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Eye Resident Handbook - Cornea

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Eye Resident Handbook

Cornea
I. Basics | II. Lids/conjunctiva | III. Cornea | IV. Misc | V. Tumors DISCLAIMER--PLEASE READ

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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. The authors are not liable for any action taken on the basis of this manual. Cornea and External Disease Revised by Diane Song MD, Meagan Celmer MD Editor Balaji Gupta MD

All Pages home Basic Science Cornea Glaucoma Neuro Ocular Pharmacology Pediatrics Plastics Retina RETINA PAGE 2 Uveitis

Table of Contents
I. Basics II. Lids/conjunctiva Congenital Epitarsus Osler Weber Rendu Congenital lymphedema EKC adenovirusEBV virus Newcastle disease Measles Catscratch Tularemia Sporotrichosis Reiters Floppy Lid SLK

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

membrane true membranes suffuse epithelium, bleeds when stripped from the conjuntiva pseudomembrane when removed, does not cause bleeding

Thygeson's keratitis Molluscum Adult chlamydial conjunctivitis Neonatal chlamydial conjunctivitis Allergy Seasonal Allergic Conjunctivitis

filaments:

Vernal (VKC) Atopic (AKC) general guidelines for treatment of allergic conjunctivitis GPC of CL Contact dermatitis Blepharitis Subconjunctival hemorrhage Superglue in eye

Enlarged corneal nerves

Kaposi's sarcoma III. Cornea Congenital Anomalies Megalocornea microcornea

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 corneal edema whenever epithelium disrupted, can stimulate iritis via reflex arc

Anterior segment dysgenesis Axenfeld anomaly-Reiger syndrome: Axenfelds Rieger's Peters Haab's striae Congenital opacities Sclerocornea

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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 epithlial cells and mucus) cell death increases permeability to these dyes, but Rose Bengal can still be blocked with tears

Viral HSV HZO Bacterial keratitis Phlyctenular ulcer Staph Marginal Infiltrates Infectious Crystalline keratopathy Acanthameoba Fungal Interstitial keratitis Syphillis Cogan's syndrome Degenerations Pinguecula Pterygia Amyloidosis Involutional Deposits Marginal thinning Dystrophies Anterior MDF Meesman's Reis-Bucker's Stromal Macular Granular Lattice (Amyloid) Central crystalline (Schnyder) Fleck CCD (central cloudy dystrophy) Polymorphic stromal dystrophy Pre-Descemet farinata Posterior amorphous stromal dystrophy CHSD (congenital hereditary stromal dystrophy) Posterior Corneal guttata Fuchs's dystrophy PPMD, posterior polymorphous dystrophy CHED (congenital hereditary endothelial dystrophy) Ectatic Keratoconus Ehler Danlos VI Keratoglobus Pellucid marginal degeneration IV. Misc Dry eyes Eyelid Burns Erythema multiforme OCP Pemphigus vulgaris Bullous Phemphigoid Epidermolysis bullosa Recurrent Erosions Metabolic disorders Alkaptonuria (ochronosis) Cystinosis Fabry's Familial LCAT deficiency

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

Conjunctivitis

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EKC adenoviruspeak intensity days 5-7 stage ranges from superficial epithelial keratitis to subepithelial infiltrates (SEI) subepithelia 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

Tyrosinemia Type II Wilson's disease Vitamin A deficiency Iris Degenerations V. Tumors Non-Pigmented Papillomas CIN (intraepithelial neoplasia) Lymphoma Benign heriditary intraepithelial dysplasia Mucoepidermoid Oncocytoma Misc Pigmented racial melanosis congenital melanosis oculi (ocular melanocytosis) nevus PAM (primary acquired melanosis) melanoma Kaposi's sarcoma

EBV virus
belongs to herpesvirus 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

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 mucopurlent 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

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?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 currette, 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, seropyes 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)

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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 partam, r/o GC Giemsa inclusions bodies 40%, 90% with + clamydiazime otitis, pneumonitis in 15%, recurrance 20% Erythromycin syrup 50mg/kg qid x 2 wks, treat mom

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

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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) Symptoms 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

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Blepharitis

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 tetrycycline 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:

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 mon duration, Gr 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 Gr I, II on eyelids simple excision of conjunctiva for bulbar conjunctiva leaving bare sclera excision s/p vessel delineation by flourescein angiogram for Gr III on conjunctiva chemotherapy

III. Cornea
Congenital Anomalies
Megalocornea
corneal diameter >13mm, bilateral, most commonly X-linked rec., (therefore 90% are male) 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

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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, Marfans, 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
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

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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, HZO, filaments, 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) endothelitis 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
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)

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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 immunecompromised 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: capsiacin, 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 factors: 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

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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 oclock can progress to ring ulcer, usually spread toward limbus not centrally can get superinfected

Infectious Crystalline keratopathy


poor response to topical therapy

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alpha strep viridens, rx with concentrated topical bacitracin

Acanthameoba
found in water, existing as a cyst or trophozoite risk factors: contamin 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 calcofluo 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 lexions that leads to endocarditis and gastrointestinal hemorrhages remember congenital lues is also associated with decreased hearing serology Other conditions associated with interstitial keratitis

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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 may be associated with chronic sun exposure

Pterygia
similar histology as pinguecula, but extends from the conjunctiva to involve peripheral cornea risk factor: UV exp 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 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) crocadile 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, sacoidosis, milk-alkali syndrome, thiazide, bone mets other causes: gout with urates, discoid lupus erythematosis, tuberous sclerosis Rx: removal with 3% EDTA, give topical anestheic 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

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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

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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 IIIType 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 wirh congo red, PAS, and Masson's trichrome stain recurrance post PKP common

Central crystalline (Schnyder)


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

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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 dystrophy


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

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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, lagopthalmos, 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

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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 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. 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

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Fabry's
X-linked recessive female carriers have corneal lesions accumulation of ceramide trihexoside pinhead hyperkeratotic vascular eruptions on breasts, buttocks, and extrem, parathesias 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 bx 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 flouroscein 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%

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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
Non-Pigmented
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 benign premalignant/dysplastic carcinoma-in-situ 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

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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

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 bx, 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

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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

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