96 CORNEA AND CONJUNCTIVA

Signs and Symptoms

BLUE SCLERA (Mnemonic: GNOME-P): Goltz syndrome (focal dermal hypoplasia), Ota’s nevus (oculodermal menlanocytosis), osteogenesis imperfecta type I (decreased type I collagen, autosomal dominant), Marfan syndrome, Ehlers-Danlos syndrome type VI (autosomal recessive; rule out cardiac disease), pseudoxanthoma elasticum (also may have keratoglobus)

BLEPHARITIS (Table 3–1) May be anterior (staphylococcus or seborrheic), posterior (meibomian seborrhea or posterior meibomitis), or mixed (‘‘triple S’’ syndrome: staphylococcus, seborrhea, and sicca). Patients often have keratoconjunctivitis sicca, tylosis (irregular lid margin), poliosis (lash whitening), madarosis (lash loss), distichiasis, or trichiasis. Treat all types with lid scrubs and warm compresses; may need antibiotic ointment.

Staphylococcal lid disease (SLD): characterizd by collarettes; seen mainly in younger patients, 50% of whom have tear deficiency. May cause punctate epithelial erosions (PEE), peripheral infiltrates, and phlyctenulosis (leaves a triangular scar at limbus). Ninety-five percent of normals are culture positive for Staphylococcus epidermidis, but 50% of staphylococcal blepharitis patients are culture positive for Staphylococcus aureus versus 10% of normals.

Seborrhea: characterized by scurf on lashes; coexistant seborrheic dermatitis with oily, flaky skin. Thirty-threee percent of patients have keratoconjunctivitis sicca (KCS).

Meibomian gland dysfunction (MGD): characterized by rapid TBUT, papillary reaction, and PEE; 60% of patients have rosacea. May be meibomian seborrhea with foamy tears, or posterior meibomitis with ‘‘toothpaste-like’’ secretions composed of increased cholesterol esters with increased melting point and increased free fatty acids.

Demodex follicularum: characterized by ‘‘sleeves’’ present on lashes from hair mite.

Angular blepharitis: Staphylococcus aureus is the leading cause, but classically it is from Moraxella; also Candida and HSV.

BULBAR FOLLICLES Chlamydia, sarcoid, lymphoma

CONGENITAL CORNEAL OPACIFICATION (Mnemonic: glaucoma þ STUMPED): glaucoma, sclerocornea, tears in Descemet’s membrane (e.g., forceps delivery or other trauma), ulcer (HSV, interstitial keratitis, rubella), mucopolysaccarides (MPS-1; Hurler’s syndrome), Peters’ anomaly, endothelial or stromal dystrophy (CHED < CHSD, PPMD; bilateral), and dermoid. Also chromosomal abnormalities 21, 18, 13, and XO.

CONJUNCTIVAL GRANULOMA Parinaud’s oculoglandular syndrome (usually from Bartonella/cat-scratch disease in North America, tuberculosis

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98 CORNEA AND CONJUNCTIVA

elsewhere), sarcoidosis (discrete noncaseating granulomas), fungal, parasitic (increased eosinophils), syphilis (vessel in middle of granuloma), foreign body reaction, and chalazion

CONJUNCTIVAL HYPEREMIA Inflammation (infection, allergy, toxicity, neoplasm), irritation (foreign body, eyelash), reflex (eyestrain, crying), vasodilation (alcohol use, increased oxygenation, carcinoid), autonomic dysfunction (sympathetic paresis, sphenopalatine ganglion syndrome), and vascular engorgement (venous obstruction, hyperviscosity)

CONJUNCTIVAL PIGMENTATION (Mnemonic: like a dirty ‘‘damp apron’’): drugs (e.g., chlorpromazine, adrenochrome from epinephrine), Addison’s disease, melanoma, pregnancy, argyrosis, primary acquired melanosis (PAM), radiation, ocular or oculodermal melanocytosis (congenital, blue, episcleral), nevus

CONJUNCTIVAL TELANGIECTASIAS Louis-Bar’s syndrome (ataxia telangiectasia; associated with cerebellar and immunologic abnormalities), or HIV

CONJUNCTIVAL ULCERATION Stevens-Johnson syndrome (SJS), OCP, factitious conjunctivitis, Bartonella infection

CONJUNCTIVITIS, CICATRIZING OCP, SJS, Lyell’s disease, toxic epidermal necrolysis (TEN), trauma (e.g., chemical injury foreign body), HZV, trachoma, sarcoid, prior infection (epidemic keratoconjunctivitis, diphtheria), primary Sjo¨gren’s syndrome, atopic keratoconjunctivitis (AKC), rosacea, certain drugs (e.g., practolol and glaucoma medications), malignancy (e.g., CIN, SCC, sebaceous gland CA, fibrosarcoma, lymphoma, melanoma), porphyria cutanea tarda, and lichen planus

CONJUNCTIVITIS, ACUTE FOLLICULAR Pharyngeal conjunctival fever (adenovirus), herpes simplex virus (HSV), EKC (adenovirus), hemorrhagic viral (coxsackievirus, enterovirus), chlamydia (acute trachoma or adult inclusion conjunctiva), Newcastle disease (poultry handlers), molluscum contagiosum (may also cause Parinaud’s oculoglandular syndrome) or medications (e.g., dipivefrin). Follicles are small ‘‘lymph nodes’’ in the conjunctiva.

CONJUNCTIVITIS, CHRONIC FOLLICULAR (Mnemonic: Passing tough tests mostly means late cramming): Parinaud’s oculoglandular syndrome (cat-scratch disease), trachoma, toxins (miotics, neomycin, gentamycin, atropine, Iopidine, propine), molluscum, Moraxella, Lyme disease, chlamydia.

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CONJUNCTIVITIS, HEMORRHAGIC Most cases are coxsackieviruses; also caused by enteroviruses and Newcastle disease (found in poultry workers). Conjunctivitis with subconjunctival hemorrhage.

CONJUNCTIVITIS, MEMBRANOUS Bacterial infection (streptococcus, pneumococci), ligneous conjunctivitis (true membranous), adenovirus, HSV, chemical reaction, and diphtheria (true membranous). Exudation that adheres to conjunctiva epithelium; causes bleeding when removed.

CONJUNCTIVITIS, PAPILLARY The result of infections (bacterial, chlamydia), allergic or vernal conjunctivitis, foreign body, many medications (especially glaucoma), giant papillary conjunctivitis (GPC) from contact lens wear. Papillae are inflamed mounds formed from conjunctival septae and contain a central blood vessel.

CONJUNCTIVITIS, PSEUDOMEMBRANOUS Chlamydia in newborns, SJS, superior limbal keratoconjunctivitis (SLK), OCP, and bacteria (e.g., gonococci and staphylococcus). Characterized by exudation that congeals on the conjunctiva surface; can be removed without bleeding.

CONJUNCTIVITIS, VESICULAR HSV, HZV, varicella, erythema multiforme, and OCP

CORNEAL ABRASION, RECURRENT CORNEAL EROSIONS Trauma, HSV, EBMD, stromal dystrophies, diabetes, and microcystic edema. Treat with 5% NaCl, 40% glucose ointment, bandage soft contact lens (BSCL), eyelid taping, debridement, stromal puncture, or PTK.

CORNEAL DYSTROPHIES VERSUS DEGENERATIONS Dystrophies are familial (all autosomal dominant except macular, lattice type III, and CHED type II, which are autosomal recessive), appear early in life, and are avascular, bilateral, symmetrical, central, and slowly progressive. Degenerations are acquired midto late life, vascularized, unilateral, asymmetric, peripheral, and frequently associated with systemic or other disease.

CORNEAL EDEMA, ACUTE (Mnemonic: THICK): trauma (epithelial defect, surgery), high pressure, inflammation (e.g., infection or graft rejection), contact lens hypoxia, keratoconus hydrops (ruptured Descemet’s membrane). Acute edema is often a barrier problem.

CORNEAL EDEMA, CHRONIC PPMD, iridocorneal endothelial syndrome (ICE), trauma, toxins, failed graft, PBK, ABK, and Fuchs’ dystrophy. Chronic edema is usually an endothelial pump problem.

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100 CORNEA AND CONJUNCTIVA

CORNEAL EDEMA, CONGENITAL Glaucoma, CHED, PPMD, and birth trauma (forceps delivery)

CRYSTALS IN CORNEA Schnyder’s crystalline dystrophy, interstitial crystalline keratopathy (from Streptococcus viridans), Bietti’s crystalline dystrophy, multiple myeloma, gout, uremia, cystinosis, indomethacin or chloroquine (crystals in epithelium), and calcium oxalate deposits from

Dieffenbachia plant sap

DENDRITIC KERATITIS (Mnemonic: HAZE ETC): HSV, Acanthamoeba, zoster, epithelial healing defect, Epstein-Barr virus, tyrosinemia II, and CLW

DESCEMET’S MEMBRANE BREAKS OR SCROLL Keratoconus, trauma (vertical striae usually seen with forceps delivery), and congenital glaucoma (horizontal Haab’s striae)

DISCIFORM KERATITIS HSV, HZV, mumps, varicella, chemical injury; rule out bacterial or fungal keratitis and anesthetic abuse

DRY EYE, KERATOCONJUNCTIVITIS SICCA (KCS) Hyperosmolar tears from aqueous deficiency or evaporative loss (MGD). Symptoms typically worse at the end of the day, with foreign body sensation, punctate keratopathy, interpalpebral exposure pattern seen best with 1% rose bengal stain, and filamentary keratopathy (mucous strands). Treat with artificial tears, lubrication, punctal occlusion, oral pilocarpine (not proven to increase tear secretion), or acetylcysteine 10% for mucolytic.

Sjo¨gren’s syndrome: diagnosis requires xerostomia or coexistent connective tissue disorder. Work-up includes rheumatoid factor (RF), autoantibodies against Sjo¨gren’s syndrome antigen A (SS-A/Ro; present in about 70%), and Sjo¨gren’s syndrome antigen B (SS-B/La; present in about 50%), antinuclear antibodies (ANA), and rheumatology consult. In primary Sjo¨gren’s, 90% have HLA-B8 autoantibodies. The disease is called secondary Sjo¨gren’s syndrome if another connective tissue disorder is identified.

Non–Sjo¨gren’s syndrome:

Congenital: Riley-Day syndrome (familial dysautonomia), alacrima, Adie’s syndrome

Acquired: Shy-Drager syndrome (idiopathic autonomic dysfunction), CLW

Secondary lacrimal dysfunction from inflammatory disease, trauma, anticholinergic medications, and neuroparalytic hyposecretion. Common after bone marrow transplant. Risks for KCS following bone marrow transplant include increased age, chronic graft versus host disease, female sex, and use of methotrexate.

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Meibomian gland dysfunction (MGD): characterized by lipid deficiency, increased evaporation, foreign body sensation, injection, papillary reaction, punctate erosions on inferior cornea, foamy tears, and increased TBUT. Sixty percent of cases are from rosacea; also from oral retinoids. Treat with lid hygiene.

Delayed tear currents: often coexistent with dry eye from poor pump or tear clearance (e.g., punctal plugs) and exacerbated by preservatives in many eyedrops, often with morning symptoms. Treat with nonpreserved steroids.

Loss of conjunctival goblet cells from OCP, SJS, or chemical burn

Vitamin A deficiency (xerostomia): characterized by Bitot’s spots (foamy gray triangles on bulbar conjunctiva), keratomalacia, nyctalopia

Darier’s disease (keratosis follicularis): rare autosomal recessive disorder of vitamin A metabolism causing decreased desmosomes. Presents with keratotic eyelid plaques, belpharoconjunctivitis, corneal opacities (central, whorllike), pannus, and retinitis pigmentosa (RP)– like retinal changes with flat electroretinogram (ERG) and cataract. Treat with vitamin A, isoretinoin, or steroids.

Ichthyosis: characterized by eyelid scaling, ectropion, conjunctiva thickening, and corneal opacities. Vulgaris is the most common, autosomal dominant form. Also Sjo¨gren-Larsson syndrome, RudConradi syndrome, and congenital vascularizing keratitis-icthyosiform- deafness (KID) syndrome. Worsens with Retin-A. Also seen in Refsums disease and congenital hemidysplasia with icthyosiform erythroderma and limb defects (CHILD) syndrome.

Xeroderma pigmentosum: autosomal recessive defect in DNA repair after it is damaged by UV light. Presents with photophobia, tearing, blepharospasm, and KCS, leading to exposure keratitis or ulceration. Eleven percent of patients have ocular neoplasms: SCC (primary), BCC (secondary).

EOSINOPHILS IN CONJUNCTIVA Allergy, parasite, leukemic eosinophilic granuloma, and Kimura’s disease (eosinophilic granulomas of the soft tissues, usually in Asian patients)

GRANULOMAS, CLASSIC ASSOCIATIONS Caseating ¼ tuberculosis; noncaseating ¼ sarcoid; vessel in middle ¼ syphilis; eosinophilic ¼ parasitic

HYALINE IN CORNEA Granular dystrophy, Salzmann’s nodules

HYPHEMA (Mnemonic: hemorrhages too numerous to count): HSV or HZV iritis (causes a vasculitis), trauma or surgery, neovascularization (Fuchs’ HIC, neovascularization of the iris, PDR, retinopathy of prematurity, Coats’ disease, persistent hyperplastic primary vitreous), tumor

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102 CORNEA AND CONJUNCTIVA

(juvenile xanthogranuloma, RB, leukemia, intraocular lymphoma), clotting disorder or blood dyscrasia

HYPOPYON (Mnemonic: CLUE HIT): contact lens too tight, lens reaction (intraocular lens or retained lens proteins), ulcer (bacterial, fungal, or viral keratitis), endophalmitis, HSV, iritis (any severe hyperacute uveitis, especially Behc¸et’s or B-27–related, including Reiter’s syndrome and ankylosing spondylitis), tumor (pseudohypopyon from intraocular tumor necrosis can be seen with RB, leukemia, and large cell lymphoma).

INTERSTITIAL KERATITIS (IK) 90% of cases from syphilis (87% congenital and 3% acquired; also called Grayson’s disease). Other causes include HSV, Cogan’s disorder (hearing loss, IK; associated with polyarteritis nodosa), onchocerciasis, tuberculosis, HZV, mumps, leprosy, Acanthamoeba, psoriasis, and inflammatory bowel disease. Signs: ghost vessel, stromal scarring.

KERATIC PRECIPITATES, CHARACTERISTICS Arlt’s triangle (sarcoid, inferior between 4 and 8 o’clock), diffuse (Fuchs’ HIC, HSV, cytomegalovirus retinitis, Vogt-Koyanagi-Harada disease), ‘‘greasy’’ (sarcoid, syphilis), stellate (HSV, Fuchs’ HIC, toxoplasmosis, sarcoid), geographic (tuberculosis, syphilis). Represent collections of inflammatory cells that settle on the corneal endothelium.

KERATITIS, BILATERAL (PREFERENTIALLY) Leprosy, syphilis (congenital), phlyctenulosis, Cogan’s disorder, mumps, sarcoidosis, collagen vascular diseases, systemic vasculitis, onchocerciasis, psoriasis, and inflammatory bowel disease

KERATITIS, UNILATERAL (PREFERENTIALLY) HSV, HZV, bacterial or fungal keratitis, Acanthamoeba, syphilis (acquired), and tuberculosis

LEUKOCYTES IN THE CORNEAL EPITHELIUM SLK, acute infection

LIMBAL CELL DEFICIENCY OCP, aniridia, SJS, chemical injury, multiple endocrine neoplasis (MEN) syndrome, pterygium surgery

LYMPHANGIECTASIA Dilated lympatics on bulbar conjunctiva; may be developmental or acquired from trauma or inflammation. Treat with excision.

MARGINAL LESIONS OR INFILTRATES Staphylococcal blepharitis, Herbert’s pits (trachoma), Horner-Trantas dots (vernal keratoconjunctivitis), periperal ulcerative keratitis (long differential diagnosis)

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MAST CELLS IN CONJUNCTIVA Allergy (especially AKC), OCP, pterygium, NF

NEUROTROPHIC ULCER Trigeminal nerve palsy or inflammation, HSV, HZV, surgery, strokes, tumors, chemical burn, anesthetic abuse, and diabetes. Usually oval-shaped, inferior cornea with rolled edges and stromal loss. Treat with lubrication, antibiotics, patching, lid taping, punctal occlusion, or tarsorrhaphy.

ORGANISMS THAT CAN PENETRATE AN INTACT EPITHELIUM Neisseria gonorrhoeae and meninigitis Corynebacterium diphtheriae, Haemophilus aegyptius, Listeria, Shigella. Note: Pseudomonas is not on this list.

ORGANISMS SEEN ON H & E STAIN Bacteria plus Acanthamoeba and

Mucor

PANNUS (Mnemonic: LAB VERSUS PORCH): limbic (SLK micropannus), aniridia (limbal stem cell deficiency), burns (chemical), vernal KC, staphylococcal hypersensitivity, phlyctenule, overwear or tight CL (superior pannus), rosacea, chlamydia (superior pannus from trachoma or adult inclusion conjunctivitis), HSV. Pannus is anything that is between Bowman’s layer and the epithelium; if Bowman’s layer is not intact, it is a degenerative pannus.

PERIPHERAL CORNEAL THINNING Collagen vascular disorder (rheumatoid arthritis or systemic lupus erythematosus, usually ulcerated), Terrien’s marginal degeneration, Mooren’s ulcer, pellucid marginal degeneration (‘‘shaggy cone,’’ epithelium intact), furrow degeneration (peripheral to arcus), dellen, dry eye, vernal KC, rosacea, inflammatory bowel disease, neurotrophic cornea, surgery, and leukemia.

PERIPHERAL ULCERATIVE KERATITIS (PUK) Blepharitis-associated marginal infiltrates, ulcerative keratitis connective tissue disease (RA, SLE, etc.), Mooren’s ulcer (autoimmune, chronic, progressive), HSV, and hepatitis C–associated

PIGMENT LINES IN THE CORNEA Hudson-Sta¨hli (physiologic, lower lid apposition to cornea), Ferry’s (filtering bleb), Stocker’s (pterygium), Fleischer ring (keratoconus, iron in epithelium), Kayser-Fleischer ring (Wilson’s disease, copper in Descemet’s membrane), Waring (along radial keratotomy incisions)

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