Cornea and External Disease

Robert Cykiert, M.D.

Revised 2003

I. Basics

papilla
vascular response
if giant, the differential includes atopy, vernal, GPC, prosthesis, suture

follicles
lymphatic response

acute chronic
EKC, pharygoconjunctival fever adult inclusion conjunctivitis
medicamentosa (epinephrine, neosynephrine) toxic
Parinaud's oculoglandular,syndrome r/o sarcoid
HSV primary conjunctivitis r/o GPC, vernal conjunctivitis
Newcastle's conjunctivitis
with acute follicles, always check lid margin for HSV vesicles, ulcers

membranes
conjunctivitis ocular cicatricial pemphigoid erythema multiforme
Stevens Johnson syndrome Srogrens syndrome atopy
Symblepharon scieroderrna burns
radiation burns trachoma EKC
sarcoid drugs

filaments
exposure (keratoconjunctivitis sicca, neurotrophic, patching
recurrent erosion)
bullous keratopathy HSV
meds superior limbic keratoconjunctivitis psoriasis
aerosol keratitis
diabetes mellitus radiation
retained FB Thygeson's SPK
ptosis

Enlarged Corneal nerves

MEN TIIb icthyosis Hanson's
Kconus 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 NFI
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
Flouresecin staining when disruption of cell-cell junctions
Rose Bengal stains with disruption of precorneal tear film
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
retinopatby
problems with epistaxis and GI bleeding

Congenital lymphedema
XLR, AD, usually massive edema of legs
dysplasia of lymphatics

Medicamentosa

Anaphylactic
sulfonamides, bacitracin, anesthetic

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

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

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

EKC adenovirus 8,9,13; can cause symblepharon

EBV virus with mono can cause conjunctivitis, keratitis
Newcastle disease virus with poultry exposure
measles may have papillary corjunctivitis withwhite avascular spots ci
caruncle/conjunctiva like Koplik spots in mouth

Parinaud's OGS
cat scratch
children with cats, hx of scratch, sneeze, 2 wk latency, nodule in
superior or inferior conjunctiva
intense chemosis, injection, lymph nodes may appear up to 2 wks
later
systemic fever, malaise, maculopapultar rash
DX:
Hanger Rose test 90% sensitivity skin test
Warthin Starry stain for bacilli
RX:
Doxycycline 100mg bid x 1 month

tularemia
lymph nodes, fever, chills, vomiting, pneumonia but ocular involved
<5%
necrotizing, ulcerating conjunctivitis, corneal ulcer, optic neuritis,
dacryocystitis, panophthalmitis
rabbit hunters, hx of tick bites with punched out lesion
DX:
with agglutination titers 1: 160 or higher in 2 weeks and peak
in 4-8 wks
RX:
streptomycin, tetracycline
sporotrichosis
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 HI pts
no systemic illness
DX:
culture on Sabouraud's
RX:
KI 1 ml/day
Misc

sarcoid leptothrix chancroid

 glanders Crohn's
fungi lues TB

lymphgranuloma veneream
culture conjunctiva and scrape, blood clx if febrile, VDRL, FTA,
PPD, viral titers, biopsy

Reiters
bilateral conjunctivitis, iridocyclitis, urethritis, polyarthritis
fever, lymph nodes, pericarditis, pneumonitis, myocarditis
think if chronic nonfollicular mucopurlent conjunctivitis
SPK, corneal infiltrate, corneal neovascularization
steroids, chlamydia/dysentery antibx?

Floppy Lid

SLK
burning, no itch or discharge, symp 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:
scrape conjunctiva, pressure patch, soft contact lens, resect conjunctiva

Thygeson's keratitis
can often mimic SEI
recurrent hx 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
dramatically responds to topical steroids often after 2 doses, taper in one wk
some need chronic therapy due to rebound if steroids stopped

molluscum
SPK, pannus, follicles, pseudotrachoma

Graft vs Host
S1 conjunctiva hyperemia
S2 chemosis, exudate
S3 pseudomembranous
S4 corneal epithelial slough
higher stage correlates with increased severity of disease and mortality
keratoconjunctivitis sicca most commonly, cicatticial lag, ectropion, persistent
epithelial defects, iritis
Chlamydia

Trachoma
superior pannus, SPK, corneal infiltrates,
lid destruction and exposure are key elements
tetracycline, erythromycin, or sulfonamides x 3 months
S1 conjunctival follicles, cytoplasmic inclusion bodies
S2 inflammation, increased corneal pannus
S3 scaning flerbervs pits (lirnbal depressed necrotic follicles), Arlt's line
S4 end stage

Adult TRIC
5% with urethritis, 1-2 wk latency, meibomianitis, lid edema and redness
follicular conjunctivitis, EKC-like SEI
NO membranes
superior pannus (not seen in EKC)
doxycyline 100mg bid 10 days or erythromycin 250mg qid 3 wks, treat partner

Newborn TRIC
no follicles, more discharge, + pseudomembrane
4-12 days post partum, r/o GC
Giemsa incl bodies 40%, 90% with + clamydiazime
otitis, pneumonitis in 15%, recurrence 20%
Erythromycin syrup 50mg/kg qid x 2 wks, treat mom

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

Atopy

RX
allergy testing and environmental control
compresses, pressure patch, air conditioning, pulse steroids
vasoconstrictors, Acular, livostin, mast cell stabilizers
immunosuppressives

Hayfever
rapid, lid swelling, chemosis (pale palpebral conjunctiva), itching, mucus,
dellen
pressure, rhinitis/asthma, episodic, may have no signs
T1 hypersensitivity, elevated tear IgE, eosinophils in scraping in chronic
cases
Vernal (VKC)
bilateral seasonal young (3-25 year old) in warmer climates, M>F
FHx of atopic allergies
self limited average 4- 1 0 years
ITCH (worsens in evening, dust, lights, wind, rubbing), clear tears
ropy discharge but lids don't get crusted or stick together unless bacterial
superinfection
GPC (may see a membrane form at the slit lamp),
limbal involvement more in blacks (can be 360 degrees)
Homer Trantas dots (clumps of degenerated eosinophils), clear elevated cysts
SPK, flour dust 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
topical cyclosporine 2% qid can be used as alternative to steroids

Atopic (AKC)
M>F, teens to 40's, burns out by 40-50 year old, small papilla, milky edema,
corneal neovascularization
fix of atopic eczema (3% of pop), similar to venial findings but no seasonal
changes
symblepharon, foreshortening of inferior fornix, usually lower palpebral
conjunctiva affected
in severe cases bilateral cataracts (anterior subcapsular, or posterior polar)
10% of all 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)

increased serum IgE, few eosinophils in scraping and rarely free granules

systemic antihistamines, nasal cromolyn, topical mast cell stabilizers,

steroids and cream, doxycycline
path
increased T helper, macrophages, increased class If HLA similar to
OCP and rosacea.
more complex than simple mast cell allergic rx
No BM deposition

GPCofCL
usually develops within first year of lens wear, but RGP can develop after
years
also seen in art eyes, sutuIres
r/o VKC (no tear histamine, no free eosinophil granules, only 1/4 have
eosinophils in scrapings
 early mucous discharge, mild itch with increased in severity, then pain with
CL, blurred Va
papilla may stain (sign of activity), whitish material on lens
conjunctival injection or chemosis when severe, thick shem of mucus, soft <
hard CL
switch brands/ types of lenses, increased use of enzyme, topical steroids,
mast cell stabilizers, stop lenses

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

Blepharitis
all get hot compresses and lid scrubs bid
can try antibiotic ungt qhs to qid, if severe may use blephamide
chronic doxycycline antibx associated with vaginitis, allergy,
photosensitivity, take on empty stomach, no breastfeeding

Staph
collarettes, ulcers at base of lashes, papilla, purulent discharge, marginal
ulcers
absent, thin, broken, misdirected, or white lashes
younger, F (80%), short duration, dry eyes

Seborrheic
older, more chronic, oily margins, crusting, papilla, follicles, dermatitis, dry
eyes

Meibomian Gland Dysfunction (MGD)

acne rosacea, conjunctival injection, SPK, Rose Bengal stain, more burning,
bulbar injection
Doxycyline 100mg bid for a month
maybe even chronically bid/qd

Rosacea
F, 30-50, rhinophyma, telangiccwia, pustules, crythema
MGD, marginal keratitis, inferior corneal pannus with subepitlielial
infiltrates

Subconjunctival hemorrhage
Anemia ASHD conjunc;tivitis decreased platlets
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
Grade I, II patchy and flat and <4 mon duration, Gr III > 3mm thick
on bulbar conjunctiva or eyelid
standard rx with radiation
consider double freeze thaw cryo for Gr I, II on eyelids
simple excision of conjunctiva for bulbar conjunctiva leaving bare sclera
excision s/p vessel diueation by flourescein angiograrn for Gr III on
conjunctiva

Ill. Cornea
Congenital Anomalies

megalocornea
>13mm, bilateral, most commonly XLR, usually isolated, nonprog
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
<10mm, hyperopes, AD>AR
20% with angle closure glaucoma, usually eye nl
r/o nanophthalmos, microphthalmos, trisomy 13, Ehlors Danlos, dwarfism

Anterior seg dysgenesis AD

Posterior embryotoxin
Axenfelds
hyperteloric, small shoulders, facial asymmetry, 1/2 with glaucoma
(syndrome)
Rieger's
craniofacial anomalies, mental retardation, Marfans, 1/2 with
glaucoma
syndrome with maxillary hypoplasia, telecanthus, microdontia,
skeletal and spine deformities
Peter’s
80% bilateral, clearing of leukoma with waiting, glaucoma
Tl- nl lens and no systemic involvement
T2- lens involved, bilateral, often glaucoma
r/o von Hippel's internal corneal ulcer (no lens abnormal)
 local posterior keratoconus (endothelium/descemet present)
Haab's striae
Congenital opacities
facets, nebula, macula, leukoma
corneal keloid probably from intrauterine trauma
sclerocornea
bilateral, often with systemic and other ocular problems

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
most common cause of central infectious corneal ulcer
sunlight, fever, stress, memses, steroids
1 yr 25%, 2 yrs 50% chance of recur
lids (psuedozoster)
rare follicular conjunctivitis
epithelial keratitis
SPK, dendritic, geographic, marginal
shaggy borders, ghost scars of prior dendrites, decreased
corneal sense
often with mild stromat edema
1/4 recur in 1 yr, 1/2 recur in 2 yrs
metaherpetic lesion from poorly healing epithelium, gray
thickened heaped up edges
RX:
debride, viroptic 9x/day taper off 2 wks
see in weekly, don't overtreat
limbal lesions resist antivirals, slower healing

diff dx of dendrite
CL filaments
HZO Mygeson's
tyrosinemia TII healing corneal abrasion

disciform
immune rx at endothelium
central corneal edema with fuie KPs under edema, mild
iridocyclitis, increased IOP, Wessley ring
 diff dx HZO, local bullous keratopathy
self limited 2-6 months with variable scar
cycloplegic, may use steroids, Muro 128, antiviral cover, later
PKP

peripheral stromal keratitis

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

necrotizing
live virus but even - on bx
dense deep stromal infiltrate
no pain, mild iridocyclitis, increased IOP, corneal
neovascularization, indolent
self limited 2-12 months
antivirals with steroids and slow taper (even yrs)

endothelitis
progressive corneal edema with line of KP (looks like PKP
rejection), uveitis, increased IOP
acyclovir

uveitis
iritis with diffuse iris atrophy
multifocal choroiditis

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, episcieritis
SPK, microdendrites, corneal neovascularization, uveitis, glaucoma,
sectoral iris atrophy
stromal nummular keratitis, scleritis (nod>diff, limbal can spread to
cornea)
1% with optic neuritis, Homees, EOM palsy (25%)
decreased corneal sensitivity leads to nearotrophic ulcers
 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- 1 00 mg po x 3 days
pain medication
r/o HIV especially if < 45 year old or risk group
if microdissemination, pt may be im. immunecompromised
consider IV steroids, use IV acyclovir

HZO Neurological syndromes

postherpetic neuralgia
pain decreased with time (80% gone in I yr), worse if pt is
older
Rx capsiacin, stellate ganglion block, cimetidine,
carbamazepine, TCA
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/SJ, 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
Pseudomonas often with hypopyon, diffuse inflammation, epithelial edema
Staph often with distinct borders, may satellite
consider calcium alginate swab soaked in trypticase soy broth
blood, chocolate, tliio, sabourouds for fungus

Phlyetenular ulcer
pinkish white limbal elevation with gray crater, can be on conjunctiva
children 10-20, allergic rx to staph, TB, Candida, Chlamydia,
nematodes
early Teri4xen's can look similar
Marginal ulcer
gray limbal ulcer usually with clear cornea all around, early corneal
neovascularization
allergic rx usually to staph at 8/10 and 2/4 oclock
can progress to ring ulcer, usually spread toward lhbus not centrally
can get superinfected
atopy Wegenees periarteritis nodosa
dysentery Crohn's/ UC influenza
GC arthritis dengue fever hookworm
gold poisoning
porphyria brucellosis acute leukemia
rheumatoid arthritis SLE scleroderma

Acanthameoba
mimics HSV early, later ring infiltrate, often misdiagnosed as HSV
symptoms wax and wane with photophobia, FBS, severe pain
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 cuffmg of nerves
risk factor contamin CL, corneal trauma, r/o topical anesthetic abuse
bx , stains faster and better than culture
touch material instead of smearing on slides-
use spray fixative instead of air drying
IFA, calcofluor white, conjugated lec-tin fluorescein stain
culture on non-nutrient agar with E. Coli overlay

RX with neosporin, Brolene 0.1%, neomycin, and clotrimazole 1% ql5-qlh, +/-

ketoconazole 200 mg po bid, no steroids
once infection in peripheral cornea, poor candidate for graft

Fungal
trauma, south, mostly filamental, Fusarium, Aspergillus >50 %
Candida 10% in older, keratoconjunctivitis sicca, looks Re bacterial
feathery edges, endothelium plaque, satellite lesion
can penetrate Descemet’s, culture Sabourauds and blood agar
RX:
first line natamycin 5% susp (50mg/ml) q1hr,
Ampho 0. 15% (1.5 mg/ml) especially for candida
flucytosine for yeasts

interstitial keratitis
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

TB leprosy HSV
Mumps LGV sarcoid
Kaposi's sarcoma Hodgkins Incontinentia pigmenti
mycosis fungoides HZO protozoan/heiminths

Cogan's Syndrome
tinnitus, periarteritis nodosa, 30-40, decreased hearing, syst vasculitis
remember congenital lues also with decreased hearing
- serology

Infectious CrystaUine keratopathy

no epithelial defect, feathery "crystalline" edaes, associated with chronic
steroids eg PKP
poor response to topical therapy, bx usually for dx
Alpha strep viridens, rx with conc topical bacitmcin

Degenerations

Pinguecula

Pterygia
risk factor UV exp especially < 5 year old, no glasses, no ha@ equatorial living
excision, radiation, conjunctival transplant or flap, mitomycin
surgery for astigmatism, VF changes, cosmesis, restricted EOMS, inflammation

Amyloidosis
subepithelial, salmon color, avascular
primary and mycloma tend toward mesenchyrnal deposit
secondary to organ deposit
metachromasia crystal violet, flourescent thiaflavine T, biref@@gence/di@hroism
Congo Red, +with Siruis Red
primary localized
most common form with palpebral conjunctival asymmetry
(brown/yellow waxy firm subconj nodules)
lattice is special form
can make cornea into pudding
does NOT affect lids, but can be orbital presenting as VI n palsy
primary systemic
bilateral symmetric yellow or ecchymotic lid papules, light near
dissoc
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
in Bowman's
chronic disease, system increased Ca, Phos, mercury, hereditary
gout with urates, renal failure
uveitis, long standing glaucoma, interstitial keratitis, phthisis,
pilocarpine, dry eyes
rx with 3% EDTA, scrape and sponge until it clears
Spheroidal degeneration
bilateral M>F, golden brown spheres in anterior stroma/Bowmans in
palpebral zone
risk factor age, exposure, probably elostatic degeneration of colilgen
usually not elevated, small lesions, can be in conjunctiva, can have
decreased Va
Salzmann's nodular degencration
unilateral F>M middle age, non-inflamm end years later to old kerititis
usually by hx
gray white elevated subepithelial nodules at end of old pannus
paracentral cornea
asymmetry, tearing, photophobia, or decreased Va
rx with simple excision
Coat's white ring FB remnant
Lipid Keratopathy
unilateral or bilateral, 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
nl Va, products of cellular degeneration

Marginal thinning
Mooren's
idiopathic unilat painful inflamed eye often inferiorly
 trauma hx, rare perf, circumferential spread early
central edge undermined in stroma, blunt edge peripherally
two types, one benign, unilateral, in older, responds to surgery
the other, relentlessly progressive and bilateral in 25%, young
RX steroid (top, sys), 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
astigmatism in 20-40 and 60-70 year old M>F with steep central wall,
mild inflammation
later, lipid deposts at edge of pannus, epithelium intact, decreased Va
from astigmatism
rare perforation, rx with mild steroids chronically to suppress
inflammation
RX lamellar or PKP
Marzinal 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
>30 y.o. 10% have corneal abrasions
50% of pts with recurrent comeal abrasions have MDF
fingerprint best seen with retroillumination, maps with oblique,
usually negative floureseein staining
debridement with cotton swab
lubricants, soft contact lens, ? excimer
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
childhood, with irritation, small decreased Va
thick BM, intraepithelial microcysts with peculiar PAS+ substance
 no rx, retroilluminate, punctate staining
diff dx cystinosis
Reis-Blacker's
childhood, progressive gray white at Bowman's layer
reticulated pattern of scarring
painful recurrent erosions, by 50's marked corneal opacities
recur post PKP

Stromal
Marilyn Monroe Gets Hers in LA
Granular
onset in childhood with decreased Va later >201200
white deposits in anterior stroma
hyaline, + trichrome
periphery may be + for amyloid (ancestry to Avellino, Italy)
may recur yrs after PKP
Lattice (Amyloid)
childhood, central lines, dots, haze (ground glass)
recurrent corneal abrasion, decreased Va by 40 years old
TI AD, can be deep, spares limbus, retroillumination, starts as
dots
T2 AD, Meretoja- secondary amyloiddosis with progressive
cranial neuropathy and skin changes
T3 AR, thicker lines across entire cornea easily seen, later onset
T3a AD, frequent corneal erosions

recurance post PKP common

Macular
*AR, periphery involved*
decreased Va in childhood with dimse stromal clouding
GAGs, inability to breakdown KS
gray white opacities with indefinate margins
may be even full thickness, guttata
alcian blue, colloidal iron stain
can recur after PKP
Central crystallin (Schnyder)
infant, progressive polychromatic crystals in stroma and haze
arcus and limbal girdle
doughnut Re crystals are cholesterol and fats
Oil red 0 stain, frozen section
Va OK, associated genu valgum, hyperlipidemia
Fleck
nl Va, no symptoms
rare childhood with assymmetry
grey white dandruff to periphery, increased GAGS, lipids
 associated limbal dermoid, keratoconus, CCD, PXE, decreased
corneal sense
atopy, cortical lens changes
CCD (central cloudy dystrophy)
bilateral symm nonprog dense with clear zones
nebulous gray areas with nl vision
polymorphic stromal dystrophy
bilateral sym progressive late in life
nl Va, associated with systemic amyloidosis
PreDescemet farinata
>30 year old, 0-red-O+, Sudan black+, and PAS+,
deep stromal punctate above Descemet’s
nl Va
Posterior amorphous stromal dystrophy
rare bilateral child with good Va
extends to limbus, iris processes, endothelium disrupt focally
peripheral varient with clear center
CHSD
flaky feathery anterior central opacity at birth
can have decreased Va

Posterior
guttata
abnormal coliagen, orange peel look, if 2-3+, probably asympt Fuchs
Fuchs's dystrophy
guttata are focal retractile clumps of colilgen posterior to Descemet's
AD, F>M, post menopause, bilateral and assymetric, rare in Orientals
central guttata rust, pigment in endothelium (can have borders)
1 st 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, PPMI),
disciform keratitis
RX:
only if symptomatic, treat as in other causes of corneal edema
if considering phacoemulsification check corneal thickness
if corneal thickness is > 0.6 mm then do triple procedure
pachymetry is optional

PPMD, posterior polymorphous dystrophy

AD bilateral childhood, progressive, asymmetry
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
bilateral congenital corneal edema, Descemet’s thick
due to degen of endothelial cells at 5 months gestation
T1 AR, most common, no pain or tearing
stationary, diffuse,
TII AD, 1-2 year old, painful tearing, progressive
no nystagmus, cornea blue ground glass

Ectatic
keratoconus
sporadic bilateral asymmetric with early astigmatism, F, 10-30 year
old
associated with Down's, Marfan's, eye rubbing, vernal, HLA B27,
MVP, RP, PMMA CL
Vogt’s striae, Fleischer ring, scarring
can be familial (<10% of occur in blood relative)
keratoglobus
thinning greater in periphery, mild sear, not genetic, connective tissue
disorders
associated Ehlors-Danlos
Pellucid marginal degeneration
bilateral clear inferior thinning 2 mm from limbus, 2mm in width
no iron ring, no cone but cornea protrudes above thinned area, no
scar, striae
20-40 year old with high astigmatism

IV. Misc
dry eyes
80% show excess mucus, thinned tear film with debris, SPK, corneal mucus plaques
and filaments
Rose Bengal (>3/9 score), TBT unreliable meniscus height variable
bothered by wind, reading, smoke, steep, @; of skin diseases, tearing/ mucus
check conjunctival scaning, V, VII CN, avitaminosis A, lagopthalmos, sarcoid
medications implicated include antihistamines, HCTZ, antibx, Inderal, Valium,
Pyridium,
Timoptic (damages mucus layer, decreased goblet ceffi)
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
 if great improvement perform permanent punctal closure
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 ql/2-1 hr
moisten skin with antibx ungt/frequent saline soaks
Intermediate
healing takes place 3-28 days later with corneas 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, recurrent vesiculo bullous skin lesions on
extremities
sparing trunk, took for target lesion
angiitis in dermis and conjunctiva
minor lasting only 2-3 wks
major (Steven's Johnson) M>F 6wks, usually self limited with fever, URI, HA,
malaise
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), allergy, connective tissue disorders, vaccines
reported after topical sulfonamides, scopolamine, tropicamide, proparacaine
RX:
topical steroids, glass rods ?, lubrication, wet dressings, surgery for trichiasis
and dry eyes
OCP
F >60 with vesiculobullous skin disorder usually extremeties, inguinal without scar,
but localized form on head with atrophic scars
ocular and mucous membrane involvement
active disease with small gay conjunctival mound, conjunctival hyperemia/edema
bilateral dry eyes with epidermilization, progressive subconj scarring, thin flaccid
conjunctival bullae
 decreased motility, trichiasis,. eosinophils in scraping, Ig in BM, increased
pathogens in lids
drugs associated with (?induced) OCP are IDU, PI, pilo, T1/2, epinephrine
HLA DR4/DQW3, incidence 1:20,000
RX:
steroids (topical and systemic)
Cytoxan in severe cases
Dapsone
for mild involvement
check for G6PD prior to therapy
25 mg bid with gradual increase, stop at I5O mg/day
check LFT, CBC, and Met HgB
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
Epidennolysis bullosa
blisters after mild trauma
ocular problems with dystrophic varient with symblepharons
junctional have recurrent corneal erosions

Recurrent Erosions
painful erosions usually in early morning
pts often aware of something wrong before they open eyes
pain similar to prior episode
often with MDFor trauma from nail, paper
RX:
pressure patch, muro 128, soft contact lens, mild steroids (decreased Bowman's
inflammation)
debride epithelium for MDF
anterior stromal puncture is better in traumatic cases (see MDF)

Metabolic disorders
Alkaptonuria (oclironosis)
AR, no homogentisic oxidase, so homogentisic acid sent to kidneys and with
alkali urine turns brown
pigment of eyes, ears, nose, dura, arthritis, sclerotic heart valves, early
ASHD
brown dots near limbus at Bowman's layer, triangular patches pointing to
MR, LR, pigmented piguecula, and coloring of tarsal plates/lids
quinacrine and hydroquinone can case ochronosis
no ocular or medical rx
 Cystinosis
AR, fine polychromatic needle like crystals under epithelium that migrate
deeper (more peripheral than central) so by 7 y.o. full thickness cornea, in
conjunctiva
decreased corneal sensation, glistening dots on iris
photophobia may be incapacitating, can have blepharospasm
dx with conjunctival bx. crystals are water soluble so ask for frozen sections
infantile
previously fatal from CRF but now have kidney transplants,
salt/pepper retina
adolescent
with less nephropathy, no retinopathy
adult form
nl 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
XLR, but F carriers have corneal lesions, decreased ceramide trihexosidase
pinhead hyperkeratotic vascular eruptions on breast-, buttocks, and extrem,
parathesias hands/feet with hot weather/exercise, ASHD, CRF with lipid
buildup
corneal verticillata, periob edema 1/4, PSC cataract 1/2, conjunctival
aneurysms ½ associated with ON edema, ON atrophy, CME
diff dx

chloroquine indomethicin amiodorone

chloropromazine striate melanokeratosis

Familial LCAT deficiency

lecithin:cholesterol Acyl Transferase deficiency, AR
small gray stromal dots in early childhood, peripheral arcus
no ocular therapy
Gauchers
AR sphingolipidosis, excess glucocerebroside
TI-adult onset nonneuropathic, most common, anemia and thrombocytopenia
TII-infant, retroflexion, TIII-adolescent milder neuro
Ocular-pinguecula like lesion with Gaucher cells, can bx
Hyperlipoproteinemia
5 types based on class of lipoprotein
xanthelasma/arcus (most with type II)
Mucolipids
cornea cloudy in GM I type 1, T1, Tlll
 Mucopoysaccharidosis (MPS)
entire cornea cloudy, colloidal iron, 7 types
all AR except Hunters (XLR) with gargoyle faces, heart/ liver/ skeletal/
mental retardation/ deafness
RPE changes, ON atrophy, 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
palm/sole painful erosive/hyperkeratotic skin lesions with mental retardation
pediatric bilateral painful dendriticlike lesions with purulent debris as a result
of crystal rupture of cells
ddx of HSV (but no pseudopods, min staining, stellate, and no response with
antivirals)
NOTE eye lesions may be first
tyrosine load test, serum levels, diet may prevent mental retardation
decreased tyro aminotranferase, or no parahydroxy phenylpyruvate
hydroxylase
Wilson
AR, chromosome 13q,
Kaiser Fleischer ring is golden, red, or green, 1-3 mmn wide, at Descemet’s,
and starts superiorly
rare sunflower cataract
check copper level and ceruplasmin
DDX:
primary biliary cirrhosis, progressive intrahepatic cholestasis of
childhood, chronic active hepatitis
multiple myeloma
RX:
penicillamine
avitaminosis A
night blindness, keratoconjunctivitis sicca, limbal Bitot’s spots
pts have decreased mucus, GI, GU, and pulmonary problems
corneal ulcer, keratomalacia, decreased corneal luster
malabsorption syndrome: cystic fibrosis, pancreatic disease

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
sicke cell, quinine, angle closure, vasculitis ftom HZO, HSV
 Neurogenic
neurosyphillis, lesions of ciliary ganglion

V. Tumors
NonPigmented
Papillomas
viral
younger, pedunculated, bilateral, multiple
fornix, palpebral, caruncle, toxic keratlitis
RX observe, cryo, beta radiation
incomplete or partial excision can multiply them
neoplastic
older, unilateral, single, bulbar/perilimbal
sessile, severe conjunctivitis
Rx excise with speciman
CIN (intraepithelial neoplasia)
unilateral, 95% males, light exposed areas, papilloma virus
gelatinous, thickening with leukoplakia, or papilliform (use Rose bengal)
histo-epithelium disarray, disturbed maturation, dysplasia to carcinoma in
situ
limbal, excise and cryo (double freeze thaw), scrape Bowman's, recurrence
up to 50%
Lymphoma
diff dx benign hyperplasia, dermoid, orbital fat lacrimal gland
salmon color, few vessels, no symptoms, flat smooth and soft, fornix
most conjunctival 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
systemic lymphoma in 2/3 of lid, 1/3 of orbital
Benign hereditary intraepithelial dysplasia
AD, North Carolina ancestry, corneal neovascularization, corneal plaques
bilateral gray horseshoe plaques in bulbar conjunctiva near Iambus
often vascularized, buccal leukoplakia, recur after excision
Mucoepidermoid
like SCC but more aggressive, >60
Oncocytoma
apocrine usually benign tumor of caruncle, elderly F
Misc
inclusion cysts
pyogenic granuloma
Dermoid
Epibulbar Osseus Choristoma
rhabdomysarcoma (embryonal)
Pigmented
racial melanosis
nl, bilateral, fades toward fornices, in 95% of blacks, 5% of whites
congenital melanosis oculi
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
nevus
bulbar conjunctival
can grow, often discrete light tan
moveable without extension onto cornea
bx if on palpebral or fornix conjunctiva
often cystic and can be very pigmented
path
rare to have junctional nevus of conjunctiva over 25 year old,
probably PAM
usually compound or subepithelial nevi
iris
no growth, <3mm width <1mm height minimal vessels, no
glaucoma or cataract
PAM
flat, golden brown to chocolate, mobile, indistinct margins
unilat, middle age, 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 bx, excise smaller lesions, cryo more diffuse lesions,
especially with modularity
melanoma
conjunctiva
bulbar, vessels, nodular, mobile, NO CYSTS
most from nevi, 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
no surgery or trab due to increased 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