CORNEA AND EXTERNAL DISEASE

1) Rule of “7’s” for the cornea

 78% hydrated
 7.8mm average radius of curvature
 70% of eye’s total refractive power (~43D)
 7 day turnover time for healthy epithelial cells
 71% collagen by dry weight
 tear film is 7 microns thick

2) Differential diagnosis of enlarged corneal nerves

DANK MEN CONTACT HERPES
1.Downs syndrome 5.MEN IIB
2.Acanthamoeba 6.Contact lens wear
3.NF1 7.HSK
4.Keratoconus *also Ichthyosis,Riley-Day,Refsums,Hansens,congenital glaucoma

3) Causes of persistent epithelial defect

MILD VITAMIN A DEFICIENCY
1.Medicomentosa 4.Dystrophy/Dry eyes
2.Infection (esp. HSK) 5.Vit A deficiency
3.Limbal stem cell deficiency *also trauma (trichiasis),neurotrophic disease,ant segment necrosis

4) Causes of neurotrophic keratopathy

SHIT MEDS
1. Systemic: diabetes,ocular ischemic syndrome, multiple sclerosis, vitamin A deficiency, CN5 stroke
2. Hereditary: Riley-Day syndrome, Moebius syndrome, Goldenhars syndrome
3. Infectious: HZO, HSK, acanthamoeba, leprosy
4. Trauma: lasik, PRP, PKP, contact lens wear, trigeminal ganglion ablation for tic doloreux
5. Meds: tetracaine, atropine, diclofenac, timolol

5) Causes of limbal stem cell deficiency

CONGENITAL ACQUIRED
Aniridia Surgery: pterygium
MEN IIB Trauma: chemical, thermal, contact lens
Ectodermal dysplasia Infectious: trachoma
*others: KID syndrome,sclerocornea Autoimmune: OCP, SJS
6) Ocular surface problems due to contact lens wear
 Sattler’s veil (central corneal edema, usually with rigid lenses)
 Microcystic epitheliopathy (aka “anoxic keratitis”)
 Toxic keratopathy (BAK, thimeresol, chlorhexidine, H2O2)
 Allergic keratoconjunctivitis (thimeresol related)
 GPC
 NVK
 Limbal stem cell deficiency

7) Causes of papillary conjunctivitis

Think “Papillary ABC’s”
1.Allergy
2.Bacterial infections
3.Contact lens wear
*also blepharitis,floppy eyelid syndrome,SLK,foreign body,toxic (Gent,tetracaine,trifluridine,all fortified abx,BAK)
*for any bilateral papillary purulent conjunctivitis, think of REITERS SYNDROME

8) Causes of acute follicular conjunctivitis (<4 weeks)

Adeno Hardly Happens to Careful Nuns
1. Adenovirus (EKC 8,11,19, PCF 3,4,7) 4. Chlamydia
2. Herpes 5. Newcastles (paramyxo)
3. Hemorrhagic conjunctivitis (coxsaki, entero)

9) Causes of chronic follicular conjunctivitis (>4 weeks)

Passing Tough Tests Mostly Means Late Cramming
1. Parinauds (Bartonella henselae,Warthin-Starry) 5. Moraxella
2. Trachoma (serotypes A-C) 6. Lyme (Borrelia burgdorferi)
3. Toxic (trifluridine,atropine,miotics,epinephrine) 7. Chlamydia
4. Molluscum (poxvirus)

10) Membranous/pseudomembranous conjunctivitis

Vast Pseudomembranous List A-I
1. Vernal 7. Diptheria
2. Pemphigoid 8. Erythema multiforme
3. Ligneous 9. Fungal (candida)
4. Adenovirus 10. Gonococcal
5. B-strep 11. HSV
6. Chemical 12. Inclusion conjunctivitis of newborn
*can also think NONINFECTIOUS: Vernal,OCP,Ligneous,SJS,Chemical and INFECTIOUS:
Adeno,HSV,Diptheria,B-Strep,Gonococcus,Candida
11) Causes of Corneal Dendrite
CHEETAH
1. Contact lens wear 5. Tyrosinemia/Toxicity of meds
2. HSV 6. Acanthamoeba/Adenovirus
3. EBV 7. HZO
4. Epithelial regen line

12) Normal flora of the ocular surface

“Think Positive!”: Gram + cocci = Staph aureus, Staph epidermidis, Strep
species (most common in kids); Gram + rods = Proprionibacterium acnes,
Corynebacterium

13) WHO Grading Scale of Trachoma

FISTO
Stage I: Follicles
Stage II: Inflammation (severe)
Stage III: Scarring/symblepharon/Arlt’s line
Stage IV: Trichiasis
Stage V: Opacification of cornea

14) Bacteria that can penetrate intact corneal epithelium

Nasty Neisseria Can Lyse Holes in Skin Fast!
1. Neisseria gonorrhea 5. Hemophilus influenza
2. Neisserie meningitides 6. Shigella
3. Corynebacterium 7. Fungus (…okay, not a bacteria)
4. Listeria

15) Signs of fungal keratitis (“Fungal Footprints”)

 Rough gray/white lesions with elevated borders
 Irregular ‘feathery’ margins
 Satellite lesions
 Overlying epithelium is intact
 Intrastromal absess
 Endothelial plaques
 *Perforation & growth through Desemets (“desmetotrophic”)
 *Growth into adjacent sclera
 Hypopyon harbours organisms (ie: is infectious whereas bacterial is sterile)
*poor prognostic indicators
16) How does atopic KC differ from vernal KC?
 AKC not seasonal
 AKC papillae rarely giant, usually small/medium
(‘velvety’ appearance)
 Milky edema of tarsal and bulbar conj more common
in AKC, in both upper & lower lids (VKC usually upper)
 Cicitrization & symblepharon more common in AKC
 Corneal scarring (‘shield ulcer’) & NVK more common
in AKC
 Keratoconus seen more commonly in AKC
 Cataract in 10% of AKC (anterior shield or PSCC)
 Less eosinophils on cytology in AKC
 VKC may be palpebral or limbal; no such distinction
with AKC
 Age of onset older in AKC, on average (VKC onset usually
childhood/adolescence with burnout in early 20’s, when AKC is usually just getting started)

 Pathogenesis is fundamentally different:

AKC: Type I hypersensitivity + decreased T-cell fxn (remember,40% of
bilateral HSK are seen in AKC pts b/c of their poor T-cell function)

VKC: Type I hypersensitivity + Type IV hypersensitivity

“AKC is the most vicious of all atopic eye conditions. It is frequently sight-threatening, with 30% of all AKC
patients having a BCVA of 20/200 or worse on presentation in my practice.” C.S. Foster

17) Causes of cicatricial conjunctivitis/symblepharon

M.I.T. IMMUNOLOGY
1. Meds: Pilocarpine,epinephrine,Timolol,idoxuridine
2. Infectious: bacterial (Niesseria,Chlamydia,B-hemolytic Strep,Corynebacterium)
viral (adeno, HZO,HSV rarely)
3. Trauma: chemical,thermal,surgical,radiation
4. Immunology: OCP,SJS,GVHD,atopic, (RARELY vernal,rosacea,scleroderma,Sjogrens)

18) Causes of interstitial keratitis (Type IV hypersensitivity rxn)

In Kenya Cogan Saw Two Laughing Hyenas Make Love
1. Cogan’s syndrome (IK,vertigo,hearing loss,negative FTA-ABS,possible PAN development)
2. Syphilis (Hutchinsons teeth,saddle nose,saber shins,perf’d palate,deafness,rhagades,retarded)

3. TB/Trachoma 5. HSV/HZO
4. Lyme/Leprosy 6. Leishmaniasis
 *classically most common cause = syphilis, nowadays most common cause = HSV by far

19) Causes of peripheral ulcerative keratitis (Type III Hypersens rxn)

1. Ocular infectious: Staph, Strep, Moraxella, HSV
2. Ocular noninfectious: SMK, Neurotrophic, Med/toxic (tetracaine, NSAID
melt), Terriens, Moorens
3. Systemic noninfectious: RA, vasculitis (PAN,Wegners,Behcets,GCA,Lupus),
rosacea, IBD, sarcoidosis, leukemia, Sjogrens
4. Systemic infectious: TB, syphilis, gonorrhea

20) Differences between scleritis (“S”) and episcleritis (“E”)

 S has deep boring pain. E just has irritation/burning.
 S is deeply violaceous. E is pinkish.
 S is tender to touch. E isn’t.
 S involves deep, criss-crossing immobile vessels. E has
vessels that are easily moved and are radially oriented to
the limbus.
 S doesn’t blanch with 10% Phenyl. E does.
 S often has marked scleral edema. E doesn’t.
 1/3 of S has uveitis. E doesn’t get associated uveitis.

21) Causes of congenital cloudy cornea

G, I’M STUMPED!
1. Glaucoma 6. Ulcer
2. Infectious (Rubella) 7. Mucolipidosis
3. Mucopolysaccharidosis 8. Peters anomaly
4. Sclerocornea 9. Endothelial dystrophy
5. Trauma 10. Dermoid

22) Stromal corneal dystrophies

Marilyn Monroe Always Gets Her Man In L.A. County
1. Macular, Mucopolysaccharide, Alcion Blue (stains blue)
* only AR inherited; Chrom #16; types I & II; blood test (ELISA) for keratan sulfate
2. Granular, Hyaline, Masson Trichrome (bright “red rock candy” throughout stroma)
* AD inherited, Chrom #5 Big-H3 gene (keratoepithelin); Types I, II, & III (I most common, III presents earliest
with recurrent erosions)
3. Lattice, Amyloid, Congo Red (stains red)
* AD inherited, Chrom #5 Big-H3 gene (keratoepithelin); Types I, II, & III (worst for recurrent erosions along with
Reis-Bucklers; Type II has systemic amyloidosis “Meretoja syndrome”; Type III rare AR inherited in older pts)
23) Corneal dystrophies on the Big-H3 gene, Chrom #5q31
LARGe is BIG
1. Lattice
2. Avellino Big-H3 gene on chromosome 5q31,
3. Reis-Bucklers codes for keratoepithelin protein
4. Granular

24) Clinical signs of keratoconus

1. Irregular myopic astigmatism 6. Prominent corneal nerves
2. Fleischer ring 7. Retinoscopy scissoring
3. Vogt’s striae 8. Unable to do keratoscopy
4. Munson’s sign 9. Apical stromal scarring
5. Rizzuti’s sign 10. Hydrops

25) Diseases associated with keratoconus

Lebers FAMED Contact lenses
1. Lebers congenital amaurosis 5. Ehlers-Danlos syndrome
2. Floppy eyelid syndrome 6. Down syndrome
3. Atopy 7. Contact lens wear
4. Marfan’s syndrome

26) Causes of crystals in the cornea

Cruising Chicks In Schnyders Gold BMW
1. Cystinosis 5. Gout/Gold (for RA)
2. Ciloxin gtts 6. Bietti’s crystalline dystrophy (RARE!)
3. ICK 7. Multiple myeloma/Monoclonal gammopathy
4. Schnyders dystrophy* 8. Waldenstrom’s macroglobulinemia
*begins in childhood, early arcus, AD inher. Chrom #1p34, 50% have elevated cholesterol, use oil red O on path

27) Causes of corneal pannus

BLU STAPH CAR
1. Blepharitis 6. Atopy
2. Limbal stem cell deficiency 7. Phlyctenule
3. Ulcer 8. HSV
4. SMK 9. Contact lens wear
5. Trachoma 10. Acid/alkali burn
11. Rosacea
28) Causes of band keratopathy
O-SHIT!
1. Ocular: uveitis, PPMD, IK, severe kerititis, phthisis, any chronic edema
2. Systemic: hypercalcemia, sarcoidosis, Paget’s, gout, hyperparathyroidism,
3. Hereditary: Norrie’s disease, congenital hypophosphatasia
4. Iatrogenic: silicone oil in aphakia
5. Toxic: mercury exposure (either occupational or from old eyedrop preps)

29) Causes of corneal verticillata

IN FACT
1. Indomethacin 4. Amiodarone
2. Naproxen 5. Chloroquine/Chlorpromazine
3. Fabry’s (alpha-galactosidase defic.) 6. Tamoxifin

30) Properties of amyloid

A,B,C,D,E,F
1. Autofluorescence (under UV light with Thioflavin-T stain)
2. Birefringence (apple-green)
3. Congo red staining/Crystal violet dye metachromasia
4. Dichroism
5. EM shows “microfilament” ultrastructure

31) Causes of scleral pigmentation

Scleral Thinning Can Make Choroid Magically Appear
1. Senile plaque 5. Congenital thinning (Ehlers-Danlos,osteo imp)
2. Thinning from prev. scleritis 6. Minocycline staining
3. CB melanoma with penetration 7. Alkaptonuria (a.k.a.ochronosis)
4. Melanocytosis

32) Hughes-Roper-Hall classification of chemical trauma

 Grade I: epithelium only, no limbal ischemia (excellent
prognosis, heals within 7 days)

 Grade II: <1/3 limbal ischemia, cornea hazy but

iris detail visible (good prognosis, heals within 3 weeks, may have transient NVK)
 Grade III: 1/3-1/2 limbal ischemia with total
epithelial loss, poor view of iris details (guarded prognosis,
heals within weeks to months, variable degree of deep & superficial pannus)

 Grade IV: >1/2 limbal ischemia, opaque cornea (poor

prognosis, persistent ulceration common, deep pannus common, failure of differentiation of ocular
surface common)
33) Admission orders for ocular chemical injury
AFTER INITIAL MNGMNT OF COPIOUS IRRIGATION UNTIL ph = 7, AND FORNICEAL DEBRIS CLEARED

 PredForte, 2gtts q1h

Intense use is CRITICAL in first 2 wks to inhibit PMN fxn and prevent future melting and pannus

 Celuvisc, 2gtts q1h

Promotes healing by providing lubrication and diluting inflammatory mediators

 Sodium ascorbate 10%, 1gtt q1h

Promotes collagen synthesis

 Sodium citrate 10%, 1gtt q1h

Chelates Ca++, thereby inhibiting PMN-mediated keratolysis

 Atropine 1%, 1gtt BID

For pain relief and stabilization of the blood-ocular barrier (mild anti-inflammatory)

 Tetracycline ung, QID

Infection prophylaxis AND collagenase inhibition

 Timolol 0.5%, 1gtt BID if necessary for IOP

 Diamox 250mg PO QID if necessary for IOP
 Sodium ascorbate (Vit C), 2grams PO QID
Promotes collagen synthesis… but potentially RENAL TOXIC at these doses

 Doxycycline 100mg PO BID

Collagenase inhibition, better tolerated than tetracycline

34) Prognosis of corneal grafts according to disease

1. GOOD: keratoconus, chronic edema (Fuch’s,PBK), scars, most
dystrophies
2. FAIR: inactive HSK, inactive pannus, 1st re-graft
3. POOR: active inflammation (ulcer,HSK,melt), active pannus, severe
surface disease (OCP, SJS, chemical trauma,KC sicca), multiple re-graft, children,
poor lid/adnexal support

35) Contraindications to corneal donor tissue use

 Death from unknown cause
 HIV, Hep B, Hep C seropositivity
 Social/clinical evidence suggestive of HIV or hepatitis
 Disseminated infectious disease (sepsis, syphilis, rabies, endocarditis)
 Chronic neurodegenerative disease (CJD, SSP, multifocal leukoencephalopathy)
 Intrinsic ocular inflammation or malignancy (BUT CHOROIDAL MELANOMA OK AAO p413)

 Intrinsic corneal disease (dystrophy, central scar, band keratopathy, pterygium)

 Previous ocular surgery (pseudophakia OK provided endothelial cell count OK)
 Active leukemia or lymphoma (systemic SOLID tumours not a contraindication)
 Endothelial cell count <2500; >12hrs death-to-preservation time (both relative)
36) DDx of a pigmented iris lesion
Foreign MEXICAN MAN
1. Foreign body 7. Atrophy
2. Melanoma 8. Nevus
3. Ectropion uveae 9. Metastasis/Medulloepithelioma
4. Xanthogranuloma 10. A/C lens material
5. Inflammatory nodule 11. Neurofibroma
6. Cyst

37) Types of viruses affecting the eye

 Herpes simplex: blepharitis, conjunctivitis, keratitis, uveitis, ARN
 Herpes zoster: same as HSK + cavernous sinus inflammation
 Adenovirus: conjunctivitis, keratitis
 Picornavirus (coxsacki- and entero-): hemorrhagic conjunctivitis
 Poxvirus (molluscum): blepharo-conjunctivitis
 Paramyxovirus: Newcastle’s conjunctivitis
 Human papillomavirus: papilloma, CIN, SCC
 Rubella (RNA togavirus): cataract, keratitis, glaucoma, retinopathy
 Rubeola (measles): interstitial keratitis
 Mumps: interstitial keratitis
 CMV: hemorrhagic necrotic retinitis
 EBV: dacryoadenitis, keratitis
 Herpes simplex-8: Kaposi’s sarcoma
 HIV: retinopathy + lots of opportunistic infections
 OCULOPLASTICS
1) Causes of proptosis in adults
I BLAME Thyroid!
1. Inlammatory: Cellulits, Idiopathic, Sarcoidosis, Wegener’s, ruptured dermoid
2. Benign neoplastic: Cavernous hemangioma, lymphangioma,
hemangiopericytoma, unruptured dermoid, neurogenic tumours (meningioma,
Schwannoma, Glioma)
3. Lacrimal Gland: BMT, adenoid cystic carcinoma, inflamm, lymphoma
4. A-V Fistula: high-flow, or low-flow
5. Malignant neoplastic: Mets, Lymphoma
6. Extra-orbital infiltrating tumours: SSC, nasopharyngeal ca, mucocele
7. THYROID #1 CAUSE OF ALL!!!
*can also think NEOPLASTIC: Mets,Lymphoma,Adenoid cystic ca, Benign mixed tumour of LG, Cav
hemangioma, Lymphangioma, AV fistula, neurogenic tumours (meningioma,Schwannoma,glioma), extra-orbital
infiltrating tumours (SCC, nasopharyngeal ca, mucocele) and INFLAMMATORY: Thyroid, Cellulitis
(bact,viral,fungal), IOIS, Wegener’s, Sarcoid

2) In the orbits of adults, most common cause of…

 Unilateral proptosis = TRO
 Bilateral proptosis = TRO
 Benign solid tumour = cavernous hemangioma
 Malignant tumour = mets (women breast, men lung)
 Primary orbital malignancy = lymphoma

3) Causes of proptosis in kids

RICHLy GOLDeN Histiocytoma
1. Rhabdomyosarcoma 6. Glioma
2. Idiopathic inflammatory syndrome 7. Other sarcomas
3. Cellulitis 8. Leukemia
4. Hemangioma 9. Dermoid
5. Lymphangioma 10. Neuroblastoma
11. Histiocytoma

4) In the orbits of kids, most common cause of…

 Unilateral proptosis = cellulitis
 Bilateral proptosis = idiopathic orbital inflamm
 Benign solid tumour = capillary hemangioma
 Primary orbital malignancy = rhabdomyosarcoma
 Secondary orbital malignancy = neuroblastoma
5) Causes of pseudo-proptosis
Bupthalmos is the APPLE of my eye!
1. Buphthalmos 4. Paralysis of EOMs
2. Anisometropia 5. Lid retraction
3. Ptosis of the other eye 6. Enophthalmos of the other eye

6) Proptosis associated with pain

 Any inflammatory/infectious process
 Metastasis
 Adenoid cystic carcinoma of the LG
 Nasopharyngeal carcinoma
 Osteogenic sarcoma
 High-flow fistula
 Acute intraconal hemorrhage

7) Orbital tumours to be excised In-Toto,without biopsy

 BMT of the lacrimal gland
 Hemangiopericytoma
 Histiocytoma

8) Orbital tumours causing bony destruction

 Adenoid cystic carcinoma of the LG (common)
 Metastases (common to all types, but especially prostate)
 Histiocytoma
 Meningioma (may also cause hyperostosis)
 Osteogenic sarcoma
 Burkitt’s lymphoma (other lymphomas very rarely)
 Wegener’s granulomatosis

9) Reasons to do Enucleation, Evisceration, Exenteration

Enucleation: 1) intraocular malignancy; 2) NLP eye with cloudy cornea
suspected of having a malignancy; 3) blind painful eye; 4) phthisis;
5) severely traumatized eye (if ANY useful VA, try primary closure first, discuss enuc in 14 days)
Evisceration: 1) post-endophthalmitis NLP eye; 2) blind painful eye
NOT due to tumour (ADV: don’t spread bugs, better motility DISADV: persistent pain, theoretic SO)
 Evisceration: 1) intraocular melanoma or RB with extra-scleral
extension; 2) malignant tumours of the lacrimal gland; 3) orbital
malignancy not responding to treatment (mostly sarcomas); 4) fungal infections
(esp mucor); 5) destructive tumours extending from adjacent structures
(TYPES: 1)Subtotal leaves lids & periosteum; 2)Total; 3)Radical takes bones too)

10) Complications of the anophthalmic socket

1. Deep superior sulcus 6. Infection of implant
2. Contraction of fornices 7. Anophthalmic entropion/ectropion
3. Contraction of socket 8. Anophthalmic ptosis
4. Extrusion/exposure of implant 9. Chronic irritation/granuloma formation
5. Migration of implant 10. Cosmetic imperfection

11) Lid lesions with malignant potential

1. Actinic keratosis (SCC)
2. Keratoacanthoma (SCC)
3. Lentigo maligna (malignant melanoma)
4. Nevus (malignant melanoma, RARE and only in ACQUIRED nevi)
5. PAM (highly suspicious for melanoma if PAM extends onto lids)
6. Recurrent chalazion (sebaceous cell ca)

12) Lid lesions as markers of internal malignancy

1. Oculodermal melanocytosis (1/400 have choroidal melanoma)
2. Sebaceous hyperplasia (if multiple, Muir-Torre syndrome = adeno ca of colon)
3. Seborrheic keratosis (if multiple, Leser-Trelat syndrome = any malignancy)
4. Molluscum contagiosum (if multiple, consider HIV/AIDS)
5. Tricholemmomas (if multiple, Cowden’s syndrome = breast,thyroid,gastro ca)
6. Mucosal neuroma (if multiple, MEN IIB = pheochromocytoma, thyroid ca)
7. Kaposi sarcoma (AIDS)
8. Amyloid deposits (multiple myeloma, leukemia)
9. Acanthosis nigricans (rare around lids, but may signify GI malignancy)

13) Types of Ectropion (examine anterior lamella)

1. Congenital rare
2. Involutional Mild: medial spindle, conj cautery; Severe: LTS, Bick, Reinsertion of retractors
3. Paralytic CN VII palsy; check K sens + tearing + lagophthalmos; Mild: lubrication, lid taping;
Moderate: consider LTS + gold weight + brow plasty; Severe: tarsorrhaphy
4. Cicatricial ant lamellar scarring; 3 Step Repair:1)Incise/relax scar, 2)LTS, 3)Lengthen lamella with FTSG
5. Mechanical due to excess skin, mass, or glasses; excise + LTS if lid/tendon stretched out

14) Types of Entropion (examine posterior lamella)

1. Congenital rare
2. Involutional 3 Key Features: 1) Lid laxity: poor snapback, >6mm distraction 2) Retractor disinsertion:
deep fornix, high-riding lower lid, white sub-conj line, no lid movement on downgaze 3) Over-riding pre-septal
orbicularis seen best after squeeze; Rx: Mild: Quickert sutures; Severe: LTS, retractor reattachment, Weiss
procedure
 3. Spastic chronic ocular irritation or CNS disease; Rx underlying cause as appropriate!!, lid taping, Botox,
Quickert’s sutures, LTS
4. Cicatricial forniceal foreshortening + symblepharon (“M.I.T Immunology”), Mild: BCL, epilation/cryo,
lubrication; Moderate: tarsal fracture with everting sutures; Severe: mucus membrane/amniotic membrane graft
with cartilage/sclera for support ***DO NOTHING IF ACTIVE INFLAMMATION PRESENT***

15) Types of Ptosis (VPF, MRD, lid crease, levator fxn)

1. Pseudoptosis microphtalmia, enophthalmos, phthisis, hypertropia, dermatochalasis, contra lid retract
2. Congenital maldev levator; poor elevat + lagophth downgaze; R/O Horners, CNIII palsy, MG, jaw-wink
3. Involutional Causes = age, recurrent lid edema, steroid use, CL wear, chronic eye rubbing, SR traction
suture, fatty infiltration; Signs = high lid crease, good LF > 15mm, only 4-5mm ptosis, can see iris thru lid
4. Neurogenic Causes = Horners, CN III palsy, Marcus-Gunn, MS, trauma, brain surgery
5. Myogenic Causes = MG, CPEO, myotonic dystrophy, muscular dystrophy, oculopharyngeal dystrophy
6. Mechanical Causes = plexiform neurofibroma, chalazion, hemangioma, malignancy, edema
7. Traumatic may result in a combo of the above etiologies

16) Signs of Thyroid Related Ophthalmopathy (TRO)

No signs/symptoms Resistance to retropulsion
Only signs (lid retraction, lid lag, lagophthalmos) Edema of the conjunctiva
Soft tissue signs & symptoms Lacrimal gland enlargement
Proptosis Injection of the conj and caruncle
EOM involved Edema of the lids
Cornea involved Fullness of the lids
Sight threatened
 CATARACT and SURGERY
1) Brief embryology of lens development
 25 days: out-pouchings from diencephalon form optic vesicles
 27 days: cells thicken to form lens plate
 29 days: lens plate indents to form lens pit
 33 days: lens pit deepens and separates to form lens vesicle
 40 days: primary lens fibers fill vesicle = embryonic nucleus
 1 month: post. hyaloid artery forms posterior vascular capsule
 2 months: anterior ciliary arteries form anterior vascular capsule;
together these vascular capsules form the tunica vasculosa lentis
 2-8 months: secondary lens fibers form fetal nucleus; fiber
growth is ant. to post. with interdigitations forming Y-sutures
 3 months: zonules are secreted by ciliary epithelium

2) Some points on lens anatomy and physiology

 Size at birth: 6.4mm wide, 3.5mm deep, 90mg
 Adult size: 9mm wide, 5mm deep, 250mg
 Index of refraction: 1.4, decreases with age b/c more insoluble protein
 Power: 15D – 20D
 Ionic composition: Sodium 20mM (AC 120mM), Potassium 150mM (AC
5mM); maintained via ATP-dependent pump-leak mechanism; Calcium
30mM (AC 2mM)
 Protein composition: 33% protein by weight; 80% water-soluble
(CRYSTALLINS: alpha largest 24%, beta most numerous 55%, gamma smallest 1.5%) ; 20% water-
insoluble (CYTOSKELETAL proteins are urea-insoluble, PLASMA MEMBRANE proteins are urea-soluble)
 Cataract composition: decreased water-soluble proteins from 80%
down to 50%; brunescent cataracts may have 90% insoluble proteins with
decreased glutathione and increased oxidative changes and disulfide
bonds
 Protective mechanisms: enzymes that protect against free-radical and
oxidative damage include glutathione peroxidase, superoxide
dismutase, and catalase; other protective “free-radical scavengers”
include vitamin E, ascorbic acid, and glutathione
 Metabolism: glucose enters via simple diffusion and facilitated diffusion;
converted to glucose-6-phosphate by hexokinase before entering one of 3
pathways: 1) Anaerobic glycolysis (burns 78% of glucose, but very inefficient giving only 2
 ATP per glucose molecule);2) Hexose monophosphate shunt (burns 5% of glucose);
3) Aerobic glycolysis (burns 3% of glucose but gives you 25% of all ATP b/c generates 36 ATP
per glucose molecule); hexokinase is a very slow molecule, resulting in some
glucose being shunted to a fourth pathway: 4) Sorbitol pathway (burns 5 % of
glucose, generating sorbitol as an intermediate product before fructose, together acts as an osmotic sponge if
present in large quantities, as in DM)

3) Some points on accommodation

 Ciliary muscle contracts, zonules relax, lens becomes fatter
 Most of lens change occurs at central anterior surface
 4 stimuli can induce accommodation: 1) Known or apparent
size/distance of an object; 2) Blur; 3) Chromatic aberration; 4)
continual oscillation of ciliary tone
 The amount of change in the eye’s refractive power induced by
accommodation is the accommodative amplitude for that eye; normal in
youth is 12D-18D, adults age forty is 4D-8D, over age 50 is <2D
 Can measure accommodative amplitude 3 ways: 1) Find near-point with
refractive Rx in place; 2) Measure with Prince rule; 3) Method of
spheres

4) Systemic disease associated with cataract

1. Diabetes (nuclear most common, cortical intumescent also)
2. Myotonic dystrophy (polychromatic cataract, ptosis, tight grip, frontal balding, heart conduction defects)
3. Galactosemia (def. in transferase = “classic”, 75% get oil-droplet cataract, or kinase or epimerase)
4. Wilson’s disease (pigmentation and sunflower cataract)
5. Fabry’s disease (glycolipidosis, alpha-galactosidase def., XLR, K-verticillata, conj. & ret. tortuosity,
heart/kidney/brain vascular anomalies; burning hands and feet)
6. Atopic dermatitis (ASCC, shield-like)
7. Hypoparathyroidism
8. Marfan’s syndrome (cataract + subluxation, classically up & out)
9. Alport’s syndrome (anterior lenticonus, hemorrhagic nephritis, deafness)
10. Lowe’s syndrome (post. lenticonus + microphthalmos + glaucoma, nephritis, hypotonia, retardation)
11. Congenital rubella (complete dense white cataract, harbours infectious viral particles for years)

5) Ocular disease associated with cataract

1. Myopia 7. Peter’s anomaly
2. Uveitis 8. Axenfeld-Reiger syndrome
3. RP (PSCC) 9. Intraocular tumour (esp melanoma)
4. Aniridia (polar or nuclear) 10. Intraocular foreign body (iron, copper)
5. Microphthalmia 11. Any previous ocular surgery, esp for RD
6. PHPV
6) Trauma and cataracts
1. Blunt trauma (Vossius ring, stellate PSCC, nuclear, luxation)
2. Penetrating trauma (intumescence)
3. Surgical trauma
4. Radiation (X-ray 200 rad minimum (CXR = 0.1 rad); infrared glassblowers; UVB (not UVA))
5. Chemical (common with alkali injury)
6. Electrical (lightning ASCC + PSCC, industrial PSCC)
7. Foreign body retention (iron depositis in epithelium, copper in basement membranes)

7) Drug-Induced cataracts
CAMP
1. Corticosteroids ( PSCC 30% - 80%, higher with higher doses, sometimes reversible in kids)
2. Amiodarone (ASCC + pigmentation)
3. Miotics (20% Pilo, 60% Phospholine iodide, over 5 years)
4. Phenothiazines (esp chlorpromazine, ASCC + pigmentation)

8) Causes of PSCC cataracts

STRIPPER
1. Steroids 5. Pilocarpine
2. Trauma 6. Phospholine iodide
3. Radiation 7. Electricity
4. Inflammation 8. Retinitis pigmentosa

9) Causes of ectopia lentis

SHE-MALE WIG Trauma
1. Sulfite oxidase deficiency (sulphur metab problem b/c low hepatic molybdenum cofactor ; seizures,
retarded)
2. Homocystinuria (AR inher; 90% get lens dislocation, due to low serum homocystine ; thromboembolic
occlusions, cardiomegally, HTN ; RX = high cystine, low methionine diet + B6 supplement benefit 50%)

3. Ehlers-Danlos (blue sclera, angioid streaks, risk of RRD and corneal rupture)
4. Marfan’s syndrome (AD inher, chrom #15, 50-80% get lens dislocation, due to defect in fibrillin, tall,
arachnodactyly, scoliosis, chest-wall defects, dilated aortic root and dissection common; also get high myopia,
risk of RRD and vitreous loss during surgery; RX = cardiology referral, dilated exam, oral Abx prior to sx to
prevent endocarditis)
5. Aniridia (2/3 AD inher, 1/3 sporadic and assoc’d with Wilm’s tumour, GU defects, and retardation ; chrom #11
PAX-6 gene)
6. Hyperlysinemia (rare; high serum lysine with retardation + hypotonia)
7. Ectopia lentis et pupillae (AR inher; oval/slit pupils bilaterally; cataracts + glaucoma)
8. Weill-Marchesani (AR inher; 90% get lens dislocation, usually forward, ‘anti-Marfan’s’ = short stature,
stubby fingers, broad hands, poor joint flexibility, microspherophakia + high myopia + glaucoma; RX =
cyclopegics + laser PI)
9. Idiopathic
10. Glaucoma (both congenital and PXFG)
 11. Trauma

10) Associated conditions with microspherophakia

Weill-Marchesani CRAMP
1. Weill-Marchesani 5. Alport’s
2. Congenital 6. Marfan’s
3. Rubella 7. Peter’s

11) Causes of Infantile/Childhood cataracts

MIDNIGHT
1. Metabolic (galactosemia) 5. Inflammation
2. Infection (rubella) 6. Genetic syndrome (aniridia, Marfan’s)
3. Developmental (PHPV) 7. Hereditary (posterior polar)
4. Nutritional 8. Trauma

12) Workup for childhood cataract

 Unilateral: TORCH screen + VDRL/MHA-TP/FTA-ABS
*not inherited, not metabolic! 80% idiopathic, 10% traumatic, 10% non-inherited developmental
(PHPV, posterior lenticonus)

 Bilateral: examine parents & sibs; TORCH screen +

VDRL/MHA-TP/FTA-ABS; urine non-reducing substances after milk
(galactosemia); urine heme/protein (Alport’s); urine amino acids (Lowe’s); blood
glucose (diabetes), blood galactokinase (galactosemia); blood calcium
(hypoparathyroid)
*60% idiopathic, 30% hereditary, 10% metabolic (diabetes, galactosemia, hypoglycemia,
hyperparathyroid, Fabry’s) or part of genetic syndrome (Lowe’s, Alport’s, Marfan’s, Aniridia, Myotonic
dystrophy)

13) Symptoms of cataract

 Decreased visual acuity
 Glare
 Decreased contrast sensitivity
 Myopic shift/anisometropia
 Monocular diplopia

14) Medical management of cataract

 Refraction
 Increased ambient lighting
 Pupil dilation
 Contast enhancing lenses
  Telescopes/magnifiers

15) Tests of macular function

 Pinhole refraction
 PAM
 Laser fringe interferometry (not useful in <20/200 VA, may miss small macular lesions)
 Maddox rod test
 Photostress recovery test (normal = 27s + 11s; <50s in 99% of normal eyes)
 Blue light entoscopy (a.k.a “flying corpuscle test”)
 Purkinje’s entoptic phenomenon (ability to perceive own retinal vascular shadows)
 ERG
 VEP

16) Complications of retrobulbar anaesthesia

1. Hematoma/Ecchymosis 6. Globe penetration
2. Retrobulbar hemorrage 7. CRAO
3. Ptosis 8. Optic nerve trauma
4. Strabismus 9. I.V. injection (arrhythmia)
5. Glaucoma 10. Intradural injection (seizures, resp arrest, brainstem)

17) Management of malignant hyperthermia

1. Stop surgery, D/C anaesthetic!! 6. Monitor urine output
2. 100% oxygen 7. Monitor cardiac status
3. Dantrolene 5mg/kg IV 8. Monitor electrolytes
4. Two large bore IV’s give cooled NaCO3 9. Insulin for hyper-K
5. Actively cool pt

18) Risk factors for expulsive choroidal hemorrhage

1. Old age 6. Bleeding diatheses
2. Uncontrolled glaucoma 7. Myopia
3. HTN 8. Prolonged hypotony
4. Atherosclerosis 9. Recent trauma/inflammation
5. Blood thinners 10. Previous expulsive hemorrhage

19) Notes on viscoelastics

 Purposes: maintain space, improve visibility, improve maneuverability,
improve stability of anterior chamber, protect tissues
 Properties: non-toxic, non-inflammatory, clear, IOP-neutral,
  Descriptive terms: 1) Viscoelasticity = at high energy frequencies
substance acts as a gel, at low energy frequencies it acts as a more
viscous compound (i.e. is “elastic” behaviourally); 2) Viscosity =
resistence to flow or shearing forces (higher MW = more viscosity, better cohesion); 3)
Pseudoplasticity = ability to transform from a gel to a liquidlike
substance under conditions of pressure; 4) Surface tension =
determines coating ability (lower surface tension = better coating, lower contact angle)
 Types: two general types: 1) Cohesives = higher molecular weight,
higher surface tension, higher pseudoplasticity, adhere to themselves
better therefore easy to remove (ex: Healon, Provisc, Amvisc) generally
better for space maintenance, like during capsulorrhexis; 2) Dispersives = lower
molecular weight with low surface tension, therefore better coating
ability (ex: Viscoat, Ocucoat) generally better for tissue protection, like soft-shell technique
during capsulorrhexis for endothelial protection, or to tamponade vitreous face during cortical cleanup
following capsular rupture

20) Types of phacoemulsification machines

 Parastaltic pump: once tip is occluded vacuum is created by a set of
rollers moving along a flexible tube, thereby forcing (or “milking”) fluid
through the tubing and creating a relative vacuum at the aspiration
port; results in a RAPID LINEAR STEPWISE rise in vacuum,
proportional to the speed of the rollers
 Venturi pump: creates a vacuum via gas flowing across a port, the
strength of the vacuum is proportional to the speed of the gas (Venturi
Principle); results in a RAPID LINEAR rise in vacuum that is
INDEPENDENT OF TIP OCCLUSION
 Diaphragm pump: a flexible diaphragm overlies a fluid chamber with
one-way valves at the inlet and outlet; with occlusion of the
aspiration tip, the diaphragm moves out causing pressure in the
chamber to drop and fluid to flow into the chamber; the relative vacuum
in the chamber also causes the one-way exit valve to close; results in
an INITIALLY SLOW RISE IN VACUUM that, with continued
occlusion, continues to RISE IN AN EXPONENTIAL manner

21) Complications of cataract surgery

 Intraoperative: Most serious: Complications of retrobulbar,
Complications of GA, Wrong eye/wrong patient, Retrobulbar
hemorrhage. 1. Poor incision with persistent leak or iris prolapse; 2.
Corneal abrasion; 3. Poorly dilating pupil; 4. Hyphema; 5. Iris
incarceration; 6. Shallow anterior chamber; 7. Posterior effusion
syndrome; 8. Endothelial damage; 9. Incomplete/uncontrolled
capsulorrhexis; 10. Zonulolysis; 11. Capsular rupture; 12. Dropped
 nucleus; 13. Vitreous presentation; 14. Suprachoroidal hemorrhage;
15. Phaco burn to wound; 16. Wrong IOL implanted
 Postoperative: Most serious: Endophthalmitis, RRD, Glaucoma,
Aqueous misdirection. 1. Astigmatism; 2. Persistent corneal edema
(Brown-MacLean syndrome following ICCE); 3. Descemet’s detachment; 4. Epithelial
downgrowth; 5. Wound leak; 6. Surface toxicity to meds; 7. Aggressive
inflammation; 8. Elevated IOP/glaucoma progression; 9. Cyclodialysis
cleft; 10. Hypotony; 11. Retained nuclear/cortical fragment; 12.Posterir
capsular block/retained viscoelastic; 13. Pupillary capture of PCIOL;
14. Capsular phimosis; 15. PCO; 16. PCIOL decentration/tilt;
17. Reverse pupil block (for ACIOL with inadequate surgical PI); 18. CME; 19. RRD;
20. Progression of retinopathy/AMD; 21. Retinal light toxicity;
22. Endophthalmitis; 23. Aqueous misdirection syndrome (malignant
glaucoma); 24. PBK; 25. UGH (usually with ACIOL of improper size)

22) Lidocaine toxicity

 Signs include drowsiness, delirium, disorientation, dysarthria,
circumoral and tongue numbness, metallic taste, vertigo, tinnitus,
nausea, tachypnea, tachycardia, twitching extremities, eventual
seizures followed by CNS and CVS depression
 Maximum safe dose without epinephrine = 4mg/kg; so a 70kg adult
should receive no more than 300mg (or 30cc) of a 1% soln (10mg/cc)
 Maximum safe dose with epinephrine = 7mg/kg; so a 70kg adult should
receive no more than 500mg (or 50cc) of a 1% soln (10mg/cc)
 PEDIATRICS

1) Types of esodeviations
PANIC
1. Pseudo-ET (wide flat nasal bridge, small IPD, epicanthal folds)
2. Accomodative acquired (refractive, non-refractive high AC/A, mixed)
3. Non-accomodative acquired (see below = “A,B,C,D’ S”)
4. Incommitent (CN VI palsy, Duane’s, Moebius, orbital #, cong fibrosis syndrome, Graves, MG)
5. Congenital

2) Types of non-accomodative acquired esodeviations

A,B,C,DD’SS
1. Acute ( usually following viral illness, stress)
2. Basic (>6m old, no hyperopia/accom factors, healthy child…must R/O lesion with CT)
3. Cyclic (very rare, ET manifests only every 24-48hrs, eventually becomes constant)
4. Deprivation (sensory that is) (K scar, cataract, retinal path, anisometropia)
5. Divergence insufficiency (hard to tell from mild CN VI palsy—CT head!)
6. Surgical (overcorrection or slipped muscle from XT surgery)
7. Spasm of near synkinetic reflex (most common in myasthenia and malingerers)

3) Types of intermittent exodeviations

B,C,D’S
1. Basic (XT = XT’)
2. Convergenve insufficiency (XT < XT’)
3. Divergence excess (XT > XT’)
4. Simulated divergence excess (initially XT > XT’, but following occlusion XT = XT’)

4) Traits of the monofixation syndrome

 Small angle strabismus (<8 prism diopters)
 Central scotoma with peripheral fusion
 Good horizontal fusion amplitudes
 Persistence of phoria no matter how much you chase it with prism (i.e.
“large latent phoria”)
*amblyopia common, but always have some degree of binocularity, and prognosis for long-term alignment excellent

5) Four ways to differentiate congenital vs. acquired CN IV palsy

1. Facial asymmetry 3. Large vertical fusion amplitudes
2. Look at old photos 4. S.O. tendon is long & floppy at surgery
6) Seven ways to differentiate bilateral vs. unilateral CN IV palsy
1. positive head tilt OU 5. V-pattern ET on downgaze
2. >10D excyclotorsion 6. objective excyclotorsion OU
3. chin-down head position 7. bilateral I.O. overaction
4. decreased S.O. ductions OU

7) Causes of vertical diplopia/deviations

Four Grave TOMBS
1. Fourth nerve palsy
2. Grave’s
3. Third nerve palsy
4. Overacting muscles (IO or SO)/Orbital disease (IOIS, trauma, tumour, thyroid)
5. Myesthenia gravis/Miller-Fisher variant of Guillen-Barre
6. Brown’s syndrome
7. Skew
* also LEASH EFFECT in Duane’s, DVD, DOUBLE ELEVATOR PALSY, and LAMBERT-EATON syndrome in adults

8) Four features distinguishing DVD from I.O.O.

1. Doesn’t obey Herring’s Law
2. No excyclotorsion
3. No V-pattern
4. Hyper seen in primary AND ab/adduction (I.O.O. only hyper in adduction)

9) Some points on A & V pattern strabismus

 A-pattern = 10 difference btwn 250 upgaze & 250 downgaze
 V-pattern = 15 difference btwn 250 upgaze & 250 downgaze
 I.O.O. gives V-pattern (think IOVS), S.O.O. gives A-patterns; horizontal
muscle dysfunction usually gives V-patterns
 Surgical correction involves vertical transposition of horizontal rectus
muscles M.A.L.E. = Medial rectus to Apex, and Lateral rectus to the
Empty space

10) Causes of ptosis in kids

Believe Me, Too Much Horny Giggling Can Create Crazy Mayhem!
1. Blepharophimosis 6. Graves
2. Myesthenia 7. CPEO
3. Third nerve palsy 8. Congenital fibrosis syndrome
4. Marcus-Gunn jaw wink 9. Congenital
5. Horners 10. Moebius syndrome
11) Complications of strabismus surgery
 Minor complications: redness, dellen, exposed suture, suture
granuloma, conjunctival cyst, post-op N&V
 Sensory complications: refractive changes (with-the-rule astigmatism with sx on 2
horizontal rectus muscles), diplopia, loss of fusional ability, new suppression
scotoma, amblyopia
 Motor complications: over/undercorrection, new deviation involving
other muscles (very common, ~20% will require a second operation due to these two),
change in upper/lower lid position (with vertical muscle sx), slipped muscle, lost
muscle, fibrosis/adherence syndrome
 Serious complications: malignant HTN (metabolic disorder of Ca++ being released
from sarcoplasmic reticulum in response to inhaled anaesthetic + muscle relaxants; SIGNS = high end-
tidal CO2, tachycardia, tachypnea, sweating, trismus, drop in BP, renal failure, metabolic acidosis, muscle
rigidity, and TEMPERATURE ELEVATION; see surgery note #15 for Rx),oculo-cardiac reflex,
globe penetration + sequelae (occurs in /1000, but only ~1/20,000 get RRD, VH, etc),
endophthalmitis, infection, anterior segment ischemia ( RISK FACTORS:
multiple muscle surgery, DM, HTN, thyroid, elderly, anemia, smoker, re-operation; SIGNS: K-edema,
uveitis, correctopia, choroidals; RX: steroids; PREVENTION: staged procedure, vessel-sparing technique,
transpositions instead of resections, perform EOM angiography prior to surgery )

12) Types of congenital nystagmus (onset <3 months)

Some Pupils Like Motion
1. Sensory 3. Latent
2. Periodic-alternating (albinism) 4. Motor

13) Causes of sensory nystagmus

C2 M2 A4
1. CSNB 5. Aniridia
2. Cataract 6. Achromatopsia
3. Myopia 7. Albinism
4. Macular hypoplasia 8. Amaurosis (of Leber)

14) Causes of paradoxical pupil response

CALOR
1. CSNB 4. Optic nerve hypoplasia
2. Achromatopsia/Aniridia 5. Retinitis pigmentosa
3. Leber’s congenital amaurosis

15) Types of acquired nystagmus (onset >3 months)

Some Classes Of Movements Sound Vexing
1. Spasmus nutans 4. Monocular
2. Convergence-retraction 5. See-saw
3. Opsoclonus 6. Vertical
16) Causes of vertical nystagmus
LAMB SCAM
1. Lithium 5. Stroke
2. Anticonvulsants 6. Cerebellar degeneration
3. Magnesium deficiency 7. Arnold-Chiari malformation
4. B12 deficiency 8. Meningitis/MS

17) DDx of dacryocystocele

Dirk Diggler Had Ridiculously Large Erections
1. Dacryocystocele 4. Rhabdomyosarcoma
2. Dermoid 5. Lymphangioma
3. Hemangioma 6. Encephalocele

18) DDx of Axenfeld-Reigers syndrome

1. Aniridia 4. I.C.E. syndrome
2. Peter’s anomaly 5. Congenital ectropion uveae
3. PPMD 6. Ectopia lentis et pupillae

20) Developmental ocular abnormalities with glaucoma

Many Conditions Adversely Affect Little Peoples Pressures
1. Microphthalmia 4. Lens dislocation
2. Cornea plana 5. Peter’s anomaly
3. Aniridia 6. PHPV
4. Axenfeld-Reigers
*SYSTEMIC CONDITIONS associated with glaucoma = Sturge Weber, NF-1, Lowes syndrome
*OTHER OCULAR DISEASE associated with glaucoma = ROP, trauma, uveitis, steroid use, tumour

21) Conditions with epiphora & photophobia & blepharospasm

(i.e.mimic the “classic triad” of congenital glaucoma sx’s)
ACUTE Nasolacrimal duct obstruction
1. Abrasion 4. Trauma
2. Conjunctivitis 5. Ectropion/entropion
3. Uveitis 6. NLD

22) Conditions with corneal enlargement

Corneal MAGnification
1. Megalocornea
2. Axial myopia
3. Glaucoma
23) Causes of optic nerve cupping in kids (other than glaucoma)
PHACO the Optic nerve
1. Physiologic
2. Hypoplasia (de Morsiers) …of the optic nerve
3. Atrophy (Kjers, Behrs)
4. Coloboma

24) Causes of iris heterochromia

FILTHy SPERM WIN
1. Fuch’s 9. Essential iris atrophy/ectropion uveae
2. Idiopathic 10. Rubeosis
3. Leukemia 11. Melanocytosis
4. Trauma 12. Waardenberg’s syndrome
5. Horner’s 13. Inflammation (chronic)
6. Siderosis 14. Nevus
7. Prostaglandin

25) Causes of exudative retinopathy in childhood

Coats Affects Prospective Little FATHERS
1. Coat’s (sporadic, 90% unilateral, 80% boys) 7. Toxocariasis (serum eosinophilia)
2. Angiomatosis retinae (VHL) 8. Hemangioma
3. PHPV (microphthalmia, cataract, glaucoma, RD) 9. Eele’s
4. Leukemia 10. Retinoblastoma/ROP
5. FEVR 11. Sickle cell
6. Anemia

26) Treatment of retinopathy of prematurity

THRESHOLD DISEASE: 3 + II = 5
 Stage 3 disease (raised ridge with active neo), with “PLUS” disease, in
zone II (or I), over five contiguous clock hours or eight interrupted clock
hours
 Screen all babies <1500g and <32 weeks gestation at one month of
age, then q2 weeks until vessels reach zone III
 If ROP detected, follow q1 week until regression occurs (happens in
85%) or until threshold met
 CRYO-ROP study shows Rx decreases progression and unfavourable
outcomes by 50% (22% vs 42%), but BCVA is equivalent at 5 and 10
years; surgery in stage 4 & 5 ROP successfully reattaches retina in
1/3, but only 10% get ambulatory VA)
 Complications of ROP = amblyopia, strabismus, cataract, TRD,
myopia, ACG, macular dragging, retinal folds, phthisis (5% of babies
undergoing Rx will have cardio-respiratory arrest)
27) Causes of leukocoria in kids
Reading Pornography Regularly Can Cause Very
Uncomfortable Chaffing To Tossers
1. Retinoblastoma 6. VH
2. PHPV 7. Uveitis
3. ROP 8. Coloboma
4. Coat’s 9. Toxocariasis
5. Cataract 10. Tumour

28) Types of oculo-renal syndromes

RenAL
1. Renal-retinal syndrome: AR inher, interstitial nephritis + RP (OCULAR = RP)
2. Alport’s syndrome: AD inher, hematuria + renal failure + HTN + deafness (OCULAR = Ant erior
lenticonus, Anterior polar cataracts, Fundus albipunctatus)
3. Lowe’s syndrome : XLR inher, proteinuria/hematuria/amino aciduria, rickets, retarded, areflexic
(OCULAR = Posterior polar cataract, Posterior lenticonus, Glaucoma)

29) Causes of blindness in a baby with a normal exam

FOCAL AXIS
1. Foveal hypoplasia 6. Albinism
2. Optic nerve atrophy/hypoplasia 7. X-linked retinoschisis
3. Cone dystrophy 8. Intracranial pathology
4. Achromatopsia 9. Stationary night blindness
5. Leber’s congenital amaurosis
* because #8 cannot be ruled out on exam, all kids with poor vision and a normal exam get a CT head,
and whenever an anterior sensory problem is not found (i.e. cataract, etc.), all get an ERG + VEP as well
** causes of #8 = hydrocephalus, hemorrhage, tumour, encephalitis, hypoxic damage

30) Causes of optic nerve hypoplasia

IQ de SALAD
1. Idiopathic 5. Alcohol
2. Quinine toxicity 6. LSD
3. de Morsier’s syndrome 7. Aniridia
4. Seizure meds 8. Diabetes (during pregnancy)
* ON hypoplasia mostly caused by drugs/alcohol, but ~10% will have CNS abnormalities like absent corpus
collosum, absent septum pellucidum, pituitary hyoplasia, and seizures = de Morsier’s syndrome; suspect it in
kids who are small or have a hx of seizures; order MRI & endocrine workup

31) Causes of optic atrophy in kids

MOTHRA Trauma
1. Metabolic: gangliosidoses, MPS, Charco-Marie-Tooth disease
2. Optic neuritis: burned out
3. Tumour/toxic: glioma, craniopharyngioma
 4. Hereditary: Kjer’s (AD, gradual onset in preschool), Behr’s (AR, retarded, ataxia, spastic), Leber’s
(maternal mito DNA, fairly rapid onset 20s-40s with rapid decline within weeks; poor colour VA, cecocentral
scotoma, disc swelling & telangiectasis but no leakage on IVFA; bilateral within weeks; quit smoking & EtOH)
5. Retinal degenerations: gangliosidoses, choroideremia, any chronic toxicity
6. Anoxia at birth
7. Trauma

32) Causes of swollen discs in kids

HOT DIP
1. Hydrocephalus 4. Drusen
2. Optic neuritis 5. Infiltrative (leukemia)/Infectious (toxo, meningitis)
3. Tumour/Trauma 6. Pseudotumour

33) The phakomatoses

 Neurofibromatosis 1: AD inher, chrom #17, Dx: 2/7 of: 1) >5 café-au-lait spots; 2) >1 nodular
neurofibroma, or 1 plexiform; 3) optic nerve glioma; 4) axillary freckling; 5) sphenoid wing dysplasia;
relative with NF-1; eye findings: lid neurofibromas, plexiform neurofibroma with hypertrichosis,
hyperpigmentation, ptosis, S-shaped lid, glaucoma, enlarged corneal nerves, iris Leisch nodules, choroidal
neurofibromas, retinal astrocytic hamartomas, optic nerve glioma, absence of the sphenoid wing with
pulsatile proptosis

 Neurofibromatosis 2: AD inher, chrom #22, bilateral acoustic neuromas; eye findings:

PSCC, CN VII palsy

 Tuberous sclerosis (Bourneville’s disease): “Zits, Fits, & Nitwits!”, AD inher,

Chrom #9 or #15 but 80% sporadic, Dx: Ash-leaf macules, adenoma sebaceum on cheeks, mouth &
tongue, gingival hyperplasia, lumbo-sacral shagreen patch, mental retardation, seizures due to tuberous
growths in cortex with obstructive hydrocephalus; eye findings: astrocytic hamartoma seen in 1/3-1/2

 Von-Hippel Lindau Disease: AD inher, chrom #3, Dx: retinal capillary hemangiomas (bil in
50%), cerebellar hemangiomas (in 25%), reanl cysts, renal cell ca, pheochromocytoma, spinal chord
syrinx eye findings: retinal capillary hemangiomas, often multiple & bilateral

 Sturge Weber syndrome: sporadic non-inher; Dx: cutaneous facial angioma over V1-3,
hemifacial hypertrophy, ipsilateral leptomeningeal vascular malformation, seizures, mental retardation
(uncommon); eye findings: thickened lids, dilated conj vessels, enlarged cornea, heterochromia,
glaucoma (~1/3), diffuse choroidal hemangioma, at risk for massive choroidal hemorrhage in surgery

 Wyburn-Mason syndrome: sporadic non- inher; Dx: AVM of retina + brain; eye findings:
closed-loop vascular malformation without intervening capillary bed; CT head to R/O intracranial AVM

 Ataxia telangiectasia: AR inher; chrom #11; defect in ability to repair DNA; Dx: recurrent
pulmonary infections, progressive clumsiness in childhood, poor motor control, low T-cells and IgG,
susceptibility to lymphoma/leukemia, extra-sensitive to radiation, telangiectasias on skin of face & neck;
eye findings: dilated conj vessels, inability to initiate saccades

34) Ocular & other findings in child abuse

1. Vitreous hemorrhage 6. Hyphema
2. Multiple, multi-level retinal hemorrhages 7. Cataract/lens disloc.
3. Hemorrhagic retinochisis in the posterior pole 8. Bilateral ecchymoses
4. Swollen and hemorrhagic disc 9. Chlamydia/Gonococ inf
5. CN palsies (III, IV, or VI)
*Other: subdural/intracranial hemorrhage, cerebral edema, multiple #’s various healing, bruises/burns esp on back
**Other causes of retinal heme in infant/child: anemia, leukemia, hemophilia, childbirth (usually very few in number,
85% cleared by 3 days, all have cleared within 4-6 weeks of birth)
 GLAUCOMA
1) Factors that increase or decrease IOP
APES FART Coffee
(decrease IOP) (increase IOP)
1. Alcohol 1. Fluid intake/Family Hx/Female sex
2. Pot 2. Age
3. Excersise 3. Race/Respirations/Recumbancy
4. Systemic meds (B-blockers) 4. Time of day
5. Coffee

2) Aqueous production and the Goldmann equation

IOP = F/C + EVP <or> F = (IOP – EVP)
R
F = flow, or aqueous formation (ul/min)
C = facility of outflow (ul/min/mmHg); normally C = 0.2-0.3 ul/min/mmHg; low C = high resistance, and high IOP
EVP = episcleral venous pressure (mmHg)
R = resistence to outflow (inverse of “C”)
Aqueous is produced via active transport (majority, dependent upon ENERGY),
ultrafiltration (dependent on PRESSURE, not energy), and diffusion (dependent on
CONCENTRATION GRADIENT, not energy or pressure)

3)Factors that falsely elevate or decrease IOP measurement

ELEVATE DECREASE
Lid squeezing Lasik
Breath holding/Valsalva Corneal edema
Pressure on the globe Soft contact lens
Thick cornea (>550 um)* Thin cornea (<550 um)*
Too little fluorescein Too much fluorescein
Tight collar * For every 10um > 550um, subtract
Corneal scar 0.5mmHg, or for every 50um > 550um,
subtract 3mmHg to arrive at “real” IOP
EOM restriction Reverse is true for corneas <550um.

4) Causes of increased pigment in the angle

SHAMU’S PP
1. Surgical trauma 5. Uveitis
2. Hyphema 6. Siderosis
3. Age 7. PDS/PDG
4. Melanoma 8. PXFS/PXFG
PIGMENT GRADING: Gr.0 = none; Gr.1 = faint; Gr.2 = normal; Gr.3 = heavy; Gr.4 = very heavy
5) Causes of blood in Schlemm’s canal
THE GASH
1. Thyroid 5. A-V fistula
2. Hypotony 6. Sturge-Weber
3. Elevated EVP 7. Hyphema
4. Gonioprism pressure

6) Diagnostic signs of a glaucomatous disc

1. Vertical cup elongation 9. Baring of circumlinear vessel
2. Cup enlargement 10. Arteriolar narrowing
3. Assymetric cupping 11. Bayonetting of vessels
4. Notching of the rim 12. Vessel overpass
5. Focal pallor of the rim 13. Vessel nasalization
6. Peripapillary atrophy (esp atrophic “B-zone”) 14. Saucerization of cup
7. NFL defects 15. Exposure of lamina cribrosa pores
8. Splinter NFL hemorrhages (1/3 NTG, 1/10 POAG, progression in 70%)

7) DDx of Drance NFL disc hemorrhage

Drance Hemorrhages Never Bleed Aggressively,
Practically Pinpointing Glaucoma
1. Diabetes 5. Anticoagulants
2. HTN 6. PVD
3. NAION 7. Papillitis
4. BRVO 8. Glaucoma

8) Risk factors for POAG

I’M HARD
1. IOP elevation 4. Age (age >80 = 3% whites, 11% blacks)
2. Myopia (3X greater for >-6.00) 5. Race (4X greater in blacks, 8X blindness)
3. Heredity (4X greater if +FHx, = ~10%) 6. Diabetes (2-3X greater prevalence)

9) Some points on ocular hypertension (OHTS study)

 4-10% of people >40 years old have OHTN
 9.5% will progress to glaucoma over 5 years
 if IOP lowered by 20% or <24 mmHg, this is reduced to 4.4%
 risk factors for progression: 1) old age; 2) higher IOP; 3) larger C:D
ratio; central K thickness <555um (most powerful predictor of all, if K <555um, then
risk of progression increased 3X if you had elevated IOP)
10) DDx of normal tension glaucoma
O,P,Q,R,S
1. Optic nerve pathology: drusen, AION/NAION, coloboma, disc pit
2. Previously undiagnosed glaucoma: POAG with fluctuating IOP,
burned out PDG, burned our uveitic glaucoma, previous phacomorphic
CACG now pseudophakic, intermittent CACG, previous hyphema,
undiagnosed angle recession
3. Qualitative error: tech error (IOP really is high), unmeasured corneal
thickness
4. Retinal pathology: RD, retinoschisis, maculopathy, chorioretinopathy,
vascular occlusion
5. Systemic disease: previous hypotensive/shock episode, previous
blood loss, anemia, previous steroid use…. BRAIN TUMOUR!!
*for exam purposes, EVERYONE with NTG should get a CT head to R/O brain tumour (Dr. Trope at UofT scans
everyone in real life); in reality you may choose only to scan those who are young and those whose disc pallor>cupping
** CNTGS showed that 35% of controls prodressed to glaucoma in 3 yrs; reduced to 12% with 30% reduction in IOP

11) Eight clinical signs of PXF

1. Double ring of PXF on ALC 5. Shallow A/C
2. PXF on pupil margin 6. Phacodynesis
3. PXF on iris surface/corneal endothelium 7. Heavily pigmented TM
4. Peripupillary atrophy 8. Sampaulesi’s line

12) Eight surgical challenges of PXF

1. Poorly dilating pupil 5. Vitreous presentation
2. Deep A/C 6. Capsular rupture/dropped nucleus
3. “Bouncy” lens-iris diaphragm 7. Post-op IOP spike
4. Zonular dehisence 8. Heavy post-op inflammation

13) Eight clinical signs of PDG

1. Krukenberg spindle 5. Peripheral posterior iris bowing
2. Pigment on iris surface 6. Pigment on posterior lens capsule
3. Iris TI defects 7. “Mascara” TM pigment
4. Pigment in aqueous 8. Deep A/C
*MYOPIA seen almost always as well
** 30-50% will progress from PDS to PDG; Rx: Meds (Pilo) + ALT for IOP control; laser PI to stop chaffing

14) DDx of phacolytic glaucoma

TUNA
1. Trauma
2. Uveitic
3. NVG
4. AACG **Always do gonio !!**
15) How tumours can cause glaucoma
OPEN ANGLE CLOSED ANGLE
Direct angle invasion Anterior rotation of C.B
Tumor cell clogging Anterior displacement of lens/iris
Pigment clogging Neovascularization
Hyphema Suprachoroidal hemorrhage
Inflammation
*TUMOURS MOST LIKELY TO CAUSE GLAUCOMA IN ADULTS = melanoma, lymphoma, leukemia, met
*TUMOURS MOST LIKELY TO CAUSE GLAUCOMA IN KIDS = retinoblastoma, medulloepithelioma, JXG

16) How uveitis can cause glaucoma

1. Edema of TM 6. Scarring of outflow channels
2. TM endothelial cell dysfxn 7. Neovascularization
3. TM clogging by cells/fibrin 8. Synechiae formation
4. Increased aqueous viscosity 9. Pupillary block
5. PG-mediated disruption of the blood-aqueous barrier
*KNOWN UVEITIC ENTITIES THAT CAN DIRECTLY ELEVATE IOP = HSK, HZK, toxo, rheum arthritis, pars planitis

17) Ways in which Fuch’s differs from Posner-Schlossman

1. Only ~15% get glaucoma (100% in PS, IOP often in 50’s, KP on TM frequently seen)
2. Low-grade inflammation constant (intermittent in PS)
3. Patients asymptomatic (present with red eye + blurry VA in PS, but almost never pain!)
4. Iris heterochromia (not seen in PS)
5. Cataract (not seen in PS)
6. Fine vessels over TM (not seen in PS)
7. Poorly responsive to steroid (good response in PS within weeks, prophylaxis not needed)
8. May have toxo-like chorioretinal scars (not seen in PS)

18) Causes of elevated episcleral venous pressure

STARVES
1. Sturge-Weber 5. Varix
2. Thyroid 6. Elevated EVP of unknown cause
3. A-V malformation 7. SVC syndrome
4. Retrobulber tumour

19) Dr. Campbell’s “7 Rings of Trauma”

1. Sphincter pupillae: traumatic mydriasis
2. Iris root: iridodialysis
3. Anterior face of ciliary body: angle recession
4. Scleral spur: cyclodialysis cleft
5. Trabecular meshwork: glaucoma
6. Zonules: phakodynesis/lens subluxation
7. Ora serrata: retinal dialysis/detachment

20) Causes of glaucoma associated with trauma

 For Alkali Exposure, TEACH Safety Goggles
1. Foreign body retention 6. Angle recession
2. Alkali burn 7. Closure of cyclodialysis cleft
3. Epithelial downgrowth 8. Hyphema
4. TM trauma 9. Suprachoroidal hemorrhage
5. Ectopia lentis 10. Ghost cell

21)More on some types of glaucoma associated with trauma

1. Hyphema & glaucoma: Risk factors: 1) large hyphema; 2) rebleed; 3) sickle cell disease; 4)
pre-existing trabecular dysfunction/glaucoma
2. Angle recession glaucoma: 6-20% of pts with angle recession >180 degrees will get
glaucoma; Signs of angle recession: 1) deep A/C; 2) broad angle recess wider than TM width; 3) absent or
torn iris processes; 4) glistening white scleral spur; 5) depression in overlying TM; 6) localized PAS at border of
recession; 7) irido-phacogynesis; 8) visible tears or “shagginess” or less pigment on CBB face… AVOID
MIOTICS! AVOID ALT!
3. Ghost cell glaucoma: requires a VH with a disrupted anterior hyaloid face; RBCs degenerate
over ~1 month, then khaki-coloured ghost cells clog TM; A/C aspiration shows empty RBC membranes with
HgB clumps (= “Heinz bodies”)
4. Phacoanaphylactic glaucoma: requires disruption of lens capsule followed by
sensitization period; glaucoma uncommon (10-15%), usually get hypotony; pathology = zonal granulomatous
inflammation
5. Closure of cyclodialysis cleft: IOP elevation can be dramatic

22) Causes of glaucoma following surgery

PUMP SHIVER
1. Pigment release 6. Hyphema
2. UGH 7. Inflammation
3. Mechanical damage to TM 8. Viscoelastic retention
4. Pseudophakic pupil block 9. Epithelial downgrowth
5. Steroid use 10. Retained lens fragment

23) Risk factors for steroid-induced glaucoma

More Drops Risk Glaucoma
1. Myopia 3. Relative with POAG
2. Diabetes 4. Pre-existing glaucoma
*CAUSES OF STEROID GLAUCOMA: 1) increased GAGs in TM; 2) increased tight junctions in Schlemm’s
canal endothelium; 3) inhibition of phagocytosis by TM... all cause decreased facility of outflow = LOW “C”!!
** Occurs in 5% of pts using steroids, usually 2-3 weeks after start (but can occur ANYTIME!)

24) Types of secondary open angle glaucomas

EXULT PDT!!
1. Elevated EVP 5. Trauma
2. Xfoliation 6. Pigment dispersion
3. Uveitic 7. Drugs (steroid… topiramate and sulfonamides can cause ACG)
4. Lens induced 8. Tumour
25) Causes of peripheral anterior synechiae
 1. NVI 7. ALT burn
2. ICE 8. Previously flat A/C
3. PPMD 9. Previous pupil block
4. Uveitis 10. Plateau iris
5. Epithelial downgrowth 11. Any “posterior pushing” cause
6. Trauma

26) Causes of angle closure glaucoma

PUSHING PULLING
1. Relative lens pupil block 1. Fibrovascular membrane (NVG)
2. Lens dislocation 2. Endothelial membrane (ICE, PPMD)
3. Aphakic pupil block 3. Epithelial membrane (downgrowth)
4. CB cysts/tumours 4. Inflammatory membrane (PAS)
5. Choroidal effusions/hemorrhage
6. Fundus tumours
7. Plateau iris syndrome
8. Scleral buckle
9. Aqueous misdirection
10. Contracting retrolental membrane (PHPV)

27) Risk factors for acute angle closure glaucoma due to

relative pupil block (most common cause by far)
HARD IOP
1. Hyperopia 5. Inuit/Asian decent
2. Age 6. Other eye having ACG
3. Relative with ACG 7. Positive von-Herik/Purse-carrier
4. Dilation of pupil
*PROTOTYPICAL PATIENT: middle-aged Asian female hyperope in a movie theater
**REMEMBER YOU NEED TO GONIO TO MAKE DX!...r/o NVI (b/c LPI contraindicated), and check status of
other eye as well (b/c prophylactic LPI indicated in unaffected eye), also examine fundus r/o “pushing” mech.
***DDx of RPB-AACG includes all of the items listed in #26, so look carefully… B-scan if necessary!

28) Causes of choroidal effusions with secondary ACG

Venous Drainage Totally Sucks In Nanophthalmos!
1. Venous congestion: CRVO, CCSF, A-V malformation
2. Drugs: topiramate, sulpha compounds, acetazolamide
3. Tumours : mets, melanoma, retinoblastoma
4. Surgery: scleral buckle, PRP, PPV
5. Inflammation: pars planitis, syphilis, lyme, TB, toxo, SLE, IOIS, scleritis
6. Nanophthalmos : <20mm axial length with normal lens +++hyperope!

29) Absolute diagnostic criteria for aqueous misdirection

  central AXIAL shallowing of A/C
 presence of patent PI
 absence of suprachoroidal hemorrhage or fluid
 elevated IOP (may not be too high if bleb present)

30) Causes of neovascular glaucoma

1. Diabetes 5. Inflammation
2. CRVO 6. Tumours
3. OIS 7. Radiation
4. CRAO 8. Other retinal causes (TONS!...chronic RD,PHPV,ROP,sickle cell,
Eele’s,Coat’s,FEVR,etc etc etc)

31) DDx of I.C.E. syndrome

PAINFUL
1. PPMD 5. Fuch’s
2. Axenfeld-Reigers 6. Uveitis (HSK/HZK iris atrophy)
3. Iridoschisis 7. Leisch nodules
4. Nevus

32) Side effects of parasympathomimetics (pilo et al)

A,B,C SLUDGE
1. A-V heart block 6. Urination
2. Bradycardia 7. Diarrhea
3. Confusion 8. GI upset
4. Salivation 9. Excessive sweating
5. Lacrimation
*CONTRAINDICATIONS TO PILO: Any secondary ACG (effusions, hemorrhage, aqueous misdirection, NVG,
uveitic, ICE/PPMD, etc), previous RRD, hyphema, high myopia (relative b/c of high risk of RRD), MAOI use

33) Risk factors for trabeculectomy failure (a.k.a. reasons to

use antimetabolites during filtration surgery)
NAÏVE African American YOUTH are Poorly compliant
1. NVG 7. Young age
2. Aphakia 8. Ocular surgery
3. Injured conjunctiva 9. Uveitis
4. Vitreous in A/C 10. Trab failure previously
5. Endothelial/epithelial etiology 11. Hyphema
6. African race 12. Poor med compliance
*CONTRAINDICATIONS TO TRAB SX: blind eye (cyclodestruction instead), tumour, bad conj scarring (OCP,SJS etc),
NVG (relative), active uveitic (relative), pt anticoagulated (relative)

34) Complications of filtration surgery

 1. Intraoperative: 1) retrobulbar hemorrhage; 2) conjunctival buttonhole;
3) corneal perforation (bridal suture); 4) subconjunctival hemorrhage; 5)
scleral flap amputation; 6) scleral flap shrinkage (cautery); 7) posterior
sclerostomy with prolapse of ciliary processes + VH; 8) vitreous loss; 9)
non-patent iridectomy; 10) hyphema; 11) suprachoroidal hemorrhage; 12)
choroidal effusions
2. Early post-op (DO GONIOSCOPY!!): 1.HIGH IOP, DEEP A/C: 1) tight flap; 2)
blocked sclerostomy (blood, vitreous, fibrin, iris); 3) retained viscoelastic;
4) early Tenon’s cyst; 2. HIGH IOP, SHALLOW A/C: 1) papillary block
(incomplete iridectomy); 2) suprachoroidal hemorrhage; 3) aqueous
misdirection; 3. LOW IOP, SHALLOW A/C: 1) overfiltration; 2) bleb leak;
3) choroidal effusions; 4) cyclodialysis cleft; 5) RRD; 4. OTHERS: 1) CME;
2) exuberant inflammation; 3) worsened VA or VFD; 4) corneal abrasion;
5) topical med or antimetabolite toxicity
3. Late post-op: 1) chronic/late wound leak; 2) encapsulated bleb; 3)
blebitis; 4) endophthalmitis; 5) choroidal detachment/hemorrhage; 6) bleb
fibrosis/failure; 7) overhanging bleb; 8) bleb dysesthesia; 9) dellen; 10)
topical med or antimetabolite toxicity; 11) hypotonous maculopathy; 12)
cataract

35) Causes of visual loss with hypotony

1. Corneal edema 6. Choroidal effusions
2. Astigmatism 7. Ciliary body detachment
3. Hyperopic shift 8. Suprachoroidal hemorrhage
4. Iris-lens-cornea touch 9. Hypotonous maculopathy
5. Cataract 10. Disc edema

36) Risk factors for developing post-op hypotony

1. High pre-op IOP 4. Use of MMC
2. High myopia 5. Early suture lysis
3. Young age 6. Loose/poorly made scleral flap

37) Complications of tube-shunt surgery

…same as trabeculectomy surgery, PLUS…
1. Corneal trauma from tube 7. Slippage of filter
2. Iris trauma/hyphema from tube 8. Perf’d globe
3. Tube blockage 9. Strabismus
4. Tube retraction 10. Retrobulbar hemorrhage
5. Tube erosion/exposure 11. Aggressive overfiltration
6. Extrusion of filter 12. Filter plate encapsulation
 NEUROOPHTHALMOLOGY
1) Comparison of CT and MRI
CT MRI
Advantages: better for bone, better Advantages: better for vascular
for calcium, better for acute blood, stuff, better soft tissue resolution,
only choice for metal, cheaper, better for cavernous sinus, better for
CT-A can detect aneurysms 2mm orbital apex, gad less allergenic
Disadvantages: metallic artifact, Disadvantages: not good for bone,
cumulative radiation, allergy to not good for calcium, not good for
contrast, coronals difficult in elderly, acute blood, not good for
poor soft tissue contrast, infracted clausterphobics, not good for pts
tissue doesn’t show up for first 24- who cannot remain still, cannot use
48hrs, then appears as darker area in presence of metal, costly
that enhances with contrast Contraindications: metal FB,
pregnancy, pacemaker, old
aneurysm clip, trauma initial study,
clausterphobia/non-cooperative

2) Causes of swollen optic disc(s)

VISIT Raised ICP
1. Vascular: AAION, NAION, diabetic papillopathy
2. Inflammatory/Infectious: sarcoidosis, lupus, Wegener’s, optic neuritis,
toxo, TB, lyme, syphilis, cat scratch
3. Systemic: HTN, DM, CHF, Leber’s, eclampsia, meningitis, neck
tumours, drugs (ethambutol, vitamin A)
4. Infiltrative: leukemia, lymphoma, mets
5. Tumour of the optic nerve: glioma, meningioma
6. Raised ICP: IIH, intracranial tumour

3) Ocular signs of optic neuritis

1. Swollen disc (optic neuritis) 8. NFL defect
2. RAPD (optic neuritis) 9. Uveitis
3. CN palsies 10. Pars planitis
4. Supranuclear palsies 11. Peri-venous sheathing
5. Nystagmus 12. Ptosis
6. Dorsal midbrain syndrome 13. Accomodative problems
7. VF defects

4) Ocular symptoms of optic neuritis

1. Decreased BCVA 8. Painful EOM
2. Decreased colour vision 9. TVO’s
3. Decreased brightness 10. Utoff’s phenomenon
4. Visual field loss 11. Diplopia
5) Causes of toxic/metabolic optic neuropathy
CREAMI BLT
1. Chloroquine/Carcinomatous 6. Isoniazid
2. Rifampin 7. B vitamin deficiency (2,6,&12)
3. Ethambutol 8. Lead toxicity
4. Amiodarone/Arsenic 9. Thiamine deficiency/tobacco + EtOH
5. Methanol

6) Causes of a cecocentral field defect

1. Toxic/metabolic optic neuropathy 5. Swollen disc
2. Hereditary optic neuropathy 6. Macular scar/atrophy
3. NAION/AAION 7. Malingering
4. Vascular occlusion 8. Neuroretinitis

7) Ddx of headache
I’M a MEATHEAD
1. IIH 6. Tumour
2. Meningitis 7. Hemorrhage
3. Migraine 8. ENT problem
4. Eye problem 9. Arteritic (GCA)
5. Aneurysm 10. Different type of headache (icepick, tic dolereux, cluster, etc.)

8) Ddx of lesions causing chiasmal compression

MASCARA MAG
1. Meningioma 6. Radiation necrosis
2. Adenoma 7. Apoplexy
3. Sarcoid grannuloma 8. Mets
4. Chraniopharyngioma 9. Astrocytic hamartoma
5. Aneurysm 10. Glioma

9) Causes of a relative afferent papillary defect (RAPD)

1. Unilateral optic nerve disease
2. Bilateral asymmetric optic nerve disease
3. Optic tract disease (53% of nasal fibres cross, so RAPD is contralateral to lesion)
4. Retinal disease affecting ganglion cells
5. Amblyopia (subtle)
*things that do not cause an RAPD include refractive error, cataract or other media opacities, cortical lesions,
hysteria, or malingering
10) Signs of Horner’s syndrome
1. Miosis anisocoria 8. Heterchromia
2. Pupil dilation lag 9. Ipsilateral lack of facial flushing
3. Ptosis (2mm) 10. Ipsilateral lack of curly hair (Dr.Arthur)
4. Reverse ptosis (CONGENITAL HORNER’S)
5. “Pinch” sign 11. Sore throat
6. Conjunctival injection 12. Dysgeusia
7. Anhydrosis 13. Anterior neck pain
(CAROTID DISSECTION)
*COCAINE 4% TEST: confirms Horner’s, blocks re-uptake of NE (normal eye dilates, Horners doesn’t)
*HYDROXYAMPHETAMINE 1% TEST: differentiates 3rd order from 1st or 2nd order; stimulates release of NE
from nerve terminal; (1st & 2nd order dilates, 3rd order doesn’t)

11) Causes of Horner’s syndrome

1st Order (CLUES: numbness, weakness, ataxia, nystagmus, skew deviation, personality changes): 1)
vertebral artery/PICA stroke (Wallenberg syndrome); 2) brainstem tumour;
3) cervical disc disease; 4) demylinating disease
2nd Order (CLUES: cough, hemoptysis, chest pain, surgical hx, cancer hx, trauma hx, forceps delivery):
1) apical lung tumour (Pancoast tumour); 2) metastases (to apical lung,
neck, chest wall); 3) brachial plexus injury; 4) neck tumour/surgery; 5)
chest surgery; 6) thoracic/subclavian artery aneurysm; 7) neuroblastoma
3rd Order (CLUES: neck pain, HA, diplopia from CN VI palsy, numbness over V1 & V2, epistaxis):
1) neck tumours; 2) migraine/cluster headache; 3) cavernous sinus
inflammation/thrombosis; 4) CCS fistula; 5) sinus tumours; 6) orbital
infection/inflamm/tumour/trauma; 7) complicated otitis media; 8) carotid
artery dissection

12) Characteristics of the dorsal midbrain syndrome

(a.k.a. “Parinaud’s syndrome”, “Pretectal syndrome”)
CLUES
1. Convergence-retraction nystagmus
2. Light-near dissociation
3. Upgaze paralysis
4. Eyelid retraction
5. Skew deviation
*also spasm of near kinetic reflex, and optic nerve swelling if cause is pinealoma/tumour

13) Causes of light-near dissociation pupil response

 Adie’s pupils
 Argyll-Robertson pupils
 Dorsal midbrain syndrome
 Aberrant regeneration of CN III palsy
 Any severe optic neuropathy or retinaopathy
14) Causes of supranuclear ophthalmoplegia
I’M WHIPPED
1. Infarcts (multiple) 6. Parkinson’s
2. Multiple sclerosis 7. Progressive supranuclear palsy
3. Whipple’s disease 8. Encephalitis
4. Huntington’s disease 9. Dorsal midbrain syndrome
5. Infiltrative disease (lymphoma, carcinomatosis)

15) DDx of vertical diplopia

Four Grave TOOMBS
1. Fourth nerve palsy 5. Orbital pathology (blowout #, IOIS, tumour)
2. Graves disease 6. Myesthenia gravis
3. Third nerve palsy 7. Brown’s syndrome
4. Overaction of obliques 8. Skew deviation

16) Causes of cavernous sinus syndrome

1. Vascular: ICA aneurysm, CCS fistula, thrombosis
2. Inflammatory: sarcoidosis, Wegener’s, Tolosa-Hunt
3. Infectious: HZO, cellulites extension, TB, syphilis, mucormycosis
4. Infiltrative: leukemia, lymphoma, mets
5. Tumour: pituitary adenoma, pituitary apoplexy, meningioma,
craniopharyngioma, local invasion from SCC, local invasion from
nasopharyngeal carcinoma, metastases

17) Imitators of (DDx of) cavernous sinus syndrome

(a.k.a. DDx of multiple cranial neuropathies)
Many Other Things Beget Frozen Globes,Like PSP & CPEO
1. Myesthenia gravis 4. Botulism
2. Orbital pathology 5. Fisher syndrome
3. Thyroid disease 6. GCA
7. Lambert-Eaton
8. PSP
9. CPEO

18) Causes of CN VII palsy

Intracranial Extracranial
1. CP angle tumour 1. Sarcoidosis
2. Geniculate ganglionitis (Ramsay-Hunt) 2. Gillan-Barre
3. Moebius syndrome 3. Vasculitis
4. Stroke/hemorrhage 4. Multiple sclerosis
5. Meningitis 5. Bell’s palsy
Has many “neighbourhood friends”, like Ask about recent viral illness, history of
dysacusis, decreased tearing, other cranial sarcoiditis and family history of Bell’s palsy.
neuropathies, (these imply disturbance before- Palpate parotid gland. Order serum ACE,
proximal to- the geniculate ganglion so LOOK FOR lysozyme, ESR, and ANA. Bell’s palsy should
THEM!), may also see hyperacusis, decreased get better within 3/12 (80%), if not better by 6/12
taste, cerebellar signs, and meningismal signs then CT scan! Ten day course of steroid and
if meningitis. Look for papilledema and vesicles acyclovir may improve odds of full recovery +
on pinna. speed recovery.
19) Types of CPEO
1. Oculopharyngeal dystrophy (French Canadian AR inherited muscular dystrophy
with swallowing problems)
2. Kearns-Sayer syndrome (mitochondrial myopathy with CPEO + pigmentary
retinopathy + cardiac conduction defects)
3. MELAS (mitochondrial encephalopathy with lactic acidosis and stroke; affects young ~20yrs)
4. Myotonic dystrophy (AD inherited muscular dystrophy with CPEO + polychromatic
cataracts + miotic pupils + retinal pigment degeneration + prolonged muscle contractions + frontal
balding + “hatchet facies” + hearing loss + testicular atrophy + low intelligence + cardiomyopathy)
 RETINA
1) Common causes of hyperfluorescence on IVFA
1. Window defect: atrophic AMD, toxic atrophic changes (chloroquine),
chorioretinal scars (histo, toxo)
2. Intraretinal leakage: CME, NVE, microaneurysma, mac edema
3. Subretinal leakage: CNVM, CSCR
4. Subretinal pooling: CSCR, VKH, any exudative RD (HTN, tumour)
5. Staining: CNVM scar, choroidal rupture, drusen
6. Autofluorescence: occurs without fluorescein; ex. Disc drusen
*Fluorescein large molecule, 80% bound to albumin, absorbs blue light 480nm, emits yellow light 530nm

2) Common causes of hypofluorescence on IVFA

1. Normal hypofluorescence: macular center, FAZ
2. Blockage: subretinal/intraretinal/preretinal hemorrhage, pigment (old
PRP burns, scars), lipofuscin (Stargardt’s, vitelliform)
3. Hypoperfusion retinal: CRVO, BRVO, CRAO, BRAO, diabetes
4. Hypoperfusion choroidal: OIS, ophthalmic artery occlusion, HTN,
GCA, lupus, choroideremia

3) Side effects of IVFA

1. Yellow skin (6-12 hrs) 6. Tissue extravasation of dye
2. Orange urine (24-36 hrs) 7. Anaphylactoid reaction
3. N & V (10%) 8. MI
4. Pruritis/urticaria (1%) 9. death (1/225,000)
5. Sycope

4) Causes of extinguished/flat ERG

1. Leber’s congenital amaurosis 6. DUSN
2. Choroideremia 7. Chronic chorioretinitis
3. End-stage retinitis pigmentosa 8. Ophthalmic artery occlusion
4. Metallic FB retinopathy (iron,copper) 9. Cancer-associated retinopathy
5. Med toxic retinopathy (chloroquine)

5) Causes of reduced B-wave ERG with normal A-wave

1. Juvenile X-linked retinoschisis
2. Congenital stationary night blindness
3. CRAO
4. Ischemic CRVO
5. Severe diabetes
6) Causes of abnormal photopic ERG + 30Hz flicker, with
normal scotopic ERG
1. Achromatopsia
2. Cone dystrophy

7) What’s the EOG and what’s it good for?

 Electrophysiologic test the measures the resting potential of the RPE
under both light and dark adapted conditions; thought to be a reflection
of how well the RPE is functioning
 The change in amplitude of the resting potential between the
maximally light-adapted state and the minimally dark-adapted state is
the Arden Ratio = 1.9 – 2.8 normally, anything <1.7 is ABNORMAL
 Normally the EOG parallels the ERG, so it isn’t used much except for
these conditions/situations:
1. Best’s disease: will have a normal (even supernormal) ERG
and an abnormal EOG (Arden Ratio <1.5… even <1.1!)
2. Carriers of Best disease gene: ditto
3. Rubella retinopathy: also can have a normal (or near-
normal) ERG and an abnormal EOG
4. Juvenile X-linked retinoschisis: will have a normal EOG
and an abnormal ERG (reduced B-wave, normal A-wave)
5. Testing retinal fxn in contact lens intolerant pt
6. Monitoring pts taking chloroquine: faster & easier than
ERG

8) DDx of bullseye maculopathy

Royal College Summer CAMP
1. Rod-cone dystrophy 5. AMD
2. Cone dystrophy 6. Macular hole
3. Stargardt’s disease (AR inher; ABDR gene) 7. Pattern dystrophy (age >50,good VA)
4. Chloroquine toxicity (rare with daily dose <250mg, cumulative dose <300g)

9) DDx of macular cherry-red spot

C.N.T.G.S. (…you know, as in the glaucoma trial)
1. CRAO
2. Niemann-Pick disease (essential lipid histiocytosis = “Sea blue histiocyte syndrome”; Sphingomyelinase)
3. Tay-Sachs disease (gangliosidosis type I; Hexosaminidase A; retardation, early death, Ashkenazi Jews)
4. Gentamicin macular infarction
5. Sandhoff’s disease (gangliosidosis type II; Hexosaminidase A & B; retardation, early death)
10) Causes of CRAO
CRAO may S.T.E.M. from G.C.A.
1. Sickle cell disease 5. GCA
2. Talc embolus 6. Collagen vascular dx/ Coagulopathy
3. Embolus (carotid, valve) 7. Atherosclerosis
4. Migraine
*RISK FACTORS/IMPT HX: older age, atherosclerosis, HTN, DM, valve disease/replacement; prev stroke, coagulopathy,
collagen vascular disease, vasospastic history, IV drug use hx; GCA Sx’s?
**IMPT FACTS: embolus seen in 20%; NVI = 20%; NVG = 10%; NVD <5%; cilioretinal artery = 15%
***DO NOT FORGET TO ENQUIRE ABOUT GCA SYMPTOMS!!!

11) DDx of chorioretinal folds

SHITTIER Hyperopia
1. Scleritis (posterior) 6. Idiopathic
2. Hypotony 7. Effusions
3. IOIS 8. RD (shallow)
4. Thyroid 9. Hyperopia
5. Tumour

12) DDx of cystoid macular edema

JJ’s Veiny RED TULIPS
1. Juvenile X-linked retinoschisis 7. Tumour
2. Juxtafoveal telangiectasia 8. Uveitis
3. Vein occlusion 9. Latanoprost
4. Retinitis pigmentosa 10. Irvine-Gass syndrome
5. Epinephrine use (30% irreversible in aphakes) 11. Pars planitis
6. Diabetes 12. Surgery (other than cataract)

13) Twelve signs of retinitis pigmentosa

1. Bone spicules (late) 7. ERM/loss of foveal reflex
2. Yellowish deep retinal lesions 8. PSCC
3. Vessel attenuation 9. Vitreous cells/pigment
4. Waxy disc pallor 10. Ring scotoma VFD
5. Disc drusen 11. Window defects + CME on IVFA
6. CME 12. Abnormal ERG (decreased A & B-waves, eventually
extinguished; prolonged dark adaptation
recovery to threshold time; delayed rod-
cone break)

14) Eight variations of retinitis pigmentosa syndromes

KALA BLUR (“kala” means black in Hindi I think)
1. Kearns-Seyer (RP + CPEO + heart block) Rx = cardiology consult/EKG
2. Abetalipoproteinemia (RP + fat malabsorption) Rx = vitamin ADEK suppl.
3. Leber’s (oculodigital reflex, flat ERG, nystagmus, paradoxic pupil, keratoconus, hyperopia, 1/3 normal fundus)
4. Alstrom’s disease(RP + Bardet-Biedl + diabetes + deafness)
5. Bardet-Biedl (RP + obese + retardation + polydactyly + hypogonadism)
6. Lawrence-Moon (retarded + spastic paresis)
7. Usher’s (RP + deafness + vestibular dysfunction; Type 1 BAD: no speech, totally deaf; Type 2 BETTER:
speech, may have some hearing & vestibular fxn)
8. Refsum’s disease (RP + phytanic acid excess due to paroxisome storage problem + deaf + heart problems
+ polyneuropathy + ataxia) Rx = restrict phytanic acid precursors + cardiology consult

15) DDx for causes of choroidal neovascular membrane

A.M.D. U-TRAIN
1. AMD 5. Trauma/Tumour
2. Myopia/Macroaneurysm 6. Retinitis pigmentosa
3. Disc drusen 7.Angioid streaks (50% get CNVM)
4. Uveitis (POHS, MFC, serpiginous) 8. Idiopathic
9. Nevus

16) Sixteen signs of pathologic myopia

1. Axial length > 26.5mm, or >-6.00D error 9. Generalized RPE thinning

2. CNVM (10-15%) 10. Cobblestone degeneration
3. Macular hole 11. Lattice degeneration
4. Atrophic maculopathy 12. Peripheral holes/tears
5. Posterior staphyloma 13. RRD
6. Laquer cracks (= breaks in Bruch’s) 14. Tilted discs
7. Isolated subretinal hemorrhage (no CNVM) 15. Peripapillary atrophy
8. Fuch’s spot (RPE hyperplasia) 16. Glaucoma

17) DDx for associations with angiod streaks

A Lead PEPSI
1. Abetalipoproteinemia 5. Paget’s disease
2. Lead poisoning 6. Sickle cell anemia
3. Pseudo-xanthoma elasticum 7. Idiopathic
4. Ehlers-Danlos

18) DDx for causes of cotton wool spots

Diabetic LOVE SEARCH
1. Diabetes (most common by far) 6. Sickle cell anemia
2. Leukemia 7. Embolic disease
3. Optic disc swelling 8. AIDS (most common cause of CWS in otherwise healthy pt)
4. Vasculitis 9. Radiation
5. Endocarditis 10. CRVO
11. HTN (…second most common)
19) DDx ofcauses of Purtcher’s retinopathy
RAT TRAPS
1. Renal failure 5. Retrobulbar injection into nerve
2. Autoimmune disease 6. Amniotic fluid embolus
3. Trauma, head/chest 7. Pancreatitis
4. Trauma, long bones 8. Steroid injection into nerve
*usually rapid painless visual loss; ++ retinal whitening, hemorrhages & CWS, but no disc swelling
**thought to be caused by complement-mediated leukoembolization

21) DDx of retinal lipid exudates

CONGENITAL ACQUIRED
1. Coat’s disease (non-inher, boys 85%, telangiectasias) 1. Diabetes
2. Juxtafoveal telangiectasis (Types I,II,& III) 2.HTN
3. Familial exudative vitreoretinopathy 3. CRVO
4. Macroaneurysm
5. Tumour (esp. hemangioma)
6. Radiation
7. Chronic RD
8. Neuroretinitis

22) Sixteen signs of hypertensive retinopathy

(HTN = BP>140/90; Scheie scheme: Gr. 0 = no retinopathy, Gr.1= barely detectable narrowing; Gr2= obvious narrowing
+ A-V nicking; Gr.3= hemorrhages + CWS; Gr.4= all previous + swollen disc)
1. Arteriaolar narrowing 9. ERM
2. A-V nicking 10. NVD/NVE
3. Cotton wool spots 11. VH
4. Hemorrhages 12. TRD
5. Macroaneurysms 13. Swollen disc
6. Exudate/Macular star 14. Elschnig spots
7. Vascular occlusions 15. Seigreist streaks
8. CME 16. Serous RD

23) DDx of patchy macular atrophy (not bullseye)

1. Congenital: cone dystrophy, Best’s, choroideremia, gyrate atrophy
2. Vascular: CSCR
3. Infectious/Inflammatory: histo, toxo, multifocal choroiditis, serpiginous
4. Toxic: chloroquine, radiation, phototoxicity, solar (usual tiny right in fovea)
5. Trauma: commotion retinae, choroidal rupture, macular hole
6. Degenerative/Structural: angioid streaks, myopic degeneration
…REMEMBER, AMD IS #1 BY FAR!
24) DDx of swollen disc & macular star
(a.k.a. DDx of neuroretinitis)
Cats In Heat Like To Spray Their Territory
1. Cat scratch disease (B. henselae; nodes; titres; biopsy & stain with Warrin-Starry silver; Rx: tetracyc)
2. Idiopathic (i.e. Leber’s stellate neuroretinitis)
3. HTN
4. Lyme disease (B. burgdorferi; nodes; rashes; joints; titres; Rx: tetracyc/erythro/pen)
5. TB (Mantoux test; Rx: rifampin, ethambutol, isoniazid)
6. Syphilis (VDRL + FTA-ABS; Rx: pen)
7. Toxo (T. gondii; titres; Rx: pyramithamine + sulfadiazine + folinic acid + prednisone; spiromycin if pregnant)
8. Tumour (esp hemangioma)

25) DDx of peripheral retinal hyperpigmentation

1. Congenital: RP + “KALA BLUR”, choroideremia, gyrate, Stargardt’s
2. Infectious: histo, toxo, syphilis, rubella, ARN
3. Inflammatory: multifocal choroiditis, serpiginous, sarcoidosis, Becet’s,
VKH, sympathetic ophthalmia, pars planitis
5. Trauma: PRP burns, radiation, old RD demarcation lines
6. Toxic: chloroquine, siderosis

26) DDx for RPE detachment

AMD is a BITCH
1. AMD 4. Tumour
2. Best’s disease 5. CSCR
3. Idiopathic polypoidal choroidal vasculopathy 6. Harada’s disease (VKH)
*An RPED is a dome-shaped elevation that may be fibrovascular, hemorrhagic, or serous… but only
AMD can present as ANY of these 3 types

27) DDx of Dalen-Fuch’s nodules

1. Vogt-Koyonagi-Harada syndrome 4. TB
2. Sympathetic ophthalmia 5. Syphilis
3. Sarcoidosis

28) DDx of three-level hemorrhages (subretinal, retinal, and pre-retinal +/- VH)
1. Diabetes 5. Macroaneurysm
2. PVD 6. Leukemia
3. Valsalva 7. Terson’s syndrome
4. Trauma 8. Shaken baby

29) DDx of rubeosis irides

1. Diabetes 5. chronic Coat’s 9. Radiation
2. CRVO 6. chronic ROP 10. Other rare retinopathy
3. CRAO 7. chronic RD 11. Systemic (anemia)
4. OIS 8. Tumour
30) DDx of crystalline retinopathy
CONGENITAL ACQUIRED
1. Bietti’s 1. Tamoxifen
2. Cystinosis 2. Canthaxanthin
3. Hyperoxaluria 3. Talc
4. Juxtafoveal telangiectasia 4. Calcified drusen

31) DDx of peripheral retinal neovascularization (peripheral NVE)

1. Congenital: ROP, FEVR, Incontinentia pigmenti, Norrie’s disease
2. Vascular: Diabetes, Sickle cell, CRVO, CRAO, Eale’s, any vasculitis
3. Inflammatory: sarcoidosis, pars planitis, toxo, birdshot, any vasculitis
5. Other: radiation, choroidal melanoma, retinitis pigmentosa,
retinoschisis, chronic RD, hyperviscosity syndromes

32) Nine signs of sickle cell retinopathy

(Sickle cell anemia = valine substituted for glutamic acid in B-polypeptide chain of HgB molecule; phenotypes are SS, SC,
AS, AC, and S-thal; HgB SC most often gets retinopathy, AS & AC only rarely; order protein electrophoresis on
hyphema pts of African or Mediterranean heritage)
1. Cotton wool spots 5. Sea-fan NVE
2. Salmon patch hemorrhages (intraretinal heme) 6. Macular ischemia (causes VA loss)
3. Refractile/irredescent spots (hemosiderin under ILM) 7. VH (second most common reason VA loss)
4. Black sunbursts (localized RPE hyperplasia) 8. TRD
9. Angioid streaks (DON’T FORGET!)

33) Causes of epiretinal membrane

PIE CRUST
1. PVD 5. Retinal tears
2. Idiopathic 6. Uveitis
3. Edema 7. Trauma
4. CRVO

34) Eight clinical features of epiretinal membrane

(20% of ERM’s are bilateral; 75% maintain BCVA >20/50; many are asymptomatic = no Rx; for those with symptoms of
decreased BCVA/micropsia/metemorphopsia; 80% will gain 2 lines of BCVA with PPV + membrane peel)
1. “Cellophane” retinal wrinkling 5. CME
2. Retinal folds 6. Retinal whitening (stasis of axoplasmic flow)
3. Dragging of vessels 7. Intraretinal hemorrhage
4. Foveal ectopia 8. Macular hole/pseudohole

35) DDx of white-centered retinal hemorrhages

Traumatic Leukemia DATE
1. Trauma 4. Anemia
2. Leukemia 5. Thrombocytopenia
3. Diabetes 6. Endocarditis

36) Causes of retinitis

CATS MEOV (…must be a Ukranian cat)
1. CMV (most common opportunistic infection in HIV; CD4<50 ; Rx = ganciclovir is BM toxic; cidofovir is renal toxic;
foscarnet ?; 85% respond, but resistence to meds/ recurrences/intolerance expected—just switch agents ; immune
reconstitution helps induce resolution; Histo = Cowdry-A intranuclear inclusions with clear zones (“Owl’s eye”))
2. ARN (HZO/HS1; healthy or immunocomp; rapidity of progression and/or health of the pt are what distinguish
ARN from CMV; “swiss cheese” retinal necrosis with complicated RD in 75%;Rx = admit, IV Acyclovir, ASA, Prednisone
after 3 days of acyclovir, PRP to edge to prevent RRD; if RRD occurs usually needs PPV + cryo + silicone oil)
3. Toxoplasmosis (self-limiting in healthy pts, so only Rx lesions 2-3mm from disc/fovea + debilitating vitiritis;
chronic suppressive Rx & CT head needed in immunocomp pts; Rx all lesions in kids!, Rx = pyramithamine is BM toxic;
sulfadiazine; folinic acid counters BM toxicity; prednisone after 3 days; alt agents = clinda, Septra, erythro, atovaqoune ; in
pregnancy only safe agent is spiromycin, but don’t treat if you don’t have to)
4. Syphilis (T.pallidum; 1 0
= painless chancre; 20 = fever, malaise, rash, joint pain, N&V; 30 = “neurosyphilis”,
seizures, stroke, confusion, CN palsies, optic atrophy, encephalitis; 10% will have ocular symptoms in Stage 2 – can
be anything, but uveitis most common; 60% have Argyll-Robertson pupils in stage 3; Rx = penicillin
5. Multifocal choroiditis (idiopathic, bilateral, anterior uveitis & vitritis differentiate it from histo;
widespread fundus lesions differentiate it from PIC); Rx = steroids
6. Endogenous endophthalmitis
7. Outer retinal necrosis, idiopathic
8. Vasculitis (ex. Behcet’s): (immune-complex occlusive vasculitis more common in Middle Eastern &
Asian pts; HLA-B5 & HLA-Bw51; “explosive panophthalmitis” often with hypoyon uveitis + retinal vasculitis + vitritis +
papillitis; Rx = steroid + dapsone + chlorambucil

37) Causes of retinal vasculitis

GLIMPSE PAST Behcets
1. GCA 7. Eale’s disease
2. Lupus 8. Polyarteritis nodosum
3. IBD 9. ARN
4. MS 10. Syphilis
5. Pars planitis 11. Toxo
6. Sarcoidosis 12. Behcet’s

38) Sixteen ocular signs of sarcoidosis

(chronic granulomatous inflammation; idiopathic; 50% get ocular involvement; TESTS= CXR, serum ACE, serum
lysozyme, serum Ca++, bronchial biopsy, conjunctival biopsy, Rx= steroids; Histo= non-caseating granulomas of
epithelioid cells and giant cells)
1. “Millet seed” lid granulomas 9. Vitritis
2. Conjunctival granulomas 10. Vasculitis/Peri-venous sheathing
3. Lacrimal gland infiltration 11. Vascular occlusion/hemorrhage
4. CN VII palsies (and others) 12. NVE
5. Granulomatous uveitis 13. Granulomas of retina/choroids/ONH
6. Koeppe & Busaca nodules 14. CME
7. PAS 15. ERM
8. Secondary glaucoma 16. Dalen-Fuch’s nodules (DON’T FORGET)

39) DDx of chorioretinal tumours

 Good Muslims Make Only Low-fat Halal Appetizers
1. Granuloma 3. Mets 5. Lymphoma 7. Astrocytic hamartoma
2. Melanoma 4. Osteoma 6. Hemangioma
40) Features of and management of choroidal melanoma
 Most common primary intraocular tumour
 Risk factors: older age, fair skin, blue irides, nevus of Ota
 Symptoms: asymptomatic, photopsias, metamorphopsia, decreased
VA from serous RD, VH, or direct obstruction
 Clinical features: 20% amelanotic, usually dome/mushroom shaped,
2mm-15mm thick, orange pigment on surface (lipofuscin), SRF,
CNVM, serous RD, VH, sentinel episcleral vessels, extraocular
extension, lenticular astigmatism, angle/CB invasion
 Risk factors for growth: 1) thickness >2mm; 2) Posterior tumour
margin touching disc; 3) Visual symptoms; 4) Orange surface
pigmentation; 5) Presence of SRF; 6) Absence of drusen or RPE
changes; 7) Pinpoint hyperfluorescence on IVFA (Shields says: 0 risk factors =
4% of tumours grow; 5 risk factors = 56% grow!)

 Ancillary tests: 1) B-scan most important test – demonstrates

“mushroom shape” from breakthrough Bruch’s; 2) A-scan shows low-
to-medium reflectivity (mets, osteoma, and hemangioma all have med-to-high reflectivity); 3)
IVFA shows “double circulation” of intrinsic tumour vessels
 Metastatic workup: LFTs, abdo U/S, CXR (only positive in ~1% at time of
diagnosis, but do anyway to establish baseline)

 Histopathology: employs Callendar system to classify based on cell

type: Spindle A = coffee-bean shape, 92% survival; Spindle B = oval
with small nuclei, 75% survival; Epithelioid (worst prognosis!) = round, owl-
eye cells, 40% survival; Mixed = mixture of spindle & epithelioid cells
 Treatment: 1) Small tumours: 1.5 – 2.5mm thick, >5mm diameter,
observation = 94% survival, but photo-document and follow closely &
best to treat any lesions with risk factors for growth; 2) Medium
tumours: 2.5 -- 10mm thick, <16mm diameter, enucleation or plaque
radiotherapy offer equal 80% survival, plaque therapy often
preferable b/c you keep the eye but may develop complications
(phthisis, cataract, glaucoma); 3) Large tumours: >10mm thick or
>16mm diameter, enucleation offers 75% survival, COMS study
showed no added benefit from pre-op radiation
 How do you tell nevus vs melanoma?: nevus present from early life,
no growth, usually <1.5mm thick no matter how large, presence of
drusen, RPE changes, and fibrous metaplasia all evidence of
chronicity & stability; do a B-scan to confirm <1.5mm thickness and
confined to choroid (i.e. no break through Bruch’s); if any “risk factors
 for growth” as above, watch very closely; assume growth =
malignant transformation

41) DDx of a vitreous hemorrhage

1. Vascular: Diabetes, Sickle cell, CRVO/BRVO, Eale’s, valsalva,
macroaneurysm, leukemia
2. Inflammatory: sarcoidosis, pars planitis, toxo, birdshot, any vasculitis
3. Traumatic: retinal tear, Purtscher’s, Therson’s, ON avulsion
4. Degenerative: PVD, AMD
5. Congenital: ROP, Coat’s, juvenile X-liked retinoschisis

42) DDx of vitreous material that isn’t blood or simple vitritis

1. Syneuresis 5. Lymphoma
2. Asteroid hyalosis 6. Amyloid
3. Cholesterolosis 7. Snowballs
4. Old VH 8. Vitreous veils in JXR

43) Reasons to do a vitrectomy

1. Diabetes (persistent VH/PVR after full PRP or preventing PRP)
2. Hemorrhage (or any persistent opacity)
3. To establish a diagnosis (suspecting lymphoma)
4. RRD (when PVR present, when GRT present, to aid visualization, for posteriorly located breaks)

5. TRD
6. Dislocated lens/Dropped nucleus
7. ERM
8. Macular hole
9. Endoretinal biopsy
10. Aphakic CME/ Aphakic vitreal-corneal touch

44) DDx of white chorioretinal nodules in the posterior pole

with associated vitritis
1. Toxo 5. Sarcoidosis
2. TB 6. Endogenous endophthalmitis
3. Syphilis 7. CNS Lymphoma
4. Lyme 8. Whipples
*depending on appearance: AMPEE & PIC (less nodular, less vitritis); MFC & birdshot (more peripheral than
just posterior pole); Cryptococcus/pneumocystis/mycobacterial choroiditis (all seen in immunocomp pts)

45) Signs & symptoms of a retained iron intraocular FB

1. Peripheral retinal pigmentation 6. Cataract
2. Vessel attenuation 7. Rusty stromal deposits
3. Optic atrophy 8. Nyctalopia
 4. Photoreceptor & RPE toxicity 9.VF constriction
5. Heterochromia 10. ERG (initially OK, then chronicly get
decreased B-wave, and eventually flat
ERG, therefore serial ERGs helpful in
NOTE: Iron does its damage via the Haber-Weiss reaction, management)
generating free radicals. Iron has an affinity for epithelium,
while copper has an affinity for basement membranes and
Descemet’s. Pure copper (85%) causes acute chalcosis and
severe inflammation, requiring immediate removal. Copper
alloy (brass, bronze) will cause sunflower cataract, Kayser-
Fleischer ring, heterochromia, metallic flecks on retina, and
greenish particles in aqueous & vitreous.

46) Management of endophthalmitis as per the EVS

 Better than LP vision, perform A/C and vitreous tap & injection of Abx
(Vanco 1mg in 0.1ml + Amikacin 0.4mg in 0.1ml OR Ceftaz 2.25mg in 0.1ml OR Tobra 0.1mg in 0.1ml)

 Vision is LP or worse, then perform immediate PPV (3X more likely to get BCVA
> 20/40, 2X more likely to get BCVA > 20/100, severe visual loss reduced by 50%)

 No benefit from IV antibiotics (Vanco + Amikacin)

 50% of all patients achieved final BCVA > 20/40.
 Poor prognosis if present with <LP vision, RAPD, old age, cornea
involved, abnormal IOP, diabetes, or NVI.
 Protocol for endophthalmitis Rx:
1. Complete ocular hx and exam.
NOTE: In the EVS, all pts were 2. B-scan if poor view
post-cataract surgery, all 3. Admit to hospital
presented <6wks post-op, all 4. A/C & vitreous tap for cultures (25g or 27g needle on TB syringe, take 0.1ml)
were only bacterial etiology, and 5. Vitreous injection of fortified antibiotics (Vanco 1mg in 0.1ml + Amikacin 0.4mg in 0.1ml OR
all received PO steroids as per Ceftaz 2.25mg in 0.1ml OR Tobra 0.1mg in 0.1ml) Inject 3.5 – 4.0mm from limbus.
#8. The only abx used were 6. Topical fortified antibiotics (Vanco 50mg/ml + Amikacin 20mg/ml OR Tobra 15mg/ml)
Vanco & Amikacin, for IV, topical, 7. Atropine 1% TID + Predforte 1% q1h
and injected. 8. Consider oral prednisone 30mg BID X 5-7 days, 24hrs after antibiotics given (as per EVS)
9. Follow q4-8h initially until stability/improvement seen. Refine abx as per culture results.

47) Factors that increase risk of RRD

1. High myopia 9. Lattice degeneration SIGNS OF ACUTE RRD: Schafer’s sign,
lower IOP, VH, corrugated retinal
2. Aphakia 10. Cystic retinal tuft appearance, presence of fixed folds
3. Acute PVD 11. Zonular traction tuft (=PVR), possible shifting fluid
SIGNS OF CHRONIC RRD: demarcation
4. Trauma 12. Meridional fold line, intraretinal cysts, atrophic thin
5. Pilo use 13. Enclosed oral bays retina, extensive PVR, hypotony, NVI

6. YAG capsulotomy 14. Peripheral retinal excavations

7. Family history
8. Previous RRD in other eye

48)How to differentiate senile retinoschisis vs RRD

 SRS has split in outer plexiform layer. RRD has split in photoreceptor layer.
 SRS associated with peripheral cystoid degeneration. RRD not (see #47).
 SRS associated with hyperopia. RRD associated with myopia.
 SRS usually bilateral, inferotemporal quadrant. RRD usually superotemporal.
 SRS has smooth domed surface. RRD corrugated.
 SRS has no Schafer’s sign. RRD does.
 SRS has absolute scotoma on VF. RRD has relative scotoma.
 SRS will blanch with argon burn. RRD will not.
 SRS has no shifting fluid. RRD may have.
 SRS may have outer holes (outer holes + inner holes = possible RRD, with
collapse of schisis cavity).
49) Contraindications to pneumatic retinopexy
1. Giant tear (> 900) or retinal dialysis
2. Unable to find all tears with certainty (pseudophakia)
3. Multiple tears over >5 clock hours
SF6 doubles in size in 1 day and lasts 2 wks.
4. Inferiorly located tears C3F8 quadruples in size in 2 days and lasts 8 wks.
5. Patient unable to properly position Air doesn’t expand and lasts about 1 week.
Regardless of whether you choose buckle, pexy, or
6. Uveitis PPV, success is generally good at ~90%.
7. PVR 80% Mac-on > 20/50.
8. Aphakia (relative contraindication) 40% Mac-off > 20/50, better if mac-off < 1 week.

50) Ocular manifestations of HIV

1. HIV +, without AIDS: asymptomatic non-infectious retinopathy
consisting of CWS, hemorrhages, and microvascular disease.
2. HIV + with AIDS, fundus pathology: 1) CMV retinitis; 2)
toxoplasmosis; 3) ARN (often presents with no vitritis/no vasculitis, just
rapidly progressive outer retinal necrosis = PORN); 4) Cryptococcus
choroiditis; 5) Pneumocystis carinii choroiditis; 6) Mycobacterium avium
choroiditis; 7) B-cell lymphoma
3. HIV + with AIDS, anterior segment pathology: 1) Kaposi’s sarcoma;
2) multiple molluscum contagiosum lesions; 3) severe HZO (often multi-
dermatomal or bilateral); 4) severe HSK; 5) microsporidial keratitis