Wong

B1037 The Ophthalmology Examinations Review FA
CONTENTS
Introduction to Second Edition v
About the Authors vii
Section 1: Cataract and Cataract Surgery 1

Topic 1: The Lens 3
Topic 2: Cataracts 7
Topic 3: Congenital Cataracts 11
Topic 4: Cataract Surgery 15
Topic 5: Anesthesia and Viscoelastics 19
Topic 6: Intraocular Lens 23
Topic 7: Cataract Surgery in Special Situations 29
Topic 8: Cataract Surgery Complications 35
Topic 9: Subluxed Lens and Marfans Syndrome 41
Section 2: Glaucoma and Glaucoma Surgery 45

Topic 1: Limbus, Ciliary Body and Trabecular Meshwork 47
Topic 2: Aqueous Humor and Intraocular Pressure 51
Topic 3: Optic Disc Changes in Glaucoma 55
Topic 4: The Visual Fields 57
Topic 5: Gonioscopy 61
Topic 6: Congenital Glaucomas 65
Topic 7: Open Angle Glaucomas 71
Topic 8: Angle Closure Glaucoma 77
Topic 9: Secondary Glaucomas 81
Topic 10: Medical Treatment of Glaucoma 89
Topic 11: Laser Therapy for Glaucoma 95
Topic 12: Surgical Treatment for Glaucoma 99
Contents ix
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Section 6: Neuroophthalmology 267

Topic 1: Ocular Motility and Multiple Cranial Nerve Palsies 269
Topic 2: Third Cranial Nerve Palsy 273
Topic 3: Sixth Cranial Nerve Palsy 277
Topic 4: Neurological Approach to Ptosis 281
Topic 5: Myasthenia Gravis 283
Topic 6: Nystagmus 287
Topic 7: Pupils 291
Topic 8: Optic Neuropathies 297
Topic 9: Optic Neuritis and Multiple Sclerosis 303
Topic 10: Visual Field Defects in Neuroophthalmology 309
Topic 11: Pituitary and Chiasmal Disorders 313
Topic 12: Papilledema and Intracranial Tumors 317
Topic 13: Strokes, Migraines & Other Vascular Disorders 321
Topic 14: Neuroophthalmic Manifestations of Cerebral Aneurysms 327
Topic 15: Neurocutaneous Syndromes 329
Topic 16: Head Injury 335
Topic 17: Coma, Disorders of Higher Functions & Psychiatric Diseases 337
Topic 18: Other Neuroophthalmic Problems 341
Section 7: Oculoplastic and Orbital Diseases 345

Topic 1: The Eyelids and Orbit 347
Topic 2: Ptosis 349
Topic 3: Entropian and Ectropian 353
Topic 4: Lid Tumors 357
Topic 5: Facial Nerve Palsy 363
Topic 6: Thyroid Eye Disease 367
Topic 7: Proptosis & Orbital Tumors 375
Topic 8: Epiphora 381
Topic 9: Enucleation, Evisceration & Other Orbital Surgeries 385
Section 8: Uveitis, Systemic Diseases and Tumors 389

Topic 1: Introduction to Uveitis 391
Topic 2: Systemic Infectious Diseases and the Eye I 395
Topic 3: Systemic Infectious Diseases and the Eye II 401
Topic 4: Toxoplasmosis and the Eye 403
Topic 5: Arthritis and the Eye 407
Topic 6: Connective Tissue Diseases and the Eye 413
Topic 7: Specic Uveitis Syndromes I 417
Topic 8: Specic Uveitis Syndromes II 421
Topic 9: Anterior Segment Tumors 427
Contents xi
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FA B1037 The Ophthalmology Examinations Review
Topic 10: Posterior Segment Tumors 431

Topic 11: Immunosuppressive Therapy, Steroids and Atropine 437
Section 9: Squints and Pediatric Eye Diseases 443

Topic 1: Assessment of Strabismus 445
Topic 2: Binocular Single Vision 449
Topic 3: Amblyopia 455
Topic 4: Esotropia 459
Topic 5: Exotropia 463
Topic 6: Vertical Squints and Other Motility Syndromes 465
Topic 7: Strabismus Surgery 469
Topic 8: Retinoblastoma 473
Section 10: Miscellaneous Examination Problems 481

Topic 1: Ocular Trauma 483
Topic 2: Color Vision 487
Topic 3: Lasers in Ophthalmology 491
Topic 4: Vitamins, Alcohol, Drugs and Skin 493
Topic 5: Epidemiology, Public Health and Research Methods 497
Topic 6: Last Minute Physiology 501
Explanation of Terms 503

Common Abbreviations 505
Index 509
xii The Ophthalmology Examinations Review
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Section 1
Cataract and
Cataract Surgery
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TOPIC 1
THE LENS
Overall yield: Clinical exam: Viva: Essay: MCQ:
Q Opening Question: What is the Anatomy of the Lens?

The lens is located between the anterior and posterior segments of the eye.
Anatomy of the Lens

1. Gross anatomy Non-equatorial zone

Biconvex, transparent structure that divides eye Transports solutes between lens and
into anterior and posterior segment aqueous humor

Secretes capsular material
General dimensions: 10 mm diameter, 4 mm
All epithelial cells are nucleated
thickness, 10 mm anterior surface radius, 6 mm
Cytoplasm contains organelles (ribosomes,
posterior surface radius
sER, rER, GA, mitochondria)
2. Microscopic anatomy Lens bers
Capsule Divided into cortex and nucleus
Acellular elastic structure Cortex
Similar to basement membrane (type 4 Suture lines (anterior Y shape, posterior
collagen) inverted Y)
Zonules run from ciliary processes and fuse Only the young lens bers have normal
onto outer layer of capsule cellular organelles which subsequently
Main function is to mould the shape of lens disintegrates with aging
in response to tension on zonules Newly formed cortical bers elongate,
Important barrier to forward migration of the with one end of the cell moving anteriorly
vitreous during/after lens extraction and the other end posteriorly
Anterior epithelium Nucleus
Functionally divided into 2 zones: Consists of cells that have been retained
Equatorial zone throughout life

Actively dividing and differentiating into Metabolism of cells in the nuclear region
lens cell bers is minimal
Exam tips:
Not a very common question, but considered basic anatomical knowledge.
In general, anatomy questions like What is the anatomy of. can be
answered by rst dividing the structure into gross and microscopic anatomy.
Q What is the Function of the Lens? Why is it Transparent?

The main functions of the lens are
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Functions of Lens
1. Function of lens Small differences in refractive index between
Refraction components
Accounts for 35% of total refractive power of Little cellular organelles
eye (15D out of total of 58D) Little extracellular space (tight ball and socket
Light transmission junctions)
2. Maintenance of transparency
Regular arrangement of lens bers
Q What is the Embryology of the Lens?
Embryology
1. Formation of lens vesicle Cells in posterior portion of lens vesicle
4 mm stage (4 weeks) elongate to ll vesicle
Optic vesicle induces lens placode from Secondary lens bers
Cells in anterior portion of vesicle divide
ectoderm
actively and elongate
Lens placode invaginates and becomes lens pit
Tertiary lens bers
Optic vesicle also invaginates and becomes the
Cells in equatorial zone of lens epithelium
optic cup divide and differentiate into long lens bers
Lens pit separates from ectoderm to become Lens zonules
lens vesicle Develop from neuroepithelium running from
2. Formation of lens bers and zonules inner surface of ciliary body to fuse with lens
Primary lens bers capsule
Q How is Glucose Metabolized in the Lens?
Carbohydrate and Energy Metabolism

1. Energy production entirely dependent on glucose 1 mole of glucose gives 38 moles of ATP
metabolism Only 3% of lens glucose metabolized by this
Glucose enters lens by simple diffusion and pathway
facilitated diffusion But generates up to 20% of total ATP needs
Glucose is rapidly metabolized via glycolysis so of lens

that level of free glucose in lens < 1/10 glucose Hexosemonophosphate shunt
Accounts for 5% of glucose metabolism by
in aqueous
lens
2. 4 pathways Important source of NADPH required for
Anaerobic glycolysis other metabolic pathways e.g. sorbitol
Accounts for 85% of glucose metabolism by pathway and glutathione reductase
lens Sorbitol pathway
Provides > 70% of energy for lens Glucose sorbitol via aldose reductase
1 mole of glucose gives 2 moles of ATP fructose via polyol dehydrogenase
Lactate generated undergoes 2 pathways of Accounts for 5% of glucose metabolism by
metabolism: lens
Further metabolism via Krebs cycle When sorbitol accumulates within cells of
Diffusion from lens into aqueous lens, it sets up an osmotic gradient that
Aerobic metabolism (Krebs cycle) induces inux of water and lens swelling and
Limited to epithelium ultimately loss of lens transparency
Q What is the Biochemical Structure of Lens Proteins?
There are 2 types of lens proteins.
4 The Ophthalmology Examinations Review
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Biochemical Structure of Lens Proteins

1. Water soluble lens crystallins Gamma crystallin
90% of total lens protein Smallest crystallin
Alpha crystallin Least abundant
Largest crystallin 2. Water insoluble proteins includes:
Accounts for 35% of total lens protein Membrane proteins urea insoluble
Beta crystallin Cytoskeletal proteins and crystallin
Most abundant crystalline; accounts for 55%
aggregates urea soluble
of total lens protein
Most heterogenous group; 4 distinct
subgroups
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TOPIC 2
CATARACTS
Q Opening Question: What are the Causes of Cataracts?

By far the most common cause of cataract is aging.
Other causes can be classied into 2 types: congenital and acquired.
Etiology of Cataracts
1. Congenital 2. Acquired
Genetic and metabolic diseases Age-related cataract
Downs syndrome, galactosemia, Lowes Traumatic cataract
syndrome Metabolic diseases
Intrauterine infection DM
Rubella Toxic
Ocular anomalies Steroid use, chlorpromazine
Aniridia Secondary to ocular disease
Hereditary cataract Uveitis
Angle closure glaucoma (glaukomecken)
Exam tips:
In general, etiology questions like What are the causes of? can be
answered in a common opening statement: The causes can be classied into
congenital or acquired. Congenital causes include.
In other situations, it may be best to answer directly the most common cause
rst (which gives the impression that youre not memorizing from the book!).
Do not list the rare conditions. For example, under metabolic diseases, say
diabetes, and avoid hyperparathyroidism.
Q What is the Pathophysiology of Age-related Cataracts?
Pathophysiology of Age-related Cataracts

1. General risk factors 2. Cortical cataract
Age Usually results from derangement of electrolyte
Smoking and water balance
Ultraviolet light exposure Increased levels of sodium, chloride and
Medications and other environmental exposures calcium
(controversial) Decreased levels of potassium
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Associated with marked increase in lens Other lens metabolism changes

membrane permeability Increase in proteolysis
Associated with liquefaction of lens material Decrease in ATP production
and osmotic phenomena (lens intumescence) Decrease in glutathione levels
Inability to withstand oxidative stress
3. Nuclear cataract
Associated with protein modication and
increased coloration (urochrome pigment)
Q What is the Pathophysiology of Diabetic Cataracts?
There are two pathogenic mechanisms in diabetic cataracts.

Pathophysiology of Diabetic Cataracts
1. Osmotic effect
into cells swelling and rupture of cells
Glucose sorbitol via aldose reductase (rapid)
results in opacication and cataract formation
fructose via polyol dehydrogenase (slow)
Sorbitol cannot diffuse out of intracellular 2. Direct damage
Glucose may directly interact with lens proteins
compartment accumulates in lens creates
via glycosylation, leading to protein aggregation
an osmotic gradient and movement of water
and cataract formation
Q Tell me about Galactosemia.
Galactosemia is an inborn error in metabolism. The inheritance is AR and there are 2 types.
Galactosemia
1. Galactosemia (type II or classic galactosemia) 2. Galactokinase deciency (type I)
Pathophysiology Pathophysiology
Deciency of galactose-1-phosphate uridyl Deciency of galactokinase
transferase (GPUT) Galactose dulcitol/galactitol via aldose
Galactose dulcitol/galactitol via aldose reductase (no further metabolism)
reductase (no further metabolism) Accumulation of dulcitol results in similar
Accumulation of dulcitol results in osmotic pathway as in Galactosemia type II
disturbance in lens, leading to cataract Clinical features
formation Lamellar cataract (early cataract formation
Clinical features that sinks into lens substance with subsequent
Central oil droplet cataract growth following institution of dietary
Nonglucose reducing substance present in control)
urine Generally healthy
Generally sick (failure to thrive,
hepatosplenomegaly, CNS disease,
renal disease)
Exam tips:
Note that aldose reductase is important in the pathogenesis of both diabetic
and galactosemic cataracts.
Q What are the Ocular Signs in Downs Syndrome?
The ocular features of Downs syndrome can be divided into anterior segment and posterior segment
signs.
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Downs Syndrome
1. Inheritance Mongoloid facies
Nondisjunction (95%) Congenital heart defects
47 chromosomes (3 chromosome 21)
3. Ocular features
Nonhereditary
Anterior segment
Risk to siblings 1%
Lid (blepharitis, epicanthal fold, mongoloid
Translocation (4%)
slant)
46 chromosomes (segment of chromosome
Nasolacrimal duct obstruction
14 translocates to chromosome 21)
Hereditary Cornea (keratoconus)
Risk to siblings 10% (with high rates of Iris (brusheld spots, iris atrophy)
spontaneous abortion) Cataract
Mosaic (1%) Posterior segment
47 chromosomes in some cells, 46 in others Increased retinal vessels across optic disc
Nonhereditary Others
2. Systemic features High myopia
Mental retardation Strabismus, nystagmus and amblyopia
Stunted growth Macular hypoplasia
Exam tips:
Questions like What are the ocular signs of? can be answered with a
common opening statement, The ocular signs can be divided into anterior
segment or posterior segment signs. Anterior segment signs include.
You may consider either answering directly the commonest eye sign rst,
The commonest ocular feature is.
Or answering the most important eye sign rst: The most important eye sign
is.
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TOPIC 3
CONGENITAL CATARACTS
Q Opening Question No. 1: What are the Causes of Congenital Cataracts?

Congenital cataracts can be classied into 2 types: primary and secondary.
Secondary causes include.
Classication of Congenital Cataract
1. Primary Maternal infections (toxoplasmosis)
Idiopathic (50% of all congenital cataracts) Ocular developmental disorders

Hereditary (30%, usually AD) Persistent hyperplastic primary vitreous
Anterior segment dysgenesis, aniridia
2. Secondary
Congenital ectropian uvea, nanophthalmos
Systemic disorders
Ocular diseases
Chromosomal disorders (Downs syndrome)
Trauma, uveitis, retinoblastoma
Metabolic disorders (galactosemia, Lowes
syndrome)
Exam tips:
Do not list the rare causes of congenital cataract. For example, remember
galactosemia but avoid Alports syndrome (unless you know it well!)
The classication is identical to that of congenital glaucoma (see page 57)
and subluxed lens (see page 35).
Q Opening Question No. 2: How do You Manage Congenital Cataracts?

The management of congenital cataract is difcult.
And involves a multidisciplinary team approach.
The important issues are.
And factors that will inuence the decisions include.
The management consists of a thorough history.
Management of Congenital Cataracts
1. Important issues (see below for detailed 2. Factors that inuence the decisions
discussion) Cataract factors (type of cataract, location of
Indications for cataract extraction cataract, severity of cataract, unilateral or
Timing of surgery bilateral cataract)
Type of surgery Child factors (age of onset, associated systemic
Aphakic correction diseases)
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Exam tips:
This is a difcult question to answer. Provide a precise opening statement to
capture the gist of problem.
The issues are important and must be addressed.
The factors that help in addressing the issues are derived from the history and
examination.
Parent factors (motivation of amblyopia Posterior segment

correction) Vitreous strands (persistent fetal
3. History vasculature, Sticklers syndrome)

Retinal abnormalities
Age of presentation
ROP, retinoblastoma,
Unilateral/bilateral
Pigmentation (rubella, Bardet-Biedl
Family history (AD, AR, etc), consanguinity syndrome, Refsums disease)
Birth history Low birth weight (ROP), trauma Atrophy (Cockaynes syndrome)
at birth (cataract)? White ecks (Alports syndrome)
Maternal infection? Optic nerve abnormalities
Drug exposure? Associated systemic anomalies

Naphthalene, phenothiazines, steroids, Chromosomal (Downs syndrome and
sulphonamides others)
Radiation exposure? Skin rash (atopic dermatitis)
Deafness (Alports syndrome, Rubella,
4. Clinical examination
Refsums disease)
Visual acuity
Hepatic dysfunction (Wilsons disease,
Forced preferential looking charts (hundreds
Zellwegers syndrome)
and thousands), Catford drum, optokinetic
Renal disease (Lowe, Alports, Zellwegers
drum
syndrome)
Kay picture chart, Sheridan Gardiner,
CNS disease (Zellwegers syndrome, Lowe)
Illiterate E
Lens opacity 5. Investigations
Location Serum
Polar, subcortical, cortical, lamellar, total Complete blood count
Type Renal function tests, serum calcium
Spoke-like (Fabrys disease, mannosidosis) (hypoparathyroidism)
Vacuoles (DM, hyperalimentation, ROP) Serology for virus (toxoplasmosis)
Multi-color ecks (hypoparathyroidism, GPUT and galactokinase activity in red blood
myotonic dystrophy) cells (galactosemia)
Oil droplet (galactosemia, Alports Arterial blood gas (Lowes syndrome)
syndrome) Urine
Thin, wafer-shaped (Lowes syndrome) Reducing substance (galactosemia)
Green sunower (Wilsons disease) Amino acid (Lowes syndrome)
Associated ocular anomalies Sediments (Fabrys disease)
Anterior segment Copper (Wilsons disease)
Microophthalmos (rubella)
Blood (Alports syndrome)
Megalocornea, sclerocornea, keratoconus SXR for calcications (toxoplasomosis,
Cloudy cornea (Peters plus syndrome, hypoparathyroidism)
Lowes syndrome, Fabrys disease, Others
glaucoma) Karyotyping (chromosomal disorders)
Uveitis (juvenile rheumatoid arthritis) Cultured broblasts with low mannosidase
Aniridia, mesenchymal dysgenesis, level (mannosidosis)
coloboma Conjunctival biopsy with birefringent cell
Glaucoma (aniridia, Peters plus syndrome, inclusions (Fabrys disease)
Lowes syndrome, rubella, trisomy 18) Audiological evaluation
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Important Issues in Cataract Management Primary posterior capsulotomy

1. Indications for surgery Anterior vitrectomy: At least 1/3 of anterior
Severe cataract vitreous must be removed
May be either corneal/transpupillary or
Frequent assessment of visual function
needed pars plana approach
IOL material: Single piece PMMA lens has
In general, operate when cataract is severe
enough to affect visual function and longest safety record, acrylic foldable
development of the eye lenses increasingly utilized
IOL frequently captured in posterior CCC
Earlier surgery indicated when cataract is
unilateral (more amblyogenic) to improve centration and block vitreous
migration
Common indications cataract is associated
Additional considerations
with:
< 18 months (corneal 2-stab incision
Compromised xation (infants)
lensectomy, 1 for AC maintainer, 1 for
Snellen VA or equivalent VA < 6/24 (older
ocutome/vitrector)
baby)
1824 months (scleral tunnel lensectomy,
Strabismus
with phacotome)
Poor visualization of fundus
Opacity occupying central 3 mm 4. Aphakic correction
Depends on laterality and age
2. Timing of surgery
IOL implantation
Depends on laterality and severity
Indication: unilateral aphakia in children
Bilateral severe
6 months to 1 year or older (not indicated for
< 23 months
children < 6 months)
Operate fellow eye within 1 week of rst
Biometry done under GA prior to operation
operation
How do you choose the IOL power?
Unilateral severe Difcult to estimate because of the
< 4 months (best outcomes when performed
progressive myopic shift with age (axial
< 16 weeks)
length of 16.8 mm at birth, this space
If persistent hyperplastic primary vitreous
becomes 20 mm by 1 year)
present, consider operating earlier
5 different approaches
After 9 years of age, operate for cosmetic
Preferred approach: Undercorrecting eye
results only
by 14D from IOL power that is
Bilateral or unilateral mild calculated for emmetropia based on age
May consider waiting until child is older
of child (aim for initial hypermetropia)
3. Type of surgery IOL based on emmetropia
What are the problems associated with IOL that matches refractive error of
congenital cataract surgery? fellow eye

Intraoperative problems IOL based on axial length alone
Risk of GA (prematurity, systemic diseases) IOL of 2122D in all normal sized eyes
Small eye in children more than 12 months old

Hard to dilate pupil Aphakic glasses
Low scleral rigidity Indication: Bilateral aphakia in older child
Solid vitreous Contact lens
Elastic anterior capsule Indication:
Postoperative problems bilateral or unilateral aphakia in infants
Higher incidence of posterior capsule frequently needed for correction of
opacication anisometropia after IOL implantation in

Increased inammation very young children/infants
IOL decentration Family support/compliance essential
Difculty in refraction Extended wear soft lens
Refractive correction and amblyopia Keratometry under GA
treatment Lens diameter of 13.5
Standard technique is usually combination Overcorrect by 2.53D (near vision more
of: important to prevent amblyopia)

Lens aspiration/lensectomy Infantile Aphakia Treatment Study (in progress)
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Background: Aim: To compare the use of contact lenses

IOL use for correction of infantile apahakia and IOL implantation in the correction of
still controversial due to problems with unilateral aphakia
IOL power selection and safety concerns Study groups: Infants up to 7 months of age
Two-thirds of aphakic infants treated with with unilateral cataracts randomized to
contact lenses remain legally blind in the cataract surgery with IOL vs contact lens
aphakic eye correction
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TOPIC 4
CATARACT SURGERY
Q Opening Question: What are the Types of Cataract Surgery?

There are 3 basic types of cataract surgery.
Cataract Surgery
1. Intracapsular cataract extraction (ICCE)
2. Extracapsular cataract extraction (ECCE)
3. Phacoemulsication
Exam tips:
In general, give short simple answers to straightforward questions.
Q What are the Advantages of ECCE over ICCE?
Advantages of ECCE over ICCE

1. Smaller wound size Lower risk of cystoid macular edema
Faster healing time Lower risk of retinal detachment
Fewer wound problems (wound leak and iris Lower risk of glaucoma
prolapse) 3. Intact posterior capsule
Less astigmatism PC IOL implantation possible
Less risk of iris incarceration Eliminate complications associated with
2. No vitreous in AC AC IOL
Lower risk of bullous keratopathy
Q What are the Advantages (and Disadvantages) of Phacoemulsication over ECCE?
Phacoemulsication vs ECCE
1. Advantages Less risk of expulsive hemorrhage
Smaller wound size Operation can be performed under topical
Faster healing time anesthesia
Fewer wound problems (wound leak and iris Conjunctival sparing (important in patients
prolapse) with glaucoma)
Less astigmatism
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2. Disadvantages
Machine dependent Higher complication rate during learning
Longer learning curve curve
Q How do you Perform an Extracapsular Cataract Extraction?
I would perform an extracapsular cataract extraction with IOL implantation as follows....

ECCE with IOL
1. Preparation Express nucleus with alternating superior and
Retrobulbar or peribulbar anesthesia inferior pressure
Superior rectus suture with 4/0 silk 6. Soft lens aspiration
2. Conjunctival peritomy Temporarily close the wound and reform the AC
3. Partial thickness limbal incision with adequate 10/0 nylon sutures
2-plane technique (vertical and horizontal) Aspirate soft lens with infusion-aspiration
Vertical component made with Beaver blade to cannula
2/3 of scleral thickness, from 102 oclock 7. IOL implantation
4. Anterior capsulotomy Reform AC with viscoelastics
Enter AC with 27 G needle Insert IOL into PC capsular bag
Fill AC with viscoelastic 8. Wound closure
Perform anterior capsutomy using tin can 10/0 nylon sutures
technique Subconjunctival steroid/antibiotic injection
5. Nucleus expression
Complete horizontal component of the limbal
incision with scissors
Exam tips:
When asked about a certain surgical technique, describe what you are
familiar with and make your own notes.
Be prepared to answer further questions related to the procedure you choose.
Be concise but accurate with the steps, as if you had done the procedure a
hundred times. Say, I will make a 2-plane limbal incision from 10 to 2 oclock
with a beaver blade rather than I will make an incision at the limbus.
Avoid abbreviations. Say extracapsular cataract extraction instead of
ECCE.
Q What are Some Potential Problems with Anterior Capsulotomy and Nucleus Expression During ECCE?
Anterior Capsulotomy and Nucleus Expression

1. Problems with anterior capsulotomy 2. Problems with nucleus expression
Zonulolysis Wound too small (PCR)
Endothelium damage Incomplete capsulotomy (PCR)
Miosis Difcult to express nucleus in soft eye
Loss of aqueous (AC shallowing) Sphincter rupture with small pupil
Tumbling of nucleus (endothelium damage)
Q How do you Perform an Intracapsular Cataract Extraction?
Currently, the only common indication for planned ICCE is a subluxed lens.
I would perform an intracapsular cataract extraction with IOL implantation as follows
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ICCE with AC IOL

1. Preparation Apply cryoprobe between iris and cornea onto
Retrobulbar or peribulbar anesthesia lens
Superior rectus suture with 4/0 silk Alternatively, use vectis and forceps to remove
2. Conjunctival peritomy lens
3. Full thickness limbal incision with Beaver blade 6. AC IOL implantation
Constrict pupil
and complete incision with scissors, from 93
Reform AC with viscoelastics
oclock (larger than ECCE) Insert AC IOL with help of lens glide
4. Peripheral iridectomy with Vanna scissors 7. Wound closure
5. Lens removal 10/0 nylon sutures
Dry lens with Weck sponge Subconjunctival steroid/antibiotic injection
Q How do You Perform Phacoemulsication?
I would perform phacoemulsication as follows.

Phacoemulsication
1. Preparation Low vacuum: 30 mm Hg
Retrobulbar, peribulbar or topical anesthesia Low ow: 2030 cc/min
2. Clear corneal tunnel Nucleus disassembly:
Burst/pulse/hyperpulse power: 2530%
Stab incision with 2.5 mm keratome for main
High vacuum: 250300 mm Hg
wound at 12 oclock
High ow: 3040 cc/min
Stab incision with Beaver blade for side port at
Dealing with chamber instability:
3 or 9 oclock
Proper wound construction to prevent leaks
3. Capsulorrhexis Lower vacuum and ow/aspiration
Fill AC with viscoelastic Raise bottle height
Perform continuous circular capsulorrhexis Check inow for kinks/interruptions in tubing
with 27 G bent needle or connections
Perform hydrodissection (cleavage between Viscoelastic tamponade
cortex and capsule) and hydrodelineation Start with central sculpting
(cleavage between nucleus and epinucleus) Remove rest of nucleus with various techniques
Divide and conquer
4. Phacoemulsication of nucleus
Chop techniques
The aim of phacodynamic control is to remove
lens material in a stable chamber, thereby 5. Soft lens aspiration
minimizing the risk of complications related to Aspirate soft lens with automated infusion-
chamber instability aspiration cannula
Main parameters: 6. IOL implantation
Flow/aspiration Reform AC with viscoelastics
Vacuum Enlarge main wound to 3 mm (depending on
Power lens insertion technique)
Bottle height Insert foldable IOL into capsular bag
Peristaltic systems: Flow and vacuum 7. Wound closure
dissociated and can be controlled individually One 10/0 nylon suture if necessary
Venturi systems: Only vacuum can be Subconjunctival steroid/antibiotic injection
controlled, and serves to determine ow
Common settings:
Sculpting:
Continuous power, 30%
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TOPIC 5
ANESTHESIA AND
VISCOELASTICS
Q Opening Question: How do You Administer Regional Anesthesia? What are the Possible
Complications?
I prefer to use the peribulbar anesthesia technique.

Retrobulbar and Peribulbar Anesthesia
1. Amount Apply intermittent pressure to compress eye
5 ml of lignocaine 1% + Lateral canthotomy if pressure is too high
Wydase (1 ml diluted into 20 ml of Globe penetration
lignocaine) + Abort surgery
Adrenaline (2/3 drops of 1:1000) Fundal examination
Usually no need to explore scleral wound
2. Advantages of peribulbar over retrobulbar
(self-sealing)
anesthesia B-scan if vitreous hemorrhage obscures
Lower risk of optic nerve damage view
Lower risk of systemic neurological effects of Refer for retinal consult
anesthesia Cryotherapy or laser photocoagulation for
Lower risk of globe perforation (controversial) the retinal break

No need for facial block Optic nerve damage
Direct damage or ischemia
3. Disadvantages of peribulbar over retrobulbar
Extraocular muscle damage (post-operative
anesthesia motility problems)
Need more anesthetic Neurological effects of anesthetic agents
Less akinesia Ptosis (from compression/ocular massage)
Longer onset (30 min to attain maximum
5. Alternatives to regional blocks
effect)
Topical anesthesia
Higher intraorbital pressure Advantages:
Greater degree of chemosis No complications of injection
May need additional superior oblique block No increase in orbital volume
(therefore ending up with 2 injections around Avoids periorbital bruising
the globe) Disadvantages:

4. Complications and management No akinesia
Less anesthetic effect (intraocular

Retrobulbar hemorrhage
Most common complication structures till sensitive; e.g. iris)
Proceed with surgery if hemorrhage is Sub-Tenons injection
small Intracameral anesthesia
Abort surgery if hemorrhage is large 1% preservative-free lignocaine
B1037_Ch-01.indd 19 12/24/2010 2:34:11 PM

Exam tips:
Be precise about the concentration of drugs and amount you give. Say In my
practice, Ill use 5 ml of 1% lignocaine. rather than Ill use ligno-
caine.
Q How are the Irrigating Solutions Used During Cataract Surgery?
Irrigating Solutions
1. Balance salt solution (BSS) Epinephrine/antibiotics can be added as
Physiological balanced salt solution well
Sterile 2. BSS Plus
Isotonic Enriched with bicarbonate, dextrose,
Preservative-free glutathione
Includes: Sodium chloride, potassium chloride, Less endothelial damage and better lens
calcium chloride, magnesium chloride, acetate, nutrition (not proven)
citrate
Q Tell me about Viscoelastics.
Viscoelastics are substances with dual properties of a viscous liquid

and elastic solid.
They are used extensively in intraocular surgeries.
They display various physical properties.
The ideal viscoelastic material has the following characteristics.
Viscoelastics
1. Physical properties Refers to resistance to ow maintain
Related to chain length and molecular anterior chamber and intraocular volume
interaction of substances Pseudoplasticity
Refers to ability to transform under
4 characteristics:
Viscoelasticity pressure from gel to liquid ease of
Refers to tendency to retain original shape
insertion with increase in pressure
and size shock absorption and Surface tension
Refers to coating ability endothelial
endothelial protection
Viscosity protection and surface coating
Exam tips:
Remember the properties of ideal viscoelastic and compare the advantages
and disadvantages of cohesive vs dispersive viscoelastics in terms of these
factors.
2. Ideal viscoelastic
Optically clear, nontoxic, noninammatory Surface coating
Chamber maintenance Ease of insertion
Shock absorption Ease of removal
Endothelial protection No IOP rise
B1037_Ch-01.indd 20 12/24/2010 2:34:11 PM

3. Example
Cohesive Dispersive
Examples Healon Viscoat
Sodium hyaluronate 1% Hyaluronate 3% + Chondroitin sulfate 4%
Derived from rooster combs Derived from shark cartilage
Generally better shock Generally better coating and endothelial
absorption, easier to insert protection, but poorer shock absorption,
and remove, better view poorer view, difcult to insert and remove
Properties
1. Optically clear +++ +
2. Chamber maintenance +++ +++
3. Shock absorption +++ +
4. Endothelial protection + +++
5. Surface coating + +++
6. Ease of insertion +++ +
7. Ease of removal +++ +
8. IOP rise ++ ++
4. Indications aspiration of soft lens, tamponade vitreous

Cataract surgery commonly used during: after PCR
Anterior capsulotomy Penetrating keratoplasty
Prior to nuclear expression (for ECCE) Glaucoma surgery
IOL insertion Corneal laceration
Other scenarios in which it is used pupil Retinal detachment surgery (retinal
dilation, free soft lens matter during incarceration during SRF drainage)
B1037_Ch-01.indd 21 12/24/2010 2:34:11 PM

TOPIC 6
INTRAOCULAR LENS
Q Opening Question: What are the Types of Intraocular Lens?

An intraocular lens (IOL) is a clear optical device.
Implanted into the eye to replace the crystalline lens.
Intraocular lens can be classied as follows.
Intraocular Lens
1. Rigid posterior chamber intraocular lens (PC IOL) 3. Foldable PC IOL
Divided into: Optic and haptic components For small incision cataract surgery
Either one or three pieces Materials:
Overall length (1214 mm) Silicone
Optic Acylic
Material (PMMA Polymethylmethacrylate) Hydrophobic
Diameter (4.57 mm) Hydrophilic
Design (piano-convex, biconvex, meniscus) Optic color/tints:

Clear
Haptic
Yellow (blue-blocker)
Material (PMMA, prolene easily deformed,
Violet
nylon gradually hydrolyzed)
Conguration (closed loop, J or C loop) Optic design
Convexity (Cornea has positive spherical
Angulation of haptic to optic (at, 10
posterior bowing) aberration (SA), normal lens has negative SA)
Biconvex std IOL Positive (SA)
Additional features
Aspheric:
Positioning hole
Tecnis: Prolate anterior surface =
Problem of postoperative diplopia
Laser ridges
negative SA
Acrysof IQ: Negative SA on posterior
Prevent PCO and decrease damage with
Nd:YAG laser capsulotomy surface

Sorport AO: Pos aspheric SA free (even
Problem of postoperative diplopia
Multifocal
power from centre to periphery not
Central portion for near vision
affected by centration/tilt)
Edge design:
Mechanisms 3 types
Square
Refractive, diffractive and aspherical
Round
Problems of postoperative diplopia,
OptiEdge (Tecnis)
haloes, glare and loss of VA
Toricity
Heparin-coated
Multifocality
Surface more hydrophilic
Refractive
Decrease inammation, pigment
Diffractive
dispersion and synechiae formation (not
proven) Haptic design
Material:
2. Anterior chamber intraocular lens (AC IOL)
PMMA (dyed)
(see below) Prolene MA60
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Same as optic 4. Injectable IOL

PVDF (Tecnis) 5. Scleral-xated IOL
Shape:
6. Phakic IOL
Plate
Loop
Four-point
Exam tips:
Remember that you are implanting this foreign object into a patients eye.
You are therefore expected to know quite a bit about it!
Q Tell me about AC IOL.
AC IOL is divided into 3 different types.

It is usually indicated for.
AC IOL
1. Types During planned ICCE
Pupil plane suture or clip 3. Complications with AC IOL
Iris supported suture or clip Endothelial fallout (bullous keratopathy)
Angle supported divided into: CME (most common cause of poor VA after AC
Rigid angle supported IOL implant)
Flexible angle supported further divided
Chronic pain and ache (older AC IOL with rigid
into:
haptics)
Closed loop
Glaucoma (uveitis-glaucoma-hyphema (UGH)
Open loop S/Z shaped or 2/3/4 legs
syndrome)
2. Indications (3 scenarios)
4. Calculations of AC IOL power (see below)
Secondary IOL implant
During ECCE/phaco after PC rupture/zonulolysis
Clinical approach to anterior chamber IOL

This patient is pseudophakic with an AC IOL.
There is a peripheral surgical iridectomy seen at 2 oclock.
Look for
Bullous keratopathy
AC activity (uveitis)
Vitreous in AC/vitreoendothelial touch
Outline of PCR/shape of pupil (round/peaked)
Ill like to
Check IOP
Check fundus (CME, RD)
B1037_Ch-01.indd 24 12/24/2010 2:34:11 PM

Q What are the Indications and Complications with PC Scleral-Fixated IOL?
1. Indications Persistent iritis (IOL instability)

Similar to AC IOL indications plus CME
Relative contraindication to AC IOL implant Pupil distortion
Younger patient Hyphema
Glaucoma Endophthalmitis
Peripheral anterior synechiae Suture erosion over time
Corneal/endothelial problems
2. Complications of PC scleral-xated IOL
IOL tilt/dislocation
Q Under What Situation During Cataract Surgery would You Consider NOT Implanting an IOL?
In certain scenarios, IOL is not routinely used.

Patients With PCR with signicant vitreous loss
Congenital cataract (most common Severe glaucoma
contraindication) Corneal endothelial dystrophy
Aphakia in fellow eye
Recurrent uveitis
Posterior segment problems (proliferative DR,
RD) and IOL will interfere with treatment of
such problems
Q What are the Different IOL Materials?
What are the different IOL materials?

There are many different types of IOL materials.
These include.
IOL Materials Similar optical quality as PMMA

1. Ideal material Cast/injection molded (no polishing required)

Autoclavable
High optical quality
Minimal trauma to tissues
High refractive index (RI)
But (compared to PMMA)
Lightweight
Low RI (thicker)
Durable Low tensile strength (tears easily)
Nontoxic/inert (no inammation, antigenicity, Slippery (needs dry instruments)
carcinogenicity) Elastic (needs controlled release in anterior
Ease of manufacture and sterilization chamber)
2. PMMA Capsular phimosis
All the above properties except ease of Discoloration

Contraindicated in patients who need silicon
sterilization (altered by heat, steam, gamma
oil later (e.g. DM)
radiation)
5. Acrylic
3. Glass
Potential advantages
Potential advantages
More control over folding and release of IOL
Good optical quality
Sticky (less PCO, less capsular phimosis)
Autoclavable
Less inammation
But
More resistant to Nd:YAG capsulotomy
Heavy
But (compared to silicone)
Crack after Nd:YAG capsulotomy
Less compliant (longer time to compress and
4. Silicone results in larger wound)
Potential advantages Low tensile strength (tears easily)
Foldable, inserted into small wound Higher cost
B1037_Ch-01.indd 25 12/24/2010 2:34:12 PM

Q How do You Calculate IOL Power?
There are 3 types of formulas available.

IOL Power Calculation
1. Theoretical formulas 4. Choice of formula
Based on optics/vergence equations Main factor is axial length of eye
E.g. Holliday, Binkhorst Royal College of Ophthalmologists
2. Empirical formulas recommendations:
Based on regression analysis on refraction results AL < 22.0 mm Hoffer/SRK-T
AL 22.024.5 mm SRK-T/Holladay, Haigis
from patients with cataract surgery and IOL
AL > 24.6mm: SRK-T
E.g. SRK (Sander-Retzlaif-Kraff)
3. Combination formulas
Theoretical formula with regression analysis
added to optimize the equations
E.g. SRK-T
Q Tell me about the SRK Formula.
The SRK is an IOL power calculation formula and stands for.

SRK Formula
1. Power = (A constant) 2.5 (axial length) 0.9 3. Choosing power of AC IOL when PCR occurs
Suppose a 20D PC IOL was chosen with an A
(keratometry)
constant of 118
2. Factors which affect the A constant The AC IOL has A constant of 114
Position of IOL in eye (closer the IOL to retina, Then the desired power of AC IOL is
higher the A constant, therefore AC IOL has 20D (118 114) = 16D
lower A constant!)
Shape of IOL (convex, biconvex etc)
Haptic angulation
Q How do You Choose the Final IOL Power?
Selection is based on the patients refractive status in the eye due for cataract surgery, the patients visual
requirements and the state of the fellow eye.
IOL Power Selection
1. Emmetropic eye (0.5 to +0.5D) 5. High myopes
Active patient aim for emmetropia Many surgical issues involved must be
Sedentary, elderly aim for slight myopia explained (see page 27)
2. Slight hyperopia (+0.5 to 3.0D) Fellow eye needs cataract operation aim for
Aim for emmetropia slight myopia (then aim for emmetropia in
3. High hyperopia (> +3.0D) fellow eye)
Fellow eye needs cataract operation aim for Fellow eye is as myopic but does not need
emmetropia cataract operation aim for myopia with
Fellow eye does not need operation aim for 23D difference compared with fellow eye
slight hyperopia (or aim for emmetropia and use contact lens
for fellow eye)
4. Slight myopia (1.0 to 3.0D)
Fellow eye is emmetropic consider the
Active patient aim for emmetropia
possibility that operated eye may have
Sedentary, elderly aim for slight myopia of
amblyopia!
2.0 to 2.5D
B1037_Ch-01.indd 26 12/24/2010 2:34:12 PM

Q What are the Principles of Ultrasound Biometry?
Ultrasound Biometry
1. Principles Accuracy of axial length
Ultrasound = acoustic (sound) waves at 0.1 mm = error of 0.25D in an emmetropic
frequency > 20 kHz (20,000 cycles/sec) eye, more in a short eye and less in a longer
Produced from an electric pulse in piezoelectric eye (see SRK formula)
crystal (keyword) Standard dimensions
Multiple measurements between two eyes
Echoes
A-scan (timeamplitude)
should be within 0.2 mm and difference in
B-scan (brightness modulated)
length between two eyes should be within
0.3 mm
Frequency
Mean values
Increase in frequency is associated with a
Axial length = 23.5 mm
decrease in penetration, but an increase
Corneal thickness = 0.55 mm
resolution
AC depth = 3.24 mm
Ophthalmic use (812 MHz) vs obstetric use
Lens thickness = 4.63 mm
(1 MHz)
Sound velocity 3. Measurement errors and other issues
Faster through denser medium Articially too short
Velocities Corneal compression
Cornea/lens (1,641 m/sec) Sound velocity too slow, improper gate
Aqueous/vitreous (1,532 m/sec) settings or gain too high
PMMA (2,718 m/sec) Misalignment of sound beam
Silicone (980 m/sec) Articially too long
Acoustic impedance Fluid between cornea and probe
Impedance = density sound velocity Sound velocity too fast, improper gate
Acoustic interface settings or gain too low
Formed when sound travels between media Misalignment of beam
of differing acoustic impedances Staphyloma
Silicone oil
2. Measurements
Pseudophakia
A-scan ultrasound determines time required for
PMMA IOL eye measure shorter
sound to travel from cornea to retina and then Silicone IOL eye measure longer
back to probe Conversion factors (measure using aphakic
(Distance = velocity time/2) sound velocity and add the above factors)
Gain PMMA (+0.4)
Increase in gain is associated with an Silicone (0.8)
increase in tissue penetration and sensitivity Acrylic (+0.2)
but decrease in resolution
Q What is Partial Coherence Biometry?
Partial coherence biometry (e.g. IOL Master) is a new biometric tool that utilizes infrared light
(wavelength 780 nm) instead of sound waves for biometric measurements.
Advantages Over Ultrasound Biometry: Measures along xation beam: More accurate
Non-contact for high myopia (almost mandatory in presence
Faster (< 1 sec) of staphyloma!)
Easier to perform No correction needed for silicone oil lled eyes
B1037_Ch-01.indd 27 12/24/2010 2:34:12 PM

Q What is the Role of Ultrasound in Ophthalmology?
Ultrasound is used for diagnosis and treatment.

Diagnostic
1. Anterior segment Post-Keratorefractive Surgery Biometry
Pachymetry
1. Main challenges in post-refractive surgery biometry
Biometry
Keratometers only measure K in central zone
Biomicroscopy (AC angles)
which may not encompass entire treatment/
Lens thickness
ablation zone
2. Post segment Standard Keratometry index (1.3375) assumes
Vitreous opacity (vitreous hemorrhage, posterior constant relationship between anterior and
vitreous detachment) posterior corneal surfaces not true after
Retina (RD, tumors) corneal ablation
Choroid (choroidal detachment, tumors) 2. Approaches to biometry:
IOFB Methods which do not rely on current data
3. Orbit (historical data methods)
Tumor, cyst, mucocoele, FB (superceded by CT Based on records of pre-refractive surgery
scan) K values, with adjustment based on amount
4. Doppler of refractive correction
Various formulae
Carotid duplex
Methods based on current data
Blood ow to optic nerve head
Contact lens overrefraction: Effective
Orbital color doppler imaging
K = contact lens base curve difference
Ophthalmic artery duplex between refraction with and without contact
lens
Therapeutic Pentacam based formulae (direct measurement
1. Phacoemulsication of posterior corneal curvature)
2. Ciliary body destruction for end stage glaucoma
B1037_Ch-01.indd 28 12/24/2010 2:34:12 PM

TOPIC 7
CATARACT SURGERY
IN SPECIAL SITUATIONS
Q Opening Question: How do You Manage this Patient with Glaucoma and Cataract?
In this patient, there are 2 clinical problems that have to be managed simultaneously.
This would depend on the severity of each condition.
Factors to consider would include.
Management of Glaucoma and Cataract
Severity of glaucoma Severity of cataract Possible options
+++ + Trabeculectomy rst, cataract operation later

Alternatively, discuss with patient about
advantages of combined cataract operation and
trabeculectomy (triple procedure) (see below)
+ +++ Cataract operation rst, manage glaucoma
conservatively
Alternatively, discuss with patient about
advantages of triple procedure (see below)
+++ +++ Consider triple procedure
Factors that Determine the Management of

Glaucoma and Cataract 3. Patient factors
1. Severity and progression of glaucoma Age
IOP level (most important factor) Race (blacks higher rate of glaucoma
Optic nerve head changes progression)
Visual eld changes Family history of blindness from glaucoma
Ocular risk factors (CRVO, Fuchs endothelial Fellow eye blinded from glaucoma
dystrophy, retinitis pigmentosa) Concomitant risk factors for glaucoma
2. Severity and progression of cataract (DM, HPT, myopia, other vascular diseases)
VA and visual requirements Compliance to follow-up and medication use
B1037_Ch-01.indd 29 12/24/2010 2:34:12 PM

Exam tips:
Remember there are no RIGHT or WRONG answers. You must be able to
come up with a position and defend it.
Be as conservative as possible. Therefore give extremes of each scenario rst
(least controversial), then go on to the more difcult and controversial areas.
Opening statement is similar in all situations in which there are 2 problems,
There are 2 clinical problems that must be managed simultaneously. Factors
to consider in these patients include.
See factors that determine glaucoma management (page 63).
Q What are the Indications for a Combined Cataract Extraction and Trabeculectomy?
In general, this procedure is indicated when there is SIMULTANEOUS need for trabeculectomy and
cataract operation.
Combined Cataract Extraction and 4. Alternative ways to perform the combined
Trabeculectomy operation
1. Indications Corneal section ECCE plus trabeculectomy
General principle: Indications for Advantages
trabeculectomy (when IOP is raised to a level More control
that there is evidence of progressive VF or ON Less conjunctival manipulation
Smaller wound (lower risk of leakage and

changes despite maximal medical treatment)
plus indication for cataract surgery (visual shallow AC)
Disadvantages
impairment)
Longer
2. Advantages Higher corneal astigmatism
One operation Limbal section ECCE plus trabeculectomy
Faster visual rehabilitation Advantages
Patient may be able to be taken off all glaucoma Faster
medications Less astigmatism
Prevents post-op IOP spikes Disadvantages

HVF monitoring easier with clear media Larger wound
More conjunctival manipulation

No subsequent cataract operation needed
Increased risk of at AC
(lower risk of bleb failure)
Phacoemulsication plus trabeculectomy
3. Disadvantages
Advantages
Strong evidence that IOP control with More control of AC
trabeculectomy alone is better than combined Less conjunctival manipulation (main
surgery reason)
More manipulation during the combined Smallest wound of the 3 techniques
operation (higher risk of bleb failure) Less astigmatism
Vitreous loss during cataract surgery (higher risk Faster
of bleb failure) Disadvantage:

More difcult operation for the
Larger wounds created (higher risk of wound
leakage and shallow AC) inexperienced surgeon
Exam tips:
Essentially identical to the indications for trabeculectomy (page 80).
B1037_Ch-01.indd 30 12/24/2010 2:34:12 PM

Notes
What are the common scenarios for trabeculectomy?
Uncontrolled POAG with maximal medical treatment
Failure of medical treatment (IOP not controlled with progressive VF
or ON damage)
Side effects of medical treatment
Noncompliance with medical treatment
Additional considerations
Young patient with good quality of vision
One-eyed patient (other eye blind from glaucoma)
Family history of blindness from glaucoma
Glaucoma risk factors (HPT, DM)
Uncontrolled PACG after laser PI and medical treatment

Secondary OAG or ACG
Q What are the Potential Problems in Removing a Cataract in a Patient with High Myopia?
There are several potential problems, which can be divided into.

High Myopia and Cataract Surgery
1. Preoperative stage 2. Intraoperative stage
Need to assess visual potential (amblyopia, Risk of perforation with retrobulbar anesthesia
myopic macular degeneration) (consider topical anesthesia or GA)
Choose IOL power carefully (counseling for Lower IOP (harder to express nucleus during
anisometropia) ECCE)
Harder to do biometry (need special formulas to Deeper AC (harder to aspirate soft lens material)
adjust for longer axial lengths) Increased risk of PCR (weak zonules)
IOL Master biometry in view of high prevalence 3. Postoperative stage
of staphylomata Risk of RD
Q What are the Potential Problems in Removing a Cataract in a Patient with Uveitis?
Uveitis and Cataract Surgery
1. Preoperative stage Increased inammation (consider heparin
Need to control inammation coated IOL or leave aphakic)
Consider waiting 2 to 3 months until Increased risk of bleeding
inammation settles after an acute episode 3. Postoperative stage
Consider course of preoperative steroids
Higher risk of complications
Assess visual potential (CME, optic disc edema) Corneal edema
Dilate pupil in advance (atropine, Flare up of inammation
subconjunctival mydriacaine) Glaucoma or hypotony
Perform gonioscopy (if synechiae is severe Choroidal effusion
superiorly, consider corneal section) CME
2. Intraoperative stage Consider prophylaxis for infectious etiologies

Problem of small pupil (see below) (e.g. herpetic lesions)
Increased risk of PCR (weak zonules)
B1037_Ch-01.indd 31 12/24/2010 2:34:13 PM

Q How do You Manage a Small Pupil During Cataract Surgery?

Small Pupil During Cataract Surgery
1. Preoperative stage Use viscoelastics to dilate pupil
High risk patients (uveitis, DM, Remove pupillary membrane (previous
pseudoexfoliation syndrome, Marfans inammation)
syndrome, glaucoma on pilocarpine treatment) Stretch pupil gently (with Kuglen hooks)
Prior to operation, prescribe mydriatics (3 days Perform sphincterotomy at 6-, 3-, 9- and 12
of homatropine 2% three times a day or oclock positions
atropine) Perform broad iridectomy at 12 oclock position
2 hours before operation, intensive dilation with Perform basal iridectomy and mid-peripheral
Tropicamide 1% iridotomy (better apposition than broad
Ocufen 0.03% iridectomy)
Phenylephrine 10% Iris hooks
2. Intraoperative stage Pupil expansion devices (e.g. Morcher pupil
Infuse AC with balanced salt solution mixed expansion ring)
with a few drops of 1:1000 Adrenaline
Exam tips:
Common follow-up question of pseudoexfoliation (page 18) and uveitis (see
above).
Give practical answers. Do not say iris hooks rst or you will be asked in
detail how to do it!
Q What are the Problems Operating on a Mature Cataract?

Mature Cataract
1. Need to assess visual potential Consider using air instead of viscoelastics
Pupils (optic nerve function) Use of capsular stains (vision blue/trypan blue):
Light projection (gross retinal integrity), color possibly toxic to endothelium, capsular fragility,
perception teratogenic
Potential acuity meter (macular function) 3. High intra-capsular pressure
B-scan ultrasound (gross retinal anatomy) CCC runs out/splits easily
2. Poor view of capsulotomy/capsulorrhexis edge 4. Floppy capsule due to chronic bulky lens
Consider endocapsular technique Viscoelastic tamponade
Q What are the Issues in Cataract Extraction for Diabetic Patients?

The 2 main issues are.
Diabetes and Cataract
1. Issues Laser PRP if necessary prior to the surgery
Difcult cataract surgery Medical consult
Progression of diabetic retinopathy after operation List for rst case in morning
2. Preoperative stage 3. Intraoperative stage
Assess visual potential Protect corneal epithelium (risk of abrasion and
Consider FFA poor healing)
B1037_Ch-01.indd 32 12/24/2010 2:34:13 PM

Problems with small pupil (see above) Avoid AC IOL

Consider stitching wound Consider heparin-coated IOL
Selection of lOL 4. Postoperative stage
Large optics (7 mm) Control inammation (especially in eyes with PDR)
Use acrylic IOL (avoid silicone lOL) Risk of PDR/CSME
Avoid IOL if PDR (risk of neovascular Risk of glaucoma
glaucoma) Risk of PCO
Notes
Why does diabetic retinopathy progress?
Removal of anti-angiogenic factor in lens
Secretion of angiogenic factors from iris
Increased intraocular inammation
Decreased anti-angiogenic factor from RPE
Migration of angiogenic factors into AC
B1037_Ch-01.indd 33 12/24/2010 2:34:13 PM

TOPIC 8
CATARACT SURGERY
COMPLICATIONS
Q Opening Question: What are the Issues in Cataract Extraction for Diabetic Patients?
The complications can be classied into 3 categories: Preoperative, intraoperative and postoperative
complications.
Complications of Cataract Surgery
1. Intraoperative Undetected intraoperative PCR with vitreous in
Posterior capsule rupture (PCR) and vitreous loss AC
Suprachoroidal hemorrhage Cystoid macular edema (CME)
Dropped nucleus 3. Late postoperative
2. Early postoperative Late endophthalmitis
Endophthalmitis Wound astigmatism
Wound leak Glaucoma
IOP related problems (raised IOP, low IOP and Bullous keratopathy
shallow AC) Posterior capsule opacication
Corneal edema (striate keratopathy) Retinal detachment
Exam tips:
Complications of all eye operations are extremely important, because you are
expected to manage them.
There are a few ways to answer these questions, choose one and be comfort-
able with it.
The most common complication answer, The commonest ocular complica-
tion is.
The most important complication answer, The most important complication
is endophthalmitis.
The clinical classication answer, The complications can be classied into
preoperative, intraoperative and postoperative complications.
The anatomical classication answer, The complications can be divided into
anterior or posterior segment .
Exam tips:
Notice an intentional grouping of early postoperative and late postoperative
complications into similar groups (i.e. endophthalmitis, would problems, IOP
problems, corneal problems, PC problems and retinal problems)!
B1037_Ch-01.indd 35 12/24/2010 2:34:13 PM

Q How do You Manage a Posterior Capsule Rupture (PCR) During Cataract Surgery?
The management depends on the stage of the operation, the size and extent of PCR and whether
vitreous loss has occurred.
The risk factors include.
Management of PCR
1. Management depends on Phaco over Sheets glide (reduce
Stage of operation at which PCR occurs, parameters)
commonly during: Large fragment convert to ECCE/posterior
Nucleus expression assisted techniques

Aspiration of soft lens Remove remaining soft lens matter with
IOL insertion gentle and dry aspiration

Size and extent of PCR Vitreous loss
Presence or absence of vitreous loss Anterior vitrectomy (sponge vitrectomy or
2. Risk factors automated vitrectomy) settings

Consider IOL implantation
Ocular factors
PC IOL (small PCR) convert rupture to
Difcult cataracts (brunescent, morgagnian,
round posterior CCC
pseudoexfoliation, posterior polar cataracts)
Sulcus IOL (moderate to large PCR with
Glaucoma
adequate PC support)
High myopia
Avoid one-piece lenses: Chang and
Increase in vitreous pressure observed after
instability
retrobulbar and peribulbar anesthesia
IOL power has to be adjusted (reduced)
Small pupils
to account for more anterior position
Small CCC
AC IOL (large PCR with inadequate
Patient factors
posterior capsule support)
HPT
Leave aphakic (large PCR with inadequate
Chronic lung disease
posterior capsule support)
Obese patient with short thick neck
Avoid silicone lenses in PCR higher rate
3. Clinical signs of PCR of endophthalmitis
Presence of ring reex in the posterior capsule Consider IOL capture in CCC for stability
Inability to aspirate soft lens matter (vitreous Checklist at the end of operation
stuck to port) Obvious vitreous at pupil borders?
Outline of PCR seen Inject miotic agent round pupil
Peaked pupil observed?

Traction at wound edge with weck
Vitreous seen in AC
sponge peaking of pupil?
Sudden deepening of AC
(Marionette sign)
Fragments disappear form view
Inject air bubble regular round bubble
Pupil-snap sign: PC rupture during observed?
hydrodissection Sweep iris movement in AC
4. General principles of management Postoperative risk of

Intraoperative stage Endophthalmitis
Stop surgery immediately and assess Glaucoma
situation Inammation
Limit size of PCR (inject viscoelastic into AC) Bullous keratopathy
Dealing with remaining lens matter Suprachoroidal hemorrhage
Small fragment CME
Enlarge wound and express with Vectis RD
Q How do You Manage a Suprachoroidal Hemorrhage?
Suprachoroidal hemorrhage is a rare but blinding complication of cataract extraction.
B1037_Ch-01.indd 36 12/24/2010 2:34:13 PM

Suprachoroidal Hemorrhage
1. Risk factors Dark mass behind pupil seen
Ocular factors Extrusion of all intraocular contents
Glaucoma 3. General principles of management
Severe myopia Intraoperative
PCR during surgery Stop surgery
Patient factors Immediate closure with 4/0 silk suture (use
HPT superior rectus stitch)
Chronic lung disease IV mannitol
Obese patient with short thick neck Posterior sclerostomy
2. Clinical signs Controversial and may exacerbate bleeding
Progressive shallowing of AC Postoperative

Increased IOP Risk of glaucoma (need timolol) and
Prolapse of iris inammation (need predforte)
May need to drain blood later on (vitrectomy)
Vitreous extrusion
Loss of red reex
Exam tips:
The risk factors are nearly identical to that for PCR!
Q How do You Manage a Dropped Nucleus During Phacoemulsication?
The management of a dropped nucleus depends on the stage of the operation, the amount of the lens
fragment dropping into the vitreous and whether vitreoretinal surgical help is available.
Dropped Nucleus
1. Why in phacoemulsication, but not in ECCE? 3. General principle of prevention
PCR more difcult to see in phacoemulsication Good sized and shaped capsulorrhexis
High pressure AC system (infusion solutions) Careful hydrodissection
2. Types of dropped nucleus Clear endpoints in nuclear management
Prior to nucleus removal Recognition of occult PCR
Whole nucleus drop 4. Management
Runaway capsulorrhexis or during Stabilize AC with viscoelastics and remove
hydrodissection phacoprobe. Enlarge wound
During nucleus removal Inject viscoelastics under nucleus if possible
Nuclear fragment drop
Retrieve fragments with vectis/forceps/posterior
Phacoemulsication of posterior capsule,
assisted techniques
puncture or aspirate capsule
Controversial: May increase vitreous traction
After nucleus removal and risk of retinal breaks
PCR is associated with vitreous loss but no
Either close wound and remove fragments at
nuclear drop
later date, or proceed with immediate
Management similar to PCR in ECCE
vitrectomy and nucleus removal
Notes
What are the signs of an impending nuclear drop?
Runaway capsulorrhexis
Pupil snap sign (pupil suddenly constricts)
Difculty in rotation of nucleus
Nuclear tilt
Receding nucleus
B1037_Ch-01.indd 37 12/24/2010 2:34:14 PM

Q Tell me about Postoperative Endophthalmitis.

Postoperative endophthalmitis is a rare but blinding complication after cataract surgery.
The management depends on isolation of organism, intensive medical treatment and surgical
intervention if necessary.
Classication and Microbial Spectrum
Classication Types Incidence Microbial spectrum Onset
Endogenous Generalized septicemia Klebsiella and gram

negatives
Localized infections Depending on source
(endocarditis,
pyelonephritis,
osteomyelitis)
Exogenous Postoperative (cataract) 0.1% S. epidermidis (70%) 114 days
S. aureus, Streptococcus
Gram negatives
Propionibacterium
species (chronic)
Postoperative 1% Streptococcus Early to late
(glaucoma) Hemophilus influenze
Post traumatic 510% S. epidermidis 15 days
S. aureus
Bacillus
Gram negatives
Postoperative Endophthalmitis
1. Clinical features Vitreous tap to isolate organism (see below)
Pain Medical treatment
Decreased VA Intravitreal antibiotics
Lid edema and chemosis Intensive fortied topical antibiotics
Corneal haze Systemic antibiotics (controversial)
Steroids (controversial usually better given
AC activity, hypopyon, brin
systemically)
Absent red reex
Surgical treatment
Vitritis
Vitrectomy
2. General principles of management Endophthalmitis vitrectomy study
Prevention (Arch Ophthalmol 1995; 113:1479)
Preoperatively: Only irrigation of conjunctival 420 patients with post cataract surgery
sac with 5% povidone iodine has been endophthalmitis
shown to reduce endophthalmitis risk Randomly assigned to either early
Other measures like antibiotics not proven vitrectomy vs vitreous tap and IV
but commonly employed antibiotics vs topical and intravitreal
Intraoperatively: antibiotics.
ESCRS multi-center study on intra-cameral Results: Immediate vitrectomy only
antibiotic prophylaxis showed that use of benecial in patients with perception of
intra-cameral cefuroxime (1 mg/0.1 ml) was light vision or worse. No benet of IV
associated with 5x reduced risk of antibiotics
endophthalmitis
B1037_Ch-01.indd 38 12/24/2010 2:34:14 PM

Exam tips:
Be careful, postoperative endophthalmitis is not the same as
endophthalmitis (the latter includes endogenous and post-traumatic
endophthalmitis).
The incidence after cataract surgery is 1 in 1,000 (0.1%) but is 10 times
higher in glaucoma surgery (1%) and 100 times higher after trauma (510%).
Q How do You Perform a Vitreous Tap?
I would perform a vitreous tap in the operating room under sterile conditions.
First I would prepare the antibiotics and culture.
Vitreous Tap
1. Perform in OR under sterile conditions Enter pars plana from temporal side of the
2. Prepare antibiotics and culture media before globe, 4 mm behind limbus, directed towards
procedure center of vitreous
If no uid aspirated, reposition or consider
0.2 ml of antibiotic
use of hand-held automated vitrector
Cephazolin 2.5 mg in 0.1 ml
Vancomycin 1 mg in 0.1 ml
Withdraw 0.2 ml of vitreous, remove syringe
(Alternatives: Amikacin 0.4 mg in 0.1 ml, and inject pus/contents onto culture media
ceftazidime 2.25 mg in 0.1 ml) Inject 0.2 ml of antibiotics
Topical LA, clean eye with iodine
3. Procedure
Use 23G needle mounted on Mantoux syringe
with artery forceps clamped 10 mm from tip of
needle
Q Tell me about Posterior Capsule Opacication (PCO) after Cataract Surgery.
Posterior capsule opacication is a common complication after cataract surgery.

There are 3 types of PCO.
Management of PCO
1. Types of PCO 4. General principles of management
Proliferation of epithelium (Elschnigs pearls and Intraoperative stage prevention of PCO
Soemmerings ring) Surgical factors
Complete removal of soft lens matter
Primary opacication of capsule
Polish posterior capsule
Primary brosis of capsule
Consider primary posterior capsulotomy
2. Problems with PCO (pediatric cataract)
Visual dysfunction (VA, contrast, color) IOL design factors
Decrease view of fundus management of Acrylic IOL (lower risk because more IOL/
Diabetic retinopathy posterior capsule apposition)
RD Posterior bowing of optic (more IOL/
IOL decentration with capsular phimosis posterior capsule apposition)
3. Risk factors for PCO Laser barrier ridges (prevent epithelium
Young patient from migrating behind IOL)

Heparin-coated IOL (not proven)
DM, uveitis
Retained lens matter during surgery 5. Postoperative treatment
IOL design (controversial) square edged Nd:YAG capsulotomy [timing]
designs reduce PCO risk
B1037_Ch-01.indd 39 12/24/2010 2:34:14 PM

Q What are the Causes of Raised IOP/Low IOP/Shallow AC after Cataract Surgery?
Management depends on the severity and cause of the shallow AC.
The severity is graded as follows (see page 82).
The possible causes of shallow anterior chamber are.
IOP Shallow AC Deep AC
High Malignant glaucoma Retained viscoelastics

Suprachoroidal hemorrhage Retained soft lens matter
Pupil block glaucoma Inflammation, hyphema
Low Wound leak Ciliary body shutdown
Choroidal effusion Retinal detachment
Exam tips:
Very similar causes to shallow AC after trabeculectomy (see page 82).
Q How do You Control Postoperative Corneal Astigmatism?

Corneal Astigmatism after Cataract Surgery
1. Preoperative stage Avoid wound burns
Assess amount of astigmatism Limbal relaxing incisions
Use keratometry readings (not manifest Corneal relaxing incisions (less predictable)
refraction because astigmatism may be due to Newer technique: Femtosecond assisted
lenticular astigmatism) keratotomy for greater predictability

Consider astigmatism of the other eye (with- or Toric IOLs: Currently correct up to 3D
against-the-rule astigmatism) astigmatism
Prerequisites:
Plan surgery (ECCE vs phacoemulsication)
Astigmatically neutral/predictable
2. Intraoperative prevention incision
ECCE Stable in the bag IOL xation
Decrease size of incision
3. Postoperative management
Less diathermy
Manipulate frequency of steroid drops
Place IOL centrally
With-the-rule astigmatism more steroids
Wound closure/suture techniques
(delay healing, wound will slide)
Regularly placed sutures, short, deep bits
Against-the-rule less steroids (increase
If there is overlapping of wound edges,
healing and brosis)
sutures are too tight (with-the-rule
astigmatism) Selective suture removal according to
Phacoemulsication astigmatism
Site of incision Toric contact lens
Temporary or superior incision (based on Photorefractive keratectomy/excimer laser
preoperative astigmatism) procedures
Cornea, limbal or scleral tunnel (less Accurate keratotomy
astigmatism with scleral tunnel)
B1037_Ch-01.indd 40 12/24/2010 2:34:14 PM

TOPIC 9
SUBLUXED LENS AND

MARFANS SYNDROME
Q Opening Question: What are the Causes of Subluxed or Dislocated Lens?

Subluxed lens can be classied into 2 groups: Primary or secondary.
Classication of Subluxed Lens
1. Primary Ocular developmental disorders
Idiopathic Big eyes and cornea (megalocornea, high
Familial ectopic lentis (usually AD) myopia, bulphthalmos)
Iris anomalies (aniridia, uveal coloboma,
2. Secondary
corectopia)
Systemic disorders
Ocular diseases/acquired
Marfans syndrome
Trauma
Other connective tissue disorders (Weil
Uveitis
Marchesani, Sticklers, Ehler Danlos
Hypermature cataracts, pseudoexfoliation
syndromes)
syndrome
Metabolic disorders (homocystinuria,
Anterior uveal tumors (ciliary body
hyperlysinema)
melanoma)
Exam tips:
The classication is identical for congenital glaucoma (page 57) and congential
cataract (page 9)!
Q What are the Symptoms and Signs of Subluxed or Dislocated Lens?
Clinical Features
1. Symptoms Iridodonesis (better seen with undilated pupil)
Fluctuating vision Deep or uneven AC
Difculty in accommodation Uneven shadowing of iris on lens
Monocular diplopia Superior or inferior border of lens and zonules
High monocular astigmatism seen
2. Signs Acute ACG
Phacodonesis
B1037_Ch-01.indd 41 12/24/2010 2:34:14 PM

Q How would You Manage a Patient with Subluxed Lens?
I would need to assess the cause of the subluxation and manage both the ocular and systemic problems.
If the lens is dislocated into the AC.
Management of Subluxed Lens
1. Dislocation Phaco with devices for capsular stabilization
Surgical pearls:
Into AC
Intact CCC is critical to successful
Ocular emergency, immediate surgical
removal implantation of capsular stabilization
Into vitreous devices
CCC initiation may be difcult use
Lens capsule intact and no inammation,
consider leaving it alone sharp needle
Start CCC small, use shear method to
Lens capsule ruptured with inammation,
surgical removal indicated (pars plana control size
Aim is to implant CTR as late as possible
lensectomy)
during surgery, ideally after nucleus
2. Subluxed lens removed (CTR is bulky and difcult to
If asymptomatic, conservative treatment implant smoothly when lens still
(spectacles or contact lens) present)
Surgical removal indicated if there is Temporary capsular stabilization
Lens-induced glaucoma during surgery can be achieved with
Persistent uveitis capsular tension segments and iris/
Corneal decompensation capsular hooks
Cataract Lower phaco parameters
Severe optical distortion (despite conservative Use chop techniques to minimize
treatment) zonular stress
Surgical techniques < 1 quadrant zonulysis: Capsular tension
Standard ECCE/phaco (minimal subluxation, ring (CTR)

intact zonules) 12 quadrants zonulysis: Cionni 1L
ICCE (moderate subluxation, weaken modied CTR

zonules) > 2 quadrants zonulysis: Cionno 2L
ICCE with anterior vitrectomy (associated modied CTR

with vitreous loss)
Q What are the Clinical Features of Marfans Syndrome?
Marfans syndrome is a systemic connective tissue disorder.

There are characteristic systemic and ocular features.
Marfans Syndrome
1. Systemic features 2. Ocular features
AD inheritance Anterior segment
Skeletal Subluxed lens (bilateral, upward,
Tall and long arms (inappropriately long symmetrical)
armspan to height) Glaucoma (angle anomaly)
Fingers (arachnodactyly, joint laxity) Keratoconus
High arched palate Hypoplasia of dilator pupillae (difcult to
Scoliosis and pectus abnormalities dilate pupils)
Hernias Posterior segment
Cardiac Axial myopia
Mitral valve prolapse RD
Aortic aneurysm, aortic incompetence and
aortic dissection
B1037_Ch-01.indd 42 12/24/2010 2:34:15 PM

Clinical approach to Marfans syndrome

On SLE, there is bilateral upward dislocation of lens.
However, the lens is not cataractous and the zonules can be seen inferiorly.
Look for
Corneal evidence of keratoconus
Dilated pupil
Systemic features
High arched palates
Arachnodactyl, joint flexibility
Tall, wide armspan, scoliosis, chest deformity
Id like to
Check the IOP
Perform a gonioscopy
Refract the patient (high myopia)
Examine the fundus (myopic changes and RD)
Examine the cardiovascular system (aortic incompetence, mitral valve
prolapse)
Evaluate family members (for Marfans syndrome)
Exam tips:
Listen to the question, What are the CLINICAL FEATURES? which is
different from What are the OCULAR features?
Q What are the Differences between Marfans Syndrome, Homocystinuria and Weil Marchesani Syndrome?
Marfans Homocystinuria Weil Marchesani
Inheritance AD AR AD
Intellect Normal Mental retardation Mental retardation
Fingers Arachnodactyly Short stubby fingers
Osteoporosis Severe
Vascular complications Severe
Cardiac Severe
Lens subluxation Upwards Downwards Downwards
Zonules present Zonules absent Microspherophakia
Accommodation Intact Lost
B1037_Ch-01.indd 43 12/24/2010 2:34:15 PM

Section 2
Glaucoma AND
Glaucoma Surgery
B1037_Ch-02.indd 45 12/22/2010 4:07:33 PM

TOPIC 1
LIMBUS, CILIARY BODY

AND TRABECULAR
MESHWORK
Q Opening Question: Where is the Limbus?

The limbus is the structure between the cornea and the sclera.
It can be dened in 3 ways.
Limbus
1. Anatomical limbus: Posterior limit formed by line perpendicular
Anterior limit of limbus formed by a line joining to surface of conjunctival epithelium about
end of Bowmans and end of Descemets 1.5 mm behind end of Bowmans membrane
(Schwalbes line) 3. Surgical limbus:
Posterior limit is a curved line marking transition Annular band 2 mm wide with posterior limit
between regularly arranged corneal collagen overlying scleral spur
bres to haphazardly arranged scleral collagen Divided into:
bres Anterior blue zone (between Bowmans and
2. Pathological limbus: Schwalbes line)
Anterior limit same as in one Posterior white zone (between Schwalbes
line and scleral spur)
Exam tips:
Some of the most commonly asked anatomy or physiology questions in the
examinations.
Q What is the Anatomy of the Ciliary Body?
The ciliary body is a triangular structure located at the junction between the anterior and posterior segment.
Anatomically it is part of the uveal tract.
B1037_Ch-02.indd 47 12/22/2010 4:07:34 PM

Ciliary Body
1. Function of the ciliary epithelium III CN
Secretion of aqueous humor by ciliary Branch to IO muscle
Ciliary ganglion
nonpigmented epithelium (NPE)
Short ciliary nerves
Accommodation
Sphincter pupillae
Control of aqueous outow
Sympathetic bers from superior cervical
Part of BLOOD-AQUEOUS Barrier:
ganglion to ciliary body as follows:
Formed by tight junctions between NPE (as
Superior cervical ganglion
well as nonfenestrated iris capillaries)
Ciliary ganglion
Maintain clarity of aqueous humor required
Short ciliary nerves
for optical function
Muscle and blood vessels of ciliary body
Secretion of hyaluronic acid into vitreous
Sensory bers from ciliary body to CNS as
2. Gross anatomy follows:
Ciliary body, iris and choroid comprise vascular Ciliary body
uveal coat Long posterior ciliary nerves
Ciliary body: Nasociliary nerve
6 mm wide ring in inner lining of globe Ophthalmic division of V CN
Extending from ora serrata posteriorly to Brainstem
scleral spur anteriorly 5. Microscopic anatomy
Triangular in cross section:
Histologically divided into three parts:
Anterior surface (uveal portion of
Ciliary epithelium (double layer)
trabecular meshwork)
Ciliary stroma
Outer surface (next to sclera, potential
Ciliary muscle
suprachoroidal space between ciliary body
Longitudinal, radial and circumferential
and sclera)
Inner nonpigmented epithelium (NPE)
Inner surface (next to vitreous cavity)
Direct contact with aqueous humor
Smooth pars plana (posterior 2/3)
Columnar cells with numerous organelles
Ridged pars plicata (anterior 1/3)
Extension of sensory retina with basal
Pars plicata 70 ciliary processes
membrane, an extension of inner limiting
3. Blood supply membrane
Arterial supply: Outer pigmented epithelium (PE)
Seven anterior ciliary arteries and two long Between NPE and stroma
posterior ciliary arteries Cuboidal cells, with numerous melanosomes,
Anastomosis of the two forms the major fewer organelles compared to NPE
arterial circle of iris Extension of RPE with basal membrane, an
Located at base of the iris within ciliary
extension of Bruchs membrane
process stroma NPE and PE lie apex to apex
Venous drainage Different types of intercellular junction join NPE
Ciliary processes venules drain into pars
and PE:
plana veins, which drain into vortex
Tight junctions between NPE (with
system
nonfenestrated iris vessels) form BLOOD-
4. Nerve supply AQUEOUS Barrier
Main innervation from branches of long Desmosomes found between internal surfaces
posterior ciliary and short ciliary nerves of NPE cells
Parasympathetic bers from Edinger-Westphal Gap junctions found between NPE and PE
nucleus to sphincter pupillae as follows:
Edinger-Westphal nucleus
B1037_Ch-02.indd 48 12/22/2010 4:07:35 PM

Exam tips:
Compare and contrast the two epithelial layers (nonpigmented vs pigmented
epithelium). Note that while the pigmented epithelium is an extension of
the RPE (as expected), it is NOT part of the BLOOD-AQUEOUS Barrier
(unexpected, as the RPE forms the blood retinal barrier).
Q What is the Anatomy of the Trabecular Meshwork?
The trabecular meshwork is located at the angle of the anterior chamber, beneath the limbus.
Its main function is drainage of aqueous.
Trabecular Meshwork
1. Gross anatomy From root of iris to Schwalbes line
Triangular in shape: 70 m in diameter (least resistance to ow)
Base located at scleral spur Corneoscleral meshwork:
Anterior tip located at Schwalbes line From scleral spur to Schwalbes line
(= termination of Descemets) 35 m in diameter (moderate resistance)
2. Microscopic anatomy Juxtacanalicular:

Three zones (from innermost to outermost): Lines the endothelium of Schlemms canal
Uveal meshwork: 7 m in diameter (highest resistance to
ow)
Exam tips:
The pore diameter in the juxtacanalicular meshwork is 10 times smaller than
the uveal meshwork, while the corneoscleral meshwork is 2 times smaller.
Q What are the Blood Ocular Barriers? When are They Breached?
There are two blood ocular barriers.

They are breached in certain circumstances.
Blood Ocular Barriers
1. Classication Endocrine modications:
Blood-aqueous barrier (BAB): Rapid, reversible increments in
Nonfenestrated iris capillaries permeability via secretion of hormones

Tight junctions between ciliary (histamine, serotonin, bradykinin, etc.)
nonpigmented epithelium (NPE) Pathological:
Blood retinal barrier (BRB): Defect in BRB in vascular diseases:
Nonfenestrated retinal capillaries Diabetic retinopathy and hypertensive
Tight junctions between RPE retinopathy
Ciliary processes and choroidal capillaries are BRVO, CRVO
fenestrated and do not contribute to barrier Defect in BAB and BRB after cataract or other
2. Breach of the barriers intraocular surgery
Physiological: Defect in BAB and BRB in ocular tumors
Defect in BRB exists at level of optic disc: Defect in BAB and BRB in ocular inammatory
Water-soluble substances may enter ON or infectious diseases
head by diffusion from extravascular space
in choroid
B1037_Ch-02.indd 49 12/22/2010 4:07:35 PM

TOPIC 2
AQUEOUS HUMOR AND

INTRAOCULAR PRESSURE
Q Opening Question: What is the Aqueous Humor?

The aqueous humor is the uid in the anterior (AC) and posterior chamber (PC).
It has the following properties.
And its functions include, rst, the maintenance of.
The aqueous humor is formed in the PC by.
Aqueous Humor
1. Properties Optical role
Clear uid 3. Formation and outow
Composition: Three formation mechanisms from ciliary body
No cells and less than 1% of proteins process (nonpigmented epithelium):
compared to plasma Active transport (most important)
Same sodium and chloride, slightly lower Ultraltration
potassium and 30% lower bicarbonate than Diffusion
plasma Three outow mechanisms:
Thirty times higher ascorbate than plasma Trabecular meshwork/pressure dependent
Refractive index (RI) = 1.33 ow
Therefore, diverges light (!) because RI of 90% of outow
cornea: 1.37 Related to IOP via Goldman equation (see
Volume in AC and PC = 0.30 ml below)
0.25 ml in AC Uveoscleral/pressure independent ow:
0.05 ml in PC 10% of ow
Rate of secretion = 3 ul/min (therefore takes Aqueous enters ciliary body into the
100 min to completely reform AC and PC!) suprachoroidal space and vortex veins
Rate of aqueous ow quite constant and
2. Three functions
independent of IOP
Maintains volume and IOP
Other routes
Nutrition for avascular ocular tissue:
Iris veins
Posterior cornea, trabecular meshwork, lens
Retinal veins
and anterior vitreous
Via the cornea
Exam tips:
Notice the importance of number 3 in aqueous humor physiology!
Another possible question is, What are the differences between aqueous and
plasma?
B1037_Ch-02.indd 51 12/22/2010 4:07:35 PM

Q What is the Goldman Equation?
The Goldman equation states that the IOP is determined by three interrelated factors.
The Goldman Equation:
1. Goldman equation states that the following Resistance encountered in outow channel (R in
factors determine IOP ul/min/mmHg):
IOP = F/C + Pr Resistance to ow related to facility of
Rate of aqueous secretion (F, in l/in) outow

R = 1/C, where R is resistance and C is facility
Level of episcleral venous pressure (Pr, in
of outow
mmHg)
Q How does IOP Vary?
The IOP can have long term variation and short term uctuations, and are affected by drugs.
IOP Variation
1. Long term variations 3. Pharmacological effects
Age: Miotics
Increase with age Generally decreases IOP
Blood pressure: Effects:
Increase with BP but not linearly Contraction of iris sphincter
Contraction of ciliary muscle pulls scleral

Body weight:
Increase with increase in body mass spur, leading to change in trabecular
Climate: meshwork and increase in outow
Direct parasympathomimetics (pilocarpine,
Increase in winter
carbachol) and indirect (phospholine
2. Short term uctuations iodide)
CVP changes Mydriatics:
Change in body position and valsalva Generally increases IOP
maneuver Effects
Diurnal variation: Allows peripheral portion of anterior iris
Increase in morning stroma to move forward towards inner
Normal variation = 4 mmHg (> 10 mmHg in aspect of uveoscleral meshwork, leading to
glaucoma) decrease in trabecular outow facility
Correlates with increase in endogenous Others:
cortisol and catecholamines Beta adrenergic blockers (e.g. timolol)
Accentuated in POAG reduces production of aqueous and IOP
Eye movement Carbonic anhydrase inhibitors (e.g. diamox)
Clinically important in restrictive affects membrane transport of bicarbonate
ophthalmopathy (e.g. thyroid eye disease, and water across ciliary epithelium, thereby
pseudotumor) reducing production of aqueous and IOP
Exercise: Hyperosmotic agents (e.g. mannitol, glycerol)
Decrease in IOP elevates blood osmolality with resulting uid
Correlated to metabolic acidosis and changes shift out of the vitreous
in extracellular uid volume and osmolality Steroids (page 36)
Exam tips:
The variations can be easily remembered as A, B, C, D, E and F (pharmaco-
logical)!
B1037_Ch-02.indd 52 12/22/2010 4:07:35 PM

Q What is Tonometry?
Tonometry is the measurement of the IOP.

There are three types of tonometry.
Tonometry
1. Applanation tonometry Method:
Determines force necessary to atten a xed Patient in supine position
Topical anesthetic
area of cornea
Tip of plunger allowed to rest on surface of
Based on the Imbert-Fick principle:
Force required to atten an area of a perfect
eye forcing an indentation
Depth of indentation registered on scale in
sphere is proportional to pressure inside
sphere mm
IOP in mmHg read off from conversion
Goldman tonometer:
chart
Double prism in tonometer tip to facilitate
visualization 3. Combined applanationindentation tonometry
Tonometer tip has diameter of 3.06 mm Mackay-Marg tonometer:
Surface tension of tear meniscus = Contains a spring-mounted plunger and
corneoscleral rigidity (cancels each other) surface footplate
End point occurs when inner border of two Plunger has 1.5 mm area that protrudes
semicircular halves of tear meniscus touch 10 m through center of footplate

one another Initially plunger indents cornea (indentation)
IOP measured in mmHg End point occurs when plunger indents
Air puff tonometer: enough to be ushed with footplate
Non-contact (applanation)
Pressurized air current directed against a Other examples (e.g. tonopen)
xed area of cornea 4. Tonometry that reduces the inuence of the
Not reliable in extremes
corneal biomechanical properties
2. Indentation tonometry Dynamic control tonometry (DCT)
Determines extent of indentation of cornea by Ocular response analyzer tonometry (ORA)
xed weight
Schiotz tonometer
Plunger with known weight indents cornea
Weights of 5.5, 7.5 or 10 g
B1037_Ch-02.indd 53 12/22/2010 4:07:35 PM

TOPIC 3
OPTIC DISC CHANGES

IN GLAUCOMA
Q Opening Question: What are the Optic Disc Changes in Glaucoma?

Optic disc changes in glaucoma can be divided into specic and less specic signs.
Specic signs include an increase in cup disc ratio (CDR).
Optic Disc Changes In Glaucoma
1. Specic signs Splinter (Drance) hemorrhage
Optic disc cupping: Nerve ber layer thinning
Large optic cup (vertical CDR 0.7 or more) 2. Less specic signs:
Asymmetry of optic cup (difference of CDR Lamellar dot sign
0.2 or more) Nasalization of vessels
Progressive enlargement of optic cup
Peripapillary crescent (Beta zone)
Does not obey the ISNT rule
Barring of circumlinear vessels
Focal signs:
Notching of rim
Regional pallor
Exam tips:
There are four cup signs, four focal signs and four less specic signs.
Q What are the Clues that a Large Optic Cup is Physiological?
Physiological Cupping
1. Optic disc: Optic disc may be large
No progression in cupping No focal changes or vessel abnormalities
Symmetrical cupping 2. Associated with consistently normal IOP and VF
Follows ISNT rule
B1037_Ch-02.indd 55 12/22/2010 4:07:36 PM

Q What are the New Imaging Techniques Available for Glaucoma Evaluation?
The imaging techniques can be classied into anterior segment and posterior segment techniques.
Imaging Techniques in Glaucoma
1. Anterior segment HRT software automatically denes a
Ultrasound biomicroscopy: reference plane:
Evaluate angle of AC and ciliary body Cup: Structures below reference plane
Requires contact of ultrasound probe with Rim: Structures above reference plane
ocular surface Optic nerve head analysis (HRT-III)

Indications: Stereometric parameters:
Angle closure glaucoma Linear cup/disc ratio
Malignant glaucoma Cup shape measure
Plateau iris syndrome Rim area
Anterior segment optical coherence tomography Rim volume
(AS-OCT): Height variation contour
Evaluates angle of AC, but penetration Mean RNFL thickness
insufcient for imaging the ciliary body Disc size
Advantages: Moorelds Regression Analysis (MRA)
Non-contact modality no distortion of Glaucoma Probability Score (GPS)
AC angle Progression analysis
More objective than gonioscopy Advantages:

Faster and easier to obtain images Higher resolution
compared to ultrasound biomicroscopy Miotic pupils and media clarity not
Disadvantages: important
Scleral spur not easily identied in at least Automated denition of disc margin
20% of AS-OCT images (HRT-III)

Unable to image ciliary body Superior to OCT in detecting progression
Quality of vertical images inferior to Disadvantages:

horizontal images Physiologic variability of optic nerve head
2. Posterior segment conguration high

Poor accuracy in:
Stereoscopic optic disc photography (stereodisc
Tilted discs
photography):
Very large or small discs
Document optic disc changes
Peripapillary atrophy
Advantages:
Cheap and simple
Optical coherence tomography:
More objective than clinical evaluation
Image formation based on optical
Glaucomascope: backscatter, similar to ultrasound B scan of

Computer raster stereography where a series
optic disc
Advantages:
of equidistant parallel lines are projected
Highest resolution
onto optic disc at an oblique angle.
Noncontact, noninvasive
Deection of lines gives an indication of
Miotic pupils and media clarity not
depth of optic cup
Advantage:
important
More quantitative than stereodisc photos
Disadvantages:
Time domain OCT inferior to HRT in
Needs minimal pupil size of 4 mm and clear
media detecting glaucoma progression
Glaucoma software for spectral domain
Confocal scanning laser ophthalmoscopy:
OCT still in initial phases and expensive
Heidelberg Retinal Tomograph (HRT):
RNFL reduced in other conditions
Sequential images of coronal sections of
e.g. myopia
optic disc are obtained via laser
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TOPIC 4
THE VISUAL FIELDS

Q Opening Question: What is the Visual Field? What is an Isopter? And What is a Scotoma?
The visual eld (VF) is one of the functional components of vision.
It is dened as the area that is perceived simultaneously by a xating eye.
Visual Field Basics
1. Denition: VF defect
Area that is perceived simultaneously by a Absolute or relative decrease in retinal
xating eye sensitivity extending from edge of VF
Not 2- but 3-dimensional 5. Luminance and visual threshold
Island of vision in a sea of darkness (Traquairs Luminance:
denition) Intensity of light
2. Limits: Apostilb (asb) is an absolute unit of
60 nasally, 50 superiorly, 90110 temporally, luminance
70 inferiorly Normal human range: 2 to 9,000 asb
Humphrey VF can measure from 0.08 to

Blind spot 15 temporal to xation
10,000 asb
3. Isopter
Decibel (dB) is a relative unit of luminance
Line in VF connecting points with same visual Inverse log scale
threshold 10,000 asb = 0 dB; 1 asb = 40 dB
Encloses an area within which a target of a Visual threshold:
given size and intensity is visible Luminance of stimulus which is perceived
4. Scotoma and VF defect 50% of time
Scotoma The brighter the stimulus needed to be
Absolute or relative decrease in retinal perceived, the lower the visual threshold
sensitivity within VF, bounded by areas of Therefore, bright stimulus = high asb =
normal retinal sensitivity low dB = low visual threshold
Exam tips:
See also the visual eld examination in neuroophthalmology (page 255).
Q What is Perimetry? What are the Types and Advantages of Each?
Perimetry is the quantication of the VF.

It can be divided into.
B1037_Ch-02.indd 57 12/22/2010 4:07:36 PM

Perimetry Basics
1. Classication Extend beyond 30 (peripheral elds)
Campimetry (at surface): Humphrey visual eld analyzer (HVF):
Tangent screen: Automated and static
Manual and kinetic Test central 30
Test central 30
2. Advantages of automated (over manual):
Subject seated 1 or 2 meters from black
More quantitative
screen
No examiner bias
Target is presented by examiner
Constant monitoring of xation
Perimetry (curved surface):
Automated re-testing of abnormal points
Lister:
Manual and kinetic
Computer software for analysis
Extend beyond 30 (peripheral elds) 3. Advantages of static (over kinetic):
Goldman bowl perimeter: More objective and quantitative
Manual and kinetic or static More sensitive to shallow scotomas
Hemispherical bowl with radius of 33 cm Random presentation of stimuli (less
(subject at 33 cm) anticipation of subject)
Stimuli has different intensities (14) and
Faster
sizes (IV)
Exam tips:
Comparison between Goldman and HVF is a common question.
Q What are the Uses of Visual Field in Ophthalmology?
VF is used for diagnosis and follow-up of ophthalmic conditions.

Uses of Visual Field
1. Diagnosis of Unexplained visual loss
Glaucoma Malingering patients
Optic nerve diseases (optic neuritis, anterior 2. Follow-up of
ischemic optic neuropathy, toxic neuropathy) Glaucoma
Tumors (pituitary adenoma)
Q Tell me about the Humphrey Visual Field Analyzer.
Humphrey visual eld analyzer is an automated static perimetry.

Maps the VF by quantifying the visual threshold of the retinal at predetermined locations.
Humphrey Visual Field Analyzer
1. Basic Stimuli intensity increases in 4 dB steps until
Automated static perimetry threshold is crossed (patients see stimuli)
Stimuli (size = Goldman size III with duration of Threshold is recrossed with stimuli intensity
0.2 s) decreasing in 2 dB steps
Test pattern:
Background illumination = 31.5 asb
242 test pattern:
2. Test strategies Test central 24 of xation and on either
Full threshold strategy: side of meridian (242) as opposed to
Uses the 42 bracketing algorithm at each tests on meridians as well (241)
retinal point 301 or 302 (Test central 30 of xation)
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Related threshold strategies: values and then tests only abnormal

Full threshold with prior data: points
Faster, uses prior VF data, presents each Suprathreshold test strategy:
point at 2 dB higher than patients Fast screening test
previous threshold values and tests each Presents stimuli at 6 dB higher than expected
point in 2 dB decrement threshold
Fast threshold: Each point is recorded as normal vs
Even faster, presents entire eld at 2 dB abnormal
higher than patients previous threshold
Q How do You Read the Humphrey Visual Field?
This is a HVF for the left and right eyes respectively.

Done on January 2nd, 1999, using the 242 threshold test pattern.
First, the reliability indices are.
Evaluating the HVF
1. Reliability indices Short-term uctuation (SF):
Fixation loss: Retests 10 pre-selected points and calculates
Positive response to blind spot stimulation differences between original test and retest
Moving eyes around thresholds
Normal: Less than 20% Measures consistency
False positive: One of earliest signs of glaucoma (patient
Positive response but no stimuli sometimes sees it and sometimes does not)
Happy clicker May also be indicator of reliability
Normal: Less than 33% Corrected pattern standard deviation (CPSD):

False negative: PSD corrected for SF
Negative response with brighter than Measures localized variability/irregularity (a

threshold stimuli high CPSD indicates localized irregularity)
Falling asleep Glaucoma hemield test (GHT):
Normal: Less than 33% 5 clusters of points in upper and lower half of
Other clues: VF are compared
Short-term uctuation signicantly raised Early indicator of glaucoma
Clover leaf pattern on grayscale 3. Figures

(inattentiveness with time) Raw numeric values and grayscale:
Increased eye (upstroke) or lid (downstroke) Threshold at each point (e.g. 25 dB at that
movement on eye tracker line point means visual threshold is 25 dB)
2. Global indices Total deviation:
Mean deviation (MD): Age-corrected normal minus raw threshold
Average deviation of each point from value (e.g. 2 dB at that point means visual
age-corrected normal (e.g. 5 dB MD means threshold is 2 dB less than what is normal at
that on average, each point has a 5 dB lower that point)
threshold than normal) Similar to MD
Minus is bad Pattern deviation:
Pattern standard deviation (PSD): Total deviation adjusted upwards or
Standard deviation of each point from downwards to pick up localized defects
age-corrected normal Measures localized defects
Measures the variability/irregularity of eld Similar to CSPD
(e.g. a high PSD indicates irregular eld)
Plus is bad
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Interpretation
Total deviation Pattern deviation
MD CSPD plot plot Diagnosis
Normal Normal Clean Clean Normal
Abnormal Normal Many abnormal Clean Generalized loss of

points sensitivity (e.g. cataract)
Normal Abnormal Many abnormal Similar abnormal Localized defect
points points (e.g. glaucoma)
Abnormal Abnormal Many abnormal Less abnormal points Both generalized and
points but not clean localized defects
Exam tips:
You may be given a HVF printout to read. You need to be systematic and not
jump at the obvious VF defect seen.
Remember Mean deviation = Minus is bad. Pattern standard deviation = Plus
is bad.
Q What are the Visual Field Defects in Glaucoma?
The VF defects can be divided into early and late.

Glaucoma Visual Field Defects
1. Early: 2. Late:
Paracentral nasal defect Arcuate defect/double arcuate
Nasal step/temporal wedge Temporal island with central vision
Scotoma in Bjerrums area (1020 of xation) Central vision only (tunnel vision)
Siedals scotoma (defect extending from blind spot)
Q What are the Newer VF Techniques?
Newer Perimetry Techniques

1. SITA (Swedish Interactive Thresholding Algorithm) Knows when to quit when standardized
Aims to increase speed without losing accuracy amount of information is obtained
Uses all information from every point
SITA Standard:
Full version comparable to standard threshold 2. SWAP (Short Wavelength Automated Perimetry)
algorithm in sensitivity and accuracy twice Blue on yellow perimetry
but as fast Blue-yellow ganglion cells rst lost in
SITA Fast: glaucoma
Similar to suprathreshold algorithm in SWAP detects abnormal VF 25 years before
sensitivity and accuracy but twice as fast white on white VF abnormal
How SITA works: The presence of cataracts may affect the
Smart questions and smart pacing accuracy of SWAP
All factors considered as test occurs,
3. Frequency doubling perimetry
producing estimate of threshold at each
point Low spatial frequency sinusoidal grating
Uses normal age-corrected threshold values undergoing high temporal frequency icker
as starting point Tests magnocellular pathway, which appears to
Real time calculation and re-calculation of be rst lost in glaucoma
threshold values as test proceeds Possible screening tool for future
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TOPIC 5
GONIOSCOPY
Q Opening Question: What is Gonioscopy?

Gonioscopy is an evaluation of the AC angle.
It overcomes total internal reection.
There are two types of lens used to evaluate the angle.
Gonioscopy
1. Principle Second largest at 67 (visualize equator to
Light from AC angle exceeds critical angle at retinal periphery)
cornea-air interface, undergoes total internal Smallest (semicircular) at 59 (visualize
reection and cannot be seen angles)

Goniolens has similar refractive index as cornea Zeiss goniolens:
Diameter of 9 mm and atter than cornea
and alters cornea-air interface to allow light to
Can be used for indentational gonioscopy
pass from AC through cornea into lens
Lack stability and therefore not good for
Critical angle of new interface between lens and
argon laser trabeculoplasty
air is not exceeded and therefore images from 4-mirror angled at 64
angle can be visualized Can see entire extent of angle
Indirect goniolens provide mirror image of angle, No coupling uid needed
while direct lens provide actual view of angle Better visualization
2. Indirect goniolens Posner 4-mirror and Sussman 4-mirror lens

Goldman goniolens: (modied Zeiss with handle)
Diameter of 12 mm 3. Direct goniolens
Stabilizes globe and therefore good for argon Diagnostic:
laser trabeculoplasty Koeppe (prototype diagnostic goniolens):
Needs coupling uid Stable
2-mirror lens angled at 62 Entire extent of angle can be viewed
3-mirror lens:
Surgical:
Largest mirror at 73 (visualize posterior
Barkan (prototype surgical goniolens),
pole to equator) Medical Workshop, Thorpe and others
Exam tips:
Candidates seldom answer the principle of gonioscopy well.
The comparison between Goldman and Zeiss is another favorite question.
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Q How do You Perform a Gonioscopic Examination?
Gonioscopy is an evaluation of the AC angle.

A systematic evaluation of the structures of the angle is done as follows.
Gonioscopic Examination
1. Grade the angle (see below) M elanomosis of angles (oculodermal
Standard gonioscopy melanosis)
Indentational gonioscopy E ndocrine (diabetes and Addisons
Differentiate appositional closure from syndrome)
synechial closure N evus (Cogan Reese syndrome)
T rauma
2. Assess the structures
Peripheral anterior synechiae
Anterior displacement of Schwalbes line
Posterior embryotoxon
Blood in Sclemms canal (raised episcleral
Trabeculum pigmentation: venous pressure):
Carotid cavernous stula
P seudoexfoliation and pigment dispersion
Sturge-Weber syndrome
syndrome
Superior vena cava obstruction
ritis
Ocular hypotony
G laucoma (post angle closure glaucoma)
Post gonioscopy
Exam tips:
The differential diagnoses for trabecular pigmentation can be remembered by
the mnemonic PIGMENT!
Notes
Compared to iris processes, peripheral anterior synechiae are denser, more
irregular and extend beyond scleral spur
Q How is the Angle Clinically Graded?
The angle is graded according to classication systems such as.

Grading of Angle
Shaffer system (1 V. 4) Scheies system

(IIV)
Grade 4 (40)
Ciliary body seen Grade I
Grade 3 (30)
Scleral spur seen Grade II
Grade 2 (20)
Trabeculum seen Grade III
Grade 1 (10)
Schwalbes line seen Grade IV
Grade 0 (closed angle)
Iridocorneal contact
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Spaeth system
Angular approach (degrees)
Curvature of peripheral iris
Regular (normal)
Steep (risk of closure)
Queer (aphakia, pigmentary glaucoma,
subluxed lens)
Iris insertion
A = Above Schwalbes line
B = Below Schwalbes line
C = At scleral spur
D = Anterior part of ciliary body
E = Ciliary body
Q How do You Clinically Assess the AC Depth?
We can clinically assess separately the central or peripheral AC at the slit lamp.
Clinical AC Depth Assessment
1. Peripheral (Van Hericks method): 2. Central (Redman-Smith method):
Narrow slit beam from 60 Narrow slit beam from 60
Beam aligned vertically and directed just inside Beam is aligned horizontally and focused
limbus between the corneal endothelium and lens
Distance between corneal endothelium and iris Adjust width of slit:
compared with corneal thickness Align the nasal end of corneal slit with
AC considered shallow when this distance is temporal end of lens slit
< 1/4 corneal thickness This length is measured in mm
AC depth = length of slit (mm) + 1.1 + 0.5
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TOPIC 6
CONGENITAL
GLAUCOMAS
Q Opening Question: What are the Causes of Congenital Glaucomas?

Congenital glaucoma can be classied into two groups: Primary or secondary.
Secondary causes include.
Classication of Congenital Glaucoma
1. Primary: Phakomatoses (Sturge-Weber syndrome)
Congenital (birth), infantile (12), juvenile Ocular developmental disorders:
(216) Anterior segment dysgenesis, aniridia
Congenital ectropian uvea, nanophthalmos
2. Secondary:
Systemic disorders: Ocular diseases:
Retinoblastoma, ROP, persistent hyperplastic
Chromosomal disorders
Metabolic disorders (Lowes syndrome,
primary vitreous, trauma, uveitis
Zellwegers syndrome)
Exam tips:
The classication is exactly the same as for congenital cataracts (page 9)!
Q What are the Causes of Cloudy Cornea at Birth?
Cloudy corneas can be caused by many different disorders.

They can be classied based on the size of the eye.
Cloudy Cornea at Birth
1. Large eye: 3. Normal size eye:
Congenital glaucoma Diffuse opacity:
Mesenchymal dysgenesis Congenital hereditary endothelial dystrophy
Congenital hereditary stromal dystrophy
2. Small eye:
Sclerocornea
Microphthalmos
Mucopolysaccharidosis
Severe prenatal infection Mucolipidosis
Mesenchymal dysgenesis Interstitial keratitis
Congenital glaucoma
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Regional opacity Round:

Linear: Infective keratitis
Forceps injury Peters anomaly
Congenital glaucoma (Haabs striae) Localized mesenchymal dysgenesis
Exam tips:
Differential diagnosis of cloudy cornea at birth:
S Sclerocornea
T Trauma
U Ulcer
M Metabolic disease
P Peters anomaly
E Endothelial dystrophy
D Descemets membrane breaks
Q How do You Manage Congenital Glaucomas?
The management of congenital glaucoma is difcult.

And involves a multidisciplinary team approach.
The important issues include.
A complete history and physical examination, usually under anesthesia, is needed.
Management of Congenital Glaucoma
1. Issues in management Photophobia
Assessing etiology and inheritance of congenital Blepharospasm
glaucoma Red eye
Poor vision
Managing systemic problems of secondary
congenital glaucoma Signs:
A xial myopia
Deciding on type of surgery (corneal diameter
B uphthalmos
as a guide):
C loudy cornea
< 13 mm: Goniotomy/trabeculotomy
D escemets breaks (Haabs striae)
> 14 mm: Trabeculotomy/trabeculectomy/
D iameter of cornea enlarged
valve implant
D isc cupping
> 16 mm: Cyclodestructive procedures
E xamination under anesthesia
(usually very poor prognosis)
Examination under anesthesia:
Managing associated ocular problems and
Ketamine anesthetic (other agents like
amblyopia: isourane, halothane give falsely low IOP)
Refractive errors
IOP (tonopen or Perkins)
Corneal opacity
Opthalmoscopy (disc)
Cataract
Gonioscopy (Koeppe)
Squint
Refraction (retinoscopy)
2. Physical examination Corneal diameter (horizontal and vertical)
Symptoms:
Tearing
Exam tips:
Like management of congenital cataracts (page 9), this is a fairly difcult
question to handle.
Provide precise opening statements to capture spectrum of related problems.
B1037_Ch-02.indd 66 12/22/2010 4:07:37 PM

Notes
The clinical signs can be remembered as ABCDE
Q What is Goniotomy and Trabeculotomy?
Goniotomy and trabeculotomy are surgical operations for congenital glaucoma.

Treatment Options for Congenital Glaucoma
1. Goniotomy Withdraw trabeculotome and introduce it in
Establish communication between AC and the opposite direction
Schlemms canal Result:
Indications: Similar results as goniotomy but conjunctiva
Usually in children < 3 years violated

Common conditions: Primary congenital 3. Trabeculodialysis
glaucoma, Sturge-Webers syndrome, Lowes Similar to goniotomy
syndrome Usually for children with secondary glaucoma
Requires clear cornea from inammation ( Juvenile chronic arthritis)
Procedure: Differs from goniotomy in that knife cuts are
Incision made at supercial layer of made at Schwalbes line
meshwork, midpoint of trabecular band Meshwork is pushed inferiorly using at side of
(midpoint of Schwalbes line and scleral spur)
blade and is disinserted from scleral spur
Each sweep for 120
Iris should drop posteriorly 4. Trabeculectomy
Repeat from opposite side Needs mitomycin C/5 uorouracil application
Results: Problems with trabeculectomy in children
Good initial results (85% success) Thick Tenons
However, 40% need re-operation Thin sclera
Repeated up to three times Difculty in identifying limbus
Higher rates of scarring and trabeculectomy
2. Trabeculotomy
failure
Establish communication between AC and
Risk of endophthalmitis
Schlemms canal by removal of a portion of
Chronic post-op hypotony frequent
trabecular meshwork (goniotomy ab externo)
5. Glaucoma drainage implants
Indications:
Usually in children > 3 years
Mitomycin C or 5-uorouracil application
Common conditions: Juvenile glaucoma, usually indicated in congenital glaucoma
Axenfelds anomaly, Peters anomaly Some advocate the use of glaucoma drainage
Poor corneal visibility implants as a primary surgical intervention in
Procedure: congenital glaucoma, due to the problems with
Scleral ap fashioned, usually inferotemporal trabeculectomy in children
region (preserve superotemporal conjunctiva Often more than one implant needed
for trabeculectomy later) Indication: Refractory primary congenital
Radial incision made over Schlemms canal glaucoma
until it is inserted Complications (similar to those in adults):
Check location of Schlemms canal by
Hypotony
threading 5/0 nylon into canal Tube exposure
Trabeculotome inserted into canal and
Endophthalmitis
rotated into AC, tearing meshwork Corneal decompensation
Persistent Iritis
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6. Cyclodestruction Compliance:
Indications: Toxicity, especially systemic toxicity
Painful blind eye Alpha2 agonists: Bradycardia, hypotension,
Glaucoma refractory to other forms of apnea (cross blood-brain barrier in

treatment children)
Patient not t for surgery Prostaglandin analogues and miotics:
Complications: Often ineffective in congenital glaucoma

Chronic hypotony/phthisis Beta blockers: Asthma, bradycardia
Vision loss Side effects are usually very different from
Cataracts those of adults (e.g. failure to thrive, bed

wetting, abnormal school behavior)
7. Medical therapy
Problems
Not very effective
Q What are Mesodermal Dysgeneses?
Mesodermal dysgeneses are a group of congenital disorders.

Which involves the cornea, iris and AC angle.
And frequently associated with congenital glaucoma.
Mesodermal Dysgeneses and Aniridia
Riegers anomaly
Axenfelds anomaly and syndrome Peters anomaly Aniridia
Inheritance AD AD AD AD
AR (Mental
retardation)
Sporadic (Wilms
tumor)
Iris Posterior embryo- Posterior embryo- Posterior embryo- Aniridia
toxon toxon toxon
Iris strands Iris hypoplasia, Iris hypoplasia,
corectopia, corectopia,
polycoria, polycoria,
ectropion uvea ectropion uvea
Cornea Corneal opacity Corneal opacity
Keratolenticular Keratolenticular
adhesions adhesions
Corneal plana, Corneal plana,
sclerocornea sclerocornea
Others Cataract Cataract
Foveal hypoplasia
Nystagmus
Choroidal
coloboma
Glaucoma Glaucoma rare Glaucoma in 50% Glaucoma in 50% Glaucoma in 50%
Systemic None Dental and facial None Wilms tumor in
malformations in AR trait
Reigers syndrome
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Clinical approach to mesodermal dysgenesis

The most obvious abnormality is the presence of posterior embryotoxon.
There are also diffuse areas of iris atrophy, corectopia, ectropian uvea.
Look for
Corneal opacity (Peters plus syndrome)
Lenticular opacities anterior polar cataracts (Peters syndrome)
Keratolenticular adhesions (Peters plus syndrome)
Abnormality in fellow eye (bilateral condition)
Maxillary hypoplasia, teeth (hypodontia, microdontia)
This young patient has mesodermal dysgenesis.
Ill like to
Check IOP
Perform gonioscopy
Assess optic disc
Look at visual fields
Assess family members for similar condition
Clinical approach to aniridia

The most obvious abnormality is the absence of iris.
Look for
Corneal opacity, microcornea, sclerocornea
Limbal dermoid
Lenticular opacities
Keratolenticular adhesions
Check fellow eye (bilateral condition)
Nystagmus
This young patient has aniridia.
Ill like to
Check IOP
Perform gonioscopy
Check fundus (foveal and disc hypoplasia, choroidal coloboma)
Assess the family members for similar condition
If sporadic, refer to renal physician to exclude Wilms tumor
Exam tips:
Another name is iridocorneal dysgenesis. Do not confuse this with the
iridocorneal endothelial syndromes (ICE).
Aniridia is NOT part of the spectrum, but included in the table for comparison.
Remember that Wilms tumor is associated with AR type of aniridia.
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TOPIC 7
OPEN ANGLE GLAUCOMAS

Q Opening Question: What is Glaucoma?

Glaucoma is a specic type of optic neuropathy.
With characteristic optic disc changes and VF abnormalities.
The major risk factor is an increase in IOP.
Glaucoma can be classied into open and closed angle and also as primary and secondary.
Glaucoma
1. Denition Trabecular (pigment dispersion,
Optic neuropathy with characteristic optic disc pseudoexfoliation syndrome, neovascular
changes and VF abnormalities glaucoma)
Post-trabecular (raised episcleral venous
IOP is one of the risk factors
pressure)
2. Classication
Angle closure glaucoma (ACG):
Open angle glaucoma (OAG):
Primary (PACG)
Primary (POAG)
Secondary:
Secondary:
Posterior pushing forces (posterior
Paratrabecular (membrane)
synechiae, phacomorphic glaucoma)
Anterior pulling forces (peripheral anterior
synechiae, neovascular glaucoma)
Q What is the Pathogenesis of Primary Open Angle Glaucoma?
1. Genetics Abnormal blood coagulability

MYOC: Myocilin (GLC1A), associated with Nocturnal hypotension, signicant blood loss
juvenile open angle glaucoma and ~4% of 4. Neurodegenerative factors
adults with POAG Primary ON damage leads to release of
OPTN: Optineurin (GLC1E) glutamate, which interacts with cell receptors that
Other loci: GLC1B, GLC1C etc. leads to an increase in intracellular calcium levels
2. Pressure-dependent (mechanical) factors This triggers cell death via apoptosis and leads
Increased IOP compression and backward to further release of glutamate and a vicious
bowing of lamina cribrosa obstruction of cycle occurs
axoplasmic transport ganglion cell death
3. Ischemic factors (especially signicant for NTG)
Vascular perfusion compromise (DM, HPT,
migraine, Raynauds phenomenon)
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Q What is Steroid Response?
Steroid response is the change in IOP with steroid administration.

Steroid Response
1. Denition 2. Risk in IOP dependent on:
Based on 6-week course of topical Strength of steroids
betamethasone, there are three groups of Strong steroids (dexamethasone,
persons: betamethasone and prednisolone, etc.) more

High responders (> 30 mmHg):
likely to produce IOP rise than weak steroids
5% of population
(uorometholone etc.)
90% of POAG
Route of administration:
25% of POAG relatives
Systemic steroids less likely to produce IOP rise
Moderate responder (2230 mmHg): Duration, frequency and dose

35% of population 3. Mechanism
Low responder (21 mmHg or less): Decrease phagocytosis
60% of population
Interfere with transport in trabeculum
Decrease in prostaglandin activity
Q What are the Factors Which Inuence the Management of Open Angle Glaucoma?
The factors which will inuence the management of a patient with open angle glaucoma include.
Factors that Determine the Management
of Open Angle Glaucoma
1. Severity and progression of disease: Family history of blindness from glaucoma
IOP level (most important factor) Only eye or fellow eye blind from glaucoma
Optic nerve head changes Concomitant risk factors (DM, HPT, myopia,
Visual eld changes other vascular diseases)
Ocular risk factors (CRVO, Fuchs endothelial Compliance to follow-up and medication use
dystrophy, retinitis pigmentosa) Socioeconomic status (costs of drugs vs
2. Patient factors: surgery)
Age Resources Available to the Patient
Race (blacks higher rate of progression) Surgery for POAG in places which has no
resources for long term follow-up
Exam tips:
Similar to factors affecting management of cataract and glaucoma (page 25).
A very useful approach to many different glaucoma questions. For example,
the examiner may ask, How do you manage a 70-year-old man with
uncontrolled POAG in one eye and is blind in the other eye from advanced
POAG?
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What is the Relationship between IOP and Glaucoma? What are Ocular Hypertension and Normal
Q Tension Glaucoma (NTG)?
Ocular hypertension is dened as IOP > 95th percentile of the normal distribution in that population
(see below). NTG is dened as. (see below).
Spectrum of POAG, Ocular Hypertension
and NTG
IOP Optic disc VF Diagnosis Clinical approach

Increased Abnormal Abnormal Glaucoma (POAG)
Exclude POAG with diurnal IOP variation
Normal Abnormal Abnormal NTG Exclude optic neuropathy
Decide on treatment approach
Determine risk of POAG
Increased Normal Normal Ocular hypertension
Decide on treatment approach
Normal Normal Abnormal Glaucoma suspect Exclude either POAG or NTG
Normal Abnormal Normal Glaucoma suspect Exclude either POAG or NTG
Normal Normal Normal Normal
Q What is Ocular Hypertension (OHT)? How do You Manage OHT?
Ocular hypertension is dened as an IOP > 95th percentile of the normal distribution in that population.
The ON and VF are normal.
But the IOP is consistently > 21 mmHg.
The management has to be individualized.
I would discuss the management options with the patient: He can either be observed or glaucoma
treatment can be commenced.
I would be more inclined to start treatment if these risk factors for developing POAG are present: .
Ocular Hypertension
1. Natural history Family history of glaucoma (though not
VF loss about 2% per year signicant in OHTS)
Treatment decreases VF loss to 1% per year Myopia, migraine, hypertension were not found
However, mean years from initial VF loss to to be risk factors
death (12 years in whites, 16 years in blacks) DM was protective against development of POAG
Therefore elderly patient with OHT rarely 3. Management
becomes blind even without treatment! Establish baseline and follow-up optic disc
2. Risk factors for developing POAG (Ocular appearance (stereodisc photos, HRT)
Hypertension Treatment Study) Establish baseline and follow-up VF (to detect
Age of patient (older) progression and to improve patient reliability)
Larger vertical CDR Determine central corneal thickness (CCT)
Higher IOP Patients preference is an important factor in the
Thinner CCT (< 555 m) greater PSD on HVF management
Notes
Refer to the Collaborative Normal Tension Glaucoma Study (Am J
Ophthalmol 1998;126:487 497)
Multi-center randomized controlled trial to determine whether a substantial
drop in IOP would halt/slow the progression of NTG
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Q What is Normal Tension Glaucoma (NTG)? How do You Manage NTG?
Normal tension glaucoma is a common form of POAG.

In which the ON and VF changes are characteristic of POAG.
But the IOP is consistently < 21 mmHg.
The management of NTG is difcult and controversial.
It includes establishing the diagnosis and follow-up for progression of disease.
The ndings of the Collaborative Normal Tension Glaucoma Study (CNTGS) suggest that NTG is often
non-progressive. When VFD or optic nerve damage is progressive, medical and surgical treatment is indicated.
Some patients progress despite treatment, suggesting that factors other than IOP may play a more
important role in optic nerve damage.
Normal Tension Glaucoma
1. Clinical examination and diagnostic approach If there are reliable and progressive optic disc
Aim and VF changes, consider phasing:
Exclude other types of glaucoma: If raised IOP found, treat as POAG
POAG with diurnal variation? (daily changes) If normal IOP with wide diurnal uctuation
Intermittent ACG? found, treat as for NTG
Old secondary glaucoma? If normal IOP with no uctuation, exclude
Exclude optic neuropathy: optic nerve disease, and consider
Compressive optic neuropathy (consider neurological consultation and CT scan
neurological consultation and CT scan) If neurological consultation and CT scan are
Congenital disc anomalies normal, treat as for NTG
Anterior ischemic optic neuropathy
2. Treatment
Radiation and toxic optic neuropathy
No proven treatment
History Maintain IOP as low as possible (latanoprost
Risk factors for NTG:
and other new drugs):
Severe vascular compromise (shock, major
CNTGS found that decreasing IOP by 30%
accidents, major surgery)
reduced rate of VF progression from 35% to
Vascular diseases (DM, HPT, migraine,
12%
smoking, Raynauds disease)
Treat associated vascular risk factors (DM, HPT)
Family history of POAG
Intermittent ocular pain (intermittent ACG)
Systemic vasodilator and Calcium channel
Radiotherapy, TB treatment (optic neuropathy) blockers (nifedipine, namodipine, lisinopril)
Establish baseline and follow-up optic disc Neuroprotective agents (betoptic, brominidine,
appearance (stereodisc photos, HRT) akatinol/memantine)
Establish baseline and follow-up VF (to detect Trabeculectomy
Has been shown in some studies to preserve
progression and to improve patient reliability)
VF in NTG
Determine CCT to exclude articially low IOP
readings
Notes
What optic discs changes are more common in NTG compared with POAG?
Greater rim thinning
Peripapillary crescent more common
Splinter hemorrhage more common
Optic disc pallor more than cupping
Optic disc pits more common
Notes
What VF changes are more common in NTG compared to POAG?
VF loss closer to xation
Steeper slopes
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Q What are the Other Important Glaucoma Studies?
Early Manifest Advanced Glaucoma Collaborative Initial

Glaucoma Trial Intervention Study Glaucoma Treatment Study
Aim To compare the effects of To assess the long-term To compare long-term effect
immediate treatment outcome of intervention of treating newly diagnosed
(Betaxolol and ALT) vs sequences (TAT vs ATT) in POAG with standard
no/late treatment on the eyes that have failed initial medical treatment
progression of newly medical treatment for (meds ALT trab) vs
detected POAG glaucoma ltration surgery
To determine the extent (trab ALT meds)
of IOP reduction attained
by treatment and explore
factors that can inuence
glaucoma progression
Design Multicenter, randomized Multicenter, randomized Multicenter, randomized
controlled trial controlled trial controlled trial
Inclusion Newly diagnosed and POAG uncontrolled by Newly diagnosed and
untreated POAG medication untreated POAG
Endpoint Glaucoma progression Visual acuity, visual eld Visual acuity, visual eld,
(visual eld and optic disc) IOP, quality of life
Results 25% reduction in IOP Black patients tended to pre- Both groups had substantial
reduced progression from serve vision with ATT while and sustained reduction in
49% to 30% at 4 years whites did better with TAT IOP (surgical group had IOP
Eyes with IOP < 18 about 23 mmHg lower)
mmHg did not progress on No substantial difference
the visual elds over 6 years in VF or quality of life
between the 2 groups
Q What is the Relevance of Central Corneal Thickness to Glaucoma?
The Ocular Hypertension Treatment Study (OHTS) showed CCT to be a powerful predictor of the
development of glaucoma.
Eyes with CCT < 555 m had a threefold greater risk of developing glaucoma than those who had
CCT > 588 m.
1. Importance of CCT:
Thicker corneas cause falsely higher IOP Scanning slit topography (Orbscan): Non-contact,
readings, and thinner corneas cause falsely higher readings compared to pachymetry
lower IOP readings Specular microscopy: Provides pachymetric
Thin CCT may be an independent risk factor for measurements and specular microscopy
the development of glaucoma (evidence simultaneously
lacking) 3. Tonometry that reduces the inuence of the
2. Measurement of CCT: corneal biomechanical properties:
Ultrasound pachymetry: Contact required, Dynamic control tonometry (DCT)
accuracy dependent on technicians expertise Ocular response analyzer tonometry (ORA)
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TOPIC 8
ANGLE CLOSURE
GLAUCOMA
Q Opening Question: What is Primary Angle Closure Glaucoma? How do You Get Angle Closure?
Primary Angle Closure Glaucoma (PACG) is a specic type of glaucoma.
Aqueous outow is blocked as a result of closure of the angles.
The risk factors can be divided into patient and ocular factors.
Primary Angle Closure
1. Pathogenesis risk factors Relative anterior location of iris-lens
Patient factors: diaphragm
Risks increases with increasing lens
Age (increases with age)
Sex (females)
thickness, small corneal diameters and
Race (more common in orientals, Eskimos)
short axial lengths (hypermetropia)
Physiological
Ocular factors:
Relative pupil block:
Anatomical:
Mid-dilated pupil (semi-dark lighting)
Shallow AC
Autonomic neuropathy (loss of pupil
Narrow angle
hippus)
2. Stages
Stages Clinical presentation Treatment options

Primary Angle Closure Iridotrabecular contact of 180 Consider no treatment vs
Suspect degrees or more benet of Laser PI
Normal IOP, no peripheral anterior
synechiae (PAS)
Primary Angle Closure Iridotrabecular contact of 180 Laser PI for both eyes
degrees or more Medical treatment or surgery if
Raised IOP or PAS present IOP still raised
Primary Angle Closure Iridotrabecular contact of 180 Laser PI for both eyes
Glaucoma degrees or more Medical treatment or surgery if
Glaucomatous optic neuropathy IOP still raised
and visual eld loss
Acute PAC Acute presentation Acute management
Laser PI
Surgery might be required
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Exam tips:
The pathogenesis of PACG is usually not well answered. There must be a clear
and systematic plan.
Q What are the Mechanisms of Angle Closure?
Pupil block is the most common mechanism of angle closure.

However, mechanisms other than pupil block are now increasingly recognized.
Mechanisms of Angle Closure
1. Pupil block (most common) Iris and ciliary body cysts
2. Abnormalities anterior to iris Thick peripheral iris, peripheral iris roll
PAS 4. Abnormalities of lens
ICE syndrome: Abnormal endothelial cells Thick intumescent lens: Phacomorphic
Rubeotic glaucoma: Neovascular membranes glaucoma
3. Abnormalities of iris and ciliary body Subluxed lens
Plateau iris 5. Abnormalities posterior to lens
Malignant glaucoma
Notes
Causes of raised IOP post PI for angle closure:
Plateau iris
PAS
Steroid response
Non-patent PI
Malignant glaucoma
Subluxed lens
Q What is the Plateau Iris Syndrome?
Plateau Iris syndrome is a form of angle closure glaucoma.

There is no pupil block but the ciliary body is anteriorly positioned and the iris is inserted anteriorly.
With characteristic clinical features.
Plateau Iris Syndrome
1. Clinical features: Angle crowding (keyword)
Younger patient Indentation gonioscopy does not open angles
Less hypermetropic (may be myopic) (double hump sign)
AC normal depth, at iris plane 2. Treatment:
Gonioscopy Laser PI should still be performed (plateau iris
Iris inserted anteriorly (A or B under Spaeth syndrome is diagnosed only after PI)
classication, page 56) Laser iridoplasty
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Clinical approach to angle closure glaucoma

On examination, the AC is shallow.
Look for
Pigmented deposits on the endothelium of cornea, which is otherwise clear
Shallow AC, especially peripheral AC
Iris:
Widespread iris atrophy (spiral atrophy)
Patent laser PI at superonasal quadrant
Old laser iridoplasty scars
Posterior synechiae on pupil margin
Pupil may be dilated and fixed (sphincter ischemia)
Glaukomflecken (grayish opacities)
Trabeculectomy blebs
This patient has a previous attack of angle closure glaucoma.
Ill like to
Check IOP
Perform a gonioscopy
Assess optic disc
Look at VF
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TOPIC 9
SECONDARY GLAUCOMAS
Q Opening Question: What are the Causes of Neovascular Glaucoma?

Neovascular glaucoma is a secondary glaucoma.
Can be either open angle or closed angle.
The most common etiologies are diabetic retinopathy, CRVO and ocular ischemia.
Management is extremely difcult and prognosis is usually very poor.
Treat underlying condition and the glaucoma.
Etiology of Neovascular Glaucoma:
R etinopathy and Retinal vein occlusion E ales disease
(proliferative diabetic retinopathy, CRVO) O cular ischemic syndrome
R etinal detachment T rauma
U veitis I ntraocular tumors (choroidal melanoma)
B RVO C arotid cavernous stula
Clinical approach to neovascular glaucoma

On examination of the anterior segment, the most obvious abnormality is at
the iris.
New vessels are seen at the pupil border at 3 oclock.
Look for
Ciliary injection
Cornea clarity
AC activity (microscopic hyphema) and AC depth
Trabeculectomy/filtering shunt
Iris:
Peripheral anterior synechiae at limbus
Posterior synechiae on pupil margin
Pupillary distortion and ectropion uveae
Pupil may be fixed and dilated
Lens clarity (cataract)
This patient has Rubeosis iridis.
Ill like to
Check IOP
Perform gonioscopy (new vessels and peripheral anterior synechiae at the
angle)
(Continued )
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(Continued )
Do a fundus examination to look for proliferative DM retinopathy, CRVO,
RD, retinal tumors
Assess optic disc
Examine other eye
Ask for VA and whether patient has any pain (management purpose)
Exam tips:
A very good anterior segment examination case.
Remember the etiology by the mnemonic, "RUBEOTIC."
Q How would You Manage this Patient?
Management of this condition is difcult and prognosis is usually very poor.

Ill need to manage both the underlying disease and the neovascular glaucoma in both eyes.
This will depend on the patients visual potential and whether there is signicant pain.
In the case of good visual potential, Ill.
Management of Neovascular Glaucoma
1. Scenario 1: Good visual potential Cyclodestructive procedure as a last resort
Treat underlying condition (PRP for DM 2. Scenario 2: Poor visual potential
retinopathy and CRVO) Not in pain
Control IOP with medications Treat underlying condition
Consider early surgical ltering operation if IOP Symptomatic relief (steroids, timolol,
not controlled atropine)
Shunts In pain with high IOP
Modied trabeculectomy with MMC Control IOP with medication
Cyclodestructive procedure early
Q Tell me about Pigment Dispersion Syndrome and Pseudoexfoliation.
Pigment dispersion syndrome is a type of secondary open angle glaucoma.

Pseudoexfoliation syndrome is a type of secondary open angle glaucoma.
Pigment Dispersion Syndrome and Pseudoexfoliation Syndrome
Pigment dispersion syndrome Pseudoexfoliation syndrome

Demographics 3050 years (a decade younger) 60 years
Men Men and women
Related to myopia Related to aortic aneurysms
White race (abnormal basement membrane)
Scandinavian countries
Pathogenic Posterior bowing of iris Systemic disease of abnormal
mechanisms Constant rubbing of posterior basement membrane (skin,
pigment iris and zonules viscera, eyes)
Release of pigments Secretion of amyloid-like material
Trabecular block (oxytalon) in AC
Deposit in zonules and trabeculum
Trabecular block
(Continued)
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(Continued)
Pigment dispersion syndrome Pseudoexfoliation syndrome
Clinical features Krukenbergs spindle Pseudoexfoliative material,
Deep AC, with iris bowing dandruff-like appearance
posteriorly (reverse pupil block) throughout AC
Iris atrophy in periphery of iris Pupil difcult to dilate
Pigment deposit on lens Iris atrophy at edge of pupil margin
(Zentmayers line) Deposit on lens is characteristic
(target-like appearance, called
hoarfrost ring)
Lens subluxation (weak zonules)
Gonioscopy Heavily pigmented over entire Sampaolesis line (pigmented line
angle anterior to Schwalbes line)
Queer iris conguration Pseudoexfoliative material
Treatment Glaucoma risk: 10% at 5 years, Glaucoma risk: 1% per year (5%
15% at 15 years in 5 years, 15% in 15 years)
Bilateral disease: 90% Bilateral disease: 30%
Good prognosis Fair prognosis
Medical treatment same as POAG Medical treatment not very
Argon laser trabeculoplasty more effective
effective Argon laser trabeculoplasty more
Pilocarpine and laser PI may work effective in the short term
sometimes (reverse pupil block) Trabeculectomy same as POAG
Trabeculectomy same as POAG
Cataract surgery is particularly
difcult:
Weakened zonules
Small pupil
Raised IOP (risk of
suprachoroidal hemorrhage)
Q Tell me about Uveitic Glaucoma.
Uveitic glaucoma is a type of secondary glaucoma.

Management can be difcult and involves controlling both the inammation and the raised IOP.
Uveitic Glaucoma
1. Mechanisms of raised IOP NSAIDS: Topical, systemic

Angle closure with pupil block (seclusion Immunomodulators

pupillae) Lowering IOP
Angle closure without pupil block (PAS) Medication:
-blockers, agonists and carbonic
Open angle glaucoma 2
anhydrase inhibitors are rst-line agents
Steroid responders
Relatively contraindicated: Miotics,
Specic hypertensive uveitis syndromes:
prostaglandin analogues (though can be used
Fuchs heterochromic uveitis
cautiously in stable, well-controlled uveitis)
Posner-Schlossman syndrome
Hyperosmotic agents (e.g. mannitol):
2. Management Rapid reduction of IOP in acute settings
Controlling inammation: Surgery:
Steroids: Topical, periocular, intravitreal or When IOP is not well-controlled with
systemic medication
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Ensure inammation is well-controlled Glaucoma drainage devices: Increasingly

before surgery: Consider preoperative used for uveitic glaucoma
steroids Laser:
Higher risk of complications: Hypotony, Longer duration of steroids post-laser
inammation, malignant glaucoma, PI: When pupil block present
choroidal effusion TCP: Use with caution because high rates
Trabeculectomy: With antimetabolites of hypotony post-TCP

(5FU, MMC) No role for ALT
Q Tell me about Lens-Induced Glaucomas.
Lens-induced glaucomas are a group of common secondary glaucomas.

They are classied into.
Lens-Induced Glaucomas
1. Classication Lens particle glaucoma:
Phacomorphic: Secondary OAG
Secondary ACG Following cataract extraction, capsulotomy or
Intumescent lens causing pupil block trauma. Lens particles obstruct trabecular
Phacolytic: meshwork
Secondary OAG Lens subluxation and dislocation
Hypermature cataracts, leakage of lens 2. Management
proteins through an intact capsule Phacomorphic:
Phaco-antigenic: Manage acute attack like for acute PACG
Secondary OAG Avoid miotics
Autoimmune granulomatous reaction to If IOP is not controlled, carry out urgent
exposed lens proteins from a ruptured capsule cataract surgery
Notes
Possible questions:
How would you manage this patient if he had a symptomatic cataract?
How would you remove this patients cataract?
Clinical approach to trabeculectomy patients

This patient has had a trabeculectomy operation.
The bleb is at 10 oclock and is avascular, not inflamed.
Look for signs of
Post acute angle closure glaucoma:
Pigments on endothelium
Shallow AC
Spiral iris atrophy
Glauckomflecken
Laser PI
Pigment dispersion syndrome:
Krukenbergs spindle
Deep AC
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Peripheral iris atrophy

Posterior bowing of the iris
Pigmentary deposits on lens
Pseudoexfoliation syndrome:
Pigments on endothelium
Deep AC
Gray white dandruff-like deposits at the pupil border
Iris atrophy at pupil border
Hoarfrost ring
Subluxed lens
Ill like to
Check IOP
Perform gonioscopy (pigmentation, Sampaolesis line and pseudoexfoliation
material)
Assess optic disc
Perform VF
Phacolytic and phaco-antigenic glaucoma
Medical control of IOP
Semi-elective cataract surgery
Q What are the Effects of Intraocular Hemorrhage?
Intraocular Hemorrhage
1. Hyphema 3. Ghost cell glaucoma
Acute glaucoma (trabecular blockage) 4. Vitreous hemorrhage
Chronic glaucoma (trabecular damage) Synchysis scintillans
Corneal blood staining (hemosiderin) Tractional retinal detachment
2. Hemosiderosis bulbi 5. Expulsive suprachoroidal hemorrhage
Exam tips:
Remember that size affects rebleeding rate, both of which affect IOP levels,
and all three increase risk of corneal blood staining!
Therefore, surgical intervention is targeted specially at these complications.
Q How do You Manage a Patient with Hyphema?
Hyphema is commonly caused by blunt ocular injury, but may also occur under other circumstances.
The main management issues are.
Hyphema
1. Etiology Tumors
Trauma (blunt, penetrating) Clotting disorders (sickle cell, anticoagulant
Spontaneous: treatment, blood dyscrasias)
Vascular abnormalities (rubeosis and its 2. Clinical classication
causes, page 68) Microscopic
Grade I (< 1/3 AC volume)
B1037_Ch-02.indd 85 12/22/2010 4:07:41 PM

Grade II (1/34/5 AC volume) Hyphemas that remain unresolved for 9 days

Grade III ( > 1/2 AC volume) (to prevent PAS)
Grade IV (total) Patient factors:
Risk of glaucoma damage (elderly, glaucoma
3. Problems and complications
patient, vascular diseases)
Rebleeding:
Sickle cell anemia
Dependent on size of hyphema
Risk of corneal blood staining and amblyopia
Grade I hyphema (25% will rebleed)
(children)
Grade III hyphema (75% will rebleed)
Increased IOP 5. Management
Dependent on size and rebleeding Conservative:
Corneal blood staining Bed rest, head elevation
Dependent on size, IOP and rebleeding Topical steroids and cycloplegic agents
Others:
4. Indications for surgical treatment
Topical glaucoma medication
Ocular factors (Ann Ophthalmol 1975;7:
Aminocaproic acid
659662): Tranexamic acid
Corneal blood staining
Avoid aspirin
Total hyphemas with IOP > 50 mmHg for
Types of surgical treatment:
5 days (to prevent optic nerve damage)
AC paracentesis and washout
Hyphemas that are initially total and do not
Clot expression and limbal delivery
resolve below 50% at 6 days with IOP
Automated hyphectomy
25 mmHg (to prevent corneal blood staining)
Q Tell me about Angle Recession Glaucoma.
Angle recession glaucoma is a complication of blunt ocular trauma.

The main management issues are.
Angle Recession Glaucoma
1. Denition: 2. Risk of glaucoma:
Angle recession: Rupture of anterior surface of More than 50% of patients with gross traumatic
ciliary body, extending between longitudinal hyphema have angle recession but only 10%
and oblique/circular bers develop glaucoma
Cyclodialysis: Disinsertion of longitudinal bers Risk of glaucoma depends on extent of
of ciliary body from scleral spur recession (risk signicant if > 180)
Iridodialysis: Rupture of iris diaphragm at iris Mechanism: Trabecular damage (not the
base from ciliary body recession itself)
Exam tips:
Know the difference between angle recession, iridodialysis and cyclodialysis.
Q Tell me about Aphakic Glaucoma.
Aphakic glaucoma is a difcult glaucoma to manage.

Aphakic Glaucoma
1. Mechanisms Vitreal-trabeculectomy contact (secondary
Irido-vitreal/pupillo-vitreal block (secondary OCG)
ACG) Vitreal-peripheral iridectomy block
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2. Prevention 3. Treatment
Two or more peripheral iridectomies during Miose pupils with pilocarpine
surgery Decrease production (diamox, mannitol)
Extensive anterior vitrectomy during surgery High risk trabeculectomy
Clinical approach to iridocorneal endothelial (ICE) syndromes

On examination of the anterior segment of this middle aged lady.
There are diffuse areas of iris atrophy seen.
Look for
Corectopia, ectropion uvea, peripheral anterior synechiae
Iris nevus (Cogan-Reeses syndrome)
Corneal edema (Chandlers syndrome)
Lenticular opacities
Trabeculectomy blebs
Symptoms in fellow eye (should be normal)
This patient has iridocorneal endothelial syndrome.
Ill like to
Check IOP
Perform gonioscopy
Assess optic disc
Look at VF
Exam tips:
Remember ICE as consisting of Iris nevus, Chandlers syndrome (with Corneal
involvement) and Essential iris atrophy!
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TOPIC 10
MEDICAL TREATMENT
OF GLAUCOMA
Q Opening Question: What is the Ideal Drug for Glaucoma?

The ideal drug carries certain characteristics.
Ideal Drug
1. Effective (in lowering IOP) 3. Minimal side effects
2. Active on multiple fronts (decrease production, 4. Convenient dosage regiments
increase outow, neuroprotective) 5. Relatively inexpensive
Exam tips:
This is a good approach to most What is the ideal steroid for uveitis? or
What is the ideal antibiotic for endophthalmitis?
Q What are the Current Drugs Available for Treatment of Glaucoma?
Current drugs available can be divided into their effectiveness in lowering IOP.
Effectiveness in lowering IOP Examples

Class I (30% reduction in IOP) Beta blockers
Latanoprost
Alpha 2 agonist (brimonidine)
Unoprostone
Class II (20%) Pilocarpine
Dorzolamide
Alpha agonist (apraclonidine)
Beta 1 blockers (betoptic)
Class III (10%) Propine
Other older alpha agonists
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Exam tips:
One of the most important pharmacological questions in the examinations.
Classify according to IOP effect, mode of action (difcult) or traditional
vs new drugs.
Q What are the Traditional Drugs for Treatment of Glaucoma?
Traditional Drugs
Drug Pharmacodynamics Effectiveness/Advantages Side effects

Beta blockers Decrease aqueous Class I prototype Mild local side effects
(timolol, betaxolol, production 30% drop in IOP in (decrease corneal
carteolol, meti- Twice daily dosage 8090% of patients (e.g. sensation, allergic
pranolol) (T1/2 = 12 hours 24 to 16 mmHg) reaction, cicatricial
Concentration: 0.25 Good compliance conjunctivitis)
and/or to 0.5%) Additive effects with Severe systemic side
pilocarpine but not with effects (pulmonary
sympathetic agents bronchospasm, brady-
Cheap cardia, hypoglycemia)
Common systemic side
effects (lethargy,
decreased libido,
depression)
Miotics Increase aqueous Class II prototype Miosis (impairment of
(pilocarpine) drainage (miosis with 20% drop in IOP night vision)
opening of angle and Additive effects with Myopia and headache
contraction of longitudi- beta blockers and (spasm of accommoda-
nal bers of ciliary body) sympathetic agents tion from circular
Works only if sphincter Cheap muscle contraction)
pupillae is not ischemic Retinal detachment
Four times daily dosage (longitudinal muscle
Concentration: 116% contraction)
Uveitis (increased
permeability for
blood-aqueous barrier)
Angle closure glaucoma
Sympathetic Decrease aqueous Class III prototype Allergic conjunctivitis
agents (adrenaline production (alpha 2 10% drop in IOP (20% in one year, 50%
and propine) effect) Additive with pilo- in 5 years)
Increase aqueous carpine but not with Angle closure glaucoma
drainage (beta 2 effect) beta blockers Adrenachrome deposi-
Twice daily dosage Cheap tion
Concentration: 0.5%, Aphakic cystoid macular
1%, 2% (adrenaline) edema
Concentration: 0.1% Risk factor for
(propine) trabeculectomy failure
(Continued)
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(Continued)
Carbonic anhydrase Decrease aqueous Effect independent of Tingling of ngers and
inhibitors (acetazola- production (inhibit IOP levels toes
mide) carbonic anhydrase) Useful for short term Renal (metabolic
Oral/IV treatment acidosis, hypokalemia
Concentration: and renal stones)
250 mg/500 mg Gastrointestinal
symptoms
Stevens-Johnson
syndrome
Malaise, fatigue, weight
loss
Bone marrow suppres-
sion (aplastic anemia)
Contraindications:
Sulphur allergy
Kidney or liver disease
Sickle cell anemia
Renal stones
G6PD
Pregnancy or lactation
Hyperosmotic agents Dehydrates vitreous Rapid onset: Peak action Cardiovascular overload
(glycerol, mannitol, Oral/IV within 30 minutes for (use with caution when
isosorbide) Concentration: mannitol cardiovascular or renal
Glycerol and disease present)
isosorbide 1g/kg Urinary retention
Mannitol 20% 1g/kg Nausea
Headache
Backache
Alteration of mental
state, confusion
Exam tips:
The side effects of adrenaline can be remembered by A.
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B1037_Ch-02.indd 92
92
FA
Q What are the New Drugs for Treatment of Glaucoma?
The Ophthalmology Examinations Review
New Drugs
B1037 The Ophthalmology Examinations Review

Latanoprost PGF2 alpha agonist Better or as effective as Little systemic SE (T1/2 in plasma = 7 s):
(Xalatan) Increase uveoscleral timolol (depending on which study) occasional headache/URTI symptoms
Travoprost outow Class I drug. 30% drop in IOP in 8090% Conjunctival injection (10% will complain of
(Travatan) Once nightly dosage of patients (e.g. 24 to redness, 30% objective injection)
Bimatoprost (T1/2 = 12 hours) 16 mmHg) Inammation (contraindicated in uveitis)
(Lumigan) Concentration: 0.005% IOP effect at night Hypertrichosis (increase in length, number and
Good compliance thickness)
Additive effects with other Iris pigmentation (melanin deposition, no melano-
medications cytic hyperplasia, therefore no risk of melanoma)
Effective for 2 years with no drift Cystoid macular edema (pseudophakics/
aphakics)
Expensive
Brimonidine Alpha 2 agonist three effects: Class I drug Allergic blepharoconjunctivitis (10%)
(Alphagan) 1. Decreases aqueous Alpha 2 selectivity aqueous production Corneal irritation (10%)
production suppression (without vasoactivity effects of Dry mouth (10%)
Apraclonidine 2. Increase uveoscleral alpha 1)
(Iopidine) outow Less side effects compared with older
3. Neuroprotective non-specic alpha agonists (apraclonidine)
Twice daily dosage 1. Tachyphylaxis (30%)
Concentration: 0.2% 2. Chemosis and stinging (30%)
Rapid onset (30 min) Additive effects with other medications
(Continued)
12/22/2010 4:07:43 PM
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B1037 The Ophthalmology Examinations Review

(Continued)

Dorzolamide Topical carbonic anhydrase Class II drug Injection and stinging (30%)
(Trusopt) inhibitor Less side effects compared with oral Less effective than timolol
Brinzolamide Only 1/3 as effective as oral Corneal opacication in compromised corneas
(Azopt) Three times daily dosage (inhibits endothelial pump function)
Concentration: 0.2% Less systemic SE compared to
acetazolamide
Section 2: Glaucoma and Glaucoma Surgery
Unoprostone PGF2 alpha metabolite agonist: Class I drug Similar to latanoprost

(Rescula) 1. Increase conventional As effective as timolol
outow May also increase optic nerve head
2. Increase uveoscleral outow perfusion
Twice daily dosage
Concentration: 0.12%
FA
12/22/2010 4:07:43 PM
93
Combination Eyedrops:
1. Cosopt: Timolol + dorzolamide 4. Combigan: Timolol + alphagan
2. Xalacom: Timolol + latanoprost 5. Duotrav: Timolol + travoprost
3. TimPilo: Timolol + pilocarpine
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TOPIC 11
LASER THERAPY
FOR GLAUCOMA
Q Opening Question: What are the Uses of Lasers in Glaucoma?

Lasers can be used for diagnostic and therapeutic purposes.
Therapeutic use can be divided anatomically into.
Diagnostic
1. Confocal scanning laser ophthalmoscope (optic 2. Laser retinal doppler owmetry (optic nerve head
nerve head evaluation) perfusion)
Therapeutic
Anatomical Procedure Type of

site name laser Indications Notes
Iris Peripheral Nd:YAG or PACG Settings:
iridotomy (PI) sequential Narrow, occludable Argon (1.1 W, 0.05 s, 50 m)
Argon-YAG angles followed by Nd:YAG
Secondary ACG (23 mL)
(phacomorphic, uveitic) Lens:
Abrahams syndrome or
Wises syndrome
Laser iridoplasty Argon Medically Laser 1 ring around iris
unresponsive PACG (stretches angles and
Angle crowding dilates pupil to relieve
Plateau iris pupil block)
Laser PI block
Prior to ALT in POAG
with narrow angles
Laser pupilloplasty Argon As in laser iridoplasty Laser 3 rings around
pupils (dilates pupil to
relieve pupil block)
(Continued)
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(Continued)

Angles Laser Argon Temporizing procedure that Settings:
trabeculoplasty tends to fail in the long term. Argon (0.2 W, 0.1 s, 50 m)
(ALT) Less effective than medica- Extent: 180 or 360o
tions and surgery in VF Number of shots:
preservation 40
Medically unresponsive
POAG 1/3 of patients do not
Pigment dispersion and respond at all
pseudoexfoliation Average decrease in
Elderly patient not t for IOP ~ 30% (replaces
surgery one eyedrop)
Selective laser Does not cause structural/ Settings:
trabeculoplasty (SLT) coagulative damage to TM 400 m, 0.61.2 mJ, 3 ns
unlike ALT Extent and number of shots:
4555 spots over 180o

Laser trabeculo- Argon Neovascular glaucoma Laser new vessels at iris
coagulation
Ciliary Ciliary body ablation Diode Refractory glaucomas Why not cryotherapy?
body 1. Transscleral (1.82 W) Neovascular Advantages of laser,
cyclophotocoa- Continuous wave Uveitic lower risk of:
gulation (TCP) YAG Traumatic Phthisis bulbi
2. Transpupillary (89 W) Failed trabeculec- Sympathetic
cyclophotocoa- tomy ophthalmia
gulation Congenital Chemosis and pain
3. Endoscopic
cyclophotocoa-
gulation
Sclera Laser sclerostomy Holium YAG POAG Makes 300 m hole in
sclera
Little collateral damage
because using picosec-
onds pulses
High incidence of failure
Laser suture lysis Argon Post trabeculectomy Settings:
(Useful 13 weeks Argon (0.2 W, 0.1 s,
after trabeculectomy 50 m)
to improve ltration) Lens: Hoskins
Vitreous YAG capsulotomy YAG Malignant glaucoma Settings:
for malignant YAG (22.5 mJ, 1 pulse
glaucoma per burst)
Lens:
Capsulotomy lens
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Exam tips:
One of the more commonly asked lasers and procedures questions in
examinations. Develop notes based on your own technique.
Q How do You Perform Cyclodestruction Using Laser?
I would use a diode laser to perform a transscleral cyclophotocoagulation (TCP).

Diode TCP
1. Procedure: 2. Post-procedure:
Retrobulbar anesthesia Analgesics
Contact ber-optic probe Steroids
Settings: Check IOP 3 weeks later
1.8 to 2 W 3. Complications:
12 s
Pain
3040 shots
Hypotony, phthisis bulbi
Extent: 360 13 mm from limbus, sparing 3
Inammation, iritis
and 9 oclock areas
Scleral thinning
Hear pop sound (microablation of ciliary
Decrease in VA
body epithelium)
Hyphema
Malignant glaucoma
Q When do You Perform a Laser Peripheral Iridotomy (PI)?
The laser peripheral iridotomy is indicated for therapeutic and prophylactic purposes.
Indications for a Laser Peripheral Iridotomy
1. Therapeutic: Secondary ACG (irido-IOL block, irido vitreal
PACG (acute ACG, intermittent ACG, chronic block, subluxed lens with pupil block)
ACG) 2. Prophylactic:
POAG with narrow angles Fellow eye of patient with PACG
Narrow occludable angles
Q How do You Perform a Laser PI?
I would perform a Nd:YAG laser PI as follows.

or I would perform a sequential Argon YAG laser PI as follows.
Procedure for Laser Peripheral Iridotomy
1. Prepare the patient: 4. Location of PI:
Miosed pupil with 2% pilocarpine Upper nasal iris (to avoid diplopia and macular
Instil 1% apraclonidine 1 hour before procedure burn)
Topical anesthetic and position patient at laser 1/3 distance from limbus to pupil
machine Iris crypt if possible
2. Argon blue green laser settings: 1.1 W, 0.05 s, Apply 2030 burns until iris is penetrated
50 m 5. Signs of penetration:
Plomb of iris pigments
3. Abrahams iridotomy lens
Deepening of AC
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Retroilluminate to see patent PI 7. Post-procedure:

Gonioscopy to see opened angles Instil 1% apraclonidine
6. Nd:YAG laser setting: 2.5 mJ, 35 shots: Check IOP 1 hour later
PI size should ideally be 300500 m Topical steroids for 1 day
Notes
What are the unique features of Abrahams iridotomy lens?
Contact lens with +66D lenticule
Stabilizes globe during procedure
High magnication
Increases cone angle and energy at site by 4x:
Therefore, the spot area is effectively reduced 4 and radius reduced 2
(square root of 4) (i.e. 50 m spot size is reduced to 25 m)
In addition, the energy around the cornea and iris reduced by 4
Q What are the Complications of a Laser PI?
Complications
1. Contiguous damage: 3. Malignant glaucoma
Cornea 4. Monocular diplopia
Cataract
2. Iris:
Iris bleeding
Iritis
Increased IOP
Exam tips:
Remember MIC.
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TOPIC 12
SURGICAL TREATMENT
FOR GLAUCOMA
Q Opening Question: What are the Indications for Trabeculectomy in Glaucoma?

There are no absolute indications for trabeculectomy.
In general.
Common scenarios include.
Indications
1. Treatment should be individualized with no xed Noncompliance with medical treatment
rule Additional considerations:
Young patient with good quality of
2. General principle: When IOP is raised to a level vision
where there is evidence of progressive VF or ON One-eyed patient (other eye blind from
changes which will threaten quality of visual glaucoma)

Family history of blindness from
function, despite adequate medical treatment
glaucoma
3. Common scenarios include: Glaucoma risk factors (HPT, DM)
Uncontrolled POAG with maximal medical Uncontrolled PACG after laser PI and
treatment: medical treatment
Failure of medical treatment (IOP not Secondary OAG or ACG
controlled with progressive VF or ON
damage)
Side effects of medical treatment
Q How does Medical Therapy Compare with Surgical Therapy in Glaucoma?
It is difcult to compare medical with surgical treatment, with new research showing both having
advantages and disadvantages. We can compare the two in four major areas.
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Medical treatment Surgical treatment

Effectiveness 40% respond readily and 510% poor response
consistently to low dose medicine to medical treatment
50% eventually require in rst instance and
complex medical regimen, require surgery
adjuvant ALT and Improved surgical
ltration surgery technique has led to
8090% success rates
Better control of IOP (delay
VF/ON progression)
Increase morbidity
associated with delaying
surgery until evidence of
VF/ON damage
Cost Cheaper initially, but accumulates over years Actual cost may be less
In the United States, cost of in the long term
bilateral surgery = cost of 8 years
of topical medication
Safety/Problems Poor compliance with multiple Even after surgery, may
medications require adjuvant medical
Less control of IOP with treatment
continuing ON damage No long term proof that
Minor side effects is troublesome good IOP control alone
Major side effects can occur: will stop ON damage
Aplastic anemia (with diamox) (IOP only one risk factor)
Respiratory and cardiac side Usually no minor side
effects (beta blockers) effects
Increase risk of bleb failure (with Major side effects (com-
chronic topical eyedrop use) mon):
Anesthetic and surgical
morbidity
Risk of endophthalmitis
and malignant glaucoma
Shallow AC, hypotony,
progression of cataracts
Quality of life Poorer quality of life (with use of Better quality of life
multiple eyedrops)
Exam tips:
As expected, this topic will be a constant debate with new research ndings
every month. Stick to a conservative approach but keep an open mind about
new ideas.
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Q How do You Perform a Trabeculectomy?
I would perform a trabeculectomy as follows.

Trabeculectomy
1. Preparation: Dissection with crescent blade until surgical
Retrobulbar anesthesia limbus is seen (page 41)
Inferior corneal traction suture with 7/0 silk Where is the surgical limbus?
2. Conjunctival ap: 4. Paracentesis performed at distant location

Superonasally or superotemporally 5. Sclerostomy:
Fornix or limbal based ap (stick to one Enter AC through scleral ap with a beaver blade
approach, see below) Excise 2 1 mm block of sclera with Kellys
Dissect Tenons with Wescott scissors punch or Vanna scissor
Remove all episcleral tissue 6. Peripheral iridectomy:
3. Scleral ap: Prevent blockage of sclerectomy site by iris
Outline ap with diathermy 7. Closure:
Size 4 x 3 mm Scleral ap sutured with 8/0 vicryl or 10/0 nylon
Cut with beaver blade 1/2 to 2/3 scleral Reform AC and check aqueous egress
thickness Conjunctiva sutured with 8/0 vicryl
Exam tips:
As in cataract surgery, be concise but accurate with the steps, as if you had
done the procedure a hundred times. Say, I will perform a superotemporal
limbal-based conjunctival ap instead of conjunctival ap.
Notes
Why perform a paracentesis?
Decompress AC prior to sclerectomy
Reform AC later
Check aqueous egress later
Q What are the Advantages and Disadvantages of Fornix-Based vs Limbal-Based Flaps?
Fornix-based vs Limbal-based Conjunctival Flap
Fornix-based Limbal-based
Advantages Faster to create and close Easier to excise Tenons
Good exposure Less risk of wound leak and at AC
Easier to identify limbal landmarks No limbal irregularity (dellen)
Less dissection (less bleeding Allows adjunctive use of anti-
and risk of button hole) metabolites with less corneal
Avoids posterior conjunctival scarring toxicity
(limits posterior ltration of aqueous)
Disadvantages Increase risk of at AC Slower and more surgical experience
Harder to excise Tenons needed
IOP control not as good as with Poorer exposure
limbal-based ap Risk of button hole higher
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Q What are the Complications of a Trabeculectomy?
The complications can be divided into intraoperative, early postoperative and late postoperative.
Complications Hyphema
1. Intraoperative (not common, usually due to poor Suprachoroidal hemorrhage
surgical techniques): Wipe-out syndrome
Suprachoroidal hemorrhage (most important Cystoid macular edema
complication, as in cataract surgery) 3. Late postoperative:
Button hole in conjunctival ap Filtration failure (see below)
Subconjunctival hemorrhage from bridle suture Endophthalmitis, blebitis
Hyphema Cataract progression
2. Early postoperative: VF loss progression
Flat AC and malignant glaucoma (see below) Refractive errors
Endophthalmitis Hypotony
Q How do You Manage a Shallow AC after a Trabeculectomy?
Management involves an assessment of the severity of shallowing and the etiology.

This depends on the IOP and presence/absence of a bleb.
Shallow AC
1. Grades of shallow AC: Grade II: Pupillo-corneal touch
Grade I: Irido-corneal touch (can afford to be Grade III: Lenticulo-corneal touch (need to
conservative) intervene surgically)
2. Etiology
IOP Bleb Differential diagnoses Management

High No bleb Malignant glaucoma See below
Siedals sign +ve Suprachoroidal Hb Fundus examination (dark brown mass)
Pupil block glaucoma Dilate pupil (AC may deepen)
Enlarge surgical PI with laser
Low No bleb +ve Wound leak Conservative:
Siedals sign +ve Usually will resolve within 24 hours
Decrease steroids and increase antibiotics
(gentamicin) to induce scarring
Dilate pupil with mydriatic (atropine)
Decrease aqueous production (timolol and
diamox)
Pressure pad/bolster
Simmons shell/oversized (ex) contact lens
Surgical:
Resuture
Good bleb Excessive ltration Conservative:
Siedals sign ve Decrease steroids, increase antibiotics,
dilate pupil and decrease aqueous produc-
tion
Pressure pad/bolster
Inject gas (air or SF6) or viscoelastic into AC
Injection of autologous blood into bleb
Compression mattress suture
Surgical:
Resuture
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Q How do You Manage a Malignant Glaucoma?
Malignant glaucoma is a serious complication of glaucoma surgery.

Management involves an assessment of the severity (grades of AC shallowing).
And can be conservative or surgical.
Malignant Glaucoma
1. Conservative: 2. Surgical:
Topical mydriatics (atropine) Chandlers procedure (see vitreous tap, page 33):
Lower IOP (diamox and osmotic agents) 19G needle inserted into vitreal cavity, about
Enlarge PI 12 mm from tip to drain 11.5 ml of aqueous

Nd:YAG laser to disrupt anterior vitreous face and separate solid vitreous from trapped
aqueous
(see laser therapy, page 78)
Vitrectomy
Q How do You Manage a Filtration Failure?
Management involves an evaluation of the causes of failure.

And can be conservative or surgical.
Filtration Failure
1. Etiology Digital massage
Early: Laser suture lysis:
Blockage by ocular components (lens, iris, Done usually at 13 weeks after surgery (see
Descemets membrane, vitreous, scleral laser therapy, page 78)
remnants) Needling of bleb:
Blockage by surgical intervention (blood, Done usually at 6 weeks after surgery
viscoelastic) Topical anesthetics under sterile conditions
Late: Approach from unoperated conjunctiva with
Subconjunctival brosis 27G needle
2. Conservative May be combined with 5 FU injection
Increase topical steroids 3. Surgical

Medical control of IOP Revision of trabeculectomy/new trabeculectomy
Scleral depression at posterior lip of scleral ap with antimetabolites
Notes
Risk factors for subconjunctival brosis = indications for antimetabolite
used
Q What are the Indications for Using Antimetabolites in Trabeculectomy?
Antimetabolites are used in trabeculectomy when a high risk of failure with the conventional operation
is anticipated.
This is related to either patient or ocular factors.
Indications for Antimetabolites
1. Patient factors: Previous failed trabeculectomy
Young (< 40 years) Previous conjunctival surgery (e.g. pterygium
Black race surgery)
Previous chronic medical therapy (especially 2. Ocular factors:
with Adrenaline) Secondary glaucomas (neovascular and uveitic
glaucoma)
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Traumatic glaucomas Congenital/pediatric glaucomas

Aphakic/pseudophakic glaucomas Conjunctival scarring
Iridocorneal endothelial syndromes (ICE)
Notes
What additional measures must be taken in trabeculectomies with
antimetabolites?
Prevent antimetabolites from entering eye:
Limbal-based aps
Watertight wound (interrupted non-absorbable conjunctival sutures)
Careful dissection to prevent button hole formation
Q Tell me about Antimetabolites Used in Glaucoma Surgery.
5 Fluorouracil (5 FU) Mitomycin C (MMC)

Pharmacology Fluorinated pyrimidine analogue Natural antibiotic compound/alkylating
Binds intracellular thymidylate agent
synthetase (inhibits thymidine and Cross-links with DNA strands by
DNA synthesis) formation of covalent bonds
Affects only cells in mitotic phase of Affects cells in all phases
cell cycle Kills broblast permanently and stop
In the eye, inhibits broblast brosis
proliferation and delays brosis
Dosage Intraoperative dose: 2550 mg/ml Intraoperative dose: 0.20.4 mg/ml
Postoperative drops: 5 mg/ml for 1 week
Results Improves success rate of ltration No randomized trial results
operation Better than 5 FU?
Complications Corneal epithelial toxicity Prolonged hypotony
Hyphema Avascular bleb
Wound leak Endothelial, ciliary body and retinal damage
Infection Hyphema
Wound leak
Infection
Exam tips:
Refer to three-year follow-up of the Fluorouracil Filtering Surgery Study (Am
J Ophthalmol 1993;115:8292).
Q What is the Fluorouracil Filtering Surgery Study?
The Fluorouracil Filtering Surgery Study is a multicenter prospective randomized clinical trial which
evaluates the effectiveness of adjuvant subconjunctival 5 FU following trabeculectomy.
1. Inclusion criteria
213 patients considered to be at a higher risk of cataract extraction or previous failed ltering
surgical failure on the basis of having previous surgery in a phakic eye
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2. Treatment 4. Results
Trabeculectomy with adjuvant postop Treatment failure at 3 years: 51% with 5 FU
subconjunctival 5 FU injections vs vs 74% without 5 FU
trabeculectomy alone Complications associated with 5 FU:
3. Outcome Corneal epithelial erosions
Early wound leaks
Treatment failure dened as:
Reoperation to decrease IOP, or 5. Conclusion
An IOP > 21 mmHg with or without adjuvant 5 FU indicated after trabeculectomy only in
IOP-lowering medications patients at high risk of failure
Q Tell me about Filtering Shunts.
Filtering shunts are communications between AC and sub Tenons space.

They are indicated when a high risk of failure with the conventional operation is anticipated.
This is related to either patient or ocular factors.
The shunts can be divided into.
The complications include.
Filtering Shunts
1. Indications Postoperative:
Patient factors: Functional (excessive drainage, blockage of
Previous failed trabeculectomy tube by blood and uveal tissue)
Previous major anterior segment surgery Mechanical (corneal endothelial
Ocular factors: decompensation, cataract)
Secondary glaucomas (neovascular and Diplopia due to extraocular muscle limitation
uveitic glaucoma) Bleb encapsulation over footplate poor
Traumatic glaucomas drainage
Aphakic/pseudophakic glaucomas Late endophthalmitis
Iridocorneal endothelial syndromes (ICE) 4. Efcacy compared to trabeculectomy
Pediatric glaucomas Tube vs Trabeculectomy Study (Am J
2. Type of shunts Ophthalmol 2009;148:670684)
Vary with shape and size Tube shunt surgery had a higher success rate
Valves (Krupin-Denver, Ahmed) vs no valves compared to trabeculectomy with MMC during
(Molteno, Baerveldt) rst 3 years of follow-up
Material: PMMA, silicon, polypropylene Both procedures associated with similar IOP
3. Complications reduction and use of supplemental medical
Intraoperative: therapy at 3 years
Hyphema Postoperative complications higher following
Lens damage and cataract trabeculectomy with MMC relative to tube
Globe perforation shunt surgery, but most complications were
Muscle disinsertion and laceration transient and self-limited
Exam tips:
The indications are nearly IDENTICAL to that of antimetabolite use.
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Section 3
Corneal and
External Eye
Diseases
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TOPIC 1
THE CORNEA
Q Opening Question: What is the Anatomy of the Cornea?

The cornea is a transparent structure in the anterior segment of eye .
Anatomy of the Cornea
1. Gross anatomy Ends abruptly at limbus
General dimensions: Deep layers appear to merge into stroma
11.5 mm horizontal diameter Stroma (substantial propia):
10.5 mm vertical diameter 90% of cornea thickness, 400 m centrally
1 mm thick periphery 80% water
0.5 mm thick centrally Glycosaminoglycans in extracellular matrix
Anterior surface radius 7.7 mm Three major fractions:
Posterior surface radius 6.8 mm Keratan sulphate (50%)
Chondroitin phosphate (25%)

2. Microscopic anatomy
Chondroitin sulphate (25%)
Five layers:
Epithelium:
Descemets membrane:
Basement membrane of the endothelium
Stratied squamous, nonkeratinized,
nonsecretory epithelium (keyword) (keyword)

10 m thick
56 layers deep
Secreted and regenerated by endothelial cells
Supercial cells have microvilli (needs
Type IV collagen brils
tears to keep cornea smooth)
Hassall-Henle bodies
Basement membrane strongly attached
Terminates abruptly at limbus (Schwalbes
to Bowmans layer
line)
Bowmans layer:
8-12 m
Endothelium:
Single layer, polygonal, cuboidal cells
Acellular
Tight junctions (control of corneal hydration)
Consists of interwoven type IV collagen brils
Microvilli
which are anterior condensation of substantia
Incapable of regeneration: Repair occurs by
propia
Incapable of regeneration, replaced by
cellular hypertrophy and sliding
Line passages of trabecular meshwork
brous tissue if damaged (i.e. scars)
Exam tips:
One of the most common basic science questions in life viva and MCQ
examinations.
Section 3: Corneal and External Eye Diseases 109
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Q What is the Function of the Cornea?
Physiology of Cornea
1. Three main functions: Glucose:
Light transmission (400700 nm light) Cornea obtains glucose mainly from aqueous
Light refraction: Tears and limbal capillaries appear to provide
Total refractive power of cornea 43 D (70% minimal contribution
of eyes refractive power) Glucose can be stored in epithelium as
Refractive index of cornea 1.376 glycogen
Protection ATP obtained through glycolysis and Kreb's
cycle
2. Corneal metabolism:
Oxygen:
Energy needed for maintenance of transparency
Endothelium acquires oxygen from aqueous
and dehydration Epithelium acquires oxygen from either
capillaries at the limbus or precorneal lm
Q Why is the Cornea Transparent?
Corneal transparency is due to a combination of factors including .

Cornea Transparency
Relative dehydration of cornea due to: Normal intraocular pressure (if too high,
Anatomic integrity of endothelium and relative hydration occurs)
epithelium Relative acellularity, lack of blood vessels and
Endothelial pump removes uids from stroma
pigments
Evaporation of water from tear increases
Regular matrix structure of corneal brils:
osmolarity of tear, which draws water from
Destructive interference of light occurs
cornea
Consistent refractive index of all layers
Exam tips:
Variations to questions include What are the factors which keep the cornea
dehydrated?
Q What is the Nerve Supply of the Cornea?
The cornea is innervated by the V CN.

Nerve Supply
V CN Subepithelial plexus just below Bowmans
Ophthalmic division membrane
Long posterior ciliary nerves gives off: Intraepithelial plexus
Annular plexus at limbus
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TOPIC 2
CONGENITAL CORNEAL
ABNORMALITIES
Q Opening Question: What are the Congenital Abnormalities of the Cornea?

Megalocornea
1. Corneal diameter > 13 mm (or 12 mm at birth): Facial hemiatrophy
Buphthalmos must rst be excluded (no axial Dwarsm
myopia, no cornea opacity, normal IOP)
Distinction between simple megalocornea Microcornea
(large cornea without structural abnormalities 1. Corneal diameter < 10 mm
and associated ocular malformations) and 2. May occur as:
anterior megalophthalmos (large cornea with Isolated cornea abnormality
structural abnormalities/ocular malformations) Nanophthalmos (small but normal eye)
2. Inheritance: AD (simple megalocornea) or SLR Microphthalmos (small and abnormal eye)
(anterior megalophthalmos) 3. Inheritance: AD, AR, sporadic
3. Clinical features (anterior megalophthalmos): 4. Ocular associations:
Congenital Shallow AC
Males (90%) Glaucoma
Bilateral (80%), symmetrical, nonprogressive Hyperopia
Normal cornea Persistent hyperplastic primary vitreous
Normal thickness and endothelial cell density Congenital cataract
No Descemets rupture (i.e. no Haabs straie) Anterior segment dysgenesis
Normal posterior segment Optic nerve hypoplasia
Normal visual development 5. Systemic associations:
4. Ocular associations (anterior megalophthalmos): Dwarsm
Astigmatism Achondroplasia
Atrophy of iris stroma Myotonic dystrophy
Ectopic lentis and cataract Fetal alcohol syndrome
Glaucoma (but not congenital glaucoma!)
Cornea Plana
5. Systemic associations (anterior
1. Flat cornea:
megalophthalmos):
Radius of curvature < 43D (may be 2030D)
Downs
Pathognomonic when corneal curvature is the
Connective tissue diseases (Marfans syndrome,
same as adjacent sclera!
Ehlers-Danlos syndrome)
Craniosynostosis (Aperts syndrome) 2. Inheritance: AD, AR, sporadic
Alports syndrome
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3. Bilateral, peripheral opacication of cornea 3. May be AD/sporadic

4. Ocular associations: 4. Classication:
Sclerocornea Isolated sclerocornea: No other abnormalities
Microcornea Sclerocornea plana: With at cornea
Congenital cataract (mean K < 38D)
Glaucoma Peripheral sclerocornea with anterior chamber
cleavage abnormalities: Peters, Riegers
Sclerocornea anomalies
1. Diffuse scarring and vascularization of cornea Total sclerocornea
2. Epithelium thickened, Bowmans membrane
absent
Q Tell me about Goldenhars Syndrome.
Clinical Features
1. Ocular features: 2. Systemic features:
Megalocornea Wide mouth
Coloboma of iris and lids Maxillary and mandibular hypoplasia
Squint, Duanes syndrome Preauricular tags and hearing loss
Fundus optic nerve hypoplasia, coloboma Vertebral defects
Refractive errors
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TOPIC 3
CHEMICAL INJURY
Q Opening Question: What are the Complications of Chemical Injuries?

Chemical injuries are ocular emergency.
They can be mild or potentially blinding.
Complications of Chemical Injury
1. Acute problems: Cornea:
Corneal abrasion and perforation Persistent epithelial defect
Infection Limbal stem cell failure and persistent ocular
Glaucoma surface disease
Stromal scar
2. Long-term problems:
Intraocular complications:
Ocular surface:
Glaucoma
Trichiasis, distichiasis, entropion
Diffuse trabecular damage iritis
Cicatricial conjunctivitis, dry eyes,
Cataract
symblepharon, ankyloblepharon
Classication of Chemical Injury (Hughs Classication)
Grade Signs Prognosis

1 Corneal epithelial damage Excellent
No limbal ischemia
2 Corneal hazy but iris details seen Good
Ischemia < 1/3 of limbus
3 Corneal hazy but iris details hazy Fair
Ischemia < 1/2 of limbus
4 Opaque cornea Poor
Ischemia > 1/2 of limbus
Updated classication (ref Dua H et al, Br J Ophthal- Area of bulbar and forniceal conjunctival
mol 85(11):1379 staining: Conjunctiva important in ocular
Updated grading scheme with prognostic value surface stabilization when limbus severely
6 grades compromised
Main criteria for classication:
Extent of limbal staining (vs ischemia):
Staining of greater value in predicting
epithelial recovery
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Exam tips:
One of the few true ocular emergencies.
Need to know difference between acidic and alkaline injuries.
Notes:
What are the possible mechanisms of glaucoma?
Acute shrinkage of collagen
Uveitis, trabeculitis
Lens-induced inammation
Steroid response
Q How do You Manage a Patient with Severe Chemical Injury?
Chemical injury is an ocular emergency .

Management of Chemical Injury
1. Acute management Lid closure (taping, pressure pad, tarsorraphy)
Irrigate eyes immediately copious amounts of Tissue glue
sterile uid (normal saline preferred): Conjunctival ap
Remove particulate matter Lysis of conjunctival adhesions (glass rods)
Debride devitalize tissues 3. Long-term management
Start antimicrobial treatment Ocular surface reconstruction:
Start steroids immediately (to decrease Lid surgery (cicatricial entropion, trichiasis
mediators of inammation and stabilize and distichiasis)
lysosome in white blood cells) Conjunctival replacement (AMT or cultivated
Minimize steroids after 10 days (because conjunctiva)
steroids decrease broblast and collagen Ocular surface surgery
synthesis) Principles:
Alternatively, medroxyprogesterone can be Removal of dysplastic epithelium
used (no anti-anabolic effects) Wide excision of broblastic
undergrowth
2. Manage epithelial defect
Judicious diathermy
Conservative:
Epithelial replacement (autografts/
Tear substitutes and lubricants
allografts/cultivated transplants)
Vitamin C (antioxidant, cofactor in collagen
Cornea:
synthesis)
Keratoplasty (limbal, lamellar, penetrating)
Ascorbate or citrate (antioxidant, cofactor in
collagen synthesis) Intraocular:
Antiglaucoma treatment
N acetylcysteine (collagenase inhibitor,
Cataract surgery
contributes to cross-linkages and maturation
of collagen) Controversial:
Sodium EDTA (collagenase inhibitor Retinoic acid (promote surface keratinization)
calcium chelator, calcium required for Fibronectin (growth factor)
collagenase activity) Epidemal growth factor
Bandage contact lens Subconjuctival heparin (to facilitate limbal
Surgical: reperfusion)
Punctal occlusion in severe dry eyes
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Q What are the Other Causes of Cicatricial Conjunctivitis?
Cicatricial conjunctivitis can be divided into.

Cicatricial Conjunctivitis
1. Infectious: Vernal/atopic keratoconjunctivitis
Adenovirus Dermatological:
Herpes simplex Ocular rosacea
Trachoma Scleroderma
Corynebacterium diphtheriae Neoplasia:

Beta hemolytic streptococcus Squamous cell carcinoma, Bowens disease
Trauma:
2. Noninfectious:
Mechanical, chemical injury
Autoimmune:
Others:
Ocular cicatricial pemphigoid
Long-term timolol use
Steven-Johnson syndrome
B1037_Ch-03.indd 115 12/22/2010 5:44:43 PM

TOPIC 4
CORNEAL OPACITY,
SCARRING AND EDEMA
Q Opening Question: What are the Causes of Corneal Scarring?

Corneal scarring can be divided into the location of the scarring .
Corneal Scarring
1. Superior cornea: 3. Inferior cornea:
Superior limbic keratoconjunctivitis Neurotrophic keratopathy
Trachoma Exposure keratopathy
Vernal keratoconjunctivitis Marginal keratitis
2. Central cornea: 4. Diffuse scarring:
Disciform keratitis Interstitial keratitis
Keratoconus (hydrops) Trauma
Fuchs endothelial cell dystrophy Ocular surface diseases (Stevens-Johnson
Bullous keratopathy syndrome, ocular cicatricial pemphigoid)
Lipid keratopathy Trachoma
Band keratopathy
Clinical approach to a superior corneal scar

This patient has stromal scarring seen at the superior half of the cornea .
Look for
Trachoma:
Trichiasis, entropion of upper lid
Herberts pits
Evert upper lid (Arlts line)
Vernal keratoconjunctivitis:
Punctate epitheliopathy, macroerosions, shield ulcers, plaque,
subepithelial scar
Trantas dots
Pseudogerontoxon (cupids bow)
Evert upper lid (giant papillae)
Superior limbic keratoconjunctivitis:
Superior conjunctival injection
Punctate epitheliopathy, corneal filaments
B1037_Ch-03.indd 117 12/22/2010 5:44:43 PM

Clinical approach to central corneal scar or edema

This patient has a central corneal stromal scar/edema.
The visual axis is involved.
Look for
Disciform keratitis:
Lid scarring (usually very subtle)
Epithelial edema
Descemets folds
Wessley ring
Keratic precipitates
AC activity
Keratoconus:
Parastromal thinning
Vogts straie
Fleischers ring
Prominent corneal nerves
Fuchs endothelial cell dystrophy:
Epithelial edema
Subepithelial scarring
Stromal thickening
Corneal guttata
Pseudophakic bullous keratopathy:
Epithelial bullae
IOL
Ill like to
Check corneal sensation (disciform keratitis)
Check IOP (Fuchs endothelial dystrophy, disciform keratitis)
Q How do You Manage a Patient with Bullous Keratopathy?
Management of bullous keratopathy depends on the etiology, severity and visual potential
and whether patient has symptoms of pain.
In mild cases, conservative treatment is usually adequate .
In severe cases, if the visual potential is good .
On the other hand, if the visual potential is poor and the eye is painful .
Bullous Keratopathy
1. Etiology: Therapeutic contact lens
Pseudophakic bullous keratopathy Hair-dryer
Fuchs endothelial cell dystrophy 3. Surgical treatment:
End-stage glaucoma If good visual potential, consider optical
Long-standing inammation keratoplasty
Chemical burns If poor visual potential and eye is painful,
2. Conservative treatment: consider palliative procedures for pain relief:
Lubricants Tarsorrhaphy, botox to lids
Hypertonic saline Conjunctival ap (see page 139)
Lower intraocular pressure Retrobulbar alcohol
Enucleation (very last resort)
Avoid steroids
B1037_Ch-03.indd 118 12/22/2010 5:44:44 PM

Exam tips:
Very similar approach to management of neovascular glaucoma! (see page
69).
See also management of Fuchs endothelial dystrophy (page 113) and
glaucoma and cataract (page 25).
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TOPIC 5
CORNEAL ULCERS
Q Opening Question No. 1: How do You Manage a Patient with a Corneal Ulcer?
Corneal ulcer is a potentially blinding condition which needs immediate ophthalmic management.
Management of Corneal Ulcer
1. Admit patient if necessary 4. Intensive topical antibiotic treatment:
2. Identify predisposing factors: Gutt. gentamicin 15 mg/ml hourly (or gutt
Contact lens wear tobramycin)
Ocular trauma Gutt. cephazolin 50 mg/ml hourly (or gutt
Ocular surface disease cefuroxime)
Systemic immunosuppression 5. Systemic antibiotic treatment if:
3. Perform a corneal scrape for microbiological Ulcer near limbus (scleral extension)
analyses Perforated ulcer (endophthalmitis)
Exam tips:
Prepare specic antibiotic regimes with exact dosage and frequency of
treatment gentamicin frequently does not sound as impressive as I would
prescribe topical gentamicin 15 mg/ml hourly for the next 24 hours.
Q When will You Consider Using Monotherapy with Antibiotics?
Cautious in using monotherapy Culture positive

Broad spectrum antibiotic (e.g. gutt. Organism is NOT Pseudomonas or
ciprooxacin) Streptococcus
Indications: Organism sensitive to antibiotics
Small, peripheral ulcer
Patient follow-up and compliance good
Q When will You Consider Using Steroids?
Use of steroids is controversial, extreme caution Sensitive to antibiotics

needed Responding clinically
Use only after adequate antimicrobial treatment Ulcer has been sterilized
Indications: Patient follow-up and compliance good
Culture positive
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Q What do You do When the Ulcer is not Responding to Treatment?
Stop antibiotics for 24 hours Specimen divided and sent for microbio-
Rescrape and/or corneal biopsy logical analyses and histological
Steps in corneal biopsy: staining
LA or GA Re-start intensive antibiotics
Corneal tissue on standby (for tectonic Consider other diagnosis (e.g. sterile ulcers?)
replacement) Consider penetrating keratoplasty
Dermatological trephine with biopsy
area encompassing base and active edge

of ulcer
Q What are the Causes of Sterile Ulcers?
Sterile Ulcers
1. Post infection (treated, resolved) Lagophthalmos
Herpes (metaherpetic ulcer) Proptosis
Bacterial 5. Nutritional keratitis (Vitamin A deciency)
Fungal 6. Neoplasia (acute leukemia)
2. Nearby (contiguous) ocular surface inammation 7. Immune-mediated
Lids and lashes (entropian, ectropian, trichiasis, Connective tissue diseases:
lid defects) Rheumatoid arthritis
Skin (Stevens-Johnson syndrome, ocular Wegeners granulomatosis
pemphigoid, ocular rosacea) Systemic lupus erythematosis
Lacrimal gland (keratoconjunctivitis sicca) Polyarteritis nodosa
3. Neurotrophic keratitis Moorens, Terriens
DM Marginal keratitis
V CN palsy Allergic conjunctivitis
Herpes zoster 8. Iatrogenic/trauma
4. Exposure keratitis Postsurgical, topical eyedrops
VII CN palsy Chemical, thermal, radiation injury
Exam tips:
Remember Non Infected ulcers.
Q Opening Question No. 2: Tell me about Fungal Keratitis

Fungal keratitis is a potentially blinding condition which needs immediate ophthalmic management.
Fungal Keratitis
1. Types of fungi: Yeasts (unicellular, no hyphae)
Filamentous fungi (multicellular, hyphae Candida, Cryptococcus
present): Dimorphic (lamentous at 25 and yeasts at 37):

Septate (most common cause of fungal Blastomyces, Coccidioides (orbital infections,
keratitis) rarely affect cornea)
Monilial (Fusarium, Aspergillus, 2. Predisposing factors:
Penicillium) Ocular trauma (lamentous), particularly with
Dermatiaceous (Curvularia)
organic matter (e.g. vegetation, soil)
Nonseptate (cause orbital infections) Ocular surface disease and systemic
Mucor, Rhizopus
immunosuppression (yeasts)
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3. Clinical features: 4. Stains:

Grayish white ulcer Indian ink
Elevated Gram stain
Indistinct borders, feathery edges Giemsa
Satellite lesions Periodic acid shift
Ring inltrate Methanamine silver
Endothelial plaque (may be pigmented)
Exam tips:
Another common variation is, Tell me about fungal keratitis.
Q How do You Treat Fungal Keratitis?
Fungal keratitis is a potentially blinding condition which needs immediate ophthalmic treatment.
It is difcult to treat, requires multiple drugs, long duration of treatment and may involve surgery.
Treatment
1. All medication work by interfering with 3. Imidazoles
ergosterol metabolism (keyword) Act by interfering with CYP450 mediated
2. Polyenes pathways in ergosterol synthesis
Amphotericin B: Miconazole, uconazole, ketoconazole
Forms complex with ergosterol that Voriconazole:
destabilizes the fungal wall Relatively new agent with good oral
Good for yeasts bioavailability (96%) and ocular penetration

Epithelial debridement may improve Effective in cases that have failed therapy
penetration (highly lipophilic) with other agents
Unstable, rapid degradation to light Toxicity: Transient visual disturbances, liver,
Systemic toxicity: Renal, anemia, fever renal

Natamycin: 4. Flucytosine
Mechanism of action similar to Converted to 5 Fluorouracil
amphotericin B Adjunct treatment
Good for lamentous fungi
Q Opening Question No. 3: What are the Characteristics of Acanthamoeba Keratitis?

Acanthamoeba keratitis is a potentially blinding condition which needs immediate treatment.
Acanthamoeba Keratitis
1. Microbiology Pain (severe and disproportionate to lesion)
Protozoan: (keyword)
Active trophozoite form Multifocal inltrates and microabscess
Dormant cystic form Ring inltrate (keyword)
Highly resistant to hostile environment
Keratoneuritis (keyword)
(e.g. chlorinated water) Complication: Scleritis, secondary bacterial
2. Predisposing factors: keratitis
Contact lens wear 4. Stains:
Ocular trauma Giemsa
3. Clinical features: Calcouor white
In early cases, mimics herpetic epithelial Acridine orange
keratitis 5. Culture (nonnutrient agar with E. coli lawns)
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Q How do You Treat Acanthamoeba Keratitis?
Acanthamoeba keratitis is a potentially blinding condition which needs immediate treatment.

It is difcult to treat, needs multiple drugs, long duration of treatment and may involve surgery.
Treatment:
1. Aminoglycosides: 3. Diamidines (disrupts cell membrane):
Neomycin (but not gentamicin) Propamidine isethionate (brolene)
2. Biguanides (disrupts DNA): Hexamidine
Polyhexamethylene biguanide (PHMB) 4. Imidazoles:
Chlorhexidine Econazole
Exam tips:
Notice the answer to this question is identical to the answer to the question
on fungal keratitis treatment.
The drugs are difcult to remember. A simple mnemonic is ABCDE.
B1037_Ch-03.indd 124 12/22/2010 5:44:45 PM

TOPIC 6
HERPETIC EYE DISEASES

Q Opening Question: What is the Difference between the Ocular Manifestations of Herpes
Simplex vs Herpes Zoster?
Herpes simplex is caused by the virus herpes simplex virus type 1.
Herpes zoster is caused by the virus zoster varicella virus.
The different manifestations can be divided into .
Herpes simplex Herpes zoster

Age pattern Primary < 5 years Elderly
Recurrent Middle ages Immunosuppressed
Skin manifestations:
1. Dermatome Incomplete Complete
2. Bilaterality Rarely Never
3. Pain Less More
4. Postherpetic neuralgia Rare Common
5. Skin scarring Rare Common
Ocular manifestations
1. Dendritic keratitis Central Peripheral
Large Small
Well-dened dendrite Broad, stellate-shaped
Central ulceration (stains with Raised, plaque-like (stains with
Fluorescein) Rose Bengal)
Terminal bulbs No terminal bulbs
2. Spectrum 1. Blepharoconjunctivitis: Each stage has skin, ocular and neural
Follicular complications
Cicatricial A) Acute herpes zoster
2. Epithelial disease: 1. Episcleritis/scleritis
Dendritic ulcer 2. Conjunctivitis
Geographic ulcer 3. Keratitis:
Marginal ulcer Punctate epithelial keratitis
Neurotrophic/metaherpetic Microdendrite
ulcer Nummular keratitis
(Continued)
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(Continued)
Herpes simplex Herpes zoster

3. Stromal keratitis: Disciform keratitis
Necrotizing keratitis 4. Anterior uveitis
Interstitial (immune) keratitis 5. Acute retinal necrosis
4. Endothelitis: B) Chronic herpes zoster
Disciform 1. Mucous secreting conjunctivits
Diffuse 2. Keratitis:
Linear Nummular keratitis
5. Corneal complications: Disciform neurotrophic
Pannus, stromal vascularization Neutrophic and exposure
and scarring keratitis
Trophic keratitis Mucous plaque
Lipid keratopathy
6. Acute uveitis
7. Episcleritis / Scleritis
8. Acute retinal necrosis
Q What are the Results of the Herpetic Eye Disease Study?
1. Three components: To assess effectiveness of: antivirals effective in uveitis (Ophthalmol

Topical steroids in stromal keratitis in patients 1996; 114: 1065)
on topical antivirals safe and effective in 2. Additional trial:
stromal keratitis (Ophthalmol 1994; 101: 1871) Oral Acyclovir (400 mg bd for 1 year after
Oral Acyclovir (400 mg 5x/day) in stromal resolution of ocular HSV) in preventing
keratitis in patients on topical steroids and recurrence of HSV decreased rate of
topical antivirals no benet in stromal recurrence of ocular HSV (all disease types),
keratitis (Ophthalmol 1994; 101; 1871) especially important after resolution of stromal
Oral Acyclovir (400 mg 5x/day) in uveitis in keratitis (New Engl J Med 1998; 339: 306)
patients on topical steroids and topical
Q What are the Causes of Iris Atrophy?

The causes of iris atrophy include .
Causes of Iris Atrophy
1. Iatrogenic (postoperative) Iridocorneal endothelial syndromes (scattered
atrophy with corectopia, pseudopolycoria)
2. Injury to iris
Pigment disperson syndrome (atrophy at
3. Inammation:
periphery of iris)
Herpes simplex (sectoral atrophy), herpes zoster
Pseudoexfoliation syndrome (atrophy at pupil
Fuchs uveitis, Posner Schlosmann syndrome
border)
4. Increased IOP (glaucoma):
5. Ischemia:
Post angle closure glaucoma (spiral atrophy)
Anterior segment ischemia
B1037_Ch-03.indd 126 12/22/2010 5:44:46 PM

Exam tips:
Be careful, this clinical sign can be easily missed.
The causes can be remembered by Iris atrophy.
Q What are the Causes of Corneal Hypoesthesia?
Corneal hypoesthesia can be physiological or pathological.

Corneal Hypoesthesia
1. Physiological: Acquired:
Increasing age Diabetes mellitus
Peripheral cornea Leprosy
In the morning Herpes simplex
Brown eyes Iatrogenic:

Topical eyedrops (timolol, atropine, sulphur
2. Pathological
drugs)
Congenital: Surgery (limbal section ECCE, penetrating
Riley Day syndrome
keratoplasty, epikeratophakia)
Congenital corneal hypoesthesia
Contact lens wear
Corneal dystrophies (Reis Buckler dystrophy,
lattice dystrophy)
B1037_Ch-03.indd 127 12/22/2010 5:44:46 PM

TOPIC 7
PERIPHERAL ULCERATIVE
KERATITIS
Q Opening Question: What are the Causes of Peripheral Ulcerative Keratitis?

Causes of Peripheral Ulcerative Keratitis
1. Systemic 2. Ocular
Connective tissue diseases: Infective:
Rheumatoid arthritis (RA) Bacterial, viral, acanthamoeba, fungi
Systemic lupus erythematosus Noninfective:
Wegeners granulomatosis Moorens ulcer
Polyarteritis nodosa (PAN) Terriens marginal degeneration
Relapsing polychondritis Marginal keratitis
Sarcoidosis Pellucid marginal degeneration
Leukemia Acne rosacea
Exposure keratopathy
Neurotrophic keratopathy
Trauma
Exam tips:
Peripheral ulcerative keratitis (or PUK) differential diagnoses can be classied
either as systemic and ocular or infective and noninfective.
PUK is a limbal-based disease with inammatory changes in the limbus;
therefore, it is more immune-related than infective.
See also Connective tissue diseases and the eye (page 34).
Clinical approach to peripheral ulcerative keratitis

The most obvious lesion in this patient is peripheral corneal thinning seen
at the interpalpebral region.
Look for
Moorens ulcer:
Overhanging central edge of ulcer
Stromal white infiltrate central edge of ulcer
Epithelial defect
Cataract
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Terriens marginal degeneration:

Gradual outer slope and central steep slope (but not overhanging)
Intact epithelium
Gray white demarcation line central edge of thinning
Other eye:
Unilateral (Moorens ulcer)
Bilateral (Terriens, connective tissue diseases)
Scleral involvement (important sign):
Scleritis (connective tissue diseases)
No scleral involvement (Moorens ulcer, Terriens marginal degeneration)
Exclude:
Blepharitis (marginal keratitis)
Skin hyperemia, telangiectasia, papule, nodules, rhinophyma (rosacea)
Systemic features:
Hands (RA)
Malar rash (systemic lupus erythematosus)
Ill like to
Examine fundus for evidence of vasculitis, optic neuropathy (connective
tissue diseases)
Examine patient for systemic signs (connective tissue diseases)
Q How would You Manage a Patient with PUK?
I would like to investigate the specic etiology of the PUK and manage accordingly.
Management of PUK
1. Investigation C-ANCA
Ocular: RF
Scrapings for culture and sensitivity CXR
Systemic: Mantoux test
CBC, ESR 2. Treatment
VDRL, FTA Systemic steroids
ANA, dsDNA Immunosuppressives
Q How do you Differentiate Terriens Marginal Degeneration from Moorens Ulcer?
Terriens Marginal Degeneration vs Moorens Ulcer
Terriens marginal degeneration Moorens ulcer

1. Early onset: 1. Two forms:
Males (75%) Early onset: Progressive, bilateral
Bilateral Later onset: Limited, unilateral
2. Symptoms: 2. Symptoms:
Little pain and redness Severe pain and redness
3. Clinical features: 3. Clinical features:
Starts at superior and Starts at interpalpebral
inferior quadrant cornea
Epithelium intact Epithelial defect
(Continued)
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(Continued)
Terriens marginal degeneration Moorens ulcer
Sloping inner edge of ulcer with Overhanging inner edge
lipid line and bridging vessels of ulcer
No clear interval Clear interval between lumbus
and ulcer
Low risk of perforation Risk of perforation
Eye not inamed Eye is inamed
Otherwise eye is normal Cataract, glaucoma may be
present
Q How do You Manage a Patient with Moorens Ulcer?
The management of Moorens ulcer depends on the severity of disease.

And involves both medical and surgical treatment.
Stepwise Treatment Approach for
Moorens Ulcer
Step 1: Topical steroids Step 3: Conjunctival excision/recession
Step 2: Oral steroids and immunosuppressives Step 4: Tectonic Lamellar keratoplasty/
penetrating keratoplasty
Q Tell me about Acne Rosacea.
Acne rosacea is a skin disease of idiopathic origin.

It commonly occurs in middle-age women.
It has both skin and ocular manifestations.
Acne Rosacea
1. Skin involvement: Conjunctiva:
Persistent erythema Papillary conjunctivitis
Telangiectasia Cornea:
Papules, pustules Punctate epithelial keratitis
Hypertrophy of sebeceous glands Stromal keratitis, peripheral thinning,
Rhinophyma vascularization
Subepithelial opacication
2. Ocular involvement: Ulceration, scarring and melting
Blepharitis almost always develops at some time
Severe lesions occur in region of 3% Treatment
20% eyes involved rst Oral tetracycline:
50% skin involved rst Effective for both skin and ocular lesions
25% simultaneous skin and eye involvement Basis of therapeutic response unknown, not
Eyelids: related to antibacterial effect on Staph aurevs
Recurrent blepharitis Ampicillin and Erythromycin also found to be
Meibomitis effective
Styes, Chalazoins Possible to taper and stop therapy but
recurrence is high (50%)
Also given topically
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TOPIC 8
INTERSTITIAL KERATITIS
Q Opening Question: What is Interstitial Keratitis?

Interstitial keratitis is a nonsuppurative, chronic inammation of the stroma.
Without primary involvement of the epithelium or endothelium.
The common causes include .
Causes of Interstitial Keratitis
1. Infective Onchocerciasis
Congenital (or acquired) syphilis Lyme disease
TB 2. Noninfective
Leprosy Cogans disease (associated with polyarteritis
Herpes nodosa)
Sarcoidosis
Clinical approach to interstitial keratitis

On examination of the anterior segment .
There is midstromal corneal opacity.
Involving the visual axis.
There are ghost vessels seen within the lesion.
Look for
Mutton fat keratic precipitates (TB, syphilis, leprosy, sarcoid)
AC activity
Lens opacity
Fellow eye bilateral (congenital syphilis)
Ill like to
Check corneal sensation (herpes, leprosy)
Check fundus for: Optic atrophy, salt and pepper retinopathy (syphilis)
Ask for history of deafness, tinnitis, vertigo (Cogans dystrophy)
Systemic examination for rheumatic conditions
Investigate for cause:
CBC, ESR
CXR
Mantoux test
VDRL, FTA
Connective tissue screen (polyarteritis nodosa)
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TOPIC 9
CORNEAL DYSTROPHY
Q Opening Question: What are Corneal Dystrophies? How are They Different from Corneal
Degenerations
Corneal dystrophies are a group of inherited, noninammatory corneal conditions characterized by .

Corneal degenerations are a group of sporadic, age-related corneal conditions characterized by .
Dystrophy Degeneration
Inherited, noninammatory condition of cornea Sporadic, age-related condition of cornea
1. Inherited, AD (1) 1. Sporadic
2. Early onset 2. Late onset
3. Nonprogressive/slowly progressive 3. Progressive
4. Clinical features: 4. Clinical features:
Bilateral Unilateral or bilateral
Symmetrical Asymmetrical if bilateral
Axial, do not extend to periphery (2) Peripheral
One layer of cornea Different corneal layers
Otherwise eye is normal Other age-related changes present
Except for Macular (AR)

Except for Macular and Meesmanns Dystrophy
(Extends to periphery)
Q What are the Pathological Features of Epithelial Corneal Dystrophies?
Epithelial dystrophies affect the epithelium, basement membrane (BM) and Bowmans
membrane of the cornea.
Microcystic (map-dot-ngerprint) Reis-Buckler Meesmanns

Inheritance AD (Incomplete penetrance) AD AD
Pathology Abnormal epithelial cells Granular deposis in BM Periodic acid shift positive
with microcysts that stain with Massons substance deposited in
Thickened BM trichrome BM
Duplication of BM
Fibrillar material deposited between
BM and Bowmans membrane
(Continued)
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(Continued )
Microcystic (map-dot-ngerprint) Reis-Buckler Meesmanns
Clinical features Recurrent corneal erosion RCE Photophobia
(RCE) in 10%; most Honeycomb appearance Tiny epithelial cysts
asymptomatic Corneal hypoesthesia extend to periphery
Lesions look like dots, cysts, lines,
ngerprint, maps
Treatment Conservative One of earliest to require Conservative
Treat RCE PKP
Highest risk of recurrence
after PKP
Q What are the Pathological Features of Stromal Corneal Dystrophies?
Stromal corneal dystrophies affect the stroma of the cornea.

There are three classical types .
Lattice Granular Macular

Inheritance AD AD AR
Pathology Amyloid material Hyaline material Mucopolysaccharides
Stains: Stains Masson trichrome Stains Alcian blue
Congo red
PAS positive
Birefringent
Dichroism
Crystal violet
metachromasia
Clinical RCE RCE Decreased VA
features Linear, branch-like Bread-like crumbs Gray opaque spots
pattern Intervening stroma clear Stroma diffusely cloudy
Intervening stroma clear Peripheral stroma clear Peripheral stroma involved
Peripheral stroma clear
Notes Type 2: Type 2 Occurs in much younger
Patients older Patients older patients compared with
VA better VA better the other two stromal
Systemic amyloidosis Larger ring-shaped dystrophies
associated lesions
Less numerous lines
Lines more peripheral
PKP rarely needed
Treatment Treat RCE PKP needed early PKP needed very early
PKP by 40 years Highest recurrence rate
of stromal dystrophies
Exam tips:
Common clinical and viva examination
Remember the mneumonic, Marilyn Monroe Always Gets Her Man in L A
City = Macular Mucopolysaccharides Alcian Granular Hyaline Masson
Lattice Amyloid Congo
B1037_Ch-03.indd 136 12/22/2010 5:44:48 PM

Q What are the Features of Amyloidosis?
Amyloid is an eosinophilic hyaline substance with some characteristic staining characteristics.

The manifestations can be classied as .
Amyloidosis
1. Staining characteristics Most common form of ocular amyloidosis
Congo red positive Conjunctival involvement
Birefringent and dichroism Lattice dystrophy
Crystal violet metachromasia Primary systemic amyloidosis
Fluorescence in ultraviolet light with thioavin Secondary localized amyloidosis:
T stain Long standing ocular inammation
Typical lamentous structure on electron e.g. trachoma, interstitial keratitis

microscopy Secondary systemic amyloidosis:
Long standing chronic systemic diseases
2. Classication e.g. RA, leprosy
Primary localized amyloidosis:
Q What is Crystalline Dystrophy of Schnyder?
Crystalline dystrophy of Schnyder is a stromal dystrophy associated with abnormal cholesterol

metabolism.
The clinical features include .
Crystalline Dystrophy of Schnyder
AD Stromal haze
Localized abnormality in cholesterol Associated with corneal arcus and Vogts limbal
metabolism girdle
Minute crystals in stroma Associated with hypercholesterolemia in 50%
Stain: Oil Red O
Q What are the Pathological Features of Endothelial Dystrophies?
Endothelial dystrophies affect the Descemets membrane and endothelium of the cornea.
There are three classical types .
Fuchs endothelial Posterior Polymorphous Congenital Hereditary

dystrophy dystrophy (PPMD) Endothelial dystrophy (CHED)
Inheritance AD AD or AR AR
Pathology Abnormal deposition Focal thickening of
of collagen material in Descemets membrane
Descemets membrane Multilayering of endothelium
(pseudoepithelium)
Clinical features Middle aged women At birth or young Endothelium not visible
4 signs: Polymorphous picture Stroma diffusely thickened
Corneal guttata Vesicles, geographical or and opacied
Stromal edema band-like opacities on
Bowmans membrane Descemets membrane
scarring (tram-tracks)
Epithelial May be associated with
edema/bullous glaucoma (progressive
keratopathy synechial angle closure)
and Alports syndrome
Treatment See below PKP in about 10% PKP needed very early
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Q How do You Manage a Patient with Fuchs Endothelial Dystrophy and Cataract?
The management of Fuchs endothelial dystrophy with cataract can be a difcult decision.
There are two clinical problems which must be managed simultaneously, depending on the severity of
each condition.
Factors to consider include patient and ocular factors.
Management of Fuchs Endothelial Dystrophy
1. Patient factors consider surgery early if Severity of cornea decompensation:
Young age History of blurring of vision in morning
High visual requirements Greater than 10% difference in corneal
Poor vision in fellow eye thickness between early morning and
measurements later in the day
2. Ocular factors
Severity of edema on clinical examination
Severity of cataract Pachymetry > 650 m corneal thickness
Endothelial cell count < 800 cells/mm2
Severity of corneal
decompensation Severity of cataract Possible options
+ 0 Conservative treatment (lubricants, hypertonic saline, lower IOP,
soft bandage contact lens)
+++ +++ Combined cataract extraction and PKP (triple procedure)
+++ 0 PKP rst
Cataract extraction later, after development of cataract
+++ + Triple procedure indicated
Alternatively PKP rst, cataract extraction later but discuss with
patient about advantages of triple procedure*
+ +++ Cataract extraction rst, PKP later
Alternatively, discuss with patient about advantages of triple
procedure
0 +++ Cataract extraction rst
Corneal decompensation likely to develop, PKP later
*Disadvantages of individual procedures (PKP and cataract extraction in separate sittings):
Two operations, increased cost and increased rehabilitation time
Corneal graft more likely to fail
Visibility poor during the second procedure
IOL power difcult to calculate
*Fuchs endothelial dystrophy is one of the main indications for endothelial keratoplasty
Exam tips:
Remember there are no RIGHT or WRONG answers.
First, be as conservative as possible. Give extremes of each scenario, then go
to the more controversial middle ground.
Opening statement is similar in all situations: There are two clinical prob-
lems which must be managed simultaneously. Factors to consider include .
Then give your own scenario.
See also management of neovascular glaucoma (page 69), bullous keratopa-
thy (page 97) and glaucoma and cataract (page 25).
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TOPIC 10
KERATOCONUS
Q Opening Question: What are the Clinical Features of Keratoconus?

Keratoconus is a noninammatory ecstatic corneal condition.
Characterized by central or paracentral stromal thinning, apical protrusion and irregular
astigmatism. (classical triad)
The clinical features can be early and subtle or late and gross.
Clinical Features of Keratoconus
1. Early signs: 2. Late signs:
Keratoscopy/Placidos disc (irregular rings) Paracentral stromal thinning
Retinoscopy (scissoring reex) Fleischers ring
Direct fundoscopy (oil drop sign) Corneal scarring
Vogts striae Munsons sign (bulging of lower lids when
Prominent corneal nerves patient looks down)
Rizuttis sign (conical reection off nasal cornea
with slit lamp light from temporal side)
Q What are the Causes of Keratoconus?
Causes of Keratoconus
1. Primary Connective tissue disorders (e.g. Marfans
Idiopathic (prevalence: 400/100,000) syndrome, osteogenesis imperfecta)
AD in 10% Cutaneous disorders (e.g. atopic dermatitis)
Ocular:
2. Secondary
Congenital ocular anomalies (e.g. aniridia,
Systemic:
Lebers congenital amaurosis, retinitis
Chromosomal disorders (e.g. Downs
pigmentosa)
syndrome)
Contact lens wear
Exam tips:
Remember the causes of CONES are the 5 Cs!
Q What are the Histological Characteristics of Keratoconus?
Triad of
Thinned stroma Breaks in Bowmans membrane layer
Epithelial iron deposit (Descemets membrane and endothelium are normal
unless hydrops has developed)
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Clinical approach to keratoconus

On examination of this patients anterior segment, there is evidence of
keratoconus.
Look for
Classical signs:
Paracentral stromal thinning
Vogts striae
Fleischers ring
Corneal scarring
Secondary causes:
Downs syndrome, Turners syndrome
Marfans syndrome
Aniridia, ectopic lentis
Ill like to
Evert lids to look for features of vernal keratoconjunctivitis
Examine fundus to exclude RP
Exam tips:
Be very careful when you are asked to examine a young patient with an
otherwise NORMAL SLIT LAMP EXAM (page 121) because the ocular
ndings of keratoconus can be subtle.
Clue: A Placidos disc may be conveniently located next to the patient!
Q When would You Consider Corneal Grafting for Keratoconus?
1. Conservative treatment rst (usually good enough 3. Special preoperative and intraoperative factors to
in 90% of patients): consider:
Spectacles Most cases are treated with lamellar
Special contact lens keratoplasty these days
Collagen cross-linking: Lower risk of endothelial rejection
New technique in which photo-sensitizing Young patients
agent (riboavin) is applied to cornea and Treat vernal keratoconjunctivitis aggressively
collagen cross-links are induced with Large graft (but reduce graft over-sizing to
UV-irradiation reduce myopia)
Shown to retard keratoconus progression Eccentric graft
2. Indications for corneal grafting: Trephination:
Unable to achieve good vision with contact lens Hard to t trephine (may need hot probe to
Intolerant to contact lens wear atten cornea)
Scarring after acute hydrops Shallow trephine (0.3 mm)
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Q What are the Other Causes of Prominent Corneal Nerves?
Causes of Prominent Corneal Nerves

1. Ocular diseases: Neurobromatosis
Keratoconus Multiple endocrine neoplasia type IIb
Keratoconjunctivitis sicca (medullary CA thyroid, parathyroid CA,
Fuchs endothelial dystrophy pheochromocytoma)
Trauma Refsums disease
Congenital glaucoma Ichthyosis
2. Systemic diseases: Normal variant with increasing age
Leprosy
B1037_Ch-03.indd 141 12/22/2010 5:44:50 PM

TOPIC 11
CRYSTALLINE
KERATOPATHY
AND MISCELLANEOUS
KERATOPATHIES
Q Opening Question: What are the Causes of Crystalline Keratopathy?

Crystalline Keratopathy
1. Infectious diseases Chrysiasis (gold)
Infectious crystalline keratopathy: Protein deposit:
Occurs when there is suboptimal inammatory Cystinosis
response to organisms (e.g. post PKP) Dysproteinemia (multiple myeloma)
Common organisms: Streptococcus viridans, Medication deposit:
Staphylococcus epidermidis) Topical ciprooxacin
2. Noninfectious diseases Amiodarone
Lipid deposit Tamoxifen
Crystalline dystrophy of Schnyders Phenothiazines

Indomethacin
Mineral deposit:
Chloroquine
Argyrosis (silver)
Band keratopathy (calcium) Idiopathic:
Crystalline dystrophy of Bietti
Clinical approach to vortex keratopathy

On slit lamp examination, there are .
Grayish/brownish corneal epithelial deposits.
Radiating from a point below the pupillary axis.
The lesions are seen in both eyes.
And are consistent with a diagnosis of vortex keratopathy.
Look for
Lens opacity (amiodarone, Fabrys disease)
Bulls eye maculopathy (chloroquine), crystalline retinopathy (tamoxifen)
Optic disc (tamoxifen)
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Ill like to
Ask patient for a history of:
Arthritis (indomethacin)
Breast CA (tamoxifen)
Cardiac diseases (amiodarone)
Connective tissue diseases (chloroquine)
Dementia, psychiatric diseases (chlorpromazine)
Exam tips:
One of few differential diagnoses for NORMAL SLIT LAMP EXAM (page 121).
The causes can be remembered as ABCD.
Q Tell me about the Mucopolysaccharidosis.
Mucopolysaccharidoses are a group of systemic storage diseases due to deciency of lysosomal

enzymes.
There are numerous specic types, each with its own systemic and ocular features.
The systemic features include mental retardation, coarse facies, skeletal abnormalities and cardiac
diseases.
In general, the ocular features include corneal deposits, retinal degeneration and optic atrophy.
Cornea Retinal
Type Name deposition degeneration Optic atrophy Notes
1H Hurler +++ + + All are AR except Hunters
(SLR)
1S Scheie +++ + + Hurler and Scheie have the
most severe corneal lesions
2 Hunter ++ +++ Hunter are males and
have clear corneas
3 Sanlippo +++ +
4 Morqio + + 4 and 6 no retinal
degeneration
5 None 5 became Scheie
6 Maroteaux + +
Lamy
Q Tell me about Wilsons Disease.
Wilsons disease is a metabolic systemic disease.

Characterized by deciency in alpha 2 globulin (ceruloplasmin).
Resulting in deposition of copper throughout the body.
Wilsons Disease
1. Systemic features CNS (40%)
Liver (40%) No mental retardation
Basal ganglia (apping tremors)
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Spasticity, dysarthria, dysphagia

Deposition in Descemets membrane
Psychiatric problems
Green sunower cataract
Laboratory results: Accommodation difculty (deposition in ciliary
Normal total serum copper muscles)
Low serum ceruloplasmin
3. Treatment
High urine copper
Decrease copper intake
2. Ocular features
Penicillamine (KF ring will resolve with
Kayser Fleisher ring (KF ring):
treatment)
90% of all patients: Almost 100% if CNS
involved
Exam tips:
KF rings in one of few differential diagnoses for NORMAL SLIT LAMP EXAM
(page 121).
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TOPIC 12
SCLERITIS
Q Opening Question: What is Scleritis?

Scleritis is an inammatory disease of the sclera.
It can be classied into .
Scleritis
1. Classication Surgically induced necrotizing scleritis (SINS)
Anterior scleritis (Watson and Hayreh) Infective:
Non-necrotizing (40%): Herpes zoster
Diffuse (benign disease) TB, syphilis
Nodular (visual loss in 25%)
3. Investigations
Necrotizing (40%): CBC, ESR
With inammation (visual loss in 75%:
VDRL, FTA
Mortality in 25%):
Collagen disease markers
Veno-occlusive
CXR
Granulomatous
SINS 4. Treatment
Without inammation (scleromalacia Treat associated systemic diseases
perforans (benign)) Treat associated ocular complications
Posterior scleritis (20%) (glaucoma, cataract)
2. Systemic associations (50%) Treatment of scleritis depends on type and severity:
Noninfective: Anterior scleritis, non-necrotizing (NSAIDs)
Rheumatoid arthritis (RA) (40%) Posterior scleritis (oral systemic steroids)
Systemic lupus erythematous, Wegeners Anterior scleritis, necrotizing with

granulomatous, polyarteritis nodosa, inammation (IV steroids and
relapsing polychondritis immunosuppressive agents)
Q What are the Clinical Features of Posterior Scleritis?
Posterior scleritis is an inammatory disease of the sclera posterior to the equator.

It represents about 20% of all scleritis .
Posterior Scleritis
1. 20% of all scleritis 80% present as either disc swelling or exudative
2. 20% associated with systemic diseases RD (important differential diagnosis of VKH)
3. 80% associated with concomitant anterior Other presentations:
Subretinal mass (more common in females)
scleritis Ring choroidal detachment (more common in
4. Visual prognosis is poor (80% develop visual loss) males)
5. Clinical presentation vary Vitritis
Macular edema, subretinal exudation,
choroidal folds
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Exam tips:
20:80 rule.
Clinical approach to scleritis

There is a yellowish necrotic nodule seen in the superior sclera.
There is associated inflammation of the surrounding sclera and injection of
scleral vessels.
Or
There is marked thinning of the superior sclera with little
inflammation seen.
Look for
Corneal peripheral thinning (important sign for RA, systemic lupus,
Wegeners granulomatosis, polyarteritis nodosa)
AC activity and keratitic precipitates
Previous cataract or pterygium surgery (SINS)
Bilateral disease (RA, systemic lupus, Wegeners granulomatosis,
polyarteritis nodosa)
Lid scarring (herpes zoster)
Systemic features (RA, systemic lupus, Wegeners
granulomatosis, polyarteritis nodosa)
Ill like to
Check IOP (glaucoma in scleritis)
Check fundus for optic disc swelling, choroidal folds, RD
Examine patient systemically (RA, systemic lupus, Wegeners
granulomatosis, polyarteritis nodosa)
Exam tips:
Be very careful when you see NORMAL SLIT LAMP EXAM (see below). Look
at the sclera!
Clue: Patients may have systemic features such as rheumatoid arthritis (RA)
or appear Cushingoid from prolonged steroid therapy!
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Notes
Differential diagnoses for a NORMAL SLIT LAMP EXAM
1. Cornea:
Keratoconus
Vortex keratopathy
Microcystic epithelial corneal dystrophy
KayserFleischer ring
Fuchs endothelial dystrophy
2. Iris:
Rubeosis
Atrophy
3. Lens:
Phacodonesis
Glankomecken
4. Sclera:
Scleritis
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TOPIC 13
CORNEAL GRAFTS
Q Opening Question No. 1: Tell me about Corneal Grafts.

Corneal graft is a surgical procedure in which diseased host cornea is
replaced by healthy donor cornea.
Broadly, corneal grafts can be either partial thickness/lamellar or full thickness/penetrating.
The indications for full thickness corneal graft are .
Prior to the operation, the patient must be evaluated for .
Exam tips:
This is a gift question! You should be able to talk for at least a few minutes
without any interruption.
Q Opening Question No. 2: What are the Indications for Penetrating Keratoplasty (PKP)?
The indications for corneal grafts can be .
Indications for PKP
1. Optical Corneal traumatic scars
Bullous keratopathy (pseudophakic and Failed grafts
aphakic) 2. Tectonic
Keratoconus Corneal perforation
Corneal dystrophy Peripheral corneal thinning
Corneal inammatory diseases interstitial 3. Therapeutic
keratitis, HSV Infective keratitis
Q What are the Preoperative Factors and Manage Poor Prognostic Factors Prior to PKP?
1. Evaluate patients ocular condition and manage

poor prognostic factors prior to PKP
Cornea irregularity
Factors (Big 4 poor prognostic factors)
Pre-existing cataract (consider triple procedures)
Ocular inammation
Structural changes of AC (peripheral anterior
Glaucoma
synechiae, rubeosis)
Corneal vascularization
Ocular surface abnormalities: 2. Assess visual potential
Associated lid abnormality (entropion, Retinal and macular conditions (e.g. cystoid
ectropion) macular edema)
Tear lm dysfunction and dry eyes Amblyopia
Other factors to consider: Optic atrophy
Corneal hypoesthesia
Topical antibiotics / steroids / cyclosporin A if necessary
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Exam tips:
Remember the BIG 4 poor prognostic factors well.
Q Opening Question No. 3: How do You Perform a PKP?

Steps in PKP
1. Preoperative preparation Set trephine to 0.4 mm depth
GA Enter into AC with blade
Maumenee speculum Complete incision with corneal scissors
Superior and inferior rectus bridle suture with 4. Fixation of graft
4/O silk: Fill AC with viscoelastic
Flieringa ring if necessary (indications: Post
Place donor button on recipient bed
vitrectomy, aphakia, trauma, children)
Four cardinal sutures with 10/0 nylon (at
Overlay suture if necessary (7/0 silk at limbus)
12 oclock rst, followed by 6, 3 and then 9)
Check recipient bed size with Weck trephine
16 interrupted sutures
(usually 7.5 mm)
Advantages of interrupted sutures:
2. Donor button Easier for beginners
Check corneoscleral disc Better for inamed eyes and eyes with
Harvest donor cornea button with Weck vascularization

trephine on Troutman punch: Other suture techniques
Approach from posterior endothelial side Continuous suture:
Use trephine size 0.250.5 mm larger than Faster
recipient bed Better astigmatism control
Keep button moist with viscoelastic Not for cases where selective suture
3. Recipient bed removal may be needed (e.g. infections)

Combined continuous and interrupted
3-point xation (two from bridle suture, one
sutures
with forceps)
Weck trephine imprint to check size and 5. End of operation
centration Check water tightness and astigmatism with
Other types of trephine: keratometer
Baron Hessberg trephine and Hannah Subconjunctival steroids/antibiotics
trephine (suction mechanism)
Notes
How do you check the corneoscleral disc?
Container (name, date of harvest, etc.)
Media (clarity and color)
Corneal button (clarity, thickness, irregularity, surface damage)
Notes
Why is the donor button made larger than the recipient bed?
Because donor button is punched from posterior endothelial surface
Tighter wound seal for graft
Increases convexity of button (less peripheral anterior synechiae postop)
More endothelial cells with larger button
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Q How is the Donor Corneal Button Stored?
Storage media can be divided into .

Storage Media
1. Short term (days) Temp 4C
Moist chamber: Storage duration: 12 weeks
Humidity 100% Organ culture:
Temp 4C Advantages: Decreased rejection rate?
Storage duration: 48 hours (Culture kills antigen-presenting cells)
McCarey-Kaufman medium: Disadvantages: Increased infection rate?
Standard tissue culture medium (TC199, Temp 37C
5% dextran, antibiotics) Storage duration: 4 weeks
Temp 4C 3. Long term (months)
Storage duration: 24 days
Cryopreservation:
2. Intermediate term (weeks) Liquid nitrogen
Dexsol/Optisol/Ksol/Procell: Temp 196C
Standard tissue culture medium (TC 199) plus Storage duration: 1 year
chondroitin sulphate, HCO3 buffer, amino Disadvantages: Expensive and unpredictable
acid, gentamicin results; usually not suitable for optical grafts
Q What are the Contraindications for Donation of Corneas?
The contraindications included patients with .

Contraindications for Cornea Donation
1. Systemic diseases History of glaucoma and iritis
Death from unknown cause Intraocular tumors
CNS diseases of unknown cause 3. Age
Creutzfeld-Jakob disease, CMV encephalitis, < 1 year old
slow virus diseases Corneas are difcult to handle
Infections: Small diameter; friable
Congenital rubella, rabies, hepatitis, AIDS, Very steep cornea (average K = 50D)
Syphilis > 65 years
Septicemia Low endothelial cell count
Malignancies
4. Duration of death > 6 hours
Leukemias, lymphomas, disseminated cancer
5. Severe hemodilution: Affects accuracy of
2. Ocular diseases
Intraocular surgery serological testing
Q What are the Complications of Corneal Grafts?
The complications can be divided into complications specic to corneal grafts and general
complications of intraocular surgery.
They can occur in the early or late postoperative period .
Complication of Corneal Grafts
1. Early postoperative: 2. Late postoperative:
Glaucoma or hypotony Rejection
Persistent epithelial defect Infective keratitis
Endophthalmitis Recurrence of disease
Wound leak Astigmatism
Recurrence of primary disease Persistent iritis
Late endothelial failure
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3. Other complications of intraocular surgery: Expulsive hemorrhage

Cataract Retrocorneal membrane
RD CME
Q What are the Causes of Graft Failure?
Graft failure can be divided into early failure and late failure.
Graft Failure
1. Early failure (< 72 hours): Others:
Primary donor cornea failure Glaucoma
Unrecognized ocular disease Infective keratitis
Low endothelial cell count 2. Late failure (> 72 hours):
Storage problems Rejection (30% of late graft failures)
Surgical and postoperative trauma: Glaucoma
Handing Persistent epithelial defect
Trephination Infective keratitis
Intraoperative damage Recurrence of disease process
Recurrence of disease process (e.g. infective Late endothelial failure
keratitis)
Exam tips:
Do not confuse graft failure with graft rejection (which is one of the causes of
graft failure and may or may not lead to failure).
Q What are the Factors Which Affect Graft Survival?
The factors which affect graft survival can be divided into .

Graft Survival
1. Factors associated with higher risk of graft Position of graft (eccentric graft)
rejection: Presence of peripheral anterior synechiae
Young age Exposed sutures
Blood group incompatibility (Collaborative Deep stromal vascularization
Corneal Transplant Study) 2. Other factors associated with graft failure:
Repeat grafts Preexisting glaucoma and high IOP
Size of graft (large graft) Ocular surface (lids, tears)
Intraocular inammation (iritis)
Exam tips:
Remember the BIG 4 poor prognostic factors!
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Q How do You Grade Corneal Graft Prognosis According to Disease Categories?

Brightbills Classication
GRADE I (Excellent) Active HSV keratitis
Keratoconus Congenital hereditary endothelial dystrophy
Lattice and granular dystrophy Failed Grade II PKP
Traumatic leukoma GRADE IV (Guarded)
Supercial stromal scars Active fungal keratitis
GRADE II (Good) Congenital glaucoma
Bullous keratopathy Pediatric grafts
Fuchs dystrophy Mild keratoconjunctivitis sicca
Macular dystrophy Mild chemical burns
Small vascularized scars Corneal blood staining
Interstitial keratitis Corneal staphylomas
Failed Grade I PKP Failed Grade III PKP
Combined PKP and cataract op GRADE V (Poor)
GRADE III (Fair) Severe keratoconjunctivitis sicca (Stevens
Active bacterial keratitis Johnson ocular cicatrical pemphigoid, chemical
Vascularized cornea and thermal burns)
Exam tips:
Just remember the ones in BOLD!
Q Tell me about Graft Rejection.
Graft rejection is a type 4 immune reaction.

It can be divided into epithelial, subepithelial, stromal and endothelial rejection.
Graft Rejection
1. Pathophysiological basis of rejection: Usually low grade, asymptomatic, eye is
Type 4 immunological reaction quiet
Divided into: Epithelial, subepithelial, stromal Subepithelial rejection:
and endothelial rejection Nummular white inltrates (Krachmers spots)
Immunological phenomenon Mild AC activity
Stromal rejection:
2. Risk factors:
Most important of the 4 types
Age (young age)
Symptoms:
Repeat grafts Decreased VA
Size of graft (large grafts) Redness
Position of graft (eccentric graft) Pain
Peripheral anterior synechiae Signs:
Exposed sutures Limbal injection
Deep stromal vascularization AC activity
Keratic precipitates
3. Clinical features:
Endothelial rejection line (Khodadoust
Two weeks onwards (if less than two weeks,
line)
consider other diagnosis)
Stromal edema
Epithelial rejection:
Endothelial rejection:
Epithelial rejection line (advancing
Combination of stromal and endothelial
lymphocytes, replaced by epithelial cells
rejection
from recipient)
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Notes
What is the evidence that rejection is an immune phenomenon?
Rejection of 2nd graft from same donor begins after shorter interval and
progresses more rapidly
Brief period of latency (2 weeks) before rejection
Rejection correlates with amount of antigen introduced in graft
Neonatally thymectomized animals reject grafts with difculty
Notes
What are the problems of large grafts?
Increased risk of rejection (nearer vessels)
Increase IOP (more peripheral anterior synechiae)
Large epithelial defect (limbal stem cell failure)
Clinical approach to corneal grafts

This patient has a corneal graft .
The graft has interrupted sutures .
Look for
Type of graft (important in modern context)
Anterior lamellar graft
Interface usually visible (may have fine debris)
Endothelial graft:
Posterior lamella (look for the edge)
Venting incisions (aid egress of fluid from graft-host interface)
Temporal scleral tunnel incision
PIs (prevent pupil block during air tamponade)
Age of graft: Interface scarring, sutures
Pseudophakic/aphakic (pseudophakic or aphakic bullous keratopathy)
Rejection:
Hazy graft/local edema
Keratic precipitates, AC cells, Khodadoust line
Stromal vascularization
Other eye for corneal dystrophies, Keratoconus, (underlying disease)
Ill like to
Check IOP
Assess with Placido disc
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Q What is the Role of Cyclosporin A in Corneal Grafts?
1. Indications (high risk of graft rejection):

Young patient Urine tests
Repeat grafts CXR
Large grafts/sclerokeratoplasty ECG
Deep stromal vascularization 3. Treatment regime:
Limbal allografts (chemical injury, SJS) Cyclosporin A (neoral) 5 mg/kg/day in two
Post-graft rejection divided doses
2. Investigations prior to treatment Treatment continued for at least one year
Blood tests: Dosage gradually tapered after three months
CBC 4. Monitoring during treatment:
Renal function tests and uric acid levels BP, height and weight
Fasting blood glucose and HB A1C
CBC, renal function, liver function
Liver function tests
CXR, ECG
Hepatitis B screen and serology for
Serum cyclosporin level
hepatitis C, herpes zoster, CMV and HIV
Co-management with renal transplant physician
Q Tell me about Lamellar Keratoplasty.
Lamellar keratoplasty is a partial thickness corneal graft.

Lamellar Keratoplasty
1. Indications Smaller wound lower risk of wound
Partial thickness corneal diseases: rupture
Supercial corneal dystrophies (Reis-Buckler) Replaceable and repeatable
Supercial corneal scars Reduced risk of rejection (controversial)
Recurrent pterygium 3. Disadvantages
Corneal thinning (Terriens marginal Interface scarring
degeneration) Technically more difcult
Corneal perforation
Not suitable when residual lamella (Descemets/
Congenital lesions (limbal dermoid)
endothelium in anterior lamellar keratoplasty,
Supercial tumors
Endothelial dystrophies/endothelial failure
anterior stroma in endothelial keratoplasty) is
not clear/intact
2. Advantages
Anterior lamellar keratoplasty: 4. Complications
Minimal donor tissue requirements Anterior lamellar keratoplasty:
No intraocular entry Intraoperative perforation
Faster wound healing and rehabilitation Astigmatism
Lower risk of rejection and less use of topical Double anterior chamber
steroids Interface haze/scarring
Endothelial keratoplasty (DSAEK Descemets Endothelial keratoplasty:

Donor dislocation
Stripping Automated Endothelial Keratoplasty):
Primary endothelial failure
Reduced astigmatism
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TOPIC 14
BASICS IN CONTACT LENS

Q Opening Question: What are the Indications for Contact Lens in Ophthalmology?
The indications can be divided into .
Indications for Contact Lens
1. Refractive (most common) Leukocoria
2. Therapeutic (see below) Phthsis bulbi
3. Cosmetic: 4. Diagnostic and surgical (goniolens, fundus
Corneal scar contact lens)
Q What are the Therapeutic Indications for Contact Lens?
Therapeutic Indications for Contact Lens

1. Optical Thygesons keratitis
Uniocular aphakia Superior limbic keratoconjunctivitis
Irregular astigmatism keratoconus Filamentary keratitis
2. Pain relief 4. Protect cornea
Bullous keratopathy Exposure keratopathy
Corneal abrasions Entropion, trichiasis
Post-photorefractive keratectomy Post-ptosis operation or Post-op ptosis
3. Promote corneal healing 5. Perforated corneas
Recurrent corneal erosion Descemetocele
Persistent epithelial defect 6. Pharmaceutical delivery device
Exam tips:
One O and ve Ps!
Q What are the Materials Used for Contact Lens?
The ideal material for contact lens should be .

Currently, (the) materials include .
Ideal Material
1. Optically clear Thin
2. High oxygen transmission Related to Dk/L, where Dk = Permeability,
Water soluble L = thickness
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3. Comfortable 2. Soft hydrogel (HEMA)

Soft High water content extended wear soft
Surface wettability contact lens (EWSCL)
4. Low complication rate Low water content daily wear soft contact
5. Durable lens (DWSCL)
High tensile strength Silicone hydrogels high gas permeability, for
Resistant to deformation, tear extended wear
6. Ease of sterilization 3. Semi-exible/rigid gas permeable (RGP)

CAB (cellulose acetate butyrate)
Current Contact Lens Material Silicone
1. Hard PMMA (polymethylmethacrylate) Polycon (90% PMMA and 10% silicone)
Q Tell me about Contact Lens. What are the Advantages and Disadvantages?
Soft contact lens can be broadly divided into extended wear (EWSCL) or daily wear (DWSCL).
They are made of hydrogel, with varying water contents .
Soft Contact Lens
1. Advantages of soft CL: Higher risk of complications
Comfortable Durability low
Greater stability 3. Indications for DWSCL:
Ease of tting First time wearer
Ease of adaptation Part time wearer
Rarely get overwear syndrome Failed extended wear
Lack of spectacle blur 4. Indications for EWSCL:
2. Disadvantages: Infants, children and elderly
Poorer VA in eyes with astigmatism Lack of manual dexterity
Therapeutic indications
Q What are the Pathophysiological Changes to the Eye with Contact Lens Wear?
The pathophysiological changes included .

Pathophysiological Changes to the Eye
1. Desiccation 3. Hypoxia
2. Microtrauma 4. Hypersensitivity/toxicity
5. Endothelial blebbing (transient)
Q What are the Complications of Contact Lens?
Contact lens complication can be divided into blinding and nonblinding complication.
Complications of Contact Lens Wear
1. Blinding Related to microtrauma:
Infective keratitis Punctate epithelial erosions
Corneal scarring Corneal abrasion
Corneal warping (rarely) Superior limbic keratoconjunctivitis

Related to hypoxia:
2. Nonblinding (Note: Related to the four
Corneal edema
pathophysiological changes!) Epithelial microcysts, acute overwear
Related to desiccation syndrome (rupture of cysts)
Dry eye syndrome Corneal vascularization
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Related to hypersensitivity/toxicity: 3. Contact lens changes

Giant papillary conjunctivitis Distortion, breakage
Allergic conjunctivitis (disinfectant, Deposits:
preservative thiomersal) Minerals iron, calcium
Sterile inltrates Organic mucin, lipid, protein
Microorganisms bacteria, fungi
Q Tell me about Giant Papillary Conjunctivitis.
GPC is one of the common contact lens complication .

Secondary to hypersensitivity.
GPC presents in different stages .
GPC
1. Stages Hypersensitivity (asthma, hay fever)
Stage 1: Preclinical GPC (symptoms only) Trauma (foreign body and prosthesis)
Stage 2: Macropapillae (0.3 mm1 mm) 4. Management
Stage 3: Giant papillae (> 1 mm) Stage 1 and 2:
Stage 4: Subconjunctival scarring Lens hygiene
2. Zones Decrease wearing time
Zone I: Forniceal Reevaluate t and material/change to RGP if
Zone II: Tarsal needed
Topical antihistamines and mast cell
Zone II: To lid margin
stabilizers
3. Etiology Topic steroids if necessary
Contact lens wear: Stage 3 and 4:
30% of patients with EWSCL
Consider discontinuation of contact lens
15% of patients with DWSCL
wear
15% of patients with RGP
Q How do You Fit Contact Lens?
Contact Lens Fitting

1. History Assess t (with uorescein staining):
Visual requirements, ocular diseases Tightness (too at or too steep)
2. Fitting procedure for soft contact lens Centering
Mobility
Base curve inversely proportional to the
Aim for three-point touch
keratometry (K) reading:
Take mean K + 1 (aim for atter contact lens) Over-refract with contact lens on (e.g. If 1.5D
Choose from three standard curves available gives VA of 20/20)
(8.1, 8.4, 8.7 mm) Prescribe nal t (e.g. 8.4/5.5D/13.5)
Refraction 3. Fitting procedure for hard contact lens
Corneal diameter (13, 13.5, 14 mm) Base curve:
Ocular examination: Take mean K (do not need to add 1)
Palpebral aperture and tightness Choose from different individual curves
SLE (7.2 to 8.5)
Fundus exam Refraction (choose from different powers
Select trial lens (base curve/refraction/corneal for each base curve)
diameter e.g. 8.4/4.0D/13.5) Corneal diameter (8.8, 9.2, 9.6 mm)
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TOPIC 15
REFRACTIVE SURGERY
Q Opening Question: What are the Different Types of Refractive Surgery Available?
Refractive surgery is a procedure to alter the refractive status of the eye.
This usually involves procedure on the cornea or the lens.
They can be broadly divided into incisional procedures, laser procedures or
intraocular surgical procedures.
Correction of Myopia
1. Incisional procedures LASIK (laser in-situ keratomileusis)
RK (radial keratotomy) Modication of ALK, using laser for the
Up to 5D second pass
PERK (prospective evaluation of RK) study Up to 15D
showed that 40% had hyperopic shift of 1D Variants:
or more after 10 years Epi-LASIK: Bladeless LASIK: Epithelial
Obsolete procedure sheet cleaved off surface (no ap)

Disadvantages of RK: LASEK: Surface epithelial sheet loosened
Weakened cornea with alcohol

Diurnal variation in refraction Wavefront optimized ablations: Reduce
Hyperopic shift spherical aberration reduce night vision

Epikeratoplasty problems, glare and halos
Remove corneal epithelium and create Wavefront guided ablations: Customized
peripheral annular keratotomy incision ablation guided by individual wavefront

Frozen donor corneal lenticule xed to map
recipient cornea 3. Intraocular surgery
Current indications (not many left with ICSR (intracorneal stromal ring)
advances in PRK and LASIK): PMMA half rings are threaded into peripheral
Childhood aphakia mid stroma to effect a attening of the cornea
Keratoconus Up to 6D
Extremely high myopia
High myopia procedures (> 12D)
Keratomileusis Clear lens extraction (with IOL)
Cornea sliced off with microkeratome AC phakic IOL implantation
Cornea cap then frozen, shaped and Conventional four-point xated AC IOL
reapplied to corneal bed Iris-xated phakic IOL
ALK (automated lamellar keratoplasty) Suffers from complications of ACIOLs:
Cornea cap sliced off with automated Glaucoma
microkeratome Iris chang/uveitis
Second pass of microkeratome to cut a Hyphema
corneal disc from stromal bed Corneal endothelial damage
Cornea cap is then reapplied to cornea bed PC phakic IOL implantation
2. Laser procedures Sulcus xated phakic IOL
PRK (photorefractive keratectomy) Silicon injectable IOL (implantable collamer
Up to 6D lens)
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Complications: Scleral sling:

Glaucoma (angle closure, hence need for PI) Up to 1822D
Iris chang/uveitis Use donor sclera/synthetic materials to sling
Hyphema around globe
Cataract
Exam tips:
Extremely common and important essay or viva topic. Keep up with the latest
refractive surgery trends.
Q What are the Options in the Correction of Hyperopia?
Hyperopia
1. Hexagonal keratotomy 6. Laser thermokeratoplasty
2. Epikeratoplasty Small coagulation burns applied to cornea
3. ALK stroma with holmium laser
4. PRK and LASIK 7. AC phakic IOL
5. Radial intrastromal thermokeratoplasty

Small coagulation burns applied to cornea
stroma with retractable cautery probe
Q What are the Options in the Correction of Astigmatism?
Astigmatism
1. AK (Astigmatic keratotomy) 68 mm optical zone
Preoperatively need to have keratometer Each cut corrects 1 to 1.5D of astigmatism
readings, corneal topography and pachymetry 2. PARK (Photoastigmatic refractive keratectomy)
Procedure: and LASIK
Guarded diamond knife
3. Toric IOL
95% corneal depth cut
45 degrees at the steep axis
Plate haptic silicon design IOL after lens removal
Need precise axis orientation
Q Tell me about PRK.
PRK is photorefractive keratectomy and is a form of refractive surgery.

PRK
1. Procedure For myopia < 6D
193 nm argon uoride excimer laser used to 8090% see 20/40 or better
ablate cornea 7080% predictability
1% signicant corneal haze
Every 10 micron = 1D of myopia
15% loss of BCVA
Three types of ablation:
Wide area ablation
High myopia > 6D
5075% see 20/40 or better
Scanning slit
3070% predictability
Flying spot
515% corneal haze
2. Indications and limitations Up to 20% loss of BCVA
PRK works well for low and moderate myopia More regression
and astigmatism Higher retreatment rate
3. Advantages and disadvantages of PRK (see below)
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Q What is LASIK?
LASIK stands for Laser In-situ Keratomileusis and is a form of refractive surgery.
LASIK
1. Procedure: Compound myopic and hyperopic
Microkeratome creates corneal ap that is astigmatism
hinged, either nasally or superiorly 3. Advantages of LASIK (ve distinct advantages):
Flap is reected Better predictability
Excimer laser ablates stroma of cornea for More stability
refractive correction Minimal pain
Flap is replaced without sutures Rapid visual rehabilitation (< 24 hrs)
Femtosecond laser Low risk of corneal haze/scarring and therefore,
Newer technique for creating aps using less steroids needed
multiple extremely short (femtosecond)
4. Disadvantages:
pulses of IR light
Expensive and complex microkeratome
Pattern of pulses programmable
Theoretical advantages:
required, in addition to an excimer laser
Lower risk of ap complications
More technical and surgical expertise required
Fewer higher order aberrations induced
with steep learning curve
Possible disadvantages Risk of visually threatening complications
Irregularity/haze at interface Risk of ap complications (see below) and
Cost reduced residual stromal thickness because of
Longer operating time need for tissue for ap
2. Indications and limitations: 5. Complications:
Maximum refractive errors that can be treated Flap complications:
are dependent on central corneal thickness Free aps/incomplete aps/button hole aps
Contraindications: Flap striae/dislodged aps
Thin corneas and ectatic disorders Flap melts
(keratoconus) absolute contraindication Flap striae
Wound healing problems (e.g. connective Interface complications:
tissue disease, diabetes) Epithelial ingrowth
Corneal infections (HSV) Interface debris
Pregnancy (unstable refraction) Interface haze
Glaucoma (relative contraindication high Diffuse lamellar keratitis (Sands of
pressure applied during procedure) Sahara)
Dry eye and ocular surface problems Induced irregular astigmatism
Current limits: Decentration of ablation zone
Myopia up to 5D Night vision problems (hence aspheric
Hyperopia up to 5D corrections)
Astigmatism up to 4D Bacterial keratitis
Progressive ectasia of cornea
PRK LASIK
Predictability/Accuracy Up to 6D Up to 15D
Stability Up to 6D Up to 15D
Pain and rehabilitation Pain from epithelial defect (12 days) Minimal pain
Prolonged visual rehabilitation Rapid visual rehabilitation
(up to 1 week) (< 24 hrs)
(Continued)
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(Continued )
PRK LASIK
Corneal haze Up to 10% (destruction of Minimal haze
Bowmans layer)
Poor contrast sensitivity
Halos
Glare
Complications Rare Uncommon
Irregular astigmatism 1% 310%
Training and equipment Short training period Steep learning curve
Less expensive equipment More expensive equipment
Retreatment Easier More difcult
Q What is Corneal Astigmatism?
An optical aberration resulting from variation in the refractive power of the cornea due to an asymmetry
in its curvature.
Classication Causes
1. Regular 1. Idiopathic
Steepest and attest meridian are 90 from each 2. Secondary to ocular diseases
other Developmental keratoconus
Subdivided into with the rule and against the Degeneration pellucid marginal
rule degeneration, Terriens degeneration
Blurred retinal images can be improved with an Infection scar formation
appropriate cylindrical correction Inammation peripheral ulcerative keratitis
2. Oblique (RA, Moorens ulcer)
Steepest and attest meridians are not at 90 Traumatic scar formation
from each other 3. Iatrogenic
3. Irregular Large incision cataract surgery
Amount of astigmatism changes along a given Penetrating keratoplasty
meridian and varies from meridian to meridian
Secondary to irregular corneal surface Assessment of Astigmatism
1. Refraction
Further Classication 2. Keratometry
4. Simple myopic astigmatism 3. Corneal topography
Emmetropic in one meridian and myopic in Placido disc
other Computerized videokeratometry
5. Compound myopic astigmatism Elevation based systems
Both steepest and attest meridians focused in Orbscan:
front of retina One of the commonest systems used
Combination of Placido and elevation based

6. Simple hyperopic astigmatism
systems
7. Compound hyperopic astigmatism Generates four maps:
8. Mixed astigmatism Anterior oat (anterior elevation)
One meridian focused in front of retina, one Posterior oat (posterior elevation)
behind Keratometry
Pachymetry
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Indications: Signs:
Assessment of regular and irregular Inferior-superior asymmetry
astigmatism Inferior steepening
Diagnosis of forme fruste keratoconus Elevated anterior and posterior oat
(important in pre-keratorefractive surgery Inferior thinning
assessment) Inter-eye asymmetry
Q What are the Options in the Management of Corneal Astigmatism?
1. Glasses Semiradial incision

2. Contact lens Trapezoidal keratotomy
3. Photorefractive keratectomy
4. Surgery cuts in steep incision
Transverse and arcuate keratotomy
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TOPIC 16
MISCELLANEOUS
CORNEAL PROCEDURES
Q Opening Question: When and How do You Perform a Corneal Biopsy?

1. Indications:
Infective keratitis (culture negative, not Debride slough
responding to treatment) Avoid visual axis
Amoebic keratitis Choose between lesion and good cornea
Carcinoma intraepithelial neoplasia Use a dermatological trephine with 2, 3
2. Procedure: or 4 mm diameter to mark tissue
Stop antibiotic for 24 48 hours Lamellar dissection of tissue with blade
Topical anesthesia Divide tissue for histology and culture
Q When and How do You Perform Corneal Gluing?
1. Composition:
Corneal glue made of isobutyl cyanoacrylate Debride slough and necrotic tissue
(histoacryl) Dry cornea
2. Indications: Apply glue onto cellophane plastic disc
Small perforation < 1 mm in size Apply glue and cellophane disc on
3. Procedure: perforation leave to dry
Topical anesthesia Apply bandage contact lens
Q When do You Perform a Conjunctival Flap?
1. Indications: 2. Problems with conjunctival ap:

POOR visual potential Temporary treatment
Chronic epithelial/stromal ulcer after resolution No view of cornea
of active infective disease Low drug penetration
Neurotrophic ulcer Postoperative complication (button hole,
Chemical injury epithelial cyst, retraction of ap, bleeding,
Bullous keratopathy ptosis)
Descematocoele
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Section 4
Surgical Retina
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TOPIC 1
THE RETINA
Q Opening Question No. 1: What is the Anatomy of the Retina?

The retina is the innermost layer of the globe .
It is divided into the inner neurosensory layer and the outer retinal pigment epithelium.
Gross Anatomy of the Retina
1. Neurosensory retina and RPE Ora serrata:
Classic 10 layers: Anterior limit of retina (8 mm from nasal
RPE limbus, but 8.5 mm from temporal limbus)
Outer segment of photoreceptor Vitreous base rmly adherent to ora serrata
Outer limiting membrane 2. Bruchs membrane
Outer nuclear layer (nuclei of photoreceptor) Separates RPE from choriocapillaris
Outer plexiform layer (Henles layer, synapse
Classic ve layers:
between photoreceptor and bipolar cells) Basement membrane of RPE
Inner nuclear layer (nuclei of bipolar cells,
Inner collagen layer
plus nuclei of horizontal, amacrine, Mullers Middle elastic layer
cells) Outer collagen layer
Inner plexiform layer (synapse between
Basement membrane of choriocapillaris
bipolar cells and ganglion cells)
Ganglion cell layer
3. Choroid
Nerve ber layer Separates retina from sclera
Inner limiting membrane Classic three layers:
Choriocapillaris
Middle vascular layer
Outer vascular layer
Exam tips:
Remember there are 10 layers in the retina, 5 in Bruchs membrane and 3 in
the choroid.
Q Opening Question No. 2: Tell me about the Rods and Cones.

Rods and Cones
1. Rods Outer (ellipsoid) contains mitochondria
120 million; 50 m long Outer segment:
Nucleus Composed of 1,000 stacked discs
Inner segment: Discs separate from cell membrane
Inner (myoid) contains Golgi apparatus Discs have visual pigments

Renewal of outer segment
Section 4: Surgical Retina 173
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Discs formed at proximal end (i.e. near inner Nucleus

segment) and shed at distal end (next to RPE) Inner segment
Old discs phagocytosed by RPE cells Outer segment:
Rate of shedding: 15 per hour Stacked disc connected to cell membrane
Regeneration over 14 days Renewal of outer segment:
Shedding maximal in early light cycle, when Diffuse renewal (no proximal to distal
functionally less active direction)
2. Cones Regeneration over 9 months
6 million (i.e. only 5% of rods) and 25 m long Shedding maximal in early dark cycle,
(i.e. half as long as rods) when functionally less active
Q What are the Visual Pigments?
Visual Pigments
1. Outer segment discs made up of lipid bilayer Blue cone contains short wavelength
membrane sensitive/blue sensitive iodopsin (max: 440 nm)
2. Visual pigments contained in lipid bilayer Green cone contains medium wavelength
membrane sensitive/green sensitive iodopsin (max: 535 nm)
3. Visual pigments made up of chromophore plus Red cone contains long wavelength sensitive/
protein (opsin) red sensitive iodopsin (max: 570 nm)
4. Chromophore: 6. Opsin in rods called rhodopsin:
Linked to opsin via Schiff base reaction Transmembranous protein
11-cis retinal is chromophore in all four types of N terminus exposed to intradisc space
visual pigments C terminus exposed to interdisc (cytoplasmic)
Chromophore aligned parallel to plane of lipid space
bilayer (to increase light capture)
5. Four types of visual pigments (based on different
absorption characteristics):
Rods contains rhodopsin (max absorption:
500 nm)
Q What is the Visual Cycle?
Visual Cycle
1. In the dark: All-trans-retinal converted to all-trans-retinol
Outer segment cell membranes allow entry of All-trans-retinal transported out of
sodium ions photoreceptor into RPE cells
Inner segment actively secretes sodium out via Intermediate retinal (metarhodopsin II) causes a
sodium potassium ATPase pump dark series of reactions which blocks sodium
current (electric current ows from inner to channels in outer segment decreased
outer segment) intracellar sodium graded hyperpolarization
2. In the light (bleaching): (From 40 mV to 70 mV) reduced
Light causes change in visual pigments neurotransmitter release
11-cis retinal converted to all-trans-retinal
Q What is the Vitamin A Cycle?
Vitamin A Cycle
1. Vitamin A occur in four forms: Aldehyde (retinal)
Acid (retinoic acid) Alcohol (retinol)
Ester (retinyl ester)
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2. Three sources of vitamin A During bleaching all-trans-retinal released

From diet and liver: from opsin converted to all-trans-retinol in
Vitamin A stored in liver as retinyl ester outer segment
Hydrolyzed to retinol combined with All-trans-retinol transported to RPE
serum retinol binding protein delivered to converted back to 11-cis retinol by
RPE isomerase
Stored as retinyl ester 11-cis retinol transported back to outer
From fragments of rod outer segments during segment converted to 11-cis retinol by
shedding and phagocytosis reductase combined with opsin to form
From rod outer segments during bleaching rhodopsin again
Q Tell me about the Bipolar Cells.
Bipolar cells are rst-order neurons of the visual pathway.

They are located in .
Bipolar Cells
1. Anatomy: In periphery (100 rods synapse with single
30 million bipolar cell)
Located in inner nuclear layer 2. Five types
First-order neurons Rod bipolar cells
Account for b wave of ERG Invaginating:
Synapses: Midget
Single or multiple dendrites synapse with Diffuse
cones and rods (and other cells) Flat:
Single axon synapse with ganglion cell (2nd Midget
order) Diffuse
In fovea (single cone synapse with single
bipolar cell and then with single ganglion cell)
Q Tell me about the Ganglion Cells in the Retina.
Ganglion cells are second-order neurons along the visual pathway.

They are located in.
Ganglion Cells
1. Anatomy PARVO cellular pathway to lamella 14 in
1 million (ratio of rods: cones: ganglion cells = lateral geniculate body
120:6:1) Responsible for visual sensation of What do
Located in ganglion cell layer: I see?
Macula (more than one layer of ganglion At periphery:
cells) Rod: ganglion cell ratio may be up to
Fovea (piled eight layers high) 10,000:1
Foveola (absent) MAGNO cellular pathway to lamella 56 in
Second-order neurons lateral geniculate body
Synapse (connect bipolar cells to lateral Responsible for visual sensation of Where
geniculate body) do I see it?

2. Functions
At fovea:
Cone: ganglion cell ratio is 1:1
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Q What are the Functions of the Retinal Pigment Epithelium?
RPE is a single layer of cells interposed between Bruchs membrane/choroid and

the neurosensory layer.
RPE
1. Anatomy Active transport of water from subretinal

6 million (like cones!) space via ocular dipole

Single layer of cuboidal epithelium Embryogenesis (development of
Base (in contact with Bruchs membrane, photoreceptors)
extensive basal infoldings) Optical:
Apex (in contact with neurosensory layer, Absorption of stray light
extensive apical microvilli) Metabolic:

Vitamin A cycle (uptake, transport, storage,
Side (zona occludens for blood retinal barrier)
metabolism, re-isomerization of vitamin A)
Melanin granules (absorb light)
Transportation of materials to and from retina
2. Functions Phagocytosis (recognition, ingestion and
Physical: phagocytosis of shed outer segment)
Outer blood retinal barrier Detoxication
Adhesion to neurosensory retina:
Secretion of mucopolysaccharides
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TOPIC 2
THE VITREOUS
Q Opening Question: Tell me about the Anatomy of the Vitreous.

Vitreous
1. Gross anatomy Area of Martegiani:
Transparent viscoelastic gel Posterior funnel shaped region of Cloquets
Located behind lens and in front of retina canal; clinically seen as Weis ring with
4 ml (80% of volume of globe) and 4 g posterior vitreous detachment occurs
Shape is a sphere with anterior depression 2. Microscopic anatomy
(hyaloid fossa): Water content: 9899.7%
Central Cloquets canal PH: 7.5
Intermediate zone Refractive index: 1.33 (less than aqueous)
Vitreous cortex > 90% of visible light transmitted through vitreous
Various named regions: Acellular, normal vitreous cells restricted to
Hyaloideocapsular ligament (of Weiger): cortical layers
Annular region 2 mm wide and 8 mm in
Main constituent:
diameter where vitreous is attached to Type II collagen bers, which are entrapped
posterior lens capsule in hyaluronic acid (HA) molecules
Bergers space:
Collagen provides solid structure to vitreous,
Center of hyaloideocapsular ligament;
which is inated and stabilized by HA:
potential space behind posterior capsule If collagen is removed vitreous becomes
Cloquets canal:
viscous solution
Arises from Bergers space and courses
If HA is removed gel shrinks
posteriorly through central vitreous Large domains of HA spread apart collagen
bers to minimize light scattering
Exam tips:
The three most important sections here are the functions, attachments and
embryology. You may want to skip the rest!
Q What are the Functions of the Vitreous?
The vitreous has several functions .

Functions of the Vitreous
1. Mechanical function Shock absorbing function for lens and retina
Prevents globe from collapsing during eye movement and physical activity
Blunt trauma: Direct force is dissipated within
Viscoelastic property of HA-collagen interaction
vitreous
Prevents retinal detachment
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Majority of retinal breaks not associated with Vitreous ascorbic acid scavenger for free
RD: Sealed by post-vitreous cortex radicals from lens and retina metabolism
Simple RD: Intact, albeit detached HA acts as an anionic shield against
post-vitreous cortex requires only SB or potentially destructive electrons from ionizing
internal tamponade operations (e.g. radiation
pneumatic retinopexy), retinal breaks will be Movement of water and solutes within eye
sealed by intact vitreous cortex Transvitreous diffusion and bulk ow across
Complex RD: Derangement of post-vitreous retina involved in maintaining retinal
cortex more difcult to repair, increased attachment
risk of proliferative vitreoretinopathy 3. Optical functions
2. Metabolic functions Refractive index: 1.33 (same as aqueous)
Lens clarity and retinal function dependent on Transmits 90% of light between 3001,400 nm
presence of normal vitreous Optical transparency achieved by:
Oxygenation of interocular tissues HA-collagen interaction: Large HA molecules
Metabolic repository: separating collagen bers
Presence of glucose, galactose, mannose, Lack of macromolecular solutes: Molecular
fructose and amino acid sieve as a barrier to inux
Provides nutrients to retina in emergency 4. Role in accommodation
situations (e.g. ischemia) Supporting role to ciliary body: Vitreous may
Waste depository: push lens forward during accommodation
Physical depository for lactic acid, which is
(however, vitrectomized eyes can still
toxic to retina
accommodate)
Q What are the Attachments of the Vitreous?
The attachments can be physiological or pathological.

Attachment of Vitreous
1. Physiological attachments aqueous actually accumulates in this
The vitreous is adjacent to retina posteriorly and space!)
behind ciliary body and lens anteriorly Anterior to ora serrata, cortex attaches to BM of
Areas of attachment: ciliary body
Ora serrata (via the vitreous base at pars Posterior to ora serrata, cortex attaches to BM of
plana) Muller cells of the retina (i.e. internal limiting
Post lens capsule (via the hyaloideocapsular membrane or ILM)
ligament of Weiger) Complex of post-vitreous cortex and ILM acts
Optic disc margins (via Cloquets canal) as a molecular sieve, preventing cell
Retinal vessels (via vitreoretinovascular bands) inltration (when this is breached, formation
Macula (via attachment plaques) of epiretinal membrane)
At all sites, interface with adjacent tissues ILM is thin over macula but vitreous
consists of a complex formed by vitreous cortex attachment is strong (pathogenesis of macular
and basal membrane (BM) of adjacent cells hole)
Only region not adjacent to basal laminae is ILM is absent over optic disc (increased
peripheral annulus of anterior vitreous cortex frequency of neovascularization at optic disc)

Directly exposed to zonules and aqueous 2. Pathological attachments:
humor of posterior chamber (important in New vessels
malignant glaucoma pathogenesis where Lattice and other retinal degenerations
Exam tips:
This is a rather complex but fairly important topic for the pathogenesis of
most disorders.
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Q What is the Anatomy of the Zonules?
Zonules
1. Referred to as tertiary vitreous 3. Arise from ciliary processes to insert onto lens
2. Resemble collagen brils in terms of diameter: capsule via two bundles
More tightly packed Orbiculo-anterocapsular bundle
Resist collagenase digestion Orbiculo-posterocapsular bundle
Solubilized by alpha-chymotrypsin (basis of its Between the two the canal of Hannover
use in ICCE) Between orbiculo-posterocapsular bundle
Have amino acid composition that resembles and anterior vitreous cortex is canal of Petit
elastin
Q What is the Embryology of the Vitreous?
There are classically three overlapping phases of vitreous development.

Embryology of Vitreous
1. Primary vitreous (vascular vitreous) Bergmeisters papillae:
4th week of gestation: Early vitreous forms in Posterior portion of hyaloid artery with
space between lens plate and optic vesicle associated glial tissues
5th week: Optic ssure fuses, vitreous becomes Vitreous cysts:
Benign lesions with abnormal regression of
closed compartment
either anterior or post hyaloid vascular system
6th week: Hyaloid artery develops and reaches
Persistent fetal vasculature (formerly known as
post pole of lens vesicle
Persistent hyperplastic primary vitreous (PHPV)):
Primary vitreous atrophies during development of
Anterior or posterior manifestations
secondary vitreous, by 7th month, hyaloid artery
Abnormal regression and hyperplasia of
no longer carries blood and is resorbed at birth primary vitreous
2. Secondary vitreous (avascular vitreous) Adherent to post lens capsule and extends
6th week: Secondary vitreous formation begins laterally to ciliary processes
between the retina and posterior branches of 90% unilateral (although fellow eyes may
hyaloid vessels have Mittendorfs dot)
Essentially acellular, this consists of Usually retina not involved (posterior PFV is
extracellular matrix of type II collagen, with less common)

Important differential diagnosis of leukocoria
little HA at this stage
Signs:
Demarcation line between primary and
Persistent pupillary membrane
secondary vitreous becomes walls of Cloquets Iridohyaloid vessels: Curve around pupillary
canal margin causing atypical colobomas
3. Tertiary vitreous (zonules) brittle star posterior capsular opacity
Begins at 6th month, product of ciliary Mittendorfs dot
epithelium Persistant hyaloid vessel
Muscae volitantes
4. Anomalies of hyaloid vessel regression
Bergmeisters papilla
Mittendorfs dot:
Congenital non-adherence of the retina/
Post lens surface
congential retinal detachment (rare and
Site of anastomosis between hyaloid artery
severe form)
and tunica vasculosa lentis
Microphthalmia
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Q What are the Differences between Asteroid Hyalosis and Synchisis Scintillans?
Comparison Between Asteroid Hyalosis and Synchisis Scintillans
Asteroid hyalosis Synchisis scintillans

Etiology Vitreous bril degeneration of unknown Chronic vitreous hemorrhage
cause
Prevalence: 0.5% of population
Biochemical Calcium soaps Cholesterol crystals
structure
Clinical features Yellow-white spherical opacities Flat, refractile bodies, golden-brown
Absolute acoustic shadowing on B-scan
Associations Intimately associated with vitreous gel, Freely mobile, associated with liquid
move with vitreous displacement vitreous, settles in dependent portion
of eye
Q What are the Changes in the Vitreous with Age?
There are two distinct phases in the development of the vitreous .

Vitreous Changes with Age
1. Development of vitreous from childhood to adult In adult, alteration of HA-collagen interaction
Development not complete until eye reaches causes dissociation of the two components:
adult size Collagen brils aggregate with each other
Human embryonal vitreous is dense and scatters macroscopic bers seen clinically in adult
light (collagen and HA concentration low at vitreous coursing antero posteriorly
Pooling and redistribution of HA in areas
birth)
adjacent to collagen liquid vitreous in
With age, increasing concentration of collagen
between bers
and HA: Central vitreous rst to liquefy
HA separates collagen brils vitreous
Reduction in size of vitreous posterior
becomes less dense and optically more vitreous cortex detaches
transparent Escape of liquid vitreous into subhyaloid
HA concentration reaches adult levels by
space leads to posterior vitreous detachment
12 years of age (PVD)
Total collagen content also does not change
Abnormalities in retinal adhesions lead to
after 2030 years of age retinal traction (pathogenesis of macular
2. Posterior vitreous detachment pucker/ holes)
During childhood, vitreous is homogeneous and
collagen brils not seen
Exam tips:
Alternate question is What is the pathogenesis of posterior vitreous
detachment?
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TOPIC 3
RETINAL BREAKS AND

DEGENERATIONS
Q Opening Question: Tell me about Retinal Breaks.

A retinal break is a full-thickness defect in the neurosensory retina.
Retinal Breaks Classication:
1. Hole: 2. Tear
Atrophic
Operculated
Q When do You Need to Treat Retinal Breaks?
Not all retinal breaks need to be treated.

Treatment depends on the characteristics of the break, the type of eye and the type of patient.
Indications for Treatment of Retinal Break
1. Type of break: Aphakic or pseudophakic eye
Acute, symptomatic (absolute indication) High myopia
Associated with subclinical RD Vitreoretinopathy (e.g. Wagners syndrome)
U-shaped tear (absolute indication) 3. Type of patient:
Large (relative indication) Family history of RD
Superotemporal or posterior location (relative Systemic conditions (Marfans, Sticklers,
indication) Ehlers-Danlos syndrome)
Absent pigments (relative indication) No access to health care
2. Type of eye: Not compliant to follow-up
Only eye High risk occupation
Fellow eye has history of RD
Q Tell me about Retinal Degenerations.
Retinal degenerations can be broadly divided into benign degenerations and those associated with
higher risks of RD.
Retinal Degenerations
1. Benign degenerations: Snowakes
Retinal hyperplasia/hypertrophy Pavingstone degenerations
Microcystoid changes Peripheral drusens
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2. Degenerations with increased risks of RD: White with pressure and white without pressure
Lattice degenerations (see below) (associated with giant retinal tear)
Acquired retinoschisis (see below) Cystic retinal tufts
Q When do You Need to Treat Retinal Degenerations?
Indications for Treatment of Retinal

Degenerations
1. White without pressure fellow eye with giant 3. Lattice degeneration:
retinal tear Associated with retinal breaks
Associated with other risk factors (type of eye,
2. Retinoschisis 2-layer retinal break plus risk
type of patient)
factors
Q Tell me about Lattice Degeneration.
Lattice degeneration is a common retinal degeneration.

Lattice Degeneration
1. Epidemiology Adherence of vitreous to edge of lattice
810% of general population (but 2040% of (posterior edge)
RD) Sclerosis of retinal vessels
Higher frequency in myopia, Marfans 3. Clinical features
syndrome, Ehlers-Danlos syndrome Well dened areas of retinal thinning
2. Pathology Circumferentially orientated
Discontinuity of internal limiting membrane Location:
Atrophy of inner layers of retina Between equator and ora serrata
Overlying pocket of liqueed vitreous Temporal
Superior
Q How do You Distinguish Acquired Retinoschisis from Retinal Detachment?
Acquired retinoschisis is a retinal degeneration in which .

The retina is split into two layers, an outer choroidal layer and an inner vitreous layer .
The typical type involves a split in the plexiform layer, while the reticular type involves a split
in the nerve ber layer.
Acquired Retinoschisis
Retinal detachment Acquired retinoschisis

High risk group Myopia Hypermetropia
Location Superior temporal Inferior temporal
Scotoma Relative Absolute
Pigments Present Absent
Surface Corrugated Smooth
Shifting uid May be present Absent
Reaction to photocoagulation No reaction Present
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Exam tips:
The typical type is split in the plexiform layer while the reticular type is split
in the nerve ber layer.
Q What is Proliferative Vitreoretinopathy?
Proliferative vitreoretinopathy (PVR) is the commonest cause of late failure after RD operation.
Proliferative Vitreoretinopathy
1. Pathology Grade B
Retinal tear or detachment causes break in Wrinkling of inner retina
inner limiting membrane and blood retinal Retinal stiffness
barrier Vessel tortuosity
Rolled edge of retinal break
RPE cells migrate into vitreous
Decreased vitreous mobility
RPE cells proliferate and transform into
Grade C
myobroblasts
Full thickness retinal folds:
Further stimulus for migration and proliferation
Focal
from blood derived products Diffuse
RPE cells release transforming growth factors Circumferential
(TGF) which stimulates brosis and collagen Subretinal
production Anterior
Membranes contract (in anteroposterior and Dened as either anterior or posterior and by
tangential directions) and this leads to tractional number of clock hours
RD 4. Treatment
2. Risk factors Surgery usually required:
RD factors: Pneumoretinopexy/scleral buckling (grade B
Retinal break (large and multiple) and below)
Associated vitreous hemorrhage and Vitrectomy usually required
inammation Pharmacological adjuncts: 5FU, low molecular
Re-detachment weight heparin (not shown to be effective)
Iatrogenic factors: Timing of surgery:
Excessive cryotherapy or laser Balance between threat to macula (if still
photocoagulation attached) and easier and more complete
Use of viscoelastic, gas or silicone oil removal of mature membranes
Iris trauma In general, if macula attached and chance
3. Classication (know the differences between the for preservation of useful vision early
surgery
Retina Society and Silicone Study grading
If macular chronically detached, better to
schemes) await membrane maturation (48 weeks)
Grade A before surgery
Vitreous haze or pigment clumps
Q What is the Silicone Study?
The Silicone study was randomized trial to compare the use of silicone oil (SO) vs gas
in the treatment of PVR.
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Silicone Study (Arch Ophthalmol, 1992;110:770

and 780)
1. Aim: 2. Conclusions:
Study effect of SO vs gas in treatment of PVR SO more effective than SF6
Cases had rhegmatogenous RD and silicone SO and C3F8 equally effective in maintaining
retinal attachment
study PVR grade C-3 and above
Hypotony higher in C3F8
Report 1: SO vs SF6
Keratopathy same between SO and C3F8
Report 2: SO vs C3F8
Re-detachment occurs in 20% of eyes after SO
removal
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TOPIC 4
RETINAL DETACHMENT
SURGERY
Q Opening Question: What are the Principles of Retinal Detachment Surgery?

The principles of RD surgery are .
Principles of RD Surgery
1. Find all retinal breaks Laser photocoagulation
Indirect ophthalmoscopy with scleral 3. Drain subretinal uid (SRF) if necessary
indentation 4. Relieve vitreoretinal traction
Based on Lincoffs rule (see below) Scleral buckle OR
2. Seal all retinal breaks Vitrectomy OR
Cryopexy OR Pneumatic retinopexy
Exam tips:
These principles are shown step-by-step in the scleral buckles section
(page 158)
Q What are Lincoffs Rules?
Lincoffs rules are a set of guidelines on nding the retinal break based on the conguration of the RD.
Based on retrospective analysis of 1,000 cases of RD.
Only applicable to primary Rhegmatogenous RD (96% of cases conformed to these rules).
Lincoffs Rules (Arch Ophthalmol 1971;85:56569)
1. Lateral RD (inferior RD with SRF higher on one 3. Inferior RD (inferior RD with equal SRF on
side of the disc) (98%) both sides of disc) (95%)
Break is at one and a half clock hours from Break is inferior, at 6 oclock position
higher side of RD 4. Inferior bullous RD
2. Superior RD (SRF crosses the vertical midline Break is on higher side of RD, above horizontal
above disc) (93%) meridian, with SRF tracking inferiorly
Break is superior, within a triangle with its apex
at 12 oclock at ora serrata and base at 11 and 1
oclock at equator
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Q What is Cryotherapy?
Cryotherapy is the treatment technique involving the use of cold temperature.

Cryotherapy
1. Mechanism of action Small pupil
Freezing temperature causes conversion of Peripheral retina which cannot be treated
liquids to solids, and intracellular and adequately with lasers
Opaque media
extracellular water to ice
Ciliary body (cyclodestructive procedures for
This leads to tissue death and a sterile
glaucoma)
inammatory reaction
Cataract extraction (ICCE)
2. Cryoprobe
Destruction (lid and intraocular tumors,
Probe temperature is between 40oC (carbon
trichiasis)
dioxide) or 70oC (liquid nitrogen), temperature
4. Procedure:
at tissue around 10C
Check cryoprobe by freezing and unfreezing a
Thermal energy is absorbed by rapid expansion
few times
of carbon dioxide and liquid nitrogen into
Place probe over scleral area with indirect
gaseous state (Jules-Thomson effect)
ophthalmoscopic view of retinal break
Expansion occurs at gas tip with an exhaust
Initiate freezing
system drawing away gas
Observe for whitening of retina
An insulation compartment limits freezing to tip
Spray water on cryoprobe and wait for complete
of probe
melting of ice-ball before removing from sclera
Heater wire in probe defrosts tip after each
Thaw and repeat again
freeze
Retinal adhesion takes about two weeks to gain 5. Complications:
strength (slower than laser retinopexy) Early:
Pain and chemosis
3. Indications in ophthalmology:
Conjunctival brosis (increase risk of
Adhesion (retinal breaks) trabeculectomy failure)
When would you prefer to use cryopexy
Vitritis
instead of laser photocoagulation? CME
Small pupil
Late:
Peripheral retina which cannot be treated
PVR
adequately with lasers
ERM
Opaque media
Diplopia from muscle injury
Blood vessels (PDR new vessels, telangiectasia Scleral necrosis
of Coats disease) Depigmentation (cutaneous application)
When would you prefer to use cryopexy for
PDR? (same as for retinal breaks)
Exam tips:
The indications for cryotherapy are ABCD.
Q When and How do You Perform Subretinal Fluid (SRF) Drainage?
SRF drainage is not an essential part of RD surgery.

This is because in most cases, SRF will usually be absorbed spontaneously with adequate support and
sealing of the retinal breaks.
SRF drainage can be divided into internal or external drainage.
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SRF Drainage
1. Indications Use either 27G needle or endolaser to puncture
RD is: choroid at an oblique angle:
Bullous (unable to appose retina for adequate Controlled SRF drainage with cotton tips and
retinopexy) nger
Long-standing (viscid SRF) Milk out viscid SRF if necessary
Immobile because of PVR Check for attening of retina:
Inferior (usually bullous, long-standing and Check for hypotony (have syringe of air or
breaks cannot be localized) saline on standby)
Break cannot be localized or sealed Suture lips with 5/0 vicryl
Patient factors: 3. Complications
Elderly (less efciency of RPE pump) Hypotony (commonest complication):
Preexisting glaucoma Choroidal folds
Undergone recent cataract surgery Macular and disc edema
Thin sclera Corneal edema
2. Procedure Suprachoroidal hemorrhage (most dangerous
Choose site of drainage complication)
Cauterize site of drainage Iatrogenic break formation
Incise sclera radially until choroid can be seen Retinal prolapse and incarceration
Cauterize lips of incision, pre-place 5/0 vicryl Vitreous prolapse
sutures to lips Postoperative endophthalmus
Notes
Where would you choose your site of drainage?
Above or below horizontal recti (avoid vortex veins near the vertical
recti)
Between ora and equator
Temporal retina (usually more accessible)
Away from break (less risk of vitreous incarceration)
At the point where RD is most bullous
In the bed of the scleral buckle
Q Tell me about Scleral Buckles.
Scleral buckles (SB) are devices to relieve vitreoretinal traction in RD surgery.

They can be divided into .
Scleral Buckles
1. Classication: Band
Radial Usually for encirclage
Segmental circumferential Hydrogels:

Encirclage circumferential Soft and elastic, nontoxic and nonpyogenic,
imbibe uid postoperatively to increase
2. Materials
tension but more expensive than silicon
Silicon: No longer in use due to high risk of long-term
Tires
complications continued swelling leads to
Provide even indentation, low risk of
mass effect, diplopia, infections
infection, extrusion or migration
Gelatin
Sponges
Temporary (lasts 3 to 6 months)
Imbibe uid postoperatively to increase
tension, but more complications
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3. Factors affecting choice of SB: Dissect tenons and isolate recti with squint
Retinal break (size, number and location) hook
Distribution of SRF Sling recti with 5/0 silk suture
Amount of vitreous traction and PVR Position buckle beneath recti
Phakic status Localized all breaks with indirect
Available eye volume ophthalmoscopy and scleral indentation
State of sclera (Principle No. 1)
4. Indications for radial SB Seal all breaks with cryopexy or indirect laser
Usually in two situations: (Principle No. 2)
Large U-shaped tears with shmouthing Decide whether to perform SRF drainage
Posterior breaks (Principle No. 3)
5. Indications for segmental SB Relieve vitreoretinal traction by suturing SB with
Standard buckles for most RD: 8/0 nylon (Principle No. 4)
Small medium size breaks in single Check for position of buckle and check
location pulsation of central retinal artery (to exclude
Multiple small medium size breaks in one CRAO)
or two quadrants Close conjunctiva with 8/0 vicryl
6. Indications for encirclage SB 8. Complications
Used for more complicated RD (although Intraoperative:
some surgeons use this routinely): Scleral perforation
Large breaks and multiple breaks in three or Rectus muscle trauma
more quadrants Complications of SRF drainage
Extensive RD without detectable breaks Conjunctival buttonholing, tears
Mild PVR Complications of gas/SO tamponade
Aphakic RD Postoperative:
Fish-mouthing (large breaks, high buckles)
Lattice degeneration in three or more quadrants
and posterior slippage (GRT)
Excessive drainage of SRF
Extrusion, exposure
Failed segmental buckle without apparent
Refractive changes: myopia, astigmatism
reason Anterior segment ischemia
7. Procedure: Diplopia/motility problems
GA or LA Glaucoma (vortex vein compression)
Conjunctival peritomy 360o or limited peritomy
Exam tips:
The principles of RD surgery (page 155) are shown in the procedures section.
Q What are the Indications for Vitrectomy in Retinal Detachment?
The indication for vitrectomy can be divided into uncomplicated RD and complicated RD.
Indications for Vitrectomy in RD
1. Rhegmatogenous RD Complicated RD:
Uncomplicated: Severe proliferative vitreoretinopathy grade
Posterior breaks and macular holes C or more
Multiple breaks in different meridians Giant retinal tear
Associated vitreous hemorrhage 2. Tractional RD threatening fovea
Controversial (high myopes, pseudophakics,
bullous superior RD with no breaks seen)
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Q What is Pneumoretinopexy?
Pneumoretinopexy is a form of RD surgery.

It is indicated for .
Pneumoretinopexy
1. Principles: If macula threatened: Steamroller technique
Works by intravitreal injection of an expansile (position patient such that gas bubble
volume of gas (100% concentration) tamponades macula rst, then slowly reposition
0.6 ml of 100% SF6 will give 1.2 ml after full bubble over break)
expansion 4. Advantages:
0.3 ml of 100% C3F8 will give 1.2 ml after full
No hospitalization
expansion
No complications of SB
The retinal break is sealed with tamponade from
Minimal tissue trauma
buoyancy and surface tension of gas (see
Shown to be as effective as SB (~80%
intraocular gas, page 161)
reattachment rate with single procedure in
2. Indications: both)
Retinal breaks in superior 8 oclock hours
5. Disadvantages and complications:
Not indicated when there is:
Failure of retinal attachment
Preexisting glaucoma
Grade C PVR
New retinal tears/breaks (up to 20%)
Hazy media Risk of glaucoma and CRAO
Unable to move or travel by air Vitreous hemorrhage and vitreous
3. Procedure: incarceration
Injection site: Pars plana Gas can migrate into subretinal space with
Aim to inject gas into cortical vitreous (prevent extension of RD
sub-hyaloid gas), forming single large bubble
(avoid sh-eggs)
Q What are the Complications of RD Surgery?
Complications of RD Surgery
1. Early: 2. Late:
Missed breaks and re-detachment (commonest PVR (commonest cause of LATE failure)
cause of EARLY failure) Induced refractive error
Acute ACG (forward displacement and Diplopia
congestion of ciliary body) Scleral buckle problems (infection, dislocation
Anterior segment ischemia and extrusion)
Vitritis (usually from cryopexy) CME
Choroidal detachment (hypotony usually from ERM
SRF drainage)
Endophthalmitis (SRF drain)
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TOPIC 5
VITRECTOMY AND
VITREOUS REPLACEMENT
Q Opening Question: What are the Indications for Vitrectomy?

Vitrectomy can be used for either therapeutic or diagnostic purposes.
Common indications may include .
Indications for Vitrectomy
1. Complicated retinal detachments 5. Macular diseases:
Rhegmatogenous RD Epiretinal membrane (ERM)
Uncomplicated RD: Macular hole
Posterior breaks and macular holes
6. Complications of anterior segment surgery:
Multiple breaks in different meridians
Postoperative endophthalmitis
Associated vitreous hemorrhage
Dropped nucleus
Controversial (high myopes,
pseudophakics, bullous superior RD with Massive expulsive hemorrhage

no breaks seen) Malignant glaucoma
Complicated RD: 7. Chronic posterior segment inammation/vitritis:
Severe proliferative vitreoretinopathy grade Diagnostic vitrectomy
C or more
Giant retinal tear
Recent Developments in Vitrectomy
Techniques
Tractional RD threatening fovea
1. Vitrectomy instrumentation
2. Advanced diabetic retinopathy (see page 192)
23G and 25G vitrectomy:
3. Other proliferative vitreoretinopathies
Self-sealing sclerostomies faster recovery
4. Severe ocular trauma and intraocular foreign Better fuildics (smaller bore lower
body (IOFB): likelihood of aspirating retina)
Associated with endophthalmitis Chandelier illumination: Self-retaining
IOFB impacted on retina illumination allowing bimanual surgery
No view of IOFB (e.g. vitreous hemorrhage) 2. Chemical vitrectomy
Large, nonmagnetic or organic IOFB Vitrase: Bovine hyaluronidase
Shown to be safe and effective in clearing
vitreous hemorrhage in two multinational
RCTs with > 1,100 patients
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Q What are the Complications of Vitrectomy?
Complications of Vitrectomy
Intraoperative Postoperative
Retinal break Retinal break
Suction near mobile retina Intraocular hemorrhage
Intraocular hemorrhage Choroidal hemorrhage
Suprachoroidal hemorrhage Vitreous hemorrhage
Vitreous hemorrhage Cataract
Cataract Glaucoma
Lens trauma with instruments Cornea
Raised IOP Recurrent corneal erosion
Infusion bottle too high Filamentary keratitis
Decreased IOP Bullous keratopathy
Infusion bottle too low Phthsis bulbi
Miosis of pupils Endophthalmitis
Subretinal infusion Failure of surgery
Q What are Vitreous Substitutes?
They are substances injected into the vitreous cavity during a vitrectomy.
The main purpose is either for volume replacement or as a tamponade.
The common vitreous substitutes include .
Vitreous Substitutes
1. Ideal substitute (important): 2. Classication:
Clear/transparent Intraocular gas
Inert/nontoxic Saline
Low viscosity Silicone oil
Immiscible with H2O Heavy liquids
Durable/slowly absorbed
Exam tips:
The ideal substitute has a LIST of properties that can be used for ALL the
individual substitutes and therefore is well worth to be remembered.
Q Tell me about Intraocular Gases.
Intraocular gases are common vitreous substitutes .

They can be divided into.
The common indications are for either volume replacement (nonexpansile volume) or as a tamponade
(expansile volume) .
Intraocular Gas
1. Classication: 2. Biomechanical properties
Nonexpansile Physical properties:
Air, helium, argon, nitrogen, xenon Properties of ideal substitute (page 161)
Expansile Clear/transparent
Sulphur hexaoride (SF6), peruoreopropane Inert/nontoxic
(C3F8), C2F6, C4F10 Low viscosity
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Immiscible with water

Maximum at 68 hours (therefore be
Durable/slowly absorbed PLUS
careful of CRAO during this time)
High surface tension Equilibrium
Bubble of gas does not enter retinal break Nitrogen last to diffuse
and prevents uid from entering break Maximal expansion when gas diffusion
High buoyancy in = diffusion out

10 times greater force than SO Dissolution
Force maximal at apex of angle Exponential decline
Dynamic properties Depends on size and water solubility
Three phases: First order kinetics bubble has useful
Expansion ARC of contact for approximately 25% of

Oxygen, carbon dioxide diffuses in total duration of gas in eye
3. Comparison of air, SF6, C3F8
Time of maximal Time to maximal Expansion Nonexpansile

Gas Duration expansion rate expansion volume concentration
Air 5 days NA NA 1 NA
SF6 2 weeks 68 hours 2448 hours 2 20%
C2F6 3 weeks 68 hours 4872 hours 3 18%
C3F8 2 months 68 hours (same as 7296 hours 4 15%
SF6)
4. Indications for SF6 or C3F8 5. Complications:

Volume replacement after vitrectomy Glaucoma and CRAO
(nonexpansile volume) Especially during maximum rate of expansion
Indications for vitrectomy (see page 160) (68 hours after operation)
Tamponade retina (expansile volume): Cataract (posterior subcapsular feathery
Adjunct to RD surgery (posterior breaks or cataract)
macular holes) Bullous keratopathy
Adjunct to DRF drainage New/enlarged breaks
Selected giant retinal tear Subretinal seepage
Flatten radial folds on a high buckle
Dislocation of IOLs
Pneumoretinopexy (use 100% gas without
PVR
vitrectomy; see page 159)
Q Tell me about Silicone Oil.
Silicone oil is a vitreous substitute.

It has the following properties .
Silicone Oil (SO)
1. Properties: Refractive index close to vitreous:
Properties of ideal substitute (page 161) Acts as plus lens in aphakic eyes
Clear/transparent Acts as minus lens in phakic eyes
Inert/nontoxic High viscosity
Low viscosity 1,00030,000 centistokes (water = 1
Immiscible with water centistoke)
Surface tension of SO however lower than Lighter than water (0.93G)
gases tendency to emulsify Heavy silicone oil (denser than water)
Durable/not absorbed PLUS occasionally used for inferior detachments
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2. Indications: Air travel not contraindicated

Long-lasting volume replacement following Lower risk of hypotony
vitrectomy Control over timing of repeat surgery
Common indications: 4. Complications
PVR Glaucoma:
Giant retinal tear ACG (from pupil block, therefore inferior
Intraoperative control of vitreous iridectomy (Ando) is usually needed)
hemorrhage Delayed OAG (from emulsication)
Elderly patient who cannot posture Hypotony (not common)
One-eyed patient who needs immediate good Cataract
vision postoperation Filamentary keratitis, band keratopathy
Patient who needs to travel by air
Emulsication of SO:
3. Advantages Uveitis
What are the advantages of SO over intraocular Subretinal seepage
gases? Subconjunctival cyst formation
Intraoperative advantages: Retinal toxicity? (ERG and EOG detected
Better intraoperative visualization abnormalities)
Easier retinopexy ERM/retro-silicone proliferation:
Control of hemorrhage and effusion Accumulation of growth factors,
Postoperative advantages: inammatory debris at lower meniscus of
Longer-lasting tamponade bubble
Posturing less critical Cause of recurrent epiretinal proliferation
Better immediate VA Recurrent RD (2540%)
Q What are Heavy Liquids?
Heavy liquids are vitreous substitutes used as intraoperative tools.

They are essentially extension of peruorocarbon gases with seven or more carbon atoms (and therefore
liquid at room and body temperature).
Heavy Liquids
1. Properties 3. Indications
Properties of the ideal substitute (page 161): Intraoperative tool for complicated VR surgery
Clear/transparent (not for prolonged tamponade):
Inert/nontoxic (with short-term use) PVR
Low viscosity (0.88 centistokes) Giant retinal tear
Immiscible with water Subluxed/dislocated lens
Durable/slowly absorbed PLUS IOFB
High specic gravity (2G, two times more than Subretinal macular hem
water or SO) Traumatic RD
High tamponade force (4G, like gas and 10
times more than SO)
2. Examples
Peruorodecalin (C10F18)
Peruoro-N-octane (C8F18)
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Section 5
M Retina
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TOPIC 1
THE MACULA
Q Opening Question: What is the Anatomy of the Macula?

The macula is an area in the posterior pole of the fundus.
Anatomy of the Macula

1. Macula Thick internal limiting membrane
Location: Area bounded by temporal arcades, 3. Foveola
4 mm temporal, 0.8 mm inferior to optic disc Location: Central oor of fovea
Size: 5 mm in diameter/3.5 disc diameter/18o of Size: 0.35 mm in diameter/0.2 disc
visual angle diameter/0.54 minutes of visual angle
Histologically, Histologically,
> 1 layer of ganglion cells Thinnest part of retina
Xanthophyll pigments No ganglion cells or rods
2. Fovea Only cones: 150,000/mm2
Location: Depression inside macula 4. Foveal avascular zone (FAZ)
Size: 1.5 mm in diameter/1 disc diameter/5o of Location: Area bounded by fovea and foveola
visual angle Histologically (accounts for darkness in FFA),
Histologically, Avascular
68 layers of ganglion cells Tall RPE cells with increased melanin
Tall RPE cells Increased xanthophyll pigments
Q How do You Evaluate Macular Function?
The macula can be assessed clinically and with appropriate tests.
Macular Function
1. Clinical Standard chart with 10 cm large square and
VA 5 mm small square
Color vision and pupil (should be normal unless Chart should be read at 1/3 of meter (small
the macular is extensively damaged) square subtends 1)
Binocular ophthalmoscopy and slit lamp Seven charts in total:
Chart 1: Standard chart
biomicroscopy (with 78D or 90D lens)
Chart 2: Diagonal lines to help central
Confrontational VF and light projection
xation, when central scotoma is
2. Ancillary tests present
Amsler grid: Chart 3: Standard chart, but red lines on
Screening test for macular function black background, for color scotoma
Evaluates 20 of visual angle (macular Chart 4: No lines, only dots, reveals only
subtends only 18) scotoma
Section 5: Medical Retina 197
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Chart 5: Parallel horizontal lines, to show Patient should see 15 or more white blood
metamorphopsia cells in entire area
Chart 6: Similar to Chart 5, but with ner Abnormal macular function:
horizontal lines in the central area No corpuscles/decreased number of
Chart 7: Similar to Chart 1, but with ner corpuscles

lines in central area Slow speed of corpuscles
Photostress test: No corpuscles in a specic area
Principle of the dark adaptation test (evaluate Laser interferometer:

recovery time of photoreceptors to Principle of interference
re-synthesize visual pigments) Two coherent light beams creates fringe
Procedure: pattern (black and bright bands) by process of
Snellen VA assessed interference
Patient xates on torch light for 10 seconds Fringe pattern in different orientations and
Photostress recovery time = time taken to progressively ner gratings are used to
read Snellen letters one line above the estimate VA and macular function
pre-test level (normal: 30 seconds) Potential acuity meter:
Compare with other eye Projection of a miniature Snellen acuity chart
Flying corpuscle test: into retina, through a clear area of cataract or
Principle of the entoptic phenomenon other media opacities
(subjective perception of white blood cells Usually best for VA < 20/200
moving in perifoveal capillaries)
Procedure:
Patient asked to look into blue light of
entoptoscope
Exam tips:
An alternate question may be, How do you assess the macular function in a
patient with a dense cataract?
Remember to talk about the clinical examination rst (what you would
normally do in your daily practice), before going on to the more esoteric tests.
B1037_Ch-05.indd 198 12/28/2010 9:35:22 AM

TOPIC 2
FUNDAL FLUORESCEIN
ANGIOGRAPHY
Q Opening Question: What are the Clinical Uses of Fluorescein?

Clinical Uses of Fluorescein
1. Lacrimal system Siedals test (wound leak)
Tear break-up time (dry eyes) Contact lens tting (assess contact lens t)
Jones test: Dye disappearance test (blockage of 3. Anterior chamber
lacrimal system) Detect iris neovascularization
2. Cornea Applanation tonometry
Detect epithelial defect (corneal abrasion, 4. Retina
supercial punctate keratopathy) FFA
Exam tips:
Fundal uorescein angiography (FFA) is NOT the only use of uorescein!
Q What are the Principles of Fundal Fluorescein Angiography (FFA)?
Fundal uorescein angiography is based on two principles.

The principle of uorescence, which is the ability of.
And the principle of the blood-retinal barrier, which consists of.
Principles of FFA
1. Fluorescence through a blue excitation lter, which allows
Fluorescence: Ability of a substance to emit only blue light to enter eye
light energy of a longer wavelength (emission Interaction of blue light with uorescein
wavelength) when stimulated by light of a molecules in blood vessels, with emission of
shorter wavelength (excitation wavelength) yellow-green light (uorescence)
Yellow-green and reected blue light travel
Fluorescein:
out of retina to camera, passes through blue
Excitation wavelength peak: 490 nm (blue)
interference lter, allowing only yellow-green
Emission wavelength peak: 530 nm (green)
light to be imaged onto lm
Basic FFA:
White light from retinal camera passes
B1037_Ch-05.indd 199 12/28/2010 9:35:22 AM

2. Blood-retinal barrier (BRB) On the other hand, there is leakage of free

Inner blood-retinal barrier: Tight junctions of uorescein from choroidal vessels, but outer
retinal capillary endothelial cells BRB prevents free uorescein from traveling
Outer blood-retinal barrier: Tight junction of across RPE into sensory retina
RPE Therefore, leakage of uorescein from either
When uorescein is injected into blood stream, retinal vessel or RPE is abnormal
inner BRB prevents leakage of uorescein and
allows retinal vessels to be seen
Notes
What is autouorescence?
Ability of a substance to emit yellow-green light when stimulated by
blue light in the absence of uorescein
Classic example: Drusens
Notes
What is pseudouorescence?
Ability of substance to emit yellow-green light when stimulated by blue
light in the presence of mismatched lters
Q What are the Indications for FFA?
FFA is useful as an aid in the diagnosis and management of various posterior segment diseases.
Indications for FFA

1. Aid in diagnosis of: Optic nerve disorders:
Macular diseases: Disc drusens, papilledema, optic neuritis
AMD, central serous retinopathy, CME Tumors:
Retinal vascular diseases: Choroidal hemangiomas
Neovascularization in DR, CRVO, BRVO, 2. Aid in laser treatment of:
retinal telangiectasia CNV, central serous retinopathy, DM
Inammatory retinal/choroidal diseases: maculopathy, CRVO and BRVO
Posterior uveitis, CMV retinitis
Q What are the Side Effects of FFA?
FFA is usually a safe procedure with few side effects.

1. Mild and more common side effects: 2. Serious but rare side effects:
Nausea and vomiting Anaphylactic shock
Yellow discoloration of skin, urine and tears Airway spasm
Pain at injection site (especially if there is Collapse and death
extravasation)
Rashes
B1037_Ch-05.indd 200 12/28/2010 9:35:22 AM

Notes
Resuscitation for anaphylactic shock
Secure the airway, give 100% oxygen (intubate if respiratory obstruction is
imminent)
IM adrenaline 0.5 mg (repeat every 5 min if needed)
Secure IV access
IV chlorpheniramine 10 mg and hydrocortisone 200 mg
IV normal saline: Titrate against blood pressure
May require ventilator support
Q Tell me about the Normal FFA. What Abnormalities can FFA Detect?
The abnormalities are either related to hyperuorescence or hypouorescence.

Results of FFA
1. Normal FFA Occurs early in FFA
Phase 1: Pre-arterial phase/choroidal lling Leakage (increase in size and intensity of
Arm-retina time: 912 seconds hyperuorescence) from:
Phase 2: Arterial phase Choroidal vessels occur early (e.g. choroidal
One second after Phase 1 neovascularization)
Phase 3: Arteriovenous phase/capillary phase Retinal vessels occur late (e.g. NVD in DR)
Complete lling of arterioles and capillaries Optic nerve head occurs late (e.g.
Early venous lling (lamellar ow) papilledema)
Phase 4: Venous phase Staining (dye in tissue):
Complete venous lling Retinal scars, sclera, abnormal vessels
Recirculation of dye Occurs late
Intensity of uorescence decreases 3. Cause of hypouorescence
2. Cause of hyperuorescence Masking (blockage of dye transmission from
Window defect (transmission of dye from choroid):
choroid): Retinal hemorrhages
Atrophy or destruction of RPE cells (e.g. Edema and hard exudates
AMD), RPE tear Pigments (melanin and xanthophyll)
Occurs early in FFA Lipofuscin (e.g. Best disease)
Pooling (increase in intensity of Filling defect (delay of lling or occlusion of
hyperuorescence but not in size): vessels):
Dye in sub-RPE space (e.g. pigment epithelial Retinal ischemia (e.g. CRVO, DR)
detachment) Choroidal ischemia (e.g. HPT retinopathy)
Dye in sub-retinal space (e.g. exudative RD) Retinal atrophy (e.g. myopia)
Notes
Why is the fovea dark?
Blockage of choroidal uorescein by RPE
Increases: Melanin pigments in RPE
Increases: Height of RPE cells
Increased xanthophyll pigments

Avascular
B1037_Ch-05.indd 201 12/28/2010 9:35:22 AM

Q Tell me about Indocyanine Green (ICG) Angiography.
ICG is a complementary test to the FFA in the diagnosis and management of various posterior segment
diseases.
Indocyanine Green Angiography
1. Advantages over FFA 2. Indications
ICG is highly bound to plasma proteins (98%) AMD:
as compared with FFA (80%) Occult and recurrent CNV
Choroidal circulation is more easily seen PED
ICG has maximum absorption at 805 nm and Submacular hemorrhage
uorescence at 835 nm Inammatory choroidal diseases:

Wavelength of ICG can penetrate RPE and White dot syndromes
macular xanthophyll better Vogt-Koyanagi-Harada syndrome
Tumors:
Choroidal melanoma
B1037_Ch-05.indd 202 12/28/2010 9:35:23 AM

TOPIC 3
OPTICAL COHERENCE
TOMOGRAPHY
Q Opening Question: What is Optical Coherence Tomography?

OCT is a low coherence interferometry which allows non-contact in vivo imaging of ocular tissues at a
high resolution.
OCT
1. Principle Frequency domain OCT:
Works as an optical ultrasound, by using light Spectral/Fourier domain OCT
reections to provide cross-sectional images Mirrors not used, all wavelengths of returning
light analyzed simultaneously
2. Types
Acquires images at 20,000 axial scans/
Time domain OCT:
second
Mirrors used to scan tissue and provide
Less artifacts and improved quality of
cross-sectional image of optic nerve
images
Acquires images at 400 axial scans/second
Axial resolution 5 m
Artifacts from eye movement
More expensive
Axial resolution of 10 m
More widely used
Q What are the Applications of Optical Coherence Tomography in Ophthalmology?
OCT is widely used in ophthalmology.

It is used to image the macula both the anterior and posterior segment.
Applications of OCT
1. Posterior segment RNFL:
Macula: Reduced in:
CSME Glaucoma
AMD and PCV: Macular edema, PED High myopia
CSR Increased in:

CME Swollen disc
ERM and VMT 2. Anterior segment

Macular hole AC angles: Open or closed
RPE atrophy
Mechanisms of angle closure: Pupil block, angle
Maculoschisis
crowding, bulky lens
B1037_Ch-05.indd 203 12/28/2010 9:35:23 AM

TOPIC 4
ELECTROPHYSIOLOGY
Q Opening Question: What is the Electroretinogram, Electrooculogram and Visual Evoked Potential?
ERG = Measure of electrical mass response of VEP = Measure of electrical response of the
the retina, reecting electrical activity of occipital visual cortex to stimulation of the
photoreceptor and bipolar cells retina with either light or pattern stimulus,
EOG = Measure of electrical mass response of reecting activity of the entire visual system
the eye, reecting the metabolic activity of the (from retina, especially macula to cortex)
RPE
Exam tips:
A clear denition of the three main types of electrophysiological tests is
important.
Q Tell me about the Principles of Electroretinography.
Electroretinography (ERG)
1. Anatomical basis and abnormal cone ERG implies
Rods widespread retinal disorder)
Distribution: Sensitivity:
120 million Maximal in photopic conditions
Absent in foveola, increase to peak density Maximal to green-yellow light
15 from foveola center, then decrease Able to follow icker greater than 8 to 10
slightly towards periphery cycles/second

Sensitivity: 2. Normal ERG waveform
Maximal in scotopic conditions
A-wave:
Maximal to blue green light
Negative waveform
Unable to follow icker greater than 8 to
Photoreceptor hyper-polarization when
10 cycles/second (longer refractory period) exposed to light stimulus
Cones Reects photoreceptor function
Distribution: B-wave:
6 million
Positive waveform
Peak density in foveola, decrease in
Midretinal cells, initiated by bipolar cells,
density 15o from foveola center, lowest magnied by Mller cells
density in peripheral retina Reects Mller cells and bipolar cells
However, majority of cones lie outside
C-wave:
fovea (therefore localized disease of the Occasionally present
fovea will still result in a normal cone ERG Reects RPE cells
B1037_Ch-05.indd 205 12/28/2010 9:35:23 AM

Oscillatory potential: Interaction between Amacrine and

Wavelets on rising B-wave Interplexiform cells
Reects primarily cone function
Q What are the Types of ERG and How do You Use/Apply Each of Them?
Types of ERG
1. Equipment Dark adapted ERG:
Stimulator: Reects rod function
Ganzfeld stimulator, diffuse illumination of Dark adaptation (for 20 to 30 minutes)
entire retina Low intensity blue ash or low intensity
Electrodes: white ash stimulus
Active electrode (contact lens electrode, gold Absent/minimal A-wave
foil lid electrode) Light adapted ERG:
Reference electrode (forehead) Reects cone function
Ground electrode (earlobe) Light adaptation (for 10 minutes)
Amplication and display system Bright ash stimulus
Waveform amplitude is about 30% smaller
2. ERG waveform measurement
Amplitude: Flicker ERG:
Reects cone function
Trough of A-wave to the peak of B-wave
Light adaptation
(microvolts)
Flicker 30 Hz stimulus
Reects efciency of the retina in producing
an electrical signal, dependent on pupil size, Focal ERG (FERG):
refractive error, fundal pigmentation and age ERG evoked by a small focal stimulus
Retina is bleached by background light
Implicit latency:
Focal stimulus applied on to retina
Time of stimulus to peak of B-wave
Usually only cone function at macula can be
(milliseconds)
assessed easily
3. Types of ERG
Pattern ERG (PERG):
Maximal response ERG: Reects ganglion cell layer function
Reects a combination of cone and rod
Stimulus is a pattern reversal checkerboard
functions May have applications in glaucoma and optic
Dark adaptation
nerve disease
Bright ash stimulus
Q Tell me about the Electrooculogram.
Electrooculogram (EOG)
1. Principle Amplitude of voltage is recorded
Measures standing potential between electrically 3. Interpretation
positive cornea and negative retina/RPE Amplitude swings increase with light exposure
Exposure to light causes a rise in standing and decrease in darkness:
potential (apical portion of RPE cells depolarize, The swings are expressed as light peak to
giving rise to a positive wave seen on the EOG) dark trough ratio (Arden ratio)
2. Procedure Normal ratio = 1.65
Electrodes are placed on medial and lateral Abnormal ratio reects widespread RPE
canthal area on either side of eye abnormality:
EOG generally parallels ERG readings in
The eye is made to perform saccades between
assessing rod function. However, EOG
two points about 30o apart
cannot assess cone function well
The electrodes pick up voltage differences
Most useful in Bests disease (EOG light rise
between front and back of the eye as it rotates is absent but ERG is normal)
back and forth
B1037_Ch-05.indd 206 12/28/2010 9:35:24 AM

Q Tell me about the Visual Evoked Potential.
Visual Evoked Potential (VEP)

1. Principle Relatively constant positive waveform occurs
Measures the potentials generated at occipital at 100 millisecond (P100 wave)
lobe by visual stimuli The P100 latency is therefore the most useful
Primarily the foveal areas are represented at clinical indicator
supercial part of occipital lobe where the 4. Clinical indications
potential is measured VEP acuity (decrease checkerboard size until
Abnormalities of the VEP are caused by lesions VEP approaches zero)
anywhere between photoreceptor and occipital Optic nerve disease (increased latency/
lobe decreased amplitude to ash or pattern VEP)
2. Procedure Fovea or macular disease (increased latency/
Stimulus: decreased amplitude to pattern VEP)
Flash (variable response, useful in opaque Amblyopia (increased latency/decreased
media) amplitude to pattern VEP)
Pattern reversal (reversing checks or stripes, Malingering (should be normal)
generates maximal cortical activity)
Amplitude of voltage is recorded
3. Interpretation
Waveform:
Extremely variable in size and shape.
Amplitude may vary
B1037_Ch-05.indd 207 12/28/2010 9:35:24 AM

TOPIC 5
AGE-RELATED MACULAR
DEGENERATION
Q Opening Question: What is Age-Related Macular Degeneration (AMD)?

Age-related macular degeneration refers to a spectrum of disease associated with.
The pathogenesis is poorly understood, but the risk factors include.
There are two classic forms of AMD.
Age-Related Macular Degeneration
1. Denition Later, PED occurs and CNV follows (sequence
Spectrum of disease here is unclear)
Visual loss, RPE changes, drusens, geographical 3. Classication
atrophy of retina, choroidal neovascularization Early AMD:
(CNV) Drusens
Usually in persons aged 50 years or above +/ RPE changes
Drusens alone without visual loss is not Late AMD:
considered AMD Geographic atrophy (dry AMD):
Extensive RPE atrophy
2. Pathogenesis
Exudative AMD (wet AMD):
Multifactorial etiology:
Classic/occult CNV
Genetic predisposition, racial patterns
Pigment epithelial detachment
Environmental risk factors:
Subretinal hemorrhage
Smoking
Vitreous hemorrhage
HPT, cardiovascular disease
Disciform scar
UV exposure
Massive exudation (intra/subretinal)
Initial changes include RPE dysfunction,
followed by deposition of drusens, thickening
of Bruchs membrane and RPE atrophy
Exam tips:
The denition of AMD is important but usually poorly answered.
B1037_Ch-05.indd 209 12/28/2010 9:35:24 AM

Q What are Drusens?
Drusens are lipid-like materials deposited in.

Drusens
1. Denition and pathology Nodular drusens (younger onset, may have
Lipid-like/lipofuscin material deposited in family history)
Bruchs membrane (between basement 3. Risk of AMD
membrane of RPE and inner collagen layer) Size of drusens
2. Types Type of drusens:
Hard drusens Hard drusens nonexudative AMD
Soft drusens Soft/conuent drusens exudative AMD
Conuent drusens Family history of AMD
Calcied drusens Fellow eye has AMD
Q What are the Causes of CNV?
The commonest cause of CNV is AMD, but other causes include.

CNV
1. Denition Others (optic disc drusen, angioid streaks)
Proliferation of brovascular lesions from Inammatory disease:
choriocapillaris through defects in Bruchs POHS (presumed ocular histoplasmosis
membrane into subretinal space syndrome)

Posterior uveitis (toxoplasmosis, Vogt-
2. Causes
Koyanagi-Harada syndrome)
Degenerative:
Traumatic (choroidal rupture, laser
AMD
photocoagulation)
Pathological myopia
Tumor (choroidal nevus, choroidal hemangioma)
Exam tips:
There are a number of possible scenarios. If drusens are seen, it is important
to exclude CNV. If a disciform scar is seen, it is important to not only
consider AMD, but myopic degeneration and trauma as well.
Clinical approach to AMD/subretinal hemorrhage

On examination of this patients fundus.
The most obvious lesion is at the macula where a large subretinal hemor-
rhage/disciform scar about 2 disc diameter in size is seen.
Look for
Drusens (AMD)
Tessellated fundus, peripapillary atrophy (myopic degeneration)
Rare causes of CNV:
Optic disc drusen, angioid streaks, choroidal nevus
Iatrogenic (excessive laser photocoagulation)
Other eye:
Bilateral drusens, disciform scar (Likely CNV)
Normal fellow eye (likely PCV)
(Continued)
B1037_Ch-05.indd 210 12/28/2010 9:35:25 AM

(Continued)
Ill like to
Check VA
Perform a FFA to delineate site of leakage from CNV, and ICG to determine
presence of polyps
Q What are the FFA Changes in AMD?
FFA Changes in AMD

1. Early AMD Scars: Staining
Drusens:
3. MPS denition (How is classic and occult CNV
Autouorescence on red-free photograph
Staining dened in MPS?)
Can be hypo- or hyperuorescent Classic CNV well dened CNV occurring
RPE atrophy: early in the course of FFA:
Window defects Extrafoveal (> 200 microns from foveola/FAZ)
Juxtafoveal (< 200 microns from foveola/FAZ
2. Late AMD
but not involving center itself)
Geographic atrophy:
Subfoveal (encroaching into center of
Window defects
foveola/FAZ)
Exudative AMD:
Occult CNV 2 basic patterns:
Pigment epithelial detachment (PED):
Fibrovascular PED (irregular elevation of RPE
Pooling
lls more slowly than classic CNV)
RPE tear: Window defects,
Late leakage of undetermined source (region
hypouorescence (masking)
of hyperuorescence at the level of RPE, best
CNV:
appreciated in late phases, without the
Leaking
features of classic CNV in the early phases)
Hemorrhage: Blocked uorescence
Notes
Classication of occult CNV by ICG (Ophthalmology 1996;103:20542060)
Hot spots (< 1 DD):
Second most common
Can be treated by laser
Plaques (> 1 DD):
Most common
Poor natural history
Combination of hot spots and plaques:
Rare
Q What are the Results of the Macular Photocoagulation Study (MPS)?
The MPS is a multicenter randomized clinical trial to evaluate the effectiveness of.
MPS
1. Hypothesis: Is argon laser photocoagulation Juxtafoveal CNV (Arch Ophthalmol
effective in preventing severe visual loss in eye 1990;108:825831)
with. Subfoveal CNV (Arch Ophthalmol
Extrafoveal CNV (Arch Ophthalmol 1991;109:12201231)
1982;100:192198) 2. Treatment
Argon laser photocoagulation vs no treatment
B1037_Ch-05.indd 211 12/28/2010 9:35:25 AM

3. Outcome Subfoveal:
SVL (severe visual loss) dened as loss of six No prior laser: 20% SVL (laser) vs 37% SVL
lines or more of VA (no laser) at 2 years
Recurrent CNV: 9% SVL (laser) vs 37% SVL
4. Results
(no laser) at 2 years
Extrafoveal: 45% SVL (laser) vs 64% SVL (no
laser) at 5 years 5. Conclusion
Juxtafoveal: 47% SVL (laser) vs 58% SVL (no Laser benecial in all types of classic CNV
laser) at 3 years SVL occurs in both treated and untreated cases
Risk of immediate decrease in VA following
treatment
Notes
Relative indications for PDT
PCV
Myopic CNV
Small classic CNV
CNV associated with POHS
Chronic CSR
Hemangiomas
When anti-VEGF contraindicated
Q What is the Role of PDT in Exudative AMD?
Verteporn is an intravenous photosensitizer which is activated by a diode laser source.

With the introduction of anti-VEGF, the use of PDT is less common.
1. Mechanism of action Results better for pure classic CNV
Selectively damages CNV by: compared to predominantly classic CNV
Preferential localization to CNV (complexes Verteporn also shown to be effective in:
with LDL and CNV has increased LDL Pure occult CNV: Verteporn in
receptors) Photodynamic Therapy (VIP) study
Irradiation conned to CNV Minimally classic CNV: Verteporn in
Light activation causes free radical release, Minimally Classic CNV (VIM) study
endothelial cell damage and thrombus formation CNV associated with pathological myopia:
VIP study
2. Clinical trials
Treatment of AMD with photodynamic therapy 3. Treatment regime
(TAP) study Intravenous verteporn (6 mg/kg body weight)
Inclusion criteria: infused over 10 minutes
Subfoveal, classic/predominantly classic After ve minutes, lesion treated with diode
CNV, 5400 m laser for 83 seconds
VA between 6/12 and 6/60 Patient reviewed every three months, treatment
Treatment groups: PDT vs placebo repeated if there is persistent or new leakage
Main outcome measures: Stable (< 15 logMAR
4. Complications
letters lost) or improved vision
Results:
Ocular:
Hemorrhage from CNV
Stable/improved vision at 12 months: 61%
RPE rip
treated, 46% placebo (Arch Ophthalmol
1999;117:11611173) Systemic:
Stable/improved vision at 24 months:
Pain at injection site
53% treated, 38% placebo (Arch Photosensitivity
Ophthalmol 2001;119:198207) Low back pain
B1037_Ch-05.indd 212 12/28/2010 9:35:25 AM

Q What is the Role of Anti-Vascular Endothelial Growth Factor (VEGF) Antibodies in Exudative AMD?
Anti-VEGF is the established treatment for exudative AMD.

The optimal treatment regime is still being evaluated.
1. Mechanism of VEGF Serous PED, retinal angiomatous proliferation
VEGF is an important mediator in the (RAP), PCV: Can be considered for treatment
pathogenesis of exudative AMD but detailed evidence not available
It stimulates angiogenesis and the development 4. Treatment regime
of CNV, and also promotes vascular leakage Loading dose of monthly injections for 3
2. Anti-VEGF antibodies used in the treatment of months (ANCHOR and MARINA trials)
exudative AMD Maintenance dose (controversial):
Ranibizumab (Lucentis): Recombinant, Monthly: MARINA and ANCHOR trials
humanized murine antigen-binding fragment. (evidence for best VA outcomes)

3-Monthly: Initial VA improvement not
Approved by the FDA for the treatment of
maintained
exudative AMD
Individualized
Bevacizumab (Avastin): Humanized, full-length
Can be combined with PDT or IVTA
monoclonal antibody. Off-label use in the
(combination therapy)
treatment of exudative AMD
5. Outcomes (ANCHOR and MARINA)
Pegaptanib (Macugen): Aptamer that binds and
Gain of 15+ ETDRS letters: 3340% 0.5 mg
inhibits only the extracellular isoforms of VEGF
Lucentis vs 49% sham
3. Which lesions should be treated?
Loss of < 15 ETDRS letters: ~ 95% 0.5 mg
All major CNV subtypes (predominantly classic,
Lucentis vs 6264% sham
minimally classic, occult) in subfoveal and
6. Complications
juxtafoveal location
Ocular:
Active disease:
Retinal detachment
Retinal thickening
Vitreous hemorrhage
Intraretinal/subretinal hemorrhage
Uveitis
New/persistent leakage on FFA
Endophthalmitis
Enlargement of CNV size on FFA
RPE rip
Worsening VA
Systemic (uncommon):
Any baseline VA
Stroke, myocardial infarction
Notes
What is the natural history of exudative AMD if left untreated?
Four lines lost over 24 months
Three lines lost over 12 months
Two lines lost over 6 months
One line lost over 3 months
Q How do You Manage a 70-Year-Old Patient with AMD?
Management of AMD must be individualized to the patient and depends on the type and severity of
AMD and the amount of visual disability and visual requirements of the patient.
If the patient has the nonexudative type of AMD, there is no. On the other hand, if the patient has
exudative type of AMD, I will consider..
B1037_Ch-05.indd 213 12/28/2010 9:35:26 AM

Management of AMD
1. Early AMD and geographic atrophy 2. Exudative AMD
No effective curative treatment FFA to detect and localize CNV
Stop smoking Treatment would depend on:
High levels of antioxidants (Vit C, Vit E, Vit A), Location of CNV
zinc and copper reduce progression to Duration of symptoms
advanced AMD and vision loss: Age-Related Presence of active disease
Presence of scarring
Eye Disease Study (AREDS)
High levels of beta-carotene increases risk of Extrafoveal CNV: Direct laser
lung cancer in smokers Juxtafoveal and subfoveal CNV:
Reassure patient that total blindness rarely Offer monthly injections of intravitreal
occurs anti-VEGF for at least three months

Offer combination therapy with PDT if PCV is
Follow-up patient with Amsler grid monitoring
present
Monitor fellow eye
No proven benet in treating disciform scar.
Low vision aid if necessary
Long-term follow-up required
20% risk to fellow eye in three years
Exam tips:
Give a short concise answer, be as conservative as possible, and lead the
examiner to ask you about a topic you know well.
Notes
How do you monitor a patient with exudative AMD?
Visual symptoms
Visual acuity
New hemorrhages/exudation
OCT
Repeat FFA/ICG when new signs/symptoms occur
Q How do you Administer an Intravitreal Injection?
I would administer intravitreal injections under aseptic conditions.

1. Topical anesthesia 9. Check that patient has at least Counting Fingers
2. Clean and drape patient vision post-injection
3. Lid speculum 10. (Optional) Check IOP
4. strength iodine to sterilize eye 11. Prescribe topical antibiotics for a week after the
5. (Optional) Subconjunctival lignocaine injection
6. Measure and mark injection site with calipers: 12. Warn the patient of risk of:
Pseudophakic: 3 mm behind limbus Endophthalmitis: Red eyes, pain, blurring of
Phakic: 4 mm behind limbus vision
7. Administer intravitreal injection at marked Retinal detachment: Floaters, ashes, visual
injection site with 27G30G needle eld defects
8. Withdraw needle and apply pressure at injection
site with sterile cotton bud
B1037_Ch-05.indd 214 12/28/2010 9:35:26 AM

Q What is Polypoidal Choroidal Vasculopathy (PCV)?
PCV is an abnormality of the inner choroidal vessels.

It typically affects darker-skinned races such as Asians and African Americans.
1. Pathogenesis 3. ICG ndings
Etiology is idiopathic, but similar genetic Large choroidal vascular network associated
mutations are associated with PCV and AMD with localized terminal polyp-like bulbs
Dilated network consisting of multiple terminal Bunch of grapes
aneurysmal protuberances in a polypoidal 4. Treatment
conguration Extrafoveal: Direct laser
Predilection for macular and peripapillary areas Juxtafoveal and subfoveal:
2. Signs PDT
Orange nodules may be visible Anti-VEGF
Serosanguinous maculopathy Combination therapy
Vitreous hemorrhage and massive subretinal/

sub-RPE bleeds more common than CNV
associated with AMD
Q What is Retinal Angiomatous Proliferation (RAP)?
RAP is an uncommon cause of exudative AMD, in which the abnormal vessels arise from the retinal
vasculature rather than the choriocapillaris.
1. Pathogenesis 4. Angiography
Etiology is idiopathic but thought to be age- FFA: Indistinct leaking simulating occult CNV
related ICG: Hot spot in mid or late frames
Angiomatous proliferation originates from the CSLO-ICGA: Hot spot appears after, instead
retina and extends posteriorly into the of before, retinal artery lling
subretinal space, eventually intersecting, in 5. Treatment
some cases, with choroidal new vessels Anti-VEGF
2. Signs PDT with adjunctive triamcinolone/anecortave
Similar to AMD may be successful
Patients tend to be older than patients with CNV Conventional laser photocoagulation often
3. Stages disappointing
Stage I: Intraretinal neovascularization Experimental: Surgical excision of feeder artery
Stage II: Subretinal neovascularization and draining vein
Stage III: CNV 6. Prognosis
Aggressive course and poorer prognosis
B1037_Ch-05.indd 215 12/28/2010 9:35:26 AM

TOPIC 6
OTHER MACULAR
DISEASES
Q Opening Question:Tell me about Pathological Myopia.

Degenerative or pathological myopia is dened as a progressive form of severe myopia.
Usually in patients with axial length of > 26 mm.
It may be an isolated anomaly or associated with.
Pathological Myopia
1. Associations Systemic association:
Ocular: Marfans, Sticklers, Ehlers-Danlos syndrome
ROP Downs syndrome
Congenital glaucoma Alports syndrome
Albinism, congenital stationary night 2. Problems
blindness Higher risk of nuclear sclerosis cataract and
Ectopic lentis POAG
RP
Myopic macular degeneration, CNV, macular hole
Wagners syndrome
Retinal breaks and RD
Clinical approach to myopic fundus

Describe
Tessellated fundus, chorioretinal atrophy
Tilted disc, peripapillary atrophy
Posterior staphyloma
Lacquer cracks
CNV
Foster Fuchs spots (old subretinal hemorrhage and pigmentation)
Related complications
Macular changes
Macular hole
Lattice degeneration, retinal breaks, RD
Ill like to
Examine the anterior segment for cataract
Check the IOP for POAG
B1037_Ch-05.indd 217 12/28/2010 9:35:26 AM

Exam tips:
Also refer to What are the potential problems with high myopia? (page 27).
Q How does Atropine Affect Myopia Progression?
Atropine has been shown to be effective in reducing myopia progression.

It is generally well-tolerated, but the ocular and systemic side effects
may be bothersome in some children.
Atropine and Myopia Progression
Atropine reduces childhood myopia progression Side effects of atropine:
and axial elongation Ocular:
Atropine in the Treatment of Myopia Poor near vision
(ATOM) study (Ophthalmology Photophobia
2006;113:22852291) Conjunctival irritation
After cessation of treatment, eyes treated with Long-term side effects of pupillary
atropine demonstrated higher rates of myopia dilatation (e.g. retinal phototoxicity, early
progression compared with eyes treated with cataract formation) unknown
Systemic (rare):
placebo
Hypotension, arrhythmias
However, the absolute myopia progression
Dry mouth, abdominal distension
after three years was signicantly lower in the
Respiratory depression
atropine group compared with placebo
Confusion, hallucinations, drowsiness
Q What is an Epiretinal Membrane?
Epiretinal membrane (ERM) is a common acquired maculopathy.

It may be idiopathic or associated with.
Epiretinal Membrane
1. Pathogenesis 2. Classication
Histology: Fibro-cellular layer with varying Idiopathic:
degrees of cellularity (retinal glial cells, Age-related
astrocytes, hyalocytes, brocytes, Up to 20% bilateral
myobrocytes) Secondary associations:
Pathogenesis: PVD vitreomacular traction Vascular disease (diabetic retinopathy, CRVO)
Inammatory disease (posterior uveitis)
dehiscence of internal limiting membrane at the
Trauma
macular migration and proliferation of
Retinal surgery (RD surgery, laser
cells epiretinal membrane contraction of
photocoagulation, cryotherapy)
membrane macular pucker
B1037_Ch-05.indd 218 12/28/2010 9:35:27 AM

Clinical approach to ERM

On examination of this patients fundus, the most obvious lesion is at the
macula....
There is a translucent membrane seen, associated with tortuosity of sur-
rounding retinal vessels.
This patient has an epiretinal membrane.
On examination of the rest of the retina, there is evidence/no evidence of:
Look for
Diabetic retinopathy
Retinal vein occlusion
Retinal detachment
Photocoagulation or cryotherapy scars
Retinitis pigmentosa
Choroiditis (POHS, white dot syndromes)
Ill like to
Examine fellow eye bilateral in 20%
Ill like to ask for this patients
VA
Duration of poor vision
Visual requirements
Q How Would You Manage a Patient with ERM?
The management must be individualized.

Factors to consider are.
Management of ERM
1. Factors affecting management: Duration of visual loss < 6 months
Patient factors: VA < 20/60
Age of patient Well-dened ERM edge
Duration of visual loss No associated CME
Visual requirements
3. Complications of surgery:
Ocular factors:
Recurrence of ERM
VA
Well-dened ERM edge
Progression of cataract
Associated CME Iatrogenic breaks/RD
VH
2. Indications for surgery (vitrectomy and
Endophthalmitis
membrane peeling) include:
High visual requirements (occupation, young
age)
B1037_Ch-05.indd 219 12/28/2010 9:35:27 AM

Q Tell me about Macular Holes.
Macular holes are common acquired maculopathies.

They can be idiopathic or associated with.
Macular Hole
1. Pathogenesis: Yellow spot seen at foveola (xanthophyll),
Pathogenesis: Cellular inltration of internal intrafoveal cyst
limiting membrane/posterior hyaloid face of Stage 1b (occult macular hole):
vitreous tangential vitreomacular traction Centrifugal displacement of foveolar retina
occult macular hole secondary contraction and xanthophylls

Characterized by yellow ring
fully developed macular hole PVD
Decrease in visual acuity and metamorphopsia
2. Classication: ~ 50% of Stage 1 holes resolve spontaneously
Idiopathic: Stage 2 (early macular hole)
Age-related Enlargement of yellow ring
Post-menopausal women < 400 micron in size
Up to 20% bilateral
Stage 3 (fully developed macular hole):
Secondary associations: Punched out area surrounded by rim of
High myopia subretinal uid
Trauma Yellow deposits within hole
Solar retinopathy > 400 micron in size
3. Stages (Gasss macular hole classication): Stage 4 (macular hole associated with PVD):
Stage 1a (impending macular hole): > 400 micron in size
Absent foveal reex Associated with PVD
Clinical approach to macular hole

There is a full thickness round punched out defect sees at the fovea.
With a rim of subretinal fluid surrounding the lesion.
Look for
ERM
Myopic fundus
Retinal detachment
Weiss ring (PVD)
Ill like to
Examine the fellow eye bilateral in 20%
Ill like to ask patient for
A history of trauma or solar exposure
VA (If < 20/200 usually means Stage 3 or 4 hole)
Duration of decreased VA
Exam tips:
The pathogenesis and management of the macular hole and epiretinal
membrane are almost identical but the pathogenesis sequence is different.
PVD occurs early in the course of epiretinal membrane, but is a late event in
the macular hole.
B1037_Ch-05.indd 220 12/28/2010 9:35:27 AM

Notes
Why macular?
Macula is thin
Macula is avascular
Increased vitreoretinal traction at this location
Notes
Diagnostic tests for macular hole:
Watzke-Allen test
Laser beam aiming test
OCT
FFA
Q How Would You Manage this Patient with a Macular Hole?
The management must be individualized.

Management of Macular Hole
1. Factors affecting management: Good VA in fellow eye
Patient factors: Surgical treatment if:

Age of patient Young
Duration of visual loss Recent onset of visual loss
Visual requirements High visual needs
Ocular factors: Poor VA in fellow eye
VA Principles of surgery: Vitrectomy/gas
Etiology of macular hole exchange/laser/posture

Stage of macular hole Partial thickness macular hole:
Associated RD Conservative treatment will usually sufce
Follow up patient with Amsler grid
2. Macular hole not associated with RD:
monitoring
Full thickness macular hole:
Conservative treatment if: 3. Macular hole associated with RD:
Elderly Need to look for peripheral retinal breaks
> 1 year duration of visual loss Not common in idiopathic type of macular hole
Low visual need (usually traumatic and myopic types)
Q What is Central Serous Retinopathy (CSR)?
Central serous retinopathy is a common acquired macular disorder.

High risk groups include.
Central Serous Retinopathy (CSR)
1. Pathogenesis: 2. Classication:
Choroidal vascular abnormalities (including Idiopathic:
increased permeability of choriocapillaris) and Young
RPE pump dysfunction/reversal of RPE pump Males
Type-A personality, psychiatric disorders
breakdown of outer blood retinal barrier
High serum epinephrine level
accumulation of subretinal uid CSR
Associated with steroid consumption
B1037_Ch-05.indd 221 12/28/2010 9:35:28 AM

3. Secondary associations: RPE window defect in fellow eye (indicates

Optic disc pit previous subclinical CSR in that eye)
Optic disc coloboma 6. Management:
4. Choroidal tumor clinical presentation: Prognosis:
Patient presents with blurred vision, relative 60% spontaneous resolution in three months
80% spontaneous resolution in six months
scotoma, micropsia and metamorphopsia
Near 100% within one year
VA moderately reduced (20/30 to 20/40),
Minority will have chronic course with
correctable with weak plus lens
decreased VA
Serous RD
Recurrence:
Blister-like localized detachment
40% of all cases
May be associated with:
What are indications for laser
PED
photocoagulation?
Subretinal precipitates
High visual requirements
RPE atrophic changes
Persistent leakage beyond six months
Pseudo RP changes
Recurrent CSR with decreased VA after each
CNV
attack
5. FFA: Fellow eye with CSR associated with
Classic smoke stack pattern (1020% of cases) decreased VA
Ink blot pattern (8090% of cases) Does laser work?
Others: Laser speeds up resolution, but does not alter:
PED Final VA
RPE window defect Recurrence rate
Extramacular RPE atrophic tract Risk of chronicity
Q Tell me about Cystoid Macular Edema.
Cystoid macular edema is a condition in which uid accumulates within the retina (outer plexiform
and inner nuclear layers) in the macula region.
Cystoid Macular Edema
1. Pathogenesis: Intraocular inammation
Breakdown of the blood-retinal barrier Treatment: Immunosuppression,
leakage of uid formation of cystoid spaces acetazolamide
Inammatory mediators and mechanical factors Post-surgery (Irvine-Gass)
may play a role Treatment: Acetazolamide, steroids (topical),
NSAIDS (oral and topical), vitrectomy
2. Investigations:
Drug-induced (adrenaline, latanoprost)
OCT: Intraretinal thickening with cystoid spaces
Treatment: Cessation of medication
FFA: Petaloid pattern of leakage in macula
Vitreomacular traction and ERM
3. Causes and treatment: Treatment: Vitrectomy and relief of traction
Retinal vascular disease (DR, CRVO etc) Others: Retinal dystrophies, tumors
Treatment: Laser photocoagulation (not for
CRVO), intravitreal anti-VEGF and steroids
B1037_Ch-05.indd 222 12/28/2010 9:35:28 AM

TOPIC 7
DIABETIC RETINOPATHY
Q Opening Question No. 1: What are the Ocular Manifestations of DM?

Diabetic retinopathy (DR) is the most important and common ocular complication.
Other ocular manifestations can be classied into.
Ocular Manifestations of DM
1. Anterior segment: 2. Posterior segment:
Cornea: DR
Corneal hypoesthesia (risk of neurotrophic Retinal vascular occlusions
keratitis) Asteroid hyalosis
Decrease corneal healing (risk of recurrent Lipemia retinalis
corneal erosion)
3. Neurological manifestations:
Iris and pupils:
CN palsies (classically pupil sparing III CN
Ectropion uvea
Increase pigment at angles
palsy)
Difculty in dilating pupils Anterior ischemic optic neuropathy
Argyll Robertson pupils 4. Others:
Glaucoma: Xanthelasma
POAG and neovascular glaucoma Orbital mucormycosis
Lens:
Cataract
Exam tips:
Listen carefully to the question; this question is related to ocular manifesta-
tions of DM, not ocular features of DR (see next question).
Q Opening Question No. 2: What are the Ocular Features of DR?

DETAILED ANSWER
Diabetic Retinopathy
1. Nonproliferative retinopathy (NPDR) Venous beading
Mild NPDR Arteriolar narrowing
Microaneurysm (one or more) Intra-retinal microvascular abnormalities
Moderate NPDR (IRMA)

Microaneurysm Severe NPDR (= preproliferative DR):
Retinal hemorrhages (dot and blot) All of above plus any one of the following
Hard exudates three (the famous 4:2:1 rule in ETDRS):
Cotton wool spots (CWS) Blot hemorrhages in four quadrants
B1037_Ch-05.indd 223 12/28/2010 9:35:28 AM

Venous beading in two quadrants VIVA ANSWER

IRMA in one quadrant Diabetic retinopathy (DR) can be divided into two
2. Proliferative retinopathy (PDR) stages.
Early PDR: And can occur with or without macular edema.
New vessels at disc or within 1 DD of disc Diabetic Retinopathy
(NVD) or elsewhere (NVE)
1. Nonproliferative retinopathy (NPDR)
High risk PDR:
Microaneurysms, dot and blot hemorrhages,
NVD greater than 1/4 disc diameter
hard exudates
NVD less than 1/4 disc diameter with
vitreous hemorrhage Pre-proliferative stage (CWS, venous beading,
NVE greater than 1/2 disc diameter with arteriolar narrowing and IRMA)
vitreous hemorrhage 2. Proliferative retinopathy (PDR)
3. Macular edema New vessels at the disc (NVD) or elsewhere
Early macular edema: (NVE)
Retinal thickening or hard exudates within 1 Vitreous hemorrhage, tractional RD and
disc diameter from fovea neovascular glaucoma
Clinically signicant macular edema (CSME): 3. Maculopathy
Retinal thickening or edema less than 500 Exudative maculopathy
micron from fovea Edematous maculopathy
Hard exudates less than 500 micron from
Ischemic maculopathy
fovea associated with retinal thickening
Retinal thickening greater than 1,500 micron
in size, any part of which lies within 1,500
micron from fovea
Exam tips:
The latest convention uses nonproliferative vs proliferative and NOT
background vs proliferative.
The DETAILED answer is based on denitions used by DRS and ETDRS
(page 189).
The VIVA answer ignores these research denitions and is more useful from a
clinical perspective.
Clinical approach to diabetic retinopathy

On examination of this patients fundus, there are
Diffuse dot and blot hemorrhages seen in four quadrants.
Associated with scattered hard exudates, cotton wool spots and venous
tortuosity.
Look for
Disc new vessels (NVD)
New vessels at arcades (NVE)
Macular edema and thickening
(Ill like to confirm the macular edema by further examination using a 78D
lens at the slit lamp)
This patient has: Mild NPDR, severe NPDR or proliferative DR and/or CSME.
(Continued)
B1037_Ch-05.indd 224 12/28/2010 9:35:29 AM

(Continued)
Ill like to
Check fellow eye
Examine anterior segment for cataract and rubeosis iridis
Check IOP and perform gonioscopy (rubeosis at angles)
Ask for associated risk factors for progression (DM control, DM
complications like nephropathy and neuropathy and HPT)
Follow-up question: How would you manage this patient? (page 188)
Q When is FFA Useful in the Diagnosis of DR?
FFA is not routinely indicated in the diagnosis of DR and macular edema.

Indications for FFA
1. Diagnosis: 2. Aid in treatment:
Ischemic maculopathy Delineate fovea and fovea avascular zone
Areas of capillary nonperfusion Delineate area of leakage
Differentiate new vessels from IRMA
Notes
What are the causes of visual loss from diabetic retinopathy?
Diabetic macular edema
Ischemic maculopathy
Consequences of neovascularization (VH, TRD)
Q What are the Risk Factors for Developing Retinopathy in a Patient with DM?
Risk Factors for Developing DR

1. Increased duration of DM 5. Pregnancy
2. Poor metabolic control 6. Others: Smoking, obesity, hyperlipidemia, anemia
3. Hypertension
4. Nephropathy
B1037_Ch-05.indd 225 12/28/2010 9:35:29 AM

TOPIC 8
MANAGEMENT OF
DIABETIC RETINOPATHY
Q Opening Question No. 1: How do You Manage a Patient with DR?

DETAILED ANSWER
Summary of DRS and ETDRS Denitions and Treatment
Denition Criteria Treatment

Mild NPDR 1 microaneurysm Observe (ETDRS)
Moderate NPDR Microaneurysm, hard exudates, hemorrhages, Observe (ETDRS)
cotton wool spots, etc (not meeting criteria below)
Severe NPDR Blot hemorrhages in four quadrants PRP (ETDRS)
(Preproliferative) Venous bead in two quadrants
IRMA in one quadrant
Early PDR NVD or NVE (not fullling criteria below) PRP (ETDRS)
High risk PDR NVD > disc diameter PRP (DRS)
NVD < disc diameter with VH
NVE > disc diameter with VH
Advanced High risk PDR with tractional RD involving Early vitrectomy for
PDR and VH macular or with VH Type I DM (DRVS)
Macular edema Retinal thickening or hard exudates within 1 Observed at 6-monthly
disc diameter from fovea intervals (ETDRS)
CSME Retinal edema < 500 micron from fovea Focal/grid laser
Hard exudates < 500 micron from fovea with (ETDRS)
adjacent retinal thickening
Retinal edema > 1,500 micron, any part of which
is within 1,500 micron from fovea
Exam tips:
The DETAILED answer is for your information, based on treatment guidelines
used by DRS and ETDRS. It is not wise to start talking about the multicenter
studies at this stage.
The VIVA answer ignores these research denitions, summarizes the main
problems and gives the examiner the impression that you know the issues,
have thought through the results of the trials, and are now applying it for
patients in your clinical practice!
B1037_Ch-05.indd 227 12/28/2010 9:35:29 AM

VIVA ANSWER
The management of DR involves an assessment of the risk of progression.
Management depends on the stage of DR and the presence or absence of macular edema.
Management of DR
1. Assess risk of progression of disease and control Prior to cataract operation or pregnancy
high risk factors 4. Proliferative DR

Joint management with family physician or I would consider this an ocular emergency
endocrinologists I would perform full PRP immediately with
Ensure good DM control 2,0003,000 laser shots over 23 sittings
Treat associated systemic disease (e.g. HPT, DM, Watch patient very closely
hyperlipidemia) 5. Macular edema
2. Mild NPDR I would perform macular laser (focal / grid) if
Follow-up patient and watch for progression there is clinically signicant macular edema
and macular edema I would follow-up the patient at 4-month
3. Severe NPDR intervals, and would repeat the macular laser if
Follow up patient very closely the edema is not resolved
In my practice, I would consider scatter PRP if: In cases of persistent macular edema despite
Patient is a young insulin dependent diabetic multiple laser attempts, I would consider
(IDDM) administering intravitreal anti-VEGF or
Patient has poor DM control with associated corticosteroids
DM complications (nephropathy) If the macular edema is secondary to
Fellow eye is blind from DR vitreomacular traction, I would offer the patient
Family history of blindness from DR
vitrectomy to relieve the traction
Poor patient compliance to follow-up
Q What are the Major Clinical Trials in the Management of Diabetic Retinopathy?
DRS (Diabetic Retinopathy Study) (Ophthalmology 1981;88:583600

Ophthalmology 1987;94:739760)
1. Aim: Assess effect of PRP on PDR. Determine if 1. Aims:
PRP reduces the incidence of severe visual loss Assess effect of PRP on DR and less than
(VA < 5/200) high-risk PDR
2. Inclusion criteria: PDR in both eyes (1,758 patients) Assess effect of aspirin on preventing the
3. Treatment: PRP in one eye vs no treatment in progression of DR
other eye Assess effect of macular laser on diabetic
4. Outcome: SVL (severe visual loss) dened as VA < macular edema
5/200 on two follow-up visits 2. Inclusion criteria: Mild DR to PDR (not meeting
5. Results: criteria for high-risk PDR) with or without
50% decrease in rates of SVL in treated eyes as macular edema in both eyes (3,711 patients).
compared with controls at ve years VA 20/200
Eyes that beneted most from PRP were 3. Treatment:
high-risk PDR PRP in one eye vs no treatment in other eye
Complication of Argon laser (10% decrease in until high-risk PDR developed
VA by one or more lines) Grid laser vs no treatment for macular
6. Conclusion: Early PRP recommended for high risk edema
PDR Aspirin vs placebo
4. Outcome: MVL (moderate visual loss) dened as
ETDRS (Early Treatment Diabetic Retinopathy
doubling of visual angle, drop of 15 or more
Study) (Arch Ophthalmol 1985;88:583600 Ophthalmol-
ogy 1991;98:757; Ophthalmology 1991;98:766785) letters or three or more Snellen acuity lines
B1037_Ch-05.indd 228 12/28/2010 9:35:30 AM

5. Results: 4. Outcome:
Efcacy of laser treatment on CSME 50% Rates of onset or progressive DR from
decrease in rates of MVL in treated eyes baseline
Optimal timing of PRP: Rates of progression to high risk PDR
PRP recommended for severe NPDR and Rates of laser treatment
early PDR (decreases incidence of developing 5. Results:
high risk PDR)
Tight control delays onset and progression of
Follow-up for mild or moderate NPDR
DR, neuropathy and nephropathy
Aspirin treatment:
Tight control lowers the onset of DR in
No effect on rates of progression
No effect on VA
the primary prevention group by 50% at
No increased risk of VH ve years
Not contraindicated for use in cardiovascular Tight control decreases the rate of progression
or medical conditions of DR in the secondary prevention group by
50%
DRVS (Diabetic Retinopathy Vitrectomy Study)
50% decrease in the development of severe
(Arch Ophthalmol 1985;103:16441652; Ophthalmol-
ogy 1988;95:13071320) NPDR, PDR and the need for PRP
Early worsening of DR in the rst year of tight
1. Aim: Assess effect of early vitrectomy on advanced
glycemic control. At least three years needed to
PDR and vitreous hemorrhage (VH)
demonstrate the benecial effect of tight
2. Treatment: Early vitrectomy vs late vitrectomy control
(1 year) However, tight control increases severe
3. Inclusion criteria: VH or advanced PDR with hypoglycemia events by 23 times, and there
useful vision (VA < 5/200) is a 33% increased risk of becoming
overweight
4. Outcome: Percentages of eyes with 20/40 VA at 2
and 4 years UKPDS (United Kingdom Prospective Diabetes
Study)
5. Results:
VH (in Type I DM, 36% recovered to 20/40 for 1. Aim:
early vitrectomy vs only 12% for late Assess effect of intensive glycemic control on
vitrectomy) the risk of microvascular complications of DM
Advanced PDR with useful vision (44% (including DR)
recovered to 20/40 for early vitrectomy vs 28% Assess effect of BP control in hypertensive
for late vitrectomy) patients on the risk of microvascular
complications of DM (including DR)
6. Conclusion: Early vitrectomy recommended for
2. Treatment:
vitreous hemorrhage and advanced PDR in
Diet control vs intensive treatment with
Type I DM hypoglycemic agents
Tight vs less tight BP control
DCCT (Diabetic Control and Complications
Trial) (New Engl J Med 1993;329:977986; New 3. Inclusion criteria: Type 2 DM, with and without
Engl J Med 2000;342:381389) hypertension
1. Aim: 4. Outcome: Progression of DR and other
Primary prevention: Assess effect of tight microvascular and macrovascular complications
glycemic control on DM complications
of DM
(nephropathy, neuropathy and DR)
Secondary prevention: Assess effect of tight 5. Results:
glycemic control on rate of progression of DM Intensive glycemic control slows the
complications (nephropathy, neuropathy progression of DR and reduces the risk of
and DR) other microvascular complications of DM
2. Treatment: Tight glycemic control vs normal Tight BP control slows progression of DR and
control reduces the risk of other microvascular and
3. Inclusion criteria: Type I DM macrovascular complications of DM
B1037_Ch-05.indd 229 12/28/2010 9:35:30 AM

Exam tips:
You must be fairly comfortable with the ve major studies in DR over the
past three decades.
Q What are the Indications for Laser PRP in Your Practice?
While the DRS and ETDRS dened the ideal indications for PRP.
In my practice, I would consider PRP in the following patients if.
Indications for PRP
1. PRP for high-risk PDR Patient has poor DM control with associated
2. Consider PRP in cases of less than high-risk PDR: DM complications (nephropathy)
Early PDR (any NVD or NVE) Fellow eye is blind from DR
Family history of blindness from DR
Severe NPDR
Poor patient compliance to follow-up
Ischemic NPDR (FFA indicates ischemia)
Prior to cataract operation or pregnancy
In my practice, I would consider scatter PRP for
these cases, especially if:
Patient is a young insulin dependent diabetic
(IDDM)
Exam tips:
This is an opportunity to show that you have developed your own approach
based on practice guidelines.
Q How do You Perform PRP?
I would elect to perform fractionated PRP with an aim of between 2,0003,000 laser shots in patients
with PDR, divided over 2 to 3 laser sessions.
Procedure for PRP
1. I would use the following: 3. The laser is then performed:
Contact lens: Mainster wide eld Mark vascular arcades with two rows of laser
Laser type: Argon blue-green laser Start on inferior fundus
Laser settings: 200 m size, 0.18 s, 0.18 W Avoid disc, macular, vessels, brovascular
2. I would rst instill topical LA, position patient, membranes and areas with vitreoretinal traction
Target: Gray-white burns
xation target
4. Follow-up patient within next week for top-up
PRP
Q What are the Signs that DR is Less Active Post-PRP?
Post-PRP Changes
1. Regression of new vessels and increased brosis 3. Resolution of retinal hemorrhages
2. Vessel attenuation 4. Disc pallor
B1037_Ch-05.indd 230 12/28/2010 9:35:30 AM

Q What are the Complications of PRP?
Complications
1. Early: 2. Late:
Iris burns Loss of VA of one line (11%) and two lines (3%)
Macular burns VF defects
Retinal tears Deterioration of night vision and color vision
VH Tractional RD
CME ERM
Choroidal detachment CNV
Malignant glaucoma
Q How does PRP Work?
Mechanisms of PRP
1. Decrease retinal demand for oxygen 2. Decrease release of angiogenic factors
PRP destroys healthy peripheral retina, allowing PRP decreases amount of hypoxic retina and
diseased retinal vessels to deliver limited therefore less angiogenic factors are released
oxygen to remaining central retina 3. Mechanical inhibition of NV formation
Scars contain new vessel growth
Q What are the Indications for Vitrectomy in DR?
The common indications for vitrectomy include.

Indications for Vitrectomy
1. Common: Rubeosis with VH (preventing adequate PRP)
Tractional RD involving macula Dense premacular VH
Combined tractional and rhegmatogenous RD Ghost cell glaucoma
Persistent VH (more than 6 months for NIDDM, Macular edema with macular traction
3 months for IDDM) Signicant recurrent VH despite maximal PRP
2. Less common:
Progressive brovascular proliferation
(especially anterior hyaloid brovascular
proliferation)
B1037_Ch-05.indd 231 12/28/2010 9:35:30 AM

TOPIC 9
RETINAL ARTERY
OCCLUSION
Q Opening Question No. 1: What are the Causes of Retinal Artery Occlusion?
Retinal artery occlusion can be caused by systemic or ocular conditions.
Retinal Artery Occlusion
1. Systemic Vasculitis:
Carotid emboli (most common cause of BRAO): Giant cell arteritis
Cholesterol emboli (small size, causes Systemic lupus, polyarteritis nodosa and
Hollenhorst plaques in retinal arterioles) others
Fibrinoplatelet emboli (medium size, causes Coagulation disorders: Protein C/S deciencies,
transient ischemic attacks/amaurosis fugax) antithrombin-III deciency, anti-phospholipid
Calcic emboli (large, causes CRAO or
syndrome
BRAO)
2. Ocular
Cardiac emboli (most common cause in young
Raised IOP:
patient with either BRAO or CRAO):
Retrobulbar hemorrhage during retrobulbar
Thrombus (from myocardial infarct or mitral
anesthesia
valve stenosis)
Orbital tumor, orbital inammatory disease
Calcic (from aortic valve)
RD surgery
Bacteria (from endocarditis)
Neurosurgery
Myxomatous (from atrial myxoma) very rare
Exam tips:
As a general rule, CRAO is caused by atherosclerosis of the carotid and
retinal arteries, while BRAO is caused by an emboli.
Q How do You Manage a Patient with CRAO?
CRAO is an ocular emergency.

The acute treatment is aimed at restoring normal circulation as far as possible.
Management of CRAO
1. Clinical features: Cherry red spot in white retina
Acute decrease in VA (10% bilateral) Macular sparing due to macular perfusion
RAPD from cilioretinal artery (20% of population)
B1037_Ch-05.indd 233 12/28/2010 9:35:31 AM

Isolated cilioretinal artery occlusion leads to

EAGLE study (Graefes Archive of Clinical
macular infarct (rare) and Experimental Ophthalmology 2006;
Attenuated retinal arterioles 244L:950956): terminated due to adverse
Optic disc pallor events)
Retrobulbar tolazoline (to decrease
2. Acute management:
retrobulbar resistance to ow)
Patient should lie at
Patient given carbogen (mixture of 5% carbon 3. Manage systemic disease:
dioxide and 95% oxygen) Mortality in 20% over 5 years
Investigate and treat systemic disease
Ocular massage for 15 minutes
Iris neovascularization in 20% within 3 months
Intravenous Diamox
(lower risk than CRVO)
Anterior chamber paracentesis
But neovascular glaucoma < 5%
Other treatment options:
Intra-arterial brinolysis
Notes
What are the causes of cherry red spot in the macula?
Acquired
CRAO
Macular hole
Commotio retinae
Drug (quinine toxicity)
Congenital
Niemann-Pick disease
Tay Sachs disease
Gangliosidoses
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TOPIC 10
RETINAL VEIN
OCCLUSION
Q Opening Question No. 1: What are the Causes of Vitreous Hemorrhage?

1. Trauma is an important cause of VH 3. Other less common causes include
2. The most common nontraumatic causes are Vascular diseases with ischemia
Proliferative DR (50%) HPT, ocular ischemic syndrome
Eales disease
BRVO/CRVO (10%)
ROP/familial exudative vitreoretinopathy
RD, tears (10%)
Retinal telangiectasia
CNV with breakthrough bleed (10%)
Inammatory diseases with ischemia
PVD (10%)
Blood dyscrasias
Q Opening Question No. 2: What are the Features of Branch Retinal Vein Occlusion (BRVO)?
BRVO is a common retinal vascular disease.
Branch Retinal Vein Occlusion
1. Risk factors 4. Clinical features
Systemic: Acute:
Age Dilated and tortuous veins, retinal
HPT hemorrhages, VH, cotton wool spots, disc
Blood dyscrasias swelling
Ocular: Subacute:
Vasculitis (Behcets syndrome, sarcoidosis) Vascular sheathing and collaterals, CME
2. Classication Chronic:
Main BRVO: Pigmentary changes (pseudo retinitis
At disc pigmentosa), macular RPE changes and

Away from disc epiretinal membrane
Macular BRVO 5. Prognosis and complications
Peripheral BRVO 50% of patients will have uncomplicated BRVO
3. Sites (usually at AV crossing) and recover to VA 20/40 or better
Superotemporal (50%) In the other 50%, one or more
Inferotemporal (30%) complications:
Macular edema (most common cause of
Hemispherical (15%)
persistent poor VA)
Supero/inferonasal (5%)
B1037_Ch-05.indd 235 12/28/2010 9:35:31 AM

Macular ischemia Neovascularization (40% of ischemic BRVO):

Combined macular edema and macular 10% NVD; 2030% NVE
ischemia
Q Opening Question No. 3: What are the Features of Central Retinal Vein Occlusion (CRVO)?
CRVO is a common retinal vascular disease.
Central Retinal Vein Occlusion
1. Risk factors Ocular:
Systemic: Raised IOP
Age Hypermetropia
HPT Congenital anomaly of central retinal vein
Blood dyscrasias Vasculitis (Behcets syndrome, sarcoidosis,
AIDS, systemic lupus)
2. Classication and clinical features
Ischemic Nonischemic
Frequency 75% 25%
VA 20/400 or worse (90%) Better than 20/400 (90%)
RAPD Marked Slight
VF defect Common Rare
Fundus ndings Extensive hemorrhages and cotton Less extensive hemorrhages, few
wool spots cotton wool spots
FFA Widespread capillary non-perfusion Good perfusion
ERG Reduced b-wave amplitude Normal
Reduced b:a wave ratio
Prognosis 50% develop rubeosis and neovascular 3% develop rubeosis and neovascular
glaucoma in 3 months (100 day glaucoma while 50% return to VA of
glaucoma) 20/40 or better
VA improves in 1/3, stable in 1/3, and Conversion to ischemic CRVO: 34%
deteriorates in 1/3 in 3 years
Exam tips:
The differentiation between ischemic and nonischemic CRVO is important.
Clinical approach to BRVO/CRVO

On examination of this patients fundus, there are.
Flame-shaped hemorrhages seen along the superotemporal vascular arcade.
Associated with scattered hard exudates, cotton wool spots, vessel tortuosity
and dilatation.
Look for
Disc swelling
Cup disc ratio (glaucoma can lead to CRVO and vice versa)
B1037_Ch-05.indd 236 12/28/2010 9:35:31 AM

New vessels (NVD, NVE)

Macular edema
(Id like to confirm the edema using a 78D lens at the slit lamp)
Treatment (PRP scars and ERM)
Ill like to
Check fellow eye (10% bilateral)
Check IOP and perform gonioscopy (new vessels at the angle)
For CRVO:
Undilated SLE for new vessels on the iris
Check RAPD
Ask for VA
Ask whether patient has a history of DM, HPT, hyperlipidemia
Check BP
Conduct the following investigations:
CBC (polycythemia and hyperviscosity), blood sugar levels, lipids
FFA (after 3 months)
Exam tips:
Be careful here, old BRVO or CRVO can have features similar to RP
(pseudo RP).
Q How do You Manage BRVO/CRVO?
The management of BRVO/CRVO must be individualized.

The factors to consider are the patients VA and whether there are associated complications like macular
edema or neovascularization.
The prognosis for CRVO is poor.
Management of BRVO
1. Investigate and treat associated systemic disease Once new vessels are seen sector PRP is
(e.g. HPT, DM, hyperlipidemia) indicated
2. Ocular management Other treatment modalities:
IVTA: Does not improve VA in macular
Two main complications are: Macular edema
edema from BRVO and there are signicant
and neovascularization at disc
side effects of raised IOP and cataract
Wait for hemorrhage to clear (3 months) (SCORE Study: Arch Ophthalmol
Perform OCT and FFA at 3 months 2009:127:11151128)
If macular edema is present and VA < 20/40: Dexamethasone implant: Improves VA in
Grid laser photocoagulation macular edema from BRVO, but side effects
Intravitreal anti-VEGF if grid laser treatment of raised IOP and cataract TPPV with AV
failure or in eyes with extensive macular sheathotomy
hemorrhage (BRAVO Study: Ophthalmology
2010; Epub) Management of CRVO
Consider intravitreal anti-VEGF as primary 1. Investigate and treat associated systemic disease
treatment if VA > 20/40 (e.g. HPT, DM, hyperlipidemia)
If 5 disc diameter of nonperfusion is seen
close follow-up to look for neovascularization
or sector PRP
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2. Ocular management IVTA: Improves VA in macular edema

Two main complications are: Macular edema secondary to non-ischemic CRVO, but
and neovascular glaucoma signicant side effects are raised IOP and
Need to differentiate between ischemic/ cataracts (SCORE study)
No benet in treating macular edema in
non-ischemic CRVO
CRVO with grid laser
Wait for hemorrhage to clear (3 months)
Other treatment modalities:
Perform OCT and FFA at 3 months:
Dexamethasone implant: Improves VA but
If nonischemic no treatment is needed,
signicant side effects are raised IOP and
prognosis is good (50% 20/40 of better VA)
cataracts
If ischemic prognosis is poor (50%
Laser-induced chorioretinal venular
neovascular glaucoma in 3 months), close
anastomosis (Central Retinal Vein Bypass
follow-up is needed to look for new vessels at
Study: Ophthalmology 2010; in press):
iris; or full PRP
Once new vessels at iris are seen full PRP
May improve VA in non-ischemic CRVO
is indicated Complicated by CNV and vitreous hemorrhage
If macular edema is present and VA < 20/40: Radial optic neurotomy and retinal vein
Intravitreal anti-VEGF (CRUISE Study: cannulation with tPA infusion
Ophthalmology 2010; Epub)
What are the Main Findings of the Branch Vein Occlusion Study (BVOS)? The Central Vein Occlusion
Q Study (CVOS)?
BVOS CVOS
Macular edema Is argon grid laser useful in improving Is argon grid laser useful in preserving
VA in eyes with BRVO and macular or improving VA in eyes with CRVO and
edema with VA 20/40? macular edema with VA 20/50?
Conclusion: Yes Conclusion: No
Gain of at least two lines of VA from Treatment clearly reduced FFA evidence
baseline of macular edema but no difference in
When should laser be performed? Not nal VA
answered in the study
Neovascularization Can peripheral argon sector PRP Can prophylactic argon PRP in ischemic
prevent CRVO prevent two clock hours of iris or
1. Neovascularization? angle neovascularization? Or is it more
2. VH? appropriate to start PRP only when new
Conclusion: Yes vessels are seen?
PRP prevents neovascularization and Conclusion:
VH 1. No, PRP does not prevent iris or
angle neovascularization
When should laser be performed?
2. Regression is faster in untreated eyes
Should be started after development
3. Therefore, PRP should be started
of neovascularization
after development of iris or angle
neovascularization
Recommendations 1. FFA when hemorrhage clears (3 1. If 10 disc diameter of nonperfusion,
months) careful observation with undilated
2. If macular edema and VA < 20/40 SLE and gonioscopy
seen grid laser 2. PRP indicated only after neovascu-
3. If 5 disc diameter of nonperfusion, larization develops
follow-up closely for new vessels 3. No benet for treatment of macular
4. Once new vessels are seen edema
sector PRP is indicated
B1037_Ch-05.indd 238 12/28/2010 9:35:35 AM

Exam tips:
One of the more common clinical trials asked in exams. Refer to BVOS (Am J
Ophthalmol 1984;98:271282; Arch Ophthalmol 1986;194:3441) and
CVOS (Ophthalmol 1995;102:14251433).
Exam tips:
Refer to BRAVO and CRUISE (Ophthalmology 2010; Epub).
Q What are the Main Findings of the BRAVO and CRUISE Studies?
BRAVO CRUISE
Aim To assess efcacy and safety of 0.3 mg or To assess efcacy and safety of 0.3 mg
0.5mg ranibizumab in patients with macular or 0.5mg ranibizumab in patients with
edema following BRVO macular edema following CRVO
Design Prospective randomized multicenter studies
Inclusion criteria 1. Macular edema involving foveal center secondary to BRVO/CRVO
2. Central foveal thickness 250m
3. BCVA < 20/40
Treatment groups Monthly intraocular injections of 0.3mg or 0.5mg ranibizumab, or sham injections
Outcome measures 1. Mean change from baseline BCVA at month 6
2. Gain of 3 lines (15 letters) of BCVA at month 6
Central foveal thickness at month 6
Results Mean change in baseline BCVA at month 6: Mean change in baseline BCVA at month 6:
0.3mg ranibizumab: 16.6 letters 0.3mg ranibizumab: 12.7 letters
0.5mg ranibizumab: 18.3 letters 0.5mg ranibizumab: 14.9 letters
Sham: 7.3 letters Sham: 0.8 letters
Gain in 3 lines (15 letters) at month 6: Gain in 3 lines (15 letters) at month 6:
0.3mg ranibizumab: 55.2% 0.3mg ranibizumab: 46.2%
Sham: 28.8% Sham: 16.9%
Mean reduction in foveal thickness at Mean reduction in foveal thickness at

month 6: month 6:
Sham: 27.9% Sham: 23.9%
Safety prole consistent with previous Safety prole consistent with previous
ranibizumab trials ranibizumab trials
Conclusions Intraocular injections of 0.3mg or 0.5mg Intraocular injections of 0.3mg or
ranibizumab provided rapid, effective and 0.5mg ranibizumab provided rapid,
safe treatment for macular edema following effective and safe treatment for
BRVO macular edema following CRVO
B1037_Ch-05.indd 239 12/28/2010 9:35:35 AM

Q How do You Investigate a 20-Year-Old Female with CRVO?
CRVO in a young patient is not common.

I would need to evaluate the patient carefully through a detailed history,
physical examination and appropriate investigations.
CRVO in Young Patient
1. Basic evaluation Heart murmur
Medical conditions: BP
HPT, DM, hyperlipidemia, coagulopathy 3. Laboratory investigation
Cardiac disease (mitral valve prolapse), CBC, ESR
autoimmune disease, AIDS Blood sugar levels, lipid levels
Ocular conditions: VDRL, FTA
Glaucoma
HIV
Medication (oral contraceptive pills, hormone
CXR
therapy)
Coagulation (PTT, APTT), homocysteine,
2. Physical exam antiphospholipid antibody
Carotid bruit Autoimmune markers
Exam tips:
Think of SECONDARY causes (page 196).
Q What is the Antiphospholipid Syndrome?
The antiphospholipid syndrome refers to a coagulation disorder.

Characterized by circulating antiphospholipid antibodies.
Antiphospholipid Syndrome
1. Classication 2. Clinical features
Primary: In vitro, antiphospholipid antibodies cause
No secondary disease anticoagulation (i.e. bleeding)
Circulating antiphospholipid antibodies In vivo, they are associated with paradoxically
(includes lupus anticoagulant, anticardiolipin with coagulation (i.e. thrombosis):
antibodies) CRVO and BRVO, CRAO and BRAO
Afnity for phospholipids (important in Retinal vasculitis
conversion of prothrombin to thrombin) Choroidal infarction
Secondary: Arteritic anterior ischemic optic neuropathy
Systemic lupus, other auto-immune diseases
AIDS
Phenothiazine and procainamide use
B1037_Ch-05.indd 240 12/28/2010 9:35:35 AM

TOPIC 11
CARDIOVASCULAR
DISEASE
Q Opening Question: What are Ocular Associations of Cardiac Disease?

The ocular effects are usually secondary to an embolic phenomenon from cardiac disease.
Cardiac Disease
1. Embolic (thrombotic, calcic, bacterial material) 2. Generalized decreased perfusion state from heart
Ophthalmic artery: failure
Ophthalmic artery occlusion Fainting spells
Retinal artery: Ocular ischemic syndrome:
Amaurosis fugax What are the features of ocular ischemic
CRAO syndrome? (see page 202)
Retinal arterioles: Anterior segment ischemia
BRAO (cardiac emboli is most common Hypoperfusion retinopathy
cause of BRAO in younger persons)
3. Right heart failure
Precapillary arterioles:
Superior vena cavae congestion
Cotton wool spots, Roths spot
Increase in episcleral venous pressure
glaucoma
Notes
What are the features of ophthalmic artery occlusion?
Anterior segment ischemia (anterior ciliary artery)
Posterior segment ischemia (posterior ciliary artery)
Ophthalmoplegia (extraocular muscle involvement)
ERG shows decreased a and b wave amplitude affected)
Q What are the Ocular Associations of Carotid Artery Disease?
The ocular effects are usually secondary to either a thrombotic or embolic phenomenon.
Carotid Artery Disease
1. Embolic (cholesterol, brinoplatelet, calcic) 2. Thrombotic
Similar spectrum to above (carotid emboli is Carotid artery
most common cause of BRAO in older persons) Ocular ischemic syndrome (most common
cause of ocular ischemic syndrome)
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Ophthalmic artery: Anterior choroidal artery (homonymous

Ophthalmic artery occlusion hemianopia)
Anterior ischemic optic neuropathy Anterior cerebral artery (hemialexia)
Retinal artery: Middle cerebral artery (homonymous
Amaurosis fugax (most common cause of hemianopia, Gaze palsies)
amaurosis fugax) Aneurysm (branches of carotid arteries):
CRAO (most common cause of CRAO) Subarachnoid hemorrhage from ruptured
3. Others aneurysm
Compressive III CN palsy
Horners syndrome
Stroke (branches of carotid arteries):
Q What are the Principles of Management of a Patient with Carotid Artery Disease?
Management of Carotid Artery Disease

1. Modify risk factors 4. Carotid endarterectomy
HPT, DM, hyperlipidemia, smoking North American Symptomatic Carotid
2. Antiplatelet therapy Endarterectomy Trial (New Engl J Med
Aspirin 1998;339:14151425)
Indications for carotid endarterectomy:
Dipyridamole
Symptomatic patients with amaurosis,
Clopidogrel
hemispheric TIA, nondisabling strokes
3. Anticoagulation therapy Plus 7099% carotid artery stenosis
Indications: Prognosis
If aspirin fails 2-year stroke rate is 9% (endarterectomy)
Recurrent cardiac source of emboli (atrial vs 26% (no surgery)
brillation, mitral valve stenosis, etc.) European Carotid Endarterectomy Trial
(Lancet 1998;351:13791387)
No benet with carotid endarterectomy
Q What is Amaurosis Fugax?
Amaurosis fugax is a transient monocular blindness less than 24 hours by denition.

The causes can be either systemic or ocular in nature.
Amaurosis Fugax
1. Systemic Systemic lupus, polyarteritis nodosa and
Carotid artery atherosclerosis (most common others
cause) Coagulation disorders
Carotid emboli 2. Ocular
Cardiac emboli Raised IOP
Vasculitis: Drusens
Giant cell arteritis Papilledema
Migraine
Anterior ischemic optic neuropathy
Exam tips:
Causes almost identical to those for retinal artery occlusion (page 233).
B1037_Ch-05.indd 242 12/28/2010 9:35:36 AM

Q What is the Ocular Ischemic Syndrome?
The ocular ischemic syndrome is a disorder secondary to hypoperfusion of the globe due to either
chronic carotid artery obstruction or ophthalmic artery obstruction.
Ocular Ischemic Syndrome
1. Cause 4. Investigations
Carotid atherosclerosis (most common cause): FFA:
90% or more carotid artery obstruction Delayed choroidal lling time (60%)
before symptoms Delayed arteriole-to-venule transit time
Bilateral in 20% (95%)
Generalized decreased perfusion from cardiac Capillary nonperfusion
failure Prominent arteriolar wall staining
Others (GCA, arteritis) ERG:
Decreased amplitude of both a and b
2. Symptoms
waves (like ophthalmic artery occlusion, see
Decrease VA (for weeks and months)
above)
Aching ocular pain
Systemic conditions:
Light-induced visual loss (with prolonged
ESR, lipids levels
recovery from exposure to bright light)
5. Prognosis
3. Signs
Systemic associations:
Anterior segment ischemia: 50% have ischemic heart disease
Injected eye
25% have previous stroke
Corneal edema
20% have severe peripheral vascular
Iris neovascularization, iris atrophy,
disease
iridoplegia
5 year mortality is 40% (higher than for CRAO;
AC are (more are than cells)
page 194)
Swollen mature cataract
Raised IOP (50%), low IOP (50%) 6. Treatment
Posterior segment (hypoperfusion retinopathy): Ocular:
Vessel tortuosity, venous dilation Laser PRP for new vessels (regression in small
Microaneurysm, retinal hemorrhage, hard percentage)
exudate Manage NVG
New vessels, VH Manage systemic risk factors
Choroidopathy (Elschnigs spots, serous RD) Carotid endarterectomy
Papilledema, macular star
B1037_Ch-05.indd 243 12/28/2010 9:35:36 AM

TOPIC 12
RETINOPATHY OF
PREMATURITY
Q Opening Question: What is Retinopathy of Prematurity?

Retinopathy of prematurity (ROP) is a proliferative vascular retinal condition.
It commonly occurs in premature and low birth weight babies exposed to high ambient oxygen.
There are classically two phases, the active ROP phase and cicatricial ROP phase.
Retinopathy of Prematurity
1. Risk factors: Stage:
Prematurity < 32 weeks Stage 1: Demarcation line
Low birth weight < 1,500 g Stage 2: Ridge
Stage 3: Extraretinal brovascular
Supplemental oxygen
proliferation
Others:
Stage 4: Subtotal RD (4a: Extrafoveal; 4b:
Intraventricular hemorrhage
Foveal involvement)
Necrotizing enterocolitis
Stage 5: Total RD
Maternal theophylline treatment
Abruptio placentae
Plus disease:
Dilatation and tortuosity of veins
Quality of NICU care
Vitreous haze
Others: Twins, respiratory distress syndrome,
Engorged iris vessels
history of blood transfusion
Poor pupillary reaction
2. Active ROP
3. Cicatricial ROP:
Location:
Zone I: Circle centered on disc with radius
20% of active ROP will progress to cicatricial
twice the distance from disc to fovea ROP without treatment
Zone II: Circle from edge of Zone 1 to a point Stages:
tangential to nasal ora serrata and around to Stage 1: Myopia, pigmentary changes
an area near temporal ora Stage 2: Temporal vitreoretinal brosis,
Zone III: Remaining crescent from Zone 2 to dragged disc
temporal ora Stage 3: Peripheral brosis and falciform
Extent: retinal fold
Number of clock hours Stage 4: Partial RD
Stage 5: Total RD, secondary glaucoma
Exam tips:
The denition of the various parameters (zones, extent and stage) has to be
committed to memory. Many candidates do not dene zones well.
B1037_Ch-05.indd 245 12/28/2010 9:35:36 AM

Q What is the Pathogenesis of ROP?
ROP occurs as a result of failure of vascularization of the immature retina.

There are two theories.
Pathogenesis
1. Normal retinal angiogenesis 3. Spindle cell theory of Kretzer and Hittner
Starts at 16 weeks Nascent blood vessels form in normally hypoxic
Reaches nasal ora at 36 weeks uterine condition by canalization and
Complete vascularization at 40 weeks (up to endothelial cell differentiation behind a
one month post-term) migrating sheet of spindle cells
2. Biphasic theory of Aston and Patz High ambient oxygen triggers extensive gap
High ambient oxygen vasoconstriction and junction formation between spindle cells which
toxic obliteration of retinal capillaries on interferes with migration and canalization of
return to room air relative ischemia develops blood vessels
angiogenic factors secreted vascular Spindle cells secrete angiogenic factors
proliferation vascular proliferation
Notes
What is Rush disease?
Rapidly progressive Stage 3 ROP in Zone 1 or posterior Zone 2
Poor prognosis
Q How do You Screen a Baby for ROP Born at 32 Weeks of Gestation?
The examination schedule for ROP in our center is.

The indications for treatment of ROP are.
Screening and Management of ROP
1. Who should be screened? 4. What are the indications for treatment?
1,500g, born 32 weeks gestation Threshold ROP:
Selected infants 1,500g2,000g or born at Zone I or II
gestational age > 32 weeks, with unstable Five contiguous clock hours or eight
clinical course noncontiguous clock hours

Stage 3
2. When to start screening?
Plus disease
Median age of ROP = 37 weeks
Threshold ROP is associated with 50% risk
90% of ROP occur between 34 to 42 weeks
of having VA 20/200 or worse without
Therefore, start screening at 34 weeks
treatment
(alternatively, can start screening four weeks
More recent evidence support the treatment of
postnatally)
prethreshold ROP
3. How often should subsequent screening be? Early Treatment for Retinopathy of
If rst screening examination shows: Prematurity Study (ETROP) recommends
No ROP repeat exam in four weeks if treatment for:
no ROP repeat exam in three months Zone I any stage with plus
ROP in Zone III repeat in two weeks Zone I Stage 3, no plus
Zone II Stage 2 or 3 with plus

Prethreshold ROP (Zone I or II) repeat in
one week if threshold ROP treatment
B1037_Ch-05.indd 246 12/28/2010 9:35:36 AM

5. What treatments are available? Indirect laser photocoagulation

Prevention: PaO2 < 80 mmHg Vitamin E therapy:
Cryotherapy: Controversial
Ablate avascular retina anterior to ridge Inhibit gap junction formation in spindle cells
Multicenter Cryotherapy for ROP Study Antioxidant
(Arch Ophthalmol 1996;114:417424): Complications (necrotizing enterocolitis,
50% reduction in poor VA with vitreous hemorrhage)
cryotherapy Stage 4 ROP might require sclera buckle or
50% reduction in poor fundal status with vitrectomy
cryotherapy
Clinical approach to dragged disc

There is dragging of the optic disc by temporal vitreoretinal fibrotic tissues.
There is also a divergent squint seen.
Id like to ask patient for a history
Prematurity
Contact with dogs
Family history of blindness
The possible causes are:
1. Proliferative vitreoretinopathy
ROP
Familial exudative vitreoretinopathy (AD inheritance)
Incontinentia pigmenti (SLD inheritance)
2. Uveitis
Toxocara
Pars planitis
3. Tumor
Combined hamartoma of RPE and retina
4. Trauma
Notes
Why not earlier?
Limited value in picking up ROP
Difculty in screening (poor pupil dilation, vitreous haze)
Complications of mydriatic eyedrops (cardiac, respiratory effects) and
ocular examination (oculocardiac reex, hypotension, apnea)
Exam tips:
Refer to ETROP study (Arch of Ophthalmol 2003;121:16841696).
B1037_Ch-05.indd 247 12/28/2010 9:35:37 AM

TOPIC 13
OTHER RETINAL
VASCULAR DISORDERS
Q Opening Question: What are the Ocular Effects of Pregnancy?

Ocular Effects of Pregnancy
1. Physiological Coagulation disorders
Lid Telangiectasia Disseminated intravascular coagulation
Cornea Thrombotic thrombocytopenic purpura
Decreased corneal sensitivity 3. Preexisting conditions (endocrine and tumors)
Corneal edema increased corneal Diabetic retinopathy
thickness change in refractive error Increased incidence of background DR
Increased incidence of Krukenbergs spindle Increased incidence of macular edema
IOP Increased facility of aqueous outow Increased progression to PDR
and decreased episcleral venous pressure Prophylactic photocoagulation important
lower IOP Graves disease
Lens Transient loss of accommodative ability Progression during pregnancy
Enlarging pituitary gland various VF changes Pituitary adenoma
towards end of term Normal pituitary gland enlarges with
increased prolactin secreting cells
2. Pathological (5 Cs)
May present for the rst time during
CSR
pregnancy
Secondary to hormonal and hemodynamic
Uveal melanoma
changes change in permeability of
Increased size secondary to increased
blood-retinal barrier
melanin-stimulating hormone secretion
Resolve post-partum with residual RPE
mottling and pigmentation Meningioma
Secondary to increased estrogen/progesterone
CRVO, CRAO, hypertensive retinopathy
May present for the rst time during
Secondary to hypertension
pregnancy
CRVO may be sign of impending t in
pre-eclampsia Ocular pharmacology
Avoid FFA uorescein can pass through
Cortical blindness
placenta
Transient phenomenon, secondary to chronic
Avoid timolol if breastfeeding
edema of occipital lobe
Diamox may be teratogenic
Pseudotumor cerebri
Topical steroids are not contraindicated
Secondary to chronic edema (controversial)
B1037_Ch-05.indd 249 12/28/2010 9:35:37 AM

Exam tips:
Remember the 3 Ps (physiological, pathological, pre-existing) and 5 Cs!
Q What are the Ocular Effects of Radiation?
There are two kinds of radiation: Non-ionizing and Ionizing radiation.

Ocular Effects of Radiation
1. Non-ionizing radiation Anterior segment
Microwave (> 12,000 nm): Lids and conjunctiva:
Cataract Dermatitis of lids
Infrared (12,000770 nm): Damage to eyelashes
True exfoliation of lens (glassblowers Damage to meibomian glands (dry eyes)
cataract) Punctal occlusion (wet eyes)
Cicatricial conjunctivitis
Visible light (760400 nm):
Photic damage: Cornea:
Radiation keratitis
Mechanical (e.g. photodisruption)
Limbal stem cell failure
Thermal (e.g. photocoagulation)
Aseptic necrosis and perforation
Photobiochemical (e.g. solar retinopathy,
photic retinopathy) Lens:

Cataract
Ultraviolet (180390 nm):
Equatorial cells damaged by radiation
Surface epithelial disease and radiation
keratitis Posterior segment
Radiation retinopathy (see below)
2. Ionizing radiation
Optic neuropathy
Damage depends on tissue sensitivity:
Lens > cornea > retina > optic nerve
Damage can be:
Direct on actively reproducing cells
Indirect on blood vessels
Q What is Radiation Retinopathy?
Radiation Retinopathy
1. Pathology Perivascular sheathing, telangiectasia
Damage to retinal vasculature after exposure to Complications
ionizing radiation New vessels vitreous hemorrhage,
Microangiopathy (like DR) tractional RD, neovascular glaucoma
Dose dependent (high risk if > 7,000 rads) CRAO and CRVO
Maculopathy (exudative, edematous,
2. Presentation
ischemic like diabetic maculopathy)
Asymptomatic early on
Papillopathy
Present with decreased VA four months to three
4. Treatment
years after treatment (external beam or local
FFA look for capillary nonperfusion
plaque therapy)
Focal laser and PRP
Progressive loss of VA
Papillopathy: Systemic steroids
3. Clinical ndings
Retinopathy (hemorrhages, cotton wool spots,
hard exudates, microaneurysms)
B1037_Ch-05.indd 250 12/28/2010 9:35:37 AM

Q Tell me about Sickle Cell Hemoglobinopathy.
Sickle cell disease is a red blood cell disorder.

Characterized by the presence of abnormal hemoglobin and sickling of the red blood cells in condi-
tions of hypoxia.
The ocular features can be divided into proliferative retinopathy, nonproliferative retinal disease and
anterior segment disease.
Sickle Cell Hemoglobinopathy
1. Pathogenesis Stage 2: Peripheral arteriovenous anastomosis
Mutant S or C hemoglobin allele: (ridge)
Substituted for normal hemoglobin A allele Stage 3: Sea-fan neovascularization
Valine substituted for glutamate at beta-6 (extraretinal brovascular proliferation)
chain location of hemoglobin Stage 4: Vitreous hemorrhage (subtotal RD)
Hypoxia leads to sickling, which causes Stage 5: RD (total RD)
obstruction of small blood vessels and tissue 4. Non-proliferative retinal disease
ischemia (leading to further sickling) Salmon patch (fresh intraretinal hemorrhage)
2. Types (classied on abnormal hemoglobin Black sunburst (old subretinal hemorrhage)
combinations) Silver wiring of peripheral arterioles
AS (sickle cell trait): Angioid streaks
8% of black population Retinal breaks and detachment
Mild systemic disease 5. Anterior segment
SS (sickle cell anemia): Tortuous corkscrew conjunctival vessels
0.4% of black population Ischemic iris atrophy and rubeosis
Severe systemic disease and anemia Hyphema
Mild ocular disease
6. Management
SC (sickle C disease) and Sthal (sickle cell
Management of hyphema:
thalassemia):
Higher risk of optic nerve damage with
Mild systemic disease
hyphema (compared to normal person)
Severe ocular disease
Indication for surgical intervention: > 24
3. Proliferative retinopathy mmHg > 24 hours
Stage (note: Stages in ROP are shown in RD surgery:
brackets): Risk of anterior segment ischemia with
Stage 1: Peripheral arteriolar occlusion scleral buckle
(demarcation line)
Exam tips:
The stages are VERY SIMILAR to ROP stages (page 245).
Notes
How do you prevent anterior segment ischemia during RD surgery?
Intraoperative oxygen, no epinephrine given
Minimize manipulation of muscles
SRF drainage (lower IOP)
Postoperative oxygen
Consider vitrectomy instead of scleral buckling
Prophylactic laser photocoagulation of all breaks
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Q What are the Ocular Effects of Leukemia?
Ocular effects of leukemia are usually only seen in advanced cases of acute or relapsing disease.
They are related to both direct and indirect effects of leukemia (e.g. anemia, immunosuppression).
Leukemia
1. Direct effects Venous tortuosity and dilatation, CRVO
Anterior segment: Neovascularization
Subconjunctival hemorrhage Exudative RD
Orbital inltration Optic nerve inltration
Iris: 2. Indirect effects
Diffuse white nodular thickening Anemia (ame-shaped hemorrhage, cotton wool
Heterochromia
spots, Roths spots, etc.)
Pseudohypopyon
Thrombocytopenia
Spontaneous hyphema
Hyperviscosity (ischemic optic neuropathy,
Secondary glaucoma
proliferative retinopathy)
Posterior segment:
Immunosuppression (opportunistic infections)
Leopard spot retina (deposits in choroid)
Flame-shaped hemorrhage, hard exudates,
cotton wool spots, Roths spots
Q What are the Causes of Roths Spots?
Roths spots are essentially retinal hemorrhages with a brin thrombus occluding the vessel.
Differential Diagnoses of Roths Spots
1. Blood disorders Sepsis
Anemia AIDs retinopathy
Leukemia Candida retinopathy
Scurvy 3. Vasculitis
2. Infective DM
Infective endocarditis Systemic vasculitis (systemic lupus etc.)
Q What are the Ocular Effects of Renal Disease?
Renal Disease
1. Congenital (concurrent ocular involvement): Posterior polymorphous dystrophy
Lowes syndrome RPE abnormalities (looks like fundus
SLR inheritance albipunctatus)
Renal problems (aminoaciduria, metabolic Aniridia
acidosis, renal rickets) Sporadic form
CNS problems (mental retardation) Renal problems (Wilms Tumor)
Ocular effects: Ocular effects (page 60)
Cataract and microphakia 2. Acquired (ocular involvement occurs LATER)
Glaucoma
Secondary effects (more common, especially
Alports syndrome after renal transplant)
AD inheritance HPT retinopathy
Renal problems (proteinuria, HPT and renal DM retinopathy
failure) Anemia
CNS problems (sensorineural deafness) Bleeding diathesis
Ocular effects: Opportunistic infections (CMV, candida)
Anterior polar cataract, anterior lenticonus
Steroid induced glaucoma and cataract
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Primary effects: Retinal edema

Band keratopathy Disc edema
Cataract Exudative RD
Q Tell me about Hypertensive Ocular Disease.
Hypertension can affect the retina, choroid and optic nerve.

Hypertensive Ocular Disease
1. Hypertensive retinopathy Sclerotic phase

Pathogenesis Exudative phase

Four stages: Complications phase (macroaneurysms,
Vasoconstrictive phase (autoregulatory CRVO)

response) Grading (Keith, Wagener, Barker classication)
Grade Description
1 Mild narrowing or sclerosis of retinal arterioles (silver wiring)
2 Generalized and localized narrowing of arterioles, moderate or marked sclerosis of retinal
arterioles with exaggeration of arteriolar reex and arteriovenous compression (AV nicking)
3 Retinal edema, cotton wool spots, retinal hemorrhages superimposed on sclerotic vessels
4 Diffuse retinal and optic disc edema with narrowing of arterioles (malignant hypertension)
2. Hypertensive choroidopathy 3. Hypertensive optic neuropathy

Elschnigs Pearls (choroidal infarcts) Ischemic optic neuropathy
Clinical approach to macroaneurysm

There is an area of flame-shaped hemorrhage and hard exudates at the
superotemporal arcade.
Associated with a localized dilatation of the retinal arteriole at that location.
Look for
Macular edema/hard exudates at macula
DR
HPT changes
Ill like to ask patient for history of
HPT, DM, hyperlipidemia
In this patient, my management will be
Conservative (if macular is not involved, macroaneurysm is located at
inferotemporal arcade)
To consider laser photocoagulation (macular edema, hard exudate,
superotemporal arcade location)
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Exam tips:
Hypertensive retinopathy is only ONE of the three manifestations.
Notes
Ocular conditions associated with hypertension
Retinal vein occlusion
Retinal artery occlusion
Retinal artery macroaneurysm
AION
Ocular motor nerve palsy
Uncontrolled hypertension may adversely affect DR
Q What is Parafoveal Telangiectasia?
Parafoveal telangiectasia is an uncommon retinal vascular anomaly that primarily involves the
juxtafoveolar capillaries.
It can be congenital or acquired, primary (idiopathic) or secondary to underlying systemic disease
(e.g. diabetes)....
Idiopathic Parafoveal Telangiectasia
Pathogenesis: Group 3A: Associated with systemic disease
Poorly understood (e.g. polycythemia, multiple myeloma etc)
Characterized by dilatation and tortuosity of Group 3B: Associated with neurological
retinal blood vessels and multiple disease
aneurysms conned to the parafoveal Clinical presentation:
region. These leak and result in deposition Patient presents mostly in the 5th to 6th decades
of hard exudates with slowly progressive loss of central vision
Primarily involves the capillary bed, although FFA:
arterioles and venules may also be involved Leakage from telangiectactic vessels conned
Gass classication of parafoveal telangiectasia: to the parafoveal region
Group 1: Unilateral Staining of scars (advanced disease)
Group 1A: Congenital
Treatment:
Group 1B: Idiopathic focal
There is no denitive treatment, although
Group 2: Bilateral several modalities have been studied:
Group 2A: Idiopathic acquired (most
Laser photocoagulation
common) PDT
Group 2B: Juvenile familial occult
Intravitreal anti-VEGF
Group 3: Occlusive Intravitreal steroids
Q What is Coats Disease?
Coats disease is an idiopathic, non-hereditary, mostly unilateral retinal vasculopathy characterized by

the presence of retinal telangiectasias leading to intraretinal and subretinal exudation, and frequently,
exudative RD.
Coats disease, Lebers miliary aneurysms and idiopathic parafoveal telangiectasia are thought to
represent a spectrum of the same disease, with Coats disease being the most extensive.
Before reaching the diagnosis of Coats disease, it is important to exclude retinoblastoma in a young
patient with leukokoria and RD.
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Coats Disease
Clinical presentation: Investigations:
Mostly in the rst decade (average ve years) OCT
with unilateral visual loss, strabismus or FFA:
leukokoria Leakage from telangiectactic vessels
Occasionally may present in later childhood, Hypouorescence: Blockage from hard
and rarely in adult life exudates

Examination: Ultrasonography CT/MRI: Exclude
Retinal telangiectasia retinoblastoma
Intraretinal and subretinal yellowish Management:
exudation: Observation:
Often affects areas remote from vascular Mild, non-vision-threatening disease or
abnormalities, especially macula Total RD with no hope of restoring vision
Complications: Laser photocoagulation: On telangiectatic

Exudative RD vessels
Rubeosis iridis Cryotherapy: For telangiectatic vessels
Uveitis Vitreoretinal surgery: Very bullous RD
Glaucoma unsuitable for cryotherapy
Phthisis bulbi Enucleation: Painful eyes with NVG
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TOPIC 14
RETINITIS PIGMENTOSA
Q Opening Question: What is Retinitis Pigmentosa?

Retinitis pigmentosa refers to a heterogeneous group of photoreceptor dystrophies.
Characterized by a/the triad of.
Retinitis Pigmentosa (RP)
1. Denition ERM
Heterogeneous group of disorders, Atrophic degeneration
characterized by a TRIAD of: Other posterior segment signs, TRIAD of:
Night blindness Optic disc drusen
Progressive visual eld loss from Myopic degenerative changes
photoreceptor and RPE dysfunction PVD
Abnormal ERG ndings Anterior segment signs, TRIAD of:

Keratoconus
Prevalence: 1/4,000 to 1/7,000 in population
Open angle glaucoma
Abnormality of both rods and cones, but rods
Posterior subcapsular cataract
are affected more than cones
3. ERG
2. Clinical features
Amplitude is markedly subnormal:
Symptoms:
Both a and b waves are affected
Bilateral eye involvement, but may be
Both rod and cone ERG are affected
asymmetrical
(although rod abnormality is predominant)
Loss of peripheral vision rst
Night blindness
Parallel EOG abnormality
Signs: 4. What is atypical RP?
Classical TRIAD of: Variants of RP, characterized by unilateral,
Bone-spicule pigmentation asymmetrical and atypical clinical ndings
Arteriolar attenuation (earliest feature) Four classical atypical RPs:
Waxy optic disc pallor (least reliable Retinitis punctata albescens
feature) Sector RP
Macular involvement, TRIAD of: Pericentric RP
CME Exudative RP
Exam tips:
Remember the different TRIADS!
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Clinical approach to retinitis pigmentosa

On examination of this young patients fundus.
The most obvious lesions are bone spicule-like pigmentations.
Distributed along the vascular arcades in the peripheral retina.
The retinal vessels are attenuated.
The optic disc is pale and waxy in appearance (not common, so be careful
here).
Look for
Macula:
CME
ERM
Atrophic scar
Optic disc:
Peripapillary atrophy (myopia)
Optic disc drusen
Other eye:
Bilateral (if unilateral, think of pseudo RP)
Id like to
Examine anterior segment for evidence of keratoconus, cataract
Check IOP (glaucoma)
Check EOM and presence of ptosis (Kearne-Sayre syndrome)
Examine systemically for diseases associated with RP:
Hearing (Usher, Refsums, Kearne-Sayre syndrome)
Neurologically (Bassen-Kornzweig, Refsums, Kearne-Sayre syndrome)
Cardiac (Kearne-Sayre)
Examine family members for RP
Q What are the Differential Diagnoses for RP (Causes of Pseudo RP)?
1. Drugs Laser PRP scars

Quinine / hydroxychloroquine RD
Phenothiazine Trauma
2. Infective Uveitis (Vogt-Koyanagi-Harada syndrome)
Syphilis 4. Vascular
Rubella CRAO
Measles Ophthalmic artery occlusion
3. Scarring 5. Cancer-related retinopathy
Chronic CSR
Exam tips:
Pseudo RP is usually unilateral, asymmetrical and atypical. Remember
DISC.
Do not confuse pseudo RP with atypical RP.
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Q Tell me about the Genetics of RP.
Different mutations have been isolated for RP.

Molecular Genetics of RP
1. Genes for normal retinal proteins 2530% of AD type of RP
Rhodopsin (Chromosome 3) Rhodopsin, Pro347LeuHis mutation:
Red and green pigments (Chromosome X) Less common
Blue pigments (Chromosome 7) Poorer visual prognosis than rhodopsin,
2. Rhodopsin mutations Pro23His mutation

Rhodopsin, Pro23His mutation: RDS (retinal degeneration slow) gene mutation:
Classic molecular genetic defect Encodes for peripherin
Substitution of histidine with proline at 23 Nonsense mutation in rhodopsin:

amino acid position In AR type of RP
Q How do You Provide Genetic Counseling Advice to Patients with RP?
Genetic Counseling
1. AD 2. AR/isolated RP
20% of all RP Worst prognosis
Dened as three consecutive generations of Legally blind by 3040 years
parent-to-child transmission 3. SLR
Best prognosis Same visual prognosis as AR
Retain VA after 60 years If patient is male, all sons will be normal, all
Affected patient has one in two chances of daughters will be carriers
passing defect to child
Q What are the Systemic Associations of RP?
There are numerous systemic diseases associated with RP.

These disorders have in common several features.
Systemic Associations
1. Common features 3. Bassen-Kornzweig syndrome
Systemic: Key features, TRIAD of:
Inherited as AR condition Ataxia
Mental handicap Acanthocytosis (red blood cell abnormality)
Neurological abnormalities Abetalipoproteinemia (fat malabsorption)
Metabolic abnormalities Treatment:
Skeletal abnormalities Vitamin E may be benecial (page 413)
Deafness (fairly common)
4. Refsums syndrome
Pigmentary retinopathy:
Key features, TRIAD of:
Usually unilateral, asymmetrical and atypical
Phytanic acid metabolic defect
VA may be normal
Peripheral neuropathy
ERG may be normal
Palpitations (cardiac arrhythmia)
2. Kearns-Sayre syndrome
5. Usher syndrome
Ptosis
Deafness
Chronic progressive external
Ataxia (vestibular dysfunction)
ophthalmoplegia
Neurological abnormalities
Heart block
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6. Bardet-Biedl syndrome 7. Laurence-Moon syndrome

Key features, TRIAD of: Key features, TRIAD of:
Obesity Spastic paraplegia
Hypogenitalism Hypogenitalism
Polydactyly Mental handicap
Exam tips:
This is potentially a difcult question. Discuss rst only systemic diseases you
are familiar with (e.g. start with Kearns-Sayre syndrome).
The triad of Bassen-Kornzweig can be remembered by A.
Q What Ocular Conditions are Associated with Deafness?
Ocular Associations of Deafness

1. Systemic diseases associated with pigmentary 3. Uveitis
retinopathy Congenital syphilis
Usher syndrome Congenital rubella
Kearns-Sayre syndrome Vogt-Koyanagi-Haradas syndrome
Refsums syndrome 4. Interstitial keratitis
Bardet-Biedl syndrome Cogans interstitial keratitis
Mucopolysaccharidoses (page 118) 5. Metabolic diseases
2. Retinal dystrophies DIDMOAD (diabetes insipidus, diabetes
Lebers congenital amaurosis (page 219) mellitus, optic atrophy and deafness)
Norries disease
Alports syndrome (page 210)
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TOPIC 15
FLECK RETINA
SYNDROMES AND
RELATED DYSTROPHIES
Q Opening Question: What are Retinal Dystrophies?

Group of genetically heterogeneous disorders involving the retina.
Isolated abnormality in otherwise normal patients or may be associated with systemic abnormalities.
Retinal Dystrophies Classication
1. Stationary (congenital stationary night blindness) Widespread (entire retina) or focal (macula)
or progressive (most others) 3. Age of onset
2. Anatomical Congenital, infantile or childhood
Diffuse or isolated (photoreceptor, RPE,
choroidal or vitreous)
Q What are the Fleck Retina Syndromes?
The classical causes of Fleck retina syndromes are Stargardts disease, fundus avimaculatus and familial
dominant drusen.
Other causes include.
Fleck Retina Syndromes
1. RPE dystrophies (classic eck retinas) Fundus albipunctatus
Stargardts disease Congenital stationary night blindness
Fundus avimaculatus syndrome with abnormal fundus (see below)
Familial dominant drusen 3. Posterior uveitis
Others: Presumed ocular histoplasmosis syndrome
Pattern dystrophy Birdshot disease
Fleck retina of Kandori
4. Others
Alport syndrome
Crystalline retinopathy
2. Photoreceptor dystrophies Peau dorange (pseudoxanthoma elasticum)
Retinitis punctata albescens
Atypical RP
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Q What are the Causes of Night Blindness?
Night blindness can be classied as stationary or progressive.

Night Blindness
1. Stationary night blindness Patients demonstrate Mizuos phenomenon
Congenital stationary night blindness (CSNB) (retina exhibits yellow sheen with light
with normal appearing fundus exposure, which disappears with dark
CSNB with abnormal looking fundus adaptation)
Fundus albipunctata (What is fundus 2. Progressive night blindness
albipunctata?): Retinal dystrophies:
Form of CSNB with abnormality in visual RP and RP variants
pigment regeneration Choroidal dystrophies:
Patients have slow dark adaptation Gyrate atrophy
Oguchis disease (What is Oguchis disease?): Choroideremia
Form of CSNB with abnormality in retinal
Vitreous dystrophies:
circuitry Goldmann-Favre disease
Q What are the Causes of Blindness at Birth?
The different causes can be classied into those with gross abnormalities, those with.
Differential Diagnoses of Poor Vision at Birth
1. Gross ocular abnormality Photoreceptor dystrophies:
Bilateral cataract Lebers congenital amaurosis
Bilateral glaucoma Achromatopsia (severe photophobia)
Bilateral retinoblastoma CSNB
2. Normal looking eyes with nystagmus 3. Normal looking eyes with no nystagmus
Optic nerve hypoplasia (septooptic dysplasia) Severe ametropia
Macula diseases: Cortical blindness
Foveal hypoplasia (idiopathic, congenital Ocular motor apraxia
albinism, aniridia) Delayed visual maturation (VA usually normal
Juvenile retinoschisis by six months)
Infectious disease (toxoplasmosis, CMV retinitis)
Notes
How do you differentiate Lebers congenital amaurosis, achromatopsia
and CSNB?
ERG:
Lebers: Decreased rod and cone function
Achromatopsia: Decreased cone function
CSNB: Absent bipolar cell function (decreased b wave)
Q What is Lebers Congenital Amaurosis?
Lebers Congenital Amaurosis

1. Denition: Common cause of blindness in children (between
Age-related variant of RP, involving rods and 1018 % of children in blind institutions)
cones Inheritance: AR (some AD cases have been
reported)
B1037_Ch-05.indd 262 12/28/2010 9:35:41 AM

2. Presentation: Optic disc pallor and retinal arteriolar

Poor vision or blindness at birth or rst few attenuation is uncommon
years of life Disc edema and Bulls eye maculopathy
Nystagmus, roving eye movement, strabismus Other ocular features:

Oculodigital syndrome: Hypermetropia
Constant rubbing of eyes leading to Keratoconus, keratoglobus
enophthalmos Cataract
Children see best under bright light Systemic features:

Mental handicap, deafness, epilepsy and
Pupillary reactions to light diminished
other neurological abnormalities
(amaurotic pupil)
4. ERG
3. Clinical features:
Markedly diminished, even in early cases with
Fundus usually normal looking
normal fundal appearance
Abnormal signs include:
Peripheral chorioretinal atrophy with
pigmentary changes but classical bone
spicule pattern is uncommon
Notes
What are the causes of Bulls eye maculopathy?
Ocular disease:
Cone dystrophy
Lebers congenital amaurosis
Stargardts disease
Systemic disease:
Chloroquine toxicity
Bardet-Biedl syndrome
Q Tell me about Ocular Albinism.
Ocular Albinism
1. Pathogenesis Decreased number of uncrossed nerve
Deciency of tyrosinase (enzyme converts bers (abnormal binocular vision)
tyrosine to dopaquinone) Abnormal visual pathway (from lateral
Biochemical pathway: Phenylalanine geniculate body to occipital cortex)

L-tyrosine L-DOPA dopaquinone Oculocutaneous (tyrosinase positive with
melanin variable melanin)
Milder version
2. Classication
Positive hair bulb test:
Oculocutaneous (tyrosinase negative with no Hair bulb will darker when incubated in a
melanin at all) solution with L-dopa or L-tyrosine
AR Associated systemic diseases:
Very pale skin and blond hair Chediak-Higashi syndrome (white blood
Ocular features: cell defect)
Translucent, diaphanous iris
Hermansky-Pudlak syndrome (platelet defect)
Axenfeld anomaly
Skin solar keratosis, basal cell CA,
Depigmented fundus
squamous cell CA
Fovea hypoplasia
Ocular (decreased melanosomes):
Optic disc hypoplasia
SLR or AR
Refractive errors
Ocular features only
Neuro-ophthalmic features:
No systemic features
Nystagmus
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Q What is Gyrate Atrophy?
Gyrate Atrophy
1. Denition: Differential diagnoses:
Inborn error of metabolism Choroideremia (SLR, earlier onset,
AR diffuse granular pigmentary changes)

Reduced activity of ornithine ketoacid High myopia (lacquer cracks, Foster-
aminotransferase (mitochondria enzyme which Fuchs spot, peripapillary atrophy)

Generalized choroidal atrophy (AD,
catalyzes reactions in several amino acid
pathways) widespread choriocapillaris atrophy)
Myopia and astigmatism (90%)
Gene for enzyme Chromosome 10
Posterior subcapsular cataract
2. Pathogenesis: Abnormal ERG and EOG
Photoreceptor atrophy with abrupt transition to Abnormal FFA (window defects)
near normal retina 4. Investigations:
Not caused by high levels of ornithine (other Raised ornithine levels in aqueous, blood,
metabolic disorders with high ornithine levels spinal uid and urine
do not develop similar changes) Reduced enzyme activity in different tissues
3. Clinical features: (cultured broblasts, lymphoblasts and hair
Symptoms: roots)
Onset 1030 years Carriers have 50% of normal enzyme activity
Night blindness in rst decade
5. Treatment:
VF defects
Rarely decrease in VA
High dose Vitamin B6 (cofactor in ornithine
Signs: aminotransferase, stimulates enzyme activity)
Characteristic chorioretinal atrophic changes Protein restriction (reduces arginine levels)
(patchy chorioretinal atrophy with scalloped Lysine and a-aminoisobutyric acid supplement
borders) (augmentation of renal losses of ornithine)
Q What is Juvenile Best Disease?
Juvenile Best disease is also termed vitelliform macular dystrophy.

It is a rare autosomal dominant disorder, with macular lesions that evolve through several stages over
many years.
Juvenile Best Disease
1. Pathophysiology Stage 3 (pseudo-hypopyon): At puberty:
Disorder in the RPE lipofuscin accumulation RPE break accumulation of yellow
in RPE cells and sub-RPE space secondary substance in subretinal space
photoreceptor loss Stage 4 (vitelliruptive): Egg yolk begins to break
2. Inheritance up (scrambled egg), VA drops:
Pigment clumping and early atrophic changes
AD with variable penetrance
may be present
Bestrophin (Chr 11q13) implicated
Stage 5 (atrophic): Marked decrease in VA
3. Stages
4. Investigations
Stage 0 (pre-vitelliform): Subnormal EOG,
EOG: Severely abnormal during all stages
asymptomatic, normal fundus
ERG: May be normal
Stage 1: Pigment mottling at macula
FFA: Hypouorescence (blockage by lipofuscin),
Stage 2 (vitelliform): Well-circumscribed, round
window defects
egg-yolk (sunny side up) macular lesion:
Infancy or early childhood 5. Prognosis
VA not impaired Reasonably good till fth decade
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Causes of poor VA: Geographic atrophy

CNV Macular hole RD
Macular scarring
Q What are Stargardts Disease and Fundus Flavimaculatus?
Stargardts disease and fundus avimaculatus are genetically inherited retinal dystrophies. They are
variants of the same disease, which present at different times and carry different prognoses.
Fundus avimaculatus presents in adult life and may not have macular changes, while Stargardts disease
presents in the rst and second decades of life, and mainly affects the macula.
Stargardts Disease and Fundus
Flavimaculatus
Geographic atrophy (Bulls eye atrophy) in
1. Inheritance advanced Stargardts disease
AR: ABCA4 gene (1p21-22)
4. Investigations
2. Presentation ERG: Photopic ERG may be subnormal
Stargardts disease presents in the rst and EOG: May be subnormal
second decades of life with impairment of FFA:
central vision Dark choroids
Fundus avimaculatus often presents in adult Flecks may show increased or reduced
life and may be an incidental nding as vision uorescence
may not be impaired Window defects: May have Bulls eye
3. Signs conguration in Stargardts disease

Early ndings: Non-specic macular mottling 5. Prognosis
Pisciform ecks at level of deep retina: Poor for Stargardts disease
Stargardts disease: Flecks located mainly at Relatively good for fundus avimaculatus
the macula, snail-slime or beaten-bronze Small minority develop CNV
foveal appearance
Fundus avimaculatus: Flecks at the mid-
periphery, may involve posterior pole
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Section 6
NEUROOPHTHALMOLOGY
B1037_Ch-06.indd 267 12/22/2010 7:01:36 PM

TOPIC 1
OCULAR MOTILITY AND

MULTIPLE CRANIAL
NERVE PALSIES
Possible Clinical Cases

1. Neurological lesions Browns syndrome
CN III, IV, VI palsies, multiple CN palsies Moebius syndrome
Inter-nuclear ophthalmoplegia (INO), 3. Others
one-and-a-half syndrome Myasthenia gravis
Dorsal midbrain syndrome Thyroid eye disease
Nystagmus Blow-out fracture
2. Pediatrics problems Myopathies
Esotropia/exotropia
Duanes syndrome
Clinical approach to ocular motility examination

Examine this patients ocular motility.
Look at
Head posture:
Head turn towards side of abduction weakness, e.g. Duanes
syndrome, VI CN palsy
Head tilt away from side of SO palsy
Chin down in bilateral SO palsy, chin up in bilateral ptosis
Ptosis III CN palsy
Primary position manifest strabismus, e.g. ET, XT, SO palsy
Pupils anisocoria
Check versions (both eyes) and ductions (one eye)
(Continued )
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(Continued )
Test EOM in all nine positions of gaze
Primary
Two horizontal
VI CN palsy (look at abducting eye)
INO (look at ADDUCTING eye)
Duanes syndrome (look at palpebral aperture)
If INO is found on one side, check for one-and-a-half
Two vertical
III CN palsy
Supranuclear gaze palsy
Thyroid (IR restriction)
Blow-out fracture (IR restriction)
A and V patterns if ET/XT present
Four vertical in abduction and adduction

SO palsy (depression in adduction)
Browns syndrome (elevation in abduction)
Park Bielchowskys three-step test

Primary position
Head turn
Head tilt
Clinical approach to diplopia

This patient has diplopia. How do you approach the diagnosis?
Id like to ask the patient the following questions
Blurring of vision or diplopia?
Uniocular or binocular? (Disappear on covering one eye)
Vertical, horizontal or oblique displacement of images?
Is diplopia worse in any position of gaze?
Test
EOM, asking for diplopia
Cover paretic eye in position of maximal separation of images
(outermost image will disappear)
Q Opening Question: What are the Possible Causes of Multiple Cranial Nerves Palsies?
Cavernous Sinus Syndrome (III, IV, V, VI CN)
1. Clinical features Orbital apex
Pure cavernous sinus III, IV, VI plus V1 and II
III, IV, VI plus V1, V2, V3, Horners syndrome 2. If VI nerve involved, either
(depending on extent of involvement) Cavernous sinus syndrome (look for III, IV, V CN
Superior orbital ssure palsies and Horners syndrome)
III, IV, VI plus V1
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Cerebellopontine angle syndrome (look for V, Nasopharyngeal CA

VII, VIII CN palsies and cerebellar signs) Cholesteatoma
Clivus meningioma
3. Etiology (note: Classic big four in pathology)
Pontine glioma
Vascular
V CN neuroma
Aneurysm intracavernous sinus carotid
Trauma
aneurysm, post-cerebral aneurysm
Basal skull fracture
Cavernous sinus thrombosis
Migraine 2. Clinical features of vestibular schwannoma:
Giant cell arteritis V and VIII CN rst
Inammatory Corneal reex lost
Tolosa-Hunt syndrome Nystagmus
Meningitis VI and VII CN next
Bacterial syphilis, TB Raised intracranial pressure (papilledema, etc.)
Viral herpes zoster
Lateral Medullary Syndrome (V, VIII, IX, X CN)
Wegeners granulomatosis
Sarcoidosis 1. Etiology
Tumor Stroke
Primary-pituitary adenoma, meningioma, Multiple sclerosis
craniopharyngioma 2. Clinical features
Secondary nasopharyngeal CA, V CN and spinothalamic tract (crossed
lymphoma, distant metastasis hemihypalgesia)
Trauma VIII CN (nystagmus)
Carotid-cavernous sinus stula
IX and X CN (dysarthria and dysphagia)
Cerebello-Pontine Angle (V, VI, VII, VIII CN) Sympathetic tract (Horners syndrome)
1. Etiology Cerebellum (nystagmus and other cerebellar signs)
Tumor
Vestibular schwannoma
Clinical approach to multiple cranial nerves palsies

On examination of this patients ocular motility, there is generalized limitation
in all positions of gaze.
Look for
Proptosis carotid cavernous fistula, Tolosa-Hunt syndrome, thyroid eye
disease, pseudotumor
Conjunctival injection carotid cavernous fistula, pseudotumor
Posture of head
Ptosis III CN palsy, Horners syndrome
Primary position manifest strabismus
Pupils anisocoria
Check
EOM
Horizontal (VI CN)
Vertical (III CN)
Intorsion at abduction (IV CN)
Close eyes (VII CN)
Check pupils (II CN)
Facial sensation (V1, 2, 3 CN), open jaws (V3 CN)
(Continued )
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(Continued )
Exclude
Thyroid eye disease
Myasthenia gravis
Ill like to
Check corneal sensation (V1 CN)
Check fundus for papilledema
Examine other cranial nerves
VIII, cerebellar signs (cerebellopontine angle syndrome)
Refer to ENT to rule out nasopharyngeal CA
Exam tips:
For examinations purposes, there are three syndromes of ophthalmic interest
(cavernous sinus, cerebellopontine angle and lateral medullary syndromes).
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TOPIC 2
THIRD CRANIAL
NERVE PALSY
Q Opening Question: Tell me about III CN Palsy.

III CN palsy is a common neuroophthalmic diagnostic problem.
The causes can be divided into.
Clinically, III CN palsy can be either medical or surgical III.
Anatomy Etiology Clinical features

Nuclear: Vascular (stroke) Nuclear III syndrome:
Midbrain Demyelination Unpaired levator nucleus (bilateral ptosis)
Level of superior (multiple sclerosis) Unpaired Edinger-Westphal nucleus
colliculus Tumors (bilateral mydriasis)
Ventral to sylvian Paired SR nuclei supplying contralateral
aqueduct muscle (contralateral SR palsy)
Paired MR, IR, IO (ipsilateral MR, IR, IO palsies)
Fasciculus: Webers syndrome Crossed syndromes:
Through red Benedicts Pyramidal tract (III CN palsy and contralateral
nucleus syndrome hemiparesis)
Emerge from midbrain Nothnagels Red nucleus (III CN palsy and contralateral
medial to cerebral syndrome hemitremor)
peduncle Claudes Superior cerebellar peduncle (III CN palsy
syndrome: and contralateral cerebellar ataxia)
Benedicts + Isolated III CN palsy
Nothnagels
Basilar: Aneurysm
Between posterior (between posterior
cerebral and superior communicating and
cerebellar arteries internal carotid)
Lateral and parallel Raised intracranial
to posterior pressure (uncal
communicating artery herniation)
(Continued )
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(Continued )

Intracavernous: Causes of Cavernous Cavernous sinus syndrome (page 224):
Pierces dura lateral to sinus syndrome Multiple CN palsies
posterior clinoid process to (page 224) Incomplete III
enter cavernous sinus May be pupil sparing (DM)
Above IV nerve in lateral wall Isolated III CN palsy
Divides into superior and
inferior branches
Intraorbital: Vascular (DM)
Enters through SOF Trauma
Branch to inferior oblique
carries parasympathetic bers
Clinical approach to III CN palsy

This patient has an exotropia in his right eye.
And fixes with his left eye.
Look for
Ptosis not overcome by frontalis action
EOM limitation in all directions except abduction (VI CN)
Watch for aberrant regeneration
Check intorsion in abduction (IV CN)
Check pupils (pupil sparing or not)
Decide quickly whether you are dealing with medical or surgical III
Medical III Surgical III

Features Age > 60 Young
Vascular risk factors (DM, No vascular risk factors
HPT, smoking) Pupil involved (90%)
Pupil sparing (80%) Progression of pupil
Pupil continues to be spared involvement
after one week Incomplete III CN palsy
Complete III CN palsy Multiple CN palsies
Isolated III CN palsy Aberrant regeneration
No aberrant regeneration No recovery
(see below)
Recovery within 3 months
Common etiology Vascular causes Cerebral aneurysm
DM Raised intracranial
Giant cell arteritis (GCA) pressure (from uncal
Ophthalmic migraine herniation)
Inammatory Tumor
Tolosa-Hunt syndrome
Miller Fisher syndrome
(Continued )
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(Continued )
Medical III Surgical III
Evaluation History of DM and HPT Check fundus for
Check BP (HPT) papilledema uncal
Headache (GCA, ophthalmic herniation
migraine) Examine
Painful III CN palsy neurologically
(DM, migraine, History of head injury
Tolosa-Hunt syndrome) History of headache,
Ataxia, areexia nausea and vomiting
(Miller Fisher) (raised intracranial
pressure)
History of HPT
(aneurysm)
Investigations CBC, ESR CT scan (CNS bleed,
Fasting blood sugar level meningioma,
VDRL, FTA stroke, trauma)
Autoimmune markers
Exam tips:
There are two ways to remember etiology:
Anatomical classication is good for answering essay question.
Medical/surgical III classication is good for viva/clinical exams.
Q How do You Manage III CN Palsy?
The management of III CN palsy involves treating the underlying cause, and addressing the diplopia.
1. Underlying cause 2. Diplopia
Ligation of aneurysm (endoscopic/surgical) Non-surgical: Fresnel prisms, uniocular
Optimize control of diabetes/hypertension/ occlusion
hyperlipidemia if the etiology of III CN palsy is Strabismus surgery (at least 6 months after onset,
vascular when all spontaneous improvement has ceased)
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Q Tell me about Aberrant Regeneration.
Aberrant regeneration of CN usually follows damage of the nerve by TRAUMA or TUMOR.

There are recognized syndromes involving III, VII and IX CN.
Aberrant Regeneration of CN
1. Aberrant regeneration of III CN palsy: 2. Aberrant regeneration of VII CN palsy
Lid gaze dyskinesis: (crocodiles tears):
Elevation of lid on adduction (inverse Synkinesis between salivation bers and
Duanes syndrome) lacrimation bers
Elevation of lid on depression (pseudo Von
Tearing when eating
Graefes sign)
3. Aberrant regeneration of IX CN palsy
Pupil gaze dyskinesis:
Constriction on adduction (pseudo Argyll (Freys syndrome):
Robertson pupil) Synkinesis between salivation bers
Constriction on depression and sympathetic bers
Flushing and sweating when eating
B1037_Ch-06.indd 276 12/22/2010 7:01:39 PM

TOPIC 3
SIXTH CRANIAL
NERVE PALSY
Q Opening Question: Tell me About VI CN Palsy.

VI CN palsy is a common neuroophthalmic diagnostic problem.

Nuclear: Vascular (stroke) Nuclear VI syndrome
Mid-pons, ventral to Demyelination (multiple and gaze palsy:
oor of fourth ventricle sclerosis) Abducens nucleus
VII CN fasciculus Tumors (ipsilateral LR palsy)
curves around Encephalitis PPRF (ipsilateral failure of
VI CN nucleus horizontal gaze)
Facial nucleus (ipsilateral VII
CN palsy)
Fasciculus: Raymonds syndrome Crossed syndrome:
Leaves brainstem ventrally at Millard Gubler syndrome Pyramidal tract (VI CN palsies
ponto medullary junction Fovilles syndrome and contralateral hemiparesis)
Pyramidal tract and VII
nucleus (VI, VII CN palsy and
contralateral hemiparesis)
V, VII nucleus, sympathetic,
PPRF (V, VI, VII CN palsy, gaze
palsy and Horners syndrome)
Basilar: Causes of cerebellopontine Cerebellopontine angle
Enters prepontine basilar cistern angle syndrome (page 225): syndrome (page 225)
Passes upwards close to base Vestibular schwannoma
of skull, crossed by anterior Raised intracranial pressure
inferior cerebellar artery (false localizing sign)
Pierces dura below clinoids Nasopharyngeal CA
Angles over petrous Basal skull fracture
temporal bone Clivus meningioma
Passes through Dorellos canal Gradenigos syndrome
and enters cavernous sinus
(Continued )
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(Continued )

Intracavernous: Causes of Cavernous sinus Cavernous sinus syndrome
Below III and IV CN syndrome (page 224) (page 224)
Close to internal carotid artery
in the middle of the sinus
Carries sympathetic branches
from paricarotid plexus
Intraorbital: Vascular (DM) Isolated VI CN palsy
Enters orbit through SOF within Trauma
annulus of Zinn to innervate LR
Clinical Notes
1. Isolated medical VI palsy 3. Bilateral VI CN palsy
Same workup as for medical III CN palsy Nuclear:
(page 228) CVA
Recovery within 46 months MS

Tumor
2. Six causes of pseudo VI CN palsy
Encephalitis (Wernickes syndrome)
Myasthenia gravis
Basilar:
Thyroid eye disease False localizing sign
Duanes syndrome Clivus meningioma
Medial wall fracture Intracavernous (big four in pathology):
Esotropia (longstanding) Inammation (Tolosa-Hunt)
Convergence spasm Vascular
Tumor (nasopharyngeal CA)
Trauma (carotid-cavernous sinus stula)
Clinical approach to VI CN palsy

This patient has a right esotropia with abduction weakness.
Look for other CN
EOM (III)
Check intorsion in abduction (IV)
Check pupils (II)
Ill like to
Check fundus for papilledema (false localizing, pseudotumor)
Examine neurologically:
Contralateral hemiparesis (Raymonds syndrome)
VII CN and contralateral hemiparesis (Millard Gubler syndrome)
Horizontal gaze palsy, V, VII, Horners syndrome (Fovilles syndrome)
V, VII, VIII, cerebellar signs (cerebellopontine angle tumor vestibular
schwannoma)
Check ears for otitis media (Gradenigos syndrome) and battle sign (petrous
bone fracture)
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Exam tips:
Do not confuse raised intracranial pressure causing uncal herniation (III CN
palsy) with false localizing sign (VI CN palsy).
Q What are Causes of VI CN Palsy in Children?

VI CN palsy is a common neuroophthalmic diagnostic problem in children.
First, a squint (ET, Duanes syndrome) must be excluded.
1. Congenital
Isolated idiopathic Tumor:
Moebius syndrome Pontine glioma
Metastasis form neuroblastoma
2. Acquired
Trauma
Infection:
Gradenigos syndrome (otitis media,
V, VI, VII, VIII CN palsies)
Q What is the Surgical Management of VI CN Palsy?
Surgical intervention for VI CN palsy is undertaken at least six months after onset, when all spontaneous
improvement has ceased.
The choice of surgery depends on the residual abduction power of LR.
1. Poor residual LR power 2. Some residual LR power

Hummelsheim procedure Ipsilateral MR recession and LR resection
Jensen procedure +/ contralateral MR recession
B1037_Ch-06.indd 279 12/22/2010 7:01:40 PM

TOPIC 4
NEUROLOGICAL
O
APPROACH TO PTOSIS
Decide Quickly If
1. Senile aponeurotic ptosis EOM (SR rectus weakness)
Describe: Bells reex
High lid crease Marcus Gunn Jaw Winking
Atrophic lid tissues and tarsal plate Levator function usually poor
Deep UL sulcus Check the VA and refraction of patient
Test: 3. III CN palsy
Ptosis in downgaze more severe Severe, complete ptosis
EOM full and pupils normal
Eye is out and down
Levator function usually good
Dilated pupils
2. Congenital ptosis See CN III approach (page 228)
Describe:
4. Horners syndrome
Absent lid crease
Mild ptosis, overcome by looking up
Visual axis blocked (if blocked, risk of
amblyopia) Miosis
Test: Enophthalmos
Ptosis in downgaze lid lag present See Horners approach (page 243)
Clinical approach to ptosis

Describe
Unilateral/bilateral
Total/severe/mild
Shine torchlight to look at visual axis
Decide whether anisocoria is present now!
If present, either III CN palsy or Horners syndrome
If absent, either myasthenia gravis, congenital ptosis or senile ptosis
Overaction of frontalis
Lid crease, lid sulcus, lid mass
Eye position (squints)
Head tilt
(Continued )
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(Continued )
This patient has a mild unilateral ptosis in the right lid.
Associated with smaller pupils in the right eye.
or
This patient has severe bilateral ptosis in both lids, covering the
visual axis.
There is no anisocoria noted.
Test
EOM
Upgaze
Ptosis overcome by frontalis (Horners syndrome)
Limitation in upgaze (III CN palsy)
Downgaze (lid lag in congenital ptosis vs aponeurotic, aberrant III CN

regeneration pseudo-von Graefes sign)
Sidegaze (III CN palsy, aberrant III CN regeneration reverse Duanes
syndrome)
Myasthenia gravis tests fatigability in upgaze, Cogans lid twitch,
eyelash sign, eyepeek sign
Lagophthalmos and Bells (important for management purposes)
Pupils
Marcus Gunn Jaw Winking sign
Ill like to
Measure the degree of ptosis and levator function (important for
management purposes)
Perform an SLE and fundal examination
Perform myasthenia gravis tests
Exam tips:
One of the most common clinical examination cases.
See also the ptosis chapter in the oculoplastic section (page 291), III CN
palsy (page 228) and Horners syndrome (page 243).
B1037_Ch-06.indd 282 12/22/2010 7:01:40 PM

TOPIC 5
MYASTHENIA GRAVIS
Q Opening Question: What are the Features of Myasthenia Gravis (MG)?

Myasthenia gravis is a systemic neurological disorder.
Caused by a disorder occurring at the neuromuscular junctions.
It has both systemic and ocular features.
Myasthenia Gravis
1. Classication: Not consistent with single CN palsy (mimic
Ocular: any of CN palsy)
60% of MG patients present with ocular MR rst muscle to be involved (therefore
features; 90% will have some ocular need to exclude internuclear
involvement in course of disease ophthalmoplegia)
Prognosis (important): Saccadic abnormalities: Hypermetric and
40% remain ocular hypometric saccades
10% remission Accommodative fatigue
50% progress to generalized MG Orbicularis oculi weakness:
Generalized: Eyelash sign (eyelash cannot be buried by
Mild forcible lid closure)
Severe acute (respiratory) Eye peek sign (lids drift open slowly after
Severe chronic closure)
2. Natural history: 4. Diagnosis:
Labile phase Tensilon test (80% sensitivity)
Slow progressive phase Electromyogram (EMG)
Refractory phase Repetitive stimulation at 3 mHz (5090%
sensitivity)
3. Ocular features (in 90%):
Look for decremental amplitude with
Ptosis:
repetitive stimulation
Fatigable:
Single ber EMG (8095% sensitivity)
Enhanced with light and passive elevation
Look for variability between individual
of contralateral lid
muscle bers within a motor unit
Asymmetrical
Anticholinesterase antibody (7090%
Variable (different time of day)
Shifting (left and right eyes)
sensitivity): No correlation with disease severity
Cogans lid twitch Anti-skeletal muscle antibody:
Lid hopping/uttering Increased in females and patients with
Ophthalmoplegia: thymomas
Increased risk of bulbar involvement
Pupils normal
Exam tips:
A differential diagnosis for almost any neuroophthalmic condition.
B1037_Ch-06.indd 283 12/22/2010 7:01:41 PM

Notes
Ossermans grading
I: Ocular
II: Generalized
III: Fulminant and crisis
IV: Late progressive
V: Muscle atrophy
Q How do You Perform the Tensilon Test?
The tensilon test is a diagnostic test for MG with sensitivity of 80%.

Tensilon Test
1. Preparation: 3. Endpoint:
Edrophonium hydrochloride: Most respond within 2045 seconds
Anti-acetylcholinesterase (e.g. increases Objective endpoints must be determined:
acetylcholine effects at neuromuscular Ptosis (measured before and after)
junction) Ophthalmoplegia (Hess chart or
One vial 10 mg/ml Lancaster Red-Green Test)
Dilute to 10 ml (concentration: 1 mg/ml)
4. Side effects:
Precaution: Increased salivation
Atropine 0.51 mg on standby
Sweating
Consider giving IM 0.4mg 15 min before test
Perioral fasciculation
Resuscitation equipment on standby
Nausea
2. Injection (rule of 2s):
Hypotension, bradycardia, arrhythmia
IV 2 mg, watch for 2 min
Bronchospasm
If no response, inject another 2 mg, watch for
5. False positive result: Eaton-Lambert syndrome,
further 2 min
If still no response, inject remaining 6 mg, Guillain-Barre syndrome, amyotrophic lateral
watch for 2 min again sclerosis
Exam tips:
One of few procedures in neuroophthalmology you need to know well.
Q How to Manage a Patient with MG?
MG is usually co-managed with a neurologist.

Need to treat both the systemic condition and the ocular complications.
Management of MG
1. Systemic manifestations: Immunosuppressive therapy:
Anti-acetylcholinesterase (pyridostigmine/ Prednisolone:
mestinon): Given to reduce dose of
30 mg bid dose pyridostigmine

65% remission, 30% improvement
Thymectomy:
Better for generalized MG than ocular MG Azathioprine
B1037_Ch-06.indd 284 12/22/2010 7:01:41 PM

Plasmapheresis and IV 2. Ocular complications:

immunoglobulin indications: Lid crutches for ptosis
Myasthenic crisis: Prisms for ophthalmoplegia
Notes
Causes of lid retraction in myasthenia gravis:
Contralateral lid retraction when there is unilateral ptosis
Cogans lid twitch
Thyroid eye disease
Q What are the Drugs to Avoid in MG?
Drugs to Avoid in MG
Aminoglycosides (gentamicin) Respiratory depressants (morphine)
Neuromuscular blocker (curare, Procainamide
suxamethonium) Penicillamine
Chlorpromazine
Exam tips:
Remember ABCD and P!
B1037_Ch-06.indd 285 12/22/2010 7:01:41 PM

TOPIC 6
NYSTAGMUS
Q Opening Question: Tell me about Nystagmus.

Nystagmus can be dened as.
A simple classication is.
Nystagmus
1. Denition: Motor imbalance
Ocular oscillation which is .... Congenital nystagmus
Rhythmic in nature and biphasic with at least Spasmas nutans
one slow phase Latent nystagmus
The slow phase is abnormal and the fast Periodic alternating nystagmus
phase is corrective Convergence-retraction nystagmus
The direction is named after the fast phase Downbeat nystagmus
Upbeat nystagmus
2. Classication:
See-saw nystagmus
Clinical:
Ataxic nystagmus
Primary position or gaze evoked
Pendular (two slow phases) or jerk (one quick
Sensory deprivation
phase, one slow phase) Nystagmoid movements:
Ocular utter and opsoclonus
Horizontal, vertical, rotatory or mixed
Ocular bobbing
Conjugate (same in both eyes) or dissociated
Before and after thymectomy
Etiological:
Awaiting response to immunosuppression
Physiological:
Total body irradiation
End-point nystagmus
Optokinetic nystagmus
Vestibular nystagmus
Exam tips:
Nystagmus is a difcult topic. You need to remember the basic principles
and certain types of nystagmus.
Notes
Physiological nystagmus:
End-point nystagmus
Optokinetic nystagmus
Vestibular nystagmus
B1037_Ch-06.indd 287 12/22/2010 7:01:41 PM

Clinical approach to nystagmus

This patient has a nystagmus.
Describe
Direction horizontal/vertical/rotational
Waveform pendular/jerk (direction of fast phase)
Amplitude large/small (effect of gaze position on amplitude)
Rest primary position (at rest/gaze evoked)
Frequency fast/slow
Exam tips:
Remember DWARF.
Q What are the Features of Congenital Nystagmus?
There are several distinct ocular features of congenital nystagmus.

Congenital Nystagmus
1. Classication: Nystagmus is:
Primary/idiopathic Binocular (3)
Secondary Horizontal (4)
Anterior segment disorders Conjugate (5)
Corneal opacity, cataract, glaucoma Uniplanar (6)
Visible posterior segment disorders Associated with:

ROP, optic atrophy, coloboma, macular scar Head tilt (7)
Normal looking eyes: Titubation (8)
Lebers amaurosis Null point (9)
Achromatopsia Dampens on convergence but increases on
Cone dystrophy xation (10)

Congenital stationary night blindness Diminishes in darkness/during sleep/when
eye is covered (11)
2. Ocular features:
Latent nystagmus may be present (12)
Starts at birth or 23 months after birth (1) Paradoxical OKN response (13)
No oscillopsia (2)
Exam tips:
A favorite question. Remember all the 13 points but DO NOT say, There are
13 features. in case you cannot remember all of them!
B1037_Ch-06.indd 288 12/22/2010 7:01:42 PM

Clinical approach to congenital nystagmus

On inspection, this young patient has a pendular nystagmus in the primary
position.
Describe features
Binocular (3), horizontal (4), conjugate (5)
Associated head tilt (7), titubation tremors (8)
Check in different directions of gaze
Uniplanar in all directions of gaze (6)
Null point (7)
Dampens on convergence, but increases on fixation (8)
Cover one eye, observe for latent jerk nystagmus in contralateral eye (11)
This patient has congenital nystagmus.
Id like to
Ask patient for history of onset of nystagmus (1) and whether patient has
symptoms of oscillopsia (2)
Test for paradoxical response to OKN drum (13)?
Q How would You Manage this Patient?
My management will be conservative.

Ill do the following.
Refract and prescribe glasses (reading is not
impaired) I will offer surgery only if there is an abnormal
Prescribe contact lens if glasses are not head posture (AHP) which is stable:
suitable Horizontal AHP: Kestenbaum procedure
Give base-out prism to induce Vertical AHP: Recess and Resect vertical recti
convergence
Q How do You Differentiate a Peripheral from a Central Cause of Vestibular Nystagmus?
There are several distinct features which will help in differentiating peripheral from central cause of
vestibular nystagmus.
Vestibular Nystagmus
Peripheral Central
Nystagmus Unidirectional, away from site of lesion Multidirectional or unidirectional,
May be associated with a rotatory towards side of lesion
component
Association Dampens on xation No dampening
Rarely lasts more than 3 weeks May be permanent
Marked vertigo Mild vertigo
Tinnitus/deafness No tinnitus/deafness
Location Vestibular nerve Cerebellum
Labyrinth Brainstem
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Q Tell me about the Optokinetic Response.
Optokinetic (OKN) nystagmus is induced by looking at the rotation of a striped drum the OKN drum.
There is the initial pursuit eye movement following the direction of the rotation.
This is followed by the saccade corrective movement in the opposite direction.
Use of OKN
1. Diagnosis of congenital nystagmus (paradoxical 5. Differentiate vascular from neoplastic cause
response) in patient with homonymous hemianopia
2. Detect internuclear ophthalmoplegia (rotate (see page 255):
If vascular, lesion is usually conned to
drum in direction of eye with adduction failure)
occipital lobe (OKN response is symmetrical)
(see page 253)
If neoplastic, lesion may extend to parietal lobe
3. Detect Parinauds syndrome (rotate drum
(OKN response is asymmetrical)
downwards to elicit convergence retraction
nystagmus) (see page 244)
4. Differentiate organic from nonorganic blindness
(see page 285)
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TOPIC 7
PUPILS
Possible Clinical Cases

1. Afferent defects Causes:
Clinical problem: RAPD Unilateral:
Optic nerve and tract Tonic pupil
lesions Aberrant III CN regeneration
Herpes zoster ophthalmicus

2. Efferent defects
Afferent conduction defect
Clinical problem: Anisocoria
Bilateral:
Parasympathetic (III CN palsy) or sympathetic Argyll Robertson pupil
(Horners syndrome) Dorsal midbrain syndrome
3. Light near dissociation Dystrophia myotonica
Clinical problem: Reaction to accommodation Diabetes
but not to light Chronic alcoholism
Q Opening Question: What are the Anatomical Pathways of the Pupil Reexes?
There are three important pupil reexes, with different anatomical pathways for each.
Pupil Pathways
1. Light reex pathway CN nasociliary nerve long ciliary
1st order: Retina (ganglion cells) optic nerve iris dilator pupillae pupil dilation
nerve optic tract bypasses lateral 3. Accommodation reex
geniculate body (LGB) Not well dened, the orders are an
2nd order: Pretectal nucleus approximation only (important to emphasize
3rd order: Edinger-Westphal nucleus this in the beginning):
4th order: Ciliary ganglion short ciliary 1st order: Retina (ganglion cells) optic
nerves iris constrictor pupillae pupil nerve optic tract
constriction 2nd order: LGB optic radiation
2. Sympathetic pupillary pathway 3rd order: Visual cortex visual association
1st order: Hypothalamus brainstem areas internal capsule brainstem
2nd order: C8 to T2 spinal cord 4th order: Oculomotor nucleus (MR nucleus
3rd order: Superior cervical ganglion and Edinger-Westphal nucleus) pupil
pericarotid plexus ophthalmic division of V constriction and convergence
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Q What are the Causes of Anisocoria?
Causes of anisocoria depend on which pupil is the abnormal one.

Anisocoria
1. Dilated (mydriatic) pupil abnormal Pancoast syndrome
III CN palsy Cluster headache
Tonic pupil: Argyll Robertson pupil
Holmes Adie syndrome Pharmacological
Pharmacological mydriasis Iris abnormalities:
Iris abnormalities Posterior synechiae
Trauma Pontine hemorrhage
2. Constricted (miosed) pupil abnormal
Horners syndrome:
Brainstem stroke
Q What is the Marcus Gunn pupil?
The Marcus Gunn pupil is also known as the relative afferent papillary defect.
It is elicited with the swinging torchlight test.
There is paradoxical dilation of the pupil when the torchlight is swung from the contralateral eye to the
affected eye.
Marcus Gunn Pupil
1. Etiology: Grade II: No initial constriction, stall, then
Optic nerve lesion (most important) dilatation
Other possible sites: Grade III: Immediate dilatation
Extensive retinal damage Grade IV: Immediate dilatation following
Dense macular lesion prolonged illumination of the good eye for six
Optic chiasma/tract (RAPD in contralateral
seconds
eye because nasal retina larger than
Grade V: Immediate dilatation with no
temporal)
secondary constriction
Dorsal midbrain (RAPD in contralateral eye)
2. Grading (Bell et al. Clinical grading of relative

afferent pupillary defects. Arch Ophthalmol
1993;111:938942):
Grade I: Weak constriction then greater
dilatation
Exam tips:
One of the most important denitions asked in exams.
Q What is Tonic Pupil? What is the Holmes Adie Pupil? What is the Holmes Adie Syndrome?
Tonic Pupil
1. Clinical features: Asymmetrical accommodation
Light-near-dissociation (response to 2. Investigation:
accommodation better than to light) 0.1% pilocarpine (constriction due to
Dilated pupil (pupil small in longstanding Adies) denervation supersensitivity)
Slow constriction and dilatation 3. Site of lesion:
Constriction in segments (bag of worms) Ciliary ganglion or short ciliary nerves
B1037_Ch-06.indd 292 12/22/2010 7:01:42 PM

4. Etiology: Secondary:
Primary = Holmes Adie pupil: Syphilis (bilateral tonic pupil)
Monocular (80%) DM
Female (80%) Trauma, surgery
Age 2040 Degenerative
Areexia (= Holmes Adie syndrome)
Exam tips:
Tonic pupil Holmes Adie pupil Holmes Adie syndrome.
Q What is Horners Syndrome?
Horners syndrome is a neurological syndrome caused by lesion in the sympathetic pathway

in the head and neck.
Horners Syndrome
1. Clinical features: Apraclonidine 0.5%
Miosis 1 and 2 receptor agonist
Ptosis, inverse ptosis Reverses anisocoria in both central/
Enophthalmos preganglionic and postganglionic Horners
Heterochromia syndrome
Ocular hypotony 3. Site and etiology:
Anhidrosis (if lesion is below superior cervical Central (rst order):
ganglion) Brainstem CVA
Trauma
2. Investigation:
Spinal cord tumor
Cocaine 410%
MS
Blocks reuptake of norepinephrine
Syringomyelia
pupillary dilatation
Lateral medullary (Wallenburg) syndrome
Conrmation test
Preganglionic (second order):
Affected pupil does not dilate
Pancoast tumor
Hydroxyamphetamine 1%
Thyroid CA
Promotes release of norepinephrine from
Vertebral metastasis
terminal axon
Subclavian/aortic/common carotid
Differentiates between central/preganglionic
aneurysm
from postganglionic
Trauma
Postganglionic lesion (3rd order) pupil fails to
Aortic/common carotid dissection
dilate
Postganglionic (third order)
Phenylephrine 1%
Internal carotid dissection
Denervation supersensitivity
Cluster headache
Differentiates central/preganglionic from
Cavernous sinus syndrome (Raeders
postganglionic
syndrome)
Postganglionic lesion pupil dilates more
Nasopharyngeal carcinoma
widely
Exam tips:
The pharmacological tests for Horners syndrome are one of the favorite
exam questions.
A preganglionic or second order Horners syndrome is important because of
the possibility of Pancoast tumor. Therefore, differentiation of central/
preganglionic from postganglionic is important.
B1037_Ch-06.indd 293 12/22/2010 7:01:43 PM

Notes
Causes of acquired Horners syndrome in children:
Neuroblastoma
Thyroglossal cyst
Branchial cyst
Q What is the Argyll Robertson Pupil?
Argyll Robertson Pupil

1. Clinical features: 4. Etiology:
Light near dissociation (response to Syphilis
accommodation better than to light) Pupil signs:
Constricted (miosed) pupil AR pupil or tonic pupil
Pupillary irregularity (iritis)

Speed of constriction and dilatation is normal
Poor dilation to atropine
Bilateral pupil involvement common
Other ocular signs (page 328):
2. Investigation: Interstitial keratitis
Cocaine 410% (affected pupil does Optic atrophy
not dilate) Chorioretinitis
3. Site of lesion: DM
Dorsal midbrain (pretectal interneurons to MS
Edinger-Westphal nucleus involved, ventrally Alchoholism
located accommodative reex neurons being Trauma, surgery
spared) Aberrant III CN regeneration
Comparison Between Tonic Pupil

and Argyll Robertson Pupil
Tonic pupil Argyll Robertson pupil

Demographics Young Old
Female Male
Pupil Dilated Miosed
Unilateral Bilateral
Slow reaction to light and Normal reaction to both
accommodation
Common cause Holmes Adie pupil or syndrome Syphilis
Exam tips:
Argyll Robertson pupil tonic pupil.
Argyll Robertson pupil syphilis.
B1037_Ch-06.indd 294 12/22/2010 7:01:43 PM

Q What is the Dorsal Midbrain Syndrome/Parinauds Syndrome?
Dorsal Midbrain Syndrome

1. Clinical features: 3. Site of lesion:
Light-near-dissociation (response to Dorsal midbrain (pretectal interneurons to
accommodation better than to light) Edinger-Westphal nucleus involved, ventrally
Lid retraction (Colliers sign) located accommodative reex neurons being
Supranuclear gaze palsy (normal vestibular spared, similar to AR pupil)
ocular reex and Bells reex) 4. Etiology (by age group):
Convergence retraction nystagmus Hydrocephalus (infant)
Spasm of convergence Pinealoma (10 years)
Spasm of accommodation Head injury (20 years)
Skew deviation AV malformation (30 years)
2. Investigation: MS (40 years)
MRI brainstem to exclude lesion on dorsal Vascular (50 years)
midbrain Degenerative (Wernickes) (60 years)
Exam tips:
Remember the seven classical signs and seven classical causes!
Clinical approach to pupils Please examine this patients pupils
Describe
On general inspection, there is ptosis/exodeviation.
Please look at the distant (fixation target).
I would like to examine this patients pupils first in the light and
then in the dark.
Greater anisocoria in light dilated pupil abnormalities (III CN, Holmes
Adie.)
Greater anisocoria in dark constricted pupil abnormalities (Horners.)
Perform light reflex
Direct reflex (consensual reflex)
RAPD
Decide quickly which scenario
1. RAPD
Check EOM (INO, other CN)
Ill like to check
Fundus (optic disc atrophy, retinal lesions)
VA, VF, color vision
2. Dilated pupil unreactive to light, anisocoria more pronounced in light
Ptosis/divergent squint
Check EOM
Watch for lid retraction (inverse Duanes syndrome or lid lag (pseudo-
von Graefes sign) from aberrant III CN regeneration)
(Continued )
B1037_Ch-06.indd 295 12/22/2010 7:01:43 PM

(Continued )
This patient has a complete III nerve palsy.
Ill like to check
Fundus (papilledema)
Examine patient neurologically for long tract signs
No ptosis/no divergent squint
Check EOM
Id like to examine this patient under slit lamp.
Irregularity of pupils/vermiform movement (Holmes Adie)
Posterior synechiae (posterior synechiae)
Rupture sphincter/iris damage (traumatic mydriasis)
This patient has tonic pupil.

Id like to
Examine fellow eye (Holmes Adie)
Check tendon reflexes
Perform pharmacological tests (0.1% pilocarpine)
Ask for history of trauma, eyedrop use (can also use 1%
pilocarpine to confirm)
3. Small pupil, both reactive to light, anisocoria more pronounced in the dark
Mild ptosis, inverse ptosis
Enophthalmos
Flushing, anhidrosis
Check EOM ptosis overcome by frontalis
This patient has Horners syndrome.
Id like to
Check IOP (hypotony)
Confirm diagnosis by performing pharmacological tests (cocaine,
hydroxyamphetamine)
Examine systematically and neurologically for:
Neck scars, neck mass (trauma, thyroid CA, lymph nodes)
Clubbing, hypothenar wasting, finger abduction weakness (Pancoast
tumor)
III, IV, V CN palsies (Cavernous sinus syndrome, Raeders syndrome)
VIII, IX, X CN palsies crossed hemiesthesia, cerebellar signs (lateral
medullary syndrome)
Ask for history of pain and headache (cluster headache, carotid artery
dissection)
4. Small pupil, unreactive to light
Light-near-dissociation
This patient has Argyll Robertsons pupil.
Ill like to
Ask for history of DM, HPT, sarcoidosis, syphilis
B1037_Ch-06.indd 296 12/22/2010 7:01:43 PM

TOPIC 8
OPTIC NEUROPATHIES
Q Opening Question No. 1: What are the Causes of Optic Neuropathy?

The causes of optic neuropathy can be divided into.
Optic Neuropathy
1. Congenital Arteritic (giant cell arteritis)
Hereditary optic neuropathy Nonarteritic (atherosclerotic)
2. Acquired Autoimmune diseases (systemic lupus)
Others (hypotension, hypovolemia)
Optic neuritis (retrobulbar, papillitis,
neuroretinitis): Compressive optic neuropathy (tumors)
Demyelinating Inltrative/granulomatous optic neuropathy
Postinfectious (sarcoidosis, lymphoma, leukemia)
Autoimmune diseases (systemic lupus) Traumatic optic neuropathy
Idiopathic Toxic optic neuropathy
Ischemic optic neuropathy (anterior ischemic Radiation optic neuropathy
optic neuropathy, posterior ischemic optic
neuropathy):
Exam tips:
Remember the causes of optic neuropathy as NIGHT TICS (Neuritis,
Ischemic, Granulomatous, Hereditary, Traumatic, Toxic, Irradiation and
Compressive).
But classify it as congenital vs acquired.
Q How do You Differentiate Optic Neuritis From Anterior Ischemic Optic Neuropathy
(AION) and Compressive Optic Neuropathy?
Compressive
Optic neuritis Non-arteritic AION Arteritic AION
optic neuropathy
Presentation
1. Age of onset 2040 4065 7080 Any age
2. Gender Female Male = Female Female Male = Female
3. Onset Acute and Dramatic sudden Dramatic Gradual
progressive onset sudden onset and progressive
4. Pain Yes No Yes No
(Continued )
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(Continued )
Compressive
optic neuropathy
5. Other features MS symptoms Diabetes, Amaurosis fugax Headache,
(e.g. Uhthoffs hypertension, Giant cell arteritis nausea
phenomenon) atherosclerosis symptoms (80%) and vomiting
risk factors Jaw claudication
Small, crowded Neck pain
disc CRP > 2.45 mg/dl
ESR 47 mm/h
Scalp
tenderness
Polymyalgia rheumatica
Malaise/
weight loss
Signs
6. VA 6/186/60 Severe loss (70%) HM NPL May be normal
Mild loss (30%) (50%)
7. Bilateral Rare in adults Unilateral Common Rare
involvement May occur in Second eye
children may be involved
May alternate later on
between left
and right eyes
8. Pupil RAPD RAPD RAPD Normal
9. Fundus Normal or Disc swelling Disc swelling Disc swelling
Papillitis (pink) (sectoral, pale) (chalky white (diffuse)
disc edema) Optociliary
Cilioretinal artery shunt
occlusion (meningioma,
optic nerve
glioma)
10. Color vision Dramatic loss, Loss proportional
disproportional to VA loss
to VA loss
Investigation
11. VF Diffuse (50%) Inferior nasal Enlarged
Central (10%) sectoral defect blind-spot
Inferior altitudinal
defect
12. Blood Normal ESR raised ESR markedly raised

C-reactive protein
markedly raised
(more sensitive
than ESR)
(Continued )
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(Continued)
Compressive
optic neuropathy
13. FFA Mild leakage Moderate Severe leakage Severe leakage
at disc leakage Filling defect
margins seen (decrease capillary
and
choroidal
perfusion)
14. VEP Latency Amplitude Amplitude Amplitude
increase decrease decrease decrease
(Myelination (axonal (Axonal (Axonal
abnormality) abnormality) abnormality) abnormality)
15. Other MRI Temporal artery
investigations biopsy
Prognosis 100% recover 30% recover Very poor Good if

75% to 6/9 30% deteriorate prognosis compressive
30% stay the same 65% blind in lesion removed
both eyes
within a few
weeks if left
untreated
Exam tips:
The three most important and common causes of optic neuropathy.
Remember that there are ve differentiating symptoms, ve differentiating
signs and ve differentiating investigations.
Read treatment of non-arteritic AION from Ischemic Optic Neuropathy
Decompression Trial Research Group (JAMA 1995;273:625632).
Q How would You Perform a Temporal Artery Biopsy?
Temporal artery biopsy is the gold standard test for making a diagnosis of temporal arteritis.
I would commence corticosteroid therapy prior to temporal artery biopsy if the biopsy cannot be
performed immediately.
I would perform temporal artery biopsy ideally within three days of commencing corticosteroid therapy,
and preferably not after one week of commencing steroids.
Procedure
1. Identify branch of supercial temporal artery by 4. Blunt dissection of space below fat but above
palpation or Doppler ultrasound supercial temporalis muscle fascia with small
2. Local anesthesia: Lignocaine 12% and adrenaline curved artery forceps (supercial temporal
3. Incise skin over the artery with a No. 15 scalpel artery runs within the fascia), avoiding injury to
until subcutaneous fat is seen the facial nerve
B1037_Ch-06.indd 299 12/22/2010 7:01:44 PM

5. Carefully dissect supercial temporal artery 10. Close skin with interrupted 6/0 or 7/0 prolene
from fascia with Wescott scissors Complications
6. Tie both ends of segment to be excised with 4/0 Bleeding
silk Damage to branches of the facial nerve
7. At least 3cm of artery is excised (because of skip Failure to identify artery (especially if non-
pulsatile)
lesions)
Scalp necrosis
8. Cauterize cut ends of artery
Stroke (rare)
9. Close subcutaneous tissue with 5/0 interrupted
vicryl suture
Q Opening Question No. 2: What are the Causes of Optic Atrophy?

Optic atrophy can be either unilateral or bilateral.
Unilateral optic atrophy Bilateral optic atrophy Evaluation

Congenital (not a common cause) Congenital
1. Hereditary optic neuropathy 1. Hereditary optic neuropathy Family history
Dominant (Kjer)
Recessive (Behr, DIDMOAD)
Mitochondrial Lebers optic
neuropathy
Acquired Acquired
1. Old ischemic optic neuropathy 1. Pituitary tumor CT scan
2. Compressive optic 2. Chronic papilledema Drug history, anemia
neuropathy/pituitary tumor (secondary OA) Serum vitamin levels
3. Inltrative optic neuropathy: 3. Toxic optic neuropathy ESR
Sarcoidosis 4. Consecutive ischemic optic History of MS, VDRL
Malignancies (lymphomas, neuropathy FTA
optic nerve tumors) 5. Consecutive optic neuritis History of nasopharyngeal
4. Old optic neuritis 6. Radiation optic neuropathy CA
5. Traumatic optic neuropathy 7. Chronic glaucoma IOP, VF
6. Radiation optic neuropathy
7. Chronic glaucoma
8. Toxic optic neuropathy:
TB drugs (ethambutol, isoniazid,
streptomycin),
Chloramphenicol, digitalis,
chloroquine
Toxins (lead, arsenic,
methanol)
Thiamine, vitamin B2, B6,
B12, niacin, folate deciency
Tobacco-alcohol toxicity
9. Others PRP, retinitis pigmentosa
B1037_Ch-06.indd 300 12/22/2010 7:01:44 PM

Clinical approach to optic atrophy

The most obvious abnormality is a pale optic disc.
Comment on
Sectoral pallor, altitudinal pallor, bow tie, cupping
Ill like to
Test for RAPD
Check EOM for other CN involvement
Examine the fellow eye
If unilateral, think of
Old optic neuritis (internuclear ophthalmoplegia, other CN palsies)
AION (vascular risk factors)
Compressive optic neuropathy (headache, nausea, vomiting, optociliary
shunt, Foster Kennedy syndrome)
Traumatic optic neuropathy (history of trauma)
Radiation (history of DXT)
If bilateral, think of
Pituitary tumors (bitemporal VF defect)
Consecutive optic neuritis
Toxic optic neuropathy (ethambutol and other drugs)
Hereditary optic neuropathy (Lebers optic neuropathy)
Exam tips:
Very similar answer to causes of optic neuropathy but this question requires a
more clinical approach.
Causes are listed slightly differently for unilateral vs bilateral optic atrophy.
The common ones are in bold.
Q Opening Question No. 3: What are the Causes of Optic Disc Swelling?
Optic disc swelling can be either unilateral or bilateral.
The causes are either congenital or acquired.
Unilateral disc swelling Bilateral disc swelling
Acquired Acquired
1. Optic neuritis 1. Papilledema
2. Ischemic optic neuropathy Space occupying lesion
3. Compressive optic neuropathy Idiopathic intracranial hypertension
4. Inltrative optic neuropathy Malignant HPT
5. Traumatic optic neuropathy 2. Pseudopapilledema
6. Toxic optic neuropathy Drusen
7. Radiation optic neuropathy Congenital optic disc anomaly
3. Consecutive ischemic optic neuropathy
(Continued )
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(Continued )
Unilateral disc swelling Bilateral disc swelling
PLUS 4. Consecutive optic neuritis

8. Ocular disease central retinal 5. Compressive optic neuropathy
vein occlusion, posterior uveitis, 6. Toxic optic neuropathy
posterior scleritis 7. Diabetic papillopathy (may be
9. Orbital disease pseudotumor, unilateral)
thyroid eye disease
PLUS
Congenital (not as common as acquired) 7. Ocular disease posterior uveitis,
1. Hereditary optic neuropathy posterior scleritis
8. Orbital disease pseudotumor,
thyroid eye disease
Congenital (not as common as acquired)

1. Hereditary optic neuropathy
Exam tips:
Unilateral disc swelling cause of optic neuropathy plus ocular and orbital
diseases.
B1037_Ch-06.indd 302 12/22/2010 7:01:45 PM

TOPIC 9
OPTIC NEURITIS AND

MULTIPLE SCLEROSIS
Q Opening Question No. 1: What is the Anatomy of the Optic Nerve?

Optic neuritis is the second cranial nerve.
It is divided into four segments.
Segment Length Diameter Blood supply

Intraocular 1 mm 1.5 1.75 mm Retinal arterioles from CRA
Optic disc Peripapillary choroidal vessels*
Prelaminar Circle of Zinn Haller*
Laminar Pial branches*
Postlaminar
Intraorbital 25 mm 3 mm Pial branches of CRA
Intracanicular 410 mm 3 mm Ophthalmic artery branches
10 mm Internal carotid artery and ophthalmic artery
Intracranial 7 mm
branches
*From posterior ciliary arteries
Exam tips:
A gift question and one of the favorite anatomy questions. Answer it well.
Q Opening Question No. 2: Tell me About Optic Neuritis.

Optic neuritis is an acute inammatory optic nerve disease.
Optic Neuritis
1. Classication Anatomical
Etiological: Sarcoidosis
Idiopathic/demyelination Retrobulbar neuritis:
Secondary to: Common variant in adults
Autoimmune diseases Frequently associated with multiple
Infectious diseases (e.g. syphilis, viral sclerosis

infection)
B1037_Ch-06.indd 303 12/22/2010 7:01:45 PM

Papillitis: Usually self-limiting and resolves within

Common variant and usually bilateral in
612 months
children
2. Clinical features and investigations (see page 248)
Neuroretinitis:
Associated with viral infection, cat scratch 3. Prognosis (see below)
disease, Lymes disease, syphilis 4. Treatment (see below)
Not associated with multiple sclerosis
Notes
Signs of optic nerve dysfunction:
Decrease in VA
RAPD
Dyschromatopsia (usually red-green)
Diminished light-brightness and contrast sensitivity
Visual eld defects
Q What is the Prognosis of a Patient with Optic Neuritis Diagnosed Two Days Ago?
Prognosis
1. Recovery White race
Onset within a few days, maximal impairment Family history of MS
Winter onset
within 12 weeks
Ocular:
Recovery begins within 23 weeks, maximal at
History of Uhthoffs phenomenon
6 months FFA leakage around disc margin
Almost 100% have some recovery Recurrence of optic neuritis
Full recovery in 75% Optic neuritis in fellow eye
2. Recurrence Systemic:
No recurrence in 75% History of nonspecic neurological
symptoms
3. Risk of MS
HLA DR2
MS develops in 75% of women (35% in men)
CSF oligoclonal bands
Risk factors: MRI periventricular lesions ( 3, each
Patient:
3 mm in size)
Age 2040
If MRI normal, risk of developing MS only
Female sex
16% in 5 years
Exam tips:
3R for recovery, recurrence and risk of MS.
3/4 rule for the chance of each outcome.
Risk factors for MS: 4 patient factors, 4 ocular factors, 4 systemic factors.
B1037_Ch-06.indd 304 12/22/2010 7:01:45 PM

Q What are the Main Findings of the Optic Neuritis Treatment Trial (ONTT)?
The ONTT is a multicenter trial to evaluate treatment of optic neuritis with steroids.
There were 457 patients enrolled.
The patients were randomized to three treatment regimes.
Treatment Regimes
1. IV steroids Oral steroids vs placebo: no difference
3 days IV methylprednisolone (1g/day) plus 2. Recurrence
11 days of oral prednisolone (1mg/kg/day) IV steroids vs placebo: No difference
2. Oral steroids Oral steroids vs placebo: Higher recurrence
14 days of oral prednisolone 3. Risk of MS
3. Placebo IV steroids vs placebo: Lower risk in rst two
14 days of oral placebo years but same after that
Results Oral steroids vs placebo: No difference
MRI important predictor of MS ( 3, each
1. Recovery
3 mm or more in size increases risk by
IV steroids vs placebo: Faster recovery, but nal
12 times)
VA same, although color vision, contrast
sensitivity and VF better
Exam tips:
An easy way to commit to memory the effects of each type of treatment on
the 3Rs of prognosis.
One of the few big trials you are expected to know well. N Engl J Med
1992; 58188, Surv Ophthalmol 1998;43:291, Arch Ophthalmol
1997;115:15451552.
Q What are the Main Findings of the Controlled High Risk Subjects Avonex Multiple Sclerosis
Prevention Study (CHAMPS)?
The CHAMPS is a prospective randomized study designed to determine whether the effect of early
treatment with interferon beta-1a (Avonex) delayed the risk of developing clinically denite multiple
sclerosis.
There were 383 patients enrolled, who had their rst demyelineating event (including optic neuritis,
myelitis, brainstem-cerebellar syndromes) and had at least two MRI white matter abnormalities characteristic
of MS.
The patients were randomized to two treatment regimes.
Treatment Regimes
Group 1: ONTT protocol of IV and oral Fewer new/enlarging lesions and fewer
steroids followed by weekly intramuscular 30g gadolinium enhancing lesions in interferon
interferon beta-1a beta-1a group
Group 2: ONTT protocol of IV and oral steroids
followed by weekly intramuscular injection of Trial terminated because of clear benet of treatment
placebo over placebo.
Benet of interferon beta-1a also shown in other trials
Results (including Rebif), and continues over a 10-year period
1. Interferon beta-1a reduces the three-year of observation (CHAMPIONS study)
likelihood of conversion to clinically denite MS
Side effects of interferon beta-1a:
2. MRI ndings:
Depression, uid-like symptoms, liver toxicity,
Reduction in volume of brain lesions in anaphylaxis
interferon beta-1a group
B1037_Ch-06.indd 305 12/22/2010 7:01:45 PM

Q Tell me about Multiple Sclerosis.
Multiple sclerosis is an idiopathic demyelination disorder of the CNS.

MS does not involve the peripheral nervous system.
The CNS lesions are separated in TIME and SPACE.
Diagnosis is therefore made when there are two or more different neurological events occurring at
different times.
Multiple Sclerosis
1. Systemic features Two-thirds will have INO in course of
Hemisphere: disease
Dementia Clinical features (see below)
Hemiparesis, dysphasia, hemianopia Horizontal gaze palsy
Brain stem: One-and-a-half syndrome
Dysarthria, dysphagia Ocular dysmetria
Nystagmus, ataxia, diplopia Dorsal midbrain syndrome
Skew deviation
Spinal cord:
Nystagmus
Motor
Sensory Isolated CN involvement
Bladder, bowel and sexual disturbances Paroxysmal eye movement disorders
Transient disturbances: Rare:
Lhermittes sign Intermediate uveitis
Uhthoffs phenomenon Retinal periphlebitis
Trigeminal neuralgia
3. Investigations
2. Ocular features Lumbar puncture:
Sensory (hemisphere lesions) Oligoclonal bands
Optic neuritis: Leukocytosis
One-third of MS will present with optic IgG > 15% total protein
neuritis VEP:
Two-thirds will have optic neuritis in Increase in latency typically exceeds
course of disease decrease in amplitude
Risk of MS with optic neuritis (see above)
MRI:
Posterior visual system lesions (VF defects) Periventricular and corpus callosum plaques
Motor (brainstem lesions) Acute lesions may enhance with gadolinium
Gaze abnormalities:
Internuclear ophthalmoplegia:
One-third of MS will present with INO
Q Tell me about Internuclear Ophthalmoplegia.
INO is motor abnormality caused by lesions in the medial longitudinal fasciculus (MLF).
1. Classical feature (triad)

Failure of adduction of ipsilateral eye Vestibuloocular reex (VOR) impaired (note:
(e.g. side of MLF lesion) VOR is not intact because lesion is not
Ataxic nystagmus of contralateral eye on supranuclear)
abduction Abnormal convergence (note: Cogans
Normal convergence (posterior INO) anterior INO, implies lesion extents to

midbrain convergence center)
2. Other clinical features Manifest exotropia (wall-eyed bilateral INO
Horizontal: or WEBINO)
Slowing of saccades in adducting eye
Vertical:
Horizontal nystagmus of adducting eye
Vertical nystagmus
Vertical pursuit impaired
B1037_Ch-06.indd 306 12/22/2010 7:01:46 PM

Vertical VOR impaired ENG (electronystagmogram):

Upgaze maintenance impaired Reduction of peak velocity of adduction
3. Investigations 4. Etiology
OKN drum: MS (40%)
Slowing of saccades in adducting eye (to CVA (40%)
elicit, rotate drum in direction of ipsilateral Others (tumor, trauma, infection)
MLF lesion)
Exam tips:
There are 10 clinical features of INO (not three).
Clinical approach to INO

The most obvious abnormality is a failure of adduction of the right eye.
With a horizontal nystagmus seen in the left eye.
Examine
Exclude one-and-a-half syndrome (failure of abduction of ipsilateral eye and
adduction of contralateral eye)
Vestibulo-ocular reflex/Dolls eye reflex (should be impaired)
Convergence (if impaired, implies Cogans anterior INO)
Vertical movements
Id like to
Examine pupils for RAPD (old optic neuritis inflamed optic nerve)
Examine fundus (optic atrophy)
Examine neurological system (signs of MS or stroke)
Ask for history of trauma
Exam tips:
An extremely common clinical exam case. Usually asked to examine the
ocular movements.
Remember to look at the adducting eye when testing for horizontal
movements!
Q What are the Supranuclear Disorders of Ocular Motility?
Supranuclear disorders of ocular motility are characterized by the absence of diplopia, and the presence
of normal vestibulo-ocular reexes.
1. Horizontal gaze palsies: 2. Vertical gaze palsies:
Parapontine reticular formation (PPRF) lesions: Parinaud dorsal midbrain syndrome
Ipsilateral horizontal gaze palsy Progressive supranuclear palsy (Steele-
Medial longitudinal (MLF) lesions: Internuclear Richardson-Olszewski syndrome)
ophthalmoplegia
PPRF and MLF lesions: One-and-a-half
syndrome
B1037_Ch-06.indd 307 12/22/2010 7:01:46 PM

TOPIC 10
VISUAL FIELD DEFECTS IN

NEUROOPHTHALMOLOGY
Q Opening Question: What are the Ocular Manifestations of a Meningioma Compressing the Left
Optic Tract?
Lesion in Left Side of the Visual Pathway

1. Left optic nerve OKN asymmetry (defect when drum rotates
Decrease in VA in left eye towards left)
Decrease in color vision in left eye Dominant parietal lobe (Gerstmann syndrome)
RAPD in left eye Aphasia/agraphia/acalculia
Left-right disorientation
Optic disc swelling or atrophy in left eye
Dense central/diffuse scotoma in left eye Non-dominant parietal lobe
Neglect/inattention
2. Optic chiasm Impaired constructional ability
Bitemporal hemianopia (classic) Dyscalculia
Dense central/diffuse scotoma (optic nerve)
5. Left temporal lobe
Incongruous homonymous hemianopia (optic
Incongruous right upper homonymous
tract)
hemianopia
Bitemporal central scotoma (macular bers)
Formed visual hallucination
Junctional scotoma (junction of optic nerve and
Auditory hallucinations
chiasma)
May be complicated by motor seizures
Other features of chiasmal syndrome
6. Left visual cortex
(see pituitary disorders, page 260)
Congruous right homonymous hemianopia
3. Left optic tract
OKN symmetry
Incongruous right homonymous quadrantanopia
Unformed visual hallucination
RAPD in right eye
Other features of visual cortex involvement
4. Left parietal lobe (see stroke, migraine and other vascular
Incongruous right lower homonymous disorders, page 266)
quadrantanopia
Pursuit defect to left
Hemiparesis or hemianesthesia in right side of
body
B1037_Ch-06.indd 309 12/22/2010 7:01:46 PM

Clinical approach to visual field examination

Please examine this patients visual field.
Common clinical syndromes
Bitemporal hemianopia
Homonymous hemianopia
Bitemporal or homonymous quadrantanopia
Altitudinal hemianopia
Preliminary instructions
I am going to test the area that you can see.
Please cover your left eye and look at my nose.
Is there any part of my face that is not clear?
How many fingers are there?
Now look at my face and do not move your eyes.
Quadrant testing
How many fingers are there?
Neglect testing
How many fingers are there altogether?
Central scotoma testing
Test fellow eye
This patient has bitemporal hemianopia, my clinical diagnosis is a pituitary
lesion.
Id like to
Check EOM (see-saw nystagmus, CN palsies)
Check fundus (bow-tie atrophy, papilledema)
Ask history of diplopia (nonparetic), metamorphopsia, visual hallucination
Look for features of hypersecretion from an adenoma
Growth hormone (acromegaly)
Prolactin (history of amenorrhea, galactorrhea, infertility in females or
impotence in males)
ACTH (Cushings syndrome)
Assess for etiology of pituitary lesion ask for history of
Trauma
Radiation
Shock, blood loss during pregnancy (pituitary apoplexy)
Adrenalectomy for Cushings (Nelsons syndrome)
Secondaries to pituitary, infiltrative lesions (TB, sarcoidosis)
Or
This patient has right homonymous hemianopia, my clinical diagnosis
is a left postchiasmal lesion.
Id like to
Check fundus (optic atrophy, papilledema)
Perform full Humphrey VF to assess congruity of lesion
Left optic tract
Incongruous right homonymous quadrantanopia
RAPD in right eye
(Continued )
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(Continued )
Left parietal lobe
Incongruous right lower homonymous quadrantanopia
Check EOM (failure of pursuit to left)
Check for right hemiparesis or hemianesthesia
Assess reading (alexia) and writing (agraphia)
OKN asymmetry (move drum towards left)
Left temporal lobe

Incongruous right upper homonymous hemianopia
Formed visual hallucination
Left occipital lobe

Congruous right homonymous hemianopia
Assess visual attention (inattention) and visual recognition (agnosia)
OKN symmetry
Unformed visual hallucination
Exam tips:
Alternate question can be What are the ocular manifestations of stroke
involving the right optic radiation?
Few important principles to remember
1. VF defect becomes more congruous as you move further back along the
visual pathway.
2. Visual hallucination is formed in temporal lobe lesions vs unformed for
cortex lesions.
3. OKN asymmetry indicates that a lesion has involved the parietal lobe
(usually a tumor) as compared with OKN symmetry in a pure cortex lesion
(usually a stroke) (page 240).
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TOPIC 11
PITUITARY AND
CHIASMAL DISORDERS
Q Opening Question: Tell me about the Pituitary Gland.

1. Anatomy
Situated in sella turcica of sphenoid bone Optic nerve features (RAPD, color vision,
Anterior lobe: etc)
Secretes ACTH, GH, Prolactin, FSH, LH and Optic nerve VF defect: Dense central/
TSH diffuse scotoma
Posterior lobe: Optic nerve and chiasmal junction:
Secretes ADH and oxytocin (actual Junctional scotoma (von Willebrands
hormones produced from hypothalamus) knee)
Relationship with chiasma: 2. Spectrum of pituitary disorders
Central: Tumors:
80%
Pituitary adenoma
Classic chiasmal syndrome features
Craniopharyngioma
(see below) Meningioma
Optic chiasmal VF defect: Bitemporal
Others:
hemianopia, involving the superior elds Chordoma
rst Nasopharyngeal CA
Prexed (chiasma is anterior to pituitary Rathkes pouch cyst
gland): Secondaries
10%
Vascular (aneurysm)
Optic tract features
Inammation (Tolosa-Hunt syndrome,
Optic tract VF defect: Incongruous
meningitis)
homonymous hemianopia
Macular involvement: Bitemporal central
Demyelination (MS)
scotoma Trauma, surgery and DXT
Postxed (chiasma is posterior to pituitary Non-neoplastic masses: Fibrous dysplasia,
gland): sphenoidal sinus mucoceles, arachnoid cysts
10%
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Q Tell me about the Pituitary Adenomas.

Pituitary adenomas are benign tumors of the pituitary gland.
They can be classied as either secreting or nonsecreting, which can be subdivided into chromophobes,
acidophils.
Or classied as either microadenoma or macroadenoma, dened as.
The clinical presentation is that a combination of local mass effects and systemic endocrine effects.
Classication Hormones Clinical features

Secreting (75%) Chromophobes Prolactin Infertility-amenorrhea-galactorrhea in
(50%) women (like a lactation state, except
you cannot get pregnant!)
Hypogonadism, impotence, sterility,
decreased libido, gynecomastia and
galactorrhea in men
Acidophils (20%) GH Acromegaly in adults (see below)
Gigantism in children
Basophils (5%) ACTH Cushings disease (causing Cushings
FSH and TSH syndrome)
FSH and TSH tumors are extremely rare
Nonsecreting (25%)
Microadenoma Less than 10 mm in Usually that of secreting adenomas
diameter Nonsecreting microadenomas are not
discovered!
Macroadenoma More than 10 mm in Mass effects
diameter Usually nonsecreting in nature
1. Localized mass effects Endocrine evaluation: Prolactin, FSH, TSH,

Chiasmal syndrome (see below) GH
Compression of other adjacent structure: Treatment:
Cavernous sinus (CN palsies) Factors to consider:
Pituitary gland (hypopituitarism) Age
Raised intracranial pressure (papilledema) Presenting problem (vision, mass effect,
2. Endocrine effects endocrine effect)

Size and stage of tumor
Hypersecretion
Surgery (transphenoidal, transethmoidal,
3. Management craniotomy)
Investigation: Bromocriptine for prolactinomas (increases
Skull XR (note: In practice, skull XR is not prolactin inhibition factor)/somatostatin for
very useful): GH tumors
Expansion or ballooning of fossa
Radiotherapy (complications include
Erosion of clinoid
radiation optic neuropathy and
Double oor sign (asymmetrical fossa
panhypopituitarism):
expansion) Gamma-knife stereotactic radiotherapy
CT scan/MRI
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Exam tips:
The clinical presentation is often a combination of either: Endocrine effects
(from secreting tumors) or mass effects (from macroadenomas).
Most ophthalmologists end up seeing macroadenomas with mass effects on
the chiasma.
To aid memory (not exactly accurate!), microadenomas = secreting
adenomas = endocrine effects; macroadenomas = nonsecreting
adenomas = mass effects.
Q What are the Ocular Manifestations of a Pituitary Adenoma Pressing on the Chiasma?
Clinical Features of the Chiasmal Syndrome
VF defects (depending on location of chiasma): Temporal pallor (papillomacular bundle)
Bitemporal hemianopia involving superior Bow tie atrophy
elds rst (classic VF defect) Dense optic atrophy
Incongruous homonymous hemianopia (optic Hemield slip (nonparetic diplopia)
tract) Postxation blindness
Bitemporal central scotoma (macular bers)
Visual hallucination
Dense central/diffuse scotoma (optic nerve)
See-saw nystagmus
Junctional scotoma (junction of optic nerve
and chiasma)
Optic atrophy (spectrum of changes):
Normal looking disc
Exam tips:
The chiasmal syndrome is an important syndrome and bitemporal
hemianopia is but one of ve different VF defects.
Q What are the Ocular Features of Acromegaly?
Acromegaly
Angioid streaks Optic atrophy, papilledema
Chiasma syndrome Muscle enlargement
Retinopathy (DM and HPT retinopathy)
Exam tips:
Remember the clinical features as ACROM.
Q What is Pituitary Apoplexy?
Pituitary Apoplexy
Infarction of pituitary gland Presents with hyperacute chiasmal
Tumor outgrows blood supply or tumor syndrome
compresses hypophyseal portal vessels Treatment: High dose steroids/surgery
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Q Tell me about Craniopharyngioma.
Craniopharyngioma is an intracranial tumor arising from remnants of Rathkes pouch.

Craniopharyngioma
1. Histological features Children often present with panhypopituitarism
Solid component with squamous epithelium secondary to hypothalamic dysfunction
and calcication (dwarsm, delayed sexual development,
Cystic component with greenish uid obesity), while adults often present with visual
2. Clinical presentation depends on growth of impairment and visual eld defects
tumor 3. Diagnosis
Superiorly into ventricles (most common, CT scan/MRI (suprasellar calcication in 70%)
hydrocephalus and raised intracranial pressure) 4. Treatment: Surgical excision +/ postoperative
Anteriorly to frontal lobe (dementia) radiotherapy
Anteroinferiorly to optic nerve and chiasma Recurrence common and requires lifelong
(chiasmal syndrome) follow-up
Posteroinferiorly to hypothalamus and pituitary
gland (diabetes insipidus and hypopituitarism)
Notes
Differential diagnoses of bitemporal visual eld defects:
Chiasmal lesions
Dermatochalasis of upper lids
Tilted discs
Optic nerve colobomas
Nasal retinitis pigmentosa
Nasal retinoschisis
Functional visual loss
Q What is the Empty Sellar Syndrome?
The empty sellar syndrome is a neurological condition in which the subarachnoid space extends into the
sella, remodeling the bone and enlarging the sella.
Empty Sellar Syndrome
1. Classication Decrease VA rare
Primary: Due to herniation of suprasellar contents (e.g.
Common, 25% of autopsies optic nerve) into sella or vascular
Transfer of CSF pressure through a compromise
congenitally large opening in diaphragm sella VF defects:
Risk factors: Multiparous women, elderly Binasal (classically)
atherosclerotic patients, idiopathic Bitemporal, altitudinal and generalized
intracranial hypertension constriction of VF possible
Secondary: Headache
Pituitary surgery Elevated prolactin levels
DXT
3. Diagnosis
Pituitary apoplexy (need to exclude
CT scan/MRI
concomitant pituitary adenoma)
VA usually normal
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TOPIC 12
PAPILLEDEMA and
INTRACRANIAL TUMORS
Q Opening Question: What are the Clinical Features of Papilledema?

Papilledema is bilateral optic nerve head swelling secondary to raised intracranial pressure. It can be
divided into four stages.
Clinical features are related to either vascular or mechanical changes of the optic disc.
Papilledema
Stage Vision Optic disc vascular changes Optic disc mechanical changes
Early VA normal Hyperemia of margins Blurring of margins (superior
Loss of spontaneous venous and inferior margin rst)
pulsation (absent in 20% Edema of peripapillary nerve
of normal individuals) ber layer
Indistinct disc margins
Established Transient visual Venous tortuosity and dilation Elevated disc
disturbances Peripapillary ame-shaped Obliteration of cup
VA normal or hemorrhage Retinal/choroidal folds
impaired Cotton wool spots
Enlarged Hard exudates
blind spot Obscuration of small vessels
traversing disc
Longstanding VA impaired Vascular changes resolves Champagne cork appearance

Constricted VF Optociliary shunts
Drusen-like crystalline deposits
Atrophic VA severely Secondary optic atrophy
impaired
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Q What are the Ocular Features of Idiopathic Intracranial Hypertension?
Idiopathic Intracranial Hypertension (IIH) is a neurological syndrome of raised intracranial pressure in

the absence of.
Idiopathic Intracranial Hypertension
1. Denition (important)
Raised intracranial pressure Empty sella (see page 261)
> 250 mm water (need lumbar Venous sinus thrombosis
puncture) lateral decubitus position Lumbar puncture
In absence of (triad): Metabolic/endocrine evaluation
Space occupying lesion (need CT scan) Perimetry
Hydrocephalus (need CT scan)
5. Prognosis
Abnormal CSF (need lumbar puncture)
Spontaneous remission (312 months)
2. Etiology May be remitting and relapsing or develop into
Associated with obesity chronic condition
Exclude: Recurrence (10%)
Sagittal sinus thrombosis (most important to
Visual loss risk factors:
exclude. Need MRV to diagnose) Vascular disease (HPT, DM, anemia)
Metabolic disorders (Cushings disease,
Raised IOP
Addisons, hypoparathyroidism) Recent weight gain
Vitamin A toxicity (see page 413) and lead
poisoning 6. Treatment
Drugs (steroids, nalidixic acid, amiodarone, Conservative: Lose weight, discontinue inciting
tetracycline) drug, treat systemic disorders
3. Clinical features Indications for treatment:
Severe symptoms (headache)
Headache (90%)
Visual loss
Visual loss:
High CSF pressure
Transient (70%) or persistent (30%)
Repeat lumbar puncture:
Variable (different time of day)
25% remit after rst lumbar puncture
Shifting (left and right eyes)
However, CSF usually replenishes within 12
Loss of contrast sensitivity
hours
VF loss (essentially like VF defects in
papilledema): Diuretics (acetazolamide):
Oral diamox 500 mg bid for 46 weeks
Enlargement of blind spot and constriction
Topiramate (anticonvulsant that also inhibits
of VF
Other VF defects (nasal defects, central
carbonic anhydrase)
If there is no response, consider
defects)
Tinnitis (60%) Steroids:
Dexamethasone 4 mg 6 hourly for 12 weeks
IIH has been called otitic hydrocephalus
If no response, consider
precisely because of this symptom
Others (photopsia, retrobulbar pain, diplopia) Optic nerve sheath decompression or
ventriculo/lumbo peritoneal shunt
4. Investigations
Stenting of transverse sinus
CT scan/MRI:
Normal looking ventricles
Small ventricles
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Q What are the Ocular Signs of a Meningioma? A Pinealoma?
The ocular features of a meningioma include general signs due to raised intracranial pressure.
And focal signs depending on where the meningioma is.
Ocular Signs of Intracranial Tumors
1. General (raised intracranial pressure) VI CN palsy (false localizing sign)
Symptoms: III CN palsy (uncal herniation)
Visual blurring (transient or persistent)
2. Focal
Diplopia
Supratentorial (mainly sensory)
Signs:
Suprasellar/sphenoidal ridge/midbrain (both
Papilledema and optic atrophy
sensory and motor)
Foster Kennedy syndrome
Triad of optic atrophy in one eye,
Infratentorial (mainly motor)
papilledema in other eye and anosmia
Exam tips:
The ocular features of ANY brain tumor can be divided into general signs due
to raised intracranial pressure (see papilledema above) and focal signs,
depending on location of tumor.
For neuroophthalmology and strokes (see page 255), for migraines and
other vascular disorders (see page 265).
Q What are the Principles in the Management of Brain Tumors?
Principles of Management
1. Histological diagnosis 3. Palliative excision for malignant tumors
Access via burr hole or craniotomy (e.g. glioblastoma)
Guidance via free hand or stereotactic 4. Adjunctive therapy (e.g. DXT, chemotherapy)
technique
2. Curative excision for benign tumors
(e.g. meningioma)
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TOPIC 13
STROKES, MIGRAINES &

OTHER VASCULAR
DISORDERS
Q Opening Question: What are the Ocular Manifestations of Strokes?

Strokes cause a variety of different ocular syndromes.
Depending on the arterial system and location of the stroke.
The ocular features can be either sensory or motor.
Vertebrobasilar System Stroke
1. Vertebral or basilar artery IX and X CN palsy (dysarthria and
Complete brainstem infarct: dysphagia)
CN involvement: Sympathetic involvement (Horners
III, IV, VI CN palsies (ophthalmoplegia) syndrome)
V CN palsy (loss of corneal reex) Cerebellar involvement (ataxia and other
VII CN palsy (lagophthalmos) cerebellar signs)
VIII CN palsy (nystagmus) 2. Posterior cerebral artery
PPRF, convergence center, MLF involvement: Lateral geniculate body (posterior choroidal
Conjugate gaze palsies
artery):
INO, WEBINO (see page 253)
Incongruous homonymous hemianopia
One-and-a-half syndrome (see page 254)
Homonymous sectoranopia (wedge-shaped)
Parinauds syndrome (see page 244)
Anterior visual cortex (see below):
Sympathetic involvement:
Congruous homonymous hemianopia with
Horners syndrome
macular sparing
Midbrain: Others
Crossed syndromes (Webers syndrome,
Benedikts syndrome and others) 3. Cerebellar arteries
Pons: Cerebellar involvement (see nystagmus, page 238)
Crossed syndromes (Millard Gubler and Carotid System Stroke
others)
1. Internal carotid artery
Medulla:
Amaurosis fugax (see below and page 201)
Lateral medullary syndrome (posterior inferior
Venous stasis retinopathy (page 202)
cerebellar artery):
V CN palsy and spinothalamic tract
Ischemic optic neuropathy (page 247)
involvement (crossed hemianesthesia) Central retinal artery occlusion (page 193)
VIII CN palsy (vertigo and nystagmus) Anterior segment ischemia (page 202)
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2. Anterior cerebral artery Frontal eye elds:

Hemialexia (page 283) Conjugate gaze palsy (saccade defect)
Parietal eye elds:

3. Anterior choroidal artery
Conjugate gaze palsy (pursuit defect)
Important branch of internal carotid artery
Posterior parietal regions:
Causes full blown CVA (hemiplegia, Alexia and agraphia (see page 283)
hemianesthesia) Lateral geniculate body:
Involves different parts of the visual pathway: Homonymous sectoranopia
Optic tract: Optic radiation:
Incongruous homonymous hemianopia Incongruous homonymous hemianopia
Lateral geniculate body (compare with Posterior visual cortex:
posterior choroidal artery involvement Congruous homonymous central eld
above): defect (macular VF defect)
Homonymous superior and inferior Balints syndrome:
sectoranopia Ocular apraxia (cannot move eyes on
Optic radiation: command but can move spontaneously!)
Incongruous homonymous hemianopia Visual inattention (eyes wander around)
4. Middle cerebral artery
Involves different parts of cortex and visual
pathway (anterior to posterior):
Exam tips:
Knowledge of the anatomy of the blood supply to the brain is important.
Alternate questions can be, What happens when the basilar artery is
blocked? or What are the ocular manifestations of middle cerebral artery
stroke?
Q What are the Ocular Signs in Visual Cortex Lesions?
The ocular manifestations of visual cortex lesions depend on the area and extent of the involvement.
The signs are either predominantly anterior cortex or posterior cortex.
And the ocular features can be either VF defects or various psychosomatic syndromes.
Visual Cortex
1. Visual eld defects
Congruous homonymous hemianopia with Superior (bilateral involvement of inferior
macular sparing (anterior cortex, posterior cortex)
cerebral artery) Checkerboard defect (homonymous anopia,
Congruous homonymous central eld superior defect in one eye, inferior defect in
defect/macular VF defect (posterior cortex, other)
middle cerebral artery) 2. Psychosomatic syndromes
Temporal crescent unilateral VF defect (most Cortical blindness (page 283)
anterior portion of visual cortex) Antons syndrome (cortical blindness plus
This is the only monocular VF defect in the denial of blinding)
visual pathway posterior to the chiasma! Riddochs phenomenon (can see moving targets
Others: but not stationary targets)
Bilateral homonymous hemianopia with Balints syndrome (ocular apraxia, visual
macular sparing (bilateral anterior cortex) inattention)
Bilateral homonymous altitudinal defect:
OKN asymmetry (see OKN, page 240)
Inferior (bilateral involvement of superior
Unformed visual hallucinations (see visual
cortex)
hallucinations, page 282)
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Exam tips:
An alternate question can be, What are the ocular features of a meningioma
impinging on the visual cortex?
Differentiate signs of the anterior visual cortex (supplied by posterior
cerebral artery) from those of the posterior visual cortex, where the macular
representation is localized (supplied by middle cerebral artery).
Note that macular area is supplied by the middle cerebral artery!
Q What is Amaurosis Fugax?
Amaurosis fugax is an ocular transient ischemic attack (TIA).

The etiology is usually.
Amaurosis Fugax
1. Most common ocular TIA (transient ischemic Carotid stenosis > 70% (eight-year benet)
attack) Carotid stenosis 51 to 69% (consider this
only if other risk factors are present)
2. Due to cholesterol/platelet emboli from carotid
Carotid artery stenting:
atheroma Carotid Revascularization Endarterectomy vs
3. Clinical notes Stenting Trial (CREST) (N Engl J Med;2010:
Visual loss: Epub):
Transient, lasting less than 10 minutes to Risk of stroke, MI or death similar between
2 hours (never more than 24 hours by TIA stenting and endarterectomy groups
denition) During periprocedural period, higher risk
Starts in the central VF, expanding outwards of stroke with stenting, and higher risk of
or altitudinal (curtain like effect) MI with endarterectomy
Contralateral hemiplegia (12.5%) European International Carotid Stenting Study
Carotid bruit (20%) (Lancet 2010;375:985997):
Stents had a higher rate of complications
4. Treatment and prognosis
compared to endarterectomy (stroke,
Carotid ultrasound myocardial infarction, death)
Carotid endarterectomy (NASCET results): Commonest cause of death: Cardiac causes
Carotid stenosis < 50% (no benet)
(not stroke!)
Exam tips:
This is an important differential diagnosis of sudden visual loss and an
extremely common problem referred to by ophthalmologists in emergency
room settings requiring decisions made on the spot (see page 201).
Read results of the North American Symptomatic Carotid Endarterectomy
Trial (NASCET) N Engl J Med 1998;339:141525.
Q Tell me about Migraines.
Migraine is a neurovascular disorder.

It can be classied into.
The ocular features can be either sensory or motor..
Migraine
1. Classication With aura:
Without aura: Classic migraine
Common migraine (= classic migraine Migraine equivalent
without the aura) Aura without headache
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Ophthalmoplegic migraine Gradual build up

Painful III, IV and V CN palsies
Starts in central VF, expanding outwards
Retinal migraine to peripheral VF
Photopsia
Photopsia
Basilar migraine Metamorphopsia
III, IV and V CN palsies
Inhibitory:
Fortication spectra
Blurring of vision
Cluster headache (variant of migraine) Amaurosis fugax
2. Ocular features of classic migraine Scotoma
Sensory Others:
Excitatory: Photophobia
Fortication spectra: Motor:

Zig-zag patterns III, IV and V CN palsies
Exam tips:
This is important because the migraine is one of the most common neurologi-
cal conditions and often presents with ocular symptoms rst.
Q Tell me about Carotid Cavernous Fistula.

Carotid cavernous stula (CCF) is an arteriovenous stula which connects the carotid artery and the
cavernous sinus.
Direct CCF Indirect CCF

Classication Barrows Type I (internal carotid artery to Type II (meningeal branches of internal
cavernous sinus) carotid artery to cavernous sinus)
Type III (meningeal branches of external
carotid artery to cavernous sinus)
Type IV (meningeal branches of both
internal and external carotid artery to
cavernous sinus)
Etiology Head trauma with base of skull fracture Congenital malformation (associated with
(young men) Ehlers-Danlos or pseudoexanthoma)
Spontaneous rupture from atherosclerosis Spontaneous rupture from atherosclerosis
(postmenopausal hypertensive women) (postmenopausal hypertensive women)
Clinical features Acute pulsatile proptosis (with thrill Slowly progressive proptosis (with thrill
and bruit) and bruit)
Severe EOM impairment (abolished by Mild EOM impairment (VI CN palsy)
carotid compression) Subtle anterior segment signs
Anterior segment: Dilated cockscrew episcleral vessels
Engorged corkscrew episcleral vessels Raised IOP
Glaucoma (from increased episcleral Increased ocular pulsation
venous pressure, orbital congestion,
secondary angle closure, neovascular
glaucoma from CRVO)
Anterior segment ischemia
(Continued )
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(Continued)
Direct CCF Indirect CCF
Posterior segment: Posterior segment:
CRVO Features of venous congestion
Optic nerve head swelling (ON
compression)
Blinding Glaucoma
complications Exposure keratopathy
Optic nerve compression
Ocular ischemia
Investigation CT scan (What are CT scan features?): Similar
Proptosis
Distended superior ophthalmic vein
Enlarged EOM
Bowing of cavernous sinus
Orbital Doppler
Dilated superior ophthalmic vein
Reversal of ow
Carotid angiogram
Indicated if surgery considered
Indications for Blinding complications
treatment Severe diplopia
Severe bruit
Treatment Most stula close spontaneously Most stula close spontaneously
Interventional radiology (embolization):
Balloon, glue, sphere
Complications (stroke in 5%, failure of
procedure common)
Surgery (progressive carotid artery ligation)
Exam tips:
Common differential diagnosis of unilateral proptosis (page 307).
Notes
Glaucoma in CCF:
Increased episcleral venous pressure ishemia secondary
open angle glaucoma
Anterior segment ischemia neovascular glaucoma
Congestion of uveal tissues angle closure glaucoma
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TOPIC 14
NEUROOPHTHALMIC
MANIFESTATIONS OF
CEREBRAL ANEURYSMS
Q Opening Question: Tell me about Cerebral Aneurysms.

Cerebral aneurysms are saccular or fusiform dilatations of intracranial arteries.
Cerebral Aneurysm
1. Prevalence and presentation Infraclinoid:
16% of population in autopsies, of which Motor: III, IV and VI CN involved:
Anterior (V1 CN involved)

2030% have multiple aneurysms
Middle (V1 and V2 CN involved)
Presentation:
Posterior (V1, V2 and V3 CN involved)
90% present acutely with subarachnoid
hemorrhage from ruptured aneurysm Middle cerebral artery (20%)
10% present with chronic mass effects: Posterior to Circle of Willis (10%)
Headache is most common symptom 4. Ocular features
The median time of signs due to mass Acute with subarachnoid hemorrhage:
effect to aneurysm rupture is 14 days Increased intracranial pressure (see page 262)
(therefore important to diagnose aneurysm Terson syndrome
early to prevent devastating subarachnoid Chronic with mass effects:
hemorrhage) Motor (70%):
2. Etiology Infraclinoid aneurysms
Primary/idiopathic Painful, incomplete, pupil-involved III CN
Hypertension palsy (60% of aneurysms will have III CN

Others (rare): involvement)
Multiple CN involvement (IV, VI and V,
Connective tissue disorders (Ehlers-Danlos
syndrome, polycystic kidneys) depending on location)
Bacterial/fungal aneurysm Sensory (20%):
Supraclinoid aneurysms
Traumatic
Chiasmal syndrome (see page 260)
3. Location
Mixed motor and sensory (10%):
Anterior to the Circle of Willis (70%): Cavernous sinus carotid artery aneurysms
Anterior cerebral artery, anterior
Triad of pulsatile proptosis (with bruit),
communicating artery, internal carotid artery at conjunctival injection and VI CN palsy
bifurcation and posterior communicating artery (see carotid cavernous stula, page 268)
Supraclinoid:
Sensory: Optic nerve, chiasma and tract
involved
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Exam tips:
Ophthalmologists have an important role in detecting cerebral aneurysms
because 70% are anterior to the Circle of Willis and are present with ocular
sensory and motor signs.
Remember the 70:20:10 rule, which describes the location of aneurysm and
the clinical features.
Q How would You Manage a Patient Suspected of Having a Cerebral Aneurysm?
Management of Cerebral Aneurysm

1. Investigations 2. Treatment
CT scan: Medical:
Sensitivity = 60% Control of BP
If subarachnoid hemorrhage has occurred, Antivasospasm
sensitivity increases to 90% Anticonvulsant
MRI: Antiedema (dexamethasone)
Sensitivity = 80% Surgical:
MR angiogram, sensitivity = 90% Clipping of aneurysm
Carotid angiogram: Proximal ligation of parent artery
Gold standard, sensitivity = 95% Interventional radiology: Embolization of
Need four vessel angiograms to see both aneurysm with coils
anterior and posterior circulations
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TOPIC 15
NEUROCUTANEOUS
SYNDROMES
Q Opening Question: What is Neurobromatosis?

Neurobromatosis (NFM) is one of the neurocutaneous syndromes. With systemic and ocular
features.
Classical Neurobromatosis
1. Hereditary pattern Ocular features:
AD (with incomplete penetrance and Prominent corneal nerves
expressitivity) Lisch nodules
Chromosome 17 mutation Ectropion uvea
Glaucoma 50% have unilateral UL
2. Skin features
neurobroma/facial hemiatrophy
Caf au lait spots Choroidal hamartomas
Neurobroma
5. Others
Plexiform neurobroma
Skeletal abnormalities:
Axillary freckles
Short stature
3. CNS features Scoliosis
Neural tumors in brain, spinal cord, CN and Macrocephaly
peripheral nerves (neurobroma, glioma, Facial hemiatrophy
schwannoma) Childhood malignancies
4. Ocular features Hypertension from pheochromocytoma
Orbital features: 6. Diagnostic criteria for neurobromatosis type I
Lid plexiform neurobroma and (NF-1) ( 2 of the following)
ptosis 6 cafe au lait spots
Nonaxial pulsatile proptosis (congenital
2 neurobroma or 1 plexiform neurobroma
defect of sphenoid bone with spheno-
Axillary/inguinal freckles
orbital-encephalocele)
Axial nonpulsatile proptosis (optic nerve
Optic nerve/chiasmal glioma
glioma) 2 lisch nodules
Nonaxial nonpulsatile proptosis (other orbital Osseous lesions e.g. sphenoid dysplasia
nerve tumors, e.g. neurilemmoma) First degree relative with NF-1
Exam tips:
Remember that ALL the syndromes have the three cardinal sites of involve-
ment: Skin, CNS and eye(s).
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Notes
What are the possible mechanisms?
Obstruction of outow by neurobroma
Angle abnormality
Angle closure from ciliary body neurobroma
Q What is the Difference between Neurobromatosis Type I and II?
Type II or Central NFM

Rarer Diagnostic criteria for neurobromatosis type II
Chromosomal 22 mutation (NF-2):
Less caf au lait spots Bilateral vestibular schwannomas or
First degree relative with NF-2 and either
Ocular features include:
Bilateral vestibular schwannomas (V, VI, VII,
unilateral vestibular schwannoma or any two
VIII palsies) of the following:
Meningioma
Posterior subcapsular cataracts
Glioma
Combined hamartomas of retina and RPE
Schwannoma
No risk of glaucoma, no Lisch nodules
Juvenile posterior subcapsular lenticular
opacities/juvenile cortical cataract
Clinical approach to neurofibromatosis

This patient has multiple nodules on his face and neck.
Look for
Lid neurofibroma and ptosis (plexiform neurofibroma)
Anterior segment:
Lisch nodules
Ectropion uvea
Proptosis:
Pulsatile, no bruit (spheno-orbital encephalocele)
Nonpulsatile (optic nerve glioma)
This patient has neurofibromatosis.

Id like to
Check IOP and gonioscopy (glaucoma)
Check pupils for RAPD (optic nerve glioma, meningioma) and then dilate
the pupils to.
Examine fundus (optic atrophy, papilledema, optociliary shunts, choroidal
hamartomas)
Examine systemically for other features of NFM:
Skin (caf au lait, axillary freckles, plexiform neurofibromas,
neurofibromas on other parts of body)
Skeletal (short stature, scoliosis, macrocephaly, hemiatrophy of face)
Neurologically (CNS tumors)
BP (HPT from pheochromocytoma)
Examine family members
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Q What is Tuberous Sclerosis?
Tuberous sclerosis is one of the neurocutaneous syndromes.

With systemic and ocular features.
The classical triad consists of: Mental handicap, epilepsy and adenoma sebaceum.
Tuberous Sclerosis
1. Hereditary pattern Hydrocephalus
AD Mental handicap
Chromosome 9 mutation 4. Ocular features
2. Skin features Retinal astrocytoma
Adenoma sebaceum Hypopigmented iris and fundal lesions
Ash leaf spots 5. Others
Shagreen patches Visceral hamartoma:
Caf au lait Kidneys
Skin tags Heart
Subungal areas
3. CNS features
Astrocytic hamartoma:
Epilepsy
Q What is the Sturge-Weber Syndrome?
Sturge-Weber syndrome is one of the neurocutaneous syndromes.

Sturge-Weber Syndrome
1. Hereditary pattern Epilepsy, hemiparesis and hemianopia
None (like Wyburn Mason syndrome) 4. Ocular features
2. Skin features Glaucoma:
Nevus ammeus/cavernous hemangioma/port 30% of patients
wine stain: Ipsilateral to side of facial angioma
First and second divisions of V CN Higher risk if upper lid involved
Hypertrophy of face Choroidal hemangioma:
40% of patients
3. CNS features
Ipsilateral to side of facial angioma
Angioma of meninges (Which layer is
Diffuse type of choroidal hemangioma (not
involved? Answer: Pial) and brain:
circumscribed type of choroidal
Ipsilateral to side of facial angioma
hemangioma)
Parietal and occipital lobe
Episcleral, iris and ciliary body hemangiomas
May calcify and show up in Skull XR as tram
track sign
Clinical approach to Sturge Weber syndrome

This patient has a port wine stain.
In the distribution of the 1st and 2nd divisions of the Trigeminal nerve.
Look for
Hypertrophy of face on side of hemangioma
UL involvement ptosis
Episcleral hemangioma
(Continued )
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(Continued )
Iris, ciliary body hemangioma (lens subluxation)

Trabeculectomy
Ill like to
Exclude glaucoma (30%), check IOP and look at optic disc
Perform gonioscopy (hemangioma, angle anomaly, increased episcleral
venous pressure)
Check fundus for diffuse choroidal hemangioma (40%)
Examine patient neurologically (hemangiomas of brain)
Exam tips:
Note that cavernous hemangioma (Sturg Weber syndrome) is not the same as
the benign capillary hemangioma (page 310).
Notes
What are the mechanisms of glaucoma?
Raised episcleral venous pressure
Angle abnormally
Ciliary body angioma
Q What is Ataxia Telangiectasia?
Ataxia telangiectasia is one of the neurocutaneous syndromes.

Ataxia Telangiectasia
1. Hereditary pattern 4. Ocular features
AR Conjunctival telangiectasia
Chromosome 11 mutation Oculomotor defects:
2. Skin features Nystagmus
Cutaneous telangiectasia Oculomotor apraxia
Strabismus
3. CNS features
Cerebellar ataxia
Mental handicap
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Q What is the Von Hippel-Lindau Syndrome?
Von Hippel-Lindau syndrome is one of the neurocutaneous syndromes.

Von Hippel-Lindau Syndrome
1. Hereditary pattern 4. Ocular features
AD Capillary hemangioma of retina
Chromosome 3 mutation Treatment: Laser photocoagulation (small
2. Skin features (not prominent) lesions), cryotherapy (peripheral lesions),

PDT, IVTA, intravitreal anti-VEGF
Caf au lait
Hypertensive retinopathy (pheochromocytoma)
Melanocytic nevi
Papilledema (CNS hemangioblastoma)
3. CNS features
5. Others
Hemangioblastoma
Cerebellum
Visceral tumors:
Cysts of kidney, pancreas, liver, epididymis,
Brainstem
Spinal cord
ovary and lungs
Hypernephroma
May cause polycythemia
Pheochromocytoma
Q What is Incontinentia Pigmenti?
Incontinentia pigmenti is one of the neurocutaneous syndromes.

Incontinentia Pigmenti
1. Hereditary pattern 3. CNS features
Sex linked dominant (one of only few disease) Epilepsy
2. Skin features Mental retardation
Stage 1: Erythema and bullae at extremities Hydrocephalus
Stage 2: Wart-like changes 4. Ocular features
Stage 3: Hyperpigmented macules in christmas Proliferative retinopathy (like retinopathy of
tree pattern on trunk prematurity)
Notes
Ocular conditions with X-linked inheritance
X-linked dominant:
Incontinentia pigmenti
Alports syndrome
Aicardi syndrome
X-linked recessive:
Norries disease
Juvenile retinoschisis
Choroideremia
Ocular albinism
Lowe syndrome
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Q What is Wyburn Mason Syndrome?
Wyburn Mason syndrome is one of the neurocutaneous syndromes.

Wyburn Mason Syndrome

1. Hereditary pattern Hemiparesis
None (like Sturg Weber syndrome) Mental retardation
2. Skin features (not prominent) 4. Ocular features
3. CNS features Racemose angioma
Arteriovenous malformation in CNS:
Epilepsy
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TOPIC 16
HEAD INJURY
Q Opening Question: What are the Ocular Signs in Head Injury?

Ocular signs are important in head injuries because they have immediate localizing and prognostic
values.
Ocular Signs of Head Injury
1. Visual pathway signs: Traumatic III CN palsy
Retina and optic disc: Traumatic mydriasis
Orbital blow out fracture
Papilledema
Purtschers retinopathy Small pupil:
Horners syndrome
Optic nerve:
Traumatic miosis
Traumatic optic neuropathy (see below)
Pontine hemorrhage
Optic chiasma:
Hutchinsons pupil (early stages of
Infrequent, usually from frontal contusion
transtentorial herniation)
Retrochiasmal:
Homonymous hemianopia (secondary to 4. Late signs:
occipital ischemia) Subdural hematoma:
Late III CN palsy (transtentorial herniation)
2. Motor signs:
Late VI CN palsy (raised intracranial pressure)
III, IV and VI CN palsies, conjugate gaze palsies,
Aberrant III CN regeneration
INO:
Carotid cavernous stula
Difcult to diagnose
Observe spontaneous eye movements
Late Horners syndrome
Oculocephalic reex may help
3. Pupillary signs:
Fixed dilated pupil:
Transtentorial/uncal herniation (III CN palsy)
Q How do You Manage a Patient with Severe Head Injury?

The aim of management is to limit the extent of the primary damage and to prevent secondary brain
damage.
Management of Head Injury
1. Primary brain damage: Extradural:
Open laceration Lucid interval
Contusion Trauma to temporal bone area/pterion
Diffuse axonal injury area with rupture of middle meningeal

Brainstem injury artery
III CN palsy on ipsilateral side (herniation
2. Secondary brain damage:
of uncus on same side)
Hemorrhage
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Treatment: Cerebral edema:

Immediate clot evacuation, with good
IV mannitol
prognosis Hyperventilate to vasoconstrict cerebral
Subdural: vessels
Usually associated with diffuse cerebral
Intraventricular drain to monitor
damage intracranial pressure and drain
Bilateral III CN palsy
cerebrospinal uid simultaneously
Treatment:
Cerebral hypoxia:
Immediate clot evacuation, with poorer
Give oxygen
prognosis IV uids (improve BP)
Subarachnoid/intraventricular: Infection:
Treatment:
IV antibiotics
Conservative management
Epilepsy:
Intracerebral: Antiepileptics
Treatment:
Dependent on size, if large, may need to
evacuate
Q What are the Features of Traumatic Optic Neuropathy?
Traumatic optic neuropathy is an important complication of head injury.

It occurs in about 2% of all head injuries.
Traumatic Optic Neuropathy

1. Classication Follows regime from NASCIS (National
Mechanical compression from fracture fragment Acute Spinal Cord Injury Study) (JAMA
Indirect damage from edema/ischemia 1997;277:15971604)
IV 30 mg/kg bolus dose, followed by:
5.4 mg/kg for next 24 hours
Ipsilateral frontotemporal contusion
(injury < 3 hours)
Severe enough to have some loss of 5.4 mg/kg for next 48 hours (injury
consciousness 38 hours)
Instantaneous decrease in VA Oral steroids tapering dose for 15
Need to differentiate from ON avulsion, CRAO days

and ophthalmic artery occlusion Optic Nerve Trauma Study
Improve in one-third to half of cases (Ophthalmology 1999;106:12681277):

Failed to nd benet of either
3. Management
corticosteroids or optic canal surgery
Mechanical compression from fracture
But was a non-randomized study and
fragment:
there was no uniformity of adminis-
CT scan good for diagnosis (bony fragments)
tered corticosteroid treatments
Surgical decompression
Surgical decompression:
Indirect damage from edema/ischemia: If no improvement with steroids
MRI better for diagnosis
Optic nerve canal decompression via
Medical decompression is controversial:
transethmoidal approach (not the same as
High dose steroids:
optic nerve sheath decompression!)
No contraindication for steroids
No contraindication to surgery
(e.g. sepsis)
Exam tips:
A controversial area. Read latest results from National Acute Spinal Cord
Injury Study (NASCIS), JAMA 1997;277:1597604.
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TOPIC 17
COMA, DISORDERS OF
HIGHER FUNCTIONS &
PSYCHIATRIC DISEASES
Q Opening Question: What are the Ocular Features Seen in Patients in Coma?
The neuroophthalmic signs include the eyelids, pupil, fundus and ocular motility.
Neuroophthalmic Signs in Coma
1. Eyelid signs 3. Fundus
Eye opening: Papilledema (space-occupying lesion)
Glasgow Coma Scale Retinal hemorrhage (subarachnoid hemorrhage)
Spontaneous, to speech, to pain
4. Eye motility
Eye closure: Spontaneous eye movement:
Closure (intact lower pons)
Roving, bobbing, ping-pong movements
Tone of closure proportional to depth of coma
(brainstem damage)
Asymmetrical closure (VII CN palsy on one side)
Sustained conjugate eye deviation:
Blinking: Horizontal deviation (What are the possible
Spontaneous blinking (intact reticular system)
causes?)
Reex blinking:
Ipsilateral hemispheric lesion:
To light or threat (intact anterior visual
Oculocephalic reex/caloric stimulation
pathway, brainstem and VII CN) positive
To sound (intact VIII and VII CN)
Associated contralateral hemiparesis
To corneal reex (intact V and VII CN)
Contralateral pontine lesion:
2. Pupillary signs Oculocephalic reex/caloric stimulation
Fixed, dilated pupil: negative

Unilateral (III CN palsy with transtentorial Associated ipsilateral hemiparesis
herniation) Contralateral thalamic lesion (also known
Bilateral (atropine poisoning, barbiturate as the wrong way deviation)

poisoning, severe hypoxia-ischemia) Downward deviation:
Marcus Gunn pupil (optic nerve or chiasm Dorsal midbrain lesion
damage, pituitary apoplexy) Upward deviation:

Hypoxia-ischemia
Small pupil:
Unilateral (Horners syndrome) Sustained disconjugate eye deviation:
Bilateral (pontine hemorrhage, opiates III, IV, VI CN palsies, internuclear
poisoning, severe metabolic damage, ophthalmoplegia
thalamic and basal ganglia damage) Skew deviation (brainstem lesion)
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Q What are the Dolls Eye Reex and Caloric Tests?
The Dolls eye reex is a head rotation test for the oculocephalic reex.
Caloric stimulation is a similar test of the oculocephalic reex.
Oculocephalic Reex
1. Anatomical pathway Cold water in ear to one side (caloric test):
Afferent: Labyrinth VIII CN gaze centers in Conjugate movement of eye to same side
brainstem Nystagmus to opposite side (COWS)
Efferent: Medial longitudinal fasciculus III, IV, 3. Patient with coma and normal response
VI CN Metabolic coma
2. Normal response Barbiturate poisoning
Rotation of head to one side (Dolls eye test): 4. Patient with coma and abnormal response
Conjugate movement of eye to other side Indicates brainstem damage
Exam tips:
This is an uncommon question, but is still an important neurological test.
Remember the mnemonic COWS for cold water stimulation and nystagmus
response: Cold Opposite, Warm Same.
Q Tell me about the Psychiatric Conditions with Ocular Manifestations.
Psychiatric diseases are associated with a variety of different ocular manifestations.

Psychiatric Conditions with Ocular
Manifestations Images of humans, animals, owers
1. Visual symptoms in patients with psychiatric Usually colorful, well-dened, bright and rich
disorders in theme
Visual hallucinations from traditional Individuals emotional response: Surprise,
psychiatric diseases (e.g. schizophrenia) indifference, curiosity, but NOT fear
Palinopsia: 3. Psychiatric consequences of visual loss
Persistence of image after its removal In children, can lead to:
Causes: Developmental delay
Lesion in non-dominant parieto-occipital Associated hearing loss
lobe Grieving process
Drugs (cocaine abuse) In adults, can lead to:
Metabolic (hyperglycemia) Grieving process
Release hallucinations following visual loss: Depression
Crossed modality hallucinations (e.g. hear Personality change
a vision) Communication problems
Perceive various images in blind eld 4. Drug complication
Associated with various VF defects Ophthalmic complications of psychiatric
2. Charles Bonnet syndrome drugs:
Visual hallucination in patients with visual Chlorpromazine (cataract and retinal toxicity)
impairment Thioridazine (retinal toxicity)
First described in cataract-induced blindness Anticholinergics (acute angle closure
Variable onset glaucoma)
Lithium (nystagmus)
Duration episodic or continuous
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Psychiatric complications of ophthalmic drugs: Diamox (depression, decreased libido)

Beta-blockers (depression, fatigue, Drug interactions:
hallucinations) Beta-blockers and phenothiazines (increased
Topical anti-cholinergics (tachycardia, levels of both)
transient delirium) Effect of epinephrine prolonged in patients
with tricyclic-antidepressants
Q What is Visual Hallucination?
Visual hallucination is visual perception without retinal stimulus.

It can be divided into physiological and pathological, and unformed or formed.
Visual Hallucination
1. Physiological Epilepsy (occipital lobe)
Unformed hallucination: Optic neuritis
Entoptic phenomenon (phosphenes, lightning Retinal detachment
streaks of Moores) Formed hallucination:
Formed hallucination: Epilepsy (temporal lobe)
Hypnagogic (occurs when person is falling Drugs (barbiturate, LSD, levodopa)
asleep) Alcohol
Hypnopompic (occurs when person is Release hallucination:
waking up) Charles Bonnet syndrome
2. Pathological
Unformed hallucination:
Migraine
Q Tell me about Alexia.
Alexia is the inability to read.

Alexia
1. Classication Site of lesion: Left occipital lobe (i.e. pure
With agraphia: visual sensory lesion)
Inability to read or write Associated with right homonymous
Site of lesion: Left angular gyrus hemianopia
Associated with Gerstmann syndrome Hemialexia:
Without agraphia: Site of lesion: Splenium of corpus callosum
Able to write but unable to read what he/she 2. Etiology
has written! Stroke, tumor, trauma
Q Tell me about Metamorphopsia.
Metamorphosia is the distortion of shape or size of objects.

Metamorphopsia
1. Peripheral causes 2. Central causes (occipital and temporal lobe)
Macular edema and central serous retinopathy Migraine
Epiretinal membrane Epilepsy
Chiasmal syndrome (hemield slip) (page 260) Drug intoxication
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Q What is Cortical Blindness?
Cortical blindness is decreased vision secondary to bilateral retrogeniculate lesions.

Cortical Blindness
1. Clinical features 25% of patients with unilateral occipital CVA
Decreased VA may be mild to NPL develop contralateral CVA resulting in cortical
Decreased VA symmetrical in both eyes blindness within four years
Normal fundi, normal pupils 3. Etiology
Antons syndrome denial of blindness Vascular (stroke, severe hypotension, post
(page 267) angiography)
Various degrees of dementia, memory loss Infection (meningitis, encephalitis)
2. Location Demyelination (multiple sclerosis)
Bilateral retrogeniculate lesions Tumors
Unilateral retrogeniculate lesions do not lead to Trauma
cortical blindness
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TOPIC 18
OTHER
NEUROOPHTHALMIC
PROBLEMS
Q Opening Question: How do You Tell if a Blind Patient is Malingering/Has a Nonorganic Cause?
There are several clues to differentiate organic vs nonorganic blindness.
Nonorganic Blindness
1. Clues: 3. Differentiating from partial blindness:
Walks with normal gait Look for discrepancies in vision tests:
Wears sunglasses in darkened room Failing to improve linearly with increasing
Avoids looking at doctor when talking target size or decreasing target distance
Normal pupils and normal anterior and Improvement with lens of minimal optical
posterior segment examination power
Normal or incongruous results on testing
2. Differentiating from total blindness:
stereopsis, color vision, contrast sensitivity
Evoke lid/eye movements with visual stimuli
OKN response at maximum distance
(a blind person should have no movements):
Four prism diopter lens (conjugate movement in
Visual threat
OKN drum
direction of apex of prism)
Mirror test of Troosts (movement of eye with Refraction with fogging (for unilateral poor
rotation of mirror) vision)
Test proprioception (should be normal in a blind Visual eld: Tunnel vision (failure for the visual
person): eld to become wider with increasing distance
Index nger test (point your index ngers from the patient)
at each other)
Sign your name test
Visual evoked potential
B1037_Ch-06.indd 341 12/22/2010 7:01:52 PM

Q Congenital Optic Disc Abnormality
Clinical approach to optic disc coloboma

On examination of the optic disc, there is an inferonasal defect seen.
Otherwise the retina looks normal, there is no retinal detachment seen.
Look for
Obvious dysmorphic features (trisomy 13, 18)
Choanal atresia (CHARGE syndrome)
This patient has optic disc coloboma.
Id like to
Examine anterior segment for:
Post embryotoxon
Posterior lenticonus
Lens and iris coloboma
EOM for squint and nystagmus
Systemically for other features:
Cardiac
Neurologically
Clinical approach to optic disc drusen

On examination of the fundus, the most obvious abnormality is an optic disc
swelling.
However, the disc has a waxy, yellowish, lumpy appearance.
There is no optic cup.
The blood vessels are normal looking, not tortuous or dilated and elevated
from the disc.
There is no associated rim hemorrhages seen.
This appearance is consistent with a diagnosis of optic disc drusen.
Look for
Retinitis pigmentosa
Angioid streaks
Fellow eye (bilateral)
Obvious systemic features (neurofibromatosis, tuberous sclerosis,
pseudoxanthoma elasticum, Pagets disease)
Ill like to confirm my diagnosis with
B-scan under low gain (acoustically solid optic disc)
FFA (autofluorescence, no vessel leakage)
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Clinical approach to optic disc pit

On examination of the fundus, the most obvious abnormality is a round
defect at the temporal edge of the optic disc.
The disc margin is otherwise distinct and has a normal optic cup.
The blood vessels are normal looking.
This appearance is consistent with a diagnosis of optic disc pit.
Look for
Central serous retinopathy
Id like to perform a
VF to look for enlarged blind spot
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Section 7
Oculoplastic and
Orbital Diseases
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TOPIC 1
THE EYELIDS AND ORBIT

Q Opening Question No. 1: What is the Anatomy of the Eyelid?

The eyelid is divided into the upper lid and lower lid.
They each have anterior and posterior lamellas separated by the orbital septum.
Eyelid Anatomy
1. Anterior lamella Separates preaponeurotic fat pad from levator
Skin and lower lid retractors
Thinnest skin in body 3. Posterior lamella
No subcutaneous fat
Tarsal plate:
Orbicularis oculi: Fibrous skeleton of lids
Three portions: Meibomian glands are embedded within
Orbital
structure
Palpebral:
25 mm 10 mm in upper lid but only
Preseptal
25 mm 4 mm in lower lid (therefore upper
Pretarsal
lid tarsus can be used for lid grafts)
Lacrimal (Horners muscle)
Tarsal conjunctiva:
Nerve supply: VII CN (temporal and
Tightly adherent to tarsus
zygomatic branches)
2. Orbital septum
Extension of periosteum from orbital rim to
tarsus
Exam tips:
One of the more common basic science questions in examinations.
Q Opening Question No. 2: Tell me about the Levator Palpebrae Superioris.

The levator muscle is an important extraocular muscle in the superior orbit.
The main function is in raising the upper lid.
Levator Palpebrae Superioris
1. Anatomy Mullers muscle arises from posterior
4 cm long, ending 10 mm behind orbital layer of levator inferior to Whitnalls
septum to extend as an aponeurosis ligament and inserts on superior tarsal
Aponeurosis fuses with septum 4 mm above border
tarsus 2. Origin
Identied by pre-aponeurotic fat pad Lesser wing of sphenoid
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3. Insertion (four classical sites) Pretarsal orbicularis

Skin crease 4. Nerve supply
Medial and lateral palpebral ligaments III CN (upper division)
(including Whitnalls ligament)
Anterior surface of tarsal plate (lower 1/3, NOT
upper 1/3!)
Exam tips:
Probably the most important oculoplastic muscle.
Note the importance of the number 4.
Q What is the Physiology of the Blinking Reex?
There are three types of blinking.

Blinking
1. Voluntary blinking 3. Spontaneous/involuntary
Palpebral and orbital portion of orbicularis Absent until about 3 months of life
oculi No stimuli needed
2. Reex Rate: 12/min
Stimuli: Amplitude: 9.5 mm (slightly less than palpebral
Sensory stimuli aperture)
Optical stimuli Duration: 0.3 s (less than a second)
Palpebral portion of orbicularis oculi Palpebral portion of orbicularis oculi
Q Opening Question No. 3: Tell me about the Anatomy of the Orbit.

Anatomy of Orbit
1. Gross anatomy 2. Bony orbit
Pyramidal-shaped, with base anteriorly and Medial wall (lacrimal, maxilla, ethmoid, body of
apex posteriorly sphenoid)
30 ml volume Floor (maxilla, zygomatic, palatine)
Medial and lateral wall of orbit are 45 to each Lateral (zygomatic, greater wing of sphenoid)
other Roof (frontal, lesser wing of sphenoid)
Medial walls of the two orbits are parallel to
each other, while lateral walls are perpendicular
to each other
Orbital axis 22.5 to sagittal plane
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TOPIC 2
PTOSIS
Q Opening Question No. 1: What are the Types of Ptosis?

Ptosis is dened as an abnormally low position of the upper lid in respect to the globe.
It can be divided into congenital or acquired forms.
Classication of Ptosis
1. Congenital Muscular dystrophies
Levator maldevelopment (see below) Aponeurotic:
2. Acquired Senile ptosis
Post-surgery
Neurogenic:
Post-trauma
III CN palsy
Horners syndrome Mechanical:
Marcus Gunn Jaw Winking syndrome Lid mass
Myasthenia gravis Scarring
Myogenic:
Chronic progressive external
ophthalmoplegia (CPEO)
Exam tips:
See also ptosis in the neuro-ophthalmology section (page 233).
The causes of acquired ptosis are listed from proximal (nerves,
neuromuscular junction) to distal (muscles, lids).
Q Opening Question No. 2: Tell me about Congenital Ptosis.

Congenital ptosis is dened as an abnormally low position of the upper lid with respect to the globe.
Occurring at birth or soon after birth.
Classication of Congenital Ptosis
1. Primary Congenital brosis
Levator maldevelopment (see below) Chronic progressive external
2. Secondary ophthalmoplegia (CPEO)
Neurogenic: Aponeurotic:
Post-trauma (birth trauma from forceps
Marcus Gunn Jaw Winking
III CN misdirection
delivery)
Congenital Horners syndrome Mechanical:
Myasthenia gravis Lid mass
Scarring
Myogenic:
Blepharophimosis
B1037_Ch-07.indd 349 12/24/2010 2:44:17 PM

Exam tips:
The causes are classied exactly like that of acquired ptosis.
Q What are the Differences Between Congenital and Senile Ptosis?
Primary Congenital Ptosis vs Aponeurotic

Senile Ptosis
Congenital ptosis Senile ptosis

Age Young Old
Laterality Usually unilateral (75%) Usually bilateral but may be asymmetrical
Severity Severe Milder
Upper lid crease Absent High*
On downgaze Lid lag Ptosis is worse*
Levator function Poor Good*
Other signs Superior rectus weakness (10%) Thinning of eyelids*
Deep upper lid sulcus*
Treatment Brow suspension usually needed Aponeurosis repair
*Five cardinal features of aponeurotic ptosis
Q Tell me about Marcus Gunn Jaw Winking Syndrome.
The MG Jaw Winking syndrome is an uncommon cause of congenital ptosis.

Marcus Gunn Jaw Winking Syndrome
1. Synketic innervation of levator and pterygoid 4. Treatment depends on severity of ptosis vs degree
muscle by V CN of winking:
2. Movement of jaw (to opposite side) leads to For severe winking (synkinetic excursion
retraction or wink > 2 mm), consider complete extirpation of
levator (including transaction of medial and
3. Frequently associated with monocular elevation
lateral horns) plus brow suspension
decit (double levator palsy), anisometropia, For mild winking (synkinetic excursion < 2 mm),
amblyopia and superior rectus weakness mullerectomy and levator resection may be
sufcient
Q Tell me about the Blepharophimosis Syndrome.
Blepharophimosis is a rare cause of congenital ptosis.

Blepharophimosis Syndrome
1. AD inheritance Telecanthus (note: Dened as medial canthal
2. Dened as a narrowing of horizontal palpebral distance > half of interpupillary distance, due to
aperture (note: NOT vertical!) laxity of medical canthal tendons)
Epicanthus inversus
3. Five classical features:
Lateral ectropion of lower eyelid (note: One
Ptosis with poor levator function and absent lid
of only a few causes of lower lid ectropion)
crease (note: Just like other congenital ptosis)
Hypoplasia of nasal bridge and orbital rim
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Other features: Correct lid defects rst (telecanthus and

High arched palate epicanthus inversus) at age 34 years
Protruding ears Correct ptosis later (bilateral brow suspension)
Cardiac defects 6 months later
Infertility (mainly in females, therefore
Correct lateral ectropian in teens
transmitted by males only)
4. Treatment depends on severity of ptosis, other lid
problems and presence of amblyopia
Treat amblyopia
Clinical approach to congenital ptosis

This young boy has a right ptosis.
Look for
Visual axis blockage (potential for amblyopia)
Check levator function (determine type of operation)
Check EOM (aberrant III CN, SR weakness)
Jaw movement (Marcus Gunn Jaw Wink)
Bells Reflex (determine extent of correction)
Refraction (astigmatism?)
Iris color (congenital Horners syndrome)
Exam tips:
Do not forget to test for Jaw Winking!
Q How do You Manage a Patient with Ptosis?
The management of a patient with ptosis depends on.

They can be conservative (eyelid crutches) or surgical.
Management of Ptosis
1. Factors to consider: Ptosis mild (< 2 mm) Fasanella servat
Cause of ptosis (tarsomullerectomy)
Severity of ptosis Levator function moderate (4 to 10 mm):
Levator function Levator resection
2. Type of surgery: Levator function poor (< 4 mm):

Brow suspension
Levator function good (> 10 mm):
Ptosis severe (> 2 mm) aponeurotic repair
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Q What are Complications of Ptosis Surgery?
The common postoperative complications are corneal exposure and either over or undercorrection.
Other complications include.
Complications of Ptosis Surgery
1. Corneal exposure Lash eversion and ectropion
2. Over and undercorrection Conjunctival prolapse
3. Contour defects: Lateral droop and medial are Contralateral ptosis
Orbital hemorrhage (rare)
4. Less common complications:
Lash ptosis and entropion
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TOPIC 3
ENTROPIAN AND
ECTROPIAN
Q Opening Question No. 1: Tell me about Entropian.

Entropian is an inversion of the eyelid.
Classication of Entropian
1. Congenital Noninfectious, e.g. ocular cicatricial
Rare, associated with congenital epiblepharon pemphigoid
2. Acquired Stevens-Johnson syndrome, chemical injury,
Involutional chronic blepharitis

Cicatricial, e.g. trachoma: Acute spastic:
Spasm of orbicularis oculi (ocular irritation or
Infectious, e.g. trachoma
essential blepharospasm)
Notes
What are the pathogenic mechanisms? How do you test for them?
Five classic mechanisms:
Overriding of preseptal to pretarsal orbicularis oculi (test by closure of
eyelids)
Horizontal lid laxity (test by pulling lid away from globe and watching
lids snap back)
Weakness of lower lid retractors (test by downgaze to see position of
lower lid)
Tarsal plate atrophy (test by palpation of tarsal plate)
Atrophy of retrobulbar fat leading to relative enophthalmos
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Q How do You Manage a Patient with Entropian?
The management of a patient with entropian depends on

Cause of entropian
Severity of entropian
Length of cure required and
Specic pathogenic mechanisms.
They can be conservative or surgical.
Management of Entropian
1. Involutional entropian Electrolysis
Temporary cure required transverse lid Cryotherapy
everting sutures Lash excision
Long term cure required: Mild anterior lamellar repositioning with lid
No excess horizontal laxity Weis procedure split at gray line
(transverse lid split and everting sutures) Moderate anterior
Excess horizontal laxity Quickerts Severe rotation of terminal tarso-conjunctiva
procedure (Weis procedure plus horizontal and posterior lamellar graft or advancement
lid shortening)
3. Congenital entropian
Recurrence of entropian after Weis or
Hotz procedure (tarsal xation of pretarsal skin
Quickerts procedure Jones procedure
(plication of lower lid retractors) and orbicularis)
2. Cicatricial entropian 4. Acute spastic

Management of trichiasis/distichiasis: Conservative (taping of lids, eyelid everting
Epilation sutures, Botox injection)
Exam tips:
Need to know basic surgical steps for each entropian operation. Prepare your
own surgical notes!
Q Opening Question No. 2: Tell me about Ectropian.

Ectropian is an eversion of the eyelid.
Classication of Ectropian
1. Congenital Cicatricial
Rare condition, associated with Infectious
blepharophimosis or congenital ichthyosis Noninfectious (e.g. SJS)

Paralytic (see facial nerve palsy page 302)
2. Acquired
Involutional
Exam tips:
Very similar classication to entropian. Substitute acute spastic in entro-
pion for paralytic in ectropian.
B1037_Ch-07.indd 354 12/24/2010 2:44:18 PM

Notes
What are the mechanisms?
Weakness of pretarsal orbicularis oculi (test by closure of eyelids)
Horizontal lid laxity (test by pulling lid away from globe and watching
lid snap back)
Tarsal plate atrophy (test by palpation)
Laxity of medical and lateral canthal ligaments
Q How do You Manage a Patient with Ectropian?
The management of a patient with ectropian depends on

Cause of the ectropian
Extent of ectropian (medial or entire lid) and
Whether horizontal lid laxity is present or not.
Management of Ectropian
1. Involutional ectropian Excess skin Kuhnt-Szymanowski
Medial lid involvement only: procedure (Bicks procedure plus
No horizontal lid laxity medial blepharoplasty)
conjunctivoplasty (excision of a diamond of Moderate lateral tarsal strip procedure
tarso-conjunctiva) Severe wedge resection with lateral tarsal
Mild horizontal lid laxity Lazy-T strip
procedure (medial conjunctivoplasty plus full 2. Cicatricial ectropian
thickness lid excision) Mild Z-plasty
Severe horizontal lid laxity medial canthal
Severe Skin grafts or aps
tendon plication
3. Congenital ectropian
Entire lid:
Mild:
Skin grafts
No excess skin Bicks procedure 4. Paralytic
(horizontal lid shortening) See facial nerve palsy (page 302)
B1037_Ch-07.indd 355 12/24/2010 2:44:19 PM

TOPIC 4
LID TUMORS
Q Opening Question: Tell me about Eyelid Tumors.

Eyelid tumors can be classied into benign and malignant tumors.
Malignant eyelid tumors can be further classied into.
Classication of Malignant Tumors
1. Primary 2. Non-pigmented eyelid mass
Basal cell CA (BCC) Epithelial:
Squamous cell CA (SCC) Papilloma sessile, pedunculated
Sebaceous gland CA Keratoacanthoma central crater with
Malignant melanoma keratin plug

Actinic keratosis rough, dry scales
Kaposis sarcoma
Seborrheic keratosis greasy, brown, friable
2. Secondary BCC shiny
Lymphoma SCC crusting, erosions, ssures
Maxillary CA Subepithelial:
Others Solid:
Sebaceous gland CA
Clinical Approach to Classication
Meibomian cyst
1. Pigmented eyelid mass Cystic:
BCC Cyst of Moll
Nevus Cyst of Zeiss
Malignant melanoma Sebaceous cyst
Nevus of Ota
Q How do You Manage a Patient Who Presents with a 6-Month History of Slowly Growing Eyelid Lump?
Clinical Approach
1. History Pain
Demographics: Discharge, bleeding
Older age, white race Change in color
Risk factors: 2. Examination of tumor
Prior skin CA Size and shape
Excessive sun exposure Destruction of eyelid margin architecture
Previous radiation, burns, arsenic
Loss of cilia
treatment
Ulceration
Tumor characteristics:
Telangiectasia
Growth, change in size
B1037_Ch-07.indd 357 12/24/2010 2:44:19 PM

Loss of ne cutaneous wrinkles Proptosis

Palpable induration well beyond margin EOM
3. Other examinations CN
Punctal involvement Regional lymph nodes
Fixation to deep tissue and bone Systemic features
Clinical approach to lid mass

On inspection, there is a nodular, shiny mass at the lower lid.
Describe
Size Measuring about 1 cm in diameter
Color Pearly-white color
Margin With distinct margins
Ulceration There is a central ulcer with rolled borders
Areas of pigmentation There are patches of pigmentation
Telangiectasia, bleeding, crusting However, no telangiectasia, bleeding or
crusting can be seen
Eyelid margin architecture, loss of cilia, punctal involvement
This patient has a basal cell CA.
Id like to
Examine tumor under slit lamp
Check lymph nodes
Ask for duration and change in size of lid mass, history of occupational
sun exposure, prior DXT
Q Tell me about Basal Cell Carcinoma.
BCC is the most common human malignancy.

Classically, there are three types.
Basal Cell Carcinoma
1. Epidemiology Rodent ulcer
90% of BCC located in head and neck region Sclerosing:
90% of malignant eyelid tumors Multicentric involvement
Most common site: Lower eyelid, followed by Chronic blepharitis
medial canthus, upper eyelid and lateral 3. Histology
canthus (note: Sequence is like extraocular Nodular and ulcerative:
muscle involvement sequence in thyroid eye Nests and cords of proliferating epidermal
disease!) basal cells
Worst prognosis: Medial canthus (due to Palisade of nuclei at edge of tumor
inltration into lacrimal system) Cracking artifact (artifactious separation

between tumor and stroma)
2. Classication
Collagen deposition in dermis
Nodular:
Sclerosing
Shiny translucent nodule
Branching cords penetrate into dermis, like
Pearly white appearance
tentacles (indian le arrangement)
Ulcerative: Striking dermal brosis
Rolled border
Difcult to see edge of tumor
B1037_Ch-07.indd 358 12/24/2010 2:44:19 PM

Exam tips:
The most common human malignancy!
Q Tell me about Squamous Cell Carcinoma (SCC).
SCC is the second most common eyelid malignancy.

It can arise de novo or from a precancerous lesion.
Squamous Cell Carcinoma
1. Epidemiology 3. Histology
510% of eyelid malignancy Arise from prickle cell layer
Worse prognosis as compared with BCC Dysplastic cells
2. Classication Multinucleated giant cells
Arise de novo Well to moderate to poorly differentiated
Precancerous lesion: Intradermal keratin pearls (keyword)
Actinic keratosis
Bowens disease
Q Tell me about Sebaceous Cell CA.
Sebaceous cell CA is a rare malignancy of the eyelid.

There are two types.
Sebaceous Cell Carcinoma
1. Epidemiology (therefore looks like chronic blepharitis,
15% malignant eyelid tumors chronic conjunctivitis or superior limbic
Most common site: Upper eyelid keratitis!)
(Why? More meibomian glands in upper lid!) 3. Histology
Arise from: Cords and lobules of poorly differentiated
Meibomian glands inltrative sebaceous cells
Glands of Zeiss Cells have a foamy appearance (contain
Sebaceous glands of eyebrow and caruncle fat fresh histological specimen must be
Worst prognosis of three classical eyelid tumors. sent for special fat stains)
Mortality in 30% Types of growth:
2. Classication Lobular pattern
Nodular: Papillary pattern
Looks like chalazion Comedo-acinar pattern
Supercial spreading: Combined
Pagetoid spread within epithelium of

palpebral, forniceal and bulbar conjunctiva
Notes
What are the bad prognostic features?
Site: Upper lid involvement
Site: 10 mm or more
Duration: 6 months or more
Origin: Meibomian as compared with Zeiss
Type: Supercial spreading type (pagetoid spread)
Grade: Undifferentiated
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Q Tell me about Malignant Melanoma.
Malignant melanomas are rare eyelid malignancies.

There are three types.
Malignant Melanoma
1. Classication Breslow (thickness)
Nodular 3. Suspicious nevus (ABCDE)
Supercial spreading Asymmetry
Arising from lentigo maligna Borders irregular
2. Staging Color mottled
Clark (ve levels of invasion: Level 1: Epidermis, Diameter large
Level 5: Subcutaneous) Enlargement over time
Q What are the Principles in the Treatment of Malignant Eyelid Tumors?
The management of malignant eyelid tumors involves multiple modalities.

We can use surgery, radiotherapy, chemotherapy and cryotherapy.
Principles of Surgery
No. 1: Remove as much tumor as possible, preserving No. 5: If tumor is < 4mm from margin or xed to
as much normal tissue as possible tarsal plate, need full thickness lid excision
No. 2: Keep 3 mm margin of normal tissue No. 6: Reconstruct both anterior and posterior lamella
No. 3: Use either frozen section or Mohs technique separately (posterior lamella may not be
(serial frozen section during surgery) needed if less than half of lid involved)
No. 4: If tumor is > 4 mm from margin and not xed No. 7: Reconstruct either anterior or posterior lamella
to tarsal plate, can consider partial thickness with a graft and the other layer with a ap
excision of tumor with direct closure of (keep blood supply)
margins No. 8: Aim to provide stable eyelid margin and
smooth internal surface
Size of defect Upper lid Lower lid

Less than a third of eyelid margin Direct closure Direct closure
Consider lateral cantholysis Consider lateral cantholysis
A third to half of eyelid margin Tenzel semicircular ap Tenzel semicircular ap
More than half of eyelid margin Bridging grafts: Cutler-Beard Hughes procedure (posterior
procedure (full thickness lower lamellar tarsoconjunctival
lid advancement) ap form upper lid and
anterior lamellar skin graft)
Hewes procedure (lateral
tarsal transposition of tarso-
conjunctiva) combined with
anterior lamellar skin graft
If vertical extent of defect
> 5 mm, then use Mustarde
cheek rotation procedure
(anterior lamellar skin ap
with posterior lamellar
mucosal graft)
B1037_Ch-07.indd 360 12/24/2010 2:44:20 PM

Exam tips:
Keep basic principles in mind and remember one surgery approach.
Remember to give your own scenario, For example, if a patient has a small
lower lid tumor.
Q What are the Types of Lid Grafts Available?
Lid Grafts
1. Anterior lamellar skin graft Hard palate (keratinized epithelium bandage
Skin upper lid or lower lid of either the same or contact lens needed in early postoperative
fellow eye period and better suited to lower lid use)
Retroauricular skin (full thickness) Buccal mucosa
Supraclavicular skin Ear cartilage
Inner arm skin Perichondrium
2. Posterior lamellar mucosal graft
Tarsal plate + conjunctiva of upper lid or lower
lid of either the same or fellow eye
B1037_Ch-07.indd 361 12/24/2010 2:44:20 PM

TOPIC 5
FACIAL NERVE PALSY

Q Opening Question: Tell me about Facial Nerve Palsy.

Facial nerve palsy is a common neurological condition.
Classication of Facial Nerve Palsy
1. Upper motor neuron (supranuclear causes) Cerebellopontine angle tumors (acoustic

CVA (different CVA syndromes) neuroma, nasopharyngeal CA)
Tumors Others: Sarcoma, leukemia
2. Lower motor neuron (nuclear and infranuclear Trauma:

causes) Temporal bone fracture
Facial trauma
Idiopathic:
Bells palsy Vascular:
Pontine stroke
Infectious:
Herpes zoster (Ramsay Hunt syndrome) Metabolic:
Acute or chronic otitis media DM, uremia
Others: Syphilis, mumps, meningitis
Tumors:
Parotid gland tumors
Exam tips:
A common examination topic. Alternate questions may be, What is the
anatomy of the facial nerve? and Why are the upper facial muscles spared
in supranuclear facial nerve palsies?
Notes
What is Bells palsy?
Sir Charles Bell was the founder of Royal College of Surgeons of
Edinburgh and described the anatomy of the VII CN pathway
Most common cause of lower motor neuron VII CN palsy
Etiology: Controversial, either ischemia, viral infection or demyelination
Prognosis:
75% spontaneous full recovery
25% recovery incomplete with aberrant regeneration:
Crocodile tears: Tearing at mealtimes, due to synkinetic innervation
of submandibular and lacrimal gland

(Continued )
B1037_Ch-07.indd 363 12/24/2010 2:44:20 PM

(Continued )
Reverse jaw winking: Twitching mouth with attempted blinking,
due to synkinetic innervation of orbicularis and mouth muscles
Treatment:
Oral steroids initiated within the rst week associated with a better
outcome
May be combined with oral antivirals
Q How do You Manage a Patient with Facial Nerve Palsy?
The management of a patient with facial nerve palsy depends on.

Age of patient (younger patients tolerate exposure better)
Prognosis for recovery:
Cause of the palsy
Duration of treatment needed (e.g. Bells palsy will recover)
Risk of exposure:
Lagophthalmos
Bells phenomenon
Corneal sensation
Dry eye

Management of Facial Nerve Palsy
1. Temporary treatment required for acute corneal Lower lid paralytic ectropian
symptoms Brow ptosis
Conservative: Exposure:
Punctal occlusion
Articial tears and ointments
Medial canthoplasty
Taping of lids at night
Lateral tarsorrhaphy
External gold weights
Gold weights (implanted)
Botox
Mullerectomy/levator recession
Moist chamber goggles
Surgical: Ectropian present:
No MCT laxity: Medial canthoplasty
Tarsorrhaphy
MCT laxity: Medial wedge resection
2. Permanent treatment required: May be combined with lateral tarsal strip
Surgery aims to manage: Fascia lata sling for extreme cases
Exposure
Clinical approach to facial nerve palsy

This patient has a right facial asymmetry.
Look for
Facial nerve paralysis:
Brow ptosis
Loss of forehead wrinkle
Ectropian
Loss of nasolabial fold
Drooping of outer angle of mouth
Asymmetry of blink reflex
(Continued )
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(Continued )
Corneal exposure and tearing
Esodeviation (VI CN palsy)
Examine
Eye closure (lagophthalmos, Bells palsy)
EOM (VI CN)
Hearing (VIII CN)
Corneal sensation (V CN)
Check of cause of palsy (neck scars, parotid mass, vesicles on ears)
Ill like to
Check for hyperacusis, taste on anterior 2/3 of tongue
Check fundus for papilledema
Examine neurologically:
VII and contralat hemiparesis (Millard Gubler syndrome)
VII and gaze palsy, V, VIII, Horners syndrome (Fovilles syndrome)
VII, V, VIII, cerebellar signs (cerebellopontine angle tumor
nasopharyngeal CA)
Examine slit lamp for evidence of corneal exposure
B1037_Ch-07.indd 365 12/24/2010 2:44:21 PM

TOPIC 6
THYROID EYE DISEASE

Q Opening Question No. 1: What is Dysthyroid or Thyroid Eye Disease (TED)?

TED is a chronic inammatory disease of the eye which usually occurs.
In patients with systemic thyroid disease.
Commonly in middle aged women.
It is believed to be autoimmune in nature.
The systemic features include.
The ocular features include.
Q Opening Question No. 2: What are the Ocular Signs in Thyroid Eye Disease?
The eye signs of thyroid eye disease can be classied into.
Ocular Features of Thyroid Eye Disease
1. Extraocular Macular edema
Proptosis Optic nerve swelling
Lid signs: 3. Signs of disease activity (modied Mourits score)
Lid retraction, lid lag, lid swelling, lid Lid edema (01)
pigmentation Lid injection (01)
Restrictive myopathy Conjunctival chemosis (02)
2. Intraocular Conjunctival injection (02)
Anterior segment: Pain on eye movements (01)
Conjunctival injection and chemosis Retro-orbital pain at rest (01):
Superior limbic keratitis Total score of four or more is indication of
Dry eyes active disease
Exposure keratopathy Active disease needs treatment for control of
Episcleritis/scleritis inammatory process rst
Glaucoma
Posterior segment:
Choroidal folds
Exam tips:
This is a very common question, but always poorly answered! Many candi-
dates get stuck with describing in detail the different eyelid signs. You should
quickly cover the entire spectrum of manifestations before concentrating on
one aspect.
B1037_Ch-07.indd 367 12/24/2010 2:44:21 PM

Q What are the Blinding Complications of TED?
Blinding Complications of TED

3. Glaucoma (rare)
1. ON compression
2. Severe exposure keratopathy
Q What are the Prognostic Factors for TED?
1. Age of patient (older patients poorer prognosis)

2. Race (Asian patients have higher incidence of Pretibial myxedema
optic neuropathy) High autoantibody levels
3. Nature of disease onset (acute onset worse) 5. Smoking (important!)
4. Thyroid function:
Thyroid dysfunction (hyper or hypothyroid)
associated with poorer prognosis
Q What is the Pathology of TED?
Pathology of TED
1. Acute phase Proliferation of other tissues (fat, connective
Hypertrophy of extraocular muscles tissue, lacrimal gland)
(accumulation of glycosaminoglycans 2. Chronic phase
keyword in TED pathology) Fibrosis of muscles
Increase in inammatory cells Increase in chronic inammatory cells
Q How do You Manage a Patient with TED?
The management of TED involves a team approach, with the general systemic condition managed by the
physician.
The specic ocular management will depend on several factors.
Activity of disease
Nature and severity of ocular involvement
Stability of disease
Thyroid status and general health of patient
When surgery is indicated, the sequence of surgery is: 1) Orbital decompression, 2) strabismus surgery,
and nally 3) lid surgery.
In patients with.
Management of TED
Active disease: 1. Mild TED with lid and soft tissue involvement
Immunosuppression is the key only (80%)
NSAIDs rarely effective Conservative treatment:
High-dose pulsed intravenous steroids are the Tear replacement and lubricants for dry eyes
treatment of choice: and mild exposure keratopathy
Oral steroids less effective Sunglasses for photophobia
Methotrexate may be used for long-term Sleep with head elevated and diuretics for lid
maintenance/avoidance of side effects of swelling
Chemical sympathectomy (adrenergic
steroids
blocking agents, e.g. reserpine, propranol)
Orbital radiation may be used if steroids
Topical steroids for superior limbic keratitis
contraindicated
B1037_Ch-07.indd 368 12/24/2010 2:44:21 PM

Antiglaucoma medication for raised IOP 3. Severe TED with severe proptosis and ON
Monitor patient at regular intervals: compression (5%)
VA and clinical examination What are the indications for orbital
Visual elds
decompression?
Hess chart, binocular elds
ON compression
Lid surgery is performed only when restrictive Severe exposure keratopathy
myopathy and proptosis are corrected (key Severe proptosis with choroidal folds and
principle in the management of TED): macular edema
Mild lid retraction (< 2mm): Mullerectomy Cosmesis (less common)
Moderate retraction (24 mm): Levator Involves medical decompression, surgical
recession with LCT lengthening decompression or radiotherapy
Severe retraction (> 4mm): Lid spacer (hard
Types of surgical decompression:
palate, dermis-fat graft, auricular cartilage) Two wall:
Other lid pathology that may require surgery:
Floor and posterior portion of medial wall
Epiblepharon, fat prolapsed and Three wall:
dermatochalasis Two wall plus lateral wall
2. Moderate TED where restrictive myopathy Four wall:
predominates (15%) Three wall plus sphenoidal bone at apex
Conservative: Complications:
Correct with prisms Retrobulbar hemorrhage/soft tissue
Botox injections edema potential for ON compression
What are the indications for surgery? Strabismus, hypoglobus
Diplopia in primary gaze or downgaze Lid position changes
Stable myopathy for six months
No evidence of acute congestive TED
Type of surgery:
IR recession
Adjustable squint surgery
Exam tips:
Again, this is a very common question. You need to quickly cover all aspects
of the management before going into specic details.
Notes
What is chemical sympathectomy?
Indicated in several situations in the management of TED:
Temporary relief while waiting for spontaneous correction or surgical
intervention
Subacute lid retraction of less than six months duration
Diagnostic test to assess role of Mullers muscle in lid disease
B1037_Ch-07.indd 369 12/24/2010 2:44:21 PM

Q What are the Causes of Lid Retraction?
TED is the most common cause of lid retraction, but other causes include.
Causes of Lid Retraction
1. M causes 2. P causes
Myasthenia Gravis (contralateral ptosis, Cogans Parinauds syndrome (Colliers sign)
lid twitch) Parkinsons disease (progressive supranuclear
Myotonic (hyperkalemia, hypokalemia, palsy)
dystrophia myotonica) Ptosis of opposite eye
Marcus Gunn Jaw Winking syndrome Palsy (aberrant III CN regeneration)
Metabolic diseases (uremia, cirrhosis)
Exam tips:
Causes are M and P.
What are the Mechanisms of Lid Retraction in TED? What are the Types of Surgery
Q Available to Correct Lid Retraction?
Pathophysiology of lid retraction in TED Surgery to correct lid retraction

LPS recession
Contraction/brosis of levator palpebrae superioris (LPS) Blepharoplasty
Lateral tarsorrhaphy
Contraction/brosis of inferior rectus (IR) with secondary overaction of LPS IR recession
Sympathetic overaction with overstimulation of Mullers muscle Mullerectomy
Proptosis Orbital decompression
Q Why does Ptosis Sometimes Occur in TED?
Ptosis in TED
LPS aponeurosis dehiscence (aponeurotic CN III palsy (orbital apex compression)
ptosis) Pseudoptosis (proptosis in fellow eye)
Associated myasthenia gravis
Clinical approach to proptosis

Thyroid eye disease (TED)
Inspection (eight features to describe)
On inspection, this middle aged lady has.
Unilateral or bilateral proptosis

Axial in nature (use torchlight to look at light reflex, then look at proptosis
from behind patient)
(Continued )
B1037_Ch-07.indd 370 12/24/2010 2:44:22 PM

(Continued)
Attentive gaze (Kochers sign) and infrequent blinking (Stellwags sign)
Squint
Fullness of eyelids (Enoths sign)
Lid retraction (Dalrymples sign)
Chemosis or conjunctival injection
Goitre (ask patient to swallow)
Test lid and EOM
Please follow this target, move your eyes, do not move your head.
Test downgaze first to look for lid lag (Von Graefes sign)
Then test for upgaze to look for restriction in upgaze
Test all other EOM
Do not forget to test convergence (Mobius sign)
May also see lower lid lag on testing upgaze (Griffiths sign)
Close lids to look for lagophthalmos and Bells reflex
Test pupils
Palpation
Im going to gently touch your eyes, let me know if you feel any pain.
Orbital rim
Pulsation/thrill
Look for systemic features
Hands: Pulse, sweat, tremor, acropachy
Thyroid goitre
Pretibial myxedema
Id like to
Objectively measure the proptosis
Examine the anterior segment under slit lamp for: Chemosis, superior limbic
keratitis, exposure keratopathy, keratoconjunctivitis sicca
Check IOP in primary and upgaze position
Check fundus for: Disc pallor or swelling, choroidal folds
Test VA, color vision, VF, Hess test
Investigate systemic complications: Thyroid function test, etc.
Nonthyroid eye disease

Inspection
Unilateral or bilateral
Axial or nonaxial proptosis (use torchlight, then look from behind)
Fullness of eyelids laterally (lacrimal gland)
Conjunctival injection (pseudotumor, CCF), cockscrew vessels (CCF)
Test lid and EOM
EOM
Lagophthalmos
Pupils
Palpation
Lacrimal mass
(Continued )
B1037_Ch-07.indd 371 12/24/2010 2:44:22 PM

(Continued )
Orbital rim
Globe retropulsion
Pulsation/thrill
Others (ABC)
Auscultate for bruit (CCF)
Bend down (varix, lymphangioma)
Check lymph nodes
Id like to check
Fundus for: Disc pallor, optociliary shunts, choroidal folds
Exam tips:
One of the most common clinical ocular examinations [the others being:
Ptosis (page 233, pupils (page 245) and extraocular movements (page 223)].
The KEY is to make a quick decision as to whether the proptosis is related to
TED or nonthyroid eye disease.
Exam tips:
Common exam causes include: Carotid cavernous stula (CCF), cavernous
hemangioma and lacrimal gland tumors.
Q What is Graves Disease?
Graves Disease
1. Denition 4. Sequence of presentation
Graves disease is an autoimmune systemic 20% TED hyperthyroidism
disease and is the most common cause of 40% TED and hyperthyroidism present
hyperthyroidism simultaneously
2. Pathophysiology of Graves disease 40% hyperthyroidism TED
Lymphocytes TSH receptor antibody 5. Prevalence
(TRAB) bind to TSH receptor in thyroid 30% of patients with hyperthyroidism have TED
gland goitre and hyperthyroidism 6. Investigation
3. Clinical features Thyroxine levels
Three cardinal features: TRAB
Hyperthyroidism TSI (thyroid stimulating immunoglobulin):
Pretibial myxedema Correlates well with bioactivity of eye disease
TED
Q How do You Manage a Patient with Graves Disease?
Management of Graves Disease

1. Medical 312 weeks until patient is euthyroidic:
Carbimazole: Decremental regimen 15 mg every-
Blocks all T4 production day 10 mg every day maintenance

3040 mg everyday or twice a day
B1037_Ch-07.indd 372 12/24/2010 2:44:22 PM

Block and replace maintain at Large goitre

3040 mg everyday plus supplemental Prognosis:
thyroxine 60% become euthyroidic
Advantages: Tasteless, cheap, small risk of 30% become hypothyroidic
teratogenicity 10% will relapse
Side effects: Skin rashes, loss of hair, 3. Radioiodine (I131)
neutropenia Indications:
Propylthiouracil indications: Failed medical treatment or relapse
Allergy to carbimazole Allergic to medicine
Pregnancy Contraindication to surgery (elderly, cardiac
T3 thyrotoxicosis disease)
But bitter, expensive and not as efcacious
Prognosis:
Prognosis: Euthyroidic within three months
Treat for one year: 50% become hyperthyroidic after one year
50% relapse after one year
Incidence of hypothyroidism: 4% per year
70% still have abnormal TRAB
(therefore patient needs T4 replacement for
On treatment: life)
90% of lid retraction improvement Disadvantages:
30% of restrictive myopathy improvement Worsens TED (need prednisolone to control
Only rarely does proptosis improve inammation)
2. Surgical treatment Acute bout of thyroiditis with release of
Indications: T4 (in elderly, need to make sure patient is
Failed medical treatment or relapse euthyroidic rst)
Allergic to medicine Risk of gastric cancer
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TOPIC 7
PROPTOSIS & ORBITAL

TUMORS
Q Opening Question: How do You Differentiate a Capillary from Cavernous Hemangioma?
Capillary hemangioma Cavernous hemangioma

Pathology Vascular hamartoma. Abnormal growth Benign, encapsulated tumor consisting of
of blood vessels, with varying degrees of large, endothelial-lined channels, vascular
endothelial proliferation walls with smooth muscles and stroma
Demographics Infant Adult 2030 years
Females > males Usually woman
Most common benign orbital tumor in Most common benign orbital tumor in
childhood adults
Progressive slow growth in rst year of life Progressive slow growth throughout life
Spontaneous involution by age 57
Others Associated with dermal hemangioma and May be associated with Sturge-Weber
deep visceral capillary tumors (Kasabach- syndrome (page 274)
Merritt syndrome hemangioma, anemia
and thrombocytopenia, Maffucci syndrome)
Presentation Supercial hemangioma hemangioma Deep hemangioma presents with
conned to dermis, single or multiple axial proptosis from intraconal tumor
Deep hemangioma posterior to orbital Usually presents with hyperopia
septum, present with nonaxial proptosis that
increases in size with valsalva maneuver or
crying
Combined supercial and deep
Poor vision: Amblyopia from ptosis
(supercial) or astigmatism (deep)
CT scan Either intra- or extraconal mass Well-encapsulated intraconal tumor
Moderate to poorly dened margins No bony erosion
Enhances with IV contrast
Angiography Multiple feeder arteries and draining veins No feeding arteries or veins. Staining in
(therefore hemodynamically rapid) late arterial phase (low ow lesion)
(Continued )
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(Continued )
Capillary hemangioma Cavernous hemangioma
Management Indications for removal: Mainly observation in absence of
Systemic complications: symptoms
High output cardiac failure
Kasabach-Merritt syndrome
Ocular complications:
Amblyopia (from astigmatism,
anisometropia, strabismus and ptosis)
Proptosis (ON compression and exposure
keratopathy)
Tissue necrosis
Treatment options:
Systemic steroids
Intralesional steroids
Antibrinolytic agents (aminocaproate,
tranexamic acid) in Kasabach-Merritt
syndrome (2 Rs, 2 Ts)
Angiographic embolization
Radiotherapy
Surgical excision difcult
Q How do You Differentiate a Lymphangioma from an Orbital Varix?
Orbital lymphangioma Orbital varix

Pathology Isolated vascular choristoma Dilated venous outow channels, with
Various types of tortuous vessels well-dened endothelial lined channels
containing blood or clear uid containing blood
Non-encapsulated
Similar to cavernous hemangioma but
with more smooth muscle in vessel walls
Demographics Early childhood Late middle age
Presentation Supercial lymphangioma Deep varix intermittent proptosis and
transilluminable cystic lesion beneath pain with exertion. Increases in size with
skin of eye lid valsalva maneuver
Deep lymphangioma nonaxial Supercial varix swelling of lids and
proptosis that does not increase in conjunctiva
size with valsalva maneuver. Acute Combined supercial and deep
episodes of spontaneous hemorrhage
in tumor
Combined supercial and deep
CT scan Low-density cyst-like mass Abnormally dilated irregular veins
Enhancement of Mulitlobular lesions (hemorrhage)
Enlargement of bony orbit
(Continued)
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(Continued)
Orbital lymphangioma Orbital varix
Venography No arterial or venous connection Venous connection may be present
Management Surgical removal Surgical removal
Prognosis is guarded because lesion is Prognosis is also guarded because lesion
large, friable, inltrate normal orbital is friable and bleeds easily and excision
tissue, not encapsulated and bleeds easily may be incomplete
Exam tips:
An easy way to remember is to compare the features of lymphangioma with
those of capillary hemangioma (both occur in childhood and have similar
clinical). Then compare the features of varix with those of cavernous
hemangioma.
Q Tell me about Lacrimal Gland Tumors.
Lacrimal gland tumors are common causes of nonaxial proptosis.

Classied according to epithelial or nonepithelial origin.
Benign or malignant.
The clinical presentation is.
The pathological features include.
Classication, Clinical Features and Management
Type Frequency Tumor Clinical features Management

50% epithelial 50% benign Pleomorphic Older patients Excision
adenoma Chronic presentation biopsy
Painless (keyword)
Nonaxial proptosis Lateral
CT scan affect orbitotomy
usually orbital lobe Malignant
with pressure changes transformation
in bony orbit 10%
50% malignant 50% adenoid cystic Younger Incisional
carcinoma, of which More acute history biopsy
50% is basoloid Pain (perineural (keyword)
variant (worst spread keyword) Orbital
prognosis) CT scan usually exenteration
Other malignant affect orbital lobe with Mid facial
tumors (50%) include: destructive changes resection
Pleomorphic in bony orbit Radiotherapy
adenocarcinoma,
mucoepidermoid
CA, monomorphic
adenocarcinoma
(Continued)
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(Continued)
Type Frequency Tumor Clinical features Management
50% 50% benign Inammation Signs of orbital Antibiotics
nonepithelial (dacryoadenitis) inammatory disease Steroids
50% malignant Lymphoma Older Radiotherapy
Acute history Chemotherapy
Pain
Bilateral tumor
common
CT scan affect both
orbital and palpebral
lobe and molds to the
shape of globe
Exam tips:
Famous Rule of 50 (refers to new cases in tertiary oncology referral center).
In the general ophthalmology setting, closer to 80:20 nonepithelial:epithelial
ratio.
Q What is the Pathology of Pleomorphic Adenoma? Adenoid Cystic Carcinoma?
Pathology of Lacrimal Gland Tumors

1. Pleomorphic adenoma 2. Adenoid cystic carcinoma
Involves orbital lobe Involves orbital lobe
Mixture of epithelial tissues (nests/tubules in Classic type: Swiss cheese appearance
two layers) and stromal tissues (connective (keyword)
tissues, cartilage, bone, myxoid tissues) (hence Other varieties: Basaloid variant (worst
the term pleomorphic!) prognosis), comedo acinar, sclerosing, tubular
Pseudocapsule from compression of normal No capsule (invades surrounding tissue,
tissue by slow growing tumor including nerves hence perineural spread)
Q Tell me about Lymphoma Involving the Eye.
Lymphoma is a malignant lymphoproliferative disease which can affect the ocular structures in a number
of ways.
Orbital lymphoma is a spectrum of disease which can range from.
Lymphoma and the Eye
1. Orbital lymphoma 3. Posterior segment
2. Anterior segment Vitritis (masquerade syndrome)
Conjunctival lymphoma Subretinal inltrate
Cornea (crystalline keratopathy) Primary intraocular lymphoma
Uveitis (masquerade syndrome)
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Orbital Lymphoma
Types Histology Prognosis

Orbital inammatory disease Hypocellular lymphoid lesion Benign
(pseudotumor) Mature lymphocytes
Mixture of different cells (polyclonal
proliferation)
Fibrous stroma
Benign reactive lymphoid Hypercellular lesion 20% become malignant
hyperplasia Mature (T cell) lymphocytes
Reactive stroma
Patternless or follicular
Atypical lymphoid hyperplasia Hypercellular lesion 30% become malignant
Borderline maturity
Diffuse or follicular pattern
Malignant lymphoma Hypercellular lesion Malignant
Immature malignant (B cell) cells 50% associated with
Monomorphous (monoclonal proliferation) systemic lymphoma
Follicular (10%) or diffuse (90%) pattern (20% with preceding
Commonest type is MALT lymphoma history of lymphoma)
Q Tell me about Rhabdomyosarcoma.
Rhabdomyosarcoma is the most common primary malignant tumor of the orbit in children.
It is a tumor of connective tissues that has the capacity to differentiate towards muscle.
(Note: Does not arise from extraocular muscles!)
Rhabdomyosarcoma
1. Histology Not primarily found in orbit, usually invades
Embryonal: orbit from paranasal sinus
Most common Grape-like form
Undifferentiated connective tissues 2. Clinical presentation
Alveolar: First decade (78 years)
Most aggressive Rapid progressive proptosis
Fibrovascular strands oating freely in Severe inammatory reaction:
alveolar spaces Main differential diagnoses in children:
Pleomorphic: Chloroma, Ewings sarcoma, neuroblastoma,
Best prognosis but rarest orbital cellulitis
Most differentiated and pathologically looks
Nonaxial proptosis (mass in upper orbit)
like muscles
Usually in older individuals
3. Management
(Note: Very much like pleomorphic adenoma Radiotherapy
of lacrimal gland!) Chemotherapy (vincristine, dactinomycin,
Botyroid: cyclophosphamide)
Rare variant of embryonal Exenteration
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Exam tips:
Remember the four histological subtypes: Alveolar stands for aggressive,
while pleomorphic has the best prognosis and behaves like pleomorphic
adenoma of the lacrimal gland.
Q How do You Differentiate an Optic Nerve Glioma from Meningioma?
ON glioma ON meningioma
Gross pathology Fusiform enlargement of ON Tubular enlargement of ON
Expansile intraneural or invasive
perineural form (usually seen associated
with neurobromatosis)
Histopathology Growth pattern: Intrinsic or extrinsic Arises from meninges (arachnoid layer
Arises from glial tissue (astrocytes, meningo epithelial cells)
oligodendrocytes, ependymal cells) Plump cells arranged in whorl-like
Spindle-shaped cells pattern
Rosenthal bers (keyword) Psammoma bodies (keyword)
Microcystic degeneration Intranuclear cytoplasmic inclusions
Meninges show reactive hyperplasia, Dural invasion
dura is normal
Demographics Young girls (26 years) Late middle age women
Presentation Axial proptosis occurs early Axial proptosis occurs late
VA decrease occurs early VA decrease occurs late, may have gaze
EOM normal evoked amaurosis
Optociliary shunt uncommon EOM impaired
Associated with Type I Optociliary shunt (keyword) common
neurobromatosis in 2049% (keyword) Associated with neurobromatosis
uncommon
CT scan/MRI Fusiform enlargement (keyword) Tubular enlargement (keyword)
Isodense to bone Hyperdense to bone (calcication)
Enlarged ON canal Normal ON canal
Chiasmal involvement may be present Sphenoidal bone hyperostosis (keyword)
Mucinous degeneration ON sheath enhancement on MRI
No central lucency (optic nerve) (keyword)
Management Conservative treatment if VA good Conservative treatment if VA good
Surgical removal if VA poor and life Surgical removal if VA poor and life
threatening threatening
Radiotherapy Radiotherapy
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TOPIC 8
EPIPHORA
Q Opening Question: What are the Causes of Epiphora in Adults?

Epiphora can be divided into.
Functional classication Etiology

Hypersecretion (1) 1. Entropian, ectropian
2. Trichiasis
3. Keratoconjunctivitis sicca
4. Corneal/conjunctival diseases
Obstruction 1. Congenital atresia
Canalicular 2. Acquired
Complete (2) Involutional
Partial (3) Infection canaliculitis, dacryocystitis
Nasolacrimal duct (NLD) Trauma
Complete (4) Tumor
Partial (5)
Lacrimal pump failure (6) 1. Facial nerve palsy
Combination of ectropion, punctal eversion, exposure keratopathy
and pump failure
Q How do You Evaluate a Patient with Epiphora?
Functional Evaluation
1. History 3. Syringing/irrigation with saline
Onset Soft stop
Watery or mucoid discharge Diagnosis: Complete canaliculi block (2)
Medical history (sinus disease, previous trauma, Reex from upper canaliculi common
previous dacryocystitis) canaliculi block
Surgical or radiation history Reex from lower canaliculi lower
2. Slit lamp exam canaliculi block

Lid position (entropian, ectropian) Hard stop
Puncta (position, inammatory changes) If no saline enters nose/mucoid reux into
Tear lm puncta:
Cornea and conjunctiva Diagnosis: Complete NLD block (4)
Dye disappearance test If saline enters nose:

Possibilities: Hypersecretion (1), partial
obstruction (3) or (5), lacrimal pump (6)
Proceed with Jones primary dye tests
B1037_Ch-07.indd 381 12/24/2010 2:44:24 PM

4. Jones primary dye test (largely of historical value) 5. Jones secondary dye test
Positive (dye in nose): Positive (dye in nose after ushing):
Diagnosis: Hypersecretion (1) Diagnosis: Partial obstruction NLD (5)
Negative: Negative:
Possibilities: Partial obstruction (3) or (5), Diagnosis: Partial obstruction canaliculi (3),
lacrimal pump failure (6) lacrimal pump failure (6)
Proceed with Jones secondary dye test
Q How do You Perform a Dacryocystorhinostomy (DCR)?
DCR is a tear drainage surgical procedure to bypass an NLD obstruction.

Creating an anastomosis between the lacrimal sac and the middle meatus.
Through an articial bony ostium.
DCR
1. Indications Osteotomy created:
Persistent and symptomatic epiphora form NLD With Traquairs periosteal elevator at anterior
obstruction lacrimal crest
Chronic and recurrent dacryocystitis Osteotomy enlarged with Kerrison punch to
Mucocele create 10 mm hole nasal mucosa exposed

Bowman probe inserted into canaliculi to
Congenital NLD obstruction
lacrimal sac to assess if common canalicular
2. Preoperative preparation obstruction is present
Assess nose for deviated septum /polyp: Lacrimal sac incised:
Consider ENT referral and X-ray to assess
With #12 Bard Parker blade creating vertical
nasal septum incision on medial wall of sac
Consider dacryocystogram (to look for stone,
Anterior and posterior aps created with right
tumor and anatomy) angled scissors (Weibs scissor)
Assess for bleeding tendencies: Nasal mucosa incised:
Check BP and platelet levels
After injection with lignocaine and
Stop warfarin/aspirin
adrenaline (to decrease bleeding)
3. Intraoperative procedure Posterior ap sutured to posterior lacrimal sac
GA (hypotensive anesthesia or LA less ap with 6/0 vicryl
bleeding postoperatively): Anterior ap sutured
Consider reverse-Trendelenburg position Management of canalicular obstruction:
LA injection to skin (lignocaine, marcaine, Canalicular obstruction > 10 mm from
adrenaline, wydase) 15 min before punctum lacrimal trephination to clear
operation obstruction followed by intubation
Cocaine 4% nasal pack Canalicular obstruction < 10 mm from
Skin incised: punctum carunculectomy with insertion of
With #15 Bard Parker blade Jones (pyrex) tube
10 mm medial to medial canthus and Closure:
1015 mm long incision Reattach medial canthal tendon
Blunt dissection of orbicularis oculi by blunt Close skin with 7/0 silk
dissecting scissors down to periosteum Pack nose with antibiotic soaked gauze
Divide anterior limb of medial canthal Silicon tube insertion (Bodkin intubation)
tendon 4. Postoperative care
Periosteum incised: Monitor carefully for bleeding (from anterior
With Rollet periosteal elevator (or #11
ethmoidal artery)
Bard Parker) medial to anterior lacrimal crest
Tell patient not to blow nose
Periosteum and lacrimal sac reected with
Skin suture removed by fth day
lacrimal retractor to expose operation site at
lacrimal fossa Syringing at six months
Remove tube (if present) at six months
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Exam tips:
One of the commoner surgical procedures asked in exams. The denition of
the procedure is half the battle won.
Notes
What are the indications for intubation after DCR?
Associated canalicular obstruction
Repeat DCR
Sever bleeding during operation
Shrunken and scarred lacrimal sac found during operation
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TOPIC 9
ENUCLEATION,
EVISCERATION & OTHER
ORBITAL SURGERIES
Q Opening Question: What are the Indications for Enucleation?

Enucleation is the removal of the entire globe, including the sclera and cornea.
Indications
1. Malignant tumors (e.g. retinoblastoma, choroidal 4. Blind eye with opaque media in which CA cannot
melanoma) be ruled out
2. Painful blind eye (e.g. advanced glaucoma) 5. Deformed phthisiscal eye in which CA cannot be
3. Severe ocular trauma (prevention of sympathetic ruled out
ophthalmia)
Q How do You Perform an Enucleation?
Enucleation
1. GA (or LA), clean, drape and speculum Do not clamp/crush ON if enucleation
2. Inject subconjunctival LA (lignocaine with performed for retinoblastoma
Cut ON with right angled scissors placed above
adrenaline)
the forceps
360 peritomy
Separate conjunctiva from Tenons and Tenons 5. Pack socket with 2.5 mm wide ribbon gauze to
from sclera with blunt dissection scissors secure hemostasis
3. Identify MR with squint hook 6. Insert implant either within Tenons capsule or
Suture MR with double armed 6/0 vicryl 3 mm behind posterior Tenons
behind insertion, and hold suture with artery 7. Close anterior Tenons capsule layer with 4/0
forceps
vicryl
Divide MR 1 mm behind insertion
Suture rectus muscles to fornix
Suture MR insertion with 4/O silk (suture to
Close conjunctiva with 6/0 vicryl
hold the globe)
Place prosthesis conformer to maintain fornix
Repeat for IR, LR, SR, then IO and SO
4. Lift and abduct the globe to stretch ON
Engage ON with curved artery forceps, by
strumming ON
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Q What are the Indications for Evisceration?
Evisceration is the removal of the contents of the globe, leaving the sclera and ON.
Evisceration
1. Indication Technically simpler procedure
Endophthalmitis (less orbital contamination, less Better for endophthalmitis (reduces risk of
risk of intracranial spread) dissemination of infection)
2. Advantages over enucleation 3. Disadvantages
Less disruption of orbital anatomy Risk of sympathetic ophthalmia not decreased
Better motility for prosthesis Not indicated for tumors
Q How do You Perform an Evisceration?
Evisceration
1. GA (LA), clean, drape and speculum 4. Closure
2. Corneal incised Pack scleral shell with adrenaline-soaked ribbon
With Beaver blade from 3 to 9 oclock gauze for ve min
Hold cornea with Jayles forceps and cut off Wash with 100% alcohol (destroy residual
entire cornea with corneal scissors uveal tissue), followed by gentamicin
Retract sclera at 12-, 5- and 9-oclock with Pack with ribbon gauze again
Kilners hooks Apply pressure bandage for 2448 hours
3. Insert evisceration scoop between sclera and 5. Allow sclera to granulate (healing by secondary
uvea and scoop out intraocular contents intention)
Send contents for culture
Remove uveal remnants with cellulose sponge
Q Tell me about Orbital Implants.
Orbital implants are used to replace globe volume after enucleation or evisceration.
Orbital Implants
Ideal implant Anterior coat dissolves more slowly to
Replace volume allow conjunctival healing
Enhance motility Posterior coat dissolves more quickly for
Good cosmesis bio-integration
Easy to insert, stable and promote healing Size
Material 16 mm = 2 cm3 volume
Inert (non-integrating): 18 mm = 3 cm3 volume
Glass, silicon, plastic, methyl methacrylate Ball covered with donor sclera/autogenous
Bioreactive (integrating) fascia
Hydroxyapatite, porous polyethylene For attachment of recti muscles
Bio-eye: Hydroxyapatite coated with Implanted within Tenons capsule or behind
polyethylene with differential resorption post Tenons capsule
rates: May be drilled to create peg for ocular
Does not need wrap
prosthesis
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Q What are the Complications of Postenucleation Socket Syndrome (PESS)?
Postenucleation Socket Syndrome

1. Infection Volume deciency:
2. Contracture of fornices (conjunctival deciency) Secondary orbital implants
Dermis fat grafts
3. Implant-related
Prominent upper lid sulcus (small implant) Conjunctival deciency:
Buccal mucosal grafts
Exposure of implant
Autologous conjunctival grafts
Extrusion of implant
Giant papillary conjunctivitis Lid malpositions:
Correct residual malposition after correction
4. Lid problems
of volume and conjunctival deciencies
Anophthalmic ptosis
Anophthalmic ectropion
Lash margin entropion
Management of PESS
Directed at correcting underlying mechanisms in
following order:
Prosthesis modication:
Frequently able to correct most
deciencies least invasive
Q What is Exenteration?
Exenteration is the removal of the globe and parts of the orbit.

Exenteration
1. Types of exenteration 2. Indications
Subtotal: Destructive orbital malignancies
Periorbital tissue, eyelids and apex left behind Destructive intraocular tumors with extension
Total: into orbit
All intraorbital tissue removed Malignant lacrimal gland tumors
Extended: Orbital sarcomas
All intraorbital tissue plus adjacent bony Fulminating fungal infection
structures (wall and sinus) removed
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Section 8
Uveitis, Systemic
Diseases AND Tumors
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TOPIC
TO
O PI
P C 1
INTRODUCTION
TO UVEITIS
Q Opening Question: What is Uveitis?

Uveitis is an inammation of the uveal tract.
Inammation of the iris is referred to as iritis, the ciliary body referred to as cyclitis and the choroid
referred to as choroiditis.
It can be classied in a few ways.
Classication of Uveitis
1. Anatomical Noninfectious (e.g. known systemic
Anterior, intermediate, posterior and panuveitis associations, no known systemic associations)
2. Pathological Masquerade (e.g. neoplastic, nonneoplastic)
Granulomatous vs nongranulomatous uveitis Common causes of uveitis can be classied
based on the anatomical location of the uveitis.
Clinical
Infectious (e.g. bacterial, viral, fungal, parasitic)
Q What are the Common Causes of Uveitis?

Etiology of Uveitis (by Anatomical
Classication According to the International
Uveitis Study Group (IUSG))
1. Anterior uveitis (iritis, iridocyclitis and anterior 3. Posterior (focal, multifocal, or diffuse choroiditis,
cyclitis) chorioretinitis, retinochoroiditis, neurouveitis)
Idiopathic Infective
HLA-B27 related uveitis (e.g. anklyosing Viral e.g. Acute Retinal Necrosis (ARN) or
spondylitis, Reiters syndrome, inammatory Progressive Outer Retinal Necrosis (PORN)
bowel disease and psoriatic arthritis) Fungal
Parasitic e.g. toxoplasmosis, toxocara
Fuchs heterochromic iridocyclitis, Posner-
Schlossman syndrome Noninfective
Collagen vascular diseases e.g. SLE, PAN,
Viral e.g. herpetic
Wegeners
Juvenile rheumatoid arthritis
Retinochoroidopathies
2. Intermediate uveitis (pars planitis, posterior Masquerade
cyclitis, hyalitis and basal retinochoroiditis) 4. Panuveitis (diffuse)
Pars planitis See below
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Exam tips:
There are many ways to answer this question. Decide on one and remember it.
Q What are the Causes of Granulomatous Uveitis?
The causes of granulomatous uveitis can be divided into infective, noninfective and masquerade
syndromes of granulomatous uveitis.
Infective: Noninfective:
TB Vogt-Koyanagi-Harada syndrome (VKH)
Syphilis Sympathetic ophthalmia
Leprosy Sarcoidosis
Toxoplasmosis Phacoantigenic uveitis
Lyme disease Masquerade:
Brucellosis Lymphoma/leukaemias
Viral Ocular metastases
Exam tips:
This is one of the most important LISTS to remember in uveitis.
It is especially useful in the clinical examination. If you see mutton fat
keratic precipitates, think of this list!
Remember the causes in groups (TB, syphilis and leprosy) and (VKH and
sympathetic ophthalmia).
Q How do You Manage a Patient with Uveitis?
The management involves a comprehensive history, physical examination, appropriate investigations and
treatment.
Management of Uveitis
1. History Posterior uveitis:
Symptoms of uveitis (redness, pain, Cystoid macular edema
photophobia, blurring of vision, etc.) Choroiditis, retinitis, vasculitis
Optic neuritis
Systemic review
Examination Systemic examination (heart, skin, joints, etc.)
Ocular examination: 2. Investigations
Anterior uveitis: Blood (directed by clinical evaluation):
AC cells and are, presence of hypopyon FBC (eosinophilia for parasites), ESR
(severity), brin VDRL, FTA
Keratic precipitates (small, medium size, Autoimmune markers (ANA, RF, anti-double
mutton fat) stranded DNA, ENA, HLA B27)
Posterior synechiae and peripheral anterior Calcium, serum ACE levels (sarcoidosis)
synechiae, iris nodules Toxoplasma serology and other TORCH
Complications (cataract, glaucoma, band screen (toxoplasma, rubella, CMV, hepatitis
keratopathy, phthsis bulbi) B, HIV)
Intermediate uveitis: Urine:
Snowakes and snowbanks 24-hour urine calcium (sarcoidosis)
Vitritis Culture (Bechets disease, Reiters syndrome)
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Radiological: Able to distinguish BCG immunization

CXR (TB, sarcoidosis, histoplasmosis), CT from TB infection
chest Cannot distinguish recent/active and past
Spine and sacroiliac joints X-ray (ankylosing infection

spondylitis) Pathergy test for Behcets disease:
X-ray of other joints (rheumatoid arthritis, Increased dermal sensitivity to needle trauma
juvenile rheumatoid arthritis) (increase leukotactic response)
Skull X-ray for cerebral calcications Only 10% of Behcets patients respond
(toxoplasmosis) Intradermal needle puncture
Skin tests: Look for pustule 2436 hours later
Mantoux tests Kveim test for sarcoidosis:
Intradermal injection of tuberculin puried Similar to Mantoux test
protein derivative Intradermal injection of sarcoid tissue (from
Inject ve tuberculin units in 0.1 ml to spleen of another patient with sarcoidosis)
produce a wheal of 610 mm size on Look for sarcoid granuloma four weeks later
forearm 3. Medications
Look for induration 4872 hours later
Mydriatic and cycloplegic agents
Positive if induration > 10 mm, indicates
Immunodulating:
previous infection with TB or
Corticosteroids (topical, periocular, systemic,
immunization for TB
intravitreal)
Anergy: May indicate immunosuppression
NSAIDS
(AIDS) or sarcoidosis
Immunosuppressives (e.g. cyclosporin,
TB quantiferon:
methotrexate, azathioprine)
In-vitro, quantitative Mantoux test
Useful in areas with high levels of TB

Newer medications (biologic agents):
Etanercept, iniximab, IVIG, interferon
endemicity
Measures IFN production in response to
alfa-2b
Mantoux antigens in sample of patients
blood
Exam tips:
The skin tests for sarcoidosis and Behcets disease are almost never used, but
frequently asked in the examinations!
Notes
When do you need to investigate for a specic cause?
Suggestive systemic features
Recurrent uveitis
Bilateral uveitis
Severe uveitis
Posterior uveitis
Young age of onset
B1037_Ch-08.indd 393 12/24/2010 2:46:57 PM

Q What are the Possible Causes of Panuveitis?
Panuveitis can be divided into granulomatous or nongranulomatous conditions.

They can be either infective or noninfective in origin.
Panuveitis/Posterior Uveitis
1. Granulomatous Noninfective:
Infective: Behcets disease (severe hypopyon, no
TB, syphilis, leprosy and others surgery, mention other features)
Noninfective: Candida (immunosuppression)
Sympathetic ophthalmia (previous trauma or Toxoplasmosis
surgery in other eye) and VKH Lymphoma
Sarcoidosis and others Masquerade
Masquerade
2. Nongranulomatous
Infective:
Endophthalmitis (severe hypopyon, previous
surgery)
Exam tips:
The causes of panuveitis, posterior uveitis and vasculitis are nearly IDENTICAL.
The granulomatous vs nongranulomatous classication list is very handy
here.
Q Tell me about Phthsis Bulbi.
There are three overlapping stages in phthsis bulbi.

Phthsis Bulbi
Atrophic bulbi without shrinkage: Globe becomes smaller and square-shaped
Initially size and shape of globe maintained (maintained by four recti muscle)
Continuous loss of nutritional support Atrophic bulbi with disorganization (phthsis
Lens becomes cataractous bulbi):
Serous detachment and atrophy of retina Size of globe decreases from 2426 mm to
Anterior and posterior synechiae formation, 1619 mm
leading to an increase in IOP Disorganization of ocular contents
Atrophic bulbi with shrinkage: Dystrophic calcication of Bowmans layer,
Ciliary body dysfunction leads to drop in IOP lens and retina
AC collapse with corneal edema, pannus and Sclera thickens
vascularization Bone replaces uveal tract
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TOPIC 2
SYSTEMIC INFECTIOUS
DISEASES AND THE EYE I
Q Opening Question: What are the Ocular Complications of AIDS?

Ocular complications develop in 75% of patients with AIDS.
Ocular Complications of AIDS
1. AIDS: Disease related manifestations Parasitic
Posterior: Microangiopathy Toxoplasmosis (relatively uncommon)
70% of patients with AIDS Pneumocystis carinii choroiditis
Microaneurysms, hemorrhages, cotton wool Fungi
spots Cryptococcal choroiditis:
Lesions are: Presents with optic neuritis and meningitis
Transient Candida retinitis

Smaller and multifocal Bacteria:
Located in the posterior pole TB
Anterior Syphilis
Ocular adnexa: 3. Neoplasia
Molluscum contagiosum Kaposis sarcoma of eyelid, conjunctiva and orbit
Neuroophthalmic: Lymphoma
Optic neuritis and optic atrophy
Squamous cell CA
CN palsies
Cortical blindness
4. Treatment related
Cidofovir, Rifabutin may cause uveitis
2. Opportunistic infections
Viral 5. Immune reconstitution uveitis
CMV retinitis (see below) Mediated by recovery of immune responses
Herpes zoster: specic to residual cytomegalovirus antigen
Progressive outer retinal necrosis (PORN) located in the eye
On commencement of HAART therapy
Exam tips:
Remember AIDS complications as Angiopathy, Infections Diseases and
Sarcomas.
Remember the BIG 8 opportunistic infections.
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Notes
How do you differentiate AIDS microangiopathy from CMV retinitis? In
microangiopathy:
Patient is usually asymptomatic
CD4 levels are normal (200500 cells/l)
Notes
How do you distinguish PORN from CMV retinitis and acute retinal
necrosis (ARN)? Four characteristics of PORN:
Absence of inammation (unlike ARN)
Early posterior pole involvement (unlike ARN)
Multifocal (unlike CMV)
Rapid progression (unlike CMV)
Notes
How do you differentiate toxoplasmosis in AIDS from the typical
toxoplasmosis that occurs in immunocompetent patients? In patients with
AIDS, toxoplasmosis is:
More severe
Bilateral
Multifocal
Not necessarily conned to the posterior pole
Not adjacent to old scars
Associated with CNS involvement
Requires treatment for life
Q Tell me about CMV Retinitis.
CMV retinitis is an important ocular complication of AIDS.

Developing in about 50% of patients in the past.
The clinical manifestations can be divided into central and peripheral retinitis.
CMV Retinitis
1. Clinical features Lack of inammatory signs (like presumed
50% of patients with AIDS (declining now with ocular histoplasmosis syndrome)
better treatment) Peripheral
Classication: More common than central type
Indolent/granular Foveal sparing granular retinal necrosis
Fulminant/hemorrhagic 2. Natural history
Central Relentless progression (like a bush re)
Dense, white, well-demarcated areas of Retinal detachment
retinal necrosis Retinal atrophy
Retinal hemorrhages along edge or within Resolution
areas of necrosis Recurrence
Cheese and ketchup appearance
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3. Treatment Valganciclovir:
Zone of involvement important and determines Oral prodrug of ganciclovir
urgency of treatment: Good bioavailability
Zone 1: Within 3,000m of fovea/1,500m of Useful for oral prophylaxis but main
optic disc (vision-threatening) limitation of cost (very expensive)
Zone 2: Up to vortex vein entrances Foscarnet (IV, oral and intraocular):
Zone 3: Peripheral vortex vein entrances Major complication is nephrotoxicity
All drugs inhibit DNA polymerase Cidofovir:
Ganciclovir (IV, oral and intraocular): Main complications are uveitis and
80% response nephrotoxicity
Induction phase with IV, followed by Not for intravitreal use causes severe
maintenance with oral (poor bioavailability), permanent hypotony
IV and/or intravitreal Response to treatment suggested by:
Major complication is bone marrow Decreasing size of lesions
suppression Decreasing activity of lesions
Exam tips:
One of the most important ocular complications of IADS. Remember the
natural history is 5Rs.
Q What are the Ocular Features of Syphilis?
Ocular involvement in syphilis is not common.

Usually occurs in secondary and tertiary stages.
Ocular Syphilis
1. Congenital syphilis Posterior segment:
Keratouveitis (interstitial keratitis, may be Chorioretinitis
bilateral) Neuroretinitis
Salt and pepper fundus Retinal vasculitis
Neuroophthalmic:
2. Primary syphilis
Optic neuritis and neuroretinitis
Systemic: Chancre on genitalia
CN palsies
Eye chancre (Conj chancre)
4. Tertiary
3. Secondary
Systemic:
Systemic: Skin rash, fever, weight loss, Granulomas form when immune system
arthralgias unable to clear the organism
Orbit and eyelids: Gummas produce a chronic inammatory
Eyelid rash state in the body with mass-effects upon the
Orbital periostitis local anatomy
Dacryocystitis Main features: Neurosyphilis and
Dacryoadenitis cardiovascular syphilis
Madarosis Anterior segment ndings similar to secondary
Anterior segment: syphilis (intersitial keratitis, uveitis etc)
Conjunctivitis Lens subluxation
Interstitial keratitis
Neuroophthalmic:
Episcleritis, scleritis
Pupils:
Uveitis
Argyll Robertson pupil
Iritis roseate (dilated iris capillaries)
Tonic pupils
Iritis papulosa (iris papules)
Horners syndrome
Iritis nodosa (iris nodules)
RAPD (optic atrophy)
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Others: Nystagmus
CN palsies VF defects
Ptosis
Exam tips:
Primary syphilis conjunctiva, secondary syphilis = anterior and posterior
segments and tertiary syphilis = neuroophthalmic lesions (i.e. involvement
moves deeper with each stage).
Q What are the Ocular Features of TB?
Gumma of ocular structures

Ocular involvement in TB is rare, but TB is one of the commonest causes of uveitis in Asia.
It can occur at the anterior segment or posterior segment or can be a neuroophthalmic involvement or a
result of complications from treatment.
Ocular TB 2. Posterior segment
1. Anterior segment Choroiditis (choroidal tubercles)
Eyelids (blepharitis, meibomitis) Retinitis
Lacrimal gland and system (dacryoadenitis, Vasculitis
dacryocystitis) Vitreous hemorrhage (Eales disease)
Orbital periostitis, cellulitis 3. Neuroophthalmic
Follicular conjunctivitis Optic neuritis, optic atrophy
Phlyctenulosis CN palsies
Conjunctiva nodules (tuberculomas) Internuclear ophthalmoplegia
4. Treatment related
Episcleritis/scleritis
Ethambutol and others (pages 249 and 414)
Uveitis (granulomatous)
Exam tips:
Most of the lesions are immune-related.
Q What are the Ocular Features of Leprosy?

Ocular involvement in leprosy can be divided into.
Ocular Leprosy
1. Leprosy mycobacteria favor cooler parts of the Lacrimal system
Dacryocystitis and nasolacrimal duct
body (e.g. skin). Ocular manifestations are thus
obstruction
primarily periocular or in the anterior segment
3. Cornea and sclera
2. Eyelid and lacrimal gland
Eyelid
Exposure keratopathy (VII CN palsy)
Madarosis
Trichiasis, distichiasis, entropion, ectropion
Lepromatous nodules, thickening of skin
Band keratopathy
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Thickened corneal nerves Corectopia, polycoria

Pannus and scarring Miosis (sympathetic nerves are preferentially
Episcleritis, scleritis involved)
Anisocoria, secrease response to light
4. Intraocular
Cataract and glaucoma
Granulomatous uveitis:
Iris atrophy, iris pearls, nodular iris lepromas 5. Neuroophthalmic
Pupils: Optic neuritis
Occlusio/seclusio pupillae CN palsies
Exam tips:
Most of the signs involve the eyelids and the anterior segment.
Notes
What are the possible mechanisms of pannus and scarring? Combination of:
Lid lesions
Exposure keratopathy
Secondary infective keratitis
Q Tell me about Lymes Disease.
Lymes disease is caused by the bacteria Borrelia burgdorferi.

Transmitted through the Ixodes sp. tick.
There are three classical stages.
There are systemic and ocular symptoms in each stage.
Lymes Disease
1. Stage 1 Ocular (posterior segment):
Granulomatous uveitis
Systemic (localized disease):
Intermediate uveitis
Erythema migrans rash
Retinal vasculitis
Fever, headache, arthralgia, myalgia (u like
Choroiditis
symptoms)
Ocular (anterior segment): 3. Stage 3
Follicular conjunctivitis Systemic (immune-related):
Periorbital edema Arthritis
2. Stage 2 Ocular (anterior segment):

Episcleritis
Systemic (disseminated disease):
Heart (arrhythmia, myocarditis)
Orbital myositis
CNS
Skin
Joints
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Exam tips:
Surprisingly, this uncommon condition is one of the favorite exam questions!
Remember that ocular involvement goes from the anterior segment (Stage 1)
to the posterior segment (Stage 2) and back to the anterior segment (Stage 3)
again!
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TOPIC 3
SYSTEMIC INFECTIOUS
DISEASES AND THE EYE II
Q Opening Question: What are the Ocular Complications of Toxocariasis?

Ocular complications of toxocariasis can be divided into three different presentations depending on the
age of infection.
Syndrome Age of presentation Clinical features Differential diagnoses

Chronic endophthalmitis Child (29) Leukocoria Retinoblastoma
Panuveitis (Endophytic type)
Posterior pole granuloma Teenager (414) Localized mass at posterior Retinoblastoma
pole (Exophytic type)
Peripheral granuloma Adult (640) Pseudoesotropia Dragged disc
Retinal detachment
Exam tips:
Important differential diagnosis of dragged disc and leukocoria (page 400).
Q What are the Causes of Dragged Disc?
The commonest cause is due to advanced cicatricial ROP.

Dragged Disc
1. Proliferative vascular diseases 3. Developmental disorders
Cicatricial ROP FEVR (Familial exudative vitreoretinopathy)
Proliferative DM retinopathy Combined hamartoma of retina and RPE
Sickle cell retinopathy Incontinentia pigmenti
Coats disease Norries disease
2. Infectious
Toxocariasis
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Q Tell me about Presumed Ocular Histoplasmosis Syndrome (POHS).
POHS is caused by fungal infection by Histoplasma capsulatum.

The organism is acquired by inhalation and may spread by the blood stream to the choroid.
Associated with HLA-B7.
Clinical Features of POHS
1. Atrophic histo spots 3. Subretinal neovascular membrane (SRNVM)
Yellow-white in color Usually develops adjacent to histo spot
Half disc diameter in size 4. No vitreous involvement
Asymptomatic unless macula is involved One of few white dot syndromes NOT to have
2. Peripapillary atrophy vitreous involvement
Exam tips:
Important differential diagnosis of white dot syndromes (page 353) and
SRNVM (page 176).
Notes
What are the other important causes of peripapillary atrophy?
Myopic degeneration (page 179)
Vogt-Koyanagi-Harada syndrome (page 350)
Q Tell me about Masquerade Syndromes.
Masquerade Syndromes
1. Nonneoplastic masquerade syndromes Leukemia
Ocular ischemic syndrome Uveal lymphoid proliferations
Chronic retinal detachment Nonlymphoid malignancies (e.g. melanoma,
Endophthalmitis retinoblastoma)
2. Neoplastic masquerade syndromes Metastases
CNS/systemic lymphoma
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TOPIC 4
TOXOPLASMOSIS
AND THE EYE
Q Opening Question: Tell me about Toxoplasmosis.

Toxoplasmosis is a common parasitic infection with systemic and ocular manifestations.
It is caused by Toxoplasma gondii.
The cat is the denite host and other organisms, including humans, are the intermediate hosts.
Ocular manifestations can be divided into congenital, acute acquired and recurrent.
Toxoplasmosis
1. Life cycle Anterior segment
Sporocyst: Granulomatous OR nongranulomatous
Excreted in cats feces uveitis

Human infection: Ingestion from soil Posterior segment (ALL structures in posterior
Bradyzoite: segment are involved):
Encysted in tissues (including retina) Supercial necrotizing retinitis:
Human infection: Ingestion from beef and Commonest form
other meat Distinguishing features (see toxoplasmo-
Tachyzoite: sis in AIDS for comparison, page 327):

Unilateral
Active proliferative form, responsible for
Focal
tissue destruction and inammation
Posterior pole
Human infection: Transplacental spread (from
Adjacent to scar
mother to fetus)
Vitreous haze (headlight in the fog)
2. Clinical manifestations
Local vasculitis around lesion
Congenital toxoplasmosis (see below) Deep retinitis:
Acute acquired toxoplasmosis (not common) Yellow distinct lesion with no vitritis
Immunocompetent: (deeper than supercial retinitis)
Fever
Outer retinitis:
Lymphadenopathy
Multifocal punctate white lesions
Rash
Choroid involvement:
Immunosuppressed: Massive granuloma
CNS and systemic manifestation
Optic nerve involvement:
Recurrent (congenital or acquired) Papillitis secondary to retinitis and
toxoplasmosis choroiditis next to ON
Primary lesion is an inner retinitis Vessel involvement:
Vasculitis and vascular occlusions
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Exam tips:
The life cycle can be remembered by sporocyst = soil, bradyzoite = beef,
tachyzoite = transplacental spread.
The majority of clinical features are in the posterior segment (all posterior
segment structures involved i.e. retina, choroids, ON, vessels).
Q What are the Features of Congenital Toxoplasmosis?
Congenital toxoplasmosis has systemic and ocular features.

Congenital Toxoplasmosis
1. Transmitted through placenta via tachyzoites 4. Ocular features
2. Severity depends on duration of gestation at time Bilateral chorioretinal scars:
Usually situated at macula
of maternal infection
May cause squint
3. Systemic features Should be differentiated from Best macular
CNS: dystrophy (which also causes bilateral macula
Epilepsy scars)
Intracranial calcication Optic atrophy
Hydrocephalus Others:
Fever Microphthalmos
Visceral organ involvement Cornea scars
Hepatosplenomegaly Iris scars
Cataract
Q How do You Manage a Patient with Ocular Toxoplasmosis?
The management of ocular toxoplasmosis depends on the patients immune status and severity of ocular
involvement.
In general, if the patient is not immunosuppressed, most ocular lesions do not need treatment.
The indications for treatment are.
Management of Ocular Toxoplasmosis
1. Natural history (note the number 3) 3. Treatment
Resolution Manage uveitis and associated complications
Three months (e.g. RD)
Recurrence Systemic therapy:
50% recurrence rate within 3 years Clindamycin:
Number of recurrence per person = 3 Major complication is pseudomembranous
2. Indications for treatment colitis

Patient factors: Sulphur drugs:
Sulphadiazine
Immunosuppression (AIDS)
Co-trimoxazole (septrin)
Severity of ocular involvement:
Pyrimethamine (folic acid antagonist):
Location of lesion: Macula, papillomacular
Major complication is anemia (need to
bundle or around the ON
Size of lesion: > 1 disc diameter
moniter blood counts and add oral folic
Severe inammation: Severe vitritis, cystoid
acid supplements)
Spiramycin:
macular edema
Indicated for pregnancy (spiramycin
Complications: Tractional RD, epiretinal
membrane concentrated in the placenta)
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Systemic steroids: Azithromycin

Indicated for severe vitritis (avoid in AIDS) Treatment regimes:
Atovaquone: Typically given as triple-therapy:
Cysticidal but no clinical superiority Pyrimethamine + sulphadiazine + steroids

demonstrated so far May also be given as quadruple therapy:
Triple therapy + clindamycin
Exam tips:
A common mistake is to jump straight into the myriad of drugs available.
Most do not need treatment.
Clinical approach to toxoplasmosis scar

There is a solitary, round, pigmented, punched out retinal scar.
Located temporal to the macula, measuring one disc diameter in size.
Look for
Vitritis overlying scar
Satellite lesion
Surrounding perivascular sheathing
Disc hyperemia
Id like to
Perform SLE (granulomatous or nongranulomatous uveitis, cataract)
Examine fellow eye
Frequent question: Would you treat this patient?
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TOPIC 5
ARTHRITIS AND THE EYE

Q Opening Question: What Arthritic Diseases are Associated with Uveitis?

There are three groups of arthritic conditions commonly associated with uveitis.
Spectrum of Arthritic Diseases Associated with

Uveitis 3. Others
1. Spondyloarthropathies/seronegative arthritis Systemic lupus and other connective tissue
Ankylosing spondylitis diseases
Reiters syndrome Behcets disease
Psoriatic arthritis
Inammatory bowel disease
2. Rheumatoid arthritis/juvenile rheumatoid
arthritis/seropositive arthritis
Exam tips:
See also connective tissue disease and the eye (page 342).
Q What are Spondyloarthropathies?
Spondyloarthropathies or seronegative arthritis are a group of arthritic conditions.

With systemic clinical features that involves the axial skeleton and extraarticular features.
And characteristic uveitis with intense brinous reaction.
Spondyloarthropathy/Ankylosing Spondylitis
1. Systemic features Sacroiliatis:
Men (peak 2040 years) Buttock pain alternating from one side to
Blood tests: another

Rheumatic factor negative (therefore called Extraarticular features
seronegative) 2. Uveitis (note: Six features)
ANA usually negative Acute
HLA-B27 usually positive
Anterior
Family history common Unilateral, alternating
Arthropathy: Nongranulomatous
Axial skeleton inammation:
Response to steroids
Spinal pain worse at night and with rest,
Recurrent
better with activity (compare this with
mechanical spinal disorders pain worse
with activity)
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Exam tips:
These features are useful to remember because they apply to ankylosing
spondylitis and most of the other spondyloarthropathies!
Q What is HLA-B27?
HLA stands for human leukocyte antigen.

HLA-B27 refers to a specic antigen.
Commonly found in the population.
Important Facts About HLA-B27
1. HLA (Human leukocyte antigen) Reiters syndrome: 75%
Iso/allo antigen found on surface of cells Ankylosing spondylitis: 85%
Differentiate one individual from another Ankylosing spondylitis and
Basis for graft rejection and blood transfusion anterior uveitis: 95%
reaction 3. Relative risk of AS with HLA-B27 = 90
Genotype found on chromosome 6, region
4. What are the indications for HLA testing?
called MHC (major histocompatibility complex)
To determine the cause of acute, unilateral,
2. Prevalence of HLA-B27 anterior uveitis (e.g. not useful in chronic,
General population: 8% bilateral, posterior uveitis)
Acute anterior uveitis: 45% To exclude other diseases
Psoriatic arthritis, To predict prognosis for recurrence
inammatory bowel disease: 60% To predict risk of spondyloarthropathy
Common HLA Associations
Relative risk
HLA Diseases (Normal person = 1)
HLA-A29 Birdshot retinochoroidopathy 97
Ankylosing spondylitis 90
HLA-B27
Other spondyloarthropathies 10
HLA-B5 Behcets syndrome
Presumed ocular histoplasmosis
HLA-B7
syndrome
Juvenile DM
HLA-DR4
Vogt-Koyanagi-Harada syndrome
Q Tell me about Reiters Syndrome.
Reiters syndrome is a spondyloarthropathy or seronegative arthritis.

With the characteristic TRIAD of urethritis, conjunctivitis and arthritis.
The systemic clinical features are.
Reiters Syndrome
ANA usually negative
1. Systemic features HLA-B27 usually positive
Young men Urethritis or dysentery:
Blood tests: Nonspecic
Rheumatic factor negative (seronegative) Sterile
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Arthropathy 2. Ocular
Acute arthritis (knees or ankles) Conjunctivitis:
Extraarticular features: Bilateral papillary conjunctivitis
Painless mouth ulcers (compare this with Sequence of events: Urethritis, followed by
Behcets disease, page 350) conjunctivitis and arthritis
Skin rash (keratoderma blennorrhagica) Keratitis
Penile erosions (circinate balanitis)
Uveitis:
Cardiovascular problems
Acute anterior uveitis
Q Tell me about Psoriatic Arthritis.

Psoriatic arthritis is a seronegative arthritis.
With characteristic skin rash and ocular features.
Psoriatic Arthritis
1. Systemic features Arthritis:
Both sexes 10% of psoriasis:
Blood tests: Hand joints
Rheumatic factor negative (seronegative) Extraarticular features:

ANA usually negative Nail changes
HLA-B27 usually positive 2. Ocular

Skin rash: 10% of those with psoriatic arthritis (i.e. only
Chronic scaling and plaques, red with 1% of all psoriasis patients will have eye signs)
silvery scales Conjunctivitis, keratitis, uveitis
Bilateral but asymmetrical
Q Tell me about Inammatory Bowel Disease.
Inammatory bowel disease (IBD) is a systemic condition, classically divided into Crohns disease
and ulcerative colitis.
With characteristic gastrointestinal features and ocular features.
Inammatory Bowel Disease
1. Systemic features Arthritis:
Gastrointestinal: Typical spondyloarthropathy features
Crohns disease: Others:
Whole gastrointestinal tract, especially Hepatobiliary complications
small bowels Skin rash
Segmental, skip lesions Renal complications
Transmural 2. Ocular
Risk of perforation Primary:
Ulcerative colitis (compare this with Crohns Uveitis in 10% (more common in ulcerative
colitis): colitis than Crohns disease)
Rectum and colon Conjunctivitis, keratitis, scleritis
Continuous lesions Secondary:
Conned to mucosa Hypovitaminosis (page 413)
Risk of CA colon
Q What are the Different Gastrointestinal Diseases that have Prominent Ocular Manifestations?
Gastrointestinal diseases are associated with a variety of ocular manifestations.

Gastrointestinal Diseases and the Eye
1. Corneal complications Kayser-Fleischer ring
Primary biliary cirrhosis and Wilsons disease Corneal arcus
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2. Uveitis Pancreatitis
IBD (Crohns disease and ulcerative colitis) Purtschers retinopathy
Reiters Liver diseases, chronic diarrhoea
Whipples disease Vitamin A deciency and night blindness
3. Retina complications
Familial polyposis coli
Congenital hypertrophy of RPE (CHRPE)
Q Tell me about Juvenile Rheumatoid Arthritis.

Juvenile rheumatoid arthritis (JRA) is a systemic condition in children.
It is classically divided into three types.
With characteristic systemic and ocular features in each type.
Pauciarticular (four
Systemic Polyarticular or fewer joints) Pauciarticular
(Stills disease) (ve or more joints) Late onset type Early onset type
Frequency 20% 40% 20% 20%
(20% RF positive,
20% RF negative)
Salient features Systemic disease Resembles RA in Resembles Highest uveitis rate
(fever, rash, adults, main problem ankylosing Uncommon systemic
hepatosplenomegaly) is severe arthritis spondylitis or arthritic
Uveitis rare complications
Demographics More common in More common in More common in More common in girls
boys girls boys Early childhood
Early to late Early to late Late childhood
childhood childhood
Arthritis Any joints Any joints, but small Sacroiliac and hip Large joints (knee,
joints frequent (hand, joints ankle, elbow)
ngers)
Uveitis Rare Uncommon Common (1020%) Very common
(2040%)
Rheumatoid Negative RF 50% positive RF Negative RF Negative RF
factor (RF) Negative HLA-B27 Negative HLA-B27 75% positive Negative HLA-B27
and HLA-B27 Negative ANA 50% positive ANA HLA-B27 75% positive ANA
Negative ANA
Frequency of Yearly 69 monthly 4 months 3 months
ophthalmic
follow-up
required
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Complications
Complications are frequent but usually minimally Band keratopathy
symptomatic, hence need for regular follow-up: Cataract
Glaucoma CME
Exam tips:
See also arthritis and eye (above), skin and eye (page 415), renal diseases and
eye (page 210), cardiovascular diseases and eye (page 200), and cancer
and the eye (page 360).
Exam tips:
Risk of uveitis = pauciarticular, early onset and ANA positive JRA.
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TOPIC 6
CONNECTIVE TISSUE
DISEASES AND THE EYE
Q Opening Question: What are the Ocular Features of Rheumatoid Arthritis (RA)?
Rheumatoid arthritis (RA) is a chronic inammatory disease of the joints.
The ocular manifestations can be divided into those affecting the anterior segment, those affecting the
posterior segment, and those due to treatment.
Ocular Manifestations of Rheumatoid Arthritis

1. Cornea 2. Sclera
Keratoconjunctivitis sicca (plus xerostomia = Episcleritis and RA nodules
Sjogrens syndrome) Scleritis
Peripheral keratitis (note: Most important ocular 3. Posterior segment and beyond
manifestation, 4 types, 2 central, 2 peripheral): Venous stasis retinopathy
Sclerosing keratitis CN palsies
Acute stromal keratitis
Orbital apex syndrome
Peripheral corneal thinning
Abnormal EOG
Peripheral corneal melting
Cortical blindness
Filamentary keratitis
Microbial keratitis 4. Treatment complications
Steroids, gold, chloroquine
Exam tips:
RA affects mainly the anterior segment (cornea and sclera).
Q What are the Systemic Effects of Rheumatoid Arthritis (RA)?
RA is a chronic multisystem inammatory disease.

Characterized by symmetrical arthritis, synovial inammation, cartilage and bone destruction.
Systemic Manifestations of RA
1. Diagnosis (American Rheumatological Arthritis of hand joints (wrist, metacarpal (MCP)
Association criteria: Needs four out of the seven or proximal interphalangeal (PIP) joints)
Symmetrical swelling of same joint area (wrist,
points below for diagnosis. 14 must last > six
MCP, PIP)
weeks)
Morning stiffness lasting > one hour before
Arthritis of three or more joints: Soft tissue
improvement
swelling and uid observed by physician
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Rheumatoid nodules Neurological:

Serum RF Atlantoaxial subluxation
Radiographic features of RA Peripheral neuropathy and mononeuritis
multiplex
2. Manifestations
Entrapment neuropathies
Arthritis:
Hematological:
Symmetrical inammatory polyarthropathy
Anemia
Wrist, metacarpophalangeal (MCP), proximal
Neutropenia (plus splenomegaly and leg
interphalangeal (PIP) joints affected
ulcers = Feltys syndrome),
Sparing of distal interphalangeal (DIP)
joints Renal:
Amyloidosis
Swan-neck deformity (hyperextension of
PIP) 3. Treatment
Boutonnieres sign (exion deformity of PIP) Drugs:
Skin/subcutaneous: NSAIDS
RA nodules Steroids
Vasculitis Immunosuppressants
Cardiovascular and respiratory: Physiotherapy, occupational therapy
Pleurisy and pleural effusion Surgery
Fibrosing alveolitis
Q What are the Ocular Features of Systemic Lupus Erythematosus?
Systemic lupus erythematosus is a multisystem autoimmune disease.

Commonly affecting young women.
Common systems involved include skin, blood vessels and CNS.
Ocular features are most commonly seen in the posterior segment.
Ocular Manifestations of Systemic Lupus

Erythematosus
1. Post segment (note: Clinical features are nearly 3. Neurological
IDENTICAL to that in hypertensive retinopathy) Sensory:
Retinal hemorrhages Optic neuritis, anterior ischemic optic
Cotton wool spots (because arterioles neuropathy

Pupil abnormalities
preferentially affected)
Homonymous hemianopia
Hard exudate
Cortical blindness
Arteriolar narrowing
Motor:
Venous engorgement
Ptosis
BRVO/BRAO/CRVO/CRAO Nystagmus
Disc edema III & IV CN palsy
2. Anterior segment Gaze palsy
Cornea punctuate epithelial keratitis, Inter-nuclear ophthalmoplegia
keratoconjunctivitis sicca, peripheral ulceration 4. Treatment (similar to RA)
Sclera episcleritis/scleritis
Anterior uveitis
Exam tips:
Systemic lupus erythematosus affects mainly the posterior segment (compared
to RA).
B1037_Ch-08.indd 414 12/24/2010 2:47:01 PM

Q What are the Systemic Effects of Systemic Lupus Erythematosus?
Systemic lupus erythematosus is a multisystem autoimmune disease.

Characterized by involvement of skin, joints, cardiovascular and neurological systems.
Systemic Manifestations of Systemic Lupus

Erythematosus
1. Diagnosis (4 or more of 11 features) Serositis
Malar rash Renal involvement
Discoid rash Neurological involvement
Photosensitivity Hematological involvement
Mucosal ulcers Anti-DNA antibodies, anti-Sm antibody
Arthritis ANA
Q What are the Ocular Features of Wegeners Granulomatosis?
Wegeners granulomatosis is a multisystem inammatory disease of unknown etiology.

The ocular manifestations can be divided into orbital, anterior segment, posterior segment, neurological
and treatment related.
Ocular Manifestations of Wegeners
Granulomatosis
1. Orbit 3. Posterior segment
Orbital inammatory disease (pseudotumor) Vasculitis (CRAO, BRAO, cotton wool spots)
Sinusitis leading to orbital abscess Hypertensive retinopathy
NLD obstruction 4. Neurological
2. Anterior segment Anterior ischemic optic neuropathy
Conjunctivitis CN palsies
Episcleritis/scleritis (necrotizing) 5. Treatment
Keratitis peripheral ulceration Immunosuppression (cyclophosphamide)
Uveitis
Exam tips:
Wegeners granulomatosis affects mainly the orbit.
Q What are the Systemic Effects of Wegeners Granulomatosis?
Wegeners granulomatosis is a multisystem inammatory disease of unknown etiology.

With primary involvement of lungs, vessels and kidneys.
Systemic Manifestations of Wegeners

Granulomatosis
1. Diagnosis (classic diagnostic TRIAD) 2. Investigations (to investigate the classic
Respiratory tract (necrotizing granuloma of diagnostic TRIAD)
lungs) Respiratory tract (CXR)
Vasculitis Vasculitis (serum C-ANCA levels)
Nephritis Nephritis (urine exam)
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Q What are the Features of Polyarteritis Nodosa (PAN)?

PAN is a multisystem vasculitis of unknown etiology.
It mainly involves medium size and small vessels.
There are both systemic involvement and ocular involvement.
The ocular manifestations can be divided into.
Ocular Manifestations of PAN
1. Anterior segment CN palsies
Episcleritis/scleritis 4. Treatment
Keratitis peripheral ulceration
Systemic Manifestations of PAN
Interstitial keratitis (one of the few systemic
1. General features
causes of interstitial keratitis)
Nephritis
2. Posterior segment
Cardiovascular (myocardial infarct)
Vasculitis (CRAO, BRAO, cotton wool spots) Bowel infarction
Hypertensive retinopathy Skin (vasculitic lesions)
3. Neurological Arthritis
Anterior ischemic optic neuropathy Neurological (peripheral neuropathy)
Exam tips:
If the question is What are the features?, do not forget to mention the
systemic features FIRST.
PAN affects mainly the blood vessels.
Q What are the Features of Systemic Sclerosis?
Systemic sclerosis is a multisystem disease of unknown etiology.

It mainly involves the skin and blood vessels.
There are both systemic involvement and ocular involvement.
The ocular manifestations can be divided into.
Ocular Manifestations of Systemic Sclerosis
1. Lids
Sclerodactyl
Lagophthalmos Nailfold infarcts
Punctal ectropion and epiphora Telangiectasia
Linear scleroderma Bowel (esophageal brosis)
2. Anterior segment Nephritis
Keratoconjunctivitis sicca Cardiovascular (serositis)
3. Posterior segment Respiratory (brosis)
Hypertensive retinopathy 2. CREST syndrome (a more benign form of systemic
Systemic Manifestations of Systemic Sclerosis sclerosis)
1. General features Calcinosis
Skin: Raynauds phenomenon
Sclerodermatous skin changes, bird-like facies Esophageal
Raynauds phenomenon Sclerodactyl
Calcinosis Telangiectasia
Exam tips:
Systemic sclerosis affects mainly the eyelids and skin.
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TOPIC 7
SPECIFIC UVEITIS
SYNDROMES I
Q Opening Question: What are the Clinical Features of Sarcoidosis?

Sarcoidosis is an idiopathic systemic condition.
Characterized pathologically by presence of noncaseating granuloma.
Affecting the lungs and other organs.
Sarcoidosis
1. Pathology Lupus pernio (sarcoid rash near eyelid
Noncaseating granuloma margin)
2. Systemic features Anterior segment:
Acute unilateral nongranulomatous anterior
Acute presentation:
uveitis OR chronic bilateral granulomatous
Young adult
panuveitis
Lung:
Stage 1: Bilateral hilar lymphadenopathy
Posterior segment:
Vitritis (snowballs)
Stage 2: Bilateral hilar lymphadenopathy
Retinitis
and reticulo nodular parenchymal
Vasculitis (candle wax appearance, BRVO,
inltrates
Stage 3: Reticulo nodular parenchymal
neovascularization)
ON involvement
inltrates alone
Stage 4: Progressive pulmonary brosis 4. Investigation (STEPWISE APPROACH, from
Erythema nodosum rash noninvasive to invasive)
Parotid enlargement: Step 1:
Plus VII CN palsy and anterior uveitis =
CXR
Heerfordts syndrome Serum angiotensin-converting enzyme (ACE)
Acute unilateral nongranulomatous anterior levels (monocytes secretes ACE in sarcoidosis)
uveitis Serum and urinary calcium levels
Insidious onset: Step 2:
Older adult Chest CT or MRI
Nonspecic (weight loss, fever) Gallium scan of head, neck and chest
Lung, skin, joints, CNS, CVS, renal Lung function tests
involvement, hepatosplenomegaly and Step 3:
lymphadenopathy Lung and lymph node biopsy
Chronic bilateral granulomatous panuveitis
Lacrimal gland and conjunctival biopsy
3. Ocular features Step 4:
30% of patients Bronchoalveolar lavage
Orbit and lids:
Granuloma
B1037_Ch-08.indd 417 12/24/2010 2:47:02 PM

Notes
What is a noncaseating granuloma?
Consists of:
Epitheloid cells (derived from monocytes, macrophages)
Giant cells (Langhans type):
Schaumanns body; cytoplasmic inclusion
Asteroid inclusion body (acidophilic, star-shaped)
Q What is Fuchs Uveitis Syndrome? How is it Different from Posner-Schlossman Syndrome?

Fuchs uveitis is a common idiopathic uveitis with distinct clinical features.
Posner-Schlossman syndrome is also an idiopathic uveitis characterized by recurrent attacks of glaucoma.
There are several features which help distinguish the two conditions.
The role of infectious agents, particularly rubella, toxoplasmosis, and CMV, in the pathogenesis of these
forms of uveitis is currently under investigation.
Fuchs uveitis Posner-Schlossman syndrome

Age and sex Middle age to elderly Young to middle age
Females more common Males
Presentation Asymptomatic, sometimes with blurring of Acute blurring of vision and halos
vision Acute pain
Keratic precipitates: Diffuse Inferior half of corneal endothelium
Well-dened May be conuent
Small, stellate-shaped Larger
White-gray in color Colorless
May disappear with steroid
treatment
IOP Mid-20s High 3040s
Other features AC activity mild Very similar to Fuchs uveitis
Iris: Less iris atrophy and heterochromia
Heterochromia iridis Long term risk of glaucoma up
Iris atrophy (moth-eaten pattern at pupil to 40%
border)
Iris nodules
No posterior or peripheral anterior synechiae
Rubeosis (ne): Amslers sign acute
hyphema following ocular decompression
such as paracentesis
Cataract
Retina:
Pigmentation/scarring (hence possible role
for rubella/toxoplasmosis)
No CME
Glaucoma (chronic POAG):
Common (40%) and important cause of
visual loss in FHI
Gonioscopy:
Rubeosis
Bleeding 180o opposite site of AC
paracentesis (Amslers sign)
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Clinical approach to Fuchs heterochromic uveitis

On examination of this patients anterior segment.
There are gray white keratic precipitates scattered diffusely throughout
the endothelium.
The keratic precipitates are well-defined, small, stellate-shaped and
nonconfluent in nature.
Look for
Cornea (should be clear)
AC activity (mild)
Iris:
Iris atrophy (moth eaten pattern at pupil border)
No posterior or peripheral anterior synechiae
Pupil is dilated but reactive
Rubeosis
Cataract
Fellow eye iris:
Affected eyes iris is hypochromia
Ill like to
Check IOP
Perform gonioscopy (rubeosis)
Exam tips:
Do not confuse Fuchs uveitis with Fuchs endothelial dystrophy (page 113).
The comparison between Fuchs uveitis and Posner-Schlossman syndrome is
clinically important because it is often difcult to tell the two apart in daily
practice.
Q What are the Causes of Iris Heterochromia?
Iris hypochromia or iris hyperchromia can be either congenital or acquired.
Congenital Acquired
Hypochromia Congenital Horners syndrome Uveitis (Fuchs, Posner-Schlossman, HZV,
Waardenburgs syndrome HSV, leprosy)
Hirschsprungs disease Glaucoma (pseudoexfoliation, pigmen-
Facial hemiatrophy (Parry-Romberg tary dispersion, post angle closure
syndrome) glaucoma)
Post trauma/surgery
Juvenile xanthogranuloma
Hyperchromia Oculodermal/ocular melanosis Uveitis (Fuchs)
Sector iris pigment epithelial harmatoma Glaucoma (pigmentary dispersion)
Iridocorneal endothelial syndrome
(ICE)
Diffuse pigmentation (siderosis, argyrosis,
chalosis, hemosiderosis)
Iris tumors (nevus, melanomas)
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TOPIC 8
SPECIFIC UVEITIS
SYNDROMES II
Q Opening Question: What is Behcets Disease?

Behcets disease is an idiopathic multisystem disorder.
With characteristic, systemic clinical features and uveitis.
Behcets Disease
1. Pathology Skin lesions:
Associated with HLA-B5 Erythema nodosum
Obliterative vasculitis with brinoid Papular, pustular or nodular rash
degeneration Positive pathergy test (page 325)

Type III hypersensitivity Eye lesions
Other systemic features (NOT part of diagnostic
2. Systemic features
criteria):
Young men of Japanese, Asian or Mediterranean
Arthritis
origin
Thrombophlebitis
Diagnostic criteria (ve features: Oral Gastrointestinal lesions
ulceration plus any two of the other four, Cardiovascular involvement (myocardial
International Study Group for Behcets) infarct)
Oral ulceration: CNS involvement (stroke)
Painful and recurrent
3. Ocular features
At least three times in last one year
70% of patients
99% of cases
Genital ulceration
Severe bilateral nongranulomatous panuveitis
(iritis, retinitis, vitritis, vasculitis)
Q Tell me about Vogt-Koyanagi-Harada Syndrome.
Vogt-Koyanagi-Harada syndrome is an idiopathic multisystem disorder.

With characteristic systemic clinical features and uveitis.
Vogt-Koyanagi-Harada Syndrome
1. Men of Japanese or Oriental origin CNS lesions, triad of:
Encephalopathy
2. Systemic features
Meningeal irritation
Triad of:
CSF pleocytosis
Skin lesions, triad of:
Alopecia
Auditory symptoms, triad of:
Vertigo
Poliosis
Tinnitus
Vitiligo
Deafness
B1037_Ch-08.indd 421 12/24/2010 2:47:03 PM

3. Uveitis Pigment epithelial atrophy (sunset glow

Bilateral granulomatous panuveitis fundus)
Acute, triad of Ds: 4. Imaging features
Detachment of retina (multifocal choroiditis
B-scan: Choroidal thickening
and exudative RD)
FFA: Starry sky pattern, diffuse choroidal
Disc swelling
leakage, exudative RD
Dalen-Fuchs nodules (inammatory cells in
RPE and Bruchs membrane) ICG: Hypouorescent dark areas
Chronic, triad of Ps: Diagnostic criteria formulated by Read et al. Revised
Pigmentary changes and scarring diagnostic criteria or VKH disease: A Report of an
(pseudo retinitis pigmentosa) International Committee on Nomenclature. Am J
Ophthalmol 2001;131:647652.
Diagnostic Criteria for Vogt-Koyanagi-Harada Disease
Complete Vogt-Koyanagi-Harada disease (criteria 1 to 5 must be present)

No history of penetrating ocular trauma or surgery preceding the initial onset of uveitis
No clinical or laboratory evidence suggestive of other ocular disease entities
Bilateral ocular involvement (a or b must be met, depending on the stage of disease when the patient is examined)
Early manifestations of disease
There must be evidence of a diffuse choroiditis (with or without anterior uveitis, vitreous inammatory
reaction, or optic disk hyperemia), which may manifest as one of the following:
Focal areas of subretinal uid, or
Bullous serous retinal detachments
With equivocal fundus ndings; both of the following must be present as well
Focal areas of delay in choroidal perfusionv, multifocal areas of pinpoint leakage, large placoid areas of
hyperuorescence, pooling within subretinal uid, and optic nerve staining (listed in order of sequential
appearance) by uorescein angiography, and
Diffuse choroidal thickening, without evidence of posterior scleritis by ultrasonography
Late manifestations of disease
History suggestive of prior presence of ndings from 3a, and either both (2) and (3) below, or multiple signs
from (3):
Ocular depigmentation (either of the following manifestations is sufcient):
Sunset glow fundus, or
Sugiura sign
Other ocular signs:
Nummular chorioretinal depigmented scars, or
Retinal pigment epithelium clumping and/or migration, or
Recurrent or chronic anterior uveitis
Neurological/auditory ndings (may have resolved by time of examination)
Meningismus (malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a
combination of these factors;
headache alone is not sufcient to meet denition of meningismus, however), or
Tinnitus, or
Cerebrospinal uid pleocytosis
Integumentary nding (not preceding onset of central nervous system or ocular disease)
Alopecia, or
Poliosis, or
Vitiligo
Incomplete Vogt-Koyanagi-Harada disease (criteria 1 to 3 and either 4 or 5 must be present):
No history of penetrating ocular trauma or surgery preceding the initial onset of uveitis, and
No clinical or laboratory evidence suggestive of other ocular disease entities, and
Bilateral ocular involvement
Neurologic/auditory ndings; as dened for complete Vogt-Koyanagi-Harada disease above, or
Integumentary ndings; as dened for complete Vogt-Koyanagi-Harada disease above.
(Continued )
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(Continued )
Probable Vogt-Koyanagi-Harada disease (isolated ocular disease; criteria 1 to 3 must be present):
No history of penetrating ocular trauma or surgery preceding the initial onset of uveitis
No clinical or laboratory evidence suggestive of other ocular disease entities
Bilateral ocular involvement as dened for complete Vogt-Koyanagi-Harada disease above
Clinical approach to Vogt-Koyanagi-Haradas disease

On examination of this patients fundus, there are areas of atrophy and
pigmentation seen.
Look for
Vitritis
Disc hyperemia
Multifocal areas of exudative RD
Dalen-Fuchs nodules
Pigmentary changes in periphery
Sunset glow fundus
Skin: Alopecia, vitiligo, poliosis, perilimbal vitiligo
Ill like to
Check the anterior segment (granulomatous uveitis, cataract)
Check IOP
Examine fellow eye
Ask for history of vertigo, tinnitus, deafness
Examine patient neurologically
Exam tips:
Remember the different TRIADS.
VKH may be subdivided into: Vogt-Koyanagis syndrome (anterior uveitis and
skin lesions) and Haradas syndrome (posterior uveitis and CNS lesions).
Q What are the Clinical Features of Sympathetic Ophthalmia? How does it Differ from VKH?
Sympathetic ophthalmia is a rare granulomatous panuveitis.

Sympathetic Ophthalmia
1. Exciting eye: Penetrating injury or intraocular Acute, triad of Ds:
surgery Detachment of retina (multifocal choroiditis
and exudative RD)
2. Sympathizing eye: Fellow eye
Disc swelling
3. Clinical features Dalen-Fuchs nodules (inammatory cells in
Onset: Two weeks to one year after RPE and Bruchs membrane)
initial event Chronic, triad of Ps:
Earliest symptom: Decreased accommodation Pigmentary changes and scarring (pseudo
(ciliary body involvement) retinitis pigmentosa)
Earliest sign: Retrolental cells Peripapillary atrophy
Bilateral granulomatous panuveitis Pigment epithelial atrophy (sunset glow fundus)
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VKH Sympathetic ophthalmia

Demographics 2050 years Younger
Asians and blacks No racial preference
History of trauma or Uncommon Common
surgery
Clinical features Skin changes Uncommon
CNS changes Uncommon
Hearing changes Uncommon
Pathological features Involvement of choriocapillaris Choriocapillaris spared
(sympathize with choriocapillaris)
Exam tips:
Usually compared closely with VKH because the ocular features are
IDENTICAL.
Q What is Intermediate Uveitis?
Intermediate uveitis is an uncommon idiopathic uveitis.

Intermediate Uveitis
1. Classication Vitritis with snowballs and snowbanking
Primary Periphlebitis anterior to equator
Secondary: Two spectrums:
Sarcoidosis Pars planitis: Snowbanking prominent
Retinitis pigmentosa Cyclitis: No snowbanking
Multiple sclerosis Complications:
TB, syphilis, Lymes disease, toxocara Secondary anterior segment involvement
2. Clinical features of idiopathic type (cataract)
Secondary posterior pole involvement
Young adult
(cystoid macula edema, RD)
Bilateral involvement
Quiet anterior segment and no primary posterior
pole involvement (note: Uveitis is
intermediate)
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Q What are the White Dot Syndromes?
The white dot syndromes are a group of idiopathic posterior uveitis.

They have overlapping clinical features.
Multifocal
APMPPE MEWDS PIC choroiditis Birdshot Serpiginous
Age Young Young Young Young Middle Middle
age age
Sex preference None Females Females Females Females None
Clinical Bilateral Unilateral No Severe Bilateral Bilateral

features Subacute Acute vitritis/ vitritis/ Chronic Chronic
Flu-like Flu-like anterior anterior Indistinct Amoeboid
illness illness uveitis uveitis lesions punched
Creamy Tiny Myopia half disc out
lesions granular common diameter lesions
lesions Small Radiate Radiate
Enlarged lesions from disc from disc
blind spot HLA-B29
(99%)
APMPPE: Acute posterior multifocal placoid

pigment epitheliopathy
MEWDS: Multiple evanescent white dot syndrome
PIC: Punctate inner choroidopathy
Multifocal choroiditis: Multifocal choroiditis with
panuveitis
Birdshot: Birdshot retinochoroidopathy
Serpiginous: Serpiginous choroidopathy
Exam tips:
One of the most difcult topics to remember in ophthalmology!
The rst step is to compare and contrast the syndromes in groups of two:
APMPPE and MEWDS, PIC with multifocal choroiditis and birdshot with
serpiginous. The rst four occur in young adults, the last two in middle-aged
adults.
The next step is to identify the salient associations in each.
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TOPIC 9
ANTERIOR SEGMENT
TUMORS
Q Opening Question: What are the Possible Diagnoses of a Pigmented Conjunctival Lesion?
Possible differential diagnoses include.
Racial Oculodermal Malignant

melanosis melanosis Nevus PAM melanoma
Age of onset Child Young adult Young adult Middle- Middle-aged
and race Pigmented Pigmented White aged White
races race White
Laterality Bilateral Unilateral Unilateral Unilateral Unilateral
Clinical Limbal and Subepithelial Bulbar Multifocal Pigmented
features interpalpebral (sclera or conjunctiva Any part of nodule
region episclera) Sharply conjunctiva Can be non-
Epithelial Adjacent demarcated No cysts pigmented
Static dermal Inclusion Wax and Limbus
pigmentation cysts wane in Fixed to
(nevus of Ota) appearance underlying
Spontaneous
bleeding
Other None Dermal High risk of High risk of Arise from PAM
associations pigmentation conjunctival conjuncti- (50%), nevus
on shoulder melanoma val (25%) and de
blades (palpebral melanoma novo (25%)
(nevus of Ito) and fonix (50% risk if
Uveal nevus, nevus biopsy
melanoma straddling shows
No risk of cornea, atypia)
conjunctival enlarging
melanoma nevus)
Risk of
glaucoma
(Continued )
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(Continued )
Racial Oculodermal Malignant
melanosis melanosis Nevus PAM melanoma
Treatment None Follow-up for Local excision Local Wide margin
melanoma with bare excisional local excision
Manage sclera biopsy and Lamellar
glaucoma cryothera- kerato-
py to sclera sclerectomy
with lamellar
keratoplasty
Cryotherapy
Topical MMC
Exenteration
and
chemotherapy
PAM: Primary acquired melanosis
Clinical approach to nevus of Ota

There is an area of subepithelial melanosis.
Associated with pigmentation of the lids and face.
In the distribution of the first and second divisions of the trigeminal nerve.
Look for
Proptosis (orbital melanoma)
Iris pigmentation/melanosis/melanoma
Lens subluxation (ciliary body melanoma)
Trabeculectomy (glaucoma operation)
Optic disc (cupping)
Id like to
Check IOP, gonioscopy (angle pigmentation)
Examine the fundus for choroidal melanoma
Examine patients back for nevus of Ito
Exam tips:
Fairly common clinical examination case.
Q What are the Differential Diagnoses of Iris Nodules?
The main causes can be divided into tumors and non-tumor lesions.
Iris Nodule
1. Tumors Malignant:
Benign: Primary:
Iris nevus Maligant melanoma
Iridocorneal endothelial syndrome (ICE) Leiomyoma
Oculodermal melanosis (nevus of Ota) Leukemia
Secondary
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2. Non-tumor conditions What is the histology? Nevus cells

Infection/inammation: Downs syndrome (Brusheld spots)
Granulomatous uveitis (Koeppe and Busacca What is the histology? Areas of normal
nodules) stroma surrounded by ring of hypoplasia

Fungal endophthalmitis Juvenile xanthogranuloma
Trauma: What is the histology? Granulomatous
Inclusion cyst lesion with lipid lled histiocytes and

Retained IOFB Touton giant cells
Developmental:
Neurobromatosis (Lischs nodule)
Clinical approach to iris nodule

This patient has a pigmented iris nodule at the 9 oclock position.
Measuring about 2 mm in size.
Look for
New vessels, pupil distortion, ectropian uvea, lens opacity (melanoma)
Keratic precipitates and AC cells (Koeppe or Busccada nodules)
Iris atrophy (ICE syndrome)
Conjunctival subepithelial melanosis (nevus of Ota)
Systemic features (neurofibromatosis, Downs syndrome)
Ill like to
Check IOP (ICE syndrome, melanoma) and perform gonioscopy
Ask for a history of trauma (traumatic inclusion cyst) and use of pilocarpine
(iris cyst)
Examine patient systemically (neurofibromatosis)
Exam tips:
The suggestive features of malignant melanoma can be remembered by the
mnemonic RIPPLE.
Notes
What are the suggestive features of malignancy?
Rubeosis
IOP increase
Pupil distortion
Photograph documentation of growth
Lens opacity
Ectropian uvea
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Notes
What are the other causes of giant cells?
Infections: TB (Langhans type), syphilis, leprosy
Noninfectious diseases: Sarcoidosis (Langhans type), foreign body
Q Tell me about Tumors of the Ciliary Body.
The most important ciliary body tumor is ciliary body melanoma.

Other tumors can be divided into tumors arising from either the pigmented and nonpigmented
epithelium.
Tumors of the Ciliary Body
1. Ciliary body melanoma Present in childhood
15% of uveal melanomas Clinical presentation: Ciliary body mass,
Anterior segment signs: raised IOP, subluxed lens and cataract
Dilated episcleral vessels (sentinel vessels) May be mistaken for retinoblastoma
Cataract and subluxed lens Histology: Flexner-Wintersteiner and
Uveitis Homer Wright rosettes can be seen

Glaucoma Glioneuroma (rare)
Posterior segment signs: Acquired:

Retinal detachment Fuchs adenoma (pseudoadenomatous
2. Tumors of ciliary epithelium hyperplasia) (rare)

Benign adenoma (rare)
Arising from pigmented epithelium:
Adenocarcinoma (rare)
Benign adenoma
Hyperplasia 3. Others
Arising from nonpigmented epithelium: Leiomyoma
Congenital Hemangioma
Medulloepithelioma:
Exam tips:
Remember only ciliary body melanoma and medulloepithelioma. The others
are extremely rare.
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TOPIC 10
POSTERIOR SEGMENT
TUMORS
Q Opening Question: What are the Possible Diagnoses of a Choroidal Mass?

Possible causes include tumors and non-tumor lesions.
Choroidal Mass
1. Tumors 2. Non-tumor lesions
Choroidal melanoma Choroidal and retinal detachment
Secondaries: AMD with disciform scar:
Bilateral, history of malignancy elsewhere Bilateral, drusens in both eyes, FFA diagnostic
Choroidal nevus: Exudative maculopathy:
Unilateral, at, drusens located within lesion FFA useful
Choroidal hemangioma: Posterior scleritis:
High internal reectivity on B-scan Anterior segment signs, systemic history, FFA
Congenital hypertrophy of RPE: useful
Flat, lacunae located within lesion
Melanocytoma of optic disc:
Jet black lesion at optic disc
Exam tips:
See also retinoblastoma (page 395).
Q What are the Causes of Choroidal Folds?
Possible causes include extrinsic compression, intramural lesions, ocular hypotony and idiopathic
choroidal folds.
Choroidal Folds
Mechanisms Etiology
Extrinsic compression Tumors (intraconal/extraconal)
Thyroid eye disease and pseudotumor
Retinal detachment surgery (scleral buckle)
(Continued )
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(Continued )
Mechanisms Etiology
Intramural Choroidal tumors
Uveal effusion syndrome
Posterior scleritis
Optic nerve disorders (optic neuritis, tumors)
Chorioretinal scars
Intraocular (ocular Post traumatic (rupture, cyclodialysis)
hypotony) Post surgical (trabeculectomy, wound leak)
Uveitis
Idiopathic Usually in hypermetropic males with good VA
Spontaneous resolution
Q What are the Clinical Features of Choroidal Melanoma?
Choroidal melanoma is the most common primary intraocular malignant tumor in adults.
They present with a variety of clinical features and are sometimes difcult to diagnose.
Clinical Features of Choroidal Melanoma
1. Risk factors Cataract

White race (rare in blacks and pigmented race) Glaucoma

Oculodermal melanosis (nevus of Ota) Systemic metastasis:
Neurobromatosis Liver (most common)
Nevus Lung (second most common)
2. Clinical features 3. Investigation

Age 5060 years Diagnosis
Choroidal melanoma (IDENTICAL to B-scan
FFA
pathological features, see below):
CT scan:
Pigmented or nonpigmented mass
Extraocular extension
Break through Bruchs membrane
MRI:
(mushroom-shaped)
Hyperintense to vitreous (T1 weighted lm)
Secondary exudative RD
Hypointense to vitreous (T2 weighted lm)
Orange pigment within lesion
Phosphorus-32 uptake (differentiate from
(lipofuscin)
Choroidal folds
hemangioma)
Intraocular ne needle biopsy
Anterior segment signs:
Uveitis (masquerade syndrome)
Notes
What are the features of the nevus that suggest malignant transformation?
Presence of lipofuscin within the nevus (instead of drusens)
Location near the optic disc
More than 2 mm thick
Associated with retinal complications (e.g. RD)
Notes
What are the mechanisms of glaucoma?
Direct invasion of angles
Release of pigments clogging the trabecular meshwork
Rubeosis at the angles
B1037_Ch-08.indd 432 12/24/2010 2:47:05 PM

Notes
What are the B-scan features of choroidal melanoma? B-scan shows a
collar button shaped mass with (ve key features).:
Highly reective anterior border of tumor
Acoustic hollowness (low internal reectivity on A-scan)
Choroidal excavation
Orbital shadowing
Extraocular extension
Notes
What are the FFA features of choroidal melanoma?
Hyperuorescence (window defect from RPE destruction) or
hypouorescence (masking from lipofuscin deposition)
Double circulation (this is usually not seen in secondaries)
Q What are the Pathological Features of Choroidal Melanoma?
The pathology of choroidal melanoma can be described in terms of gross pathology and histopathology.
Pathology of Choroidal Melanoma
1. Gross pathology Round nuclei
Pigmented or nonpigmented mass Prominent nucleolus
Polymorphism, varied pigmentation,
Breaks through Bruchs membrane (mushroom-
shaped) mitotic gures
Mixed:
Secondary exudative RD
Combination
Orange pigment within lesion (lipofuscin)
Modied Callender classication:
Choroidal folds
Spindle cell nevus = Spindle cell A
2. Histopathology (15-year mortality: < 5%)
Callender classication Spindle cell melanoma = Spindle cell B
Spindle A: (15-year mortality: 25%)
Cigar-shaped Epithelioid (15-year mortality: 75%)
Slender nuclei with basophilic line Mixed (15-year mortality: 50%)
No nucleolus ISDNA classication (inverse of standard
Spindle B: deviation of nucleoli area):
Oval-shaped, larger
Newer classication using pleomorphism of
Oval nuclei
cells as a guide
Prominent nucleolus
More objective quantication of risk
Syncytium
Epitheloid:
Large oval or round
Exam tips:
One of the most common pathology questions in the exams. The gross
pathology is IDENTICAL to the clinical features of the melanoma itself
(see above).
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Q What are the Treatment Options for Choroidal Melanoma?
The treatment is still being evaluated and should be individualized to the patient.
The factors to consider are.
The options include.
Treatment of Choroidal Melanoma
1. Factors to consider Tumor is situated away from the fovea
VA of involved eye and fellow eye No systemic metastasis is detected
Size, location and extent of tumor No subretinal uid
Presence of metastasis Plaque radiotherapy may be considered

General health and age of patient Close observation for growth before initiating
treatment can be considered, especially if
2. General principles
tumor is in less favorable location (macula,
Large tumor (larger than 15 mm diameter peripapillary) small tumor arm of COMS
and 5 mm thickness) showed slow progression without excess
Enucleation mortality with observation
Indicated especially if:
Medium-sized tumor (between 1015 mm
Eye has poor visual prognosis
diameter and 35 mm thickness)
Tumor has extended to the anterior
Most controversial
segment
Plaque radiotherapy vs enucleation (this is
No systemic metastasis is detected
the primary objective of COMS):
Patient is of good general health
Latest data suggests no difference in
Pre-enucleation radiotherapy affords no
survival
additional benet (COMS)
Plaque radiotherapy saves eye but does not
Bimodal incidence of death, initially at
preserve vision
2 years and later at 10 years
Small tumor (less than 10 mm diameter and 3. Other treatment options
3 mm thickness) Partial lamellar sclerectomy (for anterior tumors)
Laser photocoagulation Exenteration
Indicated especially if: Chemotherapy
Eye has good visual potential Radiotherapy
Exam tips:
Read the latest from COMS (Collaborative Ocular Melanoma Study) http://
www.jhu.edu/wctb/coms/in.
Notes
What is the Zimmerman hypothesis?
Early peak in mortality due to increased metastasis after enucleation in
the rst two years of treatment
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Q What are the Ocular Manifestations of Systemic Malignancies?
Systemic malignancies can affect the eye in one of four ways.

Systemic Malignancies and the Eye
1. Spread to the eye 3. PARANEOPLASTIC syndrome (Cancer Associated
Orbit (fairly common) Retinopathy (CAR))
Iris (rare) Usually associated with lung CA (small cell CA)
Choroid (most common): Rapid loss of VA
10 times more common than orbit
Normal looking fundus (there may be slight
Primary tumor:
narrowing of arterioles)
Breast CA in women (patient usually
Severely reduced ERG
provides previous history of breast CA)
Lung CA in men (patient usually have no
High serum levels of a particular 23 kD
history of lung CA) antibody (specic for a protein similar to
Clinical features: recovering)
Posterior pole (most common site) 4. Complications from TREATMENT
Bilateral and multiple Chemotherapy drugs
Poorly dened borders
Not elevated or pigmented
2. Spread to the CNS

Papilledema and other neuroophthalmic
features (page 262)
Q Tell me about the Combined Hamartoma of Retinal and RPE.
The combined hamartoma of retinal and the RPE has distinct clinical features.
Combined Hamartoma of Retinal and RPE
1. Clinical features 2. Associations
Males Differential diagnoses for retinoblastoma (page
Childhood 401)
Hamartoma Differential diagnoses for dragged disc (page
Mossy gray-green lesion 332)
Epiretinal membrane over hamartoma with Associated with neurobromatosis type II (page
subsequent brosis 273)
Vessel tortuosity
Exam tips:
There are three signicant ocular associations.
B1037_Ch-08.indd 435 12/24/2010 2:47:06 PM

TOPIC 11
IMMUNOSUPPRESSIVE
THERAPY, STEROIDS AND
ATROPINE
Q Opening Question No. 1: Tell me about the Types of Immunosuppressive Therapy.

Immunosuppressive therapy can be classied into four different groups.
Classication of Immunosuppressive Therapy

1. Hormones Drug of choice for thyroid eye disease
Steroids (see below) Pyrimidine analogues 5 uorouracil (5FU):
2. Alkylating agents See section on glaucoma (page 84)
Cyclophosphamide: Mycophenolic acid mycophenolate mofetil:

Nitrogen mustard derivative Metabolized to mycophenolic acid
Reacts with guanine forming DNA cross- Inhibitor of inosine monophosphate
linkages non cell cycle specic dehydrogenase (involved in guanine
Drug of choice for Wegeners granulomatosis, synthesis)
Moorens ulcer and ocular cicatricial 4. Natural products
pemphigoid T-cell specic immunosuppressive
3. Antimetabolites target DNA production and agent cyclosporin:
hence cell-cycle-specic Fungal product
Modulates T-cell function
Folate antagonists methotrexate:
Folate analogue Antibiotics mitomycin C (MMC):
Inhibits conversion of folate into See section on glaucoma (page 84)
tetrahydrofolate
Purine analogues azathioprine:
Activited to 6-mercaptopurine
Incorporated into DNA causing false protein
coding
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Q Opening Question No. 2: What are the Indications for Immunosuppressive

Therapy in Ophthalmology?
Immunosuppressive therapy is useful in ophthalmology in two broad categories.

Indications of Immunosuppressive Therapy
1. Inammatory/immune diseases Vogt-Koyanagi-Harada
Cornea Sympathetic ophthalmia
Peripheral ulcerative keratitis: Sarcoidosis
Moorens ulcer Orbit:
Rheumatoid arthritis, systemic lupus, Thyroid eye disease
Wegeners granulomatosis, polyarteritis Inammatory orbital disease (pseudotumor)
nodosa Retinitis/vasculitis
Ocular surface diseases (ocular cicatricial
Optic neuritis
pemphigoid, Stevens-Johnson syndrome)
2. Adjunctive to eye operations
Scleritis
High risk penetrating keratoplasty
Uveitis
Behcets disease
High risk glaucoma surgery
Pars planitis
Q What are the Complications of Immunosuppressive Therapy?
The complications can be divided into general complications and those specic to certain agents.
Complications of Immunosuppressive Agents

1. General Azathioprine and methotrexate group:
Bone marrow suppression Hepatotoxicity (azathioprine, methotrexate,
Increased infection cyclosporin)
Alopecia Gastrointestinal disturbance (azathioprine,
Carcinogenesis (skin, lymphoma) methotrexate)

Azoospermia (azathioprine)
2. Specic Rash/fever (azathioprine, methotrexate)
Cyclosporin and cyclophosphamide group:
Renal toxicity (cyclosporin)
Hemorrhagic cystitis (cyclophosphamide)
Hirsutism, gingivitis (cyclosporin)
Q Opening Question No. 3: What are the Indications for Steroid Therapy in Ophthalmology?
Steroid therapy is used in ophthalmology either via a topical, periocular or systemic route.
Topical therapy are used for two broad categories of diseases.
Indications of Steroid Therapy
1. Topical Ocular surface diseases (ocular
Inammatory/immune diseases: cicatricial pemphigoid, Stevens-
Conjunctival diseases: Johnson syndrome)
Atopic/allergic conjunctivitis Scleritis
Cornea: Uveitis
Marginal keratitis and other peripheral Glaucoma:
ulcerative keratitis Acute angle closure glaucoma
Specic keratitis (nummular keratitis, Adjunctive to eye operations

Thygesons keratitis, interstitial keratitis, Cataract and other intraocular surgeries
HSV stromal necrosis) (trabeculectomy, etc)
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Post-refractive surgery Optic neuritis

2. Periocular Anterior ischemic optic neuropathy
Cataract and other intraocular surgeries associated with giant cell arteritis
(trabeculectomy, etc) Adjunctive to eye operations:
High risk penetrating keratoplasty
Post-refractive surgery
High risk glaucoma surgery
3. Systemic
4. Intravitreal
Inammatory/immune diseases:
Given as 4 mg dose (0.1 ml of 40 mg/ml
Cornea:
Peripheral ulcerative keratitis
suspension)
Ocular surface diseases (ocular cicatricial
Inammatory/immune diseases:
pemphigoid, Stevens-Johnson syndrome) Uveitis:
Behcets disease
Graft failure
Pars planitis
Scleritis
Vogt-Koyanagi-Harada
Uveitis:
Sympathetic ophthalmia
Behcets disease
Sarcoidosis
Pars planitis
Vogt-Koyanagi-Harada Adjunctive to eye operations:

Sympathetic ophthalmia Highlight vitreous for anterior/posterior
Sarcoidosis vitrectomy
Orbit: Vascular diseases:
Thyroid eye disease CME
Inammatory orbital disease CNV
(pseudotumor) CSME
Retinitis/vasculitis Retinal vein occlusions
Optic nerve:
Exam tips:
Listen to the question, is it steroid therapy or topical steroid therapy?
The indications for systemic steroid therapy are IDENTICAL to that for
immunosuppressive therapy.
Q What are the Complications of Steroids?
The complications of steroid therapy can be divided into ocular and systemic complications.
Topical therapy is usually associated with ocular complications while systemic therapy can be associated
with both ocular and systemic complications.
Complications of Steroid Therapy
1. Ocular Suppression of hypothalamic pituitary
Cataract (posterior subcapsular type) adrenal axis (shock)
Raised IOP (see steroid responder, page 62) Hypertension, hirsutism, Hepatic (liver failure)
Exacerbation of infection (bacterial keratitis, Ischemic necrosis of femur and osteoporosis
fungal keratitis, HSV) Neutropenia and infection
2. Systemic Growth problems in children
Cardiac complications (arrythmias, heart failure) S for Psychosis
Ulcer (gastric ulcer)
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Exam tips:
Listen to the question, is it steroid therapy or topical steroid therapy?
There are three big ocular complications.
The mnemonic for systemic complications is CUSHINGS.
Notes
How do steroids cause cataract?
Binding of steroids to lens proteins
Disulphide bond formation
Increased glucose concentration in lens
Increased cation permeability
Decreased G6PD activity
Notes
How do steroids cause glaucoma?
Increased glycosaminoglycans in trabecular meshwork
Inhibition of phagocytic activity of meshwork cells
Inhibition of prostaglandins
Q When is Atropine Needed in Ophthalmology?
Atropine is a cholinergic receptor blocker, specically a muscarinic antagonist.

It is used topically for diagnosis and treatment, as well as systemically.
Atropine Indications
1. Diagnostic
Mydriasis for vitreoretinal surgery: Cataract (posterior subcapsular type):
Prolonged duration, onset 3040 min, duration In situations when surgery is contraindicated
1014D or patient refused surgery
Cycloplegic refraction: Amblyopia:
Indicated for poor response to cyclopentolate Penalization technique (give atropine to the
or in cases of excessive accommodation good eye)
2. Therapeutic 3. Systemic use
Uveitis Inhibit oculo-cardiac reex during orbital/squint
Glaucoma: surgery
Inammatory glaucoma Standby for tensilon test in myasthenia gravis
Neovascular glaucoma (page 236)
Malignant glaucoma
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Notes
Why use atropine for uveitis?
Decrease pain and ciliary spasm
Prevent posterior synechiae
Stabilize blood-aqueous barrier
Q What are the Complications of Atropine Use?
Atropine is a cholinergic receptor blocker, specically a muscarinic antagonist.

It has both local and systemic complications.
Complications of Atropine
1. Local Fever (hot as a hare)
Transient stinging effect Headache, dysarthria, ataxia, hallucination,
Conjunctival irritation, hyperemia, follicular amnesia (mad as a hatter)
conjunctivitis Bladder distension, decreased gastrointestinal
Acute ACG mobility (bloated as a barrow)
Visual blurring from mydriasis and cycloplegia Tachycardia, dysarrhythmia
Amblyopia in children Hypotension, respiratory depression, coma and
2. Systemic death
Flushing (red as a beetroot)
Dryness (dry as a bone)
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Section 9
Squints AND
Pediatric Eye
Diseases
B1037_Ch-09.indd 443 12/22/2010 8:23:25 PM

TOPIC 1
ASSESSMENT OF
STRABISMUS
Q Opening Question: What Clinical Tests are Used in the Assessment of Strabismus?
Assessment of Strabismus
1. Light reection tests: 3. Dissimilar image tests:
Hirshbergs test The Maddox wing
Krimskys test The Maddox rod
Bruckners test The Hess test/Lees screen
2. Cover tests: 4. Binocular single vision tests:
Cover and uncover test Base out prism test
Alternate cover test Worths four dot test
Simultaneous prism cover test Bagolini striated glasses
Alternate prism cover test Synoptophore
Stereopsis tests (Titmus test, TNO random dot
tests)
Exam tips:
Alternate questions are How do you perform the cover and uncover tests?
and What is the Maddox rod?
Notes
Common scenarios in orthoptics examination:
1. Intermittent XT
2. Esotropia
3. Duanes syndrome
4. Fourth nerve palsy
5. Browns syndrome
6. Monocular elevation decit
B1037_Ch-09.indd 445 12/22/2010 8:23:27 PM

Q What are the Light Reection Tests?
Light Reection Tests

1. Hirshbergs test: Eccentric xation
Detects gross heterotropias Large (positive) angle kappa congenital or
Based on Purkinje Sanson image no. 1 acquired (ROP)
Look at symmetry of light reex Nonseeing eye
Normal reex: 2. Krimskys test:
Just nasal to center of pupil Place prism in front of xating eye until light
Abnormal reex: reex is symmetrical
Border of pupil (15 or 30 prism D) 3. Bruckners test:
In between border and limbus (30 or Use direct ophthalmolscope
60 prism D) Look at symmetry of red reex
Limbus (45 or 90 prism D)
Brighter reex comes from deviated eye
What are the possible differential diagnoses of
an abnormal Hirshbergs test?
Strabismus (tropia)
Exam tips:
Describe each test as simply as possible.
Q What are the Cover Tests?
Cover Tests
1. Cover-uncover test: Look at uncovered eye for movement
Cover component: (movement indicates phoria in that eye)
Detects heterotropias 3. Simultaneous prism cover test:
Cover straight eye
Measures heterotropias
Look at uncovered deviated eye (movement
Simultaneous cover of one eye and placing
indicates tropia)
prism over the other until no movement
Uncover component:
Detects heterophorias 4. Alternate prism cover test:
Uncover straight eye Measures total deviation (heterotropias
Look at uncovered eye for deviation and and phorias)
rexation (movement indicates phoria in this Prism over deviated eye and alternate cover
eye) each eye until no movement
2. Alternate cover-uncover test:
Detects heterophorias
Alternate cover and uncover both eyes
Q What are the Dissimilar Image Tests?
Dissimilar Image Tests

1. The Maddox wing: Left eye sees vertical and horizontal row of
Measures heterophorias numbers
Dissociates two eyes for near xation: Patient asked which number arrow pointing at
Right eye sees white vertical arrow and red 2. The Maddox rod:
horizontal arrow Measures heterophorias
B1037_Ch-09.indd 446 12/22/2010 8:23:27 PM

Dissociates two eyes for distance xation Larger eld is from normal eye (outward
Consists of series of fused high power cylinder displacement indicates overaction in that
red rods direction)
Coverts white spot of light into red line Equal size eld indicates no deviation or
perpendicular to axis of rods equal deviation

Narrow eld indicates mechanical restriction
Rods placed in front of deviated eye and patient
of movements in opposing directions (blow
asked to locate position of red line in relation to
out fracture)
white spot of light: Sloping eld indicates A or V pattern (NOT
If red line is temporal to light, indicates
torsion)
esophoria (EP)
When do you perform a Hess test?
If red line is nasal to light, indicates
To differentiate ET from paretic squint (e.g. VI
exophoria (XP)
CN palsy)
To estimate degree of squint, place prisms until Paretic squints
red line is at center of white spot of light Thyroid eye disease
3. Hess test/Lees screen: Myasthenia gravis (tensilon test)
Principle: Herrings law of equal and Blow out fracture
simultaneous innervation of yoke muscle When is the Hess test contraindicated?
Dissociates two eyes for distance xation Patients who have monocular suppression
Hess test: Dissociates two eyes with red and Patients who do not have normal retinal
green lters correspondence

Lees screen: Dissociates two eyes with mirror
Interpretation of Hess chart/Lees screen:
Smaller eld is from abnormal eye (eye with
limited movement)
Q What are the Tests for Binocular Single Vision (BSV)?
BSV Tests
1. The base out prism test: If 3 lights are seen, indicates right
Place prism base out over eye suppression
This displaces retinal image and initiates eye If 5 lights are seen, indicates diplopia
movement in direction of apex (uncompensated ET/XT)
Examiner looks for corrective movement of eye 3. Bagolini striated glasses:
No movement indicates scotoma/suppression in Test of BSV
that eye Consists of glasses with ne striations orientated
Four prism D base out prism will not induce at 45 to each other
corrective movement in eye with microtropia Converts point of light into a line perpendicular
2. The Worths four dot test: to striations (like Maddox rod) but principle is
Test of BSV based on interference and diffraction of light
Dissociates two eyes for distance xation (not refraction as in Maddox rod)
Consists of box with 4 dots (1 red, 1 white and Patient wears glasses and sees point of light
2 green). Interpretation:
Patient wears glasses with red lens in right eye If lines cross at center, indicates normal
and green lens in left fusion

If lines cross at center in presence of squint,
Interpretation:
indicates ARC
If 4 lights are seen, indicates normal fusion
If one of the lines is missing, indicates left or
If 4 lights are seen in presence of manifest
right suppression
squint, indicates anomalous retinal
If lines do not cross at center (point of light),
correspondence (ARC)
indicates diplopia
If 2 lights are seen, indicates left suppression
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4. The synoptophore: Instrument: two cylindrical tubes with pictures

Test of BSV are inserted at end of each tube
Dissociates two eyes for both near and distance
xation
Notes
What are the uses of the synoptophore?
Determine 3 grades of BSV
Measure objective and subjective angle of deviation
Measure angle kappa
Measure primary and secondary deviation
Assessment of retinal correspondence
Measurement of IPD
Assessment of anisokonia
Therapeutic use (treatment of suppression, ARC, accommodative ET, intermittent

tropias and phorias)
Q What are the Tests for Stereopsis?
Stereopsis tests can be divided into.

Stereopsis
1. True 3-dimensional tests: Consists of 7 plates with various obvious and
Frisby plates: hidden shapes (squares, dots)
Stereopsis test (600 15 seconds) Needs red green glasses
Consists of 3 clear plastic plates consisting of No monocular clues (better than Titmus)
4 squares with hidden circle in 1 of them Lang test:
Plates are of varying thickness and tests can Stereopsis test (1200 200 seconds)
be varied by distance Consists of plates with various hidden objects
Patient asked to pick square with circle in it (moon, sun)
No need for glasses (built-in cylindrical
2. Dissociated 2-dimensional tests:
elements)
Titmus test:
No monocular clues
Stereopsis test (3000 40 seconds)
Mentor BVAT:
Consists of three components: Fly, circle,
Distance stereopsis test
animal
Needs polaroid glasses
TNO random dot:
Stereopsis test (1900 15 seconds)
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TOPIC 2
BINOCULAR SINGLE
VISION
Q Opening Question: Tell me about Fixation.

Fixation is a monocular visual phenomenon.
In which the image of an object is focused on the fovea.
Fixation
1. Axes in xation: Eccentric xation:
Optical axis (anatomical axis) = line passing A monocular phenomenon, whereby an eye
through center of cornea that bisects globe into xates upon a target with a nonfoveal area
two equal halves (OR = line passing through Develops if there is an early-onset macular
center of cornea and lens; OR = line that joins pathology and is also a very rare
complication of strabismus
all Purkinje images)
Compare with anomalous retinal
Pupillary axis = line perpendicular to corneal
correspondence (see below)
center, which passes center of pupil
3. Tests of xation:
Visual axis = line from object of regard to fovea
Gross testing:
Angle kappa = between VISUAL and PUPILLARY
Occlude one eye and test xation pattern of
axis, subtended at anterior nodal point
the other with target (e.g. cover-uncover test)
Positive if Hirschberg light reex is displaced
Visioscopy:
nasally
Performed with a direct ophthalmoscope,
Negative if displaced temporally
where examiner observes retinal position of
Normal: Up to 5 in adults/10 in infants
projected target when viewed by xating
Angle lambda = between VISUAL and
patient
PUPILLARY axes, but subtended at pupil entrance
Haidinger brushes:
Angle alpha = between OPTICAL and Entoptic phenomenon appreciated only by a
PUPILLARY axes macular area with its center located at fovea
2. Abnormalities of xation: Patient sees rotating Maltese cross when
If development in xation is disturbed, stimulated with a rotating plane-polarized
two possible consequences blue light. If eccentric xation present,
Nystagmus: patient will be unable to localize hub of
Appears at 34 months cross correctly
Exam tips:
BSV is an extremely difcult subject.
Denitions for BSV, fusion, retinal correspondence, the horoptor, Panums
space and stereopsis must be committed to memory. Use keywords for each.
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Q What is Binocular Single Vision?
Binocular single vision (BSV) is a binocular acquired phenomenon.

Whereby separate and similar images seen by two eyes are perceived as one.
The prerequisites of BSV are.
There are three grades of BSV.
BSV
1. Prerequisites of BSV: Ability of cortex to integrate images (sensory
Clear visual axis in both eyes, with normal fusion)
function of visual pathways 2. Grades of BSV (Worths three ascending levels):
Straight eyes within 8 prism D horizontally Simultaneous perception
(motor fusion) Fusion
Stereopsis
Q What is Simultaneous Perception?
Simultaneous perception is the appreciation of two separate and dissimilar images being projected to the
same position in space.
Occurs in two sets of circumstances.
Simultaneous Perception
1. Appreciation of dissimilar images (rst grade of Involves fovea in one eye and peri-foveal area
BSV): in the other (synoptophore bird in cage)
Two dissimilar images appear to be projected to 2. Appreciation of similar images too disparate to
same area fuse leading to diplopia
Q What is the Cyclopean Eye?
This is a hypothetical single eye situated between the two eyes.

An image which falls on the fovea in the two eyes is perceived to come from a straight ahead position.
This direction is the subjective visual direction from the cyclopean eye.
Q What is Fusion?
Fusion is a binocular phenomenon where separate images are perceived as one due to stimulation of
corresponding retinal areas in the two eyes.
This is associated with a 2-dimensional localization of object in space.
There are two types of fusion: Motor and sensory.
Fusion
1. Motor fusion: stereopsis. Up to 2.5 degree of vergence error
A vergence movement designed to allow can be tolerated
objects to stimulate corresponding retinal areas 2. Sensory fusion:
(reduce horizontal, vertical or torsional disparity The appreciation of two separate images located
of the retinal image) on the retina as a single unied percept
Strength of motor fusion = fusional amplitude Strength of sensory fusion = xation disparity
(in prism D) Similar foveal images of up to 14 minutes of
Experiments have shown that vergence ARC are fusible
precision is not necessary for fusion and
Distant: Convergence 15 Divergence 6 Vertical 2.5

Near: Convergence 25 Divergence 15 Vertical 2.5
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Q What is Retinal Correspondence?
Retinal correspondence is a phenomenon in which retinal areas.

Retinal Correspondence
1. Retinal correspondence When these retinal areas bear identical
Retinal areas in two eyes share a common relationship with fovea
visual direction and therefore project to same 3. Anomalous retinal correspondence
position in space and are connected to When they do not share same relationship with
approximately the same area in visual cortex fovea
2. Normal retinal correspondence
Q What is the Horoptor?
Horoptor is an imaginary surface in space.

All points of which will stimulate corresponding retinal points.
All points will therefore be projected to same position in space.
Horoptor
1. Each xating point determines a specic 3. Horoptors surface is a torus, derived from
horoptor. experiments
2. All points located just off horopter will stimulate 4. Vieth-Muller circle is an imaginary circle derived
non-corresponding retinal points, but images can from mathematical formulae, all points which
still be perceived singly as long as they are will stimulate corresponding retinal points, with
located within Panums space circle passing through optical centers of each eye
Q What is Panums Space?
Panums space is an imaginary volume in space surrounding the horoptor.

Within which objects will be seen singly, although they may stimulate non-corresponding retinal areas.
Panums Space
1. Points falling outside of Panums space are not To prevent bothersome peripheral diplopia
fusible and will lead to physiological diplopia, To help facilitate cyclofusion
which is then physiologically suppressed in 3. Not a xed space; it widens if the stimulus is:
nondominant eye Larger
Fuzzier
2. Panums space widens out toward periphery:
Slower moving
To match the increasing coarseness of
peripheral vision
Q What is Stereopsis?
Stereopsis is the binocular perception of depth.

Occurs when separate but slightly dissimilar objects are seen by two eyes as one.
Stereopsis is caused by horizontal retinal image disparity.
In contrast to fusion, there is 3-dimensional localization of the object in space.
Stereopsis
1. Prerequisites
Needs slight horizontal disparity (does not Those pointing RIGHT on the horopter are
occur for vertical/torsional disparity) not seen stereoscopically, as they project to
Images must be fusible (i.e. within Panums corresponding retinal point with no
space), BUT not all fusible images give stereo: horizontal disparity present!
B1037_Ch-09.indd 451 12/22/2010 8:23:28 PM

Retinal disparity must be large enough to

Overlay (nearer object will cover further one)
prevent simple fusion, but not great enough Aerial perspective (distant objects appear more
for diplopia to occur indistinct and less color-saturated)
Not possible beyond 700 m (insufcient image Light shading
disparity) Geometric perspective (parallel lines converge
Cortically, must be able to have: the further they are)
Binocular correlation (ability to determine
Relative velocity
that two similar images come from same
Motion parallax (when observers head is
object)
moved, closer object moves smaller amount in
Disparity detection between these correlated
images an opposite direction, while further objects
move a larger amount in same direction)
2. Monocular clues to stereopsis
Apparent size (larger of two identical objects is
nearer)
Q What is Stereoacuity?
Measure of the threshold of horizontal disparity required to perceive stereopsis.

Stereoacuity
1. Smallest binocular disparity or parallax that can 4. Normal values:
be detected Centrally: 2040 sec of ARC
2. Dependent on three parameters: Interpupillary Peripherally: 200 sec of ARC
Maximal at about 0.25 off dead-center in the
distance, object separation and object distance
foveola
3. Stereoacuity increases progressively as horoptor Minimal beyond 15 eccentricity
is approached, but reaches 0 at horoptor (where
there is zero retinal image disparity)
Q What are the Differences between Fusion and Stereopsis?
Both fusion and stereopsis are components of BSV.

Comparison between Fusion and Stereopsis
Fusion Stereopsis
Image disparity Eliminates disparity of retinal images. Based on existence of retinal image
The less the disparity, the more ideal disparity
the fusion
Motor component Yes No
stimuli Horizontal, vertical and torsional visual Only horizontal disparity will elicit
stimuli elicit a fusional response stereopsis
Localization of object In 2-dimensional space In 3-dimensional space
Range All ranges of distances Less effective as distance increases
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Q What are the Consequences of an Interruption of BSV? How do We Compensate for

an Interruption of BSV?
BSV Abnormalities
1. Compensatory mechanisms Blind spot syndrome
Physical adaptation: 2. BSV abnormalities
Abnormal head posture
Absent BSV (e.g. congenital ET)
Conscious closure of one eye
Impaired BSV (e.g. monoxation syndrome)
Sensory adaptation:
Diplopia
Suppression (leads to amblyopia)
Confusion
Anomalous retinal correspondence
Monoxation syndrome (initially a
compensatory mechanism, later on becoming
a BSV abnormality)
Q Tell me about Diplopia.
Diplopia is an abnormality of BSV.

Occurs when there is an acquired misalignment of visual axis (squint).
Single object stimulates two non-corresponding retinal points.
Object is therefore perceived to come from two different locations in subjective visual space.
Diplopia
1. Does not occur in congenital squints 4. Classied as:
2. Usually there is stimulation of fovea in one eye Crossed diplopia (XT) (note: cross = X)
and a nonfoveal area in the other eye Uncrossed diplopia (ET)
One of two areas must project to a point outside
of Panums space:
3. Compensatory mechanism is peripheral
suppression (nonfoveal area)
Q Tell me about Confusion.
Confusion is an abnormality of BSV.

Occurs when there is an acquired misalignment of visual axis (squint).
Two objects stimulate corresponding retinal points.
Two objects are therefore perceived to come from single location in subjective visual space.
Confusion
1. Less common than diplopia 3. Usually there is stimulation of both foveas by
2. Does not occur in congenital squints different objects in different locations
4. Compensatory mechanism is central suppression
Exam tips:
Confusion = corresponding retinal points = central suppression!
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Q Tell me about Suppression.
Suppression is a compensatory mechanism when there is an interruption of BSV.

Visual sensation is prevented from reaching consciousness.
Occurs when there is a misalignment of visual axis (squint).
Adaptation to prevent diplopia and confusion.
Suppression
1. Occurs mainly in children (congenital squints or Monocular (higher risk of amblyopia) vs
early acquired squints) alternating
2. Physiological suppression (prevents physiological Facultative (only when manifest squint is
present) vs obligatory (all the time, higher risk
diplopia) vs pathological suppression (squints)
of amblyopia)
3. Classied as:
Central (prevent confusion) vs peripheral
(prevent diplopia)
Q Tell me about Monoxation Syndrome (Microtropia).
Monoxation syndrome is an abnormality of BSV.

Occurs when there is a small angle squint.
The classical features include.
Monoxation Syndrome
1. Scenarios Decreased stereopsis
Primary (small angle ET most common squint, May have ARC
usually less than 8 prism D) May have central or eccentric xation
Secondary (treatment of ET with glasses or 4. Alternate prism cover test measurement will
surgery, anisometropia, macular lesions)
EXCEED simultaneous prism cover test
2. Differential diagnosis of unilateral decrease in VA
5. Treatment
when no obvious squint is present Refractive correction of anisometropia
3. Variable features Occlusion to treat amblyopia
Amblyopia is common Poor prognosis for restoring binocular single
Central scotoma with peripheral fusion vision
capability
Exam tips:
Fairly rare syndrome, but fairly common exam question.
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TOPIC 3
AMBLYOPIA
Q Opening Question: What is Amblyopia?

Amblyopia is a unilateral or bilateral decrease in visual acuity.
Caused by form vision deprivation or abnormal binocular interaction.
No organic causes can be detected by the examination of the eye.
In appropriate cases, is reversible by therapeutic measures.
Classication
1. Strabismic amblyopia Anisomyopia difference in myopia of

Most likely with constant tropias >3D
Uncommon in intermittent XT Meridonal:
2. Amblyopia related to refractive errors Due to uncorrected astigmatism of > 1.5 D
Ametropic: 3. Stimulus-deprivation amblyopia
Due to either bilateral hyperopia (> 45 D) or Complete ptosis, corneal opacities, congenital
bilateral myopia (> 67 D) cataracts, other media opacities
More common in bilateral hyperopia
Iatrogenic origin (occlusion amblyopia)
In bilateral amblyopia, high myopia less
likely as near objects will still be in focus due
to accommodation
Anisometropic:
Due to unequal refractive error between the
two eyes:
Anisohyperopia difference in hyperopia
of > 1.5 D
Q What are the Pathophysiological Changes in Amblyopia seen in Animal or Experimental Models?
Pathophysiological Changes of Amblyopia

1. Retina: 2. Lateral geniculate nucleus reduction in number
Reduction in spatial resolving powers of retinal of cells of all six layers
cells 3. Visual cortex reduction in number of cortical
Increased lateral inhibition between retinal
cells
cone cells
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Q What are the Clinical Features of Amblyopia?
Clinical Features of Amblyopia

1. Decreased VA commonly dened as loss of VA of VA better on grating tests (FPL)
two or more lines on Snellen chart 3. Normal ocular exam and no RAPD
VA paradoxically improves with neutral density 4. Eccentric xation
lter
5. Decreased contrast sensitivity and decreased
2. Crowding phenomenon
brightness perception
Represents an abnormality of contour
6. Binocular suppression of amblyopic eye
interaction between point of xation and
adjacent objects 7. Increased perception and reaction times
VA better for single optotypes than multiple
optotypes (Sheridan Gardiner)
Q How do You Manage a 3-year-old Child with Amblyopia?
The management of amblyopia will depend on the age of patient, cause of amblyopia and severity of
amblyopia.
First, we need to exclude
Management of Amblyopia
1. Exclude other organic causes of poor vision 4. Penalization
Refractive errors, cataract, tumors Usually reserved for patching failure or
2. Remove obstacles to clear vision noncompliance with patching
Refractive correction, cataract surgery Pharmacologic:
1% atropine placed in good eye to blur eye
3. Occlusion therapy (gold standard)
for near vision
Amount of occlusion depends on age of patient,
Optical:
cause of amblyopia and severity of amblyopia
Degrades image in better eye to a degree
Best achieved with adhesive patches
such that amblyopic eye has a competitive
Practical guidelines: advantage at a given xation distance
Patching should be started as soon as
Undercorrecting the refractive error in better
amblyopia is detected eye
For part-time occlusion, duration of patching
usually depends on age of child: 1 hour/day 5. Others CAM visual stimulator, pleoptics
for each year of age (unproven alternatives to patching)
Full-time occlusion should not exceed 1 6. Prevention
week per year of age Education and awareness of primary care
Patching should be continued till VA reaches
physician
and maintains a plateau for 36 months
Vision screening programs essential in any
From full-time patching, decrease to half-time
community
patching for a few months, then to several
hours per day Red reex of every baby should be checked at
If no progress is made for 3 consecutive birth
months, patching may be considered a failure Additional Management Principles
Regular follow-up to ensure that vision
1. Strabismic amblyopia
remains stable
Maintenance patching may be required until
Occlusion therapy should be instituted prior to
9 years of age when visual system is assumed surgery:
to have matured Fixation behavior will be harder to determine
once eyes are surgically aligned
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Optimal acuity may maximize chances of Part-time occlusion preferable if binocular

restoring binocular vision interaction present, amblyopia is mild and child
Parent motivation toward patching might be is in school
increased by visual reminder of strabismus
3. Stimulus deprivation amblyopia
2. Amblyopia related to refractive errors Remove barriers to vision preferably within rst
Correct refractive error rst before occlusion 6 weeks of life
therapy
Notes
What if there is no response after 3 months of patching? Consider
possible causes:
Wrong diagnosis
Noncompliance
Uncorrected refractive error
Failure to prescribe sufcient treatment
Irreversible amblyopia
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TOPIC 4
ESOTROPIA
Q Opening Question: What are the Causes of Esotropia?

Esotropia is a convergent misalignment of eyes.
They can be divided according to age of onset.
Causes of ET
1. Infantile ET (< 6 months) Consecutive ET
Essential or congenital ET Divergence insufciency
Early accommodative ET Stress-induced ET
Duanes syndrome Type 1 Cyclic ET
Mobius syndrome Incomitant ET:
VI CN palsy VI CN palsy
Nystagmus blockage syndrome Thyroid eye disease

Medial wall fracture
2. Acquired ET (> 6 months)
Mobius syndrome
Comitant ET:
Accommodative ET
Sensory ET (disruption of BSV in children
usually before 2 years of age e.g. congenital
cataract)
Q Tell me about Essential/Congenital Esotropia.
Essential or congenital ET is a common convergent squint.

Essential/Congenital ET
1. Clinical features Poor potential for BSV
Presents at 6 months of birth 2. Management
Family history common Correct amblyopia
Characteristic of ET: Timing of surgery: 6 months to 2 years (usually
Large angle (> 30 prism D) before 1st birthday):
Stable Angle of deviation stable over 2 visits, 34
Angle at distance = near weeks apart; no amblyopia; at least 2
Normal refractive error (therefore not cycloplegic refractions 34 weeks apart
accommodative ET) Why not before 6 months?
Alternating xation in primary position but cross There is a chance of spontaneous recovery
xation in side-gaze before 6 months and angle is also smaller

Need to exclude VI CN palsy (cover one eye, after 6 months
elicit Dolls reex) Why not after 2 years then?
Loses stereopsis after 2 years
Latent nystagmus, dissociated vertical deviation,
IO overaction and asymmetrical OKN response
may be present
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Type of surgery Watch for:

Bilateral MR recession with IO overaction Accommodative ET
correction Undercorrection (need further LR
Aim for 10 prism D of residual ET (allows resection)
good peripheral fusion although central BSV IO overaction and DVD
is still impaired)
Subsequent management
Manage amblyopia (develops in 40% of
congenital ET after surgery)
Q What are Accommodative Esotropias?
Accommodative esotropias are common types of convergent squints.

Due to an overaction of the accommodative reex.
There are three classical types.
Accommodative ET
1. Classication 3. Management
Fully accommodative Correct refractive error (hypermetropia):
Partially accommodative Plus bifocal component for nonrefractive
Fully and partially accommodative with high accommodative ET
AC/A ratio Miotic therapy (ecothiopate or pilocarpine):
Temporary measure for children who are
noncompliant with glasses
Presents at 2.5 years
Induces peripheral accommodation so that
5 cardinal features common to all 3 types: less accommodative effort is needed by
Usually intermittent in onset early on then
patient
becomes constant Side effects: Miosis, ciliary spasm, iris cysts,
Family history is common
cataract, RD
May be precipitated by trauma or illness
Correct amblyopia
Amblyopia is common
Surgery:
Diplopia is uncommon
If ET is not fully corrected with
Fully accommodative ET:
spectacles
ET fully corrected with glasses
Determine the amount of surgical
Deviation same near and distance
correction
Normal AC/A ratio
Standard:
Surgery not necessary. Reassure patient that
Target angle determined by residual
the hypermetropia and ET will lessen as they
distance ET that is not corrected with
grow older
spectacles
Partially accommodative: Undercorrection rate high: 2530%
ET not fully corrected with glasses
Augmented:
Deviation same near and distance
Target angle determined by averaging
Normal AC/A ratio
near deviation with correction and near
Surgery as soon as possible to correct the
deviation without correction
non-accommodative component. The patient 7% overcorrection rate for distance
will still require glasses post-surgery Prism adaptation:
Fully and partially accommodative ET with high Target angle determined using prisms
AC/A ratio: Patient prescribed base-out prism for
Deviation greater for near compared to residual ET after being prescribed full
distance hypermetropic correction. Prisms are
High AC/A ratio increased at 2 weekly intervals until ET
Patient will require bifocals (in addition to has stabilized
surgery for partially accommodative ET)
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Target angle determined by full prism- Recess-resect if amblyopia in one eye

adapted angle Other considerations:
85% success rate, but time-consuming Correct IO overaction
and costly Correct V or A pattern
Type of surgery:
Bilateral MR recession
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TOPIC 5
EXOTROPIA
Q Opening Question: What are the Causes of Exotropia?

Exotropias are divergent misalignment of eyes.
The commonest cause is intermittent XT.
Other causes include.
Causes of Exotropia
1. Congenital Sensory XT (disruption of BSV in children
Congenital XT: Present at birth, large and usually after 2 years of age)
constant angle, frequently associated with Convergence insufciency
neurological anomalies Incomitant XT:

Duanes syndrome Type 2 III CN palsy
Myasthenia gravis
2. Acquired
Thyroid eye disease
Comitant XT: INO
Intermittent XT
Consecutive XT (after correction for ET)
Q Tell me about Intermittent Exotropias.
Intermittent XT is a common divergent squint.

It can be divided into 3 types based on severity of XT for near vs far.
And into 3 phases.
Intermittent XT
1. Classication: Phase 3 (XT at distance and near)
Convergence insufciency (worse for near, 3. Clinical features:
needs MR resection or recess-resect) Age of onset: 2 years
Divergence excess (worse for distance, needs Precipitated by illness, bright light, day-
LR recession): dreaming, fatigue
Simulated excess (accommodative fusion
Goes through 3 phases
controls deviation at near)
Temporal retinal hemisuppression when eyes
True excess (diagnosed by adding plus 3D
are deviated
lens at near to control for accommodation, or
occluding one eye to relax the Amblyopia not common
accommodation) ARC and eccentric xation may be present
Basic (near and distance same or within 4. Management:
10 , needs LR recession) Correct refractive errors (myopia)
2. Phases: Correct amblyopia
Phase 1 (intermittent XP at distance) Orthoptic treatment:
Phase 2 (XT at distance, XP at near) Fusional exercise (pencil pushups, base-out
prism)
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Diplopia awareness Type of surgery:

Surgery: Bilateral LR recession
Indications (4 classic indications) If one eye amblyopic, recess LR and resect
Increase angle of XT MR of amblyopic eye

Increase frequency of breakdown (i.e. If angle of deviation is large, 3 muscle
progressing from Phase 1 to 2) surgery (bilateral LR recession + MR

Decreasing stereopsis resection)
Abnormal head posture If there is a V-pattern XT:
Determine the amount of surgical correction: Bilateral LR recession and IO weakening
Basic and pseudo-divergence excess: Bilateral LR recession and upward
Correct angle of deviation for distance transposition of LR (if there is no

Divergence excess: Correct (angle of signicant IO overaction)
deviation for distance + angle of deviation
for near)
Notes
What are the causes of abnormal head posture?
Ocular:
Strabismus
Nystagmus
Ptosis
Refractive error
Non-ocular:
Torticollis
Unilateral hearing loss
Habitual
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TOPIC 6
VERTICAL SQUINTS
AND OTHER MOTILITY
SYNDROMES
Q Opening Question: What are the Types of Vertical Squints?

Vertical Squints
1. SO and IO muscles Double elevator palsy
SO palsy Dissociated vertical deviation (DVD)
SO overaction A and V patterns
IO palsy 3. Others (III CN palsy, thyroid eye disease, blowout
IO overaction fracture)
2. Multiple muscles
Congenital brosis syndrome
Q Tell me about Inferior Oblique Overaction.
IO overaction is a common vertical squint.

50% of patients with essential or congenital ET have IO overaction.
Inferior Oblique Overaction
1. Introduction 3. Surgery
Bilateral, but may be asymmetrical Grade +1:
Clinical scenarios: IO recession 810 mm
With horizontal squints Grade +2:
Paresis of one or both SO IO myomectomy
Primary (uncommon) IO myotomy at insertion
Signicance of IO overaction: Grade +3:
Affects cosmesis Extirpation of IO muscle
Disruption of BSV Grade +4:
Contributes to large angle ET Denervation
2. Clinical features Anteriorization of IO tendon:
Marshall Parks point: 3 mm posterior to IR
V pattern difference of > 15 prism D is
considered signicant insertion + 1 mm lateral
Equivalent to 1215 mm of IO recession
Upshoot of eye in adduction
Can correct for DVD as well
Associated with SO underaction
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Notes
How do you differentiate IO overaction from DVD? In IO overaction:
Elevation of eye in adduction only (in DVD, in primary position and
abduction as well)
Hypotropia of fellow eye (in DVD, only hypertropia of affected eye)
Base-up prism over fellow eye will neutralize hypotropia (in DVD, only
base down prism over affected eye will correct hypotropia)
Q How do You Locate the IO Muscle During Surgery?
Localization of IO During Surgery

Isolate LR and IR Tubular/worm-like structure
IO is a pink tendon within white Tenons Pull IO and feel tug at point of origin at orbital
capsule rim
Notes
Complications of IO surgery:
1. Injury to the macula
2. Injury to the posterior ciliary artery
3. Breach of the posterior tenon space fat prolapse, adhesions
4. Injury to the vortex veins
Q What are the Advantages of an IO Myomectomy Compared to IO Recession?
Myomectomy Recession
Advantages Easy visualization and Graded
technique Reversible potentially
Skilled assistant not needed Anteriorization for DVD
Consistent result
Lower risk of undercorrection
Disadvantages Dilate pupils More difcult
Not reversible Need skilled assistant
Cannot be graded (all or none) Results less consistent
No benet for DVD
Q What is the Duanes Syndrome?
Duanes syndrome is an ocular motility disorder.

The main clinical feature is retraction of the globe on attempted adduction.
It can be classied into 3 types.
Duanes Syndrome
1. Classication Limitation in abduction
Type 1: Can present as an ET
60%
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Type 2: Anisocoria
15% Persistent hyaloid artery
Limitation in adduction Myelinated nerve bers
Can present as an XT Nystagmus
Type 3: Lid and iris colobomas
25% Optic disc and foveal hypoplasia
Limitation in both adduction and abduction Systemic associations:
Usually orthophoric Agenesis of genitourinary system
2. Clinical features: Bone (vertebral column abnormalities,
Females more common syndactyly, polydactyly)

CNS (epilepsy)
Left eye in 60%, bilateral in 20%
Deafness (sensory neural deafness is the most
Retraction of globe on adduction (sine qua non)
common association; 16% of all Duanes
Co-contraction of MR and LR
syndrome)
Associated with narrowing of palpebral ssure
Dermatological (caf au lait spot)
What is the underlying pathogenesis?
Wildervank syndrome (Duanes syndrome,
Pontine dysgenesis with III CN innervating
both MR and LR. deafness and Klippel-Fiel anomaly of spine)
Upshoot or downshoot (lease phenomenon, do 4. Management:
not mistake for IO overaction!) Correct amblyopia
3. Ocular and systemic associations: Indications for surgery:
Abnormal head posture
Ocular associations (8%):
Unacceptable upshoot or downshoot
Ptosis
Squint in primary position
Epibulbar dermoids (associated Goldenhar
syndrome) Liberal MR recession (may add LR recession)
Exam tips:
Systemic associations can be remember as ABCD.
Q What is Browns Syndrome?
Browns syndrome is an ocular motility disorder.

The main problem is pathology of the SO tendon.
It can be either congenital or acquired.
Browns Syndrome
1. Classication Less severe defective elevation in midline
Congenital: Normal elevation in abduction
Bilateral in 10% Vertical gaze triad:
Pathology: Short SO tendon, tight trochlea, No SO overaction (i.e. not IO palsy!)
nodule on SO tendon V pattern
Acquired: Hypotropia in primary position
Trauma Additional triad:
Tenosynovitis (rheumatoid arthritis) Positive forced duction test
Marfans syndrome Downshoot in adduction
Acromegaly Widening of palpebral ssure on adduction
Extraocular surgery (RD surgery) 3. Management
2. Clinical features Correct amblyopia
Classical triad: Spontaneous recovery common
Defective elevation in adduction (most Steroids (oral or injection into trochlear area)
important)
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Indications for surgery: Diplopia in downgaze

Abnormal head posture SO tenotomy, silicon expander or chicken
Squint (hypotropia) in primary position suture
Browns syndrome IO palsy

Deviation in primary position Slight Signicant hypotropia
Muscle sequalae Contralateral SR overaction Ipsilateral SO overaction
A or V pattern V A
Compensatory head posture Slight Marked chin elevation
Forced duction test Positive Negative
Exam tips:
Sometimes hard to differentiate form IO palsy.
The clinical features can be remembered in triads.
Notes
Causes of V pattern strabismus:

1. Browns syndrome
2. IO overaction secondary to IV nerve palsy
3. LR overaction
4. SR underaction
5. Craniofacial anomalies e.g. Crouzon syndrome
Causes of A pattern strabismus:

1. SO overaction
2. LR underaction
3. IR underaction
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TOPIC 7
STRABISMUS SURGERY
Q Opening Question: What are the Indications for Squint Surgeries?

In general, the indications for squint surgeries are.
Indications for Squint Surgeries
1. Anatomical (largely a cosmetic indication): 2. Functional:
Correct misalignment (large angle, increase Restore BSV (if child is young enough)
frequency of breakdown if intermittent) Correct abnormal head posture
Treat diplopia and confusion
Q What are the Principles of Squint Surgeries?
The principles of squint surgeries are.

Principles of Squint Surgeries
1. Recess or resect? Recession is more forgiving Amblyopia in 1 eye
2. MR or LR? If deviation at near > at distance, Previous surgery in 1 eye
consider operation on MR. If distance > near, 4. How much to correct?
Recess 1 mm = 4 prism D
consider LR
Vertical muscle surgery 1 mm = 3 prism D
3. What are the indications of recess = resect
Resect 1 mm = 2 prism D
operation on 1 eye? Recession of MR more effective than LR
Constant squint in 1 eye
Q How do You Perform a Recession (Resection) Operation?
In a simple case of an XT with deviation worse at distance, I would perform a bilateral LR recession.
Recession Operation
1. GA 4. Stitch two ends of muscle with 6/0 vicryl:
2. U shaped fornix-based conjunctival peritomy One partial and 2 full-thickness bites dividing
3. Isolate LR: muscle into 3 parts
Dissect Tenons capsule on either side of LR Clamp suture ends with Bulldog
muscle with Weskott scissors For resection, measured distance to resect from
Isolate LR muscle with squint hook insertion
Clear off fascial sheath and ligaments with 5. Cut muscle just anterior to stitches (for resection,
sponge cut muscle at the desired site)
Spread muscle using Stevens hook 6. Measure distance of recession
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7. Resuturing of LR: For resection, stitch end to insertion stump

Diathermize point of insertion to create ridge 8. Close conjunctiva with 8/0 vicryl
Stitch each end of muscle to sclera or stitch to
insertion stump using a hangback technique
Notes
Strabismus surgery with forniceal incision

Advantages:
1. Cosmetically superior
2. Less discomfort
3. No trauma to limbal area (stem cells, tear lm disturbances etc)
4. Obeys anatomical planes, hence less bleeding and scarring.
Disadvantages:
1. Adequate exposure difcult needs good assistant
2. Suitable only for younger patients (usually less than 45 years of age) as
Tenons layer is atrophic in older patients
Q What are the Indications for Adjustable Squint Surgeries?
In general, it is indicated in adult squints when a precise outcome is needed.

Adjustable Squint Surgeries
1. Indications: VI CN palsy
Adult squints: Reoperations
Best for rectus muscles 2. Contraindications:
Best with recession (principle: Recess more
Childhood squints
than necessary and adjust postoperatively)
Patient unwilling to cooperate after operation
Vertical squints
Oblique dysfunctions and DVD
Thyroid eye disease
Concomitant nystagmus
Blow out fractures
Q What are the Complications of Squint Surgeries?
The complications can be divided into intraoperative, early and late postoperative complications.
The most dangerous intraoperative complications are scleral perforation and malignant hyperthermia.
Complications of Squint Surgeries

1. Intraoperative (M) Scleral perforation:
Malignant hyperthermia (see below) Thinnest part of sclera (< 0.3 mm just
Lost muscle: posterior to insertion)
MR most common muscle lost Potential sequelae: RD, endophthalmitis,
Muscle retracts into Tenons capsule and vitreous hemorrhage
usually ends up at apex Usually end up with chorioretinal scar
Management:
Slipped muscle:
Stop operation and examine fundus
Slip within muscle capsule
Consider cryotherapy at site of scar
Prevented by adequate suture placement
Refer to retinal surgeon
Management similar to lost muscle
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Others: Preseptal cellulites/orbital cellulites

Oculo-cardiac reex Endophthalmitis (missed perforation)
Hemorrhage
3. Late postoperative (D)
2. Early postoperative (A) Diplopia:
Alignment: Can be early or late
Most common complication Scenarios:
Under- or over-correction In children, diplopia resolves because of
Late misalignment caused by scarring, poor new suppression scotoma or of fusion

fusion, poor vision, altered accommodation In adults, diplopia usually persists if squint
Anterior segment ischemia: is acquired after 10 years of age

Operate on 3 or more recti Management:
Adherence syndrome: Prisms
Tenons capsule is violated Diplopia awareness
Reoperation (adjustable surgery)

Allergic reaction
Infection: Droopy lids (ptosis)
Mild conjunctivitis Dellen and conjunctival cysts
Exam tips:
The complications can be remembered using the mnemonic MAD.
Intraoperative complications begin with M (muscle and malignant hyper-
thermia).
Early postoperative complications begin with A (anterior segment ischemia,
alignment, etc).
Late postoperative complications begin with D (diplopia, droopy lids, etc).
Notes
How do you manage a lost muscle?
Stop operation (do not dig around frantically)
Microscopic exploration (look for suture ends within Tenons capsule)
Irrigate with saline and adrenaline (Tenons usually appears more white)
Watch for oculocardiac reex when structures are pulled
If muscle cannot be found, abandon search
Postoperatively, can try CT scan localization
May consider reoperation/muscle transposition surgery
Q How do You Manage Malignant Hyperthermia?
Malignant hyperthermia is a medical emergency and requires immediate recognition and management.
Malignant Hyperthermia
1. Mechanism of action: Excess calcium binding to skeletal muscles
Acute metabolic condition characterized by initiates and maintains contraction
extreme heat production Muscle contraction leads to anerobic
Inhalation anesthetics (e.g. halothane) and metabolism, metabolic acidosis, lactate
muscle relaxants (succinlycholine) trigger accumulation, heat production and cell
breakdown
following chain of events:
Increase free intracellular calcium
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2. Clinical features: 3. Management:

More common in children Stop triggering agents and nish surgery
Isolated case or family history (AD inheritance) Hyperventilate with 100% oxygen
Early signs: Muscle relaxant (dantrolene)
Tachycardia is earliest sign Prevent hyperthermia:
Unstable BP IV iced saline
Tachypnea Iced lavage of stomach, bladder, rectum
Cyanosis Surface cool with ice blanket
Dark urine Treat complications:
Trismus Sodium bicarbonate (metabolic acidosis)
Elevated carbon dioxide levels Diuretics (renal failure)
Electrolyte imbalance Insulin (hyperkalemia)
Renal failure Cardiac agents (cardiac arrhythmias)
Cardiac failure and arrest
Disseminated intravascular coagulation
Exam tips:
One of the few life-threatening conditions in ophthalmology you need to
know.
Q Tell me about Botulinum Toxin.
Botulinum toxin or Botox is a toxin used for chemodenervation.

The mechanism is believed to be.
The indications in ophthalmology include either squint or lid disorders.
Botulinum Toxin
1. Mechanism of action: Assess possibility of postoperative diplopia
Puried botulinum toxin A from Clostridium before squint operation in adults
botulinum When surgery is contraindicated
Part of transposition operation
Permanent blockage of acetylcholine release
Cyclic ET
from nerve terminals
Lid disorders:
Injection with electromyographic guidance
Essential blepharospasm
After injection, Botox bound and internalized
Hemifacial spasm
within 2448 hours
3. Complications:
Paralysis of muscle within 4872 hours
Intraoperative:
Recovery by sprouting of new nerve terminals;
Scleral perforation
paralysis recovers in 2 (squint) to 3 months (lid)
Retrobulbar hemorrhage
2. Indications: Postoperative:
Squint: Temporary ptosis (common)
VI CN palsy (weakening of antagonistic MR Vertical squints
to prevent contracture) Diplopia
Small angle squints Mydriasis
Postoperative residual squint
B1037_Ch-09.indd 472 12/22/2010 8:23:31 PM

TOPIC 8
RETINOBLASTOMA
Q Opening Question No. 1: Tell me about Retinoblastoma (RB).

RB is a tumor of the primitive retinal cells.
Epidemiology
1. RB is the most common primary, malignant, 4. Incidence is 1 in 20,000 births (range 1 in 14,000
intraocular tumor of childhood to 1 in 34,000)
2. Eighth most common childhood cancer 5. No gender or racial variation
3. Second most common intraocular tumor (after
choroidal melanoma)
Q Opening Question No. 2: Tell me about the Genetics of Retinoblastoma.

Retinoblastoma gene is a tumor suppressor gene, which is located on.
RB can be divided into hereditary vs nonhereditary RB.
Genetics of Retinoblastoma
1. RB gene (RB1) (one of Knudsons 2 hits occur prior to
Maps to chromosome 13 q14 (13 associated conception)
with bad luck) 40% of RB is hereditary type of RB
Produces RB protein (pRB) that binds various The risk of the Knudsons second hit/new
cellular proteins to suppress cell growth mutation is extremely high (therefore RB is
RB1 is a recessive oncogene at cellular level inherited as AD trait with 90% penetrance)
Mutations of RB1 alleles result in cancer only in There is risk of bilateral RB (as all cells have
developing retina; other cell types die by inherited one mutant allele)
apoptosis in absence of RB1 There is risk of non-ocular malignancies
Primitive retinal cells disappear within rst few elsewhere (as all cells have one mutant allele)
years of life so RB is seldom seen after 3 or 4 Age of presentation: 1 year
years of age 4. Nonhereditary RB
2. Knudsons 2 hit hypothesis Both alleles are normal after fertilization, but two
Both alleles must be knocked out for tumor to or more subsequent spontaneous mutations
develop inactivate both alleles (both of Knudsons 2 hits
3. Hereditary RB occur after conception)
The patient inherits one mutant allele from 60% of RB is nonhereditary type of RB
parents and one normal allele which undergoes No risk of bilateral RB
subsequent new mutation after conception No risk of nonocular malignancies elsewhere
Age of presentation: 2 years
B1037_Ch-09.indd 473 12/22/2010 8:23:31 PM

Distinguish Between
Hereditary (inherited RB gene) Familial (positive family history)

vs nonfamilial Bilateral vs Unilateral vs nonfamilial
Hereditary (40%) Bilateral (30%) Familial (6%)
Nonhereditary (60%) Unilateral (70%): Nonfamilial (94%):
1015% of unilateral cases are still 2530% of nonfamilial cases
hereditary RB are still hereditary RB (the rate
Therefore absence of bilateral RB of new mutation is high)
does not rule out hereditary RB Therefore a negative family
history does not rule out
hereditary RB
Exam tips:
The FIRST of a few important topics in RB.
Be clear about percentages (see table below).
Distinguish between hereditary vs nonhereditary, familial vs nonfamilial and
unilateral vs bilateral RB. It is conceptually easiest to talk about hereditary vs
nonhereditary RB.
Q How do You Counsel Parents with a Child with RB?
Risk of RB depends on presence or absence of family history and whether tumor is unilateral or
bilateral.
If there is a positive family history, the risk to the next child is 40%.
If there is no family history, but the tumor is bilateral, the risk to the next child is 6%.
If there is no family history and the tumor is unilateral, the risk to the next child is only 1%.
Genetic Counseling
Chance of the following people having a baby with RB:

Parent Affected child (patient) Normal sibling
Family history 40% 40% 7%
No family history
Bilateral 6% 40% 1%
Unilateral 1% 8% 1%
Q Opening Question No. 3: What is the Pathology of Retinoblastoma?

RB is a tumor of the primitive retinal cells.
Pathological it has distinct gross and microscopic features.
Pathology
1. Originates from neuroretina (primitive cone cells) Dystrophic calcication
2. Gross pathology Presents with endophthalmitis picture
Endophytic tumor: Exophytic tumor:
Projects into vitreous cavity Grows into subretinal space
White or pink Presents with total retinal detachment
Cottage cheese appearance Diffuse inltrative tumor:
B1037_Ch-09.indd 474 12/22/2010 8:23:31 PM

Age of presentation: 6 years High mitotic activity

Presents with uveitis, glaucoma Necrosis
3. Histopathology Calcication
RB cells (5 features): Arrangement (Homer Wright, Flexner

Twice the size of lymphocytes with round or Wintersteiner rosettes and eurettes)
oval nuclei Exam tips:
Hyperchromatic nuclei with little cytoplasm
Type of
arrangements Differentiation Features
Homer Wright Neurobastic differentiation Single row of columnar cells surrounding a central
lumen
Central lumen is tangle of neural laments
Can be seen in neuroblastoma and medulloblastoma
Flexner Early retinal differentiation Single row of columnar cells surrounding a central
Wintersteiner lumen with a refractile lining
Cilia projects into lumen
Central lumen is subretinal space
Refractile lining is external limiting membrane
Can be seen in retinocytoma and pinealoblastoma
Fleurettes Photoreceptor differentiation Two rows of curvilinear cells
Inner cluster represents rod and cone inner segments
Outer cluster represents outer segment
The SECOND of important topics in RB.

The 5 histological features of RB should be contrasted with the 5 features of
retinocytoma (see below).
Retinocytoma can be considered a benign variant of RB.
Q What is a Retinocytoma?
Pathological and genetically it shares many characteristics of RD.

Retinocytoma
1. Originates from neuroretina
2. Same genetic implications Low or no mitotic activity
3. Histopathology No necrosis
Calcication not common
Retinocytoma (5 features):
Round or oval nuclei with even chromatin
Arrangement (Flexner Wintersteiner rosettes
distribution and eurettes)
More cytoplasm
Exam tips:
A fairly common follow-up question to the pathology or genetics of RB.
B1037_Ch-09.indd 475 12/22/2010 8:23:31 PM

Q How do You Manage a Patient with Retinoblastoma?
The aims of management of RB are.

This depends on a team approach involving.
Indications for Enucleation for RB
1. Large unilateral tumor Total retinal detachment
Large unilateral RB occupying more than 1/2 of Iris neovascularization
globe Ciliary body involvement
Large unilateral RB with no visual potential 3. Failure of other treatment
2. Associated complications
Massive vitreous seeds
Exam tips:
Extremely difcult question. Answer with broad principles.
Do not get into details too quickly.
Q Tell me about Chemotherapy for Retinoblastoma.
The indications for chemotherapy in RB are.

The current drugs under investigations include.
Chemotherapy for RB
1. Indications 4. Response to chemotherapy
Curative: 80% remission at 3 years
Chemoreduction for small- and medium-size RB tumors frequently become multi-drug
tumors resistant and regrow after initial response
Vitreous/subretinal seeds (isolated local Related to expression of P-glycoprotein (P170)
therapy is not good enough) (note: This is a relatively hot topic!)
Palliative: Increased P170 correlated with therapeutic
Tumor cells crossed lamina cribrosa/ failure in other tumors (neuroblastoma,
extraocular extension rhabdomyosarcoma, leukemia, myeloma,
Orbital recurrences lymphoma)
Metastasis
Favorable response to chemotherapy:
2. Drugs used Considerable shrinkage after 2 cycles
VEC (vincristine, etoposide, carboplatin) Reduced vascularization or avascular tumor
VTC (tenoposide instead of etoposide) Calcication (cottage-cheese appearance)
Cyclosporin Disappearance or signicant clearance of
vitreous seeds
3. Cycles
Resolution of extensive RD
Four cycles:
Unfavorable response to chemotherapy:
Small to medium-size tumors, 410 disc
Little shrinkage or calcication
diameters, < 4 mm thick
Remains vascular or translucent (sh-esh
Seven to nine:
appearance)
Larger tumors, vitreous seeds, RD, bone
Unchanged vitreous seeds
marrow or orbital involvement
Exam tips:
Relatively hot topic for RB.
See Ophthalmology 1997; 104: 21012111.
B1037_Ch-09.indd 476 12/22/2010 8:23:32 PM

Q Tell me about Second Cancers in Retinoblastoma.
Second cancers are leading causes of death in patients with the hereditary type of RB.
The incidence is.
The common tumors include.
The different modalities available include.
Management of Retinoblastoma
1. Aims of management Visual potential of affected eye
First goal to save life Visual potential of unaffected eye
Second goal to save eye Associated ocular problems (e.g. RD, vitreous
Third goal to maximize vision hemorrhage, iris neovascularization, secondary
2. Team approach glaucoma)
Ophthalmologist Age and general health of child
Pediatric oncologist and radiation oncologist Personal preferences of parents
Geneticist 6. Follow-up
Ocular prosthetist Patients with treated RB and siblings at risk need
Medical social worker and RB support group to be followed up indenitely
3. Treatment methods After initial treatment, re-examine patient 36
Enucleation weeks later:
Active tumor on treatment requires follow-up
External beam radiotherapy
every 3 weeks
Chemotherapy (e.g. chemoreduction, systemic
If tumor is obliterated, follow-up 612 weeks
chemotherapy, subconjunctival chemoreduction,
later
intrathecal cytosine arabinoside)
3-monthly until 2 years post-treatment, then 6
Focal therapy (e.g. laser, cryotherapy,
monthly until 6 years of age, then yearly for life
radioactive plaque, thermotherapy)
7. Risk of new or recurrent retinoblastoma
Orbital exenteration
Risk of new RB decreases rapidly after 4 years of
4. Trends
age to negligible risk after 7 years of age
In the past, enucleation was standard treatment
Risk of recurrence of treated RB negligible after
for small tumors within globe and external
2 years of completed treatment (unrelated to
beam radiotherapy was standard for large
patients age)
tumors extending out of globe
8. Prognosis
Trend towards more conservative treatment for
Location (most important factor):
small to medium-size tumors
95% 5-year survival if intraocular tumor
Increasing use of chemotherapy followed by
5% 5-year survival with extraocular
focal therapy for small tumors and plaque extension/optic nerve involvement
radiotherapy for medium-size tumors Tumor size and grade
5. Factors to consider Iris rubeosis
Tumor size and location Bilateral tumors (risk of second malignancy)
Bilateral or unilateral disease Age of patient (older is worse)
Notes
Presenting features of retinoblastoma:
Leukocoria (60%)
Strabismus (20%)
Secondary glaucoma
Masquerade syndrome/iris nodules
(Continued)
B1037_Ch-09.indd 477 12/22/2010 8:23:32 PM

(Continued)
Orbital inammation
Routine examination of a patient at risk of RB
Late presentation: Orbital invasion, metastases
Q What are the Current Indications for Enucleation of Retinoblastoma?
Enucleation remains the treatment of choice for large tumors.

And in eyes with little or no potential vision.
Second Cancers in RB Patients
1. Incidence Pineoblastoma, ectopic intracanial RB (trilateral
Hereditary RB: 6% over lifetime RB) is common up to 2 years after diagnosis of
Hereditary RB with external beam radiotherapy: RB
Incidence 1% per year in eld of radiation Beyond 2 years after diagnosis of RB:
(i.e. 30% in 30 years, 50% in 50 years) Bony and soft tissue sarcomas (Ewings tumor,
Average age of diagnosis: 13 years (note: chondrosarcoma, rhabdomyosarcoma)
Remember that RB gene is on chromosome 13!) Skin tumors (malignant melanoma, sebaceous
cell CA, squamous cell CA)
2. Type of tumors
Neuroblastoma, medulloblastoma, leukemia
Osteogenic sarcoma is the most common cancer
Q What is the Reese-Ellsworth Classication?
Refers to a classication which relates to VISUAL prognosis (not mortality).

Based on size, number, location of tumor and vitreous involvement.
Reese-Ellsworth Classication
Group I (very favorable, cure rate 95%): Group III (doubtful, 67%):
Less than 4 DD Larger than 10 DD
Solitary or multiple Anterior to equator
Behind equator Group IV (unfavorable, 50%):
No vitreous seeding Multiple, some larger than 10 DD
Group II (favorable, 87%): At ora
410 DD Group V (very unfavorable, 34%):
Solitary or multiple Massive tumors involving 1/2 of retina
Behind equator Vitreous seeding
Notes
International classication of RB (predicts those likely to be cured without
need for enucleation or EBRT):
Group A: Small intraretinal tumors ( 3 mm) away from foveola (> 3 mm) and
disc (> 1.5 mm)
Group B: All remaining discrete tumors conned to the retina
Group C: Discrete local ( 1/4 of retina) disease with minimal subretinal or
vitreous seeding
Group D: Diffuse disease with signicant vitreous or subretinal seeding
Group E: Presence of any one or more of these poor prognosis features:
Tumor touching lens
Tumor anterior to anterior vitreous face, involving ciliary body or
anterior segment
(Continued)
B1037_Ch-09.indd 478 12/22/2010 8:23:32 PM

(Continued )
Diffuse inltrating RB
Neovascular glaucoma
Opaque media from hemorrhage
Tumor necrosis with aseptic orbital cellulitis
Phthisis bulbi
Q How do You Manage a Child with Leukocoria?
In a child with leukocoria, the most important diagnosis to exclude is retinoblastoma.

However, the other common diagnoses for leukocoria are.
The management involves a complete history, ocular and systemic examination and appropriate
investigations.
Leukocoria
1. Causes of leukocoria Birth history:
Retinoblastoma Weight (ROP)
Other common causes: Trauma (congenital cataract, retinal
Persistent hyperplastic primary vitreous detachment, vitreous hemorrhage)
(PHPV) (30% of cases) Oxygen exposure (ROP)
Coats disease (15%) Family history:
Toxocara (15%) None (PHPV, Coats disease, toxocara)
Congenital cataract AD (RB)
Vascular diseases: SLR (juvenile retinoschisis, Norries disease)
ROP AD/SLD (incontinentia pigmenti)
Incontinentia pigmenti 3. Examination
Congenital/developmental anomalies: Unilateral (RB, PHPV, Coats disease, toxocara
Large coloboma and cataract)
Retinal dysplasia Bilateral (RB, ROP, cataract, Norries disease,
Juvenile retinoschisis
incontinentia pigmenti)
Norries disease
Normal size eye and no cataract (RB)
Combined hamartoma of retina and RPE
Microophthalmia or concomitant cataract
Other tumors:
(PHPV)
Medulloepithelioma
Retinal astrocytoma
Other ocular abnormalities (Norries disease)
2. History 4. Investigation
Age of presentation: Ultrasound:
Acoustically solid tumor with high internal
Birth (PHPV)
13 years (RB)
reectivity (RB)
Calcication (RB)
Preschool (Coats disease, toxocara)
Sex: CT scans:
Calcication (RB)
Male (Coats disease, juvenile retinoschisis,
Optic nerve, orbital and CNS involvement (RB)
Norries disease)
Female (incontinentia pigmenti) MRI:
Detect pinealoblastoma (RB)
Pregnancy history:
Optic nerve involvement (RB)
Gestational age (ROP)
Maternal health (TORCH syndromes)
Exam tips:
Relatively common essay question.
Remember NOT to focus solely on retinoblastoma.
B1037_Ch-09.indd 479 12/22/2010 8:23:32 PM

Section 10
Miscellaneous
Examination
Problems
B1037_Ch-10.indd 481 12/24/2010 4:03:00 PM

TOPIC 1
OCULAR TRAUMA
Q Opening Question: What are the Possible Manifestations of Blunt Ocular Trauma?
The ocular manifestations can be divided into orbit, anterior and posterior segment and neurological.
Blunt Ocular Trauma
1. Orbital fracture Retinal breaks and detachment:
Retinal dialysis
2. Anterior segment:
U-shaped tears and operculated retinal holes
Hyphema
in the periphery
Iris and angles:
Giant retinal tear
Traumatic mydriasis, miosis
Macular hole
Angle recession, iridodialysis, cyclodialysis
Choroidal rupture:
Lens:
SRNVM
Traumatic cataract (Vossius ring)
Scleral rupture
Lens subluxation/dislocation
Optic nerve avulsion
3. Posterior segment:
4. Neurological:
Vitreous hemorrhage
Traumatic optic neuropathy
Commotio retinae
SO palsy
Vitreous base avulsion
Exam tips:
Because this problem is so common in daily clinical practice, candidates
are frequently not adequately prepared for this question in examinations!
Q What are the Signs of Penetrating Ocular Trauma?
Signs of Penetrating Ocular Trauma

1. Suggestive signs: Localized cataract
Deep lid laceration Retinal tear/hemorrhage
Conjunctiva: 2. Diagnostic:
Hemorrhage, laceration Laceration with positive Siedals test
Chemosis Exposed uvea, vitreous and retina at wound
Iris and AC: Visualization of IOFB
Iridocorneal adhesion
XR diagnosis of IOFB
Iris defect
Shallow AC
Hypotony
B1037_Ch-10.indd 483 12/24/2010 4:03:02 PM

Q How would You Manage a Patient with a Penetrating Injury?
Management must be individualized.

The principles of management are to manage life-threatening injuries rst, then assess severity of the eye
injury, exclude IOFB and infection, restore globe integrity, and manage secondary injuries.
Principles of Management of Penetrating
Injury Non-depolarizing anesthesia
1. Manage life-threatening injuries rst Eyelids retracted with Jeffreys speculum (less
2. Assess severity and extent of penetrating injury pressure on globes) or lid sutures
Swab for gram stain and cultures
3. Tetanus vaccine
Clean eye with chlorhexidine (rather than
4. Exclude IOFB: iodine)
Suggestive features: Assess extent of injury
History (projectile foreign body, hammering AC paracentesis, re-form AC with
related activities) viscoelastic, reposit prolapsing uveal tissue if
Examination: Visible entry wound, iris defect still viable
Dilated fundal examination If wound limited to the cornea:
XR orbit Close wound with 100 nylon
B-scan Sutures should be longer and more widely
Consider CT scan spaced near the limbus

Central corneal sutures are shorter and
5. If IOFB is present:
more closely spaced
Removal of IOFB indicated if injury is acute
Sutures should be deep (~3/4 of corneal
(e.g. within 2448 hours): thickness)
If patient presents much later (e.g. 7 days), Water-tight closure essential (as wound
removal is indicated if: may leak when corneal edema resolves)
Endophthalmitis is present If wound involves both cornea and sclera:
IOFB is toxic (e.g. copper, iron material) Conjunctival peritomy to determine
IOFB is organic posterior limits of scleral wound (may need
Associated vitreous hemorrhage to disinsert extraocular muscles)
IOFB is impacted onto retina Appose limbus rst with 8/0 or 10/0 nylon
Secondary surgery is being considered (e.g. Suture corneal wound (see above)
RD surgery) Scleral wound sutured with 8/0 nylon
Otherwise, can consider leaving IOFB in situ Conjunctiva sutured with 8/0 vicryl
6. Exclude infection: Assess for Siedels ensure wounds are not
No obvious signs of infection leaking

Clean wound prophylactic antibiotics Subconjunctival and intracameral antibiotics
(topical) KIV bandage contact lens
Dirty wound prophylactic antibiotics Eye shield to protect eye
(topical and systemic) Post-surgery:

Endophthalmitis therapeutic antibiotics Monitor closely for signs of infection
If no signs of infection, steroids can be started
(intravitreal, topical and systemic)
cautiously
7. Restore globe integrity:
8. Assess secondary injuries and complications
Principles:
Surgical closure of wound and manage accordingly
Minimal distortion of globe anatomy
Procedure:
Eye shield to protect the eye before surgery
B1037_Ch-10.indd 484 12/24/2010 4:03:04 PM

Notes
Ocular trauma score derived from:
Initial VA
Globe rupture
Endophthalmitis
Perforating injury
Retinal detachment
RAPD
Notes
Parameters used to classify ocular trauma developed by the Ocular Trauma
Classication Group (Am J Ophthalmol 1998;125:56556):
Type of injury
Grade of injury (visual acuity)
Pupil (presence of RAPD)
Zone of injury (location)
Q How would You Manage a Patient with a Penetrating Injury?
Management of a patient with IOFB must be individualized.

The principles of management are to assess time of injury, site and nature of IOFB, exclude other
complications and decide on whether the IOFB needs to be removed.
Principles of Management of Penetrating Injury
1. Factors to consider: IOFB is organic
Time of injury: Associated vitreous hemorrhage
Acute or late presentation IOFB is impacted onto retina
Assess site of IOFB: Secondary surgery is being considered (e.g.
Anterior or posterior segment RD surgery)
Free-oating in vitreous or incarcerated with 4. Type of surgery:
tissues Small, free-oating metallic IOFB in vitreous
Assess nature of IOFB: removal with intraocular magnet
Organic or nonorganic Large nonmetallic IOFB incarcerated in retina
Inert or toxic
vitrectomy, lensectomy and intraocular
2. Exclude infection and secondary injuries forceps:
Cataract will cause poor view of posterior A small rare earth magnet can be used to
segment mobilize foreign body from retinal surface
RD
3. Removal of IOFB indicated if injury acute
(e.g. 2448 hours):
If patient presents much later (e.g. 7 days),
removal is indicated if:
Endophthalmitis is present
IOFB is toxic (e.g. copper, iron material)
B1037_Ch-10.indd 485 12/24/2010 4:03:05 PM

Clinical approach to ocular trauma
This patient had ocular injury three months ago. Please examine him.
There are periorbital and lid scars seen.
Look for
Corneal injury laceration scars, suture wounds, siderosis bulbi, blood
staining
AC depth uneven (lens subluxation)
Iris iridodialysis, traumatic mydriasis
Lens phacodonesis, cataract
Vitreous in AC
Id like to
Check IOP and perform a gonioscopy (angle recession, cyclodialysis)
Examine the pupils for RAPD (traumatic optic neuropathy)
Examine the fundus:
Macular hole
Retinal breaks, detachment and dialysis
Choroidal rupture
Optic atrophy
Check extraocular movements (SO palsy)
B1037_Ch-10.indd 486 12/24/2010 4:03:05 PM

TOPIC 2
COLOR VISION
Q Opening Question: What is Color Vision?

Color vision is the ability to perceive and differentiate colors.
It is the sensory response to stimulation of cones by light of wavelength 400700 nm.
The physiological basis is the relative absorption of different wavelengths by the three cones.
Color itself can be described in terms of its hue, saturation and brightness.
Color Vision
1. Denition: Opponent color theory = stimulation and
Sensory response to stimulation of cones by inhibition of different receptive elds
light of wavelength 400700 nm Receptive elds of color sensitive cells have
Relative absorption of different wavelengths by regions that compare intensity of:
cone outer segment visual pigments Red vs green
Blue vs yellow
2. Two basic theories:
Trichromatic theory = selective wavelength 3. Description of color:
absorption Hue (color): Refers to wavelength
Three types of photolabile visual pigments: Saturation: Refers to depth of color, purity or
Short wavelength: Absorbed by blue cones richness of color
Middle wavelength: Absorbed by green Brightness: Refers to intensity or radiant ux
cones
Long wavelength: Absorbed by red cones
Exam tips:
Extremely common question in the viva.
Q What is Color Blindness?
Color blindness can be divided into congenital vs acquired.
1. Color blindness
Congenital: No inheritance pattern
8% of all males and 0.5% all females Yellow-blue abnormality
SLR inheritance Patients use incorrect color names or report
Red-green abnormality that color appearance of familial objects
Patients are not aware of wrong color (e.g. apple) has changed
Bilateral and symmetrical between 2 eyes Unilateral or asymmetrical between two eyes
Acquired:
Males and females equally affected
B1037_Ch-10.indd 487 12/24/2010 4:03:06 PM

2. Classication: Anomalous trichromats: Require 3 colors, but

Clinical: Based on color matching: in abnormal proportions
Trichromats: Require all 3 primary colors to Pathological: Based on loss or abnormality of
match an arbitrary color (possess 3 normal cone pigments:
cones) Loss of red sensitive cone: Protan defect
Dichromats: Require only 2 colors (loss of 1 Loss of green sensitive cone: Deutan defect
type of cone) Loss of blue/yellow sensitive cone: Tritan
Monochromats: Cannot match any color (loss defect
of 2 or 3 types of cones)
Q What is Achromatopsia?
Achromatopsia is a congenital color blindness with absence of color discrimination.

It can be divided into blue cone monochromatism of rod monochromatism.
Achromatopsia
1. Types: 2. Diagnosis:
Blue cone monochromatism: Present with congenital nystagmus, poor VA and
Only blue sensitive cones present. Loss of photoaversion
both red and green cones. Because only one ERG:
cone is present, there is no effective cone Absence of cone responses
function Rod ERG normal
SLR Dark adaptation test:
Rod monochromatism: No cone plateau
Loss of 3 cones (e.g. true achromatopsia/true No cone rod break
color blindness)
AR
Sees with shades of gray
Q Tell me about Color Vision Tests.
They can be divided into quantitative or qualitative tests.

Color Vision Tests
1. Quantitative (both sensitive and specic) 15 colored tablets
Farnsworth-Munsell 100 hue test: Hues more saturated than 100 hue test
Based on matching hues/color Tablets arranged in sequence
Consists of 84 colored discs Errors plotted very quickly on a simple
Discs arranged in sequence (increasing levels circular diagram to dene nature of color
of hue) deciency
Test is then scored Not very sensitive
Difference in hues between adjacent tablets Useful in judging practical signicance of
is 14 nm color deciency
Accurate in classifying color deciency Desaturated versions available to recognize
Very sensitive more subtle degrees of color deciency
Time consuming and tiring Discriminates well between congenital and
Nagels anomaloscope: acquired defects
Based on matching luminance or brightness Congenital defects:
Good for congenital red-green color defects Very precise protan/deutan pattern
Sensitive Acquired defects:
Irregular pattern or errors

2. Qualitative (more sensitive, but less specic):
Shows tritan errors very clearly
Farnsworth 15 panel
More rapid and convenient to use than 100 Pseudoisochromatic color plate test:
Examples: Ishihara/AO Hardy Rand Rittler
hue test
B1037_Ch-10.indd 488 12/24/2010 4:03:07 PM

Gross estimate of acquired color loss and Quick, available, useful

central visual dysfunction Test congenital red-green defects
Q Tell me about the Ishihara Plates.
The Ishihara plates is a type of qualitative color vision test.

Ishihara Plates
1. Test in well-illuminated room Only reads 12: Total color blindness
2. Held 75 cm from subject and perpendicular to Reads rst 7 plates (except 12) incorrectly and
line of sight unable to read the rest: Red-green deciency
Reads 26 as 6 and 42 as 2: Protan defect
3. Literate patients use plates 117
Reads 26 as 2 and 42 as 4: Deutan defect
Answer given within 3 seconds
Unable to read all plates, including 12
4. Illiterate patients use plate 1824 (despite good VA): Suspect functional color
Lines traced with a brush within 10 seconds blindness
5. Results:
13 plates correct: Normal color vision
< 9 plates correct: Decient color vision
B1037_Ch-10.indd 489 12/24/2010 4:03:08 PM

TOPIC 3
LASERS IN
OPHTHALMOLOGY
Q Opening Question: What is a Laser? What are the Basic Components of a Laser System?
Laser stands for.
Lasers
1. Denition: 2. Basic components:
Laser = Light Amplication by Stimulated Power source:
Emission of Radiation Generate energy
Laser light is: Active medium:
Monochromatic (same wavelength) Special properties to emit photons
Coherent (in phase) Chamber:
Polarized (in one plane) Stores active medium
Collimated (in one direction and Mirrors at opposite ends to reect energy
nonspreading) back and forth (optical feedback)
High energy One of the mirror partially transmits energy
Exam tips:
See relevant sections in glaucoma (page 77) and retina (pages 178 and 190).
Q What are the Lasers available in Ophthalmology?
Lasers can be classied either by their clinical effects or by the active medium.
Laser Classication
1. Clinical effects Photodisruption:
Photocoagulation (thermal effect): Temperature raised to 15,000C
Temperature raised to 80C Interatomic forces are destroyed (electrons
Coagulation of proteins stripped from atoms)
Clinical effect: Burn tissue Plasma formation (fourth state of matter,
Example: Argon laser, Nd:YAG laser in physical properties of gas, electrical
continuous mode properties of metal)
Clinical effect: Cut tissue
B1037_Ch-10.indd 491 12/24/2010 4:03:09 PM

Example: Nd:YAG laser in Q switched or

Argon, krypton, carbon dioxide
mode locked Solid state (crystals) lasers:
Photoablation: Nd:YAG, holmium: YAG
No release of heat Liquid lasers:
Interatomic forces are destroyed (carbon- Dye lasers
carbon bonds are broken) Others:
Clinical effect: Etch tissue Diode, excimer
Example: Excimer
2. Active medium
Gas lasers:
B1037_Ch-10.indd 492 12/24/2010 4:03:10 PM

TOPIC 4
VITAMINS, ALCOHOL,
DRUGS AND SKIN
Q Opening Question: What are the Associations between Vitamin and the Eye?
Vitamin deciency or excess causes eye diseases.
Vitamins can also be used for treatment.
Vitamins and the Eye
1. Deciency: Vitamin D:
Vitamin A (see below) Band keratopathy (metastatic calcication)
Vitamin B: 3. Treatment:
Optic neuropathy Vitamin A (abetalipoproteinemia in Bassen
Angular blepharoconjunctivitis (B2) Kornzweig syndrome) (page 215)
Gyrate atrophy (B6) (page 220) Vitamin B6 (gyrate atrophy, homocystinuria)
Flame-shaped hemorrhage (B12)
Vitamin C (chemical injury)
Vitamin C: Vitamin E (abetalipoproteinemia in Bassen
Subconjunctival hemorrhage
Kornzweig syndrome, ROP)
Vitamin D:
Vitamin K (coagulation problem)
Associated with proptosis
2. Excess:
Vitamin A:
Benign intracranial hypertension (page 262)
Q Tell me about Vitamin A Deciency.
Vitamin A deciency is one of the common causes of blindness in the world.

1. Role of vitamin A: X2: Corneal xerosis
Precursor of photosensitive visual pigment X3
Outer segment turnover X3A: Corneal ulceration/keratomalacia < 1/3
Maintain conjunctival mucosa and corneal corneal surface
stroma X3B: Corneal ulceration/keratomalacia > 1/3
corneal surface
2. Clinical features/WHO classication:
XS: Corneal scarring
XN: Night blindness
XF: Xerophthalmic fundus
X1
X1A: Conjunctiva xerosis
X1B: Bitots spots
B1037_Ch-10.indd 493 12/24/2010 4:03:11 PM

Exam tips:
Vitamins affect both the front (conjunctiva/cornea) and the back (retina) of
the eye.
See also vitamin A cycle (page 145).
Q What Systemic Drugs Have Established Ocular Toxicities?
Classication Site Syndrome Drugs

Anterior segment Conjunctiva Steven Johnsons Sulphonamides
syndrome
Cornea Vortex keratopathy Amiodarone
(page 117) Chloroquine
Chlorpromazine
Tamoxifen
Indomethacin
Band keratopathy Vitamin D
Angles Glaucoma Steroids
Lens Cataract Steroids
Amiodarone
Chlorpromazine
Gold
Busulphan
Posterior segment Retina Retinotoxicity Chloroquine (see below)
Optic nerve Optic neuropathy Ethambutol, boniazid,
(page 248) streptomycin
Alcohol
Chloroquine
Chloramphenicol
Digitalis
Tamoxifen
Chemotherapeutic agents
Neurological BIH Steroid
Benign intracranial Tetracycline
hypertension (page 262) Nalidixic acid
Vitamin A
Q What are the Patterns of Retinal Toxicity with Systemic Drugs?

Retinal Toxicity
1. RPE/photoreceptor dysfunction 3. Macular edema
Chloroquine, hydroxychloroquine Nicotinic acid (treatment of hyperlipidemia)
Phenothiazines Oral contraceptive
Deferoxamine (treatment of thalassemia) 4. Crystalline retinopathy
2. Vascular Tamoxifen
Quinine Canthaxanthine (used to enhance sun-tanning)
Oral contraceptive Methoxyurane (anesthetic agent)
5. Color vision
Digoxin
B1037_Ch-10.indd 494 12/24/2010 4:03:12 PM

Q What are the Associations between Alcohol and the Eye?
Effects of Alcohol
1. Indirect effects on the eye: 2. Direct effects on the eye:
Risk factor for cardiovascular disease and ocular Toxic optic neuropathy (page 248)
ischemia (see page 200) Fetal alcohol syndrome
Risk factor for ocular trauma
Drug interactions
Q What are the Associations between Skin Disorders and the Eye?
Skin Disorders and the Eye

1. Acneiform disorders: Sclerodactyly, telangiectasia, Raynauds

Acne rosacea phenomenon, digital ulcers
2. Bullous dermatoses: Rheumatoid arthritis
Rheumatoid nodules, vasculitic skin lesions,
Ocular pemphigus, ocular pemphigoid
generalized rash of Stills disease
Steven Johnsons disease
Sarcoidosis
3. Congenital skin disorders: Erythema nodosum, lupus pernio, sarcoid
Albinism granulomas
Incontinentia pigmenti Wegeners granulomatosis
Xeroderma pigmentosum Purpura, hemorrhagic vesicles, gingival
Congenital ichthyosis hyperplasia
4. Connective tissue disorders: 5. Dermatitis:
Systemic lupus Atopic dermatitis
Buttery rash, discoid lupus, photosensitivity,
6. Infections:
alopecia, telangiectasia
Herpes simplex, herpes zoster, AIDS
Scleroderma
Exam tips:
Remember ABCD.
Q Tell me about Atopic Dermatitis.
Atopic dermatitis is a chronic inammatory dermatitis with skin and ocular manifestations.
Atopic Dermatitis
1. Skin ndings: Vernal or atopic keratoconjunctivitis
Acute: Exudation, vesicles, crusting Keratoconus
Chronic: Lichenication, pigmentation Cataract (anterior lamellar cataract)
2. Ocular ndings: RD (increased risk of RD after cataract
Blepharitis, conjunctivitis surgery)
B1037_Ch-10.indd 495 12/24/2010 4:03:13 PM

TOPIC 5
EPIDEMIOLOGY, PUBLIC
HEALTH AND RESEARCH
METHODS
Q Opening Question: How would You Test a New Drug X in the Treatment of Glaucoma?
Steps in RCT
1. Background research into clinical problem: 5. Outcome:
Do you need a new drug? VA?
What are the current therapies and how Lower IOP?
effective are they? (e.g. efcacy, side effects) Optic disc and VF changes?
What are the characteristics of the ideal drug Complications?
(long-term efcacy, side effects, costs, etc) 6. Exit protocol:
2. Dene study population: Failure denitions (when do you consider
Restricted population or generalized population? whether a drug has worked or not worked?)
Early glaucoma or advanced glaucoma? If it does not work, then what? How long do
New cases or those on previous follow-up? you wait?
Include patients with concomitant diseases If it works better than old therapy, how much is
(e.g. DM)? the effect?
3. Randomization: 7. Statistics:
New drug vs placebo? Sample size and power issues
New drug vs established drugs? Randomization protocol
Cross-over study design (drug and placebo are Statistical signicance
exchanged in course of RCT)? 8. Other issues:
4. Masking: Ethics
Single (participants), double (participants and
investigators) or triple (participants, investigators
and reviewers) masking?
Unmasking protocol (when do you stop?)
Exam tips:
Candidates will be expected to know something about research methods and
randomized clinical trials (RCT).
B1037_Ch-10.indd 497 12/24/2010 4:03:14 PM

Q What is Primary Prevention?

Prevention Strategies
1. Primary prevention prevent disease, usually 3. Tertiary prevention (controversial) prevent
before occurrence of symptoms effects of complications on morbidity (and
Screening for DR and glaucoma mortality)
2. Secondary prevention prevent progression of PRP for PDR
disease, before occurrence of complications
Lower IOP in glaucoma
Q What is Screening?
Screening
1. Denition: 3. Criteria for screening of a disease:
Presumptive identication of unrecognized Important public health problem (high
disease by application of tests which can be prevalence, high rate of morbidity).
applied rapidly Natural history understood (asymptomatic latent
Screening for asymptomatic people phase must be present)
Raises ethical and societal issues (who to Acceptable, effective and available treatment
screen? At what costs?) Early detection has effect on treatment outcome
2. Goals of screening: and natural history
Primary prevention if possible; usually this Acceptable, reliable (repeatable) and valid (high
cannot be achieved sensitivity and specicity) screening test at
Secondary prevention by improving outcome of reasonable cost
disease by early detection and treatment
Q What are the Major Causes of Blindness Today? What are the Issues?
Natural history and

Disease Public health problem treatment Screening test
Glaucoma 5060 million Denition of glaucoma? Screening tests?
56 million blind Natural history not well All screening test low
Can measure glaucoma understood sensitivity and specicity
Can treat risk factor Natural history of ocular Screening tests accept-
(IOP) hypertension (risk of able?
glaucoma is 1%/year) Costs of screening?
Need for treatment?
Better outcome with
early treatment?
Medical vs surgical?
(Continued )
B1037_Ch-10.indd 498 12/24/2010 4:03:15 PM

(Continued )
Natural history and

Disease Public health problem treatment Screening test
Diabetic Most common cause of Natural history fairly What screening tests?
retinopathy blindness in 3050 clear 1. DR photography
years (working Well-dened dilated vs nondilated
population): asymptomatic stage 2. Direct fundoscopy
2% of High risk factors dilated vs
population in U.S. identied: nondilated
(50 million patients) Duration of DM 3. FFA
30% NPDR Control of DM Who should screen?
13% vision threatening HPT 1. Ophthalmologists
DR (6 million patients/12 Smoking is not risk 2. Internists
million eyes need factor 3. Family physicians
treatment) Early treatment benecial 4. Optometrists
24% million laser Cost savings for society How often to screen?
sessions or 500,000 Training of family
per week physicians?
Cost US$4.8 billion/year
Amblyopia Usually not reected in Denition of amblyopia? Screening may be
prevalence of blindness Different types of important because
statistics amblyopia treatment ineffective
No good prevalence Long term effectiveness School children too old?
data on amblyopia of amblyopia treatment No captive population
2% of not proven for preschool children
population (estimate Optimal length of Vision screening tests
in white population) occlusion? difcult in children
15% of unilateral No value in treatment under 3
blindness after age 6 years
Early treatment benecial
(proven)
Cataract 15 million people blind Surgery is effective ICCE vs ECCE?
from cataract Cost-effectiveness of Cataract camps?
surgery? One eye vs two?
Trachoma 5 million people blind Natural history fairly
clear
Well-dened disease
Early treatment benecial
Treatment cheap
Exam tips:
Only certain issues are highlighted here. Refer to textbooks for details.
B1037_Ch-10.indd 499 12/24/2010 4:03:16 PM

TOPIC 6
LAST MINUTE
PHYSIOLOGY
Q Opening Question: What is Visual Acuity? What is Snellen VA?

Visual acuity is the measurement of spatial resolution of the eye.
Visual Acuity
1. Minimum VISIBLE VA Letters are made of different sizes and
Determines presence or absence of target designated by distance at which letter
Threshold: 1 second of ARC subtends 5 min of ARC, e.g. letters on 20/20
2. Minimum RESOLVABLE VA (ordinary VA) line subtend 5 min of ARC when viewed at
20 feet
Determines presence of identifying/
Patient asked to recognize progressively
distinguishing feature in visible target
smaller letters or forms
Threshold: 30 sec to 1 min of ARC
3. Minimum DISCRIMINABLE (hyperacuity)
Snellen VA:
Measures minimum resolvable visual acuity
Spatial distinctions can be made at lower than
In normal observer, the minimum resolvable ordinary VA
angle is 30 sec to 1 min of ARC Determines positions of 2 or more visible
Snellen chart has letters that subtend 5 min features relative to each other
of ARC, each part (stroke) of the letter Threshold: 210 sec of ARC
subtends 1 min of ARC
Q What is Contrast Sensitivity?
Contrast sensitivity is the ability to detect slight changes in luminance/brightness.

Contrast Sensitivity
1. Contrast is a variation of brightness of an object: 2. Snellen chart is a measure of VA under
Dependent on difference in maximum approximately 100% contrast condition
brightness and minimum brightness 3. Clinical measurement: Present targets of various
A 100% contrast target is a target composed
spatial frequencies and various peak contrast
of letters printed with perfectly black ink
(i.e. totally nonreecting) on perfectly white
paper (100% reecting)
B1037_Ch-10.indd 501 12/24/2010 4:03:17 PM

Q What is Visual Adaptation?
Visual adaptation is phenomenon in which exposure of eye to light results in.

Increased spatial acuity Decreased sensitivity
Increased temporal acuity And exposure to darkness results in reverse
of above
Q Tell me about Toxoplasmosis.
Dark adaptation is the measure of rod and cone sensitivity in darkness after exposure to light.
Ability of visual system (both rod and cone mechanisms) to recover sensitivity following exposure
to light.
B1037_Ch-10.indd 502 12/24/2010 4:03:18 PM

EXPLANATION OF TERMS
Overall yield:
+ =Rarely asked in any parts of the exams. Study only if you have enough time.
++ =Uncommon question in some areas of the exams. Study to do well.
+++ =Common basic core knowledge. Expected to know principals of topic fairly well.
++++ =Important and common. Expected to know topic with a fair amount of details
included.
+++++ = Extremely important and common. Condition is usually sight threatening. Expected to
have in-depth knowledge of topic. Poor answer in this topic will likely lead to failure
in that question.
Clinical exam:
+ = Rare clinical condition of no signicance. Expected to have heard of condition.
++ = Uncommon clinical condition. Expected to describe clinical signs.
+++ = Common clinical condition. Expected to come to diagnosis or differential diagnoses.
++++ = Important and common condition. Expected to diagnose condition with ease.
+++++ = Extremely important and common. Expected to diagnose condition, exclude other
conditions, ask appropriate questions and initiate discussion.
Viva:
+ = Rarely asked.
++ = Uncommon question.
+++ = Basic core viva knowledge. Expected to know principles around the topic.
++++ = Important and common. Expected to know topic reasonably well with a fair amount of
details.
+++++ = Extremely important and common. Expected to know topic inside out. Poor answer in
this topic will likely lead to failure of that question.
Essay:
+ = Rarely asked.
++ = Uncommon question.
+++ = Basic core knowledge. Expected to know principles about the topic.
++++ = Important and common. Expected to write full-length essay on topic.
+++++ = Extremely important and common. Expected to write with enough details to get a good
score.
MCQ:
+ = Rare.
++ = Uncommon.
+++ = Common.
++++ = Very common.
+++++ = Extremely common. High-yield facts for MCQ.
Explanation of Terms 503
B1037_Explanation.indd 503 12/30/2010 1:39:32 PM

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B1037 The Ophthalmology Examinations Review FA

Introduction to Second Edition v

About the Authors vii

Section 1: Cataract and Cataract Surgery 1

Section 2: Glaucoma and Glaucoma Surgery 45

B1037_FM.indd ix 12/24/2010 2:31:17 PM

Section 6: Neuroophthalmology 267

Section 7: Oculoplastic and Orbital Diseases 345

Section 8: Uveitis, Systemic Diseases and Tumors 389

B1037_FM.indd xi 12/24/2010 2:31:17 PM

Topic 10: Posterior Segment Tumors 431

Section 9: Squints and Pediatric Eye Diseases 443

Section 10: Miscellaneous Examination Problems 481

Explanation of Terms 503

xii The Ophthalmology Examinations Review

B1037_FM.indd xii 12/24/2010 2:31:17 PM

B1037_Ch-01.indd 1 12/24/2010 2:34:07 PM

Q Opening Question: What is the Anatomy of the Lens?

Anatomy of the Lens

into anterior and posterior segment aqueous humor

Equatorial zone throughout life

lens cell bers is minimal

Q What is the Function of the Lens? Why is it Transparent?

Section 1: Cataract and Cataract Surgery 3

B1037_Ch-01.indd 3 12/24/2010 2:34:08 PM

Q What is the Embryology of the Lens?

Q How is Glucose Metabolized in the Lens?

Carbohydrate and Energy Metabolism

Glucose is rapidly metabolized via glycolysis so of lens

Q What is the Biochemical Structure of Lens Proteins?

There are 2 types of lens proteins.

4 The Ophthalmology Examinations Review

B1037_Ch-01.indd 4 12/24/2010 2:34:08 PM

Biochemical Structure of Lens Proteins

Section 1: Cataract and Cataract Surgery 5

B1037_Ch-01.indd 5 12/24/2010 2:34:09 PM

Q Opening Question: What are the Causes of Cataracts?

Q What is the Pathophysiology of Age-related Cataracts?

Pathophysiology of Age-related Cataracts

Section 1: Cataract and Cataract Surgery 7

B1037_Ch-01.indd 7 12/24/2010 2:34:09 PM

Associated with marked increase in lens Other lens metabolism changes

Q What is the Pathophysiology of Diabetic Cataracts?

There are two pathogenic mechanisms in diabetic cataracts.

Q Tell me about Galactosemia.

Q What are the Ocular Signs in Downs Syndrome?

8 The Ophthalmology Examinations Review

B1037_Ch-01.indd 8 12/24/2010 2:34:09 PM

Section 1: Cataract and Cataract Surgery 9

B1037_Ch-01.indd 9 12/24/2010 2:34:09 PM

Q Opening Question No. 1: What are the Causes of Congenital Cataracts?

Idiopathic (50% of all congenital cataracts) Ocular developmental disorders

Q Opening Question No. 2: How do You Manage Congenital Cataracts?

Section 1: Cataract and Cataract Surgery 11

B1037_Ch-01.indd 11 12/24/2010 2:34:09 PM

Parent factors (motivation of amblyopia Posterior segment

3. History vasculature, Sticklers syndrome)

Maternal infection? Optic nerve abnormalities

Drug exposure? Associated systemic anomalies

12 The Ophthalmology Examinations Review

B1037_Ch-01.indd 12 12/24/2010 2:34:10 PM

Important Issues in Cataract Management Primary posterior capsulotomy

congenital cataract surgery? fellow eye

Small eye in children more than 12 months old