epidemic kerato conjunctivitis + +
Herpes zoster +
Haemorrhagic conjunctivitis +
Pharyngo conjunctival fever +
Infectious mono necleosis + +
Erythema multi formis + +
Alakali burn +
Ligneous conjunctivitis + +
Ophthalmia Neonatorum
11,12,13
Ophthalmia neonatorum or conjunctivitis in new born is a serious infection of new born.
Any conjunctivitis developing within first month of life is called ophthalmia neonatorum. It is
a notifiable disease. Etiology comprises of three groups of causative agents i.e. Bacteria, vi-
ruses and chemical.
Out of the above three, the bacterial conjunctivitis in neonate is most common. The
condition is potentially vision threatening if not managed in time. Previously Gonococcus
14
was considered to be commonest organism to cause ophthalmia neonatorum. With better
health care and antenatal screening of the mother, incidence of gonococcal ophthalmia
neonatorum has come down considerably. However it remains an ocular emergency. All
purulent conjunctivitis in neonate must be considered as gonococcal unless proved otherwise.
2
Bacteria other than gonococci that produce ophthalmia neonatorum are : Staphylococcus,
pneunococcus, haemophilus
3
, pseudomonas, and streptococcus, in fact any bacteria
that may find its way to conjunctiva of new born can cause purulent conjunctivitis. Out of all
bacteria, gonococcus has most severe form of infection and has shortest incubation period of 2-
3 days. It is seen in children born to mothers who suffer from gonococcal infection of birth
canal or there is contamination during delivery from sources other than birth passage. It is a
bilateral condition that starts as redness of the conjunctiva which is often missed because the
infant keeps the eyes closed almost throughout the day and night and has a relatively narrow
inter palpebral aperture. The disorder is noticed only after there is out pouring of muco puru-
lent discharge from the eye. The lids are swollen, rigid and may stick together. On separation
of the lids that may require use of lid retractor the conjunctiva is chemosed, may protrude
through the lids. There may be blood discharge, formation of true or pseudo membrane.
Corneal involvement is most serious vision threatening findings. Loss of lustre
of cornea is a bad sign. There may be corneal infiltration, central corneal ulcer which may
perforate in very short period. Gonococcus is one of the few organisms that can pass through
intact corneal epithelium. Once perforation has occurred endophthalmitis and panophthalmitis
is very common. Otherwise a small perforation may heal by formation of central leucoma,
leucoma adherent and anterior pallor cataract. The eye soon develops nystagmus. Even
if there is no perforation gonococcal uveitis is very common.
Neonatal conjunctivitis by other bacteria are milder than gonococcal, they have longer
incubation period i.e. 4-5 days and are generally due to secondary infection after birth.
pseudomonas infection is more common in prematures. Incidence of gonococcal conjuncti-
vitis in neonates has shown a gradual decline due to better maternal and child care and use of
prophylactic anti microbial.
Non bacterial micro organism that produce ophthalmia neonatorum are
Chlamydia trachomatous and herpes simplex. Incidence of neonatal conjunctivitis by
Chlamydia and herpes simplex is showing an upward surge. Chlamydia caused inclusion
conjunctivitis, takes five to seven days to develop. Source of infection is birth canal. It is far
milder than bacterial conjunctivitis. Corneal involvement is milder in the form of micro pannus
that may cause permanent scarring. It never perforates.
Herpes simplex conjunctivitis is also acquired from infected birth canal. If the mother
has active lesion of genital passage a caesarean sections saves the child from getting
conjunctivitis. It develops after 10 days as mild conjunctivitis, watery discharge, diffuse or
dendritic keratitis. There may be associated skin vesicles.
Diagnosis of ophthalmia neonatorum specially bacterial is not difficult. All cases of muco-
purulent bilateral conjunctivitis should be considered to be due to gonococcus unless proved to
be otherwise. Presence of gonococcus is confirmed by Grams stain and preferably by positive
culture in suitable media (Thayer Martin medium).
In all cases of purulent conjunctivitis a conjunctival smear should be taken and stained
by Gram and Giemsa stain to see if it is gram positive or negative. It also gives nature of
intra cellular or extra cellular inflammatory cells i.e. polymorph, basophils, lymphocytes and
inclusion bodies. Gram negative intracellular diplococci and polymorpho nuclear neutrophils
indicate gonococcal infection. This should be sufficient to start anti gonococcal treatment but
to find out if the stain is penicillin resistance or not and its sensitivity to other antibiotics,
culture and sensitivity test must be done because resistant stain may prove to be life threaten-
ing.
Management consists of(1) Prophylaxis, (2) Treatment of various microbial ophthal-
mia in new born.
1. Prophylaxis
15,16
. This includes (a) Antenatal screening of mother for evidence of
gonococcal, chlamydia and herpes simplex of genital tract. If found to be infected, proper treat-
ment should be initiated. In case of herpes simplex of birth canal an elective caesarean section
may be done. (b) Complete antisepsis and asepsis should be followed during and after delivery.
The eyes of new born should be cleaned by two separate wet cotton swabs, one for each eye
from lateral to medial side. (c) Prophylactic anti microbial drop :
(i) 1% silver nitrate drop (Creds prophylaxis) One drop of freshly prepared
solution (not more than seven days on the shelf) that is stored in coloured bottle
and kept in cool place is instilled in each eye. Credes prophylaxis does not al-
ways eliminate possibility of ophthalmia neonatorum. It reduces severity of the
condition. Credes method was universal prophylaxis against ophthalmia
neonatorum but has been replaced by antibiotics because AgNO
3
itself can cause
chemical conjunctivitis that mimics ophthalmia neonatorum if used in
concentration more than 2% and instillation more than two drops in each eye.
This chemical conjunctivitis develops earlier than any other conjunctivitis i.e.
within 24 hours and is mild and self limiting. Causes redness of conjunctiva,
edema of lids discharge and redness of surrounding skin.
(ii) PovidonIodine 2.5% has been claimed to be better than AGNO3 without side
effects of latter.
(iii) Antibiotic. Any of the following antibiotics can be used as drops or ointment
0.3% Ciprofloxicin, 1% Tetracyclin, 0.5% erythromycin, 0.3% gentamycin. In sus-
pected chlamydia infection of the mother 10% sulpha cetamide may be added to
any of the above.
(iv) If the mother is known to suffer from gonococcal infection, the neonates are at
high risk of developing ophthalmia neonatorum. Such infants should get single
intra muscular injection of 50,000 IU of crystalline penicillin or single dose of 1m
injection of ceftriaxone 125 mg or cefotaxime 100 mg of course the mother also
gets sufficient systemic antibiotic.
Management of clinically established gonococcal conjunctivitis
13, 16
in neonates com-
prise of :
1. Cleaning of muco purulent discharge frequently using separate swab for each eye
and each cleaning. Irrigation with balanced solution have doubtful efficacy.
2. Instillation of fresh aqueous solution of crystalline penicillin G. in strength of 100,000
unit per ml. every five minutes for six times, then every ten minutes for six instillations, then
one hourly for six instillation. Then every two hourly. As ointment of penicillin is no more
available erythromycin 0.5% may be put to reduce stickiness of lids. Other drugs like 0.3%
Ciprofloxicin, gentamycin 0.3% or any ophthalmic drop that is effective against gonococci may
be used in the same frequency. Recent studies have started doubting need and efficacy of local
antibiotic drops. They prefer systemic administration of systemic crystalline penicillin
twice a day for seven days or cefotaxime in neo natal dose in consultation with neonatologist.
3. If cornea shows any evidence of involvement 0.5% atropine ointment is put to treat
and/or prevent associated uveitis. Atropine drops are better avoided as it is likely to be
absorbed from nasolacrimal passage and throat in amount sufficient to produce systemic tox-
icity.
Treatment of other bacterial conjunctivitis :
(a) Pseudomonas conjunctivitis requires as prompt treatment as gonococcus specially
in pre mature infantsTopical gentamycin or tobramycin in strength of 0.3% as
in case of gonococcus with ointment of the same antibiotic three to four times recom-
mended.
(b) Haemophilus is best treated either by local gentamycin drops or 10-15 percent
sulphacetamide drops.
(c) Conjunctivitis with other gram positive cocci and diplococci is treated with 0.5%
erythromycin every 2 hourly.
(d) Coliform organisms can be treated with gentamycin drops.
(e) Acineto bactor
17
. In recent years hyper acute conjunctivitis similar to ophthalmia
neonatorum has been reported from various parts of the world. The organism be-
longs to family Neissericeae. It is difficult to differentiate it on grams stain from
gonococcus, both are gram negative cocci. The acinetobactor fortunately does not
cause systemic infection but is penicillin resistant. It is best treated by local 0.3%
ciprofloxacein or 0.3% Tobramycin.
(f ) Chalmydia conjunctivitis in neonate has become more common than in the past. It
is milder than bacterial infection but may have long term local as well as systemic
effect i.e. otitis, rhinitis and pneunonitis. Diagnosis of chlamydial conjunctivitis is
confirmed by Giemsa stain that shows besophilic cytoplasmic inclusion bodies. Treat-
ment comprises of instillation of 10% sulpha-cetamide drop and erythromycin 0.5%
ointment 4 times a day in severe cases erythromycin syrup in dose of 50mg/kg/day in
4 divided doses is given for 2-3 weeks.
Herpes virusconjunctivitis is uncommon but it is potentially vision and life threat-
ening infection in neonate, untreated cases may be fatal in as many as 50% of cases. Presence
of skin vesicles may point towards diagnosis of herpes simplex. Treatment with systemic anti
viral drugs is needed in consultation with neo-natologist.
Pneumococcal conjunctivitis
Pneumococci are generally present in respiratory system, but can be found as a sympto-
matic strain in conjunctival sac. It gradually produces systemic infection with ocular involve-
ment and is a common cause of hypopyon. It is one of the commonest organisms that cause
chronic dacryocystitis. It can involve the conjunctiva independent of systemic involvement. It
is mostly seen in children as acute muco purulent conjunctivitis, with patecheal haemorrhage.
It can cause ophthalmia neonatorum, membranous or pseudo membranous conjunctivitis. It is
spread by hand to hand contact in children. Management is similar to any other bacterial
conjunctivitis. Commonly used antibiotics are :
1. Fortified aqueous penicillin G. solution containing 100,000 unit per cc every hourly
during day and every four hourly by night, till the acute symptoms subside.
2. Erythromycin 0.5% may be given at night as ointment.
3. Becitricin 10,000 units per cc can also be given as hourly drop.
4. Cefazoline 50mg/cc may be given in place of penicillin.
Systemic antibodies do not seem to have any advantage over local instillation. However
every child suspected to have pneumococcal conjunctivitis should have a pediatric consulta-
tion for possible systemic involvement. Corneal involvement consists of a central ulcer with or
without hypopyon. All cases of suspected corneal involvement should be treated as emergency
with atropine, vigorous antibiotic instillation and beta blockers.
Haemophilus influenzae
18
and H. aegyptius, Koch-Weeks bacteria are common causes
of conjunctivitis in children, with acute onset that lasts for about 10 days. that can cause
patchy sub conjunctival haemorrhage. Inferior corneal infiltration is common, can have seri-
ous systemic involvement and peri orbital cellutitus. The organism is best grown on chocolate
agar medium.
Other Bacterial Conjunctivitis
Bacteria Conjunctival feature Other feature Treatment
Streptococcus Acute bilateral muco- Keratitis, and Local penicillin
purulent conjunctivitis orbital cellulitis drop. erythromycin
membranous or pseudo drop. bacitricin.
membranous conjunctivitis
ophthalmia neonatorum
Staphylococcus Hyperemia, papillary reaction Keratitis Erythromycin, sulpha
chemosis purulent or muco- cetamide. bacitricin
purulent conjunctivitis cefazolin
E. Coli Hyperemia, chemosis, acute Keratitis Chlormphenicol
mucopurulent conjunctivitis Gas in AC Gentamycin,
membrane or pseudo membrane Tobramycin
Viral conjunctivitis
Viral conjunctivitis in children can be (1) Acute or (2) Chronic.
1. Acute (i) Herpes simplex. Conjunctiva is involved in children as primary infection
mostly by type I but occasionally by type II virus as well. There are three age groups in
which herpes simplex involves a pediatric patients i.e. (1) Neonates, (2) Between six months
to 5 years, (3) Teenage.
Neonatal herpes simplex conjunctivitis can be congenital or acquired. The former
is, rare and associated with severe systemic involvement hence most of the time fatal. In
acquired form the neonate gets infection during passage through birth canal.
The child between 3 to 5 years gets infection from infected adults. The teenager gets it
by kissing an infected person and rarely as sexually transmitted disease.
Herpes simplex conjunctivitis irrespective of age of onset have cutaneous, conjuncti-
val and corneal involvement in various combinations. It is generally unilateral, incubation
period is 3 to 12 days. It develops as follicular conjunctivitis (after three months of age)
with lymphadenopathy, the eye is congested. There is watery discharge. Onset of photophobia
denotes corneal involvement. There may be pseudo membrane formation. The corneal
involvement starts as small epithelial lesions that join together to form typical dendritic
keratitis. The skin lesion consists of vesicles over the skin of the lid, forehead, lid margin. The
vesicles may cross over the mid line. The infection is self limited, it lasts for two to three
weeks. It is difficult to diagnose clinically. As herpes simplex conjunctivitis is a primary infec-
tion, the virus may pass into the trigeminal ganglion and remain dormant, recur as herpetic
keratitis later.
Treatment. As it is a self limiting disease, it does not require any specific treatment.
Corneal involvement require local use of anti viral drugs along with cycloplegic. Local
antibiotics are prescribed to prevent secondary bacterial infection. Steroids are contra indi-
cated.
(ii) Herpes zoster conjunctivitis. Herpes zoster conjunctivitis is caused by vericella
zoster virus. It is less common in children but children are not immune. The disease has
more serious corneal and lid involvement than herpes simplex. Conjunctivitis is muco puru-
lent always associated with vesicle formation on the lid. There may be actual vescicles on the
conjunctiva. The conjunctivitis to beginwith is papillary, there may be follicle formation. In
severe cases pseudo membrane develops. Conjunctivitis is self limiting and resolves in a
week. Tender pre auricular nodes develop in early stage of disease. It is always unilateral.
There may be secondary bacterial infection.
Treatment. Conjunctivitis does not require any specific treatment. Treatment is di-
rected towards the disease in toto.
(iii) Pharyngo conjunctival fever. Conjunctivitis in pharyngo conjunctival fever is
very common in children. It is a part of syndrome consisting of fever, pharyngitis, pre
auricular non tender lymphadenopathy. The conjunctivitis is follicular in nature, gen-
erally unilateral may become bilateral generally there is a watery discharge. Mucopurulent
discharge denotes superimposed bacterial infection. The lids are generally edematous. Cornea
may be involved in the form of superficial fine epithelial keratitis.
The causative viruses are adeno virus 3 and 7. Sometimes adeno virus 4 may also
cause pharyngo conjunctival fever and conjunctivitis. It sometimes develops following bath in
a contaminated swimming pool. Incubation period is 5 to 12 days.
Treatment. Conjunctivitis is self limiting lasting for seven to fifteen days. However
corneal infiltrates may persist for a month. Treatment is non specific and symptomatic.
(iv) Epidemic kerato conjunctivitis. This contagious conjunctivitis is common in
children. It is caused by adeno virus 8 and 19. Other sero types may also cause the disease.
In children it is associated with sore throat, fever and diarrhoea. It has incubation period
of 5 to 12 days. One eye is first involved and more severe than the other eye. Conjunctivitis is
follicular in nature, there may be papillary reaction and pseudo membrane formation.
Pre auricular lymphadenopathy is a common feature. The disease spreads among the school
children in epidemics. Occasionally it may be spread by contaminated instruments, lotions or
drops following ocular examination. Corneal involvement is common, it presents as sub
epithelial opacities that may appear after conjunctivitis subsides and may persist for months.
Treatment is symptomatic. No anti-viral drugs are required.
(v) Acute haemorrhage conjunctivitis. This acute conjunctivitis was first noticed in
1969 and has been known by various eponyms. The epidemics keep on appearing at regular
intervals mostly in the months of late June and early July. It is caused by entero virus type-
70. The incubation period is short i.e. 12 to 48 hours. It is most commonly seen in crowded
localities i.e. schools etc. It has an acute onset first in one eye with in few hours the other eye
gets involved. The symptoms are intense redness of conjunctiva with copious watery discharge,
lid edema and pain ranging from mild discomfort to severe pain.
Sub conjunctival haemorrhages are constant feature which generally start as small
spots on the periphery of the bulbar conjunctiva later whole of the conjunctiva gets involved.
These spots may join together and form a large sub-conjunctival haemorrhage. The patechea
disappear early, large haemorrhage take ten to fifteen days to clear. Sometimes there may be
chemosis of conjunctiva and follicle formation. There may be lymphadenopathy. Onset of
corneal involvement in form of epithelial keratitis heralds photophobia. Conjunctivitis may be
associated with fever, malaise, and body ache. Occasionally there may be uveitis, motor pa-
ralysis of lower limbs have been reported. The virus is transmitted by close person to person
contact and by fomites, water, common linens or ophthalmic instruments used to exam-
ine infected eye.
Treatment. There is no specific treatment known. The disease does not give immunity.
Recurrence in the same patient has been observed. Treatment is directed towards symptoms.
Non steroidal anti inflammatory drugs are given to relieve pain. Dark glasses help in reducing
photophobia. It is a misconception that dark glasses prevent spread. Broad spectrum antibiot-
ics are given to prevent and treat associated secondary bacterial infection. The disease is self
limiting lasting for seven to ten days. In absence of corneal involvement weak solution of
steroid drops may shorten duration.
Other viral infections that produce conjunctivitis in children :
ViralConjunctival lesions.
InfluenzaCatarrhal conjunctivitis, sub conjunctival haemorrhage.
MumpsPapillary or follicular conjunctivitis, subconjunctival haemorrhage.
MeaslesKopliks spot, follicular sub conjunctival haemorrhage.
Chicken poxMuco purulent conjunctivitis, vescicle formation, phlycten.
Molluscum contagiosumChronic unilateral conjunctivitis, pannus formation, folli-
cle formation, umblicated vesicle may be seen.
Chlamydia infection in children
19
Chlamydia (formerly known as Bedsonia) are a group of micro-organism in between
bacteria and viruses. They are intracellular organisms that are difficult to culture. Their
intracellular nature gives them partial immunity against drugs. Hence drugs are to be ad-
ministered for long time. They multiply by binary fission. Common chlamydiae are :
1. C. Trachomatis, 2. C psittacosis,
3. C pneumonae and 4. C. Lympho granuloma venerum.
The former two produce kerato conjunctivitis in children. Out of C Trachomatis and C.
Psittacosis former is more common cause of ocular manifestation while latter produces more
systemic infection. C. Trachomatis is gram negative organism that is sensitive to sulphona-
mide, it is almost exclusively human pathogen. C. Trochomatis has many types i.e. A, B,
Ba, C that produce trachoma and D to K that produce inclusion conjunctivitis also called
paratrachoma. The organism is also known as TRIC where TR stands for trachoma and IC
stands for inclusion conjunctivitis. Inclusion conjunctivitis effects patient in two distinct
age groups : 1. Neonate, 2. Young adults.
Para trachoma causes urethritis, cervicitis in adults and secondary conjunctivitis in
adults which resembles trachoma in many aspects but is milder than trachoma. it is a sexually
transmitted disease.
Neonatal chlamydiae disease is acquired by new born during birth via infected birth
canal of the mother. It produces nongonococcal ophthalmia neonatorum. The incubation
period is longer than gonococcal ophthalmia i.e. 5 to 12 days. There is lacrimation and serous
discharge, the lids are swollen. It is not prevented by usual Crede's prophylaxis but can be
prevented by tetracyclin or erythromycin. Untreated cases heal in four to five months but
may persist for years causing chronic follicular conjunctivitis and pannus formation. Infants
with inclusion conjunctivitis may develop pneumonitis and gastro-enteritis in first six months
of life.
Trachoma
20, 21, 22
Trachoma is a chronic kerato conjunctivitis due to C Trachomatous sero type A,
B, Ba and C. It is commonest preventable infectious disease of eye that may otherwise lead to
blindness in adults. It is mostly acquired in childhood. Uncomplicated trachoma in children do
not cause blindness, however secondary bacterial infection, associated malnutrition and
xerophthalmia may lead to corneal ulceration, perforation and loss of eye in children.
No race is immune. It is a bilateral condition. In children, boys and girls are equally
affected. In adults women have more serious involvement than men. Trachoma is a disease of
dry, dusty climate. It is a disease of poor children with poor hygiene. Improvement of
health care and awareness of cleanliness has drastically reduced incidence of disease in areas
where it used to be endemic. The children get trachoma from infected family members, and
playmates. The disease is transmitted from person to person by fomites. Contaminated
KAJAL, SURMA and flies play an important part in spread of trachoma in children. It has
been noticed that reduction in fly population, regular cleaning of face, availability of free flow-
ing water and ownership of flush latrine in the family reduce spread of trachoma.
29
Clinical feature of trachoma
Clinical features of trachoma in children differ from those in adults. The trachoma does
not reach the cicatricial stage in children as it takes more than decade to develop cicatricial
stage. However, most of adults acquire trachoma in childhood.
Incubation period of trachoma is short i.e. seven days (range 5 to 12 days) It begins
as mild conjunctivitis similar to bacterial conjunctivitis and indistinguishable form it. Smear
from conjunctival sac in a case of pure trachoma does not grow bacteria on culture.
Symptoms. Symptoms are mild in children unless secondary bacterial infection is su-
perimposed. Symptoms are so common in endemic areas that it is taken as natural phenom-
enon. Symptoms consist of watering, discharge, redness of the eye, moderate swelling of the
lids, pre auricular nodes may be enlarged.
Signs. Conjunctival congestion, follicular hypertrophy mostly in the fornices and
upper part of tarsal conjunctiva, papillae formation, superficial keratitis in the up-
per part and mild vascularisation.
McCallau
23, 23A
classified the conjunctival signs into following grades : (1) Incipient
trachoma, (2) Established trachoma (3) Cicatrising trachoma, (4) Healed trachoma.
Various stages are :
Stage I : Immature follicles in upper palpebral conjunctiva.
Stage II (a) : Prominent follicular hypertrophy.
(b) : Papillary hypertrophy masking follicular hypertrophy.
Stage III : Follicles and scarring at upper tarsal conjunctiva.
Stage IV : Healed trachoma. No sign of inflammation and late scarring of tarsal con-
junctiva.
Stage III and IV are generally not seen in chidden under fifteen years
Above classification has been followed for more than half a century. The classification
takes into consideration only conjunctival changes. No weightage is given to keratopathy and ;
which have great prognostic value.
WHO 1987
24
adopted a better classification.
Stage of Trachoma Clinical feature Code
Trachoma free Nil Tr D
Trachoma dubium Like early trachoma but Tr D
doubtful trachoma not confirmed
Trachoma stage I Immature follicles on Tr I
upper tarsal conjunctiva
early corneal stage.
Trachoma II Established florid trachoma Tr II
mature follicles. Papillary
hypertrophy follicles or Herberts
pits at limbus.
Trachoma Stage III Cicatrising trachoma. Tr III
Conjunctival scar. Some of the
signs of stage I and II.
Trachoma Stage IV No inflammation, disease no longer Tr IV
infectious. Scars present.
DISEASE OF CONJUNCTIVA IN CHILDREN 135
C-8\C:\N-AGE\NE-C6A.PM5
Blinding trachoma is seen in endemic areas, infection is caused by sero type A, B, Ba
and C with secondary bacterial infection superimposed. Flies in unhygienic condition where
free flowing water is not available is most important factor in causing blinding trachoma.
Trachoma in any stage does not produce perforation unless bacterial infection is superim-
posed.
WHO grading of trachoma as guidelines for treatment :
TFTrachomatous inflammationFollicular seen in children, in upper tarsal conjunc-
tiva. Presence of five or more follicles of 0.5 mm or larger is significant.
TITrachomatous inflammationIntense, pronounced inflammatory thickening of tar-
sal conjunctiva that obscure more than 50% of deep tarsal vessels.
TSTrachomatous scarring. Presence of easily visible scarring in upper conjunctiva.
TTTrachomatous trichiasis. At least on eye lash rubs on the eye ball.
COCorneal opacityEasily visible corneal opacity over the pupil.
TS TT and CO due to trachoma are not seen in children.
WHO recommended for management of trachoma
25, 26, 27
:
TFTopical antibiotic
T1Topical and systemic antibiotic
TTSurgery
Corneal changes in trachoma
Corneal changes in trachoma are early to appear. They can be due to (1) chlamydia
infection of corneal epithelium, (2) Lid changes leading to secondary changes in cornea (Not
generally seen in children).
The corneal changes are :
1. Epithelial and sub epithelial punctate keratitis, these are small in size seen gener-
ally in the upper part of the cornea. Sub epithelial punctate keratitis are large enough
to be visible without magnification.
2. Vacularization. Vascularization of cornea is superficial in nature. It consists of
epithelial invasion of conjunctival vessels with diffuse infiltration and epithelial
punctate keratopathy. Initially the vessels lie between epithelium and Bowmans
membrane, later the Bowmans membrane may be destroyed. Severity of pannus
formation depends upon duration and severity of infection. In early stage it is visible
only on slit lamp examination. The pannus of trachoma is mostly seen in the upper
part of cornea associated with cloudiness of cornea. However, it can occur at any
part. Trachomatous pannus is said to be :
(i) Progressive when cellular infiltration extends beyond the terminal end of the
newly formed blood vessels.
(ii) Regressive when the ends of the vessels extend beyond the cellular infiltration.
The cellular infiltration is lymphoid in nature with treatment the vessels disap-
pear leaving a clear cornea, only obliterated vessels may be visible on higher
magnification. A permanent haze results with destruction of Bowmans mem-
brane.
Limbal changes in trachoma
Hyperemia, edema and follicle formation are common changes in trachoma. The folli-
cles are generally small and transient, disappear without any trace but larger follicles stay for
longer time and when rupture leave depressed areas called Herberts pits which are gener-
ally not seen in children.
Diagnosis of trachoma
Diagnosis of trachoma in endemic area is easy clinically. Characteristic signs of tra-
choma in children :
1. Follicles in tarsal conjunctiva and/or limbus.
2. Epithelial keratitis.
3. Trachomatous pannus.
Combination of trachomatous pannus with any of above two is pathognomic in estab-
lished trachoma.
Early trachoma and trachoma outside endemic area present difficulty in diagnosis be-
cause there are other causes that produce similar signs.
Diagnosis of early trachoma
1. Conjunctival smear for inclusion bodies. Presence of basophilic intra cytoplasmic
inclusion bodies in epithelial cells are diagnostic.
2. Presence of immuno fluorescent monoclonal antibodies is specific.
3. Culture of C. Trachoma on McCoy cells is said to be gold standard but is very difficult
and costly.
Differential diagnosis of trachoma in children :
Consist of all case that produce following bilateral signs either alone or in combination.
1. Superficial vascularisation,
2. Follicle formation,
3. Papillary hypertrophy.
Common conditions are : Acute and chronic follicular conjunctivitis, palpebral type
of spring catarrh, multiple phlycten, riboflavin deficiency, dry eye syndrome, chronic conjunc-
tivitis, healed interstitial keratitis.
Management of trachoma in children :
Prophylaxis : (a) Chemo prophylaxis, (b) Awareness about trachoma.
Prophylaxis. Trachoma is one of major cause of blindness yet it is one of the most
treatable and preventable disease.
Chemo prophylaxis
All children in endemic area should be put on any of the local anti-trachoma chemo
therapeutic drugs :
1. 1% Tetracyclin, oxy tetracyclin ointment;
2. 1% chloramphenicol ointment,
3. 0.5% Erythromycin ointment twice a day for six weeks. Then a gap of six weeks is
given and the regime is repeated. Rifampin eye ointment, ciprofloxin eye oint-
ment have also been found to be effective. In the mean time all the other members of
community who have active trachoma are treated for trachoma.
Trachoma awareness
28, 29, 30, 31
. It is emphasised that trachoma is :
Preventable and curable disease.
Preventing use of KAJAL and SURMA from common container.
Face washRegular face wash with running water greatly reduces incidence of tra-
choma.
Vector control. Fly is commonest vector that disseminate trachoma. If fly density can
be reduced in the community trachoma can be eliminated.
Environment. Availability of running water, ownership of septic latrine in the family/
community reduces incidence of trachoma.
29
Treatment of trachoma
C. Trachoma is sensitive to both sulpha and many broad spectrum antibiotics. As
the organism is obligatory intracellular in nature prolonged treatment in effective dose is
required.
Local chemotherapeutic drugs (TF Stage) :
1. Sulpha cetamide drops 10% to 20% 4 times a day in both eyes is prescribed for six
weeks.
2. Chloramphenicol 0.5% drop or Ciprofloxin 0.3% drop 4 times a day for six weeks.
3. Tetracyclin 1% oxy tetracyclin 1%, Chlortetracyclin 1%, ciprofloxin 0.3% ointment
three times a day alone or at the bed time along with local sulpha or antibiotic drops
for six weeks.
Oral antibiotic agentsIn TT stage :
1. Sulpha methoxazole 30mg/kg in divided doses for 3 weeks along with local antibiotic
drops.
2. Tetracyclin 15mg/kg in divided dose along with local antibiotics for 3 weeks not
given under eight year of age.
3. Erythromycin 30 mg/kg in divided dose for 3 weeks.
4. Doxycycline 1.5 mg/kg single dose for 15 days.
5. Azithromycin 250 mg of single dose is said to be as effective as 1% tetracyclin TDS
for six weeks.
Systemic Tetracyclin and doxycycline should not be used in children under eight years
of age.
WHO schedule for management of trachoma in endemic area consist of SAFE method.
where S = stands for surgery
A = stands for local and systemic antibiotic
F = Face wash (personal hygiene)
E = Environmental changes (availability of running water, disposal of human
waste, vector control)
Surgery is not required in children.
Allergic conjunctivitis in children
32
Allergic conjunctivitis is almost pandemic ocular disorder. Its frequency in children
may surpass infective conjunctivitis in developed countries. In under developed countries its
presentation and outcome may be over shadowed by prevailing infective conjunctivitis and
vitamin A deficiency. Presenting features of allergic conjunctivitis are variable, its symptoms
may be so mild and insidious that it is accepted as natural phenomenon or may be so trouble-
some as to cause loss of manpower in adults and absenteeism from school in children.
All allergic conjunctivitis areself limiting, duration vary from few minutes to few
years. Allergic conjunctivitis are generally bilateral, may be asymmetrical. No race, sex or age
is immune.
Clinical presentation
33, 34
Allergic conjunctivitis is influenced by many factors which may work separately or in
various combinations. These factors areGeography, climate, race, age, sex, genetic predispo-
sition and reaction to allergen.
1. Immediate hypersensitivity (humoral),
2. Delayed hypersensitivity (cellular) or
3. Auto immune.
Allergic conjunctivitis may be seen both in atopic and non atopic patients. The allergen
responsible for immediate (humoral) hypersensitivity are exogenous. Those responsible for
delayed (cellular) hypersensitivity are endogenous. The exogenous allergen are organic mat-
ters either of vegetable origin i.e. pollen, hay, husk, moulds or of animal origin i.e. fur, wool,
dust, mites, dander, drugs, cosmetics, plastics. Polymers are also capable of producing allergic
conjunctivitis. Food and additives are known to produce allergic conjunctivitis. Many a times
it is impossible to find out the allergen.
Exact mechanism of allergic conjunctivitis is not well understood. Factors that make
conjunctiva vulnerable to allergic reaction are :
(i) Conjunctiva is most exposed mucous membrane of the body.
(ii) It is rich in blood supply.
(iii) It is very loose.
(iv) It is rich in lymphatic.
(v) It is constantly bombarded with exogenous allergen.
Most accepted theory regarding allergic conjunctivitis is that exogenous allergens dis-
rupt the mast cell membrane and cause degeneration, releasing a host of chemicals that
causevasodilatation, edema, inflammation and exudation. The chemicals released in this
process arehistamine, various prostaglandins, leucotriens. The reaction is type one allergy
and IgE mediated. In milder cases there is preponderance of mast cells. In severe cases there
is increase in T cell lymphocytes.
Classification of allergic conjunctivitis is difficult. However, following classification may
fulfil many of the clinical criteria and possible mode of management.
1. Immediate (humoral) hypersensitivity
33
(a) Acute allergic conjunctivitis.
(b) Seasonal allergic conjunctivitis.
(c) Perennial allergic conjunctivitis.
(d) Vernal kerato conjunctivitis.
(f) Atopic kerato conjunctivitis.
(g) Giant papillary conjunctivitis.
All are caused due to exogenous allergen. The first three are more common, less
troublesome and easy to manage than latter three which are less frequent but diffi-
cult to manage.
2. Delayed hypersensitivity (cellular)
3. Auto immune disease
(a) Cicatricial pemphigoid (seen in elders)
(b) Erythema multi formis (Stevens Johnson syndrome) minor and major.
1. Acute allergic conjunctivitis. Acute allergic conjunctivitis is equivalent to urticaria
of the skin. It is very common among children. It can manifest as early as first year of life. It is
due to large inoculum of exogenous allergen. The patient complains of sudden onset of copious
watering from the eye which may be unilateral or bilateral. Fast developing chemosis of con-
gested conjunctiva. The conjunctiva may be so edematous as to protrude through the inter
palpebral fissure. The cornea forms the floor of the crater (well) of the chemosed conjunctiva.
The lids are swollen. There may be moderate papillary reaction, no follicles are seen. Cornea
remains unaffected. There is no anterior chamber reaction, vision may be blurred due
to over hanging chemosed conjunctiva. There is no lymphadenopathy. The symptoms pass off
within half an hour to one hour without leaving any trace. Muco purulent discharge, corneal
involvement and lymphadenopathy point towards anti microbial conjunctivitis.
Treatment. Best is to avoid the allergen if it is known, otherwise local vasoconstrictors,
astringent and short acting systemic antihistamine tablets are sufficient. Cold compress ap-
plied within few minutes may give relief. Local steroids are not necessary. Mast cell inhibitors
have no role, recurrence are common.
2. Seasonal allergic conjunctivitis. Seasonal allergic conjunctivitis is equivalent to
hay fever, hence may be associated with rhinitis, sore throat. They have seasonal
predilection. Conjunctival reaction consists of mild to moderate papillae or follicle formation.
Cornea is bright. There is no AC reaction. There is no lymphadenopathy. Condition is
invariably bilateral with mild itching and slight ropy discharge. Conjunctival scrapping
show eosinophils. Symptoms coincide with release of allergen in the atmosphere (pollen,
hay etc.) Main symptoms are itching, redness, watering. The condition is to be differentiated
from trachoma, dry eye syndrome which are not seasonal nor produce itching.
Management of seasonal allergic conjunctivitis consists of :
(i) Oral non sedative antihistamine. Possible side effects of anti histamines should be
weighed against its therapeutic benefits while prescribing to children specially when
administration has to last longer than few days.
(ii) Instillation of combination of astringent, vasoconstrictor, antihistamine several times
a day.
(iii) Local mast cell inhibitor 2% NedoCromil two times a day is better than 4% sodium
cromoglycate BD 2% cromoglycate has less therapeutic value. It is best used as pro-
phylactic in between the attacks three to four times a day. Mast cell inhibitors are
almost devoid of any side effect.
(iv) NSAID like ketorolac tromethamine 0.5% drops three times or Flurbiprophen 0.03%
drops three times a day.
(v) Steroids are best avoided.
Perennial allergic conjunctivitis
As the name suggests the condition lingers throughout the year without remission or
exacerbation. In the past perennial and seasonal allergic conjunctivitis were grouped under
single category of chronic allergic conjunctivitis. Seasonal allergic conjunctivitis have definite
seasonal predilection which lacks in perennial conjunctivitis. The allergen in perennial aller-
gic conjunctivitis is present in the environment throughout the year which could be vegeta-
ble, animal or chemical in nature like house dust mite, animal dander, food allergen and
locally used drugs.
The symptoms are : (i) Itching, redness and watering. (ii) Signs comprise of conjuncti-
val congestion, follicle formation and papillary reaction. Cornea is never involved. The condi-
tion may be confused with trachoma, dry eye syndrome or angular conjunctivitis.
Management consists of removal of offending allergen which is not always possible.
Local NSAID, mast cell inhibitor specially long acting Nedocromil 2% two times a day are
effective. Low dose of non sedative antihistamine tablets give additional relief. In milder cases
local anti histamine drops in combination with long acting vaso constrictor are sufficient. Ster-
oids are best avoided.
Vernal (spring) kerato conjunctivitis
34, 35, 36
It is most troublesome allergic conjunctivitis in children. In spite of its name it need not
manifest in spring always. It is more common in summer in tropical and sub-tropical coun-
tries. It is a bilateral condition, may be a symmetrical specially when it develops first time. It
has remission and exacerbations. It has been reported as early as first year of life. More
common age is between six years to sixteen years, after which it may automatically heal. It
is a self limiting disease lasting for six to ten years. It is more common in boys who outnum-
ber girls in ratio of six to one. In girls the condition is milder, duration is shorter. It is more
common in coloured races and in warmer climate. The disorder is seen in two age groups
i.e. pre puberty which forms the bulk of patients, and post puberty. The post puberty cases are
less in number, generally not seen in females, are less severe and last for shorter i.e. two to
three years. Some authors consider this as variation of atopic kerato conjunctivitis. There
may be a positive history of vernal catarrh in the parents. Sometimes more than one
sibling may be affected. Atopic dermatitis is a frequent dermatological disorder in children
with spring catarrh.
Topographically there are three types of vernal kerato conjunctivitis :
1. Palpebral (tarsal). This is most common.
2. Bulbar (limbal). This causes early and more corneal involvement is more. Common
in black races.
3. Mixed. Least common but most difficult to treat.
Symptoms. Commonest symptom is intense itching that may last throughout the day.
Besides discomfort itching becomes social embarrassment to the child. Itching is associated
with redness that becomes more intense following itching, there is a ropy discharge. There
may be watering from the eyes. The child finds it difficult to open the eye in the morning but
the lids do not get stuck as seen in acute bacterial conjunctivitis. Itching and watering leads to
excoriation of the outer canthus. After sometime a pseudo ptosis develops which gives
sleepy look to the child. The skin over the lid may be darker than the surrounding area.
Most frequent physical finding is presence of moderate size papillae in upper tarsal
conjunctiva, these papillae are flat topped in the area that come in contact with the globe
firmly and pointed on the lateral side of the tarsus. The flat topped papillae are polygonal in
shape. In between adjacent papillae there are deep furrows that zigzag in between the papil-
lae. There may be pseudo membrane over the papillae (Maxwell Lyons sign)
35
in un-
treated cases. With treatment the papillae shrink in size both in height and width. The surface
loose its roughness and becomes velvety. There is a tenacious ropy discharge which is
alkaline in nature. The discharge can be pulled out in form of fine threads. The discharge
which fills the gap between the papillae, the appearance is called milk spilled over cobble
stone as the large papillae have appearance of cobble stone. The papillae are not seen in the
lower lid or fornices.
In the bulbar form there are mucoid confluent nodular papillae at the limbus. Most
commonly at the upper part, these nodules are generally seen in segments but may encircle
whole of the cornea. In untreated cases there may be formation of Horner-Trantas spot
35
which are discrete white spots at the limbus.
The corneal changes in spring catarrh are the cause of lacrimation, photophobia and
diminished vision.
The corneal changes in spring catarrh comprise of following, they may be present
as isolated lesion or in various combination :
1. Epithelial micro erosion generally seen in the upper part of cornea 1mm inside the
limbus.
2. Epithelial macro erosionThere is large area of epithelial loss leading to sterile
ulcer formation due to their shape. They are called shield or amaeboid ulcers seen
in the upper part of the cornea.
3. Plaque over the macro erosion.
4. Superficial vascularisation.
5. Sub epithelial scarring.
6. Pseudo arcus. This is segmental in nature.
7. Keratoconus has been observed more frequently with vernal kerato conjunctivitis.
The exact cause of keratoconus is not understood, may be a genetic feature. Fre-
quent rubbing of the eye has also been blamed for development of keratoconus.
Differential diagnosis. Generally there is no problem in diagnosis of vernal kerato
conjunctivitis due to its typical clinical feature of chronic, bilateral, papillary reaction in a
male child intense periodic itching with remission and exacerbation that responds dramati-
cally to local steroid drops. Common conditions that mimic as vernal conjunctivitis areOther
type of allergic conjunctivitis, chronic follicular conjunctivitis, dry eye status, acnerosacea,
blepharo conjunctivitis, trachoma, drug induced conjunctivitis. In under developed countries,
clinical features may be overshadowed by infective conjunctivitis and xerophthalmia.
Management
35, 38, 39
Management of vernal kerato conjunctivitis has special significance as it is seen mostly
in children, is chronic and requires prolonged treatment. Children with vernal conjunctivitis
try to skip school as perpetual redness of eyes and constant itching is not socially acceptable to
the children. Parents should be informed that the condition is recurrent, runs a prolonged
course, is self limiting and by itself does not produce blindness but blindness can set in by
indiscriminate use of steroids that gives prompt relief.
1. Parents should be persuaded to use nonspecific astringents vasoconstrictor and an-
tihistamine drops in various combinations as local drops rather prompt relief giving
steroids.
2. Frequent cold compress.
3. Dark glasses.
4. If these fails mast cell inhibitors are added to above treatment. Sodium cromoglycate
2%-4% three to four times a day gives comfort to the child. Chromoglycate sodium in
two percent strength can be used as prophylaxis in between exacerbation.
Nedocromil sodium 2% BD has better effect that 4% sodium cromoglycate.
Cromoglycates are mast cell inhibitors have almost no side effect.
5. Non steroidal anti inflammatory drugs have been used with good result. They
have reduced use and dependence on steroid. They lack side effects of steroid are
well tolerated.
Drugs used are :
1. Flurbiprophen 0.03% two to three times a day.
2. Ketorolac tromethamine 0.5% two to three times a day. Diclofenac sodium 0.1% may
be used above twelve years of age.
3. Systemic aspirin, Ibuprofen, indomethacin, nemesulide have been used without uni-
form result.
4. In spite of all drawbacks local steroids have remained most effective treatment of
choice. Local steroids are used as clear solution. Drawback of suspension is that the
suspended particles get lodged in between the papillae and act as irritant. The prin-
ciple of local steroid therapy is to use weakest solution used least frequently should
give maximum relief without side effect. Commonly used steroids used are -
Dexamethasone/Betamethasone 0.1%
Prednisolone0.125% to 0.5%
Fluorometholone 0.1%
In severe cases any of the above drugs is instilled in conjunctival sac every two hourly
for first twenty four hours, then frequency is gradually reduced over weeks. After a month the
child may be comfortable in once a day instillation. All children under topical steroid should be
under constant supervision of ophthalmologist who should monitor - vision, error of re-
fraction, intra ocular pressure and any change in the disc. The lens should be examined
for iatrogenic cataract.
Addition of mast cell inhibitors reduces requirement of steroid without jeopardising
result.
8. Other drugs used in vernal kerato conjunctivitis are :
(i) Acetyl cysteine two to ten percent solution in methyl cellulose four times a day
dissolves mucus.
(ii) Weak solution of acetic acid to change pH of tear from alkaline to acidic.
(iii) Tear film substitutes are given to manage ocular surface disorder that is too
common.
(iv) Cyclosporine 2% in castor oil may be used in those cases that do not respond to
steroids. These cases are generally associated with atopia.
(v) Shaving of papillae, cryo application to the papillae have not found to be very
effective.
(vi) Plano T contact lenses may be helpful in persistent superficial keratitis.
(vii) Rarely low dose systemic steroid for a short period, may have to be used in se-
vere corneal involvement under strict supervision.
Atopic kerato conjunctivitis
Fortunately this condition is rare, mostly seen in atopic adults but children in second
decade may be affected. It has more systemic symptoms of atopic disease than ocular symp-
toms. Ocular manifestation consist of blepharitis, papillae formation, corneal epithelial defect,
corneal plaque and atopic cataract, keratoconus. Systemic signs consist of eczema in the neck,
antecubital fold, asthma, rhinitis.
Management consists of local low dose of steroid, sodium cromoglycate, sodium
nedocromil, NSAID. Some cases may require topical cyclosporin in oil two times a day.
Giant papillary conjunctivitis
41, 42, 43
The malady was first reported in 1974. By 1977 a syndrome consisting of contact lens
intolerance, itching, mucus discharge, hyperemia, blurred vision has emerged. On
examination large papillae that are larger than 3mm across are seen in upper tarsal conjunc-
tiva. The symptoms diminished on discontinuation of contact lens. Severity depends produce
more encounter in on size of contact lens. Larger the lens more are the symptoms. However it
was realised that contact lens was not the offending agent. It was the preservative and protein
deposits on the lens that were the causative factors. Extendedwear lenses produced clinical
features. It is not associated with atopia. It can affect any age both sexes are equally effected .
On long run tops of papillae ulcerate and stain with fluorescein. Besides contact lens giant
papillary conjunctivitis is also encountered in protruding mono filament sutures, corneal scleral
shell, artificial eye, kerato prosthesis, ocular prosthesis, scleral buckle, cyno acrylate glue,
multiple embedded foreign bodies.
Management comprises of discontinuation of CL, change of CL polymer and design,
proper contact lens hygiene. Use of preservative free contact lens solution, steroids are best
avoided. Local NSAID and cromoglycates give sufficient relief.
Phlyctenular kerato conjunctivitis
44,45,48
This is very common allergic conjunctivitis seen world-wide, more commonly in un-
der developed countries where tuberculosis is endemic, malnutrition is frequent and
lack of public health facility common. It is a cell mediated type four allergic reaction
where sensitised corneal and conjunctival epithelium when exposed to causative endogenous
allergen develops interstitial kerato conjunctivitis in the form of a nodule. Tubercular
protein is commonest causative factor. The allergic response is attributed to lipid fraction
of tubercular antigen. Other causative organisms that can produce phlycten are
46
staphylococcus, strepto coccus, gonococcus, candida, coccidomycosis, lympho granuloma,
ascariasis. Rarely it can be idiopathic as well. Common age to develop phlycten is between
three to ten years. After ten years frequency becomes less. After fifteen it becomes very rare.
Girls are effected more than boys. It is a bilateral disease, both eyes may be involved simul-
taneously, or separately. Number of nodules vary from single to multiple when more than
one nodule is present they may be away from each other or may coalesce to involve a sector of
limbus or in severe cases they may encircle the whole of the cornea. Phlyctens are self limit-
ing in nature. They resolve in ten to fifteen days, never to recur on the same spot. However
there may be crops of lesions one after another.
Perilimbal conjunctiva is the commonest site to develop phlyctenular conjunctivitis. It
has been observed on the bulbar conjunctiva as well as tarsal conjunctiva and inter marginal
strip. Other frequent part to be involved is cornea either primarily or due to spread from
conjunctiva.
Typical conjunctival phlycten develops one to two millimetres away from limbus as
a pale vescicle that form a nodule later
44
. The initial size may be that of a pin head, soon it
enlarges to 1 mm to 2 mm. The conjunctiva round the nodule is congested, conjunctival vessels
do not pass over the nodule. Two types of presentation are encountered i.e. only phlycten
without muco purulent conjunctivitis or phlycten developing in infective conjunctivitis. Role of
infective conjunctivitis in production of phlycten is not clear. Most of the time it is coincidental.
Other possibility is that due to infection conjunctival epithelium gets sensitised and a phlycten
ensues. Acute infective conjunctivitis may facilitate transfer of large amount of tubercular
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protein to the limbus that results in formation of phlycten. Within four to five days the phlycten
reaches its maximum size and the surface epithelium gives way to form an ulcer which takes
another three to four days to heal without any scar on the conjunctiva. Though tuberculosis
has been blamed as major cause of phlycten. Tubercle bacilli have never been isolated from the
conjunctiva in phlyctenular keratoconjunctivitis. The histological changes do not show tubercle
formation it resembles more or less of a micro abscess.
Other form of involvement in phlycten is when it develops at the limbus. It may either
spread from conjunctiva and spill over the cornea or may develop directly on the limbus result-
ing in phlyctenular kerato conjunctivitis which is more congested than simple phlyctenular
conjunctivitis.
Corneal involvement in phlycten can be of following types :
Cornea may be involved as (1) Primary lesion (2) The lesion may spread from conjunc-
tiva. There are two main types of corneal involvement i.e. (1) Ulcerative (non vasularised)
and (2) infiltrative (vascularisation)47. The ulcerative form may be (1) Marginal, (2) Fascicular.
Marginal corneal phlycten is generally due to a phlycten developing at the limbus. The
phlycten sits astride the limbus i.e. half on cornea, half on conjunctiva. Like any phlycten in
due course it ulcerates and heals with out any scar. However if the ulcer is deep it destroys the
Bowmans membrane and a permanent opacity is formed.
An opacity due to marginal phlycten has a typical dome shaped configuration with base
at the limbus and convexity towards the pupil. There may be single or multiple marginal
phlyctens. More than one may coalesce to form a sectorial ulcer.
Fascicular ulcer is a typical corneal presentation of phlycten. It is generally preceded
by marginal or keratoconjunctival phlycten. The ulcer starts on the corneal periphery, has a
tendency to move towards centre followed by a leash of superficial vessels. The vessels are
typically straight and do not branch. The fascicular ulcer while shifting from periphery to-
wards pupil digs deep in the substance of cornea creating a shallow furrow. The vessels de-
velop in this furrow. There may be more than one fascicular ulcer in the same cornea. The
fascicular ulcer does not perforate, nor does it produce anterior chamber reaction. The final
location of the ulcer where it may become stationary is uncertain. Once it stops shifting it
starts healing, leaving a superficial opacity followed by fainter band shaped opacity extending
upto limbus. This band represents the place of leash of vessels that followed the ulcer.
Third rare possibility is small pin point superficial ulcers all over the cornea. Infiltrative
type of corneal phlycten may produce two types of vascularisation : First more common
superficial vascularisation and a less frequent deep vascularisation. The superficial
vascularisation may develop anywhere on the cornea even all around the cornea and called
phlyctenular pannus. It differs from trachomatous pannus in the sense that latter has
predilection for upper cornea. The vascularisation ultimately dies down leaving no scar. In a
more severe case of longer duration there may be deep vascularisation which leaves permanent
mark. Corneal involvement is more when tubercular protein is causative factor.
Symptoms. Symptoms of phlyctenular kerato conjunctivitis depend upon number of
phlycten; associated bacterial conjunctivitis, and involvement of cornea. Simple conjunctival
phlyctens do not produce much symptom except localised redness in the eye, slight irritation
and watering. Associated infective conjunctivitis invariably overshadows simple phlycten that
may be missed altogether or it may become obvious when conjunctivitis has subsided. Though
phlycten is an allergic manifestation it does not produce itching. Presence of itching with
nodule at limbus points towards limbal spring catarrh.
Symptoms become more severe as cornea gets involved. A phlyctenular kerato conjunc-
tivitis produces severe photophobia in children. Fascicular ulcer is associated with severe
blepharospasm, photophobia, lacrimation and diminished vision. Vision is greatly reduced if
the ulcer is positioned against the pupil.
Management
1. Simple phlycten without infective conjunctivitisLocal weak solution of steroid i.e.
dexamethasone 0.1% 4 hourly for first day. This reduces symptoms promptly or FML
0.1% every one to two hourly till symptom subsides then the frequency is reduced.
2. Phlycten with infective conjunctivitis is treated as any catarrhal conjunctivitis along
with local steroids under supervision.
3. If cornea is involved atropine is added to steroid to reduce ciliary spasm, this gives
prompt relief. Keratitis due to tubercular antigen though produce severe symptoms
responds better than in cases of staphylococcal antigen induced keratitis.
4. In under developed countries all children with phlycten should be investigated for
both pulmonary as well as extra pulmonary tuberculosis. BCG does not prevent a
child from developing phlycten. If the child is found to be tubercular a full course of
anti-tubercular treatment should be ensured.
5. In case of staphylococcal allergy lid hygiene along with systemic anti staphylococcal
antibiotic may be required. Children older than eight years should be put on
doxycycline 100 mg once a day for 10 days while smaller children are put on erythro-
mycin in dose of 25/mg/kg for seven to ten days along with local steroid.
6. Children suffering from intestinal helminths should get a course of anti helminth.
Albendazole is an effective drug. This is given in form of chewable tablet in single
dose. Children above 2 years should get 400 mg single dose to be repeated as re-
quired.
Differential diagnosis of phlycten comprise of nodules at limbus, trachoma, spring
catarrh, interstitial keratitis.
Complication. Conjunctival phlycten is devoid of any complication. Corneal complica-
tions consist of peripheral dome shaped opacity, Salzmanns nodular dystrophy
46
, corneal
vascularisation and various grades of corneal opacities that may diminish distant vision lead-
ing to squint and amblyopia.
Erythema multiforme
49, 50
Though this disorder is classified along with allergic conjunctivitis. It is not a true allergic
conjunctivitis. The exact mechanism is not known. Most acceptable theory is an auto immune
disease.
51
It differs from both endogenous and exogenous allergic conjunctivitis by not
producing - papillae, follicles or nodule. There is no itching either. It has profound systemic
manifest, mostly cutaneous and mucous membrane involvement. It is a disease of children
and young adults. There are two types i.e. (1) erythema multiform minor (2) Erythema multi
forme major (Stevens Johnson syndrome) The latter is not only bilateral sight threatening,
it can often be fatal. Etiological factors that can culminate into this troublesome disease are :
1. Drugs. Many drugs that are routinely used for day to day management of various
symptoms can cause Stevens Johnson syndrome. Top of the list is taken by sulpha
drugs including acetazolamide others are penicillins, phenylbutazone barbiturates,
salicylates.
2. Infection. (i) Recurrent infection by herpes simplex type I and II. (ii) Respiratory
infection with mycoplasma pneumonae.
3. Idiopathic.
The disease has three stages :
1. Prodromal 2. Vesciculo bullus stage 3. Complication.
The pro dromal stage consist of malaise, sore throat, fever, joint pain and swelling.
In second stage. The muco cutaneous involvement consists of erythematous macules
on dorsal hand and feet, exterior surface of forearm and legs. The lesion soon becomes papular,
which become bullus. Any part of the mucous membrane may be involved. The lesions are
generally symmetrical. This stage lasts for seven to fifteen days. Healing starts within a few
days and heals completely. Recurrence has been reported if the child is exposed to same pre-
cipitating factors.
The ocular lesions
Lesions of the eyes seen in 50% of cases. It is bilateral, generally symmetrical, starts as
muco purulent conjunctivitis that may become purulent. There may be vescicle formation.
Due to vasculitis part of the conjunctiva gets infarcted. There may be formation of pseudo
membrane or true membrane. Shedding of these membranes leave raw areas in the
conjunctiva. When two such raw areas come in contact with each other adhesion develop. That
result in formation of symblepharon. Shrinking of conjunctiva leads to trichiasis, entropion,
obliteration of lacrimal ducts, keratanisation.
Complications. Erythema multi form minor may pass off without any complication.
However Stevens Johnson has far reaching vision threatening complication like xerosis of
conjunctiva and cornea, keratanisation of cornea, vascularisation and opacification of
cornea.
Management. There is no effective treatment of ocular involvement. Local steroid may
minimise vasculitis and prevent conjunctival infraction which in turn reduces formation of
pseudo membrane. Attempts should be made to prevent adhesion between two raw surfaces
by instilling bland lotion systemic steroid started in early stage are life saving and minimise
scarring.
Conjunctivitis of unknown causes in children :
1. Folliculosis. This is not a true conjunctivitis. It is a benign, bilateral, non-infective
follicular hypertrophy in children. The follicles are small, seen mostly in lower fornix, tarsal
conjunctiva is not much involved. Cornea is never involved. There is hardly any discharge.
There is no itching. It is to be differentiated from other causes of follicles and papillae formation
like chronic follicular conjunctivitis, trachoma and spring catarrh. The condition does not require
any treatment except reassurance to the parents. Chronic follicular conjunctivitis is an
infective conjunctivitis of under nourished children. It is a bilateral condition. The follicles are
numerous both upper and lower tarsal conjunctiva are involved. There may be minimal
discharge.
2. Iatrogenic conjunctivitis (Drug induced). It is a non specific conjunctivitis fol-
lowing prolonged administration of host of commonly used local drops that include - pilocarpine,
atropine, home atropine, idoxuridine, neomycin, gentamycin sulpha cetamide. It is generally
associated with dermatitis of the lid margin and excoriation of the outer angle. There are some
follicles which may be associated with itching and confused with non drug induced allergic
conjunctivitis. Treatment consists of stopping the offending drug and replacement by non-
toxic drug. Weak solution of steroid. In spite of treatment follicles may persist for few days to
weeks.
3. Ligneous conjunctivitis. This is rare type of conjunctivitis seen in children may be
seen soon after birth or develop any time even in adults. It is more common in girls, is gener-
ally bilateral but asymmetrical. Most important feature is formation of membrane on the tar-
sal conjunctiva that gives it a hard feeling. There may be formation of granuloma on the tarsal
conjunctiva. Exact cause of this condition is not known. However chronic infective process and
hypersensitivity are two important factors. There is no satisfactory treatment. Initially the
condition may be confused with membranous or pseudo membranous conjunctivitis and Stevens-
Johnson syndrome.
Granulomas of conjunctiva
Conjunctival granuloma are seen in chronically inflamed conjunctiva. Common cause
in children are :
1. Burst chalazia, 2. Foreign body, 3. Rhinosporidiosis,
4. Tuberculosis, 5. ophthalmia nodosum (caterpillar hair),
6. Candida, 7. Coccidomycosis, 8. Ligenous conjunctiva.
Rhinosporidiosis
52, 53
(Oculo sporidiosis)
Rhinosporidiosis is caused by fungus Rhinosporidium Seebri. Ocular involvement is
seen mostly in children in endemic areas. The disease has wide distribution mostly in trop-
ics with moderately to heavy annual rainfall and high humidity. Almost all mucous mem-
branes of the body are known to be involved including conjunctiva and lacrimal sac; lesions
of skin and bone have also been reported. The spores of the organism get implanted in the
mucous membrane and develop into sporangia, a mature sporangium may contain as many as
16,000 spores.
Ocular involvement is said to be primary
54
when there is no other evidence of the
disease in any part of the body except eye and its adnexa. This is also known as
oculosporidiosis. In secondary rhinosporidiosis of the eye the disease spreads from the
nasopharynx to the sac.
Ocular structures involved in rhinosporidiosis are - conjunctiva, lacrimal sac and
sclera. Combined involvement of sac and conjunctiva is very rare. Conjunctival involvement
is generally primary.
Typical conjunctival lesion is a gradually developing painless granuloma in the conjunc-
tiva. Commonly involved are fornices and tarsal conjunctiva. Bulbar conjunctiva is rarely
involved. The lesion is more common among boys between 5 to 15 years, who have habit of
taking bath in a pool shared by cattle which are invariably infected.
The growths in the tarsal conjunctiva are flat and sessile, those at limbus or bulbar
conjunctiva are round. While those arising from fornices have long thin pedicle with finger
like projection. All the lesions are bright red in colour, have rough surface dotted with samolina
(sujee) like white dots that represent sporangia. The lesions bleed on slight trauma. Generally
there is only one granuloma in an eye. Bilateral conjunctival involvement is rare. The size of
the growth varies from few millimetres to few centimetres.
Symptoms. Main symptoms are bleeding in the conjunctiva and visible red mass. If
the growth hangs over the cornea, it may cause lacrimation. The lesions are non tender and
painless. They are fragile, bleed on slight manipulation like everting the lid.
Diagnosis. Diagnosis of conjunctival rhinosporidiosis is simple in endemic areas. Com-
mon lesions that form differential diagnosis are burst chalazion, foreign body granuloma,
haemangioma, papilloma. However reverse is also true i.e. above conditions may be mistaken
as rhinosporidiosis in endemic area.
Diagnosis is best confirmed by histopathology of the excised tissue. Other useful
diagnostic method is to examine small piece of tissue dipped in saline under high power that
show sporangia. Sometimes free spores are demonstrated in the tears of affected child.
Involvement of lacrimal sac in rhinosporidiosis
52,53,54
is more common than conjunctival
involvement. The sac can be involved either as primary lesion i.e. without any involvement of
nasopharynx or as a secondary spread from nose and throat. Involvement of the sac is in the
form of chronic dacryocystitis where the growth arises from the mucosa of the sac and fills the
sac to distend it, that presents as a diffuse non tender swelling on the medial side of the eye
mostly below the medial palpebral ligament, may spread under the lower lid. The swelling is
compressible but non-reducible, does not change in size on coughing or sneezing. The skin over
the swelling has orange peel appearance. Temperature over the swelling is not raised. Regur-
gitation test is negative, complete block of nasolacrimal duct is rare. Erosion of surrounding
bone is common in long standing cases. If sac is secondarily infected it presents as cellulitis.
This generally happens following syringing. Bilateral involvement is rare. Exact mechanism
of sac involvement is not clear specially as a primary lesion. Partial congenital obstruction of
nasolacrimal duct, chicken pox or measles are contributory factors in endemic area. A narrow
or obstructed nasolacrimal duct due to any of the above contributory factors allows the spore
to settle down in the sac where it reaches via puncta and canaliculi. Once the spore reaches the
sac it forms a nidus over which the growths develops. (See page 93-94 as well)
Scleral involvement is most rare. It is always associated with a conjunctival growth that
hangs over the scleral lesion which is in the form of a staphyloma. The staphyloma is located
anterior to the equator. Occasionally it is visible on indirect ophthalmoscope.
Management. There is no known drug that is effective against rhinosporidiosis. Sur-
gery is the treatment of choice. The conjunctival lesions can be removed under topical
anaesthesia. If needed short term general anaesthesia may be administered. The growth is
grasped with flat, blunt forceps and pulled away from the conjunctiva or a ligature may be tied
round the pedicle. The pedicle is cut with sharp scissors. Even without ligature the bleeding
can be stopped by firm pressure. Small growth can be treated with cryo. Surprisingly there is
no recurrence in conjunctiva once the growth has been removed surgically even if the child
lives in the same environment.
Management of rhinosporidiosis of sac is less satisfactory. It is essentially complete
removal by surgery. Unless meticulous care is taken to remove every bit of tissue involved
recurrence is the rule.
While removing the sac the nasal growth should also be taken care of simultaneously.
The growth of the sac can also be removed by nasal endoscopic surgery.
In scleral involvement the conjunctival growth is removed by usual method. For scleral
staphyloma best results are obtained by applying cryo all round the staphyloma. The scleral
bulge is indented with scleral explant of suitable size. Intraocular pressure of the eye is kept
low either by paracentesis or IV mannitol and maintained low by oral diamox and betablocker
drops.
Tumours of conjunctiva
Tumours of conjunctiva in children are rare. They are generally - Hamartomas,
choriostomas, haemangiomas and xeroderma pigmentosa.
Hamartomas that have congenital involvement are : Neuro fibromatosis (von Reckling
Hausens disease and Enccphalo trigeminal angiomatosis (Sturge Weber Syndrome).
Conjunctival involvement in neuro fibromatosis is less common than in Sturge Weber
syndrome. Conjunctiva is never involved alone, it is always associated with lid involvement
and many have associated systemic involvement.
Choriostomas. These are fairly common congenital benign tumours. There are two
types of choriostoma : 1. Dermoid, 2. Dermolipoma.
Haemangiomas of conjunctiva. These benign tumours are not true tumours. They
may develop alone in the conjunctiva or may be seen with haemangioma of lids. (See page
61-62)
Cysts of the conjunctiva
Various types of conjunctival cysts are : traumatic, retention, lymphatic or devel-
opmental. They can be parasitic in nature i.e. cysticercosis, echynoccosis, coenurosis.
It is estimated that non traumatic cysts are seen in 43% of eyes mostly in fornices. They
vary from pin head size to large enough to fill the fornix. Common causes of cysts in conjunc-
tiva are : trauma, congenital anomaly, parasitic infection and adhesion between two layers of
conjunctiva.
Cysts can be grouped into two broad groups i.e. traumatic and non-traumatic.
Traumatic cysts are mostly epithelial implantation cyst due to trapping of epithelium
under the mucosa. They are seen following wounds of conjunctiva, squint surgery, retained
conjunctival foreign body.
Non-traumatic cysts are : Dermoids, parasitic cyst, retention cysts and lymphatic
cysts.
Retention cysts
These generally develop in glands of Krause following chronic inflammation or trauma.
The ducts of the glands are obstructed resulting in formation of cysts. They are generally seen
in the upper part of the conjunctiva.
Symptoms depends on size and position of the cyst. The child may not be aware of
small cysts. They may be discovered during routine examination of the eye. Parents may be-
come aware of large cysts. There may be multiple translucent cysts. A large cyst may cause
astigmatism or may cause a diffuse swelling in the lid.
Management. Small cysts do not require any treatment. Large cysts are removed for
cosmetic purpose and to reduce astigmatism. Aim should be to remove the cyst without rup-
ture.
Parasitic conjunctivitis
Parasitic infestation of conjunctiva is far less common than bacterial or viral infection.
Parasites range from protozoa to helminth. Rarely arthropods may invade the conjunc-
tiva. Parasitic infection of conjunctiva is generally seen in tropical and sub-tropical regions.
Some of the parasitic conjunctivitis are seen only in endemic areas (river blindness). Some
of parasitic conjunctivitis are very rare and may not be seen in children.
Common parasitic conjunctivitis seen in children are : Cysticercosis, echinococcosis,
coneurosis, thelaziasis, onchocerciasis. (See Chapter Ocular Manifestation of Systemic Diseases)
Cysticercosis
56, 59
is a systemic parasitic infection. About forty percent of persons in-
fested by the parasite, have ocular involvement. The ocular involvement can be (1) less vision
threatening, extra ocular manifestation in the form of conjunctival or orbital cyst. (2) More
severe and blinding intraocular involvement.
Cysticercosis is caused by Cysticercus cellulose the larval stage of pork worm taenia
solium.
Humans harbour the parasite in two forms i.e. (1) Definite host form of adult tape-
worm or (2) Intermediate host, harbouring larval stage. Pig is the usual and natural interme-
diate host. Humans get infected by consuming under cooked pork that contains viable cysticersis
which mature in human intestine, and become adult worm. Other method of infection is con-
suming food and water contaminated with ova of the parasite. Latter mode of infection is more
common than in former. This is the mode of infection in strictly vegetarians and those persons
who shun pork due to religious reasons.
The embryo reaches the eye via ophthalmic artery. Left eye is more frequently
effected than right eye. Most of conjunctival lesions are located near the medial canthus.
However right eye is not immune. Bilateral and multiple unilateral involvement are rare.
Involvement of left eye is so frequent that all conjunctival cystic lesions in the left eye should
be considered to be cysticercosis unless proved otherwise.
57
The swellings are of variable size
generally fixed to episcleral tissue or the sclera. Initially the conjunctiva moves freely over the
translucent oval swelling. The cyst is painless. It may migrate along the extra ocular muscle
from behind to be visible on the conjunctiva and extruded
56, 60
. The cyst may occasionally
extrude through the conjunctiva. If the cyst ruptures it causes localised conjunctivitis.
Differential diagnosis consist of other parasitic cyst. Diagnosis is confirmed by
histopathology after removal. However increased eosinophils in differential count and presence
of ova in stool is highly suggestive.
Management consists of 1. Prophylaxis, 2. Surgical removal of the cyst.
Prophylaxis consists of proper cleanliness and food hygiene. Health authorities should
see that infected pigs are not slaughtered for consumption. Pork should be well cooked, raw
vegetables should be avoided least they are contaminated. Children should be dewormed regu-
larly either by Mebendazol 100 mg BD for three days or albendazol 400 mg single dose at least
once a year. Role of antihelminth as cure of conjunctival cyst is not confirmed.
Surgical treatment consists of complete removal of cyst without rupture. Rupture
causes severe conjunctivitis. Following successful removal, there is no recurrence.
Other parts of the eye that may be invaded by cysticercosis are lids, orbit, retina, vitre-
ous, and anterior chamber.
Intra ocular involvement results when the embryo reaches the interior of the eye via
posterior ciliary artery. The embryo develops in to an expanding cyst in the choroid that lifts
the retina to produce exudative retinal detachment. Perforation of the retina by cysticareus
results a free floating cyst in the vitreous.
The cyst on rupture produces larval endophthalmitis both large intra vitreal cyst and
endophthalmitis cause white reflex in pupillary area. Death of the larvae also causes
endophthalmitis severe enough to cause loss of eye.
Echinococcosis
62
Echinococcosis is systemic parasitic infection caused by larva of T. Echinococci a
helminth found in intestine of dogs. Man and other animals can get infected from affected
dogs. Human is an intermediate host who gets infected following ingestion of contaminated
food. No part of the body is immune to echinoccosis however, liver and lungs are most frequent
sites.
Ocular involvement consist ofCyst in sub-retinal space, vitreous and anterior
chamber. Conjunctival cyst formation is less common than seen in cysticercosis. Orbital
cysts cause proptosis, chemosis of conjunctiva and lid. Cyst when present in the conjunctiva
may be attached to one of the recti.
Management consists of prevention of infection in children. This consists of regular
de-worming of the pet and the child. Avoiding green raw vegetables. Definitive treatment is
removal of the cyst surgically without rupture.
Coenurosis
This is systemic and ocular involvement of an intermediate host by larval stage of dog
tapeworm multiceps. Humans act rarely as intermediate host which are generally sheep
and goats. Children get infected directly by ova of the parasite from soil, vegetable or fur of
dogs deposited in the conjunctival sac and develop cyst similar to cysticercosis. Adults get
infected by ingestion of contaminated food.
All the parts of the eye can be infected. In children subconjunctival cyst is most common
presentation. In adults it can involve sub retinal space, vitreous or orbit. There is no known
medical treatment, surgical removal is best option.
Thelaziasis
63, 64
Thelaziasis is a nematode infection of ocular tissue mostly in animals. There are about
ten species of thelazia that have been reported to infect conjunctiva. Common being thelazia
callipaeda and thelazia californiensis. Involvement of human eye is rare. In humans mostly
outer eye is involved. Parts involved are conjunctiva, lid and lacrimal passage. The nema-
tode finds its way to conjunctiva through a arthropod most probably a fly that lays eggs in the
conjunctiva which hatches there to complete its life cycle and produces conjunctivitis, hyperemia
and chemosis of conjunctiva. Unlike other nematodes thelazia does not cause cyst in the ocular
tissue. Live worm can be found in the conjunctival sac.
Intra ocular involvement have been reported. Intraocular penetration may result
through an abraded cornea or sclera. Live worm have been removed from anterior chamber.
63
Management of conjunctival involvement is manual removal of worm from conjuncti-
val sac after it has been immobilised by local anaesthetic agent. Worm from AC should be
removed surgically. Associated uveitis is treated by usual local cycloplegic and steroid. When
surgical removal is not possible, Diethyl carbamazine 50 mg three times for 14 days may prove
effective.
Ocular myiasis
Ocular myiasis or larval conjunctivitis is caused by deposition of eggs of some of the
flies. Three species of flies have been identified to cause myiasis.
61
Even common housefly can
cause ocular myiasis. The flies are attracted to the conjunctival and nasal discharge of the
filthy child. The flies lay eggs in the conjunctiva where the eggs hatch as larvae (maggots). It
is the larvae that are the cause of conjunctival inflammation. The maggot infestation is seen
mostly in emaciated children. There are two types of conjunctival infection by larvae :
1. Larval conjunctivitis. There is redness, itching, burning and lacrimation. On ex-
amination small elongated white larvae are seen crawling in the conjunctival sac.
There may be associated marginal keratitis.
2. Destructive myiasis. The larvae penetrate deep under the conjunctiva and skin
into the orbit. Occasionally the bones may be eaten away and meninges exposed.
Unless the larvae are eradicated promptly they may cause death.
Management consists of maintaining proper cleanliness in emaciated child. Use of mos-
quito net to prevent fly from depositing eggs on the conjunctiva. Attempt should be made to
remove the larvae manually as soon as detected.
REFERENCE8
1. Duke Elder S. : General consideration in conjunctival diseases in system of ophthal-
mology. Vol. VIII, Part 1. p-4 Edited by Duke Elder S., Henry Kimptom, London, 1965.
2. Nema H.V. : Development of ocular adnexa in Anatomy of the eye and its adnexa.
Second edition. p-138-139, Jay Pee Brothers, New Delhi 1991.
3. Berger B.B. : Surgical anatomy of the limbus in Principle and practice of ophthal-
mology. Vol. I, p-532-534. First Indian edition Edited by Peyman G.A., Sonders D.R.,
Goldberg M.F., Jay Pee Brothers, New Delhi, 1987.
4. Basak S.K. : The limbus in Essentials of ophthalmology. Second edition p-5-6. Cur-
rent Books International, Calcutta, 1999.
5. Ilene K. Gipson : Anatomy of the conjunctiva and cornea in The cornea. Third edition.
p-13-17 Edited by Smolin G. and Thoft R.A. Lippincot, Williams and Willkins, Philadel-
phia 1994.
6. Khamar B., Mayuri Khamar, Trivedi N. and Usha Vayas : Disease of conjunctiva in
Modern Ophthalmology. Vol. I, second edition. p-38-42 Edited by Dutta L.C., Jay Pee
Brother, New Delhi 2000.
7. Wilson F.M. : Congenital anomalies of cornea and conjunctiva in The Cornea. Edition
third. p-547-548. Edited by Smolin G. and Thoft R.A., Lippincot Williams and Wilkinsons,
Philadelphia 1994.
8. Laibon P.R. Owaring : Neonatal corneal opacities in Pediatric Ophthalmology. Vol.
I, Edition 2. p-480-483. Edited by Harley R.D., WB Saunders Company, Philadelphia
1983.
9. Chandler J.W. : Diphtheria in Current ocular therapy. Fifth edition p-22-24. Edited
by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company Philadelphia 2000.
10. Burd Eileen M. : Bacterial keratitis and conjunctivitis in The Cornea. Third edition
p-119. Edited by Samolin G. and Thoft R.A., Lippincot Williams and Wilkins, Philadel-
phia 1994.
11. Frindly D.S. : Ophthalmia neonatorum. Pediatric clinic of North America. 30:1033-1042.
1983.
12. OHara M. : Ophthalmia neonatorum. Pediatric clinic of North America. 40:715-725.
1993.
13. Khamar B., Usha H. Vyas, Trivedi N. and Mayuri Khamar : Ophthalmia neonatorum in
Modern Ophthalmology. First edition, p-19-21. Edited by Dutta L.C., Jay Pee Brothers,
New Delhi 1994.
14. Isada C.M., Meisler D.M. : Gonococcal ocular disease in Current ocular therapy. Fifth
edition p-28-31, Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company,
Philadelphia 2000.
15. American academy of pediatrics committee on drugs : Prophylaxis and Treatment of
neonatal gonococcal infection. Paediatrics 65, 1047-1048, 1980.
16. Laga M., Naamara W., Brunham R.C. : Single dose therapy of gonococcal. Ophthalmia
neonatorum. New Eng. Jr. M. 315 : 1382-1385. 1986.
17. Wand M. : Acine bactor : In current ocular therapy. Fourth edition p-3-4. Edited by
Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Philadelphia 1995.
18. Deborah Pavon-Langston. Foulks G.N. : Cornea and external diseases in Manual of
ocular diagnosis and therapy. Third edition, p-71, Little Brown.
19. Whitcher J.P. Chlamydia : Keratitis and conjunctivitis in The Cornea. Third edition.
p-282-292. Edited by Smolin G. and Thoft R.A. Lippincot, Williams and Wilkins, Phila-
delphia 1994.
20. Munoz B., West S. Trachoma : The forgotten cause of blindness. Epidemiol. Rev. 19:205-
217, 1997.
21. Kathryn I. Taylor and Taylor H.R. : Trachoma in Current ocular therapy. Fifth edi-
tion, p-76-78. Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Phila-
delphia 2000.
22. Viswalingam N.D., Darougas S. : Trachoma in Current ocular therapy. Fourth edi-
tion. p-61-63. Edited by Fraunfelder F.T., Roy F.H., WB Saunders Company, Philadel-
phia 1995.
23. Duke Elder S. Trachoma S. : System of Ophthalmology. Vol. VIII Part I, p-281-282,
Henry Kimpton, London 1965.
23A. McCallan A.F. : The epidemiology of trachoma. Br. Jr. Oph. 15:369, 1931.
24. Thylefors B., Dawson C.R., Jones B.R., West S.K., Tylor HR : A simple system for assess-
ment of trachoma and its complications. Bull WHO 65, 477-483, 1983.
25. World Health Organisation : Future approach to trachoma control. A report of global
scientific meeting. Geneva WHO/PBL 96.56.
26. WHO : Primary care level management of trachoma. Geneva. WHO/93.33.
27. Victoria Fransis : Achieving community support for trachoma control. Community Eye
Health. 7:28-30, 1994.
28. Courtright P. : Trachoma control : Community health. 7:18-20, 1994.
29. Courtright P., Sheppard, J., Lane S., Sadek A. et al. : Latrine ownership as a protective
factor in inflammatory trachoma in Egypt. B.J.O.75:322-325, 1991.
30. West S.K., Munoz B. et al. : Impact of face washing on trachoma in Kongwa, Tanzania.
Lancet 345:155-158, 1995.
31. Denise Mabey and Mabey D. : New hope for trachoma control. Community eye health.
7:17-18, 1994.
32. Friedlacnder M.H. : Ocular allergy. International Oph. Clinic. 28 : 261, 1988.
33. Donshik P.C. : Allergic conjunctivitis. International Oph. Clinic. 28, 294-301, 1988.
34. Friedlaender M.H., Okumoto M., Kelly J. : Diagnosis of allergic conjunctivitis. Arch
Oph. 102-1190-1195, 1984.
34A. Held K.S. : Itchy eye in Decision making in ophthalmology. First Indian edition
p-18-19, Edited by Hunven WAJ and Zivaan J.T., Harcourt Brace and Company Asia
Pvt. Ltd. 1988.
35. Douglas S.H. : Vernal kerato conjunctivitis in Current ocular therapy. Fifth edition,
p-341-343. Edited by Fraunfelder F.T. and Roy H.F., WB Saunders Company, Philadel-
phia 2000.
36. Colby K., Dohlman C. : Vernal kerato conjunctivitis. Int. Oph Clinic. 36:15-20, 1996.
37. Hennawi M.M. : Vernal kerato conjunctivitis in current ocular therapy. Fourth edi-
tion p-476-477. Edited by Fraunfelder F.T. Hamptom Roy F. WB Saunders Company
Philadelphia 1995.
38. Christiansen Sandra : Evaluation and treatment of the allergic patient. Int. Oph. clinic.
28:290-292, 1988.
39. Buckley R. : Vernal kerato conjunctivitis. Int. Oph. Clinic. 28:303-307, 1988.
40. Sharma A., Gupta R., Ram J. and Gupta A. : Topical ketrolac 0.5% solution for treat-
ment of vernal kerato conjunctivitis. Ind. Jr.Ph. 45 : 177-180, 1997.
41. Allen Smith M.R. : Giant paipllary conjunctivitis in Contact lens wearer. AJO 83:697-
708, 1977.
42. Srinivasan B.D. : Giant papillary conjunctivitis with ocular prostheses. Arch Oph. 97 :
892-895, 1979.
43. Allen Smith M.R., Ross R.M. : Giant papillary conjunctivitis. Int. Oph. Clinic. Vol. 28,
309-316, 1988.
44. Deborah Pavan Langston and Foulks G.N. : Phlyctenular kerato conjunctivitis in Manual
of ocular diagnosis and therapy. Third edition, p-105, Little Brown, 85.
45. Duke Elder S. : Phlyctenular kerato conjunctivitis in System of Ophthalmology. Vol.
VIII. Part one, p-461-471, Henry Kimpton, London 1965.
46. Tabbara K.F. : Phlyctenulosis in Current ocular therapy. Fifth edition. p-373-374.
Edited by Fraunfelder F.T. and Hampton Roy F., WB Saunders Company, Philadelphia
2000.
47. Wagoner M.D., Bajarat A.M., Allan Smith M.R. : Phlyctenulosis in Current ocular
therapy. Fourth edition. p-509-572, Edited by Fraunfelder F.T. and Hampton Roy F.,
WB Saunders Company, Philadelphia 1995.
48. Phlyctenular kerato conjunctivitis in Clinical ophthalmology. Vol. IV 8:1-6. Harper
and Row, Philadelphia 1986.
49. Felberg N.T., Michelson J.B. : Stevens-Johson Syndrome in Pediatric ophthalmol-
ogy. Vol. 2, second edition p-1178. Edited by Harley R.D., WB Saunders Company, Phila-
delphia 1983.
50. Dutta L.C. : Erythema multiformis major in Ophthalmology principle and prac-
tice. First edition. p-35, Current Books International, Calcutta, 1995.
51. Fraunfelder F.T. and Fraunfelder F.W. : Erythem multiformis major in Current ocu-
lar therapy. Fifth edition. p-145-146. Edited by Fraunfelder F.T. and Hamptom Roy F.
W.B., Saunders Company, Phildelphia 2000.
52. Mukherjee PK : Rhinosporidiosis in Current ocular therapy. Fifth edition. p-66-67.
Edited by Fraunfelder F.T. and Hampton Roy F., W.B. Saunders Company, Philadel-
phia 2000.
53. Mukherjee P.K., Shukla I.M., Deshpande M., Praveena Kher : Rhinosporidiosis of the
lacrimal sac. Ind. Jr. Op. 301 : 513-514, 1982.
54. Shukla IM, Mukherjee P.K., Sushma Verma : Primary rhinosporidiosis of the eye. Int.
Congress of Ophthalmology. ACTA XXVI Vol. 2, 864 : 1982.
55. Lamba P.A., Shukla K.N., Ganapathy M. : Rhinosporidiosis granuloma of conjunctiva
with scleral ectasia. Br. J. Oph. 54. 565-568, 1970.
56. Mukherjee P.K., Agrawal S. : Subconjunctival twin cysticercosis. Ind. Jr. Oph. 23 : 28-
29, 1975.
57. Malik S.R.K. Gupta A.K., Chaudhari S.K. : AmJr. Oph. 66. p-1168, 1968
59. Topilov H.W. : Cysticercosis in current ocular therapy. Fifth edition. p-92-94. Edited
by Fraunfelder F.H. and Hampton Roy F., W.B. Saunders Company, Philadelphia 2000.
60. Raina U.K., Taneja S. Lamba P.A., Bansal R.A. : Spontaneous extrusion of extra ocular
Cysticercus cyst. Am Jr. Oph 124 : 438-441, 1996.
61. Harley R.D. : Systemic protozoan and metazoan diseases in Pediatric ophthalmology.
Vol. II, Second edition. p-931-937, W.B. Saunders and Company, Philadelphia 1983.
62. Lerners S.F. Echinococcsis : In Current ocular therapy. Fifth edition. p-95-96. Edited by
Fraunfelder F.T. and Hamptom Roy F., W.B. Saunders Company, Philadelphia 2000.
63. Mukherjee P.K., Sushma Verma, Agrawal S. : Intraocular thelaziaA case report. Ind.
Jr. Op. 25 : 41-42, 1978.
64. Mukherjee PK : Thelaziasis in Current ocular therapy. Fifth edition. p-100-101 Edited
by Fraunfelder F.T. and Hampton Roy F., W.B. Saunders Company, Philadelphia 2000.
159
CHAPTER 7
Disorders of Cornea in Children
Anatomy of cornea
1, 2, 3, 4
. Cornea is the anterior most part of the outer coat of the
eyeball. It is transparent. Its main functions are optical and protective. Cornea forms roughly
one third of the outer coat. It sits over the anterior scleral foramen like shining crystal
clear watch glass with convexity anteriorly. As cornea is more curved i.e. 7.8 mm than sclera
(12 mm) it seems to bulge from the sclera resulting into a step like sulcus at the corneo
scleral junction. The bulge is 2.7 mm from the plane of the limbus. Cornea occupies anterior
1.3 sq.cm
5
of the eyeball. Though on casual look cornea seems circular, in fact it is not so.
Corneal diameter is not equal in horizontal and vertical meridian. Horizontal diameter is
longer i.e. 11.6 mm than vertical diameter i.e. 10.6 mm. Corneal diameter more than 12.7 mm
is called megalocornea while smaller than 10 mm is called microcornea.
This difference of one milliter makes the cornea horizontally oval. The cause of this
difference is due to constant pressure of the lid over the cornea making the cornea more
curved vertically resulting it more myopic vertically. Corneal curvature is not equal on
both the surfaces. Had the curvature been equal on both surfaces, the corneal thickness would
have been uniform throughout its length and width. Posterior corneal surface is more curved
than the anterior surface. This makes cornea thinnest at the centre and thicker at the
periphery. Corneal thickness is 0.52 mm in the centre, it increases to 0.70 mm in the periph-
ery. Central corneal thinness makes cornea more prone for perforation than periphery. The
central 4.0 mm that is called optical zone and is almost spherical. The corneal surface
gradually flattens on the periphery in adults.
In new born cornea is flatter than in adult and its curvature is more in the periphery.
Mean corneal diameter at birth is 10 mm, it increases rapidly to reach almost adult size by the
end of the first year. The cornea of a new born looks larger because of narrow interpalpebral
fissure. Cornea of a new born is slightly hazy that clears within few days.
Corneal layers. The cornea comprises of following distinct anatomical layers
6,7
:
1. Epithelium 2. Bowmans membrane
3. Stroma 4. Descemets membrane
5. Endothelium
1. The epithelium. The corneal epithelium is continuous with conjunctival epithe-
lium, making it easy for infection to travel from conjunctiva to cornea. The anterior epithelial
surface has microvilli that anchor the tear film to the corneal surface. The epithelium is
relatively impermeable to water soluble substances. The epithelium acts as effective barrier to
many micro-organism. Thus most of the micro-organism can not invade the cornea so long as
the epithelium is intact. The organisms that can bypass the epithelial barrier are gonococcus,
meningococcus and C. diptheria. The corneal epithelium is most mitotically active, thus
healing of the epithelium is very fast. The corneal epithelium can be rubbed or pealed off with
ease.
2. The Bowmans membrane is not a true membrane. It is formed by condensation of
anterior layer of stroma. It is one millimetre shorter than rest of the cornea in all directions.
The shortness of the Bowmans membrane is because its development is completed before full
development of rest of the cornea.
Bowmans membrane is easily destroyed and does not degenerate.
3. The stroma forms 90% bulk of the cornea and is continuous with the sclera.
4. The Descemets membrane is the basal membrane of corneal endothelium. Its thick-
ness increases with age. It is tough and can withstand considerable amount of raised intraocular
pressure and offers resistance to infection.
5. Corneal endothelium is single layered structure that is metabolically very active.
It is continuous with endothelium of the iris. The endothelium maintains deturgescence of
the cornea, keeping it transparent and bright. Damage to endothelium leads to edema of the
cornea. The damage can be brought about by trauma, inflamation, toxin and dystrophy.
Whole of the cornea has high metabolism for which it requires constant supply of oxy-
gen from the atmosphere that is dissolved in tear film, when the lids are open. During sleep,
the oxygen permeates through the vessels of tarsal conjunctiva. The other source of nutrition
to the avascular cornea is from limbal blood vessels which are present only in the peripheral 1
mm all round. The aqueous supplies nutrition to the endothelium.
The nerve supply of the cornea is by trigeminal nerve via ciliary nerve. The nerve fibers
enter the stroma from sclera, and conjunctiva. The cornea is very sensitive to pain, all sensa-
tion i.e. touch temperature and pressure are converted to pain sensation.
Physiology of The Cornea
11, 12, 13, 14
The two functions of the cornea are1. Optical, and 2. Protective.
1. Optical. For light to travel uninterrupted with out scatter, the cornea must have
(a) An optically regular surface and (b) A transparent medium.
Both these are met with due to a smooth epithelial surface, constant supply of nutrient,
relative dehydration. The cornea transmit cent percent of white light i.e. 380 nm to 760 nm.
Nutrition of Cornea
11, 12, 14
Cornea is highly metabolic tissues. It requires constant supply of oxygen and energy.
The oxygen is derived (1) mainly from the atmospheric air dissolved in tear film.(2) from the
perilimbal blood vessels (3) from the tarsal conjunctival vessels when the lids are closed.
Other nutrients are supplied by the aqueous from behind. The energy to the
cornea is supplied by metabolism of glucose in the form of adenosine triphosphate (A.T.P.).
The epithelium can metabolise glucose both aerobically and anaerobically. Most of the
metabolism takes place in the epithelium and endothelium. There are three metabolic pathways
DISORDERS OF CORNEA IN CHILDREN 161
involved in metabolism of cornea. They are 1. Glycolysis 65% (2) Kerb-cycle (3) Hexose
monophosphate shunt.
Corneal transparency depends upon following factors :
1. Anatomical
2. Deturgscence of cornea
3. Intraocular pressure
4. Uniform refractive index of various layers.
The anatomical factors are :
Absence of vessels in the centre of the cornea. There are no pigments in the cor-
nea, the epithelium is multi-layered but the cells are arranged in a regular fashion. The epi-
thelium is ten times thicker than endothelium yet metabolic requirement of endothelium is
more than epithelium. The single layered endothelium is arranged in a regular fashion so are
the stromal collagen fibrils. The epithelial cells which are exposed to atmosphere for long time
remain non keratanised in normal eye. The tear film obliterates whatever irregularity the
epithelial surfaces may have in normal eye to give a better optical surface.
Deturgscence of cornea. The normal cornea should remain in a state of relative de-
hydration. Accumulation of fluid causes the stroma to lose its transparency. The state of rela-
tive dehydration is done by active transference of fluid from stroma towards epithelium and by
way of an endothelial pump system from stroma. Loss of endothelial cells is result of embibement
of fluid in stroma and epithelial edema. Intraocular Pressure - Acute rise of intraocular
pressure causes derangement of fluid transport across the stroma and causes stromal edema
and loss of transparency.
Refractive index. The cornea is most important refractive surface of the eye. The
anterior surface has more important role as refractive surface than posterior surface. The
refractive index of cornea is 1.38.
The cornea protects the eye from most of organism and cuts off ultra violet rays as well.
It helps to maintain the structural integrity of the globe.
Development of cornea
6, 7, 8, 9, 10
. Cornea develops from two sources, surface ectoderm
and mesoderm. The former gives rise to corneal epithelium while rest of cornea is mesoder-
mal in origin. As the lens vescicle separates from the surface ectoderm a space develops. This
space is invaded by mesoderm that gives rise to all corneal layers except the epithelium.
Endothelium cells are first to be differentiated at 12 mm (5 weeks) stage. The Descemets
membrane is visible at 12 weeks. The Bowmans membrane is recognisable at 100 mm
(4 months). This is the stage when corneo scleral junction is also well defined.
Congenital anomalies of the cornea. Congenital anomalies of the cornea may be
localised to the cornea only or associated with other anomalies of the globe. They may be
unilateral or bilateral, need not be symmetrical in bilateral cases.
Common congenital anomalies of cornea are :
Microcornea
Megalocornea
Cornea plana
Keratoglobus
Keratoconus
26,27,28,29
Anterior embryotoxon
Sclero cornea
Corneal dystrophies
Microcornea
5, 16, 17
. Term microcornea is used to denote a condition where the corneal
diameter is less than 10 mm in otherwise normal eye. In these cases whole of the anterior
segment is small, hence the term anterior microphthalmia is also used to denote the same
condition. The recti are inserted more anteriorly than normal eyes. Corneal curvature is flat,
resulting into hypermetropia. Other types of errors of refraction are also possible secondary to
variation in curvature of cornea and shape of the lens in relation to the cornea. The inter
palpebral fissure is narrow. All microphthalmos eyes have small cornea but all eyes with mi-
cro cornea need not be microphthalmic. Sometimes it is difficult to differentiate between pure
micro-cornea and microphthalmos especially if there are other associated anomalies of the
globe like coloboma, microphakia, mesodermal tissue in angle of anterior chamber. In about
one fifth of cases there may be glaucoma due to anomalies of the angle. It should not be con-
fused with another congenital anomaly of the globe i.e. nanophtalmos where the eye is small
in all meridian but is normal in function.
Megalocornea. This condition is rarer than microcornea and microphthalmos, condi-
tion is bilateral, non progressive where anterior segment of the eye is larger than normal. The
corneal diameter varies from 13 mm to 16 mm. As the diameter is more there is increase in
circumference of the cornea. Corneal curvature is generally normal but may be increased with
myopic astigmatism, which is astigmatism with the rule. In absence of myopia vision is
unaffected. Anterior chamber is deep. The lens may be large. There may be both phacodonesis
as well as iridodonesis. Two common complications met with the condition are glaucoma and
cataract.
The condition must be differentiated from more serious, progressive vision threatening
buphthalmos and keratoglobus that is almost symptomless.
Megalocornea Buphthalmos
1. Bilateral, symmetrical 35% cases are unilateral. In bilateral cases involvement
may not be symmetrical in two eyes.
2. Non progressive Progressive
3. Exclusively seen in boys Boys to girlsratio is 5 : 3
4. Astigmatism with the rule Astigmatism against the rule.
5. Tension-Normal Tension raised
6. Cornea clear and bright Cornea cloudy due to rupture in Descemets membrane
7. No lacrimation Lacrimation prominent feature.
8. Angle-normal or may have minor Main pathology is gross malformed angle.
congenital anomalies.
9. No disc change Cupping prominent
10. Slight functional defect Gross functional defect.
Cornea plana. In this rare condition corneal curvature is reduced. In extreme cases
cornea may be flat generally associated with decreased scleral curvature. Sclera may encroach
over the cornea giving an impression of small cornea. Sometimes cornea itself is small. There
may be diffuse opacities in the cornea. Due to flattening of cornea there is impression of ptosis.
The eyes are generally hypermetropic. Anterior chamber is shallow. There may be associated
glaucoma. Causes of poor vision is curvature hypermetropia and corneal opacity.
Differential diagnosis consists of micro-cornea, microphthalmos, phthisis, atrophic bulbae
and nanophtalmos.
Keratoglobus. This is a relatively rare bilateral condition where corneal diameter is
normal but the corneal curvature is increased. The cornea becomes globular in contrast
to keratoconus where cornea is conical. In keratoglobus the cornea is thinner than normal
almost throughout. The sclera may also be thinned. The condition is stationary and symptomless
cases are detected on routine examination. Tension and angle remain normal throughout.
Differential diagnosis include all cases of enlarged cornea and corneal causes of deep anterior
chamber. Other members of the family may show keratoconus or irregular myopic astigma-
tism. It should also be differentiated from pellucid degeneration
14,15
of cornea that is gener-
ally seen after 30 years of age and has a toric bulge in the lower part of the cornea.
Keratoconus (Conical cornea)
18, 20, 21, 22, 23, 24, 25
. Keratoconus is a non inflammatory
ectasia of central cornea due to obscure causes. Some authors classify it as congenital disor-
der presuming that there is failure of mesoderm from periphery to centre, resulting into weak-
ness. It is worth noting that most of the pathological changes are seen in corneal structure that
are mesodermal in origin. Others have put keratoconus under degeneration
27
while most
put it under dystrophy. Keratoconus is suspected to be due to a kerato softening enzyme
14
.
Keratoconus has world-wide distribution, no race is immune. Proponents of theory of congeni-
tal anomaly feel that subtle changes are present years before the disorder becomes manifest.
The disease is bilateral and progressive. One eye is generally more involved than the
other. In rare instance the disease has been described as unilateral, in such cases the other eye
shows irregular myopic astigmatism without corneal thinning and ectasia. The disease is said
to be more common in girls. Generally symptoms start round ten years and progress for
eight to ten years, then either the progress comes to standstill or is very slow.
7
Increase in
keratoconus has been noted up to fourth and fifth decade. In few cases there is a steady in-
crease in keratoconus followed by a phase of non progression to be followed by a period of
progression again. Generally puberty heralds a year or two of rapid progression. Though
contact lenses are prescribed as treatment of keratoconus they may occasionally worsen it.
Repeated rubbing of eye has also been thought to be a contributory factor.
27
Keratoconus is
non hereditary in 95% of cases. In remaining it can either be autosomal dominant or
recessive. Keratoconus generally presents as isolated ocular disorder, however, some ocular
disorders are more frequently associated with keratoconus than others. These areSpring
catarrah, retinitis pigmentosa, ectopia lentis, anirida, anterior polar cataract, blue
sclera.
Systemic conditions that are frequently associated - atopic dermatitis, Down syndrome
Marfans syndrome, Lebers familial amaurosis, Ehler-Danols syndrome, neurofibromatosis,
mitral valve prolapse.
Clinical features consist of diminished distant vision which is progressive, glare,
photophobia, uniocular diplopia.
Signs. Vary according to severity of the case :
In early stages the corneal bulge is minimal and can not be detected on routine exami-
nation. Retinoscopy reveals compound myopic astigmatism with oblique axis. Sometimes astig-
matism is irregular which is confirmed by Placido disc, Klein keratoscope, and
keratometery. Computerised corneal topography is more informative than keratometery.
Corneal mapping through photo keratoscope detect very early and subtle changes.
Slit lamp biomicroscopy shows fine vertical lines at the level of deep stroma and
Descemets membrane, the lines disappear on pressure over the globe. Sometimes prominent
corneal nerves are also visible.
Late cases do not present any difficulty in diagnosis. In all sporadic cases of in-
creasing myopic astigmatism, keratoconus should always be kept in mind. On ob-
lique illumination, the cornea looks conical on profile with deep anterior chamber. Generally
the distance of corneal vertex to plane of limbus is 2.5 mm. In keratoconus this may increase to
4.5 mm to 5 mm.
In normal cornea, if a narrow beam of light is thrown at the corneoscleral junction from
lateral side, the opposite limbus lightsup in a circular fashion. In case of keratoconus, the light
reflex on the other side is conical.
28
If the patient is asked to look down, the apex of the cornea produces a notch in the lower
lid (Munsons sign). The apex of the cone is not opposite the centre of pupil, it is shifted down-
wards and medially. The apex may show some opacity.
On keratoscopy and keratometry the findings are exaggerated. Keratometer may fail
to measure the cone fully and may require additional plus lenses in the keratometer to meas-
ure the curvature completely.
Retinoscopy with plane mirror shows a central dark spot, corresponding to apex of the
cone, surrounded by a zone of pink retinoscopic glow, surrounded by a dark rim and then a
pink ring up to periphery. The intermediate dark rim represents the base of the cone where
there is near total internal reflection. The retinoscopy shows scissors movements and the
axis is generally oblique.
On slit lamp biomicroscopy with a bright narrow beam, the central part looks thin
while peripheral cornea has uniform, normal thickness. In 50% of eyes an incomplete yellow
green ring is visible at the base of the cone. This is due to deposition of hemosiderin superficial
to Bowmans membrane. In advanced cases there may be rupture in Descemets membrane,
leading to photophobia and lacrimation. Sometimes there may be rupture in Bowmans mem-
brane as well.
Hydrops of keratoconus denotes acute edema of the cornea due to rupture of
Descemets membrane resulting into imbibement of aqueous through endothelium. The whole
of the cornea becomes opaque, associated with pain, lacrimation and rapid fall of vision. The
stage of corneal hydrop lasts for 6-10 weeks and clears with healing of the rupture, however, it
leaves some degree of stromal scarring.
Though perforation in high degree of keratoconus has been reported, it is otherwise
very rare.
Management of keratoconus
29
1. Initially fairly good vision is attained by prescription of spectacles which require
frequent changes.
2. In moderate cases where spectacles do not give satisfactory visual improvement con-
tact lenses are prescribed initially, later on special piggy back contact lenses which are a
combination of hard and soft contact lenses are prescribed where a hard lens is fitted over soft
lens. However, sometimes contact lenses have been reported to worsen keratoconus.
3. Penetrating keratoplasty with a large donar button have very good prognosis, how-
ever, induced astigmatism may require correction by contact lens. Thermo keratoplasty,
lamelar keratoplasty and epikeratoplasty have not proved better than penetrating
keratoplasty, which is a definitive surgical treatment for keratoconus.
Acute hydrops may require lowering of tension by systemic acetazolamide and
betablocker. Local instillation of hypertonic solutions, patching or use of a bandage lens may
reduce discomfort. Local non-steroidal anti-inflammation drugs along with cycloplegic give
relief from pain and photophobia. Generally steroids are not required.
Common Corneal Disorders Seen in Children
Children are prone to suffer from various corneal disorders. Some are very mild and
may not reduce vision. Some may reduce vision marginally that pass off with treatment. Other
cause extensive visual damage that may be permanent even with best treatment. Many of the
corneal disorders are preventable.
Common corneal disorders seen in children are :
1. Congenital anomalies 2. Trauma
3. Infection 4. Inflammation
5. Nutritional 6. Degeneration
7. Dystrophies
Malignancies of cornea are not known, however a growth of conjunctiva at limbus
may invade the cornea. Generally such growths are seen only in adults except the limbal
dermoid that may be present at birth or may manifest later.
Some Pathological Signs of Corneal Disorder Seen in Children are
Disorders of cornea produce following changes in cornea :
1. MorphologyShape, size, curvature, surface, thickness
2. Loss of transparencyOpacity, edema, vascularisation, deposits.
3. Diminished sensation
4. Ulcerationits sequel and complication.
5. Xerosis
6. Degeneration
7. Dystrophies
Morphological changes in cornea :
Shape. Cornea looks circular on casual look, however, it is elliptical in shape when seen
from front. Its vertical diameter is less than horizontal diameter. The difference becomes obvi-
ous when measured by a corneal calliper. In fact the measurement in children should be taken
under general anaesthesia along with intraocular tension. In older children local anaesthesia
is sufficient. The cornea becomes horizontally oval in soft eyes i.e. phthisis, perforated globe
due to pressure of lids. In extreme degree of softness it becomes quadrilateral. Rarely the
cornea may be oval vertically or horizontally as a congenital anomaly. There may be an appar-
ent change in corneal shape with encroachment of dermoid or limbal papillae of spring catarrah.
Size of cornea :
1. Small cornea. A cornea smaller than 10 mm is called microcornea, while those
larger than 13 mm are known as megalocornea.
Small corneas are seen as congenital anomalies like microcornea, microphthalmos
and nanophtalmos. In micro-cornea it may be as small as 3-4 mm. Other causes of small
cornea are perforated globe, phthisis bulbae and atrophic bulbae.
2. Large cornea. Cornea larger than 13 mm is seen in buphthalmos, megalocornea
and keratoglobus. Myopic eyes have larger cornea than hypermetropic eyes.
3. Curvature. Curvature of corneaIn normal life, corneal curvature can be appreci-
ated by
1. Looking from temporal side when the patient looks at a distant object.
2. Looking at the depth of AC.
3. Asking the patient to look down.
Cornea with normal curvature does not produce any localised dent in the lower lid. In
case of increased curvature, the apex of the cornea causes a notch in the middle of the lower lid
when patient looks down (Munsons sign).
Accurate measurement of cornea is done bykeratoscope, keratometer and photo
keratoscope.
Corneal curvature is increased inKeratoconus, buphthalmos and keratoglobus.
In case of partial corneal staphyloma, the curvature may be increased irregularly. In pel-
lucid degeneration of corneathere is a localised bulge in the lower part. It develops in
third decade.
Corneal surface. Normal cornea has a smooth, shinning surface that acts as a convex
mirror forming miniature, virtual erect image of the object in front of the cornea without
any distortion or haze. Irregularity of the corneal surface can be observed by window reflex
keratoscope, keratometer, computerised corneal topography.
Causes of irregular corneal surface areCorneal ulcer, corneal scar, pterygium,
keratoconus, deposits on cornea i.e. foreign bodies, band keratopathy, corneal plaque. Other
causes areKeratectasia, adherent leucoma, corneal staphyloma, keratoplasty, growth at the
limbus (Dermoid), bulbus keratopathy.
Corneal brightness is diminished in :
1. loss of corneal epithelium i.e. corneal ulcer, trauma.
2. Corneal opacities
3. Deposits on the cornea
4. Epithelial edema, and stromal edema.
5. Vascularisation of cornea both superficial and deep
Changes in corneal thickness. Cornea is not uniform in its thickness. It is thicker at
the periphery and thinnest in the centre. Clinically thickness is measured by pachymeter.
Increase in corneal thickness is seen in :
1. Corneal edema.
2. It is more marked if endothelium is damaged than epithelium.
3. The other causes of increased corneal thickness is congenital hereditary endothelial
dystrophy.
Reduced thickness is seen in keratoconus, buphthalmos, Desmetocele corneal ulcer.
Changes in Corneal Transparency
Main function of cornea is optical. For this it has to remain in a state of utmost trans-
parency that is maintained by multiple factors.
2,5
They are :
1. Uniform and regular arrangement of epithelial cells.
2. Uniform size of stromal cells, their uniform arrangement and compactness.
3. Virtual absence of vascularisation.
4. Transparent corneal nerves and a
5. Constant state of deturgescence.
Deturgescence of cornea is a state of relative hydration of corneal tissue that is main-
tained by Na
+
K
+
cell pump of endothelium and epithelium. Though endothelium and epithe-
lium both contribute in maintaining corneal deturgescence, former has more pronounced ef-
fect than the later. Injury to endothelium by trauma, infection or toxin has more serious effect
than epithelial damage. The epithelium regenerates quickly thus damage to epithelium is
short, it causes localised swelling of epithelium and is transient causing diminished vision for
short time. Integrity of epithelial cells depend on (1) Tear film and (2) Available oxygen.
Derangement of any of the above factor alone or in combination can cause loss of corneal
transparency that may be short lived or permanent.
Commonest cause of loss of transparency is replacement of transparent keratocytes
by irregularly arranged opaque fibrocyte, due to trauma, infection, inflammation or infiltra-
tion. Other causes are superficial and deep vascularisation, incarceration of uvea, raised
intraocular tension, degeneration, dystrophies, deposits on the cornea, epithelial
down growth, growth of conjunctiva or conjunctival tumour spreading over cornea.
Loss of transparency of cornea is called corneal opacity that can be unilateral or bilat-
eral. Shape, size, number and depth varies according to causative factors and duration. Some
of the opacities are permanent, other may disappear with treatment or spontaneously. Some
of the opacities may increase in size, number or depth. Central corneal opacities cause more
visual loss. Peripheral opacities with clear pupillary area generally have only cosmetic effect.
Corneal opacities in the centre of cornea not only block the light rays from reaching the pupil
but also cause scattering of light and irregular astigmatism. Rarely corneal opacities can be
congenital.
Causes of Corneal Opacities in Children
Corneal opacities in children can be caused by any of the following separately or in
combinationCongenital, infection, inflammation, allergy, trauma, malnutrition, de-
generation and dystrophies.
Congenital corneal opacities :
Cornea of new born is mildly hazy. The haze gradually disappears in next two to three
days. If cloudiness persists following causes should be excluded :
1. Intrauterine infection
2. Birth trauma
3. Infantile glaucoma, mucopolysccharidosis and mucolipidosis.
The congenital causes of corneal opacity includessclerocornea.
Corneal opacities in children have far reaching consequences not only regarding vision
but also mental development. The child may remain permanently visually challenged. Thus
corneal opacities are one of the most common but neglected cause of amblyopia and squint
when unilateral and nystagmus when bilateral.
Causes of bilateral corneal opacities in children (on the basis of age of onset) :
1. New born Congenital
2. Neonate Ill managed ophthalmia neonatorum
3. First year Xerophthalmia, congenital glaucoma
4. Two to five years Xerophthalmia, trauma, bacterial corneal ulcer, con-
genital glaucoma, interstitial keratitis
5. Five year and above Trauma, xerophthalmia, bacterial corneal ulcer,
keratoconus, phlyctenular keratitis, spring
catarrah, corneal dystrophies, band keratopathy,
interstitial keratitis, limbal dermoid.
Corneal Edema
To maintain optical clarity, cornea must remain in a state of relative hydration. Accu-
mulation of fluid in cornea causes it to swell up. Corneal edema may be localised in epithe-
lium, stroma or may involve both. The corneal epithelium and endothelium regulate pas-
sage of fluid and ions. Deranged fluid transport across the cornea causes corneal edema.
Epithelial edema causes more visual loss due to irregular astigmatism that results following
accumulation of fluid in the basal cells of the epithelium and forming a bullae. Epithelial
edema presents as diminished vision specially on awakening. In well formed bullae blinking
causes foreign body sensation that becomes more marked if the bullae ruptures. There may be
associated pain, redness and photophobia. Ruptured bullae may result into anterior chamber
reaction and formation of sterile hypopyon or may lead to bacterial corneal ulcer. Stromal
edema causes less loss of vision and less symptoms. It results in haziness of stroma and thick-
ening of cornea.
Common causes of corneal edema are :
1. Trauma to endotheliumaccidental or surgical during IOL implant, placement of
glaucoma valve, penetrating keratoplasty.
2. Trauma to epitheliumIll fitting contact lens, chemical burn.
3. Aphakia
(a) Due to vitreous in AC
(b) Toxicity of irrigating fluid
(c) Trauma during manipulation of IOL
(d) Silicone oil in AC.
4. Acute rise of tension, persistent raised tension, rupture of Descemets membrane.
5. Keratoconusacute hydrops
6. Herpctic disciform keratitis
7. Fuchs dystrophy
8. Chronic uveitis
9. Keratocele (Descemetocele)
10. Iris incarceration
11. Corneal staphyloma (Anterior staphyloma).
Corneal neovascularisation
15, 30, 31
Out of all refractive media i.e. cornea, aqueous, lens and vitreous, cornea has maximum
refractive power. For a sharp retinal image all factors remaining within normal limits cornea
must remain transparent. Avascularity of cornea plays a very important role in keeping cor-
nea bright and transparent. Cornea is almost avascular except 1 mm of periphery adjacent to
the limbus and vessels do not grow into corneal substance so long cornea is not diseased.
Growth of vessels in cornea is blocked by compactness of corneal tissue and anti vascular
chemical factors present in the cornea. Once the compactness of cornea is lost, vessels start
growing into the substance of cornea both in epithelium and stroma. The anti vascular factor
may be overwhelmed by a vasoformative factor in newly formed vessels. Vessels once formed
in cornea never disappear, they may get obliterated leaving tell a tale sign of ghost vessels
which are more prominent in deep vascularisation. Presence of corneal vascularisation is al-
ways pathological. Factors that encourage corneal vascularisation aretrauma, infection, in-
flammation, hypoxia, hypo vitaminosis B, allergy and degeneration.
Corneal vascularisation can be put into three groups depending upon the layer at
which they develop i.e.
1. Superficialmostly epithelial 2. Interstitialmostly stromal
3. Retro corneal
Superficial vascularisation of cornea is commonest form of vascularisation. This is due
to extension of conjunctival vessels into the cornea in front of Bowmans membrane. According
to its extent in the cornea it is divided into
1. Micro pannus, 2. Gross pannus.
Pannus is a fibro vascular proliferation that extend up to the cornea, it is essentially
a defensive process against infection, toxin, trauma etc. It can be sectorial or may involve
whole of corneal periphery. It is always preceded by infiltration of corneal epithelium by
polymorphonuclear and mononuclear cells. Cellular infiltration is followed by development of
tufts of new vessels. It may destroy the Bowmans membrane causing corneal haze. There are
three clinical stages of superficial corneal vascularisation i.e. (1) Progressive stage, (2) Sta-
tionary stage, (3) Regressive stage.
In the progressive stage, blood vessels grow towards the centre of the cornea. They
may be parallel to each other. A zone of cellular infiltration extends beyond the tip of the
vessel terminal.
In regressive stage the zone of infiltration is between the tip of the blood vessels and
the limbus, other things remaining the same.
Superficial vascularisation can be seen asmicropannus or as gross pannus.
Micropannus
30
extend only one to two millimeter beyond the normal limbal arcade. It
is most commonly seen is contact lens wearer. Milder form of chlamydia kerato conjunc-
tivitis, vernal catarrh, chronic blepharitis.
Gross pannus extend more than 2 mm beyond normal limbal vessels. It is seen in
trachoma, phlyctenulosis, rosaceakeratitis, atopickerato conjunctivitis, contact lens
wearer, severe blepharoconjunctivitis, herpetickeratitis, riboflavin deficiency,
corneal graft rejection. In gross pannus sometimes there may be a large and prominent
vessel which may be very prominent or there may be a tuft of localised vessels as seen in
fascicular ulcer.
Deep vascularisation of cornea
In this condition anterior ciliary blood vessels invade the cornea at the level of
stroma. They have very characteristic feature :
1. They are generally straight and parallel to each other.
2. They do not anastomose with each other.
3. Branch at an acute angle from the main branch giving a bottlebrush appearance.
4. They are generally sectorial in distribution.
5. The vessel seem to disappear at limbus.
6. The vessels grow in the particular plane and remain in the same plane.
7. They are always associated with deep keratitis, and uveitis.
8. When severe, they give a pink hue to the cornea known as Hutchinsons salmon
patch.
9. Rarely there may be associated superficial vascularisation at the level of epithelium
giving an epaulet appearance to the peripheral cornea.
10. Deep vascularisation gives a ground glass appearance to the cornea.
11. When the inflammation is under control the vessels obliterate leaving a track of
ghost vessels that last for rest of the life.
Causes of deep vascularisation areCongenital syphilis, tuberculosis, leprosy, herpes
simplex, chemical burn.
Retrocorneal vascularisation. This type of vascularisation is mostly seen when blood
vessels from uvea develop on the endothelium of the cornea either due to chronic uveitis,
diabetic neovascularisation or thrombosis of retinal vein. They may also grow from conjunc-
tiva followingpenetrating injury at the limbus, penetrating keratoplasty, and conjunctival
epithelial down growth.
Inter corneal vascularisation. This happens rarely when limbal vessels grow under
the partial thickness wound of the cornea either accidentally or following large lamellar graft.
Rupture and folds in Descemets membrane
32, 33, 34
:
Descemets membrane is likely to rupture :
1. If the eyeball is compressed suddenly like in birth injury or concussion injury to the
globe.
2. The cornea stretches either due to thinning as seen in keratoconus and
buphthalmos.
Rupture of Descemets membrane is more common in buphthalmos than in keratoconus.
In buphthalmos there is not only thinning of the cornea but also an enlargement of cornea that
stretches the Descemets membrane. The tears differ in shape, size, number and position.
With ophthalmoscope they stand out as dark double lined areas in red background. The area
between the two lines also give a red glow. They may be linear, curvilinear or whirl like.
Their presence in buphthalmos is known as Haabs line which are curvilinear. Opaque lines
that represent healed breaks in Descemets membrane. With rupture of Descemets mem-
brane there is a sudden influx of fluid into the stroma leading to stromal edema, lacrimation
and photophobia. Stromal edema may subside with treatment but may persist to cause perma-
nent scarring of cornea that is so common in untreated or poorly managed buphthalmos. Rup-
tures due to elevated IOP are either horizontal or parallel to limbus. Rupture in Descemets
membrane due to trauma are generally central and vertical. Some of the dystrophies may
look similar to Haabs striae.
Folds in Descemets membrane are more common than tears. They can be caused by
trauma, both blunt as well as incised wound. Other causes are prolonged uveitis with hypotony.
Keratic precipitate (see diseases of uvea)
Loss of corneal sensation. Cornea is very richly supplied by sensory nerve endings of
trigeminal nerve. There is only one sensation in cornea i.e. pain. An intact corneal sensation
is essential for health of cornea specially the epithelium. It is of diagnostic significance not
only for local corneal diseases but also neuro ophthalmic diagnosis. Corneal sensation may be
marginally reduced or may be totally absent.
Causes of diminished corneal sensation are :
1. Recent use of local anaesthetic agent,
2. Use of contact lens.
3. Herpes simplex, herpes zoster, leprosy.
4. Corneal edema, corneal scar, corneal vascularisation.
5. Buphthalmos
6. Repair of corneal wound, keratoplasty
7. Vitamin A deficiency
8. Deposits on cornea i.e. band keratopathy, tattoo
9. Cerbropontine angle tumour
10. Trigeminal neuritis
Xerosis of cornea (see xerophthalmia)
Corneal Ulcer in Children
Inflammation of cornea secondary to micro-organisms is called keratitis. Keratitis with
loss of epithelium is called corneal ulcer. A large group of cases which are not caused by
microbes are grouped under a loose term - Keratopathy. Keratitis in children basically does
not differ from those seen in adults, however, complications are more in children. Most of
keratitis in spite of good management leave corneal opacity that may produce amblyopia and
squint. Keratitis in infancy may be responsible for nystagmus. Hence keratitis requires prompt
diagnosis and efficient management.
Keratitis in general has been described under various heading. However most accepted
classification by Duke Elder 1565 on the basis of etiology still remains most widely accepted
classification. Though it too has its short comings. The classification is as follows -
Keratitis
35
:
1. Superficial keratitis 2. Parenchymatous keratitis
3. Deep keratitis
1. Superficial keratitis has been divided into
(a) Diffuse and punctate keratitis
(b) Corneal ulcer
(c) Degenerative superficial keratitis.
(a) Diffuse superficial keratitis can be
(i) Acute superficial keratitis as seen in acute bacterial and viral conjunctivitis
that may result in corneal ulcer.
(ii) Chronic superficial keratitis is seen mostly in trachoma, leprosy, mollouscum
contagiosum, phlycten. Chronic superficial keratitis is characterised by
pannus formation.
Superficial keratitis can be diffuse or punctate.
Punctate keratitis stain irregularly and poorly with fluorescein but better with rose
bengal. A lesion may stain partly with fluorescein and partly with rose bengal depending
upon level of the lesion. The unstained lesions have a whitish appearance. The size, shape and
distribution of superficial punctate keratitis vary depending upon severity and duration of the
lesion. They may be localised in epithelium, may be sub-epithelial, there may be a combi-
nation of both forms in the same eye. They may be primary corneal or secondary to con-
junctival or lid lesions. Sometimes they are seen with various type of skin lesions.
Common conditions associated with punctate keratitis are :
Staphylococcal blepharoconjunctivitis, trachoma, inclusion conjunctivitis, herpes simplex
and zoster, molluscum, vernal conjunctivitis, ultra violet light, trichiasis, juvenile rheumatoid
arthritis.
Management of superficial keratitis. There is no specific treatment. The probable
primary causes should be treated with appropriate antibiotic. If there is photophobia and an-
terior chamber reaction, short acting cycloplegic like home atropine 2%, pad and bandage at
bed time gives early relief. However, the lids should be examined for misdirected lash, blepharitis
or foreign body in tarsal plate especially in unilateral cases.
Corneal Ulcer
35, 36, 37
Corneal ulcer is an inflammatory process (infective or sterile) where there is an epithelial
defect with some loss of stroma. However some of the processes like epithelial keratitis may be
limited to epithelium. Infectious corneal ulcers are generally associated with stromal infiltra-
tion. The epithelial defect stains with fluorescein. Infective corneal ulcer is generally associ-
ated with anterior chamber reaction.
General consideration :
1. No age, sex or race is immune to corneal ulcer, however, they are more common in
second to fourth decade.
2. Corneal ulcers are generally uniocular unless associated with systemic diseases like
Vitamin A deficiency, ophthalmia neonatorum, leprosy, blast injury, chemical burn.
3. A breach in the epithelium is a pre-requisite for all infective corneal ulcers except-
gonococcus, meningococcus, coryne bacterium diptheriae. Epithelial break
is generally due to trauma, may be a mild abrasion or the traumatic factor may be
persistent like ill fitted contact lens, trichiasis, entropion, concretion, foreign body
on the cornea or tarsal plate.
4. Intact protective mechanism saves cornea from trauma. Failure in protective mecha-
nism anatomical or neurological predispose corneal ulceration -
(a) Coloboma of the lid, ectropion of lid, entropion of lid, proptosis.
(b) Lagophthalmos
(c) Loss of sensation
(d) Abnormal tear film.
5. Vitamin A deficiency disease (VAD)
6. Though the conjunctiva is never sterile except first few hours after birth, organisms
present are generally nonpathogenic but opportunistic organisms will have a upper
hand if the resistance of the cornea is compromised due to-
(a) Local causes. Indiscriminate use of antibiotics and steroids, repeated use of
local anaesthetic agent, ill fitting contact lens, loss of sensation, raised intraocular
tension, corneal edema.
(b) Systemic. Prolonged use of steroid, resistant organisms AIDS.
7. Cornea subjected to constant inoculation by micro-organism i.e. chronic dacryocystitis,
blepharoconjunctivitis.
8. Central corneal ulcers are generally due to exogenous infection. Peripheral ulcers
are generally non infective.
Pathogenesis of corneal ulcer
35
Evolution of infective corneal ulcer has following stages :
1. Invasion by micro-organism. Organism generally get access to cornea following
trauma, gonococci meningococci and diphtheria however may invade intact epithelium.
In herpes simplex and zoster, organism reach cornea via sensory nerve ending. Organisms
also reach cornea by direct continuity of conjunctiva, in case of acute bacterial conjunctivitis.
2. Progressive infiltration. Once micro-organism has reached traumatised epithe-
lium, 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 epi-
thelium and formation of ulcer that stains with fluorescein. If proper anti-microbial drugs
are used at this stage, further progress can be averted or minimised resulting into clear cor-
nea.
3. Active ulceration. The infected epithelium is necrosed and sequesteredoff with
stromal infiltration. The ulcer deepens with infiltration and edema of the stroma. At this stage
anterior uveitis develops due to toxins liberated by the organism which percolate to endothelium
and spreads to uvea resulting into pain, photophobia, watering, circumciliary conges-
tion disproportionate to size of the ulcer. If the anterior chamber reaction is severe, hypopyon
may develop. The ulcer may spread all around towards the periphery and or it may deepen to
reach the Descemets membrane which is tougher than other structures of cornea hence is not
destroyed with ease. The Descemets membrane bulges through the defective stroma even
with normal IOP and forms a keratocele or Descemetocele. If intraocular pressure rises
suddenly or remains raised for considerable time, the Descemets membrane gives way and a
perforation of cornea results with resultant loss of AC, softening of eye and plugging of
perforation by iris. If the perforation is large, intraocular contents including lens may be
expelled through the perforation.
Commonest site for perforation is centre of the cornea which is thinnest. If the perfora-
tion is small but is not closed by iris, a corneal fistula results.
4. Regression. Healing is initiated by natural defence mechanism of host cornea. This
is enhanced by treatment. Healing starts with laying down of fibrocytes in place of normal
keratocytes. The fibrocytes are opaque and do not confirm with normal corneal architecture,
there may be associated superficial vascularisation. Over all result is formation of a corneal
opacity depending upon the depth of the opacity it can be - nebular, macular or leucomatous.
If iris is incarcerated, there will be a leucoma adherent. In case of a large perforation incar-
ceration of total iris in the margin of the ulcer causes an anterior staphyloma which bulges
even with normal intraocular tension.
Failure of regression. Small and superficial ulcers may heal in due course with
resultant corneal opacity without treatment. Deep and large ulcers heal with treatment. If
progression of ulcers is not checked, the ulcer is bound to perforate, resulting intoleucoma
adherent, anteriorpolarcataract, complicated cataract, cornealfistula, anterior
staphyloma, endophthalmitis, pano-phthalmitis, phthisis.
Clinical features of corneal ulcer
35, 37, 38
. For a corneal ulcer to develop breach in
epithelium is a prerequisite except in gonococcus, meningococcus and diphtheria. Breach in
epithelium is brought about by trauma. Trauma is generally mechanical, severity of trauma
is variable, it may be so trivial that the child may not be able to recollect. In infants trauma
by own nails is very common. Depending upon type and virulence of offending organism
ulcer may develop within hours to days. Fungal ulcers take weeks to manifest.
Pneunococcus, pseudomonas have short incubation period. Sometimes there may be more
than one offending organism responsible for corneal ulcer.
Symptoms :
1. Lacrimation and foreign body sensation. These are the two early symptoms. For-
eign body sensation becomes worse and is converted into dull ache, pneumococcal,
pseudomonas, Koch-Weeks bacilli produce severe pain.
2. Redness of eye is due to circumciliary congestion.
3. Most of the bacterial corneal ulcers are associated with mucopurulent conjuncti-
vitis.
4. Vision - Visual status depends upon position of ulcer itself, and area of surrounding
infiltration. Central ulcers produce more visual loss while peripheral ulcers may not
hamper vision at all or visual loss is minimal. With progression of ulcer, vision dimin-
ishes fast.
5. Blepharo-spasm is a prominent feature in children, this is associated with
photophobia due to irritation or trigeminal nerve. Spasm of the ciliary body is
the foremost cause of pain. Other cause of pain is stimulation of corneal nerve end-
ings. Corneal nerve endings become numb with progress of ulcer.
6. In case of hypothesia of cornea that is found in herpes simplex and neurotropic ulcers
patient may not complain of pain.
7. Late increase of pain is due to rise of intraocular pressure. Sudden relief of
pain is an ominous symptom, it means perforation.
Signs. The ulcer starts as an area of greyish infiltration that is lustureless, there is
distortion of windows reflex over the ulcer. The surrounding area is edematous and infil-
trated by inflammatory cells. Generally there is single central ulcer, peripheral ulcers may be
multiple. The ulcer stains bright green with fluorescein. The edge of the ulcer stains brighter
than floor. The surrounding edematous and infiltration takes a yellowish green stain.
Biomicroscopy shows anterior chamber reaction with flare and cells. In severe ulcer
hypopyon may become evident.
Circumcorneal congestion is prominent sign of anterior uveal inflammation that
follows deepening of the ulcer and liberation of toxin.
Corneal sensation is diminished in viral ulcers, large sloughing ulcers and neurotropic
ulcers.
Staining of corneal lesions. Corneal lesions are stained by vital stains. Two com-
monly used stains are Fluorescein and Rose Bengal. Purpose of staining is to demonstrate
presence of a stainable lesion i.e. abrasion, desquamation, ulceration, epithelial edema, mu-
cous, dead and damaged cells.
Fluorescein remains extracellular, it stains tear film also but not the dry spot. It
stains epithelial defect, large lesions are visible with ordinary flash light, small and faint le-
sions are best seen under cobalt blue filter with magnification. It is used either as one per-
cent autoclaved solution or as 1% stain impregnated, pre sterilised, single use, disposable
strip. Fluorescein does not cause irritation of cornea or conjunctiva. It is water soluble, hence
washable.
Other purpose is to know the extent of ulcer, its progress or healing. Fluorescein stain-
ing should be performed after testing corneal sensation and before instilling local anaesthetic
agent. While using fluorescein its sterility should be guaranteed. Fluorescein is notorious to
harbour pseudomonas. Other uses of fluorescein are contact lens fitting, applanation
tonometry, demonstration of leaking wound, tear film study. Extra corneal examination uses
are fluorescein angiography, evaluation of patency nasolacrimal duct i.e. Jones I and II test.
Seidel test to demonstrate leaking surgical wound 20% fluorescein solution has been used
with some success in treatment of pediculosis and phthiriasis of lid margin.
Rose Bengal. Chemically rose bengal is similar to fluorescein and is water soluble. Dry
crystals of both the chemicals are brown in colour. Rose bengal is used to stain mucous, dead
and damaged cells. It is used along with fluorescein in many superficial corneal lesion. Rose
bengal stains peripheral viral laden cells and fluorescein will stain floor of the ulcer in herpatic
ulcer. Rose bengal stains filament of epithelium in filamentary keratopathy while base of the
filament stains with fluorescein.
It does not share infectious tendency of fluorescein. Only drawback with rose bengal is
its sting. Rose bengal causes severe stinging sensation of the conjunctiva. The patient should
be informed about this phenomenon which is otherwise harmless. Prior instillation of local
anaesthetic abolishes sting of rose bengal, however, xylocaine can cause epithelial changes
that may stain with rose bengal. Proparacine hydrochloride 1% aqueous drops is the only
recommended local anaesthetic that does not interfere with rose bengal stain.
Documentation of corneal lesions. Lesions of cornea are documented by drawing
with international colour code. Like colour coded drawing of fundus for retinal lesions. Corneal
lesions are drawn in two views. One is frontal and other is vertical section of anterior segment
as seen on slit lamp. For finer details an optical section of cornea may be drawn.
Management of infective corneal ulcer :
Management consists of :
(i) Confirmation of diagnosis (ii) Specific treatment
(iii) Ancillary treatment.
Confirmation of diagnosisHistory, clinical examination and stain give definite clue to
causative factor.
Infective ulcers can be caused bybacteria, fungi and viruses. Bacterial ulcer may
be caused by single organism or there may be mixed infection. It is very common for viral
and fungal ulcers to be contaminated by bacteria. Reverse is also possible but rare. Each group
of organism have specific characteristics.
Characteristics of bacterial corneal ulcer :
They are :
1. Generally central, even when cornea is infected by bacteria on the periphery due to
some ill understood phenomenon ulcer starts in the centre.
2. Most of the bacterial ulcers are associated with mucopurulent conjunctivitis.
3. They develop in short time following injury and spread fast.
4. Bacterial corneal ulcers are likely to develop hypopyon more frequently.
Most of the bacteria are capable of producing hypoypon. However pneumococci,
pseudomonas, streptococcus and staphylococci are more common causative factors
than others.
5. Bacterial ulcers are generally associated with severe pain especially in pneumococcal
ulcers.
6. They promptly stain with fluorescein.
7. Sensation over clear cornea is generally normal.
8. Scrapping from fresh and untreated ulcer give positive smear, that can be cultured
and sensitivity of the organism to antibiotic can be determined.
Characteristics of fungal ulcer
37, 40, 41
. Like bacterial ulcers, fungal ulcers are also
central in nature, they are more indolent. In most of the cases there is history of trauma
by organic material. It takes seven to fifteen days for a fungal ulcer to develop following
injury. If an aggressive ulcer develops following corneal injury within twenty four hours, the
chances are that it is a bacterial ulcer. History of prolonged and indiscriminate use of local and
systemic steroid is second most predisposition. Exposure keratitis, anaesthetic cornea,
edematous cornea are also likely to develop fungal ulcer.
Fungal corneal ulcer has a few typical features
42
:
1. It is central, indolent, yellowishwhite ulcer with dull, dry surface that has zone of
infiltration all round, there are smaller satellite lesions away from the main ulcer.
2. There is pronounced anterior chamber reaction that results in formation of
endothelial plaque and thick almost organised hypopyon.
3. Scrapping from the ulcer show fungus in 10% KOH solution, and stains with Giemsa
stain, P.A.S. or Gomori methamine silver stain.
4. Fungi are cultured at room temperature on blood agar and Sabouraud medium.
5. Corneal biopsy may be needed some time.
Characteristics of viral ulcer. Almost all viruses that invade conjunctiva also in-
volve cornea and produce various types of superficial keratitis only herpes group of viruses i.e.
simplex type I, type II and zoster virus are of clinical significance as far as corneal ulceration
is concerned. Primary herpes simplex is disease of infants and children. Recurrent ulcers
are seen in adults. Most characteristic sign of herpetic ulcer is diminish corneal sensation.
The ulcer is unilateral, generally central and dendritic in shape without much redness
and pain. Initially the ulcer stains poorly with fluorescein and better with rose bengal. In
later stage with disquamation of epithelium fluorescein stain becomes prominent. Herpetic
ulcer does not develop hypopyon unless secondarily infected by bacteria.
The other symptoms of viral keratitis are foreign body sensation, lacrimation,
photophobia, diminished vision, sometimes diminished corneal sensation. Initially there is
hardly any circumciliary congestion. The ulcers are small and numerous. They stain with
fluorescein. Smaller ulcers may require cobalt blue filter to become visible many viral ulcers
are bilateral.
Characteristics of sterile corneal ulcer. These ulcers are generally peripheral,
mostly bilateral, relatively quiet, without circumcorneal congestion. Corneal infiltrates
and epithelial defect are minimal or absent. There is no anterior chamber reaction, no
hypopyon, corneal sensation is normal. Vision is diminished if the lesion is central. Other
symptoms are mild redness and pain. Etiological factors are generally systemic.
Morphological distribution of corneal ulcer. Corneal ulcers can be central or pe-
ripheral, both can be superficial, deep, infective or non infective.
Central corneal ulcers
42, 43
. Central corneal ulcers are mostly infective, may be caused
by bacteria, fungi and viruses. As they develop in front of pupil, diminished vision is
early and marked that becomes worse with extension of ulcer. Even successful treatment
leaves residual central opacity that causes diminished vision. Diminished vision is not only
due to obstruction of light to pass through pupil but also due to accompanied irregular astig-
matism. Diminished vision following central corneal ulcer puts child at an added disadvantage
of intractable amblyopia and squint. Opacity developing in infancy may cause nystagmus.
Peripheral (marginal) corneal ulcers
35, 44
. Marginal corneal ulcers are as common
as central ulcers. They are less dramatic than central ulcers and have a more benign course,
hence draw less and late attention than central ulcers. However incidence of peripheral ulcer
is less in children than adults. Marginal ulcers in children do not perforate unless secondary
bacterial infection is superimposed. Causes of their being superficial and non perforating is
better nutrition on the periphery of cornea and enhanced thickness of corneal periphery.
Causes of peripheral corneal ulcers are :
1. Extension of conjunctival disease in the cornea i.e. trachoma, phlycten, rosacea,
staphylococcal blepharoconjunctivitis.
2. Allergicphlycten
3. Toxin
4. Metabolic
Marginal ulcers according to their position can be (1) sectorial, (2) circumferential - may
form a ring ulcer. Circumferential ulcers generally result due to coalescence of more than one
peripheral ulcer. Circumferential ulcers have tendency to develop heavy superficial
vascularisation.
Identification of specific causative organism is done by :
(a) Corneal scrapping and examination of stained slide.
(b) Culture of organism
(c) Sensitivity of organism to antibiotic.
Identification of other ocular diseases that may influence treatment of corneal
ulcer i.e. lagophthalmos, lid deformity, loss of sensation, chronic dacryocystitis, raised
intraocular tension.
Systemic diseases. Malnutrition, vitamin A deficiency, measles. Diabetes is generally
not a problem in children but should be kept in mind if it is associated with symptoms of
juvenile diabetes.
Specific treatment : 1. Anti microbial drugs.
Local antibiotics. Once it has been established that the ulcer is infective and its causa-
tive organism identified, specific anti microbial drops in suitable strength and frequency
will eliminate the organism and enhance healing. However it is not always practical to get
smear, culture and sensitivity done, result of sensitivity to antibiotic for bacterial infection
takes minimum 24 hours to 48 hours. Fungal culture takes as much as a fortnight to be in-
formative. Culture of virus is done only in few selected and advanced centres. Most practical
method is to decide an antibiotic according to clinical feature. Generally a long acting antibi-
otic that is effective against both gram positive and negative organisms is used as aqueous
drops. Frequency depends upon severity of the condition.
Commonly used antibiotic in infective corneal ulcer are Erythromycin 0.5%,
Chloramphenicol 03.%-0.5%, Gentamycin 0.3%, Tobramycin 0.3%, Ciprofloxicin 0.3%,
Framycetin 0.5%, Ofloxacin 0.3%, sparfloxacin 0.3%. Newer antibiotics are being added fre-
quently replacing older antibiotics.
These drugs are instilled as drops, one drop each hour in the lower fornix. More than
one drop at a time is not retained in the conjunctiva and flows out without additional thera-
peutic advantage.
Sometimes a combination of antibiotics that have synergistic effect is used. A common
combination are Neomycin 0.35% + Polymyxine B. 10000 u and Bacitracin 500u. Another popular
combination is combination of neomycin with chloramphenicol.
In adults and older children subconjunctival injection may be given. Whenever it is not
possible to administer subconjunctival injection, fortified antibiotic solutions are instilled
instead of ordinary drops.
Commonly used fortified solutions used for corneal ulcer are :
Gentamycin 14 mg/ml,
Cephazoline 33 mg/ml,
Tobracin 15 mg/ml
Ophthalmic ointment is used at bed time either with or without bandage.
Advantage of ophthalmic ointments are :
They act as lubricants, they have slow absorption, slow drainage from the conjunctival
sac. They need not be applied frequently. They prevent sticking of the lids.
Subconjunctival injection. Commonly used subconjunctival injections are :
Penicillin1 million unit/ml
Gentamycin20 mg-40 mg/ml
Cephazoline100 mg-125 mg /ml
Tobramycin20 mg-40 mg/ml
Subconjunctival injection of antibiotic gives a high but short lived peak of antibiotic
concentration at the site of injection. Hence they are either given along with local instillation
or at bed time with a pad and bandage.
Generally subconjunctival injections are given once a day for five to seven days. Sys-
temic antibiotics do not have any added advantage over local antibiotics. (For anti
fungal and anti-viral drugs used in corneal ulcer see under specific ulcers)
2. Removal of discharge. Bacterial and fungal corneal ulcers are generally associated
with mucopurulent or purulent discharge. Efforts should be made to remove the discharge
from the conjunctival sac and lid margin with sterile wet swab. Irrigation of the conjuncti-
val sac has no added advantage. On the contrary it washes away tear lysozyme which has
anti microbial property.
3. Cycloplegia. Cycloplegics should be used in all corneal ulcers with anterior chamber
reaction. Cycloplegics have no action on cornea, they are not anti-inflammatory, anal-
gesics or anti microbial. Main function of cycloplegic in corneal ulcer is to prevent and
treat associated anterior uveitis that is main cause of pain and photophobia. Cycloplegics
relive spasm of ciliary body, thus reduce pain indirectly. As all cycloplegics are strong mydriatic
also, they dilate the pupil and break posterior synechea.
As such pure mydriatics have no role in management of corneal ulcer.
Commonly used cycloplegics in corneal ulcer are :
(i) Atropine sulphate. Atropine is most potent cycloplegic and prompt mydriatic. Its
action last for more than ten days following single instillation, however, in inflamed
eye more than one instillation is required. Atropine should be used with caution
in children. One drop of one percent atropine contain more than therapeutic dose.
It is better to avoid atropine as drop in children. In no case should atropine be used
more than twice in twenty four hours. If necessary short acting cycloplegic may be
added to atropine to enhance effect of atropine. Prolonged use of atropine may cause
allergic dermatitis. While prescribing atropine to children the parents should be
appraised of the side effect of atropine i.e. flushed face, fever, and dryness of
mouth which are not serious and can be managed by systemic antipyretic and plenty
of fluids orally. Dilated pupil makes the child uncomfortable due to glare.
(ii) Home atropine hydrobromide 2%. It is less powerful cycloplegic than atropine.
Its action does not last more than 48 hours. Its side effects are less than atropine so
it can be used frequently. However in children who develop allergy to atro-
pine are also allergic to home atropine. It is generally used in cases of superfi-
cial small ulcers.
(iii) Cyclopentolate hydrochloride. This is used as 1% drop, has action faster than
home atropine but action lasts for twenty four hours to forty eight hours, can be used
frequently.
Tropicamide 1% is very short acting unless used repeatedly has hardly any benefi-
cial effect in corneal ulcer.
4. Pad and bandage. Pad and bandage prevent movement of lid over the ulcer this
stabilises the epithelial growth during healing. It does not abolish movement of eye, it only
reduces movement of lid that helps epithelisation of ulcer. However pad and bandage raise the
temperature of the conjunctiva which encourages proliferation of micro organism, hence, ap-
plication of pad is contra indicated in presence of copious discharge.
5. Bandage contact lenses are good substitute for usual pad and bandage.
6. Destruction of ulcer :
(i) Cauterisation of ulcer. If ulcer fails to respond to usual treatment within a week,
debridement of the ulcer is done under local anaesthesia and edges are cauter-
ised by pure carbolic with caution.
Other drugs used to cauterise the ulcer are 7% alcoholic solution of iodine,
trichlor acetic acid.
(ii) Physical methods of destruction of ulcers are Heat cautery, cryo and laser.
7. In case of non healing corneal ulcer, penetrating keratoplasty is indicated.
Management of hypopyon corneal ulcer. Hypopyon corneal ulcer denotes presence
of severe anterior uveitis due to corneal ulcer. All bacteria are capable of producing hypopyon.
Some organisms characteristically produce severe hypopyon, others cause minimal hypopyon.
Hypopyon is acculumation of sterile pus in anterior chamber. It contains mostly
polymorphonuclear leucocytes, occasional mononeuclear cells, macro-phages and
fibrin. The pus is due to out pouring of exudates from iris and ciliary body. In bacterial
corneal ulcer, the pus remains sterile so long as Descemets membrane is intact. The pus gets
infected following perforation of ulcer. It is a prominent feature in central corneal ulcers and
penetrating injury. The pus in bacterial corneal ulcer is worsened by absorption of toxin liberated
by causative organism.
Hypopyon in fungal ulcer is generally infected because fungus can penetrate intact
Descemets membrane. Bacteria produce hypopyon earlier than fungi.
Virus by themselves do not produce hypopyon. Presence of pus in viral keratitis denotes
presence of secondary bacterial infection.
Development of pus is preceded by severe anterior chamber reaction. The aqueous be-
comes plasmoid and fibrinous that entangles leucocytes. Presence of pus cells in the anterior
chamber gives it characteristic white colour, however the pus is tinged green in
pseudomonas infection, and is yellowish in fungus, it may be occasionally tinted with
blood. As pus is heavier than aqueous, hypopyon settles down at the most dependent part of
anterior chamber, it assumes a horizontal level at the upper part. Though hypopyon is thicker
than aqueous yet it freely moves unless it gets organised as is seen in fungal ulcer. Amount of
hypopyon is directly proportionate to virulence of organism. Some organisms produce early
and severe hypopyon. Organisms that produce severe hypopyon arepneumococci,
pseudomonas, streptococci pyogenes, gonococcus. Extent of hypopyon is measured by
its height from bottom of anterior chamber in millimeters or roughly as trace, mild, moder-
ate, half of AC or full of AC. Best position of the eye to look for early hypopyon is to make the
patient sit up for few minutes and allow hypopyon to settle down and ask the patient to look
down, light is thrown from the above in front of the iris. In recumbent position the pus spreads
all over the iris or may pass through the pupil, making it difficult to be visualised.
Hypopyon in fungal corneal ulcer takes more time to develop, it is associated with se-
vere anterior chamber reaction. The pus is generally thick, does not move freely with
change in position of the eye, is fluffy, yellowish in colour and infected with fungal ele-
ment. The ulcer has multiple satellite lesion and an endothelial plaque under the original
ulcer.
Management of hypopyon corneal ulcer is basically the same as any infective ulcer
with severe uveitis. Hypopyon corneal ulcer should be treated as an emergency. It is
better to hospitalise the patient and start vigorous antibiotic drop instillation. Subconjunctival
injection in children is difficult to administer. Hence frequent local instillation of suitable
fortified antibiotic is most important part of treatment. In bacterial ulcers hypopyon may
disappear with in twenty four hours only to appear after frequency of instillation is reduced
unless infection has been eradicated.
Bandage lenses and occuserts are good alternative when available and possible.
In children sub-conjunctival injection and cauterisation should be done under short
acting general anaesthesia or good sedation.
All cases of hypopyon should receive atropine sulphate 1% as an ointment. Atropinisation
should be done under supervision of trained person. Atropine drops are better avoided
because an illiterate attendant may instil atropine frequently in place of antibiotic without
anticipating serious complication.
Outline of treatment of hypopyonCorneal ulcer should be as follows :
1. Hospitalise the child.
2. Affective and safe atropinisation.
3. Frequent instillation of single or combination of two broad spectrum synergestic
antibiotic drops that will eradicate both gram positive and gram negative cocci and
bacilli.
4. Corneal scrapping :
(i) Examine part of scrapped material after staining with Grams stain, Giemsa
stain, 10% KOH to identify bacteria and fungus and find out if the characteris-
tics present are in favour of bacteria or fungi.
(ii) Inoculate part of the material to grow bacteria or fungi.
(iii) Identify organism and test for sensitivity to antibiotic.
5. Once best possible antibiotic has been identified, replace initial antibiotic with sensi-
tive antibiotic as fortified drops. If possible give sub-conjunctival injection of the
same antibiotic.
6. Cauterise under short acting general anaesthesia.
7. Keep intraocular pressure low by oral carbonic anhydrase, inhibitor and beta blockers.
8. Pad and bandage at night may help in early healing of ulcer.
9. Systemic antibiotics do not have any direct influence on hypopyon, they may be added
in consultation with pediatrician for systemic infection when present.
Non Healing Corneal Ulcer
Aim of treatment of corneal ulcer is to
1. Eliminate infection,
2. Encourage healing,
3. Restore transparency of cornea and
4. Reduce complication.
Bacterial ulcers have better prognosis than fungal ulcers. Viral ulcers especially herpes
simplex is notorious for recurrence. Generally a bacterial ulcer of moderate size takes ten to
twenty days to be free from organism and heal with proper treatment. However sometimes the
ulcer does not heal as expected. Causes of non healing corneal ulcers are :
1. Failure to identify the causative organism.
2. Failure to eliminate causative organism.
3. Incomplete management of predisposing factors.
4. Iatrogenic
1. Failure to identify causative organism. It is very common to miss fungus infec-
tion 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 devel-
oped resistance, use of anti-microbial in sub-clinical strength and frequency is
one of the common factors.
This may result in use of ordinary antibiotic drop instead of fortified drop or not
administering sub conjunctival injection when indicated. Hesitation to cauterise a
fulmination ulcer too has similar result
3. Incomplete management of predisposing factorsspecially presence of chronic
dacryocystitis, lid of deformity, loss of sensation, dry eye, vitamin A
deficiency, rise of intraocular tension, vascularisation of cornea, missed
foreign bodies in cornea, upper lid or upper tarsal plate are some of the factors
that prevent healing of corneal ulcer.
4. Iatrogenic causes of non healing corneal ulcers are -
(i) Indiscriminate use of steroid.
(ii) Introduction of exogenous infection by way of contaminated drops i.e. fluorescein
drops, local anaesthetic agent.
(iii) Over enthusiastic chemical cauterisation.
Management of non healing corneal ulcer is basically treatment of simple corneal
ulcer with suitable antibiotic, cycloplegic and correction of above mentioned factors. In case of
penicillinase producing organism i.e. staphylococci, methicillin should be used. Pseudomonas
corneal ulcers generally require two antibiotic drops one aminoglycocide and other cefazolin.
Common ancillary management consists of cauterisation of ulcer edge, sub-conjunctival anti-
biotic injection, bandage lens, local beta blockers or systemic acetazolamide, cauterisation of
superficial vascularisation. Under no condition should a subconjunctival injection or cauteri-
sation 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 rec-
ommendation.
Corneal ulcer threatening to perforate :
There are two groups of ulcers that perforate :
1. Very fast developing ulcer like pneumococcal, and pseudomonas ulcers and
keratomalacia.
2. Slow developing ulcers - Fungal ulcer, stromal involvement in HSV, Herpes simplex
keratitis.
Perforation of an ulcer is a catastrophe that must be avoided. Once an ulcer
perforates, the prognosis changes to worst. It may lead to endophthalmitis or even
panophthalmitis. If perforation seals, it leads to adherent leucoma or corneal staphyloma.
Management of impending perforation are :
1. Anticipation of perforation. A fast developing ulcer, especially in depth with large
hypopyon, severe anterior chamber reaction and pain is most likely to perforate.
Intensive instillation of two synergistic antibiotic with subconjunctival injection may
stop the progress.
2. Prevent
(i) A sudden rise of intraocular pressure by preventing cough, sneezing, vomiting,
and constipation. Protect the eye from direct trauma by metal or plastic shield
placed properly. Otherwise the edge of shield itself can traumatise cornea.
(ii) Fast build up of intraocular tension is managed by local beta blocker two times a
day or acetazolamide 15 mg/kg body weight in divided doses for short period.
Pilocarpine is contra indicated.
(iii) Paracentesis. Paracentesis is a guarded and planned perforation on the corneal
periphery. It should be done under general anaesthesia in children. It lowers
intraocular tension instantaneously. Advantage of paracentesis are :
1. Pus drains out through the wound.
2. Pus and aqueous so drained can be examined for microbes.
3. If necessary, intra cameral antibiotics can be injected.
4. Following paracentesis, there is out pouring of fresh aqueous that has heal-
ing 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 antibi-
otics 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 treat-
ment unless it involves the epithelium only. The size and depth of opacity varies with extent of
ulcer and promptness of treatment. Ulcers anterior to the Bowmans membrane give faintest
opacity unless they are vascularised. Full thickness extension with or without iris incarcera-
tion 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 glau-
coma 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 com-
monly known as corneal opacity. The opacity is generally white in colour. According to col-
our 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, infiltra-
tion and edema. Infiltration and edema generally pass off with healing, may take weeks to
months to disappear. Corneal opacities following destruction of Bowmans membrane are al-
ways permanent.
Nebular opacities in corneal ulcer develop due to infiltrating edema and cicatrisation
of epithelial defect. They are generally situated in front of the Bowmans membrane.
Small nebular opacities may be missed on oblique illumination but are best visualised on slit
lamp biomicroscopy. On retinoscopy they cause irregular reflex. Though they do not cause
much visual loss diminished vision due to nebulae are sufficient to cause amblyopia.
Macular opacities are denser than nebulae and visualised by ordinary flashlight. They
are due to destruction of Bowmans membrane and superficial stroma. They are permanent in
nature.
Leucomass are densest opacities that are visible in diffuse light and when large, the
parents may notice their presence. They are generally due to lesions extending from epithe-
lium to Descemets membrane. They may cover whole of the cornea, may have facets in them,
are often vascularised. They cause maximum loss of vision, amblyopia, and squint in children.
As corneal ulcer perforates following events take place :
1. Aqueous drains out causing softening of the eyeball, flattening of cornea, collapse of
AC and miosis.
2. The iris is drawn towards the perforation and gets attached to it.
3. The iris plugs the wound over which fibrosis occurs and
4. A leucomatous opacity develops.
This is called leucoma adherence.
Eye with leucoma adherence has following features :
1. A corneal opacity in which either the iris is adherent or there is an evidence that iris
was once incarcerated, which is betrayed by presence of iris pigment in the centre of
the opacity. Thus any corneal opacity with evidence of iris incarcerated is adherent
leucoma.
2. Key hole pattern of pupil with narrow end towards the opacity.
3. Absence of AC at the site of iris incarceration.
4. Tent like appearance of the iris with apex at the opacity.
Ectatic Changes in Cornea Following Perforation
1. Staphyloma. Perforation is very common catastrophic complication of corneal ulcer.
They are most common in large central, untreated or poorly managed ulcers that extend deeper
than Descemets membrane. Small corneal perforation is plugged by a knuckle of iris to form
an adherent leucoma. A large perforation is not immediately sealed. As has been discussed
earlier, a perforation leads to draining of aqueous, flattening of cornea, softening of eyeball,
miosis and shift of iris lens diaphragm forwards. This forwarded shifted iris blocks the
perforation and the constricted pupil is closed by exudate that acts as a scaffolding over
which fibrocytes from stroma and epithelium develop to form a white scar or pseudocornea
of uneven thickness which is very thin and plastered with iris on posterior surface at places
the whole of the scar gets ectetic and bulges forward. This ectatic cicatrix with iris
incarcerated is called corneal or anterior staphyloma. It can engulf whole of cornea and
called total anterior staphyloma or may be partial. The anterior staphyloma may be
vascularised, may have facets. There is always associated secondary glaucoma, which produces
loss of vision which is very common. A large staphyloma may bulge through inter palpebral
fissure. Generally the staphylomatous eyes are divergent and have nystagmus. Commonly
they are not painful, uncontrolled glaucoma or break down of scar make them painful. In
unfortunate children anterior staphyloma can be bilateral. Small pox used to be commonest
cause of large bilateral anterior staphyloma before small pox was eradicated. In under developed
countries, keratomalacia still causes bilateral anterior staphyloma. Blast injury especially
due to cracker burst is another cause of bilateral, total or partial staphyloma in children.
2. Descemetocele. Bulging of thin cornea is a common feature of poorly managed corneal
ulcer. If there is incarceration of iris in ectatic cornea, it results in corneal staphyloma that has
been discussed earlier. If there is no perforation, iris does not get incarcerated in the wound
and the Descemets membrane bulges through the floor of the ulcer. It is called Descemetocele
or keratocele. In the infiltrative stage of corneal ulcer, the ulcer can spread either towards
the periphery or may infiltrate deep. In unfortunate conditions both may happen simultane-
ously. Deeper infiltration is rapid through Bowmans membrane and stroma. The Descemets
membrane offers real resistance and can withstand the infiltrative processes for sometime. As
the ulcer reaches the Descemets membrane, the membrane bulges forward as a translucent
blister through the floor of ulcer under normal intraocular pressure. If intraocular pressure is
high, not only will the surrounding Descemets membrane bulge but also surrounding thin
cornea.
Corneal ectasia may follow thinning of cornea without Descemets membrane bulging.
In simple ectatic cicatrix of cornea, there is no incarceration of iris. The cicatrised ectasia is a
weak spot in cornea hence is liable to perforate either following trauma or gradual rise of
tension. Perforation of ectatic cornea is not sealed so perfectly as a simple small perforation
seals. It may seal and anterior chamber may reform only to be lost following opening up of the
wound resulting into a corneal fistula.
Corneal fistula
45
. 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.
Complication of corneal fistula are :
1. Endophthalmitis, panophthalmitis due to introduction of infection.
2. Phthisis due to persistent hypotony.
3. Epithelial down growth.
Summary of perforation of corneal ulcer :
1. Small perforation with mobile iris. Adherent leucoma.
2. Moderate perforation with miotic pupil and mobile iriscorneal staphyloma.
3. Large perforation not closed by iris - Extrusion of intraocular content, lens and
vitreous resulting in phthisis bulbae.
4. Small central perforation not plugged by iris - Corneal fistula.
5. Small central perforation sealed without incarceration of irisanterior po-
lar cataract.
Corneal opacity
45, 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 sin-
gle 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 glau-
coma.
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. Bow-
mans membrane and Descemets membrane once destroyed do not regain transpar-
ency.
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 obliter-
ated 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.
DISEASES OF CORNEA IN CHILDREN 189
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 dimin-
ished 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 incar-
cerated 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 eradi-
cated. 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 penetrat-
ing 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.
4. Painted contact lenses mask the corneal opacity.
5. Local instillation of ethyl morphine (Dionin), and iodides do not have any beneficial
effect even on faint corneal opacity.
Bilateral corneal opacities pose more problem than unilateral opacities because they
may virtually make a child grossly visually challenged.
Common causes of bilateral corneal opacities in children as per age of onset
are :
1. Congenital Intrauterine infection
Buphthalmos
Limbal dermoid
Dysgenesis of anterior chamber
Iridocorneal dysgenesis
2. Neonatal Ophthalmia neonatorum
(Bilateral central leucoma, anterior polar cataract, nystagmus)
IatrogenicFolk medicines
3. Infancy Bacterial corneal ulcer
Buphthalmos, Iatrogenic
4. Childhood Above causes, xerophthalmia cracker injury
Interstitial keratitis
Phlyctenular keratitis
5. Adolescence 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. Combi-
nations may be :
(i) Multiple bacterial infection of all possible combinations.
(ii) Bacterial infection over viral infection, reverse is rare.
(iii) Bacterial superimposition on fungal ulcer.
(iv) Bacterial ulcer may be primary.
(v) Corneal pathogen or opportunistic organism which remain dorment without ul-
cer 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 per-
forate, most of them are painful. They are generally due to acute or chronic bacterial conjunc-
tivitis 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 antibod-
ies 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 periph-
eral herpes simplex and pseudomonas ulcer should get special attention.
Important Bacterial Central Corneal Ulcers
1. Pneumococcal ulcer
47, 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.
Management
49, 50
. Scrapping from the active edge promptly grow Gram positive,
diplococci. Occasionally the organism can be seen in short chain. Generally there is a capsule
surrounding each pair. The organism should be cultured and its sensitivity to various antibiotics
graded. The pneumococci is known to become resistant to antibiotics specially penicillin.
Generally systemic antibiotics are not recommended unless patient has systemic involvement.
Systemic antibiotic should be administered in full dose in consultation with physician. All
cases of pneumococcal keratitis threatening to perforate should also get systemic antibiotic.
Usually available commercial ophthalmic drops are not sufficient for management of
suppurative keratitis. Fortified aqueous drops should be administered frequently. Frequency
may be as often as every five minutes for six times followed by every ten minutes for
another six times. This is followed by hourly instillation during day and two hourly at night.
Commonly used fortified drops are :
Penicillin G100,000 1u/cc
Bacitracin10,000 IU/cc
Cefazolin50 mg/c.c.
Other antibiotics used are erythromycin, vancomycin. Ciprofloxacin is less effective.
If topical antibiotic drops are not effective or there is a moderate hypopyon concurrent
sub-conjunctival injection of either 0.5 to 1.0 milli units of penicillin or 50mg to 100mg of
cefazolin BD may evert further extension.
As there is always associated severe uveitis, use of atropine is one of the most impor-
tant 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 keratitis
46, 48, 51
. Pseudomonas aeruginosa causes one of the most seri-
ous 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 infec-
tion are - instillation of contaminated fluorescein or anaesthetic drops in hospitals or by con-
taminated contact lenses specially extended wear. It has also been cultured from contact
lens solutions. Pseudomonas has a short incubation period. Otherwise it may follow mild
trauma.
Pseudomonas corneal ulcer starts as a small central area of infiltration following trivial
injury and spreads in all directions. Occasionally it may start in the periphery. The ulcer
spreads rapidly, is always associated with severe pain. Hypopyon develops fast and
increases with spread of ulcer. Hypopyon has greenish blue hue due to pigment produced by
the organism. Pseudomonas ulcer may spread so fast as to cause large central perforation
within twenty four hours. Ulcer is always associated with mucopurulent conjunctivitis
that may have satellite conjunctival abscess. Peripheral ulcers may spread into the sclera
as tunnel lesions. All fast spreading corneal ulcers in contact lens users should be considered
to be due to pseudomonas unless proved otherwise by culture.
Management of pseudomonas ulcer does not differ from any other central ulcer.
When pseudomonas is suspected but its presence has not been confirmed a combination of
fortified cepfazolin and tobramycin should be used as drops very frequently. Once
pseudomonas has been confirmed cefzolin may be discontinued and any of the following alone
or in combination with tobramycin is continued i.e. gentamycin, any of fluroquinols. In no
condition should steroid be used. Systemic acetazolamide or local betablockers lower the risk
of perforation.
Streptococcus pyogenes. Central corneal ulcer caused by streptococcus does not have
any particular characteristic except that the surrounding cornea is infiltrated and edematous.
It produces a large hypopyon. The organism is sensitive to many antibiotics which should be
prescribed as fortified drops to be instilled very frequently. Antibiotics commonly used are -
erythromycin, cefazolin, gentamycin, chloramphenicol, tetracyclin. However fortified penicillin
100,000 unit/cc be tried as first drug of choice. It can be given as subconjunctival injection also
in a dose of 0.5 to 1.0 million unit/cc. Patient should get usual dose of atropine with all
precautions.
Staphylococcus corneal ulcer. Staphylococcus cause both central as well as
peripheral ulcer. Latter is commonly seen in associated with staphylococcal
blepharoconjunctivitis. Incidence of staphylococcal central ulcer has increased due to
prolonged and indiscriminate use of steroid. The ulcer is indolent, is generally associated
with moderate hypopyon. The surrounding cornea is infiltrated. The ulcer is generally
superficial hence perforation is relatively less common. The organism is sensitive to many
drugs i.e. penicillin, erythromycin, chloramphenicol cefazolin, gentamycin.
Central Viral Corneal Ulcers
Almost all viral keratitis are central. Most important virus that produce central corneal
ulcer belong to herpes group of viruses i.e. :
Human herpes virus
Varicella zoster virus
Cyto megalo virus
Epstein Barr virus
All the above viruses are D.N.A. viruses, besides ocular involvement all of them have
systemic manifestation of various degrees which could be life threatening in children.
Herpes simplex keratitis
52, 53, 54, 61
. Human herpes virus or herpes simplex is most
important virus causing keratitis, incidence of which is gradually increasing. It is a self limiting
disease in immunocompetent cornea. Most of the time it is recurrent. Management of recurrent
disease is more difficult than primary disease. In spite of apparent cure of single incidence,
next attack can not be predicted or prevented, which may be delayed for one to two years.
Incidence of herpes simplex keratitis increases with age, no age or sex is immune to herpes
simplex infection. It is generally unilateral.
1. Herpes simplex infection can be passed as an intrauterine infection, resulting in
multiple congenital malformation including ocular.
2. A neonate may acquire the disease from infected birth canal.
On the basis of antigenicity, herpes simplex has been classified as type I and type II.
Each having various strains, some of the strains are more virulent than others.
Traditionally type I herpes simplex is said to involve the body above the waste and
type two is said to effect below waste (genital herpes). However, infection above waste by
type two and infection below waste by type one is also known. Both types infect skin,
mucousmembrane, mucocutaneous junction and cornea. Herpes keratitis is ocular coun-
terpart 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 ex-
posed 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 ma-
ternal 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 conjunc-
tivitis, keratitis, micro dendrile, dendrile, and mild kerato uveitis. Neonates have severe
iritis. Primary lesions are generally self limiting.
Recurrent herpes simplex infection of cornea is seen in persons who have been exposed
to primary infection. The lesions are mostly central but can be peripheral as well that are more
difficult to manage.
Following primary infection, the virus gains access to the central nervous system and
remains dormant in trigeminal or spinal ganglion only to be reactivated by some catalyst
(trigger mechanism) like stress, ultra violet ray, fever, menses, systemic infection,
general anaesthesia, food allergens etc., resulting into lesions of skin, lips, mouth and
followed by corneal lesion. However some of the corneal lesions are independent of trigger
mechanism.
In recurrent lesions active viruses travel down the nerves to infect the target organ that
develop classical lesions.
Corneal lesions can be : 1. Epithelial, and 2. Stromal.
1. Epithelial lesions are
54
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 com-
monly 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 sensa-
tion is dull which masks the pain sensation, preventing the patient from an early consulta-
tion. However only symptom may be lacrimation and foreign body sensation, some con-
junctival 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 for-
mation of dendritic ulcer or may co-exist with it to be transformed into typical dendritic ulcer.
Subepithelial opacities may develop under the original epithelial defect in the form of
ghost image. The shape of which is identical to original dendritic ulcer but larger. The ghost
image may last as long as one year. They may become worse by use of anti viral drugs
specially idoxuridine.
As ninety five percent of HSV keratitis are unilateral, all cases of unilateral watering of
short duration, without any definite history of trauma, or foreign body in the cornea or upper
tarsal conjunctiva should be stained and examined under cobalt blue light for ulcer. Staining
should be preceded by examination of corneal anaesthesia.
Other causes of formation of dendritic ulcers are :
Herpes zoster, corneal abrasion, Thygesons keratitis and contact lens.
Gepgraphic (amoeboid) ulcer. If dendritic ulcer does not heal it spreads in all direc-
tion to form a geographic or amaeboid ulcer that stains with fluorescein. Commonest cause
of geographic ulcer is fast growth of virus on the periphery due to compromised immunity of
cornea especially following indiscriminate use of local steroid or cytotoxic drugs. Vision is
hampered as the epithelial defect is large and covers the pupillary area.
Metaherpatic keratitis. These are non viral component of dendritic and geographic
ulcer due to problem of healing at the level of damaged basement membrane. They are caused
by corneal denervation, they are trophic, indolent in nature. These ulcers last for long time
with very little inflammatory reaction. The lesions heal with continuously worn therapeutic
contact lens, prolonged pad and bandage, use of lubricants.
Stromal involvement. Stromal involvement is commonest complication of recurrent
HSV keratitis. It is generally preceded by recurrent attack of dendritic or geographical ulcer.
It is commonest cause of diminished vision that is more pronounced than in epithelial keratitis
because healing of stromal keratitis is always associated with stromal scarring. Uveal
involvement is universal in stromal involvement. There may be vascularisation, stromal
necrosis, and perforation.
Various types of stromal involvement are :
Disciform keratitis
Interstitial keratitis
Immune ring
Limbal vasculitis
Necrotising keratitis
Stromal involvement can be :
1. Necrotising and 2. Non-necrotising
Necrotising keratitis is cause by active replication of virus in the lesion. It is an
active stromal infiltrative process that may develop with or without epithelial defect. Stroma
becomes necrotic with cheesy appearance. It is associated with anterior uveitis with keratic
precipitates, just under the infiltration. There is no hypopyon. Presence of hypopyon is
associated with secondary infection. Vascularisation and scarring are common. Necrosis of
stroma may lead to perforation.
Disciform keratitis is an example of non necrotising deep keratitis. It is an antigen
antibody reaction, generally associated with local or systemic immuno suppression that is
produced following use of strong chemical to cauterise the ulcer, use of strong steroid, or
iodoxuridine. There is a localised area of stromal edema generally in the centre of cornea.
The epithelium over the stromal thickening is elevated. There may be an endothelial
defect just under the stromal thickening. Large white KP are very common just beneath the
edematous area which is circular as the term disciform suggests. There may be folds in
Descemets membrane. It is always associated with anterior uveitis. IOP may be raised. The
condition is self limiting, it may take few weeks to resolve. Even when it resolves, it leaves a
ring shaped opacity that represent the periphery of the lesion.
Complications of herpes simplex keratitis are :
Anterior uveitis, secondary glaucoma, complicated cataract, deep vascularisation of cor-
nea, scleritis, corneal scarring, trophic ulcer, perforation.
Management of HSV infection of cornea :
Herpes simplex keratitis is said to be a self limiting disease, it takes weeks to month for
both epithelial and stromal disease to heal, recurrence keratitis makes prognosis poor.
Secondary bacterial and fungal infection are potentially dangerous. Condition becomes worse
with indiscriminate use of steroid. Strong chemical cautery, loss of sensation are important
causes of perforation. HSV infection does not impart local or systemic immunity. There is no
known method of immunisation.
Aim of treatment is :
1. Eliminate virus by anti viral drugs - local or systemic.
2. Management of associated uveitis and glaucoma.
3. Judicious use of weak steroid for stromal involvement.
4. Protect the hyposthetic cornea from physical, chemical and microbial onslaught.
5. Increase local and systemic immunity.
6. Keratoplasty.
Antiviral agents used in HSV keratitis
52, 53, 54, 55, 56
1. Idoxuridine. This is a thymidine analog. It is used as 0.1% drops or 0.5% oint-
ment. It is effective in epithelial lesions both primary and recurrent. It does not penetrate the
stroma and is insoluble in water, hence it has little use in stromal involvement. It is used in
dendritic and geographical ulcers. Its main drawbacks areinsolubility in water, local
epithelial toxicity and development of resistance, hypersensitivity, development of
superficial punctate keratitis. Idoxuridine is used as 0.1% drops every hourly by day and
two hourly by night for five days with 0.5% ointments three times a day. It may have to be used
for two to three weeks. It is better to discontinue the drug after three weeks due to its corneal
toxicity.
Trifluridine (Trifluorothymidine) is also an analog to thymidine. It inhibits DNA
both in virus as well as host. It is used as 1% clear solution. It is used against both primary
and recurrent HSV virus. It is more effective than idoxuridine and vidarabine. It is less
toxic than the two. It is effective in stromal keratitis as well. Cross resistance with other
antiviral drugs do not occur. It is used as nine times a day for ten days.
Vidarabine. This is an adenosine analog, a DNA inhibitor of both virus and host
cell. DNA inhibition in host cell is less marked than in virus. It is available as 3% ophthalmic
ointment to be used five times a day for five days. It is effective against epithelial keratitis.
Treatment is not extended beyond three weeks for fear of corneal toxicity, which is however
less than met with idoxuridine. Is is as effective as idoxuridine. Sometimes it is effective when
idoxuridine is ineffective. It is less effective than trifluridine.
Acyclovir. This is an analog of guanosine. It stops viral replication. It is highly effec-
tive 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 associ-
ated anterior uveitis which is inevitable in stromal keratitis. They also abolish pain of cyclospasm
associated with epithelial defect.
Commonly used cycloplegics are :
1. Atropine sulphate 1% ointment (drops should be avoided in children to eliminate
over dosage).
2. Home atropine hydrobromide 5% drops two to three times a day.
3. Cyclopentolate HCL. One percent drops three times a day.
(ii) Cortico steroid. These are double edged medicament that are indicated in stromal
involvement but contra indicated in epithelial ulcer. They should be used under um-
brella 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 bacte-
rial 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 car-
bolic 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 in-
fection. 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 HSV
57
infection is a life threatening disease, it requires management
by neonatologist along with local treatment by antiviral drops.
4. Recurrent HSV keratitis. There is no effective method of preventing recurrence
because primary infection does not provide any immunity. Latent period between primary
attack and subsequent recurrence is variable even gaps between attacks of two episodes of
recurrence is not definite. Number of recurrence is also uncertain. It is claimed that if trigger
mechanism like fever, stress, food allergen, ultra violet exposure can be avoided, recurrence is
averted.
One of recommendation is to put patients above twelve years on oral 200 mg acyclovir
every alternate day few months after patient has received a full course of oral acyclovir i.e. 200
to 400 mg five times a day for five to ten days.
5. Dendritic and Geographic ulcer.
(i) First antiviral drug of choice should be trifluridine locally whenever available or
acyclovir locally supplemented by oral acyclovir.
Idoxuridine and vidarabine are effective against epithelial lesions only idoxuridine is
most kerato toxic.
(ii) Debridement. Simple debridement is an effective treatment for epithelial keratitis,
debridement may be followed by local instillation of trifluridine or acyclovir. Chemical cau-
tery by 7% alchoholic solution of iodine and carbolic was standard treatment before advent of
antiviral drugs. Now they have been given up. However when antiviral drugs are not avail-
able, they still hold the fort when done carefully.
(iii) Cycloplegic. Cycloplegics are neither antiviral nor anti inflammatory analgesic.
Their main function is to treat associated anterior uveitis. Whenever there is associated
lacrimation and photophobia, instillation of cycloplegic drugs relieve discomfort.
6. Stromal involvement. Stromal involvement both necrotising or non necrotising
are most difficult to manage. Idoxuridine and vidarabine have no role in stromal in-
volvement. Trifluridne and acyclovir are effective in stromal herpes. Stromal HSV infec-
tion is always associated with uveitis making use of cycloplegic almost mandatory. Use of
steroid is one of the trickiest decisions. Whenever steroids are deemed necessary it should be
under cover of effective antiviral drug, well supervised and with weakest strength, least fre-
quently for shortest possible period.
7. Metaherpatic keratitis. Besides antiviral drops, lubricants, pad and bandage, band-
age contact lens help to epithelise the effected cornea.
Varicella Zoster Viral Keratitis
Herpes zoster ophthalmicus
54
. This is an acute multi systemic disease with chronic
sequel caused by a DNA virus that produces two distinct type of systemic disease i.e. vericella
(chicken pox) and herpes zoster (shingles). The herpes zoster virus is similar to herpes
simplex virus in structure but different antigenically and clinically. Both viruses have
primary infection followed by a period of latency and a recurrence. In herpes zoster there is a
single recurrence that may have a prolonged course while more than one recurrences are
common in HSV infection. In both conditions the virus lies dormant in posterior root gan-
glion only to be activated after an indefinite latent period when the virus travels down the
nerve to produce mucocutaneous and ocular lesion. Herpes zoster virus involves single
dermatome HSV has irregular distribution. Herpes zoster infection does not cross the
midline. Herpes zoster gives life long immunity.
Herpes zoster virus infection differs from vericella in following ways. Vericella is very
common, self limiting disease with predominant skin involvement and less pronounced ocular
involvement in children. It is rare in adults, while herpes zoster infection is more common in
adults, however children are not immune. Ocular involvement in herpes zoster is known as
herpes zoster opthalmicus that has severe vision threatening ocular involvement with
neurological defect, which may involve other cranial nerves as well. In rare cases it may
produce contra lateral hemiplegia. In severe viremia encephalitis is possible. Involvement
of cranial nerves other than ophthalmic is mostly immunological rather than infectious in
nature. (See Chapter 22 as well)
Though involvement of first division of trigeminal is commonest manifestation. The
second and third divisions may also be involved. Involvement of maxillary division produces
vesicular eruption in the lower lid, which is otherwise rare. Any of the branches of ophthalmic
division may be involved but common involvements are supra orbital, supra trochlear,
nasociliary and lacrimal. Involvement of nasociliary nerve that supply the lateral part of
the skin of nose and tip of the nose is invariably associated with corneal involvement
(Hutchinsons sign). However there may be corneal involvement without involvement of
nasociliary nerve, reverse is also possible. There may be localised involvement of any of the
branches of ophthalmic division without ocular involvement. Only ocular involvement without
cutaneous manifestation has not been observed.
Ocular involvement in herpes zoster ophthalmicus can be divided under following heads :
1. Cutaneous, 2. Kerato conjunctival, 3. Scleral,
4. Uveal, 5. Others.
1. Cutaneous. Skin of the lids, forehead and scalp are most commonly involved. There
is generally mild pain in the distribution of dermatome where vescicles develop in due course.
Initially the lesions are maculo papular which become pustular within few days, that rup-
ture to form crust. The crusts gradually fall off leaving depressed marks similar to chicken
pox. The crusts are loaded with live viruses, which are infective to others and can cause chicken
pox in children. The papulo macular stage is associated with intense edema that may spill
over the mid line but not the rashes.
Edema of the lids may obliterate the interpalpebral fissure due to mechanical pseudo
ptosis. As the scabs fall of the edema subsides with punched out scar marks that can either be
hyperpigmented or hypopigmented. Vescicles on the lid margin may heal by producing
notch in the lid margin. It can produce madarosis, trichiasis, ectropion or entropion.
There may be ptosis due to various factors i.e. :
1. Pseudo ptosis is generally due to inflammatory edema.
2. True ptosis is due to involvement of upper division of third nerve.
Some degree of loss of sensation is always present, may last for years associated with
post herpetic neuralgia.
Generally orbiculosis is not involved in herpes zoster ophthalmicus. Commonest cause
of paralysis of orbicularis is Ramsay Hunt syndrome where vescicles develops in the exter-
nal auditory canal. However rarely orbicularis palsy may develop independent of Ramsay Hunt
syndrome. Involvement of nasociliary nerve produces development of vescicle on the lateral
side of nose and tip of nose. This is invariably accompanied with corneal involvement.
2. Kerato conjunctival involvement :
1. Conjunctival involvement become obvious within first few days as mucopurulent
conjunctivitis. This is always associated with vescicles of the lid. Mucopurulent
conjunctivitis becomes worse with obliteration of I.P.A. Rarely there may be vescicles
on the conjunctiva itself. Conjunctival sensation is either lost or diminished.
2. Corneal involvement. Corneal involvement is one of the most serious complica-
tions of herpes zoster ophthalmicus, effect of which could last for years and may
cause permanent damage.
(i) Hypoethesia of cornea. First and foremost sign is diminished corneal sensa-
tion that may range between reversible hypoethesia to irreversible anaesthe-
sia that on long run may lead to trophic ulcer.
(ii) Keratitis. Earliest infective manifestation is punctate lesions in epithelium. They
are generally multiple, mostly on the periphery, stain with rose bengal and
contain live viruses, which may respond to local antiviral drugs. They appear
within forty-eight hours of onset of skin lesion. They are transient in nature,
however, some of them may coalesce to form lesions similar to dendrites. Dendrites
of herpes zoster are smaller than dendrites of herpes simplex. They stain with
rose bengal, they are transient, are seen between fourth to sixth day. By tenth
day there is infiltration of superficial stroma in the form of nummular keratitis
that may last for months and cause vascularisation of cornea.
Disciform keratitis is late feature, by this time the rashes have crusted and may have
fallen i.e. three weeks after appearance of rashes. Disciform keratitis are associated with
thickening of stroma and anterior uveitis. It may be vascularised and cause lipid degen-
eration.
Loss of sensation leads to formation of trophic ulcer. Due to loss of sensation patient
does not complain of pain that may lead to secondary bacterial infection resulting in perfora-
tion. Mucous plaque formation is an unique feature of herpes zoster ophthalmicus that de-
velops between third and sixth month in the form of deposit of mucus on swollen epithelium.
The plaque can be removed with ease. It is associated with anterior uveitis and stromal
infiltration.
3. Scleral. Episcleritis and scleritis are very common in herpes zoster ophthalmicus in
first week following onset of rashes, may be missed due to swollen lid and inflamed conjunc-
tiva. Scleritis may cause sclerokeratitis and sclero uveitis
4. Uveitis. Uveitis in herpes zoster ophthalmicus is limited to anterior uvea causing
acute iridocyclitis within fortnight.
Iridocyclitis in herpes zoster is a non granulomatous in nature. To begin with
iridocyclitis is mild with fine KP and few cells and flare. In severe cases hypopyon may de-
velop due to ischemic necrosis of iris which in turn leads to iris atrophy and formation of
holes in iris.
5. A common complication of herpes zoster uveitis is secondary glaucoma that may be
overshadowed by keratitis and missed altogether unless sought specifically. It is caused due to
associated trabeculitis, the swollon trabecular meshwork and inflammatory debris
may cause obstruction of the drainage.
Neurological involvement in herpes zoster ophthalmicus :
(i) Commonest neurological sign of herpes zoster ophthalmicus is loss of sensation.
On the distribution of the dermatome involved leading to anaesthesia of cornea,
conjunctiva and skin resulting in delayed healing and may lead to trophic ulcer.
Anaesthesia masks pain of corneal and uveal involvement.
(ii) The next bothersome symptom is post herpetic neuralgia that may persist for
months to years. Fortunately it is rare and milder in children. It is very severe in
old age. Exact cause of post herpetic neuralgia in absence of sensation is not well
understood. It is called anaesthesia dolorosa.
(iii) Other neurological involvements areoptic neuritis, cranial nerve palsy and
contra lateral hemiplegia that may take few months to develop. Encephalitis is
a rare manifestation of herpes zoster ophthalmicus.
Management of herpes zoster ophthalmicus
58
. Management of herpes zoster
ophthalmicus in children does not differ from management in adults. Children have advan-
tage of less frequent post herpetic neuralgia which is very mild.
1. Skin lesions are best treated with oral acyclovir in dose of 50-60 mg/kg/day divided
in five doses. Children can get as much as 400 mg acyclovir five times a day for 7
days. This reduces viremia and enhances healing, resulting in scar formation. Acyclovir
does not have much effect on corneal lesions or postherpeticneuralgia but is known to
diminish uveal involvement. Acyclovir should be started within 48 hours of onset of
rashes. Local skin ointment of acyclovir is applied in the vesicular stage. In the ve-
sicular stage combination of calamine with talcum powder or starch should be avoided
as it masks the changes in the vescicles, cause secondary infection and deep scar
formation. Combination of antibiotic and steroid is recommended at the crusting stage.
Systemic corticosteroid under the umbrella of systemic antiviral drugs reduces
lid edema, and deep scar. Steroids also helps in reducing post herpetic neuralgia. They help to
treat stromal involvement and uveitis.
As pain is not a very prominent feature of herpes zoster ophthalmicus in children, mild
analgesic given orally is sufficient.
Active immunisation against vericella has been recommended to prevent herpes zoster
but its efficacy has not been proved beyond doubt.
2. Conjunctival lesions are treated by local broad spectrum antibiotics instilled in the
conjunctiva. Role of local antiviral drugs to combat conjunctivitis is not established.
3. Corneal involvement in herpes zoster is a clinical riddle. Local antiviral drugs are
of doubtful value. Local steroids must be given under strict medical supervi-
sion. Steroid in weakest possible strength is recommended. The patient must be put
on steroid for months. Loss of corneal sensation is a perpetual problem that re-
quires frequent instillation of broad spectrum antibiotic, long acting cycloplegic. If
necessary, tarsoraphy must be performed. Loss of corneal sensation is a contra indi-
cation for keratoplasty and contact lens.
4. Optic neuritis, cranial nerve palsy are self limiting. Systemic steroid helps in short-
ening the course.
Adeno virus keratitis in children
62
Almost all adeno viruses that cause conjunctivitis in children involve cornea to some
degree. Corneal involvement is seen five to seven days after onset of conjunctivitis which has
various degree of follicular reaction and sometimes pseudo membrane formation. Adeno
viral keratitis is associated with systemic symptom and pre auricular lymphadenopathy.
Corneal involvement in adeno viral infection is less common and less troublesome than seen in
herpes simplex. Corneal involvement are less marked in children than in adults. They are
generally epithelial in nature and present as fine superficial keratitis that stain brightly
with fluorescein. Sub-epithelial involvement is less frequent, transient and leave no scar.
Fungal keratitis
42, 63, 64
Fungal infection of cornea is less frequent than bacterial or viral keratitis. It is gener-
ally unilateral and central following history of injury. The injury is caused by foreign
body of vegetable origin. It is more common in hot and humid climate than in cold and dry
climate. Besides trauma, other conditions that predispose fungal keratitis are situations where
corneal defence mechanism is compromised i.e. loss of sensation, stromal herpetic
keratitis, prolonged local or systemic steroid therapy, paralysis of orbicularis, dry eye, vitamin
A deficiency. Fungal keratitis is a suppurative, indolent corneal ulcer with severe ante-
rior chamber reaction, hypopyon and conjunctival congestion. It takes more than ten
to fifteen days for a fungal corneal ulcer to develop following trauma by vegetable material.
There is an initial corneal abrasion that either heals spontaneously or is treated as bacterial
corneal ulcer generally with antibiotic drops. Use of steroid in such situation makes the condi-
tion worse. Severity of infection depends upon size of inoculum and immuno competence of the
cornea.
Fungi that cause corneal ulcer can be divided into two main groups i.e.
1. Filamentous and 2. Non filamentous. Common fillamentous fungi that cause corneal
ulcer are aspergillus, fusarium, cephalosporium. (See Chapter 22 also)
Following trauma with vegetable matter, the initial breach in epithelium heals as simple
corneal ulcer. After a gap of ten to fifteen days acute fungal keratitis becomes manifest causing
lacrimation, photophobia, blurring of vision and pain. The appearance is characteristic;
first a sub epithelial gray white lesion develops at the site of trauma. The epithelium is
raised over this and a dirty white, raised dry rough lesion with raised margin develops. Sub
epithelial and stromal feathery extension may develop. At the end of the feathery extension
multiple satellite lesions may develop which vary in number, shape and size. Their appearance
is similar to original central ulcer. The satellite ulcer may join to form a ring called Wessely
ring. There may be a epithelial plaque formation. The conjunctiva is severely inflamed.
There is intense anterior chamber reaction with thick yellowish white hypopyon and
severe anterior uveitis. The ulcer may extend in to surrounding sclera.
It is not always possible to differentiate non filamentous fungal ulcer from filamentous
ulcer. Unlike bacterial ulcers there are no characteristic features that can help clinically to
differentiate various filamentous ulcers.
Management and complication of all fungal corneal ulcers is almost the same. If a fun-
gal ulcer is not treated in time, it invariably perforates and the eye is lost. Even if the ulcer
does not perforate, it leaves a dense central opacity that compromises vision to great extend.
Other common complications are secondary glaucoma, complicated cataract, and phthisis.
Diagnosis :
Diagnosis of fungal keratitis depends on :
1. History 2. Appearance of ulcer
3. Its clinical course and 4. Laboratory finding
History of injury by organic matter specially of vegetable origin about a fort night ago
with development of ulcer should alert the physician for possible fungal ulcer that have typical
features described above and not responding to usual antibiotic and cycloplegic drops.
Laboratory finding consists of :
1. Examination of corneal scrapping :
1. In KOH solution
65
2. Staining Grams stain
Giemsa stain
Gomori methamine silver stain
Lactophenol cotton blue
66
Caleoflour white
67
Culture
68
Sabourauds medium
Blood agar
Brain heart infusion
1. 10% KOH solution is used most frequently to visualise septed fungal hyphae di-
rectly under microscope. It is a reliable test.
2. Gram stain is useful to visualise candida, it fails to stain filamentous fungi.
3. Giemsa stain is more useful to define filamentous fungus.
4. Gomori stain provide better fungal cytology.
Culture is done at room temperature, it may take as long as fifteen to twenty days to
show positive result.
Management of fungal keratitis :
Management of fungal corneal ulcer is one of the most frustrating forms of treatment
due to :
1. Failure to diagnose.
2. Non availability of broad spectrum antifungal antibiotic.
3. Frequent serious complications.
Antifungal drugs
69
:
Three groups of antifungal drops are available.
They are :
1. Polyenes, 2. Imidazoles, 3. Pyrimidines
1. Polyenes. Amphotericin B, nystatin, natamycin
2. Imidazoles. Clotrimazole, miconazole, ketoconazole, econazole
3. Pyrimidine. Flucytocine
In all cases of suspected fungal keratitis, initial treatment is instillation of natamycin
5% drop every hourly by day. If stain confirms presence of yeast (candida) nystatin 50,000 IU,
flucytosine 1% and amphotericin B 0.1% to 0.2% should be given every hourly.
In case of confirmed filamentous fungal ulcer, miconazole, ketaconazole or clotrimazole
may be given hourly.
Antifungal treatment should be continued for at least ten days before any improvement
occurs.
All cases should get 1% atropine sulphate as ointment two times a day. In children
atropine should be administered under supervision to avoid serious systemic complication.
Local betablockers and oral acetazolamide keeps IOP low and lowers incidence of perfo-
ration.
Local broad spectrum antibiotic drops are given to prevent secondary bacterial infec-
tion.
Cortico steroids have no role in management of fungal keratitis
Surgical treatment. Good debridement if necessary under general anaesthesia helps
to remove infected material. Debridement may be followed by chemical cautery. Too enthusi-
astic cauterisation may enhance perforation hence it should be done carefully under magnifi-
cation.
Lamellar keratoplasty fails to eliminate the infection. Penetrating keratoplasty shows
better results. However the donor cornea itself may get infected in course of time.
Peripheral keratitis
70
Peripheral keratitis may result due to many local and systemic condition both infective
as well as non infective. Non infective causes outnumber infective causes. They are generally
bilateral, in spite of a benign course they are more painful than infective keratitis. They are
caused by allergy, or auto immune disorders. Peripheral ulcers by themselves do not per-
forate. Perforation is due to secondary bacterial invasion. The keratitis may be self limiting.
Most of them respond to local steroid. Anterior chamber reaction is either absent or minimal.
Out of many marginal keratitis only phlyctenulosis of cornea is commonly seen in older
children. Some times marginal (catarrhal) ulcer may be seen in older children who may also be
effected by autoimmune diseases like rheumatoid arthritis.
Phlyctenulosis of cornea. Phlyctenular kerato conjunctivitis is very common in de-
veloping countries. It may be limited to conjunctiva only, may involve limbus effecting both
cornea and conjunctiva or may primarily develop in the cornea.
Other causes of peripheral keratitis are - Vernal kerato conjunctivitis and rheumatoid
arthtitis, Moorens ulcer, which is an important marginal keratitis is not seen in children.
Parenchymatous (Interstitial) Keratitis in Children
71, 72, 73, 74
. Interstitial keratitis
is a chronic disease of cornea with acute onset and protracted course. It can be diffuse or
central. In children interstitial keratitis is generally diffuse and bilateral. The other eye get-
ting involved few days after the first. It is due to an antigen antibody reaction to spirochete
trapenoma pallidum, tuberculosis and leprosy. Other causes are - Lymphogranuloma
venereum, herpes simplex, malaria, onchocerciasis.
In children commonest type of interstitial keratitis is due to syphilis in the form of
congenital syphilis or hereditary syphilis. The child is infected in utro between first and
second trimester, interstitial keratitis manifesting between 5 to 15 years of age.
Primarily interstitial keratitis is a disease of stroma. The epithelium and endothelium
getting involved later. In late stages all the layers of cornea get effected. Anterior uveitis is
early and prominent feature.
Uveal involvement is so pronounced that it is thought that basic pathology starts in the
uvea and cornea gets involved secondarily. Exact pathology of interstitial keratitis is not
well understood. Trapenoma has been isolated from syphilitic foetus and new born without
evidence of interstitial keratitis. Conversely trapenoma has not been demonstrated in cornea
in interstitial keratitis. The disease is self limiting, it responds well to steroids but not to pure
anti syphiletic treatment. Interstitial keratitis has a long latent period of five to ten years
following intrauterine infection.
The clinical picture has been divided into :
1. Progressive ; 2. Florid and ; 3. Regressive stages.
1. Progressive stage lasts for two to three weeks following clinical symptom of
lacrimation, photophobia, blepharospasm. On examination there is well developed
circumcorneal congestion and haziness of cornea that starts from periphery extend-
ing towards centre followed by sprouting of new vessels in the stroma. This is the
time when uveitis develops mostly in the form of acute iridocyclitis. There may be
anterior choroiditis as well. Changes in iris are masked by corneal haze. There
may be involvement of retina and choroid that result in to salt pepper appear-
ance.
2. Florid stageProgressive stage is followed by florid stage that lasts for two to
three months. This is the stage when the symptoms are in peak. The cornea develops
heavy deep vascularisation that gives a red hue to the cornea. Vascularisation
spread from all sides.
It is expected that vessels in cornea should have bright red colour, due to corneal haze
the redness is toned down to a peculiar pink colour known as Salmon red colour.
Causes of haziness are due to changes in all the layers of cornea i.e. from epithelium to
endothelium with keratic precipitates. These changes when added result into a hazy cornea
(ground glass appearance). Other change seen in cornea on biomicroscopy are; bedewing of
epithelium, cellular infiltration round the newly formed vessels in front of the Descemets
membrane, which is wrinkled. The Bowmans membrane is wavy while deep vascularisation is
developing in the stroma. Superficial vascularisation may be superimposed in the epithelium
on the periphery resulting into a crecentic pile of blood vessels known as epaulet.
3. Regressive stageAfter four to five weeks, stage of regression starts which lasts
for two to three years. Process of regression starts as the advancing vessel meet in
the centre. Once the cornea is fully vascularised, inflammation suddenly subsides
with clearing of the cornea, improvement in vision, reduction of lacrimation, pain
and photophobia. Corneal clearing starts from periphery and slowly regains its trans-
parency leaving only obliterated vessels as white streaks that are visible on slit
lamp for years called ghost vessels. Besides ghost vessels late sequele are band
keratopathy and overall thinning of cornea.
Systemic involvement in congenital syphilis :
1. Dental changesHutchinsons teeth
The permanent upper central incisors are peg shaped reduced in length and breadth.
They have a notch on the cutting edge.
2. Changes in earpermanent deafness.
Interstitialkeratitis, Hutchinsons teeth and deafness constitute Hutchinsons triade.
3. Foreheadshows frontal eminence.
4. NoseSaddle shaped depression.
5. Rhagades at angle of mouth.
6. Other changes areRetarded physical and mental growth, anterior bowing of shin,
Cluttons joint, cardio vascular disorders.
Differential diagnosis consists ofAll causes of hazy cornea i.e. buphthalmos, tra-
choma and congenital corneal haze.
Diagnosis. Diagnosis is clinically simple, if possibility of interstitial keratitis is kept in
mind especially in a child borne to known syphilitic mother. Otherwise stigmata of congenital
syphilis i.e. saddle nose, frontal bossing, Hutchinsons teeth, deafness with severely vascularised
cornea leads to diagnosis.
Laboratory investigations
72
include
1. Positive serological test in mother and child.
2. Reactive CSF-VDRL.
3. Elevated cell or protein in CSF without any other cause.
Treatment
72
. Though systemic anti syphilitic treatment does not influence the ocu-
lar condition it is mandatory to treat the child with appropriate dose of penicillin to
prevent future cardiovascular and maningovascular complication.
Interstitial keratitis is self limiting disease which starts improving within two to three
months. The condition is very troublesome to the child who has intense lacrimation, photophobia
and diminished vision. Local steroids reduce this period of ocular morbidity to few weeks.
Cortico steroids are instilled at a rate of one drop every hour during day for first two days then
gradually reduced in frequency over months. Once acute phase has subsided subconjunctival
depot steroid can be given at an interval of 15 days. To treat associated uveitis that is always
C-8\C:\N-AGE\NE-C7B.PM5
present and is a dominant feature cycloplegic drugs are used in acute phase, atropine
sulphate is used as one percent drop two times a day under supervision along with local
steroids. Once acute phase has subsided atropine can be replaced by any of the following short
action cycloplegic i.e. 2% home atropine hydrobromide, 1% cyclopentolate hydrochlo-
ride or 1% tropicamide. Only sympathomimetic drug like phenyl pherine has no role in
management of uveitis. It only produces immediate blanching of conjunctival vessels giving a
false impression of white eye. Children on prolonged local steroids should be closely monitored
for rise of tension, steroid induced cataract and secondary infection.
Acanthamoebae keratitis
38, 75, 76
Acanthamoebae keratitis was unknown three decades earlier because the organism,
which is found normally in human throat and pharynx was thought to be non-infective as far
as eyes were concerned. However, it remains a relatively rare disease, incidence of which is
increasing. There are eight species of acanthamoebae that cause corneal ulcer. The organ-
ism is found in two forms i.e. trophozoite and cyst. Besides throat and pharynx the organ-
ism is commonly found in fresh water, soil, home made contact lens solutions, all types
of contact lenses.
Keratitis produced by acanthamoebae is indolent, non-suppurative, resistant to
antibiotic, antiviral and antifungal drugs. It is difficult to culture. It thrives on gram
negative bacteria and cynobacteria at 2535C temperature.
Predisposing factors include :
1. Use of any type of contact lens
2. Use of non standard contact lens solutions specially home made.
3. Swimming in a contaminated pool.
4. Trauma by vegetable matter. It is being reported more commonly among non contact
lens users following trauma.
Corneal lesions may be as mild as epidemic kerato conjunctivitis or as severe as
Descemtocele or perforation. The condition can be put in following three stages :
Stage I. This initial stage mimic herpes simplex or bacterial keratitis and is confused as
such. The changes consist of one or combination of many punctate lesions, pseudo dendrite
and epithelial defect. Symptoms at this stage are foreign body sensation, redness and
photophobia.
Stage II. This consists of anterior stromal ring, disciform keratitis, formation
of double ring, limbitis and scleritis.
Stage III. Corneal abscess, severe disciform keratitis, Desmetocele, iritis, scleritis,
hypopyon and sometimes hyphaema.
All the stages are very painful. Pain is out of proportionate to the size of corneal involve-
ment.
Diagnosis
77, 78, 79
. Diagnosis is generally missed. A high index of suspicion in all pain-
ful ulcers developing in contact lens users or following trauma that do not respond to usual
corneal ulcer treatment generally leads to correct diagnosis. However diagnosis after four
weeks of onset is always fraught with failure.
All suspicious cases of kerato acanthamebae should undergo examination of corneal
scrapping to exclude bacterial, viral and fungal ulcer. Unfortunately herpes simplex keratitis
is very common with acanthameba keratitis. The scrapped material should be stained with
hematoxylin and eosin, Grams stain, Giemsa stain, lactophenol cotton blue and
celluflour white. The material can be examined after immuno fluorescent stain.
Cysts have a typical polygonal double walled appearance. It is difficult to culture the
organism, however, the material may be inoculated on blood agar, non nutrient agar
79a
or
chocolate agar that has been overlaid with killed E.Coli.
Management. Treatment of kerato acanthameba is difficult. It requires constant and
prolonged treatment with specific drug, result of which may be frustrating.
Management consists of
76
:
1. Prevention by proper contact lens storage and cleaning.
2. Instillation of anti acanthamebial drugs.
3. Usual treatment of corneal ulcer, uveitis and secondary glaucoma.
4. Penetrating keratoplasty.
Anti acanthamebic drugs are :
1. BiguanidesPolymeric-polyhexa methylene biguanide 0.02% solution hourly for first
three days then alternate hourly for 2 to 3 weeks. The frequency is reduced over months.
2. Diamidine
1. Propamindine 0.1%
2. Hexamidine 0.1% as solution to be instilled for 2 days, followed by six times a day for
3 months.
3. AntibioticsNeomycin 5.0 mg/drops to be instilled 2 hourly.
4. Antifungal
A. Local
(i) Ketaconazole
(ii) Clotrimazole 1% drop
B. Systemic
(i) Ketaconazole
(ii) Fluconazole
Usual treatment of non healing corneal ulcers consists of :
(i) Strong cycloplegic i.e. atropine sulphate 1% drop two times a day (in children atro-
pine should be used under supervision).
(ii) Repeated debridement
(iii) Local beta blockers to keep the tension low.
Role of local steroids is controversial and better avoided.
Penetrating keratoplasty seems to be the ultimate method to salvage vision and save
the eyeball. The infected corneal button removed from the patient should be subjected to
histopathological identification of the organism.
All persons using contact lens should be warned about possibility of contamination by
various organism. They should be instructed to use standard cleaning solution. Use of home
made solution should be a taboo.
Corneal dystrophies
80, 81, 82
Corneal dystrophies are a group of bilateral, non-inflammatory disorder of cornea.
They are hereditary in nature of varied mode of transmission, however autosomal domi-
nant trait is most common type of inheritance. Exact causes of corneal dystrophies are not
known. They may be seen at any age but most of them start in childhood. Some of them
may be present at birth. Corneal dystrophies generally involve central cornea, only few
extend to the periphery, loss of vision depends upon position of the opacity in relation to the
pupil and density of the opacities. Generally there are multiple opacities. Some of the
dystrophies have loss of corneal sensation many of them develop recurrent erosion of
corneal epithelium, dystrophic cornea do not get vascularised. There is no evidence of
any other ocular disease in the affected eye. As corneal dystrophies are relatively uncommon
and many of them may not require any treatment hence they are missed most of the time
specially in under developed countries where bilateral opacities due to infection, inflammation
and malnutrition are very common. All corneas with small central bilateral opacities with-
out congestion and anterior segment reaction should arouse suspicion of corneal dystro-
phy and as many members of the family in as many generation possible should be examined to
confirm diagnosis. There is no known medical treatment for corneal dystrophy. La-
mellar corneal graft or penetrating keratoplasty or excimer may help to remove the
opacities and improve vision. Recurrence of disease in grafted cornea is known.
Classification. Corneal dystrophies are best classified according to topographic loca-
tion of the dystrophy.
A. Affecting anterior membrane
(i) Affecting epithelium
(ii) Affecting Bowmans membrane
(iii) They involve superficial stroma.
B. Affecting stroma
C. Affecting the posterior membrane i.e. Descemets membrane and endothelium.
D. Combined
Following are a few common dystrophies that are seen in children.
Anterior corneal dystrophies Commonly seen in children :
1. Hereditary juvenile epithelial dystrophy (Meesmanns). This is an autosomal
dominant disorder, generally seen between 3 to 4 years of age. It has been reported under
one year also. Visual acuity is not much reduced the lesions are best seen on retro illumination
as micro vescicles in the deeper layers of epithelium. In the inter palpebral fissure corneal
sensation is reduced. The disorder progresses very little. If vision is reduced sufficiently to
hamper patients routine then only lamellar keratoplasty is recommended. The epithelial micro
vescicles may raise the epithelium, this causes tear film abnormality. There may be irregular
astigmatism. If corneal erosion occurs, which is rare, bandage contact lenses may be helpful.
2. Hereditary epithelial dystrophy (Stocker and Holt). This is an autosomal domi-
nant dystrophy, which is sometimes considered as variant of Messmanns dystrophy. It starts
in infancy, loss of vision varies between slight haze to severe loss due to total opacification of
the cornea. The epithelial surface is irregular leading to irregular astigmatism. Corneal
sensitivity is sub-normal. Treatment depends upon degree of visual loss. In severe visual loss
lamellar keratoplasty is advocated. In both Messmanns dystrophy and Stocker and Holt dys-
trophy, anterior stromal puncture along with bandage corneal lens may help epithelisation
of erosion. Anterior stromal puncture is generally done by fine needle under magnification.
This can also be done by YAG laser. Excimer laser is also used in case of recurrent corneal
erosion.
3. Reis-Bucklers dystrophy. This condition is less common than previous two condi-
tions. Inheritance is autosomal dominant. There are two types of this Bowmans membrane
dystrophy i.e. (1) Classical less common variety and (2) More frequent type II. Common age of
onset is three to five years. Loss of vision is severe as compared to other anterior
dystrophies. Corneal sensation is reduced. Erosion is common. The lesions may lead to central
corneal opacification.
Management consist of local instillation of lubricating drop and ointment,
hyperosmotic agents and antibiotic agent. Patching of the eye at bed time gives much
relief to the patient, which can also be achieved by bandage contact lens. Soft contact lens
also helps to reduce irregular astigmatism. Lamellar or penetrating corneal graft is re-
quired if(1) Vision is much hampered, (2) Lesion has reached anterior stroma, (3) There is
vascularisation secondary to recurrent erosion.
Excimer laser photo therapeutic keratoplasty helps to ablate the epithelium. This
gives much respite from recurrent corneal erosion.
4. Hereditary anterior membrane dystrophy (Grayson-Wilbrandt). This is
autosomal dominant dystrophy not seen before ten years of age. There is gradual loss of vision.
Corneal sensation remains normal. On slit lamp examination the corneal nerves are found
to be thickened corneal opacity may require lamellar keratoplasty.
Stromal Dystrophies in Children
1. Lattice dystrophy (Biber-Haab). Lattice dystrophy is an autosomal dominant dis-
order that manifest at the end of first decade or early. In childhood vision is good that
gradually diminish over years. There may be recurrent corneal erosion. There is gradual in-
crease in corneal opacification that extend from limbus to limbus. The opacities are lattice in
nature. The branching opacities may be confused with corneal nerve. Corneal sensation is
good initially but gets reduced by third decade. Histochemically the opacities stain with Congo
red. In childhood vision is good and does not require treatment. Recurrent corneal erosion in
third and fourth decade is treated with artificial tear, lubricating ointment, soft contact
lens. The eye may require patching. As the lesion is deep, penetrating keratoplasty is
preferred. The donor cornea may develop opacities after ten years. Excimer laser, photo
therapeutic keratectomy may help in removal of opacities.
2. Granular corneal dystrophy (Groenouws I). The condition has autosomal inher-
itance, starts in the first decade of life in the central cornea as white granules. Stroma in
between the granules is clear hence initially there is no visual disturbance. The condition is
almost symptomless in childhood. Symptoms manifest after fourth decade as irritation
photophobia and diminished vision. Corneal sensation is normal. No treatment is warranted
in childhood. Penetrating keratoplasty may be required after forty years of age.
3. Macular dystrophy (Groenouws II). This differs from most of the corneal
dystrophies in being autosomal recessive. The disorder starts round about five years of age
as greyish white opacities in the mid cornea that may spread up to limbus and involve the
endothelium. By third decade there is troublesome corneal erosion.
Definite treatment is keratoplasty in third and fourth decade. Children do not require
any specific treatment. In suitable cases contact lens may help in improvement of vision.
Crystalline dystrophy (Schnyders). This dystrophy is seen under ten years of age,
may be present in infants. It has been reported at birth. It is autosomal dominant in inher-
itance. It starts in the centre of cornea as needle shaped crystals. The needles may come to-
gether to form a disc shaped opacity. The condition becomes stationary after third decade.
There is no vascularisation. Corneal sensation is normal. No treatment is required.
Posterior Dystrophies in Children
Cornea deeper to stroma is less frequently involved in dystrophic conditions in child-
hood. There are two condition that are seen in childhood. They are :
1. Posterior polymorphus dystrophy.
2. Congenital hereditary endothelial dystrophy.
Endothelial dystrophies are only conditions that may have other congenital anomalies
of anterior segment.
Posterior polymorphous dystrophy (P.P.M.D.). This condition is mostly seen at
birth, when not associated with other anomalies of anterior segment like lridocorneal
dysgenesis, correctopia, lrisatrophy, peripheral synechiae, congenital corneal edema, the con-
dition is almost symptomless, does not cause visual impairment. It is mostly autosomal in
nature, rarely it may be recessive.
Congenital hereditary endothelial dystrophy (CHED). This dystrophy manifests
before ten years, may be present at birth. There are two forms -
1. Autosomal dominant. This presents in early childhood, progresses slowly but is
symptomatic.
2. Autosomal recessive. This is present at birth but non symptomatic, diagnosed on
routine examination.
One unique feature of CHED is increased thickness of cornea. Vision may be be-
tween 20/40 to CF.
Treatment. Most of the children do not require any treatment. Other associated con-
genital anomalies should be treated individually.
COMPARATIVE FEATURE OF CORNEAL DYSTROPHY IN CHILDREN
Dystrophy Age of Heredity Visual Corneal Erosion Compli-
onset loss sensation cation
Treatment
Anterior Dystrophy
Meesmanns 3-4 yrs Autosomal Mild to Slight Nil, Not
dominant moderate reduction required.
BCL may
help in
erosion
Stocker-Holt Infancy Autosomal Mild to Reduced Irregular
dominant severe astigmatism
L.K., BCL
Reis-Buckler 3-4 years Autosomal Severe 6/60 Reduced Recurrent Corneal
dominant or less opacity
P.B., CL,
P.K,
Graysom- 10 yrs Autosomal Gradual loss Normal CL
Wilbrandt dominant
Stromal dystrophies
Groenouws 10 yrs Autosomal Moderate Reduced + + Corneal
I Lattice dominant opacity PK
Groenouws <10 yrs Recessive Severe Corneal
II Macular opacity PK
Granular 10 yrs Autosomal Mild N + + Corneal
dominant vasculari-
sation
Nil
Schnyder Infancy Autosomal Mild Normal Nil Nil
dominant
Posterior dystrophy
Posterior Congenital Autsomal Mild N Nil May develop
polymorphus dominant/ Glaucoma
corneal recessive Endothelial
dystrophy Decompen-
sation
Sympto-
matic
BCL = Bandage Contact Lens
CL = Contact Lens
LK = Lamellar Keratoplasty
PK = Penitrating Keratoplasty
PB = Pad and Bandage
CO = Corneal Opacity.
Corneal degenerations
83, 85, 86
Degenerations of cornea are far more common than dystrophies. They are not geneti-
cally predisposed. They can start at any age. They can be unilateral. Bilateral degeneration
are secondary either to bilateral ocular disorder like trauma, uveitis or systemic disorder like
abnormal calcium metabolism. Commonest type of corneal degeneration seen in children is
Band keratopathy.
Band keratopathy
84
. (See page 248-249 as well) Band keratopathy can be divided into :
1. Primary (rare)
2. Secondary
A. Secondary to ocular cause
B. Secondary to systemic cause.
Band keratopathy secondary to ocular disorder is commonest form of corneal degen-
eration seen in children. Chronic uveitis, chronic keratitis, penetrating in-
jury, chemical injuries, congenital anomalies are common predisposing fac-
tors. Though it takes months to years to develop band keratopathy, it is reported to
manifest within two to three weeks following alkali burn. The band keratopathy
itself does not produce any symptom initially. It takes few months to years to be
symptomatic. Band keratopathy develops in the inter palpebral fissure. It ex-
tends across the cornea in a band shaped opacity which starts from periphery and
spread towards the centre, pupillary area is last to be affected. However in rare
instance it may spread from centre to periphery. The opacity starts as a linear band
one millimetre inside the limbus on either side. The outer edge of the opacity is
sharply defined while the inner edge is irregular and serrated. Gradually opacities
from each end meet to cover the cornea. There are few areas of deficiency in the
opacity. They are called holes. Such holes correspond to corneal nerves. The opac-
ity is caused by extra cellular deposition of non crystalline salts of calcium
carbonate and phosphate at the level of Bowmans membrane and superfi-
cial stroma. The epithelium shows no change except that it may be irregular due to
underlying deposits.
Corneal sensation is not disturbed. The endothelium is not involved. Anterior cham-
ber reaction when present is legacy of primary disorder i.e. uveitis etc. Exact cause
of deposition of calcium at the level of Bowmans membrane in band keratopathy is
not well understood, so is its predilection for exposed part of cornea. In case of band
keratopathy secondary to ocular disorders, serum calcium is within normal range.
Band keratopathy has been reported following use of silicone oil, sodium hyaluronate,
sodium chordrontin sulphate, phenyl mercuric nitrate. Anterior segment ischaemia
produces early band keratopathy. It has been observed in failed keratoplasty.
Band keratoplasty secondary to systemic disorder. Band keratoplasty secondary
to systemic disorders are less common than the former. Topography of the opacity is similar in
both conditions. In latter the calcium is intra cellular and there is an alteration in calcium and
phosphorous metabolism.
Systemic conditions that cause band keratopathy areVitamin D toxicity, hyper
parathyroidism, uremia, milk alkali syndrome, sarcoidosis, chronic renal failure,
hereditary.
Primary band keratopathy. This is rarest form of band keratopathy. There is lack of
unanimity regarding its nature i.e. Is it a degeneration or dystrophy ? as there is overlaping of
characteristics of both conditions. The condition is X linked recessive trait, present at birth or
early childhood. There is no associated ocular disease. Besides cornea other parts of eye like
lens, choroid, retina may also be involved. These eyes generally become blind in early life.
Treatment of band keratopathy
87
. There is no known prophylaxis. However prop-
erly managed uveitis, glaucoma and trauma reduce the incidence. In band keratopathy sec-
ondary to systemic hyper calcimia, reduction in serum calcium sometimes clear the cornea.
Similarly discontinuation of vitamin D. Enthusiastic supply of vitamin D in children
should be discouraged.
There are two modes of treatment for band keratopathy
1. Use of a chelating agent
2. Keratoplasty
Treatment is indicated only in symptomatic cases. The success depends upon presence
or absence of effect of primary disorder. Treatment of band keratopathy will restore vision
only if the cause of defective vision is corneal. In presence of active inflammatory lesion or
reactivation of lesion, the calcium deposit may start again.
Use of chelating agent. Method consists of anaesthetising the cornea with local an-
aesthetic agent. In children the procedure should be done under general anaesthesia. Once
the cornea and conjunctiva have been anaesthetised the epithelium is removed by scrapping
with any suitable method. The calcium deposit is next scrapped off, the stroma preferably
under microscope. A Week-cel sponge is soaked in 0.5% solution of EDTA. This is applied on
the exposed calcium deposit, a to-and-from movement helps to remove the deposits better. If
0.5% EDTA fails to produce desired effect the strength of the solution is raised to 1% or 1.5%.
Once all the deposits have been removed, the eye is patched with cycloplegic and local antibi-
otic drops.
The aim of chelation is to remove the calcium without scarring of the stroma.
A lamellar keratectomy or excimer laser photo therapeutic keratoplasty are better op-
tions than chelation. Drawback with excimer laser treatment is that it can cause post opera-
tive undesirable hypermetropia.
Corneal opacity in new born and infants
88, 90
. Cornea of a new born is hazier than
normal. This haze clears within few days without any treatment and is generally not noticed
by mother or nurse unless looked for. However a child may be born with corneal haze sufficient
to be called opacity. A child may have bright and clear cornea at birth but later becomes hazy.
Commonest cause is buphthalmos. Corneal haze in new born may be unilateral or bilateral.
Commonest cause of unilateral haze is birth trauma. Buphthalmos that is more advanced in
one eye may also produce unilateral hazy cornea.
Common causes of corneal opacity are :
1. Sclero cornea of various degrees.
2. Tear in Descemets membrane 1. Birth trauma
2. Buphthalmos
3. Ulcer (keratitis) 1. Herpes simplex
2. Congenital rubella
3. Lid anomaly (Neurotrophic)
4. Metabolic Mucopolysaccharidosis
5. Posterior corneal defect Anterior chamber cleavage syndrome
6. Endothelial defect 1. Congenital hereditary endothelial
defect (CHED)
2. Posterior polymorphs dystrophy
3. Congenital hereditary, stromal
dystrophies
7. Dermoid 1. Corneal
2. Limbal
The above conditions are acronym as STUMPD
89
with beginning alphabet of each cause.
Interstitial keratitis is a common cause of bilateral corneal haze but seen only after
third year of life with redness, photophobia and lacrimation.
Xerophthalmia and Keratomalacia
91, 92, 93
Logically the term xerophthalmia should include all the conditions that produce non
wetability leading to dryness of ocular surface i.e. cornea and conjunctiva. However term
xerophthalmia
93
is generally used to denote dryness of conjunctiva and cornea due to vitamin
A deficiency. Similarly xerosis is a term used for which hypovitaminosis A is the prime cause.
There are many other factors that lead to dryness of conjunctiva. Keratomalacia
93
denotes
softening and aseptic and necrosis of cornea due to deficiency of vitamin A, that is generally
preceded by xerophthalmia.
Tear is essential to keep the cornea and the conjunctiva bright and moist. Normal tear
has three layers of different chemical composition and each has specific purpose. The layers
areLipid, aqueous and mucin. Deficiency of any layer will lead to tear film abnormality.
Aqueous layer is voluminous when compared to other layers. It is sandwiched between
lipid and mucin layer. The lipid layer prevents excess evaporation of aqueous layer and gives
a shinning surface while, the mucin layer anchors the aqueous layer to the cornea and the
conjunctiva. In hypo vitaminosis A there is hyposecretin from goblet cells of conjunctiva. The
aqueous and lipid layers are unaffected, lack of mucin causes abnormal break up time result-
ing in formation of large dry spot on the ocular surface. There is thickening and loss of
elasticity of conjunctiva, it may be keratinised which leads to opacification of conjunctiva.
There is increased pigment deposit on the conjunctiva that appears as dusty white patch.
Normally the conjunctiva is not thrown into vertical folds when the eye is rotated but in
case of dry conjunctiva, the conjunctiva is thrown into three to four vertical folds in the intra
palpebral aperture.
The exact mechanism of dryness of cornea is not understood. Lack of mucin hasten tear
break up and delayed bridging of the gap. There is degeneration of Bowmans membrane infil-
tration of stroma by inflammatory cells and fluid.
Vitamin A is essential for maintenance of conjunctival goblet cells that secrete mucous.
Daily requirement of vitamin A varies with age. An infant requires five times more than
an adult. An infant requires 65 mg/kg of vitamin A while requirement of adult is 12 mg per kg
of body weight that roughly comes to 2500 IU of pure vitamin A or 4000 IU of carotene.
Retinol is fat soluble vitamin A, that is of animal origin found in fish, meat, poultry and milk.
Carotenes are precursors of vitamin A. It is found mostly in green leafy vegetables, yellow
fruits, red palm oil. The carotenes are converted to retinol in the small intestine from where
it is absorbed and transported to liver, which is reservoir of vitamin A. The retinol stored in
liver is released in blood stream as retinol binding protein, hence systemic protein deficiency
may precipitate xerophthalmia if it is not corrected. Retinol besides maintaining health of
epithelial cells of conjunctiva also plays an essential role in night vision. Retinol combines
with protein in rods and act as light stimulus, exciting the optic nerve in scotopic vision.
Vitamin A is involved in photo chemistry of night vision. It is involved in breakdown and
resynthesis of rhodopsin. Night blindness sets in when serum vitamin A level falls below 50
IU/L. This may happen in :
1. Reduced consumption
2. Increased loss or
3. Reduced storage in liver.
4. Deficiency of vitamin A responds quickly to administration of water soluble vitamin
A by intra muscular injection within twenty to twenty four hours.
Clinical features. Clinical features depend upon severity, duration, associated
secondary infection of the eye and systemic infective diseases like pneumonitis, gastro
enteritis, measles etc.
Xerophthalmia has been classified variously
91
1. Prexerosis, 2. Conjunctival xerosis, 3. Corneal xerosis,
4. Keratomalasia, 5. Perforation.
Most commonly used classification is that given by WHO
94
that divides xerophthalmic
signs into two broad groups i.e. primary and secondary signs. Each group has been di-
vided into three sub groups :
Primary signs
96
X 1A Conjunctival xerosis
X 1B Bitots spot with conjunctival xerosis
X 2 Corneal xerosis
X 3A Corneal ulcer with xerosis
3B Keratomalacia
Secondary signs
XN Night blindness
XF Xerophthalmic fundus
XS Xerophthalmia scar
Where X stands for xerosis
Symptoms of xerophthalmia can be related to
1. Conjunctival and corneal
2. Night blindness.
One may precede the other or may be found together. There are instances where the eye
may advance into conjunctival and corneal xerosis without night blindness, vice versa is rare.
Clinical signs
1. ConjunctivalVertical folds on looking laterally
2. Dryness of conjunctiva
3. Pigmentation on dry conjunctiva
4. Bitots spot.
Bitots spot
94
. These are triangular raised white areas developing mostly on the lateral
side of bulbar conjunctiva in the inter palpebral aperture little away from the limbus. The
triangle has its base towards the cornea and apex towards outer canthus. The surface of the
triangle is foamy, the foamy substance can be removed with ease only to reappear. The foam is
caused by action of xerosis bacilli. The surface of Bitots spot does not wet with tear. Kajal
and Surma applied to lid margin do not stain normal conjunctiva but the surface of the Bitots
spot and dry conjunctiva stains with Kajal and Surma. Bitots spot is not specific of vitamin A
deficiency. It can be idiopathic as well. Bitots spot due to vitamin A deficiency disappears
after sometimes but idiopathic Bitots spot does not respond to administration of vitamin A
and is seen in older children and adults.
2. Corneal signs. Cornea develops dry spots, tear film break up time is less than
10 seconds. Tear production and composition of tear is within normal limits, abnormal tear
break up is due to changes in corneal epithelium. The cornea is lusturless. These early changes
are reversible and respond promptly to systemic administration of vitamin A. If the condition
is not arrested at this stage it proceeds to corneal ulceration which is generally central,
surrounded by hazy or opaque cornea. Peculiarity of these ulcers is that they are aseptic in
nature. There is minimal congestion and hardly any pain. The stroma is involved following
break in epithelium. The stroma is involved more easily and severely in vitamin A deficiency
than seen in normal eye when epithelium is damaged. At this stage vigorous local and systemic
treatment may avert perforation but complete recovery with clear cornea is never achieved.
Keratomalacia. This is advanced stage of xerophthalmia. Here the cornea becomes
soft and virtually melts away within twenty to twenty four hours. This has not been pro-
duced in experimental animals. The central part is covered by a white gelatinous material due
to collagenase enzyme. Perforation with protrusion of intraocular tissue is inevitable. Scar
following keratomalacia is generally bilateral, one eye may be more advanced than the other.
In developing countries with eradication of small pox, keratomalacia has become foremost
cause of bilateral corneal staphyloma in children.
Night blindness. Night blindness due to vitamin A deficiency is of acute onset. The
child prefers to sit in one place and avoid moving. The child is so incapacitated that he may not
locate his food in the plate placed in front of him. This has been termed as chicken blind-
ness. Night blindness due to vitamin A deficiency responds to systemic vitamin A therapy
quickly.
Xerophthalmia due to vitamin A deficiency is never isolated. It is invariably associated
with protein caloric malnutrition. It is very common following attack of measles, diar-
rhoea, malaria, worm infection, mal-absorption syndrome.
Management of xerophthalmia
97, 98
1. Prophtlaxis
2. Therapeutics
Prophylaxis
98
. Xerophthalmia is one of leading cause of bilateral blindness in children
which is fully preventable by simple and cost effective methods. Moreover vitamin A defi-
ciency is also responsible for a large number of deaths in infants.
Prophylaxis in mother and new born. Vitamin A is stored in liver and secreted in
mothers milk in sufficient quantity to prevent deficiency in infants. Hence it is of utmost
importance that mother has good storage of vitamin A during pregnancy and lactation. The
colostrum is rich in vitamin A, hence soon after birth the child should be put to breast of the
mother and allowed to take the collustrum. If the child gets sufficient amount of mothers milk
and thrives on it, there is no need of any supplementation of vitamin A. The child should be
breast fed as long as there is sufficient mothers milk for the child. If top feed is to be started
bovine milk is preferred over formula milk. From the age of five months solid food should
be started in consultation with pediatrician, adding green leafy vegetables, yellow fruits and
vegetables to cereals and pulses.
Vitamin A prophtlaxis between 6 months to 6 years. Every child between six months
to six years should get an oral dose of 200,000 unit of vitamin A concentrate. Under National
Programme of Prevention of Xerophthalmia, vitamin A is distributed free from primary health
centres, government hospitals. The first dose may be delayed up to nine months and given
along with measles vaccine. It is not clear if vitamin A deficiency precipitates attack of mea-
sles or vice a versa. However administration of vitamin A along with management of measles
has reduced mortality in measles.
Fortification of food with naturally occurring vitamin A. Children and young
adults should be encouraged to take vitamin rich food as part of daily diet in the form of green
vegetables, yellow tubers, yellow fruits.
Daily requirement of vitamin in normal individual :
1. Pregnancy and lactation3000-3500 IU (300 mg to 750 mg)
2. 0-4 years1000 to 1200 IU
3. School children2250 IU
Management of clinical xerophthalmia :
Night blindness
99
. Intra muscular injection of water soluble 100,000 IU of vitamin A
followed by (1) 200,000 IU oral after seven days or (2) Repeat 100,000 IU intra muscular after
seven days.
Xerosis200,000 IU of oral vitamin.
Keratomalacia
WHO recommendation :
1. Immediate upon diagnosis - 200,000 IU orally.
2. Next day200,000 IU orally
3. After seven days200,000 IU orally.
4. Repeat 200,000 IU every six months up to six years of age to prevent recurrence.
5. If the child is unable to take oral feed or has vomiting, 100,000 IU of water soluble
vitamin A is given as injection.
Children less than 8 kg should receive half the above dose and under 4 kg quarter of the
above dose.
Ocular therapy
A. Local instillation of antibiotic drops every 2 hourly and ointment during sleep to
prevent sticking of lids.
B. AtropineIn all cases of corneal involvement irrespective of actual ulceration atro-
pine is used as 1% ointment two times a day. Atropine as drop is better avoided in
children for one drop of atropine contains more than therapeutic dose of atropine.
Thus one drop in each eye reaches almost lower level of toxicity especially when the
child is under weight.
C. Local retinolTretinoin 0.1% as drops in oil three times a day is claimed to retard
progress of corneal involvement.
D. Local tear substitutesThough aqueous part of tear film is not reduced yet it fails to
wet the cornea and conjunctiva due to changes in epithelium. Local artificial tear
may help in preventing the cornea from drying.
The childs total protein and calories should be calculated and supplied because vitamin
A deficiency does not occur in isolation. There should be adequate supply of other vitamins and
minerals specially the trace elements.
Other causes of xerosis. Coloboma of lids, ectropion of lid, lagophthalmos, proptosis,
Steven-Johnsons syndrome, Riley Day syndrome, acid alkali burn.
REFERENCE8
1. Nema H.V. : The cornea and limbus in Anatomy of the eye and its adnexa. Second
edition, p-3-9, Jay Pee Brothers, New Delhi, 1991.
2. Doughman D. : Corneal physiology in Principle and practice of ophthalmology.
Edited by Pyeman G.A., Sander D.R. and Goldberg M.F. First Indian edition. p-360.368.
Jay Pee Brothers, New Delhi, 1987.
3. Meharotra A.S. : Applied anatomy of the eye and adnexa in Modern ophthalmology.
Second volume. 1st edition. p-105-1056. Edited by Dutta L.C., Jay Pee Brothers, New
Delhi, 2000.
4. Ilene K. Gipson : Anatomy of the conjunctiva, cornea and limbus in The cornea. Third
edition p-3-25. Edited by Smolin G. and Thoft R.A., Lippincot Williams and Wilkins,
Philadelphia, 1994.
5. Deborah Pavan Langston : Cornea and external eye disease in Manual of ocular diag-
nosis and therapy. Fifth edition 67-68. Lippincott Williams and Wilkins, Philadel-
phia, 2002.
6. Duke Elder S. : System of ophthalmology. Vol. III, Part I. First edition. p-137, Henry
7. Nancy Anderson Hamming and Apple D. : Anatomy and embryology in Principle and
practice of ophthalmology. First Indian edition, edited by Pyeman G.A., Sanders
D.R. and Goldberg M.F. p-18-19, Jay Pee Brothers, New Delhi, 1987.
8. Banumathy S.P. : Development of cornea and sclera in Anatomy of the eye. p-165,
Arvind Eye Hospital, Madurai.
9. Nema H.V. : Development of eye and its adnexa in Anatomy of the eye and its adnexa.
Second edition. p-129, Jay Pee Brothers, New Delhi, 1991.
10. Duke Elder S. : System of ophthalmology. Vol. III, Part II. p-164-169, Henry Kimpton,
London, 1963.
11. Basak S.K. : Physiology of the eye in Essentials of ophthalmology. Second edition.
p-24-25, Current Books International, Calcutta, 1999.
12. Khurana A.K. : Diseases of the cornea in Ophthalmology. Second edition. p-114-115,
New Age International, New Delhi, 2000.
13. Sharma P. : Cornea and its diseases in Essentials of ophthalmology. First edition.
Modern Publishers, New Delhi, 2000.
14. Ramanjeet Shihota and Radhika Tandon : Metabolism of ocular tissues in Parsons
diseases of the eye. Nineteenth edition, p-20-21, Butterworth Heineman, Oxford, 2003.
15. Edelhauser, H.F., Geroski D.H. and Ubels J.L. : Corneal transparency in The Cornea.
Third edition, p-28-32. Edited by Smolin G. and Thoft R.A., Lippincot Williams and
Wilkins, 1994.
16. Wilson F.M. : Congenital anomalies of cornea and conjunctiva in The Cornea. Third
edition, edited by Smolin G. and Thoft R.A. p-535-550, Lippincot Williams and Wilkins,
Philadelphia, 1994.
17. Duke Elder S. : Symptom of ophthalmology. Vol. III, part II. Edited by Duke Elder S.
p-497-525, Henry Kimpton, London, 1964.
18. Nema H.V. : Anomalies of cornea in Anatomy of the eye and its adnexa. Second
edition. p-145-146, Jay Pee Brothers, New Delhi, 1991.
19. Rabinowitch V.S. and Rasheed K. : Pellucid marginal degeneration in Current ocular
therapy. Fifth edition. Edited by Fraunfelder F.T., Roy F.H. p-371-372, W.B. Saunders
Company, Philadelphia, 2000.
20. Kanski J.J. : Pellucid marginal degeneration in Clinical ophthalmology. Second edi-
tion p-119, Butter Worth, London, 1987.
21. Kennedy R.H., Bourne W.M. and Kyer J.A. : A 48 year clinical and epidemiologic study
of keratoconus. Am.Jr.Oph. : 101, 267-273, 1986.
22. Nesburn A.B. and Kenney M.C. : Keratoconus in Current ocular therapy. edition 5.
Edited by Fraunfelder F.T. and Roy F.H. p-364-365, W.B. Saunders Company, Philadel-
phia, 2000.
23. Nagy K.A. and Abel R. : Keratoconus in Current ocular therapy. Edition 4. Edited by
Fraunfelder F.H. and Roy F.H. p-501-502, W.B. Saunders Company, Philadelphia, 1995.
24. Duke Elder S. : System of ophthalmology. Vol. III, Part II. First edition. p-964-974,
Henry Kimpton, London, 1964.
25. Duke Elder S. : System of ophthalmology. Vol. III, Part II. First edition. p-509, Henry
26. Raber I. : Keratoconus in Ophthalmic secrets. Edited by Vander J.E. and Gault J.A.
First Indian edition p-95-101, Jay Pee Brothers, New Delhi, 1998.
27. Samolin G. : Corneal dystrophies and degeneration in Cornea. Third edition. Edited by
Samolin G. and Thoft R.A. p-522-524, Lippincot Williams and Wilkins, Philadelphia,
1994.
28. Rizutti A.B. : Diagnostic illumination test for keratoconus. Am. Jr. Oph. 70 : 141, 1970.
29. Tuft S.J., Moodaley L.C. and Gregory W.M. : Prognostic factors for progression of
keratoconus. Ophthalmology 1012 : 439, 1994.
30. Stone Chiper K.G. : Corneal neovascularisation in Current ocular therapy. Fourth
edition. Edited by Fraunfelder F.T. and Roy F.H. p-484-485, W.B. Saunders Company,
Philadelphia, 1995.
31. Duke Elder S. : System of ophthalmology. Vol. VIII, Part II. p-676-691, Henry Kimpton,
1964.
32. Sridhar Rao B. : Congenital glaucoma in Modern ophthalmology. Part I, Second edi-
tion. p-454, Edited by Dutta L.C., Jay Pee Brothers, New Delhi, 2000.
33. Kanski J.J. : Congenital glaucoma in Clinical ophthalmology. Second edition. p-223-
224, Butterworth, London, 1989.
34. Shields M.B. : Congenital glaucoma in Text Book of glaucoma. Fourth edition. p-198,
Williams and Wilkins, Philadelphia, 1998.
35. Duke Elder S. : Inflammation of the cornea in System of ophthalmology. Vol. VIII,
Part II. Edited by Duke Elder S. and Leigh A.B. p-729-860, Henry Kimpton, London,
1967.
36. Vastine David : Corneal ulceration : Classification, signs and symptoms in Principle
and practice of ophthalmology. Vol. I, p-285-294, Edited by Peyman G.A., Sander
D.R. and Goldberg M.F. 1st Indian edition, Jay Pee Brothers, New Delhi, 1987.
37. Dhanda R.P. and Kalever V. : Infected corneal ulcer in A Text Book of Ophthalmol-
ogy. New edition p-207-216, Galgotia Publication Pvt. Ltd., New Delhi, 1993.
38. Reddy M., Savitri Sharma and Rao G.N. : Corneal ulcer in Modern ophthalmology.
Vol. I. Second edition. Edited by Dutta L.C. p-200-214, Jay Pee Brothers, New Delhi,
2000.
39. Coster D.J. and Badenoch P.R. : Bacterial corneal ulcer in Current ocular therapy.
Fifth edition. Edited by Fraunfelder F.T. and Roy H.F. p-345-346, W.B. Saunders Com-
pany, Philadelphia, 2000.
40. ODay D.M. : Fungal keratitis in Current ocular therapy. Fifth edition, edited by
Faunfelder F.T. and Roy H.F. p-360-361, W.B. Saunders and Company, Philadelphia,
2000.
41. Roberson M.C. : Fungal keratitis in Current ocular therapy. Fourth edition, Edited
by Fraunfelder F.T. and Roy F.H. p-492-493, W.B. Saunders Company, Philadelphia,
1995.
42. ODay D.M. and Eileen M : Burd Fungal keratitis and conjunctivitis in The Cornea.
Third edition. Edited by Smolin G. and Thoft RA. p-229-252, Lippincot William and
Wilkins, Philadelphia, 1994.
43. Yee R.W. and Cheng C.J. : Central corneal ulcer in Decision making in ophthalmol-
ogy. First Indian edition. Edited by Heuven WAJ and Zwaan J.T. p-154-155, Harcort
Brace, Singapore, 1998.
44. Liegner J.T. and Yee R.W. : Marginal corneal ulcer in Decision making in ophthal-
mology. First Indian edition. Edited by Heuven WAJ and Zwaan J.T. p-152-153, Harcort
Brace, Singapore, 1998.
45. Duke Elder S. : Corneal scar in System of ophthalmology. Vol. 8, Part II. p-639,
Edited by Duke Elder S. and Leigh A.G, Henry Kimpton, London, 1965.
46. Dhanda R.P. and Kalever V. : Corneal scars in A Text Book of Ophthalmology. New
First edition. p-258-264, Galgotia Publications Pvt. Ltd., New Delhi, 1993.
47. Ostler H.B., Whitcher J.P. and Cevallos V. : Pneumococcus in Current ocular therapy.
Fifth edition, edited by Fraunfelder F.T. and Roy F.H. p-58-59, W.B. Saunders Com-
48. Duke Elder S. : Central corneal ulcer in System of Ophthalmology. Vol. VIII, Part II.
Edited by Duke Elder S. and Leigh A.G. p-777-800, Henry Kimpton, London, 1965.
49. Wilkins J. Whitcher J.P. and Margolis T.P. : Penicillin resistant streptococcus pneumonae
keratitis. Cornea 15 : 99-100. 1996.
50. OBrien T.P. : Bacterial keratitis study research group. Efficacy of ofloxacin vs. cifazolin
and tobramycin in Bacterial keratitis. Arch. Oph. 113:1257-1265, 1995.
51. Laibson P.R. : Pseudomonas aeruginosa : In Current ocular therapy. Fifth edition.
p-61-63, Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Philadel-
phia, 2000.
52. Gittinger J.W. : Herpes simplex keratitis in Manual of clinical problems in oph-
thalmology. First edition. Edited by Gittinger J.W. and Asdourian G.K. p-46-47, Little
Brown and Company, Boston, 1988.
53. Deborah Pavan Langston : Herpes simplex virus kerato conjunctivitis and iritis in
Manual of ocular diagnosis and therapy. Fifth edition. p-89-92, Lippincot Williams
and Wilkins, Philadelphia, 2002.
54. Deborah Pavan Langston : Herpetic infection in The Cornea. Third edition. p-183-208,
Edited by Smolin G. Thoft R.A., Lippincot Williams and Wilkins, Philadelphia, 1994.
55. Mudgal P.C. : Antiviral agents in Diagnosis and management of ocular
inflammations. Second edition, p-54-62, Edited by Jain M.R., Jay Pee Brothers, New
Delhi, 1990.
56. Missotten L. : Management of herpes simplex keratitis in Diagnosis and manage-
ment of ocular inflammation. Second edition. p-63-65, Edited by Jain M.R. Jay Pee
57. Malouf D.J. and Oates R.K. : Herpes simplex virus infection in neonate. Jr. Op. Paed.
and Clin. health. 31:332-335, 1995.
58. Athmanathan S., Garg P. and Rao Gullapalli N. : Ophthalmic antiviral chemotherapy.
An overview. Ind. Jr. Oph. 45 : 203-210, 1997.
59. Kanski J.J. : Dendriticular in Clinical ophthalmology. Second edition. p-99-101,
Butterworth, London, 1989.
60. Laibson P.R. and Waring G.P. : Diseases of the Cornea in Paediatric Ophthalmology.
Vol. I, p-490-498, Edited by Harley R.D., W.B. Saunders Company, Philadelphia, 1983.
61. Suresh P.S. and Tullo A.B. : Herpes Simplex Keratitis. Ind. Jr. Oph. 47 : 155-165, 1999.
62. Gorden J.S. : Adenoviral ocular infection in The Cornea. Third edition. p-215-225,
Edited by Samolin G. and Thoft. P.A., Lippincot Williams and Wilkins, Philadelphia,
1994.
63. Forster R.K. : Fungal keratitis and conjunctivitis in The Cornea. Third edition. p-239-
251, Edited by Samolin G. and Thoft R.A., Lippincot Williams and Wilkins, Philadel-
phia, 1994.
64. ODay D.M. : Fungal keratitis in Current ocular therapy. Fifth edition. p-360-361,
Edited by Fraunfelder F.T. and Roy H.F., W.B. Saunders Company, Philadelphia, 2000.
65. Savitri Sharma, Silverberg M., Mehta P., Usha Gopinath, Agrawal W. and Naduvilath
T.J. : Early diagnosis of mycotic keratitis predictive value of potassium hydroxide prepa-
ration. Ind. Jr. Oph. 31-35, 1998.
66. Thomas P.A., Kuriacose T., Kirupa Shankar M.P. and Maharajan V.S. : Use of lactophenol
cotton blue mount of corneal scrapping as an aid to the diagnosis of mycotic keratitis.
Diag. microbio. 14 : 219-224, 1991.
67. Arffa R.C., Avni I. Aschibashiy, Robin J. and Kaufmann H.E. : Calco flurol ink potas-
sium hydroxide preparation for identifying fungi. Am. Jr. Oph. 100: 719-723, 1985.
68. Vajpayce R.B. Angra S.K., Sandramoulis, Honavar S.G. and Chabra V.K. : Laboratory
diagnosis of keratomycosis, comparative evaluation of direct microscopy and culture
results. Annl. Oph. 25 : 68-71, 1993.
69. Jain M.R. : Keratomycosis and antifungal drugs in Diagnosis and management of
ocular inflammation. Second edition. p-28-32, Jay Pee Brothers, New Delhi, 1990.
70. Ram J. and Pandey S.K. : Peripheral corneal ulcers in Modern Ophthalmology. Vol.
I. Second edition, p-238-241, Jay Pee Brothers, New Delhi, 2000.
71. Duke Elder S. : System of ophthalmology. Vol. VIII, Part II. Edited by Duke Elder S.
and Leigh A.G. p-815-830, Henry Kimpton, London, 1965.
72. Ogawa G.S.H. and Hyndiuk R.A. : Non ulcerative keratitis in The Cornea. Third edi-
tion p-144-149, Edited by Smolin G. and Thoft R.A. Lippincot Williams and Wilkins,
Philadelphia, 1994.
73. Shalaby A. and Dunn J.P. : Acquired and congenital syphilis in Current ocular therapy.
Fifth edition. p-2-5, Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company,
Philadelphia, 2000.
74. Margo C.E. and Hamed L.M. : Ocular syphilis. Surv. Ophth. 37 : 207-220, 1992.
75. Wilhelmus K.R. Acanthamoeba keratitis in The Cornea. Third edition. p-262-266, Ed-
ited by Smolin G. and Thoft R.A., Lippincot Williams and Wilkins, Philadelphia, 1994.
76. Stuphin J.E. : Acanthamebiasis in Current ocular therapy. Fifth edition, p-89-90,
Edited by Fraunfelder F.T. and Roy F.H., Philadelphia, 2000.
77. Singh S. and Sachdeva M.P.S. : Acanthameoba keratitis. B.M.J. 309:273, 1994.
78. Sharma S., Srinivasan M., and George C. : Diagnosis of acanthamoeba keratitis. Ind.
Jr. Oph. 38:50-56, 1990.
79. Dart J.K.G. : Clinical features of acanthamoeba keratitis. Community eye health. 8 : 1,
1995.
79.A. Seal S.K. : Acanth amoeba keratitis in G.N. Seals Textbook of ophthalmology 5th
Ed. p-162-163, current book int Kolkata. 2002.
80. Barua C.K. and Dutta L.C. : Corneal degenerations and dystrophies in Modern Oph-
thalmology. Vol. I, second edition. 193-199, Edited by Dutta L.C., Jay Pee Brothers,
New Delhi, 2000.
81. Duke Elder S. : Corneal dystrophies in System of ophthalmology. Vol. 8, Part II,
p-921-932, Edited by Duke Elder S. and Leigh A.G. Henry, Kimpton, London, 1965.
82. Laibson P.R. and Waring G.O. : Corneal dystrophies and degeneration in Pediatric
Ophthalmology. Vol. I, Second edition. p-502-506, Edited by Harley R.D., W.B. Saunders
83. Samolin G. : Corneal dystrophies and degeneration in The Cornea. Third edition.
p-499-508, Edited by Samolin G. and Thoft R.A., Lippincot Williams and Wilkins, Phila-
delphia, 1994.
84. Duke Elder S. : Band shaped keratopathy in System of ophthalmology. Vol. VIII,
Part II. p-893-902, Edited by Duke Elder S. and Leigh A.G., Henry Kimpton, London,
1965.
85. Doughman D. : The cornea in Principle and practice of ophthalmology. Vol. I. First
Indian edition. p-414-419, Edited by Peyman G.A. Sander D.R. and Goldberg M.P., Jay
Pee Brothers, New Delhi, 1987.
86. Tran D.M. and Schanzlin D.J. : Corneal and conjunctival calcification in Current ocu-
lar therapy. Fifth edition. p-329-330, W.B. Saunders Company, Philadelphia, 2000.
87. O,Brart D.P.S. and Gartry D.S. : Treatment of band keratopathy by excimer laser photo
therapeutic keratectomy. B.J.O. 77 : 702-708, 1993.
88. Peterson R.A. and Boger W.D. : Pediatric ophthalmology in Manual of ocular diagno-
sis and therapy. Fifth edition. p-288, Edited by Deborah Pavan Langston. Lippincot,
Williams and Wilkins, Philadelphia 2002.
89. Liabson P.R. and Waring G.O. : Diseases of the cornea in Pediatric ophthalmology.
Vol. I Edition II. p-456-490, Edited by Harley R.D., W.B. Saunders Company, Philadel-
phia, 1983.
90. Zwaan J.J. : Cloudy cornea in a neonate in Decision making in ophthalmology.
First Indian edition. p-128-129, Edited by Vantteuven W.AJ and Zwaan J.T., Harcort
Brace, 1992.
91. Duke Elder S. : System of ophthalmology. Vol. VIII. Part 2. p-1115-1125, Edited by
Duke Elder S., Leigh A.G. Henry Kimpton, London, 1965.
92. Sommer A. : Xerophthalmia and keratomalacia in Nutritional Blindness. Oxford Uni-
versity Press, 1982.
93. Kenyon K.R. and Chaves H.V. : Morphological and pathological response of cornea and
conjunctiva diseases in The Cornea. Third edition. p-71-78, Edited by Smolin G., Thoft
R.A. Lippincot Williams and Wilkins, Philadelphia, 1994.
94. WHO : Vitamin A deficiency and xerophthalmia. Technical report No. 590, 1976.
95. Vaughan D. and Asbury T. : Corneal ulcer due to vitamin A deficiency in General
Ophthalmology. Tenth edition. p-97, Lange Medical Publication, California, 1983.
96. McLaren D.S. and Frigg M. : Xerophthalmia in Sight and life manual of vitamin A
deficiency. Second edition. p-51-62, Published by Task Force Sight and Life, Basel,
2001.
97. Sommer A. : Impact of vitamin A supplemention of childhood mortality. Lancet 1:1169-
173.
98. Sharma P. : Xerophthalmia in Essentials of ophthalmology. First edition. p-114-116,
99. Sommer A. : Hypovitaminus A in Current ocular therapy. Fifth edition. p-107-108,
Edited by Fraunfelder F.T. and Roy. H.F., W.B. Saunders Company, Philadelphia, 2000.
Uvea is the middle vascular pigmented coat of the eye ball. It is divided into three
parts.
1
1. Iris 2. Ciliary body 3. Choroid. All the three parts develop from neuro ectoderm
and mesoderm.
The iris
Iris is the most anterior part of the uvea that is visible on oblique illumination. It is
practically a diaphragm of blood vessels and unstripped muscle fibres, held together by loose
stroma. It is coronal to the cornea but not parallel to it. It is 12 mm. in diameter, from root to
root, all round with a circular hole in the center the pupil. Thickness of the iris is not uniform.
It is thinnest at its insertion (root) to the middle of the anterior border of the ciliary body. Its
thickness is maximum at the collarette, then its thickness gradually diminishes towards the
pupil in a rounded fashion. The iris divides the aqueous chamber into anterior and posterior
chambers. The colour of the iris not only varies from race to race, but also from person to
person. It may vary in two eyes or in the same eye. Colour of the iris is due to the presence of
pigments in the iris. More the pigment, darker is the iris.
Colour of the iris of the new born is lighter than that of adults. Some of the stromal
vessels are visible in the iris of the new born, which disappear after a few weeks.
Difference in the colour of the iris in two eyes is called heterochromia. Difference in
colour of one iris from its counterpart is called heterochromia iridum while difference in
colour of iris in the same eye is called heterochromia iridis.
2
Heterochromia is generally congenital in nature. In albinism the iris is generally blu-
ish, almost translucent that transilluminates with reflected light.
The anterior surface of the iris is rough. It is divided into two zones by a circular raised
irregular lines called the collarette i.e.
1. Pupillary zone that is 1.5 mm. wide from the collarette to pupillary margin, is lined
by a thin zone of pigment called iris ruff, which is in fact continuation of the poste-
rior pigment layer. The pupillary zone contains the sphincter pupillae muscle that
is a smooth muscle and originates from the neuroectoderm and is supplied by
parasympathetic nerve.
2. The ciliary zone is a wider zone that extends from collarette to the root of the iris.
It is relatively rough due to the presence of series of radial elevated ridges that
227
CHAPTER 8
Disorders of the Uvea in Children
C-8\D:\N-AGE\NE-C8.PM5
represents radial blood vessels in the stroma. Besides the ridges there are scattered
depressions that are formed due to lack of superficial layers of the iris. The peripheral
crypts communicate with the anterior chamber.
3
As the root of the iris is the thinnest
part of iris, it is most liable to be torn following a blunt injury without bleeding. The
detachment of the iris from the root is called iridodialysis.
The posterior surface though not very smooth, is flatter than the anterior surface. The
posterior surface rests on the anterior surface of intact lens and glides smoothly over the lens
capsule. The posterior surface is dark brown or black due to the pigment in the epithelium.
Microscopically the iris is divided into:
1. Endothelium. 2. Stroma. 3. Posterior epithelium.
1. The endothelium. The presence of endothelium is controversial
4,5
. Traditionally it
was thought to be an extension of the cells that line the posterior layer of cornea. Now it is
presumed to be distributed in patches. The endothelial layer is absent over the crypts.
2. The stroma has been divided into:
(i) Anterior limiting layer, (ii) Stroma proper.
(i) Anterior limiting layer. The Anterior limiting layer is a modification of super-
ficial layer of stroma
1
. Some authors consider that what was thought to be endothelium of the
iris is in fact a layer of condensation of anteriors limiting layer.
5
This layer consists of
melanocytes, fibroblasts and collagen fibres. Amount of pigment and the thickness of
this layer is responsible for the colour of the iris. In blue coloured iris there is less pigment and
in dark coloured iris there is more pigment in this layer. Nerve endings and capillaries are
present in this layer. This is the place where neovascularisation of iris begins.
(ii) Stroma proper. Stroma proper consists of blood vessels, nerves, unstratified mus-
cles, fibroblasts, lymphocytes and mast cells. The blood vessels arise from the greater circle
of the iris that is in fact situated in the ciliary body in a radiating fashion towards the collarettes
where they anastomose to form the lesser circle of the iris. The vessels in the iris are non-
fenestrated, hence not suitable for ordinary fluorescein angiography. The nerves present in
the stroma arebranches of trigeminal, oculomotor and sympathetic.
The unstratified muscles are:
1. The sphincter pupillae is present in the pupillary zone, it is a narrow strip of about
1 mm. width that encircles the papillary border, it comes in close proximity of the
posterior pigment epithelium. The function of the muscle is to constrict the pupil.
The pupil continues to constrict even in presence of iridectomy. It is supplied by the
parasympathetic fibres of the third nerve.
2. The dilator pupillae lies in the ciliary zone of the iris. Direction of the fibres in this
zone is radial, it is more bulky than the sphincter, it lies in the posterior part of the
stroma. Its function is to dilate the pupil, it is innervated by cervical sympathetic
nerve. The dilator pupillae arises from the anterior epithelial layer.
3. Posterior epithelium. It is two layered.
A part of it represents outer wall of the optic cup. It is the forward extension of pig-
ment epithelium of retina and ciliary body and is commonly referred to anterior epithelium.
DISORDERS OF THE UVEA IN CHILDREN 229
The posterior epithelium is the anterior extension of non pigmented epithelium of cili-
ary body and merges with the anterior epithelial layer.
The pigment epithelium acts as blood aqueous barrier besides preventing light from
entering the eye.
The pupil
The pupil is not a tissue, it is a deficiency in the iris. It is circular in shape, situated in
the center and slightly nasally in the iris. The periphery of the pupil is black in colour due to
posterior pigment epithelium of the iris spilling anteriorly. This is called ruff of the iris. The
size of a normal adult pupil is 2 mm. to 4 mm. It is smaller in new born and in old age. Pupil
smaller than normal is called miotic pupil, while larger than the normal is called mydriatic
pupil. The drugs that constrict the pupil are called miotics, while those that dilate are called
mydriatics. Generally the size of the pupil is equal in the two eyes. Difference in the sizes of
the pupil in two eyes is called anisocoria. Presence of more than one pupil in each eye is
called polycoria, which is a congenital condition. Pupil in true polycoria should have inde-
pendent sphincter and dilator muscles. Pseudopolycoria can be seen as congenital anomaly
i.e. dysgenesis of anterior chamber or may be acquired following trauma which is mostly sur-
gical or may be accidental. In some chronic uveitis, there may be loss of iris tissue resulting in
holes in the iris. A deficiency in iris is called coloboma that can be congenital or acquired.
It is very common for the coloboma of the iris to extend up to pupil resulting in a vertically
enlarged key hole shaped pupil. Such pupil are capable of reacting to light and accommodation
to a limited extent. Corectopia is a term used to denote the position of the pupil other than
normal, this is generally bilateral and congenital.
Normally the pupil is never stationary and its size keeps on changing within a normal
range, with change of intensity of light and accommodation. An appreciable flicker of pupil is
called hippus, which is of no clinical significance.
The function of the pupil is to control the light entering the eye. It acts as communica-
tion between anterior and posterior chamber through which aqueous passes from the poste-
rior chamber to the anterior chamber.
The ciliary body
This is the middle part of the uvea. It extends from scleral spur anteriorly, to ora
serrata posteriorly. It encircles the interior of the sclera. In section it looks like an isosceles
triangle, the base of which points towards the pupil. The outer edge of the base is attached to
the scleral spur, 1.5 mm. away from the limbus. From the middle of the base arises the iris
dividing the base into two parts, the anterior part forming the angle of the anterior chamber.
This part is visible on gonioscopy only. The posterior part forms the boundary of the posterior
chamber.
The ciliary body is highly vascular and bleeds profusely on trauma, both blunt and
penetrating.
Its colour is brownish black. The ciliary body is empirically divided into two parts:
1. Pars plicata and 2. Pars plana.
1. Pars plicata. The Pars plicata is anterior 2 mm. of the ciliary body. This is the
thickest part of the uvea. It comprises of ciliary stroma, ciliary muscles, blood vessels
and nerves. The inner surface of para plicata is divided into 70 to 80 ridges or plications.
These are known as ciliary processes, they extend towards the lens, in finger like processes.
Each of them is 0.5 mm. x 2.00 mm. in size. From the tips of these processes arise the zonules
of the lens. The lens and the zonules together form a barrier that separates the vitreous
chamber from the aqueous chamber. The ciliary processes are made up of capillaries covered
by two layers of ciliary epithelium. They do not have any stroma or muscles, their length
depends on tightness of the ciliary muscles
3
. Besides acting as attachment of zonules, that are
responsible for accommodation, the other function of the ciliary process is to secrete aque-
ous.
The ciliary epithelium acts a blood aqueous barrier. The non-pigment epithelium
secretes aqueous while the pigment epithelium continues as retinal pigment epithelium.
2. Pars plana. The pars plana is posterior 6 to 7 mm. of the ciliary body. This gradually
looses its musculature and tappers as a thin layer to end in ora serrata that ultimately blends
with the choroid and the retina. The vascular layer of the pars plana is similar to that of
choroid without choriocapillaries. The vitreous base extends from retinal periphery up to 2
mm. of pars plana. The pars plana is thought to secrete mucopolysaccharides of the vitreous.
6
The pars plana being less vascular than any other part of the uvea with minimum thickness is
the natural choice of entry for intra vitrial surgery and lensectomy.
The ciliary muscles form the main bulk of the ciliary body. The muscles are non-
striated muscles that has three parts. The most important are the longitudinal fibres. The
middle part is formed by the radial fibres. While the innermost are the circular fibres. Most of
the bulk of the ciliary muscle is located in the anterior two-third of the ciliary body. The exact
function of the ciliary muscles are not well understood, however, the ciliary muscles take a
major part in accommodation. Contraction of the ciliary muscles open up the angle of the
anterior chamber to facilitate aqueous out flow.
The nerve supply of the ciliary body is mostly by parasympathetic nerve. Sympathetic
supply has lesser role to play in the ciliary body. The sensory supply is via trigeminal.
The choroid
The choroid is the posterior most part of the uvea and forms a major part of the middle
layer of the coats of the eyeball. It extends from optic nerve to the ora serrata between the
sclera on the outer side and retina on the inner side. It is continuous with ora serrata anteriorly
with loose attachment to the 1. The sclera, 2. The exit of the vortex veins near the equator, 3.
round the disc at posteriorly. Its thickness is not uniform throughout, at the posterior pole it is
0.25 mm. and anteriorly 0.1 mm. in thickness. Its attachment to most of the sclera is loose.
There is a potential space in between the sclera and the choroid called suprachoroidal space
that is liable to distended by fluid. The separation is more pronounced anteriorly resulting in
a ciliochoroidal detachment following trauma and inflammation. The choroid can not be
separated at the exit of the vertex veins and round the disc due to firm attachment. The
suprachoroidal space extends anteriorly under the ciliary body as supraciliary space. This
space is traversed by ciliary vessels and nerves. The ciliary vessels do not give any branch in
the suprachoroidal space but the ciliary nerves give fine branches to the outer layer of the
choroid.
Histologically the choroid is divided into:
1. Supera choroidal lamina
2. Vascular layer
3. Bruchs membrane.
1. Suprachoroidal lamina. The suprachoroidal lamina contains melanocytes, fibrocytes
and thin fibres. This layer is attached to the choroid on its outer surface, and is attached to the
inner surface of the sclera.
2. The vascular layer forms the main bulk of the choroid. It consists of three layers of
blood vessels i.e.
(i) The layer of large vessel
(ii) The layer of small vessels
(iii) The choriocapillaries.
The size of the blood vessels diminish in size from outer surface to the inner surface.
The outer layer is mostly venous in nature. The veins unite to form vortex veins which are
four in numbers, one in each quadrant. The lower vortex veins are formed by union of choroidal
vessels 3 mm behind the equator. Each vortex system unites to form an ampula that drains
obliquely through a scleral canal. All the vortex veins drain into ophthalmic veins, there are no
valves in choroidal veins
7
. The spaces in between the vessel is occupied by strands of stroma,
melanocytes and fibrocytes like iris and ciliary body. There is no muscle in the choroids.
Choriocapillaries. The choriocapillaries are the largest capillaries in the body. They
lie in between the layers of choroidal vessels and Bruchs membrane. They are channels lined
by endothelium in which the choroidal arterioles end. The endothelium is fenestrated in
contrast to iris vessels which are non-fenestrated. The choriocapillaries do not anastamose
freely, they supply a globular area rather a sector. The choriocapillaries end at ora and rest of
the vessels continue in ciliary body. The choriocapillaries are denser and relatively larger
under the macula. The main function of the choriocapillaries is to supply nutrients to the outer
part of the retina.
3. Bruchis membrane. The Bruchis membrane is an acellular thin structure that
develops from neuro ectoderm as well as uvea. It is thickest around the optic disc. In high
magnification, it has five layers. The Bruchis membrane forms the blood retinal barrier
and acts a filter for metabolic exchange between choriocapillaries and pigment epithelium of
the retina
8
.
The functions of the choroids is to supply blood to the outer retina. Other functions are
to keep the interior of the globe dark, to regulate the temperature of the eye and enhance the
outflow of aqueous through uveoscleral channel. The choroid does not have any motor func-
tion. It is not influenced by parasympathetic nerve. Sympathetic nerve supply regulates
choroidal circulation. The choroid is said to act like a lymph node following inflammation.
Blood supply of uvea
4, 7
Uvea is a vascular structure, vascularity is maximum in the choroid and least in the
iris. The blood supply to the eye ball is from the ophthalmic artery which gives two
independent system of blood supply. The retinal system and the ciliary system, the two
generally do not anastomose. The former supplies the inner layer of the retina while the latter
supplies the outer layer of the retina, uvea and extra ocular muscles. The ciliary circulation
consists ofa posterior ciliary system consisting of long and short posterior ciliary
arteries and complex of anterior ciliary arteries.
The long posterior ciliary arteries are two in number. They arise from the opthalmic
artery in the orbit, either by a common trunk or two separate trunks. These vessels travel
forward to reach the posterior part of the globe. They pierce the sclera one on the lateral side
and other on the medial side of the optic nerve in the horizontal plane obliquely to enter the
suprachoroidal space. They do not give any branch before reaching the posterior part of the
ciliary body. They divide into two branches on entering the ciliary body. The two branches give
off multiple smaller twigs, most of which go into the substance of the ciliary body to anastomose
with the seven anterior ciliary arteries to form the major arterial circle of the iris that
lies just behind the root of the iris in the ciliary body. One of the branches from the main artery
passes backward to from the recurrent branch of choroids that supplies the choroid between
the oraserrata and the equator.
The posterior ciliary arteries : They also arise from the ophthalmic artery in the
orbit as a group of 6 to 8 vessels that divide to form 20 smaller vessels, which travel forward
encircling the optic nerve all around. They are more in number on the lateral side to give more
blood to the macula. On reaching the choroid these vessels branch profusely. There are two
type of vessels in the choroid, a group of shorter vessels that branch and supply the choroid
near their entry into the sclera and a group of longer vessels that travel up to the equator to
supply the anterior choroid.
The anterior ciliary vessels are continuation of muscular arteries that supply the
four recti two for each except the lateral rectus that has only one artery. They also give
some branches that are not involved with uveal circulation before perforating the sclera near
the limbus and pass through the supraciliary space to end in anterior part of the ciliary body.
At this level, they anastomose with the tiny long posterior ciliary arteries to form the major
arterial circle. 10 to 12 recurrent branches go backward to supply the anterior choroid.
The iris gets its blood supply from the major circle of iris that also supplies part of
the ciliary body and by minor circle of iris that lies just inside the pupillary border and is
formed by centripetal branches from major circle of the iris.
Venous drainage of uvea is mostly by vortex veins. The choroid drains via vortex
veins exclusively. The iris and ciliary body have two systems of venous drainage-1. A well
developed vortex system and 2. A poorly developed ciliary system. The anterior ciliary venous
drainage is more developed than the posterior venous drainage.
There are no formed lymphatics in the uvea.
Development of the uvea
9, 10, 11
The uvea as a whole has bipartite development. The choroid is dominantly mesodermal
except the Bruchs membrane which is partly mesodermal and partly ectodermal. The iris and
the ciliary body are mostly ectodermal in origin except the stroma and the blood vessels.
The ectodermal structure in the iris are the two layers of epithelium, the sphincter, the
dilator pupillae and melanocytes.
The epithelium of the ciliary body and the ciliary processes are also ectodermal in origin.
The mesodermal structures in iris and ciliary body arethe iris stroma, blood vessels
and ciliary muscles.
The iris and the ciliary body develop from the anterior lip of the optic cup. The
edges of the optic cup grow in front of the lens as a double row of epithelium behind the
mesoderm. The two layers of the iris epithelium become pigmented in ciliary body. The non-
pigmented ciliary epithelium is thrown into folds in which the vessels develop to form the
ciliary process. The sphincter muscles starts developing earlier than the dilator, they develops
from the non-pigmented iris epithelium surrounding the pupil. The dilator muscles develop
from the non pigmented layer near the root.
The mesoderm that lies in front of the developing iris epithelium forms the stroma of
the iris. The growth of the iris is influenced by two factors, i.e. Closure of the embryonic fissure
(choroidal fissure) and atrophy of tunica vasculosa lentis.
The embryonic (foetal) fissure is a deficiency at the under surface of the optic vesicle
and optic stalk extending from the tip of the optic cup almost as far as forebrain. The embry-
onic fissure is the gap through which the mesoderm surrounding the optic vesicle gets entry
into the cavity of the cup and form the retinal and hyaloid system of the vessels. The
embryonic fissure starts closing at 10-11 mm. stage at the middle of the fissure and spreads in
both directions, the anterior end fuses later than the posterior end. The closure is completed
by 18 mm. well before the development of the iris. Failure of the closure of the embryonic
fissure results in various congenital anomalies of the uvea ranging from notch coloboma of
the iris to extensive coloboma involving all parts of the uvea at the inferior surface of the
cup. Non closure of the foetal fissure from end to end leads to formation of colobomatous
cystic eye ball.
The choroid develops from mesodermal mass at about 6 mm. A network of capillaries
that surround the optic cup develop into choroid. The Bruchs membrane is secreted by neural
epithelial layer. By third month large and medium choroidal vessels develop. The vortex veins
also develop at the same time.
Congenital anomalies of the uvea
Congenital anomalies may involve all the three parts of uvea or individually iris, ciliary
body and choroids in various combination. Close proximity of iris and ciliary body and choroids
to retina may involve them as well. Frequently the congenital anomalies of uvea are due to
faulty closure of the foetal fissure resulting into colomboma of the uvea of various types. A
coloboma is said to be complete when all the layers of iris, ciliary body or choroids are involved
and incomplete where instead of full thickness involvement only partial thickness is
underdeveloped. A coloboma is called typical when it develops at the site of the closure of
foetal fissure i.e. inferior nasal part of the uvea and atypical when coloboma is located in
place away from foetal fissure. The coloboma may be unilateral or bilateral when present in
both the eyes they are generally symmetrical. colobomas are generally hereditary, involve
both sexes equally.
Coloboma of the iris
Coloboma of the iris may be typical or atypical, can be complete or incomplete.
Involvement of the iris in coloboma varies from a small notch at the pupillary border to extensive
involvement of the uvea from pupillary border to the optic nerve. The typical coloboma is
situated at inferionasal part of the iris. It extends from pupillary margin up to the root of the
iris, may extend in the ciliary body. The eye may be of normal size but most of the times it is
smaller than normal. The pupil is inverted pear shaped or key hole like. If the coloboma
extends into the ciliary body, a bluish streak is seen through the conjunctiva. The lower end of
the lens is visible with its zonules. If the ciliary body is involved there may be absence of
zonules at the site adjacent to the coloboma. There may be localized peripheral lenticular
opacity in the lens behind the coloboma. Vision varies from almost normal to severe loss
depending upon the degree of involvement of intraocular structures. The eye have variable
degrees of errors of refraction.
Psudo polycoria denotes full thickness defects in the substance of iris. The defect is
generally circular, looking like additional pupil. Such pupil do not have sphincter or dilator
muscles or pigments on its border. These pupil does not react to light or accommodation
independent of usual pupil. A similar hole near ciliary body is called iridodiastasis
12
. A
coloboma is called bridge coloboma when a strand of iris tissue spans over a coloboma, bridging
the gap, this tissue is mesodermal remnant of pupillary membrane.
No specific treatment is required for coloboma of iris, however, presence of error of
refraction should be managed as and when present to prevent amblyopia.
Anomalies of position, size and shape of pupil - Normal pupil is central, circular
with slight nasal shift. Position of both the pupil is identical in two eyes. If the pupil is shifted
from its normal position it is called ectopia pupil or corectopia . These pupil have their own
sphincter and dilator muscles. The two pupil generally do not have symmetrical displacement.
The corectopia is generally associated with ectopic lens, the lens generally subluxates away
from the decentred pupil.
Congenital microcoria or miotic pupil is due to faulty development of sphincter
pupillae. A pupil is said to be microcoria if its diameter is less than 2 mm. in distant gaze.
Anisocoria
When the size of two pupil are different the condition called anisocoria. The two pupils
are never of the same size. A change of more than 2 mm. is called clinical anisocoria.
Dyscoria is a rare condition where there is congenital abnormality in shape of pupil
other than the coloboma. It is generally bilateral. The pupil is generally slit like in presence of
light but becomes almost circular in dim light.
Aniridia
13, 13A, 14
Tough the term aniridia should mean total absence of iris, in clinical practice it is not so.
Even in most advance cases, there are always some strands of iris present at the roots. In
about 50 percent of cases, they obstruct the trabecular mesh work in later life. These strands
are generally detected on 360 degrees gonioscopy.
13
The condition is present at birth and is
bilateral. There are two types of inheritance, 1. Dominant 2. Sporadic.
Besides poor development of the iris the eye has other signs as well. The cornea shows
peripheral fine pannus, sensory nystagmus, the pupil is almost as large as the cornea,
the lens shows various degrees of opacities on slit lamp examination. The zonules are visible
all round and the ciliary process are also seen. The macula is hypo plastic under develop-
ment of macula is the cause of poor vision and nystagmus.
Secondary glaucoma develops in the second and third decade of life, most probably
due to obstruction of trabeculum by iris tags. There may be other causes of glaucoma than
simple obstruction of trabecular mesh work. The glaucoma is difficult to manage and the con-
dition does not respond to medical treatment, surgery fails to reach the desired goals unless
prophylactic goniotomy is done before onset of glaucoma.
13
Half of the sporadic cases may develop Wilms tumor. All sporadic cases of aniridia
should undergo abdominal examination and to examination by ultrasonography on first
presentation and repeated yearly for next few years. Presence of Wilms tumor and aniridia is
known as Millers syndrome.
15
Management is difficult, poor vision should be corrected as
far as possible and glaucoma controlled as much as possible. The child may require low vision
aid and trained as visually handicapped.
Persistent pupillary membrane
14, 15, 16
It is an associate anomaly that looks like anomaly of the iris. Persistent pupillary
membrane is not a true congenital anomaly of the iris. Persistent pupillary membrane
are more common than coloboma of the uvea. It has been reported to be present in 96% of new
born, most of which disappears by first year. It persists for few years and then gets absorbed,
they are very rare in old age. Persistent pupillary membrane may be unilateral or bilateral
and equal in genders. They represent anterior part of tunica vasculosa lentis that supplies
nutrition to the lens in foetal life, for the first six month of foetal life and then disappears.
Failure of complete disappearance results in shred of mesodermal tissue and obliterated blood
vessels that are attached to the collarette. Development of pigment is a post natal feature of
the shreds. The strands vary in number and length. They may be gross enough to be seen by
oblique illumination without magnification or may be fine enough to be seen by slit lamp. The
shreds move freely with the movement of the pupil without restricting it. They normally distort
pupillary shape but may be mistaken as posterior synechea which are at pupillary margin.
The strands may float freely in the aqueous with one end attachment to the collarette.
It may span the pupil and get attached to the opposite collarette or may cross the pupil
in segments. Sometimes it gets attached to the anterior lens capsules where a small opacity
may be present. It may get attached to the posterior surface of the cornea with a faint opacity.
The condition does not hamper vision or cause any complication hence does not require any
treatment.
Coloboma of ciliary body
The isolated congenital coloboma of the ciliary body alone is infrequent. It is generally
associated with coloboma of the iris, may be associated with the coloboma of choroids.
Commonest congenital anomaly of the ciliary body is typical coloboma due to non-fusion of the
foetal fissure in six O clock position. However a typical coloboma may be seen at other parts.
Coloboma of the ciliary body is most commonly seen in the microphthalmic eyes. It may be
associated with subluxation of the lens due to non-development of zonules at the site of the
ciliary coloboma.
Colobomata are visualised on indirect opthalmoscopy with scleral indentation.
Occasionally it can be seen while doing goneoscopy or ultrasonography. It does not require any
treatment.
Coloboma of choroids
Coloboma of choroids can be typical when situated at the site of foetal fissure or atypical
when at other than foetal fissure. The former is more common. The coloboma when present are
bilateral in two third of the cases. It may extend from the iris to optic nerve and sometimes
involving it or it may be localized as a single oval patch in the line of the closure of foetal
fissure. There may be more than one such patch in the line of closure of the fissure. Generally
a choroidal coloboma has a parabolic appearance with its broader end towards the ciliary body
and rounded head towards the optic nerve.
The colour of the coloboma is white due to exposed sclera underneath, the edges are
clear cut, sometimes these may be irregular. The edges are pigmented. The retina is absent
over the coloboma so it is better to call it a retino choroidal coloboma. The retinal vessels are
seen to traverse over this coloboma. The scleral bed is generally depressed, in extreme cases it
may be ectatic. In rare instances of bridge coloboma, a patch of retinal tissue may cross the
coloboma from side to side. Sometimes the edges of the coloboma may reach very near the
macula but not involve it. Vision is generally poor. There is corresponding negative scotoma.
The diagnosis is straight forward.
1. On retinoscopy a white reflex in the lower part amidst a pink glow is seen.
2. Outer margin of the small coloboma is visible with direct opthalmoscopy.
3. The periphery of large coloboma is seen by indirect opthalmoscope that may show up
scleral ectasia.
4. Ultrasonography may also show choroidal defect and scleral ectasia.
One of the complications besides sub normal vision is development of rhegmatogenous
retinal detachment, when holes may develop at the edge of the coloboma.
Coloboma of macula
Coloboma of the macula may be considered as modified form of atypical choroidal
coloboma. It has also been thought to be dysplasia of macula. It is generally bilateral, it may be
very small or larger than the optic nerve. The colobomas are punched out horizontally oval
areas with clumps of pigments on the periphery and a white floor representing sclera. The
area is devoid of choroidal and retinal tissues, rarely abnormal retinal vessel may traverse the
gap. The sclera may be ectatic. Vision is greatly reduced leading to nystagmus, squint and
amblyopia. A macular coloboma may be mistaken as part of congenital toxoplasmosis.
A large coloboma may give rise to grey reflex on retinoscope. Diagnosis is confirmed by
direct and indirect opthomoscopy. An X-ray study is done to exclude intera cranial calcifica-
tion which is seen in congenital toxoplamosis.
Albinism
18
Albinism is hereditary disorder due to abnormal metabolism tyrosine hydroxylase
resulting in complete or partial absence of pigment in the body. Clinically albinism has been
broadly divided into two types, 1. Oculo cutaneous and 2. Ocular. The former can be divided in
biochemically into two sub groups i.e. tyrosinase positive and tyrosinase negative. Oculo
cutaneous albinism have extensive systemic as well as ocular involvement. The other group
known as tyrosinase positive, have some feature in milder degree of oculcutaneous albinism, it
is usually autosomal recessive inheritance.
The ocular albinism is generally X-linked and autosomal recessive in inheritance.
This involvement is limited to the globe only.
The oculocutaneous albinism produces fair coloured skin that is sensitive to light. Hair
all over the body are light coloured including eye brows and eye lashes.
In ocular albinism the lids, lashes and eye brows are normal. In both the types the eyes
are described as red eye because the pupil looks reddish instead of black and the iris is pale
blue. The vision is generally greatly reduced. There is nystagmus and squint. The iris may
transilluminate at places but there are no holes in the iris. Asymptomatic female carrier of
ocular albinism may also have iris trans illumination.
19
The eyes generally have various types
of errors of refraction, hypermetropia is more common than other types of refractive error. The
fundus looks pale against which the retinal and choroidal blood vessels stand out prominently
and albinotic fundus is further divided into two types of i.e. with developed macula and without
properly differentiated fovea. The eyes with undeveloped macula have congenital colour
blindness. In these cases decussation of optic fibers at optic chiasma has also been found to be
defective.
Management of albinism is difficult. Vision can rarely be improved, however, full
correction should be given to salvage as much of vision as possible, use of tinted glasses reduce
glare. Contact lenses are not suitable due to associated nystagmus. Albino children may require
low vision aids to pursue studies.
Heterochromia of the uvea
Heterochromia of the uvea is confined to the iris. It can be unilateral, that is one eye has
lighter iris than the other. The iris with lighter shade is abnormal. Unilateral heterochromia
of iris is called heterochromia iridum. The eyes with different colours are called
heterochromia iridus.
The exact cause of congenital heterochromia is not known. Acquired difference is
generally due to trauma, inflammation and new growth.
There are many types of heterochromia. Simple heterochromia is common without any
other ocular or systemic involvement. Sympathetic heterochromia is seen commonly with
Horners syndrome. Complicated heterochromia is seen in Fuchs heterochromic cyclitis.
Some of the systemic anomalies associated with heterochromia of iris are :
Waardenburg syndrome, Rombers syndrome and various types of status dysraphicus.
Simple heterochromia is symptomless and does not require any treatment. Other types
may require ocular treatment i.e. Fuchs heterochromia cyclitis. Systemic conditions require
multi system work up and management.
Congenital anomalies of uvea of late onset
20
There are some conditions that are thought to be congenital in origin but do not mani-
fest before ten years of age and progress relentlessly towards legal blindness by third or fourth
decade. Fortunately they are rare, but neither preventable nor treatable.
They are
1. Gyrate atrophy of choroid
2. Choroideremia
3. Choroidal sclerosis.
One of the suspected causes of Fuchs heterochromic cyclitis is congenital in origin, it
can be seen at any age in both the sexes, 10% cases are bilateral. However, some consider it be
inflammatory or degenerative and do not put in category of congenital anomalies.
Gyrate atrophy of choroid
This is an inborn error of aminoacid metabolism. It is associated with raised level of
ornithine in plasma, aqueous, CSF and urine. The condition is heriditory bilateral presenting
in the first decade as progressive night blindness. There is progressive atrophy of choroid as
well as retina. The lesions start in mid periphery as oval patches with scalooped border.
The size and shape of the lesion vary, they merge with each other to form a larger patch
and girdle the mid periphery. Choroid and retina are absent over the patch but the retinal
blood vessels remain normal for long time and then attenuate. Macular involvement is late
either as macular edema or a patch may cover the macula. Secondary cataract is common EOG
and ERG are sub normal.
There is no specific treatment. Constant supplementation of diet with pyridoxine (B6) is
supposed to delay the progress. Low protein diet is also recommended.
Choroideremia
This is a bilateral progressive disease, seen only in males, women are carriers who
show mild form of the disease. The condition is first noticed between five to ten years of age as
progressive night blindness which is misdiagnosed as vitamin A deficiency by general physician
or as retinitis pigmentosa unless fundus is examined. The fundus has no resemblance to
retinitis pigmentosa. The two features common among the two conditions are night blindness
and ring scotoma in the peripheral field. The disease progresses to legal blindness in ten to
twenty years. Central vision is retained till late. The disease is caused due to atrophy of choroid
and retinal pigment epithelial. There is no known treatment. Low vision aids and mobility
support may help. Ultimately the patient has to be rehabilitated as visually challenged.
Choroidal sclerosis
Choroidal sclerosis has two forms: 1. Adult and 2. Juvenile. The adult form is probably
is degenerative process. The juvenile form is most probably congenital in nature and manifest
round about fifteen years of age. The lesion begins on the macula that looks similar to patch of
choroiditis. The central vision is poor. There is no specific treatment. Low vision aid and reha-
bilitation are the only paliatives available.
Uveitis in children
General consideration
The term uveitis means inflammation of any part of the uvea. It is the commonest disor-
der of the uvea. No age sex or ethnic group is spared. It can be unilateral or bilateral. Due to
proximity of lens, retina, vitreous and optic nerve to the uvea, these parts are invariably drawn
into the clinical manifestation of uveitis as well as complication.
Commonest age group inflicted by uveitis is between second to sixth decade after which
incidence of uveitis diminishes rapidly. About five percentage of all uveitis is seen in pediatric
age group. Uveitis in children may range between mild self limiting anterior uveitis to sever
vision threatening endophthalmitis involving all parts of uvea as well vitreous. In one third of
all uveitis cases no definite cause can be pinpointed as causative factor. This rises to fifty
percent in uveitis in children. Undetected uveitis in children may result in permanent sub
normal vision leading to strabismus and amblyopia. Some of the uveitis in children are due to
systemic infection and are bilateral i.e. tuberculosis, syphilis, toxoplasmosis.
There is no satisfactory way to classify uveitis. Various classifications have been put
forward depending upon 1. Parts of the uvea involved. 2. Mode of onset and duration. 3. Etiology.
4. Pathology. 5. Associated with various systemic manifestation. 6. Masquerade syndromes.
1. Anatomical classification
(i) Anterior uveitis. This is inflammation localized to iris and or ciliary body. Due to
lack of anatomical barrier between iris and ciliary body the inflammation may involve both
resulting in to iridocyclitis. Inflammation limited to iris is called iritis while that involving
ciliary body (pars plicata) is known as cyclitis.
(ii) Intermediate uveitis. Here the process involves posterior part of the ciliary body
(parsplana) peripheral choroid and retina and called as parsplanitis.
(iii) Posterior uveitis. The part involved is mostly choroid, that may involve the whole
of choroid, may be localized to macula, may be near the disc or involve the periphery. Choroiditis
invariably involves retina and the condition is called chorioretinitis as seen in tuberculosis
syphilis or retinochoroiditis when the primary lesion begins in the retina i.e. toxoplasmosis.
(iv) Pan uveitis. All the parts of uvea are involved as in sympathetic ophthalmia.
(v) Endophthalmitis. In this sight threatening condition there is pan uveitis with
involvement of vitreous, not extending beyond sclera.
(vi) Panophthalmitis. Panophthalmitis is the severest form of uveitis involving all coats
of eye and periocular tissue. It is a blinding condition.
(vii) Keratouveitis. This is a very common form where primary lesion starts in cornea
and involvement of anterior uvea is due to spread of toxins to the endothelium. In case of
interstitial keratitis the inflammation starts in the uvea and spreads to the cornea.
(viii) Juxta papillary
22
. The lesion is a choroidal inflammatory patch near the disc
producing sector shaped lesion and corresponding field defect.
(ix) Disseminated choroiditis. Small areas of inflammation are scattered all over the
fundus behind the equator.
(x) Central choroiditis. It is located in the posterior pole involving the macula.
(xi) Sclero uveitis is involvement of uvea along with sclera.
2. Onset (Chronological)
On the basis of mode of onset, uveitis can be
I. Acute
II. Chronic
III. Recurrent
IV. Acute exacerbation on chronic.
3. Etiological
Etiological classification of uveitis is most logical from point of management but it is not
possible to pinpoint the exact cause in about one third cases.
Eiologically uveitis can be divided into :
(i) Microbial
(a) Endogenous.
(b) Exogenous.
(a) Endogenous microbial uveitis is either caused due to direct invasion of uvea by
micro organism or due to immune reaction of the micro organism to the uvea.
(b) Exogenous microbial uveitis is due to direct entry of pathogenic organism,
following perforation of globe, accidental and surgical or spread from outer coat of the eye
mostly as a result perforating corneal ulcer.
The organism can be bacteria, viruses, fungi, protozoa and nematodes.
All bacteria are capable of producing some type of uveitis or other if they get access to
the uvea. They can produce acute or chronic lesions. Acute lesions are generally produced by
cocci i.e. gonococcus and pneumococcus. The bacilli generally produce chronic uveitis i.e.
tuberculosis, syphilis, leprosy
23
.
Viruses that cause uveitis are herpes simplex, herpes zostor Epistein Barr virus, rubella,
influenza, measles and mumps.
24
Fungi that produce uveitis are candida, fusarium.
Out of many protozoan infection that produces uveitis is Toxoplasma gondi.
25
It
generally produces bilateral congenital or acquired posterior uveitis.
Toxocara canis and catis are two round worms of dogs and cats that produce unilateral
posterior uveitis in children that is always vision threatening, but not fatal and must be
differentiated for more serious retinoblastoma which is both sight threatening as well as life
threatening. Both present with white reflex in pupillary area. Other nematodes that cause
uveitis are cysticerosis and onchocercisasis.
(ii) Auto immune disease. Auto immune disorders that generally have systemic mani-
festation form the largest group of diseases that produce both acute and chronic uveitis.
They are generally associated with certain specific HLA type. These are juvenile rheuma-
toid arthritis, ankylosing spondylitis, rheumatoid arthritis, Reiters syndrome, Vogt
Koyanagi Harada disease, Behcet disease. HLA typing is now considered an important
diagnostic test in uveitis.
24
Sympathetic ophthalmia is presumed to be due to hypersensitivity
to uveal pigment. Lens protein sensatization cause phacogenic uveitis.
(iii) Other non specific uveitis arePars planitis, Fuchs heterochromatic iritis,
sarcoidosis, malignant tumor of eye, long standing retinal detachment, iritis glaucomatosa,
retained intra ocular foreign bodies specially pure copper, anterior segment ischaemia follow-
ing squint surgery, pulse less disease also produces uveitis but all are not seen in children.
4. Pathological classification
The pathological classification most widely used was suggested by Woods in 1947
27,28
,
that divides uveitis into two groups i.e. granulomatous and non-granulomatous, on the
basis of histopathology presentation without sharp line of demarcation in clinical presenta-
tion.
The granulomatous uveitis generally involves all the part of uvea with predilection
for choroids. It is more commonly a bilateral involvement. There is general actual invasion of
the uvea by organism. There is a proliferation of tissue. The proliferation of tissue due to
microbial invasion depends upon the virulence of the organism and the state of immunity of
the tissue. The onset is generally insidious without much pain, the keratic precipitates are
large mutton fat in nature, aqueous flare is relatively low with scanty cells. Hypopyon is
unusual, the vitreous shows marked flare and cells. The posterior synechia are thick, broad
and difficult to break. In late cases peripheral anterior synechia are seen. The iris develops
nodules. The condition has long slow course.
The non-granulomatous uveitis has an acute painful onset, generally uniocular to
begin-with, other eye gets involved later, mostly self limiting. An episode lasts for four to six
weeks without treatment. There is marked circumciliary congestion. There may be ciliary
tenderness. This may involve all parts but anterior uveitis is more common. KPs are generally
small and numerous. There may be fibrinous exudation and formation of hypopyon. Anterior
chamber reaction is marked with flare and cells. Vitreous does not show cells, pupil is miotic.
Posterior synechia are small and narrow, break with ease. In late stages there may be peripheral
anterior synchia. Iris module are generally not formed.
Division of uveitis into two classes of granulomatous and non granulomatous is not
always clinically possible. A case of acute anterior uveitis of non infectious origin may show
large mutton flat KPs or a case of chronic anterior uveitis may show indolent character of
granulomatous uveitis. Some of the examples areuveitis due to leptospirosis should cause
a granulomatous uveitis but in fact it presents as a non granulomatous uveitis. Similarly lens
induced uveitis is expected to be an allergic reaction to lens protein and should behave like non
granulomatous which on slit lamp, looks like granulomatous. Similarly sympathetic uveitis
also presents as granulomatous lesion with epithelial cells and giant cells when it is supposed
to be an allergic reaction. Sarcoidosis is a non infective multi systemic disorder. Logically it
should produce non granulomatous uveitis but in most of the eye, it present as granulomatous
uvietis with nodule formation.
Clinical features of uveitis in general
Symptoms and signs in uveitis greatly differ in different types. There may just be blurring
of vision without pain or redness as seen in pars planitis, white iritis in girls and Fuchs
heterochromic iritis to severe loss of vision with pain as in endophthalmitis. Symptoms of
posterior uveitis may differ from those of anterior uveitis in such an extent that they may look
like two separate entities. Many conditions may masquerade as uveitis when in fact they are
either life threatening serious conditions of intra ocular malignancy or benign condition like
persistant primary hyper plastic vitreous. Complications and sequaelae may change this clinical
picture entirely.
It is better to divide clinical features related to following types of uveitis, i.e. those
belonging to 1. anterior uveitis, 2. posterior uveitis, 3. intermediate uveitis, 4. pan uveitis in
separate groups. Moreover the signs and symptoms differ greatly between acute and chronic
uveitis. Complications like glaucoma or cataract may overshadow the primary features of uveitis.
Symptoms of acute anterior uveitis
1. Onset. Generally sudden and unilateral but may occasionally be bilateral. Simulta-
neous onset is extremely rare.
2. Pain. Pain may be the first symptoms of acute anterior uveitis. The pain ranges from
dull ache to throbbing pain, radiating along the distribution of ophthalmic branch of fifth
nerve on the same side. This pain is worst at night. Tenderness of the globe is indicative of
ciliary involvement.
3. Lacrimation and photophobia are common symptoms due to irritation of sensory
supply to anterior uvea. They are more marked in children.
4. Redness. The eye may present as red eye due to circum ciliary congestion caused by
dilated anterior ciliary vessels.
5. Diminished vision. Initially patient may not have diminished vision. Diminished
vision if at all present, develops after three four days, or from the beginning in a case of recur-
rent attack.
Causes of diminished vision areEdema of corneal epithelium, deposition of
precipitates on the posterior surface of cornea, turbid aqueous, small constricted pupil, exudates
in the papillary area, anterior vitreous cells and flares, spasm of ciliary body resulting in the
myopia, secondary glaucoma. Recurrent anterior uveitis is commonly associated with macular
edema, sometime there may be papillitis result in diminished vision.
Signs of anterior uveitis
1. Mild edema of the lids : Exact cause of such edema is not well understood.
2. Circum corneal congestion.
Redness around the cornea starts as a pink hue that becomes gradually darker red.
The congestion fades towards periphery. The circum ciliary congestion does not blanch
with weak solution of vasoconstrictor.
3. Keratic precipitates:
Keratic precipitates are deposition of cells, macrophases, uveal pigments or RBC on
the endothelium of cornea. It may be a passive process where cells circulating in the
aqueous come in contact with the swollen endothelium and settle on the endothelium
or as an active process where the endothelial cells become phagocytic and engulf the
circulating cells in the aqueous.
27
The large keratic precipitates may be visible on bright oblique illumination with corneal
loupe. However, to visualise them in details they should be examined with good illumination
of slit lamp. The number, shape, size and distribution depend up on severity and duration of
inflammation. Large to medium sized keratic precipitates are generally deposited in a trian-
gular fashion with apex up towards the pupil. Fine KPs are generally dusted all over the
endothelium, few KPs may be deposited in an irregular fashion.
Various types of keratic precipitates according to their size, colour and characteristic
are: large mutton fat KP., medium KP, endothelial dusting, pigmented KP and red KP.
Mutton fat KP. These are largest KPs arranged in a broad based triangular distribution.
The apex of the triangle is upwards, larger KPs occupy lower position. Larger KPs may reach
upto 1 mm. in size. They are called mutton fat KPs due to their greasy lardaceous appearance.
Mutton fat KPs primarily consists of macrophages and clusters of epithelial cells, they have a
sandy white colour, they may at times be pigmented, their number varies between ten to
twenty. They are generally seen in so called granulomatous uveitis but may be seen in recurrent
non granulomatous uveitis also. Common causes beingTuberculosis, toxoplasmosis,
endophthalmitis anaphylectica, sympathetic ophthalmia, sarcoidosis. As inflammation subsidies
they become hylanised and fade in colour.
Small and medium sized KPs are due to lymphocytes and plasma cells, their borders
are clear cut. There may be as many as forty to fifty in one eye. They are seen in non-
granulomatous anterior uveitis both acute and chronic.
Endothelial dusting is caused by very small KPs that may run into hundreds. They
are scattered all over with predilection for lower part, commonly seen in acute non-
granulomatous anterior uveitis and recurrence of such conditions.
Pigmented KPs. These are seen in chronic inflammation of long duration. They are
due to entanglement of iris pigment in white KPs or the iris pigment has been engulfed by the
cells.
Red KPs are seen in uveitis that cause hyphaema i.e. herpes simplex and herpes zoster.
Old KPs. As time passes, KPs shrink in size, fade in colour, these borders become
crenated, they have ground glass appearance and may be pigmented.
4. Anterior chamber reaction. Normal aqueous is colourless, clear fluid. In inflam-
mation there is outpouring of protein from the anterior uvea resulting into plasmoid aqueous
giving it a turbid appearance. Increased turbidity of aqueous is due to suspended particles :
cells fibrin, protein, RBC etc.
The cells. In aqueous cells originate in the iris and ciliary body due to active migration
from uvea to aqueous humor. The cells may circulate in the aqueous by convection current or
settle on the endothelium. They may be seen on the iris, ciliary body, trabeculum lens, suspen-
sory ligament and anterior vitreous face.
27
Initially the cells are poly-morphonuclear, as days
pass, they are replaced by lymphocytes, plasma cells and macrophages. More are the cells,
more is the involvement of ciliary body. The cells are examined by the slit lamp with maximum
illumination and magnification by a 3 mm. 1 mm. slit. The cells should be counted and
graded between 0 to 4+ where 0 stands for absence of cells and 4+ means over 50 cells per field.
Aqueous flare. Presence of protein in the aqueous makes it turbid as beam of light
passes through the turbid aqueous the suspended particles stand out as shining bodies against
the iris background, this phenomena is called aqueous flare. To see a flare an intense beam
of light 2 mm. wide is directed on the iris at an oblique angle and obscuration of details of the
iris is noted. Clearer details denote less flare and total obscuration is maximum flare. Flare is
again graded between 0 to 4+. Flare in not always a sign of active uveitis, it can be seen due to
leak of damaged vessels without iridocyclitis. To diagnose uveitis a combination of cells and
flare must be present.
Fibrenous exudates in the AC. In severe anterior uveitis the cells may get enmeshed
in fibrinous aqueous and form a sheet over the iris surface and lens capsule. Presence of fibrinous
aqueous is called plastic iritis which is a form of severe iritis.
Pus in AC. In severe cases of uveitis there is down powering of WBC, that due to their
weight gravitate at the bottom of AC. Accumulation of pus in AC is called hypopyon that is
sterile.
5. Iris. The iris gets fluid laden due to dilation of radial vessel and cellular infiltration of
the stroma. The iris looses its roughness, the contours of the pits and collarettes are lost. As
the iris gets fluid filled and increases in thickness it spreads towards the centre causing shrink-
age in pupillary diameter. Nodules on iris are late phenomenon mostly seen in recurrent and
chronic uveitis. A swollen peripheral iris may come in contact with posterior surfaces of cornea
resulting in peripheral anterior synechia.
6. Pupil. It is miotic which on careful examination looks irregular that reacts poorly to
light. Causes of miosis are engorged radial vessels, infiltration of stroma by cells, relatively
stronger sphincter muscles and irritative action of toxins on the nerve ending. The pupil becomes
irregular due to presence of posterior synechia between the posterior surface of iris and anterior
surface of lens in phakic eye. Irregularity of pupil becomes more marked when pupil is dilated
by mydriatic. Initially the pupil does not dilate at the site of synechia, but dilates inbetween
the synechia. This gives the pupil a scalooped or festooned appearance. With repeated use of
mydriatic, the synechia are broken resulting into a round dilated pupil and leaving iris pigment
on the anterior lens capsule which lasts for days but disappear ultimately.
If the synechia persists for few weeks, but do not break, they require stronger mydriatic
cum cycloplegia. Long standing posterior synechia are the site where future anterior capsular
opacities may develop. In untreated cases posterior synechia may spread all along the
circumference and bind down the pupillary margin all around, resulting in to a ring or annular
posterior synechia that prevents aqueous from passing through the pupil resulting in rise of
pressure in the posterior chamber that pushes the iris forward in a bow like fashion called the
iris bombe. Deposition of exudates that obscures pupillary area is called occlusio pupil with
loss of third Purkinje image.
7. Lens. In early stages that lens is clear, later the iris gets adhered to it which on
dilatation leave fragments of iris pigment on the lens, varying in number and size. In long
standing and recurrent cases anterior capsular cataract develops at the site of posterior synechia.
In case of recurrence complicated cataract develops starting from posterior capsule which on
slit lamp give a polychromatic lusture. In fibrinous iridocyclitis a membrane similar to
occlusio pupil develops behind the posterior capsule and is called posterior cyclitic
membrane.
8. Vitreous. Changes in vitreous are common specially when ciliary body in inflamed,
the changes are in content and structure. Vitreous involvement is directly proportionate to
severity of inflammation. The anterior vitreous can be seen by a bright beam of slit lamp. The
changes can be in the form of opacities that are due to cells, coagulated exudates and fibrin.
Presence of cells in the anterior vitreous is seen in anterior uveites while that in the posterior
vitreous is due to inflammation of choroid. A comparison between the cell count in anterior
chamber and posterior chamber gives a rough idea differentiating involvement of iris and
ciliary body. In case of cyclitis cell density in anterior vitreous is more than in anterior chamber,
in case of iritis the finding is reversed.
9. Fundus changes
A. The media may look hazy due to presence of KPs, fibrin in pupillary area, posterior
capsular opacification and vitreous debris. In case of a small rigid pupil fundus may
not be visible.
B. The retinal changes can be diffuse, peripheral or macular. Diffuse retinal edema may
be present in anterior uveitis. Macular changes are seen mostly as cystoid macular
edema (CME), best seen by Goldman three mirror lens or Volks + 68 to + 90D and
fluorescein angiography. The macular edema is a self limiting feature but may leave
permanent pigmentation. Peripheral changes are seen in pars planitis.
C. The disc may be blurred due to optic neuritis, this is more common in cyclitis.
10. Intraocular pressure. Intraocular pressure changes are variable, while in most of
the eyes it remains normal, in some cases it may become abnormal in initial few days, especially
in children. Lowering of intraocular pressure is more pronounced in cyclitis due to lowered
secretion in ciliary process. Intraocular pressure rise in acute anterior uveitis is due to plasmoid
aqueous and cells obstructing the trabecular meshwork, trabeculitis, peripheral anterior
synechia.
Symptoms of Intermediate Uveities
24, 27, 30, 31
Intermediate uveitis is also known as pars planitis and chronic cyclitis. The symptoms
are floaters, diminished or foggy vision that may worsen over years without any attributable
cause. Children complaining of floaters in eye should not be dismissed as simple floaters unless
proved other wise. Pars planitis is a disease of children and young adults. In 70% cases
it is bilateral.
Signs of intermediate uveitis
There is hardly any sign visible on oblique illumination the eye is white in most of the
cases. There are very few cells in anterior chamber. There may be fine KPs in the lower part,
the iris is normal, so is the pupil. The anterior vitreous shows more cells than in aqueous, the
posterior vitreous is clear.
The real diagnosis lies in the peripheral fundus examined by indirect opthalmoscope
and scleral dentation that shows peripheral retinitis, perivasculitis and vitritis. The inferior
periphery shows maximum changes in the form of white fluffy exudates as spherical deposits
or plane sheat referred to as snowball and snow banking.
The macula gets involved in late stages in the cystoid macular edema. Rarely there
may be papillitis. Posterior sub capsular cataract is a common late feature. Shrinkage of
perivascular membrane may lead to traction detachment.
Symptoms of posterior uveitis
Posterior uveitis can be acute or chronic. It may be localized either in posterior pole or
periphery, may be diffuse, scattered all over the choroids. Retina and optic nerve are fre-
quently involved. The symptoms and signs depend upon the above factors.
Pain : In contrast to anterior uveitis pain is almost always absent unless associated
with anterior segment involvement specially in the form of secondary glaucoma. Lacrimation,
photophobia, redness and ciliart tenderness are also absent.
Diminished vision : Diminished vision is a prominent feature of posterior uveitis spe-
cially if the lesion involves the macula, maculopapillar bundle and optic nerve. Peripheral
lesions do not produce much of visual symptoms. They are mostly associated with floaters in
the field of vision. Acute posterior uveitis with muscular involvement have deterioration of
vision that may be preceded by metamorphopsia.
Causes of diminished vision in posterior uveitis consists of simple macular edema,
cystoid macular edema, pigmentation of the macula, scar or the macula, vitreous haze, papillitis,
lenticular opacity and secondary glaucoma. An unilateral lesion of central choroiditis may
present as strabismus and amblyopia in a child. In case of congenital toxoplasmosis loss of
vision may be detected only when the child is brought as a case of squint.
Signs of posterior uveitis are better divided into 1. Acute and 2. Chronic.
Signs of posterior uveitis are mostly localized in the posterior pole, however, whole of
the fundus should be scanned with a direct ophthalmoscope as far as possible, followed by
indirect opthalmoscopy with scleral indentation.
Acute lesion of choroids is a patch of choroiditis seen as an area over which retina is
invariably involved, the edema may not obscure the retinal vessel, border of the lesion is well
defined. Colour of the area involved is yellowish or grayish. A large area in the posterior pole
may give grayish reflex and retinoscopy that disappears from retinoscopy field on movement
of the eyeball. The posterior polar lesions are best seen with slit lamp. Vitreous changes are
very prominent in acute lesions of the choroids. They are in the form of vitreous floaters,
vitreous flare and posterior vitreous detachment. In case of endophthalmitis there may
be severe vitreitis with accumulation of exudates. In a case of acute choroiditis, sudden loss of
red reflex denotes onset of endophthalmitis.
The vitreous opacities may vary in shape and size. They may be fine coarse, large or
stringy. Fine opacities are due to inflammatory cells. Large opacities on the periphery and
snow ball opacities are seen commonly in pars planitis, candidiasis and sarcoidosis
32
. The
vitreous opacities are graded by number of cells counted in a field by direct opthalmoscope and
graded between 0 to 4 +.
29
This grading has a disadvantage that it fails to measure the actual
inflammatory activity in the vitreous which is best seen by indirect opthalmoscope with inter-
mediate magnification, medium field, with diminished illumination, examined by + 20 D lens.
33
Visibility of three fundus landmarks i.e. optic nerve head, retinal blood vessels and retinal
nerve layer are noted and graded between 0 to 4+. Zero being clear is view of nerve fibre
striation and four plus stands for obscuration of optic nerve head.
32
Other changes noted on fundus examination are retinitis, vasculitis, papillitis and
exudative retinal detachment.
Symptoms of chronic posterior uveitis
The symptoms are same as in acute posterior uveitis i.e. there is no pain, redness,
photophobia or lacrimation. However, seeing of floaters, scotomas and diminished vision
persists.
Causes of diminished vision in chronic posterior uveitis are
Complicated cataract mostly posterior capsular opacification with polychromatic lusture,
vitreous haze, vitreous bands, scar over the macula and maculopapillar bundle. Cystoid macular
edema, macular pigmentation, macular hole, sub retinal neovascularisation, periphlebitis,
secondary glaucoma and traction detachment.
Signs of chronic posterior uveitis are same as seen in acute choroiditis with changes
due to passage of time. Some of the lesions get healed, other may develop satellite lesion
adjacent to healed or healing lesion or there may be development of new lesions independent
of original lesion.
Vitreous floaters which are hallmark of acute posterior uveitis subsides to a great extent,
however, some haze may persist. Posterior vitreous detachment is more common with
development of traction bands.
A healed patch of choroiditis has an irregular margin with clumps of uveal pigment
on the periphery. The retina generally disappears over the healed patch but the vessels persist,
there may be clumps of choroidal pigment away from the patch. The floor of the patch is white
due visible sclera over which the choroid has been destroyed. Large areas of the choroiditis
may result into a scotoma that becomes negative with passage of time. Other fundus changes
consists of sheathing of blood vessels, traction detachment, post neuritic optic atrophy, macular
scar, membrane formation over macula and neovascularisation. Intraocular tension may be
normal or raised. Hypotony is general far less frequent than seen in acute anterior uveitis.
Complication in acute anterior uveitis:
Common complications are secondary glaucoma, hypotony, massive deposit of KPs on
the endothelium, changes in refraction, cystoid macular edema, papillitis, hypopyon and
hyphaema.
Secondary glaucoma in acute anterior uveitis is generally open angle glaucoma but
can be narrow angle as well due to extremely narrow angle that gets blocked by swollen iris at
the periphery or due to dilatation of pupil by strong cycloplegic like atropine.
Causes of secondary open angle glaucoma in acute anterior uveitis are many that
may act separately or in combination, they areObliteration of the trabecular mesh work by
swelling of the meshwork in the form of trabeculitis. These already narrowed channel may
further be clogged by fibrinous aqueous. Obstruction of aqueous flow from posterior to anterior
chambers is caused due to ring synechia, aqueous flow can be hampered due to small pupil,
extensive contact of iris over the lens without the formation of actual synechia.
Hypotony
In initial phase of acute iridocyclitis aqueous secretion by ciliary body is diminished
more is the inflammation lower is the tension. This is popularly known as ciliary shutdown,
complete stoppage of aqueous production never takes place. The hypotony presents as flattering
of cornea, shallowing of AC resulting in increased iridocorneal contact that ends in formation
of peripheral anterior synechia. In most of the eyes production of aqueous returns to normal
and the tension building up to normal level or may overshoot the normal limit to result in
glaucoma. In some cases hypotony persists. The tension may be as low as 6 mm to 7 mm.
Corneal complications
Transient epithelial edema, massive deposition of precipitates on the endothelium that
may itself lead to stromal and epithelial haze.
Myopia is induced due to irritative spasm of ciliary body, plasmoid aqueous and edema
of cornea. This is transient and reversed by use of cycloplegic. Cycloplegic itself can cause
iatrogenic loss of accommodation and unmasking of facultative hypermetropia. A school going
child following bilateral use of atropine may require near correction for the period of treat-
ment. Loss of accommodation persists for two weeks following stoppage of atropine.
Cystoid macular edema and papillitis are common feature of loss of vision, exact
causes of these are not well understood most probably they are toxic in nature.
Hypopyon : Accumulation of pus in anterior chamber is common in many bacterial
uveitis. It is most commonly seen in pneumococcal inflammation. It is sterile in nature.
Presence of pus in anterior chamber is an emergency that may be ominous sign of ensuing
endophthalmitis. It is also seen in ankylosing spondylitis and Behcets disease.
Hyphaema in acute uveitis is seen in cases of gonococcal uveitis and uveitis due to
herpes simplex and zoster.
Complication of chronic anterior uveitis
These complications develop after days or months of unchecked uveal inflammation.
They can be grouped in following categories :
1. Keratopathy.
2. Iris and pupillary changes.
3. Altered aqueous dynamics.
4. Lenticular changes.
5. Vitreous changes.
6. Fundus changes.
7. Amblyopia and squint in children.
Keratopathy in chronic anterior uveitis
Involvement of cornea in long standing anterior uveitis in inevitable due to direct
continuation of corneal endothelium and iris endothelium. The commonest chronic form of
keratopathy in anterior uveitis is band kerotopathy (See page 214) which develops on the
cornea like a horizontal band in the inter palpebral area. It is due to the deposition of calcium
carbonate and calcium phosphate in the Bowmans membrane and anterior stroma.
The calcium deposited in band keratopathy is extra cellular. The epithelium in the initial
stage is normal with intact corneal sensation. On long run the epithelium may become irregular
and stains with fluorescein giving a fake impression of inflammatory keratitis. The exact cause
of band keratopathy is not known, especially why it should have predilection for central cornea
in a horizontal fashion and its more frequent occurrence in children than in senile age group.
It is generally seen in badly damaged eyes of long duration. Generally there is grave lose of
vision however there may be sufficient salvageable vision. There seems to be an unanimity in
the theory that it is a degenerative condition, other hypothesis put forward are that its deposition
is influenced by actinic rays, resulting into a chemical change between cornea and air causing
a low CO
2
and low pH in the cornea.
27
The corneal hemidesmosome may act as a lattice over
which calcium crystals are built.
35
Children are more capable of mobilizing calcium than adults.
The condition starts as faint vertical line on each cornea 1 mm inside the limbus in the
inter palpebral zone and gradually spreads towards the center. The progress is very slow, it
may take one to two years for the two ends to meet in front of the pupil. May be slightly below
the center of the cornea. The band is wider in the mid cornea. A rare type begins in the center
and spreads towards the periphery. The band is white colour with a few deficiencies in be-
tween in circular fashion, these deficiencies represents the entry of the corneal nerves. In late
stages the cracks appears in the band. The overlying epithelial is not invaded for a long time
but ultimately the crystals protrode through the epithelium making it susceptible to infection.
The calcium crystals can be scrapped off the Bowmans membrane leaving a clear stroma.
Ocular causes of band keratopathy are : chronic anterior and pan uveitis in children,
chronic uveitis in young adults phthis bulbae, trauma, absolute glaucoma, failed keratoplasty,
anterior segment ischaemia.
37
Symptoms
Band keratopathy is almost symptomless except gradual diminished vision. Diminished
vision is commonest symptoms which the patient attributes to underlying ocular disease. Some-
times there may be recurrent redness, watering and photophobia.
Signs
Signs consists of horizontally placed sub-epithelial calcarious opacity with holes in
between. There is always signs of primary disease i.e. chronic uveitis, glaucoma.
Management of band karatpathy is difficult. It is presumed that an early and satisfac-
tory treatment of underlying ocular pathology wards-off band keratopathy. There are two
possible modes of treatment, 1. Lamellar corneal graft and 2. Medical treatment.
Medical treatment is an outdoor procedure where under local anaesthesia the epithe-
lium is scrapped off the band. The calcarious deposits is rubbed with sodium EDTA 0.5% for
about 10 minutes. This should remove all the deposits, if it fails strength of EDTA is increased
to 1% and rubbed for 20 minutes, the eye is bandaged with antibiotic and cycloplegic till the
cornea is reepithelised. Recently excimer photo keratotomy has given encouraging results.
38
Iris changes in chronic anterior uveitis are generally associated with changes in pupil.
Common iris changes are formation of nodules, posterior synechia, peripheral anterior
synechia, iris atrophy, increased translucency of iris and neovascularisation.
Iris nodules : There are many types of nodules that develop on the iris which fade
away with treatment, some take just few days to disappear, others may linger for longer dura-
tion. Common nodules are Keoppe nodule, Busaccas nodule, tubercular, syphilitic,
lepromatous nodules and nodules of sarcoid.
27, 32, 39
Keoppe nodules are equivalent of mutton fat KPs. They are seen on the pupillary
margin and may project in the pupil. They develop from ectodermal tissue, vary in number,
not exceeding more than fifteen in each eye. They are accumulation of large epitheloid cells
and lymphocytes. They were thought to be diagnostic feature of granulomatous uveitis, how-
ever, they are metwith in both types of uveitis. Generally posterior synechia develops at the
site where this nodule comes in contact with the lens capsules.
Busaccas nodules : These nodules are less common, fewer and smaller than Koeppes
nodule. They develop from mesodermal tissue at the periphery of the iris, around the collarette.
They take weeks to months to disappear. They may get hyalinised on long run, they are not
diagnostic of any specific disease. However they may be allergic in nature.
Tubercular nodule : Prior to the advent of anti tubercular drugs tuberculosis was
thought to be commonest cause of endogenous both anterior and posterior uveitis. With avail-
able effective anti tubucular chemotherapy incidence of tubercular uveitis have come down to
a large extent still it remains an important cause of uveitis in developing countries in all ages.
The tubucular nodules are scattered throughout the iris and ciliary body mostly in the
stroma. They are more common in the pupillary margin, they vary in size from 1 mm. to 1.5
mm. They are grayish yellow in colour some times new vessels develop round the base of the
nodule. Incidence of nodules is greatly reduced by systemic chemotherapy. Large single nod-
ule has predilection for iris root. They disappear with systematic chemotherapy and local ster-
oids.
Syphilitic nodules
40
: The syphilitic nodules are unilateral even in case of bilateral
involvement, only one eye develops nodules and can be considered as papule. They are gener-
ally located near the pupillary margin, size and number vary. The nodules are generally
hyperemic. Like other nodules the syphilitic nodules disappear with treatment, sometimes
leaving a patch of atrophy behind. In rare instances one of the nodules may be converted in to
a gumma. Hyperemia of iris in syphilitic iridocyclitis is known as roseola.
Sacroid nodule : Iritis is most frequent ocular manifestation of sarcoidosis. The nod-
ules of sarcoid look similar to that of tubercular nodule. They may be seen on the pupillary
margin or in the stroma of the iris. They are more hyperemic and vascularised than tubercular
nodules. The vessels form a lattice like pattern over the nodule.
There are many conditions that may mimic iris nodule. Some of them may be benign not
requiring any treatment.
Posterior synechia in chronic uveitis are generally broad and difficult to break when
dilated with strong cycloplegic. These pupil take a festooned appearance. It is directly related
to amount of fibrin present in the aqueous. They are generally initiated by Koeppe nodules.
Long standing posterior synechia may start anterior capsular opacification. If the posterior
synechia involves the whole periphery of the pupillary border it is called seclusio pupillae.
In severe cases of whole of this posterior surface of iris can get plastered on the anterior lens
capsule that does not separate with maximum permissible cycloplegic drugs. An annular pos-
terior synechia prevents the posterior chamber from communicating with the anterior cham-
ber via pupil, resulting in forward bowing of the iris causing the anterior chamber to be deep in
the center and shallow at the maximum bulge, such condition is called iris bombe, the pe-
riphery of the iris may touch posterior surface of the cornea resulting into peripheral ante-
rior synechia. Peripheral anterior synechia may result even in absence of iris bombe i.e. due
to prolonged contact between edematous iris and corneal endothelium at the periphery. Pro-
longed dilatation of pupil and nodules on the periphery may obstruct an anatomically narrow
angle causing rise of tension. The peripheral anterior synechia may obstruct the trabecular
mesh work and form a barrier between the anterior chamber and the trabeculum such a con-
dition is called pseudo angle formation.
The exudates may be deposited in front of the lens as a white membrane in the pupillary
area. The membrane may be dusted by iris pigment obscuring the third Purkinge image mak-
ing it difficult to evaluate transparency of the lens, this condition is known as occlusio pupillae.
Iris atrophy
In some types of anterior uveitis especially in herpes zoster and Fuchs heterochromic
iritis patches of atrophy develop. In herpes vesicles develops on iris which on healing leaves
white areas on the iris. These are areas of hyalinised scars from where pigment is removed.
These patches may have sectorial removal of pigment, this accentuates already distorted pu-
pil. The pupil adjacent to the patches of iris atrophy does not react to light or reacts weakly.
Fluorescein angiography of iris in healed herpes zoster show occuluded iris vessels at the site
of atrophy. In Fuchs heterochromic iridocyclitis patches of atrophy are most marked at the
pupillary margin. This is a non-inflammatory process.
Areas of atrophic patch of full thickness transmit reflected light of retinoscope and retro
illumination. Other condition that allows passage of reflected light through iris are-
iridectomics, iridotomies, essential iris atrphy and polycoria.
Neovascularisation of iris is a rare phenomenon in iridocyclites, it is seen in some
chronic anterior uveitis, generally at the site of previous nodule. It can be seen anywhere but
is more common at the collarette. New vessels lead to formation of small patches of haemor-
rhage or may cause frank hyphaema.
29, 32
Rubeosis of iris is due to chronic iridocyclitis. It should be differentiated from other
causes of neo vascularisation of iris and ciliary body which in adults, are central vein thrombosis,
diabetic retinopathy, sickle cell retinopathy, and Coats disease.
Altered aqueous dynamics in chronic uveitis:
Glaucoma. Formation and flow of aqueous may be normal, maintaining normal intra
ocular pressure in majority of cases of chronic anterior uveitis. There is a rise of intra ocular
pressure, which may prove to be the important missed cause of loss of vision. Glaucoma thus
developed is generally chronic secondary open angle. Sometimes there may be acute rise
of tension in eyes that are predisposed to angle closure due to crowding of iris at the periph-
ery following use to long acting cycloplegic.
Causes of secondary glaucoma are multiple, which may act separately or in combination.
They areInflammatory edema of trabecular meshwork that reduce the diameter of the pore,
making it difficult for the plasmoid aqueous to pass through. The outflow is also hampered due
to fibrosis and hyalin deposits in the mesh work. Peripheral anterior synechia may plaster the
face of the meshwork to form a pseudo angle in front of the original angle. In case of
anatomically narrow angle swollen peripheral iris may block the entrance to trabecular
meshwork, this is worsened by the use of long acting cycloplegic in anterior uveitis of long
duration. There may be neovascularisation of angle. A seclusio pupil may cause not only
pupillary block but also angle block. Long term use of cortico steroid locally or systemically in
genetically predisposed eyes is a common cause of rise of intra ocular tension, which generally
comes down following stoppage of the steroid. Chronic swelling of ciliary body can cause forward
rotation of lens iris resulting in angle closure glaucoma. Miotics are contra indicated in all
types of uveitis as they worsens acute iridocyclitis, reactivate chronic uveitis and results in to
pupillary block glaucoma. Glaucoma should be suspected in all cases of anterior uveitis, hence
it should be a practice to measure intra ocular pressure not only on first visit but also on
subsequent visits also. A reduction of vision with normal macula and no evidence of papillitis
should always warn about possibility of glaucoma, specially if the patient is on the steroid or
cycloplegic. The tension should be brought down by the use of betablockers, alpha agonist,
carbonic anhydrase inhibitors. Latanoprost which is an anti prostglandin drug that increases
uveo scleral out flow is better avoided because it can reactivate uveitis. Short term systemic
carbonic anhydrase inhibitors may be used to bring down the tension fast. Patients not
responding to medical therapy are subject to standard glaucoma surgery.
Hypotony
Reduction in intra ocular tension is common in initial stages of acute anterior uveitis
due to hypo secretion of aqueous. This is a self limiting condition, after a few days aqueous
production is resumed and intra ocular tension returns to normal or may rise to cause second-
ary glaucoma, however some times, this hypotony persists for a long time or may develop after
few weeks. It is common is patient with juvenile rheumatoid arthritis. Intra ocular tension
as low as 6.5 mm is well tolerated by the eyes without any immediate deleterious effect. Pro-
longed hypotony leads to shallowing of anterior chamber that brings peripheral iris in contact
with cornea resulting in formation of peripheral anterior synechia that may cause pseudo
angle closure glaucoma. A narrow inter palpebral fissure with reduced corneal curvature and
shallow anterior chamber should arouse suspicion of hypotony. There is no specific treatment
cycloplegic and steroid are continued. If the patient is on oral carbonic anhydrase inhibitor or
local anti glaucoma drops they are withdrawn. Prolonged hypotony over months may results
in total ciliary shutdown and result in phthisis bulbae.
Lenticular changes in chronic anterior uveitis
Lenticular complications are common aftermath of chronic uveitis of all types, anterior,
posterior, intermediate and pan uveitis.
Cause of cataract in uveitis is not well understood, it is mostly due to altered bio-chem-
istry of aqueous, vitreous or lens in various proportion. Cataract formation is more common in
cyclitis than in posterior uveitis. It takes months to years for lenticular opacity to develop. The
opacity is mostly posterior subcapsular in the form of polychromatic lusture. The corti-
cal fibre in front of this may also be involved, the nucleus becomes yellowish. The anterior
capsule also develops sub capsular opacity later, ultimately whole of the lens is opacified.
Broad posterior synechias that fail to break develop opacities under the synechia and just
ahead of the synechia, these do not progress. Deposition of exudates in occulsio pupilli may be
confused as opacity in the lens without a true cataract formation. In prolonged cyclitis the
zonules may give way resulting in subluxation or complete dislocation either spontane-
ously or with minimal trauma. A common cause of posterior capsular opacity is prolonged use
of steroid both local and systemic.
Vitreous changes in chronic anterior uveitis
Vitreous changes in chronic uveitis is less common. The vitreous may develop floaters
and liquefaction. Chorioretinitis and vasculitis may result in formation of vitreous bands
leading to rhegmatogenous retinal detachment. Posterior vitreous detachment is more
common than detachment from the base.
Fundus changes consists of : Macular edema, macular pigmentation papillitis,
peripheral retinal degeneration, peripheral vaseulitis and chronic papillitis or post neuritic
optic atrophy. Patches of chroiditis and chorioretinitis may be present.
Complications of posterior uveitis consists of loss of vision, scattered scotomas in
the field, exudative retinal detachment, neovascularisation, traction detachement complicated
cataract, vitreous detachment and liquefaction, optic neuritis and optic atrophy, glaucoma,
hypotony, phthisis.
Pan uveitis
Involvement of all the parts of uvea in an inflammatory process
24
is called pan uveitis.
All the parts may not be equally involved. Generally pan uveitis is discussed under chronic
uveitis, however, it can have both acute and chronic onset with or without recurrence depending
upon the etiology. It is generally bilateral, one eye may be involved earlier than the other.
Clinical presentation in the eye vary according to etiology, duration and treatment undertaken.
The signs and symptoms of pan uveitis are a combination of both anterior and posterior uveitis.
It can be bacterial or viral. Common bacteria are tuberculosis, syphilis, brucellosis and
lyme disease. The viruses involved in pan uveitis are herpes zoster and simplex, rubella,
rubeolla, Aids, Epistein-Barr virus. Commonest parasite causing pan uveitis is toxoplasmosis.
Conditions that have uncertain etiology areSympathelic ophthalmia, parsplanitis, Vogt
Koyanagi Harada syndrome, sarcoidosis and Behcets disease. The course tends to be
chronic with fair prognosis.
Endophthalmitis
Endophthalmitis is a severe form of pan uveitis with involvement of vitreous, retina
and optic nerve. The inflammation is always intra ocular. The organism may reach the eye
via the blood stream, may spread from sloughing corneal ulcer. Commonest source of infection
is penetrating injury either accidental or surgical. Endophthalmitis can be acute or chronic,
it may be infective or sterile. Among infective organism, bacteria are the commonest causa-
tive factors, which invariably produces acute endophthalmitis while fungal endophthalmitis
develops late and progresses slowly. Viral endophthalmitis is unknown.
Panophthalmitis
Panophthalmitis is an acute superative inflammation of eye as well as periocular struc-
tures basically it is an endophthalmitis that crosses the scleral barrier. It is generally unilat-
eral. With profound loss of vision, pain, copious discharge, edema of lids, proptosis, changes of
conjunctivitis or sloughed hazy cornea, massive hypopyon and restricted movement.
Management of uveitis
Management of uveitis may be very simple with recovery without recurrence or
complications. Otherwise it may be frustrating, long drawn with complications. Treatment
depends upon its onset, severity, part of the uvea involved, probable cause of uveitis, associated
complication, tolerance of the patient towards therapy and compliance.
Aim of the treatment for acute anterior uveitis is to 1. Reduce discomfort i.e. pain,
lacrimation, photophobia. 2. Bring inflammation under control. 3. Eliminate infective process,
when detected. 4. Prevent and treat complication as and when they arise.
Treatment of acute uveitis is divided into non-specific and specific treatment.
Non specific can be
1. Physical : Hot fomentation. This gives some relief from pain in the eye. Dry
fomentation is better as it is convenient to apply. The eye is fomented for three to five minutes
at a time for two to three times a day.
Dark glasses : In initial stages it helps in reducing photophobia, once the pupil is
dilated it reduces glare.
2. Medical treatment of acute anterior uveitis consists of
Cycloplegic, cortico steroid, non steroidal anti inflammatory drug, immuno suppressive
drugs.
Specific antibiotic for tuberculosis, syphilis, toxoplasmosis etc.
Cycloplegics : Purpose of cycloplegic is to relieve spasm of ciliary body which is a
major cause of pain in and around the eye. All cycloplegics are mydriatics. Instillation of
cycloplegic also causes much needed mydriasis that keeps the pupil dilated, prevents post
synechia, breaks posterior synechia. Mydriatics themselves do not have any therapeutic value
in treatment of uveitis, they may be added to cycloplegics to enchance mydiratic effect of the
later.
Cycloplegics are parasympatholytic agents they are of two types :
1. Long actingAtropine.
2. Short actingHome atropine, cyclopentolate and tropicamide.
Action of these drugs are not neutralized by miotics in therapeutic dose. The cyloplegics
have local as well as systemic action especially with long acting drug i.e. atropine. Cycloplegics
are used as drops, ointment and subconjunctival injections. As drops they may be used
singly or in combination of two cycloplegics or a cycloplegic with mydriatic. Commonest
combination is that of tropicamide, cyclopentolate with phenyle pherine. Out of all cycloplegics
only atropine is available in ointment and injectable form. Cycloplegics are metabolized faster
in inflamed uvea, hence their action passes off faster in uveitis and require more frequent
instillation. Atropine is strongest of all cycloplegic, it is an alkaloid. Its action starts within one
hour and lasts ten to fifteen days in normal eye. In inflamed eye the action starts late and for
shorter period, requiring frequent use for long period. Some times increase in concentration
may be required to produce desired therapeutic results. Atropine is used as sulphate either
as ointment or drops. Commonly used concentration is half to one percent as ointment and
one to two percent as drops. Concentration of more than two percent is hazardous in children.
Drops should be avoided because each moderate sized drop contains 0.6 mg. of atropine, which
is within therapeutic limit, this means that two drops contain 1.2 mg that is more than upper
limit of safety. Similarly each drop of two percent will contain 1.2 mg and such two drops when
absorbed through conjunctiva and nasolacrimal duct will produce toxicity. Absorption through
nasolacrimal duct can be minimized if the punctum is obliterated by finger for one minute just
following instillation.
Common side effect of atropine areGlare, loss of accommodation, unmasking of
facultative hypermetropia, dryness of mouth, flushing of face, thirst, rise of body temperature,
constipation, in eyes with anatomically narrow angle atropine can precipitate angle closure
glaucoma, which is not responsive to local miotics. Fortunately angle closure glaucoma follow-
ing mydriasis is rare in children. In case there is rise of tension it should be brought down by
systemic acetazolamide, local beta blockers or alpha agonist. Atropine is one of the local drugs
that is frequent cause of dermatitis medicamentosa. Fortunately this passes off within a few
days after stopping the drug. The patient may require local steroid ointment to treat allergic
blepharitis and dermatitis of the lids.
If pupil does not dilate with BD dose, it is better to add five percent phenyl pherine,
rather than increasing strength and frequency of drops in children.
In case of allergy to atropine, the drug should be withdrawn and replaced by home
atropine hydrobromide 2% drops or cyclopentolate one percent. However many of the patients
allergic to atropine are also allergic to home atropine. Similarly cyclo pentolate is known to
aggravate iritis.
To summarise
Avoid atropine drops in children.
Inform parents about possible side effect of atropine.
Once synechia are broken maintain mydriasis with other cycloplegic or combination of
cycloplegic and mydriatic drugs.
Sympathomimetic drugs have no role in uveitis except as adjuvant to cycloplegics. They
may release uveal pigment in aqueous that may be mistaken as flare.
In case of glaucoma control tension by acetozolamide, beta blockers and alpha agonist.
Latanoprost and cyclopentolate may aggravate uveitis.
Miotics are contra indicated in uveitis and do not counteract cycloplegic action. They
also do not counteract drug induced mydriatic in therapeutic dose.
If pupil does not dilate with atropine BD or combination of tropicamide / cyclopentolate
/ home atropine with phenylpherine, every half an hour for four to five instillation, the pupil
will most probably not dilate with subconjunctival injection of mydricane that is a combination
of atropine, epinephrine and procaine/xylocaine.
Cortico steroids
Most commonly used drugs in uveitis is steroid. Steroids are effective in all types of
uveitis, are easy to be administered, evaluate and adjust their dose. Steroid are very cost
effective provided they are administered as per norms otherwise they can be potentially
dangerous both locally and or systemic. Exact mode of action of steroid is not well understood,
most widely accepted theory is that they act as potent anti inflammatory agent. They do not
change the basic pathology. The steroids protect the tissue from the onslaught of inflammation
till the immune system is capable enough to overcome inflammation by reducing phagocytes,
fibrosis and neovascularisation
24
. As steroid has no effect on micro organism they should be
used under umbrella of anti microbial drugs other wise the condition may worsen. Steroids
can be divided into two groups i.e. long acting and short acting. Both groups are available
as local drops and ointments, tablets and injectable forms. The drops can be clear solution
or suspension. The clear solution have better corneal penetration hence are preferred over
suspension. Injectable solutions are also available in soluble state or suspension form. The
solution are generally short acting i.e. dexamethasone or betamethasone, which are absorbed
quickly requiring repeated injection every day or alternate day till the inflammation has been
brought under control. The suspensions are absorbed slowly from the site, they form depot
from which the drug is released. The depot may remain visible under the conjunctiva as long
as 20 to 25 days but its therapeutic efficacy is considerably reduced by seven to ten days. The
injection may be repeated every fortnight. If the condition requires prolonged use of steroid
they may be given every month. Depot injections are suitable for chronic cases. They are not to
be used in acute states.
Injectable steroids can be given as sub conjunctival, anterior and posterior
subTenon and retrobulbar. They may be give intra muscular or IV as well. Commonly used
IV steroid are methylprednisone and ACTH.
Subconjunctival and subtenon injections are absorbed by conjunctiva which passes it
over the aqueous or via sclera. A part of subconjunctival injection is bound to absorbed by
systemic circulation. SubTenon and retrobulbar injections are given for lesions behind the
lens and suspensory ligament. Retrobulbar injection is given less frequently mostly for optic
nerve involvement and causes less rise of IOP, cataract is also less frequent following retrobulbar
injection.
The tablets can be given daily or alternate day or in pulse form. The daily dose can be
given in divided dose of TDS or QID or as a single dose in the morning just before breakfast.
The divided dose is more effective but produce more side effects. Both short and long acting
drug can be used. Daily single dose or alternative dose reduces adrenalin suppression, hence
they cause less side effects. Short acting steroids like dexamethasone or betamethasone are
not suitable for alternate day schedule as steroid in therapeutic dose is not available on the
skipped day. The commonest drug used for daily and alternate day regime is prednisolone in a
dose of 1 mg to 1.5 mg per kg. In an adult this may be as high as 60 mg to 120 mg. per day.
Children are put on steroid in consultation with pediatrician. The steroid should never be
stopped abruptly. They are generally tapered over weeks or months.
General principles of use of steroid are : Use enough, soon enough, often enough
and long enough. The above dictum is true for systemic and local steroids.
Local steroids
Local steroids are available as ointment as well as drops. Ointments are poorly ab-
sorbed, they form an oily layer over cornea that reduces vision and interferes with examina-
tion by slit lamp or opthalmoscope. The advantage of ointment is that it has prolonged effect
hence can be used during sleep.
The drops are available as clear solution or suspension. The frequency of instillation
depend upon severity of uveitis. In acute iridocyclitis they may be used initially every hour for
first 6 hours followed by seven to eight times a day and gradually tapered to twice or once a
day. The steroids can be tapered not only in frequency but also in strength. They can be diluted
as much as one tenth, not loosing much effectiveness when used for long time. Patients on
systemic steroids require less local steroid. It is better to use a broad spectrum antibiotic drop
along with local or systematic steroid to ward off possible secondary infection. Such prophylactic
use of antiviral and antifungal drugs have doubtful role. It has been observed that eye under
steroid therapy requires less cycloplegic to dilate the pupil.
Side effect of steroids:
Steroids are most potent anti inflammatory agents with a very wide spectrum utility, as
expected such potent drugs have far reaching side effects both local as well as systemic. Local
and periocular use of steroids produce less side effects but are not altogether free from them.
Local steroids are capable of producing corneal complication and rise of intraocular tension
like systemic used steroid, they may even produce systemic side effect to less extent when
used for a long time. Local complication include delayed healing of both post traumatic and
infective process. Reactivation of dormant infective process, corneal staining, rise of intra ocular
pressure, moderate mydriasis, posterior subcapsular opacity and ptosis.
Systemic use produce all above side effects along with following in various combination
: Precipitate latent diabetes in steroid responders, worsens existing diabetes, salt retention
leading to weight gain, edema, hypertension. There is potassium loss, generilsed weakness,
ptosis, personality change, sleep disturbance, Cushing syndrome, acne and poor healing.
Non steroidal anti inflammatory drugs:
These drugs are mostly prostaglandin inhibitors. They block the synthesis of enzyme
prostaglandinsynthatase and cycloxygenase. They also block polypeptide of kinin system,
lysosmal enzyme, lymphokinase. They are available as local drops, ointments, oral tablets
and injection. They include age-old analgesic like salicylate, aspirin, indomethacine and recent
drugs like ibuprofen, diclofenac sodium ad potassium, nimesulide, ketarolac etc. Newer drugs
are being added frequently claming to be more effective and less toxic. Local drops are usually
used twice a day. Their use is restricted to mild to moderate anterior uveitis, their simultaneous
use with steroid results in lowering requirement of steroid. They do not replace steroid
altogether. They can be used to prevent recurrence for long time. They hardly produce any
ocular side effect. They antagonise prostaglandin induced miosis which may otherwise be
atropine resistant. They also antagonise vasodilatation, and reduce aqueous flare. They are
used to enhance preoperative mydriasis and maintain intraoperative mydriasis. They are also
used in cystoid macular edema.
All NASID are potent analgesic and moderate antipyretic. Systematic side effects produce
by NSAID arehyper acidity and peptic ulceration. Systemic allergy has been reported.
Anti metabolites
These potentially dangerous drugs are used in following conditions: Bilateral vision
threatening condition not responding to permissible dose of steroid or where steroids are not
tolerated. The patient should follow the instructions and agree for adequate follow-up and
have a knowledge of side effects of antimetabolites. There should be no contra indication of
their use like tuberculosis, toxoplasmosis, fungal infecton, herpetic disease, cytomegalo virus
infection.
The drug should always be prescribed under supervision of oncologist. Their benefit
should always be weighed against their potential dangers. All of them produce cytopenaea
and liver toxicity. The dose used in ophthalmology is comparatively low. Hence side effects
encountered in ophthalmic use are fewer in comparison to malignancy.
They are mostly used in sympathetic ophthalmia, Vogt-Koyanagi- Harada disease,
Baheets disease and serpiginous choroditis. They may be used in parsplanitis, chronic
cyclitis, retinal vasculitis. Their role in childhood paraplanitis is questionable. Cytotoxic
drugs are not available as drops or ointment commonly used drugs are cyclophosphamide,
cyclosporin, chlorambucil, azathioprine, methotrexate. Cyclosporine does not come under true
antimetabolite, it is basically an antibiotic. Combination of steroid with cyclosporin reduces
total dose of cyclosporin which is available as drops.
Specific type of uveitis in children:
While pediatric population comprises of 15% of general population, incidence of pedriatric
uveitis ranges between 2 to 10 percent
27, 42
, average being 5%. These figures are definitely less
when compared to adult uveitis, however, it is important not to miss uveitis in children be-
cause it can be bilaterally sight threatening. Some of the peculiarities of pediatric uveitis
are :
1. Incidence of bilateral uveitis in children is more than adult uveitis.
2. Unilateral chronic uveitis (white uveitis syndrome) may go undetected.
3. Girls are effected more than boys in a ratio of 6:4 except sympathetic uveitis where
boys outnumber girls.
4. Endogenous uveitis is commonest form of childhood uveitis.
5. Acute anterior uveitis is less frequent than chronic recurrent uveitis.
6. One third of childhood uveitis have undetermined etiology.
7. Prognois is poor due to chronicity, uncertain etiology, late diagnosis, poor response
and poor compliance.
8. It can be congenital or acquired.
Though all adult form of uveitis can inflict patients under fifteen, some are seen less
commonly in children.
Pediatric uveitis can be:
CongenitalToxoplasmosis, rubella, herpes simplex syphilis.
Acquired
1. Infective : Generally chronic but may be acute. They may be due to tuberculosis,
syphilis, streptococcal, gonococcal. Other organism causing uveitis are pneumococcus,
staphylococcus, bacillus cereus, leprosy (rare in children), leptospirosis, propioni
bacterium, lyme disease, various fungi, toxoplasmosis, toxocara, herpessimplex
herpeszoster, aids.
2. Connective tissue diseases effecting joints
43
: Juvenile rheumatoid arthritis,
juvenile Reiters syndrome, juvenile spondylitis, inflammatory bowel disease.
3. Trauma : Sympathetic ophthalmia, sensitization to lens protein following rupture of
lens capsule, retained in intraocular foreign body.
4. Others : Vogt Koyanagi - Harada syndrome, Behcets syndrome, sarcoidosis, Fuchs
heterochronic uveitis. All these are very infrequently seen in children. All except
Fuchs heterochronic iritis generally produce bilateral chronic pan uveitis. Pars
planitis is common in children that produce intermediate uveitis. Other causes
are ischeamic ocular inflammation following squint and retinal surgery.
5. Masquerade syndromes : These disorders are mostly non inflammatory in nature
diagnosed as chronic idiopathetic uveitis. They can be life threatening malignant
lesions or non malignant lesions in children. Common examples areRetinoblastoma
leukemia, medulo epithelioma, intraocular foreign body, Coats disease,
peripheral retinal detachment.
Toxoplamosis uveitis
Toxoplasmosis has emerged as commonest cause of posterior uveitis. It is caused by
protozoa, toxoplasma gondi that is an obligatory, intra cellular parasite. Cat is the first
definite host and humans are the intermediate hosts. In cat it is an intestinal parasite that
reproduces sexually in the gut and is shed in stool as oocytes which infect the human being by
ingestion of food contaminated by oocytes. The food may be directly infected by the dirt
containing the parasite or may be transmitted to exposed food by vectors like house fly and
cockroach.Toxoplasma can be transmitted via unpasteurised milk, half cooked meat. It is also
transmitted by way of inhalation, through blood transfusion, skin wounds and transplants.
Once it reaches human intestine it multiplies there and pass to the regional lymph nodes.
From the lymph nodes they get circulated in regional circulation
44, 45
In intermediate host
toxoplasma multiplies within, asexually and form a protective cyst within the cells where the
cyst remains protected from the immune system hence dormant occasionally. So long as the
organism is intracellular it does not give a positive serological test. Titer of serological test is
not related to ocular involvement, however, a rising titer is indicative of recurrence that is
very common. Toxoplasmosis occurs in two forms i.e. acquired that is very mild and self
limiting does not require any treatment and gives immunity to reinfection, but recurrence due
to rupture of toxoplasma cyst is possible. However a pregnant mother who is infected during
pregnancy can transmit the infection to the growing foetus. The foetus is infected via plancental
blood or by direct extension from a dormant lesion on the uterine wall. A mother who is sero
positive for toxoplasma before first pregnancy will not infect the foetus nor the subsequent
foetuses. Thus only one of the pregnancies is at risk. There is no prophylaxis against toxoplasma.
Congenital toxoplasmosis
Once it was thought that all ocular and brain lesions of toxoplasmosis were congenital
and consequently lesion as recurrences. However now it is known that these lesions can be
acquired also. The congenital lesions have predilection for brain and retina. In the brain it
produces encephalitis, the patches of which get calcified in long term. In eye the lesion are
patches of retinochoroiditis in the posterior pole on the macula, close enough to the macula
to cause loss of vision or juxta papillary. Scattered smaller patches away from the posterior
pole without visual symptoms, these lesions are detected only on routine fundus examination
by indirect ophthalmoscope.
All lesions are generally bilateral : The lesions starting at early pregnancy may heal
and the child born with a patch of healed choroiditis. The healed patch is not noticed unless
looked for particularly. A healed patch is oval in shape may be larger than disc, has patches of
pigment on the periphery. The center of the patch is white. The blood vessels pass over the
patch. There are no cells in the vitreous or aqueous. A child infected late in pregnancy has
active lesion in the posterior pole with fluffy appearance, the retina is edematous. There are
no pigments on the periphery, vitreous may have cells.
A child with congenital toxoplasmosis has three Cs representing Chorioretinitis,
Calcification and Convulsion. All neonates with convulsions should undergo fundus examination
and X-ray skull. However, the child may have only ocular lesions. In severely infected child,
there is microcephally and mental retardation.
Children with ocular lesion are generally diagnosed when they are examined for infentile
strabismus which is invariably estropia or diminished vision. Some times there may be
nystagmus unilateral cases are generally amblyopic.
Recurrence is seen between ten years and forty years. They are more common in teen
age girls
24,42
Recurrences generally occur either on the periphery of the healed lesions or as
satellite lesion adjacent. The recurrence has all the characteristics of acute lesion with
involvement of vitreous that is studded with cells and floaters sufficient to reduce the visibility
of the lesion in a head light in the fog manner
24
. Posterior vitreous detachment is
common, the detached vitreous is dusted with vitreous precipitates similar to Kps. Other
involvements are - papillitis and vasculitis. Some times the lesions may be punctate, scattered
on wide area of retina without much vitreous reaction.
Complications include cataract, glaucoma, subretinalneovascularisaton, exudative
retinal detachment
45
Vitreous haemorrhage, nystagmus, opportunistic systemic infection in
immuno compromised individual.
Differential diagnosis consist of congenital coloboma of macula, tubercular choroiditis,
cytomegalo virus infection, retinoblastoma, herpes simplex chorio retinitis.
Diagnosis : Diagnosis of congenital toxoplasmosis is straight forward when the child is
brought with systemic symptoms of convulsion, mental retardation, microcephaly and squint.
On examination a typical patches of central choroiditis is visible. On X-ray there is invariably
intracranial calcification. CT and MRI may show small lesions in the cortico medulary region.
Other investigation are (1) serological test for serum antitoxoplasma antibody. Any titer of
serum antibody is significant.
45
2. Elisa test, 3. IgG and 3 IgM titer - A positive IgG may be
present without ocular involvement, positive IgM is indicative of recent infection.
Management
There is no prophylaxis for toxoplasmosis. There is no treatment for infected cats as
cats may be asymptomatic. Best way is to look after personal hygiene, avoid raw vegetables.
Meat should be well cooked.
Once diagnosis has been confirmed the treatment is by anti-toxoplasmosis chemo-
therapy, cortico steroid, photo coagulation of peripheral lesions and management of
uveitis.
Chemotherapy
Drugs available for treatment arePyrimethamine, Sulphadiazine alone, triple
sulpha, sulpha methoxazole with trimethoprim, clindamycin, spiromycin, minocylin and
atovaquone, vancomycin, tetracycline. The dose should be administered in consultation with
pediatrician who should also monitor for side effects.
Once the lesion has healed no treatment is required, but chemotherapy may prevent
recurrence. Pregnant mothers who test positive should be given full course of treatment in
consultation with gynecologist and neonatologist. For active lesion a combination of
pyrimethamine triple sulpha with systemic steroid is the best combination available. Steroid
alone may suppress an acute attack for some times only to flare up later. The chemotherapy
should be continued for weeks, the steroid is generally tapered after seven to ten days.
Pyrimethamine can cause marrow suppression, nausea and leucopenia. Hence it is necessary
to get weekly WBC count to overcome the side effects. 5 mg. of leucovorin (folinic) acid is given
twice a week. Eye with multiple recurrence and vitreous haemorrhage may require vetrectomy.
Rubella uveitis
Rubella uveitis in children is part of congenital rubella and over shadowed by congeni-
tal cataract and glaucoma. In seventy percent of cases it produces non progressive
chorioretinitis giving a pepper and salt fundus appearance with good central vision and
normal ERG. Other causes of pepper salt fundus should be excluded. In some percentage of
cases, there may be iritis or iridocyclitis. The iris stroma is severely damaged leading to
partial hole formation that trans-illuminates reflected ray, pupil is generally small and
dilates poorly with atropine. Both dilator and sphincter muscle may be involved. There is no
specific treatment, congenital rubella can be prevented only if the mother has been immu-
nized in childhood.
Herpes simplex uveitis
24, 42, 49
In children herpes simplex uveitis is generally secondary to herpetic keratitis but can
be due to systemic involvement as well. It is mostly produced by type II herpes simplex virus,
however, type I can also cause cutaneous lesions that are self limiting. Virus may migrate to
trigeminal ganglion only to be reactivated with reduced resistance. It can cause non
granulomatous anterior uveitis with or without evidence of cutaneous or corneal involve-
ment. It can also cause chorioretinitis and retinitis in older children. The condition is pain-
ful with intense lacrimation and photophobia with evidence of active keratitis or stromal
keratitis, cells, flares, posterior synechia and occasional hypopyon. The neonate gets the infec-
tion during passage through birth canal and lesion develops between two weeks to 18 months
after. It may occasionally be life threatening. Other common age groups to be affected is be-
tween 6 months to five years.
Management is by way of cycloplegic for lacrimation, and photophobia, three times a
day and weakest possible steroids under supervision if cornea is not involved. Systemic anti
viral drugs should be administered in consultation with pediatrician.
Syphilitic uveitis in pediatric age group
Syphilis has been called greatest imitator in clinical medicine. It can inflict any age of
both sexes, all ethnic groups and involve any part of body in various combinations. It used to
be a major cause of uveitis both acute and chronic before present era of antisyphilitic treatment
came into vogue. In children commonest form of uveal involvement is associated with congenital
syphilis, however, acquired syphilis can also be seen in pediatric age group due to frequent
child abuse and precocity. Uveal involvement in children may be (1) congenital and quiet at
birth, (2) active at birth, (3) may become active at teens, (4) may be acquired in teens.
24
There
is no primary stage in congenital syphilis. The congenital syphilis in initial years are comparable
with secondary syphilis becoming tertiary, if not treated.
45,47
Hence uveal involvement in
children may show features of secondary or tertiary syphilis. It can be acute
(1) Associated with interstitial keratitis
(2) Without involvement of cornea
(a) Acute iritis
(b) Diffuse chorioretinitis.
Uveitis in interstitial keratitis preceeds corneal involvement, however, corneal reaction
may be severe enough to obstruct examination of the iris and anterior chamber. Finding of the
uveitis may only be visible after the cornea has cleared.
Corneal endothelium may be damaged by KPs and secondary guttatae may develop,
hyaline strands may project in anterior chamber, angle may get hyalinised resulting in
glaucoma. The iris may be atrophic.
30
Other syphilitic lesions include chronic iridocylitis,
chorioretinitis, retinal phlebitis, vitreous opacity.
45
Pupillary changes can be due to
involvement of iris stroma or part of neurosyphilis.
Acute iritis can start as network of fine capillaries, vascular papules and nodules.
24
There are multiple synechia resulting into miosis which does not respond to atropine, flare
and cells in anterior chamber are common.
39
Posterior segment involvement results in salt and pepper spots of chorioretinitis. It
is generally bilateral and peripheral may involve posterior pole or a quadrant. There is no
visual loss. In later stages, salt and pepper spots may be confused as retinitis pigmentosa.
Diagnosis of syphilitic uveitis is often missed as it is generally thought that syphilitic
uveitis is only seen in adults. Interstitial keratitis related anterior uveitis is easy to diagnose
and responds well to treatment. The diagnosis is confirmed by variety of serological tests.
The laboratory investigations in syphilis are same for all ages. They can be
(1) NontreponemalVDRL and rapid plasma regain
(2) Specific treponemalFTA - ABS - Flourescent treponemal antibody absorption MH4-
TP - Micro hemo agglutination test.
Non treponemal test V.D.R.L. becomes negative in late stages even with or without
treatment but FTA-ABS never become negative.
Treatment
Treatment of syphilitic uveitis with interstitial keratitis does not pose much difficulty.
Difficulty arises when uveitis in children is suspected to be due to secondary or tertiary stage.
In all such cases it should be confirmed by FTA-ABS and treated as acquired syphilis in con-
sultation with pedriatician.
Local treatment consist of atropine ointment under five, drops may be used in older
children with caution. Once desired mydriasis has been achieved, atropine may be replaced by
1% cyclopentolate. Topical steroids are administered as per severity, to begin with its can be
as often as hourly to be reduced to three times a day. Steroids drops may have to be used for
months and the child monitored constantly for possibility of side effects. In older children
peribulbar steroid can be given in recalcitrant cases. Oral steroids are not required.
Uveal involvement is generally hematogenous. As anterior and posterior uvea have
different blood supply, the involvement is independent of each other but can be concurrent.
Surprisingly involvement of uvea is rare along with active pulmonary lesion. Miliary
tubercle of choroids is common in military tuberculosis and meningitis. Tubercular lesion of
uvea is thought to be due to sensitization of uvea to tuberculosis protein. The sensitization
depends on systemic resistance and host immunity. Inflammatory and destructive process is
due to hyper sensitivity while healing is due to host immunity and host resistance.
In children, following primary infection a fast developing hypersensitivity takes place
due to low or absent immunity. It is possible to get either anterior or posterior uveitis sepa-
rately or together to results in a pan uveitis. Uveal involvement is generally granulomatous
with mutton fat KP, aqueous flare and cells in anterior chamber, edema of iris and ciliary
body. Formation of Koeppe nodules at the pupillary margin, broad posterior synechia,
exdates over the anterior lens capsule. Formation of tubercular nodule all over the uvea,
either as solitary or miliary tuberculosis. Tuberculoma is now a days extremely rare. Vitritis
is common, there may be papillitis. Macular involvement leads to early and permanent loss of
central vision. A self limiting acute iritis develops following cutaneous injection of tuberculin.
This is non granulomatous and without synechia.
Posterior uveitis is less common in children than in adults. Common presentations are :
(1) Miliary tubercle in the choroid secondary to tuberculous meningitis
(2) Circumscribed lesion in the posterior pole
(3) Involving the macula. A healed macular involvement may be confused as congenital
coloboma of macula or toxoplasmic chorioretinitis. Occasionally there may be
perivasculitis with sheathing and new vessel formation that may cause vitreous haem-
orrhage ranging from small pre retinal haemorrhage to full blown vitreous haemor-
rhage.
Diagnosis
Diagnosis of uveal tuberculosis is one of baffling problems. It is done by exclusion,
presumption and confirmation. Tuberculosis should be differentiated from other forms of
granulmatous uveitis like toxoplasmosis, syphilis, and sarcodosis.
Presumptions
In case of presumed uveal tuberculosisthe child has raised ESR and lymphocytosis.
The uveitis does not respond favourably to local cycloplegic and steroids or becomes worse. A
therapeutic trial with isonniazide 10 to 20 mg/Kg per day not exceeding 300 mg is administered
to the child as a single dose daily for 2 to 3 weeks with local treatment. If there is significant
improvement other systemic anti tubercular antibiotic agents are added in consultation with
pediatrician.
57E
Confirmatory investigations : (1) Routine TLC, DLC and ESR. (2) X-ray chest to
exclude pulmonary involvement is undertaken. (This generally comes out to be negative.) (3)
A thorough examination of abdomen and lymph nodes are done to exclude extra pulmonary
tuberculosis and if found to be positive a full course of three drugs anti-tuberculosis
chemotherapy is started.
Skin test (Mantoux test). Purified protein derivative is used. First 5(TU) of PPDT is
used as interdermal injection and reaction is red after 48 hours. If this test is negative 25(TU)
is used for confirmation. Any reaction even erythema is suggestive of tubercular origin. However,
a positive test is seen in children who have received BCG as a part of immunization or suffered
from tuberculosis in the past.
Other test : Anti tubercular immunoglobin IgA. IgG and IgM.
Polymerase chain reaction is very sensitive in pulmonary tuberculosis and less sensi-
tive in extra pulmonary tuberculosis.
Management of tubercular uveitis
Management of tubercular uveitis consists of local treatment by cycloplegic and
steroids. Posterior synechia of tubercular iridocyclitis are difficult to break hence they require
prolonged use of strong cycloplegic like atropine used with usual caution. Once the pupil has
been dilated atropine may be replaced by cyclopentolate or a combination of cyclopentolate
with phenylephenine. Local steroids have to be used for long time than other non infectious
uveitis and gradually tapered off over months. The child should get minimum three drugs
for systemic anti tubercular chemotherapy for 6 to 9 months.
Toxocara infection of uvea (See page 489 also)
Toxocarasis is a nematod infection. It is a c ommon cause of chronic posterior uveitis. It
is caused due to ingestion of embryonated ova of an intestinal parasiteToxocara canis
round worm of the dogs.
50
The puppies that are most infectious, the puppies get infected via
two sources1. a more common way is ingestion of infected food and 2. Less common by trans
placental infection.
42
In both instances this ova hatches in the small intestine to form larvae.
The larva penetrates the intestinal wall and is taken up by systemic circulation to reach distant
organs. Some larvae reach the trachea and are coughed up. The coughed larvae is reswallowed
to reach the intestine and get converted into an adult worm.
51
A female adult worm produces
200,000 eggs per day, that are excreted in feces of the dog. The eggs can survive in the soil for
years without hatching. They only hatch in the intestine of either dogs or human being. The
second stage of infection develops in the intestine of a toddler who may ingest either the soil or
food contaminated with by ova. The ova form larvae in the intestine that penetrate the intestinal
wall, from where they are carried to distant organs including eye. The larvae do not mature to
become mature worm in human beings, nor they return to the gut, hence they are not excreted
in the feces of human being. The systemic infection of by toxocara has two distinct stages. (1)
Stage of viscereal larva migrans. (2) Distant spread to liver, lungs, brain, muscle and eye.
Involvement of eye is commonly referred to as ocular toxocariasis.
Visceral larva migrance is characterized by malaise, fever, cough, hepato
spleenomegaly, leucocytosis, eosionphilia and cutaneous lesion without ocular manifestation.
Ocular toxocariasis is a late feature of systemic involvement. It is rarely seen with
systemic involvement. Average age of a child with visceral larval migrans is two years while
ocular toxocariasis is seen between 18 months to 18 years, average being 7.5 years.
51
There
is a latent period of few years between visceral larval migrans and ocular toxocariasis. Ocular
toxocariasis is always unilateral condition, seen mostly in male, exact cause of this preference
is not known. All races are equally affected. Its incidence in under developed countries must be
more than reported. Once this larvae reache the eye, it can produce various types of posterior
uveitis.
51, 52
A typical lesion is posteriorly placed. Anterior segment signs and symptoms are
minimal but not altogether absent. Children are mostly brought with strabismus, diminished
vision or rarely white reflexes in pupillary area. On examination the eye is generally white
with minimum cells and flare in aqueous. Ocular toxocariasis generally produce one of the
following types of lesions.
1. Solitary posterior polar granuloma in the macular region or between the macula and
nerve head.
2. Peripheral granuloma
3. Chronic endophthalmitis.
Solitary posterior polar granuloma is most common type of lesion that causes
diminished vision due to involvement of macula. It is mostly seen in age group 6 to 14 years.
The lesion is one or two disc diameter, raised yellowish white area, may be surrounded by hard
exudates. Retinal vessels disappears in the lesion. There may be peri retinal gliosis with traction
lines, there may subretinal haemorrhage with serous retinal detachment. The lesion is
discovered either during routine eye examination or by chance or when the child closes the
normal eye and discovers diminished vision in uncovered eye or the child may be brought for
squint, more rarely with a white reflex in pupillary area.
Peripheral retinal granuloma. This is generally associated with parsplanitis, seen
above 6 years of age, may be discovered in adults by chance. The peripheral lesion is a solitary
mass associated with extensive peripheral gliosis. There may be vitreous bands extending
from the lesion to the macula, disc or both causing traction displacement of macula that may
present as pseudo squint. As the lesion is peripheral vision remains unaffected unless macula
is involved. The vitreous band may cause retinal breaks that lead to localized, or extensive
tractional retinal detachment.
Chronic endophthalmitis. This type of lesion is generally seen in younger children
who are brought with either diminished vision squint or white reflex in pupillary area. There
may be involvement of anterior segment with cells, flare, posterior synechia, even hypopyon.
The vitreous is infiltrated with cells. In late cases a cyclitic membrane may develop. Vision is
grossly diminished and it becomes difficult to visualize the fundus, macular edema and cataract
further worsens the vision. Tractional or rhegmatisgenous retinal detachments are common.
Less common presentations areExudative retinal detachment, diffuse chorio-
retinitis, diffuse unilateral sub acute neuro retinitis (DUSN)
50, 51
, optic neuritis unilateral
pars planatis.
Most commonly mistaken diagnosis is retinoblastoma which cannot always be
differentiated clinically, however, toxocariasis does not show intraocular calcification on X-ray
and ultrasonography, it is extremely rare to get bilateral toxocariasis. Anterior chamber
aspiration does not show tumour cells but gives polymerase chain reaction to toxocara antigens.
Other disorders that form differential diagnosis are : Coats disease, other forms of
endophthalmitis, persistent hyper plastic primary vitreous, retinopathy of prematurity.
Diagnosis
Diagnosis of ocular toxocariasis is always presumptive. All cases of unilateral granuloma
in a white eye should be suspected to have ocular toxocariasis unless proved otherwise. Contact
with infected puppies is so common in general population that history of contact with puppies
is of hardly any use, so are the skin changes of viscerallarval migrans. Differential count may
show eosinophilia, X-ray orbit, ocular USG, CT and MRI are negative for intraocular
calcification. Most reliable test is ELISA test to detect toxocara antibodies in serum, aqueous
or vitreous. A titer of 1:8 in serum is diagnostic. However, sometimes serum may give negative
results while aqueous/vitreous give positive results that is reliable.
Management
1. Prophylaxis. There are no sure way to prevention except personal hygiene and
non consumption of uncontaminated food, however, pica cannot be stopped in toddlers who are
most susceptible. Role of routine deworming of child may not be sufficient to expel the larvae.
However, regular deworming of puppies from second week has been recommended. It is virtually
impossible to deworm stray puppies that are the main source of contamination of soil.
2. Therapeutic.
(A) Medical
I. Anti helminths : Though broad spectrum anti helminths are routinely prescribed
in children suspected to be suffering from toxocariasis. There role has been
questioned. Anti helminths may cause increased inflammatory reaction due to
deaths of the organism. Anti helminths whenever administered should be given
under umbrella of full dose of cortico steroids in uveal toxocariasis.
II. Corticosteroids : Corticosteroids can be used in conjunction with anti helminths
or alone. They can be used orally or by way of periocular injection.
Anterior segment reaction is managed by local cycloplegies and steroids.
(B) Surgical method. Vitreous surgery not only removes all inflammatory process and
antigen but also vitreous bands and helps in placing of scleral buckle. The material
removed by vitrectomy may be used for confirm diagnosis. Vitrectomy may be used
to remove posterior cyclitic membrane as well.
Laser photo coagulation
51
of live nematod when detected, should be tried only when the
nematode is away from posterior pole.
Cryo freezing
24
: When laser is not available trans scleral cryo coagulation of peripheral
lesion may be attempted under direct vision.
Acquired immuno deficiency syndrome and uveitis
Acquired immuno deficiency syndrome is an infectious disease with world wide
distribution, both sexes are affected. No age is immune. AIDS can be congenital due to
transplacental transmission of human immunodeficiency virus type I (HIV-I) or infection may
be acquired during the passage of the child through birth canal of infected mother. Number of
children affected by AIDS is increasing. Children who receive blood transfusion regularly
due to various blood disorders are also at high risk. In older children HIV can be acquired due
to use of contaminated needle especially in drug users. The HIV virus targets the CD4
lymphocytes and destroy it.
The HIV virus is a retrovirus known as human immuno deficiency virus type I. It has
only RNA in its structure, it uses patients cellular reproductive system to manufacture DNA
for multiplication.
53
Fifty percent of HIV positive persons are transient non infectious. However,
about 90 percent will develop AIDS which is uniformally fatal over the years. There is a gap of
few years when an HIV infected person is converted to AIDS. 50% to 75% of AIDS patients will
ultimately develop ocular lesion, which may result into blindness.
The HIV virus has affinity for T helper lymphocytes (CD
4
) which are destroyed by
HIV virus leading to sever immune deficiency as a result of some organism which may otherwise
lie dormant and become active. There may be many fold increase in virulence of infective
organism in the body. Some of the microorganisms have greater predilection for AIDS patients.
These opportunistic organism can be bacteriamycobacteria, fungi, herpes simplex, herpes
zoster, cytomelagic virus, Epstein Barr virus, adeno virus, toxoplasma and pneumocystis.
Ocular manifestation may be either due to 1. AIDS itself or 2. AIDS related com-
plex.
24
3. AIDS by itself can clog this micro vasculature by deposition of immune complexes or
4. AIDS related opportunistic organism and malignancies.
The uveal lesion may be a nongranulomatous pan uveitis due to HIV or due to her-
pes simplex or herpes zoster. Commonest type of ocular infection is CMV retinitis that
cause multiple cottonwool spots, acute retinal necrosis or retinal detachment.
53
CMV retinitis
is cell CD4 dependent.
53
It is seen in patients who have CD4 count less than 50 cells/mm3. The
disease is bilateral due to haemotogenous spread. Other ocular manifestations may involve
any or many parts of the eye from simple follicular conjunctivitis to severe retinal necrosis
leading to bilateral blindness. They include keratitis, retinal vasculitis, retinal haemorrhage,
Roths spot, microaneurysm, papillitis and ischemic maculopathy.
Kaposi sarcoma is commonest form of malignancy met in patients with AIDS. Though
this is malignancy of adults it occurs in teens in patients with AIDS as well. The tumor is of
endothelial origin
54
, it is seen in 35% of with HIV infection. It involves lids more often than
conjunctiva, orbit is involved rarely. It is mostly seen in males. It is considered to be an indi-
cator that the patient has reached the last stage of primary disease
55
. Once Kaposi sarcoma
develops life, expectancy is less than two years. Extremities are involved earlier than periocular
structures. The lesions are multicentric.
Diagnosis
A person is said to have AIDS if following criteria are present.
53
1. Unequivocal evidence of HIV I infection confirmed by ELISA and Western blot test.
2. CD4 cells count less than 200 cells/mm
3
.
3. Evidence of systemic infection by at least one opportunistic organism.
4. Presence of Kaposi sarcoma.
Management
AIDS is uniformly fatal in spite of treatment. It is a blinding disease in 60% of patients
but it is fully preventable disease. Once the disease sets-in, management is by multiple
HIV drugs given systemically. Ocular management is paliative. Malignancy do not require
separate treatment however concurrent anti cancer drugs may be required. Ocular treatment
for anterior uveitis is by local steroids and cycloplegic. Posterior segment treatment consists of
systemic anti HIV drugs and surgical treatment for retinal detachment with uniformly poor
prognosis.
Connective tissue and uveitis in children
Connective tissue disease involving joints are common causes of both acute and chronic
anterior uveitis in children most of them are positive for HLA B-27, antinuclear antibodies
and negative for rheumatoid factor. Some of the characteristic features of arthritic uveitis
areThey are generally acute, unilateral that may be recurrent in the same eye or may alternate
between the two eyes, posterior synechia. Hypopyon is common.
Common joint disorders associated with uveitis in children are :
Juvenile rheumatoid arthritis (JRA), Stills disease, juvenile spondylitis, juvenile Reiters
syndrome, arthritis with bowel disease.
Juvenile rheumatoid arthritis
24, 29, 42, 43
It is the commonest form of arthritis seen in children between 2 to 16 years of age, peak
being 2 to 4 years. It is more frequent is girls. Ratio of girls to boys is roughly 3:2. Antinuclear
antibodies is positive in 75% of cases while 95% of patients have positive HLA B-27, it is sero
negative.
According to clinical features in first three months the disease has been divided into
three sub groups.
1. Predominantly systemic involvement with minimum uveal involvement. It manifests
with intermittent fever, lymphadenopathy, hepato spleenomegaly and maculo papular
rash. There may be pericarditis.
2. Polyarticular involvement with moderate uveal involvement.
This type affects four or more than five joints during first to three months. Knee joint
is mostly involved followed by wrist and ankle. Arthritis may last for many years
resulting in permanent joint deformity. The condition is generally symmetrical.
3. Pauci articular involvement with more uveal involvement - About 5% of children
with JRA come in this group. Number of joints involved in first three months is four
or less, involvement is asymmetric, knee happens to be most commonly involved.
Twenty percent of children develop uveitis. Arthritis usually precedes ocular
involvement by several years, occasionally reverse may be true.
Uveitis in juvenile rheumatoid arthritis
Uveitis in ninety percent of cases is mostly anterior uveitis, pan uveal involvement is
extremely rare so is involvement of vitreous and choroids.
It is bilateral in 70%-80% of cases and recurrent. It is non granulomatous in nature.
There are two groups of patients who have uveitis.
1. Silent white uveitis seen mostly in girls between 4 to 16 years of age. The onset is
so insidious without any discomfort that it is called silent uveitis. The condition
may be diagnosed not due to redness, lacrimation or photophobia but due to dimin-
ished vision or on routine slit lamp examination that shows one to two plus cells +
fine KPS and flare.
2. The pausi articular involvement as idiopathic anterior uveitis in adolescent. The ocular
manifests may continue despite treatment and apparent cure of joint involvement
leading to frequent complications of band keratopathy, cataract secondary glaucoma
and macular edema. Somehow hypotony is also a common feature.
The complications are potential threat to vision, hence the child should be under
surveillance for at least 6 to 7 years following development of arthritis. Children with
iridocyclitis in pausi articular disease should be examined every three months and
cases of iris with positive ANA more frequently. Examination should include recording
of acuity of vision, measurement of intraocular tension both for glaucoma and hypotony.
Child should undergo assessment of errors of refraction and examination of fundus
and detailed slit lamp biomicroscopy.
Diagnosis is not difficult with history of joint involvement in child with non
granulomatous uveitis, positive ANA and HLA B-27 but negative serology for
rheumatoid factor.
Differential diagnosis consists of idiopathic iritis, infective iritis and sarcoid in children.
Management
Management depends upon severity and duration of disease, presence of complications
are managed as and when they arises. Severity of the disease can be
1. Mild with +1 cells and flares.
2. Moderate attack lasting more than 4 months with +2 to +4 aqueous cells.
3. Moderately severe lasting more than for four months with acute exacerbation.
4. Severeattack lasts for years in spite of treatment, respond poorly to treatment, are
more prone to develop complications.
Mild to moderate cases can be controlled by cycloplegic and local steroid drops. In initial
stages the eye should be atropinised to break the posterior synechia. Once the posterior synechia
have been broken the child is put on short acting cycloplegic like home atropine 2%two to
three times a day or cyclopentolate once a day. Children should not be put on atropine for long
times as it may induce atropine induced amblyopia. A school going child under atropine require
bifocal for near work. Once the inflammation has been reduced, a short acting cycloplegic like
tropicamide at bed time gives sufficient accommodation during day to go to school. For mydriasis
induced glare dark glasses are prescribed.
Next line of medicines are steroids in the form of drops or periocular injection. Drops
may be required hourly for first few days in acute stages, otherwise instillation four times a
day is sufficient. Strength of steroid should be minimum with maximum benefit. Ocular side
effects of local steroids should be weighed against its advantages and its dose adjusted
accordingly. Periocular steroids are given in children in teens, younger children do not cooperate
for periocular injection. Systemic steroids should be avoided under ten years of age due to its
systemic side effect. If at all it has to be given it should be prescribed in consultation and under
supervision of pediatrician. Older children are given steroid as in adults.
Non steroidal anti-inflammatory drops are generally prescribed as maintenance therapy
for long time. So far no ocular side effect has been reported following local administration of
NSAID. Systemic non steroidal anti inflammatory drugs are better avoided in children.
Chronic cases not responding to local steroids or intolerant to systemic steroids are put
on systemic immune suppressives drugs in consulation with oncologist. Dose of immuno
suppressive drugs for uveitis is less than that for malignancy. A constant watch on side effects
especially haemotological must be kept. They are also better avoided under ten years of age.
Complications are frequent both due to disease itself and sometimes due to prolonged
use of steroids.
Common complications are cataract, secondary glaucoma, band keratopathy,
amblyopia and hyptony.
Cataract is managed by surgery for which a quiet eye is a prerequisite. The eye should
be white without flare for at least three month before surgery. Surgery should be followed by
use of steroid in sufficient dose and frequency for long time. An unnecessary delay in cataract
surgery may lead to amblyopia. All surgeries should be done under general anaesthesia with
endo tracheal intubation. Thick posterior capsular membrane formation is very common that
may require repeated manipulation. Role of intra ocular implant is unsatisfactory, may be
avoided as far as possible. Aphakia may be managed either by contact lens, with spectacle
reading correction or bifocal. In smaller children contact lens fitting is really difficult though
not impossible.
Glaucoma
Possibility of glaucoma should always be kept in mind, it can be due to inflammatory
process and its aftermath, steroid induced or both. Secondary post inflammatory glaucoma
respond poorly to anti glaucoma drugs. Miotics and prostaglandin analogs are contra
indicated. A child can not be put on oral carbonic anhydrase inhibitor for a long time due to
systemic side effect. Surgery also does not give satisfactory results, failure is common.
Band keratopathy is managed initially by EDTA otherwise lamellar keratoplasty or
excimer photo therapeutic keratectomy may be tried.
Amblyopia is a tumbling block that is produced due to prolonged use of atropine,
opacities in media, macular edema, undetected glaucoma, anisometropia following unilateral
lens extraction. Failure to use contact lens and unsatisfactory I0L transplant.
Uveitis in ankylosing spondylitis
42,45,56
It is a chronic inflammatory disease of unknown origin that involves mostly the axial
spine in adults. In children the disease is mostly seen in peripheral joints of lower limb. The
disease has positive HLA B 27 in 80 to 90 percent patients. It is sero negative for rheumatoid
factor.
About 25% children suffering from ankylosing spondylitis develops anterior uveitis. 50%
uveitis with positive HLA B 27 suffer from ankylosing spondylitis.
Ocular manifestations are mainly anterior uveitis that is acute, recurring, unilat-
eral, non granulomatous. About 80% children with uveitis will develop bilateral disease
that is rarely simultaneous. An attack of anterior uveitis generally lasts for 4 to 6 weeks.
Recurrence may vary between every month to one year. Uveitis may be proceeded, may be
simultaneous or may follow diagnosis of spondylitis.
Signs
Consists of circum corneal congestion, fine dusty or small KPs cells and flares are
generally in the range of 3+ and 4+. In more severe cases there may be frank hypopyon, posterior
synechia results in iris bombe and secondary glaucoma. Cataract, CME and band keratopalthy
are less common than in juvenile rheumatoid arthritis. It is more common in adolescent boys
than girls.
Diagnosis
Unilateral acute iridocyclitis in second decade in a boy with pain in peripheral joint,
positive HLA-B 27 should undergo X-ray of sacro iliac joint for possibility of sacroilitis.
Management is with cycloplegic local and periocular steroids.
Reiters syndrome is a rare disease of unknown origin, parts of it may be infective,
(urethritis and dysentery) it was originally described as triad of urethritis, conjunctivitis
and arthritis. However it is associated more commonly with anterior uveitis, keratitis than
conjunctivitis. Conjunectivitis when present is generally bilateral and mucopurulent. Arthritis
is poly articular and asymmetrical. uveitis is generally unilateral, non granulomatous.
Treatment comprises of local treatment of mucopurulent conjunctivitis by frequent
antibiotic drops. Uveitis is treated with cycloplegic and steroids. Urethritis is non gonococcal
and treated with erythromycin. Dysentery is treated by combination of trimethoprim and sul-
pha methoxazol or ciprofloxacin.
Pars planitis or intermediate uveitis
57A,57B
Pars planitis is a common chronic bilateral uveitis that is either not diagnosed till late
or misdiagnosed. Its exact etiology is not known. It has variable clinical presentation, hence it
has been called by various names since 1908. When Fuchs called it cyclitis. The Internaational
uveitis study group has finally labelled it as intermediate uveitis in 1987.
57C
Since than it is
being referred as such.
Pars planitis is a disease of pars plana of ciliary body adjoining choroid, peripheral
retina mostly peripheral retinal vessels. In large series it has been reported to be as common
as 10 to 15 percent of all uveitis cases observed. It is a disease of young, adults and children.
There are reports that it may run in families. Both boys and girls are affected equally.
To begin with it is almost symptomless, the earliest symptoms that may attract childs
attention is floaters in the affected eye or mild diminished vision. Floaters are generally not
taken very seriously by the parents or treating general physician. By the time the next symptoms
of diminished vision occurs the disease has progressed far. The vision may be within normal
limits initially. Common cause of diminished vision are- Posterior subcapsular cataract
and cystoid macular edema.
On slit lamp examination there are cells and flares is aqueous. Posterior synechia are
so uncommon in pars palanitis that some authors consider that presence of posterior synechia
is evidence strong enough to exclude pars planitis.
57D
Sometimes there may be few KPs and
peripheral corneal edema.
The anterior vitreous shows flares, cells and strands. Examination of peripheral retina
with indirect opthalmoscope and scleral indentation shows evidence of peri artrititis and
periphlebitis. Most important finding on examination of peripheral retina are snowball
opacities and snow banking mostly in the inferior quadrant. There may be new vessel
formation near the snow bank that may be source of haemorrhage
58
. Cystoid macular edema
is a late feature and major cause of diminished vision. The course is variable. Some children
may have the disease for years with or without loss of vision, others may have choroidal effusion
and serous retinal detachment. Hypotony is common. However some patients may have rise
of intraocular pressure due to spilling over of the pathology in the angle.
There is no HLA phenotypes. Fundus fluorescein angiography is the diagnostic of CMC
and periphlebitis.
Differential diagnosis consists of - idiopathic iridocylitis. Absence of joint systems
rule out juvenile rheumatoid arthritis associated with uveitis. Peripheral granuloma of
toxocara is excluded by negative serology test. Careful examination of retinal periphery,
presence of snowball makes it imperative to exclude sarcoid by X-ray chest, gallium scan and
raised serum calcium.
Management
Almost eighty percent of children do not require any treatment. Local and peripbulbar
steroids are required if vision falls below 6/18. Cycloplegic do not have any added advantage.
Children not improving with local peribulbar steroids should be put on systemic steroid for
long time with usual precaution. Immuno suppressive drugs may be required. Rarely surgical
intervention like lensectomy or vitrectomy is required.
Sympathetic ophthalmia
57, 60, 61
Sympathetic ophthalmia is a bilateral potential blinding disorder. It is said to be
rare disease in advanced countries and urban areas of developing countries but its incidence
in under developed countries must be more than reported. It is a bilateral chronic pan uveitis
with acute exacerbation and recurrences. It is most commonly seen following penetrating
injury within five mm. of limbus with incarceration of uvea. However, rupture of the globe
anywhere with incarceration of any intra ocular structure can cause sympathetic ophthalmia.
About 60% of the cases are seen following accidental penetrating wound. However, it has been
reported following blunt injury. About 30% are seen following surgical manipulation in which
the globe is penetrated. Common surgical procedures arelens extraction, any glaucoma
operation, iris inclusion surgeries for glaucoma were notorious to produce sympathetic
ophthalmia, retinal detachment surgeries with drainage of sub retinal fluid. Even laser photo
coagulation have been reported to cause sympathetic ophthalmia. It is said that sympathetic
ophlhalmia does not develop if the injured eye has developed suppuration. However, it has
been reported following perforation of corneal ulcer.
The eye that is injured is called exciting eye and the eye that develops chronic pan
uveitis is called sympathizing eye. No race, age or sex is immune. Children are affected more
than adults, number of boys developing sympathetic uveitis is more than girls. This is perhaps
due to the fact that boys are more prone to injury than girls.
Interval between injury in exciting eye and development of sympathetic uveitis varies.
It spans between 5 days to 66 years, 70% of cases develop within three months and 90% within
first year.
Etiology of the condition is not well understood, however, there is sufficient evidence
to consider auto immunity as prime factor. The reaction is delayed type-four
hypersensitivity to uveal antigens located in retinal pigment epithelium and melanocytes.
Anti retinal antibodies have been found in some cases
24, 66, 61
. The immune reaction results in
chronic granulomatous lesion that forms a giant cell similar to tuberculosis without casea-
tion. The chorio capillaries are spared. There is thickening of all the parts of uvea, spe-
cially the choroid. Small yellowish white spots called Delen Fuchs nodule are seen scat-
tered in the choroid. They are common in the lower mid periphery, however, they are not
diagnosed of sympathetic uveitis.
Clinical features
Clinical features are variable, all eyes with penetrating injury do not develop sympa-
thetic uveitis. Some may develop it within few days, others may not be effected for decades
after initial injury. Clinical presentation depends upon inflammatory process which in turn is
perhaps genetically predisposed. The disease generally develops in iris but may start any-
where in the uvea and it is bound to become panuveal.
The injured eye has evidence of penetrating injury and its complication that may mask
the presence of indolent plastic iridocyclitis.
The early symptoms of uveitis in the sympathizing eye are heralded by photophobia,
loss of accommodation, lacrimation and blurring of vision. The eye at this stage may
not be congested. First sign of sympathetic uveitis is flare the retrolental space. The anterior
chamber may show 2+ to 3+ cells and flare. The cornea has various shapes of KPs. Generally
they are large mutton flat in nature but may be fine dusty. The iris is greatly thickened. The
pupil is miotic and irregular due to strong posterior synechia. There may be both Koeppe and
Busaccas nodule present. Onset of inflammation in sympathetising eye generally worsens,
the signs and symptoms in the exciting eye.
The posterior segment involvement results in the posterior vitreous cells, papillitis,
retinal vasculitis and serous retinal detachment, the eyes are generally soft. Extra ocular
manifestation are rare, they may be vitiligo, poliosis, alopecia and polycytosis of CSF.
Common complicationa in untreated cases areComplicated cataract, secondary
glaucoma, band keratopathy, retinal detachment, phthises and blindness. Sometimes the
exciting eye may have better vision than the sympathetising eye.
Diagnosis is mostly clinical based on
1. History of trauma, that has either not been repaired or repaired without freeing of
uvea from the wound. Sometime the injured lens may cause lens related uveitis. This
does not involve the other eye and can be managed-well by removal of lens material.
2. Clinical evidence of anterior uveitis or choroiditis.
3. Ultrasonography shows thickening of choroids.
4. Fluorescein angiography may initially show areas of hypo fluorescence due to mask-
ing of choroid by Delen Fuchs nodule followed by hyper fluorescence due to the leak
of the dye.
Management
Correct and early diagnosis helps to restore vision better and prevent permanent loss of
vision. Management of sympathetic ophthalmia can be divided in the two groups (1) Surgical
and (2) Medical.
Surgical treatment is restricted to injured eye that consists of management of (1)
Injury and (2) Enucleation of unmanageable injury. All penetrating injuries in children should
be repaired under general anaesthesia, under microscope with use of viscoelastic substance.
Before embarking on repair of the visible wound possibilities of other sub conjunctival wounds
and wounds under the extra ocular muscle should be explored and repaired. The uvea should
be dis-engaged from the wound and excised by standard procedure, if necessary. The lens
matter should be removed and if needed anterior vitrectomy done. The anterior segment should
be reconstructed as far as possible, a sub conjunctival injection of broad spectrum antibiotic
with steroid is given along with atropine. The eye is kept under observation for evidence of
uveitis.
The other eye is examined regularly. The parents are warned about possibilities of
sympathetic ophthalmia and instructed to bring the child for examination if the child develops
lacrimation or unexplained blurring of vision.
Enucleation
If the injured eye is not salvaged it is better to enucleate it within the first ten days of
injury. The purpose of enucleation is to remove the uvea in toto, evisceration does not serve
this purpose hence it is not recommended. Role of enucleation after onset of sympathetic
opthalmia is doubtful. Sometimes the exciting eye may have better vision than sympathizing
eye.
Medical treatment is usual treatment of non infective granulomatous uveitis that can
be local and systemic.
Local treatment consists of judicious use of atropine and local steroid drops. Initially
steroid may be required as frequently as hourly which is generally tapered to two times a day.
If steroid drops do not give relief within 48 hours peribulbar injection of steroid is advocated,
that may be given two to three times in a week by way of soluble steroids followed by monthly
injection of depot steroid.
Systemic
Systemic drugs used in sympathetic ophthalmia consists of two groups of drugs. They
are
1. Long acting steroids and
2. Immunosuppressive drugs.
Cortico steroids are the main stay of management of sympathetic ophthalmitis. Most
commonly used steroid is prednisolone in a dose of 1 to 1.5 mg/kg/day. It can be given in
divided doses, daily single dose or alternate day. It may be started with divided dose and
switched over to alternate day dosage and gradually reduced by 5 mg. a week and later
maintained either on 5 to 10 mg. a day or 10 to 20 mg. alternate day for months with usual
precaution.
Immunosuppressive drugs are prescribed when steroids are ineffective or producing
local and systemic side effects.
Commonly used immunosuppressive drugs are :
1. Cyclosporin A 5 mg/kg/day oral
2. Azothioprine 1 to 2 mg/kg day oral
3. Methotrexate 5 to 30 mg/week
4. Cyclophosphamide 1 to 2 mg/kg/day
Immunosuppressive drugs can be given alone or in combination with steroid. They should
always be prescribed in consultation with oncologist.
Vogt Koyanagi Harada Syndrome
24, 63, 64, 65
This is a syndrome of unknown etiology and it looks very similar to sympathetic
ophthalmia except that there is no trauma involved in its causation. It is rarer than
sympathetic ophthalmia in children. Both the conditions produce chronic panuveitis. There is
no exciting eye or sympathizing eye in Vogt Koyangi Harada disease. Extra ocular features
dominate in Vogt Koyanagi syndrome over ocular findings while in sympathetic opthalmia
ocular presentations overshadow systemic features which are late features as well.
The syndrome has
(1) Prodromal stage that may be mild and consisting of headache, vertigo, vomiting,
neck stiffness and periorbital pain.
(2) Systemic features generally precede ocular features, which are :
(i) Skin changes in the form of vitiligo, poliosis and alopecia.
(ii) Neurological signs and symptoms are headache, neck stiffness, meningism,
encephalopathy, myopathy, cranial neuropathy, CSF lymphocytosis.
(iii) Auditory features are tinitus, vertigo and deafness. The auditory symptoms
lasts only few months.
(3) Uveitis phase : This can be divided into two groups :
(i) Anterior segment involvement or Vogt Koyanagi form - This consists mostly of
granulomatous uveitis that begins with blurring of vision, diminished near
vision, photophobia, redness, lacrimation, dull pain. On examination, there
is ciliary flush, mild mutton fat KPs, anterior chamber reaction, edema of iris,
strong and broad posterior synechia, the iris nodule and atrophy of iris stroma.
(ii) Posterior segment involvement (Harada syndrome) consists of multiple exu-
dative choroidal lesion that results in exudative retinal detachment which is
self limiting and resolve spontaneously. There may be papilitis. Involvement of
macula results in metamorphopsia. Fluorescein angiography shows multiple area
of hypo fluorescence. Area of retinal edema and detachment show hypo fluores-
cence. Areas of choroidal ischeamia are better shown by indocyanine green.
Exact etiology is not known but it is thought to be a T-cell mediated auto immune reac-
tion to melanocytes resulting into affection of organs that contain melanocytes i.e. skin, uvea
etc.
Complications consists of complicated cataract, secondary glaucoma band keratopathy,
phthises.
Management is similar to any chronic non-infective granulomatous uveitis. It is
generally treated by local (1) Cycloplegia (2) Steroid drops (3) Periocular injection of depot
steroids and (4) systemic steroids in divided multiple dose, daily single dose or alternate day
single dose. (5) Immunosuppressive agents are effective in recurrence and helps reduce dose
of steroids.
Sarcoid uveitis
It is a disease of adults but may be seen in children as well. Sarcoid is a systemic disorder
of unknown cause of most probably T-cells mediated immunity that involve almost all organs
of the body. All parts of the eye may be affected. Uveitis in sarcoidosis can be acute or chronic
both granulomatous and non-granulomatous lesion are possible. Both anterior and
posterior segments may be involved. Common posterior segment involvement can be vitritis,
vitreous haemorrahage, pars planitis with typical snow bank appearance and candle wax
lesion in retina. Diagnosis is confirmed by histopathological examination of skin or conjunctival
nodule. Management is systemic steroids, local, cycloplegic, local and periocular steroids.
Fuchs heterochronic iriodcyclitis
It is commonly presumed that Fuchs heterochromic iridocyclitis is a disease confined to
adults only but it is known to be present in all ages, in all ethnic groups and both sexes. It is a
predominantly uniocular disease. Only 10% cases are bilateral. It is generally asymptomatic.
Attention may be drawn to uniocular light colour of iris. The iris with light colour is the
affected eye. The eye is generally white, on slit lamp examination small non pigmented
keratic precipitates are seen. There may be iris nodules but no posterior synechia are seen,
there may be vitreous cells. It has a protracted course with good prognosis. Most of the cases
do not require any treatment unless cataract develops. Some cases may require local steroids.
Cycloplegies are not required.
Lens induced uveitis
So long as the lens is inside intact capsules it does not produced any reaction either
allergy or chemical. Leak of lens material causes two types of reaction.
(I) A chemical reaction due to leak of lens protein through non ruptured lens capsule
in hyper mature cataract.
(II) Type two hyper lens induced hyper sensitivity. In children hyper mature lens
may result either in traumatic cataract following blunt injury or in unattended
congenital cataract. The condition may produce acute or chronic anterior uveitis and
occasional glaucoma. Uveitis subsides with complete removal of lens matter. If lens
matter is left, it may result into second type of lens induced uveitis i.e.
endophthalmitis phacoanaphylactica. Other mode is traumatic rupture of lens
either due to accidental penetrating injury or surgical injury resulting in severe
uveitis that may involve all the parts. After uveitis subsides in the first eye, the
second eyes get effected in similar ways i.e. if the lens ruptures accidentally or during
surgery even years after the original incident. This may be confused with sympathetic
uveitis. Treatment consists of local cycloplegic, steroid, beta blockers. Complete
removal cortical material in the first eye.
Behcets disease
42, 66, 67
The disease is seen world over as rare condition, is more common among Japanese.
Its share as causative factor of uveitis in children is very less. Exact etiology of the disease
is not known. HLAB-51 is closely associated with it. There are two types of ocular involve-
ment. 1. Mostly anterior segment involvement. 2. Mostly posterior segment involvement
with systemic involvement that includes recurrent aphthous ulcer of mouth and genitals,
skin lesions, joint pain. There may be sudden development of hyppyon in iridocyclitis. There
may be cystoid macular edema, retinal exudates and haemorrhage, cataract and glaucoma.
Treatment is unsatisfactory. Local treatment consists of cycloplegic and steroids, systemic
steroids are given only in cystoid macular edema for short period. Prolong systemic steroids
results in poor visual outcome, some times immuno suppressive may be required.
Anterior segment ischeamia and uveitis
Anterior segment ischemia is mostly an iatrogenic disease following strabismus and
retinal surgery. This is more common in adults and incidence rises with age, however, it can
occur in children. Basic pathology is cutting blood supply of the anterior uvea by way of
disruption of anterior ciliary artery following disinsertion of more than two recti.
68
The disorder
starts within 24 hours of following surgery with pain, lacrimation, photophobia. It presents as
hazy cornea due to edema and folds in Descemets membrane, keratic precipitates, aqueous
flare and cells. If not attended to it may lead to iris atrophy causing distorted pupil and
complicated cataract. Anterior segment ischeamia may also develop in sickle cell
haemoglobinopathy. Flourescein angiography of iris shows well demarcated areas of non
perfusion.
69
Management consists of intensive local steroids and use of cycloplegic. It is
presumed that if only two muscles are disinserted at a time, the condition can be avoided.
Endophthalmitis
Endophthalmitis is one of the most dreaded catastrophes in ophthalmology. It is defined
as inflammation of intraocular tissues due to infection by bacteria, fungi and parasite (toxocara).
Immune reaction, physical or chemical reaction, vasculitis or neoplasm can also cause it. The
lens is not capable of inflammation hence it is spared.
70
It is generally acute that can become
chronic or may have chronic onset.
Clinical presentation depends upon1. Virulence of the causative organism,
2. Chemical composition of the offending causative factor, 3. Treatment received. It is gener-
ally unilateral but can be bilateral in endogenous type. No age, sex or race is immune, how-
ever, endogenous endophthalmitis is more common in children in developing countries due to
prevailing malnutrition, xerophthalmia and systemic pyogenic disease and unattended
or poorly managed trauma. Clinically there are two main types of endophathalmitis,
1. Endogeneous (metastatic) end opthalmitisThis is caused by micro organism.
Bacteria or fungi reach the eye from a primary site due to hematogenous spread.
Viruses are not known to cause endophthalmitis.
2. Exogenous endophthalmitis is caused due to direct entry of bacteria or fungi in-
side the eye following trauma or spread from neighbouring structure i.e perforated
corneal ulcer. The trauma can be (1) accidental with or without retained foreign body,
(2) Surgical.
Surgical
Commonest surgery that results in endophthalmitis is lens extraction of any type with
or without IOL. Other surgeries include all intraocular surgeries i.e. paracentesis, iridectomy,
trabeculectomy, iris inclusion procedures, mechanical capsulotomy, penetrating keratoplasty,
vitrectomy. Rarely extra ocular surgeries may produce endophthalmitis, this happens following
inadvertent perforation of globe during squint and retinal surgery, periocular or peribulbar
injection. The basic pathology is lodgement of the offfending organism and multiplication of it,
in case of micro organism or setting an immune reaction or release of chemicals. It may start
in the uvea and spread to the vitreous involving the retina in between or it may start in the
vitreous and spread to the uvea and retina. While the first route is generally seen in endogenous
endophthalmitis. The second path is commonly seen in exogenous endophthalmitis. In all the
cases, the vitreous is severely inflamed as it acts as a good growing medium for microbia to
proliferate resulting in formation of a pocket of pus in the vitreous commonly referred to as
vitreous abscess. According to the site of its commencement it can be anterior vitreous
abscess seen following intraocular surgeries of anterior segment or posterior abscess as in
endogenous endophamitis, vitrectomy, fluid gas exchange, retained intraocular foreign body.
Ultimately whole of the vitreous is involved in abscess formation. The inflammatory process
from choroid spreads to ciliary body and iris resulting in purulent pan uveitis.
Endogenous endophthalmitis in children
Endogenous endophthalmitis in children is due to spread of micro organism from primary
site that can be abdomen, lung, para nasal sinus, infected teeth, middle ear, bones and joints,
heart, skin, lacrimal sac etc. Causative organisms can either be bacteria or fungus or parasite.
Presenting features of bacterial endophthalmitis differ from fungal in many ways. Endogenous
endophthalmitis generally has an indolent course that may be initially masked by more serious
systemic condition. frequently it can be bilateral. As the infection is hematogenous it starts in
posterior uvea. The anterior uveal inflammation lags behind in contrast to exogenous
endophthalmitis where anterior segment inflammation is more pronounced than posterior.
Endogenous endophthalmitis is more common in children who are malnourished, chronically
ill, have under gone extensive abdominal surgery, have been on systemic antibiotic or steroids
for long time, under radiation therapy or have immuno compromised status. Fungi are
considered to be more common causative organism than bacteria. However, in developing
countries bacterial metastatic endophthalmitis is equally common if not more than fungal
endophthalmitis. Parasitic endophthalmitis is far more common than expected, about 16% of
all white reflexes in pupillary area are caused by toxocara.
71
Many fungi have been isolated as causative factor but common are Candida and
Aspergillus. Candida is more frequent than aspergillus. Candidal infection starts in the choroids
as multifocal round lesions that spread to cause perivasculitis, retinal haemorrhage, may lead
retinal necrosis. The symtoms are more indolent as compared to Aspergillus which is more
severe, painful and associated with hypopyon.
Endogenous endophthalmitis is more effectively treated by parental antibiotic. As the
lesion starts and remains under the internal limiting membrane. For the same reason vitrectomy
is less effective in endogenous endophthalmitis. Systemic steroids help in controlling
inflammation in bacterial endophthalmitis but are contra indicated in fungal endophthalmitis.
Local antibiotic drops are prescribed to combat secondary corneal and conjunctival infection
while combination of local cycloplegic and steroids are given for anterior uveitis.
Exogenous endophthalmitis
Exogenous endophthalmitis is three times more common than endogenous
endophthalmitis. In adult common type of exogenous endophthalmitis is post surgical, which
is less common in children whose accidental wounds with or without retained foreign bodies
cause more inflammatory reaction. Retained organic foreign bodies more inflammatory than
inorganic. Some of the organic foreign bodies are inert and do not cause any inflammatory
reaction but foreign bodies containing copper and iron cause various degrees of chemical
changes. Pure copper produces more severe endophthalmitis than copper alloys. Pure copper
causes severest form of endophthalmitis. However, sometimes inert foreign bodies can also
cause sterile endophthalmitis. Thus exogenous endophthalmitis can be 1. Bacterial 2. Fungal
3. Sterile. All of them can follow surgery or be accidental.
Bacterial exogenous endophthalmitis is commonest form of endophthalmitis.
72
Post
surgical endophthalmitis is more common than accidental. Exact percentage of post operative
endophthalmitis is just a guess. It has been reported to be in the range of 0.05% to 0.2%. It is
more common following secondary I0L than primary I0L. In penetrating keratoplasty it is
perhaps the micro organism in donor cornea that is responsible for the infection. Break down
of asepsis and antisepsis protocol is the commonest cause of post surgical endophthalmitis.
Organism get access into intraocular structure via1. Contaminated instrument,
irritating fluid, visco-elastic, 2. Saprophytes from the lid and conjunctiva that become
opportunistic, 3. Undiagnosed nasolacrimal duct obstruction.
There are three forms of endophthalmitis according to the onset of the disease
1. Acute within first forty eight hours following surgery, always bacterial, caused by
gram negative bacteria and gram positive cocci i.e. streptococci and
staphylococcus aureus.
2. Delayed between seven to tens days mostly caused by staphylococcus epidermis
and coagulase negative cocci.
3. Lateafter four to six weeks, commonest organisms being various fungi, P. acne
and staphylococcus epidermis.
4. Remote infected bleb, prolapsed uvea.
All types of bacteria both gram positive or negative are capable of causing post operative
exogenous endophthalmitis. However, gram positive strains are responsible for 90 to 95% of
endophthalmitis.
71
Gram negative organism are more virulent, spreads fast and produce
endotoxin. In children streptococci are more common causative organism than staphylococci
which is a major cause of endophthalmitis in adults. Propioni bacterium acne, which is an
anaerobic gram positive commensal is becoming more frequent cause of chronic post surgical
endophthalmitis.
Symptoms and signs of acute post surgical endophthalmitis arePain more than
usually expected pains of surgery, lacrimation, photophobia, drop of vision below
anticipated level. On examination, the lids are swollen, the conjunctival congestion is prominent,
their may be chemosis of conjunctiva, hazy cornea. Intense flare and cells may have fibrinous
aqueous or frank hypopyon, edema of iris. Non reacting, small pupil if cycloplegics have not
been used. Exudative membrane over the I0L or vitreous face, retrolenticular flare and cells in
phakic eye. The fundal glow is either very faint or absent on retinoscopy and direct
ophthalmoscopy. An indirect ophthamoscope may circumviate the exudates in the vitreous
and allow visualization of the fundus behind. It also gives extent and depth of the vitreous
abscess that can be used to assess progress of the disease. The movements of the eyeball are
normal and there is not proptosis. Loss of ocular movement and onset of proptosis denotes
panophthalmitis. At this stage ultra sonography may clinch the diagnosis.
Delayed post operative endophthalmitis undergoes same pathological process as acute
but in a milder form. The symptoms consist of chronic reduced vision. On examination all
signs of persistent uveitis are present that are initially responsive to steroids. Thus masking
the exact pathology resulting into delay in treatment. On slit lamp examination there are
large KPs with 2+ to 3+ flare and cells, aqueous may have fibrous strands. Lower angle of AC
should be carefully scrutinized for small hypopyon that changes its level and position with the
eyeball. There is generally exudates in the pupillary area. There may be exudates behind the
posterior capsule. Generally there is formation of vitreous abscess. Marked reduction of vision
and loss of fundal glow are two ominous signs. Fundus when visible may show retinal
hemorrhages and exudates.
Late onset post surgical endophthalmitis
This is generally due to otherwise non virulent commonsels. The eye remains
congested with mild to moderate pain and vision that was good is lost in days. On examination,
there are signs of recurring pan uveitis with loss of fundal glow or a white reflex in pupillary
area. Intraocular tesion is variable. Rise of tension results in unexplained pain that makes the
child restless. Ultrasonography at this stage helps to clinch the diagnosis and differentiate
from other condition that may mimic endophthalmitis.
Post operative fungal infection is more often missed than diagnosed, as it has relatively
less symptoms than bacterial endophthalmitis, most of these eyes have generally received
usual high doses of steroids. The exact features may be masked, even the eye may be relatively
white. The child invariably does not complain of lowering of vision. It takes several weeks to
months for fungal endophthalmitis to develop. Generally there is a fixed thick hypopyon
and rise of intraocular pressure. Vitreous may show snowball and fluffy opacities that
may mistaken as cortical matter.
Sterile post operative endophthalmitis
24,71
is mostly iatrogenic in nature, less
frequent than infective endophthalmitis. The condition has to be kept in mind to be correctly
diagnosed. The inflammation is caused due to chemical insult of irrigating agents, residue
from chemicals sterilizers, lens matter, monomers on the I0L. Too much manipulation of anterior
uvea, vitreous disturbance. Inadvertent intraocular injection of xylocain during peribulbar
injection and antibodies / steroid during periocular injection talc from the surgical gloves,
cotton swabs, suture material are some of the frequent causes of sterile post operative
endophthalmitis. Sterile endophthalmitis have an acute or delayed onset and respond fairly
well to medical treatment, non responding cases may require vitrectomy.
Post traumatic endophthalmitis
About 5% of eyes sustaining accidental penetrating injury develop endophthalmitis.
Onset may be as short as 1-2 days or as long as 2 to 3 months depending upon the virulence of
the organism, size of the inoculum, immune status of the eye and treatment received. Accidental
trauma has been found to be responsible for about one third of all cases of exogenous
endophthalmitis. Increased frequency of endophthalmitis following accidental injury is high
because they are caused by infected objects that are mostly vegetable in nature and carry
virulent organism with them along with dirt, dust contaminated fluid etc. Common causes of
perforating injuries in children are - Bow and arrow, tip cat (gilli), sling shot (gulel), twigs and
leaves, protruding nails from walls, latches of the door. In fact any relatively sharp object
cause penetrating injury. They are more common in rural set up and in boys. Most of the time
they are uniocular. The overall profile of the organism isolated from post traumatic
endophthalmitis are similar to post surgical endophthalmitis.
71
Fulminating endophthalmitis develops in short period when infected by B Cereus the
organism that has never been isolated in post surgical endophthalmitis. Late onset of
endophthalmitis is generally seen in fungal infection.
Diagnosis of post traumatic traumatic endophthalmitis is not difficult. The presentation
is similar to post surgical endophthalmitis. The difference being disorganization of the globe
due to involvement of multiple ocular structures. The eyes are generally soft, they may have
retained intraocular foreign body. X-ray orbit is required to exclude presence of radio opaque
foreign body. Ophthalmic ultrasonography gives information like position of lens, vitreous
haemorrhage, retinal detachment, presence of foreign body.
Management consists of prompt repair of wounds as per standard method. Construc-
tion of anterior segment. Prophylactic use of broad spectrum antibodies is debatable. Once the
endophthalmitis develops, it should be treated as any endophthalmitis.
Differential diagnosis of post surgical and post traumatic endophthalmitis consists of
endophthalmitis phaco anaphylactica, dislocated lens nucleus, large fragments of cortical
material, unresolved haemorrhage, ciliochoroidal detachment, retinal detachment.
Management of endophthalmitis
I. Endogenous -
1. Supportive treatment by cycloplegic and steroid locally.
2. Systemic antibiotic
3. Systemic steroid under umbrella of systemic antibiotic
4. Systemic antifungal
5. Vitrectomy
II. Exogenous endophthalmitis (post surgical) is an ocular emergency that requires
urgent and intense treatment by way of sub conjunctival and intra vitreal injection of antibiotic
alone or with steroid as per standard dose. Role of vitrectomy is not yet clear.
Panophthalmitis
This is acute purulent infection of all the structures of the eye including Tenons capsule,
extraocular muscles and orbital tissues. Panopthalmitis is generally bacterial in nature, all
pus forming bacteria can cause pan opthalmitis. The organism reach the exterior of the globe
following exogenous endophthalmititis which generally follow intraocular surgery or accidental
penetrating wound. It may take few days for panophthalmitis to develop following penetrating
injury surgical or otherwise. It may also result following perforation of a sloughing corneal
ulcer. Xerophthalmic children and eyes with lagophthalmos are more likely to develop
panophthalmitis. Sometimes the period may be few hours only and starts with malaise,
headache, fever, pain in and around the eye.
On examination the lids are swollen red and hot. The interpalpebral fissure is obliter-
ated. Pus trickles through the lids, the conjunctiva is chemosed and hyperemic. Initially the
cornea is steamy but later on develops purulent keratitis and sloughs, the anterior chamber is
full of pus. Details of this structure beyond the hypopyon is not visible. The whole of the uvea
is smothered with pus and the vitreous is almost a bag of pus, the lens may dislocate. The
globe is immobile and proptosed. Absence of perception of light is the rule.
In post operative eyes pus may exude through the section. It is sometimes difficult to
differentiate between severe endophthalmitis and early panophthalmitis. The condition is to
be differentiated from orbital cellulitis which generally does not involve the globe and vision
is retained. Absence of perception of light and immobile globe is more in favour of panopthalmitis
than endophthalmitis.
Treatment of panophthalmitis is one of the most frustrating things. Broad spectrum
antibiotic may suppress the infection. Visual recovery is almost impossible. The eye when
untreated goes into phthisis after a prolonged course. The main role of antibiotics in
panophthalmitis is to prevent septicemia, meningitis and cavernous sinus thrombosis.
Definitive treatment is evisceration of the eye
Masquerade syndrome
51
This consists of a group of intraocular disease that are not uveitis per se but mimic
uveitis in many ways hence diagnosed and managed as uveitis with disastrous result. They
are seen mostly under 15 years of age in both the sexes, but may be seen as early as first
year of life. Some of them are malignant and potentially life threatening, others may be non
malignant growth or non inflammatory conditions.
Two common malignant disorders are retinoblastoma and myelogenous leukemia.
Medulo epithilioma is a locally invasive tumor, may be malignant. While juvenile xantho
granuloma is a benign dermatological condition that causes spontaneous hyphaema in children.
Intra ocular foreign bodies and peripheral retinal degeneration are two common
non neoplatic condition that may present as uveitis. Other less common conditions are retinitis
pigmemtosa, multiple sclerosis and post vaccination status.
All the above conditions may cause aqueous flare, cells in AC, posterior synechia, cells
in vitreous.
Retinoblastoma is generally seen is children under five years of age with strabismus,
redness, photophobia which on examination shows a posteriorly placed growth or may come
with white reflex in pupillary area, congestion pain. KP flare cells in AC and accumulation of
pus like fluid in AC (pseudo hypopyon) which is infact accumulation of tumor cells, posterior
synechia, nodules on iris, tension is invariably raised. Pseudo hypopyon may disappear and
uveitis subside with local steroid and cycloplegic only to reappear. X-rays shows intera ocular
calcification ophthalmic ultrasonography may outline the tumor and intraocular calcification.
Serum and aqueous LDH may be raised. Aqueous tap may reveal tumor cells.
Leukemia : Acute myeloid leukemia may present as anterior uveitis in children. Spon-
taneous hypheama, heterochromia and glaucoma. Fundus examination may show Roths spot.
Examination of blood gives clue to presence of leukemia. The child is generally ill and pale.
Differential diagnosis consists of all causes of white reflex in pupillary area.
Investigation includes : Routine Hb%, TLC, DLC, ESR, X-ray chest and orbit,
ultrasonography, CT of orbit and MRI.
Management depends upon causative factor. All cases of uveitis in children that im-
proves with cycloplegic and steroid and recur should be suspected to be masquerade syndrome.
New growth of uvea in children
24, 42, 51, 74
New growths of uvea are rare in children, when present they are generally benign or
locally invasive. However, malignant melanoma which is malignant growth of sixth decade
can sometimes be seen in second decade. Such occurrences are extremely rare. However,
malignant melanoma of iris is more common in children than adults. Benign tumor of the iris
are hemangioma, may be associated with angioma of the lid and cause secondary angle clo-
sure glaucoma.
Neuro fibroma of iris is seen as nodule on the iris in association with generalized
neurofibromatosis. They do not require any treatment.
Nevi are common tumors of the iris that make their presence felt just before puberty.
They do not require any treatment, unless they start growing in size or there is change in
pigmentation, which may be sign of malignant change.
Juvenile xanthogranuloma
73
is a benign dermatological tumor seen at the age of
one year. The skin lesions are yellow multiple nodules. There may be visceral involvement.
Ocular lesions are generally associated with cutaneous lesions.
Ocular involvement areIris nodules, spontaneous hyphema, secondary glaucoma
and uveitis. They lesions may involve all parts of the eye. The condition of self limiting.
Hyphema and uveitis are treated with local steroids.
Diktyoma
75
: This is a rare tumor, mostly arising from ciliary body but may arise from
iris, retina or optic nerve. It is a congenital growth, commonest age of presentation is between
two to four years. It is unilateral slowly progressive may remain confined to globe or may
become locally invasive. Metastasis is rare, only occurring when the growth becomes extra
ocular. It is also known as medullo epithelioma because it was thought to arise from medullary
epithelium of fore brain. The term diktyoma is derived from its lace like appearance when it
invades the iris.
76
There are two types of medullo epithelioma - The teratoid and the non teratoid. The
former besides ciliary epithelium contains hyaline, cartilage, rhabdomyocytes, brain tissue
etc. Both types can be benign or malignant. One third of the medulla epitheliomas are
malignant. On histology it shows rosettes similar to retinoblastoma. The tumor is very often
mistaken as retinoblastoma because retinoblastoma is commonest ocular growth in the same
age group. Medullo epithelioma may present as white reflex in pupillary area, secondary
glaucoma and proptosis, which are common features of retinoblastoma as well. However,
incidence of bilateral retinoblastoma is common presenting feature which is extremely rare in
medullo epithelioma.
There is no known genetic predisposition or known family history. Presenting features
depend upon site, duration and location of the growth. It may only be detected accidentally
when the child presents with diminished vision and squint. Otherwise common presenting
features include diminished vision, pain and redness of the eye, whitish reflex in the pupillary
area, buphthalmos and late proptosis.
Diagnosis is difficult, however, unilateral growth without extra ocular involvement,
absence of intra ocular calcification should arouse suspicion of medullo epithelioma. All cases
should be examined with indirect ophthalmoscope. X-ray, CT, MRI, USG may help to
differentiate medullo epithelioma from other disorders.
Treatment
There is no definite treatment
77
for small growth of iris and cilliary body be cured by
wide iridectomy or irido cyclectomy. Eyes with large tumors are best enucleated. Role of chemo-
therapy and radiation are not well established.
REFERENCE8
1. Miller S.J.H. : Parsons disease of the eye, 17th edition, p. 151, Churchill Livingstone,
London, 1984.
2. Nema H.V. : Anatomy of the eye and its adnexa, 2
nd
edition p. 19 to 30, Jay Pee
3. Jogi Renu : Basic ophthalmology, 1st edition, p. 148, Ajeet Publication New Delhi,
1994.
4. Miller S.J.H. : Parsons disease of the eye, 17th edition, p. 6, Churchill Livingstone,
London, 1984.
5. Duke Elders S. : System of ophthalmology, Vol. III, p. 167-184, Henry Kimpton, Lon-
don, 1963
6. Khurana A.K. : Ophthalmology, 2
nd
edition, p. 152-156, New Age International (P)
Limited, 2000, New Delhi.
7. Nancy Anderson Hamming, Apple D. : Anatomy and embryology of eye in Principle
and practice of ophthalmology, Vol. 1, First Indian edition, pp 33-44, Jay Pee,
8. Banumathy S. P. : Anatomy of the eye, 1st edition p. 52, Arvind Eye Hospital, Maduri.
9. Ahmed E : A text book of ophthalmology, First edition, p. 24, Oxford University
Press, Calcutta, 1993.
10. Duke Elders : System of ophthalmology, Vol. III, Part I, pp. 158 to 162, 1
st
edition
Henry Kimpton London 1963.
11. Vaughan D., Asbury T. : General Opthalmology tenth edition, p. 10-11, Lange Medi-
cal Publication, California, 1983.
12. Duke Elders : System of ophthalmology Vol. III part I, p. 36-40 1
st
edition. Henry
Kimpton London 1963.
13. Duke Elders : System of ophthalmology Vol. III Part II, p. 573, First edition. Henry
Kimpton London, 1963.
14. Walton D.S. : Aniridia in Current ocular therapy, 5th edition, p. 508-509, Edited by
Fraunfelder F.T. and Roy F.H., WB Saunders Company, Philadelphia, 2000.
15. Deborah Pavan Langston : Pediatric ophthalmology in Manual of ocular diagnosis
and therapy. Third edition, p. 290.
16. Duke Elders : System of ophthalmology, Vol. III, p. 775, Ist edition, Henry Kimpton,
London, 1963.
17. Nema H.V. : Anatomy of the eye and its adnexa, Second edition, p-150, Jay Pee
18. Biswas J. : Disease of uveal tract in Modern ophthalmology, Vol. 2, Second edition, p.
559, Jay Pee Brothers, New Delhi, 2000.
19. Sorsby A. : Albinism in Modern ophthalmology, Vol. 2, p. 37-39, Butterworth London,
1963.
20. Kanski J.J. : Heriditory disorders of the retina and choroids in Clinical ophthalmology,
2
nd
edition, p. 385-386, Butterworth, London, 1989.
21. Sharma P. : Essentials of ophthalmology, first edition, p. 155, Modern Publication,
New Delhi, 2000.
22. Ahmed E. : Disease of the uveal tract in A text book of ophthalmology. First edition,
p. 227, Oxford University Press, Calcutta, 1993.
23. Miller S.J.H. : Disease of the uveal tract in Parsons disease of the eyes. Seventeenth
edition, p. 157, Churchil Livingstone London, 1984
24. Carolene R Baumal : Granulomatous uveitis in ophthalmology secretes. edited by
Vander JF and Gault JA. First Indian edition, p. 258, Jay Pee Brothers New Delhi,
1998.
25. Debora Pawan Langston : Uveal tract iris ciliary body and choroids in Manual of ocu-
lar diagnosis and therapy, 3rd edition, p. 174-213.
26. Gittinger J.W. : Asdorian G KToxoplamosis in Manual of clinical problems in oph-
thalmology. First edition, p. 147, Little Brown and Co., Boston, 1998.
27. Sharma P. : Uvea and its diseases in Essentials of ophthalmology. First edition, p.
156-175, Modern Publishers, New Delhi, 2000.
28. Coles R.S. : Uveitis in Modern Opthalmology, Vol 4, edited by Sorsby A. First edition
p. 636-686. Butterworth, London 1964
29. Wood A.C. : Endogenous inflammation of the uveal tract, Williams and Willkino,
Baltimore 1961
30. Hogan M.H., Kimura S. J. and Thygeson P. : Signs and Symptoms of uveities I. Anterior
uveitis AJO 47-155, 1959.
31. Tesseler M. : Uveitis in Principle and practice of ophthalmology Vol. 2, First Indian
edition, Edited by Peyman G A, Sanders D and Goldberg FM, p. 1554-1566, Jay Pee
32. Biswas J. : Intermediate uveitis (Pars Planitis) in, Vol. 2, Second edition, Edited by
Dutta L C, p. 571-580, Jay Pee Brothers, New Delhi 2000.
33. Kanski J.J. : Uveitis in Clinical ophthalmology, second edition p. 136-177,
34. Nussenblat R.B. : et al. Standardisation of vitreal inflammatory activity in
intermediate and posterior uveitis. Opthalmology, 92-467-471, 1985.
35. Duke Elders in Systems of ophthalmology, Vol. III part II p. 893-902, Henry Kimpton,
London, 1963.
36. Doughman D.J. : Olson G.A. and Nolan S. : Experimental band Keratopathy, Arch
Oph 81: 264; 1969.
37. Barua C.K. and Dutta L.C. : Corneal degeneration and dystrophies in Modern
Opthalmology Vol. 1, Second edition, edited by Dutta L C, p. 183-184, Jay Pee Broth-
ers New Delhi, 2000.
38. Tran D.B. and Schanzlin D.J. : Corneal and conjunctival calcification in Current of
ocular therapy. 5th edition page 329-330 edited by Fraunfelder F.T., Roy F.H., Randall
J., W. B. Saunders Company, Philadelphia, 2000.
39. OBrat DPS, Gantry D S et al. : Treatment of band keratopathy by excimer photo
keratectomy BJO 77: 702-708 1993.
40. Igerscheimer J. : Spirochaetal infection-Aquired syphilis in Modern Opthalmology
Vol. II, edition second, p. 217-219 Butterworth London 1963.
41. Dutta L.C. : Ophthalmology : Principles and practice, 1st edition, p. 121-122, Cur-
rent Book International, Calcutta.
42. Rosenbaum J.T. and George R.K. : Uveitis in Current ocular therapy, 5th edition p.
519-521. edited by Franfelder F.T. and Roy F.H. and Randall J., W.B. Saunders Com-
pany Philadelphia, 2000.
43. Knox D.L. : Disorders of the uveal tract in Paediatric Ophthalmology, Vol. I, second
edition, edited by Harley R.D., W.B. Saunders Company, Philadelphia 1983.
44. Biswas J. and Wagle A. : Anterior uveitis and arthritis in Modern Ophthalmology.
Vol. 2, Edited by Dutta L.C., p. 566-571, Jay Pee Brothers, New Delhi, 2000.
45. Vaishali Gupta and Gupta A : Ocular toxoplasmosis in Modern Ophthalmology, Vol.
2, edition 2, edited by Dutta L.C. p. 580-585, Jay Pee Brothers New Delhi 2000.
46. Shaffer D B : Paediatric ophthalmology in Text books of ophthalmology. 9th Edi-
tion, edited by Scheic H.G. and Albert, D M, WB Saunders company Philadelphia, 1977.
47. Shalaby I.A. and Dunn J.P. : Acquired and congenital syphilis in Current therapy. Ed
5, Edited by Fraunfelder F.T., Roy F.H., Randell J., p. 2-5, W.B. Saunders Company
Philadelphia, 2000.
48. Wood A.C. : Chronic bacterial infection: Ocular tuberculosis in Modern ophthalmology.
Vol II, first edition, edited by Sorsby A., p. 116-153, Butterworth, London, 1963.
49. Pamela S. Chavis : Tuberculosis in Current ocular therapy, fifth edition, edited by
Fraunfelder F.T., Roy F.H. and Randel J., p. 78-81, W.B. Saunders Company Philadel-
phia 2000.
50. Helm C.J. : Holland G.N. and Ocular tuberculosis, Survey ophthalmology 38 : 229-
256 1993
51. Gittinger J.W. : Herpes simplex keratitis in Manual of clinical problems in oph-
thalmology, first edition, edited by Gittinger J.W. and Asdaurion G.K., p. 46-47, Little
Brown and Co. Boston, 1998.
52. Gillespie S.H. : Ocular toxocariasis in Current ocular therapy, edition 5, p. 97-98,
edited by Fraunfelder F.T., Roy F.H. and Randall J., W.B. Saunders Company Philadel-
phia 2000.
53. Gupta V. and Gupta A. : Pediatric posterior uveitis in Modern Ophthalmology. Vol.
II, edition two, edited by Dutta L.C., p. 561-565, Jay Pee Brothers, New Delhi 2000.
54. Asdourian G.K. : Toxocariasis in Manual of Clinical Problems in Ophthalmology,
first edition, edited by Gittinger J.W. and Asdourian G.K., p. 149-151, Little Brown,
Boston 1998.
55. Duker J.S. : Acquired immune deficiency syndrome in Current ocular therapy. Edition
5, edited by Fraunfelder F.T., Roy F.H. and Randall J., p. 5-7, W.B. Saunders Company,
Philadelphia 2000.
56. Vrabee T.R. and Baldassano V.F. : Ocular manifestation of AIDS in Ophthalmology
Secrets, first edition, p. 267-273, Jay Pee Brothers New Delhi 1998.
57. Faunfelders F.T. : Kaposi sarcoma in Current ocular therapy. fifth edition, edited by
Fraunfelder F.T. Roy F.H. and Randall J., p. 241, W.B. Saunders Company Philadel-
phia 2000.
57A. Spitzberg D.H. : Pars planitis in Current ocular therapy. Edition fifth, edited by
Fraunfelder F.T., Roy F.H., p. 517-519, W.B. Saunders Company, Philadelphia, 2000.
57B. Biswas J. : Intermediate uveitis in odern ophthalmology. Vol. 2, p. 571-579, edited by
Dutta L.C., Jay Pee Brothers, New Delhi, 2000.
57C. Block-Michele and Nussen Blat B. : International uveitis study group : Recommenda-
tions for evaluation of intraocular inflammation diseases. AJO, 103-234, 1987.
57D. Knox D.N. : Disorders of uveal tract in Pediatric ophthalmology. Vol. II, Second
edition, edited by Harley R.D., p. 528-529, WB Saunders Company, Philadelphia, 1983.
57E. Deborah Pavan Langstone : Uveal tract in Manual of ocular diagnosis and therapy.
Third edition, p. 191-196, Lippincott.
58. Dunn J P : Ankylosing spondylitis and Reiters disease in Current ocular therapy,
5th edition, page 555-556, edited by Fraunfelder F.T., Hampton Company, Philadelphia
2000.
59. Roper Hall M.J. : Injuries in Modern ophthalmology. Vol. 3, First edition, p. 442-450,
Butterworth, London 1964.
60. Kanski J.J. : Rare idiopathic specific uveitis syndrome in Clinical ophthalmology,
2
nd
edition, p. 169-170, Butterworth, London, 1989.
61. Duke Elder S. and Perkin E.S. : Disease of the uveal tract in Systems of ophthalmol-
ogy, Vol. 9, p. 558-593, edited by Duke Elder S., Mosby, St. Louis 1966
62. Rao N.A. : Sympathetic ophthalmia in Retina. Editor Ryan NR, Second edition, 1729 -
35, Mosby 1994.
62A Rao N.A., Forster D.J. and Spalton D.J. : The uvea in Text book of ophthalmology.
Editor Podos S.M., Yanoff M., Mosby, 1992
63. Moorthy R.S. and Rao N. A. : Sympthathetic ophthalmic in Current ocular therapy.
Edition 5, edited by Fraunfelder F.T., Roy F.H. and Randall J., p. 320-322. W.B. Saunders
64. Biswas J. : Common Panuveitic entities in Modern Opthalmology, Vol 2, edition II, p.
561- 598, edited by Dutta L.C., Jay Pee Brothers, New Delhi 2000.
65. Moorthy R.S., Inomata H., Rao N.A. Vogt Koyangi : Harada Syndrome. Sur Opthal 39 :
265-292, 1995.
66. Oku H. : Vogt Koyangi Harada Syndrome in Current Ocular Therapy, edition 5, page
293-295, edited by Fraunfelder F.T. Roy FH and Randall J., W.B. Saunders Company,
Philadelphia, 2000.
67. Kone Paul I., Yurdkul S. and Bahabri, S.A. : Clinical features of Behcets disease.
J.Pedia. 134, 721-725, 1998.
68. Masuda K : Behcets disease in Current Ocular Therapy. 5
th
edition, p. 160-161. Ed-
ited by Fraunfelder F.T., Roy F.H., Randall J., WB Saunders Company Philadelphia,
2000.
69. Vinita Singh : Surgical treatment of Strabismus in Modern Ophthalmology, Vol. 2, p.
925, edited by Dutta L.C,, Jay Pee Brothers, New Delhi, 2000.
70. Kottow M.H. : Fluorescein angiography of the anterior segment in Principles and Prac-
tice of Opthalmology, Vol. 2, edited by Peyman G.A., Sanders D.R. and Goldberg
M.F., Jay Pee Brothers, New Delhi, 1987.
71. Agrawal L.P. : Essentials of Opthalmology, first edition, p. 235, CBS Publishers and
Distributors, New Delhi 2000.
72. Verma L., Venkatesh P. and Tewari H.K. : Management of Endothalmitis, CME Series-
4, All India Opthalmology Society.
73. Hunyor A.P. and Roberston J.E. : Bacterial Endothalmitis in Current Ocular Therapy,
edition-5, p. 456-460, edited by Fraunfelder F.T., Roy F.H. Randall J., W.B. Saunder
74. Wheeler D.T. : Juvenile Xanthogranuloma in Current Ocular Therapy, Edition 5, p.
239-241, Edited by Fraunfelder F.T., Roy F.H. and Randall J., W.B. Saunder Company
Philadelphia 2000.
75. Duke Elders and Perkins E.S. : Disease of the uveal tract in Systems of Opthalmology,
Vol. 9, first edition, edited by Duke Elders, Henry Kimpton London 1966.
76. Zimmerman L.E. : A critical reappraisal in light of new observation and current concept
of embryopic tumors. Am. J. Oph. 72: 1039, 1971.
77. Apple D.J. and Pulkin J.E. : Leucokoria in Principles and practice of ophthalmol-
ogy, Vol. II, p. 1181-1182, edited by Peyman G.A. Sander D.R. and Goldberg M.F., 1
st
Indian Edition, Jay Pee Brothers, New Delhi 2000.
78. O Keef M. : Medullo epithelioma in Current Ocular Therapy, Edition-5, p. 249, edited
by Fraunfelder F.T., Roy F.H., Randall J., W.B. Saunder Company, Philadelphia 2000.
CHAPTER 9
Disorders of Lens in Children
GENERAL PRNCPLE
Lens is a unique tissue in the body. It is solely ectodermal in origin. It has only one
function i.e. to focus light on the retina. It is avascular, does not have any nerves hence lens is
devoid of any sensation. It is not capable of getting infection, inflammation and degeneration
or dystrophic. No new growth is known to develop in the lens. For its nutrition it depends
exclusively on aqueous humour in postnatal period. There are only three possible disorders
that can afflict the lens they are
A. Change in transparency
B. Change in shape
C. Displacement.
Disorders of lens are met from intrauterine life to ripe old age.
ANATOMY OF LEN8
1,2,3,4
The lens is suspended between the iris and the vitreous. The lens along with its suspen-
sory ligaments divides the interior of the eye into two chambers, a large vitreous chamber
behind and a smaller aqueous chamber in front. The posterior surface of iris is in contact with
the anterior surface of the lens. The pupil is in front of the central point of the anterior surface
around the anterio posterior axis of the lens. The iris glides smoothly over the anterior surface
of lens in presence of light, accommodation and convergence.
The lens is a disc shaped structure that looks circular when looked from front and back
and elliptical when seen from the sides.
Average diameter of the adult lens is 9.0 mm, at birth it is 6.00 mm to 6.50 mm, its
maximum anterior posterior thickness is 3.50 mm to 4.00 mm in adults. The anterior and
posterior surface differ in curvature, the anterior surface is less curved than the posterior
surface. The anterior surface has radius of curvature of 10 to 11 mm and that of posterior
surface is 6 mm. The curvatures of the lens is variable. In accommodation the curvature in-
creases. It is more curved in infancy and child hood.
290
DISORDERS OF LENS IN CHILDREN 291
The two surfaces merge into each other in a rounded equator. The central part of the
anterior surface is called anterior pole while corresponding posterior point is called poste-
rior pole. The imaginary line joining the two is called axis of the lens. Opacities in the poste-
rior pole and posterior part of axialcortex cause more visual loss than on the anterior pole or
anterior part of the axis, extensive, opacities on the periphery do not cause any visual change.
The normal lens is for all practical purpose crystal clear in childhood. As age advances the
colour becomes yellowish white that gives a false impression of cataract after fifty years of age.
So long the colour of the lens does not interfere with vision it should not be called cataract.
Anatomically the lens has following parts
A. The capsule
B. Epithelium
C. Lens fibres
1. Cortex
2. Nucleus.
D. Zonule
A. The structure commonly referred to as capsule of the lens is not a true capsule. It
is the basement membrane of the under lying. epithelium. It is a homogenous layer without
any cells. It envelops the lens from all sides its thickness is not uniform. It is thickest at the
equator, thinner at the anterior pole and thinnest in the posterior pole. This uneven thickness
is due to metabolism of epithelial cells and amount of direct stress exerted on it. There are no
cells over the posterior capsule, it is supported by the vitreous behind, does not bulge much in
accommodation and force directed by contraction of zonules is minimal. There is a small de-
pression in the anterior vitreous called patelar fossa; the posterior capsule rests in this fossa.
In infancy and childhood there is an ill-defined adhesion between the posterior capsule and
anterior vitreous fossa called capsulo hyaloid adhesion. This adhesion gradually disap-
pears by fifth decade. In children any traction on the lens like attempted intracapsular lens
extraction, repeated needing put undue traction on the vitreous that is transmitted to the
retinal periphery resulting in traction detachment. The capsule has no healing capability of its
own.
B. The lens epithelium is metabolically most active part of lens. It consists of a single
layer of cuboidal cells that are smallest in the centre of anterior capsule and gradually in-
crease in length towards the periphery. The lens epithelium is limited in an area between the
equator and under the anterior lens capsule. The posterior capsule is devoid of any epithelium.
The future growth of the lens depends on the integrity of these epithelial cells. The shape of
epithelium varies from place to place, in the central part they are polygonal, in prequatioreal
area they are cuboidal, at equator they are columnar and gradually become conical with
apex towards the centre of the lens. These cells form the lens fibbers.
C. There are two types of lens fibbers
(1) Primary lens fibres that develop from the posterior layer of the lens vesicle. These
are ultimately converted to embryonic nucleus.
(2) Secondary lens fibres are derived from the equatorial part of the lens epithelium
2
.
The secondary lens fibers keep multiplying through out the life. The older fibers are pushed
towards centre and are converted in to the nucleus. The central fibers are the oldest and
peripheral are most recent.
1. The peripheral soft fibers are called Cortex while relatively hard fibers are called
Nucleus. There is no histological demarcation between the cortex and the nucleus. It may be
said, nucleus of present day is the cortex of yesterday
5
.
2. The nucleus of the lens. The nucleus of the lens is not a separate entity; it is mere
condensation of centrally pushed lens fiber. The oldest fiber are deepest, the number of fiber
keep on increasing throughout the life without much gain in size of the lens. According to
period of development the nucleus has been assigned various names.
They are
(a) Embryonic
(b) Foetal
(c) Infantile
(d) Adult.
(a) The embryonic nucleus is the innermost and earliest to come into existence it is
formed by primary lens fibre, initially there is a circular empty space surrounded by single
layer of cuboidal cells. The posterior cells elongate to obliterate the space, as the posterior cells
elongate the lumen becomes crescent shaped and becomes completely-extinct.
(b) The foetal nucleus develops from the secondary lens fibber over the embryonic
nucleus. The size of the embryonic and foetal nuclei do not change, in life they are pin point in
size.
(c) The infantile and adult nuclei are formed after birth. The infantile nucleus is
completed before puberty, the adult nucleus develops there after. Size of adult nucleus keeps
on increasing without much change in overall dimension of the lens. The lens fiber that are not
converted into nucleus are known as cortex. So child has more cortex and small nucleus while
a person in sixth decade has larger nucleus and scanty cortex. The nucleus not only enlarges in
size it gets sclerosed as the person ages.
The secondary fiber are not long enough to span the lens pole to pole, they do not meet
at a point. The fiber in the same level meet in a peculiar fashion forming a Y shaped figure
called.
Lens suture. There are two sutures the anterior one is an erect Y while the posterior
one is inverted Y. The sutures start forming in the first two months and seen in foetal life. The
advantage of these sutures are that they give an elliptical shape and better optical property to
the lens.
(d) The zonules are entirely different from the lens, developmentally and functionally,
their only purpose is to keep the lens in the papillary area and help change its curvature
during accommodation. The zonules are infact tertiary vitreous. They extend from the api-
ces of the ciliary process to the capsule. There are three groups of zonule. The anterior,
middle and posterior attaching to anterior, middle and posterior part of equator respectively.
The anterior zonule extends for a few millimetres on the anterior lens capsule. The posterior
zonule may extent from oraseratta to equator of the lens.
The tunica vasculosa lentis. The postnatal lens is avascular and gets its nutrition
solely form aqueous. In first half of the gestation the lens gets nutrition from a rich framework
of vessels that envelop the lens all round and is called tunica vasculosa lentis.
Developmentally, it has two parts; the anterior vasculosa lentis and posterior vasculosa lentis.
The anterior is a part of pupillary membrane while the posterior is formed by the terminal
branches of hyaloid artery which is part of ophthalmic artery and enters the eye through the
foetal fissure, it covers the developing posterior capsule and the equator. The tunica vasculosa
lentis starts regressing even before the whole of the lens has formed. By fifth month it is fully
atrophied.
DEVELOPMENT OF THE LEN8
The lens is ectodermal in origin. Its presence becomes evident at 4mm stage
7
, when
the optic vesicle comes in contact with the surface ectoderm. This contact between two surfaces
triggers thickening in the surface ectoderm called the lensplate, at the same time another
important change takes place in the optic vesicle that starts inveginating to form the opticcup
a two layered structure from which the retina develops. The cells of lens plate elongate and
increase in number and the single layered structure instead of spreading laterally bows inside
forming a pitcher like structure, the mouth of which gradually narrows and by 8mm stage it
closes completely and separates from the surface ectoderm. The surface ectoderm closes to
form the subsequent corneal epithelium. The closed single layered structure separated from
the surface ectoderm is called lens vesicle that starts moving away from the surface ectoderm
towards the optic cup in the space between the two will later develop cornea proper, iris and
AC.
There is difference in development of cell forming the anterior and posterior walls of the
lens vesicle, the anterior cells remain cuboidal while the posterior cells elongate forward to
obliterate the space of the lens vesicle resulting into a solid globular structure called embryonic
lens nucleus. The lens capsule that is a true basement membrane starts forming and by
13mm stage, the capsule envelops the growing lens fully.
After formation of embryonic nucleus, formation of new lens fibber is shifted to the
equatorial region . These fibber are called secondary lens fiber. As the fibers are formed the
nuclei of elongated cells disappear this is one of the causes for clarity of the lens.The fibber
meeting at the centre of the core form the anterior and posterior Y sutures.
As more and more fibers are formed at the equator, the earlier fiber are pushed cen-
trally and compressed to form various nuclei : the foetal , infantile and adult nucleus.
PHY8OLOGY OF THE LEN8
8,9,10
Lens is avascular, depends upon aqueous for its nutrition. It has both anaerobic and
aerobic metabolism. The lens contains 65% water and 35% protein, the highest concentration
of protein in any tissue. Ninety percent of protein is water soluble, they are devided into three
types alpha , beta and gamma crystalline and remaining portion is insoluble albunoid. The
concentration of insoluble protein increases with age. In cataract almost all the protein is
converted into insoluble albunoid. The lens protein is organ specific and not species specific i.e.
a body sensitised to lens protein will behave adversely later if exposed to lens protein of any
species. This has an important bearing in lens induced uveities and endophthalmitis
phacoanaphylactica.
The lens has a good concentration of ascorbic acid which is synthesised by the lens and
ciliary epithelium. It also contains glutathion both in oxidised and reduced form. The amount
of ascorbic acid and glutathion decrease with age and cataract formation. The transparency of
the lens depends greatly on its metabolism and physiochemical status of protein.
CONGENTAL ANOMALE8 OF THE LEN8
Congenital anomalies of the lens are common but all need not be treated as many are
asymptomatic.
Anomalies that involve embryonic and foetal nuclei are true congenital and others
are called developmental defects.
Some of the congenital anomalies are obvious at birth, others are detected late due to
associated diminished vision, squint and nystagmus.
A. The congenital anomalies may be :
1. Limited to Lens
2. Associated with other anomalies of the eye
3. Associated with systemic disorders
4. Combination of all above
B. Congenital anomalies can be :
1. Loss of transparency
2. Change in shape of the lens
3. Change in position
4. Various combination of above three.
B. Congenital anomalies can be
1. Congenital loss of transparency. Loss of transparency complete or partial is clini-
cally known as congenital or developmental cataract. They are in the form of opacities.
The opacities that involve embryonal and foetal nuclei are called congenital while rest
excluding traumatic are developmental. Many a times it is difficult to differentiate between
the two. The congenital opacities are generally central, dens and may be large enough to cover
the entire papillary area and, are generally soft. Congenital and developmental cataracts are
often bilateral but can be unilateral. The cataracts in childhood are frequently stationary be-
cause the cataractogenic factor effects a particular zone of developing lens, the lens fibers laid
before or after the stoppage of cataractogenesies remain clear, 25% to 30% of cataracts are
hereditary how ever some time they may be sporadic.
Etiology
Etiology of congenital and developmental cataracts are not well understood it can be:
(i) Idiopathic in 40 % to 50 % of cases. These are generally sporadic
11
.
(ii) Heredity one third of all congenital and developmental cataracts are
hereditary.
12,13,14,15
due to genetically determined chromosomal anomaly.
(iii) Maternal
Maternal infection during pregnancy commonest virus resulting in cataract
is Rubella, others are cytomegalo inclusion virus, rubeola, mumps influenza.
Toxoplasmosis is yet another cause of congenital anomaly if acquired during late
months of pregnancy maternal syphilis can also cause congenital cataract.
13
Maternal malnutrition specially fat soluble vitamins.
Drugs : many of the drugs if taken during first trimester result in foetal cataract
i.e. thalidomide, steroids.
Maternal radiation during pregnancy.
Endocrine disturbance.
12
(iv) Foetal
Birth Trauma
Placental haemorrhage leading to poor oxygen supply to foetus.
Metabolic disorders
(i) Foetal
(ii) Infantile
Various syndromes
Prematurity
Persistence of anterior hyloid system
Intra ocular tumours.
CLA88FCATON OF CONGENTAL AND DEVELOPMENTAL
CATARACT
It is difficult to have a uniform, universally accepted classification. A broad classifica-
tion
16
is dividing these cataracts into the broad categories as :
A. Capsulo lenticular (Capsulo cortical)
B. Lenticular
Congenital cataract can be either nuclear or cortical. It is not uncommon for both to
exist in the same eye. It is frequent for nuclear cataract to become total cataract.
A. The capsulo lenticular cataracts are
1. Anterior polar and anterior capsular cataract.
2. Posterior capsularMittendorf dot. posterior lenticonous.
3. Posterior polar cataract.
B. The lenticular cataracts are
1. Zonular
(a) Lamellar
(b) Total Cataract
(c) Cataracta central pulverulenta.
(d) Membrenous
2. Sutural
3. Axial
According to shape the catracts can be :
1. Disciform
2. Coraliform
3. Coronary
4. OthersCorkscrew cataract, spear shaped cataract, Christmas tree cataract, blue
dot cataract.
A. The Capsulo lenticular cataracts
1. Anterior Polar and Anterior Capsular Cataract. Sometimes these are described
separately but as their clinical presentation, pathogenesis and management are similar they
are being discussed in one group.
Anterior polar cataracts are common but not detected unless specifically looked for
under magnification, are generally bilateral and do not block the pupil due to their size that
may be pin point to 1 to 2 mm in diameter. They are generally small slightly raised plaque,
may project in anterior chamber as a triangular growth with base on the centre of the anterior
capsule and called pyramidal cataract they are of two type:
(a) Developmental due to delayed separation of anterior lens capsule from the surface
ectoderm.
(b) Acquired. This occures frequently in badly managed ophthalmia neonatorum result-
ing in central perforation, loss of A.C., prolonged contact between anterior lens capsule and
cornea. These are generally bilateral and associated with nystagmus. Sometimes there may be
localised opacification of anterior lens capsule resulting into true anterior capsular cataract.
More frequent are reduplication or imprint cataract where a second opacity develops little
below the anterior polar cataract with a clear zone in between, the size of the reduplication
cataract is either same as the base of the pyramidal cataract or larger. Sometimes there
may be a third imprint still posteriorly. Occasionally the two opacities may be joined by a
strand of opacity traversing the clear zone in between. The anterior polar cataract may be
associated with persistent pupillary membrane or central corneal opacity. The opacities are
stationary and do not cause much visual disturbance, hence do not need any treatment.
2. Posterior polar and posterior capsular cataracts. Posterior polar and posterior
capsular opacities are as common as their anterior counterpart. There are two forms of posterior
polar cataracts, one commonly known as Mittendorf dot. This is formed due to incomplete
absorption of anterior part of the hyaloid artery that supplies nutrition to the lens during
first three months of gestation. Some times a small stalk of vessel may remain attached to the
lens. This condition is stationary and cause visual disturbance due to its proximity to the nodal
point of the eye, how ever it is generally discovered and diagnosed during routine retinoscopy
or direct ophthalmoscopy. It does not require any treatment. The other progressive form
causes great visual loss as the opacity increase in size after birth by developing opacities in the
posterior cortex either as a sheet of opacity or radiating opacities from a central opacity. The
condition is not known to involve the nucleus
16
. If the opacity is dense enough it causes
diminished vision, and amblyopia it should be operated by any standard microsurgical
procedure.
CATARACT8 NVOLVNG THE NUCLEU8 AND DEEPER LAYER8 OF
CORTEX
Most of these cataracts are developmental and have been put under category of lenticu-
lar developmental cataract
2,16
.
The commonest in the group is lamellar or perinuclear also refereed as zonular
cataract.
1. Lamellar cataract is commonest congenital cataract, it amount for about 50% of all
developmental cataracts. It is generally bilateral, symmetrical and stationary, boys are more
effected than girls, it may be prenatal or postnatal. There is a rough measurement to find out
whether it is prenatal or postnatal which says that if the opacity is smaller than diameter of
lens of new born i.e. 5.75 mm it is most probably prenatal otherwise post natal. Actual size
varies according to on set of cataractogenesis and level of lens fibres involved. It most com-
monly develops in foetal and infantile nuclei in lamellar formation, the lamellae may be single
or multiple. The opacity inside and out side the affected nucleus are clear. The opacity may be
fully opaque or may be translucent. On the surface of the opacity many ridges are visible on
oblique illumination. These ridges radiate from the centre and vary in number they may be
limited at the periphery of the cataract or may project in the clear cortex with club or spine
shaped projections called riders.
Most of the lamellar cataracts are hereditary, transmitted as autosomal dominant trait
may be present in parents or in siblings. The sporadic cataracts are generally due to derange-
ment of calcium metabolism in the mother with vitamin D deficiency during growth of the
lens, the opacity develops in a layer that corresponds with maximum level of noxious sub-
stance. The fibres laid before and after this cataractogenesis are clear.
On examination the opacity may cover whole of the pupillary area, on dilatation a clear
zone of the cortex is visible both on retinoscopy and ophthalmoscopy.
Treatment depends up on size and density of the cataract. Translucent lenses with good
vision need no treatment, some may benefit with dilatation of the pupil. Otherwise the treat-
ment is removal of opacity by standard microsurgical method preferably with P.C.I.O.L.
2. Congenital nuclear cataract. This type may be confined to the embryonic nucleus
due to changes at 3 months gestation. These are dens, powdery hence called pulverulent
cataract which are generally bilateral, non progressive with out much visual loss.
The other type is total nuclear cataract that may involve infantile, foetal or embry-
onic nuclei. The condition is bilateral, symmetrical and stationary may hamper vision.
3. Other cataracts
(a) Sutural Cataracts. These are discrete opacities in the Y sutures of the embryonic
nuclei. They are generally bilateral and stationary. One or both the sutures may be involved
but need not be symmetrical. A type of sutural cataract seen in premature children are known
to fade by third month post natal
19
. Other variants are : Floriform, (Coralliform) and ante-
rior axial cataract.
(b) Blue dot Cataract. This is very common congenital anomaly of lens. The opacities
of various sizes, some of them visible only with slit lamp, others may be visible with bright
oblique illumination. The opacities are scattered in the centre of the lens. They are not really
coloured blue, they are white but due dispersal of violet light
20
they look blue. The opacities do
not hamper vision, do not cause any complication hence do not require any treatment.
(c) Membranous Cataract. These are not separate type of cataracts. They result due
to absorption of soft cortical matter in congenital cataract of long standing spontaneously or
following trauma . The two capsules come close to each other with some opaque lens fibres in
between without nucleus. They generally do not require any surgery but thick membrane may
be needled folled by standard correction of aphakia.
RUBELLA CATARACT
Rubella cataract is caused by systemic infection of the mother by rubella virus in first
trimester. Infection acquired after first trimester have very less chances of causing congenital
defects in the foetus and subsequent pregnancies are not effected by the earlier infection. The
rubella virus belongs to a group of toga viruses and has single antigenic type
21
. The virus is
found all over the world with pockets of endemic areas, and can be prevented by prophylactic
vaccine given in school going age. There is no antiviral agent known to be effective against
rubella virus. The virus passes to the developing foetus via placenta and affects organogenesis
of multiple organs, main being eyes, ear and heart. The infected mother has 50% chances of
having child with congenital anomalies half of them will have ocular involvement
12
. The systemic
manifestations in infected mother are mild and passed off as common cold with enlargement of
occipital lymph glands.
In utero the virus causes cells to have increased doubling time and short survival time.
The growing lens develops areas of necrosis that loose transparency.
The rubella cataract is central dense opacity to begin with, which becomes pearly and
spreads to cover whole of the lens and may loose fluid later to become membranous. Hence in
all cases of congenital membranous cataracts possibility of rubella should be kept in mind. The
virus remains alive in the lens for months, may be as long as three years
22
and when liberated
in aqueous following surgical or accidental trauma is likely to cause sever and long lasting
uveitis that it self may be sight threatening.
Congenital rubella cataract is part of a syndrome of triad that consist of
A. Congenital cataract
B. Neurosensory hearing loos
C. Congenital heart disease.
Besides cataract there are other congenital anomalies of the eye they are :
Microphthalmos, rubella retinopathy, iris hypoplasia, transient corneal haze.
Congenital glaucoma is a common feature with congenital rubella cataract but is
independent of pathological process of cataract formation.
The systemic manifestation besides heart disease and neurosensory hearing loss are
many. The neurosensory hearing loss is not always present at birth but may develop in pre-
school age. Bone lesions, hepatitis, thrombocyotopaenic purpura, hemolytic anaemia and occa-
sional central nervous system involvement are infrequent systemic manifestation. Congenital
rubella is known to cause still birth, prematurety and mental retardation making the manage-
ment more difficult.
D. Management of rubella Cataract
1. Prophylaxis in mother : Rubella is fully preventable disease following rubella vac-
cination between 12 to 14 years of age. If rubella vaccination is made part of universal immu-
nisation the scourge of rubella syndrome with its morbidity and mortality can be abolished
altogether.
2. There is no known medical treatment for the infected mother or child..
3. Only definitive treatment of congenital cataract of (a) Surgical removal of Cataract
(b) Management of aphakia (c) Prevention and treatment of amblyopia, squint and nystagmus
(d) Associate glaucoma requires separate management.
CONGENTAL AND DEVELOPMENTAL CATARACT8 DUE TO NBORN
ERROR8 OF METABOL8M CON88T OF
A. Galactosaemia
B. Hypoglycaemia
C. Diabetes mellitus
D. Homocytinuriaand other amino aciduria (Lows Syndrome)
E. Hepato lenticular degeneration (Wilsons disease)
F. Hypoparathyroidisionm.
A. Galactosemia
Galactosemia is one of the inborn errors of metabolism that causes developmental
cataract. Galactose is not available separately as source of food. It is a metabolic product of
lactose which is an important carbohydrate content of mammalian milk. The other source
being endogendus in form of neurolipids in small quantity. The lactose is hydrolyzed in to
glucose and galactose in the intestine, galactose itself is converted into glucose. In galactocemia
this conversion of galactose to glucose is hampered due to deficiency of one of the three enzymes
required to complete the metabolic cycle. A sugar alcohol that is responsible for cataract
formation is formed that on entering the lens fibres cause a state of hypertonicity which attracts
water to neutralise the alcohol leading to swelling of lens of fibbers, and opacification of the
lens.
The three enzymes involved in galactose metabolism are Transferase, galactokinase
and epimerase.
Commonest being transferees deficiency the epimerase deficiency is extremely rare.
Transferase deficiency galactosemia is so frequent among other galactosemics that
it is called Classical galactosemia The ocular components consists of bilateral central
cataract also known as oildropelet cataract due to its appearance. The first change may
be posterior lenticonous that on examination under slit lamp within few days of birth, show
refractile opacities in the centre of the lens. Concentric refractile rings may develop round the
central opacity. The opacity may initially look like a zonular opacity with clear zone all round.
The opacity spreads rapidly to cover whole of the lens within few months. The progress of the
opacity can be stopped or altogether reversed if milk and milk products are fully eliminated
from the infants feeds.
The systemic changes are serious and even life threatening they consists of vomiting,
diarrhoea hepatospleenomegaly, hepatic and renal dysfunction, cirrhosis, anaemia,
failure to gain weight, mental retardation.
Glactokinase deficiency galactosemia is rarer than the former it is more benign
26
has no systemic changes. The infant is healthy, the cataract develops in first few years of
life. The cataracts are central and bilateral, their natural history is similar to transferase
deficiency cataract except that kinase deficiency cataract is slower than the former.
1. Diagnosis of galactose cataract consist of
(a) Clinical examination of the new born with diarrhoea, vomiting, dehydration and
hepatio spleenomegaly
(b) Examination of urine for reducing substances.
(c) If reducing substances are present the urine should be examined for osazone crys-
tals which are absent in galactosemia to exclude diabetes. The urine is subjected to chroma-
tography for specific band denoting galactosemia, other specific tests consists of
(d) Estimation of urinary and serum galactose.
(e) Assay for uridyltranasferase in peripheral red cells.
(f) Urine examination for albumin and aminoacids.
(g) Hepatic function test.
2. Management. As soon as it is conformed that the child has galactosemia all mamma-
lian milk products are withdrawn from the diet. This may retard or reverse the opacity. If
opacity persists it is managed by any of the standard procedure.
B. Hypoglycaemic cataract
Hypoglycaemic Cataract in infant is a cataract developing in a hypoglycaemic infant or
child. Hypoglycaemia in new born can result due to various causes in the infant. Generally an
infant of a diabetic mother without biochemical control of hyperglycaemia, produces sufficient
insulin to ward off foetal hyperglycaemia but as soon as the child is born and cord separated
this increased insulin present in the child results in sudden lowering of blood sugar leading to
acute hypoglycaemia which if not corrected cause formation of lens opacities which may not be
detected at birth and may disappear if hypoglycaemia is corrected. Repeated attacks of
hypoglycaemia lead to lamellar cataract by 1 to 2 years of age. Some neonates may be
hypoglycaemic without mother being diabetic, incidence of hypoglycaemia is more in prema-
ture infants. Deprivation of sugar for first forty eight hours also result in hypoglycaemia,
repeated diarrhoea is another cause of hypoglycaemia all these condition may precipitate
hypoglycaemic cataract which is reversible if treated well in time.
C. Diabetic cataract
Diabetic cataracts are not seen in new born they are generally met with in juvenile
diabetics. It is an acute cataract due to accumulation of glucose in the lens. The accumulated
glucose is converted into sugar alcohol which imbibes water and over hydrates the lens ,
these cataracts are bilateral. To begin with they are snow flake like opacities under the
capsule, diabetic cataract has been seen in children as young as one year. Initially these
cataracts are reversible if water electrolyte and metabolic disorders are corrected within first
two or three days. Management of diabetic cataract is like any other cataract of childhood done
with proper control of hyperglycaemia.
D. Cataract of childhood in miscellaneous errors of metabolism
26,27,28
1. Hypoparathyroidism. Bilateral lens opacities in the shape of punctate opacities
and multicoloured crystals in the cortex are seen in untreated cases of long duration of
hypoparathyroidism. Progress of cataract can be prevented by treatment of hypoparathyroidism.
2. Manosidosis. Spots like opacities in the posterior capsule is caused due to deficiency
of mannosidase.
3. Fabrys Disease. Caused due to lac to galactosidase. It results in spoke like opaci-
ties in cortex without disturbance of vision.
4. Lowes syndrome. Also called oculo cerebro renal syndrome effect mostly boys. Cause
congenital cataract associated with congenital glaucoma. The lens is microphakic. Lens
opacities may be capsular, lamellar, nuclear or total, these children have mental retardation.
5. Wilsons disease (Hepatolenticular degeneration) This is due to inborn error of cop-
per metabolism. Lenticular changes cause anterior capsular sunflower cataract.
6. Homocystinurea (see ectopia lentis)
CHANGE N 8ZE OF THE LEN8
Microspherophakia Normal diameter of an adult lens is 9 mm and that of new born is
5.75 mm. The edges of the lens are not visible even with maximum mydriasis. The infantile
lens gradually increases to adult size in the second decade. There is no condition where the
lens has congenitally larger diameter Microspherophakia
29
is a congenital anomaly of the
lens where the average diameter of the lens is 6.75 as against 9mm of an adult lens. The
anterior posterior diameter of the lens is 25% more than normal. Reduced equatorial diameter
and increased thickness of the lens gives it an appearances of a spherical lens with small
diameter hence the name microspherophakia. The condition is bilateral. The child is brought
due to diminished distant as well as reading difficulty. The cornea is of normal size so is the
globe. The anterior chamber may be deep on the periphery with mild iridodonesis the real
diagnosis is made on dilatation of the pupil. With a widely dilated pupil the periphery of the
lens gives a crescent like reflex. On retinoscopy the central part shows high myopia with or
without astigmatism. On slit lamp examination the zonules are visible all round in early
years but later on there may be rupture of some of the zonular fibres, resulting is subluxation
which is common. A peculiar phenomenon called glaucoma inverse results if the pupil is
constricted either due to prolonged exposure to bright light or use of miotic. The spherical lens
obstructs the miotic pupil resulting in pupillary block and rise of tension. There may be
associated angle closure. The tension falls with mydriasis. The lens may occasionally come in
the anterior chamber. Generally there are no other congenital anomalies of the globe. The
condition may have only ocular component or may be associated with systemic anomalies
resulting in Weill-Marchesani syndrome. The exact cause of the deforming is not known. It
is presumed that it results due to arrest of development of lens during 5th or 6th month of
gestation.
Management of the condition is difficult due not only to index myopia, poor
accommodation but also secondary pupillary block glaucoma. Besides correction of index myopia
a constant watch should be kept on possibility of pupillary block. Prophylactic iridectomy or
iridotomy, may prevent rise of tension, use of mydriatic is better avoided because the lens may
subluxate in the A.C.. Cataract is common and should be managed like any other cataractus
ectopia lentis.
CONGENTAL ANOMALE8 OF 8HAPE OF THE LEN8
There are two types of anomalies of shape of the lens due to congenital cause.
A. Lenticonus.
31
B. Coloboma of the lens.
A. Lenticonous is a condition where the poles of the lenticular capsule bulge out side the
confines of the capsule. If the projection is anterior where the lens bulges in the anterior
chamber the condition is called anterior lenticonous and when similar changes appear in
the posterior capsule with bulge extending into the vitreous the condition is known as poste-
rior lenticonous. The terms lenticonous and lentiglobus are used as interchangable terms.
Posterior lenticonous is more common. The cone is situated within 5mm of the posterior
pole, however it can be eccentric or peripheral. There is a very rare condition where the poste-
rior cortex develops a local bulge with in the confines of the capsule
32
.
The anterior lenticonous are bilateral while posterior lenticonous are unilateral. Changes
incurvature of lens is seen equally in both sexes. The exact cause is not known. There may be
a history of lenticonous in the family lenticonous may be associated with many systemic
congenital anomalies.
The condition may not be diagnosed unless the child is brought for improvement of
vision or correction of squint which is common. The cause of diminished vision is curvature
myopia. amblyoipia is common.
On examination there is an oil drop appearances. with dilated pupil The change in
curvature is visible on slit lamp. On retinoscopy there is central myopic and peripheral
hypermetropic refraction, scissors movement are common. The lens shows progressive opacity
in the posterior cortex in the cone and around the cone. Combination of posterior lenticonous
and posterior cortical cataract is so common that all cases of unilateral progressive opacification
of posterior cortex in an eye with normal dimension should be investigated for possibility of
posterior lenticonous. If the media is dense and the bulge is not visible on slit lamp, U S G may
delineate the bulge.
Management. If there is not much of visual loss, spectacles, contact lens and mydriatics
my help. However danger of amblyopia is always present that should be managed as per stand-
ard methods. Dense opacities can be managed either by limbal or pars plan route with poste-
rior capsulotomy anterior vitrectomy and IOL.
B. Colobama of the lens
Coloboma of lens depends upon development and integrity of the zonules. In what is
called coloboma of lens, there is no actual loss of substance of the lens matter as the name
suggests. A notch in the lens is very rare. There is flattening of the circumference. Generally it
is situated at down and in position, a site common for uveal coloboma. The coloboma is
generally not visible if the pupil is circular and requires myriasis to be visible. On slit lamp
examination zonules are generally absent at the site of coloboma. Coloboma of the lens is
usually unilateral, the lens is generally partially cataractous, adjacent to an area of
coloboma. It may involve the cortex or adult nucleus and not embryonic or foetal nuclei. As the
area of coloboma is deprived of normal pull of the zonule, it is thicker and the periphery is
more rounded than normal lens.
33
The lens generally has curvature myopia with astigmatism
and poor power of accommodation. Occasionally there may be a dent in the lower equator
34
.
The condition is to be differentiated from subluxation of lens the only difference be-
tween the two is that colobomalous lens has flatter periphery while subluxaled lens retains
usual circular curvature of the equator.
One of the complications of colobomatous lens is subluxation. Coloboma of the lens does
not require any specific treatment.
CONGENTAL ANOMALE8 OF ZONULE
Congenital anomalies of zonules are not uncommon but they are generally not discussed
as separate entity simply because the result of such anomaly are reflected as anomalies of the
vitreous. The anomalies can be 1. When the zonules are weak alround, they result in
speherophakia. 2. When the defect is localised the result is a coloboma of the lens, where
the lens does not subluxate 3 when the defect is excessive on one side the lens gets sub luxated
to the opposite direction 4. if the zonules are lacking all-round the lens is dislocated.
CONGENTAL ANOMALE8 N THE PO8TON OF THE LEN8
A. Ectopia lentis
Next to cataract, displacement of lens is most common congenital anomaly of the lens. It
may be mild enough to be missed unless examined with slit lamp under complete mydriasis.
The congenital displacement is generally bilateral and nearly symmetrical most of the
time the displacement of the lens in the one eye is mirror image of the fellow eye.
1. A lens is said to be ectopic when
(a) Its centre is not in the anterio posterior axis of the eye.
(b) Some of the zonule are still attached to the lens.
(c) The lens is behind the iris.
(d) Partly visible in the pupil.
(e) The edge of the lens in the pupillary area dividing the pupil into two parts:- one
phakic which is generally myopic and the other area aphakic that is hypermetropic.
2. A lens is sub luxated when some of the zonules are still attached to the lens capsule.
3. A lens is dislocated when all the zonules are severed from the lens and the lens
can move in the
(a) Anterior chamber
(b) Incarcerted in the pupil
(c) Bobbling in the vitreous
(d) Fall at the bottom of the vitreous
(e) Get fixed to the retina.
Unilateral ectopia is rarely congenital it is mostly due to trauma or other ocular causes
like chronic uveits . In all cases of unilateral ectopia lentis the other eye should be carefully
examined for subtle signs of ectopia, and systemic cause of ectopia lentis ruled out.
B. Symptoms of ectopia lentis depend mostly on
1. Area of the lens present in the pupil,
2. Tilt of the lens,
3. Transparency of the subluxated lens
4. Associated error of refraction and
5. Complications like amblyopia, glaucoma, retinal detachment.
6. Associated systemic condition.
C. The symptoms can be divided into
Mild, moderate, gross and associated complications.
1. A lens that has subluxated but its edge lies in between the pupillary margin and iris
root may not produce any visual symptom, may have normal accommodation of the age. Occa-
sionally there may be diminished vision due to errors of refraction independent of subluxation,
or due to subluxation i.e. myopic astigmatism.
2. A lens that divides the pupil in two halves, one aphakic and other phakic causes
moderate visual symptoms of unilateral diplopia and fleeting visual disturbances. The phakic
part is myopic or myopic astigmatism with accommodation. The aphakic part which is
hypermetropic and has both diminished distant as well as near vision. Bilateral moderate
subluxation may cause polyopia.
3. The edge of a subluxated lens that is not visible in undilated pupil causes gross loss of
distant as well as near vision without diplopia because the whole of the pupillary area is
uniformly absolute hypermetropic.
4. Ectopia lentis with associated complication have various grades of visual loss due not
only to ectopia but also associated lesions like glaucoma, retinal detachment etc. (See below)
D. Signs of Ectopia lentis consist of
1. Diminished distant vision that may or may not be fully corrected by glasses or contact
lens.
2. Diminished near vision
3. Uniformly or partially deep A.C.
4. Generalised or localised tremulousness of iris
5. Pupillary shape and size are variable, depending upon other associated and anoma-
lies.
6. The edge of the lens may give a crescent shaped reflection on retinoscopy; the phakic
area is myopic whereas the aphakic area is hypermetropic.
7 On fundus examinationdoubling of the fundus i.e. a large area seen through the
phakic myopic area and a small area of fundus seen through hypermetropic area.
8. The refraction may not be symmetrical and equal in both the eyes.
9. The eye invariably has strabismus in unilateral cases and also when the refraction is
grossly different in both the eyes.
10. On slit lamp examination the zonules may be visible in the aphakic area and vitre-
ous may herniate though the aphakic area.
11. Complication
(a) Raised intra ocular tension
(b) Myopic degeneration
(c) Rhegmatogenus retinal detachment
12. Associated anomalies
(a) Mesodermal dysgenesis of anterior chamber
(b) Megalocornea.
(c) Cornea plana
(d) Persistent primary hyperplastic vitreous
(e) Retinitis pigmentosa
(f) Congenital glaucoma.
(g) Keratoconus
(h) Ectopic pupil
(i) Aniridia
(j) Ptosis.
13. Associated systemic anomalies
(a) Marfans syndrome.
(b) Homocytinuria.
(c) Weill Marchesani syndrome
(d) Reigers anomaly
(e) Crouzons syndrome
(f) Oxycephaly
(g) Hyperlysinemia
(h) Sulphite oxidise deficiency
(i) Ehlers - Danlos syndrome
(j) Mandibulo facial dysostosis.
(k) Sturge Weber syndrome.
8MPLE ECTOPA LENT8
Simple ectopia lentis represents isolated ectopia without any other ocular or systemic
disorder. It is generally familial, transmitted as autosomal dominant inheritence.
35
It may
rarely be sporadic, the lens is subluxated up and out. On set of subluxation is gradual and
may go unnoticed unless looked for specifically in a child with positive family history or the
child is brought with complaints of diminished vision or squint. They generally have good
distant as well as near vision for many years but may develop anterior dislocation, glaucoma,
cataract or retinal detachment
36
.
ECTOPA LENT8 et PUPLLAE
37
This is transmitted as autosomal recessive inheritance, is bilateral symmetrical, the
normal pupil is replaced by a slit like or oval opening complete with constrictor and dila-
tor muscles, the pupil is shifted opposite the shift of the lens i.e. if the lens is displaced up and
out , the pupil is shifted down and in. The edge of the pupil generally bisects the pupillary slit.
The angle of anterior chamber is clear. The condition may be considered as mildest form meso-
dermal dysgenesis.
MARFAN8 8YNDROME
38,39,40
Marfans syndrome is an autosomal dominant disorder of mesodermal tissue with multi
systemic involvement. It is seen equally in boys and girls with world wide distribution.
Though the condition beings in infancy its full blown presentation is felt in teens. It has been
reported to run in generations and common among the siblings. The disorder does not have
any biochemical deficiency that may be linked to the anomaly. The syndrome has
following component, all of which need not be present simultaneously:
A. Ocular
B. Skeletal
C. Cardiovascular
D. Miscellaneous
A. Ocular
Most important, frequent and prominent ocular changes are seen in the lens. The changes
are bilateral almost symmetrical.
1. The lens. The lens is subluxated upwards in both eyes; they are mirror images of
each other. The subluxation is gradual but does not progress much. Occasionally the subluxation
may increase and the lens may even dislocate apparently without any reason. Common pre-
disposing factor being trivial blunt injury, subluxation may occur at any age. The lens is gen-
erally clear but may have localised opacification at the lower equator. Generally the lens is
slightly smaller in diameter it may be microspherophakic. It is usual for patient to have nor-
mal accommodation for the age but if the subluxation is more i.e. the aphakic portion covers
lower half of the undilated pupil, the child has difficulty in near work. The subluxated lens
gives a crescentic dark reflex on oblique illumination due to reflection of light from rounded
edge of the clear lens.
2. On retinoscopy the phakic area generally gives a compound myopic astigma-
tism while the aphakic portion has hypermetropic refraction generally less than +10D.
3. On ophthalmoscopy duplication of fundus is seen. The fundus seen from the phakic
area is generally larger while seen through the aphakic area is smaller than emmetropic fundus.
The eyes commonly have axial myopia with corresponding myopic fundus changes.
4. On slit lamp examination the zonules are visible some of them may be broken, the
lens rarely shows a scalloped lower border or a notch.
5. The anterior chamber is irregularly deep, deeper over the aphakic part and shal-
low or normal over the phakic part. The lower iris is generally tremulous, occasionally there
is phacodonesis.
6. Pupil. The pupil is smaller than normal due to poor development of dilator fibbers.
The pupil is resistant to usual mydriatic and cycloplegic. The pupil retains normal light and
near reflex.
7. Iris. The surface of the iris is relatively smooth.
8. Angle of A.C. Gonioscopy shows wide angle with multiple changes that include ill
defined Schwalbes line.
9. Cornea. The cornea is normal but may show megalo cornea, keratoconous or even
cornea plana.
10. Sclera. The sclera is thin and stretched may have small staphylomas.
11. Error of Refraction. If there is gross difference in refraction between the two eyes
there may be squint and ambloyopia, rarely their may be ptosis.
12. Glaucoma: Eight percent of the eyes have developmental glaucoma that is gener-
ally open angle glaucoma rarely the lens may be entrapped in the pupil causing pupillary
block glaucoma.
B. Skeletal Changes
Skeletal Changes are prominent and may be the first cause to bring the child to the
physician. The child is tall and thin for age, most striking are long slender limbs, the span of
out stretched arms is more than the height of the child, the fingers are long, spidery hence
called arachnodyctalous. The tip of the thumb passes beyond the ulnar border of the palm
when flexed. The thumb and index finger of one hand when wrapped round the wrist of the
other hand, over lap each other. The thumb can be extended back wards to touch the radial
border of the forearm. The metacarpals and phalanges are long and thin. The large joints are
prone for subluxation. The head is often dolicocephalous with high arched palet.The spine
shows scolio kyphosis. Occasionally there may be hemivertebrae, and spinabifeda. The chest
is long and narrow, the sternum shows pactus deformity.The muscle are hypoplastic, the
skin is loose without subcutaneous fat, weakness in abdominal muscles predispose hernia
formation.
C. Cardiac changes
Cardiac changes begin with dilatation of aorticroot, leading to aortic insufficiency,
fuciform aneurysm of aorta is common and the aneurysm may show dissecting changes.
D. Other changes consists of
Winged scapulae, flat foot, contracture of joints, pulmonary and renal anomalies, mal-
formation of ears. The children have normal I.Q and there is no biochemical changes.
E. Management
Management depends upon severity and duration of the condition it consists of:-
Improvement of vision
1. Some children may not have much visual disturbance inspite of subluxation if there
is no error of refraction.
2. When error of refraction is present it is generally axial myopia, because the aphakic
is exclude from the pupil due to small, rigid central pupil.
3. With a large aphakic area in the pupil it should be decided if, the child will benefit by
minus glasses with constricted pupil or aphakic correction with dilated pupil. Aphakic power
is considerably counter balanced by presence of axial myopia hence it is invariably less than
+ 10D.
4. The lens should not be tempered with unless it is :
Cataractous, dislocated or causing glaucoma.
(a) The lens is generally removed through limbal route.
(b) The posteriorly dislocated lens is removed through parsplana with extensive
vitrectomy.
(c) Aphakia is managed by spectacle or contact lens. It is not possible to put an IOL in
the bag in subluxated lens following phacofragmentation. The options open areiris clip lens,
lens in the sulcus or sclera fixated lens all which has their advantages and disadvantages.
5. The retinal detachment can be as a part of axial myopia or vitreous disturbance. It
may require extensive surgical procedures.
HOMOCY8TNURA
Homocystinuria is second most common cause of bilateral subluxated lens. Its skel-
etal and ocular features have many similarities with that of Marfans syndrome. Important
differentiating point are:- Homocytiruria is associated with mental retardation, gives posi-
tive bio chemical test for uninary homocystine, and is autosomal recessive in nature
41
How-
ever it is of utmost importance that the two conditions be differentiated because symptoms of
homocystinuria can be eliminated by proper diet and medication.
Homocystinuria is one of congenital errors of metabolism due to deficiency of more than
one enzyme i.e. methionine metabolism
42
. The main causes of the clinical features are excess
of methionine (an essential amino acid) and homocystine in blood. The patients excrete large
amount of homocystine in urine. The syndrome is caused mostly due to lack of cystathionine
B. synthase that helps to convert methionine to cystine . The result is rise of methionine and
homocystine in body fluid.
The disorder is equally common in boys and girls.
The clinical signs are not noticed at birth. The condition manifests itself by eight to
ten years. However the child may show signs of delayed physical milestone from early age.
The mental retardation becomes obvious as the child goes to school. This is initially attributed
to illhealth and diminished vision. The subluxation of lens becomes apparent by tenth year.
The condition has following components:
A. Ocular
B. Skeletal
C. Mental
D. Cardiovascular
E. Positive biochemical test.
A. The ocular changes comprise of
1. Lens. Bilateral progressive subluxation of lens that has the tendency to fully dislo-
cate. The accommodation is poor or absent. As the zonules are broken which may be matted
over the lens surface
39
. The shape of the lens may be small and spherical. The lens may de-
velop lamellar opacification.One of the initial presentation may be painful acute loss of vision
due to pupillary block or endothelial damage caused by the lens dislocating in A.C. . If the lens
dislocates in vitreous it may start phacogenic uveitis and glaucoma or may remain silent. The
aphakia produced due to subluxation or dislocation compensates pre existing axial myopia,
thus the aphakic power is generally less than +10 D.
2. Iris. The iris is generally tremulous over the aphakic part. Occasionally there may
be aniridia which makes diagnosis more difficult.
3. Sclera. The Sclera may be stretched and thin .May develops staphyloma due to asso-
ciated buphthalmous.
4. Retina. There is high incidence of retinal detachment mostly due to myopia. How-
ever aphakic detachment is very common following lens exaction.
5. Vision. Most of the children have diminished distant vision due to:
(a) Error of refraction
(b) Glaucoma
(c) Squint
(d) Amblyopia
B. The skeletal changes are
The skin is light coloured, with malarflush, the check bones are flat, and the skin is dry.
The limbs are long, the fingers are spidery (arachnodactyly),
Joints are prone for subluxation.
Feet are flat with floppy gait.
The spine shows kyphosis and scoliosis.
Osteoporosis is common leading to fractures.
Hernia is common due to under developed abdominal muscles.
C. The mental changes consist of
Low IQ and other psycho somatic disorders are common. However some of the children
may have normal intelligence.
D. Cardio vascular changes consist of
Various congenital anomalies of heart.
Thromobo embolic episodes are common leading to-cerebrovascular accidents. Myocardial
infarction in early adulthood and pulmonary embolism are frequent. All these factors make
these children very poor surgical risk. They have high rate of death during general anaesthe-
sia. The child with homocystinura generally does not live beyond third decade.
E. Biochemical test
43, 44
Cyanide sodium nitropruside test is a good secreting test but not conformatory
due to high rate of false positive results.
The test consists of:
Adding two ml of 5% sodium cyanide to 5ml of fresh and acidified urine. This mixture is
allowed to stand for ten minutes, then two to four drops of sodium nitropruside is mixed to the
previous solution. This gives bright red colour which is common both to homocystine and cystine
present in urine. To confirm the diagnosis of homocystinuria electrophoresis is required. Bac-
terial contamination of urine of normal child may also give false positive result.
F. Management
Management of homocystinuric child is more complicated due to :
(1) Associated mental changes which may hinder visual rehabilitation.
(2) Increased risk of thromboembolic phenomenon that makes them poor surgical risk.
(3) Only positive point in homocystinuria is that the condition can be helped by :
(a) Restricting dietary methonine and supplementation of oral cystine
42
.
(b) High doses of vitamin B6 (Pyridoxine). The dose recommended is 600 mg to 1200
mg daily and folic acid orally.
(c) Those individuals not responding to pyridoxine may be put on Betaine that re-
verses degradation of methonine.
F. Ocular Management is similar to any other ectopia lentis.
G. Mentally retarded children may have to be put in institutions meant for mentally
and visually challenged.
XV. WELL-MARCHE8AN 8YNDROME
45a,45b,45c
This is the third of the three common disorders that cause ectopia lentis with systemic
involvement. It is rarer than Marfans syndrome and homocystinuria both of which have many
common features. The only common feature of Weill Marchesani syndrome with the former
two is ectopia lentis.
It is an inherited disorder of mesoderm. It may have both types of inheritance i.e.
autosomal dominant or recessive.
The components of the disorder are
A. Ocular
B. Non ocular
A. The Ocular features are
1. Microspherophakia where the lens is small in equatorial diameter but has greater
than normal anterio posterior diameter. The shape of the lens is not apparent unless the pupil
is dilated, with dilated pupil the lens has a circular bright crescent on the periphery. The
zonules are visible on slit lamp examination. The lens has tendency to move anteriorly rather
than upward or downward. This results into a ball and socket pupillary block. The block is
enhanced by use of miotics. The spherophakia results in index myopia. Occasionally the A.C.
is shallow and tremulousness of iris is better seen on the periphery. The lens may completely
dislocate in anterior chamber with its resultant complications. Posterior dislocation is less
commaon.
2. Secondary pupillary block glaucoma is most troublesome complication. As the
pupil constricts over the spherical lens a pupillary block results that shuts off the flow of
aqueous from posterior chamber to anterior chamber, resulting in shallowing of A.C. and nar-
rowing of angle which itself may have strands of mesodermal tissue. The tension is relived
flowing maydriasis or establishing a path between A.C. and P.C. by iridectomy or laser
iridotomy.
B. Non ocular signs
The non ocular signs are striking. The patients have a short stubby stature. Their
maximum height seldom exceeds five feet and many are considered to be dwarfs and
investigated as such. They have brachycephly, well developed subcutaneous fat, muscles are
hypertrophied, arms and legs are short, the hands and feet have squarish shape, and the joints
are prominent and stiff. The thorax looks larger as compared to the body. They have tendency
to develop carpal tunnel syndrome. There is no fixed pattern of cardiac disorder, the children
have assorted cardiac anomalies, there is no mental retardation, life expectancy is better
than in homocystinuria and no specific biochemical change have been attributed to the
conditions.
Management Consists of
1. Correction of error of refraction.
2. Prevention of glaucoma by doing prophylactic surgical peripheral iridectomy or laser
iridotomy.
3. Management of lenticular opacity by standard microsurgical procedure.
4. Removal of dislocated lens.
Comparison between Marfan, homocystinuria and Weill-Marchesani syndrome.
Marfans Syndrome Homocystinuria Weill-Marchesahi syn-
drome
Inheritance Autosomal dominant Autosomal of recessive Intermediate
Lens Sub luxate up and out
Non progressive may
become cataractous
have tendency to dislo-
cate
Sub luxale down and
may dislocate
Spherophakia displaced
in anterio posterior direc-
tion may dislocate
Pupil Small resistant to
mydriatic
normal normal
Iris Poorly developed dila-
tor muscles, loss of pat-
tern iridodonesis.
No Specific changes ex-
cept iridodonesis
No specific change except
iridodonesis
Ocular Changes
Retina Retinal detachment
rhegmatogenous or
traction
Rhegmatogenous or
traction retinal detach-
ment
No specific change
Skeletal Changes
Height Tall, Slender Tall Slender Short Stubby
Skull Dolicocephalic Dolicocephalic Brachycephic
Spine Scoliosis, kyphosis Scoliosis kyphosis No Specific Change
Thorax Long slender Long slender Short broad
Subcutaneous Tissue Poorly developed Poorly developed Well developed
Musculature Hypoplastic Hypoplastic Hypertrophied
Limbs Long thin Long thin Short broad
Fingers Arachno dactyly Arachno dactyly Spade like
Joints Loose, hyper extensile Loose hyper extensile stiff
Cardiovascular
Change
Common Aortic valve
dilatation, aneurysm
Variable Variable
Thrombi embolic
episodes
Nil Common Nil
Intelligence Normal Low Normal
Life span Normal Short Normal
Biochemical test Nil Sodium Cyanide test
Positive
Nil
Glaucoma Pupillary block, phaco-
genic or due to meso-
dermal change in angle
Inverseglaucoma
Refraction Axial myopia, myopic
astigmatism
Axial myopia, myopic
astigmatism.
Index myopia
Accommodation Retained Lost early Variable
A.C. Deep in lower part
shallow in upper part
Deep in upper part
shallow in lower part
Irregular, iris may bulge
in A.C. due to pupillary
block
Non-specific
OTHER 8YNDROME8 WTH ECTOPA LENT8
A. Ehlers Danlos syndrome
B. Hyperlysinemia
C. Sulphite oxidase deficiency
A. Ehlers Danlos syndrome is a rare disease generally seen in adults but becomes
apparent in childhood, may be noticed in infancy, is caused due to inherent defect in colla-
gen. Its two systemic findings are hyper elasticity of the skin that ruptures on slightest
trauma, causing extensive ecchymosis and hematoma. The other systemic presentation is
hypermobility of joints.
The ocular features are Sub laxation of lens in both eyes, thinning of cornea,
kertoconous, microcornea, megalocornea, blue sclera. Angioid streak is a common feature in
fundus. Stretching of cornea and sclera predispose laceration of eyeball. Ruptures in Bruchs
membrane and stretching of retina result in retinal detachment. There is no biochemical
defect, intelligence and life span is normal. There is no specific treatment.
Hyperlysinemia
45
. This is an inborn error of metabolism due to lysinedehydrogenase
deficiency. Its ocular manifestations include ectopia lentis, microspherophakia, may have
ophthalmoplegia. These children have low IQ and have retardation of growth and laxicity of
joints there is no specific treatments.
Sulphite oxidase deficiency
In the broad sense this is derangement of cystine metabolism due to sulphite oxidase
deficiency. The children have short life span and mental retardation there is bilateral
dislocation of lens. No specific treatment is known.
OCULAR CAU8E8 OF ECTOPA WTH OUT 8Y8TEMC
NVOLVEMENT
A. High myopia
B. Buphthalmos : Primary or Secondary.
C. Keratoglobus
D. Aniridia
E. Reigers anomaly
F. Uvitis
G. Blunt trauma, (Generally unilateral rarely bilateral.)
PAEDATRC CATARACT
Incidence and prevalence of paediatric cataract is far less than adult cataract however
cataract in childhood has far-reaching consequences. Adult cataracts have excellent visual
result following lens extraction, same is not true with childhood cataract. Childhood cataract
surgery has poorer visual prognosis and more complications.
It is estimated that congenital cataract is found roughly one in every 250 live births
46
Very few have clinical symptoms, only few require treatment, incidence is higher in develop-
ing countries where hardly any prophylactic measures are taken for maternal rubella, and
correction of maternal malnutrition, incidence also increases due to consanguinity
47
. Congeni-
tal cataract is a leading cause of blindness in children about 20% of blindness in children is due
to congenital cataracts
46
.
In one third of the cases of cataract no cause can be detected. In developing countries
trauma is a major cause of cataract in children. Congenital and developmental cataracts have
a strong heredity. However sporadic cases are also common. Though most of the congenital
cataracts are bilateral, many of the children present as unilateral cataract, in such cases the
other eye must be examined in detail for subtle signs of cataract. Unilateral cataracts have
poorer prognosis as compared to bilateral cases. Trauma and intraocular diseases are common
causes of unilateral cataracts.
A. The cause of congenital cataracts can be
1. Commonest cause being maternal rubella in first trimester, other causes can be
maternal diabetes, parathyroid disorder and malnutrition.
2. In another group of children, the mother is normal but the child has various errors of
metabolism i.e. galactocimia, hypoglycaemia, homocystinuria and other aminoaciduria.
3. There are numerous syndromes associated with congenital cataract. Some frequent
syndromes are Lowes syndrome, Alports syndrome and Turners syndrome.
4. Congenital cataract may be the only anomaly or else it may be associated with other
maldevelopments of the eye.
5. All opacities in the lens do not cause visual disturbance and may go unnoticed till
later life, only to be discovered on routine examination.
B. Symptoms of congenital and developmental cataract
46, 48
Symptoms depend on
1. Position of the opacity. Visual loss is more in central cataracts than peripheral,
opacity near the nodal point is likely to cause more visual disturbance than one away from it.
2. Size of the opacity. Opacities larger than normal pupil cause more visual loss. The
matter is worsened when pupil constricts in bright light or the child has moitic pupil due to
any cause.
3. Number of the opacities. Scattered small opacities hardly produce any symptom
but nuclear opacity with posterior capsular opacification cause more visual loss than any one
of them.
4. Density of the opacities. Denser opacities cause more loss of vision than translu-
cent opacities.
5. Unilateral cataracts. They may go unnoticed if the other eye has good vision; these
eyes develop amblyopia and squint more frequently.
6. Age of on set. Congenital cataracts that are apparent before three months of age
cause intractable nystagmus.
7. Associated ocular malformation. Microphthalmos, persistent hyaloid system,
persistan primary hyperplastic vitreous, retrolental fibbroplasia, Reigers anomaly have poorer
vision and prognosis.
8. Associated with symptoms of other systemic syndromes.
9. Physical under development is a frequent associated feature of congenital cataract.
10. Frank mental retardation is common in homocystinuria. Even children with normal
IQ may fail to attain expected academic grades due to poor vision.
C. Presenting Features of Congenital and Developmental Cataract
Various age groups have different presenting features.
1. An infant may be brought with following complaints :
(a) White Reflex in pupillary area
(i) A dense opacity that fills the pupillary area is obvious even in natural light and
noticed by the mother, midwife or the attending neonatologist.
(ii) Faint, Peripheral and posterior opacities are missed initially. Such an infant is
brought to ophthalmonologist by parents with the suspicion that the child does
not have expected vision. It is only with the dilation of pupil the cataract be-
comes obvious.
(b) The child is brought with nystagmus or squint.
(c) There is positive history of developmental cataract in the family, so the child is
brought to get it excluded.
2. Older Children are brought with white reflex, nystagmus that dates back to first
three months, squint or diminished vision in various combinations.
3. Some children are brought with glare. The child with unilateral cases may close the
effected eye to wardoff the glare.
4. Congenital cataract per se does not cause pain, photo phobia or redness of eye. If these
symptome are present the child should be investigated for retinoblastoma.
5. Sick children who fail to thrive are likely to have metabolic cataract or rubella cataract.
6. Unilateral cataracts are detected late because the child carries out his routine with
better vision in the other eye. They are fist brought with squint. Unilateral dense cataracts
draw attention earlier than posterior cortical and posterior polar cataracts.
7. Some of the children are referred by paediatricians who have diagnosed a syndrome
that is likely to have cataract.
UNLATERAL CATARACT
Unilateral congenital and developmental cataracts are unique in many ways:
A. 30% of idiopathic congenital and developmental cataracts are unilateral.
B. Some of the heredity cataracts are also unilateral.
C. Children with unilateral cataract seek medical help later than bilateral cases. Gen-
erally they are brought with strabismus.
D. In case of obvious cataract in one eye the other eye should be examined for of subtle
signs of cataract.
E. All cases of uniocular cataract should be examined in details for evidence of occult or
forgotten trauma, uveitis, retinal detachment etc.
F. Post operative visual prognosis in unilateral congenital or developmental cataract is
always poorer than bilateral cases.
G. Unilateral traumatic cataracts have better vision following cataract surgery than
congenital and developmental cataract.
H. The purpose of operating unilateral cataract is to improve vision even when it is
known that there are no chances of improvement of central vision following successful surgery,
the lens should be removed to improve peripheral field on that side.
I. Developmental cataracts that have relatively clear lens for first few years like poste-
rior lenticonous have good visual prognosis.
J. All cases of unilateral congenital and developmental cataracts should be encouraged
to have P.C.I.O.L than conventional extra capsular cataract operation
49
with spectacle or contact
lens.
WORK UP N A CA8E OF PAEDATRC CATARACT
A. Eyes of all new born children should be examined routinely by attending obstetri-
cian, neonatologist for evidence of dense lenticular opacity. The traditional midwives and nurses
can be taught to screen neonates for lenticular opacities.
B. All neonates who have hepato spleenomegaly, abnormal heart sound and fail to thrive
should be examined for possibility of galactocemia, rubella and toxoplasmosis. Urine ex-
amination for reducing substance will exclude galactosaemia while torch test
28
is helpful for
rubella and toxoplasmosis. Galactokinase deficiency is seen in otherwise healthy children with
congenital cataract.
C. Positive family history of developmental cataract in parents, siblings and first cous-
ins should alert the physician for possibility of lamellar cataract.
D. All white reflexes in pupillary area in children are not congenital cataracts.
Cataract should not be confused with retinoblastoma and vice versa. It should be kept in
mind that all non lenticular opacities need not be retinoblastomas. (See differential diagnosis
of white reflex in pupillary area.)
E. Examination of an eye with congenital cataract
1. As infants can not be examined on usual slit lamp they should be examined either
with hand held slit lamp or under operating microscope. In absence of these an examination
with uniocular loupe with a bright torch is good enough. Binocular loupes give very low mag-
nification.
2. Pupillary light reflex in infants is poor due to strong constrictor muscle. It can be seen
well under magnification.
3. Anterior chamber is examined for other malformation of anterior segment and evi-
dence of other diseases.
4. Next step should be examination of anterior segment under full mydriasis.
The choice of mydriasis is as follows:
(a) Tropicamide 0.5% to 1% is most suited for children and infants.
(b) Atropine should be used with usual caution to avoid side effects. It is contra indi-
cated in Down syndrome.
(c) Cydopentolate 0.5% is also a suitable cycloplegic.
(d) Phenylpherine is generally used as 2.5% drop along with 2% home atropine.
Poor dilatation of pupil means a rigid pupil that may cause difficulty during subsequent
surgery.
A widely dilated pupil delineates outer border of the opacity. In lamellar cataract the
periphery is clear. Fundus can be examined through this clear zone both by direct and indirect
ophthalmoscope. If the eye has a large clear lens on the periphery, surgery can be postponed
and child is kept on mydriatic. If no clear zone is visible between the lens and iris, prompt
surgery is indicated.
5. The posterior segment is examined for presence of remnants of hyaloid system,
retrolental fibroplasia and other fundus changes.
6. The macula is specially examined for evidence of central choroiditis in case of suspected
toxoplasmosis.
7. An eye with bright cornea of normal size, clear A.C and brisk pupillary reaction
generally has normal, intraocular tension. A large cornea with haze, deep A.C. is most likely to
be a buphthalmic eye where examination of tension under generally anaesthesia is a
prerequisite. So are the eyes with mesodermal abnormalities.
8. In cases where fundus can not be seen with dilated pupil USG of the eye and some
time C.T. may have to be under taken. B.Scan can help to find out posterior capsular defect
that is common in many congenital cataracts. In traumatic cataracts, B.Scan delineates
vitreouscortexmix, associated detachment, haemorrhage, foreign body, exudates.
9. All children posted for surgery should under go a pre anaesthetic check. A preoperative
check up by neonatologist may be ordered by an anaesthetist.
10. It is very important to explain the procedure and its utility to the parents, espe-
cially, in case of bilateral dense opacities and all unilateral cataracts. Possibility of IOL and
other measure should also be discussed with them as far as in language and terms they can
under stand avoiding medical jargons.
MANAGEMENT OF PAEDATRC CATARACT
A. Management of paediatric cataract is difficult and frustrating because of many
factors some of them are:
1. The eye of an infant is small and changes rapidly with age.
2. The visual system of a child is immature and likely to suffer irreversible damage if
the macula does not receive sharp image of the outer world. Nystagmus and amblyopia de-
velop fast in infancy and child hood.
3. All the opacities are not of uniform nature as regards density and position hence a
uniform protocol can not be advised for all cases.
4. Cataracts in childhood may be associated with anomalies of other intra ocular struc-
ture that hamper improvement of vision.
5. Cataracts may be associated with larger systemic syndromes including errors of me-
tabolism.
6. The child has a long life to live that includes education, vocational training, choice of
profession where diminished vision, diminished accommodation, loss of peripheral field and
appearance may be some of the hindering factors.
7. There is not medical treatment for cataract
60
.
8. Scope of prophylaxis is limited to rubella.
9. Genetic counselling is not always possible.
10. Definitive treatment is surgery which requires high degrees of skill and effi-
ciency.
B. Various modes of management available for paediatric cataracts
1. Prophylaxis
(a) Prophylaxis is limited to rubella cataract and to some extant galactosemic cataract.
If every girl in school going age receives a shot of rubella prophylaxy as part of national immu-
nisation, the danger of rubella cataract can be wiped out altogether. It should be kept in mind
that rubella is not only a blinding disease it is also a crippling and some times a fatal disease.
(b) In galactosemia, removal of milk products from the childs diet will not only remove
the possibility of child developing cataract but may improve transparency of the lens.
(c) Trauma as a cause of cataract requires involvement of parents, teacher and social
workers. Children should be explained about possibility of blindness by sharp objects like
spears, arrows, and catapult, tipcats.
2. Non-Surgical
This depends on presence of useful clear zone between the pupillary border and periph-
ery of the opacity. A small opacity that does not cover the whole of normally acting pupil does
not require any treatment including mydriasis which is required when the opacity covers the
entire normally acting pupil but has useful clear zone when dilated. These are generally vari-
ous types of zonular cataracts the regime consists of keeping the pupil dilated with lowest dose
of atropine that gives maximum mydraiasis. Atropine has a draw back that it abolishes accom-
modation that is an important factor for learning, in children .These children under atropine
may be provided with near correction. Dark glasses during day may also serve in limited way.
The children should be taught to sit with back towards the light and light falling on the books.
3. Surgery
Surgery is the definitive treatment for all paediatric cataracts. Lensectomy with IOL
is the standard procedure for most of paediatric cataracts, IOL is with held only when its
placement is not possible. In such cases Lensectomy followed by contact lens, spectacles or
epikeratophakia are some of the alternatives available. The aims of surgery are to pre-
serve vision, prevent amblyopia and maintain fusion as far as possible.
IOL in Children
Older children can tolerate P.C.I.O.L as much as adults. Till few years ago it was thought
that intra ocular lens be implanted only after 2 years of age
50
Now a days, implanting a lens
within 48 hrs of birth is considered safe
47,49
.
Some of the anatomical difficulties in implanting IOL in children are
1. The eye ball is small in all dimensions.
2. Comparison between various dimensions of a new born and adult eye is given below :
Feature New Born Adult
Axial length 16.5mm 23.5mm
Volume 2.8ml 7.1ml
Lens diameter 6.00mm 9.1mm
Corneal diameter 10.00mm 11.8mm
A.C. depth 2.5mm 3.2mm
3. The childs axial length will reach its adult size by seven years. At 2 years it is about
20mm
51,52
and at four years it is 22 mm.
4. An IOL of 32D will be required to make an eye of a new born (axial length 16.5mm)
emmetropic. But this is not correct because the axial length of the child will be as much as 21
to 22mm at 3 to 4 years of age, which, with an IOL of 32D will make it myopic by 8 to 10D. This
amount of myopia reduces the distant vision greatly. The most ideal choice will be to use an
IOL that will make the eye slightly myopic. This will give a comfortable near vision as well.
5. The other difficulty faced is difficulty in calculating IOL power in children below
2year of age. For accurate reading this has to be done under general anaesthesia which by it
self is a highly specialised discipline.
6. Next difficulty is to decide when to implant an IOL. Children with dense opacities,
where no part of the fundus is visible under maximum mydriasis should be operated as soon as
the child may under go general anaesthesia for considerable time. In bilateral cases lensectomy
is followed by IOL implant, otherwise a simple aphakia is left to be corrected by, spectacles,
contact lens, and epikeratophakia. The last method has been given up in favour of secondary
implant. It is better not to operate both the eye at the same time.
7. Unilateral cataracts of all densities have uniformly poor visual prognosis.
8. If the discs in both eyes are visible, are of normal colour, elevation and size, both
maculae are visible with out any abnormality chances are that vision in both eyes are equal.
The child should be watched for evidence of increase in size of opacity. An opacity larger
than 3 mm requires removal. If the opacity is stationary the eye can be atropinised and
bifocal prescribed when the child starts schooling.
9. If there are evidences suggestive of asymmetry in vision, amblyopia must be treated
vigorously.
10. Presence of nystagmus is always a very poor prognostic factor. It generally develops
by two month in case of congenital cataract.
11. Next problem is to decide which type of lens should be implanted. A.C. IOL are out
of question because of high rate of complication. It is better to leave an eye aphakic rather than
pseudophakie with A.C.IOL.
In majority of cases P.C.IOL are put with satisfactory results. Of course there is a school
of thought that considers the infants eye to be too small and too immature to put a P.C.IOL
because except an iris claw lens all lenses are over sized for the placement and fixation
14,47
and
recommend an iris claw lens with maximum width of 6mm to 7 mm with optics of 4mm.
Many of the pediatric aphakics need secondary lens implant, if the posterior capsule is
intact. An iris claw lens may be put even in a partially absent posterior capsule.
4. Other methods of management of aphakia in children
Commonest cause of aphakia in children is surgical removal of lens. Other causes are
spontaneous absorption of a congenital cataract due to posterior leak or absorption following
penetrating wound. There may be traumatic dislocations or spontaneous dislocation in
buphthalmos, megalocarnea or chronic uveitis.
Aphakia in children is managed by
(a) Spectacles
(b) Contact lens
(c) Secondary implant
(d) Epikaratophakia
(e) Combination of more than one method.
(a) Spectacles. Spectacles in children are generally prescribed in all ages more for
economic reasons than its optical efficiency in developing countries. Its advantages are: It is
cheap, easy to manage, gives fairly good distant vision, bifocals may suffice for near vision. If
power is equal in both eyes chances of amblyopia is reduced. They are not very suitable for
unilateral alphakia because the child with good vision in other eye prefers normal single vi-
sion with out glasses and if the child is forced to wear them, have a tendency to look over the
frame. This negating the optical advantage of spectacle.
(b) Contact lenses
49
. Contact lenses are best alternative to IOL, some surgeons fit
contact lens just after the lensectomy, others prefer to fit extended bear lens after 10 days.
Hard contact lenses are difficult to insert due to tightness of the lid, strong blink reflex, fre-
quent displacement of lens and expulsion of the contact lens. All the parents can not be taught
to insert them. Up to age of six daily wear or extended wear contact lenses may be tried. Once
the child can manage the contact lenses gas permeable lenses may be prescribed.
(c) Combination. Contact lenses do not give use full near vision. They are given near
correction in the form of spectacles. Some time spectacles may be required to correct residual
power over contact lens or IOL.
AFTER CATARACT
53, 54, 55
A. After cataract is not a true cataract. It is the after math of rupture of lenticular
capsule with partial absorption of cortex, opacification of posterior capsule with proliferation
of lens fibers. In milder form it is also known as posterior capsular opacification or simply
P.C.O. Almost all eyes develop P.C.O. following rupture of lens capsule that may be surgical,
accidental or spontaneous. P.C.O. is by far denser in children than in adults. It is a major
cause of gradual lowering of vision following uneventful I.O.L. implant.
B. Common symptoms of after cataract are
1. White reflex in pupil which may be faint enough to be seen with magnification or
denser that is visible in diffused light
2. Non improvement of vision
3. Frequent change of refraction.
Common surgical procedures that result in after cataract are extra capsular cataract
extraction, micro surgical extra capsular cataract extraction with or without IOL phacoemulsi-
fication, needling aspiration of soft cataract. Rarely injury to lens during vitrectomy may also
result in after cataract formation.
Incidences of after cataract depends upon :
1. Age of the patient : almost all aphakic children following extra capsular lens extrac-
tion develop PCO.
2. Duration following lens extraction : may develop soon after if coxtex has not been
removed well. Most of the children will develop PCO with in one year.
3. Amount of cortical matter left,
4. Placement of IOL and type of IOL,
5. Associated pre existing uveitis
6. Management of post operative uveitis.
C. Mechanism of after Cataract Formation
The posterior capsule of lens is devoid of epithelium. The anterior capsule is lined by
cuboidal cells up to beginning of the equator where the cuboidal cells get elongated gradually
and are converted in to lens fibres.
In an extra capsular cataract extraction following capsulotomy as much of cortex as
possible is removed, yet some of the fibres are left behind, that continue to proliferate as
opaque fibres, these opaque fibbers may spread over the posterior capsule, may proliferate as
globular bodies or may be entrapped between the anterior and posterior capsule. The first is
called simple P.C.O the second, Elschnigs pearl and the last Soemmerring ring.
The after cataracts besides lens fibbers also contain pigments, exududate and blood in
the initial stages. Elschnigs pearls are formed due to proliferation and migration of epithelial
cells to form a fish spawn like small white translucent bodies. The size varies from pin
head to 23 mm in diameter. They are generally seen in the pupillary area. Their numbers
vary from single to many. Dilatation of pupil may expose more.
Soemmerring ring is doughnut shaped ring opacity. This is formed due to adhesion
of anterior capsule to posterior capsule entrapping cortex as well as equatorial epithelial cells.
The ring is generally hidden behind the iris and visible only with mydriasis, the space inside
the ring has fainter P.C.O. The ring may occasionally be dislocated in the anterior chamber.
Incomplete capsulotomy as was fashion in traditional operation for congenital and trau-
matic cataract resulted in thicker after cataract than present day microsurgical capsulorrhexis.
Continuous curvilinear capsulorrhexis cause less P.C.O than canopener capsulorrhexis.
Posterior capsulorrhexis is done routinely with or without anterior vitrectomy in chil-
dren to reduce P.C.O. Lens extraction with out P.C. IOL causes more opacity than with P.C.IOL.
A biconvex IOL or a lens with posterior convexity also lessens P.C.O. Heparin coated IOL or
heparin used in irrigating fluid diminishes chance of after cataract. Occasionally the posterior
capsule develops wrinkles this causes, stretch lines on the posterior capsule resulting in fluc-
tuating vision and glare.
Capsule contraction syndrome is caused due to contraction of anterior capsular open-
ing this is more common if the eye has suffered from uveitis in the past.
Bacteria of low virulence like propionibacteriumacnes and staphylococcus epidermis
45
may be entrapped in the capsular sac and cause capsular opacification with out causing
endophthalmitis but may cause endophthalmitis when released following capsulotomy.
D. Clinical Features of aftercataract depend upon
Location of opacity, density of opacity, age of the patient, time lapse after surgery, and
associated uveitis.
Pre School children do not complain of diminished vision, they have to be tested for
vision, and examined for amblyopia strabismus and nystagmus. On set of nystagmus in in-
fants following surgery means that vision has not improved. This generally happens if the
child is less than two years of age.
In dense after cataracts the parents complain that cataract has not been cleaned well or
has developed again.
E. Signs consist of white reflex in pupillary area. When examined under mydriasis
with slit lamp shows various grades of opacity ranging from translucent to dense white mem-
brane. There may be gaps in between. Other features observed are Elschnigs pearls,
Soemmerring ring and posterior capsular traction lines.
F. Treatment
1. Best treatment of after cataract is its prevention or reduction in its density. It must
be remembered that cent percent children develop some degree of P.C.O. that can be
prevented by posterior capsulorrhexis with anterior vitrectomy, large capsulorrhexis,
use of biconvex IOL and use of heparin coated IOL. Most important thing is to remove as much
of cortical matter as possible during the initial surgery. The post operative uveitis should be
managed promptly.
2. Once the after cataract has developed and is dense enough to cause reduction in
vision it should be cut either surgically or by laser.
3. Laser capsulotomy by N.D. YAG is the treatment of choice.
(a) The indications of laser capsulotomy are diminished vision, monocular diplo-
pia, glare, to facilitate intra-ocular examination and other manipulation.
(b) Relative contra indications consist of
(i) Clear view of fundus by direct ophthalmoscope through the centre of the pupil,
because if observer can see the fundus, the observed eye should also be able to
see. Otherwise other causes of non improvement of vision such as cystoid macular
oedema, amblyopia, retinal detachment, vitreous bands and chronic
endophthalmitis should be looked for
(ii) Central corneal opacity
(iii) Corneal oedema
(v) Already existing glaucoma and cystoid macular oedema.
(v) Peripheral retinal degeneration.
(vi) Unwilling parents of the child and uncooperative child.
(vii) Glass I0L
The laser capsulotomy is done either in normal or semidilated pupil
Complications of laser capsulotomy
1. Cystoid macular oedema or worsening of cystroid macular oedema
2. Transient rise of IOP
3. Rhegmatogenous retinal detachment.
TRAUMATC CATARACT N CHLDREN
General Consideration
Trauma accounts for 80% of cataract in paediatric age group. They are less common in
first three years of age, incidence gradually rises as the child grows. Most of them are unilat-
eral however blast injury, cracker injury and chemical injuries, electric shock, radiation can
cause bilateral traumatic cataract. Domestic injuries are commonest cause in younger chil-
dren while sports injuries are more in older children. Ninety percent of injuries are avoidable.
Traumatic cataract have better visual prognosis than congenital and developmental cataract
even when they are uni-ocular specially if traumatic cataract occurs after development of bin-
ocular vision. Posterior segment and retinal involvement remain major cause non improve-
ment of vision.
Injuries commonly associated with traumatic cataract are: Penetrating injury with or
without retained intra ocular foreign body, blunt injury, chemical injury, electrical injury and
radiation.
Penitrating injuries do not involve lens in isolation they are associated with corneal,
corneo scleral or scleral injuries. Penetrating injuries passing through pupil generally have
centre corneal scar and injury to the lens with out injury to iris, but it is common to find iris
incarcerated in the wound, otherwise injury to iris and or ciliary body is very common.
Small penetrating injuries
Depending up on the size of the penetrating injury the cataract may be localised if the
wound is small and heals fast otherwise complete, if the capsular wound heals quickly. These
injuries are generally caused by sewing and knitting needles, nibs, pins, sharp long thorns etc.
These small opacities are generally stationary.
Large penetrating injuries cause
1. Total opacification in short time that may cause swelling of the lens, shallowing ante-
rior chamber and glaucoma if the cortical matter does not leak.
2. If the capsule ruptures and the opaque cortical matter spills out of the capsular bag it
may fill the entire anterior chamber.
3. If the external wound still leaks the eye remains soft.
4. With a watertight wound the tension may rise resulting into secondary glaucoma.
5. If there is associated zonulolysis : sub luxation or dislocation of cataractous lens. In
such conditions vitreous may herniate through the aphakic area, some times the cortical mat-
ter gets mixed with vitreous.
6. Prolapse of uvea is common.
7. There may be associated hyphema.
8. There may be irido dialysis, chroidal repute or retinal detachment.
9. IOFB are common.
Lenticular changes following blunt injury are less dramatic than penetrating in-
juries. Blunt injury may cause small, faint, discrete opacities that may fade with time. It may
be permanent and stationary or may spread over the years. Both contusion and concussion
can cause traumatic cataract. These closed globe injuries do not cause incarceration of the
uvea. The uvea may suffer the effect of blunt injury along with lenticular changes that always
hampers future vision.
For traumatic cataract to develop there should be some damage to any part of lens
capsule that allows aqueous to reach the cortex. This hydrates the cortex, which converts
soluble protein into insoluble protein and leads to ultimate opacification
Opacities may be:
1. Transient, static or progressive.
2. Localised as discrete punctate spots, rosette shaped or scattered subepithelial
opacities.
3. Rarely may be total.
Rosette cataracts
Rosette cataracts are common in blunt injuries. It can occur early or late.
Early rosette cataract develops with in few days of blunt injury. It develops in the
posterior cortex as opaque lines radiating from a central point, each line has feathery appear-
ance due to finer linear opacities radiating from its trunk giving a star like appearance. There
is a clear zone between the opacity and the equator of the lens.
Late rosette cataract develops in the posterior cortex one to two years after the injury
that may have been trivial and forgotten.
Vossius ring is not a cataract but is a common effect of blunt closed globe injury
where black pigments are imprinted on the anterior lens capsule in a circular fashion. The
deposits on the lens capsule correspond to constricted pupil. These are transient and may be
confused with post inflammatory pigment on the lens capsule. They fade out over months
some times leaving behind faint subepithelial opacities.
Other uveal injuries associated with concussion and contusion cataract are:
Hyphema, rupture of sphinter, iridodialysis, recession of angle of anterior chamber,
choriodal haemorrhage, choroidal rupture, retinal haemorrhage, retinal detachment and Berlins
oedema. All of them may have adverse effect on visual improvement of vision.
D. Symptoms of traumatic cataract may be
1. Acute following penetrating injury in the form of loss of vision, lacrimation,
photophobia, with or without rise of tension.
2. Concussion cataract may cause diminished vision after days or months.
3. Diminished vision weeks after blunt injury is seen in early rosette concussion cataract.
4. Late rosette cataracts cause gradual loss of vision after months.
5. No appreciable visual loss is seen in Vossius ring, punctate opacities, localised opaci-
ties away from pupil.
6. Unexplained pain in a closed globe with cataract denotes rise of intra ocular tension.
7. Faulty projection should warn about possibility of large retinal detachment.
8. A penetrating wound should always arouse suspension of intra ocular foreign body.
9. Injury with organic matter like thorns, twigs may present as endophthalmities.
E. Investigation
1. The first and fore most investigation is careful history taking. In case of penetrating
injury the child can not hide the history of injury but may not come out with the truth about
the circumstances leading to injury and the offending object causing injury. The child may
altogether withhold history of blunt injury.
2. Careful examination of vision in both eyes should be under taken in all cases of injury.
In infants, pupillary reaction and projection of light gives a gross assessment of vision.
Examination of vision has not only diagnostic and prognostic value. It has far-reaching medico
legal value.
3. Examination of media by oblique illumination, retinoscope, direct and indirect
ophthalmoscope, slit lamp should be done when ever possible. They will give information
regarding status of posterior chapsule associated uveitis, subluxation of lens, type and extant
of lenticular opacity, state of vitreous, Berlins oedema, cystoid macular oedema, choroidal
tear, retinal detachment, retained intraocular foreign body, vitreous haemorrhage etc.
4. In opaque media when fundus is not visible, the first investigation ordered should be
x-ray of the orbit. If the x-ray shows retained intraocular foreign body, it should be localised,
using any of the standard method and foreign body extracted. Ocular ultrasonography C.T
and M.R.I are useful to visualise small, I.O.F.B. vitreous haemorrhage, endophthalmitis, retinal
detachment, and posterior perforation of globe. However USG may not resolve foreign bodies
less than 2 mm. For such a small foreign body CT is more suited as it can detect bodies as small
as 1.06 mm
3
. Glass fragments are visible only when they are as large as 1.5mm
3
.MRI is gener-
ally not used in suspected metallic foreign bodies because movement of metallic foreign bodies
during MRI can result in MRI induced blindness
56
.
Management
Management of traumatic cataract in children does not differ basically from adult trau-
matic cataract but there are some special features that need to taken into consideration. which
are :
1. Development of amblyopia and squint.
2. Difficulty in management of resultant aphakia in traumatic cataract.
3. Difficulty in placement of IOL.
4. Frequent development of P.C.O. in children.
The management of traumatic cataract in children
1. Prevention: The best management is prevention. It is easier said than done. Parents
should see to it that children do not play with pointed objects. The teachers and coaches should
be instructed to encourage the children to use standard protective gears when ever required in
sports and games.
2. Management of other injuries.
3. Surgery of cataract. Traumatic cataract is very often complicated by corneal, corneo
scleral injuries along with damage to uvea, vitreous, retina in various combinations. These
injuries are caused due to penetrating injuries. If the lens is already opaque it should be re-
moved along with repair of above injuries as primary process with reconstruction of anterior
segment. It may require vitrectomy. Most of the time it is not possible to put a PCIOL in such
cases. Presence of central corneal opacity always jeopardises visual improvements.
Simple traumatic cataracts are generally due to blunt injuries or small penetrating
injuries of the lens with other tissue intact. These are managed by lensectomy, anterior
vitrectomy and primary P.C.I.O.L implantation.
OTHER FORM8 OF CATARACT8 N PAEDATRC AGE GROUP
A. Steroid induced cataract
Steroids when used for long time locally or systemic have profound side effects. Two
common vision threatening conditions are cataract and glaucoma. Longer and stronger the
steroids used more are the chances of development of the cataract. Exact duration and strength
that causes cataract is not known, perhaps it is influenced by genetically determined fac-
tor. Reduction in concentration, frequency, and duration can arrest progress of cataract for-
mation but does not reverse the process. Children are more prone to develop steroid
induced cataract than adults.
Conditions where steroids are used for long time and most of the time in both the eyes
are-vernal catarrh, interstitial keratitis, iridocyclitis due to juvenile rheumatoid
arthritis, post operative and post traumatic status, keratoplasty and sympathetic
ophthalmitis.
In these cases steroid induced cataract may have to be taken as inevitable therapeutic
risk and dealt with accordingly.
Out of all the conditions listed above spring catarrh is the most offending condition
that causes steroid induced cataract in children because the parents are not explained the
regime to follow who allow the children to use strongest possible steroid for quick relief.
Exact mechanism of production of cataract by steroids is not known. Steroid induced
cataract has been reported following use of skin ointment, nasal drops, and inhalers contain-
ing steroids of various strength and for a long time.
Most widely accepted theory is that steroids act on the lens epithelium that is re-
sponsible for hydration of lens fibers. How ever it does not explain why steroid induced cata-
racts always start in posterior cortex.
Exact time taken for cataract to develop is not known. It never develops following use of
steroid for short duration even in high dose like sub conjuctival injection.
The opacity develops as a posterior sub capsular opacity that is denser in the axis of
the lens. It may be in the form of circular dense disc surrounded by lighter areas of
opacification. The opacity being near the nodal point causes much visual disturbances for its
size.
Common symptoms are : Glare, diminished distant vision, diminished vision in bright
light, difficulty in near vision. It is frequent to find associated glaucoma that may contribute to
further loss of vision. Hence, it is essential that all children who have been on steroid
on long term should under go tests for raised intraocular pressure as well.
Management. As cataract is a common feature of steroid use, steps should be taken to:
1. Reduce the strength and frequency of steroid to a level that gives best therapeutic
response without causing cataract.
2. Replace steroid by non steroidal anti-inflammatory drugs when ever possible.
3. Manage spring catarrh by weakest solution of steroid, mastcell stabiliser, astringent,
anti histamine etc.
4. Examine both eyes periodically for diminished vision, glaucoma and amblyopia.
5. Surgical intervention when required with P.C.I.O.L and management of amblyopia.
Less common cataracts in children
1. Electric cataract
57,58
. The child is likely to develop cataract when struck by electric
current or lightening of more than 200 volts that passes through the head. The Electric
cataract is generally bilateral, how ever it may be denser on the side affected more severely
than the other. Generally these children have sever skin and multisystemic involvement that
may over shadow the ocular manifestation which becomes apparent only if the child survives
and acute systemic effects of electric shock has subsided.
There are various types of opacities in electric cataract. They can be vacuoles, streaks,
both in anterior and posterior cortex. Generally the complete lens gets opacified rapidly.
It may some times take months and years to develop cataract. If there are only few vacuoles
present in anterior cortex with in first few months chances are that no significant opacities
will develop.
Management. Cataract is to be managed as any other cataract following blunt injury,
after the acute multisystemic signs and symptoms have subsided.
2. Sunflower Cataract
59
. This is due to electrical dissociation of retained intra ocular
copper that does not combine with the cells. The reaction depends upon copper content of
retained foreign body. More the copper in the foreign body, more sever and faster the reaction.
The copper is mostly deposited on continuous membrane like lens capsule and corneal periph-
ery
59
various manifestations of intra ocular copper foreign body are - Endophthalmitis that is
supurative in nature, chronic non granulomatous panuveitis and sun flower cataract.
The sun flower cataracts involve both anterior and posterior cortex and sub capsular
area as yellowish dust in the centre of the lens with radiating yellowish petals pointing to-
wards the periphery. Fully developed sun flower cataract is visible with on ordinary oblique
illumination. Copper also gets deposited on corneal periphery as Kayser-Fleischer ring. Glau-
coma is common. It is rare in children.
Management. It consists of removal of foreign body, vitrectomy, lens extraction and
management of glaucoma.
C. Cataract in siderosis
59
Iron is one of the commonest forms of intraocular foreign bodies in adults it is rare in
children. It is rarely elemental iron, it is generally an alloy of iron. It is always associated with
penetrating wound. The elemental iron is converted into its ferrous and ferric compounds. The
metal is disseminated through out the tissues of the eye by electrolytic dissociation and gets
combined with cellular protein that destroys the cells causing atrophy of the cells. The overall
effect of dissemination depends upon ferrous content of the foreign body. Occasionally a small
particle may get embedded in the avascular lens through the pupil and remain localised as a
small dot of lenticular opacity never progressing to full blown cataract.
The earliest change is deposition of iron in the anterior sub capsular area of lens. The
metal is deposited as brown rings resembling rust ring in the mid periphery. The lens subse-
quently develops larger opacities. The iris also develops brown iron deposits. The retina
develops degenerative changes similar to pigmentary degeneration causing diminished
night vision, dyschromatopsia and diminished vision. Diagnosis is based on history of pen-
etrating injury on examination an external wound of entry is always present. There may be
hole in the iris through which the foreign body has passed. Inter lenticular particle may be
visible. It the media is clear the foreign body can be seen with direct and indirect ophthalmo-
scope. Brown pigment on lens periphery and iris should arouse suspicion of iron as foreign
body.
In all cases of suspected intra ocular iron foreign bodies, the X-ray reveals its position
unless it is very small CT and USG may show such bodies. MRI is contra indicated in metal
foreign bodies.
Management depends on correct diagnosis of retained iron foreign bodies. Very small
inter lenticular bodies do not require removal but the eye is kept under observation for possi-
bility of development of siderosis on long run. Large foreign bodies are removed after proper
localisation through vitrectomy. Removal of iron foreign body reduces chance of siderosis. The
lens, if opaque is removed by any of the standard procedures.
Cataract secondary to ocular inflammation
Cataract is a common complication of chronic uveitis of long duration. Exact cause of
cataract formation is not known. It is multi factorial. The two common offending factors are
the toxic effect of inflammation on the lenticular metabolism and Corticosteroids,
which form the sheet anchor of the treatment of chronic uveitis. Commonly opacification starts
in the posterior capsule. Later involving posterior cortex and ultimately covering whole of
the lens. Sometimes broad posterior synechiea which are difficult to break cause anterior sub
capsular opacities that are generally stationary.
Management consist of early and proper treatment of uveitis and steroid should be
used in its minimum possible strength and duration. No surgical intervention should be un-
dertaken unless the eye is quiet for at least six weak. The surgery should be followed by pro-
longed instillation of steroids as trauma of surgery results in flare up of uveitis.
Cataract in Lowes syndrome
Lowes syndrome consists of mental retardation, convulsion, renal stone formation, dia-
betes, albumen in urine. Ocular manifestation consists of bilateral nuclear cataract that may
be microspherophekic. Associated glaucoma may worsen the prognosis.
Cataract in myotonic dystrophy
Myotonic dystrophy is a heredofamilial disorder where muscles have increased tone
that is difficult to relax. This increased tone may be associated with progressive weakness of
the muscles. The condition is generally seen in adolescents. There is progressive loss of
facial expression and ptosis. Lenticular features consist of fine dots beneath the capsule that
may have polychromatic lusture. The stelate appearance of such opacities are called Christ-
mas tree cataract.
REFERENCE8
1. Duke Elder.S. and Wyber K.C. ; System of Ophthalmology, Vol. II : Anatomy of the
visual system 1st Edition pp.311338, Henry Kimpton, London, 1961.
2. Nema, H.V., Singh V.P. and Nema.N. ; Anatomy of the Eye and Adnexa, 2nd Edi-
tion pp.3135, Jay Pee Brothers, New Delhi, 1991.
3. Nancy Anderson Hamming, Apple D. ; Anatomy and embryology of the eye in Principle
and Practice of ophthal mology, Edited by Peyman G.A. Sander D.R. and Goldberg
M.F. Vol.I pp. 312 First India Edition. Jay Pee Brothers, New Delhi, 1987.
4. Banumathy S.P.; Anatomy of the eye Edited by G.Natchiar P-59-60 published by
Arvind Eye hospital and post graduate Institution, Maduri.
5. Sharma P. ; Essentials of Ophthalmology, First Edition pp. 133, Modern Publishers,
New Delhi, 2000.
6. Dutta L.C. ; Ophthalmology Principles and Practice, First Edition Page 226, Cur-
rent Book International Kolkata, 1995.
7. Scheic H.G. and Albert D.M. ; Embryology of the human eye in Text book of oph-
thalmology, Ninth Edition, pp. 8182 Wb Saunders Company, Philadelphia. 1977.
8. Ahmed E. ; Physiology of the crystalline lens in A Text book of ophthalmology , First
Edition, pp. 5152 Oxford University Press Kolkata.1993.
9. Adler F.H. ; Physiology of the eye, Sixth Edition, Edited Moses R.A. C.V. Mos by Co
St. Louis, 1975.
10. Duke Elder S. ; System of ophthal mology, Vol. 4th, The physiology of the eye and of
vision, Henry Kimpton, London, 1968.
11. Khurana A.K. ; Diseases of the lens in Ophthalmology second edition pp. 183212
New age international, New Delhi 2000.
12. Berger B.B. ; The lens cataract and its management in Principle and practice of
ophthalmology, Editors, Peyman. G.A.
13. Gittinger J.W. ; Congenital Cataracts in Manual of clinical problems in Ophthal-
mology Editors Gittinger J.W. and Asdourian G.K. , First Edition pp. 7576 Little Brown
and Co Boston 1998.
14. Singh D. ; Congenital and Infantile Cataracts in Current Ocular Therapy Fifth Edi-
tion, Edited By Fraun felden FT and Roy F.H. P-538-541 W.B. Saunders Comp. Philaled
phia 2000.
15. Foster A Gilbert C. Rahi F. ; Epidemiology of Cataract in childhood, A global Perceptive,
Jr Cat and Ref. Surgery 23: 601-604(99).
16. Duke Elders ; System of Ophthalmology Vol.-III, Part-II, First Edition pp. 688760.
Henry Kimpton London 1564.
17. Miller SJH ; The Lens in Parsons diseases of the eye 17th edition pp. 171173 Church-
ill Living stone, London 1984.
18. Dobree J.H. ; Cataract in Modern Ophthal Mology Vol-4, First Edition, Edited by A.
Sorsby, pp. 598618, Butter work London 1964.
19. Sharma P. ; Essentials of Ophthalmology first edition p. 136-, Modern Publishers
New Delhi 2000.
20. Dutta L.C. ; Blue Dot Cataract in Ophthalmology Principle and Practice, First
Edition Current Book Inter national Calcutta 1995.
21. Bonuik. V. ; Rebella in Current Ocular, Therapy Fifth Edition, Edited By Fraunfelder
F.T. and Roy F.M. pp. 6869 W B Saunders Company.
22. Beger W.P. and Paterson R.A. ; Paediatric Ophthalmology in Manual of ocular diag-
nosis and therapy, Edited By Deborah Pavan Langston , Third Edition.
23. Alcon D.M. ; Galactocemia in Current Ocular Therapy Edition 5th , Edited by Fraun
felder F.T., and Roy F.M. pp. 119120, WB Saunders Company Philadelphia 2000.
24. Cordes F.C. ; Galactosemic Cataract Am.J.Oph Oph 50:115 1960.
25. Epstein R.L. ; Inborn metabolic disorders and the eye in Principles and practice of
ophthalmology Vol. III, Edited by Peyman G.A Sauders D.R. , Goldberg M.F., First
Indian Edition pp. 17391741 I.F jay Pee Brother , New Delhi 1987.
26. Digeorge A.M. ; Ocular Changes in endocrine disease, in Pediatric ophthalmology,
Vol.II, Edited by Harley R.D Second Edition pp. 9881012, WB saunders company Phila-
delphia 1983.
27. Kansky J.J. ; Clinical Ophthalmology , Second Edition pp. 234258, Butter Worth
London 1989.
28. Calhoun J.H and Hiles D.A. ; Cataract and Intraocular lens implant in Pediatric oph-
thalmology Vol. I, Edited by Harley R.D. , Second Edition pp. 549582, WB Saunders
Company Philadelphia 1983.
29. Duke Elder S. ; Abnormalities in size of lens in system of ophthalmology Vol. III,
Part-II, First Edition pp. 694675, Hunry Kimpton London 1964.
30. Guemes A and Elias Iraboulsi ; Microspherophakia in Current ocular therapy fifth
edition edited by fraunfelder FT and Roy F.M. pp. 543544, WB saunders company
Philadelphia.
31. Guemes A and Elias Traboulsi ; Lenticonous and lengiglobus, in current ocular
therapy, fifth edition edited by Fraunfelder FT and Roy F.M pp. 542543.
32. Duke Elders ; Anomalies of the lens in system of ophthalmology Vol. III part-II first
edition pp. 688710, Henry Kimplon London 1964.
33. Ida Caroline Mann ; Developmental anomalies of the eye, Second Edition, London
1957.
34. Khurana A.K. ; Congenital anomalies of the lens in Ophthalmology Second Edition, p.
212, New age international (p) Ltd., New Delhi 2001.
35. Casper D.S. Simon J.W. etal ; Familial simple ectopia lentis. A case study , J Pediatric
ophth and strab 22 : 227, 230 1985.
36. Nelson L.B. ; Maumene IHEctopia Lentis, Surv Oph 27:143,1982.
37. Cross HE ; Ectopia Lentis et pupillae- Am.J. Oph 88:381 1979.
38. Duke Elders ; Multiple Syndrome in System of ophthalmology , Vol. III, Part-II , pp.
11021112, Henry Kimpton London 1964.
39. Gittinger J.W. ; Ectopia lentis in Manual of clinical problems in ophthal mology,
First Edition, Edited by Gittenger and Asdourian G.K. pp. 7880 little Brown and com-
pany, Boston 1998.
40. Epistine RL ; In born metabolic disorders of the eyes. In Principles and practice of
Ophthalmology Vol. III Edited by Peyman G.A. Sander D.R. Goldberg, M.F First,
Indian Edition pp. 17461754, Jay Pee brother , New Delhi 1987.
41. Cross H.E. and Jensen A.D. ; Ocular manifestation of Marfan syndrome and
homocystinuria. Am. J. Oph 75 : 405 : 1973.
42. Bloch R.S. ; Homocystinuria in Current ocular therapy , fifth edition, edited by
Fraunfelder FT and Roy F.H. pp. 110111, WB Saunder Company Philadelphia 2000.
43. Speath G L Barber G.W. ; Prevalence of homocystimuria among mentally retarded
evaluation of specific screening test, Pediatric 40 : 586 : 1967.
44. Hayasaka. S. AsanoY, Tadeka H. ; Lens subluxation in homocystinuria Acta Oph
62-425-431 1984.
45. Smith T.M. Holland M.G. Woody NC ; Ocular manifestation of familial
hyperlysinemia, Tran Am Acc of oph and otolyr 75 : 355 1971.
45a. Walton D.S. ; Weill marchesani Syndrome in current oculartherapy Fifth edition
edited by Fracenfeldes. F.T. Roy F.M. p. 179- WB Saunders company Philadelphia 2000.
45b. Wright K.W. ; Chrous G.A. Weill Marchesani Syndrome with bilateral angle closure
glaucoma. J. Pedioph Strab 22:129-132, 1985.
45c. Jones R.F. ; The syndrome of Marchessani Br J. oph 45: 377-381, 1961.
46. Zwann, J.T. ; Cataract in children : Diagnosis in Decision making in ophthalmol-
ogy, First Indian Edition, Edited by VanHeuven , W.A. J and Zwaan J.T. pp. 130131,
Har court, Brace and company Asia Ltd. Singapore 1998.
47. Singh D. ; Paediatric Cataract in CME Series, Published by All INDIA OPHTHAL-
MOLOGICAL SOCIETY, New Delhi.
48. Dutta N.K. Dutta L.C. ; Congenital cataract and other congenital abnormalities of the
crystalline lens in Modern ophthal mology Vol.-I edited by LE Dutta pp. 308312,
First Edition Kay Pee Brothers, New Delhi. 2000.
49. Mehta K.R. ; Lens Implantation in children in Modern ophthal mology Vol. I Edited
by Dutta L.C. pp. 372378, Jay Pee Brothers , New Delhi 2000.
50. Dada V.K. ; 10L in Children in 10L secrets , First Edition pp. 1924, Jay Pee Brothers
, New Delhi 1992.
51. Jagat Ram Pandey S.K. ; Infantile cataract surgery : Current techniques, complica-
tion and their management in Modern ophthalmology Vol. I, Edited by Dutta L.C., pp.
378384, Jay Pee Brothers, New Delhi 2000.
52. Wilson M.E. Apple D.J. Bluestin E.C.Wang XH ; Intra ocular lenses for Pediatric
implant, Biomaterials design and sizing, J-cat reft sur 20 : 584-591 1994.
53. Sharma Y.R. Neena Mittal Dinesh ; Pathogeneses and management of posterior
capsule opacification in Modern ophthalmology edited by Dutta L.C., Vol. I Edi-
tion - 2, pp. 400404, Jay Pee Bro, New Delhi 2000.
54. Allinson R.W. ; After Cataract in Current ocular therapy Ed. Fifth Edited by
Fraunfelder F.M. and Roy F.M pp. 535538 W.S. Saunders Company Philadelphia 2000.
55. Gimbal H.V. ; Posterior capsulor rehexis with optic capture in paediatric cata-
ract and intra ocular lens surery ophthalmology 103 : 175 -187 1996.
56. Haywood M ; and Lina M Marouf, Eye Trauma in Decision making in ophthalmol-
ogy edited by Heuven W.A.J and Znaan J.T., First Indian Edition 1998 pp. 5859,
Harcourt Brace & Comp, Singapore.
57. Dutta L.C. ; Diseases of the crystalline lens in Ophthalmology principle and prac-
tice, First Edition pp. 224243, Current book international Calcutta 1995
58. Fraunfelder F.T. ; Electric Injury in Current ocular Therapy Fifth Edition, Edited by
Fraunfelder FT. Roy F.M. pp. 272, WS Saunders Company Philadelphia 2000.
59. Miller S.J.H. ; Injuries to the eye in Parsoris Diseases of the eye, 17th Edition pp.
249250 Churchill Livingstone, London 1984.
CHAPTER 10
Glaucoma in Children
Anatomy of the structures related to glaucoma :
The structures of the eye related to glaucoma can be divided into :
A. Structure related to aqueous production.
B. Structures related to circulation of aqueous.
C. Structures related to drainage of aqueous.
D. The limbus
A. Ciliary processes
1
. These are the sites of production of aqueous humour. They are
70-75 in number. They arise from pars ciliaris of the ciliary body and project towards the
centre of the eye ball all around.
They have following layers :
1. Epithelium,
2. Tuft of blood vessels,
3. Stroma.
1. The epithelium is two layered.
(a) Pigmented epithelium
2
which is neuro ectodermal in origin and is forward
continuation of pigment epithelium of the retina. It is the outer layer of the
ciliary process. It extends from ora serrata to the iris. It is a single layer of cuboidal
cells.
(b) Non pigmented epithelium. This is the inner layer of ciliary process. It is also
neuroectodermal in origin. It is continuation of non pigmented layer of the retina.
It extends from ora serrata to the root of the iris. The non pigmented and
pigmented layers of the ciliary process are firmly attached to each other and do
not detach from each other like in retina.
3
2. Tuft of blood vessels. Each ciliary process has a tuft of blood vessel in the central
core. They are similar to choroidal vessels in nature without choricapillaries. The
endothelium of these vessels are thin and fenestreted
4
.
3. The stroma. The stroma of ciliary processes is very thin, it surrounds the blood
vessels and separates them from the surface epithelium. It is mostly muco poly
saccaharide with few collagen fibres.
4
334
GLAUCOMA IN CHILDREN 335
B. Structures related to circulation of aqueous. The aqueous is produced from the
ciliary processes and poured in posterior chamber from there it passes through the pupil in to
the anterior chamber where it circulates and leaves the anterior chamber via angle of anterior
chamber.
The structures related to circulation of aqueous are :
1. The aqueous chambers of the eye.
2. The angle of the anterior chamber.
1. The aqueous chambers. The ciliary body, zonules and the lens divide the eyeball in
two unequal chambers - A large vitreous chamber and a smaller aqueous chamber.
The aqueous chamber is again divided into two unequal parts by the iris into :
(a) A smaller posterior chamber
(b) A larger anterior chamber
(a) The posterior chamber is a space bounded anteriorly by the posterior surface of
the iris. Posteriorly it is bound by periphery of anterior lens capsule, anterior plane
of zonule and anterior part of the ciliary process.
(b) The anterior chamber is a convex space with convexity forward. The convex sur-
face is bounded by the posterior surface of the cornea. The flat posterior surface is
formed by a chink of anterior surface of ciliary body, the iris and the anterior lens
capsule. The anterior chamber is lined by endothelium except on the posterior sur-
face formed by lens capsule. The endothelium is absent in crypts of the iris also. The
junction of the anterior and posterior surfaces is the angle of the anterior chamber.
Being a plano convex space, its depth is maximum at the centre and gradually di-
minishes on the periphery where the two surfaces meet. Depth of the anterior cham-
ber is genetically determined. It is shallow in new born, attaining adult depth after
two years. It is deep in myopia, buphthalmos, megalo cornea, keratoconus, subluxation
of lens and aphakia. It is shallow in micro-ophthalmos, micro-cornea, cornea plana,
dysgenesis of anterior chamber, narrow angle glaucoma, swollen lens, hypotony and
intraocular tumour pushing from behind.
The angle of anterior chamber. The angle formed by the posterior surface with the
anterior surface is almost 45. In normal eye the angle is not blocked even with maximum
mydriasis.
Structures related to drainage of aqueous
C. The Trabecular mesh work. At the extreme periphery of the cornea on its inner
side of the anterior chamber directly under the limbus is a depression that runs all round the
angle of the anterior chamber. This is called scleral sulcus. The posterior lip of the sulcus is
sharp and is called the scleral spur. The ciliary body is attached to the scleral spur. A sieve
like structure bridges the scleral sulcus and is called trabecular meshwork. The meshwork
extends from the scleral spur to periphery of the cornea. The line where the trabecular meshwork
is attached to the corneal periphery is called Schwalbes line which is the thickened end of
Descemets membrane. It forms an important landmark in gonioscopy. The trabecular
meshwork converts the scleral sulcus into canal of irregular calibre called Schlemms canal.
The trabecular meshwork is a collection of tissues that on section looks more or less like
a triangle, the apex of which lies at the Schwalbes line. The base is formed by scleral spur and
ciliary body, the outer wall is formed by canal of Schelemms, the inner wall faces the anterior
chamber.
The meshwork as per histological picture is divided into
8
Corneoscleral meshwork,
uveal meshwork, and endothelial meshwork. The uveal meshwork is adjacent to canal of
Schlemm hence called juxta canalicular meshwork.
The canal of Schlemm. This is a venous channel that runs all round the angle of the
anterior chamber in the sclera. It has irregular calibre. At most of the places it is a single
endothelium lined channel, at places it breaks into tributaries and becomes multi channelled.
The tributaries may merge with the main channel at places.
On the outer side the canal-of-Schlemm, is joined to episcleral and conjunctival veins.
The episcleral veins drain into anterior ciliary veins, the conjunctival veins drain into
palpebral and angular veins.
D. The limbus. The limbus is most important anatomical landmark for glaucoma sur-
gery. It is an area 1 mm wide all round the cornea representing the junction of cornea on one
hand, and conjunctiva, episclera and sclera on the other hand. From surgical point of view it
has been divided into two circles and two zones in between
5
. The circles are formed by ante-
rior limbal border, that is insertion of conjunctiva and Tenons capsule in the cornea. It
overlies the termination of Bowmans membrane. The posterior limbal border lies ap-
proximately over the scleral spur. An imaginary line between the anterior and posterior
limbal border is called mid limbal line that lies over the Schwalbes line. The mid limbal line
is the junction of blue corneal limbus to the scleral limbus.
The space between the anterior limbal border and mid limbal line is called anterior
limbal zone. The space beyond the mid limbal line upto posterior limbal line is called poste-
rior limbal zone.
The limbus does not form a ring of uniform width all round the cornea. The anterior
limbal zone is narrowest at the horizontal plane of cornea and widest at superior limbus.
The development of anterior chamber. The presence of anterior chamber becomes
evident at an early stage of embryo genesis i.e. 20 mm (7 weeks)
6
. As the lens separates from
the surface ectoderm, the surface ectoderm is converted into corneal epithelium. A wave of
mesoderm invades the space between the corneal epithelium and the lens to develop stroma of
the cornea, endothelium of cornea, iris and iris stroma. The space between the iris mesoderm
and the corneal stroma is anlage of the anterior chamber.
7
This space remains very narrow as
a cleft up to fifth month antenatal and then develops rapidly with the growth of the anterior
segment.
The canal of Schlemm develops at about nine weeks
6
as a vascular channel at the level
of recess of angle and gradually moves anteriorly in relation to the iris.
The angle of the anterior chamber develops two months after the canal of Schlemm.
8
The trabecular meshwork develops from vascular mesoderm originating from the mar-
gin of the optic cup.
6
At birth the canal of Schlemm and the trabeculum both lie at the angle of
the anterior chamber.
9
The drainage system becomes functional just before birth.
Congenital anomalies of anterior chamber. Majority of glaucomas in children are
either congenital or developmental hence it is necessary to know the common congenital
anomalies of anterior chamber where most of the lesions lie.
The anterior chamber develops as a chink in the mesoderm between the corneal stroma
and iris stroma. It is but natural that congenital anomalies of anterior chamber may involve
cornea, iris and angle of anterior chamber in various combinations.
Roughly anomalies of anterior chamber can be divided into following groups :
A. Iridocorneal dysgenesis.
B. Mesodermal dysgenesis (anterior chamber cleavage syndromes)
C. Trabeculodysgenesis
In the first two groups the cornea is frequently involved and glaucoma is a common
feature that manifests in childhood.
In trabeculodysgenesis, the trabecular tissue is mostly involved and cornea is either
spared or effected minimally. Glaucoma in the form of primary congenital glaucoma is the
most important clinical finding.
A. Irido corneal dysgenesis
13
. This is a rare condition, is most commonly known as
essential iris atrophy
14
and also as Cogan Reese syndrome or Chandler syndrome due
to overlapping signs however some authors divide them as different entities. Endothelial de-
fect being a prominent feature, the term iridocorneal defect is more appropriate.
The exact etiology of the condition is not known
15
. This is non-hereditary more com-
mon in females, it is unilateral mostly, rarely the other eye may be involved. Common age
for it to become symptomatic is third and fourth decade. The disease starts in childhood and
is detected during routine eye examination. No associated systemic disorders have been
reported with the condition. Specular microscopy reveal peculiar cells in the endothelium,
the cells are known as iridocorneal endothelial cells (ICE)
16,17,18
. The ICE cells are
pathognomic and are supposed to be the cause of endothelial damage. The ICE cells migrate to
the trabecular meshwork and cause mechanical obstructing resulting in glaucoma. The abnor-
mal endothelial cells also cause corneal decompensation resulting in corneal edema. Corneal
edema is independent of raised intraocular tension. The progress of the disease is slow,
only 50% of eyes require antiglaucoma treatment for raised intraocular pressure that range
between moderate and severe.
The ocular findings are :
Peripheral anterior synechea, full thickness hole in the iris, pseudo polycoria, shift-
ing of pupil away from the holes towards the peripheral anterior synechea. The iris is wider in
the sector where the holes develop, ectropion of uvea, heterochromia of iris. The iris is atrophic
at places, there may be nodules on the iris. A thin transparent membrane is seen over the iris
surface. The vision is generally poor due to corneal edema and glaucoma.
The condition should be differentiated from posterior polymorphic corneal dystrophy
which may be seen in children, may be associated with glaucoma and hazy cornea and is
bilateral.
Treatment is directed towards glaucoma. Initially medical treatment is tried if it fails.
Trabeculectomy is the best surgical choice. The trabeculectomy hole may be blocked by ICE
cells. Next alternative is Molteno valve.
B. Mesodermal dysgenesis (Anterior chamber cleavage syndrome)
Mesodermal dysgenesis consists of :
1. Posterior embryotoxon
2. Axenfeld anomaly
3. Riegers anomaly
4. Peters anomaly
1. Posterior embryotoxon
18, 19
. This is the mildest form of mesodermal dysgenesis.
20
This anomaly is present in all forms of mesodermal dysgenesis. Ten to thirty percent of normal
eyes show posterior embryotoxon without any other symptom
21
except cosmetic blemish. The
Schwalbes line is prominent and shifted centrally. It is formed by splitting of Descemets mem-
brane into two leaves that contains collagenous tissue that is nearer to trabecular meshwork
than cornea. The thickened Schwalbes line projects in the AC as a ridge all around 1 mm
inside the limbus or it may be fragmented. Its appearance is similar to arcus juvenilis that
develops at the end of the Bownans membrane, rest of the cornea, iris, angle and trabecular
mesh work are normal. The condition does not require any treatment.
2. Axenfelds anomaly. This condition may be considered to be more advanced form of
posterior embryotoxon. In this condition strands of peripheral iris tissue are attached to the
thickened Schwalbes line. Glaucoma need not be present in all cases because angle remains
unaffected in most of the eyes. The iris and pupil are also normal. Glaucoma when present is
difficult to manage. The condition is bilateral without any predilection for sex. The condition
becomes obvious in childhood due to prominent posterior embryotoxon. Glaucoma is not met
with in childhood. It is a feature of late adolescence or adulthood.
3. Riegers anomaly
22, 23
. Riegers anomaly represents gross anomaly of the struc-
tures that arise from mesoderm. It involves cornea, iris and trabeculum in various combi-
nations. The corneal change is represented by posterior embryotoxon. Iris shows hypoplasia of
stroma with full or partial thickness holes that may be present as deformity of the pupil in the
form of corectopia, dyscoria, pseudocoria or slit like pupil. There may be positive trans-
illumination of the iris. The peripheral iris is attached to the thickened Schwalbes line as
slender or broad adhensions. These adhesion may be in patches or may encircle the whole of
the angle. The trabecular meshwork itself is normal. The iridocorneal adhesion is the
cause of glaucoma that is seen in half of the cases. Rise of intraocular tension may go unno-
ticed unless looked for specifically. Severe rise of tension in infancy causes typical buphthalmos.
Other less common findings are
23A
. Persistent hyaloid vasculature, partial or total absence of
choroidal and retinal pigment epithelium, dysplasia of retina. The lens is not primarily in-
volved, lenticular changes are secondary to glaucoma.
Skeletal changes are sometimes seen in Riegers anomaly limited to face and teeth.
There is hypoplasia of maxilla, hypodontia and micro-dontia.
The ocular features are bilateral but not symmetrical. Common inheritance is autosomal,
sporadic cases are also known.
Once glaucoma develops the aim is to keep the tension low medically if that fails surgi-
cal intervention may be required.
4. Peters anomaly
24, 25, 26
. This condition is rarest among all mesodermal
dysgenesises. The most striking feature is a central corneal defect. It is a bilateral condi-
tion, present at birth, seen equally among boys and girls, has autosomal recessive inherit-
ance. It can be divided into following groups :
(a) Type Iassociated with posterior embryotoxon. It has combined picture of Axenfelds
and Riegers anomaly with central corneal defect in the form of central facet
at the level of Descemets membrane. The endothelium is absent in the facet. The iris
is attached to the cornea all around the defect, leading to stromal opacity. The ante-
rior chamber is slit like in between the central adhesion and peripheral iridocorneal
synechea. The lens is in place.
(b) Type IINo evidence of Axenfeld or Riegers anomaly. Only feature is central
iridocorneal adhesion as in type one.
(c) Type IIIThe findings are similar to type II. The anterior lens capsule is ad-
hered to central corneal defect. The anterior chamber is shallow. The lens soon be-
comes opaque. Glaucoma is seen in fifty percent of cases. Other anomalies include
microphthalmos, cornea plana, sclerocornea, vitreo retinal defects.
C. Trabeculo dysgenesis
27, 28, 29
. In this condition the developmental anomaly lies in
the trabecular meshwork, rest of the angle, anterior chamber and iris are developmentally
normal. It is present at birth, more common in boys, it is familial, more than one sibling may
be effected. The inheritance is heterogeneous but may be autosomal recessive. The exact
mechanism of the anomaly is not well understood. There are two schools of thought, the first
believes that there is an anatomical mal-developmental. The other postulates that there is
arrest of maturation. There is an incomplete cleavage of uveal periphery from the corneoscleral
wall. The meridional fibres are inserted either in the trabecular meshwork or in Schwalbes
line.
Gonioscopically the angle is wide but the trabecular meshwork is not clearly visible
due to a thin membrane like structure on the surface of the trabeculum. This is eponymed as
Barkans membrane. Many authors have doubt about its presence. As the trabecular
meshwork is plastered with mesodermal tissue, the aqueous fails to reach the canal of
Schlemm resulting into rise of intra ocular tension that causes primary infentile glau-
coma or buphthalmos with its consequences.
Comparison between iridocorneal dysgenesis and mesodermal dysgenesis.
Irido corneal dysgenesis Mesodermal dysgenesis
Age Symptoms develop in second and third
decade, can be discovered in childhood
if looked for.
Symptoms develop late but can mani-
fest in childhood.
Sex More in females Equal in both sexes
Inheritance Non hereditary Polygenic inheritance
Aqueous humour
A. This is a crystal clear fluid that fills both the anterior and posterior chamber. The
exact time it starts forming is not establishing but must be before birth because the aqueous
drainage system is ready to function before birth
6
.
B. The functions of aqueous humour are to :
1. Give and maintain an optimal intraocular pressure compatible with vision.
2. Provide nutrition to avascular structures of the eye i.e. the cornea and lens.
3. Remove waste products of metabolism.
4. Act as an important optical medium.
C. The composition of aqueous humour :
The aqueous humour differs greatly from plasma from which it seems to be derived by
the way of its low, protein, sodium, bicarbonate and glucose. The ascorbate content is
very high, chlorides and lactic acid have higher concentration.
The aqueous humour is acidic, and slightly hypertonic. The albumin globulin, ratio
is same as in plasma. The human aqueous contains only IgG and not IgA and IgM.
There is hardly any change in the composition of aqueous in anterior and posterior
chamber. The composition of aqueous in phakic and aphakic eyes are the same proving thereby
that lens does not participate in formation of aqueous. The refractive index of aqueous is 1.337
10
which is less than both the cornea and lens.
D. Formation of aqueous humour. The aqueous humour is derived from the plasma
in the capillary network of the ciliary process. There are three theories of production of
aqueous in the ciliary processes.
1. Diffusion
2. Ultra filtration
3. Secretion
The plasma passes through three tissue layer that allow fluid with specific quality to
pass through it. They are : The wall of the capillary, the stroma of the ciliary process and the
Laterality Generally unilateral Generally bilateral
Corneal involvement Mostly due to endothelial damage, but
may have corneal opacity at a place
where iris touches the cornea.
Posterior embryotoxon, strands of iris
touching cornea.
Corneal edema Present, independent of glaucoma Glaucoma is the cause of corneal
edema.
Pupil Distorted, shifted towards peripheral
anterior synechea.
Distorted may have pseudo polycoria,
corectopia and ectropion of uvea.
Systemic involvement Nil May have facial, dental and skeletal
abnormality.
Visual prognosis Poor Poor
ciliary epithelium. None of the above process fulfil all the conditions to be fit into one process.
It seems all the processes take part in different proportion. The most important out of the
three is secretion.
E. Rate of formation of aqueous humour. The rate of aqueous production is called
in flow of aqueous. It is measured in micro litres per minute (ml/min). It is not uniform
throughout the day and night. It shows a diurnal variation. The normal range in adults is
2.8 to 4.3 ml/mt The production is least in the night
12
. The inflow increases following drinking
of large volume of water and is decreased by ingestion of many locally and systemic acting
drugs.
F. Rate of outflow. To maintain intraocular pressure within normal range, there should
be an equilibrium between inflow and outflow of aqueous. The aqueous is produced in the
ciliary processes that passes successively through posterior chamber, pupil and anterior chamber
where the aqueous circulates between the cornea and the iris by convection current. The
aqueous leaves the anterior chamber via trabecular meshwork, canal of Schlemm and is
finally picked up by episcleral vessels. This mode of outflow that is about 85% of total out-
flow is called conventional outflow, rest is drained by what is known as unconventional
outflow. The unconventional path comprises of uveoscleral and uveovertex pathway. Pres-
ence of uevoscleral channel is well etablished. There is no unanimity regarding presence of
uveo vertex path.
The facility of outflow is measured by a non invasive method called tonography. The
outflow of facility is expressed in ml/min/mm.Hg. The outflow canbe increased by applying
pressure over the globe and by topical drugs. The normal value is about 0.20 ml/mt/mm Hg.
Intraocular pressure :
A. Normal intraocular pressure ranges between 15-20 mm of Hg. It is hardly influenced
by age or sex. It shows a physiological variation of 2 to 3 mm between the highest and lowest
pressure during the days and is called diurnal variation. In most of the eyes the tension is
maximum in the morning however some persons may show an evening rise. A biphasic rise is
also possible.
B. Measurement of intraocular pressure - Intraocular pressure is the lateral pressure
exerted by intraocular contents on the outer coats of the eye i.e. the cornea and the sclera.
There are two clinical methods of clinical methods of measuring it.
1. Indentation tonometer
2. Applanation tonometer
The applanation tonometer is more accurate than indentation as it is not influenced by
scleral rigidity. The advantages of indentation tonometer are that it is very cheap, easy to
handle and gives fairly accurate reading. Out of all applanation tonometers most widely used
tonometer is Goldmanns tonometer. The other is Draeger tonometer. The applanation
tonometer can be mounted on a slit lamp or may be handheld (Perkins).
Other used applanation tonometers are :
A. The pneuno tonometer
B. The air puff non contact tonometer.
Gonioscopic anatomy of angle of anterior chamber. The angle of anterior cham-
ber has been called the cockpit of glaucoma. It is not visible in normal eye with oblique illumi-
nation. To see it, special optical devices called gonioscopes are employed. The gonioscopic
anatomy of the anterior chamber has following visible landmarks from peripheral iris to pe-
ripheral cornea.
A. Ciliary body. This is the part of the ciliary body visible from the insertion of the iris
to the ciliary body to the insertion of the ciliary body in the scleral spur. The width of ciliary
body is wider in myopia and narrower in hypermetropia. The colour is grey to dark brown.
B. Scleral spur. This is the part of the sclera to which the ciliary body is inserted. This
represents the posterior sharp edge of the scleral sulcus. The ciliary body is attached little
behind the posterior attachment of trabecular meshwork. On gonioscope, it looks as a promi-
nent white line in front of ciliary band and behind the trabecular meshwork.
C. The trabecular meshwork. The trabecular meshwork is porous structure that
stretches across the angle of anterior chamber, bridging it between the scleral spur and
Schwalbes line. It has two distinct parts, a non-functioning anterior part and a func-
tioning posterior part.
The angle of anterior chamber is examined for presence of pigment, new vessels,
exfoliations, synechia and foreign body. Common causes of increased pigmentation are -
acute and chronic glaucoma, uveitis, trauma.
D. Schwalbes line. This is the termination of Descemets membrane and represents
the anterior limit of the trabecular meshwork. It looks like a translucent circular band. In case
of posterior embryotoxon, it is very prominent and shifted anteriorly.
E. The Schlemms canal. This is not visible in all eyes. When visible it looks like a
darker line in the lower trabeculum. If the episcleral pressure is higher than intraocular
pressure blood may be seen in Schlemms canal. However blood can also be seen in hypotony
with normal episcleral pressure.
F. The angle recess is the term used to denote dipping of iris in the ciliary body as it
inserts in the ciliary body.
Gonioscopy. Gonioscopy is a non invasive clinical method of visualising the angle of
the anterior chamber.
The rays arising from the angle of anterior chamber do not leave the eye. They are
reflected back hence are not visible because the rays arising from angle pass through aqueous
and cornea into air which has refractive index less than the former two. The critical angle of
the rays is more than 46 hence they are reflected back. This difficulty is overcome by elimi-
nating air corneal interface and allowing the rays to pass a contact lens making the angle
visible. The device is called gonioscope.
There are two types of gonioscopes
1. Direct gonioscope
2. Indirect gonioscope
The direct gonio-lens has an anterior curvature that does not allow the critical angle
to be reached hence the rays are refracted at goniolens air interface. In case of indirect
gonioscope, it has a contact lens and either a reflecting mirror or a prism. The reflecting
angle is almost 90 to the lens air interface hence can be picked up by slit lamp or an operating
microscope. A large number of gonioscopes in both the categories are available, each with
specific design and purpose.
Childhood Glaucoma
Roughly two percent of population above forty years suffer from some type of glau-
coma or other, mostly chronic symptomsless primary open angle glaucoma. The incidence rises
with age and by eighty years the percentage reaches an alarming figure of five percent.
Fortunately, awareness of adut prevalence of glaucoma is quite high nowadays and more and
more persons are benefited by early detection and proper treatment. Childhood glaucoma dif-
fers from adult glaucoma in many ways.
30
Most of the cases are symptomatic hence seek medi-
cal advice early unless it is unilateral and mild. Loss of vision is fast and gross. If the condition
is not treated, permanent visual damage is inevitable. Prevalence of childhood glaucoma is far
less than adult glaucoma hence chances of missing it are more.
Classification of glaucoma in children. There is no unanimity regarding classifica-
tion of glaucoma. There are many ways to classify them. Some of them are :
A. According to age of onset
1. Those manifesting within first two years of age.
2. Those developing after two years but before puberty.
3. Developing in early childhood.
B. According to involvement of ocular structures -
1. Those due to developmental anomalies in the eyes.
(a) Anomaly in the angle only.
(b) Multiple anomalies of anterior segment including angle.
2. Secondary to causes not related to developmental anomalies of the eye.
The group A is designated as congenital or developmental glaucoma.
The group B consists of conditions that constitute a heterogeneous group of glaucoma
called secondary glaucoma, both the groups can have wide or narrow angle.
C. The most widely accepted classification that fulfils almost all criterions is one given by
Duke Elder in 1964
30,31
.
This divides congenital glaucomas into :
1. Developmental buphthalmos
(a) Simple buphthalmos
(b) Associated buphthalmos
2. Secondary congenital buphthalmos
All the eyes under three years of age that have raised intraocular tension will go into
buphthalmos irrespective of cause.
Simple buphthalmos
35, 36
. The term buphthalmos is not a specific clinical en-
tity. It embraces many diverse conditions. The only common feature among them is enlarge-
ment of the globe secondary to raised intraocular tension. The eye ball is enlarged and
looks more or less like a bovine eye, to be specific eye of an Ox. Buphthalmos is seen not only
in congenital glaucoma but in all conditions that cause rise of intraocular tension under three
years of age.
The cornea stops enlarging after three years of age so enlargement of the globe is either
arrested or minimal by three years.
The term buphthalmos will be used in the text to denote primary developmental glau-
coma as a general practice rather than its accuracy.
Primary buphthalmos is relatively uncommon disorder. It is inherited as autosomal
recessive trait. It may be present in the siblings. Some of the older members of the family
may have early onset of chronic simple glaucoma. In sixty percent cases it is bilateral which
need not be symmetrical. Due to some ill understood causes it is more common in boys. The
ratio between boys to girls is 3:2. The disease may either be present at birth as true congeni-
tal glaucoma, or may manifest in first year as infantile glaucoma. On rare instances the
disease may have still late presentation. These are the cases where instead of complete shut-
down of flow of aqueous, the flow is slower than normal and rise of tension late and slow.
In true congenital glaucoma rise of intraocular tension and its consequences are present
in intrauterine life. The disorder is caused due to trabecular dysgenesis of angle of ante-
rior chamber
32, 33
, rest of the eye does not suffer from any other congenital anomaly, the
child does not have any other extra ocular or systemic congenital anomaly.
The eye in primary buphthalmos :
1. Enlargement of cornea. The most spectacular changes are seen in cornea, which
is enlarged in all directions. At birth the diameter of the normal cornea is 10.5 mm to
11.0 mm, by one year it varies between 11.5 mm and 12.0 mm, at two years it should not
exceed 12 mm and enlargement virtually comes to stand still. Corneal diameter more than
13 mm at any age and more than 12 mm at 1 year should be seen with suspicion and inves-
tigated for buphthalmos.
34
In advanced cases of buphthalmos diameter as high as 18 mm have
been reported. The size of the cornea depends on range of intraocular tension and duration of
raised intraocular tension. Very high tension cause enlargement quickly. Relatively low ten-
sion takes more time to reach the same dimension.
2. Stretching of the cornea. Stretching of the cornea in all meridians cause thinning
of the cornea that alters the curvature of the cornea, initially the corneal curvature is in-
creased. The enhanced corneal curvature is not uniform. There is a relative flattening in the
centre of cornea. As the cornea stretches, the Descemets membrane gives away giving rise
to striated opacities that assume various forms. They start as fine branched striae in the cen-
tral part, the rupture in the centre looks like a horizontal line while ruptures on the periph-
ery are concentric with limbus. The peripheral ruptures are more common in lower part of
the cornea. There may be more than one rupture in the same cornea. The lines produced by
rupture of Descemets membrane in primary buphthalmos are known as Haabs striations.
They may be present in other forms of congenital or developmental glaucoma as well. The
lines become opaque and more prominent with time. The Haabs striations must be differenti-
ated from rupture in Descemets membrane due to birth trauma, specially following forceps
delivery. Such ruptures are vertical. The Haabs striations may become whirl like sometimes.
The ruptured Descemets membrane allows aqueous to permeate into the stroma and cause
clouding of corneal epithelium, lacrimation and photophobia.
3. The stretching, clouding, development of opacity in the cornea, relative flattening in
the centre of the cornea lead to irregular astigmatism.
The stretching is not limited to the cornea, whole of the globe undergoes stretching and
enlargement, due to high intraocular pressure in an immature eye.
4. Stretching of sclera :
1. The stretching of sclera is not uniform in all directions. It is most pronounced in
anterior posterior diameter followed by horizontal diameter. The vertical diameter is
least effected.
2. Due to unequal stretching of the globe instead of becoming an enlarged sphere it
becomes more or less like an egg.
3. Over all enlargement of globe specially in anterio posterior axis gives an appearance
of pseudo proptosis.
4. The other effect of enlargement of outer coat of the eye is obliteration of the
corneoscleral sulcus that brings about significant change in the surgical anatomy of
the limbus resulting in accidental placing of sclerotomy too posterior during surgery
5
.
5. Stretching is most marked in pre-equatorial area than posterior sclera. In long standing
cases there may be formation of equatorial or ciliary staphyloma.
5. Anterior uvea. Stretched sclera causes an increase in limbal diameter in all merid-
ians. The ciliary body that is attached to the scleral spur, is shifted peripherally resulting in
stretching of the zonules and the iris. The stretched iris becomes thin and the pupil be-
comes larger than normal. Other cause of large pupil in buphthalmos of course is optic nerve
change and tension related iridoplegia in late stages.
As the sclera moves more peripherally, the suspensory ligament becomes tight and flat-
ten the lens. A strained suspensory ligament may give way, resulting in subluxation or dis-
location of the lens.
6. Anterior chamber. The effect of increased corneal curvature, thinning of iris, and
flattening of lens is deepening of anterior chamber. A clear deep anterior chamber with
large cloudy cornea in a child should be investigated for primary buphthalmos.
7. Lens. Flattening of lens and sometimes subluxation of lens leads to tremulousness of
lens. After sometimes the clear lens or lens with variable degree of opacity may dislocate,
commonest direction of dislocation is posteriorly, however large pupil can predispose anterior
dislocation.
8. The vitreous, choroids and retina do not show any significant change.
9. Optic disc. The changes in optic nerve in primary buphthalmos are prominent. The
changes are mostly due to stretching of the sclera and backwards shifting of lamina cribrosa.
The optic nerve changes are reversible. If the intraocular tension is brought low and kept
low, the optic nerve changes shift towards normal. However after three years when anterior
segment enlargement stops, the optic nerve changes become accentuated and progress.
10. Intraocular tension. Intraocular tension is generally above 40 mm Hg., when the
child presents for the first time. It may be as high as 80 mm Hg.
11. Error of refraction. Due to increased axial length, the eyes are moderately my-
opic. This myopia is partially neutralised due to flattening of central cornea, flattening of lens
and posterior shift of lens. Myopia is invariably associated with irregular astigmatism.
12. Vision. Vision in an eye with primary buphthalmos is always subnormal, due to :
1. Corneal opacity, 2. Error of refraction, 3. Lenticular changes, 4. Changes in optic
nerve head, 5. Amblyopia, 6. Nystagmus.
Main diagnostic features in primary buphthalmos :
A. The main diagnostic feature of primary buphthalmos lies in the angle of anterior
chamber. The angle should be examined by a suitable gonioscope in every eye that
has large, cloudy cornea with deep and clear anterior chamber.
B. The examiner should have a good knowledge of gonioscopic appearance of angle of ante-
rior chamber in neonate and infants that differs from that of an adult angle.
C. The angle of a new born has following features :
1. The angle recess is very narrow.
2. The peripheral iris and blood vessels produce a scalloped appearance.
3. The trabecular sheets are translucent.
37, 38
4. The anterior part of the ciliary body and its insertion in the scleral spur is seen
clearly.
D. The gonioscopic examination in case of primary buphthalmos is done by direct
gonioscope of Koeppe or its modification.
E. The advantages of direct gonioscopy are :
1. It can be done by placing the child on its back.
2. Both the eyes can be examined simultaneously by using two gonioscopes and the
changes compared in two eyes.
3. 360 of an angle is visible at a given time.
4. It can be done either by handheld microscope with suitable illumination or operating
microscope.
5. An additional advantage of Koeppes gonioscopy is that direct ophthalmoscopy
can be done over the gonioscope because it gives extra magnification.
Neonate need not be given general anaesthesia to do direct gonioscopy but a child above
three months may not co-operate without sedation, may even require general anaesthesia.
If the cornea is hazy due to epithelial edema, the epithelium should be scrapped either
mechanically by the blunt side of a cataract knife or chemically by rubbing the cornea with
swab soaked in alcohol
32,33
. Instillation of 5% hyper tonic sodium chloride drop few times may
also clear epithelial haze.
F. Gonioscopic findings
34,35,36
:
1. Gonioscopic findings are not uniformly spread all over the angle, it need not be sym-
metrical in two eyes.
2. The other eye should also be examined even when it is apparently normal.
3. In primary buphthalmos, the anterior chamber is of normal depth before corneal
stretching and enlargement ensue, with rise of tension and passage of time the ante-
rior chamber deepens.
4. The angle is wide open.
5. On the inner surface of trabecular meshwork a translucent membrane called
Barkans membrane may be present.
6. There is no angle recess.
34
7. The peripheral iris is inserted in the line of Schwalbe.
8. The scleral spur is not well visible.
G. Measurement of Intraocular tensionIntraocular tension is primary buphthalmos
is always raised, it may vary between 40 mm to 80 mm Hg depending upon severity of
trabecular block, duration and therapeutic measures undertaken. The tension is meas-
ured either by Schiotzs tonometer, Perkins handheld applanation tonometer
or tonopen. In children over three years tension should be recorded under general
anaesthesia under a competent anaesthetist well versed in paediatric anaesthesia. Al-
lowance should be made for general anaesthesia induced rise of tension. Some of the
anaesthetic agents themselves alter intraocular tension. halothene and barbiturates
lower tension, while ketamine elevates the tension.
38,39,40
Tension recorded in a strug-
gling child is not good enough. Digital recording of tension should always be
avoided.
Diagnosis
A. Diagnosis of full blown case of primary buphthalmos is not difficult, provided there is
enough awareness among the attending neonatologist, obstetrician and attending nurse.
B. Unless looked for carefully, chances of missing the condition in very high specially in
bilateral cases with mild to moderate manifestation.
C. Unilateral cases draw early attention of the mother to relatively prominent eye. Besides
prominent eye/eyes, other symptoms that point towards possibility of buphthalmos are:
lacrimation, photophobia, blepharospasm, and large cloudy cornea. The child
with buphthalmos has poor appetite and the child is withdrawn. The child with bilat-
eral disease prefers to be in dark place, keeps both the eye closed and moves the face
away from light. In unilateral cases the child keeps the effected eye closed. As the child
grows, the symptoms become more pronounced. The child may open the eyes at night
due to reduced intensity of light and then it is realised that the child has poor vision.
D. A positive history in siblings, in first cousins, require thorough examination of a new
born. Similarly children and grand children of persons who had early onset of wide
angle glaucoma should also be suspected to have primary buphthalmos and investi-
gated.
E. The diagnosis is confirmed by measuring intraocular tension, corneal diameter,
gonioscopy and evaluation of optic nerve head.
Differential diagnosis of primary buphthalmos consists of all cases with
A. Watering of the eye.
B. Photophobia and blepharospasm
C. Cloudy cornea
D. Prominent eyeball in a neonate.
A. Watering should not be mistaken as congenital nasolacrimal duct obstruction, oph-
thalmia neonatorum or corneal abrasion.
1. The congenital nasolacrimal duct obstruction generally manifests after three weeks
of birth, has mucoid or mucopurulent discharge may have positive regurgitation test.
The cornea is bright, anterior chamber depth is normal and pupil of normal size and
reaction.
2. Ophthalmia neonatorum develops within first three to four days. It is invariably
bilateral, the lids are moderately edematous, discharge is purulent. Regurgitation
test is negative, cornea AC and pupil are within normal range.
3. Corneal abrasion are mostly due to birth trauma. The discharge is watery. The abra-
sion stains with fluorescein, otherwise cornea, AC and pupil are normal.
B. Photophobia and blepharospasm are generally due to corneal abrasion, neonatal
keratitis and anterior uveitis. Interstitial keratitis comes into differential diagnosis only
if a child with lacrimation and photophobia present after three years of age. The cornea
in interstitial keratitis has uniform haze due to deep vascularisation.
C. Cloudy cornea :
Cloudy cornea may be due to
1. Endothelial dystrophy
2. Mucopoly saccharoidosis
3. Mucolipidosis
D. Prominent eyeball. causes of prominent eyeball in neonate may be :
1. True proptosis due to a retro bulbar mass and craniocynostosis and congenital myopia.
E. Other causes of secondary and associated glaucoma in children should be excluded.
Clinically and by specialised investigation like :
1. Ultrasonography
2. X-ray skull and orbit
3. CT
4. M.R.I.
The conditions that require special attention are retinoblastoma, retinopathy of pre-
maturity, persistent primary hyper plastic vitreous
41,42
.
Management of primary buphthalmos. The definitive treatment of primary con-
genital glaucoma is surgery. Medical treatment is employed for temporary reduction of
intraocular pressure till the facility of surgery becomes available, sometime medical treat-
ment may have to be re-introduced after surgery to control residual glaucoma.
Treatment of primary buphthalmos is divided into :
A. Specific
1. Surgery
2. Laser
3. Medical
B. Ancillary treatment consists of management of
1. Associated errors of refraction.
2. Prevention and management of amblyopia.
3. Low vision aid.
4. Rehabilitation.
1. Surgery consists of various microsurgical procedures.
34, 35, 37, 40, 41, 42
They are :
(a) (i) Goniotomy through operation gonioscope.
(ii) Direct goniotomy in cloudy cornea.
(iii) Gonio puncture.
(b) (i) External trabeculotomy
(ii) Trabeculectomy
(iii) Trabeculotomy combined with trabeculectomy.
(c) Draining devices
(d) (i) Laser
(ii) Cryo
(iii) Ultrasound
(a) (i) Goniotomy. Goniotomy is the preferred surgical procedure for all buphthalmic
eyes where surgery is indicated. This is a highly skilled microsurgical procedure done
through a suitable operating gonioscope under general anaesthesia. It can be done be-
tween one month to two years with excellent result. The success rate falls with increasing
age. It is less effective after three years. In this procedure a fine specially designed goniotome
is introduced in the anterior chamber via a peripheral corneal stab incision under operating
gonioscope and operating microscope. The superficial layers of one-fourth circumference of
trabecular meshwork are cut just below the Schwalbes line. Some eyes may require repeat
surgery.
Prior to surgery, intraocular pressure is lowered to a safe limit by oral acetazolamide 15
mg/kg in divided doses. The pupil is constricted by local pilocarpine. It is a very safe procedure
in competent hands, some surgeons prefer to do bilateral surgery. It is claimed that only com-
plication during goniotomy is anaesthesia induced. This type of goniotomy is not possible in
cloudy cornea.
(ii) Direct goniotomy. This is a less accurate method of opening the trabecular
meshwork. This is done in presence of cloudy cornea. The goniotomy knife is passed almost
blindly across the anterior chamber to reach the trabecular meshwork on the opposite side.
The superficial meshwork is cut as in classical goniotomy. This method is less effective and
produces more complications.
(b) (i) External trabeculotomy is a procedure where the conjunctiva is incised and
reflected to expose the sclera near the limbus. A vertical scletotomy is done to reach the canal
of Schlemm. The canal is divided, the two cut ends are identified. A specially designed
trabeculotome is passed in the canal. Once whole length of the trabeculotome has gone inside,
the trabeculotome is swept in the anterior chamber ripping open a passage in the trabecular
meshwork. The procedure is less effective than goniotomy. It is not suitable for advanced case
where due to altered surgical anatomy of the limbus, it is difficult to identify the canal of
Schlemm.
(ii) Trabeculectomy. Trabeculectomy is more successful in moderately advanced cases
does not require special device like goniotome, operating gonioscope or trabeculotome.
The difficulty arises due to changed anatomy of the surgical limbus. A sclerotomy wound
may be inadvertently placed too posteriorly, resulting in injury to the ciliary body, loss of
vitreous, the lens may be injured during the procedure. The advantage of the method is that it
can be done in presence of hazy cornea.
Thermal sclerotomy, iris inclusion surgery, corneo scleral trephine, cyclodialysis which
were done in past have been given up in favour of trabeculectomy. trabeculectomy with use of
anti metabolities is said to give better results.
43
C. Artificial drainage device
44
or aqueous drainage device (A.D.D.)
44
. These are
miniature artificial drainage devices that
45
are transplanted under the Tenons capsule to
bypass the trabecular meshwork and canal of Schlemm. They are generally used in ad-
vanced glaucoma or where previous surgeries have failed. There are three types of glaucoma
devices, the first two are of historical importance and are no more in use the devices are :
(a) Seton, (b) Shunts, (c) Valves.
(a) Seton. This is a solid rod. One end of which is introduced in the AC and the other
end is anchored under the subconjunctival tissue. The aqueous drains by the side of rod by
capillary action. The drainage is uncontrolled.
(b) Shunt. This is a tubular structure one end of which rests in the anterior chamber.
The fluid passes through the lumen passively. This is also uncontrolled.
(c) The valves. These are tubular structures similar to shunts, have one-way flow from
anterior chamber to sub-Tenons space. The flow is regulated, once the tension falls below a
critical level the flow stops.
These devices are seldom used as primary procedure. They are used only in eyes where
other surgical procedures have failed.
These devices are associated with high risk of intraoperative and post operative compli-
cations.
Common complications of these devices are :
Perforation of thin sclera.
Injury to the ciliary body
Vitreous loss
Endothelial damage during insertion
Intra operative hyphaema
Expulsive haemorrhages
Persistent shallow AC
Corneal decompensation
Tube touching the iris
Moderate to severe uveitis
Closure of the lumen
Extrusion of the device
Laser in buphthalmos. Q switched Nd-YAG has been used to produce laser goniotomy,
otherwise laser is mostly used as cyclo destructive procedure. Either transscleral or through
pupil
46
. Transscleral Diode or Nd-YAG is used to ablate ciliary body as cyclo destructive proce-
dure. Argon or Nd YAG is also used to open the clogged end of aqueous drainage device. Laser
is also used to make extra iridotomies if need arises.
(a). Medical treatment. Medical treatment have limited role in management of pri-
mary buphthalmos. Miotics, the drug used widely in adult wide angle glaucoma is only used to
constrict the pupil before glaucoma surgery or iridotomy. It is claimed that pilocarpine may
cause paradoxical rise of tension due to collapse of trabecular meshwork because of high
attachment of uvea in posterior meshwork
42
. Beta blockers, alpha agonist should be used in
consultation with paediatrician. Acetazolamide and IV Mannitol is seldom required.
(b). Ancilliary treatment. This is divided into two main parts :
1. Correction of existing error of refraction.
2. Utilising salvageable vision by low vision aids.
1. The buphthalmic eyes are generally myopic with astigmatism. There may be a change
in refractive power following surgery, mostly astigmatism. In uniocular cases amblyopia sets
in early and if not managed becomes permanent, vision with best corrected glasses is rarely
normal due to corneal opacity, irregular astigmatism, optic nerve changes, residual glaucoma
and amblyopia.
Complication in buphthalmic eyes :
A. First and foremost complication is missed diagnosis. Either the glaucoma is mistaken
as simple epiphora or conjunctivitis or these conditions are mixed up as glaucoma and
the child exposed to unnecessary investigation. The later is a better option. In all cases
of buphthalmos occult retinoblastoma should be suspected and excluded. The child
must be examined with an indirect ophthalmoscope and if needed an ultrasound may be
ordered.
B. Amblyopia squint and nystagmus are common both in treated as well as untreated eyes.
C. Persistent lacrimation and photophobia may be seen even when tension has been brought
under control.
D. Large equatorial staphylomas are seen in untreated or poorly managed cases. The thin
sclera is prone for rupture that may lead to bleeding, warranting removal of eye.
E. Subluxation and dislocation of clear or cataracts lens are occasionally seen.
F. The buphthalmic eyes generally have high axial myopia hence they are prone for retinal
detachment.
Associated and secondary congenital glaucomas
32
. A heterogeneous group of con-
genital anomalies of eye with or without systemic manifestation produce glaucoma in chil-
dren.
They are :
A. Associated causes of congenital glaucoma. In contrast to primary congenital
glaucoma where the pathology lies in the trabecular meshwork with normal cornea and iris.
The associate glaucomas may or may not have abnormality in the trabecular meshwork.
B. The other group consists of few primary conditions that cause secondary glaucoma.
These are :
1. Congenital uveitis
2. Congenital keratitis
3. Intrauterine trauma
4. Neoplasm
A. The associated causes of congenital glaucoma is long. Some of the causes are :
1. Iridocorneal dysgenesis (see page 337)
2. Mesodermal dysgenesis (see page 338)
3. Congenital anomalies of uvea :
(a) Aniridia
(b) Congenital ectropion of uvea
4. Congenital anomalies of globe
(a) Microphthalmos
(b) Nanophthalmos
5. Congenital anomalies of posterior segment
(a) Persistent primary hyperplastic vitreous
(b) Retrolental fibroplasia
6. Phacomatosis
(a) Struge Weber syndrome
(b) Neurofibromatosis
7. Miscellaneous
(a) Lowes syndrome
(b) Nevus of ota
(c) Mucopolysaccharidosis
Most of the common conditions have been discussed in various chapters except congeni-
tal ectropion of uvea, glaucoma in Lowes syndrome and nevus of ota.
Congenital ectropion of uvea
47
. This is non progrgessive, unilateral condition.
The pupil is circular, react well to light and accommodation. In normal eyes there is no pig-
ment in the stroma, in this condition iris pigments are present in anterior stroma. Glau-
coma is caused due to maldevelopment of angle of anterior chamber. Glaucoma develops in
childhood or during puberty. The condition may be associated with neurofibromatosis.
Glaucoma in Lowes syndrome. Lowes syndrome is a systemic disorder that involves
not only eyes but multiple organs. The ocular manifestation are bilateral congenital cataract,
glaucoma, iris atrophy, miosis, microphthalmos. Glaucoma is bilateral in one third of cases.
Female carriers have cortical lenticular opacity. Management of glaucoma is difficult, goniotomy
may help. Cataract and glaucoma are independent of each other. The systemic involvement
consists of mental retardation, renal ricket, aminoaciduria and hypotony
48
.
Nevus of ota. In this condition, there is deposition of melanocytes in the skin, and
conjunctiva. This is generally an isolated congenital anomaly or may be associated with nevus
of skin on the distribution of second and third division of the trigeminal. Besides melanosis
bulbae a small percent of cases develop glaucoma on the side of malanosis. The pigments
obscure scleral spur and ciliary band, the trabeculum may be fragmented.
46
Glaucoma in older children. Glaucoma in children fall in following broad groups :
A. True congenital glaucoma, diagnosed between birth and first three months.
B. Infentile glaucoma
1
is between three months to three years.
C. Juvenile glaucoma
1
seen over three years of age.
Juvenile glaucomas form a large group of conditions of diverse cause that results in rise
of intraocular tension. These are either :
1. Late manifestation of congenital glaucoma
2. Early onset of adult chronic simple glaucoma.
3. Secondary glaucoma
(a) Wide angle
(b) Narrow angle
Common causes of junevile glaucoma are :
1. Associated congenital glaucoma
29,30
, may be hereditary or non-hereditary.
2. Trauma
3. Uveitis
4. Steroid induced
5. Post surgical status
6. Phacogenic
7. Tumour related
8. Other less common glaucomas seen in children are :
(i) Neovascular glaucoma
(ii) Glaucomatocyclitis crisis
(iii) Epidemic dropsy
From the above list of causes it is obvious that except juvenile open angle glau-
coma, all other glaucomas are secondary to local or systemic causes. Most of them are wide
angle. Presenting signs, symptoms and management of these glaucomas are identical to adult
glaucoma.
Traumatic glaucoma in children
49
All kinds of trauma mechanical, physical, chemical, radiation can result in raised
intraocular tension in all ages. As trauma is more common in male children, incidence of trau-
matic glaucoma is more common in boys. Most of the eyes have a initial fall of intraocular
pressure following injury, this is most marked following penetrating injury due to obvious
reason that the wound acts as a large channel for aqueous to drain. In contusion the hypo-
tension is due to reduced ciliary secretion, which builds up later to reach normal level and
may remain so, in some cases there is a delayed rise that may take months or year to manifest.
Immediate rise of tension is seen due to large hyphaema, swollen lens, cortical material
filling the anterior chamber. In chemical burns the immediate rise of tension is due to shrink-
age of outer coat of the eye and release of prostaglandin
50
.
Post traumatic glaucoma may sometimes be traced to earlier surgical intervention like
aphakia, pseudophakia, penetrating keratoplasty, vitrectomy, retinal surgery, laser
capsulotomy and laser photo-coagulation.
Post traumatic glaucoma can be divided in following groups :
(a) Open globe injury
(i) Accidental
(ii) Surgical
(b) Closed globe injury
Contusion and concussion
(c) Chemical injuries
Glaucoma in non surgical penetrating injury. Immediately after penetration of
the globe, the eye goes soft due to the open wound draining constantly at a very high rate. If
the wound does not close, the eyes goes into a state of perpetual hypotony, phthisis and blind-
ness. If the wound is repaired surgically or heals by natural process, the intraocular pressure
builds up. If there is not much of tissue disruption, the intraocular tension remains within
normal range, only on rare occasions the tension may rise after months or years.
The tissue disruption following penetrating injury and subsequent repair may lead to
secondary angle closure, formation of peripheral anterior synechia, pseudo-angle-
formation, fibrosis of trabecular meshwork and obliteration of canal of Schlemm.
There may be pupillary block due to swollen lens, after cataract, occlusio pupillae,
seclusio pupillae, iris bombe, cyclitic membrane. The vitreous may finds its way in anterior
chamber or block of angle. Angle closure may worsen pupillary block and vice versa. Associ-
ated infection, inflammation may also worsen the condition. An associated retained intraocular
foreign body may contribute to rise of tension separately or with uveitis.
Management of glaucoma in penetrating injury. Best management of glaucoma
in penetrating injury is to minimise tissue disruption rectify, tissue disruption that is already
present, minimise uveitis by use of local and systemic steroids, antibiotics and cycloplegics.
There are two methods of repairing the penetration wound :
1. Repairing of the corneal, scleral and corenoscleral wound with removal of incarcer-
ated uvea, lens and vitreous. A non incarcerated lens may too have to be removed by
lensectomy along with anterior vitrectomy. Any retained intraocular foreign body is
also removed while doing extensive vitrectomy. The next important step is to reform
the anterior chamber, this prevents closure of trabecular meshwork.
2. Primary repair of cornea, scleral wound and excision of incarcerated uvea. This is
followed by well planned extensive surgery that include lensectomy, various grades
of vitrectomies, removal of foreign body, scleral buckling and reformation of anterior
chamber with or without intraocular implant after the primary wound has healed
well.
Once the glaucoma sets-in, inspite of best surgical management of penetrating injury,
the eye may be put on medical therapy of glaucoma by way of local beta blockers, alpha agonist,
carbonic anhydrase inhibitors or systemic carbonic anhydrous inhibitors for short period. Uveitis
is treated by steroids and cycloplegic. Miotics and latanoprost are contra indicated. If medi-
cal treatment fails or the child does not comply with the prescribed regime, best alternative is
to undertake any of standard anti-glaucoma surgeries. Laser may be used to do an iridotomy
or iridoplasty. Laser trabeculoplasty generally fails. In some cases an aqueous drainage
device may be the last alternative.
Glaucoma following ocular surgery. Both types of surgical wounds i.e. open globe
and close globe wounds may lead to secondary glaucoma. Common intraocular surgeries that
are associated with glaucoma are :
Needling, aspiration, lensectomy in congenital and traumatic cataracts.
Pupillary block in aphakia and pseudoaphakia
Repair of penetrating injury
Vitrectomy
Penetrating keratoplasty
Closed globe surgeries that cause post operative rise of tension are :
Scleral bucking
NdYAG posterior capsulotomy
Glaucoma following lens surgery. From the conditions mentioned above it is clear
that surgery on lens is a common cause of post operative glaucoma. It can be seen in all ages
but more common in children, even a surgery of short duration and minimal handling like
needling, aspiration is fraught with rise of tension
51
that may be seen between 6% and 24%
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 combi-
nation of more than one factor.
1. Distortion of angle of anterior chamber.
2. Formation of peripheral anterior synecha.
3. Effect of visco-elastic.
4. Pupillary blockVitreous in the pupillary area, cortical material in pupil.
5. Angle closure
6. Uveitis glaucoma haemorrhage syndrome
7. Pigment dispersal syndrome.
There is initial fall of tension following incision that remains low for first twenty
four to forty eight hours and then builds up to normal range and is maintained with in
physiological limits. Occasionally there is acute rise of tension following lens surgery, this is
due to relatively small eye
53
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.
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 progno-
sis.
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.
Uveitis glaucoma hyphaema (UGH). This condition was very common in era of ante-
rior chamber IOL. The AC IOL caused, repeated trauma to the iris that resulted in acute non
granulomatous uveitis after months of surgery. It presented as haemorrhagic uveitis with rise
of tension. Many factors like 10L itself, ischamia of iris, pressure necrosis of iris, chaffing of
iris trigger onset of uveitis. P.C. 10L can also cause UGH if the edge of the lens presses against
the ciliary body.
Management consists of active and prolonged treatment of uveitis, hyphaema does not
require specific treatment. Glaucoma is initially treated by medical methods, if it fails surgery
may have to be restored to.
Glaucoma following vitrectomy. Fortunately few children require vitrectomy. The
conditions that need vitrectomy in children are mostly traumatic with or without intraocular
lens implant, lost PC IOL and vitreous haemorrhage. It is thought that post vitrectomy glau-
coma is seen in about one fifth of the cases. Post vitrectomy rise of intraocular tension is more
common when long acting gases are used as temponade. Early rise is caused by expansion of
the gases. Silicone oil also causes rise of intraocular tension. Neovascularisation following
vitrectomy is another cause of post vitrectomy glaucoma. Management is similar to any open
globe post traumatic glaucoma.
Glaucoma following penetrating keratoplasty
56
. Post penetrating keratoplasty glau-
coma is more frequent than diagnosed. Glaucoma following P.K. is a major cause of graft
failure. It is more common if PK is associated with lens extraction. Intra capsular lens extrac-
tion with penetrating keratoplasty is more formidable combination in producing glaucoma
than extra capsular lens extraction. Incidence of glaucoma increases with each repeat surgery.
Tight sutures, collapse of trabecular meshwork, small trephine size, donor larger than recipi-
ent are probable causes of glaucoma.
Malignant glaucoma (ciliary block glaucoma, aqueous misdirection glau-
coma
57,58
. Malignant glaucoma is a rare but most difficult of secondary glaucoma that has
both pupillary block and angle closure. Classically this form of glaucoma results in uniform
shallowing of both central and peripheral anterior chamber, small pupil, very high intraocular
tension that becomes worse with miotics but may be relieved by cycloplegic. Though spontane-
ous cases without any provocation have been reported. Commonest situation where it is seen
is following intraocular surgery. Paradoxically antiglaucoma filtering surgery heads the list of
surgical procedures that cause malignant glaucoma. They are :
1. Glaucoma surgeries. Filtering and fistulous procedures, iridectomy, both types of
iridotomies i.e. surgical or laser.
2. Lens surgeries. Needling, aspiration, extra-capsular cataract extraction,
intracapsular lens extraction with any type of IOL or without IOL.
3. Repair of penetrating corneal or corneoscleral injuries.
The condition is most commonly seen in incisional procedures following narrow angle
glaucoma. Malignant glaucoma is not related to any specific type of glaucoma surgery. Pre
operative intraocular tension also does not influence its clinical presentation.
Malignant glaucoma is more common is phakic eyes than in aphakic eyes. Rarely it can
be bilateral. There are two mechanisms that work in combination, they are :
1. Ciliary block
57
. The normal flow of aqueous is from posterior chamber to anterior
chamber via pupil and from anterior chamber to canal of Schlemm through trabecular
meshwork. Hardly any aqueous percolates to the vitreous. In case of pupillary block, flow of
aqueous from posterior chamber to anterior chamber is impeded but aqueous does not find its
way into the vitreous. In case of malignant glaucoma there is severe spasm of ciliary body, the
ciliary body moves centrally towards the lens. In this process the ciliary body may come in
actual contact with peripheral part of lens when it is present. In case of intracapsular lens
extraction the ciliary body may throttle the vitreous cone projecting in the pupillary area. In
phakic eyes the iris lens diaphragm moves forwards shallowing the AC and obliterating the
angle. Here the second mechanism takes over i.e. misdirection of aqueous flow.
2. Aqueous misdirection
55
. The aqueous instead of finding its usual anterior path via
pupil, drains into the vitreous or behind the vitreous. Retrovitreal accumulation is assisted by
posterior vitreous detachment. The ever increasing aqueous pushes the vitreous body for-
ward, which in turn pushes the iris lens diaphragm forward. This along with ciliolenticular
block worsens the flow of aqueous via conventional anterior route causing rise of tension.
Diagnosis. The condition is diagnosed by severe post operative pain and unexplained
diminished vision. On examination the cornea is edematous, the anterior chamber is very
shallow, the pupil is constricted and tension is very high. Gonioscopy when possible shows
almost obliterated angle. Ocular ultrasonography reveals ciliolenticular block and aqueous in
or behind the vitreous.
Treatment. Treatment consists of
59, 60
1. Lowering of tension by
A. reducing formation of aqueous by carbonic anhydrase inhibitors orally and local
instillation of beta blockers or alpha agonists.
B. Reducing volume of vitreous by IV 20% mannitol, oral glycerine.
2. Relieving pupillary block :
A. Use of strong cycloplegic i.e. atropine sulphate 1% drops two times a day. If nec-
essary 5% to 10% phenylepherine or 1% tropicamide every five minutes for five
times may be supplemented with atropine Pilocarpine is contra indicated. It
worsens the ciliary and pupillary block.
B. If medical treatment fails, surgical intervention is required that may be surgical
iridectomy, laser iridotomy, rupture of anterior vitreous faces by laser or me-
chanically in aphakic eyes. In phakic eyes a subtotal vitrectomy may be required.
Sometimes aspiration of aqueous entrapped in vitreous may also give relief.
Closed globe surgical procedures that cause glaucoma
60
:
Glaucoma following scleral buckling. Glaucoma following scleral buckling has two
phases. The first is intra operative, where indentation of the sclera and tightening of the scleral
buckle causes sudden rise of intraocular tension. The raised tension may cause central retinal
artery obstruction and if not relieved may cause permanent damage. It is mandatory that all
retinal surgeries be done with indirect ophthalmoscope and while tying the buckle, the pulsa-
tion in the central artery of retina be monitored. Absence of pulsation requires immediate
lowering of tension either by loosening the sutures or intra venous injection of a bollus of
acetazolamide or mannitol.
The second phase is late and chronic rise of tension that develops after six to seven days
and may go unnoticed. The tension is rarely very high. These eyes generally have low scleral
rigidity so recording of tension by indentation tonometer gives a false low tension.
Paradoxically some of the eyes that have undergone scleral buckling may develop
hypotony that may last for years.
Glaucoma following Nd-YAG posterior capsulotomy, laser trabeculoplasty and
iridotomy :
All these conditions have two types of pressure elevation. The first - that is seen within
one hour following the procedure. This is seen in twenty five percent of all eyes, is transient
and controlled by local beta blockers two times a day for two to three days. Possibility of this
type of rise of tension should always be remembered in paediatrics patients who have cent
percent chance of developing PCO following extra capsular cataract extraction with or without
IOL. The acute rise of tension is most probably due to lens particles blocking the trabecular
meshwork. The second type of glaucoma is late onset of glaucoma in some patients only. The
cause is not well understood.
Uveitis as Cause of Glaucoma in Children
Glaucoma secondary to anterior uveitis is one of the major causes of childhood blindness.
60
Its presentation is so varied that a high degree of suspicion is required to diagnose the condition
specially in children where an immature eye is liable to suffer visual loss if glaucoma is not
treated well. The condition is over shadowed by the overwhelming signs of anterior
uveitis.
The pitfall can be eliminated if tension is monitored on every visit. Intraocular ten-
sion under local anaesthesia can be recorded in children above five years with little persuasion
and reassurance. Similarly recording of vision with P.H. retinoscopy and fundus exami-
nation should also be done more frequently. It is better to do gonioscopy examination every
three months. Pressure changes in uveitis swing between hypotony to moderate elevation.
Generally tension in anterior uveitis is either normal or low to begin with, both the conditions
can culminate in glaucoma over a period of time
61
.
Initial low tension in anterior uveitis is caused due to ciliary shut down, which results
in hypo secretion of aqueous in presence of normal resistance to outflow. However persist-
ent low tension is an important cause of formation of peripheral anterior synechea that causes
increased resistance to aqueous outflow. This leads to gradual rise of IOP which reaches nor-
mal range or may overshoot to cause glaucoma.
Rise of intraocular tension can be acute, intermediate or delayed
63,64
. Basically there
are two types of secondary post inflammatory glaucoma :
(a) Open angle glaucoma and
(b) closed angle glaucoma
65
. The latter can either be with pupillary block or without
pupillary block. Incidence of open angle secondary glaucoma is more common than
closed angle. Exact mechanism of raised intraocular tension is not well understood. It is
multifactorial that may work separately or in combination.
Probable causes of open angle glaucoma in uveitis
66
(i) Spread of inflammation into trabeculum leading to trabeculitis. Edema of
trabeculum results in narrowing of trabecular meshwork that impairs outflow of
aqueous.
(ii) This is worsened by increased viscosity of plasmoid aqueous which finds it diffi-
cult to pass through narrow meshwork.
(iii) The cellular debris and iris pigments may plug the pores of the meshwork.
(iv) Prolonged use of steroid in a steroid responder causes rise of tension.
(v) Release of prostaglandin also plays an important role in causing glaucoma.
(vi) On long run sclerosis and scarring of trabecular meshwork may be the cause of rise
in intraocular tension.
Probable cause of angle closure glaucoma in uveitis :
(i) Closed angle glaucoma with pupillary block - Extensive posterior synechiae
leads to formation of ring synechiae which prevents aqueous to escape through the
pupil into the anterior chamber. This pushes the iris forward in the form of iris
bombe narrowing the peripheral part of anterior chamber and formation of pe-
ripheral anterior synechiae. However 360 of posterior synechiae without iris
bombe can also cause rise of tension. This is worsened if the pupil is small and cov-
ered by exudate. Thickened iris, miosis and plasmoid aqueous also raise tension, so
does release of prostaglandin.
(ii) Closed angle glaucoma without pupillary block. This is mostly due to deposi-
tion of inflammatory debris on the trabecular meshwork, subsequent contracture of
iris over the trabecular meshwork. A paradoxical situation arises when dilation of
pupil causes embarrassment of the angle. This happens if the angle is already nar-
row. Multiple peripheral anterior synechiae may form a pseudo angle proximal to
trabeculum preventing aqueous to reach true angle. Swollen ciliary body may
push the lens iris diaphragm forward, narrowing the angle, resulting rise of IOP.
Diagnosis of glaucoma in anterior uveitis is often missed unless its possibility in every
case is kept in mind. It is a misconception that glaucoma is rare in children in uveitis. It is a
major cause of childhood blindness
62
. In all cases where vision is low and does not improve
with PH, tonometry, retinoscope and ophthalmoscopy should be performed. Gonioscopy helps
to differentiate between an open and closed angle.
Management. In acute stage and to some extent chronic rise of tension can be lowered
by usual treatment of anterior uveitis with cycloplegic and steroids otherwise management
depends whether it is an open angle or close angled glaucoma.
Open angle glaucoma in uveitis is managed by :
Continuing treatment with cycloplegic and steroid/non steroidal anti- inflammatory
drugs.
Treatment of open angle glaucoma by instillation of local, beta-blockers, alpha agonist
or carbonic anhydrase inhibitors. The last is least effective in children, miotics and
prostaglandin analogs are contra indicated.
Systemic use of carbonic anhydrase inhibitors should be employed for short period. Slow
release, CAI are better tolerated by children.
Surgical treatment consists of modified goniotomy
64
, trabeculodialysis and
trabeculectomy. In rare cases aqueous drainage devices may be required.
Closed angle glaucoma with pupillary block is managed by establishing a passage
between the anterior and posterior chamber first therapeutically and if it fails either by laser
or by surgery.
Therapeutic measures used are strong cycloplegic to break the synechiae. Frequent
use of steroid helps in lysis of synechiae. Addition of epinephrine has synergistic action with
cycloplegic that enhances mydriasis. Betablockers and alpha agonists do not have any action
on size of pupil, they are used to lower the intraocular tension. Miotics and latanoprost are
contra indicate. Acute rise is controlled by oral carbonic anhydrase inhibitors.
The argon laser or NdYAG laser iridotomy establishes a permanent passage between
anterior and posterior chamber. Surgical iridectomy is indicated when facility of laser iridotomy
is not available or when such procedure has failed.
Closed angle glaucoma without papillary block is treated medically by betablockers and
alpha agonist, surgical management consists of trabeculactomy.
Steroid Induced Glaucoma in Children
Steroid induced glaucoma is an avoidable iatrogenic glaucoma. It is seen in steroid
responders and those who have family history of chronic open angle glaucoma. It can
occur in any age and sex. However it is more frequent in children
67
. Steroid induced glau-
coma is secondary open angle glaucoma. Its physical findings are similar to COAG. Pres-
sure elevation depends on many factors that include - response to steroids, family history of
chronic open angle glaucoma, strength of drops used, frequency and duration of steroids used.
It is more common in steroids like prednisone, prednisolone, dexamethsone, beta methasone,
and triamsonolone. It is less common in steroids like medrysone and flurometholone
67
. How-
ever none of the steroids are free from intraocular tension raising property. Generally glau-
coma becomes evident after three weeks of instillation of dexa or beta methasone in high
steroids responders. It may take months in low responders with same regime.
All routes of administration of steroids are fraught with glaucoma. Commonest being
ocular use as drops or ointment. This is followed by periocular use. Systemic use of steroids are
more likely to result in glaucoma than skin ointment, inhalers and nasal spray.
On the top of the list of common ocular conditions that culminate in steroid induced
glaucoma in children is spring catarrh where unnecessary, prolonged use of steroids that
use cortisol as foundation are the most offending drugs. This is followed by anterior uveitis,
trauma both accidental or surgical. The surgical condition that need to be monitored for rise of
IOP are : aphakia, pseudoaphakia, glaucoma surgery and keratoplasty.
The exact mechanism of rise of tension following use of steroid is not known. It is said to
be enzyme related.
65
Clinical presentation of steroid induced glaucoma in children is similar to that of chronic
open angle glaucoma. Only difference is that in most of the eyes with steroid induced glaucoma
tension falls within normal limits in two to three weeks after stoppage of drug. Associated field
and fundus changes are permanent, these changes may be confused as due to low tension
glaucoma in later life.
The eye is non congested except in spring catarrh where the eyes remain red due to
existing allergic conjunctivitis. The anterior chamber is of normal depth and the angle is wide
open. In rare instances where steroid induced glaucoma develops before three years of age, the
eye may be buphthalmic.
All children under steroid therapy local or systemic, should be warned about possibility
of glaucoma, these children should get their IOP and vision recorded every three months and
disc evaluated twice a year.
Treatment consists of :
1. Prophylaxis
2. Medical treatment
3. Surgical treatment
1. Prophylaxis :
(a) Awareness among treating ophthalmologic, paramedical personnel, pediatrician,
general physician and dermatologist about possibility of steroid induced glau-
coma in children.
(b) Parents should be warned about possibility of steroid induced glaucoma espe-
cially in spring catarrh. It should be emphasised upon them that, no child
goes blind due to spring catarrh but hundreds of children loose sight due
to steroid.
(c) use of lowest dose of steroid for shortest period that gives the child comfort.
(d) Residual allergy, inflammation should be treated by non steroidal anti- inflam-
matory drugs, local antihistamine and astringents.
2. Medical treatment :
(a) Once it has been established that the child has steroid induced glaucoma, the
drug should be tapered and steroid replaced by non steroidal anti- inflammatory
drugs. This generally brings down the tension to normal range.
(b) If tension remains high in spite of discontinuation of steroid or steroid can not be
discontinued, the tension is brought down by miotics, beta blockers, alpha
agonist, epinephrine and its pro-drug. In case the child was suffering from
uveitis, miotics and latano prost are contra indicated.
3. Surgical treatment :
Only a few children may require surgical intervention. Eyes with steroid induced glau-
coma do not respond well to laser trabeculoplasty. Surgery of choice is trabeculectomy.
Glaucoma following blunt injury can be caused by :
(i) Blood and blood constituents in anterior chamber.
(ii) Recession of angle of anterior chamber.
(iii) Phacogenic
Glaucoma due to blood and blood constituents in the anterior chamber constitute a
group of conditions that are grouped under hemogenic glaucoma
69
that may present as :
Glaucoma secondary to total hyphaema
Hemolytic glaucoma
Ghost cell glaucoma
Out of these, glaucoma secondary to total hyphaema red cell glaucoma
67
is most com-
mon following severe blunt injury. The eyeball is compressed anterio posteriorly pushing the
aqueous laterally. The force of compressed aqueous acts as a shearing force at the angle and
causes damage to the root of the iris, and ciliary body. Normal iris generally does not bleed
between the root and the pupil but may bleed when injured at the root. The ciliary body bleeds
more frequently than iris. The bleeding may occur within few minutes of injury or may be
delayed for three to four days. Bleeding after four days is more severe than initial bleeding.
Rebleeding is frequent.
There is an acute rise of tension as blood fills the whole of the anterior chamber blocking
the trabecular meshwork all around. The blood especially the clotted blood may cause pupillary
block, worsening the condition. The rise of tension varies between moderate to very high.
With sudden rise of tension there is severe ocular and periorbital pain. Pain radiates
on the same side of the head. The eye is generally congested with lacrimation, vision is re-
duced to perception of light or at the most counting finger from close quarter, vision improves
as blood settles down at the lower parts of the anterior chamber. The other cause of dimin-
ished vision and lacrimation is associated corneal haze. The child is generally irritable may
have bouts of vomiting.
The fresh blood in anterior chamber is visible through the transparent cornea. The
parents may be able to say that there is blood in the eye. With passage of time, the blood starts
clotting and its colour becomes darker, ultimately it assumes black and oval shape, such a
condition is called eight ball or black ball hyphaema. There may be re-bleeding, that may be
spontaneous or follow repeat trauma. There may be rebound bleeding following instilla-
tion of epinephrine or its derivatives.
Total hyphaema of more than five days duration and raised intraocular tension is most
likely to develop blood staining of cornea.
Blood staining of cornea is due to infiltration of endothelium and posterior stroma by
blood constituents. As the epithelium is also effected there is associated haze of cornea with
epithelial edema.
Glaucoma secondary to total hyphaema requires early treatment to :
1. Prevent blood staining of cornea, which may last for years causing prolonged dimin-
ished vision that leads to squint and amblyopia.
2. Protect the optic nerve from glaucomatous neuropathy and permanent visual loss.
Management of glaucoma secondary to total hyphaema :
The management consists of :
1. Immediate steps to prevent glaucoma.
2. Management of glaucoma.
Immediate management is essentially treatment of total hyphaema, which consists of :
1. The child is hospitalised to minimise movement.
2. The affected eye is bandaged over a pad. Bilateral patching does not have advantage
over unilateral bandage.
3. The head end is kept raised. This helps the heavy blood particles to settle down at
the bottom of the AC to facilitate opening of trabecular meshwork in the upper part.
4. Instillation of local drugs
71
- Miotics are contra indicated as they may worsen
associated anterior uveitis. Epinephrine is known to cause rebound bleeding hence
it is also not used. cycloplegics give relief to cyclopasm and reduce pain. Role of
local steroid has not been proved beneficial in every case. Beta blockers are help-
ful to keep IOP low hence they may be used not only in proved glaucoma but also as
prophylaxis against rise of tension.
If the child bleeds repeatedly without trauma, the child should be investigated to ex-
clude sickle cell disease or other bleeding disorders.
The child may be put on oral fibrinolytic i.e. a aminocaproic acid in a dose of 50 mg/kg/
day in consultation with paediatrician.
Raised intraocular tension is brought down by :
1. Oral carbonic anhydrase inhibitor in dose of 10mg-20mg/kg/day in divided dose with
potassium supplement.
2. Oral glycerine in dose of 1 to 1.5 cc/kg/day in two divided doses.
3. Intravenous mannitol in dose of 1 gm to 1.5 gm/kg may be given in fast drip. This is
generally done in cases where paracentesis is planned.
If tension does not come down within forty-eight hours with medical treatment, blood is
evacuated either by simple paracentesis or washed with balanced salt solution. To clear
clotted blood two stab incisions are given, one at temporal side of the cornea and the other
opposite to it. Through the first viscoelastic is introduced which forces the clot out through the
other hole.
75
Visco-elastic itself is then washed with BSS and anterior chamber reformed, the
eye is observed for rebleeding and rise of tension. Once the acute episode has passed, the eye is
kept under watch for delayed rise of tension over years which is generally due to recession of
angle of anterior chamber. (see page 265).
(b) Hemolytic glaucoma
69,73
. This secondary wide-angle glaucoma is less common
than glaucoma associated with hyphaema. It may be an early or late feature in blunt injury. In
hemolytic glaucoma the break down products of blood in anterior chamber i.e. red blood cells
or hemosiderine are phagocytosed by large macrophages that plug the trabecular
meshwork and cause rise of intraocular pressure in milder form, the condition is self limit-
ing. However it is better to keep the tension within normal range in all cases of hyphaema by
betablockers and alpha agonist. Miotics epinephrine and latanoprost are contra indicated.
Some clinicians feel that hemolytic glaucoma is a milder form of ghost cell glaucoma.
Ghost cell glaucoma
69, 70, 73
. The secondary open angle glaucoma was previously known
as erytholastic glaucoma.
75
It is mostly seen in vitreous haemorrhage with broken ante-
rior vitreous face. It is also met within total hyphaema especially in eight ball hyphaema. In
adults it is mostly seen following lens extraction and pre-existing vitreous haemorrhages. If
the vitreous face gives away, the blood finds its way to anterior chamber to cause ghost cell
glaucoma.
In children traumatic vitreous haemorrhage with subluxated or dislocated lens can cause
ghost cell glaucoma.
The mechanism of glaucoma is multifactorial. The RBCs which are circular, biconvex,
and pliable can pass through trabecular meshwork without any obstruction. When these are
converted into larger, spherical, rigid bodies in the vitreous, these cells contain denatured
haemoglobin called Heinzbodies
76
and are called ghost cells.
The ghost cells due to rigid walls and spherical shape, fail to pass through the pores of
the trabeculum causing rise of intraocular tension. These cells may collect at the bottom of the
anterior chamber with a soft-white or khaki coloured pseudo hypopyon. It takes red cells
ten to fifteen days to be converted into ghost cells in the vitreous.
Rise of intraocular tension that is mild to moderately is directly proportionate to number
of ghost cells present, with too many ghost cells, the IOP may be raised sufficiently to cause
pain, photophobia and diminished vision due to corneal edema.
Management consist of lowering of intraocular pressure by standard anti-glaucoma
drugs and keep it within normal range with minimum drug. If medical treatment fails, stand-
ard surgical procedure may be required. Miotics epinephrine and latanprost are contra indi-
cated.
73
Glaucoma secondary to angle recession
71, 77, 80
. Angle recession glaucoma may de-
velop months or years after blunt injury. Many a times the patient may even forget about
receiving blunt injury in childhood or may think such trivial incident not worth mentioning.
This makes enquiry about blunt trauma mandatory in all cases of uniocular wide angle glau-
coma.
Angle recession glaucoma is generally uniocular. Being traumatic it is more common
in boys. In the beginning, it may be masked by hyphaema and hemogenic of glaucoma.
Initial hypotony, which is very common, does not exclude possibility of angle recession glau-
coma in future. About ninety percent of eyes with blunt injury have some degree of angle
recession, only ten percent of eyes with angle recession develop glaucoma. Recession may
involve only a small sector or may be extensive. Recession less than 180 is less liable to de-
velop glaucoma. Recession more than 270 is most likely to develop glaucoma.
The recession of angle which is in fact a laceration on the face of ciliary body that
extends between the longitudinal and circular muscle develops immediately following blunt
injury. The recession may initially be missed due to corneal changes and associated hyphaema
or when not suspected. Hence all eyes with blunt injury should undergo gonioscopic exami-
nation after six weeks. Bilateral gonioscopy facilitates comparison between normal and in-
jured eye.
The parents of injured children should be told about possibility of glaucoma in later life
and instructed to get the childs eye examined for vision, rise of tension, changes in angle and
disc every six months and then yearly.
It has been widely noticed that angle recession glaucoma is more common in persons
who have positive history of wide angle glaucoma in the family. The child may develop chronic
simple glaucoma in the uninjured eye later.
The exact mechanism of development of glaucoma in angle recession is not well under-
stood. One point is established that angle recession perse does not cause glaucoma. It is the
effect of trauma on the trabeculum that is the prime suspect in producing glaucoma. A mem-
brane like structure covers the trabecular meshwork facing the pupil, which is actually prolif-
eration of Descemets membrane.
The force may extend in the trabecular meshwork and disrupt it that heals by scarring.
The force may rip off the ciliary body from the scleral spur and cause cyclodialysis.
Iridodialysis is also very common.
Diagnosis is confirmed by gonioscopy. Bilateral gonioscopy by Koeppe lens helps to
compare the findings of injured eye with the normal eye, slightest deepening of AC on the
injured side is always suspicious finding.
Frank tremulouness of iris with deep chamber denotes subluxation of lens. Common
findings include mild to moderate mydriasis, widening of ciliary band. The width of cili-
ary band is irregular, whitening of scleral spur and the trabecular meshwork. The trabeculum
may be sprinkled with iris pigment giving a false impression of pigmentary glaucoma. The
angle is wide.
Treatment of angle recession glaucoma is similar to any secondary open angle glau-
coma, which consists of local instillation of betablockers or alpha agonist. Pilocarpine is not
favoured because it is presumed that in a traumatised eye with damage to the trabecular
meshwork, the outflow is diverted to uveo-scleral outflow that is impaired by miotic
79
hence
latanoprost may be more effective than miotic. Sustained released carbonic-anhydrase in-
hibitors may be given orally for short term, local carbonic anhydrase do not have much effect
in children.
When glaucoma can not be brought under control, surgery may have to be employed.
Argon laser trabeculoplasty does not give uniform result
78
. Conventional trabeculectomy with
mitomycine-C or 5 fluro uracit gives better results
77
.
Traumatic phacogenic glaucoma
Following blunt injury, the lens may be dislocated or subluxated. Blunt injury rarely
cause fast opaification of lens which is very common in penetrating injury. Hence blunt trauma
rarely causes phacogenic glaucoma except when either the dislocated lens has come in AC or is
trapped in pupil. Rarely the lens may become intumescent and cause both pupillary and angle
closure glaucoma.
Glaucoma in post surgical status (page 355)
Non traumatic Phacogenic glaucoma in children
81, 82
. Lens may cause glaucoma
by any of the following causes separately or in various combinations. The causes can be
congenital manifesting in childhood or acquired. Opaque lens is more likely to cause glaucoma
than transparent.
(a) Phaco-morphic
(i) Sherophakia
(ii) Microphakia
(iii) Tumescent
(b) Phacotopic
(i) Congenital ectopic
(ii) Acquired Subluxated
Dislocated
Incarcerated in the pupil.
(c) Phacolytic
(i) Cortical matter
(ii) Due to leak of protein
(iii) Phacoanaphylactic
Phaco morphic glaucoma. This type of glaucoma is caused by a small relatively spheri-
cal lens that closes the pupil like a ball and socket. The pupil may either be normal or miotic.
Small pupil is more likely to cause pupillary block glaucoma. This type of glaucoma is relieved
by mydriasis and is called glaucoma inversus or paradoxical glaucoma.
Intumescent lens. Intumescent lens as cause of glaucoma is mostly seen in age
related cataract where a lens swells to cause pupillary block and secondary angle closure. The
same effect is brought about in children when aqueous finds its way through a microscopic
wound mostly in blunt trauma and causes the lens to swell. The swollen lens causes pupillary
block and secondary angle closure.
Management consists of lowering of tension by oral acetazolamide, local beta blockers.
Local steroids may be helpful to supress associated uveitis. Once tension has been brought to
safe limit an ECCE with PCIOL is the best available treatment.
Cortical matter glaucoma. This is a step ahead of intumescent of lens. Here the
capsule is ruptured either accidentally as following penetrating injury or has been surgically
cut to perform ECCE. The condition is very common following needling in children either for
congenital or traumatic cataract. The anterior chamber is filled with soft cortical matter that
blocks both pupil and the angle. Management consists of removal of as mush of cortical mat-
ter as possible.
Microscopic leak of denatured lens protein, which does not cause antigen antibody reac-
tion is ingested by macrophages that swell up and block the trabecular meshwork leading to
secondary open angle glaucoma.
Management consists of lowering IOP by any standard method followed by removal of
lens.
Phacoanaphylatic glaucomaIn contrast to above, in rare instances, the lens pro-
tein acts as an antigen and causes severe local reaction in the form of anterior uveitis and is
associated with glaucoma. This is rare in children.
Management consists of control of uveitis, lowering of intraocular tension to a safe limit
and removal of lens.
Chemical injuries as cause of glaucoma
83
. Both types of chemicals i.e. alkali or
acids cause initial rise of tension for first few hours to few days, then it comes to normal. This
initial rise of tension is attributed to shrinkage of sclera and cornea and release of
prostaglandin.
80
This phase is followed by return to normal tension. The tension may be
established within normal range and remain so but in many eyes there is rise of tension due to
continued action of prostaglandin, uveitis and tissue disruption brought about by action of the
chemicals.
Management consists of immediate management of chemical burn by standard method,
administration of carbonic anhydrase inhibitors for short period. Local betablockers and alpha
agonist. Miotics are contra indicated. Frequent instillation of steroids reduces uveitis, if ten-
sion is not controlled a fistulising surgery with anti metabolites may be required. In some
cases glaucoma implants may be the last resort.
Tumour related glaucoma
84, 85, 86
. All types of intraocular tumours, benign or malig-
nant cause glaucoma. In adults common tumours producing glaucoma are related to uvea,
followed by intruocular metastases. In children the common tumours causing glaucoma are
retinoblastoma
81,82
, diktyomo, juvenile xanthogranuloma, leukaemia. Benign hamartomas that
produce glaucoma are von Recklinghausens syndrome and Sturge Weber syndrome. Glau-
coma by Sturge Weber syndrome inflicts children while that due to Von Recklinghausens
syndrome causes glaucoma in young adults.
Tumour related glaucoma are generally unilateral. They can cause both open angle as
well as narrow angle glaucoma with or without pupillary block.
Severity of glaucoma depends on size, location and duration of the growth.
Common causes of tumour related glaucoma are
86
:
(a) Direct infiltration of the angle by tumour.
(b) The eyes have associated congenital anomalies (phacomatosis).
(c) Angle may be blocked by tumour cells present in anterior chamber as pseudohypopyon
(Retinoblastoma).
(d) The iris lens diaphragm may be pushed by a large tumour mass.
(e) Neovascularisation of angle (Retinoblastoma).
(f ) Raised episcleral pressure.
All eyes with unexplained unilateral rise of intraocular tension should be examined
well, to exclude possibility of intraocular tumour by, indirect ophthalmoscope, gonioscope and
ultrasonography.
Management of tumour related glaucoma is essentially treatment of primary cause spe-
cially if it is life threatening like retinoblastoma or leukaemia.
Neovascular glaucoma. Neovascular glaucoma in children is far less common than
neovascular glaucoma in adults. Diabetic retinopathy and central vein thrombosis are two
causes that top the long list of causes in adults.
87, 88
These conditions are not seen in children.
Common causes of neovascularisation glaucoma in children are retinoblastoma, Coats
disease, retrolental fibroplasis. Neovascular glaucoma can be either open angle or closed
angle secondary glaucoma.
Management of neovascular glaucoma in children is as difficult as in adult and does not
differ much. Miotics are contra indicated. Tension should be brought down by betablockers or
alpha agonist. Local steroids and cycloplegics help to reduce associated inflammation.
Filtering surgeries are not very effective. Best results are cyclodestructive procedures
i.e. transcleral cyclocryo, and Nd-YAG cyclophto coagulation.
Glaucomcyclitis crisis. This is rarely seen in children. It may present as unilateral
periodic rise of tension in late teens with signs of cyclitis. The affected pupil is larger than the
other. There are signs of cyclitis. The rise of tension is periodic. Each episode may last from
few hours to few weeks. During crisis the tension may reach between 40 mm to 60 mm coming
down even without treatment
89
. Persistent raised tension is controlled by betablockers. Asso-
ciated uveitis is treated by local steroids and rarely by mydriatic. Surgery is contra indicated.
EPIDEMIC DROPSY GLAUCOMA
90, 91, 92, 93
Epidemic dropsy is an acute toxic systemic condition with multi-systemic involve-
ment seen as epidemics in certain areas of the world. The endemic countries are mostly lo-
cated in South East Asia, Fiji and South Africa. Worst effected country is India, specially
eastern and northeastern states where mustard oil is used as cooking media or anoint. Epi-
demics have been reported from other parts of India from time to time outside the endemic
state.
Mustard oil itself does not cause the diseases. Epidemic dropsy occurs only when edible
oil even other than mustard oil gets contaminated by oil of argemone mexicana (yellow
prickly poppy) that grows as weed with mustard plants. Mustard oil may be adulterated
intentionally or may be contaminated accidentally
95, 96, 97
.
Argemone mexicana contains sangunarine and dihydro sangunarine that cause rise
in blood pyruvate level which is thought to be the cause of the disease
93
. Ocular symptoms are
late to develop following systemic involvement.
Systemic features are
94, 95
1. Warm erythromatous, tender, hypopigmented skin lesions.
2. Pitting edema of feet and legs.
3. Non specific cardiac failure leading to dyspnea.
4. Diarrhea.
5. Malaise and fever.
In severe cases the condition can be fatal due to cardiac failure.
Ocular signs and symptoms develops six to eight weeks after development of pedal
edema. Glaucoma develops mostly after subsidence of systemic signs and symptoms.
Glaucoma in epidemic dropsy is bilateral, involving both the sexes. No age is im-
mune. Children in first decade may not be affected. It is as common in children in second
decade as in adults.
The glaucoma is wide angle glaucoma, with normal chamber and angle. Outflow
is within normal limits. The aqueous shows raised level of histamine, prostaglandin and
protein suggesting that the glaucoma is hypersecretory. There is no anterior chamber
reaction. The eye is white, cornea may show haze due to rise of intraocular tension that may
be as high as 60 to 80 mm Hg. The patient may only complain of irritation of the eye, col-
oured haloes, round the light which is marked and persistent. The disc and field changes are
similar to those seen in chronic simple glaucoma.
The exact mode of hypersecretion is not known, it is said to be of central origin. Other
ocular findings are toxic vasculitis, venous dilatation and superficial haemorrhages.
In milder form the systemic features subside with discontinuation of adulterated oil from diet.
It is worth noting that epidemic dropsy can be caused by consuming other vegetable oils that
have been adulterated not only by argemone maxicana but also other chemicals like some
mineral oils.
Glaucoma starts late and may linger on for long time. It is partly self limiting. Even
when it subsides with or without treatment it leaves stigma in the form of glaucomatous
disc and corresponding field changes. All patients with juvenile chronic simple glaucoma
should be asked about their food habits specially about cooking medium.
Management. Glaucoma being hypersecretory does not respond to pilocarpine.
Drugs that reduce aqueous formation like epinephrine, its product dipivefrin give better
results. Topical beta-blockers also give favourable results. Systemic acetazolamide is em-
ployed for short term relief. Systemic indomethasin is used as adjunct with topical drops.
It takes few weeks to months to bring the tension to normal range. Once this has been
reached the drugs are gradually withdrawn and maintained on minimum dose. The patients
are followed up twice a year for next two to three years.
If tension can not be brought down, by medical treatment any of the standard filtering
surgeries can be employed.
Juvenile primary open angle glaucoma
98
. Juvenile glaucoma is a non specific term
used to designate a large group of conditions that cause rise of intraocular tension in a patient
after three years of age and before third decade.
Most of these conditions are secondary or associated glaucomas
30, 31, 32
. These may be
wide or narrow angled. In some cases pupillary block may be present. In contrast to above
conditions juvenile primary open glaucoma is a term that designates a specific disorder, which
is similar clinically to adult primary open angle glaucoma with a distinct genetic predisposi-
tion
98
.
Some people consider juvenile primary open angle glaucoma to be the late onset of con-
genital glaucoma where trabecular meshwork was not obstructed enough to cause rise of
intraocular tension before three years and was not open enough to maintain normal outflow
after three years. Others postulate that the condition is early onset of adult primary open
angle glaucoma.
Incidence of juvenile primary open angle glaucoma is far less than its secondary coun-
terparts, commonest age of diagnosis is eighteen years. The range being eight to thirty years.
The condition is bilateral. There may be a history of P.O.A.G. or myopia in the family. The
condition is autosomal dominant. It is more often missed than diagnosed till it is advanced.
The cornea
77
is devoid of stigma of congenital open angle primary glaucoma, or traces of
mesodermal dysgenesis. Only suspicious corneal change may be an enlarged cornea.
There is no evidence of trauma, uveitis or use of steroids.
The children are brought only for diminished vision. On examination the child may be
myope. Positive signs that warrant measuring of intraocular tension are changes in the optic
nerve head. There may be asymmetry of the cup, notching of the cup, change in CD ratio.
Tension can be recorded under local anaesthesia either by Schiotzs tonometer or handheld
applanation tonometer in children as young as six years provided the child is explained that
the procedure is painless, but is an essential procedure for treatment. Generally the child is
more co-operative during subsequent visits. No touch tonometer is better alternative.
The tension may be as high as 40-50 mm kg. The anterior chamber is clear with normal
depth, the angle is wide without any uveal tags, synaechia or pigments. The field changes are
similar to those seen in adult primary wide angle glaucoma.
Treatment :
The principle and mode of management is same i.e. medical/surgical as in adults.
I. Medical treatment consists of
(a) Local instillation of 1. Direct acting miotics, 2. Beta blockers, 3. Alpha agonist, 4.
Epinephrine and dipiveprin, 5. Local carbonic anhydrase inhibitor.
(b) Systemic carbonic anhydrase inhibitors for short term.
II. Surgical treatmentSurgery is indicated if local treatment fails to keep the tension
within normal range.
1. Goniotomy has uniformly poor result over three years of age.
2. Trabeculectomy gives better results. However the results are not as good as in
adults because children have increased tendency for subconjunctival fibrosis that
obliterate the opening. The fibrosis can be minimized by use of antimetabolites.
Besides keeping intraocular tension within normal range and stabilising the field
changes, the childs refractive error should also get due importance. These children are
invariably myopes. There is always a change towards myopic astigmatism following filter-
ing surgery. Uniocular poor vision usually produces amblyopia that should be managed by
standard procedures.
Outline of various modes of management of glaucoma. Aim of management of
glaucoma is to lower the intraocular tension, to make it compatible with vision and stop the
progress of field and fundus changes. Fundus changes are reversible under three years
of age.
This can be brought about by :
A. Medical treatment
B. Surgical treatment
C. Non invasive physical methods
A. Medical treatment comprises of
1. Local drops
2. Systemic drugs
Medical treatment
1. Local drops mostly act on autonomic nervous system either as agonist or antagonist
can either be :
(a) Parasympathomimetic
99, 100, 105
:
(i) Direct acting
(ii) Indirect acting
(iii) Combined
(b) Beta blockers :
Non selective. These block both beta one and beta two receptors.
Selective. These are also called cardio selective. They are more potent beta one
blocker than beta two.
(c) Sympatho-mimetics, they can be :
(i) Alpha adrenergic agonists
(ii) Beta adrenergic agonists
(Adrenergic antagonists are no more in vogue in management of glaucoma)
(d) Prostaglandin analogue (analogs) Latanoprost
(e) Prostamides (LumiganBimatoprost .005%)
(f ) Other ocular hypotensive lipids
(g) Local carbonic anhydrase inhibitors :
(i) Short acting CAI
(ii) Delayed acting CAI.
2. Systemic drugs
(a) Carbonic anhydrase inhibitors
(i) Short acting
1. Oral labels
2. Injectables
(ii) Delayed acting
(b) Hyperosmotic agents
(i) Oral glycerol
Isosorbide
(ii) Injectable Mannitol
Urea
The anti-glaucoma drugs can be put in two groups i.e.
(i) Those reduce formation of aqueous
(ii) Those that increase outflow.
The drugs that decrease formation of aqueous are Beta blockers, epinephrine, depivprin,
apraclonidine, brimonidine and C.A.I.
Those that increase outflow are : Miotics, epinephrine, depiveprin, brimonidine and
latanoprost.
Hyper osmotic agents are altogether different from above two groups, they act by
increasing blood osmolarity and drawing water from vitreous.
Direct acting parasympathomimetic drugs. Pliocarpine is an alkaloid
100
used ei-
ther as nitrate or chloride in strength of 1% to 2% and rarely 4%. Percentage higher than 4%
do not have any additional advantage however side effects increase with increased concentra-
tion. It has best IOP lowering effect at pH 6.5. Pilocarpine is mostly used as drops
102
. How-
ever it can be used as gel, ointment, ocuserts and soft contact lens soaked in
pilocarpine
101, 102
. Action of pilocarpine starts with in 20 minutes and passes off in 4 hours.
Stronger solutions have more prolonged effect. Hence drops in lower strength have to be used
more frequently, action of gel and ocusert last longer than aqueous solution. Addition of
betablockers and alpha agonist have synergistic effect on pilocarpine and vice versa. Pilocarpine
when used alone has to be instilled four times a day. This frequency is reduced to twice a day
when used with beta blockers or alpha agonist.
Pilocarpine acts directly on cholenergic receptors. Most visible action of pilocarpine is
miosis. It also causes cyclotonia, increasing ciliary tone. Cyclotonia and miosis are insepara-
ble but they lower IOP in two different ways. Miosis is effective in lowering IOP by pulling the
iris away from the trabecular meshwork thus opening a mechanically closed angle in primary
closed angle glaucoma. The miosis has an adverse effect i.e. pupillary block. Synecheal blocks
are however not relieved by pilocarpine.
In primary open angle glaucoma, pilocarpine contracts the longitudinal fibres of ciliary
body which being anchored to scleral spur, automatically pull the scleral spur and trabecular
meshwork, opening the meshwork thus increasing aqueous outflow.
Pilocarpine in no way acts on production of aqueous. It has an adverse effect on uveoscleral
outflow. Hence pilocarpine is contra indicated in permanent secondary angle closure. Fre-
quent instillation of pilocarpine increases permeability of uvea blood vessels leading to de-
rangement of blood aqueous barrier. This results in plasmoid aqueous rendering pilocarpine
unsuitable in inflammatory and neovascular glaucoma. Pilocarpine has no role in
buphthalmos.
Adverse effects of pilocarpine are local as well as systemic. Side effects depend upon
strength, frequency, duration and amount of systemic absorption. Immediate local side effects
is burning in the conjunctiva. This is pH dependent, lesser the pH more is the sting, pH 6.5 to
7.5 is better tolerated than ph 4.0.
Conjunctival congestion, miosis and spasm of accommodation are other local side
effects.
Miosis leads to following adverse effects. Distant vision is drastically reduced in
presence of central corneal and lenticular opacities, night vision is diminished due to non-
dilatation of pupil in dark. Generally there is constriction of peripheral field and apparent
increase in size of field defect.
Cyclotonia leads to spasm of accommodation, transient myopia and frontal headache.
Delayed effect consist of epiphora due to spasm of puncta and canaliculi. Cholinergic effect of
pilocarpine on lacrimal gland enhances tear production.
In myopic eyes strong miotics are known to produce retinal detachment and subscapular
opacification of lens. Prolonged use of pilocarpine may produce iris border cysts, which can be
prevented by use of short acting mydriatic like phenylepherine from time to time.
Abnormal tear film status is a common feature of prolonged use of pilocarpine. Increased
salivation, colic and diarrhoea are some systemic effects.
Other miotics are not used in children to treat glaucoma.
B. Sympathomimetic drugs (adrenergic agonist). There are two types of drugs in
this group. The drugs that stimulate both alpha and beta receptors are known as non selec-
tive alpha agonist. Those drugs that stimulate only alpha two receptors are called selective
alpha agonist. These drugs increase outflow facility and to some extent reduce formation of
aqueous.
The drugs in the first group are :
Non selective alpha agonist are :
(i) Epinephrine (ii) Depivefrin
I. Epinephrine. Epinephrine is used as aqueous drop, commonly used strength is 1%
to 2% given either once a day or at the most two times a day. The salts of epinephrine available
are hydrochloride, bitarterate and borate. Out of all salts the borate is least irritating due to
its pH 7.4, which is nearer to pH of normal tear.
Epinephrine directly stimulates both alpha and beta receptors. The IOP lowering mecha-
nism of epinephrine is most probably a dual one, the first an early but short period of de-
creased aqueous formation and the later a long period of increased outflow. The increased
outflow has an early and late phase. The early phase of increased outflow starts within few
minutes but the late phase takes weeks to months fully to operate.
103, 107
Untowards effect of epinephrine consists of :
1. Tachyphylaxis is common as epinephrine.
2. Besides lowering intraocular pressure, epinephrine causes - constriction of conjunc-
tival vessels leading to blanching of conjunctiva. This is short lived, followed by re-
bound hyperemia.
3. Mydriasis with epinephrine is prompt without cycloplegia. The mydriasis produced
by epinephrine, is not counter acted by any drug in therapeutic dose. Hence
epinephrine is contra indicated in narrow angled eyes.
4. Lid retraction. Due to stimulation of Mullers muscle, there is always some lid
retraction.
5. Prolonged use of epinephrine causes :
(a) Cystoid macular edema specially in aphakic and pseudophakic eyes.
(b) Allergic blepharo conjunctivitis.
(c) Pigmentation of conjunctiva.
6. The systemic side effects consist of :
Tachycardia, cardiac arrhythmia and hypertension.
Epinephrine is not a suitable drug for children.
II. Dipiveprin. This is synthetic epinephrine substitute. It is a pro drug i.e. it under-
goes bio-transformation before exhibiting pharmacological action
104, 105, 106
. The compound is
hydrolyzed to epinephrine after absorption in the eye. It is more lypophilic than epinephrine
making its corneal penetration many times more than epinephrine. Its action is similar to
epinephrine. It is used as 0.1% solution two times a day, its mydriasis is more than epimephrine.
It does not stain contact lenses, maculopathy is less common. Additions of betablockers have
an additive effect. It can be used in children. Its role in buphthalmos is uncertain.
Selective alpha agonist. These drugs selectively stimulate alpha two receptors. They
reduce intraocular tension in two ways i.e. reducing aqueous formation and increasing
uveoscleral outflow.
The two commonly available drugs are Apraclonidine and Brimonidine. Apraclonidine
is a selective alpha two agonist. It is a derivative of clonidine
107, 108
, which is no more used in
management of glaucoma due to its untoward side effects. Apraclonidine has prompt IOP
reducing property when used as one percent solution. This property has been utilized to re-
duce sudden rise of tension following anterior segment laser procedures for glaucoma. It is
used as 0.5% drops two times a day for management of glaucoma. It crosses the blood brain
barrier minimally so its systemic side effects are very low and has no cardiopulmonary contra
indication. Minor systemic side effects consists of dry nose and dry mouth. Commonest ocular
side effect is chronic follicular conjunctivitis. Other ocular side effects consists of unwanted
mydriasis, lid retraction and conjunctival blanching.
Brimonidine. It is selective alpha two agonist that reduces production of aqueous and
increases uveoscleral outflow. It is used as 0.2% drop once or twice a day. It has minimal
systemic side effect.
Betablockers. Betablockers are a group of drug that antagonise the adrenergic beta
receptors. They are effective locally as well as when used orally or parentally. Locally, they are
used only as drops. The local drops have systemic side effects as well. In fact betablockers
when used for cardiological disorders were found to lower the intraocular pressure as well.
The betablockers are most widely used local drops for glaucoma therapy. They have
replaced pilocarpine as first drug of choice. Their main function is to reduce aqueous pro-
duction. The other modes of action are minimal lowering of episcleral pressure and
slight increase in uveascleral outflow
106, 107
. They do not have any effect on pupil size or
ciliary tone.
According to their cardio pulmonary action, they have been divided into two groups i.e.
1. nonselective that block both B1 as well as B2 adrenergic receptors and 2. cardio-selective
that have no or very mild cardiac action.
Both types of betablockers have good additive effect when used with miotic.
Commonly used non-selective betablockers are :
Timolol 0.25%-0.5%
Levobunotol 0.5%
Cartelol 1%-2%
Metiprantol 0.1%-0.3%
0.5% Betaxolol is the only cardio selective betablockers presently available.
Action of betablockers starts within 30 minutes and reach peak effect in 2 hours. The
effect last for twelve to twenty hours. Hence they are administered twice a day. Initial fall of
I.O.P with B.D. dosage is very good which becomes less in few weeks. This is called short
term escape. Reduction in efficacy over months is called long term drift. Betablockers are
effective in all types of glaucoma irrespective of width of angle. They have very little local side
effects. Some eyes may experience ocular surface disorder. Other minor ocular side effects
are burning, diminished corneal sensation and punctate erosion of cornea. The systemic side
effects are bradycardia, reduced cardiac output, arrhythmia, heart failure and syncope. It
causes bronchospasm, depression, anxiety and confusion.
The side effects can be minimised by using smallest possible drop and obstructing the
lower puncta by pressing it with thumb for a minute following instillation in the conjunctiva.
In children it should always be used in consultation with pediatrician.
(d) Prostaglandin analogues. Prostaglandins are locally acting hormones scattered
in various forms in various organs of the body. They are potent ocular hypotensive agents in
very low dose. For ocular use they are available as latanoprost 0.005% drop in form of buff-
ered isotonic solution with pH 6.7 and as unoprostane.
The latanoprost is used once in twenty four hours preferably at night as it reduces the
nocturnal variation better than diurnal variation.
Latanoprost have synergistic effect with all anti-glaucoma drugs except pilocarpine.
Latanoprost and pilocarpine both are known to reactivate uveitis and they are contra indi-
cated in glaucoma secondary to uveitis.
Latanoprost is absorbed through cornea. It is an isopropyl pro drug that is hydrolysed
by esterase in cornea to biologically active acid latanoprost. Peak concentration in aqueous
is reached in two hours.
Its mode of action is by increasing uveoscleral outflow by 100% that reduces IOP by
35%. It is hundred times more potent than timolol 0.5%, has hardly any systemic side effects
however hypersensitivity to latanoprost may occur.
Local side effects consists of reactivation of dormant anterior uveitis, worsening of ex-
isting iridocyclitis. Hence it is contra indicated in uveitis induced glaucoma. It also causes
cystoid macular edema, less serious complications are darkening, thickening and elongation of
lashes, darkening of skin of the lids.
E. Prostamides. These are chemical substances present in the eye like prostaglandin
and histamine. They are neither hormones nor enzymes. They are members of fatty acidamide
family. Prostamides are potent ocular hypotensive agents. They are believed to be involved in
the endogenous regulation of IOP via prostamide sensitive receptors in the eye. Commonest
form of prostamide used in bimotoprost that is a structural analogue of prostamide. It is
used in once a day or twice a day dosage.
Carbonic anhydrase inhibitors (local as well as systemic). Oral carbonic anhydrase
inhibitors are being used to reduce intraocular tension for last half a century
109,111,112
. Local
use of carbonic anhydrase inhibitors (CAI) is a recent development and has not yet become
very popular. Advantages of CAI oral or local as ocular hypotensive agent lies in its versatility
to lower IOP in all types of glaucomas, in all ages, irrespective of width of angle, pupillary size
and duration.
Carbonic anhydrase is an enzyme widely distributed throughout the body that is
responsible for the catalytic hydration of CO
2
and dehydration of H
2
CO
3
. In the eye it is
mainly concentrated in the ciliary epithelium where it is an integral part of aqueous forma-
tion by maintaining pH at optimum level.
Carbonic anhydrase inhibitors are a group of drugs that block the enzyme carbonic
anhydrase and reduce formation of aqueous. By nature they are sulphonamides. They re-
duce formation of aqueous by twenty to twenty five percent. Being a sulphonamide they causes
systemic acidosis and depletes serum potassium.
Acetazolamide is a prototype of all CAI, it is the most widely used systemic ocular
hypotensive agent, mostly orally and rarely as intravenous injection to reduce acutely elevated
IOP especially during retinal surgery.
Action of acetazolamide starts within one to three hours, reaches its peak in four to
six hours. The effect passes off in ten to twelve hours. In children it is given in a dose of 10-20
mg/kg/day in divided doses. Acetazolamide does not change the pupillary size, depth of ante-
rior chamber, width of angle of anterior chamber or structure of trabecular meshwork. Though
CAI are mild diuretics, hypotensive property is independent of diuresis. As ocular hypotensive
agent it can be used with all local and systemic oculo hypotensive agents. Sustained release
acetazolamide has a delayed onset and prolonged therapeutic effect with less untowards ef-
fects. Acetazolamide is not administered for more than seven to ten days due to its systemic
toxicity. However low dosages of acetazolamide i.e. as low as one fourth of usual dose have
been found to give good oculohypotensive effect without much toxicity on long term.
Other carbonic anhydrase inhibitors are : Diclorphenamide 50mg once or two time a
day, Methazolamide 25mg-50 mg pm once or twice a day.
Carbonic anhydrase inhibitors have a long list of systemic toxicity. They consist of 1.
Metabolic acidosis, 2. Paresthesia, 3. Fatigue, 4. Headache, 5. Nausea, 6. Diarrhoea, 7. Metallic
taste, 8. Diuresis, 9. Renal stone formation, 10. Thrombocytopenia, 11. Mild to severe
mucocutaneous involvement may lead to Stevens Johnson syndrome. Only ocular side effect
is transient myopia.
Locally acting CAI. Action of local carbonic anhydrase is same as systemic CAI i.e.
blocking the enzyme carbonic anhydrase at ciliary epithelium due to change in pH thus reduc-
ing production of aqueous. The drug is absorbed through cornea. Absorption depends upon its
lipid and water solubility, contact time and strength. Commonly used local CAI inhibitors are
dorzolamide 2% two times a day, brinzolamide 1% and acetazolamide 5% two times a day.
Local CAI are less effective in children than in adults.
Common side effects are local irritation, conjunctival congestion, metallic taste. Sys-
temic side effects are rare with local drops.
Hyperosmotic agents. Hyperosmotic agents are a group of drugs that lower intra
ocular pressure following oral or intravenous administration by reducing vitreous volume.
These drugs create a fluid gradient between the vitreous and blood which pulls fluid from the
vitreous.
The hyperosmotic agent can either be given : (i) Orally or (ii) Intravenously.
(i) The oral hyperosmotic agents commonly used are :
Gylcerol (Glycerine). This is a sweet, viscous fluid administered orally in a dosage of
1gm to 1.5 gm/kg, a single dose once or twice a day. It is generally given as 50% solution with
lime or orange juice. Glycerol is distributed throughout the extra cellular body fluid. It has
poor ocular penetration that helps to create a better ocular gradient to draw fluid from the eye.
It is well metabolised and does not cause diuresis. Ocular hypotensive effects starts in less
than half an hour, the peak is reached in one hour. The effect lasts for five to six hours. It can
be administered along with CAI in reduced dose. All local antiglaucoma drugs are compatible
with oral glycerine. Only side effect is nausea.
Isosorbide. This less popular oral hypotensive agent is also given as fifty percent solu-
tion that contains 1 to 1.5 gm of isosorbide per kg of body weight, about 90% of it is excreted in
the urine. Peak ocular hypotensive effect is reached in one to three hours that lasts for four to
six hours.
Intravenous ocular hypotensive agents :
The two drugs commercially available are :
Mannitol and urea.
Out of the two, urea is no more in use due to high incidence of side effects.
Mannitol is administered as fast intravenous drip. The dose is 1 gm to
2 gm/kg or 5-10 ml/kg solution. The rate of drip should not exceed 60 drops/mt. Thus it takes
about 30 minutes for 200 ml drug to be administered. Its action starts with in few minutes,
peak is reached in half and hour and effect last for six hours. It is generally given only once a
day and repeated.
Surgery in childhood glaucoma :
Surgical procedures in glaucoma in general. It is difficult to classify surgical pro-
cedures in glaucoma because of diverse etiology and clinical presentation. A procedure may be
suitable for congenital glaucoma i.e. goniotomy that may utterly fail in other type of childhood
glaucoma. Surgery for glaucoma after ten years of age and adult glaucoma are same in princi-
ple. The procedure in childhood requires adequate modification.
The aim of surgery. The aim of surgery is to give stable, near normal intraocular
pressure that prevents further field loss and changes in optic nerve head. A well executed
surgery generally meets with above criteria but many a times a particular surgery may fail
altogether and require either re-operation or addition of medical treatment. Even when a
surgical procedure does not fail it may just be a partial success needing other modes of lower-
ing IOP.
Surgery may be
1. A primary procedure.
2. May follow when medical methods have failed.
3. May be repeat procedure.
Following is an outline of various types of glaucoma that requires different procedures :
1. Glaucoma in childhood :
(a) Congenital primary glaucoma
(b) Congenital glaucoma associated with anomalies of anterior chamber and angle.
(c) Glaucoma in older children.
(d) Secondary glaucomas.
2. Glaucoma in adults :
(a) Wide angle glaucoma
(b) Narrow angle glaucoma
(i) With pupillary block
(ii) Without pupillary block.
3. Absolute glaucoma :
The other possible classification is according to anatomical location of the surgery.
1. Surgery on the iris
2. Surgery on the angle of AC.
3. Filtering surgery.
4. Drainage device
5. Cyclodestructive procedures.
1. Surgery on the iris :
(a) Surgical procedures consist of :
(i) Peripheral basal iridectomy
(ii) Sector or broad based iridectomy
(iii) Button hole iridectomy
(iv) Sphincterotomy
(v) Four dot iridotomy
(b) Laser procedures on irisLaser iridotomyArgon, NdYAG
2. Surgery on anterior chamber angle :
(i) Primary where the surgical procedure involves the trabecular meshwork only :
(a) Goniotomy
(b) Trabeculotomy
(ii) As part of filtering surgery.
Thermal sclerotomy, corneoscleral trephine, iridencleisis, cyclodialysis
3. Glaucoma surgeries can either be a non filtering or a filtering procedures. In the
latter the aqueous drains either outside the eye in subconjunctival space as in trabeculotomy
or may drain in suprachoroidal space as in cyclodialysis. In trabeculactomy a part of trabecular
meshwork is removed. In other procedures the aim is not to disturb the trabecular meshwork.
4. Aqueous drainage devices (See page 350.)
The non filtering surgeries are :
1. Paracentesis. This gives a short term lowering of IOP which returns to previous
level in few hours to few days generally used in secondary glaucoma.
2. Goniotomy and trabeculotomy. They are meant to give sustained lowered IOP.
Various methods employed to lower sudden rise of intraocular pressure :
Acute rise of intraocular pressure may occuras part of ocular disease i.e. primary or
secondary acute glaucoma that may be iatrogenic as is common in various retinal surgeries. A
growth may press the globe from outside. The procedures to reduced acute rise of IOP are :
1. ParacentesisThis is indicated in acute secondary rise of tension.
2. Retrobulbar of anaesthesia
3. Removal of external implants in retinal surgery.
4. Bollus IV mannitol.
5. Massage of
1. Globe
2. Cornea
3. Orbital contents.
Massage of globe and cornea force the aqueous out of the anterior chamber either with
normal or wide angle. This method is used prior to lens extraction. The closed angle may open
up in acute narrow angle glaucoma for a short time following massage of the globe.
The mode of action of orbital massage is not well understood. It has effect of bulbar
massage as far as intraocular tension is concerned. Otherwise it expels fluid from retrobulbar
structures and muscles. This method is more effective following retrobulbar and peribulbar
anaesthesia. Relaxation of extraocular muscles, reduce tension over the sclera. This method is
also used in cataract surgery, has no role in glaucoma. However gentle massage of the globe
through the lid may be used in failed surgery to re-establish the fistula.
Non filtering surgery with permanent lowering of IOP :
1. Surgery to relieve pupillary block.
2. Cyclodestructive procedures.
1. Surgery to relieve pupillary block :
(a) (i) Peripheral button hole iridectomy.
(ii) Peripheral basal iridectomy
(iii) Sector iridectomy
(b) Iridotomy
(i) Laser iridotomy
(ii) Four dot iridotomy
(c) Sphincterotomy and iridoplasty
2. Cyclodestructive procedures :
(a) Cyclo cryo
(b) Laser cyclo ablation
(c) Ultrasonic cycloablation
(d) Cyclodiathermy
Filtering surgeries
1. External filtration procedures
2. Internal filtration procedures
1. External filtration surgeries are :
A. Trabeculectomy
B. Sclerotomy
C. Sclerectomy
D. Iridencleisis
E. Corneo scleral trephine
F. Cyclodialysis
G. Glaucoma valves
2. Internal filtering surgeries are :
(a) Goniotomy
(b) Goniopuncture
(c) Trabeculostomy
Surgical procedures for glaucoma in children :
1. Goniotomy
2. Goniopuncture
3. Trabeculotomy
4. Combined trabeculotomy and trabeculostomy
5. Thermal sclerotomy
6. Shunt operations
7. Cyclodestructive surgeries
8. Iridotomy
Laser in glaucoma
1. General information about ophthalmic laser.
2. Various uses of laser in glaucoma.
General information about ophthalmic laser. Laser is an acronym of light ampli-
fication by stimulated emission of radiation
115, 116
. Laser is artificially created bright light with
high source of energy that is formed when electric charge is passed through a medium. The
medium can be gas, liquid or solid. The media generate photons to emit light energy.
110
The light produced by laser is of uniform in frequency and direction.
111
(a) Usual laser consist of
112, 120
(i) Source of energy. Energy used is generally electric discharge or burst of white
light.
(ii) The medium that generates light energy by emitting photons.
(iii) A chamber (lasing cavity) to reflect energy back and forth by mirrors, one at
each end of the chamber. One transparent, only to emit laser and other totally
reflecting mirror. It also houses the medium.
(iv) Aperture to control laser output.
(v) Delivery system. The delivery system can be a slit lamp, an ophthalmoscope,
an endoscope, an operating microscope or a surface probe.
(b) Properties of laser. The laser is near parallel, coherent, monochromatic linear,
unidirectional, light that can be converted into heat when absorbed by tissue.
The laser can be delivered either as continuous wave or in pulse.
113
Property, colour and
mode of action of laser depends upon wave length and pulse duration. Wave length of laser
used in ophthalmology vary between 193 nm (Excimer) which is nearer to ultraviolet and 1060
nm (carbon dioxide) which is nearer to infra-red.
Ophthalmic laser passes through clear ocular media without being absorbed except
excimer. Laser can get absorbed in hazy media i.e. senile sclerosis of lens. Damage to cornea
and lens is more with long wave laser like neodymium (1016 nm) and CO
2
(1060 nm). Darker
tissues absorbs laser better than lighter tissue. Laser is absorbed by melanin in uvea, RPE,
xanthophyll of macula and haemoglobin.
The density of photons depend upon spot size of laser and power used. Density of pho-
tons increases as spot size is reduced. Energy used in laser is expressed in Joules (MJ) or
Watts (Mw) depending upon the mode of transmission. A continuous wave laser like argon is
expressed in watts while NDYAG which is pulsed laser is expressed in Joules.
C. Various types of accessories used with ophthalmic laser are cooling system and various
types of condensing lenses that concentrate the laser beam at desired spot i.e. Abrahams
lens or a gonioscope to direct beam on the trabecular meshwork.
D. Lasers have various modes of applications in ophthalmology. All of them are not used in
glaucoma. The common uses of laser are :
(i) Photodisruption to do capsulotomy, iridotomy and rupture the vitreous face.
(ii) Photocoagulation of vessels and to create a chorioretinal scar in disorders of
choroids, retina and vitreous.
(iii) Photo vaporising is utilised in vitreoretinal vascular disorders and to seal
fibrovascular fronds at the time of vitrectomy in association with photo coagulation.
(iv) Photo radiation is used in malignant melanoma of choroids and A.M.R.D.
(v) Photo sublimation is used in excimer laser, which acts without heating or coagula-
tion.
E. Laser is delivered to cornea, iris and after cataract by directly focusing the target issue.
Intraocular delivery is achieved either by transpupillary or transscleral route.
Transscleral route is used in diode laser, which is absorbed minimally by the sclera.
F. Types of lasers used in ophthalmology can be solid state or gas lasers. The former
consist of Ruby and Nd-YAG while latter laser consists of argon, carbon dioxide,
krypton, helium, neon, excimer etc.
(i) Argon Laser. Argon Laser is one of the most commonly used lasers in ophthalmic
disorders. It is used to treat disorders of both anterior segment as well as posterior segment. It
has no use in corneal diseases. The medium is argon gas that works on an electric discharge.
Wave length for blue argon is 488 nm, green argon has wave length of 514 nm. It is a continu-
ous wave laser. The energy is in watts. It is least absorbed by ocular media but well absorbed
by melanin, retinal pigment epithelium, haemoglobin and xanthophyll making it very useful
for treatment of glaucoma and vascular retinopathy, as it is absorbed by xanthophyll, it should
not be used too close to the fovea.
In glaucoma it is used to perform argon laser trabeculoplasty in open angle glaucoma
and laser iridotomy in angle closure glaucoma. It has no use in congenital glaucoma and most
of the childhood glaucoma except to establish a passage between AC and PC in limited cases. It
is delivered either by slit lamp or by fibre optics endoscope for intraocular lesions. To be effec-
tive in ALT, a suitable gonio lens and for iridotomy an Abrahams lens is used.
The Abrahams lens
114
consists of a contact lens which looks similar to Goldmann
three mirror gonioscope. On the anterior surface, a plano convex lens of +66D is glued. The
plus lens is decentred to focus the laser beam on the iris. The function of the lens is to double
the diameter of the beam on the cornea and reduces the beam diameter to half on iris. The
advantage of this is that it reduces power density at the cornea to one fourth and increases it
by a factor four on the iris. The Wise lens has a button of +113D.
115
This is not used very
widely. The Abraham lens can be used both for argon and NdYAG laser while using argon
laser the magnification in the slit lamp should be x40.
114
Optics of eye under treatment does
not take any part during argon laser treatment.
II. NdYAG Laser (Neodymium Yuttrium Aluminium Garnet). It is commonly used
laser for capsulotomy in aphakia and pseudophakia. It can also be used on iris. In this the
neodymium atoms are embedded in crystal of yuttrium, aluminium, garnet and energised by
xenon flash light.
112
It works by photodisruption of tissue. Its function is independent of pigment absorption.
It works on relatively transparent media like posterior capsular opacification, after cataract
and anterior vitreous band. It has a short pulse but a long wave length of 1064 nm. Its power
is set in Joules.
When a tissue is exposed to NdYAG, the tissue is ionised and plasma is produced. The
plasma has mechanical properties of gas and electric properties of metal. Expansion of plasma
sends waves inside the eye. Shock produced by these waves mechanically disrupts the tissue.
The shock waves can be transmitted to the retina causing retinal detachment. NdYAG laser
can be used to do iridotomy, iridoplasty and cyclo-ablation.
Advantages of NdYAG. When compared to argon laser, the NdYAG laser has follow-
ing advantages :
Action of NdYAG is independent of absorption by pigment hence it is most widely
used laser for capsulotomy.
Its action on light coloured iris is as good as dark coloured iris. Latter requires more
energy than former.
Number of burst in NdYAG is almost one tenth of that required for argon laser.
Argon laser produces more tissue edema than NdYAG.
Iridotomies done by NdYAG are well circumscribed and have less chances of closure.
Pupillary distortion is less in NdYAG.
The disadvantages of NdYAG are :
Hyphaem : is short lived and stops by pressure bandage. The other disadvantage is
retinal detachment in predisposed eye.
(iii) Diode Laser. This is a semiconductor solid state laser where two light emitting
diodes are used to produce wave length between argon and NdYAG (800-820 nm). Its absorp-
tion by melanin is better than that by NdYAG. It has better scleral penetration than argon. It
does not require separate cooling system. Its small portable size, durability and low cost of
maintenance makes it very popular ophthalmic laser. It is used in treatment of choroidal
neovascular membrane without damaging nerve fibre layer above it and for cycloablation. It
can be used either through the pupil or over the sclera.
(iv) Krypton Laser. There are two types of krypton lasers i.e. yellow and red. Krypton
laser is poorly absorbed by haemoglobin and xanthophyll. This makes it useful in treatment of
lesions near the macula. Good chorioretinal sear makes it useful in treatment of retinopathies
associated with haemorrhage. Presence of blood in vitreous and moderate opacities do not
hamper its use.
(v) Carbon dioxide laser. It has longest wave length among all ophthalmic lasers i.e.
10,600 nm, which is nearer to infra-red and invisible. It is mostly used in occuloplastic surger-
ies. Its photo evaporation property is used for treatment of cutaneous nevus.
(vi) Ruby Laser. This was the first laser used in ophthalmology. Now this is no more
used as better lasers are available.
(vii) Excimer laser. The word excimer is contraction of two words i.e. excited and dimer
(excited dimer). Dimer is a molecule created by the mixture of gas halogen. When these dimers
are subjected to electric field, they are transformed to higher discharge. The excimer has shortest
wave length among all ophthalmic lasers. Its wave length varies between 193 nm when argon
fluoride is used, to 351 nm when krypton fluoride is used.
Excimer laser is absorbed by all layers of the eye specially cornea. This property has
been utilised to reshape cornea in treatment of refractive errors i.e. P.R.K., laser in situ
keratomileusis (lasik) photo therapeutic keratectomy, removal of superficial corneal opacities
116 and lasek (laser sub-epithelial keratomileusis).
The most commonly used wave length is 193 nm because it is just sufficient to break up
interatomic links without effecting adjacent tissue. Thermal effect is minimal. Excimer has
no use in any type of glaucoma.
2. Various uses of laser in glaucoma
122, 123, 124
. From the previous paragraphs, it is
clear that all glaucomas are not suitable for treatment by all laser procedures. There are specific
procedures for specific groups of glaucoma. Laser is not a suitable mode of management
of glaucoma in children in general and congenital primary glaucoma in particular.
Overall result of laser treatment for glaucoma under thirty years of age and secondary glaucomas
is unsatifactory. However laser iridotomy has definite places in pupillary block glaucoma.
Argon, NdYAG and diode lasers are the commonly used lasers for glaucoma.
Commonly used laser procedures in glaucoma are
122, 123, 124
(a) Iridotomy
(b) Trabeculoplasty
(c) Gonioplasty
(d) Cyclophoto coagulation
(e) Panretinal photo coagulation
(f ) Suturelysis
(g) Bleb failure.
(h) Laser scarring to reduce over filtering bleb also.
(i) Gonio photo coagulation
(j) Goniopuncture
(k) Opening failed sclerotomy
(l) Cycloablation
REFERENCE8
1. Duke Elder and Weber K.C. ; Ciliary body in system of ophthalmology. Vol. II, pp.
163, Henry Kimpton, London, 1961.
2. Nema HV, Singh V.P., Nema N. ; The ciliary process in Anatomy of the eye and its
adnexa, second edition, pp. 24. Jaypee Brothers, New Delhi, 1991.
3. Banumathy S.P. ; Uveal tract in Anatomy of the eye, p. 55. Arvind Eye Hospital,
Madurai.
4. Shield M.B. ; Basic aspects of glaucoma in Test Book of Glaucoma. Fourth Edition,
pp. 925, Williams and Wilkins Philadelphia, 1998.
5. Berger B.B., Emery J.M., Brown N.V., Sander D.R. and Peyman G.A. ; The lens cataract
and its management in Principle and Practice of Ophthalmology; Vol. first Indian
edition, pp. 533534. JayPee Brothers, New Delhi, 1987.
6. Vaughan D. and Asbury T. ; Embryology of the eye in General Ophthalmology; Tenth
edition, pp. 12, Lange Medical Publication, California, 1983.
7. Naney Anderson, Hamming and Apple D.- Anatomy and embryology of the eye in Prin-
ciple and Practice of Ophthalmology, Vol. I; First Indian edition, pp. 18-19. Jay Pee
8. Duke Elder S. and Cook S. ; The anterior chamber in System of ophthalmology, Vol. III,
part 1, pp. 171178; Henry Kimpton, London, 1963.
9. Nema H.V., Singh V.P. and Neema N. ; The angle of anterior chamber in Anatomy of
the eye and its adnexa. Second edition, pp. 129130. Jay Pee Brothers, New Delhi,
1991.
10. Agrawal L.P. ; Principles of optics and refraction; second edition, p. 35, CBS Publi-
cation, New Delhi, 1979.
11. Agrawal L.P. ; Glaucoma in Essentials of Ophthalmology. First edition, p. 290,
C.B.S. publishers, New Delhi, 2000.
12. Moses, R.A. ; Intraocular circulation and pressure in Modern Ophthalmology. Vol. I,
First edition, pp. 284503. Edited by Sorsby A. Butterworth, London, 1963.
13. Kanski J.J. ; Iridocorneal endothelial dysgenesis in Clinical ophthalmology second edi-
tion pp. 222, Butter worth London, 1989.
14. Levy S.G. ; Iridocorneal endothelial dysgenesis in Current ocular therapy. Fifth edi-
tion, p. 512513, Edited by Fraunfelder F.T. and Roy F.H., B. Saunders company, Phila-
delphia, 2000.
15. Duke Elder ; System of ophthalmology. Vol. III, part 11. First edition 565606. Henry
16. Howell, D.N., Dammst, Brchette, J.L. : Endothelial metaplasia in iridocorneal endothelial
syndrome. Invt.Oph.Sc. 38:18961901, 1999.
17. Lee W.R., Marshall, G.E., Karkness C.M. ; Corneal endothelial cell abnormality in early
stage of iridocorneal endothelial syndrome. BJO 78 : 629-631, 1994.
18. Sheild B.M. ; Iridocorneal dysgenesis in Text Book of Glaucoma; fourth edition, pp.
226234, Williams and Wilkins, Philadelphia, 1998.
19. Scheie H.G., Albert D.M. ; Glaucoma in Text Book of Ophthalmology. Ninth edition,
pp. 294295. W.B. Saunders company, Philadelphia, 1977.
20. Chaudhary S. and Mitra S. ; Glaucoma associated with developmental and congenital
anomalies in Modern Ophthalmology. Second edition, pp. 511519. Edited by Dutta
21. Shield M.B. ; Developmental glaucoma with associated anomalies in Text Book of
Glaucoma; fourth ediiton, pp. 207250, Williams and Wilkins Philadelphia, 1998.
22. Mann Ida C. ; Developmental abnormalities in eye. First edition, JB Lippincot,
Philadelphia, 1957.
23. Duke Elder S. ; System of ophthalmology. Vol. III, Part 2, First edition, pp. 543547.
24. Deborah Pavan Langston ; Manual of ocular diagnosis and therapy, 2nd edition, p.
260, Littlebrown, 1985.
25. Waring, G.O. Rodrigues M.M., Laibson P.R. ; Anterior chamber cleavage syndrome - A
stop ladder classification. Survey oph. 20:3-10, 1975.
26. Kivlin J.D., Finaman R.M. Crandal. A.S. ; Peters anomaly as consequence of genetic
and non-genetic syndromes. Arch.oph. 104:61-64, 1986.
27. Jain M.R. ; Congenital glaucoma in Text Book of Glaucoma. First edition, pp. 224
227, JayPee Brothers, New Delhi, 1991.
28. J.W. Gitteriger ; Primary congenital glaucoma in Manual of clinical problems in
ophthalmology, First edition, pp. 6566, edited gittenger J.W. and asdourion G.K.
Little Brown, Toronto, 1988.
29. Hoskin H.D., Shatter R.N., Hetherington J. ; Anatomical classification of the develop-
ment glaucoma diagnosis and classification in symposium on glaucoma. Tr new Orleans
acade oph. CV Mosby, 1981.
30. Duke Elder S. ; System of ophthalmology, Vol. III, Part II, edition first, pp. 548563,
31. Walton DS ; Glaucoma Pediatric Ophthalmology. Vol. I, second edition, pp. 585
598, edited by Harley RD. WB Saunders company, 1983.
32. Duke Elder S. ; Buphthalmos in System of Ophthalmology. Vol. III, Part 2, edition
first, pp. 548585. Henry Kimpton, London, 1964.
33. Guttinger J.W. ; Primary congenital glaucoma in manual of clinical problems in
ophthalmology. First edition pp. 6567, edited by Gittinger J.W. and Asdourian G.K.
Littlebrown, Boston 1988.
34. Hoskin H.D., Shaffer R.N. ; Classification of developmental glaucoma. Arch. oph. 102-
1331-1336, 1984.
35. Sridhar Rao B. ; Congenital glaucoma in Modern ophthalmology. Vol. I, Second edi-
tion, pp. 454458, edited by Dutta L.C. JayPee Brothers, New Delhi, 2000.
36. Feitl M.E. ; Juvenile glaucoma in Current ocular therapy. Fifth edition, pp. 483
485, edited by Fraunfelder F.T. and Roy F.H. WB Saunders company, Philadelphia,
2000.
37. Walton D.S. ; Infantile glaucoma in Current ocular therapy. Fifth edition, p. 482,
edited by Fraunfelder F.T. and Roy F.H., WB Saunders company, Philadelphia.
38. Kanski J.J. ; Glaucoma in Clinical Ophthalmology. Second edition, pp. 223225,
Butterworth London, 1989.
39. Wilensky, J.T. ; Glaucoma in Principle and practice of Ophthalmology. Vol. I, pp.
723726, First Indian edition. Edited by Peyman G.A., Saunders D.R. and Goldberg
M.F. JayPee Brothers, New Delhi, 1987.
40. Boger W.P. and Peterson R.A. ; Pediatric ophthalmology in Manual of ocular diagno-
sis and therapy. Second edition, pp. 269273. Edited by Deborah Pavan Langstone
Littlebrown and company.
41. Jain M.R. ; Congenital glaucoma in Text Book of glaucoma. First edition, edited by
Jain M.R. Jay Pee Brothers, New Delhi, 1991.
42. Shields M.B. ; Congenital glaucoma in Text Book of glaucoma. Fourth edition pp.
195206, Williams and Wilkins, Philadelphia 1999.
43. Mandal A.K., Walton D.S., John J. Metomycine augmented trabeculectomy in refrac-
tive congenital glaucoma. Ophthalmology : 101997.
44. Thomas R. Gieser S.E. ; Artificial drainage device in glaucoma surgery in Modern Oph-
thalmology. Vol. I, Second edition, p. 551555, edited by Dutta L.C., Jay Pee Brothers,
New Delhi, 2000.
45. Thoma R. Barganza, Chandrashekher G. ; Role of A.D.D. in glaucoma surgery, ind Jr
oph, 46 : 41.45, 1998.
46. Kanski J.J. and McAlester J.A. ; Glaucoma surgery in Glaucoma A colour manual of
diagnosis and treatment. First edition, pp. 116123, Butter, London.
47. Kanski J.J. and McAlester J.A. ; Congenital glaucoma syndrome in Glaucoma colour
manual of diagnosis and treatment. First edition, pp. 101103, Butterworth, London
48. Shield M.B. ; Developmental glaucoma with associated anomalies in Text book of glau-
coma. Fourth edition, pp. 221, Williams and Wilkins, Philadelphia, 1999.
49. Jain M.R. ; Traumatic glaucoma in Text book of glaucoma. First edition, pp. 163
170, Edited by Jain MR. Jay Pee Brothers, New Delhi, 1997.
50. Shields M.B. ; Glaucoma associated with ocular trauma in Text book of glaucoma.
Fourth edition, pp. 343. Williams and Wilkins, Philadelphia.
51. Asrani S.G., Wilensky J.T. ; Glaucoma after congenital cataract surgery, ophthalmology
: 102:863, 1995.
52. Samples J.R. ; Pediatric aphakic glaucoma in Current ocular therapy. Fifth edition,
p. 499500, edited by Fraunfelder F.T. and Roy H.F. W.B. Saunders Company Philadel-
phia 2000
53. Cioffi G.A., Sullivan P. Buskisk, EMV ; Glaucoma associated with intraocular lenses in
Current Ocular therapy. Fifth edition, p. 472, edited by Fraunfelder F.T. and Roy
H.F. WB Saunders company, Philadelphia, 2000.
54. Dada V.K. in IOL secrets. First edition, pp. 171, JayPee Brothers, New Delhi, 1992.
55. Singh D. Paediatric cataract. A CME series published by All India Ophthalmological
society.
56. Karesh J.W. and Nirankari V.S. ; Factors associated with glaucoma after penetrating
keratoplasty. Am.Jr. Oph. 96:160-1983.
57. Shields M.B. ; Malignant glaucoma in Text Book of Glaucoma, fourth edition, pp.
345350. Williams and Wilkins, Philadelphia, 1999.
58. Simmons R.J. ; Malignant glaucoma BJO 56:263-272, 1972.
59. Chandleir F.A., Simmons R.J., Grant WM ; Malignant glaucoma - medical and surgical
treatment. Am.Jr. Oph. 66:459-502, 1968.
60. Morrision J.C. ; Malignant glaucoma in Current ocular therapy. Fifth edition, pp.
490491, edited by Fraunfelder F.T. and Roy F.H. W.B. Saunders company, Philadel-
phia, 2000.
61. Sebestyn J.G., Schepens C.L., Rosenthal M.L. ; Retinal detachment and glaucoma. Arch.
opl. 67 : 736, 1962.
62. Kanski J.J., McAlester J.A. ; Inflammatory glaucomas in Glaucoma A colour manual
of diagnosis and treatment; first edition, pp. 7177. Butterworth, London.
63. Ritch R. ; Pathophysiology of glaucoma in uveitis. Trans Oph. Soc. of the UK, 101, 321-
324, 1981.
64. Herndon L.W. ; Glaucoma associated with anterior uveitis in Current ocular therapy;
fifth edition, pp. 420471. Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Com-
65. Kwon Y.H. and Dreyer E.B. ; Inflammatory glaucoma. Int.Oph. Clin. 36 : 81-89, 1996.
66. Deborah Pavan Langstone ; Secondary glaucoma in Manual ocular diagnosis and
therapy. Third edition, p. 188, Little Brown, 1991.
67. Sample J.R. ; Corticosteroid induced glaucoma in Current ocular therapy; fifth edi-
tion, pp. 464465. Edited by Fraunfelder F.T. and Roy F.H. WB Saunders company,
Philadelphia, 2000.
68. Kaye L.D., Kalenak J.W., Frice RL, Cunningham R. ; Ocular implications of long term
prednisone therapy in children : J. Pediate Oph. 112:450, 1991.
69. Welensky J.T. ; Hemogenic glaucoma in Principle and practice of ophthalmology.
Ist Indian edition, pp. 706708, Edited by Peyman G.A., Sanders D.R. and Goldberg
M.F. JayPee Brothers, New Delhi, 1987.
70. Kanski J.J. and McAllister J.A. ; Miscellaneous acquired secondary glaucoma in glau-
coma. A colour manual of diagnosis and treatment. First edition, pp.8489,
Butterworth, London.
71. Deborah Pavan Langstone : Glaucoma in Manual of ocular diagnosis and therapy,
third edition pp. 243244, Little Brown and Co., 1991.
72. Gittinger J.W. ; Traumatic hyphaema in Manual of clinical problems in ophthal-
mology; first edition, edited by Gittinger J.W. and Asdourian G.K., pp. 68, Little Brown
and Co., Boston, 1998.
73. Phelps C.D., Watzke RE - Hemolytic glaucoma. Am.Jr.Oph. 80:690, 1975.
74. Shields M.B. ; Glaucoma associated with intraocular hemorrhage in Text book of glau-
coma; Fourth edition, pp. 333336, Williams and Wilkins, Philadelphia, 1999.
75. Jain, M.R. ; Traumatic glaucoma in Text book of glaucoma; first edition, pp. 163
170, JayPee Brothers, New Delhi, 1991.
76. Julia White Side Michael - Ghost cell glaucoma in Current ocular therapy. Fifth
edition, pp. 466469. Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company,
Philadelphia, 2000.
77. Morrison J.C. ; Current ocular therapy. Fifth edition, pp. 469470. Edited by
Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia, 2000.
78. Bartholomew R.S. ; Viscoelastic evacuation of traumatic hyphaema. Br.Jr. Oph. 71 : 27-
28, 1987.
79. Shields M.B. ; Glaucoma associated with ocular trauma in Text book of glaucoma;
fourth edition, pp. 339342. Williams and Wilkins, Philadelphia, 1999.
80. Walton D.S. ; Glaucoma in infants and children in Pediatric ophthalmology. Vol. I,
second edition, pp. 585598. Edited by Harley RD. WB Saunders company, Philadel-
phia, 1983.
81. Jain MR ; Phacogenic glaucoma in Text book of glaucoma present and future.
First edition. Jay Pee Brothers, New Delhi, 1991.
82. Epistein D.L. ; Diagnosis and management of lens induced glaucoma. Ophthalmology
89:227-230, 1982.
83. Paterson C.A., Pfister R.R. ; Intraocular pressure changes after alkali burn. Arch. Oph.
91:211, 1974.
84. Yoshizumu M.O., Thomas J.S., Smith T.R. ; Glaucoma induced mechanism in
retinoblastoma. Arch. Oph. 96; 105, 1978.
85. Walton D.S. and Grant W.M. ; Retinoblastoma and iris neovascularisation. Am.J.Oph.
65:598, 1968.
86. Caron L. Shield and Shield J.A. ; Glaucoma associated with intraocular tumour.
Currentocular Therepy fifth edition p. 476-479 Ed. Fraunfelder F.T., Roy F.H. W.B.
87. Hoskins H.D. ; Neovascular glaucoma - Current concepts. Tr. Ac. Oph. oto 78:330, 1974.
88. Brown G.C., Manargal L.E., Schachat A., Shah H. ; Neovascular glaucoma, etiological
considerations Ophthalmology 91 :315, 1984.
89. Dutta N.K., Dutta L.C. ; Secondary glaucoma in Modern Ophthalmology. Vol. 1, sec-
ond edition, pp. 500510, edited by Dutta L.C.; JayPee Brothers, New Delhi, 2000.
90. Rathod M.K. ; Ophthalmological study of epidemic dropsy. BJO 66 :573-575, 1982.
91. Duke Elder S. ; Epidemic dropsy in System of ophthalmology. Vol. II, pp. 684687,
92. Sood N.N., Sachdev M.S., Gupta S.K. ; The eye in epidemic dropsy VIII National sympo-
sium on glaucoma, Ahmedabad, Academy of Ophthalmology, p. 79, 1987.
93. Sachdev M.S., Sood, N.N., Verma L. ; Pathogenesis of epidemic dropsy glaucoma Arch
Opthalmology 16 : 1221, 1988.
94. Jain M.R. ; Miscellaneous glaucomas in text book of glaucoma present and future,
pp. 213214, Jaypee Brothers, New Delhi, 1991.
95. Ahmed E. ; Epidemic glaucoma in a Textbook of Ophthalmology. Ist edition, pp. 270,
Oxford University Press, Calcutta, 1993.
96. Hakim S.A.E. ; Argemone oil, sangunarine and epidemic dropsy glaucoma. British Journal
of Ophthalmology. 38:193, 1954.
97. Hakim S.A.E. ; Sangunarine and hypo thalamic glaucoma. Journal of All India Oph-
thalmic Society, 10., pp. 83102, 1962.
98. Feitl M.E. and Krupin T. ; Juvenile glaucoma in Current ocular therapy. Fifth edi-
tion, pp. 483485, edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company,
Philadelphia, 2000.
99. Kanski J.J., McAllister J.A. ; Antiglaucoma drugs in A colour manual of diagnosis
and treatment. pp. 104-105, Butterworth, London.
100. Duke Elder S. ; parasympathomimetic drugs in System of ophthalmology. Vol. III,
pp. 557561, Henry Kimpton, London, 1962.
101. Ruben M, Watkin R. ; Pilocarpine dispensation for soft hydrophilic contact lens. Br.J.Oph.
59 :455, 1975.
102. Ramer R.M., Gasset A.R. ; Ocular penetration of pilocarpine, the effect of pH on ocular
penetration. Ann.Oph. 7:293, 1975.
103. Nagataki D.J., Brubaker R.E. ; Early effect of epinephrine on aqueous formation in
normal human eyes. Ophthalmology 88 : 278, 1981.
104. Sears, M.L. ; The mechanism of action of adrenergic drugs in glaucoma. Invest Ophth,
14 :83, 1975.
105. Shields M.B. ; Adrenergic timulators in Text book of glaucoma. Fourth edition, pp.
398408. WB Saunders company, Philadelphia, 1999.
106. Dutta L.C. - Drugs acting on sympathetic system in Ophthalmology principles and
practices; pp. 314315, Current Books International, Calcutta, 1995.
107. Boger WP - III short term escape and long term drift. The dissipation effects of the beta
adrenergic blocking agents. Suv Oph. 28:235, 1983.
108. Wand M., Schaffer R.N. ; Open angle glaucoma in Current ocular therapy; Fifth
edition, pp. 494498, edited by Fraunfelder F.T. and Roy F.H.; WB Saunders company,
Philadelphia, 2000.
109. Wilson R.D. ; The medical treatment in glaucoma in Ophthalmology secrets; pp. 136
141. Edited by Vander J.F., Gault J.A. Jay Pee Brothers, New Delhi, 1998.
110. Camras C.B., Aim A., Watson P., Stymschantz J. ; Latanoprost a prostagalndin ana-
logue for glaucoma efficacy and safety. Ophthalmology. 103:1916-1924, 1996.
111. Becker B. ; Decrease in intraocular pressure in man by carbonic anhydrase inhibitor
(Diamox) Amj. Jr. Oph. 37:13, 1974.
112. Ramkrishna R. and Puthalath S. ; Pharmacognosy and pharmaco kinetics and ocular
hypotensive agents in Modern ophthalmology. Vol. I, second edition, pp. 527538.
Jay Pee Brothers, New Delhi, 2000.
113. Hunter D.G. and West E.C. ; Instrument in Last minute optics. First Indian edition,
pp. 115117. Jay Pee Brothers, New Delhi, 1997.
114. Fried W. ; Optics and refraction in Principles of Ophthalmology. Vol. 1, first Indian
edition, edited by Pyerman G.A., Sander R. and Goldberg M.F. Jay Pee Brothers, New
Delhi, 1987.
115. Shields M.B. ; Principles of laser surgery in glaucoma in Text book of glaucoma.
Fourth edition, pp. 461465. Williams and Wilkins, Philadelphia, 1999.
116. Brown N.A.P. ; The laser treatment of glaucoma in Scientific foundation in ophthal-
mology; first edition pp. 306316, edited by Perkins E.S., William. Heineman Medical
Books, London, 1977.
117. Shields M.B. ; Surgery of the iris in Textbook of glaucoma. Fourth edition, pp. 490
500. Williams and Wilkins, Philadelphia, 1999.
118. Wise J.B., Munnerlyn CR, Erickson P. ; A high efficiency iridotomy sphincterotomy lens.
Am.J. Op. 101, 546, 1986.
119. Singh D. ; Is refractive surgery justified. Jr. I.M.A. 98:748-751, 2000.
120. Ramalingam S. ; Laser application in glaucoma in Modern Ophthalmology. Vol. I,
second edition, pp. 539, edited by Dutta L.C. Jay Pee Brothers, New Delhi, 2000.
121. Sood N.N., Singhotra R. ; Primary open angle glaucoma in Modern ophthalmology.
Vol. I, second edition, pp. 463468. Jay Pee Brothers, New Delhi, 2000.
122. Kanski J.A., McAllister J.A. ; Laser in glaucoma. Glaucoma - A colour manual of
diagnosis and treatment. First edition pp. 116123, Butterworth, London.
123. Sharma P. ; Laser ophthalmology in Essentials of ophthalmology. First edition, pp.
363365. Modern Publishers, New Delhi, 2000.
124. Boyds : Lasek ; Laser subepithelial keratomileusis. Highlights of ophthalmology, Indian
edition 30 : 15-17, 2002.
CHAPTER 11
Disorders of the Retina and
the Vitreous in Children
The retina is highly specialised neural tissue of the eye meant for, vision, colour vision,
night vision and field of vision. Rest of the eye is constructed round it, to house it, protect it,
nourish it and focus on it. It may be considered as an out pouching of the forebrain. It is so
similar to forebrain in structure that half of the layers of sensory retina are considered as
cerebral layers
1
. The light rays reaching the sensory retina form an inverted miniature image
of the object of regard on the most photo sensitive part i.e. fovea. This light thus received is
converted into electric impulse by a complex photo-chemical process and transmitted to the
visual central nervous system
2
via optic nerve which is a bundle of axons of retinal ganglion
cells. The other similarity between retinal cells and cells of brain is that both have nerve fibres
arranged (1) Vertically, (2) Horizontally. The vertical fibres connect photo receptors to the
visual centres directly. The three neurons connected arePhoto receptors (rods and cones),
bipolar cells and ganglion cells. The horizontal fibres provide connections within the retina
mediated by amacrine cells and horizontal cells.
2
The retina is cellophane like transparent structure with a pink hue. The colour of the
retina is due to underlying pigment epithelium, visual purple in rods and retinal capil-
laries.
3
The retina develops from both the layers of optic cup. The outer layer forms the pig-
ment epithelium. The remaining layers develop from the inner layer of the optic cup with a
potential space in between, which is liable to be distended in pathological states separating
the two leaves of the retina. There are no separate sensory, motor or autonomic nerves in the
retina. The retina does not have pain sensation.
Applied anatomy of the retina
1,4,5,6
The retina is the innermost layer of three coats of the eyeball. It lies between the choroid on
the outer side and vitreous on the inner side. It extends from the edge of the optic nerve
posteriorly to ora serrata anteriorly. The ora serrata is not a continuous sheet, it has finger
like projections. The spaces between these projections are U shaped gaps, which are open
towards the ciliary bodies, these are called oral bays. The attachment of the retina at ora
serrata is not uniform all round. It extends more anteriorly nasally and less on temporal side.
1
All the layers of retina are resolved into one layer of non pigmented epithelium that is contin-
ued over the ciliary body.
393
Thickness of the retina is not uniform through out, it is thickest at the optic disc
7
and
thinnest at the centre of the fovea. The outer border of the fovea is the thickest area of the
retina.
7
In the ora, the arterioles and venules look to have same diameter and colour.
8
The ora serrata is represented by an imaginary line joining the attachment of the four
recti on the scleral surface. In adults it is 6 mm away from the limbus. The equator is 6-8 mm
away farther behind the ora.
Functionally the retina can be divided into two areas, the central and peripheral. The
central area is 5 mm to 6 mm in diameter which has dense cone population and less rod popu-
lation, hence it is meant for day vision, fine vision and colour vision. In the centre of the
central arc is the macula. Number of cones fall fast on the periphery. The peripheral retina is
used for coarse vision, night vision and peripheral field of vision.
The macula is a relatively large area as compared to optic disc. It is an area 5.5 mm in
diameter without any definite anatomical demarcation. It lies lateral to the optic disc between
the superior and inferior temporal retinal vessels. Its medial border is very close to the lateral
border of the disc. It subtends an angle of 15 on the nodal point, the disc subtends an angle of
4.5. The centre of the macula is 2 disc diameter away from the lateral margin of the disc and
0.8 mm below the horizontal meridian. The macula looks darker than the rest of the retina
because it contains xanthophyll, which is lipid soluble carotinoid in nature with yellow colour.
The pigment epithelium is more pigmented under the macula than rest of the retina.
The macula is packed with cones. About 10 percent of all cones are located in the macula. The
ratio between rods to cones in the macula is 1 : 2. The number of rods increases on the periphery
and there is proportionate reduction in number of cones on the periphery.
The macula is sub divided into four zonesFoveola, fovea, para fovea and peri fovea.
The foveola is innermost 0.2 mm area. This is little depressed than rest of the macula.
The fovea contains only photo receptors and their nuclei. This makes the retina thinnest at the
foveola. The second and third neurones are displaced circumferentially.
The foveaThe surrounding area 1.5 mm in diameter is called fovea. The cones in the
fovea are more elongated than in other areas. In contrast to foveola which is thinnest part of
the retina, the outer border of the fovea is the thickest part of the retina which is 2.5 mm in
diameter and called para fovea, the remaining part of the macula constitutes the peri fovea.
The central 0.4 mm to 0.5 mm area of the fovea is devoid of retinal capillaries and called
foveal avascular zone (FAZ), which is demonstrated on fluorescein angiography. The expla-
nation for absence of capillaries in the foveola is as follows : The retina has dual blood supply.
The inner part up to inner nuclear layer gets blood supplies from the retinal capillaries. The
remaining outer layers get blood supply from chorio capillaries. As the inner half of retinal
layers are absent in the fovea, the capillaries do not exist at this place.
In a cross section of the retina, through the fovea, following points are noted
9
There are hardly any rods, the cones are covered directly by internal limiting membrane
without other layers in between. The cones are tallest at fovea and are densely packed. The
cones are arranged vertically all over the retina except the foveola where they are placed
obliquely.
In the macula each cone is connected to a single bipolar cell, which synapses with
single ganglion cell. This arrangement is the cause of higher visual threshold and best colour
DISORDERS OF THE RETINA AND THE VITREOUS IN CHILDREN 395
sense at the macula, both of which fall on the periphery. The macula contributes one third of
all fibres to the optic nerve. The macular fibres enter the lateral part of the optic nerve in a
straight line and immediately assume the central part of the optic nerve.
One of the landmark that denotes location of macula is insertion of the inferior oblique
on the sclera.
Layers of Retina
The retina develops from the two walls of the optic cup with a potential space in be-
tween. This potential space divides the retinal layers in two distinct structures -
1. The outer leaf called retinal pigment epithelium (RPE) that is firmly attached to
the Bruchs membrane and is single layered.
2. The inner leaf called sensory retina is multi-layered.
Functionally the sensory retinal layers are arranged in three strata
10
-
1. Visual cells (rods and cones)
2. Bipolar cells
3. Ganglion cells
Histopathologically the retinal layers are arranged in following ten layers:
1. Retinal pigment epithelium (RPE)
2. Photo receptors
3. External limiting membrane
4. Outer nuclear layer
5. Outer plexiform layer
6. Inner nuclear layer
7. Inner plexiform layer
8. Ganglion cell layer
9. Nerve fibre layer
10. Internal limiting layer
Retinal pigment epithelium layer
This is the outermost layer of the retina closely attached to the Bruchs membrane. It is
continuous with pigment epithelium of the ciliary body as a single layer of cells. The pigment
epithelial cells are cuboidal in shape. They rarely undergo division. Hence their number
remains almost constant throughout the life. On a flat preparation, the cells have hexagonal
outline. There are micro villi on the inner surface of the pigment epithelium that anchor the
outer segment of the photo receptors firmly to the pigment epithelium. The pigment epithelium
contains melanin granules, phagosomes and lysosomes. The melanin increases the
efficiency of the cones by absorbing scattered light. In albinos these granules are colourless.
The RPE forms the blood retinal barrier and help in storage and transport of vitamin A
11
Layer of photo receptors
The nine sensory layers of the retina are separated from the RPE by a potential space. The
photo receptors are located next to this potential space and deepest to other eight layers.
Though this layers is designated as layer of photo receptors i.e. cones and rods. The photo
receptors structure wise are not exclusively localised in this layer. This layer consist only of
peripheral processes of rods and cones. The layer of photo receptors in fact extends from the
RPE to outer plexiform layer beyond which they are joined to bipolar cells, and horizon-
tal cells. In between the pigment epithelium and outer plexiform almost mid way lies the
external limiting membrane that divides the peripheral process from the nuclei of rods and
cones.
General structure of photo receptors
Though the rods and cones have different distribution and function structurally, they have
many common points:
Each photo receptor has an inner segment and an outer segment demarcated by
external limiting membrane. Each photo receptor has a cell body, a nucleus, a cell proc-
ess. The long axis of the receptor is at right angles to the retinal surface. The rods and cones
derive their name from the shape of their outer segment. The rods have long cylindrical shape
while the cones have short conical shape. The apexes of the outer segment of rods and cones
are attached in the micro villi of the pigment epithelium.
The outer segments of the photo receptors consist of a set of lamellar disks made up of
lipid proteins. The nuclei of the cones are larger and paler than that of rods. The synaptic
end of the rod is called rod spherule and that of cone is called cone pedicle due to their
difference in shape.
The rods are meant for scotopic vision and peripheral field of vision. The cones are
meant for photopic vision, central vision and colour vision.
External limiting membrane
This is not a true membrane. It is a modified layer of plasma of photo receptors and Mullers
fibre. It is so stretched that the photo receptors seem to be suspended in it with peripheral part
towards RPE. The external limiting membrane ends at ora serrata anteriorly, posteriorly it
blends round the edge of the optic nerve.
The outer nuclear layer
This layer contains nuclei of photo receptors. The nuclei of cones are larger than that of rods
and are situated near the external limiting layer. The nuclei of rods are smaller and placed
away from the external limiting membrane. The nuclei are joined to the second order neu-
rones. Each cone is joined to only one bipolar cell. This is not true for rods. Each rods spherule
is connected to two to three bipolar cells.
The outer plexiform layer
Outer plexiform layer is a narrow space. It lies between the outer and inner nuclear layers,
containing synapses between photo receptors, bipolar cells and horizontal cells. As it lies midway
between the internal limiting membrane and RPE, it does not get sufficient blood supply either
from choroio capillaries or retinal artery, hence it is more likely to suffer from metabolic disorder.
The inner nuclear layer
This layer consists of bipolar cells, horizontal cells, amacrine cells and Muller cells. The capil-
laries of central retinal artery reach up to this level.
The inner plexiform layer
In the inner plexiform layer the axons of bipolar cells, branches of ganglion cells and amacrine
cells synapse. It also contains branches of Mullers cells.
The ganglion cell layers
This layer contains neurologia in which lie the ganglion cells. It is four to five times thicker
under the macula as compared to other parts of the retina. There are about 1.2 million gan-
glion cells each with an axon. All the axons ultimately get together to form the optic nerve.
One ganglion cell serves four to six cones and about a hundred rods. The number of ganglion
cells get reduced towards the periphery, they are densest at the posterior pole.
The nerve fibre layer
It is also called stratum opticum. It consists of axons of ganglion cells. The axons form the
unmyelinated nerve fibre and the optic nerve. The nerve fibres are arranged parallel to the
surface of the retina. The arrangement of the nerve fibres is not similar all over the retina. The
fibres originating from the macula go straight to the temporal side of the disc and are collec-
tively called papillomacular bundle. The fibres arising from the nasal retina travel in radial
fashion to reach the disc. The nerves arising temporal to macula sweep over the macula to
reach the disc in the upper and lower pole of the disc. The peculiar arrangement of nerve fibre
layer is responsible for different shapes of field defect.
The internal limiting membrane
This is a true basement membrane that separates the vitreous from the nerve fibre layer.
Blood supply of the retina
12
The retina has double blood supply. The inner layer up to outer plexiform layer, (the cer-
ebral layer) get their blood supply from the retinal arteries. Rest of the retina gets its blood
supply from the chorio capillaries.
The central retinal artery arises from the first part of the ophthalmic artery near the
apex of the orbit. Its orbital part runs anteriorly under the dural sheath of the optic nerve as
far as a point 10 mm behind the globe, then it takes an upward course, piercing the dural
sheath, arachnoid covering of the optic nerve almost vertically to reach the subarachnoid space,
this constitutes its inter vaginal part. It then passes through the substance of the optic
nerve as inter neural part. After reaching the middle of the optic nerve its course is forward,
as central retinal artery proper to supply the inner layers of the retina and the optic nerve
head. Before terminating as artery of the retina, it give following branches - The pial branches,
the recurrent retinal branch, the inter neural branches.
The central retinal artery divides into two i.e. the superior and inferior branches,
each again dividing into temporal and nasal branches. These four branches are arranged in
such a way that there is one branch for each quadrant of the optic nerve and corresponding
retinal quadrant except the foveal a vascular zone. The rods and cones that are metabolically
most important get their blood supply exclusively from the chorio capillaries. The retinal ves-
sels lie superficially in the nerve fibre layer under the internal limiting membrane.
Generally there is no anastomosis between the retinal circulation and the chorio capil-
laries.
The retinal arteries divided and re-divide to be reduced to arterioles which are end
arteries, ultimately to form capillary network. There are two such networks. The first is at
the level of nerve fibre layer and the second deeper at the level of outer plexiform layer and
inner nuclear layer. There are connecting channels joining the two layers of capillaries.
All the blood vessels are controlled by autonomic nerve system except the retinal system.
15
The blood retinal barrier
13,14
The permeability of retinal blood vessels differ from vessels of other organs-
1. There is a selective exchange of fluid with protein molecule from retinal capillaries to
retinal cells.
2. The retinal capillaries consist of a single layer of non fenestrated endothelium
cells that does not allow passage of fluorescein in normal vessels.
3. The retinal blood vessels are without internal elastic lamina and a continuous layer
of smooth muscles.
4. A basal lamina covers the outer surface of the vessels.
The blood retinal barrier is a physiological barrier meant to optimise fluid passage into
the retinal cells. Only a few metabolic products can pass this barrier from outside.
The barrier is two layered. Tight junction between the pigment epithelium cells forms
the outer barrier. The inner is due to tight junction of endothelial cells in retinal capillaries.
Retinal veins
The retinal veins have the same name as retinal arteries. The retinal veins start on the periphery
and end in the central retinal vein that leaves the optic nerve 12 mm behind the globe, in more
angular fashion than the central retinal artery. Thus the central retinal vein has a longer
course in the inter vaginal space than retinal artery. This makes it more exposed to pressure
changes in the inter vaginal space. The retinal veins have larger diameter than the arteries in
a ratio of 3:2. The arteries and veins share a common sheath, generally the artery crosses over
the vein. The veins drain the retina in quadrants. The central retinal vein generally drains
directly into cavernous sinus. It also partly drain in superior ophthalmic vein, rarely it drains
in inferior ophthalmic vein.
Development of retina
16
The retina except the blood vessels is neuro ectodermal in origin. Its development becomes
obvious from very early stage before even the closure of the neural tube i.e. 2 mm stage and
continues to develop (macula) after birth. It develops from the optic cup. The two layers of the
optic cup are involved in development of the retina. The outer layer gives rise to a single layer
of retinal pigment epithelium (RPE). The remaining layers that form the sensory retina
develop from the inner layer of the optic cup. A potential space is left in between the two layers
throughout the life and when distended causes separation of sensory retina from RPE.
Development of retinal pigment epithelium
The cells of the external walls of the optic cup become pigmented. Its posterior part actually
form the pigment epithelium of the retina. The anterior part continues forward to form the
anterior pigment layer of iris and ciliary body
16
.
Development of sensory (pars optica) retina
To begin with, the inner layer of the optic cup is a single layer of epithelium. It divides further
to give rise to nine layers of retina which can be grouped broadly in following three groups:
1. The cells that give rise to rods and cones;
2. The cells that give rise to bipolar cells, ganglion cells and supporting tissue.
3. Cells that form ganglion cells axons and nerve fibre layer.
Stages of development of retina
16,16A
Stage IDevelopment of outer nuclear zone and non nucleated marginal zone.
Stage IIDifferentiation into inner non nucleated zone and inward migration of the
outer nuclear zone.
Stage IIIDevelopment of inner neuroblast and outer neuroblastThe ganglion cells,
Mullers fibres and amacrine cells develop from the inner neuroblast. The outer neuroblast
gives rise to horizontal cells, nuclei of rods and cones. From the Mullers fibres develop the two
limiting membranes, from the ganglion cells develop the nerve fibre layer.
Stage IVThis is the final stage of organisation of all nine layers.
Development of macula
Development of macula differs from development of rest of the retina. All the layers of adult
retina are well formed by fifth month of intra uterine life. Vascularisation of inner retinal
layers is complete by eighth month. The macula starts developing very early stage of
embryogenesis and progresses fast only to be slowed down and then continues to grow several
months post natal. The differentiation of macula is fast up to first three months after which
there is sluggish differentiation of central area. The rest of the retina continues to be differen-
tiated and grow. This retardation continues up to eighth month. Then the differentiation con-
tinues like rest of the retina. At six months of life the macular area is thicker than rest of the
retina. By seventh to eighth month the macula starts thinning due to shift of ganglion cells
towards the periphery. At birth the ganglion cells at fovea are reduced to single layer.
Congenital anomalies of retina
Congenital anomalies are numerous and present variable clinical features. They may be
symptomless or may cause profound visual loss. They may be obvious at birth, may start at or
before birth but manifest late. They can be uniocular or bilateral. They may be present in
retina only or may be associated with congenital anomalies of choroid or optic nerve. Anomalies
of retinal vessels are very common. As development of vitreous is clearly associated with that
of retina, congenital vitreo retinal lesions are common. The congenital anomalies of retina and
vitreous have frequent hereditary background, however, sporadic cases are also known.
It is difficult to classify congenital anomalies of retina and vitreous due to their clinical
diversity.
They can be classified in following groups:
1. Ophthalmoscopic appearance. They include coloboma, opaque nerve fibre,
Bergmeister papillae, macular anomalies, anomalies of blood vessels, anomalies of
pigmentation, albinism, retinal detachment, retinal folds, hamartomas.
2. Physiological (visual). Diminished night vision, diminished day vision, abnormal
colour vision.
3. Anatomical. This is most probably best suited classification.
The anatomical classification may be :
1. Congenital anomalies of the retina
(a) Anomalies of structures arising from outer layer of the optic cup.
(b) Anomalies of the structures arising from the inner layer of the retina
(i) Anomalies of the retinal periphery
(ii) Anomalies of the macula
2. Congenital anomalies of retinal vessels
3. Congenital anomalies of optic nerve
4. Congenital anomalies of vitreous
5. Combined vitreo retinal anomalies
Anomalies of structures arising from outer layer of the retina
The only retinal layer that arises from the outer wall of the optic cup is retinal pigment
epithelium. The congenital anomalies of this layer are fewer than those of sensory retina.
The anomalies mostly lead to pigmentary disturbance. Commonest anomaly is embryopathic
pigmentary retinopathy and localised congenital pigmentation of the retina.
Congenital embryopathic pigmentary retinopathy
This is secondary to intrauterine infection by syphilis, rubella and influenza in the first trimester.
The condition is bilateral without any symptoms. The retina is studded with bluish black
pigmented dots. The appearance is called salt pepper fundus. The appearance lasts life long.
The condition is confused with retinitis pigmentosa. There are no vascular changes. There
may be associated optic atrophy. No treatment is required.
Other major anomaly of pigmentation is albinism.
Albinism
18
Albinism is a common congenital disorder of metabolism of amino acid tyrosine. The condi-
tion has world-wide distribution, the incidence is as high as 1:20,000. However it is reported to
be more among coloured races. This may be due to the fact that change in colour of hair, skin
and eyes are more visible in dark person than in blonds. Both the sexes are affected equally.
Women are the carriers in x linked ocular albinism where they show some features of albi-
nism. Albinism can be either an autosomal recessive or an autosomal dominant trait. It
has been observed that sometimes when both the parents have some traits of albinism, they
have normal children, this happens when one of the parents is tyrosinase negative and the
other is tyrosinase positive. (See page 236)
Clinically albinism can belong to any of the following:
1. Cutaneous albinism
2. Oculo cutaneous albinism
3. Ocular albinism
4. Albinism associated with systemic syndromes.
Whatever may be the clinical presentation, the basic pathology is failure to form mela-
nin, the pigment that is responsible for colour of the skin and hair
19
. The melanin is formed in
a special type of cells called melanocytes which are derived from neural crest. The formation
of melanin involves a chemical reaction involving amino acid, tyrosine and enzyme
tyrosinase. The melanocytes form melanosomes, which are packed with granules that con-
tain the pigment melanin. The melanin is formed from tyrosine that is converted to tyrosinase,
which in turn is converted to dihydro phenylalaline and to quinone.
Cutaneous albinism
The skin is hypo-pigmented in patches. The patches may be congenital or may develop later.
The patches may involve the skin of the lids, lashes or eyebrow without any ocular defect.
Oculo cutaneous albinism
This is the commonest type of albinism met with. Clinically there are two types
20
of oculo
cutaneous albinism:
1. Tyrosinase positive oculo cutaneous albinism
2. Tyrosinase negative oculo cutaneous albinism
The difference is made out by hair bulb incubation test. The test consists of incuba-
tion of plucked hair bulb in tyrosine solution. In case of tyrosinase positive albinism, the hair
bulb becomes dark. Tyrosinase negative shows more clinical features than positive group.
The ocular features of oculo cutaneous albinism consist of
The skin of the lids is white so are the eyebrows and lashes. The conjunctiva looks
hyperaemic. The cornea and AC are normal. The iris is light coloured, devoid of any pigment
and transmits reflected ray. The pupil is of normal size and reaction, the colour of the pupil is
pink in contrast to a black pupil of a normal child.
The eyes may have squint due to errors of refraction. Horizontal nystagmus is almost
constant feature.
Vision is poor. Myopia is more common than hypermetropia. Myopia is frequently
associated with high astigmatism. Near vision is normal but the child may not be able to
read due to nystagmus. To counteract the nystagmus and use the null zone to maximum ben-
efit, the child develops a head tilt, may have head nodding.
Colour vision is fairly good. There is no night blindness. The child is more comfortable in
dim light. In bright light the child has photophobia and glare.
On fundus examination the retinal vessels and choroidal vessels stand out prominently
against the white sclera. The retinal pigment epithelium has granules similar to melanin but
no pigments. The macula is hypo plastic. The optic nerve head can not be differentiated, its
position is denoted by the convergence of the retinal arteries. All types of oculo cutaneous
albinism show some type of abnormal crossing of nerve fibres at the chiasma.
20
Less common ocular findings areParesis of extra ocular muscles, corneal opacity, partial
aniridia, persistent pupillary membrane, developmental cataract, hypo plasia of retina.
The common systemic findings consist of pale white skin, which is sensitive to the sun
light. The body hair is as white as lashes and eyebrows.
Less common extra ocular features areDeafness, mental retardation, and coagu-
lation defect. These features are seen in children associated with syndromes involving mul-
tiple systems.
Syndromes associated with oculo cutaneous albinism -
Chediak-Higashi syndrome
This is a serious, often fatal condition with multi systemic involvement. Chief among them is
chronic lymphadenopathy, lymphomas, mental retardation, hepatospleenomegaly
besides oculo cutaneous albinism. The condition is autosomal recessive. The causes of death
is recurrent infection and lymphoid infiltration.
Ocular findings
Usual findings of oculo cutaneous albinism are i.e. Bilateral corneal opacity, developmental
cataract, there may be disc edema due to lymphoid infiltration.
Waardenburgs syndrome
There is partial albinism, localised in some places. The systemic manifestations are more
than ocular feature. The systemic findings include - Hearing defect, vestibular dysfunc-
tion, epilepsy, and a white forelock.
The ocular findings are:
Fused eyebrows which may be white, poliosis of lashes, long lashes, wide inter canthal
distance, lateral displacement of puncta, frequent chronic dacryocystitis, pseudo or real squint,
blue iris, heterochromia of iris.
Cuteneous albinismThe skin shows patches of hypo-pigmentation with or without
involvement of skin of the lid. It should be differentiated from other causes of vitiligo and
poliosis.
Ocular albinismThe pigmentary deficiency is exclusively seen in the eyes. Exter-
nally the eye looks normal because the iris has normal pigmentation. The pupil is not as pink
as in oculo cutaneous albinism but the hypo-pigmented retina may give a pale reflex. Nystagmus
is common. The retina is devoid of pigmentation that may be generalised or localised. Women
carriers may have less findings in the fundus.
Management
There is no specific treatment for any type of albinism. The treatment is directed to correct
error of refraction, give dark glasses, correct amblyopia as far as possible. Instruct the child to
use full sleeves and full pants, use broad brimmed hat to protect the skin from sunrays. Some
children may require rehabilitation or low vision aids.
Anomalies of structures arising from inner layer of optic cup can be
21
1. Dysplasia and a plasia of retina
2. Retinal folds
3. Retinal cysts
4. Retinal detachment
5. Retinos chisis
6. Retinal dystrophy
7. Retinal degeneration
Retinal dysplasia
Retinal dysplasia represents a group of congenital conditions that are due to faculty differen-
tiation of retinal premordium. It can be of two types:
1. Congenital rosette formation and 2. Congenital gliosis.
Congenital rosette formation can be seen in microphthalmic eye or otherwise
normal eye. The rosettes are formed when development of neural element overshoots its normal
limits. The number of rosettes in an eye may vary from single to multiple. They may be associated
with folds in non pigmented epithelium of the ciliary body. The rosettes may be seen anywhere
on the retina as masses that may be embedded in the retina, may protrude in the vitreous. On
histopathology the appearance is similar to retinoblastoma with central lumen. In fact
sometimes they are mistaken as retinoblastoma.
Retinal gliosis
There is proliferation of glial tissue in excess of normal limit. The proliferated glial tissue may
be mistaken as intraocular tumour.
Retinal folds
They are caused due to proliferation of inner layer of optic vesicle. Sometimes persistent primary
hyper plastic vitreous may cause a traction fold where features of persistent primary hyper
plastic vitreous may overshadow the whole picture. Sometimes the folds may span between
the optic disc and ciliary body. The common site being inferior temporal retina. Large congenital
retinal folds may give a white reflex in pupillary area.
Retinal cysts
Sometimes retinal dysplasia or microphthalmia may be associated with retinal cyst. Periph-
eral retinal cysts may be asymptomatic and discovered only on routine indirect ophthalmoscopy.
They are presumed to be precursors of retinal dialysis or retinoschisis.
Congenital retinal detachment
Congenital retinal detachment takes place when the two layers of the optic cup fail to come in
intimate proximity due to unequal rate of growth of the two layers. It is generally seen on the
periphery. It may be caused by intrauterine inflammation, is common in microphthalmic eyes.
Congenital retinoschisis
Retinos chisis is a condition where there is a split in the sensory retina, in contrast to a split
between sensory retina and retinal pigment epithelium in retinal detachment which is far
more common than retinoschisis. In retinoschisis the split is at the level of outer plexiform
layer. Congenital retinoschisis is a familial condition. The inheritance is variable. It effects
both boys and girls. It is present at birth but diagnosed late either during routine examina-
tion or the child presents with diminished vision or squint. A late stage of congenital retinoschisis
is termed as juvenile or idiopathic retinoschisis.
The condition is generally bilateral and symmetric. The inner layer is raised, dome
like area of elevated sensory retina. It may have a hole in the inner table. However presence of
a full thickness hole will cause rhegmatogenous retinal detachment.
Congenital retinal degeneration and dystrophies
Almost all degeneration/dystrophies are congenital in nature, may be present at birth but
diagnosed later. They are bilateral and almost symmetrical most of the times. Commonest
symptoms being diminished night vision with constricted peripheral field. For details
(See page 434.)
Congenital functional defects of vision due to anomalies of retina are:
1. Congenital night blindness
2. Congenital day blindness
3. Congenital colour blindness
Congenital night blindness
This is a rare condition that runs in families with variable inheritance. The striking features
are:
1. Absence of signs of hypovitaminosis A, which is otherwise commonest cause of dimin-
ished night vision in children in developing countries.
2. Normal ophthalmoscopic appearance
The central vision remains normal in contrast to retinitis pigmentosa which is commonest
cause of non reversible night blindness in older children where central vision may be effected.
Diminished vision in congenital night blindness is attributed to associated myopia. The colour
vision is normal. Night blindness is stationary and permanent. The fields are normal,
may have subnormal ERG and EOG.
Differential diagnosis consists of xerophthalmia in developing countries that responds
to administration of vitamin A and other causes of diminished night blindness i.e. retinitis
pigmentosa and its variations.
Congenital day blindness
This is even rarer than congenital night blindness. There is better vision in dim light. The
colour vision is also defective. The eyes are generally amblyopic. There may be some mental
retardation. The cases are generally passed as amblyopia. The condition is hereditary.
Congenital colour blindness
Except for a few acquired conditions, all colour defects are congenital in origin with strong
family history and variable inheritance.
Congenital anomalies of retinal vessels
Congenital anomalies of retinal vessels are common. They may be in the form ofDivision of
arteries, their course, tortuosity, capillary malformation, cilio retinal vessel and telangiectasia.
Generally the central retinal artery bifurcates into superior and inferior branches at
the optic nerve head. As a congenital anomaly the bifurcation can be in the orbit. The superior
and inferior division may trifurcate instead of bifurcation. Abnormal tortuosity is more frequent.
It may be primary anomaly without any clinical significance or may be part of some other
vascular malformation. Telangiectasia is seen in Wyburn-Mason syndrome, Sturge Weber
syndrome, Coats disease. Angioma formation is characteristics of von Hippel-Lindau disease.
Cilio retinal artery
It is seen in about 15% to 20% eyes as a small twig arising from the temporal border of the
optic disc and supplying the retina between the macula and the disc though it is called cilio
retinal artery and supply the retina, it is in fact part of ciliary circulation. It arises from the
ophthalmic artery. It is occasionally seen in both eyes. It is more common in hypermetropic
eyes
22
. The clinical significance of this anomaly is that the macular blood supply is partly
sustained in case of central retinal artery block.
Congenital anomaly of retinal vessels of late onset
There are few conditions of congenital and hereditary origin that manifest late with profound
loss of vision. The main pathology in such cases is not presence of congenital anomaly itself but
associated endothelial defect that cause escape of exudate, blood or both from the
incompetent endothelial lining of abnormal vessels
24
causing superficial and deep exudates
and haemorrhage. Formation of vitreous bands and circinate retinopathy is common. In
all cases with past evidence of exudation, the retinal periphery should be examined with indirect
ophthalmoscope and fluorescein angiography. Most of the cases are bilateral and
simultaneous but not always symmetric. One eye may be involved more than the other.
The common condition seen in pediatric age group are:
1. Retinal telangiectasis (Coats disease)
2. Retinal angiomatosis (von Hippel-Lindau disease)
3. Retinal cavernous hemanioma disease (see chapter on retinoblastoma)
4. Arterio venous aneurysm (Racemose aneurysm).
Coats disease (Primary/Congenital retinal telangiectasis)
25,26
The condition is also known as Lebers miliary aneurysm. (see chapter on retinoblastoma).
It is an exudative retinal vasculopathy that can be seen on any part of the retina but
peripheral lesions are more. The temporal retina is most commonly involved. Both the
arteries and veins are involved. The condition is non hereditary, boys are affected more
than girls in a ratio of 80 : 20. In 80% of cases the condition is unilateral. However the other
eye should always be examined for evidence of peripheral lesion because such lesions may be
managed better than the original eye, which may be in advanced stage of disease and difficult
to treat.
Though most of the children with Coats disease seek help only after 8 to 10 years of age.
The condition has been divided into two forms - (1) Juvenile, (2) Adult. The adult cases are in
fact the juvenile cases that have been asymptomatic. Juvenile form has been detected even
before one year of age. As far as progression is concerned earlier the lesion develops faster is
the growth and poorer is the prognosis. Some peripheral lesion may become stationary.
Coats divided the condition in three groups:
1. No vascular changes, only massive retinal or sub retinal exudation, no haemorrhage.
2. Multiple vascular changes, retinal and sub retinal haemorrhage, massive retinal and
sub retinal exudates
3. Prominent retinal vascular changes, in the form of arterio venous communication
and late exudation. This stage was later considered as separate entity not related to
Coats disease and named as angiomatosis retinae.
To these three groups a separate clinical feature of miliary aneurysm was added and
the condition was also known as Lebers miliary aneurysm. However the condition was
proved to be a milder and early form of Coats disease.
27
The exact cause of the disease is not known, most probably there is incompe-
tence of retinal vessels.
Clinical features
28,29
Clinical features depend upon stage of the disease at which the child is brought.
1. It may be quiescent
(a) Discovered on routine fundus examination
(b) The child is brought with unilateral squint, diminished vision.
In the above stages the eye is non-congested without any signs in the anterior segment.
2. Stage of white reflexThis is commonest stage when a child either is brought by
parents or referred by pediatrician who is justified to think it as retinoblastoma.
3. Stage of complication
(a) Congested eye
(b) Raised tension
(c) Complicated cataract
(d) Retinal detachment
4. Stage of blindness due to above conditions and total retinal detachment.
Signs in a well established case:
1. There may be a retro lental white reflex in the pupil on oblique illumination.
2. On retinoscopy the reflex is grey instead of usual pink.
3. Fundus examinationInitially the media are clear. The changes are in the
(a) Vessels
(b) Retina
Retinal vessels
The changes are seen both in the artery as well as veins. The changes are - Bunches of
beading, kinking, and loop formation.
The calibre of the vessels are irregular, the vessels are tortuous, may show aneu-
rysm formation. There may be establishment of communication between the artery and the
veins. Neovascularisation may also take place.
Changes in the retina
In type I of original classification of Coats, the changes are predominantly in the retina
or sub retinal space with almost normal vessels that become involved later leading the eye to
the category of type II.
The retinal changes consist of ophthalmoscopically visible areas of greenish white or
yellowish white areas of exudates with shinning spots. The shinning spots are cholesterol
crystals, which are also seen in sub-retinal space. The exudation in the sub retinal space may
elevate the retina and form a mass. The retinal detachment is exudative in nature. If not
treated the retinal detachment extends leading to total detachment and the retina may be
pushed as far as the posterior pole of the lens or very near to it. By this time a white reflex is
visible that must be differentiated from retinoblastoma. There may be some haemorrhages
in the vitreous as the disease progresses, the eye goes into secondary glaucoma, uveitis,
complicated cataract and blindness. It may become painful and require enucleation.
Investigation
Most important investigation in presence of clear media is fluorescein angiography. The
findings are variable according to stage of the disease and type of the disease. Common
fluorescein angiographic findings in established cases of Coats disease are:
1. Vascular changes in the form of tortuosity, beading, loop formation, neovasculari-
sation, multiple-aneurysms which were not visible with ophthalmoscope but stand
out prominently on FFA.
2. Retinal changes consist of leak, area of non perfusion and capillary drop out.
One of the characteristic appearance of fluorescein angiography is light bulb
appearance of dilated aneurysm.
The next important investigation especially in hazy media is CT of the eye, orbit and
skull and ultrasonography of the globe.
CT not only delineates the position of the retina, it shows the sub-retinal mass, there is
no intraocular calcification which is common in retinoblastoma. CT of the brain excludes
possibility of intracranial angiomatosis which is common in von Hippel Lindaus disease, a
condition that may otherwise be confused with Coats disease.
The conditions that come in differential diagnosis are long. It is headed by retinoblastoma
which is not only sight threatening but the life of the child is also jeopardised. The main points
in favour of Coats disease are - Unilaterality, more common in boys; late age of onset, slow
progressive. It never invades choroid or sclera hence it never becomes extraocular, being benign,
no metastasis is seen on x-ray and CT. Intraocular calcification is absent in Coats disease. The
most important similarity between the two conditions is white reflex in pupillary area. Other
conditions that should be excluded are:
Persistent hyper plastic primary vitreous
Congenital cataract
Traumatic cataract
Intra ocular toxocariasis
Retinal angioma
Familial exudative vitreo retinopathy
Total retinal detachment
Management
28,29
There is neither prophylaxis nor specific treatment for the condition. The aim is to clinch the
correct diagnosis. The treatment is directed towards vascular changes mostly on the periph-
eral telengectasis.
This is achieved by
1. Photo coagulationThis is a preferred treatment. The leaking vessels are treated
directly by laser. This may initially increase exudation, which can be reduced by
using lesser energy and smaller spots. The preferred laser is argon laser. It takes
about six weeks for any improvement to be visible and takes months or years for the
exudation to clear.
2. Cryo therapyThe cryo is applied through trans conjunctival route on the periphery
and reaction monitored under indirect ophthalmoscope.
3. If retina is elevated, a scleral buckle may be required.
Recurrence after adequate treatment are known.
Retinal angiomatosis (von Hippel Lindau disease)
The angioma formed in this condition is a hamartoma, which comes under broad classification
of phacomatosis. The lesion develops from retinal capillaries and endothelium of retinal
vessels. In contrast to Coats disease, which is non inherited, this condition is inherited as
autosomal dominant trait. It again differs from Coats disease due to multi systemic
involvement that can be intracranial or visceral.
In fifty percent of cases it is bilateral which is less frequent in Coats disease. It can be
seen on optic nerve head as well. It may be present in the members of the same family
without any symptoms. The condition is most commonly detected in teens or early twenties.
Both the sexes are equally affected.
The lesion starts on the periphery either as single or multiple lesions, all lesions need
not appear simultaneously. The first change occurs in the capillary bed as micro aneurysm
that gradually enlarges to become a small nodule. The final growth is a spherical orange
nodule, may be larger than the disc. The nodule has a feeding artery and a draining vein.
The calibre and the colour of both the feeding and draining vessels become identical with
passage of time.
30
The angioma on the optic nerve is generally not associated with abnormal vessels.
The systemic involvement is seen in about 25% of cases. The systemic involvement becomes
symptomatic later than ocular. It is not always possible to predict which child with angiomatosis
retinae will have systemic involvement. Hence all cases should be subjected to neurological
and systemic examination including USG for visceral lesions, CT and MRI for brain lesion.
The parts of the brain involved areCerebellum, medulla and pons. The spinal cord may
also be involved. Commonest cerebellar growth is hemangio blastoma.
The visceral involvement consists of cyst in liver, lung, kidney, pancreas, cpididymis
and ovaries may also be involved. The skin is never involved, which is common in other
vascular hamartomas. Rare systemic involvement consists of polycythemia, hypernephroma
and pheo chromocytoma.
The clinical pictures are variable.
The retinal lesion may be dormant for years and become symptomatic when there is
exudation under the macular which may form a macular star. In absence of macular involve-
ment, the other causes of diminished vision are retinal detachment, retinal and vitreous haem-
orrhage, secondary glaucoma and complicated cataract.
Management
Treatment of choice is Laser photo coagulation of lesions smaller than optic nerve. Xenon
arc photo-coagulation gives better result in larger angiomas. Small lesion are treated in
single sitting, larger growth require multiple sittings. The aim should be to destroy the growth
directly and not the feeding or draining vessels. Peripheral lesions are difficult to treat with
laser. They are best treated by cryo coagulation.
Retinal cavernous haemangiomas
These are rarer than angiomatosis retinae. They are also hamartomas, may be seen in the
retina or optic nerve head. The condition is also included in group of phacomas. Skin
involvement is frequent. The condition is asymptomatic, does not require any treatment.
However the patient should have regular follow up.
Arterio venous aneurysm
The condition is known by many other names but none of them are appropriate because the
lesions are neither aneurysm, talengiectasis or angioma, though sometimes they may resemble
one of them. It is a congenital malformation of the main retinal vessels, which have end to end
anastomosis without capillary bed in between. Generally there are no systemic involvement.
Rarely brain especially the mid-brain may have similar changes with corresponding neuro
vascular presentation. Involvement of brain is called Wyburn-Mason syndrome. Occasionally
facial bones may be involved.
Common age when the diagnosis is made is generally in the third decade. However it is
not uncommon to see the condition in first decade. It is equally seen in both sexes. On
examination the patient is generally asymptomatic but on a long run due to retinal exudation
there is diminished vision. The anterior segment is normal. The fundus picture is characteristic,
best seen by indirect ophthalmoscope. The veins and arteries are of same colour and calibre. It
is difficult to find out the line of demarcation between the two. There may be some retinal
exudation and haemorrhages. Fluorescein angiogram differentiates it from other angiomas.
Initially the sensory retina is not involved. In well established cases micro-aneurysms and
capillary non perfusion may be seen. Generally there is no leak. A few cases may regress
without any treatment. There is no specific treatment. Rarely photo coagulation with laser
may be required in selected cases.
Congenital anomalies of the macula
The development of the macula differs from rest of the retina. It starts differentiating early
during embryogenesis, there is a period or retardation followed by growth that lasts in post
natal period. The macula gets its blood supply mostly from the underlying chorio capillaries. It
is almost exclusively packed with cones hence is the seat of central vision and colour vision.
Congenital anomalies may start in early days of gestation or the anomaly may start late and
be discovered late.
Commonest congenital anomaly of macula are macular colobomas. They are frequent
but not as common as coloboma of the uvea. They can be unilateral or bilateral and single,
or multiple, generally asymmetric coloboma is generally elliptical, are placed horizontally
over the macula and extending well beyond the macula. Its size may vary between one disc
diameter to ten disc diameter. The edges are generally irregular with uveal pigments. As per
appearance of the coloboma it has been classified into following groups -
(a) With normal retinal vessels
(b) With abnormal retinal vessels
The second possible classification is
(a) Coloboma with pigment
(b) Without pigment
Both the two groups may overlap.
The pigmented coloboma has heaped pigment. The retinal vessels course over the
mound of pigment. The chorio capillaries under the macula are absent.
Non pigmented colobomaThese are more common. The area is white due to under-
lying sclera that may be ectatic, there are no vessels seen in the coloboma. On the periphery,
which is irregular, clumps of pigmentation are seen.
Coloboma with abnormal vesselsThis type of coloboma is rarest. There may be
anastomosis between the choroidal and retinal circulation or a vessel may pass from the
coloboma towards the vitreous.
The macular coloboma has a strong hereditary predisposition. The coloboma may be
associated with anomalies of the globe, optic disc or choroid.
The exact etiology is not understood. It is said to be a variation of an atypical coloboma
of the uvea. This theory is supported by absence of chorio capillaries and presence of retinal
vessels in the coloboma. This further gets credence due to extension of vessels from the coloboma
into the vitreous. However possibility of maternal toxoplasmosis should always be kept in
mind.
Common clinical presentations areDiminished central vision, nystagmus, myo-
pia, convergent squint.
Hypoplasia of the maculaSome authors consider it as variation of coloboma
23
. It is
generally associated with albinism but can be a primary defect. The exact mechanism is not
known. These children have poor vision with absent foveal reflex.
Other congenital anomalies of macula consist of degeneration of macula.
Retinal diseases in children
Retinal diseases in children can be
1. Congenital
2. Acquired
Out of all the acquired conditions, the most common group of diseases in children are
the retinal dystrophies and degenerations. The sequelae of which spill over adulthood
and may terminate in legal blindness in adulthood. In contrast to this vascular retinopathies
which are a major cause of blindness in adults are less frequent in childhood. Some of them
may be seen in childhood in milder form. One of the unique condition that is seen in children is
retinoblastoma, a life threatening malignant tumour (It has been discussed in a separate
chapter). Rhegmatogenous detachment is also less common in childhood but when present, is
a potential cause of blindness if not treated. Trauma and myopia are two common causes of
retinal detachment in children.
The acquired causes can be
1. Inflammation
2. Trauma
3. Degeneration
4. Retinopathies
5. Tumours
6. Retinal detachment.
Symptoms of retinal diseases in the children
The symptoms hardly differ from those in adults. The difficulty arises when the child can not
express it or fails to realise its importance. This is more so because all retinal diseases per se
are painless.
The symptoms
1. Vision. Commonest symptom is diminished vision. Degree of diminished vision de-
pends on extent of the macular involvement. A large lesion on the periphery may not
produce any dimness of vision, a small lesion on the macula produces extensive loss
of vision. Macular lesions are more likely to produce sudden loss of vision except in
degenerations and dystrophies.
2. Diminished night vision is foremost cause of retinal dystrophies which is gradual,
progressive and associated with poor dark adaptation.
3. Loss of peripheral field again is a symptom of retinal dystrophies.
4. Metamorphopsia is common in retinal detachment and acute macular lesions.
5. Photopsia or flashes of light generally indicate retinal irritation or beginning of retinal
or vitreous detachment.
6. Black spots in front of the eyesSudden development of black spots, increase in
number and size of black spots in front of eyes require elaborate examination because
they may herald retinal detachment. Slowly developing black spots are not harmful.
7. Colour defect may be present in macular degeneration.
Signs of retinal diseases
The signs of retinal diseases are generally not visible with oblique illumination or routine slit
lamp bimicroscopy because the retina is beyond the focus of ordinary slit lamp. The optics of
the eye/slit lamp needs to be modified to bring the retina in focus of the slit lamp. Rarely the
condition is visible by oblique illumination if it is large enough or near enough the lens as
white reflex in the pupillary area.
The retina is best examined by
1. Direct ophthalmoscopy
2. Indirect ophthalmoscopy
3. Slit lamp biomicroscope with minus contact lens - Goldmann three mirror contact
lens
Non contact lens
(a) Minus lensHruby lens
(b) Plus lensVolk, El-Bayadi lens
4. Retinoscopy for error of refraction, retinoscopy shows coloboma, large exudate, edema
growth, detachment as grey reflex.
5. Fluorescein angiography
6. Ultrasonography, A Scan, B Scan
7. OCT (Optical Coherence Tomography)
8. Dark adaptometry
9. Photo stress test
10. Field examination
(a) Central 10
(b) Central 30
Peripheral (i) Kinetic
(ii) Static
11. Electro physiological tests
(a) Electro oculography (EOG)
(b) Electro retinography (ERG)
(c) Visual evoked response (VER)
12. X-ray skull, orbit
13. CT
14. MRI
15. Trans-illumination.
Principles of some of the methods used in examination of retina
Direct ophthalmoscope
This instrument uses the optics of the eye as simple magnifier of + 60D, the retina acts as
the object. The ophthalmoscope acts partly as source of light to illuminate the retina and
partly to focus the image of the retina. This gives a virtual image of the retina which is erect
and fifteen times magnified in emmetropia. The magnification is less when the diopteric
power of the eye is less i.e. aphakia or high hypermetropia where a small image is seen. In
contrast to this, the myopic eye gives a larger image than emmetropia. A raised lesion on the
retina or in front of the retinal plane requires addition of plus lenses in the viewing system of
the ophthalmoscope. Similarly minus lenses are required to see a spot behind the retinal plane
i.e. floor of optic cup in glaucoma or floor of posterior staphyloma. The direct ophthalmoscope
has a small field i.e. 10 at a time, which requires to be shifted frequently to see a peripheral
lesion, retina beyond equator is not visible, scleral indentation which makes peripheral lesion
visible by indirect ophthalmoscopy is not possible while using direct ophthalmoscope. The
illumination of direct ophthalmoscope is not very bright. The direct ophthalmoscope lacks
stereopsis. Its higher magnification is better suited for small lesions of macula and examination
of optic nerve head by incorporating a grid, the size of a lesion can be measured by direct
ophthalmoscope.
Indirect ophthalmoscope
The condensing lens of the indirect ophthalmoscope forms an inverted, real and magnified
image of the retina between the condensing lens and the observer. The indirect ophthalmo-
scope uses the principle of astronomical telescope where both the eye pieces and the object are
plus lenses. The indirect ophthalmoscope has a stereopsis and a large field, the condensing
lens commonly used are + 15D, + 20D and + 30D. Commonly used condensing lens is a plano
convex large + 20 lens which has anti reflection coating. More is the dioptre of the condensing
lens, lesser is the magnification and wider the field. A condensing lens of + 15 gives magnifica-
tion of 4x while that of + 20 and + 30 give magnification 3x and 2x respectively. Most commonly
used indirect ophthalmoscope is binocular indirect ophthalmoscope which has head held view-
ing system and a hand held condensing lens.
Biomicroscopy of the posterior segment (Slit lamp examination of the retina)
Slit lamp is a compound microscope with a limited focal length. It is a versatile instrument to
examine the anterior segment up to anterior few millimetres of the vitreous, structures beyond
this are out of focus. To see the objects deeper to this, it is essential to modify the optics of the
eye suitably. A normal emmetropic eye has a dioptric power of + 60, out of this about + 45D-
+ 47D is attributed to cornea and rest to the lens.
The optics can be modified either by
1. Neutralising the power of + 60D by a 60D lens kept away from the eye i.e. Hruby
lens which is placed 10 mm to 12 mm in front of the eye under examination. The
pupil should be well dilated. This forms a virtual image 18mm in front of the retina.
The biomicroscope is focussed on this, one of the disadvantages of Hruby lens is that
the size of the pupil is reduced by high minus glass, this hampers the field of exami-
nation.
2. Replacing the corneal power by an afocal lens in contact with the cornea as by
Goldmann three mirror gonioscope by which not only the posterior pole of retina,
but also peripheral retinal and angle of anterior chamber can be examined. This has
a disadvantage of being large and heavy. This is avoided by use of Koeppe lens which
is light but focuses only the posterior poles. It is good for examination of macula and
optic nerve head. The size of pupil is also reduced by these contact lenses and it
reduces the field to 20.
3. Using principle of indirect ophthalmoscopy with slit lamp. Plus lenses between
+ 60 to + 90 are held in front of the eye with a dilated pupil and image is seen through
the slit lamp. The image is real, inverted and of same size. The image is away from
the observers eye. Stronger the power of the lens lesser is the size of the image, with
pan fundic lens which is self illuminating, the magnification is 0.7x.
Fundus fluorescein angiography
This is one of the most important procedures in all retinal and choroidal disorders especially
involving the retinal vessels, the retinal pigment epithelium, chorio capillaries and
Bruchs membrane. Fluorescein angiography is less required in children than in adults where
diabetic retinopathy, retino phlebitis, central vein thrombosis are main indication. In children
it is mostly used in congenital anomalies of the retinal blood vessels, sickle cell retinopathy,
retrolental fibroplasia, haemangiomas.
Fluorescein is a dye that when exposed to light of short wave length gives fluorescence
and becomes visible. Intra vascular fluorescein is not visible in white light. To make it visible
it should be excited by blue light in the wave length of 490 nm. This excitement of fluorescein
then emits a yellow grey light in the wave length of 530 nm which can be recorded on a
fundus camera.
For this purpose, the fundus camera which is essential part of fundus fluorescein
angiography has two filters:
(1) A blue excitation filter and (2) Yellow grey barrier filter.
70-85% of fluorescein when injected in ante cubital vein gets bound to the serum protein
and is called bound fluorescein, remaining part is called free fluorescein.
Free and bound fluorescein differs from each other by virtue of their ability to pass
across the cells, RPE and Bruchs membrane.
Neither free nor bound fluorescein can pass through the walls of large choroidal vessels.
They also do not pass through tight junction of retinal capillary endothelium. A leak from
retinal capillary is abnormal.
Free fluorescein can pass through fenestrated walls of the chorio capillaries. It can also
pass across the Bruchs membrane. It can escape into extra vascular space as well. Free
fluorescein does not pass through tightly placed RPE.
Fluorescein injected in ante cubital vein reaches the systemic circulation. It reaches the
fundus by two routes:
1. Short posterior ciliary arteries. This reaches the choroid earlier than the central
retinal artery.
2. Central retinal artery. This delineates the retinal vessels. The dye drains by cen-
tral retinal veins.
The normal fundus fluorescein angiogram has following phases:
1. Pre arterial (Choroidal) phase. The whole of the choroid gives a bright glow un-
der six seconds. Brightness of the glow depends on amount of pigment present in the
RPE. A cilio retinal artery when present stands out in choroidal phases.
2. Arterial phase. It takes about 8 seconds for the retinal arteries to fill up. The supe-
rior and inferior branches fill early, then the rest of the retinal vascular pattern stand
out brightly.
3. Arterio venous phase. This indicates beginning of venous drainage. The dye is
visible in both arteries and veins. The retinal capillaries become visible.
4. Venous phase. It is divided into three distinct phase i.e. early, mid and late phase.
Early venous phase. The fluorescein stains the peripheral part of the venous blood.
This is attributed to the lower pressure in the veins. The arteries still show fluorescein
in them.
Mid venous phase. The lamellar appearance of the dye is no more visible. The whole
of the vein is uniformly filled with fluorescein. The arteries still retain some stain.
The capillaries become visible.
Late venous phase. Fluorescence in the vein is less than mid venous phase. This is
followed by an increased fluorescence of short period due to recirculation of the dye.
Then the dye fades away.
Appearance of normal macula in fluorescein angiogram
The macula is avascular, more so in foveal avascular zone. Presence of xanthophyll, tall and
more pigmented RPE masks the choroidal flush in early phase. Otherwise also a small area of
central macula remains hypo fluorescent. The peri foveal arcade is seen in the venous phase.
The optic disc. Fluorescence in the vessels of the optic nerve head show variations in
various phases due to difference in blood supply in different depth of the optic nerve. The
capillaries at the level of lamina are visible in choroidal phase. The capillaries on the surface of
the disc and peri papillary capillaries are seen after the choroidal flush. In late venous phase
there is staining of optic nerve head. Fundus fluorescein angiogram helps to differentiate be-
tween pseudo neuritis, neuritis and papilledema.
Abnormal fluorescence
The two types of abnormality of fluorescein areHyper fluorescence and hypo fluorescence.
Hyper fluorescence is a state of enhanced fluorescence, this can be localised or
generalised. The hyper fluorescent area stands out more prominently than the surrounding
area. The causes areAbnormality in the vessels, telangiectasis, aneurysms, shunts,
neovascularisation, chorio retinal scar, ARMD (not seen in children), myopic degeneration,
choroidal rupture.
New growthVarious hamartomas, angiomatosis retinea, retinoblastoma, malignant
melanoma (not seen in children).
Increased transmissionAlbinism (there is lack of pigment in RPE).
Window defectAtrophic RPE, angioid streak, drusen.
LeakPoolingSeen in late venous phaseRPE detachment, retinal detachment, cyst-
oid macular edema, central serous retinopathy.
StainingDrusen, soft exudate, scar.
Hypo fluorescenceThis is a state of fluorescence that is either less than normal
fluorescence or is totally absent.
It can be brought about either by masking of fluorescence or due to filling defect.
Masked (blocked) fluorescence can be due to pigment, exudate or serous fluid.
The pigments areMelanin, lipofuscin and xanthophyll.
Blood is a common cause of blocked fluorescence. It could be a haemorrhage in
the choroid, retina. Both hard and soft exudates block the fluorescence so do foreign bodies
and scar tissues, CSR, disciform degeneration and serous retinal detachment.
Filling defectVascular occlusion
Retinal artery obstruction, venous obstruction, choroidal infarct, healed scar of choroiditis,
coloboma of choroid.
Indocyanine green angiography
This is less commonly used method of angiography. It is used to see outline of choroidal circu-
lation because it delineates choroidal vessels better than fluorescein. Indocyanine green is a
tricarbocyanine compound containing free iodine.
31
It is totally bound to serum protein. It is
excreted in bile, peak fluorescence is at 835 nm. It is injected intravenous like fluorescein. The
fluorescence of indocyanine green is in the infra red range. The angiogram requires an excit-
ing filter at 835 nm. ICG has 25 percent less fluorescence. It requires infra red sensitive
video camera and video cassette recorder. It is mostly used to see choroidal neo
vascularisation. It is used alternatively in patient sensitive to fluorescein. As ICG is iodine
containing dye it is also likely to cause allergic reaction. Other use of indocyanine green is in
maculorrhexis.
32
Optical coherence tomography
33,72,73,74
This is an imaging technology that give high resolution image of retina and optic disc, with
modification it can be used to image cornea, lens and iris in diseases of anterior segment. The
principle is almost the same as in ultrasonography, CT or MRI. The only difference is that the
ultrasound uses sound, CT uses X-ray, MRI uses spin resonance, the OCT uses low coherent
near infra red light beam (820 nm - 830 nm) from a super luminescent diode. It is important
to have clear ocular media, the pupil should be atleast 4 mm in diameter. The results are
reproducible, the method is non invasive, non contact.
The system consist of an inter ferometer that gives high resolution (10 to 20 ) in
contrast to 100 of ultrasound. The light is delivered through a slit lamp with + 70D lens. It
requires an infra red sensitive video camera. OCT is used to detect disorders of retina, espe-
cially the macula. It can differentiate between full thickness and partial thickness macular
hole, the exact diameter of the hole and thickness of the retina surrounding the hole can be
assessed. It can be used to document the progress of the treatment
34
. It is useful in central
serous chorio retinopathy, macular edema, epiretinal membrane and age related macular de-
generation, macular cyst. OCT has proved to be a valuable imaging method in evaluation of
optic nerve changes in glaucoma.
Ultrasonography
35
This is yet another non invasive procedure of multiple uses in ophthalmology. It uses inaudi-
ble sound. Sound audible to human ears range between 20 to 20,000 hertz (Hz). Sound used for
ultrasonography is by far more than audible sound, it ranges in millions of cycles and noted as
MHz or megahertz. Sound does not have any radiation or magnetism. Ultrasound used for
ocular ultrasonography does not produce heat, it uses very low energy.
The ultrasound when directed towards the interior of the eye or orbit is reflected back
towards the source as it meets a changed density or elasticity in the media through which it
passes.
The ultrasound is generated in a transducer, which converts electric energy into sound
waves. The transducer contains piezo electric crystal. This not only produces echo but also
receives it back and converts it into electric potential that can be either photographed or printed
on paper.
There are two main modes of ocular ultrasonography(1) A scan, (2) B Scan. Each has
its own utility. Sometimes both the methods may be required to clinch the diagnosis.
The A scan is time amplitude that gives linear one dimensional picture.
The B scan is intensity modulated and gives two dimensional picture.
Interpretation of A mode requires more skill than B mode.
A scan is used to find out ocular dimension i.e. AC depth, thickness of lens, thickness of
the cornea. Length of the globe it is most widely used in calculating power of IOL. It has
limited value in diseases of globe or orbit. The tracing is shown as a series of spikes. Distance
between the two spikes is the distance between two acoustically different surfaces. Normal
aqueous, vitreous do not produce any spike but anterior and posterior surfaces of lens give two
spikes of almost same height with space in between. The space in between the two spikes is the
thickness of the lens. Other important use of A scan is measurement of corneal thickness -
Ultrasonic pachymetry (Pachometry).
Abnormal content of vitreous like blood, blood clot, foreign body, cysts give positive
spike. Retinal detachment, retinal tumours and choroidal growths also give positive spike
provided they are in the path of the echo.
B scan gives a two dimensional picture of the globe and orbit. Instead of spikes it gives
dots. Brightness of the dots depends upon size of the echoes reflected. The resulting picture
though not very exact but is comparable to histopathological cross section. Different types of
lesions have different shadows, some have specific appearance. B scan is mostly used to see
vitreo retinal pathology in opaque media or hazy media where indirect ophthalmoscopy is not
possible. It delineates opacities in vitreous. It differentiates between traction detachment,
exudative detachment, celio choroidal detachment and rhegmatogenous. It also outlines intra
ocular tumours, IOFB, endophthalmitis, cysts and parasites, position of dislocated lens. It is
more useful in orbital disease than A scan. It is used in thyroid myopathy, orbital pseudo
tumours, tumours of the optic nerve.
One of the advantages of ultrasonography is that it can be used as many times as possi-
ble in all ages in sitting or recumbent position with out any radiation hazard.
Dark adoptometery
36,37
This investigation is performed less frequently, it measures the difference between recovery of
rod and cone functions after they have been exposed to bright light. The cones recover faster
than the rods. The cones have no vision in dark. They are photopic while rods function only in
dark, they are scotopic. It is common experience that when some one walks in a dark area
after being in the bright light for some time, it takes few seconds to start seeing in the dark.
Similarly a person who has been in dark for some time, when he goes out in bright light takes
few seconds to get adapted to the bright light.
The instrument is called dark adaptometer that is used in cases of defective night
vision due to diseases of retina and choroid. It is used mostly in rod disorders and has less
utility in cone disorders.
Light sensitivity of the eye has three grades:
1. Photopic Bright light, normal room light or outdoor, this is cone function.
2. Mesopic Dim room light or twilight, this is partly rod and partly cone function.
3. Scotopic This is in a room without light or dark night outside, this is rod function.
Commonly used dark adaptometer is Goldmann Weekers adaptometer. Besides this
there are other adaptometers likeFeldman adaptometer, Henkind and Seigel scotometer,
Combergs nyctometer.
To perform the test both the pupil are fully dilated. The patient is made to sit in a semi-
dark room to get dark-adapted for few minutes. Then the eyes are exposed to bright light
similar to pre-adaptation light for six to eight minutes. Dark adaptation is started. Retinal
sensitivity is tested for first ten minutes at an interval of ten seconds then every one minute,
first in ascending order i.e. the light sensitivity is gradually increased until the patient becomes
aware of it, then the illumination is gradually reduced until the patient can not see the light.
The average of the two is plotted against time. Illumination is plotted vertically and time
horizontally. The normal dark adaptation graph is biphasic. It comprises of two concave curves
joined to each other. The first part is short and steep, lasts for initial ten minutes and denotes
cone threshold while the remaining a longer and shallower curve denotes rod threshold and
lasts for about 30 minutes. The junction of the two is called rod-cone-break.
38
The rod threshold
is prolonged in retinitis pigmentosa, vitamin A deficiency, retinitis albi punctatus.
39
Electro retinography
38,40
This is an objective method to measure retinal function. It is used to detect function of both
rods and cones, was mostly used for peripheral retinal disorders. With development of better
computerised methods it is now being used more frequently to assess macular function as
well especially in colour vision defects
41
It is relatively crude method where all the layers of
the retina participate. However involvement of rods, cones, bipolar cells and Mullers
cell are reflected better than remaining layers. This is brought about due to transmission of
ions across the retina.
The test is done by a computerised oscilloscope that gives a visual picture on a
screen which may be obtained as print out (electro retinogram) for permanent record. The
instrument has two electrodes, one is a modified contact lens that is applied to cornea and
the other electrode is applied on the forehead.
ERG can be photopic for cones, scotopic for rods. It can be for colour. Mostly used
colours are red (long wave) or blue (short wave). A mesopic response can be seen on the ascend-
ing arm of the b wave.
The electro retinogram is a continuous tracing along a straight line. The spike below the
base line is called negative while those above are known as positive waves.
The various components of ERG are:
1. Early receptor potential (ERP)
2. a wave
3. b wave
3. c wave
4. d wave
Out of these only a and b waves have clinical significance.
The a wave is a small negative wave, due to hyper polarisation of rods and cones.
The b wave is the largest wave both in amplitude and duration. It is a positive wave,
it is due to elevation of extra cellular potassium.
42
The waves are measured in amplitude and time. The amplitude is measured in
microvolts and time is measured in milliseconds.
Method
The pupil is fully dilated. The eye is adapted, then flash stimulus are given and the electro
retinogram is obtained.
Uses of ERG are many. They are all diagnostic and have some prognostic value.
1. Diagnose retinal degeneration and dystrophies.
2. They are utilised to differentiate between disorders of rods from cones.
3. They are used to detect possibility of presence of retinal degeneration in the asympto-
matic members of the family.
4. They can be used to detect malingers.
5. They may be used to assess retinal function in presence of opaque media.
The common conditions where ERG is useful in conforming the diagnosis are:
Retinitis pigmentosa and allied retinal degeneration
Lebers congenital amaurosis
Achromatopsia
Congenital stationary night blindness
Oguchis disease
Choroideremia
Gyrate atrophy of choroid
Quinine amblyopia.
It has no role in ganglion cell disorder, hence it is not used in glaucoma and diseases of
optic nerve.
Rubella retinitis gives fundus picture similar to retinitis pigmentosa but have normal
ERG.
The female carriers in X linked retinitis pigmentosa may show abnormal b wave in
absence of night blindness.
The ERG may be extinguished in advanced retinitis pigmentosa and allied conditions,
total retinal detachment, diffuse unilateral sub acute neuro retinitis, ophthalmic artery occlu-
sion.
Electro oculograph
42
Electro oculograph has relatively less use than ERG in evaluating the retinal functions because
in most of the cases that have, either absent or grossly subnormal ERG, also have subnormal
EOG except in few cases i.e. stationary night blindness, X linked retino chisis,
dominantly inherited Bests vitelli form, macular dystrophy.
38
Electro oculography like ERG is an objective method to measure the retinal function
like ERG. It also measures mass response of retina. It basically measures difference in cor-
nea and retinal potential on horizontal movement of the eye. The corneal potential is positive
while the retinal (back of the eye) is negative. The difference between the two is 6 mv. The
difference depends upon excursion of the eye, level of illumination and adaptation.
It is also useful in some conditions of subtle extra ocular muscle palsy with modifica-
tion. It can also be used to record nystagmus, where the instrument is called electro
nystagmograph.
The instrument is an oscilloscope. It has two electrodes, one is attached near the
medial canthus and the other near the lateral canthus. The former is corneal electrode and
is positive while the latter is retinal electrode and is negative.
For retinal function test, it indirectly records the standing potential of the eye, which is
caused due to voltage difference between inner and outer retina. It evaluates retinal pigment
epithelium mostly.
To get the reading the pupil is dilated. First, the patient is dark adapted. The electrodes
are placed one on the medial canthus and the other on the lateral canthus. The patient is
asked to move the eye alternately, medially and laterally with approximate 60 excursion
between the two. The oscilloscope is activated and the spikes noted. After dark adaptation the
response progressively decreases reaching the trough in 8-12 minutes, which is lower than the
base line. The light adapted trough is well above the base line and is reached in 6-9 minutes.
The whole process requires about 20 minutes for each eye. To obtain the graph, the amplitude
in microvolts is plotted against time. The final reading is a ratio between the light peak and
dark trough called Arden ratio i.e. Maximum height of light/depth of the dark trough. The
normal is 185 or more. Reading between 125 and 150 is subnormal and less than 125 are flat.
The response is independent of opacity in media. Myopia, trauma and ocular surgery give low
response.
Visual evoked response (VER)
This is an objective test to assess function of visual path beyond the retinal ganglion cells.
It is commonly used to access macular as well as optic nerve function in pre geniculate
path. It has some use in amblyopia and colour blindness. It is basically modified form of electro-
encephalogram. It records electric activity of the visual cortex area 17,18 to light stimulus.
The VER records primarily the central visual function
43
. Opacities in the media do not
influence the response.
The VER is of two types:
1. Flash VER
2. Pattern reversal VER
A typical pattern consists of a latency of 75-80 ms and a positive wave of 110 ms. The
positive wave has a peak of 9 ms.
Method of recording
The patient sits in a quiet dark room for sometime in front of a screen at a distance of 1.5
metres. One eye is tested at a time. The potential is recorded by an electrode placed over the
occiput. The other electrode is put on the vortex. Hundreds of responses are summated and
averaged by a specially programmed computer.
In case of flash VER, a flash of light is used to start the response. In case of pattern
reversal VER, a chequer board with alternate black and white squares of equal size and
intensity are displayed on the screen. The colour of the squares can be reversed i.e. the black
becomes white and white becomes black. The flash VER is used to test the function of the
macula and visual pathway in non verbal children, mentally retarded children and
aphasic children.
44
It can detect malinger and visual potential in opaque media especially
in large corneal opacity where the pupillary reaction can not be elicited.
The form reversal VER uses form sensation hence this gives a rough idea of visual
acuity. VER is useful in optic neuritis due to demyelinating disease where VER may be absent
in acute stages. VER is abnormal in multiple sclerosis even before diminished vision sets in. It
is useful in compression lesions of optic nerve. In cortical blindness the response is absent.
45,46
Photo stress test
This is a crude form of dark adaptation test. The visual pigments are rendered insensitive to
ordinary light causing a temporary relative scotoma which passes in some time with fading of
scotoma. The recovery time depends upon ability of the photo receptors to re-synthesise visual
pigment. This is used for macular lesion and has hardly any use in peripheral disorders. This
differentiates macular cause of loss of vision from optic nerve cause.
To perform the test, the distant vision is recorded with best correction. Then the eye is
dazzled by the light of indirect ophthalmoscope kept at about 3 cm from the eye and the patient
is asked to read the best corrected line. Normally it takes about 30 seconds to recover. In a case
of macular disease, this is prolonged over 1 minute. Visual loss due to optic nerve lesion is not
effected by photo stress.
Interpretation of physical examination of retina (Signs of retinal disorders)
The signs of retinal disorders are many and varied. They depend on part of the retina involved
and associated involvement of vitreous, choroid and optic nerve.
The signs may be limited to retina only where it can be central (macular), peripheral or
combined. The signs can be secondary to choroid as chorio retinitis. This is more common than
reverse i.e. retino choroiditis that is pathology spreading from retina to choroid. However
neuro retinitis is as common as inflammation spreading from retina to optic nerve. Tumours of
optic nerve do not metastasise in retina. But spread of retinoblastoma through optic nerve is
common.
Duration of the disease too plays an important role in appearance of the signs.
The common signs are:
Signs of retinal disorders are essentially situated on the fundus which is visible either
by ophthalmoscope or by slit lamp.
They are changes in the blood vessels, they include1. Attenuation, 2. Dilatation,
3. Tortuasity, 4. Anastomosis.
Attenuation. Normal arteries are thinner than veins but in some pathological cases
the arteries become thinner than normal or may look to be so if the veins are dilated. The
thinning can be generalised or localised. Generalised thinning is more common than segmented
attenuation. The most common causes of generalised thinning of the arteries is retinitis
pigmentosa and allied conditions. Other causes of attenuation like arterio sclerosis and
hypertension is not seen in children. However retinal artery occlusion may occasionally seen
in children. Commonest cause being embolism from the cardiac valves in sub acute bacterial
endocarditis. In post papilledematous and consecutive optic atrophy, there is thinning of the
arteries.
Dilatation of arteries is far less common, it is seen in leukaemia and in angiomatosis
retinae or cirsoid aneurysm of the retina. Dilatation of the veins is more common. It is seen in
cases of papilledema and venous obstruction. In angiomatosis retinae the vein may be greatly
dilated.
Tortuosities of both arteries are uncommon in children. They are mostly seen as
congenital anomalies like angiomatosis, Coats disease, telangiectasis.
Anastomosis. Anastomosis between two systems of circulation i.e. retinal and ciliary
and between artery and veins are always pathological.
The effect of abnormal vascularity leads to:
1. Hyperemia of the retina
2. Anaemia of the retina
3. Edema of the retina
4. Exudates on the retina
5. Haemorrhages on the retina
6. Neovascularisation.
Hyperaemia of the retina may be arterial or venous. Arterial or active hyperaemia is
seen in dilatation of the arteries and increased tortuosity of the retina. It can be generalised or
localised. Common causes are retinitis and uveitis.
Venous or passive congestion is caused due to impeded venous outflow. Common
causes being papilledema and papillitis. Other causes are systemic condition like leukae-
mia, polycythemia and chronic heart failure. Children in teens may have periphlebitis.
Other causes are sickle cell retinopathy and cavernous sinus thrombosis. Diabetes and
hypertensive retinopathy rarely cause hyperaemia in children.
Anemia
Commonest cause of pallor of retina is systemic anemia. Not only the general appearance of
the retina is pale but the optic nerve also looks pale. Anemic fundus is common in leukemia
and lipidosis also. Pallor can be true pallor due to diminished blood supply or may look pale
with normal blood supply Arterial obstruction is the commonest cause of pallor. It may be
generalised when the main trunk of the central retinal artery had been obstructed or segmen-
tal when one of the branches has been obstructed. Other causes of generalised pallor of retina
with normal blood supply and normal haemoglobin are albinism, myopia, Waardenburgs
syndrome, choroideremia.
Causes of localised pallor are- Retinal edema, coloboma of retina, retino schisis, flat
retinoblastoma, atrophic areas.
Retinal edema
The main pathology in retinal edema is break down of blood retinal barrier, either the
inner barrier or outer barrier. This is brought about by any of the following two or
combination of the two1. Excessive leak of fluid from capillaries due to increased permeability
as is seen in inflamation or vascular retinopathy. 2 Break down of larger protein molecules.
Break down of the inner blood retinal barrier leads to localised edema as cystoid
macular edema or post traumatic edema. Diffuse retinal edema is seen in vascular
retinopathies. Break down of outer blood retinal barriers lead to central serous retinopathy
and wet type of age related macular degeneration. Retinal edema has greater predilection for
posterior pole mostly macula.
The common causes of retinal edema in children areblunt injury, optic neuritis,
papilledema, neuro retinitis, retinitis, chorio retinitis, retinopathies, cystoid macular edema.
Edema of the macula is associated with diminished central vision, there may be acquired
hypermetropia, central scotoma, metamorphopsia. Peripheral retinal edema may be
symptomless or may be associated with localised scotoma.
It is difficult to detect mild retinal edema with ophthalmoscope. In moderate edema the
retinal surface has an albuminous appearance, the retina looks thickened and has granular
appearance. Macular edema generally presents with a circular raised area. A macular edema
is said to be clinically significant macular edema if it has following features-
1. Retinal edema is within 0.5 mm of fovea centralis
2. Retinal edema is larger than 1 disc diameter within one disc diameter of fovea.
3. Hard exudates within 0.5 mm of fovea associated edema of the retina near by.
Presence of any one of the above is diagnostic
47
Clinically significant macular edema is best elicited by + 90 D on slit lamp biomicroscopy.
Separation of nerve fibres due to accumulation of fluid near the disc is visible with red free
light of ophthalmoscope.
Exudates on the retina
Presence of retinal exudate is always pathological. There are conditions that look like
exudates but are not exactly exudates. Common among them are- Small opaque nerve fi-
bres and drusen of the retina.
Clinically exudates have been classified into two types on the basis of their
ophthalmoscopic appearance. They are:
1. Soft exudates
2. Hard exudates
Soft exudates. They are due to disturbance of axoplasmic flow that result in
accumulation of axoplasmic waste product in the nerve fiber layer, hence they are called
superficial exudates. Their cotton wool appearance imparts the term soft exudate to
them. They are basically vascular in nature. There is sudden focal obliteration of capillaries in
small areas resulting in ischemia followed by anoxia. The infracted area leads to proliferation
of neural element in the retina. The soft exudates are white in colour, their edges are
irregular or feathery, they are scattered, do not coalesce to form specific pattern, are acute
in onset, disappear in two to three month. On fluorescein angiography, they are non fluorescent,
surrounded by area of dilated capillaries. Common causes are retinitis, hypertensive
retinopathy, severe anemia, dysproteinemia, collagen diseases, angiomatosis,
telangiectasis. leukaemia, trauma, papillitis, papilledema, septicimia, rheumatic
heart disease, sub acute bacterial endocarditis.
Hard exudates. Though they are seen more commonly in vascular retinopathy, there
are more of exudative nature than vascular. To begin with there is neural degeneration due to
defective oxidative process. The products of degeneration are generally lipid in nature, may
be associated albumin, cholesterol, hyaline material or even calcium. They are situated
in deeper layer of the retina hence are known as deep exudate. They are round in shape with
clear-cut edges, size may vary from pin head to as large as disc, generally seen in posterior
pole and are always underneath the blood vessels. Lipid content is most probably the causes of
their yellowish grey colour. They are responsible for formation of macular star and arranged
in a wreath like pattern in non specific circinate retinopathy. They develop over months
and take months to disappear.
Common causes of hard exudate areDiabetic retinopathy, late stages of hyper-
tensive retinopathy, Doynes choroiditis, disciform degeneration of macula, Coats disease.
Both the types of exudates may exist in the same eye at the same time and one may be
superimposed on the other. Both the types are non-specific, may be present without
haemorrhages. However presence of haemorrhage along with exudate is very common.
Retinal haemorrhage
Retina is supplied by an elaborate network of blood vessels with large capillary bed. Hence
bleeding from them is very common.
The causes of retinal haemorrhages are many. Sometimes more than one causative
factor may be responsible.
Causes of retinal hemorrhages can be broadly divided into:
1. Traumatic. Closed globe and open globe.
It is common for retinal vessels to be torn in traction retinal detachment, however
there may be tear in the blood vessels in rhegmatogenous retinal detachment as well.
2. Inflammation. More common cause is periphlebitis, less common is periarteritis.
3. Retinopathy. Perhaps largest numbers of retinal haemorrhages are seen in various
types of retinopathies, fortunately many of them are not seen in children. Common
retinopathies areSickle cell retinopathy, malignant hypertension and
nephritis.
4. Blood disorders. Anaemia, leukemia, purpura, haemophilia, sickle cell anaemia
are common in children.
5. Miscellaneous. Papilledema, papillitis, telangiectasis, hamartomas, Coats disease,
retinoblastoma
6. There are two conditions that are not true hemorrhages but look like hemorrhage:
(a) Cherry red spot
(b) Macular hole
Causes of cherry red spot are:
1. Various errors of metabolism and storage diseases - Tay Sachs disease, Niemann-
pick disease, Farbers disease, Sandhofts disease, Hurlers disease, mucopolysaccha-
ridosis.
2. Central retinal artery occlusion (rare in children).
Types of retinal haemorrhages
Depending up on anatomical location of the hemorrhage in relation to the retina they have
been divided into following groups:
1. Inter retinal (a) Superficial
(b) Deep
2. Pre retinal (a) Sub hyaloid haemorrhage
(b) Vitreous haemorrhage
3. Sub retinal (a) Choroidal hematoma
(b) Sub retinal neovascularisation.
Inter retinal haemorrhages
There are mostly capillary in nature but occasionally can be arterial or venous. They are called
superficial when they are in the nerve fiber layer. Superficial haemorrhages are bright
red in colour, are typically flame shaped or leaf shaped with irregular border. They
are deeper to the retinal vessels and are known to fade over weeks to months without treat-
ment. Their number and size may vary from single to multiple, from small spots hardly visible
to larger than manydisc diameter. They may be localised or scattered. Peripheral hemorrhages
do not cause visual disturbance but a small hemorrhage on the fovea causes extensive visual
loss. The hemorrhage themselves are non fluorescent and block fluorescein. Common causes
are hypertension, papillitis, papilledema, retinitis and trauma.
Roths spots are peculiar superficial haemorrhages, which are flame shaped haemor-
rhages with central white area commonly seen in sub acute, bacterial endocarditis, anaemia
and purpura.
Deep haemorrhages
Haemorrhages in deeper layer are small, circular. Their colour is less brighter than superficial
haemorrhages. Compactness of the deeper layers of the retina prevent them to spread, hence
they are localised and small dot shaped. The retinal vessels pass over them, commonly found
in the posterior pole, may be scattered or may be in bunches mimicking retinal micro aneurysm
from which they are differentiated by fluorescein angiography. The deep haemorrhages are
not fluorescent and block the fluorescein. The micro aneurysms are fluorescent. Commonest
cause of deep haemorrhages is diabetic retinopathy. It is not uncommon to see mixture
of both superficial and deep haemorrhages in the same eye in case of diabetic retinopathy.
Sub hyaloid haemorrhage
These are also known as pre retinal haemorrhages. They occur between the internal limiting
membrane of the retina and vitreous without invading into the substance of the vitreous. The
source of the bleeding is retinal vasculature. They are relatively rare but not altogether
absent in children. They may be seen even in new born.
The common causes in children areBlunt trauma, retinal tear with or without
detachment, Purtschers retinopathy, penetrating injury, accidental perforation of the globe
during squint surgery.
Children in second decade may develop pre retinal haemorrhage in retro lental
fibroplasia, persistent hyaloid artery, Coats disease, angiomatosisretinae, juvenile
retino schisis, and drusen of optic nerve.
Periphlebitis retinae (Eales disease) is commonest cause of pre retinal and vitreous
haemorrhage in young male adults. Commonly seen in third and fourth decade. Eales disease
may have an early onset, may be seen as early as fifteen to sixteen years. Similarly diabetic
retinopathy is commonest cause of pre retinal and vitreous haemorrhage after fourth decade.
In rare instances untreated juvenile diabetes may present as retinal or pre-retinal haem-
orrhage.
Characteristics of pre-retinal (sub hyaloid) haemorrhages
Symptoms and signs depend upon position of the haemorrhage, size of the haemorrhage and
associated lesions.
Symptoms
It may be symptomless if the haemorrhage is in the lower part of the retina, away from the
macula. Peripheral haemorrhage may go unnoticed.
A lesion in front of the macula is cause of sudden painless loss of vision. In very large
haemorrhages vision may be reduced to hand movement with good projection. A patient with
fresh haemorrhage may complain of red vision (erythropsia) which passes off in few days.
Generally vision improves with passage of time and may return to normal as blood absorbs.
The patient may complain of black spots, after the vision has come to normal.
A typical sub hyaloid haemorrhage if seen within few hours of onset, starts as a circular
patch of bright red spot generally in the posterior pole. Gradually the heavy particles of blood
settle down at the bottom of the original oval patch. The upper border becomes straight and
parallel to the ground and retains its bright red colour. The upper part contains serum hence
is either clear or yellowish in colour. The sub hyaloid haemorrhage has no tendency to clot. In
case of Tersons syndrome, a sub-arachnoid haemorrhage may find its way on the retina
along the optic nerve and may look flame shaped and rarely keel shaped which is a common
appearance of pre macular haemorrhage.
All the cases with sub hyaloid haemorrhage should routinely be examined under
mydriasis with indirect ophthalmoscope. The advantages of examination by indirect oph-
thalmoscope are that it can circumviate a large haemorrhage, and makes examination of the
retina possible inspite of its presence. It gives excellent information for peripheral-lesions
especially in periphlebitis. It also gives information about the haemorrhage in three dimen-
sion.
Vitreous haemorrhage
So long the outer membrane of the vitreous is intact, it is not affected by haemorrhage. Only
when new blood vessels grow in the vitreous, a haemorrhage develops. A small haemorrhage
initially behaves like a pre retinal haemorrhage. The causes of vitreous haemorrhages are the
same as pre retinal haemorrhage. Generally the haemorrhages absorb without any treatment
within days to weeks. However if bleeding occurs frequently, the clearance time is proportionally
prolonged and a stage may come when the haemorrhage does not clear and a stage of
hemophthalmos develops, which may require vitrectomy followed by endo laser photo
coagulation.
Fate of retinal haemorrhages
Most of the fresh and small haemorrhages absorb without leaving any trace. This happens
following hemolysis of RBC when there is no repeat bleeding and the haemorrhage is small. In
case of multiple haemorrhages, larger the size, longer is the time taken for absorption. This is
generally followed by proliferation of glial tissue called retinitis proliferance, which are
vaso proliferative in nature. The proliferation can be from the superficial layer of the retina or
the retinal blood vessel. The bands develop more commonly near the disc and extend into the
vitreous body. They may be only a few or very extensive. Fine capillaries develop along or on
the proliferative bands. As these vessels are unsupported, they bleed more commonly than
other retinal blood vessels. The conditions that cause retinitis proliference areTrauma
mostly penetrating, retained intra ocular foreign bodies, retro lental fibroplasia, angiomatosis
retinae, Coats disease, sickle cell retinopathy, Eales disease. In contrast to this common causes
in adults areEales disease, proliferative diabetic retinopathy and venous occlusion.
Sub retinal haemorrhage
They are not true retinal haemorrhages, they are in fact choroidal in nature. The bleeding
occurs between the pigment epithelium of the retina and the choroid. They are less frequent in
children than in adults.
Frequent causes of sub retinal haemorrhage are - Blunt trauma, choroidal rupture,
myopia, drusen of optic nerve head, chorio retinitis and rubella retinopathy.
The sub retinal haemorrhage looks like blackish oval raised area over which retinal
vessels pass. The lesion is better visualised by indirect ophthalmoscope and biomicroscope,
confirmed by fluorescein angiography and ultrasonography. The basic pathology is formation
of new vessels under the retina.
Retinal aneurysm
They are very common in adult diabetics, occasionally can be seen in juvenile diabetic. In
adults, they are small hence are called micro aneurysms. In children they are relatively larger.
On fundus examination they look similar to bunch of deep haemorrhages. They are best
demonstrated by fluorescein angiography. The causes of retinal aneurysm in children are Coats
disease and early onset peri phlebitis.
Anastomosis of retinal vessels
The retinal arteries are end arteries, they do not anastomose normally except as congenital
anomaly between central retinal artery and cilio retinal artery. There may be anastomosis
between veins as in angiomatosis. There may be anastomosis between the retinal circulation
and choroidal circulation in toxoplasmic chorio retinitis.
48
Inflammation of retina
Inflammation localised to retina is less common than retino choroiditis and neuro retinitis.
Associated vasculitis is common. Retinitis can be localised or diffuse. It may involve sensory
retina or retinal pigment epithelium separately or in combination.
Retinitis has been divided into two basic types:
1. Primary, where retina and retinal vessels are involved as primary site.
2. Secondary, when inflammation spreads from choroid mostly and optic nerve less
frequently.
It can be non specific or specific like viral, bacterial, fungal or parasitic when
associated with systemic diseases it can be bilateral, otherwise in case of unilateral retinitis,
the other eye may get involved later. Retinitis in children is mostly endogenous. It can be
purulent, exudative or granulomatous. Purulent retinitis can be acute or sub acute. Acute
purulent retinitis is mostly due to lodging of pus producing bacteria or fungus in septicimic
child. Generally the bacteria reaches the retinal vasculature from distant organ and cause
purulent retinitis which may pass into endophthalmitis or even panophthalmitis within days
or weeks. The condition starts in an acutely ill, malnourished child, as sudden gross loss of
vision without pain or congestion. On examination the retina is oedematous with dilated
veins. There may be superficial exudate and haemorrhages. If the disease process
progresses, it becomes painful and congested, with still further loss of vision going into
endophthalmitis and loss of eye. The condition is generally unilateral but may be bilateral.
Management is early recognisation of ocular problem and treatment of systemic septicimia.
Sub acute infective retinitis (Retinitis of Roth)
This is milder form of embolic retinitis where an embolus from heart may get lodge in the
capillaries of the retina in the posterior pole. Commonest condition leading to sub acute retinitis
is sub acute bacterial endocarditis. The lesion may be single or multiple. There may be
recurrence of the episode. There is generally very low grade of retinal reaction. The typical
lesion is a large superficial haemorrhage with white centre. A lesion near the macula draws
attention early. Most of the time the systemic condition overshadows the ocular pathology.
Treatment is directed to the systemic condition. Other causes of Roths spot should be excluded.
Specific retinitis of clinical importance in children are:
1. Viral
2. Bacterial Non granulomatous
Granulomatous
3. Parasitic
Viral retinitis in children
Many of the viruses with systemic involvement may cause primary retinitis. Viral infection of
choroid is invariably associated both DNA group of viruses i.e. herpes viruses which produce
chorio retinitis. RNA viruses i.e. rubella, rubeola (measles) cause retinitis. Healthy adults
rarely develop viral retinitis. It is common in neonates as part of intra uterine infection, the
two examples are rubella and cytomegalo viruses. The viruses are inter cellular, obligatory
parasites that cause necrosis and death of the cell leading to permanent damage to the retina.
Vasculitis is common in retinitis, the inflammatory response to virus is immune mediated.
Retinal hypoxia is frequent. It is due to occlusion of retinal vessels. The patches of the retinitis
may be localised or scattered. The after effect may be simple pigmentary disturbance without
visual loss to devastating visual loss due to exudates, haemorrhages, gliosis, retinal
detachment, secondary glaucoma and cataract. Optic neuritis is more common in lesions
of the posterior pole. It is difficult to diagnose the exact virus responsible for retinitis by
ophthalmoscopy because most of them have similar features of retinal edema, superficial
exudates and haemorrhages, cells in the vitreous. Specific diagnosis is possible only with positive
viral culture and serology.
49,50
Common viruses that cause retinitis in children are:
Rubella, herpes simplex, herpers zoster, cytomegalo virus, Epstein-Barr virus.
Rubella retinitis is a congenital bilateral retinitis, also known as embryonic
pigmentary retinopathy as the predominant feature is pigmentary disturbance of retina
without vascular changes or night blindness. The condition is seen in 50% of children born of
mothers who have contracted rubella in first trimester of pregnancy. In absence of more
serious ocular involvement like rubella cataract and rubella glaucoma, the condition may
go unnoticed unless looked for specifically. The condition does not involve macula, hence there
is no diminished vision. However as a late complication there may be sub retinal
neovascularisation. As there is loss of pigment from the RPE, fluorescein angiography shows
area of hyper fluorescence. There is no treatment for rubella retinitis. The best management is
prevention of rubella in mother that is achieved by immunising every girl child by rubella
vaccine in childhood. Equally important is to differentiate the fundus picture from other
embryonic retinopathies like congenital syphilis, varicella, influenza and radiation to
the mother in first trimester by doing torch test and other serological tests for rubella.
Commonest retinal dystrophy that presents with pigmentary changes is retinitis pigmentosa
and its allied conditions. Retinitis pigmentosa is always associated with night blindness, have
attenuated blood vessel and coarse spider like pigments, mostly in mid periphery. The pigments
in rubella retinopathy have salt and pepper mottling.
Rubeola retinitis (Measles retinitis)
Involvement of retina is rarer than involvement of cornea and conjunctiva. Retina is involved
when the child is serious enough to develop subacute sclerosing panencephalitis which
itself is a devastating neurological disease. The ocular involvement consist of bilateral
necrotising retinitis involving macula. There may be evidence of optic neuritis with superficial
haemorrhages and exudates which is healed by formation of chorio retinal scar.
There is no separate treatment for ocular manifestation. The child should be under
treatment of pediatrician. The best management consist of immunisation of children routinely
by measles vaccine.
Herpes simplex retinitis
It is seen in neonates who suffer from systemic herpes simplex infection that may include
herpes simplex encephalitis. It may develop in children who are on systemic immuno
suppressive drugs. Fundus picture consists of superficial exudates, haemorrhages, sheathing
of vessels with vitritis. Rarely there may be exudative retinal detachment.
Management consists of treatment of systemic herpes simplex.
Herpes zoster retinitis
As a general rule, herpes zoster infection is rarer in children as compared to adults. Retinitis
is still infrequent. It mostly involves posterior pole. It develops after cutaneous lesions have
subsided. The intra ocular lesions are immune mediated. There is localised necrosis of retina,
vasculitis is common.
Treatment consists of systemic antiviral drugs along with judicious use of steroids.
Cytomegalo virus retinitis (C.M.V.)
Incidence of cytomegalo virus has shown a sharp upward swing in past one decade due to
increased awareness among the ophthalmologists and increase in number of susceptible persons.
There are two modes of contracting the disease; the first is transplacental spread from the
mother and second is seen in immuno- compromised children. More than 80% of general
population show antibodies to the virus without manifest signs or symptoms. The incidence of
retinal infection rises with fall in immuno compatibility in children by way of spread of HIV
from mother to the foetus. Use of infected needles, transfer of infected body fluid, repeated
blood transfusion by unsafe blood transfusion, prolonged chemotherapy for malignancy and
immuno suppressive drugs for organ transplant increase the risk.
Congenital cytomegalo virus retinitis presents as localised necrotising retino
choroiditis any where on the retina, common site being macula and retinal periphery that
may lead to optic atrophy. On some occasion there may be pan retinal involvement with
acute necrosis. Differential diagnosis consists of toxoplasmosis, and nematode infection.
The acquired disease is common among children with AIDS, The virus is a member of
herpes virus family. It reaches the retina by hematogenous spread. Onset on CMV is CD4 plus
cell dependent. It never occurs in AIDS patient who have CD4+ count more than 50/mm
3
. It
begins as unilateral disease to become bilateral soon. The condition is uniformly vision threat-
ening when not treated. Retinitis does not require separate treatment, usual treatment by
specific triple anti viral drugs for AIDs.
Bacterial retinitis
Bacterial retinitis could be acute non granulomatous due to spread of pyogenic organ-
ism from systemic focus of infection or may be exogenous due to penetrating injury, most of the
time leading to endophthalmitis.
Incidence of granulomatous retinitis has been reduced in recent years due to effective
chemotherapy of tuberculosis, syphilis, which used to be two main causes.
Acquired syphilitic retinitis rarely occurs in children. More common is congenital syphi-
litic retinitis in children that causes embryonic pigmentary retinopathy with typical pepper
salt appearance without night blindness.
Treatment consists of treatment of systemic syphilis.
Tuberculosis produces tubercular chorio retinitis.
More common granulomatous retinitis are due to toxoplasmosis and toxocara infesta-
tion.
Toxoplasmal retinitis
Toxoplasmosis retinitis is perhaps the most common causes of retino choroiditis involving
macula in children. There are two modes to acquire toxoplasmosis. The commonest is
transplacental transfer of the parasite from the infected mother to the foetus. The second
less common is acquired-form seen in adults.
In between the two is a condition where old lesions are reactivated and present as active
lesion little away from the original macular lesion, and called satellite lesion.
Systemic acquired involvement is mild, which may be passed off as usual cold unless the
patient is pregnant or immuno-suppressed. In an infected pregnant woman there are 40%
chances that the foetus will be involved and the child will have multi systemic involvement.
The foetus is only at risk if the mother is infected during pregnancy. Subsequent pregnancies
are safe from toxoplasmosis.
The three clinical features of congenital toxoplasmosis are chorio retinitis,
convulsion and intracranial calcification. 80% of infants with congenital toxoplasmosis
will have retinal involvement.
The parasite has predilection for the nerve fibre layers of the retina. It is bilateral in
80- 85% of cases. Commonest site in retina to be involved is macula, which is generally missed
unless looked for or the child has other systemic signs of toxoplasmosis like convulsion,
hepatespleenomegaly. All children with convulsion and hepatospleenomegaly should have their
fundus examined for evidence of toxoplasmal chorio retinitis.
The condition is generally diagnosed late when the child presents with esotropia. By
that time the lesion is a healed scar, that may be many times larger than optic disc, with chorio
retinal scar on the periphery and white centre.
The condition should be differentiated from congenital coloboma of the macula. At
this stage there is no effective treatment available. However the child should be under regular
observation for years, as there is a possibility of reactivation of the lesion.
The reactivated lesions may be single or multiple, always behind the equator. It is asso-
ciated with anterior chamber reaction, vitreous precipitates, vitreous detachment and papillitis.
Diagnosis
There are many tests available to diagnose toxoplasmosis, common among them are serological
tests to detect anti toxoplasmo anti bodies, Hemagglutination test, indirect fluorescent
anti body test, ELISA test. Titers of these tests are lower in ocular lesions. Any positive titer
is significant with characteristic fundus picture. x-ray skull is associated with intra cranial
calcification.
Differential diagnosis of congenital toxoplasmosis in congenital coloboma of macula. Commonest
presentation of acquired toxoplasmosis of retina is a localised necrotising retinitis. All the
infective conditions that produce similar picture form a long list. Possible causes are
Tuberculosis, syphilis, cytomegalo virus retinitis, herpes simplex retinitis, acute retinal necrosis,
candida retinitis. The above conditions may be superimposed on toxoplasma infection, hence
mere presence positive serological results for toxoplasma does not rule out these.
Management
There is no prophylaxis against toxoplasmosis.
Treatment consists of:
1. Systemic anti toxoplasma chemotherapy
2. Local treatment of anterior segment involvement
3. Management of squint, amblyopia.
Chemotherapy is indicated in vision threatening retino choroiditis involving the macula,
papillo macular fibre lesions near the disc or severe vitritis.
The basic pathology of toxoplasmosis retinitis is active multiplication of organism ac-
companied by inflammatory reaction and scar formation. The aim of the treatment is to mini-
mise multiplication and destroying the already present organism by chemotherapy along with
reduction in inflammation by anti-inflammatory drugs. Chief out of them being systemic ster-
oids.
Common drugs used are (They should be given in consultation with
pediatrician)
1. Sulphonamide
(a) Sulphadiazine alone
(b) Triple sulpha.
2. Pyrimethamine. Adult dose is loading dose of 75 mg-150 mg followed by 25-50 mg
daily for four weeks. The drug is folic acid antagonist so it should be seen that the
child is not prescribed folic acid during this treatment. Folinic acid in a dose of
5 mg. three times is said to protect bone marrow.
3. Clindamycin is given four times a day for three weeks. Clindamycin is known to
produce pseudo membranous colitis.
4. Other newer drugs available are Atovaquone, roxithromycin and azithromycin.
Systmic steroid should be used under cover of chemotherapy with usual precaution.
Retinal toxocariasis
51,52
Ocular toxocariasis is a world wide problem. It is seen more commonly in developing countries
and in children who come constantly in contact with puppies that are infected by toxocara
canis. Children are infected by swallowing eggs of the parasite that are found in the soil,
contaminated by faeces of infected puppies. The eggs are converted as larva in the stomach
and intestine of the child and penetrate the wall of the bowel and reach systemic circulation
that takes them to distant organs including retina. One of the peculiarities of the parasite is
that it is never converted in to adult worm is humans hence the stool of the child never shows
evidence of worm infestation. It can infect the eye without systemic involvement. In fact con-
current ocular and systemic infection is rare.
There are three modes of presentation of intra-ocular toxocariasis:
1. Sub macular granuloma that later involves whole of the macula
2. Peripheral granuloma that may present as parsplanitis
3. Larval endophthalmitis.
The macular granuloma is limited to posterior pole hence there is no anterior seg-
ment reaction or redness but vision is poor. Solitary localised macular lesions may go unno-
ticed as they are generally unilateral or may present with squint, otherwise they are discov-
ered on routine vision check up. There is generally pre retinal gliosis that may cause grey
reflex on retinoscopy.
The peripheral retinal granuloma is also solitary and unilateral with minimal ante-
rior chamber reaction similar to parsplanitis. The lesion is oval nodule with pre retinal gliosis
and formation of vitreous bands that may extend back into to macula or even disc causing
traction retinal detachment.
Larval endophthalmitis is the most extensive form of the infection of chronic nature
and is a cause of white reflex in pupillary area. It may terminate in complicated cataract,
glaucoma and blindness. Differential diagnosis consists of retinoblastoma, persistent pri-
mary hyperplastic vitreous, Coats disease, endo-genous bacterial endophthalmitis,
toxoplasmosis, retinopathy of prematurity, congenital and traumatic cataract, fa-
milial exudative vitreo retinopathy.
The diagnosis is by exclusion. There is no specific test for toxocariasis. ELISA is most
sensitive test but also gives false positive result in about 10 percent of cases.
Management consists of prevention, as far as possible, deworming of household pup-
pies is best available method. Ant helminthics given to the child have not proved to be effec-
tive.
Retinal vasculitis
Retinal vasculitis is rare in children. Idiopathic periphlebitis (Eales disease), which is
commonest form of periphlebitis in adults between 20 to 40 years of age is not seen in children,
however there may be secondary vasculitis in older children due to parsplanitis, tuberculosis,
cytomegalo virus. They may present as Eales disease. The peripheral retina is involved
more than posterior pole. The peripheral lesion may go unnoticed unless they present with
diminished vision. Retinal changes include peripheral vasculitis, sheathing,
neovascularisation, superficial and subhyaloid haemorrhage, retinal band, traction
detachment, non perfusion of vessels.
Treatment consists of:
1. Treatment of the primary cause
2. Management of neovascularisation and non-perfusion by laser photo coagulation.
3. Management of non absorbing hemorrhage by vitrectomy with endo laser photo
coagulation.
4. Management of retinal detachment.
Vascular retinopathies in children
Vascular retinopathies especially diabetic retinopathy are common cause of blindness in adults,
fortunately they are very rare in children.
Common vascular retinopathies in adults are:
1. Diabetic retinopathy
2. Hypertensive retinopathy
3. Renal retinopathy
4. Toximia of pregnancy
5. Sickle cell retinopathy
6. Atreosclerotic retinopathy.
Proliferative diabetic retinopathy may be seen in older children with type I diabetes
53
Similarly hypertensive retinopathy can be seen in secondary hypertension due to any cause as
Schies grade IV classification. Chronic glomerulo nephritis may present as renal retinopathy
comprising of sclerotic changes in arteries, retinal edema, superficial haemorrhages, occa-
sional deep haemorrhages, soft exudates, may have macular fan and blurring of disc margin.
Retinal dystrophies and degenerations
Although both the terms dystrophies and degeneration are used as interchangeable terms for
same group of disease, they are not synonymous.
Dystrophies are abiotrophic disorder, which means that they are genetically deter-
mined disorders, which are not evident at birth, manifest late in life. There is premature
degradation of highly differentiated cells in specialised tissue like brain, and retina. The
cause of dystraphy is supposed to be a programmed death called apoptosis. The dystrophies
are progressive, and bilateral, generally symmetric. Some of them may have faculty en-
zyme or some other bio-chemical defect. Most of the conditions are not preventable and do
not have effective treatment.
Degenerations are generally acquired, secondary to infection, inflammation and
trauma, may be unilateral, may be prevented by directing treatment towards the primary
cause and are non-hereditary.
The retinal dystrophies are a group of disorders that are difficult to classify. Some of
them are more common i.e. retinitis pigmentosa than others.
Most useful classification is on the basis of layers of retina involved:
1. Photo receptor dystrophy
(i) Rod and rod-cone dystrophy
(ii) Cone and cone-rod dystrophy
(a) Progressive cone dystrophy
Type I and type II
(b) Stationary cone dystrophy
(c) Cone monochromatism.
2. Ganglion cell dystrophies
(i) Cherry red spots
(ii) Pigmentary dystrophies
(a) Fundus flavi maculatous
(b) Stargards disease
(c) Vitelliform dystrophy
(d) Dominant drusen
Other less common dystrophies are Kandori syndrome and dominant progressive
dystrophy.
3. Bruchs membrane dystrophies
(a). Sorsby pseudo inflammatory dystrophy
(b) Disciform degeneration of macula (not a true dystrophy)
(c) Angioid streak
Some of the dystrophies, which are altogether different from the above condition also
involve vitreous and are known as vitreo retinal dystrophies. They include:
1. Juvenile retinochisis
2. Wagners vitreoretinal dystrophy
3. Familial exudative vitreoretinal dystrophy
4. Goldmann -Favre vitreoretinal dystrophy
Out of all the conditions listed above (all are not seen in childhood, very few are seen in
first decade), the most common disorder is rod and rod cone dystrophy where rods are affected,
predominantly involvement of cone is less marked and of late onset. They are generally known
as retinitis pigmentosa and allied conditions. Again the term retinitis is a misnomer because
there is no evidence of inflammatory process in the condition. It is better to call the condition
as primary neuro sensory dystrophy of the retina or simply primary retinal dystrophy.
The conditions in the group can be:
1. Typical retinitis pigmentosa
2. Variation of retinitis pigmentosa
3. Retinitis pigmentosa associated with other ocular disorders - Myopia, chronic simple
glaucoma, keratoconus
4. Retinitis pigmentosa with systemic involvement
Retinitis pigmentosa
54,55,56
This is commonest of all hereditary retinal dystrophies. It is seen among all races with
equal distribution all over the world with pockets of large numbers in communities where
consanguinity is common. It is seen both in boys as well as girls. Boys are more affected
than girls. Women are generally the carrier in X linked diseases. They may be asymptomatic
or may have mild symptoms, however fully developed retinitis pigmentosa is not exception in
carriers.
The condition is mostly hereditary, however sporadic cases are also known. Sporadicity
is due to sudden mutation of genes. There are about twenty genes that have been blamed for
the disease. There are about hundred possible mutations. The sporadic cases are also called
isolated cases. They are seen in about 15 to 20 percent cases.
There are three modes of inheritance:
1. Autosomal dominant
2. Autosomal recessive
3. x linked
Autosomal recessive is the commonest form of transmission followed by autosomal domi-
nant i.e. mildest, with late onset but diagnosed early. The x linked is the least common but
worst. It is most disabling and progresses fast.
Symptoms
1. Diminished night vision. This is the first symptom developing around ten years of
age. It may be seen earlier in some children who may be confused as suffering from
vitamin A deficiency and treated as such without any improvement. The night blind-
ness is gradually progressive and becomes worse as the child ages. By third decade
the person may be incapacitated in dim light. To begin with the children have pro-
longed dark adaptation i.e. they take more time to get accustomed to indoor illumina-
tion when they come from bright light.
2. Diminished peripheral field. The patient soon becomes aware about his loss of
peripheral field. He complains that he has to move his head to see on the periphery.
He may bump into others and may find difficulty in negotiating on the road or drive.
The end result is greatly reduced peripheral field resulting into a tubular vision as
if the patient has been looking down the barrel of a gun. Generally the central part
surrounded by scotoma retains good vision for long time.
3. Diminished distant vision. Diminished vision is late to appear unless it is due to
myopia which is very common in retinitis pigmentosa. Onset of diminished vision
can be due to
(i) Macular lesion. Cystoid macular edema, atrophic maculopathy, cellophane
maculopathy
(ii) Lenticular. Posterior sub capsular cataract is the commonest type of lenticular
changes in retinitis pigmentosa. However development of central nuclear sclero-
sis is also frequent.
(iii) Optic nerve. Consecutive optic atrophy is very common in late stage.
(iv) Chronic simple glaucoma. Incidence of chronic simple glaucoma is high in
retinitis pigmentosa. Field changes of chronic simple glaucoma when superim-
posed on that of retinitis pigmentosa worsen each other.
Signs of retinitis pigmentosa
The triad of signs i.e. pigments, attenuation of blood vessels and waxy pale disc are
characteristics of retinitis pigmentosa in presence of night blindness.
1. Pigmentary changes. The pigmentary changes are brought about due to migra-
tion of pigment from deeper layer. They start in the mid periphery of the retina. In
early stages, they are nothing but mottling of retinal pigment with prominence of
chorio capillaries. With passage of time bunches of pigment begin to collect round
the veins, then they cover all the vessels. Later the pigments become spider shaped
or bone spicule like in shape. In the same eye various shapes and sizes of pigment
can be seen. The pigments gradually spread centripetal as well as centrifugal but
never reach the posterior pole unless it is central type. Affinity of the pigments for
mid periphery is not well understood. Most common argument put forward is pau-
city of blood supply of the mid periphery.
2. Thinning of retinal blood vessels. Though pigmentary changes are more con-
spicuous than attenuation vessel, the vessel changes may precede the pigmentary
changes. The vessels may become thread like in late stages.
3. Optic atrophy is a late feature. The colour of the disc is waxy pale. It may be asso-
ciated with other abnormalities like drusen, may have subtle glioma of the disc.
4. Error of refraction. About three fourth of eyes with retinitis pigmentosa have myo-
pia of moderate degree.
5. Vitreous opacities are very common in retinitis pigmentosa, myopia may be partly
responsible for it. The opacities increase with age.
6. Macular involvement are more common than previously thought to be. Macular
changes are being reported more frequently following fluorescein angiography. It
may just be a decreased reflex or as serious as cystoid macular edema.
7. Lenticular changes. Posterior sub capsular opacification is the commonest form of
lenticular changes. It may be seen as early as ten to fifteen years of age. The inci-
dence increases loss of central vision due to posterior sub capsular opacification.
Posterior polar cataract add to the misery of the patient who already has a very
narrow field.
8. Chronic simple glaucoma is seen in a small percentage of cases in third and fourth
decade.
9. Other ocular findings areKeratoconus, squint, progressive external ophthalmi-
oplegia and rarely microphthalmos.
Variants of a typical retinitis pigmentosa
The triad for diagnosis of retinitis pigmentosa are bilateral annular pigmentation in mid
periphery, attenuation of retinal vessels, waxy pale disc with diminished vision. In some cases
the pigmentation is unilateral or just sectorial. Sometimes night blindness may precede
pigmentation or there may be white dots in the periphery with night blindness. In rarer cases
the involvement of the posterior pole is disproportionate to peripheral lesion. All these conditions
are called atypical form of retinitis pigmentosa. Some authors are of opinion that these cases
except those with white dots will culminate into typical retinitis pigmentosa. The common
forms are:
Sector retinitis pigmentosa
In this rare form, retinal dystrophy is localised to a sector of the retinal mid periphery. Com-
monest area being one of the inferior quadrants, generally involvement in both eyes is similar.
The pigmentation and attenuation of vessels are same as in typical retinitis pigmentosa. The
area of dystrophy being localised, there is no night blindness, field defect corresponds to the
area of retina involved. Dark adaptation may be abnormal. ERG is also subnormal. The pa-
tient has good central vision. The two common causes of localised pigmentation should be kept
in mind before diagnosis of sector retinitis pigmentosa. They are trauma and localised retinal
inflammation. The condition does not require any treatment.
Central retinitis pigmentosa (Inverse retinitis pigmentosa)
In these cases pigmentary changes similar to retinitis pigmentosa occur round the macula.
Due to central involvement, these cases are misdiagnosed as cone or choroidal dystrophy. As
the central retina is involved, diminished central vision, reduction of vision in bright light,
defective colour vision and positive central scotomas are common. On rare occasion the retinal
vessels get attenuated and pigmentation spread to the periphery over years. ERG is always
subnormal. In adults it should be differentiated from chloroquine maculopathy and other
dystrophies of macula. There is no specific treatment. The children may require rehabilita-
tion.
Retinitis pigmentosa sine pigmento
The patient present with diminished night vision without pigmentation. On examination they
have attenuated blood vessels and waxy pale disc. It may develop pigmentation years after
onset of night blindness. The ERG is not recordable. Family members may suffer from
typical retinitis pigmentosa.
Unilateral retinitis pigmentosa
Like sector retinitis pigmentosa, there is doubt if unilateral retinitis pigmentosa can exist
without involvement of the other eye. The cases that have been reported as unilateral retinitis
pigmentosa had abnormal ERG and EOG in the eye with pigmentation. However possi-
bility of trauma, inflammation and vascular anomaly should be excluded. It is possible that
the other eye may develop pigmentation later.
57
Retinitis punctata albescens
This is a condition of diminished vision, has multiple white dots, scattered all over the retina
instead of black pigments of typical retinitis pigmentosa. The vessels are thin and the field is
constricted like in retinitis pigmentosa. The condition must be differentiated from other causes
of white spots on the retina i.e. retinal drusen and asteroid hyalitis.
Retinitis pigmentosa associated with systemic disorders
The list of systemic disorders that have signs of retinitis pigmentosa are either hereditary or
have disturbances of metabolism, is long. They can be divided into:
1. That have predominant skeletal involvementLaurence-Moon-Biedel-Bardet
syndrome, Marfans syndrome.
2. Hearing defect Refsums syndrome
Ushers syndrome
Cockaynes disease
Alstorm syndrome
3. Renal disease Cystonosis, cystinuria, oxalosis
4. Bone disease Pagets disease,
5. Mucopolysaccharidosis
6. Lipidosis
7. Spino ponto cerebellar degeneration
8. MiscellaneousProgressive external ophthalmoplegia, myotonic dystrophy.
Laurence-Moon-Bardet-Biedle syndrome
Obesity in a child is most striking feature, which is present in 90% of cases. On close look, the
child may have polydactyly and syndactyly which is obvious at birth and hypogenitalism.
These children have low IQ and overall mental retardation. Commonest symptom is night
blindness that starts by ten years of age. Soon to this is added diminished central vision due
to involvement of macula. Some children may have nystagmus. The fundus picture is similar
to retinitis pigmentosa i.e. bone spicule pigments on the periphery, thinning of retinal vessels
and waxy pale disc. The field changes, ERG and EOG changes are similar to typical retinitis
pigmentosa. Some children may show albipuntate changes. Other systemic disorders include
- brachycephaly, short stature that makes these children look more obese, renal disorder,
deafness, congenital heart disease. The disease is an autosomal recessive disorder.
All children with polydactyly or syndactyly should be observed for unusual weight gain
and screened for pigmentation changes in the retina. There is no specific treatment.
Rafsums syndrome
This is an autosomal recessive disorder. The syndrome consists of peripheral neuropathy,
ataxia, night blindness, and atypical pigmentary dystrophy with constricted retinal
vessels, poor dark adaptation and sub normal ERG. The pigments are in clumps and look more
or less like pepper and salt appearance rather than typical bone spicule. The condition is a
disorder of metabolism of fatty acid. Both serum and urine contain phytanic acid. It is claimed
that dietary restriction of fatty acid can improve the condition. Other systemic involvements
are - Progressive deafness, reduced corneal sensation, loss of hair, icthyosis. There may be
conduction defect in the heart that may prove to be cause of sudden death.
Ushers syndrome
Ushers syndrome is a major cause of genetic hearing defect. It includes neuro sensory
deafness, vestibular disturbance and night blindness. Association of neuro sensory defect along
with degeneration of pigment may be traced to similarity in development of retinal pigment
and the epithelium of organ of Corti. It is an autosomal recessive disorder.
Night blindness may start between ten to twenty years of age and progress relentlessly.
Associated macular lesion is cause of diminished distant vision. Peripheral field is constricted,
there is sub normal ERG, EOG and dark adaptation.
Cokaynes syndrome
This is autosomal recessive disorder that has diminished night vision, retinitis pigmentosa,
infantile deafness, pre mature old age, dwarfness and mental retardation.
Bassen-Kornzweig syndrome (abetalipo proteinemia)
It is an autosomal recessive disease seen mostly amongst Jews. The condition is detected
in first decade with diminished night vision with pigmentary changes in the retina. RBCs
show acanthocytosis. These children generally suffer from malabsorption syndrome. Serum
cholesterol and serum beta lipo proteins are less than normal. There may be spinocerebellar
ataxia, which progresses slowly and is stabilised in adults. Oral administration of large doses
of vitamin E and A are said to be helpful.
Causes of retinal pigmentation without dystrophy are:
1. Salt pepper fundus due to congenital rubella, syphilis, influenza, cystinosis, rubeola.
2. Drug induced pigmentationPhenothiazine, chloroquine (macular pigmentation),
indomethacin, quinine.
3. TraumaSmall laser burns, cryo spots
4. Congenital melanosis (in clumps)
Diagnostic procedure for retinal dystrophy
1. HistoryHistory gives important clue in diagnosis of retinitis pigmentosa.
(a) Night blindness. If a child under ten years of age presents with night blindness
first is to find out if it is due to vitamin A deficiency or retinal dystrophy.
A child with vitamin A deficiency and night blindness invariably has other signs
of protein caloric malnutrition, xerosis or even keratomalacia. Such night blind-
ness improves within 24 hours to 72 hours following administration of water
soluble injection of vitamin A or within a week following oral administration of
vitamin A concentration 200,000 IU on two consecutive days. An adult with night
blindness does not improve with vitamin A administration.
(b) Positive family history
(i) Night blindness.
(ii) Proved retinitis pigmentosa among siblings, parents and other blood relatives.
(iii) Consanguinity among parents, grand parents.
(c) Evidence of other neuro sensory disorders like deafness, ataxia, mental retarda-
tion.
2. Field changes
(i) Ninety percent of cases with retinitis pigmentosa have ring scotoma, by the time
they report with night blindness which is not cured by therapeutic dose of
vitamin A.
(ii) The field changes usually start as isolated scotoma concentric with equator in
the inferior temporal area. This gradually enlarges on either end to become a
ring scotoma. Once the ring has been formed, the scotoma expand both anteriorly
and backwards leaving only an island of vision.
3. Dark adaptation. This is a sensitive test second only to ERG to show abnormality
even before the fundus findings appear. It shows elevation of rod threshold. The cone
threshold may also be raised.
4. Electro retinogram. ERG is almost always sub normal even before the fundus
changes develop. Scotopic ERG is more markedly involved than photopic ERG.
ERG of the family members may show abnormality without night blindness. Hence
this is used to screen members of the family for retinitis pigmentosa.
5. Colour vision defect is more marked in cone dystrophy.
6. Fluorescein angiography. This is not a specific test. It shows thinning of retinal
vessels, diffuse hyper fluorescence due to hypertrophy of pigment epithelium.
Cone and cone-rod dystrophies
These disorders are as common as rod dystrophies but do not have prominent symptoms like
night blindness. They are associated with diminished central vision. The subtle cases may
be missed or misdiagnosed as amblyopia.
The symptoms consist of diminished central vision, poor vision in bright light,
photophobia, defective colour vision and nystagmus.
The cases are generally sporadic but autosomal dominant and autosomal recessive in-
heritance is also possible.
The conditions are broadly divided into two groups:
1. A rarer form of progressive cone dystrophy which again on the basis of fluorescein
pattern have been divided into type I and type II.
2. Commoner form of stationary cone dystrophy.
Progressive cone dystrophy
The condition is bilateral, first detected in first decade as acquired diminished distant
vision, photophobia and nystagmus. On examination the children have severe colour
vision defect which may end in achromatopsia in later stages of life.
The macula has a bulls eye appearance, which consists of clear central area surrounded
by zone of pigmentary changes, which is best seen by fluorescein angiography that have multiple
window defects without staining.
To begin with, the optic nerve may show temporal pallor which may become pale later.
Thinning of retinal vessels also is a late feature.
Visual field shows central scotoma.
ERGPhotopic ERG is abnormal with small b wave. Scotopic ERG is normal, EOG is
generally normal.
Stationary cone dystrophy
The condition is congenital and non progressive autosomal recessive.
The symptoms are similar to progressive cone dystrophy, as far as diminished central
vision, abnormal colour vision, nystagmus, photophobia, diminished vision in bright light are
concerned. Only difference is that the condition is stationary in nature. The condition is more
frequent than progressive form. The colour defect is rodmonochromatism.
On examination, the retina may look normal with loss of foveal reflex and pigmentary
change in the macula.
Fluorescein angiogram is normal so is EOG.
The dark adaptation and ERG are sub normal. The dark adaptation shows normal rod
adaptation and abnormal cone adaptation. In ERG the photopic response is poor.
Congenital achromatopsia
This is an autosomal recessive disorder of macula without many visible clues. The condition
is congenital in nature. It can be rod monochromatopsia (typical) or cone monochromatopia
(atypical). The latter is rarer than the former. To these children all colours are but shades of
grey.
The rod monochromat has diminished central vision, photophobia and nystagmus.
Congenital night blindness (stationary)
There is early onset of night blindness, associated diminished distant vision. Moderate to high
myopia is common. Colour vision is not affected, photopic field is normal. The ERG is sub
normal, which is not influenced by mode of inheritance. There are three modes of inheritance
i.e. autosomal recessive, autosomal dominant and x linked. The x linked have poorest distant
vision. The fundus does not show any change except usual myopic change. The ERG shows
normal scotopic a wave and minimal response in scotopic b wave.
Ganglion cell dystrophy of retina
Cherry red spot
Red spot (Cherry red) in the macula is a non specific finding in many retinal conditions
mostly due to disturbance of metabolism of specific enzymes. These are generally bilateral,
hereditary disorders that have deposition of glycosides of various tissues of the body like
central nervous system, besides eye. The fovea is mainly involved. Other non biochemical
causes can also cause cherry red spot of macula.
Common causes of cherry red spot of macula can be brought about by:
1. Biochemical causes (glycolipids and phospholipid)
2. Macular hole Traumatic
Non traumatic
3. Macular cyst
4. Central retinal artery obstruction
5. Comotio retinae.
Cherry red spot is a contrast phenomenon where normal macular vasculature stands
out prominently among pale retina. The pallor of the retina is due to deposit of various chemicals
in the ganglion cells leading to cell death.
Cherry red spot maculopathy
Common causes are:
Tay-Sachs disease (Infantile, amaurotic, familial idiocy) Batten-Mayou disease
Niemann-Pick disease
Sandhoff disease
Generalised gangliosidosis
Sialidosis
Metachromatic leuco dystrophy
Most of the above conditions are hereditary, may have family history. Some of them are
seen in specific races (Tay-Sachs), starts early in life, have various degrees of visual loss, have
multiple systemic involvement.
The other type of involvement of macula leads to pigmentation of macula and flecks on
retina. They are calledflecked retina syndromes. These disorders are dystrophies of retinal
pigment epithelium.
The two main causes of flecked retina syndrome are fundus flavimaculatous and
Stargardts disease. There is some difference of opinion regarding nomenclature of these
conditions. Some consider that Stargardts disease is late manifestation of fundus
flavimaculatous because they have similar symptoms, both have same hereditary and age of
onset is similar.
The main point of differentiation is presence of macular atrophy, which is present in
Stargardts disease and absent in fundus flavimaculatous.
Fundus flavimaculatous
This is an autosomal recessive disorder, is bilaterally symmetrical. The flecks, which are
accumulation of acid mucopolysaccharides and lipofucin in the retinal pigment epithelium,
develop in the posterior pole. The flecks are small and discrete yellowish white spots with
irregular margins. The lesions may be of various shapes i.e. fish like, semi circular or circular.
The flecks are known to disappear or absorb leaving areas of atrophy of retinal pigment
epithelium. New flecks may appear later so long the flacks are away from the macula, the
patient is asymptomatic, the flecks appear on the macula the vision falls and continue to fall
gradually, never reaching a level of legal blindness. Involvement of macula may cause poor
colour sense. Dark adaptation, ERG and EOG are normal. Fluorescein angiography shows
hypo fluorescence in early stage and hyper fluorescence in later stage. The lesions have blurred
margin without leak.
Stargardts disease
58
As mentioned early some authors consider Stargardts disease as a late presentation of fundus
flavimaculatous. The characteristics of Stargardts diseases are progressive, bilateral,
symmetric loss of vision in young patients who had subnormal central vision due to
atrophy of macula. It is autosomal recessive disorder. It is more common in consanguinity.
The patient has poor vision in bright light, which he may express as glare or photophobia.
Part of the diminished vision is explained on the basis of myopia, which is common error of
refraction. In early stages the child is brought with loss of one or two lines on Snellens chart
that gradually progresses to 1/60 or less. The children with Stargardts disease have red green
colour defect that may terminate into achromatopsia.
Diagnosis is on the basis of fundus findings and fluorescein angiography.
FundusIn initial stages the fundus may look normal with moderate uncorrectable
diminished distant vision that may be confused with amblyopia or functional. The first visible
sign is loss of foveal reflex. This is followed by bulls eye appearance that has normal
retina surrounded by area of de-pigmentation. By this time some flecks may develop in the
macula giving a name of varnished macula. This is followed by onset of atrophic changes in
macula and the macula has a brownish appearance called beaten bronze appearance. The
choroidal vessels may become visible through the atrophic macula. Small flecks may appear
round and into the macula. Optic nerve may show temporal pallor.
Fluorescein angiography
Changes in angiography may be visible before the ophthalmoscopic changes occur. The typical
picture in normal retina with zone of hyper fluorescence all round without leak.
Dark adaptation and ERG are normal but EOG findings are abnormal. The central
field may show relative scotoma. The condition should be differentiated from dominant
progressive foveal dystrophy.
There is no specific treatment.
Differential diagnosis of flecked macula consist of
Progressive and stationary albipunctate retinal degeneration and Uyemuras disease
all have diminished night vision, abnormal ERG, EOG and dark adaptation as all of them are
rod dystrophies with flecks on the retina.
Vitelli form macular dystrophy
This is autosomal disease, starts between four to ten years of age, may be bilateral but
not always simultaneous or symmetric. Mode of presentation is variable. The child may be
asymptomatic yet can have a full blown disease. The diagnosis is straight forward, on
ophthalmoscopy typical egg yolk appearance of the macula is seen from which it gets its name
of vitelli form dystrophy. This is also known as Bests disease. The carriers can be asymptomatic
with normal macula but abnormal EOG. Association of hypermetropia, esotropia and
amblyopia is common.
Symptoms depend upon the stage of the disease. The disease has been divided into pre
vitelli form and vitelli form stages, the latter is again divided in to stage of pseudo
hypopyon, rupture of cyst and complication of rupture.
The pre vitelli form stage is asymptomatic, even fundus changes are absent but EOG
done at this stage is abnormal. Suspicion of the disease arises when one eye is already in
vitelli form stage.
The vitelli form stage consists of diminished central vision. On fundus examination
an ill defined yellow spot develops generally over the fovea but may be eccentric. The spot is in
the retinal pigment epithelium most probably due to accumulation of lipofuscin forming
an orange cyst similar to sunny side up egg yolk that may be 1/3 or half of the optic nerve
head. Retinal vessels travel over the cyst.
Stage of pseudo hypopyon
There is no accumulation of pus. The name is acquired due to settling of the lipofucin at the
bottom of the cyst, upper part of the lesion may be absorbed. The pseudo hypopyon blocks the
fluorescein, the upper part shows hyper fluorescence due to atrophy of the retinal pigment
epithelium. It takes about ten to twenty years for this stage to develop, vision is minimally
reduced, EOG is abnormal.
Stage of rupture of the yolk
The cyst changes from sunny side up to appearance of scrambled egg with diminished vision.
Stage of complication
The complication consist of formation of choroidal neovascularisation, with sudden and
profound fall of central vision, there may be vitreous haemorrhage. There may be glial
proliferation and formation of a hypertrophic scar. The macula may develop an atrophic
patch through which the underlying vasculature may show as pink glow surrounded by white
glial tissue.
Dominant drusen of the retina
This condition should not be confused with more frequent condition i.e. drusen of optic nerve.
They are of different etiology, clinical presentation and pathogenesis.
In drusen of retina there is deposition of yellowish white material in the posterior
pole in retinal pigment epithelium and Bruchs membrane in the macula or round it.
There are two types of retinal drusens -
1. The primary (familial)
2. The secondary - Secondary to local ocular disease like benign choroidal nevus or
systemic conditions.
The primary familial drusen is inherited as autosomal dominant disorder. It is a bilateral
condition, which becomes evident in second decade of life as yellowish white circular raised
area, which may be discrete or may confluent to form large plaque that may get calcified in
fifth decade. In early stages the deposits may be confused with other white dots on the retina
i.e. hard exudates, fundus albipunctatus, and asteroid hyalopathy. Lesion on the nasal
side of the disc is more common in familial type.
The drusens in pediatric age group are symptomless and are discovered on routine fundus
examination, do not require any treatment. Persons with macular drusen are predisposed
towards age related macular degeneration, a major cause of blindness in aged.
Peripheral retinal degenerations
59
There are many retinal degeneration on the periphery of the retina. Most of them develop
after third decade. Some of them predispose rhegmatogenous retinal degeneration, others are
diagnosed on routine examination and are asymptomatic. The degenerations are bilateral
and may be symmetric.
Common peripheral retinal degeneration are:
1. Lattice degeneration
2. Snail track degeneration
3. Cystoid degeneration
4. White with pressure
5. White without pressure
6. Peripheral chorio retinal degeneration (paving stone or cobble stone degeneration)
7. Chorio retinal atrophy
8. Retinoschisis
All the above conditions are not seen in children though many of them start in child-
hood. Only peripheral retinal degeneration is seen in childhood is lattice degenera-
tion.
Lattice degeneration of the retina
Lattice degeneration is the commonest form of peripheral retinal degeneration about eight
percent of eyes have asymptomatic lattice degeneration which are discovered on routine
examination of the retinal periphery by indirect ophthalmoscope. Commonest site for such
patches is lower temporal quadrant near the ora. They are more common in moderately
myopic eyes. It is common in Marfans syndrome as well as Ehlers Danlos syndrome
and Wagners disease. In about 30% of cases of rhegmatogenous retinal detachment, lattice
degeneration may be the predisposing cause. The degeneration starts in about ten years of
age as scattered small patches on the periphery. The condition is well established in second
decade. There may be family history of retinal detachment. It is equally seen among boys
and girls.
Fundus picture
The lesion may be single or multiple, they are generally parallel with the equator but can
be radial, parallel to peripheral blood vessels as in Sticklers syndrome. There may be more
than one row of patches of lattice degeneration in the same quadrant. The degeneration derives
its name from white lace like pattern caused due to sclerosed vessel in the patch. The area
of degeneration is generally a spindle shape patch. The lattice may be associated with
snowflakes, white with pressure and hyperplasia of retinal pigment epithelium.
Symptom
The condition may go without symptoms but may be associated with flashes of light and
floaters, which should be taken seriously and eye examined with indirect ophthalmoscope.
Retinal holes are common in lattice degeneration, they develop in the substance of the
lattice. All holes are not symptomatic, occasionally they are responsible for retinal detachment
which is heralded by development of retinal tear. The tear generally develops outside the
island of lattice on the posterior border. They are more common in moderate myopes and eyes
with acute posterior vitreous detachment. Occasionally latticed part may be seen on the flap of
retinal tear.
Snail track degeneration
There is doubt if this condition is a separate entity or precursor of lattice degeneration or its
variation because their position and distribution on the retinal periphery is the same, both are
associated with snow flecks. Complications of snail track degeneration are same as lattice
degeneration.
Retinoschisis
In retinoschisis, the sensory retina is splits into two layers, in contrast to retinal detach-
ment where the retina separates between the pigment epithelium and sensory retina. In
retinoschisis the split occurs between the nuclei of visual cells and bipolar cells.
The condition can be divided into juvenile (idiopathic) and senile retinoschisis. (See
page 403.)
Congenital retinoschisis is present at birth but not diagnosed unless looked specifically in a
neonate, born to parents, either of whom is known to suffer from congenital retinochisis. In-
heritance is varied. Commonest inheritance is x linked receive. Generally the condition is
diagnosed when the child reaches school age and is more often diagnosed as shallow retinal
detachment rather than retino schisis. This is true with other types of retinoschisis as well.
Juvenile retinoschisis
This is a bilateral, symmetrical condition, the hereditary is same as congenital retino schisis.
Some authors feel that juvenile retinoschisis is nothing but late feature of congenital
retinoschisis.
The lesion starts in the lower temporal quadrant in a hypermetropic eye. The sepa-
rated inner table balloons inwards, the ballooned layer is almost transparent, with blood ves-
sels. The two separate layers are best seen by +90 D or gonio lens. It may have round holes in
it, some times there may be hole in the inner table also. The balloon extends up to ora anteriorly
and may involve the macula behind.
Management
The congenital retinoschisis is generally non progressive, may have fair vision without any
surgical intervention. However if the condition progresses to involve the macula or
rhegmatogenous retinal detachment develops, then the lesion must be treated by laser photo-
coagulation and scleral buckling.
Retinal detachment
60
The term retinal detachment is a misnomer as a clinical diagnosis because whole of the retina
is not detached but there is inter retinal separation of the layers and it is better to call it
retinal separation. However it is difficult to replace old terminology by new, hence, the term
retinal detachment has stayed in clinical practice.
The retina develops from the two layers of the optic cup. The outer layer contributes to
form the pigment epithelium. The rest of the nine layers i.e. the sensory retina develop form
the inner layer of the optic cup. In early stages of embryogenesis, the gap between the two is
visible, the gap gradually diminishes and ultimately disappears leaving a potential space which
is liable to be opened up through out the life if something could get in to it.
What keeps the two layers in contact with each other is not well understood. The two
theories that are generally put forward are
60
:
1. The microvilli of retinal pigment epithelium inter digitate with the outer end of the
photo receptors, creating a bond between the two layers.
2. Acid mucopolysaccharides form biological glue between the photo receptors and the
RPE.
The other contributory factors are:
1. Presence of semisolid vitreous on the retina, which exerts a force to keep the two
layers in apposition.
2. Almost lack of mobility of normal vitreous.
3. Normal intra ocular tension.
The factors other than apposed layers of retina that keep this space devoid of
fluid are:
1. Active metabolic transport between retinal pigment epithelium and sub retinal fluid
2. Oncotic pressure gradient between sub retinal space and choroid also keep the space
free of fluid.
The vitreous plays an important role in keeping the retina apposed. So long the vitreous
is semi-solid and it does not exert any traction on retina even at places where it is attached i.e.
the ora serrata, round the optic nerve, at the fovea and along the major blood vessels. Abnor-
mal vitreo retinal attachments develop over chorio retinal scar.
Retinal detachment has been divided into two groups:
1. Rhegmatogenous
2. Non rhegmatogenous
(a) Tractional
(b) Exudative
Rhegmatogenous retinal detachment is most common detachment in children followed
by traction. Exudative retinal detachment is least common in children.
Rhegmatogenous retinal detachment is a treatable cause of diminished vision
and party preventable.
The two factors that contribute to the development of rhegmatogenous detachment are:
1. Retinal break
(a) Hole.
(b) Tear
2. Liquifaction of vitreous
Presence of only one of the above rarely leads to retinal detachment.
Retinal breaks
These are full thickness passages between the vitreous cavity and space between RPE and
sensory retina. Retinal breaks may take different shapes with varied size and number. One
thing common about retinal breaks is that they predispose retinal detachment.
61
The retinal
breaks look dark red because the retinal pigment epithelium is exposed. About six percent of
eyes have retinal holes without retinal detachment. It develops only in one in seventy such
cases
62
. The circular breaks are called holes while others are called tears. All Retinal breaks
are almost exclusively seen on the retinal periphery except a macular hole or a traumatic
tear. Macular holes, generally do not lead to detach-ment. Macular holes can be full thickness
or partial. Macular holes are rare in children.
Round holes
These are punched out circular holes, size of which vary from pin head to almost as large as
the optic disc. There number also vary from single to multiple. When a piece of retina is
hinged to one edge of the hole or attached the retracted vitreous near the hole and protrudes in
the vitreous, it is called operculated hole. Most of the holes are non operculated, the round
holes develop in the area of chorio retinitis or in lattice degeneration. The non operculated
holes are also known as atrophic holes. They have low incidence of detachment.
Tears according to their shape are called - Horse shoe shaped; giant tear and dialysis.
The horse shoe holesThese are crescent shaped tear with concavity towards the
periphery. The tongue of the retina towards the tear is called the flap. Sometimes bands
arising from the vitreous may be attached to the apex of the flap. They are common in chil-
dren. They are never as numerous as round holes.
Giant tears
These are also called limbus parallel tears due to their orientation in relation to limbus.
They are slightly posterior to ora serrata and parallel to it. These are the largest tears, gen-
erally there is only one giant tear in one eye. The tear extends more than 90 along the circum-
ference, it may rarely extend all round. They are seen in younger patients. In about 80% of
giant tears, no predisposing factor is detected. It is common in blunt injury, they can be
bilateral and have poor prognosis.
Dialysis of retina
This is disinsertion of retina at the ora serrata. Commonest site is inferio temporal quad-
rant, which is most stretched and least developed. Commonest cause is trauma otherwise it
can be idiopathic in some cases, they are bilateral. The detachment progresses slowly, as
the progression is slow, macular involvement is not felt abruptly. They are more common in
young males.
Liquefaction of vitreous
Normal vitreous is a semisolid gel incapable to flow. The vitreous does not have a formed
capsule, still it keeps its shape in the vitreous cavity. It is only liquified vitreous that causes
retinal detachment but not unless it has come out of the vitreous cortex. The fluid vitreous
when associated with deficiency in the vitreous body flows in between the vitreous body and
the retina and fill the newly formed retro hyaloid space. This pushes the vitreous forwards and
the condition is called posterior vitreous detachment (P.V.D.). Following PVD the retina
looses its support from a stable vitreous. The retina is directly affected by traction of vitreous.
The traction may be normal anatomical adhesion or pathological chorio retinal scar.
In adults the liquefaction of the vitreous is an ageing process and called synchisis
senilis. This does not happen in children. In children the vitreous is liquified following trauma
more in penetrating injury than blunt injury, chronic inflamation of choroid and retina, retained
intra ocular foreign bodies and myopia.
In the next stage the fluid vitreous finds its way into the potential space in between the
sensory retina and the retinal pigment epithelium and distends the space resulting into a
retinal separation or rhegmatogenous detachment. The sub retinal fluid gravitates down to
most dependent part and due to this it separates more retinal area from its natural apposition.
Hence a hole at 12 oclock is likely to cause more extensive detachment on either side of the
hole while a relatively large rent at six oclock detaches smaller area and the progression is
slow.
Symptoms
1. A shallow detachment at the lower part away from the macula may be symptomless.
2. Two common symptoms of rhegmatogenous retinal detachment are-
Photopsia (Flashes of light) due to retinal irritation and floaters. The floaters
may be due to RBC from the torn blood vessel at the site of tear, or due to liquified
vitreous. A ring shaped shadow the Weiss ring denotes posterior vitreous detach-
ment, occasionally a cobweb like opacity may be seen due to condensation of colla-
gen fibres in collapsed vitreous.
3. Metamorphopsia develops in early stages of detachment due to unevenness of the
retinal surface.
4. Diminished visionDegree of loss of vision depends on macular involvement. In
large detachment with a superior hole, loss of vision may be acute, it may fall from 6/
6 to hand movement of less.
The causes of diminished vision in retinal detachment are:
1. Involvement of macula
2. Overhanging detachment in front of macula
3. Blood in front of macula
In large detachment there may be associated loss of projection in the corresponding
quadrant.
5. Field changesThe field defects start from the periphery and spread towards the
centre. The field defect is brought about by spread of sub retinal fluid behind the
equator. The patient perceives the field loss as either a shadow, approaching from
the side, or a curtain coming down from above. Lower field defect is generally felt
earlier than upper field loss.
Signs of retinal detachment are variable. They depend upon site of the detach-
ment, extent of the detachment, duration of detachment.
1. Uncorrectable diminished visionThis may be associated with faulty projection
when elicited by small pinpoint light source.
2. Externally the eye is non congested and non tender.
3. PupilIn case of large retinal detachment the pupil may show Marcus Gunn pupil.
In small detachments the pupillary reaction may be normal.
4. Intra ocular tension is lower by 5-6 mm than previously noted IOP.
5. Anterior chamber reaction is common in long standing detachment, may occa-
sionally be present in recent cases.
6. Retinoscopy
(a) In case of extensive retinal detachment the retinal glow may be absent.
(b) Retinal detachment in the posterior pole may give a grey reflex due to pallor of
the detached retina.
(c) Hypermetropic shift of refractive error i.e. the pre existing myopic refraction
is lessened and hypermetropia increased.
7. Fundus examinationThe clue to diagnosis is in ophthalmoscopic examination of
the fundus under maximum mydriasis by indirect ophthalmoscopy, preferably by
scleral indentation.
Direct ophthalmoscopy may be sufficient to diagnose presence of retinal detachment.
It fails to pinpoint the cause i.e. the break, its position, number and size.
(i) With direct ophthalmoscope, the detached retina is slightly opaque due to
loss of underlying choroidal pattern. The grey surface is convex. The surface in
corrugated due to intra retinal edema. The retinal vessels both arteries and
veins look darker than in undetached retina. The colour difference between the
arteries and veins is less marked. The detached retina may undulate with
movement of the eye with out shifting in the fluid. The sub retinal fluid may
extend up to the ora serrata. The above findings are better appreciated by indirect
ophthalmoscope as it has stereopsis and larger field.
(ii) The retinal breaksThe presence of retinal break is the most important sign
in rhegmatogenous retinal detachment, both as a diagnostic as well as
prognostic feature and mode of treatment to be undertaken. The breaks are
generally on the periphery, may vary from solitary hole to multiple holes and
breaks. There may be very small or may present as large dialysis.
(iii) VitreousVitreous may show large floaters. Posterior vitreous detachment
is almost always present. In detachment of recent origin, blood may be present
in the subhyaloid space or in the liquefied vitreous. The anterior vitreous may
have tobacco dusting. The vitreous may be attached to the operaculum of a
hole or flap of a tear.
(iv) The periphery besides break may show peripheral degeneration especially lattice
degeneration or patches of chorio retinal scar.
The fundus finding of long standing retinal detachment differ considerably from that of
recent detachment. They are :
1. High-water mark if the retinal detachment is about a month old, pigments are
deposited at the border of the detachment which gradually fade. They do not represent
edge of sub retinal fluid. They lag behind the edge of sub retinal fluid.
2. Retinal thinning - This looks like retinoschisis.
3. Sub retinal fibrosis- Opaque strands develop on the detached retina.
4. Intra retinal cysts - If retina remains detached for more than one year, intra retinal
cyst may develop that disappear with re attachment of retina.
Other investigation that may be required
1. Ultrasonography is indicated in opaque media.
2. Slit lamp biomicroscopy may be required but does not have much advantage over
indirect ophthalmoscope.
Predisposing factors
1. Males are more predisposed to retinal detachment.
2. Family historyRetinal detachment can be seen in siblings or parents.
3. MyopiaIt is a well known factor that incidence of rhegmatogenous retinal detach-
ment is more in myopia. Incidence of detachment increases with increase in degree of
myopia. It is more common in high myopia but low and moderate myopes are not
immune. Incidence of rhegmatogenous retinal detachment is higher in myopicaphakics
and myopicpseudophakics. Blunt trauma may precipitate detachment more frequently
in myopia than in non myopic. Myopic males are more prone to detachment than
females. Myopes are more likely to have bilateral retinal detachment.
The causes of higher incidence of retinal detachment in myopia are many and may work
in combination, they are:
(i) The apposition of sensory retina to pigment epithelium is poorer.
(ii) Myopes suffer from lattice degeneration more commonly than non myopes.
(iii) Myopic retina is more stretched than non myopic retina, hence thin and likely to
develop tears.
(iv) A genetic factor may also be a predisposing cause.
4. TraumaBoth blunt as well as penetrating injury can cause retinal detachment.
Blunt trauma causes more detachment than open globe injury. Most of the time the
tear develops at the time of original injury. Any type of break is possible, holes are
less common. Out of all tears retinal dialysis is more common that progresses slowly.
Liquifaction of vitreous is common following blunt injury so is posterior vitreous
detachment.
5. Aphakia
(a) Aphakia following intra capsular lens extraction used to be a major cause of
senile retinal detachment prior to IOL surgery became popular. However intra
capsular lens extraction was never a cause of aphakic detachment in children in
whom this surgery was not performed. Traumatic aphakia remains a cause of
retinal detachment in children. Spontaneous dislocation of lens in Marfans
syndrome is also associated with rhegmatogenous retinal detachment.
(b) Detachment following extra capsular lens extraction is less common than intra
capsular lens extraction but never free from it. The incidence increases if repeated
needling are performed.
(c) Pseudophakia is not a guarantee against rhegmatogenous retinal detachment.
The incidence is higher in children because incidence of PCO is very in high in
children that require laser capsulotomy which itself is a cause of retinal detach-
ment.
6. Congenital anomalies - Choroidal coloboma, persistent hyperplastic primary vit-
reous, congenital pit of the optic nerve head are some of the congenital anomalies
that predispose rhegmatogenous retinal detachment.
7. Syndromes associated with rhegmatogenous detachment are - Marfans, Ehler
Danlos, Wagner, Pierre-Robinson, Stickler syndrome are frequently associated with
rhegmatogenous detachment.
Untreated rhegmatogenous retinal detachment may follow any of the follow-
ing course
1. A small detachment especially with superior hole rapidly expands and may become
total.
2. Many a times, lower detachment may not progress and remain so due to formation of
line of demarcation. Such detachment can have good anatomical apposition following
surgery but visual prognosis is guarded.
3. Small localised detachment have been reported to get reattached over prolonged bed
rest only to recur following strain.
4. Most of the untreated detachments develop complicated contract, chronic uveitis,
hypotony, rarely phthisis that may terminate in blindness.
Differential diagnosis of rhegmatogenous retinal detachment
Differential diagnosis consist of two types of disease:
1. Where there is actual retinal detachment, these comprise of non rhegmatogenous
retinal detachments
(i) Tractional retinal detachment
(ii)Exudative retinal detachment
2 Those condition that mimic retinal detachment
(i) Congenital and juvenile retinoschisis
(ii) Choroidal detachment
(iii) Cilio choroidal detachment
(iv) Vitreous bands.
(v) Dislocated lens.
(vi) Parasitic cysts
Non rhegmatogenous retinal detachment
Non rhegmatogenous retinal detachment may it be tractional or exudative have following
common features:
1. They do not have breaks in retina as a primary cause of detachment. Exudative
detachment never develops a break. A tractional detachment can develop a hole due
to abnormal traction on the retina.
2. The detachment surface does not have corrugation.
3. The sub retinal fluid is not a liquefied vitreous.
4. There is no hyaluronic acid in sub retinal fluid.
Tractional retinal detachment
Common cause of tractional retinal detachment in adults are - Proliferative diabetic retinopathy,
Eales disease, cataract extraction with incarcerated vitreous. These conditions are not seen in
children.
The common causes of tractional retinal detachment in children are:
1. Persistent hyper plastic primary vitreous
2. Retinopathy of prematurity
3. Hemoglobinopathy
4. Parsplanitis
5. Intra ocular foreign body
6. Congenital toxoplasmosis
7. Penetrating injury
The tractional retinal detachments develop slowly, many a times the primary cause like
injury may overshadow and a child may not complain of symptoms especially in unilateral
cases.
On examination
There is no break. The detachment has scalloped border. It does not reach the ora except
in parsplanitis, the surface is concave, and the detached retina is peaked at the site of the
traction. There is no movement of sub retinal fluid. The elevation is moderate. If breaks
develop due to pull of the traction, already existing detachment becomes worse and behaves
like a rhegmatogenous detachment, fluorescein angiography does not contribute much in
diagnosis. There is no leaking or pooling of dye, occasionally there may be some window defect.
Treatment is directed towards reduction of tractional force either by denting the sclera
in by way of scleral buckle, scleral resection or scleral encircling band or cutting the traction
band. In most of the cases both are required.
Exadative retinal detachment
The incidence of exudative detachment is for less than rhegmatogenous and tractional detach-
ment in children.
The common causes of exudative retinal detachment are:
Angiomatosis retinae, Coats disease, retinoblastoma, peripheral uveitis, dysproteine-
mias, choroidal rupture.
The characteristics of an exudative retinal detachment areThe surface is convex,
the boundaries are convex, neither retinal breaks or traction bands are present. The border
does not reach the ora. The sub retinal fluid shifts. The elevation of detached retina is moderate,
on fluorescein angiography the sub retinal fluid is tinged by the dye but there is no leak. There
is always evidence of primary disease. The condition waxes and wanes, may either spontaneously
disappear or retina gets attached with treatment of primary cause. Ultrasonography may show
a sub retinal growth, generally a tumour. In case of sub retinal tumour the retina is stretched
over the tumour with exudate round the tumour. There may be a tuft of blood vessel on the
retinal highest spot of elevation. Such elevations in the past were referred to as solid detachment.
Management of rhegmatogenous retinal treatment
There is no medical treatment for rhegmatogenous retinal detachment, prolonged bed rest
in particular position, prolonged patching of two eyes, use of pinholes and stenopic slits all
have proved to be futile.
Prophylaxis
All children with family history of rhegmatogenous retinal detachment, especially if they have
myopia and all children who have sustained closed or open globe injury with history of floaters
and photopsia, should be examined by indirect ophthalmoscope with scleral indentation. The
examination should be repeated every six month for next few years and if a hole or tear is
found, it should be closed adequately by laser or cryo. Some of them may be large enough to
be closed by local scleral explant.
Some of the points to remember are:
1. A tear is more likely to produce retinal detachment than a hole in the same location.
2. Larger breaks produce more detachment than smaller but size of break is not always
proportionate to its ability to produce detachment.
3. Superior breaks produce early and more extensive detachment.
4. Lower breaks produce less and shallow detachment.
5. Breaks behind the ora are more likely to cause detachment than breaks at ora.
6. Breaks with vitreous band attached to its flap are more likely to enlarge in size and
cause larger detachment. Such breaks are more difficult to manage than without
traction band.
7. All breaks specially holes do not require any prophylaxis. They must be observed
regularly.
The common conditions that do not require prophylaxis are - Breaks surrounded
by pigment all round, small holes near the ora serrata, white without pressure, retinoschisis.
Surgical treatment
Surgical treatment is the specific treatment of all rhegmatogenous retinal detachment. The
outline of the principle of treatment consists of
1. Searching and localising each and every retinal break by indirect ophthalmoscope
and scleral indentation.
2. All breaks associated with detachment should be closed irrespective of their shape,
size, number, by the following, either alone or in combination.
(i) Cryo coagulation, photo coagulationDiathermy has mostly been given up in
favour of the former two.
(ii) Scleral indentation either by an explant or rarely by implant. The explants are
either localised plombs or large encircling bands. Sometimes local implants may
be needed to be put over the large hole under the encircling band.
(iii) Drainage of sub retinal fluid is indicated in cases of longstanding retinal detach-
ment, immobile detachment, inferior tears. In some instances a break that was
not visible earlier become visible following drainage of sub retinal fluid.
(iv) Internal temponade byAir, balanced salt solution and silicone oil.
(v) Parsplana vitrectomyIn rhegmatogenous retinal detachment, parsplana
vitrectomy is required in giant tear, traction band, for gas fluid exchange.
D8EA8E8 OF VTREOU8
Anatomy of the vitreous
63,64,65
Vitreous is a semisolid transparent gel that constitutes the largest intra ocular structure.
Even few decades ago it was considered to be of little significance except that it filled a large
space, kept the retina in place with optical properties, and when disturbed led to unpleasant
events of redness, pain, lacrimation and loss of vision. With better understanding of vitreo
retinal disorders, it is clear that vitreous plays an important role in retino vitreal and retino
vascular disorders.
The vitreous fills the vitreous cavity. In adults, it measures about 4 CC in volume in
normal eye. It is surrounded by the posterior lens capsule, zonules, the ciliary epithe-
lium, inter limiting membrane of the retina and the optic nerve. The main function of
the vitreous is optical Its refractive index is 1.336. The other function is to stabilise the
retina to which it is loosely attached without any potential space. The vitreous is attached to
various intra ocular structures.
The attachments are:
1. Vitreous baseThis is the strongest of all physiological attachments. The
attachment of the vitreous is strongest at ora serrata. The area attached over the
ora is called vitreous base, which spans over the ora serrata about
2 mm in front and equal distance behind. The vitreous base is attached to the ciliary
epithelium and blood vessels at the ora. The basal attachment lasts life long and if
detached following trauma, it takes the pigment epithelium and the blood vessels
alongwith. The posterior vitreous does not have firm attachment, hence is capable of
detachment. Posterior vitreous detachment (PVD) is a common occurrence.
2. Round the foveaThis is a smaller, localised attachment, it is weaker than other
attachments.
3. Round the optic nerveThis is fairly stronger attachment.
4. Round the retinal blood vesselsThis is controversial, so are the weak attach-
ments to the internal limiting membrane of the retina.
5. Hyaloid capsular attachmentIn childhood the posterior lens capsule has a very
strong attachment with the anterior vitreous membrane. The attachment is in the
form of a ring to a natural depression in the anterior vitreous face. This depression is
called patellar fossa or Bergers space.
The adhesion is so strong under 25 years of age that traction on the lens fails to pull the
lens out in toto, thus making intra capsular lens extraction impossible under twenty five years.
The zonules, which are known as tertiary vitreous, are also strong enough to make intra
capsular lens extraction impossible. The adhesion weakens with age and by sixth decade it
become non existent.
In intrauterine life, the hyaloid artery traverses from the optic nerve head to the
posterior part of the lens in the middle of the vitreous to form the tunica vasculosa lentis
that supplies blood to the developing lens, through a space called Coloquets canal that
disappears at birth leaving a narrow empty space which is straight at birth but becomes curved
with age. It is not visible with direct ophthalmoscope. If some part of the hyaloid system has
not been absorbed completely it is visible as fixed opacity in the mid vitreous without any
symptoms, that may be visible as a black dot during retinoscopy.
Pathological adhesion of the vitreous
These are mostly vitreo-retinal attachment. They are found in chorio retinal scar,
peripheral pigment clumps, posterior border of lattice degeneration, borders of
choroidal rupture and borders of coloboma of the choroid, peripheral retinal vessels,
site of retinal periphlebitis.
The shape of the vitreous body follows the shape of the globe. Its volume is less in
microphthalmos. It is not capable of expanding unless something i.e. air or fluid is injected
into its substance. However administration of hypertonic solutions orally or intravenous and
carbonic anhydrase may shrink its volume considerably. Constant pressure on the globe forces
fluid out of the vitreous and the vitreous shrinks. These two methods are employed to reduce
vitreous volume during cataract surgery.
Vitreous once removed from the eye is not capable of replenishment. The space is occu-
pied by aqueous.
The vitreous does not have a true capsule. The peripheral vitreous due to its compact
collagen content is arbitrarily called cortex while the central part is called nucleus.
The vitreous is devoid of any nerve, hence it is not capable of any sensation. It has no
blood supply so it is also not capable of any inflamation. However, vitreous acts as a rich
medium for growth of bacteria and fungi.
The composition of vitreous
Vitreous is 98-99% water to which other solids are dissolved. The solid constituents are
Collagen, hyaluronic acid and soluble proteins. Vitreous has more ascorbic acid than in plasma.
It also containsSodium, potassium, bicarbonates, chloride and phosphates. There are about
fifty enzymes in vitreous, the functions of which are not clear. The collagen is capable of bind-
ing with water, which could be as much as 200 times the weight of the collagen, to form a hydro
gel of highest viscosity.
Development of vitreous
16,17,18
Developmentally vitreous has three phases:
1. Primary vitreous
2. Secondary vitreous
3. Tertiary vitreous
Developmentally vitreous has dual origin it is partly ectodermal and partly
mesodermal.
The primary vitreous starts developing very early and its development is completed by
13 mm stage. It develops in the space between the lens and the retina. During first month of
development cells from the lens and retina form the primary vitreous. The retinal cells domi-
nate over the lenticular cells. The cells from the lens loose their contact from the primary
vitreous due to formation of lens capsule. The cells from the retina continue to contribute
towards development of primary vitreous. The lens is further isolated from the vitreous by
developing tunica vesculosa lentis.
The tunica vasculosa lentis is mesodermal part of primary vitreous. The hyaloid artery,
which is a branch of developing internal carotid, (actually primitive dorsal ophthalmic artery)
enters the optic cup through the embrayonic fissure. The hyaloid artery communicates with
the capillaries on the ventral aspect of the developing lens to form the tunica vesculosa lentis.
The hyaloid system starts disappearing by 65 mm and at birth there is no evidence of the
hyaloid artery only the proximal part of the hyaloid artery remains patent and forms the
central artery of the retina.
The secondary vitreous
The secondary vitreous is the part of the developing vitreous, which lasts for rest of the life. It
is avascular and is secreted by the cells from the inner surface of the optic cup and pushes the
primary vitreous centrally. The primary vitreous gradually disappears leaving no trace except
the Coloquet canal.
The tertiary vitreous
The tertiary vitreous is secreted by the ciliary epithelium. This occupies a triangular area
between the developing ciliary body and the lens. With the development of the eye, the ciliary
body that was very near to the lens, withdraws away from the lens and the fibres become more
clearly defined. The fibres divide into two groups - The anterior part in just behind the iris and
gets attached to the anterior lens capsule, the posterior part runs parallel to the anterior
vitreous membrane and get attached to the posterior lens capsule. These fibers, ultimately
form the zonules of the lens.
Congenital anomalies of the vitreous
In spite of being most voluminous intra ocular structure, the congenital anomalies of the vitreous
are few. The congenital anomalies directly correspond to the stage of development of the vitreous.
The congenital anomalies may be as small and insignificant as vitreous floaters or as extensive
as to replace the whole of the vitreous by mesodermal tissue. The former is very common while
the latter is very rare.
The congenital anomalies mostly involve the mesodermal tissue. They can be:
1. Limited to hyaloid vascular system without hyperplastic changes in vitreous or,
2. They may be associated with hyperplastic changes in the vitreous.
Anomalies of hyaloid vascular system
There are three possibilities:
1. The whole of the artery may be seen to exist between the posterior lens capsule to
optic nerve head. The vessel is attenuated, may contain blood. This is very rare.
2. The anterior end of the artery may persist as a free floating stalk attached to the
posterior lens capsule.
3. The posterior end may remain attached to the optic nerve head.
4. Occasionally the remnants of the posterior part of the tunica vasculosa lentis may
persist as grey opacity in the posterior lens capsule and is called Mittndrof dot. This
is almost asymptomatic, may be visible on oblique illumination, look like a black dot
in pink retinoscopy glow, seen best by slit lamp.
5. The remnants of the posterior part of the hyaloid artery is known as Bergmeisters
papilla. This too is asymptomatic but when large may be mistaken as growth on the
optic nerve. The papilla is made up of central core of vessel surrounded by fibro glial
tissue.
Persistent hyper plastic primary vitreous (see chapter Retinoblastoma)
Symptoms of diseases of vitreous
Diseases of vitreous are rare in children as compared to adults.
The two functions of the vitreous are optical and support to the retina, one or both may
be involved. Moreover diseases of surrounding structure may also involve the vitreous i.e. the
retinal vessels may bleed to cause bleeding in the vitreous.
The vitreous being avascular is not capable of bleeding. The vitreous itself is not capable
of inflamation either, but is generally involved in inflamation of choroid or retina that may be
either in the form of endophthalmitis or vitritis. Vitritis is a condition where inflammatory
cells from choroid, retina or optic nerve invade the vitreous without exudation.
Commonest symptom of vitreous disorder is vitreous floaters. Other symptoms are
- photopsia, a visible ring shaped opacity, erythropsia, diminished vision, sudden painless loss
of vision. The symptoms may be unilateral or bilateral. The vitreous is devoid of any nerve
supply, hence disease of the vitreous alone is painless.
Signs of vitreous disorders
The sings comprise of
1. Diminished vision.
The vitrial causes of diminished vision are:
(i) Large opacities in front of the macula
(ii) Haemorrhage in front of the maculaDiminished vision may range from loss of
one to two lines on Snellens chart or the vision may be reduced to perception of light.
Disorder of vitreous never causes loss of perception or faulty projection.
2. Oblique illuminationOnly a few physical findings of vitreous can be seen on ob-
lique illumination. They are:
(i) Retrolental white mass
(ii) Strands of liquid anterior vitreous
(iii) Less commonly a bloch of fresh haemorrhage can be seen just behind the lens or
in the lower part of the vitreous, when the patient looks down with dilated pupil.
3. Slit lamp biomicroscopyOrdinary biomicroscopy can not focus the whole depth
of the vitreous unless the optics of the eye is modified to bring the various depths of
vitreous within the focus of the slit lamp biomicroscope. This can be brought about by
(i) Goldman three-mirror contact lensIts central part brings the posterior
pole in focus. The mirror with an angle of 55 focuses the vitreous up to ora
serrata, while the mirror with 67 angulation can focus the peripheral fundus.
(ii) Hruby lens this is a pre set concave lens of 55D
(iii) El Bayadi lensThis is a pre set convex lens of + 55D.
Beside these there are other lenses i.e. + 90D or + 78D that are also used to view
the vitreous with slit lamp.
4. OphthalmoscopyBoth direct and indirect ophthalmoscopes are used to examine
the fundus. Indirect ophthalmoscope has added advantage of stereopsis, larger field
and brighter illumination.
5. UltrasonographyBoth A and B scans are used. B scan gives better view than A
scan. Both are useful in opaque media.
6. X-ray. It is indicated only when intra ocular foreign body is suspected. Both AP and
true lateral view should be taken.
7. ElectroretinographyERG has limited role in transparent media. In opaque media,
a non recordable ERG is not an absolute contra indication for vitreous surgery because
a totally detached retina that gives non recordable ERG on successful attachment
may gain some visual function.
8. Fluorescein angiography is indicated in proliferative vascular retinopathy and
cystoid macular edema that is common following vitreous loss.
Vitreous opacities (floaters)
Vitreous opacities are commonest symptom of disease of vitreous. They cause loss of
transparency of the vitreous, may be in the form of scattered dots, strings, sheets. They are
generally mobile, hence called floaters. They may change their position in space in different
direction of movement, may be felt all through the waking hours or may be more visible after
sleep. They are not visible in darkness or when the eyes are closed.
Vitreous opacities can result due to
1. Primary change in the vitreous
2. Secondary changes in the vitreous due to pathology in retina, choroid, and optic nerve.
Primary changes in vitreous
Muscae volitantes
These opacities are so common that they are considered to be physiological. They represent
remnants of primary vitreous. They can be unilateral or bilateral, are not associated with
loss of vision. They are perceived either as scattered dots or dots in string. They are best seen
against light coloured background such as sky or white wall. They have no pathological
significance nor do they require any treatment. Sudden onset of shower of vitreous floaters
should not be taken lightly. All such eyes should be examined with indirect ophthalmoscope,
as it may be the beginning of parsplanitis, retinal break or inflammation of the uvea. Floaters
are common in moderate to high myopia due to degenerative change in the vitreous.
Vitreous degeneration
Vitreous is a semisolid gel, does not show any movement or ripple within its body unless it is
liquefied. Vitreous in a childs eye is more firm and solid than vitreous in senile group.
The liquefication of the vitreous is known as synchisis. It is a common feature of old
age. In children liquefication is seen in high myopia and trauma with or without retained
foreign body. It is also seen following vitreous haemorrhage, endophthalmitis and severe
posterior uveitis. The vitreous looses its semisolid nature and becomes watery in consistency.
It may be so fluid as to flow out during intra capsular lens extraction in age related cataract.
The liquification invariably leads to syneresis or collapse of vitreous. Liquified vitreous
does not require any specific treatment.
Synchisis scintillans
This is a different kind of liquifaction of vitreous seen in younger patients, is bilateral without
much complains. The patient may not be aware of its presence. There is deposition of
cholesterol crystals in the vitreous. The crystals are suspended in the vitreous. They may
remain hidden at the bottom of the vitreous and become visible on movement of the eye like
golden shower. Sometimes they are visible on oblique illumination in clear media. They are
best seen by slit lamp. They are also visible with direct ophthalmoscope. On retinoscopy they
may be visible as cloudy dots. Synchisis scintillans does not require any treatment and
persist life long. They are not to be confused with astroid hyalitis, which is seen in elderly
males. They are accumulation of calcium soaps and are fixed bodies, show minimum
movement.
Vitreous detachment
Normal vitreous does not detach from its attachment. For a vitreous to detach its consist-
ency must change towards fluid rather than semi solid gel. It is more common after sixth
decade but not exceptional under twenty. The common causes of vitreous detachment in
children areMyopia, chronic uveitis, trauma, both concussion and penetrating, vitre-
ous haemorrhage, vitreous disturbance during intra ocular surgery.
According to configuration of the detachment, it has been divided into two broad groups:
1. Posterior vitreous detachment (P.V.D.)
2. Anterior vitreous detachment.
The posterior vitreous detachment is more common than anterior vitreous detach-
ment.
Posterior vitreous detachment can be
1. Small peripheral, which may later increases in size.
2. Infundibular or conical detachmentWhen the vitreous is attached round the
disc and base but separated from internal limiting layer of the retina all round.
3. Globular detachmentWhen the attachment round the disc has been severed but
the basal attachment is maintained.
The posterior vitreous detachment can be simple or with collapse. In simple PVD, the
detached posterior surface retains its spherical shape. In detachment with collapse, the detached
posterior border of the vitreous hangs vertically from the region of its superior base.
Anterior vitreous detachment
The whole of the anterior vitreous cortex is separated from the lens, zonule and base, pushing
the vitreous posteriorly. It can be localised to zonule or lens or both.
Symptoms of vitreous detachment are variable. It may be symptomless. Common
symptoms are photophobia, and floaters in front of the eye or a Weiss ring, which is an
annular vitreous floater due to detachment of posterior vitreous from the optic nerve head.
There is no specific treatment for vitreous detachment. All cases of vitreous detachment
should be examined by indirect ophthalmoscope for possibility of peripheral retinal degenera-
tion and holes, which may require treatment to prevent rhegmatogenous retinal detachment.
Vitreous haemorrhage
Vitreous haemorrhage is in fact an inter vitreal bleeding originating from retinal vessels.
Vitreous being avascular, is incapable of bleeding.
Vitreous haemorrhage is a common causes of painless, sudden loss of vision which
when unilateral may go unnoticed in children. If the cause of the bleeding is local, the
bleeding is unilateral. It is bilateral if the cause is systemic. Vitreous haemorrhage is less
common in children. The commonest cause of bleeding in third and fourth decade is
periphlebitis (Eales disease) which in not seen under fifteen years of age. In fourth decade
onwards incidence of diabetics proliferative retinopathy and venous obstruction, which
are again not seen in children are the main causes of vitreous haemorrhage. However, prolif-
erative retinopathy in juvenile diabetes may on occasion present as vitreous haemorrhage.
The common causes of vitreous haemorrhage in children are:
1. CongenitalPersistent primary hyper plastic vitreous, angiomatosis, Coats disease,
remnants of hyaloid vessels.
2. TraumaBy far trauma is the commonest cause of vitreous haemorrhage in children.
It can be blunt injury or may be penetrating injury. Blast injuries may cause bilateral
vitreous haemorrhage. Rupture of retinal vessels due to vitreo retinal traction, a
vessel passing across a large tear can also bleed.
3. Blood dyscrasiaLeukaemia, purpura and other coagulation disorders can cause
vitreous haemorrhage.
4. InflammationPeriphlebitis is commonest cause of vitreous haemorrhage in third
and fourth decade and parsplanitis is less common cause in that group. Parsplanitis
is more likely to cause haemorrhage in children than periphlebitis.
Miscellaneous
1. Tersons syndrome, where a sub arachnoid haemorrhage near the optic disc may
trickle down to sub hyaloid space.
3. Inadvertent perforation a globe during squint, retinal surgery, retro bulbar or peri
bulbar injection can also cause vitreous haemorrhage.
Symptoms of vitreous haemorrhage
A bleeding away from the posterior pole may be asymptomatic and go unnoticed. Common-
est symptom of a pre macular haemorrhage is sudden, painless loss of vision that may vary
between loss of one to two times on Snellens chart to only perception of light. The patient may
have better vision as the blood settles down only to be reduced when the blood comes in front
of the macula.
One rare occasions, small haemorrhage may present as showers of floaters. In still rarer
conditions there may be erythropsia.
Signs of vitreous haemorrhage
On rare occasions fresh blood in anterior vitreous may be visible on oblique illumination.
Commonest form of presentation is a keel shaped sub hyaloid haemorrhage that
has red colour with round bottom with a horizontal line denoting the upper limit of settled
blood.
A massive intra vitrial haemorrhage may obscure the fundus when seen by direct
ophthalmoscope. The same when viewed by indirect ophthalmoscope makes it possible to see
the retinal periphery that may have the clue to the haemorrhage i.e. tear, degeneration,
disinsertion, parsplanitis.
Fate of vitreous haemorrhage
Vitreous haemorrhage is a self-limiting disease. In four to six weeks the haemorrhage is
absorbed without leaving trace of haemorrhage. However, recurrence of bleeding takes more
time to absorb than original bleeding. Each episode of bleeding lasts longer than the previous
one. Recurrent and non absorbing haemorrhage may lead to formation of vitreous bands and
are called retinitis proliferans. It generally starts near the disc where the central retinal
artery is rich in mesoblastic tissue. If haemorrhage does not clear within six months the condition
is called hemophthalmos where no part of the retina is visible on ophthalmoscopy, retinoscopy
or slit lamp examination. The only useful investigation in such cases is ocular
ultrasonography that may show retinal detachment as well.
Management of vitreous haemorrhage
There is no specific treatment for vitreous haemorrhage. Most of the vitreous haemorrhage get
absorbed by four to six weeks. Some of the new vessels may require closure by laser or cryo-
coagulation. Vitreous haemorrhage not clearing by three months require parsplana
vitrectomy, some may require additional endo laser coagulation. Associated retinal
detachment is treated by standard methods.
Vitreo retinal degeneration
66,67,68,69
These are a group of inherited conditions that manifest in childhood due to close proximity
of retina and lens during development of the vitreous. The vitreous is seldom involved alone in
these degenerative conditions.
The condition can be divided in two broad groups:
1. Those that have only ocular involvement
2. Those that have associated systemic involvement along with vitreo retinal degenera-
tions:
The group I consist of
Stickler syndrome.
Wagners disease.
Favre Goldmann syndrome
Exudative vitreo retinopathy
Snow flake degeneration
The group II Consist of
Marfans syndrome.
Homocystinuria.
Ehlers-Danlos syndrome
Lawrence-Moon-Biedl syndrome.
Most of the conditions in-group I have predominant ocular involvement but may have
systemic involvement as well especially in Stickler syndrome.
Stickler syndrome
It is an autosomal dominant disorder, the children may have skeletal changes similar to
Marfans syndrome. Joint involvement is common, the ocular change consist of vitreo retinal
degeneration that consist of liquified vitreous with collapse giving an optically empty
vitreous. Translucent membrane may develop on the periphery. The retinal changes include
lattice degeneration. There may be sheathing of peripheral retinal vessels. Peripheral retina
shows clumps of vitreo retinal scar. Retinal detachment is common. There is mild to
moderate degree of myopia. Cataract and open angle glaucoma are common complications.
Wagners disease
This is as an autosomal dominant inheritance. The ocular structures involved besides
vitreous are retina, optic nerve and lens. The vitreous changes are similar to those seen in
Sticklers disease without retinal detachment. There may be diminished night vision
with abnormal ERG. The disease is generally bilateral, starts in infancy and continues in
adolescence. Liquification of vitreous is seen after second decade.
Congenital retinoschisis (see retina also page 403)
It is also known as juvenile retinochisis, though the retinochisis is a dominant feature both
vitreous and retina are involved simultaneously. It is commonly seen in childhood, more common
in boys. It is known to be present at birth. The children may be brought to ophthalmologist for
presence of strabismus and/or nystagmus and on examination found to have congenital
retinochisis. The condition grows slowly, there may be periods of worsening in between the
main fundus finding consists of retinochisis. The inner layer of retinochisis is extremely thin,
the blood vessels on the inner leaf are normal. The inner leaf has large hole resulting into
smooth ballooning of immobile inner leaf. The outer layer is difficult to distinguish until it is
detached from the RPE, which happens when the outer layer also develops hole. There may be
some haemorrhage in the vitreous. The posterior border of schisis may show line of pigmentary
demarcation.
The retinoschisis may remain on the periphery or may extend into the macula. The
maculopathy consists of small cystoid spaces with radial lines, which probably represent folds
in the internal limiting membrane. The appearance is called bicycle-wheel pattern, which
does not fluorescence. The Cloquets canal seems to be larger, the vision is diminished if either
the macula is involved or there is retinal detachment.
The condition has x linked inheritance. It is bilateral and ERG shows subnormal B
wave with normal A wave.
Management
There is no specific treatment that may give uniformly satisfactory results. The modalites
tried arePhoto coagulation of outer layer this may regress the condition and the balloon may
flatten. Photo coagulation is more indicated if there is a hole in the outer leaf. Retinal detach-
ment when present, it is treated by standard retinal detachment surgery.
FavreGoldmann syndrome
This is a vitreo retinal degeneration that has night blindness as main symptom. It is an
autosomal recessive disease. It affects boys and girls equally, it is generally bilateral. The
vitreous is liquified, may have peri retinal membrane. Retinochisis and retinal detachment
are seen. The other symptoms are diminished vision, floaters, ERG is sub normal or absent.
Cataract is a common complication. There is no specific treatment.
Familial exudative vitreo retinopathy
This autosomal dominant disorder is also known as Criswick-Schepen Syndrome. The
ocular involvement is generally bilateral and symmetrical. The condition can be present in
two forms:
1. The child is asymptomatic and the condition is detected on routine ophthalmoscopy.
2. The child is brought with squint and found to have diminished vision.
The fundus findings are less in the first group that includes peripheral avascular zone,
arterio venous anastomosis, vitreo retinal adhesion. The condition may be mistaken as
retinopathy of prematurity.
In second groupThe vitreo retinal findings are more extensive and have more similar-
ity to retinopathy of prematurity. This includes retinal and pre retinal haemorrhage, exuda-
tion, fibro vascular bands, retinal fold, retinal detachment.
The children have moderate myopia, the retina may show white with pressure or with-
out pressure.
There is no specific treatment.
Snow flake degeneration
This is rarer than those described above. It is an autosomal dominant disorder that has
myopia and pre senile cataract for which the patient may seek help. On examination it is
found to have following:
Wide spread areas of white with pressure that may have snow flake deposits. Posterior
to snow flakes there may be increased pigment deposit. The peripheral retinal vessels show
sheathing, there may be breaks in the retina leading to detachment.
Lebers congenital amaurosis
70
This is one of the frequent causes of blindness in new born. The child may be blind from birth
or goes blind during first year. Poor vision from early months predispose nystagmus.
Poor vision and nystagmus herald sttrabismus. Parents of these children with squint
and/or nystagmus bring the child to the ophthalmologist to know if the child has vision or
not
71
. Unfortunately they are told that the child has very poor uncorrectable vision. The
child develops a peculiar habit of rubbing the eyes. The condition is autosomal recessive
in nature. Fundus findings are variable, initially the fundi may look normal or may have
pepper and salt like pigmentation all over the retina. Later they may develop bone spicule
pigmentation similar to retinitis pigmentosa, which is not seen in such a young child.
These children generally have high hypermetropia. The pupillary reaction may be
sluggish or paradoxical.
ERG both photopic and scotopic are subnormal. The patients may have keratoconus,
keratoglobus, and cataract. Mental retardation is common.
REFERENCE8
1. Duke Elder S. ; Layers of retina in System of Ophthalmology. Vol. II, pp. 230231,
2. Nancy Anderson Hamming and D. Apple ; Sensory retina in Principles and practice
of Ophthalmology. Vol. I, first Indian edition, pp. 4758. Edited by Peyman G.A.,
Sanders D.R. and Goldberg M.F. Jay Pee brothers, New Delhi 1987.
3. Dutta L.C. ; Anatomy of retina in Ophthalmology, principle and practice. First
edition, pp. 142145. Current Book International, Calcutta 1995.
4. Inderbir Singh ; Visual path way in Text book of human neuro anatomy. Sixth
edition, pp. 229231. Jay Pee brothers, New Delhi 2002.
5. Nema H.V. ; The retina in Anatomy of the eye and its adnexa. Second edition, pp.
3951, Jay Pee brothers, New Delhi 1991.
6. Banumathy S.D. ; Anatomy of the visual path way in Anatomy of the eye. pp. 6568,
Arvind Eye hospital, Maduri.
7. Kumas A. and Nainiwal S.K. ; Macular and sub macular surgery in Clinical practice
in Ophthalmology. First edition, pp. 430, Edited by Saxena S., Jay Pee Brothers, New
Delhi 2003.
8. Khamas B. ; Anatomy retina and vitreous in Modern Ophthalmology. Vol. II, Second
edition, pp. 624629, Edited by Dutta L.C., Jay Pee Brothers, New Delhi 2000.
9. Samant P. M., Hemalini. P. Samant and Nabar S.M. ; Structures of fovea centralis in
Ophthalmology theory and practical. First edition, pp. 164165, Bhalani Publish-
ing House, Mumbai 2004.
10. Miller S.J.H. ; Retina in Parsons disease of the eye. Seventeenth edition,
pp. 911, Churchill Livingstone, London 1984.
11. Dutta L.C. ; Structure of the retina in Ophthalmology principles and practice. first
edition, pp. 143150, Current books international, Calcutta 1995.
12. Duke Elder S. ; Retinal circulation in System of Ophthalmology, Vol. II,
pp. 363377, Henry Kimpton, London 1961.
13. Seal S.K. ; Blood retinal barrier in G.N. Seals text book of Ophthalmology. Fifth
edition, pp. 293, Current Books International, Kolkata 2002.
14. Dutta L.C. ; Capillaries of retina in Ophthalmology principle and practice. First
edition, pp. 149157, Current books International, Calcutta 1995.
15. B. Khamar ; Blood supply of retina in Modern Ophthalmology. Vol. II, second edition,
pp. 624622, edited by Dutta L.C. Jay Pee brothers, New Delhi 2000.
16. Ahmed E. ; Embryology of neuro ectodermal Structures in A text book of Ophthal-
mology. First edition, pp. 4344, Oxford University Press, Calcutta 1983.
16A. Duke Elder S. ; Differentiation of neural ectoderm in System of ophthalmology Vol.
III part I pp. 83, Heury Kimpton London, 1963.
17. Nema H.V. ; The vitreous body in Anatomy of the eye and adnexa. Second edition,
pp-137138, Jay Pee brothers, New Delhi 1990.
18. Jay B. Carrot W. ; Albinism Recent advances. Tran. Ophth. Soc., UK., pp. 100467,
1980.
19. Dutta L.C. ; Albinism in Ophthalmology principle and practice. 1
st
Edition, pp. 476
478, Current Books International, Calcutta 1993.
20. Boger W.P., Peterson R.A. ; Albinism in Manual of ocular diagnosis and therapy.
Third edition, pp. 273, Edited by Deborah Pavan Langston, Lippincot William and
Wilkins, Philadelphia 1991.
21. Duke Elder S. ; Congenital abnormalities of the inner layer of the optic vesicle in Sys-
tem of Ophthalmology. Vol. III, part 2, pp. 628633, Henry Kimpton, London 1964.
22. Duke Elder S. and Wybar K.C. ; Ciliary circulation in System of Ophthalmology.
Vol. II, pp. 345346, Henry Kimpton, London 1961.
23. Mann I. ; Developmental anomalies of the eye. Second edition, 1957. Quoted by Duke
elder in System of Ophthalmology. Vol. III, part II, pp. 614.
24. Kini. M.D. ; Retinal vascular anomalies in Manual of ocular diagnosis and therapy.
Third edition, Edited by Deborah Pavan Langston, pp. 152153, Little Brown, 1991.
25. Tasman W. ; Retinal telangiectasis (Coats disease) in Pediatric Ophthalmology. Vol. I,
Edition two, pp. 612614, Edited by Harley R.D., W. B. Saunders Company, Philadel-
phia, 1983.
26. Haller. J. ; Coats disease in Retina. Vol. II, edition 2, pp. 14431460, Edited by Ryan S.
J., Mosby, St Louis, 1994.
27. Reese A.B. ; Telangiectasia of retina and Coats disease. A.J.O. : 42-1, 1956.
28. Wu Lihteh. ; Coats disease in Current ocular therapy. Fifth edition, pp. 607608,
Edited by Fraunfelder F.H. and Roy H.F., W.B. Saunders Company Philadelphia 2000.
29. Asdourian G. ; Vascular anomalies of the retina in Principle and practice of
Ophthalmology. Vol. II, First Indian edition, pp. 12991306, Edited by Peyman G.A.,
Sander D.R., Goldberg M.F., Jay Pee brothers, New Delhi 1987.
30. Kanski J.J. ; Retinal capillary hemangioma in Clinical Ophthalmology. Second edition,
pp. 407405, Butterworth, London 1989.
31. Aaberg T. M. ; Fluorescein angiography in Principle and practice of Ophthalmology.
Vol. II, first Indian edition, pp. 910911, Edited by Peyman G.A., Sanders D.R., Goldberg
M.F., Jay Pee brothers, New Delhi 1987.
32. Kumar A, Nainiwal S.K. ; Macular and sub macular surgery in Clinical practice in
Ophthalmology. pp. 440441, Edited by Saxenas, Jay Pee brothers, New Delhi 2003.
33. Harmohina Bagga, Chandra Sekhar G. ; Optical coherence tomography in Clinical
practice in Ophthalmology. pp. 122, Edited by Saxena S., Jay Pee Brothers, New
Delhi 2003.
34. Saxena S., Nancy M. Holekamp ; Idiopathic macular hole in Modern Ophthalmology.
Vol. II, second edition, pp. 781, Edited by Dutta L.C., Jay Pee brothers, New Delhi 2000.
35. Cynthia J. Kendal. ; Ultrasound Principles in Ophthalmic echography. First Indian
edition, pp. 122, Jay Pee Brothers, New Delhi 1991.
36. Duke Elder S. ; The light sense in System of Ophthalmology. Vol. VII, pp. 384392,
37. Dark adaptation in Manual of retinal and choroidal dystrophia. First edition, pp.
1315.
38. Caroline R. Baumal and Elizabeth L. Affel ; The electro-retinogram, the electro-oculogram
and dark adaptation in Ophthalmology Secrets. First Indian edition, pp. 3036, Ed-
ited by Vander J.F. and Gault J.A., Jay Pee Brothers, New Delhi, 1998.
39. Sharma P. : Adaptometers in Essentials of Ophthalmology. First edition, pp. 8182,
40. Duke Elder S. : Electro retinogram in Symptoms of Ophthalmology. Vol. VII, pp.
41. Maji A.B., Sharma Y.R., Raj Shekher Y.L. and Nath R. ; Colour vision and colour blind-
ness in Modern Ophthalmology. Vol. II, Second edition, pp. 1035, Edited by Dutta
L.C., Jay Pee Brothers, New Delhi, 2000.
42. Sharma P. ; Electro physiological tests in Essentials of Ophthalmology. pp. 7881,
43. Seal S.K. ; Visual evoked response in GN Seals Text book of Ophthalmology. Fifth
edition, pp. 1416, Current Book International, Kolkata, 2002.
44. Zwaan J.T. ; Does the baby see in Decision making in Ophthalmology. pp. 124125,
Edited by Heuvan WAJ and Zwaan J.T., Mosby, St. Louis, 1998.
45. Dutta H. ; VEP studies in Clinical methods in Ophthalmology. First edition, pp.
149150, JayPee Brothers, New Delhi 1995.
46. Anita Panda ; Visual evoked response in Clinical examination of the eye. First edition,
pp. 168, CBS Publishers, New Delhi, 2003.
47. Samant P.M., Hemalini P. Samant and Nabar S.M. ; Diabetic maculopathy in
Ophthalmology - Theory and practical. First edition, pp. 171, Bhalani Publishing
House, Mumbai, India, 2004.
48. Ahmed E. ; Diseases of the retina in A text book of ophthalmology. First edition, pp.
281, Oxford University Press, Calcutta, 1993.
49. Fischer D.M. ; Viral diseases and retina in Viral diseases of the eye. Edited by Darrell,
R.W; pp. 487-497, Lea and Febiger, Philadelphia 1985.
50. Asdourian G.K. ; Viral retinitis in Manual of clinical problems in ophthalmology.
Edited by Gittinger JW and Asdourian G.K. Little Brown and Co., Boston, 1998.
51. Ashton N. ; Larval granuloma of the retina due to toxocara. Br. Jr. Oph. 44 : 129-148,
1960.
52. Shield J.A. ; Ocular toxocariasis A review. Survey ophth. 28 : 361-381, 1984.
53. Kini M.D. ; Diabetic retinopathy in Manual of ocular diagnosis and therapy. Third
edition, pp. 155, edited by Deborah Pavan Langston, Little Brown.
54. Tasman W. ; Retinitis pigmentosa in Pediatric Ophthalmology. Vol. I, Second edi-
tion, pp. 610-612. Edited by Harley, R.D., W.B. Saunders Company, Philadelphia 1983.
55. Fishman G. ; Variants of retinitis pigmentosa in Principles and practice of Oph-
thalmology. Vol. III, First Indian edition, edited by Peyman GA, Sander DR and
Goldberg MA. Jay Pee Brothers, New Delhi, 1987.
56. Asdourian GK ; Retinitis pigmentosa in Manual of clinical problems in clinical
Ophthalmology. pp. 115117, Edited by Gittinger JW and Asdourian GK. Little Brown,
Boston, 1988.
57. Carr, RE, Siegel IM ; Unilateral retinitis pigmentosa. Arch.oph. 90:21,1973.
58. Venkatesh P., Verma L., Tewari H.K. ; Fundus flavimaculatous (Stargardts dystrophy)
in Clinical practice in Ophthalmology, First edition, pp. 339. Edited by Saxena S.,
Jay Pee Brothers, New Delhi 2003.
59. Schepens C.L. ; Retinal detachment and allied diseases. WB Saunders Company, Phila-
delphia, 1983.
60. Namperumalswamy P., Madhvan G., Hirode Dwarkanath D. and Lal S. ; Retinal de-
tachment in Modern Ophthalmology. Vol. II, Second edition, Edited by Dutta L.C.,
pp. 650674, JayPee Brothers, New Delhi, 2000.
61. Johnson R.L. ; Retinal breaks in Retina, vitreous and choroid clinical procedures.
pp. 54, Butterworth - Heinemann, Boston, 1995.
62. Dutta L.C. ; Rhegmatogenous retinal detachment in Ophthalmology principle and
practice. First edition, pp. 174, Current Books International, Calcutta, 1995.
63. Duke Elder S. and Wyber K.C. ; The vitreous body in System of Ophthalmology.
Vol. II, pp. 294-309, Henry Kimpton, London, 1961.
64. Nema H.V., Singh V.P. and Nema N. ; The vitreous in Anatomy of the eye and its
adnexa. Second edition, p. 36-38, Jay Pee Brothers, New Delhi, 1991.
65. Pahwa J.M. and Billore O.P. ; Anatomy of the vitreous in Vitreous. First edition, pp. 1-
9, Oxford and IBH Publishing Company, New Delhi, 1985.
66. Pahwa J. M. and Billore O.P. ; Vitreo retinal degeneration in Vitreous. First edition,
pp. 7581. Oxford and IBH Publishing Company, New Delhi.
67. Seal SK. ; Hereditary vitreo retinal degeneration in G.N. Seals Text book of Oph-
thalmology. Fifth edition, pp. 287, Current Books International, Kolkata, 2002.
68. Kanski J. J. ; Vitreo retinal degeneration in Clinical Ophthalmology. Second edition,
pp. 381384. Butterworth international, 1989.
69. Tasman W. ; Disease of the retina and vitreous in Pediatric Ophthalmology. Vol I,
pp. 599602, Edited by Harley R.D. W.B. Sanders company Philadelphia, 1983.
70. Tasman. W. ; Lebers congenital amaurosis in Pediatric Ophthalmology. Vol. I, edition
II, pp. 611, Edited by Harley R.D. WB Saunders Company Philadelphia, 1983.
71. Zwaan J. T. ; Does this baby see in Decision making in Ophthalmology. First indian
edition, pp. 124, Edited by Heuven WAJ And Zwaan J.T., 1992.
72. Verma. L., Ravi K. Nainiwals and Tewari H.K. ; Optical coherence tomography. DOS
times 8: 26-27, 2002.
73. Gupta Sonal, Kader M.A, RamKrishnan R. ; Optical Coherence Tomography: A new
Tool for glaucoma diagnosis. Journal of Arvind eye care system. Vol. III, 12 3-9, 2003.
74. Tewari H.K., Wagh V.B., Parul Sony, Venkatesh P., Singh. R. : Macular thickness
evaluation using the optical coherence tomography in normal Indian eyes. Ind. Jr. Oph.
52-199-204, 2004.
CHAPTER 12
Retinoblastoma
1,2,3,4
RETNOBLA8TOMA
Retinoblastoma is most common intraocular malignancy of early childhood. It is more
frequently encountered malignancy of childhood than rhabdomyo sarcoma, which is com-
monest orbital neoplasm in children. Retinoblastoma is uniformly fatal
5
if not treated. When
treated early i.e. at intraocular stage without extension, five years survival rate is as high as
90%.
A. Incidence of retinoblastoma varies between 1 in17.000 to 1 in 30.000 per live births
6,7
.
However incident is becoming more frequent. There are many factors for these upwards swing
in frequency i.e.
1. Change in environment.
2. Ambient radiation.
3. Higher survival rate in persons who had heritable retinoblastoma and have passed
the gene to the next or second generation.
4. Increased awareness among parents about retinoblastoma.
5. Reduction in neonatal death.
It is equally common among males and females. No race is immune to retinoblastoma.
However there may be clustering of gene in some communities
7
. It is detected early in devel-
oped countries where cure rate is high. It is detected late in under developed countries obvi-
ously due to lack of awareness, lack of diagnostic facilities.
B. About seventy percent of tumours are unilateral. In bilateral cases, the tumour
need not be symmetrical in both eyes, one may be more advanced than the others.
Bilateral cases present earlier than unilateral. Average age of presentation of bilat-
eral cases is twelve months and that of unilateral is twenty four months. In under devel-
oped countries children with retinoblastoma are brought for treatment late, generally with
extra ocular metastasis and poor prognosis. Children with positive family history of
retinoblastoma are brought very early. They are often examined for its evidence in neonatal
age. Incidence of retinoblastoma shows a sharp decline after three years of age. It be-
comes very rare after seven years of age. However retinoblastoma has been reported in
adulthood. The two possibilities in such presentation are:
471
1. The adult had retinoblastoma in childhood which has under gone spontaneous re-
gression and only a scar is left.
2. On rare occasion the tumour has remained dormant and shown activity later.
C. Retinoblastoma is thought to be a congenital tumour, which may be seen in some
cases at birth if looked for specifically.
Contrary to belief, retinoblastoma is a slow progressive neoplasm. So long it is intra
ocular it takes months to years to become extra ocular. Once the tumour becomes extra ocular
distant metastasis is rapid and fatal.
D. Heredity
Heredity in retinoblastoma.
It is confusing to an average ophthalmologist who is not trained in intricacies of genet-
ics. Some of the salient features of heredity in retinoblastoma are:
1. Retinoblastoma is inherited as autosomal dominant trait
3
2. 60% of retinoblastomas are non hereditary.
3. 40% are hereditary.
4. Incidence of spontaneous mutation is high
5. Both somatic and germinal mutation are met with in retinoblastoma
8
6. Patients with germinal mutation are capable of passing the gene to the offspring.
7. Sporadic cases are generally unilateral
8. Bilateral cases are mostly hereditary
9. 10% to 20% unilateral cases are hereditary
10. 94% of all cases of retinoblastoma are sporadic.
11. Only 6% of all cases of retinoblastoma have family history.
12. To summarise
(a) 2030% are bilateral and hereditary
(b) 1020% unilateral and hereditary
(c) 5560% unilateral non hereditary.
(d) None are Bilateral and non hereditary.
13. Genetic mutation is due to chromosome 13 in region of 13q 14
9
.
Genetic counselling is assuming more importance in management of retinoblastoma
because more children survive to reach reproductive age. If any of the parents is affected with
either familial retinoblastoma or bilateral retinoblastoma chances are that their offspring will
have 50% chances of inheriting retinoblastoma. Risk of passing the gene to next generation is
greatly reduced if the children of parents suffering from familial retinoblastoma are healthy.
Clinical Presentation of retinoblastoma depends upon
Position of the lesion, size of the lesion, its extension in side the globe, duration of the
growth, extra ocular extension and family history.
RETINOBLASTOMA 473
Traditionally retinoblastoma has been divided in to following stages.
A. Stage of quiescence
B. Stage of white reflex
C. Stage of secondary glaucoma
D. Stage of extra ocular extension
E. Distant metastasis
All eyes need not go through the above sequence always. A child may pass in to stage of
glaucoma or even extraocular extension without going to stage of white reflex in the pupillary
area ( Leucokoria) if the growth is near the disc.
Ellsworth
10
has divided retinoblastoma into five groups that spread over clinical Stages
A & B for better planning of management of retinoblastoma. Prognosis depends upon the
group in which the tumour belongs. Group I has best prognosis.
Group Number of Size Position Prognosis
Tumours
I A Single < 4DD At or behind equator Very favourable
B Multiple > 4DD Behind equator Very favourable
II A Single 4-10 DD At or behind equator Favourable
B Multiple 4-10 DD Behind equator Favourable
III A Any Any Anterior to equator Doubtful
B Single < 10 DD Behind equator Doubtful
IV A Many > 10 DD Anywhere Unfavourable
B Any Variable Anterior to ora Unfavourable
V A Single Massive Half of retina Most unfavourable
B Vitreous Seeds Most unfavourable
PRE8ENTATON
Children with retinoblastoma or suspected to have retinoblastoma seeking medical aid
belong to three age groups.
A. Between 20 to 30 months these are generally unilateral and non hereditary
B. Round about 15 months of age these are generally bilateral and hereditary.
C. Within few days of birth, these infants belong to families known to harbour retino-
blastoma.
Commonest mode of presentation in first two age groups is white reflex in pupillary
area Leucokoria: unilateral white reflex is by far most common, even in cases of bilateral
involvement one eye has whiter reflex unlesss both eyes have equally advanced growth. The
white reflex is generally detected by parents. The colour of the reflex is due to reflection of
light from the tumour that lacks pink hue of normal retina. The growth is raised from the
surface of retina. Sometimes white reflex is visible only when the child moves the eye from
side to side. Second mode of presentation is strabismus which is commonly esotropia this
makes detailed fundus examination with indirect ophthalmoscope mandatory.
The child may be brought with red and watery eyes, the parents may correlate red-
ness to some trivial, sometimes, imaginary injury. By the time the child develops photophobia
the eye has passed in to stage of secondary glaucoma.
Proptosis is a late presentation and means extra ocular extension.
EXAMNATON OF THE EYE NCLUDE8
A. Flash light examination
1. For conjunctival and ciliary congestion
2. Corneal size
3. Anterior chamber for pseudohypopyon
4. Pupil for size and colour leucocoria
5. Iris
(a) Heterochromia
(b) Nodule
(c) Neovascularisation
6. Lens. The lens is examined for its transparency and retro lenticular mass which is
mistaken as cataract. Lenticular opacities are late to develop in retinoblastoma.
B. Slit lamp examination. To confirm findings of torch light examination the eye
should be examined under magnification preferably with a slit lamp. In very small children it
is better done under anaesthesia either with a hand held slit lamp or with an operating
microscope.
C. Indirect ophthalmoscopy, both eyes should be examined with complete mydriasis
and sclera indentation under general anaesthesia.
D. Recording of intra ocular tension and measurement of corneal diameter should also
be performed under general anaesthesia, Anesthetics that cause rise of IOP should be avoided.
E. B.scan ultrasonography. If the child does not co-operate it should also be done
under general anaesthesia. Retinoblastoma appears as a solid mass arising from retina with
high reflectivity.
F. Other Investigation
1. X-ray orbit for evidence of intra ocular calcification.
2. CT for intra cranial extension, condition of optic canal, presence of Trilateral
Retinoblastoma, intraocular calcification.
3. M.R.I
11
. This confirms intraocular calcification, shows extra ocular extension includ-
ing optic nerve infiltration, MRI of brain show presence of Pinealoblastoma (trilateral
retinoblastoma)
4. Fine needle biopsy
RETINOBLASTOMA 475
G. Systemic examination for
1. Distant metastasis,
2. Primaries that may produce proptosis mimicking retinoblastoma
H. Fundus examination of parents grand parents, in case of bilateral tumours.
DFFERENTAL DAGNO88 OF RETNOBLA8TOMA
Differential diagnosis of retinoblastoma comprise of two groups of disorders
A. The first group comprises of the most important causes that present with white re-
flex in the pupillary area. Infact it is best to suspect each case of white reflex in the pupillary
area specially if it is retrolental to be retinoblastoma unless proved otherwise. It should be
remembered that retinoblastoma is a life threatening condition.
B. Second group consists of those diverse conditions that produce proptosis with or
without white reflex in the pupillary area.
CONDTON8 THAT PRODUCE WHTE REFLEX CAN BE GROUPED
NTO
A. Lenticular
B. Retrolenticular
C. Pre lenticular
Lenticular causes of white reflex comprise of congenital cataract, developmental cata-
ract and traumatic cataract.
Retrolenticular causes of white reflex comprise many diverse conditions that give
white reflex in pupillary area traditionally they were grouped as Glioma and Pseudoglioma.
This is due to early misconception that retinoblastoma was a glioma. It is more logical to group
them as
Retinoblastoma and non-retinoblastoma
The non retinoblastomic causes could be congenital anomalies, iatrogenic (toxic ), in-
flammatory and vascular.
NON RETNOBLA8TOMC RETROLENTAL CAU8E8 OF WHTE RE-
FLEX N PUPLLARY AREA
4,12,13,14
A. Congenital
1. Persistent hyperplastic primary vitreous
2. Retinal dysplasia
3. Congenital retinoschisis
4. Incontenentia pigmenti
5. Large coloboma of choroid
6. Congenital retinal fold
7. Large opaque nerve fibre
B. Iatrogenic
Retinopathy of prematurity
C. Vascular
1. Persistent hyperplastic primary vitreous
3. Coats diseases
4. VonHippels disease
5. Lebers miliary aneurysm
D. Inflammatory
1. Ocular toxocariasis
2. Metastatic endophthalmitis
3. Retino choroiditis
4. Metastatic retinitis
E. Newgrowths
1. Meduloepithelioma
2. Leukaemia
3. Choriodal hemangioma
F. Miscellaneous
1. Norries disease
2. Familial exudative vitreo retinopathy
NFLAMMATORY CAU8E8 OF WHTE REFLEX N PUPLLARY
AREA CAN BE
A. Prelenticular exudative deposits in front of the lens in the form of membrane obscur-
ing lens i.e. occlusiopupillae.
B. Complicated cataract.
C. It can produce retrolental pathology mimicking retinoblastoma. These causes are
1. Posterior cyclitic membrane
2. Parsplanitis
3. Metastatic endophthalmitis
4. Parasitic endophthalmitis.
RETINOBLASTOMA 477
D. The vascular causes of white reflex that also produce retinal mass are
1. Coats disease
2. Lebers miliary aneurysm
3. Angiometosis retinae
E. Proptosis itself is a late mode of presentation. It is produced only when retinoblastoma
breaks the scleral barrier. Once the growth is extra ocular, its spread inside the orbit and
distant metastasis is fast.
Other cause of proptosis that simulate retinoblastoma are -
1. Secondries
(a) Leukaemia
(b) Neuroblastoma
2. Inflammatory
(a) Orbital cellulitis
(b) Cavernous sinus thrombosis
(c) Sub periostial abscess
(d) Parasitic cyst
(e) Panophthalmitis
3. Primary tumourRhadbomyosarcoma
PATHOGENE88 OF RETNOBLA8TOMA
Pathogenesis of retinoblastoma is poorly understood. It may have hereditary predispo-
sition, however, majority of them are sporadic. Advanced parental age and history of can-
cer in excess of demographically expectation in the family and 13q deletion syndrome
are some other predisposing factors
9
.
Primitive precursor of photoreceptors which normally produce rods and cones under go
anaplastic change to give rise to retinoblastoma cells in the outer layer of retina.
Endophytic tumours originate in inner nuclear layer while exophytic tumours
originate in external nuclear layer.
The retinoblastoma cells are semi columnar cells with large dense nuclei and scanty
cytoplasm. The retinoblastoma cells have a tendency to arrange themselves in the form of
rosettes, fleuerettes and pseudorosettes. There are two types of rosettes i.e. Flexner-
Wintersteiner, and Homer-Wright rosettes.
A. Flexner-Wintersteiner rosettes
7
are typical of retinoblastoma. The cells are
arranged in circular fashion leaving a central empty lumen, that is lined by a clear membrane
over which the cells are arranged with large hyperchromic nuclei away from the lumen figure.
Multiple rosettes are seen to be scattered all over the field. The membrane over which the cells
are arranged is similar to the external limiting membrane of retina. The membrane may
be penetrated by blunt processes that are similar to photoreceptors. The lumen contains
acid mucopoly saccharides.
B. Homer-Wright rosettes. These rosettes are less commonly found in retinoblastoma
as compared to Flexner-Wintersteiner rosettes they are more common in neuroblastomas
and medulo blastomas. The main difference is that there are no acid mucopoly saccharides
in the lumen. It is said -
More are the rosettes better is the prognosis.
C. Fleurettes. These are yet another type of retinoblastoma cell arrangement without
having a central lumen, the cells are more elongated which are arranged like bouquet in a
radiating fashion not forming a complete rosettes. The cells resemble photoreceptors more
than rosettes. Sometimes the whole of tumour may be composed of fleurettes only. Fluerettes
represent more benign character than rosettes. Tumours composed of only such cells have
been termed Retinoma or Retinocytom.
D. Retinoma or retinocytoma
8.16
. This is a rare benign tumour of retina due to
mutation of retinobastoma gene. Rarely it may under go malignant change. The retinomas
generally do not form rosettes. They generally form fleurettes. Calcification is common. The
growth is translucent, homogenous, inter retinal mass. Sometimes the term retinoma is used
for spontaneously arrested retinoblasoma with out necrosis.
E. Pseudo rosettes. A rosette is called a pseudo rosette if the retinoblastoma cells are
arranged round a blood vessel that replaces the central lumen of a true rosette. The cells
receive nourishment from these blood vessels and show a luxuriant growth that may be too
much for blood supply, resulting in to ischemic, coagulative necrosis of tumour cells away
from the blood vessels where calcium is deposited that show up on x-ray, ultra sound, CT or
MRI. This ischemic necrosis is most probable cause of spontaneous regression
4,8,16
is met with
in one to three percent of cases. Other hypothesis is that spontaneous regression is an
immune response
15
. In some cases of spontaneous regression, recurrence has been reported.
Genetic predisposition is unaffected by spontaneous regression.
Spontaneously regressed tumour looks like a healed scar this makes examination of
fundi of parents and siblings by indirect ophthalmoscope an important clinical evaluation.
Specially if the child has bilateral involvement.
There are two clinical presentation of spontaneous regression.
1. A flat scar similar to scar of post radiation treatment of retinoblastoma.
2. Phthisis bulbae of effected eye due to extensive necrosis of tumour infiltration of optic
nerve. These growth have good prognosis.
Endophytum retinoblasoma arises from inner nuclear layer and grows preferen-
tially towards vitreous as a pink white well demarcated mass. Calcium deposit over the
mass is common, there may be new vessel formation on the mass, vitreous seeding or cells are
common. These are more prone to optic nerve involvement. Differential diagnosis consists of
various inflammatory causes.
Exophytum retinoblastoma this type of tumour arise from outer nuclear layer and
spread towards the chorid, lifting the retina, initially causing a retinal detachment and have
more haemato genous spread. The tumour is not visible with ophthalmoscope. Differential
diagnosis comprise of Coats disease, retinal capillary haemangioma and retinal de-
tachment.
RETINOBLASTOMA 479
Differentiation of retinoblastoma into endow or exophytum is limited to early stages. In
late stages it is very difficult to separate them from each other.
8PREAD OF RETNOBLA8TOMA
Retinoblastoma may have multicentric origin in the same eye. The lesions are inde-
pendent of each other, may be of same duration or varied duration. In bilateral cases the other
lesion has no connection with the fellow eye. However there may be seeding from one of the
growths into the vitreous which may get deposited any where on the retina or may be carried
into the anterior chamber and implanted on the iris as nodule, may clogs the angle or may be
deposited at the bottom of A.C. as Pseudohypopyon. There may be heterochromia of the
iris, formation of nodule on the iris or neovascularisation of the iris, leading to second-
ary glaucoma. It takes few months for retinoblastoma to become extra ocular.
EXTRA OCULAR 8PREAD MAY BE
A. In the orbit. This happens when the tumour breaches the sclera. Orbital deposits
produce. Proptosis, chemosis, extra ocular muscle involvement, conjunctival infil-
tration and lid infiltration. Once tumour cells are picked up by conjunctival vessels
haematogenous spread occurs. As orbit has no formed lymphatic, logically there should not be
any lymphatic spread but lymphatic spread to preauricular and sub mandibular
lymphnodes are common. They are secondary to conjunctival and lid involvements, these
structures are very rich in lymphatic.
B. In the optic nerve. There may be direct infiltration of the optic nerve. Initially it is
prelamilar, once it passes through the laminacribrosa, it may either spread along the central
retinal vessels or may infiltrate the pial vessels and get deposited in the subdural and
subarachnoid space from where it reaches the cerebro spinal fluid and is carried into the
brain and spine. Chances of optic nerve involvement are more in large endophytum and in
the stage of secondary glaucoma.
C. In the choroid. The choroid lies flush with retina, it is highly vascular hence its
chances of being infiltrated by tumour cells are more than that of optic nerve. Once the choroid
is invaded haematological spread is rapid, so is orbital extension.
D. Trilateral Retinoblastoma is infiltration of retinoblastoma in the mid line in the
Pineal body in case of bilateral retinoblastoma before five years of age. It may be suprasellar
or parasellar. Histopathologically it has appearance similar to retinoblastoma in contrast to
distant metastasis which do not have characteristic of retinoblastoma. The trilateral
retinoblastoma is best seen on CT
16
.
E. Second Neoplasm in Retinoblastoma If the child survives the first retinoblastoma
he still has chances of developing second neoplasm unrelated to original retinoblastoma.
Common tumours being osteogenic sarcoma, cutaneous melanoma, fibrous histocystoma
and other sarcomas. Second neoplasm is more common in bilateral cases, average age being
13 years but can occur at any age, radiation may also cause second neoplasm.
15,17
.
OTHER CONDTON8 THAT PRODUCE RO8ETTE8
Rosettes are hall mark of diagnosis of retinoblastoma. However , there are other condi-
tions that produce rosettes can be malignent or benign they are:
Medulla epithelioma, neuroblastoma, retinal dysplasia, incontinentia pigmenti,
and retinoma.
MANAGEMENT OF RETNOBLA8TOMA
Management of retinoblastoma consists of
A. Definite diagnosis of the condition. In the past eyes have been removed due to missed
diagnosis of non retinoblastoma as retinoblastoma.
B. To treat the tumour.
C. Prevent spread of tumour
D. Treat distant metastases
E. Prevent recurrence
F. Retain as much vision as possible.
G. Genetic counselling
H. Management of second primary
TREATMENT OF THE NEOPLA8M
Depends upon
A. Size, number and position of the tumour as per Ellsworth classification group I to III
B. Is it still intraocular
C. Has it become extra ocular
(i) is there evidence of optic nerve involvement
(ii) is there orbital deposit
(iii) is there distant metastasis.
Bilateral retinoblastomas are more difficult to manage than unilateral, but unilateral
retinoblastomas generally present late hence are more advanced when seen for the first time.
In cases belonging to Ellsworth group I to III local treatment with Cryo,
photocagulations, radioactive cobalt plaque or controlled radiation may be sufficient.
Cryotherpy is suitable only for tumours anterior to equator.
THE ENUCLEATON 8 A DEFNTVE TREATMENT N
CA8E8 OF
A. The tumour is still intra ocular.
B. Uniocular
RETINOBLASTOMA 481
C. There is gross loss of vision
D. In bilateral cases, one eye is always more advanced than the other, enucleation is
advocated for eye containing more advanced tumour.
E. Bilateral enucleation is tried only if there no salvageable vision in either eye or other
modes like chemotherapy or radiation are not possible.
While enucleating all efforts should be made to cut the optic nerve as far from the globe
as possible and subject it for histopathological examination. Infiltration of distal end of optic
nerve denotes intra cranial extension. The outer surface of the enucleated eye ball should
be closely scrutinised for evidence of breach in the sclera. In no case should an eye contain-
ing retinoblastoma be eviscerated. It is good practice to subject all materials removed by
evisceration in children for any cause for histopathology to exclude masquerade syndromes
18
.
Where a retinoblastoma may present as endophthalmitis or posterior uveitis.
RADATON
4
A. External beam radiation
B. Plaque radiation
External beam radiation is the mainstay of treatment of retinoblastoma. It can be used
as eye preserving device in.
1. Moderate isolated tumour
2. Small multiple tumours
3. Involvement of optic nerve
4. Bilateral cases
5. Cases that do not respond to local treatment by photocogulation or cryotheraopy
Advantages of liner accelerator external beam radiation is that the eye under radiation
is least damaged and the other eye can be protected. The eye is radiated for five days a week
with daily dose of 250 cGy daily, total dose ranges from 2500 to 4500 cGy ( 4000-5000 rads) All
bilateral cases should be subjected to radiation either alone or in combination with chemo-
therapy. Eyes with vitreous seeds do not response well to radiation. Side effects of
irradiation have been divided in to
1. Short term
2. Long term effects.
The short term complications are:- redness of the conjunctiva and skin, madarosis,
dry eye.
Long term side effects are radiation cataract, radiation retinopathy and optic
neuropathy, dry eye, arrest of orbital growth and second neoplasm.
B. Plaque radiations
4
. This mode of treatment consist of suturing a radio active plaque
on the sclera over the tumour commonly used plaques are iodine 125, iridium 192, ruthenium
106. Radon and cobalt 60 are no more popular plaques. The treatment with radioactive plaque
is indicated for eyes with relatively large, localised, isolated nodule without involvement of the
optic nerve or macula. This method can be used either as primary treatment or in case that
have failed to respond to: Cryotherapy, photo coagulation or external beam radiation. The aim
is to impart radiation of 40 cGy to the apex of the tumour. It should be remembered that the
tumour base that is nearest to the plaque gets far more radiation than the apex. The plaques
are custom built for each eye. The method has very few complication i.e. post radiation cataract
during first year . A thin macular exudate may develop after months.
The treatment period with plaque period is short that is 2 days as compared to exter-
nal bean that requires five weeks
16
.
CHEMO THERAPY
Various chemotherapeutic agents have been used with variable cure rate and compli-
cations. Each agent has its own route of administration i.e. Oral, intra muscular, intra venous
and intrathecal. Most commonly used drugs are Vincristine, ActinomycinD and
Cyclophosphamide. Other drugs used are methotrexate, doxorubicin, carboplatin. The dose
and frequency should be adjusted in consultation with pediatric oncologist.
PROGNO88
A. Untreated retinoblastomas are uniformly fatal. Death occurs within two to three
years of detection of tumour in such cases.
B. Prognosis is largely influenced by cell pattern of the growth :
1. Tumours composed of entirely fleurettes have excellent prognosis.
2. Tumours with rosettes have better prognosis than without rosettes.
3. Tumours with abundant Flexner Wintersteiner cells have good prognosis but
less than those which have fleurettes only.
4. Tumours with no rosettes have the poorest prognosis
C. Prognosis also depends upon infiltration of the tumour :-
1. Optic nerve infiltration
(a) No infiltration:- mortality is about 8%
(b) Invasion upto laminacribrosa : the mortality goes to 15%.
(c) Infiltration beyond lamina but confined to the cut (scleral) end : Mortality is
44%.
(d) Invasion into the cranial end the mortality is as high as 65%.
(e) Trilateral retinoblastoma :- cent percent mortality, even with multi modality
treatment.
2. Choriod
A. Slight infiltration:- mortality is about 25%
B. It rises to 65% in heavy infiltration
C. Mortality is 80% to 100% if the sclera is breached.
RETINOBLASTOMA 483
3. Distant metastasis have uniformly fatal out come even with best treatment due
to multiple organ involvement and side effects of chemotherapy and radiation.
OTHER PARAMETER8 U8ED TO EVALUATE PROGNO88 N
RETNOBLA8TOMA ARE
A. Clinical grouping
16
1. Early lesions
2. Moderately advanced lesion
3. Advanced lesion
4. Distant metastasis
B. Histological classification
19
1. Resse Ellosworth Classification.
2. Essen prognosis Classification
4
3. Tumour node metastasis system
20
4. St. Judes hospital classification
Out of above systems most widely used system are Resse Ellosworth classification and
tumour node metastasis evaluation.
DFFERENTAL DAGNO88 OF WHTE REFLEX N PUPLLARY AREA{
LEUCOCORA}
Retinoblastoma is the most important cause of leucoria in children as far as loss of
vision and life expectancy is concerned. Hence the differential diagnosis should be between:-
A. Retinoblastoma (Glioma) and
B. Non retinoblastoma ( Pseudo glioma)
Most of the non retinoblastomic causes are sight threatening but not life threatening.
They can be : Prelenticular Exudate in pupillary area
Lenticular Cataract congenital or traumatic
Retrolenticular See page 475 also
1. Vascular Retrolental fibroplasia
Persistent Primary hyper plastic vitreous
Coats disease.
2. Inflammatory Posterior cyclitic membrane.
Endophthalmitis
Parasitic granuloma
Metastatic retinitis
3. Neoplastic Medullo epithelioma, leukaemia.
4. Retinal Conditions Retinal dysplasia, Incontenintia pigmenti, massive
retinal fibrosis, congenital non attachments of retina,
secondary retinal detachment, juvenile retinochisis,
large coloboma uvea.
LENTCULAR CAU8E8 OF WHTE REFLEX N PAPLLARY AREA
A. Congenital Cataract. There should be no difficulty in diagnosis of congenital cata-
ract. These are :
1. Generally bilateral
2. Positive family history is frequent.
3. Corneal diameter is within normal limits with normal AC in depth and contents.
4. Pupil are central circular and react brisk to direct and indirect light stimulus.
5. Generally there is no history of prematurity, low birth weight or prolonged adminis-
tration of oxygen.
6. The third Purkenjes image is present, fourth may be absent if the lens is fully opaque.
7. Intra ocular tension is normal.
8. In case of incomplete cataract the fundus is visible through widely dilated pupil.
9. Ultra sonography of retrolential structure is normal.
B. Traumatic cataract. These are still easier to diagnosis, if there is a clear cut history
of injury : penetrating or blunt. However the child may hide history of trauma or parents may
co-relate presence of white reflex in pupil to some trivial injury this is more common in
retinoblastoma than in any other cause. Redness, raised intra ocular tension and presence of
flakes of cortical matter in AC lead to more confusion which are best settled by B scan ultra
sonography and CT. Traumatic cataract does not show intra ocular classification but may
reveal unsuspected intraocular foreign body.
RETRO LENTCULAR CAU8E8 OF WHTE REFLEX N
PAPLLARY AREA
A. Retro lental fibro plasia (Retinopathy of prematurity)
21,22,23,24
This is a vasoproliferative retinopathy seen in premature infants with low birth weight
who have been on hyper braic oxygen for more than seven days. However with lesser birth
weight and shorter gestational age, the chances of getting retrolental fibroplasias is more. It is
less common in children born after 32 weeks of gestation and birth weight more than 1500gms.
Twining and maternal diabetes may be contributory factors. It is a bilateral condition seen
equally in boys and girls. It is incidence is less in developing countries only due to the fact that
most of the children do not get oxygen therapy and die before the condition develops. However
prematurity has superseded oxygen as chief cause of retinopathy of prematurity. Hence all
children under 1500gms should be examined by indirect ophthalmology after six weeks of
birth irrespective of oxygenation.
RETINOBLASTOMA 485
Early (acute Phase) is mostly self limiting in 90% of cases. In early retinopathy of pre-
maturity normal development of retina goes awry and there is proliferation of abnormal new
vessels which lead to fibrous proliferation. To understand retinopathy of prematurity it is
better to recapitulate the development of retinal vasculature and effect of oxygen on it.
1. In intrauterine life normal vascularisation of retina begins at optic disc and spreads
towards the periphery in second half of gestation. The temporal periphery is last to get
vascularised i.e. by about 36 weeks. However it may take few more days post natal for the
temporal periphery to get vascularised fully.
2. In a child born before 32 weeks the vessels are far too short of the periphery. It may be
noted that rarely vessels may reach upto periphery even in a premature child, such a child will
not develop RLP.
3. The vessels in pre term child are immature and are sensitive to high concentration of
oxygen.
4. Toxic effect of hyper baric oxygen has two phases.
(a) Oxygen due to some ill understood mechanism causes spasm and obliterate under
developed vessels. Obliteration of vessels interrupt further growth of the vessels. At this stage
a mesenchymal ridge in the form of complete ring or a broken ring is formed all round. The
ridge is more prominent on the temporal periphery.
(b) The next phase is conversion of the mesenchymal ridge in to an arterio venious
shunt. New capillaries sprout from these shunts anterior to the mesenchymal shelf. In milder
form the neovascularisations spontaneously regresses. This type of change is called early
retinopathy, while a sever from is called late retinopathy. There is formation of
microaneurysms and new vessels that may invade the vitreous bleed resulting in traction
vitreo retinal bands leading to traction detachment in the form of a retrolental mass that gives
a white reflex in pupillary area. It takes about ten weeks for retinopathy of prematurity to
develop.
NTERNATONAL CLA88FCATON OF RETNOPATHY OF
PREMATURTY
The classification consists of five stages which are :-
A. Stage of line of demarcation. Here a white tortuous line running parallel to the
ora is seen. The line is flat on the retinal surface, mostly on the temporal side. It divides retina
in to an anterior avascular area and a vascularised retina.
B. Stage of ridge formation. The line of demarcation thickens in width and height .
Few vessels on the vascularised retina form localised neovascular tuft but there is no fibro
vascular growth from the ridge.
C. Stage of fibro vascular proliferation. In this stage extra retinal fibro vascular
proliferation is seen on thickened ridge. The retinal blood vessels become tortuous and dilated.
There may be retinal or vitreous haemorrhage.
D. 1. Stage of sub total temporal retinal detachment without macular involvement.
2. Stage of Subtotal traction detachment with macular involvement
E. Stage of total retinal detachment
CHARTNG OF LE8ON8 N RETNOPATHY OF PREMATURTY
Charting of retinopathy of prematurity differs from usual retinal charting where macula
is taken as centre of the chart. While charting R.P.P.M. optic disc in taken as center and two
concentric circles are drawn dividing the retinal chart in to three zones
A. The first zone has diameter twice the distance between the fovea and the disc.
B. The second circle extends from centre of disc to nasal ora serrate. The area between
first and second circle is second zone.
C. Remaining part which is widest on temporal side and almost obliterated on nasal
side is called zone three. Lesions extending in zone one are most sever while those
localised in zone three are mildest.
Clinically on the basis of indirect ophthalmoscopy findings retinopathy of prematurity
has been divided in to five stages
25,26
. They are :
Stage 1. Visible line of demarcation, it consists of a clear cut line of demarcation be-
tween the avascular and vascular retina. The line is pale in colour, not raised, is as flat as rest
of the retina.
Stage 2. In this stage the line of demarcation is elevated over the retina and is said to
have height and width. It is more towards pink than white as compared to line of demarcation,
small tufts of vessels may be seen posterior to the ridge and these vessels do not form the fibro
vascular stage that is seen in the next stage.
Stage 3. There is a definite ridge of extra-retinal fibro vascular proliferation.
Stage 4. This is state of partial traction retinal detachment that has been divided in to
two substages i.e. stage 4 a and 4 b former consist of sub total tractional detachment without
involving the macula while in the later the macula is involved.
Stage 5. This is a stage of total retinal detachment.
Plus disease retinopathy of prematurity plus disease denotes progressive vascular com-
promise. Presence of plus disease is an ominous sign. It is called anterior plus when there is
neovascularisation of iris and difficulty in dilation of pupil. The posterior plus represents retinal
venous dilation and arterial tortuasity in the posterior pole.
Thresh hold disease
This stage is defined as presence of at least five contiguous or eight cumulative clock
hours (30o) sector of stage 3 in zone I or II in presence of plus disease. Importance of this stage
lies in the fact that 50% of children in this group are predicted to go blind if not treated.
Prethresh hold disease this comprises of
1. Any stage of disease in zone I
2. Stage 2 disease in zone II with plus disease
3. Extra fibro vascular proliferation zone III.
RETINOBLASTOMA 487
The disease is called Rush disease when this occurs in an infant weighing less than
1000 gms who develop the disease earlier and faster between 3 weeks and total retinal detach-
ment is almost certain.
Management of retinopathy of prematurity
24,25,27
The best method of treatment is prevention of the condition by monitoring oxygen. How-
ever retrolental fibroplasia has been observed even in infants who have either been on oxygen
for very short period or within recommended period and concentration. Besides gestation pe-
riod birth weight and oxygen administered there are other factors that may be influencing
development of retrolental fibroplasia they are - Vitamin E deficiency, phototoxicity, blood
pH, CO
2
tension, maternal diabetes and prostaglandin level.
Predisposed premature infants must be examined :
1. First between four to six weeks post delivery by indirect binocular ophthalmoscope
under complete but safe mydriasis.
2. Then at interval of ten days till there is complete vascularisation of retina all round
3. Then every fortnightly and
4. Every month.
Treatment of retrolental fibro plasia is controversial because
1. Spontaneous regression is seen in 80 to 90% of cases in acute stage.
2. All modalities of treatment have poor visual outcome.
3. There is no known clinical parameter that can evaluate efficiency of any or combina-
tions of treatments.
4. Over all prognosis is poor, children with best treatment also have subnormal vision.
Most Commonly used therapeutic and surgical methods are
1. Cryo therapy,
2. Laser photocoagulation delivered by indirect ophthalmoscope either diode or argon
lasers are used. Anterior lesions of zone III have best prognosis while those in zone I have
worst prognosis. Stage four and five are treated by vitreo retinal surgery.
Complications of retinopathy of prematurity consist of High myopia, retinal pigmen-
tation, lattice degeneration, dragging of retina, retinal hole formation,
rhegmatogenous retinal detachment, secondary glaucoma and squint.
PER88TENT HYPERPLA8TC PRMARY VTREOU8
28,29,30
This is a congenital anomaly of primary vitreous and hyaloid artery. It is due to fail-
ure of regression of primary vitreous. This is non hereditary generally uniocular. These
eyes are microphthalmic the child is full term with normal expected birth weight, there
is no history of administration of hyperbaric oxygen in first week of life. There are two types
of this disorder
A. A more common anterior and
B. Rarer form of posterior type
Anterior type
The eye is microphthalmic the anterior chamber is shallow, undeveloped angle, iris has
large visible blood vessels. Initially the lens is clear with visible retrolental white mass. The
ciliary processes are pulled towards the mass and are elongated. The retrolental mass may
contract and produce a rent in the posterior capsule leading to cataract formation that may
cause swelling of the lens, pushing the iris lens diaphragm forward, narrowing the already
faulty angle. The cortical matter may absorb leaving a membranous cataract. The retrolental
mass may cause traction detachment.
Posterior type
This is less common than former. Generally cornea is smaller than normal, anterior
chamber is either normal or deep, lens is clear and normal in size, there may be coloboma of
lens or subluxation of lens. Commonest finding is in the posterior part of the globe re-
sulting in a white opaque membrane or retinal fold extending from optic disc to periphery of
the retina, it may extend in the retrolental space resulting in to a white reflex. Posterior
variety may be bilateral.
Milder form of this anomaly is Bergmeister papillae and Mitten drofs dot. Some
times remnants may be seen in the mid vitreous at the level of Cloquets canal.
Management. There is no known preventive method or medical treatment. Surgical
intervention in the form of lensectomy and vitrectomy if done early may have good vision . As
the condition is unilateral, amblyopia is common and must be treated vigorously.
COAT8 D8EA8E
31,32,33,34
This is a non hereditary non inflammatory disorder of retinal vascularture. The exact
pathogenesis of the condition is not known, the most probable cause is congenital malforma-
tion of retinal vessels i.e. telengiectasis that is associated with subretial exudation and non
rehegmatiogenous retinal detachment. Condition is invariably seen in male children in first
decade and in ninety percent cases it is unilateral. In bilateral cases the changes are
minimal. In rare instances the condition is detected in infancy. The lesion starts in the retinal
periphery as dilated vessels with capillary drop out, rarely there is vascular sheathing. Exu-
dation is an important feature, the exudation is mostly seen in the posterior pole, sub
retinal fluid contains cholesterol crystals but no calcium. The natural history of the
condition is progression toward formation of white reflex in pupillary area behind the lens.
The cause of white reflex in pupillary area is to exudation and retinal detachment. There may
be secondary and complicated cataract. Neovascularisation of iris and angle is com-
mon feature in poorly managed cases that lead to neovascular glaucoma which may termi-
nate in enucleation. Many blind eyes with glaucoma in Coats disease have been removed
with mistaken diagnosis of retinoblasoma. The eyes may go into phithisis. In rare instances
milder forms may regress spontaneously. There should be no difficulty in diagnosis of a case of
Coats disease if media is clear. How ever in advanced cases ultra sonography and CT are two
most helpful investigation. CT is single most valuable test as it delineates intra ocular mor-
phology.
RETINOBLASTOMA 489
Differential diagnosis of coats disease consists of retinoblastoma, angiomatosis reti-
nae (generally seen in third and four the decade) familial exudative vitreo retinopathy,
retinopathy of prematurity and endopahthalmitis.
Management of Coats disease consists of early detection exclusion of other causes of
white reflex in pupillary area confirmation of diagnosis. Best results are seen if the treatment
is initiated before exudation starts. There is no medical treatment. Two modes of treatment
that are followed are
1. Cryotherapy and
2. Laser phopcoagulation.
It takes eight to ten months for exudate to disappear after laser photocoagulation. Re-
currence due to formation of new telengectasis is know to occur in poorly managed cases or
years after initial successful treatment.
LARVAL GRANULOMA OF EYE - TOXOCARA88
35,36,37
(See page 264)
This chronic systemic disorder is caused by parasite Toxocara canis a roundworm of
dogs. There is doubt if toxocariasis can be caused by toxocara catis the round worm of cats.
The child gets infected by the ova of the parasite that is passed in the faeces of the infected
dog. The children who develop toxocariasis have history of pica. These children come in close
contact with puppies that are infected at an early age, consuming food accidentally infected by
ova. The commonest age for a child to get infected is Under two years. By two years the child
gets symptoms of systemic involvement. The systemic involvement comprises of low grade
fever, hepatosplenomegaly, pneumonitis convulsion and cutaneous erythema. The systemic
involvement is known as Viscereal larval migrans which does not cause ocular involve-
ment. Ocular involvement without intestinal involvement is most improbable. Visceral larval
migrans causes leucocytosis, and eosinophilia Elisa test is positive in titre of 1:8. Positive
Elisa helps to differential ocular toxocaria from retinalblastoma.
Ocular toxoariasis In contrast to age of systemic involvement which is two year, aver-
age age to develop ocular toxocariasis is 7.5 years. It is generally unilateral. There are three
known forms of ocular toxocariasis. They are
A. Chronic endophthalmitis.
B. Posterior pole involvement
C. Peripheral granuloma.
Chronic endophthalmitis is most common and devastating. It is seen between 2 to 9
years of age. The ocular involvement is typically a combination of chronic uveitis with vitrities
resulting in to loss of vision, leucokoria and strabismus. A combination uvea and vitrities leads
to posterior cyclitic membrane, retinal detachment and complication cataract. Visual progno-
sis is very poor. These eyes are generally enucleated as they become painful blind eyes. The
lingering fear of reinoblasoma is perhaps the most important motive to remove such eyes.
Histopathology may reveal dead larva.
Posterior pole involvement This occurs in slightly older, children i.e. between 6 to
14 years and is unilateral leading to loss of vision. The granulomn is situated between the
macula and the disc or may be seen over the macula. It is a raised yellowish mass wider than
optic nerve head. The mass may be surrounded by retinal edema, hard exudates or stress
lines, retinal vessels may dip in the mass.
Peripheral granuloma : This is least vision threatening seen in children as well as
adult upto third and fourth decade is again unilateral produces least inflammatory signs.
Generally vision is unaffected however it may produce cystoid macular edema or retinal
detachment. The lesion is a hemi spherical mass anterior to equator with vitreous band.
Management
The management comprises of prevention of infection, treatment of systemic involvement
and treatment of ocular involvement.
Prevention
All domestic dogs and cat should be dewormed from time to time. Domesticated puppies
should be toilet trained. This reduces chances of contamination of food. Pica in the child should
be managed in consultation with pediatrician. Deworming of children has doubtful role.
Treatment of visceral larval migrans is systemic administration of anti helmenthic
drugs like thiabendazole, albendazol, Dead worm may cause systemic allergic reaction.
Treatment of ocular involvement:
There is no medical treatment that can treat larval granuloma. Curiously by the
time ocular involvement takes place systemic signs and symptoms subside with normal WBC
count, however Elisa remains positive. Local treatment comprises management of uvetitis
with cycloplegic and local steroid mostly peri-ocular depot injection.
Surgical treatment Parsplana vitrectomy removes larval antigen cuts posterior cyclitic
membrane and releases traction vitreo retinal bands that may cause traction or rhegmatogenous
retinal detachment. Retinal detachment in treated by sclera buckling. Over all visual re-
sults are poor.
Features
P.H.P.V. R.O.P.M.
Retinoblastoma
Coats Disease
Age of Presenta-
tion
Bilateral Unilat-
eral-18 Months
12 At Birth
10 Weeks post Na- tal
First decade may
be seen in infancy
Sex
Equal in both Equal in both Equal in both 90% males Laterality
Unilateral 70% Bilateral 30% Unilateral Bilateral Unilateral
Size of Globe Initial Normal
Later buphthalmic
Microphthalmn Normal Normal
Ciliary Process Not Visible Visible Not visible Not Visible
Birth Weight Normal Normal Low less than
1500gm
Normal
RETINOBLASTOMA 491
Prematurity No No Premature No
Effect of Oxygen
administration
Not Significant Not Significant Significant Not Significant
Heredity May be Significant be Not Significant Not significant Not Significant
Development of
Retrolentalmass
1012 Months At birth 2-3 Months Late
X-ray Intra Ocular
Classification
Not Significant,
Non Specific
Not significant,
Non Specific
Not Significant
U.S.G Tumour arising
from retina that is
acoustically solid,
high internal re-
flection, may show
calcification
May show mass in
vitreous
Mass in anterior
viterous
Exudative retinal
detachment
CT Tumour appears as
solid mass with in
globe, areas of cal-
cification that has
density of bone
No significance No significance Most important
investigation in
opaque media it
delineates intra
ocular morphol-
ogy
NTRA OCULAR GROWTH OTHER THAN RETNOBLA8TOMA THAT
CAU8E WHTE REFLEX N PUPLLARY AREA
They can be
A. Malignant
1. Meduloepithelioma
2. Leukaemia
3. Glioneuroma
B. Benign (Hamartomas)
1. Astrocytoma
2. Haemangiomas
(a) Capillary hemangioma
(b) Cavernous hemangioma
C. Phacomas
A. Malignant
1. Meduloepithilioma See page 284.
2. Leukemia
40
Ocular signs are never presenting features of leukaemia. Intraocular involvement is
late. It is generally seen as recurrence after initial remission and always have poor prog-
nosis. All the intra ocular structures that have blood vessels in them i.e. uvea, retina and
optic disc may be involved. The vitreous may get seeded by leukaemia infiltration. A
pseudohypopyon may also develop. Infiltration of optic nerve is always associated with vi-
sion loss which is not recovered following apparent cure by radiation or chemotherapy. Infil-
tration of iris and ciliary body may be mistaken as anterior uveitis specially with
pseudohypopyon. There may be localised or diffuse infiltration of choroid. As most of the chil-
dren with leukaemia are immune compromised either due to malignancy or chemophathapy
or both it is difficult to state if the uveal lesion is due to malignant infiltration or due to
opportunistic organism. The controversy can be settled by fine needle biopsy of the lesion.
The fundus lesions of the retina may be due to
1. Direct infiltration of retina or optic nerve or
2. Due to associated anaemia and thrombo cytopenia.
The hemato logical retinal lesions are superficial linear or flame shaped haemorrhages.
Superficial haemorrhage with white centres are due to anaemia and thrombo cytopenia.
Other lesions may be non rhegmatogenous retinal detachment, retinal pigment
epithelial detachment.
Management. Treatment is essentially directed to systemic involvement, local external
beam radiation to eye results in regression but improvement of vision is not always predictable.
3. Glioneuroma. This is very rare tumour, arises from anterior lip of the optic cup,
unilateral involves anterior sigment, produces white reflex but does not involve posterior
sigments.
B. Benign (Hamartomas)
1. Retinal astrocytoma
41,42,43
. Retinal astrocytoma is a rare hamartoma of the retina
seen in children they are non invasive and non malignant, generally stationary they
may be solitary or multicentric. Unilateral lesions are frequent. Commonest lesion is a white
superficial, raised lesion little larger than disc. On fluroscein angiography it has slow fill-
ing, late staining. It does not require any treatment. The condition is most commonly seen is
tuberous sclerosis that has multiple systemic involvements.
2. Retinal haemangiomass
41,42,43,44
(a) Retinal capillary haemangioma
These rare vascular tumours that share autosomal dominant trait with retinoblastoma.
They are tumours of aduldt hood may become apparent in childhood. They also arise from
sensory retina and are multicentric may be bilateral. The similarity with retinoblastoma ends
here. The difference are: their colour, afferent and efferent vessels, sub retinal and inter
retinal exudale. They never metastasise.
These growths are haemartomas that may be seen on the retina as well as optic nerve
head. They may be confined to eye only or may have systemic involvement in twenty five
percent cases and is known as vonHippels disease In the intra ocular growth may be uniocular
RETINOBLASTOMA 493
or bilateral may be single growth or there may be more than one growth of different size and
duration multiple growths have poorer visual out come.
The single haemangioma of the retina is discovered on routine retinoscopy and fundus
examination before retinal detachment sets in. The detached retina may produce a white re-
flex in pupillary area and draw attention.
The characteristics of retinal capillary haemangioma are : Spherical , raised, bright
red growth with afferent vesseles and efferent vessles. The growth generally are one to
two disc diameter in size. Both the feeding and draining vessles are dilated and tortous. Larger
growths have larger vessels.
There may be inter or sub retinal exudates with macular star formation. These exu-
dates may give a pale reflex during retinoscopy. The exudate cause retinal detachment that
may be partial or total. Retinal detachment results in white reflex in pupillary area. Second-
ary glaucoma and iris neovascularisation are some of the complications.
Diagnosis is confirmed by : Indirect ophthalmoscopy, Bscan, CT. MRI and fundus
fluorescing angio graphy.
Fundus fluorescing angio graphy is most important diagnostic procedure in absence of
total detachment. Angiography shows rapid filling of the entire vessels and the tumour, hyper
fluorescence of the growth, rapid filling of the draining channels. If is leak from the tumour it
may colour the vitreous.
Management small peripheral tumours need not be treated but observed periodically
for enlargement in size, development of internal and subretinal exudates, retinal detachment,
if any of above manifest, the lesion requires treatment. The best mode of treatment is by photo
coagulation, peripheral lesion many be treated by cryotherapy. Diathermy has been given up
in favour of former two.
There is difference of opinion if
(i) the growth along with its feeding vessels
45
should be treated
(ii) only the vessels be obliterated
43
and hope that the growth will shrink
(iii) the tumour only should be treated
42
.
The best results are claimed to be by Argon laser with large spot size, longer dura-
tion and low intensity. The tumour should not be treated in one session. It should be treated
by several sittings spread over months. The retina around the dilated vessels and angioma
may require scattered photo coagulation to prevent exudation.
(b) Cavernous hemangioma of the retina
41, 44
This is a rare congenital malformation of retinal vasculature , this is a hamartoma, non
progressive, non malignant growth may have positive family history generally uniocular and
single. Situated behind the equator either in the retina or on the optic nerve head. It is slowly
progressive generally symptom less, diagnosed on routine fundus examination in late teens or
early third decade.
The retinal growths are inter retinal in nature with slight elevation of the ritual surface.
The single growth is in fact a cluster of many smaller thin walled sacular micro aneurysm. The
over all size of the growth varies between 2-4 mm in diameter there may be few micro aneurismal
dilatation in the surrounding area. The surface of the retina invariably shows moderate glial
proliferation over the growth that may be confused with superficial exudate. There is no arterial
dilatation in contrast to capillary haemangioma.
Fluroscein angiograpthy is diagnostic, there is slow filling of the lesion, each saccule
accumulates fluorescing individually and remains fluorescent for long time.
As the lesion is non progressive and does not have symptoms it does not require any
treatment.
REFERENCE8
1. Abramson D.H. ; Retinoblastoma - Diagnosis and management, Cancer, 32-130-140, 1982.
2. Biswas J. ; Intra ocular tumours, In Modern Ophthalmology, 1st Edition, pp. 650
669, Edited by Dutta L.C., Jay Pee brothers, new Delhi, 1994.
3. Boger W.P. Peterson.R.A. ; Tumours of childhood in Manual of ocular diagnosis and
therapy. Fifth edition edited by Deborah Pavan Langston p- 318-320, Lippin cott William
and Wilkins 2002.
4. Augsburger J.J. Yanoft. M. ; Retinoblastoma in Text book of ophthalmology Vol-
318. pp. 18.118.14, Edited by Podos S.M. and Yanoff.M. Mosby London 1991 .
5. Muhprea A.L., Christensen L.E. ; Retinoblastoma in Current ocular therapy Edition 5,
p-262-263, Edited by Fraunfelder F T and Roy F H. W B saunders company Philadel-
phia 2000.
6. Kanski J.J. ; Tumours of uvea and retina in Clinical ophthalmology, Second Edition
pp. 401407 , Butter worth London 1989.
7. Puklin J.E. Apple D.J. ; Retinoblastoma and leucokoria in Principles and practice of
ophthalmology Vol. 2 First Indian edition pp. 11361182, Edited by Peyman G.A.
Sander D.R. and Goldberg M.F. Jay Pee Brothers New Delhi 1987.
8. Asdourin G.K. ; Retinoblastoma in Manual of Clinical Problem in Ophthalmology
first edition p-125-128 Edited by Gittinger J.W. and Asdouria G.K. Little Brown Boston
1998.
9. Shields C.L. ; Retinoblastoma in Ophthalmology Secrets First Indian Edition pp.
322326, Edited by Vander J.F. and Gault J.A. Jay Pee brothers, New Delhi 1998.
10. Ellsworth R.M. ; The practical management of retinoblastoma, Trans Am. Oph So 67:
462-534 1969.
11. DePotter P. ; Orbital imaging in Ophthalmology secrets first Indian edition pp. 231
233, Edited by Vander J.F. and Gault J.A. Jay Pee brothers, New Delhi 1998.
12. Shields J.A. ; Augs burger J.J.,Current approach to the diagnosis and management of
retinoblastoma surv. ophth. 25: 347-372 1981.
13. Augstburger J.J., Yanoft. M. ; Benign tumours in the differential diagnosis of
retinoblastoma in Text book of ophthalmology vol. 18. pp. 19.119.11, Edited by
Podos SM. and Yanoff M. Mosby, London 1991.
RETINOBLASTOMA 495
14. Augosburger J.J., Yanoft. M. ; Malignant tumours in the differential diagnosis of
retinoblastoma in Text book of ophthalmology vol.18 pp. 20.120.5 edited by Podos
SM and Yanoft M mosby, London.
15. Ratnakar K.S. ; Retinoblastoma in Pathology of the eye and orbit first edition pp-
224-230 Jay Pee brothers New Delhi 1997.
16. Biswas J. and Dutta Nitin ; Retinoblastoma in Modern Ophthalmology Vol. 2 Second
Edition, pp-750, Edited by Dutta L.C. Jay Pee Brother , New Delhi- 2000.
17. Ellsworth R.M. and Notic C.M. ; Retinoblastoma in Current ocular therapy , Edition
4, Edited by Fraunfelder F.T. Roy H.F. pp. 355357, W B Saunders Co Philadelphia 1995.
18. Desai V.N. Duker J.S. ; Masquerade Syndromes in Ophthalmology Secrets Edited by
Vander J.F. and Gault J.A. First Indian Edition pp. 263266 Jay Pee brothers , New
Delhi 1998.
19. Resse A.B., Ellsworth. R.M. ; The evaluation and current concept of retinoblastoma
therapy. Tran, Am Acd Oph Oto 67: 164-172 1963.
20. Campbell J.R., Sobin L, Zimmerman L.E. ; The T.N.M. Classification of malignant tu-
mours of the eye and ocular adnexa, Am Jr. Oph. 100:83-84 1985.
21. Donahue S.P. ; Retinopathy of prematurity, Editorial B.J.O. 86 pp. 1071 2002
22. McNamara J.A. Tasman W. ; Retinopathy of prematurity Oph Cli Nor Am, 3: 413-
4271990.
23. G.E. Quinn ; What do you do about ROP screening in big babies B.J.O. 86: 1072-1073
2002.
24. McNamara J.A. ; Retinopathy of prematurity in ophthalmology secrets edited by
Vander J.F Gault J.A. pp. 294299, Jay Pee Brothers , New Delhi 1998.
25. Natrajan S. and Dutta B. ; Retinopathy of prematurity in Modern ophthalmology ,
Vol. 2, second edition, edited by Dutta L.C. pp. 641649, Jay Pee brothers, New Delhi
2002.
26. Committee for the classification of retinopathy of prematurity, An international classi-
fication of retinopathy of prematurity, Arch Oph 105: 906 1984
27. Perterson R.A. and Boger III W P ; Retinopathy of prematurity in Manual of ocular
diagnosis and therapy, 5th Edition pp. 32324, Edited Deborah Pavan Langston,
Lippincot Williams and Wilkins Philadelphia 2002.
28. Antoszyk, J.K. Antoszy K. A.A. ; Persistent Primary hyperplastic viterous in Current
ocular therapy, Edition 5, edited by Fraunfelder F.T. and Roy F.H pp. 642644, W.B.
Saunders company Philadelphia 2000.
29. Federman J.L., Shields J.A., Altman B, Koller H. ; The surgical and non surgical man-
agement of persistent hyperplastic primary vitreous ophthalmology 89 : 20-24 1982.
30. Augsburger J.A. and Myron Yanoff ; Retinoblastoma in Test book of Ophthalmology
Vol.18 pp. 18.118.14, Edited bt Podos S.M. and Yanoft M. Mosby London 1991.
31. Shield A.J. Stephens R.E., Sarin L.K. ; The differential diagnosis of retinoblastoma in
Paediatric Ophthalmology vol. 2, Second Edition edited by Harley R.D. pp. 1144
1157, W B Saunders Comp Philadelphia 1983
32. Wu Lihtech and Murphy R.P. ; Coats disease in Current ocular Therapy edition 5,
Edited by Fraun Felder F T and Roy F H. pp. 607608, W B Saunders Company Phila-
delphia 2000
33. Tasman W. ; Coats Disease in Current ocular therapy edition 4 , Edited by Fraunfelder
F T and Roy F H. pp. 777778, W B Saunder Company Philadelphia 1995
34. Ridley M Shields J A Brown J.C. ; Coats Disease evaluation of management Ophthal-
mology 89: 1381-1384 1982.
35. Shileds J.A. Stephens R.F, Sarin L.K. ; Differential diagnosis of retinoblastoma in
Paediatric ophthalmology Vol. 2, Second Edition edited by Harley R.D. pp. 11451147,
W B Saunders Company Philadelphia 1983.
36. Greer C. H. ; Ocular Infestation by toxocara in Ocular pathology first edition pp. 54
55, Black Well Scientific Publication Oxford 1963
37. Kanski J.J. ; Uveitis : In Clinical ophthalmology, Second Edition 157-159, Butter worth,
London 1989.
38. Gillespie , S H. ; Ocular toxoariasis in Current Ocular Therapy, Edition 5 pp. 9798,
Edited by Fraunfelder F H and Roy F H, W B Saunders Company Philadelphia 2000.
39. Gillespic S H Dinning W J Voller ; A Crow crowft N. S.,The spectrum of ocular toxocariasis
Eye 7 : 415-418 1993.
40. Augsberger J. J and Yanoft M. ; Malignant Tumours in the differential diagnosis of
retinoblastoma in Text book of ophthalmology, Edited by Podo S.M. and Yanoft M.
pp. 20.220.5, Vol. 18. Mosby London 1991.
41. Augsberger J J and Yanoff M. ; Benign tumours in the differential diagnosis of
retinoblastoma in Text book of ophthalmology edited by Podos S. M. and Yanoff M
pp. 19.119.11, Vol. 18. Mosby London 1991.
42. Kanski J J. ; Tumours of uvea and retina in Clinical ophthalmology , Second Edition
pp. 405409, Buffer worth, London 1989
43. Shields J.A. Stephens R F and Sarin L. K. ; The differential diagnosis of retinoblastoma
in Paediatric Ophthalmology, Vol. 2 Second Edition, Edited by Harley R.D pp. 1144
1157, W B Saunders comp Philadelphia 1983.
497
CHAPTER 13
Di sor der s of Pupi l , Ac c ommodat i on and
Conver genc e i n Chi l dr en
GENERAL CONSI DERATI ON
The choroid and ciliary body are placed parallel to the sclera. The iris is placed at right
angles to the sclera at the level of scleral spur like a curtain that divides the aqueous chamber
into two unequal parts i.e. a large anterior chamber and a smaller posterior chamber. The two
chambers communicate through a natural defect in the centre of the iris called the pupil.
The pupil though for all practical purposes is central, in fact is slightly decentered
medially and inferiorly with in a physiological limit. When the position of the pupil exceeds the
physiological limits, the condition is called corectopia
1
or ectopic pupil, which generally
occurs as congenital anomaly, trauma or post inflammatory status.
In rare instances of congenital anomaly there may be more than one pupil in the same
eye. This is known as polycoria.
1
A true polycoria should have its own constrictor and dilator
muscles independent of main pupil and should react independently. In contrast to this, pseudo
polycoria is a condition where there is a hole in the iris without separate constrictor or dilator
muscle and the hole does not react to light, or near reflex. Common causes are congenital
anomaly of iris and anterior chamber, trauma, accidental or surgical or post
inflammatory status.
The function of the pupil is mostly optical. It regulates the light reaching the retina.
The pupil constricts in presence of bright light and dilates in darkness. The other function is to
cut the spherical aberration and chromatic aberration and mildly increases the depth
of focus. Depth of focus is inversely proportionate to diameter of pupil. Larger the pupil lesser
is the depth of focus. Besides optical purpose, the pupil regulates passage of aqueous from
posterior chamber to anterior chamber. A very small pupil may cause pupillary block resulting
into rise of intraocular tension. If the pupil is bound down to the structures behind it, as
happens in seclusio pupillae, the iris may bulge forward producing iris bombe.
The size of pupil varies from person to person. Generally in an adult it is about 2.5 mm
in diameter. It is small in extremes of ages i.e. pupil of a new born is smaller than an adult.
The pupil in myopia is larger and smaller in hypermetropia in comparison to emmetropia.
The most commonly given reason is that a pupil constricts when the eye accommodates, a
myopic eye requires very little accommodation. In contrast to this, the hypermetropic eye
requires more accommodation hence the pupil is smaller in hypermetropia and larger in myopia.
The size of pupil is equal and shape symmetrical in two eyes in 80% of persons. Only in
remaining, the size of the pupil differs in two eyes. This is called anisocoria. Anisocoria is
said to be simple or essential when the size of any of the pupil does not change due to change
in illumination. If the size of the pupil changes with illumination it means a pathological
phenomenon. In such cases it must be decided, which pupil, the larger or the smaller is patho-
logical.
2
For example, in Horners syndrome the degree of anisocoria is most marked in dim
illumination.
4
However in case of unilateral visual defect size of the pupil does not change
irrespective of opacity in media, or pathology of retina or optic nerve.
The size of the pupil depends on a delicate balance between the potency of constrictor
and dilator muscles. Constrictor muscle has an enhanced tone than dilator more so in childhood
and irritation of parasympathetic as seen in iridocyclitis. The pupil of a child is more resistant
to mydriatic than that of an adult. A light coloured eye has a larger pupil that dilated better
than a dark coloured iris.
The neural control of constrictor pupillae
The constrictor of the pupil is called the sphincter pupillae.
5
It is ectodermal in origin
and is placed in a circular fashion round the pupil. It is supplied by third cranial nerve via
its parasympathetic system. The fibres start in the Edinger-Westphal nucleus and pass in
the third nerve up to orbit where the third nerve divides into two branches. The lower branch
supplies the inferior oblique. After leaving the inferior oblique, the nerve enters the ciliary
ganglion as its motor root. From the ciliary ganglion start the post ganglionic fibres that pass
along the short ciliary nerve and enter the sclera around the optic nerve.
Once the post ganglionic fibres enter the sclera, they pass in the supra choroidal
space and terminate in the iris and ciliary body. Ninety percent of the fibres go to the ciliary
body and only three percent to the iris, remaining supply the blood vessels.
6
The neuro trans-
mitters released at neuro muscular junction is acetylcholine.
Neural control of dilator pupillae
The dilator pupillae is also ectodermal in origin, it is arranged in radial fashion away
from the pupillary margin. In comparison to constrictor it is a poorer muscle. The dilator
muscle is differentiated later than sphincter and continues to develop even after birth. This
may be the cause of smaller pupil in neonates. The dilator pupillae is supplied by cervical
sympathetic. The post ganglion fibres from superior cervical ganglion accompany the
internal carotid artery and then pass along the first division of the trigeminal nerve and
emerge from the cavernous sinus with nasociliary nerve and long ciliary nerve to the
iris. The neuro transmitter released at neuro muscular junction is nor epinephrine.
The pupil is kinetic indicator of functional state of iris, optic nerve, third cranial
nerve and ocular sympathetic system.
The size of the pupil keeps on fluctuating with change in intensity of illumination,
accommodation, convergence and integrity of parasympathetic and sympathetic path. Part of
the parasympathetic path is common with visual path.
DISORDERS OF PUPIL, ACCOMMODATION AND CONVERGENCE IN CHILDREN 499
The two neural paths involved in pupillary reaction are
1. Light reflex
2. Near reflex
(a) Accommodation
(b) Convergence
(c) Miosis
The pupillary light reflex is a four-neurone arc with an afferent and an efferent
component. The former spreads from retina to Edinger Weshphal nucleus and latter from
Edinger Westphal nucleus to the iris.
The first neuron extends from rods and cones to pretectal nucleus in the mid brain.
The fibres from the temporal half go straight into the optic tract without decussation. The
fibres from nasal retina decussate at the chiasma in the same manner as visual pathway. The
crossed and uncrossed fibres travel in the optic tract up to its posterior third where they leave
the visual path and turn to pretectal nucleus.
The second neuron connects pretectal nucleus on each side to the Edinger Westphal
nuclei. The neurones that join the pretectal nucleus to the Edinger Westphal nucleus are
called inter nuncial neurons. A decussation take place in the mid brain before entering the
Edinger Westphal nucleus. This decussation explains consensual light reflex in the other eye
when light is shown in one eye. In normal eye, the consensual light reflex is equal and
symmetrical to direct reaction.
The third neuron spreads from Edinger Westphal nucleus to ciliary ganglion.
The para sympathetic fibres change their relative position in third nerve during its course.
Between the, mid brain and the cavernous sinus, the pupillo motor fibres are superficial in
the nerve and are liable for a compressive lesion. Fortunately these lesion are less common in
children. In the cavernous sinus and beyond, the pupillo motor fibres become central hence are
not affected by mass lesion even when the third nerve is paralysed. In the orbit, the pupillo-
motor fibres reach the ciliary ganglion via nerve to inferior oblique.
The fourth neuron leaves the ciliary ganglion and accompanies the short ciliary nerve
to reach the sphincter pupillae.
The whole of the pupillary light reflex is subcortical.
NEAR REFLEX
This is not a true reflex. It is better called near synkinesis of accommodation,
convergence and miosis.
7
In contrast to pupillary light reflex where Edinger Westphal nucleus acts as nucleus
for pupillary light reflex, there is no definite nucleus for near reflex. These probably are
supra nuclear areas one each in frontal and occipital lobe that mediate the reflex. The various
components of the synkinesis can be modified either by optical device i.e. lens/prism or
therapeutically. The accommodation can be relaxed by plus lenses while it can be enhanced
by minus lenses. The convergence can be neutralised by base out prism and enhanced by
base in prism. The miosis is overcome by mydriatic. Cycloplegic will abolish both
accommodation and miosis in normal eye. The reflex can be initiated either by accommodation
or convergence. Vision is not needed for near reflex. It can be elicited in blind eyes if the
patient is made aware of a near object like his own index finger and asked to look at it when
kept near the eye. There is no condition where light reflex is present but near reflex is
absent.
2,4,7
The near reflex path is also parasympathetic in nature. The near reflex starts in
visual cells of retina. It follows the same path as light reflex up to the posterior third of the
optic tract where the pupillary light reflex fibres leave the tract to join the pretectal nucleus.
The near reflex fibres continue with visual fibres along the optic radiation to the occipital
cortex area
17,18
. Here the visual fibres terminate. The near reflex fibres continue to the frontal
occulomotor centre where they become part of a mass reflex that is mediated through
cortico-tectal tract to the occulomotor nuclei.
The efferent path starts from the Edinger Westphal nucleus and follows the third
nerve like pupillary light reflex.
Convergence reflex can not be separated from other two synkinesises i.e. accommodation
and miosis. Convergence enhances both accommodation and miosis. Miosis due to bright light
does not influence accommodation or convergence. However accommodation strengthens
convergence and increases miosis.
The reflex arc for convergence starts from medial rectus and follows the third nerve
up to third nerve nucleus complex. The Perlias nucleus is supposed to control the conver-
gence. The efferent path extends from the Perlias nucleus to the ciliary ganglion and its post
ganglionic fibres.
Other pupillary reflexes
The other pupillary reflexes are not associated with either light or near reflex.
They are
Orbicularis reflex
Vestibular and cochlear reflex
Trigeminal reflex
Vagotonic reflex
Psychosensory reflex.
The orbicularis reflex is constriction of pupil on the side of forceful contraction of
orbicularis against resistance. It is present in about 80% of persons. The exact path of the
mechanism is not known.
The vestibular and cochlear reflex have little value in ophthalmic evaluation. They
represent caloric, acoustic and rotational sensation from vestibular and cochlear area in the
mid brain.
The trigeminal reflex - On touching the cornea, there is momentary mydriasis followed
by miosis. The exact path of the reflex is not known. It is presumed that the afferent trigeminal
fibres also stimulate the third nerve via mid brain.
The vagotonic reflex consists of mild dilatation of pupil on inspiration and slight
constriction on expiration.
The psychosomatic reflexThis represents an overall sympathetic over action. Hence
the pupil are dilated in surprise fear, anxiety and hysteria.
Effect of drugs on pupil and accommodation
5,8,10
The drugs that affect the pupil and accommodation can be
1. Peripheral (ocular) action
2. Centrally acting.
The drugs acting on the intraocular muscles act via autonomic nervous system. They
are -
1. Cholinergic A. Agonist Miotic and cyclotonic
B. Antagonist Mydriatic, cycloplegic
2. Adrenergic A. Agonist Mydriatic
B. Antagonist Miotic
The cholinergic agonists are commonly known as parasympathomimetic drugs, while
the cholinergic antagonists are commonly known as para-sympatholytic drugs.
The parasympathomimetic drugsMiotics can be
1. Direct stimulators of cholenergic receptorsPilocarpine, methacholine.
2. Drugs causing release of acetylcholine at nerve endingCarbachol.
3. Drugs conserving acetylcholine, Anticholine esterase drugs.
(I) Short actingPhysostigmine, neostigmine, pyridostigmine, edrophonium (Tensilon)
(II) Long actingDFP Echothiophate, demecarium.
All the above drugs are miotics and cyclotonics. Some of them are miotics on local
instillation i.e. pilocarpine, methacholine, carbachol, but may have systemic side effect.
Indirectly acting drug i.e. physostigmine, DFP, echothiophate and demecarium are also locally
acting drugs which are no more in use.
Edrophonium (Tensilon) has no therapeutic use, it is used to diagnose myasthenia gravis.
Pyridostigmine and neostigmine are used to treat myasthenia gravis.
Other two miotic drugs used in diagnosis of pupillary abnormalities are - Pilocarpine
0.125% and methacholine 2.5%. They do not constrict normal pupil in such low strength but
constrict pupil in Adies pupil.
Other miotics
These are
1. Locally acting sympatholytic drugs
They are
A. Those that block alpha repectors i.e. thymoxamine, phentolamine and priscoline.
(These drugs are rarely used)
B. Those that block sympathetic neuron Guanethidine (These also have very limited use
in adults only).
2. Directly acting on iris musculature
These are prostaglandines and histamine. They do not have any therapeutic use.
3. Centrally acting
These are central depressents that reduce supra nuclear inhibition on third nerve i.e.
morphine and barbiturates.
Mydriatics and Cycloplegics
Mydriatics are the drugs that dilate the pupil. They are not cycloplegics. They can be
locally acting or centrally acting.
Cycloplegics are the drugs that paralyse ciliary body and abolish accommodation. They
are mydriatics as well. They can be locally acting or centrally acting.
Mydriatics and cycloplegics can be put into two broad groups i.e.
1. Cholinergic antagonistsCycloplegic cum mydriatic.
2. AdrenergicAgonistMydriatic.
1. Cholinergic antagonistThese are also known as parasympatholytic drugs. They
are
A. Block cholinergic receptors (Atropine, home atropine, cyclopentolate, tropicamide,
scopolamine and eucatropine. The last two are no more used in clinical practice.
B. Drugs that block parasympathetic nerve ending i.e. Botulium toxin.
2. Adrenergic agonist - These are also known as sympathomimetic drugs. They are
(a) Directly stimulating alpha receptorsEpinephrine, phenyle-pherine, nor
adrenaline. The last has no therapeutic use.
(b) Drugs that release nor adrenaline from nerve ending - Ephidrine and hydroxyam-
phetamine. They do not have much therapeutic use. Hydroxyamphetamine (Pare-
drine 1%) is used to diagnose level of lesion in Horners syndrome.
Cocaine is a sympathomimetic drug that blocks the re uptake of nor adrenaline at
nerve ending is a good mydriatic and local anaesthetic, is no more in use. It has synergistic
action with parasympatholitic drugs. It is exclusively used to diagnose Horners syndrome.
Cocaine 4% will dilate normal pupil but not Horners pupil.
Abnormal pupillary reaction
11
Abnormality of pupillary reaction can be abnormality of light reflex or abnormality
of near reflex. Or there may be a dissociation of light near reflex.
The abnormalities can be in:-
Afferent path of third nerve
Efferent path of third nerve
Oculosympathetic chain
The anomalies are either neurological or pharmacological.
Afferent pupillary defect are -
1. Total afferent pupillary defect.
2. Relative afferent pupillary defect.
3. Wernickes hemianopic pupil.
4. Argyll Robertson pupil
The characteristics of afferent pupillary defects are
1. The lesion may be anywhere from retinal visual cells to the pretectal nucleus.
2. There is decreased amplitude of contraction to light stimulus.
3. The latency in reaction to light stimulus is increased.
4. The pupil dilates on prolonged exposure to light.
5. Both the direct and consensual reflexes are involved.
6. The afferent pupillary light reflex can be present in blind eye if the cause of blindness
is retro chiasmal.
While testing pupillary light reflex, it should be ensured that the accommoda-
tion is at rest. The room is dimly lit and the source of light is small, sharp and bright.
Total afferent pupillary defect (TAPD)
This is also known as amaurotic pupil.
11
The lesion causing the defect is unilateral
either as an extensive damage to the retina or complete loss of conduction in the optic nerve
anywhere from disc to the chiasma. There is no perception of light in the affected eye. Both
the pupil are of same size. In case of bilateral blindness due to pre geniculate lesion, the pupil
tend to be larger than normal. When light is thrown in the pupil of the blind eye neither pupil
react i.e. there is absent direct pupillary reaction in the affected eye and absent indirect reaction
on the contra lateral side. When normal eye is stimulated by light, both the pupil react. Any
amount of opacity in media is not capable of producing total afferent pupillary defect.
Relative afferent pupillary defect (RAPD)
This happens when the affected eye has some vision. The cause of which is located in
anterior visual path i.e. extensive retinal lesion or lesion in optic nerve. There is asymmet-
ric conduction defect. If both eyes have diminished vision, the eye with poorer vision develops
relative afferent defect. The difference between the conduction in two eyes can be estimated
with use of neutral density filter. The condition is also called Marcus Gunn pupil and is
demonstrated by swinging flash light test. Swinging flash light test consists of following
steps :
11,12
1. Vision in both eyes is noted with and without correction separately.
2. The room is semi-darkened.
3. The patient is asked to fix at a distant object.
4. Size of the pupil is noted in diffused light.
5. Direct and indirect light reflex are noted.
6. The eye withless vision, larger pupil and sluggish direct light reaction in the eye
effected.
7. Throw bright well-focussed light in one eye and note the reaction.
8. Quickly shift the light to the other eye and note the reaction.
9. Repeat above steps by moving the light from one eye to the other and note the reac-
tion.
Interpretation
1. In normal eye both the pupil constrict equally and symmetrically i.e. isocoria.
2. In presence of RAPD, the affected pupil shows reduced amplitude of miosis and en-
hanced dilatation.
The swinging flash light test is positive when only afferent pre-chiasmal conduction
defect is present and efferent arc is intact.
A modified and less sensitive test is
1. Occlude the normal eye for sometime, observe the affected eye. The pupil of
unexposed but affected eye will be larger than before the normal eye was occluded.
2. Occlude the affected eye in same fashion. The pupil of the non occluded normal eye
will be smaller.
The causes of RAPD are
1. Optic neuritis
2. Optic atrophy
3. Retro bulbar neuritis
4. Traumatic optic neuropathy
5. Advanced glaucomatous optic atrophy
6. Advanced and extensive retinal disease
Pupillary reaction in mid chiasmal lesion
The nasal fibres of retina decussate at the middle of the chiasma, the temporal fibres
pass without decussation. Hence in a mid chiasmal lesion, light directed from temporal field
produce less pupillary constriction than light directed from nasal field. The causes are pitui-
tary tumour, cranio pharyngioma, tubercular meningitis and trauma.
Pupillary reaction in anterior junctional lesion
Anterior chiasma syndrome is caused due to a lesion involving one optic nerve and
half of chiasma on the same side. The pupil when seen in diffuse light are slightly dilated and
large. The pupillary reaction is a combination of Marcus Gunn pupil in one eye and
chiasmatic hemianopia pupillary reaction in the other eye. Importance of this type of
pupillary reaction lies in the fact that it is seen in craniopharygioma and parasellar
extension of pituitary tumour which are common in children.
Pupillary reaction in lesion of optic tract
Lesions of anterior optic tract cause Wernikes hemianopic pupil. A lesion near the
lateral geniculate body does not produce any pupillary change because the pupillary fibres
leave the optic tract anterior to the junction of posterior 1/ 3 to anterior 2/ 3. Wernickes
hemianopic pupil requires special efforts to demonstrate its presence. It is done by throwing a
bright narrow beam of light from the field of hemianopia that produces less constriction than
light thrown from the side of normal field. A bright beam from slit lamp give best result. The
lesion is associated with incongruous homonymous field defect. It is seen in trauma to tem-
poral lobe, abscess in temporal lobe and Schilders disease.
A lesion near the end of the optic tract and pretectal area causes loss of light reflex with
preserved accommodation. This is called light near dissociation.
13
A classical example of light near dissociation is Argyll Robertson pupil due to
neurosyphilis which is not seen in children but there are other causes of light near dissociation
which are put collectively as pseudo Argyll Robertson pupil and are seen in children.
The exact site of lesion in classical Argyll Robertson pupil is not known. Commonly
agreed lesion is pretectal. In Argyll Robertson pupil accommodation reflex is retained while
light reflex is lost but vision is maintained.
Characteristics of Argyll Robertson are
1. It is a bilateral condition.
2. One eye is more involved than the other.
3. It is seen after third decade.
4. The pupil are miotic and irregular.
5. Pupillary light reflex is absent.
6. Pupil dilates poorly with atropine.
7. Accommodation reflex is present.
8. Vision is good.
9. Patches iris atrophy are present these may be mistaken as heterochromia.
10. Orbicularis reflex is often retained.
11. The cases are always associated with positive serological test for syphilis.
Sometimes unilateral Argyll Robertson pupil like reaction has been reported for
which the lesion has to be small that blocks input to only one Edinger Westphal nucleus.
Inverse Argyll Robertson pupil is very rare where the pupillary light reflex is present
with failure of pupil to near reflex is usually associated with failure to converge the eye. Most
probable site of the lesion is Perlias nucleus in mid brain.
Pseudo Argyll Robertson pupil
In these condition, the pupil is generally normal in size or mildly dilated but light
reflex is poor. There may be spasm of accommodation, diminished amplitude of accommoda-
tion. Other presenting signs may be lid retraction, ptosis, nuclear oculomotor palsy, conver-
gence palsy, spasm of convergence, vertical gaze palsy and vertical nystagmus.
12
Commonly
seen in Parinauds syndrome, aqueductal stenosis and aberrant regeneration of third
nerve.
The list of conditions that cause light near dissociation are
1. Pseudo Argyll Robertson pupil.
2. Parinauds syndrome.
3. Aqueductal stenosis.
4. Unilateral Argyll Robertson pupil.
5. Aberrant regeneration of third nerve.
6. Adie-Holmes pupil.
7. J uvenile diabetes.
Efferent pupillomotor defect
Size of the pupil and its reaction to light is a delicate balance between parasympathetic
and sympathetic system. The parasympathetic system is little more effective than its
sympathetic counterpart. As the efferent path of the pupillary reaction is parasympathetic in
nature, slightest under action results in relatively greater loss of pupillary and ciliary reflex.
The efferent pupillomotor fibres may be involved anywhere between the third nerve
nucleus and the eye. It is a peripheral lesion, generally unilateral. It may involve only the
pupil but more common is internal ophthalmoplegia that is a combined paralysis of pupil
and ciliary body. It is present in total third nerve palsy and in total ophthalmoplegia.
However pupil is spared in diabetic third nerve. A third nerve lesion in cavernous sinus has
normal sized pupil because it is also associated with involvement of oculosympathetic under
action. The mydriasis of third nerve palsy in cavernous sinus is counteracted by miosis of
sympathetic, resulting in normal sized pupil in cavernous sinus disease.
Before diagnosing efferent pupillary defect, it is essential to rule out
1. Congenital anomaly of pupil
2. Trauma to iris
3. Instillation of mydriatic and cycloplegic in the eye
Pharmacological mydriasis and cycloplegia is not counteracted by instillation of
pilocarpine but a neurological mydriasis will respond to pilocarpine.
1. The causes in children may be in the mid brain i.e. Pineal tumour, encephalitis,
brain abscess, trauma.
2. Oculomotor nerve
3. Ciliary ganglionAdies pupil
4. Short ciliary nerveTrauma.
In total oculomotor nerve palsy the eyeball is deviated down and out with ptosis and
dilated pupil that does not react either to light or accommodation, convergence is also absent.
Hutchinsons pupil
This is a common pupillary defect due to compression of third nerve due to an expanding
subtentorial or subdural lesion. Commonly seen in head injury, there is always lowering
of consciousness with raised intracranial pressure. The mechanism consists of raised
intracranial pressure that compresses the third nerve either against the pteroclinoid ligament
or dorsum sella causing progressive herniation of brain, resulting acute internal
ophthalmoplegia, which is an ominous sign following head injury requiring early surgical
intervention.
Classical Hutchinsons pupil consists of
1. Initial homelateral miosis due to irritation of third nerve.
2. Followed by moderate dilatation of same pupil with sluggish light reflex and
convergence.
3. Dilatation of pupil with loss of light and convergence
4. Signs of third nerve palsy.
5. Miosis of contra lateral pupil.
6. Bilateral dilated pupil.
Adies pupil (Tonic pupil)
This is an efferent disorder of pupil with light near dissociation. It is caused by
denervation of post ganglionic constrictor fibres and ciliary muscle. Most probable cause is
presumed to be viral in origin. In 80% cases, it is unilateral generally seen in women between
20-40 years. It can be seen less frequently in children. If the tonic pupil in a child is
hypersensitive to methacholine, the child has familial dysautonomia i.e. Riley Day syndrome.
Characteristics of Adies pupil
1. The pupil is large and circular.
2. The pupil when examined in dim light after exposure to usual light is smaller.
3. Light reflex in affected pupil is either absent or very slow or is localised as vermiform
movement of a sector of iris when seen on slit lamp.
4. Near reflex miosis is slow so is redilatation on distant vision.
5. Accommodation is low, amplitude of accommodation is less, relaxation of accommo-
dation is poor resulting in to temporary blurred vision for distance following near
work.
6. Consensual reflex is intact.
7. Vision is normal.
8. The pupil is hypersensitive to parasympathomimetic drops.
Normal pupil does not constrict following instillation of 0.125% of pilocarpine or 2%
methacholine. In Adies pupil the pupil constricts to both the drugs in 30 minutes.
Horners pupil
12,15,16
The pupillary anomaly is part of a syndrome which has following characteristics
1. It is mostly acquired but may be congenital or may be due to a birth trauma.
2. It is generally unilateral.
3. There is mild ptosis not more than 2 mm due to paralysis of Mullers muscles.
There is no change in superior rectus.
4. Upside down ptosis i.e. slight upward shift of lower lid margin due to weakness of
inferior tarsal muscle.
5. Miosis due to unopposed action of sphincter pupillae.
6. Pupil is central, circular, reacts normally to light and accommodation.
7. Heterochromia is seen mostly in congenital cases.
8. Reduced sweating on the side on the lesion if the lesion is below the superior cervical
ganglion.
9. The miosis is greater in dim light.
10. There is increased amplitude of accommodation.
11. Hypotony may be present.
12. Enophthalmos
13. There is rise of skin temperature of the ipsilateral lid and face.
14. Brittle and dry hair
Diagnosis
Diagnosis is easy. A case of unilateral mild ptosis, miosis, enophthalmos with normal
extraocular muscles, preserved light and near reflex is diagnostic.
Diagnosis is confirmed, specially the site of lesion by pharmacological tests.
1. Cocaine test. The test confirms sympathetic denervation. Normal pupil will dilate
with 4%-10% cocaine when instilled in the eye in about 30 minutes to 45 minutes.
One drop of 4-10% cocaine is instilled in each eye. The drop is repeated after one
minute and size of the pupil is noted after 30 to 45 minutes. In a positive test the
affected pupil either does not dilate or dilates poorly irrespective of level of lesion.
2. Paredrine (Hydroxy amphetamine) - One percent paredrine will dilate the pupil
only in pre ganglionic lesion and not in post ganglionic lesion.
3. Adrenaline test - Adrenaline 1:1000 is instilled in both eyes.
(a) In normal eyes, pupil will not dilate.
(b) In pre ganglionic lesion both pupil will not dilate.
(c) Horners pupil will dilate with 1:1000 adrenaline. Phenylepherine in 1% drop
does not dilate normal pupil but will dilate a pupil in post ganglionic Horners
syndrome.
In children, common causes of Horners syndrome arebirth trauma due to
stretching of brachial plexus, mediastinal and neck neuroblastoma. Surgical or accidental
trauma to mediastinum or neck may also cause the defect.
Horners syndrome may be associated with cluster headache i.e. Raeders para-trigeminal
syndrome.
REFERENCES
1. Duke Elder S. : Polycoria in System of Ophthalmology. Vol. III, Part II, pp. 592-596,
2. Glasser J .S. : Essential anisocoria in Neurophthalmology. pp. 174 : Harper and Row,
London, 1978.
3. Brain W. Russel : The pupil and the eye lids in Diseases of nervous system. Sixth
edition, pp. 71-75, Oxford University Press, London, 1962.
4. Kanski J .J . : Abnormalities of the pupil in Clinical Ophthalmology. Second edition,
pp. 472-475, Butterworth, London, 1989.
5. Seal S.K. : Pupillary pathways and reflexes in G.N. Seals Textbook of Ophthalmol-
ogy. Fifth edition, pp. 21-25, Current Book International, Kolkata, 2002.
6. Rosenberg M.A. : The pupil physiology and anatomy in Principles and practice of
ophthalmology. Vol. III, First Indian edition, pp. 1943-1951, Edited by Peyman G.A.,
Sander D.R. and Goldberg M.F. J ay Pee Brothers, New Delhi, 1987.
7. Natchair G. Subhuram P. : Normal and abnormal pupils in Modern Ophthalmology.
Vol. 2, Second edition, pp. 941-944, J ay Pee Brothers, New Delhi, 2000.
8. Duke Elder S. : Autonomic effectors in System of Ophthalmology. Vol. VII, pp. 535-
9. Garg A. : Mydriatics and cycloplegics in Textbook of ocular therapy. First edition,
pp. 139-146, J ay Pee Brothers, New Delhi, 2001.
10. Garg A. : Miotics in Textbook of ocular therapy. First edition, pp. 78-85. J ay Pee
11. Samant P.M., Hemalini Samant and Nabar S.M. : Abnormal pupillary reaction in
Ophthalmology theory and practice. First edition, pp. 313-316. Bhalani Publishing
House, Bombay, 2004.
12. Harley R.D. : Pretectal pupillary defects in Pediatric ophthalmology. Vol. II, Second
edition, pp. 785-787, WB Saunders Company, Philadelphia, 1983.
13. Shirley H. Wray : Argyll Robertson pupil in Manual of ocular diagnosis and therapy.
Third edition, pp. 357, Edited by Devorah Pavan Langston, Lippincot Williams and
Wilkins, Philadelphia, 1991.
14. Dutta L.C. : Argyll Robertson pupil in Ophthalmology principle and practice. First
edition, pp. 404. Current Book International, Calcutta, 1995.
15. Kanski J .J ., Thomas D.J . and Holmes ; Adies pupil in The eye in systemic disease.
Second edition, pp. 64, Butterworth - Heinnemann, London.
16. Laties A.M. : Horners syndrome in Textbook of Ophthalmology. Ninth edition, pp.
516, Edited by Scheic H.G. and Albert D.M., WB Saunders Company, Philadelphia,
1977.
17. Gittinger J .W. : Horner s syndrome in Manual of clinical problems in
Ophthalmology. First edition, pp. 190-192, edited by Gittinger J .W. and Asdourian
G.K. Little Brown and Company, Boston, 1998.
CHAPTER 14
Disorders of Optic Nerve in Children
Optic nerve, the second cranial nerve is not a true nerve. It is a white matter tract of
the brain
1, 2, 3
that joins the ganglion cells of the retina to the chiasma. It does not have power
to regenerate. Hence severance of optic nerve leaves permanent loss of its function. Optic
nerve has five types of nerve fibres out of which presence of the first two i.e. the afferent
visual and afferent pupillary fibres have been proved beyond doubt. Presence of efferent
fibres to retina, photostatic and autonomic fibres have not been proved conclusively.
Each optic nerve extends from the optic nerve head in the globe to the anterior part of
the chiasma in the brain.
The length of the optic nerve varies between 3.5 cm to 5.5 cm in average person. How-
ever there is large variation not only from person to person but may also be in two sides in the
same person. It is comparatively short in children. It has been divided into following parts
4
:
1. Intraocular 0.7 mmThe smallest part
2. Intra orbital 3.0 cmThe longest part
3. Intra canalicular 6.0 mm to 1.00 cm.
4. Intra cranial 1.0 cm
The intraocular part extends from the surface of the optic nerve to the posterior end
of scleral foramen. The visible part of the intraocular part is called the optic nerve head or
papilla. The optic nerve head is a circular structure of about 1.5 mm width. In children it is
smaller in size. It is not myelinated. It has pink appearance due to multiple fine capillaries on
its surface. If the capillaries become less the papilla loses its colour and becomes pale. The disc
of a new born is pale. There is an eccentric pale saucer shaped depression in the centre of the
disc. This is called cup of the disc. From the bottom of the cup emerge the central retinal
vessels. The floor of the cup may have blue dot like appearance. They represent the underlying
lamina cribrosa.
The intraocular part is divided into two parts i.e. pre lamilar and retro lamilar
4
. The
retina stops short at the temporal side of the disc but the underlying choroid continues up to
the disc margin resulting in a choroidal crescent in the form of a visible pigmentary cres-
cent. If both choroid and retina fall short of the optic nerve head, the resulting pale crescent is
called scleral crescent.
The intraocular part passes backward through a vent in the sclera called scleral canal
that is narrower anteriorly. The scleral canal is bridged by lamina cribrosa, a sieve like
510
DISORDERS OF OPTIC NERVE IN CHILDREN 511
structure. Through the gaps of sieve pass the bundles of optic nerve fibres. The lamina is
slightly curved backwards.
The optic nerve anterior to the lamina cribrosa in non-myelinated. Myelation begins
behind the lamina. The optic nerve is the last nerve in the body to be myelinated.
3
Intraorbital part. The intra orbital part of the optic nerve is the longest part of the
optic nerve. It extends from back of the globe to the anterior end of the optic canal. The optic
nerve is not a straight structure, it has a downward bend in the anterior part and a medial
bend in the posterior part. This curve prevents the optic nerve from being stretched. It be-
comes straight in extreme lateral gaze and proptosis.
The intra orbital part lies in the centre of the muscle cone surrounded by the orbital fat.
It is covered throughout, its intra orbital cover by dura, arachnoid and pia. These coverings
are continuous with meninges of the brain. The dura is the outermost and the pia is the inner-
most layer. The dura is continuation of intra cranial dura that passes through the optic canal
and on reaching the orbit divides into two, the outer becomes periorbita while the inner
continues to travel on the optic nerve up to back of the sclera where it merges with the outer
surface of the sclera.
The arachnoid lies deeper to the dura. In between lies the sub dural space, unlike
brain the sub dural space of the optic nerve is only a potential space. The arachnoid also blends
with the sclera. The space between the arachnoid and the pia ends blindly at the posterior part
of the sclera but continues intra cranially. The space is filled with cerebro spinal fluid. The
central retinal artery and central retinal vein passes through the sub arachnoid space from
beneath to get into the optic nerve, 10-12 mm behind the globe. During its course through the
sub-arachnoid space, the vein may be pressed by the increased CSF pressure, resulting into
edema of the disc.
At the apex of the orbit, the nerve is surrounded by annulus of Zinn from which the
four recti originate. The superior and medial recti at their origin are closely attached to the
dural sheath of the optic nerve. Stretching of this attachment when the eye is moved causes
pain in inflammation of the optic nerve.
The ciliary ganglion lies lateral to the nerve almost in the middle of muscle cone. The
lower division of oculomotor, the abducent, the nasociliary nerve, the sympathetic fi-
bres and sometimes the ophthalmic veins lie between the nerve and the lateral rectus.
The nerve is surrounded by the long ciliary nerves, short ciliary nerves and pos-
terior ciliary arteries at the back of the sclera.
Most posteriorly the nerve is crossed above by the ophthalmic artery.
The intra canalicular part. This part is about 1 cm in length and is the immobile part
of the nerve. It is well protected in the bony canal. The canal not only transmits optic nerve but
also its meninges, ophthalmic artery, the sympathetic fibres.
The ophthalmic artery has a close relation with the optic nerve. The ophthalmic artery
crosses the nerve inferiorly in the dural sheath. The sphenoidal and posterior ethmoidal si-
nuses lie medial to the optic nerve in the canal. The nerve is separated from the sinuses by
very thin bone, hence infection of the sinuses can infect the nerve with ease.
The intra cranial part. The intra cranial part of optic nerve lies in the middle cranial
fossa. It extends from the posterior end of the optic canal to the anteriolateral angle of optic
chiasma. It measures about 1 cm. It is a flattened band, spear shaped in section with apex
laterally.
The dura and arachnoid that covers the optic nerve from back of the globe to the end of
the optic canal stop short at the back of the optic canal. The intra cranial part is covered by pia
only.
The intra cranial part of optic nerve first lies on the diaphragma sella and then on the
anterior part of the cavernous sinus. The internal carotid lies atfirst below the nerve and
than lateral to the nerve. The opthalmic artery originates from the internal carotid under the
nerve in the middle of the nerve.
Blood supply of the optic nerve :
The blood supply of the optic nerve is divided into supply to :
1. Optic nerve head
2. Intra orbital
3. Intra canalicular and
4. Intra cranial
The main blood supply of the optic nerve is derived from :
1. Internal carotid via its branches
2. Anterior cerebral artery
Blood supply to the optic nerve head is most important in relation to glau-
coma.
Blood supply to the optic nerve head
5, 6
. Blood supply of the optic nerve is derived
from two sources(1) Retinal, (2) Ciliary.
For description, blood supply of optic nerve head is divided into following groups :
1. Blood supply to the surface nerve fibres.
2. Blood supply to pre laminar and laminar region.
3. Blood supply to retro laminar area.
The surface of the optic nerve gets its blood supply from branches of central retinal
artery that anastomose with branches of the prelaminar area.
The prelaminar and laminar area get blood supply from short posterior ciliary ar-
tery.
The retro lamilar area gets its blood supply from both retinal and ciliary circulation.
Development of Optic Nerve
7, 8
The optic stalk is the precursor of the optic nerve. It joins the eye and the forebrain.
The embryonic fissure that develops at the lower side of the optic cup also extends into the
optic stalk. The optic stalk elongates with a groove at its underside. By the end of six weeks
axons from the optic cup (retina) gets into the optic stalk (nerve). At the same time the hyaloid
artery also enters the optic nerve. The axons surround the hyaloid artery.
By third month of gestation small capillaries develop in the nerve. The coverings of the
optic nerve i.e. the pia, arachnoid and dura become well defined by seventh month.
The lamina cribrosa develops partly from the choroid and partly from the sclera.
As the axons of the ganglion cells grow in the optic nerve, some retinal cells get cut off
from the main body and get separated as a small clump of glial tissue. This clump of glial
tissue forms the early optic nerve head. At birth the raised area atrophies to form the optic
cup.
Optic nerve is myelinated very late. Myelination of the optic nerve actually starts in the
lateral geneculate body
9
and spread towards the eye. By birth the myelination is complete and
stops generally behind the lamina.
Congenital Anomalies of Optic Nerve
Congenital anomalies of optic disc are common
10
. They may vary from complete ab-
sence to megalo papilla
11
both of which are very rare. The congenital anomalies of optic
nerve are rarely confined to the optic nerve itself. It is generally associated with developmen-
tal anomalies of retina, uvea, central nervous system and mid facial defects.
They are caused mostly due to defect in
1. Closure of embryonal fissure.
2. Failure of axons to reach the optic stalk.
3. Failure of hyaloid system to atrophy.
4. Midline facial defects.
The common congenital anomalies can be clinically divided into
1. Opaque nerve fibre
2. Abnormality of hyaloid system
3. Coloboma Total
Partial
Pits
Morning glory syndrome.
4. Dysplasia
5. Dysversion
6. Pseudo papilledema (pseudo neuritis)
12
Common symptoms of congenital anomalies of optic nerves are :
1. Diminished vision ranging from loss of few lines on Snellens chart to total absence of
vision.
2. Sluggish or absent pupillary reaction.
3. Diminished colour sense.
4. Field defect.
5. Associated symptoms of error of refraction, squint, amblyopia, and nystagmus.
Importance of correct diagnosis of congenital anomalies of optic nerve lies in the fact
that they may masquerade as optic neuritis or papilledema and the child may be subjected to
unnecessary investigations.
Myelinated nerve fibres (opaque nerve fibre). This is most probably the common-
est congenital anomaly of the disc and the retina. Generally myelination of the anterior visual
path begins in the lateral geniculate body and progresses towards the optic nerve head. The
myelination starts late in the intra uterine period (seventh month). The myelination stops
short at the lamina. However due to some unknown causes in some infants, the myelination
extends well beyond the disc and spreads over the axons of ganglion. The myelination is not
equally distributed on the retina. It is generally seen in patches extending from the disc to-
wards the retinal periphery in leaf like pattern. On rare occasions it may spread all round the
disc. Less frequently the opaque nerve may develop away from the disc as isolated patch.
The condition may be bilateral but need not be symmetrical. It is more common among
the boys. It is not present at birth. It takes about a month to be visible in a new born. Hence
it may be considered as a developmental anomaly
13
of the retina.
Symptoms. The symptoms depend on involvement of retina and macula. Patches near
the disc do not cause any visual symptoms. Loss of vision occurs only when the macula is
involved.
Signs. There are no external signs of myelinated (medulated) nerve fibres. However
large patches may give a gray reflex on retinoscopy. The condition is diagnosed following
ophthalmoscopy.
The ophthalmoscopic picture is characteristic and diagnostic. The picture consists of
white, fluffy, irregular patches extending from disc towards the retinal periphery.
The patches are frayed and feathered. The large vessels pass over the white patch on the
periphery but near the disc they are entirely or partially covered by the medulated fibres.
Rarely the medulated fibres may obscure the disc itself. Generally the macula is spared. It
may be surrounded by two arches of opaque nerve fibres one above and the other below.
The medulated fibres do not transmit light hence they produce scotomas. The scotomas
as they are gradual to develop, are negative in nature. The scotomas may cause enlarge-
ment of the blind spot either locally or all round the disc depending upon the spread of the
myelination. The enlarged blind spot may be joined by arcuate nerve fibre defect or there
may be isolated scotoma away from the disc corresponding to peripheral patches.
The myelination are permanent feature. only occasions when they have been seen to
disappear are following optic atrophy, due to demyelination or ischaemia.
10
There are no complications known to be caused by myelinated nerve fibres and the
condition does not require any treatment.
Congenital crescent (conus) of the optic disc. This condition is also a common
ophthalmoscopic finding. The condition presents as a semi lunar white area adjacent to the
optic nerve. The maximum length of the crescent is parallel to the long axis of the disc, which
is invariably oval. The condition is present at birth, does not change its shape or size
with age. Cup of the disc is nearer the crescent. The edge of the disc away from the crescent is
raised. The condition is generally associated with error of refraction which is astigmatic in
nature, both myopia or hypermetropia are common. The cause of astigmatism is due to
associated change in corneal curvature. The crescent may cause nerve fibre defect in the
corresponding area.
The exact mode of formation of crescent is not well understood. Some think it to be a
modified coloboma of the optic nerve head. Hence this is also known as Fuchs coloboma.
The other theory is that it is a fault in mesodermal development of the eye.
The condition itself is symptomless but the child is brought with diminished vision,
which is a commonly associated with feature due to error of refraction.
The treatment is correction of error of refraction.
Congenital Coloboma of the Disc
Congenital coloboma of the disc may be localised to the disc itself or may be associated
with uveo retinal coloboma. The cause of both the forms is faulty closure of the embryonal
fissure, hence the coloboma is situated in the lower part of the disc. The coloboma when asso-
ciated with coloboma of choroid and retina may be extensive enough to spread from pupillary
margin to the optic nerve head. In severe form the retina and choroid may herniate through
the embryonal fissure below resulting in to congenital cystic eyeball. In less extensive case
a chink of uveo-retinal fissure may divide the coloboma in two parts resulting into a bridge
coloboma. This must be differentiated from duplication of optic nerve head that is extremely
rare. In case of congenital coloboma the scleral canal is wider than normal and the nerve fibres
are generally atrophic, the blood vessels are normal.
The symptoms of coloboma localised to disc itself is generally superior nerve fibre defect
only. The coloboma associated with retinouveal coloboma is always associated with gross visual
loss and field defect.
The coloboma of the disc should be differentiated from glaucomatous cup.
There is no specific treatment. The associated error of refraction should be corrected to
give best possible vision.
Morning glory syndrome
10, 14, 15
. Morning glory syndrome is a rare unilateral con-
genital condition. It is perhaps a colobomatous disorder. The optic nerve head is enlarged,
pale and excavated. The base of the cup contains remnants of hyaloid system. The blood
vessels instead of arising from the centre of the disc arise from the rim in spoke of wheel
pattern. The disc is surrounded by rim of chorioretinal tissue that is elevated. This is sur-
rounded by a pale zone all round. The appearance resembles the flower morning glory after
which the syndrome is named. The condition is associated with non rhegmatogenous retinal
detachment. The large lesion may give a gray reflex on, retinoscopy and the condition may be
confused as leucocoria that is excluded by examination with indirect ophthalmoscopy and ul-
tra sonography. There is no known treatment.
Congenital pit of the optic nerve. This condition was considered a rarity. It is prov-
ing to be more common than expected. The incidence is as high as 1 in 10,000 live births
16
. The
condition is generally unilateral but in one fifth instances it can be bilateral. The exact
cause of the condition is not known but fault in closure of embryonal fissure is most widely
accepted theory.
The pits are present in childhood but symptoms develop in second or third decade. It is
equally common among boys and girls. It may be present in siblings who may or may not be
symptomatic.
The symptoms vary from no symptoms to severe loss of vision, metamorphopsia. On
examination the effected disc is generally larger than the unaffected. The pits are more
prominent than the holes in lamina. The number, shape, size and position of the pits vary from
eye to eye. There may be one pit or may be multiple pits. The shape may be circular dot like,
slit like or triangular. The pit is mostly seen in inferio temporal quadrant. Pits situated on
temporal side produce more symptoms than on the central part. The pit may be pigmented
occasionally.
The cause of diminished vision is macular involvement. The macular lesion is serous
detachment of sensory retina. The lesion looks similar to central serous retinopathy and is
often mistaken as such. The commonest shape of the macular lesion is tear drop raised area
with apex towards the disc, rarely the lesion may be circular. There may be a gray membrane
on the floor of the disc. The field changes are common. They are - Enlargement of blind spot,
central or paracentral scotoma and arcuate scotoma. The central scotoma may be absolute or
relative. The eyes are externally normal.
In all cases of central serous retinopathy, the disc should be carefully examined by
direct ophthalmoscope or +90D lens. The spread of the lesion is best seen on indirect
ophthalmoscopy.
The detachment of the sensory retina may be : (1) Self limiting, (2) There may be perma-
nent loss of vision due to scarring and subretinal neovascularisation.
The nature and source of sub retinal fluid is controversial, three possible sources are :
(1) CSF
(2) Vitreous and
(3) Leak from the capillaries.
10
As the exact etiology and natural history of the disease is not well understood, there is
no specific treatment. Laser photo coagulation of the leaking vessels is most logical treatment
but the result have not been uniform.
The differential diagnosis consists of :
1. Central serous retinopathy
2. Small coloboma of the disc.
3. Traumatic maculopathy
4. Subretinal neovascularisation.
Hypoplasia of the optic disc
10, 12, 17
This is again one of the congenital anomalies that was thought to be rare but the preva-
lence is more than previous estimates.
The effected nerve is smaller than usual. It is almost one third of the average size. The
colour vary from pink to pale white. The retinal vessels are of normal size and distribution.
The hypoplastic disc is surrounded by a halo of hypo-pigmentation of retina and choroid. The
condition is bilateral in sixty percent of children.
The symptoms are mostly visual and cause of subnormal vision.
Vision may be normal or slightly reduced and mistaken for amblyopia and child sub-
jected to anti amblyopia treatment. However in severe form of hypoplasia the vision may be
reduced severely.
In presence of severe loss of vision, there is generally an afferent pupillary defect.
Many a times the children are brought with squint and nystagmus.
The hypoplastic optic disc may be seen as an isolated defect in otherwise normal eye or
the eye may have associated aniridia, nystagmus, absent foveal reflex.
The optical canal is narrower on the involved side. This is a good diagnostic feature in
unilateral cases where the small optic foramen and canal can be compared with the normal
side.
The condition may be associated with mild to severe deformity of the central nervous
system, which are more common in bilateral cases. There may be deformity of ventricle, such
patients have short stature and low level of growth hormone.
The exact cause of hypoplasia of optic disc is not understood. The commonest theory
advanced is primary failure of retinal ganglion cells and nerve fibres in otherwise normal
retina
18
. An absence of axons in the nerve reduce the size of the disc.
There is no specific treatment. Children with bilateral cases may have to be treated as
visually challenged children.
Bergmeisters Papilla
The normal lens during its development gets nutrition from the hyaloid artery that
extends from the optic disc, passes through the vitreous and reaches the lens. It normally
regresses during the development of the eye and should disappear at birth. However in some
children it may be seen in patches in the anterio posterior axis of the eye.
When the posteriormost part fails to regress, the condition is called Bergmeisters
papilla. The papilla is in fact, is a central core of vascular tissue surrounded by fibroglial
tissue.
Sometimes there may only be fibroglial tissue that looks like a translucent membrane
over the disc or may project in the vitreous.
The condition does not cause any symptoms. No complications are known to arise from
it, hence, the condition does not require any treatment.
Drusen of Optic Nerve
Drusen is not a congenital anomaly in true sense as it is not detectable clinically at
birth. It is better called a developmental anomaly. There is no structural change in the tissue
of the optic nerve. Exact cause of drusen is not known, it is said to be due to excess of neurolgia
that undergoes degeneration and calcification.
12
The drusens contain calcium,
mucopolysaccharides, carbohydrate and iron.
19
There is strong heredity. It is inherited as autosomal dominant trait. The drusen may
be seen in siblings and parents. This feature is so constant that in all cases of suspected
drusen in the eye of a child, fundus of his parents and siblings should be examined. If they
have fundus picture suggestive of drusen the child may be spared of costly investigations for
papilledema.
Drusen is bilateral in 75% of cases. It is equally seen in boys and girls. It is rarely
detectable in first decade. They become well established in second and third decade. In ad-
vanced case the drusen may be visible as an irregular growth over the disc.
About 0.3% of children have drusen but they go undetected because drusen by itself
does not cause any visual symptoms. Presence of drusen comes to light on routine examination
for causes other than ocular. Mostly the children are referred as cases of papilledema.
To begin with drusens are deep in the optic nerve head, always anterior to lamina and
not visible by direct ophthalmoscope. Examination by Hruby or other suitable lens may give
away their presence. Number of drusen may vary from solitary to multiple, when more than
one, they may join to form a larger mass.
Ophthalmoscopic finding of drusen are :
1. The colour of the disc is pinkish pale but not hyperemic.
2. Blood vessels on the disc have abnormal branching.
3. Spontaneous vernous pulsation is present in 80% of the eye.
4. The optic cup is shallow to begin with, gradually it fills up and after years, the surface
of the disc may be beyond the plane of retina, which is normal.
5. The veins are not dilated and can be traced to the centre of the disc.
6. On fluorescein angiography there is no leak. The drusen shows auto florescence.
7. Ultrasonography is helpful as drusens are good reflector of ultrasound wave even at
low decibel.
8. CT of optic nerve head may show buried drusen.
Field changes. Depending upon the position of the drusen in relation to retinal fibres,
the field changes can beenlargement of the blind spot, arcuate or nerve fibre defect. Con-
striction of lower nasal field is most characteristic.
18
Differential diagnosis. Other causes of elevated disc in children i.e. papilledema, neu-
ritis, pseudoneuritis, hamartoma of the disc, Bergemeister papilla.
Complications. The condition is benign, does not cause blindness. However on rare
occasions there may be haemorrhage in the lesion.
Management. The condition per se does not require any treatment. However child
should be kept under observation for few years least the diagnosis of raised intracranial pres-
sure has been missed. This is best done by serial photographs of the disc.
Pseudo neuritis. Pseudo neuritis sometimes mentioned as pseudo papilledema is nei-
ther neuritis nor papilledema in true sense. Both of which are acquired. It is better to call it
congenital disc swelling. The condition is very common and unless examined carefully, the
child is subjected to lots of unnecessary investigation.
The condition is reverse coloboma of the disc where the scleral canal is large and
nerve fibres are deficient. In pseudo neuritis the scleral canal is smaller for number of nerve
fibres. The condition is ectodermal in origin.
In 80% cases it is bilateral, may be seen in other members of the family.
The condition is common with hypermetropia and hypermetropic astigmatism. How-
ever it can be seen in emmetropes as well.
The condition is a asymptomatic. It is generally discovered when children are examined
for symptoms of hypermetropia. In such cases the corrected vision remains less normal.
The fundus picture is diagnostic. The disc is small, the margins are indistinct. There is
disc swelling that does not exceed +3D. The blood vessels are normal in calibre, and distribu-
tion. The retina round the disc is normal, no exudates or haemorrhages are seen. The retina
has a peculiar appearance known as shot silk look. There are no field changes. The pupil are
normal.
Differential diagnosis consists of all the conditions that cause swelling of the optic disc.
They are drusen of optic nerve, remnants of hyaloid system, Bergmeister papilla, neuritis and
papillaedema.
Fluorescein angiography differentiate pseudoneuritis from early papillaedema and neu-
ritis.
The condition is non progressive and symptomless, hence it does not require any spe-
cific treatment except prescription of glasses for error of refraction when present.
Disorders of Optic Nerve
Disorders of the optic nerve can beCongenital or acquired.
The acquired cases can be :
1. Infective and/or inflammatory
2. Non infective or non infective.
(a) Papillaedema
(b) Degeneration
(c) Dystrophy
(d) Traumatic
(e) Neoplastic
(f ) Nutritional
(g) Vascular
Disorders of optic nerve not only produce visual disturbance that be temporary or
permanent but also be indicators of serious systemic disorders that may not only be vision
threatening but also fatal.
Symptoms of optic nerve disorder :
1. Visual. Commonest symptom is diminished vision that may range from slight re-
coverable vision loss to total and permanent loss. However, there may not be any
visual loss in the beginning as in papilledema.
2. Amaurosis. Temporary sudden loss of vision (with recovery) is common in
papilledema.
3. Colour sense is always affected in disorder of the optic nerve. It is not a total loss of
colour vision but the coloured objects look dull especially the red coloured objects.
4. Light brightness is reduced.
5. Field changes. Central and centrocecal field changes are very common in inflam-
mation of optic nerve. In papilledema there is enlargement of blind spot. Peripheral
constriction of field is sign of onset of optic atrophy in neuritis and papilledema.
6. Pupillary changes. Afferent pupillary defect is common when the afferent path-
way is involved. It is very common in children.
7. Pain on movement of the eyeball is common in acute inflammation of posterior
part of the optic nerve.
The disorder of the optic nerve can be present at birth as congenital anomaly or
present as developmental anomaly some year after birth or it may be acquired.
The acquired condition can be :
1. Inflammatory and/or infectious
2. Non inflammatory and/or non infectious.
In the first group come papillitis, optic neuritis and retrobulbar neuritis.
The second group has a long list of conditions that include :
1. Papilledema
2. Degenerations
3. Dystrophies
4. Trauma
5. Toxins
6. Nutritional
7. Neoplastic
8. Vascular
9. Unclassified.
Papilledema
22
Papilledema
23
is not a disease. It is a sign that reflects a more serious primary sys-
temic condition mostly intra cranial. Papilledema has great diagnostic, therapeutic and
prognostic value. It reflects the pressure in the intra cranial structures. It also represents
the pressure gradient between the pre lamilar and retro lamilar part of the disc.
The term papilledema should be used only to denote bilateral passive edema of the
optic disc due to raised intracranial pressure. The retro lamilar part of the disc is not
involved in papilledema. The rise of intracranial pressure may be due to lesions of intracranial
structures or the cause may be systemic. The latter is less frequent than the former.
Other cause of elevation of the disc should be designated as disc swelling only. The
causes of which can either be orbital or ocular.
Papilledema can be seen in all ages equally in both sexes all over the world and does not
have any genetic predisposition. However in children it can be seen as early as one year.
It is very common between one to five years.
20
However it should be remembered that other
age groups are not immune.
Papilledema develops fast in children because the location of the primary lesion is
such that it causes more embarrassment to CSF flow than others. In children infra tentorial
lesion both infective and neoplastic are common causes of papilledema. Progress of papilledema
is slow before the sutures are united. However a fast growing growth may cause separation of
sutures and cause silver beaten impression on the cranium.
Common growths that cause papilledema are tumours of cerebellum, fourth ventricle,
tempro sphenoidal lobe of cerebral hemisphere and mid brain tumours.
20
Tumours of pons do
not produce early papilledema. All patients with papilledema may not have intracranial tu-
mour.
Papilledema develops fast and subsides slowly.
21
In case of acute intracranial
hemorrhage, it may develop with in 2-8 hours. Otherwise it may take 1-5 days to develop in
case of raised CSF pressure. There is no possibility of papilledema subsiding spontaneously. It
may regress only when the primary cause of raised intra cranial pressure has been eliminated
either surgically or medically. In such occasions it takes 6-8 weeks for papilledema to start
subsiding.
Pathogenesis
23
. The exact pathogenesis of development of papilledema is not clear.
One fact that is universally accepted in production of papilledema is rise of CSF pressure. The
other factors are secondary to raised CSF pressure.
Following are some possible mechanism to cause raised intracranial pressure.
1. Block in circulation of CSF by congenital lesion like aqueduct stenosis or by acquired
condition like an intracranial tumour.
2. Space occupying lesions
3. Retarded absorption of CSF due to cortical venous thrombosis or block of arachnoid
villi by blood or protein.
23
Most widely accepted theory is derangement of axoplasmic flow secondary to raised
intra cranial tension that forces cerebrospilal fluid under pressure to throttle the central
retinal vein as it passes through the sub-arachnoid space round the optic nerve. The in-
creased pressure in the sub arachnoid space changes the pressure gradient behind the lamina.
There is intracellular axonal swelling which is followed by extra cellular edema.
Commonest disorders that cause papilledema in children are :
1. Congenital anomalies Various forms of craniostenoses, syrengiomyelitis.
2. Infection Meningitis, encephalitis
Lateral sinus thrombosis
Brain abscess, tuberculoma
3. Trauma Intracranial hemorrhage
Subarachnoid hemorrhage
Subdural hematoma
4. Tumours Benign
Malignant
Secondary
5. Drugs Hyper vitaminosis A
Steroid over dose
Tetracyclin
Nalidoxic acid
6. Miscellaneous Pseudo tumour cerebre
Hydrocephalus
Hypoparathyroidism
Cysticercosis
Leukemia
Symptoms of papilledema. Symptoms of papilledema are essentially symptoms of
raised intracranial pressure. The symptoms are directly proportionate to level of intracranial
pressure and its duration.
Papilledema of short duration are generally seen following head injury with extensive
intracerebral bleeding, subarachnoid haemorrhage or encephalitis. Systemic symptoms of all
these conditions overshadow ocular symptom.
Symptoms of papilledema can be grouped into : (1) General, (2) Ocular
General symptoms consist of headache, nausea, vomiting, neurological deficiency.
Cases associated with infective process may have fever.
Headache. Headache may be acute or chronic, there is no localisation of pain. It is
generally worse in the morning and worsened by coughing, sneezing and straining. Location
of pain in the cranium have no localising value.
Vomiting is generally projectile, it may be inter-spaced by nausea.
Neurological deficiencies are late to develop.
Ocular symptoms differ in early and late stages.
Early ocular symptoms are mostly visual.
First visual symptom is sudden loss of bilateral vision for less than a minute with recov-
ery to original level. The blurring of vision may be just like a veil that lifts spontaneously, to
total loss of light sense. The colour sense during these episodes are also diminished.
Diplopia. Diplopia may be the presenting sign, it is generally due to sixth nerve palsy.
There may be skew deviation.
Late ocular symptoms are :
1. Diminished visionSudden diminished vision in fully developed papilledema is due
to haemorrhage over the macula or infarction in the optic nerve itself.
Gradual loss of vision is due to onset of secondary optic atrophy that may culmi-
nate in loss of perception of light. There may be associated diminished colour sense.
2. Diplopia persists and may become permanent unless the cause of papilledema is re-
lieved.
Signs of papilledema. In initial stages of papilledema, the eyes look normal. Onset of
paralytic squint in a child with headache, vomiting is an ominous sign and always points
towards possibility of papilledema. A sluggish pupillary reaction, afferent pupillary reaction
and absent pupillary reaction are seen only when secondary optic atrophy has set in.
Ophthalmoscopic signs. Most important signs are ophthalmoscopic.
Ophthalmic signs can be divided into following groups
24
1. Early papilledema
2. Established papilledema
3. Late papilledema (chronic)
4. Optic atrophy (atrophic)
Early papilledema. Diagnosis of papilledema on the basis of ophthalmoscopic findings
is one of the great riddles in clinical ophthalmology. It needs to be corroborated with careful
history and other investigations i.e. field changes, fluorescein angiography, X-Ray, CT, MRI
etc.
1. The earliest change is blurring of disc margin that does not follow a set pattern but
generally it starts at upper pole followed by lower then nasal and lastly the tempo-
ral margin.
2. Hyperemia of the disc due to capillary dilatation.
3. Filling of optic cup, swelling of the disc.
4. Swelling of the disc is most important sign. It may have to be examined by +90D to
confirm.
5. Loss of spontaneous venous pulsation - It should be remembered that in 20% of
persons venous pulsation is absent so its absence is not confirmatory but presence of
venous pulsation excludes papilledema.
6. Blurring of peripapillary nerve layers. They look striated.
Established papilledema. Once papilledema is well established, diagnosis is not
difficult.
1. The veins become dilated.
2. The disc surface is elevated over the surrounding retina which itself may be edematous.
Difference of 3D between the top of the disc and retina means elevation of 1 mm. The
disc may develop as much as 2 to 3 mm of elevation. The blood vessels over the disc
may be obscured by edematous nerve fibres.
Exudates and haemorrhages. Initially the exudates are soft cotton wool in appear-
ance associated with flame shaped haemorrhages. As the condition progresses changes de-
velop on the macula in the form of hard exudates that radiate from the centre of the fovea
towards the disc in the form of a macular fan.
Chronic papilledema. It takes months to develop chronic changes where the eleva-
tion is maximum almost like a small pink mushroom in which the retinal vessels disappear.
By this time, the exudates and haemorrhages disappear.
Secondary optic atrophy. The edema of the disc diminishes and acquire a milky white
appearance. The gliosis develops not only on the disc but also spreads on the blood vessels
round the disc in the form of sheathing. The lamina remains invisible. Generally it takes 6 to
8 months to develop secondary optic atrophy.
Investigations in papilledema
25, 26
. Fundus fluorescein angiography is useful in
differentiating pseudo elevation from true papilledema i.e. drusen, pseudoneuritis and high
hypermetropia. Fluorescein angiography does not differentiate papilledema from papillitis,
both show diffuse staining with profuse leaking that spreads into surrounding nerve layers.
Field changes. Fundus changes are not conclusive in papilledema. The two changes
seen are :
1. Enlargement of the blind spot. This happens only when the papilledema is well es-
tablished.
2. Peripheral constriction of field, this is met with in stage of secondary optic atrophy.
X-Ray skull, CT and MRI are used to pinpoint the location and size of the intracranial
lesion.
Differential diagnosis of papilledema :
Papilledema should be differentiated from all other causes of disc edema i.e.
1. Papillitis
2. Pseudoneuritis
3. Drusen of optic nerve
4. Bergmeister papilla.
Unilateral Disc Swelling
It is better not to term unilateral disc swelling as papilledema. Though the basic pathol-
ogy is the same i.e. venous stasis with derangement of axoplasmic flow, the lesion may ex-
tend well beyond the lamina, which never happens in papilledema.
The causes can be :
1. Ocular and
2. Orbital
Ocular causes of disc swelling are :
1. Papillitis
2. Posterior uveitis
3. Hypotony
4. Acute rise of intraocular pressure.
Orbital causes of disc edema are Retro bulbar growths may be inflammatory like
specific granuloma, orbital abscess, non specific pseudo tumour of orbit.
Treatment. Management of papilledema is essentially management of its cause. Once
intraocular pressure has been reduced to normal, the papilledema starts regressing. It takes
six to eight weeks for papilledema to subside. Vision may improve with regression of edema.
However nerve fibre damage may have some residual effect.
Vision lost due to optic atrophy is permanent. In sudden rise of intra cranial pressure
I.V. mannitol, may relieve the tension. In case of head injury, use of mannitol may be given in
consultation with neurosurgeon. In chronic papilledema, the mass may be treated medically
when infective. Growths both benign or malignant are removed surgically. Sometimes a shunt
may be required to drain CSF in the peritoneum in the form of ventriculo peritoneal or
lumbo peritoneal shunt.
Local fenestration of optic nerve sheath has not been reported to give uniform good
result.
Optic Neuritis
26, 27, 28
The term optic neuritis is non specific that includes all the conditions, which have loss
of central vision, dyschromatopsia and central field defect, which can be acute or chronic
due to involvement of optic nerve from papilla to beginning of the chiasma.
In 20% to 30% cases no cause can be found and are put in group of idiopathic optic
neuritis. Other causes in children are :
1. Inflammation/infection
2. Demyelination
3. Toxic
4. Hereditary
5. Post vaccination
6. Degeneration
Direct invasion by micro-organism is rare. Most of the time, it is the inflammatory proc-
ess of microbial infection that is responsible for optic neuritis, which can be
26
Ocular
Orbital
Brain and its coverings
Systemic
Ocular. The optic nerve is direct extension of axonal fibres of the retina so it is very
easy for infection to travel from retina to optic nerve in the form of neuro retinitis.
Other causes are choroiditis, endophthalmitis, panophthalmitis, sympathetic
ophthalmia.
Orbital causes are Orbital cellulitis, thrombophlebitis, arteritis, Wegner granulo-
matosis, other granulomas, foreign bodies, secondary to sinus diseases.
Intracranial diseases :
Meningitis
Encephalitis
Meningo encephalitis
Systemic disease :
Tuberculosis
Various types of pneumonias
Poliomyelitis, influenza, mumps, measles.
Demyelination. Demyelination is a major cause of optic neuritis in adult. It is less
commonly seen in younger children but children in teens may be effected by demyelination.
Two common forms are Devics neuro myelitis optica and Schilders disease.
ToxicMany drugs when given systemically can cause optic neuritis. Common drugs
areethambutal, isoniazide, streptomycin, chloramphenicol, fluoroquinolones, metronidazole,
chloroquin. The list is very long. One thing worth noting is that almost all of them are widely
used for systemic diseases in children.
Hereditary. Lebers optic neuropathy.
According to location of the lesion, optic neuritis can be :
Papillitis. Inflammation of optic nerve head.
Neuroretinitis
Retrobulbar neuritisAcute or chronic.
If the papillo macular bundles are predominantly involved. It is called axial neuritis.
If the peripheral nerves are involved, it is perineuritis.
General features of optic neuritis. It is generally an acute condition mostly unilat-
eral. Simultaneous symmetrical involvement of other side is rare. However bilateral in-
volvement is more common in children than adults. Optic neuritis is slightly more com-
mon in girls. Incidence of optic neuritis after fifth decade is less than in second and third
decade.
The main symptoms are :
I. Visual that consists of :
1. Diminished central vision
2. Dyschromatopsia
3. Defective contrast sensitivity
4. Defective stereopsis
5. Uthoff phenomenon
6. Scotoma
II. Pain in the eye
III. Neurological symptoms
Vision in optic neuritis :
1. Unilateral acute loss of vision that may deteriorate within hours to days.
Vision may go down to perception of light.
2. Recovery of vision without treatment starts by second week and original vision
is reached by four to five weeks.
3. Normal vision with signs of central scotoma which is relative, diminished colour
and depth sense.
4. Failure to recover vision generally, is common in adults who have multiple scle-
rosis is not seen in children.
2. Dyschromatopsia. The coloured objects look drab and washed out. There is no
real colour blindness.
3. Defective contrast. The surrounding area looks dim as if the lights in the room
have been turned down.
Defective stereopsis. The patient may not be able to judge the depth well.
Bilateral optic neuritis is commonly seen in Devics neuromyelitis optica, Schilders dis-
ease, Acute disseminated encephalo myelitis, post vaccination status.
Uhthoff phenomenon. In this phenomenon vision is decreased if body temperature is
raised either by exercise or taking hot food and drink, hot bath, hot weather, and anger. Even
stress and anxiety can precipitate the phenomenon. Previously positive Uhthoff effect was
considered to be diagnostic of multiple sclerosis. It has been pointed out that there are other
conditions like Lebers optic neuritis, toxic neuropathy, Friedreichs ataxia where this effect
can be positive.
Scotoma. Commonest field defect is central scotoma, which is more for colour than for
white. Scotoma for red is most effected, other scotomas are centrocecal and paracecal, nerve
bundle defect and even sectorial peripheral field loss is possible. Kind of scotoma is directly
related to type of nerve fibres involved. In axial neuritis where central i.e. papillo macular
fibres are involved the scotoma is central.
Pain in the eye. In case of optic neuritis patient may complain of pain with or without
movement of eye. Pain on movement of eye is more common in optic neuritis and may be
absent in papillitis. It is more marked if the lesion is near the apex of orbit. There are two
possible explanations :
1. Due to contraction of superior or medial rectus muscles. The dural sheath that is pain
sensitive gets stretched and pain is precipitated.
2. The sheath of optic nerve is stretched in swelling of the nerve and becomes painful.
There may be tenderness at the insertion of superior rectus.
Pain may precede loss of vision sometimes.
Fundus in optic neuritis :
1. Early changes consist of blurring of disc margin and hyperemia. In some cases disc
may look normal in spite of diminished vision and central scotoma when lesion is
away from the disc.
2. It may be difficult to differentiate between fully developed optic neuritis and early
papilledema.
3. Late changes are pallor of disc.
4. Fully developed neuritis is always associated with cells in the vitreous in front of the
disc.
Neurological symptoms. Sometimes patient have numbness of limbs which the pa-
tient may ignore because loss of vision is more alarming than numbness. In neuromyelitis
optica the disc involvement may precede myelitis or vice versa.
Optic Neuritis in Children
52
Optic neuritis in pediatric age group differs from adult optic neuritis in many ways
though pathology in both age groups is the same.
1. Optic neuritis in children is less frequent than adults.
2. Both boys and girls are affected equally.
3. Optic neuritis in children is bilateral more often than in adults.
4. Involvement of anterior part of nerve is more common in children.
5. Commonest cause of optic neuritis is viral infection. Optic neuritis starts two to three
weeks after onset of systemic viral infection.
6. Optic neuritis is a frequent complication of many vaccines. Optic neuritis sets in
after 2-3 weeks of vaccination. Antiviral vaccine cause neuritis, more commonly than
antibacterial vaccines.
7. Multiple sclerosis that is a major cause of optic neuritis in adult inflicts children less
frequently.
8. Severity is more in children. Initial loss of vision is profound and disc swelling rela-
tively more than in adults.
9. Chances of visual recovery in children is very good.
10. Optic neuritis in children responds well to steroids.
Management of optic neuritis. Management of optic neuritis is unsatisfactory. There
is no specific treatment that will be equally effective in all cases. All efforts should be made to
find out the cause as far as possible by neuro imaging, CSF examination, X-ray, chest X-Ray,
PNS X-ray orbit.
Thirty percent cases are idiopathic and recover without treatment. In idiopathic optic
neuritis recovery of vision following initial abrupt loss of vision take few weeks and complete
recovery may take as much as 6-8 months. If there is no improvement in recovery phase, the
child should be investigated for some other cause.
Cortico steroids are given only to reduce inflammatory process and hasten recovery.
They do not cure neuritis, nor do they influence ultimate recovery. They do not reduce chance
of relapse.
Some Specific Type of Optic Neuritis
1. Post vaccination optic neuritis. One of the untoward side effects of prophylactic
vaccination against host of diseases, both viral and bacterial diseases, are delayed optic neuri-
tis which is more frequent in antiviral vaccination than bacterial immunisation. It develops
two to three weeks after vaccination, is generally bilateral and self limiting. Common
prophylaxises that are known to cause optic neuritis are - Hepatitis B, rabies, measles, mums,
rubella, and tetanus toxoid.
2. Neuro neuritis. Commonest cause of neuro retinitis in children is toxoplasmosis.
Other systemic causes like syphilis, tuberculosis which are common in adults are generally do
not cause neuro retinitis in children.
3. Retro bulbar neuritis. Acute retrobulbar neuritis is common in older children
due to acute sinusitis. Chronic retrobulbar optic neuritis which is also known as toxic amblyopia
is unknown in children. Retrobulbar neuritis may not show signs of inflammation on optic
nerve head.
4. Drug induced optic neuritis. Drug induced optic neuritis is common following
administration of anti tubercular drugs i.e. streptomycin, isoniazide and ethambutol,
another drug used commonly in children is chloroquine. Fortunately chloramphenicol is no
more used as routine drug for typhoid hence incidence of chloremphenicol induced optic neuri-
tis is rare. Drugs may involve any part of the optic nerve. Retro bulbar involvement is more
common. Management consists of withdrawal of the drug whenever possible. In some instances
vision may not return to normal even after withdrawal of offending drug due to irreversible
neuronal damage. In such cases other possibilities should be excluded by complete physical
examination, CT and MRI.
Demyelinating diseases. Demyelinating diseases are common systemic causes of
visuocortical tract involvement that may spread from disc to lateral geniculate body and even
the optic radiation
56
. The common demyelinating diseases that involve optic nerves are
53
1. Multiple sclerosis
2. Devics neuromyelitis
3. Schilders disease
4. Acute disseminated encephalomyelitis
5. Krabbes disease.
Multiple sclerosis
54, 55
is a very prominent cause of optic neuritis in adults. It is not a
frequent cause of optic neuritis in children. One third of all patients with multiple sclerosis
develop optic neuritis, sometimes or other in the course of disease that may last for 20 to 30
years. About thirty percent of acute idiopathic optic neuritis develop multiple sclerosis. In
fifteen percent of cases optic neuritis is presenting sign of multiple sclerosis. Spinal cord is the
commonest site of involvement. Visual loss is unilateral with deep central scotoma and marked
colour defect. Diminished nerve conduction is the cause of visual impairment. There may
be an afferent pupillary defect. There is pain on movement of the eyeball. There may be
involvement of any of the cranial nerve that supply the extra ocular muscles in various combi-
nation. Muscles may be involved in inter nuclear ophthalmoplegia Uhthoff sign is common, so
is Pulfrich phenomenon in which the patient has better vision in dim light.
Devics neuromyelitis optica. This is seen in children and young adults but progresses
well into pre senile age. It involves boys and girls equally. It is an acute disease that starts as
acute, bilateral loss of vision, which need not be equal in two eyes. Myelitis, which is a
constant feature may follow neuritis or precede it. It involves the spinal cord, and the optic
nerve. Other cranial nerves are spared. It may involve sympathetic chain as well.
Visual field defects depend upon presence of demyelination in the optic nerve.
Myelitis results in paraplegia. It is generally acute in nature and profound. Most pa-
tients show considerable improvement in motor function, some residual effect is universal.
Some visual recovery is the rule.
Recurrence of neuritis and myelitis is common. It should be differentiated from other
causes of bilateral optic neuritis.
Fundus changes depend upon part of the optic nerve involvement and its severity. It
may range between normal disc when the lesion is far back in the nerve, to frank swelling
when the lesion extends in the pre lamilar part.
Schilders disease
56
. This is a genetically determined in born error of metabolism
where there is primary degeneration of adrenal gland. The disorder belongs to a group of
disorders known as leukodystrophies. The condition has profound neurological deficiencies
besides loss of vision. Loss of vision is due to neuritis that is followed by atrophy of optic
nerve. There may be cortical blindness. It is associated with hearing loss, slurred speech,
mental retardation, ataxia, ophthalmoplegia and nystagmus. There is no known treatment.
Krabbes disease
56
. This is also a leukodystrophy. There is relentless demyelination
of not only the optic nerve but also that of cerebrum. There is mental retardation, diffuse
sensory loss and motor loss. Blindness is very common not only due to involvement of optic
nerve but also optic radiation. There is no specific treatment.
Comparison between optic neuritis, papilledema and pseudo neuritis. Many a
times it is difficult to differentiate between fully developed optic neuritis and early papilledema.
Same is true between pseudo neuritis (pseudo papilledema) and early papilledema
ophthalmoscopically. Other clinical features and investigations have to be gone through when
in doubt.
Field change Central or centrocecal,
may have nerve fibre
defect
Enlargement of the
blind spot initially nor-
mal. Peripheral constric-
tion late.
Nil
Pupil May show afferent de-
fect
Pupillary reaction ab-
sent if third nerve is in-
volved or optic atrophy
has set in
Nil
Other neurological as-
sociation
Generally none Signs of raised intra cra-
nial tesion
Nil
Fundus Variable degree of disc
swelling not more than
3D. May be normal ini-
tially, blurring of disc
margin, few flame
shaped haemorrhages,
macular star absent, vit-
reous haze over the disc
Swollen disc more than
3D. Absent venous pul-
sation. Edema of
macula. Macular star.
May have flame shaped
haemorrhages.
Small hyperemic disc
not more than 3D. No
haemorrhage, no exu-
date.
Fluorescein
angiography
Non specific, Minimal
leakage of dye.
Vertical pool of dye due
to leakage.
Normal
CT No specific change. May show intracranial
lesion
No change
MRI Shows plaque in
demyelination
Intra cranial lesion. Normal
Signs and
symptoms
Papillitis Papilledema Pseudo neuritis
Age Any, rare after 50 years Any rare before the su-
tures close
Any
Sex Both Both Both
Onset Acute Slow Present from birth
Bilaterality Generally unilateral Bilateral Bilateral, may be uni-
lateral
Vision Rapid fall of vision Good, fall of vision is late Normal, may have er-
ror of refraction.
Cause of visual loss Early conduction defect Involvement of macula
and late conduction
defect
Nil
Other visual defects Defective colour sense,
stereopsis and contrast
Transient loss of vision Nil
Causes of unilateral disc swelling in children :
1. Ocular
57
:
Papillitis
Drusen of optic nerve head
Ocular hypotony
Severe intraocular inflammation
Neuro retinitis
Pseudo neuritis
Optic nerve glioma
Orbital cellulitis
2. Intracranial :
Foster Kennedy syndrome
Unilateral high myopia with raised intra cranial tension. The normal eye devel-
ops disc swelling.
Cavernous sinus thrombosis
3. Pseudo tumour ceribri (initial stage)
Sub arachnoid haemorrhage.
Optic Atrophy
Optic atrophy. Optic atrophy is not a disease. It is the end result of many diseases,
where the optic nerve is virtually a scar with loss of its function. It has been defined in various
ways, the sum total of which says that optic atrophy occurs due to degeneration of axons in the
anterior visual pathway.
Anatomically it has been divided into :
1. Ascending optic atrophy where the pathology spreads from the retinal ganglion
cells towards lateral geniculate body.
2. Descending optic atrophyHere the degeneration spreads from lateral geniculate
body to optic nerve.
Other classifications are :
On the basis of ophthalmoscopic appearance :
1. Primary (simple) optic atrophy
2. Secondary optic atrophy Post neuritic
Post papilledematous
3. Consecutive optic atrophy
4. Cavernous optic atrophy
5. Glaucomatous optic atrophy
6. Temporal pallor.
7. Bow tie pallor
Etiological classification
The causes of optic atrophy in children can be :
1. Heredo familial
2. Post papilledematous
3. Post neuritic
4. Trauma
5. Consecutive
6. Toxic
7. Nutritional
8. Metabolic (Juvenile diabetes mellitus, mucopolysaccharidosis)
Primary optic atrophy. In this type of optic atrophy no visible cause that may involve
the optic disc i.e. disease of choroid, retina, or optic nerve (optic neuritis) is detectable. There is
no evidence of raised intra cranial tension. The lesion is in the second visual neuron proximal
to disc.
30
This is due to degeneration of optic nerve fibres with minimal glial proliferation without
any mesenchymal reaction.
31
The myeline sheath and axis cylinders disappears, there is shrink-
age of optic nerve. At the disc there is reduction in nerve tissue, this results in increase in
width and depth of optic cup and exposes the holes in lamina cribrosa. The optic cup becomes
pale. The cause of whitening was thought to be paucity of capillaries on the disc head.
30
Now it
is thought to be due to impaired transmission of light into the substance of the disc. This is
brought about due to absence of axonal fibres, which are responsible for transmission of light
32
.
Commonest cause of primary optic atrophy in adults is tabes dorsalis which is not seen
in children. In children the cause of primary optic atrophy are - hydrocephaly, toxic, retrobulbar
neuritis, traumatic opticneuropathy, heredo familial optic neuropathy.
The clinical presentation consists of visual, pupillary change and pallor of disc.
Visual symptoms consist of diminished distant vision, central scotoma, colour defi-
ciency, reduced contrast sensitivity and stereopsis.
Pupillary changes consist of large pupil and afferent pupillary change. In bilateral pri-
mary optic atrophy both pupil are large and sluggish. Bilateral immobile pupil is sign of gross
loss of vision.
Fundus change. The media is clear. The margin of the disc is clear cut. The colour of
disc has been called by various adjectives i.e. chalk white, marble white or paper white. The
number of vessels on the disc is less than ten.
33
However on fluorescein angiography, the
number of capillaries are more than visible on ophthalmoscope but atrophic, no leak or pooling
is seen. The cup is deeper than normal physiological cup and may be mistaken as glaucoma-
tous. The width of the cup is also more than normal. The lamina cribrosa is visible as bluish
dots. The vessels have normal calibre and do not show any sheathing. The peripapillary retina
and choroid are normal. The primary optic atrophy is an example of descending optic atro-
phy where the lesion may be anywhere from retrobulbar part of optic nerve to the optic radia-
tion.
33
Secondary optic nerve. This is more common than primary optic atrophy in children.
This is always preceded by swelling of the disc either due to inflammation, which is associ-
ated with active congestion or with passive congestion as in papilledema or uniocular disc
edema.
The atrophy is also a degenerative process of the optic nerve, have more reactive elements
than primary atrophy.
The degenerative process is obscured by proliferation of astrocytes, glial tissue and to
some extent blood vessels.
It is more common than primary optic atrophy in all ages.
The condition is equally common following either papillitis or papilledema. In chil-
dren secondary optic atrophy is usually bilateral.
The symptoms of secondary optic atrophy are similar to that of primary optic atrophy,
only difference is that the former is preceded by symptoms of either papillitis or papilledema.
The signs are also same as far as field changes and pupillary reaction is concerned. The
main difference lies in fundus picture.
The fundus picture. The media is not affected. The margin of the disc is blurred. The
disc may be raised in spite of onset of atrophy. In fully developed secondary optic atrophy, the
swelling subsides but never regresses in the disc, thus the physiological cup is always obliter-
ated and lamina is not visible. The vessels always show some degree of sheathing. The retina
and choroid are not affected. The disc has dirty yellow colour.
It is not possible to differentiate between post papilledematous and post papillitis optic
atrophy.
The field when possible to chart shows enlargement of blind spot. Onset of atrophy is
heralded by gradual constriction of peripheral field, ultimately only a central island of vision
may be left.
Consecutive optic atrophy. This is an ascending type of optic atrophy, which is
essentially secondary to disease of retina, choroid or both. It may be unilateral or bilateral.
The fundus picture comprises of : The disc is pale but colour is waxy white. The
disc margin is almost normal but not as clear cut as in primary optic atrophy. The cup is
maintained unless there was a concurrent involvement of optic nerve as seen in neuroretinitis.
The calibre of the blood vessels depend upon their involvement in primary chorioretinal le-
sion. They are attenuated in retinal abiotrophy i.e. retinitis pigmentosa. The surrounding
fundus shows evidence of chorioretinal scar.
In case of a macular lesion, the atrophy is generally confined to temporal side. In case of
patch of choroiditis adjacent to the disc, the part of the disc near the lesion is atrophied so is in
case of choroidal rupture near the disc.
Commonest cause of consecutive optic atrophy is retinitis pigmentosa. Other causes
are diffuse chorioretinitis, toxic retinopathy, extensive photocoagulation.
33
The symptoms of consecutive optic atrophy is overshadowed by symptoms of primary
chorioretinal pathology.
Cavernous optic atrophy. This type of optic atrophy is rare in children. There is
mucoid degeneration of the optic nerve head. There is no proliferation of glial tissue. On histol-
ogy empty spaces are formed in between the nerve fibres which are filled with mucoid mate-
rial.
32
The fundus picture is in between primary optic atrophy and glaucomatous optic atro-
phy, there is prominent pale cup in pale nerve with nasal shift of blood vessels.
Temporal pallor. This is partial optic atrophy due to involvement of papillo macular
bundle. Commonest cause is a lesion either in the macula or in between the macula and the
disc. It could be a congenital anomaly of macula i.e. macular coloboma, toxoplasmal
chorioretinitis or rupture of choroid between the macula and disc. Laser burns too near the
disc on temporal side. Other causes are toxic neuritis, old retro bulbar neuritis, heredofamilial
optic atrophies, Lebers optic atrophy, Behrs optic atrophy, Kjers optic atrophy.
Some specific types of optic atrophy :
Traumatic optic neuropathy. Trauma to optic nerve direct or indirect is the cause of
traumatic optic atrophy. The injury can be a closed head trauma, an injury to the frontal bone,
or a trivial injury on the eyebrow. The optic nerve can be injured anywhere from chiasma to
entry of nerve in the sclera. It can be avulsed if the eyeball is caught in a curved object and
pulled anteriorly besides avulsion such injuries can stretch the optic nerve, can cause haemor-
rhage in the sheath. The optic nerve may be severed by a bullet injury, fragment of granade, or
by a stab injury in the orbit. The intraorbital part may be injured during retrobulbar injection,
or during facio orbital surgery.
In avulsion of optic nerve, there is sudden loss of vision with severe pain, may be
associated with mild proptosis and restricted movement. On ophthalmoscopy there are large
blotches of haemorrhages round the disc. Gradually as the haemorrhages clear, a pale disc
almost devoid of any vessels becomes visible. The resultant atrophy is primary optic atro-
phy in nature.
Injury behind the entry of central retinal vessels and optic canal does not pro-
duce any change on fundus examination initially. It takes four to five weeks for the change to
appear in the form of primary optic atrophy. The intracanalicular part of the nerve is most
likely to be injured in closed head injury.
The mechanism of damage to intra canalicular part of optic nerve is :
Rotational and shearing force that push the frontal lobe, pull the nerve back.
The shearing force is transmitted to immobile optic nerve in the canal.
Accumulation of blood or edema may throttle the blood flow of the optic nerve. Bone
fragments from the fractured canal may get lodged in the substance of the nerve or may press
the nerve from outside.
Traumatic optic neuropathy is a common accident in children, more frequent in boys. It
is commonly seen following a cricket ball injury to the lateral side of the eyebrow, in fact any
hard object hitting the frontal bone is likely to cause traumatic optic neuropathy.
It is generally unilateral. Bilateral traumatic optic neuropathy is seen in closed head
injury that damages the chiasma. The symptoms may be acute loss of vision if the injury is
with in 10 mm of the posterior scleral canal and is generally profound. Vision may be reduced
to perception of light, which may be lost altogether. Loss of vision following injury to posterior
part of the optic nerve is variable between moderate loss to loss of perception.
In uniocular profound loss of vision there may be afferent pupillary defect.
Diagnosis of traumatic optic neuropathy is generally delayed due to overwhelming signs
and symptoms of head injury. Diminished unilateral loss of vision following frontal head in-
jury with afferent pupil on the same side is a strong point in favour of traumatic optic neuropathy
that is confirmed by - fundus examination, field charting, CT and MRI. CT is superior to MRI
in outlining fracture of bones. MRI gives better image of soft tissue damage. MRI is contra
indicated if there is suspicion of intra orbital or intra cranial metallic foreign body that should
be excluded by plain X-ray first.
Treatment consists of :
1. Management of head and cranio facial injury. This should get priority over ocular
treatment because these can be life threatening to the child.
2. Ocular therapy should be started as soon as the child is stable.
Ocular treatment is :
1. Medical. This consists of heavy dose of I.V. steroid. The drug of choice is methyl
prednisolone as 30 mg/kg
35
as loading dose followed by 5.4 mg/kg/hr for 48 hours to
72 hours or dexamethasone 3-5 mg/kg/day is given if vision improves. Methyl
prednisolone is continued for 2-3 days and then replaced by oral prednisolone in ta-
pering dose. If there is deterioration of vision while on oral steroid, I.V. methyl
prednisolone is restarted. If there is no improvement in first 72 hours following ini-
tial start of I.V. methyl prednisolone, surgery should be considered which consists of
fenestration of optic nerve sheath or removal of roof of optic canal via trans nasal or
trans ethmoidal route by endoscope.
Heredo Familial Optic Neuropathy
36, 37, 38
Heredo familial optic neuropathies comprise of a group of hereditary degeneration of
optic nerve that manifest under 15 years of age. The causative factor may be present at
birth but the condition manifests late. They are generally bilateral, most of the clinical pres-
entation in two eyes is similar. They progress gradually, may terminate in complete blind-
ness, may have only ocular manifestation or may be multi-systemic in nature.
Heredo familial optic degeneration can belong to following groups :
1. Optic abiotrophies
2. Associated with retinal dystrophies
3. Storage diseases.
The common conditions are
39
1. Lebers optic neuropathy
2. Kjers dominant juvenile neuropathy
3. Behrs recessive neuropathy
4. Optic neuropathy in juvenile diabetics.
Lebers optic atrophy
40
. This is a heredo familial optic neuropathy also known as
Lebers disease. This is not to be confused with Lebers congenital amaurosis which is a con-
genital retinal pigment degeneration and not a neuropathy.
This disease is seen between 5 years to 30 years of age, commonest manifestation is at
onset of puberty. Ninety percent affected are boys.
The disease is passed on to children of carrier women who may or may not have the
disease. The affected males do not pass the disease to their children including daughters. The
affected child may have fully developed disease in his sibling or younger siblings may develop
the disease later. Besides brothers there may be positive evidence of disease in maternal
uncles. Most of the sisters of the affected child may be carriers. Female carrier do not have
affected father. The social aspect of the inheritance is that it is not the affected man who
spreads the pathological gene but the unaffected sister.
The biogenetic defect of the disease lies in the mitochondrial DNA of the mother due to
mutation. Several different mutations have been identified.
41
There is defect in :
Oxydative phosphorylation
Cyanide metabolism
Reduced zinc in plasma
The disease starts as rapid fall of vision in both eyes. Occasionally one eye may be
involved earlier than the other. Initial fall may be up to 6/60 or less. The initial fall may
continue in slower rate and stops at lowest level then may start improving. Improvement is
never beyond 6/24. There is partial or total loss of colour sense, stereopsis and contrast. There
is a large dense absolute central or centrocecal scotoma which is surrounded by relative scotoma
or a relative scotoma may become denser and absolute. On fundus examination, the disc may
look normal or may be swollen. The disc develops secondary optic atrophy. The basic pathology
is retrobulbar. The commonest fibres to be affected are papillo macular hence commonest type
of optic atrophy is temporal pallor to begin with which can spread all over the disc. Peripapillary
telangiectasia and micro vasculo angiopathy is common that disappears in late stages. Rest of
the fundus is normal. There are no exudates or haemorrhages as seen in optic neuritis.
The disease can be divided into following stages :
1. AsymptomaticThese are generally the siblings and carriers. They may show
peripapillary telangiectasia and tortuosity of large vessels.
2. Stage of rapid fall of vision.
3. Stage of slow fall of vision and slight improvement of vision.
4. Stage of optic atrophy with some vision.
5. Blindness.
Treatment. There is no specific treatment. In stage of neuritis, systemic steroids have
been used without much success. Commonly used drugs are :
1. High doses of hydroxycobalamin given intra muscular.
2. Oral cystine.
3. Oral zinc.
Fenestration of optic nerve sheath too has not shown good results.
The children with non improvable vision should be given low vision aids and treated as
partially visioned children. They should be encouraged to stay in schools with normal children
and encouraged to learn profession that is compatible with low vision.
Kjers juvenile optic neuropathy. This condition is less common than Lebers optic
neuropathy but commonly diagnosed as Lebers because of its bilaterality and persistent visual
loss. There is a strong family history. The disorder starts between 4 to 8 years. Vision is never
less than 6/60. Never goes to loss of perception of light. There is acquired blue yellow
dyschromatopsia. There is a central or centrocecal scotoma. Peripheral fields are normal. The
optic nerve shows mostly temporal pallor with mild excavation of cup. There is no nystagmus.
No treatment is effective. Low vision aid may help the child.
Behrs recessive optic neuropathy. There are two types of recessive optic neuropathy,
one is called simple and the other complicated.
The simple starts in neonates hence also wrongly called congenital neuropathy. Though
the disease starts in infancy, it is missed unless the eyes are examined with special reference
to vision and changes in the disc. Generally the children are brought for examination round
about school going age i.e. 3-4 years. Visual loss is severe that may be as low as H.M. Nystagmus
is common which is generally associated with achromatopsia otherwise severe
dyschromatopsia is common. However vision remains stationary. Consanguinity is com-
mon.
38
There is no known treatment.
Complicated. Commonest age of presentation is between one to nine years. Loss of
vision is moderate. Children never go blind. In about half the children there is nystagmus and
strabismus, there is moderate loss of colour vision. Disc shows temporal pallor. The vision is
generally stable at 6/60-6/36. No treatment is known.
Optic neuropathy in juvenile diabetics. This has a recessive form of inheritance.
There is severe loss of vision that may be as low as counting fingers. It is a progressive condi-
tion. There is severe colour defect. Nystagmus is absent, there may be hearing loss. The disc
has marked pallor. No treatment is available for optic neuropathy.
Congenital optic atrophy
42, 43
. These rare conditions are generally confused with
heredo-familial degeneration of optic neuropathy. The heredo familial neuropathies are not
present at birth. There are two types of congenital optic atrophy i.e. dominant and reces-
sive. The loss of vision is not severe and stable in spite of dead white colour of disc, and
excavation. The whole of the disc is involved. Nystagmus is common. There may be other
members of the family who have uncorrected visual defect from childhood. There may be
dyschromatopsia mostly for blue colour.
Gray (grey) pseudo optic atrophy
37
. This rare condition is seen in premature chil-
dren, who are born with very poor vision that improves with in few months to normal level. On
examination, the pupil are dilated and fixed. The colour of the disc is grey. The condition is
thought to be delayed myelination of the disc.
42
Tumours of Optic Nerve
44, 49
Tumours of optic nerve are far less common than that of retina. The tumours of optic
nerve can be seen any where from optic nerve head to chiasma.
They can be grouped broadly into two groups
45
1. EctodermalAnterior visual pathway gliomas
2. MesodermalMeningioma.
The former is mostly seen in children and rarely in adults while glioma of the optic
nerve is a benign or marginally malignant tumour in children. Its counterpart in adults is
highly malignant.
Meningiomas are not met with in children.
The optic nerve glioma. It is a slow progressing mostly unilateral painless growth
of substance of optic nerve. On rare occasions it can be bilateral. It can be divided into two
types according to its clinical presentation i.e. glioma of the optic nerve and glioma of the
chiasma. Glioma of optic nerve can arise anywhere in its orbital part. Posteriorly located
tumours are more common than anteriorly located tumours. Occasionally the tumour may
start in the anterior part and spread like a fusiform growth all along its length. Posteriorly
placed gliomas are more likely to invade the optic canal. Similarly a glioma of chiasma may
spread towards the orbit.
The tumour is said to be congenital hamartoma, which does not manifest before 4
years of age. Its association along with neurofibromatosis is seen in 20-30% of cases. Hence in
all cases of neurofibromatosis the optic nerve function should be evaluated that should in-
clude, vision, pupillary reaction, fundus examination, CT and MRI. A child with caf au lait
spot anywhere on the body with diminished vision should be examined for presence of proptosis
and visual function. Neurofibromatosis type I
46
is more likely to be associated with glioma of
optic nerve.
Pathologically glioma arises from neuroglia of the optic nerve. Histopathologically the
tumour is an astrocytoma. It is generally called juvenile pilocytic astrocytoma
47, 49
due to
thread like appearance of astrocytes in the tumour. There are two known patterns of the
tumour :
1. Tumour related to neurofibroma are circumferential, perineural tumour, proliferat-
ing in subarachnoid space.
2. Intraneural tumour is not related to neurofibroma.
48
Presentation. Common age is between 4 to 8 years. It may be diagnosed as late as end
of second decade if the growth is small and slowly progressively.
There are two modes of presentation :
1. Proptosis with diminished vision.
2. Strabismus with diminished vision.
The later happens when diminished unilateral vision has not been noticed because of its
gradual painless nature. Such children are brought mainly for squint and found to have glioma.
Proptosis in case of glioma of optic nerve is axial, of gradual progress. Degree of
proptosis depends upon position of growth, a smaller growth near the orbital apex causes more
proptosis than a larger growth just behind the globe. The growth is always intraconal. It takes
one to three months for a growth to cause proptosis in younger patients
50
. The proptosis is non
tender, non pulsative and non compressible, there is no bruit present and the proptosis does
not increase either by lowering the head or Valselva procedure. Generally examiners finger
can be insinuated all round the globe and the orbit. The movements are normal or may be
minimally reduced.
Strabismus is due to loss of vision.
Occasionally there is rapid progression of proptosis due to mucinous degeneration in
the tumour and not true increase in number of cells.
There is slow and uniform enlargement of orbit.
Rarely in extreme degree of unattended proptosis, cornea may be abraded and develop
ulcer.
Vision. Loss of vision is a common feature of optic nerve glioma and is variable ranging
between loss of few lines on Snellens chart to loss of perception depending upon number of
visual fibres involved.
Generally visual loss is early that deteriorates slowly, proptosis follows visual loss.
Generally there is central or centrocecal field defect.
Loss of vision is due to resultant optic atrophy. Anteriorly placed tumour cause post
papilledematous optic atrophy while posteriorly placed and chiasmal lesions produce primary
optic atrophy.
Pupillary reaction also depends upon involvement of afferent pupillary fibres. Poor vi-
sion is associated with afferent pupillary reaction.
Diagnosis of optic nerve glioma is not difficult in cases of moderate glioma which cause
unilateral loss of vision with proptosis in a child under ten years of age.
Investigation
1. X-ray :
(a) X-ray of orbit shows uniform enlargement of orbit behind the orbital rim without
any intraocular or intra orbital calcification.
(b) X-ray optic foramen (Rhese view) shows circular enlargement of optic foramen
with intact margin due to slow growth of the tumour. If the posterior end of the
glioma has not extended in to the optic canal the x-ray of the foramen is unal-
tered and equal on both sides. In early cases X ray of both the foramen should be
taken for comparison.
When the growth extends in the optic canal, it too is enlarged most of the time in a
funnel shape i.e. wider anteriorly. Size of the foramen is reduced following removal of growth.
2. Ultrasonography of orbit shows uniform fusiform or secular growth inside the cone
of the eye.
3. CT and MRI are useful in early cases.
4. Fine needle biopsy has not proved to be more useful than CT, MRI or X-ray.
Management. Management of optic nerve glioma is controversial. There is no medical
treatment available. The main argument revolves round the question if the growth should be
left as such or be surgically removed.
Points against surgical intervention are :
1. It is slow progressive benign growth that is self limiting and after some time stops
growing.
2. By removing the growth, the vision can not be restored.
3. The growth has to be removed either by a lateral orbitotomy, craniotomy or
endoscopically via trans nasal, trans ethmoidal route that requires help of
neurosurgeon and ENT surgeon.
Only point in favour of surgical intervention is cosmetic. To a growing child, a large
proptosed strabismic eye is not cosmetically acceptable.
Surgical options available are :
1. Tarsorrhaphy if cornea is compromised before definitive surgical procedure.
2. Removal of the growth along with the eye if the exposed cornea has sloughed and
perforated.
3. Removal of the growth leaving blood vessels either via orbitotomy, craniotomy or by
transnasal and trans ethmoidal endoscopic surgery.
Radio therapy is not of much use unless the growth is malignant, which is rare in chil-
dren.
Chiasmal glioma
50, 51
. Chiasmal gliomas are more common than isolated orbital glioma
but are not diagnosed as early as orbital glioma for obvious reason that there is no proptosis
which is a hallmark of optic nerve glioma and unilateral loss of vision in child may go unno-
ticed unless the child develops squint or nystagmus.
The chiasmal gliomas have same pathological features as optic nerve glioma except that
they are more likely to be invasive than optic nerve glioma.
Chiasmal gliomas may produce bilateral loss of vision due to spread to the contra lateral
side of the midline.
Chiasmal glioma may extend :
1. In the optic canal and become intraorbital that results in proptosis.
2. Extend into the third ventricle and cause internal hydrocephalus, raised intracranial
pressure, and hypothalamic signs and symptoms of precocious puberty, obesity, som-
nolence, dwarfism and diabetes insipidus. There may be involvement of pituitary as
well.
The presenting features are :
Loss of vision, initially unilateral that may become bilateral. Vision may vary between
mild to severe. In late cases there may be loss of perception as well.
Squint and nystagmus are common.
Afferent pupillary reaction is seen in unilateral involvement. In bilateral involvement
both pupil are large and sluggish.
Field changes of chiasmal glioma are variable, so long the growth has not crossed the
mid line, the field changes are same as in optic nerve glioma. The other eye may also show
unilateral field change of optic nerve type i.e. central or centrocecal scotoma.
Bi-temporal hemianopia, which is expected in chiasmal lesion is absent in chiasmal
glioma.
X-rayOptic canal : Widening of optic canal means intra orbital extension.
X-ray skull lateral view shows change in sella that appears either as Jor W shaped sella
without supra sellar calcification.
Management of chiasmal glioma is difficult more so when there is extension in the sur-
rounding structure. Palliative radio therapy may arrest the growth.
Tumours of optic nerve head :
Growth of optic nerve head may be benign or malignant.
Benign growths are : Drusen of optic nerve that may be intra neural and non visible
ophthalmoscopy or may protrude in the vitreous as irregular grey mass. Other such growths
are : neurofibromatosis and tuberous sclerosis.
Another rare growth is benign melanocytoma which is not common in children.
Remnants of hyaloid system may be mistaken as tumours arising from the disc. A ma-
lignant tumour that invades optic nerve head in children commonly is retinoblastoma. It is
always associated with visible retinoblastoma and means extra ocular spread. Similarly
neuroblastoma may occasionally invade the optic nerve head
45
so can leukaemia.
REFERENCE
1. Nancy Anderson, Apple D. ; Optic nerve in Principles and practice of ophthalmol-
ogy. Vol. I. p-5962, Edited by Peyman G.A., Sanders D.R. and Goldberg M.F. Jay Pee
2. Duke Elder S. ; Optic nerve in System of ophthalmology. Vol. II. p-273294, Henry
3. Banumathy S.P. ; Optic nerve in Anatomy of the eye. p-6873, Edited by Natchair G.
Arvind Eye Hospital, Maduari.
4. Nema H.V. ; The optic nerve in Anatomy of the eye and its adnexa. Second edition.
5. Shields M.B. ; Optic nerve head and peripapillary retina in Text Book of glaucoma.
Fourth edition, p-7277, Williams and Wilkins, Philadelphia, 1997.
6. Anderson D.R., Braveman S. ; Re-evaluation of the optic disc vasculature. Am.J.Op.
82:165, 1976.
7. Nancy Anderson Hamming and Apple D. ; Anatomy and embryology of the eye in Prin-
ciples and practice of ophthalmology. Vol. I. p-1314, Edited by Peyman G.A., Sand-
ers D.R. and Goldberg M.F. Jay Pee Brothers, New Delhi, 1987.
8. Duke Elder S. and Cook C. ; Embryology of the eye in System of ophthalmology.
Vol. III, part I. p-109118. Henry Kimpton, London, 1963.
9. Dutta L.C. ; Congenital anomalies of the retina in Ophthalmology principles and
practice. First edition. p-151153, Current Book International, Calcutta, 1995.
10. Kanski J.J. ; Congenital optic disc anomalies in Clinical ophthalmology. Second edi-
tion. p-451455, Butterworth, London, 1989.
11. Duke Elder S. ; Anomalies of optic disc and nerve in System of ophthalmology. Vol. II.
p-668687, Henry Kimpton, London, 1964.
12. Shirly H. Wray ; Congenital optic disc anomalies in Manual of ocular diagnosis and
therapeutics. Third edition. Edited by Deborah Pavan Langston, p-341342,
Littlebrown.
13. Duke Elder S. ; Medulated nerve fibres in System of ophthalmology. Vol. II. p-646
14. Radhika Tandon, Verma L. ; Optic disc pit in Clinical practice in ophthalmology.
First edition. Edited by Saxena S., p-494, Jay Pee Brothers, New Delhi, 2003.
15. Shields J.A., Stephen R.E. and Sarin L.K. ; Morning glory syndrome in Pediatric oph-
thalmology. Vol. II. Second edition. Edited by Harley R.D., p-1152, WB Saunders Com-
16. Mukherjee P.K. ; Congenital pit of the optic nerve in Current ocular therapy. Fifth
edition. Edited by Fraunsfelder F.T. and Roy F.H., p-558, WB Sanuders Company, Phila-
delphia, 2000.
17. Glaser J.S. ; Optic disc - congenital anomalies of shape and size in Neuro ophthalmol-
ogy. p-7374, Harper and Row, London, 1978.
18. Martyn L.J. ; Optic nerve hypoplasia in Pediatric ophthalmology. Vol. II. Second
edition. Edited by Harley R.H. p-831, WB Saunders Company, Philadelphia, 1983.
19. Levine R.A., Rosenberg, M.A. and Rabb M.A. ; Drusen in Principle and practice of
ophthalmology. Vol. III. Edited by Peyman G.A., Sanders D.R. and Goldberg M.P.
20. Dutta L.C. ; Papilledema in Modern ophthalmology. Vol. II. Second edition. p-977-
21. Shirley H. Wray ; Papilledema in Manual of ocular diagnosis and therapy. Third
edition. Edited by Deborah Pavan Langston. p-343, Little Brown.
22. Kuhn F., Halda, T. ; Optic nerve in Optic differential diagnosis. Edited by Roy, H.
p-618619, Williams and Wilkins. Baltimore, 1996.
23. Manikavasagan T. ; Pathogenesis of papilledema. Recent concept in Neuro ophthal-
mology. Edited by Natchiar G. p-1101 to 11-04, Arvind Eye Hospital, Maduri.
24. Kanski J.J. ; Papilledema in Clinical ophthalmology. Second edition. p-447448,
25. Badrinath S.S. ; Papilledema in Sankar Netralaya Clinical practice and pattern
in ophthalmology. First edition. p-288-289, Jay Pee Brothers, New Delhi, 2004.
26. Sundarajan S. ; Optic neuritis in Neuro ophthalmology. Edited by Natchiar G.
p-12.112.8, Arvind Eye Hospital, Maduri.
27. Horton J.C. ; Disorders of the eye in Harissons principles of internal medicine.
14th edition. Vol. I. p-159172, McGraw Hill Book Company, 1988.
28. Brain W.R. ; Lesion of optic nerve in Diseases of nervous system. 6th edition, p-125
132, Oxford University Press, London, 1962.
29. Bajandas F.J. and Kline L.B. ; Pale optic disc in Neuro ophthalmology review
manual. Third edition. p-135140, Jay Pee Brothers, New Delhi, 1989.
30. Miller S.J.H. ; Degeneration of the optic nerve : optic atrophy in Parsons diseases of
the eye. Seventh edition. p-229230, Churchill Livingstone, 1984.
31. Lois J. Marthyn ; Opticatrophy in Pediatric ophthlmology. Vol. II. Second edition.
Edited by Harley RD. p-817820, W.B. Saunders Company, Philadelphia, 1983.
32. Levine R.A., Rosenberg M.A., Rabb M.F. ; Optic atrophy in Principles and practice of
ophthalmology. Vol. III. p-21252129, First edition. Jay Pee Brothers, New Delhi,
1989.
33. Hebber K.G. ; Optic atrophy in Neuro ophthalmology. Ist edition. Edited by Natchair.
34. Dutta L.C. ; Optic neuritis : Optic neuropathy in Modern ophthalmology. Vol. II.
Second edition. p-956962, Jay Pee Brothers, New Delhi, 2000.
35. Miller M.R. ; Traumatic optic neuropathy in Current ocular therapy. Edition V.
p-567568, Edited by Fraunfelder F.T. and Hampton Roy F., WB Saunders Company,
Philadelphia, 2002.
36. Sorsby A. ; Maldevelopment defectsLebers disease in Modern Ophthalmology.
Vol. III. First edition. p-311319, Butterworth, London, 1964.
37. Lois Martyn ; Lebers hereditary optic atrophy in Pediatric ophthalmology. Vol. II.
Second edition. p-818820, W.B. Saunders Company, Philadelphia, 1983.
38. Glasers J.S. ; Heredo degenerative optic atrophies in Neuro ophthalmology. p-93
100, Harperz Row, New York, 1977.
39. Bajandas F.J. and Kline L.B. ; Pale optic disc - optic atrophy in Neuro ophthalmol-
ogy. Review manual. Third edition. p-139, Jay Pee Brothers, New Delhi, 1989.
40. Gittinger J.W. ; Lebers optic atrophy in Manual of clinical problems in ophthal-
mology. First edition. p-167168, Edited by Gittinger J. and Asdourian G.K. Little Brown
and Co., Boston, 1998.
41. Seal S.K. ; Retrobulbar neuritis in G.N. Seals Text Book of Ophthalmology. Fifth
edition. p-340341, Current Book International, Kolkota, 2002.
42. Duke Elder S. ; Congenital optic atrophy in System of ophthalmology. Vol. III, part II.
43. Sorsby A. ; Congenital optic atrophy in Modern ophthalmology. Vol. III. p-264-265,
44. Green C.H. ; Orbital gliomas in Ocular pathology. First edition. p-181-183, Blackwell.
Scientific publication, Oxford, 1963.
45. S.J.H. Miller ; Primary tumours of optic nerve and its sheath in Parsons diseases of
the eye. Seventh edition. p-335337, Churchill Livingstone, London, 1984.
46. Hoyt W.F., Baghdassarian S.A. ; Optic glioma of childhood; natural history and ration-
ale of conservative treatment. BJO 53:793-798, 1969.
47. Levine R.A., Rosenberg M.A., Rabb M.F. ; Glioma in Principles and practice of oph-
thalmology. Vol. III. p-21362142, Edited by Peyman G.A., Sander D.R. and Goldberg
M.F. First Indian edition. Jay Pee Brothers, New Delhi, 1987.
48. Lois J. Martyn ; Optic glioma in Pediatric ophthalmology. Vol. II, Second edition.
Edited by Harley R.D. p-839841, W.B. Saunders Company, Philadelphia, 1983.
49. Nicholson D.H. and Green W.R. ; Glioma in Pediatric ophthalmology. Vol. II. Second
edition. Edited by Harley R.D. p-12591262, W.B. Saunders Company, Philadelphia,
1983.
50. Natrajan M. ; Tumours of the optic nerve in Neuro ophthalmology. Edited by Natchiar.
51. Glaser J.S. ; Optic gliomas in Neuro ophthalmology. p-116120, Harper and Row,
London.
52. Radhika Tandon and Verma L. ; Optic neuritis in children in Clinical practice in
ophthalmology. First edition. p-480, Edited by Saxena S. Jay Pee Brothers, New Delhi,
2003.
53. Scheie H.G., Albert D.M. ; Optic neuritis in Text Book of ophthalmology. 9th edition.
p-39, W.B. Saunders Company, Philadelphia, 1977.
54. Dutta L.C. ; Multiple sclerosis in Modern ophthalmology. Vol. II. Edition II. p-958
55. Radhika Tandon and Verma L. ; Optic nerve disorders in Clinical practice in oph-
thalmology. Edited by Saxena. S., First edition. p-477495, Jay Pee Brothers, New
Delhi, 2003.
56. Levine R.A., Rosenberg, M.A. and Rabb M.F. ; Degenerative optic neuritis in Princi-
ples and practice of ophthalmology. Vol. III. First Indian edition. p-21152129,
Edited by Peyman G.A., Sanders D.R., Goldberg M.F. Jay Pee Brothers, New Delhi,
1987.
57. Miller S.J.H. ; Diseases of the optic nerve in Parsons diseases of the eye. Nineteenth
edition. p-224, Churchill Livingstone, London, 1984.
CHAPTER 15
Errors of Refraction in Children
1, 2, 3, 4
A normally developed eye (by 5 to 6 years of age) acts as a convex lens of +60D. This
power is divided into two major components i.e. the corneal and lenticular. The converging
power of cornea is +43D. This leaves the lens with +17D of power, normal aqueous and
vitreous contribute a negligible converging power.
In an eye with normal refractive (diopteric) power, parallel rays are brought to focus on
the retina with accommodation at rest, the parallel rays actually form a circle of least
diffusion
4
. This refractive status is called emmetropia. An emmetropic eye has clear image
of a distant object without internal adjustment of its optics.
6
In contrast to this, if all parallel
rays are not brought to focus on the retina in all the meridian with accommodation at rest, the
condition is called ametropia.
Almost all emmetropic eyes have an axial length of 24 mm, any change in its length
should produce ametropia. However a larger eye can be emmetropic if its total optical power is
stronger.
8
An absolutely emmetropic eye is extremely rare, most of the eyes have some degree of
ametropia.
A full term normal child at birth is about +2 to +3D hypermetropic. This is due to shorter
axial length of the new borns eyeball. As the child grows, this power is neutralised by corre-
sponding lengthening of the eyeball up to 5-7 years when all eyes should become emmetropic.
However, if the increase in length does not stop at this point, the eye becomes myopic and if
the eye fails to reach the emmetropic length (24 mm) the eye becomes hypermetropic.
The ametropia eye can be :
1. Myopic
2. Hypermetropic
3. Astigmatic
Anisometropia is a term used when the diopteric power in both eyes are not the same.
Possible causes of ametropia
4
are:
1. Axial. In this condition, the retina is either in front or behind the circle of least
diffusion. If the focus is behind the retina, the condition is called hypermetropia and if the
image is formed in front of the retina, the condition is called myopia. Axial astigmatism is
very rare. 1 mm change in axial length leads to ametropia of 3D.
546
ERRORS OF REFRACTION IN CHILDREN 547
2. Curvature. If the curvature of the cornea is uniformly less than normal, the eye
develops curvature hypermetropia, similarly reduction in lenticular curvature also results
in curvature hypermetropia. Increase in corneal or lenticular curvature results in curvature
myopia. 1 mm change in corneal curvature leads to 6-7D ametropia.
If the corneal or lenticular curvatures are not equal in all meridian, there will be an
ametropia called astigmatism.
3. Index. If the refractive index of optical system is low, it will result in hypermetropia
and high refractive index will result in myopia.
4. Relative position of lens. A forward shift of lens causes myopia, backward shift
results in hypermetropia. An absence of lens from the pupillary area results in aphakia,
which is an example of high hypermetropia. An obliquely placed lens causes astigmatism.
MYOPA
Myopia is the commonest cause of gradually developing painless diminished vision in
pediatric age group. It is equally common among boys and girls. It has strong heredity.
Fifty percent of myopic children have myopia in both the parents, about a quarter of myopic
children have one parent with myopia, and rest are sporadic. Both the hereditary and sporadic
cases can transmit myopia in the next generation. It is common to have history of myopia in
grand parents from maternal or paternal side. The first cousins too may be myopes. It is very
common to have myopia in all or some of the siblings.
Myopia invariably is a bilateral disorder that manifests simultaneously in both eyes
that need not be equal, generally difference in two eyes is not much.
In simple myopia rest of the eye is generally as normal as any non myopic eye.
Myopia is the error of refraction in which rays from far point, real or apparent (myopic
far point) are focussed in front of photo sensitive layer of retina when accommodation is at
rest.
1, 2, 3
Myopia is also known as short sightedness or near sightedness as the patient finds
it difficult to see beyond a certain distance that may be very short.
8
The word myopia represents the phrase I shut the eye in Greek. This is because a
myopic child narrows the inter-palpebral fissure so much as to make it like a slit. This gives a
pin hole effect that improves vision by few lines on Snellens chart.
Loss of vision in myopia is so gradual that the child fails to notice its onset and progress.
Sometimes the child considers visual status as normal and is resigned to it.
Classification of myopia. Myopia has been classified in various ways, none of the
classifications have universal acceptance.
It could be primary or secondary.
Primary could be congenital or developmental. In secondary myopia some detect-
able cause other than increased axial length of the eyeball is present.
near the eye gives slight enlargement of the image. These two phenomenon makes the child to
keep the reading material close to the eyes.
Myopic eyes are prone for peripheralretinal and vitreous degeneration that make my-
opic eyes more prone for retinal detachment.
Congenital myopia. This is a rare form of myopia. The child is born with eyes longer
than 24 mm, may be as long as 27 to 28 mm. It is mostly unilateral, occasionally both eyes may
be involved. The condition is not diagnosed at birth. It may take as much as two to three years
for parents to be aware of the condition. Unilateral larger eye, squint or nystagmus may
be the early signs to draw attention of the parents. Generally the child is brought for
unilateral squint and on examination presence of myopia is revealed, which is generally 10
diopter and generally does not progress.
9
Congenital myopia is more common in pre-term
babies. It may be associated with other congenital anomalies of the globe i.e. microphthalmos,
aniridia, megalocornea, cataract and retinal separation. Congenital myopia develop all the
complication that are seen in simple myopia i.e. peripheral retinal degeneration and retinal
degeneration.
Management consists of early detection of myopia and optical correction either by spec-
tacles or contact lenses after refraction under cycloplegia. Contact lenses are generally pre-
scribed when the child is grown enough to manage the contact lenses. However, extended
wear lenses may be prescribed in younger children with instruction to parents regarding its
management.
Amblyopia when present should be treated as per standard method. Surgical interven-
tion is not indicated before eighteen years of age.
Simple myopia
10
. This is the commonest form of myopia met in children. It is also
known as developmental myopia. The exact etiology is not known. It is considered to be
derangement of physiology of the developing eye. It is inherited as autosomal dominant.
However, some children may have autosomal recessive inheritance. It is equally distributed
among various races. Boys and girls are equally effected. There is a strong hereditary ten-
dency. Simple myopia is common among siblings. Commonest type of simple myopia is axial
myopia, where the eye is too long for its refractive status. Role of excessive near work has
been unequivocal, so is the role of diet and general health.
Simple myopia is generally progressive but not pathological any progress is mild to
moderate, about one third children have stationary myopia, only 15% have progressive in-
crease in power. Even in progressive form, the vision can be corrected to normal with change
of lens.
The children at birth are not myopic. Simple myopia is generally detected between five
to seven years of age and continues to grow up to late teens. Hence called school myopia
which may start as 1.00 D or less but does not exceed 5D or 8D when fully developed. There
is no known method to stop the growth. Associated astigmatism is common. Another type
of myopia that develops after 15 years of age and progresses into second decade is called col-
lege myopia. It is a low grade of myopia which rarely exceeds 2D to 2.5D diopter and differ-
ence between the initial power and final power is seldom more than one diopter.
As the punctum remotum of a myopic eye is always finite, the myopic eye need not
accommodate. Accommodation worsens the vision. Hence accommodation in simple myopic
eye remains unused. Convergence is within normal range, or may be in excess. The results in
dessociation between accommodation and convergence leading to exophoria which may break
into exotropia.
10
However excess convergence may lead to esotropia as well. A negative
angle alpha in myopia leads to pseudo convergent squint.
Symptoms. Most frequent and striking symptom is diminished distant vision. A child
may have normal distance vision at a given time, which may rapidly deteriorate to 6/24 to 6/36
with in one year. These children may not complain of diminished vision as their vision is good
enough for reading, writing and indoor activities. They find it difficult to see the writings on
the black board in the school, or read the sign boards. These children do not see the television
from usual distance. They have tendency to move nearer to the TV set for better view. These
children prefer to keep books closer to eyes than other children.
As the child has poor distant vision that he takes for granted, the child concentrates on
near work like reading, writing and other indoor activities with less interest in outdoor sports.
They become introvert. They generally do well in studies.
Other symptoms are glare, and asthenopia. Asthenopia is more common in low myo-
pia associated with astigmatism.
Signs. A eye with low degree of myopia is difficult to differentiate from emmetropic eye.
The child may be termed as squinting by parents. The child actually may narrow the inter
palpebral fissure for better vision. This phenomena is mistaken as squint by parents. However
eyes with more than three diopters may look larger than normal eye due to axial increase in
length. Higher degree of myopia may present with pseudoproptosis. The axial enlargement
is confined to posterior sclera.
The cornea is relatively larger and anterior chamber is deeper than normal. The
pupil is larger than normal, may look sluggish on torch light examination but brisk on slit
lamp.
Fundus is within normal range. After few years temporal crescent and peripheral retinal
changes in the form of lattice degeneration may take place putting the child on higher risk
of retinal detachment.
Diagnosis. Diagnosis is simple on the basis of diminished distant vision, good near
vision, positive family history and improvement of vision by three four lines with PH.
The diagnosis should be confirmed by retinoscopy under cycloplegia. Subjective test
with minus glasses that improve should not be relied as that fails to unmask pseudomyopia.
Management of simple myopia. The aim of treatment is to bring the image back on
the retina from its forward position. This is achieved by placing concave glasses in front of the
eye either as spectacles or contact lens. The next alternative available is a group of surger-
ies that flatten the cornea. These surgeries are not indicted in children.
In an attempt to form the image on the retina, the aim should be to give minimum
minus power that gives maximum and comfortable vision.
In children under 7 to 8 years, with low to moderate degree of myopia (up to 6D),
myopia is fully corrected by spectacle. The child is instructed to use the glasses constantly.
This gives a bright image on retina for maximum waking hours. This prevents development of
squint and helps develop normal accommodation convergence reflex. A fully corrected myopic
child does not find it difficult to perform near work with distant correction. The child can read
smallest letter without glasses by bringing the reading material near the eye, hence develops
a habit of reading without glasses. The child should be encouraged to use the glasses for both
distant and near work to develop a proper reading habit.
The spectacles are the most practical form of device to correct myopia, they are easy to
handle. A child as young as one year can be taught to wear them, the spectacles give good and
comfortable vision. They are cheap and the power can be changed with ease. The disadvan-
tages of spectacle correction of myopia consist of minification of image. A child who was accus-
tomed to larger image finds a smaller image unacceptable and too bright. The other disadvan-
tage is unacceptability of glasses in teens for cosmetic purpose. The field is also restricted.
The next alternative is to prescribe contact lenses. They are not suitable for children
under ten years who can not manage to wear and remove them. The first advantage of contact
lens is its invisibility that removes the cosmetic blemish of spectacle. Other advantages are
optical. They give larger image, improved field all around, aniseikoina can be minimised when
present. Greatest disadvantage of contact lens is childs inability to handle the contact lens
and maintain its sterility. They are expensive and may require frequent change.
7
Pathological myopia. This type of myopia is also known as progressive or degen-
erative myopia. It begins as simple myopia but increases relentlessly and always associated
with extensive vitreoretinal degeneration. Macular involvement is common. The refraction
may increase by -3D to -4D every year in contrast to simple myopia that increases by 1D to
1.5D yearly and get stabilised before end of first decade. In small percentage of cases of patho-
logical myopia, the increment in power may extend up to 30 years of age and final may be as
high as 20D to 25D.
The exact cause of pathological myopia is not well understood. Heredity seems to play
a definite role. It runs in families
11
, female are more prone to develop pathological myopia. It
is more common in Asians and rare Africans.
Symptoms. Most prominent symptom is diminished distant vision, which is very poor.
But the child can read and write, in fact can do fine work without difficulty from very close
quarter.
Next common symptom is seeing fine black spots in front of eyes. The spots may range
from single to multiple, mostly in straight or curved lines. These opacities move with move-
ment of the eyes. They are called muscae-volitantes. They are due to degeneration of vitre-
ous. They do not require any treatment. Sudden increases in number of black spots and en-
largement in size of spots require examination of fundus by indirect ophthalmoscope to
detect presence of retinal detachment.
Children with pathological myopia have poor dark adaptation which they may call
night blindness. This is due to extensive peripheral chorioretinal degeneration.
Squint mostly esotropia is common in pathological myopia due to excess of convergence
and under action of lateral rectus.
Sudden profound loss of vision, photopsia, loss of field always means myopic
retinal detachment. Other cause of sudden painless loss of vision are haemorrhage in
front of the macula or large vitreous haemorrhage.
Signs. The signs are similar to those seen in simple myopia. However prominence of
eyeball is more due to stretching of the globe mostly beyond the equator giving an impression
of pseudo proptosis.
Cornea is larger but not as large as in megalocornea or buphthalmos. Any cornea larger
than 12 mm in diameter should be investigated to rule out buphthalmos. AC is deep and pupil
larger and sluggish.
On retinoscopy the retinal glow looks dull and moves slowly against the movement of
the retinoscope, as more and more minus lenses are added, the glow looks brighter. A con-
cave mirror retinoscope is helpful in retinoscopy in progressive myopia. The refraction may
be between 10-20D. Large vitreous floaters may be visible as black spots against pink back-
ground. White reflex amidst pink glow is due to large patches of choroidal degeneration. Ab-
sence of glow in presence of normal anterior segment is caused due to large retinal detach-
ment or vitreous haemorrhage.
Fundus examination. Most striking features of pathological myopia is seen with oph-
thalmoscope. Direct opthalmoscope gives out findings only round the posterior pole. To
have a good view of whole of the retina of every fundus should be examined by indirect oph-
thalmoscope under full mydiasis.
The fundus changes are seen on optic disc, macula, choroid and retina in various
combination. The fundus changes clinically can be divided into early and late changes. The
fundus changes increase with duration of myopia and not always directly proportional to de-
gree of myopia.
The early changes develop in first and second decade
12
consisting of diffuse atrophic
changes in the retina and choroid in the posterior pole, which include pale retina through
which prominent choroidal vessels are seen. The retina gradually gets atrophic, soon a my-
opic crescent develops mostly on temporal side of the disc extending from upper pole of the
disc to lower pole with widest area at temporal side. Occasionally a nasal crescent may also
develop. A complete ring is rare. The crescent is more pale, almost atrophic near the disc and
pink on temporal side that is dotted with choroidal pigment. The crescent gradually becomes
more extensive and spreading towards macula. The nasal margin may overhang, the optic disc
resulting in distortion of retinal vessels. The optic nerve seems to enter the globe obliquely.
The macula in early stages is normal. The retinal periphery may be normal or may show
minimal degenerative changes.
The late changes are :
Optic disc is pale and large. The largeness of the disc is not real, it is optical. The disc
has prominent myopic crescent. Peripapillary crescent is more frequent in late stages. There
may be super traction of retina over the disc. The cup is proportionately large but cup disc
ratio is within normal limits.
Choroid. In early stagesThe choroidal vessels are visible through the pale retina. In
late stages there is atrophy of chroiocapillaries and retinal pigment epithelium. In still late
stages there is total disappearance of choroidal tissue with large patches of atrophy. The
white sclera shines through the atrophic, choroid and retina. There is heaping of pigment on
the periphery similar to be seen in healed patch of choroiditis. The choroidal changes do not
extend in anterior segment.
The macula. The macula develops a dark red circular patch surrounded by a pale ring.
This is called Foster-Fuchs spot of Fuchs fleck. This is due to choroidal thrombosis
13
and
choroidal neovascularisation.
14
Bruchs membrane develops breaks due to over-stretching. These breaks look like
fine yellow irregular lines that may branch. They are more common in young myopes.
They are called lacquer cracks. Presence of these breaks in Bruchs membrane carry poor
prognosis as they may cause necovascularisation and haemorrhage.
14
Peripheral retinal degeneration in the form of lattice, snail-track or cystoid de-
generation are common. They themselves are not dangerous but may result in retinal hole
formation leading to rhegmatogenous retinal detachment which are more common in myopia
than emmetropia or hypermetropia. Trauma to pathological myopic eye is more likely to cause
retinal detachment than in other eyes.
Posterior staphyloma is unique to pathological myopia. It is due to thinning and
stretching of sclera in the posterior pole. The thin sclera becomes ectatic and lined by choroid
with all the layers of retina along with blood vessels. The blood vessels dip in the ectatic area.
On retinoscopy, the posterior staphyloma has more minus power than the surrounding area.
Direct ophthalmoscopy requires more minus addition to see the floor of the staphyloma, which
is better seen by indirect ophthalmoscope. Ultrasonography also shows up its presence,
depth and extent.
Vitreous changes are very common. They are degenerative that include posterior
vitreous detachment, formation of large vitreous opacities, liquefaction of vitreous. Like
all degenerative changes in eye, the changes in vitreous are also not directly proportional to
myopic changes.
Peripheral fields also shows constriction.
Management. Management of pathological myopia is same as in simple myopia except
that pathological myopia does not improve to 6/6 with best correction. 6/12 or 6/18 may be the
best possible correction in pathological myopia. The second problem is more frequent change
in power, the yearly change may be as much as 3D to 4D. It is better to under correct high
myopia by -1 to -2D for more comfortable distant as well as near vision than better and uncom-
fortable distant vision. With increase in power, the image becomes smaller and smaller that
adds to discomfort to the child. As pathological myopia have large and sluggish pupil, they are
more prone to develop glare, which can be reduced by prescribing tinted glasses.
While prescribing spectacles to children it is better to inform their parents that glasses
are being given to improve distant vision. Use of glasses is neither going to stop progress of
myopia nor reduce the power of myopia. It should also be impressed that the glasses are not
specific for reading but should be used for near work to keep balance of accommodation and
convergence.
The next alternative is contact lens. Contact lenses are preferred in high myopia be-
cause they do not cause minification of images as seen in spectacles. They reduce peripheral
distortion that is common with high myopia glasses. Peripheral field also improves with con-
tact lens. Moreover contact lenses are cosmetically more acceptable than spectacles. The dis-
advantages of contact lens are their high cost, frequent change, difficulty in handling by chil-
dren and possibility of non tolerance of contact lenses.
Previously, it was thought that if hard contact lenses, that were flatter than corneal
curvature, if worn for sometime will flatten the cornea and reduce the power. This hypothesis
is known as orthokeratology.
15
The philosophy behind the procedure has not been found to
be acceptable.
Surgical procedures for correction of myopia :
Many surgical procedures have been developed to correct myopia. They can be broadly
divided into following groups :
1. Surgery on cornea
2. Surgery on lens
3. Intraocular lens implant
4. Posterior scleral support
Common surgical procedures on cornea are :
1. Radial keratotomy (RK)
2. Photo refractive keratectomy (PRK)
3. Lasik (Laser in situ keratomileusis)
16
4. Lasek (Laser sub epithelial keratomileusis)
5. Intracorneal ring
Surgical procedures on lens :
1. Clear lens extraction
2. Phakic intraocular lenses
(i) Posterior chamber lens
(ii) Angle supported lens
(iii) Iris supported lens
Of all the surgical procedures mentioned above, only clear lens removal and phakic
intraocular lenses are suitable for myopia in older children. Rests of the surgeries are at
present considered to be contra indicated. They are suitable only after myopia has stabi-
lised which occurs round about twenty-one years of age.
Other forms of myopia :
1. Curvature myopiaCornealKeratoconus
LenticularLenticonus
2. Index myopia. This is more common in adults beyond fourth decade as nuclear
sclerosis. However any condition that increases refractive index of lens in children
can also result in myopia i.e. diabetes, concussion injury.
Similarly stromal edema of the cornea can also produce some degree of myopia.
3. Positional myopia. This infrequent condition occurs due to forward displacement
of lens which by itself is rare.
4. Pseudophakic myopia. This is common following IOL implant in congenital cata-
ract where the power of the IOL may be too strong for the eye.
5. Pseudomyopia. This is common in hypermetropic children who accommodate too
much and make the eye myopic. Pseudomyopia may occur due to spasm of accommo-
dation that may follow blunt injury to the globe, iridocyclitis or due to instillation of
miotics.
6. Myopia of prematurity
23
. This type of myopia is commonly seen in children with
birth weight of 1250 gm or less (a birth weight that predisposed retinopathy of pre-
maturity). There is a fluctuation of myopia that may range between 10D to 20D. The
myopia is reduced to 2 to 6D by 6 months and is altogether disappears by 1 year. The
cause of the condition is not known.
7. Drug induced myopia. Some of the drugs taken orally can cause transient low
degree of myopia. Commonest example is acetazolamide induced myopia.
8. Space myopia
9. Night myopia
The last two are not seen in children.
Hypermetropia (Hyperopia)
Hypermetropia is commonest form of error of refraction seen in children under five
years.
3
Most of them are symptom less. The condition draws attention less frequently than
myopia where diminished vision is poor enough to draw attention of the child or relatives.
Hypermetropia is also called farsightedness, which is irrelevant
8
as the children may
sometimes have diminished distant vision as well, in contrast to myopia where the children
always have poor distant vision but good near vision.
Hypermetropia is a state of refraction where parallel rays are brought to focus behind
the photo sensitive layer of the retina and the image formed on the retina is blurred circle
when accommodation is at rest.
3
At birth almost all eyes are hypermetropic by 2 to 3 diopters which gradually gets neu-
tralised due to growth of the eyeball and theoretically the eye should be emmetrope at pu-
berty, but in practice only half of the eyes reach a state of emmetropia. About 90 percent of
these children who do not reach emmetropia remain hypermetropic at five years of age. This
percentage is reduced to 50 percent by sixteen years.
Hypermetropia is considered to be a state of underdeveloped eye. The eye is shorter
than emmetropic eye. As a rule the shortening is not too much in contrast to myopia where the
lengthening is generally in higher order. In hypermetropia, the shortening is rarely more
than 2 mm, hence it is unusual to have more than 6D of hypermetropia in case of simple
hypermetropia.
Etiologically hypermetropia can be :
1. Axial
2. Curvature
3. Index
4. Displacement of lens
Out of all, the axial hypermetropia is the commonest form. This can be part of a
shortened length or the sclera may be pushed forward by a retrobulbar mass or the detached
retina may come forward. The later two are pathological causes of hypermetropia. Other causes
of short eyeball are microphthalmos and nanophthalmos.
Curvature hypermetropia occurs when curvature of either cornea or lens is smaller
than normal. Reduced curvature of cornea is more probable cause of curvature hypermetropia
than lens. A flattening of curvature by 1 mm results in hypermetropia of 6 diopter. Astigma-
tism is more common in curvature hypermetropia than axial hypermetropia. The causes of
flattening of cornea are : Cornea plana, micro cornea, microphthalmos, post trauma
and post surgical status. The lens is flattened in buphthalmos.
Index hypermetropia is least common cause of hypermetropia in children. This is
commonly seen in diabetic children under treatment.
Displacement of lens posteriorly causes hypermetropia that could be congenital or fol-
lowing trauma.
Absence of lens from pupillary area (aphakia) causes highest amount of hypermetropia.
Optical status of a hypermetropic eye. The parallel rays are focussed behind the
retina. The rays arising from the retina going out of the eye are divergent and seem to meet
behind the globe. As the retina is nearer the nodal point, the hypermetropic image is smaller
than emmetropic.
Accommodation in hypermetropia. Accommodation plays an important role in cor-
recting part of hypermetropia as against myopia where accommodation worsens the myopia. A
child has stronger accommodation than an adult. Hence a child is in advantageous position in
correcting hypermetropia without glasses than an adult.
According to available accommodation, hypermetropia can be divided into two main
groups i.e.
1. Latent hypermetropia and
2. Manifest hypermetropia. The sum total of the two is called total hypermetropia.
Latent hypermetropia is that part of hypermetropia that is corrected by physiological
tone of ciliary muscles. This ranges between 0.5D to 1.0D. This is measurable only following
paralysis of accommodation by cycloplegia. Latent hypermetropia is the difference between
total hypermetropia and manifest hypermetropia.
The manifest hypermetropia is divided into :
1. Faculatative hypermetropia and
2. Absolute hypermetropia.
1. Facultative hypermetropia. This is corrected by available accommodation over
and above the latent hypermetropia. Most of the children have more facultative hypermetropia
and less absolute hypermetropia. With age amount of facultative hypermetropia gets reduced
and replaced by absolute hypermetropia.
2. Absolute hypermetropia is that part of manifest hypermetropia that is not cor-
rected by accommodation.
Symptoms of hypermetropia. Symptoms of hypermetropia depends upon amount of
accommodation available.
1. No symptoms. Children with low degree of hypermetropia may have no symptoms
and hypermetropia may be unmasked only when cycloplegic is used for examination
of fundus without defective vision.
2. Asthenopia. The next stage is when moderate hypermetropia is fully corrected by
accommodation, resulting in normal or near normal distant vision. This accommoda-
tion has to be exerted throughout the day to maintain normal vision. This sustained
action of ciliary body leads to asthenopic symptoms of
1. Headache that is generally frontal.
2. Tiredness of the eye
3. Mild photophobia and watering.
The asthenopic symptoms worsen by evening or after prolong near work. These chil-
dren generally complain of headache after coming from school and do not complain on holi-
days.
3. Asthenopia with diminished vision. When the hypermetropia is not corrected by
accommodation, the child complains of diminished vision, first in the form of diffi-
culty in near work and then diminished distant work. The latter is less frequent in
healthy children.
4. Defective vision. If hypermetropia is high between 4D to 6D, the patient generally
gives up accommodation and absolute hypermetropia is precipitated and patient com-
plains of diminished near vision in pre-presbyopic age and diminished distant vi-
sion.
5. Pseudo myopia. A child may over accommodate to achieve good distant vision. In
this effort the child may overshoot the emmetropia and become myopic, which again
causes diminished distant vision that may be wrongly corrected subjectively by mi-
nus glasses worsening the condition. Thus all children should get their glasses only
after refraction under cycloplegia.
6. Squint sensation. Some children may complain of squinting without diplopia, in
absence of obvious squint, however, there may be esophoria.
7. Squint. A child presents with esotropia and is found to have high hypermetropia.
8. Recurrent blepharitis, stye and chalazion.
9. Some children may hold the book too close to the face and may be mistaken as my-
opic. The phenomenon of keeping books near the eyes give larger retinal image that
compensates for poor vision.
10. The children may have other ocular deformity like microphthalmos, microcornea or
nanophthalmos.
Signs :
1. Similar to low grade of myopia, an eye with low hypermetropia looks as normal as
emmetropic eye. An eye with moderate to high hypermetropia is typically a small eye
not only in anterio posterior diameter but in all directions.
3
The cornea is smaller than emmetropia and the anterior chamber is shallower. The
pupil is also small. In case of pathological hypermetropia coloboma of uvea is not an
uncommon finding.
2. The face of the child on close inspection may not be symmetrical. Hypermetropia is
generally more on the asymmetrical side and is associated with astigmatism.
3. Pseudo divergent squint. This is due to large positive angle alpha. (kappa)
4. Esotropia. This is due to high hypermetropia i.e. between +4D to +7D. And mostly is
an accommodative esotropia.
5. Fundus. Overall size of the fundus is smaller than the emmetropic eye with small
disc, which resembles optic neuritis without visual symptoms. This is called pseudo
neuritis.
Generally fundus background has a peculiar sheen that is called shot silk appear-
ance.
The blood vessels reflect more light than emmetropia.
The macula is generally situated away from the disc.
Management of hypermetropia. Management of hypermetropia like myopia is opti-
cal or surgical. Surgical treatment is not indicated in children. Optical treatment con-
sists of prescription of spectacles or contact lens, which depend on available accommoda-
tion. There is no rule of thumb according to which power of the lenses can be determined. A
rough method is -
1. Children below six years. Some accommodation for distance is physiological at
this age and the child may not be aware of presence of hypermetropia. These children are
asymptomatic and need no treatment. If there is a family history of hypermetropia, these
children should be refracted under cycloplegia.
Treatment is called for if there is evidence of asthenopia or muscle imbalance.
2. In school going children. Who requires prolonged near work, require correction
even of smaller degree.
3. Children with accommodative squint and diminished vision should be corrected. If
the error of refraction is more than +3D, the child should be given glasses for constant use.
Glasses less than +3D may be worn for near work only.
In children under six years, the power of glasses should be +1.5D to +2.0D less than the
objective finding.
In children above six years, full correction with good vision should be prescribed.
As the child grows, some of axial hypermetropia gets reduced and the refraction may
swing towards emmetropia. In rarer instances the accommodation may worsen with corre-
sponding increase of hypermetropia in children in second decade. Keeping in mind the above
factors, all hypermetropic children should get yearly ophthalmic check and undergo
corresponding adjustment.
Contact lenses in hypermetropia. In principle contact lenses can correct any type of
hypermetropia. It is better to prescribe contact lenses in hypermetropic children after
hypermetropia has stabilised. Other indications are anisometropia and uniocular
hypermetropia.
Surgical management of hypermetropia. Refractive surgery for hypermetropia are
not as rewarding as in myopia. Following surgical procedures are available.
1. Hexagonal keratotomy
2. Photo refractive keratectomy
3. Lasik
4. Laser thermal keratoplasty
5. Phakic intraocular lens
Like surgical treatment of myopia, the above procedures are not indicated in children.
Other types of hypermetropia seen in children are :
1. Aphakia
2. Pseudophakia
3. Over corrected myopia.
Aphakia :
Aphakia in children is less common than seen in adults.
Aphakia literally means absence of lens from the eye. From clinical point of view
aphakia means absence of lens from pupil, complete or partial.
Aphakia is an example of extreme form of hypermetropia where there is no accom-
modation. Hence aphakia is a state of absolute hypermetropia which equals total
hypermetropia. Any amount of cycloplegic does not increase hypermetropia in aphakia.
Causes of aphakia in children are :
1. Congenital. This is one of the rare congenital anomalies, where either the lens
vescicle has not formed (primary aphakia) or the partially formed lens has been
absorbed in intrauterine life (secondary aphakia)
17
.
2. Trauma :
(i) Surgical. This follows surgery on congenital, developmental or traumatic cata-
ract in children.
(ii) Accidental trauma. Spontaneous absorption of cortical matter following injury
to the lens.
(iii) Traumatic extrusion of the lens from the globe, generally the lens becomes
subconjunctival and forms a phacocele.
3. Displacement. This can either be congenital or may be traumatic. Sometimes the
lens may be displaced in congenital glaucoma or aniridia.
Posterior dislocation is more common than anterior dislocation.
An aphakia is said to be complete when whole of lens i.e. capsule, cortex nucleus are
absent from the pupillary area. It is said to be partial when a part of the pupil is devoid of the
lens.
Optics of aphakia. Though aphakia is a high degree of hypermetropia, its optic differs
greatly from phakic hypermetropia.
The first and foremost component of aphakia is absence of lens from optical system
and equally important part is total absence of accommodation.
1. The total refractive power of the eye is reduced to +43+44D from +60D of normal
emmetropic eye with only one refractive element i.e. cornea.
2. The eye becomes highly hypermetropic without accommodation.
3. The anterior focal point shifts to 23.2 mm from usual 17.05 mm in front of the cornea.
4. The posterior focal point shifts to 31 mm, which is 7 mm more than the normal anterio
posterior length of a normal eyeball.
5. The two principal points are almost on the anterior surface of the cornea.
6. The two nodal points almost merge with each other and are 7.75 mm behind the
anterior surface of the cornea. Thus the nodal point moves forward in absence of lens.
The image is formed away from the nodal point, hence it is smaller than in emmetropic
eye. The image gets magnified when aphakia is corrected by plus lenses, may be in spectacle or
as contact lens. Magnification by spectacle is about 30% to 40% more than that of emmetropic
eye. The corresponding magnification range between 5% to 12% in contact lens. If an IOL is
put in the posterior chamber, the magnification is abolished altogether.
Symptoms of aphakia. Symptoms of aphakia consists of diminished distant vision
and near vision. Uncorrected distant vision in aphakia is generally 1/60-2/60. The child can
not read the usual prints in books due to total absence of accommodation.
Signs of aphakia :
1. Scar :
(a) At the limbus if a small incision lens extraction has been done.
(b) A small scar inside the limbus was a common site when needling was a popular
mode of lens extraction in children.
(c) A small opacity in the cornea away from the limbus is seen in case of penetrating
injury by pointed objects like needle, thorn, pin etc.
(d) An extensive corneoscleral scar is seen in corneoscleral penetrating injury.
(e) Phaco emulsification done in older children may have barely visible scar away
from the limbus.
(f ) Scar is absent in blunt injury, buphthalmos, Marfans syndrome.
2. Anterior chamber is deeper than normal.
3. The iris is tremulous.
4. An iridectomy may be present.
5. Pupil
(a) colour :
(i) Jet black in case of posterior dislocation of lens due to any cause.
(ii) After cataract is common following needling, needling and aspiration, and spon-
taneous absorption of cortical matter following penetrating injury. In children
posterior capsular opacification is almost universal even following micro-surgi-
cal lens extraction.
(b) Position. A shifted pupil denotes incarceration of iris in the section. It is more
common in accidental aphakia than in surgical aphakia.
5. Refraction. Refraction in adults shows compound hypermetropic astigmatism
against the rule following conventional intracapsular or extra capsular cataract ex-
traction. In children because the scar is very small, the refraction is generally con-
fined to simple hypermetropia.
6. Loss of accommodation. There is total loss of accommodation.
7. Fundus examination shows small hypermetropic disc.
8. In all cases of aphakia following trauma or cases where mechanical capsulotomy has
been done, the fundus should be examined by indirect ophthalmoscope for evidence
of holes or traction bands.
9. Muscle imbalance and amblyopia are common in uniocular aphakia.
Management. Management of aphakia in children, in principle does not differ from
adult aphakia that is to correct hypermetropia by convex lenses. Cylindrical errors should also
be taken into consideration. Additional plus lenses are required for near vision.
The various options available are :
1. Spectacle
2. Contact lens
3. Intraocular lens
4. Refractive surgery.
Spectacles are suitable in bilateral aphakia in children. They are not tolerated in
uniocular aphakia due to anisometropia and aniseikonia that leads to intractable diplopia and
child prefers single vision without aphakic correction. This in turn leads to development of
squint and amblyopia. An aphakic child may tolerate spectacle correction if the other eye has
no vision, poor vision, has deep suppression or is divergent.
Even in case of binocular aphakic correction with spectacles, the child has :
1. Spherical aberration leading to pin cushion distortion. The straight lines look curved,
the whole scenario consist of paraboles which keep on changing with movement of
the eyes.
2. Prismatic aberration causing a roving ring scotoma between 5065 all round.
3. Overall restriction of peripheral field.
4. Absent or poor near vision. A child is less likely to tolerate bifocal so he requires
thicker near glasses.
5. Cosmetic blemish. A child especially in teens does not like using thick heavy glasses
that give an enlarged view of the eye.
6. Sliding of the spectacles on the nose. This increases the effective power of the
plus lens which may help the child in near work but blurs distant vision and in-
creases cosmetic blemish.
Few of the advantages of spectacle correction are that they are cheap, can be replaced
with ease, do not require specialised handling.
Contact lens. Contact lens correction is better option than spectacle correction espe-
cially in uniocular aphakia because :
1. Magnification by contact lens is only 5% to 10% in contrast to 30% by spectacle cor-
rection.
2. The field of vision is better, the roving scotoma is abolished.
3. Prismatic aberration is almost abolished.
4. The cosmetic blemish is eliminated.
It has been seen that small children can also tolerate contact lenses well.
Intraocular lenses are the best possible answer to aphakic correction as they are de-
void of all the aberrations met with spectacle and contact lens correction. Most advantageous
property being complete elimination of magnification, which by itself abolishes aniseikonia
when placed in posterior chamber. Intraocular lenses are generally used as primary procedure
for cataract in children but can be used as secondary procedure where the eye was left aphakic
following lens extraction.
Refractive surgeries are not suitable aphakic children.
Pseudophakia. Pseudophakia is the best method of correcting aphakia. In this the
lens is removed partially or totally and supplemented by an artificial lens.
The intraocular implants are made up of polymethacrylate (PAMA), silicon, acrylic
or hydrogel. The first is a rigid, hard lens while the others are malleable.
The IOL can be implanted as a primary procedure i.e. removal of lens with simultane-
ous implantation of lens or as a secondary procedure where the lens is removed in first session
and IOL implantation later.
According to position of lens, the implant can be in the :
1. Anterior chamber
2. Iris fixed
3. Posterior chamberIn the bag
In the sulcus
4. Scleral fixed.
Anterior chamber lenses have been given up as they are prone for immediate and
delayed complication and are implanted following intracapsular lens extraction mostly, which
is not possible in children.
Iris fixated lens can either be fixed in the pupil or on the surface of iris.
Posterior chamber IOL in the bag is the most popular and suitable lenses in children.
IOL in sulcus has also been abandoned in favour of IOL in bag.
Scleral fixed lens require high degree of skill and are used in subluxated and dislo-
cated lenses.
The IOLs mostly are unifocal that require additional near correction unless the child
is left slightly myopic. In contrast to this, there are multifocals that have power to focus
distant as well as near objects. Role of multifocals in pediatric aphakia has not been
evaluated well.
If the power of IOL has been calculated accurately and lens placed well, the IOL should
make the eye emmetropic but this is not always possible. Some residual error is almost
universal. The post operative corneal astigmatism is not corrected by best calculated IOL.
The IOL reduces aniseikonia to well tolerated limit. Thus reducing incidence of diplo-
pia. However most of the IOLs leave some loss of stereopsis.
Some of the children may require spectacle correction even after IOL mostly to neutral-
ise astigmatism.
Diminished Near Vision in Children
Diminished near vision is universal in emmetropic adult after forty years of age. Chil-
dren due to their reserve of accommodation do not have difficulty in finest near work.
The condition where the child has difficulty in near vision are :
1. TherapeuticAccidental or therapeutic use of strong cycloplegic.
2. Neurological
(a) Internal ophthalmoplegia
(b) Third nerve palsy
3. Refractive
(a) Uncorrected or under corrected hypermetropia
(b) Over corrected myopia
(c) High astigmatism
(d) Aphakia
(e) Pseudophakia
4. Disparity between accommodation and conversions.
5. Nystagmus
Astigmatism
This is a state of refraction where a point focus of light is not formed on the retina with
or without accommodation. Instead of one focal point there are two focal lines. The distance
between the two focal lines is known as focal interval that represents the astigmatic power
of the eye. The aim of treatment is to abolish this distance by optical methods i.e. spectacle,
contact lens or surgery so that the two focal lines merge with each other, and a pinpoint focus
is formed on the retina. In between the two focal lines is the circle of least diffusion which
represents the spherical power.
A perfect stigmatic eye is an exception. All eyes have some minor degree of astigmatism
without any symptoms. The cornea of a new born is almost spherical
18
, with age slight astig-
matism is natural due to pressure of the lids on the cornea. The vertical meridian is steeper
hence slightly more myopic than flatter horizontal meridian, which remains emmetropic. This
is known as direct astigmatism or astigmatism with the rule. In contrast to this, where
horizontal curvature steeper, the condition is called indirect astigmatism or astigmatism
against the rule.
Types of astigmatism :
1. According to site of involvement Corneal
Lenticular
Retinal
2. According to axis of astigmatism
3. According to position of the image.
4. Clinical classification Regular astigmatism
Irregular astigmatism
According to site : Corneal astigmatism is the commonest form of astigmatism both
regular and irregular.
Lenticular astigmatism :
1. Decentered lens
2. Different curvature in different meridian i.e. lenticonus, subluxation, coloboma of
lens.
3. Difference in refractive index i.e. early cataract.
Retinal astigmatism. This is seen generally with retrobulbar mass or oblique place-
ment of macula.
19
According to axis of image :
Regular astigmatism
1. The two meridians are at right angles to each other, one of them is horizontal and
the other is vertical. This is the commonest type of astigmatism.
2. The two axises are at right angles to each other but not horizontal and vertical, the
condition is called oblique astigmatism. The axises are off the horizontal or verti-
cal by 20 or more. The axis in one eye is mirror image of the other eye i.e 80 and
110 or 20 and 160.
3. The difference between the two meridians is less than 90, the condition is called bi-
oblique astigmatism.
Irregular astigmatism is that astigmatism where astigmatism in the principal me-
ridian vary. This is caused due to corneal irregularity like corneal scar, keratectasia,
keratoconus, pterygium, limbal dermoid, post keratoplasty.
According to position of image in relation to retina. The astigmatism can be sim-
ple, compound or mixed.
Simple astigmatism is that astigmatism in which one meridian is emmetropic, the
other is either myopic or hypermetropic. The former is called simple myopic astigmatism,
while the latter is called simple hypermetropic astigmatism. Each is corrected by suitable
single cylinder of appropriate sign, axis and power.
Compound astigmatism is that astigmatism where both the meridians are either
myopic or hypermetropic and known as compound myopic or compound hypermetropic
astigmatism respectively. They are corrected by sphere and cylinder of same sign i.e. com-
pound myopic astigmatism is corrected by myopic sphere with myopic cylinder.
Mixed astigmatism is that astigmatism where two meridians have different signs i.e.
one is myopic, the other is hypermetropic or vice-versa. In mixed astigmatism the power of
the cylinder is always more than the sphere. They are most difficult to correct. They are
very common following IOL implant.
Symptoms of astigmatism :
1. Small degree of astigmatism may be symptomless.
2. Diminished distant visionAs the eye fails to form a sharp image on the retina, the
visual loss is considerable. However sometimes high astigmatism may not cause cor-
responding degree of loss of vision i.e. a child with 6/12 vision may require just 0.75 to
1.00 Dsp to bring the vision to 6/6. But an astigmatic may require as much as 2 to 2.5
Dcyl to attain same vision.
3. Difficulty in focusing near objectsAs accommodation fails to correct astigmatism,
the near vision is never comfortable for an astigmatic.
4. Head turning and tiltingThe child may turn or tilt the head to compensate for the
axis of astigmatism.
5. Narrowing of interpalpebral fissureThe child tries to produce a pinhole effect to
improve the vision. This attempt is less effective than seen in spherical ametropia.
6. Muscle imbalance is more common in astigmatism than in other errors of refraction.
7. Asthenopia and asthenopia related symptoms are more common in astigmatism
of low grade. They are : Watering, redness of eye, recurrent blepharitis, stye, chalazion,
frontal headache.
8. Muscle imbalance.
Signs of astigmatism :
1. Externally the eyes may not look abnormal. In case of compound high myopic astig-
matism the eye may look large, may similarly look small in case of compound
hypermetropic astigmatism. Recurrent stye, chalazion, blepharitis, presence of
haemangioma or neurofibroma on the lid should arouse suspicion of astigmatism.
2. Placido disc may show irregularity in the circles, in irregular astigmatism and crowd-
ing and elliptical shape in high regular astigmatism.
3. Computerised keratoscopy shows irregularity in the corneal surface.
4. Refraction always shows difference in refraction in two principal meridians.
5. Keratometry shows difference in power in different meridian and their axises.
6. On fundus examination the optic disc looks oval. Vertically oval disc is more common
than horizontal.
7. An astigmatic child when examined on E chart may not be able to tell the direction of
the arms of E. He may be able to tell the direction in vertical direction but fumble in
horizontal direction and vice versa.
8. On astigmatic fan the child may see some lines clearly and not those at right angles
to it.
9. Vision with pinhole does not improve satisfactorily in astigmatism. A child with -2D
myope will improve to 6/6 with a pinhole but a child with -1Dcyl may improve only to
6/12.
Management
1. No treatmentIf there is no visual loss or there are no symptoms of eye strain,
asthenopia or muscle imbalance, no treatment is required. The child is examined
once every year and his retinoscopy is done under cycloplegia.
2. Prescription of glasses :
1. Simple astigmatism is corrected by single cylinder of appropriate power and sign
at proper axis.
2. Compound astigmatism is corrected by prescribing sphere and cylinder of same
sign.
3. Mixed astigmatism is corrected by sphere and cylinder of opposite sign.
As a rule every attempt should be made to correct cylindrical defect fully.
18
The spherical correction should be done on usual lines of spherical ametropia.
While correcting astigmatism utmost attention should be given to correct axis of the
cylinder. A wrongly placed cylinder is more troublesome than without one.
In mixed astigmatism the combination that is most comfortable should be prescribed
i.e. minus sphere with plus cylinder at right angles or plus cylinder with minus cylinder at
horizontal axis.
The power and axis of the cylinder can be checked by :
1. Auto refractionThese are good for non-verbal children who can not be tested sub-
jectively. However refraction under cycloplegia should get proper weightage.
2. KeratometryThis is more important in contact lens fitting than spectacle. This
measure the curvature of a anterior corneal surface only 3 mm wide. It measures the
astigmatism of the front surface of the cornea at two points about 1.25 mm on either
centre side of the cornea.
3. Foggin
4. Astigmatic fan
The last two are not suitable for small children.
5. Jacksons cross cylinder. This is the best and most accurate method to verify power
of cylinder and axis of cylinder.
The spectacles given for astigmatism should have proper fitting otherwise the axis may
change giving poor vision and discomfort.
Contact lens. Contact lenses are prescribed only when the child can manage the con-
tact lens himself. Though small degree of regular astigmatism are well corrected by contact
lens, it is better to go for spectacle correction because it is cheap, easily replaceable, and re-
quire no maintenance. Contact lenses are more suitable for high astigmatism, bi-oblique astig-
matism and irregular astigmatism.
Spherical contact lenses fail to correct astigmatism more than 2 to 3 Dcyl. For high
cylindrical error toric contact lenses are best suited.
A toric lens
20
should have full corneal coverage and good centering. The lens should
have good movement with each blink, the up and down gaze. The lens should give vision
comparable to spectacle vision if not better. The toric contact lenses are difficult to fit.
21, 22
Anisometropia
When the refractive power of two eyes is different, it is called anisometropia in con-
trast to isometropia where refraction in two eyes is equal. Small degree of anisometropia is
more or less the rule. Perfect isometropia is exception like perfect emmetropia and stigmatic
eye. Anisometropia is more common in astigmatism, may be congenital. A difference in refrac-
tion in two eyes results in difference in retinal size in two eyes. One diopter of difference
results in 2% difference in size of image. In myopia the size is reduced while in
hypermetropia the size is magnified. A difference in 2.5D between two eyes will result in 5%
difference in size of image. Difference in size of two retinal images is called aniseikonia. A
difference in size up to 5% between the two eyes is the limit of tolerance beyond which diplo-
pia results.
A difference of 2.5D or more is called clinical anisometropia. Anisometropia upto
2.5D is well tolerated, of course some may not tolerate even a smaller difference. However,
some individuals may tolerate difference upto 4D.
Etiology of anisometropia. Anisometropia can be congenital or acquired. Congenital
anisometropia is generally hereditary and due to differential growth of two eyes.
Acquired anisometropia. Commonest cause of anisometropia is uniocular aphakia.
Second cause that is being seen more often is wrong calculation of IOL power. Other such
cause is over or under correction following refractive surgery and uniocular keratoplasty.
Types of anisometropia :
1. Simple Spherical
Astigmatic
2. Compound Spherical
Astigmatic
3. Mixed Spherical
Astigmatic
Simple anisometropia. In this condition one eye is emmetropic, the other eye is either
myopic or hypermetropic.
Compound anisometropia. Both eyes have same type of error of refraction i.e. myo-
pia or hypermetropia but power in one eye is more than the other.
Mixed anisometropia or Anti metropia. In this, one eye is myopic, the other eye is
hypermetropic.
In simple astigmatic anisometropia one eye is emmetropic, the other has simple
astigmatism.
In compound astigmatic anisometropia both the eyes are astigmatic but at differ-
ent degree.
In mixed astigmatic anisometropia one eye has myopic astigmatism, the other eye
has hypermetropic astigmatism.
Symptoms of anisometropia. Symptoms of anisometropia depends upon difference
between the refractive status in two eyes.
Visual :
1. Binocular single vision. This is present when the difference between the two eyes
is not more than 2.5D to 3.0D.
2. Uniocular vision. This happens when the difference in power in two eyes exceeds
4D. Generally one of the eye is emmetropic or has less power than the fellow eye. The
eye with higher power is generally suppressed, develops and becomes amblyopic and
starts squinting.
3. Alternate vision. This happens when one eye is either emmetropic or has low error
of refraction and the other eye has myopia more than 2D. The patient uses the my-
opic eye for near vision and the other eye for distant vision. These patients may not
know about their visual short coming and are comfortable. They do not seek optical
aid. Such children are diagnosed on routine school survey or if they wish to join some
project that requires binocular vision.
Muscle imbalance. The child may be brought with squint and discovered to have
anisometropia and many a times amblyopia. Anisometropia may cause both eso or exodeviation
but concomitant convergent squint is more common.
Diagnosis. Diagnosis of anisometropia is simple by doing retinoscopy which should be
done under complete cycloplegia. Otherwise, it can be done by Friend test or Worth four dot
test.
The Friend test. This is a simple test. It consists of a panel generally incorporated at
the bottom of usual Snellens drum. In the chart the letter FIN are written in green and RED
are written in red. The size of all the letters are same. The background is illuminated. The size
of the letters correspond to size of 6/18 or 6/12. The child sits at a distance of six metres with
red green glasses in the trial set or may wear a diplopi goggles. The red glass is kept in front of
right eye as convention and not for any clinical significance. The child is asked to read the line
keeping both the eyes open. The right eye can see only red letters through the red glass and
the left eye only green letters through the green glass if the patient has binocular single vision
the child will be able to read the full line as FRIEND. If the child has alternate vision, the child
will read either FIN or RED at a given time. The child with uniocular vision will read either
FIN or RED depending on which eye has better vision.
Worth four dot test. This panel is also incorporated at the bottom of usual Snellens
vision drum. It has four dots of the size of 6/18 arranged in a diamond shape. The spots have
following colours - one white, one red and two greens.
The child sits at a distance of six metres with red glass in front of the right eye and
green glass in front of the left eye and asked to count the number of spots and tell their number
with both eyes open. Before starting the test, it is seen if the child has manifest squint or not.
Interpretation :
1. In arthophoria, if the child sees all the four dots and tells their number correctly that
matches with the panel, the child has normal binocular single vision.
2. If the child has manifest squint and the child sees all the four dots as in the panel, the
child has abnormal retinal correspondence.
3. If the child sees only two red lights, the left eye is suppressed.
4. If the child sees five lights i.e. two red and three greens, the child has diplopia which
otherwise means fairly good vision in each eye.
Management :
1. Spectacles. Spectacles are tolerated well if the difference of power in two eyes is not
more than 2.5D. Some patients may tolerate difference up to 4D. Usually the eye
with more power is under corrected. In children care should be taken to see that the
child sees through the centre of the glasses and not above the glasses and uses glasses
constantly.
2. Contact lenses. The contact lenses are better option than spectacles because the
change in size of the image is less. The child has no chance to look above the lens and
the lens moves with the movement of the eye.
3. Intraocular lens implant. This is most suitable form of treatment in uniocular
aphakia. All uniocular lens extraction in children should have IOL implanted as pri-
mary procedure if not contra indicated. In cases of old uniocular aphakia, secondary
IOL is a safe procedure.
4. Clear lens extraction may be tried in case of uniocular high myopia. High myopes
may also have phakic IOL in presence of clear lens.
5. Refractive surgeries are not indicated in children.
Aniseikonia
An aniseikonia is a rare disturbance of binocular vision. Prevalence of the condition is
more than the numbers diagnosed. It is defined as a condition where images presented to
cortex from two retinae are of unequal shape or size or both.
Causes of aniseikenia are
1. Optical. This is the comonest and most easily diagnosed type of aniseikonia where
due to high anisometropia, images of two different sizes are formed on the retina and
presented as such to the cortex. This can be congenital or acquired. The acquired
cause consist of difference in power, thickness of the lens and cylinders in the lens of
the spectacle prescribed.
2. Retinal
1. Shift of the retinal element towards the nodal point.
2. Separation of retinal element due to edema of the retina.
Clinical types of aniseikonia
1. Aniseikonia is said to be symmetrical when there is difference in size of the two
images. The difference can be overall i.e. in all meridians or meridinal when the
difference is greater in one meridian and compound when both types i.e. overall and
meridinal are present.
2. Asymmetrical when there is difference in shape of the two images. It could be regu-
lar, pin cushion, barrel type or irregular. In regular type, there is progressive in-
crease or decrease in size across the field.
Symptoms of aniseikonia vary from patient to patient. Some of the patients may not
be able to express the correct symptom except diplopia. Other symptoms are asthenopia. In
some patients there may be faulty stereopsis. There may be disturbances of spatial orientation
in the form of objects on right side of the field looking farther away than objects of same size
and distance on the left side. A flat surface slants down on the right side and up on the left. The
square appears rectangular and circles seem to be elliptical.
Diagnosis. Diagnosis of aniseikonia is difficult, however, a rough estimate can be made
on the basis of anisometropia present, 1% change in size per dioptre is most accepted estimate.
Treatment. Optical causes of aniseikonia are best treated by contact lens. Best treat-
ment for unilateral aphakia is IOL implant either as primary procedure or secondary proce-
dure.
Treatment of retinal aniseikonia is directed towards treatment of retinal condition mostly
medically.
Asthenopia
Asthenopia is one of common symptoms, which the patient may not be able to express
precisely. It is defined as uncomfortable vision that may cause unexplained pain and irrita-
tion in the eyes, fatigue, after near-work, heaviness of lids, sleepy feeling.
Causes of asthenopia include
1. Refractive error.
2. Muscle imbalance
3. Accommodation and convergence insufficiency.
Errors of refraction. Uncorrected errors of refraction are commonest cause of
asthenopia. High errors of refraction rarely cause asthenopia because the child is resigned to
blurred vision and does not try to get a clear vision. Smaller errors of refraction cause more
asthenopia. Astigmatic eyes are more likely to develop asthenopia than spherical errors. Hori-
zontal axis and oblique axises cause more trouble than other meridians. Under corrected or
over corrected errors are more likely to cause asthenopia. In myopia asthenopia develops more
for near work because myope requires more convergence for clear view without or with least
accommodation. The child has to depend on increased fusional convergence. Some myopic
patients who had no asthenopia before correction may develop asthenopia following correc-
tion.
A hypermetropic has to use accommodation more than emmetrope for near as well as
distance, putting constant strain on ciliary muscle. Hypermetropic astigmatism causes more
problem because hypermetropic tries to get clear vision by accommodating, as accommodation
is equal in all meridians the astigmatism is worsened in opposite meridian.
In anisometropia, the blurring of vision is unequal in two eyes and accommodation which
acts equally in both eyes may worsen in one of the eyes.
Muscle imbalance. Asthenopia is more common in phorias than in tropias. The child
constantly tries to keep the eye in orthophoric state and a discrepancy between amplitude of
motor fusion and deviation results in asthenopia. Vertical deviations cause more asthenopia.
Accommodation convergence insufficiency. It can either be deficiency of accom-
modation or convergence.
Some patients with good vision with low degree of errors of refraction may be unable to
accommodate without convergence.
Convergence insufficiency is seen in uncorrected myopes or hypermetropes corrected
for first time. Otherwise they are orthophoric for distance.
Symptoms. The symptoms of asthenopia may be ocular or non ocular.
Ocular symptoms can be visual or non visual.
The visual symptoms are intermittent. They are seen following prolonged non accus-
tomed visual effort or general dibility. There is slight fall in vision.
Non visual symptoms are tired eyes, aching eyes and bleary eyes. The ocular pain is
described variously as deep seated, boring or dull. Chronic conjunctivitis, blepharitis, styes,
chalazia are common. Commonest non ocular symptom is headache. It is very common for
general physicians to refer all cases of headache to ophthalmologist as asthenopia. On exami-
nation most of them may turn out to be non asthenopic. Headache in asthenopia does not
follow any fixed pattern. It may be referred as browache, frontal, temporal or occipital in
location. Most probable explanation for headache in asthenopia is pain referred from the cili-
ary body.
Management of asthenopia. Management of asthenopia is frustrating. It is directed
to cause of asthenopia.
The examination that should precede management are :
1. Recording of distant and near vision.
2. Checking near point of accommodation.
3. Checking near point of convergence.
4. Assessment of muscle imbalance.
5. Refraction under cycloplegia.
Refraction should be followed by prescription of glasses that are most comfortable even
with slightly reduced vision. Axis of astigmatism should be checked meticulously.
Orthoptic treatment or orthoptic exercise in the form of - adduction exercise are given
for convergence insufficiency.
PrismsBase in prisms also give good result in convergence insufficiency.
REFERENCE8
1. Duke Elder S. ; Ophthalmic optics and refraction in System of ophthalmology. Vol. V,
C.V. Mosby, St. Louis, 1970.
2. Duke Elder S ; The foundation in ophthalmology. System of ophthalmology. VII,
3. Primrose J. ; Anomalies of refraction and accommodation in Modern Ophthalmology.
Vol. III, Edited by Sorsby A. Butterworth, London, 1964.
4. Agrawal L.P. ; Principles of optics and refraction. Second edition. CBS Publishers
and distributors, New Delhi, 1979.
5. Singhal N.C. ; Principles and practice of refraction and optics. JayPee Brothers,
New Delhi, 1996.
6. Khurana A.K. ; Theories and practice of optics and refraction. B.I. Churchill,
Livingstone, New Delhi, 2001.
7. Sharma P. ; Myopia in Essentials of ophthalmology. First edition p-2124, Modern
Publishers, New Delhi, 2000.
8. Debhorah Pavan Langston ; Refractive errors and clinical optics in Manual of ocular
diagnosis and therapy. Third edition, p-361-388, Lippincot William and Wilkins,
Philadelphia, 2002.
9. Khurana A.K. ; Congenital myopia in Opthalmology. Second edition, p-56, New Age
International, New Delhi, 2000.
10. Agrawal L.P. ; Myopia in Essentials of Ophthalmology. First edition, p-469473,
CBS Publication, New Delhi, 2000.
11. Basak S.K. ; Myopia in Essentials of ophthalmology. Second edition, p-5660, Cur-
rent Book International, Calcutta, 1999.
12. D.S.T. Clair Roberts ; Fundus changes in myopia in Modern ophthalmology. Vol. 4,
p-761762, Edited by Sorsby A. Butterworth, London, 1964.
13. Miller S.J.H. ; Myopic choroido retinal degeneration in Parsons diseases of the eye.
Seventeenth edition, p-165166, Churchill Livingstone, London, 1984.
14. Kanski J.J. ; Myopic maculopathy in Clinical ophthalmology. Second edition, p-360,
15. George E. Garcia and Deborah Pavan Langston ; Orthokeratology in Manual of ocular
diagnosis and therapy. Third edition, edited by Deborah Pavan Langston, p-387,
Lippincot, William and Wilkins, Philadelphia, 2002.
16. Boyd S. Lasek ; Laser subepithelial keratomileusis. Highlights of ophthalmology. Vol. 30,
p-1517, 2002.
17. Duke Elder S. ; Congenital aphakia in System of ophthalmology. Vol. III, Part I,
18. Duke Elder S. ; Astigmatism in Duke Elders Practice of refraction. Ninth edition,
p-5255, BI Chirchill Livingston, New Delhi, 1989.
19. Khurana A.K. ; Astigmatism in Theory and practice of optics and refraction. First
edition, p-70, Bi Churchill Livingston, New Delhi, 2001.
20. Phyllis L. Rakow ; Soft lenses and astigmatic eye in Contact lenses. First edition,
21. C.Y. Khoo ; 101 Questions and answers about contact lenses. P.G. Publication,
New Delhi, 1985.
22. Hunter D.G. and West. C.E. ; Contact lenses in Last minute optics. First Indian edi-
tion, p-72, Jay Pee Brothers, New Delhi, 1997.
23. Schaffer D.B. ; Prematurity as related to ocular abnormalities in Text Book of Oph-
thalmology. 9th edition, edited by Scheie H.G. and Albert D.M., p-323, W.B. Sanuders
CHAPTER 16
8ymptomatic Disturbance of
Vision in Children
1, 2, 3
Commonest visual symptom in all ages is diminished vision either for distance, near or
both. Causes of which can be traced mostly to the eye itself and considered to be peripheral.
Besides these there are a host of complaints which may or may not have diminished
vision, have systemic origin, mostly in neuro-visual paths.
Many of them are suffixed with term blindness i.e. night blindness, colour blindness.
These terms do not strictly meet the criteria laid down in definition of blindness. They are
used clinically more as habit than academic accuracy.
They are :
1. Night blindness (nyctalopia) 2. Day blindness
3. Chromatopsia 4. Metamorphopsia
5. Colour blindness 6. Visual aphasia
4
7. Dyslexia 8. Agnosia
9. Visual hallucination 10. Scotomas
11. Amaurosis 12. Amblyopia
Out of all the conditions in the long list of disturbances mentioned above night blindness
is the most important because acquired night blindness due to vitamin A deficiency is a major
cause of preventable and curable blindness in children in developing and under developed
countries where more than half of blind children are found.
Night blindness is a state of diminished vision in reduced illumination (scotopic vi-
sion) with prolonged dark adaptation and abnormal ERG. In extreme degree the child
finds it difficult to locate his food placed in front of him after dusk or even in a darker room
during day, with no complain in bright light.
Night blindness can be :
1. Congenital 2. Developmental
3. Acquired
Congenital night blindness is far rarer than acquired night blindness. It can have
normal fundus as in congenital stationary night blindness or with fundus changes as in
Oguchis disease and fundus albipuntatus.
574
SYMPTOMATIC DISTURBANCE OF VISION IN CHILDREN 575
Congenital stationary night blindness. They have early onset of diminished vision
that may be moderate or crippling. The children have normal field when tested in normal
illumination. Their colour sense is impaired. The fundi are normal except myopic changes
due to associated moderate myopia. Myopia is common accompaniment in stationary congeni-
tal night blindness. The condition does not progress much. It is bilateral.
The condition is genetic, there are three types of genetic presentation i.e.
Recessive either X linked or autosomal and autosomal dominant.
Children with autosomal variety have normal vision while other two have diminished
distant vision due to associated myopia. Children with diminished vision may have strabis-
mus and or nystagmus.
The children have abnormal ERG and EOG.
Oguchis disease. This is a very rare congenital anomaly of rods, mostly seen in Japa-
nese. It is an autosomal recessive disorder with high incidence of consanguinity. The
main symptom is night blindness (nyctalopia) followed by diminished distant vision which
is moderate, mostly due to myopia. The condition is non progressive.
The diagnosis lies in the fundus picture. The fundus has a yellowish gray phospho-
rescent sheen
2
that spreads from disc to periphery. Occasionally only peripheral sheen is
also met with. The choroidal vessels are not visible. It is difficult to differentiate between the
retinal arteries and veins as they have almost same dark colour.
The confirmatory feature is Mizuo sign or Mizuo phenomenon that consists of re-
versal of fundus finding from yellowish gray sheen to normal fundus picture if the eyes are
kept closed for 2 to 3 hours. Then the retina returns to its yellowish gray colour after exposure
to normal light in half an hour to one hour. Colour vision is not affected. ERG shows only
scotopic a wave, b wave is extinct, EOG is normal.
Fundus albipunctatus
5
. This is a condition of congenital night blindness of
autosomal recessive type. The condition is stationary in nature. There is a progressive
type which is a tapeto retinal dystrophy and is called retinitis punctata albescens. Both the
conditions have uniform sized white or yellow white dots scattered all over the fundus. The dot
may increase in number and area but do not involve the macula. The retinal vessels are of
normal size. The disc does not undergo atrophic changes that is common in true retinal
dystrophies. The peripheral fields are within normal limits. The ERG results are vari-
able. EOG is normal. There is no known treatment.
Developmental causes of night blindness. This comprises a number of conditions of
diverse etiology and pathology with common features of night blindness, constricted field and
fundus changes in the form of pigmentation. The condition may be localised to eyes only or
may be associated with number of systemic condition.
All of them are genetically determined conditions, are most probably present at birth
but clinically manifest after few years hence they are put in category of developmental disor-
ders.
They are all abiotrophies. They can be put into two broad groups according to parts of
the eye involved :
1. A large number of conditions that are retinal in origin.
2. A smaller number of uveal dystrophies.
Though some of them are called degeneration, they are in fact dystrophies. The retinal
conditions are also known as tapetoretinal degeneration. Some of the terms like retinitis
pigmentosa may suggest an inflammatory etiology. They are non infective or inflammatory.
The retinopathies may be confined to the eye only like in primary retinitis pigmentosa
or may have systemic involvement like Laurence Moon Biedl Bardet Syndrome. The
number of systemic conditions with night blindness is a long one. See page. 433439 also.
Acquired night blindness
Acquired night blindness in children is a serious ophthalmic problem in developing coun-
tries, which if not managed may lead to permanent loss of vision.
The commonest cause is vitamin A deficiency either due to short supply in
malnutrition, high depletion in measles or poor absorption in diarrhoea. It may be seen in
many children in the same family. It is fully preventable as well as curable in early stages. It
is generally associated with xerophthalmia, which may precede onset of night blindness,
may accompany night blindness. In lesser number of children, night blindness may be the first
symptom that develops over few weeks. This is called chicken blindness because the child
fumbles in diminished light.
Other causes of acquired night blindness in children are less common.
They are high myopia with extensive chorioretinal degeneration, peripheral cataract
and juvenile glaucoma.
Day blindness. This is less common than night blindness and not serious enough to
cause blindness. Nonetheless there is a troublesome symptom that comprises of constriction of
pupil resulting in lowering of vision. The lesion is either in front of the pupil i.e. dense central
corneal opacity or behind the pupil as in central nuclear or posterior polar cataract. Large
macular lesion also causes diminished vision in bright light.
Day blindness should not be confused with glare and photophobia. Glare is intoler-
ance to bright light without loss of vision as seen in - Coloboma of iris, congenital or surgi-
cal, aniridia, albinism, myopia and following mydriasis either therapeutic or neurologic.
In photophobia the child involuntarily shuts the lid in presence of light or may turn
the head away from the light, may bury the face under the pillow. This is due to irrigation of
trigeminal nerve. It has not been conclusively proved if photophobia can be present in absence
of light perception. Common causes of photophobia in children are - Interstitial keratitis,
corneal abrasion, corneal ulcer, phlyctenular kerato conjunctivitis, fascicular ul-
cer, buphthalmos, hydrops of cornea, acute iridocyclitis, meningitis and encephali-
tis. It is also seen in congenital achromatopsia.
Photopsia. This represents seeing flashes of light. This is due to irritation of visual
elements of the retina. This is a serious symptom that is almost always a warning to
development of retinal detachment especially in myopic children and eyes that have sustained
injury. Cases of photopsia should not be dismissed casually. All children complaining of flashes
of light should undergo complete ocular examination, which should include indirect
ophthalmoscopy with scleral indentation.
Rarely cerebral irritation may also cause flashes of light.
Chromatopsia. In this condition, the persons gets a coloured hue on a light coloured
object i.e. white washed wall, white cloth etc.
This is mostly due to faulty absorption of a particular colour due to changes in the lens
or may be drug induced.
Erythropsia (Seeing red). This is common following lens extraction in first few days.
Occasionally a child with fresh thin blood film in front of the pupil or a fresh vitreous haemor-
rhage can complain of red coloured vision. It also occurs in snow blindness.
Cyanopsia (seeing blue). This is also a common complain after lens extraction.
Xanthopsia (seeing yellow). Commonest cause is jaundice. There is a big list of drugs
that cause xanthopia.
Changes in the form of objects (Metamorphopsia) :
Micropsia. When the things took smaller than their actual size. This is due to crowding
of cones. In contrast to this, minification is smaller image formation on retina by minus
glasses as is seen in myopic correction.
Macropsia. When the things look larger than its actual size due to separation of cones.
Magnification like minification is an optical phenomenon where the image of the object real
or virtual look larger than its actual size as in hypermetropic correction, aphakic correction,
use of magnifiers.
Metamorphopsia is distorted shape, this is best appreciated on Amsler grid.
Micropsia, macropsia and metamorpopsia are signs of macular lesion and retinal de-
tachment.
Colour Blindness
Human eye have a highly developed colour sense. Most of the animals and birds have
poor colour sense. A species nearer the human beings has better colour sense than those away
from human beings. None of the animals have colour sense as good as human. Human eyes can
perceive about 200 colours between 380-760 nm. The visible spectrum ranges between 397 nm
to 723 nm roughly between 400-725 nm.
5
The visible spectrum is called white light. Ultra
violet has shortest wave length while infrared has longest wave length.
Colour vision is mediated through cones and is transmitted via optic pathway like white
light. Hence defect in retina specially in macula and disorders of anterior optic pathway lead
to colour defect.
Red, green and blue are the three primary colours. Others are made up of various
combinations.
There are three types of cones responsible for red, green and blue colour sense. The
cones contain three different types of pigments one for red, the other for green and last for
blue.
Colour defect can occur due to :
1. Absence of all cones responsible for colour vision i.e. achromatopsia.
2. All the three cone pigments are present but one of them is abnormal and the condi-
tion is called anomalous trichromacy. They are divided into -
(i) ProtanomalousWhen there is abnormal red sensitive cone.
(ii) Deuteranomalous have abnormal green sensitive cone.
(iii) Tritanomalous have abnormal blue pigment.
Former two are common, the last one is rare.
Distribution of pigment sensitive cones is not uniform, the central part is devoid of
blue sensitive cones. Trichromatic vision extends up to 30 round the point of fixation.
Total absence of colour sensitive cone is suffixed with anopia that is protanopia
deuteranopia and tritanopia respectively for red, green and blue blindness.
8
3. Lesions of optic pathway also leads to acquired colour defect.
4. Changes in colour absorption by lens causes minor colour defect.
Sensitivity to colour depends on many factors either in isolation or in combination.
They areBrightness, hue this describes the name of the colour that the person sees, it
refers to dominant wave length saturation. This is also called purity of colour and represent
amount of white light present, lesser the white element purer is the colour.
Classification of colour blindness :
There are various methods to classify colour blindness :
According to age of onset :
1. Congenital 2. Acquired
According to type of cones involved
9
1. Monochromatism
(i) Rod monochromatism
(ii) Cone monochromatism
2. Dichromatism
(i) Protanopia
(ii) Deuteranopia
(iii) Tritanopia
3. Anomalous trichromatism
(i) Protanomaly
(ii) Deuteranomaly
(iii) Tritanomaly
Colour blindness is very common among boys, about 8% boys suffer from some type of
colour blindness or others. It is very rare in girls. Women are as often affected by acquired
coloured blindness as men. In congenital colour blindness women are the carriers but
do not suffer from colour blindness. This is a classic example of x linked inheritance in
red green blindness.
10
As colour blindness is a dominantly male affliction without deterioration or improve-
ment of vision in otherwise normal boys and is a bilaterally symmetrical in both eyes, all boys
during their entry in primary school should be screened for presence of colour defect that may
be mild to moderate. Total colour blindness where everything looks gray is very rare with
normal vision.
Screening is necessary not for treatment but for planning the future profession of the
boys. Persons with colour defect are not suitable for many jobs.
It is surprising that a colour blind does not find it difficult with traffic lights. This is
most probably due to brightness of the traffic light and his experience. The boy learns to call
leaves green and rose red by experience but when given to match different hues they fumble or
fail. Hence ability of a boy to name a particular colour correctly does not exclude colour defect.
To determine for colour vision there are various tests. None of them are perfect, some are
simple and good for screening incidence of colour blindness and its probable type.
They are :
1. Pseudo isochromatic
11
(polychromatic) charts
(i) Ishihara plates (only good for red-green deficiency)
(ii) Stilling plates
(iii) Hardy-Rand-Ritler plates (Useful in blue blindness)
2. Lantern test (Edridge green lantern)This is widely used test in railways and air-
ways. It examines ability for a person to differentiate red from green signal under
various conditions of brightness and size from a standard distance.
3. Fransworth hue discrimination test
(i) Frans worth D-15 test.
(ii) Frans worth Munsell 100 hue test.
4. Nagels anomaloscopeThis fails to detect blue blindness.
5. Holmgrens wool test is a complicated time consuming test.
6. Inter Society Colour Council aptitude testIt has too many negative results.
7. Sloan achromatopsia testThis is useful in total colour blindness.
Common Clinical Types of Colour Blindness
1. Achromatopsia. This is an autosomal recessive condition. The children have poor
vision, sensory nystagmus, photophobia and total colour blindness. They have either
absent or have marked abnormal cone function. To them all colours are various shades of gray
which is confirmed by Sloan achromatopsia test.
Photophobia in achromatopsia is not a true photophobia seen commonly in children
due to irritation of trigeminal nerve. These children avoid bright light to keep the rods dark
adapted
12
to use them for better vision.
All children with nystagmus and poor vision should be examined for achromatopsia.
Similarly all children with photophobia without evidence of irritation of fifth nerve should also
be tested for achromatopsia. There is no treatment, however extra dark glasses with side
covers give some improvement in vision. Any error of refraction should be corrected. Low
vision aids do not have much value. The children have to be brought up as visually challenged.
2. Other congenital colour defects. These defects are present at birth but detected
later. The child and his parents may not be aware of the defect unless pointed out. They have
generally normal vision with normal eyes, do not suffer from any neurological deficit, lead a
normal life. They generally have anomalous pigments in the cones.
Acquired colour defect :
The causes can be :
1. Lenticular (not seen in children)
2. Retinal Macular
Pan retinal photo coagulation
3. Optic pathway
Retinal :
1. Lesions of outer layers of retina give rise to rarer form of blue-yellow defect.
2. Lesions of inner layer give rise to red green defect.
The conditions that cause retinal colour defect are - pan photo coagulation, macular
degeneration, high myopia and chorioretinitis.
Optic pathway. Optic pathway not only carries visual impulses but also colour sense
to the central nervous system. Hence it is but natural that lesions of optic pathway should
produce colour defect. In lesions of optic nerve, the colour sense is not totally abolished, it
seems to be washed out
13
. Reduced colour sense is so consistent with lesion of optic nerve that
diminished vision with normal colour senses excludes possibility of optic nerve lesion. In le-
sions of optic nerve, the coloured object when centrally fixed has duller colour than when
shifted to periphery due to central relative scotoma.
Colour vision is diminished even before central vision is affected.
14
Colour field may be
lost in not only in central field but also in peripheral field also.
Inability to recognise colours when no colour blindness is present is called colour
agnosia. Inability to recall the name of colour is called amenestic colour aphasia.
14
Visual aphasia
3
(word blindness). In this condition, the child has normal vision and
colour sense but can not recognise words, letters or colours. The child can not copy written
letters but can write spontaneously. It is generally seen in involvement of optic radiation.
Agraphia is inability to express meaning in written language while alexia means in-
ability to read.
Dyslexia
15
. Dyslexia is more serious and common disorder in children than alexia. In
alexia the child can not comprehend written words. Incidence of dyslexia in developed coun-
tries is as high as 4 to 6 percent. No data is available for developing countries, most probably
because its presence is not appreciated and the child is considered to be dull. In fact dyslexic
children have normal or higher intelligence than other children.
The condition is thought to be central in origin, exact location of the lesion is not
known. It affects both the sexes equally. There may be positive history of learning difficulty
in members of the same family.
The condition is congenital but detected only when the child is admitted to school. The
child has difficulty in relating letters and groups of letters with appropriate sound
15
. The
condition is permanent.
Diagnosis is not simple. It requires specially trained, multi disciplinary team to diag-
nose and train the child to lead as normal life as possible. Ophthalmologist role is to exclude
other causes of reading difficulty i.e. error of refraction, muscle imbalance, bilateral amblyopia
and treat them as far as possible.
Agnosia. Visual agnosia is a condition where a person can not recognise an object by
sight but can recognise the object by touch or sound. This is caused due to lesions of Broadmanns
area 18.
16
Visual hallucination. This is a condition where the patient complains of seeing an
object that is not present. There are two types of hallucinations :
(i) Formed hallucination where the objects claimed to be seen are identifiable objects.
(ii) Unformed hallucination consists of lights, colours or amorphous images. There may
be a mixture of both.
Many visual hallucination may be associated with auditory hallucination in psychologi-
cal causes. Many of the drugs are known to produce visual hallucination.
Scotomas. Scotomas are defects in field of vision. They can be central i.e. round 30 of
point of fixation or peripheral beyond 30.
They are called absolute if the size and densities of scotoma does not change with size,
colour of target and illumination. They are called relative if the size and density of scotoma
changes with size and colour of target and illumination.
A scotoma is said to be steep if the margins of the field defect do not change with change
in size of object. If the margins of the scotoma is different with different targets then the
scotoma is called sloping.
A congruous field defect refers to similarity of scotoma to the contra lateral scotoma
when they are superimposed on each other, they should match in outline.
The scotomas can be :
1. Choroioretinal
2. Retino neural
3. Optic nerve type
4. Chiasmal
5. Optic tract type
6. Geniculate
7. Optic radiationTemporal, perital
8. Cortical
Chorioretinal scotomas can be isolated or multiple without any fixed pattern. They
do not respect either and horizontal raphe of retina or vertical line of chiasmal lesion. They
may be unilateral or bilateral.
Retino-neural scotomas can be :
Central
Centrocecal or
Paracentral. They are due to involvement of papillo macular fibres. A scotoma is said
to be central when it covers the point of fixation. When the central scotoma extends in to the
blind spot it is called centrocecal scotoma. If some of the papilomacular fibres other then
macular are involved, the scotoma is called para central. This does not involve either the
point of fixation or blind spot.
The lesion when involve, the nerve fibre bundle cause :
Bjerrums scotoma
Seidel scotoma
Roennes step
The two Bjerrums scotoma may join to form an annular scotoma with Rennes step.
These scotomas are monocular. The involvement of the other eye is independent of first
eye.
Scotomas due to lesions of optic nerve produce central scotoma that may be rela-
tive or absolute. They are unilateral unless the contra lateral nerve is also involved. The
scotomas of optic nerve are associated with :
Diminished vision
Diminished colour sense
Diminished contrast sensitivity and
Afferent pupillary defect.
If the lesion is nearer the chiasma, it will produce ipsilateral diminished vision and
central scotoma with absolute or relative contra lateral hemianopia.
Scotomas due to chiasmal lesions :
The scotoma contains uncrossed temporal fibre of ipsilateral eye and crossed nasal
fibres of contra lateral eye. The ratio of crossed to uncrossed fibres is 3:2
17
. Crossing of nasal
fibres is not straight, the inferior nasal fibres form a curve that encroaches the posterior end of
the contra lateral optic nerve. The superior nasal fibre similarly form a bend into the anterior
end of ipsilateral optic tract. These arrangements result in various types of scotomas due to
location of the lesion, size of the lesion and number of lesions.
1. The anterior chiasmal lesion on one side only produces ipsilateral central scotoma
and contra lateral hemianopia.
2. Lesions posterior chiasma will produce homonymous hemianopia i.e. tempo-
ral loss on the one side and nasal loss on the contra lateral side. The defects are
incongruous.
3. Lesions pressing the mid chiasma - This is the commonest type of field defect
produced by sellar or suprasellar lesions. It is a heteronymous field defect. It
produces bitemporal hemianopia. The bitemporal hemianopia may start in one
eye in the superior temporal quadrant and gradually increasing towards inferior tem-
poral quadrant. In the right eye, the progress is clockwise, in the left eye it is
anticlockwise. Initially the vertical line is respected i.e. the field change does not
spread towards the nasal side. Once the whole of the inferior temporal quadrant has
been lost, the field encroaches beyond the midline.
If the lesion is small enough to involve the junction of crossing of macular fibres, there
will be small central bitemporal hemianopia more for red object than for white.
18
Lesions of chiasma do not cause afferent pupillary defect. Commonest cause of mid
chiasmal lesions is pituitary tumour, other causes that cause bitemporal hemianopia are
hydrocephalus, inflammation, infection, craniophryngioma, glioma of optic nerve or chiasma.
4. Binasal hemianopias are rare, are not met with in children. It requires two lesions
pressing upon the chiasma from two temporal sides.
Scotomas due to optic tract lesions. The optic tract on each side contain ipsilateral
uncrossed temporal fibres and crossed nasal fibres from the contra lateral side. Hence they
produce homonymous hemianopia and the field is split through the fixation
19
. They may be
associated with afferent pupillary defect, the lesions are incongruous. Posteriorly placed
lesions produce more congruity
20
, vision is generally good.
Scotomas due to geniculate lesions. Lesions of lateral geniculate body are rare. As
the lateral geniculate body is relatively small area, and contains predominantly macular fi-
bres, the lesion is incongruous homonymous hemianopia. The scotomas are sectorial,
the apex points towards the point of fixation. Good vision is possible.
Scotomas due to lesions of optic radiation. After synapsing in the lateral geniculate
body, the visual pathway fans out in the brain substance. There are mainly two types of
scotomas :
1. Due to lesions of temporal lobe (Meyers loop). The scotoma is called pie in the
sky due to its shape which is sectorial with apex towards point of fixation in the
superior quadrant. The scotoma is homonymous and respect the midline. Small vas-
cular lesions generally cause quadraniopia. Large lesions cause hemianopia. The le-
sions are due to involvement of anterior inferior fibres. Good vision is rule. The
pupillary response is unaffected.
2. Due to lesions in parietal lobe. The lesions involve superior fibres of optic radia-
tion produce quadranopia in lower quadrant and called pie on the floor. The scotoma
are homonymous and congruous. There is good vision with normal pupillary reac-
tion.
Scotomas due to lesions of visual cortex. The fibres from the optic radiation synapse
in calcarine fissure in occipital lobe are mostly macular that is located at the top of the occipital
cortex. This area has dual blood supply from middle cerebral and posterior cerebral
arteries.
The scotomas are congruous, homonymous and hemianopic. The scotomas may
involve macula or macula may be spared. The scotomas respect vertical meridian can re-
spect horizontal meridian as well. Sometimes a temporal crescent may be present. Probable
causes of macular sparing are :
1. Dual blood supply to the occipital cortex.
2. The macular area is spread over a large area hence it is possible that enough macular
fibres are spared to retain macular vision.
3. It is presumed that there is double innervation of macular area.
Some specific type of field defects :
1. Enlargement of blind spot is not always diagnostic. It is seen in :
(i) papilledema
(ii) Chronic simple glaucoma
(iii) Opaque nerve fibres
(iv) Drusen of optic nerve
(v) Coloboma of optic disk
(vi) Peripapillary edema of retina
(vii) Juxtapapillary chroiditis.
2. Constriction of peripheral field :
(i) Onset of optic atrophyPost papilledematous or post neurotic.
(ii) Retinitis pigmentosa
(iii) Advanced glaucoma
(iv) Hysteria
(v) Malingeing
3. Altitudinal field defect :
(i) Acute ischaemic optic neuropathy
(ii) Occlusion of central retinal artery branch
(iii) Trauma to superior occipital lobe
(iv) Optic tract syndrome
Cortical blindness. In these cases the patient is blind but denies blindness. The
pupillary reaction is normal. There are no fundal findings that can be attributed to blindness.
The lesion is bilateral, occipito cortical.
Bilateral homonymous hemianopia. These scotomas are difficult to chart, have poor
prognosis. Vision may not improve beyond hand movement, is caused due to ischaemic lesion
of both the occipital cortexes. There may be some gap between involvement of two cortexes.
Amblyopia
22, 23, 24, 25, 26
Amblyopia is a common cause of diminished vision in children which if not treated early
results in permanent loss of vision generally in one eye but on occasions it may be bilateral.
It has been defined variously from time to time.
Most accepted description states it to be a symptomatic disturbance of vision in children
due to deprivation of vision or abnormal binocular interaction.
24
The cause of which can not be
explained by presence of disorders of ocular media or visual pathway. When amblyopia is
present without any detectable cause, is called pure amblyopia.
23
Prevalence of amblyopia ranges between 0.5 to 3.5 percent in school going children.
25
It is seen equally in boys and girls. No race is immune. There is no fixed hereditary factor,
however, the predisposing causes like error of refraction or concomitant squint may have ge-
netic background.
Amblyopia develops due to failure of visual pathway development, which may start at
birth or soon after. Development of amblyopia after full maturation of visual pathway is rare.
Onset after six to seven years is almost unknown.
For development of visual pathway it is essential to have a clearly formed image on the
macula. The macular development is stretched up to 6 to 7 years post natal. Any derangement
of formation of vision during this period is bound to cause amblyopia. Amblyopia developing
early is more difficult to manage than those developing later. Anything that prevents forma-
tion of equally clear image on both the maculae i.e. physical obstruction like ptosis, corneal
or lenticular opacity, anisometropia or squint will result in amblyopia.
Characteristics of amblyopic eyes :
1. Amblyopia starts in the critical period of development of visual pathway.
2. It is seen in children under six to seven years of age.
3. If amblyopia is not treated before eight years of age it becomes permanent.
4. Amblyopia is mostly uniocular disorder however in a very small percentage of cases
it can be bilateral.
5. It is not possible for the parents to know that the child is amblyopic unless the child
undergoes examination of the vision. This happens more often in children who have
amblyopia in spite of straight eyes.
6. In contrast to this a child may be brought with squint and found to have amblyopia.
This is also known as amblyopia with squint.
7. Diminished distant vision - The amblyopic eye always has poorer vision than the
normal fellow eye. A difference of two lines on Snellens chart after best correc-
tion is diagnostic.
8. Vision in amblyopic eye may be as low as perception of light in congenital squint.
Generally vision in amblyopic eye ranges between 6/12-6/18. A child may have 6/36
vision before starting treatment and end up with 6/12 vision. This eye will still be
considered to be amblyopic.
9. Crowding phenomenon. An amblyopic eye has better vision when single optotypes
are shown but when letters of same size in a line are shown, the child is unable to
read them. Greater the difference between the single letter vision and linear
vision, poorer is the prognosis.
10. Vision with neutral density filter. In non amblyopic eye if neutral density fil-
ter is put in front of the eye, the vision is reduced by one to two lines but not in
amblyopic eye when neutral density filter of increasing strength is put in front of the
eye the vision either remains same or even improves.
27
In case of organic amblyopia
i.e. toxic amblyopia vision is markedly reduced with neutral density filter.
26
The
explanation for the phenomenon isThe scotopic and mesopic vision of an
amblyopic eye is equal to non amblyopic eye but less in photopic vision. Placing a
neutral density film simulates scotopic vision, thus it is not affected.
11. Colour vision and dark adaptation are normal in amblyopia.
12. Contrast sensitivity, grating acuity, Vernier acuity and spatial localisation are low-
ered.
13. Visual evoked response to pattern stimuli is poorer in amblyopic eye.
14. There may be milder degree of relative afferent pupillary response.
15. The amblyopic eye may have mobility defect in the form of pursuit, fixation and
saccades.
16. There may be latent nystagmus.
17. There may be poor accommodation.
18. Severe degree of amblyopia may have eccentric fixation.
Classification of amblyopia. There is no unanimity in classification of amblyopia.
Various classification have been done on the basis of presumed physiopathology of the condi-
tion. Many of the terms used in the past are either avoided
22
or altogether discarded.
Various terms used in amblyopia are :
1. Strabismic amblyopia. Amblyopia due to abnormal interaction between two eyes
due to squint.
2. Anisometropic amblyopia. Amblyopia due to uncorrected unequal refraction into
eye.
3. Stimulus deprivation amblyopia is caused due to blurring of retinal image due to
opacity in the media, i.e. congenital or traumatic cataract, corneal opacity, ptosis. It
was formerly called amblyopia exanopsia. The term is no more in vogue
22, 24
.
4. Ametropic amblyopia is caused due to high uncorrected error of refraction.
5. Meridional amblyopia due to high astigmatism in a particular meridian.
6. Amblyopia of arrest due to arrest of development of vision.
7. Amblyopia of extinction due to secondary loss of vision.
The last two terms are also not used any more.
The toxic amblyopia, nutritional amblyopia and amblyopia in various neuropathies like
retrobulbar neuritis do not fit into the definition of amblyopia hence they are not included in
this chapter.
One of the widely used classification is to divide amblyopia into :
(1) Organic amblyopia and (2) Functional amblyopia. The latter is used to denote
psychological disturbance and does not fulfil the criteria of amblyopia.
Organic amblyopia is also known as developmental amblyopia and can be unilat-
eral or less commonly bilateral. The bilateral developmental amblyopia is generally
stimulus deprivation amblyopia and the causes are - Bilateral cataract of equal density, high
uncorrected hypermetropia and motor nystagmus.
The uniocular developmental amblyopia can be strabismic or anisometropic separately
or in combination.
For practical purposes the best way is to classify amblyopia is amblyopia with squint
and amblyopia without squint. The latter is called straight eye amblyopia.
Amblyopia with squint :
Characteristics :
1. The child constantly uses one eye for fixation. The deviating eye becomes amblyopic,
patients with alternate fixation do not develop amblyopia.
2. Esotropes develop more amblyopia than exotropes. Congenital esotropes do not de-
velop amblyopia due to cross fixation.
3. Hypertropes generally do not develop amblyopia as they manage to maintain fusion
by abnormal head posture.
26
4. Micro strabismus causes more amblyopia when compared to large degree squint
27
.
5. Strabismus causes more amblyopia than anisometropia.
6. Strabismic amblyopia is caused due to suppression of image in the squinting eye.
7. Amblyopia with squint report for checking earlier than straight eye amblyopia, not
because they are aware of diminished vision but for cosmetic blemish of squint.
8. Degree of amblyopia is influenced by duration of squint rather than age of onset of
squint.
9. Earlier the squint develops, deeper is the amblyopia and more difficult to treat. Thus
in case of congenital squint where inhibition is present at birth, the vision in the
amblyopic eye will not improve beyond perception of light.
25
10. The suppression is at the level of cortex.
27
11. The condition is less amenable to treatment than straight eye amblyopia.
12. No correlation exists between severity of amblyopia and angle of squint. Micro tropias
are known to cause severe amblyopia.
Straight eye amblyopia :
Characteristics :
1. Error pf refraction :
(i) High degree of hypermetropia in one eye, the amblyopic eye called anisometropic
amblyopia.
(ii) Both eyes have equal errors of refraction mostly of high degree.
(iii) No significant error of refraction.
2. Amblyopia is obligatory i.e. suppression is present when the eye squints and con-
tinues during forced fixation
28
.
3. The condition goes unnoticed because the child is unaware of less vision in one eye
and the parents do not notice because there are no external signs.
4. The eyes have fairly good binocular function.
5. The steriopsis is good.
6. Chances of eccentric fixation is minimal or absent.
7. The blurred image in the amblyopic eye acts as hindrance to binocular vision.
8. Only rough correlation exists between degree of anisometropia and depth of
amblyopia.
9. It is possible to have amblyopia in both eyes.
10. The condition has better chance of improvement following treatment.
Diagnosis. Diagnosis of amblyopia in squint is easy. All squinting eyes should be ex-
amined for possibility of amblyopia. Strabismic children have 15 times more chances of devel-
oping amblyopia than non strabismic children. Squint is responsible for one third case of
amblyopia.
The diagnosis depends on :
1. Recording of vision.
2. Crowding phenomenon.
3. Unchanged or improved vision with neutral density filter.
Treatment :
1. Management of amblyopia is rewarding
(i) If diagnosed early,
(ii) If treatment is initiated early,
(iii) If treatment is continued for sufficient time and maintained for sufficient time
after vision has improved and come to equal level in both eyes.
All above factors depends upon compliance of the patient and co-operation of parents
and teachers.
Management begins withRefraction under cycloplegia followed by prescrip-
tion of best possible power in both eyes which gives the child a comfortable vision according to
standard protocol of prescription of glasses for children with special attention to astigmatism.
Any opacity in the media should be removed, so should be any physical obstruction like ptosis,
tumours of lid.
This is followed by occlusion of sound eye to begin with. Occlusion is a time honoured
method of treatment for amblyopia in children.
Occlusion therapy should take into consideration following parameters :
1. When to start occlusion.
2. Gap between two visits.
3. Type of occluder to be used.
4. How long should occlusion be continued.
5. Should the better eye be occluded and how long.
Occlusion should be started as soon as amblyopia has been confirmed. Occlusion after
six years of age do not have much impact on amblyopia though it has been observed to give
fairly good result up to age of fifteen.
23
The occlusion should be complete and constant to begin with.
There are various types of occluders. The best is a skin patch attached to the fore-
head and check by an adhesive tape. This occludes the eye fully. The child has no chance to
peep over the patch which is always possible with spectacle occluders i.e. Doynes occluder
or ground glass occluders. These are not favoured because the child has tendency to peep
over the occluders. In a co-operative child an opaque contact lens may give equally good
result like a patch especially if the child is already using contact lens.
1. Minimum amount of occlusion required per day is not known.
2. Constant occlusion for a period calculated as per age of the child gives good results.
3. 75% waking hours occlusion also gives fairly good result and has the advantage of not
being amblyogenic to the better eye.
4. Occlusion for less than 50% waking hours are not likely to be of much help.
Calculation of duration of occlusion. The commonly used formula is one week for
each year of age in between the visits. Younger the child better is the result of the treatment.
Occlusion of better eye is called conventional occlusion that is effective irrespective of fixa-
tion pattern of fixing eye. Occlusion of amblyopic eye is called inverse occlusion.
A rough estimate of occlusion as per age of the child is as follows
26
Age Conventional occlusion Inverse occlusion
0-1 year Three days One day
1-3 years Four days One day
4-6 years Six days One day
More than 6 years Seven days Nil
As far as possible occlusion should be full time i.e. during all waking hours. Part time
occlusion means that the eye is occluded only for a few hours. During rest of the time both the
eyes are open which goes against the principle of total occlusion.
Occlusion therapy may be accompanied by enhanced near vision work like tracing pic-
ture, joining dots, hand video games.
Follow up. The child should be followed up at the frequency of 1 week per year of
age, i.e. a child aged one should be examined every week while a child of three years is exam-
ined after three weeks of occlusion.
What to look for on follow up visits :
1. On every visit childs vision in both eyes must be recorded separately with correction
when needed on the same Snellens chart in the same setting always.
2. The fixation pattern of the amblyopic eye should be noted on every visit.
How long should occlusion be continued ?
Occlusion is continued till :
1. Vision in amblyopic eye is as good as in normal eye.
2. Fixation becomes alternating.
3. There is no improvement of vision following occlusion for three months.
4. Once vision has been equalised or the child has developed alternating. Full time oc-
clusion can be stopped and the dominant eye is occluded for part of the waking hours.
This part time occlusion is continued up to age of eight years after which chances of
improvement are less. Visual improvement is generally better in straight eye
amblyopia than amblyopia with squint. Anisometropic amblyopia may require
occlusion in addition to correction of refraction. Amblyopia is known to recur follow-
ing completion of occlusion. So all children with amblyopia should be examined fre-
quently and as soon as there is recurrence, occlusion is started as was done on the
first occasion.
Occlusion of sound eye is contra indicated in amblyopia with eccentric fixation.
CAM vision stimulator. The normal eye is occluded for 7 minutes. The child uses the
amblyopic eye to draw patterns on the transparent cover placed over the stimulator that has
slow rotation, high contrast grating with sharp edges. The child may be exposed to the stimulator
10 to 20 times at a stretch. This method is not superior to conventional occlusion.
Complication of occlusion. Occlusion is a safe, simple and sound method for treat-
ment of amblyopia provided it is done properly in selected children for sufficiently long time.
The co-operation of the child and parent are some of the prerequisites.
30
They are :
1. Occlusion amblyopia in better eye.
2. Development of new strabismus.
3. Worsening of pre-existing strabismus
4. Intractable diplopia
5. DVD
6. Allergic reaction to occluder
7. Infection may develop in occluded eye.
Pharmacological defocusing (Penalisation). This may be considered as a modified
type of occlusion without actually covering the eye. This uses cycloplegic drug and spectacle in
various combination to change the accommodation. The vision is blurred in better eye and
forcing the amblyopic eye to see. The best results are obtained in amblyopia of moderate depth
in moderate hypermetropia. The amblyopic eye is given full correction. The better eye is de-
nied spectacle correction. One drop of one percent atropine is instilled in the better eye once a
day. Follow up and duration is similar to that of occlusion. It is recommended if the child does
not tolerate occlusion or peeks over the occlusion. It takes more time for improvement than
occlusion.
Surgery. Squint surgery is performed after amblyopia has been treated. However ptosis,
haemangioma of lid, cataract are operated before starting occlusion.
Medical treatment. Levodopa has been tried in small groups of amblyopic children
with improvement of vision in both amblyopic and non amblyopic eye without any side effect.
REFERENCE8
1. Miller S.J.H. ; Symptomatic disturbance of vision in Parsons disease of the eye.
Seventeenth edition. p-231236, Churchill Livingston, London, 1984.
2. Marmor M.F. ; Clinical physiology of the retina in Principles and practice of oph-
thalmology. Vol. II. p-868887, Edited by Peyman G.A., Sanders D.R., Goldberg M.F.
First Indian edition. Jay Pee Brothers, New Delhi, 1987.
3. Brain R.W. ; Visual fibres and visual fields in Diseases of nervous system. Sixth
edition. p-4958, Oxford Medical Publication, London, 1962.
4. Brain R.W. ; Aphasia in Diseases of nervous system. Sixth edition. p-8490, Oxford
Medical Publication, London, 1962.
5. Shaffer D.B. ; Fundus albipunctatus in Text Book of ophthalmology. Edited by Scheie
H.G. and Albert D.M. Ninth edition. p-307, W.B. Saunders Company, Philadelphia, 1977.
6. Agarwal L.P. ; Properties of light in Principles of optics and refraction. Second
edition. p-34, C.B.S. Publishers, New Delhi, 1979.
7. Majji A.B. ; Sharma Y.R., Rajashekhar Y.L. and Nath R. Colour vision and colour blind-
ness in Modern ophthalmology. Vol. II. Second edition. p-10321036, Edited by Dutta
L.C. Jay Pee Brothers, New Delhi, 2000.
8. Speath G.L., Auerbach V.H. ; Cone vision in Pediatric ophthalmology. Vol. II, second
edition. p-10611064, Edited by Harley R.D. WB Saunders Company, Philadelphia, 1983.
9. Seal S.K. ; Symptomatic disturbance of visual function in G.N. Seals Text Book of
Ophthalmology. Fifth edition. p-352353, Current Book International, Kolkata, 2002.
10. Boger W.P., Peterson R.A. ; Pediatric ophthalmology in Manual of ocular diagnosis
and therapy. Third edition. p-262263, Edited by Deborah Pavan Langston. Lippincot
11. Duke Elder S. ; Colour sense in System of Ophthalmology. Vol. VII. p-380383, Henry
12. Boger W.P. and Peterson R.A. ; Achromatopsia in Manual of ocular diagnosis and
therapy. Third edition. p-273274, Edited by Deborah Pavan Langston. Lippincot
13. Kanski J.J. ; Neurophthalmology in Clinical ophthalmology. Second edition. p-441,
14. Martyn Lois J. ; Colour vision disorder in Pediatric ophthalmology. Vol. II. Second
edition. p-781, Edited by Harley R.D. WB Saunders Company, Philadelphia, 1983.
15. Keys M.P. ; Dyslexia in Current ocular therapy. Fifth edition. p-213214, Edited by
Fraunfelder F.T., Roy F.H. W.B. Saunders Company, Philadelphia, 2000.
16. Roy H.F. ; Visual agnosia in Ocular differential diagnosis. Third edition. p-453, Jay
17. Shirley H. Wray. ; The visual fields in Manual of ocular diagnosis and therapy.
Third edition. p-329, Edited by Deborah Pavan Langston. Lippincot. Williams and Eilkins,
Philadelphia, 1991.
18. Rosenberg M.A. ; Topographic diagnosis of field defect in Principles and practice of
ophthalmology. Vol. III. First Indian edition. p-19361943, Edited by Peyman G.A.,
Sanders D.R. and Goldberg M.F. Jay Pee Brothers, New Delhi, 1987.
19. Norma Graber. Matching field defect to pathology in Visual field examination. First
Indian edition. p-18-33, Jay Pee Brothers, New Delhi, 1989.
20. Bajandas F.J., Kline L.B. ; Visual field in Neurophthalmology review manual. Third
21. Rabinowicz M. ; Amblyopia in Pediatric ophthalmology. Vol. I, edition second.
p-293341, Edited by Harley R.D. WB Saunders Company, Philadelphia, 1983.
22. Eggers H.M. ; Amblyopia in Text Book of Ophthalmology. First edition. p-13.1 13.16,
Edited by Podos S.M. and Yanoff M. Gower Medical Publication, London, 1993.
23. Rohatgi J.N. ; Amblyopia in Modern ophthalmology. Vol. II. Second edition. p-875-
878, Edited by Dutta L.C. Jay Pee Brothers, New Delhi, 2000.
24. Sharma P. ; Amblyopia in Strabismus simplified. First edition. p-4149, Modern Pub-
lishers, New Delhi, 1999.
25. Rohatgi J.N. ; Amblyopia in Squint basic and clinical aspect. First edition. p-8091,
CBS Publication, New Delhi, 2003.
26. Mohan K. ; Amblyopia in Clinical practice in ophthalmology. First edition. p-529-
533, Edited by Saxena S. Jay Pee Brothers, 2003.
27. Seal S.K. Amblyopia in GN Seals Text Book of Ophthalmology. Fifth edition.
p-349351, Current Book International, Kolkata, 2002.
28. Boger W.P. and Peterson R.A. ; Pediatric ophthalmology in Manual of ocular diagno-
sis and therapy. Third edition. p-307, Edited by Deborah Pavan Langston. Lippincot.
Williams and Wilkins, philadelphia, 1991.
29. Lyle T.K. and Wyber K.C. ; Adaptation to development of ocular deviation in Lyle and
Jacsons practical orthoptics. Fifth edition. p-82, First Indian edition. Jay Pee Broth-
ers, New Delhi, 1994.
30. Keech R.V. ; Functional amblyopia in Current ocular therapy. Fifth edition. p-214
216, Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia,
2000.
31. Fiona J. Rowe. ; Amblyopia in Clinical orthoptics. First edition. p-131135, Blackwell
Science Ltd., London, 1997.
593
CHAPTER 17
Disorders of Extra Ocular Muscles and
Paralytic 8quint in Children
General information about extra ocular muscle
There are six muscles attached to each eye, to move the eye in various directions. The
movements are either voluntary or reflex. The muscles are striated in nature and are sup-
plied by cranial nerves - Three, four and six for motor function. The sensory supply to all of
them are through fifth nerve, they are not innervated by autonomic nerves.
The six extra ocular muscles (EOM) areFour recti i.e. the superior rectus (SR), the
lateral rectus (LR), the medial rectus (MR) and the inferior rectus (IR). The four recti
muscles combine to exert a retracting influence also on the globe
1
because they originate
from the apex of the orbit and are inserted in front of the equator of the globe. The two
oblique muscles are the superior oblique (S.O.) and the inferior oblique (I.O.), the func-
tional origin of both are in the rim of the orbit in front of the eye ball and are inserted behind
the equator of the globe. The two obliques combine to give a protracting influence on the globe.
1
The six muscles can be divided into two main groupsHorizontally acting muscles
i.e. the medial and lateral recti. They have only primary action of moving the eye in
horizontal plane. They are inserted in front of the equator in a straight line. Their muscle
plane coincides with visual axis hence they have only horizontal action.
Cyclo vertically acting muscles
They are four in numbers, two recti i.e. the superior and inferior recti both are supplied
by single cranial nerve i.e. oculomotor and both are adductors. The remaining two muscles are
the two obliques i.e. the superior and inferior obliques, both have functional origin in front
of the globe and are abductors of the globe. Unlike the vertically acting recti, they have differ-
ent motor supply. The superior oblique is supplied by the trochlear nerve while the inferior
oblique is supplied by the lower division of the oculomotor nerve. The muscle plane of
cyclo vertical muscles do not coincide with visual axis in primary position.
The extra ocular muscles contain two types of fibers.
2
The slow fibres and the fast
fibres. The former are on the exterior surface of the muscles and produce graded contracture
of the muscles. The latter are situated on the interior surface of the muscles and are concerned
with rapid movements.
The ocular movements can be divided into four broad groups
3
The saccades, the
smooth pursuit movements, the vergence and vestibulo ocular movements.
The saccades are rapid conjugate eye movements, they are voluntary movement due
to quick burst of activity. They jump from one fixation to a new object of regard.
4
They are
present at birth.
3
The smooth pursuits are involuntary movements that follow a slow moving object.
They are also conjugate movements.
The vergence
5
When the eye movements are disjugate (disjunctive), the movements
are called vergence. The eyes move in opposite direction to allow both fovea to maintain fixation
at the same time even when the object moves near or farther away, the former is called
convergence and the other is called divergence. The advantage of this bifoveal fixation is
binocular single vision and stereopsis.
4
The vestibulo ocular movements
3
These movements are related to maintaining eye position when there is an altered head
position or body posture.
The ability of the human eyes to move in various directions is immense, the human eyes
can have almost 180 of binocular field. The eyes can be moved 50 in each direction from the
primary position.
Various position of the eye (Gaze)
1. Primary position. When the eyes fix an object at 20 feet straight ahead with the
head straight.
2. Secondary position consists of right gaze, left gaze, straight up and straight down
gaze.
3. Tertiary position. There are four oblique position of the eye.
4. The cardinal position. They are six in numbers brought about by one muscle and
its yoke muscle.
Agonist and antagonist muscles
These are descriptive terms used to denote the action of an extra ocular muscle.
An agonist is the prime mover for a required direction.
An antagonist of the same eye acts directly against the agonist.
If right lateral rectus is agonist for right lateral gaze, the medial rectus is its direct
antagonist in the same eye.
Synergist muscle
A muscle in the same eye that enhances the action of a particular muscle to achieve a
particular action is called synergist. In fact both of them are synergist to each other. For
example superior rectus is synergist to inferior oblique for elevation. It is synergist to superior
oblique for intortion. Adduction is enhanced for medial and inferior rectus.
Yoke muscles. These are pairs of muscles one from each eye that work together in
binocular movement in one direction (version).
DISORDERS OF EXTRA OCULAR MUSCLES AND PARALYTIC SQUINT IN CHILDREN 595
Duction is movement of one eye i.e. abduction moving laterally, adduction moving
medially.
Sursum duction (elevation)
7.8
is upward movement either in primary position or when
the eye is either adducted or abducted. Deorsum duction is downward movement (depression
or infraduction). This can be in primary position or secondary position of adduction or abduction.
Similarly there can be in cycloduction when the twelve Oclock meridian dips nasally or
excycloduction when twelve Oclock meridian dips temporally.
Versions. These are binocular movements in the same direction i.e. conjugate
movement. Versions are noted in three diagnostic directions. Right sided binocular
movements are called dextroversion, levo version denotes left binocular movements,
dextro elevation and dextro depressions represent right and up, left and down movement
respectively.
Sursum version means moving both eyes up. Deorsum version is moving both eyes down.
The binocular torsional movements are Dextro cycloversion when the right eye extorts
and left eye intorts at the same time. Levo cyclo version similarly denotes right intorsion and
left extorsion.
Advantage of testing version is that paresis that may be missed on uniocular
movement can be diagnosed on binocular testing.
Vergences
Binocular movements in opposite directions are called vergences.
They areConvergence, when both eyes move inwards and divergence when both
eyes move outwardly.
Laws governing ocular movements
They are Herings law of equal innervation and Sherringtons law of reciprocal
innervation.
Herings law of equal innervation means that the synergists of one eye gets equal and
simultaenous innervation for any movement, e.g. amount of innervation of the right medial
rectus is exactly the same as that of left lateral rectus and inhibitory responses to the right
lateral rectus is exactly the same as that of left medial rectus.
Sherringtons law states that increased action of an extra ocular muscle associated
with diminished contraction of its antagonist for example during convergence there is increased
contraction of both medial recti with reduced contractibility of both lateral recti.
Neural control of ocular movements
Binocular movements of the eye consists of a delicate control of many systems and not
confined to motor nerve supply to the muscles only. They consist of two independent groups
of movement i.e. version and vergence
7
, both are binocular movements. The versions are
conjugate movements while vergence are disjugate movements. Depending on input, the ocular
movements can be classified into fast eye movement, slow eye movement and vergence
eye movement.
The fast eye movements :
1. Saccade
2. Quick phase of nystagmus.
The fast eye movements bring the object of interest on the fovea.
The slow eye movements :
Smooth pursuit
Optokinetic
Vestibular
Vergence
The slow eye movements hold the image of the object of interest on the macula.
In vergence the eyes move in opposite direction i.e. converge or diverge so that images
of single object are placed on both foveae.
8
The fast eye movements (saccade) are brought about by fronto mesencephalic path
ways and superior colliculus. The quick phases of nystagmus is brought about by para
median reticular formation in mid brain and pons.
The slow eye movements (smooth pursuits) are brought about by parieto-occipial
temporal pathway.
The vestibular slow eye movements are brought about by labyrinthine-pontine-path-
way.
8
Anatomy of extra ocular muscles
The horizontal muscles
The medial rectus muscle
This is a straight muscle, the plane of the muscle coincides with the visual axis of the
eye. It originates from the apex of the orbit from the medial side of circle of Zinn. It is 41 mm
(actually 40.8 mm) in length. It passes forward between the medial wall of the orbit and the
globe with in the horizontal meridian of the eyeball. The anterior 3.7 mm to 4.5 mm (mean 4
mm) is its tendon that is 10.3 mm wide. It inserts in the sclera in vertical linear fashion 5.5
mm away from the limbus. Its insertion is nearest to the limbus when compared to other extra
ocular muscles insertion. It is innervated by lower division of third cranial nerve that pierces
the muscle from its ocular surface. It gets its blood supply by two twigs of anterior ciliary
artery. The venous drainage is through the corresponding veins of the same name that
drains in the superior and inferior opthalmic vein. The sensory supply is through the
trigeminal. It has only one action in horizontal plane that is adduction. Adduction is
better in slightly lowered gaze, this helps in converging the eye to near work. Under action of
this muscle causes exotropia. Its arc of action with the globe is 7 mm. Near the apex of the
orbit, the dura of the optic nerve is closely related to the muscle.
The lateral rectus
The lateral rectus arises from the apex of the orbit mostly from the lateral part of the
circle of Zinn and partly from spina lateralis recti which is a spike in the greater wing of the
sphenoid. Its muscle plane coincides with the visual axis so it has only one horizontal action
i.e. abduction. Its average length is 41 mm. It passes between the lateral orbital wall and the
globe. Its tendon is about 9 mm in length and 9.2 mm wide. It gets inserted in front of the
equator 7 mm from the limbus in a vertical line. It is innervated by abducent nerve that
pierces the muscle from the inner surface. It gets its blood supply from a single anterior
ciliary artery, all other recti get their blood supply by two anterior ciliary arteries. The anterior
ciliary arteries do not supply any of the obliques. Its venous supply is via the veins of the same
name that drain in the ophthalmic vein. Its sensory supply is through trigeminal. The action
of lateral rectus is best for distant fixation in slight up gaze.
The upper border of the lateral rectus almost reaches the lower border of the lacrimal
gland along with lacrimal artery in the anterior part of the orbit. The tendon of the inferior
oblique lies between the lateral rectus and the globe at its insertion.
The cyclo vertical muscles
These are two recti, the superior and inferior rectus and two obliques - the superior
and the inferior obliques. The actions of the cyclo verticals are more in number and more
complex in nature. They have all the three actions i.e. primary, secondary and tertiary in
different gaze. Their long axes do not coincides with the visual axis in primary position.
The two cyclovertical recti are adductors while the two obliques are abductors. The superior
cyclo verticals, the S.R. and S.O. are intortors.
The two inferior cyclo verticals are extortors. The primary action of all the cyclo vertical
muscles is best in abduction. The obliques have effective origin in front of the globe and insertion
behind the equator. The recti originate from the apex of the orbit and are inserted anterior to
the equator.
The cyclo vertical recti
The superior rectus (S.R.)
The superior rectus originates from the upper part of the circle of Zinn, lateral side of
optic foramen and sheath of the optic nerve, it travels forward under the roof of the orbit. Its
average length is 42 mm. Its tendon is 5.5 mm in length and is 10.5 mm wide at the insertion,
which is 7.7 to 7.8 mm away from the limbus. The tendon does not insert in a straight line but
has a mild convexity forward. The superior rectus lies under the levator and above the superior
oblique. Its insertion is farthest from the insertion of any other rectus.
In primary position, the long axis of the superior rectus form an angle of 23 with the
visual axis. The axis of the muscle coincides with visual axis only when the eye is abducted 23.
At this position, if the superior rectus contracts, the eyeball will be elevated without any torsion.
Elevation is the primary action of the superior rectus. Ability to elevate the globe increases
with abduction and decreases in adduction. For intortion, the eyeball rotates to 67 medially,
it is no more an elevator but is an intortor which is secondary action of superior rectus. Adduction
is the tertiary action of the superior rectus. Thus superior rectus is an elevator, intortor and
adductor of the globe. It is supplied by the upper division of the third nerve. It gets its blood
supply by two anterior ciliary arteries. It has its corresponding venous drainage. Its sensory
supply is through the trigeminal. One of its protective reflex action is Bells phenomenon.
The superior rectus and inferior rectus work together as adductor but oppose each other as
cyclo vertical muscles.
Inferior rectus (I.R.)
Inferior rectus originates from the circle of Zinn. It travels slightly down and anteriorly
under the globe and inserts in the sclera slightly temporal to its origin, 6 mm from the limbus
in a convex fashion with convexity anteriorly. Its arc of the contact with the globe is 6.5 mm.
Its length is 40 mm. It has a tendon 5.0 mm long, the width of the tendon is 10.0 mm at
insertion. The inferior oblique passes under it at the anterior part. The inferior rectus is intimate
with inferior oblique and the lower lid muscle. It merges with the ligament of Lockwood to
give support to the globe from below.
Its direction from its origin to insertion is similar to that of superior rectus. Its long axis
forms an angle of 23 with the visual axis when the eyeball is in primary position.
When the eyeball is abducted 23, the axis of the muscle and visual axis coincide at this
position, it is a depressor of the globe that increases with abduction and decreases in adduction.
The primary action of the inferior rectus is depression. Its secondary action is
extorsion, which is best when the eye is adducted by 67. The tertiary action of inferior
rectus is adduction.
Thus the inferior rectus is a depressior, extortor and adductor.
The oblique muscle
The two obliques, the superior and inferior obliques are unique in their arrangement
in relation to the globe and visual axis. The effective origins of both the muscles are in front of
the globe from the medial side of the rim of the orbit, both get inserted behind the equator
farther away from the farthest insertion of any rectus i.e. 14 mm and 17 mm for SO and IO
respectively. Their main function is to keep the upper pole of the limbus at 12 Oclock position
irrespective of the tilt of the head. They have a forward pulling effect on the globe, which is
counteracted by backward pull of the recti. Hence in case of paralysis of third nerve with sixth
nerve the eyeball is slightly proptosed. Both of them are abductors.
The superior oblique
The superior oblique has the distinction of having longest muscle belly, and tendon. The
muscle is divided into a long and a short part. The shorter part is the effective part of the
muscle. It is also a muscle that is innervated by single cranial nerve, a distinction shared by
lateral rectus that too has individual nerve supply. The muscle is supplied by contra lateral
nucleus of fourth nerve. Due to its peculiar position, the muscle is most susceptible to trauma.
Congenital anomaly of superior oblique is very common.
The superior oblique takes origin from the periosteum of the body of sphenoid little
superior and medial to the optic foramen.
The muscular part 40 mm in length runs anteriorly and nasally and upwards up to the
trochlea at the superio medial aspect of the rim of the orbit where it passes over the pully and
is converted almost fully to its tendon that is 20 mm in length. The tendon runs backwards,
downwards and laterally below the superior rectus. The tendon is inserted in the sclera in a
wide insertion behind the equator in the posterio lateral quadrant. The muscular part is called
direct part while the tendon is called the reflected part. The attachment of the muscle to
the trochlea is called its effective origin. The temporal side of the muscle is inserted nearer
the limbus than the medial end. The temporal end is inserted about 14 mm from the limbus,
the medial end is inserted about 19 mm from the limbus. The insertion is rounded with con-
vexity away from the limbus. At the insertion, the posterior border is very near the vertex
vein.
The effective muscle plane is formed by the reflected part of the muscle, which forms
an angle of 54 with the visual axis in primary position.
When the muscle contracts from this position, the eyeball intorts. The primary action
of superior oblique is intorsion. When the globe is adducted 54, the muscle plane coincides
with visual axis and when the muscle contracts the eyeball is depressed, the secondary
action is depression while the tertiary action is abduction.
The muscle is supplied by fourth nerve that enters the orbit outside the circle of
Zinn and enters the muscle belly from the orbital surfaces. All other extra ocular muscles get
their nerve supply from the ocular surface. As the muscle lies outside the muscle cone, its
motor nerve enters the orbit outside the circle of Zinn and the motor supply is from outside,
the muscle is least affected by retro bulbar anaesthesia but well anathesized by peribulbar
injection.
Test for fourth nerve in presence of third nerve palsy. Involvement of superior
oblique in presence of third nerve palsy is an important neuro ophthalmic test. In complete
paralysis of third nerve, the eye can not be adducted, the vertical action of SO which is best in
adduction can not be tested. The eye is already abducted which is best position for intorsion so
if the patient attempts to look down the eyeball intorts.
Blood supply. The muscle gets it blood supply from the superior muscular branch of
the ophthalmic artery.
The inferior oblique
The inferior oblique too has few peculiarities. It is the shortest extra ocular muscle
37 mm. It has almost no tendon (only 1 mm). It is only extra ocular muscle that have true
anterior origin from posterior lacrimal crest of the inferio medial part of rim of the orbit.
Its insertion is very close to the fovea.
After origin the muscle passes backwards and laterally between the floor of the orbit
and inferior rectus with in the Lockwoods ligament. It passes under the globe and gets in-
serted 17 mm from the limbus, 1 mm anterior and below the fovea.
The anterior end of the insertion lies in the same plane as lower end of insertion of the
lateral rectus. The insertion of the muscle is covered by the lateral rectus.
The action of the inferior oblique is as complex as superior oblique. Its primary action
is extortion, which is best in abduction. The secondary action is elevation and tertiary action is
abduction.
Its motor supply is through the inferior division of the third nerve.
Its blood supply is through the inferior orbital artery and medial muscular branch of
ophthalmic artery.
The muscle is most likely to be injured in fracture floor of the orbit.
Spiral of Tillaux
The attachment of the extra ocular muscles is not the same for all the recti, the distance
vary from muscle to muscle. The medial rectus is inserted nearest to the limbus followed by
interior and lateral rectus. The superior rectus is farthest from the limbus, the curved imagi-
nary line joining the insertion of the recti form a spiral called spiral of Tillaux. Its knowledge
helps in finding the attachment of the muscles during squint surgery.
Summary of action of extra ocular muscles
All recti are Adductors except lateral. The Superiors are Intortors (SR and SO). The
Inferiors are Extortors (IR and IO). The mnemonic for cyclo vertical muscles is SIN RAD.
Where S stands for Superior
In stands for Inferior
R stands for Recti
AD Stand for Adductor
Thus superiors are Intortors. The recti are adductor, this leaves the obliques as abduc-
tors and inferiors as extortors.
Muscle actions
Muscle Primary Secondary Tertiary
1. Medial rectus Adduction Nil Nil
2. Lateral rectus Abduction Nil Nil
3. Superior rectus Elevation Intorsion Adduction
4. Inferior rectus Depression Extorsion Adduction
5. Superior oblique Intorsion Depression Abduction
6. Inferior oblique Extorsion Elevation Abduction
Development of extra ocular muscles
12,13,14
All voluntary muscles in the body develop from para-axial mesoderm. Ocular muscles
are no exception. Presence of extra ocular muscles is visible as early as four weeks of gestation.
The para axial mesoderm first forms a common pre muscle mass that condensates into two
groups i.e. the pre mandibular and the maxillo mandibular condensation. The first is
supplied by oculo motor. From the first condensation develop all the muscles supplied by the
third nerve. The remaining two develop from maxillo mandibular condensation which again
divides into two groups called second head somite and third head somite. From the former
develops the superior oblique. The lateral rectus develops from the latter.
The three muscle masses are destined to be supplied by three different cranial nerves.
The oculomotor reaches the pre mandibular mass while the trocheal supplies the superior
oblique developing from the second head somite and the abducent supplies the lateral rectus
developing from the third head somite.
The insertion of the muscles develop separately from the scleral condensation near the
limbus. The muscle masses differentiate from behind forward hence the origin of the muscle is
more constant than the insertion. The levator is the last to differentiate from the superior
rectus. As the two muscles remain undifferentiated for long time and have a common motor
supply i.e. upper division of third nerve that terminates in the levator after piercing the superior
rectus. The congenital anomalies of levator are mostly associated with congenital anomalies of
superior rectus. The medial rectus is the first muscle to differentiate.
The third nerve reaches its designated muscle mass roughly at 7-8 mm stage while the
abducent reaches the lateral rectus at 8-9 mm and the trochlear reaches the superior oblique
at 10-12 mm stage.
Development of oculomotor nerves
12
The nerves concerned with movement of the eye develop from the neuroblast nearest to
the cranial part of the neural tube. The nucleus of the third nerve is first to appear and the
abducent the last. The fourth nerve reaches its target muscle last, its nucleus develops in
between development of III and VI nucleus. The nerves start developing from the cranial end
and terminate in the ocular end.
Anatomy of the nerves involved in ocular movement
Three cranial nerves i.e. the oculo motor, trochlear and abducent nerves are directly
involved in ocular movements. Other neural pathway involved indirectly with ocular movements
are
8
:
Fronto mesencephalic path
Parieto-occipital temporal mesencephalic path
Occipito mesencephalic path
Para median pontine reticular formation
Labyrinthine pontine path.
The oculomotor nerve
This is the third cranial nerve, it is the only nerve that supplies more than one extra
ocular muscles, and intra ocular muscles. It has largest nucleus situated in the mid brain
at the level of superior colliculum. It is divided into paired and unpaired nuclei. The paired
nuclei are again divided into three sub-groups and supply the ipsilateral, medial and infe-
rior rectus and inferior oblique individually. The unpaired nuclei are motor cell, pool of
superior rectus, Perlias nucleus, caudal central nucleus for levator. The Perlias nucleus is
supposed to be the center of convergence. The pre-ganglionic parasympathetic fibres origi-
nate from the Edinger Westphal nucleus. This nucleus also contains fibres for accommoda-
tion and light reflex.
The nucleus of the four nerves is below the nucleus for inferior rectus.
The superior rectus and superior oblique muscle have their motor pool in the contra
lateral side.
Connections of the third nerve nucleus
15
The third nerve nucleus is connected with following tract and nuclei :
1. Cortico nuclear tract of both sides.
2. Visual cortex through tecto bulbar tract.
3. Pretectal nuclei of both sides.
4. Nuclei of fourth, sixth and eighth nerve through medial longitudinal fibres.
The nuclear mass is supplied by posterior cerebral artery, its branches.
For ease of description, course of all nerves that supply extra ocular muscles have been
divided into :
1. Fascicular,
2. Basilar,
3. Intra cavernous, and
4. Orbital parts.
This helps to understand the relation of various structures that come on the way of the
nerves. The lesions of these structures produce definite clinical features that help to pin-point
the exact location of the lesion and many a times the nature of the lesion.
1. The fascicular part. The fibres of the third nerve travel anteriorly in the substance
of the mid brain to pass through the red nucleus and the medial aspects of the cerebral
peduncle. The fascicles join to form the two trunk of the third nerve on either side of the mid
line and emerge in the inter peduncular space (fossa) in front of the mid brain. Involvement of
fascicles at this level produces a symmetric lesions of the muscles supplied by individual nu-
clei.
2. The basilar part. As the oculomotor nerve travels forward in the inter peduncular
space. The nerve passes through the sub arachnoid space, and passes under the posterior
cerebral artery and above the superior cerebraller artery. The nerve passes parallel to
the posterior communicating artery. The fourth nerve also passes in between the poste-
rior cerebral and superior cerebellar artery, lateral to the third nerve. It is clear that in the
inter peduncular space the nerve is not related to any nerve, tract or cranial nerve hence
lesion in this area do not have any systemic neurological deficits only defect produced is in the
ocular muscles and the lesions are mostly vascular. It is the commonest site for isolated third
nerve involvement.
3. Inter cavernous part. At the anterior aspect of the inter peduncular space, the
nerve passes between the posterior clinoid and the free edge of the tentorium to pierce
the dura to enter the cavernous sinus. Initially it lies near the roof of cavernous sinus and
then in the lateral wall of the sinus. The nerve throughout its course in the lateral wall of the
cavernous sinus lies above the fourth nerve, ophthalmic and maxillary nerves. The fourth
nerve crosses the third nerve to be superior and medial to the oculomotor nerve before the
third nerve leaves the cavernous sinus to enter the orbit, however, the ophthalmic and maxil-
lary division of the fifth nerve remain inferior to the third nerve through out its inter cavern-
ous course. At the anterior end of the cavernous sinus, the third divides in to two division i.e.
a smaller superior division that supplies the levator and the superior rectus and a larger
inferior division that supplies the medial rectus, inferior rectus and inferior oblique. The
branch to inferior oblique also carries the parasympathetic fibres to the ciliary ganglion.
4. Intra orbital part. The two divisions enter the orbit through the superior orbital
fissure inside the circle of Zinn.
The superior division passes over the optic nerve for some distance and pierces the
superior rectus from its ocular surface. Some fibres travel superiorly to supply the levator
palpebral from below.
The inferior division enters the inferior rectus, medial rectus and inferior oblique from
the ocular surfaces.
The trochlear nerve
15,16,17
Trochlear nerve is an unique cranial nerve. The special features of the nerve are :
1. It is the nerve with longest intra cranial course.
2. It is the thinnest cranial nerve.
3. It is the only cranial nerve that emerges from the brain on the dorsal side.
4. It is the only cranial nerve that decussates completely.
5. Its nucleus supplies single extra ocular muscle i.e. superior oblique on the contral
lateral side.
6. Its fascicles are so small and so close to the nucleus that its not possible to differenti-
ate between a nuclear and a fascicular lesion.
7. It enters the orbit through the superior orbital fissure outside the circle of Zinn.
8. It innervates the superior oblique from the orbital surface.
The nucleus of the fourth nerve
The fourth nerve has a single nucleus on the either side of the mid line just caudal to the
third nerve nucleus in the grey matter of aqueduct at the level of inferior colliculus. The
nucleus is so close to the third nucleus that it seems to be continuous with it. The nucleus of
the fourth nerve innervates contra lateral superior oblique.
Fascicular part
The fibres of the fourth nerve bend round the aqueduct and decussate completely in the
anterior medullary velum and leave the brain stem on the dorsal surface. Thus fourth nerve is
the only cranial nerve that decussates fully and emerges on the dorsal side of the brain stem.
The fascicles are so small and so near the fourth nerve nucleus that it is impossible to
differentiate between nuclear and fascicular lesions. The lesions in the mid brain are best
called fasciculo nuclear lesion.
A small lesion dorsal to the aqueduct in the mid line produces bilateral fourth nerve
palsy.
The basilar part
The fourth nerve curves round the brain stem from behind to have an anterior route to
reach the superior oblique. In the basilar part its course is similar to that of third nerve. It
passes between the posterior cerebral and superior cerebellar arteries lateral to third nerve
away from the posterior communicating artery. Hence it is not involved in aneurysm of poste-
rior communicating artery which is a common cause of isolated third nerve palsy.
In the basilar part it does not come in relation to any other cranial nerve or nerve tract
hence lesion at this level produce isolated fourth nerve palsy. The commonest cause of involve-
ment of fourth nerve in the basilar part is head injury by which the nerve is pressed against
the free edge of tentorium. The same traumatic force when directed posteriorly may cause
bilateral fourth nerve palsy in the anterior medullary velum causing bilateral fourth nerve
palsy.
Cavernous part
After leaving the basilar part the fourth nerve pierces the dura to enter the cavernous
sinus lateral and inferior to the third nerve in the wall of the cavernous sinus. The fourth
nerve changes its course upwards in the anterior part of the cavernous sinus and becomes
superior to the third nerve to enter the orbit by the superior orbital fissure at a higher level
than third and sixth nerve outside the circle of Zinn.
The orbital part
The fourth nerve supplies the belly of the superior oblique from its orbital surface. The
third and sixth nerve enter the other extra ocular muscles from their bulbar surfaces.
The abducent nerve (abducens nerve)
16,18
The nucleus. The abducent or the sixth cranial nerve takes origin from its nucleus that
is situated in the mid pons under the floor of the fourth ventricle.
The fascicular part. The fascicles of the seventh nerve encircle the nucleus of the
sixth nerve at this level making it impossible for an isolated sixth nerve palsy due to a nuclear
lesion. The medial longitudinal fibres pass medial to the sixth nerve nucleus. There after
the fibres of the sixth nerve pass through the substance of the pons with superior-olivary-
nucleus on the lateral and pyramidical tract on the medial side.
Basilar part. Each abducent nerve comes out of the brain stem at the lower border of
the pons at the ponto medullary junction one centimeter from the mid-line. The nerve goes
up on the anterior face of the pons for a short distance only to be crossed by anterior inferior
cerebellar artery.
The sixth nerve pierces the dura 2 cm below the posterior clinoid process and passes
above the inferior petrosal sinus beneath the Grubers (petro clinoid) ligament to enter
the cavernous sinus.
The cavernous part
In the cavernous sinus unlike other cranial nerves that lie in the lateral wall of the
sinus the sixth nerve lies in the substance of the cavernous sinus between the internal carotid
and the wall of the sinus.
The orbital part
The sixth nerve enters the orbit through the superior orbital fissure within the circle
of Zinn. It supplies only the ipsilateral lateral rectus from its orbital side.
Clinical features of lesions at various levels of cranial nerves :
The lesions can be divided into :
1. Nuclear
2. Infra nuclear
(a) Fascicular
(b) Basilar
(c) Cavernous
(d) Orbital
Characteristics of oculomotor nerve palsy
20,21
Nuclear. Nuclear lesions are rarer than lesions at other locations. A nuclear lesion
should have following essential features :
1. Bilateral superior rectus palsy with bilateral ptosis.
2. Bilateral ptosis
3. Unilateral third nerve with contra lateral superior rectus and levator involvement.
4. Bilateral total third nerve palsy
5. Bilateral external ophthalmoplegia
6. Bilateral internal ophthalmoplegia.
The cause of such selective involvement lies in the fact that :
1. Each superior rectus is innervated by contra lateral third nerve nucleus.
2. Both leavtors are supplied by one central caudal nucleus.
3. Large lesion may involve all the nuclei and cause bilateral third nerve palsy.
Webino. Wall eyed bilateral inter nuclear ophthalmoplegia is associated with
bilateral exotropia with absent convergence due to involvement of both medial rectus sub
nuclei and medial longitudinal fascicular (not seen in children).
Inter nuclear ophthalmoplegia
22,23
is caused due to lesion of medial longitudinal
fasciculus (MLF) that block the impulse from contra lateral para median pontine reticular
formation (P.P.R.F.) to ipsilateral third nuclei. The lesion is in between the nucleus of third
and fourth nerve. Its clinical features consist of - diminished adduction, adduction nystagmus,
may have absent convergence (not seen in children).
One and half syndrome
22,23
There is a combination of horizontal gaze palsy on one side with inter nuclear
ophthalmoplegia (INO) due to lesion of P.P.R.F. and M.L.F. in the pons. Horizontal gaze
palsy constitute the One of the syndrome and INO is the remaining half, both together consti-
tuting one and half syndrome (Not seen in children).
Fascicular lesion
24
The fascicular lesions of third nerve are associated with involvement of either red
nucleus, cortico spinal tract or superior cerebellar peduncle depending upon the position
of the lesion. The various syndrome are :
1. Webers syndrome. Third nerve palsy on one side and contra lateral hemiplegia
due to lesion involving third nerve fascicle.
2. Claudes syndrome. Ipsilateral third nerve palsy with contra lateral ataxia/tremor
due to involvement of third nerve fascicle and the red nucleus.
3. Benedicts syndrome. Third nerve palsy with contra lateral hemiplegia and tremors
due to lesion involving third nerve fascicle, red nucleus and cortico spinal tract.
4. Nathnagels syndrome. Ipsilateral third nerve palsy, cerebellar ataxia on the
same side due to involvement of third nerve fascicle and superior cerebellar peduncle.
Basilar lesions
The basilar parts of the third nerve is in close relation with three vessels without any
other cranial nerve or tract in the vicinity. The basilar lesions are mostly vascular and isolated.
The third nerve trunk passes between the posterior cerebral and superior cerebellar
artery near the trunk of the basilar artery, hence the nerve may be compressed by aneurysm
of these vessels or that of posterior communicating artery, which runs parallel to the third
nerve trunk in the inter peduncular (basilar) part. These lesions generally are painful and
involve the pupil.
The basilar part of the third nerve is involved in uncal herniation at this level, which
can be brought about due to an extra dural hematoma that presses the nerve trunk over the
tentorial edge. It manifests as total third nerve palsy with fixed dilated pupil (Hutchinsons
pupil).
Traumatic lesion of third nerve
Traumatic paralysis of third nerve is less common than that of fourth nerve. It is mostly
seen in closed head injury. The common sites for involvement of third nerve in closed head
injury is extends between its exit from the ponto medullary junction to its entry in the cavern-
ous sinus. The nerve may be avulsed near the brain stem. It may be forced down over the
tentorial edge, there may be hemorrhage in the nerve, or there may be contusion of the nerve
during its passage in the inter peduncular space. Such lesions are generally associated with
fracture base of skull, loss of consciousness, total third nerve palsy and Hutchinsons pupil.
Cavernous sinus lesions
In the cavernous sinus, the third nerve lies in the lateral wall of the sinus with fourth
nerve and branches of the fifth nerve. The fourth nerve initially lies inferior to the third nerve
but before it leaves the sinus it takes an upward course. The sixth nerve lies in the substance
of the sinus. The oculosympathetic accompanies the internal carotid. Hence the lesion of the
cavernous sinus does not produce isolated lesion of any single nerve, it generally involves all
the nerves along with sympathetic. The lesions are generally partial. Involvement of fifth
nerve is the cause of pain along with ophthalmoplegia. In some instances there may be loss of
sensation on the distribution of fifth nerve. Generally pupil is of normal size. This is
explained on the basis of two diagonally opposite mechanism acting simultaneously. The
paralysis of third nerve should cause dilatation of pupil, which is counteracted by superimposed
miosis of associated Horners syndrome. Aberrant regeneration of third nerve is common.
Orbital lesions
The third nerve divides into two parts just before entering the orbit, the upper supplies
the levator, and superior rectus, the inferior supplies the inferior rectus and inferior oblique
and the medial rectus. It also contains parasympathetic fibres. The orbital lesions are gener-
ally partial, associated with involvement of fourth and sixth nerve. Sixth nerve under action is
more common than fourth nerve under action. It is generally associated with mild proptosis.
Pupil may be involved in lesions of lower division.
Painful ophthalmoplegia. Painful ophthalmoplegia may be confined to third nerve
palsy or with multiple nerve palsies.
The causes of painful third nerve palsy are :
1. Posterior communicating artery aneurysm.
2. Basilar artery aneurysm
3. Aneurysm of intra cavernous carotid
4. Diabetic oculomotor palsy
5. Ophthalmoplegic migraine
Painful ophthalmoplegia involving more than one nerve can be due to any
of the following :
1. Cavernous sinus thrombosis
2. Superior orbital fissure syndrome
3. Gradenigos syndrome
4. Pseudo tumours of the orbit
5. Metastatic tumours
6. Lymphoma
7. Naso pharyngeal carcinoma
8. Sinusitis
9. Para sellar growths
Tolosa Hunt syndrome
25
This is a chronic non specific granulomatous involvement of anterior cavernous sinus
and/or superior orbital fissure in isolation or combination. This is also called superior orbital
fissure syndrome or anterior cavernous sinus syndrome. A localised lesion may involve
the apex of the orbit and is called as orbital apex syndrome. One of the important features
of which is diminished vision due to involvement of optic nerve.
The Tolosa Hunt syndrome may start as an acute episode of unilateral boring pain with
diplopia and mild proptosis that may become chronic. Spontaneous recovery is known. Recur-
rence is common even with treatment.
The muscle involvement is varied, may begin with involvement of one muscle only to be
followed by involvement of other muscles. The ocular palsy is partial. Pupillary involvement
is variable. It may be normal, dilated or constricted depending upon the part of the third nerve
involved and its effect being counteracted by sympathetic system.
As the first division of the fifth nerve is affected, there may be hypothesia or anaesthe-
sia on the distribution of the fifth nerve.
ESR is always raised. The condition shows improvement following administration of
systemic steroids that has to be used for months. Most probable cause is an auto immune
disturbance resulting in formation of non specific granuloma of the orbit or cavernous sinus.
In adults it is generally unilateral but in children it may be bilateral.
The diagnosis is by exclusion supported by x-ray, CT, MRI, USG and fine needle biopsy.
Ophthalmoplegic migraine
It is a frequent but missed cause of headache in children. It could be the beginning of
migraine in adulthood. Generally there is history of migraine in the family. A typical
episode comprises of pain in or around the eye on one side. The eye is non congested, vision is
unaffected. There is associated nausea or actual vomiting. There is diplopia due to involvement
of extra ocular muscles. Commonest group of muscles to be involved are those supplied by the
third nerve. Other muscles may also be involved, the pupillary changes are not constant. The
headache generally subsides with onset of ophthalmoplegia. Ophthalmoplegia generally
subsides and clears with in one month, may not leave any residual sign or the recovery may be
partial. Recurrence is common. There are no other neurological signs. The optic nerve
is normal. Exact cause of the condition is not known. Most widely put forward cause is dilated
edematous internal carotid pressing the third nerve.
Cyclic oculomotor palsy
26
This is an unilateral condition most probably of congenital origin, birth trauma
may be a contributory factor. It may be noticed in early childhood.
There are two phases :
1. A paralytic phase consisting of partial third nerve palsy resulting in ptosis, mydriasis,
diminished accommodation, the eye is generally abducted.
2. Spastic phase. This begins with lid twitching, elevation of the lid, miosis and spasm
of accommodation and the eye may be adducted. The cycles last for less than a minute.
Exact cause is not known.
Aberrant regeneration of third nerve
27
The two cranial nerves that can develop aberrant degeneration (misdirection) are third
and seventh nerve. (The seventh nerve aberrant regeneration results in crocodile tear and
facial sweating on movement of the jaw.)
The oculomotor aberrant regeneration follows injury to the third nerve trauma, aneu-
rysm or neoplasia. There is abnormal sprouting of axons from the affected nerve that are
misdirected to areas for which they are not destined. It takes three to four months for aberrant
regeneration to take place. It is generally unilateral resulting into paradoxic movements.
There are three possible variations of paradoxic movements :
1. Elevation of lid on attempted movement of inferior rectus and medial rectus (Pseudo
Grafes sign).
2. Adduction or retraction of globe on attempted downwards or up gaze (Inverse Duanes
syndrome).
3. Light near dissociation on attempted convergence (Pseudo Argyll Robertson pupil).
There are two forms of aberrant regeneration of the third nerve :
1. Primary without previous history of acute oculomotor palsy. Third nerve weakness
develops gradually along with aberrant regeneration.
2. Secondary. Always follows acute non ischeamic oculomotor palsy during recovery.
The exact cause is not known. No medical treatment is known to be effective. Surgical
intervention should be attempted after minimum six months of onset of aberrant regeneration
that comprises of correction of ptosis and multiple muscle surgeries.
Double elevator palsy
28,29,30
This is a congenital anomaly of elevators of eye. It is a unilateral condition where
elevation of the eye is subnormal in abduction or adduction. Most probable cause is hypoplasia
of elevator nucleus. There is true or pseudo ptosis of affected eye. The eye is hypotropic in
primary position. Bells phenomenon is present. Hypo deviation may be manifest or latent.
Chin is elevated. Elevated chin with hypotropia means absence of amblyopia, which is other-
wise common.
There are three possible types of double elevator palsy
1. Prominent inferior rectus and normal superior rectus saccade
2. Weakness of elevation negative forced duction test, diminished superior rectus saccade.
3. Combination of both
Differential diagnosis consist of
1. Browns syndrome
2. Congenital fibrosis of inferior rectus
3. General fibrosis syndrome
4. Blow out fracture
5. Dysthyroid oculopathy (not seen in children)
6. Anomalous superior and inferior rectus muscle insertion.
Management
Surgery is the only definitive treatment indicated in large vertical deviation or abnormal
head posture. Surgery consists of recession of inferior rectus and transposition of lateral and
medial recti to superior rectus.
Double depressor palsy
This is rarer than former. It is a congenital anomaly that has paralysis of inferior rectus
and superior oblique in the same eye. In primary position the affected eye is hypertropic.
Complete third nerve palsy
The eye is abducted, slightly depressed and intorted. There is associated ptosis, there is
mild proptosis. All the muscles supplied by this nerve show no movement or very little
movement. The pupil is dilated not reacting to light and accommodation, the eye does not
converge. Common causes of complete third nerve palsy in children are head injury, and raised
intra cranial pressure.
Paralysis of individual muscles supplied by third nerve.
Superior rectus palsy
Superior rectus palsy is invariably associated with paralysis of levator palpebral superior.
Isolated superior rectus palsy is very rare. Common cause is congenital either in the muscle
or in its nerve supply. In primary position the affected eye is hypotropic. The Bells
phenomenon is absent. When the paretic eye fixes the sound eye becomes hypertropic due
to over action of inferior oblique of the sound eye. The chin is elevated and head is tilted
towards the sound side.
Medial rectus palsy
Isolated medial rectus palsy is very uncommon. The paretic eye is abducted due to
unopposed action of lateral rectus on the same side. There is no change in position of chin or
tilt of the head.
Inferior rectus palsy
Commonest cause of this rare palsy is congenital. Commonest acquired cause is fracture
floor of the orbit. The depression of the involved eye is restricted along with the superior
oblique of the contra lateral eye. The ipsilateral superior rectus is over active. The chin is
depressed, the face is turned to the same side.
Inferior oblique palsy
The condition is rare. The affected eye is hypotropic in primary position. When fixation
is shifted to sound eye, it becomes hypertropic. When the eyes are moved up and out, there is
restriction of conjugate movement.
Characteristics of fourth nerve palsy
Fourth nerve under action is a common cause of vertical squint and compensatory head
posture. It is however less common than third or sixth nerve palsy in children. In children two
major causes of fourth nerve palsy are congenital anomaly and trauma. However infection,
inflammation and neoplasm have their share towards fourth nerve palsy. Ischaemic lesion
like diabetes, hypertension, arterio sclerosis which are major causes of trochlear nerve palsy
in adults are not seen in children.
Congenital fourth nerve palsy
It may not be obvious unless it is severe, which presents as hypertropia in primary
position. In moderate form the child may not complain of diplopia but may have compensatory
head posture which gradually disappears when the palsy become concomitant, which is common.
Hence old photographs may show abnormal head posture i.e. head tilted to the opposite
shoulder. The anomaly may be seen in other members of the family. An old family photograph
may show abnormal head posture in other members of the family as well. The child may read
with one eye closed to avoid diplopia. Facial asymmetry is common, the antagonist may show
contracture, difference between primary and secondary deviation is absent due to spread of
comitance. There may be suppression in the affected eye that nullifies diplopia. Other
neurological defects are generally not seen.
Effect of congenital palsy :
1. The palsy may be constant at birth and remain so.
2. The squint is small and the child may control it without compensatory head posture.
3. The squint manifests in particular gaze with binocular vision.
4. It may develop latent squint.
5. It may develop intermittent squint.
6. Intermittent squint becomes constant
Unilateral fourth nerve palsy
This is more common than bilateral. It can be congenital or acquired. It presents as
ipsilateral hypertropia in primary position that increases when the eye is moved medially and
increases more when the head is tilted on the same side (Park-Bielschowskys test). The
above features are true for recent fourth nerve palsy where the child may complain of vertical
diplopia and parent notice abnormal head posture. With passage of time there is spread of
comitance and diplopia is minimised or abolished due to suppression. Associated exotropia is
best measured by double Maddox rod test or Bagolinies striated glass
32
. It can be
demonstrated on synoptophore with torsion slides. Exccyclotropia due to uniocular superior
oblique palsy is about 7.
The child may tolerate up to 4 of excyclotropia. Occasionally the contra lateral eye may
show hypotropia when the child fixes with the paralysed eye.
Acquired superior oblique palsy should be differentiated from congenital palsy because
an acquired palsy may be associated with other neurological findings. Second important point
to remember is that the superior oblique may be absent in congenital palsy, which becomes
obvious only during surgery.
Bilateral fourth nerve palsy
Recent unilateral superior oblique palsy is less difficult to diagnose than bilateral palsy.
Trauma is a major cause of bilateral palsy than congenital anomaly which is otherwise an
equally important cause of bilateral superior oblique palsy. Asymmetry in signs is common in
bilateral palsy. In symmetrical bilateral case, there is either no vertical deviation or very little
vertical deviation in primary position.
The child may be orthophoric or show mild exo or eso deviation. The esotropic eye
generally shows V pattern. There is left hypertropia on right gaze and right hypertropia on left
gaze.
Excyclotropia may be as much as 25-30. It increases in down gaze.
Diplopia is present in all position of gaze except up gaze.
Bielschowsky head tilt test is positive on both sides.
Occasionally the cases may present as unilateral palsy, may develop highly asymmetric
palsy in the other eye.
Both the contra lateral synergists i.e. both inferior recti show over action.
Asymmetric palsy develops ocular torticolis more often than symmetric palsy.
Neurological characteristics of fourth nerve palsy
Traditionally all cranial nerve palsies have been put into nuclear, fascicular, basilar,
cavernous and orbital types except the fourth nerve because
1. The fourth nerve nucleus and its fascicles are so close to each other making it impossible
to differentiate between nuclear and fascicular vision.
2. There are no structure in the viscinity that may cause other neurological signs as
seen with third and sixth nerve.
3. Only structure that passes through the mid brain adjacent to fourth nerve fascicles is
ocular sympathetic. The lesions are best put as nuclear-fascicular lesion.
34
Nuclear-fascicular lesion produce contra lateral superior oblique palsy, may be
associated with contra lateral Horners syndrome. A lesion in the anterior medullary velum
will involve both the fourth nerves causing bilateral superior oblique palsy. In children
commonest cause of which is head injury.
The basilar part of the fourth nerve winds round the brain stem and passes between
the posterior cerebral and superior cerebellar arteries lateral to the third nerve and then
passes forward to enter the cavernous sinus. At this location it gets involved in head injury.
The nerve is pressed against the tentorium and damaged causing palsy of superior oblique on
the same side without any other localising sign.
Cavernous sinus lesion
The fourth nerve is generally involved along with third and sixth nerve in various
combinations along with fifth and oculo sympathetic in the cavernous sinus.
Orbital lesionsOrbital lesions can be of two types :
1. Involving the nerve along with third and sixth nerve.
2. Injury to the muscle itself, or trochlea of the muscle. Such injuries involves the orbital
rim in the superio medial aspect.
Causes of fourth nerve palsy in children
In adults the cause of fourth nerve palsy are mostly vascular. They produce ischaemia,
bleeding or compression by aneurysm. Other cause is neoplasm. In contrast to this, the causes
in children are rarely vascular. They are mostly congenital, traumatic, infective. In one
third cases no cause can be found out.
Other causes of vertical squint
Vertical squints are less common than horizontal squint. Small degree vertical squint
may be missed due to development of compensatory head posture. However in later life there
may be decompensation and the squint may manifest with diplopia and obvious deviation.
There are some conditions that may look vertical deviation when actual deviation is
absent. The binocular vision is intact. These are called pseudo vertical squints.
The causes of pseudo vertical squints are :
1. Asymmetry of orbit.
2. Cranio facial dysostosis
3. Orbital growth pushing the globe vertically.
5. Ectopic macula
6. Vertical angle kappa.
The true vertical squint can be latent or manifest. It can be concomitant or paralytic.
In paralytic vertical squint, spread of comitance is common.
Causes of non comitant vertical squint
These are due to involvement of cyclovertical muscles. Involvement of muscle may
be restricted to one muscle in one eye, to involvement of multiple muscles in both eyes in
various combinations. The non concomitant vertical squint can be neurological or restric-
tive. The neurological causes may be :
Supra nuclear
Nuclear
Infra nuclear
The supra nuclear lesions are :
Vertical gaze palsy
Skew deviation
Double elevator palsy
Parinauds syndrome
The nuclear and infra-nuclear lesion have been discussed in lesions of third, fourth and
sixth nerve.
The other lesions are :
Dissociated vertical deviation
Brown syndrome
Inferior oblique over action
Superior oblique over action
Inferior rectus muscle paresis
Superior oblique myokymia
The restrictive causes of vertical squint are :
1. Fracture floor of the orbit.
2. Myasthenia gravis
3. Dysthyroid ophthalmopathy
5. Progressive external ophthalmoplegia
Vertical gaze palsy
35
The vertical gaze movements are under bilateral cerebral and brain stem centres.
Transient tonic vertical deviation downwards is seen in neonates which does not indicate
any neurological defect and does not require investigation. Another vertical gaze palsy seen in
children is setting sun sign in infantile hydrocephalus. This is associated with retraction of
the lid. The condition is serious but reversible and is treated by ventricular decompression
by standard neurosurgical procedure.
The condition consists of inability to move the eye vertically up or down. There are
three types of vertical gaze palsies :
1. The most common, inability in up gaze
2. Difficulty in both up and down gaze
3. Least common difficulty in down gaze.
The condition may be associated with light near dissociation, rigid pupil correctopia,
dyscoria, spasm of accommodation, lid retraction or ptosis, convergence palsy, vertical
nystagmus.
Common causes of vertical gaze palsy in children are : Stenosis of aqueduct,
hydrocephalus. Tumors of the third ventricle, mid brain and pineal body.
35
Skew deviation
36,37
In this supra nuclear defect, there is divergence of the eyes in vertical plane which is
large. It may vary in different position of gaze. The deviation is more in uniocular lesion. There
is no restriction on forced duction test. No single muscle could be pin pointed as cause of skew
deviation on Park Bielschowsky three step procedure. The condition may be associated with
inter nuclear ophthalmoplegia. Commonest cause is a posterior fossa lesion that is con-
firmed by CT and MRI. Other causes are vestibular or cerebellar. It may develop in congenital
esotropia for which no cause is detectable. The pathology is generally ipsilateral to the in-
volved eye.
Parinauds syndrome
This is an acquired lesion. It is bilateral, there is paresis of up gaze. In an attempt to
look up, the eyes converge. The pupils are semi dilated, there is light near dissociation.
There may be lid retraction on down gaze. This is seen more commonly in children with
setting sun sign of hydrocephalus. Other common cause is tumor of pineal body.
Dissociated vertical deviation (DVD)
42
Dissociated vertical deviation is a neuro-muscular anomaly of obscure nature. It is
known by many other names also. Common among them are : dissociated hyper deviation,
alternate sursum-duction, alternate hyper phoria.
38
As the last term indicates, it is basically a latent squint that manifests under cover,
however, in some instances when the child is tired or day dreaming, one of the eyes may
deviate upwards.
It is a binocular disorder with variable asymmetry in two eyes. The difference may be
so much that one eye seems to divert all the time and the other looks normal.
It can be an isolated feature without any squint. Its association with strabismus is more
frequent than without. When it presents as manifest deviation, it can be intermittent or
constant. It can be seen with any types of squint but commonest squint associated with
dissociated vertical deviation is infantile esotropia. It is more common with non
accommodative esotropia. It is rare if squint is acquired after eighteen months
39
. In case of
infantile squint, it takes about two years for DVD to set in. It is seen in about 40-50% of cases
of infantile esotropia. Another group of children who develop DVD are those who have undergone
successful surgical correction for infantile esotropia. In such cases the anomaly develops two
years following initial surgery. It is better if the parents are informed about its possibility at
the time of first surgery.
The other common features are :
1. Development of manifest nystagmus that may become latent.
2. It is possible to have binocular single vision with DVD.
3. Extorsion of the deviated eye.
4. The eye intorts as the child looks down.
5. Cover test. The eye under cover deviates slowly up. The drift is more on prolonged
cover. It is more for distant than near. Once the cover is removed, the eye gradually
comes down and may over shoot the normal position and become hypotropic.
6. Measurement of deviation is made by using base down prism in front of the deviat-
ing eye. The occluder is shifted to the other eye, which starts deviating and base
down prism is added to measure the deviation.
7. The Bielschowsky phenomenon.
Besides DVD, the other condition where an eye deviates upwards under cover is
alternation hyper phoria. The two conditions are differentiated by positive Bielschowsky
phenomenon or Dark wedge test.
Any of the eyes is occuluded by a translucent occluder (Spielmann occluder) through
which the movement of the eye can be seen behind the occluder. The other eye fixes a spot light
at distance. A Sbiza bar is placed before the fixing eye and density of the filter is gradually
increased. The eye under Spielmann occluder is noticed to drift down and pass below the
mid line if DVD is present. Now the filter density is gradually reduced and the eye under cover
is seen to elevate once again. This phenomenon is absent in alternating hypertropia.
Sbiza bar (Bagolini filter). This instrument consist of a series of red filters of the
density (photo metric neutral density filter). The end has lightest red colour, the other end has
darkest colour. The instrument is generally used to measure depth of suppression in amblyopia
for which the test is done at one third of a meter in contrast to Bielschiwsky phenomenon
where the test is done at six metres. The bar is placed before the fixing eye in increasing
density until diplopia develops. The strength of the density gives the depth of suppression
40,41
.
8. DVD may be associated with A and V pattern.
9. There may be associated oblique muscle over action.
Differential diagnosis consists of all conditions that cause upward shift of eye in pri-
mary position. They are :
1. Alternate hyper phoria
2. Inferior oblique over action
3. Superior oblique over action
4. Inferior rectus palsy
5. Skew deviation
Treatment
Treatment is correction of error of refraction and amblyopia when present. Surgery is
indicated if vertical deviation occurs spontaneously so frequently that it causes cosmetic blemish.
The alternatives available are :
I. Weakening of superior rectus
1. Recession of superior rectus 7-8 mm
2. Retro equatorial myopexy (Faden operation).
II. Strengthening of inferior rectus (resection).
III. Total anterio positioning of inferior oblique.
Inferior oblique over action
Inferior oblique over action is common in infantile esotropia where it develops be-
tween one to six years of age. However it may develop in exotropia less commonly. Gener-
ally there is no hypertropia in primary gaze.
Hyper deviation is best seen in adduction. Secondary hypertropia is seen in under action
of antagonist superior oblique. There may be pseudo paresis of contra lateral superior rectus.
Associated V phenomenon is common. Latent nystagmus is frequent. Negative Bielschowsky
phenomenon differentiates it from DVD. Cause may be mechanical or innervational.
Treatment consists of weakening of inferior oblique either by recession or myectomy.
Superior oblique over action
Superior oblique over action can be primary or secondary. It can be unilateral or
bilateral. In bilateral cases it can be asymmetric enough to seem to be unilateral. All bilateral
cases are primary. In unilateral cases, a vertical deviation is common in primary position. The
side of over action causes hypotropia. It may be associated with A pattern. In case of bilateral
over action of superior oblique bilateral, weakening of SO is indicated.
Inferior rectus muscles palsy
Palsy of inferior rectus is rare. It can be congenital due to mal-development of the
muscle or its faulty insertion. Trauma is more common. Commonest type of injury is fracture
floor of the orbit with or without entrapment of the muscle. The muscle may be injured at the
time of original injury or may be accidentally injured during repair of fracture floor of the
orbit. If the muscle is not entrapped, the eye become hypertropic in primary position. In
entrapment of the muscle the eyeball may fail to elevate and hypotropia results. It is commonly
associated with reduced down and out movement due to inter action of contra lateral superior
oblique. The compensatory head posture comprises of face turned towards the paretic side,
chin depressed and head tilted on the same side.
Treatment consists of strengthening the muscle by resection of involved muscle and
recession of ipsilateral superior rectus.
Brown syndrome (superior oblique sheath syndrome) :
Brown syndrome is one of the common conditions of abnormal head posture in children.
It is mostly congenital but can be acquired, it may be intermittent or constant. It is
bilateral in 10% of cases. Congenital Brown syndrome may spontaneously resolve over years.
Post inflammatory Browns syndrome also resolve with treatment.
Probable congenital causes of Brown syndrome are :
1. Short anterior sheath of superior oblique.
2. Inelastic superior oblique tendon
3. Defective innervation of both the obliques.
4. Thickened muscle not moving freely over the trochlea.
5. Anomalous insertion of superior oblique.
The acquired causes are
1. Trauma. Trauma, accidental or surgical is the commonest acquired cause of Brown
syndrome. The traumas that result in the syndrome are - direct trauma to the trochlea either
blunt or penetration. There may be post traumatic scar formation round the trochlea, hematoma
or blood cyst formation in the muscle. Orbital floor fracture with entrapment of inferior ob-
lique muscle.
The surgical traumas responsible areexcessive tucking of superior oblique, ptosis
correction, injury to superior oblique or entrapment during scleral buckling.
The next common cause is rheumatoid conditions involving the trochlea. Frontal sinusitis
and frontal osteoma may also cause the disorder.
The presenting symptoms and signs consists of -
In mild cases, the disorders may go unnoticed as there is no deviation in primary position.
Otherwise sometimes parent notice abnormal head posture and defective eye movements. Vision
may be diminished and amblyopia is common if squint is present.
The chin is slightly elevated, the head is tilted towards the affected side and face turned
away from the affected side.
On cover test mild to moderate latent hypo deviation may be noticed.
The affected eye fails to elevate is adduction. There may be down shoot of the eye in
adduction and there may be widening of the inter palpebral fissure in attempted adduction.
Elevation capacity increases as the eye is abducted. There is over action of contra lat-
eral synergist. AV phenomenon is common when squint is manifest. There is positive forced
duction test.
Browns syndrome has been put in three grades i.e. mild, moderate and severe.
In mild form, there is no hypotropia in primary gaze, there is no down shoot on adduc-
tion.
Moderate form also does not show hypotropia in primary gaze but down shoot is dem-
onstrated on adduction.
Severe form has hypotropia in primary gaze, down shoot in adduction and abnormal
head posture.
The mild and moderate forms go unnoticed as there is no manifest squint, minimal
abnormal head posture. They constitute about sixty percent of all cases.
Binocular single vision is present in primary position and lower field.
Management
Any error of refraction is treated following refraction under cycloplegia. Amblyopia when
present should be treated vigorously.
Prisms help only in small degree deviations.
Steroids may be needed if there is element of rheumatoid disease.
Surgery is rarely indicated as many show - spontaneous improvement, no manifest stra-
bismus and minimum head tilt.
Surgery is indicated in
Diplopia in primary position,
Marked abnormal head posture,
Decompensation of squint
Surgery is not very rewarding as it rarely corrects the ocular rotation. Surgery consist
of stripping of superior oblique tendon sheath, removal of adhesion round the trochlea and
recession of contra lateral superior rectus.
Superior oblique myokymia
This is an ocular dyskinesia not seen in children. The exact cause is not known, there
is periodic vertical and torsional oscillopsia each lasting for few minutes.
There is no definite treatment for the condition.
Characteristics of abducent nerve palsy
42,43
The sixth nerve supplies only lateral rectus on ipsilateral side. The lateral rectus is
involved in one horizontal movement i.e. abduction.
The sixth nerve has a long intra cranial course second only to fourth nerve. Lesions
extending from its nucleus to its end in the muscle may involve lateral rectus. There are two
types of lesions of sixth nerve i.e. those
(1) That have other neurological signs in pons and
(2) Those that cause isolated sixth nerve palsy.
Isolated sixth nerve palsies are non specific and non localising. An isolated
sixth nerve palsy is never nuclear.
The signs and symptoms of isolated sixth nerve palsy produce :
1. Convergent squint.
2. Head turned towards the affected muscle.
3. No change in position of chin or tilting of head.
4. There is limitation of lateral movement.
5. Homonomous horizontal diplopia that becomes worse on looking towards the involved
side.
6. Bilateral paralysis of sixth nerve produces diplopia in all gazes.
7. Paralysis of lateral rectus is frequent in children. It may be unilateral or bilateral.
Common causes of sixth nerve palsy in children are
Congenital
Trauma
Infection
Inflammation
Demyelination
Vascular lesions like haemorrhage, aneursym, ischaemia are rare in children but are
major cause of sixth nerve palsy in adults.
Neoplasm of mid brain are also major causes of sixth nerve palsy in children.
Majority of congenital cases are bilateral. The common congenital causes are :
1. Developmental anomaly of lateral rectus
2. Maternal infection
3. Birth trauma (common)
4. Hereditary.
In new borns, a transient lateral rectus palsy due to viral infection is seen occasionally
developing one to three weeks after non specific upper respiratory tract infection. Isolated
sixth nerve palsy in children without neurological features of headache, vomiting, papilledema,
ataxia is caused due to :
1. Middle ear infection
2. Post viral status
3. Sinusitis
4. Raised intra cranial pressure
Topographic location of sixth nerve lesion
Nuclear lesion
An isolated sixth nerve palsy can never be nuclear.
A nuclear lesion is associated with horizontal gaze palsy towards the side of the lesion
due to involvement of pontine paramedian reticular formation. Involvement of seventh nerve
loop round the sixth nerve nucleus causes ipsilateral facial palsy. Involvement of medial
longitudinal fascicles cause ipsilateral inter nuclear ophthalmoplegia. Involvement of oculo
sympathetic will cause ipsilateral Horners syndrome.
Fascicular lesion (Brain stem lesion or anterior inferior cerebellar artery syndrome) :
This can cause three types of lesions :
1. Dorsal pons lesionMillard Gubler syndrome
2. Dorso lateral pons lesionFovilles syndrome
3. Combination of the twoRaymonds syndrome.
Millard Gubellers syndrome consists of
1. Sixth nerve palsy
2. Ipsilateral seventh nerve palsy.
3. Contra lateral hemi paresis due to involvement of pyramidal tract.
Fovilles syndrome consists of :
1. Horizontal conjugate gaze palsy due to involvement of P.P.R.F.
2. Ipsilateral fifth, seventh and eighth nerve palsy
3. Ipsilateral Horners syndrome.
Raymonds syndrome consists of :
1. Ipsilateral sixth nerve palsy
2. Contra lateral hemiparesis.
The above lesions are generally seen in elderly persons with micro vascular lesion of
diabetes, hypertension, athero sclerosis and not in children. Moreover such patients are seriously
ill and first seen by neuro physician.
Basillar lesion
The three common causes of sixth nerve palsy in children at this level are :
1. Raised intra cranial pressure
2. Gradenigo syndrome
3. Trauma
Other causes like acoustic neuroma and nasopharyngeal tumors are seen in elders.
1. Raised intra cranial pressure. Raised intra cranial pressure pushes the brain
stem down pulling the sixth nerve on each side. It stretches the sixth nerve in the Dorellos
canal over the apex of the petrous bone. This produces bilateral sixth nerve palsy without any
localising sign. The causes are posterior fossa tumor and pseudo tumor ceribri.
Gradenigos syndrome -
This is a common cause of unilateral sixth nerve palsy in children in developing coun-
tries. This is secondary to otitis media which leads to infection of petrous bone and extra
dural abscess formation that comes in close proximity of the sixth nerve.
The syndrome consists of sixth nerve palsy, facial palsy, diminished hearing, facial pain
in the distribution of first division of fifth nerve.
Battles sign. This consists of bilateral sixth and seventh nerve palsy, bleeding or leak
of CSF from external ear and ecchymosis over the mastoid. The cause of the condition is closed
head injury. The skull is compressed in horizontal diameter, there is transverse fracture of the
temporal bone or base of the skull.
Differential diagnosis of sixth nerve palsy
Differential diagnosis of sixth nerve palsy consists of many unrelated non neurological
conditions that produce more or less degree of mechanical restriction of lateral rectus. The
conditions can be congenital or acquired. They are :
1. Mobius syndrome
2. Duanes retraction syndrome
3. Fractured medial wall of the orbit
4. Strabismus fixus
6. Alternate day esotropia
7. Conjunctival shortening syndrome
8. Spasm of near reflex
9. Break in fusion of congenital esophoria
10. Infantile esotropia
11. Nystagmus blockage syndrome
Besides these myasthenia and thyroid myopathy are two systemic conditions that may
masquerade any motor palsy.
Mobius syndrome
44,45
This is a multi systemic congenital anomaly where bilateral sixth nerve palsy with
bilateral seventh nerve palsy are the two constant features. The Bells phenomenon is retained.
The eyes can not be moved in horizontal direction, both duction and version are equally affected.
Vertical movements are retained. There are no pupillary changes. No vestibular nystagmus
can be demonstrated by caloric or rotatory tests. Occasionally there may be esotropia. Rarely
there may be exotropia, A and V pattern is common. Involvement of other nerves from fifth to
twelfth have been reported.
45
Most probable cause is aplasia or hypoplasia of nuclei of various
cranial nerves.
Other associated deformities are - Deafness, deformed tongue, dental defects, skeletal
defects in hands, feet. General hypotonia of muscles, especially pectoral muscle, variable men-
tal retardation.
Management
Associated error of refraction and amblyopia when present should be treated by standard
method.
Lateral tarsorrhaphy may be required in presence of seventh nerve palsy.
Results of squint surgery are not very good. Maximum recession of medial rectus may
be done.
Duanes retraction syndrome
Duanes retraction syndrome is a common motility disorders seen in children. The
condition is congenital and sporadic. Though it is present at birth, it is generally not diagnosed
before three years of age. The child is generally brought for squint or abnormal movements of
the eye and lids. It is generally unilateral but can be bilateral. When unilateral the left eye is
affected more often than right. It is more common in girls.
The essential features are :
Narrowing of the inter palpebral fissure on adduction.
Widening of IPA on abduction.
Retraction of globe on adduction.
Upshoot of the eyeball on adduction.
The eye may be orthophoric, esotropic or exotropic. A-V pattern is common. There are
many classification of the condition based on clinical presentation and electro myographic
findings.
Clinically there are three types :
1. Narrowing of IPA and retraction of globe with limited abduction, this is the commonest
clinical type.
2. Other things remaining same as in type I, there is limited adduction.
3. Both adduction and abduction are limited. This is the rarest type.
The exact cause of Duanes retraction syndrome is not known. Many congenital anomalies
have been blamed for the condition alone or in combination. They are : 1. Mechanical, 2. Neural
The mechanical factors are :
1. Fibrotic, thin, non elastic lateral rectus
2. Relatively posterior insertion of medial rectus
3. Tight medial rectus acting as band
4. Abnormal attachment of lateral rectus
5. Muscles bound to the orbital wall
6. Abnormal attachment of medial rectus at its origin near the orbital apex
7. Fibrosis or atrophy of lateral rectus
The neural anomalies can be :
1. Absent sixth nerve nucleus
2. Poorly formed abducent trunk
3. The lateral rectus is partially supplied by third nerve
4. The lateral rectus is supplied by third nerve
5. Simultaneous contraction of medial and lateral rectus in an attempt to adduct with
retraction of globe
The other classification is based on electro physiological changes in extra ocular
muscle
48
1. Paradoxical innervation of lateral rectus getting maximum impulses on adduction
and diminished impulse abduction. This is the commonest type of Duanes retraction
syndrome.
2. Lateral rectus gets maximum impulse on abduction with normal medial rectus.
3. Innervation of both lateral or medial rectus in primary as well as adduction or abduc-
tion.
Duanes retraction syndrome may be just an ocular anomaly which is very common.
Involvement of other systems are not rare.
The ocular changes commonly seen with Duanes retraction syndrome are
microphthalmos, lid defects, crocodile tear, heterochromia of iris, lenticular opacities, dermoids,
coloboma of choroid.
The auricular changes include - changes in external ear, external auditory meatus
and defects in vestibular canals.
The skeletal system changes consists of changes in palate and vertebrae, hand and
feet.
The neural changes - mal-development of third, fourth and sixth nerve.
Variants of Duanes retraction syndrome
47
1. The retraction of globe is so minimal that the condition is called Duanes retraction
syndrome without retraction.
2. Similarly there may be very little narrowing of palpebral aperture.
3. There may be retraction of upper lid.
4. There may be hypotropia or hypertropia in primary position.
The term pseudo retraction syndrome is used to denote entrapment of medial rectus
in fracture of medial orbital wall. There is retraction of globe in attempted abduction.
Management
Management consist of correction of any error of refraction when present, prevention
and treatment of amblyopia. Some children have no visible change in primary position and
maintain good binocularity with slight change in head posture.
Surgery
There are many types of surgeries performed to treat the condition. Each case seems to
be requiring separate method. Multiple bilateral surgeries are required to strengthen the lat-
eral rectus. Faden procedure may be used to correct abnormal head posture. This prevents up
and down shoot with stabilising effect on horizontal muscles.
Commonest type of surgery advocated is bilateral medial rectus recession.
Strabismus fixus
This is a congenital condition of large bilateral esotropia due to anchoring of extra
ocular muscles. The medial rectus is inelastic and replaced by a fibrous band, which is shorter
than usual length of medial rectus. The muscle is inserted in a larger than normal area.
Occasionally the lateral rectus and vertical muscles may also be affected. The eye is fixed in
adduction. Forced duction test is positive. In an attempt to adduct, the globe retracts.
General fibrosis syndrome is more severe form of strabismus fixus. All the extra
ocular muscles are fibrosed. There may be adhesion between Tenons capsule and the globe.
Alternate day esotropia (Intermittent cyclic esotropia)
This is an unusual form of squint that has rhythmic cycle of esotropia for twenty
four hours followed by orthophoria for next twenty four hours. Making the squint to manifest
every alternate day, such rhythm cycle may last for six months to one year, by then the esotropia
becomes constant. Treatment is required when esotropia becomes constant and most com-
monly practiced surgery is bimedical recession.
Conjunctival shortening syndrome
This form of restrictive tropia is seen when conjunctiva is kept contracted for months as
is seen in third nerve palsy, sixth nerve palsy, long standing concomitant squint or following
trauma, surgery where conjunctiva has not been recessed well at the time of muscle surgery.
The condition is also met with in thyroid eye disease.
There is limited ocular movement, forced duction test is positive.
Progressive external ophthalmoplegia
This is a rare chronic multi systemic disorder generally seen in second to fourth
decade. Infantile and juvenile are also known.
48
The exact cause of the disease is not known.
It is said to be associated with micro chondrian mutation.
49
Others consider it to be an
abiotrophy.
It is a bilateral progressive disease that beigns with bilateral ptosis which is gener-
ally equal on both sides, gradually other extra ocular muscles also get involved. There is no
fix pattern of involvement of the muscles, however, down gaze is unaffected for long time.
At the end all the muscles loose function and the eye has a fixed position. The orbularis may
also be involved. The iris and ciliary body are spared.
KearnsSayer syndrome is a triad of external ophthalmoplegia, retinal pigment
degeneration and complete heart block.
Ophthalmploegia plus generally develops in childhood, there is no family history,
CSF protein is elevated. The children have short stature, may have subnormal intelligence
and hypogonadism.
The condition has to be differentiate from myasthenia, thyroid eye disease, orbital pseudo
tumor, myotonic dystrophy, progressive supra nuclear palsy.
There is no known specific treatment.
Differential diagnosis of abducent palsy in children
Diagnosis of recent lateral palsy is not difficult. However, occasionally the child with
esotropia may fail to abduct the involved eye in conditions other than sixth nerve palsy i.e.
infantile esotropia. In such situation of simulated sixth nerve palsy it is essential to find out if
the lateral rectus in question is really paralytic. The best way to demonstrate action of lateral
rectus in such conditions is to patch the straight eye for few hours. This will move the eye
laterally in cases of pseudo paralysis of lateral rectus but not in paralysed lateral rectus. The
only exception is entrapment of medial rectus in fracture of the medial wall of the orbit, which
is confirmed by forced duction test. The child with lateral rectus palsy has his face turned
towards paralysed muscle to avoid diplopia. The other advantage of face turning is increased
temporal field on the side of the paralysed lateral rectus. The child with prolonged lateral
rectus palsy may cross fix with the paralysed eye like in infantile esotropia. The other test is
Dolls eye movement which is negative in lateral rectus palsy.
Other conditions that simulate like lateral rectus palsy areDuannes retraction
syndrome, Mobieus syndrome, congenital fibrosis syndrome, pseudo tumor orbit, myasthenia.
Investigation generally done to clinch the diagnosis of lateral rectus areUltrasonography of
the orbit, CT, MRI, in cases of suspected myasthenia Tensilon test is positive. Forced duction
test helps in finding out entrapment of the muscle and fibrosis syndrome.
Long term effect of extra ocular muscle palsy
1. Presentation of acute ocular palsy greatly differ from chronic palsies
2. Some cases may present with palsy of one particular muscle only to involve other
muscles later. This happens frequently in thyroid eye disease and myasthenia.
3. Following are the changes that are seen in other muscle/muscles when one muscle is
involved :
(a) Over action of ipsilateral antagonist
(b) On long term, the ipsilateral antagonist may end up in contracture
(c) Secondary inhibition of contra lateral antagonist (under action)
(d) By the time secondary changes (sequel) take place, the other muscles get prima-
rily involved.
(e) Compensatory head posture - The child may overcome diplopia by keeping the
head in abnormal position.
(f) Suppression and amblyopia are common in children with manifest squint.
REFERENCE8
1. Lyle K.T. and Wybar K.C. ; The extrinsic ocular muscles and related fascia in Lyle and
Jacksons Practical Orthoptics in treatment of squint. First Indian edition. p-8
2. Rowe Fiona ; Extra ocular muscles anomaly and innervation in Clinical orthoptics.
First edition. p-312, Blackwell Science Limited, London, 1997.
3. Rosenberg, M.A. ; Ocular motor system in Principle and practice of ophthalmology.
Vol. III, edited by Peyman G.A., Sander D.R. and Goldberg M.F. First Indian edition.
4. Virginia Carlson Hansen ; Extra ocular muscles anatomy and function in Ocular
motility. First Indian edition. p-111, Jay Pee Brothers, New Delhi, 1989.
5. Datta H. ; Anatomy of extra ocular muscle and their fascia in Strabismus. First edition.
6. Sharma, P. ; An overview of strabismology in strabismus simplified. First edition.
p-18, Modern Publishers, New Delhi, 1989.
7. Glasser, J.S. ; Eye movements characteristics and recording techniques in
Neurophthalmology. p-187198, Harper and Row, London, 1978.
8. Bajandas, F.J., Kline, L.B. ; Supra nuclear and inter nuclear gaze pathway in
Neurophthalmology review manual. Third edition. p-4365, Jay Pee Brothers, New
Delhi, 1989.
9. Duke Elder S. and Wybar, K.C. ; Extrinsic muscles of the eye in System of
ophthalmology. Vol. II. First edition. p-414463, Henry Kimpton, London, 1961.
10. Nema, H.V. ; Extra ocular muscles in Anatomy of the eye and its adnexa. Second
edition, p-7784, Jay Pee Brothers, New Delhi, 1991.
11. Banumathy, S.P. ; Muscle in orbit in Anatomy of the eye. Ist edition. p-2332, Arvind
eye hospital, Madurai.
12. Duke Elder S. and Cook, C. ; Extrinsic ocular muscles in System of ophthalmology.
Vol. III, part I, p-220230, Henry Kimpton, London, 1969.
13. Nema, H.V. ; Development of the eye and its adnexa in Anatomy of the eye and its
adnexa. Second edition. p-139142, Jay Pee Brothers, New Delhi, 1991.
14. Banumathy, S.P. ; Development of extrinsic muscles of orbit in Anatomy of the eye.
First edition. p-167, Arvind eye hospital, Maduri.
15. Nema, H.V. ; The oculomotor nerve in Anatomy of the eye and its adnexa. Second
16. Duke Elder S., Wybar K.C. ; Efferent ocular motor pathways in System of ophthal-
mology. Vol. II. p-701718, Henry Kimpton, London, 1961.
17. Banumathy, S.P. ; Trochlear nerve in Anatomy of the eye. p-115118, Arvind eye
hospital, Maduri.
18. Banumathy, S.P. ; Abducent nerve in Anatomy of the eye. p-119123, Arvind eye
hospital, Maduri.
19. Nema, H.V. ; The abducent nerve in Anatomy of the eye and its adnexa. Second
20. Glasser, J.S. ; Oculomotor palsies in Neurophhalmology. p-255260, Harper and Row,
London, 1978.
21. Bajandas, F.J., Kline L.B. ; Seven syndromes of third nerve in Neurophthalmology
review manual. Third edition. p-8794, Jay Pee Brothers, New Delhi, 1989.
22. Bajandas, F.J. and Kline L.B. ; One and half syndrome in Neurophthalmology re-
view manual. Third edition. p-6364, Jay Pee Brothers, New Delhi, 1989.
23. Rowe, Fiona ; Inter nuclear and supra nuclear disorders in Clinical orthoptics. First
edition. p-238244, Blackwell Science Ltd., London, 1997.
24. Natchair, G. ; Syndrome of neurophthalmic significance in Neurophthalmology, a
manual for post graduate students, p-27.127-6, Arvind eye hospital, Maduri.
25. Shult, WT ; Tolosa Hunt syndrome in Current ocular therapy. Fifth edition. p-223
225, Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadelphia,
2000.
26. Bruin H.M. and Vanallen, M.W. ; Cyclic oculomotor paralysis. AJO 55 : 529, 1963.
27. Bajandas F.J. and Kline LB ; Aberrant regeneration of third nerve in
Neurophthalmology review manual. Third edition, p-93-94, Jay Pee Brothers, New
Delhi, 1985.
28. Sharma, P. ; Differential diagnosis of cyclo vertical strabismus in strabismus simpli-
fied. First edition. p-152153, Modern Publishers, New Delhi, 1999.
29. Janice A. Gault ; Miscellaneous ocular deviations in Ophthalmology secrets. First
Indian edition. Edited by Vander J.F. and Gault J.A. p-193, Jay Pee Brothers, 1998.
30. Rohatgi J.N. ; Acquired paralytic squint in Squint basic and clinical aspect. First
edition. p-197201, C.B.S. Publishers, New Delhi, 2003.
31. Gittinger J.W. ; Fourth nerve palsy in Manual of clinical problems in ophthalmology.
First edition. Edited by Gittinger J.W. and Asdourian G.K. p-177178, Little Brown,
Boston, 1998.
32. Dutta H. ; Superior oblique palsy in Strabismus. First edition. p-104109, Jay Pee
33. Lyle T.K. and Wybar KC : Paralytic strabismus in Lyle and Jacksons practical
orthoptics. First Indian edition. p-528575, Jay Pee Brothers, New Delhi, 1994.
34. Bajandas F.J. and Kline L.B. ; Five syndromes of fourth nerve in Neurophthalmology
review manual. First Indian edition, p-97105, Jay Pee Brothers, 1989.
35. Lois J. Martyn ; Vertical gaze palsy in Pediatric ophthalmology. Vol. II, Second edition,
p-798800, Edited by Harly RD, WB Saunders Company, Philadelphia, 1983.
36. Branett J., Dieterich M. ; Skew deviation with ocular torsion. A Vestibular brain stem
sign of topographic diagnostic value. Annals of neurology 33 : 528, 1993.
37. Keane J.R. ; ocular skew deviation. Arch. Neurology 32-185, 1975.
38. Lyle T.K. and Wybar KC ; Alternating hypertopia in Lyle and Jacksons practical
orthoptics. First Indian edition, p-341343, Jay Pee Brothers, New Delhi, 1994.
39. Folk R. Eugene ; Alternating hyper deviation in Principle and practice of ophthal-
mology. Vol. III, Ist Indian edition, p-18581860, Edited by Peyman G.A., Sanders DR
and Goldberg MR. Jay Pee Brothers, New Delhi, 1987.
40. Rowe Fiona ; Bielschowsky phenomenon (Dark wedge test) on Clinical orthoptics.
First edition, p-68, Blackwell Science, London, 1997.
41. Jampolsky, A. ; Surgical leashes and reverse leash in Strabismus surgical manage-
ment in symposium on strabismus. Transection of New Orleans Academy of Oph-
thalmology, Mosby, St. Louis, 1978.
42. Lois J. Martyn ; Abducence palsies in Pediatric ophthalmology. Vol. II, Second edi-
tion, p-795, Edited by Harley RD, WB Saunders Company, Philadelphia, 1983.
43. Glasser, J.S. ; Abducence palsies in Neurophthalmology. p-248253, Harper and Row,
London, 1978.
44. Duke Elder S. ; Congenital oculo facial palsies in System of ophthalmology. Vol. III,
part 2, p-10311032, Henry Kimpton, London, 1964.
45. Janice A. Glout ; Miscellaneous ocular deviation in Ophthalmology secrets. First In-
dian edition, p-194, Edited by Vander J.F. and Gault J.A. Jay Pee Brothers, New Delhi,
1998.
46. Huber A. ; Electro physiology of the retraction syndrome. B.J.O. 58:293, 1974.
47. Sharma P. ; Restrictive strabismus in Strabismus simplified. First edition, p-136
141, Modern Publishers, New Delhi, 1999.
48. Kerrison J.B. and Nancy Newman : Chronic progressive external ophthalmoplegia in
Current ocular therapy. Fifth edition, p-208210, Edited by Fraunfelder F.T. and
Roy F.H. WB Saunders Company, Philadelphia, 2000.
49. Moraces C.T., DiMauros ; Mito chrondrial DNA deletion in Progressive external
ophthalmoplegia. New Eng. Jr. Med. 320, 1293-1299, 1989.
CHAPTER 18
Nystagmus
1, 3
Nystagmus is a clinical sign. It is not a diagnosis, is a disturbance of ocular posture
caused due to long list of lesions that can be ocular or non ocular (central).
The exact mode of development of nystagmus is not well understood. It is fully co-
ordinated function with reciprocal innervation.
In normal steady fixation, the eyes are motionless. The steady fixation is maintained by
afferent path, efferent path and intracerebral components.
1
Deficiency in any of them
will result in involuntary movement of eyes. These involuntary movements are grouped as
nystagmus.
Ninety percent of nystagmuses are caused due to afferent defect in poor vision, rest
are caused due to efferent defect in ocular motor disturbance.
2
However there are occasions
when nystagmus can be physiological that can be elicited by appropriate stimuli.
3
Nystagmus can be congenital (infantile) or acquired. The latter can be physiologi-
cal that lasts for short time, only during the body is exposed to appropriate stimuli or it can be
pathological. Congenital nystagmus is always pathological.
Some terminologies
3
used in describing nystagmus areMorphology, plane, amplitude,
rate, direction, and grade.
Morphologically nystagmus can bependular nystagmus
4
, jerky nystagmus and
mixed.
Pendular nystagmus has oscillation of equal speed and amplitude on each side like a
swing of a clock-pendulum. They are generally present in primary position, they can be seen in
any plane i.e. horizontal, vertical or rotatory. Horizontal pendulum nystagmus is the common-
est. It is generally seen with sensory deprivation causing diminished central vision
5
due to
congenital lesion i.e. macular hypoplasia, achromatopsia, Lebers congenital amaurosis,
optic nerve hypoplasia or conditions acquired early in infancy like congenital and devel-
opmental cataract, high errors of refraction, corneal opacities.
It is seen as hereditary pendular nystagmus that runs in the families without other
ocular involvement and last for rest of life.
3
It is also seen in spasmus nutans. Other causes
of pendular nystagmus are miners nystagmus, acquired unilateral nystagmus, lesions
of brain stem, and drug toxicity.
The horizontal pendular nystagmus is a slow smooth to and fro movement that is re-
duced on convergence. It persists in dim light but disappears on closure of eyes. It is
associated with diminished vision.
628
NYSTAGMUS 629
Jerk nystagmus. This type of nystagmus is biphasic, it has a slow phase in one direc-
tion and a rapid phase on the opposite direction. Jerk nystagmus is customarily named after
the direction of rapid phase. The slow phase is the fundamental phase, while the rapid
phase is a compensatory phase.
6
The slow phase is pursuit phase while the rapid phase is
saccadic phase.
7
Common causes of jerk nystagmus can be congenital or acquired. They areOpto
kinetic nystagmus, end point nystagmus, vestibular nystagmus, defect in fixation,
latent nystagmus, congenital jerk nystagmus, gaze paretic.
Mixed nystagmus. This is a rarer than the previous two. In this condition there is
pendular nystagmus in primary position and jerk nystagmus in deviated eyes.
Null point (zone) in nystagmus is a point at which the oscillations are either mini-
mal or absent. It is situated mid way between levo version and dextro version. The child
tries to keep the eye in such a position that it is nearest to the primary gaze. If the null point
falls outside the primary gaze, the child will change the head posture to maintain the gaze in
a position that has least oscillation.
Plane of nystagmus can be horizontal (commonest), vertical, torsional, oblique or mixed.
Amplitude. This is a measurement of excrusion of the eyeball in degree during oscilla-
tion. It can be fine, medium or coarse. A fine amplitude has an excrusion between 5-15
degrees, excrusion more than 15 degrees is called coarse.
Rate (frequency)
8
of nystagmus gives the number of oscillation in a given time. It is
roughly divided into rapid and slow. It is given in cycles per second or Hertz, slow is 1-2 Hz,
while 5 or more Hz is called rapid or fast.
Relation between amplitude and frequency. Generally the rate varies with ampli-
tude, faster the rate, finer the amplitude and vice versa.
3
Intensity of nystagmus is calculated by multiplying amplitude with frequency.
Grades of nystagmus. Alexanders law states that amplitude of jerk nystagmus is
largest in the gaze of the direction of fast component. On the basis of this, nystagmus has been
put in to three grades
3, 8
First degree. Nystagmus is present only in the direction of fast component.
Second degree. Nystagmus present even in primary gaze.
Third degree. Nystagmus is present in both the above two positions.
Classification of nystagmus. Classification of nystagmus is one of the most difficult
tasks, because there are about forty types of nystagmus and all of them do not fall in to a
simple classification. Some of the useful classifications are :
I. Congenital ; or Acquired
II. As there is overlap of characteristics in many fields, the above classification is not
very suitable clinically. The other classification can be
1. Ocular (sensory deprivation) or
2. Non ocular (motor imbalance).
The term ocular nystagmus denotes that the cause is in the eye i.e. macular hypoplasia,
macular scar, optic nerve hypoplasia, Leber congenital amaurosia, congenital cataract.
While extra ocular nystagmus is caused byvestibular, brain stem, cerebellum or spi-
nal cord disorder.
9
Ocular nystagmus follows the rule of 2-4-6
4
, which states :
1. If the child has poor vision before 2 years of age, he will always develop nystagmus.
2. Between 2 to 6 years some children will develop nystagmus if central vision is lost.
Some will not develop nystagmus.
3. Ocular nystagmus does not develop after 6 years of age with loss of central vision.
Some of the nystagmuses are called physiological. They can be produced artificially
by appropriate stimuli, these last only during the presence of the stimulus. They are :
1. Optokinetic nystagmus
2. Rail road nystagmus
3. Evoked vestibular nystagmus
4. Voluntary nystagmus.
The remaining types are put under category of pathological nystagmus.
They are sub-divided into :
1. Ocular type of fixation nystagmus
2. Neurological type of fixation nystagmus
The former group includes :
1. Congenital jerk nystagmus
2. Hereditary pendular nystagmus
3. Latent nystagmus
4. Latent (manifest latent) nystagmus6
5. Pendular nystagmus of subnormal vision.
6. Spasmus nutans
7. Miners nystagmus
8. Acquired unilateral nystagmus
The second group consists of :
1. Nystagmus of neurologic origin (brain lesions i.e. chiasma, posterior fossa, brain
stem). They are :
(i) Fixational nystagmus
(ii) See-saw nystagmus.
2. Defects in gaze mechanism
(i) Gaze paretic nystagmus
(ii) Toxic nystagmus
(iii) Inter nuclear ophthalmoplegia
NYSTAGMUS 631
3. Nystagmus of vestibular defect
(i) Nystagmus of peripheral vestibular disorder
(ii) Nystagmus of central vestibular disorder.
A comprehensive classification given by Lyle and Wybar
9
is as follows :
1. Ocular fixation nystagmus
(a) Physiological
(i) Deviational nytagmus
(ii) Optokinetic nystagmus
(iii) Latent nystagmus
(b) Pathological
(i) Blindness
(ii) Defect in central vision
(iii) Spasmus nutans
(iv) Miners nystagmus
2. Labyrinthine and vestibular nystagmus
(i) Labyrinthine
(ii) Lesions of vestibular nerve
3. Nystagmus of central origin
(i) Brain stem lesion
(ii) Cerebellar lesion
(iii) Spinal lesion
4. Congenital idiopathic
5. Voluntary and hysterical
Symptoms of Nystagmus
1. The child is unaware of nystagmus especially if it is congenital or acquired in early
childhood. The parent may observe presence of nystagmus and seek help. There may
be history of nystagmus in the family, without any other sign of ocular involvement.
2. Diminished central visionThe vision is poor, there is no correlation between degree
of error of refraction and degree of nystagmus. However there may be associated
errors of refraction. It is the diminished central vision that is the cause of nystagmus
and not the other way.
3. A child may keep the objects very near to see clearly. This induces convergence which
in turn reduces nystagmus.
4. Changed head postureIn order to use the null zone, the child may assume abnor-
mal head turn. In vertical nystagmus the child may have abnormal chin position,
there may be head tilt.
5. Nystagmic child need not be blind. There should be some vision to develop
nystagmus.
10
6. SquintVarious types of squint are possible with nystagmus.
7. Other common symptoms consists of photophobia, glare, albinism, achromatopsia.
8. Neurological nystagmus may have symptoms of central nervous involvement i.e.
tinitus, hearing loss, vertigo, hemianopia, see-saw nystagmus, head nodding, torticolis.
Diagnosis. Diagnosis of nystagmus is not difficult, large amplitude nystagmus are ob-
served by parents. Important factor in diagnosis is to find out if nystagmus is congenital,
acquired in infancy or has developed after six years. Nystagmus developing after six years is of
central, vestibular or toxic origin. Fine nystagmus may require examination either by slit
lamp or by direct ophthalmoscope.
Various Types of Nystagmus
Physiological nystagmus
1. Optokinetic (opticokinetic)
11
nystagmus. This happens when a person gazes at a
succession of objects moving past in the field of gaze in one direction. The eye follows one
particular object slowly to the limit of comfortable conjugate gaze. After this the object of
interest disappears from the field of gaze, the pursuit is given up and the eyes move rapidly to
the opposite direction to fix another object of interest and repeat the same. In quick succession
resulting in to a jerk nystagmus. The commonest example of this is rail road or train
nystagmus. Where a person looking out picks up a part of floating scenario and follows in-
voluntarily till it disappears from the field of gaze and then fixes next object of interest. The
fixing movement is towards the direction of the train. The person is not aware of the nystagmus
but a person sitting opposite can see the nystagmus.
The same effect can be produced under specific condition by moving an optico kinetic
drum (Catford drum) which has alternating white and black strips of same width. The con-
trast between the white and black strips should be the same all through. When the drum is
moved in front of the child, the eyes develop nystagmus, similar to rail road nystagmus and
observed by the examiner. Presence of nystagmus means presence of vision, in a child sus-
pected to be blind, absence of opto kinetic nystagmus is diagnostic confirmation of blindness
unless the child has serious central nervous system defect including mental deficiency. The
other use of opto kinetic drum is to detect malingering as well.
Opto kinetic drum rotated vertically will produce vertical nystagmus.
2. Vestibular evoked nystagmus. This is a jerk nystagmus that can be produced by
various methods. Commonly used methods are :
1. Displacement of the endolymph in the semi circular canal.
2. Acceleration and deacceleration of the body.
If cold water is injected in the right ear, a jerk nystagmus will be produced on the left
side and irrigation of the ear by warm water will produce nystagmus on the same side, the
mnemonic is COWS i.e. cold opposite warm same side.
NYSTAGMUS 633
Pure horizontal nystagmus can be produced by rotating the upright body with head
flexed at 30. A vertical nystagmus can be produced by rotating the upright body in same
manner with head tilted towards either shoulder.
3. End point nystagmus. This is ill sustained jerk nystagmus that develops on ex-
treme lateral gaze, when the object of interest is outside the binocular field of vision. It does
not occur in vertical gaze. It is more marked in abduction. The nystagmus consists of ten to
fifteen beats and occurs on the side of the gaze.
4. Voluntary nystagmus. Some persons can induce pendular nystagmus at will for
very brief period, best during convergence. Children learn sooner than adults. Many number
of the family may have the skill to produce voluntary nystagmus.
Pathological nystagmus :
1. Latent nystagmus. When both the eyes are open and straight, there is no nystagmus
as soon as any of the eyes is covered both the eyes go for jerk nystagmus towards the uncov-
ered eye. The vision in the uncovered eye also diminishes. It is a congenital condition, bilat-
eral, mostly seen in esotropia and in hypertropia. If both eyes are covered the nystagmus
disappears. The condition lasts for whole life
9
.
If the weaker of the two eyes is occluded the nystagmus is finer, the nystagmus is also
reduced and vision improved if the eye under observation is adducted.
A nystagmus is termed manifest latent nystagmus when there is manifest nystagmus
that becomes more severe if the dominant eye is covered.
8
2. Manifest nystagmus. This is that type of nystagmus that is present when both eyes
are uncovered and does not increase following covering of either eye. It is bilateral, conju-
gate mostly horizontal. It may have a mixture of both pendular and jerk nystagmus. These
children have better near vision. The nystagmus is reduced on convergence. Generally there is
abnormal head posture to bring the eyes in null zone. It disappears during sleep.
3. Nystagmus blockage syndrome. It is common for infantile esotrope children to
have either manifest nystagmus or manifest latent nystagmus. On occasions on adduc-
tion, the nystagmus is reduced and vision improves. Nystagmus is otherwise present when
the eyes are straight. This is called nystagmus blockage syndrome. The nystagmus in
primary gaze is horizontal and increases on abduction. They may have cross fixation. They try
to see either by converging both eyes or changing the head posture towards the adducted eye.
4. Miners nystagmus. Obviously this is not seen in children. This develops in miners
after years of work underground in dim illumination. The nystagmus is mostly horizontal but
can be oblique as well. It is mostly seen in up gaze.
5. Spasmus nutans
3, 12, 13
. Spasmus nutans consists a triad of nystagmus, head nod-
ding and troticollis in infants. Its cause is unknown. It is a benign condition, does not
denote any disease, lasts for only a few months and disappears leaving no untoward affect.
However sometimes intracranial lesion may produce signs similar to spasmus nutans. The
nystagmus is pendular, very fine, rapid. It is horizontal in plane, the nystagmus varies in
different gazes. It is asymmetric. The head nodding is not uniform and irregular in rhythm,
may be horizontal, vertical or both. Torticollis begins with nystagmus and passes off with it.
The condition does not require any treatment.
6. Hereditary pendular nystagmus. It is a congenital nystagmus that may be seen in
other members of the family. It lasts for life, horizontal in nature that remains horizontal even
on up gaze. However the nystagmus may become jerk nystagmus on lateral gaze.
7. Nystagmus secondary to diminished vision. Clinical presentation is similar to
the hereditary pendular nystagmus of congenital origin. The difference is that vision becomes
grossly defective in first year of life. The common causes that result in this condition are -
congenital optic atrophy, macular lesion, achromatopsia, congenital or developmental cata-
ract, high error of refraction. There is no family history, the condition follows the genetic
pattern of the primary cause of defective vision.
8. Congenital jerk nystagmus. There is horizontal jerk nystagmus, which is coarse.
The vision is poor but best in null zone. There is abnormal head posture to improve vision.
Some neurological conditions that produce nystagmus
Disorders Type of nystagmus
1. Posterior fossa disease Acquired fixtaional nystagmus
2. Demyelination of mid brain Acquired fixtaional nystagmus. Not common in
children.
3. Chiasmal lesion See saw nystagmus
4. Gaze paresis Jerk nystagmus, jerk opposite to side of lesion
5. Inter nuclear ophthalmoplegia Jerk nystagmus on lateral gaze
6. Vestibular lesion Horizontal rotary jerk, may be of constant direction
in peripheral lesion. In central lesion, the direction
may change, may become vertical on up gaze.
See Saw nystagmus. In contrast to nystagmus described earlier, which were conju-
gate, this is a disjunct nystagmus. In this as one eye elevates and the other eye depresses. It
is often associated with chiasmal lesion producing chiasmal type of field defect.
Down beat nystagmus
13
. This consists of downwards jerking movements on down-
wards gaze but can happen in any head posture. Nystagmus increases in amplitude with gaze
down and out. It is found in lesions of spinomedullary junction of congenital origin.
Upbeat nystagmus. It may be of congenital origin, drug induced or posterior fossa
lesion. In primary position the fast component is upwards.
Management of nystagmus. Management of nystagmus is most frustrating both for
the ophthalmologist and the child. Poor vision of nystagmus rarely responds well with optical
correction. Medical treatment for oscillation of the eye has limited role in selected cases. Sur-
gical intervention may correct associated squint and reduce oscillation to some extent which in
turn increases foveation time and increased distant vision.
NYSTAGMUS 635
Various optical options available are :
1. Prescription of glasses after cycloplegic refraction.
2. Adding concave glasses to distant correction, induces artificial accommodation that
is accompanied with secondary convergence. This induced convergence diminishes
amplitude and rate of nystagmus thus enhancing vision.
3. Similarly prism may be given to strengthen convergence.
4. Contact lenses have been reported to have reduced amplitude and frequency.
Medical treatment. Many drugs have been used to treat nystagmus. They are - Baclofen,
gabapentin, clonazepam, valproate, and carbamazepine.
2
Retrobulbar injection of botulin toxin A (Botox) has been found to abolish nystagmus for
short period.
Surgical treatment consists of Anderson or Kesten baum procedures. The principle of
which is to move the eye to the null zone.
Recession of all the recti have also been advocated.
REFERENCE8
1. Lyle T.K. and Wybar K.C. ; Nystagmus in Practical orthoptics in the treatment of
squint. First Indian edition. p-592600, Jay Pee Brothers, New Delhi, 1994.
2. Wheeler D.T. ; Nystagmus in Current ocular therapy. Fifth edition. p-407410, Ed-
ited by Fraunfelder F.T. and Roy H.F. WB Saunders Company, Philadelphia, 2000.
3. Martyn Lois J. ; Nustagmus in Pediatric ophthalmology. Vol. II. Second edition.
4. Kanski J.J. ; Nystagmus in Clinical ophthalmology. Second edition. p-475477, But-
ter worth, London, 1989.
5. Deborah Pavan Langston. ; Nystagmus in Manual of ocular diagnosis and therapy.
Third edition. p-323325, Lippincot Williams and Wilkins, Philadelphia.
6. Rohatgi J.N. ; Nystagmus in Squint basic and clinical aspect. First edition. p-211
218, CBS Publishers and Distributors, New Delhi, 2003
7. Seal S.K. ; Nystagmus in G.N. Seals Text Book of ophthalmology. Fifth edition.
p-404405, Current Book International, Kolkata, 2002.
8. Sharma P. ; Nystagmus in Strabismus simplified. First edition. p-155163, Modern
9. Lyle T.K. and Wybar K.C. ; Nystagmus in Practical orthoptics in the treatment of
squint. First Indian edition. p-595, Jay Pee Brothers, New Delhi, 1994.
10. Reinecke R.D. ; Nystagmus in Ophthalmology secrets. First Indian edition. p-202
205, Edited by Vander J.F. and Gault J.A. Jay Pee Brothers, New Delhi, 1998.
11. Rosenberg M.A. ; Nystagmus in Principles and practice of ophthalmology. Vol. III,
p-19711974, Edited by Peyman G.A., Sanders D.R. and Goldberg M.F. First Indian
edition. JayPee Brothers, New Delhi, 1987.
12. Glaser J.S. ; Nystagmus and related ocular oscillation in Neuro ophthalmology.
p-221-236, Harper and Row Publishers, London, 1978.
13. Gittinger J.W. ; Down beat nystagmus in Manual of clinical problems in ophthal-
mology. First edition. p-182184, Edited by Gittenger J.W. and Asdourian G.K.
Littlebrown and Co., Boston, 1998.
CHAPTER 19
Non Paralytic 8quint in Children
Binocular vision
1
Binocular single vision gives animals of higher order, a better view. Binocular single
vision is a pre-requisite for larger field of vision, stereopsis and better visibility. For this both
the eyes should have a well coordinated movement and a sharp image formed on the two
foveae.
The binocular single vision is not present at birth, it is an acquired faculty. It starts
developing by six weeks of age. The child starts following a bright light for a short distance.
This is the beginning of fixation reflex. The refixation reflex takes some more time to develop
and develops by four to six months of age. By the age of six years, the fovea develops fully
and the child has almost 6/6 vision in each eye with binocular single vision and stereopsis if
the eyes are straight.
Fixation reflex
The two eyes are inter-related anatomically and physiologically. The physiological bond
is enforced via various reflexes, fixation reflex is one of them.
2
Fixation reflex is defined as an involuntary reflex which adjusts the eyes in such a
way that the image of object of regard fall on the foveae at the same time and the image of
moving objects are retained on the foveae.
The re-fixation reflex brings the eyes to the original point of fixation. This could be
active or passive.
Fusion reflex is an involuntary reflex that is controlled by a fusional centre in the
occipital cortex. This reflex adjusts the eyes in such a fashion that binocular single vision is
produced and maintained.
To have a single binocular vision, it is essential that the two images fall on
corresponding points of the two retinae and fused as one.
The retinal points that do not correspond are called disparate points. If an image is
formed on disparate points, the eye will not have binocular single vision and will result in
binocular diplopia.
637
Horoptor
Horoptor is an imaginary surface in space. All points lying on the horopter curve will
stimulate corresponding retinal element and will be seen as one. For every retinal point in one
eye, there is a limited area around the corresponding point in the other eye. Stimulation of
this area along with original point can also result in binocular single vision. This limited area
is called Panums fusional area. Objects in front and behind the Panums area cause diplopia.
Projection is defined as interpretation of the position of an object in the space due to
stimulation of retinal element. The foveal projection is straight ahead. A point in temporal
retina is projected in the nasal field and vice-versa.
Prerequisite of binocular single vision :
1. Reasonably clear media in both eyes.
2. Accurate co-ordination between the two eyes in all directions of gaze
3. Ability of the brain to cause fusion of two slightly different images.
Grades of binocular vision are :
1. Simultaneous perception
2. Binocular fusion
3. Stereopsis
Advantages of binocular single vision are :
1. Greatest advantage of binocular single vision is stereopsis or perception of depth.
2. Binocular field of vision is larger than uniocular field.
3. Due to overlapping of the uniocular fields, defect in one field is masked by the other
field.
4. Binocular single vision is greater than uniocular vision.
Lack of binocular single vision results in double vision.
3
This has two components :
1. Confusion
2. Diplopia
1. Confusion. A patient without squint has a correspondence between the two foveae.
The brain superimposes and fuses the two images provided they are similar enough.
In case of a manifest squint fovea of fixing eye sees the object of regard while the squinting
eye sees something other than the object of regard. The brain in an effort to superimpose these
two dissimilar images create a state of confusion. This confusion is the result of corresponding
retinal points being stimulated by two different objects.
Confusion can not be explained well by the patient. It is very rare for a patient to describe
two different images superimposed over one another.
Confusion is soon replaced by binocular diplopia.
2. Diplopia is the result of one object stimulating non corresponding retinal points i.e.
fovea of fixing eye and non foveal point in the deviating eye. Pathological binocular diplopia
develops only in presence of manifest squint. Esotropia cause uncrossed (homonymous)
NON PARALYTIC SQUINT IN CHILDREN 639
diplopia while exotropia cause crossed (heteronymous) diplopia. Direction of diplopia depends
on involvement of particular muscle or group of muscles. It is most marked in acute paralytic
squint. Diplopia can be horizontal, vertical or torsional.
Diplopia is generally associated with paralytic squint, though there is misalignment of
eyes in concomitant squint as well, it is not associated with diplopia because of the following
mechanism :
1. Anomalous retinal correspondence
2. Suppression
3. Amblyopia
Anomalous retinal correspondence (abnormal retinal correspondence ARC)
4
Normal retinal correspondence is a state where fovea of one eye corresponds with fovea
of the fellow eye, nasal retina of one eye corresponds with temporal retina of the fellow eye, the
two images are fused together to give a binocular single vision. Once normal retinal
correspondence has been well established up to adulthood, it is not possible for ARC to develop
hence ARC is seen in children.
Abnormal retinal correspondence
Abnormal retinal correspondence is an attempt to restore binocular single vision as
far as possible. The binocular single vision may not be real but may resemble it. ARC is an
adaptation in squint to avoid diplopia and confusion. It is not an anatomical change in the
retina, it is a cortical adaptation. ARC may lead to formation of pseudo macula.
2
It co-
exist with some suppression. Amblyopia may be present in ARC. It has various degrees of
severity.
ARC develops more commonly and is more severe when the child is small, with smaller
degree of squint, longer the duration of squint more severe is the ARC. It is more common in
infantile esotropia, less common is exotropia and least in vertical squint.
1
Clinically there are two types of abnormal retinal correspondence :
1. Harmonious
2. Unharmonious
In harmonious ARC, angle of anomaly is equal to angle of squint i.e. subjective angle
of squint is zero. In unharmonious ARC, the subjective angle of squint is less than objective
angle.
Objective angle of squint is the actual measurement of deviation. This is measured
on synoptophore. The patient sits on the synoptophore with chin on chin rest and head
touching the head band. The inter pupillary distance is adjusted. The fixing eye fixes the slide
in the tube. The non-fixing eye deviates. The examiner moves the tubes of the synoptophore so
that the corneal reflexes produced by the illumination from the tubes are located in the centre
of the cornea. The examiner than does alternate cover test by alternatingly switching off the
lights in each tube and moving the tube until there is no movement in either eye. Angle between
the two eyes gives the objective angle of deviation.
In the other method the slide of lion is put in front of the fixing eye and the cage before
the deviating eye, the tube in front of the fixing eye is set at zero. The other tube is moved by
the examiner till the corneal reflex is brought to the centre of the deviating eye. At this point,
the patient can see the lion and the cage simultaneously but can not superimpose. This gives
the objective angle. To measure subjective angle the patient moves the tube to put the lion
inside the cage and the corneal reflex is in the centre of the squinting eye.
The difference between the objective and subjective angle is the angle of anomaly.
In normal retinal correspondence (NRC) the two angles are equal.
In harmonious ARC, the subjective angle is zero.
Various methods available to diagnose ARC
4
1. Bagolini glasses (striations)
2. Worth four dot test
3. Synoptophore
4. Titmus fly test
5. After image test
6. Vertical prism test
7. Binocular convergence test
Abnormal retinal correspondence does not develop in all cases of squint always.
The conditions where ARC may develop are :
1. Small angle of non paralytic esotropia
3. Esotropia developing under three years of age
4. Congenital alternating esotropia
5. Residue in post operative status
Conditions where ARC may not develop :
1. Concomitant divergent squint
2. Concomitant convergent squint after 15 years
3. Esotropia with large degree of deviation
Management of ARC
It is not always possible to treat ARC. It may be so mild that it is not worth the trouble
to treat it. In early stages occlusion of the sound eye may help. In cases of alternate squint,
alternate occlusion should be tried in children. Orthophoric treatment may be required.
Suppression
Suppression is an in-built central faculty that develops in young children with manifest
squint. It is meant to ward off diplopia. It develops in the squinting eye that gets a blurred
image. The brain learns to ignore the blurred image. It is a binocular feature, disappears if the
fixing eye is closed. Suppression is mostly uniocular but may be alternating. Suppression is
not limited to the fovea. There are larger areas where suppression is present. These areas are
called suppression scotomas. They are seen both in esotropia as well as exotropia. Esotropic
suppression scotomas extends nasally while the exotropic suppression scotoma spread towards
temporal side.
There are two types of suppression :
1. Facultative
2. Obligatory
1. Facultative suppression. It is that type of suppression that develops in the eye
when it deviates. The moment the eye takes up fixation, the suppression disappears. This is
met with in alternating deviation.
2. Obligatory suppression. In this, suppression is present even when the squinting
eye takes up fixation. It is most commonly seen in monocular esotropia. In such cases, in initial
stages the suppression is facultative which becomes obligatory later.
Suppression is diagnosed by :
1. Worth four dot test
2. Red glass test
3. Bagolinis glasses
Eccentric fixation
This is a uniocular feature in which an object is fixed by a retinal point other than fovea
without change in the principle visual direction. It develops in long standing esotropia and
microtropia. There are various types of eccentric fixation according to location of the retinal
point that acts as eccentric point as per findings of visuscope.
5
Visuscope is a modified direct ophthalmoscope that has an in-built star which can be
projected on the retina. The instrument is used as any other direct ophthalmoscope with dilated
pupil. To do the test one eye is closed. The beam of the visuscope is focussed on the retina, this
casts a shadow of the star on the retina. The patient is asked to fix the star. In normal eye,
the star will be superimposed on the macula but not in eyes with eccentric fixation. As per
position of the star, the eccentric fixation can be classified asPara foveal, para macular,
para central, centrocecal. In erratic fixation, the star seems to jump from one place to
another near the macula.
Squint
In normal person the visual axes of the two eyes are parallel in primary position and all
direction of gaze except convergence and divergence, there too the angle of convergence or
divergence of one eye is equal to that of the fellow eye. This ideal condition is called orthophoria.
The position of rest in a normal person is slight divergence.
Orthophoria like many physiological conditions is exception than rule. All such non
orthophoric need not have manifest deviation. In contrast to this, a person may be orthophoric
but looks to have squint due to anatomic configuration of the eye, lids or orbit. Such conditions
are called pseudo squint.
Causes of pseudo squint are :
1. Prominent epicanthic foldThis gives an appearance of convergent squint.
2. HypertelorismThis gives an appearance of divergent squint.
3. Facial asymmetry results in pseudo vertical squint.
4. Hypermetropia due to large positive angle kappa gives an appearance of apparent
divergent squint.
5. Myopia due to large negative angle kappa gives an appearance of convergent squint.
An apparent convergent squint worsens true convergent squint and neutralises diver-
gence squint. Similarly an apparent divergent squint worsens appearance of divergence.
In pseudo convergent squint, obliteration of epicanthic fold abolishes squint.
In pseudo squint, the corneal light reflex is always in the centre of the cornea. The eyes
do not move under cover test or alternate cover test. Pseudo convergent squint gradually dis-
appears as the child grows and epicanthic fold fades away. But this is not true for hypertelorism
that is a permanent feature. Pseudo squint due to errors of refraction may disappear with
correction.
It is a wrong notion that a true manifest squint will disappear as the child grows. In fact
with age, the child adapts a compensatory head posture that may mask the squint to a casual
observer.
A high degree of fusion is required to keep the eyes in near orthophoric condition. A
breakdown of fusion will cause one of the eyes to deviate. This is called strabismus or squint
and is defined as a state when only one of the visual axes is directed towards the point of
fixation and the other deviates away.
There are two possibilities :
In one condition, the fusion is maintained under strain and as soon as the fusion is
broken, the eyes deviates only to return to normal position when the fusion is restored. This
state is called heterophoria or latent squint.
In the other type of squint, the fusion is already broken down or is absent resulting into
a visible deviation of eye. This is called heterotropia.
In between the two are the conditions that cause intermittent heterotropia.
The deviation may not be equal for near and distances. It may vary during the day, or
may be present from time to time.
Classification of squint
Varieties of squints are caused due to diverse causes with great variation in signs and
symptoms making it impossible to have an uniform classification of squint. Most practical is to
divide ocular deviation into :
1. Latent : (apparent) heterophoria
2. Manifest : heterotropia
In case of manifest squint, it is essential to find out if the deviation is
1. Pseudo squint or
2. Real squint
Pseudo squint does not require any specific treatment and does not develop sequel of
manifest squint.
Once it has been decided that the child really has a manifest squint, the next step is to
decide if the squint is
1. Concomitant
2. Non comitant
Non comitant is further divided into
1. Paralytic
2. Restrictive (obstructive)
A flow chart given below is helpful
Eyes
Orthophoric
(Rare)
Non orthophoric
Latent Intermittent Manifest
Pseudo squint
(Never latent)
Real squint
Concomitant Noncomitant
Restrictive Paralytic
Secondary
(Late)
Primary change
(Acute)
Latent squint (Heterophoria)
Heterophoria is that binocular state of ocular balance where the two eyes are kept in
physiological alignment (orthophoric) for distance as well as near and in all direction of gaze so
long the child is able to maintain binocular fusion.
Once the fusion has been broken, any of the eyes will deviate only to come to near
orthophoric position when the fusion has been restored.
Fusion can be broken in following conditions :
1. One eye is occludedCover test
2. Dissimilar images fall on two foveaeMaddox rod test
3. The two images have different colourRed glass test
4. Separate images are being projected on two foveaeMaddox wing test
Etiology of heterophoria is not well understood. Some of the following conditions may
cause heterophoria singly or in combination.
1. Uncorrected error of refraction :
(a) Hypermetropia is more associated with esophoria than myopia.
(b) Myopia is more associated with exophoria than hypermetropia.
2. Muscular anomaly :
(a) Faulty origin
(b) Faulty insertion
(c) Poorly developed muscle
3. Abnormality of the orbit
4. Defective innervation of extra ocular muscles, muscles in each eye getting different
tonic innervation.
5. Debilitating disease
6. Fatigue
7. Drugs, toxins, psychomatic disorder
8. AgeChildren are more likely to develop esophoria.
9. Heredity
Types of heterophoria
Heterophoria is not a manifest condition, neither the child nor the parents are aware of
it so long the fusion is maintained. Its presence is elicited by cover test and its extent is evaluated
by various tests. Maddox rod, Maddox wing, red glass test on Maddox tangent, prism test,
synoptophore etc.
As per direction of the eye when fusion is broken i.e. cover test. Heterophoria can be :
1. Esophoria. The eye deviates nasally under cover and move temporally to take up
orthophoric position when cover is removed.
2. Exophoria. When the eye deviates out under cover and moves inward when cover is
removed.
3. Hyper phoria/hypo phoria. One eye has a tendency to turn upwards in relation to
the other and called hyper phoric. Logically there is a possibility that one eye can
deviate down in relation to the other eye and should be termed as hypo phoria but in
practice the term has been given up in favour of right hyperphoria or left hyper phoria.
Here it is worth noting that esophorias and exophorias are not specified as right or left
phoria.
In horizontal phorias the amount of deviation in each eye is the same for a given
distance but may change in relation to distant or near fixation.
Cyclophoria
This is the rarest form of phoria. In this there is tendency for one eye to wheel rotate in
relation to the other. If the upper pole of the eye in question dips inwards, it is called
incyclophoria and when it dips outward it is called excyclophoria.
There is no condition when the two eyes deviate in different direction under cover.
Diagnosis of heterophoria
Cover test
This simple test which does not require any special instrument denotes presence and
type of heterophoria. The child is made to look at a distant object, than any of the eyes is
covered and the other is made to fix. After a few seconds the occluder is removed and the
movement of the eye is noted. Movement in any direction denotes presence of heterophoria. If
the eye moves out to take up fixation means that the eye has moved in under cover, in a
convergent position and the condition is called esophoria.
If the eye moves in, it is called exophoria. If the eye moves down, it is hyperphoria. If
the upper pole dips medially, it is incyclophoria, if the upper pole moves out it is
excyclophoria.
The test is repeated for near. It is better to do the test with correcting glasses also, if
phoria is still present with correction, the exact number that abolishes deviation should be
noted. If movement still persists with best correction, other method of treatment should be
considered.
Once the direction of deviation has been known by cover test, the next step is to find out
the degree of deviation that can be done by :
1. Prism and cover test
2. Maddox rod test
3. Red glass test
4. Synoptophore test
5. Prism dissociation test
6. Maddox wing test
7. Double Maddox rod test
8. Maddox double prism
Measurement of phoria for distance
1. Prism and cover test. Prism of increasing strength with apex towards the deviation
is placed in front of one eye. The child fixes with the other eye. Cover uncover test is performed
till all the movements in the eye under cover are abolished. This gives the amount of deviation
in prism diopters. This test is equally useful in both heterophoria and heterotropia.
2. Maddox rod test. The child is made to sit in a semi darkened room 6 meters away
and in front of the Maddox tangent. The Maddox rod is traditionally put in front of the right
eye and position of the red streak noted on the scale, both horizontal and vertical. In orthophoria,
the streak passes through the central light of the scale, otherwise it passes away from the
central light, the distance of streak from the central light is directly read from the scale inscribed
on the Maddox tangent. The test fails to differentiate between phoria and tropia.
3. Red glass test. This is similar to Maddox rod test. In this test, instead of a Maddox
rod, a red glass is used. The procedure is the same as in Maddox rod test. If the vision is
unequal, the glass is put in front of the better eye to prevent suppression of the poorer eye. The
red glass should be dark enough so that the patient sees only central light of the Maddox scale.
This test fails to differentiate between phoria and tropia.
4. Synoptophore test. Synoptophore is a versatile instrument that not only give degree
of deviation but also can be used for cover test, alternate cover test, degree of deviation, measure
range of fusion, suppression and retinal correspondence. It can also be used for orthoptic
treatment for both phorias and tropias.
5. Prism dissociation test. This is less frequently used test in which a 6 Pd prism,
base down is held before one eye and a rotary prism is held in front of the other eye. In presence
of horizontal phoria, the patient will have both vertical and horizontal diplopia. The rotary
prism is rotated to align the images horizontally one above the other. The power of the prism
that causes horizontal alignment is the degree of heterophoria.
Measurement of phoria for near
Phorias for near can be measured roughly by cover test, prism and cover test, but the
most accurate measurement is obtained by Maddox wing. It not only gives information about
horizontal or vertical phoria but also measures cyclophoria for near.
Detection and measurement of cyclophoria
Careful observation during cover test gives a rough idea about cyclophoria. It is better
measured by :
1. Maddox wing
2. Double Maddox rod, one usual red and other white
3. Maddox double prism
Double Maddox rod test
In this test a red Maddox rod and a white Maddox rod is put in front of each eye, in the
trial frame with their axes at 90. This results into two horizontal lines one red and other
white. The patient fixes a white bulb at six metres. If there is no cyclophoria, both the lines will
be parallel to floor. If they are not parallel, the glass causing oblique image is moved to make
the streak parallel. The axis of rotation gives the amount of cyclo deviation.
Maddox double prism test
Maddox double prism consists of two prism each 4 prism diopter in strength mounted on
a lens ring in such a way that they are base to base with apex away. The double prism is put in
the trial frame in front of one eye, the other eye is left uncovered without any lens or prism.
The patient is given a white sheet of paper which has a line drawn in the middle. The patient
keeps the paper at usual reading distance in such a way that the black line is horizontal. The
two prisms in the trial lens separate the line away from each eye. Thus a patient without
cyclophoria will see three parallel lines. The central line is the line seen by the eye without the
prism. The other two lines one above and one below the central line are formed by the two
prisms. If the central line is oblique to the two parallel lines, the patient has cyclophoria or
cyclotropia.
Symptoms of heterophoria
6
Most of the time the child is not aware of heterophoria and has no symptoms. The child
is generally able to overcome the deviation without much effort. The parent may notice the
deviation occasionally when the child is either daydreaming or fatigued. So long the fusion is
well maintained, the phoria is said to be compensated. The symptoms arise only in
decompensated phorias.
Factors that precipitate decompensation are :
1. Inadequate fusional reserve
2. General debility
3. Prolonged near work
Horizontal phorias are least disturbing, symptoms are more in vertical phorias and
most troublesome in cyclophoria.
Common symptoms are :
1. Asthenopia and asthenopia related :
(a) Burning
(b) Redness
(c) Itching
(d) Stye, chalazion
2. Visual
(a) Blurring of vision
(b) Running of letters
(c) Intermittent diplopia
3. OthersDizziness, vertigo, nausea
Esophoria
Here the eyes move in on dissociation. It is more common in children. Hypermetropic
children have more tendency to develop esophoria than emmetropic or myopic.
Type of esophoria
1. Convergence excess typeHere esophoria is more for near than for distance.
2. Divergence weaknessHere esophoria is greater for distance than for near.
3. In between the above two there is not much change in near or distant deviation.
Management
1. First and foremost treatment is correction of any error of refraction under cycloplegia
and prescription of glasses for constant use.
2. Orthoptic treatment. The fusion is the factor that keeps the eyes in alignment in
heterophoria. When the eye becomes decompensated, improvement of fusional divergence (rela-
tive negative convergence) is desired.
They consist of
(i) Divergence exercises on synoptophore
(ii) Home stereogram exercise
(iii) Bar reading in case of convergence excess.
3. Prism may sometimes reduce symptoms. Children using glasses accept prisms bet-
ter than those who do not use spectacles. Fresnel prism may be pasted over the spectacle
glasses for temporary use.
4. Surgery is seldom required.
Exophoria
Here the eyes diverge on dissociation.
Types of exophoria
1. Convergence weaknessHere exophoria is greater for near than distance.
2. Divergence excessHere exophoria is greater for distance than near.
3. In between the twoThere is not much difference between near and distant deviation.
Exophoria should be differentiated from intermittent exotropia. Exophoria rarely
measures more than 20 prism dioptres while in case of intermittent exotropia the measure-
ment is more. Intermittent exotropia develop suppression hence they do not develop diplopia
and become manifest.
Management
In children treatment is required only if the eye has tendency to become exotropic. Here
also first step is to correct any error of refraction under cycloplegia followed by orthoptic
treatment.
Hyperphoria
This is a vertical phoria, less common than horizontal phorias. However a combination
of vertical phoria associated with horizontal phoria is common. As vertical fusional amplitude
is relatively less, asthenopic symptoms are more in vertical phoria. Even a deviation of 2-4
prism diopters produces sufficient symptoms. The treatment is prescription of prism with
base down in case of hyperphoria and base up in case of hypophoria. Strength of the prism is
equally divided in two eyes. It is better only to neutralize one third to half of deviation by
prism
7
. Surgery is indicated in large deviation with paretic element.
Dissociated vertical divergence (DVD) is also a type of hyper deviation and called double
hyperphoria.
Cyclophoria
This is rarest form of latent squint with profound symptoms and is most missed type of
phoria unless specifically looked for and eye is examined by Maddox wing, Maddox double rod,
and Maddox double prism. In this case the eyes have tendency to rotate along anterio-posterior
axis. Excyclophoria is more common than incyclophoria. It is generally associated with vertical
phoria.
Treatment is directed toward treatment of vertical phoria.
Manifest squint (Heterotropia)
Heterotropia is that state of muscle imbalance in which one of the eyes deviates in
relation to the other eye. This can be :
1. Concomitant squint
2. Incominant (non comitant) squint
(a) Paralytic squint
(b) Restrictive
(c) Kinetic
8
Kinetic strabismus is a rare, often missed clinical condition, is due to irritative
intracranial pathology resulting in unequal stimulation of nerves, nerve centres or muscles.
Concomitant squint
Characteristics of concomitant squint :
1. It is a visible diviation deviation.
2. The deviation is present most of the time.
3. The deviation may vary for distance and near.
4. The object is fixed by one eye at a time.
5. The fixation may alternate between two eyes.
6. The angle of squint remains constant in all directions of gaze.
7. The ocular movements are normal in all direction.
8. The squint may be mono-ocular or alternate.
Etiology of concomitant squint are the same as in latent except that there is a
permanent break down of the fusion. Any eye can have concomitant squint -
1. As primary disorderThe deviation starts without being latent.
2. Latent squint getting so much decompensated that the eyes fail to revert to
orthophoria.
Phoria Intermittent tropia Constant tropia
3. Spread of comitance in case of congenital squint.
Duane
9
has divided squint into two basic types :
Near dysfunctionMedial rectus dysfunction
Distance dysfunction lateral rectus dysfunction.
This is based on the fact that medial recti are responsible for near and convergence and
lateral recti are responsible for distance and divergence.
The etiological factors are :
1. CongenitalMal-origin or insertion of one or more extra ocular muscles. There may
be under development of muscle/muscles.
2. Defective vision in one eye due to any cause i.e. opacity in media, optic atrophy, macular
lesion, ptosis, error of refraction.
3. Defective vision in both eyesMore in one than the other, the eye with less vision is
more likely to squint.
4. Dissociation between accommodation and convergence - This is one of the most com-
mon causes of squint in children.
(a) HypermetropiaIn moderate degree of hypermetropia, the eye accommodates
more to see clearly. This stimulates convergence as well as produces convergent
squint.
(b) MyopiaAn acquired myopic eye does not require accommodation for clear vision
hence there is reduced convergence and the eye deviates out.
A child with congenital myopia has a very short far point, and can see only near object.
To see near things he has to converge more than an emmetrope of same age, this results in
convergent squint.
5. Deficient fusional power
6. Secondary change in paretic muscle
A child born with paralysis of one of the muscles, may recover over a period of time,
but its direct antagonist develops contracture and under action of muscle that was
limited to one gaze only, spreading to all directions of gaze.
7. HeredityAbout 10% of squint have family history.
Types of heterotropia
Heterotropia can be unilateral (monocular) or alternate. Unilateral heterotropia is
more common than alternate. A squint is said to be unilateral when one the eyes squints
constantly and the other eye fixes. If the fixing eye is covered, the squinting eye takes up the
fixation and the fixing eye deviates under cover. In both eyes the angle of squint is equal. As
soon as the cover is removed the previously covered eye takes up fixation and the other eye
squints.
In alternate squint when the fixing eye is covered, the squinting eye deviates under
cover and remains deviated when the cover is removed. The angle of deviation is equal in both
eyes. The vision in both eyes is almost the same and loss of vision is not much. Generally both
the eyes are emmetropic. Even if there is any error of refraction present, it is of low grade.
Alternate esotropia is more common than exotropia. The alternating eyes lack all the grade of
vision.
According to direction of deviation, the eye can be esotropic, exotropic, hypertropic or
rarely cyclotropic.
Diagnosis of heterotropia
Diagnosis of well established moderate manifest squint is simple. In fact the parents
bring the child for complaints of squint. The child is generally unaware of squint. Once the
child becomes aware especially the teenagers, they try to hide the cosmetic blemish keeping a
downward gaze.
1. Examination of distant vision should get preference over all other examination and
whenever there is diminished vision in any of the two eyes, the cause of the diminished
vision should be determined by refraction, examination of anterior segment and fundus
examination. Diminished vision in the squinting is common but the other eye too
may have diminished vision due to same error of refraction or different errors of
refraction may exist between the two eyes. There may be difference in vision in two
eyes due to other causes.
2. The next step is to find out if the squint is real or pseudo squint.
3. Once pseudo squint has been ruled out, the next step is to exclude possibility of
paralytic squint.
Paralytic (incomitant) Concomitant
Age Any Any
Onset Sudden Usually gradual
Symptoms Diplopia (a) Diplopia is a major symptom. Generally there is no diplopia
(b) Diplopia can be reduced by
change in head posture.
(c) Diplopia disappears with closure
of any eye.
(d) Diplopia is most marked in
recent cases that passes off
with time
False projection The child can not locate the object No false projection
in the space.
Diminished vision Generally there is no reduction The squinting eye may have
of vision that can be related to diminished vision
squint.
Systemic symptoms 1. Nausea, vertigo due to confusion Not Present
2. Symptoms of primary cause may
be present NIL
3. Other neurological deficit may be
present NIL
Movement Ocular movements are absent or Movements are normal
restricted in the direction of
action of paralysed muscle
Angle of squint 1. There is change in angle of squint Angle of squint is constant
in various direction of gazes. in all direction.
2. Secondary deviation is greater Secondary and primary
than primary deviation. deviation are equal.
Afferent path and Intact Faulty
nerve centres
Efferent path Faulty Normal
ARC Generally absent Common
Amblyopia May not develop
if the squint disappear Common
Cyclovertical deviation More common Less common
4. The above test leaves with only diagnosis i.e. concomitant squint that should be
examined under following heads :
Cover test
This is most important but often neglected test. It differentiated between
(I) Pseudo and true squint, (II) Uniocular and alternate squint and (III) Paralytic and non
paralytic squint.
It should be done for distance and near.
Measurement of angle of squint
(i) Hirschberg test (Corneal reflex test). The child keeps both eyes open and fixes a
distant object with normal head posture.
A bright pin-point light is directed on the squinting eye from 33 cm distance and posi-
tion of the image of the light is noted. In a normal eye, it should be centred over the pupil. In
squinting eye, the image is formed opposite the direction of squint i.e. if the eye is deviated
medially the reflex will form on temporal side of the pupil. Each 1 mm decentering denotes
7 or 14 PD of squint.
Besides manifest squint there are some causes of decentred corneal reflex.
They are :
1. Angel kappaA positive angle kappa may look like an exodeviation and a negative
angle kappa may look like esodeviation.
2. In case of eccentric fixation in an amblyopic eye the light reflex remains decentred
upon covering the second eye.
3. Ectopic macula
The corneal light reflex test is done for near also.
(ii) Krimsky test (Prism reflex test). This is a better test than Hirshberg test, but
Hirshberg test is only the first part of this test. In this test prism of increasing power are
placed before the eye until the light reflex is centred in the deviating eye. The child should be
able to fix a light with the dominant eye. It is not influenced by eccentric fixation. However
there may be a confusion in presence of large angle kappa. The observer should place himself
exactly in front of the child to avoid parallax from the prism. The test is suitable even in
children with poor vision in one eye.
(iii) Perimeter test. This is good only for grown up children. The child sits on a Listers
perimeter with chin on the chin rest and fixes the central white dot. The other eye is allowed
to deviate. A torch light is moved on the arc of the perimeter from periphery to the centre until
the light reflex is centred on the deviating eye. The position of the torch is read on the arc of
the perimeter. This gives the angle of squint in degree, which is half of prism dioptre.
(iv) The synoptophore test. This is the most accurate method to measure angle of
squint for distance and near in all direction of gaze, objective and subjective angle, angle of
anomaly. The synoptophore also gives away presence of ARC and its type, grades of vision,
measurement of range of fusion after image testing and AC/A.
It can be used for therapeutic use also in :
1. Amblyopia treatment with Haidinger brush.
2. Management of suppression
3. Treatment of ARC
4. Fusional exercises
In manifest squint, simultaneous macular perception is absent. There may be a
suppression scotoma. The size and position of which varies according to type of squint. In
esotropia, a small suppression scotoma is seen on nasal side of the disc, may involve macula. In
exotropia, a large suppression scotoma is seen on temporal half of the retina including the
fovea.
In uniocular squint, the scotoma is constant in the squinting eye, may lead to strabismic
amblyopia. In alternate squint the scotoma alternates between the two eyes.
Binocular fusion may be absent in concomitant squint so is the stereopsis.
The stereopsis can be tested on various types of stereoscopes.
Test for suppression :
(i) Worth four dot test
(ii) Synoptophore
(iii) Four dioptre prism test
(iv) Bagolini striated glass test
(v) Red glass test
Test for abnormal retinal correspondence
1. Bagolini striated glass test
2. Synoptophore
3. After image test
Horizontal concomitant squint
Classically horizontal squints are divided in -
1. Simple horizontal squint
(a) Esotropia
(b) Exotropia
2. Horizontal squint associated with vertical deviation
(a) Esotropia with (i) Inferior oblique over action
(ii) Dissociated vertical deviation
(iii) AV pattern
(iv) Superior rectus under action
(b) Exotropia (i) AV pattern
Esotropia
Out of many causes of esodeviation in children, esotropia is commonest. It could be
congenital or acquired. It can be unilateral or alternate. Deviation may vary for distance
and near.
Other causes of esodeviation are
10
Esodeviation
Esophoria Esotroria
Acquired Congenital
2. Accommodative esotropia
3. Non accommodative esotropia
4. Consecutive esotropia
5. Lateral rectus palsy
6. Entrapment of medial rectus
7. Microtropia
8. AV pattern
9. Cyclic esotropia
10. Uniocular loss of vision
11
Congenital
esotropia
Congenital
syndrome
1. Mobius
2. Duanes
3. Strabismus fixus
4. Congenital lateral
rectus palsy
True esotropia Pseudo esotropia
Clinically esotropia has also been classified according to its relation with accommodation,
on the basis of this esotropia can be
Esotropia
Non accommodative Partial accommodative Accommodative
Refractive Non refractive
Less
accommodation
More
accommodation
I. Essential
(a) Infantile (Congenital)
(b) Acquired
(c) Late on set
Essential non accommodative
esotropia can be
(i) Convergence excess
(ii) Divergence deficiency
(iii) Basic
II. Acute concomitant
III. Consecutive esotropia
IV. Microtropia
V. Cyclic esotropia
Non accommodative Partial accommodative Accommodative
Essential Microtropia Cyclic esotropia
Congenital (Essential infantile)
12,13
esotropia
This is an important type of esotropia seen under six months of age. The importance
lies in the fact that it causes permanent loss of binocular vision if not treated in time and the
next important factor is that many a times early onset retinoblastoma may present as esotropia
in an infant. Hence all children with real esotropia should be examined by ophthalmologist.
Characteristics of infantile esotropia
14
1. It is rarely present at birth. It develops between two to six months.
2. It is not possible to predict which child will develop esotropia.
3. It can be hereditary.
4. It is uniocular, may show alternate fixation.
5. Angle of squint is always large i.e. 30-40 PD.
6. The deviation is equal for far and near.
7. The child has a tendency to cross fixate i.e. the child fixes the left field with right eye
and right field with left eye. This sometimes gives an impression of bilateral lateral
rectus palsy. In fact inspite of being non paralytic squint, adduction is stronger than
abduction in infantile esotropia.
Abduction can be demonstrated by :
(i) The fixing eye is covered, an object of interest - a feeding bottle which is the most
interesting object to an infant is moved in front of the squinting eye from medial
to lateral. The chances are that the child will follow it to full abduction position.
(ii) If this fails the fixing eye is occluded for few hours. The child may develop abduc-
tion or it may take two to three days of occlusion of the fixing eye.
(iii) A feeding bottle is kept straight ahead of the child, both eyes are kept open. The
child is allowed to fix it, then as the child fixes it, obviously with the fixing eye
the head of the child is rotated quickly in the vertical axis i.e. to the right and
then to the left. This will invariably demonstrate presence of abduction.
(iv) Stimulating oculo vestibular reflex - The child is held by the examiner at an
arms length, face to face. The examiner rotates himself by 360 holding the child
in the same position. The child will be observed to have conjugate deviation. The
rotation is than repeated in the opposite direction.
8. Dissociated vertical squint is common. It generally develops by two years. It is a
bilateral condition. The eyes deviates up and rotates outward under cover. As soon as
the cover is removed the eye drift down and rotated medially. DVD may develop long
after successful surgery as well.
9. Inferior oblique over action - There is decrease of convergence in up gaze showing
V pattern (This is absent in DVD). The condition is unilateral to begin with but may
become bilateral. It is also a common feature after successful surgery and the parents
should be informed and forewarned about this. Fortunately most of the parents fail
to notice it. When present it requires a separate surgery.
10. Unilateral superior oblique paresis
15
may be present. The incidence is less than infe-
rior oblique over action and DVD.
11. Nystagmus
16
may be latent or manifest, resulting in abnormal head postureTo
locate null point of nystagmus, to get better vision, convergence may be utilised to
reduce nystagmus.
12. Vision is generally good. Amblyopia is common but may not develop in an alternator.
13. Refractive errorThe child has low degree of hypermetropia. Refraction should
always be tested under atropine.
14. There is no neurological defect.
Differential diagnosis consists of accommodative squint, unilateral abducent palsy,
Duanes retraction syndrome, high AC/A ratio, nystagmus blocking syndrome, unilateral blind
eye, incomplete Mobius syndrome, albinism, cerebral palsy.
Variants
1. Congenital esotropia syndrome consists of esotropia with other muscle malfunc-
tion i.e. SO over action, DVD, V syndrome etc.
2. Ciancia syndrome consists of
(a) Esotropia
(b) Bilateral abduction defect
3. Manifest nystagmus that is latent.
4. Abnormal head posture
5. Langs syndrome. This consists of :
(a) Torticollis
(b) DVD
(c) Nystagmus
(d) Excyclodeviation
(e) Early onset esotropia
Management
Management is essentially surgical. However before embarking upon surgery :
1. Any error of refraction should be corrected. Commonest error of refraction is low
grade of hypermetropia. Refraction should always be done under atropine with usual
precaution. If error of refraction turns out to be more than +3D, an accommodative
factor should be considered. It is better to make the child slightly myopic.
2. Management of amblyopia by standard method.
I. ConventionalFull time complete occlusion by eye pad of the fixing eye. The
duration should be 1 day for every year between two years to six years. Upto two
years 2 days of occlusion is done.
II. Alternate occlusionOne day of occlusion of amblyopic eye i.e. in a three year
child, the fixing eye is occluded for 3 days and on the fourth day the amblyopic
eye is similarly occluded. In a four year child, it should be four to one and in a
five year child, it should be five to one.
Occlusion should be continued :
(i) Until vision has been equalised in both eyes.
(ii) Optimal vision for the eye has been achieved. Sometimes an eye has to be occluded
for three to six months.
(iii) There is no further improvement of vision in spite of sufficient occlusion.
Frequency of follow up depends upon age of the child. Younger the child more frequent
should be the follow up.
6 months Every 3 days
1 year Every week
2 years Every two weeks
The visual acuity should be checked on every visit.
3. Miotics. Some children may respond to local miotics. Miotics should be used only
when some binocularity has been reached. However children with non accommodative
esotropia respond poorly with miotic. Commonly used miotic is phospholine iodide
0.03% to 0.125% once a day. If surgery is required, the anesthetist should be told
about use of miotic during pre anaesthetic check up. The miotics are stopped for two
three weeks
17
before general anaesthesia is administered especially if succinyl choline
is to be used to intubate the child, otherwise other muscle relaxtants that do not
depend upon body choline esterase may be used.
18
4. Surgery is the definitive treatment of congenital esotropia. Sooner it is done better
are the results, but vision should be improved, alternation should be achieved and
amblyopia should be managed before surgery.
19
Chances of development of DVD and
inferior oblique over action after 2 years are positive hindrances. The parents should
be told about possibility of binocular surgery and repeat surgery before planning the
surgery. Basically there are two groups of surgeries for congenital esotropia :
(1) Bilateral equal recession of medial rectus
(2) Recession one medial rectus with re-section of lateral rectus on the same eye.
5. Botulinum toxin (Botox) injection in the medial rectus - The injection is given under
general anaesthesia under electromyographic control in the medial rectus. A common
needle used to inject botox also acts as electrode for EMG. Effect of injection starts
within twenty four to forty eight hours and lasts for two to three weeks after which
the injection may be repeated. The injected muscle is paralysed and is said to be
elongated, while its antagonist contracts. Drawbacks are cost of injection, its
unpredictability and systemic toxicity. It should be given only by a person especially
trained.
Other uses of botox in ophthalmology are :
1. Paralytic sqint (single muscle) injection is made in the direct antagonist
2. Cyclic esotropia
3. Thyroid ophthalmopathy
4. Ptosis
5. Essential blepharo spasm
6. Exotropia
20
Acquired esotropia
Acquired non paralytic esotropia is commonest type of esodeviation seen in children.
They have been classified in different ways -
1. On clinical presentation
2. Etiological basis
The second seems to be better than the first. It divides acquired esotropia into -
(i) Primary
(ii) Secondary and consecutive (They are far less in number)
The primary or the common variety is divided into -
(a) Accommodative
(b) Non accommodative
(a) Accommodative esotropia can be fully accommodative with normal Ac/A or high
Ac/A ratio. However many of the accommodational esotropias may be partially accommoda-
tive.
(b) Non accommodative esotropia can be
Basic type
Convergence excess type
Esotropia in myopia
Microtropia
Noorden
21
1990 gave a comprehensive classification
Acquired esotropia
Consecutive Secondary
Uniocular loss
of vision in a child
Surgical over
correction of
divergent squint
Non accommodative
Convergence
Excess
Myopic Basic
Fully
accommodative
Partially
accommodative
Accommodative
Accommodative esotropia
Accommodative esotropia is one of the commonest forms of ocular deviation seen in
children. Its diagnosis is simple and treatment is effective. This is a type of manifest squint
that can be treated mostly by optical methods. However those who present late may have to
undergo surgery and anti amblyopic treatment.
Characteristics of fully accommodative esotropia
1. Age of onsetIt is seen between six month to six years. Average age of presentation
being 2 years.
2. HeredityThe condition has strong hereditary background. It may be present in
parents, near relatives or in siblings.
3. DeviationOcular deviation is
(i) Uniocular
(ii) Variable greater for near than distance
(iii) IntermittencyThe child may have intermittent esotropia that may become con-
stant.
(iv) Angle of deviation is not very large, it ranges between 20PD to 45 PD.
(v) The child does not alternate.
(vi) There is no cross fixation.
4. Amblyopia is common but not deep.
5. Error of refraction is typically hypermetropic ranging between +4D and +7D. The
squinting eye is more hypermetropic than the other.
6. AC/A is normal i.e. between 3D-5D.
7. Near distance relationship is either normal or when different it is not more than 10
PD.
8. The deviation is fully corrected by spectacles and the eyes remain straight, hence do
not require surgery.
9. Binocularity is present due to anomalous retinal correspondence.
10. Children with higher hypermetropia more than 8D generally do not develop ac-
commodative squint.
22
They have bilateral amblyopia. Most probably they give up
accommodation. These children do not improve with glasses.
23
11. Spontaneous recovery with reduction of hypermetropia is possible, the condition may
pass into partially accommodative or non accommodative squint.
24
12. There may be associated DVD or inferior oblique over action.
13. Binocular single vision is possible for far and near when hypermetropia is fully
corrected.
Etiology of accommodative esotropia
There are two factors that lead to accommodative esotropia i.e.
1. Uncorrected hypermetropia
2. Abnormally high Ac/A ratio.
1. Hypermetropia. An emmetropic child needs no accommodation for distance vision,
a myopic eye becomes worse by accommodation. It is only hypermetropia that is relieved by
accommodation.
These children with accommodative esotropia generally have an uncorrected
hypermetropia of moderate degree. The child uses excessive accommodation to overcome this
hypermetropia. This by itself does not explain the complete mechanism. The second part of the
mechanism is in sufficiency of fusional divergence. So long fusional divergence is sufficient,
the child does not develop manifest squint. An occassional esophoria may result. This may
lead to intermittent esotropia. Once the fusional divergence breaks down esotropia results.
2. Abnormally high Ac/A ratio. Normal Ac/A ratio is 3D-5D. It can be measured by
following methods
25
:
1. Heterophoria method
2. Gradient method
3. Graphic method.
Management of accommodative esotropia
Optical
A. The first line of management is prescription of hypermetropic correction for constant
use with correction of associated astigmatism. Glasses should be prescribed after
refraction under 1% atropine only. Children under four years are given full cycloplegic
correction. If a child older than four years is prescribed full cycloplegic correction, the
child may not tolerate it. They are generally given as much plus lens possible that
keeps the eyes straight and vision clear. The power of the plus lenses may be required
to be reduced over years.
B. Fully hypermetropic correction for distance generally keeps the eyes straight for
distance as well as near. In some children there is some esodeviation for near. In such
situation the first step is to see if the distant correction has been fully corrected or
not. This should follow repeat refraction under atropine. If it is found that the child
has been given full correction at the first instance, the second step is to give bifocals.
The near power should be just sufficient to keep the near axis straight. The best
bifocals for children are executive type where the junction of far and near correction
pass across the pupil.
Miotics
The next alternative is use of miotic. All children do not respond equally well to miotic
therapy. About 10% may not improve, some may show poorer result than bifocals.
The beneficial effect of miotics should be weighed against the local and systemic side
effects of miotics.
Orthoptic treatment
Orthoptic treatment is recommended in older children with hypermetropia less than
+3D.
Surgery
Surgery is not required for horizontal deviation but DVD and inferior oblique over action
requires surgical management.
Accommodative esotropia with high Ac/A
In case of fully accommodative esotropia Ac/A is within normal limits. There are some
children who have high Ac/A. They have more esodeviation for near than distance. Some may
not even squint for distance.
26
They may have various types of error of refraction that range
between low hypermetropia to mild myopia, even emmetropia in some cases. Chances of
high Ac/A are more when cycloplegic refraction shows low hypermetropia, myopia or
emmetropia. High hypermetropes have more or less normal Ac/A. The condition is not fully
corrected by glasses, some esotropia is left uncorrected.
Management consists of correction of any error of refraction and improvement of vi-
sion for distance. Adding plus lenses for near deviation either as bifocal or clipon near correc-
tion. While adding near correction minimal addition that corrects near deviation is prescribed.
The bifocal should be executive type, the upper border of the near correction should divide the
pupil in two equal parts horizontally. Non correction of near deviation by bifocals is strong
indication of non accommodative factor that can be corrected by surgery only.
Miotics can also be given for near esodeviation.
Partial occlusion and orthoptic exercises help to overcome amblyopia and help
increase amplitude of divergence fusion.
Comparison between infantile esotropia and accommodative esotropia
Infantile esotropia Accommodative esotropia
Age of onset Before six months Average 30 months
Angle of deviation 35 PD-50 PD Average 20 PD
Laterality Uniocular Uniocular
Alteration Very common Nil
Cross fixation Common Nil
Refraction Moderate Hypermetropia does not
exceed +2D +4D to +7D is common
Restriction of abduction Common but no under action of No restriction of abduction
lateral rectus
Intermittency Absent May be present as intermittent
esotropia
Deviation Equal for far and near Esotropia slightly more for near
Vertical deviation DVD and inferior oblique over DVD and IO over action may
action common be present
Nystagmus Very common Absent
Amblyopia Common, Alternators do not develop May be present but not deep
Management Surgery is the treatment of choice Glasses will correct esotropia
for far and near
Partially accommodative esotropia
Partially accommodative esotropia is a mixture of accommodative as well as non accom-
modative esotropia.
There is always some esodeviation left after full spectacle correction of hypermetropia
even with bifocals and/or miotics.
There are two possible explanation for the condition :
1. The child was initially an infantile esotrope to which accommodative element has
been added as the child ages. There may be increase in hypermetropia. Final
hypermetropic refraction is more than initial hypermetropia.
2. The child had accommodative esotropia to which non accommodative element has
been added due to decompensation. Generally there is hypertrophy of medial rectus
or conjunctiva on the medial side.
The residual esodeviation is constant, it is same for near and distance. It is associated
with amblyopia.
Treatment
27
Treatment consists of surgery for esodeviation and continuation of glasses post
operatively.
Non accommodative acquired esotropias
There are three types of these esotropias
28
1. Basic type
2. Convergence excess
3. Esotropia in myopia
1. Basic type non accommodative acquired esotropias :
Characteristics :
1. This generally manifests after 6 months of age and limited to childhood.
2. It is unilateral.
3. Vision is less in squinting eye.
4. There is hardly any error of refraction.
5. There is no accommodative factor.
6. Deviation is almost same for distance and near.
7. To begin with, the deviation is small that may become very large with age.
8. Being monocular, the squinting eye develops amblyopia which requires proper
management.
9. Glasses or miotics do not abolish squint. However associated error of refraction should
be treated when present.
10. Surgical treatment is definitive treatment. Commonest surgery is bi-medial recession
with conjunctival recession.
2. Convergence excess esotropia :
Characteristics :
1. Seen between 2 to 3 years of age
2. Deviation for distance is minimal
3. Near deviation is between 20 to 40.
4. The child may have low grade hypermetropia or may be emmetropic.
5. There is no accommodative factor responsible for deviation.
6. Surgery is the treatment of choice.
Other esotropias :
1. Microtropia
2. Esotropia in myopia
3. Cyclic esotropia
4. Nystagmus blocking esotropia
5. Consecutive esotropia
6. Secondary esotropia
7. Esotropia in DVD and inferior oblique over action
8. Acute esotropia
1. Microtropia
29,30,31
. Commonest form of presentation is small degree of esotropia,
occasionally it may present as exotropia, hypertropia and rarely as orthophoric, hence it is also
called micro-strabismus. The fixation is restricted to non squinting eye, hence it is called
mono fixation syndrome.
Characteristics :
1. It is a permanent condition.
2. It may follow Congenital or infantile esotropia
Surgical correction of above
3. Angle of deviation is small8 to 10 PD.
4. Mild amblyopia is common even in spite of small angle of deviation.
5. Central fusion is absent.
6. There is facultative macular scotoma that is made obvious on Worth four dot test
and Bagolinies test.
7. Cover uncover may show small esotropia that increases during prism cover test.
8. Sometime 4 prism diopter base out test may be positive.
9. The condition is more common in anisometropic amblyopia.
10. There is para foveal fixation, peripheral fusion.
11. Harmonious ARC.
12. Almost normal stereo acuity.
Management :
Mono-fixation syndrome can be treated by orthoptic treatment or surgery. Microtropia
persists even after orthoptic treatment, it may strength peripheral fusion.
2. Esotropia in myopia. Myopic children have good near vision and poor distant vision.
In small children onset and progress of myopia is so gradual that the child does not realize his
poor distant vision unless pointed out.
In moderate uncorrected myopia, the near vision is either within near point or near to
it. Accommodation worsens myopia. The child is only left with one option i.e. to converge to
have clear vision. The child may altogether give up any effort for clear distant binocularity,
retain binocularity for near and convergent squint.
In older children who have acquired myopia, fusional deficiency of divergence enhances
convergence and esotropia.
The treatment consists of correction of myopia and surgical correction of esodeviation.
3. Cyclic esotropia
32,33
. This is an uncommon esotropia of unexplained origin. Generally
seen in children between three to four years. It is most often mistaken as intermittent esotropia.
There is a fixed cycle of esotropia and orthophoria appearing with a fixed gap that ranges
between 24 hours to 96 hours. Typical presentation is that the child has a large angle esotropia
for a day or two followed by a gap of same duration of orthophoria (non squinting days). Such
episodic attacks last for few months to two years before the condition becomes constant for all
days. Initially there is no amblyopia, amblyopia develops when the squint becomes constant.
See chapter 17 page 608.
Treatment consists of treatment of amblyopia when present and surgical correction.
4. Nystagmus blocking esotropia. Children with congenital esotropia are known to
have nystagmus either as manifest nystagmus or manifest latent nystagmus independent of
esotropia. In some children who have nystagmus in straight eye, the nystagmus disappears if
the eye is adducted. This is called nystagmus blocking esotropia. This is often confused as
infantile esotropia with nystagmus. The treatment is recession of both medial recti with myopexy
behind the equator. See page 633.
5. Consecutive esotropia. It is common for a child to develop small degree esotropia
following surgery for exodeviation. This is transient and associated with diplopia. However if
esotropia persists after few weeks, it should be evaluated and treated. Possibility of amblyopia
should be kept in mind and managed.
EXOTROPA
34
Exotropia is manifest deviation of any of the eyes outward.
Normal position of the eye at rest is slight exodeviation. This is so small that it is taken
as physiological and the eye is considered to be orthophoric.
General features of exotropia-
1. Exotropia can be congenital or acquired.
2. It can be uniocular or alternate.
3. To begin with most of the exotropias are exophoric. However an eye can be exotropic
without going through the phase of exophoria.
4. It can be equal for far and near, it can be greater for distance and lesser for near or
vice versa.
5. The angle of deviation may increase with passage of time.
6. Exotropias are generally large degree of deviation.
7. Associated errors of refraction are generally of small degree and there is no predilec-
tion for any specific type of error of refraction.
8. Suppression is common but amblyopia is less frequent.
9. An exotropic eye generally have poor adduction that may be diagnosed as medial
rectus paresis. In such cases adduction can be demonstrated by patching the other
eye for few hours.
10. Exotropia is more common among girls. There may be a hereditary basis.
There are various modes of development of exotropia. Commonest mode is as follows :
OrthophoriaExophoriaIntermittent exotropiaConstant exotropiaIncrease in
exotropia.
The other less frequent possibility is esotropia passing into straight eye and then
exotropia.
The third possibility is consecutive exotropia where there is surgical over correction of
esotropia.
Lastly, secondary exotropia develops in eyes that have long standing poor vision with
amblyopia in one eye due to - anisometropia, uncorrected unilateral aphakia, unilateral cataract,
corneal opacity, macular scar, optic atrophy.
It has been observed that uniocular loss of vision under five years of age may produce
both esotropia or exotropia. Esotropia is more common. After five years chances of exotropia is
far more than esotropia.
35
Various combinations of exotropia
Distance Near Symptoms
1. Exotropia Orthophoria Nil
2. Intermittent exotropia Orthophoria or exophoria for near Symptoms for distance
3. Exotropia Exotropia Suppression scotoma for
distance
4. Exotropia Intermittent exotropia Binocular vision for near
5. Exotropia Exotropia Loss of binocular vision
Classification of exotropia
Exotropia
Pseudo
exotropia
True
exotropia
Congenital Acquired
Secondary Consecutive Primary
Mixed Convergent
weakness
Divergent
excess
Paralytic Restrictive
Motor obstacle Sensory obstacle
Cyclovertical
deviation
Defective
convergence
Orbital
anomaly
Uniocular error
of refraction
Uniocular loss
of vision
Pseudo exotropia
An exotropia is called essential when it is
1. Primary concomitant
2. There is no binocularity
3. There is no neurological defect
4. There is no anatomical defect
5. There is no detectable cause of diminished vision i.e. opacities in media, defect in
conduction, unilateral high error of refraction.
Pseudo exotropia
In this case the eye/eyes seem to diverge due to any of the following causes-
Telecanthus, positive angle kappa, temporal drag of the macula as in retinopathy of
prematurity.
On Hirschberg test, the corneal light reflex is central. The cover test does not show any
deviation. Pseudo exotropia unlike pseudo esotropia does not disappear with age.
Congenital exotropia
33,35,36
Incidence of congenital exotropia is less than congenital esotropia. The exotropia be-
comes obvious by two months of age. The angle of deviation is constant for distance as well
as near. The deviation ranges between 40 to 80 PD. There is no cross fixation. The child has
homonymous fixation i.e. the child fixes right field by right eye and left field by left eye.
This increases temporal field of vision. Alternation is common hence amblyopia is less
frequent. DVD and nystagmus may be seen in some cases. Error of refraction when present
are of small degree.
Management
The aim of the treatment is to bring the alignment within 10 PD before two years.
Surgery is the treatment of choice. However if there is error of refraction more than 3D
hypermetropia or 2D myopia, it should be corrected before surgery.
Primary acquired exotropia
Intermittent exotropia
37
Intermittent exotropia is the commonest type of exotropia and second most com-
mon squint in children. The child has exotropia especially when the child is tired or day-
dreaming. A child may be brought with history of exotropia but on initial examination he may
seem to be orthophoric and requires repeated examination in the same sitting to unmask the
deviation. Sometimes an intermittent exotropia may resolve spontaneously.
37
In about 75% cases the deviation does not change over years but in a small group of
cases the deviation may increase. Generally the condition becomes manifest by four to five
years of age but not before two years.
The child generally has good vision or small error of refraction with normal binocularity
in primary position. When the eyes are straight there is good stereopsis, bifoveal fusion without
suppression. Facultative suppression develops only when the eye deviates. This prevents
diplopia.
The child himself may not be aware of deviation, looking at a mirror corrects the devia-
tion. However presence of exotropia may become obvious in a photograph. Some children may
complain of blurred vision and/or asthenopia. The parents may complain that the child closes
one eye in bright light. This phenomenon may persist even after surgical correction.
Both A and V pattern are met with, however V pattern is more common in intermittent
exotropia. DVD and nystagmus are less frequent than in esotropia.
Clinically it can be divided into :
1. Divergence excess exotropia
2. Convergence deficient exotropia
3. Basic exotropia
4. Pseudo divergence
Divergence excess type of exotropia
The exotropia is more when measured for distance. It is better to measure distant
deviation when the child is fixing beyond usual six meters. The difference between far and
near is more than 15 PD. The near deviation may occasionally be exophoria. If the
accommodation is relaxed by putting +3.00D sph. in front of each eye and near distance is re-
measured, there is generally no difference from the original deviation.
Convergence deficient exotropia-
The near exotropia is more than distant exotropia at least by 15 PD with full correction.
It may be present with diminished accommodation as in myopes who need not accommodate or
rarely in hypermetropes more than 6 Dsph who give up accommodation. They may have low
Ac/A ratio.
Basic exotropia
Here exotropia for far and near are equal or with in 15 PD.
Pseudo divergence (Simulated divergence excess)
The distant exotropia is greater by 15 PD than near as in divergence excess type when
examined for first time. This difference decreases to less than 10 PD after half an hours patching
of one eye. The measurement should be taken immediately before fusion develops. The other
diagnostic test is to measure near deviation with +3.00 Dsph in front of both eyes. The near
deviation becomes as much as or greater than distant deviation. The causes of this phenomenon
is not understood.
Management
Treatment is indicated only if the intermittency is replaced by constant exotropia. The
mode of management depends upon age of the patient and severity of symptoms.
Treatment consist of
1. Optical
(a) Glasses for any significant error of refraction. Myopia is fully corrected and
hypermetropia is under corrected.
(b) PrismBase in prisms are given for short time before or after surgery. They
give better results in small deviation and have hardly any effect on large degree
of exotropia.
2. Orthoptics are given in convergence deficient exotropia to strength convergence
and eradicate suppression.
3. SurgicalSurgery is the definitive treatment. It is better to operate round about
four years of age. Chances of suppression increase after four years and visual prognosis
is not good.
In small degree squint orthoptics is preferred over surgery. The aim of surgery is to
produce small degree of esotropia.
In divergence excessRecession of both lateral recti is done to clear distant exotropia.
This results in slight esotropia.
In basic exotropiarecession of lateral rectus with resection of medial rectus is required.
If there is recurrence of exotropia, it should be corrected by base in prism, myopia is
over corrected, hypermetropia is under corrected by one dioptre. Addition of 1D stimulates
accommodative convergence.
Consecutive exotropia
This develops following over correction of esotropia especially when esotropia was
variable, and partially accommodative. This is common in esotropic eyes with uniocular high
hypermetropia. Time lapse between surgery and development of consecutive esotropia is
variable. It may develop soon after surgery or may develop months to years after surgery.
Sometimes consecutive exotropia develops spontaneously in an esotropic child with amblyopia.
Management consists of under correction of hypermetropia, prescription of base in prisms
or re surgery for cosmetic purpose.
Secondary exotropia
This is generally sensory exotropia due to long standing visual loss in one eye. The
causes may be - amblyopia, anisometropia, either as high error of refraction in one eye or
uncorrected uniocular aphakia, corneal opacity, unilateral dense cataract, maculopathies or
optic neuropathies. If diminished vision sets in after five years of age, exotropia is the rule.
Before five years the deviation can be either eso or exo.
Mechanical factors like scarring of medial rectus following accidental or surgical trauma
or paralysis of adductors mostlymedial rectus pareses can cause secondary exotropia.
Convergence paralysis
This is an acquired form of near esotropia due to CNS involvement as in Parinauds
syndrome. Convergence paralysis is associated with palsy of up gaze. It can be seen in
encephalitis. In adults it may be caused by multiple sclerosis or neurosyphilis.
On examination the convergence is greatly reduced with intact accommodation and
miosis. Fusional convergence is invariably poor.
Management consists of treatment of primary cause and prisms.
A-V syndrome
38,39
It is a common observation that the eyes diverge slightly on vertical gaze and is consid-
ered to be physiological in nature. However in some cases of squint both eso or exodeviation,
the divergence is prominent not only on up gaze but also in down gaze. In some cases there is
no deviation in straight gaze but have exotropia on up gaze and equal amount of exotropia in
down gaze. Similarly still less number of children may show exotropia only on up or down
gaze.
The conditions have been named after alphabets A, V, X or lambda (inverted y ) due
to their imaginary symmetry with these letters.
Out of above five condition, the patterns A and V are most common, about 20% of all
horizontals squints are associated with A or V pattern.
The exact cause of the condition is not known. There are three schools of thoughts for
three different causative factor i.e.
1. Horizontal school
2. Vertical school
3. Oblique school
The horizontal school of thinking is based on the assumption that the fault lies in
horizontally acting muscles only.
The vertical school of thought is built on presumption that the A and V patterns are due
to symmetrical underaction of vertically acting muscles.
The third group believes that both the horizontal and vertically acting muscle are at
fault.
As there is no single opinion as far as cause of the condition, the mode treatment also
vary widely.
Classification
A esotropia/
phoria
A exotropia/
phoria
V esotropia/
phoria
Exotropia Exotropia Exotropia
A V X Y l
Vexotropia/
phoria
Phenomenon
The following figures roughly explain A and V pattern :
V
Exotropia more
on up gaze
Esotropia more
on down gaze
A
Esotropia more
on up gaze
Exotropia more
on down gaze
Presentation
A child may not be aware of these patterns even in presence of manifest squint and the
symptoms are attributed only to horizontal deviation.
Sometime A exotropia or V esotropia cause discomfort for near work. A esotropic generally
has a chin up position while A exotropic keeps the chin down. The former may have mongoloid
slant of inter palpebral fissure. The later has anti mongoloid slant.
Diagnosis
A case of V esotropia may be mistaken as convergence excess due to accommodation and
a V exotropia may be mistaken as divergence excess.
To avoid this the measurement should be taken not only on straight gaze but also at 25
up and 25 down gaze and deviation measured by prism bar and cover test. The
measurements should be done with full correction whenever needed.
In V pattern the difference should be at least 15 PD and for A pattern 10 PD difference
is sufficient for diagnosis.
A-V syndrome may be associated with dysfunction of obliques, DVD or cranio facial
anomalies.
Treatment of AV syndrome consists of management of error of refraction, elimination of
amblyopia, correction of horizontal deviation by surgery with appropriate modification of placing
of horizontal insertion or modification of action of obliques.
For A esotropia
Without oblique dysfunctionBilateral recession of medial rectus with upward shift of
the insertion or recession of medial rectus with shift and resection of lateral rectus with shift
downwards.
If there is associated superior oblique over action either both medial recti are recessed
or recession of one medial rectus and resection of lateral rectus. Both the above procedures are
done with weakening of superior oblique.
A exotropia with normal oblique action - Bilateral recession of lateral rectus with down
shift.
A exotropia with superior oblique over action is treated by recessing both lateral recti
and weakening superior oblique.
V esotropiaRecession of medial rectus with downward shift of insertion.
V ExotropiaRecession of lateral rectus shift upward.
Oblique may have to be tackled along with horizontals.
Care should be taken not to operate more than three muscles in the same eye in single
sitting to avoid ischemia of anterior segment.
REFERENCE8
1. Lyle T.K., Wybar K.C. ; Development of binocular vision in Lyle and Jacksons Practi-
cal Orthoptics in the treatment of squint. First Indian edition, p-4759, Jay Pee
brothers, New Delhi, 1994.
2. Dutta L.C. ; Physiological binocular vision in Ophthalmology principle and practice.
First edition, p-533536, Current Book International, Calcutta, 1995.
3. Dutta H. ; Abnormalities of binocular vision in Strabismus. First edition, p-2122, Jay
4. Rohatgi J.N. ; Abnormal retinal correspondence in Squint basic and clinical aspect.
p-7377, CBS Publishers, New Delhi, 2003.
5. Agarwal L.P. ; Viscuoscope in Optics and refraction. Second edition, p-209210, CBS
6. Agarwal L.P. ; Heterophoria in Essentials of Ophthalmology. First edition, p-393
398, CBS Publishers, New Delhi, 2000.
7. Vaughan D. and Asbury T. ; Hyperphoria in General ophthalmology. Tenth edition,
p-192193, Lange Medical Publication, California, 1983.
8. Miller S.J.H. ; Kinetic strabismus in Parsons disease of the eye. Seventeenth edition,
p-289, Churchill Livingstone, London, 1984.
9. Virginia Carlson Hansen ; Duanes classification of horizontal strabismus in Ocular
motility. First Indian edition, p-35, Jay Pee Brothers, New Delhi, 1989.
10. Manley D.R. ; Classification of esodeviation in Pediatric ophthalmology. Vol. I, sec-
ond edition, p-227228, Edited by Harley R.D., WB Saunders Company, Philadelphia,
1983.
11. Lang J. ; Microtropia Arch. Ophthal. 81: 758, 1969.
12. Buckley E.G. ; Congenital esotropia in Current Ocular therapy. Fifth edition,
p-391392, Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Company, Philadel-
phia, 2000.
13. Diamond G.R. ; Congenital esotropia in Text book of ophthalmology. Vol. 5, p-8.1,
Edited by Podos S.M. and Yanoff M., Mosby, London, 1991.
14. Manley D.R. : Congenital esotropia in Pediatric ophthalmology. Vol. I, Second edi-
tion, p-228229, Edited by Harley RD, WB Saunders Company Philadelphia, 1983.
15. Dutta H. ; Esotropia in Strabismus. First edition, p-7476, Jay Pee Brothers, New
Delhi, 2004.
16. Fiona Rowe ; Congenital/infantile esotropia in Clinical orthoptics. First edition,
p-99100, Blackwell Science Ltd., Oxford, 1997.
17. Deborah Pavan Langston : Accommodative esodeviation in Manual of ocular diagnosis
and therapy. Third edition, p-310311, Lippincot Williams and Wilkins, Philadelphia,
1991.
18. Dy Santiago Alvina Pauline ; Rosen Baum A.L., Esotropia, High accommodative con-
vergence to accommodation in Current ocular therapy. Fifth edition, p-400401, Ed-
ited by Fraunfelder FT and Roy FH, WB Saunders Company, Philadelphia, 2000.
19. Ing MR ; Early surgical alignment for congenital esotropia. Tr. Am. Oph. Soc. 79:625-
663, 1981.
20. Eggers H.M. : Non surgical treatment exotropia in Text book of ophthalmology
strabismus and pediatric ophthalmology Vol. 5. Vol. 5, p-77, edited by Podos S.M.
and Yanoff Mosby, London, 1991.
21. Noorden GK ; Binocular vision and ocular motility. 4th edition, CV Mosby, St. Louis
1990.
22. Sharma P. ; Accommodative esotropia in Strabismus simplified. First edition, p-93,
23. Eugene R. Folk : Accommodative esotropia in Principles and practice of ophthal-
mology. Vol. III, First Indian edition, p-6-27, Edited by Peyman GM, Sanders DR and
Goldberg MF, Jay Pee Brothers, New Delhi, 1987.
24. OHalloran H.S. ; Accommodative esotropia in Current ocular therapy. Fifth edition,
p-386387, Edited by Fraunfelder FT and Roy FH, WB Saunders Company, Philadelphia,
2000.
25. Fiona Rowe : Method of measurements of Ac/A ratio in Clinical Orthoptics. First
edition, p-58, Black Science, Oxford, 1997.
26. Mohan K. ; Non refractive esotropia in Clinical practice in ophthalmology. First
edition, p-536, Jay Pee Brothers, New Delhi, 2003.
27. Kenan J.M., Willshaw H.E. ; The outcome of strabismus surgery in childhood esotropic,
Eye : 7:341-345, 1993.
28. Rohatgi J.N. ; Concomitant convergent squint in Squint basic and clinical aspect.
First edition, p-142145, CBS Publishers, New Delhi, 2003.
29. Park M.M. ; Mono fixation syndrome. Tr. Am. Oph. Soc. 67:609-657, 1969.
30. Lang J. ; Microtropia. Arch. Oph. 81 : 758, 1969.
31. Wilson M.E. ; Mono fixation syndrome in Current ocular therapy. Fifth edition,
p-406407, Edited by Fraunfelder FT, Roy FH, WB Saunders Company, Philadelphia,
2000.
32. Burian H.L.M. ; Cyclic esotropia in strabismus ophthalmic symposium, edited by
H. Allen C.V., Mosby, St. Louis, 1958.
33. Manley D.R. : Cyclic esotropia in Pediatric ophthalmology. Vol. 1, second edition,
p-233240, Edited by Harley RD, WB Saunders Company, Philadelphia, 1983.
34. Burian H.L.M. ; Exodeviation, their classification, diagnosis and treatment. AJO. 62 :
1161-1166, 1966.
35. Eggers H.M. ; Exotropia in strabismus and pediatric ophthalmology. Text book of
ophthalmology. Vol. 5, First edition, p-717.13, Edited by Podos SM and Yanoff M.,
Mosby, London, 1991.
36. Moore, S., Cohen R.L. ; Congenital exotropia, AJO 35 : 68-70, 1985.
37. Steinkuller P.G. ; Intermittent exotropia in Current ocular therapy. Fifth edition,
p-404405, Edited by Fraunfelder F.T., Roy F.H. WB Saunders Company, Philadelphia,
2000.
38. Rohatgi J.N. ; A and V syndrome in Squint Basic and Clinical aspect. First edition,
p-164173, CBS Publishers, New Delhi, 2003.
39. Lyle, T.K. ; A and V phenomenon pattern or syndrome in Practical orthoptics in the
treatment of squint. First Indian edition, p-397405, Jay Pee Brothers, New Delhi,
1994.
673
CHAPTER 20
Disorders of Orbit in Children
Orbits are two spaces surrounded by craniofacial bones on all sides except in front,
they are roughly pyramidal in shape with base anteriorly and apex posteriorly, unlike pyramids
all the four sides the walls of orbit are not equal in length nor their inclination to each uniform.
The medial wall is shortest. The medial wall of two sides are more or less parallel to each
other, distance between two medial walls is 25 mm. This is called inter orbital distance, the
lateral walls are inclined to medial wall at an angle of 45. The two lateral walls if extended
backward form an angle of 90 with each other. The point where the four walls of orbit meet
is known as apex of orbit, which is posterior-most part of orbit. The orbits lie under the
anterior cranial fossa and above the maxillary sinuses. The medial wall is mostly formed by
the ethmoid and sphenoid that separate the orbit from the nasal cavity. The lateral wall
separates the orbit from middle cranial fossa and temporalis muscle. The anterior border of
the orbit is not circular in adults. In children they are almost circular.
The base of the orbit is not the widest part of the orbit, the widest part of the orbit lies
behind the orbital rim.
1
It corresponds to the maximum diameter of the globe i.e. the equator.
The orbit is formed by seven bones of different sizes. They are frontal, zygomatic, maxilla,
lacrimal, palatine, ethmoid and sphenoid. The orbit is said to have an apex, a roof, a lateral
wall, a floor, a medial wall and rim.
The rim is formed by frontal, maxillary and zygomatic bones. The rim has been divided
into four parts - superior, lateral, inferior and medial corresponding to roof, lateral wall, floor
and medial wall respectively. The superior rim has two notches, the supra orbital notch and
supra trochlear notch. They transmit supra orbital and supra trochlear nerves and vessels.
The lateral orbital margin is strongest part of the orbit, the infra orbital margin transmits
infra orbital nerve. In fracture of the orbital rim, this nerve is invariably injured resulting in
hyposthesia of the skin lateral to the nose.
The medial margin has fossa for lacrimal sac in the inferior medial aspect. It is formed
between the anterior and posterior lacrimal crests. The lower part of the fossa narrows to
form canal for nasolacrimal duct.
The roof of the orbit is formed by orbital plate of frontal and lesser wing of sphenoid.
On the anterio lateral part is fossa for lacrimal gland and medially there is trochlear fossa.
The apex of the orbit lies in the posterior most part of the roof. The frontal lobe of the
brain and its meninges are above the roof while the periorbita LPS, RS, SO, lacrimal gland,
frontal and trochlear nerves lie under the roof. The roof is not thick and strong. Defect in the
roof can cause herniation of the brain and meninges in the orbit either as a congenital anomaly
or following trauma.
The medial wall is mostly formed by ethmoid and sphenoid, which are very thin. They
are the weakest parts in the orbit and liable for fracture. The thin wall of the ethmoid can
transmit infection from the ethmoidal sinuses, which is the most common cause of orbital
cellulitis in children. The frontal process of maxilla and lacrimal bone form a small part of
medial wall, the maxilla forms the anterior lacrimal crest while the posterior lacrimal crest is
formed by lacrimal bone. The anterior lacrimal crest is palpable in normal individuals. The
ethmoidal sinuses and middle meatus form the medial relation while the superior oblique
medial rectus, terminal part of the ophthalmic artery and nasociliary nerve pass over the
inner surface.
The floor of the orbit is formed by maxilla, zygomatic and palatine bones. The maxillary
part is very thin and common site for blow out fracture of orbit. The contents of the orbit can
get herniated in the maxilla following blow out fracture. The floor is separated from the
lateral wall by infra orbital fissure, the part anterior to this fissure is infra orbital groove. The
anterior most part is converted into infra orbital canal that opens in infra orbital foramen.
The inferior oblique takes origin from a rough area on the anterio medial part of the floor and
traverses back. This is the only extra ocular muscle that has its origin in front of the globe.
The inferior rectus, the inferior oblique, the nerve to inferior oblique lie above the periorbita.
Under the floor lies the maxillary antrum and palatine air cells.
The lateral wall of the orbit is formed by the zygomatic bone anteriorly and greater
wing of sphenoid behind. The greater wing of sphenoid separates the superior orbital and
inferior orbital fissures. The lateral wall separates temporal fossa laterally and middle cranial
fossa posteriorly from the periorbita, lateral rectus, lacrimal nerve vessels and zygomatic
nerve.
The orbit is lined by the periostium of the orbit, called the periorbita on its inner surface.
The periorbita extends from the apex of the orbit to the rim of the orbit where it merges
with the periostium of face and forehead. The periorbita is loosely attached on the inner
surface of the orbit and a potential space lies between the bone and the periorbita, which can
accumulate fluid resulting in periorbital abscess. The periorbita is attached firmly to the apex
of the orbit, round the optic foramen where it merges with dura of optic nerve, at the margins
of the superior and inferior orbital fissure. At the posterior lacrimal crest, it splits into two
parts that cover the lacrimal sac as lacrimal fascia. It is also firmly anchored to the sutures of
all the bones of orbit and borders of various foramena.
The orbital septum
The orbital septum is a sheet of strong elastic tissue that prevents the contents of the
orbit to come out into the lid. In fact it divides orbit into two parts, a smaller anterior part and
a larger posterior part. The orbital septum prevents spread of infection from the anterior part
to the orbit and vice a versa. The septum originates from the periostium of the orbital margin.
In the upper part, it terminates 10-15 mm above the upper tarsus and gets inserted in the
aponeurosis of levator. In the lower lid, it originates from the periostium of the inferior orbital
rim, goes up tobe inserted in the capsulopalpebral ligament of the lower lid. The
DISORDERS OF ORBIT IN CHILDREN 675
capsulopalpebral ligament is anatomic equivalent of levator aponeurosis in the lower lid. The
septum terminates 6mm below the lower tarsus.
Surgical spaces of the orbit
3,4
The orbit is divided into four unequal surgical spaces by various membrane like struc-
ture. They are :
1. Periorbital
2. Peripheral
3. Muscle cone and
4. Tenons space
The spaces help in localising infection, inflammation and neoplasm in a restricted zone.
Involvement of each space has its own clinical presentation that differentiate one from the
other, however there may be some overlap.
1. Periorbital space. This is a potential space between the periorbita and the bones of
the orbit. The periorbita can be surgically lifted with ease except at its attachment at various
sutures, edge of the fissures and optic foramen. Pus, blood or tumours lift the periorbita,
pushing the globe to the opposite side. The tumours may remain in this space for considerable
time without breaking the periorbita. Common growth that are seen in this space are tumours
of the bone of the orbit, leukaemia deposits, dermoids, meningiomas and mucocele arising
from various paranasal sinuses. This is a frequent site for accumulation of blood. The growths
of this space are diagnosed by direction of displacement of globe, X-ray or CT. While surgically
opening the space, care should be taken not to spill the infection to other surgical spaces.
2. Peripheral space. This space lies between the periorbital space and the muscle cone.
It is bounded anteriorly by the septum orbitale, tarsal plates, tarsal ligaments and levator
aponeurosis. In the posterior part, it merges with the central space. It contains lacrimal
gland, superior and inferior oblique muscles, and levator palpebral muscle. The frontal, lacrimal
and ethmoidal nerves pass through this space. The superior and inferior ophthalmic veins lie
in this space. The position of superior ophthalmic vein is very constant hence it is used for
orbital venography, which differentiates intra conal lesions from extra conal lesion. Space
occupying lesions of this space cause displacement of the globe to the opposite side and proptosis,
which are best seen on CT and explored either via anterior or lateral orbitotomy. Posterior
placed lesions are best removed by transnasalendoscope. Exudation from this space may
permiate through the septum orbital and cause edema of the lids.
3. Central space. There is a conical spaces with apex towards the apex of the orbit, the
wall of this space are formed by the four recti and their inter muscles attachments. The inter
muscular septa is deficient at the posterior part of the space hence the apex of the cone
merges with peripheral space. The optic nerve traverses the cone from its apex to the back of
the globe along with its covering. The long and short posterior ciliary nerves and vessels, the
ciliary ganglion, the two divisions of third nerve; the sixth nerve, the nasociliary nerve, oph-
thalmic artery, lie in this space. These structures are embedded in the orbital fat. Growths
arising from any of these structures, neoplastic deposits, pus, blood or foreign body cause an
axial proptosis. Commonest cause of axial proptosis in children is optic nerve glioma. The
growths are explored through a lateral orbitotomy. Posteriorly placed growths are removed
endoscopically or by transcranial route.
The Tenons capsule and Tenons space
4. Tenons capsule or fascia bulbi is a thin membrane that covers the globe from the
limbus upto optic nerve. Anteriorly it is deeper to the conjunctiva and merges with the
subconjunctival tissue to form a strong attachment, posteriorly it merges with the dura of the
optic nerve. The sclera lies within the capsule. With the movement of the eye, the Tenons
capsule also moves. Posteriorly the orbital fat is in contact with the capsule, the posterior
part is pierced by the optic nerve, ciliary vessels and nerves. The venae verticosae pierce the
capsule behind the equator. At the insertion of the extraocular muscle, the Tenons capsule
assumes a tube like shape in which the muscles pass like hand in the glove.
The Tenons capsule sends few extensions beyond the muscle. The expansion of the
capsule from the lateral rectus is attached to the Whitnall ligament and is called lateral check
ligament of the eye. Similarly an extension from the medial rectus is attached to the lacrimal
crest and called the medial check ligament. The function of these check ligaments is to stabilise
the globe and prevent excessive movement of the globe. The expansion from the sheath of the
medial rectus is joined to the levator palpebral superior and co-ordinate upward movement of
the globe and the lid. It is essential to cut these extensions during ptosis surgery otherwise
the eye will be rotated down resulting in to hypotropia. In case of surgery of the recti, the
capsule should not be cut beyond 10mm from the insertion otherwise orbital fat will herniate.
Similarly an extension from the inferior rectus is attached to the capsulopalpebral fascia of
lower lid. The expansion from the superior oblique is attached to the trochlea, that of inferior
oblique is attached to the lateral part of the floor. Expansions from the lateral and medial
recti are joined by the extension from the two oblique to form a sling on which the eyeball
rests and this is called Lockwood ligament.
3
The Tenons space is a potential space between the Tenons capsule and the outer wall
of the globe. The space is traversed by the insertion of the extraocular muscles, the ciliary
nerves and vessels the venae verticosae. Fluid accumulated in the posterior part of the space
cause proptosis. The fluid accumulated in the Tenons space is drained through the conjunc-
tiva. This space is also utilised to give anterior and posterior Tenon injection of antibiotic,
steroids and anaesthesia.
Fissures of the orbit
The orbit has two elongated fissures i.e. the superior orbital or sphenoidal fissure and
inferior orbital fissure, the former is larger and transmit more anatomical structures to and
fro the orbit.
The superior orbital fissure
This is an elongated inverted comma like defect between the lateral wall and the roof of
the orbit. It is a gap between the greater and lesser wing of the sphenoid, wider medially and
inferiorly and narrow in superio lateral aspect. It is about 22 mm in length. The lateral most
point of the fissure is about 35 mm from the frontozygomatic suture of the rim of the orbit. It
is lateral to the optic foramen. A tendenous ring called annulus of Zinn that divided the
fissure into superior, central and inferior part forms the common origin for all the recti except
the lateral rectus. The lateral rectus originates slightly lateral to this ring from a boney
prominence called the spine for the lateral rectus. The fissure communicates with the middle
cranial fossa posteriority.
The upper part transmits trochlear, frontal and lacrimal nerves, superior ophthalmic
vein and recurrent lacrimal artery. Through the middle part pass the superior division of
third nerve, the nasociliary nerve is the sympathetic connection to ciliary ganglion, inferior
divisions of third nerve and abducent nerve. The inferior ophthalmic vein is the only structure
that passes through the lower part of the fissure. The optic nerve and ophthalmic artery
enter the orbit through the fibrous ring outside the superior orbital fissure.
The inferior orbital fissure
The inferior orbital fissure lies between the lateral wall and floor of the orbit. It is
separated from the superior orbital fissure by a thin piece of bone. The inferio orbital fissure
transmits veins from the lower part of the orbit to join the pterygoid plexus, the zygomatic
nerve and intra orbital nerve.
Development of the orbit
Development of orbit is a complicated process involving bones of orbit, paranasal si-
nuses, contents of the orbit and brain. By 3 months of intrauterine like boundaries of the
orbit are apparent but not complete.
6
The walls develop along the development of the face and
are well grown by fourth month. The bones are mesodermal in origin that develop around the
developing eyeball. The floor and lateral walls develop to form the maxillary process, the roof
develops from paraaxial mesoderm forming capsule covering brain. The medial wall develops
from lateral nasal process. The developing eye does not influence development of walls of
orbit. However size of the orbit depends on the size of the eyeball as size of cranium depends
on size of the brain. (See page 7 also)
The extra ocular muscles develop from the paraxial mesoderm, the six muscles are
supplied by three cranial nerves. The mesodermal masses are arranged into three groups,
each supplied by one cranial nerve. The cranial nerves grow from the brain into specified
mesodermal mass. The oculomotor starts developing earliest and trochlear last. (See page 4
also)
The orbit at birth
Orbits are fully developed at birth but are larger in proportion to the face when com-
pared to adult face. The orbital walls are nearer to the globe. The volume of the orbit is small
in infancy that makes the children more prone to develop proptosis. The orbits are placed
nearer to each other, with age they shift laterally to assume normal adult position. The
intraocular contents are well developed, any defect in development of extraocular muscles
lead to strabismus. Some of them may be congenital others develop in childhood.
The orbits of the children are placed nearer to each other than adults, with age of
orbits move laterally. The depth of the orbit is shallower in children. Volume of average adult
orbit is 30 ml, the volume of average adult eye ball is 7.1 ml, giving a ratio of 4.5 : 1. The
volume of eyeball of an infant is 2.8 ml and volume of orbit is about 13 ml. The orbital walls
are very close to the eyeball at birth. The size of eyeball in children is an important factor in
growth of the orbit. Smaller or absent eyeball retard the growth of orbit in children giving
asymmetrical orbit. Bilateral microphthalmia or anophthalmos result in smaller orbit on both
sides. Large eyeball i.e. congenital myopia, buphthalmos, retinoblastoma cause enlargement
of the orbit. So do other intra orbital growths in children.
Congenital anomalies of orbit
5
The common congenital anomalies are :
1. Hypertelorism
2. Hypotelorism
3. Shallow orbit
4. Orbital meningocele
5. Orbital encephalocele
6. Orbital and periorbital dermoids
7. Plexiform neurofibroma
8. Sturge Weber syndrome
9. Orbital teratoma
10. Cranio facial dysostosis.
Hypertelorism
6,7,8
Hypertelorism is a condition where the intra orbital distance is more than 33 mm in
adult and correspondingly less in children. It may be isolated defect but generally associated
with other deformities of face and skull. It is inherited as autosomal recessive or dominant
trait, sporadic cases are also known, there may be family history. The clinical features are
broadening of nose bridge, depressed nose bridge, prominent forehead, exotropia, optic atrophy,
astigmatism, displacement of lacrimal puncta laterally, ptosis, inverse epicanthus.
Hypertelorism is common in Crouzons and Aperts syndrome. Generally the condition
is bilaterally symmetrical, however it may be asymmetrical and does not require treatment.
Hypotelorism
This is a very rare condition where intra orbital distance is reduced. The eyes are set
nearer as in Hallermann-Streiff syndrome. No treatment is required.
Shallow orbits - Shallow orbits are very common in craniofacial dysostosis resulting
into proptosis.
Meningo encephalocele orbit
8,9
During the development of the skull and orbit, the covering of the brain/ brain substance
with its covering may herniate through a bony defect in the orbit. The common sites for such
herniation are root of the nose and superio nasal angle of the orbit. When only the meninges
herniate, this is called meningocele. This is rarer than encephalocele. The meningocele presents
as round, soft, pulsatile growth that may have cough impulses. It is generally noticed at birth
or soon after. The encephalocele commonly develop at root of the nose, may remain localised
or spread to the orbit. Ocular involvements depend on position and size of the encephalocele.
It can cause lateral displacement of the globe, proptosis. Squint, may be associated with
microphtlamia. The systemic findings include mental deficiency, hydrocephaly, spina bifida,
harelip and cleft palate. Treatment is surgical removal of the growth and repair of the defect
in the orbital bone.
A small meningocele or encephalocele near the medial canthus may be confused as
congenital mucocele in infant and encysted mucocele in adult. The difference in two conditions
is settled on CT and MRI.
Orbital and periorbital dermoids
Dermoids are choriostomas, which means that they are benign growths, which arise
from tissues that are not normally found in the site of the origin of the growth i.e. skin and
sebaceous glands in case of orbital dermoids, epidermoids and teratomas. These growths are
due to sequestration of primitive ectoderm in case of epidermoid and dermoid while teratomas
contain more than one germinal layer
5
, i.e. cartilage, bone, teeth etc. Dermoids result when
deeper layer of skin containing sweat glands and sebaceous glands, hair follicles are
incarcerated. Epidermoids contain only superficial layers of skin. It is not always possible to
differentiate between the dermoid and epidermoid. The choriostomas generally arise at the
suture lines of the orbit. Commonest site being superio temporal followed by superio medial
in front of the orbitalseptum, sometimes they may arise from greater wing of sphenoid and
cause proptosis. The dermoids arising on the globe are called limbal dermoids. Twenty five
percent of dermoid anterior to the orbital septum are noticed at birth or soon after as palpable,
non-tender, well circumscribed growth with rubbery consistency. They may be movable or
immobile, generally cystic. On rupture they produce pultaceous material. The deeper dermoids
are discovered in the first two decades, they may not produce proptosis and are palpable only
if placed anteriorly. Posteriorly placed dermoids are seen in third decade and cause proptosis.
The epidermoids are solid growths in contrast to dermoids that are cystic. Both produce sharply
demarcated bone defect with well defined margin and sometime sclerotic bone changes.
Occasionally the dermoid can be dumb-bell shaped. The radiology of deep orbital dermoids
have classical cystic appearance with well defined wall. CT and MRI give better results.
Treatment consist of surgical removal. Anteriorly placed cysts present no problem in removal.
However, they should be differentiated from meningo encephalocele prior to surgery. The
posterior orbital dermoids are best removed by lateral orbitotomy or craniotomy.
Orbital teratomas
5
Orbital teratomas are uncommon, they may contain all the three germinal layers.
Occasionally a miniature second foetus may be present in the orbit. Generally these are small
at birth but grow fast enough to produce severe proptosis and exposure, with loss of vision.
Treatment consists of complete surgical removal.
Cranio synostosis (Craniostenosis, craniofacial dysostosis) oxycephaly (Tower skull)
Oxycephaly is a common form of craniostenosis with ocular involvement. In this case
there is premature closure of coronary and lambdoid sutures that results in growth of the
skull vertically and arrested growth in anterio posterior and lateral planes before the brain
growth is complete.
1,2,8
Growth of brain is an essential factor in growth of skull like growth of eyeball influences
growth of orbit, if the brain stops growing the skull remains small even with normal sutures
resulting in microcephaly. The frontal and perital bones grow steeply and vertically. It is
more common in boys. It is generally present at birth or noticed soon after birth. Orbital
involvement is roughly symmetrical. Due to premature closure of the sutures, the orbit fails
to develop in all directions, resulting in shallow orbit that is too small to house growing globe.
The globe is generally normal, the effect on the globe is secondary to shallow orbit. The
shallowness of the orbit is caused by medial and forward rotation of greater wing of sphenoid.
The pituitary fossa is also small.
5
Most striking ocular feature is gradually increasing bilateral proptosis. In advanced
degree of proptosis, the lids fail to cover the cornea and cause exposure keratitis leading to
ulceration and perforation. In extreme proptosis, the globe may subluxate. The first dislocation
is very frightening to the child and the parents who learn to reposit the globe themselves
later. Proptosis is very often associated with divergent squint, nystagmus and other motility
disorders. There may be associated developmental cataract.
Commonest symptom next to cosmetic blemish is diminished vision that is brought
about by corneal changes, change in optic nerve. Optic nerve changes are caused by narrowness
of optic canal leading to primary optic atrophy or post papilledematous atrophy due to raised
intra cranial tension. Raised intra cranial tension leads to separation of non-fused sutures,
thinning of the cranial bones and digital impression on the cranium. The vertical growth of
the skull stops with completion of brain growth round about seven to eight years of age.
There is no known treatment except craniotomy under six months of age. The exposed cornea
should be kept moist by lubricants and antibiotic ointment. Tarsorrhaphy may be required in
moderate cases and cases of frequent subluxation of globe. The tarsorrhaphy usually is
ineffective in severe cases.
Other forms of congenital deformities of skull are
5
1. Scaphocephaly (Scaphoid head)Long narrow head due to premature union of sagital
sutures.
2. BrachycephalyThe skull is short and broad due to fusion of coronal suture, may be
considered as variation of oxycephaly.
3. DolichocephalyA prominent frontal region.
4. Trigonocephaly (Triangular head)There is under development of frontal region of
skull with over development of posterior part of the head. This results in close set
orbits and optic atrophy.
5. Microcephaly (Microencephaly)Overall dimension of the skull is smaller than
normal, the primary fault lies in the smallness of the brain. There is premature
fusion of all the sutures and closure of the fontanelle, neurological defects which
include mental retardation, idiocy, paraplegia, diplegia. Epilepsy is common. The
condition may be noticed at birth or soon after. The orbits are deformed. The child
may not only be blind but deaf and dumb. Multiple ocular defects are met with.
6. Hydrocephalus - In this condition there is normal production of CSF with obstruction
in its flow. There are three possible sites where the obstruction may develop.
(i) Sylvius aqueduct
(ii) Foramen of Magendie
(iii) Foramen magnum.
Obstructed CSF circulation results in accumulation of CSF in ventricles, which get
dilated, pressing the overlying brain, and thinning of the skull resulting into enlargement of
the head in all direction with normal face. The sutures are wide open. The face looks triangular
in relation to monstrous head, which the child may not be able to hold. The skin over the
forehead is stretched, dragging the upper lid leading to lagophthalmos and lid retraction. The
lower lid is similarly pulled up and covers the lower part of the cornea compensating the up
drawn upper lid. The eyes are pushed down, giving a setting sun appearance.
There may be associated proptosis, paralytic squint. In spite of accumulation of CSF,
papilledema is rare and primary optic atrophy is more common. The condition may be noticed
on ultrasonography before birth, may cause obstructed labour or may be noticed soon after
the birth. Some of the predisposing factors are high maternal age, Rh incompatibility, maternal
rubella and hydramnios.
Crouzons Syndrome
5,6,13
This craniofacial dysostosis has important ocular manifestation. This is an autosomal
dominant trait seen more commonly in boys. It is a bilateral condition that may be suspected
at birth or within few months. The child has dolicocephaly and frog like face. Bilateral
exophthalmos is commonest feature caused by shallow orbit with normal sized eyeball. There
is downward slant of outer canthus. Other findings are exotropia with tendency towards V
pattern. Paralysis of extra ocular muscles, nystagmus, under developed maxilla resulting
small upper jaw and large lower jaw, hypertelorism and optic atrophy without papilledema.
X-ray shows sutural closure with increased digital marking. There may be hearing loss and
various degrees of mental retardation.
Management consist of protection of cornea from exposure keratitis that may end up in
perforation. Early craniotomy have been advocated to arrest the progress of disorder.
Aperts syndrome (Acrocephalo syndactylia)
This is a combination of oxycephaly with fusion of fingers of both extremities of variable
nature. It may run in families in various combinations and inheritance is autosomal dominant
however sporadic cases are frequent. Increased maternal age is a contributing factor like in
Crouzons disease, the orbits are widely separated and shallow resulting in to hypertelorism,
proptosis and anti mongoloid slant of palpebral fissure. The forehead is prominent, maxilla is
hypoplastic with high arched palate. The ears are low set. The middle third of the face is more
involved than the lower two third, causing crowding of the teeth, parrot shaped nose. Oculo
motor disorders are more common, commonest being exotropia with V pattern followed by
vertical squint that is brought about by displacement of trochlea at the superior orbital margin.
There may be over action of inferior oblique. Due to pushing forward of the globe, there is
stretching of extra ocular muscles, resulting in various degree of external ophthalmoplegia.
Diminished vision is brought about by corneal changes, astigmatism, amblyopia and
optic atrophy. Generally intra cranial tension is normal. Occasionally papilledema has been
reported. Mental retardation is common so are cardiac and visceral malformation.
Management consists of protection of cornea by lubricant, antibiotic. Tarsorrhaphy
may give temporary respite, early craniotomy may arrest the progress of the disease.
First and second branchial arch syndromes
5,6,8,14
Abnormal development of first and second branchial arches lead to a group of congeni-
tal disorders of face and eye, that are called mandibulo facial dysostosis without proptosis.
Common syndromes are -
1. Franceschettis syndrome
2. Pierre Robin syndrome
3. Goldenhars syndrome
4. Hallermann Streiff syndrome
5. Waarden burg syndrome
Franceschettis
Franceschettis syndrome (also known as Treacher Collins syndrome when the ears are
not deformed)The syndrome is bilateral, symmetrical. There is no gender predilection. No
race is immune. It has autosomal dominant inheritance. Sporadic cases are not uncommon.
Severity increases with successive generations. Claims of unilateral cases are doubted.
Increased parental age increases chances of the syndrome. Both upper and lower facial bones
are involved, resulting in to hypoplasia of maxilla and mandible. The general features are
striking with macrostomia, micrognathia, abnormal hair line, deformity of external and middle
ear, hearing loss, sinus between the ear and angle of mouth, coloboma of the lip, high arched
palate and abnormality of dentition has been seen.
The ocular manifestation consists of normal globe with fairly good vision. The orbit is
egg shaped, most of the anomalies are located in the lower lid with changes in the lateral
canthal ligament and lower lid margin. The change in lower lid contains various types of
colobomas of different length and depth. The minimal change is a S-shaped curve in the outer
third of a lower lid. There may be a full thickness defect with loss of lashes and tarsus.
Sometimes a large coloboma may cause deficiency of outer third. There may be trichiasis,
distichiasis. The orbicularis is also under developed. All these lead to abnormal tear film,
corneal irritation and corneal exposure. Various types of strabismus are possible. There is
always some degree of astigmatism. Squint, astigmatism and corneal opacity lead to amblyopia,
which is the main cause of defective vision. Optic nerve does not show any atrophy or
papilledema. Intelligence and growth of the child is good. The condition can be noticed at
birth if looked-for carefully, especially if there is a positive family history or it becomes evident
by first year. Presence of syndrome can be seen on intra uterine ultrasonography.
The ocular changes do not require any treatment except correction of error of refraction,
management of amblyopia and protection of cornea. Facial deformities are managed by plastic
surgeons. The children are prone to repeated respiratory problems due to narrow upper
respiratory passage, which may cause difficulty in anaesthesia.
Pierre Robin syndrome
8,15
This syndrome has fewer ocular features. Main features are micrognathia. U Shaped
cleft palate without cleft lip, glossoptosis, bird like face, dysphasia, difficulty in feeding lead-
ing to failure of development. Ocular findings are microphthalmia, high myopia, glaucoma,
and retinal degeneration.
Goldenhars syndrome (Oculo auriculo vertibral syndrome)
5,16
This syndrome is sporadic, slightly more in boys. Right side is more commonly involved
than the left. The triad consists of ocular changes, changes in external ear and changes in
cervical vertebra. Heart and central nervous system may also be effected. There is hypoplasia
of temporo mandibular and pterygo mandibular complex
16A
.
1. The ocular components arecoloboma of upper lid, dermoid or dermolipoma,
nasolacrimal duct obstruction, reduced corneal sensation, squint, microphthalmia,
2. The auricular components consists ofPre auricular appendages, distorted pinna,
absent auditory meatus.
3. Vertebral changes areFusion of cervical vertebra, hemivertebrae, occipitalisation
of atlas.
The condition can be diagnosed by ante natal ultrasonography. Post natalit can be
diagnosed at birth due to limbal dermoid, coloboma of lid and auricular appendages. The
limbal dermoids are met within three fourth of cases, out of which about 25% are bilateral.
The dermoids are uniformly situated at lower temporal area.
Management of the cases is multidisciplinary. Ocular treatment consists of repair of
upper lid coloboma and removal of limbal dermoid. The coloboma and dermoid can cause
permanent corneal scarring and astigmatism.
Hallerman Streiff syndrome
This relatively rare syndrome, which is sporadic, consists of a parrot beak nose, loss of
eyebrow and lashes, microphthalmia, congenital cataract, micrognathia, short stature. There
is an antimongoloid slant of the inter palpebral fissure. Hypotelorism, nystagmus and squint
are some of the other ocular features. Vision is generally poor and does not improve with
glasses, may be due to corneal or lenticular changes, cataract is generally bilateral and may
become mature and soft, predisposing lens induced uveitis and glaucoma. Systemic conditions
are infection of respiratory system, difficulty in breathing.
Management consists of lensectomy either with posterior chamber implant, failing which
aphakia is corrected by contact lens or spectacle.
Waardenberg syndrome (White forelock syndrome)
32
The syndrome is autosomal dominant
32A
in nature. There is a defect in melanocyte,
which runs in the families. The syndrome consists of band of grey forelock, fusion of promi-
nent eyebrows, broad nose bridge, displacement of medial canthus along with lacrimal puncta.
The puncta may be shifted as much as the lower part of cornea, there is heterochromia of the
iris, albinism and unilateral deafness. The condition is compatible with normal life except
occasional aganglionic megalocon. Visual loss is minimal. Ocular conditions do not require
much attention. However deafness requires early management.
Symptomatology of orbital disorders
The orbits are surrounded by paraorbital air sinuses from all sides except anteriorly
and laterally, the brain with its coverings lies above the roof of the orbit, the optic foramen,
and superior orbital fissure communicate with the brain, the ophthalmic veins, the vena
verticosae open in the cavernous sinus. The orbit besides the globe, contains muscles, vessels,
nerves, fascia, meninges and fat. Hence the orbits are seats of many diverse disorders that
spread between intra uterine life to ripe old age.
The symptoms of orbital disorder can be broadly divided into two groups :
1. Forward propulsion of the globethe proptosis/exophthalmos
2. Retraction of globeEnophthalmos
All other symptoms are sequelae or complications of these two symptoms. Incidentally
proptosis (exophthalmos) and enophthalmos themselves are signs of other clinical importance
as well.
Signs and symptoms of proptosis are :
1. Pain
2. Conjunctival congestion and chemosis
3. Diminished vision
4. Ptosis
5. Lagophthalmos
6. Squint
7. Diplopia
8. Exposure keratitis
9. Loss of corneal sensation
10. Rise of intraocular tension
11. Rise of intra orbital tension
12. Marcus Gunn pupil
13. papilledema
14. Optic atrophy
15. Central vein stasis
16. Central retinal artery occlusion
17. Choroidal fold
18. Change in refraction
19. Pressure changes in the bones
20. Systemic involvement
All the signs and symptoms need not be present in all eyes at the same time. They may
be present in various combinations. There is no fixed chronological order, some signs and
symptoms may be of acute onset, others may be slow in progression.
1. Pain - Children have greater threshold of pain, a non-verbal child may cry in dis-
comfort. Gradually progressing proptosis is generally devoid of pain. Some of the painful
proptosis are :
Accumulation of fluid under periorbita i.e. periorbital abscess or hematoma.
Acute orbital cellulitis and abscess.
Cavernous sinus thrombosis.
Exposure of cornea is always associated with some degree of pain.
2. Conjunctival congestion and chemosis are very common when the child is unable to
close the eye. Common causes of chemosis of conjunctiva are orbital cellulitis, panophthalmitis,
almost all cases of proptosis have some degree of conjunctival congestion.
3. Diminished vision in proptosis can be due to following :
(i) Error of refraction
(ii) Corneal changes
(iii) Fundus changes
(iv) Squint
(v) Amblyopia
(i) Errors of refraction are brought about by compression of the globe at various sites.
Pressure on the cornea by the lids produce astigmatism, pushing of the retina from
behind resulting in to hypermetropia, distortion of the globe due to pressure from
the orbital side produces irregular astigmatism.
(ii) Amblyopia is caused by :
(a) Over hanging lid over the cornea, corneal opacity cause deprivation amblyopia.
(b) AstigmatismCommon causes of astigmatism areCapillary haemangioma,
neuro fibromatosis, dermoids, epidermoids and dermolipoma.
(iii) Changes in cornea results from exposure keratitis due to lagophthalmos.
(iv) Fundus changes consist of optic nerve changes i.e. optic atrophy, venous conges-
tion, rarely central retinal artery occlusion, retinal haemorrhages and fold in the
choroid.
4. Ptosis is not a very consistent feature it can be :
(i) Mechanical Neurofibroma
Capillary haemangioma
Orbital cellulitis
(ii) Paralytic as part of - Paralysis of third nerve, isolated paralysis of third nerve is
rare in proptosis. It may be an early manifestation of wide range of paralysis of
other extra ocular muscle due to involvement of third, fourth and sixth nerve, may
be associated with corneal hyposensation.
5. Lagophthalmos (Inability to close the eyes)It can be mechanical due to extreme
degree of proptosis which does not allow the two lids to meet or due to paralysis of orbicularis.
6. Squint - Ocular deviation can be brought about by paralysis of extra ocular muscles,
mechanical restriction of extra ocular muscle movement. Concomitant squint is generally
associated with errors of refraction, opacity in media and optic nerve changes.
7. Exposure keratitisExposure of the cornea is one of the most unpleasant complica-
tions that may lead to sloughing and perforation of cornea. It is caused due to inability to
close the lids and diminished corneal sensation, any of these alone or in combination with
infection cause corneal involvement. Post keratitis corneal opacity is a cause of persistent
diminished vision.
8. Loss of corneal sensation is met in cavernous sinus thrombosis, superior orbital
fissure syndrome and pseudo tumour of the orbit where fifth nerve is involved along with
other cranial nerves.
9. Rise of intra ocular tensionGlaucoma in proptosis is mostly due to extra ocular
causes i.e. increased episcleral pressure, pressure of the globe from outside and restrictive
squint.
10. Rise of intra orbital pressureThere is no known method to measure intra orbital
pressure but clinically this happens if there is an expanding orbital lesion with intact orbital
septum.
11. Marcus Gunn pupilPresence of Marcus Gunn pupillary reaction denotes involve-
ment of optic nerve resulting in conduction defect in unilateral proptosis.
12. PapilledemaSwelling of optic disc denotes either raised intra cranial pressure,
which is common in congenital anomalies of skull, extension of growth in to the cranial cavity
or compression of central retinal vein.
13. Optic atrophyBoth primary as well as post papilledematous optic atrophy are
possible. The first is seen in glioma of optic nerve while the second is noted as end result of
papilledema.
14. Central vein stasis results in dilatation of retinal veins, brought about by many
expanding lesions of the orbit. However full blown central vein thrombosis has not been
observed.
15. Central retinal artery obstruction and optociliary shunts are rare phenomenon.
16. Change in refraction - Refractive errors are induced due to change in curvature of
the cornea or due to pushing of posterior pole by a retrobulbar mass. Paralysis of third nerve
leads to loss of accommodation.
17. Pressure changes in the orbital bones are evident on X-ray.
18. Some of the orbital disease may be associated with systemic disorders i.e. Storage
disease, leukaemia, parasites, neuroblastoma, Ewings sarcoma, thyroid eye disease and
secondaries.
Evaluation of a case of proptosis
Orbit is a Pandoras box that contains so many tissues. Involvement by diverse etiology
and presentation make it difficult to pinpoint the exact nature of the disorder. Hence proptosis
requires meticulous evaluation under following heads.
1. History
2. Ocular examination
3. Systemic examination
4. Investigation
5. Therapeutic trial
History
Detailed history is one of the most important clinical methods in evaluation of proptosis.
The child may be non-verbal, or may not co-operate. In such cases parents should be quizzed
about the disorder, children have a tendency to hide history of trauma, they have greater
threshold for pain. A child who has good sleep without analgesic and sedation most probably
has mild or no pain.
History should comprise of :
1. Age of onset
2. Mode of onset
3. Duration
4. Recurrence
5. Trauma
6. Pain
7. Diminished vision
8. Diplopia
1. Age of onset. Proptosis can be present at birth and the causes are invariably congenital.
They are craniostenosis, meningocele, meningoencephalocele, and microphthalmia with
congenital cyst. In infancy above conditions continue to be the cause of proptosis and may
cause progression of proptosis. To this list are added following benign growth in first decade.
Dermoid, epidermoid, haemangioma, neurofibroma. Lymphangiomas are generally seen
after first decade. Average age for optic nerve glioma is seven years. Intra orbital meningiomas
too manifest in early part of second decade. It must be kept in mind that all the above growths
have congenital background.
The malignant growths that cause proptosis in infancy and childhood belong to two
distinct groups i.e. primary malignant tumours and secondaries. Commonest primary
malignant orbital tumour in childhood is rhabdomyosarcoma followed by Burkitts lymphoma,
Common secondaries are neuroblastoma, Ewing sarcoma, Wilms tumours leukaemias. Orbital
retinoblastoma have a place of its own though orbital extension of retinoblastoma is a late
feature that is preceded by latent stage and stage of secondary glaucoma. Sometimes there
may be extension of retinoblastoma before stage of secondary glaucoma.
Less common causes of proptosis are orbital varix and fibrous dysplasia. Though the
list of new growths is long and impressive, infections are the foremost cause of proptosis in
children. They areOrbital cellulitis, orbital abscess, cavernous sinus thrombosis,
panophthalmitis, retained intra orbital foreign bodies.
Orbital pseudo tumours manifest in late childhood, occasionally retro bulbar
haemorrhage or emphysema may result in proptosis in children mostly due to trauma.
To summarise, causes of proptosis in childhood are
17,18
I. Congenital 1. Craniostenosis
2. Meningocele
3. Meningoencephalocele
4. Encephalocele
5. Microphthalmia with cyst
6. Dermoid
7. Teratoma
II. Traumatic 1. Retrobulbar haemorrhage
2. Emphysema
3. Retained orbital foreign body.
III. Infective
1. Acute
(a) Orbital cellulitis
(b) Orbital and periorbital abscess
(c) Cavernous sinus thrombosis
(d) Panophthalmitis
2. Chronic
(a) Pseudo tumour orbit
(b) Intraorbital parasitic cyst
IV. Neoplasm
1. Benign
(a) Dermoid and epidermoid
(b) Capillary haemangioma
(c) Neurofibroma
(d) Lymphangioma
(e) Optic nerve glioma
(f) Orbital varices
(g) Fibrous dysplasia
(h) Intraorbital meningioma
2. Malignant
(a) Primary
Rhabdomyosarcoma and Burkitts lymphoma
(b) Secondary
Neuroblastoma, orbital retinoblastoma, Ewings sarcoma Wilms tumour
leukaemia.
V. MiscellaneousHand Schuller Christian disease, juvenile xantho granuloma,
eosinophilic granuloma, sinus histocytosis.
Mode of onset
Acute onset and increase in proptosis is seen in orbital cellulitis, panophthalmitis,
cavernous sinus thrombosis and retro bulbar hae-morrhage.
Rhabdomyosarcoma, neuroblastic and leukeamic deposits have moderate to fast pro-
gression.
DurationLong duration with minimal symptoms are seen in congenital causes and
benign growths.
RecurrenceRecurrence is common following treatment of rhabdomyosarcoma,
neuroblastoma, leukaemia, retinoblastoma. Retinoblastoma treated by radiation are known
to develop second non retinoblastoma malignancy.
Trauma
Frank trauma with laceration of lid, fracture base of the skull and orbit cause
accumulation of blood in retro bulbar space. Accumulation of air results in orbital emphysema,
which denotes fracture of paranasal sinuses. Retained intra orbital foreign bodies not only
cause retro bulbar hematoma but also cause orbital cellulitis if the foreign body is infected
and are known to develop foreign body granuloma.
Pain
Pain is seen in acute infective lesions like orbital cellulitis, orbital abscess, cavernous
sinus thrombosis or any fast expanding mass. The pain suddenly subsides if the fluid
spontaneously discharges outside the orbit via conjunctiva or in to the lid after perforating
the orbital septum. Corneal involvement is a common cause of pain, lacrimation and
photophobia. In case of unexplained pain, intraocular tension should be measured by tonometer.
A non contact tonometer is preferred over contact tonometer.
Diminished vision
Loss of vision followed by proptosis is pathognomic of optic nerve glioma in children.
However the child may not be aware of such painless loss and may not complain. Loss of
vision with pain is due to corneal involvement.
Diplopia
Diplopia denotes early stage of paralytic squint or restrictive strabismus, which may
subside due to loss of vision, or onset of deep amblyopia.
Ocular examination
VisionRecording of vision should precede all other examinations. In a non-verbal
child clear, bright cornea, normal anterior chamber, brisk direct and indirect pupillary reaction,
clear media, absent error of refraction and normal fundus invariably denotes good vision
except in case of deep amblyopia and cortical blindness.
In a slow progressive proptosis, vision is generally good and remains so for sometime. In
glioma of optic nerve, visual loss is followed by proptosis, sudden painful loss of vision means
corneal involvement. Sudden painless loss of vision is due to fundus changes that may be optic
neuropathy, optic atrophy, haemorrhage in the retina or vitreous, vascular obstruction, choroidal
fold or hypermetropia due to growing mass behind the globe that pushes the retina forward.
Pupillary reactionA Marcus Gunn phenomenon denotes optic nerve compression.
Cavernous sinus thrombosis and superior orbital fissure syndrome may result in dilated slug-
gish pupil.
InspectionA well established proptosis is not difficult to diagnose. An early proptosis
requires proper examination. In such case most important is to determine if the proptosis is
real or apparent (pseudo proptosis).
Pseudo proptosis is that condition where there is no pathology in the orbit. The pathology
is either in the lid or eyeball. Sometimes the pathology lies in the contra lateral eye in the
form of enophthalmos.
The causes in the lid that mimics proptosis areLid retraction and lagophthalmos in
the ipsilateral eye or ptosis, pseudo ptosis of the other eye.
Ocular conditions that may be mistaken as proptosis arePathological myopia,
buphthalmos, large ciliary staphyloma, in the eye under examination. Contra lateral
microphthalmos, phthisis or enophthalmos are also sometimes mistaken as proptosis of the
ipsilateral eye.
Next step involves finding out laterality of the proptosis i.e. is at unilateral or bilateral.
If bilateral, is it equal in both eyes or advanced in one eye.
Causes of unilateral proptosis in children are :
Orbital cellulitis, orbital and peri-orbital abscess, early stage of cavernous sinus
thrombosis, rhabdomyosarcoma, pseudo tumours, neurofibroma, capillary haemangioma,
lymphangioma, dermoids, retro bulbar haemorrhage, rarely congenital.
Causes of bilateral proptosis in children are :
1. Mostly systemic condition
2. Congenital anomalies of the orbit
Systemic conditions that cause bilateral proptosis areneuroblastoma, leukaemia,
bilateral retinoblastoma, second malignancy following radiation for retinoblastoma, fully
developed cavernous sinus thrombosis.
Measurement of proptosis
Relative position of cornea in relation to orbital margin gives clue regarding proptosis
of ipsilateral eye, enophthalmos of the other eye, extent of proptosis when present and its
progression. This can be done clinically by noting the position of corneal apex in relation to
superior orbital margin. It can be conveniently done in a co-operative child. The examiner
stands behind the child, asking the child to tilt the head backwards and fix at a distant object
keeping both eyes open and noting the position of the cornea in relation to superior orbital
rim. Normal corneal apex is generally not visible beyond the superior orbital margin. A visible
cornea beyond orbital rim denotes proptosis. A false impression is given by high myopia. 3D
myopia is equal to 1mm enlargement of globe in anterio posterior diameter. The other method
consists of actual measurement of distance between the corneal apex and the lateral orbital
margin. There are various types of exophthalmometer (Proptometers) to measure this distance,
they are - Optical and mechanical.
The optical exophthalmometer is more popular and widely used than mechanical. They
are Leuddes transparent scale and Hertels mirror proptometer.
Normal exophthalmic measurement vary between a wide range of 10 to
20 mm, average is taken as 15 mm. Any reading higher than 21 mm is considered as definite
proptosis. The other criteria is any difference of more than 2 mm between the two eyes provided
the other eye is normal. An enophthalmic contra lateral eye gives a false positive impression.
CT pictures can also be useful in evaluating extent of proptosis.
Inspection
1. Position of upper lid. Normal lid covers upper one fifth of the cornea and lower lid
just touches the lower limbus. No strip of sclera is visible either above or below the cornea.
Visible sclera above, below or both denotes proptosis or lid retraction. Generally lid retraction
is associated with lid lag. Lagophthalmos gives an impression of pseudo proptosis. All cases of
lid retraction with or without clearcut proptosis should be investigated for dysthyroid eye
disease. Incidence of dysthyroid orbitopathy is far less in children than seen in adults.
2. Direction of proptosis. Abnormal position of globe gives clue to location of orbital
pathology in relation to globe.
The globe may be displaced :
1. Centrally (axially) or
2. EccentricallyEccentric proptosis may be unidirectional or otherwise.
The causes of axial proptosis lies in the muscle cone. Commonest causes in children are
being glioma of optic nerve. Other causes are foreign body in the muscle cone, orbital varices,
pus localised in muscle cone.
A growth from the medial wall displaces eye laterally. Pathology from the floor pushes
the eyeball up. However the displacement always does not follow such simple rule. A growth
on the superio medial aspect pushes the eyeball forward, downward and laterally. Similarly a
growth from superio temporal orbit shifts the globe down, out and medially.
3. Visible pulsation. Sometimes the proptosed eye may have pulsation synchronised
with heart beat. Gross pulsation is evident without magnification. However slit lamp exami-
nation may show very mild pulsation that later becomes evident without biomicroscope. Such
pulsations are more common in adults, however pulsations are met with in neuro fibromatosis
when associated with defect in sphenoid, fibrous dysplasia and rarely in post traumatic carotico
cavernous fistula.
4. Cough impulse is visible and palpebal in cases of encephalocele and meningo
encephalocele.
5. Palpation
(i) Palpation of the orbital marginNormal orbital margin is smooth without any notch
or protuberance except superior orbital notch and the trochlea occasionally. The
medial palpebral ligament is palpable as a horizontal band, this is also a normal
feature.
Palpation or orbital margin may reveal irregularity, bone formation or bone
dehiscence. Growth arising from bony sutures i.e. dermoids can not be palpated
separately from orbital margin.
(ii) Palpation of orbital massAnteriorly placed growths are easily palpable. While
palpating a mass, if a finger can be insinuated between the mass and the orbital
wall, the growths is not arising from the orbital wall or the rim. The finger can not
be insinuated between the growth and the wall in case of encephalocele, meningocele,
dermoids, mucocele from the paranasal sinuses and lacrimal gland growth. Lacrimal
gland tumours are not met within children.
Compressibility of the proptosis
The child is asked to close the lids and the mass is pushed back through the closed lid
by first three fingers of the examiner. This should be avoided when tenderness of globe is
present. A compressible mass means a soft or vascular growth, solid tumours cause retro
bulbar resistance.
Valselva maneuver, juglar vein compression and head bending
None of the above procedures change the position of the eyeball in any proptosis unless
it is caused by vascular anomaly like capillary haemangioma and congenital varices.
In head bending test, the child is asked to bend the head and trunk forward for about
a minute and then come to normal position. The position of the globe is measured, an increase
in proptosis is considered positive denoting a vascular tumour.
Forced duction test
20
This test, when performed properly differentiates obstructive squint due to fibrotic
contracture from neurological defects. In a co-operative child this can be done as outpatient
procedure under local anaesthesia, otherwise it should be done under short term general
anaesthesia.
The test consists of :
1. Anaesthetise the conjunctiva by instilling local anaesthetic agent in both the eyes
for two to three times. If the child feels pain, a cotton swab dipped in same anaesthetic
solution may be left at the insertion of the muscle to be tested for three to four
minutes.
2. Ask the patient to look in the direction of limited movement.
3. Grasp the insertion of the muscle to be tested with a conjunctival fixation forceps,
move the globe passively in the direction of limited movement.
4. The procedure is repeated in the other eye and findings are compared.
A test is considered to be positive if the eye can not be moved passively in the direction
of limited duction and the cause is fibrotic contracture of the muscle or entrapment of the
muscle in a fracture. If the eye can be moved passively in the direction of limited action, the
test is called negative and the cause of limitation is neurological.
5. While performing the test, care should be taken not to push the globe backward deep
in the orbit otherwise there will be no limitation in presence of fibrotic band.
In children the test is frequently used in case of superior oblique sheath syndrome,
Duanes retraction syndrome, entrapment of muscle in fracture orbit or in case of strabismus
fixus. The test is most frequently used in adults in case of thyroid myopathy.
When the child does not allow forced duction test under local anaesthesia, a less painful
test is differential intra ocular pressure test. Intraocular pressure is noted in usual manner
in primary gaze. The patient is asked to look toward the field of action of the muscle under
question and tension is repeated. A rise of 6 mm denotes fibrosis or entrapment of the muscle.
No rise of tension or rise less than 6 mm is suggestive of neurological cause.
Special investigation in proptosis
They consist of :
1. Orbital imaging
A. Non invasive
(a) Plain x ray
(b) CT
(c) MRI
(d) Ultrasonography
(e) Venography
(f) Arteriography
B. Invasive
(a) Fine needle aspiration
(b) Biopsy
(c) Surgical excision and histopathological examination
2. Rhinological consultation
3. Blood picture
4. Blood chemistry
5. Systemic examination
Radiology of orbit in relation to proptosis
Posterio anterior view without angulation of head or beam, has very limited value in
evaluation of orbital lesions. The petrous part of the temporal bone and the para nasal sinuses
get superimposed on the soft fissure of the orbit and obscure their view. As most of the anatomic
landmarks form various degree of angulation in anterio posterior axis, they are required to be
positioned in such a way that they do not interferes with the structure of radiological interest.
Commonly used X-ray views are :
1. Caldwell view
(a) View with central X-ray tilted 25 downward
(b) Hypoangulated viewCentral X-ray is tilted less than 15
2. Waters view
3. Lateral view
4. X-ray for optic canal and foramen
(i) Rhese view
(ii) Ruggiero
5. Axial basal view
6. Submento vertex view
7. Townes view.
1. Caldwell view. In this view the patient lies face down, the forehead and the nose
touching the X-ray table with X-ray tilted downward. (a) at 25 and (b) 15.
The advantage of this projection is that the petrous part of temporal bone is shifted down
so that the orbital rim, roof, greater wing of sphenoid, lesser wing of sphenoid, the superior
orbital fissure are clearly visible. The foramen rotundum is shifted below the lower rim of the
orbit. The greater wing forms a larger part of lateral wall, the lesser wing of sphenoid is seen
near the medial wall. The superior orbital fissure is seen in between the two wings. It also
shows frontal sinuses, sphenoid ridge, floor of sellaturcia and innominate or oblique orbital
line. An angulation less than 15 gives better view of some of the above structures.
2. Waters view. This is also a posterio anterio projection. The patient lies prone on the
table with the chin resting on the table and nose lifted 4 cm off the table. This makes the
canthomental line form an angle of 37 to the central X-ray, which is at right angles to the X-
ray plate.
It gives a clear view of maxillary, frontal and ethmoidal sinuses, the lateral wall,
zygomatic arch and inferior orbital rim. This is useful when the pathology is in the sinuses
and fracture of the orbit is suspected.
3. Lateral view
The X-ray plate is kept parallel to the sagital plane, the central ray is at right angle to
this plane that passes 2.5 cm in front and 2 cm above the external auditory meatus.
This projection shows anterior and posterior clinoid process. The sellaturcia, sphenoid
sinuses, intra orbital canal. Part of the floor of the orbit is also visible.
The projection is used to localise intraocular, intra orbital foreign bodies, calcification
and pathology in sella.
4. Ray of optic canal and foramen
(i) Rhese view. This requires two separate exposures, one for each orbit. This is used to
visualise the optic foramen and optic canal.
The patient lies prone on the table with zygoma, nose and chin on the table. The
mid sagittal plane of the skull makes an angle of 53 with the film. The central ray
is projected posterio anteriorly with 12 angulation towards feet.
(ii) Ruggiero view
21
. These projections show optic canal, optic foramen, ethmoid cells,
lesser wing of sphenoid and superior orbital fissure.
5. Axial basal view shows ethmoid and sphenoid air cells.
6. Submento vortex view is taken on a supine patient. This view shows base of the
middle cranial fossa, the maxillary sinus, and the nasal cavity.
7. Towens view shows infra orbital fissure. This view is exposed when the patient is
supine and the X-ray is projected in anterio posterior direction.
Points to be noted of a X-ray of orbit :
The radiologist should see the patient and the ophthalmologist see the X-ray.
Single projection may not be sufficient to give all the required information.
X-ray of both the orbits should be taken, this helps in comparing changes in two
orbits especially in unilateral proptosis and a systemic bilateral proptosis.
1. Intraorbital distance. The intra orbital distance refers to distance between right and
left junction of frontal process of maxilla and maxillary process of frontal bone. The two
medial walls of the orbit are parallel to each other and the distance between the two varies
between 12.5 mm in child of one year to 25 to 28 mm at 12 years of age. If the orbits are set
apart, the condition results in hypertelorism. The cause of which are Crouzons disease, Aperts
syndrome, fibrous dysplasia, osteogenesis imperfecta, encephalocele. The intraorbital distance
may be as much as 30 to 40 mm in adult.
22
Hypotelorism
When the intra orbital distance is less than expected normal value of a given age. This
is seen in Downs syndrome, trigonocephaly and craniofacial anomalies.
Premature closure of cranial sutures
This is associated with deformity of skull, expansion of the skull at the right angles to
the closure of suture, shallowing of the orbit, rotation of wall of the orbit and increased digital
marking commonly seen in craniostenosis.
Widening of cranial suture
Normally a cranial sutures should close at 18 months of age. However if the intra
cranial tension rises before the sutures are closed, the skull expands due to increased intra
cranial pressure widening the cranial sutures, in such cases no digital markings are seen on
the bones of the skull. However, if there is rise of intra cranial pressure after the sutures are
closed, X-ray evidence of raised intra cranial tension will be observed. If some of the sutures
are closed, remaining sutures will open up; the example is craniostenosis.
Enlargement of the orbits
Size of the normal eyeball is essential for the growth of the orbit. In most of the cases of
proptosis the size of the eyeball is within normal limits, other orbital structures force the
orbit to expand. However a large eyeball itself can cause enlargement of the orbit.
The enlargement of the orbit can be unilateral and bilateral. Unilateral enlargement is
more rapid in children. The orbit of a child takes two to three months to show enlargement
while similar enlargement in adults take 1 to 2 years. Unilateral enlargement can be
symmetrical or asymmetrical. Unilateral buphthalmos either primary or secondary produce
enlargement of orbit. Retinoblastoma produces more and frequent enlargement than primary
buphthalmos, other causes of unilateral symmetrical enlargement are due to intra conal lesions
i.e. optic nerve glioma, neuro fibromatosis, haemangioma, extra ocular retinoblastoma inside
the muscle cone and intra conal pseudo tumour. Causes of asymmetrical enlargement of orbit
are - haemangioma, extra conal retinoblastoma, neurofibroma, dermoid, rhabdomyosarcoma,
orbital dysplasia, microphthalmos with cyst, congenital teratoma.
Diminished size of orbit
Orbital size may be smaller than normal either due to small or absent eyeball, a growth
encroaching from orbital wall or craniostenosis.
The common causes of small orbit areAnophthalmos, microphthalmos, early
enucleation of the eyeball, post radiation. Mucocele of the frontal sinus and growth from
maxillary sinus (rare in children) also reduce size of the orbit.
Changes in shape of orbit
Orbital shape can be deformed as part of craniostenosis, orbital dysplasia and dermoids.
Changes in the bone
The changes in the orbital bones can be :
1. Increased density
2. Decreased density
3. Destruction of bone
4. Fracture of the orbit
1. Density of bone is increased in osteoblastic metastases, chronic periostitis, fibrous
dysplasia.
2. Decreased bone density is seen in dermoid.
3. Dehiscence of bone is seen in neuro fibromatosis, mucocele and fracture of roof and
floor.
Calcification in the orbit
Intra orbital calcification may be confined to intra ocular structure, extra ocular structure
or both. In posterior anterio view, intra cranial calcification like cranio pharyngioma, congenital
toxoplasmosis, Sturge Weber syndrome may be superimposed on orbit, however a lateral
exposure separates intra orbital calcification from intra cranial calcification.
Causes of intraocular calcification areRetinoblastoma, parasitic cysts, choroidal bone
formation, complicated cataract, retrolental fibroplasia, phthisis blubae. Extra ocular
calcification is seen in orbital retinoblastoma, orbital varix, optic nerve glioma, parasitic cysts,
haemangioma, neurofibroma. Dermoids get calcified occasionally.
Changes in superior orbital fissureThe superior orbital fissure widening is more
common than its narrowing which is brought about by encroachment of growth over the
fissure and as a congenital malformation.
Widening of the superior orbital fissure is generally seen in adults as part of intra
clinoidal aneurysm. Intra cavernous aneurysm, carotico cavernous fistula, extension of intra
orbital growth backwards or spread of growth from cranium. Sometimes neurofibroma and
pseudo tumours of orbit may also widen superior orbital fissure.
Changes in optic canal and foramen
Usual diameter of optic canal varies between 4.4 to 6 mm in adults. At birth the canals
are narrower, reaching the adult size by 5 years of age, diameter greater than 7 mm is always
pathological. Generally diameters of both the canals are more or less similar. Any difference
of more than 1 mm between the two canals is also pathological. For comparison both the
canals should have identical X-rays preferably taken on the same plate. The enlargement
may be concentric or irregular. Uniform concentric uniocular enlargement is seen in optic
nerve glioma. Other causes in children are neuro fibromatosis, intra neural extension of
retinoblastoma. In children neurofibroma and retinoblastoma cause uniform irregular
enlargement.
Computed tomography
23
Computed tomography has emerged as an important tool in orbital imaging. It is safe
with minimal radiation hazard, which is as less as in X-ray. It is a non invasive outdoor
procedure which uses computer assistance. X-ray Tomography is axial as well as coronal in
section. A combination of axial and coronal section when programmed enables a lesion to be
seen in three dimensions. Its use in orbit is based on ability in differentiating between relative
tissue densities. Retrobulbar fat is in plenty in orbit and surrounds many structures. The
retrobulbar fat is hypo dense on X-ray in relation to other structure, hence a good contrast is
ensured. The bones have maximum density, the orbital fat and vitreous have least density,
other structures have intermediate densities. The vascular structures are best visualised by
using intravenous iodine containing contrast dye.
24
Besides orbit the intra cranial and paraorbital structures are also well delineated in
the same exposure. This enables to visualise growths extending from the orbit into the cranial
cavity and vice versa. It also demonstrates other intra cranial lesions not directly related to
the orbital lesion. The structures well visualised are the walls of the orbit, optic canal, optic
nerve, extra ocular muscles, globe, intra ocular mass. Intra ocular, orbital and cranial foreign
bodies. It is very sensitive in detecting orbital, intra ocular, and cranial calcification. Fractures
are well demonstrated. The superior orbital fissure is better visualised in contrast to inferior
orbital fissure. The sclera is demarcated clearly. It is not possible to differentiate the layers of
the globe separately in normal eye. However anterior chamber, lens and vitreous chambers
are seen separately. Generally 4-5 mm sections are taken routinely from vertex to base of the
skull, parallel to the optic nerve. The coronal plane is parallel to equator of the globe similar
to Caldwell view of X-ray. The computerised tomography can not differentiate between
pathological and normal tissue of same density. The walls of the orbit are clearly seen in both
axial as well as coronal slices. The roof and floor are seen better in coronal section so are the
superior, medial and lateral recti. The levator palpebral superior is not very clearly seen. All
these muscles are seen as elliptical masses in coronal section, in axial view they have fusiform
shape. The optic canal, intra orbital part of the optic nerve, sometimes its meningeal covering
are seen in axial view. The medial and lateral recti are seen as dense masses extending from
equator of the eyeball to the apex of the orbit.
Computed tomography is ordered in children to evaluate orbital soft tissue mass, intra
ocular and intra cranial calcification. Evaluation of trauma, intra orbital and intra cranial
foreign bodies.
Common intra orbital lesion where CT is used are
24
:
1. Retinoblastoma of all stages, post radiation status, recurrence and intra cranial
extension.
2. Rhabdomyosarcoma
3. Capillary haemangioma is best seen in contrast, may show calcification.
4. Optic nerve glioma with or without neurofibroma
5. Dermoids and teratomas
6. Pseudo tumours
7. Orbital cellulitis
CT may be a good diagnostic tool in optic neuritis. Moderate and symmetric enlarge-
ment is seen in raised intra cranial pressure.
In children dermoid cyst, rhabdomyosarcoma, optic nerve glioma, lymphangiomas give
well circumscribed and sharply defined appearance. Capillary hemanioma, pseudo tumours,
plexiform neuroma, leukaemia give ill defined view.
25
Non ocular causes of loss of vision in one eyeoptic neuritis, uniocular papilledema,
ophthalmoplegia of unexplained origin. Conformation of ultrasonic findings are some other
indications of CT of orbit.
Magnetic Resonance Imaging (MRI)
Magnetic resonance imaging is a non invasive method of imaging that does not use
ionising radiation used in common X-ray and computed tonography. The principle of MRI is
that thin anatomic section similar to CT are viewed by exposing the patient to an intense
magnetic field
26
. Besides magnetic field, a radio frequency wave and an atomic nuclei possessing
an electric charge should also be available. Hydrogen atoms is used to generate MR signals
because of its abundance in the body. The magnetic field is measured in a unit called Tesla
(T). Most of the MRIs uses 0.1 to 1.5 T of magnetic field. The process is not hampered by bone.
It can very well differentiate between normal and abnormal tissue due to its high resolution.
The metabolic profile of the tissue is also obtained.
Indications of MRI are ocular, orbital and neuro ophthalmic.
The ocular indication consists of ocular trauma, suspected nonferro magnetic intra
ocular foreign bodies, growths obstructed by opacity in media. In such cases first choice should
be ultrasonography but if ultrasonography findings are equivocal, the eye should be subjected
to CT and MRI. Latter gives better view due to excellent resolution. The MRI can differentiate
between retinoblastoma and intra ocular haemorrhage, exudates, Coats disease, choroidal
efflusion and toxocariasis. Papilledema, optic neuritis, lens, vitreous and sclera do not normally
enhance on post contrast MR.
29
Orbital indication areProptosis of any kind, orbital cellulitis, pseudo tumours,
neoplastic growths. Haemangiomas are well visualised in dye contrasted MRI. Magnetic
resonance imaging is better than CT in evaluation of intra canalicular lesions. Orbital fat and
optic nerve sheath do not enhance. MRI does not image bones well. MRI is contra indicated in
suspected fero-magnetic foreign bodies. It should only be performed when such foreign bodies
have been excluded by CT. MRI induced blindness in presence of metallic foreign body has
been reported.
29A
Neuro ophthalmic indications consists of :
Optic neuropathy of unknown origin, unexplained cranial nerve lesions, multiple
sclerosis, paralytic squint, visual field defects not related to optic neuropathy. Lesions of
sellaturcica, cavernous sinus and optic chiasma. It is widely used in suspected extension of
retinoblastoma in the intra canalicular part of optic nerve and chiasma.
Some of the important indication of MRI in children areevaluation of proptosis of
sudden origin, suspected optic nerve sheath - complex lesions, organic foreign bodies,
retinoblastoma with extra ocular extension. In children dermoid, rhabdomyosarcoma and
glioma of optic nerve give well circumscribed image while capillary haemangioma, pseudo
tumour, plexiform neuroma and leukaemia give ill defined image. The child may have to be
sedated if not cooperative.
Advantages of MRI are :
1. The process is devoid of radiation hence it is safer than CT and X-ray. The child can
be exposed to MRI for long period without any harm.
2. The procedure is not hampered by boney orbit.
3. Soft tissue image is seen better.
Ultrasonography with special reference to orbital diseases in children
General
Ultrasonography is one very important non invasive, non traumatic method. It does
not use ionic radiation or magnetic field as used in X-ray, CT or MRI. Ocular ultrasonography
does not have any side effect, can be used very frequently. It can be done as office procedure,
bed side examination or in the operation theatre as this is a small handy instrument. It can
be used in children with ease. However a non co-operative child should be suitably sedated by
a trained anaesthetist.
Ultrasound
Speed of sound in vacuum is far less than that of light which is 3x10
10
cm/sec or 186,000
mile/second. Ultrasound is produced in the transducer mechanically at a rate of 20,000 cycle
per second (20 kHz). This is not audible to human ears. Diagnostic ophthalmic ultrasonography
uses 10 omega hertz (10Mhz). Ultrasonography of other parts of the body use 2.5 to 5 MHz.
Higher the frequency of ultra sound, lower is the penetration. Thus, to measure corneal thick-
ness or depth of AC 12-15 MHz are used while that for globe is 10 MHz and orbit is 7 MHz. The
ultrasound behaves more or less like light wave, it is reflected, absorbed and refracted. Maxi-
mum ultrasonic waves are reflected when it falls at right angles. Ultrasound can also disrupt a
target tissue and generate heat, the later two are used in treatment and not diagnosis.
Basically a diagnostic system consist of (1) a tranducer, (2) a black box that detects the
reflected beam and prepare for display, (3) Display.
The transducer (The probe)
This part of the system is most important and has been called the heart of the system.
It is both a transmitter as well as a receiver. The transducer is made up of either quartz
crystal or a ceramic plate, which send out sound wave when charged electrically. The same
crystal picks up the returning waves and prepare it for visual display and photography. The
mechanism is called piezoelectric effect. This converts one form of energy to another form, in
ultrasound it converts electrical energy to sound energy, which is again converted to visible
electric form. The transducer of A scan is called stationary because there is no moving part
inside the probe. Though the probe itself is moved by the examiner to scan various parts of
the eye. In the transducer of B. Scan, the crystal inside the probe moves to and from. The
pulse is focussed and directed into the ocular tissue. The pulse after striking the tissue travels
back to the probe. This process is repeated may times each second.
The distance of the target tissue can be calculated by noting the time to travel the echo
from the tissue, if the velocity of the sound is known. In A scan this is processed by a built in
computer that automatically measure the distance and displays on the screen, which can be
photographed for future reference. The resolution depends upon the frequency. Higher is the
frequency, greater is the resolution but as frequency increases penetration decreases.
30
The electronically amplified sound wave signal received by the probe is called its
sensitivity or gain. When the sensitivity is more, the spikes of A mode are taller and the dots
of B mode are brighter.
Other factors on which the height of A Scan and brightness of B. Scan depend are
angle of sound beams when it strikes the tissue, relative difference between the two tissues,
the size and shape of the inter-face.
Sometimes some echoes are noted during ultrasonography, which are not produced by
anatomical structures, these are called artifacts. Commonest artifact is an air bubble either
in the probe itself, in the coupling medium used during ultrasonography or in the eye. Air
bubble can get into the eye either by accident as in penetrating injury. It can be produced by
gas forming organism or may be introduced deliberately as part of surgery. Intra ocular foreign
bodies sometimes bounce back and forth waves inside the eye causing a chain of echoes behind
the foreign body. This phenomenon is called reverbation. The foreign bodies that produce
such artifacts are - intraocular lens, glass particles or steel pieces. Air is a potent reflector of
sound in case of a penetrating injury. It is often difficult to differentiate echo produced by
retained intra ocular foreign body and air bubble.
31
Modes of display
30
There are two modes of display on screen that can be photographed as well. They are A
and B modes also called A Scan and B Scan respectively.
A Scan. A scan is a simple instrument but findings are more difficult to interpret. This
mode is called time amplitude. The picture produced is unidimensional. The spike has a base
line and a vertical deviation. The spike is a triangular configuration, base of which is formed
by the base line and the point where two slanting arms meet is the apex. The distances
between the two arms on the base line is called width of the spike. The left arm is called
ascending limb while the other arm is called descending arm. The height of each vertical
deflection is proportional to intensity of the echo. The base line represents the distance and
depends on the time required for the sound to reach the target, to be bounced back to reach
the probe.
A Scan is used to find out axial length of eyeball, AC depth and corneal thickness. It is
most widely used to calculate power of the intra ocular lens. However intra ocular growths,
intraocular foreign bodies and retinal detachment can also be seen by A mode. The difference
between anterior corneal echo to retinal echo in a normal eye is the axial length of the eye.
The cornea gives two spikes, one from the anterior surface and other from the posterior
surface as the cornea is relatively thinner than other structures i.e. lens, IOL. The distance
between the two is very small. The lens when clear also gives two sharp spikes, one from the
anterior surface and other from the posterior surface in case of homogenous lens. In case of
nuclear sclerosis, two additional echoes represent anterior and posterior surface of nucleus.
In an aphakic eye, the corneal spike is followed by the retinal spike, however, if the posterior
capsule has been left it gives one additional spike. Occasionally the aqueous, vitreous interface
may also give a spike. A clear vitreous is echo free and gives widest gap between the two
peaks. Floaters, haemorrhages, endophthalmitis, foreign bodies produce echo inside vitreous.
A high sharp echo from the retina denotes a normal retina. To measure the axial length, the
beam should pass through the centre of cornea and macula. A posterior staphyloma can also
be seen on A Scan.
B Scan. This is a two dimensional display that is shown as dots of various brightness.
Clinical interpretation depends on the brightness of the dot. A structure that produces a tall
spike on A Scan will give bright picture on B Scan as well. The B Scan is said to reveal not
only geometry of the structure but also its geography in the form of shape, size and position.
It gives the information in all quadrant as the probe is moved all over the globe including the
axial length. However for power calculation, A Scan should only be used. It is more useful
than A Scan in diagnosis of vitreous opacity, vitreous haemorrhage, retinal detachment, intra
ocular tumour, intra ocular calcification. B Scan is used to detect path of intraocular foreign
body and occult rupture of the globe.
Following conditions show calcification on ultrasonography(All ages)
Retinoblastoma, optic nerve sheathmeningioma, osteoma, choroid, haemangiomachoroid,
toxocara, granuloma, chronic retinal detachment, drusen of optic nerve head, phthisis bulbae,
intumescent and hyper mature cataract asteroid bodies in the vitreous.
Orbital inflammatory disorders in children
Orbit in children may have acute or chronic inflammation of contents of the orbit,
periorbita or bones. Acute orbital inflammation is generally unilateral. Anatomically infec-
tions of orbit may be preseptal or orbital. A useful classification of orbital infection based on
anatomical spaces is -
1. Preseptal or periorbital
2. Orbital cellulitis
3. Orbital abscess
4. Periorbital abscess
5. Cavernous sinus thrombosis
Preseptal orbital cellulitis (cellulitis lid) is very common, easy to diagnose and treat
unilateral inflammation limited by skin anteriorly and orbital septum behind. Generally the
orbital contents are not involved. Pre auricular and sub mandibular glands are enlarged.
Common organism that produce preseptal cellulitis are streptococcus, staphylococcus,
pneumococcus and haemophilus influenzae. The last organism is more common in children
below 3-4 years. It is associated with systemic involvement especially respiratory tract. It can
prove to be both sight and life threatening.
Predisposing factors in preseptal cellulitis areinjury, insect bite, stye, acute sinusitis.
The symptoms are mild, may have fever and malaise. It is more frequent in upper half than
lower. Clinical pictures depend upon duration and severity of the condition. In a fully
established case, the lid is swollen, hyperemic and tense, obliterating in palpebral fissure. It
is impossible to evert the lid. In case of H. influenzae, the skin has a bluish pink tinge. There
may be tender points on the lid margin denoting a stye. The swelling may spill over the
midline, causing edema of the other side without infection. The pre auricular and sub
mandibular lymph nodes are enlarged. Most of the time lid retractors are required to examine
the eyeball. The eye is generally non congested, the cornea is bright and papillary reaction is
normal, movements are not restricted and the vision is not reduced.
Treatment consist ofHot fomentation, oral analgesic oral antibiotics in divided doses.
Complications are formation of a lid abscess and rarely cavernous sinus thrombosis. If
a lid abscess develops fluctuation it should be drained through a horizontal incision and the
pocket of pus broken. The wound may be packed with cotton gauze impregnated with antiseptic
for twenty four hours.
Periorbital cellulitis
This is generally periostitis, there may be bony involvement
9.36, 37
. It may be peripheral,
inflicting the orbital margin or may develop deep in the orbit near the apex of the orbit. The
infection reaches the periostium either via veins that drain the paranasal sinuses or due to
direct spread from the paper-thin walls of the sphenoid and ethmoidal sinuses. It may result
following compound fracture of the orbital rim or due to penetrating injury.
There are two distinct types :
1. An acute stage due to strepto, staphyloor pneumococci or haemophilus influenza.
This presents as tense swelling due to accumulation of pus between the rigid bony
wall and periostmus. This is a painful condition, may push the eyeball to the opposite
side, cause mild proptosis, is generally confused with acute orbital cellulitis. The
diagnosis is confirmed by CT and MRI. The condition may subside following admin-
istration of broad spectrum antibiotic without any residual effect or the pus may
find its way to the orbital margin, conjunctival fornix, inside the orbit or even the
cranium. This may lead to a sinus formation.
2. The deep type of periostitis is associated with proptosis, extra ocular muscle palsy
and optic nerve involvement generally mistaken for cavernous sinus thrombosis.
The chronic variety is generally seen in the orbital margin due to trauma, tuberculosis,
unresolved acute peri orbititis. It presents with hard tender swelling on the orbital margin
that is fixed to the bone. There may be an external sinus or a fistula. Sinus formation is more
common with tuberculosis.
Treatment of the chronic tubercular lesion is treatment of the primary cause. Other
causes require long term broad-spectrum antibiotic, drainage of the abscess and curettage of
the sinus.
Orbital cellulitis
32,33
Orbital cellulitis is common in children. It may be seen at any age but common at about
five years.
38
It is an acute purulent infection of contents of orbit that may involve more than
one structure. It is a potentially sight threatening and sometimes may lead to loss of life due
to involvement of central nervous system and septicimia.
35
It is an unilateral condition caused by strepto, staphylo and pneumococci. It is generally
single organism infection. H. influenza has emerged as a main causative organism especially
in children under three years of age. The commonest route for organism to reach the orbit is
via paranasal sinuses. All paranasal sinuses can cause orbital cellulitis, however, ethmoidal
sinusitis is the most frequent source.
39
Other common route is vascular spread from the
sinuses, yet another mode of spread from the sinuses is dehiscence of the orbital wall of the
sinus or its fracture. Generalised septicimia in a malnourished child and infection following
penetrating injury can also introduce infection from outside. Occasionally septic teeth or
acute dacryocystitis has also been observed to cause orbital cellulitis.
The infection is always acute. It starts with symptoms of fever, malaise, pain in the
periorbital region and headache. The lids are swollen, in case of H. influenza the lid has a
bluish red hue.
The lid itself is not primarily involved as the infections start in the orbit and is localised
behind the orbital septum. Accumulation of pus, inflammatory substances and edema in a
closed compartment cause sever pain, the eyeball is proptosed. As ethmoid and sphenoid are
common source of primary infection. The eyeball is proptosed forward and laterally. The
movements are restricted either due to edema of the muscles or paralysis of extra ocular
muscle due to cranial nerve involvement. The conjunctiva is chemosed, however, the cornea
is bright and AC does not show any reaction. Evidence of anterior chamber reaction denote
uveal involvement. Dilated pupil is common with third nerve involvement. Fast deterioration
of vision should warn about involvement of optic nerve that may be optic neuritis, central
artery occlusion or central vein thrombosis.
Investigation show :
1. Leucocytosis
2. X-ray orbit shows a hazy orbit with hazy paranasal sinuses
3. CT and MRI shows fluid and air in the sinuses
4. Ultrasonography helps to differentiate other conditions that cause fast growing
proptosis in a child. Swabs from conjunctiva or nasopharynx are seldom conclusive.
Differential diagnosis consist of cellulitis lid, cavernous sinus thrombosis, rhabdomy-
osarcoma, leukimic deposits, orbital deposit of neuroblastoma, retained intra orbital foreign
body.
Complications may vary from discharging sinus on the orbital margin to meningitis,
brain abscess, cavernous sinus thrombosis. If the cornea is exposed due to proptosis, cornea
may develop ulceration that may slough and perforate. Occasionally tenonitis may develop.
Management of orbital cellulitis
As the condition is potentially life and sight threatening efforts should be made to save
both in consultation with ENT specialist and pediatrician. Treatment of choice is parentral
administration of ampicillin in divided doses. If there is chance of penicillinase producing
organism, the antibiotic should be methacellin. Other antibiotic generally used arenaficillin
or oxacillin
41
.
With intense antibiotic administration the condition should subside in five to seven
days. If the condition does not subside CT should be repeated to locate the pus and drain it.
The abscess is drained through an area that shows maximal fluctuation near the lid margin
avoiding trochlea, superior oblique and lacrimal gland. Proptosis, chemosis of conjunctiva
and lateral rectus palsy of the contra lateral eye strongly suggest cavernous sinus thrombosis.
Orbital and sub periosteal abscess
38,42
Primary abscess of orbital and periostial space is extremely rare. It is generally second-
ary to non resolving orbital and periosteal cellulitis. Accumulation of pus among the contents
of orbit is known as orbital abscess and pus under periostium is called as sub periosteal
abscess. The organisms are same as that cause orbital cellulitis and sinuses are generally the
source of infection. There is always some proptosis depending upon the size and location of
abscess. Periosteal abscess generally leads to eccentric proptosis. An orbital abscess in the
vicinity of optic nerve can cause purulent optic neuritis and retinal vascular obstruction. If
the lids fail to protect the cornea, there may be exposure keratitis, ulcer and perforation.
Diagnosis is conformed by CT and MRI.
Treatment consists of change of antibiotic and its dose. If there is palpable fluctuation,
the abscess should be drained through the maximum area of fluctuation. Posteriorly placed
abscess are best drained via ethmoid.
Cavernous Sinus Thrombosis
To understand the pathology of cavernous sinus, it is essential to know general outline
of applied anatomy of cavernous sinus.
The cavernous sinuses are situated on either side of the sphenoid bone. The body of the
sphenoid lies between the right and left halves of cavernous sinus. The sellaturcia lies above
the sphenoidal sinus. The cavernous sinus starts at the end of the superior orbital fissure and
ends posteriorly near the apex of petrous bone. The Gasserian ganglion comes in close prox-
imity of the posterior end of the sinus. The sinus is formed by splitting of the dura and is
traversed by trabeculae that gives it a spongy look. The sinuses of each side communicate
with the sinuses of other side by transever sinuses of cavernous sinus.
The walls of the trabecular space are rough, making is vulnerable for thrombus forma-
tion. Each cavernous sinus is formed by superior ophthalmic vein, central retinal vein, part of
inferior vein and spheno palatine sinus. Thus, the cavernous sinus is the reservoir of venous
drainage from scalp, face, orbit and most part of the cerebrum. The superior and inferior
petrosal sinuses drain backwards. The petrosal sinuses drain into sigmoid sinus that finally
drain into juglar vein. The tributaries of cavernous sinus are valve less and are prone for
infection to travel both ways.
42
The contents of the cavernous sinus are internal carotid artery along with sympathetic
plexus. The sixth nerve lies inferio lateral to the artery in the substance of the sinus making
it more vulnerable than other cranial nerves. The other nerves lies in the lateral wall of the
sinus in the posterior part. The third nerve is superior most followed by fourth and two
divisions of fifth nerve. In the anterior part of the sinus, the fourth nerve changes its course
to be superior to the third nerve. However this superior course of the fourth nerve is very
short.
42
Infection of the cavernous sinus is basically thrombo phlebitis which used to be almost
cent percent fatal in pre antibiotic era. Even today it is common in malnourished and chronically
ill children and remains sight threatening and life threatening condition. The cavernous
sinuses can get infected as part of acute bacterial inflammation or by chronic granuloma. The
former is generally refined to a cavernous sinus thrombosis while the later is known as anterior
cavernous or superior orbital fissure syndrome. A third kind is known as a septic thrombosis
that results following head injury and neuro surgical manipulation.
44
Diagnosis of cavernous sinus thrombosis is straight forward when there is tenderness
over the mastoid, proptosis, in 50% cases proptosis is bilateral, restricted movement of the
globe, contra lateral sixth nerve palsy. Only condition that mimic cavernous sinus thrombosis
is orbital cellulitis which is invariably unilateral. Presence of orbital cellulitis can be confirmed
by CT and MRI. X-ray skull has no role in diagnosis of cavernous sinus thrombosis.
Management of cavernous sinus thrombosis is a medical emergency. The child should
be hospitalised and put on heavy dos of parental antibiotic. The cornea should be protected by
antibiotic ointment and lubricants.
Pseudo tumour orbit
Pseudo tumour orbit is the cause of proptosis in ten percent of all cases. It is generally
seen in adult male in fourth and fifth decade. In adults, it is generally unilateral. About one
tenth of proved cases of pseudo tumour of orbit however belong to the pediatric age group,
where it is invariably bilateral.
47
Early diagnosis of this benign growth in children is required
because it mimics rhabdomyosarcoma, metastatic neuroblastoma and leukaemic infiltration,
all of which pose a threat to the life of a child and mistaken diagnosis of pseudo tumour in a
case of malignancy can cause death of the child.
The condition has been defined as an idiopathic localised inflammatory disease
47
. The
disease acquires its name from the fact that it masquarades as neoplasm with a histopathological
picture of inflammatory granuloma. The exact etiology is not known, the condition is perhaps
multifactorial in which auto immune responses to antigens has been implicated.
48
Vasculitis, myositis granuloma, thickening of tissue, lymphatic infiltration are com-
mon.
9
The disease generally has a torpid course, it may have an acute onset or may linger for
long time causing extensive fibrosis leading to a condition called frozen orbit. Children may
show variable systemic symptoms.
The characteristic presentation is a slow growing proptosis which may be axial when
the growth is intra conal or eccentric when it is extra conal. The growth may involve any part
of the orbit from the lacrimal gland to apex of orbit. It may even invade anterior part of the
cavernous sinus. The proptosis is irreducible and does not change with posture or valselva
manoeuvre. The mass is palpable between the orbit and the globe when it is anteriorly placed.
The extra ocular muscle are involved in various combinations causing tropia resulting in
diplopia or complete immobilisation of the globe. The conjunctiva is generally congested and
chemosed. Corneal anaesthesia is a common feature due to involvement of fifth nerve otherwise
cornea is bright and transparent unless it protrudes beyond the lid. In such cases, there may
be exposure keratitis.
As multiple structures are involved, the symptoms are variable. Initial symptoms are
redness of eye, headache and diplopia. The parents may complain of gradually developing
enlargement of the eye, which turns out to be a proptosis. Pain in the distribution of fifth
nerve is common. Squint and diplopia are invariably present, loss of the vision should draw
attention to optic neuritis, a growth behind the globe pushes the posterior pole forwards
leading to hypermetropia.
Diagnosis is by exclusion, confirmed by X-ray orbit + ultrasonography, CT, MRI, fine
needle biopsy and excessional biopsy. A therapeutic trial by oral steroid is advocated in adults.
It is avoided in children. The first step is to give proper attention to possibility of fatal condi-
tions i.e. rhabdhomyosarcoma, leukaemia and metastatic neuroblastoma. Foreign body
granuloma, unresolved orbital cellulitis and specific granuloma of the orbit are rare but pos-
sible differential diagnosis.
X-ray orbit may also show enlargement of the orbit.
49,50
A high resolution CT is more
helpful because it provides more information about periorbital structures as well.
42
A Scan
has no value in this condition. On B Scan a characteristic irregular mass in retro bulbar space
is visualised. Other findings on CT are orbital myositisthickening of the muscle, lateral and
inferior recti are more involved, the insertion of the muscles also thickened. The posterior
sclera and choroid may also show thickening.
48
MRI is more sensitive and help to localise the
small growth more precisely. Fine needle biopsy unless done by a person who is well versed
with the procedure may be futile. Even in case of positive specimen, histopathologist may
only be able to see if it is a malignancy or not but not the exact nature of the growth. Excisional
biopsy under general anaesthesia through anterior orbitotomy or trans ethmoidal approach
gives the best result to pinpoint the diagnosis.
Management
Most important part of the management is to be sure that the case under consideration
is not malignant. Rhabdomuosarcoma, which is the commonest malignant tumour of the orbit
in children may present with feature of inflammation and may show initial regression with
systemic steroid.
There is no specific treatment for pseudo tumour. The tumours completely regress
under full dose of steroids orally over few months. Inter lesional steroids also give some relief
but is difficult to administer in a child. While the child is under steroid careful watch should
be kept on possible ocular and systemic side effects. The drug should be tappered over weeks.
Other methods used areradiation and cytotoxic drugs in case of steroid resistant
cases. Radiation consists of 15-25 Gy given in consultation with radiologist in divided dose.
Cytotoxic drugs commonly used are cyclophosphamide, chlorumbucil, metho trex ate, cyclosporin
separately or in combination in collaboration with pediatric oncologist and physician.
Complications of pseudo tumours arerecurrence, poor response to steroids, strabismus,
amblyopia, exposure keratitis, optic neuritis, papilledema, frozen orbit, permanent diminished
vision.
Tenonitis
9,37
This is inflammation of Tenons capsule. It is rare in children except as complication of
orbital cellulitis. Besides orbital cellulitis, severe scleritis and posterior uveitis can cause
tenonitis in adults. The condition is painful, the eye is axially proptosed. There may be diplo-
pia with restriction of ocular movement without paralysis of extra ocular muscles. There may
be associated optic neuritis.
Diagnosis is confirmed by CT and MRI.
Management in children is meticulous treatment of orbital cellulitis.
Common orbital tumours in children
Tumours in children can be primary or secondary or extension from other structures
(retinoblastoma). The secondary tumours are always malignant. They may metastasise from
a distant organ i.e. neuroblastoma, Ewings sarcoma, Wilms tumour or leukaemia. The primary
tumours are mostly benign. The benign tumours inflicting orbit in children areNeurofibroma,
capillary haemangioma, dermoids, teratomas, optic nerve glioma.
Commonest type of primary malignant tumour of orbit in children is rhabdomyosarcoma.
Rhabdomyosarcoma
54, 55
Rhabdomyosarcoma may develop at any age even at birth. In children it is the most
common primary malignant tumour of the orbit. Average age of presentation is seven years.
In adults it spares the orbit and develops in skeletal muscles. Surprisingly in children it does
not develop from extra ocular muscles as it was thought to be in the past.
45
However
rhabdomyosarcoma may present as a mass in the lid.
42
No race is immune, there is no
predilection for right or left eye. It is unilateral. Boys have slightly more predilection. It is
highly malignant and cent percent fatal when not treated. Mortality level has been considerably
reduced due to radio therapy and chemo-therapy. It develops from pluri potential embryonal
mesoderm which commonly differentiates to form rhabdomyoblast as seen in foetus.
52
The
growth may originate in the ethmoid and extend in the orbit. It may develop as second
malignancy in case of retinoblastoma. Histopathologically there are three types of
rhabdomyosarcoma1. The embryonal, 2. Aluelar and 3. pleomorphic. The first is seen in
about 70% of cases, the last is least common.
The tumour may arise anywhere in the orbit but commonest site is superio nasal
quadrant. It starts as a painless rapidly growing mass that pushes the globe towards the
opposite direction. However it may present with inflammatory signs and mistaken as orbital
cellulitis or vice versa. The mass can be palpated by insinuating a finger between the globe
and the orbital wall. The growth is non compressible. It does not invade the globe, which may
otherwise be damaged and lost due to proptosis, exposure keratitis. The tumour may extend
into surrounding structures by direct invasion. Distant metastasis is via blood stream.
Lymphatic spread occurs when lid, conjunctiva and para orbital tissues are involved. In such
case preauricular submandibular and neck glands may be involved.
Differential diagnosis consists of orbital cellulitis, orbital neuroblastoma and
retinoblastoma, leukaemic deposits, infected dermoids, Burkitts sarcoma, pseudo tumour.
As the tumour is relatively fast growing and fatal, it should be treated early. Diagnosis
is confirmed by X-ray that may or may not show boney erosion and enlargement of the orbit.
CT and MRI are more definitive to assess the location, size of the tumour and plan treatment.
Presence of bone erosion carries less favourable prognosis. Excisional biopsy is better avoided.
Fine needle biopsy is not always reliable.
1. Treatment directed towards the local growth.
2. Treatment of distant metastasis
3. Handling of local complicationsOnce upon a time debulking and excentration were
only treatment available. At present exentration has been given up. Sometime
debulking is used to be followed by chemotherapy and radiotherapy. The tumour
responds very well to combined chemotherapy and radiotherapy in which the survival
rate is as high as 90% when the tumour is confined to orbit. With extra orbital
extension this drops to 65%. The tumour is fatal within 18 months if not treated.
Death is due to multiple organ failure following distant mertastasis. Many chemo-
therapeutic drugs have been used from time to time with variable results. They
areandriamycin, vincristine, cyclo phosphamide and actinomycin D (dactinomycin).
A combination of vincristine, actinomycin D and cyclophosphamide (VAC) is most
commonly used as adjunct to radiotherapy.
Radiotherapy consists of external beam radiation directed to the orbit. Total dose ranges
between 4000 cGy to 6000 cGY given over four to six weeks protecting the cornea and lens.
Side effects of radiation aremadarosis, dry eye, cataract, retinopathy of radiation. In children
with diminished orbital volume, the size of the orbit may stop growing.
51
Common secondaries that involve orbit in children are :
Neuroblastoma
Leukaemia
Retinoblastoma
Ewings sarcoma
Wilms tumour
Burkitts lymphoma
Out of the above retinoblastoma and Burkitts lymphoma are not true metastatic tu-
mour of orbit. They invade the orbit from primary source i.e. from the eyeball in case of
retinoblastoma and from the maxilla in case of Burkits lymphoma.
Neuroblastoma
Neuroblastoma is a common malignancy of infants and children. It develops from
embryonal neuroblast generally from adrenaline gland and sympathetic chain. However in
3% cases it develops from sympathetic plexus in orbit. Most of the neuroblastomas develop
under seven years of age. The primary develops either in abdominal or thoracic segment of
the sympathetic chain. It may develop in the cervical sympathetic as well. Fifty percent of
children who develop neuroblastoma are under two years of age.
54
The metastatic
neuroblastoma rarely show rossette formation which is seen mostly in primary site. There
are two types of neuroblastoma i.e. (1) that has predilection for orbits known as Hutchinson
type; (2) that metastasise in liver known as Pepper type. The metastasis is mostly blood
borne, which develop in the orbital bones and from which it spreads to the orbital
54
contents.
Metastatic neuroblastoma is generally bilateral, which is detected few months after
the primary has been discovered. However proptosis may be the first sign of neuroblastoma
and generally attributed to a doubtful trauma because of one of the clinical feature is ecchymosis
of the lid. Commonest presentation is fast growing proptosis in a child with a lump in abdomen
or a para vertebral shadow on X-ray. The lids are swollen and ecchymotic. Forty percent of
children with neuroblastoma develop orbital metastasis.
53
The mass not only invades the
orbit but may form a lump in the cheek, temple. Zygomatic bone is the commonest bone to be
involved.
Neuroblastoma should be differentiated from orbital cellulitis, infected dermoid, black
eye, retrobulbar haemorrhage, rhabdomyosarcoma, Burkitts lymphoma and leukaemia. or-
bital retinoblastoma is diagnosed with ease due to presence of white reflex in pupillary area.
Diagnosis is confirmed by CT, MRI, X-ray chest, ultrasonography of abdomen. A body
scan may be required to locate the primary, fine needle biopsy may be helpful.
Treatment consist of radiation and systemic chemotherapy. In radiation external beam
photon is directed to the orbit. Total dose may vary 1500 to 3000 cGy in divided doses. Chemo-
therapy in neuroblastoma gives long term regression in about one third cases. However it
should be remembered that radiation and chemotherapy are only paliative.
Ewings sarcoma
55,56,57
This is less common metastatic tumour of the orbit in children than neuroblastoma.
Common age is first and second decade. The tumour has been observed more frequently in
second decade. In contrast to neuroblastoma this tumour has para sympathetic origin. It
generally develops in bones and from there it spreads to orbit. It may develop as second
tumour following radiation in children who have undergone treatment for retinoblastoma. It
presents as proptosis with or without visual loss. It must be differentiated from
rhabdomyosarcoma, neuroblastoma and other metastatic tumours of the orbit in children.
Treatment consists of chemotherapy and radiotherapy.
Burkitts lymphoma (Lympho sarcoma) is a relatively uncommon tumour, no race or
gender is immune to it. However, it is more common among coloured Africans. Common age is
three to seven years with affinity for orbit. It has been postulated that this tumour has a viral
etiology. The tumour starts in maxilla and erodes the floor of the orbit. It does not invade the
eyeball. It starts as a painless swelling over the maxilla, spread into the lower lid and con-
junctiva. The proptosis is rapid in progress. It may be bilateral. There may be involvement of
central nervous system and invariably leads to death of the child. Some of the children
58,59
may have abdominal mass. Proptosis may cause exposure keratitis with sloughing of cornea
and perforation. The lymphoma responds to chemotherapy and radiation. Commonly used
cytotoxic drugs are cyclophosphamide, methotrexate and vincristine. Relapse are common
and fatal.
Optic nerve glioma
61,62
Glioma of the orbital part of optic nerve is a manifestation of congenital hamartoma of
the anterior visual path.
It is a benign slow growing tumour of ectodermal origin. Commonly associated with
neurofibreomatosis.
45
Topographically gliomas of visual path are divided into two parts :
1. Orbital, 2. Cranial.
1. Glioma of the orbital part is again divided into two groups according to its potential
malignancy :
(i) Benign glioma of childhod and
(ii) Malignant glioblastoma of adult.
The benign glioma of childhood arises from the supportive tissue of the optic nerve i.e.
astrocytes and oligodocytes. The tumour is generally formed predominantly by one type of
these cells and has been divided into astrocytomas and oligodendro glioma according to
predominance of type of cells. Commonly found cells are pilocytic astrocytes.
The incidence of glioma of optic nerve falls gradually with age. Though the growth is
designated as congenital hamartoma, its common age is around seven years. The incidence
falls sharply from first to second decade, however, when found in adults it is malignant in
nature.
The optic nerve glioma is an unilateral slow growing tumour that starts near the optic
canal and spreads towards the globe in a fusiform shape. It is an intrinsic tumour of optic
nerve and does not involve the sheath of the nerve. The growth is generally not palpable.
Optic nerve glioma itself is painless swelling but the eye may become painful due to severe
proptosis. The proptosis is axial and slowly progressive over years. It is non pulsative,
irreducible, is not influenced by change in the position of the eye or straining. After years the
lids may fail to protect the cornea, resulting in exposure keratitis, corneal ulcer and perforation.
Reduction of vision is common but some vision may be retained for years, which is eventually
lost due to optic atrophy. In later stages the growth presses on the posterior pole of the globe
and results in axial hypermetropia. Unilateral diminished vision in childhood leads to
strabismus and amblyopia. The squint is always non paralytic.
Other clinical findings are presence of frank neurofibromatosis in about 25% of cases
or presence of neurofibroma in close relatives. Suspicion of neurofibromatosis amy be aroused
by presence of caf au lait in absence of obvious neurofibroma.
A child with :
1. Unilateral painless diminished vision
2. Slow proptosis
3. With caf au lait anywhere in the body is a case of optic glioma that can be confirmed
by X-ray, CT and MRI.
Biopsy of the growth is seldom indicated. Sometimes the iris may show neurofibromatosis
nodule (Lisch nodule). otherwise anterior segment has no findings that can be attributed to
glioma except afferent pupillary reaction. Fundus may show papilledema, post papilledematous
optic atrophy and even primary atrophy. The posterior pole shows retinal striation.
X-ray of orbit and optic foramen are most important diagnostic investigation. On plain
X-ray orbit :
(1) There may be uniform enlargement of the orbit on the side of the growth.
(2) Most characteristic finding is seen on X-ray examination of optic foramen by Rhese
view.
(i) Both the optic foramena should be examined at the same time.
(ii) The diameter of normal optic foramen is almost constant i.e. 6.5 mm.
(iii) Any foramen larger than 7 mm is sure evidence of enlargement.
(iv) When both foramena are compared, a difference of more than 1 mm between
the two is also diagnostic.
(v) The enlargement is uniform without erosion showing slow expansion of the
growth.
(vi) The edge gives a polished sclerosed appearance.
(vii) The optic canal is generally not enlarged as the tumour rarely invades the canal,
which is more common in retinoblastoma.
(viii) The sella may also show some changes in the form of J. shaped sella instead of
usual round sella. Changes in sella are more common in glioma of optic chiasma.
Ultrasonography and MRI show a well circumscribed, well delineated, fusiform
growth.
Chiasma glioma
63
does not differ in histopathology. However it may extend in to the
third ventricle or hypothalamus or may spread backwards in the optic tract. Involvement
of chiasma does not produce typical bitemporal field changes. The field changes are generally
stable as the growth is slow in expansion. The field changes are bizarre.
64
They are more
like optic nerve field changes than chiasma. Invasion of third ventricle leads to hypothalamic
syndrome consisting of obesity, diabetic insipidus, precociuspuberty, dwarfism, pan
hypopituitarism.
Management
As the growth is slow progressive, unilateral, non malignant and non fatal, there is no
urgency in instituting definite treatment unless the cornea is at risk. Corneal involvement
should be managed by - antibiotic and cycloplegic. If necessary the eye should be patched. In
case of poor response to patching a median tarsorrhaphy is advised.
Surgery is indicated for cosmetic reason and protection of cornea. In the past, the eye
was enucleated with as much part of glioma as possible. This procedure is seldom practised.
The surgical procedure is to remove as much of tumour tissue as possible either through an
orbital or transcranial route leaving the eyeball intact.
Radiation is indicated only when there is a tendency towards malignancy.
Leukaemia deposit in orbit in children
Lymphoblastic leukaemia is the commonest type of leukaemia that involves eye. It has
both intra ocular as well as orbital manifestation. Myeogenous and monocytic leukaemia has
less common ocular presentation. Orbital deposits are less common than intra ocular lesion.
The child is generally anaemic and has thrombocytopenia that may result in bleeding form
gums and cause epistaxis. The deposits are bilateral. The optic nerve may be infiltrated leading
to loss of vision. The lymphoblastic leukaemia have good prognosis with modern chemotherapy.
The proptosis regresses with chemotherapy combined with radiation.
Capillary haemangioma of orbit
Capillary haemangiomas are non neoplastic growths. They are hamartomas. Capillary
haemangioma may be present in orbit, in the lid, on the skin of the face. There may be a
combination of orbital and lid tumour. Some of the orbital tumours may be mistaken for lid
tumours due to purple blue coloration of the skin of the lid. Histopathologically, they are
blood filled endothelial channels without a true capsule. The growth is general diffuse but
may be well localised. Most of the capillary haemangiomas are congenital but not recognised
at birth. The presence of tumour becomes evident within first few months of birth when the
growth increases in size while the child strains. The growth is compressible without bruit.
The proptosis is unilateral and non tender. The growth is generally situated in the anterior
orbit in the superio nasal quadrant. The tumour has a very predictable mode of evolution that
is(1) Stage of rapid growth, (2) Stage of stability and (3) Stage of regression. The stage of
growth is generally limited to the first year, stage of stability lasts for one to two or three
years followed by regression by five years.
The tumours situated deep in orbit are less common and difficult to diagnose. Capillary
haemangioma may be associated with strawberry patches on the lid or skin. Sometimes dilated
vessels may be visible in the fornices. Besides cosmetic blemish the common complications
are associated myopia, astigmatism and amblyopia.
Diagnosis is straight forward in case of growth in anterior orbit by :
1. Careful history
2. Examination of earlier photographs that may reveal proptosis not noticed by par-
ents.
3. Presence of strawberry growth elsewhere.
4. X-ray orbit may show diffuse enlargement of orbit.
5. B scans show diffuse irregular mass.
6. CT and MRI give an ill defined appearance.
7. Contrast CT delineates the endothelial lined channel along with its feeding vessels.
Differential diagnosis consist oforbital dermoid, orbital cellulitis, rhabdomyosarcoma,
lymphangioma. Rhabdomyosarcoma has rapid progress without stage of stabilisation and
commonly seen at about seven years of age while lymphangioma which is also a hamartoma
is seen in teens with sudden enlargement due to haemorrhage in the growth that causes
chocolate cyst formation.
Management is divided into two groups -
1. Management of error of refraction and amblyopia. Amblyopia may be due to
uncorrected error of refraction or deprivation due to edema of lid or a growth in the
lid.
2. Management of the tumourAs the growth is self-limiting regresses without
interference. Most of them do not require any treatment however if there is evidence
of corneal exposure or compression of the optic nerve medial treatment with steroids
and interferon is indicated, both of them are always associated with possible side
effects, that should be monitored regularly in consultation with pediatrician.
Steroid can be given either (1) orally or (2) intra lesional injection. Oral steroid is
generally given in the form of prednisolone 1 to 2 mg/kg/day or 2 to 4 mg /kg/alternate day.
Shrinkage of tumour starts within two weeks but there is high incidence of rebound growth.
Intra lesional injection consist of 40 mg of triamcinolone with 6 mg of betathethasone
given in the growth under general anaesthesia and repeated after 8 weeks.
Orbital varices
Orbital varices are commonest cause of intermittent unilateral proptosis. The orbital
varices are part of vascular malformation of the orbit, which can be arterial, venous or arterio
venous
42
. The venous formation are known as orbital varices. They are pathological dilatation
of pre-existing venous channel. The commonest form is simple or primary varix that causes
unilateral, intermittent, non pulsatile proptosis in a child. Proptosis may be present at birth
and may be overlooked. The proptosis may disappear when the child is lying flat only to
appear when the venous pressure is increased as in crying, sneezing, straining or pressure on
the jugular vein. The proptosis becomes more when the child lowers the head. It may be
associated with prominent vessels on the conjunctiva, lid or scalp on the same side.
65
Besides
congenital malformation orbital varices can be seen following orbital trauma where the superior
orbital veins is torn. Some people presume that congenital varices are a variation of capillary
haemangioma and lymphangioma, which is slow progressive, non intermittent and stationary.
There may be dilatation of orbital veins secondary to intracranial AV malformation, generally
in adults. The primary varices keep on growing at slow pace till the child is in teens when the
progress stops.
The plain X-ray picture is characteristic which shows (1) Enlargement the orbit, (2)
Presence of round pheboliths, (3) There is a prominent vascular markings in the frontal bone.
Venography may show either localised dilatation or a system of diffuse abnormal channels
throughout the orbit.
Ultrasonography shows dilatation of superior ophthalmic vein that may increase in
size if venous pressure is raised. MRI gives better result as compared to CT.
Orbital varices rarely cause visual disturbance or strabismus. Only complain is cosmetic.
No specific treatment is required.
Histocytosis
Histocytosis is a systemic disease that affect children predominantly in various
combinations of ocular and non ocular clinical presentation. Previously they were known by
various names i.e. Histocytosis, Lettercrswie disease, Hand Shuller Christian disease and
eosinophilic granuloma
67
. Since 1987, they have been brought under only one name that is
Langerhans cell cytosis.
66
The Langerhans cells do not have any definite function. They are derived from bone
marrow. They are basically monocytes and present in various tissues in the body. The disorder
histocytosis seems to be an auto immune disease that responds to systemic steroid as well as
anti cancer drugs and radiation. The disease may start in infancy or early childhood. Common
orbital manifestations can be unifocal, eosinophilic granuloma of the bones of the orbit. However
bones involvement sometimes can be at more than one site. Multi focal involvement have
multiple organ involvement, with bilateral proptosis, involvement of orbital bone and diabetes
insipidus. Involvement of posterior pituitary is the cause of diabetes insipidus. There may be
multiple intra ocular and periocular involvement. X-ray of the orbit shows lytic lesion of bone.
Treatment consists of systemic steroid, vincristine, vinblastin, antibiotic and radiation.
Some probable features and probable cause of proptosis in children
Features Probable Cause
1. Pale child, moderately ill, bleeding from gums, 1. Leukaemia
thrombocytopaenia and bilateral
proptosis.
2. Palpable abdominal mass, radiologically visible 2. Metastatic neuroblastoma
thorasic mass, hepatomegaly, ecchymosis of lid,
unilateral proptosis.
3. Unilateral fast growing proptosis, fever toxic 3. Orbital cellulitis
child, X-ray showing sinusitis.
4. Fast growing palpable mass, unilateral proptosis. 4. Rhabdomyosarcoma
5. Proptosis with white reflex. 5. Retinoblastoma
6. Unilateral axial proptosis with early visual loss. 6. Glioma of optic nerve
7. Bilateral proptosis, fever, toximic, child cranial 7. Cavernous sinus thrombosis
nerve palsy.
8. Unilateral axial proptosis with caf au lait on the 8. Optic nerve glioma with neuro-
body. fibroma.
9. Port wine stain on the lid, forehead or anywhere 9. Capillary haemangioma
with proptosis.
10. Bilateral proptosis with deformity of skull and 10. Congenital anomaly of skull and face.
face.
11. Intermittent proptosis that increases with 11. Orbital varix
increased venous pressure.
12. Unilateral proptosis, pain, fever, hazy cornea. 12. Panophthalmitis
Loss of vision.
13. Bilateral proptosis, diabetes insipidus, involve- 13. Histocytosis (Hand-Schuller Chris-
ment of boney orbit. tian disease)
Orbital compartment syndrome
67
This consist of a group of condition that cause acute rise of intra orbital pressure that
result in elevation of intra ocular pressure, obliteration of central retinal artery and diminished
ocular perfusion. The causes could beHaemorrhage, emphysema, profound edema of retro
ocular structure due to trauma infection or inflammation like orbital cellulitis or expanding
orbital abscess and namely pseudo tumours.
The clinical features areDiminished vision, restricted movement of globe, afferent
pupil, proptosis and raised IOP tight lids.
Management
This should be treated as emergency and treatment consist of lateral canthotomy and
cantholysis. Reduction of IOP by IV Mannitol oral glycerine, broad spectrum systemic antibiotic
and systemic steroids.
REFERENCES
1. Nema H.V. ; The orbit in Anatomy of the eye and its adnexa. 2nd edition. p-5260, Jay
Pee Brothers. Medical Publishers, New Delhi, 1991.
2. Singh D. ; Pediatric cataract. CME series. All India Ophthalmological Society, New
Delhi.
3. Duke Elder S. ; The orbit (connective tissueplanes and spaces) in System of
Ophthamology. Vol. II. p-465466, Henry Kimpton, London, 1961.
4. Khurana A.K. ; Diseases of orbit in Modern Ophthalmology. Edited by Dutta L.C. 1
st
Ed. p-8587, Jay Pee Brothers, New Delhi, 1994.
5. Duke Elder S. ; Anomalies of the orbit and its contents in System of Ophthalmology.
Vol. III, Part II. p-9421072, First edition, Henry Kimpton, London, 1964.
6. Marilyn Miller, Pruzansky S. ; Cranio facial anomalies in Principle and practice of
ophthalmology. Edited by Peyman G.A., Sander D.R. and Goldberg M.F. Vol. III,
p-23552417, 1
st
Indian edition, Jay Pee Brothers, New Delhi, 1987.
7. Harrison A.R. ; Orbital hypertelorism in Current Ocular Therapy. Ed. 5, Edited by
Fraunfelder F.T., Roy F.H. p-189190, W.B. Saunders Company, philadelphia, 2000.
8. Schaffer D.B. ; Pediatric Ophthalmology in Text Book of Ophthalmology. 9
th
edition.
Edited by Scheie H.C. and Albert D.M. p-283286, W.B. Saunders Company,
Philadelphia, 1977.
8A. Nicholson D.H., Green W.R. ; Tumours of the eye, lid and orbit in Pediatric
ophthalmology. Vol. II. Edited by Harley R.D. Second edition. p-248249, W.B. Saunders
9. Ahmed E. ; Text Book of Ophthalmology. 1
st
edition. p-130137, Oxford University Press,
Calcutta, 1993.
10. Boger W.P., Peterson R.A. ; Pediatric Ophthalmology in Manual of ocular diagnosis and
therapy. 3
rd
edition. Edited by Deborah Pavan Langston. p-275, Littlebrown.
11. Robb R.M. ; Dermoid in Current ocular therapy. Edition 5. Edited by Fraunfelder F.T.,
Roy F.H. p-234235, W.B. Saunders Company, Philadelphia, 2000.
12. Vaughan D., Asbury T. ; General Ophthalmology. 9
th
ed. p-243244, Lange Medical
Publication, California, 1980.
13. Vaughan D. and Asbury T. ; General Ophthalmology. 10
th
edition. p-229282, Lange
Medical Publication, California, 1983.
14. Maya Eibschitz and Delmonte M.A. ; Mandibulo facial dysostosis in Current ocular
therapy. Ed. 5. Edited by Fraunfelder F.T., Roy H.F. p-185188, W.B. Saunders Com-
15. Christiansen S.P. ; Robin Sequence : in Current ocular therapy. Ed. 5. Edited by
Fraunfelder F.T., Roy H.F. p-192193, W.B. Saunders Company, Philadelphia, 2000.
16. Fernando H., Murillo Lopez ; Oculo auriculo vertebral dysplasia in Current ocular
therapy. Ed. 5. p-188189, Edited by Fraunfelder F.T., Roy H.F. WB Saunders Com-
16A. Sargent R.A. and Ousterhout D.K. ; Ocular manifestation of skeletal diseases in Pediatric
Ophthalmology. Vol. II, Second edition. p-10411042, Edited by Harley RD. W.B.
Saunders Company, Philadelphia, 1983.
17. Khurana A.K., Ahluwalia B.K. ; Investigations of orbital space occupying lesion in
Modern Ophthalmology. Ed. Dutta L.C. p-89102, 1
st
edition. Jay Pee Brothers, New
Delhi, 1994.
18. Arthur S. Grove Jr. ; Orbital disorders in Manual of ocular diagnosis and therapy. 4
th
edition. Edited by Deborah Pavan Langston. p-5765, Little Brown
19. OConnor P.S. ; Ancillary clinical procedures in Neurophthalmology. Review Manual.
3
rd
edition. p-178, Edited by Bajandas F.T. and Kline L.B. Jay Pee Brothers, New Delhi,
1989.
20. Kanski J.J. ; The orbit in Clinical ophthalmology. Second ed. p-25, Butter Worth, Lon-
don, 1989.
21. Dutta H., Mandal D. and Chakravarty A. ; Clinical Methods in Ophthalmology. 1
st
22. Harrison A.R. ; Orbital hypertelorism in Current ocular therapy. 5
th
edition. Edited by
Fraunfelder F.T. and Roy F.H. p-189190, W.B. Saunders Company, Philadelphia, 2000.
23. Grawler J., Saunders M.D., Bull J.W.D., du Bouley G., Marshall J. ; Computer assisted
tomography in Orbital disease. BJO 58:571, 1974.
24. Rao VRV and Ravimandalam K. ; Computerised tomography in Neurophthalmology
a manual for post graduates. Edited by Natchair G. P. 25.01 to 25.19. Arvind Eye Hos-
pital, Madurai.
25. Buerger D.G. ; Proptosis in Ophthalmology secrets. p-235, Edited by Vander J.F. and
Gaulet J.A. Jay Pee Brothers, New Delhi, 1998.
26. Dutta H., Mandal D. and Chakarvarty A. ; Clinical methods in Ophthalmology.
27. Deborah Pavan Langston : Ocular examination and diagnostic tests in Manual of ocular
diagnosis and therapy. p-27, Edited by Deborah Pavan Langston, Little Brown.
28. DePotter P. ; Orbital imaging in Ophthalmology secrets. p-232, Edited by Vander J.F.
and Gault J.A. Jay Pee Brothers, New Delhi, 1998.
29. Haywood M. and Lina M. Marouf ; Eye trauma in Decision making in Ophthalmology.
First Indian edition. p-58, Edited by Heuven WAJ and Zwaan J.T., Jay Pee Brothers,
New Delhi, 1998.
30. Diamond G.J., Ossoinig KC, Fisher Y.L. : Ultrasonography in Principles and practice of
Ophthalmology. Ed. Peyman G.M., Sanders D.R. and Goldberg M.F. p-14021487, Jay
31. Cynthia J. Kendall ; Ophthalmic echography. First Indian edition. p-19, Jay Pee Broth-
32. Bergin D.J., Wright J.E. ; Orbital cellulitis. BJO 70:174-178, 1986.
32A. Leyden JJ ; Ocular changes in skin disorders in Pediatric ophthalmology. Vol. II, Ed. 2,
33. Eustis H.S., Armstrong D.C., Buncic J.R. ; Staging of orbital cellulitis in children.
J.Paed.Oph. and Strab. 23:246-251, 1986.
34. Spire J.R., Smith R.J.H. ; Bacterial infection of the orbital and periorbital soft tissue in
children. Laryngoscope. 96:763-767, 1986.
35. Boger W.P. and Peterson R.A. ; Pediatric Ophthalmology in Manual of ocular diagnosis
and therapy. 3
rd
edition. p-260, Edited by Deborah Pavan Langston, Little Brown.
36. Dhanda R.P. and Kalevar V. ; A Text Book of Clinical Ophthalmology. p-588589, Galgotia
Publication Pvt. Ltd., New Delhi, 1993.
37. Agarwal L.P. ; Essentials of Ophthalmology. p-116120, 1
st
edition. CBS Publishers and
Distributors, New Delhi, 2000.
38. Maus M. ; Orbital inflammations in Ophthalmology secrets. p-244247, Edited by Vander
J.F. and Gault J.A. First Indian edition. Published by Jay Pee Brothers, New Delhi,
1998.
39. Ahemed E. ; A Text Book of Ophthalmology. Ist edition, p-129, Oxford University Press,
Calcutta, 1993.
40. Kanski J.J. ; The orbit in Clinical ophthalmology. Second edition, p-3640, Butterworth,
London, 1989.
41. Catherine E. Durboraw, Stasior G.O., Krohel G.B. ; Orbital cellulitis and abscess in
Current ocular therapy. Ed. 5, p-577579, Edited by Fraunfelder F.T., Roy F.H. WB
Saunders Company, 2000.
42. Gitternger J.W. ; Orbit in Manual of clinical problems in ophthalmology. Edited by
Gittinger J.W. and Asdourian G.K. First edition, p-1740, Little Brown and Company,
Boston, 1988.
43. Duke Elder S. ; System of ophthalmology. Vol. II, p-474478, Henry Kimpton, London,
1960.
44. Melen Onur and Klein J.W. ; Ophthalmic manifestation of vascular diseases of the
brain in Principle and practice of ophthalmology. Vol. III, p-2064, Edited by Pyeman
G.A., Sander D.R. and Goldberg M.F. First Indian edition. Jay Pee Brothers, New
Delhi, 1987.
45. Miller S.J.H. : Parsons diseases of the eye. Seventeenth edition, p-332335, Churchill
Livingstone, London, 1984.
46. Glasser J.S. ; Neurophthalmology. p-258, Harper and Row, London, 1982.
47. Butten C.L., Young B.R. : Chronic orbital myositis. Arch. Oph. 100 : 1749-1751, 1982.
48. Ratnakar K.S. ; Pathology of orbital space occupying lesion in Pathology of the eye and
orbit. First edition, p-204205, Jay Pee Brothers, New Delhi, 1997.
49. Scheie, H.G. and Albert D.M. : Ophthalmic radiology in Text Book of Ophthalmology. p-
252253, 9
th
edition, W.B. Saunders Company, philadelphia, 1977.
50. Scheie H.G. and Albert D.M. : Medical ophthalmology in Text book of ophthalmology. p-
454455, 9
th
edition, WB Saunders Company, Philadelphia, 1977.
51. Carol L. Shields and Sheilds J.A. ; Orbital rhabdomyosarcoma in Current ocular therapy.
Edition fifth, p-256258, Edited by Fraunfelder F.T. and Roy F.H. WB Saunders Com-
pany, philadelphia, 2000.
52. Levin R.A. ; Orbital tumors in Principle and practice of ophthalmology. Vol. III,
p-21612165, First Indian edition, edited by Peyman G.A., Sander D.R. and Goldberg
MF. Jay Pee Brothers, New Delhi, 1987.
53. Kanski J.J. ; The orbit in Clinical ophthalmology. Second edition, p-41, Butterworth,
London, 1989.
54. Levin R.A. ; Orbital tumors in Principle and practice of ophthalmology. Vol. III,
p-215657, First Indian edition, edited by Pyeman G.A., Sander D.R. and Goldberg
M.F. Jay Pee Brothers, New Delhi, 1987.
55. Fraunfelder F.T. ; Ewings sarcoma in Current ocular therapy. Edition fifth, Edited by
Fraunfelder F.T. and Roy F.H. p-235236, WB Saunders Company, Philadelphia, 2000.
56. Jakobicc F.A. Root man J., Jones I.S. ; Secondary and metastatic tumors of orbit in
Clinical opthalmology. Vol. II, Edited by Duane T.D., p-167, Harper and Row, 1982.
57. Khurana A.K., Ahluwalia B.K. and Gupta S. ; Bilateral proptosis due to metastatic
Ewings sarcoma. Ind.Jr.Oph. 40 : 15-17, 1992.
58. Karp, L.A., Zimmerman L.E., Payne T. ; Intra ocular involvement in Burkitts lymphoma.
Arch. Oph. 85 : 295, 1971.
59. Feman S.S., Niwayama G., Hapler R.S., Foos R.Y. ; Burkitts tumor with intra ocular
involvement. Surv. Oph. 14 : 106, 1969.
60. Vyas P. and Chandrashekhar G. ; Orbital lymphoma and allied conditions in Modern
ophthalmology. Edited by Dutta L.C. First edition, p-124, Jay Pee Brothers, New Delhi,
1994.
61. Natrajan M. ; Tumors of optic nerve in Neurophthalmology. First edition, p-14.114.14,
Arvind eye hospital, maduri.
62. Glaser J.S. : Neurophthalmology. p-116117, Harper and Row, 1982.
63. Boger, P.W., Peterson R.A. ; Paediatric ophthalmology in Manual of ocular diagnosis
and therapy. Edited by Deborah Pavan Langston, p-283284, Little Brown.
64. Melen O. ; Ophthalmic manifestation of brain tumors in Principle and practice of oph-
thalmology. Edited by Peyman G.A., Sander D.R., Goldberg M.F. Ist Indian edition, p-
65. Scheie H.G. and Albert D.M. ; Ophthalmic radiology in Text book of ophthalmology. 9
th
edition, p-147148, WB Saunders Company, Philadelphia, 2000.
66. Apple D.J., Pandey S.R., Gomez M.E. ; Langerhans cell histocytosis in Current ocular
therapy. 5
th
edition, p-289290. Edited by Fraunfelder F.T. and Roy F.H. WB Saunders
67. Rubin P.A.D. ; Orbital disorders in Manual of ocular diagnosis and therapy. 5
th
edition,
p-5666, Edited by Deborah Pavan Langston, Lippincot Williams and Wilkins,
philadelphia, 2002.
Disorders of the Sclera
The Sclera is the largest part of the outer coat of the eye, also the largest visible part of the eye
and forms the white of the eye. It constitutes the posterior five-sixth of the outer coat of the
eyeball. It extends from the limbus anteriorly to optic nerve posteriorly. It is opaque and
white. It is tough. All the vessels that supply the uvea pass through the sclera without giving
any specific branches to it. It gets some blood supply at the limbus. The sclera gets its nutrition
from the episcleral vessels on the outer and choroid on the inner side. Absence of vessels in the
substance of the sclera makes sclera less favourable site for infection, degeneration and new
growths. Number of diseases that inflict sclera in all ages is not very long. Disorders of
sclera in children is relatively rare except changes in colour, dehiscence and trauma.
The sclera forms a hollow sphere of 22 mm diameter when fully developed. It has two
large openings and three sets of smaller apertures in its coat. The anterior opening is called
anterior scleral foramen it corresponds to the limbus and is 11mm in diameter while the
posterior scleral foramen is the circular opening through which the optic nerve passes. The
posterior scleral opening is bridged by lamina cribrosa. The opening for the optic nerve is
situated 2 mm nasal to the posterior pole of the globe.
The other apertures:
1. For passage of long and short posterior ciliary arteries and ciliary nerve.
2. For exist of venaeverticosae. They are four in numbers. One in each superior and
inferior temporal or nasal quadrant, just behind the equator at 1, 5, 7 and 11 oclock.
Sometimes there may be more than four vertex veins. The distance of vertex vein
from limbus is not constant. The venaverticosae have an oblique passage through the
sclera.
3. The anterior apertures near the limbus are for passage of anterior ciliary vessels.
Thickness of the sclera is not uniform through out. It is thickest round the optic nerve
i.e. 1.00 mm. It is about 0.75 mm at limbus and thinnest at the attachment of the four recti i.e.
0.3 mm.
The sclera is covered intimately by the Tenons capsule. The capsule is attached to the
sclera 1 mm to 2 mm away from the limbus and covers it snugly up to optic nerve where it
blends with the covering of the optic nerve. There are some fine trabeculae, which keep the
Tenons capsule anchored to the sclera. The space between the sclera and the Tenons capsule
CHAPTER 21
Disorders of the 8clera in Children
720
DISORDERS OF THE SCLERA IN CHILDREN 721
is a potential space without much clinical importance except that antibiotics and steroids can
be injected in this space for better penetration. The Tenons capsule acts as synovial-mem-
brane for the sclera.
The visible part of the sclera is covered by bulbar conjunctiva, which can also be lifted
off the sclera by fluid injected in the sub conjunctival space.
In normal eyes the vessels seen over the sclera are conjunctival in nature and can be
moved with conjunctiva. There are only a few visible episcleral vessels in normal eye, in chroni-
cally inflamed eyes and absolute glaucoma, prominent episcleral vessels can be visible.
Attachment of the extra ocular muscles
All extra ocular muscles are inserted in the sclera. The four recti are attached in front of the
equator and the two obliques behind the equator. The distance of attachment of the recti are
not equal from the limbus. The medial rectus is attached 5.5 mm away from the limbus. The
inferior rectus is attached 6.5 mm away from limbus and the lateral and superior rectus 7.00 mm
and 8.1 mm respectively. The tendons of the extra ocular muscle get mingled with the sclera
very intimately. The inferior oblique has the shortest tendon i.e. 1 mm. Attachments of recti
are on the thinnest part of the sclera. Care should be taken not to nick the sclera during
detaching these muscles or passing sutures during strabismus surgery.
Weak spots in the sclera
The sclera is not of uniform thickness, it is precariously thin at the equator. The sclera has
multiple apertures through which vessels and nerves pass. These are also potentially weak
spots. Besides these anatomical weak spots the sclera may be thinned at other place following
injury and inflammation.
Thus the weak spots on the sclera are - Corneo scleral junction, equator, over the
ciliary body and posterior pole.
Colour of the sclera
The colour of the adult sclera is white. At birth it has a bluish tinge due to choroid shinning
under thin sclera. The sclera is thin in childhood, by five to seven years it becomes as white
and as thick as adult sclera.
Histologically sclera is divided into:
1
1. Episclera
2. Scleraproper
3. Lamina fusca.
The episclera is an ill defined layer of connective tissue that lies between the Tenons
capsule and the sclera. It is loosely attached to both. It is thin at the equator and under the
bulbar conjunction and thick round the optic nerve. It is less vascular than conjunctiva but
more vascular than sclera proper, is more likely to be inflamed than sclera.
The sclera proper consist of large bundles of collagen, packed densely. The superficial
fibers are larger than the deeper. The space in between the fibers is filled by
mucopolysaccharides. The fibers round the limbus and optic nerve are concentric with these
structures, the fibers in between are arranged irregularly.
Lamina FuscaIt is a thin ill defined layer that contains melanocyte on the inner side
of the sclera.
Functions of the sclera
The main function of the sclera is to protect intra ocular structures, to maintain the shape
of the globe and to keep the retina in optically correct position. For sharp focus it is essential
that the interior of the eye remains in darkness, which is brought about by small mobile pupil,
pigment in uvea and opaque sclera.
The opacity of the sclera is due to irregular arrangement of its large fibres and low
water content.
Development of sclera
2
Developmentally sclera is mesodermal. It develops from condensation of mesodermal tissue
round the optic cup. The anterior part develops early, to which the developing recti are attached.
The limbus is placed well behind its normal position initially, the limbus gradually moves
anteriorly. By 12 weeks the posterior sclera develops round the optic nerve and a well formed
posterior pole becomes obvious. The lamina also develops at this time. The scleral spur develops
by 12th week. The Tenons capsule develops along with the eyeball. Surprisingly the shape
and configuration of the eyeball are least influenced by sclera. These are regulated by developing
retina
3
. (See page 3)
Congenital anomalies of the sclera (See page 8-9)
The congenital anomalies of the sclera are few and are mostly due to defect in:
1. Development of optic cup and its further progress
2. Failure of development of mesodermal tissue round the optic vesicle resulting into
malformation of globe with
(a) Intact scleraMicrophthalmos
(b) With scleral defect i.e. microphthalmos with cyst and cystic eyeball.
The other congenital anomalies of sclera are:
1. Blue sclera
2. Inter scleral nerve loop
3. Nanophthalmos
4. Sclerocornea
5. Congenital staphyloma
Blue sclera
Sclera of the new borne is more translucent than adult. The sclera of a new borne is thinner
than adult sclera and number of scleral fibres are also less. With age both increases and the
sclera becomes white. The colour of a blue sclera is in fact a blue tinge due to the uvea shining
through. In some cases, the sclera fails to attain normal white colour and remains blue, with-
out any other ocular anomaly and does not predispose any disease.
Sometimes blue sclera is associated with systemic anomalies. Commonest combination
is blue sclera with brittle bone in osteogenesis imperfecta with deafness.
DISORDERS OF THE SCLERA IN CHILDREN 723
The other condition associated with blue sclera is Ehlers-Danlos syndrome. This is
generally an autosomal dominant disorder; occasionally it can be autosomal recessive as well.
Most striking feature of the condition is hyper elasticity of the skin due to loss of collagen
tissue in the dermis. The skin of the upper lid can be pulled as far as the check. The ligaments
of the joints are also lax leading to hyper extensibility of joints with frequent subluxation of
joints. The other possible ocular components are blue sclera, keratoconus, micro cornea,
subluxation of lens, angioid streak, may have choroidal haemorrhage.
Inter scleral nerve loop
3
In this case, a branch of long ciliary nerve changes its course to enter the substance of the
sclera and emerges on the scleral surface under the conjunctiva as a tender nodule. It is generally
appears on the nasal side, but has been reported from other sites also. It is generally a grey
nodule but sometimes, it is surrounded by choroidal pigments. The cause of the anomaly is not
known. It does not require any treatment.
Nanophthalmos
This is generally sporadic in nature, does not follow any fixed hereditary pattern. It can be
unilateral or bilateral. The development of the globe is arrested in all dimension after the
foetal fissure has fused leading to a small globe even smaller than a globe of a new born and
remain small throughout the life. There are no other anomalies in the globe. The lens continues
to grow as in other eyes. The result of small globe manifests as narrow inter palpebral aperture
and deep set eyes. The distant vision is poor, occasionally with poor near vision in childhood,
brought about by axial hypermetropia. The hypermetropia is always more than + 10 D. The
child may have nystagmus; squint is common. Other cause of diminished vision is hypoplasia
of macula. The eyes have tendency to develop simple glaucoma. Commonest complication of
the condition is uveal effusion syndrome
6
following penetrating injury accidental or surgical.
Sclerocornea
This is a combined anomaly of cornea and sclera, generally discussed under congenital anomaly
of cornea along with corneaplana. The cornea and sclera blend into each other without trace of
limbus. The condition may be localised on the peripheral cornea or may extend well into the
centre of cornea.
Congenital staphyloma
Congenital staphyloma is congenital dehiscenes of sclera with bulging of the scleral coat that
is lined either by ciliary body or choroid. The congenital scleral staphylomas are equatorial
and posterior. Ciliary staphylomas are rarely congenital.
About 6% to 14% of eyes have staphylomas
5
without symptoms and are discovered dur-
ing strabismus or retinal surgery.
Posterior staphylomas develop in eyes with myopia more than 8 D. All high myopic
eyes do not develop posterior staphyloma. Posterior staphylomas develop in the posterior pole.
It may involve the optic nerve head also. These eyes are generally large eyes with pseudo
proptosis. The distant vision is poor and not corrected fully by glasses. On retinoscopy the
staphyloma shows more myopia than rest of the globe. Direct ophthalmoscope requires more
minus lenses to focus the bottom of the staphyloma. The retinal vessels seem to disappear in
the wall of the excavated staphyloma.
A posterior staphyloma is best visualised with indirect ophthalmoscope.
In presence of hazy media, presence of posterior staphyloma is confirmed by B scan
ultrasonography. It is also well delineated by C.T. scan.
Other cause of scleral staphyloma in children is buphthalmos
7
, which produces ciliary
as well as equatorial staphylomas. Ciliary staphylomas are more common. Raised intra
ocular tension does not produce posterior staphyloma.
Intercalary staphylomas are least common in children. They are seen following badly
repaired wounds, accidental or surgical at the limbus where the root of the iris in incarcerated
in the dehiscenced wound.
Scleral staphyloma are occasionally seen in older children in cases of oculo sporidiosis
in endemic areas
8
.
Infection and inflamation of the sclera do not develop in children. However inflamation
of episclera i.e. episcleritis may occur in older children suffering from collagen disease. It is
generally confused with phlycten of the conjunctiva, which is very common in children.
REFERENCE8
1. Nema H.V., Singh V.P. and Nema N. ; The sclera in Anatomy of the eye and its
adnexa. Second edition, p-1012, Jay Pee Brothers, New Delhi 1991.
2. Nema H.V., Singh V.P. and Nema N. ; Development of the eye and its adnexa in Anatomy
of the eye and its adnexa. Second edition, p-129, Jay Pee brothers, New Delhi 1991.
3. Duke Elder S., Cook C. ; System of ophthalmology. Vol. III, Part II, p-162167, Henry
Kimpton, London 1963.
4. Duke Elder S. Wybar K.C. ; The nerves of the eye in system of ophthalmology. Vol I,
p-385, Henry Kimpton, London 1961.
5. Faris. B.M. and Freeman H.M. ; Scleral staphytoma and dehiscences in Current ocu-
lar therapy. Fifth edition, p-336337, Edited by Fraunfelder F.T. and Roy. F.H., W.B.
6. Dutta L.C. ; Uveal effusion syndrome in Ophthalmology Principle and practice.
First edition, p-122123, Current book international, Calcutta, 1995.
7. Duke Elders S. Leigh A.G. ; Disease of the sclera in system of ophthalmology. Vol. VIII,
Part 2, p-9981003, Henry Kimpton, London 1965.
8. Mukherjee P.K. ; Rhino spordiosis in Current ocular therapy. Fifth edition, p-6667,
Edited by Fraunfelder F.T. and Roy F.H., W.B. Saunders Company, Philadelphia 2000.
CHAPTER 22
Ocular Manifestation of 8ystemic
nfection in Children
725
The eyes are involved in many disorders of the body that may be present at birth as
Congenital disorders:
(i) Anatomical or functional disorder
(ii) Infection
(iii) Errors of metabolism
(iv) Central nervous system disorders.
Or be Acquired later, as infectious, allergic, auto immune, metabolic, degenerative or
dystrophic disorder. It is common for malignancy of the eye to metastasise to distant organs,
the reverse though rare is also possible.
Ocular manifestation of infectious disease of the eyes:
Infectious disease can be caused by:
1. Bacteria
(i) Bacilli (ii) Cocci
2. Viruses
3. Fungi
4. Protozoa
5. Helminths
6. Myiasis (larva)
7. Arthopods
Ocular manifestation of systemic bacterial disease
1,2,3,4
(The following are short descriptions of systemic manifestation of infections that outline
important systemic signs and symptoms, followed by ocular manifestation.)
Bacterial ocular infection can either be acute or chronic. Acute infection is generally
due to spread from neighbouring structure, or hematological spread. Acute systemic bacterial
infection may release bacterial toxin as well.
Chronic bacterial diseases are generally associated with granulomatous ocular disease
or can cause allergic reaction to bacterial protein.
Various systemic bacterial diseases that have ocular involvement are arranged in
alphabetical order. All of which are not equally common. Some may have minimal ocular ocular
morbidity. Others may have widespread ocular involvement. Most of them respond to specific
antibiotics.
Acinetobacter (Herellea Vaginicola)
This is an opportunistic organism, present widely in water and soil. The organism belongs to
family Neisseriaceae. It is an aerobic, gram-negative cocobacilli, infects children with
compromised immunity.
Systemic involvement
Multi systemic infectionPneumonia, meningitis, urinary tract infection, and septicemia.
Ocular involvement is less commonThey include: Blepharitis, purulent conjunc-
tivitis, superficial punctate keratitis, sloughing corneal ulcer, and endophthalmitis, mostly
following traumaaccidental or surgical.
Management consists of correct diagnosis by culture. It is sensitive to many antibiotics,
both local as well as systemic, common antibiotics used areTrimethoprimesulpha methoxazole,
aminoglycocides, fluroquinolones.
Actino mycosis
This was previously thought to be a fungus infection, now it is classified as mycobacterium.
This is an anaerobic organism. The organism is of low virulence with chronic course, does
not cause impairment of vision.
Systemic involvementCutaneous especially skin of face and neck, teeth and gums.
Ocular involvement is generally unilateral, canaliculitis of lower lid leading to
epiphora, swelling over the canaliculi. On pressing the inflamed canaliculus, there is
regurgitation of yellow creamy material, which on microscopy contains concretion, like sulphur
granules. Rarely there may be chronic dacryoadenitis.
Management consists of systemic tetracycline, cephalosporin or penicillin. Local
antibiotics are tetracycline ointment, or aminoglycocide drops frequently.
Bacillus cereus
The organism B. cereus belongs to genus bacillus that also contains bacillus anthracis or
anthrax bacillus, which has acquired notoriety as a tool of biological warfare with lethal effect.
Bacillus cereus is commonly found in water, soil, air and dust. It is highly virulent, generally
resistant to penicillin and cephalosporins. It is aerobic, gram-positive, spore forming.
Systemic involvement is relatively rare, seen in drug addicts who use infected needles.
It reaches the eye as hematogenous spread due to contaminated intravenous injection or more
commonly due to penetrating injury, mostly accidental rarely surgical.
Ocular involvement is fast developing endophthalmitis often leading to panophthal-
mitis and loss of the eye.
OCULAR MANIFESTATION OF SYSTEMATIC INFECTION IN CHILDREN 727
Commonest cause of post operative endophthalmitis is due to failure to destroy B Cereus
by usual autoclaving because of its spore bearing character.
The endophthalmitis develops within twenty-four hours, may have fever and raised
WBC Count. The severity is directly proportionate to the capacity of the organism to produce
exotoxin.
The outcome of the condition is devastating visual loss.
Management is an ocular emergency, fast developing endophthalmitis with rapid fall
of vision, fever, leucocytosis points toward possibility of B Cereus infection that is conformed
by AC or vitreous tap, and positive culture of organism.
IV ciprofloxacilin 400 mg BD has been reported to give good result.
Intra vitreous injection of vancomycin, gentamycin and clindamycin give better result
than intra venous antibiotics. Early vitrectomy may salvage some vision.
Brucellosis
Brucellosis is mostly a zoonosis i.e. a disease that spreads from animal to man. The disease
spreads following consumption of unpasteurised milk and milk products or direct contact
with infected animals.
The organism is a gram negative, intra cellular, non-motile, aerobic organism.
There are four types of organisms that may infect man. The animals transmitting the disease
may be any of the following: dogs, cattle, horses, sheep, goats and pigs.
Intermittent fever, headache, chills, joint pain, weight loss, speenogegaly and generalised
lymphadenopathy. Other organs can also be involved.
Ocular involvement
In milder form it can cause swelling of lid, conjunctivitis, nummular keratitis. In sever form it
causes sever anterior uveites, retinal haemorrhage, retinal edema, endophthalmitis, optic neu-
ritis, extra ocular muscle palsy.
Management
The organism is sensitive to many antibiotics i.e. tetracycline, doxycycline, trimethoprin-
sulphamethoxazole, streptomycin and rifampin. It requires four to eight weeks continuous
medication to eradicate the organism.
Clostridium perfringes
This is commonly known as gas gangrene organism that has dangerous systemic involve-
ment, mostly involving large muscles.
The organism is opportunistic, gram positive bacillus. It is an anaerobic gas form-
ing organism.
The systemic and ocular diseases are of acute onset, starting within twenty four hours
of onset of infection. It can produceIntestinal disorder, suppurative deep tissue infection,
skin and soft tissue infection.
Ocular involvement includes sever uveitis with coffee coloured hypopyon,
endophthalmitis, panophthalmitis, orbital cellulitis. Edema of the lids, chemosis of conjunc-
tiva, sever ocular pain, fever, loss of vision, acute rise of intra ocular pressure are the
hallmarks of the disease.
Management
In all cases of gas gangrene, initial treatment should be directed towards systemic manifesta-
tion of shock, haemolytic anaemia, fever by IV injection of gas gangrene antitoxin 50,000
units every six hourly for first two days. Hyperbaric oxygen when available may be useful.
Antibiotics to which the organism is sensitive arePenicillin G. sodium, cefazoline,
chloramphenical, amoxicillin, cefotoxamime.
Management of ocular involvement is more difficult. Hyperbaric oxygen therapy seems
to have some beneficial effect. Local antibiotic drops do not have any therapeutic effect. Sub
conjunctival injection is better alternative. Intra vitreous route is the best mode of administra-
tion of antibiotic. Early vitrectomy removes highly toxic necrotic material, improving visual
prognosis.
Clostridium tetani
Tetanus is a severe neuromuscular disease, it is one of the top ten causes of death in children
in under developed countries where there is lack of universal immunisation. It can infect a
person at any age. Clostridium tetani is found as spores in soil, dust, animal and human
faeces.
Symptomless humans can carry tetanus spores in gastro intestinal tract in about ten
percent of population in non-immunised community.
The organism is gram positive, anaerobic, motile spore wearing organism. The
spore is terminal and colourless. The organism produces a neurotoxin called tetanospasmin,
which causes muscle spasm.
The disease is caused following injury on any part of the body with introduction of
organism. The incubation period varies between two hours to several months. Short incubation
period is associated with more severe infection. Distance of the injury from the central nervous
system is important, nearer the wound to CNS more serious is the condition.
Clinically there are four forms of tetanusNeonatal, cephalic, generalised and
localised.
Systemic involvement is wide spread neuromuscular involvement.
Typical findings areLock jaw (trismus), risus-sardonicus, restlessness, irritabil-
ity, stiffness, cramps, dysphagia and arched back.
Ocular involvementOcular involvements are due to neuromuscular involvements
and their sequel. They areBilateral blepharo spasm, paralysis of all the cranial nerves from
third to twelfth resulting into ptosis, external ophthalmoplegia, internal ophthalmoplegia,
total ophthalmoplegia, facial palsy, supra nuclear palsy, corneal exposure, conjunctival con-
gestion, corneal ulcer, conjunctivitis.
Management
5
Tetanus is one of the most fatal diseases, which is fully preventable following proper immuni-
sation.
All expectant mothers should get two shots of tetanus toxoid irrespective of immunisa-
tion status. All children should get usual DPT as per national programme of universal immu-
nisation. The child must get a booster dose by seventh year and repeated every ten years or
following tetanus prone injuries that could be systemic or ocular. The systemic tetanus
prone injuries are unattended wound of more than six hours, with depth more than 1 cm, all
road traffic accidents, sports injuries, burns where the wound is contaminated by dirt.
Tetanus prone ocular injuries are - Corneal and scleral perforation, lacerated wounds
of lids, peri orbital structures.
Non immunised children with tetanus prone injury should get injection of human teta-
nus immunoglobulin.
Treatment of actual disease is mostly symptomatic and supportive:
1. The child should be admitted in specialised wards the child is put on life supportive
measure with sedation under supervision of expert trained person dealing with such
emergencies.
2. Antibiotics are given to prevent secondary infection. Common antibiotics are
Penicillin G, tetracycline, erythromycin, metronidazole. The child should get an extra
dose of tetanus toxoid and human tetanus immunoglobulin.
Clostridium botulinum
Botulism is a paralytic disorder that begins with cranial nerves and spreads downwards, is a
fatal disease. Main types of botulism are - Infantile botulism, wound related botulism
and food related botulism.
C. Botulinum is gram positive that forms sub terminal spore, anaerobic in nature,
found in soil and seawater. It produces most potent bacterial toxin. Incubation period is
short i.e. 18 to 36 hrs in food related botulism and may be as long as 10 days in wound related
botulism.
Clinical picture in both is almost same. The systemic involvement includes descending
paralysis beginning with ptosis, going through diplopia, dysphagia, dysarthria, may involve
neck and thorax. Other symptoms are that of food poisoning i.e. nausea, vomiting, abdominal
pain, paralytic ileus, urinary retention.
Ocular involvement consists of bilateral ptosis, diplopia, fixed dilated pupil, and
cycloplegia.
Management
The child should be hospitalised in food related botulism. Trivalent antitoxin is administered,
intestinal antibiotics are given to reduce the organism present in the gut.
Corynebacterium diphtheriae
This is an acute, life threatening disease with extensive ocular involvement. Healthy asympto-
matic carriers may be responsible for spread of infection in non-immunised community. The
disease is fully preventable except in a few individuals who may be infected by the disease in
spite of proper immunisation. The effect of disease in such cases in mild and nonfatal.
The organism is a surface saprophyte affecting naso pharyngeal area. It is gram
positive, club shaped, non motile, pleomorphic organism. It produces a strong toxin
that affects heart, central nervous system, cranial nerves, and eyes. Respiratory obstruction is
caused due to accumulation of necrotic exudate consisting of leucocytes, RBC and respiratory
exudates.
Systemic involvement comprises of fever, chills, cutaneous diptheria, myocarditis,
and polyneuritis. Respiratory tract involvement includes sore throat, rhinorrhea, hoarseness,
dysphagia, cough, and pneumonia.
Ocular involvements are mostly extra ocular. The commonest involvement is
conjunctival that start as congestion proceeding tocatarrhal conjunctivitis, purulent
conjunctivitis, membranous and pseudo membranous conjunctivitis. There may be small sub
conjunctival haemorrhage, as the condition proceeds towards membrane formation the lids
become swollen and hard due to development of tarsitis and meibomianitis.
Corneal involvementSuperficial keratitis, frank corneal ulcer, sloughing corneal ul-
cer and perforation.
Cranial nerve involvement includesPtosis, paralysis of extra ocular muscles,
paralysis of accommodation without mydriasis, internal ophthalmoplegia, convergence and
divergence paralysis. Optic neuritis is rare. Late complications of conjunctival and corneal
involvement lead to trichiasis, entropion, symblepharon, and xerophthalmia.
Management
The most effective management begins with immunisation of all children against diphtheria
along with tetanus and whooping cough. The immunisation needs to be repeated after 3 to 5
years.
Once the disease has been diagnosed, the management consists of neutralising the
circulating toxin and reducing the bulk of bacteria by antibiotic, along with life supportive
measures.
Ocular involvement is treated by instillation of anti toxin, local instillation of
penicillin G., erythromycin, mydriatic and cycloplegic.
Escherichia Coli
This is a commensal found in the gastro intestinal tract. Other common portal of entry is
urinary tract. The organism is gram negative rod.
Systemic involvement cause diarrhoea, dysentery, haemorrhagic colitis, urinary tract
infection. It can cause fatal haemolytic- uremic syndrome in children.
Ocular involvements are relatively less frequent. It may be as mild as mucopurulent
conjunctivitis to as serious as metastatic endophthalmitis. It can also cause pseudo membra-
nous conjunctivitis, keratitis, corneal ulcer, uveitis, gas in AC, hyphema, hypopyon, and
endophthalmitis.
Management consists of systemic antibiotic. Common antibiotics used areAmpicillin,
cephalosporin, nitrofurantoin, trimethoprinsulfamethoxazole, gentamicin, kanamycin,
amikacin.
Ocular management is management of conjunctivitis, corneal ulcer, uveitis and
endophthalmitis by standard methods along with management of systemic condition.
Hemophilus influenza and Koch-Weeks bacillus
Both the organisms belong to the group of hemophilus, have identical staining pattern, clini-
cal presentation and are sensitive to same type of antibiotics. The Koch-Weeks bacillus is also
known as H. aegyptius. There are many more strains of hemophilus. Both are pleomorphic,
gram-negative cocco-bacilli.
Hemophilus is a common bacterial infections in children that cause upper respiratory
tract infection, have seasonal variations, seen in small epidemics, may present as pink eyes
followed by respiratory infection or vice versa.
Systemic involvement consist ofFever, malaise, upper respiratory tract infection,
sinusitis, bronchitis. In sever form it causes pneumonia, Otitis media is less common. Severe
infection may cause meningitis and bacteremia, septic arthritis.
Ocular involvements are common. They are - Rapidly developing mucopurulent
conjunctivitis, pseudo membrane formation, edema of the lids with violet colour, keratitis,
central ulcer is rare. Conjunctivitis resolves in eight to ten days that may leave conjunctival
scar in the tarsal conjunctiva. Phlycten are common after clearing of acute conjunctivitis.
Ocular management is instillation of broad spectrum antibiotics like ciprofloxacilin drops
frequently with same ointment at bed time along with management of corneal ulcer when
present.
Mycobacterium tuberculosis
Infection by this bacillus is one of the oldest known chronic disease world-wide. It still is a
major cause of death in developing countries. It can infect any organ in the body in all ages in
both sexes. It is not a hereditary disease but it is common to see more than one person infected
by tuberculosis in the same family. Broadly it is divided in to two groups i.e. pulmonary
tuberculosis and extra pulmonary tuberculosis.
Mycobacterium tuberculosis is a rod shaped, non spore forming, slender, aerobic
bacterium. It does not stain with gram stain. It is an acid-fast organism.
It spreads by droplet from the infected person who is positive for tuberculosis organism.
Systemic involvement - Commonest organs to be infected are the respiratory organs.
Common infections are seen in lungs and pleura. It can infect the small intestine, urinary
tract, meninges. It can form intra cranial tuberculoma. Bones and joints are also involved.
The disease is characterised by tissue destruction and replacement by granulomatous
tissue forming typical tubercles, which has central caseation. The severity of the lesion depends
on many factors i.e. organ involved, tissue hypersensitivity, immunity and overall resistance
power of the individual. Inflammatory process and tissue destruction is mostly due to tissue
hypersensitivity while healing is immunity dependent.
Ocular involvement
Primary ocular involvement is rare and mostly seen in the lids as cutaneous tuberculosis
or tubercular conjunctivitis, which may produce a cocks comb like growth in the conjunctiva.
Incidence of tuberculoma of the conjunctiva has become very rare.
It is rare for a case of frank pulmonary tuberculosis to develop tuberculosis of
the eye. The common conjunctival lesion is phlyctenular conjunctivitis. Conjunctival ulcera-
tion is seen with cutaneous tuberculosis involving the lid margin.
Cornea
Commonest involvement is phlyctenular kerato conjunctivitis, followed by fascicular ulcer of
the cornea. Frank keratitis by tubercular bacilli is very rare; more common is interstitial
keratitis. Superficial keratitis is more common in cases of non tubercular myco-bacterial infec-
tion.
In sclera two common involvements are episcleritis and scleritis. A scleritis nodule
near the limbus may cause sclerosing keratitis.
Involvement of orbit is rare but may cause mild proptosis. A tubercular sinus may de-
velop on the skin or conjunctival fornix.
The conjunctival, corneal and scleral involvements are immunity mediated but orbital
involvement is either due to extension from infected sinuses or via blood.
Intraocular involvement
All vascular intra ocular structures may be involved either as hematogenous spread which is
rarer than more common hypersensitivity to the tubercular protein.
It can be present as acute mild iritis to severe posterior uveitis. The uveitis is
granulomatous in nature with vitritis. Tubercular endophthalmitis has not been reported.
The iris may have multiple tubercular nodules in chronic stage. Ciliary body may be in-
volved as part of iridocyclitis or may have large nodule that may be large enough to cause
angle closure glaucoma and confused as a neoplasm which can be differentiated only on B scan
ultrasonography.
Choroidal involvement may be in the form of solitary patch of choroiditis or there
may be multiple patches of choroiditis. The choroid may be involved in miliary tuberculosis.
Retinal involvement is rare. It is involved as patch of chorioretinitis, periphlebitis is
more common than retinitis.
Extra ocular muscle palsies are seen in cases of tubercular meningitis and intracranial
tuberculoma. Sub tentorial tuberculoma are common in children that my lead to papilledema
and post papilledematous optic atrophy. Optic neuritis is less common, however, anti tubercu-
lar drug induced optic neuritis is common which is dose related and resolves following reduc-
tion in dose of the drug.
Management
ProphylaxisBCG injection soon after birth saves the child from serious forms of tubercular
infection like miliary tuberculosis and meningitis.
The best prophylaxis is early detection and complete eradication of infection in the
individual.
7
Preventive chemotherapy is popular in developed countries. Its place in under developed
countries has not been fully evaluated.
All cases of proved tuberculosis should get appropriate anti tubercular chemotherapy
for adequate period.
Ocular tuberculosis is managed by
1. Appropriate systemic anti tubercular treatment for adequate period.
2. Local treatment by cycloplegic and local steroid
3. Posterior uveites, neuro retinitis, periphlebitis require systemic steroid for long time
that should be always given under cover of systemic anti tubercular chemotherapy.
Neisseriae gonorrhoeae
6
or gonococcal infection is a world wide health problem, more
in developing countries than in developed countries. It is an acute multi systemic disease
that can be fatal in new born and infants. It is a cause of bilateral diminished vision in children
who had inadequate treatment. Neisseriae gonorrhoeae are intra cellular, gram negative
diplococci, seen inside the polymorphonuclear lencocytes. The organism is oxidase and
catalase positive. Many strains produce B. lactamase that makes the organism develop
resistance to penicillin, which is still considered to be the first line of treatment of gonococcal
infection. Chromosomal mutation is yet another cause of penicillin resistance.
Humans are the only natural reservoir of the organism. It is a foremost cause of
sexually transmitted disease. Though largest number of new cases start in second and third
decade, no age, sex or race is immune to the disease.
The infection begins as acute inflamation of colummar and cuboidal epithelium.
Systemic involvementIn adults it begins mostly as purulent urethritis in males
and cervicitis in females. The incubation period is 2 to 5 days. However some persons do not
develop acute stage and remain asymptomatic, do not seek treatment and act as reservoir of
infection. While the symptomatic patients especially males always seek medical treatment for
dysuria, meatal inflamation, local swelling, pain, fever, unilateral epidedymitis, inguinal
lymphadenitis.
In females the infection starts as endo cervicitis, leading to yellowish discharge. The
infection may travel up and cause acute endometritis and salpingitis. Urethritis is common.
Other systemic involvements arePolyarthritis, tenosynovitis, pericarditis,
endocarditis, toxic hepatitis and rarely meningitis.
Gonococcal urethrites is a major cause of stricture urethra in males and cause of pelvic
inflammatory disease in females.
Ocular inflamation was a major cause of bilateral blindness before advent of chemo-
therapy. Though the pathogenesis of ocular gonococcus infection is almost the same in all
ages, clinical presentation and management differ in different age groups.
Ocular manifestations in children can be broadly divided into - Neonatal gonococcal
infection and gonococcal infection in pediatric and adolescent.
Neonates develop gonococcal infection in two ways:
1. A less frequent intra uterine infection
2. Common direct infection during passage through an infected birth canal.
The first is possible if mother develops premature rupture of the membranes. This results
in serious septicaemia, pneumonia and meningitis. Ocular involvement is restricted to
chorioretinitis. This type of infection is not preventable by caesarean section. The second is
more common and called as gonococcal ophthalmia neonatorum.
Pediatric and adolescent gonococcal ophthalmia is clinically similar to adult gonococcal
ophthalmia. It is generally associated with child abuse or assault. However with development
of early sexual activity and precociousness, incidence of adolescent ophthalmia is on the rise.
Other ocular involvements are:
Bilateral central corneal ulcer, gonococcus is one of the few organisms that can pass the
intact corneal epithelium causing not only corneal ulcer but also anterior uveitis. Hence all
cases of ophthalmia neonatorum should be treated as gonococcal unless proved otherwise. All
cases of gonococcal conjunctivitis should also get local atropine in appropriate dose.
Perforation of central corneal ulcer may lead to acute endophthalmitis, panophthalmitis
and loss of eye. Smaller perforation generally lead to collapse of AC, bringing the lens in con-
tact with the cornea resulting in formation of anterior polar cataract. The perforation gener-
ally heals and a central leucoma results. This leads to a triad of central corneal opacity,
anterior polar cataract and nystagmus.
Gonococcal infection may lead to mild iritis to sever plastic uveitis.
Management
Management of gonococcal infection in new-born child begins with ante natal examination of
mother and if found to be suffering from genital gonococcal infection, she should be treated
adequately. Caesarean section may save the child from neonatal infection. All children born of
infected mother should also get adequate systemic antibiotic.
All neonates irrespective of their infective status should get prophylaxis for ophthalmia
neonatorum either as single drop of 2% silver nitrate solution in each eye or 2.5% single
drop of povidone iodine in each eye. In absence of above two, any broad spectrum antibiotic
may be used as local drop.
Neisseriae meningitidis
Neisseria meningitidis or meningiococcus is a gram negative diplococcus that perish on
exposure to drying and chilling. It is found only in human beings. The natural habitat of the
organism is the nasopharynx. Meningococcal infection is mostly seen in children world wide.
The infection may be sporadic or there may be localised outbreak, epidemics are no more seen.
It spreads either by inhalation of infected droplets or direct oral contact of infected
person.
Systemic involvement is more common and serious than ocular involvement. The
organism has a short incubation period. The clinical manifestations vary from transient
bacteremia with good health to irreversible shock, hemorrhagic shock and death.
The systemic involvement follows a set pattern of clinical manifestation that begins as
upper respiratory infection followed by bacteremia and meningitis. To begin with there is soar
throat, rhinorrhea, cough, headache, redness of the eye, with fever, meningeal irritation.
Less frequent manifestation are arthritis, pneumonia, sinusitis, otitis media, endocarditis,
pericarditis.
Neurological complications are common they include encephalitis, brain abscess, extra
ocular muscle palsy.
Ocular involvement
Ocular involvements are less frequent and may be overlooked due to more severe systemic
manifestation. Ocular involvement is mostly metastatic, however acute conjunctivitis may
be due to direct droplet infection or spread from nasopharynx. Meningococcus like gonococcus
can pass intact corneal epithelium. Fortunately corneal involvement is rare, there may be
pseudo membrane formation over the conjunctiva.
Other ocular features arePupillary changes due to involvement of third nerve in
basal meningitis. Mydriasis is more common than miosis. Transient extra ocular muscle palsy
is common. So is optic neuritis ; there may be papilledema due to raised intra cranial pressure.
There may be metastatic uveitis that may be mild or as severe as endophthalmitis.
Management is mostly medical by systemic antibiotic for meningitis, pneumonia and
endocarditis.
Local treatment consists of atropine ointment once a day and broad spectrum antibiotic
drops given frequently.
Moraxella
This group of mild to moderate infectiveness consists of many strains. The same organism is
known by more than one name. Previously due to its similarity in morphology and staining
characteristic to gonococcus, it was thought to belong to neisseriae. With more study of DNA,
it has been put in genus branhamella. It was formerly known as diplococcus of Morax-
Axenfeld, Moraxella lacunata, Moraxella catarrhalis or even Neisseria catarrhalis.
The organism is a gram negative paired organism that resembles gonococcus due to
its bean shaped appearance. It is found in the upper respiratory tract of almost fifty percent
asymptomatic children.
Systemic involvement includesOtitis media, sinusitis and pneumonia. Ocular
manifestations are seen in malnourished, chronically ill children. It causes blepharitis,
dermatitis of the outer canthus, chronic angular conjunctivitis, corneal ulcer that may
terminate in hypopyon corneal ulcer.
The organism is sensitive to fluoroquinolones, erythromycin, and many other antibiot-
ics. The organism produces an enzyme protease that is specifically counteracted by zink sul-
phate as 0.25 % to 0.5% drops.
Pneumococcal infection
The organism is better called streptococcus peumoniae due to its tendency to form chains
and predilection for respiratory tract. It is one of the major causes of pneumonia in all ages.
Children under two years are more prone to get the infection than older children.
The natural habitat of the organism is human nasopharynx. It has been isolated from
nasopharynx of 5% to 10% of healthy adults and 20% of healthy children. It is also found as
commensal of many healthy persons. The organism is a gram positive, capsulated
diplococcus with short incubation period. Mode of spread is through droplet from in-
fected person. Once the organism gets into the nasopharynx it spreads quickly. The common
modes of spread are - Direct spread over the respiratory epithelium mostly in the respiratory
tract, middle ear, lacrimal sae and conjunctiva.
Conjunctiva gets infected by:
1. Change of organism from commensal to pathogenic state.
2. Spread via lacrimal passage
3. Hematogenous spread
4. Direct infection by droplet.
Systemic involvement consists ofBilateral pneumonia, consolidation of lung, otitis
media, sinusitis, meningitis, peritonitis, salpingitis, osteomyelitis and pericarditis. Pneumonia
and consolidation may lead to empyema. Pneumococcus is known as true ocular pathogen.
Ocular involvements are mostly conjunctival. There may be acute, bilateral
mucopurulent conjunctivitis with or without petechial haemorrhages. Pseudo mem-
brane formation is common. Conjunctivitis may be associated with superficial keratitis,
corneal ulcer, which is generally fast spreading serpiginous ulcer with large hypopyon.
There are three ways by which the uvea may get involved:
1 By way of pneumococcal ulcer
2. Spread of toxin from lacrimal sac
3. Metastatic spread from other organs.
Uveal involvement may present as endophthalmitis. Chronic dacryocystitis is
very common. The lacrimal sac may harbour pneumococci without symptom, may have chronic
dacryocystitis. Occasionally there may be acute orbital cellulitis.
Management
Pediatrician should manage the systemic conditions by antibiotics. Common anti microbials
used areB. lactame antibiotics; once penicillin was the first drug choice, now more and more
organisms have become resistant to it. Other commonly used drugs areTrimethoprime,
cephalosporins, erythromycin, clindamycin, chloramphenicol. The organism is less susceptible
to the qunolones.
Ocular management
Conjunctivitis responds well with local erythromycin ointment, bacitracin drops. Even fresh
solution of penicillin, but poorly by qunolones. In all cases of suspected pneumococcal conjunc-
tivitis, cornea should be carefully watched for any evidence of its involvement. The anterior
chamber should be examined for AC reaction, cells, flare or even hypopyon. Hypopyon is so
common with pneumococcal dacryocystitis that in all cases of hypopyon with or without ulcer
the sac should be examined for stagnation or frank infection.
Acute dacryocystitis, orbital cellulitis, endophthalmitis require systemic anti pneumo-
coccal antibiotic in appropriate dose for sufficient time.
Corneal ulcer is treated as ophthalmic emergency.
Pseudomonas areuginosa
The organism is one of the commonest sources of hospital infection and infects accidental as
well surgical wounds frequently. It is ubiquitous, free living, opportunistic, gram nega-
tive, small, straight or slightly curved motile organism. It produces a blue green pigment
called pyocyanin that colours the pus and exudate making its diagnosis easy.
It produces large quantity of proteases that rapidly cause stromal necrosis. It is found
in 50% of water, plants, animals and humans. About 5% of symptomless persons have stool
infected by the organism. The organism does not produce clinical symptoms in healthy adults.
It mostly invades the tissue following trauma accidental or surgical or following invasive pro-
cedures. It may involve the skin or mucous membrane primarily, from where it may spread by
continuity or metastasis. Out of all infections, ocular infections are most devastating.
The systemic involvement consists of:
Respiratory infection that may be mild or life threatening, associated with cough, fever,
chills, dyspnea, cyanosis. Ear infection is common. Generalised bacterimia, endocarditis, CNS
involvement, Bones and joint involvement may be traumatic or non traumatic.
Ocular involvement by pseudomonas is a serious problem. It should be treated as
ocular emergency. Predisposing factors areContact lenses, soft extended wear contact lens
are more likely to be contaminated by pseudomonas; hard contact lenses are no exception. The
organism may be adherent to the contact lens. Other source of contact lens contamination is
contact lens solutions.
Fluorescein drops are notoriously contaminated by pseudomonas. Hence it is better
to use sterilised fluorescein strip rather than hospital prepared drops. If use of drops is
inevitable, it should be autoclaved frequently and kept away from any possible source of
contamination. The organism may be passed from one patient to other via tonometer, gonioscope
etc.
Clinical presentation of pseudomonas infection consist of redness, pain, discharge,
mucopurulent conjunctivitis, foreign body sensation, excess of watering, increased pain herald
onset of corneal ulcer. The ulcer is generally central with massive bluish green hypopyon.
Peripheral ulcer may be associated with scleritis. The ulcer if not treated progresses fast and
leads to perforation that may end up in endophthalmitis and panophthalmitis.
Management
Pseudomonas is sensitive to many broad spectrum antibiotics. Some of them areTobramycin,
ciprofloxacilin, ofloxacilin.
Staphylococcus
This is one of the most common infection world over. The organism is ubiquitous that com-
monly develops colonies on the skin and mucous membrane without much symptom or may
remain asymptomatic, 15 to 50% of population being the reservoir. They are a public health
hazard. They are the commonest organism to cause nosocomial infection. Beside skin and
mucous membrane, they are found in water, dust and soil, inanimate objects of the
hospital. Many of the persons working in the hospital are carriers of the organism. Contami-
nation of bioprosthesis including IOL, contact lenses, irrigating tubes, irrigating solutions are
common by staphylococci. The stain S. aureus is most important human pathogen.
It is one of the most tenacious organism that is potentially destructive, difficult to destroy
by sterilisation and has tendency to develop resistance to antibiotic.
The organism is a small, gram positive, non motile, non sproulating that is seen in
various forms i.e. single, in chains or in bunches. It is easily grown. The growth is best at
30-37C. It is resistant to desiccation and many disinfectants. They are aerobic or faculta-
tive anaerobic.
Basically there are two types of organism i.e. coagulase positive and coagulase nega-
tive. Out of the two the former is pathogenic and the organism is called staphylococcus
aureus, that produces golden coloured colonies. It produces many enzymes and toxins.
There are many species of coagulase negative staphylococci. They are known by various
names i.e. S. albus, S. epidermis, S. hemolyticus, S. saprophyticus.
Coagulase negative staphylococci were thought to be only contaminants in the past,
they are now considered as important opportunistic organism.
There are no known vaccines, anti toxins or toxoids that can be used as prophylaxis
against all strains and species.
Systemic involvement - Almost all organs can be infected by staphylococci. Multiple
organ involvement is common. It may develop in new born as toxic epidermal necrolysis to ripe
old age as pneumonia. It may manifest as simple single boil, carbuncle, erysipelas. Other
common systemic involvements are respiratory infection, rhinitis, otitis media, CNS infec-
tion, urinary tract infection, osteomyelitis, food poisoning and sepic shock syndrome.
Ocular involvement is common due to continuity of ocular surface with skin through
lids and nasopharynx via lacrimal passage. Other sources of intra ocular infection are mostly
intra operative and post operative.
Common ocular lesions areStye, lid abscess, boil on the lid, chronic blepharitis,
dacryocystitis, orbital cellulitis due to generalised septicimia, there may be periostitis or orbital
abscess.
Most common infection is acute or chronic conjunctivitis, other involvement in children
is phlycten. Corneal involvement may be peripheral catarrhal keratitis due to staphylococcal
toxin and suppurative keratitis.
Most dangerous infection is acute or delayed endophthalmitis following any intra ocular
surgery, or penetrating injury. S. aureus causes acute endophthalmitis while coagulase negative
strain cause delayed and chronic endophthalmitis.
Staphylococcal infection can cause anterior non granulomatous uveites, scleritis even
dacryoadenitis.
Cavernous sinus thrombosis though basically a systemic CNS infection invariably re-
port to the ophthalmologist.
Management
The infection may be caused by penicillin and methicillin resistant organism. Hence it is essential
to find out the cultural character and sensitivity to antibiotic for complete eradication.
Ocular
Blepharitis: Lid hygiene, frequent application of erythromycin 0.5% ointment or
bacitracin ointment for weeks. Oral doxycilin 100 mg OD 10
ConjunctivitisSulphacetamide drop, ciprofloxacilin 0.3% drops, frequently.
Keratitis- Fortified cefazoline drops.
Vancomycin 50 mg/ml in phosphate buffered artificial tear is used in methicillin resistant
staphylococci along with cycloplegic, may require sub-conjunctival injection.
Endophthalmitis is an emergency that requires early detection and management. Intra
vitireal injection of proper antibiotic is the most effective method to combat endophthalmitis.
Role of vitrectomy is doubtful.
Streptococcus
Human respiratory, gastro intestinal and genito urinary tracts commonly have colonies of
streptococci. A person may be asymptomatic yet may harbour the organism. It is a major cause
of neonatal sepsis, post infective acute rheumatic fever and post streptococcal
glomerulonephritis. The enteroccocci cause mostly urinary tract infection while viridans
cause endocarditis frequently.
The organism is gram positive, oval shaped, non motile, non sporulating. When
grown in liquid media, they are seen in pairs or chains. The human pathogenic strains are
facultative anaerobes. They are difficult to culture, requiring enriched media. Many strains
cause hemolysis round the colonies. This property to causes hemolysis has been utilised to
classify them into alpha and beta hemolyticus, when cultured on blood agar. About 20
percent of persons in a population may be asymptomatic carriers who are responsible for food
borne infection and nosocomial infection.
All parts of the body can be infected by streptococci. The common conditions are pyoderma,
cellulitis, erysipelas, necrotising fasciitis, scarlet fever, pharyangitis, tonsillitis, tonsilar abscess,
pneumonia and empyema, streptococcal shock syndrome, neonatal sepsis and meningitis.
Ocular manifestation can be direct involvement of the conjunctiva, spreading from
the skin, nasopharynx or metastatic.
Streptococcal conjunctivitis is generally self-limiting. It can cause membranous or
pseudo membranous conjunctivitis that can lead to corneal infiltration, ulceration and even
perforation. It can cause ophthalmia neonatorum.
Streptococci can cause preseptal cellulitis or frank orbital cellulitis. Preseptal
cellulitis is common in children.
Endophthalmitis due to streptococci has uniformly poor prognosis. It is worst with S
pyogenes and slightly better with S. viridans.
Mild to moderate anterior uveitis is also known to be due to streptococci.
Management
Most of the strains of streptococci are sensitive to penicillin. Alternate drugs include
Cephalosporins, erythromycin, clindamycine, tetracycline and chloramphenicol. Enterococci
are generally resistant to penicillin and cephalosporins. They respond well to vancomycin and
aminoglycocides.
Spirochaetaceae
8, 9
There are many organisms in this group. The most important out of them is treponema
pallidum that causes syphilis. The other two are leptospira and Borrelia. The former
cause leptospirosis, the latter results in relapsing fever, vincent angina and lyme disease.
Syphilis is one of the most widely spread chronic disease that can infect at any age and
both the sexes in all races. It can be congenital or acquired.
The organism treponema pallidum is a spiral shaped, thin. delicate looking organism
that has 6 to 14 spirals along its long axes. It is a motile organism. It is best seen on dark
field examination. It can not be cultured invitro. Humans are the only known natural
host.
The disease is mostly sexually transmitted though it can be acquired following blood
transfusion with infected blood. Congenital syphilis is mostly due to transplacental spread
from infected mother to the growing foetus generally in the third or fourth month of gestation.
However, older children can acquire it following child abuse. In such cases they go through all
the stages of adult acquired syphilis i.e. the primary, secondary, latent and tertiary stage.
Systemic involvement in congenital syphilis
The disease can be transmitted to the foetus any time during pregnancy if the mother is
serologically positive. It develops more commonly by fourth month. Mothers in early stage of
infection are more likely to infect the foetus than those who had infected period of more than
two years. Early treatment of the mother before sixteen week is said to protect the foetus.
Abortion and still births are two common modes of termination of congenital syphilis. It is a
major cause of neonatal death.
The clinical presentation of congenital syphilis in surviving neonates can be divided
into three stages:
1. Early features that appear within first two years of age.
2. Late manifestation that develop after two years
3. Residual effect of late manifestation
The systemic manifestations differ in infants and older children.
InfantsPresence of congenital syphilis is not felt at birth. They become obvious by
second to tenth week post natal with hepato spleenomegaly, jaundice, anemia
thrombocytopenia, lymphadenopathy. Skin rashes that may be bullae or vesicle,
rhinitis, osteo chondritis.
The ocular signs are absent at this stage:
Older childrenAnterior bowing of shin, periostitis, frontal bossing, Clutton joints,
saddle nose, mulberry molars, peg shaped teeth, eighth cranial nerve involvement and ocular
manifestation.
Ocular manifestations are late manifestation of congenital syphilis that has remained
untreated for more than two years.
Commonest ocular manifestation of congenital syphilis is interstitial keratitis that
develops between two to twenty years, is a bilateral condition.
It is always associated with sever anterior uveitis that is associated with endothelial
edema, leading to generalised edema of the cornea and development of deep vascularisation.
The deep vascularisation gives rise to salmon patch appearance, which gradually subsides to
leave obliterated corneal vessels as ghost vessels. It takes years for the vessels to regress. A
ground glass appearance of the cornea is common.
Other features are bilateral chorioretinitis, which gives an appearance of salt and pep-
per pigmentation also known as pseudo retinitis pigmentosa. Secondary glaucoma is com-
mon due to changes in the angle secondary to anterior uveites.
The differential diagnosis of interstitial keratitis include other condition of hazy
cornea in childrenCorneal birth injury, late corneal injury, congenital glaucoma, corneal
endothelialdystrophy, congenitalrubellasyndrome, mucopolysaccharidosis, mucolipidosis.
Conditions that give pepper salt appearance of the retina besides congenital syphilis
are rubella, cytomegalovirusretinitis, influenza.
Neonatal congenital syphilis should be differentiated from - Rubella, cytomegalo virus
infection, toxoplasmosis, herpes simplex and erythroblastosis fetalis.
Management
The condition is preventable if the mother is adequately treated before sixteenth week of ges-
tation. The infected child is treated with aqueous crystalline penicillin in consultation with
pediatrician.
Ocular condition is treated with atropine and local steroid over months.
Leptospirosis
Leptospirosis as an organism that belongs to order spirochaetales. Leptospirosis is less
frequent disease. It is seen as epidemics in certain parts of tropical and subtropical countries
where financially less privileged persons live in unhygienic conditions in close contact the
animals. The disease is zoonosis, is spreads commonly from certain animals to humans. Human
to human spread is possible but less frequent. Drinking water contaminated by urine of infected
animals or human beings is also a mode of spread. Many animals, fish or birds are the reservoir
of the organism.
The organism is a spirocheate, it is a thin highly motile spiral organism that stain
poorly but is clearly visible on dark field examination.
Many animals act as reservoir for the organism. These animals rarely develop the disease.
Humans acquire the disease by contact with urine or tissue of infected animals, or by
drinking contaminated water. The organism commonly reaches the body through abraded
skin or mucous membrane.
The incubation period is one to two weeks. The organism can be recovered from blood,
CSF, aqueous within twenty four hours.
The virulence depends on toxin production. Clinically the disease has two stages-
1. Phase of leptospiracmia
2. Immune phase
The first phase consists of influenza like symptoms of pain all over the body, fever,
chills, skinrashes, gastrointestinal disturbance, respiratory infection. One of common findings
at this stage is conjunctival effusion.
The second phase lasts for several weeks. The child may become asymptomatic or
may have serious complications like encephalitis, Guillain-Barre syndrome, multiple cranial
nerve palsy, peripheral nerve involvement and myocarditis.
Ocular manifestations are common and variable. Commonest finding is conjunctival
effusion without lacrimation or discharge. If there is discharge, it may contain leptospira.
Other ocular manifestations areIridocyclitis that may be mild and self limiting.
Rarely may have chronic uveitis. Neovascularisation of iris, hypopyon, mutton fat KPs, broad
posterior synechia, complicated cataract and secondary glaucoma. Retinal haemorrhage,
choroiditis, chorio retinitis, vitreous membrane have also been reported.
Management consists of:
Prevention by personal hygiene, food hygiene, drinking potable water and chemo prophy-
laxis during epidemics by way of 100 mg doxycyclin once a day for a week in adults.
The organism is sensitive to ampicillin, erythromycin, doxycyclin, tetracyclin. Severe
cases may require injection IV penicillin G.
Conjunctivitis does not require any separate treatment if the patient is on systemic
antibiotic otherwise erythromycin ointment or fresh penicillin drops may be required.
Iridocyclitis is treated by standard method.
Lyme disease
The disease is spirochetal disease caused by Borreliaburgdorferi. It is a multi systemic
disease. It is one of the arthopod related diseases that is spread by bite of tick ixodes. Many
mammals are host of the tick.
The disorder involves multiple symptoms. Common areSkin rash. neurological signs consist
of Bells palsy, other cranial nerve palsy, meningitis, peripheral neuritis, late presentation are
arthritis and peripheral neuropathy.
Ocular involvementAny part of the eye may be involved. They can be divided into
two groups i.e. secondary to neurological causes and those due to involvement of the eyeball
proper. These are mostly immune mediated inflamations. The signs and symptoms of both the
groups can run simultaneously. They are facial palsy, paralytic squint, optic neuritis, retrobulbar
neuritis, optic atrophy, pseudo tumour cerebri, papilledema, conjunctivitis, symblepharon,
keratitis, episcleritis.
Intra ocular manifestations areIritis, parsplanitis, and chorioretinitis.
The involvement may be unilateral or bilateral. One eye may be involved later than the
other may. The lesion can be single or multiple.
Management
The disease mimic many diseases. It is relatively rare hence knowledge of the disease and high
degree of suspicion is required. Elisa test and immuno fluorescent assays are the two
investigations that may clinch the diagnosis.
The organism is sensitive toPenicillin, amoxycillin, erythomycin, roxithromycin,
tetracycline and doxycyclin.
Ocular management consists of management of Bells palsy, conjunctivitis, keratitis
and episcleritis by standard methods.
Proteus
This is a saprophyte that is found in decaying organic matter, soil, water and GI tract of
many animals and human beings. Generally infection by proteus is mild and may be asympto-
matic unless they become opportunistic and cause wide spread infection.
The organism is gram negative, actively motile, non lactose fermenting. It pos-
sesses an enzyme urease, making it a potential urinary tract infective organism.
Systemic manifestationCommonest infection is urinary tract infection. The uri-
nary tract acts as portal of entry for other organs. Proteus invades immuno compromised
individuals and debilitated individuals. It is a known contaminant of wounds, accidental or
surgical and burns.
Other systemic manifestations areOtitis media, mastoiditis, lateral sinus throm-
bosis, meningitis, even brain abscess.
Ocular manifestationThough external ocular infections like conjunctivitis, keratitis
is known to be caused by proteus, they are mostly due to trauma. However proteus is a poten-
tially blinding organism following penetrating injury that may be accidental or surgical. Rarely
it can cause dacryocystitis and scleritis.
Management
The organism is sensitive to many antibiotics i.e. ampicillin, third generation cephalosporin,
all aminoglycocides.
Ocular treatment is by fortified gentamycin 14 mg/ml or tobramycin 14mg/ml fre-
quently or by 0.3% ciprofloxacilin or ofloxacilin 0.3% drops hourly.
Endophthalmitis following either accidental or surgical is a serious condition requiring
intra vitreal injection of gentamycin or amikacin. Vitrectomy may be required.
SalmonellosisTyphoid (enteric fever)
Typhoid is one of the common acute febrile conditions in all ages. It is very common in chil-
dren. It is a preventable public health problem that may cause death if not treated.
There are many organism in the salmonella group, only few cause human infection. The
salmonella typhi the causative organism for typhoid fever is found only in humans.
The disease is transmitted by ingestion of contaminated drink or food. The disease
also spreads due to person to person contact during acute phase. It can also spread from
asymptomatic carriers. Faecal oral contact is commonest mode of spread in all ages, more so in
children.
S. typhi is a gram negative, motile, facultative anaerobe. Incubation period varies
between 3 days to 60 days.
Systemic involvement is basically enteritis, which is heralded by fever that is pro-
longed and persistent. There is step like rise of temperature, rose spots all over the body.
Relative bradycardia is supposed to be a strong suggestive factor. There may be diarrhoea or
constipation. The small intestine is commonly involved. There is hyperplasia of Peyers patches,
mucosal ulceration, bleeding and perforation.
The child is generally delirious, has severe anorexia and loss of weight. The child may
recover from these early phases or may pass into more widespread systemic involvement that
includes almost all systems. There may be meningitis, encephalitis, paralysis of cranial nerve,
pneumonia, bronchitis, myocarditis, cholecystitis, osteomyelitis, arthritis.
The child becomes afebrile with adequate antibiotic in four to seven days. In about 10%
cases there is relapse. About 2% to 3% cases develop chronic carrier status and public health
hazard.
Ocular manifestations are rare, when present are due to hematological spread of
the organism. They areLid abscess, dacryoadenitis, hemorrhagic spots on the conjunctiva,
keratitis, uveitis, vitreous and retinal haemorrhages, paralysis of extra ocular muscles.
Management : ProphylaxisThe disease is preventable if environmental, sanita-
tion, water supply and sewage disposal could be improved. However if all children are sup-
plied with potable water and instructed to follow proper food hygiene, the incidence falls sharply.
Other prophylactic method is to give prophylactic oral vaccine and repeated every two years.
The first antibiotic found to be effective against typhoid was chloramphenical. The drug
though very effective had many untoward side effects. The chloramphenical was followed by a
long list of antibiotics that includes-Trimethoprim-sulphamethoxazole, ampicillin, amoxycillin,
fluoroquinolones, third generation cephalosporins.
Ocular manifestations are treated by standard methods along with systemic antibi-
otics.
Ocular manifestation of systemic virus disease
11, 12, 13, 14
Viruses are smallest infective organisms. They are called primitive organisms because they
contain only one single nucleic acid either DNA or RNA. The viruses consist of nucleic
acid surrounded by one or more proteins. The viruses themselves can not replicate for
which they require a host-cell, hence they are called obligate intracellular parasites. Some
of the viruses have an outer cover membrane that protects and stabilizes the nucleoid from
environment outside and helps the virus to invade host cell. The outer cover, which is protein
in nature, is responsible for antigenicity of the virus. On which depends the immunity and
diagnostic tests. The antigenecity is usefully utilised to manufacture vaccine.
The invading viruses get attached to a special receptor site on the host cell membrane.
The viruses are then pagocytosed.
The viruses do not possess enzyme required for reproduction and survival. They depend
on the host cell for the enzyme, energy and precursor for multiplication.
The RNA viruses replicate in the cytoplasm of the host cell. The DNA replicate in the
nuclei.
The RNA viruses that have ocular involvement are
Entero virus (Picorna viruses)They includePoliomyelitis, Coxsackie virus and ECHO
viruses.
Paramyxo virusesMumps, rubella and measles viruses.
Human immuno deficiency virus
The DNA viruses that have ocular involvement are
Pox virusesSmall pox virus (variola) and vaccinia virus.
Herpes virusesChicken pox (varicella), herpes simplex, herpes zoster, and cytomegalo vi-
ruses.
Other virusesMolluscum contagiosum, human papilloma virus and human adeno viruses,
Epistein-Barr virus.
Poliomyelitis
This viral disease has world-wide distribution. Though it has almost been eradicated in devel-
oped countries, it remains a major cause of death and physical handicap in developing coun-
tries. Its ocular manifestations are few and overshadowed by motor paralysis and deformity.
Like any other entero virus, poliovirus is transmitted by fecal-oral route. It has a
short incubation period of three to six days. Most of the time it is asymptomatic. Only five
percent of children have mild symptoms of fever, malaise, and body ache that is generally
passed as influenza or other viral fever. Only one percent develops aseptic meningitis which is
the cause of most visible sequel i.e. motor paralysis.
The central nervous system involvement has been divided anatomically into- meningeal,
encephalic, bulbar, cerebellar and spinal.
The bulbar form is most likely to cause ocular involvement in the form of cranial nerve
palsy. Commonest extra ocular muscle to be involved is lateral rectus. Involvement of fourth
nerve is least common. Some children may develop seventh nerve palsy. Nystagmus is
very frequent. Pupillary abnormalities are part of third nerve involvement. Horners syndrome
develops rarely. Involvement of optic nerve is the cause of transient visual loss. The ocular
involvements are self limiting.
Coxsackie virus
They cause less ocular manifestation than poliomyelitis. Systemic infection may lead to necrosis
of skeletal muscles, tremor, spasticity, flacid paralysis, encephalo myelitis, aseptic meningitis.
The ocular involvements are either secondary to involvement of CNS or due to primary
infection of conjunctiva and cornea resulting is kerato conjunctivitis, keratitis, phlycten, corneal
pannus, pseudo membranous conjunctivitis.
ECHO viruses produce ocular manifestation similar to Cox sac kie virus.
Mumps
Mumps is common acute contagious disease of childhood. Commonest presentation is bilat-
eral, almost symmetric painful swelling of parotid glands. The condition has a
prodromal stage of fever, body ache, anorexia, and malaise. It gives a life long immunity.
Life long immunity is also acquired following immunisation.
The virus is a paramyxo virus, which is a RNA virus. It is one of the smaller viruses.
It is pleomorphic.
The disease spreads as droplet infection via saliva and fomitis. The incubation period is
14-20 days.
After prodromal period that may last for one to two days, the child develops fast growing
bilateral swelling, difficulty in swallowing and pain in the ear. It is common to develop similar
swelling in the sub mandibular glands. Sub mandibular glands are always associated with
parotitis. The swelling of the gland increases for two to three days and then subsides gradually
leaving no trace of infection.
Other organs to be inflamed are testes, pancreas, ovaries. The orchitis is common in
post pubertal patient. About 20% males are effected. The swelling is generally unilateral and
painful. Late complication of orchitis is testicular atrophy. As testicular atrophy is unilateral
it does not lead to sterility in men.
Oophoritis is less common, presents as pain in lower abdomen and does not cause steril-
ity.
More serious manifestations are
Aseptic meningitis, encephalitis, transverse myelitis, cerebellar ataxia, facial palsy.
Ocular involvement
Commonest ocular involvement is acute dacryoadenitis, which is bilateral, may cause nar-
rowing of palpebral fissure and mild proptosis. Other ocular involvements are conjunctivi-
tis, keratitis, scleritis, iridocylitis. There may be sub conjunctival haemorrhages and rarely
interstitial keratitis. Bilateral optic neuritis is self limiting and has good prognosis. Rarely
extra ocular palsy or pupillary abnormality may develop.
Management : Prophylaxis
An effective mumps vaccine is available that is administered as part of MMR vaccine that
includes measles and rubella vaccine. It is generally administered after 12 months of age as
subcutaneous injection. It gives life long immunity.
TherapeuticThere is no known antiviral drug effective against mumps virus. The
management is symptomatic and supportive in the form of analgesic. Role of steroids is not
established.
Ocular management is symptomatic by standard methods of antibiotic drops, cycloplegics
and local steroids.
Rubella (German measles)
This RNA paramyxo virus causes a serious systemic infection that is fully preventable. It
causes multiple crippling congenital anomalies in the foetus if the mother acquires the disease
in first trimester. Infection of the mother after fourth month does not cause much damage to
the foetus but the foetus is not altogether safe. The infection gives a life long immunity, subse-
quent pregnancies are safe.
The disease occurs in endemics in population that have not been immunised. The dis-
ease spreads by droplet. The incubation period is 12 to 23 days. The infected droplets cause
infection in the respiratory tract which is followed by hematogenic spread.
There are two modes of presentation
1. Common mild post natal infection that may go unnoticed.
2. More serious life and vision threatening congenital infection
Commonest age to get post natal infection is school going children. This does not mean
that other ages are immune. It can occur at any age. Rubella is contagious in all ages even in
asymptomatic cases.
The disease has a prodromal phase of malaise, fever and anorexia. The actual infection
is always associated with suboccipital, postauricular lymphadenopathy, fever and
rashes.
Congenital rubellaMaternal infection during first trimester is most important cause
of severe multiple congenital anomalies in the developing foetus. The foetus is infected
transplacentally. The classical signs of congenital rubella is rubella syndrome that in-
cludes cataract, heart disease and deafness. Other systemic involvements areLow birth
weight, retardation of growth, hepatospleeanomegaly, pneumonia, thrombocytopenia. The com-
mon cardiac anomalies arePulmonic stenosis and ductusarteriosus. Other anomalies are
dental anomalies, cleftplate, and microcephaly.
The ocular manifestations are
There are hardly any ocular involvement in acquired rubella except catarrhal conjunctivitis
and superficial keratitis.
Ocular manifestations of congenital rubella are many. They areMicrophthalmos, cen-
tral cataract and retinopathy.
Less common ocular features are congenital glaucoma, corneal haze, iris hypoplasia,
nystagmus and squint.
Late complications include progress in retinopathy, subretinal neovascularisation and
maculopathy.
The rubella cataract is bilateral, almost symmetrical, slightly eccentric, dense, nuclear
type. The lens is known to harbour live virus up to two years of age. Hence surgery should be
followed by frequent and prolonged use of local steroids and cycloplegic with usual caution.
Every effort should be made to aspirate the cortical matter as much as possible.
The congenital glaucoma develops later than cataract and is severe in nature. The cause
of glaucoma is multifactorial i.e. spherophakia, microphthalmos, anomalies in the angle, sec-
ondary to uveitis or post cataract surgery status.
Measles (Rubeola)
16
Measles is a common acute exanthematous respiratory disease, which is highly contagious
resulting in epidemics. The disease is potentially fatal. It is met with all over the world,
more in under develop countries. The organism belongs to family paramyxo viridae and genus
Morbilli virus. There is only one genetic type. Humans are the only natural host.
The disease is mostly seen in children between 6 months to 2 years. The disease spreads by
droplet infection. The disease is most contagious from one to two days before the onset of
symptoms and remains so for four days after the rashes have appeared.
The incubation period is of 10 days. The disease has following stages:
1. The prodromal stage
2. Stage of exenthema
3. Complications
In prodromal stageThere is fever, rhinitis, cough, Koplic spots in the buccal mucosa,
and conjunctiva. The Koplic spots are blue-white spots against pink background.
In stage of exenthema, the body is covered with rashes. They last for 3-4 days and then
disappear without leaving any scar.
The complications are related to respiratory system, central nervous system and GI
tract. It is not uncommon for more than one system to be involved at the same time, which
includes laryngitis, pneumonia, otitis media, deafness, encephalitis, gastroenteritis.
The ocular involvement
The conjunctiva is involved in prodromal stage and continues to be infected through the
exanthematous stage. Commonest conjunctival involvement is catarrhal conjunctivitis, Koplic
spot, swelling of plica, edema of lids. This is followed by severe photophobia due to epithelial
keratitis. The cornea if infected by bacteria at this stage is bound to end in corneal ulcer, which
may perforate if the child is malnourished with vitamin A deficiency. Other ocular manifesta-
tions are dacryocystitis
15
and frequent stye.
The neuro ophthalmic complications due to measles are - Encephalopathy, that may
lead to optic neuritis, papilledema, or paralytic squint.
Measles and vitamin A deficiency
Severity of measles is more in malnourished children. Death is more frequent in malnourished
child. Children with borderline vitamin A deficiency develop night blindness and xerophthalmia
soon. Measles itself can cause protein caloric malnutrition. It also depletes liver store of vita-
min A.
Management
ProphylaxisMeasles is a fully preventable disease by live attenuated measles vaccine. All
children between 6 months to 6 years should get oral vitamin-A 1,00,000 IU.
Systemic involvementThere is no specific antiviral drugs against measles. Only
symptomatic and supportive treatment is required with a close watch on possibility of pneu-
monia, enchephalopathy and gastroenteritis which require special attention.
Ocular involvement is treated by local antibiotic, cycloplegic and non steroidal anti
inflammatory drugs.
Once the child has been diagnosed to have measles, irrespective of his nutritional sta-
tus, the child should get extra vitamin A. Common practice is to give 50,000 IU under six
month, 100000 IU under one year and 200000 IU over one year in two equal divided doses.
Human immuno deficiency virus
17,18
Human immuno deficiency virus contains RNA but no DNA. The virus uses hosts cellular
mechanism to form DNA for its reproduction, depleting the immune system of the host. The
human immuno deficiency virus type 1 (HIV 1) causes a multi systemic chronic contagious
disease called acquired immuno deficiency syndrome (AIDS). It takes years to develop
the systemic and ocular involvement in AIDS. Two third of the patients with AIDS will de-
velop ocular symptoms. The condition is uniformly fatal. Presently available drugs do not
change the immune deficiency but act against mostly the opportunistic organisms.
The disease is spread by body fluid. It is most commonly transmitted sexually. It can
also be transmitted via infected blood products. The mother can pass the infection to the foetus
via placenta. The neonate may acquire the disease during passage through infected birth ca-
nal. The breast-fed baby may get it from mothers milk. All body fluids including tears, aque-
ous, or saliva may be source of infection. Older children get infected either through infected
blood products, infected needles or following sexual assault.
The HIV virus has affinity for CD4+ lymphocytes. The virus cripples the CD4+ cells,
resulting in diminished and later deficient host immunity.
The diagnosis is confirmed when:
1. There is confirmed diagnosis of HIV I infection.
2. The CD4+ cell count is less than 200 cells/ mm
3
.
3. There is evidence of atleast one opportunistic infection.
4. There is some AIDS related malignancy.
The ocular infections are early to develop. The ocular lesions can be:
1. Retinal micro angiopathy
2. Ocular infection:
(i) Intra ocular
(ii) Extra ocular.
By one or more opportunistic organisms
The opportunistic organism can be viral, bacterial or protozoal. More than one opportunis-
tic infection may be present.
3. Uncommon neoplasms
4. Neuro ophthalmic complications
Retinal micro angiopathy is commonly known as HIV retinopathy, is the commonest
ocular manifestation. The angiopathy is due to direct invasion of endothelium of the retinal
vessels. The result is development of cotton wool spots, micro aneurysm, ischemic
maculopathy. The lesions are produced by deposition of immune material in the vessels after
the endothelium has been invaded by the virus.
Ocular infection
Intra ocular infectionRetinitis
Common opportunistic organisms areCytomegalovirus, herpes simplex, herpes zoster,
toxoplasma, pneumocystiscarinii, mycobacterium, candida, and cryptococci.
Commonest and early intra ocular infection is cytomegalovirusretinitis (CNV). The vi-
rus is member of herpes family. The CNV affects neonates and adult immuno compromised
patients. Almost one third of AIDS patients develop CNV in under developed countries where
triple anti viral therapy is not available. The lesions develop along the retinal vessels due to
invasion of the endothelium by the virus. The lesions are white granular dots. Superficial
haemorrhage are common. Other findings are - Retinal edema, retinal vasculitis, and
full thickness necrosis of the retina. The scattered spots, coalase to form larger area of
necrosis that is replaced by gliosis. Posterior hole formations are common, leading to retinal
detachment that is difficult to treat.
Early symptoms include diminished distant vision, floaters, central or ring shaped
scotomas.
Treatment of CNV retinitis is difficult and costly. It includes IV ganciclovir for fifteen
days followed by oral maintenance dose. Other mode of treatment is called highly active anti
retro viral therapy (HART) which is a combination of anti retroviral drug and protease inhibi-
tor. The anti retro viral drugs can also be given intra vitreous.
Progressive outer retinal necrosis (PORN)
This is rarer opportunistic infection in AIDS. It is caused by herpes zoster and other herpes
simplex viruses. It starts as necrotising lesion of the peripheral retina that progresses re-
lentlessly in circumferential fashion without involving retinal vessels. The condition is poten-
tially blinding disease; about 80% of patients with PORN go blind inspite of treatment.
Uveitis
Multi focal choroiditis is caused by many organisms. The infection may be due to a single
organism or a combination of organism like mycobacteriumtuberculosis, atypical
mycobacteria, pneumocystitiscarinii, cryptococcus, toxoplasmosis. In contrast to multi
focal choroiditis there may be solitary choroiditis due to syphilis which is not an opportunistic
organism because those who develop syphilitic choroiditis have CD4+ cell count >200. It gen-
erally occurs in patients with neuro-syphilis.
The other intra ocular manifestations are non-granulomatous iritis, optic neuritis,
retrobulbar neuritis, and papilledema.
Extra ocular involvement may be due to infection by opportunistic organism, neo-
plasm or CNS involvement.
They are:
Follicular conjunctivitis, keratitis, punctate or geographic that may look like herpes
simplex keratitis.
Neoplasms associated with AIDS are:
Kaposis sarcoma, Burkitts lymphoma, squamous cell carcinoma of the
conjunctiva.
Kaposis sarcoma is very aggressive neoplasm of endothelial cells. About one fifth of
persons with AIDS develop Kaposis sarcoma, lesions of the skin of the lids are more common
than conjunctiva. Orbital involvement is still rarer.
CNS involvement may cause encephalitis, meningitis, papillitis, papilledema, retrobulbar
neuritis and extra ocular muscle palsy.
Smallpox virus (Variola)
Variola was a common, often fatal that left surviving children with life long scarred body and
many a times blindness is no more seen. It has been eradicated in the year 1980 as a result of
simultaneous global immunisation. It was a common cause of bilateral anterior staphyloma
which can still be seen in patients in remote areas, who were borne before 1977, when the last
case of small pox was seen.
Vaccinia
This is also a DNA virus that is closely related to smallpox virus but not identical to it. The two
conditions have different presentation, mode of infection and host range. Vaccinia virus is
used as prophylaxis against smallpox. Primary systemic vaccinia does not occur but is a dis-
tinct possibility. The vaccinia presents as auto inoculation, contact inoculation, complicated
scar of vaccination and rarely vaccinia encephalopathy.
The lesion following auto inoculation or contact inoculation resembles single blister of
small pox. The pastular lesion develops three days after vaccination; it can be anywhere on the
body but more common on the face.
The ocular involvement is mostly in the lids. Less common are the conjunctival and
corneal involvement. There is intense swelling of the lids, the preauricular lymph glands may
be enlarged. It takes eight to ten days for the condition to subside.
Conjunctival lesion may be a pustule associated with purulent conjunctivitis. Pseudo
membrane may form.
The cornea is involved secondary to lid lesion. It may be acute or chronic. The former
is in the form of superficial punctate keratitis of mild nature that heals without any treat-
ment unless contaminated by bacteria. The chronic form is a severe but rare disciform
keratitis.
Rarely there may be iridocylitis and peri vasculitis.
No specific treatment is required.
Herpes viruses
This group consists of herpes zoster, varicella (chicken pox), herpes simplex and
cytomegalo virus.
Varicella (chicken pox). This is very common, benign, highly contagious disease of
childhood seen world wide. Unlike small pox there is no universal immunisation against chicken
pox.
The virus that causes chicken pox causes herpes zoster in adults. However it should be
kept in mind that herpes zoster ophthalmicus is seen in pediatric age group as well. The virus,
which is more commonly, called varicella zoster virus looks similarly to herpes simplex
virus, but differs in chemical properties, antigenically and clinical presentation.
The chicken pox, which is an acute exanthematous disease spread by droplet infection. The
incubation period is of 10-20 days. The patients are infectious two days prior to development of
rashes. The patient remains infectious during the vesicle formation and during crusting.
The skin lesions are the most visible signs of the disease. The lesions begin as maculo-
papules, gradually going to vesicle that scab in about ten to fifteen days. The scabs fall leaving
no permanent scar. The lesions can be seen on all exposed mucous membranes and skin. The
skin lesions are associated with fever.
The complications arise if there is secondary bacterial infection superimposed, or the
child is immune compromised. Extra cutaneous lesions are seen mostly in CNS in the form of
meningeal irritation and ataxia. There may be meningitis, encephalitis, and transverse myeli-
tis.
Varicella infection of the respiratory tract in the form of vericella pneumonia is most
serious complication.
The ocular manifestations areVesicles may form on the lids and conjunctiva. In-
fection may spread to the lacrimal sac from conjunctiva or through nasopharynx. Chicken pox
is a common cause of chronic dacryocystitis in children.
15
Corneal involvement can occur
at the vesicular stage as punctate keratitis or may develop disciform keratitis late.
Herpes zoster
13, 19, 20
Herpes zoster is a sporadic disease mostly seen in adults but not an exception in children.
Manifestation in children is milder and less painful. The disease in all ages in caused by same
virus i.e. vericella herpes zoster virus. It is equally seen in both the sexes.
Herpes zoster is a unilateral disease. There is no predication for right or left side of the
body. The virus involves one single dermatome at a time. Both cranial nerves and spinal nerves
are involved. Simultaneous involvement of cranial and spinal nerve never occurs. The com-
monest dermatomes involved are T3 to L3. Commonest cranial nerve to be involved is
trigeminal. Out of three divisions of fifth nerve, the 1st division is most commonly involved
followed by maxillary. The third division is least involved. It is not uncommon to have involve-
ment of only few branches of 1st division, commonest being supra orbital. The involvement of
first division of the trigeminal is called herpes zoster ophthalmicus.
The infection starts most probably as varicella in the childhood and the organism lies
dormant in the dorsal roof ganglion and manifests only when the organism is reactivated.
Most of the patients do not have recent history of exposure. It is said that about 2 percent of
patients may develop second attack of herpes zoster.
19
The typical lesion develops as unilateral vesicular eruption along the dermatome
anywhere on the body that may be painful or preceded by pain. Most of the lesions will be
followed by post herpetic neuralgia that is more marked in adults. Older the patient greater
is the pain, eruption in the distribution of cranial nerves is more painful than those of spinal
nerves. The vesicular lesions look very similar to those of varicella. Generally it takes 7 to 10
days for the vesicles to crust and leave hypo pigmented shallow scars. There is hyposthesia
along the dematome.
In herpes zoster ophthalmicus, the posterior roof ganglion involved is trigeminal gan-
glion. The geniculate ganglion is involved in Ramsay Hunt syndrome that includes
Vescicles in the external auditory canal, pain in the ear, loss of taste in the anterior two third
of the tongue.
Most troublesome part of herpes zoster of spinal nerve is acute neuritis, result in pain,
absence of touch sensation. This lasts for months to years.
Other complication areMeningeal irritation and granulomatous angitis that may re-
sult in contra lateral hemiplegia, transverse myelitis and cranial nerve palsy otherthan the
fifth and seventh.
Most important systemic complication of vericella herpes zoster virus infection is cuta-
neous dissemination where the skin lesions last for weeks. Other causes of dissemination are
herpes zoster pneumonia and meningo encephalitis.
Ocular manifestation of herpes zoster
21
is herpes zoster ophthalmicus (see page 52).
Some important points are:
1. Ten percent of all cases of herpes zoster infection are herpes zoster ophthalmicus.
2. Fifty percent of cases of herpes zoster ophthalmicus develop serious complication.
3. Ocular involvement is possible if maxillary branch is involved, may involve nasociliary
nerve.
4. Lacrimal branch is generally spared.
5. Hutchinsons sign is not a sure sign of corneal involvement.
6. Corneal hyposthesia even anaesthesia may develop and linger for years.
7. Anaesthesia dolorosa is common.
8. Lagophthalmos may develop in Ramsay Hunt syndrome.
9. Other extra ocular muscle palsy is possible which recover spontaneously in few
months.
10. In acute stage mucopurulent conjunctivitis is common.
11. Corneal lesions are many. They may start as superficial punctate keratitis or micro
dendrites; may become nummular or even disciform lesion may develop.
12. Episcleritis and scleritis are generally over shadowed by skin and conjunctival le-
sion.
13. Iridocyclitis is common.
14. Secondary glaucoma is frequent.
15. Optic neuritis has been reported frequently.
Herpes Simplex virus
Herpes simplex (HSV) infection is a world wide health problem that is seen in all ages includ-
ing neonates, in both the sexes. About 90% of adults are sero positive for herpes simplex
virus.
Herpes simplex virus is a complex DNA virus. There are two types of herpes simplex
viruses i.e. type I and type II. The latter is also known as genital virus that can infect the
eye.
It is acquired sexually and from the genitals spread to other parts of the body after a
period of latency followed by reactivation. It can also involve the eye. The type I generally
infects the body above waist. The lesions spread by infected saliva.
The infection is said to be primary when it develops in a non-immune person through
infected secretions. It is generally acquired in early ages. The primary infection can be sub-
clinical or clinical. The infection then travels to the sensory ganglion, serving the area of
the primary infection i.e. trigeminal ganglion for HSV1 and spinal ganglion for HSV2.
The virus remains inactive in the root ganglion for variable time called latency, then
following some trigger mechanism the virus travels down the axon to the target tissue and
cause recurrent infection that keeps on recurring with period of apparent cure only to recur
at irregular intervals.
The incubation period is 6 to 8 days. The infection starts either through mucous
membrane or via an abraded skin. The foetus can get the infection via placenta in an in-
fected mother. The neonate gets the infection from the birth canal of the infected mother.24
Systemic involvement depends on the type of the virus, i.e. type 1 or type 2, site of the
primary lesion, target tissue, and immune status of the patient. Except for neonates, the her-
pes simplex virus is not a lethal virus. In immuno compromised especially in patients suffer-
ing from AIDS the organism behaves like an opportunistic organism and hasten the death.
The systemic involvement consists of:
1. Labial infection, vesicles on the lips or muco-cutaneous junction of the mouth
2. Infection of the upper respiratory passage i.e. pharyngitis, tonsillitis.
3. Upper GI tractgingivitis, stomaititis, infection of hard pallet, tongue, soft pallet.
4. Genital infection
5. Infection of central nervous system
6. Infection of peripheral nervous system
7. Herpetic whitlow
Ocular manifestations are many, some of them are benign, others are potentially sight
threatening.
They are:
1. Vesicles on the lid skin or at the junction of conjunctiva and skin. The vescicles that
crust soon and heal without scar or loss of pigment. Sensation is normal.
2. Acute follicular conjunctivitis with enlargement of pre auricular lymph nodes. There
may be pseudo membrane formation.
3. KeratitisEpithelial : Half of the children develop superficial punctate keratitis
scattered over the cornea. They become coarse within few days. This is followed by
dendritic ulcer. The dendritic keratitis begins as coarse epithelial lesions in a lin-
ear form, which later develops branching. The branching is on one end, new lesions
develop at the terminal of the branching. The dendrite heals from one-end and
progresses at the other end. The course of events may be one of the following:
(i) The dendritic ulcer heals within ten to twenty days with treatment, may even
heal without treatment.
(ii) The dendritic figure expands in width and a geographic or amaeboid ulcer
develops. Rarely the condition may pass into disciform keratitis.
(iii) Stromal Necrotic keratitis. This is due to invasion of the stroma by the virus.
Some times the epithelium over the lesion may be intact. In majority of cases the
epithelial defect persists.
(iv) Disciform keratitis. This may appear following dendritic ulcer or even without
it. It is a hypersensitive reaction. The disciform lesion is central, oval diffuse.
It may be sub-epithelial lesion with stromal thickening surrounded by ring
of infiltration. There may be folds in Descemets membrane. Anterior
uveitis is common at this stage. The condition takes few weeks to months to
clear.
5. Keratitis meta herpetica. This is not caused by viral invasion neither is an
immune reaction. It is a persistent defect in the basement membrane.
6. Loss of corneal sensation is common. It starts from the stage of superficial
punctate keratitis and lingers for months.
7. Uveitis is a common feature of HSV. It may be associated with keratitis at any
stage or happen independent of keratitis. The uveitis is generally acute. That
may lead to iris atrophy.
8. Secondary glaucoma is very frequently associated with HSV keratitis and
uveitis and may be the cause of severe pain disproportionate to corneal lesion.
9. Superficial and deep vascularisation are common in long standing epithelial and
stromal defects. (For details see chapter on cornea)
Cytomegalo virus
Cytomegalo virus is a DNA virus, morphologically similar to herpes simplex. The exact route
of infection is not known. Percentage of persons with positive titer is high. It is estimated that
about 4.5% pregnant women excrete virus in urine. The virus is also secreted in breast milk
and saliva.
The condition can be congenital or acquired, can be seen in neonates or in adults. Immuno
compromised person are very often infected by the virus.
The foetus gets the infection from the infected mother. The neonate can be infected
during birth via infected birth canal.
Congenital CMV infection can be asymptomatic or the neonate may be seriously ill.
Common features are under weighed child, microcephaly, hepatospleenomegaly,
thrombocytopenia, patechial haemorrhage. There may be intra cranial calcification.
There may be asymptomatic chorioretinitis.
Acquired CMV differs in presentation in immuno competent and immuno compromised.
In immuno competent, it presents as mono nucleosis, mostly in sexually active males.
Incubation period is 20-60 days. There is fever with chills, body ache, headache, spleenomegaly;
there may be pneumonitis.
CMV infection in immuno compromised person is a serious infection. It is a multi sys-
temic disease.
Ocular manifestations is chorioretinitis. In neonates it is similar to toxoplasmic chorio
retinitis. In AIDS, it is bilateral and extensive potentially blinding condition.
Molluscum contagiosum
11,22,23
This is a DNA virus in the family of poxviridae. It has limited systemic involvement in nor-
mal healthy persons except the skin lesion which are self healing. In immuno deficient, it has
prolonged course and the lesions are far more in number than in immuno competent. Its sig-
nificance has increased since it has been observed to be too frequent in patients with AIDS.
The disease involves the skin mostly, however, the mucous membranes are also known
to be involved.
The exact incubation period is not known. The commonest mode of spread is by direct
close contact with the patient. The infection spreads to other parts of the body from the initial
site by auto inoculation. Sexual transmission is also known. It is known to spread via swim-
ming pools.
The typical lesion can be seen anywhere on the skin. Its number vary from a single
lesion to few dozens. The lesion is a translucent or pearly growth few millimeters in diameter,
raised, and circular in shape. The most striking feature is umblication in the center of the
lesion. A caseous material can be expressed with pressure on the lesion. The expressed mate-
rial is infectious. The lesions are also known to shed virus. The common sites are face, abdo-
men, back and groin.
It is a disease of childhood and adolescent; there is no immunity following initial infec-
tion.
The ocular manifestations areSkin of the lids, lid margin, the lesion of lid margin
causes chronic follicular conjunctivitis by the viruses shed from the lid lesion. The infec-
tion can spread from the conjunctiva to cornea in the form of kerato conjunctivitis.
The corneal involvement consists of punctate keratitis, pseudo dendrite, superficial
pannus, and corneal ulcer. A limbal nodule may also develop. There may be notching up of the
lid margin and scaring of the conjunctiva.
There is no specific treatment. The lesion are destroyed mechanically, chemically (tinc-
ture iodine or carbolic) by laser or cryo, only a few at a time. However patient with AIDS
require intra venous cidofovir.
Chlamydiasis
Infection by chlamydia is very common. One of its manifestation trachoma is a major cause of
preventable and treatable cause of blindness in a large part of the world. (See chapter on
conjunctiva and cornea).
Chlamydias are a group of organism that are placed in between smallest bacteria
and largest virus. It has a cell wall similar to bacteria. It possess both RNA and DNA. It is
gram negative, obligate intra cellular organism. The organism is sensitive to many com-
monly used antibacterials but not to anti virals.
The organisms of chlamydia group are antigenetically similar. The three species are C
trachomatis, C pneumoniae and C psittaci. The last one causes a rare form of respiratory
infection that spreads from birds to men. The C pneumoniae is a frequent cause of upper
respirator infection and pneumonia in children.
The organism C trachomatis is exclusively human pathogen and is the cause of
trachoma, sexually transmitted diseases and infection in neonates.
Sexually transmitted Chlamydiasis
Genital infection is caused by C trachomatis, serovars D and K, generally known as geni-
tal trachoma.
Systemic involvements are:
In males, non-gonococcal urethrites. Common age group is late teens and early twenties.
Many of them may be asymptomatic. In males it may cause epididymitis, Reiters syn-
drome, proctitis. In females it causes mucopurulent cervicitis, pelvic inflammation dis-
ease that includes salpingites, endometritis and infertility.
Inclusion body conjunctivitis of new born
Mucopurulent cervicitis in pregnant mother is the cause of neonatal inclusion conjunctivi-
tis. It causes neonatal trachoma in about ten percent children born to infected mothers.
Chlamydial inclusion conjunctivitis in children is replacing gonococci as main cause of oph-
thalmia neonatorum. Many a times it is difficult to differentiate between the two. Inclusion
conjunctivitis develops five to fifteen days after birth while gonococcal infection has shorter
incubation period i.e. one to three days. Gonococci stains grams negative. Intra epithelial in-
clusion cells are seen on Giemsa stain in inclusion conjunctivitis.
Inclusion body conjunctivitis in adults
The second peak of incidence is seen in late teens and early sexually active adults, follow-
ing either direct infection of the eye or as finger to eye infection from infected genitals. Incuba-
tion period is 5-7 days.
The condition presents as mucopurulent conjunctivitis, generally unilateral with en-
larged pre auricular glands. May spread to other eye after 5-7 days or more. The eyes may
stick at night. There may be small area of superficial vascularisation of cornea. The conjuncti-
vitis dies down over month without treatment, without scarring.
Management
Prophylaxis- All new born children should get one drop of 2% AgNO3 in each eye. It should
be remembered that AgNO3 itself can cause self limiting chemical conjunctivitis. The better
way is to instill 2.5% povidon in each eye. In absence of which any broad spectrum antibiotic
drop may be used.
Once ophthalmia neonatorum has developed, it should be treated by standard method.
Adult inclusion body conjunctivitis is treated with 20% sulphacetamide drop three times
a day along with oxytetracyclin eye ointment at bed time. Severe cases may require oral tetra-
cycline 250 mg QID for three weeks or doxycyclin 100 mg once a day for same period.
Lymphogranuloma venerum
It is more of a sexually transmitted disease of genital tract and regional lymph nodes than
ocular disease, however ocular manifestations are possible, which are generally missed due to
lack of suspicion of the condition in children. The children are generally infected following
child abuse and may pass among themselves especially in institutions like hostels or shelters
for abandoned orphans.
The disease is caused by C. trachomatis serovars L1, L2 and L3. The incubation period
varies between 3 days to three weeks.
The systemic involvement comprise of painless ulcer of genital followed by matted
enlarged, inguinallymph nodes that may become tender that suppurate to form a sinus.
Ocular involvement consist of:
Granulomatous conjunctivitis with non suppurative preauricular lymph node, enlargement
sub-mandibular. Sub clavian or neck lymph nodes may be enlarged.
The other mode of presentation is unilateral edema of lid, mucopurulent conjunctivitis,
keratitis, interstitial keratitis, episcleritis, uveitis and retinal hemorrhages.
Diagnosis requires high index of suspicion that should be corroborated with genital
lesion.
Other systemic viral disease with ocular manifestation
Almost, all systemic viral disease have ocular manifestations.
Adeno viruses
Adeno viruses are common cause of upper respiratory tract infection. They are DNA viruses.
Man is the only known host. Incubation period is short i.e. two to ten days. The infection starts
as pharyngitis with high fever and sub mandibular enlargement of lymph nodes. The disease
is mostly self limiting. The infection commonly spreads from swimming pools.
Ocular involvement is mostly acute unilateral mucopurulent conjunctivitis. The
other eye may get involved in three to four days. There may be superficial punctate keratitis.
No specific treatment is required. However cornea should be protected from secondary
bacterial infections which may lead to frank corneal ulcer. The condition does not impart im-
munity.
Influenza
Influenza is caused by orthomyxo virus. There are mainly three types i.e. influenza A, B
and C. The virus causes acute respiratory tract infection that starts in the upper part and
spread to lower respiratory tract, with fever, pain all over the body and weakness. It is pan-
demic with episodes of out breaks mostly in winters. Though the condition is self limiting it
can be fatal if it produces myocarditis, pericarditis or pneumonia. CNS involvement is rare.
Ocular involvement consists of congestion of the conjunctiva, conjunctivitis, retrobulbar
pain, superficial punctate keratitis, dendritic ulcer, dacryoadenitis, anterior uveitis and optic
neuritis. There may be difficulty in near work and diplopia when CNS is involved. There is no
specific treatment.
Epstein-Barr virus
25
This is a herpes virus that causes infectious mononucleosis. It has been blamed to be a
causative factor in Burkitts lymphoma and nasopharyngeal carcinoma.
26
Ocular involvement consists of conjunctivitis, membranous conjunctivitis, superficial
punctate and dendritic ulcer, uveitis, chorioretinitis, optic neuritis, paralysis of internal as
well as external ocular muscle, and dacryoadenitis.
MYCOTIC (FUNGUS) INFECTION
Fungi are micro organisms between bacteria and smallest plant. They belong to the
phylum thallophyta
29
. The fungi are said to be plants without leaves, trunk or twigs. They
do not contain chlorophyll and can not form carbohydrates. They have multiple ramified fila-
ments called hyphae
30,31
. A network of hyphae is called mycelium. The fungi develop on
living organism or on dead organic matter. They are either saprophytes or parasites. The
saprophytic fungi can become pathogenic. Almost all fungi may become facultative pathogen if
the environment is changed in their favour that can be brought about by change in the immu-
nity of the host. An immune compromised body is more likely to develop fungal infection than
an immuno competent. This has assumed an alarming situation due to rapid spread of AIDS.
Prolonged use of antibiotics may eliminate the causative bacteria but encourages a relatively
benign fungus to be invasive. This is worsened by use of steroid both local and systemic. Gen-
eral debility, diabetes, drug abuse, chemotherapy and radiation predispose mycoses.
The fungi can reproduce sexually or asexually. The spores are the reproductive bodies.
There are about 30-35 species of fungi that cause systemic mycoses in humans. All sys-
temic mycoses have ocular involvement. Occasionally fungi may get access to the ocular struc-
ture following trauma that may be accidental or surgical. The pathogenic fungi have been put
in two broad groups:
1. SchizomycetisNocardia and Actinomycosis (Actinomycosis is no more considered
to be a fungus. It has been put between bacteria and higher moulds).
2. Fungi imperfecti (without spores)
Most of the fungi that have ocular manifestation belong to this group they cause:
1. Aspergillosis
2. Blastomycosis
3. Coccidioidomycosis
4. Candidiasis
5. Dermatophytosis
6. Histoplasmosis
7. Mucormycosis
8. Nocardiosis
9. Sporotrichosis
Fungal infection is suspected mostly clinically in predisposed patients.
The predisposing factors areAIDS, prolonged use of antibiotics, steroids, immuno sup-
pressive drugs, patient on radiation patients who had organ transplant, drug abusers, general
debility, diabetics and trauma.
Fungus infections are always chronic in nature. They have long incubation period
except candida. Most of the fungi grow slowly in culture media, can not be developed in labora-
tory animals.
They are identified:
1. Visually, by typical lesions on the skin, and mucous membrane.
2. Observing the characters of mycelium.
3. Microscopically by type of spores, hyphae, septae
4. Culture
5. Histologically. All fungal infection result in giant cell granuloma similar to typical
tubercle.
The fungi can cause immediate hypersensitivity generally by non pathogenic fungus
or delayed hypersensitivity by pathogenic fungi. Thus they can cause contact dermatitis,
angioneurotic edema, reaction similar to serum sickness, sensitisation and
agranulocytosis.
Aspergillosis
Aspergillosis is caused by a saprophyte fungus which is commonly found in soil, and decay-
ing vegetable matters. It is found all over the world but it is more common in tropical and
temperate countries.
The systemic manifestation starts with inhalation of spores leading to aspergillosis
of respiratory tract and para nasal sinuses from where the disease is disseminated to
distant organs like kidney, brain, skin and bones. Ear drum is rarely involved.
Ocular involvement. Common ocular involvement are orbital granuloma and
corneal ulcer, followed by endophthalmitis that can either be exogenous following penetrat-
ing injury or surgery. Less common mode is metastatic endophthalmitis due to embolus of
fungus from distant organ in an emaciated critically ill child.
Other parts of the eye involved can be chronic granulomatous ulcer in the lid and
conjunctiva, canaliculitis and dacryocystitis. Orbital granuloma may spread to lacrimal
gland. Intra ocular involvement are uveites, vitreous abscess, retinitis, retinal vasculitis.
Intra ocular involvement is mostly endogenous.
Blastomycosis
Blastomycosis is caused by a dimorphic fungus balsomycisdermatidis, which is found in two
phasesMycelial phase at lower temperature and Yeast phases at body temperature.
The fungus is found mostly in the soil. The organism is generally inhaled leading to acute
pulmonary infection that may be fatal or pass into chronic phase, which is difficult to eradi-
cate. Skin is the next part to be involved. Other organs are bones, genitourinarytract and
CNS. The common presentation is agranuloma.
Ocular manifestation
32, 33
Granuloma of the skin of the lid is the commonest ocular involvement followed by orbital
granuloma, granulomatousconjunctivitis, keratitis, rarely uveitis, endophthalmitis and
panophthalmitis.
Coccidioidomycosis
34
Coccidioidomycosis is caused by a saprophyte coccidioidesimmitis. It is a dimorphic fungus
that changes with change in environment. In one form it grows as white fluffy mould on
culture media. The second form is a spherule in the host. The organism multiplies in the host
by small endospores in spherules. The mature spherules on maturity rupture releasing
endospores that are transformed to spherules and the cycle is repeated. The infection occurs
following inhalation of airborne spherules.
Systemic involvement is pulmonary infection. The disease may be asymptomatic
and self limiting or may form a cavity in the lung. The fungus may spread to other organs by
dissemination.
The patients with altered immunity are at a risk of relapse and pulmonary as well as
extra pulmonary lesions. Person with AIDS are at greater risk than other immuno compro-
mised. Other systemic lesions are arthritis, erythema multiforme and nodosum.
The ocular involvements are due to hematogenous spread from pulmonary lesion or due
to hypersensitivity.
The ocular manifestation are
35
The hematogenous lesions areProgressive chronic pan uveitis, which is granulomatous
in nature. The uveal lesions are independent of severity of pulmonary lesion. Other intra
ocular lesions areChorioretinitis, vitritis, retinal exudates and haemorrhages.
The cornea may show keratitis. Granuloma of the lid, conjunctiva and lacrimal gland
have been reported.
Candidiasis
Systemic and ocular infection by candida are very common world over. There are more than
hundred species of candida, out of which candida albicans is the most common pathogen.
The candida is a part of the normal flora of mucous membrane, skin, gastro intestinal tract,
genito urinary tract and respiratory tract. It is found in conjunctiva and on lid margin. Candida
grows profusely in keratitis sicca. It is also present in the soil, water and sometimes in food.
It is also a dimorphic fungus that has two phases, the yeast and mycelia. The yeast form is
more common.
Mode of infection is either due to local inoculation via abrasion or endogenous spread
from primary systemic infection.
Systemic involvement is common in neonates, children and malnourished children.
It may be seen as oral thrush of mucous membrane of neonates and infants. Less common is
chronic mucocutaneous candidiasis, that may be associated with multiple endocrinal dys-
function that may result in diabetes, Addisons disease, hypoparathyroidism,
hypothyroidism. Ovarian dysfunction may be associated with pernicious or iron deficiency
anaemia, hepatitis, cystitis, pyelitis, and vaginitis.
The ocular manifestations are:
Keratitis is commonest form of candidiasis that requires prompt treatment. It may
produce pseudo-membranous-conjunctivitis, sometimes purulent conjunctivitis, con-
junctival ulcer or phlycten. The lids may have blepharitis, granulomatous canaliculitis.
The intra ocular involvement may be in the form of frank endophthalmitis either
endogenous or exogenous following penetrating injury, accidental or surgical. Other mode of
intra ocular presentation is milder form of choroiditis, retinitis, chorioretinitis, papillitis,
peri vasculitis, free floating snow balls in vitreous are almost diagnostic.
Dermatophytosis
It is a very common mycotic infection of skin commonly known as ringworm infection. It can
involve nails. According to part of the skin involved it is called tinea capitis (scalp) T corporis
(body), T pedis (athletes foot), T facici (face). No systemic involvement is known.
Ocular involvements. Mostly dermatitis of the lids, blepharitis, madarosis, chronic
conjunctivitis.
Mucormycosis
36, 37, 38
Mucormycosis is a serious infection that may be fatal. It is an opportunistic fungus belong-
ing to species rhizopus, class phycomycetis and order mucorale. The organism is found
all over the world in soil, water, manure, skin. It may contaminate the food with high sugar
content. The non pathogenic strain changes to pathogenic strain in diabetic ketosis, AIDS,
organ transplant, lymphomas and leukaemias.
The fungus is non-septate with branching hyphae that can be seen in culture and
histology. The infection is acquired from the nature but not from person to person contact.
Most probably the organism is inhaled or ingested.
The common systemic involvements arepara nasal sinuses, orbit, brain, respiratory
system, gastro intestinal tract and skin.
The disease is considered to begin in the para nasal sinuses and from there it spread to
adjacent structures like orbit and brain, trickle down the respiratory tract or GI tract. The
main tissue to be involved is vascular, it produce invasive angiopathy that leads to ischemic
or hemorrhagic necrosis.
The typical presentation is similar to bacterial sinusitis with fever, headache, running of nose,
blood discharge from nose, facial pain, swelling of lids, and over the cheek. Common presenta-
tions are involvement of orbit and signs of CNS infection.
Ocular involvement
Commonest form is orbital involvement, most of the time due to extension from para nasal
sinuses and less commonly due to injury to the skin of the lid or retained orbital foreign body.
The orbital involvement starts as unilateral apical pain, headache, diplopia, and dimin-
ished vision. The lids are edematous, the conjunctiva is chemosed. Proptosis is common that
may lead to cavernous sinus thrombosis, superior orbital fissure syndrome and orbital apex
syndrome.
Extension back into cranium leads to clouding of sensation, diminished mental faculty,
cerebro vascular accident and multiple cranial nerve palsy.
The diagnosis is best confirmed by histopathology of tissue removed. The biopsy or exci-
sion is generally not associated with bleeding due to pre existing vasostasis. CT and MRI are
two useful diagnostic methods to demonstrate involvement of sinus and intracranial tissue.
Nocardiosis
Nocardiosis is caused by an organism nocardia. The organism was previously thought to be
fungus along with actinomyctes. Now both are classified as higher bacteria, which are found
world over in the soil and decaying organic substance. It usually affects debilitated and
immuno compromised persons. It is a gram positive, filamentous organism that stain with
Grocott-Gomori stain and periodic acid Schiff stain. It grows on most of the media but
takes four to six weeks to grow. On agar plate the organism produces a star shaped colony
which imparts the name nocardia asteroides to the fungus. They can survive inside the
phagocytes.
The disease is more common in adults than in children. Males outnumber females in a
ratio of 4:1. Systemic involvement is mostly pulmonary as pneumonia, which is sub acute
with anorexia, fever, and loss of weight. There may be pleural pain and hemoptysis. The
extra pulmonary lesions are due to hematogenous spread to many organ including skin.
One of the possible modes of skin involvement is trans-cutaneous inoculation that may pro-
duce cellulitis, lympho cutaneous syndrome and mycetoma.
The ocular manifestations are keratoconjunctivitis, keratitis, anterior uveitis,
chorioretinitis, endophthalmitis, orbital cellulitis and scleritis.
Histoplasmosis
38
Histoplasmosis is a chronic disease caused by fungus histoplasma capsulatum. The organ-
ism is found world over in soil and dropings of bats and birds. However its manifestation
in humans is not universal. It is mostly seen in white Americans. It has rarely been reported
from Asia or Africa. Though it is a disease that is commonly seen in second to fifth decade,
it can infect children as well.
The organism grows with ease on Sabourauds media at room temperature. The fungus
is dimorphic, it shows both yeast as well as hyphae form. The yeast form is seen in various
tissues causing granuloma. The hyphae develops spores at the ends. The disease is spread by
liberated spores in the air.
The commonest mode of infection is by inhaling the small spores that on reaching lung devel-
ops budding forms. The multiplying organisms produce granuloma that caseates to necrose,
may get calcified. The lesions mimics tuberculosis in children. The lesions may heal without
much treatment and get calcified at the hilum. In some cases the infection may continue to
become fatal in children and immuno compromised. The organism can travel to liver and
spleen.
Ocular manifestations
It is not clear if the ocular involvement is really caused by histoplasma at all. The ocular
involvement is called presumed ocular histoplasmosis syndrome (POHS) that is confined
to posterior segment. The anterior segment is unaffected. The ocular involvement is most
probably hematogenous spread from the primary pulmonary focus in childhood which heals to
leave multiple chorio retinal scars, which may be activated in adulthood.
The ocular syndrome consists of peri papillary chorio retinal scar, punched out
inactive chorio retinal scar, hemorrhagic macular lesion. The left eye is involved more
frequently
39
. In two third cases the condition is bilateral. Histoplasmosis is a major cause of
choroidal neovascularisation. Rarely there may be a macular scar. The condition remains
asymptomatic untill the macula is involved. Macular involvement is late but symptomatic.
The common symptoms are metamorphopsia, diminished central vision, and central scotoma.
The patient complains that his vision straight ahead is poor. The treatment of choice is laser
photo coagulation of extra foveal and juxta foveal lesion following fluorescein angiography.
Sporotrichosis
It is a rare fungal disease. The fungus is found all over the world on plants and soil, hence it is
but natural that those who work with soil and plants are most likely to get infected. It may
manifest at any age. The fungus is found in yeast form and grows as budding yeast on media
at 37C.
The commonest route of entry of the organism is via cutaneous wound. In a few days a sub
cutaneous lesion develops which most of the time is a self healing lesion. It generally does not
spread beyond the limb infected initially, though rarely hematogenous spread to lung and
brain are known.
The commonest skin lesion is sub cutaneous painless diffuse nodule at the site of injury.
Other form are lymphangitis, ulcer or nodule formation.
Ocular manifestations are rare but all parts of the eye have been reported to be in-
volved from lid to the retina. Exogenous endophthalmitis occurs following penetrating injury.
Parasitic infection
40, 41, 42
The systemic parasitic infection can either be due to protozoa or metazoa. The metazoa
infection can be due to helminths that can either be cestodes or nematodes. Other organ-
ism that may infect eyes and its adnexa are arthropods or by insect larvae.
The commonest protozoa that involves the eye is toxopalsma. The other protozan in-
fections are relatively uncommon and are caused by entamoebahistolytica, plasmodia,
giardia, Leishmania, and trypanosoma.
The common helminths areTaenia echinococcus, taenia solium, causing
echinococcosis and cysticersosis respectively. The disease coenurosis is caused by taenia
multiceps and taenia glomeratus. The helminth toxocara canis causes toxocariasis. The
organism onchocerea volvulus causes river blindness. Thelaziasis is caused by the thelazia
callipaeda, and thelazia californiensis. Loasis is caused by worm Loa loa also known as
African eye worm. Trichinosis is caused by trichinellaspiralis. Less common ocular mani-
festations are Dracunculosis caused by dracanculus medinensis. Bilharziasis,
sparganosis and ganthostomiasis are still less common in eyes.
Rarely the eyes are infested by acariasislumbricoidis and ankylostoma duodenale.
The arthropods found on ocular adnexa cause pediculosis, phthiriasis and demodicosis.
Infections by insect larvae are called myiasis.
Toxoplasmosis
42, 43, 44, 45, 46, 47
Toxoplasmosis is caused by protozoa toxoplasma gondii that is found universally. The
prevalence of the disease may be as high as 50% in adults who are either asymptomatic or
have some systematic or only ocular manifestation. The domestic cat is the definitive host.
Other mammals are intermediate host that acquire the disease by eating contaminated food or
water by faeces of the cats who excrete the parasite. The organism is obligatory intra
cellular. The parasite has two phases:
1. Homologous host phase and
2. Heterologous host phase that is seen in humans.
Two forms are met with i.e. the pseudo cystic phase and cystic phase.
The disease can either be congenital or acquired.
The congenital toxoplasmosis. The foetus is infected via transplacental route from
already infected mother who is symptomsless but serologically positive. The mother may have
acquired the disease during any phase of pregnancy or has been infected years ago; the disease
has been dormant and has been activated during pregnancy.
The congenital toxoplasmosis
43
is a common cause of abortions and still birth.
The two organs commonly involved are the brain and the eyes that produce triad of three Cs
i.e. convulsion, calcification (intra cranial) and chorioretinitis. Less frequent features
are low birth weight, hepatospleenomegaly, purpuric spots, neonatal hypoxia, raised intra
cranial pressure, and hydrocephalus.
The ocular lesion in congenital toxoplasmosis is generally bilateral central
chorioretinitis that may be mistaken as coloboma of the macula.
There is a rough guideline regarding possibility of intra uterine infection i.e.
1. If the mother has been infected six months prior to the onset of pregnancy the chance
of infection to the foetus is almost nil.
2. If the mother gets infected less than six months before the conception the foetus may
be involved.
3. The chances of infection increases as the duration of infection and conception de-
creases.
4. If the mother is infected during first trimester the chances of transplacental spread
is least but the disease in neonate is most severe.
5. In contrast to this if the mother is infected in last trimester the chances of foetal
infection is most but features in new born are mildest.
Acquired toxoplasmosis
44
Toxoplasmosis is acquired by ingestion of food or water contaminated by faeces of infected
cats. The exact incubation period in not known. The common systemic involvements consist of
skin, lymph glands, lungs, CNS, and heart.
The organism is known to become opportunistic and have a widespread involvement
including eyes.
The ocular involvement consists of chorioretinitis that is located posteriorly as
necrotising retinitis that has yellowish white cotton wool appearance with indistinct margins
and the borders are hyperemic. The lesion may be unicentric or multi-centric and heal by
scaring that has central white area with irregular pigmentation all round. Satellite lesion may
develop little away from the active lesion during its active phase or after it has healed.
The acute stage of acquired toxoplasmosis does not produce any ocular changes. The
changes are reactivation of either congenital lesion or a dormant acquired lesion. Anterior
segment involvement is less common but is possible. Rarely the extra ocular muscles are
involved. Involvement of optic nerve, cataract and glaucoma are common.
A child with a lesion in first three months has nystagmus, squint and very low vision.
An asymptomatic child may have diminished vision and prone to develop reading difficulty.
Entamaeba histolytica
Infection by entamaeba histolytica is very common in tropics. It is estimated that one fifth of
the population either suffers from this parasitosis or is carrier of the disease who are asympto-
matic. Commonest systemic involvement is amaebic dysentery, this is followed by amaebic
liver abscess and rarely cerebral abscess.
Ocular involvements are very few and rare. They do not have any distinguishing
clinical features. There are no specific tests to establish the ocular diagnosis. The diagnosis is
presumptive that is supported by improvement following systemic administration of anti
amaebic chemotherapy.
The commonly reported ocular manifestations are
40
Anterior and posterior uveitis,
that may cause hypopyon, retinal periphlebitis and vitreous haemorrhage.
41
Acanthameba is a free living amaeba found world over. It is mostly found in contami-
nated water. Contamination of water by acanthamoeba has become more frequent in last two
decades. It causes granulomatous amaebic encephalitis. The organism reaches the brain
via blood stream from sinuses, lungs or skin. Persons with AIDS are at higher risk.
Commonest ocular involvement is kerato acanthamoeba.
Malaria
47, 48
It is an infection caused by protozoa plasmodium. There are four types of plasmodia that
are responsible for malaria. They arePlasmodium vivax, P ovale, P malariae and P
falciparum. The last variety causes most serious form of malaria and can be fatal.
The disease is acquired and spread by bite of female anopheles mosquito.
Malaria is found in all parts of the world, but is more common in tropics and subtropical
countries. The incubation period is different in different species of plasmodium.
The systemic features are fever, chill, body ache, abdominal discomfort, all of which
can be mistaken as viral infection. Besides fever that may be periodic with definite cycle of
fever alternating with afebrile period; there is enlargement of spleen, liver is less enlarged.
There may be mild jaundice. There may be rashes all over the body with patechial haem-
orrhage on the skin and other mucous membrane including conjunctiva. Anaemia is very
common.
It may cause dysentery like symptom. Cerebral malaria is caused by p falciparum.
This is a life threatening condition.
Other manifestations are hypoglycemia, lactic acidosis, pulmonary edema and
renal failure.
Ocular manifestations are rare. They can be divided into two groups:
1. Those caused by malaria.
2. Those caused due to toxicity of anti malarial drug.
The common ocular involvements areSub conjunctival patechial haemor-
rhage, superficial corneal ulcer, simulating dendritic ulcer. However patients with
malaria are more prone to develop herpes simplex keratitis both superficial and deep. The
deep keratitis is generally attributed to be interstitial keratitis due to malaria, which clears
following systemic treatment with anti malarial drugs suggesting that plasmodium is capable
of producing interstitial keratitis. Other ocular involvements are retinal haemorrhages and
optic neuritis.
Commonest ocular feature of anti malarial treatment is quinine amblyopia.
49
This is
a dose related condition. The effect varies from patient to patient. It consists of sudden loss of
bilateral central vision with central scotoma, due to optic neuritis that may end in
optic atrophy. Other ocular features of quinine toxicity are retinal pigmentepitheliopathy,
damage to bipolar and ganglion cells.
Giardiasis
Giaradiasis is world wide problem. It is caused by protozoa giardialamblia. It is found in
small intestine of many mammals including humans. It causes endemic and sometime epi-
demic form of enterocollitis and diarrhoea. It is spread by drinking polluted water and ingest-
ing contaminated food.
Ocular involvements are rare but can cause uveitis, keratitis, chorioretinitis and retinal
haemorrhage all of which respond to anti giardial treatment.
40
Leishmaniasis
50
The disease is seen only in tropical countries. It is caused by a protozoa belonging to genus
leishmania. It is a vector borne zoonosis. Humans are incidental host, small mammals are
reservoirs. The disease is spread by bite of sand fly Phlebotomus.
There are three common types of leisheniasis in humans they areKala azar, cutane-
ous leishmaniasis and espundia (nasopharyngeal leishmaniasis).
Out of these Kala-azar is most commonly seen in endemic areas, can be life threatening.
This is caused by L. donovani. Incubation period is long that may range between two to six
months following residence in endemic area. The symptoms are long-lasting-fever with enor-
mous enlargement of spleen with moderate enlargement of liver.
It is more common in HIV infected persons. It is common in first decade of life. The
organism can be transmitted by way of blood transfusion.
Ocular manifestations are rare in Kala-azar. The children may have retinal haem-
orrhage, sub conjunctival haemorrhages. The adults can develop retinal vein throm-
bosis.
Dermal leishmaniasis is associated with ulcers on the lid, episcleral nodules near
the limbus causing vascularisation of cornea. There may be iritis.
Trypansomiasis (Sleeping sickness)
51
This disease is confined to certain parts of Africa and South America. In Africa, it is caused
by trypansoma gambiense transmitted by bite of tsetse fly, and in South American, it is
caused by trypansoma cruzi transmitted by bite of a bug of genera triatoma.
Systemic manifestation of African trypansomiasis - Following bite by the fly the
organism spreads via blood and lymphatics to lymphatic glands, CSF and brain.
After few weeks the patient develops a skin nodule called trypansome chancre. This
is followed by fever that may last for weeks. The organism reaches lymph glands and spleen
resulting into their enlargement. The episode of fever has an intermitted pattern. After years
the patient reaches a stage of sleeping sickness. The patient develops shuffling gate and
finds difficulty to walk. The face becomes mask like with drooping swollen lids. The patient
goes to sleep even during working hours. In late stages, there are convulsions, paralysis and
coma that terminates in death.
Ocular manifestations
Unilateral allergic edema of the lids, enlargement of pre auricular and sub mandibu-
lar glands, bilateral interstitial keratitis and iridocylitis. In terminal months there may
be paralysis of extra ocular muscles and papilledema. The condition is often associated
with onchocerciasis.
South American trypansomiasis is spread by bite of a bug and not fly, is called Chagas
disease. It does not cause somnolence. The cutaneous lesion is called Chagoma, which is a
localised area of edema with enlargement of draining lymph node. It is associated with fever
that lasts for months. There is hepatospleenomegaly. There may be involvement of heart.
Death occurs due to meningo encephalitis.
Ocular manifestation is unilateral Chagoma of the lid, which is known as Romanas
sign.
Taenia echinococcous (Hydatidcyst)
52, 53
Taenia echinococcus is smallest of all tape-worms that infest humans. It has world wide distri-
bution. There are three types of echinococi that are pathogenic. Each type has specific geo-
graphical distribution. Their life cycle is almost identical with slight dissimilar clinical presen-
tation. The various types are echinococcous granulosus, E multi locularis, E oligoarthus
and E vogeli. Out of this granulosus causes ocular disease. The cyst of E granulosus is unilocular
while that of rest are multi locular. Occasionally multi locular cysts can be seen in the orbit.
The dog is the definitive host while many mammals including humans are interme-
diate hosts. Humans can be primary or intermediate host when they directly ingest food
contaminated by dog faeces or secondary intermediate host when they eat contaminated meat
which is either uncooked or under cooked of primary intermediate host like sheep, goat, cow
etc.
Children are infected by swallowing the eggs. Embryos escape from the eggs, penetrate
the intestinal mucosa and reach the portal circulation from there they may travel to any dis-
tant part and develop the cyst. Common sites where the cysts are deposited are liver, lung,
and orbit. Other organs are spleen, kidney and brain.
The cyst is generally single, grow very slowly without any discomfort. It becomes symp-
tomatic only when it is large enough to press over the adjacent structures or when it rupture
and cause anaphylaxis.
A typical echinococcous cyst has two layersAn outer pseudo membrane and an in-
ner germinal layer. The outer layer is surrounded by granulomatous reaction. From the
germinal layer develop daughter and grand daughter cysts. The cyst of echinococcus is called
hydatid cyst. The cyst is filled with clear fluid. Accidental ruptures of cyst gives rise to severe
allergic reaction that may be anaphylactic.
Only 1% of cases of echinococcosis may involve the eyes and its adnexa. Commonest site being
the orbit. Next in frequency is sub retinal space from where it may come to vitreous.
Rarely it can come into the anterior chamber. Orbital involvement is common in older children
and young adults. It is unilateral, generally the cyst is intraconal causing slow, painless,
progressive axial proptosis.
The diagnosis is by exclusion and with high index of suspicion. Commonly used investi-
gations are X Ray orbit that may show enlargement of the orbit. A small cyst may not pro-
duce any change on X ray. Ultrasonography shows cystic growth without internal reflectivity.
CT and MRI give more definite picture.
Sub retinal and intra vitrial cysts are best seen by indirect ophthalmoscope and con-
firmed by CT.
Cysticercosis
54, 55
Cysticercosis is a common parasitic infection all over the world in all ages. The infection is
caused by a cestode, taenia solium which is a pork tape worm. Humans are definitive
host while pork is an intermediate host and harbouring larval form. Sometimes humans
can become intermediate host by ingesting ova of the cestode.
The definitive hosts pass the gravid proglotidis of the adults worm in stool, which is
ingested by intermediate host the hog or sometimes dogs and other mammals. In the intestine
of the intermediate host the embryos are released which pass the intestinal mucosa and reach
the systemic circulation and can be deposited in any organ of the body. Commonest tissues to
be infected are the striate muscles. The embryos get entangled in small vessels and are
converted into larval stage called cysticircus.
Humans get infected by two ways:
1. By eating under cooked pork that is infested by live cysticircus.
2. By eating food contaminated with ova thus becoming an intermediate host. The ovum
ultimately is converted to cysticircus.
Systemic involvement consists of cysticircosis of brain called neuro cysticercosis,
muscles, skin and eyes. Less commonly involved are kidney, liver and spleen.
Intestinal cysticercosis is most of the time asymptomatic but may cause vague pain
in abdomen, diarrhoea, weight loss. Patient may notice segments of tapeworm in stool.
Neuro cysticercosis is most important and dangerous part of the disease. The clinical
manifestation depends on size, location and inflammatory reaction caused by the cyst, which
includes - Focal seizures and neurological deficits, generalised convulsion, meningi-
tis, headache, vomiting, and raised intracranial tension. Extra ocular muscle palsy
and papilledema are some other features. The presence of intracranial lesion is confirmed by
CT and MRI.
Ocular involvements are common. Almost half of the patients with systemic cysticercosis have
ocular involvement. All most all parts of the eye, its adnexa and orbit except the lens may be
involved.
Involvement of left eye in more frequent than right eye
54,55
. The cyst may develop in
the orbit and migrate anteriorly along the recti muscles and present as sub conjunctival
cyst. There may be more than one cyst in the conjunctiva, occasionally the cyst may extrude
automatically, may cause proptosis, sub retinal cysts may pass into vitreous, small uveal
cysts may pass in AC, involvement of optic nerve is possible. Other ocular involvement is
severe intra ocular inflamation following death of the organism inside the eye during anti
helmenthic chemotherapy or rupture of the cyst during surgical treatment.
Toxocariasis
47, 56, 57
Infection by nematode toxocara is called toxocariasis. It is a zoonotic disease that spreads
from dog to the children. Now it is established that toxocariasis, of cats do not infect chil-
dren. Puppies are more infective than adult dogs.
58
Children are more prone to develop
toxocariasis than adults. The age ranges between two years to fifteen years mean being
seven years. Due some ill understood cause the disease is more common in boys. Ocular
lesions are more common on the left side. It is more common in children who eat dirt habitu-
ally.
The puppies get infected via transplacental route, during suckling or direct ingesting
food infected by eggs. Children get infected by swallowing soil contaminated by eggs. In chil-
dren the ingested eggs hatch to form larva that penetrate the gut wall to reach systemic circu-
lation, that carries them to distant organs like liver lung, brain and eyes. The passage of the
organism from gut to other organs is called visceral larval migrans, in contrast to cutaneous
larval migrans that is caused by hookworm of dogs. Besides visceral larval migrans the para-
site can cause abdominal pain, anaemia and eosinophilia that may be as high as 90%.
The ocular toxocariasis is less common manifestation than systemic.
Ocular manifestation consist ofPosterior pole granuloma, that when large gives a white
reflex in pupillary area and mistaken as retinoblastoma, peripheral granuloma,
parsplanitis, diffuse chorioretinitis, exudative retinal detachment and chronic
endophthalmitis.
Onchocerciasis
59, 60
The disease onchocerciasis is a major cause of preventable and treatable infectious cause of
blindness in Africa and South America. The disease is caused by nematode onchocerca
volvulus and onchocerea caecutiens. The former is found in central Africa while the
latter is found in South America. As the maximum number of cases are found near the riv-
ers, the condition is called river blindness. One of the main complications of the disease is
blindness hence the worm is also called blinding worm. Man is the definitive host in
whom the micro filaria develops while female black fly-simulium is the intermediate host.
It is only the female fly that is responsible for spread of the disease.
The adult larva develops in the mouth part of the fly. The disease spreads to infected
host to non infected host, multiple infections are known.
The systemic involvement is mostly cutaneous, less common is involvement of the
lymph nodes. Other systems are not known to be involved.
The cutaneous involvement begins with intensive itching along with papular rash.
Long term infection leads to hypo-pigmentation of the skin, wrinkled and loose skin. Some-
times there may be hyper pigmentation. Onchocercomata is a subcutaneous nodule, which
may be palpable, and visible. In African countries they are seen over the scalp. Mild to moder-
ate lympadenopathy is common generally in the inguinal and femoral area. Hydocele is com-
mon. The enlarged nodes may be large enough to hang down.
Ocular involvement
Almost all parts of the eyes except the lens is known to be infected by onchocerciasis. It in-
cludes:
1. Edema of lids that is transient. Some times mild proptosis is seen.
2. Conjunctiva becomes thickened and pigmented though free micro filarae are not
seen on the conjunctiva, they are demonstrable in fresh biopsy even without stain-
ing.
Involvement of the cornea is most important aspect of onchocerciasis and the main
cause of blindness. It is present in all most all cases and both the cornae are symmetrically
involved.
The corneal involvements are:
Nummular opacity, presence of dead micro filaria, frosted glass appearance of cornea, superfi-
cial vascularisation, deposition of calcium, folds in the Descemets membrane. There may be
punctate keratitis or sclerosing keratitis, KPs. Anterior chamber may show number of free
floating micro filaria, which do not elicit any reaction. The dead organisms are accumulated at
the bottom of AC.
Uvea may show iritis, distorted pupil, inverted pear pupil, spongy iris called pumic
stone iris. Iridocylitis and chorioretinitis is common.
Optic neuritis and atrophy are also seen though the lens is not invaded by microfilaria.
Complicated cataract is secondary to uveitis.
Microfilaria may be seen floating in the vitreous.
Thelaziasis
60, 61
Thelazia is a small nematode, life cycle of which is not well understood as most of the cases
have been reported from South East Asia and far east, it is also called oriental worm. It
commonly lives in the lacrimal glands and lacrimal passages of many mammals, common
being dogs, and horses. There are two species of thelazia i.e. thelazia calipaeda and thelazia
californiensis. Cockroaches are suspected to be intermediate host. It is presumed that
the disease is spread by common house fly.
Systemic infection has not been reported.
It is presumed that the flies deposit the eggs of the nematode in the conjunctiva. The
source of these eggs is not well understood. The larvae hatch from the eggs and migrate to the
lids and lacrimal sac. The larvae may produce conjunctival nodule. The larvae may penetrate
the cornea or the sclera to become intra ocular, causing uveitis. The dead worm like most of
the nematodes can cause allergic reaction. The worm may be seen as free floating organism in
the AC.
Loasis
47
The disease loasis is caused by nematode Loa-Loa or African eye worm, Central and west-
ern African countries form the endemic area. The microfilaria may live for many years in the
subcutaneous tissue of an asymptomatic person who have lived in the endemic area. The life
cycle is not well understood. The disease is spread from host to host by bite of the mangrove
fly of species chrysops. It is the female fly that bites and spreads the disease. The microfilaria
are diurnal, they are seen in the peripheral blood between 12.00 noon and 2.00 noon. The
exact incubation period in not known. The disease may be seen concurrently with onchocersiasis.
The disease is caused by adult worm that creeps under the skin and produce localised nodule
called Calabar swelling.
Systemic involvement is rare but may cause nephropathy, encephalopathy and
cardiomyopathy. Eosinophilia is constant and the count may go as high as 50-60%.
The ocular lesions consist of Calabar swelling of the lids. The adultworm may be seen
under the conjunctiva.
Trichinosis
Trichinosis is a disease caused by larva of nematode trichinella spriralis and trichnatis
pseudo spiralis. Besides these two, there are many more types of trichinal nematode that
are found in many mammals. The parasite is found all over the world but the disease is mostly
confined to Western Hemisphere.
The disease is spread by eating under cooked food, infected pork or beef. The larva has
predilection for striated muscles. The systemic involvement consists of diarrhoea, abdominal
pain, nausea, vomiting. The cutaneous involvement includes migration of larva, allergic reac-
tion, maculo papular rash.
Myocarditis and tachyarrthymias, and encephalopathy are rare. Muscle involvement is
common.
Ocular involvements are edema of peri orbital tissue, orbicularis and extra ocular
muscles palsy, sub conjunctival haemorrhages, retinal haemorrhages.
Dracanculosis
Dracanculosis is caused by dracanculus medinensis which is the largest thread worm. The
worm is found mostly in tropical countries. A crustacean - cyclope is the intermediate host.
The cyclope is found in water. Humans acquire the disease by drinking water contaminated
by cyclope. The cyclope contains the larvae of the parasite. The larvae penetrate the wall of the
stomach and intestine and mature. The male dies, the female lives for years and migrate
under the skin mostly the lower extremities or any part that is frequently soaked in water like
back and head of water carriers. The gravid larva migrates under the skin, which is habitually
wet and form a blister. The blister ruptures, releasing large number of larvae in stagnant
water and ingested by cyclope and the cycle goes on.
There are no systemic involvement.
The ocular involvement consists of local edema, pain and blister in the lid that may
rupture and release larvae. An orbital larva may cause mild proptosis. The worm may form a
blister on the skin surface to release the larvae. Sometimes the organism may die in the orbit
and get calcified even without any symptom.
Ocular myiasis
61
Ocular myiasis is caused due to deposition of eggs of some flies that generally breed in the
nasal cavity and sinuses of sheep. In persons with poor hygiene and debility of any age may
attract the offending flies that lay eggs in the conjunctiva, nose or any open and non-healing
wound.
There are no systemic involvement.
Ocular myasis can either be myiasis externa, which is more common and caused by
first stage larvae or myiasis interna, which is rarer and caused by second stage larvae.
The ocular myiasis externa consists of conjunctival irritation, itching, edema,
photophobia. A sticky white membrane is formed in the fornix and many larvae can be seen
under this. Sometimes they may be seen in lacrimal sac or canaliculi. The myiasis externa
does not erode the tissue. In myiasis interna, the maggots may burrow in the anterior chamber
or the vitreous through sclera leading to sever inflammatory reaction.
Management consist of picking up the maggots by forceps after they have been immobi-
lised by xylocaine.
REFERENCE8
1. Purvin V. A.Bacterial disease in Walsh and Hoyts clinical neuro ophthalmol-
ogy. Fifth edition, fourth volume, P 4109-4117, Williams and Wilkins, Baltimore, 1990.
2. Chan G. H.Systemic bacterial disease in Pediatric ophthalmology. vol. II, Second
edition, edited by Harley R.D., P 924-929, WB Saunders Company, Philadelphia 1983.
3. Bellows J.G.Acute general bacterial infections in Modern ophthalmology, Vol. 2, P
95-115, Edited by Sorsby A., Butterworth, London 1963.
4. Scheie H.G. and Albert D.M.Ocular manifestation of systemic infection in Text book
of ophthalmology. Ninth edition, P 366-399, WB Saunders Company, Philadelphia
1977.
5. Richardson J.P., Kinght A. L.Management and prevention of tetanus. Jr. Emerg. Med.,
11: 737-42, 1993.
6. Isada, C. M., Meisler D.M.Gonococcal ocular disease in Current ocular therapy.
Fifth edition, P. 28-31, Edited by Fraunfelder F. T. and Roy, F.H. WB Saunders Com-
pany Philadelphia, 2000.
7. Raviglioni M.C. and OBrien.Tuberculosis in Harrisons principles of internal medi-
cine. Fourteenth edition, Vol. I, p. 1004-1013, Edited by Fauci AS and Longo D.L.,
McGraw Hill New York, 1998.
8. Whitcup S.M., Raizman, M.B.Spirochetal infection of the eye in Principles and prac-
tice of ophthalmology. Vol. 5, P. 3078-3092. Edited by Albert D.M. and Jakobies, WB
Saunders company, Philadelphia 1994.
9. Margo C.E., Hamed L.M.Ocular syphilis, surv. Ophth. 37: 203-220, 1992.
10. Keusch G.T.Salmonellosis in Harrisons Principles of internal medicine. 14th
edition, Vol. I, P. 950-956, Edited by Fauci A.S. and Longo D.L., McGraw Hill, New
York, 1998.
11. Thygeson P.Viral and rickettsial disease in Modern ophthalmology. Vol. II, First
edition, P 180-210, Edited by Sorsby A. Butterworth. London 1963.
12. Duke Elder S.Viral kerato conjunctivitis in System of ophthalmology. Vol. VIII,
Part I, P. 249-295, Henry Kimpton, London 1965.
13. Hiles D.A. and Cignetti F.E.Ssystemic viral disease in Pediatric ophthalmology.
Vol. II, Second Edition, Edited by Harley R.D., P. 911-922, WB Saunders Company,
Philadelphia 1983.
14. Vastine D.Infections of the ocular adnexa and cornea in Principle and practice of
ophthalmology. Vol. I, First Indian edition, P 316-349. Edited by Peyman G.A. Sander
D.R. and Goldberg M.F. Jay Pee Brothers, New Delhi 1987.
15. Mukherjee, P.K., Jain P.C. and Mishra, R.K.Exanthemata : A causative Factor of
chronic dacryocystitis in children. Jr. All Ind. Oph. Soc. 17: 27-30 1969.
16. World heath organisation (WHO)The child measles, and the eyes. Geneva WHO 1993.
17. Duker S.J.Acquired immune deficiency syndrome in Current ocular therapy. Edi-
tion fifth, P 5-7, Edited by Fraunfelder FT and Roy F.H. WB Saunders Company, Phila-
delphia 2000.
18. Fauci A.S., Longo D.L.The human retro virus in Harrisons principles of internal
medicine. Fourteenth edition, Vol. I, P. 1105-1111, Edited by Fauci A.S., McGraw Hill,
New York 1998.
19. Whitley R.J.Varicella zoster virus infection in Harrisons principles of internal
medicine. Fourteenth edition, Vol. I, P.1086-1088, McGraw Hill, New York 1998.
20. Gittinger J.W.Herpes zoster ophthalmicus in Manual of clinical problems in oph-
thalmology. First edition, p 5-7, Edited by Gittinger J.W. and Asdourian G.K., Little
Brown and Co., Boston 1998.
21. Marsh R.J.Varicella and Herpes zoster in Current ocular therapy. Edition fifth, P.
83-84. Edited by Fraunfelder FT and Roy FH. W.B. Saunders Company Philadelphia,
2000.
22. Vastine D.Molluscum contagiosum in Principle and practice of ophthalmology.
Vol. I, first Indian Edition, P. 341-342, Edited by Peyman G.A., Sander D.R. and Goldberg
M.F. Jay Pee Brothers, New Delhi 1987.
23. Tabbara K.F., Hyndiuk R.A.Infection of the eye. Second edition, Little Brown and
Co., Boston 1996.
24. Malouf D.J., Oates R.K.Herpes simplex virus infection in the neonates. J. Paed. Child
health, 31: 332-335, 1995.
25. Matoba A.Y.Ocular diseases associated with Epstein-Barr virus infection. Surv. Ophth,
35, 145-150, 1980.
26. Deborah Pavan LangstonEpstein-Barr virus in Manual of ocular diagnosis and
therapy. Third edition, P. 195. Edited by Deborah Pavan Langston, Little Brown and
Co., 1991.
27. Dutta L.C., Dutta N.K. and Barua C.K.Ocular infections of AIDS in Modern oph-
thalmology. Vol. II, Second edition, P. 1042-1048, Edited by Dutta L.C., Jay Pee Broth-
28. Rao N.A.Acquired immuno deficiency Syndrome and its ocular complication. Ind. Jr.
Oph., 42: 51-63, 1994.
29. Birge, H.L.Mycotic infection in Modern ophthalmology. Vol. II, First edition, Ed-
ited by Sorsby A., P. 253-271, Butterworth London, 1963.
30. Rezende, C.D.Ocular mycosis. XIX, Con Oph., Vol. 2, P. 299, New Delhi, 1962.
31. Ahmed, E.Mycotic infections in A Text book of ophthalmology. First edition, P. 72-
73, Oxford University Press, Calcutta, 1993.
32. Bartley G.B.Blastomycosis of the eye lid. Ophthalmology 102 : 2020-2023, 1995.
33. Barr, C.C. and Gamel J.W. : Blastomycosis of eye lid, Arch. Oph., 104: 96-97, 1986.
34. Bennett., J.E.Coccidiodimycosis in Harrisons principles of internal medicine.
Fourteenth edition, Vol. I, P 1151-1152, McGraw Hills, New York, 1998.
35. Rodenbiker H.T., Ganley, J.P.Ocular coccidiodimycosis. Surv., Oph., 24: 263-290, 1980.
36. Harrisons A.R., WirtschaflerMucormycosis in Current ocular therapy. Fifth edi-
tion, P. 48-50, Edited by Fraunfelder FT and Roy FH, W.B. Saunders Company, Phila-
delphia 2000.
37. Bennett J.E.Mucormycosis in Harrisons principles of internal medicine. Four-
teenth edition, Vol. I, P. 1158, McGraw Hills, New York, 1998.
38. Harley R.D.Mycotic infections in Pediadric ophthalmology. Vol. II, Edition 2, P.
930-931., Edited by Harley R.D. W.B. Saunders Company Philadelphia 1983.
39. Deborah. Pavan Langston.The fungal disease histoplasmosis in Manual of ocular
diagnosis and therapy. Third edition, P. 199-200, Edited by Deborah Pavan Langston,
Little Brown 1991.
40. Mukherjee R.Ocular manifestation of tropical disease in Modern ophthalmology,
Vol. 2, second edition P. 1037-1041. Edited by Dutta L.C., Jay Pee Brothers, New Delhi,
2000.
41. Bhaduri, BNEye complications in Protozoal parasitic disease of the tropics. XIX,
Concillum, ophthalmologium Acta I, P. 79, New Delhi, 1962.
42. Palestine A.G.Toxoplasmosis in Current ocular therapy. Fifth edition, P. 101-102.
Edited by Fraunfelder FT and Roy F-H. W.B. Saunders Company, Philadelphia 2000.
43. Hogan M.J.Congenital toxoplasmosis in Modern ophthalmology. Vol. II, First edi-
tion, P. 19-22, Edited by Sorsby A. Butterworth London 1963.
44. Hogan M.J.Acquired toxoplasmosis in Modern ophthalmology. Vol. 2, First edi-
tion, P. 212-213, Edited by Sorsby A. Butterworth London 1963.
45. Perkins S.Ocular toxoplasmosis. B.J.O., 51: 1, 1973.
46. Rathinam S.R. and Namperumalsamy P.Toxoplasmosis in Clinical practice in oph-
thalmology. First edition, P. 268-269, Edited by Saxena S. Jay Pee Brothers, New
Delhi, 2003.
47. Harley R.D.Ocular parasitosis in Pediatric ophthalmology. Vol. 2, Second edition,
P. 931-937. Edited by Harley R.D., W.B. Saunders Company, Philadelphia 1983.
48. Biswas J., Fogla R.Ocular malaria. Ophthalmology. 103:, 1471. 1996.
49. Kanski J.J.Toxic maculopathies in Clinical ophthalmology. Second edition, P. 365,
50. Berman J.D.Human Leishmaniasis : Clinical diagnosis and chemo therapeutic devel-
opments in last 10 years. Clin. Inf. Dis. 24: 684. 1997.
51. Somerset E.J.Trypansomiasis in Modern ophthalmology. Vol. 2, First edition, P.
292-294, Edited by Sorsby A. Butterworth London, 1963.
52. Sun T. Echinococcosis in Pathology and clinical findings of parasitic disease. P.
287-295, Edited by Sun T., Masson, New York 1982.
53. Dutta L.C.Hydatid cyst in Ophthalmology principles and practice. First Edition,
P. 369-370, Current Book International, Calcutta 1995.
54. Malik S.R.K., Gupta A.K., Chaudhri S.K. Am J. Oph. 66: 1168, 1968.
55. Mukherjee P.K., Agrawal S. - Sub conjunctival twin cysticercosis. Ind. Jr. Oph. 23: 28-
29, 1975.
56. Gille Spel S.H.The Spectrum of ocular toxocariasis. Eye: 7: 415,1993.
57. Shields J.A.Ocular toxocariasis : A review. Surv. Oph. 28: 361-381, 1984.
58. Liv. L.X., Weller P.F.Visceral and ocular larval migrans in Harrisons Priciple and
practice of internal medicine. Fourteenth edition, Vol. I, P. 1206-1207, McGraw Hill,
New York 1997.
59. World health organisationOnchocerciasis and its control. Report of a WHO expert
committee on onchocerciasis control. WHO Tech Report Series 852, 1995.
60. Mukherjee P.K., Verma S., Agrawal S.Intra ocular thelazia. A case report. Ind. Jr.
oph. 25: 41-42, 1978.
61. Mukherjee P.Thelaziasis in Current ocular therapy. Fifth edition, P. 100-101, Ed-
ited by Fraunfelder FT, Roy F.H. W.B. Saunders Company, Philadelphia 2000.
62. Ridley H.Ocular myiasis or ophthalmo myasis in Modern ophthalmology. Vol. 2,
first edition, P. 251-252, Butterworth London 1963.
778
1@AN
A
A and V pattern, 615, 621, 667
A-V pattern, 621
A-V syndrome, 669
A.D.D., 350
A.M.R.D., 382
Abducens nerve, 604
Abducent nerve, 511, 597, 600, 601,
604, 677
Abduction, 595, 597, 599, 618
Abductors, 593, 597, 598
Aberrant lacrimal gland, 94, 95
Aberrant regeneration of third nerve,
509, 606, 608
Abiotrophic, 434, 576
Ablate, 211
Ablepharon, 41
Abnormal colour vision, 441
Abnormal fluorescence, 415
Abnormal head posture, 587
Abnormal retinal correspondence,
569, 639
Abnormal tear film status, 173, 374,
682
Abnormality of light reflex, 506
Abnormality of the orbit, 644
Abortion, 740, 765
Abrahams lens, 382, 383
Abrasion, 23
Abscess of lids, 53, 92
Absence of lacrimal gland, 104
Absolute glaucoma, 116, 721
Absolute hypermetropic, 305, 556
AC depth, 417, 701
AC IOL, 357
AC/A, 653
Ac/A ratio, 667
Acanthameba, 766
Acanthamebial drugs, 209
Acanthamoebae keratitis, 208
Acanthocytosis, 440
Acariasislumbricoidis, 765
Accessory elevator, 7, 67
Accessory glands, 88
Accessory glands of Krause and
Wolfring, 103
Accessory Lacrimal glands, 7, 86, 87,
98, 112, 113
Accommodation, 15, 70, 292, 502, 503,
546, 548, 557, 586, 601, 608, 668
Accommodation and convergence in-
sufficiency, 570, 571
Accommodation in hypermetropia,
556
Accommodative esotropia, 22, 654,
658, 661
Accommodative esotropia with high
Ac/A, 660
Accommodative squint, 558, 656
Acetazolamide, 148, 184, 193, 255,
351, 370, 377, 555
Acetic acid, 143
Acetyl choline, 75
Acetyl cysteine, 143
Acetylcholine, 502, 505
Achromatopsia, 17, 419, 441, 443,
538, 578, 579, 628, 632, 634
Acid alkali burn, 221
Acid mucopoly saccharides, 443, 448,
477
Acid-fast, 731
Acidosis, 377
Acinetobacter, 129, 726
Acnerosacea, 142
Acoustic neuroma, 55, 56, 620
Acquired, 73, 121, 229, 258, 259, 611
Acquired anisometropia, 567
Acquired esotropia, 658
Acquired immuno deficiency syn-
drome (AIDS), 267, 749
Acquired ptosis, 68
Acquired syphilitic retinitis, 430
Acquired unilateral nystagmus, 628,
630
Acrocephalo syndactylia, 681
Acronym, 381
Acrylic, 562
Actinic rays, 248
Actinomycosis, 126, 482, 708, 726, 759
Action of acetazolamide, 377
Active immunisation, 202
Active uveitis, 188
Actively motile, 743
Acuta glaucoma, 116
Acute allergic conjunctivitis, 24, 91,
139
Acute dacryocystites, 92, 786
Acute disseminated
encephalomyelitis, 527, 529
Acute esotropia, 663
Acute hydrops, 169
Acute iridocyclitis, 206, 271, 576
Acute iritis, 262
Acute peri orbititis, 702
Acute retinal necrosis, 267, 432
Acute rheumatic fever, 739
Acute rise of intraocular pressure, 525
Acute serpiginous ulcer, 191
Acute sinusitis, 529, 702
Acyclovir, 197, 198, 199, 202
Adaptation, 639
Addisons disease, 117, 762
Adduction, 72, 595, 596, 597, 598, 605
Adductors, 597
Adeno virus, 131, 203, 758
Adenoma sebaceum, 59, 87
Adenosine triphosphate, 160, 197
Adherent leucoma, 166, 184, 186, 188
Adies pupil, 505, 511
Adrenal gland, 530
Adrenaline, 117, 512, 708
Adrenergic, 374, 505
Adult, 2, 238
Advanced glaucoma, 584
Advantage of paracentesis, 184
Advantages of binocular single vision
are, 638
Advantages of direct gonioscopy, 346
Advantages of NdYAG, 383
Aerobic, 726, 727, 731, 738
Aerobic metabolism, 293
Afferent pupil, 715
Afferent pupillary defect, 510, 517,
520, 523, 530, 533, 536, 540,
541, 517
Afferent visual, 510
Afocal lens, 413
African eye worm, 765
After cataract, 323
After image test, 640, 653
Aganglionic megalocon, 683
Age related macular degeneration,
416
AgNO
3
, 128, 757
INDEX 779
C-8\C:\N-AGE\Index.PM5
Agnosia, 574, 581
Agonist, 594
Agranulocytosis, 760
Agraphia, 580
AIDS, 174, 253, 430, 749, 754, 756,
759, 760, 761, 762, 766
Air corneal interface, 342
Air puff non contact tonometer, 341
Alacrima, 94, 95
Albendazol, 147, 153, 490
Albinism, 16, 17, 18, 27, 227, 399, 400,
410, 415, 423, 576, 632, 656, 683
Albinism associated with systemic
syndromes, 401
Albinos, 395
Albinotic fundus, 237
Albipuntatus, 439, 574
Albumin, 103
Albunoid, 293
Alcaine blue, 105
Alchoholic solution of iodine, 181, 199
Alexanders law, 629
Alexia, 580
Alkali burn, 214
Alkaline, 141
Alkaloid, 373
Allergen, 140, 144
Allergic blepharo conjunctivitis, 103,
116, 374
Allergic chemosis, 118
Allergic conjunctivitis both endog-
enous, 23, 97, 103, 122, 142, 147
Allergic conjunctivitis in children, 138
Allergic dermatitis, 180
Allergy, 13, 205
Alopecia, 274, 275
Alpha agonist, 371, 372
Alpha agonist, 252, 255, 351, 355, 356,
360, 361, 362, 365, 368, 369, 371,
372
Alports syndrome, 315
Altered aqueous dynamics in chronic
uveitis, 251
Alternate, 649
Alternate cover test, 639, 646
Alternate day esotropia, 620, 623
Alternate hyper phoria, 614, 615
Alternate occlusion, 656
Alternate squint, 650
Alternate sursum-duction, 614
Aluelar, 707
Amacrine cells, 393, 396, 399
Amaebic dysentery, 766
Amaebic liver abscess, 766
Amaeboid ulcer, 141, 195, 755
Amaurosis, 442, 507, 520, 574
Ambloyopia screening, 30
Amblyopia, 8, 28, 39, 41, 56, 68, 70,
79, 147, 168, 172, 178, 185, 186,
189, 236, 239, 246, 248, 260, 270,
297, 303, 315, 319, 323, 324, 327,
351, 371, 402, 404, 432, 444, 488,
513, 517, 549, 574, 585, 586, 587,
590 609, 615, 617, 618, 621, 623,
625, 639, 653, 656, 659, 662, 664,
665, 666, 668, 670, 681, 682, 710,
712
Amelanotic melanoma, 57
Amenestic colour aphasia, 580
Ametropic amblyopia, 546, 586
Amikacin, 730, 743
Aminoaciduria, 315
Aminocaproic acid, 364
Aminoglycocides, 184, 726, 740, 743
Amoeboid, 195
Amoxycillin, 728, 743, 744
Amphotericin, 205
Ampicillin, 126, 740, 742, 743, 744
Amplitude of accommodation, 509,
628, 629
Ampula, 231
Amsler grid, 577
Anaemia, 422, 425, 492, 712, 767
Anaerobic, 293, 726, 727, 729
Anaesthesia dolorosa, 171, 202, 607,
753
Anaesthetic agent, 173
Anaesthetic cornea, 177
Analog of guanosine, 197
Anastomosis of retinal vessels, 428
Anatomy of cornea, 159
Anatomy of the nerves involved in
ocular movement, 601
Anatomy of the structures related to
glaucoma, 334
Anatomy of the vitreous, 456
Andriamycin, 708
Anemia thrombocytopenia, 740
Aneurysm, 409, 415, 608, 612, 619
Aneurysm of intra cavernous carotid,
607
Angel kappa, 652
Angio matosis retinae, 55
Angio neurotic oedema, 117
Angioid streak, 314, 415, 435, 723
Angioma, 283, 409
Angiomatosis retina, 57, 406, 415,
422, 424, 426, 427, 428, 454, 462,
477, 489
Angioneurotic edema, 44, 760
Angiopathy, 537
Angle closure glaucoma, 97, 162, 251,
357, 370
Angle kappa, 642, 652, 666
Angle of anomaly, 640, 653
Angle of anterior chamber, 335, 346
Angle of squint, 587, 651
Angle of the anterior chamber, 335,
336
Angle recess, 342
Angular artery, 89
Angular conjunctivitis, 140, 735
Angular intra orbital, 89
Angular veins, 89, 336
Anhydrosis, 74
Animal dander, 140
Aniridia, 12, 17, 19, 163, 234, 306,
310, 314, 352, 401, 517, 549, 560,
576
Aniseikonia, 551, 561, 562, 563, 567,
569
Anisocoria, 19, 229, 234, 502
Anisometropia, 60, 70, 271, 559, 561,
570, 585, 587, 665, 668
Anisometropic amblyopia, 586, 590,
663
Ankyloblepharon, 7, 18, 39, 121
Ankyloblepharon filiformis adnatum,
39
Ankyloplepharon, 39
Ankylosing spondylitis, 240, 248, 271
Ankylostoma duodenale, 765
Annular posterior synechia, 244, 250
Annular scotoma, 582
Annulus of zinn, 511, 677
Anomalies, 18, 234
Anomalies of structures arising from
outer layer o, 400, 402
Anomalous retinal correspondence,
639, 659
Anomalous trichromatism, 578
Anopheles mosquito, 767
Anophthalmos, 8, 12, 16, 18, 68, 94,
678, 696
Anopia, 578
Anorexia, 747
Antagonist muscles, 594
Ante cubital vein, 414
Anterior axial cataract, 227, 256, 298
Anterior capsular cataract, 244, 295,
296
Anterior capsular opacities, 244, 250,
323
Anterior cavernous sinus syndrome,
607
Anterior cerebral artery, 512
Anterior chamber, 3, 5, 15, 19, 153,
154, 162, 163, 173, 175, 177, 184,
243, 302, 335, 337, 451, 501, 563,
Anterior chiasma syndrome, 508
Anterior choroiditis, 206
Anterior ciliary arteries, 114, 116,
232, 277, 596, 597
Anterior ciliary veins, 336
Anterior ciliary vessels, 232, 242, 720
Anterior cranial fossa, 673
Anterior dislocation, 306
Anterior embryotoxon, 162
Anterior inferior cerebellar artery,
604, 619
Anterior junctional lesion, 508
Anterior lacrimal crest, 89, 674
Anterior lens capsule, 339
Anterior lenticonous, 302
Anterior limbal border, 112, 336
Anterior limbal zone, 336
Anterior limiting layer, 228
Anterior lip of the optic cup, 233
Anterior medullary velum, 603, 604,
612
Anterior membrane, 210
Anterior mesoderm, 3
Anterior microphthalmia, 162
Anterior plus, 486
Anterior polar cataract, 127, 163, 175,
188, 189, 291, 295, 296, 734
Anterior scleral foramen, 159, 720
Anterior segment ischaemia, 215, 249
Anterior segment ischeamia and
uveitis, 240, 277
Anterior staphyloma, 62, 169, 174,
175, 186, 751
Anterior stromal puncture, 211
Anterior synechea, 57
Anterior uveitis, 24, 116, 174, 180,
181, 196, 239, 201, 242, 268, 269,
271, 274, 348, 361, 376, 741, 755,
759
Anterior vasculosa lentis, 293
Anterior visual pathway, 272, 278,
507, 532, 462
Anterior vitrectomy, 274, 323
Anthrax bacillus, 726
Anti histamine, 328
Anti inflammatory agent, 255
Anti metabolites, 257
Anti metropia, 568
Anti microbial drugs, 179
Anti mongoloid obliquity, 41
Anti mongoloid slant, 670, 681
Anti retinal antibodies, 273
Anti syphilitic treatment, 207
Anti toxin, 730
Anti toxoplasma chemotherapy, 432
Anti toxoplasmo anti bodies, 431
Anti tubercular treatment, 733
Anti vascular chemical factors, 169
Anti viral drugs, 131, 196
Anti-toxoplasmosis chemotherapy,
260
Antibacterial vaccines, 147, 528
Antibodies, 268
Antibody to anti actycholine, 77
Anticholine esterase drugs, 75, 77,
505
Anticholinergic, 103
Antigen antibody reaction, 367
Antiglaucoma filtering surgery, 357
Antihistamine, 103, 139, 140, 142,
362
Antimongoloid slant, 683
Antinuclear, 268
Antioxidants, 198
Antisepsis, 110
Antitoxin, 125, 728
Antitoxoplasma antibody, 260
Antiviral drugs, 49
Antiviral ointment, 52
Antiviral vaccine, 528
Aortic insufficiency, 308
Aorticroot, 308
Aperts syndrome, 678, 681, 695
Aperture, 382
Apex of the orbit, 511, 596, 673
Aphakia, 28, 270, 321, 335, 354, 412,
452, 547, 556, 559, 563, 665, 668
Aphakic correction, 8, 304
Aphthous ulcer, 277
Apical pain, 763
Aplasia, 621
Aplastic anaemia, 119
Apocrine sweat glands, 38
Aponeurosis of levator, 36, 87, 674
Apoptosis, 434
Applanation tonometry, 176, 341
Applied anatomy of the retina, 393
Apraclonidine, 372, 375
Aqueduct stenosis, 509, 521, 603
Aqueous chamber, 98, 160, 216, 230,
290, 335, 501
Aqueous deficiency, 103
Aqueous drainage device, 350, 355,
356
Aqueous flare, 241, 243, 263, 283
Aqueous formation, 375
Aqueous humour, 26, 340
Aqueous layer, 102
Aqueous misdirection glaucoma, 357,
358
Aqueous penicillin G. solution, 130
Aqueous production, 375
Arachnodyctalous, 308, 310
Arachnoid villi, 5, 511, 513, 521
ARC, 639, 653
Arcuate or nerve fibre defect, 514, 518
Arcuate scotoma, 516
Arcus juvenilis, 190, 338
Arden ratio, 420
Area of non perfusion, 407
Argemone mexicana, 369
Argon gas, 61, 382, 383, 385, 487
Argon laser, 58, 383, 408, 493,
Argyll robertson pupil, 507, 509
Argyrosis, 117
ARMD, 415
Arrhythmia, 376
Arterial arcades, 38
Arterial phase, 414
Arterio sclerosis, 422
Arterio venous anastomosis, 415, 466,
713
Arterio venous aneurysm, 405, 409
Arterio venous shunt, 58
Arterio-sclerosis, 119
Arteriography, 693
Arteriovenous malformation of retina
and brain, 55
Arteritis, 526
Arthophoria, 569
Arthopods, 725, 742
Arthritic uveitis, 268
Arthritis with bowel disease, 268, 271,
742, 744
Arthropods, 46, 152, 154, 764, 765
Artificial drainage device, 350, 700
Artificial eye, 144
Artificial tear, 211
Ascariasis, 144
Ascending limb, 534, 700
Ascending optic atrophy, 532
Ascorbic acid, 294, 457
Aseptic, 218
Aseptic and necrosis of cornea, 216
Aseptic meningitis, 745, 746
Ash-leaf area, 59
Aspergillosis, 759, 760
Aspergillus, 203, 279
Aspirin, 142, 257
Associated buphthalmos, 343
Associated causes of congenital glau-
coma, 352
Associated congenital glaucoma, 56
Associated glaucoma, 57
Asteroid hyalitis, 438
Asteroid hyalopathy, 445
Asthenopia, 32, 47, 48, 60, 70, 143,
152, 178, 302, 303, 515, 546, 547,
549, 550, 557, 558, 564, 565, 566,
567, 570, 588, 647, 648, 667,
678, 681, 682, 685, 712
Astringent, 139, 140, 328, 362
Astringents vasoconstrictor, 142
Astrocytoma, 491, 534, 539, 710,
Astroid hyalitis, 461
INDEX 781
Astronomical telescope, 413
Asymmetry of face, 22, 611, 614
Asymmetry of orbit, 612
Asymmetry of the cup, 371
Ataxia talengectasia, 55, 138, 439,
605, 752
Atopic cataract, 143
Atopic conjunctivitis, 120
Atopic dermatitis, 44, 141, 163
Atopic kerato conjunctivitis, 139, 140,
170
Atovaquone, 261, 432
Atrasia of the inferior osteum, 90
Atreosclerotic retinopathy, 434
Atresia of puncta and canaliculus, 90,
92
Atrophic bulbae, 163, 166
Atrophic eye, 68
Atrophic holes, 449
Atrophic maculopathy, 436
Atrophic RPE, 415
Atrophy of choroid, 238
Atrophy of macula, 443
Atrophy of optic nerve, 530
Atrophy of the lid, 59
Atropine drops, 20, 77, 129, 130, 147,
149, 192, 193, 208, 220, 247, 254,
255, 506
Atropine induced amblyopia, 270
Atropine sulphate, 180, 182, 187, 198,
205, 209, 358
Attenuation of blood vessels, 436
Atypical form of retinitis pigmentosa,
233, 437
Atypical mycobacteria, 750
Atypical pigmentary dystrophy, 439
Auricular lymph, 125
Auto florescence, 518
Auto immunity, 75, 82, 138, 139, 147,
205, 240, 273, 276, 607, 714,
Auto refraction, 566
Autoclaving, 727
Autoimmune disease, 13
Autoimmune disorder, 75
Autonomic nervous system, 56, 505,
510
Autosomal dominant, 55, 70, 163, 210,
212
Autosomal recessive, 212
AV phenomenon, 617, 653
Avascularity of cornea, 169
Avulsion of optic nerve, 535
Axenfeld anomaly, 338
Axenfelds, 339
Axial basal view, 296, 694
Axial hypermetropia, 8, 556
Axial length, 14, 546
Axial length of eyeball, 701
Axial myopia, 549
Axial neuritis, 526
Axial proptosis, 676, 769
Axialcortex, 291
Axis of the lens, 291
Axons, 397, 532
Axoplasmic flow, 424, 521, 524
Azathioprine, 78, 258
Azithromycin, 137, 432
B
B cereus, 281
B scan ultra sonography, 412, 417,
424, 484, 700
B. lactamase, 733
Bacillus cereus, 258, 726
Bacitracin, 179, 192, 736
Baclofen, 635
Bacteria, fungi, 177
Bacterial conjunctivitis, 174
Bacterial endocarditis, 425
Bacterial exogenous endophthalmitis,
279
Bacterial keratitis, 208
Bacterial retinitis, 430
Bacterial ulcer, 183
Bacteriostatic, 102
Bagolini filter, 615
Bagolini glasses, 640
Bagolinies striated glass, 611, 653,
663
Bagolinis glasses, 641
Baheets disease, 258
Ball and socket pupillary block, 311
Band keratopathy, 166, 168, 172, 188,
190, 214, 248, 269, 270, 271, 274
Bandage lenses, 106, 181, 182, 184,
210, 211
Bar reading, 648
Barbiturates, 148, 347, 506
Barkans membrane, 339, 347
Barrel type, 570
Basal cell carcinoma, 54, 59
Basal meningitis, 82
Basal tearing, 105
Base down prism, 503, 615
Basic and reflex tear, 95
Basic exotropia, 667
Basic or resting tear, 102, 658, 662
Basic type non accommodative ac-
quired esotropias, 662
Basilar artery, 602, 606
Basilar artery aneurysm, 607
Basilar part, 602
Bassen-Kornzweig syndrome
(abetalipo proteinemia), 440
Batten-Mayou disease, 442
Battles sign, 620
BCG does, 147, 198, 732
Beaten bronze appearance, 444
Becitricin, 130
Bedsonia, 133
Behcets disease, 240, 253, 277
Behcets disease, 253
Behcets syndrome, 259
Behrs optic atrophy, 535
Behrs recessive neuropathy, 537
Bells palsy, 81, 82, 83, 742
Bells phenomenon, 14, 61, 71, 79, 81,
82, 597, 609, 610, 621
Bells protective reflex, 20
Benedicts syndrome, 73
Benign glioma, 710
Benign tumour, 478
Benzene hexachloride, 46
Bergemeister papilla, 518
Bergers space, 457
Bergmeisters papillae, 6, 399, 488,
459, 517, 519, 524,
Berlins oedema, 326
Bests disease, 444
Beta adrenergic agonists, 372
Beta blockers, 130, 151, 183, 184, 193,
205, 252, 255, 351, 355, 356, 360,
361, 362, 364, 365, 367, 368, 369,
370, 371, 372, 375
Betamethasone, 143, 256, 713
Betaxolol, 376
Bi-medial recession, 662
Bi-oblique astigmatism, 564
Biber-Haab, 211
Biconvex, 323
Bicycle-wheel pattern, 465
Bielschowsky, 611, 616
Bielschowsky phenomenon, 615
Bifoveal fixation, 270, 594, 660
Bifoveal fusion, 666
Big hypopyon, 191
Biguanides, 209
Bilateral amblyopia, 581
Bilateral exophthalmos, 81
Bilateral fourth nerve palsy, 603, 611
Bilateral irideplegia, 28
Bilateral ptosis, 624
Bilateral retinoblastoma, 690
Bilharziasis, 765
Bimatoprost, 372, 376
Binary fission, 133
Binasal hemianopias, 583
Binocular convergence test, 640
Binocular diplopia, 637
Binocular fusion, 638, 653
Binocular indirect ophthalmoscope,
28, 413
Binocular vision, 324, 569, 594, 595,
637
Bio microscope, 105
Biological glue, 448
Biomicroscopy of the posterior seg-
ment (Slit lamp, 413
Bioprosthesis, 737
Biphasic, 418
Bipolar cell, 393, 394, 395, 396, 399,
418
Birth injury, 171
Birth trauma, 16, 168, 216
Bitemporal hemianopia, 583
Bitotspot, 24, 105, 218
Bjerrums scotoma, 582
Black ball, 363
Black eye, 44, 709
Black fly-simulium, 771
Black spots, 411, 551
Blast injury, 173, 187, 324
Blastomycosis, 759, 760
Bleb failure, 385
Blepharitis, 45, 50, 104, 143, 173, 194,
198, 557, 565, 571, 726, 735, 738,
739, 762
Blepharo conjunctivitis, 142, 170, 173,
174, 198
Blepharo phimosis, 18, 22, 39, 40, 41,
42, 67, 71, 79
Blepharo spasm, 50, 67, 123, 147, 175,
206, 347, 728
Blepharochalasis, 39, 41
Blind spot, 524, 531, 582, 680
Blinding trachoma, 135
Blinding worm, 771
Blindness, 407
Blink mechanism, 14
Blink reflex, 321
Blocked intubatinon, 101
Blood agar, 177, 204, 209
Blood aqueous barrier, 229, 230, 373
Blood brain barrier, 375
Blood dyscrasia, 44, 119, 463
Blood pH, 487
Blood pyruvate, 369
Blood retinal barrier, 231, 395, 398,
423
Blood staining of cornea, 189, 363
Blood supply of the optic nerve, 512
Blood supply of the retina, 397
Blood supply of uvea, 231
Blood tinged hypopyon, 52
Blood vessels, 233
Blow out fracture, 609, 674
Blue dot cataract, 296, 298
Blue excitation filter, 414
Blue sclera, 163, 314, 722
Blue sclera brittle bone syndrome, 26
Blue-yellow defect, 580
Bluesclera, 12
Bluish green hypopyon, 737
Blunt injury, 324, 423, 560
Blunt trauma, 427
Blurring of disc margin, 523
Blurring of vision, 273
Board like rigidity, 125
Body, 89
Boeck candy bead test, 30
Boil, 53
Bollus IV mannitol, 380
Bone spicule, 154, 437, 466, 679
Bony nasolacrimal duct, 93
Bony orbit, 10
Borrelia, 740
Borreliaburgdorferi, 742
Botox, 635, 657
Botulin toxin A, 506, 635, 657
Bound fluorescein, 414
Bournvilles disease, 55, 58
Bow tie pallor, 532
Bowmans membrane, 3, 112, 135,
146, 159, 160, 161, 170, 185, 186,
187, 188, 210, 214, 217, 249, 336,
338
Brachial plexes, 71, 73, 512
Bradycardia, 312, 376,439, 680, 744
Brain stem, 56, 96, 259, 479, 510, 522,
674, 677, 703, 735, 743, 765
Brain stem glioma, 81
Brain stem lesion, 619
Brain stem trauma, 81
Branchial arch syndromes, 682
Branhamella, 735
Break up time, 217
Bridge coloboma, 234, 236, 515
Brightness, 578
Brimonidine, 372, 373, 375
Brinzolamide, 377
Broad based iridectomy, 379
Broad posterior synechia, 263
Bronchitis, 744
Brown rings, 329
Browns syndrome, 609, 613, 616
Brucellosis, 253, 727
Bruchs membrane, 4, 9, 231, 232,
233, 314, 395, 414, 435, 445, 553
Bulbar, 111, 141
Bulbar conjunctiva is rarely, 56, 110,
144, 150, 721
Bulbar conjunctiva, 111
Bulbar muscles, 76
Bulbus keratopathy, 166
Bulls eye appearance, 168, 441, 444
Buphthalmos, 18, 23, 24, 25, 26, 28,
62, 97, 166, 167, 171, 172, 190,
207, 216, 284, 314, 321, 335, 373,
375, 552, 560, 576, 678, 690, 724
Burkitts lymphoma, 687, 688, 708,
709, 750, 759
Burkitts sarcoma, 708
Burst chalazia, 49, 121, 149, 150
Busaccas nodule, 249, 250
BUT, 105
Button hole iridectomy, 379
By Schiotzs tonometer, 371
C
C pneumoniae, 757
C psittaci, 757
C trachomatis, 757
C. diptheria, 160
C. trachomatis, 758
C.A.I, 372
Cafaulait spot, 55, 539, 710
Calabar swelling, 772
Calcarine fissure, 583
Calcarious opacity, 249
Calcification, 57, 260, 478, 765
Calcium carbonate, 248, 478
Calcium metabolism, 297
Calcium phosphate, 248
Calcium soaps, 461
Calculation of duration of occlusion,
589
Caldwell view, 693
Caleoflour white, 67, 204
Calliper, 25
CAM vision stimulator, 590
Canal of Schlemm, 336, 339, 341, 350
Canalicular block, 93
Canaliculi, 7, 86, 88, 92, 98, 726, 760
Candida albicans, 144, 149, 279, 750,
761
Candida retinitis, 432
Candidiasis, 126, 246, 759, 761
Candle wax lesion, 276
Canopener capsulorrhexis, 323
Canthal ligaments, 36
Cantholysis, 715
Canthomental line, 694
Capillary drop out, 407
Capillary hemangioma, 60, 491, 685,
688, 690, 692, 698, 707, 712
Capillary non perfusion, 409
Capsulated diplococcus, 735
Capsule contraction syndrome, 323
Capsule of the lens, 291
Capsulo cortical, 295
Capsulo hyaloid adhesion, 291
Capsulo lenticular cataracts, 295, 296
INDEX 783
Capsulopalpebral fascia, 676
Capsulopalpebral ligament, 674
Capsulorrhexis. Continuous
curvilinear capsulorrhe, 323
Capsulotomy, 382
Carbachol, 505
Carbamazepine, 635
Carbolic acid, 50, 198, 199, 756
Carbon dioxide laser, 61, 382, 383,
384
Carbonic anhydrase, 183, 457
Carbonic anhydrase inhibitors, 252,
270, 355, 358, 360, 361, 364, 368,
371, 372, 376
Carboplatin, 482
Carbuncle, 738
Cardiac arrhythmia, 374
Cardiff, 30
Cardinal position, 594
Cardio selective, 372
Cardio selective betablockers, 375,
376
Carotene, 217
Carotico cavernous fistula, 691, 697
Carotinoid, 87, 394
Carpal tunnel syndrome, 312
Cartelol, 375
Cartilage, 284, 679
Caruncle, 35, 87, 89, 112, 113
Cataract in Lowes syndrome, 13, 20,
52, 162, 260, 270, 277, 306, 330,
429, 549, 590, 665, 668, 708
Cataract in siderosis, 329
Cataract secondary glaucoma, 269
Cataract secondary to ocular inflam-
mation, 330
Cataracta central pulverulenta, 296
Cataractogenesis, 297
Catarrhal conjunctivitis, 50, 123, 132,
147, 205, 748
Catford drum test, 16, 29, 632
Catis, 240
Causes of aphakia in children, 559
Causes of asthenopia include, 570
Causes of axial proptosis, 691
Causes of bilateral proptosis in chil-
dren are, 690
Cause of congenital cataracts can be,
315
Causes of conjunctival chemosis are,
117
Causes of cornal opacity, 188
Causes of corneal opacities in chil-
dren, 168
Causes of deep vascularisation, 171
Causes of diminished vision in retinal
detachm, 450
Causes of diminished vision in chronic
posterior u, 242, 247
Causes of RAPD, 508
Cauterise, 181, 183
Cavernous haemangioma of retina,
54, 55, 60, 61, 491, 493
Cavernous optic atrophy, 532, 535
Cavernous sinus, 73, 398, 502, 503,
512, 602, 604, 606, 612, 699, 704
CD ratio, 371
CD4 lymphocytes, 267
CD4 plus, 430
CD4+ lymphocytes, 749
Cefazolin, 129, 130, 184, 192, 192,
193, 728
Cefotoxamime, 728
Cell mediated type four allergic reac-
tion, 144
Cellophane maculopathy, 436
Cells, 272, 393
Cells in AC, 283
Cells in the vitreous, 283, 528
Cells in vitreous, 283
Celluflour white, 209
Cellular debris, 138, 360
Cellulitis lid, 44, 50, 51, 53, 97, 118,
703, 739, 763
Cellulose derivative, 106
Cematidine, 198
Central artery occlusion, 703
Central artery of the retina, 458
Central bitemporal hemianopia, 583
Central cataract, 747
Central choroiditis, 239, 318
Central corneal defect, 339
Central corneal opacity, 296
Central corneal ulcer, 127, 178, 190
Central gaze palsy, 76
Central leucoma, 127
Central nervous involvement, 59
Central retinal artery occlusion, 684
Central retinal vein, 398, 511, 521,
704
Central retinal vessels, 479, 510
Central retinitis pigmentosa (Inverse
retinitis pi, 438
Central serous chorio retinopathy,
416, 423, 527, 767
Central serous retinopathy, 415, 423,
516
Central space, 675
Central ulcer, 130
Central vein stasis, 684
Central vein thrombosis, 251, 414,
703
Central viral corneal ulcers, 193
Central vision, 396
Centre of budge, 73
Centrocecal scotoma, 520, 527, 531,
582, 641
Cephalic, 728
Cephalosporin, 726, 730, 739, 743
Cephalosporins, 203, 736, 744
Cephazoline, 179, 180
Cerbropontine angle tumour, 172
Cerebellar ataxia, 59, 606, 746
Cerebellum, 521
Cerebral abscess, 59, 766
Cerebral malaria, 767
Cerebral palsy, 656
Cerebral peduncle, 602
Cerebro spinal fluid, 479, 511
Cerebropontine angle lesions, 82
Cerebrovascular accidents, 310
Cerebrum, 530
Cervical sympathetic nerve, 37, 67,
71, 72, 228, 502
Cervical vertebra, 683
Cervicitis, 133, 733, 757
Cestodes, 764, 769
Chaffing of iris, 357
Chalazion, 48, 557, 565, 571, 647
Chamber, 382
Chandler syndrome, 337
Changes in corneal thickness, 167
Characteristics of abducent nerve
palsy, 618
Characteristics of afferent pupillary
defects, 507
Characteristics of amblyopic eyes, 585
Characteristics of an exudative
retinal detachment, 455
Characteristics of bacterial corneal,
177
Characteristics of concomitant squint,
649
Characteristics of fourth nerve palsy,
610
Characteristics of fully accommoda-
tive esotropia, 659
Characteristics of fungal ulcer, 177
Characteristics of infantile esotropia,
655
Characteristics of oculomotor nerve
palsy, 605
Characteristics of sterile corneal ul-
cer, 178
Characteristics of viral ulcer, 177
Chediak-Higashi syndrome, 402
Chelating agent, 215
Chemical burn, 169, 171, 173
Chemical cautery, 198, 199, 205
Chemical conjunctivitis, 46, 120, 128,
757
Chemical injuries as cause of glau-
coma, 214, 324, 368
Chemo prophylaxis, 136
Chemosis of conjunctiva, 117, 132,
139, 153, 479
Chemotherapy, 707, 708, 709, 759
Chequer board, 421
Cherry red spots, 425, 435, 442
Chiasma glioma, 56, 401, 510, 535,
541, 711
Chiasmatic hemianopia, 508
Chicken blindness, 219, 576
Chicken pox, 14, 49, 50, 93, 123, 132,
199, 745, 751
Chief causes of central corneal ulcers
are, 191
Childhood glaucoma, 343
Chlamydia, 12, 23, 133, 135
Chlamydia kerato conjunctivitis, 170
Chlamydia trachomatous, 127
Chlamydiasis, 756
Chlorambucil, 258
Chloramphenicol, 137, 179, 193, 526,
529, 728,736, 739, 744
Chloramphenicol ophthalmic drops,
100
Chloride, 103
Chloroquine, 440, 526, 529
Chloroquine maculopathy, 438
Chlortetracyclin, 137
Chlorumbucil, 706
Chocolate agar medium, 130, 209
Chocolate cyst, 712
Cholecystitis, 744
Cholesterol crystals, 407, 461, 488
Choline esterase, 657
Cholinergic antagonists, 505, 506
Chorio capillaries, 394, 397, 414
Chorio retinal atrophy, 446
Chorio retinal scar, 415, 429, 448, 457
Chorio retinitis, 421, 423, 427, 431,
449, 742
Choriocapillaries, 231
Choroid, 230
Choriodal hemangioma, 326, 476
Chorioretinal degeneration, 576
Chorioretinal scar, 382, 534
Chorioretinal scotomas, 582
Chorioretinitis, 52, 239, 260, 261, 262,
732, 741, 742, 759, 761, 762, 763,
765, 766, 767, 771
Choriostomas, 114, 151, 679
Choroid, 4, 153, 227, 230, 239, 393,
448, 701, 721
Choroidal bone formation, 696
Choroidal circulation, 416
Choroidal coloboma, 453
Choroidal crescent, 510
Choroidal degeneration, 552
Choroidal detachment, 453
Choroidal dystrophy, 438
Choroidal efflusion, 698
Choroidal fissure, 1, 233
Choroidal fold, 684
Choroidal haemangioma, 57
Choroidal infarct, 416
Choroidal neovascular membrane,
384
Choroidal neovascularisation, 445,
553, 764
Choroidal nevus, 445
Choroidal rupture, 326, 415, 427, 454,
457
Choroidal sclerosis, 237, 238
Choroidal tear, 326
Choroideremia, 237, 419, 423
Choroiditis, 238, 274, 526, 732, 742,
762
Choroio capillaries, 396
Christmas tree cataract, 296, 330
Chroid, 10
Chroidal effusion, 57
Chroidal repute, 325
Chroiditis, 52
Chroiocapillaries, 552
Chromatic aberration, 501
Chromatopsia, 574, 577
Chromosomal aberration, 12
Chromosome, 472
Chronic anterior uveitis, 97
Chronic blepharitis, 124, 170
Chronic congestive, 24
Chronic conjunctivitis, 45
Chronic cyclitis, 245, 258
Chronic dacryo adenitis, 97
Chronic dacryocystitis, 92, 94, 99, 129,
150, 174, 179, 183, 736, 752
Chronic dacryocystitis in children, 93
Chronic dacryodenitis, 96
Chronic endophthalmitis, 265, 324,
489, 771
Chronic follicular conjunctivitis, 142,
149, 756
Chronic glomerulo nephritis, 434
Chronic granulomatous, 48, 273
Chronic granulomatous inflamma-
tion, 48
Chronic granulomatous ulcer, 760
Chronic heart failure, 422
Chronic iridocylitis, 262
Chronic keratitis, 214
Chronic kerato conjunctivitis, 133
Chronic pan uveitis, 272, 275, 761
Chronic papillitis, 253
Chronic progressive external
ophthalmoplegia, 69, 76, 78
Chronic retinal detachment, 701
Chronic secondary open angle, 251
Chronic simple glaucoma, 344, 436,
437, 584
Chronic uveitis, 169, 214, 321, 453,
462
Chronic uveitis in young adults phthis
bulbae, 249
Chroroideal effusion syndrome13, 9
Chrysops, 772
Cicatricial, 133
Cicatricial ectropion, 62
Cicatricial pemphigoid, 139
Cicatricial trichiasis, 40
Cicatrisation, 125, 188
Cicatrising trachoma, 134
Cidofovir, 756
Cilia, 7
Ciliary band, 4, 624, 342
Ciliary block glaucoma, 357, 358
Ciliary body, 4, 181, 227, 284, 342, 393
Ciliary body, 229
Ciliary circulation, 405
Ciliary congestion, 116, 242
Ciliary epithelium, 4, 230, 341, 377,
456, 458
Ciliary ganglion, 502, 510, 511, 675,
677
Ciliary muscles, 10, 230, 233
Ciliary nerve, 160, 720
Ciliary processes, 4, 230, 233, 335,
340, 344, 488
Ciliary shutdown, 247, 359
Ciliary staphylomas, 62, 345, 723
Ciliary stroma, 230
Ciliary system, 231
Ciliary vessels, 230
ciliary zone, 227
Cilio choroidal detachment, 453
Cilio retinal artery, 405, 414
Cilio retinal vessel, 404
Ciliochoroidal detachment, 230, 282
Ciliolenticular block, 358
Ciprofloxacilin, 727, 737, 739, 743
Ciprofloxin, 128, 137, 179, 192, 271,
Circinate retinopathy, 405, 424
Circle of least diffusion, 546, 564
Circle of zinn, 596, 598, 599, 602, 603,
604
Circular fibres, 4, 230
Circum corneal congestion, 242, 271
Circum corneal flush, 24
Circumciliary congestion, 24, 62, 174,
175, 188, 241
Cirsoid aneurysm, 422
Classical galactosemia, 300
Classification of allergic conjunctivi-
tis, 139
INDEX 785
Classification of amblyopia, 586
Classification of exotropia, 665
Classification of glaucoma in children,
343
Classification of myopia, 547
Classification of nystagmus, 629
Classification of squint, 642
Claudes syndrome, 605
Clear lens extraction, 554, 569
Cleft lip, 682
Cleft palate, 679, 682, 747
Clindamycin, 126, 261, 727, 736, 432,
739,
Clinical anisocoria, 234
Clinical anisometropia, 567
Clinical feature of trachoma, 133
Clinical features of corneal ulcer, 175
Clinical features of uveitis in general,
241
Clinical presentation of retinho-
blastoma, 472
Clinically significant macular edema,
423
Clonazepam, 635
Clonidine, 375
Cloquets canal, 6, 465, 488
Closed fistula, 101
Closed globe injuries, 325
Closed head injury, 535
Clostridium botulinum, 729
Clostridium perfringes, 727
Clostridium tetani, 728
Clotrimazole, 205, 209
Cloudy cornea, 348
Clutton joints, 207, 740
CME, 245, 271
CMV retinitis, 267
CO
2
tension, 487
Coagulase negative cocci, 280, 738
Coagulase positive, 738
Coarse vision, 394
Coats disease, 259, 266, 368, 405, 422,
424, 425, 433, 426, 427, 428, 454,
462, 476, 477, 478, 483, 698
Coats of the eyeball, 230
Cobalt blue, 60, 195, 481
Cobalt blue filter, 101, 105, 176, 178
Cobble stone, 446
Cocaine, 74, 506
Cocaine test, 512
Coccidomycosis, 144, 149, 759, 761
Cockaynes disease, 439
Cockroaches, 772
Coenurosis, 153, 765
Coffee coloured hypopyon, 728
Cogan lid twitch, 76
Cogan Reese syndrome, 337
Coin test, 31
Cokaynes syndrome, 440
Cold test, 76
Colic, 77, 374
Colitis, 730
Collagen diseases, 424, 457
Collagen fibres, 228
Collagenase, 219
Collagenous tissue, 338
Collarette, 227, 235
College myopia, 549
Colliers sign, 81
Coloboma, 18, 39, 81, 162, 229, 233,
236, 416, 476, 622, 683
Coloboma of ciliary body, 235
Coloboma of iris, 19, 27, 187, 576
Coloboma of lens, 303, 488, 564
Coloboma of lid margin, 22, 104, 221
Coloboma of lower lid, 98
Coloboma of macula, 236
Coloboma of optic disk, 584
Coloboma of optic nerve head, 71
Coloboma of retina, 423
Coloboma of the choroid, 457
Coloboma of the disc, 516
Coloboma of the iris, 19, 233
Coloboma of the lens, 304
Coloboma of the lid, 16, 39,62, 173
Coloboma of the lip, 682
Coloboma of the lower lid, 101
Coloboma of the macula, 765
Coloboma of the pupil, 39
Coloboma of the uvea, 12, 19, 484, 558
Coloboma of upper lid, 7, 39
Coloboma with abnormal vessels, 410
Colobomas, 682
Colobomatous cystic eye ball, 8, 233
Colobomatous microphthalmos, 8, 9
Coloquets canal, 3, 457
Colostrum, 219
Colour agnosia, 580
Colour blindness, 574, 577
Colour defect, 13, 32, 411, 530, 538
Colour field, 580
Colour of pupil, 28
Colour of the iris, 227
Colour sense, 520, 537, 575, 582
Colour vision, 394, 396, 401, 586
Coloured haloes, 370
Columnar cells, 89
Combergs nyctometer, 418
Combined trabeculotomy and
trabeculostomy, 381
Commensal, 280, 730, 735
Common canaliculus, 86, 88
Common causes of exudative retinal
detachment, 454
Common causes of junevile glaucoma
are, 353
Common causes of sixth nerve palsy
in children, 618
Common causes of vitreous haemor-
rhage in children, 462
Common clinical types of colour blind-
ness, 579
Common conditions that do not re-
quire prophylaxis, 455
Common conditions where ERG is
useful, 419
Common peripheral retinal degenera-
tion, 445
Common secondaries that involve or-
bit in children, 708
Common side effect of atropine are,
255
Common vascular retinopathies in
adults, 434
Comotio retinae, 442
Compensatory phase, 629
Complete cataract, 17
Complete dislocation, 252
Complete third nerve palsy, 609
Complicated cataract, 175, 196, 204,
244, 274, 407, 488, 696
Complicated congenital ptosis, 70
Complicated contract, 453
Complicated heterochromia, 237
Complicated microphthalmos, 8, 9
Complication in acute anterior
uveitis, 247
Complication in buphthalmic eyes,
351
Complication of chronic anterior
uveitis, 248
Complication of corneal fistula, 188
Complication of naso lacrimal duct
obstruction, 91
Complication of occlusion, 590
Complication of rupture, 444
Complications of corneal ulcer, 185
Complications of herpes simplex
keratitis, 196
Complications of posterior uveitis,
253
Complications of retinopathy of pre-
maturity, 487
Composition of aqueous humour, 340
Composition of vitreous, 457
Compound astigmatism, 565
Compound hypermetropic, 565
Compound microscope, 413
Compound myopic, 565
Compound myopic astigmatism, 164,
307
Computed tomography, 697
Computerised corneal topography,
164, 166
Computerised keratoscopy, 566
Computerised oscilloscope, 418
Concave glasses, 550
Concave mirror retinoscope, 552
Concomitant, 612, 643, 651, 666
Concomitant divergent squint, 640
Concomitant squint, 585, 623, 649,
652
Concretion, 173
Concussion cataract, 325, 326
Concussion injury, 171, 554
Condensing lens, 413
Conditions where ARC may not de-
velop, 640
Conduction defect, 531
Cone and cone-rod dystrophies, 394,
434, 441
Cone monochromatism., 434, 442, 578
Cone pedicle, 396
Cones, 396, 418, 577
Coneurosis, 152
Confusion, 638
Congenial block of nasolacrimal Duct,
90
Congenital, 41, 68, 73, 121, 229, 258,
263, 303, 433, 611
Congenital achromatopsia, 442, 576
Congenital alternating esotropia, 640
Congenital and developmental cata-
ract, 316
Congenital anisometropia, 567
Congenital anomalies, 214, 453, 475
Congenital anomalies of anterior
chamber, 337
Congenital anomalies of optic disc are
common, 513
Congenital anomalies of orbit, 678
Congenital anomalies of retinal ves-
sels, 399, 404
Congenital anomalies of the cornea,
161
Congenital anomalies of the lens, 294
Congenital anomalies of the macula,
409
Congenital anomalies of the sclera,
722
Congenital anomalies of the uvea, 233
Congenital anomalies of the vitreous,
458
Congenital anomalies of uvea of late
onset, 237
Congenital anomaly of retinal vessels
of late onse, 405
Congenital aphakia, 19, 26
Congenital axial myopia, 26
Congenital melanosis, 24, 117, 440
Congenital membranous cataracts,
298
Congenital microcoria, 234
Congenital miosis, 74
Congenital mucocele, 18, 90, 92, 679
Congenital myopia, 13, 28, 348, 549,
678
Congenital nasolacrimal duct block,
92
Congenital nasolacrimal duct obstruc-
tion, 91, 348
Congenital night blindness, 404, 442,
574, 575
Congenital nuclear cataract, 297
Congenital nystagmus, 628
Congenital optic atrophy, 634
Congenital pigmentation of the
retina, 400
Congenital pit of the optic nerve, 453,
515
Congenital ptosis, 41, 68, 78
Congenital retinal degeneration and
dystrophies, 404
Congenital retinal fold, 476
Congenital retinoschisis, 403, 447,
464, 465, 475
Congenital rosette formation, 403
Congenital rubella, 25, 216, 261, 747
Congenital staphyloma, 722, 723
Congenital stationary night blind-
ness, 419, 574
Congenital syphilis, 171, 262, 429,
430, 740
Congenital telangiectasis of conjunc-
tiva, 115
Congenital toxoplasmosis, 236, 246,
454, 696, 765
Congenital tumour, 472
Congenital uveitis, 352
Congenital varices, 692
Congenitial myopia and myopia of
pre-maturity, 548
Congo red, 211
Congruous field defect, 581, 584
Conical detachment, 462
Conjugate gaze palsy, 619
Conjugate movement, 595, 610
Conjunctiva, 7, 14, 34, 56, 86, 93, 95,
102, 149, 154, 336, 676, 692, 759
Conjunctiva and sclera, 111
Conjunctival, 152
Conjunctival biopsy, 106
Conjunctival blanching, 375
Conjunctival bleeding, 119
Conjunctival congestion, 116, 134,
203
Conjunctival cysts, 121, 153
Congenital block of nasolacrimal duct,
90
Congenital brown syndrome, 616
Congenital cataract, 301, 321, 353,
407, 475, 484, 630, 683
Congenital coloboma of macula, 260,
263, 431
Congenital coloboma of the disc, 515
Congenital coloboma of the iris, 27
Congenital coloboma of the lower lid,
90
Congenital coloboma of the macula,
431
Congenital colour blindness, 237, 404
Congenital conjunctival lympha-
denoma, 114
Congenital corneal opacities, 168, 212
Congenital corneo scleral cysts, 121
Congenital crescent (conus) of the
optic disc, 514
Congenital cyst of lacrimal gland, 94,
95
Congenital cystic eyeball, 8, 515
Congenital cytomegalo virus retinitis,
430
Congenital dacryocystitis, 22, 90
Congenital day blindness, 404
Congenital disc swelling, 519
Congenital dysautonomia, 26
Congenital ectopic, 367
Congenital ectropion of uvea, 63, 353
Congenital esotropia, 40, 666
Congenital esotropia syndrome, 656
Congenital exotropia, 666
Congenital fibrosis of inferior rectus,
609
Congenital fibrosis syndrome, 624
Congenital fistula of the lacrimal sac,
90, 92
Congenital folds, 13
Congenital fourth nerve palsy, 610
Congenital glaucoma, 13, 17, 168,
301, 306, 370, 560, 741, 747
Congenital gliosis, 403
Congenital hamartoma, 539, 710
Congenital hereditary endothelial
defect, 216
Congenital hereditary endothelial
dystrophy, 25, 167, 212
Congenital hereditary stromal dystro-
phy, 25, 216
Congenital horners syndrome, 28, 42
Congenital internal ophthalmoplegia,
28
Congenital jerk nystagmus, 629, 630,
634
Congenital keratitis, 352
Congenital lymphe-dema, 114
INDEX 787
Conjunctival discharge, 24, 120
Conjunctival effusion, 742
Conjunctival epithelium, 110, 113,
120, 159, 171
Conjunctival follicles, 119
Conjunctival glands, 98, 113
Conjunctival hood flap, 189
Conjunctival impression cytology, 106
Conjunctival infiltration, 479
Conjunctival injection, 116
Conjunctival involvement, 201
Conjunctival membrane, 121
Conjunctival papillae, 120
Conjunctival recession, 662
Conjunctival scrapping, 106
Conjunctival shortening syndrome,
620, 623
Conjunctival smear, 123, 127, 136
Conjunctival ulcer, 762
Conjunctival veins, 336
Conjunctival xerosis, 24, 217
Conjunctivitis, 47, 50, 83, 86, 93, 116,
271, 571, 742, 743, 746
Conjunctivitis in new born, 126
Conjunctivitis of unknown causes in
children, 148
Connections of the third nerve nu-
cleus, 601
Consanguinity, 16, 47, 315, 435, 440,
443, 538, 575
Consecutive esotropia, 533, 663, 664
Consecutive optic atrophy, 422, 436,
532, 534
Consensual light reflex, 28
Consolidation of lung, 736
Constant exotropia, 665, 667
Constant occlusion, 589
Constricted peripheral field, 404, 584
Constrictor muscle, 306, 502
Contact dermatitis, 760
Contact lens, 103, 165, 169, 171, 195,
203, 208, 212, 270, 321, 309,
320, 375, 418, 550, 554, 558, 564,
567, 569, 635, 737
Contact lens fitting, 176
Contact lens intolerance, 143
Contact lens solutions, 208, 737
Contact lens wearer, 170
Contagious conjunctivitis, 131
Contaminated fluorescein, 192
Contents of anterior chamber, 26
Contra lateral antagonist, 625
Contra lateral hemiplegia, 202
Contrast, 537
Contrast sensitivity, 526, 533, 582,
586
Controlled radiation, 480
Contusion, 325
Convection current, 341
Conventional dacryocystography, 102
Conventional occlusion, 589
Conventional outflow, 341
Conventional trabeculectomy, 366
Convergence deficient exotropia, 667
Convergence excess esotropia, 662
Convergence paralysis, 668
Convergence reflex, 504
Convergent squint, 410, 618, 642
Convex mirror, 166
Convulsion, 260, 330, 431, 489, 765,
770
Coralliform, 296, 298
Corectopia, 27, 229, 234, 338, 501
Corkscrew cataract, 296
Cornea, 14, 56, 86, 102, 111, 418, 546
Cornea plana, 25, 26, 161, 163, 306,
308, 335, 339, 556
Corneal, 23, 585
Corneal abrasion, 24, 62, 90, 97, 195,
348, 576, 723
Corneal abscess, 208
Corneal antigen, 191
Corneal astigmatism, 563, 564
Corneal biopsy, 177
Corneal calliper, 18, 166
Corneal changes in trachoma, 135,
681
Corneal complication, 125, 248, 257
Corneal curvature, 25, 162
Corneal decompensation, 351
Corneal degenerations, 214
Corneal diameter, 474
Corneal dystrophies, 162, 168, 210,
190
Corneal dystrophies, 168
Corneal edema, 24, 167, 172, 173, 337
Corneal electrode, 420
Corneal endothelium, 160
Corneal epithelium, 2, 9, 110, 159,
293
Corneal fistula results, 174, 175, 187,
188
Corneal graft rejection, 170
Corneal haze, 16, 19, 24, 747
Corneal involvement in phlycten, 146,
201
Corneal light reflex, 642
Corneal loupe, 242
Corneal mucus plaque, 188
Corneal nerves, 56, 164, 175, 211, 214
Corneal oedema, 324
Corneal opacity, 39, 95, 147, 163, 167,
172, 174, 185, 324, 628, 665, 668,
686
Corneal opacity in new born and in-
fants, 215
Corneal plaque, 143, 166
Corneal reflexes, 639, 652
Corneal scarring, 166, 172, 196, 565
Corneal scleral shell, 144
Corneal sensation, 42, 210, 175, 195
Corneal staphyloma, 166, 169, 184,
188, 219
Corneal stroma, 10, 336
Corneal surface, 166
Corneal thickness, 701
Corneal toxicity, 197
Corneal transparency, 189
Corneal ulcer, 24, 50, 91, 97, 133, 166,
167, 172, 173, 188, 576, 710, 726,
760
Corneal ulcer in children, 172
Corneal ulcer threatening to perfo-
rate, 184
Corneal vascularisation, 147, 172
Corneal xerosis, 217
Corneo scleral junction, 159, 161, 324
Corneo scleral trephine, 350, 381
Corneo scleral meshwork, 336
Corneo scleral sulcus, 345
Corneo scleral trephine, 379
Coronal suture, 680
Coronary, 296
Corporis, 46
Correcting glasses, 645
Correctopia, 212, 614
Corresponding points, 637
Corrugated, 451
Cortex, 2, 292, 457, 587
Cortical adaptation, 639
Cortical blindness, 18, 21, 28, 421,
530, 584, 689
Cortical material, 26, 354
Cortical matter glaucoma, 367
Cortical venous thrombosis, 521
Cortico nuclear tract of both sides, 601
Cortico spinal tract, 605, 606
Cortico steroids, 251, 255, 260, 529
Corticosteroids, 330
Cortisol, 361
Corynebacterium diphtheriae, 122,
173, 729
Cosmetic blemish, 68
Cotton wool appearance, 424
Cotton wool spots, 267, 749
Cover test, 79, 615, 640, 643, 645, 652
COWS, 632
Coxsackie virus, 745
Cracker injury, 324
Cranial cavity, 697
Cranial nerve palsy, 52, 202, 203
Cranial neuritis, 56, 97
Cranial neuropathy, 276
Cranio facial anomalies, 38, 670
Cranio facial dysostosis, 16, 612, 678
Cranio facial injury, 536
Cranio pharyngioma, 508, 583, 696
Cranio synostosis, 348, 522, 679, 687,
688
Craniofacial, 13, 18
Craniofacial dysostosis, 678, 679, 681
Craniotomy, 541, 679, 680, 681
Crater, 139
Creds prophylaxis, 128, 133
Criswick-Schepen Syndrome, 465
Critical angle, 342
Crocodile tear, 608, 622
Cross fixation, 587, 633, 655, 659
Crouzons disease, 678, 695
Crouzons syndrome, 306, 681
Crowding phenomenon, 586, 588
Cryo, 58, 181, 349, 455, 480, 756
Cryo application, 143
Cryo coagulation, 409, 456
Cryo freezing, 266
Cryo therapy, 408, 487
Cryo-coagulation, 464
Cryotherpy, 480, 489, 493
Cryptococci, 750
Cryptococcus, 750
Cryptocoria, 19, 27
Cryptophthalmos, 16, 94
Crypts of henle, 103, 228
Crystalline dystrophy (Schnyders),
212
Crystalline penicillin, 128, 129
Crystalline salts of calcium carbonate,
214
CSF examination, 528
CSR, 416
CT, 416
Culture, 179
Cup of the disc, 510
Curvature hypermetropia, 163, 547,
556
Curvature myopia, 303, 547, 554
Curvature of cornea, 166
Cushing syndrome, 257
Cutaneous, 147
Cutaneous albinism, 400
Cutaneous diptheria, 730
Cutaneous erythema, 489
Cutaneous involvement, 58
Cutaneous melanoma, 479
Cutaneous tuberculosis, 731
Cyanide metabolism, 537
Cyanide sodium nitropruside, 310
Cyanoacrylate, 198
Cyanosis, 577, 737
Cycles per second, 629
Cyclic esotropia, 654, 657, 663, 664
Cyclic oculomotor palsy, 608
Cyclitic membrane, 354
Cyclitis, 239, 245, 271, 369
Cyclo cryo, 381
Cyclo destructive procedure, 351, 369,
380
Cyclo phosphamide, 708
Cyclo vertical muscles, 597
Cyclo vertical recti, 597
Cyclo vertically acting muscles, 593
Cycloablation, 384, 385
Cyclodestructive surgeries, 381
Cyclodialysis, 350, 366, 379, 381
Cyclodiathermy, 381
Cycloduction, 595
Cyclope, 773
Cyclopentolate HCL, 198, 208, 254,
263, 264, 269, 506
Cyclopentolate11, 20
Cyclophoria, 645, 648
Cyclophosphamide, 78, 482, 706, 708,
709
Cyclophoto coagulation, 385
Cycloplegia, 32, 46, 47, 49, 549, 618,
647
Cycloplegic, 8, 50, 124, 126, 131, 173,
180, 184, 197, 199, 208, 254, 264,
269, 563, 730
Cyclosporin, 78, 143, 258, 706
Cyclotonia, 373, 505, 650
Cyclovertical muscles, 613
Cycloxygenase, 257
Cydopentolate, 318
Cyno acrylate glue, 144
Cynoacrylate, 185
Cyst, 408
Cystathionine B. synthase, 309
Cystic eye ball, 1, 18, 722
Cystic phase, 765
Cysticercosis, 152, 522, 769
Cysticercus cellulose, 122, 152
Cysticerosis, 240, 765
Cysticircus, 769
Cystine, 42, 311, 537, 762
Cystinuria, 439
Cystoid cicatrix, 122
Cystoid degeneration, 445, 553
Cystoid macular, 272, 324
Cystoid macular edema, 57, 245, 246,
247, 257, 272, 277, 374, 376, 415,
423, 436, 437, 460, 490
Cystoid macular edema and papillitis,
248, 324, 326
Cystonosis, 439
Cysts and parasites, 417
Cysts of the conjunctiva, 151
Cytology of discharge, 121
Cytomegalo inclusion virus, 295
Cytomegalo virus, 72, 260, 433, 751,
755
Cytomegalo virus retinitis, 429, 430,
432, 741, 745, 750
Cytomelagic virus, 267
Cytopenaea, 258
Cytotoxic drug, 191, 195, 258, 706
D
D.N.A. viruses, 193
Dacroyocystography, 102
Dacryo cysto rhinostomy, 91
Dacryoadenitis, 96, 726, 738, 744,
746, 759
Dacryocystectomy, 100, 101
Dacryocystitis, 4, 49, 91, 703, 736,
738, 743, 748, 760
Dacryocystocele, 92
Dacryocystography, 93
Dacryolith, 98
Dactinomycin, 708
Dark adaptation, 411, 436, 441, 442,
443, 551, 574, 586
Dark adaptometry, 412
Dark field examination, 740, 741
Dark wedge test, 615
Daughter cysts, 769
Day blindness, 576
Day vision, 394
Deaf and dumb, 680
Deafness, 276, 621
Debridement, 181, 198, 199, 205
Decentered lens, 564
Deep exudate, 424
Deep keratitis, 94, 170
Deep vascularisation of cornea, 167,
170, 196, 206
Defective colour vision, 441
Defective contrast, 527
Defective stereopsis, 527
Defective vision, 557
Definite host, 152, 769
Deformity of medial turbinate, 101
Degeneration of cornea, 188
Dehiscence of levator, 69
Delayed (cellular) hypersensitivity,
138, 760
Delayed lid changes, 52
Delayed type-four hypersensitivity,
273
Delayed visual maturation, 21
Delen fuchs nodule, 273
Delivery system, 382
Demecarium, 505
Demodicosis, 765
Demonstration of leaking wound, 176
INDEX 789
Demyelinating diseases, 529
Demyelination, 514, 525, 526, 619
Dendrile, 194, 195, 753
Dendrites of herpes zoster, 201
Dendritic and geographic ulcer, 197,
199, 754
Dendritic keratitis, 127, 131
Dendritic ulcer, 195, 759
Deorsum duction, 595
Deorsum version, 595
Depiveprin, 372, 373, 374
Deposits on cornea, 26, 167, 188
Depot steroid, 60, 275
Depression, 595, 598, 599
Deprivation amblyopia, 60, 685
Depth of amblyopia, 588
Depth of focus, 501
Depth of suppression, 615
Dermal leishmaniasis, 768
Dermatitis medicamentosa, 44, 149,
255
Dermatome, 199, 200, 752
Dermatophytosis, 759, 762
Dermoid, 4, 25, 39, 114, 151, 152, 166,
189, 216, 622, 675, 683, 685, 687,
688, 690, 692, 696, 698, 707
Dermolipomas, 39, 114, 151, 683, 685
Descemet membrane, 3
Descemetocele, 174, 187
Descemets membrane, 112, 159, 161,
164, 174, 181, 186, 188, 210, 335,
338, 342, 344, 366
Descending arm, 700
Descending optic atrophy, 532
Desmetocele corneal ulcer, 167, 208
Detachment coloboma, 13
Detection and measurement of
cyclophoria, 646
Deturgscence, 160, 161
Deuteranomalous, 578
Deuteranomaly, 578
Deuteranopia, 578
Development of anterior chamber,
336
Development of chriocapillaries, 5
Development of cornea, 161
Development of extra ocular muscles,
4, 13, 600
Development of lacrimal and acces-
sory lacrimal, 87
Development of lacrimal system 7, 7
Development of lid, 7
Development of macula, 399
Development of oculomotor nerves,
601
Development of optic nerve, 512
Development of orbit, 677
Development of retinal pigment epi-
thelium, 398
Development of sclera, 722
Development of sensory (pars optica)
retina, 399
Development of the lacrimal drainage
system, 89
Development of the lens, 1
Development of the macula, 5
Development of the orbit, 7
Development of the uvea, 232
Development of vitreous, 458
Developmental amblyopia, 587
Developmental buphthalmos, 343
Developmental cataracts, 294, 315,
401, 475, 680
Developmental causes of night blind-
ness, 575
Developmental glaucoma, 343
Deviational nytagmus, 631
Devics neuro myelitis optica, 526,
527, 530
Devics neuromyelitis, 529
Dewormed, 153
Dexamethasone, 143, 147, 256, 361,
536
Dextro cycloversion, 595
Dextro elevation, 595
Dextro version, 629
Dextroversion, 595
Diabetes, 119, 179, 257, 330, 554, 759,
762
Diabetes insipidus, 541, 714
Diabetes mellitus, 299
Diabetes under treatment, 16
Diabetic cataracts, 301
Diabetic insipidus, 711
Diabetic ketosis, 762
Diabetic neovascularisation, 171
Diabetic oculomotor palsy, 607
Diabetic retinopathy, 414, 424, 426,
434
Diabetics proliferative retinopathy,
462
Diagnosis, 150
Diagnosis of amblyopia, 588
Diagnosis of conjunctival
rhinosporidiosis, 150
Diagnosis of fungal keratitis depends,
204
Diagnosis of galactose cataract, 300
Diagnosis of heterophoria, 645
Diagnosis of myasthenia, 77
Diagnosis of trachoma, 136
Diagnostic features in primary
buphthalmos, 346
Diagnostic procedure for retinal dys-
trophy, 440
Dialysis, 449
Dialysis of retina, 449
Diameter of cornea, 79
Diamidine, 209
Diaphragma sella, 512
Diarrhoea, 77, 219, 374, 576, 730, 767
Dichromatism, 578
Diclofenac, 257
Diclofenac sodium, 142
Diclorphenamide, 377
Dietary deficiency, 13
Diethyl carbamazine, 154
Differential diagnosis, 49, 153, 615,
624
Differential diagnosis of phlycten, 147
Differential diagnosis of primary
buphthalmos, 348
Differential diagnosis of
rhegmatogenous retinal, 453
Differential diagnosis of sixth nerve
palsy, 620
Differential intra ocular pressure test,
693
Diffuse chorioretinitis, 262, 534
Dihydro phenylalaline, 401
Dihydro sangunarine, 369
Diktyomo, 284, 368
Dilator muscles, 15, 306, 502
Dilator pupillae, 228
Dimer, 384
Diminish corneal sensation, 178
Diminished central vision, 441
Diminished distant vision, 436
Diminished night vision, 329, 404,
411, 436, 465
Diminished peripheral field, 436
Diminished vision, 242, 411
Dimorphic fungus, 761, 763
Diode Laser, 351, 382, 384, 416, 487
Dionin, 190
Diopteric, 546
Diphtheretic conjunctivitis, 123, 125
Diphtheria, 121, 174, 175
Diphtheroid, 110
Dipiveprin, 371
Diplegia, 680
Diplococci, 191
Diplopia, 75, 76, 77, 78, 523, 567, 570,
608, 610, 611, 618, 625, 638,
639, 651, 684, 689, 706, 729, 759,
763
Direct astigmatism, 564
Direct gonioscope of koeppe, 342, 346
Direct goniotomy, 349
Direct opthalmoscope, 246, 412, 552,
553, 632, 723,
Direct part, 598
Direct trauma to conjunctiva, 118
Disc angiomas, 58
Disc swelling, 521
Disciform, 296
Disciform degeneration, 416
Disciform degeneration of macula,
424, 435
Disciform keratitis, 49, 51, 53, 196,
201, 208, 751, 752, 755
Disinsertion of levator, 69, 449
Disjugate movements, 595
Disjunct nystagmus, 634
Dislocated lens, 417, 453
Dislocated lens nucleus, 282
Dislocation of cataractous lens, 325,
345
Disorders of glands of lid margin, 47
Disparate points, 637
Disseminated choroiditis, 239
Dissociated hyper deviation, 614
Dissociated vertical squint, 613, 614,
648, 653, 655
Dissociation of light near reflex, 506
Distance dysfunction, 649
Distant metastasis, 483
Distichiasis, 22, 39, 40, 682
Diuretics, 103
Diurnal variation, 341
Divergence excess, 648
Divergence excess exotropia, 667
Divergence weakness, 648, 647
Divergent squint, 642, 680
Diverticule, 102
DNA, 744, 749
DNA inhibitor, 197
Documentation of corneal lesions, 176
Dog tapeworm, 153
Dolichocephaly, 680
Dolicocephalous, 308, 681
Dolicocephaly, 681
Dolls eye movement, 624
Dominant drusen of the retina, 445,
435
Dominantly inherited bests vitelli
form, 420
Dorellos canal, 620
Dorsal, 6
Dorsal mid brain lesion, 80
Dorsal ophthalmic artery, 458
Dorsal pons, 619
Dorsal roof ganglion, 752
Dorso lateral pons, 619
Dorsum sella, 510
Dorzolamide, 377
Dot visual acuity test, 31
Double depressor palsy, 609
Double elevator palsy, 609, 613
Double eversion, 112
Double hyperphoria, 648
Double Maddox rod, 611, 645, 646
Double ring, 208
Double vision, 638
Doubling of the fundus, 305
Down beat nystagmus, 634
Down syndrome marfans syndrome,
40, 163, 318, 695
Doxorubicin, 482
Doxycyclin, 47, 137, 147, 727, 739,
742, 743, 758
Doynes choroiditis, 424
Doynes occluder, 589
Dracanculosis, 773
Dracanculus medinensis, 765, 773
Dracunculosis, 765
Draeger tonometer, 341
Dragging of retina, 487
Draining devices, 349
Draining vein, 408
Drops, 256
Drug abuse, 759
Drug induced, 149
Drug induced cycloplegia, 32
Drug induced myopia, 555
Drug induced optic neuritis, 529
Drugs, 148
Drusen, 59, 415, 437, 524
Drusen of optic nerve, 426, 427, 445,
517, 519, 524, 532, 542, 584, 701
Drusen of the retina, 423
Dry eye, 103, 120, 136, 139, 140, 142,
183, 481, 708
Dry spot, 105
Duanes retraction syndrome, 68, 621,
624, 656, 693
Duct of the meibomian gland, 48
Duction, 595
Ductusarteriosus, 747
Duplication of fundus, 307
Duplication of optic nerve head, 515
Dura, 5, 511, 513, 674, 676
DVD, 590, 656, 659, 666
Dwarfism, 541, 711
Dysarthria, 729
Dyschromatopsia, 329, 525, 526, 527,
538
Dyscoria, 234, 338, 614
Dysentery, 271, 730
Dysgenesis of anterior chamber, 12,
190, 229, 335
Dyskinesia, 618
Dyslexia, 574, 580
Dysplasia and a plasia of retina, 338,
402, 513, 682, 728, 729, 730
Dysplasia of macula, 236
Dyspnea, 737
Dysproteinemias, 424, 454
Dysthroid oculopathy, 75
Dysthyroid eye disease, 691
Dysthyroid myopathy, 77, 80
Dysthyroid oculopathy, 609
Dysthyroid ophthalmopathy, 613
Dystrophy, 13, 160, 163, 167, 171
Dystropies of cornea, 188
Dysuria, 733
Dysversion, 513
E
E. coli, 126
E. multi locularis, 768
E. oligoarthus, 768
E. vogeli, 769
Eales disease, 426, 427, 433, 454, 462
Early papilledema, 523
Early retinopathy, 485
Early rosette cataract, 325
Early venous phase, 415
Eccentric fixation, 586, 588, 590, 641,
652
Eccentric proptosis, 691
Ecchymosis, 43, 44, 314, 620, 709
Echinococcosis, 152, 153, 765
Echinococcous granulosus, 768
ECHO viruses, 745
Echothiophate, 505
Econazole, 205
Ectasia, 26, 185
Ectatic cicatrix, 186
Ectodermal, 1, 87, 458, 519, 539
Ectopia lentis, 13, 163, 301, 304,
311, 314
Ectopia pupil, 234
Ectopic lens, 234
Ectopic macula, 612, 652
Ectopic pupil, 306, 501
Ectopion, 39
Ectropion, 40, 63, 81, 98, 101, 111,
173, 200, 221, 337, 340
Edema, 16, 37, 43, 111, 143, 188, 212,
422
Edematous cornea, 177
Edinger westphal nucleus, 502, 503,
601
Edridge green lantern, 579
Edrophonium, 505
EDTA, 215
Effect of congenital palsy, 610
Effective origin, 598
Efferent disorder, 511
Efferent path, 628
Efferent pupillomotor defect, 510
Egg yolk, 444
Ehler Danlos, 453
INDEX 791
Ehler-Danols syndrome, 163, 306,
314, 446, 464, 723
Eight ball hyphaema, 363, 365
Eiphora, 94
El Bayadi lens, 412, 460
Elective caesarean section, 128
Electric cataract, 328
Electric shock, 324
Electro nystagmograph, 420
Electro oculography, 412, 420
Electro retinogram, 412, 418, 441
Electro-encephalogram, 21, 420
Electro-retinogram, 21
Electrocautery, 50
Electromyographic control, 657
Electrophoresis, 103
Electrophysiological investigations,
21
Elephantiasis neuro fibromatosa, 56
Elevation, 595, 597, 599
Elisa test, 260, 266, 268, 433, 489, 743
Ellsworth classification, 480
Elschnigs pearls, 322, 323
Embedded foreign bodies, 121
Embrayonic fissure, 458
Embryonal, 2, 513, 515, 707, 708
Embryonic, 1, 8, 233, 513
Embryonic fissure, 8, 233, 512
Embryonic lens nucleus, 293
Embryonic nucleus, 2, 291, 292
Embryonic pigmentary retinopathy,
100, 400, 429, 430
EMG, 657
Emmetropia, 14, 412, 519, 546, 650
Emphysema, 36, 687, 688, 689, 736
Enccphalo trigeminal angiomatosis,
151
Encephalitis, 49, 52, 97, 98, 200, 202,
259, 510, 522, 526, 576, 668,
735, 742, 744, 746, 748, 752
Encephalo facial angiomatosis, 55
Encephalo trigeminal syndrome, 57
Encephalocele, 678, 688, 691, 692, 695
Encephalopathy, 276, 748
Encircling bands, 456
Encroachment of conjunctiva on cor-
nea, 189
Encysted mucocele, 679
End point nystagmus, 629, 633
Endemic, 133
Endo cervicitis, 733
Endo laser coagulation, 464
Endo laser photo coagulation, 427,
433
Endo-genous bacterial endo-
phthalmitis, 433
Endocarditis, 733, 734, 737
Endogenous, 13, 117, 144
Endogenous endophthalmitis, 278
Endogenous uveitis, 240, 258
Endolymph, 632
Endometritis, 733, 757
Endophthalmities, 44, 118, 127, 175,
184, 188, 239, 241, 243, 246, 248,
253, 266, 278, 323, 326, 329, 417,
428, 430, 459, 461, 481, 483,
489, 526, 701, 726, 727, 728, 730,
731, 734, 736, 737, 738, 739,
743, 760, 762, 763, 764
Endophthalmitis phacoanaphy-
lactica, 277, 282, 294
Endophytic tumours, 477
Endophytum, 58, 478
Endophytum retinoblasoma, 478
Endospores, 385, 761
Endothelial channels, 712
Endothelial decompensation, 97
Endothelial defect, 405
Endothelial dusting, 243
Endothelial dystrophy, 212, 348, 741
Endothelial meshwork, 336
Endothelial plaque, 177, 182
Endothelial pump system, 161
Endothelium, 160, 161
Endothelium of cornea, 10
Endothelium of the iris, 160
Endotoxin, 280
Enlargement of blind spot, 516, 520,
534, 584
Enlargement of cornea, 344
Enlargement of the blind spot, 514,
518
Enlargement of the orbits, 695
Enophthalmos, 8, 20, 68, 74, 512, 684,
690
Entamoebahistolytica, 764, 766
Enteritis, 744
Entero virus, 132, 745
Enteroccocci, 739
Enterocollitis, 767
Entrapment of muscle, 693
Entropion, 39, 63, 116, 121, 125, 148,
173, 200, 730
Entropion in children, 63
Entropion of lid, 173
Enucleated, 284, 274, 696
Enucleated and eviscerated sockets,
68
Enucleation, 274, 696
Environment, 471
Enzyme, 377
EOG, 238, 420, 443, 575
Eosin, 209
Eosinophilic granuloma, 689, 713
Eosinophils, 121
Epaulet, 170, 207
Ephidrine, 506
Epiblepharon, 39, 40, 41
Epibulbar congenital tumours, 114
Epicanthic, 22, 39, 40, 79, 642
Epidedymitis, 733
Epidemic conjunctivitis, 119
Epidemic dropsy, 354, 369
Epidemic haemorrhagic conjunctivi-
tis, 24
Epidemic kerato conjunctivitis, 23, 44,
121, 123, 126, 131
Epidermoids, 679, 685, 687, 688
Epididymitis, 757
Epikaratophakia, 320, 321
Epikeratoplasty, 165
Epilate, 184
Epilation forceps, 46
Epilepsy, 680
Epimerase, 300
Epinephrine, 255, 361, 362, 364, 365,
371, 372, 373, 374, 506
Epiphora, 46, 82, 93, 100, 373
Epiretinal membrane, 416
Episclera, 336, 721
Episcleral congestion, 24
Episcleral nodules, 768
Episcleral pressure, 57, 342, 368, 375,
686
Episcleral tissue, 152
Episcleral veins drain, 336
Episcleral vessels, 341, 720, 721
Episcleritis, 201, 732, 742, 753, 758
Epistaxis, 115
Epistein-Barr virus, 253, 745
Epitarsus, 114
Epitaxis, 118
Epithelial cells, 243
Epithelial debries, 90
Epithelial defect, 185
Epithelial down growth, 167, 188, 189
Epithelial implantation cyst, 151
Epithelial keratitis, 53, 131, 132, 195
Epithelial keratopathy, 50
Epithelial macro erosion, 141
Epithelial punctate infiltrates, 51
Epithelial tissue, 87
Epithelioma, 59
Epithelium, 160, 161, 159, 168
Epithelium of ciliary body, 4
Epithora, 98
Epstein-Barr virus, 193, 267, 759
Equator, 291, 394, 473, 721
Equatorial staphylomas, 352, 724
ERG, 238, 419, 442, 443, 574, 575
Erosion, 210, 211
Erosion of cornea, 376
ERP, 419
Erratic fixation, 641
Error of refraction, 47, 70, 143, 346,
402, 513, 515, 581, 585, 615, 617,
621, 623, 644, 647, 650, 670
Errors of metabolism, 309
Errors of refraction, 13, 46, 47, 124,
234, 237, 587, 631, 642, 664
Erysipelas, 738, 739
Erythema multi forme major, 148
Erythema multi formis, 121, 126, 139
Erythema multiform minor, 148
Erythematous, 148
Erytholastic glaucoma, 365
Erythroblastosis fetalis, 741
Erythromycin, 47, 128, 129, 130, 133,
137, 147, 179, 192, 193, 271, 729,
730, 735, 736, 739, 742, 743
Erythropsia, 426, 459, 577
Escherichia coli, 644, 647, 730
Esophoria, 557, 621
Esotropia, 83, 431, 444, 474, 550, 551,
557,558, 587, 621, 623, 624, 633,
638, 653, 654
Esotropia in myopia, 658, 662, 663
Esotropic, 621, 650
Espundia, 767
ESR, 607
Essen prognosis Classification, 483
Essential amino acid, 309
Essential blepharo spasm, 658
Essential iris atrophy, 251, 337
Established papilledema, 523
Estropia, 260
Ethambutol, 526, 529
Ethmoid, 673, 694
Ethmoidal sinuses, 511, 674
Ethyl morphine, 190
Etiology, 273
Etiology of accommodative esotropia,
659
Etiology of anisometropia, 567
Etiology of concomitant squint, 649
Etiology of congenital, 295
Eucatropine, 506
Euryblepharon, 39, 41
Evaluation of a case of proptosis, 78,
686
Eviscerated, 481
Evisceration, 274
Evisceration of the eye, 283
Evoked vestibular nystagmus, 630
Ewings sarcoma, 686, 688, 708, 709
Exadative retinal detachment, 454
Examination of anterior chamber, 26
Examination of iris, 26
Examination of lens, 28
Examination of pupillary light reflex,
28
Extra pulmonary tuberculosis, 147,
731
Exudates, 423, 429
Exudative membrane, 280
Exudative retinal detachment, 246,
260, 265, 276, 453, 771
Exudative retinal vasculopathy, 405
Exudative vitreo retinopathy, 464
Eye, 97
Eye in primary buphthalmos, 344
Eye speculum, 20
Eye with leucoma adherence, 186
Eyebrow, 22, 34
Eyes, 765
F
Fabrys disease, 301
Face wash, 137
Facial, 38
Facial asymmetry, 610
Facial canal, 61, 82
Facial diplegia, 62, 81, 83
Facial nerve, 81
Facial palsy, 98, 619, 728, 742
Facicular, 73
Faciomandibular abnormality, 16
Factors that precipitate decom-
pensation, 647
Faculatative hypermetropia, 556
Facultative anaerobes, 738, 739, 744
Facultative hypermetropia, 248, 255
Facultative suppression, 641, 666
Faden operation, 616
Faden procedure, 623
Failed DCR, 101
Failed keratoplasty, 215, 249
Failed sclerotomy, 385
Failure to identify causative organ-
ism, 183
Falciparum, 766
Famciclovir, 197
Familial dysautonomia, 511
Familial exudative vitreo retino-
pathy, 28, 407, 433, 476, 489
Familial exudative vitreoretinal dys-
trophy, 435
Familial idiocy, 442
Farbers disease, 425
Farsightedness, 555
Fascia bulbi, 676
Fascicle, 603, 606
Fascicular, 602
Fascicular lesion, 619
Fascicular part, 602
Fascicular ulcer, 146, 170, 576, 732
Fasciculo nuclear, 603
Fast eye movement, 595
Examination of sclera, 26
Examination of the lids, 79
Examination of vitreous, 28
Exanthematous, 49, 747, 752
Exccyclotropia, 611
Excentration, 708
Excimer, 210, 211, 215, 249, 270, 382,
383
Exciting eye, 273
Exciting filter, 416
Excoriation, 141, 149
Excursion, 79
Excyclodeviation, 656
Excycloduction, 595
Excyclophoria, 611, 645, 649
Exfoliations, 342
Exocrine, 87
Exogenous, 13, 117, 139
Exogenous endophthalmitis, 279
Exogenous microbial uveitis, 240
Exophoria, 76, 550, 644, 648
Exophthal, 117
Exophthalmometer, 691
Exophthalmos, 62, 80, 81, 111, 681,
684
Exophytic tumours, 477
Exophytum retinoblastoma, 478
Exotoxin, 727
Exotropia, 550, 596, 605, 611, 616,
621, 639, 653, 658, 664, 678, 681
Exposure keratitis, 82, 177, 680, 681,
684, 685, 686, 704, 707, 710
Expulsive haemorrhages, 351
Extended wear lenses, 144, 321
Extensive coloboma, 233
External auditory, 82
External beam radiation, 481, 708
External carotid, 38
External filtration procedures, 381
External limiting membrane, 395,
396, 477
External nares, 101
External ophthalmoplegia, 605, 681,
728
External pterygoid muscle, 72
External trabeculotomy, 349, 350
Extortion, 598, 599, 615
Extortors, 597
Extra capsular cataract extraction,
322
Extra conal lesion, 675
Extra dural abscess, 620
Extra dural hematoma, 606
Extra ocular metastasis, 471
Extra ocular muscle involvement, 479
Extra ocular muscle palsy, 420, 702
Extra ocular muscles, 10, 42, 697
Extra ocular retinoblastoma, 695
INDEX 793
Fast fibres, 593
Fate of retinal haemorrhages, 427
Fatty acidamide, 376
Faulty projection, 326
Favre goldmann syndrome, 464, 465
Feeding artery, 408
Feldman adaptometer, 418
Fenestrated, 231
Fenestrated walls, 414
Fenestration of optic nerve sheath,
536, 538
Fenestreted, 334
Festooned, 244, 250
Fetal distress, 16
Fever, 747
Fibrenous exudates in the AC, 244
Fibrin, 181
Fibro vascular proliferation, 170
Fibroblastic reaction, 188
Fibroblasts, 228
Fibrocytes, 167, 174, 185, 186, 188
Fibroma molluscum, 56
Fibrosis, 256
Fibrosis of trabecular meshwork, 354
Fibrous dysplasia, 687, 688, 691, 695,
696
Fibrous histocystoma, 479
Field changes, 370
Fifth nerve, 97
Filamentary keratitis, 105, 195
Filamentary keratopathy, 176
Filamentous, 203, 763
Filling defect, 102, 416
FIN, 568
Fine KPs, 242
Fine needle biopsy, 540, 706
Fine pannus, 234
Fine vision, 394
First neuron, 73, 503
Fish spawn, 322
Fissures of the orbit, 676
Fistula, 92
Fistula of lacrimal gland, 94, 95
Fistula of sac, 91, 99
Fixation forceps, 692
Fixation reflex, 16, 637
Fixational nystagmus, 630
Fixed bodies, 461
Fixed thick hypopyon, 281
Flame shaped haemorrhages, 524
Flares, 242, 272
Flash VER, 421
Flashes of light, 411, 446
Flat retinoblastoma, 423
Flecked retina syndromes, 443
Flecks, 444
Fleurettes, 477, 478, 482
Flexner Wintersteiner, 477, 482
Floaters, 245, 246, 252, 446, 450, 462
Floating cyst in the vitreous, 153
Floor of the orbit, 599, 673
Florid stage, 206
Floriform, 298
Flourescein angiography, 58, 176,
245, 251, 274, 276, 277, 394, 405,
407, 409, 412, 424, 426, 444, 523,
764
Fluconazole, 209
Fluid gas exchange, 278
Fluid shifts, 455
Fluorescein, 172, 173, 174, 175, 176,
177, 178, 184, 203, 348, 398
Fluorescein drops, 737
Fluorescein sodium, 46, 101
Fluorescence, 91, 414
Fluoresecin staining, 105
Fluoreseine clearance, 101
Fluorometholone, 143
Fluoroquinolones, 526, 735, 744
Flurbiprophen, 140, 142
Flurometholone, 361
Fluroquinolones, 726
Fluroquinols, 193
FML, 147
Focal interval, 564
Focal point, 563
Foetal, 2
Foetal fissure, 293
Foetal nucleus, 292
Foetus, 754
Foggin, 567
Folds in descemets membrane, 171,
189, 196
Folic acid antagonist, 432
Folicular conjunctivitis, 53
Folinic, 261
Folinic acid, 432
Folk medicines, 190
Follicle, 23, 37, 116, 131, 132, 134,
139, 140, 147
Follicular conjunctivitis, 50, 119, 131,
132, 136, 750
Follicular hypertrophy, 134, 148
Follicular reaction, 203
Follicular conjunctivitis, 194
Folliculosis, 119, 148
Fomites, 132
Fontanelle, 680
Foramen rotundum, 694
Forced choice preferential looking
(F.P.L), 16
Forced duction test, 623, 624, 692
Forehead, 418
Foreign body, 149, 173
Foreign body granuloma, 49, 150, 689
Foreign body on the cornea, 97
Foreign body sensation, 195
Formation of aqueous humour, 340
Formation of peripheral anterior
synechia, 354
Formed hallucination, 581
Fornices, 37, 94, 134, 150, 712
Fornix, 7, 110, 111, 112, 115, 148
Fortified, 130
Fortified antibiotic, 179, 182
Fortified aqueous drops, 192
Fortified cefazoline, 739
Fortified cepfazolin, 192
Fortified drops, 183, 193
Fortified gentamycin, 743
Fossa for lacrimal gland, 37, 86, 673
Fossa for lacrimal sac, 88, 673
Foster Kennedy syndrome, 532
Foster-Fuchs spot, 553
Four dioptre prism test, 653
Four dot iridotomy, 379, 380
Fourth nerve nucleus, 73, 599, 602,
745
Fourth ventricle, 521, 604
Fovea, 393, 394, 599
Foveal avascular zone (FAZ), 394, 415
Foveal projection, 638
Foveal reflex, 15
Foveola, 394
Fovilles syndrome, 82, 619
Fracture base of skull, 45, 118
Fracture floor of orbit, 68, 616
Framycetin, 179
Franceschettis syndrome, 39, 682
Frank corneal opacity, 19
Frans worth D-15 test, 579
Frans worth munsell 100 hue test,
579
Fransworth hue, 579
Free edge of the tentorium, 602
Free fluorescein, 414
Free iodine, 416
Friedreichs ataxia, 527
Friend test, 568
Fronds, 382
Frontal, 114, 673, 677
Frontal occulomotor centre, 504
Frontal process of maxilla, 89
Frontal sinuses, 694
Frontalis, 42
Fronto mesencephalic, 596, 601
Frontomaxillary, 36
Frontozygomatic suture, 676
Frozen orbit, 705
Fuchs coloboma, 515
Fuchs dystrophy, 169
Fuchs fleck, 553
Fuchs heterochromatic iritis, 240,
241, 251
Fuchs heterochromic cyclitis, 237
Fuchs heterochronic iriodcyclitis, 276
Fuchs heterochronic uveitis, 259
Fuciform aneurysm, 308
Functional amblyopia, 587
functions of aqueous humour, 340
Functions of the choroids, 231
Functions of the sclera, 722
Fundal glow, 280
Fundamental phase, 629
Fundus, 15, 89, 558
Fundus albipunctatus, 445, 575
Fundus camera, 414
Fundus changes, 245
Fundus changes consists of, 253
Fundus flavimaculatous, 443, 414,
524
Fundus in optic neuritis, 528
Fundus of the lacrimal sac, 35
Fungal culture, 179
Fungal endophthalmitis, 253
Fungal hypopyon, 190
Fungal keratitis, 203
Fungal ulcer, 175, 183, 184
Fungi, 267
Fungus, 759
Fusarium, 203
Fusion, 22, 642
Fusion reflex, 637
Fusional amplitude, 648
Fusional centre, 637
Fusional convergence, 22, 571
Fusional divergence, 648, 660
Fusional reserve, 647
G
Gabapentin, 635
Galactocemia, 315, 317
Galactokinase deficiency, 317
Galactosaemia, 299
Galactose, 299
Galactosemic, 319
Gallium scan, 272
Gama camera, 102
Gamma crystalline, 293
Ganciclovir, 750
Ganglion cell, 5, 393, 394, 395,399,
435, 442, 510, 767
Ganglion cell layers, 397
Gangliosidosis, 443
Ganthostomiasis, 765
Gas, 381
Gas gangrene, 727
Gas in AC, 730
Gas lasers, 383
Gas permeable lenses, 321
Gasserian ganglion, 704
Gastroenteritis, 133, 748
Gaze, 594
Gaze palsy, 619, 630
Gaze paretic nystagmus, 630
Gel, 373
General features of exotropia, 664
General features of optic neuritis, 526
General fibrosis syndrome, 609, 613,
620, 623
General information about ophthal-
mic laser, 381
Genetic counselling, 472
Genetic mutation, 12
Geniculate ganglion, 82, 753
Genital herpes, 53
Genital trachoma, 757
Genital virus, 754
Gentamycin, 128, 149, 179, 180, 193,
727, 730
Geographic, 195, 197, 755,
German measles, 746
Germinal mutation, 472
Ghost cell glaucoma, 363, 365
Ghost vessels, 169, 171, 207, 741
Giant cell, 48, 273
Giant papillary conjunctivitis, 139
Giant tear, 449
Giardia, 764
Giardialamblia, 767
Giardiasis, 767
Giemsa, 129, 757
Giemsa stain, 127, 177, 182, 204, 209
Gingivitis, 754
Gland of Krause, 113, 152
Gland of Wolfring, 113
Glands of Moll, 38
Glands of Zies and Moll, 9, 45, 47, 102,
103
Glare, 164, 324, 550, 553, 576, 632
Glass , 324, 667
Glass particles, 700
Glaucoma, 8, 13, 24, 56, 57, 162, 163,
185, 260, 271, 277, 306, 324, 325,
339, 353, 383, 683
Glaucoma implants, 368
Glaucoma in Lowes syndrome, 353
Glaucoma in non surgical penetrat-
ing injury, 354
Glaucoma in older children, 353
Glaucoma inverse, 302, 367
Glaucoma operation, 273
Glaucoma secondary to angle reces-
sion, 365
Glaucoma secondary to anterior
uveitis, 359
Glaucoma valves, 169, 381
Glaucomatocyclitis crisis, 354
Glaucomatous disc, 370
Glaucomatous neuropathy, 363
Glaucomatous optic atrophy, 508, 532
Glaucomcyclitis crisis, 369
Glial proliferation, 445, 533
Glial tissue, 534
Glioma, 17, 475, 483, 539, 583, 691,
699, 711
Glioneuroma, 491, 492
Gliosis, 429, 524
Globe, 8, 116, 697
Globular detachment, 462
Globulin, 103
Glomerulonephritis, 739
Glossoptosis, 682
Glucose, 100, 103, 160, 299
Glutathion, 294
Glycerine, 93, 358, 364, 378, 715
Glycerol, 372
Glycolipids, 442
Glycolysis, 161
Goats, 153
Goblet cell, Krauses cells, 112
Goblet cells, 14, 35, 87, 89, 103, 110,
113, 217
Golden shower, 461
Goldenhar gorlin syndrome, 115
Goldenhars syndrome, 39, 115, 682,
683
Goldman three mirror, 245
Goldman three-mirror contact lens,
412, 460
Goldmann-favre vitreoretinal dystro-
phy, 435
Goldmann three mirror gonioscope,
413
Goldmann Weekers adaptometer,
418
Goldmanns tonometer, 341
Gomori methamine silver stain, 177,
204
Gonio lens, 342, 383
Gonio photo coagulation, 385
Gonio puncture, 349
Goniolens air interface, 342
Gonioplasty, 385
Goniopuncture, 381, 385
Gonioscopes, 342, 382
Gonioscopic anatomy of angle of an-
terior chamber, 342
Gonioscopy, 342
Goniotome, 349
Goniotomy, 349, 361, 378, 379, 380,
381
Gonococcal, 120, 126, 144, 160, 173,
175, 182, 240, 248, 258, 734
INDEX 795
Gonococci, 126, 174
Gonococci meningococci, 174
Gonorrhoea, 12
Gradenigo syndrome, 607, 620
Grades of binocular vision are, 638
Grades of nystagmus, 629
Gradient method, 660
Gram, 127
Gram negative, 192, 727, 735, 737,
743, 744, 757
Gram negative bacteria, 280
Gram negative diplococci, 733, 734
Gram negative rod, 730
Gram positive, 191, 729, 730, 735,
738, 739, 763
Gram positive cocci, 280
Gram-negative cocobacilli, 726
Grams stain, 123, 127, 182, 204, 209
Granular corneal dystrophy
(Groenouws I), 211
Granuloma, 149, 701
Granulomas of conjunctiva, 121, 149
Granulomatous, 241, 263, 276, 726,
732
Granulomatous amaebic encephalitis,
766
Granulomatous angitis, 753
Granulomatous canaliculitis, 762
Granulomatous conjunctivitis, 758,
761
Granulomatous reaction, 769
Granulomatous uveitis, 241, 243, 276
Graphic method, 660
Grating acuity, 586
Gray (grey) pseudo optic atrophy, 538
Gray reflex, 514, 515
Grayson-Wilbrandt, 211
Greater circle of the iris, 228
Greater wing of sphenoid, 596, 674,
679, 680, 694
Green argon, 383
Grey line, 38
Grey reflex, 433, 451
Grocott-Gomori stain, 763
Gross pannus, 170
Ground glass, 171, 206, 273, 589, 741
Grubers (petro clinoid) ligament, 604
Guanethidine, 505
Guillain Barre syndrome, 82, 742
Gumma, 250
Gusto lacrimal reflex, 96
Guttatae, 262
Gylcerol, 378
Gyrate atrophy of choroid, 237, 419
H
H. aegyptius, 122, 731
H. influenza, 92, 126
Haabs, 24, 171, 344
Haemangioblastoma, 58
Haemangioma, 49, 92, 116, 119, 150,
565, 687, 695, 696
Haemangioma of lid, 590
Haemangiomachoroid, 701
Haemangiomas, 151, 414, 491
Haematuria, 115
Haemoglobin, 383, 384
Haemolytic anaemia, 728
Haemolytic-uremic syndrome, 730
Haemophilia, 44, 425
Haemophilus, 53, 119, 122, 124,127,
130, 702
Haemorrhages, 123, 126, 422, 428,
429, 460, 518, 552
Haidinger brush, 653
Hair bulb incubation test, 401
Hair follicles, 679
Hallerman Streiff syndrome, 678,
682, 683
Halogen, 384
Halothene, 347
Hamartoma, 55, 59, 151, 368, 399,
408, 409, 415, 425, 491, 493, 518,
712
Hand Schuller Christian, 689, 713
Harada, 259, 276
Hard contact lenses, 321, 554
Hard exudates, 424, 445
Hardy-Rand-Ritler, 579
Harelip, 679
Harmonious, 639
Harpes simplex keratitis, 53
Hay, 23, 139
Head injury, 118
Head light in the fog manner, 260
Head nodding, 632, 633
Headache, 275, 522, 571
Healed iridocyclitis, 74
Healed trachoma, 134
Heart block, 78
Heinzbodies, 365
Helium, 383
Helminth, 152, 764
Helminths, 764
Hemagglutination test, 431
Hemangio blastoma, 408
Hemangioma, 18, 22, 24, 68, 283
Hematoma, 314, 685
Hematoxylin, 209
Hemianopia, 509, 582, 584, 632
Hemiatrophy of the face, 57
Hemidesmosome, 249
Hemiparesis, 620
Hemiplegia, 82, 200, 605, 753
Hemivertebrae, 308, 683
Hemoglobinopathy, 454
Hemolytic anaemia, 299
Hemolytic glaucoma, 363, 364
Hemophilus influenza, 731
Hemophthalmos, 427, 464
Hemoptysis, 763
Hemorrhagic macular lesion, 764
Hemosiderin, 164, 364
Hemosiderine, 364
Henkind and seigel scotometer, 418
Henles gland, 113
Heparin coated IOL, 323
Hepatespleenomegaly, 431
Hepatitis, 299, 529, 762
Hepato lenticular degeneration
(Wilsons disease), 299
Hepato spleenomegaly, 268, 317, 402,
489, 740, 747, 755, 765
Hepatolenticular degeneration, 301
Herberts, 134, 136
Herberts pits, 136
Hereditary, 210, 477
Hereditary epithelial dystrophy, 211
Hereditary juvenile epithelial dystro-
phy, 210
Hereditary pendular nystagmus, 630,
634
Heredity in retinoblastoma, 472
Heredo familial, 533
Heredo familial optic neuropathies,
533, 536
Heredofamilial optic atrophies, 535
Herellea vaginicola, 726
Herings law, 595
Heritable retinoblastoma, 471
Herpes , 12, 16, 23, 26, 44, 49, 50, 52,
53, 82, 96, 121, 123, 126, 127,
129, 131, 148, 170, 171, 172, 173,
174, 175, 183, 191, 195, 193, 199,
200, 206, 208, 216, 240, 243,
248, 251, 253, 258, 260, 261, 267,
429, 430, 432, 741, 745, 750, 751,
752, 753, 754
Hertels mirror proptometer, 691
Hertz, 629
Heterochromia, 74, 227, 474, 509,
511, 683
Heterochromia iridis, 27, 227, 237
Heterochromia of iris, 57, 337, 402,
479, 622
Heterochromia of the uvea, 237
Heterologous host phase, 765
Heteronymous, 583, 642, 643, 649,
660
Hexagonal keratotomy, 559
Hexamidine, 209
Hexose monophosphate shunt, 161
High AC/A ratio, 656
High arched palate, 308, 682
High astigmatism, 401, 563
High axial myopia, 352
High errors of refraction, 628
High hypermetropia, 412
High myopia, 461, 487, 576, 683
High-water mark, 451
Higher bacteria, 763
Highly active anti retro viral therapy
(HART), 750
Hippus, 229
Hirschberg test, 652, 666
Histamine, 138, 370, 376, 506
Histocytosis, 713
Histoplasma capsulatum, 763
Histoplasmosis, 759
HIV, 430
HIV retinopathy, 749
HLA B-27, 268
Hole in the iris, 201, 337
Holes, 448
Holmgrens wool test, 579
Home atropine, 149, 173, 254, 269,
318, 506
Home atropine hydrobromide, 180,
198, 208
Home stereogram, 648
Homer-Wright rosettes, 477
Homocystinurea, 301, 306, 309, 311,
315, 464
Homocytinuria and other amino
aciduria, 299
Homologous host phase, 765
Homonomous horizontal diplopia, 618
Homonymous, 583, 584, 638, 666
Hordeolum externum, 47
Hordeolum internum, 48
Horizontal cells, 396
Horizontal concomitant squint, 653
Horizontal gaze palsy, 605
Horizontal muscles, 596
Horizontal nystagmus, 401
Horizontal phorias, 644
Horizontal school, 669
Horizontally acting muscles, 593
Horner-Trantas spot, 141
Horners pupil, 511
Horners syndrome, 42, 69, 73, 237,
502, 506, 606, 612, 619, 745
Horoptor, 638
Horse shoe, 449
HOTV Test, 31
House dust mite, 140
House fly, 772
Hruby, 412, 413, 460, 518
Hue, 578
Human adeno viruses, 745
Human herpes, 193
Human immuno deficiency virus, 749
Human papilloma virus, 745
Human tetanus immunoglobulin, 729
Humoral, 138
Hurlers disease, 425
Hutchinson sign, 51
Hutchinson type, 709
Hutchinsons pupil, 510, 606
Hutchinsons salmon patch, 170
Hutchinsons sign, 200, 753
Hutchinsons teeth, 207
Hutchinsons triade, 207
Hyaline, 284
Hyaloid artery, 293, 457, 458, 512
Hyaloid capsular attachment, 457
Hyaloid system, 6, 459
Hyaluronic acid, 454, 457
Hydatid cyst, 122, 769
Hydocele, 771
Hydrocephalus, 522, 583, 613, 680,
765
Hydrocephaly, 533, 679
Hydrogel, 562
Hydrophobic, 103
Hydrops of cornea, 576
Hydrops of keratoconus, 164
Hydroxy amphetamine, 74, 506, 512
Hydroxycobalamin, 537
Hyper acute conjunctivitis, 129
Hyper braic oxygen, 484
Hyper fluorescence, 415, 443, 444
Hyper mature cataract, 277, 701
Hyper parathyroidism, 215
Hyper phoria, 644
Hyper vitaminosis A, 522
Hyperacusia, 82
Hyperbaric oxygen, 487, 728
Hyperemia, 111, 422
Hyperemia of conjunctiva, 115
Hyperlysinemia, 45, 306, 314
Hypermetropia, 163, 215, 237, 304,
342, 401, 405, 444, 466, 515, 519,
546, 555, 642, 644, 650, 685
Hypermetropic astigmatism, 519
Hypermetropic eye, 447
Hypermetropic refraction, 307
Hypermetropic shift, 451
Hypernephroma, 408
Hyperosmotic agents, 211, 372, 377
Hyperphoria, 648
Hypersecretory, 370
Hypersensitivity, 138
Hypertelorism, 40, 642, 681, 678, 695
Hypertension, 119, 257, 374, 425
Hypertensive retinopathy, 424, 434
Hyperthyrodism, 80
Hypertropes, 68, 587, 610, 616, 633,
650
Hypertrophic scar, 445
Hyphae, 759, 760, 763
Hyphaem, 61, 208, 243, 247, 251, 248,
325, 326, 354, 384, 730
Hypo fluorescence, 274, 415, 443
Hypo phoria, 644
Hypo plasia of retina, 401
Hypo vitaminosis B, 169
Hypoangulated, 693
Hypodontia, 338
Hypoethesia of cornea, 201
Hypogenitalism, 439
Hypoglycaemia, 16, 299, 315
Hypoglycaemic cataract in infant, 301
Hypoglycemia, 767
Hypogonadism, 624
Hypoparathyroidism, 299, 301, 522,
762
Hypopituitarism, 711
Hypoplasia, 609, 621
Hypoplasia of disc, 20
Hypoplasia of macula, 8, 723
Hypoplasia of maxilla, 338, 682
Hypoplasia of optic nerve, 17
Hypoplasia of the macula, 410
Hypoplasia of the optic disc, 516
Hypoproteinaemia, 44, 118
Hypopyon, 26, 129, 130, 174, 175, 177,
178, 184, 190, 192, 196, 201, 203,
208, 241, 244, 247,248, 253, 265,
268, 271, 280, 730, 735, 736, 742,
766
Hypopyon in fungal corneal ulcer,
181, 182
Hypopyon in fungal ulcer, 181
Hyposthesia, 752
Hyposthetic cornea, 197
Hypotelorism, 678, 683, 695
Hypothalamic syndrome, 711
Hypothalamus, 73, 711
Hypothesia, 607
Hypothesia of cornea, 175
Hypothyroidism, 762
Hypotony, 171, 247, 252, 269, 272,
335, 342, 359, 453, 532, 525
Hypotropia, 68, 611, 616, 617
Hypotropic, 609, 610
Hypovitaminosis, 50, 104, 404
Hypoxia, 169
Hyppyon, 270, 277
Hysteria, 584
I
Iatrogenic, 183, 190, 281, 361, 475
INDEX 797
Iatrogenic causes of non healing
corneal ulcers, 183
Iatrogenic conjunctivitis, 149
Iatrogenic disease, 277
Ibuprofen, 142, 257
Icthyosis, 439
Idiocy, 680
Idiopathic iridocylitis, 272
Idiopathic iritis, 269
Idiopathic optic neuritis, 525
Idoxuridine, 149, 197
Ill fitted contact, 173
Imidazoles, 205
Immature follicles, 134
Immediate hypersensitivity, 760
Immune ring, 196
Immune suppressives drugs, 270
Immuno fluorescent assays, 743
Immuno fluorescent monoclonal an-
tibodies, 136
Immuno fluorescent stain, 209
Immuno suppression, 196
Immuno suppressive drugs, 272, 760
Immunoglobin, 49, 264, 410
Immunosuppression, 191
Immunosuppressive drugs, 78, 275
Imprint cataract, 296
Imuno potentiating drugs, 198
In flow of aqueous, 341
In KOH solution, 204
In posterior chamber, 335
Inborn errors of metabolism, 13
Incarcerated, 186
Incarcerated in the pupil, 367
Incarceration of uvea, 167, 273
Inclusion bodies, 127, 129
Inclusion body conjunctivitis in
adults, 757
Inclusion body conjunctivitis of new
born, 757
Inclusion conjunctivitis, 12, 127, 173
Incominant, 649, 651
Incongruous, 583
Incongruous homonymous, 509
Incontenentia pigmenti, 476, 480, 484
Increase in corneal thickness, 167
Increased corneal curvature, 25
Increased thickness of cornea, 212
Incubation of nasolacrimal duct, 91
Incubation period, 127, 134
Incyclophoria, 645, 649
Indentation tonometer, 341
Index hypermetropia, 556
Index myopia, 302, 311, 554
Index myopia curvature myopia, 548
Indirect astigmatism, 564
Indirect fluorescent anti body test,
431
Indirect gonioscope, 342
Indirect ophthalmoscope, 150, 245,
246, 408, 412, 413, 451, 551, 552,
553, 724
Indiscriminate use of steroid, 183
Indocyanine green, 276, 416
Indocyanine green angiography, 416
Indolent, 177, 208
Indolent corneal ulcer, 203
Indolent plastic iridocyclitis, 273
Indomethacin, 142, 257, 440
Induced conjunctivitis, 142
Infantile, 2, 442
Infantile botulism, 729
Infantile esotropia, 614, 616, 621, 624,
633, 639, 640, 661, 662, 663
Infantile glaucoma, 168, 344
Infantile nucleus, 292
Infarction in the optic nerve, 523
Infected bleb, 280
Infected chalazion, 53, 97
Infected dermoid, 709
Infection, 198
Infectious mono necleosis, 23, 126,
759
Infective conjunctivitis, 144
Infective iritis, 269
Infective mononucleosis, 96
Infentile glaucoma, 353
Infentile strabismus, 260
Inferior colliculus, 603
Inferior division, 602
Inferior fornix, 112
Inferior meatus, 90, 100
Inferior nasal concha, 89
Inferior oblique, 72, 395, 502, 593,
594, 597, 598, 601, 616, 674, 676,
681
Inferior oblique muscles, 675
Inferior oblique over action, 613, 615,
616, 653, 655
Inferior oblique palsy, 610
Inferior oblique under action, 42
Inferior ophthalmic vein, 398, 414,
677
Inferior orbital artery, 599
Inferior orbital fissure, 676, 677
Inferior orbital margin, 34
Inferior orbital rim, 694
Inferior osteum of the duct, 90
Inferior rectus, 72, 593, 598, 599, 601,
616, 674, 676
Inferior rectus muscle paresis, 613
Inferior rectus muscles palsy, 616
Inferior rectus palsy, 610, 615
Inferior turbinate, 101
Inflamation, 160
Inflammation of retina, 428
Inflammatory bowel disease, 258
Inflammatory infiltration, 188
Influenza, 132, 240, 400, 429, 741, 758
Infra orbital canal, 674
Infra orbital fissure, 674
Infra orbital foramen, 674
Infra orbital glands, 87
Infra orbital groove, 674
Infra orbital margin, 673
Infra red range, 416
Infra red sensitive video camera, 416
Infraduction, 595
Infrared, 577
Infundibular, 462
Inherited genetic, 12
Inner barrier, 423
Inner germinal layer, 769
Inner neuroblast, 399
Inner nuclear layer, 395, 396
Inner plexiform layer, 395, 397
Insect bite, 51
Insect larvae, 764
Intensity modulated, 417
Inter cavernous part, 602
Inter ferometer, 416
Inter limiting membrane of the
retina, 456
Inter marginal strip, 38, 144
Inter muscles attachments, 675
Inter neural branches, 397
Inter neural part, 397
Inter nuclear ophthalmoplegia, 76,
77, 530, 605, 614, 619, 630, 634
Inter nuncial neurons, 503
Inter orbital distance, 673
Inter palpebral aperture, 34, 74, 127
Inter palpebral fissure, 53, 67, 71,
117, 210, 214, 550
Inter palpebral fissures, 14
Inter palpebral zone, 249
Inter peduncular space, 602
Inter pupillary distance, 639
Inter retinal, 493
Inter retinal haemorrhages, 425
Inter retinal separation, 447
Inter scleral nerve loop, 722, 723
Inter vaginal part, 397
Inter vitreal bleeding, 462
Inter-palpebral aperture, 41
Inter-palpebral fissure, 547
Intera ocular calcification, 283
Intercalary staphylomas, 724
Interferon, 713
Interior turbinate, 89
Intermediate host, 152, 153, 769
Intermediate uveitis, 242, 271
Intermittent cyclic esotropia, 623
Intermittent exotropia, 648, 659, 664,
665, 666
Intermittent heterotropia, 642
Internal carotid, 458, 512, 604, 606,
608
Internal carotid artery, 502, 705
Internal filtration procedures, 381
Internal hydrocephalus, 541
Internal limiting layer, 395
Internal limiting membrane, 279, 397
Internal ocular muscles, 78
Internal ophthalmoplegia, 510, 563,
605, 728, 730
Internal pterygoid muscle, 72
Internal temponade, 456
International colour code, 176
Interpalpebral aperture, 105
Interpalpebral fissure, 41, 51, 282
Interstitial, 206
Interstitial keratitis, 25, 26, 136, 147,
168, 190, 196, 216, 239, 262, 328,
348, 576, 732, 741, 746, 758, 767,
768
Interstitial kerato conjunctivitis, 144
Intestinal helminths, 147
Intorsion, 597, 599
Intortors, 597
Intra cameral antibiotics, 185
Intra canalicular part, 511
Intra cavernous, 602
Intra cavernous aneurysm, 697
Intra cellular, 727, 733
Intra clinoidal aneurysm, 696
Intra conal lesions, 675
Intra cranial calcification, 57, 755
Intra cranial extension, 474, 481
Intra epithelial epithelioma, 54
Intra mural part, 89
Intra ocular calcification, 474
Intra ocular foreign bodies, 283, 454,
460
Intra ocular haemorrhage, 698
Intra ocular implant, 270
Intra ocular malignancy, 241
Intra ocular mass, 697
Intra ocular pressure, 143
Intra ocular tension, 474
Intra ocular toxocariasis, 407
Intra ocular tumours, 295, 417
Intra ocular vasculature, 6
Intra operative hyphaema, 57, 351
Intra orbital canal, 694
Intra orbital nerve, 677
Intra retinal cysts, 452
Intra thorasic pressure, 117
Intra uterine factors, 12
Intra uterine ocular disorders, 12
Intra vitreal cyst, 153
Intra vitreal injection, 282
Intra vitreous, 727
Intra-ocular toxocariasis, 433
Intracapsular lens extraction, 291
Intracellular diplococci, 127
Intracerebral bleeding, 522
Intracerebral components, 628
Intraconal, 539, 769
Intraconjunctival bleeding, 118
Intracorneal ring, 554
Intracranial calcification, 260, 431
Intracranial pressure, 510
Intractable diplopia, 590
Intractable nystagmus, 316
Intraneural, 539
Intraocular calcification, 407, 696
Intraocular foreign body, 259
Intraocular growth, 116
Intraocular lens, 700
Intraocular part, 510
Intraocular pressure, 160, 161, 175,
183, 245, 341, 342
Intraocular tension, 346
Intraocular tumours, 13
Intraoperative mydriasis, 257
Intraorbital, 541
Intraorbital distance, 695
Intraorbital meningioma, 688
Intraorbital parasitic cyst, 688
Intraorbital part, 511
Intrauterine infection, 13, 168, 193
Intrauterine trauma, 352
Intravenous ocular hypotensive
agents, 378
Introseous, 89
Inverse argyll robertson pupil, 509
Inverse Duanes syndrome, 608
Inverse epicanthic fold, 71, 678
inverse epicanthus, 678
Inverse Marcus Gunn jaw winking,
72
Inverse occlusion, 589
Inverse retinitis pigmentosa, 438
Inverted follicular keratosis, 54
Investigations in papilledema, 524
Iodides, 190, 198
Iodine 125, 198, 481
Iodoxuridine, 196
IOFB, 417
IOL implant, 169
IOL in children, 320
Ipsilateral, 601
Ipsilateral antagonist, 625
Ipsilateral hypertropia, 611
Ipsilateral lateral rectus, 604
Ipsilateral lateral rectus palsy, 82
Ipsilateral sixth nerve palsy, 96
Iridectomics, 251
Iridectomy, 187, 228, 278, 284, 358,
561
Iridencleisis, 379, 381
Iridium 192, 481
Irido cyclectomy, 284
Irido dialysis, 325
Iridocorneal adhesion, 339
Iridocorneal contact, 247
Iridocorneal dysgenesis, 190, 337, 352
Iridocorneal endothelial cells, 337
Iridocyclitis, 201, 228, 239, 247, 261,
269, 326, 328, 366, 376, 502, 555,
732, 742, 746, 751, 753, 768, 772
Iridodiastasis, 234
Iridodonesis, 162, 302
Iridoplegia, 28, 125, 345
Iridotomies, 251
Iridotomy, 302, 359, 380, 381, 382,
385
Iris, 4, 15, 181, 503, 227, 624
Iris atrophy, 201, 249, 251, 277, 353,
755
Iris bombe, 244, 250, 271, 354, 360,
501
Iris border cysts, 374
Iris claw lens, 321
Iris clip IOL16, 26
Iris clip lens, 309
Iris collarette, 27
Iris fixated, 563
Iris hypoplasia, 299, 747
Iris incarceration, 169
Iris inclusion procedures, 278
Iris inclusion surgeries, 273, 350
Iris inclusion surgery, 350
Iris lens diaphragm, 186, 358
Iris mesoderm, 336
Iris neovascularisation, 493
Iris nodules, 249, 277, 284
Iris pigments, 360
Iris ruff, 227
Iris sphinter, 4
Iris stroma, 10, 233
Iritis, 208, 239, 261, 732, 742, 768, 772
Iritis glaucomatosa, 240
Irradiation, 61
Irregular AC, 189
Irregular astigmatism, 114, 168, 189,
210, 211, 345, 346, 351,564, 565,
685
Irregular myopic astigmatism, 163
Irregular pupil, 189
Irritating fluid, 279
Ischaemia, 514
Ischaemic optic neuropathy, 584
Ischamia of iris, 357
Ischemic, coagulative necrosis, 478
INDEX 799
Ischemic maculopathy, 267, 749
Ischemic necrosis, 201
Ischemic necrosis of iris, 52
Ishihara, 579
Isocoria, 508
Isolated sixth nerve palsy, 618
Isoniazide, 263, 526, 529
Isosorbide, 372, 378
Itching, 141
J
J. shaped sella, 711
Jacksonian epileptoform attack, 57
Jacksons cross cylinder, 567
Japanese, 575
Jaundice, 117, 740
Jaw winking, 43
Jerk nystagmus, 628, 629
Jews, 440
Jones I, 101
Jones I and II test, 93, 101, 176
Joules, 382
Juglar vein, 692, 704
Juvenile, 41, 238, 447
Juvenile diabetes, 179, 301, 426,427,
462, 537
Juvenile diabetes mellitus, 533
Juvenile glaucoma, 353, 370, 576
Juvenile myasthenia, 76
Juvenile or idiopathic retinoschisis,
403
Juvenile pilocytic astrocytoma, 539
Juvenile primary open angle glau-
coma, 370
Juvenile Reiters syndrome, 258, 268
Juvenile retino schisis, 426, 435, 447,
465, 484
Juvenile rheumatoid arthritis, 173,
240, 252, 258, 268, 272, 328
Juvenile spondylitis, 258, 268
Juvenile xanthogranuloma, 54, 283,
284, 368, 689
Juxta canalicular meshwork, 336
Juxta papillary, 239, 259
Juxtapapillary chroiditis, 584
K
Kajal, 117, 133, 218
Kala azar, 767
Kanamycin, 730
Kandori syndrome, 435
Kaposi sarcoma, 267, 750
Kayser-Fleischer ring, 329
Kearns, 624
Keoppe nodule, 249
Keratanisation, 26, 148
Keratanisation of cornea, 148
Keratanised, 111
Keratectasia, 166, 565
Keratic precipitates, 242
Keratitis, 24, 46, 50, 83, 91, 97, 172,
188, 194, 201, 208, 762, 742,
743, 744, 745, 746, 750, 758, 761,
763, 767
Keratitis in children, 206
Keratitis meta herpetica, 198, 755
Keratitis sicca, 761
Kerato acanthameba, 54, 209
Kerato conjunctival involvement, 201
Kerato conjunctivitis, 133, 205, 745,
756
Kerato conjunctivitis sicca, 104
Kerato malacia, 25
Kerato prosthesis, 144
Keratoacanthamoeba, 191
Keratocele, 169, 174, 187
Keratoconess, 25
keratoconjunctivitis, 763
Keratoconus, 142, 143, 162, 163, 166,
167, 168, 169, 171, 306, 308,335,
437, 466, 548, 554, 565, 723
Keratocytes, 167, 174, 185, 188
Keratoglobus, 26, 162, 163, 166, 314,
466, 548
Keratomalacia, 117, 184, 187, 191,
216, 217, 219, 440
Keratometer, 164, 166, 566
Keratonisation of conjunctiva, 83
Keratopathy, 134, 172, 207, 248
Keratopathy in chronic anterior
uveitis, 248
Keratoplasty, 166, 172, 185, 189, 197,
203, 212, 215, 328
Keratoscope, 164, 166
Keratouveitis, 52, 239
Kerb-cycle, 161
Kertoconous, 314
Kertometer, 14
Kesten baum procedures, 635
Ketaconazole, 209
Ketamine, 347
Ketarolac, 257
Ketoconazole, 205
Ketorolac tromethamine, 140, 142
Key hole, 186, 189, 229
Khaki coloured pseudo hypopyon, 365
Kinetic, 649
Kinin system, 257
Kjers dominant juvenile neuropathy,
537
Kjers juvenile optic neuropathy, 538
Kjers optic atrophy, 535
Klebsiellapneumonae, 191
Klein keratoscope, 164
Klumkes paralysis, 73
Koch Weeks bacillus, 23
Koch-Weeks, 122, 123, 175, 731
Koeppe, 273
Koeppe lens, 366, 413
Koeppe nodules, 263
Komotos tetrad, 42, 40, 263
Koplic spots, 50, 132, 748
KPs, 271
Krabbes disease, 529, 530
Krauses gland, 87, 113
Krimsky test, 652
Krypton, 383
Krypton laser, 384
L
L. donovani, 768
Labyrinthine pontine path, 596, 601
Laceration of conjunctiva, 24
Lacquer cracks, 553
Lacrimal, 18, 200, 673
Lacrimal apparatus, 86
Lacrimal artery, 87, 114, 597
Lacrimal bone, 89
Lacrimal branches, 114
Lacrimal canule, 100
Lacrimal crest, 89, 973
Lacrimal drainage system, 88
Lacrimal ducts, 87
Lacrimal facia, 88
Lacrimal fistula, 100
Lacrimal gland, 7, 9, 86, 94, 96, 98,
597, 673, 675, 704
Lacrimal gland growth, 15, 692, 772
Lacrimal gland tumours, 692
Lacrimal nerve, 87, 674, 677
Lacrimal nucleus, 87
Lacrimal out flow, 101
Lacrimal passage, 7, 9, 15, 94, 154,
736, 772
Lacrimal puncta, 111
Lacrimal sac, 7, 15, 86, 88, 89, 110,
149, 191
Lacrimal sac in rhinosporidiosis, 150
Lacrimal scintilography, 93, 102
Lacrimal syringing, 93, 100, 102
Lacrimal vein, 87
Lacrimation, 60, 82, 96, 97, 106, 141,
164, 175, 178, 195, 272, 273, 347,
689
Lactic acidosis, 767
Lactoferrin, 102
Lactoferrinassay, 106
Lactophenol cotton blue, 204, 209
Lactose, 299
Lacus lacrimalis, 35, 98
Lagophthalmos, 18, 22, 36, 41, 61,67,
81, 82, 83, 104, 111, 173, 179,
184, 684, 685, 753
Lamelar keratoplasty, 165
Lamellar, 211, 296, 297
Lamellar cataract, 16, 297, 301, 318
Lamellar corneal graft, 249
Lamellar graft, 171
Lamellar keratectomy, 205, 210, 215,
270
Lamina, 3, 534
Lamina cribrosa, 5, 510, 513, 533, 720
Lamina fusca, 721
Landolts broken C, 31
Lanellar, 189
Langerhans cell cytosis, 713
Langs syndrome, 656
Large ciliary staphyloma, 690
Large cloudy cornea, 347
Large penetrating injuries cause, 325
Larva, 432
Larval conjunctivitis, 154
Larval endophthalmitis, 153, 433
Laryngeal muscle, 76
Laryngitis, 748
Lasek, 384, 554
Laser, 61, 181, 189, 323, 349, 455, 464,
756
Laser burns, 535
Laser capsulotomy, 354, 453
Laser cyclo ablation, 381
Laser in buphthalmos, 351
Laser in glaucoma, 381
Laser in situ keratomileusis, 554
Laser iridotomy, 312, 358, 379, 380,
383
Laser phopcoagulation, 266, 273, 354,
409, 433, 447, 487, 489, 516, 764,
Laser scarring to reduce, 385
Laser thermal keratoplasty, 559
Laser trabeculoplasty, 355, 356, 359
Lasik, 384, 554, 559
Lasing cavity, 382
Latanoprost, 252, 255, 355, 365, 366,
372, 373, 376
Late onset post surgical endo-
phthalmitis, 280
Late papilledema, 523
Late retinopathy, 485
Late rosette cataract, 325
Late venous phase, 415
Latent hypermetropia, 556
Latent nystagmus, 586, 616, 629, 630,
631
Latent squint, 614, 642, 643
Lateral canthal ligament, 682
Lateral canthotomy, 715
Lateral canthus, 35, 89, 115, 420
Lateral check ligament, 676
Lateral fornix, 112
Lateral geneculate body, 513, 514,
529, 532
Lateral nasal process, 89, 677
Lateral orbital tubercle, 36
Lateral orbitotomy, 541, 676
Lateral rectus, 87, 593, 596, 618, 623,
670, 674, 745
Lateral sinus thrombosis, 522, 743
Lateral tarsorrhaphy, 83, 621
Lateral view, 693
Lateral wall, 602
Lattice, 211, 553
Lattice degeneration, 445, 449, 451,
457, 464, 487, 550
Lattice degeneration of the retina,
446
Lattice dystrophy, 211
Laurence Moon Biedl Bardet Syn-
drome, 438, 439, 576
Layers of retina, 395
LDH, 283
Learning difficulty, 581
Leber congenital amaurosia, 21, 419,
537, 628, 630
Lebers amaurosis, 17
Lebers disease, 537
Lebers familial amaurosis, 163
Lebers miliary aneurysm, 405, 406,
476, 477
Lebers optic atrophy, 535
Lebers optic neuritis, 526, 527, 537,
538
Legal blindness, 410
Leishmania, 764, 767
Lens, 9, 15, 503, 546
Lens capsule, 2, 235
Lens epithelium, 291
Lens extraction, 273, 315
Lens fibbers, 291
Lens in the sulcus, 309
Lens induced uveitis, 277, 683
Lens iris diaphragm, 56
Lens pit, 2
Lens plate, 1, 2, 293
Lens protein, 277
Lens suture, 292
Lens vesicle, 2, 161, 291, 293
Lensectomy, 28, 272, 320, 488, 683
Lenticonus, 31, 302, 548, 554, 564
Lenticular, 483
Lenticular changes in chronic ante-
rior uveitis, 252
Lenticular metabolism, 330
Lenticular opacity, 585
Lentiglobus, 302
Lepromatous nodules, 249
Leprosy, 62, 81, 121, 171, 172, 173,
206, 240, 258
Leprotic lagophthalmos, 62
Leptospira, 740
Leptospiracmia, 742
Leptospirosis, 258, 741
Lesser circle of the iris, 228
Lesser wing of sphenoid, 36, 673, 694
Lettercrswie disease, 713
Leucokoria, 473, 489, 515
Leucoma, 185, 186, 188
Leucoma adherence, 127, 174, 175,
185, 186, 188
Leucomatous, 174
Leucoria, 483
Leucotriens, 138
Leucovorin, 261
Leuddes transparent scale, 691
Leukaemia, 24, 44, 119, 283, 368, 422,
424, 425, 463, 476, 477, 483, 491,
522, 542, 675, 686, 689, 690, 698,
706, 707, 708, 709, 762
Leukaemia deposit in orbit in chil-
dren, 711
Leukaemic deposits, 708
Leukaemic infiltration, 705
Leukodystrophies, 530
Levamesol, 198
Levator, 4, 597, 600
Levator aponeurosis, 675
Levator function, 79
Levator palpebral muscle, 675
Levator palpebral superior, 35, 42, 67,
609, 676, 697
Fevator resection, 78
Levo cyclo version, 595
Levo version, 595, 629
Levobunotol, 375
Levodopa, 591
Lid, 44, 86, 153, 154, 702, 738
Lid abscess, 68, 744
Lid deformity, 179
Lid fatigue, 76
Lid hygiene, 147
Lid infiltration, 479
Lid lag, 42, 74, 80
Lid margin, 101
Lid of deformity, 183
Lid retraction, 19, 20, 23, 67, 80, 127,
374, 375, 509, 614, 681, 690
Lid vescicles, 194
Ligament of lockwood, 598
Ligenous conjunctiva, 149
Light adapted trough, 420
Light brightness, 520
Light bulb, 407
Light near dissociation, 509, 511, 614
Light reflex, 18, 81, 503, 601
INDEX 801
Ligneous conjunctivitis, 126, 149
Limbal, 141
Limbal blood vessels, 160
Limbal changes in trachoma, 136
Limbal dermoid, 12, 19, 25, 121, 165,
168, 190, 565, 679
Limbal papillae, 120, 166
Limbal vasculitis, 196
Limbitis, 208
Limbus, 3, 25, 105, 110, 111, 114, 144,
159, 171, 273, 336, 676, 720
Limbus parallel tears, 449
Lindane, 46
Liner accelerator external beam ra-
diation, 481
Lingua plicata, 83
Lingual palsy, 62
Lipid, 98, 216
Lipid abnormality, 103
Lipid degeneration, 201
Lipid fraction, 144
Lipid layer, 102
Lipid layer of the tear, 37
Lipidosis, 423, 439
Lipofucin, 416, 443, 444
Lipoidal ultra fluid, 102
Lipoprotein, 103
Liquefaction, 252
Liquefaction of vitreous, 449, 553
Liquid, 381
Lisch nodule, 710
Listers perimeter, 652
Live worm, 154
Liver, 153, 769
Loa-Loa, 765, 772
Loasis, 765, 772
Local anaesthetic, 191
Local retinol, 220
Local steroids, 256
Lock jaw, 728
Lockwood ligament, 599, 676
Long acting steroids, 275
Long acting sulpha, 47
Long ciliary nerves, 73, 502, 511
Long posterior ciliary arteries, 232
Long term drift, 376
Long term effect of extra ocular mus-
cle palsy, 624
Longitudinal fibres, 230
Loss of AC, 174
Loss of accommodation, 255, 273
Loss of foveal reflex, 444
Loss of sensation, 179, 183
Louis bar syndrome, 55
Low IQ, 439
Low vision aids, 238, 538
Lower division of the oculomotor
nerve, 593
Lower formix, 56, 110, 119
Lower puncta, 35, 88, 100
Lower tarsal conjunctiva, 119
Lowes syndrome, 301, 315, 352
LPS, 74
Lridocorneal dysgenesis, 212
Lrisatrophy, 212
Lubricants, 195, 211
Lubricating drop, 211
Lumbo peritoneal shunt, 525
Lumigan, 372
Lung, 153, 731, 769
Lyme disease, 82, 253, 258, 740, 742
Lymphadenitis, 733
Lymphadenopathy, 123, 131, 132,
194, 268, 402, 740
Lymphangioma, 687, 688, 690, 698,
712
Lymphangitis, 764
Lymphatic, 151
Lymphatic cyst, 122, 152
Lympho granuloma, 144
Lympho granuloma venerum, 133
Lympho sarcoma, 709
Lymphoblastic leukaemia, 711
Lymphocytes, 121, 228, 276
Lymphogranuloma venereum, 206,
758
Lymphoid tissue, 113
Lymphokinase, 257
Lymphoma, 402, 607, 762
Lypophilic, 375
Lysinedehydrogenase, 314
Lysosmal enzyme, 257
Lysosomes, 102, 110, 180, 395
Lysozymeassay, 106
M
Macro-phages, 181, 242, 243, 364, 367
Macropsia, 577
Macrostomia, 682
Macula, 5, 15, 185, 188, 231, 246, 259,
394, 401, 431, 490
Macular, 174
Macular coloboma, 410, 535
Macular cyst, 416, 442
Macular dystrophy, 212, 420
Macular edema, 242, 253, 269, 271,
416
Macular granuloma, 433
Macular hole, 247, 416, 425, 442, 449
Macular hypoplasia, 115, 628, 630
Macular lesion, 577, 634, 650
Macular opacities, 186
Macular pigmentation papillitis, 253
Macular scar, 247, 630, 665
Macular star, 58, 409, 424, 493
Macules, 148
Maculo papular, 200, 268
Maculopapillar bundle, 246, 247
Maculopathies, 668
Maculopathy, 375, 416, 747
Madarosis, 45, 200, 708, 762
Maddox double prism, 645, 646, 649
Maddox rod test, 643, 645
Maddox tangent, 645
Maddox wing, 644, 645, 646, 648
Maggots, 154
Magnetic resonance imaging, 698
Magnification, 560, 562, 577
Magsulf, 93
Main elevator, 67
Main lacrimal gland, 103
Major arterial circle, 232
Major circle of iris, 232
Mal-absorption syndrome, 219
Malaise, 747
Malaria, 189, 206, 219, 766
Malignant epithelial tumours, 87
Malignant glaucoma, 357
Malignant glioblastoma, 710
Malignant hypertension, 425
Malignant melanoma, 59, 283, 382,
415
Malingeing, 32, 421, 584, 632
Malnutrition, 144, 179, 278, 576
Management of accommodative
esotropia, 660
Management of acute dacryocystitis,
92
Management of aphakia in children,
561
Management of ARC, 640
Management of chalazia, 48
Management of glaucoma in penetrat-
ing injury, 355
Management of glaucoma secondary
to total hyphaema, 364
Management of herpes zoster
ophthalmicus, 202
Management of hypopyon corneal
ulcer, 181
Management of impending perfora-
tion, 184
Management of infective corneal ul-
cer, 176
Management of nystagmus, 634
Management of optic neuritis, 528
Management of primary
buphthalmos, 348
Management of retinopathy of prema-
turity, 487
Management of rhegmatogenous
retinal treatment, 455
Management of stye, 47
Management of trachoma in children,
136
Management of traumatic cataract,
327
Management of uveitis, 253
Managementof xerodermapig-
mentosum, 60
Mandibulo facial, 13, 18, 38
Mandibulo facial dysostosis, 306, 682
Mangrove fly, 772
Manifest hypermetropia, 556
Manifest latent, 630
Manifest latent nystagmus, 633, 664
Manifest nystagmus, 615, 633, 664
Manifest squint, 569, 638, 640, 649
Mannitol, 151, 351, 358, 364, 525, 372,
715
Mannitol and urea, 378
Manosidosis, 301
Mantoux test, 264
Manz, 87
Marchesani syndrome, 306
Marcus Gunn, 28, 43, 72, 43, 450, 507,
508, 684, 686, 690,
Marcus Gunn jaw winking, 72
Marcus Gunn phenomenon, 43, 690
Marcus Gunn pupil, 450, 507, 508,
684, 686
Marfans, 311, 453
Marfans syndrome, 306, 307, 309,
446, 452, 464, 560
Marginal conjunctiva, 111
Marginal corneal phlycten, 146
Marginal keratitis, 123
Marginal corneal ulcers, 178
Masquerade, 241
Masquerade syndrome, 259, 283, 481
Mass reflex, 504
Massaging of the sac, 91
Mast cell inhibitor, 140, 142
Mast cell membrane, 138
Mast cells, 228
Mastcell stabiliser, 328
Mastoid, 620, 705, 743,
Mastoiditis, 743
Maternal diabetes, 315, 487
Maternal infection, 295
Maternal malnutrition, 315
Maternal rubella, 315
Maternal syphilis, 295
Maxilla, 89, 673, 674, 709
Maxillary antrum, 674
Maxillary artery, 87
Maxillary division, 200
Maxillary nerves, 602
Maxillary process, 89, 677
Maxillary sinus, 673, 694
Maxillo mandibular condensation,
600
Maxwell Lyons sign, 141
McCoy cells, 134, 136
Measles, 44, 47, 49, 87, 93, 106, 123,
132, 179, 189, 217, 219, 240, 529,
576, 745, 747
Measles vaccine, 219
Measurement of intraocular pressure,
341
Measurement of phoria for near, 646
Measurement of proptosis, 690
Measuring intra ocular pressure, 26
Mebendazol, 153
Mechanical capsulotomy, 278
Mechanical causes, 62, 81
Mechanical ptosis, 60
Medial, 601, 673
Medial canthal ligament, 35, 101
Medial canthus, 35, 88, 92, 112, 152,
420
Medial check ligament, 676
Medial fornix, 112
Medial long posterior ciliary artery,
6
Medial longitudinal fascicles, 605, 619
Medial longitudinal fibres, 604
Medial meatus, 89
Medial palpebral ligament, 89, 150
Medial rectus, 72, 504, 593, 596, 610,
623, 670, 676
Mediastinum, 77
Medical treatment, 60, 249
Medium, 382
Medrysone, 361
Medulated, 514
Medulated nerve fibers, 5
Medulla epithelioma, 480
Medullary, 59
Medullo epithelioma, 259, 283, 284,
476, 483, 491
Medulo blastomas, 478
Meesmanns, 210
Megahertz, 416
Megalo cornea, 18, 25, 159, 161, 162,
166, 305, 308, 314, 321, 335, 549,
552
Megalo papilla, 513
Meibomian, 37, 104
Meibomian cell carcinoma, 54
Meibomian gland, 7, 9, 37, 40, 48, 102,
104, 115
Meibomianitis, 104, 730
Melanin, 383, 384, 395, 416
Melano phores, 112
Melanocyte,4, 228, 278, 401, 683, 722
Melanocytoma, 542
Melanosis bulbae, 353
Melanosomes, 401
Melanotic tumours of conjunctiva, 117
Melkerrson Rosenthal syndrome, 81,
83
Mellar corneal graft, 210
Membrane formation over macula,
247
Membranous cataract, 298, 488
Membranous conjunctivitis, 63, 759
Meninges, 56, 154, 511, 674
Meningiococcus, 23, 160, 173, 175,
734
Meningioma, 276, 539, 675, 687
Meningitis, 97, 522, 526, 576, 703,
726, 731, 736, 742, 743, 744, 752,
770
Meningo encephalitis, 526, 753
Meningo encephalocele, 678, 679, 687,
688, 691
Meningocele, 92, 678, 687, 688, 692
Mental retardation, 309, 330, 402,
439, 681
Mercuro chrome, 101
Meridional amblyopia, 586
Mesenchymal, 485, 533
Mesoderm, 10, 19, 26, 27, 88, 161, 233,
305, 337, 338, 352, 458, 539, 677,
722,
Mesopic, 418
Mesopic vision, 586
Metabolic cataract, 316
Metabolism, 160
Metachromatic leuco dystrophy, 443
Metaherpatic keratitis, 195, 199
Metamorphopsia, 246, 276, 411, 423,
450, 516, 574, 577, 764
Metastasis, 278, 473
Metastatic endophthalmitis, 476, 730,
760
Metastatic neuroblastoma, 705, 706
Metastatic retinitis, 476, 483
Metastatic uveitis, 735
Metazoa, 764
Methacholine, 511
Methazolamide, 377
Methicillin, 184, 738
Methionine, 309
Metho trex ate, 706
Methonine, 311
Methotrexate, 258, 482, 709
Methyl prednisolone, 536
Metiprantol, 375
Metronidazole, 526, 729
Meyers loop, 583
Miconazole, 205
Micro aneurysm, 408, 493, 749
Micro chondrian mutation, 624
Micro cornea, 26, 556, 723
INDEX 803
Micro dendrile, 194
Micro dendritic ulcer, 195
Micro erosion, 141
Micro filarae, 771
Micro pannus, 127, 170
Micro strabismus, 587
Micro surgical extra capsular cataract
extraction, 322
Micro-aneurysms, 409
Micro-cornea, 163, 335
Micro-dontia, 338
Micro-ophthalmos, 335
Micro-strabismus, 663
Microaneurysm, 267, 485
Microbial conjunctivitis, 122
Microblepharon, 41
Microcephally, 19, 25, 27, 159, 166,
260, 314, 558, 680, 747, 755,
Microdendrites filamentary keratitis,
51
Microfilaria, 772
Micrognathia, 682, 683
Microphakia, 162, 367
Microphthalmia, 235, 403, 487, 678,
683, 687, 688
Microphthalmos, 8, 12, 16, 18, 19, 25,
26, 27, 39, 68, 115, 162, 163, 166,
299, 316, 339, 352, 353, 437, 457,
549, 556, 558, 622, 690, 696, 722,
747
Microphthalmos with cyst, 8, 9, 696,
722
Microphtlamia, 679
Micropsia, 577
Microspherophakia, 29, 301, 302, 307,
311, 314, 330
Microtropia, 641, 654, 658, 663
Microvilli, 103, 159
Microvilli of retinal pigment epithe-
lium, 447
Mid brain, 503, 521, 602
Mid brain lesions, 73
Mid limbal line, 112, 336
Mid pons, 604
Mid venous phase, 415
Middle cerebral, 584
Middle cranial fossa, 512, 673, 674,
677, 694
Middle meatus, 674
Midline facial defects, 513
Migraine, 608
Mikuliczs syndrome, 87
Mild ptosis, 79
Miliary aneurysm, 406
Miliary tubercle, 263
Miliary tubercle of choroids, 263
Miliary tuberculosis, 732
Milk alkali syndrome, 215
Milk spilled over cobble stone, 141
Millard Gubler, 82, 619
Millers syndrome, 235
Mineral oils, 370
Miners nystagmus, 628, 630, 631, 633
Miniature toy test, 31
Minification, 577
Minocylin, 261
Minor circle of iris, 232
Minus lenses, 503
Miosis, 74, 186, 257, 262, 353, 373,
503, 608, 668, 735
Miotic and cyclotonic, 505
Miotic pupil, 229
Miotics, 229, 251, 255, 270, 273, 355,
365, 371, 373, 505, 555, 657, 660,
661
Mirror image, 304, 307
Missed foreign bodies in cornea, 183
Mitochondrial DNA, 537
Mitomycin C, 356, 366
Mitral valve prolapse, 163
Mitten drofs dot, 488
Mittendorf dot, 295, 296
Mittendrof s spot, 6
Mixed astigmatism, 565
Mixed nystagmus, 629
Mizuo phenomenon, 575
Mizuo sign, 575
Mobius syndrome, 20, 62, 81, 83, 620,
621, 624, 656
Moderate, 79
Moll, 47
Mollouscum contagiosum, 50, 123,
132, 172, 745, 756,
Molluscum, 44, 49, 173
Molteno valve, 338
Mongoloid obliquity, 41
Mongoloid slant, 670
Mono filament sutures, 144
Mono fixation syndrome, 663
Mono nucleosis, 756
Mono-ocular, 649
Monochromatism, 578
Monocular diplopia, 324
Monocular esotropia, 641
Moorens ulcer, 206
Morax-Axenfeld, 735
Moraxella, 735
Moraxella catarrhalis, 735
Moraxella lacunata, 735
Morbilli, 49
Morbilli virus, 748
Morning glory syndrome, 513, 515
Morphine, 506
Morphological changes in cornea, 166
Morphology, 165, 628
Motile, 740, 744
Motor imbalance, 629
Motor nystagmus, 587
Motor paralysis, 745
Movements of the eyes, 14
MRI, 416, 698
MRI induced blindness, 327
Mucin, 87, 98, 216, 217
Mucin deficiency, 103
Mucin layer, 102
Mucinous degeneration, 540
Mucinous discharge, 120
Muco cutaneous, 148
Muco purulent conjunctivitis, 148
Muco purulent discharge, 120, 123
Mucocele, 93, 675, 692, 696
Mucoid degeneration, 535
Mucolipidoses and cystinosis, 13, 25
Mucolipidosis, 168, 348, 741
Mucolytic, 106
Mucopoly saccharidoses, 19, 25, 168,
190, 216, 348, 352, 425, 439, 533,
721, 741
Mucopuralent, 91, 180
Mucopurulent conjunctivitis, 175,
177, 191, 192, 731, 758
Mucopurulent discharge, 24, 90
Mucorale, 762
Mucormycosis, 759, 762
Mucous, 176
Mucous plaque, 201
Mucus discharge, 95
Mucus plaque, 52
Mucus secreting conjunctivitis, 52
Mulberry molars, 740
Muller cells, 396
Mullers, 74
Mullers cell, 418
Mullers fibre, 396, 399
Mullers muscle, 8, 35, 36, 67, 76, 80,
81, 374, 511
Multi focal choroiditis, 750
Multicentric, 479
Multiceps, 153
Multifocals, 563
Multiple phlycten, 136
Multiple sclerosis, 283, 528, 529, 668,
699
Multiple styes, 53
Mumps, 87, 96, 132, 240, 745
Mumps influenza, 295
Mums, 529
Munsons sign, 164, 166
Muscae volitantes, 461, 551
Muscle cone, 511, 599, 675
Muscle dystrophies, 75
Muscle imbalance, 558, 570, 581
Muscle plane, 593
Muscles of face, 76
Muscles of mastification, 76
Muscular anomaly, 644
Muscular arteries, 232
Muscular dystrophies, 69
Mustard oil, 369
Mutation in mitochondrial DNA, 78
Mutation of genes, 436
Mutton fat, 241, 243, 249, 263, 742
Myasthenia, 69, 79, 82, 621, 624
Myasthenia gravis, 75, 505, 613
Myasthenia in mother, 16
Myasthenia of childhood, 76
Mycelia, 761
Mycelium, 759, 760
Mycetoma, 763
Mycobacteria, 267, 726, 750
Mycobacterium tuberculosis, 731, 750
Mycoplasma pneumonae, 148
Mydriasis, 257, 366, 374, 375, 552,
608, 735
Mydriatic, 180, 502, 505, 730
Mydriatic pupil, 229
Mydriatic cycloplegic, 503
Mydriatics, 229, 254
Mydricane, 255
Myelinated, 513
Myelinated nerve fibres, 514
Myeline sheath, 533
Myelitis, 97, 530
Myelogenous leukemia, 283
Myiasis, 725, 765
Myiasis externa, 773
Myiasis interna, 773
Myocardial infarction, 310
Myocarditis, 730, 742, 744, 758
Myogenic, 75
Myographic, 621
Myoneural junction, 75
Myopathies, 75, 276
Myopia, 162, 242, 248, 335, 342, 401,
404, 410, 423, 427, 436, 437, 443,
452, 515, 532, 575, 576, 644, 650,
712, 723
Myopia of prematurity, 555
Myopic, 159, 304, 546
Myopic astigmatism, 42, 371
Myopic crescent, 552
Myopic degeneration, 305, 415
Myopicaphakics, 452
Myopicpseudophakics, 452
Myositis, 705, 706
Myotonic dystrophy, 77, 330, 439, 624
Myxedema, 44, 118
N
N. meningitis, 122
N.D. YAG, 324
Naevus, 45
Naefus flammeus, 54
Nagels anomaloscope, 579
Nalidoxic acid, 522
Nanophthalmos, 8, 162, 163, 166, 352,
556, 558722, 723
Nanophthalmos, 8
Nasal, 114
Nasal cavity, 694
Nasal cord, 90
Nasal endoscopic surgery, 151
Nasal mucous, 97, 106
Nasal veins, 89
Naso lacrimal, 100
Naso lacrimalduct, 7, 34, 86, 88, 89
90, 92, 94, 98, 101, 110, 176, 673
Naso pharyngeal carcinoma, 607
Nasociliary, 73, 114, 200
Nasociliary nerve, 51 201, 502, 511,
674, 675, 677, 753
Nasolacrimal duct obstruction, 279
Nasopharyngeal, 620
Nasopharyngeal carcinoma, 759
Nasopharynx, 93, 110, 125, 149, 150
Natamycin, 205
Nathnagels syndrome, 606
National programme of prevention
of xerophthalmia, 219
Nausea, 77, 522
Nd-YAG, 61, 382, 383, 385
Nd-YAG cyclophto coagulation, 369
Nd-YAG posterior capsulotomy, 359
Near dysfunction, 649
Near reflex, 503, 504
Near sightedness, 547
Near synkinesis, 503
Near vision, 589
Nebula, 174, 185, 188
Nebular opacities, 185
Neck, 89
Necrosis of the retina, 750
Necrotising, 196
Necrotising retinitis, 429, 766
Necrotising retino choroiditis, 430
Nedocromil, 140
Nedocromil sodium, 142
Needing, 291, 452
Needling aspiration, 322
Neisseria catarrhalis, 735
Neisseria gonorrhoea, 122, 733
Neisseriaceae, 726
Neisseriae meningitidis, 734
Neissericeae, 129
Nematode, 154, 772
Nematode infection, 430
Nematode onchocerca volvulus, 771
Nematode toxocara, 770
Nematodes, 764
Nemesulide, 142
Neo natal herpes simplex, 53
Neo vascularisation of iris, 251
Neodymium, 382
Neodymium Yuttrium Aluminium
Garnet, 383
Neomycin, 124, 149, 179, 209
Neon, 383
Neonatal, 728
Neonatal chlamydiae disease, 133
Neonatal conjunctivitis, 127
neonatal dacryocystitis, 90
Neonatal gonococcal infection, 733
Neonatal HSV, 198
Neonatal hypoxia, 765
Neonatal infection, 13
Neonatal keratitis, 348
Neonatal myasthenia, 42, 71, 75
Neonatal nasolacrimal duct obstruc-
tion, 23
Neonatal sepsis, 739
Neonatal trachoma, 757
Neonate, 130, 133, 168, 538
Neostigmine, 505
Neovascular glaucoma, 354, 368, 488
Neovascular tuft, 485
Neovascularisation, 228, 247, 249,
251, 256, 357, 368, 406, 415,
422, 433, 474, 479, 488, 742
Nephritis, 44, 425
Nephrotic syndrome, 118
Nerve fibre layer, 395, 397
Nerve fibre layers of the retina, 431
Nerve to inferior oblique, 503
Nervous system involvement, 56
Neural control of dilator pupillae, 502
Neural control of ocular movements,
595
Neural control of constrictor pupillae,
502
Neural crest, 401
Neural ectoderm, 9
Neural fold, 1
Neural groove, 1
Neural plate, 1
Neural tube, 1, 398, 601
Neuritis, 415, 518, 519, 530
Neuro cysticercosis, 770
Neuro ectodermal, 227, 398
Neuro fibroma, 283
Neuro fibromatosis, 45, 151, 685, 691,
695, 696, 697
Neuro imaging, 528
INDEX 805
Neuro muscular, 75
Neuro neuritis, 529
Neuro retinitis, 421, 423, 428, 525,
532
Neuro sensory deafness, 439
Neuro tropic keratitis, 52, 104, 107
Neuro-syphilis, 750
Neuroblast, 601
Neuroblastoma, 44, 477, 478, 480,
512, 542, 686, 687, 688, 689,
690, 703, 707, 708
Neuroectoderm, 4, 227
Neurofibreomatosis, 54, 163, 283,
352, 539, 542, 710
Neurofibroma, 68, 565, 687, 688, 690,
696, 697, 698, 707
Neurogenic cause of lagophthalmos,
61
Neuroglia, 28, 518, 539, 613
Neurological involvement in herpes
zoster ophthal, 202
Neurological characteristics of fourth
nerve palsy, 611
Neurological lesions, 52
Neurological-internal ophthal-
moplegia, 32
Neuromyelitis optica, 528
Neuropathy, 28
Neuroretinitis, 526, 534
Neurosyphilis, 509, 668
Neurotic, 584
Neurotoxin, 728
Neurotrophic, 104, 175, 184, 216
Neutral density filter, 507, 586, 588
Nevi, 284
Nevus of ota, 352, 353
Nevusflammeus, 60, 61
New born cornea, 159
New born eyes, 14
New vessels, 342
Nictitating membrane, 112
Niemann-pick disease, 425, 442
Night blindness, 218, 401, 436, 440,
439, 465, 551, 574, 575
Night myopia, 555
night vision, 217, 373, 394
Nimesulide, 257
Nitrofurantoin, 730
Nocardia, 759, 763
Nocardia asteroides, 763
Nocardiosis, 760, 763
Nocturnal lagophthalmos, 81
Nodal point, 560
Nodular papillae, 141
Nodule, 147, 241, 249, 474
Nodule formation, 764
Nodule on the iris, 337, 479
Nodules at limbus, 147
Nodules of sarcoid, 249
Non accommodative acquired
esotropias, 662
Non accommodative esotropia, 614,
658
Non atopic, 138
Non cataractous white reflex, 13
Non comitant, 643, 649
Non conjugate, 14
Non fenestrated endothelium cells,
398
Non filamentous, 203
Non fluorescent, 424, 425
Non granulomatous, 201, 261, 263,
269, 271, 329, 738
Non healing corneal ulcer, 181
Non keratanised, 161
Non lactose fermenting, 743
Non motile, 738, 739
Non nucleated marginal zone, 399
Non pigmented coloboma, 410
Non pigmented epithelium, 334, 393
Non rhegmatogenous, 448, 453, 492,
515,
Non sporulating, 738, 739
Non steroidal anti inflammatory
drugs, 142, 257, 362
Non steroidal anti-inflammatory, 362
Non teratoid, 284
Non traumatic phacogenic glaucoma,
366
Non-fenestrated, 228
Non-gonococcal ophthalmia
neonatorum, 194
Non-granulomatous, 52, 241, 276, 750
Non-motile, 727
Non-myelinated, 511
Non-necrotising, 196
Non-pigmented ciliary epithelium,
233
Non-septate, 762
Nongonococcal ophthalmia
neonatorum, 133
Nongranulomatous pan uveitis, 267
Nontreponemal, 262
Nor adrenaline, 506
Nor epinephrine, 502
Normal macula in fluorescein
angiogram, 415
Normal retinal correspondence, 640
Norries disease, 476
Nose bridge, 678
Nosocomial, 737, 839
Notch coloboma, 233
Notching of lid margin, 51
Notching of the cup, 371
Notifiable disease, 126
NSAID, 140
Nuclear, 295
Nuclear sclerosis, 701
Nuclear-fascicular lesion, 612
Nuclei, 2
Nucleic acid, 744
Nucleus, 292, 547
Nucleus of the lens, 292
Null point, 629
Null zone, 401, 631
Nummular keratitis, 51, 201, 727
Nutrition of cornea, 160
Nutritional amblyopia, 586
Nyctalopia, 574, 575
Nystagmoid movements, 76
Nystagmus, 8, 16, 20, 21, 29, 32, 168,
172, 178, 185, 186, 189, 234, 236,
237, 260, 316, 319, 323, 402, 410,
420, 439, 441, 442, 465, 513, 517,
538, 541, 549, 563, 575, 580,
596,628, 632, 656, 666, 680, 681,
683, 723, 734, 745, 747, 766
Nystagmus blockage syndrome, 621,
633
Nystagmus blocking esotropia, 656,
663, 664
Nystatin, 205
O
Obesity, 541, 711
Object of regard, 594
Objective angle of deviation, 639
Objective angle of squint, 639
Obligate aerobe, 192
Obligate intra cellular, 744, 757
Obligatory intra cellular, 765
Obligatory suppression, 641
Oblique, 4, 164, 593
Oblique astigmatism, 564
Oblique axis, 164
Oblique illumination, 164, 307, 326
Oblique muscle, 598, 615
Oblique orbital line, 694
Oblique school, 669
Obliteration of canal of Schlemm, 354
Obliteration of lacrimal ducts, 148
Occipital cortex, 17, 504, 637
Occipital lobe, 584
Occipital lymph glands, 298
Occipito cortical, 584
Occipito mesencephalic path, 601
Occiput, 421
Occlusio pupillae, 244, 251, 354
Occlusion, 588
Occlusion amblyopia in better eye,
590
Occuloplastic surgeries, 384
Occulsio pupilli, 252, 476
Occult retinoblastoma, 351
Occuserts, 182
Ocular, 236
Ocular albinism, 401, 402
Ocular changes, 307
Ocular emergency, 126
Ocular fixation nystagmus, 631
Ocular hypotensive, 376
Ocular involvement, 56, 57, 58, 59,
149
Ocular management, 49
Ocular manifestation of systemic vi-
rus disease, 744
Ocular myiasis, 154, 773
Ocular myopathy, 78
Ocular nystagmus, 630
Ocular pemphigoid, 104, 120
Ocular posture, 628
Ocular prosthesis, 144
Ocular surface, 102
Ocular surface disorder, 96, 376
Ocular symptoms of myasthenia
gravis, 75
Ocular torticolis, 611
Ocular toxoariasis, 264, 476, 489
Ocular ultrasonography, 326, 358,
464
Oculo auriculo vertibral syndrome,
683
Oculo cutaneous, 236, 400
Oculo motor, 600, 601
Oculo sporidiosis, 149, 724
Oculo sympathetic, 74, 612
Oculohypotensive, 377
Oculomotor, 38, 43, 228, 510, 511,
593, 600, 601
Oculomotor palsy, 73
Oculomotor ptosis, 73
Oculosporidiosis, 121
Oculosympathetic, 73, 510, 606
Ocuserts, 373
Oedema, 324
Ofloxacilin, 179, 737
Oguchis, 419, 574, 575
Oil drop, 303
Oil dropelet cataract, 300
Ointment, 256, 373
Old retro bulbar neuritis, 535
Oligodendro glioma, 710
Oligodocytes, 710
Onchocerciasis, 152, 206, 768, 771
Onchocercomata, 771
Onchocerea caecutiens, 771
Onchocerea volvulus, 765
Oncotic pressure gradient, 448
One and half syndrome, 605
Oocytes, 259
Oophoritis, 746
Opacification of cornea, 148
Opacity, 315
Opacity in media, 650
Opaque contact lens, 589
Opaque nerve fibers, 56, 399, 423,
476, 513, 514, 584
Open angle glaucoma, 308
Operating gonioscope, 349
Operating microscope, 349, 382
Operculated hole, 449
Ophthal micartery, 89
Ophthalmia neonatorum, 117, 122,
123, 126, 129, 168, 173, 190
Ophthalmia nodosa, 121
Ophthalmia nodosum, 149
Ophthalmianeonatorum, 18
Ophthalmic, 87, 602
Ophthalmic artery, 87, 152, 231, 293,
397, 405, 511, 599, 674, 675, 677
Ophthalmic neonatorum, 90
Ophthalmic veins, 231, 511
Ophthalmoplegia, 78, 314, 698
Ophthalmoplegia plus, 78, 624
Ophthalmoplegic migraine, 607, 608
Ophthalmoscope, 171, 326, 382
Opportunistic, 192, 279, 727, 762, 766
Opportunistic infection, 749
Opportunistic organism, 173, 492, 726
Opportunistic systemic infection, 260
Opthalmic vein, 596
Optic abiotrophies, 536
Optic atrophy, 508, 514, 520, 523, 532,
540, 650, 665, 678, 680, 681, 684,
685, 686, 742, 767
Optic canal, 511, 540, 694, 697
Optic chiasma, 512, 699
Optic cup, 1, 228, 233, 393, 395, 398,
447, 512
Optic disc, 15, 394, 415
Optic foramen, 597, 674, 677, 694
Optic gliomas, 56
Optic nerve, 51, 56, 230, 246, 393, 395,
456, 507, 530, 675, 676, 677, 680,
697, 720
Optic nerve anomaly, 115
Optic nerve glioma, 532, 539, 676,
687, 688, 689, 695, 696, 697, 698,
707
Optic nerve head, 246, 401, 408, 510
Optic nerve hypolplasia congenital
cone dystrophy, 21
Optic nerve hypoplasia, 628, 630
Optic nerve sheath-complex, 699
Optic nerve sheathmeningioma, 701
Optic neuritis, 52, 96, 202, 203, 245,
423, 429, 508, 520, 530, 698, 703,
706, 727, 742, 746, 748, 750, 753,
759, 767
Optic neuropathies, 481, 668, 690, 699
Optic pathway, 577
Optic pit, 1
Optic radiation, 504, 529, 530, 580
Optic stalk, 5, 233, 512
Optic tract syndrome, 584
Optic vesicle, 1, 233, 293
Optical, 159, 160
Optical coherence tomography, 412,
416
Optical iridectomy, 189
Optical zone, 159
Optically elicited movements, 17
Opticcup, 293
Optico kinetic drum, 632
Opticokinetic, 632
Optics of aphakia, 560
Opto kinetic nystagmus, 629
Optociliary shunts, 686
Optokinetic, 596, 632
Optokinetic drum, 30
Optokinetic drum of harcourt, 29
Optokinetic nystagmus, 630, 631
Optokinetic target, 16
Optotypes, 21, 586
Ora serrata, 229, 230, 393, 448, 456
Oral acetazolamide, 205, 349, 367
Oral bays, 393
Oral diamox, 151
Oral steroid, 78
Oral thrush, 762
Orange peel appearance, 150
Orbicularis, 35, 42, 67, 76, 79, 685
Orbicularis muscle, 81
Orbicularis oculi, 36
Orbicularis reflex, 504
Orbiculosis, 200
Orbit, 56
Orbits of the children, 677
Orbit at birth, 677
Orbit, 86, 116, 153, 697, 701, 769
Orbital abscess, 687, 690, 701, 704
Orbital and periorbital dermoids, 678,
679
Orbital apex, 539
Orbital apex syndrome, 607, 763
Orbital causes, 62
Orbital cellulitis, 44, 97, 282, 477, 526,
532, 674, 685, 687, 688, 689, 690,
698, 701, 703, 708, 709, 712, 728,
736, 738, 739, 763
Orbital cyst, 152
Orbital dermoid, 712
Orbital dysplasia, 696
INDEX 807
Orbital encephalocele, 678
Orbital fat, 87, 675, 676
Orbital granuloma, 760
Orbital meningocele, 678
Orbital neuroblastoma, 708
Orbital part, 87, 602
Orbital part of the palpebral, 111
Orbital plate of frontal, 673
Orbital pseudo tumours, 417, 624, 687
Orbital retinoblastoma, 687, 688
Orbital rim, 89, 673, 694
Orbital septum, 35, 37, 87, 674
Orbital teratoma, 678, 678
Orbital tubercle, 37
Orbital tumours, 13
Orbital varices, 688, 691, 713
Orbital varix, 687, 696
Orbital venography, 675
Orbitalseptum, 679
Orbitotomy, 115, 675, 679
Orbits, 14
Orbularis, 624
Orchitis, 746
Organ of corti, 439
Organ transplant, 762
Organic amblyopia, 586, 587
Oriental worm, 772
Ornithine, 238
Ortho voltage radiation, 61
Orthokeratology, 554
Orthomyxo virus, 758
Orthophoria, 571, 572, 611, 621, 623,
641, 646, 648, 660, 661, 664,
665, 668
Orthoptic, 648
Osazone crystals, 300
Oscillopsia, 420, 618
Oscilloscope, 420
Osteo chondritis, 740
Osteoblastic metastases, 696
Osteogeneses, 26
Osteogenesis imperfecta, 695, 722
Osteogenic sarcoma, 479
Osteoma, 701
Osteomyelitis, 736, 744
Otitis, 129
Otitis media, 620, 731, 734, 735, 736,
743, 748
Outer barrier, 423
Outer neuroblast, 399
Outer nuclear layer, 395, 396, 403
Outer nuclear zone, 399
Outer plexiform, 396
Outer plexiform layer, 395, 396, 403
Outflow is within normal limits, 370
Outflow of aqueous, 341
Ovaries, 746
Over correction of ptosis, 81
Oxalosis, 439
Oxidase, 733
Oxycephaly, 306, 679, 681
Oxydative phosphorylation, 537
Oxygen, 102, 160
Oxytetracyclin, 758
P
P malariae, 766
P ovale, 766
P. acne, 280
P. capitis, 46
P.A.S., 177
P.C. I.O.L., 26
P.H.P.V., 28
P.P.R.O., 619
Pad and bandage, 181
Pagets disease, 439
Pain, 242
Painful ophthalmoplegia, 607
Painted contact lenses, 190
Paired nuclei, 601
Palatal, 62, 622
Palatine, 673
Palatine air cells, 674
Palatine bones, 674
Pallor, 115
Pallor of conjunctiva, 115
Palpebral, 62, 87, 111, 141
Palpebral conjunctiva, 110
Palpebral fascia, 37
Palpebral fissure, 18, 34, 70
Pan fundic lens, 414
Pan retinal photo coagulation, 580
Pan uveitis, 239, 242, 253, 263
Pan uveitis in children, 249
Pancreas, 746
Pannus, 50, 133, 170, 172, 745, 756
Pannus of trachoma, 135
Pano-phthalmitis, 44, 117, 127, 175,
184, 188, 239, 253, 280, 282, 428,
477, 526, 685, 687, 688, 726, 728,
734, 737
Panophthalmitis, 44, 117, 127, 184,
188, 239, 280, 282, 428, 477, 526,
685, 687, 688, 726, 728, 734, 737
Panretinal photo coagulation, 385
Panums fusional area, 638
Panuveitis, 24
Papilitis, 276
Papillae, 23, 37, 116, 139, 141, 147,
510
Papillae formation, 134
Papillaedema, 415, 422, 423, 424, 425,
518, 519, 520, 521,528, 530, 534,
681, 684, 686, 710, 732, 735,
742, 748, 750, 768, 770
Papillary, 140
Papillary hypertrophy, 134
Papilledematous, 532
Papillitis, 242, 245, 246, 247, 260, 263,
267, 274, 422, 424, 425, 520, 524,
525, 526, 532, 534, 762
Papillo macular, 526, 537, 582
Papillo macular bundle, 535
Papillo macular fibre, 432
Papilloma, 49, 54, 150
Papillomatous granuloma, 48
Papule, 49, 250
Para central, 582, 641
Para fovea, 394, 641
Para macular, 641
Para median pontine reticular forma-
tion, 601, 605
Para median reticular formation, 596
Para sellar growths, 607
Para sympathetic origin, 709
Para-amino benzoic acid, 60
Para-axial mesoderm, 600
Para-sympatholytic drugs, 505
Paraaxial mesoderm, 677
Paracecal, 527
Paracentesis, 151, 184, 278, 364, 380
Paracentral scotoma, 516
Paradoxic movements, 608
Paradoxical glaucoma, 367
Paradoxical reflex, 95
Paradoxical rise of tension, 351
Paralysis of accommodation, 125, 730
Paralysis of convergence, 125
Paralytic, 612
Paralytic ectropion, 82
Paralytic ileus, 729
Paralytic phase, 608
Paralytic squint, 22, 523, 639, 649,
651, 681, 689, 699, 742, 748
Paramyxo viridae, 748
Paramyxo virus, 745, 746
Paranasal sinuses, 677, 689, 692, 703
Paraplegia, 530, 680
Parasellar, 479, 508
Parasites, 45, 759
Parasitic blepharitis, 46
Parasitic conjunctivitis, 152
Parasitic cyst, 152, 453, 477, 696
Parasitic endophthalmitis, 279, 476
Parasitic granuloma, 483
Parasympathetic, 20, 87, 227, 228,
230, 254, 502, 601, 602,
Parasympathomimetic, 95, 372, 505
Paratrachoma, 133
Paraxial mesoderm, 6
Paredrin, 74, 506, 512
Parenchymatous, 172, 206
Parieto-occipial temporal pathway,
596
Parieto-occipital temporal
mesencephalic path, 601
Parinauds syndrome, 509, 613, 614,
668
Park Bielschowsky, 611, 614
Parotids, 96, 746
Parrot beak nose, 683
Pars ciliaris, 334
Pars plana, 229, 230, 272
Pars planitis, 240, 241, 245, 246
Pars plicata, 229
Parsplana vitrectomy, 456, 464, 490
Parsplanitis, 239, 253, 258, 265, 433,
454, 461, 463, 476, 742, 771
Partial accommodative, 654
Partial albinism, 402
Partial occlusion, 661
Partially accommodative esotropia,
661
Passive congestion of conjunctiva, 116
Patechea, 119, 211
Patecheal haemorrhage, 123, 129,
755, 767
Patelar fossa, 291, 547
Pathological adhesion of the vitreous,
457
Pathological lid retraction, 80
Pathological myopia, 551, 690
Pathological nystagmus, 633
Pattern reversal VER, 421
Pauci articular, 268
Paving stone, 446
PCO, 453
Peculiarities of pediatric uveitis, 258
Pediatric uveitis, 258
Pediculosis, 46, 176, 765
Pellucid degeneration, 163, 166
Penalisation, 590
Pendular, 633
Pendular nystagmus, 628, 630
Penetrating corneal graft, 211
Penetrating diathermy, 58
Penetrating injuries, 274, 324, 454
Penetrating keratoplasty, 165, 169,
171, 181, 198, 205, 209, 210, 211,
278, 279, 354
Penicillin, 126, 148, 180, 184, 192,
193, 726, 728, 729, 730, 739, 743
Pepper, 439
Pepper and salt, 430, 466
Pepper and salt fundus, 261
Pepper mottling, 429
Pepper type, 709
Perennial allergic conjunctivitis, 139,
140
Perforation, 185
Perforation a globe, 463
Perforation of cornea, 174
Peri artrititis, 272
Peri fovea, 394
Peri foveal arcade, 415
Peri orbital cellutitus, 130
Peri papillary chorio retinal scar, 764
Peri phlebitis, 428
Peri retinal gliosis, 265
Peri retinal membrane, 465
Peri vasculitis, 751, 762
Peribulbar, 599
Peribulbar anaesthesia, 380
Peribulbar injection, 275, 278
Pericarditis, 268, 733, 734, 736, 758
Pericystic cellulilis, 92
Pericystitis, 100
Perilimbal conjunctiva, 144
Perimeter test, 652
Perineural tumour, 539
Perineuritis, 526
Perinuclear, 297
Periocular, 278
Periocular steroids, 270
Periodic acid Schiff stain, 763
Periorbita, 37, 89, 511, 673, 675
Periorbital abscess, 674, 685, 688, 701
Periorbital cellulitis, 702
Periorbital space, 675
Periostitis, 696, 702, 740
Periostium, 87, 674
Peripapillary crescent, 552
Peripapillary edema of retina, 584
Peripbulbar steroids, 272
Peripheral, 178
Peripheral anterior synchia, 241, 244,
247, 249, 250, 337, 340,
Peripheral anterior synechiae, 360
Peripheral basal iridectomy, 379, 380
Peripheral button hole iridectomy,
380
Peripheral cataract, 576
Peripheral field of vision, 394, 396
Peripheral gliosis, 265
Peripheral granuloma, 265, 272, 433,
489, 490, 771
Peripheral involvement, 73
Peripheral iridectomy, 312, 356
Peripheral keratitis, 70, 205
Peripheral neuropathy, 439
Peripheral retinal degeneration, 253,
283, 324, 445
Peripheral retinal detachment, 259
Peripheral retinal granuloma, 433
Peripheral space, 675
Peripheral synechiae, 212
Peripheral ulcer, 193
Peripheral uveitis, 454
Peripheral vasculitis, 253, 433
Periphlebitis, 247, 272, 422, 462, 463,
732
Periphlebitis retinae, 426
Peritonitis, 736
Perivasculitis, 245
Perkins, 341
Perkin s handheld applanation
tonometer, 347
Perlias nucleus, 504, 509
Persistan primary hyperplastic vitre-
ous, 241, 316
Persistence of anterior hyloid system,
295
Persistent corneal haze, 17
Persistent hyaloid artery, 426
Persistent hyaloid system, 316
Persistent hyaloid vasculature, 338
Persistent hyper plastic primary vit-
reous, 407, 454, 459
Persistent hyper plastic primary vit-
reous, 266, 453, 475, 476,
Persistent neonatal myasthenia, 76
Persistent primary hyper plastic vit-
reous, 13, 306, 348, 352, 403,
433, 462, 483
Persistent pupillary member, 4, 19,
27, 235, 296, 401, 683
Persistent shallow, 351
Persisting staring, 21
Petechial haemorrhages, 24, 736
Peters, 19
Peters anomaly, 19, 26, 338, 339
Petrosal sinuses drain, 704
Petrous bone, 620, 704
PH, 550
Phaco-morphic, 367
Phacoanaphylactic, 367
Phacoanaphylatic glaucoma, 367
Phacocele, 122, 559
Phacodonesis, 162, 307
Phacoemulsi-fication, 322
Phacogenic, 363
Phacogenic uveitis, 310
Phacolytic, 367
Phacomas, 409, 491
Phacomatosis, 368, 408
Phacotopic, 367
Phagocytes, 255
Phagocytosed, 364
Phagosomes, 395
Phakia children, 8
Phakic, 304
Phakic eye, 244, 357
Phakic intraocular lens, 554, 559
Phakomas, 55
INDEX 809
Pharmacological causes of lid retrac-
tion, 80
Pharmacological defocusing, 590
Pharyngeal muscle, 78
Pharyngitis, 131, 754, 758
Pharyngo conjunctival fever, 126,
128, 131
Pheboliths, 713
Phenothiazine, 103, 440
Phentolamine, 505
Phenyl mercuric nitrate, 215
Phenyl pherine, 208, 255
Phenylbutazone, 148
Phenyle pherine, 20, 80, 254, 318, 506
Pheo chromocytoma, 408
Phithisis, 488
Phlycten, 23, 24, 49, 132, 172, 178,
724, 731, 738, 745, 762
Phlyctenular keratitis, 168, 190
Phlyctenular conjunctivitis, 732
Phlyctenular kerato conjunctivitis,
97, 144, 576, 732
Phlyctenular pannus, 146
Phlyctenulosis, 170, 205
Phlyctenulosis of cornea, 205
Phorias, 571
Phosphate, 214
Phospholine iodide, 657
Phospholipid, 442
Phosphorescent sheen, 575
Photo coagulation, 260, 408, 456, 465,
493
Photo keratoscope, 166
Photo keratoscope detect, 164
Photo metric neutral density filter,
615
Photo radiation, 382
Photo receptor dystrophy, 434
Photo receptors, 393, 395, 477
Photo refractive keratectomy, 554,
559
Photo stress test, 412, 421
Photo sublimation, 382
Photo vaporising, 382
Photocoagulation, 382, 480, 534
Photodisruption, 382
Photographs, 610
Photon, 709
Photophobia, 60, 91, 131, 132, 141,
164, 178, 273, 347, 441, 462, 474,
576, 579, 632, 689
Photophobia in children, 147
Photopic, 417, 418
Photopic ERG, 441
Photopic vision, 396, 586
Photopsia, 411, 450, 459, 552, 576
Photostatic, 510
Phototoxicity, 487
Phthiriasis, 46, 176, 765
Phthisis, 25, 68, 163, 175, 188, 204,
274, 282, 354, 453, 690
Phthisis blubae, 166, 188, 252, 478,
696, 701
Phthisis, perforated globe, 166
Phycomycetis, 762
Phylum thallophyta, 759
Physiology of the cornea, 160
Physostigmine, 505
Pia, 5, 511, 513
Pial vessels, 479
Pica, 266, 489, 490
Picorna viruses, 745
Pie in the sky, 583
Pie on the floor, 583
Pierre Robin syndrome, 682
Pierre-Robinson, 453
Piezo electric crystal, 417
Piezoelectric effect, 700
Pig, 152
Pigment, 342, 436
Pigment deposit, 189
Pigment epithelium, 5, 393, 447
Pigment epithelium of retina, 228
Pigmentary dystrophies, 435
Pigmentation of conjunctiva, 117
Pigmentation of the macula, 246
Pigmented coloboma, 410
Pigmented epithelium, 334
Pigmented epithelium of ciliary body,
229
Pigmented KP, 243
Pilocarpine, 95, 149, 184, 349, 511
Pilocarpine, methacholine, 505
Pilocytic astrocytes, 710
Pin cushion, 561, 570
Pin hole effect, 547
Pineal body, 479
Pineal tumour, 510
Pinealoblastoma, 474
Pinhole, 455, 565, 566
Pituitary, 541
Pituitary fossa, 680
Pituitary tumour, 508, 583
Placenta, 754
Placido, 164
Placido disc, 566
Plane mirror, 164
Plane of nystagmus, 629
Plano T contact lenses, 143
Plaque, 141
Plaque radiation, 481
Plasmodia, 181, 764, 766
Plasmodium vivax, 766
Plasmoid aqueous, 243, 245, 248, 360,
373
Plastic iritis, 244
Pleomorphic, 707, 730, 731
Pleura, 731
Plexiform neuro fibroma, 56, 678
Plexiform neuroma, 698
Plica, 87, 94
Plica semilunaris, 112, 113
Plications, 230
Pliocarpine, 373
Plombs, 456
Pluri potential embryonal mesoderm,
707
Plus disease, 486
Plus lenses, 503
Pneumo cocci, 123, 175, 184, 248
Pneumococcal conjunctivitis, 119, 129
Pneumococcal infection, 735
Pneumococcal keratitis, 192
Pneumococcal ulcer, 191
Pneumococcas, 23, 91, 92, 191, 240,
258, 702
Pneumococci, 122, 126, 177, 182
Pneumocystis, 267
Pneumocystiscarinii, 750
Pneumonae, 133
Pneumonia, 49, 526, 726, 730, 731,
736, 744, 747, 748, 758, 763
Pneumonitis, 133, 489
Pneuno tonometer, 341
Pneunococcal conjunctivitis, 24
Pneunococcus, 127, 175
Pneunonitis, 129
Poliomyelitis, 745
Poliosis, 274, 275, 402
Pollen, 139
Poly articular, 271
Polyarthritis, 733
Polyarticular involvement, 268
Polychromatic, 579
Polychromatic lusture, 244, 247, 252
Polycoria, 12, 27, 229, 251, 501
Polycythemia, 117, 408, 422
Polycytosis, 274
Polydactyly, 62, 439
Polyenes, 205
Polygenic inheritance, 339
Polymer, 144
Polymerase chain, 264
Polymeric-polyhexa methylene
biguanide, 209
Polymethacrylate (PAMA), 562
Polymorphonuclear cells, 121
Polymorphonuclear leucocytes, 181
Polymyxine, 179
Polyneuritis, 730
Polyopia, 305
Polyp, 101
Polypeptide, 257
Polyvinyl alcohol, 106
Pons, 82, 87, 618
Pontine paramedian reticular forma-
tion, 619
Ponto medullary junction, 604, 606
Poor accommodation, 302
Poor colour sense, 443
Pork tape worm, 769
Pork worm, 152
Port wine, 57, 61
Posterior ciliary arteries, 232
Positional myopia, 555
Positive HLA B 27, 271
Positive regurgitation, 93
Post ganglion, 502
Post ganglionic lesion, 74
Post herpatic neuralgia, 52
Post herpetic neuralgia, 52, 202, 752
Post keratoplasty, 565
Post neuritic, 532, 533
Post neuritic optic atrophy, 247
Post operative fungal, 281
Post papilledematous, 422, 533, 584
Post papilledematous atrophy, 680
Post papilledematous optic atrophy,
540, 686, 710, 732
Post surgical status, 556
Post trauma, 556
Post traumatic edema, 423
Post vaccination optic neuritis, 529
Post vaccination status, 283, 527
Post vitrectomy glaucoma, 357
Posterior, 256
Posterior capsular cataracts, 296
Posterior capsular opacification, 247,
561
Posterior capsular traction lines, 323
Posterior capsule, 291, 322, 323, 330
Posterior capsulorrhexis, 323
Posterior cerebral, 603, 606, 612
Posterior cerebral arteries, 584, 602
Posterior chamber, 27, 335, 501, 563
Posterior chapsule, 326
Posterior chiasma, 583
Posterior ciliary arteries, 153, 511,
720
Posterior ciliary system, 232
Posterior clinoid, 602, 694
Posterior communicating artery, 602,
603, 606, 607
Posterior conjunctival, 116
Posterior cortex, 330
Posterior cyclitic membrane, 244, 476,
483, 489, 490
Posterior dislocation of lens, 561
Posterior dystrophies in children, 212
Posterior embryotoxon, 338, 340, 242
Posterior epithelium, 228
Posterior epithelium, 229, 614
Posterior lacrimal crest, 89, 599, 674
Posterior lens capsule, 456
Posterior lenticonous, 300, 302
Posterior limbal border, 112, 336
Posterior limbal zone, 336
Posterior mesoderm, 3
Posterior pituitary, 714
Posterior plus, 486
Posterior polar, 295, 296, 437
Posterior polar granuloma, 265
Posterior pole, 291
Posterior polymorphic corneal dystro-
phy, 337
Posterior polymorphous dystrophy
(P.P.M.D.), 212, 216
Posterior root ganglion, 50, 199
Posterior scleral foramen, 720
Posterior staphyloma, 413, 553, 701,
723
Posterior sub capsular cataract, 245,
436
Posterior sub capsular opacification,
257, 328, 437
Posterior synechia, 235, 241, 244, 249,
250, 268, 269, 271, 283, 330
Posterior uveitis, 239, 242, 461, 481,
525, 732
Posterior vasculosa lentis, 293
Posterior vitreous detachment, 246,
247, 253, 260, 449, 451, 452, 456,
462, 553
Postherpeticneuralgia, 202
Potassium, 103
Potassium loss, 257
Povidon, 757
PovidonIodine, 128, 734
Power of IOL, 417
Pox viruses, 745
Poxviridae, 756
Poxvirus, 50
Prasympathomimetic, 77
Pre arterial (Choroidal) phase, 414
Pre auricular, 131, 702
Pre auricular glands, 114
Pre auricular lymphadenopathy, 132,
203
Pre auricular nodes, 38
Pre ganglionic lesion, 74
Pre geniculate, 507
Pre geniculate path, 420
Pre lamilar, 510, 521
Pre mandibular, 600
Pre retinal haemorrhages, 426
Pre senile cataract, 466
Pre vitelli form, 444
Pre-chiasmal conduction, 508
Pre-presbyopic age, 557
Preauricular, 87, 479
Preauricular glands, 92
Preauricular lymph, 51
Preauricular lymph nodes, 47
Precipitates, 196
Precocious puberty, 541, 711
Precorneal tear film, 102
Precortical path, 28
Precortical visual path, 27
Prednisolone, 143, 275, 361, 536, 713
Prednisone, 60, 361
Preferential looking, 29
Prelenticular, 483
Prematurity, 16, 484
Premethrine, 46
Preoperative mydriasis, 257
Prerequisite of binocular single vi-
sion, 638
Presenting features, 316
Preseptal, 53, 701
Preseptal cellulitis, 739
Preseptal orbital cellulitis, 701
Pressure necrosis of iris, 357
Presumed ocular histoplasmosis syn-
drome (POHS), 764
Pretectal nuclei, 602
Pretectal nucleus, 503, 504
Prethresh hold disease, 486
Prexerosis, 217
Primary acquired exotropia, 666
Primary aphakia, 559
Primary atrophy, 710
Primary band keratopathy, 215
Primary buththalmos, 90
Primary colours, 577
Primary congenital buphthatmos, 97
Primary congenital glaucoma, 337
Primary developmental glaucoma,
344
Primary deviation, 651
Primary dye test, 101
Primary herpes simplex, 177
Primary herpes simplex infection, 194
Primary infentile glaucoma, 339
Primary keratitis, 198
Primary lens fibers, 2, 291
Primary neuro sensory dystrophy of
the retina, 435
Primary open angle glaucoma, 343,
370
Primary optic atrophy, 535, 540, 681
Primary optic vesicle, 9
Primary position, 594
Primary Vitreous, 2, 3, 458, 487
INDEX 811
Primary/congenital retinal
telangiectasis, 405
Primitive internal carotid, 6
Primitive precursor, 477
Priscoline, 505
Prism, 503, 635, 648, 668
Prism and cover test, 645, 670
Prism diopters, 645, 648, 653
Prism dissociation test, 645, 646
Prism reflex test, 652
Prism test, 78, 618, 644
Pro drug, 197, 362, 375, 376
Probable cause of angle closure glau-
coma in uveiti, 360
Probable causes of open angle glau-
coma in uveitis, 360
Probable congenital causes of brown
syndrome, 616
Probe, 699
Probing, 93
Probing of the nasolacrimal duct, 91
Procaine, 255
Proctitis, 757
Prodromal, 148
Prodromal stage, 275
Proglotidis, 769
Programmed death, 434
Progressive cone dystrophy, 434, 441
Progressive external ophthal-
mioplegia, 437, 439, 613
Progressive night blindness, 238
Progressive outer retinal necrosis
(PORN), 750
Progressive stage, 170, 206
Progressive supra nuclear palsy, 624
Projection, 638
Prolapse of uvea, 280, 325
Proliferative diabetic retinopathy, 454
Propamindine, 209
Proparacaina, 105, 106
Proparacine hydrochloride, 176
Properties of laser, 382
Prophylactic iridectomy, 302
Prophylaxis, 128, 153, 219
Propioni bacterium, 258, 280, 323
Proptometers, 691
Proptosis, 16, 18, 20, 44, 56, 62, 80,
81, 96, 111, 117, 153, 173, 221,
253,474, 479, 539, 607, 609, 675,
676, 677, 678, 679, 681, 684, 699,
702, 707, 709, 710, 715, 763,
770, 771
Prostaglandin, 354, 360, 368, 370
Prostaglandin analogue, 270, 372, 376
Prostaglandin inhibitors, 257
Prostaglandin level, 487
Prostaglandins, 138, 506
Prostaglandinsynthatase, 257
Prostamide sensitive receptors, 376
Prostamides, 372, 376
Prostigmine, 76, 77, 80
Protanomalous, 578
Protanomaly, 578
Protanopia, 578
Protanopia deuteranopia, 578
Proteases, 735, 737
Protein caloric malnutrition, 219, 440
Proteolytic enzyme, 192
Proteus, 124, 743
Protozoa, 152, 259, 764
Pseudo angle, 251, 360
Pseudo angle closure glaucoma, 252
Pseudo angle formation, 250
Pseudo arcus, 142
Pseudo Argyll Robertson pupil, 509,
608
Pseudo bulbar paby, 98
Pseudo cancerous, 54
Pseudo convergent squint, 15, 18, 22,
40, 41, 550, 642
Pseudo cystic phase, 765
Pseudo dendretic keratitis, 49
Pseudo dendrite, 208, 756
Pseudo dendritic ulcer, 50
Pseudo divergence, 667
Pseudo divergent squint, 558
Pseudo enophthalmos, 74
Pseudo exotropia, 665, 666
Pseudo glioma, 483
Pseudo Graefe sign, 72, 608
Pseudo hypopyon, 283, 444
Pseudo isochromatic, 579
Pseudo macula, 639
Pseudo membrane, 121, 127, 131,
148, 735, 736, 751, 769
Pseudo membrane formation, 132,
203, 731, 754
Pseudo membrane over, 141
Pseudo membranes conjunctivitis,
119, 124, 126, 129, 149, 194, 730,
739, 745
Pseudo membranous, 125
Pseudo membranous colitis, 432
Pseudo myopia, 557
Pseudo neuritis, 415, 513, 519, 530,
532, 558
Pseudo papilledema, 513, 519
Pseudo paralysis, 624
Pseudo polycoria, 337, 501
Pseudo polycoria, corectopia, 340
Pseudo proptosis, 345, 552, 690, 723
Pseudo pterygium, 189
Pseudo ptosis, 22, 48, 68, 79, 141, 200,
609, 690
Pseudo retinitis pigmentosa, 741
Pseudo retraction syndrome, 623
Pseudo rosettes, 478
Pseudo spiralis, 772
Pseudo squint, 265, 641, 651
Pseudo tumor orbit, 624
Pseudo tumour, 620, 695, 708
Pseudo tumour cerebre, 522, 532, 742,
Pseudo tumour, 525, 607, 686, 688,
698, 690, 697, 705,
Pseudo vertical squints, 612
Pseudo-angle-formation, 354
Pseudo-membranous-conjunctivitis,
762
Pseudocoria, 186, 338
Pseudoglioma, 475
Pseudohypopyon, 26, 368, 474, 479,
492
Pseudomembranous conjunctivitis,
104
Pseudomonas, 123, 127, 175, 176,
177, 182, 191
Pseudomonas areuginosa, 737
Pseudomonas corneal, 184
Pseudomonas infection, 181
Pseudomonas keratitis, 192
Pseudomonas ulcers, 184
Pseudomyopia, 550, 555
Pseudomyopia night myopia space
myopia, 548
Pseudoneuritis, 8, 518, 524
Pseudophakia, 354, 453, 562, 563
Pseudophakic myopia, 555
Pseudopolycoria, 27, 229, 234
Pseudoproptosis, 550
Pseudorosettes, 477
Psittacosis, 133
Psychosensory reflex, 504
Pteroclinoid ligament, 510
Pterygia, 59, 122
Pterygium, 122, 166, 189, 565
Pterygo mandibular complex, 683
Pterygoid, 43
Pterygoid muscle, 43
Pterygoid plexus, 677
Ptosis, 13, 16, 18, 22, 40, 61, 67, 74,
75, 78, 96, 163, 200, 257, 306,
330, 509, 585, 588, 590, 608, 609,
614, 617, 650, 657, 678, 684, 685,
690, 728, 729, 730
Ptosis crutches, 78
Ptosis in childhood, 69
Puberty, 163
Pulfrich phenomenon, 530
Pulmonary edema, 767
Pulmonary tuberculosis, 731
Pulmonic stenosis, 747
Pulsating exophthalmos, 56
Pulse less disease, 240
Pulverulent cataract, 297
Pumic stone iris, 772
Puncta, 7, 86, 88, 98
Punctal displacement, 71
Punctal stenosis, 41
Punctate keratitis, 45, 135, 172, 726,
752, 756, 771
Punctate lesions, 201
Punctual block, 107
Punctum, 92
Punctum dilator, 100
Punctum remotum, 550
Pupil, 15, 19, 227, 244, 501
Pupil, 4
Pupil is kinetic indicator of functional
state, 502
Pupillary abnormalities, 16
Pupillary block, 302, 312, 354, 357,
360, 361, 363, 373
Pupillary light reflex, 503
Pupillary margin, 227
Pupillary membrane, 4, 6, 234, 293
Pupillary reaction, 70
Pupillary reaction in mid chiasmal
lesion, 508
Pupillary zone, 227
Pupillo motor fibres, 503
Pure amblyopia, 585
Pure carbolic, 181
Pure microphthalmos, 8
Purkenjes image, 28, 244, 251, 484
Purpura, 24, 44, 425, 463
Pursuit, 586
Purtschers retinopathy, 426
Purulent, 148
Purulent conjunctivitis, 44, 726, 751,
762
Purulent discharge, 24, 91, 120, 180
Purulent keratitis, 282
Purulent optic neuritis, 704
Purulent pan uveitis, 278
Purulent retinitis, 428
Pus in AC, 244
Pustular, 200
Pyelitis, 762
Pyocyanin, 737
Pyoderma, 739
Pyramidal cataract, 296
Pyramidal tract, 604, 619
Pyridostigmine, 76, 77, 505
Pyridoxine, 238, 311
Pyrimethamine, 261, 432
Pyrimidines, 205
Q
Q switched Nd-YAG, 351
Quadraniopia, 583
Quadrilateral, 166
Quinine, 440
Quinine amblyopia, 419, 767
Quinone, 401
R
Rabies, 529
Racemose aneurysm, 405
Radial fibres, 230
Radial keratotomy, 554
Radiation, 324, 471, 706, 709, 759
Radiation cataract, 481
Radiation retinopathy, 481
Radiation to the mother, 429
Radio isotope, 102
Radio therapy, 707
Radioactive cobalt plaque, 480
Radiology of orbit, 693
Radiotherapy, 708
Radon, 481
Raeders, 512
Rafsums syndrome, 439
Rail road, 632
Rail road nystagmus, 630
Raised episcleral pressure, 24
Raised intra cranial tension, 173, 179,
680, 770
Raised serum calcium, 272
Ralysis, 132
Ramsay Hunt syndrome, 52, 82, 200,
753
Range of fusion, 646
Rapid phase, 629
Rarely fungi, 97
Rate, 628
Rate of formation of aqueous humour,
341
Raymonds syndrome, 619
Recession of angle of anterior cham-
ber, 326, 363
Reciprocal innervation, 628
Reckling Hausens, 55, 368
Reconstruction of anterior segment,
327
Recording of distant vision, 28
Rectus, 601
Recurrent branch of choroids, 232
Recurrent erosion, 210
Recurrent herpes simplex, 194
Recurrent HSV, 197, 198
Recurrent HSV keratitis, 198
Recurrent lacrimal artery, 677
Recurrent retinal branch, 397
Red blood cells, 364
Red cell glaucoma, 363
Red glass test, 641, 644, 645, 646, 653
Red green colour defect, 443
Red green defect, 580
Red KP, 243
Red nucleus, 602, 605, 606
Reduced ciliary secretion, 354
Reduced thickness, 167
Reduced zinc in plasma, 537
Reduplication, 296
Refixation reflex, 637
Reflected part, 598
Reflex lacrimation, 103
Reflex tearing, 95, 102, 105, 106
Reflex watering, 62
Refractive error, 371, 570, 656
Refractive index, 103, 161, 340, 456
Refsums syndrome, 439
Regular astigmatism, 564
Regurgitation test, 23, 150
Rehegmatiogenous retinal detach-
ment, 488
Reigers anomaly, 306, 314, 316
Reis-Bucklers dystrophy, 211
Reiters syndrome, 240, 271, 757
Relative afferent pupillary defect, 507
Relative afferent pupillary response,
586
Relative negative convergence, 648
Remnants of hyaloid vessels, 462
Remnants of hyloid system, 20, 519
Remnants of hyoid system, 28
Remnants of primary vitreous, 461
Remote injuries, 119
Renal failure, 767
Renal retinopathy, 434
Renal stone, 330
Rendu-Osler-Weber syndrome, 55
Resse ellosworth classification, 483
Restrictive causes of vertical squint,
613
Restrictive strabismus, 689
Retained intra ocular copper, 329
Retained intra ocular foreign body,
278, 324, 326, 687,
Retained orbital foreign body, 688
Retention cyst, 122, 152
Retina, 5, 56, 153, 230, 234, 259, 274,
393, 503, 512
Retinal detachment, 403
Retinal abiotrophy, 534
Retinal aneurysm, 427
Retinal angioma, 407
Retinal angiomatosis, 405, 408
Retinal artery, 396, 397
Retinal artery obstruction, 416
Retinal artery occlusion, 422
Retinal astigmatism, 564
Retinal astrocytoma, 492
Retinal band, 433
INDEX 813
Retinal blood vessels, 246
Retinal break, 448, 461
Retinal capillaries, 393, 394
Retinal capillary haemangioma, 478,
492
Retinal cavernous haemangiomas,
405, 409
Retinal circulation, 7
Retinal correspondence, 646
Retinal cysts, 402
Retinal degeneration, 403, 683
Retinal detachment, 36, 57, 122, 253,
267, 274, 282, 300,309, 324, 325,
326, 374, 384, 403, 429, 465, 478,
490, 549, 552
Retinal dialysis, 452
Retinal diseases in children, 410
Retinal drusen, 438
Retinal dysplasia, 410, 475, 480, 484
Retinal dystrophies and degenera-
tions, 434
Retinal dystrophy, 403
Retinal edema, 423, 750
Retinal electrode, 420
Retinal exudates, 277, 761
Retinal folds, 402
Retinal ganglion cells, 532
Retinal haemorrhage, 267, 326, 424,
685, 742, 744, 758, 767, 768
Retinal holes, 446, 487
Retinal hypoxia, 429
Retinal micro aneurysm, 426
Retinal micro angiopathy, 749
Retinal nerve layer, 246
Retinal periphery, 15
Retinal periphlebitis, 457, 766
Retinal phlebitis, 262
Retinal pigment degeneration, 78
Retinal pigment epithelium, 273, 383,
395, 398, 400, 414, 445, 492, 552
Retinal pigmentation, 487
Retinal pigmentepitheliopathy, 767
Retinal plane, 412
Retinal space, 153
Retinal surgery, 277, 354
Retinal system, 231
Retinal tear, 446
Retinal telangiectasis, 405
Retinal thinning, 451
Retinal toxocariasis, 432
Retinal vasculitis, 258, 433, 750, 760
Retinal veins, 398, 768
Retinal vessels, 414
Retinalblastoma, 489
Retinitis, 245, 246, 261, 422, 423, 424,
425, 760, 762
Retinitis albi punctatus, 418
Retinitis pigmentosa, 163, 238, 262,
283, 306, 400, 404, 418, 419, 422,
429, 435, 438, 439, 534, 576, 584
Retinitis proliferance, 427, 463
Retinitis punctata albescens, 438, 575
Retino blastoma, 17, 20, 90, 240, 260,
266, 283, 316, 317, 348, 368, 403,
406, 407, 411, 415, 421, 425, 433,
454, 471, 483, 488, 489, 542, 655,
678, 689, 695, 696, 697, 698, 701,
708, 771
Retino choroidal coloboma, 236
Retino choroiditis, 421, 428, 431, 432,
476
Retino phlebitis, 414
Retino schisis, 423
Retino-neural scotomas, 582
Retinoblastoma leukemia, 259
Retinochoroiditis, 239, 259
Retinocytom, 478
Retinol, 217
Retinol binding protein, 217
Retinoma, 478, 480
Retinopathies, 411, 423
Retinopathy, 424, 747
Retinopathy of pre-maturity, 28, 266,
348, 433, 454, 463, 466, 476, 484,
489, 666
Retinopathy of radiation, 708
Retinoschisis, 403, 446, 447, 451, 453
Retinoscopy, 164, 251, 246, 326, 412,
451, 457, 550, 552, 553, 723
Retraction nystagmus, 81
Retraction of the lid, 613
Retro bulbar anaesthesia, 599
Retro bulbar growths, 525
Retro bulbar haemorrhage, 687, 690
Retro bulbar mass, 348
Retro bulbar neuritis, 508, 529
Retro bulbar space, 706
Retro hyaloid space, 449
Retro illumination, 210, 251
Retro lamilar, 510, 521
Retro lental fibro plasia, 426, 427, 484
Retro lental white reflex, 406
Retro lenticular mass, 474
Retrocorneal vascularisation, 171
Retrolental fibroplasia, 316, 318, 352,
369, 414, 483, 496
Retrolental space, 273
Retrolental white mass, 460, 488
Retrolenticular, 483
Retrolenticular flare, 280
Retrovirus, 267
Reverbation, 700
Reverse coloboma of the disc, 519
Rhabdhomyosarcoma, 53, 471, 477,
687, 688, 690, 696, 698, 699, 703,
705, 706, 707, 709, 712
Rhabdomyoblast, 707
Rhabdomyocytes, 284
Rhagades, 207
Rhegmatogenous, 253, 411, 448
Rhegmatogenous detachment, 450
Rhegmatogenous retinal degenera-
tion, 445
Rhegmatogenous retinal detachment,
236, 305, 324, 404, 448, 487, 553
Rhese view, 694
Rheumatoid arthritis, 206, 240
Rheumatoid factor, 268, 271
Rhinitis, 129, 139, 143, 740, 748
Rhinorrhea, 734
Rhinosporidiosis, 49, 93, 119, 149
Rhinosporidiosis of sac, 99, 151
Rhinosporidium seebri, 93, 149
Rhizopus, 762
Rhodopsin, 217
Riboflavin deficiency, 136, 170
Riegers, 339
Riegers anomaly, 338
Rifampin, 137, 727
Riley day syndrome, 95 104, 221, 511
Rim, 673
Ring scotoma, 238, 440
Ring shaped opacity, 196
Ring synechiae, 247, 360
Ring ulcer, 178
Ringworm infection, 762
Rise of intraocular tension, 183
Risus-sardonicus, 728
River blindness, 152, 765, 771
RNA, 744, 749
Rod and rod-cone, 393, 394, 396, 399,
418, 434, 503
Rod monochromatism, 441, 442, 578
Rod spherule, 396
Rod-cone-break, 418
Roennes step, 582
Romanas sign, 768
Rombers syndrome, 237
Roof, 673
Root, 227
Ropy discharge, 120, 139, 141
Rosacea, 178
Rosaceakeratitis, 170
Rose bengal, 51, 172, 176, 178, 195,
201
Rosenthal Malcarson, 62
Roseola, 250
Rosette cataracts, 325, 326
Rosette concussion cataract, 326
Rosettes, 284, 325, 477, 482, 709
Rotary prism, 646
Roths, 267, 283, 425, 428
Round holes, 447
Roundworm, 489
Roving ring scotoma, 561
Roxithromycin, 432, 743
RPE, 382, 414
RPE detachment, 415
Rubella, 12, 240, 253, 258, 295, 298,
316, 317, 319, 400, 429, 529, 745,
746, 747
Rubella glaucoma, 429
Rubella retinitis, 429
Rubella retinopathy, 299, 741, 427
Rubella syndrome, 299, 747
Rubella uveitis, 261
Rubeola, 49, 253, 295, 429,
Rubeola retinitis (Measles retinitis),
429
Ruby, 383
Ruby laser, 384
Ruff of the iris, 229
Ruggiero, 694
Rule of 2-4-6, 630
Running of letters, 647
Rupture and folds in Descemets mem-
brane, 19, 162, 164, 169, 171,
345
Rupture and splitting of Descemets
membrane, 189
Rupture in Bowmans membrane, 164
Rupture in Desmets membrane, 97
Rupture of anterior vitreous faces,
358
Rupture of choroid, 535
Rupture of conjunctival vessels, 119
Rupture of cyst, 444
Rupture of sphinter, 326
Rush disease, 487
Ruthenium 106, 481
S
S. albus, 738
S. aureus, 737
S. epidermis, 738
S. hemolyticus, 738
S. saprophyticus, 738
Sabouraud medium, 177, 204
Sabourauds media, 763
Sac, 98
Saccade, 586, 596, 594
Saccoidosis, 97
Sacro iliac joint, 271
Sacroid nodule, 250
Sacroilitis, 271
Safe atropinisation, 182
SAFE method, 137
Sagital sutures, 680
Salicylate, 148, 257
Saline, 100
Salivary gland, 87, 96
Salivation, 374
Salmon patch, 741
Salmon red colour, 206
Salmonella typhi, 743
Salmonellosis, 743
Salpingitis, 733, 736, 757
Salt and pepper pigmentation, 429,
741
Salt and pepper spots, 262
Salt appearance, 439
Salt pepper, 440
Salt pepper appearance, 206
Salt pepper fundus, 400
Salzmanns nodular dystrophy, 147
Samolina, 150
Sand fly Phlebotomus, 767
Sandhoff disease, 443, 425
Sangunarine, 369
Saprophyte, 110, 279, 743, 759, 760,
761
Sarcoid in children, 269
Sarcoidosis, 96, 215, 240, 243, 246,
250, 253, 259
Satellite, 177, 182
Satellite conjunctival abscess, 192
Satellite epithelial keratitis, 195
Satellite lesion, 203, 431
Saturation, 578
Sayer syndrome, 624
Sbiza bar, 615
Scalooped, 244
Scalp injuries, 118
Scaphocephaly, 680
Scaphoid head, 680
Scar, 37, 415
Scar or the macula, 246
Scarring, 111
Scarring of conjunctiva, 120
Scattering of light, 168
Schilders disease, 509, 526, 527, 529,
530
Schiotzs tonometer, 347
Schirmer, 2, 105, 106
Schirmers test 1a, 105, 106
Schizomycetis, 759
Schlemms canal, 335, 342
School myopia, 549
Schwalbes line, 112, 307, 335, 338,
342
Scissors, 164
Sclera, 8, 10, 14118, 149, 152, 160,
230, 336, 697, 720
Sclera fixated lens, 309
Scleral buckle, 144, 408, 447
Scleral canal, 510, 519
Scleral crescent, 510
Scleral curvature, 163
Scleral dentation, 245
Scleral explant of suitable, 151
Scleral fixed lens, 563
Scleral indentation, 451
Scleral injuries, 324
Scleral involvement, 52, 150, 151
Scleral rigidity, 341
Scleral spur, 3, 112, 229, 335, 342
Scleral staphyloma, 151
Scleral sulcus, 335, 342
Sclerectomy, 381
Scleritis, 52, 97, 196, 201, 208, 732,
737, 738, 743, 746, 753, 763
Sclero cornea, 162
Sclero cornea of various degrees, 216
Sclero uveitis, 20, 239
Sclerocornea, 25, 168, 339, 722, 723
Sclerokeratitis, 201
Sclerosing keratitis, 732, 771
Sclerosis, 527
Sclerotomy, 379, 381
Scolio kyphosis, 308
Scopolamine, 506
Scotoma, 247, 253, 514, 526, 537, 574,
581
Scotopic, 417, 418, 586
Scotopic ERG, 441
Scotopic vision, 396, 574
Scrambled egg, 445
Searching movement, 29
Seasonal allergic conjunctivitis, 117,
139
Sebaceous glands, 98, 679
Seborrhic keratosis, 54
Seclusio pupillae, 250, 251, 354, 501
Second head somite, 600
Second malignancy, 690
Second neoplasm, 481
Second neoplasm in retinoblastoma,
479
Second neuron, 73
Secondary deviation, 651
Secondary angle closure, 354
Secondary aphakia, 559
Secondary congenital buphthalmos,
343
Secondary exotropia, 663, 665, 668
Secondary glaucoma, 52, 186, 188,
196, 202, 204, 235, 242, 247, 270,
271, 274, 325, 343, 380, 407, 429,
473, 487, 493, 755
Secondary implant, 321
Secondary lens fiber, 2, 292, 293
Secondary open angle glaucoma, 361
Secondary optic atrophy, 523, 537
Secondary position, 594
INDEX 815
Secondary vitreous, 3, 458
Secretion, 340
Sector iridectomy, 380
Sector retinitis pigmentosa, 438
See saw nystagmus, 630, 632, 634
Seen in atopic adults, 143
Seidel scotoma, 582
Seidel test, 176
Seizures, 770
Sella, 542
Sellar, 583
Sellaturcia, 694, 704
Semi circular canal, 632
Semi lunar fold, 35
Senile retinoschisis, 447
Sensation of cornea, 26, 80
Sensitivity done, 179
Sensory deprivation, 629
Sensory nystagmus, 234, 579
Sensory retina, 5, 395, 398, 403, 447
Sepic shock syndrome, 738
Septae, 760
Septed fungal hyphae, 204
Septic arthritis, 731
Septicemia, 424, 726
Septum orbitale, 37, 86, 675
Sero negative, 268
Serous retinal detachment, 265, 274,
416
Serpiginous choroditis, 258
Serpiginous ulcer, 736
Serum potassium, 377
Serum sickness, 760
Sessile, 150
Seton, 350
Setting sun appearance, 681
Setting sun sign, 613, 614
seventh nerve, 604, 745
Severe anemia, 424
Severe ptosis, 79
Shallow orbits, 678, 680
Shape of cornea, 25
Sheath of the optic nerve, 597
Sheathing, 433, 524, 534
Sheathing of blood vessels, 247
Sheep, 153
Sheridan ball test, 30
Sherophakia, 367
Sherringtons law, 595
Shield, 141
Shingles, 199
Short ciliary nerve, 502, 503, 510, 511
Short ciliary vessels, 5
Short posterior ciliary arteries, 6, 232,
414, 512
Short posterior ciliary nerves, 675
Short sightedness, 547
Short term escape, 376
Shot silk appear, 558
Shot silk look, 519
Shrinkage of sclera, 368
Shunt operations, 381
Shunts, 350, 415
Sialidosis, 443
Siblings, 347
Sickle cell anaemia, 425
Sickle cell disease, 364
Sickle cell haemoglobinopathy, 277
Sickle cell retinopathy, 414, 422, 427,
425, 434
Side effect of steroids, 257
Siderosis, 329
Sigmoid sinus, 704
Signs, 175
Signs and symptoms of proptosis are,
684
Signs of anterior uveitis, 242
Signs of aphakia, 560
Signs of Bells palsy, 82
Signs of chronic posterior uveitis, 247
Signs of intermediate uveitis, 245
Signs of papilledema, 523
Signs of posterior uveitis, 246
Signs of retinal detachment, 450
Signs of retinal diseases, 411
Signs of retinal disorders, 421
Signs of retinitis pigmentosa, 436
Signs of vitreous disorders, 459
Signs of vitreous haemorrhage, 463
Silent uveitis, 269
Silicon, 169, 456, 562
Silicon plug, 107
Silicone oil, 215, 357
Silver beaten impression, 521
Silver nitrate drop, 128
Silver nitrate solution, 734
Simple astigmatism, 565
Simple buphthalmos, 343, 344
Simple congenital ptosis, 42
Simple ectopia, 306
Simple follicular conjunctivitis, 120
Simple heterochromia, 237
Simple hypermetropic astigmatism,
565
Simple myopia, 549
Simple myopic astigmatism, 565
Simple P.C.O., 322
Simple ptosis, 70
Simplex, 253
Simply primary retinal dystrophy,
435
Simulated divergence excess, 667
Simulating dendritic ulcer, 767
Simultaneous macular perception,
653
Simultaneous perception, 638
Sinus, 703
Sinus histocytosis, 689
Sinusitis, 607, 735, 736
Sinusitis meningitis, 736
Sixth nerve, 82, 675, 705
Sixth nerve palsy, 523
Size of cornea, 25
Size of pupil, 501
Sjogren hand, 31
Sjogrens syndrome, 104
Skeletal changes, 310
Skew deviation, 613, 614, 615
Skin involvement, 57
Sleeping sickness, 768
Sling shot, 281
Slit lamp, 101, 326, 382, 416, 632
Slit lamp biomicroscope, 164, 412, 452
Slit like pupil, 234, 338
Sloan achromatopsia, 579
Sloughing ulcers, 175
Slow eye movement, 595
Slow fibres, 593
Slow phase, 629
Slow release inserts, 106
Small and medium sized KPs, 243
Small penetrating injuries, 325
Small pox, 186, 189, 219
Small pox virus, 745, 751
Smear, 179
Smooth pursuit, 594, 596
Snail track degeneration, 445, 446
Snail-track, 553
Snellens chart, 31, 443, 463, 513, 540,
547, 585, 589
Snellens drum, 568
Snellens optotypes10, 30
Snow balls, 245, 281, 762
Snow bank appearance, 276
Snow banking, 245, 272
Snow flake, 301, 446, 464
Snowball opacities, 272
Sodium, 103
Sodium chordrontin sulphate, 215
Sodium cromoglycate, 140, 142, 143
Sodium EDTA 0.5%, 249
Sodium hyaluronate, 215
Sodium nedocromil, 143
Soemmerring, 322, 323
Soft contact, 211
Soft cotton wool, 524
Soft exudate, 415, 424
Softening of eye, 174
Solid detachment, 455
Solid state, 383
Somnolence, 541
Sorsby pseudo inflammatory dystro-
phy, 435
Source of energy, 382
Space myopia, 555
Sparfloxacin, 179
Sparganosis, 765
Spasm of accommodation, 373, 509,
555, 614
Spasm of puncta, 373
Spasm of the ciliary body, 175
Spasmolytic drugs, 77
Spasmus nutans, 628, 630, 631, 633
Spastic phase, 608
Spatial, 570
Spatial localisation, 586
Special piggy back, 165
Specific retinitis, 428
Specific treatment, 179, 254
Specific treponemal, 262
Specific types of corneal ulcer, 190
Spectacle, 165, 309, 320, 321, 549,
550, 551, 553, 558, 564, 569, 659
Specular microscopy, 337
Speherophakia, 304
Spheno palatine artery, 89
Spheno palatine ganglion, 95
Spheno palatine sinus, 704
Sphenoid, 511, 598, 673, 704
Sphenoid ridge, 694
Sphenoid sinuses, 694
sphenoidal fissure, 676
Spherical aberration, 501
Spherical lens, 302
Spherophakia, 747
Spherule, 761
Sphincter pupillae, 227, 228, 502, 503
Sphincterotomy and iridoplasty, 379,
380
Spider shaped, 437
Spielmann, 615
Spike, 700
Spina bifida, 308, 679
Spina lateralis recti, 596
Spinal cord, 56, 530
Spinal ganglion, 194, 754
Spinal nerves, 56
Spine, 479
Spine for the lateral rectus, 677
Spino ponto cerebellar degeneration,
439
Spinocerebellar ataxia, 440
Spinomedullary junction, 634
Spiral of tillaux, 600
Spirochaetaceae, 740
Spirochete trapenoma pallidum, 206
Spiromycin, 261
Spontaneous hyphema, 284
Spontaneous regression, 478
Spontaneous venous pulsation, 518,
523
Spontaneously regressed tumour, 478
Sporangia, 149
Spore forming, 726
Spores, 149, 728, 759, 760
Sporotrichosis, 760, 764
Spread of comitance, 612
Spring catarrh, 23, 24, 120, 136, 147,
149, 163, 168, 328, 361, 362
Squamous, 45
Squamous carcinoma, 54
Squamous cell carcinoma, 750
Squamous cells, 54, 89
Squint, 13, 20, 21, 29, 71, 76, 122, 147,
168, 172, 178, 185, 186, 189, 236,
237, 248, 315, 316, 327, 432, 437,
487, 513, 517, 541, 549, 551, 557,
585, 621, 632, 641, 649, 679,
683,684, 706, 710, 723, 747, 766
St. Judes hospital classification, 483
Stage of fibro vascular proliferation,
485
Stage of infiltration, 125
Stage of line of demarcation, 485
Stage of ridge formation, 485
Stage of total retinal detachment, 486
Stage of white reflex, 406
Stages of development of retina, 399
Staining of corneal lesions, 176
Standing potential of the eye, 420
Staphyloccous, 110, 191
Staphylococcal blepharo-conjunctivi-
tis, 178, 193
Staphylococci, 123, 126, 177
Staphylococcus, 23, 53, 124, 127, 144,
258, 702, 737
Staphylococcus aureus, 122, 124
Staphylococcus corneal ulcer, 193
Staphylococcus epidermis, 280, 323
Staphyloma, 150, 186, 308
Stargards disease, 435, 443
Stargardts, 443
Stationary cone dystrophy, 434, 441
Stationary night blindness, 21, 420
Stationary stage, 170
Stem cell transplant, 107
Stem cells, 111
Stenopic slits, 455
Stenosis of aqueduct, 614
Stenosis of nasolacrimal duct, 93
Stereopsis, 413, 526, 533, 537, 563,
570, 594, 637, 638, 653, 666
Sterile hypopyon, 169
Sterile post operative endo-
phthalmitis, 281
Sterile pus, 181
Steriopis, 22, 588
Steroid, 174, 360, 733
Steroid drops, 269
Steroid induced cataract, 327
Steroid induced glaucoma in children,
361
Steroid over dose, 522
Steroid responders, 361
Steroids, 80, 191, 264, 607
Steven-Johnsons syndrome, 23, 63,
104, 106, 120, 121, 139, 148, 221,
377
Stevens Johnson, 148, 149
Sticklers syndrome, 446, 453, 464
Stigmata, 207
Stigmatic eye, 564
Still birth, 740, 765
Stilling, 579
Stills disease, 268
Stimulus deprivation amblyopia, 586,
587
Stocker and Holt, 211
Stomaititis, 754
Storage diseases, 536
Strabismic amblyopia, 586, 653
Strabismus, 13, 239, 246, 265, 277,
465, 474, 489, 538, 539, 540,
575, 614, 642, 682, 710
Strabismus fixus, 620, 623, 693
Straight eye amblyopia, 587, 590
Strands of liquid anterior vitreous,
460
Stratum opticum, 397
Strawberries, 60
Strawberry patches, 712
Streaks, 328
Strepto cocci, 123, 126
Strepto coccus, 23, 127, 144, 177, 191,
702, 739
Streptococcal, 258
Streptococci, 126
Streptococci pyogenes, 182
Streptococcus peumoniae, 735
Streptococcus pyogenes, 193
Streptomycin, 526, 529, 727
Stretching of sclera, 345
Stretching of the cornea, 344
Stretching of the zonules and the iris,
345
Stroma, 160
Stroma, 159, 161, 168, 187, 210, 228
Stroma of ciliary processes, 334
Stroma proper, 228
INDEX 817
Stromal cells, 167
Stromal dystrophies in children, 211
Stromal infiltration, 201
Stromal involvement, 195, 196
Stromal necrosis, 196
Stromal necrotic keratitis, 755
Stromal ring, 208
Stromal thickening, 755
Struge Weber syndrome, 45, 54, 55,
57, 68, 151, 306, 352, 368, 405,
678, 696
STUMPD, 89, 216
Stycar test, 31
Stye, 44, 47, 51, 97, 557, 565, 571, 647,
702, 738, 748
Sub acute, 425
Sub acute bacterial endocarditis, 422,
424, 428
Sub acute infective retinitis (Retinitis
of Roth), 428
Sub arachnoid haemorrhage, 532
Sub arachnoid space, 602
Sub capsular opacity, 252
Sub conjunctival, 44
Sub conjunctival cyst, 154, 770
Sub conjunctival haemorrhages, 23,
44, 50, 118, 130, 132, 768
Sub conjunctival injection, 111, 180,
182, 192,
Sub conjunctival patechial haemor-
rhage, 767
Sub dural space, 511
Sub epithelial opacities, 132, 326
Sub hyaloid haemorrhage, 426, 433,
463
Sub laxation, 252, 302, 304, 307, 314,
, 325, 345, 365, 367, 564
Sub luxation of lens, 19, 235, 326, 335,
448, 723
Sub mandibular, 38, 92, 114
Sub mandibular glands, 87, 702
Sub mandibular lymphnodes, 479
Sub normal vision, 17
Sub periostial abscess, 477
Sub retinal cysts, 770
Sub retinal fibrosis, 451
Sub retinal fluid, 273, 448, 488, 516
Sub retinal haemorrhage, 367, 427
Sub retinal neovascularisation, 247,
260, 429, 516, 747
Sub retinal space, 769
Sub-arachnoid haemorrhage, 426
Sub-conjunctival antibiotic injection,
184
Subacute sclerosing panencephalitis,
429
Subarachnoid, 479
Subarachnoid haemorrhage, 522
Subarachnoid space, 397, 479
Subdural hematoma, 522
Subdural lesion, 510
Subjective angle, 640, 653
Submento vertex view, 694
Subscapular opacification, 374
Substantia propria, 113
Sub tenon, 256
Subtentorial, 510
Subtle glioma of the disc, 437
Subtotal vitrectomy, 358
Succinyl choline, 657
Sugar alcohol, 301
Sujee, 150
Sulcus subtarsalis, 111
Sulpha, 148
Sulpha cetamide, 128, 137, 149
Sulpha methoxazol, 137, 271
Sulpha methoxazole with tri-
methoprim, 261
Sulpha-cetamide, 129, 739, 758
Sulphadiazine, 261, 432
Sulphite oxidase deficiency, 306, 314
Sulphonamide, 377, 432
Sulphur granules, 726
Sun flower cataract, 329
Sunny side up egg yolk, 444
Sunset appearance of the globe, 20
Super traction of retina, 552
Superior orbital fissure, 676
Supera choroidal lamina, 231
Superficial corneal ulcer, 767
Superficial exudate, 424, 428
Superficial haemorrhages, 370
Superficial keratitis, 49, 123, 134,
172, 203
Superficial punctate, 751, 754, 758,
759
Superficial stroma, 214
Superficial vascularisation, 170, 174
Superficial vascularisation of cornea,
170
Superior cerebellar, 612
Superior cerebellar arteries, 602, 603,
606
Superior cerebellar peduncle, 605
Superior cervical ganglion, 73, 502
Superior colliculum, 596, 601
Superior fornix, 98, 112
Superior limbus, 112
Superior oblique, 593, 597, 598, 670,
676
Superior oblique medial rectus, 674
Superior oblique myokymia, 613, 618
Superior oblique over action, 613, 615,
616
Superior oblique sheath syndrome,
616, 693
Superior ophthalmic vein, 87, 398,
675, 677, 704
Superior orbital fissure, 602, 604, 694,
697, 704
Superior orbital fissure syndrome,
607, 686, 690, 763
Superior orbital notch, 691
Superior orbital veins, 713
Superior rectus, 4, 36, 72, 70, 72, 74,
527, 593, 594, 597, 616
Superior rectus palsy, 609
Superior rectus under action, 653
Superior-olivary-nucleus, 604
Suppression, 587, 610, 625, 639, 640,
646, 664, 666
Suppression scotoma, 641, 653, 665
Supra choroidal space, 502
Supra nuclear, 614
Supra nuclear lesions, 613
Supra nuclear palsy, 728
Supra orbital, 200, 752
Supra orbital notch, 673
Supra trochlear, 200
Supra trochlear notch, 673
Suprachoroidal space, 230
Supraciliary space, 230
Suprasellar, 479, 583
Supratarsal fold, 36
Surface ectoderm, 9, 161, 293
Surface probe, 382
Surface saprophyte, 730
Surgery, 657
Surgery in childhood glaucoma, 378
Surgical anatomy of the limbus, 345
Surgical aphakia, 26
Surgical procedures for correction of
myopia, 554
Surgical spaces of the orbit, 675
Surgical treatment, 205
Surma, 117, 133, 218
Sursum duction, 595
Sursum version, 595
Suspensory ligaments, 290
Sustained release acetazolamide, 377
Sutural, 296
Sutural cataracts, 298
Suture lines of the orbit, 679
Suturelysis, 385
Sweat glands, 679
Swinging flash light test, 507
Swollen lens, 335, 354
Symblepharon, 59, 81, 121, 125, 148,
730, 742
Sympathelic ophthalmia, 253
Sympathetic, 38, 87, 228
Sympathetic chain, 530, 708
Sympathetic fibres, 511
Sympathetic heterochromia, 237
Syphilitic uveitis in pediatric age
group, 261
Syrengiomyelitis, 522
Syringing, 93
Systemic acetazolamide, 184
Systemic herpes simplex, 430
Systemic involvement, 58
Systemic steroid, 177
T
T. corporis, 762
T. facici, 762
T. helper lymphocytes, 267
T. pedis, 762
T-cell mediated, 276
T-cells mediated immunity, 276
Tabes dorsalis, 533
Tachycardia, 374
Tachyphylaxis, 117, 374
Taenia echinococcous (Hydatidcyst),
768
Taenia echinococcus, 765
Taenia glomeratus, 765
Taenia multiceps, 765
Taenia solium, 152, 765, 769
Talengiectasis, 409
Tape-worms, 768
Tapeto meninges, 57
Tapeto retinal dystrophy, 575
Tapetoretinal degeneration, 576
Tarsal, 141
Tarsal conjunctiva, 36, 37, 111, 117,
120, 134, 144, 150
Tarsal cyst, 48, 68
Tarsal fold, 70
Tarsal glands, 111
Tarsal ligaments, 675
Tarsal plate, 35, 37, 97, 110, 117, 675
Tarsitis, 730
Tarsoraphy, 52, 62, 107, 184, 203,
541, 680, 682, 711
Tarsus, 37, 674
Taste test, 93
Tattoo, 188
Tay Sachs disease, 17, 425, 442
Tear, 88, 448
Tear film, 159, 161, 167, 176
Tear film abnormality, 210, 216
Tear film break, 105
Tear film break up, 62, 218
Tear film stability, 105
Tear film status, 80
Tear film study, 176
Tear film substitute, 143, 198
Tear in Descemets membrane, 216
Tear meniscus, 102
Tear osmolarity, 106
Tear quality, 105
Tear quantity, 105
Tear substitutes, 220
Teeth, 679
Telangiectasia, 114, 119, 404, 537
Telangiectasia of retina, 115
Telangiectasis, 415, 422, 424, 488
Telecanthus, 22, 39, 40, 71, 666
Teller acuity card, 29
Temporal bone, 620
Temporal fossa, 674
Temporal long posterior ciliary ar-
tery, 6
Temporal pallor, 532, 535
Temporalis muscle, 673
Tempro sphenoidal lobe, 521
Tenonitis, 703, 707
Tenons capsule, 3, 111, 120, 336, 350,
623, 676, 720
Tenons space, 676
Tenosynovitis, 733
Tensilon, 77, 80, 505
Tensilon test, 76, 77, 624
Tentorium, 612
Teratoid, 284
Teratoma, 679, 688, 696, 698, 707
Tersons syndrome, 426, 463
Tertiary position, 594
Tertiary vitreous, 2, 3, 9, 292, 457, 458
Test for abnormal retinal correspond-
ence, 653
Test for suppression, 653
Testes, 746
Tetanospasmin, 728
Tetanus prone injuries, 729
Tetanus prone ocular injuries, 729
Tetanus toxoid, 529, 729
Tetracyclin, 126, 128, 133, 137, 193,
261, 522, 726, 729, 739, 742, 743
Tetrad, 71
Thalidomide, 295
Thayer Martin medium, 127
Thelazia californiensis, 154, 765, 772
Thelazia calipaeda, 154, 765, 772
Thelaziasis, 152, 154, 765, 772
Therapeutic, 28, 263
Therapeutic contact lens, 195
Thermal sclerotomy, 350, 381
Thermo keratoplasty, 165
Thiabendazole, 490
Thick almost organised hypopyon,
177
Thickening of choroids, 274
Thickness of lens, 417
Thickness of the cornea, 207, 417
Third cranial nerve, 502
Sympathetic ophthalmia, 239, 240,
243, 258, 259, 272, 275, 328, 526
sympathetic over action, 80
sympathetic plexus, 708
Sympathetic ptosis, 73
Sympathetic uveitis, 258, 273, 277
Sympathizing eye, 273
Sympatho-mimetics, 372
Sympatholytic drugs, 80
Sympathomimetic, 20
Sympathomimetic drugs, 80, 255, 374
Symptomatology of orbital disorders,
684
Symptoms, 92, 150
Symptoms of acute anterior uveitis,
242
Symptoms of aphakia, 560
Symptoms of astigmatism, 565
Symptoms of chronic posterior
uveitis, 246
Symptoms of congenital and develop-
mental cataract, 315
Symptoms of corneal opacities, 189
Symptoms of diseases of vitreous, 459
Symptoms of ectopia lentis depend
mostly on, 304
Symptoms of heterophoria, 647
Symptoms of hypermetropia, 557
Symptoms of intermediate uveities,
245
Symptoms of lagophthalmos, 62
Symptoms of optic nerve disorder, 520
Symptoms of papilledema, 522
Symptoms of posterior uveitis, 245
Symptoms of retinal diseases in the
children, 411
Symptoms of traumatic cataract may
be, 326
Synchisis, 461
Synchisis scintillans, 461
Synchisis senilis, 449
Syndactyly, 62, 439
Syndrome of triad, 299
Syndromes, 13
Synechia, 342
Syneresis, 461
Synergist muscle, 594
Synkinetic, 79
Synkinetic movements, 72
Synkinetic movements of the lid, 42
Synoptophore, 611, 639, 644, 640,648,
653
Synoptophore test, 645, 646, 653
Syphilis, 12, 121, 239, 240, 249, 253,
258, 400, 430, 432, 529, 740, 750
Syphilitic iridocyclitis, 250
Syphilitic nodules, 250
INDEX 819
Third head somite, 600
Third nerve, 675
Third nerve palsy, 563
Third ventricle, 541, 711
Thread worm, 773
Three Cs, 260
Thresh hold disease, 486
Thrombo phlebitis, 705
Thrombocyotopaenic purpura, 119,
299
Thrombocytopenia, 492, 712, 747, 755
Thromboembolic phenomenon, 311
Thrombophlebitis, 526
Thrombosis of retinal vein, 171
Thromobo embolic episodes, 310
Thygesons keratitis, 195
Thymectomy, 78
Thymidine analog, 197
Thymoma, 77
Thymoxamine, 505
Thyroid eye disease, 623, 624
Thyroid myopathy, 417, 621
Thyroid ophthalmopathy, 657
Thyrotoxicosis, 44, 118
Tick ixodes, 742
Time amplitude, 417, 700
Timolol, 375, 376
Tincture iodine, 50, 756
Tinea capitis, 762
Tinitus, 276, 632
Tip cat, 281
Titanium oxide, 60
Titmus fly test, 640
Tobacco dusting, 451
Tobracin, 179
Tobramycin, 129, 179, 180, 737, 743
Toga viruses, 298
Tolosa Hunt syndrome, 607
Tonography, 341
Tonopen, 347
Tonsillitis, 754
Topographic location of sixth nerve
lesion, 619
Torch test, 317, 429
Toric bulge, 163
Toric contact lenses, 567
Torsional, 595
Torticolis, 632, 656
Total afferent pupillary defect, 507
Total anterior staphyloma, 186
Total cataract, 296
Total colour blindness, 579
Total hypermetropia, 556
Total hyphaema, 363
Total internal reflection, 164
Total nuclear cataract, 298
Total ophthalmoplegia, 510, 728
Total retinal detachment, 407
Total third nerve palsy, 510
Towens view, 695
Tower skull, 679
Townes view, 694
Toxic amblyopia, 529, 586
Toxic epidermal necrolysis, 738
Toxic neuritis, 535
Toxic neuropathy, 527
Toxic nystagmus, 630
Toxic retinopathy, 534
Toxic vasculitis, 370
Toximia of pregnancy, 434
Toxin, 160, 167
Toxocara, 258, 278, 279, 431, 701
Toxocara canis, 240, 432, 489, 765
Toxocara canis round worm, 264
Toxocara catis, 489
Toxocara infection of uvea, 264
Toxocariasis, 668, 765, 770
Toxopalsma, 764
Toxoplamosis uveitis, 259
Toxoplasma, 267, 750
Toxoplasma gondi, 240, 259, 765
Toxoplasmal chorioretinitis, 263, 428,
431, 535
Toxoplasmal retinitis, 431
Toxoplasmosis, 12, 16, 239, 243, 253,
258, 295, 317, 318, 410, 430, 431,
433, 529, 741, 750, 765
Trabecular dysgenesis of angle of
anterior chamber, 344
Trabecular mesh, 247
Trabecular meshwork, 52, 112, 234,
335, 338, 339, 341, 342, 363, 382
Trabecular meshwork develops, 336
Trabeculectomy, 57, 278, 338, 349,
350, 361, 371, 381
Trabeculitis, 52, 202, 245, 247, 360
Trabeculo dysgenesis, 339
Trabeculodialysis, 337, 361
Trabeculodysgenesis, 337
Trabeculoplasty, 385
Trabeculostomy, 379, 381, 980
Trabeculotome, 350
Trabeculotomy combined with
trabeculectomy, 349
Trachoma, 23, 104, 117, 119, 120, 133,
139, 140, 142, 147, 149, 170, 172,
173, 178, 207, 756, 757
Trachoma dubium, 134
Trachomatis, 133
Trachomatous entropion, 63
Trachomatous inflammation, 135
Trachomatous scarring, 135
Trachomatous trichiasis, 135
Traction detachment, 245, 247, 433
Traction displacement of macula, 265
Tractional, 453
Tractional retinal detachment, 453,
454
Train nystagmus, 632
Tram track, 57
Trans ethmoidal, 541
Trans nasal, 541
Trans-illumination, 338, 412
Transcleral cyclocryo, 369
Transcranial, 711
Transducer, 417, 699
Transferase galactokinase, 300
Transient corneal haze, 299
Transient myopia, 373, 377
Transient neonatal myasthenia, 76
Transilluminate, 237
Transnasalendoscope, 675
Transplacental, 431, 766
Transplacental factors, 12
Transplacental spread, 430
Transpupillary, 382
Transscleral, 382
Transverse myelitis, 746, 752, 753
Trauma, 13, 160, 188, 249, 425, 462,
508
Trauma to orbit, 118
Traumatic, 151
Traumatic aphakia, 452
Traumatic cataract, 324, 407, 433,
475, 484
Traumatic cysts, 151
Traumatic glaucoma in children, 354
Traumatic maculopathy, 516
Traumatic miosis, 74
Traumatic optic atrophy, 535
Traumatic optic neuropathy, 45, 508,
533, 535
Traumatic paralysis of third nerve,
606
Traumatic phacogenic glaucoma, 366
Treacher collins syndrome, 682
Treacher collin, 39
Treatment of acute uveitis, 254
Treatment of ecchymosis, 45
Tremulouness of iris, 305, 311, 366
Tremulous, 561
Tremulousness of lens, 345
Treponema pallidum, 740
Tretinoin, 220
Triamcinolone, 713
Triamcinolone acetonide is, 48
Triamsonolone, 361
Triangular head, 680
Triatoma, 768
TRIC, 133
Tricarbocyanine, 416
Trichiasis, 40, 41, 45, 51, 52, 63, 121,
125, 148, 173, 200, 682, 730
Trichinellaspiralis, 765
Trichinosis, 765
Trichlor acetic acid, 181
Trifluorothymidine, 197
Trifluridine, 197, 198, 199
Trigeminal, 38, 43, 87, 114, 160, 194,
200, 228, 596, 597, 752
Trigeminal ganglion, 131, 753, 754
Trigeminal nerve, 51, 171, 175, 576
Trigeminal neuritis, 172
Trigeminal reflex, 504
Trigger mechanism, 194
Trigonocephaly, 680, 695
Trilateral retinoblastoma, 474, 479,
482
Trimethoprim, 271, 736
Trimethoprimesulpha methoxazole,
726, 727, 730, 744
Triple sulpha, 261, 432
Trismus, 728
Tritanomalous, 578
Tritanomaly, 578
Ptritanopia, 578
Trivalent antitoxin, 729
Trochlea, 593, 598, 601, 612, 617, 676,
677, 691, 704
Trochlear fossa, 673
Trochlear nerve, 603, 674
Trophic, 201
Trophic ulcer, 196, 201
Trophozoite, 208
Tropias, 571
Tropicamide, 181, 208, 254, 318, 506
Troticollis, 633
True congenital glaucoma, 344
True membrane, 121, 123, 148
True membranous conjunctivitis, 125
True polycoria, 27, 229
True proptosis, 348
True ptosis, 68
Trypanosoma, 764
Trypansoma cruzi, 768
Trypansoma gambiense, 768
Trypansome chancre, 768
Trypansomiasis, 768
Tsetse fly, 768
Tubercle bacilli, 146
Tubercles, 249, 731
Tubercular chorio retinitis, 431
Tubercular choroiditis, 260
Tubercular conjunctivitis, 731
Tubercular meningitis, 508, 732
Tubercular nodule, 250, 263, 732
Tubercular protein, 144
Tubercular sinus, 732
Tuberculoma, 263, 522, 731, 732
Tuberculosis, 97, 121, 144, 149, 171,
206, 239, 240, 243, 253, 258, 430,
432, 433, 526, 529, 702, 764
Tuberous sclerosis, 55, 58, 492, 542
Tubular vision, 436
Tucking, 617
Tumescent, 367
Tumour, 19
Tumour node metastasis system, 483
Tumour related glaucoma, 368
Tumours of optic nerve, 538
Tumours of optic nerve head, 542
tumours of the optic nerve, 417
Tunica vasculosa lentis, 4, 6, 233, 235,
293, 457, 458
Tunica vesculosa lentis, 458
Turbid aqueous, 242
Turners syndrome, 315
Two focal lines, 563
Tylosis, 45, 98
Tympanic membrane, 82
Type I diabetes, 434
Type I herpes simplex, 194
Type of esophoria, 647
Type one allergy, 138
Types of anisometropia, 567
Types of astigmatism, 564
Types of exophoria, 648
Types of heterophoria, 644, 650
Typhoid (enteric fever), 743
Typical coloboma of eye, 8
Typical coloboma of uvea, 1
Typical retinitis pigmentosa, 435
Tyrosinase, 401
Tyrosinase negative, 236, 400, 401
Tyrosinase positive, 236, 400, 401
Tyrosine, 400, 401
Tyrosine hydroxylase, 236
U
UGH, 357
Uhthoff phenomenon, 527
Ulcer, 45, 62, 195, 216
Ultra filtration, 340
Ultra sonography, 488
Ultra violet, 161, 382, 577
Ultra violet ray, 194
Ultrasonic cycloablation, 381
Ultrasonic pachymetry (Pachometry),
417
Ultrasonography, 274, 281, 407, 412,
416, 452, 455, 474, 553, 699, 769
Ultrasound, 349
Ultraviolet light, 59, 173
Umblicated vesicle, 133
Uncal herniation, 606
Unconventional outflow, 341
Uncorrected errors of refraction, 116
Uncorrected hypermetropia, 32
Under action of inferior oblique, 71
Under action of orbicularis, 76
Unformed hallucination, 581
Unharmonious ARC, 639
Unifocal, 563
Unilateral abducent palsy, 656
Unilateral Argyll Robertson pupil,
509
Unilateral buphthalmos, 695
Unilateral diplopia, 305
Unilateral ectopia lentis, 304
Unilateral facial palsy, 82
Unilateral fourth nerve palsy, 611
Unilateral loss of vision, 29
Unilateral retinitis pigmentosa, 438
Unilateral sub acute neuro retinitis,
265
Uninary homocystine, 309
Uniocular aphakia, 567, 569
Uniocular diplopia, 164
Unoprostane, 376
Unstratified muscles, 228
Untowards effect of epinephrine, 374
Up gaze paralysis, 81
Upbeat nystagmus, 634
Upper fornix, 87, 110
Upper lid, 34
Upside down ptosis, 37, 74, 511
Urea, 103, 372, 378
Urease, 743
Uremia, 215
Urethritis, 133, 271, 733
Urticaria, 139
Uses of ERG, 419
Ushers syndrome, 439
Uthoff phenomenon, 526
Uvea, 4, 56
Uveal circulation, 6
Uveal dystrophies, 576
Uveal effusion syndrome, 723
Uveal meshwork, 336
Uveal pigments, 242
Uveal reaction, 191
Uveal system, 6
Uveascleral outflow, 375
Uveitis, 24, 44, 52, 170, 214, 314, 326,
407, 422, 728, 744, 758, 759, 760,
763, 766, 767
Uveitis glaucoma haemorrhage syn-
drome, 356
Uveitis glaucoma hyphaema, 357
Uveitis in children, 238
Uveitis in herpes zoster, 201
Uveo retinal coloboma, 515
INDEX 821
Uveo scleral out, 252
Uveo-scleral outflow, 366
Uveokeratitis, 52
Uveoscleral, 341
Uveoscleral channel, 231
Uveoscleral outflow, 373, 375, 376
Uveovertex, 341
Uyemuras disease, 444
V
V pattern, 611, 655
V phenomenon, 616
Vaccines, 528
Vaccinia, 745, 751
Vacuoles, 328
Vaginitis, 762
Vagotonic reflex, 504
Valaciclovir, 197
Valproate, 635
Valselva maneuver, 692
Valselva procedure, 540
Valves, 350
Vancomycin, 192, 261, 727, 739, 740
Vanishing optotypes, 30
Variants of a typical retinitis
pigmentosa, 437
Variants of Duanes retraction syn-
drome, 623
Variation of retinitis pigmentosa, 435
Varicella, 49, 429, 745, 751
Varicella Zoster Viral Keratitis, 199
Varicella zoster virus, 49, 193, 751
Variola, 745
Various methods available to diag-
nose ARC, 640
Various methods employed to lower
sudden rise of i, 380
Various uses of laser in glaucoma, 384
Varnished macula, 444
Vasa hyaloidea propria, 6
Vascular causes of white reflex, 477
Vascular occlusion, 416
Vascular retinopathies in children,
434
Vascular retinopathy, 383
Vascularisation, 148, 188
Vascularisation of cornea, 26, 135,
183, 201, 768
Vasculitis, 148, 246, 260, 274, 428,
429, 705
Vasocons-trictors, 118, 139, 140, 242,
Vasoformative, 169
Vector control, 137
Venae verticosae, 5, 676, 720
Venography, 693, 713
Venous congestion, 685
Venous drainage of uvea, 232
Venous obstruction, 416
Venous phase, 415
Venous stasis, 524
Ventral ophthalmic artery, 6
Ventricle, 59, 517
Ventricular decompression, 613
Ventriculo peritoneal, 525
VEP, 16
Vergence, 594, 595, 596
Vergence eye movement, 595
Vericella, 199, 200
Vericella herpes zoster virus, 752
Vericella zoster virus, 131
Vernal catarrh, 117, 170, 328
Vernal conjunctivitis, 120, 173
Vernal kerato conjunctivitis, 139, 206
Vernier acuity, 586
Versions, 594, 595
Vertebrae, 622
Vertex vein, 599, 720
Vertical angle kappa, 612
Vertical gaze palsy, 613
Vertical nystagmus, 614
Vertical prism test, 640
Vertical school, 669
Vertical squint, 610, 681
Vertical tropia, 79
Vertigo, 275, 276, 632, 651
Vescicle formation, 148
Vescicles, 200
Vesciculo bullus stage, 148
Vessels of tarsal conjunctiva, 160
Vestibular, 596
Vestibular and cochlear reflex, 504
Vestibular disturbance, 439
Vestibular evoked nystagmus, 632
Vestibular nystagmus, 62, 621, 629
Vestibulo ocular movements, 594
Vetrectomy, 261
Vidarabine, 197
Video cassette recorder, 416
Vincristine, 482, 708, 709
Viral conjunctivitis, 120
Viral infection, 82
Viral infection of the lids are, 49
Viral retinitis in children, 429
Viral ulcers, 175, 183
Viridans, 739
Virtual erect image, 166
Virtual image, 412, 413
Virus, 745
Visceral involvement, 56
Visceral larva migrance, 265, 489, 770
Visco elastic, 279, 356, 364
Viscoelastic substance, 274
Visible pulsation, 691
Vision, 79, 143, 175
Vision in optic neuritis, 527
Vision of a new born, 14
Vision of neonate, 16
Visual acquity, 79, 421
Visual aphasia, 574, 580
Visual axis, 593, 596
Visual cells, 395
Visual cortex through tecto bulbar
tract, 601
Visual evoked occipital potential, 16
Visual evoked response, 412, 420, 586
Visual hallucination, 574, 581
Visual purple, 393
Visual response, 15
Visually challenged, 168
Visually evoked potential, 29
Visually provoked response, 21
Visuocortical tract, 529
Visuscope, 641
Vital dye staining, 105
Vital stains, 176
Vitamin A, 198, 217, 395
Vitamin A deficiency, 111, 138, 172,
173, 179, 183, 189, 238, 418, 576,
748
Vitamin A deficiency disease (VAD),
173
Vitamin D, 297
Vitamin D toxicity, 215
Vitamin E deficiency, 487
Vitelli form, 444
Vitelli form macular dystrophy, 444
Vitelliform dystrophy, 435
Vitiligo, 45, 274, 275, 402
Vitrectomy, 266, 272, 278, 279, 281,
282, 322, 354, 427, 433, 488, 727,
728, 743
Vitreitis, 246
Vitreo retinal defects, 339
Vitreo retinal degeneration, 464
Vitreo retinal dystrophies, 435
Vitreo retinal pathology, 417
Vitreo-retinal attachment, 457
Vitreous, 10, 153, 230, 244, 305, 393
Vitreous abscess, 278, 280, 760
Vitreous bands, 58, 247, 252, 324, 405,
453
Vitreous base, 230, 456
Vitreous cells, 242, 274
Vitreous chamber, 230, 290, 335
Vitreous changes in chronic anterior
uveitis, 252
Vitreous debris, 245
Vitreous degeneration, 461, 549
Vitreous detachment, 446, 461
Vitreous disturbance, 462
Vitreous flare, 246
Vitreous floaters, 246, 459, 552
Vitreous haemorrhage, 28, 260, 326,
365, 427, 445, 461, 462, 552, 577,
766
Vitreous haze, 246, 247
Vitreous in AC, 169
Vitreous membrane, 742
Vitreous opacities, 262, 437, 460, 558
Vitreous precipitates, 260
Vitreous seeding, 473, 478, 481
Vitreous volume, 377
Vitrial causes of diminished vision,
460
Vitritis, 245, 263, 432, 459, 489, 732,
761
Vogt Koyanagi, 259
Vogt Koyanagi form, 276
Vogt Koyanagi Harada syndrome,
253, 275
Vogt-Koyanagi-Harada, 240, 258
Volk, 245, 412
Voluntary nystagmus, 630, 633
Von Hippel Lindaus disease, 55, 151,
407, 476, 492
Von Hippel-Lindau, 405
Vortex, 421
Vortex veins, 230, 231, 232, 233
Vossius ring, 326
W
Waarden burg syndrome, 40, 402,
423, 682, 683
Waardenburg, 237
Wagner, 453
Wagners disease, 446, 464, 465
Wagners vitreoretinal dystrophy, 435
Wall eyed bilateral inter nuclear
ophthalmoplegia, 605
Walls of the orbit, 697
Watch glass, 159
Waters view, 693
Watery discharge, 24, 120
Watts, 382
Waxy pale, 436, 437
Waxy white, 534
Webers syndrome, 73
Webino, 605
Wegner granulo-matosis, 526
Weill-Marchesani syndrome, 302
Weiss ring, 450, 462
Wernickes hemianopic pupil, 507,
508
Wessely ring, 203
Western blot test, 268
White forelock syndrome, 683
White granular dots, 750
White iritis in girls, 241
White KP, 196
White light, 577
White reflex, 153, 236, 265, 316, 403,
407, 411, 433, 473, 485, 488, 492
White reflex in pupil, 16, 322, 473
White reflex in pupillary, 28, 283, 284,
317, 323, 473, 475, 771
White uveitis, 269
White uveitis syndrome, 258
White with pressure, 445, 446
White without pressure, 446
Whitening of scleral spur, 366
Whitnall ligament, 9, 36, 37, 87, 676
Whitnall tubercle, 37
WHO, 134
WHO grading, 135
Whooping cough, 23, 119, 730
Wide angle, 307
Wide angle glaucoma, 356, 370
Widening of ciliary band, 366
Width of the inter palpebral fissure,
79
Wilms, 19
Wilms tumor, 235, 687, 688, 707, 708
Wilsons disease, 301
Window reflex keratoscope, 166
Windows reflex, 175
Winged scapulae, 308
Wise lens, 383
Wolfring, 87
Word blindness, 580
Worm infection, 219
Worth four dot test, 568, 640, 641,
653, 663
Worth ivory ball, 30
Wyburn-Mason syndrome, 55, 405,
409
X
X linked retino chisis, 420
X-ray, 326
X-ray of orbit, 540
X-ray optic foramen, 540
Xanthophyll, 382, 383, 384, 394, 415,
416
Xanthopsia, 577
Xenon, 383, 409
Xeroderma pigmentosa, 54, 59, 151
Xerophtalmia secondary, 106
Xerophthalmia, 106, 121, 125, 133,
142, 168, 172, 190, 278, 404, 576,
730, 748
Xerophthalmia and Keratomalacia,
216
Xerophthalmia scar, 218
Xerophthalmic fundus, 218
Xerosis, 105, 148, 165, 216, 440
Xerosis bacilli, 218
Xylocaine, 105, 106, 176, 255, 773
Y
Y sutures, 293
YAG laser, 211
Yeast, 760, 762
Yellow grey barrier filter, 414
Yellow prickly poppy, 369
Yoke muscles, 594
Yto megalo virus, 193
Z
Zeis, 38
Zinc, 537
Zinc oxide, 60
Zinc sulphate, 735
Zonular cataract, 297
Zonule, 9, 230, 303, 305, 456,
Zonules of the lens, 2, 458
Zoonosis, 727, 741, 767, 770
Zoster, 173, 174
Zoster varicella virus, 50
Zygomatic, 34, 673, 674
Zygomatic arch, 694
Zygomatic bone, 674
Zygomatic nerve, 674, 677

Pediatric Ophthamology 2005

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