The UIC Eye Manual

Produced by the faculty and residents at the Department of Ophthalmology and

Visual Sciences at the University of Illinois at Chicago

DISCLAIMER--PLEASE READ

While every attempt has been made to have dosages, medications, and treatment
recommendations as accurate and up to date as possible, this manual is no substitute for
clinical judgement and knowledge. UIC and the authors are not liable for any action taken on
the basis of this manual.

Cornea and External Disease

Revised by Diane Song MD

Editor Balaji Gupta MD

I. Basics

papilla
vascular response- central vascular tuft
if giant, the differential includes atopy, vernal, GPC
chronic GPC associated with soft CL, prosthesis, suture
follicles
lymphatic response- with active germinal center
with acute follicles, check lid margin for HSV vesicles, ulcers

acute chronic
EKC, pharygoconjunctival fever
medicamentosa (epinephrine, neosynephrine)
Parinaud's oculoglandular syndrome
HSV primary conjunctivitis
Newcastle's conjunctivitis adult inclusion conjunctivitis
toxic
r/o sarcoid
r/o GPC, vernal conjunctivitis
molluscum
membrane
true membranes suffuse epithelium, bleeds when stripped from the conjunctiva
pseudomembrane when removed, does not cause bleeding

causes of membranes:

erythema multiforme pharyngoconjunctival fever vernal conjunctivitis

alkali burn EKC bacterial conjunctivitis
ocular cicatricial pemphigoid, scleroderma
radiation burns
trachoma drugs sarcoid

filaments:

exposure (keratoconjunctivitis sicca, neurotrophic, recurrent erosion) patching

bullous keratopathy HSV
meds superior limbic keratoconjunctivitis
psoriasis aerosol keratitis
diabetes mellitis radiation
retained FB Thygeson's SPK
ptosis

enlarged corneal nerves:

MEN Type IIb icthyosis Hanson's

keratoconus Refsums Fuchs corneal dystrophy
old age failed PKP congenital glaucoma
trauma neurofibromatosis

MEN TIIb
AD with thick corneal nerves, medullary thyroid cancer, pheochromocytoma, mucosal
neuromas, and marfanoid habitus
thickened lid margin with rostral lashes, thick lips, epibulbar neuromas
cafe au lait spots, periungual, lingual neuromas
often confused with NF1
often die early from amyloid producing thyroid cancer in 10-20 year old with distant mets at dx
thick nerves precede the cancer!

corneal edema
whenever epithelium disrupted, can stimulate iritis via reflex arc
epithelial
intracellular first
intercellular with microbulla
then subcellular with frank bulla
stomal
all extracellular

factors
imbibition pressure = IOP - swelling pressure (nl 50)
fluid into cornea from IOP, gyclosoaminoglycans's
fluid out of cornea by dehydration, pump
IOP is inverse with swelling pressure
with nl endothelium, high IOP-- epithelial edema
with nl IOP, poor endothelium-- stromal edema

rx
mild
muro 128, hair dryer, control IOP
moderate
soft CL, cycloplegia, PK, conjunctival flap

Brown McClean syndrome

peripheral edema in aphakic, pseudophakic with orange pigment on endothelium

Stains
Flourescein staining when disruption of cell-cell junctions, loss of epithelial cells
Rose Bengal stains with disruption of precorneal tear film, leading to damaged epithelial cells.
Stains filaments (degenerated epithelial cells and mucus)
cell death increases permeability to these dyes, but Rose Bengal can still be blocked with tears

II. Lids/conjunctiva

Congenital

Epitarsus
fold of conjunctiva on palpebral lid

Osler Weber Rendu

hereditary hemorrhagic telangiectasia
AD, dilated blood vessels on palpebral conjunctiva in star/sunflower shape 1-3 mm, violacious,
blanches, increased with age
telangiectasia that bleed easily, rarely in retina and look like HTN or DM retinopathy
problems with epistaxis and GI bleeding

Congenital lymphedema
X-linked Recessive, AD
characterized by dilated lymphatic channels in the conjunctiva with edema
usually massive edema of legs
dysplasia of lymphatics

Medicamentosa

Anaphylactic
sulfonamides, bacitracin, anesthetic

Allergic
with eczema, SPK, red eye
atropine, homatropine, aminoglycosides, antivirals

Toxic
papilla, redness, SPK, no itch
often after 1 wk of use, especially keratoconjunctivitis sicca pts
aminoglycosides, antiviral, preservative

Follicular
big follicles, pannus, SPK
months to years later
pseudotrachoma syndrome
atropine, miotics, epinephrine, antivirals

Conjunctivitis

EKC adenovirus-
peak intensity days 5-7
stage ranges from superficial epithelial keratitis to subepithelial infiltrates (SEI)
subepithelial infiltrates are immune response to viral antigens on anterior stroma
can cause symblepharon
Rx: supportive, cold compresses, topical vasoconstrictors, antihistamines, topical steroids if
membranes and SEI's

EBV virus
belongs to herpes virus family
with mono can cause conjunctivitis, dry eye syndrome, epithelial keratitis (with dendrites),
stromal keratitis, OGS
Rx: resolve without treatment, antivirals not very active, can treat stromal keratitis with
topical steroids
Newcastle disease
virus with poultry exposure

measles
may have papillary conjunctivitis with white avascular spots on caruncle/conjunctiva like Koplik
spots in mouth
causes epithelial keratitis more often than stromal keratitis

Parinaud's OGS
catscratch
signs/symptoms
sneeze, mild fever, malaise, photophobia, irritation, FBS
papules within 3-5 days, becomes vesicular and crusting
2 wk latency, nodule in superior or inferior conjunctiva
intense chemosis, injection, lymph nodes may appear up to 2 wks later
ocular involvement usually unilateral
dx
Hanger Rose test 90% sensitivity skin test
Warthin Starry stain for bacilli
rx
Doxycycline 100mg bid x 1 month

tularemia
rabbit hunters, hx of tick bites with punched out lesion
signs/symptoms
lymph nodes, fever, chills, vomiting, pneumonia but ocular involved <5%
necrotizing, ulcerating conjunctivitis, corneal ulcer, optic neuritis, dacryocystitis,
panophthalmitis
dx
with agglutination titers 1:160 or higher in 2 weeks and peak in 4-8 wks
rx
streptomycin, tetracycline

sporotrichosis
signs/symptoms
spherical elastic movable nodule, pink then purple then black and necrotic
multiple subcutaneous nodules along lymphatics, multiple yellow nodules in conjunctiva
sporotrichosis conjunctivitis seen in HIV pts
no systemic illness
dx
culture on Sabouraud's
rx
KI 1 ml/day

Misc
sarcoid leptothrix chancroid
glanders Crohn's coccidiodomycosis
fungi lues TB
lymphgranuloma veneream

culture conjunctiva and scrape, blood cx if febrile, VDRL, FTA, PPD, viral titers, biopsy

Reiters
signs/symptoms
triad of bilateral conjunctivitis/ iridocyclitis, urethritis, polyarthritis
conjunctivitis may last days to weeks
fever, lymph nodes, pericarditis, pneumonitis, myocarditis
less commonly see keratoderma blenorrhagicum (scaling skin eruption)
think if chronic nonfollicular mucopurulent conjunctivitis
SPK, corneal infiltrate, corneal neovascularization
dx
clinical, at least 65%-75% are HLA-B27 positive
Rx
palliative, self-limited attacks last 2 to several months
may use steroids
?treat chlamydia/dysentary which may be triggered by Reiters

Floppy Lid
associated with obesity and sleep apnea
flimsy lax upper tarsus which everts easily
see papilla, punctate epitheliopathy related to exposure, keratoconus
Rx: shield eye, tape shut, or lid tightening surgery

SLK
signs/symptoms
burning, no itch or discharge, symptoms worse than signs
corridor hyperemia, velvety papilla upper tarsus
+Rose Bengal, micropannus, fine SPK, filaments in 1/2
50% with mild thyroid dysfunction
soft contact lens can also cause similar picture
rx
lubrication, punctal plugs
scrape conjunctiva, chemical cauterization with 0.5-1.0% silver nitrate, pressure patch, soft
contact lens, resect conjunctiva

Thygeson's keratitis
can often mimic SEI
recurrent hx of tearing, FBS, photophobia, decreased vision with quiet white eye
bilateral raised heaped up epithelium with microcysts seen in retroillumination can mimic HSV
coarse grey white lesions slightly elevated without flourescein stain, in central cornea
Rx
dramatically responds to topical steroids often after 2 doses, taper in one wk
some need chronic therapy due to rebound if steroids stopped
topical cyclosporin/viroptic

molluscum
SPK, pannus, follicles, pseudotrachoma
can curette, freeze or excise to produce bleeding
cryotherapy
HIV patients can have multiple recurrent lesions
in children, can avoid the needle
use topical anesthesia (2.5% lidocaine/2.5% prilocaine cream applied q10min x 2 and wait 15
min)
cryotherapy with small vitreous or large cataract probe
when ice ball forms, lift probe away from skin to avoid freezing deeper tissues
apply for 20 seconds and then repeat
post op antibiotic drops

Graft vs Host
Stage 1- conjunctiva hyperemia
Stage 2- chemosis, exudate
Stage 3- pseudomembranous
Stage 4 - corneal epithelial slough
higher stage correlates with increased severity of disease and mortality
keratoconjunctivitis sicca most commonly, cicatricial lag, ectropion, persistant epithelial
defects, iritis

Ligneous
infants and children are usually affected with females > males
may have family history
chronic condition with little pain or chance for visual loss except for secondary corneal
involvement
primary cause unknown, but patients seem to display aggressive inflammatory response
surgery can trigger the conjunctivitis
membranes or pseudomembranes form on the upper tarsus (thick white "woody" membrane
which is compact granulation tissue)
may have systemic symptoms including fever, hydrocephalus, other mucosal membrane
formation
Rx
surgical debridement followed by intensive topical heparin, steroids, and possibly alpha
chymotrypsin, ?mucolytics
topical cyclosporin
Chlamydia
trachoma, serotypes A-C; inclusion conjunctivitis, serotype E-K; LGV (lymphogranuloma
venerum, serotypes L1,L2, L3

Trachoma
superior pannus, SPK, corneal infiltrates,
lid destruction and exposure are key elements
serologic test unreliable because of prior exposure
tetracycline, erythromycin, or sulfonamides x 3 months
or azithromycin (single dose)

Stage 1:
incipient conjunctival follicles, punctate keratitis, superior pannus
cytoplasmic inclusion bodies

Stage 2: establishedinflammation
mature follicles, intense papillary hypertrophy
increased corneal pannus, SEI

Stage 3:
cicatrizing scarring Herbert's pits (limbal depressed necrotic follicles)
Arlt's line (conjunctival scarring)
symblepharon, trichiasis

Stage 4:
healedend stage, resolution of inflammation
cornea and conjunctival scarring
corneal erosions

Adult chlamydial conjunctivitis

keratoconjunctivitis in sexually active adults
5% with urethritis, 1-2 wk latency, meibomianitis, lid edema and redness
follicular conjunctivitis most prominent in lower conj
EKC-like SEI
NO membranes
superior pannus (not seen in EKC)
Rx
doxycyline 100mg bid 21 days or erythromycin 250mg qid 3 wks, treat partner
single dose azithromycin 1g may be effective
topical erythromycin drops
Neonatal chlamydial conjunctivitis
no follicles, more discharge, + pseudomembrane
4-12 days post partum, r/o GC
Giemsa inclusions bodies 40%, 90% with + clamydiazime
otitis, pneumonitis in 15%, recurrence 20%
Erythromycin syrup 50mg/kg qid x 2 wks, treat mom

adult chlamydial conjunctivitis neonatal chlamydial conjunctivitis

follicular reaction
no membranes
1-2 week latency after infection
5% associated with urethritis no follicles
pseudomembranes
4-12 days post partum
associated with otits, pneumonitis

LGV
Parinaud's OGS, follicular conjunctivitis, conjunctival granuloma
can have keratitis, corneal neovascularization, anterior uveitis

Allergy
Seasonal Allergic Conjunctivitis
signs/symptoms
rapid, lid swelling, chemosis (pale palpebral conjunctiva), itching, mucus, dellen
pressure, rhinitis/asthma, episodic
symptoms are much greater than signs
can sometimes be perennial with multiple overlying allergies
dx
clinical, Type 1 hypersensitivity only, elevated tear IgE, eosinophils in scraping in chronic cases

rx
steroids rarely indicated
antihistamines systemic and topical are useful
topical NSAIDS
mast cell stabilizers if chronic condition

Vernal (VKC)
bilateral seasonal young (3-25 year old) in warmer climates, M>F
FHx of atopic allergies
usually self limited, average 4-10 years
two types: vernal and palpebral
signs/symptoms
ITCH (worsens in evening, dust, lights, wind, rubbing), clear tears
ropy discharge but lids don't get crusted or stick together unless bacterial superinfection
giant papilla may cause ptosis
limbal involvement more in blacks and can be up to 360 degrees
Horner Trantas dots (clumps of degenerated eosinophils), clear elevated cysts
SPK, flour dusting of epithelium, intraepithelial cysts, shield ulcers usually upper cornea
pseudoarcus, myopic astigmatism, associated with keratoconus, rare corneal
neovascularization
>2 eosinophils/hpf pathognomonic, increased tear histamine
eosinophilic products are major cause of corneal epithelial destruction
rx
environmental control
lodoxamide 0.1% QID is first line drug
topical steroids- pulse rx with exacerbations
topical cyclosporine 2% qid can be used as alternative to steroids

Atopic (AKC)
keratoconjunctivits in patients with atopic dermatitis
M>F, teens to 40's, burns out by 40-50 year old, small papilla, milky edema, corneal
neovascularization
hx of atopic eczema (3% of population), similar to vernal findings but no seasonal changes
signs/symptoms
atopy shiners (bags under eyes from rubbing)
symblepharon, foreshortening of inferior fornix, usually lower palpebral conjunctiva affected
can mimic OCP
in severe cases bilateral cataracts (anterior subcapsular, or posterior polar), 10% of all cases
atopic dermatitis associated with keratoconus, iritis, cataract
RD from pars plana tears or ora dialysis (can have photoreceptor outer segments in anterior
chamber which look like cells)
diagnosis
increased serum IgE, few eosinophils in conjunctival scraping and rarely free granules
conjunctival biopsy
pathology
increased T helper, macrophages, increased class II HLA similar to OCP and rosacea.
more complex than simple mast cell allergic rx.
No BM deposition as in OCP
rx
environmental control is essential
systemic antihistamines, nasal cromolyn, topical mast cell stabilizers
pulse steroids and cream
doxycycline for blepharitis
oral and topical cyclosporin (severe cases may need immunosuppression)

general guidelines for treatment of allergic conjunctivitis

can use stepped approach depending on severity of condition
allergy testing and environmental control is essential
cool compresses and vasoconstrictors for symptoms
topical medications
antihistamines
levocabastine- 0.05% QID for up to 2 weeks, potent H1 receptor antagonist
mast cell stabilizer
lodoxamide- 0.1%, gives some symptomatic relief within two to three days, has some effect
on stabilizing eosinophils
cromolyn 4%
not for acute phase because therapeutic effect requires several weeks of use
nonsteroidal anti-inflammatory med
acular- 1 drop QID
pulse steroids
oral medications
oral antihistamines (such as claritin 10mg QD)
immunosuppressives
for sight threatening conditions

GPC of CL
usually develops within the first year of lens wear, especially with extended wear lenses
also seen in artificial eyes, suture ends
r/o VKC (no tear histamine, no free eosinophil granules, only 1/4 have eosinophils in scrapings
in GPC)
papilla apices may stain (sign of activity)

mucous discharge mild itch pain with CL

blurred Va whitish material on lens ptosis
conjunctival injection chemosis thick sheets of mucus
soft > hard CL
Rx
antihistamines, mast cell stabilizers, topical steroids
switch brands/ types of lenses, increase use of enzyme to twice weekly, stop lenses

Contact dermatitis
erythema, itching, scaling of lids, papilla
chronic meds (Neomycin most commonly), metals, cosmetics, false lashes cement, fingernail
polish

Blepharitis

Staphylococcal
Seborrheic
Meibomian Gland Dysfunction (MGD)
Rosacea
- younger, F (80%)
- short duration
- dry eyes
- purulent discharge
- collarettes, scales
- ulcers at base of lashes
- absent, thin, broken, misdirected, or white lashes
- papilla
- marginal ulcers
- older patients
- more chronic
- dry eyes
- crusting
- oily margins
- papilla, follicles
- dermatitis
- patients with acne rosacea
- more burning
- conjunctival injection
- bulbar injection
- SPK, Rose Bengal stain
- females age 30-50
- seen in 60% of cases of MGD
- rhinophyma, telangiectsia, pustules
- erythema
- marginal keratitis
- inferior corneal pannus with subepithelial infiltrates

Rx
all get hot compresses and lid scrubs bid
can try antibiotic drops qhs to qid, if severe may use antibiotic/steroid combination drop
treat MGD assoc. with rosacea with doxycycline 100mg bid for 1 month, or tetracycline 250mg
qid/ 500mg bid for 1 month with or without topical steroids
chronic doxycycline antibx associated with vaginitis, allergy, photosensitivity, take on empty
stomach, no breastfeeding
children and pregnant or nursing women must not receive systemic tetracycline. alternatively,
may use erythromycin

Subconjunctival hemorrhage
resolves in 1-2 weeks
usually not significant and no identifiable cause, but if repeated episodes of subconj.
hemorrhage, consider the following:

anemia hypertension conjunctivitis decreased platelets

DM menses nephritis subacute bacterial endocarditis
trauma trichinosis valsalva vascular anomaly

Superglue in eye
toxic to endothelium
cold water compresses to loosen adherance
wait 3-4 hours, can cut lashes

Kaposi's sarcoma
conjunctival stromal tumor which appears as reddish-blue vascular macules or nodules
Grade I,II patchy and flat and <4 month duration, Grade III > 3mm thick
on bulbar conjunctiva or eyelid
histology: spindle-shaped cells with oval nuclei and capillary channels
Rx
standard rx with radiation
consider double freeze thaw cryo for Grade I, II on eyelids
simple excision of conjunctiva for bulbar conjunctiva leaving bare sclera
excision s/p vessel delineation by flourescein angiogram for Grade III on conjunctiva
chemotherapy

III. Cornea

Congenital Anomalies

megalocornea
corneal diameter >13mm, bilateral, most commonly X-linked rec., (therefore 90% are male)
usually isolated, nonprogressive
r/o glaucoma, increased risk factor for ectopia, cataract, glaucoma
rarely associated with renal cell cancer, r/o congenital glaucoma
nl endothelial density
F carriers may have slightly larger cornea

microcornea
corneal diameter <10mm, hyperopes (because cornea is flat) AD>AR
20% with angle closure glaucoma, usually eye nl
r/o nanophthalmos, microphthlamos, trisomy 13, Ehlers Danlos, dwarfism, fetal alcohol
syndrome
Anterior segment dysgenesis
a broad spectrum of developmental anomalies invloving migration of the mesenchyme of
neural crest origin
Axenfeld anomaly-Reiger syndrome:
Posterior embryotoxin
thickened, centrally displace schwalbe's line
usually AD, normal eye development
Axenfelds
anomaly: posterior embryotoxin plus prominent iris processes
syndrome: hyperteloric, small shoulders, facial asymmetry, 1/2 with glaucoma
Rieger's
anomaly: posterior embryotoxin, prominent iris processes, plus iris stroma atrophy
syndrome: with maxillary hypoplasia, telecanthus, microdontia, partial adontia, skeletal and
spine deformities
craniofacial anomalies, mental retardation, Marfan's, 1/2 with glaucoma

Peters
80% bilateral (although this is debatable)
clearing of leukoma with waiting, glaucoma
Type 1- usually unilat., defect in descemet's membrane, central corneal stromal opacity, nl
lens and no systemic involvement
Type 2- commonly bilat., lens involved, bilateral, often glaucoma
r/o von Hippel's internal corneal ulcer (no lens abnormality) and local posterior keratoconus
(endothelium/descemet present). Neither are assoc. with iris adhesion.

Haab's striae
breaks in decemet's associated with congenital glaucoma
striae are horizontal opposed to striae associated with forceps injury during delivery, which are
vertical

Congenital opacities
facets, nebula, macula, leukoma
corneal keloid probably from intrauterine trauma

sclerocornea
peripheral sclera-like opacification of the cornea
results from defect in mesenchymal migration
bilateral, often with systemic and other ocular problems
nonprogressive, sporadic, AR (more severe) or AD

Infections

Viral
HSV Primary
conjunctivitis in children, bilateral, fever, preauricular adenopathy, URI, 3-9 days incubation
varied present (mild follicular conjunctivitis to pseudomemb)
lid vesicles, ulcerative bleph, 1/2 get small fleeting corneal dendrites
SPK, conjunctival dendrites

HSV Recurrent
may be triggered by sunlight, fever, stress, menses, steroids
1 yr 25%, 2 yrs 50% chance of recur
prevalence in U.S. is 500,000 people, one fifth develop stromal keratitis
most common infectious cause of corneal blindness in this country
blindness from HSV is from recurrent stromal keratitis and/or iritis with the subsequent
inflammation and scarring
lids (pseudozoster)
rare follicular conjunctivitis
epithelial keratitis
SPK, dendritic, geographic, marginal
shaggy borders, ghost scars of prior dendrites, decreased corneal sensation
often with mild stromal edema
metaherpetic lesion from poorly healing epithelium, gray thickened heaped up edges
usually resolves spontaneously in 3 weeks but treat to decrease the amount of damage
RX
debride with dry cotton tip applicator
viroptic 9x/day taper off 2 wks to avoid toxicity
see in weekly, don't overtreat
limbal lesions resist antivirals, slower healing

diff dx of dendrite

contact lens filaments

HZO Thygeson's
tyrosinemia Type II healing corneal abrasion

stromal keratitis: disciform

disc shaped zone of corneal edema
presumed immune mediated disease
central corneal edema with fine KPs under edema, mild iridocyclitis, increased IOP, Wessley
ring (of infiltrates)
diff dx includes HZO, local bullous keratopathy, EBV virus, connective tissue disease, interstitial
keratitis
consider obtaining antiherpes serum antibodies in patients with no hx of herpetic eye disease
or when the diagnosis is in question
Rx
cycloplegics
topical steroids with topical antiviral cover tapered slowly (Pred Forte 8X/day initially tapered
down to 2X/day by 2 months)
usually self limited in 2-6 months with variable scar

stromal keratitis: peripheral

atypical, r/o marginal ulcer
?association with HIV
corneal neovascularization, interstitial keratitis

stromal keratitis: necrotizing

presumed etiology from live virus but can be negative on biopsy
dense deep stromal infiltrate, multiple cheesy white, necrotic infiltrates
no pain, mild iridocyclitis, increased IOP, corneal neovascularization, indolent
self limited 2-12 months
antivirals with steroids and slow taper (even yrs)

endotheliitis
progressive corneal edema with line of KP (looks like PKP rejection, but PKP rejection line is
limited to the corneal graft)
uveitis, increased IOP
acyclovir

uveitis
iritis with diffuse iris atrophy
multifocal choroiditis

HEDS
multiple prospective multicenter trials with the summaries of the studies listed below:
for patients treated with topical trifluridine, topical steroids are beneficial for treatment of
stromal keratitis (decreased persistence/progression of disease)
for patients treated with topical trifluridine and topical steroids, addition of oral acyclovir
offered no benefit for stromal keratitis (however, beneficial for iritis)
for patients with HSV epithelial keratitis treated with topical trifuridine, no apparent benefit of
3 week course of oral acyclovir in preventing HSV stromal keratitis or iritis was seen during the
subsequent year

complications of HSV infection

neurotrophic ulcer
decreased visual acuity from scarring or from irregular astigmatism
lipid keratopathy
corneal perforation
if PKP needed, preferable to wait until decrease in active inflammation

HZO primary
chickenpox
rare disciform keratitis
SPK
limbal pustules
lid lesions
usually benign, may treat if corneal involvement

HZO Secondary
2% of adults > 60 year old, is not harbinger of cancer in healthy people
pathology: nerve damage, ischemic vasculitis, inflammatory granulomatous rx
lid vesicles clear in 3 wks and can result in ptosis
conjunctival hyperemia, vesicles following a dermatome, episcleritis
Hutchinson's sign- vesicles on the tip of the nose, indicates involvement of the nasociliary
branch of V1
SPK, microdendrites, corneal neovascularization, uveitis, glaucoma, sectoral iris atrophy
stromal nummular keratitis, scleritis (nod>diff, limbal can spread to cornea)
1% with optic neuritis, Horner's, EOM palsy (25%)
decreased corneal sensitivity leads to neurotrophic ulcers
in young, rule out HIV

Rx
cycloplegic, Acyclovir 800mg 5x/day x 7 days try to start within 72 hrs
topical steroids for iritis
Zovirax or warm Borow's compresses qid
consider prednisone 60-100 mg po x 3 days
pain medication
r/o HIV especially if < 45 year old or risk group
if microdissemination, pt may be immunocompromised consider IV steroids, use IV acyclovir

HZO Neurological syndromes

postherpetic neuralgia
pain decreased with time (80% gone in 1 yr), worse if pt is older
Rx capsaicin, stellate ganglion block, cimetidine, carbamazepine, tricyclic antidepressants
necrotizing angiitis
with contralateral hemiplegia and death in 15% wks to months later
PML-like syndrome
with seizures, decreased function, and death

Bacterial keratitis

risk factor: CL, trauma, atopy, prior HSV, dry eyes/exposure, bullous keratopathy, OCP,
Stevens Johnson syndrome, abnormal lid position
Staph/Pseudomonas are more than 50% of all cultured cases
Worst are Pseudomonas, Strep (B-hemolytic, pneumoniae)
direct epithelial penetration by GC, H egypticus, Diptheroids, Listeria
Staph - gram positive cocci
- ulcer is usually localized with distinct borders
- may satellite
- S. aureaus (coagulase-positive) causes deeper ulcers, more inflammation, hypopyon, and
endothelial plaque than S. epidermidis (coagulase-negative)

Streptococcus
pneumoniae - gram positive cocci in pairs and chains
- acute or subacute purulent conjunctivitis
- formerly the most common cause of ulcerative keratitis
- may develop inflammatory membranes

Neisseria gonorrhoeae
- gram negative cocci
- hyperacute onset
- most often a progression from conjunctivitis
- profuse mucopurulent discharge
- can penetrate epithelium directly
- may cause perforation

Moraxella - gram negative rod varying from coccobacilli to dumbbell shaped diplobacilli
arranged in boxcar-like chains

- more chronic, indolent keratitis

- in chronically debilitated patients

- peripheral or paracentral infiltrate

- consider calcium alginate swab soaked in trypticase soy broth

blood, chocolate, thio, sabourouds for fungus

Pseudomonas - gram negative rod

- frequent cause of bacterial keratitis
- mostly associated with contact lens
- rapid progression and perforation often occurs in few days
- ulcer with surrounding stromal edema and diffuse inflammation

Haemophilus - gram negative coccobacilli or rods

- no membranes
- purulent conjunctivitis and preseptal cellulitis in children
- often with purplish discoloration of skin
- not as common now with immunization

Phlyctenular ulcer
pinkish white limbal elevation with gray crater, can be on conjunctiva
type IV immune mediated response
caused by: staph, TB, Candida, Chlamydia, nematodes, gonococcal antigens, adenovirus
early Terrien's can look similar
Rx: treat the infection if active, topical steroids used to minimize scar formation

Staph Marginal Infiltrates

type III immune reaction
gray limbal ulcer usually with clear cornea all around, early corneal neovascularization
allergic rx usually to staph at 8/10 and 2/4 o'clock
can progress to ring ulcer, usually spread toward limbus not centrally
can get superinfected

atopy Crohn's/ UC hookworm porphyria

Wegener's influenza scleroderma brucellosis
periarteritis nodosa GC arthritis SLE acute leukemia
dysentery dengue fever gold poisoning rheumatoid arthritis

Infectious Crystalline keratopathy

poor response to topical therapy

biopsy usually for diagnosis
feathery "crystalline" edge, assoc. with chronic steroids, (eg. PKP)
alpha strep viridens,
rx with concentrated topical bacitracin
Acanthameoba
found in water, existing as a cyst or trophozoite
risk factor contaminated CL, corneal trauma, r/o topical anesthetic abuse
mimics HSV early, later ring infiltrate, often misdiagnosed as HSV
symptoms: wax and wane with photophobia, FBS, severe pain
signs: first abnormal epithelium (SPK, persist defects, SEI, edema, whorl like patterns,
dendritiform lesions)
then central/paracentral stromal infiltrate with satellites, turns into ring
neurokeratitis with cuffing of nerves
preauricular adenopathy
Dx: biopsy , stains (Giemsa, calcofluor white, acridine orange) are faster and better than
culture
touch material instead of smearing on slides
use spray fixative instead of air drying
IFA, calcofluor white, conjugated lectin flourescein stain
culture on non-nutrient agar with E. Coli overlay

Rx: difficult, often fails, use multidrug approach

antiseptic (PHMB or chlorhexidine) + Brolene 0.1%, neosporin
add clotrimazole 1% q15-q1h
+/- ketoconazole 200 mg po bid or fluconazole po
no steroids
once infection in peripheral cornea, poor candidate for graft

Fungal
trauma especially with exposure to plants or soil
south, mostly filamental, Fusarium, Aspergillus >50 %
Candida 10% in older, keratoconjunctivitis sicca, looks like bacterial

signs: feathery edges, dry gray infiltrates appearing slightly elevated above the corneal surface
endothelium plaque, satellite lesion
can penetrate Descemet's

Dx: stains with calcofluor white, 10% KOH wet mount

cytologic evaluation of corneal scrapings, Gomori methenamine silver or PAS
culture Sabouraud's and blood agar
biopsy may be necessary for diagnosis, bx needs to include at least 1/2 to 2/3 thickness of
cornea, bx from edge of infiltrate

Rx: first-line for filamentous fungi is now fluconazole with good penetration (formerly, first line
for filamentous fungi was natamycin 5% susp (50mg/ml) q1hr)
second-line drugs include amphotericin B 0.15% (1.5 mg/ml) or imidazoles (miconazole 1% or
ketoconazole 1-5%)
first-line for candida is ampho alone or in combination with flucytosine, do not use flucytosine
alone because of high resistance
second-line for candida is imidazoles
consider PKP (include wide margin beyond infiltrate), if possible treat keratitis medically first

interstitial keratitis
corneal stromal inflammation, scarring, and vascularization
syphillis
90% congenital lues with eventual bilateral involvement in 10-20 year old, can be recurrent
acute stage- salmon patch of Hutchinson, KP, stromal infiltrate, corneal edema, pain,
photophobia, discharge
often with Hutchinson's teeth, deafness, salt/pepper retina, ON atrophy
usually present endstage with ghost vessels, guttata like bumps, retrocorneal
hyaline ridges, fibrous strands into anterior chamber
10% acquired, unilateral, often sectoral, 10 yrs after infection
rare focal avascular interstitial keratitis with vessels
CL induced corneal neovascularization looks very similar
get serology, PPD, and rx for latent lues if present

Cogan's syndrome
bilateral intersitial keratitis associated with vestibuloauditory symptoms
tinnitus, hearing loss, balance problems
may also involve arteritis (polyarteritis nodosa), vasculitis, and necrotizing lesions that leads to
endocarditis and gastrointestinal hemorrhages
remember congenital lues is also associated with decreased hearing
serology

other conditions associated with interstitial keratitis

TB leprosy Hodgkins
mumps LGV sarcoid
HSV lyme disease Incontinentia pigmenti
HZO Kaposi's sarcoma protozoan/helminths

Degenerations

Pinguecula
elastoid degeneration and fragmented subepithelial collagen fibers of the bulbar conjunctiva
usually nasal, interpalbebral zone of older patients
may be associated with chronic sun exposure

Pterygia
similar histology as pinguecula, but extends from the conjunctiva to involve peripheral cornea
risk factor: UV exposure especially before 5 years of age, no glasses, no hat, equatorial living
Rx: excision, radiation, conjunctival transplant or flap, mitomycin
high rate of recurrence therefore surgery for astigmatism, VF changes, restricted EOMs,
inflammation, severe cosmetic defect

Amyloidosis
deposition of noncollagenous protein
subepithelial, salmon color, avascular
primary: tend toward mesenchymal deposit
secondary: organ deposit
metachromasia crystal violet, flourescent thiaflavine T, birefringence/dichroism Congo Red,
+with Siruis Red
primary localized
most common form with palpebral conjunctival asymmetry (brown/yellow waxy firm subconj
nodules)
lattice is special form
does NOT affect lids, but can be orbital presenting as VI nerve palsy
primary systemic
bilateral symmetric yellow or ecchymotic lid papules, light near dissociation
vitreous opacities, EOM palsies, proptosis, glaucoma
secondary local
after chronic inflammation, trichiasis, keratoconus, granular dystrophy
salmon to yellow, fleshy, waxy nodular lesion on cornea
secondary systemic
most common in general medicine and doesn't usually affect eye
lids may be purpuric

Involutional
arcus, hassal-henle bodies, Vogt's limbal girdle
farinata (lipofuscin, dots, commas in deep stroma)
crocodile shagreen

Deposits

Band Keratopathy
deposition of calcium salts in Bowman's
commonly associated with:
local inflammation: dry eyes, uveitis, long standing glaucoma, interstitial keratitis, phthisis
ophthalmic medications: pilocarpine
systemic disorders: chronic disease, conditions associated with systemic increase in Ca, Phos,
mercury such as hyperparathyroidism, excess vit. D ingestion, renal failure, sarcoidosis, milk-
alkali syndrome, thiazide, bone mets
other causes: gout with urates, discoid lupus erythematosis, tuberous sclerosis
Rx: removal with 3% EDTA, give topical anesthetic then EDTA for 1 min, scrape and sponge
until it clears

Spheroidal degeneration
bilateral M>F, golden brown spheres in anterior stroma/Bowmans in palpebral zone
risk factor age, UV exposure, probably elastotic degeneration of collagen
symptoms: decreased visual acuity, FBS, irritation
signs: usually not elevated, small lesions, can be in conjunctiva

Salzmann's nodular degeneration

usually unilateral, may be bilat. F>M middle age
gray white elevated subepithelial nodules at end of old pannus paracentral cornea
adjacent to previous corneal conditions but can also be isolated
asymptomatic or cause tearing, photophobia, or decreased Va
Rx: local excision +/- excimer smoothing

Coat's white ring

subepithelial or anterior stromal circular white ring associated with metallic corneal foreign
body
iron present on histopathology
no treatment necessary

Lipid Keratopathy
unilateral or bilateral, occurring in areas of vascularized scars especially surgical
often elevated, nodular, yellow between Bowmans and epithelium

Farinata
flour like dusting anterior to Descemet either central or entire cornea, usually bilateral
products of cellular degeneration
not affecting vision

Marginal thinning

Mooren's
idiopathic unilat painful inflamed eye often inferiorly
can have decreased visual acuity
trauma hx, rare perf, circumferential spread early
signs: usually not elevated, small lesions, can be in conjunctiva
central edge undermined in stroma, blunt edge peripherally
two types: -benign, unilateral, in older, responds to surgery -relentlessly progressive and
bilateral in 25%, young (some contest this categorization)
Rx: steroid (topical, systemic) lamellar keratoplasty with conjunctival resection,
immunosuppressives
glasses and eye shield
check for Hepatitis C Ag

Terrien's
(NOT an ulcer)
quiet thinning superiorly with fine micropannus can spread 360 degrees
commonly in adults >50 year old, but can occur at any age M>F, bilateral but more advanced
in one eye
steep central wall, mild inflammation
later, lipid deposits at edge of pannus, epithelium intact, decreased Va from astigmatism
rare perforation
Rx: mild steroids chronically to suppress inflammation
lamellar or PKP
Marginal keratolysis
autoimmune disease, most commonly rheumatoid arthritis
unilateral, inferiorly, may have infiltrate
can have rapid progression
stops if epithelium heals

Age related furrow

lucid areas of arcus, no inflammation, vessels, or perforation

Dystrophies
Anterior
MDF
maps- thickening of subepithelial collagen and basement membrane, usually negative
flourescein staining
dots- cyst like spaces in epithelium containing cellular debris
fingerprints- lines in deep epithelium best seen with retroillumination
>30 y.o. 10% have corneal abrasions
50% of pts with recurrent corneal abrasions have MDF
asymptomatic or present with recurrent erosions, rarely presents with irregular astigmatism
Rx: hypertonic saline, debridement with cotton swab
lubricants, soft contact lens, excimer PTK effective
anterior stromal puncture
topical anesthetic, debride area
use 23g needle and penetrate anterior stroma up to 1/3 depth
may perform even in visual axis but space punctures further apart
antibiotic ointment and pressure patch
warn pts about extreme pain afterwards

Meesman's
AD dystrophy with thick BM, intraepithelial microcysts with peculiar PAS+ substance best seen
on retroillumination
asymptomatic until 10-20 y.o. when develop recurrent erosions, irritation, small decreased Va
no rx necessary
differential diagnosis includes cystinosis (metabolic defect resulting in accumulation of
refractile cystine crystals, can be seen in peripheral cornea)

Reis-Bucker's
AD, childhood, gray white opacities at Bowman's layer, becoming more confluent over the
years
histology: destruction of Bowman's layer and accumulation of "rod shaped bodies" replacing
Bowman's layer
reticulated pattern of scarring
painful recurrent erosions, by 50's marked corneal opacities
Rx: excimer PTK, peeling off the superficial accumulated material, recur post PKP
Stromal
mnemonic: Marilyn Monroe Always Gets Her Man in LA County.
Macular dystrophy- Mucopolysaccharides stains with Alcian blue
Granular dystrophy- Hyaline stains with Masson's trichrome stain
Lattice dystrophy- Amyloid stains with Congo red

Macular
*AR, peripheral cornea involved*
decreased Va in childhood with diffuse stromal clouding
mucopolysaccharides accumulates
type I: error in synthesis of keratan sulfate
type II: can synthesize keratan sulfate but 30% below normal
gray white opacities with indefinite margins
may involve full thickness of stroma, guttata
stains with alcian blue, colloidal iron stain
can recur after PKP

Granular
AD, onset in childhood with decreased Va later (>40 y.o.) >20/200
milky deposits in central cornea, anterior stroma, with intervening clear areas
with time, the center of opacities becomes more white; "snowflake" like appearance
accumulation of hyaline stains with Masson's trichrome stain
periphery may be + for amyloid (ancestry to Avellino, Italy)
may recur yrs after PKP
often amenable to PTK

Lattice (Amyloid)
childhood, central lines, dots, haze (ground glass)
recurrent corneal abrasion, decreased Va by 40 years old

Type I: (Biber-Haab-Dimmer) Type II: (Meretoja) Type III Type IIIa

- AD
- type AA amyloid
- can be deep, spares limbus
- retroillumination, starts as dots
- no systemic amyloid deposition - AD
- coexistent systemic amyloidosis,
- with progressive cranial neuropathy
- skin changes
- AR
- type AP amyloid
- thicker lines across entire cornea easily seen
- later onset
 - similar to type III but inherited as AD
- frequent corneal erosions

deposits of amyloid stain with congo red, PAS, and Masson's trichrome stain
recurrence post PKP common

Central crystalline (Schnyder)

clear cornea at birth then increase polychromatic crystals in stroma by age 1
doughnut like crystals are cholesterol and fats
arcus and vogt limbal girdle
visual acuity is normal
Oil red O stain, frozen section
associated with genu valgum, hyperlipidemia, xanthelasma

Fleck
uncommon, nonprogressive stromal dystrophy
normal Va, no symptoms
begins in childhood
bilateral but asymmetric
gray white dandruff-like opacities to periphery, increased glycosaminoglycans, lipids
associated limbal dermoid, keratoconus, central cloudy dystrophy, pseudoxanthoma
elasticum, decreased corneal sense, atopy, cortical lens changes

CCD (central cloudy dystrophy)

bilateral symmetric
nonprogessive or very slowly progressive
multiple nebulous gray areas with cracks of intervening clear zones
normal vision

polymorphic stromal dystrophy

bilateral symmetric
progressive late in life
normal Va, associated with systemic amyloidosis

Pre-Descemet farinata
pre-Decemet degeneration seen in late adulthood
deep stromal punctate specks above Descemet's
O-red-O+, Sudan black+, and PAS+,
normal Va

Posterior amorphous stromal dystrophy

rare bilateral child with good Va
extends to limbus, iris processes, endothelium disrupt focally
peripheral variant with clear center

CHSD (congenital hereditary stromal dystrophy)

flaky feathery anterior central opacity at birth
can have decreased Va

Posterior
corneal guttata
thickening and localized excresences of Descemet's membrane
results from abnormal production of collagen by endothelial cells
"beaten metal" or "orange peel" appearance
do not affect vision but may lead to endothelial dysfunction and corneal edema

Fuchs's dystrophy
AD, F>M, (most common in post menopausal women)
bilateral and asymmetric, rare in Orientals
central guttata first, pigment in endothelium (can have borders)
1st degree relatives 40% with guttata, incomplete penetrance
worse Va in mornings, humid days, increased IOP
rarely pigmented gutatta can decreased Va (20/60 range)
in long standing cases, subepithelial fibrosis, grayish Descemet thickening
posterior collagenous layer can obscure all the guttata
diff dx of corneal edema- other endothelial problems, PPMD, disciform keratitis
Rx
only if symptomatic, treat as in other causes of corneal edema
if considering phacoemulsification check corneal thickness

PPMD, posterior polymorphous dystrphy

AD bilateral childhood, progressive, asymmetry
presents as a spectrum of corneal changes that occasionally involve iris and angle
endothelial cells act like epithelium, may look like ICE
posterior surface with ridges, lines and circles with scalloped edges, stromal edema,
iridocorneal adhesions
best seen in retroillumination

CHED (congenital hereditary endothelial dystrophy)

bilateral congenital corneal edema, Descemet's thick
due to degen of endothelial cells at 5 months gestation
Type I: AR, most common, no pain or tearing
stationary, diffuse, often present at birth associated with nystagmus
Type II: AD, presents at 1-2 year old, painful tearing, progressive, less corneal edema, no
nystagmus, cornea blue ground glass, associated with deafness
Ectatic

keratoconus
central or paracentral corneal thinning and bulging
sporadic, can be familial (<10% of occur in blood relative)
bilateral but asymmetric
10-30 year old
associated with Down's, Leber's, atopy
signs: Vogt's striae, Fleischer ring- iron deposits at the base of the cone, scarring, early
astigmatism

Ehler Danlos VI
keratoglobus, spontaneous perforation of globe, blue sclerae
keratoglobus
thinning greater in periphery near the apex of the protrusion, mild scar, not genetic,
connective tissue disorders
associated Ehlers-Danlos type VI

Pellucid marginal degeneration

bilateral clear inferior or superior thinning 2 mm from limbus, 2mm in width
20-40 y.o., presents as decrease in visual acuity associated with against the rule astigmatism
no iron ring, no cone but cornea protrudes above thinned area, no scar, striae, avascular
Rx: eccentric penetrating graft, resect thinned area, or crescent shaped peripheral lamellar
keratoplasty

IV. Misc

dry eyes
80% show excess mucus, thinned tear film with debris, SPK, corneal mucus plaques and
filaments
Rose Bengal stain, tear break-up time unreliable, meniscus height variable
bothered by wind, reading, smoke, sleep, hx of skin diseases, tearing/ mucus
check conjunctival scarring, V, VII CN, avitaminosis A, lagophthalmos, sarcoid
medications implicated include antihistamines, HCTZ, antibx, Inderal, Valium, Pyridium,
Timoptic (damages mucus layer, decreased goblet cells), oral contraceptives
up to 30% of pts with blepharitis have dry eyes due to lipid layer instability

Rx
tears, tarsorraphy, goggles, conjunctival flap
treat mucous with mucomyst 10% acetylcysteine (in reality, difficult to obtain, smelly to use)

punctal plugs
collagen plugs leak
silicones last indefinitely
cautery
local anesthetic
insert into canaliculus
apply while pulling back and reapply at opening
Argon laser
400 mW, paint puncta with skin marker
200 uM spot in ring 500 uM in center

Exposure
Eyelid Burns

Immediate
often with swelling (<3rd degree)
when resolves, lubricate q1/2-1 hr
moisten skin with antibiotic drops/frequent saline soaks
Intermediate
healing takes place 3-28 days later with corneal exposure developing rapidly
surgical rx if significant exposure (chronic injection, flourescein staining, dulling of light reflex)

Erythema multiforme
F>M, all ages but usually young
symptoms: malaise, fever, arthralgias precede skin lesions
recurrent vesiculo bullous skin lesions on extremities sparing trunk, look for target lesion
angiitis in dermis and conjunctiva
minor lasting only 2-3 wks, involves 1 mucus membrane
major (Steven's Johnson) M>F 6wks, skin and mucosa (2 or more mucus membranes)
involved, usually self limited
TEN if extensive denudation, mild purulent conjunctivitis, corneal erosions, and loss of lashes
the eyes are rarely involved with recurrence

associated with bacteria (Mycoplasma), viral (HSV) infections, drugs (sulfonamides, penicillin,
aspirin, dilantin, barbiturates), allergy, connective tissue disorders, vaccines
reported after topical sulfonamides, scopolamine, tropicamide, proparacaine, oral diamox
rx
topical steroids, glass rods ?, lubrication, wet dressings, surgery for trichiasis and dry eyes

OCP
F>M, age >60
vesiculobullous skin disorder usually extremities, inguinal without scar, but localized form on
head with atrophic scars
symptoms: chronic irritation, burning, tearing
ocular and mucous membrane involvement
active disease with small gray conjunctival mound, conjunctival hyperemia/edema
bilateral dry eyes with epidermilization, progressive subconj scarring, thin flaccid conjunctival
bullae, loss of goblet cells
decreased motility, trichiasis

dx: eosinophils in scraping, immunoglobulin deposition in basement membrane, increased

pathogens in lids
drugs associated with OCP are idoxuridine, phospholine iodide, pilocarpine, epinephrine
HLA DR4/DQW3, incidence 1:20,000

Rx
artificial tears
steroids (topical and systemic)
Cytoxan in severe cases
antibiotics for secondary infections
prednisone
nicotinamide
Dapsone
for mild involvement
check for G6PD deficiency prior to therapy
25mg bid with gradual increase, stop at 150 mg/day
check LFT, CBC, BUN/Cr and Met HgB
weekly CBC until effective dose is acheived and then spaced further apart
can develop neutropenia and bone marrow suppression weeks after starting therapy which is
reversible and may be dose related
agranulocytosis has never been reported for treatment of OCP

Pemphigus vulgaris
acantholysis of intraepithelial vesicles, flaccid easily broken blisters, middle age
no scarring, conjunctiva rarely involved
catarrhal or purulent conjunctivitis, rarely trichiasis

Bullous Phemphigoid
benign, >60 y.o., tense tough, subepidermal bullae
may cause shrinking, but rare

Epidermolysis bullosa
rare hereditary (AD or AR) disease of skin and other structures in which blisters form after
mild trauma
ocular problems- conjunctival vesicles and chronic blepharoconjunctivitis, symblepharons
junctional have recurrent corneal erosions

Recurrent Erosions
painful erosions usually in early morning upon awakening
pain similar to prior episode
often associated with prior trauma from fingernail, paper
rule out underlying corneal dystrophies
Rx
pressure patch initially
consider debridement
Muro 128 drops or lubricant drops qhs
extended wear soft contact lens can help up to 10% of patients
debride epithelium for MDF dystrophy
excimer PTK

Metabolic disorders

Alkaptonuria (ochronosis)
AR metabolic defect of tyrosine metabolism
no homogentisic oxidase, so homogentisic acid accumulates and forms brown-black material
(urine turns brown)
this material causes pigmentation of eyes, ears, nose, dura, sclerotic heart valves
brown dots near limbus at Bowman's layer, triangular patches pointing to medial rectus and
lateral rectus, pigmented piguecula, and coloring of tarsal plates/lids
accumulation causes arthritis, renal calculi

Cystinosis
AR, fine polychromatic needle like crystals under epithelium that migrate deeper (more
peripheral than central) so by 7 y.o. involves full thickness cornea, in conjunctiva
decreased corneal sensation, glistening dots on iris
photophobia may be incapacitating, can have blepharospasm
dx with conjunctival biopsy, crystals are water soluble so ask for frozen sections
infantile
dwarfism and progressive renal failure
previously fatal from chronic renal failure but now have kidney transplants
salt/pepper retina
adolescent
with less nephropathy, no retinopathy
adult form
normal kidney, benign and usually dx with slit lamp
transport enzyme out of lysosome is missing
rx
oral and topical cysteamine forms mixed disulfide which transports cystine out
difficulty is in early diagnosis

Fabry's
X-linked recessive
female carriers have corneal lesions
accumulation of ceramide trihexoside
pinhead hyperkeratotic vascular eruptions on breasts, buttocks, and extremeties, parasthesias
hands/feet with hot weather/exercise, ASHD, CRF with lipid buildup
corneal verticillata, periobital edema 1/4, PSC cataract 1/2, conjunctival aneurysms 1/2
associated with optic nerve edema, optic nerve atrophy, CME
diff dx

drugs: (chloropromazine, chloroquine, indomethicin, amiodorone)

striate melanokeratosis

Familial LCAT deficiency

AR, lecithin:cholesterol Acyl Transferase deficiency
small gray stromal dots in early childhood, peripheral arcus
no ocular therapy

Gauchers
AR sphingolipidosis, excess glucocerebroside
Type I: adult onset nonneuropathic, most common, anemia and thrombocytopenia
Type II: infant, retroflexion
Type III: adolescent, milder neuro
Ocular-pinguecula like lesion with Gaucher cells, can biopsy

Hyperlipoproteinemia
5 types based on class of lipoprotein
xanthelasma/arcus (most with type II)

Mucolipidoses
inherited defects of carbohydrate and lipid metabolism
varying degrees of corneal clouding which can be progressive

Mucopoysaccharidosis (MPS)
inherited disorder of carbohydrate metabolism (7 types)
all AR except Hunters (XLR)
Hunter's with gargoyle faces, heart/ liver/ skeletal/ mental retardation/ deafness
characterized by corneal clouding (entire cornea), retinopathy, and optic nerve atrophy
exception- cornea not involved in Hunter's and Sanfilippo
photophobia, papilledema due to hydrocephalus
gargoyle cells are large storage vacoles in histiocytes
dx by conjunctival bx of upper bulbar conjunctiva without sedation

Tyrosinemia Type II
decreased tyro aminotranferase, or no parahydroxy phenylpyruvate hydroxylase
painful erosive/hyperkeratotic skin lesions (palms, soles, elbows) with mental retardation
pediatric bilateral painful pseudodendrite lesions with purulent debris as a result of crystal
rupture of cells
rule out HSV (in tyrosinemia, no pseudopods, do not stain well with fluoroscein or rose bengal,
stellate, and no response with antivirals)
NOTE eye lesions may be first
dx: tyrosine load test, serum levels
restriction of tyrosine and phenylalanine in diet may prevent mental retardation

Wilson's disease
AR, chromosome 13q,
decrease in production of ceruloplasmin by the liver, copper deposition
Kaiser Fleischer ring is golden, red, or green,1-3 mm wide, at Descemet's, and starts
superiorly
rare sunflower cataract
check copper level and ceruloplasmin
ddx:
primary biliary cirrhosis, progressive intrahepatic cholestasis of childhood, chronic active
hepatitis
multiple myeloma

Rx:
penicillamine

Vitamin A deficiency
night blindness, keratoconjunctivitis sicca
pts have decreased mucus, GI, GU, and pulmonary problems
limbal Bitot's spots, corneal ulcer, keratomalacia, decreased corneal luster
may result from malabsorption syndrome: cystic fibrosis, pancreatic disease
Rx: acute vit A deficiency is a medical emergency with untreated mortality 50%

Iris Degenerations

Senile degeration
senile miosis with rigidity
may notice increasing blue color
moth eaten pupil ruff

Iridoschisis
age related, trauma, angle closure, and miotics
bilateral over 65 year old, M=F, not familial
often with shredded wheat appearance

Ischemic
sickle cell, quinine, angle closure, vasculitis from HZO, HSV

Neurogenic
neurosyphillis, lesions of ciliary ganglion

V. Tumors
NonPigmented

Papillomas
viral
younger, pedunculated, bilateral, multiple
fornix, palpebral, caruncle, toxic keratitis
RX observe, cryo, beta radiation
incomplete or partial excision can multiply them
neoplastic
older, unilateral, solitary, bulbar/perilimbal
sessile, severe conjunctivitis
Rx excise with speciman

CIN (intraepithelial neoplasia)

CIN refers to conjunctival/corneal intraepithelial neoplasia
spectrum of degree of malignancy ranges from
1. benign
2. premalignant/dysplastic
3. carcinoma-in-situ
4. frankly malignant
CIN not applied if extension beyond epithelium occurs
unilateral, 95% males, light exposed areas, papilloma virus
gelatinous thickening of epithelium
with leukoplakia, abnormal vascularization, or papilliform lesions (use Rose bengal)
histo-epithelium disarray, disturbed maturation
begins at the limbus
Rx: excise and cryo (double freeze thaw), topical mitomycin, scrape Bowman's, recurrence up
to 50%

Lymphoma
diff dx: benign hyperplasia, dermoid, orbital fat, lacrimal gland
can be difficult to distinguish from benign lymphoid hyperplasia
no symptoms
salmon color lesion, few vessels, flat smooth and soft, fornix
most conjunctival lesions are localized without systemic spread
prognosis same for unilateral or bilateral (stage I-E), 10% eventually develop systemic later as
non Hodgkins and large B-cell types
small cells better prognosis
monoclonal B lymphocyte subset suggests malignant potential
systemic lymphoma in 2/3 of lid, 1/3 of orbital

Benign heriditary intraepithelial dysplasia

AD, North Carolina ancestry, corneal neovascularization, corneal plaques
bilateral gray horseshoe plaques in bulbar conjunctiva near limbus
often vascularized, buccal leukoplakia, recur after excision

Mucoepidermoid
variant of squamous carcinoma but more aggressive, >60
basal cells produce mucus-secreting cells

Oncocytoma
apocrine usually benign tumor of caruncle
elderly females

Misc
inclusion cysts

pyogenic granuloma- raised, beefy red pedunculated tumor

associated with purulent/mucopurulent exudate
not a granulomatous inflammatory reaction
Rx: topical or intralesional steroids may help

Dermoid- choristoma (normal tissue in abnormal location)

usually located inferotemporal limbus

Epibulbar Osseus Choristoma

rhabdomysarcoma (embryonal)

Pigmented

racial melanosis
normal, bilateral, fades toward fornices, in 95% of blacks, 5% of whites

congenital melanosis oculi (ocular melanocytosis)

unilateral uveal, scleral, episcleral pigment, W>B
Nevus of Ota with lid involved, B>W
suspected increased uveal melanoma especially in caucasians
no increased in conjunctival melanoma
malignant transformation with nevus of Ota is possible but rare, occurs in caucasians
nevus:

bulbar conjunctival
iris

can grow
moveable without extension onto cornea
often discrete light tan
often cystic and can be very pigmented
bx if on palpebral or fornix conjunctiva

path:
rare to have junctional nevus of conjunctiva over 25 year old, (probably PAM)
usually compound or subepithelial nevi
no growth <3mm width <1mm height
<3mm width <1mm height
minimal vessels no glaucoma
may have sector cataract
no glaucoma
can have ectropion irides
photograph and follow for growth can have ectropion iridis

PAM (primary acquired melanosis)

flat, golden brown to chocolate lesions
mobile, indistinct margins
unilateral, middle age white patients
grows, does not fade toward fornix, no cysts
PAM without atypia suggest low malignant potential
with atypia has >50% chance of malignancy with subdivision in low and high risk lesions
multiple small biopsies, excise smaller lesions, cryo more diffuse lesions, especially with
nodularity

melanoma
conjunctival melanoma
bulbar, vessels, nodular, mobile, NO CYSTS
most arise from nevi and PAM, but de novo as well
prognosis worse if > 0.8mm, pagetoid PAM, melanoma in situ form of PAM, or if lid, caruncle,
or fornix is involved
25% mortality
local excision with cryo
metastasis first to regional lymph nodes
iris melanoma
differential diagnosis includes:
ICE foreign body
primary cyst nevus
PAS metastatic disease
atrophy pigment epithelial migration

no surgery to prevent metastasis

key is to r/o ciliary body melanoma with transillumination, scleral depression, U/S
iridic cyts should be transilluminated, gonioscopy and U/S

Kaposi's sarcoma
elevated patches of hemorrhage that do not resolve
arise from lymphatics so none in orbit or choroid